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Heart Failure in Adult Congenital Heart Disease

  • Adult Congenital Heart Disease (A Bhatt and K Niwa, Section Editors)
  • Published:
Current Treatment Options in Cardiovascular Medicine Aims and scope Submit manuscript

Opinion statement

Adult congenital heart disease (ACHD) patients represent a special population in modern cardiology: though their numbers are growing, and they represent a high-resource utilization subgroup, a robust evidence-base of randomized trials is lacking. Much of the standard therapy is adapted from the treatment of ischemic and idiopathic left ventricle systolic failure, with a small, but growing body of evidence on medical therapy in select ACHD diagnoses. At our institution, for instance, there is a long tradition of using angiotensin antagonists in patients with a systemic right ventricle to prevent deleterious remodeling. The effects of beta-blockers on functional class in ACHD are yet unproven, but there is promising data on pulmonary vasodilators. Control of coronary risk factors and aerobic exercise should be considered for all. Prevention of arrhythmias is important, and multi-site pacing is an emerging therapy. New prognostic tools including natriuretic peptides and CPET are increasingly used to guide earlier initiation of these therapies.

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Abbreviations

ACEI:

Angiotensin converting enzyme inhibitor

ACHD:

Adult congenital heart disease

ARB:

Angiotensin II receptor blocker

ASD:

Atrial septal defect

AVSD:

Atrioventricular septal defect

BAV:

Bicuspid aortic valve

BNP:

Brain natriuretic peptide

CAD:

Coronary artery disease

cCTA:

Cardiac computed tomography angiogram

CI:

confidence interval; cardiac index

cMRI:

Cardiac magnetic resonance imaging

CoA:

Coarctation of the aorta

CPET:

Cardiopulmonary exercise test

EF:

Ejection fraction

HF:

Heart failure

LV:

Left ventricle

NO:

Nitric oxide

NP:

Natriuretic peptide

NT-proBNP:

N-terminal pro brain natriuretic peptide

NYHA:

New York Heart Association

PDA:

Patent ductus arteriosus

pVO2 :

Peak oxygen consumption

RV:

Right ventricle

SHFM:

Seattle Heart Failure Model

SV:

Single ventricle

TAPSE:

Tricuspid annular plane systolic excursion

TOF:

Tetralogy of Fallot

TGA:

Transposition of the great arteries

TTE:

Transthoracic echocardiogram

VSD:

Ventricular septal defect

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Conflict of Interest

Dr. Ada Stefanescu declares no potential conflicts of interest.

Dr. Defaria Yeh declares no potential conflicts of interest.

Dr. David M. Dudzinski reports consulting work for a health care consulting firm and for Sanofi Aventis; the field of the work was on legal regulation of protein-based drugs and delivery systems. Dr. Dudzinski received payment for editing the cardiology section of Lippincott Williams & Wilkins “Pocket Medicine” text.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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  1. Division of Cardiology, Institute for Heart, Vascular and Stroke Care, Yawkey 5B, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Ada Stefanescu MD CM, Doreen DeFaria Yeh MD FACC & David M. Dudzinski MD JD FAHA

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  1. Ada Stefanescu MD CM
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  2. Doreen DeFaria Yeh MD FACC
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Correspondence to Doreen DeFaria Yeh MD FACC.

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This article is part of the Topical Collection on Adult Congenital Heart Disease

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Stefanescu, A., DeFaria Yeh, D. & Dudzinski, D.M. Heart Failure in Adult Congenital Heart Disease. Curr Treat Options Cardio Med 16, 337 (2014). https://doi.org/10.1007/s11936-014-0337-y

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