Hiromitsu Shirozu, Y. Ishikawa, Ayako Kuraoka, Y. Kodama, Makoto Nakamura, H. Ushinohama, Koichi Sagawa, Joji Hashimoto, Y. Ando, T. Nakano
{"title":"Epicardial Pacemaker Lead-induced Cardiac Strangulation Causing Asystole due to Unexpected Myocardial Ischemia during Catheterization: A Case Report","authors":"Hiromitsu Shirozu, Y. Ishikawa, Ayako Kuraoka, Y. Kodama, Makoto Nakamura, H. Ushinohama, Koichi Sagawa, Joji Hashimoto, Y. Ando, T. Nakano","doi":"10.9794/jspccs.37.35","DOIUrl":"https://doi.org/10.9794/jspccs.37.35","url":null,"abstract":"A 16-year-old male with complete congenital atrioventricular block since 1 year old was treated by epicardial pacemaker lead implantation. At 9 years old, he was diagnosed with supravalvular pulmonary artery stenosis resulting from epicardial pacemaker leads with cardiac strangulation as the possible cause. Transient ST change was seen only in the exercise tolerance test, with no chest pain, indicating that myocardial ischemia was absent. The deterioration status of supravalvular pulmonary artery stenosis was assessed by catheterization. With his arms raised, he was injected with a small amount of contrast media, suddenly, he lost consciousness. Electrocardiography showed ST segment alterations after frequent premature ventricular contractions, followed by junc-tional bradycardia that rapidly progressed to asystole. Hence, transient ischemia caused by arm raising or frequent premature ventricular contractions and deterioration of the coronary artery compression was suspected. The leads were then removed in an urgent surgery. In conclusion, contrast-enhanced computed tomography should be conducted in patients with cardiac strangulation, and earlier lead exchange should be performed because of the rapid deterioration of myocardial ischemia.","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":"44565092","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}
{"title":"Updates in Basic Research on Molecular Genetic Background of Pulmonary Hypertension","authors":"Ayako Nagai","doi":"10.9794/jspccs.37.2","DOIUrl":"https://doi.org/10.9794/jspccs.37.2","url":null,"abstract":"With dramatic advances in the eld of molecular genetics, the investigation of the genetic background in pulmonary hypertension has also remarkably improved. In 2018, the 6th World Symposium on Pulmonary Hypertension (6th WSPH) identi ed 17 genes as causative factors for pulmonary arterial hypertension (PAH). In this review, the author discusses the candidate causative genes of PAH that have been identi ed consecutively a er the 6th WSPH. Furthermore, interesting reports on new modi er and susceptibility genes for PAH and the methods of interpreting these genes are discussed. is review also presents other noteworthy basic studies.","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":"45448373","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}
Y. Miki, Toshikatsu Tanaka, Michio Matsuoka, Naoya Kamei, Yoshiharu Ogawa, K. Tominaga, S. Kido
{"title":"Secondary Cardiomyopathy Caused by Carnitine Deficiency with Short Bowel Syndrome","authors":"Y. Miki, Toshikatsu Tanaka, Michio Matsuoka, Naoya Kamei, Yoshiharu Ogawa, K. Tominaga, S. Kido","doi":"10.9794/jspccs.37.51","DOIUrl":"https://doi.org/10.9794/jspccs.37.51","url":null,"abstract":"Secondary cardiomyopathy related to a systemic disorder should be ruled out when diagnosing idiopathic cardiomyopathy. Nutrient deficiency is a known cause of secondary cardiomyopathy. Herein, we report a case of secondary cardiomyopathy caused by carnitine deficiency with short bowel syndrome. A 6-year-old girl receiving total parenteral nutrition for short bowel syndrome manifested cold symptoms for 5 days that resulted in orthopnea, thereby prompting hospitalization. Chest radiography showed a cardiothoracic ratio of 60 % , and echocardiography revealed fraction shortening of 17 % . Her operated bowel was bleeding, causing chronic anemia, obtaining a hemoglobin level of 6.7 g/dL. Cardiac function was not improved by blood transfusion and milrinone therapy but improved in merely 3 days by L-carnitine administration. The free carnitine level was found to be low after treatment (17.8 µ mol/L); therefore, she was diagnosed with secondary cardiomyopathy caused by carnitine deficiency. In conclusion, carnitine deficiency can cause secondary cardiomyopathy; hence, the serum carnitine level of patients at risk should be examined periodically. Furthermore, the risk of cardiomyopathy must be considered in patients with increased cardiac load, including anemia and infection. Thus, early administration of L-carnitine is necessary for secondary cardiomyopathy caused by carnitine deficiency.","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":"46753257","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}
D. Hazeki, Naohiro Shiokawa, K. Ueno, R. Kusubae, Y. Nomura, M. Yoshinaga
{"title":"Reference Values for the Automatic Screening of QT Prolongation in School Cardiac Examinations","authors":"D. Hazeki, Naohiro Shiokawa, K. Ueno, R. Kusubae, Y. Nomura, M. Yoshinaga","doi":"10.9794/jspccs.37.29","DOIUrl":"https://doi.org/10.9794/jspccs.37.29","url":null,"abstract":"","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":"43512155","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}
T. Ono, M. Nakazawa, S. Morishima, H. Midorikawa, K. Ueno, Rie Kageyama
{"title":"A Case of Quadruple Valve Replacement after Five-time Sternotomies for Corrected Transposition of the Great Arteries","authors":"T. Ono, M. Nakazawa, S. Morishima, H. Midorikawa, K. Ueno, Rie Kageyama","doi":"10.9794/jspccs.37.64","DOIUrl":"https://doi.org/10.9794/jspccs.37.64","url":null,"abstract":"Patients with severe and complex congenital heart diseases frequently require multiple repeat sternotomies. We recently performed cardiac surgery through the fifth sternotomy in a 29-year-old female patient with corrected transposition of the great arteries and pulmonary atresia. Previously, she underwent six cardiac operations, including four sternotomies. She also underwent double-switch operation and mechanical valve replacements for mitral and tricuspid positions. Eventually, she manifested repeated ascites, syncope, palpitation, and fatigue. Echocardiography, computed tomography, and cardiac catheterization detected severe left ventricular outflow tract stenosis, severe pulmonary valve insufficiency, and moderate aortic valve insufficiency. Hence, aortic and pulmonary valve replacement and release of the left ventricular outflow tract stenosis were performed, and all four cardiac valves were replaced with mechanical valves. Consequently, her postoperative course was unevent-ful, with remarkable attenuation of symptoms.","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":"45000526","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}
{"title":"Endoscope-Assisted Mini-Incision Repair for a Child with Atrial Septal Defect","authors":"M. Ando, T. Fujii, T. Takano, S. Sakamoto","doi":"10.24509/JPCCS.20-013","DOIUrl":"https://doi.org/10.24509/JPCCS.20-013","url":null,"abstract":"","PeriodicalId":89701,"journal":{"name":"Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery","volume":"5 1","pages":"16-19"},"PeriodicalIF":0.0,"publicationDate":"2021-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45931350","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}
{"title":"Double Outlet Left Ventricle: A Rare and Less Understood Clinical Entity","authors":"A. Singhi, K. Sivakumar","doi":"10.24509/JPCCS.20-011","DOIUrl":"https://doi.org/10.24509/JPCCS.20-011","url":null,"abstract":"","PeriodicalId":89701,"journal":{"name":"Nihon Shoni Junkanki Gakkai zasshi = Pediatric cardiology and cardiac surgery","volume":"5 1","pages":"24-29"},"PeriodicalIF":0.0,"publicationDate":"2021-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43502431","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}