Pharmacotherapy of Heart Failure in Infants with Congenital Heart Disease

Authors

  • Dr. Reiner Buchhorn

DOI:

https://doi.org/10.34257/LJMHR226113UK

Keywords:

ACE inhibitor, beta blocker, heart failure, heart rate variability, Keywords: congenital heart disease, pharmacotherapy

Abstract

Every second patient who dies from congenital heart disease is an infant with heart failure. However, infants were de facto excluded from the US Carvedilol trial and PANORAMA HF trial (Sacubitril/Valsartan), probably due to their high mortality risk. Despite the negative results of the US Enalapril trial in infants with univentricular hearts, ACE inhibitors are further recommended in the guidelines. Propranolol is the only drug that has been successful in two prospective randomized trials but was not recommended in the guidelines.

This review discusses the dif erences between myocardial heart failure and congestive circulatory failure in infants with congenital heart disease that do not benefit from vasodilators but from beta-blockers that significantly improve clinical symptoms, neurohormonal activation and heart rate variability.

The case of a non-invasive monitoring of heart rate variability, blood pressure and oxygen saturation of an infant with Down syndrome and complete atrioventricular septal defect visualize cardiac decompensation after enalapril and the benefits of beta-blocker treatment.

References

Levin, Li, Krasuski (2025) Abstract 4369203: Mortality Rates and Causes of Death in Patients with Congenital Heart Disease (CHD) Across the Lifespan: Population-Based Findings from the CDC- Funded STAR1 Study. 152(Suppl_3), A4369203–A03. https://doi.org/10.1161/circ.152.suppl_3.4369203

Apitz, Beuren (1963) Congenital Heart Defects During The 1st Year of Life. A Survey of 630 Infants with Heart Disease. 42, 264–76.

Sommers, Nagel, Neudorf (2005) Congestive heart failure in childhood. An epidemiologic study. 30(7), 652–62.

Bergh, Skoglund, Fedchenko (2023) Risk of Heart Failure in Congenital Heart Disease: A Nationwide Register-Based Cohort Study. 147(12), 982–84. https://doi.org/10.1161/circulationaha.122.061546

Abdul-Khaliq, Gomes, Meyer (2024) Trends of mortality rate in patients with congenital heart defects in Germany-analysis of nationwide data of the Federal Statistical Office of Germany.

Packer (1992) The neurohormonal hypothesis: a theory to explain the mechanism of disease progression in heart failure. 20(1), 248–54. https://doi.org/10.1016/0735-1097(92)

Katz, Feinberg, Shaffer (1960) Hemodynamic aspects of congestive heart failure. 21, 95–111. https://doi.org/10.1161/01.cir.21.1.95

Irving, Azeka, Adorisio (2025) The International Society for Heart and Lung Transplantation Guidelines for the Management of Pediatric Heart Failure (Update From 2014). 44(10), e21–e71.

Shaddy, Boucek, Hsu (2007) Carvedilol for children and adolescents with heart failure: a randomized controlled trial. 298(10), 1171–79.

Shaddy, Burch, Kantor (2024) Sacubitril/Valsartan in Pediatric Heart Failure (PANORAMA-HF): A Randomized, Multicenter, Double-Blind Trial. 150(22), 1756–66.

Hsu, Zak, Mahony (2010) Enalapril in infants with single ventricle: results of a multicenter randomized trial. 122(4), 333–40.

Goldblatt (1962) The treatment of cardiac failure in infancy. A review of 350 cases. 2(7249), 212–5. https://doi.org/10.1016/s0140-6736(62)

Breeyear, Keaton, Torstenson (2022) Diastolic Blood Pressure Alleles Improve Congenital Heart Defect Repair Outcomes. 130(7), 1030–37.

Wilson, Iyengar, d'Udekem (2016) The Use and Misuse of ACE Inhibitors in Patients with Single Ventricle Physiology. 25(3), 229–36. https://doi.org/10.1016/j.hlc

Buchhorn, Hammersen, Bartmus (2001) The pathogenesis of heart failure in infants with congenital heart disease. 11(5), 498–504.

