Congenital Cyanogenic Heart Disease in Children: About 420 Cases in Africa

A. Fall, A. Sow, I. Basse, A. M. Coundoul, Fondjo P. S. Monoue, A. Thiongane, D. Boiro, I. Ly, B. Niang, I. Ba, G. Diagne, P. Faye, O. Ndiaye
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Abstract

Congenital cyanogenic heart disease (CCHD) is a malformation of the heart and large vessels characterized by an oxygen desaturation in the arterial blood, responsible for cyanosis. The general objective was to study the profile of CCHD in Senegalese hospitals. This is a retrospective study carried out over a period of 8 years (January 1, 2010 - December 31, 2017) and including all children aged 0 to 16 years followed for a CCHD. The hospital prevalence was 0.87% for 420 cases collected. The sex ratio was 1.44 and the average age at diagnosis was 16 months. First degree parental consanguinity was noted in 36 cases (30.78%). The main reasons for consultation were breathing difficulty in 242 cases (57.62%) and fever in 136 patients (32.36%). Apart from cyanosis, the clinical signs were dominated by the heart murmur in 313 cases (74.7%), tachycardia in 283 cases (67.38%) and digital hippocratism in 162 cases (38.57%). Cardiomegaly was found in 239 patients (83.36%). The main types of CCHD were tetralogy of Fallot and transposition of the large vessels. In biology, 206 patients (49.05%) presented polyglobulia. A complete surgical cure was carried out in 22 patients (5.24%). Complications were anoxic crisis (52 cases) and hemorrhagic syndrome (17 cases). There were 97 deaths (28.28%) during hospitalization. The diagnosis of CCHD is late in our country and surgical management is poor explaining the high mortality.
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儿童先天性青紫性心脏病:非洲约420例
先天性青紫性心脏病(CCHD)是一种心脏和大血管的畸形,其特征是动脉血中的氧饱和度过低,导致青紫。总的目标是研究塞内加尔医院中慢性冠心病的概况。这是一项为期8年(2010年1月1日至2017年12月31日)的回顾性研究,包括所有0至16岁的CCHD儿童。420例患者住院患病率为0.87%。性别比为1.44,诊断时平均年龄为16个月。一级亲缘关系36例(30.78%)。就诊原因主要为呼吸困难242例(57.62%),发热136例(32.36%)。除发绀外,临床体征以心脏杂音为主313例(74.7%),心动过速283例(67.38%),数字化希波底162例(38.57%)。239例(83.36%)患者出现心脏肿大。冠心病的主要类型为法洛四联症和大血管转位。生物学方面,206例(49.05%)出现多球性贫血。手术完全治愈22例(5.24%)。并发症为缺氧危象52例,出血性综合征17例。住院期间死亡97例(28.28%)。CCHD在我国诊断较晚,手术治疗较差,故死亡率高。
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