• Bol Asoc Med P R · Jul 2007

    Case Reports

    Uncorrected tetralogy of Fallot with agenesis of the left pulmonary artery in a 70-year-old elderly female.

    • Omar N Nieves and José E López.
    • Cardiology Section, Department of Medicine, University of Puerto Rico School of Medicine.
    • Bol Asoc Med P R. 2007 Jul 1;99(3):226-9.

    AbstractTetralogy of Fallot is the most common cyanotic congenital heart disease in children. It is usually detected in early infancy due to symptoms related to cyanosis and right to left shunting of blood through a large ventricular septal defect. Uncorrected it is associated to a very poor prognosis, with only 3% of adults surviving by age 40 years of age. The combination of tetralogy of Fallot with agenesis of the pulmonary arteries is a rare congenital condition with some reports describing it in 2-3% of patients with Tetralogy of Fallot (ToF). Some rare reports of prolonged survival in patients with uncorrected ToF have been described in the medical literature with some patients living until late adulthood. Here we describe the rare presentation of an elderly female with uncorrected ToF and agenesis of the left pulmonary artery.

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