Every year, 100,000 babies don't make it to their first birthday because of CHD.
Nolan James Hill | Heart Warrior of the Week

We talked with Hannah Hill, mom to Nolan, who is a very brave CHD warrior who was diagnosed with not one, but four congenital heart defects. She describes how they found out about his CHD’s, the journey they’ve walked so far and how their small but mighty addition to the family has inspired them to never give up. This is our Heart Warrior of the Week!

Name:

Nolan James Hill.

Diagnosis:

Transposition of the great arteries (TGA), pulmonary atresia, ventricular septal defect (VSD), double inlet left ventricle.

Birthday:  

February 6, 2017.

Was Nolan diagnosed in-utero?

He was not diagnosed in utero; he was born at 4:07 PM and I noticed his feet and hands were blue. I had mentioned it to the nurse in labor and delivery about 30 minutes after he was born, but she said it was normal and it just takes time for the blood to circulate.  I wish I would have trusted my instincts, but I was a new mom and just brushed it off. Besides, I was thrilled just to be holding him. He was about 2 hours old when the nurse took him to do his checkup. It was now a few hours later and the nursery still hadn’t brought him back to our room. It was then that we received the devastating news – the pediatrician came in and told us he didn’t know exactly what was wrong, but it was definitely something with his heart. He was immediately airlifted to Vidant Medical Center that night at 11 PM and was airlifted yet again to Duke Hospital the following day.

How would you describe Nolan in a few brief words?

Nolan is the happiest child, especially having been through so much. He is resilient and such an inspiration to us. 

How many surgeries and procedures has he undergone? 

Six. First open-heart surgery at 4 days old, two heart caths at 3 and 4 months old, second open-heart surgery and third cath at 7 months old, and another cath at 1 year old.

Can you describe the above surgeries and why they were performed?

His first open-heart was the BT Shunt. This was a temporary fix with an artificial valve so that blood could flow to his pulmonary arteries.

The two caths were to increase blood flow and make his left pulmonary artery larger.

The second open-heart was the Glenn procedure – this was a permanent fix to do what the BT shunt was doing, but using his own arteries. 

His third and fourth caths were to help his arteries grow bigger and stronger with increased blood flow to the lungs and body.

Approximately how much time has Nolan spent in the hospital throughout all this? 

A total of about four weeks, with many doctors appointments and overnight stays in between.

Is Nolan on any medications as a result of his heart condition? Is it something he is taking temporarily or will he have to take it for the rest of his life?

Luckily, he is currently only on aspirin! He used to take Lasix but we have been off that for a few months now. The aspirin may be a lifelong thing or temporary, we haven’t been told by his doctors yet.

Does he face any restrictions in his everyday life?

Despite all he has been through, he does not have any restrictions at this point of his journey, which we consider to be a miracle! However, we are very nervous during flu seasons, of course.

Is there a need for any more surgeries?

He will need a third open-heart surgery between ages 2-4, depending on how fast he grows and how his heart continues to do. We will continue with lung profusion scans, heart caths, and seeing his cardiologists every 2 months or so until that time.

What challenges do you face as a CHD Family?

We worry when he gets sick, we worry that he won’t continue to develop or will have learning disabilities. He also has an eight-year-old half-brother who has been deeply by all of this.

What inspires you about Nolan?

He inspires us on a daily basis to never give up. We know in our hearts that he was put on Earth for a purpose and that he will live a fulfilling life serving our Lord and Savior. He is full of joy and happiness and is always laughing. He lives life just as any other healthy child would. We are so blessed to be his parents and couldn’t imagine our life without him. 

About Nolan’s Defects

Transposition of the Great Arteries (d-TGA)

 

Anatomy: In a heart with d-TGA, the aorta connects to the right ventricle and the pulmonary artery to left ventricle, just the opposite of a normal anatomy.

Complications: Because of this abnormal connection, deoxygenated blood is re-circulated to the body before it has a chance to pick up oxygen from the lungs. At the same time, oxygen-rich blood is continuously being pumped from the heart to the lungs in a cyclical pattern, never dispersing the oxygenated blood throughout the body.  This leaves the body with dangerously low oxygen levels.

Most babies are born with a small hole in between their left and right ventricles, after a few hours or days of life, the hole closes on its own. This hole is actually a critical component for some babies with TGA as it allows some oxygen-poor blood to mix with oxygen-rich blood, enabling the body to receive at least some oxygen. Often times, a balloon procedure is performed to enlarge or create this hole to improve the baby’s oxygen levels. Doctors go in through an artery in the leg and use a small medical instrument to create a larger hole between the ventricles.

Symptoms: Blue color of the skin (cyanosis), shortness of breath, lack of appetite, poor weight gain or weight loss.

Treatment: Often times, a balloon procedure via catheterization is performed to enlarge or create the above mentioned hole shortly after birth to improve the baby’s oxygen levels. The repair for TGA is an open-heart surgery where doctors surgically place the aorta and pulmonary valve in their proper positions. This surgery usually takes place at around one week old.

Prevalence: d-TGA accounts for approximately 5% of all congenital heart defects, affecting boys more than girls at a rate of 3:1. It occurs in 2-5 out of 10,000 live births worldwide.

Pulmonary Atresia

Anatomy: Instead of an opening (pulmonary valve) to allow blood to travel from the heart to the lungs, a solid sheet of tissue forms.

Complications: This anatomy cannot support life because the blood never makes it to the lungs to become oxygenated, and oxygen-poor blood cannot meet the body’s demands for normal growth and function.

Symptoms: Abdominal bloating, bluish color to the skin (cyanosis), chest pain, fainting, fatigue, poor weight gain or failure to thrive in infants with severe blockage, and shortness of breath.

Treatment: Depending on the severity of the condition there are a variety of treatment options, including catheterization and valve replacement via open-heart surgery.

Prevalence: This defect accounts for approximately 8% of all CHD’s, occurring in 8 out of every 10,000 live births worldwide.

Ventricular Septal Defect (VSD)

Anatomy: This defect occurs when the heart’s inner wall (septum) that separates the left ventricle from the right ventricle has a hole or multiple holes in it. 

Complications: This hole allows oxygen-rich blood from the left ventricle to mix with oxygen-poor blood from the right ventricle, resulting in a lack of oxygen circulating throughout the body.

Symptoms: Bluish tint (cyanosis) to the skin, lips, and fingernails, along with poor feeding, poor weight gain, and fast breathing.

Treatment: Most VSD’s close on their own with time, but larger ones will require catheterizations or open-heart surgery in which a patch is placed over the hole.

Prevalence: This is the most common CHD, accounting for 25-30% of all congenital heart disease. It occurs in 2-5 out of every 1000 live births worldwide.

Double Inlet Left Ventricle

Anatomy: The left and right atrium are connected to the left ventricle instead of the left atrium connecting to the left ventricle and the right atrium connecting to the right ventricle. The right ventricle is often underdeveloped as well.

Complications: As a result of this defect, oxygen-rich blood and oxygen-poor blood are combined, leaving the body with low oxygen saturations.

Symptoms: Blue skin (cyanosis), failure to gain weight normally, trouble breathing, swollen legs or abdomen, pale skin, poor feeding, sweating, rapid heartbeat, heart murmur, fluid buildup around the lungs, and heart failure.

Treatment: Double inlet left ventricle is treated with open-heart surgery to repair the defect and have each atria connect to the proper ventricle. Several surgeries may be needed in order to make the heart work effectively.

Prevalence: 5-10 out of every 100,000 live births.

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