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Increased myocardial extracellular volume in active idiopathic systemic capillary leak syndrome.

Ertel A, Pratt D, Kellman P, Leung S, Bandettini P, Long LM, Young M, Nelson C, Arai AE, Druey KM - J Cardiovasc Magn Reson (2015)

Bottom Line: There was no significant valvular disorder in either group.By contrast, T1 values did not distinguish these three subgroups (1008 ± 40, 978 ± 40, 971 ± 41, respectively, p = 0.2, active v. remission; p = 0.06 active v. controls).Patients with active SCLS have significantly higher myocardial ECV than age-matched controls or SCLS patients in remission, which correlated with histopathological findings in one patient.

View Article: PubMed Central - PubMed

Affiliation: National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.

ABSTRACT

Background: The Systemic Capillary Leak Syndrome (SCLS) is a rare disorder of unknown etiology presenting as recurrent episodes of shock and peripheral edema due to leakage of fluid into soft tissues. Insights into SCLS pathogenesis are few due to the scarcity of cases, and the etiology of vascular barrier disruption in SCLS is unknown. Recent advances in cardiovascular magnetic resonance (CMR) allow for the quantitative assessment of the myocardial extracellular volume (ECV), which can be increased in conditions causing myocardial edema. We hypothesized that measurement of myocardial ECV may detect myocardial vascular leak in patients with SCLS.

Methods: Fifty-six subjects underwent a standard CMR examination at the NIH Clinical Center from 2009 until 2014: 20 patients with acute intermittent SCLS, six subjects with chronic SCLS, and 30 unaffected controls. Standard volumetric measurements; late gadolinium enhancement imaging and pre- and post-contrast T1 mapping were performed. ECV was calculated by calibration of pre- and post-contrast T1 values with blood hematocrit.

Results: Demographics and cardiac parameters were similar in both groups. There was no significant valvular disorder in either group. Subjects with chronic SCLS had higher pre-contrast myocardial T1 compared to healthy controls (T1: 1027 ± 44 v. 971 ± 41, respectively; p = 0.03) and higher myocardial ECV than patients with acute intermittent SCLS or controls: 33.8 ± 4.6, 26.9 ± 2.6, 26 ± 2.4, respectively; p = 0.007 v. acute intermittent; P = 0.0005 v. controls). When patients with chronic disease were analyzed together with five patients with acute intermittent disease who had just experienced an acute SCLS flare, ECV values were significantly higher than in subjects with acute intermittent SCLS in remission or age-matched controls and (31.2 ± 4.6 %, 26.5 ± 2.7 %, 26 ± 2.4 %, respectively; p = 0.01 v. remission, p = 0.001 v. controls). By contrast, T1 values did not distinguish these three subgroups (1008 ± 40, 978 ± 40, 971 ± 41, respectively, p = 0.2, active v. remission; p = 0.06 active v. controls). Abundant myocardial edema without evidence of acute inflammation was detected in cardiac tissue postmortem in one patient.

Conclusions: Patients with active SCLS have significantly higher myocardial ECV than age-matched controls or SCLS patients in remission, which correlated with histopathological findings in one patient.

No MeSH data available.


Related in: MedlinePlus

CMR findings in SCLS. a Pre-and post-contrast T1 maps in the mid-ventricular short axis of a patient with SCLS. Regions of interest drawn within the septum and blood pool are used to calculate ECV. b Mid-ventricular short axis ECV map in the same patient. The color scale for displaying ECV values was chosen so that green colors represent the mean ± 3 standard deviations of normal myocardium from age-matched controls
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Fig1: CMR findings in SCLS. a Pre-and post-contrast T1 maps in the mid-ventricular short axis of a patient with SCLS. Regions of interest drawn within the septum and blood pool are used to calculate ECV. b Mid-ventricular short axis ECV map in the same patient. The color scale for displaying ECV values was chosen so that green colors represent the mean ± 3 standard deviations of normal myocardium from age-matched controls

Mentions: Myocardial ECV measurements were generated in one of two ways, both of which rely on T1 quantification techniques utilizing the MOLLI sequence as described in detail elsewhere [14]. Briefly, pre- and post-contrast T1 images obtained from the MOLLI sequence were motion-corrected and reformatted into a pixel map of T1 values. In our initial approach, average T1 values were quantified from regions of interest drawn manually within the mid-ventricular septum and blood pool on both pre-contrast and post-contrast T1 pixel maps (Fig. 1a). Because the change in myocardial relaxation rate ΔR1 (where R1 = 1/T1) between pre and post-contrast is directly proportional to the extracellular concentration of the GBCA, myocardial ECV can be estimated as follows (assuming contrast equilibrium between blood and myocardium) [10, 11].Fig. 1


Increased myocardial extracellular volume in active idiopathic systemic capillary leak syndrome.

