Elsevier

Mayo Clinic Proceedings

Volume 77, Issue 2, February 2002, Pages 139-147
Mayo Clinic Proceedings

Original Article
Surgical Pathology of Carcinoid Heart Disease: A Study of 139 Valves From 75 Patients Spanning 20 Years

https://doi.org/10.4065/77.2.139Get rights and content

Objective

To quantitate the pathologic features of carcinoid plaques in a relatively large number of surgical specimens from a single institution.

Patients and Methods

Medical records, operative reports, and surgical specimens were reviewed from all patients with carcinoid heart disease who underwent cardiac valvular surgery at Mayo Clinic, Rochester, Minn, between 1980 and 2000.

Results

The study group included 75 patients (45 men, 30 women) who ranged in age from 26 to 78 years (mean, 59 years). From these 75 patients, 139 valves had been excised surgically (73 tricuspid, 55 pulmonary, 6 mitral, 5 aortic). Pure regurgitation was the most common dysfunctional state of the tricuspid valve (80% [60/75]), mitral valve (97% [32/33]), and aortic valve (96% [23/24]). The pulmonary valve was more often both stenotic and insufficient (52% [37/71]) than purely regurgitant (30% [21/71]). In all cases, valve dysfunction was attributed to the presence of carcinoid plaques, which caused both thickening and retraction. Thickening was the result of both cellular proliferation and deposition of extracellular matrix. Proliferation of myofibroblasts was observed in all plaques and was mild in 49% (68/139) and moderate or severe in 51% (71/139). Extracellular matrix included collagen (in 99% of the 139 valves), myxoid ground substance (98% [136/139]), and elastin (20% [28/139]). Carcinoid plaques were also involved by neovascularization (94% [131/139]), chronic inflammation (94% [131/139]), and mast cell infiltration (64% [89/139]). Severe thickening was attributable primarily to collagen deposition in tricuspid valves and to myofibroblast proliferation and myxoid matrix in pulmonary valves.

Conclusions

Among patients undergoing valvular surgery for carcinoid heart disease, tricuspid and pulmonary valves represented 92% of the excised valves (128/139). Although numerous cellular and extracellular features were common to the carcinoid plaques, variability in their relative expression produced appreciable differences in the histologic appearance among the plaques.

Section snippets

PATIENTS AND METHODS

The protocol for this study was approved by the Mayo Foundation Institutional Review Board. Medical records, operative reports, and surgical specimens were reviewed from all patients who had undergone cardiac surgery on native cardiac valves for carcinoid heart disease at Mayo Clinic, Rochester, Minn, during the 20 years from July 1, 1980, to June 30, 2000.

For each patient, age and sex were recorded from the medical history, as were the dates of diagnosis of carcinoid syndrome, carcinoid heart

RESULTS

Among 79 patients who underwent valvular surgery for carcinoid heart disease, 75 had adequate surgical specimens. The 75 operations included procedures on 156 valves and excision of tissue from 139 (73 tricuspid, 55 pulmonary, 6 mitral, and 5 aortic). For 17 valves (13 pulmonary, 2 tricuspid, 1 mitral, and 1 aortic), repair was accomplished without removal of tissue. Thus, the study group included 139 valves from 75 patients. Some cases have been reported previously as part of larger series

DISCUSSION

Most of the information concerning the valvular pathology of carcinoid heart disease is derived from 40 cases reported in 7 autopsy series (Table 3),6, 7, 8, 9, 10, 11, 12 of which 21 cases appeared in 3 publications from the National Institutes of Health.9, 11, 12 Numerous case reports and a surgical series of 3 patients have also provided insights about the disease process.13 The current investigation uniquely reviews and quantitates the surgical pathology of carcinoid heart disease in 139

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