OBSOLETE: Papillary fibroelastoma of the heart

Papillary fibroelastoma (PFE) of the heart is a rare, benign primary cardiac tumor. This Orphanet entry (208600) is classified as OBSOLETE, meaning it has been retired or reclassified within the Orphanet nomenclature, potentially merged with a broader category of cardiac tumors. Papillary fibroelastomas are the most common valvular tumors of the heart and the second most common benign cardiac tumor after myxomas. They are small, avascular, papilloma-like growths typically found on cardiac valve surfaces, most frequently the aortic valve, though they can occur on any valve or endocardial surface. Papillary fibroelastomas primarily affect the cardiovascular system. Many patients are asymptomatic, with the tumor discovered incidentally during echocardiography or cardiac surgery. However, PFEs can cause serious complications including stroke, transient ischemic attacks, peripheral embolization, and, rarely, sudden cardiac death due to coronary artery embolism. Symptoms, when present, are typically related to thromboembolic events rather than the tumor itself. The tumors are composed of avascular papillary fronds of dense connective tissue covered by endothelium, with an elastic fiber core. Treatment for symptomatic papillary fibroelastomas or those at high risk for embolization is surgical excision, which is generally curative with excellent long-term outcomes. Valve-sparing surgical techniques are preferred when possible. For asymptomatic patients with small, non-mobile tumors, conservative management with close echocardiographic surveillance may be appropriate. The etiology of PFE remains uncertain, with theories including hamartomatous origin, organized thrombus, or response to endocardial damage. These tumors are most commonly diagnosed in adults, typically in the fifth to seventh decades of life.

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