Norwood and Batista Procedure in Neonatal Dilated Cardiomyopathy

The Batista procedure, or ventriculoplasty, was originally described by Dr Randas Batista and colleagues as a treatment for ischemic dilated cardiomyopathy. There have been only a few case reports for this strategy in the management of neonatal dilated cardiomyopathy. Even fewer cases have been reported of a combined Norwood and Batista procedure for the management of these patients.

The patient was born at 37 weeks and is now a 5-day-old 3.3 kg boy with an antenatal diagnosis of complex congenital heart disease. The diagnosis consisted of a poorly contractile, severely dilated left ventricle, moderate mitral regurgitation, moderate aortic regurgitation, and mild aortic valve hypoplasia. The patient was delivered at Cincinnati Children’s Hospital and was intubated shortly after for hypercarbia. The patient was started on prostaglandin E1 infusion at 0.02 mcg/kg/min and a transthoracic echocardiogram was obtained.

On day of life 0, the patient was then transferred to the catheterization lab for balloon dilatation of the aortic valve with the intent of allowing for more antegrade flow from the left ventricle. There was no improvement of antegrade flow across the aortic valve following balloon dilatation (and subsystemic left ventricular pressures). Despite PGE infusion, increasing inotropic support, and mechanical ventilation, the patient’s condition remained unchanged.

With no functional improvement despite maximized medical therapy, the patient was presented at a surgical management conference with the decision being to take the patient down the single-ventricle pathway. Given that the patient was supported by his right ventricle with no improvement in left ventricular function, a Norwood procedure with a 3.5 mm right-modified Blalock-Taussig shunt in conjunction with a Batista procedure (partial left ventriculectomy) to manage his large, globular left ventricle was decided to be the most reasonable option.

The patient had a relatively uncomplicated postoperative course with some persistent atrial ectopy that eventually resolved with Amiodarone. The patient was discharged home on postoperative day 70. The patient did well as an outpatient with only one planned re-admission for pre-Glenn hemodynamic diagnostic catheterization. The patient then underwent a bidirectional Glenn procedure at five months of age. The postoperative period was uneventful and the patient was discharged on postoperative day 20. The patient is now nearly 11 months old and doing well as an outpatient.

Resources

1. Batista RJ, Santos JL, Takeshita N, Bocchino L, Lima PN, Cunha MA. Partial left ventriculectomy to improve left ventricular function in end-stage heart disease. J Card Surg. 1996;11:96-97.
2. Yoshii S, Hosaka S, Takahashi W, Amano H, Abraham SJ, Kaga S, et al. Partial left ventriculectomy in an infant with dilated cardiomyopathy. J Thorac Cardiovasc Surg. 1999;117:616-618.
3. Berger S, Tweddell JS, Frommelt PC, Weinhaus L. Partial left ventriculectomy for dilated cardiomyopathy in a newborn. J Thorac Cardiovasc Surg. 1999;117:1017-1018.
4. Mainwaring RD, Healy RM, Murphy JD, Norwood WI. Norwood/Batista operation for a newborn with dilated myopathy of the left ventricle. J Thorac Cardiovasc Surg. 2000;120(3):612-615.
5. Myers, PO, Sologashvili T, Beghetti M, Tissot C. Norwood stage 1 with surgical ventricular reconstruction and mitral valve repair for neonatal idiopathic left ventricular dilated cardiomyopathy. Ann Thorac Surg. 2016;102(1):e15-e17.