Restrictive cardiac phenotype as primary cause of impaired aerobic capacity in Afro-Caribbean patients with val122ile variant transthyretin amyloid cardiomyopathy

Monfort, Astrid; Banydeen, Rishika; Demonière, Fabrice; Courty, Baptiste; Codiat, R.; Nevière, Rémi; Inamo, Jocelyn

doi:10.1080/13506129.2020.1722098

Cited by 10 publications

(19 citation statements)

References 32 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Between-group differences were assessed using t -test and Chi-square for normally distributed data and dichotomized data, respectively. The Mann–Whitney U test was used for non-normally distributed data comparisons [ 16 ]. Receiver-operating curve (ROC) analysis was used to establish cutoffs of relevant variables and their prognostic value and to calculate their area under the curves (AUC).…”

Section: Methodsmentioning

confidence: 99%

Soluble Urokinase Plasminogen Activator Receptor Levels Are Predictive of Covid-19 Severity in Afro-Caribbean Patients

et al. 2022

Self Cite

View full text Add to dashboard Cite

Aim: To investigate association between soluble urokinase plasminogen activator receptor (suPAR) plasma levels at admission and incidence of complications in COVID-19 patients. Patients & methods: We considered Afro-Caribbean patients (n = 64) admitted to the hospital between 1 February 2020 and 28 February 2021. Primary outcome was time from the hospital admission until intensive care unit care or death. Results: Primary outcome (hazard ratio, HR [95%CI]) was associated with higher CT scan severity score (3.18 [1.15–8.78], p = 0.025), National Early Warning Score (NEWS2; 1.43 [1.02–2.02], p = 0.041) and suPAR (1.28 [1.06–2.06], p = 0.041). Kaplan–Meier analysis indicated patients with suPAR level above 8.95 ng/ml had a worse outcome (7.95 [3.33–18.97], p < 0.001). Conclusion: Our study suggests that COVID-19 patients with increased baseline suPAR levels are at a high risk of complications.

show abstract

Section: Methodsmentioning

confidence: 99%

Soluble Urokinase Plasminogen Activator Receptor Levels Are Predictive of Covid-19 Severity in Afro-Caribbean Patients

et al. 2022

Self Cite

View full text Add to dashboard Cite

show abstract

“…Chronotropic incompetence can be objectively demonstrated by the calculation of the slope between heart rate rise and VO 2 increase during exercise. Using this metabolic-chronotropic relationship approach, no evidence of chronotropic incompetence was found in patients with ATTR CA ( 22 ).…”

Section: Characteristics Of Cardiopulmonary Exercise Testing In Cardi...mentioning

confidence: 97%

“…Another mechanism that may contribute to increased V D /V T ratio and V E -VCO 2 slope in patients with CA is the absence of tidal volume V T increase during exercise ( 32 ). While ventilatory reserve at peak exercise in patients with CA is typically unaffected, typical manifestations are rapid shallow breathing patterns characterized by low V T and increased breathing frequency ( 6 , 22 ). The inability to increase O 2 delivery to respiratory muscles, translating into reduced respiratory muscle strength, and enhanced central and peripheral chemoreflexes, may explain the absence of tidal volume V T rise during exercise ( 31 , 32 ).…”

Section: Exertional Ventilatory Inefficiency In Patients With Cardiac...mentioning

confidence: 99%

“…The inability to increase O 2 delivery to respiratory muscles, translating into reduced respiratory muscle strength, and enhanced central and peripheral chemoreflexes, may explain the absence of tidal volume V T rise during exercise ( 31 , 32 ). Accordingly, we have shown that patients with CA commonly display a restrictive lung disease characterized by reduced lung volumes ( 6 , 22 ). In this setting, reduced lung compliance, along with increased elastic loading on respiratory muscles, lung elastic recoil, and stimulated thoracic mechanoreceptors, limit the rise in V T below 50–60% of maximal lung vital capacity, leading to a characteristic shallow breathing pattern with a high respiratory frequency ( 40 ).…”

Section: Exertional Ventilatory Inefficiency In Patients With Cardiac...mentioning

confidence: 99%

See 1 more Smart Citation

Diagnostic and Prognostic Values of Cardiopulmonary Exercise Testing in Cardiac Amyloidosis

Banydeen

Monfort

Inamo

et al. 2022

Front. Cardiovasc. Med.

