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Tricuspid atresia is a type of congenital heart disease in which the tricuspid heart valve is missing or abnormally developed. The defect blocks blood flow from the right atrium to the right ventricle.

Tri atresia

Tricuspid atresia is an uncommon form of congenital heart disease that affects about 5 in every 100,000 live births. Twenty percent of patients with this condition will also have have other heart problems.

Normally, blood flows into the right atrium, then through the tricuspid valve to the right ventricle and on to the lungs. If the tricuspid valve is missing or broken, the blood cannot flow from the right atrium to the left ventricle. Blood ultimately cannot enter the lungs, where it must go to pick up oxygen (become oxygenated).

Infants with tricuspid atresia generally are cyanotic (bluish discoloration of skin) and easily become short of breath.

• Cyanosis (bluish discoloration of the skin)
• Easily fatigued
• Shortness of breath (dyspnea)
• Clubbing of fingers or toes
• Heart failure
• Fast breathing
• Poor growth

This condition may be discovered during routine prenatal ultrasound imaging or when the baby is examined shortly after birth. Cyanosis may be present from birth. A heart murmur is generally present at birth and may increase in loudness over several months.

Tests may include the following:

• ECG
• Echocardiogram
• Chest x-ray
• Cardiac catheterization
• MRI of the heart

Improvement can be expected with most surgical procedures. Unexpected death may occur with heart arrhythmias.

• Irregular, fast heart rhythms (arrhythmias)
• Chronic diarrhea (from a disease called protein loosing enteropathy)
• Heart failure
• Fluid in the abdomen (ascites) and in the lungs (pleural effusion)
• Blockage of the artificial shunt
• Strokes and other neurological complications
• Sudden death

If your infant has new changes in breathing patterns, eats less (decreased feeing), or has skin or mucous membranes that are turning blue (cyanotic), contact your health care provider immediately.

There is no known way to prevent tricuspid atresia.

Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. St. Louis, Mo; WB Saunders; 2007.