Zusammenfassung
GRUNDLAGEN: Ägyptische Patienten mit endomyokardialer Fibrose durch Schistosoma mansoni haben oft eine ausgeprägte rechtsventrikuläre Erkrankung, periportale Fibrose und Entzündung der Pulmonalarterien. Hier wollen wir unsere Erfahrung zur chirurgischen Therapie der schistosomalen endomyokardialen Fibrose darstellen. METHODIK: Fallberichte zu zwei Patienten. ERGEBNISSE: Zwei Personen, ein 61-jähriger Mann und eine 24-jährige Frau, mit rechtsseitiger endomyokardialer Fibrose mit rechtsventrikulärer Endokardiektomie (unter kardiopulmonalem Bypass) verstarben nach dem Eingriff an Rechtsherzversagen. SCHLUSSFOLGERUNGEN: Die schistosomale endomyokardiale Fibrose bietet oft ein schlechtes chirurgisches Ergebnis. Die kombinierte Herz/Lungen-Transplantation wäre möglicherweise eine bessere Therapieoption bei dieser Erkrankung.
Summary
BACKGROUND: Egyptian patients with endomyocardial fibrosis which are usually caused by Schistosoma mansoni present with the peculiar clinical picture of right ventricular disease, periportal fibrosis and pulmonary arteritis. The purpose of this report is to highlight our experience in the surgical treatment of the schistosomal endomyocardial fibrosis. METHODS: Two patients who had classical features of 'Egyptian type' endomyocardial fibrosis who underwent surgical endocardiectomy are discussed. RESULTS: Two patients, a 61-year-old man and a 24-year-old woman, are described who presented with features of right-sided endomyocardial fibrosis who had right ventricular endocardiectomy carried out under cardiopulmonary bypass. The two patients died several days after right ventricular endocardiectomy due to severe right ventricular failure and low cardiac output syndrome. CONCLUSIONS: Schistosomal ('Egyptian type') endomyocardial fibrosis is associated with a poor surgical outcome. These patients may be candidates for the daunting surgical option of combined heart-lung and liver transplantation and lifelong immunosuppressive medication.
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Onakpoya, U., El-Eshmawy, A. & Khadragui, I. Outcome of endocardiectomy in "Egyptian type" endomyocardial fibrosis: a report of two patients. Eur Surg 42, 241–244 (2010). https://doi.org/10.1007/s10353-010-0562-x
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DOI: https://doi.org/10.1007/s10353-010-0562-x