Subclinical myocardial disease in patients with primary hyperoxaluria and preserved left ventricular ejection

Ruth Lagies1, Floris E A Udink Ten Cate2,3, Markus Feldkötter4

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

Insights

Subclinical myocardial disease is present early in primary hyperoxaluria (PH) patients, even with preserved heart function. Strain imaging detects this early cardiac dysfunction, suggesting revised screening recommendations for PH patients.

Area of Science:

  • Cardiology
  • Nephrology
  • Medical Imaging

Background:

  • Primary hyperoxaluria (PH) is a genetic disorder causing chronic kidney disease (CKD) and systemic oxalate deposition.
  • Myocardial dysfunction may occur early in PH, but this has not been previously investigated.
  • Early detection of cardiac involvement in PH is crucial for timely intervention.

Purpose of the Study:

  • To investigate subclinical myocardial disease in asymptomatic primary hyperoxaluria patients using 2D speckle tracking echocardiography (2D-STE).
  • To assess if 2D-STE can detect early cardiac dysfunction before overt symptoms manifest.

Main Methods:

  • A prospective study involving pediatric and adolescent PH patients with preserved LV ejection fraction (EF) and no renal replacement therapy.
  • Conventional echocardiography and 2D-STE were performed to measure global (GLS) and segmental longitudinal strain (LS).
  • Data were compared to age- and gender-matched controls, with Z-scores calculated.

Main Results:

  • Fifteen PH patients (mean age 14.1 years) showed significantly impaired GLS (-17.1 ± 2.2%) compared to controls (-22.4 ± 1.9%), despite preserved LV EF (63 ± 6%).
  • Impaired GLS was primarily due to reduced LS in apical segments and correlated with increased ventricular wall thickness and serum creatinine.
  • No correlation was found between GLS and blood pressure.

Conclusions:

  • Subclinical myocardial disease is present early in PH patients, even with preserved LV EF and early CKD.
  • 2D-STE can detect this early cardiac dysfunction in PH.
  • Current screening guidelines for cardiac disease in PH patients may need revision to include those with earlier stages of CKD.
Abstract

Related Concept Videos

Effect of Hepatic Disease on Pharmacokinetics: Active Drug, Metabolite and Fraction of Metabolized Drug01:14

Effect of Hepatic Disease on Pharmacokinetics: Active Drug, Metabolite and Fraction of Metabolized Drug

In pharmacotherapy, monitoring drug concentrations is paramount, especially for drugs whose therapeutic effects hinge on both the active compound and its metabolite. Hepatic impairment profoundly influences drug potency by altering liver function. If the drug is more potent than its metabolite, impaired liver function amplifies drug activity due to elevated drug concentration levels. Conversely, if the metabolite holds greater potency, diminished liver function diminishes drug activity by...
218
Subcellular Fractionation01:32

Subcellular Fractionation

The homogenate obtained after cell lysis contains various membrane-bound organelles that can be further separated into pure fractions by subcellular fractionation. These isolates are used to study specific cellular components, analyze localized protein activity, and are even employed in diagnostics. Fractionation is typically achieved using centrifugation methods, the most common being density-gradient and differential centrifugation.
Differential Centrifugation
Differential centrifugation is...
8.6K
Bioavailability Study Design: Healthy Subjects Versus Patients01:15

Bioavailability Study Design: Healthy Subjects Versus Patients

Bioavailability studies are essential for evaluating a drug's therapeutic efficacy and understanding its absorption patterns under various physiological conditions. Conducting such studies on target patient populations provides more relevant data by simulating real-world disease states. However, practical challenges often necessitate the use of young, healthy adult volunteers as study subjects.Patients may exhibit altered drug absorption patterns due to the effects of the disease itself,...
144
Imaging Studies VII: Vascular Imaging01:19

Imaging Studies VII: Vascular Imaging

DefinitionRenal angiography, also known as renal arteriography, is an imaging technique used to obtain a comprehensive view of blood flow and the vascular structure of blood vessels in the kidneys and surrounding areas.PurposeRenal angiography detects blood vessel abnormalities in the kidneys, such as aneurysms, stenosis, thrombosis, vascular tumors, and renal artery stenosis. It evaluates kidney function and guides interventional treatments like angioplasty or stent placement.Pre-Procedure...
318
Imaging Studies II: Ultrasonography01:24

Imaging Studies II: Ultrasonography

IntroductionUltrasonography, or renal ultrasound, is a noninvasive medical imaging technique that uses high-frequency sound waves to visualize the kidneys, ureters, bladder, and surrounding tissues.Indications for Urinary System UltrasonographyUrinary system ultrasonography is indicated in various clinical scenarios, such as:Kidney Stones (Urolithiasis): To detect and monitor the size and presence of kidney or urinary tract stones.Hydronephrosis: To assess the dilation of the renal pelvis and...
384
Dimensional Analysis03:40

Dimensional Analysis

Dimensional analysis, also known as the factor label method, is a versatile approach for mathematical operations. The main principle behind this approach is: the units of quantities must be subjected to the same mathematical operations as their associated numbers. This method can be applied to computations ranging from simple unit conversions to more complex and multi-step calculations involving several different quantities and their units.
Conversion Factors and Dimensional Analysis
The unit...
59.5K