3.1 Patient demographics and clinical outcomes
Table 1 summarizes the demographics and clinical outcomes of the PAH patients reported in the JADER database from April 2004 to July 2022. Our study population included 1969 patients, 67.7% of which were female, and the median age was approximately 60 years old. In the PAH patients, 402 (20.4%) cases resulted in death from any cause. There were 542 adverse events reported as causes of death of the PAH patients because some patients with two or more adverse events reported in the JADER database were included. Among these adverse events, causes of PAH-related death were as follows: 67 cases of ”pulmonary arterial hypertension (PT code: 10064911)”, 18 cases of ”sudden death (PT code: 10042434)”, 12 cases of ”condition aggravated (PT code: 10010264)”, 70 cases of PTs included in ”heart failures (HLGT code: 10019280)”, and 30 cases of PTs included in ”respiratory failures (excl neonatal) (HLT code: 10052549)”. The treatment distributions of respective pulmonary vasodilators in the dataset for survival analyses (n = 1208) are shown in Figure 1. Epoprostenol (n = 199), selexipag (n = 329), tadalafil (n = 512), and macitentan (n = 538) were the most frequently used drugs in the classes of parenteral prostacyclin, oral drugs targeting the prostacyclin pathway, drugs targeting the NO pathway, and drugs targeting the endothelin pathway, respectively.