CHD Surgery Featured Articles of April 2017

Congenital Heart Surgery Reviews of April 2017 Manuscripts


The effects of postoperative hematocrit on shunt occlusion for neonates undergoing single ventricle palliation.

Anderson BR, Blancha VL, Duchon JM, Chai PJ, Kalfa D, Bacha EA, Krishnamurthy G, Ratner V.

J Thorac Cardiovasc Surg. 2017 Apr;153(4):947-955. doi: 10.1016/j.jtcvs.2016.09.085. Epub 2016 Nov 14.

PMID: 27938905

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Take Home Points:

  • In patients undergoing placement of a systemic-to-pulmonary artery shunt, increased hematocrit levels may be associated with early shunt occlusion but not mortality.
  • The optimal anticoagulation strategy following shunt procedures (e.g., aspirin and/or systemic heparin) remains undetermined.


jeremy-herrmannCommentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  Defining an optimal range of hematocrit values following cardiac surgery in pediatric patients remains a challenge, particularly for cyanotic patients undergoing placement of a systemic-to-pulmonary artery shunt. The Columbia group reports results of a retrospective analysis of their experience with these shunts from 2010-2015. Eighty neonates were included: 28 with Norwood with modified BT shunt; 39 with modified BT shunt; and 12 with a central shunt. The first postoperative hematocrit values were analyzed in comparison with shunt occlusion and 30-day mortality. Their standard postoperative anticoagulation regimen included aspirin but no systemic heparin. There were 12 instances of shunt occlusion, 5 within the first 24 hours (“early” shunt occlusions). Notably, none of these patients who developed early shunt occlusion died during the study period. There were 6 deaths in the series, 2 of which occurred after late shunt occlusion on postoperative days 12 and 18. On univariable analysis, the two factors associated with increased risk of early shunt occlusion were the first postoperative hematocrit level and later operative era (after July 1, 2014). Interestingly, the first postoperative hematocrit levels were higher in the later operative era, and for each additional 5 additional percentage points of hematocrit value, the risk of early shunt occlusion more than doubled (odds ratio [OR], 2.70; p=0.009). In terms of mortality, increased inotrope score and first postoperative PaO2 were associated with increased mortality in univariable analysis. Increased postoperative hematocrit was also associated with increased inotrope use and higher postoperative PRBC transfusion volumes were associated with increased risk of mortality, but no association between the first postoperative hematocrit and mortality was seen. The authors conclude that lower postoperative hematocrit values may be better for patients undergoing systemic-to-pulmonary artery shunt procedures, though the ideal range remains undetermined. They describe their practice of not transfusing PRBC for levels above 55%. With these results and those from other previous studies that do not strongly support or refute the routine use of systemic heparinization following shunt procedures, the authors continue to routinely use only aspirin in the postoperative period. The results of the present study are compelling regarding the avoidance of high hematocrit levels in the early postoperative period after shunt procedures. However, the question of how best to manage anticoagulation in these patients remains unanswered and may be beyond what a single institution, retrospective study can determine.




  1. Peritoneal Dialysis vs Furosemide for Prevention of Fluid Overload in Infants After Cardiac Surgery: A Randomized Clinical Trial.

Kwiatkowski DM, Goldstein SL, Cooper DS, Nelson DP, Morales DL, Krawczeski CD.

JAMA Pediatr. 2017 Apr 1;171(4):357-364. doi: 10.1001/jamapediatrics.2016.4538.

PMID: 28241247

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Take Home Points:

  • Peritoneal dialysis for postoperative oliguria in infants undergoing cardiac surgery is safe and may offer improved fluid balances, inotropic requirements, electrolyte levels, and less prolonged duration of mechanical ventilation compared to intravenous furosemide therapy.


Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  Fluid management of neonates following cardiac surgery can be challenging, and fluid overload can be difficult to avoid. In this study, the Cincinnati group conducted a prospective, randomized study comparing the use of intravenous furosemide or peritoneal dialysis (PD) for neonates with postoperative oliguria (urine output <1 ml/kg/hr over a 4-hour period in the first 24 hours). PD catheter placement was planned preoperatively for high-risk patients (age <3 mo undergoing cardiopulmonary bypass, age <4 mo undergoing tetralogy of Fallot or double outlet right ventricle repair, or age <6 mo undergoing heart transplantation) and selectively for other moderate-risk patients. The surgeon was blinded to initial group assignment. Patients were excluded if they had preexisting kidney disease, did not undergo cardiopulmonary bypass during surgery, did not have a PD catheter placed as planned, died during the study period, or required ECMO support or reoperation. Seventy-three patients were included, 32 patients in the furosemide group and 41 in the PD group. The authors observed no difference in the proportion of patients with a negative fluid balance on by the first postoperative day between groups, the but patients in furosemide group were more likely to develop a 10% fluid overload (odds ratio [OR] 3.0, 95% CI 1.3 to 6.9) and require prolonged intubation of more than 72 hours (OR 3.1, 95% CI 1.2 to 8.2). Patients in the PD group demonstrated better fluid balances on postoperative days 1-3, required less inotropic support, and exhibited fewer electrolyte abnormalities compared to the furosemide group. There were 2 adverse events related to PD catheters involving bloody drainage in one patient and a hydrocele in the other, neither of which required intervention. Pleuro-peritoneal communications were identified in 9 patients in the PD group based on drainage of dialysate from the chest tubes, and these patients did not undergo PD as assigned. No difference in mortality was observed.


The authors conclude that PD is safe and offers better clinical performance compared to furosemide therapy in oliguric, postoperative infants based on more favorable inotrope use, electrolyte levels, rate of prolonged intubation, and avoidance of fluid overload. However, they also conclude that their study was underpowered to evaluate other clinical outcomes parameters (e.g., length of ICU stay). Given the prospective, randomized nature of this study, these findings are worthy of consideration. Of further interest would be a cost comparison study of PD and furosemide treatment pathways and related clinical outcomes.

CHD Surgery April 2017


  1. Resource Utilization Associated with Extracardiac Co-morbid Conditions Following Congenital Heart Surgery in Infancy.

Tuomela KE, Gordon JB, Cassidy LD, Johaningsmeir S, Ghanayem NS.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1620-y. [Epub ahead of print]

PMID: 28456828

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  1. Lung Transplantation for FLNA-Associated Progressive Lung Disease.

Burrage LC, Guillerman RP, Das S, Singh S, Schady DA, Morris SA, Walkiewicz M, Schecter MG, Heinle JS, Lotze TE, Lalani SR, Mallory GB.

J Pediatr. 2017 Apr 28. pii: S0022-3476(17)30457-2. doi: 10.1016/j.jpeds.2017.03.045. [Epub ahead of print]

PMID: 28457522

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  1. Mechanical Concepts Applied in Congenital Heart Disease and Cardiac Surgery.

Gerrah R, Haller SJ, George I.

Ann Thorac Surg. 2017 Apr 27. pii: S0003-4975(17)30178-9. doi: 10.1016/j.athoracsur.2017.01.068. [Epub ahead of print] Review.

PMID: 28457478

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  1. Outcomes of Totally Endoscopic Atrial Septal Defect Closure Using a Glutaraldehyde-Treated Autologous Pericardial Patch.

Nishida H, Nakatsuka D, Kawano Y, Hiraiwa N, Takanashi S, Tabata M.

Circ J. 2017 Apr 25;81(5):689-693. doi: 10.1253/circj.CJ-16-0888. Epub 2017 Feb 8.

PMID: 28179595 Free Article

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  1. Hospital costs and cost implications of co-morbid conditions for patients with single ventricle in the period through to Fontan completion.

Huang L, Dalziel KM, Schilling C, Celermajer DS, McNeil JJ, Winlaw D, Gentles T, Radford DJ, Cheung M, Bullock A, Wheaton GR, Justo RN, Selbie LA, Forsdick V, Du Plessis K, d’Udekem Y.

Int J Cardiol. 2017 Apr 20. pii: S0167-5273(17)30509-0. doi: 10.1016/j.ijcard.2017.04.056. [Epub ahead of print]

PMID: 28456482

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  1. Corrigendum to: “Glial fibrillary acidic protein plasma levels are correlated with degree of hypothermia during cardiopulmonary bypass in congenital heart disease surgery” [Interact CardioVasc Thorac Surg 2017]†.

Vedovelli L, Padalino M, D’Aronco S, Stellin G, Ori C, Carnielli VP, Simonato M, Cogo P.

