
Background: Ano-rectal malformations is an abnormality of the ano-rectum at birth in which the infant born with abnormal rectal opening. Different types of ano-rectal malformations are present which occurs in 1/ 5000 live birth. Usually it’s not lethal even with complex variants and their management, the association of serious renal or cardiac anomalies increasing the morbidity and mortality of ano-rectal malformation cases. Study were carried out to determine the congenital cardiac anomalies associated to patients born with ano-rectal malformation and its rate, type , impact on the outcome of surgical reconstruction and the utility of the preoperative echocardiography. Methods: This prospective study was conducted between July 2007 and January 2016, including 67 cases born with ano-rectal malformations which were managed and the associated congenital cardiac anomalies were evaluated by preoperative Echocardiography. The cases with positive echocardiography findings were tabulated with the sex, congenital cardiac anomalies types, mortality and type of ano-rectal malformations, other associated anomalies, and the type of surgery performed. Results: Sixty-seven patients born with ano-rectal malformation were referred to the pediatric surgery unit. Age of patients ranged from 18 -38 hours with a mean age 28 hours. According to the echocardiography, 17(25.3%) of patients had congenital cardiac anomalies. Atrial septal defect (7 cases) predominated followed by Ventricular septal defect (5 cases). In 12 patients (17.9%), the associated congenital cardiac anomalies were of a mild nature, and reconstruction of the ano-rectum went smoothly. Five patients (7.4%) had significant congenital cardiac anomalies and all of them were died. Conclusion: Associated congenital cardiac anomalies are present in many patients with ano-rectal malformation. Prognosis of the ano-rectal malformations patients, mostly depend on severity of the associated anomalies than on ano-rectal malformations itself. Thorough systemic clinical and radiological examination must be done to detect any associated anomalies at an early presentation and should be managed as soon as possible to reduce the future morbidity and mortality. While most of the congenital cardiac anomalies associated with ano-rectal malformations was of the mild type and had no effect on their surgical reconstruction, some of these associated anomalies were serious and affected the prognosis. Preoperative echocardiography evaluation of all cases of ano-rectal malformations is important and mandatory to reduce the morbidity and mortality.