Importance of Early Detection and Monitoring for Disease Progression

EP: 1.Pathophysiology of Pulmonary Arterial Hypertension

EP: 2.Differential Diagnosis of Pulmonary Arterial Hypertension

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EP: 3.Importance of Early Detection and Monitoring for Disease Progression

EP: 4.The Importance of Patient-Centered Care

Clinical Presentation and Symptom Recognition

Early symptoms of pulmonary arterial hypertension are typically subtle and nonspecific, leading to frequent diagnostic delays and misdiagnoses. Progressive dyspnea on exertion represents the most common initial symptom, accompanied by fatigue and rapid exhaustion during physical activity. More severe symptoms like syncope and peripheral edema typically occur later in disease progression, often indicating advanced right heart dysfunction and poor prognosis.

The nonspecific nature of early PAH symptoms contributes to diagnostic challenges, with patients often experiencing months or years of evaluation by multiple physicians before receiving an accurate diagnosis. Recent European guidelines have introduced screening algorithms for general practitioners, incorporating ECG and biomarker measurements (BNP or NT-proBNP) to identify patients who warrant echocardiographic evaluation. While these tools lack perfect sensitivity, they provide valuable screening capabilities for symptomatic patients.

Improved diagnostic approaches emphasize the importance of reassessing patients who fail to respond to initial treatments for more common conditions like asthma or COPD. When patients continue experiencing unexplained dyspnea despite standard therapy, further evaluation becomes essential. This systematic approach, combined with increased awareness among healthcare providers, aims to reduce diagnostic delays and facilitate earlier referral to specialized pulmonary hypertension centers.