Fetal Echocardiography

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 Fetal
Echocardiography
Second Edition

Julia A. Drose, BA, RDMS, RDCS, RVT

Associate Professor
Department of Radiology
Chief Sonographer
Divisions of Diagnostic Ultrasound and Prenatal Diagnosis & Genetics
University of Colorado Hospital
Denver, Colorado
11830 Westline Industrial Drive
St. Louis, Missouri 63146

FETAL ECHOCARDIOGRAPHY, SECOND EDITION ISBN: 978-1-4160-5669-0

Copyright © 2010, 1998 by Saunders, an imprint of Elsevier Inc.

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Notice

Neither the Publisher nor the Author assumes any responsibility for any loss or injury and/
or damage to persons or property arising out of or related to any use of the material
contained in this book. It is the responsibility of the treating practitioner, relying on
independent expertise and knowledge of the patient, to determine the best treatment and
method of application for the patient.
The Publisher

Library of Congress Cataloging-in-Publication Data

Fetal echocardiography / [edited by] Julia A. Drose. — 2nd ed.

   p. ; cm.
Includes bibliographical references and index.
ISBN 978-1-4160-5669-0 (hardcover : alk. paper) 1. Fetal heart—Ultrasonic imaging.
I. Drose, Julia A.
[DNLM: 1. Fetal Heart—ultrasonography. 2. Heart Defects, Congenital—ultrasonography.
3. Echocardiography. 4. Ultrasonography, Prenatal. WQ 210.5 F4192 2010]
RG628.3.E34F48 2010
618.3′26107543—dc22
2008045320

ISBN: 978-1-4160-5669-0

Vice President and Publisher: Andrew Allen

Publisher: Jeanne Olson
Associate Developmental Editor: Luke Held
Publishing Services Manager: Patricia Tannian
Project Manager: Claire Kramer
Designer: Charles Seibel

Working together to grow

libraries in developing countries
Printed in China www.elsevier.com | www.bookaid.org | www.sabre.org

Last digit is the print number: 9 8 7 6 5 4 3 2

For Mackenzie, Caroline, Brady, and Taylor

You are fearless, powerful, and utterly amazing.

You are the loves of my life.
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 Contributors

Amanda K. Auckland, BS, RDMS, RDCS, RVT Julia A. Drose, BA, RDMS, RDCS, RVT
Sonographer Associate Professor
Division of Ultrasound Department of Radiology
University of Colorado Hospital Chief Sonographer
Aurora, Colorado Divisions of Diagnostic Ultrasound and Prenatal
Diagnosis & Genetics
Tina M. Bachman, BS, RDMS, RDCS, RVT
University of Colorado Hospital
Sonographer Denver, Colorado
Division of Ultrasound
University of Colorado Hospital Marisa R. Lydia, RDMS, RDCS, RVT
Aurora, Colorado Sonographer
Heidi S. Barrett, RDMS, RVT Obstetrix Medical Group
Sonographer Lone Tree, Colorado
Division of Ultrasound
University of Colorado Hospital Sarah H. Martinez, BS, RDMS, RDCS, RVT
Aurora, Colorado Clinical Applications Specialist
GE Healthcare
Teresa M. Bieker, MBA-H, RDMS, RDCS, RVT Denver, Colorado
Lead Sonographer
Division of Ultrasound Kristin E. McKinney, MD
University of Colorado Hospital Assistant Professor of Radiology
Aurora, Colorado Division of Ultrasound
Danielle M. Bolger, RDMS, RVT University of Colorado Hospital
Aurora, Colorado
Sonographer
Division of Ultrasound
University of Colorado Hospital
Aurora, Colorado

vii
viii Contributors

Cynthia L. Rapp, BS, RDMS, RDCS Elizabeth R. Stamm, MD

Vice President of Clinical Program Development Associate Professor of Radiology
The Medipattern Corporation University of Colorado Hospital
Toronto, Ontario, Canada Aurora, Colorado

Paul D. Russ, MD Karrie L. Villavicencio, MD

Professor of Radiology Assistant Professor of Pediatric Cardiology
University of Colorado Hospital University of Colorado Hospital
Aurora, Colorado Denver, Colorado

Elizabeth M. Shaffer, MD Marsha Wheeler, MD

Associate Professor of Pediatrics Associate Professor of Obstetrics and Gynecology
The Children’s Hospital University of Colorado Hospital
Denver, Colorado Denver, Colorado

