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Total anomalous pulmonary venous return
TAPVR; Total veins; Congenital heart defect - TAPVR; Cyanotic heart disease - TAPVR
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 ).
Congenital heart disease
Congenital heart disease (CHD) is a problem with the heart's structure and function that is present at birth.
Causes
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.
Atrial septal defect
Atrial septal defect (ASD) is a heart defect that is present at birth (congenital). As a baby develops in the womb, a wall (septum) forms that divide...
Patent foramen ovale
Patent foramen ovale (PFO) is a hole between the left and right atria (upper chambers) of the heart. This hole exists in everyone before birth, but ...
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.
Symptoms
The infant may appear very sick and may have the following symptoms:
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Bluish color of the skin (cyanosis)
Bluish color of the skin (cyanosis)
Cyanosis is a bluish color to the skin or mucous membrane that is usually due to a lack of oxygen in the blood.
ImageRead Article Now Book Mark Article - Frequent respiratory infections
- Lethargy
- Poor feeding
- Poor growth
- Rapid breathing
Note: Sometimes, no symptoms may be present in infancy or early childhood.
Exams and Tests
Tests may include:
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Cardiac catheterization
can confirm the diagnosis by showing that the blood vessels are abnormally attached
Cardiac catheterization
Cardiac catheterization involves passing a thin flexible tube (catheter) into the right or left side of the heart. The catheter is most often insert...
ImageRead Article Now Book Mark Article -
ECG
shows enlargement of the ventricles (ventricular hypertrophy)
ECG
An electrocardiogram (ECG) is a test that records the electrical activity of the heart.
ImageRead Article Now Book Mark Article -
Echocardiogram
may show that the pulmonary vessels are attached
Echocardiogram
An echocardiogram is a test that uses sound waves to create pictures of the heart. The picture and information it produces is more detailed than a s...
ImageRead Article Now Book Mark Article - MRI of the heart can show the connections between the pulmonary vessels
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X-ray of the chest
shows a normal to small heart with fluid in the lungs
X-ray of the chest
A chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.
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Treatment
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.
Outlook (Prognosis)
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.
Possible Complications
Complications may include:
- Breathing difficulties
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Heart failure
Heart failure
Heart failure is a condition in which the heart is no longer able to pump oxygen-rich blood to the rest of the body efficiently. This causes symptom...
ImageRead Article Now Book Mark Article - Irregular, fast heart rhythms (arrhythmias)
- Lung infections
- Pulmonary hypertension
When to Contact a Medical Professional
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.
Prevention
There is no known way to prevent TAPVR.
References
Fraser CD, Carberry KE. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery . 19th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 59.
Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Mann DL, Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine . 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 62.
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Heart, section through the middle - illustration
The interior of the heart is composed of valves, chambers, and associated vessels.
Heart, section through the middle
illustration
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Totally anomalous pulmonary venous return, X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease.
Totally anomalous pulmonary venous return, X-ray
illustration
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Totally anomalous pulmonary venous return, X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease. This x-ray shows an enlarged heart, a large liver, and increased size of the lung blood vessels (pulmonary vascularity).
Totally anomalous pulmonary venous return, X-ray
illustration
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Totally anomalous pulmonary venous return, X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease. This x-ray shows an enlarged heart, a large liver, and increased pulmonary vascularity.
Totally anomalous pulmonary venous return, X-ray
illustration
-
Heart, section through the middle - illustration
The interior of the heart is composed of valves, chambers, and associated vessels.
Heart, section through the middle
illustration
-
Totally anomalous pulmonary venous return, X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease.
Totally anomalous pulmonary venous return, X-ray
illustration
-
Totally anomalous pulmonary venous return, X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease. This x-ray shows an enlarged heart, a large liver, and increased size of the lung blood vessels (pulmonary vascularity).
Totally anomalous pulmonary venous return, X-ray
illustration
-
Totally anomalous pulmonary venous return, X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease. This x-ray shows an enlarged heart, a large liver, and increased pulmonary vascularity.
Totally anomalous pulmonary venous return, X-ray
illustration
Review Date: 10/22/2015
Reviewed By: Larry A. Weinrauch, MD, Assistant Professor of Medicine, Harvard Medical School, Cardiovascular Disease and Clinical Outcomes Research, Watertown, MA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
