Carcinoid Heart Disease and a Primary Ovarian Carcinoid Tumor

Introduction

Primary ovarian carcinoid tumors account for less than 0.5% of all carcinoid tumors [1]. Patients with primary ovarian carcinoid tumors may develop carcinoid heart disease by release of bioactive amines directly into the inferior vena cava or renal vein. Carcinoid syndrome is characterized by flushing, secretory diarrhea and bronchospasm. Half of patients with carcinoid syndrome exhibit carcinoid heart disease with subsequent right-sided valvular dysfunction and right ventricular failure [1]. Valve replacement is performed to relieve symptoms and improve survival [2]. The authors describe a procedure performed for a 55-year-old woman who presented with carcinoid heart disease from a primary ovarian carcinoid tumor. She underwent pulmonary and tricuspid valve replacements and patch enlargement of the right ventricular outflow tract.

Case Report

A 55-year-old woman presented with worsening dyspnea on exertion, flushing, and lower extremity edema. Auscultation revealed a holosystolic murmur at the left lower parasternal border. Chromogranin A and 5-hydroxyindoleacetic acid levels were elevated. Transesophageal echocardiography demonstrated severe tricuspid and pulmonary regurgitation. The tricuspid valve leaflets were thickened and severely restricted with failure of coaptation and visible plaques. There was also a mixed pattern of pulmonary regurgitation and stenosis. Computed tomography and octreotide scans demonstrated an 8.2 x 7 x 4.3 cm solid enhancing mass emanating from the right pelvis. Carcinoid heart disease arising from a primary ovarian carcinoid tumor was suspected. Because of the severity of the patient’s symptoms, a decision was made to replace her tricuspid and pulmonary valves.

Intravenous infusion of octreotide was initiated the day of the operation and continued postoperatively for 48 hours. The pulmonary valve was approached through a longitudinal incision made across the valve annulus onto the outflow portion of the right ventricle. White plaques were adherent to the fibrotic pulmonary valve leaflets. Chordae were also fused and deformed. A patch enlargement of the right ventricular outflow tract was performed to accommodate an adequately sized pericardial valve (Edwards Lifesciences, Irvine, CA, USA). Through a right atriotomy, the tricuspid valve was exposed. Fibrosis and fusion of the tricuspid valve leaflets and subvalvular apparatus were noted. The leaflets were excised and the tricuspid valve was replaced with a porcine valve (Medtronic, Minneapolis, MN, USA). The patient was discharged on postoperative day 10 on monthly long-acting octreotide therapy.

The patient underwent total laparoscopic hysterectomy and bilateral salpingo-oophorectomy three months later. Pathology revealed a well-differentiated neuroendocrine tumor of the right ovary. At the time of writing, the patient was alive without evidence of disease and normal tumor marker levels.

Comment

The incidence of valvular heart disease approaches 30% in cases of primary ovarian carcinoid tumors. Elevated bioactive amine levels released from carcinoid tumors promote severe endocardial plaque formation that most commonly result in right-sided valve destruction and ensuing right-sided heart failure [1].  Right ventricular failure is a major cause of morbidity and mortality in patients with carcinoid heart disease [3-5]. It has been recommended that patients with even mild symptoms undergo valve surgery to prevent progression of right ventricular dysfunction. Although operative mortality approaches 20%, valve replacement results in improved midterm outcomes [2]. Traditionally, mechanical prostheses have been favored secondary to potential valve degeneration from high circulating serotonin [2, 3]. Nonetheless, contemporary data suggest bioprosthetic valves are durable and avoid the higher risks of thrombosis and bleeding associated with mechanical prostheses [2].

Primary ovarian carcinoid tumor with carcinoid heart disease should be considered in a symptomatic female patient with a pelvic tumor, isolated right-sided valve disease, and elevated tumor markers. Surgery is necessary to provide long-term functional improvement and prolonged survival.

References

1. Chaowalit N, Connolly HM, Schaff HV, Webb MJ, Pellikka PA. Carcinoid heart disease associated with primary ovarian carcinoid tumor. Am J Cardiol. 2004;93(10):1314-1315.
2. Bhattacharyya S, Raja SG, Toumpanakis C, Caplin ME, Dreyfus GD, Davar J. Outcomes, risks and complications of cardiac surgery for carcinoid heart disease. Eur J Cardiothorac Surg. 2011;40(1):168-172.
3. Castillo JG, Milla F, Adams DH. Surgical management of carcinoid heart valve disease. Semin Thorac Cardiovasc Surg. 2012;24(4):254-260.
4. Mokhles P, van Herwerden LA, de Jong PL, et al. Carcinoid heart disease: outcomes after surgical valve replacement. Eur J Cardiothorac Surg. 2011;41(6):1278-1283.
5. Pellikka PA, Tajik AJ, Khandheria BK, et al. Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 1993;87(4):1188-1196.