Pathogenesis of the glomerular abnormality in cyanotic congenital heart disease

  • Joseph K Perloff
    Address for reprints: Joseph K. Perloff, MD, Division of Cardiology, Room 47-123, UCLA Center for the Health Sciences, 10833 LeConte Avenue, Los Angeles, California 90095-1679
    Departments of Medicine, Pediatrics, Pathology and Laboratory Medicine, and the Ahmanson Adult Congenital Heart Disease Center, University of California at Los Angeles, Los Angeles, California USA
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  • Harrison Latta
    Departments of Medicine, Pediatrics, Pathology and Laboratory Medicine, and the Ahmanson Adult Congenital Heart Disease Center, University of California at Los Angeles, Los Angeles, California USA
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  • Paola Barsotti
    Dipartimento di Medicina Sperimentale Patologia, Universitá degli Studi di Roma, Rome, Italy
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      We present evidence of 2 distinct glomerular abnormalities in cyanotic congenital heart disease—vascular and nonvascular—each believed to reflect a distinct pathogenesis. Glomeruli from both kidneys were studied with light microscopy in 13 necropsied cyanotic patients and in 8 controls. The vascular study characterized hilar arteriolar dilatation, capillary diameter, glomerular diameter, and capillary engorgement with red blood cells. The nonvascular study characterized juxtaglomerular cellularity, mesangeal cellularity, mesangeal matrix, focal interstitial fibrosis, and megakaryocytic nuclei per cm2 of renal cortex. There was a significant increase in each of the above vascular and nonvascular items of interest relative to controls. Electron microscopy identified whole megakaryocytes with their cytoplasm in glomeruli. The vascular abnormality is believed to result from intraglomerular release of nitric oxide. The nonvascular abnormality is believed to result from platelet-derived growth factor and transforming growth factor- β.
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