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A 35-year-old woman presented with rapidly progressive dyspnoea (WHO functional class III) two months after normal vaginal delivery at term, with no other symptoms. On examination, she was normotensive, with a raised jugular venous pressure, right ventricular (RV) heave, a prominent pulmonary second heart sound and mild pitting leg oedema. Oxygen saturations were 90% on air with a clear chest on auscultation. There was a single enlarged supraclavicular lymph node.
ECG showed sinus tachycardia with right axis deviation. Renal and liver function were normal but brain natriuretic peptide and D-dimer were raised. Chest radiograph showed clear lungs and enlarged proximal pulmonary arteries, with no filling defects or parenchymal disease evident on CT pulmonary angiogram (CTPA). Transthoracic echocardiogram revealed signs of severe pulmonary hypertension (PH) with severely impaired RV function. Right heart catheterisation confirmed precapillary PH with a mean pulmonary artery pressure of 39 mm Hg, pulmonary wedge pressure of 13 mm Hg, cardiac output of 2.1 L/min and pulmonary vascular …