The retrograde aortic arch in the hybrid approach to hypoplastic left heart syndrome

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.

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).

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).

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.