CHD Surgery Featured Articles of April 2016

  1. Surgical options after Fontan failure. van Melle JP, Wolff D, Hörer J, Belli E, Meyns B, Padalino M, Lindberg H, Jacobs JP, Mattila IP, Berggren H, Berger RM, Prêtre R, Hazekamp MG, Helvind M, Nosál M, Tlaskal T, Rubay J, Lazarov S, Kadner A, Hraska V, Fragata J, Pozzi M, Sarris G, Michielon G, di Carlo D, Ebels T.

Heart. 2016 Apr 13. pii: heartjnl-2015-309235. doi: 10.1136/heartjnl-2015-309235. [Epub ahead of print]

PMID: 27076374

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Take Home Points

  1. Any surgical intervention for a failing Fontan physiology carries significant risk of death and transplantation.
  2. Fontan takedown procedure has significant risk of early death.
  3. There is no difference in survival between Fontan conversion and transplantation.


Dr. Honjo 9-2015-2Commentary from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch:

This is an extremely important study, which is relevant for all practitioners dealing with patients with a failing Fontan physiology. This is a European multi-center study including 225 patients from 22 centers. Procedures performed are Fontan takedown in 38 (17%) patients; Fontan conversion in 137 (61%) patients; and transplantation in 50 (22%). At a median follow-up period of 5.9 years (0-23 years), the combined end point, i.e., death/transplantation, was reached in 44.7% of Fontan takedown, 26.3% of Fontan conversion, and 34% of transplantation. There was no survival difference between Fontan conversion and transplantation. Univariate analysis showed that interval from Fontan completion to reoperation, high NYHA class, and ventricular dysfunction were identified as predictors for death or transplantation, which are not significant in the multivariate analysis.


  1. Early Reoperations in a 5-Year National Cohort of Pediatric Patients with Congenital Heart Disease.

Fiorentino F, Stickley J, Dorobantu D, Pandey R, Angelini G, Barron D, Stoica S.

Ann Thorac Surg. 2016 Apr;101(4):1522-9. doi: 10.1016/j.athoracsur.2015.12.039. Epub 2016 Feb 26.

PMID: 26921979

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Take Home Points

  1. Requirement of early reoperation poses a significant risk of increase in mortality among patients who underwent an elective congenital heart surgery based on a UK national database.
  2. The ‘in-parallel’ palliations such as placement of a systemic-to-pulmonary shunt, Norwood procedure, and PA bandings are common procedures that required reoperation, which illustrates difficulty in managing postoperative physiology.
  3. Except for some simple lesions, reoperations result in significantly higher mortality than for most index procedures.


Commentary from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch: This is an excellent study from the UK using their National Congenital Heart Disease Audit database from 2005 to 2010. A great advantage of a mandatory national database is to illustrate the overall landscape of national performance compared to a voluntary society database such as CHSS. This study includes 18,489 patients, of which 652 (3.5%) required early cardiac reoperations. Among index procedures, they found that the most common types of reoperations include revision of arterial shunts, pulmonary artery banding, and ventricular septal defect repair. As a whole, the patients who required early cardiac reoperation had a much higher mortality compared to those who did not (15.8% vs. 3%, p<0.001). Approximately half (45%) of early deaths associated with reoperation were following 3 index procedures, including Norwood, arterial shunt, and pulmonary artery banding. This emphasized significant difficulty managing the physiology of palliated ‘in-parallel’ circulations. All index procedures, except ligation of patent ductus arteriosus and ventricular septal defect closure, had a significantly higher mortality if a reoperation was required.


  1. Does implantation of larger bioprosthetic pulmonary valves in young patients guarantee durability in adults? Durability analysis of stented bioprosthetic valves in the pulmonary position in patients with Tetralogy of Fallot†. Kwak JG, Lee C, Lee M, Lee CH, Jang SI, Lee SY, Park SJ, Song MK, Kim SH.

Eur J Cardiothorac Surg. 2016 Apr;49(4):1207-12. doi: 10.1093/ejcts/ezv298. Epub 2015 Aug 30.

PMID: 26324680

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Take Home Points

  1. Durability of the implanted tissue valves is largely affected by the age of the patients at the time of implantation rather than valve z-score
  2. Approximately 50% of the implanted valves in patients <20 years of age showed significant dysfunction by 10 years.
  3. Durability and functionality of the implanted tissue valves in adults are excellent at 10 years and beyond.


Commentary from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch: A biosynthetic valve has been widely used for pulmonary valve replacement or implantation in patients with pulmonary insufficiency and right ventricular dilatation following tetralogy of Fallot repair. Dr. Kwak and colleagues from South Korea analyzed 108 patients from 4 to 53 years of age who underwent pulmonary valve replacement. The most common size of the implanted valve was 25mm (63%), followed by 27mm (17%). Freedom from valve dysfunction was comparable among various valve z-scores, but was significantly lower in younger age groups: <10 years of age (approximately 50% in 8 years), and 10-20 years of age (approximately 50% in 11 years), p<0.001). Remarkably, implanted valves in patients >20 years of age showed 100% freedom from valve dysfunction beyond 10 years.

In Toronto, we also try to implant as big valve as possible with the notion that ‘the bigger valve, the longer duration’. We typically used a 29mm valve for both adults and adolescents. We often use a 33mm valve for adults with a dilated right ventricular outflow tract, and try to implant a 27mm valve as a minimum size even with little extension of a transannular patch. There is no good option for small patients <10 years of age regarding valve type and size. We triage between a pulmonary homograft vs. tissue valve if a patient really needs a competent valve.



  1. 3D Printing to Guide Ventricular Assist Device Placement in Adults with CongenitalHeartDisease and Heart Failure.  Farooqi KM, Saeed O, Zaidi A, Sanz J, Nielsen JC, Hsu DT, Jorde UP.

JACC Heart Fail. 2016 Apr;4(4):301-11. doi: 10.1016/j.jchf.2016.01.012. Review.

PMID: 27033018

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Take Home Points

  1. 3D printing technology is becoming a powerful tool in the field of congenital heart surgery.
  2. The 3D models are very useful for surgical planning, in this case, for ventricular assist device implantation.


Commentary from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch: 3D printing technology is one of the most exciting areas in congenital heart surgery. In neonates and infants, it is extremely useful to delineate complex segmental anatomy, especially the hearts with double outlet right ventricle. Dr. Farooqi and colleagues presented a 3D printing technology to guide ventricular assist device implantation in adults with congenital heart disease and heart failure. This review paper describes the target patient population, including d-transposition, status post atrial switch procedure, and complex single ventricle anatomy. The paper also illustrates the workflow of 3D printing from data acquisition to actual printing.



  1. Successful use of levosimendan as a primary inotrope in pediatric cardiac surgery: An observational study in 110 patients.

Joshi RK, Aggarwal N, Aggarwal M, Pandey R, Dinand V, Joshi R.

Ann Pediatr Cardiol. 2016 Jan-Apr;9(1):9-15. doi: 10.4103/0974-2069.171389.

PMID: 27011685 Free PMC Article

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Take Home Points

  1. Levosimendan is a new type of inotropic agent with limited experience in pediatric patients.
  2. Levosimendan acts through calcium stabilization and potassium channel opening, which are independent of the beta-adrenergic receptor pathway.
  3. There were acceptable rates and degree of adverse effects, hypotension and arrhythmia, by Levosimendan administration in pediatric population in the setting of early postoperative low output syndrome.


Commentary from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch: The mechanisms of actions include stabilization of the interaction between the calcium and troponin C that improves inotropy, and opening of a potassium channel on vascular smooth muscle that induces coronary and vascular vasodilation. Of note, another potential advantage of this agent is to have no negative effect on diastolic function. Dr. Joshi and colleagues from India reported 110 pediatric cases who were supported by Levosimendan. The agent was used in different ways—used as a primary agent or an additional agent who were already on other inotropic support. Data is somewhat hard to interpret due to heterogeneous administration profiles, but the study at least showed safety of this drug that was used in acutely in the postoperative period following various complex congenital heart surgeries. Transient hypotension happened in approximately 10% of the patients and arrhythmia was noted in less than 3%.


Congenital Heart Surgery April 2016 (titles in red may be of special interest)

  1. Clinical significance of serum cortisol levels following surgery for congenital heart disease.

Teagarden AM, Mastropietro CW.

Cardiol Young. 2016 Apr 29:1-7. [Epub ahead of print]

PMID: 27125662

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  1. Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years.

Asagai S, Inai K, Shinohara T, Tomimatsu H, Ishii T, Sugiyama H, Park IS, Nagashima M, Nakanishi T.

Congenit Heart Dis. 2016 Apr 29. doi: 10.1111/chd.12359. [Epub ahead of print]

PMID: 27126954

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  1. Single center experience of aortic bypass graft for aortic arch obstruction in children.

Shinkawa T, Chipman C, Holloway J, Tang X, Gossett JM, Imamura M.

Heart Vessels. 2016 Apr 27. [Epub ahead of print]

PMID: 27120172

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  1. Surgical management of congenital tracheal stenosis associated with tracheal bronchus and congenital heart disease.

Wang S, Zhang H, Zhu L, Zhen J, Liu J, Xu Z.

Eur J Cardiothorac Surg. 2016 Apr;49(4):1201-6. doi: 10.1093/ejcts/ezv317. Epub 2015 Sep 15.

PMID: 26374867

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  1. Neurodevelopmental outcomes after neonatal cardiac surgery: Role of cortical isoelectric activity.

Seltzer L, Swartz MF, Kwon J, Burchfiel J, Cholette JM, Wang H, Sweeney D, Adams HR, Meagher C, Angona R, Guillet R, Alfieris GM.

J Thorac Cardiovasc Surg. 2016 Apr;151(4):1137-44. doi: 10.1016/j.jtcvs.2015.10.065. Epub 2015 Oct 27.

PMID: 26707762

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  1. Burden of potentially pathologic copy number variants is higher in children with isolated congenital heart disease and significantly impairs covariate-adjusted transplant-free survival.

Kim DS, Kim JH, Burt AA, Crosslin DR, Burnham N, Kim CE, McDonald-McGinn DM, Zackai EH, Nicolson SC, Spray TL, Stanaway IB, Nickerson DA, Heagerty PJ, Hakonarson H, Gaynor JW, Jarvik GP.

J Thorac Cardiovasc Surg. 2016 Apr;151(4):1147-1151.e4. doi: 10.1016/j.jtcvs.2015.09.136. Epub 2015 Nov 10.

PMID: 26704054

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  1. Potts Shunt and Pediatric Pulmonary Hypertension: What We Have Learned.

Grady RM, Eghtesady P.

Ann Thorac Surg. 2016 Apr;101(4):1539-43. doi: 10.1016/j.athoracsur.2015.08.068. Epub 2015 Oct 28.

PMID: 26518375

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  1. Physics-driven impeller designs for a novel intravascular blood pump for patients with congenital heart disease.

Chopski SG, Fox CS, McKenna KL, Riddle ML, Kafagy DH, Stevens RM, Throckmorton AL.

Med Eng Phys. 2016 Apr 26. pii: S1350-4533(16)30043-1. doi: 10.1016/j.medengphy.2016.03.010. [Epub ahead of print]

PMID: 27129783

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  1. Evolving hybrid approaches: the preservation of the ‘neglected’ pulmonary valve function in patients with congenital heart disease.

Vida VL, Bacha E, Stellin G.

