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

doi:10.1159/000508045

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

Research output: Contribution to journal › Article › peer-review

Abstract

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.

Original language	English (US)
Pages (from-to)	731-739
Number of pages	9
Journal	Fetal Diagnosis and Therapy
Volume	47
Issue number	10
DOIs	https://doi.org/10.1159/000508045
State	Published - Oct 1 2020
Externally published	Yes

Keywords

Congenital heart defect
Fetal cardiac intervention
Fetal echocardiography
Pulmonary atresia with intact ventricular septum
Valvuloplasty

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Embryology
Radiology Nuclear Medicine and imaging
Obstetrics and Gynecology

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10.1159/000508045

Cite this

Hogan, W. J., Grinenco, S., Armstrong, A., Devlieger, R., Dangel, J., Ferrer, Q., Frommelt, M., Galindo, A., Gardiner, H., Gelehrter, S., Herberg, U., Howley, L., Jaeggi, E., Miranda, J., Morris, S. A., Oepkes, D., Pedra, S., Peterson, R., Sholler, G., ... Moon-Grady, A. J. (2020). Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum: International Fetal Cardiac Intervention Registry. Fetal Diagnosis and Therapy, 47(10), 731-739. https://doi.org/10.1159/000508045

Hogan, WJ, Grinenco, S, Armstrong, A, Devlieger, R, Dangel, J, Ferrer, Q, Frommelt, M, Galindo, A, Gardiner, H, Gelehrter, S, Herberg, U, Howley, L, Jaeggi, E, Miranda, J, Morris, SA, Oepkes, D, Pedra, S, Peterson, R, Sholler, G, Simpson, J, Strainic, J, Vigneswarran, TV, Wacker-Gussmann, A & Moon-Grady, AJ 2020, 'Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum: International Fetal Cardiac Intervention Registry', Fetal Diagnosis and Therapy, vol. 47, no. 10, pp. 731-739. https://doi.org/10.1159/000508045

@article{b263c4fe16024f0c9719b8626b477c9c,

title = "Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum: International Fetal Cardiac Intervention Registry",

abstract = "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",

author = "Hogan, \{Whitnee J.\} and Sofia Grinenco and Aimee Armstrong and Roland Devlieger and Joanna Dangel and Queralt Ferrer and Michele Frommelt and Alberto Galindo and Helena Gardiner and Sarah Gelehrter and Ulrike Herberg and Lisa Howley and Edgar Jaeggi and Joana Miranda and Morris, \{Shaine A.\} and Dick Oepkes and Simone Pedra and Renuka Peterson and Gary Sholler and John Simpson and James Strainic and Vigneswarran, \{Trisha V.\} and Annette Wacker-Gussmann and Moon-Grady, \{Anita J.\}",

note = "Publisher Copyright: {\textcopyright} 2020 S. Karger AG, Basel.",

year = "2020",

month = oct,

day = "1",

doi = "10.1159/000508045",

language = "English (US)",

volume = "47",

pages = "731--739",

journal = "Fetal Diagnosis and Therapy",

issn = "1015-3837",

publisher = "S. Karger AG",

number = "10",

}

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.

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 - https://www.scopus.com/pages/publications/85088694218

UR - https://www.scopus.com/pages/publications/85088694218#tab=citedBy

U2 - 10.1159/000508045

DO - 10.1159/000508045

M3 - Article

C2 - 32634804

AN - SCOPUS:85088694218

SN - 1015-3837

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JO - Fetal Diagnosis and Therapy

JF - Fetal Diagnosis and Therapy

IS - 10

ER -

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

Abstract

Keywords

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