Pulmonary Atresia with Intact Ventricular Septum: From Radiofrequency Perforation to Transcatheter Pulmonary Valve
Presenter
Nicholas Dreger, MD, Rady Children's Hospital, San Diego, CA
Nicholas Dreger, MD1, Asimina Courelli, BS2, Nicky L Herrick, MD2, Peter W. Guyon Jr., M.D.1, Kanishka Ratnayaka, MD3, John W. Moore, M.D.1 and Howaida El-Said, M.D., Ph.D., FSCAI4, (1)Rady Children's Hospital, San Diego, CA, (2)UC San Diego Health, San Diego, CA, (3)-, San Diego, CA, (4)The University of California, San Diego, San Diego, CA
Keywords: Congenital Heart Disease (CHD) and TPVR/Pulmonary Valve
Background
Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare cyanotic congenital heart lesion traditionally palliated with multiple procedures and surgeries. Entirely non-surgical transcatheter management from infancy to adulthood may be possible; however, the pathway from neonatal radiofrequency (RF) pulmonary valve perforation to later transcatheter pulmonary valve replacement (TPV) is not well described.
Methods
This retrospective study was performed at a large pediatric center between 2007-2018. All patients with PA/IVS who were managed exclusively via catheterization-based interventions as neonates (RF perforation, pulmonary valvuloplasty, or ductal stenting) were analyzed. Demographic, procedural and clinical data were collected.
Results
Fifteen patients had exclusively catheterization-based RV decompression as neonates, seven (7/15) of whom did not require subsequent surgery. Six (6/15) patients required a right ventricular outflow tract (RVOT) augmentation later in life; all were born before 2013. All six later had a TPV placed. Two (2/15) patients underwent a surgical Glenn shunt. Of the seven patients that never had surgery, three have since undergone a TPV, and four are awaiting candidacy for TPV placement. No patients with PA/IVS have had an RVOT augmentation at our institution since 2012.
Conclusions
Patients with PA/IVS that underwent catheterization-based neonatal interventions can make it to TPV without requiring surgery. At our institution, there has been a shift in management over the last eight years favoring entirely non-surgical transcatheter management from infancy to adulthood.