Marfan Syndrome Complicated by Aortic Arch Aneurysm and Aortic Dissection: A Case Report from Congo
Marfan Syndrome Complicated by Aortic Arch Aneurysm and Aortic Dissection: A Case Report from Congo作者机构：Department of Cardiology University Hospital of Brazzaville Brazzaville Republic of the Congo Department of Doctoral Studies Faculty of Health Sciences Marien Ngouabi University Brazzaville Republic of the Congo
出 版 物：《World Journal of Cardiovascular Diseases》 (心血管病（英文）)
年 卷 期：2022年第12卷第10期
页 面：463-471页
学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学]
主 题：Marfan Syndrome Heart Failure Aortic Aneurysm Aortic Dissection Congo
摘 要：Context and Aim: Marfan syndrome is a transmissible genetic disease of the connective tissue that is rarely encountered in Congo and in sub-Saharan African countries. Its cardiovascular complications can be life frightening. The management of that disease is still limited in our country because of a lack of technical capacity in cardiovascular surgery. The aim of this clinical report is to show the interest of echocardiography and especially angioscanner as the main technique in the diagnosis of the severity of this disease, elaborate a literature review, but also to highlight the difficulties encountered in the management of that affection in our countries. Observation: The authors report the medical observation of a 48-year-old adult with a history of cataract of the left eye and a subluxation of the lens for which he underwent surgery in 2016, without any etiology being found. He is a smoker at a rate of 6 packs yearly. He consulted for progressively worsening dyspnea and constrictive mediosternal pain. The clinical examination revealed a moderate alteration of the general state, apyrexia, a blood pressure of 140/90 mmHg, a SPO2 of 97% in ambient air, a respiratory frequency of 32 cycles/min, signs of left ventricular insufficiency, a diastolic murmur of aortic insufficiency of intensity 4/6th, a long-limbed morphotype with a wingspan superior to the height and a kyphoscoliosis. Chest X-ray showed cardiomegaly with a cardiothoracic ratio of 58%, a highly dilated and uncoiled aorta, convexity of the left inferior arch, and venocapillary hypertension and a quiet alveolar-interstitial pulmonary oedema. The ECG was in sinus rhythm and showed a poor R-wave progression in anteroseptal leads. Echocardiography showed significant aortic root dilatation up to 72.6 mm and aortic regurgitation grade IV. Angioscanner showed a dissected aortic aneurysm and areas of emphysema located in the lungs. The medical treatment was palliative with beta blocker and angiotensin
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