Partial and total anomalous pulmonary venous connections and associated defects

Anomalous pulmonary venous return is a relatively rare congenital cardiac malformation in which the pulmonary veins do not return to the left atrium. If all four pulmonary veins drain anomalously, the designation of total anomalous pulmonary venous return (TAPVR) is applied; if only one to three pulmonary veins drain anomalously, the designation of partial anomalous pulmonary venous return (PAPVR) is applied. TAPVR is classified into supracardiac, infracardiac, cardiac, and mixed types according to the location of the anomalous drainage. The prognosis of patients with TAPVR depends on the degree of pulmonary venous obstruction and associated defects. TAPVR generally requires neonatal surgical correction. Early and late mortality for simple TAPVR is 10 % and 4 %, respectively. Patients with TAPVR and pulmonary venous obstruction have a higher early and late mortality (17 % and 11 %, respectively) than unobstructed patients (4 % and 6 %, respectively). TAPVR associated with other cardiac anomalies (other than an ASD or PDA) have worse early and late mortality (14 % and 18 %, respectively). There are two types of PAPVR that are identified by name, sinus venosus PAPVR and scimitar syndrome. The prognosis for surgically corrected non-scimitar PAPVR is excellent. Scimitar syndrome PAPVR has a less favorable prognosis due to other associated cardiac and pulmonary lesions. PAPVR requires surgical correction whenever diagnosis is made or whenever the patient becomes symptomatic, not necessarily during the neonatal period. Patients with non-scimitar PAPVR do well with a 0 % early mortality and less than 1 % late mortality. Patients with scimitar syndrome have a higher risk of mortality (6 %) compared to other forms of PAPVR. Patients with scimitar syndrome who require a pneumonectomy or lobectomy have a higher mortality (25 %) than patients who have their anomalous drainage repaired without the need for lung resection (4 %). The prognosis for all anomalous venous return patients has improved since the first surgical corrections were described. The management of pulmonary vein obstruction continues to be the largest obstacle to successful management of anomalous pulmonary venous return.

Duke Scholars

Author Alexandre Tellechea Rotta Pediatrics, Critical Care Medicine