Objective:
To report incidence and assess outcome of hypoplastic left heart syndrome (HLHS) patients with ventriculo-coronary connections (VCC) following stage1 palliation (S1P). Impaired coronary perfusion and mitral stenosis/aortic atresia (MS/AA) subtype have both been implicated to cause early post-operative and interstage mortality in HLHS. We seek to determine if presence of VCC affects outcome of patients with MS/AA.


Methods:
We reviewed 86 patients with HLHS between September 2005 and December 2008. Besides VCC, age at S1P, birth weight (BW), ascending aorta size (AAo), anatomic type, associated lesions, surgical technique, cardiopulmonary-bypass time, cross-clamp time (XCT), circulatory arrest time and post-operative complications were reviewed.


Results:
Overall survival of MS/AA group to stage2 palliation (S2P) was 71%; 12 of 24 patients with MS/AA had VCC (50%). Eleven of twelve patients with VCC survived to S2P (92%). Presence of VCC with MS/AA did not contribute to higher mortality (p<0.05). Interstage mortality between S1P and S2P was not different among the 3 subtypes of HLHS (p<0.05). Higher incidence of restrictive atrial communication, RV dysfunction, arrhythmia and ECMO in the mitral atresia/aortic atresia group was due to chance. Multivariate analysis demonstrated that low BW, longer XCT, AAo<2mm and arrhythmia contributed to higher mortality and need for ECMO.


Conclusion:
VCC occurs exclusively with MS/AA subtype. Incidence of VCC was 50% of MS/AA. The presence of VCC was not associated with higher morbidity or mortality after S1P. We postulate this to be due to our institutional preference of performing RV-PA conduits for patients with aortic atresia during S1P.