87 A gynaecological cause of right heart failure

Abstract

Abstract Introduction A 68-year-old lady complained of pelvic pain associated with progressive abdominal distension over few months. Physical examination revealed ascites, smooth hepatomegaly and lower limb edema. CT scan of abdomen and pelvis showed a 4 cm x 5 cm right ovarian mass without other intraabdominal lesion. Surgical excision of the ovarian mass was performed with clear resection margin. The ascitic fluid was negative for malignant cells. However, the patient had persistent ascites and lower limb edema after surgery. The gynaecologist suspected that she might have cardiac problems. Procedure Transthoracic echocardiogram was performed. The right atrium and ventricle were markedly enlarged. The tricuspid valve leaflets appeared retracted and sclerotic, with failed coaptation during systole (Figure A), severe tricuspid regurgitation (Figure B) and marked dilatation of inferior vena cava without respiratory variation. The pulmomary valve was not well visualized on 2D images. Continuous wave Doppler across pulmonary valve demonstrated short pressure half time 66 msec and premature termination of diastolic flow (Figure C), suggestive of severe pulmonary regurgitation. There was no intracardiac shunt. Left sided valves were unremarkable. Additional tranesophageal echocardiogram allowed better assessment of pulmonary valve, which revealed one of the cusps to be thickened, retracted and immobile on real time 3D images (Figure D, arrow). Discussion The ovarian mass was confirmed to be a carcinoid tumour. Due to its direct drainage into systemic venous circulation the vasoactive substances (serotonin, prostaglandin etc) reach the right heart directly without being metabolized by liver, resulting in plaque like deposit of fibrous tissue on the endocardium and valve surfaces, leading to classical appearance of thickened, retracted and immobile leaflet and subsequent valvular regurgitation or, less commonly, stenosis. Left-sided valve disease is less common, and may occur in cases of bronchial carcinoid or in presence of atrial right to left shunt (e.g. PFO). Urinary 5-hydroxyindoleacetic acid (5-HIAA) is a useful non-invasive diagnostic test. Carcinoid heart disease usually does not regress even the tumour is treated, and valve replacement is the only definitive curative treatment. This lady received tricuspid and pulmonary valve replacement with subsequent symptom resolution and chest X-ray showed marked reduction of cardiomegaly (Figure E). Conclusion A baseline echocardiogram is recommended for assessment valvular involvement in patient with carcinoid tumour, as the cardiac symptoms may be mistaken to be related to regional spread of tumour. Pulmonary valve is very often the "forgotten" valve in many Echo studies. However, it is crucial to have detailed assessment (with aids by 3D images or TEE) due to its implication to the surgical treatment planning. Abstract 87 Figure.