The first stage of hypoplastic left heart syndrome (HLHS) palliation typically occurs within the first week of life. There are three competing surgical strategies for this palliation: the Norwood procedure with modified Blalock-Taussig-Thomas shunt (innominate-artery-to-pulmonary-artery), the Norwood procedure with right-ventricle-to-pulmonary-artery (RV-to-PA, Sano) modification, and the hybrid procedure, which includes a stent across the ductus arteriosus and branch pulmonary artery bands. While there are three major surgical strategies, the objectives of each procedure are essentially the same: limit pulmonary blood flow, provide unobstructed systemic and coronary blood flow, and create a nonrestrictive atrial septal defect.
This video details the neonatal palliation of a 6-day-old, 3.18 kg boy with an antenatal diagnosis of HLHS (aortic atresia (AA)/mitral stenosis (MS)). Born at 38 weeks and 4 days gestation, the patient was transferred to Cincinnati Children’s Hospital Medical Center in Ohio, USA, on room air and a continuous infusion of 0.01 mcg/kg/min of prostaglandin. Postnatal echocardiography confirmed the diagnosis of HLHS (AA/MS) with normal right ventricular function, trivial tricuspid regurgitation, a 1.5 mm ascending aorta, a nonrestrictive fenestrated atrial septum, and a patent ductus arteriosus. The patient’s care had been previously discussed at surgical case conference and the decision was made to proceed with a Norwood procedure with RV-to-PA conduit modification (5 mm ring-reinforced Gore-Tex tube graft). Of note, this particular video covers in detail Dr James Tweddell’s technique of interdigitating aortic arch reconstruction and creation of the Damus-Kaye-Stansel connection.
References
- Tweddell JS, Mitchell ME, Woods RK, Spray TL, Quintessenza JA. Construction of the right ventricle-to-pulmonary artery conduit in the Norwood: the “Dunk” technique. Oper Tech Thorac Cardiovasc Surg. 2012; 17(2):81-98.
- Tweddell JS. The Norwood procedure with an innominate artery-to-pulmonary artery shunt. Oper Tech Thorac Cardiovasc Surg. 2005;10(2):123-140.
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