ACHD Featured Articles of December 2016

Mode of Delivery and Pregnancy Outcome in Women with Congenital Heart Disease.

Hrycyk J, Kaemmerer H, Nagdyman N, Hamann M, Schneider K, Kuschel B.

PLoS One. 2016 Dec 22;11(12):e0167820. doi: 10.1371/journal.pone.0167820.

Take Home Points:

  • Primary C-section for cardiac indications may be used too liberally for the excellent vaginal delivery outcomes that have been repeatedly reported.
  • Once CHD patients have undergone a primary C-section they are more than twice as likely to undergo a repeat C-section than women without CHD.
  • Planned vaginal delivery via induction of labor, and failed induction increases C-section rates in CHD patients significantly more than in patients without CHD.

Moe_Tabitha-WEBCommentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch: In general cardiologists recommend vaginal delivery for women with CHD to avoid complications from Caesarian section as many women with CHD tolerate vaginal delivery well. This is a single-centered study evaluating their patient cohort in Munich, Germany.  They reported outcomes of 116 patients with CHD.  Of the CHD patients 46.6% of patients underwent C-section compared to 33.6% without CHD.  33.3% of women with CHD had primary planned C-section for cardiac indications.  Induction of labor was performed in 45.7% of attempt vaginal deliveries in women with CHD.  Fetal outcomes for CHD demonstrated a lower mean birth weight, and an increased frequency of small for gestational age babies in women with CHD.  One CHD patient had a post-partum hemorrhage.  This study represents 86 patients of at least moderate complexity CHD who are Functional Class I-II.  The majority of the patients are complex lesions including ccTGA, TGA, TOF, CoA, and DORV. They did not report any patients who were functional class IV.  This maternal risk factor most frequently leading to C-section was prior C-section which occurred more than twice as frequently in patients with CHD than in the non-CHD cohort. Cardiac reasons were 33.3% of primary C-section indications in women with CHD.  The concerns regarding maternal cardiac deterioration seem unfounded and may be able to reduce the rates of C-section in women with CHD. If we are to reduce the number of C-section performed in CHD patients then a reduction in planned induction of labor, a reduction in planned primary C-section, and an increase in attempts at vaginal delivery after C-section are all reasonable to consider. Additional considerations including a critical evaluation of hospital to hospital variability in C-section rates, as well as the distance the patient resides from the delivery hospital are warranted.

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Pulmonary hypertension in adults with congenital heart disease: First data from Latvian PAH registry.

Skride A, Sablinskis K, Lejnieks A, Rudzitis A.

Eur J Intern Med. 2016 Dec;36:e20-e21. doi: 10.1016/j.ejim.2016.09.002. No abstract available.

Take Home Points:

  • Late diagnosis, and referral to tertiary care centers for PAH after declining functional class leads to poor outcomes.
  • Highly effective screening tools and mechanisms in systems without established ACHD care centers would be very valuable in Latvia and similar settings.
  • Latvia demonstrates a disproportionately high PAH-CHD patient cohort in their reported PAH population which is likely multi-factorial.

Commentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch: In Pulmonary Arterial Hypertension is a known complication of unrepaired shunts, but also of shunts that were not repaired in a timely manner.  10% of adults with CHD will develop PAH, although prevalence varies widely among studies.  TTE and RHC are recommended to determine causality and course of PAH-CHD. Current guidelines classify patients into 4 clinical groupings.  Bosentan is the only ERA to be proven via an RCT to be beneficial in patients with Eisenmenger Syndrome. This study is a prospective, observational, single-center study of the Latvian PAH registry.  They included 41 patients over the age of 18.  The excluded PVOD-type diagnoses from this study.  Diagnosis was confirmed by TTE, and RHC.  11 patients had Eisenmenger Syndrome.  The majority of their patients were female.  There was no difference found in the gender distribution between Eisenmenger and non-Eisenmenger patients.  The median age at time of diagnosis was 55 years.  Patients with ES were significantly younger than those without.  ASD was the most common defect in their cohort.  The majority of the patients were in NYHA class III functional status. Most patients received disease specific therapy following their diagnosis including sildenafil, ambrisentan and bosentan.  PAH-CHD accounts for 33.3% of all PAH patients which is proportionally larger of a share than in French or American registries.  They do not have a transition program or and ACHD program for the care of adults with CHD. Patients are being diagnosed late in their course of disease with very advanced PAH and functional status decline. This further highlights the need for proper screening as well as transitional care programs for ACHD patients in the international arena. Partnering with developing programs in growing world populations will increasingly be valuable.

