• Cardiac risk
• Cardiovascular disease
• Physical capacity
• Physical fitness
• Physical function

• Humans
• Male
• Female
• Middle Aged
• Sensitivity and Specificity
• Aged
• Retrospective Studies
• Cardiovascular Diseases/mortality
• Risk Assessment
• Oxygen Consumption/physiology
• Reproducibility of Results
• Exercise Test
• Prognosis
• Survival Rate
• Heart Disease Risk Factors
• Risk Factors
• Predictive Value of Tests

• exercise pulmonary hypertension
• invasive cardiopulmonary exercise testing
• pulmonary arterial hypertension
• pulmonary vascular disease

• Cardiopulmonary exercise test
• Congenital heart disease-related pulmonary arterial hypertension
• Echocardiography
• Right ventricle-pulmonary arterial coupling

• Humans
• Female
• Male
• Exercise Test
• Heart Defects, Congenital/complications
• Heart Defects, Congenital/physiopathology
• Heart Defects, Congenital/diagnostic imaging
• Echocardiography
• Adult
• ROC Curve
• Exercise Tolerance/physiology
• Pulmonary Arterial Hypertension/physiopathology
• Pulmonary Arterial Hypertension/diagnostic imaging
• Oxygen Consumption
• Middle Aged
• Young Adult
• Hypertension, Pulmonary/physiopathology
• Hypertension, Pulmonary/diagnostic imaging
• Pulmonary Artery/physiopathology
• Pulmonary Artery/diagnostic imaging

• 82371963 National Natural Science Foundation of China
• 2021A1515012232 Guangdong Basic and Applied Basic Research Foundation
• 8197070830 NSFC lauching fund of the Guangdong Provincial People's Hospital
• B2023010 Guangdong Medical Science and Technology Research Foundation
• 2024A04J5038 Guangzhou Basic and Applied Basic Research Special Youth Doctoral "Setting Sail" Project
• NSFC lauching fund of the Guangdong Provincial People’s Hospital
• Guangzhou Basic and Applied Basic Research Special Youth Doctoral “Setting Sail” Project

• Exercise training
• Functional capacity
• Peak VO2
• Pulmonary hypertension
• Quality of life

• Gas exchange
• cardiological diseases
• cardiopulmonary exercise test
• exercise
• respiratory diseases
• respiratory pathophysiology
• respiratory physiology
• ventilatory efficiency

• Humans
• Exercise Test
• Exercise Tolerance
• Pulmonary Gas Exchange/physiology
• Pulmonary Ventilation/physiology
• Exercise/physiology
• Prognosis
• Lung/physiopathology

• exercise tolerance
• magnetic resonance imaging
• oxygen consumption
• pulmonary arterial hypertension
• right ventricle
• treatment outcome

• Humans
• Exercise Tolerance/drug effects
• Male
• Female
• Ventricular Function, Right/drug effects
• Middle Aged
• Pulmonary Arterial Hypertension/drug therapy
• Pulmonary Arterial Hypertension/physiopathology
• Adult
• Treatment Outcome
• Exercise Test
• Recombinant Fusion Proteins/therapeutic use
• Aged
• Hypertension, Pulmonary/drug therapy
• Hypertension, Pulmonary/physiopathology
• Oxygen Consumption/drug effects
• Walk Test
• Activin Receptors, Type II/therapeutic use
• Recovery of Function

• Humans
• Pulmonary Arterial Hypertension/diagnosis
• Exercise Test
• Quality of Life
• Hypertension, Pulmonary/diagnosis
• Exercise Tolerance

• Humans
• Cystic Fibrosis/diagnosis
• Exercise Test
• Prognosis
• Forced Expiratory Volume
• Exercise

• R01 NR020470 NINR NIH HHS
• T32 HL007287 NHLBI NIH HHS
• R01NR020470 NINR NIH HHS
• National Institute of Nursing Research
• Cystic Fibrosis Foundation

• connective tissue disease
• pulmonary arterial hypertension
• pulmonary hypertension
• scleroderma
• systemic lupus erythematosus

• cardiopulmonary exercise test
• prognosis
• pulmonary hypertension

• PAH prognosis
• lung transplantation
• pulmonary arterial hypertension
• risk stratification

