Pulmonary hypertension (PH) is a complex disorder that was first described in a case report as “pulmonary vascular sclerosis” in 1891 by German internist Dr Ernst von Romberg.1  Without treatment, it is a progressive, fatal disease that is characterized by elevated pulmonary arterial pressure and secondary right ventricular failure. According to the most recent evidence from a national French registry, the prevalence of pulmonary arterial hypertension (PAH) is about 15 cases per 1 million adult inhabitants with an incidence of 2.4 cases per 1 million adult inhabitants.2  According to a registry that evaluates early and long-term PAH disease management, the prevalence of PAH in the United States is 12.4 cases per million and the incidence is 2.3 cases per million.3  Unfortunately, not all cases of PAH are reported to the registries, so both the prevalence and incidence data are considered low estimates.

The clinical course of this...

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