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Background  Hypoplastic right heart syndrome with pulmonary atresia is a rare cyanotic heart disease with poor prognosis requiring urgent intervention to establish the pulmonary blood flow. Pulmonary blood flow is achieved by BT shunt or percutaneous techniques like PDA stenting or pulmonary valve perforation. Various series have shown that early surgical intervention causes high mortality in these patients. Pulmonary valve perforation is a suitable, physiological alternative to surgical techniques in selected patients.    Case presentation  We report a case of hypoplastic right heart syndrome with pulmonary atresia and restrictive VSD presenting with cyanosis from birth and underwent pulmonary valve perforation successfully.    Conclusion  Duct-dependent pulmonary circulation is a pediatric emergency, palliative procedure for establishing adequate pulmonary blood flow is essential early in the life. In the management of duct-dependent pulmonary circulation, RVOT perforation is an effective and safe option in suitable high-risk subgroups. The induced pulmonary regurgitation along with established physiological antegrade flow would be beneficial in the remodeling of tripartite/hypertrophied small RV.

the egyptian heart journal /the egyptian heart journal

Is there a transcatheter solution for a sick neonate with hypoplastic right heart syndrome?: Pulmonary valve perforation in a neonate with hypoplastic right ventricle with pulmonary atresia, restrictive VSD—a case report