Tetralogy of Fallot with absent pulmonary valve and total anomalous pulmonary venous connection

It has been shown that congenital absence of the pulmonary valve rarely occ urs by itself but tends to be associated with other heart defects, especial ly tetralogy of Fallot. Other malformations and other complex cardiac malfo rmations also have been described in patients with absent pulmonary valve. In this report we describe the findings of a patient with this combination of cardiac defects who survived spontaneously to adulthood.