Comparison of Baseline Characteristics and Survival Between Patients With Idiopathic and Connective Tissue Disease-related Pulmonary Arterial Hypertension

Despite similar functional and hemodynamic severity, patients with CPAH showed a more severe baseline impairment of 6MWT and DLCO and worse overall prognosis than IPAH. Both IPAH and CPAH survival improved in the current era. Nevertheless, CPAH still showed a poorer prognosis than IPAH.