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The debate about revising the definition of pulmonary hypertension (PH) is centered on the benefits and drawbacks of early identification, especially in patients with conditions like connective tissue disease (CTD). The FDA advises that patients should only take drugs with clear benefits, such as improved survival or prevention of noticeable complications. While early identification of group I pulmonary arterial hypertension (PAH) is appealing, the revised hemodynamic definition may not effectively aid early diagnosis. Delays are typically due to failure in recognizing symptoms, not the need for a new definition. Identifying mild precapillary PH often leads to misdiagnosis due to broad differential diagnoses and adds unnecessary costs, psychological distress, and potentially harmful treatments.<br /><br />Dr. Robert P. Frantz argues against the routine use of the revised definition, highlighting the dubious benefits of diagnosing mild PH under the new criteria, the risk of misclassification, and the unintended harm that could arise from over-diagnosis. He advocates for more careful clinical research and advises that patients meeting the revised criteria should be referred to expert centers rather than being treated as standard practice.<br /><br />Conversely, Dr. Bradley A. Maron supports the revised definition, arguing that precise disease definitions have historically facilitated better understanding and management. Although acknowledging the lack of direct therapeutic benefits from the new definition, Maron stresses the importance of correct diagnosis and broadening clinical risk spectrums. Despite the absence of proven treatments, accurate diagnosis remains fundamental to prognosis and management, akin to other complex diseases without definitive cures. He calls for further data to explore therapeutic options under the new criteria, underscoring the need for ongoing clinical research to fully appreciate the revised definition's value.
Keywords
pulmonary hypertension
early identification
connective tissue disease
revised definition
misdiagnosis
clinical research
hemodynamic definition
diagnosis
expert centers
therapeutic options
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