Hedinger Syndrome: A Rare Cardiac Manifestation of Carcinoid Syndrome

Abstract

Carcinoid syndrome can cause desmoplastic reactions to nearby tissues. When it involves the heart, it causes carcinoid heart disease (CHD) or Hedinger syndrome and usually involves the right-sided heart valves, causing tricuspid insufficiency and pulmonary stenosis (TIPS) and eventually leading to right-sided heart failure. The management of patients with CHD is complex, as both the systemic malignant disease and the heart involvement have to be addressed. Its prompt diagnosis and early treatment is paramount as CHD is associated with increased morbidity and mortality. A 61-year-old Caucasian male with a recently diagnosed metastatic neuroendocrine tumor presented to the heart failure clinic with decompensated heart failure, anasarca, flushing and diarrhea. This case highlights the common clinical features of carcinoid syndrome, its cardiac manifestations and the pathophysiology underlying the manifestations and treatment decisions that involve addressing both systemic and cardiac manifestations.

Keywords: carcinoid heart disease; gastrointestinal carcinoid tumor; hedinger syndrome; right-sided heart failure; tricuspid valve regurgitation.