Interact Cardiovasc Thorac Surg. 2017 Apr 17. doi: 10.1093/icvts/ivx072. [Epub ahead of print] No abstract available.

PMID: 28431165

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  1. New Regenerative Vascular Grafts for Hemodialysis Access: Evaluation of a Preclinical Animal Model.

Valencia Rivero KT, Jaramillo Escobar J, Galvis Forero SD, Miranda Saldaña MC, López Panqueva RDP, Sandoval Reyes NF, Briceño Triana JC.

J Invest Surg. 2017 Apr 17:1-9. doi: 10.1080/08941939.2017.1303100. [Epub ahead of print]

PMID: 28414565

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  1. The provision of pediatric cardiac anesthesia services in Germany: current status of structural and personnel organization.

Baehner T, Dewald O, Heinze I, Mueller M, Schindler E, Schirmer U, Baumgarten G, Hoeft A, Ellerkmann RK.

Paediatr Anaesth. 2017 Apr 17. doi: 10.1111/pan.13153. [Epub ahead of print]

PMID: 28419616

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  1. Non-invasive assessment of cerebral oxygen metabolism following surgery of congenital heart disease.

Neunhoeffer F, Sandner K, Wiest M, Haller C, Renk H, Kumpf M, Schlensak C, Hofbeck M.

Interact Cardiovasc Thorac Surg. 2017 Apr 16. doi: 10.1093/icvts/ivx080. [Epub ahead of print]

PMID: 28419229

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  1. Meta-Analysis of the Effectiveness of Heart Transplantation in Patients With a Failing Fontan.

Tabarsi N, Guan M, Simmonds J, Toma M, Kiess M, Tsang V, Ruygrok P, Konstantinov I, Shi W, Grewal J.

Am J Cardiol. 2017 Apr 15;119(8):1269-1274. doi: 10.1016/j.amjcard.2017.01.001. Epub 2017 Jan 25.

PMID: 28233535

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  1. Impact of International Quality Improvement Collaborative on Congenital Heart Surgery in Pakistan.

Khan A, Abdullah A, Ahmad H, Rizvi A, Batool S, Jenkins KJ, Gauvreau K, Amanullah M, Haq A, Aslam N, Minai F, Hasan B.

Heart. 2017 Apr 13. pii: heartjnl-2016-310533. doi: 10.1136/heartjnl-2016-310533. [Epub ahead of print]

PMID: 28408415

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  1. Cardiac surgery in patients with congenital heart disease is associated with acute kidney injury and the risk of chronic kidney disease.

Madsen NL, Goldstein SL, Frøslev T, Christiansen CF, Olsen M.

Kidney Int. 2017 Apr 12. pii: S0085-2538(17)30155-2. doi: 10.1016/j.kint.2017.02.021. [Epub ahead of print]

PMID: 28412020

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  1. Immunodepression after CPB: Cytokine dynamics and clinics after pediatric cardiac surgery – A prospective trial.

Justus G, Walker C, Rosenthal LM, Berger F, Miera O, Schmitt KRL.

Cytokine. 2017 Apr 11. pii: S1043-4666(17)30085-6. doi: 10.1016/j.cyto.2017.03.017. [Epub ahead of print]

PMID: 28411047

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  1. Early postoperative physical therapy for improving short-term gross motor outcome in infants with cyanotic and acyanotic congenital heart disease.

Haseba S, Sakakima H, Nakao S, Ohira M, Yanagi S, Imoto Y, Yoshida A, Shimodozono M.

Disabil Rehabil. 2017 Apr 7:1-8. doi: 10.1080/09638288.2017.1309582. [Epub ahead of print]

PMID: 28385091

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  1. Tetralogy of Fallot Surgical Repair: Shunt Configurations, Ductus Arteriosus and the Circle of Willis.

Piskin S, Unal G, Arnaz A, Sarioglu T, Pekkan K.

Cardiovasc Eng Technol. 2017 Apr 5. doi: 10.1007/s13239-017-0302-5. [Epub ahead of print]

PMID: 28382440

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  1. Anesthetic Management of a Tracheoesophageal Fistula in a Patient With a Large Uncorrected Aortopulmonary Window: A Case Report.

Adler AC, Steven JM, Jolley MA.

A A Case Rep. 2017 Apr 1;8(7):172-174. doi: 10.1213/XAA.0000000000000460.

PMID: 27941485

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  1. Transient cortical blindness following intracardiac repair of congenital heart disease in an 11-year-old boy.

Bharati S, Sharma MK, Chattopadhay A, Das D.

Ann Card Anaesth. 2017 Apr-Jun;20(2):256-258. doi: 10.4103/aca.ACA_159_16.

PMID: 28393793 Free Article

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  1. Modifying risks to improve outcome in cardiac surgery: An anesthesiologist’s perspective.

Chakravarthy M.

Ann Card Anaesth. 2017 Apr-Jun;20(2):226-233. doi: 10.4103/aca.ACA_20_17. Review.

PMID: 28393785 Free Article

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  1. Coagulopathies in cyanotic cardiac patients: An analysis with three point – of – care testing devices (Thromboelastography, rotational thromboelastometry, and sonoclot analyzer).

Bhardwaj V, Malhotra P, Hasija S, Chowdury UK, Pangasa N.

Ann Card Anaesth. 2017 Apr-Jun;20(2):212-218. doi: 10.4103/aca.ACA_4_17.

PMID: 28393783 Free Article

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  1. Tracheostomy Among Infants With Hypoplastic Left Heart Syndrome Undergoing Cardiac Operations: A Multicenter Analysis.

Prodhan P, Agarwal A, ElHassan NO, Bolin EH, Beam B, Garcia X, Gaies M, Tang X.

Ann Thorac Surg. 2017 Apr;103(4):1308-1314. doi: 10.1016/j.athoracsur.2016.09.016. Epub 2016 Nov 16.

PMID: 27865477

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  1. Fontan Patient Survival After Pediatric Heart Transplantation Has Improved in the Current Era.

Simpson KE, Pruitt E, Kirklin JK, Naftel DC, Singh RK, Edens RE, Barnes AP, Canter CE.

Ann Thorac Surg. 2017 Apr;103(4):1315-1320. doi: 10.1016/j.athoracsur.2016.08.110. Epub 2016 Nov 15.

PMID: 27863728

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  1. Postoperative Infection in Developing World Congenital Heart Surgery Programs: Data From the International Quality Improvement Collaborative.

Sen AC, Morrow DF, Balachandran R, Du X, Gauvreau K, Jagannath BR, Kumar RK, Kupiec JK, Melgar ML, Chau NT, Potter-Bynoe G, Tamariz-Cruz O, Jenkins KJ.

Circ Cardiovasc Qual Outcomes. 2017 Apr;10(4). pii: e002935. doi: 10.1161/CIRCOUTCOMES.116.002935.

PMID: 28408715

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  1. Comparison of paracorporeal and continuous flow ventricular assist devices in children: preliminary results.

Nassar MS, Hasan A, Chila T, Schueler S, Pergolizzi C, Reinhardt Z, Lord S, De Rita F, Ferguson L, Smith J, Haynes S, O’Sullivan J, Murtuza B.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):709-714. doi: 10.1093/ejcts/ezx006.

PMID: 28329112

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  1. Five-year experience with immediate extubation after arterial switch operations for transposition of great arteries.

Varghese J, Kutty S, Bisselou Moukagna KS, Craft M, Abdullah I, Hammel JM.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):728-734. doi: 10.1093/ejcts/ezw424.

PMID: 28199509

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  1. The problematic of polytetrafluoroethylene membranes for valvular repair.

Tasoglu I, Avci T, Collaku A, Paç M.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):805. doi: 10.1093/ejcts/ezw396. No abstract available.

PMID: 28040675

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  1. Total endoscopic repair of unroofed coronary sinus syndrome via right mini-thoracotomy.

Zaikokuji K, Sawazaki M, Tomari S, Imaeda Y.

Gen Thorac Cardiovasc Surg. 2017 Apr;65(4):206-208. doi: 10.1007/s11748-015-0601-4. Epub 2015 Nov 16.

PMID: 26572766

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  1. Mid-term Outcomes of Common Congenital Heart Defects Corrected Through a Right Subaxillary Thoracotomy.

An G, Zhang H, Zheng S, Wang W, Ma L.

Heart Lung Circ. 2017 Apr;26(4):376-382. doi: 10.1016/j.hlc.2015.05.028. Epub 2016 Feb 17.

PMID: 27876287

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  1. Bidirectional cavopulmonary anastomosis with additional pulmonary blood flow: good or bad pre-Fontan strategy†.

