Modified Microvascular Plug as a Flow Restrictor in Hypoplastic Left Heart Syndrome with Dysplastic Tricuspid and Pulmonary Valves
Presenter
Joshua David Kurtz, MD, University of Louisville School of Medicine, Louisville, KY
Joshua David Kurtz, MD1, Edward Kim, M.D.2, Sarah J Wilkens, MD, MPH3 and Bahaaldin Alsoufi, MD3, (1)University of Louisville School of Medicine, Louisville, KY, (2)University of Louisville, Louisville, KY, (3)University of Louisville School of Medicine, Seattle, KY
Title
Modified Microvascular Plug as a Flow Restrictor in Hypoplastic Left Heart Syndrome with Dysplastic Tricuspid and Pulmonary Valves
Introduction
While Norwood operation is the most common palliative surgery for neonates with hypoplastic left heart syndrome (HLHS), initial hybrid strategy aiming to restrict pulmonary blood flow and maintain systemic output is necessary when Norwood is contraindicated or high risk. The traditional mainstay of initial hybrid palliation is surgical pulmonary artery branch banding (PABB) plus interventional ductal stenting. We present a case of a transcatheter approach for pulmonary flow restriction (PFR) that was accomplished by modifying a Medtronic microvascular plug (MVP)TM.
Clinical Case
A 2.4 kg neonate was diagnosed with HLHS and significantly dysplastic tricuspid and pulmonary valves with pulmonary stenosis, tricuspid stenosis, and regurgitation. He was not considered a candidate for Norwood operation and the family was contemplating transplantation as an option. He started developing sequelae of unbalanced pulmonary and systemic blood flow, therefore he underwent placement of transcatheter PFR as alternative to PABB. Baseline hemodynamics showed a Qp:Qs of 3.5:1. The branch pulmonary arteries were accessed with a JR3 catheter via a femoral venous approach. Once in each branch pulmonary artery a hand injection was performed to determine the vessel size. After angiography was performed the PFR was placed in each branch. PFR was achieved using a modified MVP. These were modified by removing one of the PTFE diamonds via sharp dissection prior to deployment. Based on the size of the vessels a 5Q plug was modified and deployed in each of the branch pulmonary arteries. After placement of the PFR his oxygen saturation decreased from the 92-96% to 85-88% with an increase in renal NIRS from mid 20’s to mid 50’s. His PaO2 decreased from 52 mmHg to 38 mmHg. Twelve days later he underwent repeat diagnostic catheterization which showed a Qp:Qs of 0.88:1 and a mean pulmonary vein wedge pressures of 16-17 mmHg.
Discussion
This case suggests transcatheter branch pulmonary artery flow restriction is a safe and feasible alternative to PABB as initial palliation strategy. It may benefit patients in whom primary Norwood procedure would have increased risks or who will undergo primary transplant. More experience using this technique, early, and mid-term outcomes are needed to further determine if this is a viable treatment strategy for palliation of critical congenital heart disease.