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Assessment of Kidney Function in Survivors Following Fontan Palliation.

Sharma S, Ruebner RL, Furth SL, Dodds KM, Rychik J, Goldberg DJ.

Congenit Heart Dis. 2016 Dec;11(6):630-636. doi: 10.1111/chd.12358.

Take Home Points:

  • There are several potential reasons why Fontan survivors may have decreased renal function, but little data regarding incidence of kidney disease in this population is available.
  • This study is the first study of renal function in a series of Fontan survivors to also use a control group.
  • 10% of Fontan subjects in this study had an estimated GFR < 90 mL/min/1.73 m2.
  • Cystatin C may be a more accurate reflection of renal function than creatinine in Fontan patients due to a higher proportion of patients with low muscle mass. 

Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch:  This study comes from the Single Ventricle Survivorship Program at Children’s Hospital of Philadelphia.  A retrospective cohort of 68 Fontan subjects were compared to age- and sex- matched normal controls recruited from a general pediatrics clinic.   A variety of mechanisms in the Fontan circulation can lead to worsened kidney function, including lower cardiac output, higher venous pressures, chronic cyanosis, and use of nephrotoxic medications.

Renal function variables included creatinine, cystatin C, and intact parathyroid hormone levels, as well as the presence of proteinuria.  The primary endpoint was CKD, defined as an eGFR < 90 ml/min/1.73 m2 using equations based on creatinine and cystatin C.

Median eGFRs were comparable between Fontan patients and controls.  However, about 10% of Fontan subjects had an eGFR < 90 ml/min/1.73 m2.  Median intact parathyroid hormone levels were higher in the Fontans than the controls (59.4 pg/mL versus 23.4 pg/mL, respectively, p ≤ .001.  Proteinuria was present in 10% of the Fontan group compared to 4.7% in controls, but this difference was not statistically significant (p = .27).  Only five patients in the Fontan cohort had proteinuria, and three were on an ACE or ARB.

Of note, in this study, although median serum creatinine levels were slightly lower among Fontan subjects compared with controls, the Fontan group had higher median cystatin C levels.  The Fontan group also had a larger prevalence of lower height and BMI.  The authors hypothesize that creatinine may not be reliable way to assess kidney function in Fontan patients due to lower muscle mass in this group of patients.

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The Management of the third stage of labour in women with heart disease. 

Cauldwell M, Steer PJ, Swan L, Uebing A, Gatzoulis MA, Johnson MR.

Heart. 2016 Dec 19. pii: heartjnl-2016-310607. doi: 10.1136/heartjnl-2016-310607. [Epub ahead of print]

Take Home Points: 

  • Uterine atony is the main cause of postpartum haemorrhage in women with heart disease.
  • Standard ‘low-dose’ oxytocin infusion (10 U of oxytocin in 500 mL of normal saline given over 4 hours (12 mU/ min) is ineffective in preventing postpartum haemorrhage. Nearly 1 in four patients on standard ‘low dose’ oxytocin suffers from postpartum haemorrhage.
  • Giving an additional 2IU of oxytocin over 2 minutes results in significantly less postpartum blood loss, without compromising hemodynamics or increasing risk of arrhythmias.

Commentary from Dr. M.C. Leong Ming ChernLeong (Kuala Lumpur, Malaysia), section editor of ACHD Journal Watch:  Part of the management of third stage of labour involves prophylactically administering a uterotonic agent to prevent postpartum hemorrhage (PPH). Women with heart disease have higher propensity for PPH, however, uterotonic agents e.g. oxytocin and ergometrine affects cardiac hemodynamics. Administering ‘low dose’ oxytocin (10 U oxytocin in 500mL of normal saline, given over 4 hours) is a safe and common practice. Nevertheless, this low dose of oxytocin treatment fails to prevent PPH in nearly one fourth of the case.

The authors sought to study whether the administration of an additional 2IU on top of the conventional ‘low dose’ oxytocin, results in less blood loss postpartum without compromising the patients’ hemodynamics. This is a single centre, cohort study involving 29 patients in the conventional ‘low dose’ arm and another 30 patients in the ‘low dose’ oxytocin with an additional 2IU (high dose arm). Patients in the high dose group demonstrated lower blood loss volume compared to the low dose group. There were fewer PPH in the high dose group (2/30 vs 13/29). No statically significant difference in blood pressure and heart rate were observed. No patients in either group had any episodes of arrhythmias after initiation of oxytocin.