Nichay NR, Gorbatykh YN, Kornilov IA, Soynov IA, Ivantsov SM, Gorbatykh AV, Ponomarev DN, Bogachev-Prokophiev AV, Karaskov AM.

Interact Cardiovasc Thorac Surg. 2017 Apr 1;24(4):582-589. doi: 10.1093/icvts/ivw429.

PMID: 28093463

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  1. The “basic” approach: a single-centre experience with a cost-reducing model for paediatric cardiac extracorporeal membrane oxygenation.

Padalino MA, Tessari C, Guariento A, Frigo AC, Vida VL, Marcolongo A, Zanella F, Harvey MJ, Thiagarajan RR, Stellin G.

Interact Cardiovasc Thorac Surg. 2017 Apr 1;24(4):590-597. doi: 10.1093/icvts/ivw381.

PMID: 28077509

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  1. Lung ultrasound profile after cardiopulmonary bypass in paediatric cardiac surgery: first experience in a simple cohort†.

Vitale V, Ricci Z, Gaddi S, Testa G, Toma P, Cogo P.

Interact Cardiovasc Thorac Surg. 2017 Apr 1;24(4):598-602. doi: 10.1093/icvts/ivw357.

PMID: 28040749

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  1. The impact of liver disorders on perioperative management of reoperative cardiac surgery: a retrospective study in adult congenital heart disease patients.

Adachi K, Toyama H, Kaiho Y, Adachi O, Hudeta H, Yamauchi M.

J Anesth. 2017 Apr;31(2):170-177. doi: 10.1007/s00540-017-2308-4. Epub 2017 Jan 16.

PMID: 28091794

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  1. Preclinical study of patient-specific cell-free nanofiber tissue-engineered vascular grafts using 3-dimensional printing in a sheep model.

Fukunishi T, Best CA, Sugiura T, Opfermann J, Ong CS, Shinoka T, Breuer CK, Krieger A, Johnson J, Hibino N.

J Thorac Cardiovasc Surg. 2017 Apr;153(4):924-932. doi: 10.1016/j.jtcvs.2016.10.066. Epub 2016 Nov 14.

PMID: 27938900

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  1. Pilot randomized controlled trial on early and late remote ischemic preconditioning prior to complex cardiac surgery in young infants.

Guerra GG, Joffe AR, Seal R, Phillipos E, Wong M, Moez EK, Dinu IA, Duff JP, Ross D, Rebeyka I, Robertson CM.

Paediatr Anaesth. 2017 Apr;27(4):433-441. doi: 10.1111/pan.13125.

PMID: 28300357

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  1. Increased Atrial β-Adrenergic Receptors and GRK-2 Gene Expression Can Play a Fundamental Role in Heart Failure After Repair of Congenital Heart Disease with Cardiopulmonary Bypass.

Oliveira MS, Carmona F, Vicente WVA, Manso PH, Mata KM, Celes MR, Campos EC, Ramos SG.

Pediatr Cardiol. 2017 Apr;38(4):734-745. doi: 10.1007/s00246-017-1573-1. Epub 2017 Feb 18.

PMID: 28214967

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  1. Effect of Fenestration on Early Postoperative Outcome in Extracardiac Fontan Patients with Different Risk Levels.

Fan F, Liu Z, Li S, Yi T, Yan J, Yan F, Wang X, Wang Q.

Pediatr Cardiol. 2017 Apr;38(4):643-649. doi: 10.1007/s00246-016-1561-x. Epub 2017 Jan 23.

PMID: 28116475

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  1. Pediatric airway surgery.

Maeda K.

Pediatr Surg Int. 2017 Apr;33(4):435-443. doi: 10.1007/s00383-016-4050-7. Epub 2017 Jan 28. Review.

PMID: 28132084

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  1. Cardiac Biomarkers of Low Cardiac Output Syndrome in the Postoperative Period After Congenital Heart Disease Surgery in Children.

Pérez-Navero JL, de la Torre-Aguilar MJ, Ibarra de la Rosa I, Gil-Campos M, Gómez-Guzmán E, Merino-Cejas C, Muñoz-Villanueva MC, Llorente-Cantarero FJ.

Rev Esp Cardiol (Engl Ed). 2017 Apr;70(4):267-274. doi: 10.1016/j.rec.2016.09.011. Epub 2016 Oct 27. English, Spanish.

PMID: 28137395

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  1. Equine Cardiovascular Therapeutics.

Sleeper MM.

Vet Clin North Am Equine Pract. 2017 Apr;33(1):163-179. doi: 10.1016/j.cveq.2016.11.005. Review.

PMID: 28325177

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  1. [Association between fluid overload and acute renal injury after congenital heart disease surgery in infants].

Luo DQ, Chen ZL, Dai W, Chen F.

Zhongguo Dang Dai Er Ke Za Zhi. 2017 Apr;19(4):376-380. Chinese.

PMID: 28407819 Free Article

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  1. Cardioprotective Effects of Wharton Jelly Derived Mesenchymal Stem Cell Transplantation in a Rodent Model of Myocardial Injury.

Gaafar T, Attia W, Mahmoud S, Sabry D, Aziz OA, Rasheed D, Hamza H.

Int J Stem Cells. 2017 Apr 30. doi: 10.15283/ijsc16063. [Epub ahead of print]

PMID: 28446005 Free Article

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  1. The Evolution of a Pediatric Ventricular Assist Device Program: The Boston Children’s Hospital Experience.

Hawkins B, Fynn-Thompson F, Daly KP, Corf M, Blume E, Connor J, Porter C, Almond C, VanderPluym C.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1615-8. [Epub ahead of print]

PMID: 28456829

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  1. Three-Dimensional Modeling in Congenital and Structural Heart Perioperative Care and Education: A Path in Evolution.

Sarris GE, Polimenakos AC.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1614-9. [Epub ahead of print] No abstract available.

PMID: 28456827

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  1. Emergency department utilization in pediatric heart transplant recipients.

Wittlieb-Weber CA, Rossano JW, Weber DR, Lin KY, Ravishankar C, Mascio CE, Shaddy RE, O’Connor MJ.

Pediatr Transplant. 2017 Apr 29. doi: 10.1111/petr.12936. [Epub ahead of print]

PMID: 28455909

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  1. Impact of Surgery in Ebstein’s Anomaly Using Current Surgical Criteria.

Ávila-Vanzzini N, Fritche-Salazar JF, Herrera-Bello H, Espinola-Zavaleta N, Kuri Alfaro J, Rodriguez-Zanella H, Álvarez Bran MV, Cossio Aranda J, Gaxiola Macias MBA, Curi-Curi P.

Circ J. 2017 Apr 26. doi: 10.1253/circj.CJ-17-0062. [Epub ahead of print]

PMID: 28450668 Free Article

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  1. Heart Transplantation in a Toddler with Cardiac Kawasaki Disease.

Tirilomis T, Steinmetz M, Grossmann M, Bräuer A, Paul T, Ruschewski W, Schöndube FA.

Front Surg. 2017 Apr 20;4:21. doi: 10.3389/fsurg.2017.00021. eCollection 2017.

PMID: 28473974

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  1. Immunodepression after CPB: Cytokine dynamics and clinics after pediatric cardiac surgery – A prospective trial.

Justus G, Walker C, Rosenthal LM, Berger F, Miera O, Schmitt KRL.

Cytokine. 2017 Apr 11. pii: S1043-4666(17)30085-6. doi: 10.1016/j.cyto.2017.03.017. [Epub ahead of print]

PMID: 28411047

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  1. Unplanned cardiac reoperations and interventions during long-term follow-up after the Norwood procedure†.

Sames-Dolzer E, Gierlinger G, Kreuzer M, Schrempf J, Gitter R, Prandstetter C, Tulzer G, Mair R.

Eur J Cardiothorac Surg. 2017 Apr 11. doi: 10.1093/ejcts/ezx038. [Epub ahead of print]

PMID: 28402400

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  1. Complicated Postoperative Course after Pulmonary Artery Sling Repair and Slide Tracheoplasty.

Weber A, Donner B, Perez MH, Di Bernardo S, Trachsel D, Sandu K, Sekarski N.

Front Pediatr. 2017 Apr 10;5:67. doi: 10.3389/fped.2017.00067. eCollection 2017.

PMID: 28443268 Free PMC Article

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  1. Neonatal repair of left atrial diverticulum with gigantic thrombus without cardiopulmonary bypass.

