Heart failure & cardiomyopathies

Structural and Functional Characterization of the Aorta in Hypertrophic Obstructive Cardiomyopathy

Circ Heart FailPublished cohort

Authors' conclusion

The increased wall stiffness observed in the aortas of obstructive patients with HCM is associated with structural alterations in the medial lamellar unit, including changes in smooth muscle cells and the extracellular matrix, indicating potential arterial dysfunction.

Study context

Population
Obstructive patients with hypertrophic cardiomyopathy (HCM) undergoing myectomy, and normal controlsconsecutive patients with HCM undergoing myectomy, plus 9 normal controls, pulse wave velocity in 85 patients with HCM vs 117 age-matched normal controls · HCM patients (biopsy) 101 · normal controls (biopsy) 9 · HCM patients (pulse wave velocity) 85 · age-matched normal controls (pulse wave velocity) 117
Participants
101 patients
Exposure
Aortic wall structure and pulse wave velocity in patients with HCM compared with normal controls
  • HCMn = 101
  • Controlsn = 9
Outcome
Medial lamellar units, interlamellar distance and aortic wall thickness
Methodological detailsconsecutive patients
  • Consecutive patients

Main finding

As reported in the abstract

  • Number of medial lamellar units significantly decreased in HCM, with increased interlamellar distance and aortic wall thickness, compared with controls

Observational data: associations, not effects.

Further findings

Lamellar structure (electron microscopy)

Altered lamellar structure with disorientation of elastin fibers from the circumferential direction

Protein expression

Significant decrease in collagen content, α-smooth muscle actin, smooth muscle myosin, smooth muscle 22 and integrin β1
Significant increase in calponin and caspase-3
Fibulins 1, 2, and 5 showed reduced expression in HCM-aortic biopsies

Pulse wave velocity

Significantly higher in patients with HCM compared with healthy controls
Association between higher pulse wave velocity and more severe molecular and clinical parameters

Original publication

Structural and Functional Characterization of the Aorta in Hypertrophic Obstructive Cardiomyopathy. Circ Heart Fail. Published 23 January 2025. doi:10.1161/circheartfailure.124.012384

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