Introduction: Congenital heart disease CHD is the most common congenital anomaly in newborns. In this study it was aimed to investigate the demographic features of the patients with CHD diagnosed in neonatal intensive care unit NICU to determine the frequency of the anomalies and to elucidate the etiologic risk factors.Materials and Methods: The files of 361 newborns who were diagnosed with CHD and hospitalized at Ondokuz Mayıs University NICU were retrospectively examined from 2006 to 2010. Fourteen patients with normal echocardiography, 3patients with supraventricular tachycardia, and 44 patients with patent ductus arteriosus and/or patent foramen ovale diagnosed in the first three days were excluded from the study. Results: The frequency of CHD in the NICU was faund to be 7.5%. Out of all the cases 141 of them 47% were female, and 159 of them 53% were male. Cyanotic CHD was diagnosed in 32.3%, and non-cyanotic CHD was diagnosed in 67.7% of the cases. The most frequent CHD in newborns was ventricular septal defect 15.3% , and the most frequent cyanotic CHD was transposition of the great arteries 10% . The 46 cases 15.3% were out of consanguineous marriage and 13 cases 4.3% had CHD in their family history. Out of 300 patients with CHD, 220 of them 73.3% were discharged from the hospital, 67 of them 22.3% died in NICU and 13 of them 4.3% were sent to another hospital. Conclusions: CHD rate is approximately 5-8 per 1.000 live births; however, the frequency in the neonatal period increases 7-11 times. Therefore, cardiological and echocardiographic evaluation in neonates has great importance. In addition, early diagnosis of CHD in the neonatal period will lead to earlier life-saving interventions; therefore, significant reduction in mortality and morbidity
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