Cardiac Papillary Fibroelastoma Originating from Pulmonary Vein A Case Report

• Published in: Angiology Vol. 53; no. 6; pp. 745 - 748
• Main Authors: Gowda, Ramesh M., Khan, Ijaz A., Mehta, Nirav J., Gowda, Mamatha R., Gropen, Toby I., Dogan, Ozgen M., Vasavada, Balendu C., Sacchi, Terrence J.
• Format: Journal Article
• Language: English
• Published: Thousand Oaks, CA SAGE Publications 01.11.2002; Westminster; SAGE PUBLICATIONS, INC
• Subjects: Aged; Biological and medical sciences; Cardiology. Vascular system; Echocardiography, Transesophageal; Fibroma - complications; Fibroma - diagnostic imaging; Fibroma - surgery; Heart; Heart Neoplasms - complications; Heart Neoplasms - diagnostic imaging; Heart Neoplasms - surgery; Humans; Male; Medical sciences; Pulmonary Veins; Stroke - etiology; Tumors of the heart; Sonography; Human; Nervous system diseases; Stroke; Echocardiography; Pathophysiology; Cerebral embolism; Endocardium; Cardiovascular disease; Exploration; Review; Thrombosis; Fibroelastosis; Cerebral disorder; Vascular disease; Case study; Treatment; Heart disease; Surgery; Endocardial disease; Central nervous system disease; Complication; Diagnosis; Cerebrovascular disease
• ISSN: 0003-3197; 1940-1574
• DOI: 10.1177/000331970205300619

Cardiac papillary fibroelastoma is a primary cardiac neoplasm that typically affects the cardiac valves, mainly the aortic and mitral valves, and very rarely the endocardium of cardiac chambers. Cardiac papillary fibroelastoma is rarely diagnosed during life, as the majority are incidental findings at autopsy, but with the advent of echocardiography, it is being increasingly recognized. Although the tumor is usually small and histologically benign, it may have a malignant propensity for life-threatening complications, such as a cerebrovascular accident, myocardial ischemia or infarction, or sudden death. The patient reported here presented with an embolic stroke from a thrombus on the surface of a left atrial papillary fibroelastoma. The papillary fibroelastoma was originating from the lower portion of the left inferior pulmonary vein and was protruding into the left atrial cavity. Papillary fibroelastoma originating from the pulmonary veins has not been reported before. The tumor was successfully removed by intra operative transesophageal echocardiography-guided cardiac surgery. Grossly, the surface of the tumor was smooth and translucent. The gelatinous membrane on the surface tore easily, and soft papillary tumor with multiple fronds was visible. Histology confirmed the mass was a papillary fibroelastoma. Postoperative recovery was uneventful. Follow-up transthoracic echocardiogram revealed no residual or recurrence of tumor. The patient was in excellent health at 2-year follow-up. The case is described and the clinical characteristics of cardiac papillary fibroelastoma are reviewed.