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Original Articles: Pediatric Cardiac

The Retrograde Aortic Arch in the Hybrid Approach to Hypoplastic Left Heart Syndrome

Nationwide Children's Hospital, Columbus, Ohio

Accepted for publication June 25, 2009.

^_* Address correspondence to Dr Galantowicz, Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH 43205 (Email: mark.galantowicz{at}nationwidechildrens.org).

Presented at the Forty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Francisco, CA, Jan 26–28, 2009.

Background: Before palliative stage 2 for hypoplastic left heart syndrome, the coronary and cerebral circulations are often dependent on retrograde perfusion by means of the aortic arch. Results of hybrid palliation with a focus on patients exhibiting retrograde aortic arch obstruction (RAAO) were analyzed.

Methods: From July 2002 to March 2008 66 consecutive hybrid procedures for hypoplastic left heart syndrome were performed. Patients requiring RAAO intervention based on cardiology–surgery consensus were defined as group 1 (n = 16), whereas all other hypoplastic left heart syndrome patients formed group 2 (n = 50).

Results: At birth there were no differences between groups in terms of demographics or cardiac function. Group 1 had more patients with aortic atresia (94% versus 58%; p = 0.01), and 69% of patients had initial echocardiographic comments regarding incipient RAAO versus 26% in group 2 (p = 0.007). The type of ductal stent, balloon versus self-expandable, did not influence the subsequent development of RAAO. Before RAAO intervention (mean age, 74 days), group 1 patients had significantly more tricuspid regurgitation. The main treatment for RAAO in group 1 was coronary stent insertion, with 3 patients having a reverse central shunt. At a mean follow-up of 611 days, group 1 had reduced survival interstage (56.3% versus 88%; p = 0.005) and overall (43.7% versus 70%; p = 0.03).

Conclusions: Clinically important RAAO occurred in 24% of the hypoplastic left heart syndrome patients in this series. If RAAO is detected at birth or early interstage, a Norwood operation is now favored. Palliative interventional catheterization remains very important mid and late interstage for continuing the hybrid strategy toward comprehensive stage 2.

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