Hybrid Approach to Right Ventricle Decompression in Muscular Pulmonary Atresia with Intact Ventricular Septum
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CASE REPORT
Hybrid Approach to Right Ventricle Decompression in Muscular Pulmonary Atresia with Intact Ventricular Septum Bassel Mohammad Nijres1 · Yasser Al‑Khatib1,2 · Giedrius Baliulis1,3 · Neal D. Hillman1,3 · Joseph J. Vettukattil1,2 Received: 26 February 2020 / Accepted: 25 April 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Muscular pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is traditionally managed by surgery. We describe hybrid approach to decompress the right ventricle (RV) and establish RV to pulmonary artery connection in a neonate avoiding cardiopulmonary bypass. A 21-gauge access needle was used to perforate the atretic pulmonary valve via periventricular approach followed by stent placement. This case is an example of how patients with congenital heart disease can be palliated in creative ways through thoughtful collaboration between surgical and interventional cardiology teams. Keywords Hybrid procedure · Pulmonary atresia with intact ventricular septum · Decompression of right ventricle
Introduction
Case Report
The right ventricle (RV) is traditionally decompressed during surgery, which requires cardiopulmonary bypass (CPB), in patients with muscular pulmonary atresia with intact ventricular septum (PA/IVS) who have the potential to undergo 1.5-ventricle or biventricular repair. Transcatheter or hybrid approaches have been used exclusively in patients with membranous PA/IVS [1]. We describe a neonate with muscular PA/IVS who was managed by initial hybrid approach followed by complete repair at 9 months of age. Expanding use of the hybrid approach for children with muscular PA/ IVS will likely improve the outcomes of this subgroup of patients.
A 3-day-old full-term male with birth weight of 2.9 kg had antenatal diagnosis of PA/IVS. Postnatal transthoracic echocardiogram (TTE) confirmed the following diagnoses: muscular PA/IVS, dysplastic tricuspid valve (TV) with severe tricuspid regurgitation (TR), tripartite RV, aneurysmal right ventricle outflow tract (RVOT), and severe RV dilatation with systolic dysfunction. The muscular tissue separating the infundibulum and main pulmonary artery (MPA) stump measured 2 mm. Prostaglandin E-2 (PGE-2) infusion was initiated to maintain patency of the ductus arteriosus. Surgical approach in this setting of severe TR and RV dysfunction would have required RV decompression with a RV to PA connection and tricuspid valve repair under CPB that can be extremely challenging in a neonate. As the patient had aneurysmal dilation of the RVOT, this raised the concern for maintaining the position of the catheter against the atretic valve. Additionally, the atretic area had a muscular discontinuity between the RVOT and MPA. In this scenario, transcatheter approach increases risk of RVOT perforation. Therefore, our multidisciplinary team agreed that the hybrid approach is the best treatment option. Thus, the proposed plan was to perform a diagnostic cardiac catheterization initially to confirm suitability
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