Hypoplastic Left Heart Variants Requiring Norwood Operation with Left Ventricular Exclusion
Kyle W. Riggs, M.D., Benjamin Kroslowitz, Farhan Zafar, M.D., Haleh Heydarian, M.D., David L. S. Morales, M.D..
Cincinnati Children's Hospital, Cincinnati, OH, USA.
Objective(s): We present three HLHS patients prenatally diagnosed with severe aortic insufficiency (AI) and/or mitral regurgitation (MR), in whom survival to term was uncertain.
Methods: Between 2015-2018, three single ventricle patients underwent LV exclusion during first stage palliation.
Results: All patients were delivered, gestational ages 39,39, 36weeks, with operative teams available due to concern of fetal health. All patients, though ill, were medically stabilized for at least 12hours. At 1,3, and 10days-of-life, all patients underwent fenestrated mitral valve patch closure for severe MR. Two patients with AI underwent Norwood operations with BT shunt placement and aortic valve oversewing, leaving a washing jet. The patient with heterotaxy, RV dominant transitional atrioventricular canal defect, aortic atresia, a right arch with a criss-cross aorta and left PDA underwent bilateral PA banding and repair of the right atrioventricular valve with the exclusion to achieve stabilization. All patients survived stage-one palliation, with one currently in the inter-stage period and two alive post-discharge after second-stage palliation. The heterotaxy patient underwent aortic uncrossing with his Kawashima.
Conclusions: The fetal diagnosis of severe AI and MR in single ventricle patients can be challenging to manage but did not result in immediate need for surgery. The use of LV exclusion in conjunction with first stage palliation has proven successful in the treatment of these HLHS variants with significant AI/MR in short and midterm follow-up. 
Back to 2018 Program
