Bridge to Remission in Biventricular Cardiogenic Shock Associated with Endocrine Cardiomyopathy

Introduction Fluctuations in hormonal levels can alter cardiac myocytes at the cellular level, often resulting in deterioration of cardiac function. Endocrine cardiomyopathies have been described in the setting of hypo/hyperthyroidism, GH deficiency, acromegaly, and diabetes. However, it is rarely described in the setting of autoimmune polyglandular syndrome (APS). Case Report A 19 year-old male with a PMH of APS associated with hypothyroidism and adrenal insufficiency. He initially presented to an outside facility after a syncopal episode. Initial workup revealed a TSH of 70 and he was started on appropriate therapy. Soon after admission, he developed acute hypoxemic respiratory failure requiring urgent intubation. Due to severe pulmonary edema, an echo was obtained which revealed a severely decreased EF of 20%. An Impella CP® and Swan Ganz catheter were placed for management of cardiogenic shock. Due to persistent clinical deterioration with worsening renal and hepatic function, he underwent RPella® placement. Two days later he was transferred to our hospital for consideration of advanced Heart Failure therapies. Upon transfer, he was maintained on appropriate hormone therapies by endocrinology. An endomyocardial biopsy was unrevealing. We started low dose diuretics and noted marked improvement in right sided parameters with improving central venous pressures and resolving transaminitis. Repeat echo revealed normal right ventricular systolic function. The RPella® was then weaned off and electively removed. The Impella CP® device was weaned daily as per protocol. EF improved to 30% on repeat echo. Cardiac MRI was nonspecific. He was downgraded from ICU status after tolerance of comprehensive guideline based medical therapy. Summary Previously described is a 32 year old male with a history of APS who developed severe biventricular failure requiring inotropic support, counterpulsation, mechanical circulatory support (MCS), and ultimately cardiac transplantation. Though our patient presented to us in a highly compromised state, the use of MCS helped bridge the patient to a recovery in myocardial performance while undergoing hormonal replacement. To our knowledge, this is the first case of its nature and highlights the possibility of temporary MCS in APS-associated cardiomyopathy, and in turn, can be useful in other secondary cardiomyopathies.