Giant Right Ventricular Fibroma in an Infant
Apreviously asymptomatic 5-month-old girl was admitted because of polypnea and dyspnea, with signs of moderate heart failure. Physical examination showed diffuse wheezing, a 3/6 systolic precordial murmur, and an enlarged liver 4 cm from the right costal border. Chest x-ray demonstrated an increased cardiothoracic index (0.52) with pulmonary congestion. ECG showed sinus rhythm. An episode of ventricular tachycardia (175 bpm) occurred soon after hospitalization. Two-dimensional echocardiography showed a huge, roundish mass (6×5 cm in size) at the right ventricular (RV) free wall site, which was causing RV outflow tract obstruction (49 mm Hg). MRI (Figure 1) confirmed a solid mass, 6×4×5 cm in size, involving the RV anterior myocardial wall and causing severe RV cavity reduction. Main pulmonary artery and aorta were cranially and posteriorly dislocated.
At cardiopulmonary bypass, the mass was finally isolated and resected, through gentle dissection of myocardium from epicardium and endocardium, and the RV free wall was reconstructed with a 0.6-mm polytetrafluoroethylene patch. An intraoperative biopsy showed abundant proliferation of fibroblasts with collagen deposition, which are diagnostic for cardiac fibroma (Figure 2A). The excised, rounded mass, 81 g in weight and 6×5 cm in size (Figure 2B), appeared firm, white, and whorled.
At follow-up 5 years later, the child is alive and well, free of medications, and has sinus rhythm. Two-dimensional echocardiography shows normal RV contractility with mild dilatation and hypertrophy.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.