Disease Classification

PAH disease severity

The WHO Group Classification of Pulmonary Hypertension (Dana Point 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.

WHO Group classification of pulmonary hypertension26

1.1 Idiopathic PAH

1.2 Heritable PAH

1.2.1 BMPR2

1.2.2 ALK1, endoglin (with or without hereditary hemorrhagic telangiectasia)

1.2.3 Unknown

1.3 Drug- and toxin-induced

1.4 Associated with

1.4.1 Connective tissue diseases

1.4.2 HIV infection

1.4.3 Portal hypertension

1.4.4 Congenital heart diseases

1.4.5 Schistosomiasis

1.4.6 Chronic hemolytic anemia

1.5 Persisted pulmonary hypertension of the newborn

New York Heart Association (NYHA) Functional Classification7

NYHA Functional Assessment  
Class I: Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope.  
Class II: Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.  
Class III: Patients with pulmonary hypertension resulting in marked limitation of physical activity. They are comfortable at rest. Less-than-ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.  
Class IV: Patients with pulmonary hypertension with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity.  

Fourth World Symposium on Pulmonary Arterial Hypertension (Dana Point, 2008) updated and modified the Evian Classification based on a new understanding of disease mechanisms.26

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The Ventavis 20 mcg/mL concentration order form is intended for patients who are maintained at the 5 mcg dose and who have repeatedly experienced extended treatment times which could result in incomplete dosing. Ventavis 10 mcg/mL ampules are still available. Ventavis should be taken 6 to 9 times daily, at least 2 hours apart.

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Indication and Important Safety Information

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Indication:

Ventavis 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%).

Important Safety Information

  • Hypotension leading to syncope has been observed; Ventavis should therefore not be initiated in patients with systolic blood pressure less than 85 mmHg.
  • Stop Ventavis immediately if signs of pulmonary edema occur; this may be a sign of pulmonary venous hypertension.
  • Ventavis inhalation may cause bronchospasm and patients with a history of hyperreactive airway disease may be more sensitive.
  • Serious adverse events reported at a rate of less than 3% included congestive heart failure, chest pain, supraventricular tachycardia, dyspnea, peripheral edema, and kidney failure. Vital signs should be monitored while initiating Ventavis.
  • In clinical studies, the most common adverse events occurring more often (≥6%) in Ventavis-treated patients than in patients taking placebo included vasodilation (flushing) (27% vs 9%), cough (39% vs 26%), headache (30% vs 20%), trismus (12% vs 3%), and insomnia (8% vs 2%).
  • Ventavis has the potential to increase the hypotensive effect of vasodilators and antihypertensive agents.
  • Ventavis also has the potential to increase risk of bleeding, particularly in patients maintained on anticoagulants.

Please see full Prescribing Information.