Role Of Immunomodulators And Early Mechanical Circulation Support In A Life Threatening Presentation Of Fulminant Lymphocytic Myocarditis

Abstract

Introduction Lymphocytic Myocarditis is a rare cause of myocarditis with an incidence of 22 cases per 100,000. There is limited data in terms of role of immunomodulators and protocolized initiation of mechanical circulation support (MCS). We present a case to emphasize early recognition and prompt initiation of MCS to manage the hemodynamic derangements caused by fulminant myocarditis. Case Description A 19-year-old female with no significant past medical history presented to the ED due to a four-day history of chest pain, dyspnea, fatigue, and palpitations associated with leukocytosis, elevated lactate, and troponins. Initial EKG and telemetry showed SVT with heart rate in the 140s. Treatment with diltiazem led to hypotension, syncope, and clinical deterioration necessitating a transfer to the CVICU. Initial echocardiogram showed mild left ventricular thickness due to edema, minimal LV enlargement, and estimated ejection fraction at 25%. Patient's right heart catheterization showed a CI of 1.09 with a SVR of 2275 dynes on ionotropic support. Due to third degree heart block and junctional escape rhythm, a transvenous pacemaker was placed. Intra-aortic balloon pump (IABP) was placed to unload the left ventricle and endomyocardial biopsy (EMB) was performed. Patient further deteriorated due to development of refractory sustained ventricular tachycardia (VT) unresponsive to defibrillation. A decision was made to escalate to peripheral veno-arterial extracorporeal membrane oxygenation (ECMO). In addition, patient also developed multiorgan failure; AKI, altered mental status and shock liver which were monitored closely. EMB showed extensive lymphocytic infiltration, myocardial edema associated with myocardial cell necrosis and patient was diagnosed with lymphocytic myocarditis. Patient was initiated on 0.5 mg per kg of IVIG for two days and scheduled steroids followed by a taper. Her lactate cleared after 48 hours of ECMO, BNP and ALT down-trended significantly and creatinine improved subsequently. She was weaned off ECMO, and eventually IABP and inotropes. She was downgraded and eventually discharged after a total stay of 12 days. Conclusion Though there is scarce data regarding management of myocarditis, our case illustrates improvement in hemodynamics with prompt initiation of IVIG and steroids combined with ECMO support. We advocate quick escalation to MCS before the electrical storm sets in place from cardioversion of ventricular arrythmias. Additionally, it emphasizes the role of bedside echocardiogram, troponins, BNP, and EKG in an ED setting for early recognition and timely initiation of hemodynamic support can be lifesaving. The role of EMB is paramount. Lymphocytic Myocarditis is a rare cause of myocarditis with an incidence of 22 cases per 100,000. There is limited data in terms of role of immunomodulators and protocolized initiation of mechanical circulation support (MCS). We present a case to emphasize early recognition and prompt initiation of MCS to manage the hemodynamic derangements caused by fulminant myocarditis. A 19-year-old female with no significant past medical history presented to the ED due to a four-day history of chest pain, dyspnea, fatigue, and palpitations associated with leukocytosis, elevated lactate, and troponins. Initial EKG and telemetry showed SVT with heart rate in the 140s. Treatment with diltiazem led to hypotension, syncope, and clinical deterioration necessitating a transfer to the CVICU. Initial echocardiogram showed mild left ventricular thickness due to edema, minimal LV enlargement, and estimated ejection fraction at 25%. Patient's right heart catheterization showed a CI of 1.09 with a SVR of 2275 dynes on ionotropic support. Due to third degree heart block and junctional escape rhythm, a transvenous pacemaker was placed. Intra-aortic balloon pump (IABP) was placed to unload the left ventricle and endomyocardial biopsy (EMB) was performed. Patient further deteriorated due to development of refractory sustained ventricular tachycardia (VT) unresponsive to defibrillation. A decision was made to escalate to peripheral veno-arterial extracorporeal membrane oxygenation (ECMO). In addition, patient also developed multiorgan failure; AKI, altered mental status and shock liver which were monitored closely. EMB showed extensive lymphocytic infiltration, myocardial edema associated with myocardial cell necrosis and patient was diagnosed with lymphocytic myocarditis. Patient was initiated on 0.5 mg per kg of IVIG for two days and scheduled steroids followed by a taper. Her lactate cleared after 48 hours of ECMO, BNP and ALT down-trended significantly and creatinine improved subsequently. She was weaned off ECMO, and eventually IABP and inotropes. She was downgraded and eventually discharged after a total stay of 12 days. Though there is scarce data regarding management of myocarditis, our case illustrates improvement in hemodynamics with prompt initiation of IVIG and steroids combined with ECMO support. We advocate quick escalation to MCS before the electrical storm sets in place from cardioversion of ventricular arrythmias. Additionally, it emphasizes the role of bedside echocardiogram, troponins, BNP, and EKG in an ED setting for early recognition and timely initiation of hemodynamic support can be lifesaving. The role of EMB is paramount.