• Duke Activity Status Index
• cardiopulmonary exercise testing
• exercise capacity
• pulmonary arterial hypertension

• Humans
• Hypertension, Pulmonary/diagnosis
• Hypertension, Pulmonary/therapy
• Evidence-Based Medicine
• Algorithms

• 205188/Z/16/Z Wellcome Trust

• 4D flow CMR
• Cardiopulmonary exercise test
• Flow components
• Kinetic energy
• Pulmonary arterial hypertension

• Humans
• Pulmonary Arterial Hypertension/diagnostic imaging
• Predictive Value of Tests
• Heart Ventricles
• Biomarkers
• Ventricular Remodeling
• Magnetic Resonance Spectroscopy

• National Medical Research Council of Singapore

• Balloon pulmonary angioplasty
• Chronic thrombo-embolic pulmonary hypertension
• Congenital heart disease
• Connective tissue disease
• Endothelin receptor antagonists
• Guidelines
• Left heart disease
• Lung disease
• Lung transplantation
• Phosphodiesterase type 5 inhibitors
• Prostacyclin analogues
• Prostacyclin receptor agonists
• Pulmonary arterial hypertension
• Pulmonary endarterectomy
• Pulmonary hypertension
• Soluble guanylate cyclase stimulators

• Endothelin Receptor Antagonists
• Humans
• Hypertension, Pulmonary/diagnosis
• Hypertension, Pulmonary/therapy

• 6‐min walking test
• cardiopulmonary exercise test
• pulmonary arterial hypertension
• risk assessment

• cardiopulmonary exercise testing
• echocardiography
• pulmonary arterial hypertension
• risk prediction
• survival

• all-cause mortality
• balloon pulmonary angioplasty
• cardiopulmonary exercise testing
• chronic thromboembolic pulmonary disease
• chronic thromboembolic pulmonary hypertension
• comorbidities
• lung function
• prognosis
• pulmonary endarterectomy
• therapy

• Chronic thromboembolic pulmonary hypertension
• Follow-up
• Functional impairment
• Pulmonary embolism

• Acute Disease
• Adult
• Chronic Disease
• Female
• Humans
• Hypertension, Pulmonary/complications
• Hypertension, Pulmonary/epidemiology
• Male
• Middle Aged
• Prospective Studies
• Pulmonary Embolism/complications
• Pulmonary Embolism/diagnosis
• Pulmonary Embolism/epidemiology
• Quality of Life
• Risk Factors

• DLCO
• VO2
• long-covid
• maximal oxygen consumption
• pulmonary function
• pulmonary vascular

Pulmonary hypertension in left heart disease (PH-LHD), which includes combined post- and precapillary PH (Cpc-PH) and isolated postcapillary PH (Ipc-PH), differs significantly in prognosis. We aimed to assess whether cardiopulmonary exercise testing (CPET) predicts the long-term survival of patients with PH-LHD.

A single-center observational cohort enrolled 89 patients with PH-LHD who had undergone right heart catherization and CPET (mean pulmonary arterial pressure > 20 mm Hg and pulmonary artery wedge pressure ≥ 15 mm Hg) between 2013 and 2021. A receiver operating characteristic curve was plotted to determine the cutoff value of all-cause death. Survival was estimated using the Kaplan–Meier method and analyzed using the log-rank test. The Cox proportional hazards model was performed to determine the association between CPET and all-cause death.

Seventeen patients died within a mean of 2.2 ± 1.3 years. Compared with survivors, nonsurvivors displayed a significantly worse 6-min walk distance, workload, exercise time and peak oxygen consumption (VO₂)/kg with a trend of a lower oxygen uptake efficiency slope (OUES) adjusted by Bonferroni’s correction. Multivariate Cox regression revealed that the peak VO₂/kg was significantly associated with all-cause death after adjusting for Cpc-PH/Ipc-PH. Compared with Cpc-PH patients with a peak VO₂/kg ≥ 10.7 ml kg⁻¹ min⁻¹, Ipc-PH patients with a peak VO₂/kg < 10.7 ml kg⁻¹ min⁻¹ had a worse survival (P < 0.001).

