Prenatal Echocardiographic Assessment of Right Aortic Arch

Cosimo Marco Campanale, Luciano Pasquini, Teresa Pia Santangelo, Fiore Salvatore Iorio, Pietro Bagolan, Stephen P Sanders, Alessandra Toscano

Research output: Contribution to journalArticle

Abstract

OBJECTIVES: To report our experience with fetal diagnosis of right aortic arch (RAA) variants based on the ductus arteriosus (DA) anatomy and brachiocephalic vessel branching pattern (BVBP) respectively to the trachea and to establish whether the echocardiographic "V-shaped" or "U-shaped" appearances of the fetal upper mediastinum were sufficiently accurate for fetal aortic arch (AA) assessment.

METHODS: We retrospectively identified patients with a fetal diagnosis of RAA referred to our tertiary center from 2011 to 2017 and selected those who had post-natal confirmation of AA anatomy. Prenatal and postnatal medical records, including echocardiographic and CT/MRI scan reports, were reviewed and cardiac and extracardiac abnormalities and the results of genetic testing were recorded.

RESULTS: Among 55 consecutive fetuses with a diagnosis of RAA, 6 patients were lost to follow-up, 1 pregnancy was interrupted and 3 patients were excluded for lack of post-natal confirmation. In the remaining 45 patients the AA anatomy was studied after birth by CT in 39, MRI in 1, and only by direct exploration at cardiac surgery in 5 patients. Fetal "U-shaped" appearance was found in 37/45 patients (82%) and all had a complete vascular ring (CVR). Of those, at post-natal confirmation, 21/37 patients (57%) had RAA with Kommerell diverticulum, left posterior ductus arteriosus (LPDA) and aberrant left subclavian artery (ALSA) (RAA/LPDA/ALSA), 11/37 patients (30%) double aortic arch (DAA), 4/37 patients (11%) RAA with Kommerell diverticulum, LPDA and mirror image (MI) branching (RAA/LPDA/MI), 1/37 patient (2%) RAA with Kommerell diverticulum, LPDA and aberrant innominate artery (ALIA) (RAA/LPDA/ALIA). Fetal "V-shaped" appearance was found in 3/45 patients (7%) and all had RAA with right DA (RDA) not forming a CVR and MI branching (RAA/RDA/MI). In 5/45 fetuses (11%) with neither "U-" nor "V-shaped" appearance a RAA with left anterior ductus arteriosus (LADA) arising from the left innominate artery (IA) and MI branching (RAA/LADA/MI) was found, not forming CVR. Twelve patients (12/45 - 27%) had a congenital heart defect (CHD), among which "RAA forming CVR" (fetal "U-shaped" appearance) was associated with septal defects in 6/37 (16%) while "RAA not forming CVR" (fetal "V-shaped" appearance or none) was associated with major CHD in 6/8 (75%).

CONCLUSIONS: We conclude that "V-shaped" appearance only means that the transverse AA and DA run together on the same side of the thorax (trachea) and "U-shaped" appearance is always the sign of a CVR. Among CVR, RAA/LPDA/MI is more frequent than previously described. Finally, "RAA forming CVR" is usually not associated with complex CHD, as opposed to "RAA not forming CVR". This article is protected by copyright. All rights reserved.

Original languageEnglish
JournalUltrasound in Obstetrics and Gynecology
DOIs
Publication statusE-pub ahead of print - Aug 20 2018

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Thoracic Aorta
Ductus Arteriosus
Blood Vessels
Congenital Heart Defects
Diverticulum
Brachiocephalic Trunk
Anatomy
Patient Rights
Trachea
Fetus

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Prenatal Echocardiographic Assessment of Right Aortic Arch. / Campanale, Cosimo Marco; Pasquini, Luciano; Santangelo, Teresa Pia; Iorio, Fiore Salvatore; Bagolan, Pietro; Sanders, Stephen P; Toscano, Alessandra.

In: Ultrasound in Obstetrics and Gynecology, 20.08.2018.

