Every year, 100,000 babies don't make it to their first birthday because of CHD.
Alicia Campopiano | CHD Awareness Week

February 7th-14th is CHD Awareness Week!  We’re honoring one Heart Warrior every day; each one we have selected suffers from a different congenital heart defect.  Our hope is that we can shed some light and awareness about the many different defects that Heart Warriors all around the world are battling.

We’re kicking things off with Alicia Christine Southorn Campopiano!  Alicia was born on September 10th, 1980, unbeknownst to her parents, she was living in congestive heart failure until ten weeks old.  She had a hole in her heart and a collapsed lung.

Alicia was finally diagnosed with total anomalous pulmonary venous return, commonly referred to as TAPVR.  After her diagnosis, Alicia underwent open-heart surgery to correct the problem.  Her family and priest gathered in the hospital to pray over and baptize her before she was wheeled into surgery on Thanksgiving Day.  Her open-heart surgery was the first ever perfect correction of its kind and Alicia is the oldest living survivor of TAPVR.  The artificial valve that doctors placed in her tiny baby heart has grown and changed with her for 36 years.

TAPVR

Today, Alicia is a proud mother, wife and Heart Warrior.  She doesn’t face any restrictions as a result of her condition and isn’t on any medication for her CHD.

Alicia describes herself as determined, independent and strong.  She said, “Though my cardiologist said I may be “lazy” and no one was certain that I’d live to the ripe old age of five, I never let anyone or anything stop me from doing what I wanted to do, ever.  Though my mother tried to coddle me, I wouldn’t allow it.  I used my struggle to push me forward in life and I used my heart surgery as strength, not a weakness, proving I could do anything my peers could do, and because of my difference, I was a very spirited girl.  I have given birth to three children and I run, cycle and lift weights regularly.  I don’t live in fear and I feel very blessed to be a 36-year old mother.”

About TAPVR

This is a very rare CHD where the veins bringing blood back from the lungs don’t connect to the left atrium like usual.  Instead, the four pulmonary veins go to the right atrium of the heart by way of an abnormal (anomalous) connection.

This particular defect has many forms.  Obstructed TAPVR is the most severe form.  The anomalous veins are obstructed, or narrowed, prior to them entering the right atrium, which makes it difficult for blood to return to the heart from the lungs.  Babies with this form of TAPVR are critically ill and will require emergency intervention.  Unobstructed TAPVR is the less severe form.  In these cases, the child is usually only mildly cyanotic (blue in color) and will often go several weeks before being diagnosed.

The long-term outcome after surgical repair is very good.  Because the surgery corrects the issue and results in a normal circulation, children usually grow and develop normally.  TAPVR accounts for 1.5% of all cardiovascular anomalies.

TAPVR

Types of TAPVR

Supracardiac Total Anomalous Pulmonary Venous Return

The pulmonary veins drain to the right atrium via the superior vena cava.  In this type of TAPVR, the pulmonary veins first come together behind the heart and then drain upwards to an abnormal “vertical vein.” This vertical vein joins the innominate vein which connects to the right superior vena cava and drains to the right atrium.

Cardiac Total Anomalous Pulmonary Venous Return

The pulmonary veins come together behind the heart and then drain to the right atrium through the coronary sinus.  The coronary sinus is the vein that normally returns blood from the heart muscle itself back to the right atrium after its oxygen has been depleted.  The coronary sinus drains directly into the right atrium.

Infracardiac Total Anomalous Pulmonary Venous Return

The pulmonary veins drain to the right atrium via the hepatic (liver) veins and inferior vena cava.  In this type, the pulmonary veins join together behind the heart and then typically drain downwards, connecting to the liver’s portal vein system.  They then drain through the vascular bed of the liver and enter the right atrium from the hepatic veins.

All types of total anomalous pulmonary venous return have to have an atrial septal defect (ASD).  An ASD is a hole in the wall between the right and left upper chambers of the heart.  This hole will allow some of the oxygenated blood that has entered the right atrium from the pulmonary vein to go across to the left atrium and out to the body.

Source: cincinnatichildrens.org

February 07, 2017 by Lani O'Gorman