Disease Classification

PAH disease severity

The WHO Group 1 Classification of Pulmonary Hypertension (Sixth World Symposium on Pulmonary Hypertension version) helps categorize pulmonary arterial hypertension (PAH) by type and the New York Heart Association (NYHA) Functional Assessment measures symptoms severity associated with PAH. Both classification systems are below.

VENTAVIS® (iloprost) Inhalation Solution is indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve a composite endpoint consisting of exercise tolerance, symptoms (NYHA Class), and lack of deterioration. Studies establishing effectiveness included predominantly patients with NYHA Functional Class III-IV symptoms and etiologies of idiopathic or heritable PAH (65%) or PAH associated with connective tissue disease (23%).

WHO Group 1 classification of pulmonary hypertension19

1. Pulmonary Arterial Hypertension

1.1 Idiopathic PAH

1.2 Heritable PAH

1.2.1 BMPR2

1.2.2 ALK-1, ENG, SMAD9, CAV1, KCNK3

1.2.3 Unknown

1.3 Drug and toxin induced

1.4 Associated with:

1.4.1 Connective tissue disease

1.4.2 HIV infection

1.4.3 Portal hypertension

1.4.4 Congenital heart diseases

1.4.5 Schistosomiasis

1′ Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis
1″ Persistent pulmonary hypertension of the newborn (PPHN)

New York Heart Association (NYHA) Functional Classification24

NYHA Functional Assessment  
Functional Class I: No symptoms with ordinary physical activity.  
Functional Class II: Symptoms with ordinary activity. Slight limitation of activity.  
Functional Class III: Symptoms with less than ordinary activity. Marked limitation of activity.  
Functional Class IV: Symptoms with any activity or even at rest.  

The effectiveness of VENTAVIS was established predominantly in patients with NYHA Functional Class III-IV symptoms.