DOI: 10.36347/sasjm.2023.v09i06.023

Pages: 675-678

Introduction: Unilateral pulmonary artery agenesis is a rare congenital malformation that can be the source of serious complications. Its diagnosis must be considered in the presence of recurrent respiratory infections. Adult patients are often asymptomatic, which makes the diagnosis difficult. Chest CT angiography is the reference examination. We report a case of agenesis of the right pulmonary artery associated with patent ductus arteriosus revealed at the age of 50 years on a thoracic CT angiography. Observation: A 50-year-old female patient, treated for pulmonary tuberculosis in childhood, presented with exertional dyspnea. Thoracic CT angiography showed agenesis of the right pulmonary artery with patent ductus arteriosus and right pulmonary hypoplasia. There was significant systemic vascularity with evidence of pulmonary hypertension and right heart failure. Discussion: Unilateral pulmonary artery agenesis has been rarely reported in association with a patent ductus arteriosus. Right pulmonary artery agenesis often presents as an isolated, asymptomatic finding without associated congenital heart defects. CT angiography is used to make the diagnosis. Management is often limited to surveillance. Conclusion: Isolated pulmonary artery agenesis is a rare congenital malformation but its true frequency is not well known because of the inherent difficulties in its diagnosis. The main problem remains the risk of serious complications which requires regular follow-up.