Episode 454: Pulmonary Hypertension

This episode of The Clinical Problem Solvers presents a complex case of a 79-year-old woman with progressive fatigue, shortness of breath, and syncope who was referred to a pulmonary hypertension clinic. She had chronic hypoxemic respiratory failure on 5 liters of oxygen, a history of right heart failure, and was found to have severe pulmonary hypertension with elevated pulmonary artery pressures and normal pulmonary capillary wedge pressure—indicating pre-capillary disease. Despite aggressive management with diuresis and inotropes, her condition deteriorated rapidly. The key diagnostic dilemma arose when she worsened after starting epoprostenol, a pulmonary vasodilator, which is typically used in pulmonary arterial hypertension (PAH). This paradoxical deterioration raised suspicion for pulmonary veno-occlusive disease (PVOD), a rare and often fatal form of pulmonary hypertension that mimics PAH but is contraindicated for vasodilator therapy. The team ultimately concluded that PVOD was the likely diagnosis, supported by her isolated low DLCO, lack of alternative etiology, and clinical trajectory. The episode underscores the critical importance of recognizing PVOD, which can be easily missed due to its similarity to PAH and the risk of life-threatening pulmonary edema from inappropriate vasodilator use.