Abstract
Long-term responders for calcium channel blocker (CCB) therapy represent the minority population among the patients with idiopathic/hereditary/drug-induced pulmonary arterial hypertension (PAH). The frequency of vasoreactive testing (VRT) has been dramatically decreased over the past decade in clinical practice, while the amount of PAH specific therapy prescription has been raised substantially. Current review highlights the frequency of VRT in the modern population of patients with idiopathic PAH. Interconnections between pulmonary vascular morphology, physiology and genetics in long-term responders for CCB therapy and patients with negative VRT are analyzed. Pulmonary vasoreactive reserve prognostic value discussed.
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