TY - JOUR
T1 - Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum
T2 - International Fetal Cardiac Intervention Registry
AU - Hogan, Whitnee J.
AU - Grinenco, Sofia
AU - Armstrong, Aimee
AU - Devlieger, Roland
AU - Dangel, Joanna
AU - Ferrer, Queralt
AU - Frommelt, Michele
AU - Galindo, Alberto
AU - Gardiner, Helena
AU - Gelehrter, Sarah
AU - Herberg, Ulrike
AU - Howley, Lisa
AU - Jaeggi, Edgar
AU - Miranda, Joana
AU - Morris, Shaine A.
AU - Oepkes, Dick
AU - Pedra, Simone
AU - Peterson, Renuka
AU - Sholler, Gary
AU - Simpson, John
AU - Strainic, James
AU - Vigneswarran, Trisha V.
AU - Wacker-Gussmann, Annette
AU - Moon-Grady, Anita J.
N1 - Publisher Copyright:
© 2020 S. Karger AG, Basel.
PY - 2020/10/1
Y1 - 2020/10/1
N2 - 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.
AB - 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.
KW - Congenital heart defect
KW - Fetal cardiac intervention
KW - Fetal echocardiography
KW - Pulmonary atresia with intact ventricular septum
KW - Valvuloplasty
UR - http://www.scopus.com/inward/record.url?scp=85088694218&partnerID=8YFLogxK
U2 - 10.1159/000508045
DO - 10.1159/000508045
M3 - Article
C2 - 32634804
AN - SCOPUS:85088694218
SN - 1015-3837
VL - 47
SP - 731
EP - 739
JO - Fetal Diagnosis and Therapy
JF - Fetal Diagnosis and Therapy
IS - 10
ER -