Britt C. Smyth, BA, RDMS, RDCS, RVT Adel K. Younoszai, MD

Sonographer Assistant Professor of Pediatrics
Division of Ultrasound Director of Cardiac Imaging
University of Colorado Hospital The Children’s Hospital
Aurora, Colorado Denver, Colorado
 Reviewers

Beth Anderhub, MEd, RDMS, RSDMS Susanna Ovel, RT, RDMS, RVT
Professor and Program Director of Diagnostic Sonographer III
Medical Sonography Radiological Associates of Sacramento
St. Louis Community College Sacramento, California
St. Louis, Missouri
David Rands, BA, RDMS, RDCS, RVT, ARRT(R)
Marcia Cooper, MSRS, RT(R)(M)(CT)(QM), RDMS,
Instructor
RVT
University of Arkansas Medical Sciences
Clinical Coordinator and Associate of Imaging Little Rock, Arkansas
Sciences
Morehead State University Anthony Swartz, BS, RT(R), RDMS
Morehead, Kentucky Lead Sonography and Manager
Janice Dolk, MA, RT(R), RDMS Maternal-Fetal Medicine Department
Sonography Consultant and Lab Instructor WakeMed Health and Hospitals
Palm Beach Community College Raleigh, North Carolina
Port St. Lucie, Florida
Kerry Weinberg, MPS, RT(R), RDMS, RDCS
Charlotte Henningsen, MS, RT, RDMS, RVT, Program Director
FSDMS Diagnostic Medical Sonography
Chair and Professor New York University
Department of Diagnostic Medical Sonography New York, New York
Florida Hospital College of Health Sciences
Orlando, Florida
Cheryl Morrow, RT(R), RDMS, RDCS, RUT
Staff Sonographer
Sonowave
Tyler, Texas

ix
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 Preface

The Prenatal Diagnosis and Genetics Center at the University of Colorado Hospital
provides a multidisciplinary approach to diagnosing fetal cardiac disease that involves
sonography, perinatology, pediatric cardiology, radiology, and genetic counseling. This
combination of specialties provides the skills and different perspectives necessary to
diagnose and manage the complex problems presented by the fetus with cardiac
abnormalities.
The purpose of this book is to extend this approach to all those involved in fetal
echocardiography by providing a description of sonographic technique and diagnosis,
as well as insight into the management of these children, both before and after birth.
Each chapter has been written and critiqued by practitioners with expertise in this
field. All contributors are currently or were previously associated with our fetal car-
diovascular center.

New to This Edition

The second edition of Fetal Echocardiography provides the most current information
available on performing and interpreting fetal echocardiograms. It also acts as a study
guide for those just entering the field.

Important New Features Have Been Added

• Color Images
• All of the line art has been redrawn in full color.
• Color Doppler images are included throughout the book.
• Chapter Outlines
• Outlines are now included at the beginning of each chapter, providing readers
with an at-a-glance view of chapter contents.

xi
xii Preface

New Chapters Have Been Added On

• Prenatal Intervention in the Fetus with Cardiac Disease
• First-Trimester Fetal Echocardiography
• Three-Dimensional Fetal Echocardiography

Ancillaries
A companion CD in the back of the book has been developed for the second edition
and contains valuable resources to enhance knowledge and reinforce learning.
• Multimedia
• Video clips show scanning planes that are necessary to perform a fetal echocar­­
diogram.
• Study Questions
• More than 250 multiple-choice questions help readers reinforce information
presented in the book and are sorted by chapter.

I would like to acknowledge the contributors to this book for their willingness to share
their knowledge and their dedication to our profession.

It is hoped that obstetricians, pediatric cardiologists, radiologists, sonographers, and

others involved in the investigation of fetal heart disease will find this a useful
reference.

