A pioneering intervention to treat the typically fatal combination of fetal mitral dysplasia syndrome (which consists of critical aortic stenosis, severe mitral regurgitation and massively enlarged, dysfunctional left ventricle) is showing positive results at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine.

For a boy born at 37 weeks weighing just more than 7 pounds, the coordinated prenatal care and preparation for his first hour of life were essential for survival. The boy’s planned delivery with immediate resuscitation preceded an emergency balloon atrial septostomy and placement of atrial stent in the cardiac catheterization laboratory within the first hour of his life.

Two days later, the boy was in surgery undergoing a complex palliative operation. The baby underwent the conventional Norwood procedure with homograft arch reconstruction and the use of a modified Blalock-Taussig shunt to provide pulmonary blood flow. Additionally, to address his dilated dysfunctional mitral valve and left ventricle (which usually interfere with cardiac function and contribute to the reported 90% mortality of this lethal combination), he underwent partial left ventriculectomy and mitral valve closure, in addition to atrial septectomy and stent removal.

After the surgery, an echocardiogram showed good right ventricle function and a small left ventricle cavity with no mitral regurgitation and, most importantly, no compression on the right ventricle. Norton Children’s Heart Institute physicians Bahaaldin Alsoufi, M.D.; Edward S. Kim, M.D.; Brian J. Holland, M.D.; Erle H. Austin III, M.D.; and Deborah J. Kozik, D.O., wrote about the case in “Norwood Operation With Partial Left Ventriculectomy For Neonatal Dilated Cardiomyopathy,” published recently in The Annals of Thoracic Surgery.

After nearly seven weeks at Norton Children’s Hospital, the boy was discharged. He underwent Glenn bidirectional cavopulmonary connection and pulmonary artery augmentation at 6 months and has been doing well at home without oxygen support.

“The surgical approach that we adopted (Norwood and partial left ventriculectomy) addressed the hemodynamic insult of the dilated left ventricle and mitral regurgitation and provided survival in a very challenging neonate,” the doctors wrote. “We predict he will complete his third palliation stage without contraindication.”