Infantile Isolated Absence of Right Pulmonary Artery with an Absent Stump and No Collaterals

Abstract Unilateral absence of pulmonary artery (UAPA) is a rare entity. Diagnosis is often incidental based on echocardiography (ECHO)/computed tomography (CT) findings done for a cardiac evaluation or recurrent pulmonary pathology not responding to conventional treatment. Childhood-onset symptoms are often rare, and most of these cases are reported in adulthood or postpuberty. Here, we present a 5-month-old child with an isolated absence of right pulmonary artery without even an arterial stump or collaterals. Treatment options depend on the severity of symptoms and anatomy of the abnormal vessels, and in some cases, patients get better, if not completely well with the available medical and surgical treatments.