Pulmonary arterial hypertension involves over 20 identified genetic mutations and requires targeting four distinct treatment pathways including nitric oxide signaling, endothelin receptor antagonism, prostacyclin modulation, and activin signaling inhibition.
Treatment selection now emphasizes individualized approaches that balance disease severity with patient-specific factors including age, comorbidities, functional status, and lifestyle preferences through shared decision-making.
The evolution from traditional three-pathway to four-pathway therapeutic targeting reflects the disease's heterogeneous nature and enables clinicians to maximize treatment efficacy through combination approaches.
Risk stratification tools guide treatment decisions but must be adapted for individual circumstances, as elderly patients with mobility limitations may never achieve low-risk status while young patients may warrant aggressive interventions.
