1640 Pulmonary valve in carcinoid disease: be suspicious of functional assessment

Abstract

Abstract Carcinoid heart disease occurs in 50% of patients with carcinoid syndrome, especially on serotonin-secreting neuroendocrine tumors (NET) [1, 2]. Carcinoid heart disease"s typical findings are carcinoid plaques, composed of smooth muscle, myofibroblasts and endothelium. These plaques, most frequently localized on the right side valvular cusps and leaflets, produce fixation and retraction, causing regurgitation and/or stenosis [3, 4, 5]. Left-sided heart disease is rare (<10%), as serotonin is purified in the pulmonary circulation [3, 6, 7]. We present the case of a 67 year old female patient with previous history of right ovarian NET. She presented with dyspnea (II-III NYHA functional class), and slight edema in lower limbs. She denied symptoms of carcinoid syndrome before or after the ovarian surgery. Examination showed an increased jugular venous pulse. Transthoracic echocardiography (TTE) showed severely dilated right cavities and a rigid, immobile tricuspid valve, with massive tricuspid regurgitation (Panel A, 1). Anatomy of pulmonary valve was not properly visualized but jet area of pulmonary regurgitation was small (Panel A, 2). Left cavities and valves were intact. As TTE was not anatomically conclusive, a cardiac magnetic resonance (CMR) and a cardiac computed tomography (CCT) were performed to assess the carcinoid involvement of pulmonary valve. CMR showed severely dilated right ventricle with mild impairment of systolic function (Panel A, 3). Based on phase contrast imaging, pulmonary regurgitation fraction was 14%, suggestive of a mild grade. However, CCT showed a diffuse thickening of the pulmonary valve, with complete opening during diastole (Panel A, 4, arrowhead). Finally, the patient underwent replacement of tricuspid valve and pulmonary valve for biological prosthesis without complications. Our case is remarkable because it highlights the limitations of the functional assessment of pulmonary regurgitation in the presence of a concomitant massive tricuspid valve regurgitation, since the rapid equalization of pressure between pulmonary artery and right ventricle reduces the expression of pulmonary regurgitation. For these reason, the anatomic assessment of the pulmonary valve is mandatory to stage the involvement of pulmonary valve in carcinoid disease. Abstract 1640 Figure. Panel A