Pulmonary artery hypertension Flashcards
What is PAH
restricted blood flow through pulmonary circulation, increased pulmonary pressure, PVR, low flow, high resistance
Normal Mean pulmonary arterial pressure
14+/- 3mmHg
PAH classified by
mPAP>25mmHg at rest and >30 w/ exercise, PCWP
Group 1 PAH
idiopathic, familial, associated with drugs, toxins, congenital heart disease, portal htn, HIV, shistosomiasis, connective tissue disease
Group 2 PAH
left sided hf, valvular dysfunction
Group 3 PAH
respiratory disease, COPD or interstitial lung disease
Group 4 PAH
thromboembolic disease
Group 5 PAH
Miscellaneous
Approved drugs are aimed to target
Group 1
Drugs that can cause PAH
cocaine, amphetamines, fen-fen
3 mechanisms of vascular injury to pulmonary arterioles
vasoconstriction, platelet dysfunction leading to thrombosis, vascular smooth muscle hypertrophy proliferation and hyperplasia
Endothlial dysfunction results from and imbalance mediators
dec nitric oxide synthase, dec prostacyclin, and inc endothelin 1 all lead to vasoconstriction
Non specific clinical features
dyspnea, fatigue, exertional syncope/angina, cx pain, palpitations, peripheral edema
Severe signs of PAH, usually when diagnosed
hepatomegaly, peripheral edema, weight gain from fluid, ascites, JVD
Ways to determine severity
effort tolerance, 6MWT (
Patients who respond to vasodilator test
dec of 10 mmHg from baseline and inc or unchanged CI are considered responders and are eligible for treatment with CCBs
Class 1 PAH
no resulting limitation or physical activity