Emine Gulsah Torun, Nevin Özdemiroğlu, Velihan Çayhan, İbrahim Ece
{"title":"以大动脉导管未闭和巨大肝动静脉畸形为表现的唐氏综合征婴儿肺动脉高压的分步治疗。","authors":"Emine Gulsah Torun, Nevin Özdemiroğlu, Velihan Çayhan, İbrahim Ece","doi":"10.1017/S1047951125101662","DOIUrl":null,"url":null,"abstract":"<p><p>We report a rare case of a 3-month-old male infant with Down syndrome, severe pulmonary arterial hypertension (a large patent ductus arteriosus, an atrial septal defect, and a giant hepatic arteriovenous malformation). Initial treatment involved hepatic arteriovenous malformation embolisation using 17 Azur® CX Peripheral Coils, resulting in clinical improvement. However, persistent pulmonary arterial hypertension led to patent ductus arteriosus closure using an 8-mm Lifetech Cera™ vascular plug after a successful balloon occlusion test. Post-intervention, the patient showed symptomatic relief, reduced right ventricular overload, and atrial septal defect shunting shifted to left to right. This is the first reported paediatric case combining these features successfully managed through a multidisciplinary and stepwise treatment approach, eliminating the need for liver transplantation.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":" ","pages":"1730-1733"},"PeriodicalIF":0.7000,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Life-saving stepwise management of pulmonary hypertension in an infant with Down syndrome presenting with a large patent ductus arteriosus and giant hepatic arteriovenous malformation.\",\"authors\":\"Emine Gulsah Torun, Nevin Özdemiroğlu, Velihan Çayhan, İbrahim Ece\",\"doi\":\"10.1017/S1047951125101662\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>We report a rare case of a 3-month-old male infant with Down syndrome, severe pulmonary arterial hypertension (a large patent ductus arteriosus, an atrial septal defect, and a giant hepatic arteriovenous malformation). Initial treatment involved hepatic arteriovenous malformation embolisation using 17 Azur® CX Peripheral Coils, resulting in clinical improvement. However, persistent pulmonary arterial hypertension led to patent ductus arteriosus closure using an 8-mm Lifetech Cera™ vascular plug after a successful balloon occlusion test. Post-intervention, the patient showed symptomatic relief, reduced right ventricular overload, and atrial septal defect shunting shifted to left to right. This is the first reported paediatric case combining these features successfully managed through a multidisciplinary and stepwise treatment approach, eliminating the need for liver transplantation.</p>\",\"PeriodicalId\":9435,\"journal\":{\"name\":\"Cardiology in the Young\",\"volume\":\" \",\"pages\":\"1730-1733\"},\"PeriodicalIF\":0.7000,\"publicationDate\":\"2025-08-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Cardiology in the Young\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1017/S1047951125101662\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/8/18 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"CARDIAC & CARDIOVASCULAR SYSTEMS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Cardiology in the Young","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1017/S1047951125101662","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/8/18 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
Life-saving stepwise management of pulmonary hypertension in an infant with Down syndrome presenting with a large patent ductus arteriosus and giant hepatic arteriovenous malformation.
We report a rare case of a 3-month-old male infant with Down syndrome, severe pulmonary arterial hypertension (a large patent ductus arteriosus, an atrial septal defect, and a giant hepatic arteriovenous malformation). Initial treatment involved hepatic arteriovenous malformation embolisation using 17 Azur® CX Peripheral Coils, resulting in clinical improvement. However, persistent pulmonary arterial hypertension led to patent ductus arteriosus closure using an 8-mm Lifetech Cera™ vascular plug after a successful balloon occlusion test. Post-intervention, the patient showed symptomatic relief, reduced right ventricular overload, and atrial septal defect shunting shifted to left to right. This is the first reported paediatric case combining these features successfully managed through a multidisciplinary and stepwise treatment approach, eliminating the need for liver transplantation.
期刊介绍:
Cardiology in the Young is devoted to cardiovascular issues affecting the young, and the older patient suffering the sequels of congenital heart disease, or other cardiac diseases acquired in childhood. The journal serves the interests of all professionals concerned with these topics. By design, the journal is international and multidisciplinary in its approach, and members of the editorial board take an active role in the its mission, helping to make it the essential journal in paediatric cardiology. All aspects of paediatric cardiology are covered within the journal. The content includes original articles, brief reports, editorials, reviews, and papers devoted to continuing professional development.