{"title":"NON- DISSECTING LARGE AORTIC ARCH ANEURYSM LEADING TO CHRONIC AORTIC INSUFFICIENCY PRESENTING AS ACUTE HEART FAILURE REVEALING MARFAN SYNDROME","authors":"Mariam EL Harrak","doi":"10.47191/rajar/v9i8.02","DOIUrl":null,"url":null,"abstract":"Introduction: Marfan syndrome is an autosomal dominant disorder of connective tissue which has many clinical symptoms and whose prognosis depends on associated cardiovascular complications, dominated by progressive aortic root dilation, which may lead to aortic dissection, rupture or aortic regurgitation. Prevention of these life threatening complications is of major importance. Case presentation: We report here a case of a 42-year-old man, with a history of two retinal detachments in 2015 and 2018, who presented with severe aortic valve regurgitation and severe heart failure secondary to significant aortic root dilatation revealing Marfan syndrome. Unfortunately, before referring the patient to the surgeon, the patient ended up dying from an unrecovered cardiac arrest. Conclusion: It is very important to recognize Marfan syndrome on time and the preventive actions should be undertaken in order to avoid life-threatening consequences of this disorder. Echocardiography and especially the angioscanner represent the main tools used for the diagnosis of gravity and the follow-up of this condition.","PeriodicalId":20848,"journal":{"name":"RA JOURNAL OF APPLIED RESEARCH","volume":"71 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2023-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"RA JOURNAL OF APPLIED RESEARCH","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.47191/rajar/v9i8.02","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
Introduction: Marfan syndrome is an autosomal dominant disorder of connective tissue which has many clinical symptoms and whose prognosis depends on associated cardiovascular complications, dominated by progressive aortic root dilation, which may lead to aortic dissection, rupture or aortic regurgitation. Prevention of these life threatening complications is of major importance. Case presentation: We report here a case of a 42-year-old man, with a history of two retinal detachments in 2015 and 2018, who presented with severe aortic valve regurgitation and severe heart failure secondary to significant aortic root dilatation revealing Marfan syndrome. Unfortunately, before referring the patient to the surgeon, the patient ended up dying from an unrecovered cardiac arrest. Conclusion: It is very important to recognize Marfan syndrome on time and the preventive actions should be undertaken in order to avoid life-threatening consequences of this disorder. Echocardiography and especially the angioscanner represent the main tools used for the diagnosis of gravity and the follow-up of this condition.