The peak VO₂/kg is independently associated with all-cause death in patients with PH-LHD. The peak VO₂/kg can also be analyzed together with Cpc-PH/Ipc-PH to better indicate the prognosis of patients with PH-LHD.

The online version contains supplementary material available at 10.1186/s12872-022-02574-0.

• Combined post- and precapillary pulmonary hypertension
• Isolated postcapillary pulmonary hypertension
• Peak oxygen consumption
• Prognosis
• Pulmonary hypertension due to left heart disease

• Cardiopulmonary exercise testing
• Echocardiography
• Pulmonary hypertension
• Systemic sclerosis
• TAPSE/sPAP ratio

Periodic repetition of right heart catheterization (RHC) in pulmonary arterial hypertension (PAH) can be challenging. We evaluated the correlation between RHC and cardiopulmonary exercise test (CPET) aiming at CPET use as a potential noninvasive tool for hemodynamic burden evaluation. One hundred and forty‐four retrospective PAH patients who had performed CPET and RHC within 2 months were enrolled. The following analyses were performed: (a) CPET parameters in hemodynamic variables tertiles; (b) position of hemodynamic parameters in the peak end‐tidal carbon dioxide pressure (P_ETCO₂) versus ventilation/carbon dioxide output (VE/VCO₂) slope scatterplot, which is a specific hallmark of exercise respiratory abnormalities in PAH; (c) association between CPET and a hemodynamic burden score developed including mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR), cardiac index, and right atrial pressure. VE/VCO₂ slope and peak P_ETCO₂ significantly varied in mPAP and PVR tertiles, while peak oxygen uptake (peak VO₂) and O₂ pulse varied in the tertiles of all hemodynamic parameters. P_ETCO₂ versus VE/VCO₂ slope showed a strong hyperbolic relationship (R² = 0.7627). Patients with peak P_ETCO₂ > median (26 mmHg) and VE/VCO₂ slope < median (44) presented lower mPAP and PVR (p < 0.005) than patients with peak P_ETCO₂ < median and VE/VCO₂ slope > median. Multivariate analysis individuated peak VO₂ (p = 0.0158) and peak P_ETCO₂ (p = 0.0089) as hemodynamic score independent predictors; the formula 11.584 − 0.0925 × peak VO₂ − 0.0811 × peak P_ETCO₂ best predicts the hemodynamic score value from CPET data. A significant correlation was found between estimated and calculated scores (p < 0.0001), with a precise match for patients with mild‐to‐moderate hemodynamic burden (76% of cases). The results of the present study suggest that CPET could allow to estimate the hemodynamic burden in PAH patients.

• guideline
• idiopathic pulmonary arterial hypertension
• prognosis
• pulmonary arterial hypertension
• risk assessment

• Diagnosis and management principles
• Pediatric
• Pulmonary hypertension

Cardiopulmonary exercise testing (CPET) is a frequently used tool in the differential diagnosis of dyspnoea. Ventilatory inefficiency, defined as high minute ventilation (V′_E) relative to carbon dioxide output (V′_CO2), is a hallmark characteristic of pulmonary vascular diseases, which contributes to exercise intolerance and disability in these patients. The mechanisms of ventilatory inefficiency are multiple and include high physiologic dead space, abnormal chemosensitivity and an altered carbon dioxide (CO₂) set-point. A normal V′_E/V′_CO2 makes a pulmonary vascular disease such as pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) unlikely. The finding of high V′_E/V′_CO2 without an alternative explanation should prompt further diagnostic testing to exclude PAH or CTEPH, particularly in patients with risk factors, such as prior venous thromboembolism, systemic sclerosis or a family history of PAH. In patients with established PAH or CTEPH, the V′_E/V′_CO2 may improve with interventions and is a prognostic marker. However, further studies are needed to clarify the added value of assessing ventilatory inefficiency in the longitudinal follow-up of patients.

Ventilatory inefficiency is a hallmark feature of PH that reflects abnormal ventilation/perfusion matching, chemosensitivity and an altered CO₂ set-point. Minute ventilation/CO₂ production is useful in the diagnosis, management and prognostication of PH.https://bit.ly/3jnNdUG

Rationale: Exercise capacity predicts mortality in pulmonary arterial hypertension (PAH), but limited data exist on the routine use of maximal exercise testing.

