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A Small Mass Causing a Big Problem: Severe Aortic Regurgitation Induced by a Papillary Fibroelastoma
John Bajouka1, Rana Afram2, Rahul Deshmukh3
1Internal Medicine, Henry Ford Health System, Southfield , USA.
None:
Papillary fibroelastoma (PFE) is a benign primary cardiac tumor arising from the endocardial surface, most commonly affecting the valvular endocardium. Although often incidental, PFEs may lead to serious complications, including systemic embolization, valvular obstruction, or regurgitation. Aortic valve fibroelastoma resulting in severe aortic insufficiency (AI) may be rare. A 77-year-old woman with a history of hypertension and chronic kidney disease presented with progressive dyspnea, orthopnea, and bilateral lower extremity edema. Examination revealed a diastolic murmur at the left lower sternal border. Transthoracic echocardiography demonstrated a tri-leaflet aortic valve with preserved left ventricular systolic function and moderate eccentric aortic regurgitation due to incomplete cusp coaptation. A transesophageal echocardiogram was performed, which revealed a small, filamentous, and mobile mass that was visualized at the base of the noncoronary cusp (NCC), consistent with a PFE. There were no clinical or laboratory findings suggestive of infection, and the lesion's morphology was inconsistent with vegetation. Holodiastolic flow reversal in the descending aorta confirmed severe AI. The patient was diagnosed with acute decompensated heart failure secondary to severe aortic regurgitation. After medical optimization, she underwent surgical aortic valve replacement. Intraoperative histopathology confirmed the presence of a fibroelastoma originating from the NCC. Postoperative recovery was uneventful, and the patient demonstrated marked symptomatic improvement. This case illustrates a rare presentation of PFE causing severe aortic regurgitation through mechanical interference with cusp closure rather than leaflet destruction. PFEs are typically small, avascular, and pedunculated, and when located near the coaptation zone, they can prevent complete leaflet closure, resulting in significant hemodynamic compromise. Echocardiography remains the diagnostic modality of choice, and multimodal imaging may aid in differentiating PFEs from vegetations or thrombi. Surgical excision is recommended for symptomatic, mobile, or left-sided PFEs due to the risk of embolization and valvular dysfunction. Although rare, PFE of the aortic valve should be recognized as a potential cause of severe AI. Early diagnosis and timely surgical management are curative and prevent embolic complications.
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