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Rofo 2011; 183(11): 1037-1042
DOI: 10.1055/s-0031-1281744
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© Georg Thieme Verlag KG Stuttgart · New York

Right Ventricular Involvement in Patients with Fabry’s Disease and the Effect of Enzyme Replacement Therapy

Rechtsventrikuläre Beteiligung bei Morbus Fabry und der Effekt der EnzymersatztherapieW. Wuest1 , W. Machann2 , F. Breunig3 , F. Weidemann3 , H. Koestler2 , D. Hahn2 , C. Wanner3 , M. Beer2
  • 1Radiologisches Institut, Universitätsklinikum Erlangen
  • 2Institut für Röntgendiagnostik, Universitätsklinikum Würzburg
  • 3Medizinische Klinik I, Universitätsklinikum Würzburg
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Publication History

received: 14.2.2011

accepted: 9.8.2011

Publication Date:
28 September 2011 (online)

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Zusammenfassung

Ziel: Laut Echokardiografie betrifft der Morbus Fabry nicht nur den linken Ventrikel (LV), sondern auch den rechten Ventrikel (RV). Bis heute ist die Auswirkung der Enzymersatztherapie (ERT) auf den rechten Ventrikel mittels MRT noch nicht untersucht worden. Material und Methoden: In dieser prospektiven Studie wurden vierzehn Patienten mit genetisch gesichertem Morbus Fabry vor und nach 13 ± 1 Monaten ERT mittels 1,5 T MRT-Gerät untersucht. Alle Patienten wurden mittels Herz-MRT untersucht und die rechts- und linksventrikuläre Morphologie und die Funktionsparameter wurden ausgewertet. Ergebnisse: Der Ausgangswert für die RV Masse betrug 31 ± 6 g/m2, enddiastolisches Volumen (EDV) 88 ± 13 ml/m2, endsystolisches Volumen (ESV) 39 ± 9 ml/m2, Schlagvolumen (SV) 49 ± 7 ml/m2 und die Ejektionsfraktion (EF) 56 ± 5 %. Die rechtsventrikuläre Masse und das EDV verringerten sich signifikant nach 13 ± 1 Monaten Therapie (Masse 27 ± 7 g/m2, p < 0,05, EDV 76 ± 24 ml/m2, p < 0,05) ohne signifikante Veränderung des ESV (33 ± 13 ml/m2), SV (43 ± 12 ml/m2) und der EF (57 ± 7 %). Die LV Masse (102 ± 26 g/m2 vs. 94 ± 27 g/m2, p < 0,05), das EDV (76 ± 13 ml/m2 vs. 66 ± 22 ml/m2, p < 0,05) und ESV (29 ± 9 ml/m2 vs. 23 ± 9 ml/m2, p < 0,05) verringerten sich signifikant, während sich die EF (64 ± 7 % vs. 66 ± 5 %, p < 0,05) signifikant erhöhte. Schlussfolgerung: Neben dem bekannten Einfluss der ERT auf den linken Ventrikel verbessert die ERT die rechtsventrikuläre Masse und das EDV.

Abstract

Purpose: According to echocardiography reports, Fabry cardiomyopathy not only affects the left ventricle (LV) but also the right ventricle (RV). Until now no MRI studies about the effect of enzyme replacement therapy (ERT) on the RV are available. We evaluated the effect of ERT on the RV. Materials and Methods: In this prospective trial 14 patients with genetically proven Fabry’s disease were examined using a 1.5 T MR scanner before ERT and after 13 ± 1 months of ERT. All patients underwent cardiac MR imaging and the RV/LV cardiac morphology and function were analyzed. Results: At baseline examination the values were as follows: RV mass 31 ± 6 g/m2, end-diastolic volume (EDV) 88 ± 13 ml/m2, end-systolic volume (ESV) 39 ± 9 ml/m2, stroke volume (SV) 49 ± 7 ml/m2 and ejection fraction (EF) 56 ± 5 %. The RV mass and EDV decreased significantly after 13 ± 1 months on ERT (mass 27 ± 7 g/m2, p < 0.05, EDV 76 ± 24 ml/m2, p < 0.05), with no significant change of ESV (33 ± 13 ml/m2), SV (43 ± 12 ml/m2) and EF (57 ± 7 %). The LV mass (102 ± 26 g/m2 vs. 94 ± 27 g/m2, p < 0.05), EDV (76 ± 13 ml/m2 vs. 66 ± 22 ml/m2, p < 0.05) and ESV (29 ± 9 ml/m2 vs. 23 ± 9 ml/m2, p < 0.05) decreased significantly while the EF (64 ± 7 % vs. 66 ± 5 %; p < 0.05) increased significantly. Conclusion: Besides the known beneficial effect on the LV, ERT improves RV mass and EDV.

Key words

cardiac - MR imaging - metabolic Disorders

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Prof. Dr. Meinrad Beer

Institut für Röntgendiagnostik, Universitätsklinikum Würzburg

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