Vaida, Feins, Khei (2024) Abstract 4147589: Heart Rate Variability as a Predictor of Postoperative Outcomes in Congenital Heart Surgery. 150. https://doi.org/10.1161/circ.150.Suppl1

Buchhorn, Hulpke-Wette, Hilgers (2001) Propranolol treatment of congestive heart failure in infants with congenital heart disease: The CHF-PRO-INFANT Trial. Congestive heart failure in infants treated with propanol. 79(2-3), 167–73.

Ramakrishnan, Ghati, Ahuja (2021) Efficacy and safety of propranolol in infants with heart failure due to moderate-to-large ventricular septal defect (VSD-PHF study) - A prospective randomized trial. 14(3), 331–40. https://doi.org/10.4103/apc.APC_94_21

Steichert, Cawello, Bajcetic (2023) Influence of Age, Heart Failure and ACE Inhibitor Treatment on Plasma Renin Activity in Children: Insights from a Systematic Review and the European LENA Project. 28(12), 335. https://doi.org/10.31083/j.fbl2812335

Buchhorn, Ross, Hulpke-Wette (2000) Effectiveness of low dose captopril versus propranolol therapy in infants with severe congestive failure due to left-to-right shunts. 76(2-3), 227–33.

Brown, Mangeot, Anderson (2016) Digoxin use is Associated with Reduced Interstage Mortality in Patients with No History of Arrhythmia After Stage I Palliation for Single Ventricle Heart Disease. 5(1). https://doi.org/10.1161/jaha.115.002376

Oster, Kelleman, M cCracken (2016) Association of Digoxin With Interstage Mortality: Results From the Pediatric Heart Network Single Ventricle Reconstruction Trial Public use Dataset. 5(1). https://doi.org/10.1161/jaha.115.002566

Flórez-Rodríguez, Rubio-Duarte, Basto-Duarte (2026) Perioperative use of levosimendan in complex congenital heart disease management. 185(2), 100.

Burkhardt, Hummel, Rücker (2024) Inotropes for the prevention of low cardiac output syndrome and mortality for paediatric patients undergoing surgery for congenital heart disease: a network meta-analysis. 11(11), Cd013707. https://doi.org/10.1002/146518

Mienert, Esmaeili, Steinbrenner (2021) Cardiovascular Drug Therapy during Interstage After Hybrid Approach: A Single-Center Experience in 51 Newborns with Hypoplastic Left Heart. 23(2), 195–202. https://doi.org/10.1007/s40272-

Recla, Schmidt, Logeswaran (2019) Pediatric heart failure therapy: why β1 -receptor blocker, tissue ACE-I and mineralocorticoid-receptor-blocker?. 8(2), 127–32. https://doi.org/10.21037/tp.2019.04.08

Miller, Sharma, Gongwer (2025) Neurodevelopmental Outcomes in Early Adolescence: The Pediatric Heart Network's Single Ventricle Reconstruction Trial. 152(17), 1246–61. https://doi.org/10.1016/circulationaha.125.074523

Buchhorn, Hofmann (2025) Real-Life Measurement of Vasoregulation in Patients with Cyanotic Congenital Heart Disease: A Feasibility Study. 6(4), 33.

Kiess, Bimboese, Gausche (2026) Severity of Congenital Heart Defects Affects Long- Term Somatic Development. 47(2), 642–51.

Toyofuku, Ehrler, Naef (2024) Heart rate variability and cognitive functions in adolescents with complex congenital heart disease. https://doi.org/10.1038/s4

Buchhorn, Meint, Willaschek (2016) The Impact of Early Life Stress on Growth and Cardiovascular Risk: A Possible Example for Autonomic Imprinting?. 11(11), e0166447. https://doi.org/10.1371/journal.pone

Schöneburg, Uphoff, Oberhoffer-Fritz (2025) Heart Rate Variability in Congenital Heart Disease and Controls Using Wearable Technology and a Health Application Programming Interface & #x2014;A Cross- sectional Study. https://doi.org/10.1016/j.cjcpc.2025.07

Downloads

Published

2026-05-05

How to Cite

Pharmacotherapy of Heart Failure in Infants with Congenital Heart Disease. (2026). London Journal of Medical and Health Research, 26(3), 22-28. https://doi.org/10.34257/LJMHR226113UK