Pulmonary Valve Fibroelastoma, Still a Very Rare Cardiac Tumor: Case Report and Literature Review

Background and Clinical Significance: Primary cardiac tumors are among the rarest types of tumor, and until the mid-20th century, they were diagnosed only post-mortem or during other surgical interventions. With the rapid evolution of cardiovascular imaging and the widespread use of echocardiography, the incidence of cardiac fibroelastoma has increased, though it remains one of the rarest primary cardiac tumors. Papillary fibroelastoma is a benign primary cardiac tumor that develops from endocardial tissue, is usually solitary, and can have multiple locations, with the pulmonary valve being one of the rarest sites. The symptoms and complications depend on the tumor’s location, ranging from asymptomatic patients to cerebral ischemic embolism or pulmonary embolism. We analyzed the electronic databases PubMed, Web of Science, and Cochrane and conducted a systematic review of pulmonary valve papillary fibroelastoma (PVPF). Additionally, we included a case from the Adult and Pediatric Cardiovascular Surgery Clinic in Targu Mures. Case Presentation: We present the case of a 58-year-old patient who complained of exertional dyspnea. A transthoracic echocardiography (TTE) revealed a tumor mass attached to the pulmonary valve and coronary angiography identified severe coronary lesions. Following discussions within the Heart Team, surgical myocardial revascularization and tumor excision were decided upon due to the thromboembolic risk. Histopathological examination confirmed the diagnosis of papillary fibroelastoma. The postoperative course was uneventful, with an improvement in dyspnea. The mean age of the patients was 60 years, with half being men (n = 26, 50%). Regarding symptoms, 34% (n = 18) of cases were incidentally identified, while over 30% (n = 17) presented with dyspnea. Pulmonary embolism (PE) was reported in only two patients, and the most common associated comorbidities included high blood pressure (HBP) in 33% (n = 16) and dyslipidemia in 18%. Tumor size ranged from 0.7 cm to 3 cm with the initial benign cardiac tumor; its occurrence in the pulmonary valve remains exceedingly rare. Due to its frequent overlap with other cardiac pathologies, the clinical presentation is often a nonspecific diagnosis or suspicion of a tumor predominantly established via transthoracic echocardiography in 62% of patients. From a surgical perspective, 63% (n = 33) underwent tumor resection with valve sparing, 25% (n = 12) required pulmonary valve repair, and three patients necessitated pulmonary valve replacement. Conclusions: Although the incidence of papillary fibroelastoma is increasing, making it the most common, there is a need to highlight the indispensable role of echocardiography in diagnosis. Although papillary fibroelastoma is benign, surgical intervention is recommended, particularly in symptomatic patients, or if the tumor exceeds 1 cm in size, exhibits increased mobility, or is present alongside other cardiac surgical procedures.