Objectives To assess whether phase-resolved functional lung MRI (PREFUL-MRI) can differentiate pulmonary arterial hypertension (PAH) from chronic thromboembolic pulmonary hypertension (CTEPH) and to compare PREFUL-derived perfusion and ventilation characteristics between the two entities. Materials and methods In this single-center study (January 2024–March 2025), patients with pulmonary hypertension (PH) who underwent PREFUL-MRI, ventilation/perfusion single-photon emission CT/CT (V/Q SPECT/CT), and right-heart catheterization within 1 week were retrospectively enrolled. PREFUL-MRI was acquired with a free-breathing fast spoiled gradient echo sequence to generate perfusion and ventilation maps. PREFUL maps and V/Q SPECT/CT were assessed by four blinded readers. Quantitative PREFUL parameters were compared between PAH and CTEPH, and correlations with hemodynamics were analyzed. Results Fifty-three PH patients were included (PAH 26, CTEPH 27). All manifested multiple perfusion defects on PREFUL maps. Visual assessment with PREFUL yielded 70% sensitivity and 38% specificity for CTEPH, lower than V/Q SPECT/CT (sensitivity 95%, specificity 95%). CTEPH had higher perfusion defect percentage and lower mean perfusion than PAH (median QDPexclusive 51.9% vs. 24.4%, p < 0.001; QDPtotal 58.4% vs. 30.85%, p < 0.001; mean perfusion 2.8% vs. 3.9%, p = 0.003). QDPexclusive represents areas with perfusion defects but without ventilation defects, whereas QDPtotal indicates the overall perfusion defect percentage. Across the cohort, QDPexclusive correlated positively with pulmonary vascular resistance (PVR) (ρ = 0.313, p = 0.031). Conclusions PREFUL-MRI did not reliably distinguish PAH from CTEPH on visual assessment, although CTEPH exhibited a larger perfusion defect burden. It may be more suitable for functional evaluation of PH rather than for initial differential diagnosis. Key Points <Emphasis Type="BoldItalic">Question</Emphasis>Can phase-resolved functional lung MRI (PREFUL-MRI) noninvasively distinguish pulmonary arterial hypertension (PAH) from chronic thromboembolic pulmonary hypertension (CTEPH), and reflect their hemodynamic severity? <Emphasis Type="BoldItalic">Findings</Emphasis>PREFUL-MRI showed limited visual discrimination compared to V/Q SPECT/CT; CTEPH exhibited more severe perfusion defects; perfusion defect percentage correlated with pulmonary vascular resistance. <Emphasis Type="BoldItalic">Clinical relevance</Emphasis>PREFUL-MRI provides radiation- and contrast-free perfusion metrics reflecting hemodynamic burden in pulmonary hypertension. Though suboptimal for initial discrimination in disease subtypes, it supports functional evaluation once the diagnosis is established. Graphical Abstract

What is the difference between pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension on phase-resolved functional lung MRI? A cross-sectional observational study

期刊论文
发表日期：

2026年1月13日

查看全文

Anqi Liu,
Jie Du,
Huan Li,
Linfeng Xi,
Jianping Wang,
Yifei Ni,
Shuai Zhang,
Qiang Huang,
Jing An,
Jens Vogel-Claussen,
Andreas Voskrebenzev,
Liping Fu,
Min Liu

摘要

Objectives

To assess whether phase-resolved functional lung MRI (PREFUL-MRI) can differentiate pulmonary arterial hypertension (PAH) from chronic thromboembolic pulmonary hypertension (CTEPH) and to compare PREFUL-derived perfusion and ventilation characteristics between the two entities.

Materials and methods

In this single-center study (January 2024–March 2025), patients with pulmonary hypertension (PH) who underwent PREFUL-MRI, ventilation/perfusion single-photon emission CT/CT (V/Q SPECT/CT), and right-heart catheterization within 1 week were retrospectively enrolled. PREFUL-MRI was acquired with a free-breathing fast spoiled gradient echo sequence to generate perfusion and ventilation maps. PREFUL maps and V/Q SPECT/CT were assessed by four blinded readers. Quantitative PREFUL parameters were compared between PAH and CTEPH, and correlations with hemodynamics were analyzed.

Results

Fifty-three PH patients were included (PAH 26, CTEPH 27). All manifested multiple perfusion defects on PREFUL maps. Visual assessment with PREFUL yielded 70% sensitivity and 38% specificity for CTEPH, lower than V/Q SPECT/CT (sensitivity 95%, specificity 95%). CTEPH had higher perfusion defect percentage and lower mean perfusion than PAH (median QDP_exclusive 51.9% vs. 24.4%, p < 0.001; QDP_total 58.4% vs. 30.85%, p < 0.001; mean perfusion 2.8% vs. 3.9%, p = 0.003). QDP_exclusive represents areas with perfusion defects but without ventilation defects, whereas QDP_total indicates the overall perfusion defect percentage. Across the cohort, QDP_exclusive correlated positively with pulmonary vascular resistance (PVR) (ρ = 0.313, p = 0.031).

Conclusions

PREFUL-MRI did not reliably distinguish PAH from CTEPH on visual assessment, although CTEPH exhibited a larger perfusion defect burden. It may be more suitable for functional evaluation of PH rather than for initial differential diagnosis.

Key Points

QuestionCan phase-resolved functional lung MRI (PREFUL-MRI) noninvasively distinguish pulmonary arterial hypertension (PAH) from chronic thromboembolic pulmonary hypertension (CTEPH), and reflect their hemodynamic severity?

FindingsPREFUL-MRI showed limited visual discrimination compared to V/Q SPECT/CT; CTEPH exhibited more severe perfusion defects; perfusion defect percentage correlated with pulmonary vascular resistance.

Clinical relevancePREFUL-MRI provides radiation- and contrast-free perfusion metrics reflecting hemodynamic burden in pulmonary hypertension. Though suboptimal for initial discrimination in disease subtypes, it supports functional evaluation once the diagnosis is established.

Graphical Abstract