Progressive Myocardial Fibrosis in a Patient With Apical Hypertrophic Cardiomyopathy Detected by Cardiovascular Magnetic Resonance
A 60-year-old white man initially presented to the outpatient clinic in 2002 complaining of atypical chest pain during the last 6 months. A resting ECG showed high R waves and giant negative T waves in the anterior leads (Figure). Cardiovascular magnetic resonance (CMR) showed the typical appearance of apical hypertrophic cardiomyopathy (Figure, A and B). Delayed enhancement imaging did not show relevant scar tissue during the baseline study (Figure, C and D). The patient was put on a β-blocker and an angiotensin-converting enzyme inhibitor and was asked to come back for a follow-up examination 2 years later.
At follow-up, wall motion studies did not show any significant morphological or functional change compared with the baseline examination (Figure, E and F). Delayed enhancement imaging, however, showed newly developed fibrotic tissue located in the apical area of the left ventricle (Figure, G and H). Perfusion imaging during adenosine stress was performed and showed a circumferential subendocardial perfusion defect (Figure, I, J, and K). Because of the patient’s continued complaints of chest pain and the CMR proof of ischemia, the patient underwent coronary catheterization, during which coronary artery disease was excluded as a potential cause of ischemia. A conservative antianginal treatment including nitrates was continued.
Previous reports have shown that apical hypertrophic cardiomyopathy can be associated with apical aneurysm formation, although its underlying mechanism remains unclear. However, it can reasonably be assumed that sustained relative ischemia (ie, mismatch between blood supply and the increased oxygen demand of the severely hypertrophied myocardium) might represent a pathogenetic factor in the formation of fibrotic tissue. This case report illustrates that CMR techniques allow a detailed characterization of both morphological and functional abnormalities, including the detection of subendocardial ischemia. Furthermore, CMR can be applied for longitudinal follow-up studies to detect progression of fibrotic tissue formation associated with this rare form of hypertrophic cardiomyopathy.
Sources of Funding
Dr Nagel has received a research grant from Philips Medical Systems.
Dr Nagel has served on speakers bureaus for, received honoraria from, and served as a consultant to Bracco and Amersham. The other authors report no conflicts.
The online-only Data Supplement, which contains 3 movies, is available at http://circ.ahajournals.org/cgi/content/full/114/5/e75/DC1.