One Saturday when he was 4 years old, Aryan started to complain that his belly hurt. When his pain continued for a second day, his parents, Sweta and Chirag, took him to the emergency room at their local hospital in Freehold, NJ. They were worried it might be appendicitis.
The doctor who examined Aryan was concerned about the way he was breathing, and ordered a chest X-ray. The image showed that the right side of his heart was greatly enlarged. Something was clearly wrong, and it wasn’t his stomach that was the problem.
Pediatric pulmonary hypertension
More About Pulmonary Hypertension
Aryan was taken by ambulance to St. Peter’s University Hospital in New Brunswick, which has a pediatric Cardiac Center managed by Children’s Hospital of Philadelphia (CHOP). A cardiologist did an echocardiogram and diagnosed Aryan with pulmonary hypertension. The right side of his heart was enlarged because of high pressure in the blood flowing to the lungs, and Aryan’s heart was having to work too hard to maintain oxygen circulation to his body.
Both parents were stunned. “We’d heard of this as a condition in older people,” Sweta remembers, “But not in a child. We didn’t know what to say, what questions to ask.”
Aryan was transferred to CHOP’s main campus in Philadelphia, where Aryan was admitted to the Cardiac Intensive Care Unit. There, Sweta and Chirag met with the Pulmonary Hypertension Program team including, Brian Hanna, MDCM, PhD. Just 24 hours after going to the local emergency room, Aryan was in the care of one of the country’s leading experts on the treatment of pulmonary hypertension on children.
“The first thing he told us was how lucky we were to have caught this so quickly,” says Sweta. Dr. Hanna explained that pulmonary hypertension is still not widely understood and is often misdiagnosed when symptoms first appear.
Developing a treatment plan
About Cardiac Catheterizations
Dr. Hanna conducted a cardiac catheterization to get an inside look at Aryan’s heart, measure the pressure and refine a treatment plan. During the catheterization, Dr. Hanna also uncovered an anatomical problem. Aryan’s patent ductus arteriosus (PDA), a fetal artery that bypasses the lungs to circulate oxygenated blood from the mother during pregnancy, hadn’t closed like it was supposed to after birth. This was contributing to Aryan’s circulation problems, and Dr. Hanna closed the PDA while the catheterization instrument was inside Aryan’s heart.
Aryan was started on a medication that opens blood vessels to reduce pressure, combined with other drugs. A Broviac catheter, a type of central line, was inserted through one of Aryan's veins as a way of delivering the drugs directly to his heart. He was also given oxygen.
Aryan’s appetite quickly came back, a sign the treatment was working. During the next five weeks, while Aryan remained at CHOP, the pressures in his circulation held steady. His medical team considered his condition stable, and trained Sweta and Chirag to administer the medication and manage the central line at home. When his parents were skilled in the procedures, Aryan was released, with a plan for regular follow-up visits.
At a three-month follow-up visit that summer, Dr. Hanna was concerned about the way Aryan looked. He admitted the boy and performed a cardiac catheterization, which showed that Aryan’s left coronary artery was narrowed (stenotic). This was a critical problem that would require surgery to repair the artery.
Aryan was in for another long Hospital stay. After four weeks, when his condition was stable, he underwent surgery to repair the coronary artery. The procedure was performed by Thomas Spray, MD, Chief of the Division of Cardiothoracic Surgery at CHOP. Aryan spent four weeks recovering in the Hospital after surgery, then was able to go home.
Over time, Aryan's condition — slowly but surely — began to improve. When Aryan was 7, he was well enough that his central line could be removed, and was replaced by a device under his skin (similar to an implanted insulin pump) that could safely deliver his medication. This greatly simplified the management of Aryan’s medication and made it possible for him to go swimming and take baths — activities that had not been allowed because of the risk of infection through the central line.
Aryan is now an active and engaged 10-year-old. He loves to play basketball and hang out with his friends. He’s a straight A student. He’s interested in robotics and plays the piano. And he really loves to go on fast rides at the local amusement park.
“Aryan is a very determined little boy,” says Sweta. “He got up the day after open-heart surgery. He has adapted to it all with no fuss, no hesitation.”
It took his parents a little longer to feel confident that he was going to be OK. “A year or two after the surgery, things began to level off,” says Sweta. “We got used to everything. We thought, ‘We can do this. We have to do this.’”
For the past three years, Aryan has spent a week each summer at the Echo Hill Outdoor School (EHOS) Heart Camp, on Maryland's eastern shore. The camp is run especially for children with pulmonary hypertension, and offers nature and ecology classes, swimming, canoeing, dancing and a zip line through the trees. The all-volunteer medical staff includes physicians, nurses, therapists and psychologists. The program gives the campers an opportunity to push themselves and try new things in a safe and fun environment.
Aryan returns to CHOP every three months to see Dr. Hanna and other members of his medical team. “They know him,” says Sweta. “They know his entire story. Aryan loves Dr. Hanna. They goof around with each other. To me, Dr. Hanna is god. He saved my son. He has a knack for putting us at ease when we are worried. Everyone there is fantastic, the doctors, the nurses, the staff. We’ve had nothing but a great experience.”