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Carcinoid syndrome can cause significant heart valve issues, specifically affecting the tricuspid valve with stenosis and insufficiency. Characteristic hourglass-shaped narrowing of the pulmonary artery and outflow tract was observed in affected patients.
Area of Science:
- Cardiology
- Oncology
- Pathology
Background:
- Carcinoid syndrome, a rare condition, can lead to cardiac complications due to neuroendocrine tumor-secreted hormones.
- Cardiac involvement in carcinoid syndrome often affects the right heart valves.
Observation:
- Review of cineangiocardiograms from two patients with carcinoid syndrome and cardiac involvement.
- Pathology specimens from one patient provided detailed gross and microscopic examination.
Findings:
- Both patients presented with tricuspid valvar stenosis and insufficiency.
- A characteristic hourglass-shaped narrowing was noted in the pulmonic valve annulus, proximal pulmonary artery, and subvalvar right ventricular outflow tract.
Implications:
- The observed "hourglass" deformity may be a distinctive feature of carcinoid heart disease.
- Understanding these specific valvular and outflow tract changes is crucial for diagnosis and management.
Abstract:
Cineangiocardiograms from 2 patients with carcinoid syndrome and cardiac involvement were reviewed. Gross and microscopic pathology specimens were available for 1 case. Both patients exhibited tricuspid valvar stenosis and insufficiency. The area of the pulmonic valve was similar in both with narrowing of the annulus and tapering toward the valve of the proximal pulmonary artery and the subvalvar right ventricular outflow tract, producing an hourglass appearance, which the authors feel is characteristic of this disease.