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Impact of cardio-renal syndrome on adverse outcomes in patients with Fabry disease in a long-term follow-up


Siegenthaler, M; Huynh-Do, Uyen; Krayenbuehl, P; Pollock, E; Widmer, U; Debaix, H; Olinger, E; Frank, M; Namdar, M; Ruschitzka, F; Nowak, Albina (2017). Impact of cardio-renal syndrome on adverse outcomes in patients with Fabry disease in a long-term follow-up. International Journal of Cardiology, 249:261-267.

Abstract

Aims: Fabry disease (FD) is a rare X-linked lysosomal storage disease with a deficiency of α-galactosidase A leading to progressive sphingolipid accumulation in different organs, among them heart and kidney. We evaluated theimpactofcardio-renalsyndrome(CRS) ontheincidence of major cardiovascular complications and death in a prospective FD cohort.
Methods and results: A total of 104 genetically proven FD patients were annually followed at the University Hospitals Zurich and Bern. The main outcome was a composite of incident renal replacement therapy (RRT), hospitalisation due to decompensated Heart Failure, new onset atrial fibrillation, pacemaker/ICD implantation, stroke/TIA and death. Estimated glomerular filtration rate (eGFR) and left ventricular myocardial mass index (LVMMI) where ex-plored as the primary exposure variables. During the median follow-up of 103 [59–155] months, events occurred in 27 patients. In a Cox regression analysis, both higher LVMMI and lower eGFR were independently associated with a greater risk of developing adverse events after adjustment for multiple confounders (HR 1.67 [1.04–2.73] P = 0.03 per SD increase in LVMMI, HR 0.45 [0.25–0.83], P = 0.01 per SD decrease in eGFR). In patients with CRS, the risk to develop events was significantly increased if adjusted for demographics and RRT (HR 4.46 [1.07–18.62], P = 0.04), approaching significance if additionally adjusted for hypertension (HR 4.05 [0.95–17.29], P = 0.06). In Kaplan-Meier-Analysis, the poorest event-free survival was observed among patients with CRS. Conclusions: CRS was associated with a high risk to develop cardiovascular complications and death, emphasizing the importance of its prevention and early recognition. A focus on cardio-reno-protective therapies is crucial.

Abstract

Aims: Fabry disease (FD) is a rare X-linked lysosomal storage disease with a deficiency of α-galactosidase A leading to progressive sphingolipid accumulation in different organs, among them heart and kidney. We evaluated theimpactofcardio-renalsyndrome(CRS) ontheincidence of major cardiovascular complications and death in a prospective FD cohort.
Methods and results: A total of 104 genetically proven FD patients were annually followed at the University Hospitals Zurich and Bern. The main outcome was a composite of incident renal replacement therapy (RRT), hospitalisation due to decompensated Heart Failure, new onset atrial fibrillation, pacemaker/ICD implantation, stroke/TIA and death. Estimated glomerular filtration rate (eGFR) and left ventricular myocardial mass index (LVMMI) where ex-plored as the primary exposure variables. During the median follow-up of 103 [59–155] months, events occurred in 27 patients. In a Cox regression analysis, both higher LVMMI and lower eGFR were independently associated with a greater risk of developing adverse events after adjustment for multiple confounders (HR 1.67 [1.04–2.73] P = 0.03 per SD increase in LVMMI, HR 0.45 [0.25–0.83], P = 0.01 per SD decrease in eGFR). In patients with CRS, the risk to develop events was significantly increased if adjusted for demographics and RRT (HR 4.46 [1.07–18.62], P = 0.04), approaching significance if additionally adjusted for hypertension (HR 4.05 [0.95–17.29], P = 0.06). In Kaplan-Meier-Analysis, the poorest event-free survival was observed among patients with CRS. Conclusions: CRS was associated with a high risk to develop cardiovascular complications and death, emphasizing the importance of its prevention and early recognition. A focus on cardio-reno-protective therapies is crucial.

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Contributors:Department of Internal Medicine, Linth Hospital, Uznach, Switzerland, Department of Nephrology, Hypertension and Clinical Pharmacology, University Hospital Bern, Switzerland, Department of Cardiology, Geneva University Hospital, Switzerland
Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic and Policlinic for Internal Medicine
04 Faculty of Medicine > University Hospital Zurich > Clinic for Nephrology
04 Faculty of Medicine > Institute of Physiology
07 Faculty of Science > Institute of Physiology

04 Faculty of Medicine > University Hospital Zurich > Clinic for Cardiology
Dewey Decimal Classification:610 Medicine & health
Uncontrolled Keywords:Cardio-renal syndrome; Fabry disease; Risk factors
Language:English
Date:19 September 2017
Deposited On:16 Oct 2017 14:51
Last Modified:19 Feb 2018 08:50
Publisher:Elsevier
ISSN:0167-5273
Funders:Shire, Genzyme
OA Status:Closed
Publisher DOI:https://doi.org/10.1016/j.ijcard.2017.09.027
Related URLs:http://www.zora.uzh.ch/id/eprint/144742/
PubMed ID:28964554

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Embargo till: 2018-10-01