作者: Marjorie Barrier , Jolyane Meloche , Maria Helena Jacob , Audrey Courboulin , Steeve Provencher
DOI: 10.1007/S00018-012-0950-4
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摘要: The pathobiology of pulmonary arterial hypertension (PAH) involves a remodeling process in distal arteries, as well vasoconstriction and situ thrombosis, leading to an increase vascular resistance, right heart failure death. Its etiology may be idiopathic, but PAH is also frequently associated with underlying conditions such connective tissue diseases. During the past decade, more than welcome novel therapies have been developed are development, including those increasingly targeting process. These therapeutic options modestly patients' long-term survival, now approaching 60% at 5 years. However, non-invasive tools for confirming diagnosis, assessing disease severity response therapy, tragically lacking would help select best treatment. After exclusion other causes hypertension, final diagnosis still relies on catheterization, invasive technique which cannot repeated often optimal follow-up might require. Similarly, techniques biomarkers used treatment generally lack specificity significant limitations. In this review, imaging current future circulating prognosis, discussed.