Chest
Volume 132, Issue 6, December 2007, Pages 1906-1912
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Original Research
Pulmonary Hypertension
Noninvasively Assessed Pulmonary Artery Stiffness Predicts Mortality in Pulmonary Arterial Hypertension

https://doi.org/10.1378/chest.07-1246Get rights and content

Aims: Decreased total compliance of the pulmonary vascular bed is associated with increased mortality in patients with pulmonary arterial hypertension (PAH). We investigated whether proximal pulmonary artery stiffness, in terms of area distensibility and noninvasively assessed relative area change (RAC), calculated as relative cross-sectional area change, predicts mortality in patients with PAH.

Methods and results

Eighty-six subjects underwent right-heart catheterization and MRI to assess area distensibility and RAC. Patients were followed up to 48 months. Kaplan-Meier plot and Cox proportional hazards regression analyses assessed the predictive value of area distensibility and RAC. In 70 patients, the diagnosis PAH was confirmed, and 16 subjects served as control subjects. In comparison with control subjects, proximal pulmonary arteries of patients were distended (685 ± 214 mm2 vs 411 ± 153 mm2, p < 0.001), less distensible (area distensibility = 0.46 ± 0.38·10−2 mm Hg−1 vs 3.69 ± 1.96·10−2 mm Hg−1, p < 0.0001), and RAC was smaller (20 ± 10% vs 58 ± 21%, p < 0.0001) [mean ± SD]. RAC showed an inverse curvilinear relation with mean pulmonary artery pressure (R2 = 0.47). Eighteen patients (26%) died because of cardiopulmonary causes. Patients with a pulmonary artery RAC ≤ 16% had a worse prognosis than those with a value > 16% (log-rank p < 0.001). RAC predicted mortality better than area distensibility.

Conclusion

Noninvasively measured pulmonary artery RAC predicts mortality in patients with PAH.

Section snippets

Patient Selection

All patients referred to our institute for the evaluation and treatment of PAH from September 2001 to September 2005 were considered for enrollment in to this study. The diagnosis of PAH was assessed according to the 2003 Venice consensus guidelines on the diagnosis and treatment of PAH.15 All persons underwent an MRI scan of the heart and large pulmonary vessels within 1 week of initial right-heart catheterization. The selection of patients depended on whether the diagnosis of PAH could be

Results

Seventy PAH patients and 16 subjects without pulmonary hypertension were included in this study. Table 1summarizes patient characteristics, etiologies, and hemodynamics. The majority of patients were female and had a diagnosis of idiopathic PAH, and right-heart catheterization data yielded characteristics of RV pressure overload. In the 16 control subjects, the diagnosis of pulmonary hypertension was excluded by right-heart catheterization. The control subjects appeared to be younger, but this

Discussion

This MRI study shows that in PAH patients, the pulmonary artery becomes distended and less distensible. In comparison with subjects without PAH, both area distensibility and RAC are significantly lower in PAH patients. Furthermore, it was shown that noninvasively measured RAC is a predictor of mortality.

Conclusion

In patients with PAH, increased pulmonary artery pressure causes distension and possibly wall remodeling, both resulting in stiffening of the proximal pulmonary arteries. Noninvasively assessed pulmonary artery RAC is a good predictor of mortality.

Parameters of the Applied MRI Pulse Sequence

MRI pulse sequence type is steady-state free precession, and phase-encoding direction is anterior to posterior; field of view in read, 320 mm; field of view in phase, 81%; slice thickness, 5 mm; in-plane resolution, 150 × 256 pixels, 1.73 × 1.25 mm per pixel; temporal resolution, 35 ms; echo time, 1.6 ms; flip angle, 60°; retrospective ECG triggering, 25 phases reconstructed; receiver bandwidth, 930 Hz per pixel; and 11 phase-encoding lines per beat.

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    The authors have no conflicts of interest to disclose.

    Dr. Gan was financially supported by the Netherlands Organisation for Scientific Research, Mozaiek grant, project number 017.001.154. Dr. Lankhaar was financially supported by the Netherlands Heart Foundation grant NHS2003B274.

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