Higashida A, Hoashi T, Sakaguchi H, Ichikawa H.

Gen Thorac Cardiovasc Surg. 2017 Apr 8. doi: 10.1007/s11748-017-0775-z. [Epub ahead of print]

PMID: 28391520

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  1. Cancer recurrence and mortality after pediatric heart transplantation for anthracycline cardiomyopathy: A report from the Pediatric Heart Transplant Study (PHTS) group.

Bock MJ, Pahl E, Rusconi PG, Boyle GJ, Parent JJ, Twist CJ, Kirklin JK, Pruitt E, Bernstein D.

Pediatr Transplant. 2017 Apr 4. doi: 10.1111/petr.12923. [Epub ahead of print]

PMID: 28378408

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  1. Twin-to-Twin Heart Transplantation: A Unique Event With a 25-Year Follow-Up.

Blitzer D, Yedlicka G, Manghelli J, Dentel J, Caldwell R, Brown JW.

Ann Thorac Surg. 2017 Apr;103(4):e341-e342. doi: 10.1016/j.athoracsur.2016.09.060.

PMID: 28359493

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  1. Cavopulmonary Anastomosis During Same Hospitalization as Stage 1 Norwood/Hybrid Palliative Surgery.

Tadphale SD, Tang X, ElHassan NO, Beam B, Prodhan P.

Ann Thorac Surg. 2017 Apr;103(4):1285-1291. doi: 10.1016/j.athoracsur.2017.01.013. Epub 2017 Mar 6.

PMID: 28274521

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  1. Allograft Heart Valves: Current Aspects and Future Applications.

Lisy M, Kalender G, Schenke-Layland K, Brockbank KG, Biermann A, Stock UA.

Biopreserv Biobank. 2017 Apr;15(2):148-157. doi: 10.1089/bio.2016.0070. Epub 2017 Feb 2. Review.

PMID: 28151005

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  1. Dissecting aneurysm of the interventricular septum due to rupture of the sinus of Valsalva.

Atiq M, Ali F, Hasan SB.

Cardiol Young. 2017 Apr;27(3):609-612. doi: 10.1017/S1047951116001621. Epub 2016 Nov 22.

PMID: 27873570

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Select item 27817752


  1. Successful replacement of the systemic tricuspid valve with a mechanical valve in a 3-month-old boy with congenitally corrected transposition of the great arteries having a dysplastic tricuspid valve.

Asada D, Ikeda K, Yamagishi M.

Cardiol Young. 2017 Apr;27(3):597-599. doi: 10.1017/S1047951116001578. Epub 2016 Sep 29.

PMID: 27680744

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  1. Impact of age on incidence and prevalence of moderate-to-severe cellular rejection detected by routine surveillance biopsy in pediatric heart transplantation.

Zinn MD, Wallendorf MJ, Simpson KE, Osborne AD, Kirklin JK, Canter CE.

J Heart Lung Transplant. 2017 Apr;36(4):451-456. doi: 10.1016/j.healun.2016.09.017. Epub 2016 Oct 7.

PMID: 27865735

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  1. Vascular endothelial growth factor A is associated with the subsequent development of moderate or severe cardiac allograft vasculopathy in pediatric heart transplant recipients.

Daly KP, Stack M, Eisenga MF, Keane JF, Zurakowski D, Blume ED, Briscoe DM.

J Heart Lung Transplant. 2017 Apr;36(4):434-442. doi: 10.1016/j.healun.2016.09.013. Epub 2016 Oct 3.

PMID: 27865734

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  1. The effects of postoperative hematocrit on shunt occlusion for neonates undergoing single ventricle palliation.

Anderson BR, Blancha VL, Duchon JM, Chai PJ, Kalfa D, Bacha EA, Krishnamurthy G, Ratner V.

J Thorac Cardiovasc Surg. 2017 Apr;153(4):947-955. doi: 10.1016/j.jtcvs.2016.09.085. Epub 2016 Nov 14.

PMID: 27938905

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  1. Peritoneal Dialysis vs Furosemide for Prevention of Fluid Overload in Infants After Cardiac Surgery: A Randomized Clinical Trial.

Kwiatkowski DM, Goldstein SL, Cooper DS, Nelson DP, Morales DL, Krawczeski CD.

JAMA Pediatr. 2017 Apr 1;171(4):357-364. doi: 10.1001/jamapediatrics.2016.4538.

PMID: 28241247

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  1. Veno-Venous ECMO in Europe: are we all speaking the same language?

Pappalardo F, Montisci A, Scandroglio A, Belliato M, Malfertheiner M, Broman M, David P, Lorusso R, Taccone F, Broccati F, DI Nardo M.

Minerva Anestesiol. 2017 Apr;83(4):424-425. doi: 10.23736/S0375-9393.16.11766-3. Epub 2016 Nov 9. No abstract available.

PMID: 27827522

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  1. Routine Application of Bloodless Priming in Neonatal Cardiopulmonary Bypass: A 3-Year Experience.

Boettcher W, Sinzobahamvya N, Miera O, Redlin M, Dehmel F, Cho MY, Murin P, Berger F, Photiadis J.

Pediatr Cardiol. 2017 Apr;38(4):807-812. doi: 10.1007/s00246-017-1585-x. Epub 2017 Feb 14.

PMID: 28197644

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  1. Predicting Fluid Responsiveness in Children Undergoing Cardiac Surgery After Cardiopulmonary Bypass.

Favia I, Romagnoli S, Di Chiara L, Ricci Z.

Pediatr Cardiol. 2017 Apr;38(4):787-793. doi: 10.1007/s00246-017-1582-0. Epub 2017 Feb 11.

PMID: 28190141

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  1. Association Between Hematologic and Inflammatory Markers and 31 Thrombotic and Hemorrhagic Events in Berlin Heart Excor Patients.

Iyengar A, Hung ML, Asanad K, Kwon OJ, Jackson NJ, Reemtsen BL, Federman MD, Biniwale RM.

Pediatr Cardiol. 2017 Apr;38(4):770-777. doi: 10.1007/s00246-017-1578-9. Epub 2017 Feb 10.

PMID: 28184979

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  1. Alteration of Cardiac Deformation in Acute Rejection in Pediatric Heart Transplant Recipients.

Chanana N, Van Dorn CS, Everitt MD, Weng HY, Miller DV, Menon SC.

Pediatr Cardiol. 2017 Apr;38(4):691-699. doi: 10.1007/s00246-016-1567-4. Epub 2017 Feb 4.

PMID: 28161809

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ACHD Featured Articles of April 2017

Adult Congenital Heart Reviews of April 2017 Manuscripts, sponsored by the International Society for Adult Congenital Heart Disease


  1. Maternal and Fetal Outcomes of Admission for Delivery in Women With Congenital Heart Disease. Hayward RM, Foster E, Tseng ZH. JAMA Cardiol. 2017 Apr 12. doi: 10.1001/jamacardio.2017.0283. [Epub ahead of print] PMID: 28403428 Similar articles Select item 28412020


Take Home Points:

  • California delivery hospitals demonstrated an association with congenital heart disease, and congestive heart failure admissions, atrial arrhythmias, and fetal growth restriction.
  • Maternal complex congenital heart disease is associated with ventricular arrhythmias and maternal in hospital mortality, though rare.
  • Increased monitoring at the time of delivery may be recommended as a result.
  • Post delivery there is an increased likelihood of readmission that is markedly increased when compared to pregnant women without CHD.


Moe_Tabitha-WEBCommentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch:

This investigation from January 1st 2005 to December 31st, 2011 reviewed more than 3 million deliveries in the state of California with its diverse population, and a variety of centers.  Of those 3 million deliveries, 3189 women had noncomplex congenital heart disease and 262 had complex congenital heart disease. Women with CHD were more likely to undergo Caesarian delivery (39%), compared with women without CHD (32%). Interestingly 54.9% of the women in this cohort who had  Tetralogy of Fallot underwent C-section. This marked increase above baseline C-section rates clashes with the maternal indications for C-section, suggesting that the number of C-sections performed on women with TOF is out of proportion with those that are considered necessary by WHO risk stratification criteria. Complex congenital heart disease was associated with greater adjusted odds of serious ventricular arrhythmias and maternal in hospital mortality. Both simple and complex congenital heart disease women were at higher risk for readmission, atrial arrhythmias, heart failure, and fetal growth restriction as compared with the women without congenital heart disease.

