Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery最新文献_第7页

Clinical Features of Congenital Heart Disease Accompanied by Congenital Intestinal Atresia 先天性心脏病合并先天性肠闭锁的临床特点

Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery Pub Date : 2021-08-01 DOI: 10.9794/jspccs.37.117

Y. Hirano, Eiji Ehara, Y. Murakami, Kae Nakamura, Takeshi Sasaki, M. Fujino, Yuki Kawasaki, K. Nishigaki, Y. Yoshida, Tsugutoshi Suzuki

引用次数: 0

Clinical Features and Perioperative Management of Congenital Tracheal Stenosis in Patients with Congenital Heart Diseases 先天性心脏病患者气管狭窄的临床特点及围手术期处理

Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery Pub Date : 2021-08-01 DOI: 10.9794/jspccs.37.78

T. Hasegawa

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Unique Regional System of Heart Disease Screening in Schools 独特的学校心脏病筛查区域系统

Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery Pub Date : 2021-08-01 DOI: 10.9794/jspccs.37.104

Yuichiro Hirata

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A Large Family Report of Catecholaminergic Polymorphic Ventricular Tachycardia with Sudden Cardiac Death 儿茶酚胺能多态性室性心动过速合并心源性猝死的大家族报告

Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery Pub Date : 2021-08-01 DOI: 10.9794/jspccs.37.144

Yuko Saito, J. Sato, N. Takada, T. Aiba

{"title":"A Large Family Report of Catecholaminergic Polymorphic Ventricular Tachycardia with Sudden Cardiac Death","authors":"Yuko Saito, J. Sato, N. Takada, T. Aiba","doi":"10.9794/jspccs.37.144","DOIUrl":"https://doi.org/10.9794/jspccs.37.144","url":null,"abstract":"Catecholaminergic polymorphic ventricular tachycardia (CPVT), one of the inherited fatal arrhythmic syn-dromes, is usually diagnosed as bi-directional ventricular tachycardia during exercise. CPVT often causes faint-ness and/or cardiac arrest in younger adults resulting in a poor prognosis, with 60 ％ ten-year survival. Although 50 ‒ 60 ％ of CPVT is caused by the mutation in the cardiac ryanodine receptor gene ( RYR2 ), most are sporadic and familial cases are rare. Here we report familial CPVT cases of a 34-year-old woman (proband) and her two sisters. All of them had been implanted with a cardioverter defibrillator (ICD) due to syncope or resuscitated after ventricular fibrillation (VF). Additionally, five of her family members died in their thirties. The genetic study identified a novel pathogenic variant, F4087L, in the RYR2 gene in the proband, her sisters, and proband ʼ s son and niece. Even after ICD implantation, defibrillator shocks were needed to cope with VF in the proband and her sisters. However, additional pharmacological therapies such as beta-blockers, flecainide, and Ca channel blockers could suppress the recurrence of syncope or VF in all patients. These findings suggest that early clinical and genetic diagnosis for CPVT may provide appropriate pharmacological and non-pharmacological therapies to patients and their asymptomatic family members, including infants, for primary prevention of sudden death.","PeriodicalId":89701,"journal":{"name":"Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46581021","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Post-Transplant Lymphoproliferative Disorders (PTLD) in Pediatric Heart Transplantation 儿童心脏移植后淋巴细胞增生性疾病(PTLD)

Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery Pub Date : 2021-08-01 DOI: 10.9794/JSPCCS.37.141

N. Fukushima, 福嶌教偉

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A Neonatal Case of Congenital Femoral Arteriovenous Fistula 新生儿先天性股动静脉瘘1例

Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery Pub Date : 2021-07-01 DOI: 10.24509/JPCCS.21-007

Ryosuke Matsui, R. Fukazawa, Makoto Watanabe, K. Tsuno, M. Abe, S. Kenji, Takashi Sasaki, Y. Ishii, Y. Itoh

引用次数: 0

Ruptured Mitral Chordae Tendineae Due to Incomplete Kawasaki Disease after Spontaneous Defervescence: A Case Report 不完全性川崎病自然消退后二尖瓣腱断裂1例报告

Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery Pub Date : 2021-07-01 DOI: 10.24509/JPCCS.21-008

