Pulmonary Hypertension Flashcards
What is pulmonary hypertension?
-Pulmonary hypertension is higher than normal blood pressure in the arteries that carry blood away from the heart into the lungs
What is the mean pulmonary artery pressure?
20 mmHg at rest
What is pulmonary arterial hypertension?
Progressive disease involving endothelial function that leads to elevated pulmonary arterial pressure and pulmonary vascular resistance
Causes of pulmonary arterial hypertension
-Genetic
-Drug and toxin exposure
-Disease associated with PAH: CHD, HIV, connective tissue disorders
Treatment for pulmonary arterial hypertension
-Medications specifically for treatment of PAH
-CCB in responders
-Lung transplantation
What is pulmonary arterial wedge pressure?
-Estimates left atrial pressure
-Normal = 4-12 mmHg
-Elevated numbers signal LV failure or mitral stenosis
How is pulmonary vascular resistance calculated?
-Using a formula based on mPAP and PAWP
Negative predictors of pulmonary arterial hypertension
-Advanced functional class
-Poor exercise capacity
-High right atrial pressure
-Right ventricular dysfunction
-Low cardiac output.
Signs and symptoms of pulmonary hypertension
-Shortness of breath
-Fatigue
-Chest pain
-Edema
-Fainting or light-headedness
-Palpitations
What can be used to diagnose pulmonary hypertension?
-Echocardiogram
-Right heart catheterization
-Exercise testing
-Biomarkers
What are the effects of PAH?
-The right side of the heart has difficulty pumping against high pulmonary pressures
-Leads to right ventricular failure
WHO functional class 1
-Symptom-free when physically active or resting
WHO functional class 2
-Slight limitation of physical activity - ordinary activity may cause symptoms
-Comfortable at rest
WHO functional class 3
-Marked limitation in physical activity - less than ordinary activity causes symptoms
-Comfortable at rest
WHO functional class 4
-Significant symptoms with activity
-Symptoms at rest
Goals of therapy for PAH
-Alleviate symptoms
-Improve quality of life
-Prevent or delay disease progression
-Reduce hospitalization
-Improve survival
Pharmacotherapy options for the treatment of PAH
-Calcium channel blockers
-Direct pulmonary vasodilator
-Phosphodiesterase 5 inhibitors
-Endothelin receptor antagonists
-Prostacyclins
-Soluble guanylate cyclase stimulator
When should an oral CCB be used to treat PAH?
After a positive test of acute vasoreactivity
How do you treat PAH patients who are treatment naive with FC 2?
-Combination therapy with tadalafil and ambrisentan
-Monotherapy with either bosentan, macitentan, ambrisentan, riociguat, sildenafil, or tadalafil
How do you treat PAH patients with FC 3 without evidence of rapid disease progression?
-Combination therapy with tadalafil and ambrisentan
-Monotherapy with either bosentan, macitentan, ambrisentan, riociguat, sildenafil, or tadalafil
How do you treat PAH patients with FC 3 with evidence of rapid disease progression?
-Continuous IV epoprostenol, IV treprostinil, or SC treprostinil
-If unable to manage parenteral treatment then give inhaled or oral prostanoid
How do you treat PAH patients with FC 4?
-Continuous IV epoprostenol, IV treprostinil, or SC treprostinil
-If unable to manage parenteral treatment then give inhaled prostanoid in combination with an oral PDE-5 inhibitor and an oral endothelin receptor antagonist
How do you treat PAH patients with FC 3 and 4 with inadequate response to maximal pharmacotherapy?
-Lung transplant
-If not a candidate for lung transplant then put on palliative care
Which drugs target the nitric oxide pathway?
-PDE-5 inhibitors: sildenafil, tadalafil
-Soluble guanylate cyclase stimulator: riociguat
