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Polycystic kidney disease — ABIM Board MCQ

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HardNephrologyPolycystic kidney diseaseABIM Board

A 38-year-old with severe precapillary pulmonary hypertension has resting hypoxemia, DLCO 24% predicted, septal lines, centrilobular ground-glass opacities, and mediastinal lymphadenopathy. Pulmonary edema develops shortly after a prostacyclin infusion. Which next strategy is most appropriate?

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Correct answer: C — Refer urgently to a pulmonary-hypertension transplant center for suspected PVOD

The best answer is “Refer urgently to a pulmonary-hypertension transplant center for suspected PVOD”. The combination of severe hypoxemia, profoundly reduced gas transfer, characteristic CT findings, and pulmonary edema after pulmonary-arterial vasodilation strongly suggests pulmonary veno-occlusive disease. PAH therapies can precipitate life-threatening edema because venous outflow remains obstructed, so any use requires extreme caution in an expert center. Lung transplantation is the definitive treatment. This pattern is not diagnostic of chronic thromboembolic disease or left-heart failure.

Reference: Diagnosis and management of pulmonary veno-occlusive disease: https://pubmed.ncbi.nlm.nih.gov/37578057/