CONGRESS ABSTRACT

Introduction Carcinoid heart disease (CHD) is a rare cause of right heart failure due to tricuspid regurgitation (TR) and pulmonary stenosis secondary to valve leaflets fibrosis. Primary ovarian neuroendocrine tumors are a lesser-known cause of CHD which can develop early since the ovarian veins drain into the inferior vena cava, bypassing the liver inactivation.    Case presentation We report the case of a 66 year old woman referred to our center to undergo right heart catheterization (RHC) for suspected pulmonary hypertension and severe TR with signs of RV pressure overload. Her medical history included arterial hypertension with no previous cardiac history. She was admitted for subacute dyspnea, right sided abdominal pain and diarrhea with elevated liver enzymes. Echocardiography showed normal left ventricular function (FE 55%), mildly dilated right ventricle (RV) with normal function (TAPSE 21), severe TR and a dilated pulmonary artery. A CT-scan revealed a right ovarian tumor; subsequent gynecological assessment, including elevated Ca125 (79.8 UI/ml), led to hysterectomy and right adnexectomy. During surgery she experienced a hypotensive episode with acute heart failure which was treated with inotropes and diuretics. Follow-up echocardiogram showed worsening RV function with severe dilation and signs of RV overload. A ventilation/perfusion lung scan ruled out CTEPH. RHC showed normal mPAP and wedge pressure with a mild trans-pulmonary gradient (16 mmHg) and mild increase in PVR (2.23 WU). ECG-gated CT of the thoracic aorta and great vessels ruled out anomalous venous return. Echocardiography confirmed the enlargement of the RV and atrium, massive TR (PISAr 12mm, EROA 0.80cm², Rvol 69ml) with thin valve leaflets hypomobility and retraction of the septal leaflet with pseudo prolapse of the anterior and posterior leaflets, and mild transpulmonary gradient (Gmax 26mmHg). Chromogranin A was normal (34 ng/mL). Tumor’s histopathology confirmed a low-grade neuroendocrine ovarian tumor and our suspicion of CHD. She was treated with low-dose diuretics and assessed for tricuspid valve repair.    Conclusion TR is a common finding in echocardiography but its association with pulmonary stenosis is rare, with potential tumor-related etiology. CHD is associated with increased morbidity and mortality due to complex management. Early valve replacement, along with tumor resection, should be considered even in patients with mild symptoms to improve outcomes.