This study expertly molds a large publicly available dataset from the HCUP data at UCSF allowing the authors to investigate an important question relating to outcomes associated with pregnancy in CHD.  Though valuable overall, this large database is limited by the specificity of coding classifications, and the transition from ICD 9 to ICD 10 in recent years, which occurred following the data collected in this paper.  Complex CHD included endocardial cushion defects, hypoplastic left heart syndrome, tetralogy of Fallot, transposition of the great arteries, truncus arteriosus, and univentricular heart defects.  All other defects were classified as noncomplex.

ACHD1 v2

The figure above alludes to the increasing likelihood of readmissions for patients with congenital heart disease over the course of the 6 years following delivery. Though this data does not differentiate non pregnancy related complications from overall cardiac complications, it does suggest that pregnancy may present an important milestone determining later outcomes. However, no comparative non-pregnant data is presented, making proper interpretation of such data somewhat speculative

This is nevertheless an important study as it demonstrates an overall decrease in maternal comorbidities compared with the previously reported data by Siu et al. More study as well as collaboration is certainly needed.



NT-proBNP predicts mortality in adults with transposition of the great arteries late after Mustard or Senning correction.

Popelová JR, Tomková M, Tomek J.

Congenit Heart Dis. 2017 Apr 17. doi: 10.1111/chd.12466. [Epub ahead of print]

PMID: 28419713

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Take Home Points:


  • Patients with a Mustard or Senning operation are at risk of unexpected deaths due to heart failure and arrhythmias
  • NT-proBNP was the most useful independent predictor of mortality (HR 281.39), using univariate Cox Regression. A value of 1000 pg/mL had a 88% sensitivity and 95% specificity for the prediction of mortality.
  • Surprisingly, mitral E velocity of 0.68 m/s or greater was associated with improved survival i.e. in the subpulmonary ventricle.


Blanche CupidoCommentary from Dr Blanche Cupido (Cape Town, South Africa), section editor of ACHD Journal Watch: Mustard and Senning corrections for transposition of the great arteries have largely been replaced by the arterial switch operation. However a number of such operations continue to be performed in the developing world for simple transposition of the great arteries. Although most patients remain asymptomatic for many years, they may die unexpectedly, usually as a result of heart failure of arrhythmias.  As risk factors for mortality, especially sudden cardiac death remain elusive, the authors of this study sought to investigate the predictors for mortality in adult patients with Mustard or Senning repairs.


Eighty-seven patients (39 Senning and 48 Mustard) were followed in an outpatient setting over a 10 years (2005-2015).  They underwent serial clinical [all], echocardiographic [all] and NT-proBNP measurements. CMR data was available in 18% and VO2max measurements in 44%.


The majority of patients were male (n=64, 74%). Those with a Mustard correction were significantly older (age 30 vs 21, p<0.0001) and had higher NT-proBNP levels (287 vs 144, p=0.00058) when compared to Senning patients.


The overall mortality was 9% (n=8) with no significant differences between the two groups. One of the patients died of early rejection post transplantation, the rest were due to heart failure.



NT-proBNP and the mitral E velocity were the only two independent predictors for death that emerged on multivariable analysis (NT-proBNP HR 281.39 (CI 13.06-6063, p=0.00048; and mitral E velocity HR 0.95 (CI 0.90-0.99, p=0.01815).



An NT-proBNP level of <1000 pg/mL correlated with improved survival.



A baseline mitral E velocity of >0.68m/s was associated with improved survival. Further validation is required. NYHA class and doppler and tissue doppler parameters relating to the systemic RV, surprisingly, did not show significant differences.

The findings of this study need verification however in larger datasets. Because of the relatively small numbers in this particular study, and the small number of outcomes, multivariable time dependent analysis was not possible.


Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012.

Heart. 2017 Apr 27. pii: heartjnl-2016-310979. doi: 10.1136/heartjnl-2016-310979. [Epub ahead of print]

Hjortshøj CS1Jensen AS1Sørensen K2Nagy E3Johansson B4Kronvall T5Dellborg M6Estensen ME7Holmstrøm H8Turanlahti M9Thilén U10Søndergaard L1.

PMID: 28450553

Take Home Points:
• During the last 4 decades, the incidence and prevalence of Eisenmenger syndrome in the Nordic region has decreased.
• During the last 4 decades, the age of death increased, which may not necessarily reflect improved survival, but is more likely the result of decreased incidence and shifting of age distribution.
• Median survival of Eisenmenger syndrome patients is significantly lower than the background population.
• Down syndrome patients with Eisenmenger syndrome appear to have worse survival.

jokhadar-maanCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch: Eisenmenger syndrome (ES) is a late complication of unrepaired large systemic to pulmonary shunt that is characterized by increased pulmonary vascular resistance, pulmonary hypertension, and cyanosis with its associated features of digital clubbing and exertional dyspnea. ES was more common before modern diagnostic methods and surgical correction became available.
Though several studies report ES survival rates, the results are often influenced by so-called “immortal time bias” also known as survivor bias. Because recruit starts at time of diagnosis of start of late follow-up, there is an inherent bias toward survivors as earlier events such as mortality are not normally capture in such analyses. ES late survival is therefore likely to be overestimated when age is used as the time scale variable for Kaplan-Meier survival analyses without accounting for all events that have happened from the time of birth, which for most studies is not feasible.
Therefore, the time from diagnosis to start of follow-up should be removed from the analysis, by accounting for the age at entry to the study.
With this in mind, Dr. Hjortshøj and colleagues from Denmark, Sweden, Norway, and Finland performed a retrospective population based analysis through an extensive search of national registries and found 714 patients with ES between 1977 and 2012. It should be noted that patients with surgically corrected shunts but with persistent pulmonary hypertension were not included in the study.
The results were as follows:
ES incidence decreased from 2.5 to 0.23/million inhabitants/year.
ES prevalence decreased from 24.6 to 12.0/million inhabitants.
The mean age of the study population increased from about 19.9 in 1977 to 40.4 in 2012. The rate of deaths per million inhabitants increased as well. The absolute number of children with ES decreased throughout the study period.
The annual mortality rate was about 2.3%
The median survival was about 38.4 years, which is lower than the median survival of 81.6 years for the matched background control population.
Patients with simple shunts appear to survive longer than patients with complex lesions.
Down syndrome patients appeared to have worse mortality, particularly after factoring in the high preoperative mortality following shunt corrective surgery.
The mean age of death was 27.7 during the first era of the study (1977 to 1992), which was 38.8 during the second era (1993 to 2006), and was 46.3 during the last era (2006 to 2012). Though the study showed an increase in the age of death throughout the study, this may not represent improved longevity, but rather the result of decreasing incidence on age distribution that leads to an older study population.
The results of this study suggest that a reduced incidence of ES is due to earlier detection and treatment of patients with congenital heart disease. However, there will likely remain a small subset of patients with complex lesions who are unsuitable for surgical correction and will go on to develop ES. These findings in this Nordic population are likely similar to other developed countries, but quite different from developing countries where early access to diagnosis and treatment is less common.
Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome.

Hascoët S1, Baruteau AE2, Humbert M3, Simonneau G3, Jais X3, Petit J2, Laux D2, Sitbon O3, Lambert V4, Capderou A5.

J Heart Lung Transplant. 2017 Apr;36(4):386-398. doi: 10.1016/j.healun.2016.10.006. Epub 2016 Oct 15.



Take Home Points:


  • There is little long-term data in usefulness of PAH-specific drug therapy (PAH-SDT) in Eisenmenger syndrome
  • In this study, 69 patients with Eisenmenger syndrome were followed over 20 years using a structured follow-up including thorough hemodynamics (with measured VO2) and clinical status
  • PAH-SDT resulted in improvement in measured hemodynamics in 68.0% of patients; however after a median of 4.9 years, changes in hemodynamic measures were no longer significant
  • Even with PAH-SDT, there was a high event rate (death, heart-lung transplantation, or listing for transplantation), with a 15-year cumulative event rate of 49.2%
  • Independent predictors of events were superior vena cava oxygen saturation and hemodynamic reponse to PAH-SDT


Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch: This study from France quite thoroughly evaluated a cohort of 69 patients with Eisenmenger syndrome who were treated with PAH-specific drug therapy (PAH-SDT).  All patients underwent thorough invasive hemodynamics, including a measured, rather than calculated, VO2, as well as 6-minute walk distance and WHO functional status.    All patients had measurement of indexed pulmonary output (Qpi), pulmonary vascular index (PVRi), and superior vena cava oxygen saturation.  All patients had an initial PVRi > 8 Woods units * m2 at the start of the study, and all patients were on PAH-SDT (i.e. there was no control group).

