Tetralogy of Fallot in Four Down syndrome Patients in Setif, Algeria: Diagnosis and Clinical Aspects

Abstract

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. And represents 75% in the general population. However, its association with down syndrome is infrequent. To describe the presentation in patients with Down Syndrome (DS) suffering from Tetralogy of Fallot (TF) we reported four cases. All patients with DS who have CHD are collected from the medico-pedagogical centers and the paediatric department of the university hospital of the wilaya of Setif. Data were collected and followed between February 2009 and December 2013, the characteristics analyzed were age, sex, mother age, 1st degree cousins, and other associated cardiac or extra-cardiac anomalies, diagnosis of CHD was done in any case by the echocardio doppler. Results: 110 patients with trisomy 21 (T21) with CHD were analyzed; the most common CHD was the atrioventricular septal defect with a frequency of 44.5 followed by interventricular communication. The TF was present with 3.6%. The four patients are divided into two girls and two boys; the irregular shape was the most common 3 times out of 4. The radical cure was only made in one patient at the age of two years. We regret a death for a digestive pathology. The etiology of this disease remains of genetic origin. Conclusion: The TF is not frequent in T21. The irregular shape is more frequent but the number does not allow us to conclude, extra-cardiac anomalies including digestive make the prognosis of these patients heavier. Cardiac surgery has evolved considerably in recent years, in Algeria cardiac surgery remains a problem for this category of patients because it is expensive and patients are not insured by social security and the majority of these patients are refused by surgeons because of the trisomy.