2016 Volume 80 Issue 5 Pages 1242-1250
Background: Current Japanese transfer practices for adult congenital heart disease (ACHD) patients in pediatric departments are elucidated in this study. The focus was on 149 facilities (from the Japanese Society of Pediatric Cardiology and Cardiac Surgery Subspecialty Board and the Japanese Association of Children’s Hospitals and Related Institutions). One hundred and thirteen facilities were surveyed and the response rate was 75.8%.
Methods and Results: Twenty-six facilities (23.0%) treated ≥200 outpatients annually; 48 facilities (42.9%) treated <50 outpatients. Only eight facilities admitted ≥50 inpatients; 61 facilities (54.0%) admitted <10 inpatients. Nine facilities fulfilled international regional ACHD center criteria. The estimated median number of patients receiving outpatient pediatric department follow up was 33,806. Sixty facilities (53.6%) treated patients in pediatric departments after they reached adulthood. Of 49 facilities that transferred patients, the transfer was most commonly to another department in the same facility (20 facilities; 40.8%), typically the adult cardiology department (29 facilities; 59.2%). In future, 55 facilities (48.7%) desired the transfer of patients to regional ACHD centers, while 34 facilities (30.1%) preferred to continue treating patients in the pediatric department.
Conclusions: The number of regional ACHD centers offering sufficient outpatient and inpatient care is limited; transfer from pediatric departments is not standard in Japan. Role division clarification between regional ACHD centers and other facilities and cooperative network establishment including transitional care programs is necessary. (Circ J 2016; 80: 1242–1250)
Developments in surgical treatment, internal medicine, and testing have resulted in increasing numbers of congenital heart disease patients surviving to adulthood. Japan has approximately 400,000 adult congenital heart disease (ACHD) patients, 130,000 of whom are estimated to be moderate or severe; this number is estimated to increase by 9,000 annually.1 Due to sequelae, many of these patients experience unique issues in adulthood, such as reoperation, pregnancy,2 childbirth, and mental health issues.3 These issues require continuous observation and treatment.
Editorial p 1108
In ACHD care continuums, the central role is played by “regional ACHD centers,” also called a “specialist center” in Europe. International guidelines describe these centers’ roles as provision of medical care via teams comprising various professions and response to all latent medical care needs of ACHD patients.4–6 Therefore, these facilities require physicians specializing in ACHD, pediatric cardiologists, adult cardiologists, cardiovascular surgeons, and nurses with ACHD experience, as well as electrophysiological equipment and electrophysiologists specifically trained for ACHD care.4–6
The need for regional ACHD centers in Japan has been reported.7–10 However, many patients currently continue treatment with pediatric cardiologists even after reaching adulthood.9,11 This situation stems from the inadequacy of regional ACHD centers, particularly adult cardiologists’ passive attitudes toward ACHD care.9
Therefore, we conducted a nationwide survey on adult cardiology departments regarding ACHD care.10 Approximately 30% of all adult cardiology departments intend to treat ACHD patients including those with severe conditions. There are 14 facilities nationwide with the potential to serve as regional ACHD centers, with regional differences in geographic locations.10 In 2011, adult cardiologists from 8 facilities with active attitudes toward ACHD care formed the Task Force for Adult Congenital Heart Disease. The task force has since increased its number of member facilities to 33 as of December 2014. In addition, an ACHD division was established in the Japanese Circulation Society, and adult cardiologists are increasingly participating in the ACHD care field.12
Adult cardiologists’ participation in ACHD care has led to rapid advancement in regional ACHD center development in Japan. Consistent with this development, patients in some regions are now being transferred from pediatric departments to regional ACHD centers. Conversely, some patients remain with pediatric departments and some choose to withdraw during the transfer process; these are ongoing problems.13–15 However, there is no nationwide Japanese survey on transfer practices in pediatric departments.
The upcoming issue for Japan’s ACHD care system is development of cooperative systems between pediatric departments and regional ACHD centers to enable patients to receive optimal medical care throughout their lifetime. The present study clarified ACHD care in major pediatric departments throughout Japan and their transfer practices.
