Acute reversible cardiac involvement associated with systemic capillary leak syndrome

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

“…Myocardial involvement in Clarkson's syndrome has been previously reported in only two cases on the basis of echocardiographic findings. 4,5 Claessens et al observed reversible biventricular wall thickening, while contractility remained normal. 4 In contrast, both myocardial wall thickening and severe systolic dysfunction were observed in our case.…”

Idiopathic Systemic Capillary Leak Syndrome(SCLS): Case Report and Systematic Review of Cases Reported in the Last 16 years

“…The role of the monoclonal protein found in the sera of many of the cases remains uncertain, with one report demonstrating in vitro endothelial cell apoptosis on exposure to purified monoclonal protein (9), whereas another found no effect (8) T a b l e 1 . Ch a r a c t e r i s t i c s o (12,13). There was accompanying generalized edema in 37 cases with weight gain reported in 10 cases ranging from 2-29 kg (6,9,(14)(15)(16)(17).…”

“…Compartment syndromes or rhabdomyolysis (reflecting leakage into muscles) occurred in 12 cases, some even requiring fasciotomy (4,10,14,18,(22)(23)(24)(25)(26). Pulmonary edema was reported in 8 cases, all in the recovery phase after intravenous fluid replenishment (6,10,13,15,21,24,25,27). Also reported was biventricular enlargement on echocardiography which regressed as the case improved, possibly reflecting leak into myocardial muscle (13)…”

“…Pulmonary edema was reported in 8 cases, all in the recovery phase after intravenous fluid replenishment (6,10,13,15,21,24,25,27). Also reported was biventricular enlargement on echocardiography which regressed as the case improved, possibly reflecting leak into myocardial muscle (13)…”

“…5 (12,17,22,(24)(25)(26) and mechanical ventilation in 8 cases (9,12,13,15,20,23,24,27 (16), Doubek et al (22), Sabbadini et al (24), Kawabe et al (17). Other agents described to be of benefit include prednisone (2 cases) (14,28), verapamil (2 cases) (7,29), IVIG (intravenous immunoglobulin) (3 cases) (11,12), leukotriene modifiers (two cases) (5,21), and plasmapheresis (21).…”