1. Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum: International Fetal Cardiac Intervention Registry
- Author
-
Renuka E. Peterson, Roland Devlieger, Shaine A. Morris, Queralt Ferrer, Gary F. Sholler, Sarah Gelehrter, Dick Oepkes, John M. Simpson, Joanna Dangel, Aimee K. Armstrong, Alberto Galindo, Edgar Jaeggi, Joana O. Miranda, Michele A. Frommelt, Annette Wacker-Gussmann, James Strainic, Helena M. Gardiner, Lisa Howley, Ulrike Herberg, Trisha V Vigneswarran, Simone Rolim Fernandes Fontes Pedra, Whitnee Hogan, Sofía Grinenco, and Anita J. Moon-Grady
- Subjects
Embryology ,medicine.medical_specialty ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine ,Radiology, Nuclear Medicine and imaging ,030212 general & internal medicine ,Pregnancy ,Fetus ,030219 obstetrics & reproductive medicine ,Tricuspid valve ,Fetal cardiac intervention ,Fetal echocardiography ,medicine.diagnostic_test ,business.industry ,Congenital heart defect ,Pulmonary atresia with intact ventricular septum ,Obstetrics and Gynecology ,Gestational age ,General Medicine ,medicine.disease ,Stenosis ,Valvuloplasty ,medicine.anatomical_structure ,Pulmonary valve ,Pediatrics, Perinatology and Child Health ,Cardiology ,business ,Pulmonary atresia - Abstract
Introduction: Invasive fetal cardiac intervention (FCI) for pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (PS) has been performed with small single-institution series reporting technical and physiological success. We present the first multicenter experience. Objectives: Describe fetal and maternal characteristics of those being evaluated for FCI, including pregnancy/neonatal outcome data using the International Fetal Cardiac Intervention Registry (IFCIR). Methods: We queried the IFCIR for PAIVS/PS cases evaluated from January 2001 to April 2018 and reviewed maternal/fetal characteristics, procedural details, pregnancy and neonatal outcomes. Data were analyzed using standard descriptive statistics. Results: Of the 84 maternal/fetal dyads in the registry, 58 underwent pulmonary valvuloplasty at a median gestational age of 26.1 (21.9–31.0) weeks. Characteristics of fetuses undergoing FCI varied in terms of tricuspid valve (TV) size, TV regurgitation, and pulmonary valve patency. There were fetal complications in 55% of cases, including 7 deaths and 2 delayed fetal losses. Among those who underwent successful FCI, the absolute measurement of the TV increased by 0.32 (±0.17) mm/week from intervention to birth. Among 60 liveborn with known outcome, there was a higher percentage having a biventricular circulation following successful FCI (87 vs. 43%). Conclusions: Our data suggest a possible benefit to fetal therapy for PAIVS/PS, though rates of technically unsuccessful procedures and procedure-related complications, including fetal loss were substantial. FCI criteria are extremely variable, making direct comparison to nonintervention patients challenging and potentially biased. More uniform FCI criteria for fetuses with PAIVS/PS are needed to avoid unnecessary procedures, expose only fetuses most likely to sustain a benefit, and to enable comparisons to be made with nonintervention patients.
- Published
- 2020