WUSM clinic devoted to treating deadly, silent heart condition

Every so often we read a news report in which a young athlete collapses and dies during a competition — it’s rare, but it happens. And when it does, often the cause is a silent heart condition called hypertrophic cardiomyopathy (HCM).

The Hypertrophic Cardiomyopathy Clinic at Washington University School of Medicine is devoted to diagnosis and treatment of HCM. Directed by Keith Mankowitz, M.D., assistant professor of medicine and a cardiologist at Barnes-Jewish Hospital, the clinic has expertise in medications, electrical treatment, catheter ablation and surgical interventions for HCM.

“Most cardiologists aren’t specifically trained to manage HCM,” Mankowitz says. “And we’re the only center in the region that focuses solely on how to diagnose and care for people with HCM.”

HCM stems from a thickening of the left side of the heart. This can give rise to abnormal heart rhythms and can interfere with blood flow out of the heart. In some cases, especially with exertion, the heart simply stops beating.

People of all ages can have HCM. For every 500 people in the United States, one will be affected by this inherited disorder. But most will never know because they don’t have symptoms or their symptoms are mild. The condition is usually overlooked during routine physical exams.

The most common symptoms are shortness of breath, chest pain or fainting. And in the most tragic cases, the first and only sign of the disorder is sudden death. Many cases of sudden death in people with HCM occur in young people during exertion.

Most people with HCM have mutations in one or more genes. The mutations lead to disarray in the typically orderly strands of heart muscle. Eventually, genetic tests may be readily available for those who have reason to think they could have one of these mutations. Knowing their genetic risk would allow them to take action to avoid sudden death.

Until then, doctors can screen for HCM with an echocardiogram. Those who have had family members die suddenly, especially at a young age, are at risk for HCM. A young person who has shortness of breath, chest pain or fainting should also be evaluated, as should people with relatives who have thickened hearts or heart pacemakers or defibrillators.

The Hypertrophic Cardiomyopathy Clinic can be reached at 314-362-1291.

Washington University School of Medicine’s 2,100 employed and volunteer faculty physicians also are the medical staff of Barnes-Jewish and St. Louis Children’s hospitals. The School of Medicine is one of the leading medical research, teaching and patient care institutions in the nation, currently ranked third in the nation by U.S. News & World Report. Through its affiliations with Barnes-Jewish and St. Louis Children’s hospitals, the School of Medicine is linked to BJC HealthCare.