Review article

https://doi.org/10.15836/ccar2016.17

Heart Failure in Adults with Congenital Heart Disease: An Emerging Challenge.

Anton Šmalcelj ; University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
Maja Strozzi ; University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
Ivan Malčić ; University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
Darko Anić ; University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia

Abstract

A growing population of young heart failure (HF) patients with complex congenital heart disease, who have survived into adulthood owing to sophisticated cardiac surgery in infancy, emerges as a challenging quandary of contemporary cardiology. This new population primarily includes patients with: 1) repaired tetralogy of Fallot with consequent severe pulmonary valve regurgitation, 2)
univentricular heart and Fontan circulation, 3) complete transposition of great arteries palliated by atrial switch surgery, 4) congenitally corrected transposition of great arteries, and 5) Eisenmenger
syndrome. Right ventricular failure is a common denominator of all those entities, while in items 3 and 4, and often also in 2, the failing right ventricle is the systemic one. The grossly distorted anatomy
of complex congenital heart disease, even if palliated by an early and often staged surgery, results in peculiar HF hemodynamics grossly aberrant from norm, but probably with similar systemic response.
Time course of HF in congenital heart disease is much different from “ordinary” HF. It occurs much earlier and is heavily dependent on surgical and percutaneous interventions. Surgery, often more palliative than corrective, defers HF portending death from infancy into early or middle adulthood, but the real challenge is how to delay it further when the surgical and percutaneous interventional possibilities are used up. Heart failure in congenital heart disease defies standard concepts of medical HF treatment. The efficacy of renin-angiotensin-aldosterone antagonists and beta blockers has not
been proven yet. Antiarrhythmic drugs are quite ineffective in grossly distorted hearts, but ablative antiarrhythmic interventions and hemodynamics improvement by pacing may be useful. Advanced
pulmonary vasodilators have revived the treatment of Eisenmenger syndrome, previously deemed incurable. The diversity of congenital disease precludes unifying treatment concepts. Specific hemodynamic conditions have to be kept in mind in an individualized clinical approach, but new ways of treatment are clearly needed, medical, interventional, and surgical ones.

Keywords

congenital heart disease; heart failure

Hrčak ID:

153648

URI

https://hrcak.srce.hr/153648

Publication date:

23.2.2016.

Article data in other languages: croatian

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