A self-directed questionnaire survey, comprising the items below, was mailed to pediatric cardiologists working at 149 facilities throughout Japan. The study period was February to June 2013. Pediatric cardiologists consenting to participate in the study then completed the questionnaire and returned it by mail. The participant inclusion criteria are shown below.
Participating Facilities The participating facilities in the present study were: (1) 141 facilities registered as teaching hospitals/hospital groups for the board certification system of the Japanese Society of Pediatric Cardiology and Cardiac Surgery; and (2) 29 member facilities in the Japanese Association of Children’s Hospitals and Related Institutions. In the type (1) facilities, at least 1 temporary board-certified instructor of pediatric cardiology was required; in the type (2) facilities, they were not mandatory. Type (1) and (2) facilities overlapped each other and totaled 149 facilities. At the time of this study, the board certification system of the Japanese Society of Pediatric Cardiology and Cardiac Surgery had just begun; temporary board-certified instructors were not always board-certified pediatric cardiologists. Additionally, in certain areas in Japan, type (2) facilities played central roles in the care of congenital heart disease without being registered as type (1) facilities.
Participant Qualification Criteria Temporary board certified instructors of pediatric cardiology affiliated with the participating facilities completed the questionnaires themselves or delegated the response to a pediatric cardiologist dedicated to ACHD care in their department. For facilities without temporary board-certified instructors, pediatric cardiologists were asked to participate.
Survey ItemsReferring to a questionnaire used in a previous study10 of adult cardiology departments, we developed an original questionnaire to ask participants about the following items:
1. Basic participant information;
2. Allocation of medical care staff in the facility;
3. Current situation and trends in ACHD care in the pediatric department;
4. Current transfer practice from the pediatric department; and
5. Facilities’ intentions to serve as regional ACHD centers themselves.
Statistical AnalysisNominal-level data were expressed as percentages, and means and standard deviations were calculated for continuous variables. For all tests shown below, statistical significance was defined as P<0.05. Data were analyzed with SPSS version 22.0 (SPSS Inc, Chicago, IL, USA).
Estimated Numbers of Patients Followed up at Pediatric DepartmentsIn survey item (3), participants were asked about the number of outpatients followed up per year in their facilities using a 5-point scale: 0–49, 50–99, 100–199, 200–499, and ≥500. Nationwide patient numbers were then estimated using the following definitions.
Estimated Maximum Number of Patients We multiplied the maximum value for each answer on the scale (49 in the case of 0–49, 3,000 in the case of ≥500 according to a previous report16 on 6 major Japanese pediatric cardiology departments) by the number of facilities selecting that answer to calculate the estimated maximum number of patients.
Estimated Median Number of Patients We multiplied the median value for each answer on the scale (25 for 0–49, 75 for 50–99, 150 for 100–199, 350 for 200–499, and 1,750 for ≥500) by the number of facilities selecting that answer to estimate the median number of patients. In order to correct for numbers of patients from non-responding facilities, we respectively multiplied maximum and median numbers of patients by 100/response rate.
Identification of Current Functioning Regional ACHD CentersWe referred to the criteria provided by Moons et al (Table 1).17 However, to identify the facilities that currently meet the conditions for regional ACHD centers and their geographic distribution, we established our own conditions and assessed the degree to which each facility met those conditions. We also assessed the degree to which optimal care structure was met in facilities that fulfilled the minimal requirements. The criteria for minimal requirements and optimal care structure are listed below.
| 1. An ACHD referral center must employ at least 1, preferably 2, cardiologist(s) specifically trained and educated in the care of adults with CHD. |
| 2. Specialist centers should provide care in connection with pediatric cardiology and/or congenital cardiac surgery. |
| 3. Specialist centers must treat sufficient numbers of patients and perform a sufficient number of procedures to be effective, and to develop and maintain high levels of performance. |
| 4. General adult cardiac facilities and non-specialist centers should have an established referral relationship with a specialist center. |
| 5. A minimum of 2 cardiac surgeons trained in and practicing adult and pediatric cardiac surgery are required. |
| 6. The optimal activity for a pediatric and congenital heart surgeon is 125 operations per year. Specifically for AHCD, a minimum of 50 operations per year is recommended. |
| 7. A fully equipped electrophysiology laboratory staffed by properly trained electrophysiologists with experience in detecting arrhythmias inherent to CHD and with experience in pacemaker technology, ablation technology, and defibrillator implantation must be available. |
| 8. An ACHD referral center must employ at least 1 nurse specialist that is trained and educated in the care of ACHD patients. |
For specialist centers, recommendations 1–3 should to be fulfilled. Complying with recommendations 5–8 is critical for achieving an optimal ACHD care program. ACHD, adult congenital heart disease.