Expert Rev Cardiovasc Ther. 2016 Apr 20:1-6. [Epub ahead of print]

PMID: 26950198

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  1. Severe Acute Kidney Injury Following Stage 1 Norwood Palliation: Effect on Outcomes and Risk of Severe Acute Kidney Injury at Subsequent Surgical Stages.

Wong JH, Selewski DT, Yu S, Leopold KE, Roberts KH, Donohue JE, Ohye RG, Charpie JR, Goldberg CS, DeWitt AG.

Pediatr Crit Care Med. 2016 Apr 20. [Epub ahead of print]

PMID: 27099973

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  1. Association of Hospital Structure and Complications With Mortality After Pediatric Extracorporeal Membrane Oxygenation.

Nasr VG, Faraoni D, DiNardo JA, Thiagarajan RR.

Pediatr Crit Care Med. 2016 Apr 20. [Epub ahead of print]

PMID: 27099971

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  1. Endoventriculoplasty of the Left Ventricle for Congenital Diverticulum: A Successful Treatment and One-Year Follow-Up.

Soynov IA, Sinelnikov YS, Nichay NR, Omelchenko AY, Kornilov IA.

World J Pediatr Congenit Heart Surg. 2016 Apr 20. pii: 2150135115625204. [Epub ahead of print]

PMID: 27098606

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  1. A response to the letter by Liu et al.: Surgical correction of a Giant isolated congenital left ventricular diverticulum.

Ling Y, Fan Q, Wang Y, An Q.

Int J Cardiol. 2016 Apr 19;215:422-423. doi: 10.1016/j.ijcard.2016.04.138. [Epub ahead of print] No abstract available.

PMID: 27131262

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  1. Repair of aortic coarctation in infancy: A 10-year clinical experience.

Bigdelian H, Sedighi M.

Asian Cardiovasc Thorac Ann. 2016 Apr 15. pii: 0218492316643841. [Epub ahead of print]

PMID: 27084872

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  1. Bicuspid aortic valve complicated by pseudo-aneurysm of aortic root abscess.

Huang S, Sun JP, Sun Z, Chen Y, Li L, Hu J, Cheng Q, Yang XS, Chen D, Yu CM.

Int J Cardiol. 2016 Apr 15;209:275-7. doi: 10.1016/j.ijcard.2016.02.057. Epub 2016 Feb 3. No abstract available.

PMID: 26901788

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  1. Preoperative Intubation and Lack of Enteral Nutrition are Associated with Prolonged Stay After Arterial Switch Operation.

Iliopoulos I, Burke R, Hannan R, Bolivar J, Cooper DS, Zafar F, Rossi A.

Pediatr Cardiol. 2016 Apr 15. [Epub ahead of print]

PMID: 27084382

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  1. [Comparative results of Fontan surgery in patients with and without hypoplastic left heart syndrome].

Becker Rencoret P, Besa Bandeira S, Riveros González S, Frangini Sanhueza P, Springmüller Pinto D, González Foretic R, Urcelay Montecinos G.

Rev Chil Pediatr. 2016 Apr 14. pii: S0370-4106(16)00060-7. doi: 10.1016/j.rchipe.2016.03.001. [Epub ahead of print] Spanish.

PMID: 27091396 Free Article

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  1. Management of congenital diaphragmatic hernia with transposition of the great arteries.

Nagata H, Yamamura K, Nagata K, Uike K, Morihana E, Ihara K, Yumoto Y, Fusazaki N, Kado H, Kato K, Taguchi T, Hara T.

Pediatr Int. 2016 Apr 13. doi: 10.1111/ped.12912. [Epub ahead of print]

PMID: 27073102

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  1. Polytetrafluoroethylene Bicuspid Pulmonary Valve Replacement: A 5-Year Experience in 119 Patients With Congenital Heart Disease.

Lee C, Lee CH, Kwak JG.

Ann Thorac Surg. 2016 Apr 12. pii: S0003-4975(16)00069-2. doi: 10.1016/j.athoracsur.2016.01.056. [Epub ahead of print]

PMID: 27083247

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  1. Tracheostomy After Operations for Congenital Heart Disease: An Analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

Mastropietro CW, Benneyworth BD, Turrentine M, Wallace AS, Hornik CP, Jacobs JP, Jacobs ML.

Ann Thorac Surg. 2016 Apr 12. pii: S0003-4975(16)00058-8. doi: 10.1016/j.athoracsur.2016.01.046. [Epub ahead of print]

PMID: 27083243

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  1. Congenital pulmonary airway malformation with atrial septal defect and pulmonary hypertension for lobectomy-anesthetic considerations.

Chiluveru SA, Dave NM, Dias RJ, Garasia MB.

Ann Card Anaesth. 2016 Apr-Jun;19(2):372-4. doi: 10.4103/0971-9784.179624.

PMID: 27052089 Free Article

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  1. Serum albumin perturbations in cyanotics after cardiac surgery: Patterns and predictions.

Kapoor PM, Narula J, Chowdhury UK, Kiran U, Taneja S.

Ann Card Anaesth. 2016 Apr-Jun;19(2):300-5. doi: 10.4103/0971-9784.179633.

PMID: 27052073 Free Article

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  1. Intrapericardial rupture of a mitral subannular aneurysm: A case report and review of the literature.

Diop IB, Leye M, Diallo AD, Sarr EH, Manga SJ, Diene LL, Jobe M.

Ann Cardiol Angeiol (Paris). 2016 Apr;65(2):99-102. doi: 10.1016/j.ancard.2015.01.013. Epub 2015 Feb 4.

PMID: 25702237

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  1. Prospective Validation of a Novel Vasoactive-Ventilation-Renal Score as a Predictor of Outcomes After Pediatric Cardiac Surgery.

Miletic KG, Delius RE, Walters HL 3rd, Mastropietro CW.

Ann Thorac Surg. 2016 Apr;101(4):1558-63. doi: 10.1016/j.athoracsur.2015.11.006. Epub 2016 Feb 10.

PMID: 26872731

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  1. Mesenteric near-infrared spectroscopy and risk of gastrointestinal complications in infants undergoing surgery for congenital heart disease.

Iliopoulos I, Branco RG, Brinkhuis N, Furck A, LaRovere J, Cooper DS, Pathan N.

Cardiol Young. 2016 Apr;26(4):772-80. doi: 10.1017/S1047951115001365. Epub 2015 Sep 7.

PMID: 26343176

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  1. Matching the Market for Heart Transplantation.

Hsich EM.

Circ Heart Fail. 2016 Apr;9(4). pii: e002679. doi: 10.1161/CIRCHEARTFAILURE.115.002679.

PMID: 27072859

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  1. Aid decision algorithms to estimate the risk in congenital heart surgery.

Ruiz-Fernández D, Monsalve Torra A, Soriano-Payá A, Marín-Alonso O, Triana Palencia E.

Comput Methods Programs Biomed. 2016 Apr;126:118-27. doi: 10.1016/j.cmpb.2015.12.021. Epub 2016 Jan 2.

PMID: 26774238

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  1. Anaesthetic challenges in a child with sickle-cell disease and congenital heart block.

Bala I, Sahni N, Mitharwal SM.

Indian J Anaesth. 2016 Apr;60(4):294-5. doi: 10.4103/0019-5049.179473. No abstract available.

PMID: 27141119

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  1. Conflicting Hemodynamic Goals in an Adult Patient With Fontan Physiology Presenting for Resection of a Hepatocellular Carcinoma.

Dorsey D, Kwon S, Krieger E, Yeung RS, Natrajan K, Dembo G.

J Cardiothorac Vasc Anesth. 2016 Apr;30(2):452-4. doi: 10.1053/j.jvca.2015.08.032. Epub 2015 Sep 1. No abstract available.

PMID: 26723881

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  1. Role of intraoperative transesophageal echocardiography in pediatric cardiac surgery.

Jijeh AM, Omran AS, Najm HK, Abu-Sulaiman RM.

J Saudi Heart Assoc. 2016 Apr;28(2):89-94. doi: 10.1016/j.jsha.2015.06.005. Epub 2015 Jul 10.

PMID: 27053898 Free PMC Article

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  1. Long-Term Follow-Up of the Half-Turned Truncal Switch Operation for Transposition of the Great Arteries with Ventricular Septal Defect and Pulmonary Stenosis.

Lee JU, Jang WS, Lee YO, Cho JY.

Korean J Thorac Cardiovasc Surg. 2016 Apr;49(2):112-4. doi: 10.5090/kjtcs.2016.49.2.112. Epub 2016 Apr 5.

PMID: 27064891 Free PMC Article

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  1. Biventricular Repair after Bilateral Pulmonary Artery Banding as a Rescue Procedure for a Neonate with Hypoplastic Left Heart Complex.

Yun JK, Bang JH, Kim YH, Goo HW, Park JJ.

Korean J Thorac Cardiovasc Surg. 2016 Apr;49(2):107-11. doi: 10.5090/kjtcs.2016.49.2.107. Epub 2016 Apr 5.

PMID: 27064769 Free PMC Article

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  1. Myocardial Oxidative Stress in Infants Undergoing Cardiac Surgery.

Sznycer-Taub N, Mackie S, Peng YW, Donohue J, Yu S, Aiyagari R, Charpie J.

Pediatr Cardiol. 2016 Apr;37(4):746-50. doi: 10.1007/s00246-016-1345-3. Epub 2016 Feb 3.

PMID: 26843460

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  1. Hyperoxia Is Associated With Poor Outcomes in Pediatric Cardiac Patients Supported on Venoarterial Extracorporeal Membrane Oxygenation.

Sznycer-Taub NR, Lowery R, Yu S, Owens ST, Hirsch-Romano JC, Owens GE.

Pediatr Crit Care Med. 2016 Apr;17(4):350-8. doi: 10.1097/PCC.0000000000000655.

PMID: 27043897

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  1. A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum.

Kotani Y, Kasahara S, Fujii Y, Eitoku T, Baba K, Otsuki SI, Kuroko Y, Arai S, Sano S.

Eur J Cardiothorac Surg. 2016 Apr 26. pii: ezw124. [Epub ahead of print]

PMID: 27118312

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  1. Pulsatile blood flow in total cavopulmonary connection: a comparison between Y-shaped and T-shaped geometry.

Rajabzadeh-Oghaz H, Firoozabadi B, Saidi MS, Monjezi M, Navabi Shirazi MA, Malakan Rad E.

Med Biol Eng Comput. 2016 Apr 23. [Epub ahead of print]

PMID: 27108290

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  1. Allosensitization does not alter post-transplant outcomes in pediatric patients bridged to transplant with a ventricular assist device.

Castleberry C, Zafar F, Thomas T, Khan MS, Bryant R, Chin C, Morales DL, Lorts A.

Pediatr Transplant. 2016 Apr 21. doi: 10.1111/petr.12706. [Epub ahead of print]

PMID: 27102953

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  1. Pharmacodynamic Analysis of Morphine Time-to-Remedication Events in Infants and Young Children After Congenital Heart Surgery.

Elkomy MH, Drover DR, Galinkin JL, Hammer GB, Glotzbach KL.

Clin Pharmacokinet. 2016 Apr 20. [Epub ahead of print]

PMID: 27098060

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Select item 26477644


  1. Severe Pulmonary Vascular Obstructive Disease After Neonatal Arterial Switch Operation for Simple Transposition of the Great Arteries.

Kimura D, Briceno-Medina M, Kumar TK, Knott-Craig CJ.

