Abstract 12851: Severe Right and Left-Sided Multi-Valvular Carcinoid Heart Disease Associated With Ovarian Carcinoid Tumor

Abstract

Carcinoid tumors are neuroendocrine neoplasms that secrete vasoactive substances, primarily serotonin, and have an incidence of approximately 2 in 100,000 people. Up to 60% of carcinoid tumors result in carcinoid heart disease (CHD), and unlike other carcinoid tumors, ovarian carcinoid tumors can involve the heart without liver metastasis. By bypassing the liver, ovarian carcinoid tumors can result in CHD in the absence of liver metastasis. We present a rare case of a 40-year-old female who presented in cardiogenic shock requiring inotropic support with echocardiographic evidence of preserved left ventricular systolic function (LVEF 50-55%) and multiple retracted leaflets that exhibited poor coaptation resulting in severe mitral regurgitation, torrential tricuspid regurgitation, and moderate-to-severe aortic regurgitation. Her left heart catheterization revealed non-obstructive coronary artery disease. A non-contrast CT torso revealed an isolated large septated left ovarian cystic mass (7.1x5.2 x7.1cm) without any other mass or lesion. In the setting of an elevated CA-125 (536units/mL, normal 0-35units/mL) and 24-hour urinary 5-HIAA (12.1mg, normal 2-9mg) with a normal CEA, CHD was suspected as the most likely diagnosis given the isolated ovarian mass and severe valvular disease. She underwent triple valve surgery in which valvular pathology revealed myxoid degeneration. Her post-operative course was complicated by multi-organ failure requiring multiple inotropes and V-A ECMO shortly thereafter, but the patient unfortunately passed away. While there are a few documented cases of CHD from ovarian carcinoid (less than 0.5% of cases) and left-sided CHD (less than 10% of cases), this is the first documented case, to our knowledge, describing severe right and left-sided CHD associated with a suspected ovarian carcinoid tumor.