First report of Criss Cross Heart, diagnosed intrauterine, in Western Amazonia

Criss Cross Heart (CCH) is a rare cardiac anomaly characterized by the rotation of the ventricular mass in its major axis, which can be associated with other malformations, resulting in different relations between atrium, ventricles and great vessels. To report a case of CCH still diagnosed intrauterine in a pregnant woman in the city of Rio Branco-Acre and the complexity of diagnosis and treatment. This is an observational and descriptive case report. A fetal echocardiogram was performed at twenty-eight weeks of gestation, and a complex cardiac malformation was visualized in the fetus. The patient was referred to a specialized diagnostic center, where the compatible diagnostic confirmation of CCH was obtained. At thirty-eight weeks the delivery was performed. A newborn female with an apgar score of 9/10 maintained peripheral saturation above 90%, being discharged at twelve days of age. The child has atrioventricular agreement and a disagreement between arterial ventricle, lower left ventricle and upper right ventricle. It presents situs solitus, levocardia, double right ventricular inlet, patent foramen ovale, parallel base vessels, patent ductus arteriosus, interventricular communication, mild aortic insufficiency and pulmonary stenosis. It evolves clinically stable with surgical programming around one year of life. This case report is the first case of CCH in the Western Amazon, bringing new perspectives of early diagnosis. Prenatal diagnosis made it possible to refer the patient to a referral hospital for cardiac surgery and to schedule future surgical correction after confirmation of left ventricular hypoplasia through probable catheterization.