PAH-SDB: Sleep-Disordered Breathing and Outcomes in Pulmonary Arterial Hypertension
Trial statusRecruiting
Trial phaseNot listed
Trial typeObservational
Biological sexAll
Age18+
SponsorChinese Pulmonary Vascular Disease Research Group
About this trial
Pulmonary arterial hypertension (PAH) is a progressive pulmonary vascular disease characterized by increased pulmonary vascular resistance, right ventricular dysfunction, and clinically important morbidity and mortality. Sleep-disordered breathing (SDB) and sleep-related hypoxemia are common in patients with PAH, but their prevalence, physiological determinants, longitudinal trajectories, prognostic significance, and potential role in individualized management remain incompletely defined.
PAH-SDB is a prospective, multicenter, observational patient registry enrolling adults with right-heart-catheterization-confirmed World Health Organization Group 1 PAH at pulmonary vascular centers across China. Participants will undergo standardized nocturnal sleep and cardiorespiratory monitoring together with comprehensive PAH assessment, including clinical characteristics, functional status, biomarkers, echocardiography, hemodynamics, treatment, and outcomes. Selected participants may undergo additional polysomnography, cardiac magnetic resonance imaging, wearable monitoring, repeated sleep assessments, or biospecimen collection when available and ethically approved.
The registry will characterize the prevalence and phenotypes of sleep-disordered breathing and nocturnal physiological abnormalities; examine their relationships with PAH severity and right ventricular-pulmonary vascular function; determine their value for predicting clinical worsening, hospitalization, transplantation, and mortality; evaluate longitudinal changes in sleep physiology in relation to PAH treatment and disease trajectory; and develop and validate multimodal statistical and artificial-intelligence models for phenotyping, risk prediction, and treatment-response stratification. Clinical management will remain at the discretion of treating physicians, and the registry will not assign PAH or sleep-related therapies.
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
Age ≥18 years at enrollment.
Diagnosis of WHO Group 1 pulmonary arterial hypertension established by right heart catheterization using contemporary hemodynamic criteria: mean pulmonary artery pressure >20 mmHg at rest, pulmonary arterial wedge pressure ≤15 mmHg, and pulmonary vascular resistance >2 Wood units, with clinical classification consistent with Group 1 PAH.
PAH etiology may include idiopathic, heritable, drug/toxin-associated, connective-tissue-disease-associated, congenital-heart-disease-associated PAH after appropriate clinical classification, portal-hypertension-associated PAH, HIV-associated PAH, or other recognized Group 1 PAH subtypes.
Clinically stable enough to undergo overnight sleep/cardiorespiratory monitoring, as judged by the treating team.
Disqualifiers
Pulmonary hypertension predominantly attributable to left heart disease (Group 2), lung disease and/or hypoxia (Group 3), chronic thromboembolic pulmonary hypertension or other pulmonary artery obstruction (Group 4), or unclear/multifactorial mechanisms (Group 5).
Pulmonary veno-occlusive disease / pulmonary capillary hemangiomatosis when the clinical phenotype is considered separately from conventional PAH for the planned analysis, unless the protocol intentionally includes this subgroup and prespecifies it as a distinct stratum.
Acute decompensated right heart failure, cardiogenic shock, severe active infection, or another acute condition that would make the baseline sleep recording unrepresentative or unsafe; these patients may be reconsidered after stabilization.
Current hospitalization primarily for an acute non-PAH illness likely to substantially distort nocturnal respiratory physiology.
Trial population
Consecutive adult patients with RHC-confirmed WHO Group 1 PAH receiving care at participating pulmonary vascular and cardiovascular centers in China. The registry will include newly diagnosed and prevalent PAH, with enrollment strata defined by disease duration and treatment status. Participating centers will include Fuwai Hospital and additional Chinese centers with expertise in PAH and the ability to perform standardized clinical and nocturnal physiological assessments.
