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Isolated haemangioma of the tricuspid valve

THIS 41⁄2-YEAR-OLD GIRL PRESENTED AS A neonate with a systolic murmur and mild cyanosis. The echocardiogram at that time revealed mild tricuspid regurgitation, with a dilated right ventricle and right-to-left shunting across the oval foramen. The regurgitation was thought to be due to poor coaptation of the leaflets in consequence of rupture of the tendinous cords. Her cyanosis improved and she thrived, although a widely split second sound developed, and the 3/6 systolic murmur persisted without signs of heart failure. She had no palpitations or documented dysrhythmias. Neither the patient, nor members of her family, showed cutaneous manifestations of vascular malformations, although an older sister has tetralogy of Fallot. Increasing tricuspid regurgitation resulted in atrial and ventricular dilation, because of which she came to surgical repair. Surprisingly, at operation, a dark blue and cystic lesion, measuring 8 5 mm, was identified on the antero-superior leaflet of the tricuspid valve (Fig. 1). The appearance suggested a haemangioma. There were neither cords attached to this part of the leaflet, nor signs of past cordal rupture. The antero-superior leaflet was unusually large and pliable. The mass was removed, the valve repaired with autologous pericardium, and a 26 mm Carpentier Edwards tricuspid annuloplasty ring was inserted. Histological examination confirmed the diagnosis of haemangioma. In retrospect, a prolapsing cystic mass could be seen in a previous transoesophageal echocardiogram (arrow in Fig. 2). At follow-up at 1 month after the operation, her exercise capacity had improved, with regression of right atrial and right ventricular enlargement, and she had only mild tricuspid regurgitation. Haemangiomas are rare but benign tumours of the heart. Our patient had an isolated haemangioma of the tricuspid valvar leaflet, without other intracardiac or extracardiac lesions. Images in Congenital Heart Disease