Having an understanding of the long-term outcomes and quality of life after undergoing Tricuspid Atresia Surgery is important for both patients and healthcare providers. After undergoing surgery, there are lifestyle modifications to be adapted to prevent potential complications. This article explains in simple terms the outcomes and how the quality of life can be improved after Tricuspid Atresia Surgery.

Introduction

Tricuspid Atresia is a medical condition from birth that affects the heart. It is a heart defect in which the tricuspid valve of the heart does not form.¹ The tricuspid valve is normally present between two chambers on the right side of your heart, the right atrium (upper chamber) and the right ventricle (lower chamber). In Tricuspid Atresia, a solid tissue takes the place of the tricuspid valve. This tissue blocks blood flow from the right atrium to the right ventricle, where blood would normally go.

As a result, the right ventricle becomes small and underdeveloped. The pulmonary valve, located on the right side of the heart between the right ventricle and the lungs, can also be underdeveloped. 

People born with tricuspid atresia cannot get enough blood flowing through their heart and into their lungs, where it would get oxygen. Therefore, their lungs cannot provide enough oxygen to the rest of their body. 

In a child born with tricuspid atresia, blood flows from the upper right chamber (right atrium) to the upper left chamber (left atrium) of the heart through a hole in the septum- the wall between the chambers. This hole is always present during fetal life but sometimes the hole is big and becomes a heart defect (atrial septal defect). 

Some babies with tricuspid atresia have an additional hole between their heart’s two lower chambers (ventricular septal defect). Blood can flow through this hole and into the right ventricle, which will pump blood into the lungs.

When blood is flowing through these unnatural routes, blood high in oxygen blends with blood low in oxygen. In a normal heart, the two types of blood don’t mix. Most patients with tricuspid atresia require some form of surgical treatment during the first year of life. This is important to ensure oxygen flow is normal and to prevent further complications. 

This article covers the long-term outcomes, complications and quality of life considerations after Tricuspid Atresia Surgery. 

Understanding Tricuspid Atresia Surgery 

Most patients with tricuspid atresia require some form of surgical treatment during the first year of life.¹ The most common indication for surgical intervention is cyanosis with decreased pulmonary blood flow. In these babies, the right side of the heart is unable to pump blood. So, the goal of the operations is to bypass the heart’s right side by redirecting blue blood, returning from the body, directly to the lungs via the pulmonary arteries.

The surgery is often in stages and includes Blalock-Taussig shunt, Glenn shunt, and Fontan procedure.¹

Blalock-Taussig shunt

This operation is needed in newborns with tricuspid atresia the first one or two weeks of life. It is intended to provide a stable supply of blood to the lungs. It is needed when the oxygen levels are too low soon after birth. The surgery involves sewing a Gortex tube between the subclavian artery and the right pulmonary artery. Through the Gortex tube, a fixed amount of blood reaches the lungs with each heartbeat thereby improving oxygen levels. Babies outgrow the shunt with time and may need a new surgery to replace it.¹

Glenn Shunt 

In this operation, the superior vena cava is cut and both ends are sewn into the right pulmonary artery. The surgeon first removes the tube that was earlier implanted. One of the large veins that typically returns blood to the heart is connected directly to the lung artery instead of bypassing the heart.¹

The goal of this operation is to reduce the work of the left ventricle, which has been pumping blood to the body and the lungs. It also prevents oxygenated blood from returning to the lungs and thereby keeps the heart from doing unnecessary work.

A patch is placed over the top part of the heart’s right upper chamber. The patch prevents blood from the upper body from entering the heart and blood from the lower body from entering the lungs. It also maintains a connection that is used for the final stage of the repair and greatly simplifies the last operation.

However, after this operation, there is still blood passing through the inferior vena cava going back directly to the body without first passing the lungs. This makes the paleness persist. 

Between ages 2 and 3, children with tricuspid atresia will be ready for the third operation required to optimize their circulation¹.

Fontan Procedure 

This operation involves the connection of the inferior vena cava to the pulmonary artery. It creates a pathway so that most or all the blood that would have gone to the right heart can instead flow directly into the pulmonary artery. The patch placed over the heart’s right atrium (during the Glenn operation) is removed and a Gortex baffle is sewn in the right atrium. 

