A Case of Carcinoid Syndrome with Carcinoid Valvular Heart Disease Treated Successfully

Abstract

A 39-year-old lady presented in early 2011 with Carcinoid syndrome due to Ileal Carcinoid with liver metastases and Carcinoid valvular heart disease with Right heart failure. Liver resection combined with ethanol injection of Liver metastases was carried out after reduction of Central Venous Pressure by an initial Tricuspid valve replacement (TVR), in a staged manner, with resolution of symptoms. This case highlights the necessity of TVR prior to major Liver resection for Liver metastases in Carcinoid heart disease with Tricuspid regurgitation as well as the value of treating Carcinoid heart disease aggressively.Key words: Carcinoid heart disease; Tricuspid valve replacement; Liver resection. Clinical ReportA 39-year-old lady presented in early 2011 with history of postprandial flushing attacks of 6 years duration, pedal oedema and puffiness of face with persistent dry cough of 2 months duration. Elevated Jugular Venous Pressure (JVP), bilateral pitting pedal oedema and nontender hepatomegaly were noted on clinical examination. Ultrasound abdomen showed an 8 × 8 cm mass lesion with mixed echogenicity in the Left lobe, liver. Contrast Enhanced Computerized Tomography (CECT) of abdomen revealed a mixed density mass lesion with irregular contrast enhancement in the Left lobe of liver with multiple enhancing lesions in the right lobe, liver as well as an enhancing lesion in the terminal ileum. Carcinoid syndrome due to Ileal carcinoid with liver metastases and carcinoid valvular heart disease with right heart failure was diagnosed. 24 hours urinary 5 hydroxy indole acetic acid (24 hr urinary 5HIAA) estimation showed a very high value of 153 mg/day. Echocardiography revealed tricuspid stenosis with regurgitation, mild pulmonary artery hypertension and Right heart failure (Figure 1). At the multidisciplinary meeting, further evaluation by Cardiology and Cardiothoracic Surgery regarding the status of pulmonary artery hypertension and the feasibility of tricuspid valve replacement (TVR) was discussed as TVR is a rarely performed procedure with operative mortality in the range of 14% to 17% [1,2]. Further, the risk of TVR in the presence of Carcinoid syndrome was discussed. After detailed evaluation, it was decided to suggest TVR considering the favorable tumor biology and the fact that the resultant drop in Central Venous Pressure (CVP) would make way for a safe liver resection. It is well known that liver resection in the presence of high CVP carries higher morbidity and mortality [3].However, the relatives wanted time to think over, arrange finances and take a decision on the treatment plan. She was maintained on monthly Depot Octreotide. Meanwhile as she developed persistent pain in the lower abdomen with features of subacute bowel obstruction within a couple of months, a palliative resection of the primary in the ileum was carried out under regional anesthesia.