Tetralogy of Fallot and Discontinuous Pulmonary Arteries: Zane's Story

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At 3 days old, Zane underwent heart surgery to repair a complex combination of cardiac defects. Within days he needed a pacemaker implanted to keep his heart rhythm regular. Despite these early health challenges, Zane and his family refused to let his condition get in the way of an active life.

Zane "I grew up as a normal kid," Zane says. "I wrestled with my siblings, played outside, was on the tennis and soccer teams in high school, and have always enjoyed hiking and camping."

Now 22, Zane is a pre-med student at West Chester University in Pennsylvania, with the goal of becoming a pediatric cardiologist like the physician who first identified his condition and has treated him ever since — Jack Rychik, MD, an attending cardiologist with the Cardiac Center at Children’s Hospital of Philadelphia (CHOP).

Heart murmur leads to scary diagnosis

Zane appeared to be a healthy baby at birth. But as he and his mother, Sarah, were preparing to leave the hospital in Phoenixville, PA, she noticed his coloring had changed. She asked a nurse to listen to his heart as a precaution; a cousin had been born days before with a serious heart condition.

The nurse detected a slight heart murmur, which was confirmed by the attending physician. “They told us Zane needed to be looked at by a cardiologist at CHOP immediately,” remembers Zane’s father, Mark.

Zane was transported from Phoenixville to Bryn Mawr, where Dr. Rychik was consulted on Zane’s case and an echocardiogram was performed. After reviewing the echocardiogram images of Zane’s heart, Dr. Rychik diagnosed the baby with tetralogy of Fallot (TOF) and discontinuous pulmonary arteries. Zane had a complex combination of characteristics:

  • Ventricular septal defect (VSD): a hole between the two bottom chambers (the ventricles) of the heart that eject blood to the body and lungs.
  • Overriding aorta: The aorta, the large artery that takes blood to the body, is on top of both ventricles, instead of just the left ventricle as in a normal heart.
  • Pulmonary stenosis: a narrowing of the pulmonary valve, the area below the valve, or the pulmonary arteries, which carry blood from the heart to the lungs.
  • Hypertrophy: The right ventricle becomes thicker and more muscular than normal as a result of working harder to pump blood through the narrow pulmonary valve.
  • Discontinuous pulmonary arteries: In a normal heart, the pulmonary arteries are connected. In Zane’s case, the vessels going to the lung were separated. One was coming from the heart, and the other from a patent ductus arteriosis (PDA) — a condition in which an opening between the right side of the heart and the aorta (which is needed during prenatal development) doesn’t close at birth. The vessels weren’t communicating.

Because of the combination of Zane’s heart problems, Dr. Rychik told the family that he needed to be transferred to Children’s Hospital of Philadelphia for surgery. Medication was used to keep the PDA open until surgery, as Zane would lose blood flow to his left lung if the PDA closed before surgery.

Surgery at 3 days old

An ambulance transported Zane to CHOP. When Zane was just 3 days old, he underwent open heart surgery with Thomas Spray, MD, then-Chief of CHOP’s Division of Cardiothoracic Surgery.

Dr. Spray closed the hole between the two ventricles with a patch and opened the pulmonary valve more fully, both as planned. To treat the narrowed pulmonary artery and PDA, Dr. Spray used an innovative technique during surgery. He cut the pulmonary artery in half, flipped it over the opening, sewed a patch on it, then flipped it back.

It worked. Zane came out of surgery with stronger circulation.

Zane needs a pacemaker

A new problem soon emerged. After the surgery, Zane developed heart block. A second procedure was done to implant a pacemaker, a small device that uses electrical pulses to control abnormal heart rhythms and prompt the heart to beat normally.

Once the pacemaker was implanted, Zane’s heart problems largely resolved. He was able to go home and lead a relatively normal life, with close monitoring and regular follow-up visits with Dr. Rychik. During Zane's first year of life, he needed two angioplasties, a catheterization used to widen constricted arteries and remove scar tissue.

In the 20 years since, Zane has only needed minor surgery to replace the pacemaker when the generator needed to be replaced.

“… he can do whatever he wants”

For some children, complex cardiac conditions can impact their lives and affect everyday activities. That wasn't the case for Zane.

“Dr. Rychik told us he can do whatever he wants,” says Mark. “He told us not to baby him.”

To protect Zane's heart and pacemaker, a few activities were put off limits — waterskiing, weight lifting and rock climbing, for example — but none really interested Zane so he didn't care. Instead, he wrestled with his siblings, loved playing outdoors and joined his high school's tennis and soccer teams. Zane has also always enjoyed hiking, camping and ice skating.

Following his doctor's footsteps

Zane with his siblings at a flyers game Now 22 years old, Zane looks back fondly to his early visits to CHOP.

“I always have fun when I go to CHOP,” he says. “When I was little, I would play hide and seek in the exam room with Dr. Rychik. There were only a couple of places to hide, but he always played along and pretended to have a hard time finding me."

As Zane got older, he remembers how Dr. Rychik would "sit down with me and ask me how I was doing. He has always been genuinely interested.”

“And it wasn’t just Dr. Rychik,” Zane continues.

Everyone at CHOP was so loving and nice to me. Most of the people in the Cardiac Center have known me since I was 2 or 3 years old. They are always happy to see me, and I can tell they really care.

Dr. Rychik has followed Zane’s journey into adulthood with interest. He was pleased when Zane originally decided to go to seminary school, and equally delighted when Zane altered his path two years later and transferred to begin pre-med studies.

Zane now hopes to become a pediatric cardiologist like his mentor. “I am who I am because of Dr. Rychik.”

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