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
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Pulmonary Venous Pathology
Year of Publication: 2019
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients). Studies of Vascular Biology and Mechanism of Disease.
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No