Minimal Requirements
1. Intention to fulfill the regional ACHD center role;
2. At least 1 pediatric cardiologist or adult cardiologist specializing in ACHD;
3. At least 1 pediatric cardiologist, 1 pediatric cardiovascular surgeon, and 1 adult cardiologist;
4. Provision of complex surgical treatment for ACHD patients; and
5. At least 200 ACHD outpatients per year.
Currently, Japan has no board-certified ACHD specialists. Therefore, in the present study, we defined requirement (2) as “a pediatric cardiologist or adult cardiologist in charge of outpatients, at least 50% of whom are ACHD patients.”
Optimal Care Structure
1. A total of at least 2 pediatric cardiologists and/or adult cardiologists specializing in ACHD;
2. At least 2 pediatric cardiovascular surgeons;
3. MRI, 3DCT, CARTO/EnSite systems, or other electrophysiological equipment;
4. An obstetrics department;
5. A neurosurgery department;
6. A psychiatry department;
7. Performing at least 50 operations for ACHD per year.
After identifying currently functioning regional ACHD centers, we calculated the population that each center was expected to serve in each of Japan’s 8 regions.
Differences Among Facilities in Their Intention to Serve as Regional ACHD CentersACHD care consolidation is especially difficult in children’s hospitals. Many Japanese children’s hospitals are independent hospitals, not part of larger university hospitals. Consequently, when patients who visit independent children’s hospitals are transferred, they must be transferred to another facility (geographically separate from their current hospitals).9 Therefore, we divided the participating facilities into independent children’s hospitals and other types of facilities; “facilities’ intentions to serve as regional ACHD centers themselves” was then examined for both groups using Fisher’s exact test.
Ethical ConsiderationsThe ethics committee of Tokyo Women’s Medical University (Approval Number 2694) approved this study. We explained to participants via a letter of intent that we would not force them to participate in the survey, they would not suffer any disadvantage even if they did not consent, and we would not disclose personally identifiable information to third parties.
Of the 149 facilities invited to participate, satisfactory replies were obtained from 113 facilities (a response rate of 75.8%). Of the responding facilities, 89 (78.8%) pediatric departments were located in general/university hospitals and 12 (10.6%) were located in independent children’s hospitals. The mean age of the responding pediatric cardiologists was 50.2±6.9 years; 90 (79.6%) of them were board-certified pediatric cardiologists (Table 2).
| n or mean | % or SD | |
|---|---|---|
| Regions | ||
| Hokkaido | 5 | 4.4 |
| Tohoku | 6 | 5.3 |
| Kanto | 38 | 33.6 |
| Chubu | 20 | 17.7 |
| Kinki | 14 | 12.4 |
| Chugoku | 9 | 8.0 |
| Shikoku | 4 | 3.5 |
| Kyushu/Okinawa | 17 | 15.0 |
| Pediatric department forms | ||
| Located in general/university hospitals | 89 | 78.8 |
| Independent children’s hospitals | 12 | 10.6 |
| Other | 7 | 6.2 |
| No response | 5 | 4.4 |
| Pediatric cardiologists (n) | ||
| 0 | 3 | 2.7 |
| 1 | 9 | 8.0 |
| 2 | 26 | 23.0 |
| ≥3 | 75 | 66.4 |
| No response | 0 | 0.0 |
| Adult cardiologists | ||
| 0 | 17 | 15.0 |
| 1–5 | 15 | 13.3 |
| 6–10 | 23 | 20.4 |
| ≥11 | 51 | 45.1 |
| No response | 7 | 6.2 |
| Pediatric cardiovascular surgeons (n) | ||
| 0 | 30 | 26.5 |
| 1 | 29 | 25.7 |
| 2 | 24 | 21.2 |
| ≥3 | 29 | 25.7 |
| No response | 1 | 0.9 |
| Age of responders | 50.2 | ±6.9 |
| Responders’ years’ experience in congenital heart disease care | 20.6 | ±7.6 |
| Responders’ certifications | ||
| Board-certified pediatric cardiologists | 90 | 79.6 |
In 31 facilities (27.4%), there were 1 or more pediatric cardiologists specializing in ACHD. In 7 facilities (6.2%), there were 1 or more adult cardiologists specializing in ACHD (Table 3).
