Anomalous Pulmonary Venous Drainage


Risk

  • One percent of all congenital heart defects.

  • TAPVD, the severe form, or PAPVD, the less severe form, exists when pulm veins drain into the venous circulation.

  • M:F 4:1 in infradiaphragmatic type.

Perioperative Risks

  • Rapid CV deterioration secondary to hypercapnia and resultant acidosis

  • Sudden pulm Htn and RHF during hypoventilation

  • Periop mortality: 2-20% depending on preop status

Worry About

  • Air bubbles entering the venous circuit

  • Endocarditis risk

  • Concurrent pneumonia with hypoxemia or hypercarbia

  • Polycythemic hyperviscosity attack with:

    • Periop dehydration

    • Cold OR environment

Overview

  • TAPVD incompatible with life unless an ASD allows adequate R-to-L shunting of blood. TAPVD pts with small ASDs are more critically ill and often require balloon septostomy as a bridge to surgery. Some cyanosis, usually with O 2 saturations of 85-95%.

  • Increased flow through pulm vascular beds results in pulm Htn.

  • Four types of TAPVD:

    • Supracardiac: Pulm veins connect to the left innominate vein via an anomalous “vertical vein” or connect to the right SVC via an anomalous “short connecting vein,” or connect to the left SVC (45%).

    • Cardiac: Pulm veins drain into the coronary sinus or directly into the right atrium (23%).

    • Infracardiac: Pulm veins drain into IVC, portal veins, hepatic veins, or ductus venosus (21%).

    • Mixed: Combined supracardiac, cardiac, and infracardiac connections (11%).

Etiology

  • Embryologic atresia or malformation of the common pulm venous system resulting in persistence of abnormal connections

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