Exercise capacity reflects airflow limitation rather than hypoxaemia in patients with pulmonary arteriovenous malformations

Gawecki, F; Strangeways, T; Amin, Anjali; Perks, J; McKernan, H; Thurainatnam, S; Rizvi, Abeer; Jackson, JE; Santhirapala, Vatshalan; Myers, Jonathan; Brown, J.; Howard, Luke; Tighe, Hannah; Shovlin, CL

doi:10.1093/qjmed/hcz023

Cited by 6 publications

(14 citation statements)

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“…AVM service at a single centre were examined as described elsewhere [14,15,17,18]. These included arterial oxygen content (CaO 2 ) derived from SpO 2 values measured in the erect posture breathing room air using the established formula: …”

Section: Methodsmentioning

confidence: 99%

“…Previously reported cardiopulmonary exercise tests in patients with pulmonary AVMs where there had been striking variability in anaerobic thresholds [16,17] were reanalysed with a focus on triage/pre-operative assessments methods. Ethical approval had been granted by the National Research Ethics Service (NRES) Committee South West London REC3: 11/H0803 and NRES London Riverside Committee:15/L0/0590.…”

Section: Methodsmentioning

confidence: 99%

“…Written informed consent had been obtained from all participants. Full methodological details are provided in [16,17].…”

Section: Methodsmentioning

confidence: 99%

“…Pulmonary arteriovenous malformations (AVMs) are abnormal vascular connections between pulmonary arteries and veins, resulting in an anatomic right-to-left shunt. [11] Patients with pulmonary AVMs can demonstrate pronounced physiological abnormalities, including significant hypoxaemia, [12-18] increased minute ventilation for given increases in CO 2 production () [16,17], high cardiac output states [19], and often iron deficiency and anaemia due to inadequate replacement of haemorrhagic iron losses from underlying hereditary haemorrhagic telangiectasia (HHT) [20,21] There is no published guidance on management of individuals with pulmonary AVMs or HHT undergoing anaesthesia, and each year, our service receives requests regarding suitability for surgery and insurance, and/or reports that surgery or insurance has been withheld because of the perceived risks of pulmonary AVMs/HHT.…”

Section: Introductionmentioning

confidence: 99%

“…The detailed study aims were to explore which variables may be associated with cardiorespiratory risk defined by the anaerobic threshold in order to inform triage and develop approaches to help guide pre-exposure [23] or pre-operative [1-4,10] management. Having recently applied the VSAQ to observational studies in patients with pulmonary AVMs and HHT, [17,18] we hypothesised that this could prove to be a useful risk categorising tool for triage purposes across wider patient groups.…”

Section: Introductionmentioning

confidence: 99%

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Triage assessment of cardiorespiratory risk status based on measurement of the anaerobic threshold, and estimation by activity limitation in patients with pulmonary arteriovenous malformations and hereditary haemorrhagic telangiectasia

