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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.
Keywords: Cyanotic congenital heart disease; Echocardiography; Transposition of great arteries