Right ventricular outflow tract stenting in double outlet right ventricle with critical pulmonary stenosis and hypoplastic pulmonary arteries

Introduction

Double outlet right ventricle (DORV) is a congenital heart disease in which the great arteries (aorta and main pulmonary artery) arise from the right ventricle (RV), with concomitant ventricular septal defect (VSD), usually non-restrictive. Double outlet right ventricle can be accompanied by other cardiovascular anomalies, such as ventricular hypoplasia, restrictive septal defects and various pulmonary vascular bed anomalies, including hypoplastic pulmonary arteries (PA).
Critical right ventricle outflow tract (RVOT) stenosis and hypoplasia of pulmonary arteries in newborns with DORV necessitates the need to maintain pulmonary blood flow to provide optimal systemic oxygenation in severely cyanotic newborns. Routinely recommended treatment for cyanotic newborns is a surgical procedure with implantation of a Blalock-Taussig systemic-to-pulmonary artery shunt (BT shunt), or interventional patent arterial duct (PDA) stenting. In complex RVOT stenosis with hypoplastic pulmonary arteries alternative percutaneous multilevel RVOT and pulmonary trunk stenting could serve as an alternative treatment [1, 2].
We report multistage treatment of a hypotrophic infant suffering from DORV with RVOT stenosis and a hypoplastic pulmonary vascular bed. Initial diagnostics with angiography revealed multilevel obstruction of the RVOT with severe hypoplasia of pulmonary arteries. The baby, who presented severe cyanosis, did not meet anatomic criteria for surgical palliation while the ductal flow remained insufficient. Her initial morphology determined our alternative strategy with initial RVOT stenting, despite the hindrance caused by extremely low body weight.

Case report

The 3-day-old term born hypotrophic neonate was admitted to the Department of Pediatric Cardiac Surgery with the initial diagnosis of tetralogy of Fallot.
The girl was born by normal spontaneous vaginal delivery at the 39th week of gestation with a birth weight of 2370 g. Her prenatal medical history was complicated by maternal heavy nicotinism in pregnancy. Congenital heart disease was diagnosed at the 3rd day of life, and prostaglandin E1 (PGE1) intravenous infusion was administered.
The child was transferred to our department in an emergency setting because of rapid general deterioration with severe cyanosis. Because of hypoxemia and hypercapnia the baby was intubated on admission, and remained on mechanical ventilation during the diagnostics. Also...


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