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. 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.
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.
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.