Background:

Hypoplastic left heart disease and its variants complicated by severe semilunar and atrioventricular valve insufficiency are associated with a high mortality risk in utero and postnatally. Through the description of three cases, this report outlines the evolution of a successful strategy of morphological left ventricle isolation (ie, “left-sided Starnes”) in caring for this rare cohort of patients with hypoplastic left heart syndrome (HLHS).

Methods:

This study includes three patients: two patients prenatally diagnosed and one transferred from an outside hospital. All patients presented with complex univentricular heart disease with a nonfunctional left ventricle and severe left-sided atrioventricular and/or semilunar valve insufficiency.

Results:

Two patients were delivered with an operating room on standby due to the possibility of emergent surgery being necessary; however, all patients were able to be medically stabilized after birth. Patient 1 underwent successful left ventricle exclusion with a fenestrated polytetrafluoroethylene (PTFE) patch, despite severe mitral valve regurgitation. Patient 2 reinforced the efficacy of PTFE patch occlusion in addition to having aortic valve closure with a small washing jet. Patient 3’s clinical course was successfully predicted based on the previous patients and outlined for the family prenatally. All patients are currently growing well at home, awaiting Fontan with marked improvement in cardiac function.

Conclusion:

This rare cohort of patients with HLHS having severe left-sided atrioventricular and/or semilunar valve insufficiency appear to survive to birth and can be medically stabilized prior to semiurgent intervention. The Norwood procedure with left ventricle exclusion appears to be a successful strategy for these unique patients.