ABSTRACT
Anomalous pulmonary venous connection is a rare pathology, both partial and total forms, according to the number of pulmonary veins draining into the left atrium, respectively. Total forms are classified into four groups, depending upon the anomalous connections to the systemic veins: supra, intracardiac or below, and finally a mixed form, the less frequent. Prenatal, even postnatal diagnose is difficult, especially in partial isolated forms. The association with chromosomal abnormalities is low, however is highly associated with complex heart malformations, especially total forms, often in the context of heterotaxy syndromes. We present a case of infracardiac APVC, in the context of complex heart disease, associated with complete atrioventricular canal and conotruncal anomaly, that came to surgery at 48 hours of life with poor outcome. We described the ultrasound and magnetic resonance angiography of this anomaly and its perinatal prognosis and management.