Reversible pulmonary artery perfusion abnormalities in the postpartum period as a precursor to the development of pulmonary arterial hypertension

Deterioration, or a new presentation, of pulmonary arterial hypertension (PAH), are recognized complications of pregnancy. In this report, we describe a patient with a family history of PAH who developed peripartum breathlessness and hypoxemia with ventilation-perfusion mismatch but no evidence of thromboembolism or PAH. Significantly reduced perfusion at both lung bases was noted on perfusion scintigraphy and three-dimensional magnetic resonance (3D-MR) perfusion maps in the immediate postpartum period. These abnormalities spontaneously resolved by 16 weeks postpartum, consistent with reversible pulmonary abnormalities of pulmonary perfusion. However, she developed new breathlessness four years later and was found to have developed PAH. This case provides a mechanism which may contribute to the high mortality seen in pregnant patients with PAH in the peripartum period.