BACKGROUND: In developing countries, infants with Down
syndrome and cardiac defect are at increased risk of dying.
Congenital heart diseases occur in 40-50% of affected infants.
Endocardial cushion defect accounts for the most. Pattern of
cardiac defects in Down syndrome vary with ethnicity. The
current study aims to determine pattern of cardiac defects
and survival of patients in our institution.
METHODS: Hospital based review of cases, between April
2010 and may 2015 were made. Data were analyzed using
SPSS version 20 software quantitatively with plotted Kaplan
Meier survival curve done. RESULTS: Down syndrome cases,
53 male and 63 females with cardiac anomalies, were
described. Patent ductus arteriosus occurs in 57(36.5%),
Ventricular septal defect in 31(19.9%), Atrial septal defect in
30(19%),
Atrio-ventricular
septal
defect
in
29(18.6%),Tetralogy of Fallot in 4(2.6%) and others in
5(3.2%) cases. Cases were alive, lost to follow-up and died in
59, 35 and 22 cases, respectively. The overall death rate was
19% and two-third of them died during infancy with females
dying at an earlier age than males. Pulmonary hypertension,
hypothyroidism and gastrointestinal disorders
were
diagnosed in 46, 21 and 4 of the cases, respectively. Reasons
for lost to follow-up were discussed.
CONCLUSION: The pattern of cardiac anomalies in our
study among Down syndrome cases is dominated by Patent
ductus arteriosus while, the overall survival of cases is
guarded as majority of deaths occurred during infancy
KEYWORDS: Down syndrome, cardiovascular anomaly,
congenital heart disease, trisomy 21.

Published: 2019-05-28