O. Matsuo, Fumiya Miyamura, K. Fukae, Y. Komohara

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Pandemic of Coronavirus Disease 2019 and Pediatric Cardiology 2019冠状病毒大流行与儿科心脏病学

Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery Pub Date : 2021-04-01 DOI: 10.9794/jspccs.37.1

K. Suda

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Fenestrated Closure of an Atrial Septal Defect for Left Ventricular Diastolic Dysfunction in an Early Infant with Hypertrophic Cardiomyopathy 开窗闭合心房间隔缺损治疗早期肥厚型心肌病婴儿左室舒张功能障碍

Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery Pub Date : 2021-04-01 DOI: 10.9794/jspccs.37.44

Ken Hayashi, T. Hasegawa, Shoichi Horiguchi, Toshikatsu Tanaka

{"title":"Fenestrated Closure of an Atrial Septal Defect for Left Ventricular Diastolic Dysfunction in an Early Infant with Hypertrophic Cardiomyopathy","authors":"Ken Hayashi, T. Hasegawa, Shoichi Horiguchi, Toshikatsu Tanaka","doi":"10.9794/jspccs.37.44","DOIUrl":"https://doi.org/10.9794/jspccs.37.44","url":null,"abstract":"Hypertrophic cardiomyopathy (HCM) is generally accompanied with left ventricular diastolic dysfunction, leading to a poor prognosis in patients with congenital heart diseases. An atrial septal defect (ASD) rarely needs surgical intervention in infancy. However, we report a case of HCM-complicated ASD that required fenestrated ASD closure in early infancy. A 5-month-old girl with cardiofaciocutaneous syndrome was transferred to our institution because of severe lung congestion triggered by fever and convulsions. Echocardiography revealed right ventricle enlargement secondary to left ventricular diastolic dysfunction and severe pulmonary hypertension. She was intubated and supported with mechanical ventilation, with nitric oxide inhalation. Immediately, her ventricular interdependence and circulatory dynamics improved. On hospitalization day 18, cardiac catheterization showed pulmonary hypertension improvement with high pulmonary blood flow and low cardiac out-put. However, she had severe lung congestion triggered by a repeat convulsion on day 60. Thus, we performed ASD partial closure with 4 mm fenestration. Postoperative cardiac catheterization showed a good control of pulmonary blood flow, with a Qp/Qs of 1.3. In conclusion, fenestrated ASD closure is a safe and effective treatment option for left ventricular diastolic dysfunction in early infants with ASD and HCM.","PeriodicalId":89701,"journal":{"name":"Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47410243","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Direct Reprogramming as a Novel Approach for Cardiovascular Regeneration 直接重编程作为心血管再生的新方法

Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery Pub Date : 2021-04-01 DOI: 10.9794/jspccs.37.10

R. Fujita, Taketaro Sadahiro, M. Ieda

{"title":"Direct Reprogramming as a Novel Approach for Cardiovascular Regeneration","authors":"R. Fujita, Taketaro Sadahiro, M. Ieda","doi":"10.9794/jspccs.37.10","DOIUrl":"https://doi.org/10.9794/jspccs.37.10","url":null,"abstract":"Direct cellular reprogramming provides a new strategy for tissue regeneration, especially to the adult heart. However, endogenous cardiomyocytes have limited regenerative capacity. Since our first report of direct cardiac reprogramming from cardiac fibroblasts with three cardiac developmental transcriptional factors (Gata4, Mef2c, and Tbx5), numerous international studies have been reported to reveal the molecular mechanisms of cardiac reprogramming and improve the efficiency of this nascent technology for cardiac regenerative medicine. These advancements include additional transcriptional factors, microRNAs, epigenetic factors, secreted factors, and cellular microenvironment. Cardiac reprogramming of human fibroblasts has also improved. Although direct cardiac reprogramming may resolve the difficulties in applying induced pluripotent stem cells for cardiac regeneration, numerous challenges remain for future clinical application. In this review, we will provide an update on recent advances in research on direct cardiac reprogramming and discuss the perspectives and challenges of this technology in clinical applications.","PeriodicalId":89701,"journal":{"name":"Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41955552","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0