Unilateral absence of the pulmonary artery in a paediatric cohort.

We present six paediatric patients with unilateral absence of the pulmonary artery in pursuance of rising the clinician's recognition. Unilateral absence of the pulmonary artery was misdiagnosed in one patient by echocardiography. One patient had corrective surgery of a complete atrioventricular septal defect, and another had a right lobectomy. The remaining patients did not undergo any intervention and were asymptomatic at the end of the study period. Although there is a lack of consensus regarding optimal surgical approach, we opted for a more conservative strategy with good results.