Skip to Main Content

++

Key Concepts

++

  • Epidemiology

    • Total anomalous pulmonary venous connection (TAPVC) accounts for 1 to 3 percent of all cases of congenital heart defects. Obstructed TAPVC is one of the few true emergencies in neonatal surgery.

    • TAPVC is either an isolated lesion or associated with complex atrial isomerism.

  • Morphology

    • All the pulmonary veins drain into one or several systemic veins instead of the left atrium (LA).

    • The presence of an inter-atrial communication is crucial to sustain life and an atrial septal defect (ASD) or a patent foramen ovale (PFO) is considered as a part of TAPVC complex.

    • Four anatomic subtypes of TAPVC are recognized based on the location of the anomalous pulmonary venous drainage: supracardiac (40–50 percent), cardiac (20–25 percent), infracardiac (10–15 percent), and miscellaneous or mixed (10 percent).

  • Pathophysiology

    • TAPVC is a mixing lesion in which pulmonary venous blood completely mixes with systemic venous blood and returns to the right atrium through a systemic vein, with consequent left-to-right shunt.

    • In neonates and small infants with obstructed pulmonary venous return, pulmonary hypertension and pulmonary edema are always present.

  • Clinical features

    • Infants with obstructed TAPVC present with cyanosis, respiratory distress soon after birth. Infants with unobstructed TAPVC present later than infants with obstruction.

  • Diagnosis

    • Echocardiography is diagnostic. Angiography is not indicated. Magnetic resonance imaging (MRI) may be useful as a supplement to echocardiography.

  • Treatment

    • Surgical repair involves creation of a connection between the pulmonary venous confluence and the LA. Stabilization with preoperative extracorporeal membrane oxygenation (ECMO) is an option for desperately ill neonates.

  • Outcomes

    • The long-term outcome for patients with repaired TAPVC is very good, with an operative mortality of 10 percent. Pulmonary venous stenosis is a relatively uncommon complication but, when it occurs, the impact on prognosis is profound.

++

Introduction

++

Total anomalous pulmonary venous connection (TAPVC) is a rare and heterogeneous anomaly that accounts for only 2 percent of congenital heart defects.1 TAPVC is a condition in which all the pulmonary veins drain into one or several systemic veins instead of the left atrium (LA). Presence or absence of pulmonary venous obstruction is important for the immediate postnatal stability of newborns. Obstructed TAPVC is one of the few true emergencies in congenital heart surgery.

++

The first attempt at surgical repair was performed by Muller in 1951 by performing an anastomosis between the pulmonary vein and the LA.2 In 1956 Lewis successfully corrected TAPVC by using hypothermia and inflow occlusion.3 The first successful repair with cardiopulmonary bypass was performed by Burroughs and Kirklin in 1956.4

++

Anatomic Classification

++

Four anatomic subtypes of TAPVC are recognized based on the location of the anomalous pulmonary venous drainage: supracardiac (40–50 percent), cardiac (20–25 percent), infracardiac (10–15 percent), and miscellaneous or mixed (10 percent). In supracardiac TAPVC, the four pulmonary veins join at a venous confluence (remnant of the common pulmonary vein) behind the LA and typically drain superiorly and ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.