SUMMARY An earlier study of 25 patients who were investigated by radionuclide angiography after a Mustard procedure showed that they had had evidence of right and left ventricular dysfunction at rest and with exercise. Twenty one (mean age 17-0 years (range 13-7-20-6) 11 female patients) of the original 25 patients were followed up a mean of 4-3 years later (mean 14-6 years (range 12-5-16-0) after the procedure). The group means for resting right and left ventricular ejection fraction and exercise response were not significantly different from those reported five years before. Individual changes in values were within the normal variation seen in serial studies.This long term longitudinal follow up of patients after the Mustard operation showed that although some patients still had right and left ventricular dysfunction, resting ventricular function and exercise response remained stable over a five year period. This preservation ofcardiac function may contribute to the long term survival of patients after the Mustard procedure.
Eleven patients with anomalous left coronary artery arising from the pulmonary artery were identified from the beginning of 1970 to the end of 1985. The only male patient presented at the age of eight years for assessment of a murmur and was symptom free. The remainder presented in infancy with features of cardiac failure. The electrocardiogram was abnormal in all patients, the majority showing anterolateral ischaemia. Cross sectional echocardiography when available showed a dilated, poorly contracting left ventricle, and in two cases what appeared to be a "normal" origin of the left coronary artery. Aortography was performed in 10 patients and led to the correct diagnosis in eight. The diagnosis was made at necropsy in three infants. One patient has remained well without treatment. The anomalous left coronary artery was ligated in three of the earlier patients, including the asymptomatic boy. Two of these cases had concurrent saphenous vein graft to the anomalous left coronary artery. The four most recent cases were treated by successful direct reimplantation of the anomalous left coronary artery to the aorta when they first presented in infancy. They continue to improve symptomatically and show objective improvement of myocardial function. In this recent experience supports our policy of early surgical re-establishment of a two coronary system from the aorta.
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