ArticleJACC. Case reports2026
Quadrivalvular Carcinoid Heart Disease Via Patent Foramen Ovale as Initial Presentation of Metastatic Neuroendocrine Tumor.
Article in JACC. Case reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundCarcinoid heart disease typically affects right-sided valves. Quadrivalvular involvement is rare. CASE SUMMARY: A 52-year-old woman presented with florid heart failure. Multimodality imaging demonstrated severe multivalvular regurgitation, and a large patent foramen ovale. She underwent bioprosthetic triple valve replacement with atrial septal defect patch closure. Histology revealed fibromyxoid degeneration and postoperative evaluation confirmed a metastatic small bowel neuroendocrine tumor. Following somatostatin analog therapy as a bridge, she underwent definitive oncologic resection with complete biochemical and radiologic response. DISCUSSION: This case illustrates the indolent course of carcinoid syndrome and the dramatic presentation of severe undiagnosed carcinoid heart disease. Atypical multivalvular disease should prompt carcinoid screening, as preoperative recognition is essential to avoid life-threatening carcinoid crisis. TAKE-HOME MESSAGES: In patients with unexplained multivalvular regurgitation and suspected neuroendocrine tumor, evaluate for right-to-left shunting and carcinoid valvulopathy to enable timely, multidisciplinary management.
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