Ramiro W Lizano Santamaria1, Matthew J Gillespie1,2, Yoav Dori1,2, Jonathan J Rome1,2, Andrew C Glatz1,2,3. 1. Division of Cardiology, The Children's Hospital of Philadelphia, Pennsylvania. 2. Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. 3. Center for Clinical Epidemiology and Biostatistics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
Abstract
BACKGROUND: Symptomatic infants with unrestrictive ventricular septal defect (VSD) or single ventricle with severe pulmonary stenosis (PS) are typically treated surgically. High surgical risk infants may benefit from catheter-based palliative pulmonary valvuloplasty. METHODS: We retrospectively reviewed all patients with severe PS associated with unrestrictive VSD or single ventricle who underwent palliative pulmonary valvuloplasty as initial management and describe outcomes. RESULTS: From 2000 to 2013, 16 patients met inclusion criteria and underwent valvuloplasty at a median age and weight of 25 (range 1-352) days and 3.65 (range 2.2-12) kg. Common diagnoses included tetralogy of Fallot (50%) and double-outlet right ventricle (25%). In all, the indication was cyanosis. Reasons to defer surgery included low weight (37.5%), preference for complex operation at older age (31.25%) and significant comorbidity (18.75%). Following valvuloplasty, significant increases in oxygen saturation (83.1±8.8 vs. 74.7±8.7%, P=0.008) and QP:QS (1.6±0.5 vs. 0.8±0.3, P=0.0005) occurred. All two ventricle patients (n=10) ultimately had complete operative repair at a median of 125 (range 27-382) days after valvuloplasty, during which time there was 20.3±9.2 g/day of weight gain without change in oxygen saturation. Single ventricle patients who underwent cavopulmonary connection (n=3) had similar weight gain and palliation time, but with a decrease in oxygen saturation. Two patients required reintervention to augment QP prior to definitive repair. CONCLUSIONS: In select infants with unrestrictive VSD or single ventricle associated with severe PS, palliative pulmonary valvuloplasty provides an effective and durable method of increasing oxygen saturation until definitive surgical treatment can be performed.
BACKGROUND: Symptomatic infants with unrestrictive ventricular septal defect (VSD) or single ventricle with severe pulmonary stenosis (PS) are typically treated surgically. High surgical risk infants may benefit from catheter-based palliative pulmonary valvuloplasty. METHODS: We retrospectively reviewed all patients with severe PS associated with unrestrictive VSD or single ventricle who underwent palliative pulmonary valvuloplasty as initial management and describe outcomes. RESULTS: From 2000 to 2013, 16 patients met inclusion criteria and underwent valvuloplasty at a median age and weight of 25 (range 1-352) days and 3.65 (range 2.2-12) kg. Common diagnoses included tetralogy of Fallot (50%) and double-outlet right ventricle (25%). In all, the indication was cyanosis. Reasons to defer surgery included low weight (37.5%), preference for complex operation at older age (31.25%) and significant comorbidity (18.75%). Following valvuloplasty, significant increases in oxygen saturation (83.1±8.8 vs. 74.7±8.7%, P=0.008) and QP:QS (1.6±0.5 vs. 0.8±0.3, P=0.0005) occurred. All two ventricle patients (n=10) ultimately had complete operative repair at a median of 125 (range 27-382) days after valvuloplasty, during which time there was 20.3±9.2 g/day of weight gain without change in oxygen saturation. Single ventricle patients who underwent cavopulmonary connection (n=3) had similar weight gain and palliation time, but with a decrease in oxygen saturation. Two patients required reintervention to augment QP prior to definitive repair. CONCLUSIONS: In select infants with unrestrictive VSD or single ventricle associated with severe PS, palliative pulmonary valvuloplasty provides an effective and durable method of increasing oxygen saturation until definitive surgical treatment can be performed.
Authors: Mark A Law; Andrew C Glatz; Jennifer C Romano; Paul J Chai; Christopher E Mascio; Christopher J Petit; Courtney E McCracken; Michael S Kelleman; George T Nicholson; Jeffery J Meadows; Jeffrey D Zampi; Shabana Shahanavaz; Sarosh P Batlivala; Joelle Pettus; Amy L Pajk; Kristal M Hock; Bryan H Goldstein; Athar M Qureshi Journal: Pediatr Cardiol Date: 2022-04-05 Impact factor: 1.838