Vasodilator-induced selective pulmonary oedema in sarcoidosis-associated pulmonary hypertension with pulmonary venous stenosis

A 43-year-old woman was diagnosed with sarcoidosis-associated pulmonary hypertension (SAPH) based on transbronchial lymph node and lung biopsy (figure 1A) and right heart catheterisation. Contrast-enhanced CT revealed no signs of pulmonary embolism. Based on the medical history, laboratory examination and imaging modalities, other potential causes of pulmonary hypertension (PH) were excluded. The diffusion capacity of carbon monoxide was 81% of the predicted value; arterial haemoglobin oxygen saturation was 92% on room air. First, she was prescribed prednisolone (15 mg daily) for 6 months, after which a phosphodiesterase type 5 inhibitor (tadalafil; 40 mg daily) was initiated. Despite treatment, her PH persisted, exhibiting a mean pulmonary artery pressure, 39 mm Hg; cardiac index, 1.9 L/min/m2; pulmonary artery wedge pressure, 4 mm Hg; and pulmonary vascular resistance, 13.0 Wood units. Consequently, an endothelin receptor antagonist (macitentan; 10 mg daily), was added to her regimen. After 4 days of macitentan initiation, her shortness of breath worsened with increased body weight, leading to heart failure. Chest radiography (figure 2A) and CT (figure 2B) revealed selective pulmonary oedema. Contrast-enhanced CT showed pulmonary venous stenosis due to sarcoidosis lesions (figure 2C). Pulmonary angiography confirmed severely localised pulmonary venous stenosis and occlusion (figure …