Congenital Heart Disease and Adolescence

235 Pages·2016·5.07 MB·English

by  Markus Schwerzmann

Congenital Heart Disease in Adolescents and Adults
Series Editors: Massimo Chessa · Helmut Baumgartner
Andreas Eicken · Alessandro Giamberti
Markus Schwerzmann
Corina Thomet
Philip Moons Editors
Congenital
Heart Disease and
Adolescence
Congenital Heart Disease in Adolescents
and Adults
Endorsed by
The ESC Working Group on Grown-up Congenital Heart Disease
AEPC Adult with Congenital Heart Disease Working Group
Series editors
M. Chessa
San Donato Milanese , Italy
H. Baumgartner
Münster , Germany
A. Eicken
Munich , Germany
A. Giamberti
San Donato Milanese , Italy
The aim of this series is to cast light on the most signifi cant aspects – whether still
debated or already established – of congenital heart disease in adolescents and
adults and its management. Advances in the medical and surgical management of
congenital heart disease have revolutionized the prognosis of infants and children
with cardiac defects, so that an increasing number of patients, including those with
complex problems, can reach adolescence and adult life. The profi le of the adult
population with congenital heart disease (ACHD) is consequently changing, and in
future many adult patients will present different hemodynamic and cardiac problems
from those currently seen. A cure is rarely achieved, and provision of optimal care
is therefore dependent on ongoing surveillance and management in conjunction
with experts in this highly specialized fi eld. Specialists in ACHD management need
to have a deep knowledge not only of congenital cardiac malformations and their
treatment in infancy and childhood, but of general medicine, too. A training in adult
cardiology, including coronary artery disease, is also essential. Similarly, surgeons
need to acquire expertise and good training in both adult and pediatric cardiosurgery.
Readers will fi nd this series to be a rich source of information highly relevant to
daily clinical practice.
More information about this series at h ttp://www.springer.com/series/13454
Markus Schwerzmann
Corina Thomet
Philip Moons
Editors
Congenital Heart Disease
and Adolescence
Editors
Markus Schwerzmann Philip Moons
Center for Congenital Heart Disease Department of Public Health and Primary Care
University Hospital Inselspital KU Leuven – University of Leuven
Bern Leuven
Switzerland Belgium
and
Corina Thomet Institute of Health and Care Sciences
Center for Congenital Heart Disease University of Gothenburg
University Hospital Inselspital Gothenburg
Bern Sweden
Switzerland
ISSN 2364-6659 ISSN 2364-6667 (electronic)
Congenital Heart Disease in Adolescents and Adults
ISBN 978-3-319-31137-1 ISBN 978-3-319-31139-5 (eBook)
DOI 10.1007/978-3-319-31139-5
Library of Congress Control Number: 2016948119
© Springer International Publishing Switzerland 2016
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Preface to the Series
In Europe, we are currently faced with an estimated ACHD population of 4.2 mil-
lion; adults with congenital heart disease now outnumber children (approximately
2.3 million). The vast majority cannot be considered cured but rather having a
chronic heart condition that requires further surveillance and timely re-intervention
for residual or consequent anatomical and/or functional abnormalities. ACHD
patients have very special needs and the physicians taking care of them need expert
training. Special health care organization and training programs for those involved
in ACHD care are therefore required to meet the needs of this special population.
ACHD problems remain a small part of general cardiology training curricula
around the world, and pediatric cardiologists are trained to manage children with
CHD and may, out of necessity, continue to look after these patients when they
outgrow pediatric age.
There are clearly other health issues concerning the adult with CHD, beyond the
scope of pediatric medicine, that our patients now routinely face. Adult physicians
with a non-CHD background are therefore increasingly involved in the day-to-day
management of patients with CHD.
E xperts in congenital heart disease should work to improve the health care sys-
tem, so that teens and young adults have an easier time making the transition from
receiving health care in pediatric cardiology centers to receiving care from special-
ists in adult cardiology.
The aim of this series is to cast light on the most signifi cant aspects of congenital
heart disease in adolescents and adults and its management, like transition from
pediatric to adulthood, pregnancy and contraception, sport and physical activities,
pulmonary hypertension, burning issues related to surgery, interventional catheteri-
zation, electrophysiology, intensive care management, and heart failure.
This series wishes to attract the interest of cardiologists, anesthesiologists, car-
diac surgeons, electrophysiologists, psychologists, GPs, undergraduate and post-
graduate students, and residents, and would like to become relevant for courses of
cardiology, pediatric cardiology, cardiothoracic surgery, and anesthesiology.
v
vi Preface to the Series
We thank both the wonderful group of leading cardiovascular experts from
around the world, for donating their precious time, producing excellent textbooks
and making this book series a reality, and the members of the two Working Groups
(ESC and AEPC ACHD/GUCH Working Group) for the invaluable suggestions and
support without which this work would not be possible.
San Donato, Italy Massimo Chessa
Münster, Germany Helmut Baumgartner
Munich, Germany Andreas Eicken
San Donato, Italy Alessandro Giamberti
Foreword
