Pulmonary Valve Papillary Fibroelastoma: A Rare Source of Pulmonary Embolism

TL;DR

A rare case of a benign heart tumor at the pulmonary valve caused life-threatening blood clots in the lungs, requiring careful treatment planning.

Contribution

Reports a rare case of pulmonary valve papillary fibroelastoma causing pulmonary embolism and highlights the need for individualized treatment.

Findings

Pulmonary valve PFE is an uncommon but serious cause of pulmonary embolism.

Multimodality imaging confirmed the diagnosis and guided treatment decisions.

Anticoagulation and heart failure management were prioritized over immediate surgery due to high surgical risk.

Abstract

Papillary fibroelastoma (PFE) is the second most common benign primary cardiac tumor, usually affecting left-sided valves. Pulmonary valve (PV) involvement is extremely rare, representing less than 8% of all PFEs. Although often asymptomatic, PFEs can present with embolic events. We report a 60-year-old man admitted with acute dyspnea, orthopnea, and lower-limb edema. Transthoracic echocardiography revealed severe left ventricular systolic dysfunction (ejection fraction: 15%), severe functional mitral regurgitation, and a mobile echogenic mass (1.0 × 0.8 cm) attached to the arterial surface of the PV consistent with a PFE. Chest computerized tomography (CT) confirmed bilateral segmental pulmonary emboli with infarctions and pulmonary artery dilation. Coronary angiography excluded ischemic disease. Given the patient’s new-onset heart failure with reduced ejection fraction and high operative risk, the heart team opted for initial anticoagulation and optimized heart failure therapy, postponing surgical resection until stabilization. PV PFE is a rare but potentially fatal cause of pulmonary embolism. Multimodality imaging is crucial for diagnosis, and individualized treatment is required to balance embolic risk and surgical mortality.

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