Chest
Volume 141, Issue 4, April 2012, Pages 935-943
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Original Research
Pulmonary Vascular Disease
Progressive Changes in Right Ventricular Geometric Shortening and Long-term Survival in Pulmonary Arterial Hypertension

https://doi.org/10.1378/chest.10-3277Get rights and content

Background

Until now, many investigators have focused on describing right ventricular (RV) dysfunction in groups of patients with pulmonary arterial hypertension (PAH), but very few have addressed the deterioration of RV function over time. The aim of this study was to investigate time courses of RV geometric changes during the progression of RV failure.

Methods

Forty-two patients with PAH were selected who underwent right-sided heart catheterization and cardiac MRI at baseline and after 1-year follow-up. Based on the survival after this 1-year run-in period, patients were classified into two groups: survivors (26 patients; subsequent survival of > 4 years) and nonsurvivors (16 patients; subsequent survival of < 4 years). Four-chamber cine imaging was used to quantify RV longitudinal shortening (apex-base distance change), RV transverse shortening (septum-free wall distance change), and RV fractional area change (RVFAC) between end diastole and end systole.

Results

Longitudinal shortening, transverse shortening, and RVFAC measured at the beginning of the run-in period and 1 year later were significantly higher in subsequent survivors than in nonsurvivors (P < .05). Longitudinal shortening did not change during the run-in period in either patient group. Transverse shortening and RVFAC did not change during the run-in period in subsequent survivors but did decrease in subsequent nonsurvivors (P < .05). This decrease was caused by increased leftward septal bowing.

Conclusions

Progressive RV failure in PAH is associated with a parallel decline in longitudinal and transverse shortening until a floor effect is reached for longitudinal shortening. A further reduction of RV function is due to progressive leftward septal displacement. Because transverse shortening incorporates both free wall and septum movements, this parameter can be used to monitor the decline in RV function in end-stage PAH.

Section snippets

Patient Population

The present study was performed in an observational cohort of patients with PAH and was part of a prospective ongoing research project aimed to evaluate the RV in PAH by means of MRI. Between May 2003 and May 2005, 287 patients were referred to our hospital for the evaluation of pulmonary hypertension. A diagnosis of PAH was established while following a standard protocol that included right-sided heart catheterization (RHC).11 We selected treatment-naive patients with PAH who underwent cardiac

Patient Characteristics

The baseline demographic and hemodynamic data of both the survivors and nonsurvivors are summarized in Table 1. In four survivors and four nonsurvivors, no RHC was performed at 1-year follow-up within 2 weeks of CMR assessment. The nonsurvivors had a mean survival of 2.5 ± 1 years. There was no difference between the survivors and nonsurvivors with respect to age and sex. The nonsurvivor group comprised more patients with connective tissue disease, a greater number of patients in New York Heart

Discussion

We investigated the pathophysiologic changes in RV geometry in patients with PAH in the first year after diagnosis and related the geometric changes occurring during this initial year to subsequent survival. The major finding of this study is the following characterization of RV properties in subsequent nonsurvivors: (1) Longitudinal shortening and transverse shortening are already reduced at baseline; (2) both longitudinal shortening and RV free wall motion stay the same over time in

Conclusions

Progressive RV failure in PAH is associated with a parallel decline in RV longitudinal and transverse free wall displacement until a floor effect is reached for both. A further reduction of RV function is due to progressive leftward septal displacement. Because transverse shortening incorporates both free wall and septum displacement, this parameter can be used to monitor the decline of RV function in end-stage PAH.

Acknowledgments

Author contributions: Mr Mauritz and Dr Kind had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Mr Mauritz: contributed to the study design; data collection, analysis, and interpretation; and manuscript preparation, revision, and final approval.

Dr Kind: contributed to the study design; writing the Matlab software; data collection; and manuscript preparation, revision, and final approval.

Dr Marcus:

References (27)

  • M Humbert et al.

    Treatment of pulmonary arterial hypertension

    N Engl J Med

    (2004)
  • GE D'Alonzo et al.

    Survival in patients with primary pulmonary hypertension. Results from a national prospective registry

    Ann Intern Med

    (1991)
  • OM Ueti et al.

    Assessment of right ventricular function with Doppler echocardiographic indices derived from tricuspid annular motion: comparison with radionuclide angiography

    Heart

    (2002)
  • Cited by (0)

    Mr Mauritz and Dr Kind contributed equally to this article.

    Funding/Support: This studywas financially supported by The Netherlands Organisation for Scientific Research (NWO) Toptalent grant [021.001.120 to Dr Kind] and the NWO Vidi Grant [91.796.306 to Dr Vonk-Noordegraaf].

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).

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