A 54-year-old African American woman with nonischemic cardiomyopathy, that is, LMNA-related familial cardiomyopathy, presented for advanced therapy evaluation based on recurrent admissions for volume overload and intravenous diuresis. She was diagnosed over 10 years ago, but ejection fraction declined to 15% on goal-directed medical therapy with carvedilol, valsartan, spironolactone, hydralazine, and nitrates. She was a nonresponder to cardiac resynchronization therapy despite underlying QRS of 140 ms. Hypotension resulted in reducing medication doses during the last few hospitalizations. Primary transplantation was discussed (blood type B), and cardiopulmonary exercise testing revealed a peak VO2 of 13.1 mL/kg/min, 59% predicted with Ve/VCO2 30. Right heart catheterization found severe pulmonary hypertension with pulmonary capillary wedge pressure 31 mm Hg, PA 66/44, mean 52 mm Hg, PA sat 38%, and CI 1.5 L/min/m2 by Fick. She was started on milrinone for inotrope support. However, her pulmonary HTN did not improve significantly despite diuresis, and clinical status was tenuous. Her PVR declined to 4 WU with diuresis despite improvement in cardiac output on milrinone. She was implanted with a Heartmate II left ventricular assist device (LVAD) with the goal of bridging to transplantation until PVR improved. Because of progressive right heart failure and deteriorating renal function after LVAD, percutaneous temporary right heart support helped improve her clinical status and renal function without the need for dialysis. However, she required milrinone as a chronic infusion with her LVAD for RV support. She was listed as a 1A for VAD complication involving right heart failure. Pulmonary hypertension reversed to normal values on LVAD, and PVR was 1.8 WU. She was successfully bridged to heart transplantation approximately 14 months after LVAD with creatinine level of 1.5 mg/dL, and she had an uncomplicated first year post-heart transplantation.
Therapy for refractory heart failure (HF) has advanced both pharmacologically and nonpharmacologically in the past decades. Despite the survival benefit of defibrillators, cardiac resynchronization devices, and medical therapy, morbidity and mortality associated with advanced HF is excessive, up to 50% 5-year mortality and greater than 50% 1-year mortality for those considered end-stage. Such challenges are addressed with cardiac transplantation where the median survival ranges from 10 to 13 years based upon a patient’s surviving the first year.1 Replacement of the heart is standard treatment for select patients with refractory symptoms, whereas mechanical support is the alternative for those who are either not candidates or require more immediate support. The early field of transplantation was plagued by poor survival and refining surgical techniques. Despite the first successful human heart transplant in South Africa in 1967 and the first U.S. case in 1968, survival was limited until the introduction of cyclosporine in the 1980s2 and continues to improve with refinements in recipient and donor selection, donor management, immunosuppression, and treatment of comorbidities. Estimated annual transplant rates are >4000 cases with the limiting factor being donor availability.