Current Circumstances Regarding End-of-Life Among Adult Congenital Heart Disease Patients in Japan
Article ID: CJ-24-0043
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Article ID: CJ-24-0043
Currently, >97% of children with congenital heart disease (CHD) survive and reach adulthood because of the developments in congenital heart surgery, cardiac intensive care, and medical treatment in early childhood.1 In Japan, the number of adult patients with CHD has increased over the past decades and is now estimated to exceed 500,000. Each prefecture had at least one hospital certified by the Japanese Society of Adult CHD (ACHD). However, although the number of adult patients with CHD has increased, life expectancy is still not normal worldwide.2,3 Moreover, ACHD patients tend to experience unplanned admissions due to several cardiovascular events, including life-threatening complications.4 Therefore, end-of-life (EOL) care for ACHD patients should be considered part of routine care.
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In this issue of the Journal, Akiyama et al5 report the in-hospital death and EOL status among ACHD patients (aged ≥15 years), including age at death and treatment details before death, using the Japanese Registry of All Cardiac and Vascular Disease and Diagnosis Procedure Combination database, which is a nationwide administrative claims database (Figure). This is the first study to investigate the EOL status of Japanese patients with ACHD and provides important information and outlines the future challenges to healthcare providers in this field.
End-of-life status among Japanese patients with ACHD.
Regarding the age at death, Akiyama et al report a median (interquartile range) age at in-hospital death of 69.0 (45–81) years in ACHD patients, with 77.0 (65–84), 66.5 (45–78), and 39.0 (30.5–60.5) years for patients with mild, moderate, and severe disease, respectively.5 Japanese pediatric cardiologists, cardiac surgeons, and adult cardiologists should realize that the life expectancy of ACHD patients is shorter than that of the general population and that ACHD patients with great disease complexity die extremely young (i.e., in their 30s–50s). A study at the Hospital for Sick Children in Toronto showed that CHD patients aged 16–20 years were expected to live until 75±11 years of age,6 and patients with moderate CHD are expected to live until 78 years of age (range, 50–120 years); even those with complex CHD are expected to live until 70 years of age (range, 30–99 years). A recent study from the Netherlands also identified significant differences between estimated life expectancy by cardiologists and patients (48.3±4.8 years old) with moderate/severe defects:2 approximately 65% of the female patients with moderate/severe CHD considered their life expectancy to be normal, whereas cardiologists considered it to be normal in only 20%; 50% of male patients with moderate/severe CHD consider their life expectancy to be normal compared with 8% of cardiologists having the same opinion.2 It is unknown how Japanese patients with ACHD consider their life expectancy, and whether they think the same way as patients in Europe and North America, but better understanding of the age at death and EOL status based on the present study5 may facilitate their life planning, as well as timely discussion of both EOL and advance care planning (ACP).
Regarding EOL communication, it is difficult for both patients and healthcare providers to discuss this issue when patients are doing well. Many healthcare providers in Japan may consider it a taboo. However, a delayed discussion on EOL reportedly increases the decision-maker’s feeling of guilt (mostly partners or other family members) and reduces the quality of life of the ACHD patient.7,8 Moreover, it may be associated with prolonged and/or aggressive treatment and even unwanted treatment before death.9,10 Akiyama et al5 reveal that Japanese patients with ACHD with more complex diseases have undergone invasive treatment before death, and >25% of patients with disease of any complexity died >31 days after admission. Because they analyzed a nationwide administrative claims database, they could not investigate whether deceased ACHD patients had discussed EOL care and ACP before their last admission; however, it can be anticipated that few ACHD patients would voluntarily discuss ACP before a life-threatening event. A recent study of EOL communication revealed that 49.3% of patients and 41.7% of their partners wanted to discuss EOL, but 85.1% of patients reported that they had never discussed it with a healthcare professional.2 This trend in EOL communication may differ between countries, because of differences in medical circumstances and cultures. Thus it is necessary to facilitate timely and adequate discussions on EOL and ACP between patients and pediatric/adult cardiologists according to the patients’ disease complexity.11
The analyses of the distance between the hospital and the patient’s residence in Akiyama et al’s study are unique5 and may provide new insights into the medical care provision system in this field. Deceased patients with greater disease complexity were more likely to be admitted to JSPCCS-certified hospitals and patients with more complex disease were admitted to more distant hospitals. This information is important for developing ACHD care provision systems and healthcare economics. The strength of using nationwide administrative claims databases is the ability to perform this type of analysis.
Future studies examining EOL care provision and ACP in light of patients’ needs and preferences, and how the healthcare system can adequately respond, are awaited.
The author declares no conflict of interest.
None.
