Mitral Papillary Muscle Papillary Fibroelastoma in Obstructive Hypertrophic Cardiomyopathy: A Surgically Treated Embolic Source.

BACKGROUND

Papillary fibroelastoma, an uncommon cardiac tumor, can cause systemic embolization and raise unique diagnostic and therapeutic challenges when superimposed on a hypertrophic cardiomyopathy.

CASE SUMMARY: A 71-year-old woman with obstructive HCM, prior unilateral retinal artery occlusion (RAO), and progressive exertional dyspnea was referred for Mavacamten therapy. An incidental mitral chordal mass was found on baseline transthoracic echocardiography, suspicious for papillary fibroelastoma. Given the prior unilateral RAO and symptomatic LVOT obstruction, septal myectomy and mass excision were performed. Histopathology confirmed papillary fibroelastoma, and her symptoms and LVOT gradients improved significantly.

DISCUSSION: This case underscores the importance of comprehensive imaging to identify rare valvular tumors as potential embolic sources in patients with obstructive HCM and prior embolic events, with important management implications.

TAKE-HOME MESSAGE: Mitral papillary fibroelastoma in obstructive HCM, even if "incidental," can be a surgically correctable embolic source beyond atrial fibrillation and may warrant combined resection and myectomy.