Exploring Congenital Heart Defects:

Tricuspid Atresia

By Shanna

Maretsky&

Jennifer Richards

What is a congenital Heart Defect?

Congenital- means inborn or existing at birth.

The term Congenital heart “disease” or “defect” is commonly used to describe the condition.

A congenital heart defect occurs when the heart or blood vessels near the heart don't develop normally before birth.

Tricuspid Atresia

In this condition, there’s no tricuspid valve so blood can’t flow from the right atrium to the right ventricle. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic or an absence of the right ventricle.

This defect is contracted during prenatal development, when the heart does not finish developing. It causes the heart to be unable to properly oxygenate the rest of the blood in the body. Because of this, the body does not have enough oxygen to live, and steps must be taken to keep the child alive.

Because of the lack of an A-V connection, an atrial septal defect (ASD) must be present to maintain blood flow. Also, since there is a lack of a right ventricle there must be a way to pump blood into the pulmonary arteries, and this is accomplished by a ventricular septal defect (VSD). Blood is mixed in the left atrium.

Symptoms Since the condition is visible prenatally- its almost always diagnosed before birth. Not all symptoms are present at birth, but most commonly develop within the first few days after.

Slow Growth

ShortnessOf

Breath

Digital Clubbin

g

Heart Failure

Tachypnea

Easily fatigued

Cyanosis(Bluish skin coloration)

Statistics

About 35,000 infants (or 1 out of 125) are born with Congenital Heart Defects.

Out of all those, less than 3.7% cases are diagnosed with Tricuspid Atresia.

Without adequate blood flow to the lungs, babies are cyanotic (blue) and Surgery is ALWAYS necessary.

With technological and surgical advances, there have been dramatic increases in survival for CHD patients.

Tricuspid Atresia on Ultrasound

Surgical Procedures

Typically within the first few days or weeks of life, a surgeon will put in a shunt to form a connection between the descending aorta and the pulmonary artery. This initial procedure helps to increase blood flow to the lungs, and should reduce cyanosis with increased oxygenation in the area. There will need to be additional procedures as the heart grows larger.

Bi-directional Glenn Shunt

The next repair that would be necessary is called the “bidirectional Glenn Procedure”This involves redirecting oxygen-poor blood from the top of the body to the lungs.

The pulmonary arteries are disconnected from their existing blood supply (the shunt).

The superior vena cava (SVC), which carries blood returning from the upper body, is disconnected from the heart and instead redirected into the pulmonary arteries. The inferior vena cava (IVC), which carries blood returning from the lower body, continues to connect to the heart.

At this point, patients are no longer in that delicate balance, and the single ventricle is doing much less work. They usually can grow adequately, and are less fragile.

The Fontan Procedure

However, they still have marked hypoxia (because of the IVC blood that is not fed into the lungs to be oxygenated), and the ventricle is still doing more work than expected. Therefore most patients are referred for another surgery.

The second stage, also called Fontan completion, involves redirecting the blood from the IVC to the lungs as well. At this point, the oxygen-poor blood from upper and lower body flows through the lungs without being pumped (driven only by the pressure that builds up in the veins). This corrects the hypoxia, and leaves the single ventricle responsible only for supplying blood to the body.

The Fontan procedure is palliative, not curative. But in many cases it can result in normal or near-normal growth, development, exercise tolerance, and good quality of life. 

…In many cases, patients will eventually require heart transplantation.

Meet Paul Cardall

Born in 1973 with a complex heart defect, Paul was not expected to live.

He had 5 open heart surgeries, the first done when he was just 22 hours old. He’s also endured at least 10 other cardiac procedures.

His condition worsened when his heart began to fail 22 years after the Fontan procedure.

He was on the UNOS waiting list for just over a year when he got the call that changed his life forever. Musician, Paul Cardall is the oldest living patient in Utah with Tricuspid Atresia to survive this long without a heart transplant.

A Hometown Hero

Heart Transplantation

Sometimes…

even the best treatments fail, at that point, a

patient can go on the national registry to wait

for a donor heart, they may wait days, weeks,

months or even years before they ever get that

call. Some may not make it that long.

The unfailing optimism of Paul Cardall paid off, when on September 10th his dream came true. Paul received a new heart. It fit perfectly and began to beat instantly inside his chest without needing the defibrillator. He was off the ventilator in less than 8 hours, and walked out of the hospital 2 weeks later.

Paul with his daughter Eden. He and his family hiked Little Cottonwood Canyon this past weekend, just 3 weeks after the transplant.