The authors concluded that the use of an additional 2U of oxytocin, when given slowly, for the management of third stage of labor in women with heart disease had no cardiac consequences and was associated with a significantly lower blood loss. This supports the guidance from the ESC that suggested the addition of oxytocin dose. However, the authors cautioned that patients with different types and severity of heart disease may demonstrate different tolerance towards hemodynamic changes and adjustment of the dose of oxytocin according to the severity of the underlying heart disease may be needed.

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ACHD Articles – December 2016


  1. Are Olympic athletes free from cardiovascular diseases? Systematic investigation in 2352 participants from Athens 2004 to Sochi 2014.

Pelliccia A, Adami PE, Quattrini F, Squeo MR, Caselli S, Verdile L, Maestrini V, Di Paolo F, Pisicchio C, Ciardo R, Spataro A.

Br J Sports Med. 2016 Dec 30. pii: bjsports-2016-096961. doi: 10.1136/bjsports-2016-096961. [Epub ahead of print]

  1. Hippocampal damage and Memory Impairment in Congenital Cyanotic Heart Disease.

Muñoz-López M, Hoskote A, Chadwick MJ, Dzieciol AM, Gadian DG, Chong K, Banks T, de Haan M, Baldeweg T, Mishkin M, Vargha-Khadem F.

Hippocampus. 2016 Dec 29. doi: 10.1002/hipo.22700. [Epub ahead of print]

  1. Adult congenital heart disease: the challenges of a lifetime.

Warnes CA.

Eur Heart J. 2016 Dec 23. pii: ehw529. doi: 10.1093/eurheartj/ehw529. [Epub ahead of print] Review.

  1. Cardiac remodelling amongst adults with various aetiologies of pulmonary arterial hypertension including Eisenmenger syndrome-implications on survival and the role of right ventricular transverse strain.

Moceri P, Bouvier P, Baudouy D, Dimopoulos K, Cerboni P, Wort SJ, Doyen D, Schouver ED, Gibelin P, Senior R, Gatzoulis MA, Ferrari E, Li W.

Eur Heart J Cardiovasc Imaging. 2016 Dec 23. pii: jew277. doi: 10.1093/ehjci/jew277. [Epub ahead of print]

  1. Mode of Delivery and Pregnancy Outcome in Women with Congenital Heart Disease.

Hrycyk J, Kaemmerer H, Nagdyman N, Hamann M, Schneider K, Kuschel B.

PLoS One. 2016 Dec 22;11(12):e0167820. doi: 10.1371/journal.pone.0167820.

  1. Hopelessness among adults with congenital heart disease: Cause for despair or hope?

Eslami B, Kovacs AH, Moons P, Abbasi K, Jackson JL.

Int J Cardiol. 2016 Dec 21. pii: S0167-5273(16)34580-6. doi: 10.1016/j.ijcard.2016.12.090. [Epub ahead of print]

  1. The management of the third stage of labour in women with heart disease.

Cauldwell M, Steer PJ, Swan L, Uebing A, Gatzoulis MA, Johnson MR.

Heart. 2016 Dec 19. pii: heartjnl-2016-310607. doi: 10.1136/heartjnl-2016-310607. [Epub ahead of print]

  1. Survivorship in Children and Young Adults With Congenital Heart Disease in Sweden.

Mandalenakis Z, Rosengren A, Skoglund K, Lappas G, Eriksson P, Dellborg M.

JAMA Intern Med. 2016 Dec 19. doi: 10.1001/jamainternmed.2016.7765. [Epub ahead of print]

  1. A nationwide evaluation of spontaneous coronary artery dissection in pregnancy and the puerperium.

Faden MS, Bottega N, Benjamin A, Brown RN.

Heart. 2016 Dec 15;102(24):1974-1979. doi: 10.1136/heartjnl-2016-309403.

  1. Social determinants of health: integral to developmental risk assessment in congenital heart disease.

Wong P, Denburg A, Moore A, Ford-Jones E.

J Pediatr. 2016 Dec 14. pii: S0022-3476(16)31364-6. doi: 10.1016/j.jpeds.2016.11.058. [Epub ahead of print] No abstract available.

  1. Neurocognitive functioning in adults with congenital heart disease.

Ilardi D, Ono KE, McCartney R, Book W, Stringer AY.

Congenit Heart Dis. 2016 Dec 13. doi: 10.1111/chd.12434. [Epub ahead of print]

  1. Syphilis during pregnancy: a preventable threat to maternal-fetal health.

Rac MW, Revell PA, Eppes CS.

Am J Obstet Gynecol. 2016 Dec 9. pii: S0002-9378(16)32167-6. doi: 10.1016/j.ajog.2016.11.1052. [Epub ahead of print] Review.

