Unilateral left lung agenesis with crossed-ectopic right lower lobe combined tricuspid atresia diagnosed by ECG gated computed tomography

Abstract

Young in-fants with this condition may face a catastrophic clinicalcourse. We report an unusual case of lung agenesis withcomplexcardiacanomaliesand crossedectopiclungcompli-cating diagnosis in a 6-month-old child.CLINICAL SUMMARYAboywasbornuneventfullytoahealthymotheratagesta-tionalageof37weeks.Lowbirthweight(1700g)andmultiplecongenital anomalies were found at birth, including large an-terior fontanel and syndactyly over the second and third toes.Because tachypnea and heart murmur were heard 3 days afterbirth, serial workup was arranged. Chest radiography showedleft lung haziness (Figure 1), and echocardiography showedtricuspid atresia, small ventricular septal defect, atrial septaldefect, large patent ductus arteriosus, and suspected left pul-monary artery and pulmonary vein agenesis (Figure 2, A andB). Because ofthe suspected left lung agenesis, electrocardio-graphically gated chest computed tomography and 3-dimen-sional reconstruction were performed (Figure 2, C and D,andVideo1).Crossedectopicrightlowerlobeinthelefthemi-thoraxwasfoundincidentally,inadditiontoleftlungagenesisandtricuspidatresia.Thearteriesandveinssupplyingnotonlythislobebut alsothe bronchusofthislobewerefromthe rightlung.Thepatient’srespiratorydistressandheartfailuresymp-toms improved after anticongestive medication, and he wasthen discharged at 2 weeks old.When the boy was 5 months old, mean pulmonary arterypressure was measured as 10 mm Hg by cardiac catheteriza-tion. We then performed a staged operation including bidi-rectional Glenn shunt, atrial septal defect enlargement, andpatent ductus arteriosus ligation for his tricuspid atresia.The postoperative course was uneventful, and the patientwas discharged 10 days after the operation and followedup at our cardiology clinics. Total cavopulmonary circula-tion will be arranged in the future.DISCUSSIONLung agenesis is a rare congenital anomaly that may beassociated with multiple congenital anomalies.