Transcatheter ventricular septal defect (VSD) creation for restrictive VSD in double-outlet right ventricle

Abstract

Background: Double-outlet right ventricle (DORV) with a restrictive ventricular septum is a rare but highly morbid phenomenon that can be complicated by progressive left ventricular hypertrophy, arrhythmias, aneurysm formation, severe pulmonary hypertension, and death in the newborn. Surgical creation or enlargement of a ventricular septal defect (VSD) is palliative but may damage the conduction system or the atrioventricular valves in the newborn. This report presents a transcatheter approach to palliation for a newborn that had DORV with a restrictive ventricular septum.

Methods/results: A full-term infant girl (2.9 kg) referred for hypoxia (80% with room air) and murmur was found to have DORV, interrupted inferior vena cava, and restrictive VSD (95-mmHg gradient). Transhepatic access was performed, and an internal mammary (IM) catheter was advanced through the atrial septal defect and into the left ventricle. By transesophageal echocardiographic guidance, a Baylis radiofrequency perforation wire was used to cross the ventricular septum, and the defect was enlarged using a 4-mm cutting balloon. A bare metal stent then was deployed to maintain the newly created VSD. The patient did well after the procedure but required pulmonary artery banding 4 days later. She returned 5 months later with cyanosis and the development of obstructing right ventricle muscle bundles, requiring further surgical palliation.

Conclusions: This report describes the first transcatheter creation of VSD in DORV with a restrictive ventricular septum in a newborn infant. Use of the radiofrequency catheter in combination with cutting balloon dilation and stent implantation is an efficient method for creating a VSD in such a patient.

Fig. 1

Lateral view of the left ventriculogram demonstrating a restrictive ventricular septal defect (VSD) (white arrow). RV right ventricle, LV left ventricle

Fig. 2

Lateral view of the guidewire that has been advanced from the transhepatic approach across the newly created ventricular septal defect (VSD). The tip of the guidewire is in the pulmonary artery (white arrow), and a cutting balloon (yellow arrowhead) is inflated across the ventricular septum

Fig. 3

Lateral view of the stent (yellow arrowhead) being deployed across the ventricular septum

Fig. 4

Lateral view of the left ventriculogram after stent deployment demonstrating flow across the newly created ventricular septal defect (VSD) (yellow arrowhead) and the relatively small flow across the native VSD (white arrow)

Fig. 5

Left ventriculogram demonstrating the development of the obstructing muscle bundles (yellow arrowhead) on the right ventricular (RV) side of the ventricular septal defect (VSD) stent and in the subpulmonary region