Pediatric Cardiology and Cardiac Surgery
Vol.26 No.3 2010 (243-248)

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Hazumu Nagata,1) Yu-ichi Ishikawa,1) Shiro Ishikawa,1) Kazushi Yasuda,1) Makoto Nakamura,1) Ko-ichi Sagawa,1) Hiroya Ushinohama,1) Naoki Fusazaki,2) and Hideaki Kado3)

Departments of 1)Pediatric Cardiology, 2)Neonatal Cardiology, and 3)Cardiovascular Surgery, Fukuoka Children’s Hospital, Fukuoka, Japan

The Norwood procedure is a highly invasive, but unavoidable, palliative operation for patients with hypoplastic left heart syndrome (HLHS), followed by Fontan circulation. In high-risk HLHS patients with severely deteriorated ventricular function, we perform bilateral pulmonary artery banding (Bil-PAB) as the first palliative operation. For a long period between Bil-PAB and the Norwood or Norwood-Glenn procedure, it is necessary to maintain both interatrial and ductal blood flow. On the narrowing of interatrial communication after Bil-PAB, balloon atrial septostomy (BAS) is performed to maintain interatrial blood flow, although its effect may be unstable. On the other hand, PGE1 administration to maintain the patency of ductus arteriosus for a long period, which is necessary for retaining systemic circulation, may cause some side effects, and it therefore requires careful monitoring. We experienced a case of a girl with HLHS who underwent Bil-PAB 7 days and BAS 30 days after birth. We judged it difficult to perform the Norwood-Glenn procedure because interatrial communication became too restrictive, long PGE1 infusion caused side effects, and ventricle function did not improve. Therefore, we decided to select the less invasive procedure of double interatrial and ductal stenting by cardiac catheterization. After successful stenting, respiratory management using hypoxic gas mixture was needed against heart failure due to the increased pulmonary blood flow. Finally, she underwent the Norwood-Glenn procedure at 240 days of age. In Japan there have been few reports on double stenting, and therefore there are no definite criteria for the indication, efficacy, optimal implant-timing, maneuver, and subsequent management of double stenting. Double stenting may be one therapeutic strategy for HLHS, especially for a high-risk patient.