World J Pediatr Congenit Heart Surg. 2016 Apr 20. pii: 2150135115623288. [Epub ahead of print]

PMID: 27098605

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  1. Intramyocardial Hematoma After Ebstein Anomaly Repair: A Case of Unexpected Diagnosis and Its Management.

Lim JK, Lee JH, Mok YH, Chen CK, Loh YJ.

World J Pediatr Congenit Heart Surg. 2016 Apr 20. pii: 2150135115623528. [Epub ahead of print]

PMID: 27098604

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  1. Preoperative Intubation and Lack of Enteral Nutrition are Associated with Prolonged Stay After Arterial Switch Operation.

Iliopoulos I, Burke R, Hannan R, Bolivar J, Cooper DS, Zafar F, Rossi A.

Pediatr Cardiol. 2016 Apr 15. [Epub ahead of print]

PMID: 27084382

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Select item 27084381


  1. Pulmonary Valve Repair for Patients With Acquired Pulmonary Valve Insufficiency.

Said SM, Mainwaring RD, Ma M, Tacy TA, Hanley FL.

Ann Thorac Surg. 2016 Apr 12. pii: S0003-4975(16)00046-1. doi: 10.1016/j.athoracsur.2016.01.035. [Epub ahead of print]

PMID: 27083251

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  1. Timely Post-discharge Telephone Follow-Up is a Useful Tool in Identifying Post-discharge Complications Patients After Congenital Heart Surgery.

Lushaj EB, Nelson K, Amond K, Kenny E, Badami A, Anagnostopoulos PV.

Pediatr Cardiol. 2016 Apr 11. [Epub ahead of print]

PMID: 27064092

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  1. Bilateral Giant Coronary Artery Aneurysms Complicated by Acute Coronary Syndrome and Cardiogenic Shock.

Chiu P, Lynch D, Jahanayar J, Rogers IS, Tremmel J, Boyd J.

Ann Thorac Surg. 2016 Apr;101(4):e95-7. doi: 10.1016/j.athoracsur.2015.06.104.

PMID: 27000621

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Select item 27000620


  1. Ductus Arteriosus Banding to Facilitate Stenting During the Hybrid Stage I Procedure.

Trezzi M, Bradley SM, Bandisode VM, Kavarana MN.

Ann Thorac Surg. 2016 Apr;101(4):e133-5. doi: 10.1016/j.athoracsur.2015.10.013.

PMID: 27000620

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Select item 27000580


  1. Early Reoperations in a 5-Year National Cohort of Pediatric Patients With Congenital Heart Disease.

Fiorentino F, Stickley J, Dorobantu D, Pandey R, Angelini G, Barron D, Stoica S.

Ann Thorac Surg. 2016 Apr;101(4):1522-9. doi: 10.1016/j.athoracsur.2015.12.039. Epub 2016 Feb 26.

PMID: 26921979

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Select item 26857638


  1. Vulnerability of Coronary Circulation After Norwood Operation.

Saiki H, Kuwata S, Kurishima C, Masutani S, Senzaki H.

Ann Thorac Surg. 2016 Apr;101(4):1544-51. doi: 10.1016/j.athoracsur.2015.10.077. Epub 2016 Feb 6.

PMID: 26857638

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  1. Levoatriocardinal vein and partial anomalous pulmonary vein drainage in left-sided obstructive CHDs: diagnostic and surgical implications.

Marini D, Agnoletti G, Pace Napoleone C.

Cardiol Young. 2016 Apr;26(4):811-4. doi: 10.1017/S1047951115002425. Epub 2015 Oct 28.

PMID: 26507123

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  1. The Norwood operation: Relative effects of surgeon and institutional volumes on outcomes and resource utilization.

Anderson BR, Ciarleglio AJ, Cohen DJ, Lai WW, Neidell M, Hall M, Glied SA, Bacha EA.

Cardiol Young. 2016 Apr;26(4):683-92. doi: 10.1017/S1047951115001031. Epub 2015 Jul 14.

PMID: 26169083 Free PMC Article

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Select item 26105182


  1. Catch-up growth in term and preterm infants after surgical closure of ventricular septal defect in the first year of life.

Correia Martins L, Lourenço R, Cordeiro S, Carvalho N, Mendes I, Loureiro M, Patrício M, Anjos R.

Eur J Pediatr. 2016 Apr;175(4):573-9. doi: 10.1007/s00431-015-2676-4. Epub 2015 Dec 9.

PMID: 26646145

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Select item 25643248


  1. Serum Concentrations of Interleukin-6, Procalcitonin, and C-Reactive Protein: Discrimination of Septical Complications and Systemic Inflammatory Response Syndrome after Pediatric Surgery.

Neunhoeffer F, Plinke S, Renk H, Hofbeck M, Fuchs J, Kumpf M, Zundel S, Seitz G.

Eur J Pediatr Surg. 2016 Apr;26(2):180-5. doi: 10.1055/s-0034-1544047. Epub 2015 Feb 2.

PMID: 25643248

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  1. Double Switch Procedure and Surgical Alternatives for the Treatment of Congenitally Corrected Transposition of the Great Arteries.

Bilal MS, Avşar MK, Yıldırım Ö, Özyüksel A, Zeybek C, Küçükosmanoğlu O, Demiroluk Ş.

J Card Surg. 2016 Apr;31(4):231-6. doi: 10.1111/jocs.12728. Epub 2016 Mar 8.

PMID: 26956686

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Select item 26619954


  1. Left ventricular non-compaction cardiomyopathy in children listed for heart transplant: Analysis from the Pediatric Heart Transplant Study Group.

Lal AK, Pruitt E, Hong BJ, Lin KY, Feingold B.

J Heart Lung Transplant. 2016 Apr;35(4):540-2. doi: 10.1016/j.healun.2015.12.010. Epub 2016 Jan 6. No abstract available.

PMID: 26899765

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Select item 26774383


  1. Statin therapy is not associated with improved outcomes after heart transplantation in children and adolescents.

Greenway SC, Butts R, Naftel DC, Pruitt E, Kirklin JK, Larsen I, Urschel S, Knecht K, Law Y.

J Heart Lung Transplant. 2016 Apr;35(4):457-65. doi: 10.1016/j.healun.2015.10.040. Epub 2015 Nov 22.

PMID: 26746989

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Select item 26653933


  1. Impact of ventricular assist device placement on longitudinal renal function in children with end-stage heart failure.

May LJ, Montez-Rath ME, Yeh J, Axelrod DM, Chen S, Maeda K, Almond CS, Rosenthal DN, Hollander SA, Sutherland SM.

J Heart Lung Transplant. 2016 Apr;35(4):449-56. doi: 10.1016/j.healun.2015.10.039. Epub 2015 Nov 11.

PMID: 26653933

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Select item 26016596


  1. Epigallocatechin gallate attenuates cardiopulmonary bypass-associated lung injury.

Kasper B, Salameh A, Krausch M, Kiefer P, Kostelka M, Mohr FW, Dhein S.

J Surg Res. 2016 Apr;201(2):313-25. doi: 10.1016/j.jss.2015.11.007. Epub 2015 Nov 11.

PMID: 27020813

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  1. Impact of Timing of ECMO Initiation on Outcomes After Pediatric Heart Surgery: A Multi-Institutional Analysis.

Gupta P, Robertson MJ, Rettiganti M, Seib PM, Wernovsky G, Markovitz BP, Simsic J, Tobias JD.

Pediatr Cardiol. 2016 Apr 1. [Epub ahead of print]

PMID: 27037549

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Select item 27028328


  1. Early Initiation of Renal Replacement Therapy in Pediatric Heart Surgery Is Associated with Lower Mortality.

Sanchez-de-Toledo J, Perez-Ortiz A, Gil L, Baust T, Linés-Palazón M, Perez-Hoyos S, Gran F, Abella RF.

Pediatr Cardiol. 2016 Apr;37(4):623-8. doi: 10.1007/s00246-015-1323-1. Epub 2015 Dec 21.

PMID: 26687178

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Select item 27043910


  1. Fluid Overload Is Associated With Higher Mortality and Morbidity in Pediatric Patients Undergoing Cardiac Surgery.

Lex DJ, Tóth R, Czobor NR, Alexander SI, Breuer T, Sápi E, Szatmári A, Székely E, Gál J, Székely A.

Pediatr Crit Care Med. 2016 Apr;17(4):307-14. doi: 10.1097/PCC.0000000000000659.

PMID: 26914622

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Select item 27028947


  1. Aortic complications following pediatric heart transplantation: A case series and review.

Lang SM, Frazier EA, Collins RT 2nd.

Ann Pediatr Cardiol. 2016 Jan-Apr;9(1):42-5. doi: 10.4103/0974-2069.171354.

PMID: 27011691 Free PMC Article

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Pediatric Cardiology Featured Articles of March 2016

1. Genetic link between renal birth defects and congenital heart disease.

San Agustin JT, Klena N, Granath K, Panigrahy A, Stewart E, Devine W, Strittmatter L, Jonassen JA, Liu X, Lo CW, Pazour GJ.

Nat Commun. 2016 Mar 22;7:11103. doi: 10.1038/ncomms11103.

PMID: 27002738 Free PMC Article

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Select item 27006349

Take Home Points:

  1. This study on mutant mice with CHD suggests that renal abnormalities are associated with CHD.
  2. This study also confirmed similar findings in human subjects retrospectively with 30% of patients with CHD having documented renal abnormalities.
  3. Kidney evaluation may be beneficial in patients with CHD.

Abarbanell picture smallCommentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: This study evaluated renal abnormalities in mouse mutants identified to have congenital heart disease (CHD).  Researchers screened more than 80,000 fetal mice with in utero ultrasound imaging (4200 mutant lines) which revealed a wide spectrum of CHD.  Causative mutations for CHD could be identified in 135 mutant lines by whole exome sequencing and genotyping.  Non-cardiac abnormalities within the CHD mutants were characterized and there was a high prevalence of renal abnormalities in the CHD mutants.  39 of the 135 CHD mutant lines were observed to have kidney defects (See table 1).  The most common renal abnormality seen in the CHD mutants was a duplex kidney.  Researchers also conducted a retrospective clinical review of human patients (n=77) with CHD requiring surgical intervention.  30% of the human patients with CHD had renal defects.  See figure 5.  Researchers did not find a correlation between cardiac lesion type and the different renal abnormalities.  Researchers conclude  that the findings “probably reflects the conservation of developmental pathways and cell signaling mechanisms that regulate cardiovascular and renal development…In addition this work indicates that CHD patients would benefit from routine evaluation for renal anomalies to reduce potential renal complications and improve outcome in this high-risk patient population.”

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2. Clinical and Financial Impact of Ordering an Echocardiogram in Children with Left Axis Deviation on Their Electrocardiogram.

Ravi P, Ashwath R, Strainic J, Li H, Steinberg J, Snyder C.

Congenit Heart Dis. 2016 Mar;11(2):110-4. doi: 10.1111/chd.12294. Epub 2015 Aug 24.

