PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要：
■这项研究调查了特发性肺动脉高压（IPAH）和急性血管反应性表型患者的肺动脉组织病理学，显示长期生存（>30年）和因PAH进展以外的其他原因导致的意外死亡。将这些患者的病理变化与骨形态发生蛋白受体2型（BMPR2）突变患者的病理变化进行比较。
■我们介绍了2例肺动脉高压（PAH）患者，其偶然死于与PAH进展无关的原因。我们报告比较了CCB反应性PAH患者和具有BMPR2突变的遗传性PAH患者的长期幸存者的肺动脉组织病理学。使用Heath和Edwards（HE）分类和肺小动脉的肌壁厚度百分比（％MWT）分析肺标本。分级的严重程度（p=0.0001）和1-2、4级之间的分布（p=0.001）存在显着差异，在两名患者之间观察到5（p=0.014）。这些发现表明两种病例之间有区别的血管病理学，与BMPR2突变患者相比，CCB应答者表现出更轻微的疾病病变。
■研究表明，CCB反应者比BMPR2突变患者表现出更轻微的疾病血管病变，尽管他们长期存活，提示两种表型之间的血管病理学差异。
■我们介绍了2例肺动脉高压（PAH）患者，其偶然死于与PAH进展无关的原因。我们报告比较了CCB反应性PAH患者和具有BMPR2突变的遗传性PAH患者的长期幸存者的肺动脉组织病理学。使用Heath和Edwards（HE）分类和肺小动脉的肌壁厚度百分比（％MWT）分析肺标本。分级的严重程度（p=0.0001）和1-2、4级之间的分布（p=0.001）存在显着差异，在两名患者之间观察到5（p=0.014）。这些发现表明两种病例之间有区别的血管病理学，与BMPR2突变患者相比，CCB应答者表现出更轻微的疾病病变。
■研究表明，CCB反应者比BMPR2突变患者表现出更轻微的疾病血管病变，尽管他们长期存活，提示两种表型之间的血管病理学差异。
