Transcatheter closure of congenital VSDs offers encouraging results. COMPLICATIONS are limited; the most relevant one seems to be the device related to cAVB in perimembranous VSD. More experience and long-term follow-up are mandatory to assess safety and effectiveness of this procedure as an alternative to conventional surgery.

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ConclusionHitherto, cardiomyopathy has never been reported as the presenting symptom of a mitochondrial disorder. Although it appears premature to conclude as to the prevalence of defects of oxidative phosphorylation in hypertrophic cardiomyopathies, this study demonstrates that such defects should be regarded as a potential cause of idiopathic hypertrophic cardiomyopathy in early infancy. While an abnormal redox status in plasma and widespread multitissue defects support the diagnosis of mitochondrial cardiomyopathy, normal results in such investigations do not preclude the possibility of heart‐specific respiratory chain defect and should prompt one to carry outin vivo investigations on endomyocardial biopsies.

21 consecutive infants aged less than six months (mean (SD) 2-6 (1 2)) were admitted with anomalous origin of the left coronary artery from the pulmonary artery. In the first 12 patients, who were seen up to 1982, operation was performed after the age of one year (mean (SD) 29 (29) months) (group 1). The next nine infants, seen from 1983 to 1987, had their operations within a few weeks of the onset of symptoms (mean (SD) age 4-8 (1 4) months) (group 2). In group 2 the left coronary artery was relocated into the aorta, whereas in group 1 there was additional resection of the left ventricular wall or mitral valvoplasty or both. At presentation there were no differences in age, clinical condition, heart enlargement, and echocardiographic left ventricular dysfunction between groups 1 and 2. Seven of the 12 patients in group 1 died, five while they were awaiting operation (three died suddenly at home) and two at operation. The five survivors are doing well 6-4 (3-1) years after operation with normal left ventricular function which improved slowly over several months after operation. Two of the nine patients in group 2 died; both deaths occurred at or soon after operation. The seven survivors are doing well 1 8 (0 9) years after operation. In three, left ventricular function recovered within three weeks; and there was even partial or total regression of the Q waves in the supposedly necrotic areas. In the remaining four the pattern of improvement in left ventricular function resembled that in group 1.Operation should be undertaken early in infants with anomalous left coronary artery arising from the pulmonary artery because the procedure is relatively safe, prevents a high natural mortality, and offers a better chance of a faster recovery of left ventricular function.Anomalous origin ofthe left coronary artery from the pulmonary artery is a rare severe congenital anomaly that often causes myocardial infarction within a few weeks of birth with subsequent ventricular dysfunction, mitral valve incompetence, and severe congestive heart failure.There is disagreement about when to operate and whether prompt restoration of a systemic left coronary artery perfusion is important. There have been numerous conflicting reports about the risks of Requests for reprints to Professor Jean Kachaner, Hopital des Enfants-Malades,