Research output: Contribution to journalArticle

Campanale, Cosimo Marco ; Pasquini, Luciano ; Santangelo, Teresa Pia ; Iorio, Fiore Salvatore ; Bagolan, Pietro ; Sanders, Stephen P ; Toscano, Alessandra. / Prenatal Echocardiographic Assessment of Right Aortic Arch. In: Ultrasound in Obstetrics and Gynecology. 2018.
@article{a389a5079d8648ac8b6d9da6e4a0b7c4,
title = "Prenatal Echocardiographic Assessment of Right Aortic Arch",
abstract = "OBJECTIVES: To report our experience with fetal diagnosis of right aortic arch (RAA) variants based on the ductus arteriosus (DA) anatomy and brachiocephalic vessel branching pattern (BVBP) respectively to the trachea and to establish whether the echocardiographic {"}V-shaped{"} or {"}U-shaped{"} appearances of the fetal upper mediastinum were sufficiently accurate for fetal aortic arch (AA) assessment.METHODS: We retrospectively identified patients with a fetal diagnosis of RAA referred to our tertiary center from 2011 to 2017 and selected those who had post-natal confirmation of AA anatomy. Prenatal and postnatal medical records, including echocardiographic and CT/MRI scan reports, were reviewed and cardiac and extracardiac abnormalities and the results of genetic testing were recorded.RESULTS: Among 55 consecutive fetuses with a diagnosis of RAA, 6 patients were lost to follow-up, 1 pregnancy was interrupted and 3 patients were excluded for lack of post-natal confirmation. In the remaining 45 patients the AA anatomy was studied after birth by CT in 39, MRI in 1, and only by direct exploration at cardiac surgery in 5 patients. Fetal {"}U-shaped{"} appearance was found in 37/45 patients (82{\%}) and all had a complete vascular ring (CVR). Of those, at post-natal confirmation, 21/37 patients (57{\%}) had RAA with Kommerell diverticulum, left posterior ductus arteriosus (LPDA) and aberrant left subclavian artery (ALSA) (RAA/LPDA/ALSA), 11/37 patients (30{\%}) double aortic arch (DAA), 4/37 patients (11{\%}) RAA with Kommerell diverticulum, LPDA and mirror image (MI) branching (RAA/LPDA/MI), 1/37 patient (2{\%}) RAA with Kommerell diverticulum, LPDA and aberrant innominate artery (ALIA) (RAA/LPDA/ALIA). Fetal {"}V-shaped{"} appearance was found in 3/45 patients (7{\%}) and all had RAA with right DA (RDA) not forming a CVR and MI branching (RAA/RDA/MI). In 5/45 fetuses (11{\%}) with neither {"}U-{"} nor {"}V-shaped{"} appearance a RAA with left anterior ductus arteriosus (LADA) arising from the left innominate artery (IA) and MI branching (RAA/LADA/MI) was found, not forming CVR. Twelve patients (12/45 - 27{\%}) had a congenital heart defect (CHD), among which {"}RAA forming CVR{"} (fetal {"}U-shaped{"} appearance) was associated with septal defects in 6/37 (16{\%}) while {"}RAA not forming CVR{"} (fetal {"}V-shaped{"} appearance or none) was associated with major CHD in 6/8 (75{\%}).CONCLUSIONS: We conclude that {"}V-shaped{"} appearance only means that the transverse AA and DA run together on the same side of the thorax (trachea) and {"}U-shaped{"} appearance is always the sign of a CVR. Among CVR, RAA/LPDA/MI is more frequent than previously described. Finally, {"}RAA forming CVR{"} is usually not associated with complex CHD, as opposed to {"}RAA not forming CVR{"}. This article is protected by copyright. All rights reserved.",
author = "Campanale, {Cosimo Marco} and Luciano Pasquini and Santangelo, {Teresa Pia} and Iorio, {Fiore Salvatore} and Pietro Bagolan and Sanders, {Stephen P} and Alessandra Toscano",
note = "This article is protected by copyright. All rights reserved.",
year = "2018",
month = "8",
day = "20",
doi = "10.1002/uog.20098",
language = "English",
journal = "Ultrasound in Obstetrics and Gynecology",
issn = "0960-7692",
publisher = "John Wiley and Sons Ltd",

}

TY - JOUR

T1 - Prenatal Echocardiographic Assessment of Right Aortic Arch

AU - Campanale, Cosimo Marco

AU - Pasquini, Luciano

AU - Santangelo, Teresa Pia

AU - Iorio, Fiore Salvatore

AU - Bagolan, Pietro

AU - Sanders, Stephen P

AU - Toscano, Alessandra

N1 - This article is protected by copyright. All rights reserved.

