Hospitalizations in Adult Patients With Congenital Heart Disease in Japan
Article ID: CJ-23-0822
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Article ID: CJ-23-0822
Owing to recent advances in diagnosis and treatment, especially after surgical improvements in the 1980s, more than 95% of patients with congenital heart disease (CHD) now reach adulthood. However, several related challenges have arisen, including residual disease and complications that require long-term follow-up. Specialist visits have been reported to improve the prognosis of adult patients with CHD (ACHD)1 and reduce invasive treatments after hospitalization.2 International studies indicate that the number of hospitalizations of ACHD patients has increased in recent years, in line with the increase in the number of ACHD patients, and hospitalization for heart failure correlates with patient prognosis.3
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In this issue of the Journal, the study by Kuraoka et al4 extracted all unplanned admissions of patients with ACHD from the Japanese Registry of All Cardiac and Vascular Diseases-Diagnosis Procedure Combination (JROAD-DPC) data (2012–2018) to analyze the characteristics of the admissions, which is valuable information for understanding the broader clinical issues associated with ACHD. However, it is important to consider the clinical backgrounds of the ACHD outpatients, as recently reported by our group,5 when interpreting the findings of Kuraoka et al. To facilitate comparison, we reorganized the admission data from their paper,4 and the number of ACHD outpatients in our paper5 for each variant of ACHD, as shown in the Table. Please note that the disease names differ somewhat in the 2 studies. Furthermore, patients with moderate and severe ACHD in our study, excluding those with congenitally corrected transposition of the great arteries (ccTGA), incomplete atrioventricular septal defect (AVSD), and Ebstein’s anomaly, were expected to have undergone intracardiac repair, whereas ≥30–40% of patients with mild ACHD may not have undergone repair.5
Numbers (%) of Planned and Unplanned Admissions in the Study by Kuraoka et al4 vs. Adult Outpatients With Congenital Heart Disease in the JNCVD-ACHD Registry
| Complexity / Main diagnostic name | Total no. of admissions (%)4 |
No. of unplanned admissions (%)4 |
Ratio of unplanned admissions (%) per total admissions (%)4 |
No. of outpatients (%)5 |
|---|---|---|---|---|
| Severe | ||||
| Single ventricle (SV) | 2,982 (7.5%) | 1,048 (10.0%) | 1.3 | 1,592 (6.6%) |
| Pulmonary atresia with intact ventricular septum (PA-IVS) |
66 (0.2%) | 21 (0.2%) | 1 | 334 (1.4%) |
| Persistent truncus arteriosus (PTA) | 248 (0.6%) | 69 (0.7%) | 1.17 | 91 (0.4%) |
| Transposition of the great arteries (TGA) | 1,070 (2.7%) | 259 (2.5%) | 0.93 | 993 (4.1%) |
| Congenitally corrected transposition of the great arteries (ccTGA) |
978 (2.5%) | 331 (3.2%) | 1.28 | 589 (2.4%) |
| Moderate | ||||
| Pulmonary atresia with ventricular septal defect (PA-VSD) |
440 (1.1%) | 123 (1.2%) | 1.09 | 599 (2.5%) |
| Tetralogy of Fallot (TOF) | 3,288 (8.3%) | 1,037 (9.9%) | 1.19 | 3,093 (12.9%) |
| Total anomalous pulmonary venous return (TAPVR) | 179 (0.5%) | 36 (0.3%) | 0.6 | 292 (1.2%) |
| Atrioventricular septal defect (AVSD) | 901 (2.3%) | 312 (3.0%) | 1.3 | 1,089 (4.5%) |
| Ebstein disease | 578 (1.5%) | 180 (1.7%) | 1.13 | 390 (1.6%) |
| Coactation of aorta/interrupted aortic arch (CoA) | 492 (1.2%) | 91 (0.9%) | 0.75 | 807 (3.4%) |
| Double outlet right ventricle (DORV) | 746 (1.9%) | 275 (2.6%) | 1.36 | – |
| Mild | ||||
| Ventricular septal defect (VSD) | 4,411 (11.1%) | 1,344 (12.9%) | 1.16 | 4,920 (20.5%) |
| Atrial septal defect (ASD) | 1,4765 (37.2%) | 3,063 (29.3%) | 0.79 | 4,941 (20.5%) |
| Patent ductus arteriosus (PDA) | 1,089 (2.7%) | 272 (2.6%) | 0.96 | 599 (2.5%) |
| Other | ||||
| Pulmonary stenosis (PS) | 661 (1.7%) | 84 (0.8%) | 0.47 | 630 (2.6%) |
| Aortic stenosis (AS) | 450 (1.1%) | 114 (1.1%) | 1 | 503 (2.1%) |
| Bicuspid aortic valve (BAV) | 1,482 (3.7%) | 252 (2.4%) | 0.65 | 951 (4.0%) |
| Mitral valve disease (MV disease) | 219 (0.6%) | 105 (1.0%) | 1.67 | 287 (1.2%) |
| Patent FORAMEN OVALE (PFO) | 1,437 (3.6%) | 590 (5.6%) | 1.56 | – |
| Other | 3,194 (8.1%) | 838 (8.0%) | 0.99 | 1,348 (5.6%) |
In the JNCVD-ACHD registry, DORV patients are registered as TGA, ccTGA, TOF, SV, and VSD from embryologic and anatomic perspectives, and PFO patients are classified as other.
