Case report: Pathological differences in pulmonary arterial hypertension in long-term responders to calcium channel blockers.
| Autor: | Tamura Y; Pulmonary Hypertension Center, International University of Health and Welfare Mita Hospital, Tokyo, Japan.; Department of Cardiology, International University of Health and Welfare, School of Medicine, Chiba, Japan., Lkhagvadorj S; Department of Pathology, International University of Health and Welfare, School of Medicine, Chiba, Japan., Tamura Y; Pulmonary Hypertension Center, International University of Health and Welfare Mita Hospital, Tokyo, Japan., Furukawa A; Pulmonary Hypertension Center, International University of Health and Welfare Mita Hospital, Tokyo, Japan., Aida S; Department of Pathology, International University of Health and Welfare Mita Hospital, Tokyo, Japan., Ebinuma H; Department of Gastroenterology and Hepatology, International University of Health and Welfare, School of Medicine, Chiba, Japan., Shiomi T; Department of Pathology, International University of Health and Welfare, School of Medicine, Chiba, Japan. |
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| Jazyk: | angličtina |
| Zdroj: | Frontiers in cardiovascular medicine [Front Cardiovasc Med] 2023 Nov 02; Vol. 10, pp. 1295718. Date of Electronic Publication: 2023 Nov 02 (Print Publication: 2023). |
| DOI: | 10.3389/fcvm.2023.1295718 |
| Abstrakt: | Background: This study investigates the pulmonary arterial histopathology in patients with idiopathic pulmonary arterial hypertension (IPAH) and acute vasoreactive phenotype, who demonstrated long-term survival (>30 years) and incidental death from causes other than PAH progression. The pathological changes observed in these patients were compared with those in patients with bone morphogenetic protein receptor type 2 (BMPR2) mutation. Case Presentation: We present two cases of patients with pulmonary arterial hypertension (PAH) who died incidentally from causes unrelated to PAH progression. We report compares pulmonary arterial histopathology in long-term survivors of CCB-responsive PAH patient and a hereditary PAH patient with a BMPR2 mutation. Lung specimens were analyzed using the Heath and Edwards (HE) classification and percentage muscular wall thickness (%MWT) of pulmonary arterioles. A significant difference in the severity of grading ( p = 0.0001) and distribution between grades 1-2, 4 ( p = 0.001), and 5 ( p = 0.014) was observed between both patients. These findings suggest differential vascular pathology between the two cases, with CCB responders displaying more mild illness lesions compared to BMPR2 mutant patients. Conclusion: The study revealed that CCB responders exhibit more mild illness vascular lesions than BMPR2 mutant patients despite their long-term survival, suggesting a difference in vascular pathology between the two phenotypes. Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (© 2023 Tamura, Lkhagvadorj, Tamura, Furukawa, Aida, Ebinuma and Shiomi.) |
| Databáze: | MEDLINE |
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