Objectives: This study evaluates a simple-to-perform maximal test (the incremental shuttle walking test) and its use in risk stratification in PAH.

Methods: Consecutive patients with pulmonary hypertension were identified from the ASPIRE (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre) registry (2001–2018). Thresholds for levels of risk were identified at baseline and tested at follow-up, and their incorporation into current risk stratification approaches was assessed.

Results: Of 4,524 treatment-naive patients with pulmonary hypertension who underwent maximal exercise testing, 1,847 patients had PAH. A stepwise reduction in 1-year mortality was seen between levels 1 (≤30 m; 32% mortality) and 7 (340–420 m; 1% mortality) with no mortality for levels 8–12 (≥430 m) in idiopathic and connective tissue disease–related PAH. Thresholds derived at baseline of ≤180 m (>10%; high risk), 190–330 m (5–10%; intermediate risk), and ≥340 m (<5%; low risk of 1-yr mortality) were applied at follow-up and also accurately identified levels of risk. Thresholds were incorporated into the REVEAL (Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management) 2.0 risk score calculator and French low-risk approach to risk stratification, and distinct categories of risk remained.

Conclusions: We have demonstrated that maximal exercise testing in PAH stratifies mortality risk at baseline and follow-up. This study highlights the potential value of the incremental shuttle walking test as an alternative to the 6-minute walking test, combining some of the advantages of maximal exercise testing and maintaining the simplicity of a simple-to-perform field test.

• exercise testing
• outcome
• risk stratification

Ventilatory efficiency can be evaluated using the relationship between minute ventilation (V′_E) and the rate of CO₂ production (V′_CO2). In accordance with the modified alveolar ventilation equation, this relationship is determined by changes in dead space volume (V_D) and/or the arterial CO₂ tension (P_aCO2) equilibrium point. In this review, we summarise the physiological factors that may account for normative ageing and pregnancy induced increases in V′_E/V′_CO2 during exercise. Evidence suggests that age-related increases in V_D and pregnancy-related decreases in the P_aCO2 equilibrium point are mechanistically linked to the increased V′_E/V′_CO2 during exercise. Importantly, the resultant increase in V′_E/V′_CO2 (ratio or slope), with normal ageing or pregnancy, remains below the critical threshold for prognostic indication in cardiopulmonary disease, is not associated with increased risk of adverse health outcomes, and does not affect the respiratory system's ability to fulfil its primary role of eliminating CO₂ and maintaining arterial oxygen saturation during exercise.

The minute ventilation/carbon dioxide production response to exercise is elevated with advancing age and in healthy pregnancy due to increased dead space and lowering of the arterial partial pressure of carbon dioxide equilibrium point, respectively.https://bit.ly/2GJXm0o

• Cardiopulmonary exercise test
• Exercise capacity
• Exercise intolerance
• Pulmonary hypertension
• Pulmonary vascular disease
• Unexplained dyspnea

• cardio-ankle vascular index
• idiopathic pulmonary arterial hypertension
• pulse wave velocity
• survival
• systemic arterial stiffness

• Adult
• Cardio Ankle Vascular Index
• Familial Primary Pulmonary Hypertension/diagnosis
• Familial Primary Pulmonary Hypertension/mortality
• Familial Primary Pulmonary Hypertension/physiopathology
• Female
• Hemodynamics
• Humans
• Male
• Middle Aged
• Predictive Value of Tests
• Prognosis
• Retrospective Studies
• Risk Assessment
• Risk Factors
• Vascular Stiffness

• Cardiopulmonary exercise testing
• Combined pulmonary fibrosis and emphysema
• Pulmonary hypertension

Objective: Pulmonary vascular resistance (PVR) is a measurement obtained with invasive right heart catheterization (RHC) that is commonly used for management of patients with pulmonary arterial hypertension (PAH). Computed tomography pulmonary angiography (CTPA) is also done as part of the workup for PAH in some cases. The aim of our study was to assess the correlation of contrast dynamic changes in the main pulmonary artery (MPA) on CTPA with PVR obtained with RHC.

