Related Experiment Videos
[Dysplastic intramyocardial arteries with subaortic septum in patients with hypertrophic obstructive cardiomyopathy]
B Schwartzkopff1, J Dieckerhoff, H Frenzel
1Abt. für Kardiologie, Pneumologie und Angiologie, Heinrich-Heine-Universität, Düsseldorf.
Insights
Dysplastic intramyocardial arteries are linked to increased fibrosis and younger age in hypertrophic obstructive cardiomyopathy (HOCM). These findings highlight a distinct subgroup of HOCM patients with unique pathological features.
Area of Science:
- Cardiovascular Pathology
- Cardiovascular Physiology
- Histopathology
Context:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart disease characterized by left ventricular hypertrophy.
- Abnormalities in intramyocardial arteries have been anecdotally reported in HCM.
- The clinical and pathological significance of these arterial changes remains unclear.
Purpose:
- To investigate the presence and significance of dysplastic intramyocardial arteries in patients with hypertrophic obstructive cardiomyopathy (HOCM).
- To compare the pathological features and clinical characteristics of HOCM patients with and without dysplastic arteries.
Summary:
- Dysplastic intramyocardial arteries (external diameter >100 microns) and arterioles (external diameter <100 microns) were identified in 8 of 24 HOCM myectomy specimens.
- These dysplastic vessels showed medial thickening, fibroelastosis, intimal thickening, and reduced lumen.
- Patients with HOCM and dysplastic arteries (HOCM II) exhibited significantly increased patchy fibrosis (7.2% vs. 0.8%), were younger (30 vs. 53 years), and had a thicker anterior septum (29 vs. 22 mm) compared to HOCM patients without these arterial changes (HOCM I).
- Dysplastic arteries were not found in controls or patients with valvular aortic stenosis.
Impact:
- Identifies a distinct subgroup of HOCM patients (HOCM II) characterized by dysplastic intramyocardial arteries.
- Suggests a potential link between arterial dysplasia, increased myocardial fibrosis, and a younger age of presentation in HOCM.
- Provides histopathological evidence that may contribute to understanding the pathophysiology of HOCM and its varied clinical manifestations.
Abstract:
Abnormal, dysplastic intramyocardial arteries were reported in autopsied hearts of hypertrophic cardiomyopathy. To elucidate their significance, the operatively-excised myectomy specimens of 24 patients with hypertrophic-obstructive cardiomyopathy (HOCM), of 18 patients with valvular aortic stenosis and of 10 postmortem normal hearts were investigated. Eight patients with HOCM had dysplastic intramyocardial arteries (greater than 100 microns external diameter) as well as dysplastic arterioles (less than 100 microns external diameter). The value of the scores for the thickness and fibroelastosis of the media was nearly doubled, the tunica intima was frequently thickened, and the lumen was relatively reduced in dysplastic vessels. Neither in controls nor in aortic stenosis dysplastic arteries were found. Volume density of patchy fibrosis (scars) was increased in patients with dysplastic arterial vessels (HOCM II) (7.2 +/- 4.4 Vv%) (p less than or equal to 0.05) as compared with HOCM without dysplastic vessels (HOCM I) (0.8 +/- 2.3 Vv%), with aortic stenosis (0.9 +/- 1.6 Vv%) or with controls (0 Vv%), Patients with HOCM II were significantly (p less than or equal to 0.05) younger (30 +/- 13 years) than those with HOCM I (53 +/- 12 years), aortic stenosis (56 +/- 12 years), or controls (63 +/- 21 years). The anterior septum was significantly thicker in HOCM II (29 +/- 7 mm) than in HOCM I (22 +/- 4 mm), in aortic stenosis (19 +/- 3 mm), or in controls (12 +/- 2 mm). Syncopes were complained by about 75% (6/8) of patients in HOCM II, by 54% (9/16) in HOCM I, and by 44% (8/18) in aortic stenosis (not significant).(ABSTRACT TRUNCATED AT 250 WORDS)