Morphology and significance of the left ventricular collagen network in young patients with hypertrophic cardiomyopathy and sudden cardiac death
Jamshid Shirani, MD, FACC*,
Ruth Pick, MD
,
William C. Roberts, MD, FACC
and
Barry J. Maron, MD, FACC
* Departments of Medicine (Division of Cardiology) and Pathology, Albert Einstein College of Medicine, Bronx, New York, USA
Cardiac Morphology Laboratory, Michael Reese Hospital and Medical Center, Chicago, Illinois, USA
Baylor Cardiovascular Institute, Baylor University Medical Center, Dallas, Texas, USA
Minneapolis Heart Institute Foundation, Minneapolis, Minnesota, USA
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Figure 1 Cross section of the heart at mid-left ventricular (LV) cavity level from a 21-year-old patient with HCM showing the site from which tissue blocks were taken from ventricular septum (VS) for histologic examination (inside broken line). RV = right ventricle.
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Figure 2 Ventricular septal tissue sections from a normal control, age 36 years (A and B) and a 31-year-old patient with HCM who died suddenly (C–F). All sections are stained with picrosirius red and viewed at the same magnification (x400) using standard light (left panels) or polarization (right panels) microscopy. A and B: Structurally normal heart. Thin collagen fibers are stained red (A) and appear green-to-yellow under polarized light (B). Various components of the normal collagen matrix are visualized consisting of the perimysial coils (solid arrows), pericellular weaves (open arrows) and intercellular struts (triangles). C and D: HCM in an area of normally arranged and longitudinally sectioned cardiac muscle cells. All components of the collagen matrix are increased in amount and differ morphologically from controls. The perimysial coils (solid arrows) are thickened and appear to be stretched (reduced degree of coiling). Pericellular weaves (open arrows) and struts (triangles) are abundant and thickened. E and F: HCM in an area of disorganized cardiac muscle cells. Perimysial coils (solid arrows), pericellular weaves (open arrows) and struts (triangles) are significantly increased in amount and are also arranged in a markedly disorganized pattern.
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Figure 3 Volume fractions of interstitial, perivascular and total collagen compartments in ventricular septal tissue sections from children and young adults with HCM, compared with controls with structurally normal hearts, patients with systemic hypertension and secondary left ventricular hypertrophy and infants with HCM. Significant differences are present between the groups in total and interstitial, but not perivascular, collagen volume fraction.
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Figure 4 Transversely cut cardiac muscle cells in ventricular septum from a 17-year-old patient with HCM, stained with picrosirius red and viewed by light (A) and polarization (B) microscopy. The myoctes show characteristic encasement within a dense network of matrix collagen. (Magnification x100.)
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Figure 5 High-power view of a transversely cut abnormal intramural coronary artery in a ventricular septal tissue section from a 28-year-old patient with HCM. Picrosirius red stain shows dense perivascular (adventitial) collagen, as well as increased amounts of collagen in the thickened media. (Magnification x100.)
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Figure 6 Low-power view of ventricular septal tissue section from a 20-year-old patient with HCM stained with picrosirius red showing widely distributed interstitial collagen. Volume fraction of interstitial collagen in this patient was 18%. Abnormal intramural coronary arteries with thickened walls are also visible. (Magnification x6.)
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Copyright © 2000 by the American College of Cardiology Foundation.
