Determinants of exercise limitation in contemporary paediatric Fontan patients with an extra cardiac conduit

: In patients having undergone the Fontan operation, besides the well discussed changes in the cardiac, pulmonary and gastrointestinal system, alterations of further organ systems including the hematologic, immunologic, endocrinological and metabolic are reported. As a medical adjunct to Fontan surgery, the systematic study of the central role of the liver as a metabolizing and synthesizing organ should allow for a better understanding of the pathomechanism underlying the typical problems in Fontan patients, and in this context, the profiling of endocrinological and metabolic patterns might offer a tool for the optimization of Fontan follow-up, targeted monitoring and specific adjunct treatment.

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Cardiopulmonary exercise testing (CPET) in patients with end-stage kidney disease (ESKD): principles, methodology and clinical applications of the optimal tool for exercise tolerance evaluation

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab150 ◽

2021 ◽

Author(s):

Eva Pella ◽

Afroditi Boutou ◽

Aristi Boulmpou ◽

Christodoulos E Papadopoulos ◽

Aikaterini Papagianni ◽

...

Keyword(s):

Kidney Disease ◽

Exercise Testing ◽

Cardiopulmonary Exercise Testing ◽

Exercise Intolerance ◽

End Stage Kidney Disease ◽

Exercise Limitation ◽

Cardiopulmonary Exercise ◽

Functional Reserves ◽

Increased Risk ◽

End Stage

Abstract Chronic kidney disease (CKD), especially end-stage kidney disease (ESKD), is associated with increased risk for cardiovascular events and all-cause mortality. Exercise intolerance as well as reduced cardiovascular reserve are extremely common in patients with CKD. Cardiopulmonary exercise testing (CPET) is a non-invasive, dynamic technique that provides an integrative evaluation of cardiovascular, pulmonary, neuropsychological and metabolic function during maximal or submaximal exercise, allowing the evaluation of functional reserves of these systems. This assessment is based on the principle that system failure typically occurs when the system is under stress and, thus, CPET is currently considered to be the gold-standard for identifying exercise limitation and differentiating its causes. It has been widely used in several medical fields for risk stratification, clinical evaluation and other applications but its use in everyday practice for CKD patients is scarce. This article describes the basic principles and methodology of CPET and provides an overview of important studies that utilized CPET in patients with ESKD, in an effort to increase awareness of CPET capabilities among practicing nephrologists.

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Assessment of Exercise Intolerance in Patients with Pre-Dialysis CKD with Cardiopulmonary Function Testing: Translation to Everyday Practice

American Journal of Nephrology ◽

10.1159/000515384 ◽

2021 ◽

pp. 1-15

Author(s):

Eva Pella ◽

Afroditi Boutou ◽

Marieta P. Theodorakopoulou ◽

Pantelis Sarafidis

Keyword(s):

Cardiovascular Risk ◽

Risk Stratification ◽

Cardiopulmonary Exercise Testing ◽

Adverse Outcomes ◽

Exercise Intolerance ◽

Exercise Limitation ◽

Integrative Evaluation ◽

Prognostic Utility ◽

Risk Potential

Background: Chronic kidney disease (CKD) is often characterized by increased prevalence of cardiovascular risk factors and increased incidence of cardiovascular events and death. Reduced cardiovascular reserve and exercise intolerance are common in patients with CKD and are associated with adverse outcomes. Summary: The gold standard for identifying exercise limitation is cardiopulmonary exercise testing (CPET). CPET provides an integrative evaluation of cardiovascular, pulmonary, hematopoietic, neuropsychological, and metabolic function during maximal or submaximal exercise. It is useful in clinical setting for differentiation of the causes of exercise intolerance, risk stratification, and assessment of response to relevant treatments. A number of recent studies have used CPET in patients with pre-dialysis CKD, aiming to assess the cardiovascular reserve of these individuals, as well as the effect of interventions such as exercise training programs on their functional capacity. This review provides an in-depth description of CPET methodology and an overview of studies that utilized CPET technology to assess cardiovascular reserve in patients with pre-dialysis CKD. Key Messages: CPET can delineate multisystem changes and offer comprehensive phenotyping of factors determining overall cardiovascular risk. Potential clinical applications of CPET in CKD patients range from objective diagnosis of exercise intolerance to preoperative and long-term risk stratification and providing intermediate endpoints for clinical trials. Future studies should delineate the association of CPET indexes, with cardiovascular and respiratory alterations and hard outcomes in CKD patients, to enhance its diagnostic and prognostic utility in this population.