Ertel A, Pratt D, Kellman P, Leung S, Bandettini P, Long LM, Young M, Nelson C, Arai AE, Druey KM - J Cardiovasc Magn Reson (2015)

CMR findings in SCLS. a Pre-and post-contrast T1 maps in the mid-ventricular short axis of a patient with SCLS. Regions of interest drawn within the septum and blood pool are used to calculate ECV. b Mid-ventricular short axis ECV map in the same patient. The color scale for displaying ECV values was chosen so that green colors represent the mean ± 3 standard deviations of normal myocardium from age-matched controls
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4551171&req=5

Fig1: CMR findings in SCLS. a Pre-and post-contrast T1 maps in the mid-ventricular short axis of a patient with SCLS. Regions of interest drawn within the septum and blood pool are used to calculate ECV. b Mid-ventricular short axis ECV map in the same patient. The color scale for displaying ECV values was chosen so that green colors represent the mean ± 3 standard deviations of normal myocardium from age-matched controls
Mentions: Myocardial ECV measurements were generated in one of two ways, both of which rely on T1 quantification techniques utilizing the MOLLI sequence as described in detail elsewhere [14]. Briefly, pre- and post-contrast T1 images obtained from the MOLLI sequence were motion-corrected and reformatted into a pixel map of T1 values. In our initial approach, average T1 values were quantified from regions of interest drawn manually within the mid-ventricular septum and blood pool on both pre-contrast and post-contrast T1 pixel maps (Fig. 1a). Because the change in myocardial relaxation rate ΔR1 (where R1 = 1/T1) between pre and post-contrast is directly proportional to the extracellular concentration of the GBCA, myocardial ECV can be estimated as follows (assuming contrast equilibrium between blood and myocardium) [10, 11].Fig. 1

Bottom Line: There was no significant valvular disorder in either group.By contrast, T1 values did not distinguish these three subgroups (1008 ± 40, 978 ± 40, 971 ± 41, respectively, p = 0.2, active v. remission; p = 0.06 active v. controls).Patients with active SCLS have significantly higher myocardial ECV than age-matched controls or SCLS patients in remission, which correlated with histopathological findings in one patient.

View Article: PubMed Central - PubMed

Affiliation: National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.

ABSTRACT

Background: The Systemic Capillary Leak Syndrome (SCLS) is a rare disorder of unknown etiology presenting as recurrent episodes of shock and peripheral edema due to leakage of fluid into soft tissues. Insights into SCLS pathogenesis are few due to the scarcity of cases, and the etiology of vascular barrier disruption in SCLS is unknown. Recent advances in cardiovascular magnetic resonance (CMR) allow for the quantitative assessment of the myocardial extracellular volume (ECV), which can be increased in conditions causing myocardial edema. We hypothesized that measurement of myocardial ECV may detect myocardial vascular leak in patients with SCLS.

Methods: Fifty-six subjects underwent a standard CMR examination at the NIH Clinical Center from 2009 until 2014: 20 patients with acute intermittent SCLS, six subjects with chronic SCLS, and 30 unaffected controls. Standard volumetric measurements; late gadolinium enhancement imaging and pre- and post-contrast T1 mapping were performed. ECV was calculated by calibration of pre- and post-contrast T1 values with blood hematocrit.

Results: Demographics and cardiac parameters were similar in both groups. There was no significant valvular disorder in either group. Subjects with chronic SCLS had higher pre-contrast myocardial T1 compared to healthy controls (T1: 1027 ± 44 v. 971 ± 41, respectively; p = 0.03) and higher myocardial ECV than patients with acute intermittent SCLS or controls: 33.8 ± 4.6, 26.9 ± 2.6, 26 ± 2.4, respectively; p = 0.007 v. acute intermittent; P = 0.0005 v. controls). When patients with chronic disease were analyzed together with five patients with acute intermittent disease who had just experienced an acute SCLS flare, ECV values were significantly higher than in subjects with acute intermittent SCLS in remission or age-matched controls and (31.2 ± 4.6 %, 26.5 ± 2.7 %, 26 ± 2.4 %, respectively; p = 0.01 v. remission, p = 0.001 v. controls). By contrast, T1 values did not distinguish these three subgroups (1008 ± 40, 978 ± 40, 971 ± 41, respectively, p = 0.2, active v. remission; p = 0.06 active v. controls). Abundant myocardial edema without evidence of acute inflammation was detected in cardiac tissue postmortem in one patient.

Conclusions: Patients with active SCLS have significantly higher myocardial ECV than age-matched controls or SCLS patients in remission, which correlated with histopathological findings in one patient.

No MeSH data available.


Related in: MedlinePlus