Tags

Type your tag names separated by a space and hit enter

Cardiovascular outcomes in Fabry disease are linked to severity of chronic kidney disease.
Heart. 2015 Feb; 101(4):287-93.H

Abstract

OBJECTIVES

Assess the impact of end-stage renal disease (chronic kidney disease stage 5 (CKD5)) on cardiovascular outcomes in patients with Fabry disease on enzyme replacement therapy.

BACKGROUND

Fabry disease, an X-linked lysosomal storage disease, causes hypertrophic cardiomyopathy and cardiovascular dysfunction.

METHODS

Cardiac and renal function of 25 male patients with Fabry disease were analysed at 0, 1, 2, 5, 7 and 10 years after initiation of treatment. Patients were grouped at baseline into those with CKD5 (n=10) and those without (n=15). ECG and echocardiography were performed 6 and 12 monthly, respectively, while renal function was measured yearly.

RESULTS

After 10 years of treatment, cardiac and renal function in non-CKD5 patients remained unchanged. In contrast, CKD5 was associated with worse baseline cardiac parameters and progressive LV hypertrophy. LV mass index grew by 35.4±31.8 g/m(2.7) in CKD5 versus 5.7±7.9 g/m(2.7), p=0.044 in non-CKD5, predominantly due to increased interventricular septal wall thickness (7.7±5.5 mm vs 1.3±1.7 mm, p=0.003). Cardiovascular events, including sudden death, arrhythmia and pacing device insertion, occurred in 100% patients with CKD5 (21 events) and 26% non-CKD5 patients (7 events), p<0.0001. Additionally, estimated LV filling pressure (E/Ea) was significantly higher in patients having cardiovascular events (21.1±7.7 vs 12.5±4.5, p=0.008) irrespective of renal function.

CONCLUSIONS

End-stage renal disease was the strongest indicator of cardiovascular disease progression in Fabry disease. Enzyme replacement initiated prior to CKD5 was associated with stability in cardiac and renal disease while patients with CKD5 showed ongoing deterioration. Additionally, E/Ea ≥15 may predict risk of cardiac events.

Authors+Show Affiliations

Department of Nephrology, Royal Melbourne Hospital, Melbourne, Victoria, Australia.Department of Cardiology, Royal Melbourne Hospital, Melbourne, Victoria, Australia.Department of Nephrology, Royal Melbourne Hospital, Melbourne, Victoria, Australia Department of Medicine, University of Melbourne, Melbourne, Victoria, Australia.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

25381325

Citation

Talbot, Andrew S., et al. "Cardiovascular Outcomes in Fabry Disease Are Linked to Severity of Chronic Kidney Disease." Heart (British Cardiac Society), vol. 101, no. 4, 2015, pp. 287-93.
Talbot AS, Lewis NT, Nicholls KM. Cardiovascular outcomes in Fabry disease are linked to severity of chronic kidney disease. Heart. 2015;101(4):287-93.
Talbot, A. S., Lewis, N. T., & Nicholls, K. M. (2015). Cardiovascular outcomes in Fabry disease are linked to severity of chronic kidney disease. Heart (British Cardiac Society), 101(4), 287-93. https://doi.org/10.1136/heartjnl-2014-306278
Talbot AS, Lewis NT, Nicholls KM. Cardiovascular Outcomes in Fabry Disease Are Linked to Severity of Chronic Kidney Disease. Heart. 2015;101(4):287-93. PubMed PMID: 25381325.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cardiovascular outcomes in Fabry disease are linked to severity of chronic kidney disease. AU - Talbot,Andrew S, AU - Lewis,Nigel T, AU - Nicholls,Kathy M, Y1 - 2014/11/07/ PY - 2014/11/9/entrez PY - 2014/11/9/pubmed PY - 2015/5/28/medline KW - QUALITY OF CARE AND OUTCOMES KW - RENAL DISEASE SP - 287 EP - 93 JF - Heart (British Cardiac Society) JO - Heart VL - 101 IS - 4 N2 - OBJECTIVES: Assess the impact of end-stage renal disease (chronic kidney disease stage 5 (CKD5)) on cardiovascular outcomes in patients with Fabry disease on enzyme replacement therapy. BACKGROUND: Fabry disease, an X-linked lysosomal storage disease, causes hypertrophic cardiomyopathy and cardiovascular dysfunction. METHODS: Cardiac and renal function of 25 male patients with Fabry disease were analysed at 0, 1, 2, 5, 7 and 10 years after initiation of treatment. Patients were grouped at baseline into those with CKD5 (n=10) and those without (n=15). ECG and echocardiography were performed 6 and 12 monthly, respectively, while renal function was measured yearly. RESULTS: After 10 years of treatment, cardiac and renal function in non-CKD5 patients remained unchanged. In contrast, CKD5 was associated with worse baseline cardiac parameters and progressive LV hypertrophy. LV mass index grew by 35.4±31.8 g/m(2.7) in CKD5 versus 5.7±7.9 g/m(2.7), p=0.044 in non-CKD5, predominantly due to increased interventricular septal wall thickness (7.7±5.5 mm vs 1.3±1.7 mm, p=0.003). Cardiovascular events, including sudden death, arrhythmia and pacing device insertion, occurred in 100% patients with CKD5 (21 events) and 26% non-CKD5 patients (7 events), p<0.0001. Additionally, estimated LV filling pressure (E/Ea) was significantly higher in patients having cardiovascular events (21.1±7.7 vs 12.5±4.5, p=0.008) irrespective of renal function. CONCLUSIONS: End-stage renal disease was the strongest indicator of cardiovascular disease progression in Fabry disease. Enzyme replacement initiated prior to CKD5 was associated with stability in cardiac and renal disease while patients with CKD5 showed ongoing deterioration. Additionally, E/Ea ≥15 may predict risk of cardiac events. SN - 1468-201X UR - https://www.unboundmedicine.com/medline/citation/25381325/Cardiovascular_outcomes_in_Fabry_disease_are_linked_to_severity_of_chronic_kidney_disease_ L2 - http://heart.bmj.com/cgi/pmidlookup?view=long&amp;pmid=25381325 DB - PRIME DP - Unbound Medicine ER -