This creates a tunnel that guides blood returning from the lower body through the right atrium to the pulmonary artery. After this operation, most of the deoxygenated blood goes to the lungs so the child usually looks "pink". 

Long-Term Outcomes After Surgery 

There is a need for regular health checkups after treatment. 

After the surgery is carried out, many children can live healthily and go about their activities. They can live alongside and flow with other children, and participate in recreational activities. However, these children have lower endurance levels than other children in their age category. They require more rest during physical activities and they are restricted from vigorous exercises. Their parents need to help them find other areas of interest that are not so tasking and competitive. 

Children with Tricuspid Atresia have an increased risk of subacute bacterial endocarditis (SBE).

SBE is an infection of the heart that is caused by bacteria in the bloodstream. Children with heart defects are vulnerable to this infection because of the altered flow of blood through the heart and or abnormalities of the valves. It can occur after dental work or medical procedures on the gastrointestinal or respiratory tract because these procedures almost always result in some bacteria entering the blood. Subacute Bacterial Endocarditis can usually be prevented by taking an antibiotic before these procedures. 

Common Long-Term Complications

Each stage of treatment in tricuspid atresia comes with numerous long and short-term complications. The period between the first and second stages of surgery for single ventricles gives the highest rate of interstage death. Patients who have undergone Blalock-Taussig's Shunt face complications including the risk of shunt obstruction. Presently, the death rate is high among patients who have undergone a modified Blalock-Taussig shunt. 

A recent study examining outcomes in patients after a Blalock-Taussig's shunt revealed an in-hospital mortality rate of 12%, followed by an additional 6% interval mortality. Blalock-Taussig shunt-related thrombosis and stenosis occurred in 20% of patients, and non-cardiac complications, such as necrotizing enterocolitis and cerebral vascular accidents, were notable occurrences after BT shunts.²

In recent times, many single ventricle surgical treatment programs have incorporated home monitoring initiatives for shunted patients. This has been proven to be successful in enhancing outcomes for patients.¹ The Glenn surgery has shown excellent short and long-term outcomes, with an operative mortality rate of less than 1% and 5-year survival of 87%.³ Though complications are not common, the highest mortality is associated with thrombosis and thromboembolic events. Long-term complications associated with Fontan include arrhythmias, ventricular dysfunction, cyanosis, and protein-losing enteropathy (PLE). Atrial tachycardias are the predominant type of arrhythmias associated with the Fontan procedure. This is often attributed to suture lines in the right atrium that may interfere with atrial conduction. 

Quality of Life Considerations 

• Lifestyle modifications: Because of the volume overload present in these children and the use of diuretics, they need to eat diets low in salt. One has to pay attention to the replacement of appropriate electrolytes and the maintenance of nutrition to aid in proper growth and development
• Physical well-being: many patients have low endurance levels during exercise and this can lead to fatigue and loss of breath. It is advised to take on moderate physical activities that are not strenuous It is important for them to always be able to self-limit their activity, that is, to rest whenever they feel the need to do so. They should avoid participating in competitive activities⁴ 

Patients with tricuspid atresia need consistent and lifelong monitoring by a cardiologist specialized in congenital heart disease, such as a pediatric cardiologist or adult congenital heart disease (ACHD) specialist. The frequency of follow-up appointments differs across stages of single ventricle palliation, with those who have undergone the Fontan procedure requiring follow-up yearly.  A comprehensive history and physical examination during follow-up visits are essential for monitoring these patients effectively. Patients with surgically corrected Tricuspid Atresia can survive into childbearing age. There is no conclusive effect of pregnancy with Fontan circulation though the risk of the pregnancy is high due to increased susceptibility to arrhythmias, heart failure and thromboembolic complications. 

Summary 

Tricuspid Atresia Surgery helps improve survival rate, primarily the Fontan procedure but requires lifelong medical care. There are likely complications that can arise from the surgery, as described above. These complications can be avoided by proper medical care and checkups. The quality of life after surgery requires less strenuous and non-competitive activities. There is the utmost need for emotional support and care by caregivers.