Elsevier

The Annals of Thoracic Surgery

Volume 88, Issue 6, December 2009, Pages 1939-1947
The Annals of Thoracic Surgery

Original article
Pediatric cardiac
The Retrograde Aortic Arch in the Hybrid Approach to Hypoplastic Left Heart Syndrome

Presented at the Forty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Francisco, CA, Jan 26–28, 2009.
https://doi.org/10.1016/j.athoracsur.2009.06.115Get rights and content

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.

Section snippets

Patient Population

We carried out a retrospective review of prospectively collected data. The study was approved by the local institutional review board. In view of the retrospective design with absence of patient identifiers, the requirement for individual patient consent was waived. Between July 2002 and April 2008, 76 consecutive cases of hybrid stage 1 palliation were performed in our hospital. After excluding 1 HLHS case with extremely high-risk features (significant comorbidities, including large congenital

Patient Characteristics

Sixteen patients (24%) exhibited RAAO as defined above and received a targeted intervention at a mean age of 74 days. The characteristics of the two groups are presented in Table 1. There were no differences in terms of sex, age, or weight at either stage 1 or stage 2 operations. As for high-risk anatomic features [10, 11], the intact atrial septum and aortic atresia–mitral stenosis variants were equally distributed between the groups, but in group 1 all but 1 patient had aortic atresia (94%

Comment

In this report we highlight a number of points related to the development of RAAO in a series of consecutive patients treated with a hybrid approach for HLHS. First of all, the majority of patients (76%) do not exhibit this complication. However, in patients with RAAO there is higher morbidity and mortality interstage and probably inferior results overall. We should emphasize that including our early experience there was a negative effect on overall survival compared with results reported more

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