| n | % | |
|---|---|---|
| Pediatric cardiologists specializing in ACHD | ||
| 0 | 81 | 71.7 |
| 1 | 14 | 12.4 |
| 2 | 6 | 5.3 |
| ≥3 | 11 | 9.7 |
| No response | 1 | 0.9 |
| Adult cardiologists specializing in ACHD | ||
| 0 | 104 | 92.0 |
| 1 | 5 | 4.4 |
| 2 | 1 | 0.9 |
| ≥3 | 1 | 0.9 |
| No response | 2 | 1.8 |
| ACHD-specialized outpatient care | ||
| Yes | 22 | 19.5 |
| No | 88 | 77.9 |
| No response | 3 | 2.7 |
| Outpatients per year (n) | ||
| 0–49 | 48 | 42.5 |
| 50–99 | 23 | 20.4 |
| 100–199 | 16 | 14.2 |
| 200–499 | 18 | 15.9 |
| ≥500 | 8 | 7.1 |
| Hospitalizations per year (n) | ||
| 0–9 | 61 | 54.0 |
| 10–29 | 37 | 32.7 |
| 30–49 | 7 | 6.2 |
| ≥50 | 8 | 7.1 |
| Operations per year (n) | ||
| 0–9 | 84 | 74.3 |
| 10–29 | 24 | 21.2 |
| 30–49 | 2 | 1.8 |
| ≥50 | 3 | 2.7 |
| Surgical treatments performed | ||
| No surgical treatment provided | 25 | 22.1 |
| Only simple surgery provided | 28 | 24.8 |
| Complex surgical treatments provided | 57 | 50.4 |
| No response | 3 | 2.7 |
| Patients undergoing cardiac catheterization per year (n) | ||
| 0–9 | 76 | 67.3 |
| 10–29 | 23 | 20.4 |
| 30–49 | 6 | 5.3 |
| ≥50 | 7 | 6.2 |
| No response | 1 | 0.9 |
ACHD, adult congenital heart disease.
Twenty-two facilities (19.5%) included an ACHD specialty outpatient clinic. Twenty-six facilities (23.0%) followed 200 or more ACHD patients per year, whereas 48 (42.5%) followed fewer than 50 patients (Table 3). Estimated maximum number of patients followed up in pediatric departments throughout Japan was 53,819; the estimated median number of patients was 33,806. Based on the data from responding facilities, ~80% of patients were estimated to be treated in the facilities with 200 or more ACHD outpatients per year (Table 4).
| Outpatients (n) per year | n | Maximum (n) per category |
%* | Median (n) per category |
%* |
|---|---|---|---|---|---|
| 0–49 | 48 | 2,352 | 5.8 | 1,200 | 4.7 |
| 50–99 | 23 | 2,277 | 5.6 | 1,725 | 6.7 |
| 100–199 | 16 | 3,184 | 7.8 | 2,400 | 9.4 |
| 200–499 | 18 | 8,982 | 22.0 | 6,300 | 24.6 |
| ≥500 | 8 | 24,000 | 58.8 | 14,000 | 54.6 |
| Outpatients (n) in responding facilities | 40,795 | 100.0 | 25,625 | 100.0 | |
| Corrected for response rate (75.8%) | 53,819 | 33,806 |
*Calculated only from the estimating data of responding facilities.
Sixty-one facilities (54.0%) had fewer than 10 annual hospitalizations. Only 8 facilities had 50 or more hospitalizations. Although 57 facilities (50.4%) performed complex cardiac surgery for ACHD, 84 facilities (74.3%) performed fewer than 10 surgeries per year. Only 3 facilities (2.7%) performed 50 or more surgeries for ACHD annually (Table 3).