Self Cite

View full text Add to dashboard Cite

Cardiac amyloidosis (CA) is a myocardial disease characterized by extracellular amyloid infiltration throughout the heart, resulting in increased myocardial stiffness, and restrictive heart wall chamber behavior. Its diagnosis among patients hospitalized for cardiovascular diseases is becoming increasingly frequent, suggesting improved disease awareness, and higher diagnostic capacities. One predominant functional manifestation of patients with CA is exercise intolerance, objectified by reduced peak oxygen uptake (VO2 peak), and assessed by metabolic cart during cardiopulmonary exercise testing (CPET). Hemodynamic adaptation to exercise in patients with CA is characterized by low myocardial contractile reserve and impaired myocardial efficiency. Rapid shallow breathing and hyperventilation, in the absence of ventilatory limitation, are also typically observed in response to exercise. Ventilatory inefficiency is further suggested by an increased VE-VCO2 slope, which has been attributed to excessive sympathoexcitation and a high physiological dead space (VD/VT) ratio during exercise. Growing evidence now suggests that, in addition to well-established biomarker risk models, a reduced VO2 peak is potentially a strong and independent predictive factor of adverse patient outcomes, both for monoclonal immunoglobulin light chain (AL) or transthyretin (ATTR) CA. Besides generating prognostic information, CPET can be used for the evaluation of the impact of therapeutic interventions in patients with CA.

show abstract

“…CA amyloidosis is predominantly considered as a diastolic disease caused by increased myocardial stiffness and restrictive heart wall chamber behavior, along with poor systolic myocardial performance (Wechalekar et al, 2016;Clemmensen et al, 2017;Maurer et al, 2019). Impaired myocardial contractile reserve has also been described using either invasive monitoring or O 2 kinetics-derived parameters (Clemmensen et al, 2017;Monfort et al, 2020). Poor aerobic capacity evaluated by maximal O 2 uptake (VO 2 ) and ventilatory inefficiency suggested by elevated slope of the relationship between ventilation (V E ) and pulmonary CO 2 output (VCO 2 ) are commonly observed in exertional patients with CA (Hein et al, 2018;Yunis et al, 2019;Monfort et al, 2020;Bartolini et al, 2021;Bhutani et al, 2021;Dalia et al, 2021;Banydeen et al, 2022a).…”

Section: Introductionmentioning

confidence: 99%

Determinants of ventilatory inefficiency in transthyretin cardiac amyloidosis: The role of excessive ventilatory drive

Monfort

Thevenet²,

Lacavalerie³

et al. 2022

Front. Physiol.

View full text Add to dashboard Cite

Background and objective: Along with impaired aerobic capacity, increased slope of the relationship between ventilation (VE) and pulmonary CO2 output (VCO2), i.e., VE-VCO2 slope is a common finding in patients with cardiac amyloidosis (CA), which suggests ventilatory inefficiency. Little is known about mechanisms leading to ventilatory inefficiency in CA patients. The purpose of this investigation was to examine the factors that underlie the abnormal ventilatory efficiency in transthyretin hereditary CA patients, such as excessive ventilatory drive, inability of pulmonary blood flow to increase adequately during exercise and excessive sympathetic stimulation, which are known mechanisms of VE-VCO2 slope increase.Methods: In this single-center retrospective observational study, consecutive patients (n = 41) with known familial transthyretin amyloidosis p.Val142Ile mutation carriers with confirmed cardiac phenotype were included.Results: Compared with CA patients without ventilatory inefficiency (VE-VCO2 slope < 36), patients with ventilatory inefficiency (VE-VCO2 slope ≥ 36) had increased inter-ventricular septum thickness, lower VO2 peak along with hyperventilation, and prolonged post-exercise heart rate recovery. By multivariate analysis, only excess of minute-ventilation at anaerobic threshold (β = 0.127; p = 0.011) remained an independent predictor of ventilatory inefficiency.Conclusion: Our data suggest that high ventilatory stimulation during exercise leading to hyperventilation is the main determinant of ventilatory inefficiency in hereditary transthyretin cardiac amyloidosis patients. This novel finding helps to better understand the mechanism of exercise intolerance in these patients where physiological limitation may be related to both heart dysfunction and abnormal pulmonary response.

show abstract

Restrictive cardiac phenotype as primary cause of impaired aerobic capacity in Afro-Caribbean patients with val122ile variant transthyretin amyloid cardiomyopathy

Cited by 10 publications

References 32 publications

Soluble Urokinase Plasminogen Activator Receptor Levels Are Predictive of Covid-19 Severity in Afro-Caribbean Patients

Soluble Urokinase Plasminogen Activator Receptor Levels Are Predictive of Covid-19 Severity in Afro-Caribbean Patients

Diagnostic and Prognostic Values of Cardiopulmonary Exercise Testing in Cardiac Amyloidosis

Determinants of ventilatory inefficiency in transthyretin cardiac amyloidosis: The role of excessive ventilatory drive

Contact Info

Product

Resources

About