Challenges in pulmonary hypertension: managing the unexpected

• Main Authors: Karen M. Olsson, Massimiliano Palazzini
• Format: Article
• Language: English
• Published: European Respiratory Society 2015-12-01
• Series: European Respiratory Review
• Online Access: http://err.ersjournals.com/content/24/138/674.full

The diverse challenges associated with diagnosis and management of patients with pulmonary hypertension are illustrated in this case-based review. Case 1 describes a patient diagnosed with pulmonary arterial hypertension (PAH) with right heart failure and active systemic lupus erythematosus who was effectively treated with an up-front triple combination of PAH therapies and immunosuppressive therapy. In case 2, a diagnosis of pulmonary veno-occlusive disease was reached after a combined approach of clinical suspicion, physical examination, and invasive and noninvasive tests. Cautious PAH therapy and high-dose diuretics provided clinical benefit in this patient and served as a bridge to lung transplantation. These cases highlight the need for ongoing follow-up of patients with PAH, comprising frequent assessment of treatment success and continued diagnostic evaluation.