Extreme Left Ventricular Hypertrophy

Clinical Outcome and Phenotypic Expression in <emph type="ital">LAMP2</emph> Cardiomyopathy

“…Despite youthful age, most of our patients with this metabolic storage disease showed particularly massive LV hypertrophy, and two had the most substantial hypertrophy reported in man (16–18). Histopathology of the hypertrophied LV showed a hybrid architecture with features of traditional HCM due to sarcomere protein mutations (i.e., myocyte disarray, intramural small vessel disease, and myocardial scarring including subepicardial distribution) (17,19–21), but also distinctive evidence of a storage process in which clusters of numerous vacuolated and vesicle-like myocytes were embedded in areas of replacement fibrosis.…”

Glycogen Storage Diseases Presenting as Hypertrophic Cardiomyopathy

“…Cardiac function progressively deteriorated during a six-year period in Family Member CZ III-1, 25 The New England Journal of Medicine and he died at the age of 22 years while awaiting heart transplantation. A pathological study of his heart ( Fig.…”

Subaortic and midventricular obstructive hypertrophic cardiomyopathy with extreme segmental hypertrophy

“…This case is a rare example of a patient with subaortic and midventricular hypertrophic cardiomyopathy with extreme segmental hypertrophy exceeding the usual maximum wall thickness reported in the literature, although Maron et al have published a case of a patient with an even greater hypertrophy (60 mm) [2]. …”