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

MedStar author(s):
Citation: Journal of Cardiovascular Magnetic Resonance. 17:76, 2015.PMID: 26310790Institution: MedStar Washington Hospital CenterDepartment: Internal MedicineForm of publication: Journal ArticleMedline article type(s): Comparative Study | Journal Article | Research Support, N.I.H., IntramuralSubject headings: *Capillary Leak Syndrome/di [Diagnosis] | *Capillary Permeability | *Edema, Cardiac/di [Diagnosis] | *Magnetic Resonance Imaging, Cine | *Myocardium/pa [Pathology] | Acute Disease | Adult | Aged | Biopsy | Capillary Leak Syndrome/pa [Pathology] | Capillary Leak Syndrome/pp [Physiopathology] | Case-Control Studies | Chronic Disease | Edema, Cardiac/pa [Pathology] | Edema, Cardiac/pp [Physiopathology] | Humans | Male | Maryland | Middle Aged | National Institutes of Health (U.S.) | Predictive Value of Tests | Prognosis | Stroke Volume | United States | Ventricular Function, LeftYear: 2015Local holdings: Available online from MWHC library: 2008 - presentISSN:
  • 1097-6647
Name of journal: Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic ResonanceAbstract: 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.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.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.All authors: Arai AE, Bandettini P, Druey KM, Ertel A, Kellman P, Leung S, Long LM, Nelson C, Pratt D, Young MFiscal year: FY2016Digital Object Identifier: Date added to catalog: 2016-06-13
Holdings
Item type Current library Collection Call number Status Date due Barcode
Journal Article MedStar Authors Catalog Article 26310790 Available 26310790

Available online from MWHC library: 2008 - present

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.

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.

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.

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