PMID: 26303069

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Select item 26915011

Take Home Points:

  1. Left axis deviation on ECG in infancy is associated with significant CHD. However, in older patients with a normal physical examination the finding of LAD is most likely a benign finding.
  2. In the setting of an ECG with LAD only in older patients, a normal physical examination would suggest that no further work-up is needed.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: How valid is left axis deviation (LAD) on ECG beyond infancy in identifying structural congenital heart disease (CHD)?  This retrospective study would suggest that beyond infancy the physical examination by a cardiologist can be the used to determine whether an echocardiogram is warranted in the presence of LAD on an ECG.  It is well known that in early infancy, LAD on the ECG can be associated with atrioventricular canal defects, Ebstein anomaly of the tricuspid valve, tricuspid atresia, hypoplastic left heart syndrome, and Wolff–Parkinson–White syndrome.  However beyond infancy the sensitivity and specificity of LAD on ECG to predict CHD is unknown.  In this study, 146 patients (age 4 months to 18 years) with an abnormal ECG with LAD that were seen by cardiology and received an echocardiogram were reviewed.  Results found that 46.5% (68) had a normal physical examination (PE) and ECHO, 1.4% (2) had a normal PE and abnormal ECHO, 47.3% (69) had an abnormal PE and ECHO, and 4.8% (7) had an abnormal PE and normal ECHO. The sensitivity and specificity of PE for detecting abnormalities in this population was 97% and 90%. Positive and negative predictive value of PE was 91% and 97.5%. In patients with normal PE, the cost to identify an ECHO abnormality was $8365, and $263 for those with abnormal PE.  The 2 patients that had LAD on ECG with a normal PE and abnormal echocardiogram were found to have incidental CHD (bicuspid aortic valve and anomalous right coronary artery from left sinus of Valsalva).  The researchers conclude “The study provides evidence that in pediatric patients with LAD on their resting ECG, one should rely on the PE performed by a pediatric cardiologist as a highly sensitive and specific marker with regard to the presence of CHD”.

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3. Paracetamol for the treatment of patent ductus arteriosus in preterm neonates: a systematic review and meta-analysis.

Terrin G, Conte F, Oncel MY, Scipione A, McNamara PJ, Simons S, Sinha R, Erdeve O, Tekgunduz KS, Dogan M, Kessel I, Hammerman C, Nadir E, Yurttutan S, Jasani B, Alan S, Manguso F, De Curtis M.

Arch Dis Child Fetal Neonatal Ed. 2016 Mar;101(2):F127-36. doi: 10.1136/archdischild-2014-307312. Epub 2015 Aug 17.

PMID: 26283668

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Select item 26411865

Take Home Points:

  1. Systematic review and meta-analysis of the literature would suggest that paracetamol may have a clinical role in the management of PDA.
  2. The effectiveness of paracetamol compared to ibuprofen in this review was similar with no increased safety risk.
  3. Further well-designed studies are needed to support the use of paracetamol for treatment of PDA.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: There has been increasing research suggesting that paracetamol (acetaminophen) is an effective treatment for patent ductus arteriosus (PDA) closure in premature infants similar to ibuprofen and indomethacin.  This systematic review and meta-analysis from Italy found that paracetamol is as effective as ibuprofen with no increase in side effects.  This systematic review of the literature identified 16 studies (2 randomized control trials (RCT) and 14 uncontrolled studies) using their inclusion criteria.  Results of the meta- analysis are as follows:

  1. Meta-analysis of RCT (See tables 2 and 3)
  • No difference in PDA closure with paracetamol compared to ibuprofen, after 3 and 6 days of treatment
  • No significant difference between the paracetamol and ibuprofen groups in terms of mortality, morbidity or ductal reopening
  • Risk of hyperbilirubinaemia was higher for ibuprofen compared to paracetamol
  1. Uncontrolled studies (See table 4 and 5)
  • A pooled ductal closure rate of 49% and 76% after 3 and 6 days of treatment with paracetamol
  • Safety profiles of paracetamol and ibuprofen are similar
  • A significant improvement in efficacy was observed when paracetamol was used in subjects with GA 28 weeks, postnatal age <7 days and when it was used as first-line therapy
  • There was a trend to greater benefit when paracetamol was used by oral route and at lower dose
  • A transient increase in aspartate and alanine aminotransferases or γ-glutamyl transpeptidase was reported only in six patients enrolled in three of the 14 uncontrolled studies

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4. Changes in Ventricular Geometry Predict Severity of Right Ventricular Hypertension.

Averin K, Michelfelder E, Sticka J, Cash M, Hirsch R.

Pediatr Cardiol. 2016 Mar;37(3):575-81. doi: 10.1007/s00246-015-1317-z. Epub 2015 Dec 14.

PMID: 26667960

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Select item 26667958

Take Home Points:

  1. Left ventricular end-systolic eccentricity index (LVEI) can be easily measured from the parasternal short axis images.
  2. LVEI demonstrated a significant correlation with the right ventricular pressure/peak systolic aortic pressure obtained by cardiac catheterization.
  3. A LVEI >48 demonstrated a sensitivity of 76 % and specificity of 100 % in predicting RVp/pAo >0.50, while a LVEI >1.24 had a sensitivity of 88 % and specificity of 83 %.
  4. LVEI seems to be helpful echocardiographic measurement in evaluating the severity of pulmonary hypertension.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: This retrospective study from Cincinnati Children’s Hospital Medical Center correlated echocardiographic findings of pulmonary hypertension with cardiac catheterization finding with particular interest to the left ventricular end-systolic eccentricity index (LVEI) and cardiac catheterization measurements of the ratio of peak systolic right ventricular pressure (RVp) to peak systolic aortic pressure (pAo).  The LVEI is easily measured from the echocardiographic parasternal short axis images.  See figure 1.  46 studies in 29 subjects (median age 3.8 years), with a median time from echocardiogram to catheterization of 1.0 days, were analyzed.  Results demonstrated a statistically significant correlation between the LVEI and RVp/pAo (r =  0.76, p <0.001), mean PA pressure (r =  0.73, p<0.001), transpulmonary gradient (r =  0.74, p< 0.001) and indexed pulmonary vascular resistance (r = 0.49, p =  0.001).  See figure 2.  An LVEI >1.48 was found to have a sensitivity of 76 % and specificity of 100 % in predicting RVp/pAo >0.50, while a LVEI >1.24 had a sensitivity of 88% and specificity of 83 %. LVEI seems to be helpful echocardiographic measurement in evaluating the severity of pulmonary hypertension.

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5. Pediatric Cardiac Intensive Care Society 10th International Conference 2014 Consensus Statement: Pharmacotherapies in Cardiac Critical Care.

Salvin JW, Bronicki R, Costello JM, Moffett B, Procaccini D.

Pediatr Crit Care Med. 2016 Mar;17(3 Suppl 1):S1-2. doi: 10.1097/PCC.0000000000000634. No abstract available.

PMID: 26945323

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Select item 26928105

Take Home Point:

  1. These consensus statements on pharmacotherapies in cardiac critical are succinct and will most likely be very useful for those practicing in pediatric cardiac intensive care.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch:  Supplement 1 of the Pediatric Critical Care Journal has several consensus statements from the Pediatric Cardiac Intensive Care Society 10th international conference 2014.  Consensus statements included in the supplement include the following:

  1. Pharmacotherapies in cardiac critical care sedation, analgesia and muscle relaxants
  2. Pharmacotherapies in cardiac critical care treatment of acute heart failure
  3. Pharmacotherapies in cardiac critical care chronic heart failure
  4. Pharmacotherapies in cardiac critical care fluid management
  5. Pharmacotherapies in cardiac critical care antiarrhythmics
  6. Pharmacotherapies in cardiac critical care hormone replacement therapy
  7. Pharmacotherapies in cardiac critical care immune therapy
  8. Pharmacotherapies in cardiac critical care anticoagulation and thrombosis
  9. Pharmacotherapies in cardiac critical care pulmonary hypertension
  10. Pharmacotherapies in cardiac critical care antihypertensives

These consensus statements on pharmacotherapies in cardiac critical are succinct and will most likely be very useful for those practicing in pediatric cardiac intensive care.



6. Resource Utilization for Noncardiac Admissions in Pediatric Patients with Single Ventricle Disease.

Thomas ID, Seckeler MD.

Am J Cardiol. 2016 Mar 2. pii: S0002-9149(16)30303-4. doi: 10.1016/j.amjcard.2016.02.043. [Epub ahead of print]

PMID: 27018934

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Select item 26932151

Take Home Points: 

  1. Health care resource utilization for noncardiac hospital admissions in patients with SV CHD was significantly higher than non CHD patients.
  2. Infants with SV CHD admitted with noncardiac issues are at highest risk of mortality.
  3. Hospitalization for noncardiac diagnoses may be preventable with good outpatient management and preventive care.

Shaji Menon Portrait 12.15.14Commentary from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch: This study compare the costs and outcomes for common noncardiac hospitalizations between patients (<18 years) with single ventricle (SV) and patients without congenital heart disease (CHD). The data for this study was obtained from University Health System Consortium discharge data from January 2011 to December 2013. The University Health System Consortium (UHC) is an alliance of 115 academic medical centers and 165 affiliated hospitals. There were a total of 893,264 admissions for patients without CHD (median age 8.1 years, range 1 month to 18 years) and 2,515 noncardiac admissions for patients with SV CHD (median age 1.8 years, range 1 month to 18 years). The SV CHD admissions were more in the younger ages compared to a bimodal distribution in the non CHD cohort.  AKI had the highest ICU admission rate and mortality in patients with SV CHD. Fifteen (88%) of the deaths in patients with SV CHD were in children aged <1 year. Total length of stay (LOS) was significantly longer and costs higher for almost all diagnoses for patients with SV CHD. AKI had the longest LOS for both SV CHD and patients without SV CHD.

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7. Congenital Heart Disease and Primary Ciliary Dyskinesia.

Harrison MJ, Shapiro AJ, Kennedy MP.

Paediatr Respir Rev. 2016 Mar;18:25-32. doi: 10.1016/j.prrv.2015.09.003. Epub 2015 Sep 26. Review.

PMID: 26545972

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Select item 26739006

Take Home Points: 

  1. There is increasing evidence of PCD in patients with complex CHD.
  2. Screening for PCD is recommended in all children with complex congenital heart disease, especially those with disorders of laterality, transposition of the great vessels, double-outlet right ventricle, anomalous venous return, interrupted inferior vena cava and bilateral superior vena cava.
  3. PCD should be considered in patients with above mentioned heart diseases and recurrent oto-sino-pulmonary symptoms.
  4. PCD in patients with CHD may increase morbidity and mortality.

Commentary from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch: Primary Ciliary Dyskinesia (PCD) is an autosomal recessive disorder of ciliary motility resulting in defective mucociliary clearance. There is an increases incidence of ciliary dyskinesia in patients with congenital heart disease (CHD). There is increasing evidence of PCD in patients with complex CHD. Recent studies have shown that approximately 50% of patients with PCD have organ laterality defects and approximately 3-6% having cardiovascular malformations and at least 2.6% having a complex cardiovascular defect. This review explores this relationship between PCD and CHD. There is lack of a comprehensive genetic test for PCD. The PCD ERS Consensus Statement on Diagnostic and Treatment Approaches in Children recommends screening for PCD in all children with complex congenital heart disease, especially those with disorders of laterality, transposition of the great vessels, double-outlet right ventricle, anomalous venous return, interrupted inferior vena cava and bilateral superior vena cava. The test for PCD include nasal nitric oxide measurement, ciliary electron microscopy, or functional ciliary analysis, thoraco-abdominal imaging to evaluate for organ laterality. PCD should be considered in patients with above mentioned heart diseases and recurrent oto-sino-pulmonary symptoms. Patients with PCD have a significantly increased risk of postop respiratory complications, need for tracheostomy, and use of beta-agonists.  CHD patient with heterotaxy, in general experience higher morbidity and mortality and this may be partly secondary to PCD.

