Learn About Total Anomalous Pulmonary Venous Return

What is the definition of Total Anomalous Pulmonary Venous Return?

Total anomalous pulmonary venous return (TAPVR) is a heart disease in which the 4 veins that take blood from the lungs to the heart do not attach normally to the left atrium (left upper chamber of the heart). Instead, they attach to another blood vessel or the wrong part of the heart. It is present at birth (congenital heart disease).

What are the alternative names for Total Anomalous Pulmonary Venous Return?

TAPVR; Total veins; Congenital heart defect - TAPVR; Cyanotic heart disease - TAPVR

What are the causes of Total Anomalous Pulmonary Venous Return?

The cause of total anomalous pulmonary venous return is unknown.

In normal circulation, blood is sent from the right ventricle to pick up oxygen in the lungs. It then returns through the pulmonary (lung) veins to the left side of the heart, which sends blood out through the aorta and around the body.

In TAPVR, oxygen-rich blood returns from the lungs to the right atrium or to a vein flowing into the right atrium, instead of the left side of heart. In other words, blood simply circles to and from the lungs and never gets out to the body.

For the infant to live, an atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and the rest of the body.

How severe this condition is depends on whether the pulmonary veins are blocked or obstructed as they drain. Obstructed TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery.

What are the symptoms of Total Anomalous Pulmonary Venous Return?

The infant may appear very sick and may have the following symptoms:

  • Bluish color to the skin (cyanosis) due to low oxygen level in the blood
  • Frequent respiratory infections
  • Lethargy
  • Poor feeding
  • Poor growth
  • Rapid breathing

Note: Sometimes, no symptoms may be present in infancy or early childhood.

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What are the current treatments for Total Anomalous Pulmonary Venous Return?

Surgery to repair the problem is needed as soon as possible. In surgery, the pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed.

Who are the top Total Anomalous Pulmonary Venous Return Local Doctors?
Thoracic Surgery
Thoracic Surgery

University Of Chicago

5841 S Maryland Ave, 
Chicago, IL 
 128.0 mi
Accepting New Patients

Husam Balkhy is a Thoracic Surgeon in Chicago, Illinois. Dr. Balkhy and is rated as an Experienced provider by MediFind in the treatment of Total Anomalous Pulmonary Venous Return. His top areas of expertise are Heart Tumor, Mitral Valve Regurgitation, Constrictive Pericarditis, Heart Bypass Surgery, and Coronary Artery Bypass Graft (CABG). Dr. Balkhy is currently accepting new patients.

Interventional Cardiology | Cardiology
Interventional Cardiology | Cardiology

University Of Chicago

47 W Polk St, 
Chicago, IL 
 130.3 mi
Accepting New Patients

Atman Shah is an Interventional Cardiologist in Chicago, Illinois. Dr. Shah and is rated as an Experienced provider by MediFind in the treatment of Total Anomalous Pulmonary Venous Return. His top areas of expertise are Heart Attack, Ventricular Fibrillation, Cardiac Arrest, Transcatheter Aortic Valve Replacement (TAVR), and Aortic Valve Replacement. Dr. Shah is currently accepting new patients.

 
 
 
 
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Dupage Medical Group Ltd

17495 La Grange Rd, 
Tinley Park, IL 
 109.0 mi
Accepting New Patients
Offers Telehealth

Abdulrahman Masrani is a Radiologist in Tinley Park, Illinois. Dr. Masrani and is rated as an Experienced provider by MediFind in the treatment of Total Anomalous Pulmonary Venous Return. His top areas of expertise are Total Anomalous Pulmonary Venous Return, Lung Nodules, Gallbladder Disease, Pleural Effusion, and Liver Embolization. Dr. Masrani is currently accepting new patients.

What is the outlook (prognosis) for Total Anomalous Pulmonary Venous Return?

If this condition is not treated, the heart will get larger, leading to heart failure. Repairing the defect early provides excellent results if there is no blockage of the pulmonary veins at the new connection into the heart. Infants who have obstructed veins have worsened survival.

What are the possible complications of Total Anomalous Pulmonary Venous Return?

Complications may include:

  • Breathing difficulties
  • Heart failure
  • Irregular, fast heart rhythms (arrhythmias)
  • Lung infections
  • Pulmonary hypertension
When should I contact a medical professional for Total Anomalous Pulmonary Venous Return?

This condition may be apparent at the time of birth. However, symptoms may not be present until later.

Contact your health care provider if you notice symptoms of TAPVR. Prompt attention is required.

How do I prevent Total Anomalous Pulmonary Venous Return?

Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease.

Women who are pregnant should get good prenatal care:

  • Avoid alcohol and illegal drugs during pregnancy.
  • Tell your provider that you are pregnant before taking any new medicines.
  • Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery.
  • Pregnant women who have diabetes should try to get good control over their blood sugar level.

Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.

What are the latest Total Anomalous Pulmonary Venous Return Clinical Trials?
OPTImal Treatment of Sinus VENOSUS Defect - Efficacy and Safety of Transcatheter Correction Compared to Surgical Treatment in Patients With Sinus Venosus Defect

Summary: Sinus venosus defect (SVD) accounts for 10% of atrial septal defects and is characterized by an anomalous pulmonary venous return in the superior vena cava associated with a high situated atrial septal defect. Since 2013, transcatheter correction of this congenital heart disease has emerged as a new treatment option. The procedure involves placement of a covered stent in the superior vena cava tha...

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Who are the sources who wrote this article ?

Published Date: October 23, 2023
Published By: Michael A. Chen, MD, PhD, Associate Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, WA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Valente AM, Dorfman AL, Babu-Narayan SV, Kreiger EV. Congenital heart disease in the adolescent and adult. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 82.

Well A, Fraser CD. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 21st ed. St Louis, MO: Elsevier; 2022:chap 59.