The first thing we should notice is that for atrial septal defect (ASD), the percentage of admissions (37.2%) was extremely high, considering the percentage (20.5%) of ASD outpatients, whereas the percentage of admissions for severe ACHD, despite their severity, did not show such high numbers relative to outpatient numbers, and was even low for moderate ACHD (Table). One possible explanation for the high admission rate among ASD patients is the inclusion of both adults cases and cases previously deemed unsuitable for repair based on the guidelines at that time. It is well known that the incidence of heart failure and arrhythmias increases with age in ASD patients if shunt closure is not performed.6 Unlike other guidelines,7 the Japanese guidelines previously used pulmonary body blood flow ratio (Qp/Qs) >1.5 of as an indication for ASD closure,8 a criterion that many adult cardiologists have adhered to. In contrast, other guidelines suggest that proof of right-sided volume overload, such as right atrial and/or ventricular dilatation, is sufficient indication for ASD closure.7 Thus in Japan, patients with Qp/Qs <1.5 might have been lost to follow-up until serious symptoms due to heart failure and arrhythmias, stroke, etc., emerge. The high prevalence of heart failure as a comorbidity among patients with mild ACHD (48.5%), particularly in those with ASD, lends support to this concern. Related to ASD, the ratio of unplanned to all admissions was relatively high (1.56) among patients with patent foramen ovale (PFO; Table), indicating that unplanned admissions are common among cases of mild ACHD. These unplanned admissions seem to be primarily attributable to stroke resulting from thromboembolism via a right-to-left shunt through the PFO. A previous study revealed that recurrence of cryptogenic cerebral infarctions in patients at age 16–60 years was completely and significantly prevented not by anticoagulation or antiplatelet therapy but by shunt closure,9 further suggesting that the presence of cryptogenic infarctions is indicative of shunt closure for PFO and ASD of all sizes. In the modern era, we should note that shunt closure of type II ASD and PFO can be safely performed “percutaneously”. Taken together, shunt closure, especially percutaneous closure, should be widely applied for more cases of ASD and PFO, resulting in a reduction in unplanned admissions for these patients.
Another noteworthy aspect is the low admission rate among patients with ventricular septal defect (VSD), a mild ACHD typically diagnosed in childhood due to evident symptoms such as a holo-systolic murmur. Although surgical repair is often recommended, certain factors, including small shunt size, serious comorbidities such as pulmonary hypertension, and technical difficulties in surgery, may preclude surgical intervention in some patients. Considering the high percentage of outpatients with VSD, the percentages of all and unplanned admissions did not appear to be high (Table), possibly because VSD is a relatively mild disease among cases of ACHD.
Another notable aspect is the characteristics of the admissions among patients with severe and moderate ACHD, conditions that were typically lethal without intracardiac repair before adulthood. Hence, patients with these forms of ACHD born before the artificial heart–lung system was established (i.e., before 1970–1980) did not survive, and therefore were rarely included in the study by Kuraoka et al.4 The numbers of all and unplanned admissions were lower for patients aged >50 years than among those aged <50 years with moderate and severe ACHD (table 3 in the paper by Kuraoka et al4). Moreover, the ratio of deaths during unplanned admission was quite high at age ≥60 years for moderate ACHD (figure 3 in the paper by Kuraoka et al4). Because the number of patients aged ≥60 years admitted with severe ACHD did not reach the number of individuals with moderate ACHD, these patients may represent cases of stable survival such as patients with congenitally corrected transposition of the great arteries but no cyanosis, and non-single ventricle patients. It is not possible to completely interpret these issues in Kuraoka et al’s report.4 However, the comparison ratio between moderate and severe ACHDs among the total admissions has been increasing annually (figure 2 in the in the paper by Kuraoka et al4), confirming that a population of patients who have undergone intracardiac repair has grown up to adulthood and thus suggesting that the results presented by Kuraoka et al will change in the future.
Finally, if DPC data can be linked to registry data in the future, or if it becomes available for clinical studies, it is expected that more detailed analysis and long-term tracking of various pathophysiologies will be possible.
The authors declare no conflicts of interest.
