The term ‘primary’ pulmonary hypertension was replaced by ‘idiopathic’ pulmonary hypertension and when there was a genetic basis, ‘familial’ pulmonary hypertension.
Primary hypertension that occurred along with other conditions was referred to as ‘associated’ pulmonary hypertension.
Idiopathic pulmonary arterial hypertension is highly morbid disease characterized by progressive obliteration of precapillary arterioles.
The disease manifested by progressive pulmonary vascular remodeling, compromised pulmonary blood flow and right heart failure.
Idiopathic pulmonary arterial hypertension is rare, occurring in one to two cases per one million people per year, with an overall prevalence estimated at 1300 per one million.
According to World Health Organization (WHO) estimated, there are 5000 patients with Idiopathic pulmonary arterial hypertension in the United States and Europe. However, the prevalence may be increased because of heightened awareness and early recognition in minimally symptomatic patients.
Idiopathic pulmonary arterial hypertension
