Carcinoid Heart Disease in Patients With Bronchopulmonary Carcinoid

The prevalence of carcinoid heart disease (CaHD) in bronchopulmonary carcinoid and its relationship with left-sided valvular disease are unknown.

All patients with a pathologic diagnosis of bronchopulmonary carcinoid and echocardiography performed at our institution between 2001 and 2016 were retrospectively reviewed. Echocardiograms were reviewed for features of CaHD including valvular leaflet thickening and retraction with resulting regurgitation and/or stenosis.

Bronchopulmonary carcinoid was present in 185 patients (age 67 ± 13 years, 63% female). Carcinoid syndrome was present in 7.7% and liver metastases in 10%. Echocardiographic features of CaHD were present in just 2 (1%) patients. A 62-year-old woman underwent resection of stage 1A bronchopulmonary carcinoid without carcinoid syndrome and also received 7 months dexfenfluramine therapy. During 15-year follow-up, mitral regurgitation decreased and tricuspid regurgitation remained stable, a course more consistent with diet-drug-related valve disease than CaHD. A 71-year-old woman status post-resection of a grade 1 hilar carcinoid tumor with carcinoid syndrome, liver metastases, and elevated 5-hydroxyindole acetic acid had typical thickening and retraction of tricuspid and pulmonary valves with severe regurgitation. The aortic valve was mildly thickened and retracted with mild regurgitation. She underwent tricuspid and pulmonary valve replacement and closure of a patent foramen ovale. Pathologic examination confirmed CaHD.

CaHD occurs in less than 1% of patients with bronchopulmonary carcinoid. Bronchopulmonary carcinoid was associated with neither CaHD in the absence of liver metastases nor left-sided valve involvement in the absence of patent foramen ovale.