著者
Ryo Inuzuka Hisateru Tachimori Sung-Hae Kim Hikoro Matsui Tohru Kobayashi Atsuko Kato Takanari Fujii Mami Ho Hanako Morikawa Sara Takahashi Haruki Shirato Yuji Haishima Yoshihiro Okamoto Hideyuki Sakoda Hideshi Tomita
出版者
The Japanese Circulation Society
雑誌
Circulation Journal (ISSN:13469843)
巻号頁・発行日
pp.CJ-22-0185, (Released:2022-09-01)
参考文献数
24
被引用文献数
2

Background: Balloon atrial septostomy (BAS) is an essential catheterization procedure for congenital heart lesions. Recently, a balloon catheter for static BAS was approved for the first time in Japan as an alternative to the conventional pull-through BAS. Despite the expected increase in the use of static BAS, reports on its safety are scarce worldwide.Methods and Results: Data on static and pull-through BAS registered in a national registry between 2016 and 2018 were collected. During the study period, 247 sessions of static BAS and 588 sessions of pull-through BAS were performed on a total of 674 patients. Patients who underwent static BAS were older (P<0.001). The incidence of serious adverse events (4.3% vs. 0.9%, P=0.03) and the overall incidence of adverse events (8.1% vs. 3.2%, P=0.03) were higher in static BAS than in pull-through BAS. Among patients who underwent static BAS, the risk factor for adverse events was a body weight <3 kg at the time of the procedure (odds ratio: 4.3 [confidence interval: 1.7–11], P=0.003).Conclusions: This nationwide study revealed differences in patient background between static and pull-through BAS, as well as a higher incidence of adverse events related to static BAS. Patients weighing <3 kg are at high risk for adverse events after static BAS and may require surgical and circulatory support backup.
著者
Norifumi Takeda Hiroki Yagi Hironori Hara Takayuki Fujiwara Daishi Fujita Kan Nawata Ryo Inuzuka Yuki Taniguchi Mutsuo Harada Haruhiro Toko Hiroshi Akazawa Issei Komuro
出版者
一般社団法人 インターナショナル・ハート・ジャーナル刊行会
雑誌
International Heart Journal (ISSN:13492365)
巻号頁・発行日
pp.16-094, (Released:2016-05-13)
被引用文献数
1 50

Marfan syndrome (MFS) is an autosomal dominant heritable disorder of connective tissue that affects the cardiovascular, skeletal, ocular, pulmonary, and nervous systems and is usually caused by mutations in the FBN1 gene, which encodes fibrillin-1. MFS is traditionally considered to result from the structural weakness of connective tissue. However, recent investigations on molecular mechanisms indicate that increased transforming growth factor-β (TGF-β) activity plays a crucial role in the pathogenesis of MFS and related disorders, such as Loeys–Dietz syndrome (LDS), which is caused by mutation in TGF-β signaling-related genes. In addition, recent studies show that angiotensin II type 1 receptor (AT1R) signaling enhances cardiovascular pathologies in MFS, and the angiotensin II receptor blocker losartan has the potential to inhibit aortic aneurysm formation. However, the relationship between TGF-β and AT1R signaling pathways remains poorly characterized. In this review, we discuss the recent studies on the molecular mechanisms underlying cardiovascular manifestations of MFS and LDS and the ensuing strategies for management.
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

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

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.