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Subclinical myocardial disease in patients with primary hyperoxaluria and preserved left ventricular ejection fraction: a two-dimensional speckle-tracking imaging study

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Abstract

Background

Primary hyperoxaluria (PH) is characterized by progressive chronic kidney disease (CKD) and systemic oxalate deposition. Myocardial dysfunction might be present early in the course of the disease. However, this hypothesis has not yet been tested in the PH population. Therefore, we aimed to determine whether strain imaging using two-dimensional speckle tracking echocardiography (2D-STE) might detect subclinical myocardial disease in otherwise asymptomatic PH patients.

Methods

Prospective study of pediatric and adolescent PH patients with preserved LV ejection fraction (LV EF) and without renal replacement therapy. Subjects underwent conventional echocardiography and 2D-STE. Global (GLS) and segmental peak systolic LV longitudinal strain (LS) measurements were obtained. Data were compared with age- and gender-matched controls, and Z-scores were calculated as appropriate.

Results

Fifteen PH patients (age 14.1 ± 5.9 years; 13/15 in CKD stages 1–2) were studied. Although LV EF was preserved (63 ± 6%) in patients, GLS was significantly impaired (GLS − 17.1 ± 2.2% vs − 22.4 ± 1.9%, p < 0.001). This was mainly due to decreased LS values in the apical segments (p < 0.05). Echocardiographic indices of ventricular wall thickness were significantly increased in patients compared to controls (all p < 0.03). GLS correlated significantly with Z-scores of diastolic interventricular wall thickness (r = − 0.57, p = 0.025) and moderately with serum creatinine levels (r = 0.53, p = 0.044). No correlation was found between GLS and blood pressure measurements.

Conclusions

Subclinical myocardial disease is already present early in the course of disease in PH patients with preserved LV EF and some degree of renal dysfunction, but without overt systemic oxalosis. Current recommendations to screen only PH patients with advanced CKD for cardiac disease should be revised accordingly.

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Author notes

  1. Ruth Lagies and Floris E. A. Udink ten Cate contributed equally to this work.

Authors and Affiliations

  1. Department of Pediatric Cardiology, Children’s Hospital, University of Bonn, Adenauerallee 119, 53113, Bonn, Germany

    Ruth Lagies & Ulrike Herberg

  2. Department of Pediatric Cardiology, Heart Center Cologne, University Hospital of Cologne, Cologne, Germany

    Floris E. A. Udink ten Cate & Narayanswami Sreeram

  3. Department of Pediatric Cardiology, Amalia Children’s Hospital, Radboud University Medical Center, Nijmegen, The Netherlands

    Floris E. A. Udink ten Cate

  4. Division of Pediatric Nephrology, Department of Pediatrics, University of Bonn, Bonn, Germany

    Markus Feldkötter & Bernd Hoppe

  5. Institute of Human Genetics, Center for Molecular Medicine, Center for Rare and Hereditary Kidney Disease (ZSEN), University of Cologne, Cologne, Germany

    Bodo B. Beck

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  1. Ruth Lagies
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Lagies, R., Udink ten Cate, F.E.A., Feldkötter, M. et al. Subclinical myocardial disease in patients with primary hyperoxaluria and preserved left ventricular ejection fraction: a two-dimensional speckle-tracking imaging study. Pediatr Nephrol 34, 2591–2600 (2019). https://doi.org/10.1007/s00467-019-04330-7

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