Myocardial involvement in systemic capillary leak syndrome: first demonstration by pathologic findings

Juthier, Françis; Ennezat, Pierre Vladimir; Fornès, Paul; Hachulla, É.; Hatron, P.Y.; Robin, Emmanuel; Prat, Alain; Bouchindhomme, B.; Banfı, Carlo; Fouquet, Olivier; Vincentelli, André

doi:10.1177/2048872612455142

Cited by 18 publications

(18 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Myocardial edema without inflammatory infiltrates was seen in three out of four biopsies reported in the literature, including a post-mortem examination in a patient in the NIH cohort who died of acute SCLS. 13, 14, 33, 56 We have obtained evidence of myocardial edema in active SCLS using cardiovascular MRI (CMRI). 56, 57 Dynamic measurement of extracellular volume (ECV) of heart tissue by CMRI imaging of gadolinium contrast agents is a novel means to detect active vascular leak in vivo.…”

Section: Diagnostic Considerationsmentioning

confidence: 99%

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Druey

Parikh²

2017

Journal of Allergy and Clinical Immunology

145

172

View full text Add to dashboard Cite

In 1960, Dr. Bayard Clarkson described a woman experiencing sporadic, recurrent episodes of shock and anasarca. Plasma from an acute attack induced a “shock”-like syndrome when injected into rats. The enigmatic “Systemic Capillary Leak Syndrome” (SCLS) named for Dr. Clarkson is characterized by transient and severe, but reversible, hemoconcentration and hypoalbuminemia due to leakage of fluids and macromolecules into tissues. Although < 500 cases of SCLS have been reported in the literature since 1960, the condition is probably under-diagnosed due to lack of awareness and a high mortality without treatment. Allergists should be vigilant of this diagnosis since its presentation can resemble more common plasma leakage syndromes including angioedema or systemic anaphylaxis. Although the precise molecular etiology of SCLS remains unknown, substantial advances over the last five years have increased our understanding of SCLS pathogenesis.

show abstract

Section: Diagnostic Considerationsmentioning

confidence: 99%

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Druey

Parikh²

2017

Journal of Allergy and Clinical Immunology

145

172

View full text Add to dashboard Cite

show abstract

“…Therefore, we should have understood the pathogenesis of the SCLS and have transfused the appropriate dose of colloids with crystalloids while monitoring the central venous pressure on admission and performed mechanical ventilation with positive end-expiratory pressure (PEEP) at the latest at the occurrence of the first hemodynamic deterioration. The myocardial involvement associated with the SCLS might be another possibility [ 11 , 29 , 30 ]. Claessens et al showed the reversible biventricular wall thickening, while systolic function remained normal by echocardiography.…”

Section: Discussionmentioning

confidence: 99%

“…The ECG revealed cardiac axial rotation of −30° with nonspecific repolarization abnormalities in the attacks of SCLS, as in our patient [ 11 ]. Juthier et al observed both myocardial thickening and severe systolic dysfunction by echocardiography, and diffuse myocardial interstitial edema without inflammatory infiltrates and myocyte necrosis by endomyocardial biopsies in the shock stage in one patient with SCLS [ 29 ]. In our patient, the autopsy findings did not show myocardial interstitial edema.…”

Section: Discussionmentioning

confidence: 99%

A fatal case of acute progression of generalized edema and simultaneous flash pulmonary edema in a patient with idiopathic systemic capillary leak syndrome: a case report

et al. 2015

View full text Add to dashboard Cite

IntroductionIdiopathic systemic capillary leak syndrome is a rare and fatal disease due to the unexplained episodic attacks of capillary leakage of plasma from the intravascular into the interstitial space. The attack consists of three phases, a prodromal phase, peripheral leak phase and recruitment phase. During the peripheral leak phase, generalized edema, mainly in the trunk and extremities, with hemoconcentration and hypoalbuminemia occurs, while usually the visceral organs like lungs, brain, heart and kidneys seem not to be involved. Treatment of the acute phase is supportive, focusing on adequate but not overzealous fluid resuscitation, because pulmonary edema usually occurs in the recruitment phase.Case presentationA 65-year-old Japanese woman was admitted to our hospital because of severe hypovolemic shock with metabolic acidosis and hemoconcentration and hypoalbuminemia. Although she was considered to be in the peripheral leak phase of idiopathic systemic capillary leak syndrome, which could not be diagnosed during the treatment, the generalized edema worsened further, severe flash pulmonary edema progressed rapidly after fluid resuscitation and she died. The autopsy showed generalized edema, especially alveolar pulmonary edema without endothelial apoptosis.ConclusionsBecause hypovolemic shock and fatal pulmonary edema may progress rapidly together even in the peripheral leak phase of idiopathic systemic capillary leak syndrome, we should keep in mind this rare and fatal disease and recognize the pathophysiology to treat it effectively when the patient has hypovolemia with metabolic acidosis.

show abstract

“…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

View full text Add to dashboard Cite

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.

show abstract

Myocardial involvement in systemic capillary leak syndrome: first demonstration by pathologic findings

Cited by 18 publications

References 6 publications

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Idiopathic systemic capillary leak syndrome (Clarkson disease)

A fatal case of acute progression of generalized edema and simultaneous flash pulmonary edema in a patient with idiopathic systemic capillary leak syndrome: a case report

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

Contact Info

Product

Resources

About