ACHD achievements in the Asia-Pacific region

ACHD achievements in the Asia-Pacific region

Progress in Pediatric Cardiology 34 (2012) 57–60 Contents lists available at SciVerse ScienceDirect Progress in Pediatric Cardiology journal homepag...

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Progress in Pediatric Cardiology 34 (2012) 57–60

Contents lists available at SciVerse ScienceDirect

Progress in Pediatric Cardiology journal homepage: www.elsevier.com/locate/ppedcard

ACHD achievements in the Asia-Pacific region☆ Koichiro Niwa ⁎ Department of Cardiology, St Luke's International Hospital, Tokyo, Japan

a r t i c l e Keywords: CHD VSD Asia-Pacific APSACHD Congress

i n f o

a b s t r a c t Specialized care facilities for adults with CHD have been established in the Asia-Pacific region, but the number of specialists and facilities for ACHD is still small. Multidisciplinary ACHD teams are few in the region and formal education and training systems for adult CHD practitioners are still lacking. Further expansion of this population and evolution of specialized care facilities can be anticipated in the Asia-Pacific countries. By the Asian Pacific Society for Adult CHD (APSACHD), collaborative research, and medical support for developing countries 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 patients with congenital heart disease (CHD), even complex CHD, can be expected to reach adulthood. There have been several excellent reports about the estimated number of adults with CHD (ACHD) in Canada, UK, and US [1–3], but there are few reports on the number of ACHD patients from Asia-Pacific countries. These data regarding the prevalence of ACHD are crucial in determining the resources and special facilities required for their care. The number of adults with CHD in Japan based on the death certificates of CHD registered with the Japanese government [4] is as follows: a total of 622,800 patients, including 304,474 children (49%) and 318,326 adults (51%) were estimated to be alive in 1997. From 1997 to 2007, there has been an estimated increase of 9000 adults every year, and in 2007, 409,101 adults are estimated to be alive (Fig. 1) [5]. The prevalence of ACHD population in Korea, Taiwan and Thailand in 2000 is 22–26%, 20% and 32% of total CHD, respectively. In Singapore, the number of CHD in adults (~15,000) is higher comparing with CHD in children (~5000) in 2008. ACHD has a moderate or greater severity in 36.6% of Korean patients and 32% of Japanese patients (Figs. 2, 3) [5]. Therefore, in the Asia-Pacific area, the number of ACHD patients has been increasing as was observed in North America and Europe.

☆ Kawasaki disease is an acquired condition, and many patients need to be followed as adults. Adult cardiologists don't know a lot about Kawasaki disease, so our ACHD clinics have taken on responsibility for coordinating the care of these patients. ⁎ Tel.: + 81 3 3541 5151; fax: + 81 3 5550 7194. E-mail address: [email protected]. 1058-9813/$ – see front matter © 2012 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ppedcard.2012.05.013

2. Oriental VSD and other cardiac disorders specific to the Asia-Pacific region It is well known that the incidence of CHD is almost same internationally, but the types of or distribution of CHD is different between Asia and North America/Europe. The incidence of coarctation of the aorta and Marfan syndrome is higher in North America/Europe, but ventricular septal defect (VSD) especially subpulmonary (outlet, conus, subarterial) VSD is more prevalent in Asia (29–38% of total VSD) (Figs. 4, 5) [6–11]. Kawasaki disease is also much more prevalent in Japan, with >200,000 children affected, one third of whom need follow-up. 3. ACHD facilities in the Asia-Pacific region In the Asia-Pacific area, ACHD facilities have generally not yet been developed. Tertiary care facilities that developed in North America and Europe is rare in this area. However, 13 countries in this area already opened at least one outpatient clinic for ACHD (Table 1). The number of countries that had ACHD clinic was only 7 in 2007, so the number of clinics is growing rapidly. Also the number of specific ACHD facilities in Japan is increasing (Table 2) [12–14]. Directors in most of Asia-Pacific facilities are pediatric cardiologists (Table 3). However, adult cardiologists usually join the team. In the Japanese Society for Adult Congenital Heart Disease (JSACHD), 15% of registrants 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, Afghanistan, Myanmar, Bhutan, Nepal and Bangladesh, CHD care is available but limited, virtually nonexistent, or rudimentary. Health

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

care is very basic and suboptimal, but is gradually improving together with rapid economic growth. In India, 10,000 patients undergo congenital heart surgery each year. Fully 180,000 children with CHD are born annually in India. It is estimated that India needs 200 centers doing 1000 cases/year, but in reality there are only 20 centers now. India is a vast country with limited resources, uneven population distribution, and too few specialized centers. There are no active and organized ACHD centers, and it is difficult to travel from remote areas for follow-up appointments. There are special challenges in Pakistan, where severe CHD lesions seem unusually common, where neurodevelopmental problems are frequent, and where the challenges of tuberculosis and malnutrition are prevalent.

304,474

409,101 318,326

163,058

84,196

53,846

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

5. The Asia-Pacific Society of Adult Congenital Heart Disease (APSACHD) APSACHD was established at the 2nd Congress of Asia Pacific Pediatric Cardiology (APPCS) in Jeju Island in Korea in 2008. At that meeting, Jack Colman MD, the president of ISACHD (International Society for Adult CHD) and Harald Kaemmerer MD, the President of the ESC GUCH Working Group joined the meeting. The 2nd Congress of APSACHD was held from July 6–10, 2010, in Chiba, Japan, combined with the 46th Congress of the Japanese Society of Pediatric Cardiology and Cardiovascular Surgery and the 3rd Congress of Asia Pacific Pediatric Cardiology (APPCS). We had a total of approximately 1600 attendants from Japan and 400 from more than 25 countries all over the world. The APSACHD Society includes 15 AsiaPacific 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, and case presentations. In addition, we had joint sessions on pulmonary hypertension and pregnancy between APSACHD, the ESC GUCH Working Group and ISACHD. Attendees from the Asia-Pacific area, Europe and North America shared the data and information together and had a spirited discussion about the future development of this field. We made a lot of progress towards closer collaboration between colleagues in the Asia-Pacific area, Europe, and North America.