PY - 2018/8/20

Y1 - 2018/8/20

N2 - OBJECTIVES: To report our experience with fetal diagnosis of right aortic arch (RAA) variants based on the ductus arteriosus (DA) anatomy and brachiocephalic vessel branching pattern (BVBP) respectively to the trachea and to establish whether the echocardiographic "V-shaped" or "U-shaped" appearances of the fetal upper mediastinum were sufficiently accurate for fetal aortic arch (AA) assessment.METHODS: We retrospectively identified patients with a fetal diagnosis of RAA referred to our tertiary center from 2011 to 2017 and selected those who had post-natal confirmation of AA anatomy. Prenatal and postnatal medical records, including echocardiographic and CT/MRI scan reports, were reviewed and cardiac and extracardiac abnormalities and the results of genetic testing were recorded.RESULTS: Among 55 consecutive fetuses with a diagnosis of RAA, 6 patients were lost to follow-up, 1 pregnancy was interrupted and 3 patients were excluded for lack of post-natal confirmation. In the remaining 45 patients the AA anatomy was studied after birth by CT in 39, MRI in 1, and only by direct exploration at cardiac surgery in 5 patients. Fetal "U-shaped" appearance was found in 37/45 patients (82%) and all had a complete vascular ring (CVR). Of those, at post-natal confirmation, 21/37 patients (57%) had RAA with Kommerell diverticulum, left posterior ductus arteriosus (LPDA) and aberrant left subclavian artery (ALSA) (RAA/LPDA/ALSA), 11/37 patients (30%) double aortic arch (DAA), 4/37 patients (11%) RAA with Kommerell diverticulum, LPDA and mirror image (MI) branching (RAA/LPDA/MI), 1/37 patient (2%) RAA with Kommerell diverticulum, LPDA and aberrant innominate artery (ALIA) (RAA/LPDA/ALIA). Fetal "V-shaped" appearance was found in 3/45 patients (7%) and all had RAA with right DA (RDA) not forming a CVR and MI branching (RAA/RDA/MI). In 5/45 fetuses (11%) with neither "U-" nor "V-shaped" appearance a RAA with left anterior ductus arteriosus (LADA) arising from the left innominate artery (IA) and MI branching (RAA/LADA/MI) was found, not forming CVR. Twelve patients (12/45 - 27%) had a congenital heart defect (CHD), among which "RAA forming CVR" (fetal "U-shaped" appearance) was associated with septal defects in 6/37 (16%) while "RAA not forming CVR" (fetal "V-shaped" appearance or none) was associated with major CHD in 6/8 (75%).CONCLUSIONS: We conclude that "V-shaped" appearance only means that the transverse AA and DA run together on the same side of the thorax (trachea) and "U-shaped" appearance is always the sign of a CVR. Among CVR, RAA/LPDA/MI is more frequent than previously described. Finally, "RAA forming CVR" is usually not associated with complex CHD, as opposed to "RAA not forming CVR". This article is protected by copyright. All rights reserved.

AB - OBJECTIVES: To report our experience with fetal diagnosis of right aortic arch (RAA) variants based on the ductus arteriosus (DA) anatomy and brachiocephalic vessel branching pattern (BVBP) respectively to the trachea and to establish whether the echocardiographic "V-shaped" or "U-shaped" appearances of the fetal upper mediastinum were sufficiently accurate for fetal aortic arch (AA) assessment.METHODS: We retrospectively identified patients with a fetal diagnosis of RAA referred to our tertiary center from 2011 to 2017 and selected those who had post-natal confirmation of AA anatomy. Prenatal and postnatal medical records, including echocardiographic and CT/MRI scan reports, were reviewed and cardiac and extracardiac abnormalities and the results of genetic testing were recorded.RESULTS: Among 55 consecutive fetuses with a diagnosis of RAA, 6 patients were lost to follow-up, 1 pregnancy was interrupted and 3 patients were excluded for lack of post-natal confirmation. In the remaining 45 patients the AA anatomy was studied after birth by CT in 39, MRI in 1, and only by direct exploration at cardiac surgery in 5 patients. Fetal "U-shaped" appearance was found in 37/45 patients (82%) and all had a complete vascular ring (CVR). Of those, at post-natal confirmation, 21/37 patients (57%) had RAA with Kommerell diverticulum, left posterior ductus arteriosus (LPDA) and aberrant left subclavian artery (ALSA) (RAA/LPDA/ALSA), 11/37 patients (30%) double aortic arch (DAA), 4/37 patients (11%) RAA with Kommerell diverticulum, LPDA and mirror image (MI) branching (RAA/LPDA/MI), 1/37 patient (2%) RAA with Kommerell diverticulum, LPDA and aberrant innominate artery (ALIA) (RAA/LPDA/ALIA). Fetal "V-shaped" appearance was found in 3/45 patients (7%) and all had RAA with right DA (RDA) not forming a CVR and MI branching (RAA/RDA/MI). In 5/45 fetuses (11%) with neither "U-" nor "V-shaped" appearance a RAA with left anterior ductus arteriosus (LADA) arising from the left innominate artery (IA) and MI branching (RAA/LADA/MI) was found, not forming CVR. Twelve patients (12/45 - 27%) had a congenital heart defect (CHD), among which "RAA forming CVR" (fetal "U-shaped" appearance) was associated with septal defects in 6/37 (16%) while "RAA not forming CVR" (fetal "V-shaped" appearance or none) was associated with major CHD in 6/8 (75%).CONCLUSIONS: We conclude that "V-shaped" appearance only means that the transverse AA and DA run together on the same side of the thorax (trachea) and "U-shaped" appearance is always the sign of a CVR. Among CVR, RAA/LPDA/MI is more frequent than previously described. Finally, "RAA forming CVR" is usually not associated with complex CHD, as opposed to "RAA not forming CVR". This article is protected by copyright. All rights reserved.

U2 - 10.1002/uog.20098

DO - 10.1002/uog.20098

M3 - Article

C2 - 30125417

JO - Ultrasound in Obstetrics and Gynecology

JF - Ultrasound in Obstetrics and Gynecology

SN - 0960-7692

ER -