Double-inlet left ventricle with absent sixth aortic arch and multiple cardiac malformations

The first well-documented case of double-inlet left ventricle in association with absence of the derivatives of the sixth aortic arch of foetal life (the right and left branches of the pulmonary artery and the ductus arteriosus), is reported. In addition there were a total anomalous pulmonary venous connection to the portal vein, an ostium secundum type atrial septal defect and dextrocardia.