A 67-year-old man with a long history of combined pulmonary fibrosis and emphysema (CPFE) is diagnosed with World Health Organization (WHO) Group 3 pulmonary hypertension. An echocardiogram shows an estimated right ventricular systolic pressure of 65 mm Hg and signs of right ventricular strain. He is already on long-term oxygen therapy for hypoxemia. He complains of severe dyspnea that limits his daily activities.
What is the primary focus of management for this patient's pulmonary hypertension?
- Initiation of a prostacyclin analog such as epoprostenol.
- Treatment with a phosphodiesterase-5 inhibitor like sildenafil.
- Optimization of management for his underlying lung disease. (correct answer)
- Referral for pulmonary thromboendarterectomy.
Explanation: This patient has WHO Group 3 pulmonary hypertension, which is PH due to chronic lung disease and/or hypoxia. The cornerstone of management for this group is to treat and optimize the underlying lung disease and correct hypoxemia with supplemental oxygen. While targeted pulmonary arterial hypertension (PAH) therapies like prostacyclin analogs or PDE-5 inhibitors are used in Group 1 PAH, their use in Group 3 is controversial and not first-line, as they can worsen ventilation-perfusion mismatch. Pulmonary thromboendarterectomy is the treatment for Group 4 PH (CTEPH), not Group 3.