Tricuspid Atresia is a rare type of congenital heart disease in which the tricuspid valve is missing,
abnormally developed or blocked by a solid sheet of tissue. The defect blocks blood
flow from the right atrium of the heart to the right ventricle of the heart. As a
result, the right ventricle tends to be very small and underdeveloped (hypoplastic).
When this type of defect occurs, the right side of the heart cannot properly pump
blood to the lungs as it normally would. As a result, the lungs cannot supply the
rest of the body with the oxygen that it needs. This type of blockage causes the blood
to flow from the upper right chamber of the heart to the upper left chamber of the
heart through a hole in the wall between them. This hole is either a heart defect
(atrial septal defect) or an enlarged natural opening (foramen ovale) which is supposed
to close soon after birth. If the infant does not have a hole through which the blood
can flow, surgery may be needed to create and opening.
Some infants with tricuspid atresia also have a hole between the right ventricle and
the left ventricle (ventricular septal defect), a condition, which will need to be
medically monitored and may require surgical intervention. Infants diagnosed with
tricuspid atresia are often cyanotic (bluish discoloration of the skin), tire easily
especially during feeding, have difficulty breathing, and exhibit slow growth. These
infants may also develop symptoms of heart failure including fatigue, swelling in
the legs, ankles, feet and abdomen, sudden weight gain from fluid retention, and irregular
or rapid heartbeat.
Adults, who had corrective heart surgeries as infants, may develop problems with their
heart functioning later in life. Over time, the surgical treatments that were used
at infancy to repair the heart defect may leave scar tissue behind, increasing the
chances of abnormal heart rhythm (arrhythmia), and develop a focus for subacute endocarditis.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Echocardiogram, ECG (electrocardiogram), imaging studies, and cardiac catheterization
ONSET AND PROGRESSION
A diagnosis of tricuspid atresia is usually made shortly after birth. Infants who
survive to adulthood may have medical complications requiring multiple follow-up surgeries.
Complications such as the formation of blood clots in the arteries of the lungs, strokes,
complaints of fatigue, and heart rhythm abnormalities will require life style changes
monitoring for infection and life-long follow-up with a cardiologist. If surgical
interventions fail, then a heart transplant may be necessary.