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Exercise limitation in juvenile onset Mixed Connective Tissue Disease (MCTD)

Pediatric Rheumatology ◽

10.1186/1546-0096-6-s1-p162 ◽

2008 ◽

Vol 6 (Suppl 1) ◽

pp. P162 ◽

Cited By ~ 1

Author(s):

JJ Net ◽

A van Royen-Kerkhof ◽

T Takken ◽

B Wissink

Keyword(s):

Connective Tissue ◽

Connective Tissue Disease ◽

Mixed Connective Tissue Disease ◽

Juvenile Onset ◽

Exercise Limitation

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Induction and Increased Risk of Infections in Pediatric Fontan Patients after Heart Transplantation

The Journal of Heart and Lung Transplantation ◽

10.1016/j.healun.2021.01.380 ◽

2021 ◽

Vol 40 (4) ◽

pp. S120

Author(s):

H. Ahmed ◽

J. Lee ◽

D. Bernstein ◽

K. Weinberg ◽

D.N. Rosenthal ◽

...

Keyword(s):

Heart Transplantation ◽

Increased Risk ◽

Fontan Patients

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Myocardial Deformation in Fontan Patients Assessed by Cardiac Magnetic Resonance Feature Tracking: Correlation with Function, Clinical Course, and Biomarkers

Pediatric Cardiology ◽

10.1007/s00246-021-02650-w ◽

2021 ◽

Author(s):

Alessia Callegari ◽

Simona Marcora ◽

Barbara Burkhardt ◽

Michael Voutat ◽

Christian Johannes Kellenberger ◽

...

Keyword(s):

Ventricular Function ◽

Feature Tracking ◽

Heart Diseases ◽

Fontan Operation ◽

Myocardial Deformation ◽

Time Interval ◽

Ventricular Volumes ◽

Atrioventricular Valve Regurgitation ◽

Cine Mr ◽

Fontan Patients

AbstractCardiac MR (CMR) is a standard modality for assessing ventricular function of single ventricles. CMR feature-tracking (CMR-FT) is a novel application enabling strain measurement on cine MR images and is used in patients with congenital heart diseases. We sought to assess the feasibility of CMR-FT in Fontan patients and analyze the correlation between CMR-FT strain values and conventional CMR volumetric parameters, clinical findings, and biomarkers. Global circumferential (GCS) and longitudinal (GLS) strain were retrospectively measured by CMR-FT on Steady-State Free Precession cine images. Data regarding post-operative course at Fontan operation, and medication, exercise capacity, invasive hemodynamics, and blood biomarkers at a time interval ± 6 months from CMR were collected. Forty-seven patients underwent CMR 11 ± 6 years after the Fontan operation; age at CMR was 15 ± 7 years. End-diastolic volume (EDV) of the SV was 93 ± 37 ml/m2, end-systolic volume (ESV) was 46 ± 23 ml/m2, and ejection fraction (EF) was 51 ± 11%. Twenty (42%) patients had a single right ventricle (SRV). In single left ventricle (SLV), GCS was higher (p < 0.001), but GLS was lower (p = 0.04) than in SRV. GCS correlated positively with EDV (p = 0.005), ESV (p < 0.001), and EF (p ≤ 0.0001). GLS correlated positively with EF (p = 0.002), but not with ventricular volumes. Impaired GCS correlated with decreased ventricular function (p = 0.03) and atrioventricular valve regurgitation (p = 0.04) at echocardiography, direct atriopulmonary connection (p = 0.02), post-operative complications (p = 0.05), and presence of a rudimentary ventricle (p = 0.01). A reduced GCS was associated with increased NT-pro-BNP (p = 0.05). Myocardial deformation can be measured by CMR-FT in Fontan patients. SLVs have higher GCS, but lower GLS than SRVs. GCS correlates with ventricular volumes and EF, whereas GLS correlates with EF only. Myocardial deformation shows a relationship with several clinical parameters and NT-pro-BNP.

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