Methods: This is an IRB-approved retrospective study performed in two separate institutions (Medical College of Wisconsin and University of Alabama) between January 2010 and December 2013. During CTPA done as test bolus, serial images are acquired at the level of MPA after intravenous injection of contrast to determine timing of the CT acquisition. Since the PVR changes with the degree of PAH, we hypothesize that will be reflected in the contrast kinetics in MPA. A correlation of standard CT metrics (MPA diameter, right pulmonary artery [PA] diameter, left PA diameter, MPA/aorta ratio, and right ventricle/left ventricle [RV/LV] ratio) and dynamic (full width at half maximum) CTPA parameters in patients with known PAH was performed with PVR obtained from RHC done within 30 days. Statistical analysis was performed by Pearson correlation coefficient.

Results: Among 221 patients in our database, 37 patients fulfilled the selection criteria. There was a strong correlation between full width half maximum (FWHM) and mean pulmonary artery pressure (mPAP) (r=0.69, p value<0.00001), PVR (r=0.8, p value<0.00001) and indexed PVR (PVRI) (r=0.75, p value<0.00001).

Conclusion: FWHM obtained from CTPA strongly correlates with RHC parameters and is potentially more helpful than static measurements for follow-up of patients with known PAH to assess response to treatment or progression.

• cardiac catheterization
• computed tomography angiography
• pulmonary arterial hypertension
• pulmonary vascular resistance

• Aerobic capacity
• Exercise
• Pulmonary arterial hypertension
• Pulmonary compliance
• Right ventricle

• Adult
• Familial Primary Pulmonary Hypertension/diagnostic imaging
• Female
• Humans
• Hypertension, Pulmonary/diagnostic imaging
• Male
• Oxygen
• Pulmonary Artery/diagnostic imaging
• Ventricular Dysfunction, Right/diagnostic imaging
• Ventricular Function, Right

Cardiopulmonary exercise testing (CPET) is of great interest and utility for clinicians dealing Pulmonary Hypertension (PH) in several ways, including: helping with differential diagnosis, evaluating exercise intolerance and its underpinning mechanisms, accurately assessing exertional dyspnea and unmasking its underlying often non-straightforward mechanisms, generating prognostic indicators. Pathophysiologic anomalies in PH can range from reduced cardiac output and aerobic capacity, to inefficient ventilation, dyspnea, dynamic hyperinflation, and locomotor muscle dysfunction. CPET can magnify the PH-related pathophysiologic anomalies and has a major role in the management of PH patients.

• cardiopulmonary exercise testing
• dynamic hyperinflation
• dyspnea
• prognosis
• pulmonary hypertension
• ventilatory inefficiency

• Exercise intolerance
• Inspiratory muscle weakness
• Pulmonary hypertension
• Right heart failure

Cardiopulmonary exercise testing (CPET) is a method for evaluating pulmonary and cardiocirculatory abnormalities, dyspnea, and exercise tolerance in healthy individuals and patients with chronic conditions. During exercise, ventilation (V˙_E) increases in proportion to metabolic demand [i.e., carbon dioxide production (V˙CO₂)] to maintain arterial blood gas and acid-base balance. The response of V˙_E relative to V˙CO₂ (V˙_E/V˙CO₂) is commonly termed ventilatory efficiency and is becoming a common physiological tool, in conjunction with other key variables such as operating lung volumes, to evaluate exercise responses in patients with chronic conditions. A growing body of research has shown that the V˙_E/V˙CO₂ response to exercise is elevated in conditions such as chronic heart failure (CHF), pulmonary hypertension (PH), interstitial lung disease (ILD), and chronic obstructive pulmonary disease (COPD). Importantly, this potentiated V˙_E/V˙CO₂ response contributes to dyspnea and exercise intolerance. The clinical significance of ventilatory inefficiency is demonstrated by findings showing that the elevated V˙_E/V˙CO₂ response to exercise is an independent predictor of mortality in patients with CHF, PH, and COPD. In this article, the underlying physiology, measurement, and interpretation of exercise ventilatory efficiency during CPET are reviewed. Additionally, exercise ventilatory efficiency in varying disease states is briefly discussed.

• dyspnea
• exercise testing
• pulmonary gas-exchange
• ventilation
• ventilatory efficiency