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  • Title: Isometric cardiac contraction. a possible cause of the disorganized myocardial pattern of idiopathic hypertrophic subaortic stenosis.
    Author: Bulkley BH, Weisfeldt ML, Hutchins GM.
    Journal: N Engl J Med; 1977 Jan 20; 296(3):135-9. PubMed ID: 556638.
    Abstract:
    The bizarre septal architecture of idiopathic hypertrophic subaortic stenosis (IHSS) may be secondary to a small systolic cavity with late systolic isometric contraction. We examined ventricular muscle for IHSS-type muscle-fiber disarray in infant hearts in which isometric contraction would occur during development-namely, pulmonary or aortic-valve atresia with intact ventricular septum and normal atrio-ventricular valves. Fifteen patients with aortic atresia and 10 with pulmonary atresia were compared to 25 normal controls of matched age and heart weight. Aortic atresia showed disorganization of muscle-fiber alignment of left ventricle, particularly septum, and intramural coronary-artery changes virtually identical to IHSS. Pulmonic atresia had similar right ventricular disarray and vessel changes, again most marked in the septum. Thus, cardiac muscle-cell disorientation similar to IHSS occurs in infant ventricles with outflow-tract obstruction. This IHSS-type myocardial-fiber disarray may result from altered wall stresses related to isometric systolic contraction.
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