Discussion:
EVC syndrome is an autosomal recessive syndrome, and 30% of patients die during the first year of life due to cardiac or respiratory problems (1). A retrospective review of the cases submitted to the Pediatric Cardiac Care Consortium between 1982 and 2007 was performed, and included thirty-two pediatric patients with CHD and EVC syndrome (4), and of whom twenty-eight (88%) had an AVSD, with 15 having common atrium, and two had DOMV (4). The medical literature reported that there are three morphological types of DOMV: central type, duplicate mitral valve type, eccentric type. The most common type is the eccentric one which is characterized by a large main opening and a smaller accessory opening (6), which was the type we found during our surgery, and was associated with PAVSD and common atrium. Regarding the ideal time for surgical repair of CHD in patients with EVC syndrome, it has been reported that delayed surgical repair was associated with reduction in postoperative morbidity and improved survival (7). In our case, the patient was referred to our hospital at the age of seven years, and the decision of surgical repair was taken by the heart team. She remains completely asymptomatic 6 months after surgery. The management of EVC required the coordinated efforts of a multidisciplinary team of medical professionals. We made a plan with our team of specialists to systematically and comprehensively treat the patient. Moreover, genetic counseling for the patient and her family is recommended.