Total Anomalous Pulmonary Venous Connection (tapvc)

Introduction

• TAPVC to the left innominate vein (L Inn. V) by way of a vertical vein (VV).
• TAPVC to coronary sinus (CS). The pulmonary veins join to form a confluence designated common pulmonary vein (CPV), which connects to the coronary sinus.
• TAPVC to the portal vein (PV). The pulmonary veins form a confluence, from which an anomalous channel arises. This connects to the portal vein, which communicates with the inferior vena cava (IVC) by way of ductus venosus (DV) or the hepatic sinusoids.

  SV- Splenic vein; SMV- superior mesenteric vein; RP and LP- right and left portal veins; RH and LH - right and left hepatic veins; SVC- superior vena cava ; RA and LA- right and left atrium; RV and LV- right and left ventricle.

TAPVC Types

• Supracardiac (50%)
• Cardiac (20%)
• Infracardiac (subdiaphragmatic) (20%) - obstructive
• Mixed type (10%)

TAPVC Clinical Manifestations

• Non-obstructive: similar to ASD
• Obstructive: signs of pulmonary hypertension

Investigations

ECG: RVH

Chest X-ray: "snowman heart"(50%)