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.
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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 Items
Referring 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 Analysis
Nominal-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 Departments
In 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 Centers
We 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 |
|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.
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 Centers
ACHD 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 Considerations
The 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|
|Pediatric department forms|
|Located in general/university hospitals||89||78.8|
|Independent children’s hospitals||12||10.6|
|Pediatric cardiologists (n)|
|Pediatric cardiovascular surgeons (n)|
|Age of responders||50.2||±6.9|
|Responders’ years’ experience in congenital heart disease care||20.6||±7.6|
|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).
|Pediatric cardiologists specializing in ACHD|
|Adult cardiologists specializing in ACHD|
|ACHD-specialized outpatient care|
|Outpatients per year (n)|
|Hospitalizations per year (n)|
|Operations per year (n)|
|Surgical treatments performed|
|No surgical treatment provided||25||22.1|
|Only simple surgery provided||28||24.8|
|Complex surgical treatments provided||57||50.4|
|Patients undergoing cardiac catheterization per year (n)|
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 |
|%*||Median (n) per|
|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 Centers
A 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
|Independent children’s |
|Other facilities |
|“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|
*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 |
|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 |
|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 |
|1. A total of at least 2 pediatric cardiologists and/or adult cardiologists specializing |
|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).
|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|
|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|
Теперь он молил Бога, чтобы священник не торопился, ведь как только служба закончится, он будет вынужден встать, хотя бы для того чтобы пропустить соседей по скамье. А в своем пиджаке он обречен. Беккер понимал, что в данный момент ничего не может предпринять.
Ему оставалось только стоять на коленях на холодном каменном полу огромного собора.