  1. What Do We Know About Chagas Disease in the United States?

Montgomery SP, Parise ME, Dotson EM, Bialek SR.

Am J Trop Med Hyg. 2016 Dec 7;95(6):1225-1227.

  1. A rare case of congenital heart disease with first presentation in adulthood.

Amin R, Lee D, Camm CF, Walker DM.

Br J Hosp Med (Lond). 2016 Dec 2;77(12):718-719. No abstract available.

  1. Prevalence of Cancer in Adults With Congenital Heart Disease Compared With the General Population.

Gurvitz M, Ionescu-Ittu R, Guo L, Eisenberg MJ, Abrahamowicz M, Pilote L, Marelli AJ.

Am J Cardiol. 2016 Dec 1;118(11):1742-1750. doi: 10.1016/j.amjcard.2016.08.057.

  1. Sequential Right and Left Ventricular Assessment in Posttetralogy of Fallot Patients with Significant Pulmonary Regurgitation.

Wijesekera VA, Raju R, Precious B, Berger AJ, Kiess MC, Leipsic JA, Grewal J.

Congenit Heart Dis. 2016 Dec;11(6):606-614. doi: 10.1111/chd.12354.

  1. Sleep-Disordered Breathing in Patients with Pulmonary Valve Incompetence Complicating Congenital Heart Disease.

Miles S, Ahmad W, Bailey A, Hatton R, Boyle A, Collins N.

Congenit Heart Dis. 2016 Dec;11(6):678-682. doi: 10.1111/chd.12369.

  1. Assessment of Kidney Function in Survivors Following Fontan Palliation.

Sharma S, Ruebner RL, Furth SL, Dodds KM, Rychik J, Goldberg DJ.

Congenit Heart Dis. 2016 Dec;11(6):630-636. doi: 10.1111/chd.12358.

  1. Altered Aortic Upper Wall TDI Velocity Is Inversely Related with Left Ventricular Diastolic Function in Operated Tetralogy of Fallot.

Bassareo PP, Saba L, Marras AR, Mercuro G.

Congenit Heart Dis. 2016 Dec;11(6):598-605. doi: 10.1111/chd.12350.

  1. Acute Myocardial Infarction in Adult Congenital Patients with Bodily Isomerism.

Loomba RS, Aggarwal S, Buelow M, Nijhawan K, Gupta N, Alla V, Arora RR.

Congenit Heart Dis. 2016 Dec;11(6):548-553. doi: 10.1111/chd.12336.

  1. Pulmonary Hypertension in Heart Failure Patients: Pathophysiology and Prognostic Implications.

Guazzi M, Labate V.

Curr Heart Fail Rep. 2016 Dec;13(6):281-294. Review.

  1. Incidental finding of aberrant mitral valve chorda during a transesophageal echocardiography evaluation for suspected endocarditis.

Siddiqui I, Nguyen T, Nekkanti R, Movahed A.

Echocardiography. 2016 Dec;33(12):1931-1933. doi: 10.1111/echo.13346.

  1. [Infective endocarditis in adult patients with congenital heart disease. Experience from a reference centre].

Loureiro-Amigo J, Fernández-Hidalgo N, Pijuan-Domènech A, Dos-Subirà L, Subirana-Domènech T, Gonzàlez-Alujas T, González-López JJ, Tornos-Mas P, García-Dorado D, Almirante B.

Enferm Infecc Microbiol Clin. 2016 Dec;34(10):626-632. doi: 10.1016/j.eimc.2016.01.004. Spanish.

  1. Sense of coherence does not moderate the relationship between the perceived impact of stress on health and self-rated health in adults with congenital heart disease.

Apers S, Sevenants L, Budts W, Luyckx K, Moons P.

Eur J Cardiovasc Nurs. 2016 Dec;15(7):529-536.

  1. Pulmonary hypertension in adults with congenital heart disease: First data from Latvian PAH registry.

Skride A, Sablinskis K, Lejnieks A, Rudzitis A.

Eur J Intern Med. 2016 Dec;36:e20-e21. doi: 10.1016/j.ejim.2016.09.002. No abstract available.

  1. Blood coagulation abnormalities and the usefulness of D-dimer level for detecting intracardiac thrombosis in adult Fontan patients.

Takeuchi D, Inai K, Shinohara T, Nakanishi T, Park IS.

Int J Cardiol. 2016 Dec 1;224:139-144. doi: 10.1016/j.ijcard.2016.09.017.

  1. Bicuspid Aortic Valve: a Review with Recommendations for Genetic Counseling.

Freeze SL, Landis BJ, Ware SM, Helm BM.