Intentions to Serve as Regional ACHD CentersA total of 61 facilities (54.0%) responded, “My facility intends to fulfill the regional ACHD center role.” Of these 61 facilities, 5 were independent children’s hospitals. No significant difference was observed in the percentages of independent children’s hospitals and other facilities in their intention to serve as regional ACHD centers (P=0.326; Table 5).
| Facilities’ intentions to serve as regional ACHD centers themselves |
Total (n=113) |
Independent children’s hospitals (n=12) |
Other facilities (n=96) |
P value* | |||
|---|---|---|---|---|---|---|---|
| n | % | n | % | n | % | ||
| “My facility intends to fulfill the regional ACHD center role.” | 61 | 54.0 | 5 | 41.7 | 53 | 55.2 | 0.326 |
| “Another facility should fulfill the regional ACHD center role.” | 29 | 25.7 | 6 | 50.0 | 22 | 22.9 | |
| “Consolidation in my region seems impractical.” | 16 | 14.2 | 1 | 8.3 | 14 | 14.6 | |
| “There is no need for consolidation in the first place.” | 1 | 0.9 | 0 | 0.0 | 1 | 1.0 | |
| No response | 6 | 5.3 | 0 | 0.0 | 6 | 6.3 | |
*Calculated using Fisher’s exact test. ACHD, adult congenital heart disease.
Table 6 shows the extent to which the minimal requirement criteria for regional ACHD centers were fulfilled by the participating facilities. Nine facilities (8.0%) fulfilled all minimal requirement criteria. Criteria fulfilled by less than half of the participating facilities included the existence of at least 1 pediatric cardiologist or adult cardiologist specializing in ACHD (26.5%), and the presence of at least 200 ACHD outpatients per year (23.0%).
| Minimal requirements | Facilities meeting this criterion (n=113) |
|
|---|---|---|
| n | (%) | |
| 1. Intention to fulfill the regional ACHD center role | 61 | 54.0 |
| 2. At least 1 pediatric cardiologist or adult cardiologist specializing in ACHD | 30 | 26.5 |
| 3. At least 1 pediatric cardiologist, 1 pediatric cardiovascular surgeon, and 1 adult cardiologist |
66 | 58.4 |
| 4. Provision of complex surgical treatment for ACHD patients | 57 | 50.4 |
| 5. At least 200 ACHD outpatients per year | 26 | 23.0 |
| Facilities fulfilling minimal requirements (n) | 9 | 8.0 |
| Optimal care structure | Facilities meeting this criterion (n=9) |
|
| n | (%) | |
| 1. A total of at least 2 pediatric cardiologists and/or adult cardiologists specializing in ACHD |
6 | 66.7 |
| 2. At least 2 pediatric cardiovascular surgeons | 7 | 77.8 |
| 3. MRI, 3DCT, CARTO system, or other electrophysiological equipment | 8 | 88.9 |
| 4. An obstetrics department | 8 | 88.9 |
| 5. A neurosurgery department | 9 | 100.0 |
| 6. A psychiatry department | 7 | 77.8 |
| 7. Performing at least 50 operations for ACHD per year | 2 | 22.2 |
| 8. At least 1 nurse specializing in ACHD | 2 | 22.2 |
| Currently functioning regional ACHD centers with optimal care structure (n) | 1 | 11.1 |
ACHD, adult congenital heart disease.
Table 6 also shows the extent to which these 9 facilities fulfilled optimal care structure criteria. Nationally, only 1 facility had an optimal care structure. Criteria fulfilled by less than half of the facilities included at least 50 operations for ACHD per year (22.2%) and the presence of at least 1 nurse specializing in ACHD (22.2%).
By using Japan’s vital statistics,18 the geographic distribution of the 9 facilities fulfilling all minimal requirements and the regional populations each facility was expected to serve are shown in the Figure. In 5 of the 8 regions, each facility was expected to serve a population of 7.0–23.6 million. In the other 3 regions, there were no currently functioning regional ACHD centers. Thus, in Japan as a whole, each existing center was projected to serve a population of 14.2 million.
