Case Session
SCMR 22nd Annual Scientific Sessions
Arlene Sirajuddin, MD
Attending Physician
National Heart, Lung, and Blood Institute, National Institutes of Health
Bharath Sathya, MD
Advanced Cardiac Imaging Fellow
National Institutes of Health
Kirk Druey, MD
Attending Physician
National Institute of Allergy and Infectious Diseases, National Institutes of Health
Andrew Arai, MD, FSCMR
Chief
NIH/NHLBI
Description of Clinical Presentation:
A 34 year-old man presented to the emergency room in 2016 with flu-like symptoms. He subsequently developed respiratory failure and cardiogenic shock requiring vasopressors and inotropes for hemodynamic support. Echocardiogram showed a left ventricular ejection fraction (LVEF) of 20%. Laboratories revealed: BNP 1419 pg/mL, peak troponin I of 11.53 ng/mL, hemoglobin of 20.5 g/dL, albumin 2.6 g/dL. A diagnosis of myocarditis was made. He spent 4 days on ECMO and diuresed approximately 7 L/day during his hospital stay. He steadily improved and at discharge his hemoglobin and cardiac function had normalized.
In 2018, he presented again with flu-like symptoms. A nearly identical scenario ensued: cardiogenic shock requiring vasopressors, inotropes, and ECMO, elevated troponin I of 24.07 ng/mL, decreased LVEF (nadiring to 5%), elevated hemoglobin of 22.3 g/dL, decreased albumin of 2.2 g/dL. He diuresed approximately 5 L/day. An extensive workup to determine the etiology of the patient’s cardiogenic shock was initiated including laboratory work, imaging with cardiac MR, and myocardial biopsy.
Diagnostic Techniques and Their Most Important Findings:
Physical exam and laboratory workup revealed hypotension, hypoalbuminemia, and hemoconcentration. Serum protein electrophoresis showed monoclonal gammopathy of undetermined significance.
Cardiac MR revealed prolonged native myocardial T1 and myocardial T2 and increased extracellular volume fraction compatible with diffuse myocardial edema. Subtle, fairly diffuse enhancement throughout the myocardium on late gadolinium enhancement images likely represented edema..
Right heart catherization biopsy showed endothelial activation, fibrosis with intracellular lipid droplets, a few CD3+ cells, and many CD68+ cells. There was no evidence of classic lymphocytic myocarditis.
The combination of two discreet episodes of cardiogenic shock following flu-like symptoms with fairly rapid return to normal cardiac function, cardiac MR showing extensive myocardial edema, hemoconcentration and hypoalbuminemia during the acute illness, and monoclonal gammopathy is consistent with systemic capillary leak syndrome.
Learning Points from this Case:
Our patient had the triad of clinical features very distinctive for systemic capillary leak syndrome (SCLS): hypotension, hypoalbuminemia, and hemoconcentration. Monoclonal gammopathy of undetermined significance is present >90% of the time.
SCLS is an uncommon but life-threatening disease characterized by episodic systemic capillary hyperpermeability resulting in leak of protein and fluid from the intravascular space into the interstitium.
Cardiac involvement is extremely common and cardiac MR is important in revealing key findings of diffuse myocardial edema present in SCLS: prolonged native myocardial T1, prolonged myocardial T2, and increased extracellular volume fraction.
Monthly prophylactic treatment with IVIG has been shown to reduce SLCS flares.