Early recognition and management of pulmonary arterial hypertension: a case for profiling
Abstract
Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure ≥25 mmHg by right heart catheterization and a pulmonary artery wedge pressure ≤15 mmHg, indicating an absence of left ventricular dysfunction. In many patients, PAH is a progressive disease associated with morbidity and often fatal.1 The traditional concept of primary versus secondary pulmonary hypertension has been discarded and per recent classification underlying conditions for pulmonary hypertension are classified into five groups. These include Group 1 PAH and Groups 2, 3 and 4 include categories of pulmonary hypertension secondary to left heart disease, hypoxemia and lung disease and pulmonary hypertension due to chronic thromboembolism, respectively. Group 5 includes pulmonary hypertension secondary to diseases where the pathogenesis is unclear and includes sarcoidosis, myeloproliferative disorders and other miscellaneous conditions.1
A number of conditions with common hemodynamic and pathological features are classified as PAH Group 1. Among these conditions, idiopathic pulmonary arterial hypertension (IPAH - previously known as primary pulmonary hypertension) is a rare disease with poor outcome in the absence of effective therapy.1 Previous data on prognosis of IPAH from the National Institute of Health registry showed a median survival of 2.8 years.2 At the time, the only medications available were calcium channel blockers that are effective only in a small minority of patients.3…
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Evidence Hub reads this as a randomized trial, done in people. Its evidence level is 2 of 5 (High · randomized trial). Randomized trials of a reasonable size are the strongest single-study design.
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