Heart Failure, Cardiomyopathy, and Cardiac Transplantation
Heart failure and cardiomyopathy
Pediatric heart failure often reflects congenital heart disease, myocarditis, or cardiomyopathy rather than adult ischemic disease. Dilated cardiomyopathy causes systolic dysfunction and remodeling; hypertrophic cardiomyopathy causes diastolic dysfunction and dynamic outflow obstruction; restrictive cardiomyopathy causes elevated filling pressures with relatively preserved ejection fraction. Medical therapy includes diuretics for congestion, beta-blockers and afterload reduction in selected dilated cardiomyopathy, and careful volume management in hypertrophic disease. Mechanical support and transplantation are options for refractory failure.
Transplantation
Transplant indications include end-stage cardiomyopathy and complex congenital heart disease not amenable to repair. Rejection surveillance uses echocardiography, biomarkers, and biopsy; cardiac allograft vasculopathy is a major late complication. Immunosuppression requires adherence and infection surveillance.
Traps
Do not extrapolate adult heart-failure trials directly to children. In obstructive hypertrophic cardiomyopathy, avoid dehydration and vasodilators that worsen the gradient; murmurs become louder with Valsalva and standing. A normal ejection fraction does not exclude restrictive or hypertrophic disease. Myocarditis can recover fully, so early transplant evaluation must be balanced against potential recovery.
Worked example
A teenager has exertional syncope, a systolic murmur that increases with Valsalva, and a family history of sudden death. Echocardiography shows asymmetric septal hypertrophy. Advise avoidance of competitive sports and dehydration, start beta-blockade, and screen family members. Rule of thumb: in hypertrophic cardiomyopathy, the murmur gets louder with maneuvers that reduce preload.