What are the symptoms of TAPVR?

In partial anomalous venous return (PAPVR), if only one vein is affected, there are usually no symptoms. If two veins from the same side are affected, symptoms of shortness of breath with exertion and low stamina may occur during childhood. The symptoms are usually mild and may not ever occur.

What are the four types of TAPVR?

Generally, there are four types of TAPVR:

• Supracardiac TAPVR. The pulmonary veins drain into the right atrium through the superior vena cava.
• Infracardiac TAPVR. The pulmonary veins drain into the right atrium through the liver (hepatic) veins and the inferior vena cava.
• Cardiac TAPVR. There are two types.
• Mixed TAPVR.

Is total anomalous pulmonary venous return hereditary?

In many cases, we don’t know what causes TAPVR. It occurs because of abnormal development of the heart’s pulmonary veins during early fetal growth. Some congenital heart defects may have a genetic link, causing heart problems to occur more often in certain families.

Is TAPVR ductal dependent?

A similar situation can occur in cases of TAPVR or truncus arteriosus, which are ductal-independent mixing lesions. In obstructive type TAPVR with severe cyanosis, the pulmonary venous congestion or edema is striking and the heart size is usually normal.

What happens when there is an increase in venous return?

For example, if systemic venous return is suddenly increased (e.g., changing from upright to supine position), right ventricular preload increases leading to an increase in stroke volume and pulmonary blood flow.

How is Papvr detected?

PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocar-diography (TEE) and catheter based angiography [5, 6]. However, echocardiography can provide insufficient information, mainly due to its limited acoustic window.

How many babies are born with TAPVR?

In a 2019 study using data from birth defects tracking systems across the United States, researchers estimated that each year about 504 babies in the United States are born with Total Anomalous Pulmonary Venous Return.

How rare is TAPVR?

Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart malformation that accounts for 1% to 3% of all congenital heart anomalies [1].

How common is partial anomalous pulmonary venous return?

This is a very rare finding and mostly involves the anomalous drainage of the right upper pulmonary vein into the superior vena cava. Only 3% of patients had PAPVC from the left lung to the innominate vein.

Is TGA duct dependent?

TGA with intact ventricular septum (TGA/IVS) serve as ductus dependent lesion, but large ASD is more important to mixing of the circulation2,5).

Is Ebstein Anomaly duct dependent?

Newborns with severe forms of Ebstein’s anomaly have marked cyanosis and cardiomegaly, while the majority are dependent on a PDA patency for pulmonary blood flow [9]. Our patient was PDA dependent, and needed PGE1 to maintain ductus arteriosus patency.

What are the factors that affect venous return?

Venous return is facilitated by a number of factors, including inspiration, increased total blood volume, increased venomotor tone, the cardiac suction effect, the presence of venous valves and the skeletal muscle pump.

What does total anomalous pulmonary venous return ( TAPVR ) mean?

In a child born with total anomalous — which means abnormal — pulmonary venous return (TAPVR), the pulmonary veins connect to other veins and ultimately drain their blood into the right atrium.

When do symptoms of TAPVR start to get worse?

Symptoms can become worse and can start very early in life, even shortly after work. The type of repair recommended for TAPVR depends on the specific problems with the anatomy of the blood vessels. Typically, the surgeon will form a connection between the pulmonary veins coming from the lungs and the left atrium.

Where to go for TAPVR with pulmonary obstruction?

This is called TAPVR with pulmonary obstruction. These children are typically transported to the Cardiac Center at CHOP from their birth hospitals, and are admitted to the Tabas Cardiac Intensive Care Unit to be stabilized.

Are there any risks with TAPVR for infants?

Many infants do well with TAPVR surgery. But complications can develop. Some infants do not survive the surgery. Risk factors may vary based on the child’s overall health and the anatomy of the heart and vessel problems present. Ask your child’s healthcare provider about the specific risks for your child.