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ACHD Featured Articles of March 2016

1. Hepatic stiffness in the bidirectional cavopulmonary circulation: The Liver Adult-Pediatric-Congenital-Heart-Disease Dysfunction Study group.

Kutty SS, Zhang M, Danford DA, Hasan R, Duncan KF, Kugler JD, Quiros-Tejeira RE, Kutty S.

J Thorac Cardiovasc Surg. 2016 Mar;151(3):678-84. doi: 10.1016/j.jtcvs.2015.09.079. Epub 2015 Sep 28.

PMID: 26515874

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Select item 26481279

Take Home Points:

  1. Hepatic stiffness is increased in very young children with single ventricle physiology after BCPC and before Fontan, and is mildly elevated compared to healthy age- and gender-matched controls.
  2. Hepatic stiffness measurement using shear wave elastography is feasible in small children and could be used to monitor disease progression.
  3. In a modern cohort of Fontan patients, who are now teenagers, hepatic stiffness is significantly higher than in young children awaiting Fontan palliation.

Kay_William_MD.16.CVaCommentary from W. Aaron Kay (Indianapolis), section co-editor of ACHD Journal Watch: Much has been written about liver disease in single ventricle patients after the Fontan.  Very little to date has been published regarding hepatic changes at earlier stages.  In this paper from the University of Nebraska, patients were evaluated for liver stiffness as infants at the bidirectional cavopulmonary connection (BCPC) phase, and compared to age-matched controls, as well as to patients who underwent Fontan palliation (n=20 BCPC patients, 20 controls, data compared to a historic cohort after Fontan procedure).

Patient were an average of 18 months status post BCPC surgery, and 90% had normal systolic function by echo, and 90% had mild or less AV valve regurgitation.  Half of patients had HLHS and the other half had a single left ventricle.  The investigators measured hepatic stiffness using shear wave elastography (SWE) and correlated this measurement with other parameters, such as portal vein flow and resistance of celiac and superior mesenteric arteries.  The hepatic stiffness in BCPC was significantly higher than in control subjects (7.2 vs 5.7 kPa, p = .039).  Patients after BCPC also had significantly higher celiac artery resistive index, pulsatility index, and systolic-diastolic flow ratio.  The portal vein flow was reduced in BCPC patients, with higher resistive index and higher pulsatility index than in controls.

A significant correlation was found between hepatic stiffness with right atrial pressure obtained at catheterization (p = .002).  However, patients at BCPC stage still had lower stiffness than the historical cohort of patients who had already undergone the Fontan procedure (7.2 vs 15.6 kPa, P < .001).  The historical cohort of Fontan data was published in 2014, by the same investigators, and was based on 41 patients, averaging 13.8 years in age, at an average of 11 ± 6 years after Fontan completion.

This paper is the first paper to report on findings of hepatic stiffness based off shear wave elastography in children with single ventricles after the BCPC and prior to Fontan completion.  It is remarkable that out of only 22 patients in their entire program awaiting Fontan completion, 20 were enrolled in the study.  More robust data will thus require multicenter investigation.  It is uncertain if the elastography measures intrinsic liver stiffness, or if (potentially reversible) elevations of central venous pressure also raises the level of stiffness assessed in kPa.  There are numerous other causes for liver damage, other than congestion, in single ventricle patients, including hypoxia, CHF, shock, palliative shunting, and the effects of cardiopulmonary bypass.  However, this study is a nice starting point, and this technique could feasibly be used to a broader number of patients at more centers to obtain serial measurements.

A key limitation to this study is that hepatic stiffness was not assessed prior to BCPC, so BCPC itself cannot be assumed to be a cause of hepatic stiffness based on the data obtained.  Another limitation is that there was no correlation with liver biopsy, given ethical limitations in doing liver biopsies on young children without obvious clinical indication.

achd 1.1

FIGURE 1. Shear wave elastogram and vascular Doppler indices are shown. Elastogram maps show hepatic stiffness measurements by SWE in (A) a subject who had BCPC, and (B) a healthy subject. Hepatic ultrasound Doppler measurements are shown for (C) the main portal vein; (D) the celiac artery; and (E) the superior mesenteric artery. Min, Minimum; Max, maximum; Diam, diameter; SWE, shear wave elastography; BCPC, bidirectional cavopulmonary connection; PSV, peak systolic velocity; EDV, end-diastolic velocity; RI, resistive index; PI, pulsatility index; AT, acceleration time; TAPV, time average peak velocity; TAMV, time average mean velocity; AI, acceleration index; PG, peak gradient; VTI, velocity-time integral; HR, heart rate; VFlow, flow volume.



2. Improved Exercise Performance in Patients With Tricuspid Atresia After the Fontan-Björk Modification With Pulsatile Systolic Pulmonary Flow.

Ono M, Vogt M, Cleuziou J, Kasnar-Samprec J, Burri M, Strbad M, Hager A, Schreiber C, Hörer J, Lange R.

Ann Thorac Surg. 2016 Mar;101(3):1012-9. doi: 10.1016/j.athoracsur.2015.09.085. Epub 2015 Dec 8.

PMID: 26680312

Similar articles

Select item 26830903

Take Home Points:

  1. Patients after the Björk modification of the Fontan procedure have larger right ventricles and better exercise capacity if they have pulsatile rather than nonpulsatile pulmonary blood flow. Exercise capacity of these patients was similar to that of patients who have had more modern TCPC Fontan operations.
  2. At rest, the only difference in hemodynamics between the groups was a higher peak systolic pulmonary artery blood pressure in the group with pulsatile pulmonary blood flow, but there was no difference in mean RA pressure, mean PA pressure, PVR, or end-diastolic pressure. Given the improved exercise capacity in Group P over Group N, it is likely that there would be a different invasive hemodynamics during exercise.
  3. No significant predictor was found in preoperative data between Group P and Group N that could have predicted preoperatively which patients would develop pulsatile pulmonary blood flow. However, there was a higher transpulmonic gradient in 24/30 in Group N who had never had pulsatile flow versus the combination of the Group P patients and the 6/30 in Group N who initially had but lost pulsatile blood flow.
  4. If reliable factors that can predict pulsatile systolic pulmonary blood flow could be identified, the Björk modification could potentially be offered in a select subset of patients with tricuspid atresia.

Commentary from Dr. W. Aaron Kay (Indianapolis), section co-editor of ACHD Journal Watch:  The Björk modification of the Fontan, performed only for the diagnosis of tricuspid atresia or double-inlet left ventricle, is an abandoned procedure in which a surgical connection (see Figure below) is made to connect the right atrium to the right ventricle.   This procedure was eventually abandoned in favor of the TCPC (extracardiac or lateral tunnel Fontan) due to concerns of stenosis and regurgitation over the atrioventricular connection, as well as due to arrhythmias and unfavorable hemodynamics.

The rationale behind the Björk modification is to include the RV as a potential pumping chamber to augment forward flow to the pulmonary circulation.  Prior studies had shown no advantage to this versus the classic atriopulmonary connection, but some reports had shown that the RV in some cases grew significantly.  A hypothesis was thus made that patients with pulsatile systolic pulmonary blood flow would have larger RVs and thus would have better outcomes.

This study, from Munich, Germany, tested this hypothesis by evaluating  long term outcomes of 41 patients after the Björk modification, and compared complications of those with pulsatile pulmonary flow (Group P, n = 11) with those without no pulsatility of pulmonary flow (Group N, n = 30).  Parameters evaluated included hemodynamics, right ventricular size, exercise capacity, and clinical outcomes.  Mean follow-up time was over 25 years in both groups.  Prior to 1995, 173 patients at Munich had undergone classic Fontan (n=106 atriopulmonary connection, n=67 Björk modification).  Mean age of Björk modification was 6.2 ± 5.0 years.  Out of 67 who initially underwent the Björk modification, there were 2 early and 13 late deaths (22%).  A few others were lost to follow-up, and 41/52 survivors have been actively followed at the center.  Patients underwent cardiopulmonary exercise testing, with measurement of peak oxygen uptake (VO2).   Selected patients underwent cardiac catheterization and cardiac MRI if needed for clinical purposes.

Absence of pulsatile systolic pulmonary artery blood flow was defined as pulseless continuous systolic-diastolic flow, respiratory-dependent pulmonary flow, or flow with minimal acceleration in the end-diastolic phase (Figure 1, below).

Late atrial tachyarrhythmia occurred in 85% of the cohort.  Interestingly, despite the need for an RV incision with this procedure, no ventricular arrhythmia was observed.  Patients in Group P required fewer catheter ablation procedures for atrial arrhythmias than those in Group N (9% versus 50%, p = 0.03).  In the subset who underwent cardiac MRI, measurements of RV volumes were significantly larger in Group P than in Group N.  By MRI, there was no difference in LV volumes between groups.   Although preoperative and intraoperative records indicated significant hypoplasia of all RVs prior to surgery, at the last follow-up, 10/11 patients in Group P had reached normal (or greater than normal) RV size, whereas 20/30 in Group N still had a hypoplastic RV.  Of note, six patients in Group N had initially had pulsatility of pulmonary blood flow, but eventually lost it, whereas all patients in Group P had pulsatile blood flow on every echocardiogram during the study period.  An interesting finding was that 5/6 in Group N who initially had pulmonary pulsatility had developed normal RV size, even though they lost pulsatility.  Pulsatility was lost anywhere from 12 years to 32 years after Björk modification.

Exercise capacity was higher in Group P than in Group N.  Peak VO2 was 25.0 ± 7.3 versus 19.6 ± 6.0 mL/kg/min, respectively (p = 0.03), and VO2 at anaerobic threshold was 14.6 ± 4.7 versus 11.5 ± 3.6 ml/kg/min, respectively.

It should be noted that the echocardiograms used for inclusion in the study were the last echocardiogram performed in clinic for routine purposes, or the last echocardiogram prior to conversion to a total caval pulmonary connection (TCPC).  A variety of materials were used to form the RA-RV connection, including pericardium, Dacron, GoreTex, and homograft.

The authors concluded by developing a few hypotheses as to why patients in Group P had larger RVs and better exercise capacity.  Possible reasons presented were  1) pulsatile pulmonary blood flow may result in improved right ventricular compliance and thus promote a larger RV which can generate more stroke volume, 2) patients with pulsatile pulmonary blood flow might have better RV/LV interactions, and 3) pulsatile flow might increase more with exercise than nonpulsatile flow.  Unfortunately, no significant difference could be identified in the preoperative variables between Group P and Group N which could identify which patients would likely end up with pulsatile versus nonpulsatile pulmonary blood flow at late follow-up.  The authors did mention at the end of the paper that when combining Group P along with the 6 patients in Group N who initially had pulsatile flow, but lost it later, and comparing them to the other 24 patients in Group N, that there was a lower transpulmonary gradient, so perhaps a low PVR may have been the key difference that led to at least early pulsatility of pulmonary blood flow.

 achd 2.1

FIGURE – schematic of Bjork modification showing surgical RA-RV connection

achd 2.2

Fig 1. Parasternal short-axis echocardiographic view of the main pulmonary artery after Fontan-Björk procedure. (A) Pulsatile Doppler systolic flow in the main pulmonary artery of a patient in group P. (B) Nonpulsatile systolic diastolic flow in the main pulmonary artery of a patient in group N.



3. Clinical outcome following total cavopulmonary connection: a 20-year single-centre experience.

Ono M, Kasnar-Samprec J, Hager A, Cleuziou J, Burri M, Langenbach C, Callegari A, Strbad M, Vogt M, Hörer J, Schreiber C, Lange R.

