著者

Hiroki Mori Tadahiro Yoshikawa Hitomi Kimura Hiroshi Ono Hitoshi Kato Yasuo Ono Masaki Nii Takahiro Shindo Ryo Inuzuka Hitoshi Horigome Masaru Miura Shunichi Ogawa Junko Shiono Yoshiyuki Furutani Mikiko Ishido Toshio Nakanishi

出版者

The Japanese Circulation Society

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

vol.86, no.1, pp.109-115, 2021-12-24 (Released:2021-12-24)

参考文献数

21

被引用文献数

1 4

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Background:There has been no nationwide survey on the prognosis of pediatric dilated cardiomyopathy (DCM) in Japan. Therefore, we designed this retrospective multicenter study to investigate the long-term survival rate in pediatric patients with DCM in Japan.Methods and Results:In this multicenter retrospective observational study, data were reviewed for 106 patients aged <18 years who had been diagnosed with DCM at any 1 of 18 Japanese institutions between 1990 and 2014. The median age at diagnosis was 2.0 years and the median duration of observation was 3.3 years. Most DCM patients were diagnosed because of symptoms of heart failure. On echocardiography, the median left ventricular end-diastolic dimension z score was 5.4 and fractional shortening was 0.10. Freedom from death or transplantation rates at 1, 3, 5, 10, and 20 years after diagnosis were 76%, 66%, 64%, 58%, and 43%, respectively. Freedom from death rates at 1, 5, 10, and 20 years after diagnosis were 81%, 75%, 72%, and 53%, respectively. The incidence of heart transplantation at 1, 5, 10, and 20 years after diagnosis was 6%, 15%, 20%, and 20%, respectively, suggesting that only 15% of patients in Japan underwent heart transplantation within 5 years of diagnosis.Conclusions:In Japan, the prognosis of pediatric DCM is poor and the rate of heart transplantation is low.

著者

Shinichi Takatsuki Yoshiyuki Furutani Kei Inai Tohru Kobayashi Ryo Inuzuka Tomomi Uyeda Mitsuhiro Kamisago Jun Muneuchi Masahide Kaneko Yasushi Misaki Hiroshi Ono Hitoshi Kato Eriko Shimada Tokuko Shinohara Kenji Waki Kenji Suda Yasunobu Hayabuchi Hirotaka Ohki Reina Ishizaki Jun Maeda Hiroyuki Yamagishi

出版者

The Japanese Circulation Society

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

vol.84, no.12, pp.2270-2274, 2020-11-25 (Released:2020-11-25)

参考文献数

20

被引用文献数

5

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Background:Although advances in cardiac surgery have led to an increased number of survivors with congenital heart disease (CHD), epidemiological data regarding the pregnancies and deliveries of patients with repaired CHD are scarce.Methods and Results:In this study, we retrospectively reviewed the clinical outcomes of pregnancies and deliveries of women with repaired CHD. Overall, 131 women with repaired CHD were enrolled and there were 269 gestations. All patients were classified as New York Heart Association (NYHA) Class I or II. The prevalence of cesarean sections was higher in patients with (CyCHD) than without (AcyCHD) a past history of cyanosis (51% vs. 19%, respectively; P<0.01). There were 228 offspring from 269 gestations and the most prevalent neonatal complication was premature birth (10%), which was more frequent in the CyCHD than AcyCHD group (15.7% vs. 5.6%, respectively; P<0.01). Five maternal cardiac complications during delivery were observed only in the CyCHD group (8%); these were classified as NYHA Class II and none was fatal.Conclusions:Delivery was successful in most women with repaired CHD who were classified as NYHA Class I or II, although some with CyCHD and NYHA Class II required more attention. Cesarean sections were more common in the CyCHD than AcyCHD group, and CyCHD may be a potential risk for preterm deliveries.

著者

Ryuji Fukazawa Tohru Kobayashi Masashi Mikami Tsutomu Saji Kenji Hamaoka Hitoshi Kato Hiroyuki Suzuki Etsuko Tsuda Mamoru Ayusawa Masaru Miura Ryota Ebata Tomio Kobayashi Mayumi Yashiro Shunichi Ogawa

出版者

The Japanese Circulation Society

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

vol.82, no.1, pp.239-246, 2017-12-25 (Released:2017-12-25)

参考文献数

22

被引用文献数

38

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Background:Giant coronary aneurysm is the most severe sequela in Kawasaki disease, occurring in approximately 0.2% of patients in Japan. Regression is rare, while myocardial infarction (MI) and sudden death are relatively common. Herein, we reviewed patients with giant coronary aneurysm in a 10-year period.Methods and Results:A nationwide questionnaire survey was conducted based on a national epidemiological database from 1999 to 2010. We identified 355 giant coronary aneurysm patients, of whom 209 were analyzed. The 5- and 10-year total cardiac event-free rates were 0.72 and 0.68, respectively. Twelve patients died, and MI was observed in 32 patients (18.1%). Five and 6 deaths were due to coronary rupture and MI, respectively. All ruptures occurred within 1 month of onset, while most MI occurred within 18 months. There was no death beyond 2 years. Aneurysm size was significantly related to the occurrence of MI in both the right and left coronary arteries. At the time of writing, 55% of patients had no exercise limitations. And including patients who cannot perform strenuous exercises, 81% of patients were leading ordinary lives.Conclusions:Severe cardiac events are likely to occur within 2 years from onset of Kawasaki disease, while no deaths occurred beyond this time. Hence, careful monitoring is needed especially for the first 2 years. Most patients with giant coronary aneurysms can lead ordinary lives with appropriate management.

著者

Takahiko Kurasawa Hayato Nagasawa Eiko Nishi Hirofumi Takei Ayumi Okuyama Tsuneo Kondo Koji Nishimura Ryota Sakai Akiko Shibata Kentaro Chino Hiroe Ogawa Tatsuya Ito Koichi Amano Hitoshi Kato

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

vol.52, no.10, pp.1125-1130, 2013 (Released:2013-05-15)

参考文献数

30

被引用文献数

4 5

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A substantial number of patients with lupus nephritis (LN) are refractory to conventional glucocorticoid (GC) treatment. Although many of these patients respond to immunosuppressive drugs such as intravenous cyclophosphamide (IVCY), azathioprine (AZA), mizoribine, tacrolimus, cyclosporine A (CSA) and mycofenolate mofetil (MMF), some remain refractory to such therapies. Recent studies of multi-target therapies have reported effective outcomes for immunosuppression following renal transplantation and refractory LN when therapy consists of two or more immunosuppressive drugs with different mechanisms of action. We herein report a case of LN unresponsive to IVCY that was successfully treated with the addition of tacrolimus and discuss the usefulness of multi-target therapy for LN.