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Combined Norwood Operation And Batista Partial Left Ventriculectomy In A Neonate With Severe Mitral Regurgitation And Aortic Stenosis And Left Ventricular Dilated Cardiomyopathy
Bahaaldin Alsoufi1, Brian Holland1, Deborah J. Kozik2, Erle Austin3.
1University of Louisville, Louisville, KY, USA, 2Deborah J. Kozik, Louisville, KY, USA, 3Erle Austin, Louisville, KY, USA.

Background: Severe mitral regurgitation (MR), LV dilatation/dysfunction and aortic stenosis (AS) is a rare challenging combination that is associated with significant risk of fetal demise and death following intervention. Outcomes of single ventricle palliation in these patients are poor since the dilated thin LV and MR impede RV function following Norwood. The largest single-institution series reported survival beyond 3 months of age in only 2/21 patients.
Methods: A neonate with prenatal diagnosis of severe AS (functional atresia), severely dilated thin LV, severe MR and intact atrial septum underwent emergency atrial septostomy (1st hour of life) and Norwood operation, Batista partial left ventriculectomy and mitral valve closure on day 3.
Results: Partial left ventriculectomy was performed from the apex to base of LV, and mitral valve closure was performed through the LV opening. The Norwood operation was completed as routine and a modified Blalock-Taussig shunt was used as source of pulmonary blood flow. Echocardiogram showed regression of the dilated LV and no MR, with good RV and tricuspid valve function. Post-operative course was remarkable for higher inotropic support, delayed sternal closure and prolonged intubation and ICU stay. Patient survived and is awaiting Glenn operation.
Conclusion: Partial left ventriculectomy and mitral closure in addition to Norwood alleviate the compression effect on the systemic RV and allow survival in neonates born with an extremely challenging cardiac pathology.


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