Type IV total anomalous pulmonary venous connection. Unusual survival and review of the literature.

Total anomalous pulmonary venous connection (TAPVC) is a condition most frequently seen in the pediatric age group. Of those that survive uncorrected into adulthood, type I and type II constitute the great majority. It is most unusual to see a patient with a type IV lesion survive. We report a 34 year old female patient with TAPVC of the right upper lobe and left lung veins into the vertical vein and the right lower lobe vein into the coronary sinus. This patient has the second longest survival of those with type IV lesion of TAPVC, and the second oldest to have successful surgical correction. We attribute her long survival to the normal pulmonary artery pressure, a wide atrial septal defect and no other associated cardiac lesions. a review of the surgical survival in the English literature of patients with type IV TAPVC is presented.