History and activity of JNCVD-ACHD


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.


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