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The resulting perivascular inflammation is now considered to constitute a critical pathomechanism orchestrating remodeling from the outside-in not only in PH associated with disorders of the immune system, such as connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) (15), but also in other forms of PAH (11, 16) as well as in PH due to left heart disease (PH-LHD) (17)