Congestive heart failure in adults with congenital heart disease

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.