Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum: International Fetal Cardiac Intervention Registry

Whitnee J Hogan; Sofia Grinenco; Aimee Armstrong; Roland Devlieger; Joanna Dangel; Queralt Ferrer; Michele Frommelt; Alberto Galindo; Helena Gardiner; Sarah Gelehrter; Ulrike Herberg; Lisa Howley; Edgar Jaeggi; Joana Miranda; Shaine A Morris; Dick Oepkes; Simone Pedra; Renuka Peterson; Gary Sholler; John Simpson; James Strainic; Trisha V Vigneswarran; Annette Wacker-Gussmann; Anita J Moon-Grady; for the IFCIR Participants

doi:10.1159/000508045

Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum: International Fetal Cardiac Intervention Registry

Fetal Diagn Ther. 2020 Jul 7:1-9. doi: 10.1159/000508045. Online ahead of print.

Authors

Whitnee J Hogan¹, Sofia Grinenco², Aimee Armstrong³, Roland Devlieger⁴, Joanna Dangel⁵, Queralt Ferrer⁶, Michele Frommelt⁷, Alberto Galindo⁸, Helena Gardiner⁹, Sarah Gelehrter¹⁰, Ulrike Herberg¹¹, Lisa Howley¹², Edgar Jaeggi¹³, Joana Miranda¹⁴, Shaine A Morris¹⁵, Dick Oepkes¹⁶, Simone Pedra¹⁷, Renuka Peterson¹⁸, Gary Sholler¹⁹, John Simpson²⁰, James Strainic²¹, Trisha V Vigneswarran²⁰, Annette Wacker-Gussmann²², Anita J Moon-Grady²³; for the IFCIR Participants

Affiliations

¹ University of California-San Francisco, San Francisco, California, USA, whitnee.hogan@ucsf.edu.
² Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.
³ Nationwide Children's Hospital, Columbus, Ohio, USA.
⁴ Department of Obstetrics and Gynaecology, University Hospitals KU Leuven, Leuven, Belgium.
⁵ Department of Perinatal Cardiology and Congenital Anomalies, Centre of Postgraduate Medical Education, Warsaw, Poland.
⁶ Vall d'Hebron Hospital, Barcelona, Spain.
⁷ Children's Hospital of Wisconsin, Milwaukee, Wisconsin, USA.
⁸ Hospital Universitario, Universidad Complutense de Madrid, Madrid, Spain.
⁹ The Fetal Center at Children's Memorial Hermann Hospital, Houston, Texas, USA.
¹⁰ C. S. Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan, USA.
¹¹ University of Bonn, Bonn, Germany.
¹² Children's Hospital Colorado, Aurora, Colorado, USA.
¹³ Hospital for Sick Children, Toronto, Ontario, Canada.
¹⁴ Centro Hospitalar São João, Porto, Portugal.
¹⁵ Baylor College of Medicine, Houston, Texas, USA.
¹⁶ Leiden University Medical Center, Leiden, The Netherlands.
¹⁷ Hospital do Coração, São Paulo, Brazil.
¹⁸ Saint Louis University, St Louis, Missouri, USA.
¹⁹ Heart Center for Children, Children's Hospital at Westmead and University of Sydney, Sydney, New South Wales, Australia.
²⁰ Evelina London Children's Hospital, Guy's and St. Thomas' NHS Foundation Trust Hospitals, London, United Kingdom.
²¹ Rainbow Babies and Children's Hospital Division of Pediatric Cardiology, University Hospitals, Cleveland, Ohio, USA.
²² German Heart Center, Department of Pediatric Cardiology and Adult Congenital Heart Disease, Munich, Germany.
²³ University of California-San Francisco, San Francisco, California, USA.

PMID: 32634804
DOI: 10.1159/000508045

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.

Keywords: Congenital heart defect; Fetal cardiac intervention; Fetal echocardiography; Pulmonary atresia with intact ventricular septum; Valvuloplasty.

Grants and funding

II-LA-0716-20001/DH_/Department of Health/United Kingdom