Edward C. Kirkpatrick, Michael E. Mitchell, William G. Thilly, Pip Hidestrand, Aoy Tomita-Mitchell, Mats Hidestrand, Elena V. Gostjeva
Children’s Hospital of Wisconsin and Medical College of Wisconsin. Massachusetts Institute of Technology. Eastern Maine Medical Center.
United States
Cardiovascular Pathology
Cardiovasc Pathol 2019; 39: 51-53
DOI: 10.1016/j.carpath.2018.12.005
Abstract
No Abstract Available
Methods:Pulmonary vein tissue from five patients with PVS was collected at the time of surgical intervention. The tissue was fixed in Carnoy solution within 30 min of removal from the living patient. Slide preparation with DNA Feulgen staining was carried out in a manner documented previously [8]. Slides were examined under a light microscope for evidence of bell-shaped nuclei, an identifying property of metakaryotic cells. Patient charts were reviewed for clinical history and outcomes. PVS was defined
Results: Patient characteristics are identified in Table 1. Five patients with PVS had biopsy of the stenotic area when undergoing surgical repair of the stenosis. Median age at diagnosis was 4 months. Three of the five had TAPVR with later restenosis in two patients. One had primary PVS, and one had PVS in association with complex congenital heart disease after single ventricle palliation. Two patients died, likely as a result of progressive PVS (patients 1 and 2). One died due to interstitial lung
Discussion: This appears to be the first report that metakaryotic cells have been observed in PVS specimens. The term “metakaryotic” was coined by Drs. Gostjeva and Thilly as these cells appear to fall between prokaryotic and eukaryotic cells in terms of their nuclear organization and division mechanisms [8]. These primitive stem cells were difficult to detect by standard histology techniques. When first reported in the 19th century, they were denominated as “goblet” or “signet ring” cells and ignored as
Conclusion: We report the finding of metakaryotic cells in biopsy specimens in pediatric patients with PVS. We think that they are the likely precursor to myofibroblasts and then fibroid cells that can be seen in PVS. Separately, we think that the metakaryotic cells also create the smooth muscle cells involved in stenosis. We provide evidence that these cells may reasonably be associated with PVS and could be a target of already-discovered “metakaryocides” in the treatment of PVS.
Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Vascular Cell Biology and Mechanisms of Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
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