Measured hemodynamics improved in the majority of patients, 68.0% of the sample. The hemodynamic response and clinical response was generally initially good.  Overall increase in Qpi was 0.4 LPM/m2.  Overall increase in 6MW distane was +49 m.   Changes in hemodynamics and 6MW distance were assessed after each change in treatment (i.e. intensification or addition of a 2nd or 3rd drug).  The treatment effect was found not to be sustained over long-term follow-up, with changes no longer being significant after a median of 4.9 years.

Events were common; 33.3% of patients met the composite endpoint of death, heart-lung transplant, or listing for transplantation after a median of 7.2 years.  The 15-year cumulative event rate was 49.2%.  Median time to event was 8.1 years.  Patients with events had worse hemodynamics at baseline.  On univariate analysis, predictors of events were a PVRi > 30 Woods Units * m2, right atrial pressure > 4 mmHg, and SVC saturation < 65%, and pulmonary artery saturation < 70%.  On multivariate analysis,  SVC saturation was found to correlate with event rate.  Each 1% decrease in SVC saturation was associated with a 9.8% increase in hazard ratio for events.  SVC saturation in Eisenmenger syndrome reflects both arterial saturation as well as systemic cardiac output.

This study showed some evidence that patients with pretricuspid shunts may have more benefit from PAH-SDT than those with posttricuspid shunts; however the authors caution this may be artifact from “immortal survival bias.”  Atrial septal defects were the most common underlying CHD in this cohort (n=43, 62.3%).

The authors conclude that PAH-SDT can be helpful in Eisenmenger syndrome, but the regimen may need to be individualized base on hemodynamic changes for each patient.




Figure 4 Kaplan-Meier cumulative event curves in the sub-group (E),aortic oxygen saturation, (F) SVC oxygen saturation, (G),and pattern of hemodynamic change under PAH-SDT(H).




Is the presence of end-diastolic forward flow specific for restrictive right ventricular physiology in repaired tetralogy of Fallot?


Mori Y, Murakami T, Inoue N, Kaneko S, Nakashima Y, Koide M.

Int J Cardiol. 2017 Apr 11. pii: S0167-5273(16)34242-5. doi: 10.1016/j.ijcard.2017.04.021. [Epub ahead of print]



Take Home Points:


  • From past publications, end diastolic forward flow (EDFF) in repaired tetralogy of Fallot [TOF] has been considered to be synonymous with restrictive right ventricular (RV) physiology.
  • In the present study patients with EDFF have different hemodynamics as compared to those without: higher RA pressure, higher RA to PA pressures gradient, and had higher ANP levels
  • Not all patients who demonstrate EDFF, have restrictive RV physiology when taking invasive hemodynamics into account
  • Patients with a small RV and EDFF demonstrate restrictive RV physiology, and demonstrate associated invasive hemodynamics
  • Patients with large RV and EDFF do not demonstrate restrictive RV physiology. Instead, they may have very low pulmonary diastolic pressure, which is lower than right atrial pressure, resulting in EDFF during RA contraction.


Leong Ming ChernCommentary from Dr. M.C. Leong (Kuala Lumpur), section editor of ACHD Journal Watch:  The presence of end diastolic forward flow (EDFF) in the pulmonary arteries on echocardiography or cardiac magnetic resonance has been regarded as the hallmark echocardiopgraphic feature of restrictive right ventricular (RV) physiology in repaired Tetralogy of Fallot. However not all such patients have good late outcomes. A recent study found that TOF patients with EDFF have good effort tolerance only if associated with small right ventricles. The authors of the current study therefore sought to demonstrate that not all patients with EDFF on echocardiography have higher right ventricular filling pressures via cardiac catheterizations.


62 patients with repaired TOF were retrospectively reviewed. They were aged 15.7 ± 11.6 years, and underwent cardiac catheterization. Patients were then divided into Group 1 (n=23) with and Group 2 (n=39) without EDFF; Group A (n=53) with small RVs (<150ml/m2) and Group B (n=9) with larger RV (≥150ml/m2). Group 1 had a higher right atrial pressure, pressure gradient between the right atrium and pulmonary diastolic pressure and atrial natriuretic peptide levels. Group A had better RV and left ventricular ejection fraction and smaller LV size.


However, on further subgroup analysis, patients with EDFF and smaller RV (Group 1A) had higher right atrial pressure and less pulmonary regurgitation than the rest of the groups. Patients with large RV and EDFF (Group 1B) had right atrial pressure and RV end diastolic pressure similar to those without EDFF. This group of patients had higher pressure gradient between the right atrium and pulmonary diastolic pressure compared to patients without EDFF.



The authors hence postulate that patients in Group 1A corresponds to “true” restrictive RV physiology. However, patients in Group 1B may not have true restrictive RV physiology, despite EDFF. Instead, they may have severe pulmonary regurgitation which results in very low pulmonary diastolic pressure, which created a high gradient between the right atrium and pulmonary diastolic pressure and hence EDFF.



ACHD April 2017


  1. Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012.

Hjortshøj CS, Jensen AS, Sørensen K, Nagy E, Johansson B, Kronvall T, Dellborg M, Estensen ME, Holmstrøm H, Turanlahti M, Thilén U, Søndergaard L.

Heart. 2017 Apr 27. pii: heartjnl-2016-310979. doi: 10.1136/heartjnl-2016-310979. [Epub ahead of print]

PMID: 28450553

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Select item 28449285


  1. Research Review: Childhood chronic physical illness and adult emotional health – a systematic review and meta-analysis.

Secinti E, Thompson EJ, Richards M, Gaysina D.

J Child Psychol Psychiatry. 2017 Apr 27. doi: 10.1111/jcpp.12727. [Epub ahead of print] Review.

PMID: 28449285

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Select item 28450386


  1. Heart transplantation for adults with congenital heart disease: current status and future prospects.

Matsuda H, Ichikawa H, Ueno T, Sawa Y.

Gen Thorac Cardiovasc Surg. 2017 Apr 24. doi: 10.1007/s11748-017-0777-x. [Epub ahead of print] Review.

PMID: 28439697

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Select item 28436859


  1. Critical involvement of ZEB2 in collagen fibrillogenesis: the molecular similarity between Mowat-Wilson syndrome and Ehlers-Danlos syndrome.

Teraishi M, Takaishi M, Nakajima K, Ikeda M, Higashi Y, Shimoda S, Asada Y, Hijikata A, Ohara O, Hiraki Y, Mizuno S, Fukada T, Furukawa T, Wakamatsu N, Sano S.

Sci Rep. 2017 Apr 19;7:46565. doi: 10.1038/srep46565.

PMID: 28422173 Free PMC Article

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Select item 28422570


  1. Attitudes and perceptions of pregnant women with CHD: results of a single-site survey.

Sabanayagam A, Briston D, Zaidi AN.

Cardiol Young. 2017 Apr 18:1-8. doi: 10.1017/S1047951116002857. [Epub ahead of print]

PMID: 28416028

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Select item 28430913


  1. Person-centred transition programme to empower adolescents with congenital heart disease in the transition to adulthood: a study protocol for a hybrid randomised controlled trial (STEPSTONES project).

Acuña Mora M, Sparud-Lundin C, Bratt EL, Moons P.

BMJ Open. 2017 Apr 17;7(4):e014593. doi: 10.1136/bmjopen-2016-014593.

PMID: 28420661 Free Article

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Select item 28412993


  1. Paediatric-onset coronary artery anomalies in pregnancy: a single-centre experience and systematic literature review.

Keir M, Bhagra C, Vatenmakher D, Arancibia-Galilea F, Jansen K, Toh N, Silversides CK, Colman J, Siu SC, Sermer M, Crean AM, Wald RM.

Cardiol Young. 2017 Apr 17:1-9. doi: 10.1017/S1047951117000658. [Epub ahead of print]

PMID: 28412993

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Select item 28419713


  1. NT-proBNP predicts mortality in adults with transposition of the great arteries late after Mustard or Senning correction.

Popelová JR, Tomková M, Tomek J.

Congenit Heart Dis. 2017 Apr 17. doi: 10.1111/chd.12466. [Epub ahead of print]

PMID: 28419713

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Select item 28417257


  1. Stroke in adults with Down syndrome.

Marto JP, Dias M, Alves JN, Montes V, Beato-Coelho J, Marques-Matos C, de Sousa A, Bernardo F, Calado S, Viana-Baptista M.

