Group 3 pulmonary hypertension (PH) is a common complication of chronic lung disease (CLD), including chronic obstructive\npulmonary disease (COPD), interstitial lung disease, and sleep-disordered breathing. Development of PH is associated with poor\nprognosis and may progress to right heart failure, however, in the majority of the patients with CLD, PH is mild to moderate and\nonly a small number of patients develop severe PH. The pathophysiology of PH in CLD is multifactorial and includes hypoxic\npulmonary vasoconstriction, pulmonary vascular remodeling, small vessel destruction, and fibrosis. The effects of PH on the right\nventricle (RV) range between early RVremodeling, hypertrophy, dilatation, and eventual failurewith associated increasedmortality.\nThe golden standard for diagnosis of PH is right heart catheterization, however, evidence of PH can be appreciated on clinical\nexamination, serology, radiological imaging, and Doppler echocardiography. Treatment of PH in CLD focuses on management\nof the underlying lung disorder and hypoxia. There is, however, limited evidence to suggest that PH-specific vasodilators such as\nphosphodiesterase-type 5 inhibitors, endothelin receptor antagonists, and prostanoids may have a role in the treatment of patients\nwith CLD and moderate-to-severe PH.
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