Number and geographic distribution of the currently functioning regional adult congenital heart disease (ACHD) centers.
Sixty facilities (53.1%) reported that they currently continue treating ACHD patients in pediatric departments without transferring them to other departments or facilities. Forty facilities (35.4%) responded that they transfer patients from pediatric departments. Nine facilities responded that they make transfer judgments on a case-by-case basis. Of these 49 facilities, 19 facilities (38.8%) transfer patients to another department within their own facility; and 29 of the 49 (59.2%) transfer patients to adult cardiology departments. Thirty facilities (26.8%) established a graduation age from pediatric departments. It ranged from 15–30 years; 16 facilities (53.3%) established a graduation age younger than 18 years old (Table 7).
| n | % | |
|---|---|---|
| Current situation of transfer practice from pediatric departments | ||
| Continuing care in pediatric departments without transfer to another department or hospital | 60 | 53.1 |
| Patients transferred from pediatric department to another department or hospital | 40 | 35.4 |
| Transfer judgments made on a case-by-case basis | 9 | 8.0 |
| No response | 4 | 3.5 |
| Basic transfer destinations (n=49) | ||
| Adult cardiology department within the same facility | 18 | 36.7 |
| Department of cardiovascular surgery within the same facility | 1 | 2.0 |
| Adult cardiology department at another facility | 6 | 12.2 |
| Department of cardiovascular surgery at another facility | 3 | 6.1 |
| Adult cardiology department within the same facility or at another facility | 5 | 10.2 |
| Department of cardiovascular surgery within the same facility or at another facility | 3 | 6.1 |
| Not listed | 13 | 26.5 |
| Established graduation age from pediatric department? | ||
| Yes | 30 | 26.5 |
| No | 79 | 69.9 |
| No response | 4 | 3.5 |
| Graduation age (n=30) | ||
| ≤18 years | 16 | 53.3 |
| 18–30 years | 14 | 46.7 |
| Future intentions for transfer practice | ||
| Preference to transfer patients to specialized ACHD facilities if available | 55 | 48.7 |
| Preference to continue following patients in pediatric departments in collaboration with other departments and facilities |
34 | 30.1 |
| Other | 22 | 19.5 |
| No response | 2 | 1.8 |
Regarding future intentions for transfer practice, 55 facilities (48.7%) preferred to transfer patients to specialized ACHD facilities if available. However, 34 facilities (30.1%) preferred to continue following patients in pediatric departments in collaboration with other departments and facilities (Table 7).
This study is the first to demonstrate the current situation of ACHD care in pediatric departments and transfer practices from pediatric departments throughout Japan. The present study yielded the following 3 important findings: (1) there are only 9 currently functioning regional ACHD centers nationwide. Facilities with 50 or more annual inpatient and surgical treatments are particularly limited; (2) the median number of ACHD patients treated at pediatric departments nationwide is estimated to be approximately 34,000; (3) only slightly more than 30% of facilities transfer patients from pediatric departments. We will discuss each of these 3 points below.
(1) Limited Number of Currently Functioning Regional ACHD CentersACHD care provision requires multidisciplinary care. There are 9 currently functioning regional ACHD centers in Japan, or 1 for every 14.2 million people. A previous study of adult cardiology departments demonstrated that, as of 2011, there were 14 facilities with the potential to serve as regional ACHD centers in the future.10 Thus, the present study’s finding that there are 9 currently functioning regional ACHD centers in Japan is valid in the sense that it reflects the current state of Japanese ACHD care.
