Pulmonary Arterial Hypertension (PAH), Pulmonary Arterial Hypertension (PAH) (WHO Group 1 PH)
Conditions
Keywords
VExUS, Pulmonary Arterial Hypertension, Risk Stratification, Venous Congestion, Right Ventricular Function, doppler ultrasound
Brief summary
This prospective observational study aims to evaluate the relationship between the Venous Excess Ultrasound Score (VEXUS) and the ESC/ERS 2022 simplified four-strata risk assessment model in adult patients with World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH). The study investigates whether VEXUS can enhance risk stratification and predict haemodynamic congestion by correlating VEXUS with functional, biochemical, and invasive haemodynamic parameters.
Detailed description
Pulmonary arterial hypertension (PAH) is a progressive disorder characterised by increased pulmonary vascular resistance, right ventricular overload, systemic venous congestion, and high mortality. The ESC/ERS 2022 simplified four-strata model (low, intermediate-low, intermediate-high, high risk) guides treatment decisions using WHO functional class, 6-minute walk distance, and BNP/NT-proBNP levels. However, biochemical markers may be costly or insufficiently sensitive to early haemodynamic deterioration. VEXUS (Venous Excess Ultrasound Score), a point-of-care ultrasonographic method assessing hepatic, portal, and renal venous Doppler patterns, has shown promise in representing venous congestion. This study evaluates whether VEXUS correlates with ESC/ERS risk categories and invasive haemodynamic parameters including right atrial pressure, mean pulmonary arterial pressure, pulmonary vascular resistance, cardiac output, and pulmonary output. The study further aims to explore whether integrating VEXUS into PAH follow-up may strengthen risk assessment.
Interventions
None listed
Sponsors
Istanbul University - Cerrahpasa
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Adults aged ≥18 years * Established diagnosis of WHO Group 1 pulmonary arterial hypertension (PAH) * Stable outpatient clinical status at the time of VExUS ultrasonography * Venous Excess Ultrasound Score (VExUS) evaluation performed with adequate ultrasonographic acoustic windows * Clinically indicated right heart catheterisation (RHC) performed within ±60 days of VExUS assessment * Availability of ESC/ERS 2022 simplified risk assessment variables (WHO functional class, BNP/NT-proBNP, and 6-minute walk distance) * Ability to provide written informed consent
Exclusion criteria
* Pulmonary hypertension other than WHO Group 1, including: * PH due to left heart disease (WHO Group 2) * PH due to chronic lung disease or hypoxaemia (WHO Group 3) * Chronic thromboembolic pulmonary hypertension (CTEPH; WHO Group 4) * Multifactorial PH (WHO Group 5) * Eisenmenger syndrome * Complex or unrepaired congenital heart disease * Suspected pulmonary veno-occlusive disease (PVOD) * Pulmonary capillary haemangiomatosis (PCH) * Acute decompensated right heart failure * Severe renal dysfunction (eGFR \<30 mL/min/1.73 m²) * Severe hepatic impairment (Child-Pugh Class C) * Congestive hepatopathy * Active infection * Pregnancy * Inability or unwillingness to provide informed consent * Poor ultrasonographic acoustic window preventing adequate VExUS scoring
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Correlation Between the VExUS Score and Invasive Haemodynamic Parameters | At the single study visit (Day 0) | Correlation between the VExUS Score (0-3 Doppler-based venous congestion score) and invasive haemodynamic measurements obtained by right heart catheterisation, including pulmonary vascular resistance (PVR; Wood units), right atrial pressure (RAP; mmHg), Fick-derived cardiac output (L/min) and pulmonary blood flow (PBF; L/min). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Correlation Between the VExUS Score and Non-Invasive Clinical Markers | Day 0 | Correlation between the VExUS Score and serum B-type natriuretic peptide (BNP; pg/mL), WHO functional class (4-level ordinal scale) and six-minute walk distance (metres), which form key components of current ESC/ERS risk stratification models. |
| Correlation Between the VExUS Score and Echocardiographic RV-PA Coupling Indices | Day 0 | Correlation between the VExUS Score and tricuspid annular plane systolic excursion (TAPSE; mm) and the TAPSE/systolic pulmonary artery pressure ratio (unitless). |
| Predictive Value of the VExUS Score for Elevated Pulmonary Vascular Resistance (≥ 6 Wood Units) | Day 0 | Evaluation of the predictive performance of the VExUS Score for identifying patients with elevated pulmonary vascular resistance (≥ 6 Wood units). Predictive metrics (including odds ratios) will be reported. |
| Incremental Contribution of the VExUS Score to ESC/ERS Four-Strata Risk Classification | Day 0 | Change in ESC/ERS simplified four-strata risk category after incorporation of the VExUS Score, including net reclassification and risk-category shifts. |
Countries
Turkey (Türkiye)
Outcome results
None listed