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 difficultyin 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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