We report the case of a 3,970-g boy who had been delivered by cesarean section after an uncomplicated pregnancy. Physical examination revealed an enlarged occipitofrontal circumference and left-handed syndactyly between DIII and DIV. Further investigations were unproductive. There was no heart murmur.
The patient's 30-year-old mother had a known nevoid basal-cell carcinoma syndrome. The boy presented with signs typical of that syndrome.
At the age of 8 months, the infant experienced ventricular tachycardia and polymorphic multifocal premature ventricular contractions, and the onset of new systolic murmur. The electrocardiogram showed sinus rhythm (heart rate, 125 beats/min) intermittent with ventricular tachycardia (heart rate, 200 beats/min) (). He was treated with amiodarone. There were no signs of congestive heart failure. The chest radiograph revealed slight cardiomegaly. Two-dimensional transthoracic echocardiography revealed a 48 × 32 × 30-mm echogenic mass in the left ventricle, arising from the free anterolateral wall near the apex. There was obstruction of the left ventricular outflow tract, but no valvular incompetence; diastolic and systolic ventricular functions were good. Spiral computed tomography confirmed a heterogenous mass (Hounsfield units, –10 to 40) of the free wall of the left ventricle without local calcifications, and heterogenous tissue enhanced by contrast agent occupied 70% of the left ventricle (). Slight revascularization of the tumor from a diagonal branch was seen; the other coronary arteries appeared normal.
Fig. 1 A 12-lead echocardiogram shows ventricular tachycardia.
Fig. 2 Spiral computed tomography shows a mass of the free wall of the left ventricle.
The patient was operated on via a median sternotomy with the use of standard cardiopulmonary bypass, cold cardioplegia, and moderate hypothermia. The nonencapsulated tumor was excised en bloc by partial left ventriculectomy, after we opened the anterior left ventricular free wall (). The incision was closed directly, with a 5-0 Prolene running suture ().
Fig. 3 A) Tumor in situ before resection and B) left ventiicle after reconstruction
The aortic cross-clamp time was 30 minutes. The patient was stable throughout the procedure, remaining in sinus rhythm, and was safely weaned from cardiopulmonary bypass. Transesophageal echocardiography showed complete removal of the tumor and good ventricular function.
On histologic examination, a benign cardiac fibroma was diagnosed ().
Fig. 4 Photomicrograph of the tumor. (H & E, orig. ×50)
Follow-up echocardiography at 48 months was normal, and there was no evidence of recurrence of the tumor. Twenty-four-hour Holter monitoring showed the patient to be free of arrhythmic episodes.