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Background: Although less common than acyanotic congenital heart diseases(CHD), cyanotic group is more significant cause of the associated mortality and morbidity due to its severe nature and poor medical or surgical outcome if the diagnosis is delayed. It is thus important to have detailed information on the clinical profile of various cyanotic CHDs as well as their mode of presentation and outcome for the early detection and timely intervention.
Methods: The study was carried out over a period of two and a half years in 0-18 years age group at three different paediatric cardiac centres at Kashmir valley. All the children referred with complaints or clinical examination suggestive of congenital heart defects were further evaluated with echocardiography. On echocardiography children having cyanotic congenital heart defects were included in this study. The clinical profile and mode of presentation and midterm outcome of various cyanotic CHDs was further studied in detail.
Results: Out of total 2445 children screened on echocardiography, 1315 children had normal scans, while 1130 had CHDs. Among these, 842 (74.5%) had acyanotic CHDs while 288(25.5%) had cyanotic CHDs. Among the cyanotic CHDs, Tetralogy of Fallot (TOF) was the most common lesion (44.8 % of cyanotic CHDs). Other common cyanotic lesions included single ventricle 11.5%, DORV 11.1%, TGA 8.6% and TAPVC 8 %. Cyanosis along with cyanotic spells was the most common mode of presentation in 77.4 % patients. Many patients were lost to follow-up (19.8%) and among others 23% underwent surgical repair, 43% were medically managed and 14.2% expired.
Conclusion: Detailed study and knowledge about the clinical profile, mode of presentation and outcome of various cyanotic CHDs paves a way for the further research and referral for timely intervention in this group of CHDs with relatively dismal prognosis in absence of surgery.