Abstract:Right Atrial Isomerism (RAI) and total anomalous pulmonary venous connection (TAPVC) is a rare condition with very high mortality in neonatal period, but outcomes may be better in older children. A cyanotic 3 years old boy with dyspnea and growth retardation diagnosed with RAI and supracardiac type of TAPVC undergone a successful repair of TAPVC and simultaneous Glenn shunt. Older patients with RAI may be a distinct, more suitable group for surgical repair.
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