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Total Anomalous Pulmonary Venous Return (TAPVR)

(Total Anomalous Pulmonary Venous Connection; TAPVC)

By Jeanne Marie Baffa, MD, Associate Professor of Pediatrics;Director of Echocardiography, Thomas Jefferson University School of Medicine;Nemours/Afred I. duPont Hospital for Children

In total anomalous pulmonary venous return, the pulmonary veins, blood vessels that normally carry oxygenated blood from the lungs to the left side of the heart, connect instead to the right side of the heart. This blood then flows to the left heart through a hole in the wall separating the left and right heart chambers.

  • Children with a mild form of total anomalous pulmonary venous return may have few symptoms.

  • Children with more severe total anomalous pulmonary venous return may have bluish discoloration of the skin (cyanosis), shortness of breath and fatigue.

  • Echocardiography is needed for diagnosis

  • Surgical repair is needed

Total anomalous pulmonary venous return (TAPVR) accounts for 1 to 2% of birth defects of the heart.

Normally, the pulmonary veins return blood that has picked up oxygen in the lungs to the left atrium. This oxygenated blood then flows from the left atrium into the left ventricle, where it is pumped to the body. (see also Normal Fetal Circulation) In infants born with total anomalous pulmonary venous return, the pulmonary veins do not connect normally to the left atrium and connect instead to the right atrium. So the right atrium, which usually receives only deoxygenated blood from the body to be pumped to the lungs, receives a mix of oxygenated and deoxygenated blood. The additional blood causes the heart to have to work harder than normal. Even more importantly, flow from the pulmonary veins makes its way to the right atrium through various pathways, going up above the heart, down below the heart, or around the back wall of the heart. These pathways can be narrow or blocked, causing blood to back up in the lungs and pressure to build up in the lungs, preventing their normal function. Blood only flows to the left side of the heart through a hole between the right and left atria (a patent foramen ovale or atrial septal defect).

Total anomalous pulmonary venous return.

The pulmonary veins do not connect to the left atrium; instead, the entire pulmonary venous return enters systemic venous circulation through various connections (in this case the connecting vein is supracardiac or above the heart). Connecting veins can also be below the heart (infracardiac) or behind the heart. Systemic blood flow depends on right-to-left atrial shunting.

AO = aorta; IVC = inferior vena cava; LA =left atrium; LV = left ventricle; PA = pulmonary artery; PV =pulmonary veins; RA = right atrium; RV = right ventricle; SVC = superior vena cava.

Symptoms

Newborns with a severe form of TAPVR have difficulty breathing and bluish coloration of the skin (cyanosis).

In milder forms, symptoms of heart failure (see Figure: Heart Failure: Pumping and Filling Problems) may be present but be more difficult to detect. Symptoms of heart failure include shortness of breath and fatigue. Some infants may have no symptoms.

Diagnosis

  • Echocardiography

Diagnosis is suspected based on the findings on a chest x-ray. Diagnosis is confirmed by echocardiography. Occasionally, magnetic resonance imaging (MRI) of the heart or computed tomography (CT) angiography may be needed so doctors can more clearly view the defect.

Treatment

  • Surgical repair

  • Medical treatment of heart failure before surgery

Newborns with TAPVR require surgery early in life. When there is severe obstruction in the pulmonary venous pathway, emergency surgical repair is often needed. Heart failure should be treated with drugs to improve breathing until surgery can be done.

Surgical repair consists of creating a wide open connection between the pulmonary veins and the left atrium.

Some children need to take antibiotics before visits to the dentist and before certain surgeries (such as on the bowel or bladder). These antibiotics are used to prevent serious heart infections called endocarditis.

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