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TAPVR exists when all pulmonary veins connect anomalously. The venous return may be obstructed or nonobstructed.

Also referred to as Total Anomalous Pulmonary Venous Connection (TAPVC).

 

Types of TAPVR:

Supracardiac (50%)

• Infrequently associated with obstruction.
• Portal veins connect to a left vertical vein or azygous vein

• Pulmonary veins connect to the right atrium or coronary sinus

• Pulmonary veins connect to a vein below the diaphragm

Mixed (5%)

Clinical:

• Symptoms depends on presence or absence of obstruction
• Obstructed: Pulmonary edema within several days after birth
• Nonobstructed: asymptomatic at birth. CHF develops during first month.

Hemodynamics:

Unobstructed Pulmonary vein

• Causes a complete L-R shunt at atrial level
• To sustain life, an obligatory R-L shunt must be present. Pulmonary flow is greatly increased leading to dilation of RA, RV, PA.

• Pulmonary venous htn and pulmonary arterial htn
• Pulmonary edema
• Diminshed pulmonary return to heart resulting in low cardiac output

Radiographic Features

Nonobstructed TAPVC

• Snowman Heart in supracardiac types; the supracardiac shadow results from dilated right SVC, vertical vein , and innominate vein
• Snowman configuration not seen with other types
• Increased pulmonary vascularity

Obstructd TAPVC

• Pulmonary edema
• small heart

Infracardiac TAPVR