Origin
About our name
The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease (JNCVD-ACHD) is a committee (network) that was established at its first meeting in December 2011.
Background
Congenital
Heart Disease (CHD) collectively means patients with congenital cardiovascular
problems, but is mostly considered to be synonymous with patients with
structural heart problems, so we will use it as such. The incidence of CHD is
about 1% of all births, which is extremely high. Since many of the cases
involve shunt-related disorders, with the rapid onset of characteristic
clinical conditions such as cyanosis, pulmonary hypertension and heart failure,
CHD is traditionally considered as a pediatric illness, and the role of
cardiologists specializing in adult diseases has been limited.
As open-heart surgery was not safely available
until about 50 years ago, only some CHD patients with relatively minor abnormalities like
atrial septal defect or very small defects of the ventricular septum survived
until adulthood and most patients with cyanotic heart diseases died
during childhood or if they survived to adulthood they usually had severe
complications.
With the establishment of artificial heart lung machines in the 1970s and the
progress of surgical repair since the 1980s, the number of CHD patients who
survive to adulthood as if they have been completely cured has increased,
leading to the establishment of the field of adult CHD (ACHD) (This is sometimes
referred to as GUCH: Grown Ups with Congenital Heart defects/Grown-Up
Congenital Heart disease). Although some experts were aware of this situation, the
focus in Japan remained on the treatment and care of CHD patients until 2000,
and little attention has been given to adult cardiologists. As a
result, few measures have been taken in the field of ACHD.
In Europe and America, where a rapid increase in the number of ACHD patients
was already anticipated in the 1980s, with an expected increase in the number
not only of CHD but also of ACHD patients to 1% (prevalence) of the population,
measures were promptly taken. Though the most important factor for this was
that it became clear that even if CHD patients underwent radical surgery they
often developed various complications during adulthood, the fact that ACHD
patients are often treated by adult
cardiologists in Europe and America was also another
important factor. For these reasons, radical surgery
is currently called not curable one but just a
surgical repair. Against this background, as it was expected to be essential to
treat more ACHD patients, the necessity to establish a comprehensive medical
care system was finally recognized, so that the problems involving ACHD
patients, not only the peculiarities of the disorders, but also concomitant
diseases, complications of pregnancy and childbirth, up to complicating mental
disorders and social problems can be totally
studied. In North America and Europe, ACHD experts provided expert medical care
and established clinical guidelines for ACHD patients. However, despite the
much earlier start than Japan, the number of specialized ACHD facilities was
only a dozen in the United States as of 2007, and only 4% of patients were
receiving medical care. Based on these circumstances, discussions among major
academic societies and organizations led to the establishment of the ACHD
specialist doctor system in 2013 and the birth of ACHD specialist doctors in
2015, which became a major turning point in the United States. A total of 300
posts have already been created at 150 institutions in the United States,
showing that the United States is making great efforts to tackle ACHD.
In Japan, a study group, the predecessor of the current Japanese Society for
Adult Congenital Heart Diseases (transferred in 2010) was established in 1999
to create the ACHD clinical guidelines, but the study group was led by
pediatricians and cardiac surgeons, and only a few physicians from the adult
medical department (cardiovascular department) including adult
cardiologists were involved. The number of ACHD patients
already surpassed 400,000 in 2007, with an estimated total of almost 700,000
CHD patients, but most of the ACHD patients, in particular those who need to
receive medical care were treated by pediatric cardiologists. Additionally,
adult patients had to be admitted to pediatrics department, and cardiologists
had little knowledge of ACHD, to the extent that they did not even recognize
the necessity of ACHD treatment or the number of ACHD patients. According to a
nationwide questionnaire survey conducted in 2009, answers obtained from 109
(about 80%) of the 138 major cardiovascular departments indicated that only 30%
of them focused on ACHD treatment. We concluded that the negativity within
cardiovascular departments was a bottleneck factor in the establishment of a
comprehensive medical facility. Based on these results, JNCVD-ACHD was finally
established. In December 2011, Dr. Ryozo Nagai, who was, at that
time, the chairman of JNCVD-ACHD and Professor of
Cardiovascular Medicine at the University of Tokyo Hospital proposed the first
meeting, with the cooperation of Dr. Koichiro Niwa who was, at
that time, President of the Japanese Society for
Adult Congenital Heart Disease. The representatives of the cardiovascular
departments of 8 institutions attended the first meeting.
Through subsequent annual meetings, the number of participating institutions
has gradually increased. The current number of registered institutions is 35
(Fig. 2) at the time of this website release (as of April 2017). The aims of
JNCVD-ACHD are these four goals: 1. Fostering cardiovascular physicians who can
treat ACHD, 2. Establishment and development of ACHD comprehensive medical
institutions (Fig. 1), 3. Establishment of local ACHD medical
treatment cooperation and comprehensive system
connecting medical institutions (Fig. 1), 4. Establishment of ACHD medical
treatment evidence in Japan (promotion of multicenter clinical research). There
are still regional differences in medical care systems, but the ACHD medical
care system in each region is being improved centered on the participating
institutions.
Atsushi Yao, Secretariat of JNCVD-ACHD