T he editors and authors of “Congenital heart disease and adolescence” are to be
congratulated for identifying important gaps in our knowledge of caring for
patients with congenital heart defects. They correctly identify excellent textbooks
in pediatric cardiology and in adult congenital heart disease, but these really miss
the mark when it comes to the adolescent patient. They have assembled an excel-
lent and authoritative group of authors with a passionate commitment to their
areas of expertise. Some inspiration for this may well have come from a 2014
all-day symposium in Cincinnati entitled “Transition from Pediatric to Adult
Health Care: the Cincinnati Summit.” That meeting made abundantly clear how
much work still needs to be done to improve our management of adolescents at
this critical time in their lives, as well as their parents and family members whose
roles are changing so quickly.
It has been well documented that patients with moderate and complex congenital
heart defects are lost to care at all ages in childhood, adolescence, and adult life. It
has also been well documented that these patients face more morbidity and early
mortality unless their care is optimized in specialist centers. The emphasis on the
adolescent between ages 12 and 18 identifi es a particularly vulnerable stage of their
development. Unfortunately, we do not yet have a very good track record in manag-
ing transition and transfer, and even the leaders in our fi eld are still at an early phase
of showing us the way.
I was particularly intrigued with Part III dealing with neurodevelopmental and
psychosocial issues of adolescence. It’s extremely important that we caregivers
understand the nature and limitations of the patients we are working with and under-
stand that future progress can be expected with time, additional effort, and further
maturation.
Part IV gets quite granular in discussing various aspects of the organization of
care for adolescents. Information about transfer and transition of these patients is
reviewed in depth, along with best practices in transition care for these adolescents.
Particularly helpful are the transition toolkits provided to aid the professionals look-
ing after and evaluating these patients. The role and guidance of parents importantly
is also discussed in some depth. Finally, research directions and methods and topics
are discussed – extremely important since we still do not know enough to optimally
care for these still developing human beings and patients.
vii
viii Foreword
“ The healing professions” have come far in successfully managing congenital
heart defects in developed countries over the last two or three generations. In this
text, the authors focus on what may be our Achilles’ heel, the issues of loss to care,
patient and family engagement, and steps needed to achieve the fundamental objec-
tive of seeing these patients remain in care lifelong.
Cincinnati, OH Gary Webb
Pref ace
The successes in the diagnosis, treatment, and management of congenital heart dis-
ease are paramount. This has led to remarkable improvements of life expectancy
over the past decades. To date, about 90 % of children born with congenital heart
disease can reach adulthood [1]. This resulted in an increased prevalence of con-
genital heart disease, in such a way that nowadays, there are more adults with con-
genital heart disease in the population than children [2]. Nonetheless, the majority
of adults who underwent surgical repair in childhood are not cured and at risk of
cardiovascular complications and premature death [3].
I n the early era of congenital heart disease, pediatric cardiology and pediatric
cardiac surgery were the sole specialties that took care of affl icted patients. In the
1980s, the fi rst Adult Congenital Heart Disease programs started [4, 5], because
clinicians and hospital administrators realized that the growing group of adult
patients with congenital heart disease required dedicated care in an appropriate set-
ting. Jane Somerville in Britain and Joseph Perloff in the United States were the fi rst
advocates of the new discipline of adult congenital heart disease [6]. These pioneers
have paved the way for future developments. Indeed, as of the 1990s, an increasing
number of centers started adult congenital heart disease activities, with the steepest
growth in the 2000s. In conjunction with the increased life expectancy, the emphasis
of medical care in congenital heart disease shifted from improving infant survival
toward improving mortality, morbidity [7], and quality of life [8] throughout the
whole life span. Patients with congenital heart disease require lifelong specialist
care, and recent data suggest that adults continuously followed at specialist centers
have improved outcomes compared to CHD adults with non-specialized medical
follow-up or even lapses in their care [9]. In the early 2000s, the fi rst reports on
transition of adolescents with congenital heart disease were published. The trigger
for this was twofold. First, clinicians and researchers realized that transfer from
pediatric cardiology to adult congenital heart disease frequently failed [10, 11].
Hence, they started to look at risk factors for unsuccessful transfer and strategies
how to avoid it [12]. Second, healthcare professionals got more aware about the
challenges of young persons of growing up with a heart defect [13]. Indeed, these
individuals are developing from a dependent child toward an independent adult who
is able to take care for his/her own health. Furthermore, the challenges of the chronic
condition in adolescents are strongly interacting with their development. Indeed,
ix