However, the figure of 1 regional ACHD center per 14.2 million people is far from international guidelines; they recommend a figure of 1 regional ACHD center per 3–10 million people.4–6 Minimum requirement criteria fulfilled by less than half of the participating facilities included the existence of at least 1 pediatric cardiologist or adult cardiologist specializing in ACHD, and the presence of at least 200 ACHD outpatients per year. Because in this study we defined “pediatric cardiologist or adult cardiologist specializing in ACHD” as a physician who is in charge of outpatients, at least 50% of whom are ACHD patients, both criteria fulfilled by less than half of the participating facilities were related to outpatient volume. Additionally, 42.5% of facilities treated <50 outpatients per year. These results suggest that the number of high volume centers for ACHD outpatient care is limited; the majority of patients are treated at these centers and others are treated at relatively small facilities in a scattered fashion. The 3 regions without currently functioning regional ACHD centers are the areas with the first, second and fourth smallest populations in Japan.16 As we noted in a previous study,9 ACHD care consolidation in rural areas is not feasible; establishing strong referral relationships between non-specialist centers in rural areas and regional ACHD centers in urban areas is more efficacious. In future studies, we should evaluate the network for cooperation among multiple facilities in different areas.
The present study also demonstrated that only 2 among the 9 facilities fulfilling all minimal requirement criteria completed the at least 50 operations for ACHD per year criterion in the optimal care structure; 54.0% of responding facilities admitted <10 inpatients per year and 74.3% performed <10 surgeries per year. Thus, the number of high-volume centers for inpatient care and surgical treatment for ACHD patients is much more limited than those for outpatient care. One study has shown that facilities performing more operations for ACHD demonstrate better outcomes in surgical treatment.19 Another study has demonstrated that mortality is reduced when ACHD patients are treated in cooperation with regional ACHD centers.20 As a regional ACHD center is defined as a facility that can respond to the latent medical needs of all ACHD patients, including surgical treatment,4–6 they should perform sufficient numbers of surgeries and provide high quality care. Correcting these issues in the future will require policy guidance toward establishing regional ACHD centers such as revising remuneration for facilities treating severe patients who require inpatient and surgical treatment.
In addition, as many as 61 facilities (54.0%) showed their intention to fulfill the regional ACHD center role. However, very limited numbers of them were admitting or performing sufficient numbers of inpatients/surgeries. A regional ACHD center is not synonymous with a high volume center for outpatient care. In Japan, careful discussion and clarification of the role divisions between regional ACHD centers and other facilities and the establishment of a cooperative network between them is necessary; the discussion should also consider the number of ACHD patients and health resources in each region.
(2) Numbers of Patients Treated at Pediatric DepartmentsThe present study demonstrated that the median number of ACHD patients treated at pediatric departments is estimated to be approximately 34,000. There may also be patients treated at cardiovascular surgery departments, as well as patients treated at smaller community clinics rather than the relatively large-scale facilities that participated in the present study. However, even if the number of such patients were estimated to be as high as the number of patients treated at pediatric departments, the estimated number of patients would be approximately 70,000. This is fewer than the figure of 130,000 moderate and severe patients demonstrated in a previous study.1 The major issue is lack of a method to determine the exact number of ACHD patients being treated in each facility and how many withdraw from treatment.
In Japan, there are 4 major databases of congenital heart disease patients: the Japanese Registry of All Cardiac and Vascular Diseases (JROAD), the Task Force for Adult Congenital Heart Disease Database, the Japan Congenital Cardiovascular Surgery Database (JCCVSD), and the Japan Pediatric Interventional Cardiology Database (JPIC-DB). However, JROAD is not specialized for congenital heart disease, while the Task Force for Adult Congenital Heart Disease Database only includes ACHD patients who visit the adult cardiology departments participating in the task force. The JCCVSD and JPIC-DB include patients who undergo surgical treatment and patients who undergo catheterization, respectively. Thus, patients only being treated at outpatient clinics are not included; this renders both incomplete as databases of ACHD patients. These databases must be consolidated in order to establish a system capable of monitoring trends in the care received by all congenital heart disease patients from childhood to adulthood.
