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Creation of aorto-pulmonary window with pulmonary artery band is not good palliation for hypoplastic left heart syndrome

^a Department of Cardiac Surgery, Birmingham Children's Hospital, United Kingdom
^b Department of Cardiology, Birmingham Children's Hospital, United Kingdom

Received 13 March 2007; received in revised form 4 July 2007; accepted 5 July 2007.

^_* Corresponding author. Address: Department of Paediatric Cardiac Surgery, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, United Kingdom. Tel.: +44 121 333 9435; fax: +44 121 333 9441. (Email: david.barron{at}bch.nhs.uk).

Objective: A small sub-group of patients with hypoplastic left heart syndrome (HLHS) have normal-sized ascending aorta and arch. An alternative to the Norwood I procedure in these patients is the creation of an aorto-pulmonary (AP) window with a distal pulmonary artery band (PAB). We reviewed our experience with this technique and compared outcomes to the Norwood procedure for HLHS. Methods: All patients treated for HLHS in a single institution between 1992 and 2005 were analysed. This identified 13 patients treated with AP window and PAB compared to 333 patients undergoing stage I Norwood procedure. An unrestrictive AP window was created and the main PA was banded. Patient records and echocardiograms were analysed. Median follow-up was 10 (IQR 0–655) days and 100% complete. Results: There were seven early deaths (54%) in the AP window group and two conversions to Norwood circulation. This was a significantly worse outcome than for the Norwood procedure over the same period, which had an early mortality of 29% (p = 0.03). Kaplan–Meier actuarial analysis demonstrated a continued survival benefit of the Norwood group at 6 months (p = 0.0005). Deaths were due to either low cardiac output syndrome (n = 4) or sudden unheralded arrest (n = 3). This occurred despite aortic cross-clamp and circulatory arrest times being significantly lower in the AP window group compared to the Norwood group (35 ± 27 vs 55 ± 16 min, p < 0.01 and 16 ± 29 vs 55 ± 20 min, p < 0.01, respectively). No differences in arterial saturations or systolic blood pressure existed between the groups, but diastolic blood pressure was significantly lower in the AP window group at 27 ± 10 mmHg compared to 42 ± 8 mmHg in the Norwood group (p = 0.01) with evidence of flow reversal in the descending aorta. Differences in diastolic blood pressure between groups were abolished after conversion to stage II. Conclusions: Despite favourable anatomy and shorter ischaemic times, the AP window/PAB technique has a poor outcome compared to the Norwood procedure for HLHS. Low diastolic blood pressure with reversal of descending aortic flow in diastole was a feature of the AP window/PAB circulation. We recommend the Norwood procedure for these sub-types. This may have implications for newer ‘hybrid’ procedures for HLHS which create a similar palliative circulation.

Key Words: Aorto-pulmonary window • Hypoplastic left heart syndrome • Norwood • Hybrid