TY - CHAP M1 - Book, Section TI - Cardiac Transplantation A1 - Hasan, Ayesha A2 - Baliga, R. R. A2 - Abraham, William T. Y1 - 2018 N1 - T2 - Color Atlas and Synopsis of Heart Failure AB - 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accesscardiology.mhmedical.com/content.aspx?aid=1161018917 ER -