Eur J Cardiothorac Surg. 2016 Mar 23. pii: ezw091. [Epub ahead of print]

PMID: 27009106

Similar articles

Select item 27009890

Take Home Points:

  1. TCPC in Munich had a 98.2% 30-day survival and 93.2% 15-year survival.
  2. In Munich, partial cavopulmonary connection was performed at 3 months, and TCPC at 18-24 months, with extracardiac conduits now being procedure of choice. Fenestrations are very rarely done at this center (only 5 patients have undergone primary fenestration since 2001).
  3. When compared with classic Fontan, TCPC has dramatically decreased the incidence of tachyarrhythmia, need for reoperation, and thromboembolic complications.
  4. In this study, patients with extracardiac Fontan had significantly higher exercise capacity than those with lateral tunnel Fontan (VO20% predicted versus 59.9% predicted, respectively). Reasons for this are unclear.
  5. Freedom from late tachyarrhythmia was 91% at 15 years post TCPC. This is similar to other studies of large TCPC cohorts.
  6. PLE and plastic bronchitis were extremely rare in this cohort of patients. The only multivariable predictor of PLE in this study was an elevated PA pressure.
  7. Thromboembolic complications were exceedingly rare in this study, but this center has an institutional policy that all patients are treated with vitamin K antagonists with a target INR of 2.0-3.0.

Commentary  from W. Aaron Kay (Indianapolis), section co-editor of ACHD Journal Watch:  This study from Munich, Germany sought to evaluate long-term outcomes after total cavopulmonary connection-type Fontan procedures in single ventricle patients.  Over a 21 year period, 434 patients underwent TCPC at their center (n=50 lateral tunnels and n=384 extracardiac conduits).  Clinical endpoints, exercise capacity, and liver evaluations were reviewed retrospectively.  At the Munich center, the lateral tunnel was first utilized in 1994, the extracardiac conduit was first introduced in 1999, and since 2002, the extracardiac conduit has been their procedure of choice.  Patients who initially had a classic Fontan and were converted to TCPC (n = 33) were excluded from analysis in this study.  Early mortality was rare (8 deaths < 30 days postop, 3 in lateral tunnel, 5 in extracardiac), with overall 30 day survival of 98.2%.  Of the 426 early survivors, 10 patients were lost to follow-up after hospital discharge.  Mean follow-up period was 6.6 ± 5.4 years in all patients in the study.

Median age at TCPC was 2.3 years, with a median weight of 12.0 kg.  Most patients had a partial cavopulmonary connection prior to Fontan (88.7%).  Only one patient underwent Fontan take-down over the study period.  In Munich, the TCPC is performed at an age of 18-24 months old, and uses an 18 mm conduit in the majority of cases.  In those with prior Kawashima operation, TCPC is performed at a median age of 36 months.  Fenestration was placed when clinically indicated per the operators, with a total of 35 (8%) fenestrated procedures (48% in lateral tunnel, 6% in extracardiac conduit, p < 0.001).

Overall Kaplan-Meier 15 year survival was 92.3%.

On multivariate analysis, predictors of prolonged hospital stay or other significant clinical endpoints included a high transpulmonic gradient, hypoplastic left heart syndrome, a higher number of pre-Fontan palliations, significant AV valve regurgitation, prior Norwood procedure, dextrocardia, high mean aortic pressure, and high pulmonary artery pressure.  Postoperative complications including long hospital stay, long duration of chest tube drainage, and prolonged ICU stay were strongly correlated with late mortality, reoperations, and interventions.   See Table 6 from paper for full details.

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4. Cause of death in adults with congenital heart disease – An analysis of the German National Register for Congenital Heart Defects.

Engelings CC, Helm PC, Abdul-Khaliq H, Asfour B, Bauer UM, Baumgartner H, Kececioglu D, Körten MA, Diller GP, Tutarel O.

Int J Cardiol. 2016 Mar 2;211:31-36. doi: 10.1016/j.ijcard.2016.02.133. [Epub ahead of print]

PMID: 26970963

Similar articles

Select item 26803381

Take Home Points:

  1. Community based registries can complement data obtained from tertiary care clinical studies. Although data is not always complete or verifiable, using a community based registry can avoid selection bias inherent in studies at large university centers.
  2. The majority of patients with CHD who die in adulthood die of CHD-related complications, with progressive CHF being the most common cause, followed by sudden cardiac death.
  3. There is an increasing complexity of CHD surviving to adulthood, manifested by an increasing number of patients with severe CHD dying in adulthood.
  4. Higher severity of CHD correlates with a higher likelihood of having a CHD-related death.
  5. Those that die of CHD-related deaths are still dying at fairly young ages in their 4th decade and there is significant room to improve this.

Commentary from Dr. W. Aaron Kay (Indianapolis), section co-editor of ACHD Journal Watch:  The German National Register for CHD is voluntary for patients or parents of infants diagnosed with CHD.  This study evaluated causes of death amongst the ACHD population from 2001-2015, and adds to valuable data from prior studies in Finland, Canada, and the United Kingdom.  Unlike most prior studies, this is a population-based study, rather than a study from a single tertiary care ACHD center, thus there should be less selection bias.

A total of 2596 patients were analyzed, of which 9.2% died over a median follow-up of 3.67 years.  Patients were divided into 5 groups based on prior surgical history, including 1) no surgery, 2) corrective surgery (e.g. tetralogy of Fallot complete repair), 3) palliative surgery (i.e. transposition with atrial switch), 4) Fontan procedure, and 5) transplant.  The patients were further categorized into complexities of CHD based on the Bethesda classification, and the mode of death was classified as CHD-related or non-CHD related.  CHD-related deaths included progressive heart failure, perioperative death, sudden death, infection (endocarditis), or complications after heart or lung transplantation (infection or acute rejection).

Cause of death was determined either through medical records or by contacting the personal family physician of the deceased.  The cause of death was CHD-related in 71% of the study patients, non-CHD related in 18%, and unknown in 10% of patients.  Non-CHD related deaths were the leading cause of death in patients with simple CHD such as septal defects and coarctation.  Sudden cardiac death was the leading cause of death in Eisenmenger Syndrome, Tetralogy of Fallot, and Marfan syndrome.  There were 5 sudden deaths in transposition of the great arteries (the study did not specify if these were atrial switch or arterial switch patients).

Due to significant increases in utilization of implantable cardioverter-defibrillator around 2008 in Europe, the study period was further subdivided from 2001-2008 and 2009-2015 to evaluate differences in mortality and comorbidities between those two time periods.

Not surprisingly, patients who died during the study were more likely to be NYHA class III or IV than patients who did not die.  Patients who died of CHD-related causes were more likely to have more complex CHD.  The mean age of death for the whole study period was 39.8 ± 17.8 years, but the mean age for CHD-related death was 34.5 ± 13.1 years versus 57.6 ± 20.5 years.

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Fig. 2. Causes of death compared between time period I (January 2001–December 2008) vs. II (January 2009–January 2015).

When comparing the early era (2001-2008) versus the later era (2009-2015), mean age of death was higher in the earlier time period 41.5 ± 18.9 years whereas in the more recent period mean age of death was lower at 38.9 ± 17.2 years.  This was likely driven by a lower number of children dying and thus dying as adults rather than as children, thus driving down the average age of death, bearing in mind that the vast majority of patients in the study are still alive.  Out of the 160 patients who died in the more recent time period, 63% had severe CHD, and 25% had moderate CHD, whereas out of the 79 patients who died in the earlier time period, 18% had simple CHD, 29% had moderate CHD, and 53% had severe CHD.

An alarming finding in this study is that perioperative mortality was the cause of death in 16.3% of patients who died, with the majority of this occurring with reoperations.

The percentage of patients dying of sudden cardiac death decreased from 29% in the earlier time period to 20% in the later time period; this may likely be due to an increased utilization of ICDs over the course of the study period.

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Fig. 3. Causes of death grouped for individual congenital heart defects. VSD: ventricular septal defect; UVH: univentricular heart; TOF: Tetralogy of Fallot; TGA: transposition of the great arteries’ Others: cor triatriatum sinistrum, mitral valve anomaly, pulmonary artery anomaly, double outlet right ventricle (transposition-type), truncus arteriosus communis, aortopulmonary window, Shone complex, abnormal origin of the coronary artery from the pulmonary artery, pulmonary stenosis, mitral valve prolapse; CoA: coarctation of the aorta; ccTGA: congenitally corrected TGA; AVSD: atrioventricular septal defect; ASD: atrial septal defect; AS: aortic stenosis.



5. Rehospitalization Is a Major Determinant of Inpatient Care Costs in Adult Congenital Heart Disease.

Cedars AM, Burns S, Novak EL, Amin AP.

J Am Coll Cardiol. 2016 Mar 15;67(10):1254-5. doi: 10.1016/j.jacc.2015.12.043. No abstract available.

PMID: 26965547

Similar articles

Select item 26993217

Take Home Points:

  1. Readmissions and surgical operations in ACHD are common and expensive
  2. If certain readmissions could be prevented, significant money could be saved in annual hospital charges for ACHD patients
  3. This issue requires further study, but studies are limited due to the quality of data in administrative databases.

Commentary from Dr. W. Aaron Kay (Indianapolis), section co-editor of ACHD Journal Watch: This study analyzed data from 6 states in the United States with statewide inpatient databases.  The individual states were selected because their databases allowed for longitudinal follow-up of the same patient over multiple admissions via unique identifiers.

Admissions for ACHD-related reasons were identified by screening ICD-9 codes with further correction to adjudicate diagnosis via the Broberg algorithm.  Patients were excluded if their index admission was in the first 12 months or last 12 months of the study period.

Data was analyzed to identify patients above and below median costs over a 12-month period.  This data was further analyzed against published data as well as the investigators’ clinical experience in order to determine potential variables that may correlate with inpatient care costs.  The primary outcome of the study was total financial burden over a 12-month period.

There were 155,297 index admissions with 619,720 readmissions.  A total of 112,113 readmissions occurred within 1 year of the index admission.  After adjudication of cases that were not truly ACHD admissions, and after excluding index admissions in the 1st or last 12 months, and after excluding admissions for which there was no cost data available, the final sample size was 68,314 admissions.

The average age of ACHD patients who were admitted to the hospital was 57.0 ± 18.6 years, with 51% women.  A large percentage (40%) of these admissions were for surgical operations.  Readmissions were expensive and accounted for an average of $23,993 over a 12 month period.  The cost was $16,822 greater for patients who required surgery.  Although most surgical procedures cannot be avoided, the authors proposed that greater efficiency that might prevent readmissions could save significant costs.

The study was limited due to its use of administrative data which is not verifiable for completeness and accuracy.  Patients who left the 6 states would have been lost to follow-up in this study, and death is not accounted for in these inpatient databases, thus that is a potential source for error.



6. Multimodality imaging in congenital heart disease-related pulmonary arterial hypertension.

D’Alto M, Dimopoulos K, Budts W, Diller GP, Di Salvo G, Dellegrottaglie S, Festa P, Scognamiglio G, Rea G, Ait Ali L, Li W, Gatzoulis MA. Heart. 2016 Mar 24. pii: heartjnl-2015-308903. doi: 10.1136/heartjnl-2015-308903. [Epub ahead of print] Review. PMID: 27013702 Similar articles Select item 27018378

Take Home Points:

  1. A simple chest x-ray may be very useful in the evaluation of PAH-CHD patients.
  2. The Moceri composite risk score may be a valuable tool for the assessment of PAH-CHD patients with echo.
  3. Right heart catheterization remains the gold standard for diagnosis and individual management of patients with PAH-CHD.

