{Reference Type}: Case Reports
{Title}: Case report: Pathological differences in pulmonary arterial hypertension in long-term responders to calcium channel blockers.
{Author}: Tamura Y;Lkhagvadorj S;Tamura Y;Furukawa A;Aida S;Ebinuma H;Shiomi T;
{Journal}: Front Cardiovasc Med
{Volume}: 10
{Issue}: 0
{Year}: 2023
{Factor}: 5.846
{DOI}: 10.3389/fcvm.2023.1295718
{Abstract}: <B>UNASSIGNED: </B>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.<BR><B>UNASSIGNED: </B>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.<BR><B>UNASSIGNED: </B>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.