Pakistani Boy Travels to Rochester for Life-Saving Heart Treatment

One-of-a-kind registry tracks children with heart trouble, offers hope

September 26, 2000

When Omer Ropri collapsed and nearly died from a dangerous heart rhythm earlier this year, doctors didn't give his parents much of a choice. For the best care, they should leave their home in Pakistan and move around the world to be near Children's Hospital at Strong.

Although Omer, 12, was a tennis champion, no one knew he suffered from hypertrophic cardiomyopathy, an abnormal thickening of the heart muscle. That's the same condition his 10-year-old brother unexpectedly died of just weeks before Omer collapsed.

Physicians in Pakistan referred the family to Children's Hospital at Strong for two reasons. First, the hospital oversees the North American Pediatric Cardiomyopathy Registry, which was created in 1995. The one-of-a-kind database contains invaluable information about U.S. and Canadian children who have cardiomyopathy, which is the leading cause of heart transplantation during childhood. Secondly, the hospital has a first-class comprehensive cardiomyopathy clinical program designed to treat children for the condition.

Before the registry was created, many questions about cardiomyopathy - an uncommon and potentially devastating disease in which infants and children have weak heart muscle, or too much heart muscle - remained unanswered. Studies showed outcomes for children with cardiomyopathy weren't better than they were three decades prior.

Physicians at Children's Hospital at Strong decided to find out why. During the last five years, they've analyzed data collected through the registry to learn more about cardiomyopathy. For example, they're discovering how the disease presents itself in children, what causes it, and how those with cardiomyopathy fare over time.

In addition, researchers are sifting through information, trying to find common links among patients, whether it is their ethnicity, where they live, family history, or dozens of additional variables.

Studies done elsewhere were limited because of their small sample sizes or ethnically homogenous populations of children. For instance, a population-based study in Minnesota lacked a sufficient number of children to provide reliable estimates of incidence across all age groups.

"To our knowledge, there have been no large, population-based epidemologic investigations of pediatric cardiomyopathy in North America," says Steven Lipshultz, M.D., director of the Children's Heart Center and its cardiomyopathy program, part of Children's Hospital at Strong. "The registry has really allowed us to find out a vast amount of information about these children and their particular situations."

"No one center ever sees enough cases to study cardiomyopathy in depth, but by collecting data from throughout North America, this registry allows us to do just that," Lipshultz adds. "This research is tremendously important to children such as Omer."

The cardiomyopathy registry was detailed this year in an article published in the American Heart Journal.

After arriving in Rochester, physicians at Children's Hospital at Strong gave Omer a series of tests. Carol Buzzard, M.D., performed a cardiac catheterization that confirmed aspects of the diagnosis, and James Daubert, M.D., director of electrophysiology, recommended an implantable cardiac defibrillator to reduce the chance of sudden death.

Daubert, who implanted the device with the assistance of the electrophysiology laboratory staff and anesthesiology group, worked on a multi-center study evaluating the effectiveness of defibillators in people who have hypertrophic cardiomyopathy. That study was published this year in the New England Journal of Medicine.

A larger study to determine how to best identify high-risk patients who warrant defibrillator implantation is now underway and will again involve our electrophysiology team.

Under Lipshultz's care, Omer is taking some experimental medications designed to reduce the amount of muscle in the heart, make it easier for his heart to pump blood throughout the body, and reduce his chances of sudden death.

The program receives important funding from many sources, including the federal and state governments. Sen. James Alesi recently secured state funding to continue research for cardiomyopathy in children. He will be at the hospital for a tour on Wednesday, and will have a chance to meet Omer and his family.

"My role in securing funds for Rochester-area hospitals reflects my commitment to health care in our community," Alesi says. "The research program at Children's Hospital at Strong will help children in our area and around the world as they undergo treatment here for cardiomyopathy. Who could look in the face of a sick child and not want to help? I am pleased to continue my efforts to fund this life-saving program."

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