Thurairatnam

Gawecki²,

Strangeways

et al. 2020

Preprint

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words, Text 2729 wordsAll rights reserved. No reuse allowed without permission. What is already known: Alongside age, pre-existing medical conditions are perceived negatively during triage assessments, particularly if rare, and/or theoretically expected to influence cardiorespiratory risk; Anaesthetists use cardiopulmonary exercise testing to categorise patients to higher and lower risk independently to diagnostic labels, but this is not feasible in acute settings; Pulmonary arteriovenous malformations are an exemplar of a condition where, due to expected or measured abnormalities (hypoxaemia-low PaO2 SpO2), poor physiological capacity might be predicted. What this study adds Neither age nor usual SpO2 predicted lower/higher risk categories by anaerobic threshold, but haemoglobin-dependent indices of oxygen delivery to the tissues were associated with higher risk, offering opportunities for improvement by attention to anaemia and aerobic conditioning; Baseline exercise tolerance may override age and diagnostic labels in triage settings: the 13point VSAQ Veterans Specific Activity Questionnaire (VSAQ) is suggested as a rapid screening tool for cardiorespiratory risk assessment.Abstract 1 2 BACKGROUND: Rapid triaging, as in the current COVID-19 pandemic, focuses on age and pre-existing 3 medical conditions. In contrast, preoperative assessments use cardiopulmonary exercise testing (CPET) 4 to categorise patients to higher and lower risk independent of diagnostic labels. Since CPET is not 5 feasible in population-based settings, our aims included evaluation of a triage/screening tool for 6 cardiorespiratory risk. 7 METHODS: CPET-derived anaerobic thresholds were evaluated retrospectively in 26 patients with 8 pulmonary arteriovenous malformations (AVMs) who represent a challenging group to risk-categorise. 9 Pulmonary AVM-induced hypoxaemia secondary to intrapulmonary right-to-left shunts, anaemia from 10 underlying hereditary haemorrhagic telangiectasia and metabolic equivalents derived from the 13-point 11 Veterans Specific Activity Questionnaire (VSAQ) were evaluated as part of routine clinical care. Pre-12 planned analyses evaluated associations and modelling of the anaerobic threshold and patient-specific 13 variables. 14 RESULTS: In the 26 patients (aged 21-77, median 57 years), anaerobic threshold ranged from 7.6-24.5 15 (median 12.35) ml.min -1 kg -1 and placed more than half of the patients (15, 57.7%) in the >11 ml.min -16 1 kg -1 category suggested as "lower-risk" for intra-abdominal surgeries. Neither age nor baseline SpO2 17 predicted anaerobic threshold, or lower/higher risk categories, either alone or in multivariate analyses, 18 despite baseline oxygen saturation (SpO2) ranging from 79 to 99 (median 92)%, haemoglobin from 108 19 to 183 (median 156)g.L -1 . However, lower haemoglobin, and particularly, arterial oxygen content and 20 oxygen pulse were associated with increased cardiorespiratory risk: Modelling a haemoglobin increase 21 of 25g.L -1 placed a further 7/26 (26.9%) patients in a lower ...

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

“…Written informed consent had been obtained from all participants. Full methodological details are provided in [16,17].…”

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Triage assessment of cardiorespiratory risk status based on measurement of the anaerobic threshold, and estimation by activity limitation in patients with pulmonary arteriovenous malformations and hereditary haemorrhagic telangiectasia

Thurairatnam

Gawecki²,

Strangeways

et al. 2020

Preprint

Self Cite

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Pulmonary Arteriovenous Malformations

Shovlin

2022

Encyclopedia of Respiratory Medicine

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High definition analyses of single cohort, whole genome sequencing data provides a direct route to defining sub-phenotypes and personalising medicine

Joyce

Onabanjo

Brownlow

et al. 2021

Preprint

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Possession of a clinical or molecular disease label alters the context in which life-course events operate, but rarely explains the phenotypic variability observed by clinicians. Whole genome sequencing of unselected endothelial vasculopathy patients demonstrated more than a third had rare, likely deleterious variants in clinically-relevant genes unrelated to their vasculopathy (1 in 10 within platelet genes; 1 in 8 within coagulation genes; and 1 in 4 within erythrocyte hemolytic genes). High erythrocyte membrane variant rates paralleled genomic damage and prevalence indices in the general population. In blinded analyses, patients with greater hemorrhagic severity that had been attributed solely to their vasculopathy had more deleterious variants in platelet (Spearman ρ=0.25, p=0.008) and coagulation (Spearman ρ=0.21, p=0.024) genes. We conclude that rare diseases can provide insights for medicine beyond their primary pathophysiology, and propose a framework based on rare variants to inform interpretative approaches to accelerate clinical impact from whole genome sequencing.

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Exercise capacity reflects airflow limitation rather than hypoxaemia in patients with pulmonary arteriovenous malformations

Abstract: CL. Exercise capacity reflects airflow limitation rather than hypoxaemia in patients with pulmonary arteriovenous malformations. QJM. 2019 In Press Exercise capacity reflects airflow limitation rather than hypoxaemia in patients with pulmonary arteriovenous malformations

Cited by 6 publications

References 35 publications

Triage assessment of cardiorespiratory risk status based on measurement of the anaerobic threshold, and estimation by activity limitation in patients with pulmonary arteriovenous malformations and hereditary haemorrhagic telangiectasia

Triage assessment of cardiorespiratory risk status based on measurement of the anaerobic threshold, and estimation by activity limitation in patients with pulmonary arteriovenous malformations and hereditary haemorrhagic telangiectasia

Pulmonary Arteriovenous Malformations

High definition analyses of single cohort, whole genome sequencing data provides a direct route to defining sub-phenotypes and personalising medicine

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