Progress in Pediatric Cardiology 34 (2012) 57–60

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Progress in Pediatric Cardiology

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ACHD achievements in the Asia-Pacific region☆

Koichiro Niwa ⁎Department of Cardiology, St Luke's International Hospital, Tokyo, Japan

☆ Kawasaki disease is an acquired condition, and manas adults. Adult cardiologists don't know a lot about Kclinics have taken on responsibility for coordinating the⁎ Tel.: +81 3 3541 5151; fax: +81 3 5550 7194.

E-mail address: KNIWA@aol.com.

1058-9813/$ – see front matter © 2012 Elsevier Irelanddoi:10.1016/j.ppedcard.2012.05.013

a b s t r a c t

a r t i c l e i n f o

Keywords:

CHDVSDAsia-PacificAPSACHD Congress

Specialized care facilities for adults with CHD have been established in the Asia-Pacific region, but the num-ber of specialists and facilities for ACHD is still small. Multidisciplinary ACHD teams are few in the region andformal education and training systems for adult CHD practitioners are still lacking. Further expansion of thispopulation and evolution of specialized care facilities can be anticipated in the Asia-Pacific countries. By theAsian Pacific Society for Adult CHD (APSACHD), collaborative research, and medical support for developingcountries have begun.

© 2012 Elsevier Ireland Ltd. All rights reserved.

1. Incidence of ACHD in the Asia-Pacific region

Owing to advances of surgical and medical management, most pa-tients with congenital heart disease (CHD), even complex CHD, can beexpected to reach adulthood. There have been several excellent reportsabout the estimated number of adults with CHD (ACHD) in Canada, UK,and US [1–3], but there are few reports on the number of ACHDpatientsfrom Asia-Pacific countries. These data regarding the prevalence ofACHD are crucial in determining the resources and special facilities re-quired for their care.

The number of adults with CHD in Japan based on the death certifi-cates of CHD registered with the Japanese government [4] is as follows:a total of 622,800 patients, including 304,474 children (49%) and318,326 adults (51%) were estimated to be alive in 1997. From 1997to 2007, there has been an estimated increase of 9000 adults everyyear, and in 2007, 409,101 adults are estimated to be alive (Fig. 1) [5].The prevalence of ACHD population in Korea, Taiwan and Thailand in2000 is 22–26%, 20% and 32% of total CHD, respectively. In Singapore,the number of CHD in adults (~15,000) is higher comparing with CHDin children (~5000) in 2008. ACHD has a moderate or greater severityin 36.6% of Korean patients and 32% of Japanese patients (Figs. 2, 3)[5]. Therefore, in the Asia-Pacific area, the number of ACHD patientshas been increasing as was observed in North America and Europe.

y patients need to be followedawasaki disease, so our ACHDcare of these patients.

Ltd. All rights reserved.

2. Oriental VSD and other cardiac disorders specific to theAsia-Pacific region

It is well known that the incidence of CHD is almost same interna-tionally, but the types of or distribution of CHD is different betweenAsia and North America/Europe. The incidence of coarctation of theaorta and Marfan syndrome is higher in North America/Europe, butventricular septal defect (VSD) especially subpulmonary (outlet,conus, subarterial) VSD is more prevalent in Asia (29–38% of totalVSD) (Figs. 4, 5) [6–11]. Kawasaki disease is also much more preva-lent in Japan, with >200,000 children affected, one third of whomneed follow-up.

3. ACHD facilities in the Asia-Pacific region

In the Asia-Pacific area, ACHD facilities have generally not yet beendeveloped. Tertiary care facilities that developed in North Americaand Europe is rare in this area. However, 13 countries in this area al-ready opened at least one outpatient clinic for ACHD (Table 1). Thenumber of countries that had ACHD clinic was only 7 in 2007, sothe number of clinics is growing rapidly. Also the number of specificACHD facilities in Japan is increasing (Table 2) [12–14]. Directors inmost of Asia-Pacific facilities are pediatric cardiologists (Table 3).However, adult cardiologists usually join the team. In the JapaneseSociety for Adult Congenital Heart Disease (JSACHD), 15% of regis-trants for the annual congress are adult cardiologists (Fig. 6).

