Tricuspid Atresia

By | June 10, 2022

Tricuspid atresia is a congenital malformation of the heart. As part of the disease, the tricuspid valve is absent. Instead, there is only a membrane that is not permeable. As a result of the malformation, the organism is not supplied with enough oxygen. For this reason it is a so-called cyanotic defect of the heart.

What is tricuspid atresia?

In the context of tricuspid atresia, there is severe underdevelopment of the right ventricle of the heart. In addition, the affected patients suffer from a defect in the ventricular septum. As a result, the blood from the two vena cava flows into the atrium on the left side. The right ventricle is often not completely separated from the left and is also larger than usual. For hyperparathyroidism definition and meaning, please visit

For example, cyanosis occurs in newborn babies due to tricuspid atresia. As a result of the malformation, there is a mixture between the blood from the arteries and veins. In addition, a so-called pulmonary stenosis is possible, so that the amount of blood flowing into the lungs is limited.

If people with tricuspid atresia do not suffer from pulmonary stenosis, pulmonary hypertension develops in some cases. The reason for this is that the enlarged chamber of the heart on the left hand side supplies the pulmonary artery and the aorta with the same pressure. Basically, in tricuspid atresia, there is no connection between the right atrium and the corresponding ventricle.


Tricuspid atresia is a congenital defect of the heart. Thus, there are usually genetic dispositions that lead to the development of the disease. Tricuspid atresia accounts for about one to two percent of all cases of heart defects that are present from birth. In connection with tricuspid atresia, a so-called transposition of the main arteries is found in about one fifth of the affected patients.

Symptoms, Ailments & Signs

In tricuspid atresia, the connection between the atrium and the ventricle of the heart is missing on the right side. For this reason, the path of the blood from the right atrium leads along the foramen ovale into the atrium on the left side. Some patients also have a defect in the atrial septum. All the blood from the pulmonary veins and the circulatory system comes together in the ventricle on the left side of the heart.

Cyanosis occurs in infants as a result of tricuspid atresia, the severity of which depends on the blood flow to the lungs. If there is a defect in the atrial septum at the same time, only mild cyanosis usually develops.

Diagnosis & course of disease

The typical symptoms such as cyanosis already indicate the presence of tricuspid atresia. If the disease is suspected, the doctor will take an anamnesis and then carry out various examination procedures in order to arrive at a reliable diagnosis of tricuspid atresia.

Suitable methods are, for example, echocardiography and an examination using a cardiac catheter. A so-called balloon atrioseptostomy may be used if a special foramen ovale is present.

The first audible heart sound is usually prominent on auscultation, suggesting tricuspid atresia. A so-called systolic can also be heard, which results from the other anomalies in the anatomy of the heart. When conducting an ECG study, the results indicate left ventricular hypertrophy.


Since tricuspid atresia is a malformation of the heart, in the worst case it can lead to the death of the affected person. However, this usually only occurs if the disease is not treated. This heart problem also has a very negative effect on the patient’s circulation and can also lead to problems.

Most sufferers experience chest pain and, in many cases, breathing difficulties. The resilience of the affected person decreases significantly, so that the patient can no longer easily carry out strenuous activities or sporting complaints. The patient is also permanently tired and exhausted, which usually cannot be compensated for by sleep.

The treatment of tricuspid atresia depends on the exact symptoms and their severity. Patients usually have to undergo surgery and take medication. Complications rarely occur.

With early treatment, there is also a positive course of the disease. In most cases, this does not result in a reduced life expectancy. However, those affected are still dependent on regular check-ups and examinations by a doctor.

When should you go to the doctor?

In the case of tricuspid atresia, a doctor must be consulted in any case. This disease cannot heal itself, although the symptoms usually worsen significantly. The disease must be recognized and treated immediately after birth. The life expectancy of those affected is therefore significantly reduced. A doctor should be contacted for tricuspid atresia if the child is not getting enough oxygen.

The skin and lips turn blue, and breathing difficulties or a cough can also occur. However, the disease is usually recognized immediately after birth, so that an additional visit to the doctor for diagnosis is not necessary. Even after successful treatment, regular examinations of the heart and other internal organs are very useful in order to detect or even prevent further damage at an early stage. Since tricuspid atresia can lead to psychological upsets or depression, especially in the parents or relatives, psychological treatment should also be carried out.

Treatment & Therapy

Various potential methods are available for the treatment of tricuspid atresia. They are used in coordination with the individual case of illness, so that a specific treatment takes place. If the blood supply to the lungs is poor, the affected patients usually receive the active ingredient prostaglandin E1. In this way, the ductus Botalli is kept open.

In addition, a Blalock-Taussig shunt will be installed. A surgical intervention is also possible, which is based on Fontan. A connection is created between the arteries of the lungs and the two vena cavae. In this way, the existing cyanosis is eliminated, because now the pulmonary and systemic circulation are no longer connected to each other.

Another positive effect of the procedure is the relief of the left ventricle, which is now confronted with less blood volume. Potential complications from surgery include pleural or pericardial effusion and cardiac arrhythmias. Observations indicate that ten years after the operation, around 90 percent of the treated patients are still alive.

Under certain circumstances, the therapy of tricuspid atresia is also started with the so-called Rashkind maneuver. This ensures that the blood drains from the corresponding atrium. The success with regard to the elimination of cyanosis in children is relatively good with the appropriate surgical intervention.

In everyday life, too, the people have relatively good physical resilience. In the long term, however, cardiac arrhythmias are possible. It is therefore necessary for patients to have medical check-ups and take prophylactic measures throughout their lives.


Tricuspid atresia is a congenital malformation of the heart that causes problems even in newborn children. Prevention is therefore not possible.


Tricuspid atresia is usually treated surgically. In addition, lung damage must be prevented in the event of oxygen oversaturation. Follow-up care is always necessary in the event of a malformation of the ventricles. With early treatment in childhood, the prognosis for an unrestricted life is very favorable, life expectancy is hardly reduced.

If the malformation is not treated, it can be fatal. Even after a successful operation, the patient is dependent on regular medical checks. Prescribed medication must be taken consistently. After an operation, the patient is treated in intensive care as part of the aftercare. The postoperative measures end with discharge from the clinic. Further follow-up care is provided by a cardiologist, who accompanies the patient over the long term. The goal is an improved quality of life without permanent defects.

In later life, those affected form a risk group for cardiac arrhythmias. For this reason, aftercare is also prophylaxis: the specialist uses the ECG to determine the pulse rate. In the event of abnormalities, he prescribes regulating medication for the patient. Lifelong use of blood-thinning medicine affects patients with artificial heart valves. Cardiology records the tolerability. At his own discretion, he changes the dosage or prescribes additional preparations. Follow-up care continues into adulthood.

You can do that yourself

Tricuspid atresia is treated surgically and with medication using preparations such as prostaglandin E1. In support of medical treatment, it is important to pay attention to typical complications such as ascites and cardiac arrhythmia and to inform the doctor.

After discharge from the hospital, the child is very weak and should sleep a lot. In addition, regular visits to the doctor are indicated. Medical advice is required for the symptoms mentioned, as there may be a risk of death. The child’s parents should organize an outpatient care service that specializes in paediatrics. A lot of communication between the parents and the child is also important, because the illness represents a burden for all parties that must be worked through.

As long as no other symptoms occur, the sick child can lead a normal and symptom-free life. The child should be informed about the disease at an early stage. Routine medical examinations must be continued in adolescence and adulthood because tricuspid atresia can continue to cause complications. Unusual symptoms should also be clarified quickly, since a heart defect can lead to fainting within a short time and may even be fatal.

Tricuspid Atresia