Julia A. Drose, ba, rdms, rdcs, rvt

 Acknowledgments

First and foremost, to my family: Donna Patterson, Lou Patterson, Jack Drose, Greg
Drose, Lora Drose, Emily Patterson, Mackenzie Patterson, Caroline Patterson, Brady
Drose, Taylor Drose, Kate Huisken, Brice Huisken, and Avery Huisken. Thank you all
for your continued love and support.
Thank you also to all of the sonographers, genetic counselors, nurses, and staff
involved with fetal echocardiography at the University of Colorado Hospital: June
Altman, Liana Amarillas, Sonia Archibeque, Amanda Auckland, Tina Bachman, Heidi
Barrett, Danielle Bolger, Tracy Bieker, Stella Bizzarro, Sandy Buckley, James Cacari,
Tyra Cade, Kathleen Digiulio, Juan Gamez, George Kennedy, Cheryl Kurth, Tyanne
Rosie, Britt Smyth, Emily Todd, and Michele Winslow. We succeed because of your
dedication to the field of fetal echocardiography and your understanding of how
important it is to provide the best possible care to our patients.
I would also like to thank the physicians that make our program a collaborative
effort: Jill Davies, Lorraine Dugoff, Henry Galan, John Hobbins, Michael Manco-
Johnson, Kristin McKinney, Melissa Palmer, Nayana Patel, Paul Russ, Elizabeth Shaffer,
Elizabeth Stamm, Karrie Villavicencio, Marsha Wheeler, Virginia Winn, and Adel
Younoszai.
Finally, thank you to the individuals at Elsevier who helped with the production
of this edition: Jeanne Olson, Publisher; Luke Held, Associate Developmental Editor;
Jeanne Robertson, freelance artist; Patricia Tannian, Publishing Services Manager,
Claire Kramer, Project Manager; and Charles Seibel, Designer.

xiii
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 Contents

Chapter 1 Chapter 6

Embryology and Physiology Atrioventricular Septal

of the Fetal Heart   1 Defects   119
Julia A. Drose Britt C. Smyth

2
Chapter 7
Chapter
Scanning: Indications Hypoplastic Left Heart
and Technique   15 Syndrome   131
Julia A. Drose Sarah H. Martinez

3
Chapter Chapter 8
Cardiac Malposition   73 Hypoplasia of the Right
Paul D. Russ Ventricle   145
Julia A. Drose Heidi S. Barrett

4
Chapter
9
Chapter
Atrial Septal Defects   91 Univentricular Heart   159
Elizabeth R. Stamm Kristin E. McKinney

5
Chapter

Ventricular Septal Defects   105

Amanda K. Auckland

xv
xvi Contents

Chapter 10 Chapter 18

Aortic Stenosis and Pulmonary Congenital Cardiac Masses   268

Stenosis   169 Tina M. Bachman
Teresa M. Bieker Heidi S. Barrett

11
Chapter 19
Chapter

Coarctation of the Aorta   184 Cardiosplenic Syndromes   281

Marisa R. Lydia Teresa M. Bieker
Julia A. Drose
20
Chapter

Chapter 12 Fetal Cardiomyopathies   293

Ebstein Anomaly   197 Marisa R. Lydia
Elizabeth R. Stamm Julia A. Drose

Chapter 13 Chapter 21

Tetralogy of Fallot   211 Fetal Arrhythmias   306

Danielle M. Bolger Karrie L. Villavicencio

Chapter 14 Chapter 22

Persistent Truncus Arteriosus   223 First-Trimester Fetal

Cynthia L. Rapp Echocardiography   324
Julia A. Drose Marsha Wheeler

15
Chapter 23
Chapter

Complete and Congenitally Corrected Three-Dimensional Fetal

Transposition of the Great Echocardiography   331
Arteries   234 Julia A. Drose
Elizabeth M. Shaffer
Chapter 24

16
Chapter Prenatal Intervention in the Fetus
Anomalous Pulmonary Venous with Cardiac Disease   337
Connection   245 Adel K. Younoszai
Elizabeth R. Stamm
Index   347
Chapter 17

Double-Outlet Right Ventricle and

Double-Outlet Left Ventricle   256
Julia A. Drose
 Fetal
Echocardiography
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 CHAPTER 1

