An Illustrated Guide to Congenital Heart Disease: From Diagnosis to Treatment—From Fetus to Adult

704 Pages·2019·132.321 MB·English

by  In Sook Park (ed.)

In Sook Park
Editor
An Illustrated
Guide to Congenital
Heart Disease
From Diagnosis
to Treatment
–
From Fetus
to Adult
123
An Illustrated Guide to Congenital Heart Disease
In Sook Park
Editor
An Illustrated Guide to
Congenital Heart Disease
From Diagnosis to Treatment
–
From Fetus to Adult
Editor
In Sook Park
Asan Medical Center
University of Ulsan College of Medicine
Seoul
South Korea
ISBN 978-981-13-6977-3 ISBN 978-981-13-6978-0 (eBook)
https://doi.org/10.1007/978-981-13-6978-0
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To our patients with congenital heart disease and their families
Preface
Why do we need another textbook on congenital heart disease (CHD)? Aren’t there enough
textbooks on CHD already? This was the question I had in my mind all along since I started
working on this book 18 years ago. Despite this skepticism, I came to the conclusion that there
is a need for a “picture book” that helps in understanding CHD easily for doctors from all
related specialties, trainees, nurses, students, as well as patients and family members, which
covers all aspects of CHD from diagnosis to treatment and from fetus to adult. After all, under-
standing CHD is not easy without pictures, and this book, containing 2103 color figures, is all
about them and previous Korean editions.
This book is a revised English version of the book that I published its 1st edition in Korean
in 2001 and 2nd edition also in Korean in 2008. I believe that this book is quite unique and is
“different” from other textbooks on CHD from many aspects. Despite its large volume, this
book is intended to be a handy, quick reference for cardiologists, surgeons, intensivist, nurses,
trainees, students, researchers, and even patients and their families; therefore, it does not con-
tain an extensive literature review on new developments or controversial issues on CHD. I tried
to make the contents of this book concise and simplified as much as possible, using many
schematic diagrams, illustrations, tables, and abbreviations for visual simplicity. I also insisted
to arrange texts and figures in a certain fashion, so that the readers can have a grip on any sub-
ject at a glance every time they turn the pages. Even though the volume of this book is quite
large, it is written in a synopsis style, placing pictures before written texts on each subject, so
that the readers can have a quick and general overview of the lesion before going into the
detail.
Since Korean version has been, I believe, loved by many Korean doctors and nurses as a
quick bedside reference and has been useful for teaching the beginners and anyone interested
in CHD, I was encouraged to write this English version so that more people around the world
caring patients with CHD, from fetuses to adults, can have an access to this “picture” book, a
product of my personal lifelong work.
Originally, it all started many years ago while I was preparing a lecture for trainees and
nurses at Asan Medical Center where I worked. Probably like everyone else in this field, I had
a difficulty in finding an easy way to explain double switch operation for CC-TGA, since
CC-TGA was, among all CHDs, one of the most difficult CHDs to understand, particularly to
the beginners. It was that moment that I started to color and make morphologic modifications
on “Mullins diagrams” in order to make them look similar to the real CHD as much as
possible.
In this regard, I am most thankful and respectful to my teacher and a mentor, Dr. Charles
E. Mullins, the pioneer in catheter intervention for CHD, who was also the original designer of
the CHD diagrams (“Mullins diagram”). I have simply modified and colored his “cath dia-
grams.” Without his innovative idea, this book could not have been possible. I am also thankful
to Dr. Thomas A. Vargo, a superb clinician and humanist, whose teaching on CHD was always
simple and clear, frequently mentioning that what matters the most in CHD is to know where
the “blue blood” goes and where the “red blood” goes. Also, I am deeply thankful to Dr.
Michael R. Nihill, Dr. David J. Driscoll, Dr. Co-Burn J. Porter, and late Dr. Dan McNamara,
for their teaching and support while I was at Texas Children’s Hospital and Texas Heart
vii
viii Preface
Institute until 1989. I also express my deepest gratitude to Dr. William D. Edwards of Mayo
Clinic, Rochester, Minnesota, for his remarkable heart specimen pictures, which he kindly
allowed me to use for this book and previous Korean editions.
I am also very thankful to my former colleagues at Asan Medical Center, Dr. Young Hwue
Kim and Dr. Jae Kon Ko, pediatric cardiologists, for sharing the data and images of their
patients, and Dr. Dong Man Seo, a superb pediatric cardiac surgeon who had operated on many
patients introduced in this book at Asan Medical Center. Also, I pay deep respect to Dr. Chang
Eui Hong, an emeritus professor at Asan Medical Center and the father of pediatric cardiology
in Korea, for his mentoring and encouraging me through all these years.
Most clinical materials and images in this book are from my personal file, and in this regard,
I dedicate this book to my former patients at Texas Children’s Hospital and Texas Heart
Institute, Houston, Texas, USA, until 1987 and Asan Medical Center, University of Ulsan
College of Medicine, Seoul, Korea, from 1989 until 2012, to whom I owe a great deal. I
included certain angiograms from the 1980s which, I believe, have an important historic value.
