[Tetralogy of Fallot associated with total anomalous pulmonary venous drainage. Case report (author's transl)]

A case of a 12 year old girl who presented tetralogy of Fallot (T. o F.) associated with Total Anomalous Pulmonary Venous Drainage (TAPVD) is described. This association is extremely rare, only four autopsied cases being reported in literature. TAPVD is associated with complex cyanotic congenital heart diseases in about one third of cases; asplenia or polysplenia are generally present. Double outlet right ventricle (DORV) is the commonest trunco-conal malformation associated with TAPVD; extremely rare is T. o F. Pulmonary outflow tract obstruction can avoid the haemodynamic consequences due to obstruction on the venous channel, or progression to pulmonary hypertension. The association of the two malformations can masquerade the clinical and Xray picture of TAPVD and make difficult the diagnosis during cardiac catheterization.