|Number 13-11: Sawtooth Cardiomyopathy|
Number 13-11: Sawtooth Cardiomyopathy
Case from: Ashish Aneja MD1, Timothy Hoffman MD2, Sharon Roble MD1, Subha Raman MD MSEE1
Institute: 1. Ohio State University, Columbus, Ohio
Clinical history: A 19 year-old male presented for follow-up evaluation for cardiomyopathy following referral from a physician in West Virginia (Figure 1). Since his initial diagnosis of cardiomyopathy at the age of 2, he has remained asymptomatic from the cardiovascular standpoint. He denies any symptoms with ordinary exertion but had not undergone formal stress testing.
Figure 1: Resting 12 lead electrocardiogram
Movie 1: Cardiac MRI cine images demontrating focal areas of hypertrophy consistent with
Figure 2: Myocardial delayed enhancement (PSIR) demonstrating the absence of prior infarct as a
Follow up: The patient continued to deny symptoms despite a follow up electrocardiogram and holter monitor which showed the presence of frequent ventricular ectopy, and non sustaind wide complex tachycardia which was felt to be ventricular tachycardia (Figure 3). He subsequently received a single chamber implantable cardiac defibrillator for primary prevention of sudden cardiac death.
Figure 3: Follow up electrocardiogram demonstrating frequent ventricular ectopy
Conclusion: Sawtooth cardiomyopathy had been previously proposed as the diagnosis based on the patient’s prior invasive study. Disordered cardiac embryogenesis was also suspected. To date the patient has not received any therapies from his implantable cardiac defibrillator.
Perspective: While similar morphology has been previously described, underlying mechanisms of this unusual form of myocardial disease remains unknown.1 Further investigations that would be potentially informative, such as genetic testing and endomyocardial biopsy with precise characterization of ultrastructural abnormalities have not been pursued at this time.2
2. Harvey PA, Leinwand LA. The Cell Biology of Disease: Cellular Mechanisms of Cardiomyopathy. J Cell Biol. 2011 Aug 8;194(3):355-65.
COTW handling editor: Kevin Steel
Have your say: What do you think? Latest posts on this topic from the forum