J Neurol Sci. 2017 Apr 15;375:279-280. doi: 10.1016/j.jns.2017.02.014. Epub 2017 Feb 8. No abstract available.

PMID: 28320148

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Select item 28408455


  1. Pregnancy in Adult Congenital Heart Disease: Special Delivery.

Davidson WR Jr.

JAMA Cardiol. 2017 Apr 12. doi: 10.1001/jamacardio.2017.0365. [Epub ahead of print] No abstract available.

PMID: 28403448

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Select item 28403428


  1. Maternal and Fetal Outcomes of Admission for Delivery in Women With Congenital Heart Disease.

Hayward RM, Foster E, Tseng ZH.

JAMA Cardiol. 2017 Apr 12. doi: 10.1001/jamacardio.2017.0283. [Epub ahead of print]

PMID: 28403428

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Select item 28412020


  1. Is Takotsubo Syndrome a benign condition?

Drakopoulou MI, Gatzoulis MA.

Hellenic J Cardiol. 2017 Apr 8. pii: S1109-9666(17)30141-0. doi: 10.1016/j.hjc.2017.03.009. [Epub ahead of print]

PMID: 28400292 Free Article

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Select item 28385091


  1. Hypoplasia of the posterior mitral leaflet: A rare cause of mitral regurgitation in adulthood.

Bacich D, Braggion G, Faggian G.

Echocardiography. 2017 Apr 7. doi: 10.1111/echo.13537. [Epub ahead of print]

PMID: 28386957

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Select item 28396253


  1. Plastic bronchitis: An unusual complication of acute chest syndrome in adult.

Feray S, Mora P, Decavele M, Pham T, Hafiani EM, Fartoukh M.

Respir Med Case Rep. 2017 Apr 7;21:93-95. doi: 10.1016/j.rmcr.2017.04.006. eCollection 2017.

PMID: 28443234 Free PMC Article

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Select item 28343629


  1. Cardiopulmonary Exercise Testing in Adult Congenital Heart Disease.

Mantegazza V, Apostolo A, Hager A.

Ann Am Thorac Soc. 2017 Apr 4. doi: 10.1513/AnnalsATS.201611-876FR. [Epub ahead of print]

PMID: 28375677

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Select item 28395882


  1. Incidence and predictors of obstetric and fetal complications in women with structural heart disease.

van Hagen IM, Roos-Hesselink JW, Donvito V, Liptai C, Morissens M, Murphy DJ, Galian L, Bazargani NM, Cornette J, Hall R, Johnson MR.

Heart. 2017 Apr 4. pii: heartjnl-2016-310644. doi: 10.1136/heartjnl-2016-310644. [Epub ahead of print]

PMID: 28377476

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Select item 28377475


  1. Diagnosis, imaging and clinical management of aortic coarctation.

Dijkema EJ, Leiner T, Grotenhuis HB.

Heart. 2017 Apr 4. pii: heartjnl-2017-311173. doi: 10.1136/heartjnl-2017-311173. [Epub ahead of print] Review.

PMID: 28377475

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Select item 28359509


  1. Clinical Characteristics of Adult Dogs More Than 5 Years of Age at Presentation for Patent Ductus Arteriosus.

Boutet BG, Saunders AB, Gordon SG.

J Vet Intern Med. 2017 Apr 3. doi: 10.1111/jvim.14689. [Epub ahead of print]

PMID: 28370380 Free Article

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Select item 28384795


  1. Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature.

Smith K, Gros B.

Congenit Heart Dis. 2017 Apr 2. doi: 10.1111/chd.12465. [Epub ahead of print] Review.

PMID: 28371362

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Select item 28441822


  1. [Assessment of undiagnosed critical congenital heart disease before discharge from the maternity hospital].

Zhao QM, Liu F, Wu L, Ye M, Jia B, Ma XJ, Huang GY.

Zhonghua Er Ke Za Zhi. 2017 Apr 2;55(4):260-266. doi: 10.3760/cma.j.issn.0578-1310.2017.04.006. Chinese.

PMID: 28441821

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Select item 28441820


  1. National In-Hospital Outcomes of Pregnancy in Women With Single Ventricle Congenital Heart Disease.

Collins RT 2nd, Chang D, Sandlin A, Goudie A, Robbins JM.

Am J Cardiol. 2017 Apr 1;119(7):1106-1110. doi: 10.1016/j.amjcard.2016.12.015. Epub 2017 Feb 16.

PMID: 28242012

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Select item 28328118


  1. High prevalence of fatigue in adults with a 22q11.2 deletion syndrome.

Vergaelen E, Claes S, Kempke S, Swillen A.

Am J Med Genet A. 2017 Apr;173(4):858-867. doi: 10.1002/ajmg.a.38094. Epub 2017 Feb 12.

PMID: 28190295

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Select item 27956203


  1. Biomarkers of Calcific Aortic Valve Disease.

Small A, Kiss D, Giri J, Anwaruddin S, Siddiqi H, Guerraty M, Chirinos JA, Ferrari G, Rader DJ.

Arterioscler Thromb Vasc Biol. 2017 Apr;37(4):623-632. doi: 10.1161/ATVBAHA.116.308615. Epub 2017 Feb 2. Review.

PMID: 28153876

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Select item 28212920


  1. Bicuspid aortic valve outcomes.

Rodrigues I, Agapito AF, de Sousa L, Oliveira JA, Branco LM, Galrinho A, Abreu J, Timóteo AT, Rosa SA, Ferreira RC.

Cardiol Young. 2017 Apr;27(3):518-529. doi: 10.1017/S1047951116002560. Epub 2016 Dec 12.

PMID: 27938448

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  1. Comparison of participants and non-participants in patient-reported outcome surveys: the case of Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease – International Study.

Berghammer MC, Mattsson E, Johansson B, Moons P, Dellborg M.

Cardiol Young. 2017 Apr;27(3):427-434. doi: 10.1017/S1047951116000676. Epub 2016 May 11.

PMID: 27167242

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  1. Tricuspid annular plane systolic excursion is preserved in young patients with pulmonary hypertension except when associated with repaired congenital heart disease.

Hauck A, Guo R, Ivy DD, Younoszai A.

Eur Heart J Cardiovasc Imaging. 2017 Apr 1;18(4):459-466. doi: 10.1093/ehjci/jew068.

PMID: 27099276

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  1. Does functional capacity depend on the size of the shunt? A prospective, cohort study of adults with small, unrepaired ventricular septal defects.

Maagaard M, Heiberg J, Asschenfeldt B, Ringgaard S, Hjortdal VE.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):722-727. doi: 10.1093/ejcts/ezw420.

PMID: 28204298

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  1. The effect of a resilience improvement program for adolescents with complex congenital heart disease.

Lee S, Lee J, Choi JY.

Eur J Cardiovasc Nurs. 2017 Apr;16(4):290-298. doi: 10.1177/1474515116659836. Epub 2016 Jul 10.

PMID: 27400701

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  1. Recommendations on the Echocardiographic Assessment of Aortic Valve Stenosis: A Focused Update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

Baumgartner H, Hung J, Bermejo J, Chambers JB, Edvardsen T, Goldstein S, Lancellotti P, LeFevre M, Miller F Jr, Otto CM.

J Am Soc Echocardiogr. 2017 Apr;30(4):372-392. doi: 10.1016/j.echo.2017.02.009.

PMID: 28385280

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  1. Clinical recommendations of cardiac magnetic resonance, Part II: inflammatory and congenital heart disease, cardiomyopathies and cardiac tumors: a position paper of the working group ‘Applicazioni della Risonanza Magnetica’ of the Italian Society of Cardiology.

Pontone G, Di Bella G, Silvia C, Maestrini V, Festa P, Ait-Ali L, Masci PG, Monti L, di Giovine G, De Lazzari M, Cipriani A, Guaricci AI, Dellegrottaglie S, Pepe A, Marra MP, Aquaro GD.

J Cardiovasc Med (Hagerstown). 2017 Apr;18(4):209-222. doi: 10.2459/JCM.0000000000000499.

PMID: 28092290

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  1. Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome.

Hascoët S, Baruteau AE, Humbert M, Simonneau G, Jais X, Petit J, Laux D, Sitbon O, Lambert V, Capderou A.

J Heart Lung Transplant. 2017 Apr;36(4):386-398. doi: 10.1016/j.healun.2016.10.006. Epub 2016 Oct 15.

PMID: 27866929

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  1. [The Relationships among Perceived Parental Bonding, Illness Perception, and Anxiety in Adult Patients with Congenital Heart Diseases].

