Thirteen children (age range 6 h-11 years, median 4 months) operated upon for aortico-left ventricular tunnel since 1971 are reported. All patients had systolic and diastolic murmurs, 4 of 5 neonates were in severe congestive heart failure. Twelve children had left ventricular hypertrophy on ECG with an ischaemic pattern in the neonates. Chest X-ray revealed cardiomegaly with a broad upper mediastinal shadow from the enlarged aorta. Echocardiography (2DE) revealed a tubular communication between the aorta and the left ventricle bypassing the aortic valve and massive regurgitation through this tunnel demonstrated by Doppler sonography. In 8 patients, the diagnosis was confirmed by angiography. The tunnel arose from the right coronary sinus in 12 and from the left coronary sinus in 1 patient. The tunnel was closed at both ends in 8, at the aortic end only in 3 and at the ventricular end in the remaining 2 cases. One 6-day-old infant, operated upon in 1971 died postoperatively. There was 1 non-cardiac related late death due to a brain tumour. Echocardiographic analysis of left ventricular size and function was carried out in 9 children up to 39 months after surgery. Mild to moderate aortic incompetence was detected during the follow-up period in 2 of 12 patients. All surviving children are leading normal lives.

Competitive flow is well tolerated in the short and medium term after BCPA, and early postoperative systemic oxygen saturations are improved. The long-term influence of competitive flow on pulmonary arterial growth, arteriovenous malformation development, and ventricular function warrants investigation.

Since 1979, a total of 69 infants and children aged 0.1 to 11.9 (median 0.7) years required surgical intervention for: double aortic arch (26), anomalous origin of innominate artery (26), right aortic arch with left ligamentum arteriosum (9), pulmonary artery sling (5), retroesophageal right subclavian artery (3). Before operation, the morbidity was high: 84% of children were symptomatic in the first trimester of life with 24 patients requiring mechanical ventilation. Seven children had an accompanying heart defect. Left thoracotomy was the preferred approach. There were 2 early (asphyxic brain damage, postoperative pneumonia) and 2 late (tracheomalacia, complex heart defect) deaths. Five patients (2 originally operated elsewhere) needed reoperation for persistent symptoms. All 65 survivors are well 1 month to 11.9 (mean 3.9 +/- 3.62) years after surgery. Tracheobronchoscopy, magnetic resonance imaging, and lung function testing were helpful for postoperative evaluation. Minor tracheal compression was revealed in 4 patients despite their good clinical condition.

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