Syndrome d’hyperperméabilité capillaire idiopathique : à propos d’un cas avec atteinte cardiaque

Guillaume, M.; Tolsma, V.; Colombe, Beth; Bosseray, A.; Massot, Christian

doi:10.1016/j.revmed.2010.06.002

Cited by 11 publications

(2 citation statements)

References 10 publications

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“…Cardiac involvement in ISCLS is relatively uncommon. Findings reported in the literature included pericardial effusion [4], ventricular hypertrophy [9], left ventricular dysfunction [20], myocardial stunning [11] and myocardial oedema [21–24]. Echocardiogram showed ventricular wall thickening suggestive of myocardial oedema, cardiac dysfunction and ventricle was underfilled due to distribution of intravascular volume by capillary leak.…”

Section: Introductionmentioning

confidence: 99%

Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome

Leung

Duque

et al. 2019

BMC Pediatr

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BackgroundIdiopathic systemic capillary leak syndrome (ISCLS) is rare, and there has been about 32 cases reported in children worldwide since this disorder was first described in 1960. Clinical guidelines on the management approach stemming from robust scientific evidence are lacking. This case report presents the first reported paediatric case of severe ISCLS with significant myocardial oedema and emphasizes this disease’s impact on a child’s cardiac function.Case presentationA Chinese boy had his first attack of severe hypovolaemic shock that responded to fluid resuscitation when he was 6 years of age. His second attack developed at 8 years of age. He was then transferred to our cardiac unit for refractory hypotensive shock. The patient’s echocardiogram revealed ventricular wall thickening with significant cardiac dysfunction requiring extracorporeal membrane oxygenation support. Subsequently, he made a full recovery, including his myocardial wall thickness and function. The echocardiographic findings suggested myocardial oedema that was transient in nature. Clinical and laboratory investigation from both episodes were compatible with ISCLS.ConclusionISCLS is rare, and therefore there is only a limited understanding on the pathophysiology of this disorder. The current treatment approach is based on a few case reports and series. During the acute phase, optimal supportive management is paramount. Our case highlights the importance of early recognition and consideration for extracorporeal membrane oxygenation support in patients with a life-threatening presentation, as it was lifesaving for this child who suffered myocardial oedema and ventricular dysfunction.

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Section: Introductionmentioning

confidence: 99%

Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome

Leung

Duque

et al. 2019

BMC Pediatr

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“…Although we hypothesize that the primary vascular defect in SCLS resides in peripheral tissues, several case reports have provided evidence of primary myocardial involvement [ 7 , 8 ]. A young man with SCLS and hypotensive shock resistant to fluid resuscitation and inotropic support was described recently, which required extracorporeal life support during the leak phase [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

Increased myocardial extracellular volume in active idiopathic systemic capillary leak syndrome

Ertel

Pratt

Kellman

et al. 2015

Journal of Cardiovascular Magnetic Resonance

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BackgroundThe 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.MethodsFifty-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.ResultsDemographics 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.ConclusionsPatients 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.

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Trastuzumab-Induced Systemic Capillary Leak Syndrome in a Breast Cancer Patient

Zhang

Yang

2013

Pathol. Oncol. Res.

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Systemic capillary leak syndrome (SCLS) is a rare health condition. It is characterized by recurrent episodes of generalized edema and severe hypotension along with hypoproteinemia. The condition is under recognized because of its nonspecific signs and symptoms, and high mortality rate. SCLS triggered by trastuzumab, a target drug for Her2-positive breast cancer patients, has not been previously reported. A 59-year-old Chinese woman, diagnosed with breast cancer with accompanying liver and bone metastasis, was treated with 3 cycles of docetaxel with capecitabine and a regimen of 12 cycles of capecitabine with trastuzumab. The patient developed systemic capillary leak syndrome during the 16th cycle of chemotherapy. Post-diagnosis treatment regimen is also presented in the current case report. SCLS has been previously observed in breast cancer patients. However, SCLS incidence post-chemotherapeutic treatment with trastuzumab has not been reported elsewhere. Hence, our report highlights the need for rigorous investigation of the side effects of trastuzumab usage and the increasing need of insightful diagnosis to manage any incidence of SCLS. The case provides valuable experience for treating the uncommon adverse effects of trastuzumab in Her2-positive breast cancer patients.

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Syndrome d’hyperperméabilité capillaire idiopathique : à propos d’un cas avec atteinte cardiaque

Cited by 11 publications

References 10 publications

Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome

Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome

Increased myocardial extracellular volume in active idiopathic systemic capillary leak syndrome

Trastuzumab-Induced Systemic Capillary Leak Syndrome in a Breast Cancer Patient

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