Even in Canada, where regional ACHD centers and their network are established, only 47% of ACHD patients are transferred successfully to adult care; the necessity of multiple mechanisms such as referral letters and transition clinics has been suggested.11 The percentage of patients requiring urgent cardiac intervention is higher among patients lost to follow up than among patients who continuously receive care.21 Although insufficient regional ACHD centers and no formal transitional care programs have yet been established in Japan, work must be done to create guidelines and implement a model program to prevent treatment loss while referring to previous studies performed in the United States and Europe.22,23
(3) Transfer Practice From Pediatric DepartmentsWhile more than 50% of pediatric departments continued treating patients into adulthood, slightly more than 30% of facilities actively transferred patients from pediatric departments. The most common transfer locations were adult cardiology departments within the same facility. Since the establishment of the Task Force for Adult Congenital Heart Disease in 2011, increasingly more adult cardiologists have been participating in ACHD care. As of 2013, 8 adult cardiology congenital heart disease clinics have been established throughout Japan.12 Currently, transfer practice from pediatric departments to adult cardiology departments has begun, primarily to these adult cardiology congenital heart disease clinics. However, transfers to other facilities are still less common than transfers within the same facility. Transfers from independent children’s hospitals consequently entail transfer to other facilities. The establishment of methods for transfer practice from independent children’s hospitals is an urgent issue.
Of the 12 independent children’s hospitals that participated, 5 answered that they intended to serve as regional ACHD centers. Because independent children’s hospitals cannot easily accept adult inpatients or deal with adult diseases, they would find it difficult to function as regional ACHD centers.9,10 However, as previously discussed, the number of facilities admitting or performing sufficient inpatients/surgeries is currently very limited, with the exception of a few high-volume centers. Under these circumstances, if independent children’s hospitals can increase their cooperation with other departments and facilities to enable them to deal with adult diseases, they can at least function as high-volume centers for outpatient care. When surgery or pregnancy/delivery management is required, it may be necessary to refer patients to distant regional ACHD centers with sufficient experience in such care. This awareness may have been a factor in 5 independent children’s hospitals responding with intention to serve as consolidated facilities. Because the number of patients who receive inpatient care in distant regional ACHD centers is thus expected to increase, the ACHD care system in the future will require support for transportation and associated lodging costs.
Limitations of the Present Study and Future IssuesThere were a number of limitations in the present study. First, because we targeted ACHD care only in pediatric departments in this study, we did not investigate to what extent they collaborated with other departments in each facility and those of other facilities; ACHD care relies on both pediatric and adult specialists at the same facility and in some cases, at multiple facilities. Because some independent children’s hospitals showed their intention to serve as regional ACHD centers in cooperation with other departments and facilities, the results of this study might underscore the potential of an ACHD care delivery system where multiple facilities with different specialties collaborate and serve as regional ACHD centers as a whole.
Second, responses for numbers of patients used a 5-point scale. Because we prioritized the response rate and did not compel the participants to use internal data on the exact numbers of ACHD patients they follow, ACHD patient numbers may not be estimated precisely. In addition, although we asked all facilities about their basic transfer practice policies, we did not ask about individualized measures accounting for factors such as the patient’s severity and access to healthcare facilities. There are inherent limits to the ability of a questionnaire survey to estimate patient numbers and demonstrate detailed transfer practices. The existing databases must be consolidated in the future in order to construct a system capable of determining precise patient numbers.
We sent self-directed questionnaires to pediatric cardiologists working in 149 facilities throughout Japan and received responses from 113 facilities. The number of regional ACHD centers offering outpatient and inpatient care in sufficient volume is limited, and transfers from pediatric departments are not standard in Japan. Clarification of the role divisions between regional ACHD centers and other facilities, and the establishment of a cooperative network between them including transitional care programs, is necessary. The future direction of the ACHD care system should include: policy guidance toward establishing regional ACHD centers, such as revising remuneration for facilities treating severe patients who require inpatient and surgical treatment; construction of a database capable of monitoring care trends received by patients from childhood to adulthood; and support for the patients who receive care in distant regional ACHD centers.
We would like to offer our sincere gratitude to the contacts at the pediatric departments who participated in our study.
The present study was conducted as part of the “Research Related to the Establishment of a Care System for Adult Congenital Heart Disease Patients” (principal investigator: I.S., National Cerebral and Cardiovascular Center) under the Japanese Ministry of Health, Labour, and Welfare’s Comprehensive Research on Life-Style Related Disease including Cardiovascular Diseases and Diabetes Mellitus.
This research was supported by a grant to I.S. by the Japanese Ministry of Health, Labour, and Welfare’s Comprehensive Research on Life-Style Related Disease including Cardiovascular Diseases and Diabetes Mellitus.
