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).
TAPVR; Total veins; Congenital heart defect - TAPVR; Cyanotic heart disease - TAPVR
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.
The infant may appear very sick and may have the following symptoms:
Note: Sometimes, no symptoms may be present in infancy or early childhood.
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.
Sachin Talwar is in New Delhi, India. Talwar is rated as an Elite expert by MediFind in the treatment of Total Anomalous Pulmonary Venous Return. He is also highly rated in 41 other conditions, according to our data. His top areas of expertise are Transposition of the Great Arteries, Ventricular Septal Defects, Total Anomalous Pulmonary Venous Return, and Tetralogy of Fallot.
Matthew Yong is in Townsville, Australia. Yong is rated as an Elite expert by MediFind in the treatment of Total Anomalous Pulmonary Venous Return. He is also highly rated in 7 other conditions, according to our data. His top areas of expertise are Total Anomalous Pulmonary Venous Return, Congenital Tracheomalacia, Pulmonary Veno-Occlusive Disease, and Heart Tumor.
Vladimiro Vida is in Padova, Italy. Vida is rated as an Elite expert by MediFind in the treatment of Total Anomalous Pulmonary Venous Return. They are also highly rated in 16 other conditions, according to our data. Their top areas of expertise are Total Anomalous Pulmonary Venous Return, Congenital Heart Disease CHD, Tetralogy of Fallot, and Congenital Coronary Artery Malformation.
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.
Complications may include:
This condition may be apparent at the time of birth. However, symptoms may not be present until later.
Call your health care provider if you notice symptoms of TAPVR. Prompt attention is required.
There is no known way to prevent TAPVR.
Published Date: October 10, 2021
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 Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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: The Biological Basis of Modern Surgical Practice. 21st ed. Philadelphia, PA: Elsevier; 2022:chap 59.