The Growing Burden of Pulmonary Hypertension in the Modern Era
A Zebra No More?
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See Article by Wijeratne et al.
Pulmonary hypertension (PH) is a heterogeneous syndrome characterized by elevated pulmonary pressures. Although often suspected based on symptoms of dyspnea and elevated estimates of pulmonary arterial systolic pressures on echocardiogram, PH can only be diagnosed by right heart catheterization demonstrating a mean pulmonary artery pressure ≥25 mm Hg. Clinically, PH can result from various comorbid conditions, including underlying cardiac, pulmonary, thromboembolic, and miscellaneous diseases. PH in the absence of these comorbid conditions constitutes a small subset of PH, known as pulmonary arterial hypertension (PAH).
Over the last several years, determining the incidence and prevalence of PH in population-based studies has proven challenging, in part, because of the lack of specificity of diagnosis codes commonly used in reimbursement system databases. These codes do not align with the current classification system and thus are unable to accurately distinguish disease phenotypes. In addition, proper classification of PH, according to guideline recommendations, requires not only a high clinical suspicion but also extensive targeted testing, which is often not completed in clinical practice.1 As shown in prior studies, (1) only a minority (<6%) of patients with a diagnosis of PAH complete the entire recommended testing algorithm and (2) nearly one third of patients referred to a tertiary center for PH have a change in their diagnosis after completing the entire workup.2,3 Thus, the clinical diagnosis of PH at both the …