AN ATYPICAL PRESENTATION OF A COMMON CARDIOMYOPATHY
2363 JACC March 21, 2017 Volume 69, Issue 11
FIT Clinical Decision Making AN ATYPICAL PRESENTATION OF A COMMON CARDIOMYOPATHY Poster Contributions Po...
FIT Clinical Decision Making AN ATYPICAL PRESENTATION OF A COMMON CARDIOMYOPATHY Poster Contributions Poster Hall, Hall C Saturday, March 18, 2017, 3:45 p.m.-4:30 p.m. Session Title: FIT Clinical Decision‐Making: Heart Failure and Pulmonary Hypertension Abstract Category: Heart Failure and Cardiomyopathies Presentation Number: 1257-381 Authors: Kevin Shah, Jeffrey Hsu, University of California, Los Angeles, Los Angeles, CA, USA
Background: Hypertrophic cardiomyopathy (HCM) rarely presents with isolated posterior left ventricular hypertrophy (LVH). Knowledge of patterns on cardiac magnetic resonance (CMR) and positron emission tomography (PET) is important for diagnosis.
Case: A 31-year old female with a history of hypertension presented with ventricular tachycardia (VT), which was treated with synchronized cardioversion. Coronary angiography demonstrated no obstructive disease, and echocardiography demonstrated isolated posterior wall LVH. CMR revealed asymmetric thickening of the inferolateral wall, delayed hyper-enhancement in a transmural pattern (Figure) and akinesis. Cardiac PET was performed which demonstrated no fluorodeoxyglucose (FDG) uptake within the heart. Total body PET demonstrated posterolateral LV FDG uptake. Decision‐Making: The findings on PET helped lower the likelihood of malignancy (versus infiltrative process), but ultimately a multidisciplinary decision was made to perform myocardial biopsy, which was negative for malignancy and demonstrated myocardial hypertrophy and fibrosis. She underwent cardiodefibrillator implantation and was treated with beta-blocker therapy. The decision to biopsy in this case highlights the possible atypical presentations of HCM. Conclusions: HCM may present as an isolated posterior LVH. CMR may demonstrate transmural late gadolinium enhancement and total body PET should demonstrate physiologic uptake throughout hypertrophied myocardium.