Abstract
Heart failure in adult congenital heart disease may develop in consequence of a combination of persistent volume and pressure overload; predisposing factors are abnormal anatomy, surgical sequelae, or progression of basic pathology; neurohormonal activation due to the mentioned reasons bears the hallmarks of chronic congestive heart failure in this group of patients. It was mentioned previously that there are no significant differences between dilated cardiomyopathy and HF in adult CHD patients in terms of serum neurohormone level; of course heart failure can develop at different ages with different presentations.
According to the ACC/AHA 2008 Guidelines, typical adult congenital heart disease substrates for late heart failure are severe aortic stenosis and/or regurgitation, bicuspid aortic valve and its variants, subvalvular or supravalvular pathologies, and also superimposed coarctation; severe congenital mitral stenosis or regurgitation; unoperated atrial septal defect or partial atrioventricular septal defect; congenitally corrected transposition of the great arteries; d-transposition after the Mustard or Senning surgery, in which the morphological right ventricle is the systemic ventricle; tetralogy of Fallot with early era surgery, long-standing shunt, or severe pulmonary regurgitation; single ventricle physiology; and also Fontan surgery.
The distinction between left-sided and right-sided heart failure in congenital heart disease patients is less obvious in infants than in older children or adults. Because increased filling or raised pressure of the right ventricle in infants lessens left ventricular compliance excessively compared with older infants or adults, it gives rise to signs of both systemic and pulmonary venous congestions.
Also in this chapter we discuss about the management options, drugs, and heart and heart-lung transplantation. In these patients heart transplant recipients have a mean survival of 11 years, like patients with other forms of cardiac disease; but patients who have had Fontan surgery are likely to have worse outcomes. Also about one third of heart-lung transplants are done for CHD. Survival is about 50 % at 3 years, near 20 % in 10 years after heart-lung transplantation, and better in patients with the Eisenmenger syndrome.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Francis GS. Pathophysiology of chronic heart failure. Am J Med. 2001;110(Suppl 7A):37S–46.
The SOLVD Investigators. Effect of enalapril on mortality and the development of heart failure in symptomatic patients with reduced left ventricular ejection fractions. N Engl J Med. 1992;327:685–91.
McMurray JJ, Stewart S. Epidemiology, etiology and prognosis of heart failure. Heart. 2000;83:596–602.
McDonagh TA. Asymptomatic left ventricular dysfunction in the community. Curr Cardiol Rep. 2000;2:470–4.
Metra M, Nodari S, D’Aloia A, et al. Effects of neurohormonal antagonism on symptoms and quality-of-life in heart failure. Eur Heart J. 1919;1998:B25–35.
Fredriksen PM, Veldtman G, Hechter S, et al. Aerobic capacity in adults with various congenital heart diseases. Am J Cardiol. 2001;87:310–4.
Chua TP, Ponikowski P, Harrington D, et al. Clinical correlates and prognostic significance of the ventilatory response to exercise in chronic heart failure. J Am Coll Cardiol. 1997;29(7):1585–90.
Coats AJ. The “muscle hypothesis” of chronic heart failure. J Mol Cell Cardiol. 1996;28:2255–62.
Warnes CA, Williams RG, Thomas M, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. Circulation. 2008;52(23):e143-e263.
The CONSENSUS Trial Study Group. Effects of enalapril on mortality in severe congestive heart failure. Results of the Cooperative North Scandinavian Enalapril Survival Study (CONSENSUS). N Engl J Med. 1987;316:1429–35.
Hechter SJ, Fredriksen PM, Liu P, et al. Angiotensin-converting enzyme inhibitors in adults after the Mustard procedure. Am J Cardiol. 2001;87:660–3.
Lester SJ, Mc Elhinney DB, Viloria E, et al. Effects of losartan in patients with a systemically functioning morphologic right ventricle after atrial repair of transposition of the great arteries. Am J Cardiol. 2001;88:1314–6.
Hopkins WE, Kelly DP. Angiotensin-converting enzyme inhibitors in adults with cyanotic congenital heart disease. Am J Cardiol. 1996;77:439–40.
Laer S, Mir TS, Behn F, et al. Carvedilol therapy in pediatric patients with congestive heart failure: a study investigating clinical and pharmacokinetic parameters. Am Heart J. 2002;143:916–22.
Shaddy RE. Beta-blocker therapy in young children with congestive heart failure under consideration for heart transplantation. Am Heart J. 1998;136:19–21.
Shaddy RE, Tani LY, Gidding SS, et al. Beta-blocker treatment of dilated cardiomyopathy with congestive heart failure in children: a multi-institutional experience. J Heart Lung Transplant. 1999;18:269–74.
Troughton RW, Prior DL, Pereira JJ, et al. Plasma B-type natriuretic peptide levels in systolic heart failure: importance of left ventricular diastolic function and right ventricular systolic function. J Am Coll Cardiol. 2004;43:416–22.
Hunt SA, Abraham WT, Chin MH. ACC/AHA 2005 guideline update for the diagnosis and management of chronic heart failure in the adult: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to update the 2001 Guidelines for the Evaluation and Management of Heart Failure). J Am Coll Cardiol. 2005;46:e1–82. 202.
Pignatelli RH, Mc Mahon CJ, Chung T, et al. Role of echocardiography versus MRI for the diagnosis of congenital heart disease. Curr Opin Cardiol. 2003;18:357–65. 203.
Tulevski II, van der Wall EE, Groenink M, et al. Usefulness of magnetic resonance imaging dobutamine stress in asymptomatic and minimally symptomatic patients with decreased cardiac reserve from congenital heart disease (complete and corrected transposition of the great arteries and subpulmonic obstruction). Am J Cardiol. 2002;89:1077–81.
Williams RV, Ritter S, Tani LY, et al. Quantitative assessment of ventricular function in children with single ventricles using the Doppler myocardial performance index. Am J Cardiol. 2000;86:1106–10.
Vanderheyden M, Goethals M, Verstreken S, et al. Wall stress modulates brain natriuretic peptide production in pressure overload cardiomyopathy. J Am Coll Cardiol. 2004;44:2349–54.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer-Verlag London
About this chapter
Cite this chapter
Alizadehasl, A. (2014). Congestive Heart Failure in Adults with Congenital Heart Disease. In: Sadeghpour, A., Kyavar, M., Alizadehasl, A. (eds) Comprehensive Approach to Adult Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-4471-6383-1_7
Download citation
DOI: https://doi.org/10.1007/978-1-4471-6383-1_7
Published:
Publisher Name: Springer, London
Print ISBN: 978-1-4471-6382-4
Online ISBN: 978-1-4471-6383-1
eBook Packages: MedicineMedicine (R0)