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The characteristic echocardiographic findings for congenital absence of the pulmonary valve with tetralogy of Fallot were described in three patients, whose diagnoses were confirmed by operation and/or autopsy. The abnormal linear echo, which was thought to be derived from the rudimentary pulmonary valve tissue, was recorded anteriorly to the dense echo from the subpulmonary muscle mass. The echocardiographic signs of right ventricular volume overload were associated with overriding of the aorta.
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