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Unusual presentation of rare congenital anomaly: Isolated unilateral absence of a pulmonary artery

Unilateral absence of a pulmonary artery (UAPA) is a rare defect. It may be associated with other congenital cardiovascular malformations which present at an early age. Isolated UAPA (IUAPA), i.e., without any other cardiovascular malformations, usually is asymptomatic and presents in adulthood. In infancy, IUAPA may be suspected by the presence of recurrent respiratory infections and pulmonary hypertension (PHT). Here, we present a child with IUAPA who presented with intermittent cyanosis which is unusual. Echocardiography done showed the presence of severe PHT with a patent foramen ovale (PFO) with a right to left shunt. Detailed echo revealed the absence of right pulmonary artery which was confirmed by a multislice detector computed tomography (MDCT). Infants with unexplained PHT should be screened for the possibility of IUAPA. MDCT scan and magnetic resonance imaging (MRI) can confirm the echocardiographic diagnosis. The treatment plan depends on the presence of symptoms, size of pulmonary artery, and collaterals. Early surgical or hybrid intervention may improve survival. Medical management includes treatment for congestive cardiac failure and pulmonary vasodilators for PHT. Infants with severe PHT are difficult to treat and have poor prognosis.