PAH- therapeutics Flashcards
What is the definition of pulmonary arterial hypertension?
- ) mPAP >= 25 mmHg at rest
2. ) Normal PCWP
WHO groups of pulmonary hypertension
Group 1: Pulmonary arterial hypertension
Group 2: Pulmonary HTN due to left heart disease
Group 3: Pulmonary HTN due to lung diseases and/or hypoxia
Group 4: Chronic thromboembolic pulmonary hypertension
Group 5: Pulmonary hypertension with unclear multifactorial mechanisms
WHO Functional Classes
Patients with PAH in whom there is:
Class 1: NO limitation of usual physical activity; ordinary physical activity does NOT cause increased dyspnea, fatigue, chest pain, or presyncope
Class 2: mild limitation of physical activity; there is NO discomfort at rest, but normal physical activity causes increased dyspnea, fatigue, chest pain, or presyncope
Class 3: marked limitation of physical activity; there is NO discomfort at rest, but less than normal physical activity causes increased dyspnea, fatigue, chest pain, or presyncope
Class 4: unable to perform any physical activity at rest and who may have signs of right ventricular failure; dyspnea and/or fatigue may be present at rest, and symptoms are increased by almost any physical activity
Risk factors for PAH
connective tissue disease, female, HIV,
Gold standard for diagnosis of PAH
right heart catheterization
Acute Vasoreactivity Testing
Evaluating pulmonary vasoreactivity Uses fast-acting, short-duration vasodilators to determine: Extent of smooth muscle constriction Vasodilator response Agents: Epoprostenol Adenosine Nitric oxide***
Positive response:
↓mPAP by at least 10 mmHg
↓mPAP to a value
Non pharm treatment for PAH
Influenza vaccination, sodium restriction…
Supportive therapy for PAH
Warfarin, Furosemide….
If patient is pregnant…
sildenafil…
Patient has low socioeconomic status…
sildenafil…
Patient has chronic stable angina and is currently on a long acting nitrate…
bosenten…
Patient has hepatitis C cirrhosis and elevated LFTs…
sildenafil…
Patient has anemia…
sildenafil…
Dual drug therapy
….
AMBITION Trial
combo therapy is better than monotherapy
Ambrisentan + tadalafil