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

450000 400000 350000

Number of subjects

No. of subjects

58

Moderate-severe

78,952 (25%)

131,101(32%)

300000 250000

moderate-severe mild

200000 150000

278,001 (68%) 8,949(11%)

100000

3,195(6%)

239,374 (75%)

Mild CHD

50000 0

50,651(94%) 1967

75,247(89%) 1971

1997

2007

Fig. 3. The severity of CHD from 1967 to 2007 in Japan. Modified from Ref. [5].

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

USA (REF.7.8)

POSITION OF VSD

SUBPULMONARY VSD PERIMEMBRANOUS VSD COMMON AV CANAL

13(8%) 105(73%) 7(4%)

TYPE

VSD MUSCULAR DEFECT

32(15%)

JAPAN(REF. 9.10)

34(29%) 78(68%) 1(1%) 2(2%)

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

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Table 2 Facility survey for Japan and Korea. Institution

Chiba TWMU NCCHD Kurum Okaya Toho SMC

Established Patients Active patients Personnel Adult cardiologists Pediatric cardiologists Cardiovasc surgeons Specialty nurses

1998

1975

1980

1994

2004

Asan

1100

3000

1700

611

350

2

0

0

0

1

0

2

1

3

3

3

2

2

2

3

1

2

2

3

1

2

1

3

1

1

0

0

0

0

0

1

1

1974 1995 2005 300 1700

700

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 3 Facility survey for other Asia-Pacific countries.

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

The next APSACHD Congress will be held in Taipei in 2012. We will work together to keep our regional and international collaborations progressing towards a promising future. 6. Summary • Specialized care facilities for adults with CHD have been established in the Asia-Pacific region by physicians who were trained in North America or Europe, but the number of specialists and facilities for ACHD is still small compared to the growing number of patients.

Institution

Auk GL

ABCI

RPA

Shirir

SiNUH

SiNHC

Established Patients Active patients Personnel Adult cardiologists Pediatric cardiologists Cardiovasc surgeons Specialty nurses

1995

1998

1992

1998

2003

2005

1200

3000

1000

700

1500

1000

1 2 3 0

0 1 1 0

2 2 1 0

2 2 3 1

1 1 1 2

1 2 1 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.

• Multidisciplinary ACHD teams are few in the region, and more are needed. • Formal education and training systems for adult CHD practitioners are still lacking. • Further expansion of this population and evolution of specialized care facilities can be anticipated in the Asia-Pacific countries all.

Table 1 Facilities for ACHD in Asia-Pacific area.

Japan Korea China Taiwan Philippines Australia New Zealand Thailand Singapore Indonesia Malaysia Vietnam India Pakistan Turkey Hong Kong Total

Adult CHD facility (Y/N)

Number of facilities

Y Y Y Y N Y Y Y Y Y Y N Y N Y Y Y: 13 N: 3

14 3 2 1 0 3 2 1 2 1 1 0 1 0 1 1 33

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

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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 cooperation with ISACHD and ESC GUCH. (Web Page: http://www.apsachd. org). • Collaboration, research work, and medical support for developing countries have begun. References [1] Warnes CA, Liberthson R, Danielson GK, et al. Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol 2001;37(5):1170–5. [2] Wren C, O'Sullivan JJ. Survival with congenital heart disease and need for follow up in adult life. Heart 2001;85(4):438–43. [3] Marelli AJ, Mackie AS, Ionescu-Ittu R, et al. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 2007;115(2): 163–72. [4] Terai M, Niwa K, Nakazawa M, et al. Mortality from congenital cardiovascular malformations in Japan, 1968 through 1997. Circ J 2002;66(5):484–8. [5] Shiina Y, Toyoda T, Kawasoe Y, et al. Prevalence of adult patients with congenital heart disease in Japan. Int J Cardiol 2011;146:13–6.

[6] Tatsuno K, Konno S, Ando M, et al. Pathogenetic mechanisms of prolapsing aortic valve and aortic regurgitation associated with ventricular septal defect. Anatomical, angiographic, and surgical considerations. Circulation 1973;48:1028–37. [7] Becu LM, Burchell HB, Duchane JW, et al. Anatomic and pathologic studies in ventricular septal defect. Circulation 1956;14:349–64. [8] Goor DA, Lillehei CW, Rees R, et al. Isolated ventricular septal defect. Development basis for various types and presentation of classification. Chest 1970;58:468–82. [9] Shohtsu A, Takizawa S, Inoue T. Surgical anatomy on ventricular septal defect. Gen Thorac Cardiovasc Surg 1967;15:887 (in Japanese). [10] Tatsuno K, Konno S. Surgical anatomy of ventricular septal defect. Shinzo 1970;2: 775–81 (in Japanese). [11] Choi Y. VSD with AR In adult natural survivors in Korea. Korean Circ J 1998;28: 1782–9 (in Korean). [12] Toyoda T, Tateno S, Kawasoe Y, et al. Nationwide survey of care facilities for adults with congenital heart disease in Japan. Circ J 2009;73:1147–50. [13] Ochiai R, Murakami A, Toyoda T, et al. Opinions of physicians regarding problems and tasks involved in the medical care system for patients with adult congenital heart disease in Japan. Congenit Heart Dis 2011;6:359–65. [14] Ochiai R, Yao A, Kinugawa K, et al. Status and future needs of regional adult congenital heart disease centers in Japan. Circ J 2011;75:2220–7.