Idiopathic hypertrophic sub-aortic stenosis (IHSS)

Abstract

A new diagnostic method for idiopathic hypertrophic sub-aortic stenosis (IHSS), using synchronized ECG gated 201Tl myocardial scintigraphy, is described. Twenty patients previously diagnosed as IHSS were evaluated by sequential three-view ECG gated 201Tl and 45 degrees left anterior oblique multigated (MUGA) cardiac blood pool imaging (Group A). The results were compared with two control groups: 20 patients with no previous history of heart disease (Group B), and 20 patients with coronary ischaemic heart disease (Group C). We suggest the following combination of scintigraphic signs as typical and diagnostic to obstructive IHSS: Elevated left ventricular ejection fraction (mean 83.9% +/- 6.15S.D. versus 72.5% +/- 5.35S.D. and 51.2% +/- 13.65S.D. in Groups B and C respectively); Systolic left ventricular cavity obliteration (71% of obstructive IHSS patients versus 0% in Groups B and C); A ratio of more than 1.3:1 in septal to free wall thickness (100% of patients with IHSS); Perfusion/wall-motion mismatch of septum was demonstrated in all (100%) of patients with IHSS (versus 0% in Group B and 10% in Group C). We suggest this last finding as a new specific diagnostic sign for IHSS, with the ability to differentiate between patients with ischaemic coronary heart disease and IHSS patients; whilst both groups may present chest pains, the first group was characterized by 'matching' of perfusion and motion of the affected myocardial wall while the patients with IHSS had 'mismatching' of these parameters. We suggest gated 201Tl myocardial scintigraphy as an additional sensitive tool for the early diagnosis and evaluation of IHSS patients.