Young Hearts at risk
Level - GradSabrina was born January 5, 1981 with myotonic dystrophy, a form of muscular dystrophy. This disorder is associated with life threatening heart problems, which are poorly understood by both physicians and cardiologists. Arrhythmia and other dystrophic affects are common manifestations of the disorder.
Eleven years later, Sabrina had three diagnostic procedures done: a 12 lead EKG, cardiac ultrasound and a 24-hour Holter monitor. Although the QRS was prolonged, it was in the normal range so the pediatric cardiologist recommended regular check-ups every 2-3 years. In December 1997, Sabrina was admitted to the emergency room with atrial fibrillation. An IV was administered and she converted to normal heart rate so was discharged in the morning. In August, she was seen again and the pediatric cardiologist judged the EKG she was given to be normal. In October, her mother and a doctor familiar with myotonic dystrophy saw the most recent EKG only to learn that her pulse rate had been 44 and she was having multiple PVC’s. A new pediatric cardiologist was found and Sabrina visited him in November. Sabrina's mother showed him an article recommending early intervention with a pacemaker but he said it was too invasive and that he needed to consult with a specialist in New York before making such a decision. About a week later, Sabrina’s heart stopped while getting out of the bathtub and they were unable to revive her with CPR or defibrillation. Sabrina was 17 at the time of her death.
Given the characteristics of a pediatric myotonic heart determine the characteristics of a new, pediatric implantable pacemaker/defibrillator that might save future Sabrinas. Getting such a device through FDA approval will require that you provide a sound electrophysiological basis of how the device works in young people, (and how its function might differ than ones implanted in adults) and a candid evaluation as to the age at which such a device can be implanted.
Resources:
Paul Levine, MD FACC
Vice President and Medical Director
St. Jude Medical CRMD Plevine@sjm.com
James Duncan BS MS
770-640-8674, 877-937-4625
James and Kathy Hall 405-485-8692 (parents)
Bill Ditto- Room 3306