Long-term follow-up of an adult with left pulmonary artery stenting and absent right pulmonary artery: a case report

Unilateral absence of pulmonary artery is a rare congenital abnormality with varied clinical presentations. We present a unique case of congenital absence of right pulmonary artery (RPA) with left pulmonary artery (LPA) origin stenosis without any intracardiac or extracardiac lesion and its long-term follow-up.

A 68-year-old woman underwent successful LPA stenting 23 years back for absent RPA and LPA origin stenosis. She was recently evaluated for breathlessness, almost two decades after the procedure. On evaluation, she was found to have severe pulmonary hypertension (Group 5) without any distortion of the LPA and with normally functioning stent.

This report discusses the long-term outcome of stenting in the setting of severe stenosis of a single pulmonary artery. Over the years, the patient went on to develop severe segmental pulmonary hypertension (Group 5).