case_images: PMC10652395_01
case_images: PMC10652395_02
case_images: Comparison of histomorphological measurements. (A) According to the Heath and Edwards classification, the corresponding percentage for each grade (grades 1-6) in two cases is shown. No vascular changes were assessed as grade 6. There was a statistically significant difference in the severity and distribution of grading between cases (p = 0.0001). (B) The Box-and-Whisker diagram indicates the comparison of the percentage media wall thickness (%MWT) between case, with statistically significant differences (p = 0.0006). The box shows the interquartile range, with the median value indicated by the horizontal line; the mean value is indicated by (+); symbols indicate outliers.
case_images: fcvm-10-1295718-g001.jpg
case_images: PMC10652395_02_fcvm-10-1295718-g001.jpg
case_images: F1
case_images: According to the histologic HE classification, a statistically significant difference in the severity of grading (p = 0.0001; Figure 1A) and a distribution difference between grades 1-2, 4 (p = 0.001), and 5 (p = 0.014) were observed between both patients. The mean external diameter was 39.6 +- 8.07 and 32.7 +- 7.5 microm in patients 1 and 2, respectively, and mean medial wall thickness was 6.5 +- 1.7 and 6.7 +- 1.8 microm. %MWT in the BMPR2 mutant (41.6% +- 7.3%) was significantly higher (p = 0.0006) than in the CCB responder (33.3% +- 5.9%) (Figure 1B).
case_images: Histopathological findings of pulmonary arteries and arterioles by the heath and edwards classification (elastin van gienson stain). (A) At low magnification, two muscular arteries surrounding the bronchial tree are seen (marked with a black arrow) in the CCB responder. The remaining lung parenchyma is unremarkable (5x). (B) At high magnification, those pulmonary muscular arteries show medial hypertrophy with intimal cellular proliferation, which is classified as grade 2 (40x). (C) At low magnification, two muscular arteries on the right and left of the bronchiole are dilated and form an angiomatoid lesion in the BMPR2 mutant (5x). (D) At higher magnification, an oblique section of these formations is composed of several thin blood vascular structures having a wall of simple elastic tissue and medial and intimal fibroses, which are classified as grade 5 (20x).
case_images: fcvm-10-1295718-g002.jpg
case_images: PMC10652395_02_fcvm-10-1295718-g002.jpg
case_images: F2
case_images: In the CCB responder, the vessels presented mostly mild illness changes and a predominance of grade 2, showing medial hypertrophy (Figures 2A,B). However, in the BMPR 2 mutant case, an irreversible grade 4 was predominant, along with a worsening grade 5 (Figures 2C,D).