Pulmonary Artery Obstruction due to Papillary Fibroelastoma on the Pulmonary Valve: A Rare Cardiac Tumor

Abstract

Papillary fibroelastomas (PFEs) are primary cardiac tumors. They are rare benign tumors that occur on the endocardium of the heart, especially on the heart valves. The majority of these benign tumors have been observed on the left side of the heart and involved the aortic and mitral valves; however, occurrence on the right side of the heart has been infrequently reported, with only a few cases documented on the pulmonary valve. Most patients with PFEs are asymptomatic and the tumors are usually found incidentally. The tumors have the potential to cause systemic or pulmonary embolism or obstructive phenomena. We present a rare case of a papillary fibroelastoma occurring on the pulmonary valve with clinical presentation due to pulmonary artery obstruction. The tumor was removed surgically.

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

“…Moreover, compared with 2D echocardiography, 3D echocardiography provides additional information, mainly on the static and dynamic spatial location of the tumor as well as its relationship with neighboring structures. Additionally, MRI not only shows the anatomical structure but also helps to determine the benign or malignant origin of the neoplasm . PFE usually shows complete late gadolinium enhancement, often attaching to the upstream side of the valve leaflet .…”

“…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 .…”

Papillary fibroelastoma situated in the subvalvular structure of the mitral valve

“…The real pathogenesis of CPF is still unknown, [1] and it accounts for approximately 7-15% of all primary smaller than 1 cm in diameter. [2,5] The thromboembolic complications of this disease cause symptoms such as transient ischemic attack, stroke, acute coronary syndrome, and pulmonary embolisms.…”

“…[1] They are the third most common primary cardiac tumor, following myxomas and lipomas, and the most common primary valvular tumor. [2][3][4][5] They usually occur on the left side of the heart, especially on the aortic valve.…”

“…Although CPFs are benign in nature, they can cause severe thromboembolic complications regardless of the tumor size. [1,3,5,7,8] Therefore, surgical treatment is recommended to prevent this from happening. [2,4] Here we report a patient with papillary fibroelastoma of the subvalvular structure of the mitral valve that was treated surgically.…”