Embryology and
Physiology of
the Fetal Heart
Julia A. Drose

OUTLINE Embryology
All major organ systems are formed between the
Embryology fourth and eighth weeks of development (Table
1–1). This is called the period of organogenesis.
Aortic Arches It is during this time that the embryo is most
Coronary Arteries susceptible to factors that interfere with de­­
velopment.
Pulmonary Veins
The cardiovascular system, including the heart,
Systemic Veins blood vessels, and blood cells, originates from the
Conduction System mesodermal germ layer.18 Cardiovascular mor­
phogenesis is controlled by mechanisms that
Fetal Circulation are common to all developmental processes: cell
Cardiac Output growth, cell migration, cell death, differentiation,
and adhesion.
Congenital Heart Disease
The heart initially consists of paired tubular
Pathogenesis structures that by the twenty-second day of devel-
opment (the embryo is approximately 2.5 to 3 mm
Congenital heart disease is the most common in length) form a single, slightly bent heart tube
severe congenital abnormality found among live (Fig. 1–1). This heart tube consists of an inner
births.1–13 endocardial tube and a surrounding myoepicardial
Because development of the heart is an interac- mantle. At this stage, the heart tube connects with
tion of genes, environment, and chance, approxi- the developing arch system and with the vitelline
mately 70% to 85% of cases of congenital heart and umbilical veins.19
disease have multifactorial causes.14–17 The second stage of cardiac development begins
To delineate the etiology and pathogenesis of with the formation of the atrioventriculobulbar
congenital heart disease requires an understand- loop. The cephalic portion of the heart tube bends
ing of both normal and abnormal cardiac ventrally and to the right, whereas the caudal
development. atrial portion begins to bend in a dorsocranial


 Fetal Echocardiography

TABLE 1–1 Timing of Embryologic Heart Formation

Timing Event

Days 21–22 Umbilical veins, vitelline veins, cardinal veins form

Single heart tube forms
Pericardial cavity forms
Day 23 Heart tube grows rapidly, forcing it to fold on itself
Days 25–28 Atrioventriculobulbar loop forms
Common atrium forms
Atrioventricular canal forms connecting common atrium to early embryonic ventricles
Septum primum appears
Days 27–37 Endocardial cushions appear
Day 28 Heart begins to beat
Ventricular septum appears as a small ridge in common ventricle
Ventricles begin to dilate
Days 28–35 Absorption of bulbous cordis and sinus venosus
Four-chambered heart forms
Day 29 Pulmonary veins form
Days 31–35 Placental circulation begins
Atrioventricular node develops
Ostium secundum is formed
Sinoatrial node develops
Day 33 Tricuspid and mitral valves form
Days 35–42 Coronary arteries form
Days 36–42 Inferior vena cava forms
Days 43–49 Superior vena cava forms
Coronary sinus forms
Day 49 Formation of muscular interventricular septum
Day 56 Aorta and pulmonary arteries form
Aortic and pulmonic valves form

Arterial pole First aortic arch

Bulbus cordis

Ventricle

Sinus horn
Atrium
Cardinal
veins

A Mesenchyme of septum transversum B

Figure 1–1 Formation of a single heart tube from two paired tubular structures. A, Ventral view at
approximately 21 days showing beginning of fusion. B, At 22 days fusion is almost complete.

direction and to the left, thus forming a loop sinus venosus. From here the atrioventricular
(Fig. 1–2).18 canal forms, which connects the common atrium
As this heart loop continues to bend, a common to the early embryonic ventricles.
atrium is formed and enters the pericardial cavity, It is at this time (approximately 28 days) that
carrying along the right and left segments of the contractions are thought to begin in the ventricu-
 Chapter 1 Embryology and Physiology of the Fetal Heart 

Aortic roots
Pericardium

Pericardial cavity
Bulbus cordis
Bulboventricular
sulcus

Ventricle Left
atrium
Atrium

Sinus Sinus
venosus venosus
A B C
Figure 1–2 Formation of the atrioventriculobulbar loop at approximately 22 days (A), at
approximately 23 days (B), and at approximately 24 days (C). As this loop is formed, a common
atrium is formed and enters the pericardial cavity.