Moe_Tabitha-WEBCommentary from Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: A multimodal approach to the evaluation of complex congenital heart disease and comorbidities is becoming the gold standard of adult congenital heart disease management in the digital age. These European authors describe their approach specifically to evaluating pulmonary arterial hypertension in patients with congenital heart disease. This article discusses the use of standard chest radiography, echo, Cardiac CT, and Cardiac MRI and their risks, benefits, and applications. CXR is simple, inexpensive, and has low levels of radiation. The information of the cardiothoracic ratio is reproducible, and has diagnostic and prognostic implications. Changes in cardiac size can be followed longitudinally as a response to therapy in locations where access to more advanced imaging may be limited. Echo is valuable in assessing prognosis, and response to therapy. Moceri et al suggest that a composite risk score based on TAPSE <15 mm, RV effective systolic to diastolic duration ratio >1.5, right atrial area >2.5cm2, and right atrial to left atrial area ratio >1.5 is highly predictive of clinical outcome. CMR allows for monitoring of structural and functional changes associated with CHD-PAH, primarily affecting the RV and the pulmonary circulation.  CMR is a low risk and radiation-free diagnostic tool, but is limited by availability to tertiary and quaternary care centers, as well as limited by adequately trained technicians, and faculty. There are some limitations related to artifact of implanted pacemaking systems in the pulmonary system which limits interpretability of flows. Cardiac CT can be used when CMR is not an option although it requires increased radiation exposure as well as iodinated contrast exposure. However, CT is considered the modality of choice for assessment of the great vessels including proximal and distal pulmonary artery anatomy, and presence of thrombus.  Cardiac catheterization remains the gold standard for diagnosis and management of PAH-CHD as no other modality is able to completely assess step-wise pressures and outputs, as well as allow for initiation of therapy during the procedure.

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7. Posterior Circulation Ischemia or Occlusion in Five Adults with Failing Fontan Circulation.

Broomall E, McBride ME, Deal BJ, Ducharme-Crevier L, Shaw A, Mazwi M, Backer CL, Mongé MC, Costello J, Marino BS, DeFreitas A, Wainwright MS. Ann Thorac Surg. 2016 Mar 23. pii: S0003-4975(16)00002-3. doi: 10.1016/j.athoracsur.2016.01.001. [Epub ahead of print]

PMID: 27016841 Similar articles Select item 27005935

Take Home Points:

  1. Vertebrobasilar stroke carries poor outcomes in Fontan patients undergoing revision or heart transplantation.
  2. Pre-operative identification of altered posterior cerebral circulation allows for customizing the management of perioperative (Fontan revision/transplant) risk.

Commentary from Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch:: This article highlights the need for multidisciplinary care and evaluation of Fontan patients prior to undergoing Fontan revision, or heart transplant. Cerebrovascular anatomy may be altered with the use, occlusion, or ligation of subclavian arteries in staged pre-Fontan palliation. This changes flow to vertebral arteries and may compromise flow in patients with functionally univentricular hearts. Although this is a small series of patients, the Chicago group made a fundamental shift in their evaluation and care of patients that would lend itself to wider study, and should at least suggest the possibility that further investigation by neuroradiology, and neurocritical care is recommended prior to undergoing heart transplant or Fontan revision.  The strategic changes included higher perioperative blood pressures, advanced neuroimaging, and individual tailoring of therapy. Cerebral perfusion was maintained at >50 mmHg in the setting of normal ICP. This small case series documents abnormal posterior circulation anatomy.  The demonstration of atrophic vertebral arteries allows for customization of peri-operative management strategy. Posterior circulation stroke has very poor outcomes. Posterior circulation compromise has not previously been identified as a long-term risk in univentricular heart palliation or in Fontan failure.



9. Bodily isomerism is an independent risk factor for pulmonary hypertension in adults with congenital heart disease.

Loomba RS, Aggarwal S, Arora RR, Anderson R. Ther Adv Respir Dis. 2016 Mar 4. pii: 1753465816636810. [Epub ahead of print] PMID: 26944361 Similar articles Select item 27032711

Take Home Points:

  1. Isomerism poses a 79% increase over baseline for risk of pulmonary arterial hypertension.
  2. Fetal counseling as well as routine education and life-long screening regarding development of PAH-CHD is recommended.
  3. The risk of PAH-CHD with isomerism is still only 5.6%.

Commentary from Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: Heterotaxy and isomerism have multiple associated comorbidities that are not associated with the cardiovascular system. This article highlights a long-term comorbidity that may be overlooked in the clinical follow up and evaluation of these already complex adult congenital patients. Patients with heterotaxy spectrum may have a two-ventricle repair with arterio-ventricular concordance, or they may have a single, morphologic right ventricle with interrupted IVC, and hemiazygous continuation, or anything in between. This article highlights a group of patients who were extracted from the Nationwide Inpatient Sample with 861 patients with isomerism, and 5.6% of those patients met criteria for PAH-CHD vs. only 2.6% without.  The predictive risk factors in this population are increasing age, increasing BMI, and history of anomalous pulmonary venous connection. The authors suggest that patients with isomerism should be counseled regarding PAH-CHD at the time of diagnosis, and should be included in prenatal counseling where applicable.



10. BMPR2 mutation is a potential predisposing genetic risk factor for congenital heart disease associated pulmonary vascular disease.

Liu D, Liu QQ, Guan LH, Jiang X, Zhou DX, Beghetti M, Qu JM, Jing ZC. Int J Cardiol. 2016 Mar 7;211:132-136. doi: 10.1016/j.ijcard.2016.02.150. [Epub ahead of print] PMID: 27002414 Similar articles Select item 26956707

Take Home Points:

  1. BMPR-2 missense mutations are common (9.9%) in female patients with repaired congenital heart disease.
  2. BMPR-2 mutations are strongly associated with progressive pulmonary arterial hypertension.
  3. BMPR2 mutations should be sought in CHD patients who develop PAH.

Commentary from Dr. Tabitha Moe (Phoenix) section editor of Congenital Heart Journal Watch: This study evaluated 294 patients with PAH-CHD in China. The patients were diagnosed with PAH-CHD after an initial screening echocardiogram was suggestive of PAH-CHD. The PVR was required to be >3 Woods Units. BMPR2 mutation carries a well-known correlation with familial pulmonary arterial hypertension. It is most commonly found in female carriers, and is typically associated with identification of iPAH in juveniles.  A significantly higher BMPR2 mutation rate was found in repaired CHD.  Missense mutation of BMPR2 is the dominant mutation type.  Female repaired CHD patients are most likely to have the BMPR2 mutation detected. In patients with repaired PAH-CHD, 7.5% were BMPR2 mutation positive compared to 1.2% of patients without PAH. In female patients the mutation rate reaches 9.9%, this approaches the mutation rate in Chinese iPAH. Genetic predisposing factors may be important elements in the process of development of PAH in CHD patients. Female, repaired patients are more likely to be detected with genetic mutations.

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CHD Electrophysiology Featured Articles of March 2016

1. Electrocardiographic repolarization abnormalities and increased risk of life-threatening arrhythmias in children with dilated cardiomyopathy.

Chen S, Motonaga KS, Hollander SA, Almond CS, Rosenthal DN, Kaufman BD, May LJ, Avasarala K, Dao DT, Dubin AM, Ceresnak SR.

Heart Rhythm. 2016 Mar 2. pii: S1547-5271(16)00184-3. doi: 10.1016/j.hrthm.2016.02.014. [Epub ahead of print]

PMID: 26945851

Similar articles

Select item 26970963

Take Home Points:

  • Repolarization abnormalities, specifically JTc prolongation and ST segment shifts may be associated with increased risk for LTEs in pediatric cardiomyopathy patients.
  • These findings should be repeated in a larger pediatric population, as the present findings could not be adjusted for other covariates due to the small number of outcomes.
  • Risk stratification for sudden cardiac death in pediatric dilated cardiomyopathy patients remains ill-defined.

J MooreCommentary from Dr. Jeremy Moore (Los Angeles, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This is a single-center retrospective review of 178 pediatric patients with dilated cardiomyopathy (defined in this study as LVEF <55% and LVEDD z score ≥2) from Stanford University. Given the known risk of electrocardiographic depolarization abnormalities in this population, the authors were interested in examining the independent contribution related to repolarization abnormalities. In order to exclude influence from co-existing QRS prolongation, the corrected JT interval (JTc) was manually calculated (predominantly from lead II) by 3 cardiologists at the study site.

From this cohort of dilated cardiomyopathy patients, the upper 90th percentile for the corrected QT interval (QTc) was 510 ms and for the JTc was 390 ms. A total of 15 life-threatening events (LTE – defined as an episode of ventricular fibrillation or ventricular tachycardia that resulted in syncope or hypotension) occurred during a median follow-up duration of 12 months. The JTc was significantly longer in patients with LTEs versus unaffected dilated cardiomyopathy patients (371 ± 77 ms vs 342 ± 44 ms, p=0.01). Other variables associated with LTE in a Cox proportional hazards model included QRS duration >120 ms, JTc >390 ms, QTc >510 ms, JT dispersion ≥100 ms, QT dispersion >100 ms, T wave inversion, and ST segment depression. No adjustment for multiple comparisons was performed, and multivariable regression was limited to 2 covariates in this small sample population with few clinical outcomes.



2. Mechanism and outcomes of catheter ablation for ventricular tachycardia in adults with repaired congenital heart disease.

van Zyl M, Kapa S, Padmanabhan D, Chen FC, Mulpuru SK, Packer DL, Munger TM, Asirvatham SJ, McLeod CJ.

Heart Rhythm. 2016 Mar 4. pii: S1547-5271(16)30003-0. doi: 10.1016/j.hrthm.2016.03.002. [Epub ahead of print]

PMID: 26961296

Similar articles

Select item 26944361

Take Home Points:

  • Catheter ablation of isthmus-dependent VT with subsequent verification of conduction block across the targeted isthmus appears to be highly effective for prevention of recurrent VT in patients with CHD.
  • As many as one-third of patients may have focal VT rather than an isthmus-dependent substrate.
  • A more exhaustive approach to determine the VT mechanism (rather than a substrate-based approach) may be useful to improve procedural outcomes.

Commentary from Dr. Jeremy Moore (Los Angeles, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This was a retrospective study of 21 patients with repaired congenital heart disease undergoing catheter ablation for clinically-documented ventricular tachycardia (VT) at the Mayo Clinic over a 10-year period spanning 2004 and 2015. The electrophysiologic approach was based on a combination of substrate mapping and entrainment at the site of the suspected critical isthmus, in order to determine the optimal site for catheter ablation. Whenever possible, conduction block was confirmed after lesion placement.

Congenital diagnoses included tetralogy of Fallot in 10, transposition of the great arteries after atrial switch operation in 4, isolated ventricular septal defect in 2, Ebstein anomaly in 2, and 1 each with pulmonary atresia, truncus arteriosus, and congenital aortic valve stenosis. Seven patients (33%) were found to have a focal VT, and 14 (67%) were found to have a conduction isthmus believed to be responsible for the clinical VT. Conduction block was achieved in 8/14 isthmuses (57%). Complete procedural success was achieved in 17 patients (81%). Over a mean follow up of 33 ± 7 months, 18/21 patients were free from VT recurrence. No patient with confirmed conduction block at the targeted isthmus experienced a recurrence.


