Congenital isolated unilateral hypoplasia of the left pulmonary artery: A rare incidental anomaly in an elderly female

Abstract

Congenital isolated unilateral hypoplasia of the pulmonary artery (CIUPAH) can have late presentation in the adulthood, mostly diagnosed incidentally, as in our case. They may have symptoms such as exertional dyspnea, recurrent lung infections, and hemoptysis. Although the surgical management is a definitive treatment modality, it depends on the presence of reversible pulmonary hypertension and size of the pulmonary arteries; interventional and medical management can be attempted as a palliative therapy in cases where definitive surgical management is not feasible. We have discussed a rare case of isolated unilateral pulmonary artery hypoplasia which is a congenital anomaly of the lung vasculature without associated congenital heart disease manifested in adulthood.

Keywords

• hypoplastic lung
• hypoxia
• pulmonary hypertension
• respiratory distress