Pulmonary arterial hypertension associated with another disease

Definition

Pulmonary arterial hypertension associated with another disease is a group of conditions that lead to PAH; connective tissue diseases (lupus erythematosus, systemic sclerosis and mixed connective tissues disease), congenital heart disease (Eisenmenger syndrome), HIV infection, portal hypertension, schistosomiasis and chronic hemolytic anemia,which is characterized by elevated pulmonary arterial resistance leading to right heart failure that is progressive and potentially fatal.