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

• Published in: European journal of cardio-thoracic surgery Vol. 32; no. 5; pp. 745 - 750
• Main Authors: Hosein, Riad B.M., Mehta, Chetan, Stickley, John, Mcguirk, Simon P., Jones, Timothy J., Brawn, William J., Barron, David J.
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier Science B.V 01.11.2007; Elsevier Science
• Subjects: Aorta - surgery; Aorto-pulmonary window; Biological and medical sciences; Cardiac Surgical Procedures - methods; Cardiac Surgical Procedures - mortality; Cardiology. Vascular system; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Heart; Humans; Hybrid; Hypoplastic left heart syndrome; Hypoplastic Left Heart Syndrome - mortality; Hypoplastic Left Heart Syndrome - surgery; Infant; Infant, Newborn; Medical sciences; Norwood; Palliative Care; Pulmonary Artery - diagnostic imaging; Pulmonary Artery - surgery; Radiography; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the heart; Survival Rate; Treatment Outcome; Ultrasonics; Norwood; Hybrid; Hypoplastic left heart syndrome; Aorto-pulmonary window; Lung; Cardiovascular disease; Left Heart Hypoplasia; Windows; Congenital disease; Pulmonary artery; Syndrome; Treatment; Heart disease; Surgery; Aorta
• ISSN: 1010-7940; 1873-734X
• DOI: 10.1016/j.ejcts.2007.07.024

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.