title: [A life-threatening cardiomyopathy following port-a-cath infection under immune tolerance therapy].
abstract: 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.
authors.affiliation: Abteilung Kinderkardiologie und pädiatrische Intensivmedizin, Universitätsklinikum Tübingen, Klinik für Kinder- und Jugendmedizin, Hoppe-Seyler-Str. 1, 72076 Tübingen. gesa.wiegand@med.uni-tuebingen.de
authors.forename: G
authors.initials: G
authors.lastname: Wiegand
authors.forename: R
authors.initials: R
authors.lastname: Rauch
authors.forename: M
authors.initials: M
authors.lastname: Girisch
authors.forename: M
authors.initials: M
authors.lastname: Hofbeck
mesh_terms: D000293:Adolescent; D002311:Cardiomyopathy, Dilated / Q000209:etiology*; D002405:Catheterization, Central Venous / Q000009:adverse effects*; D005169:Factor VIII / Q000037:antagonists & inhibitors; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy* / Q000276:immunology*; D006801:Humans; D007167:Immunotherapy / Q000009:adverse effects*
chemical_list: D005169:Factor VIII