Pulmonary valve papillary fibroelastoma: management of an unusual, tricky pathology

Abstract

Papillary fibroelastomas are rare benign tumours of the endocardium, accounting for the most common primary valvular tumours of the heart. They typically originate from left-sided heart valves, whereas pulmonary valve involvement is anecdotal. They rarely cause valvular dysfunction, but they can cause turbulent flow and thrombus formation with consequent cerebral, retinal, coronary and pulmonary embolic disease and obstruction. We present here the case of a 56-year old man who was referred to our institution with an accidental finding, at transthoracic echocardiogram, of a mobile, pedunculated mass on the pulmonary valve, confirmed at cardiac magnetic resonance. He underwent surgical removal of the mass through median sternotomy with complete sparing of the valve. The postoperative course was unremarkable. Histopathological examination confirmed that the mass was a papillary fibroelastoma.

Incidental finding of an asymptomatic pulmonary valve papillary fibroelastoma: A case report

“…In recent years, with the development of cardiac imaging technologies, including echocardiography, the diagnosis rate of PFE has greatly increased. As a benign tumor with a low tendency to grow, PFE seldom leads to valvular dysfunction . However, PFE patients have a potential risk of developing life‐threatening embolic complications.…”

“…Gowda and colleagues proposed that surgery should be considered for asymptomatic PFE patients if the tumor is friable, mobile, or larger than 1 cm in diameter . However, most experts suggest surgical resection irrespective of the symptoms, location, and mobility of PFE, in order to prevent the occurrence of complications . The incidence of recurrence following operation has not yet been reported .…”

“…3 However, most experts suggest surgical resection irrespective of the symptoms, location, and mobility of PFE, in order to prevent the occurrence of complications. 4,6,16 The incidence of recurrence following operation has not yet been reported. 17 Hence, surgical intervention is believed to be a safe, effective, and welltolerated treatment for PFE patients with a good long-term prognosis.…”

Papillary Fibroelastoma of the Pulmonary Valve – A Systematic Review

“…3, movie clip S1), which can flutter or prolapse into the right ventricular outflow tract or the right ventricle during systole or diastole (movie clip S2). 26 PVPFE may be mistaken for Libman-Sacks (nonbacterial verrucous) endocarditis in patients with high antinuclear antibody titer. However, echolucencies with speckled appearance and stippled pattern near edges which shimmer may be suggestive of these tumors.…”

“…9 Other differential diagnosis for PVPFE include tumors (usually myxomas and lipomas), thrombi, and bacterial vegetations. 26 PVPFE may be mistaken for Libman-Sacks (nonbacterial verrucous) endocarditis in patients with high antinuclear antibody titer. 6 Due to their slow growth, they may serve as a nidus, for the formation of large superimposed thrombi over a short period of time.…”