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Volume 25

Number 3

February 7, 2014

Hospital of the University of Pennsylvania

CHANGING THE COURSE of Hear t D i s eas e

Jessica (Maciey) Minot, a nurse on Ravdin 9, never knew her older sister, who died of what was diagnosed as a “congenital heart defect” at eight months old. And she was only seven years old when she lost her mother to heart disease as well. Her mom was 29. “I don’t have a lot of memories of her but I do remember her being really sick,” she said. Three years ago, heart disease suddenly claimed her older brother as well, when he was 24. “He had no symptoms that we knew of.” The terrible fates in her family tree can all be traced to dilated cardiomyopathy, a weakening or enlargement of the heart muscle. Its lifethreatening presence can remain undetected for years and then suddenly strike. And it can be passed from parent to child as an inherited genetic abnormality. In a sense, Minot was lucky. When she was in elementary school, her primary care physician, knowing her family history, referred both her brother and her to CHOP. At the time,

INSIDE New Advisory Council Board ..2 Our DAISY Winners .................2 ICN Revises Primary Nursing Model ..........................3 MS-150 Results Set Record .....4

 Jessica Minot (c.) with husband, Matt, and Mariell Jessup at a conference of the American Heart Association.

her brother’s heart was fine but Minot had mitral valve regurgitation; the valve wasn’t closing tightly, which caused blood to flow backward into her heart. She received an echocardiogram each year to keep watch on the defect. Right before college, the annual echo showed that her heart had doubled in size. “I had no symptoms before I was diagnosed,” she said. “I never would have known without my yearly check-up.” Her cardiologist referred Minot to another CHOP specialist but treatment didn’t seem to improve her heart function — in fact it was decreasing. In 2007, the 20-year old was referred to Mariell Jessup, MD, of Cardiovascular Medicine. As she described her symptoms and family history during the initial visit, Jessup suddenly realized she had treated Minot’s mother, 20 years earlier. “I was watching the cycle of familial cardiomyopathy repeat itself before my eyes,” she recounted.

Early Detection is Key The sooner cardiomyopathy is detected, the more effectively it can be treated — or even prevented, such as in children or

young adults who have inherited the family genes. “When one person in a family has an unexpected death or cardiac condition, it’s wise to evaluate the whole family,” Jessup said. “We tell patients to make sure they talk to brothers and sisters, and recommend an evaluation for their children.” The Familial Cardiomyopathy program, a partnership between Penn Medicine and CHOP, specifically targets these patients. If an adult patient is diagnosed with cardiomyopathy, “we’ll send their children to CHOP to be evaluated and vice versa,” Jessup said, adding that “of people with poor heart function and no obvious cause (ie, coronary artery disease, longstanding hypertension), about 40 percent could have inherited their condition.” Today, Minot is doing well. Her medication regimen has more than doubled her ejection fraction, a measure of how well heart pushes blood through her veins. “New medical therapies have been shown not only to make patients feel better and live longer, but also improve heart function,” Jessup said. (continued on page 2)

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