Multimodality Imaging-Based Right Ventricular Phenotyping for Risk Stratification and Short-Term Outcomes in Group 1 Pulmonary Arterial Hypertension: The MIRROR-PAH Study

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by pulmonary vascular remodeling, increased pulmonary vascular resistance, and progressive right ventricular (RV) pressure overload. Despite substantial advances in targeted therapies, morbidity and mortality remain high, and patient prognosis is largely determined by the ability of the right ventricle to adapt to the increased afterload imposed by the pulmonary circulation.

Current clinical practice guidelines recommend periodic risk assessment to guide treatment decisions and monitor disease progression. Contemporary non-invasive risk stratification models incorporate clinical, functional, and biochemical parameters, including World Health Organization (WHO) functional class, exercise capacity, and natriuretic peptide levels. Although cardiac imaging provides important information regarding right ventricular structure and function and is widely used during the evaluation and follow-up of patients with PAH, imaging parameters are not routinely incorporated into simplified follow-up risk assessment models. Consequently, a considerable proportion of patients remain classified within intermediate-risk categories, representing a heterogeneous population with variable clinical trajectories and therapeutic needs.

Multimodality cardiac imaging provides comprehensive assessment of right ventricular structure, function, and remodeling. Echocardiography remains the cornerstone imaging modality for routine evaluation and longitudinal follow-up of PAH patients, while cardiac magnetic resonance (CMR) offers highly accurate and reproducible quantification of right ventricular volumes and function. Several imaging-derived parameters have been associated with disease severity and adverse outcomes in PAH. In addition, invasive hemodynamic assessment obtained by right heart catheterization (RHC) provides important prognostic information regarding pulmonary vascular disease and right ventricular adaptation. The integration of imaging and hemodynamic information may therefore provide incremental value beyond conventional non-invasive risk assessment strategies.

The MIRROR-PAH Study (Multimodality Imaging-Based Right Ventricular Phenotyping for Risk Stratification and Short-Term Outcomes in Group 1 Pulmonary Arterial Hypertension) is a single-center, prospective, observational cohort study designed to evaluate the relationship between multimodality imaging-derived right ventricular characteristics, invasive hemodynamic measurements, established risk assessment models, and short-term clinical outcomes in patients with Group 1 PAH.

Adult patients with established Group 1 PAH who are undergoing routine follow-up and have available RHC and CMR examinations will be consecutively enrolled. Baseline clinical, laboratory, echocardiographic, hemodynamic, and CMR data will be collected. Follow-up clinical assessment and transthoracic echocardiography will be performed at 6 months.