Shin N, Jang Y, Kang Y.

J Korean Acad Nurs. 2017 Apr;47(2):178-187. doi: 10.4040/jkan.2017.47.2.178. Korean.

PMID: 28470155

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  1. Use of Cardiac Computed Tomography for Ventricular Volumetry in Late Postoperative Patients with Tetralogy of Fallot.

Kim HJ, Mun DN, Goo HW, Yun TJ.

Korean J Thorac Cardiovasc Surg. 2017 Apr;50(2):71-77. doi: 10.5090/kjtcs.2017.50.2.71. Epub 2017 Apr 5.

PMID: 28382264 Free PMC Article

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  1. Readiness for Transition to Adult Health Care for Young Adolescents with Congenital Heart Disease.

Stewart KT, Chahal N, Kovacs AH, Manlhiot C, Jelen A, Collins T, McCrindle BW.

Pediatr Cardiol. 2017 Apr;38(4):778-786. doi: 10.1007/s00246-017-1580-2. Epub 2017 Feb 10.

PMID: 28184978

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  1. Decreased Diastolic Ventricular Kinetic Energy in Young Patients with Fontan Circulation Demonstrated by Four-Dimensional Cardiac Magnetic Resonance Imaging.

Sjöberg P, Heiberg E, Wingren P, Ramgren Johansson J, Malm T, Arheden H, Liuba P, Carlsson M.

Pediatr Cardiol. 2017 Apr;38(4):669-680. doi: 10.1007/s00246-016-1565-6. Epub 2017 Feb 10. Erratum in: Pediatr Cardiol. 2017 Apr 29;:.

PMID: 28184976 Free PMC Article

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  1. Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome.

Arnold R, Neu M, Hirtler D, Gimpel C, Markl M, Geiger J.

Pediatr Radiol. 2017 Apr;47(4):382-390. doi: 10.1007/s00247-016-3767-8. Epub 2017 Feb 9.

PMID: 28184962

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  1. Relationship Between Fragmented QRS Complexes and Cardiac Status in Duchenne Muscular Dystrophy: Multimodal Validation Using Echocardiography, Magnetic Resonance Imaging, and Holter Monitoring.

Cho MJ, Lee JW, Lee J, Shin YB, Lee HD.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1616-7. [Epub ahead of print]

PMID: 28456833

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  1. Validation of numerical simulation methods in aortic arch using 4D Flow MRI.

Miyazaki S, Itatani K, Furusawa T, Nishino T, Sugiyama M, Takehara Y, Yasukochi S.

Heart Vessels. 2017 Apr 25. doi: 10.1007/s00380-017-0979-2. [Epub ahead of print]

PMID: 28444501

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  1. Natural history of aortic root dilation through young adulthood in a hypermobile Ehlers-Danlos syndrome cohort.

Ritter A, Atzinger C, Hays B, James J, Shikany A, Neilson D, Martin L, Weaver KN.

Am J Med Genet A. 2017 Apr 24. doi: 10.1002/ajmg.a.38243. [Epub ahead of print]

PMID: 28436618

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  1. Topsy-turvy heart and associated imaging findings.

Bayramoglu Z, Yılmaz R, Demir AA, Yekeler E, Dursun M, Dindar A, Nisli K, Omeroglu R.

J Cardiovasc Comput Tomogr. 2017 Apr 20. pii: S1934-5925(17)30093-X. doi: 10.1016/j.jcct.2017.04.008. [Epub ahead of print] No abstract available.

PMID: 28438441

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  1. Is the presence of end-diastolic forward flow specific for restrictive right ventricular physiology in repaired tetralogy of Fallot?

Mori Y, Murakami T, Inoue N, Kaneko S, Nakashima Y, Koide M.

Int J Cardiol. 2017 Apr 11. pii: S0167-5273(16)34242-5. doi: 10.1016/j.ijcard.2017.04.021. [Epub ahead of print]

PMID: 28457561

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  1. Aortopathy in an Adult With Tricuspid Atresia and Left Ventricular Non-Compaction After Fontan Procedure.

Murakami T, Mori Y, Inoue N, Kaneko S, Nakashima Y.

Circ J. 2017 Apr 5. doi: 10.1253/circj.CJ-17-0016. [Epub ahead of print] No abstract available.

PMID: 28381694 Free Article

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  1. Presentation of missed childhood Kawasaki disease in adults: Experience from a tertiary care center in north India.

Vijayvergiya R, Bhattad S, Varma S, Singhal M, Gordon J, Singh S.

Int J Rheum Dis. 2017 Apr 5. doi: 10.1111/1756-185X.13073. [Epub ahead of print]

PMID: 28378434

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  1. Revisiting the utility of technical performance scores following tetralogy of Fallot repair.

Lodin D, Mavrothalassitis O, Haberer K, Sunderji S, Quek RGW, Peyvandi S, Moon-Grady A, Karamlou T.

J Thorac Cardiovasc Surg. 2017 Apr 5. pii: S0022-5223(17)30640-2. doi: 10.1016/j.jtcvs.2017.02.066. [Epub ahead of print]

PMID: 28461051

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  1. Fontan survival: As good as it gets?

Thankavel P, Jaquiss RDB.

J Thorac Cardiovasc Surg. 2017 Apr 4. pii: S0022-5223(17)30633-5. doi: 10.1016/j.jtcvs.2017.03.114. [Epub ahead of print] No abstract available.

PMID: 28434620

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  1. Decreased false-positive adolescent pre-athletic screening with Seattle Criteria-interpreted electrocardiograms.

Colombo JN, Samson RA, Valdes SO, Meziab O, Sisk D, Klewer SE.

Cardiol Young. 2017 Apr;27(3):512-517. doi: 10.1017/S104795111600086X. Epub 2016 Jun 20.

PMID: 27322729

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  1. Mechanism of Progressive Heart Failure and Significance of Pulmonary Hypertension in Obstructive Hypertrophic Cardiomyopathy.

Covella M, Rowin EJ, Hill NS, Preston IR, Milan A, Opotowsky AR, Maron BJ, Maron MS, Maron BA.

Circ Heart Fail. 2017 Apr;10(4):e003689. doi: 10.1161/CIRCHEARTFAILURE.116.003689.

PMID: 28396501

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  1. Resilience and personal growth: A potential resource for therapeutic programmes in people with congenital heart disease.

Dorka R.

Eur J Cardiovasc Nurs. 2017 Apr;16(4):270-271. doi: 10.1177/1474515116687223. Epub 2017 Jan 10. No abstract available.

PMID: 28071940

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  1. Loss-of-function mutations in the X-linked biglycan gene cause a severe syndromic form of thoracic aortic aneurysms and dissections.

Meester JA, Vandeweyer G, Pintelon I, Lammens M, Van Hoorick L, De Belder S, Waitzman K, Young L, Markham LW, Vogt J, Richer J, Beauchesne LM, Unger S, Superti-Furga A, Prsa M, Dhillon R, Reyniers E, Dietz HC, Wuyts W, Mortier G, Verstraeten A, Van Laer L, Loeys BL.

Genet Med. 2017 Apr;19(4):386-395. doi: 10.1038/gim.2016.126. Epub 2016 Sep 15.

PMID: 27632686 Free PMC Article

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  1. Does Body Mass Index Predict Premature Cardiomyopathy Onset for Duchenne Muscular Dystrophy?

McKane M, Soslow JH, Xu M, Saville BR, Slaughter JC, Burnette WB, Markham LW.

J Child Neurol. 2017 Apr;32(5):499-504. doi: 10.1177/0883073816687422. Epub 2017 Jan 13.

PMID: 28084148

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  1. Clinical and magnetic resonance evolution of “infarct-like” myocarditis.

Faletti R, Gatti M, Baralis I, Bergamasco L, Bonamini R, Ferroni F, Imazio M, Stola S, Gaita F, Fonio P.

Radiol Med. 2017 Apr;122(4):273-279. doi: 10.1007/s11547-016-0723-5. Epub 2017 Jan 9.

PMID: 28070840

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  1. The importance of the neutrophil-to-lymphocyte ratio in patients with hypertrophic cardiomyopathy.

Ozyilmaz S, Akgul O, Uyarel H, Pusuroglu H, Gul M, Satilmisoglu MH, Bolat I, Ozyilmaz I, Uçar H, Yildirim A, Bakir I.

Rev Port Cardiol. 2017 Apr;36(4):239-246. doi: 10.1016/j.repc.2016.09.014. Epub 2017 Mar 18. English, Portuguese.

PMID: 28318851

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