3. Gene-Specific Therapy With Mexiletine Reduces Arrhythmic Events in Patients With Long QT Syndrome Type 3.

Mazzanti A, Maragna R, Faragli A, Monteforte N, Bloise R, Memmi M, Novelli V, Baiardi P, Bagnardi V, Etheridge SP, Napolitano C, Priori SG.

J Am Coll Cardiol. 2016 Mar 8;67(9):1053-8. doi: 10.1016/j.jacc.2015.12.033.

PMID: 26940925 Free PMC Article

Similar articles

Select item 26950378

Take Home Points:

  • Mexilitine may be an effective therapeutic agent for reduction of cardiac events in LQT3.
  • The clinical efficacy of Mexilitine response may be best gauged by a resultant QTc< 500 ms after initiation of therapy.
  • Further prospective evaluation is warranted.

Commentary from Dr. Jeremy Moore (Los Angeles, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This is a retrospective study from Italy evaluating the clinical efficacy of Mexilitine in the treatment of long QT syndrome (LQTS) type 3. Although LQTS type 3 is the least prevalent of the major forms of LQTS, clinical events in these patients tend to be the most malignant.

The authors report a total of 34 patients (38% symptomatic and 7/34 with ≥1 cardiac arrest) that were given Mexilitine at an average dose of 8 mg/kg/day. The QTc shortened by a mean of 63 ± 6 ms; and for patients with a QTc >500 ms at baseline, 73% decreased to below this threshold after initiation of therapy. Three infants continued to have significantly prolonged QTc values after initiation of Mexilitine (see figure below) and continued to have clinical cardiac events.

QTc response to Mexilitine therapy. *Patients with ongoing clinical events.

Risk exposure assessment based on the comparison of periods of equal duration (median 35 months) before and after Mexilitine administration was performed in order for the patients to serve as their own controls. There was a significant reduction in the percentage of patients with arrhythmic events (22% to 3%) and mean number of events per patient (0.43 to 0.03) using this methodology.

The authors concluded that not only does Mexilitine shorten the QTc as shown previously, but also results in a clinically meaningful reduction in cardiac events. The only “nonresponders” were those with QTc values that were persistently >500 ms on therapy.

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CHD Surgery Featured Articles of March 2016

1. Mechanical stress is associated with right ventricular response to pulmonary valve replacement in patients with repaired tetralogy of Fallot.

Tang D, Yang C, Del Nido PJ, Zuo H, Rathod RH, Huang X, Gooty V, Tang A, Billiar KL, Wu Z, Geva T.

J Thorac Cardiovasc Surg. 2016 Mar;151(3):687-694.e3. doi: 10.1016/j.jtcvs.2015.09.106. Epub 2015 Oct 3.

PMID: 26548998

Similar articles

Select item 26515874

Take Home Points

  1. The novel method combining CMR and computational ventricular modeling sheds light on predictors of right ventricular response to PVR among post TOF repair population.
  2. Pre-PVR right ventricular stress is the most important single factor to predict right ventricular response.
  3. Patients with high right ventricular stress appears to have poor right ventricular response to PVR.

Dr. Honjo 9-2015-2Comment from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch: This is a sophisticated proof-of-concept study to delineate the MRI-derived predictors for right ventricular positive vs. negative response to pulmonary valve replacement (PVR) among post tetralogy repair patients. This study is quite important in the sense that we all see both positive and negative responses of the right ventricular performance after PVR but we really don’t’ have any clue to predict who is going to respond to PVR. This study extracted data from 16 patients’ cardiac magnetic resonance (CMR) images and created computational ventricular modeling. Among the parameters examined, including right ventricular stress, strain, wall thickness, curvature, and volume, right ventricular wall stress showed the largest difference between responder and non-responder to PVR. Pre-PVR right ventricular stress is the best single predictor of right ventricular response, and combination of wall thickness, curvature, volume, and strain is the best combined predictor with the an area under the ROC curve of 0.848. This method can be effectively used to predict who is going to respond to PVR, but the real potential value is to use this method to determine the timing of PVR before the right ventricle becomes unresponsive to surgical intervention.



2. Aggressive Patch Augmentation May Reduce Growth Potential of Hypoplastic Branch Pulmonary Arteries After Tetralogy of Fallot Repair.

Wilder TJ, Van Arsdell GS, Pham-Hung E, Gritti M, Hussain S, Caldarone CA, Redington A, Hickey EJ.

Ann Thorac Surg. 2016 Mar;101(3):996-1004. doi: 10.1016/j.athoracsur.2015.11.040. Epub 2016 Jan 29.

PMID: 26830224

Similar articles

Select item 26680312

Take Home Points.

  1. Extensive patching for hypoplastic branch PAs in primary tetralogy repair brings improved short-term geometry, but is associated with higher re-stenosis and re-intervention rates.
  2. The majority of hypoplastic ‘untreated’ branch PAs have catch-up growth after repair
  3. Conservative approach to those branch PAs may be an option as long as right ventricular pressure is acceptable.

Comment from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch: Primary repair for tetralogy of Fallot often faces the challenge of hypoplastic branch pulmonary arteries (PAs), which may be a cause of postoperative right ventricular hypertension after repair. Wilder and the Toronto group analyzed the effect of limited vs. extensive patch reconstruction of such hypoplastic branch PAs regarding subsequent growth potential after reconstruction. This is a well-designed retrospective study including 434 patients from The Hospital for Sick Children. The study showed that extensive PA patching improves short-term PA geometry but may lead to late stenosis and increase in re-intervention rate. The vast majority (up to 85%) of hypoplastic PAs that were not intervened at the time of repair had adequate catch-up growth. The message from the investigators is that a conservative approach may not be a bad option as long as right ventricular pressure is acceptable. This study helps us guiding our intraoperative decision making for patients with borderline branch PAs. One personal comment is that many of extensive patching were necessary at the time of surgery to relieve hemodynamically significant obstruction. I suspect that branch PA tissue in such hypoplastic PAs can be intrinsically different from other moderately hypoplastic PAs with no important hemodynamic obstruction.


3. Postoperative Outcomes of Children With Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals Undergoing Reconstruction of Occluded Pulmonary Artery Branches.

Asija R, Koth AM, Velasquez N, Chan FP, Perry SB, Hanley FL, McElhinney D.

Ann Thorac Surg. 2016 Mar 3. pii: S0003-4975(15)02047-0. doi: 10.1016/j.athoracsur.2015.12.049. [Epub ahead of print]

PMID: 26947013

Similar articles

Select item 26941015

Take Home Points

  1. With an experienced surgical program, occluded MAPCAs or pulmonary arteries are rehabilitatable by unifocalization.
  2. With unifocalization, more than three quarters of the previously-occluded MAPCAs remain patent.
  3. Despite occluded MAPCAs, approximately 70% of the patients achieved biventricular repair with adequate unifocalization.

Comment from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch: Asiji and colleagues from Stanford group report on one of the most difficult subgroups in the patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs). This group is by far the most experienced group in the world to treat this lesion and this study specifically looked at the patients who had completely occluded MAPCAs. Seventeen (18%) out of 92 patients who underwent a unifocalization procedure during the study period had occluded pulmonary artery branches. Postoperative follow-up angiograms showed 82% of the previously-occluded MAPCAs were patent. Overall 70% of the patients successfully underwent biventricular repair with reasonable right ventricular pressure. This is a remarkable work and outcome given how challenging the patient group is.


4. Fontan Conversion: Literature Review and Lessons Learned Over 20 Years.

Mavroudis C, Deal BJ.

World J Pediatr Congenit Heart Surg. 2016 Mar;7(2):192-8. doi: 10.1177/2150135115623960.

PMID: 26957403

Similar articles

Select item 26957402

Take Home Points

  1. This is an excellent overview of the evolution and current status of Fontan conversion.
  2. The review emphasize the importance of arrhythmia surgery as well as pacemaker therapy in combination with Fontan conversion.

Comment from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch: This excellent review article is written by the pioneers of the field of Fontan conversion. The article encompasses histological aspects and the evolution of Fontan conversion and concomitant arrhythmia surgery. They summarized the lessons learned over last 20 years of their experience. In the review, they touched on their recent study of 140 Fontan conversions. Freedom from death or transplant at 10 years is 84% and freedom from recurrent atrial tachycardia at 10 year was 77%.


5. Pulmonary vein stenosis: Severity and location predict survival after surgical repair.

Lo Rito M, Gazzaz T, Wilder TJ, Vanderlaan RD, Van Arsdell GS, Honjo O, Yoo SJ, Caldarone CA.

J Thorac Cardiovasc Surg. 2016 Mar;151(3):657-666.e2. doi: 10.1016/j.jtcvs.2015.08.121. Epub 2015 Sep 16.

PMID: 26481279

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Select item 26803197

Take Home Points

  1. Survival after sutureless repair for primary pulmonary vein stenosis can be predicted by pre-repair pulmonary vein cross-sectional areas and the number of pulmonary veins involved.
  2. Smaller upstream pulmonary vein cross-sectional area and the number of involved pulmonary veins are associated with early death, and smaller downstream pulmonary vein cross-sectional area is related to late death.

Comment from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch: Primary pulmonary vein stenosis is often progressive and refractory to surgical intervention. Lo Rito and colleagues from The Hospital for Sick Children reported anatomic risk factors for poor outcome after sutureless repair for primary pulmonary vein stenosis. The study analyzed 31 out of 145 patients who underwent pulmonary vein repair and had pre-repair cross-sectional imaging such as CT or MRI. The authors measured cross-sectional area of the pulmonary veins at two different points, upstream vein—before bifurcation and usually distal to the pulmonary vein stenosis, and downstream vein—at the entrance to the left atrium. The authors found that smaller upstream total cross-sectional area indexed for body surface area and greater number of stenotic pulmonary veins were associated with increased early (< 1 year) risk of death. They also found that smaller downstream total cross-sectional area indexed for body surface area tended to be associated with a late risk of death. This emphasizes the importance of early diagnosis and surgery for this lesion before stenosis becomes severe and upstream and downstream veins get small.


6. More than valve repair: Effect of cone reconstruction on right ventricular geometry and function in patients with Ebstein anomaly.

Li X, Wang SM, Schreiber C, Cheng W, Lin K, Sun JY, Yang D, Luo SH, An Q, Chen YC.

Int J Cardiol. 2016 Mar 1;206:131-7. doi: 10.1016/j.ijcard.2016.01.032. Epub 2016 Jan 7.

PMID: 26788689

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Select item 26773763

Take Home Points

  1. Successful core repair for Ebstein’s anomaly not only decreases RV volume but also improves RV global synchronization.
  2. A new index, TAMSI, is a useful index to assess RV global synchronization at the annular level.

Comment from Dr. Osami Honjo (Toronto), section editor of Congenital Heart Surgery Journal Watch: Li and colleagues from China and Germany reported an interesting study regarding right ventricular (RV) synchronization and geometry following cone repair for Ebstein’s anomaly. Using echocardiography and MRI, they developed an index called tricuspid annular movement synchronicity index (TAMSI), which is essentially a parameter of RV global synchronization at the annular plane. They examined this index in 18 patients with Ebstein’s anomaly before and after cone repair, and data was compared to 27 healthy control subjects. Results showed reduced RV volume and unchanged RV ejection fraction after the cone repair. TAMSI decreased significantly after cone repair, and became comparable to normal subjects, which indicated improved RV global synchronization.

( average: 4.00 )