When young heart patients become adults
When 41-year-old James Angus goes to the doctor, his feet dangle over the end of the examining table and paper butterflies hang from a rainbow banner on the ceiling. Instead of anatomy charts, the walls hold images of a happy little steam engine or some other whimsical character.
Angus is an adult with a childhood disease. Like an estimated five in every 1,000 babies, the Rhode Island customer service representative was born with a malformed, poorly functioning heart. Before 1960, only 10% of infants with serious heart defects survived. With surgery, the survival rate now is 80%.
Today, about 800,000 U.S. adults function with patched-up hearts. Some are fine. Others are not. Told they were “cured,” many instead find their adult lives defined by repeat surgeries and an uncertain future.
The first to test the longevity of the repaired heart, they have trouble finding specialists familiar with their conditions. Pediatric cardiologists face the unknown when little hearts mature. And adult cardiologists, unfamiliar with this type of surgical repair, routinely miss or misdiagnose treatable complications, said Amy Verstappen of Philadelphia, president of an advocacy group called the Adult Congenital Heart Assn.
“When we’re in our 30s and 40s, we completely fall through the cracks,” said Verstappen, who was born with a crisscrossed heart structure known as transposition of the great arteries. “What we hear all the time is that you should be really happy you lived this long.”
Now the medical community is taking note, following the lead of Dr. Joseph Perloff at UCLA, who opened a clinic in the late 1970s. His colleagues refer to him as a pioneer, the father of the field.
One recent patient, Los Angeles filmmaker Marc Ostrick, respectfully described Perloff as “the Yoda of adult congenital heart disease,” referring to the wise elder of the “Star Wars” movies. The depth of Perloff’s knowledge was palpable to Ostrick at their first meeting.
“When he touches you and listens to your heart, you can see it in his eyes,” he said.
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Unique medical needs
Perloff’s touch comes from years of tracking patients such as Ostrick. The long list of possible cardiac birth defects ranges from the relatively routine hole in the heart to the complex “blue-baby” syndrome called tetralogy of Fallot.
As doctors learned how to rebuild these tiny hearts in the 1970s, Perloff realized these babies were going to grow up with unique medical needs. But he said his concerns initially were met with either indifference or hostile resistance from other cardiologists.
Specialists can be territorial, and pediatric cardiologists thought they could handle these patients, he said. Perloff disagreed and pushed on.
“These are complex patients, and they are not going to thrive if they don’t see specialists,” he said. “My goal is to create the next generation of cardiologists to take care of them.”
Perloff appears to be succeeding. Today, a handful of established clinics tend to these patients, and more are emerging. In addition to UCLA, the best known are in Toronto; London; the Mayo Clinic in Rochester, Minn.; and Boston, where doctors from Children’s and Brigham and Women’s hospitals run a joint unit.
This is where Angus eventually landed when he learned the headline-making 1962 surgery that saved his life did not guarantee a long one. After a relatively normal childhood -- minus competitive sports -- he began to suffer bouts of arrhythmia in college. Unable to control recurrences of these racing heartbeats for any length of time, his doctor had some jarring news.
“They expected me to make it to 45 or 50 years old and then my heart would give out,” Angus said. “That was the first time I had heard any time frame.”
Additional surgery in 1993 helped, but the episodes of arrhythmia returned. By that time, he had moved from Vermont to Rhode Island and started going to the Boston clinic. Last winter, he was shoveling snow when his heart began racing again. He was hospitalized and by spring, Angus, like many young heart patients, had a pacemaker implanted.
Dr. Michael Landzberg and his colleagues hold one of their adult clinics Wednesdays in four examining rooms at Children’s Hospital.
On a recent fall day, “The Rugrats” played on the waiting room television. But adults without children -- patients, not parents -- clustered in a separate, quieter section.
Inside, a 48-year-old secretary with an up ‘do and a thick Boston accent got a peck on the cheek from the tall, graying Landzberg. Then she got a firm but gentle lecture about birth control. As with some heart patients, a pregnancy could be risky for her and any unborn child.
Next door, another doctor tended to a weary-looking Sheila Merchant. Like about 10% of the patients at these clinics, Merchant’s defect went undetected for years. She had surgery at age 28, and 24 years later, her suburban doctors were no help when her health began to decline.
Merchant found her way to Children’s, where doctors are treating her for pulmonary hypertension, an exhausting form of high blood pressure that strains the heart and lungs.
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Most don’t find help
People such as Angus always are relieved to find clinics like this one, but the problem, doctors say, is that most don’t. Some stick with specialists they’ve known since childhood. Others lack the resources to travel to a specialty center, or they stop going to the doctor because they seem to be fine.
Ostrick, who had two childhood operations to correct his tetralogy of Fallot, ended up in Perloff’s care only because he relocated to Los Angeles. He had no symptoms and felt fine for years, until Perloff told him his valves were leaking, dangerously straining his heart.
The result was a new pig valve for Ostrick and a documentary titled “Open Hearted,” which follows his family from diagnosis to recovery.
“This was my form of creative therapy,” said Ostrick. “What better drama -- a life-and-death story and a great cast of characters? And I didn’t know how it would end.”
The film had a happy ending, and Ostrick is back on movie sets, where he produces behind-the-scenes documentaries for DVDs. Doctors such as Landzberg urge their patients to do what Ostrick did: take ownership of their illness and play a role in their medical odyssey.
“The number of times we fix or cure is not important,” said Landzberg. “People are still left with illness. That’s the most difficult thing for them to get a grip on.”