4. CHD in developing countries in Asia

In developing countries such as Sri Lanka, Vietnam, Pakistan, Af-ghanistan, Myanmar, Bhutan, Nepal and Bangladesh, CHD care isavailable but limited, virtually nonexistent, or rudimentary. Health

No.

of s

ubje

cts

53,846 84,196

318,326

409,101

163,058

304,474

Fig. 1. The number of ACHD patients in Japan.Modified from Ref. [5].

Fig. 2. ACHD disease severity in Korea.Courtesy of Lee HJ. 2nd Congress of APSACHD, JeJu Island,Korea, 2008.

Num

ber

of s

ubje

cts

0

50000

100000

150000

200000

250000

300000

350000

400000

450000

1967 197150,651(94%)

239

78,952 (

3,195(6%)

75,247(89%)

8,949(11%)

Fig. 3. The severity of CHD froModified from Ref. [5].

58 K. Niwa / Progress in Pediatric Cardiology 34 (2012) 57–60

care is very basic and suboptimal, but is gradually improving togetherwith rapid economic growth. In India, 10,000 patients undergo con-genital heart surgery each year. Fully 180,000 children with CHD areborn annually in India. It is estimated that India needs 200 centersdoing 1000 cases/year, but in reality there are only 20 centers now.India is a vast country with limited resources, uneven population dis-tribution, and too few specialized centers. There are no active and or-ganized ACHD centers, and it is difficult to travel from remote areasfor follow-up appointments. There are special challenges in Pakistan,where severe CHD lesions seem unusually common, where neu-rodevelopmental problems are frequent, and where the challengesof tuberculosis and malnutrition are prevalent.

5. The Asia-Pacific Society of Adult Congenital HeartDisease (APSACHD)

APSACHDwas established at the 2nd Congress of Asia Pacific Pedi-atric Cardiology (APPCS) in Jeju Island in Korea in 2008. At that meet-ing, Jack Colman MD, the president of ISACHD (International Societyfor Adult CHD) and Harald Kaemmerer MD, the President of the ESCGUCH Working Group joined the meeting.

The 2nd Congress of APSACHD was held from July 6–10, 2010, inChiba, Japan, combined with the 46th Congress of the Japanese Societyof Pediatric Cardiology and Cardiovascular Surgery and the 3rd Con-gress of Asia Pacific Pediatric Cardiology (APPCS). We had a total of ap-proximately 1600 attendants from Japan and 400 from more than 25countries all over the world. The APSACHD Society includes 15 Asia-Pacific countries such as Australia, China, India, Indonesia, Japan,Korea, Malaysia, New Zealand, Pakistan, Philippines, Singapore, Taiwan,Thailand, Turkey, and Vietnam.

At the APSACHD Congress, we had sessions on the right ventricle,cardiac failure, pregnancy issues, long-term follow-up of TGA, andcase presentations. In addition, we had joint sessions on pulmonary hy-pertension and pregnancy between APSACHD, the ESC GUCH WorkingGroup and ISACHD. Attendees from the Asia-Pacific area, Europe andNorthAmerica shared the data and information together and had a spir-ited discussion about the future development of this field. We made alot of progress towards closer collaboration between colleagues in theAsia-Pacific area, Europe, and North America.

1997 2007

moderate-severe

mild

,374 (75%)

25%)

278,001 (68%)

131,101(32%)

Mild CHD

Moderate-severe

m 1967 to 2007 in Japan.

Fig. 5. Oriental (subpulmonary) VSD with aortic regurgitation in Korean adults.Courtesy of Lee HJ. 2nd Congress of APSACHD, JeJu Island, Korea, 2008.

