Lebensbedrohliche Kardiomyopathie als Folge einer Portkatheterinfektion bei Immuntoleranztherapie

Zusammenfassung

Bei Patienten mit schwerer Hämophilie A stellt die Entwicklung von Inhibitoren eine ernsthafte Komplikation dar, die einer lange dauernden Immuntoleranztherapie (ITT) bedarf. Sie wird meist über Portkatheter durchgeführt. Zu den häufigsten Komplikationen zählen Thrombosen und Infektionen. Wir berichten über einen 18-jährigen Patienten mit schwerer Hämophilie A, der im Alter von fünf Jahren einen hochtitrigen Faktor-VIII-Inhibitor entwickelt hatte. Die folgende ITT erforderte die Implantation eines Portkatheters. Postoperativ kam es zu einer schweren Sepsis mit Herzinsuffizienz. Der Katheter wurde am 26. postoperativen Tag entfernt. Der Patient entwickelte eine dilatative Kardiomyopathie. Die ITT musste beendet und auf eine Bedarfsbehandlung mit einem Prothrombinkomplexkonzentrat umgestellt werden. Die Kardiomyopathie verschlechterte sich allmählich und führte durch unkontrollierbare ventrikuläre Arrhythmien zum Tod des Patienten. Die Frage einer Herztransplantation sollte bei Patienten mit Hämophilie, Kardiomyopathie und Hemmkörpern frühzeitig evaluiert werden, bevor steigende Hemmkörpertiter oder die Entwicklung einer pulmonalen Widerstandserhöhung diese Behandlungsoption verhindern.

Summary

The development of inhibitors in patients with severe haemophilia A is a serious complication requiring long term immune tolerance therapy (ITT). ITT frequently requires implantable central venous access, mostly port catheters. Their use may be complicated by thrombosis and infection. We report on an 18 year old patient with severe haemophilia A who had developed a high-titre factor VIII inhibitor in the age of five years. ITT required the implantation of a port system. The postoperative course was complicated by severe septicaemia with congestive cardiac failure. The port catheter was removed due to recurrent fever after 26 days. Our patient developed dilative cardiomyopathy. ITT had to be stopped and was replaced by on demand therapy with an activated prothrombin complex concentrate. Cardiomyopathy resulted in congestive heart failure, severe ventricular arrhythmias and the death of the young man. In patients with haemophilia, dilative cardiomyopathy and development of inhibitors the possibility of cardiac transplantation should be evaluated before increasing inhibitors and the development of pulmonary hypertension exclude this therapeutical option.

Schlüsselwörter

Hämophilie A - Inhibitor - Portkatheterinfektion - Kardiomyopathie

Keywords

Haemophilia A - inhibitor - port-infection - cardiomyopathy

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