INTRODUCTION: Chronic obstructive pulmonary disease is the fourth leading cause of death worldwide, one serious complication being pulmonary hypertension, which occurs in up to 30% of patients and increases mortality drastically. Difficulties in diagnosis and the unclear beneficial effects of PH-specific therapy have hitherto resulted in the absence of approved therapies. Consequently, PH and right heart failure in COPD are still currently treated according to symptoms and not underlying cause Areas covered: This review focuses on the current knowledge of its pathogenesis, clinical picture, diagnosis as well as methods for treatment Expert commentary: Since PH-COPD is an orphan disease with grievous consequences, and diagnosis as well as the right choice of possible treatment is crucial, referral to an expert center in cases of suspicion is necessary. Hitherto there is no officially approved treatment available even though several studies have shown notable improvement in selected individuals, making diagnostics, prognostic markers, and the search for therapeutic agents key issues of interest in this field.
