Left atrial isomerism in the adolescence: report of two cases
Liu, C.Y.; Chiu, I.S.; Chen, J.J.; Hung, C.R.; Lien, W.P.
Journal of the Formosan Medical Association 90(1): 94-98
1991
ISSN/ISBN: 0929-6646 PMID: 1679116 Document Number: 387086
Atrial isomerism is very rare in adolescence. Two cases of left atrial isomerism are reported here in 2 females, aged 21 and 19 years. They had presented with cyanosis and dyspnea since childhood. High kilovoltage filter films showed a bilateral morphologically left bronchus. Cardiac catheterization in Case 1 revealed normal pulmonary artery pressure, severe subvalvular pulmonic stenosis, a double outlet right ventricle, a significant oxygen step-up at the atrial level and moderate systemic oxygen desaturation; while Case 2 disclosed pulmonary hypertension and mild systemic oxygen desaturation. Both cases had the following anatomical features: ipsilateral connection of pulmonary veins to the bilateral morphological left atrium; interrupted inferior vena cave with azygos or hemiazygos continuation; total anomalous hepatic venous return to the right-sided atrium; complete atrioventricular canal. The diagnoses were confirmed in both cases at surgical correction.