Early recognition of pulmonary arterial hypertension through clinical clues, appropriate initial noninvasive testing, and timely referral to specialized centers is crucial for preventing diagnostic delays and improving patient outcomes.
Pulmonary arterial hypertension (PAH) is a rare but life-threatening condition marked by progressive elevation in pulmonary vascular resistance and right ventricular failure. Because symptoms, most commonly progressive exertional dyspnea, overlap with more common cardiopulmonary disorders, diagnosis is frequently delayed. Primary and acute care clinicians are often the first to evaluate these patients, placing them in a key position to recognize early clues and initiate appropriate evaluation. This review provides practical, evidence-based guidance for the frontline assessment of suspected PAH. Historical features such as exertional dyspnea out of proportion to known disease, unexplained fatigue, syncope, or chest discomfort should raise concern, particularly in patients with connective tissue disease or other risk factors. Initial evaluation involves common tests for evaluating cardiopulmonary complaints: electrocardiography, chest radiography, and measurement of B-type natriuretic peptide (BNP) or N-terminal pro-B-type natriuretic peptide (NT-proBNP). Transthoracic echocardiography is the most helpful initial imaging modality for estimating pulmonary artery pressures and assessing right ventricular structure and function. Definitive diagnosis, however, requires right heart catheterization, preferably at an accredited pulmonary hypertension (PH) center; empiric initiation of PAH-specific therapy is not recommended. By recognizing key clinical features, safeguarding ongoing therapy, and ensuring timely referral to PH centers, frontline clinicians may reduce diagnostic delays and improve outcomes for patients with PAH.
Ahern et al. (Thu,) studied this question.