End-Stage Diastolic and Systolic Heart Failure: Evaluation and Timing of Heart Transplantation

End-stage hypertrophic cardiomyopathy occurs in an estimated 3–15% of patients and can present as either systolic or diastolic dysfunction. Risk factors for developing end-stage disease include a family history of end-stage disease, younger age at initial diagnosis, increased wall thickness, persistent arrhythmias, and high scar burden on MRI. The classic form of adverse remodeling includes left ventricular cavity dilation with regression of hypertrophy, wall thinning, and decrease in ejection fraction. Standard medical therapy for systolic heart failure and consideration of prophylactic defibrillator is indicated when LVEF is less than 50%. Heart transplant is a viable option for patients with end-stage hypertrophic cardiomyopathy, including those with systolic heart failure, diastolic heart failure, or refractory arrhythmias. Strategies used to bridge patients to transplant include continuous inotropic infusion, left ventricular assist device, intra-aortic balloon pump, and in rare cases extracorporeal membrane oxygenation. Survival after heart transplantation for hypertrophic cardiomyopathy is equal to or better than survival for patients who have other types of cardiomyopathies.