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Severe lupus and cardiogenic shock requiring rescue VA-ECMO: how many sides can fit an elusive coin?
Session:
Casos Clínicos desafiantes 2
Speaker:
João Presume
Congress:
CPC 2023
Topic:
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Theme:
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Subtheme:
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Session Type:
Speaker´s Corner
FP Number:
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Authors:
João Presume; Rita Bello; Daniel Gomes; Sónia Baldo; Rita Carvalho; Miguel Domingues; Joana Pereira; Isabel Botelho; Diogo Domingos; Patrícia Branco; Marisa Trabulo; Catarina Brízido; Christopher Strong; António Tralhão
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Autoimmune disorders have a great diversity of presentations, with cardiac predominance occurring only in a minority of cases. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">A 45-year-old African male with a history of an unclassified autoimmune disorder (earlier acute pericarditis episode with unspecific serum auto-antibodies detected) was admitted with progressive shortness of breath, worsening peripheral edema and localized skin darkening with desquamation. At admission, transthoracic echocardiogram depicted biventricular enlargement and dysfunction, together with moderate pericardial effusion and no significant valvulopathy. 12-lead ECG and negative serial cardiac troponins excluded ACS. The patient rapidly progressed into cardiogenic shock (CS) SCAI stage D with A modifier in the first 24 hours, requiring invasive mechanical ventilation, increasing doses of inotropes and vasopressors, and, ultimately necessitating rescue femoro-femoral VA-ECMO at the referring center, followed by IABP insertion for LV venting. Other relevant initial findings included KDIGO stage 3 AKI with nephrotic proteinuria and an elevated sedimentation rate (>120s). RV endomyocardial biopsy was noticeable for the absence of inflammatory infiltrate, giant cells or cardiomyocyte necrosis. Further laboratory workup revealed low serum C3 and C4 levels and high titer positivity for several auto-antibodies. Such a constellation of findings fulfilled the EULAR criteria for systemic lupus erythematosus (SLE). The patient was initiated on high-dose corticosteroid pulses and mycophenolate mofetil and cardiac function began to slowly improve. Several complications ensued during the management of this patient (Table 1), including the need for subtotal colectomy while under VA-ECMO support due to colonic non-occlusive ischemia. Continued hemodynamic improvement allowed VA-ECMO decannulation and IABP removal after 14 days of support. A first cardiac MRI showed partial recovery of LV and RV ejection fraction with a small amount of mid-septal LGE. T1 and T2 mapping pointed towards unspecific inflammation. Coronary angiography was normal. Renal biopsy was then performed showing stage III lupus nephritis and pauci-immune vasculitis. Renal function and diuresis also improved gradually, allowing the withholding of renal replacement therapy. The patient was discharged after 55 days with significant improvement of cardiac (48% LVEF) and renal dysfunctions (SCr of 1.2mg/dL), with a final diagnosis of CS due to inflammatory cardiomyopathy, SLE flare and vasculitis with multisystemic involvement. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">This highly complex and challenging case emphasizes the importance of acute recognition and referral of cardiogenic shock patients for early mechanical circulatory support. It also highlights the role of this therapy in the stabilization of patients while etiologic workup, disease-specific treatment and management of complications take place.</span></span></p>
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