Trial design
Design model
Cohort
Time perspective
Prospective
Treatments tested in this trial
Standardized Overnight Sleep and Cardiorespiratory Monitoring
Diagnostic testParticipants undergo protocol-standardized nocturnal monitoring without study-assigned therapy. The core recording includes airflow, respiratory effort, oxygen saturation, pulse/heart rate, body position, and snoring when available. Full polysomnography, extended wearable monitoring, repeated sleep studies, and other physiological signals may be obtained in nested subcohorts. All sleep-related treatment decisions remain under routine clinical care.
Treatment groups
1,500 Participants
are divided into 1 treatment groupGroup A: PAH-SDB Prospective Cohort1 intervention
Trial outcomes
Primary outcomes
1
Prevalence and phenotypic distribution of sleep-disordered breathing and nocturnal hypoxemia
Proportion of participants with sleep-disordered breathing (SDB) and its phenotypes, including obstructive sleep apnea (OSA) and central sleep apnea (CSA), together with the distribution of nocturnal hypoxemia and multidimensional sleep physiological burden. Core measures will include the apnea-hypopnea index (AHI) or respiratory event index (REI), obstructive and central respiratory event indices, oxygen desaturation index (ODI), percentage of recording time with peripheral oxygen saturation below 90% (T90), mean and minimum peripheral oxygen saturation (SpO₂), and prespecified hypoxic-burden metrics. Prevalence estimates will be reported with 95% confidence intervals.
Time frame
Baseline2
Time to first clinical worsening event
Time from enrollment to the first adjudicated clinical worsening event. The primary composite is recommended to include all-cause death, lung or heart-lung transplantation, PAH-related unplanned hospitalization, atrial septostomy, or clinically driven major PAH treatment escalation/rescue therapy because of documented disease worsening. A sensitivity definition limited to hard outcomes will also be analyzed.
Time frame
From enrollment through 5 years of follow-upSecondary outcomes
1
All-cause mortality
Time from enrollment to death from any cause
Time frame
From enrollment through 5 years of follow-up2
Transplant-free survival
Time to death or lung/heart-lung transplantation
Time frame
From enrollment through 5 years of follow-up3
PAH-related hospitalization
Time to first PAH-related unplanned hospitalization and recurrent hospitalization burden
Time frame
From enrollment through 5 years of follow-up4
Change From Baseline in REVEAL 2.0 Risk Score
The REVEAL 2.0 risk score will be calculated using available clinical variables according to the established REVEAL 2.0 algorithm. Higher scores indicate greater predicted mortality risk. Change from baseline will be evaluated at available follow-up assessments.
Time frame
Baseline to longitudinal follow-up through 5 yearsOther outcomes
1
Predictive Performance of AI-Enabled Multimodal Models for Major Clinical Worsening
Artificial intelligence and machine-learning approaches will be used to derive data-driven nocturnal physiological phenotypes and to develop multimodal prediction models integrating sleep-derived signals with clinical, hemodynamic, imaging, electrocardiographic, and laboratory variables. Predictive performance for the primary longitudinal outcome of major clinical worsening will be evaluated using the area under the receiver operating characteristic curve (AUROC) in held-out or center-level validation datasets.
Time frame
From baseline assessment through 5 years of follow-up.2
Treatment-Response Heterogeneity According to Baseline Sleep Phenotype
Treatment response will be evaluated according to baseline sleep-disordered breathing and nocturnal physiological phenotype. A clinically meaningful response will be defined as improvement by at least one category in the ESC/ERS four-strata PAH risk classification following initiation or escalation of PAH-targeted therapy. The proportion of participants achieving this predefined response will be compared across baseline sleep phenotypes. Analyses will account for treatment regimen, baseline disease severity, and other relevant confounders.
Time frame
From treatment initiation or escalation to the subsequent clinical reassessment, generally within 3-12 months, and through 5 years of follow-up.Sponsors and contacts
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Chinese Pulmonary Vascular Disease Research Group
Lead sponsor
Chinese Academy of Medical Sciences, Fuwai Hospital
Collaborator
Source ClinicalTrials.gov