Tetralogy of Fallot with unilateral absence of the pulmonary artery is a rare congenital cardiac disorder that makes the ipsilateral lung susceptible to opportunistic infections. This disorder adds to the complexity of the case and tests the surgical and decision-making skills of the surgeon. We present an adult male patient with tetralogy of Fallot and absent left pulmonary artery who underwent physiologic exclusion of the left lung for aspergilloma along with intracardiac repair as a single stage procedure.
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