Abstract Background: Cyanotic congenital heart disease (CCHD) accounts for 25% of congenital
heart defects and has a high morbidity and mortality in neonates.
Objective: The aim of this work was to study clinical profile and available therapeutic modalities
used in the management of CCHD.
Subjects and methods: This was a prospective study carried out for a period of one year, starting
from January 2011, and included 50 neonates admitted to neonatal intensive care unit (NICU) of
Sohag University Hospital, Sohag, Upper Egypt. All included patients were subjected to thorough
clinical history, full clinical examination, initial and frequent measurement of oxygen saturation by
pulse oximetry, blood gas analysis, and echocardiography. Also, therapeutic modalities used in
management of CCHD were noted.
Results: 50 neonates were diagnosed as having CCHD, out of them 39 (78%) were males with
male to female ratio 3.55:1. The mean age of presentation was 11.78± 9.4 days. CCHD frequency
was found to be 9.5% (50/524) in our NICU population. The most common type of CCHD was
d-transposition of great arteries (D-TGA) (66%) followed by complex CCHD (12%) and hypoplastic
left heart syndrome (HLHS) (12%), whereas the less common type was hypoplastic right ventricle
(2%). All cases presented with central cyanosis and needed medical treatment whereas balloon
atrial septostomy was performed in 26% of cases. Seventy-four percent of cases were improved and
referred to higher centers while 26% were expired during hospital stay.
Conclusion: CCHD is a leading cause of neonatal morbidity and mortality. CCHD frequency
was significant (9.5%) in our study population with D-TGA being the commonest type. Majority
of neonates with CCHD showed survival with suitable management. Early diagnosis and referral
to pediatric cardiac center for proper management will improve the outcome. Neonatologists and
pediatric cardiologists should be familiar with diagnosis and management of CCHD.
 2016 Ain Shams University. Production and hosting by Elsevier B.V. This is an open access article