JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
22, 2017
ª 2017 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION
PUBLISHED BY ELSEVIER
EDITORIAL COMMENT
Congenital Heart Defects
Amazing Advances and Ongoing Challenges*
Kathy J. Jenkins, MD, MPH
I n this issue of the Journal, Larsen et al. (1) of the
Aarhus University Hospital in Denmark present
nearly 4 decades of data about the treatment of
congenital heart defects (CHDs) in their country,
and similar to others, demonstrate the amazing prog-
ress that has been made over 4 decades to provide
effective treatment for CHDs in settings with mature
health care delivery systems (2–4). The authors care-
fully describe cases of both surgical and catheter-
based intervention and subsequent mortality and
reintervention, along with comparative information
from a matched non-CHD cohort. The authors
highlight key aspects of evolution of CHD treatment,
especially the rise in infant surgery and case
complexity, introduction of transcatheter interven-
tion, and dramatic fall in early mortality.
SEE PAGE 2725
The amazing part of the story is truly noteworthy:
The authors report near-universal early survival since
2003. The data tell a fabulous story of modern med-
ical advancement and the parallel tale of relief of
suffering for both parents and children. The existence
of the data itself is also pretty amazing, especially to
U.S.-based scientists, who can look with envy at the
longitudinal population-based information available
in the Danish National Registry, with only a single
case with missing follow-up in 4 decades. The public
health and health care delivery system in Denmark
appears to be deploying its resources wisely, and
*Editorials published in the Journal of the American College of Cardiology
reflect the views of the authors and do not necessarily represent the
views of JACC or the American College of Cardiology.
From the Boston Children’s Hospital, Harvard Medical School, Boston,
Massachusetts. Dr. Jenkins has received study grants from Medtronic and
NuMed; and has received free drugs for a research study from Novartis.
VOL. 69, NO.
ISSN 0735-1097/$36.00
http://dx.doi.org/10.1016/j.jacc.2017.04.008
Danish people affected by congenital heart disease
have likely benefitted from the centralization in care
in just 2 locations, to optimize clinical experience
despite a small overall case volume (5).
The challenging part of the story is also beautifully
demonstrated. Despite all the recent advances, even
in the most recent time period, survival for people
with even simple CHDs is lower than for unaffected
controls, and many patients undergo reintervention.
We know from other work that survival with CHD is
often accompanied by disability (6), from both cardiac
causes such as cyanosis, congestive heart failure or
arrhythmia, and from related noncardiac associations
such as pulmonary, renal, or hepatic dysfunction, as
well as the ubiquitous presence of psychiatric, neu-
rocognitive, or psychosocial conditions, any of
which can affect life experience. In the United States,
the Congenital Heart Public Health Consortium has
recently brought together multiple stakeholders,
including patients, families, professional societies,
and public health professionals, including the U.S.
Centers for Disease Control and Prevention, to bring
attention to these important and challenging areas
for further research and intervention. Of course from
a global perspective, many affected children with
congenital heart disease do not have access to life-
saving procedures, which is yet another challenge (7).
The Danish data tell another, more subtle story
about the personal costs of CHDs—there were
fewer children operated on with complex CHDs in the
current period than previously, part of the story
behind the near eradication of very early mortality.
The authors note that this is largely due to recom-
mendations for second trimester screening of all
pregnancies, with in utero diagnosis and frequent
terminations. This practice by Danish parents reflects
their concerns about the multiple lifelong challenges
faced by people with complex congenital heart
2734 Jenkins JACC VOL. 69, NO. 22, 2017

Congenital Heart Defects: Advances and Challenges JUNE 6, 2017:2733–4

disease, and emphasizes the need for secondary
prevention of death and disability directly from
congenital heart disease, its treatment, and associ-
ated conditions. Perhaps in time, parents in Denmark
and elsewhere will feel more optimistic about the
quality of life their children with CHD will experi-
ence. This will only occur if countries with limited
resources find a way to provide reliable treatments
for CHDs as is the story in Denmark, and if countries
with more resources dedicate them to secondary
prevention. Larsen et al. (1) are to be commended for
providing information and careful analysis to inform
decision making and conversation.
ADDRESS FOR CORRESPONDENCE: Dr. Kathy J.
Jenkins, Boston Children’s Hospital, 300 Longwood
Avenue, Boston, Massachusetts 02115. E-mail: kathy.
jenkins@childrens.harvard.edu.

REFERENCES

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Interventional treatment of patients with
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2. Erikssen G, Liestol K, Seem E, et al. Achieve-
ments in congenital heart defect surgery: a pro-
spective 40-year study of 7038 patients.
Circulation 2015;131:337–46; discussion 346.
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Coffman GA, Iezzoni LI. In-hospital mortality for
surgical repair of congenital heart defects: pre-
liminary observations of variation by hospital
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outcomes in children with congenital heart dis-
ease: National Health Interview Survey. J Pediatr
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7. The Invisible Child series 2015-16. Minneap-
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http://theinvisiblechild.childrensheartlink.org/.
Accessed April 20, 2017.
KEY WORDS catheterization, epidemiology,
surgery, survival