For example, a countercurrent aortography through radial arteries in small babies and a bal-
loon occlusion aortography are not performed anymore, since CT and MRI have replaced
diagnostic angiograms in many CHDs. Actually, some part of this book looks like a collection
of case reports, some already published or some never been published.
Eventually, I hope that this book will contribute to improve the outcome of all patients with
CHD in the future. Again, with all my heart, I dedicate this book to those patients who have
been the subject of images and stories of this book and to patients in the future from around the
globe, whose lives hopefully benefit from this book.
Seoul, South Korea In Sook Park
July, 2019
Abbreviations
3D CT Three-dimensional or volume-rendering CT
AAO Ascending aorta
AO Aorta
AP view Anteroposterior view
AP window Aortopulmonary window
AS Aortic stenosis
ASD Atrial septal defect
AV valve Atrioventricular valve
AVSD Atrioventricular septal defect
BAS Balloon atrial septostomy
BCPS Bidirectional cavopulmonary shunt
BSA Body surface area
BT shunt Blalock-Taussig shunt
CC-TGA Congenitally corrected transposition of the great arteries
CHD Congenital heart disease (or defect)
CHF Congestive heart failure
COA Coarctation of the aorta
CS Coronary sinus
CVA Cerebrovascular accident (“stroke”)
Cx Left circumflex coronary artery
DAO Descending aorta
DCRV Double-chambered right ventricle
DILV Double inlet left ventricle
DIV Double inlet ventricle
DORV Double outlet right ventricle
FSV Functional single ventricle
GA Great artery
HLHS Hypoplastic left heart syndrome
IAA Interrupted aortic arch
IVC Inferior vena cava
LA Left atrium
LAD Left anterior descending coronary artery
LAO view Left anterior oblique view
LCA Left coronary artery
LCCA Left common carotid artery
LPA Left pulmonary artery
L-R shunt Left-to-right shunt
LSCA Left subclavian artery
LSVC Left superior vena cava
LV Left ventricle
LVH Left ventricular hypertrophy
LVOT Left ventricular outflow tract
ix
x Abbreviations
LVOTO Left ventricular outflow tract obstruction
m/s Meter per second
MAPCA Major aortopulmonary collateral artery
MPA Main pulmonary artery
MR Mitral regurgitation
MV Mitral valve
Op Operation
P vein Pulmonary vein
PA IVS Pulmonary atresia with intact ventricular septum
PA Pulmonary artery
PAPVC Partial anomalous pulmonary venous connection
PAPVR Partial anomalous pulmonary venous return
PAVM Pulmonary arteriovenous malformation
PDA Patent ductus arteriosus
PFO Patent foramen ovale
PH Pulmonary hypertension
PMI Perimembranous inlet
PR Pulmonary regurgitation
PS Pulmonary stenosis
PVR Pulmonary vascular resistance
QP Pulmonary blood flow
QS Systemic blood flow
RA Right atrium
RAO view Right anterior oblique view
RCA Right coronary artery
RCCA Right common carotid artery
RELSCA Retroesophageal left subclavian artery
RERSCA Retroesophageal right subclavian artery
RIA Right innominate artery
R-L shunt Right-to-left shunt
Rp Pulmonary resistance
RPA Right pulmonary artery
Rs Systemic vascular resistance
RSCA Right subclavian artery
RV Right ventricle
RVH Right ventricular hypertrophy
RVOT Right ventricular outflow tract
SV Single ventricle
SVC Superior vena cava
SVT Supraventricular tachycardia
TA (Persistent) truncus arteriosus
TAPVC Total anomalous pulmonary venous connection
TAPVR Total anomalous pulmonary venous return
TE echo Transesophageal echocardiography
TGA Transposition of the great arteries
TOF Tetralogy of Fallot
TR Tricuspid regurgitation
TV Tricuspid valve
VS Ventricular septum
VSD Ventricular septal defect
WPW Wolff-Parkinson-White syndrome
Contents
1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
In Sook Park and Soo-Jin Kim
2 Atrial Septal Defect (ASD) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
In Sook Park and Hyun Woo Goo
3 Ventricular Septal Defect (VSD) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
In Sook Park and Hyun Woo Goo
4 Patent Ductus Arteriosus Aortopulmonary Window . . . . . . . . . . . . . . . . . . . . . . . . 57
In Sook Park and Hyun Woo Goo
5 Partial Anomalous Pulmonary Venous Connection (PAPVC) . . . . . . . . . . . . . . . . 73
In Sook Park and Hyun Woo Goo
6 Atrioventricular Septal Defect (AVSD) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85
In Sook Park, Soo-Jin Kim, and Hye-Sung Won
7 Abnormalities of the Right Ventricular Outflow Tract and
Pulmonary Arteries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99
In Sook Park and Hyun Woo Goo
8 Aortic Stenosis and Other Types of Left Ventricular Outflow Tract
Obstruction or Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .125
In Sook Park and Hyun Woo Goo
9 Coarctation of the Aorta (COA) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .145
In Sook Park, Hyun Woo Goo, and Hye-Sung Won
10 Interrupted Aortic Arch (IAA) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .169
In Sook Park, Hyun Woo Goo, and Hye-Sung Won
11 Tetralogy of Fallot (TOF) With or Without Pulmonary Atresia . . . . . . . . . . . . . . .183
In Sook Park and Hyun Woo Goo
12 Tricuspid Atresia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .253
In Sook Park, Soo-Jin Kim, and Hyun Woo Goo
13 Complete Transposition of the Great Arteries (Complete TGA) . . . . . . . . . . . . . .269
In Sook Park and Hyun Woo Goo
14 Pulmonary Atresia with Intact Ventricular Septum (PA IVS) . . . . . . . . . . . . . . . .309
In Sook Park, Hyun Woo Goo, and Hye-Sung Won
15 Double Outlet Right Ventricle (DORV) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .339
In Sook Park and Hyun Woo Goo
xi