Phenotypic expression of hypertrophic cardiomyopathy (HC) is particularly heterogenous, with a wide spectrum of patterns of left ventricular (LV) hypertrophy reported. Although absolute maximal LV wall thickness is predictive of outcome, it is unresolved whether there is a relation between the precise distribution of LV wall thickening and clinical course.
Case Descriptions
The 3 patients depicted here, recently evaluated at the Hypertrophic Cardiomyopathy Center (Minneapolis, Minnesota) during 1 month, demonstrated very similar but unusual phenotypes of nonobstructive HC, intuitively suggesting severe disease expression and poor outcome. Nevertheless, these patients presented at a broad spectrum of ages (83, 65, and 38 years) and with no or little heart failure–related disability. The 38- and 65-year-old patients are asymptomatic and the 83-year-old patient (who has achieved statistical life expectancy) has only mild New York Heart Association functional class II symptoms, despite marked left atrial dilatation, severe mitral regurgitation, chronic atrial fibrillation, and extensive LV delayed enhancement on contrast cardiovascular magnetic resonance imaging.
On the basis of echocardiographic and cardiovascular magnetic resonance imaging ( Figure 1 ), all 3 patients showed LV hypertrophy predominantly situated in the distal half of the chamber (22, 21, and 35 mm in thickness, respectively) in the absence of mitral valve systolic anterior motion and outflow obstruction. Notably, the cephalad (basal) segment of the ventricular septum was particularly thin (5 to 7 mm) and elongated (25 to 40 mm in length) and largely responsible for the bizarre morphologic appearance, which is distinct from the typical form of “apical HC.”
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