Der rechtsventrikuläre Ausflusstrakt bei korrigierter Fallotʼscher Tetralogie: Hämodynamik, Diagnostik und Therapie

Zusammenfassung

Die Langzeitprognose von Patienten mit Fallotʼscher Tetralogie (TOF) wird in erheblichem Ausmaß durch eine bestehende oder sich entwickelnde Pulmonalinsuffizienz bestimmt. In mehr als 50 % der Fälle kommt es bis zur 4. Lebensdekade zu einer pathologischen Dilatation des rechten Ventrikels mit Einschränkung der myokardialen Funktion und ventrikulären Arrhythmien. Im Folgenden sollen hämodynamische sowie diagnostische Modalitäten diskutiert werden. Interventionelle und chirurgische Maßnahmen zur Rekonstruktion des rechtsventrikulären Ausflusstraktes werden besprochen. Der optimale Behandlungszeitpunkt einer Pulmonalinsuffizienz ist derzeit Gegenstand heftiger Diskussionen, zumal die Haltbarkeit von Bioprothesen sehr begrenzt und die Rate der Re-Operationen sehr hoch ist. Die Behandlungsindikation sollte frühzeitig und zu einem Zeitpunkt gestellt werden, noch ehe es zur Entwicklung von Symptomen kommt. Im Falle einer myokardialen Funktionsstörung, einer höhergradigen Trikuspidalinsuffizienz oder im Falle von Arrhythmien ist die Behandlungsindikation in jedem Fall gegeben.

Abstract

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. Today we 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.

Schlüsselwörter

Fallotʼsche Tetralogie - rechter Ventrikel - Ausflusstrakt - Obstruktion - Diagnostik - Hämodynamik - Therapie

Key words

Tetralogy of Fallot - right ventricle - outflow tract - obstruction - diagnostics - haemodynamics - therapy

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