lobulbar portion of the heart, and the heart beat until birth when pressure in the left atrium
is initiated.20 Circulation occurs from the sinus increases, causing the two septa to press against
venosus into the right atrium, into the left atrium, each other and close this communication.20
and then into the atrioventricular canal and the Septum formation within the atrioventricular
ventricles. canal occurs when two large endocardial cushions
Stage three in the development of the heart fuse, resulting in a right (tricuspid) and left (mitral)
consists of absorption of the bulbus cordis and atrioventricular orifice (Fig. 1–4). This usually
sinus venosus. At this stage, the atrioventri­c­ occurs by day 33 of development.18
ulobulbar loop begins to untwist and the car­­ The interventricular septum is formed by the
diac septa develop, forming a four-chambered end of the seventh week of development (Fig.
heart.19 1–5). It results from the dilation of the two primi-
Formation of the septa within the heart results tive ventricles (right and left conus swellings),
from the development of endocardial cushion which causes the medial walls to become apposed
tissue in the atrioventricular canal and the trun- and fuse together. This forms the muscular portion
coconal region. This occurs between the twenty- of the interventricular septum. Formation of the
seventh and thirty-seventh days of development, membranous portion follows.
when the embryo is 4 to 14 mm in length During the eighth week of development, the
(Fig. 1–3). truncus swellings or cushions of the primitive
In the atrium, the septum primum—a sickle- heart grow and twist around each other to form
shaped crest descending from the roof of the the aorticopulmonary septum (Fig. 1–6). This
atrium—does not completely divide the atrium in septum divides the truncus arteriosus into an
two, but leaves an open ostium primum for com- aortic channel and a pulmonary channel.
munication between the two chambers. When the The cushions of the conus cordis develop simul-
ostium primum is obliterated owing to fusion of taneously. These conus cushions unite with the
the septum primum with the endocardial cush- aorticopulmonary septum. After this fusion occurs,
ions, the ostium secundum forms within the the septum divides the conus into an anterolateral
septum primum. portion (the right ventricular outflow tract) and a
Last, a septum secundum is formed, but an posteromedial portion (the left ventricular outflow
interatrial opening, the foramen ovale, remains tract).
 Fetal Echocardiography

Ostium primum Septum primum Septum primum

Ostium primum
Endocardial
cushion Interventricular
foramen
A Atrioventricular canal B

Ostium secundum
Septum secundum Septum secundum
Septum
Septum primum primum Ostium secundum

Endocardial
Endocardial Interventricular cushions fused
cushion foramen

C D

Oval Septum
foramen secundum
Superior
vena cava
Septum
primum
Valve of
oval foramen

Valve of inferior
vena cava

E F G Valve of coronary sinus

Figure 1–3 A, Development of the atrial septa at approximately 30 days. B, Same stage as in A
but seen from the right. C, Development at approximately 33 days. D, Same stage as in C but
seen from the right. E, Development at approximately 37 days. F, Newborn heart. G, View of the
atrial septum seen from the right, same stage as in F.

Common Superior endocardial Right atrioventricular

atrioventricular canal cushion canal

Lateral cushion Inferior endocardial Left atrioventricular

cushion canal
A B C D
Figure 1–4 Formation of the septum in the atrioventricular canal. A, Approximately 28 days.
B, Approximately 30 days. C, Approximately 33 days. D, Approximately 35 days. The initial circular
opening gradually becomes elongated in the transverse direction, resulting in a right (tricuspid) and
left (mitral) atrioventricular orifice.
 Chapter 1 Embryology and Physiology of the Fetal Heart 

Aorta
Truncoconal
Right truncoconal ridge Anterior limb of
septum
interventricular
Right atrium Left truncoconal ridge septum

Superior Left atrioventricular

border right orifice
atrioventricular
orifice Proliferation of inferior
A atrioventricular cushion B
Right
atrioventricular Muscular part of
orifice interventricular septum

6th week Beginning of 7th week

Aorta
Truncoconal septum

Membranosus part of
interventricular septum

Right
atrioventricular orifice
Muscular part of
interventricular septum
C

End of 7th week

Figure 1–5 Development of the truncoconal ridges and the closure of the interventricular septum.
The proliferation of the right and left conus swellings, combined with the proliferation of the inferior
atrioventricular cushion, eventually closes the interventricular foramen and forms the membranous
portion of the interventricular septum. A, At 6 weeks. B, Beginning of the seventh week. C, End of
the seventh week.

Pulmonary Aorta
artery Pulmonary
Aortico- artery
Aorta Right atrium
pulmonary
septum
Membranosus
Aorta Pulmonary
A artery
interventricular B
septum

Aorta Right
Pulmonary atrioventricular
artery orifice
Pulmonary Aorta
artery Muscular Left atrioventricular
interventricular septum orifice

Figure 1–6 A, Formation of the aorticopulmonary septum. B, Division of the aorta and pulmonary
artery at the eighth week of development. This is accomplished by the aorta and pulmonary artery
twisting around each other.