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.