POSITION OF VSDUSA (REF.7.8) JAPAN(REF. 9.10)

SUBPULMONARY VSDPERIMEMBRANOUS

VSDCOMMON AV CANAL

TYPE VSD

MUSCULAR DEFECT

13(8%)105(73%)

7(4%)

32(15%)

34(29%)78(68%)1(1%)

2(2%)

Fig. 4. Difference in prevalence in VSD anatomy in autopsied hearts between USA andJapan.Refs. [7–10].

Table 2Facility survey for Japan and Korea.

Institution Chiba TWMU NCCHD Kurum Okaya Toho SMC Asan

Established 1998 1975 1980 1994 2004 1974 1995 2005Patients

Active patients 1100 3000 1700 611 350 300 1700 700Personnel

Adultcardiologists

2 0 0 0 1 0 2 1

Pediatriccardiologists

3 3 3 2 2 2 3 1

Cardiovascsurgeons

2 2 3 1 2 1 3 1

Specialtynurses

1 0 0 0 0 0 1 1

CCVC = Chiba Cardiovascular Center, Chiba, Japan.NCCHD = National Center for Child Health and Development, Tokyo, Japan.TWMU = Tokyo Women's Medical University, Tokyo, Japan.OK = Okayama University, Okayama, Japan.KU = Kurume University, Kurume, Japan.TOHO = Toho University, Tokyo, Japan.SMC = Samsung Medical Center, Seoul, Korea.Asan = Asan Medical Center, Seoul Korea.

Table 3Facility survey for other Asia-Pacific countries.

Institution Auk GL ABCI RPA Shirir SiNUH SiNHC

Established 1995 1998 1992 1998 2003 2005Patients

Active patients 1200 3000 1000 700 1500 1000Personnel

Adult cardiologists 1 0 2 2 1 1Pediatric cardiologists 2 1 2 2 1 2Cardiovasc surgeons 3 1 1 3 1 1Specialty nurses 0 0 0 1 2 1

AukGL = Starship Hospitals, Auckland/Green Lane, NZ.ABCI = Adolph Basser Cardiac Institute, Children's Hospital, Sydney, Australia.RPA = Royal Prince Alfred Hospital, Sydney, Australia.Shirir = Siriraj Hospital Mahidol University, Bangkok, Thailand.SiNUH = National University Hospital, Singapore.SiNHC = National Heart Centre, Singapore.

59K. Niwa / Progress in Pediatric Cardiology 34 (2012) 57–60

The next APSACHD Congress will be held in Taipei in 2012. Wewill work together to keep our regional and international collabora-tions progressing towards a promising future.

6. Summary

• Specialized care facilities for adults with CHD have been establishedin the Asia-Pacific region by physicians who were trained in NorthAmerica or Europe, but the number of specialists and facilities forACHD is still small compared to the growing number of patients.

Table 1Facilities for ACHD in Asia-Pacific area.

Adult CHD facility (Y/N) Number of facilities

Japan Y 14Korea Y 3China Y 2Taiwan Y 1Philippines N 0Australia Y 3New Zealand Y 2Thailand Y 1Singapore Y 2Indonesia Y 1Malaysia Y 1Vietnam N 0India Y 1Pakistan N 0Turkey Y 1Hong Kong Y 1Total Y: 13 N: 3 33

• Multidisciplinary ACHD teams are few in the region, and more areneeded.

• Formal education and training systems for adult CHD practitionersare still lacking.

• Further expansion of this population and evolution of specializedcare facilities can be anticipated in the Asia-Pacific countries all.

Fig. 6. Medical specialties of 863 members of JSACHD in 2011. CV: cardiovascular.

60 K. Niwa / Progress in Pediatric Cardiology 34 (2012) 57–60

• Good news: we now have the Asian Pacific Society for Adult CHD(APSACHD) and this society is actively moving forward in coopera-tion with ISACHD and ESC GUCH. (Web Page: http://www.apsachd.org).

• Collaboration, research work, and medical support for developingcountries have begun.

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