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B-type natriuretic peptide (BNP) and N-terminal pro b-type natriuretic peptide (NT-proBNP) levels can rise in PAH, reflecting heart strain. Slight elevations might be tolerable but significantly high levels are concerning.

ERAs are a class of drugs that block endothelin receptors to reduce vasoconstriction and slow disease progression in PAH. Monitoring is important due to potential side effects like liver dysfunction.

PAH can be associated with connective tissue diseases like scleroderma. This subtype may require tailored therapeutic approaches and often has a worse prognosis compared to idiopathic PAH.

mPAP is a hemodynamic criterion for PAH diagnosis. PAH is present when mPAP is ≥25 mmHg at rest, measured by right heart catheterization.

The World Health Organization (WHO) classifies PAH based on symptom severity and exercise tolerance from I (no symptoms) to IV (symptoms at rest), which can guide treatment.

Prostacyclin is another vasodilator, and imbalance in its pathways contributes to PAH pathophysiology. Therapies aim to restore its levels or mitigate its dysfunction.

PVR is the resistance that blood must overcome to flow through the pulmonary circulation. Normal PVR ranges from 1.5 to 3 Wood units.

6MWD test measures the distance a patient can walk in 6 minutes, which helps assess the impact of PAH on functional capacity. Normal values are age and sex-dependent, generally around 400-700 meters.

PAH is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. Normal pulmonary artery pressure at rest is 8-20 mmHg.

CO is the volume of blood the heart pumps per minute. CO can decrease in late-stage PAH. Normal CO ranges from 4 to 8 liters per minute.