Highlights of auscultation in congenital heart disease. I.
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Morphologic evaluation of operatively excised native cardiac valves can lead to an etiologic diagnosis in most instances using a simple 10-step approach. With few exceptions, histologic evaluation of excised cardiac valves provides little additional information beyond this proposed morphologic assessment.
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A 12-year-old boy was first admitted to Tokyo Metropolitan Children's Hospital at 2 days of age with the diagnosis of tricuspid atresia without pulmonary stenosis, normally related great arteries, ventricular septal defect, and persistent left superior vena cava (PLSVC). He underwent pulmonary artery banding at the age of 1 year and atrial septectomy at 2.9 years. At the age of 6.3 years, he underwent successful Bjork operation in conjunction with closure of a trial and ventricular septal defects. Late after surgery, however, he developed progressive intolerance to exercise (NYHA III), cardiomegaly, and depressed ST segments in the left precordial leads. Catheter examination at the age of 11.9 years disclosed moderately elevated pressures (15 mmHg) in the right atrium and pulmonary artery, depressed left ventricular function with ejection fraction (EF) of 0.48, and marked dilatation of the coronary sinus (CS). Right atriography revealed retrograde filling of the dilated CS up to the junction with PLSVC. With supposition that the combined elevation of the volume and pressure loads on CS had disturbed both coronary circulation and ventricular function, he, at the age of 12 years, underwent take down of the Fontan circulation and conversion to the total cavopulmonary connection (TCPC). His postoperative recovery was uneventful and, at 2.5 years after the latest surgery, he is leading a normal life. The findings of the chest roentgenogram and electrocardiogram reverted to normal and EF returned to the normal value (0.66). This experience draws us to conclude that conversion to TCPC is a safe and effective mean to salvage the patients with failed Fontan circulation. This experience also suggests that dilatation of CS is a predictor of the failed or failing Fontan circulation.
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A 28-year-old man was admitted because of dyspnea on effort. His tricuspid valve had been affected by granulocytic sarcoma and manifested tricuspid valve stenosis 8 years previously. After chemotherapy and radiation therapy, the tumor had disappeared and the tricuspid valve stenosis was relieved. Echocardiography showed that the posterior leaflet of the mitral valve was affected by the tumor, and Doppler ultrasonography revealed mild mitral valve stenosis. Biopsy of the anterior chest wall detected granulocytic sarcoma. Chemotherapy was started. The tumor size was reduced and the mitral valve stenosis became slight. Primary cardiac granulocytic sarcoma is very rare and stenosis of the atrioventricular valve by relapse of this tumor after complete remission is extremely unusual.
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