The Right Ventricular Outlow Tract in Tetralogy of Fallot: Haemodynamics, Diagnostics and Therapy

The long-term outcome of patients with Tetralogy of Fallot (TOF) with reconstruction of the right ventricular outflow tract is often complicated by the sequelae of severe pulmonary regurgitation. Progressive enlargement of the right ventricle, biventricular dysfunction and arrhythmia are apparent in more than 50% of the patients in the fourth decade of life. Pathophysiologic implications, clinical assessment and diagnostic modalities are discussed, whereas CMR imaging seems to be the procedure of choice. Therapeutic options for re-reconstruction of the right ventricular outflow-tract are mentioned, surgical and interventional procedures are explained in detail. The optimal timing of re-operation for significant pulmonary regurgitation after TOF-repair is still a matter of controversy given the limited runtime of the lately implanted prostheses and the risk of further reoperation. Early surgery is recommended in these patients, before symptoms develop or RV-function has declined. Todaywe believe that waiting for the patient to become symptomatic is too late. Pulmonary valve replacement is at least indicated in patients developing symptoms due to severe pulmonary regurgitation, particularly if associated with substantial or progressive right ventricular dilatation, tricuspid regurgitation, and/or arrhythmias.