Spectrum of congenital heart diseases in children with Down Syndrome at Usmanu Danfodiyo University Teaching Hospital, Sokoto
Keywords:
Down syndrome, congenital heart diseases, pattern, SokotoAbstract
Objective: Congenital heart diseases (CHD) frequently occur in children with Down syndrome. A few studies in Nigeria have highlighted the pattern of CHD in such patients, but results are variable with no data from the study area for comparison. This study aims to determine the pattern of CHD among children with Down syndrome as seen at UDUTH, Sokoto
Methods: A prospective study conducted between 1st October 2011 and 31stApril 2015. Subjects included all children with phenotypic features of Down syndrome (DS) who were seen at the Pediatric cardiology clinic and had echocardiography diagnosis of congenital heart diseases.
Results: Of the 41 cases of DS seen during the study period, 32 (78.0%) children aged 2 weeks to 22 months (Mean +SD = 5.6 ± 4.0 months) were diagnosed with CHD. Male to female ratio was 2.2:1. Atrioventricular septal defect (AVSD) was the commonest CHD seen in 18 (56.3%) patients. Nine (28.1%) patients had isolated ventricular septal defect (VSD), three (9.4%) had VSD coexisting with atrial septal defect (ASD) while one (3.1%) patient each had isolated ASD and Fallot's tetralogy respectively. Only three (9.4%) patients had surgical closure abroad, with good postoperative outcome in two of the patients.
Conclusion: AVSD is the commonest CHD in our series, which is similar to previous reports. Increased access to definitive intervention is advocated since surgical outcome in such patients may be favorable.
References
WHO. Genes and chromosomal diseases:
Down syndrome (Accessed June
0 1 6 ) . A v a i l a b l e a t :
www.who.int/genomics/public/geneticdise ases/en/index1.html
Wiseman F, Alford K, Tybulewicz V, Fisher E. Down syndrome-recent progress and future prospects. Human Mol Genet 2009;18 (1): 75-
Wells GL, Barker SE, Finley SC, Colvin EV, Finley WH. Congenital heart disease in infants with Down's syndrome. South Med J 1994; 87(7):724-7.
Freeman SB, Taft LF, Dooley KJ, Allran K, Sherman SL, Hassold TJ, et al. Populationbased study of congenital heart defects in Down syndrome. Am J Med Genet 1998; 80 (3): 2137.
Asani M, Aliyu I, Also U. Pattern of congenital heart diseases among children with Down syndrome seen in Aminu Kano Teaching
Hospital, Kano, Nigeria. Niger J Basic Clin Sci 2013; 10 (2): 57.
Fatema NN. Down's Syndrome with
Congenital Heart Disease: Analysis of Cases Over Two Years in a Non-Invasive Laboratory of a Tertiary Hospital. Cardiovasc J 2010; 2 (2): 184.
Ekure EN, Animashaun A, Bastos M, Ezeaka VC. Congenital heart diseases associated with identified syndromes and other extra-cardiac congenital malformations in children in Lagos. West Afr J Med 2009; 28 (1): 33-7.
Abbag FI. Congenital heart diseases and other major anomalies in patients with Down syndrome. Saudi Med J 2006; 27 (2): 219-22.
Al-Jarallah AS. Down syndrome and the pattern of congenital heart disease in a community with high parental consanguinity. Med Sci Monit 2009; 15 (8): 409-12.
Otaigbe B, Tabansi P, Agbedeyi G. Pattern of congenital heart defects in children with Down syndrome at the University of Port Harcourt Teaching Hospital, Port Harcourt. Niger J Paed 2012; 39 (4): 164-7.
Bull C, Rigby M, Shiniebourne E. Should management of complete atrioventricular canal defect be influenced by coexistant Down syndrome? The Lancet 1985: 1147-9.
Wilson NJ, Gavalaki E, Newman CGH.
Complete atrioventricular septal defect in the presence of Down syndrome. The Lancet 1985: 834.
Reller M, Morris C. Is Down syndrome a risk factor for poor outcome after repair of congenital heart defects? . J Pediatr 1998; 132 (4): 738-41.
Fudge JC, Jr., Li S, Jaggers J, O'Brien SM, Peterson ED, Jacobs JP, et al. Congenital heart surgery outcomes in Down syndrome: analysis of a national clinical database. Pediatrics 2010; 126 (2): 315-22.
Lange R, Guenther T, Busch R, Hess J, Schreiber C. The presence of Down syndrome is not a risk factor in complete atrioventricular septal defect repair. J Thorac Cardiovasc Surg 2007; 134 (2): 304-10.
Tubman TRJ, Shields MD, Craig BG,
Mulholland HC, Nevin NC. Congenital heart disease in Down's syndrome: two year prospective early screening study. BMJ 1991; 302 (1425-7).
Mocumbi A. The challenges of cardiac surgery for African children. Cardiovasc J Afr 2012; 23 (165-7).
Bode-Thomas F. Challenges in the management of congenital heart diseases in developing countries, congenital heart diseases selected aspects. Syamasundar P (ed), ISBN: 978-953-307-472-6, In Tech, Available from: http://www.intechopen.com/books/congenitalheart--disease-selected-aspects/challenges-inthe-management-of-congenital-heart-diseasein-developing-countries. 2012.
Torfs CP, Christianson RE. Anomalies in Down syndrome individuals in a large populationbased registry. Am J Med Genet 1998; 77 (5): 431-8.
Stoll C, Alembik Y, Dott B, Roth MP. Study of Down syndrome in 238,942 consecutive births. Ann Genet 1998; 41 (1): 44-51.
Spicer R. Cardiovascular disease in down syndrome Pediatr Clin North Am 1984; 31: 1331-43.
Vilas Boas L, Albernaz EP, Costa RG. Prevalence of congenital heart defects in patients with Down syndrome in the municipality of Pelotas, Brazil. Jornal de Pediatria 2009; 85 (5): 403-7.
Khan I, Muhammad T. Frequency and pattern of congenital heart defects in children with Down syndrome. Gomal Journal of Medical Sciences 2012; 10 (2): 241-3.
Ashraf M, Malla M, Javed C, Mohd IM,
Mymoona A, Ayaz R, et al. Consanguinity and pattern of congenital heart defects in Down syndrome in Kashmir, India. AJSIR 2010; 1 (3): 573-7.
Mihçi E, Akçurin G, Eren E, Kardelen F, Akçurin S, Keser I, et al. Evaluation of congenital heart diseases and thyroid abnormalities in children with Down syndrome. Anatolian J Cardiol 2010; 10 (5): 440-5.
Sani U, Jiya N, Ahmed H, Waziri U. Profile and outcome of congenital heart diseases in children: A preliminary experience from a tertiary center in Sokoto, Northwestern Nigeria. Nig Postgrad Med J 2015; 22 (1): 1-8.
Sani U, Ahmed H, Jiya N. Pattern of acquired heart diseases among children seen in Sokoto, North western Nigeria. Niger J Clin Pract 2015;
(6): 718-25
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