Abstract
The presence of pulmonary vascular disease (PVD) in patients undergoing cardiac transplantation may result in death from acute right ventricular failure. This study was undertaken to explore the frequency and severity of PVD in pediatric patients with cardiomyopathy. We reviewed autopsy records to identify patients with pathologic changes of chronic cardiomyopathy. The pulmonary slides were reviewed by two pathologists and graded using Heath-Edwards criteria for arterial changes; and normal, mild, moderate and severe for changes in the pulmonary veins. A total of 6/16 patients had Heath-Edwards grade 2 or 3 and/or moderate to severe changes in the pulmonary veins (Group I); the remaining 10 had mild or no evidence of PVD (Group II). Group I patients generally had a longer duration of illness (42±53 mo.), but it did not differ significantly from that in Group II (12±33 mo.). The age at diagnosis was significantly greater (P<0.05) in Group I vs. Group II (103±61 vs. 31±49 mo.). Mitral regurgitation was evident at diagnosis in 3/6 patients in Group I vs. 3/10 in Group II. We conclude that moderate to severe pulmonary vascular disease is common in pediatric patients with cardiomyopathy, and its severity cannot be predicted by the clinical course.
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Murphy, A., Witte, D., Bove, K. et al. THE SPECTRUM OF PULMONARY VASCULAR DISEASE IN INFANTS AND CHILDREN WITH CARDIOMYOPATHY. Pediatr Res 21 (Suppl 4), 192 (1987). https://doi.org/10.1203/00006450-198704010-00155
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DOI: https://doi.org/10.1203/00006450-198704010-00155