Outcomes of Surgical Treatment in Carcinoid Heart Disease
What are the early and late outcomes after valve surgery for carcinoid valvular heart disease (CVHD)?
This is a retrospective review spanning a 27-year period at a single institution. Records from 195 patients with CVHD were analyzed and characterized by date and type of surgery; perioperative outcome and long-term survival were calculated. Patients offered valve surgery had symptomatic severe CVHD, progressive right-sided cardiac chamber enlargement and dysfunction, or hepatic involvement requiring hepatic surgery in the presence of elevated right atrial pressure.
Tricuspid valve replacement was undertaken in all patients, and a bioprosthesis was used in 159 patients. A concurrent pulmonary valve operation was performed in 157, which included 50 pulmonary valvectomies, and 107 pulmonary valve replacements. Mitral valve repair was required in five patients and replacement in 16, and aortic valve procedures in 15. The mean time from diagnosis of carcinoid tumor to valve operation was 4.7 ± 6 years and 125 patients were New York Heart Association (NYHA) functional class III or IV. Octriatide therapy prior to surgery was used in 184 patients (94%). Severe tricuspid regurgitation was present in all. Over the 27-year period, operative mortality was 10%, but declined steadily for the era before 1990 to the most recent decade of experience. For the 97 patients operated since 2000, there were eight perioperative deaths (6%). One-, 5-, and 10-year survival was 69%, 35%, and 24%, respectively. Multivariable analysis identified older age, preoperative chemotherapy, and preoperative tobacco use as the only significant predictors of mortality during follow-up. On multivariable analysis, only era of operation and need for intravenous loop diuretics predicted perioperative mortality. Over the follow-up period, 17 patients required reoperation, including replacement of tricuspid bioprosthesis in eight patients. In only one instance was the indication for replacement carcinoid involvement of a bioprosthetic valve, and in others, valve thrombosis was the indication. For patients in NYHA class III or IV, 69 patients (75%) had symptomatic improvement and 118 (76%) were NYHA class I or II at follow-up. Long-term survival was not different between the patients operated on with severe symptoms versus those who were operated on in a presymptomatic state.
Symptomatic and survival benefit is noted in most patients with CVHD following valve replacement, which can be performed with acceptable perioperative mortality.
This is the largest collection of patients with CVHD undergoing surgery reported to date. Carcinoid valve disease is a difficult management problem as many of the signs and symptoms of the valve disease are mimicked by the metastatic carcinoid disease itself, including nonspecific symptoms, edema, and ascites. While neither prospective nor randomized, this study demonstrates a symptomatic and survival benefit for valve replacement when compared to either historical or concurrent controls groups. It should be noted that while resulting in symptomatic improvement and improved survival compared to historical controls, 1-, 5-, and 10-year survival were only 69%, 35% and 24%, respectively. Based on these observations, patients with symptoms related to CVHD or evidence of progressive right ventricular enlargement and dysfunction should be offered valve surgery (in a high-volume experienced center).
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