• M Imai, M Yamaguchi, H Ohashi, Y Oshima, H Maeda, H Mito, T Tei, Y Hosokawa, H Tachibana, and M Miyashita.
• Department of Cardio-thoracic Surgery, Kobe Children's Hospital, Japan.
• Nihon Kyobu Geka Gakkai Zasshi. 1994 Jun 1; 42 (6): 896-905.

AbstractIn the last 14 years, 7 consecutive patients with supravalvular aortic stenosis (SVAS) underwent surgical treatment for SVAS and/or for associated lesions. There were 5 male and 2 female patients ranging in age from 3 months to 12 years. Six of them had associated other cardiac anomalies; two had severe multiple peripheral pulmonary stenoses (PPS) and one each had ventricular septal defect (VSD), valvular pulmonary stenosis, coarctation of aorta with patent ductus arteriosus (PDA), total anomalous pulmonary venous return (TAPVR) with pulmonary branch stenosis, PPS and left lower pulmonary venous obstruction. The type of SVAS were localized in all and 5 of them underwent successful surgical repair of SVAS (3 with extended aortoplasty and 2 with patch aortoplasty). In 2 patients with VSD or valvular pulmonary stenosis, associated cardiac anomalies and SVAS were repaired simultaneously. Four patients had undergone previous operations, which included repair of severe multiple PPS by extended peripheral pulmonary arterioplasty (case 4, 6), repair of coarctation of aorta and division of PDA (case 5), repair of TAPVR (Ia + IIa) and pulmonary branch stenosis (case 7). There was no operative death and one patient died late postoperatively (case 7) due to right heart failure in a follow up period of 3 to 14 years. In conclusion, it is important to select the appropriate surgical treatment according to the location and the severity of associated other cardiac anomalies as well as the severity of SVAS, and extended peripheral pulmonary arterioplasty is considered to be a recommended method for the relief of severe multiple PPS associated with SVAS.

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