J Genet Couns. 2016 Dec;25(6):1171-1178. Review.

  1. Studying the pathophysiologic connection between cardiovascular and nervous systems using stem cells.

Coskun V, Lombardo DM.

J Neurosci Res. 2016 Dec;94(12):1499-1510. doi: 10.1002/jnr.23924. Review.

  1. Does maternal exposure during pregnancy to higher ambient temperature increase the risk of hypospadias?

Kilinc MF, Cakmak S, Demir DO, Doluoglu OG, Yildiz Y, Horasanli K, Dalkilic A.

J Pediatr Urol. 2016 Dec;12(6):407.e1-407.e6. doi: 10.1016/j.jpurol.2016.06.015.

  1. Myocardial cytochrome oxidase activity increases with age and hypoxemia in patients with congenital heart disease.

Onwugbufor M, Levy RJ, Zurakowski D, Jonas RA, Sinha P.

Perfusion. 2016 Dec 1. pii: 0267659116681435. [Epub ahead of print]

  1. 3D Heart Model and 4D Flow MRI 20 Years after Spiral Arterial Switch Operation.

Sievers HH, Kheradvar A, Kramer HH, Rickers C.

Thorac Cardiovasc Surg Rep. 2016 Dec;5(1):44-46. doi: 10.1055/s-0036-1584513.

  1. Ideal cardiovascular health in childhood-Longitudinal associations with cardiac structure and function: The Special Turku Coronary Risk Factor Intervention Project (STRIP) and the Cardiovascular Risk in Young Finns Study (YFS).

Laitinen TT, Ruohonen S, Juonala M, Magnussen CG, Mikkilä V, Mikola H, Hutri-Kähönen N, Laitinen T, Tossavainen P, Jokinen E, Niinikoski H, Jula A, Viikari JS, Rönnemaa T, Raitakari OT, Pahkala K.

Int J Cardiol. 2016 Dec 23. pii: S0167-5273(16)34608-3. doi: 10.1016/j.ijcard.2016.12.117. [Epub ahead of print]

  1. Assessment of myocardial function in elite athlete’s heart at rest – 2D speckle tracking echocardiography in Korean elite soccer players.

Eun LY, Chae HW.

Sci Rep. 2016 Dec 22;6:39772. doi: 10.1038/srep39772.

  1. Changes in the Professional Lives of Cardiologists Over 2 Decades.

Lewis SJ, Mehta LS, Douglas PS, Gulati M, Limacher MC, Poppas A, Walsh MN, Rzeszut AK, Duvernoy CS; American College of Cardiology Women in Cardiology Leadership Council..

J Am Coll Cardiol. 2016 Dec 16. pii: S0735-1097(16)37115-7. doi: 10.1016/j.jacc.2016.11.027. [Epub ahead of print] Review.

  1. A Case Report of Reversible Takotsubo Cardiomyopathy after Amphetamine/Dextroamphetamine Ingestion in a 15-Year-Old Adolescent Girl.

Toce MS, Farias M, Bruccoleri R, Brown DW, Burns MM.

J Pediatr. 2016 Dec 13. pii: S0022-3476(16)31267-7. doi: 10.1016/j.jpeds.2016.11.038. [Epub ahead of print]

  1. Coronary Artery Evaluation by Screening Echocardiogram in Intercollegiate Athletes.

Hoyt WJ, Dean PN, Schneider DS, Conaway MR, Kramer CM, Battle RW.

Med Sci Sports Exerc. 2016 Dec 6. [Epub ahead of print]

  1. De Novo Migraine With Aura After Surgical Repair of Aortic Coarctation.

Kato Y, Hayashi T, Kobayashi T, Masuoka A, Abe T, Hasebe T, Tanahashi N, Takao M.

Headache. 2016 Dec 2. doi: 10.1111/head.12995. [Epub ahead of print]

  1. Parental vitamin D deficiency during pregnancy is associated with increased blood pressure in offspring via Panx1 hypermethylation.

Meems LM, Mahmud H, Buikema H, Tost J, Michel S, Takens J, Verkaik-Schakel RN, Vreeswijk-Baudoin I, Mateo-Leach IV, van der Harst P, Plösch T, de Boer RA.

Am J Physiol Heart Circ Physiol. 2016 Dec 1;311(6):H1459-H1469. doi: 10.1152/ajpheart.00141.2016.

  1. Adult patent ductus arteriosus : An unusual cause of heart failure in an octogenarian female.

Mueller S, Plank F, Klimes K, Feuchtner G, Mair J.

Wien Klin Wochenschr. 2016 Dec;128(23-24):925-927.

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