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Cardiovascular Report - When a Chance Discovery Can Be Life-Saving

Spring 2012

When a Chance Discovery Can Be Life-Saving

Date: April 23, 2012

Theodore Abraham
Theodore Abraham uses a series of ultrasound tests to evaluate patients for hypertrophic cardiomyopathy.
photo by Keith Weller

Even though he went through brain and open heart surgeries within two years, Francis McGuriman considers himself lucky. The 43-year-old postal service employee from southern New Jersey dodged two life-threatening conditions: a brain aneurysm and a classic case of hypertrophic cardiomyopathy (HCM).

During the testing before surgery for his aneurysm, a heart murmur was detected and McGuriman was told he might have HCM. “The doctor was not 100 percent sure, so after my brain surgery, I did some research on the Internet to find a specialist in HCM,” he says. “I was surprised because I had no symptoms of a heart problem.”

That is usually the case, says cardiologist Theodore Abraham, who runs the large HCM program at Hopkins, where McGuriman decided to seek treatment. “Although the disease causes thickening of part of the myocardium and can disrupt blood flow from the heart or cause arrhythmias, it’s usually only discovered as an incidental finding, when a patient has a heart murmur or an abnormal EKG.”

Proper diagnosis and treatment are important, says Abraham, because “everyone with HCM is at risk of sudden death. In fact, it is the number-one cause of sudden death in young athletes, and it can also lead to disabling cardiac symptoms, including shortness of breath and chest pain.”

McGuriman had a four-part series of ultrasound tests that Abraham performs to diagnose and evaluate the extent of HCM: a standard echocardiogram, a treadmill stress echo test, an ultrasound with contrast, and detailed strain imaging.

Strain imaging, also known as myocardial deformation mapping, shows how well individual segments of heart muscle are able to expand and relax, providing detail that cannot be obtained by other modalities. Sometimes an MRI or additional blood or imaging tests are needed to evaluate the condition. Patients also are referred for genetic testing for family members because HCM is caused by a genetic mutation, and 60 percent to 70 percent of cases are inherited.

In very mild forms of HCM, treatment may only require low doses of a medication, such as beta blockers. In patients with obstructive forms of HCM, associated with high pressures inside the heart, the first line of treatment usually includes beta blockers or calcium channel blockers, sometimes with the addition of disopyramide. Abraham says some standard hypertension medicines, such as ACE inhibitors, are contraindicated because they may increase the pressures within the heart.

“If medication isn’t tolerated or doesn’t bring the pressure down, there are effective interventional and surgical approaches to alleviate the obstruction, depending on the location and severity of the blockage and other factors” he says.

Because HCM also can cause potentially fatal heart rhythm problems, some patients may need an implantable cardiac defibrillator (ICD) to detect and correct an arrhythmia.

One treatment approach, alcohol septal ablation, is a percutaneous procedure in which alcohol is injected into a septal branch of the left anterior descending artery to close off the blood supply to the segment of heart muscle causing the obstruction. The other option is a surgical myectomy to remove the heart muscle tissue causing the obstruction.

McGuriman took high doses of medication for about two years after his HCM diagnosis but his pressure gradients did not come down to an acceptable level and he started experiencing side effects of the drugs. He ended up needing a myectomy, which was performed by Cardiac Surgery Director Duke Cameron.

“I had a fantastic recovery with absolutely no problems and I was able to go back to my job as a letter carrier in 11 weeks,” says McGuriman. “I never imagined that I’d need open heart surgery and brain surgery—all within two years—but I understand the gravity of both conditions and know how lucky I am to be here.” 

Hypertrophic cardiomyopathy specialist Theodore Abraham runs an annual heart screening program for young athletes called Heart Hype. This year’s screening will take place at the national Junior Olympics track and field competitions to be held in Baltimore in July.