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Megan McElhaney

Pathways to Resolving Pulmonary Atresia

Being a day care center director before the birth of her daughter, Megan, probably gave Beth McElhaney an edge when it came to child care. But nothing could have prepared her for the news that her first-born had life-threatening heart defects. At 7 pounds 9 ounces, and 20 inches in length, "Everything seemed fine at first," according to her mother, but when the baby was taken for a bath, nurses noted her color was bluish. Subsequent tests revealed a heart murmur and extremely low blood oxygen levels.

Within hours of her birth, Megan was rushed by ambulance to Children's Hospital of Pittsburgh of UPMC for full diagnosis and, ultimately, the open heart surgery that saved her life.

Despite that rocky start, when you see Megan today, you would never suspect the experience of August 2004 when she was born with a non-functioning critical heart valve and a hole between the right and left chambers.

A Rare Condition

Pulmonary atresia is this rare condition in which the pulmonary valve that controls blood flow to the lungs is blocked. It develops in the first eight weeks of pregnancy from unknown causes and goes undetected in utero while the baby is reliant on the mother's blood supply for oxygen. Often, as was the case with Megan, pulmonary atresia is accompanied by an underdeveloped right ventricular chamber and an atrial septal defect, a hole in the muscle wall that separates the upper heart chambers. Once the baby is born and uses her own lungs for blood oxygenation, the effects of pulmonary atresia quickly become apparent.

Helping to keep Megan alive as she was being transferred to Children's, prostaglandin was given intravenously to maintain the opening of a prenatal passageway, the blood vessel called the ductus arteriosus. This vessel, found in the heart of every fetus, bypasses the lungs, which are not needed before birth, then closes shortly after, as the lungs take over.

Upon arrival at Children's, still in shock from the sudden turn of events, Megan's father, Jason McElhaney, said that doctors from the Heart Center team calmly explained in layman's terms what was going on. Doctors completed a comprehensive examination, including a heart catheterization to look into Megan's walnut-sized heart. Surgeons led by Victor O. Morell, MD, chief of the Pediatric Cardiothoracic Surgery Division, conducted the operation on Megan's third day of life. "We were really blessed to have Dr. Morell and his team to perform the surgery on her," said Jason.

Opening the Flow

First, a transanular patch was made to widen the base of the pulmonary artery where it exited the small right ventricle to promote blood flow. A monocusp valve made of Gore-Tex material was put in place of the defective valve. As a temporary measure, a Blalock-Taussig (B-T) shunt, also made from Gore-Tex material, was put between the aorta and pulmonary artery, mimicking the function of the ductus arteriosus before birth. "The shunt helps to supplement blood flow to her lungs until her ventricle can relax enough to let blood go forward the normal way," said her cardiologist, Linda Russo, MD, FAAP.

Megan also had feeding and arrhythmia issues, not uncommon for her surgeries, which delayed her initial departure from the hospital. She was on multiple medications and dependent on a feeding tube when released from Children's, posing a parental challenge that was helped by twice-a-week visits from a nurse.

Over time Megan's right ventricle improved enough that when she reached 18 months of age, an embolization coil was placed within the B-T shunt to close it off. Children's catheterization team, lead by Jacqueline Kreutzer, MD, director of Interventional Cardiology, performed the procedure.

Megan's heart condition will require lifelong medications. Follow-up steps may include surgical closure of the opening between the two heart chambers if it doesn't close on its own, and eventual replacement of the pulmonary valve when she is a teenager or young adult.

Today Megan is a happy, carefree child who, according to her mother loves to color, play with her dolls and a kitchen set like other children her age. And considering all she has been through, that makes her nothing less than extraordinary.

Comfort and Support When Needed the Most

Most of Megan's first month of life was spent at Children's with her parents nearby. With a ratio of one nurse to each patient, the Cardiac ICU was "wonderful and very accommodating to us," Beth said.

"With me just giving birth, it was really difficult. I was in a wheelchair. I hadn't held Megan since right after she was born." Beth said. So at the time of the surgery, besides taking care of Megan, "The nurses were checking on me all the time. They took Polaroids of Megan when she was asleep and would surprise me with pictures."

The McElhaneys also found support through cyberspace. Within days of the surgery, Jason and Beth tapped into CarePages, free personal private Web pages offered by Children’s Hospital of Pittsburgh of UPMC, which help family and friends communicate when a child is receiving care. Instead of spending all their time on the phone, the McElhaneys could retire to a computer at the hospital and post updates as progress reports came in from the medical staff.

CarePages provide two-way communications, enabling Beth and Jason to receive hundreds of messages of comfort and support. More than twenty-four months after Megan's birth, initial surgery and follow-up treatment to close her shunt, the McElhaneys continue posting updates about her health and development, as well as photos. "My aunt in Alaska checks it all the time," Beth said.

"The CarePages allow you to share your experiences," Jason said, "and within the CarePages community you find you're not alone in what you're going through."

Last Update
February 19, 2014
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Last Update
February 19, 2014
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