A 51-year-old woman presented to the emergency department complaining of progressive dyspnea on exertion over the last few days, palpitations and fever. She reported sore-throat and cold symptoms 10 days before admission. Patient's medical history was significant for active smoking and thalassemia minor. On physical examination, she appeared anxious and asthenic; her pulse was 150 beats per minute, regular and narrow. Her blood pressure was 70/40 mmHg with signs of peripheral hypoperfusion. She was tachypneic (28 breaths per minute), her saturation of oxygen on room air was 93% and bilateral basal crackles were auscultated. Her temperature was 37.4 °C. Initial ECG showed a wide QRS complex tachycardia (Fig. 1a). Mild metabolic acidosis (pH 7.33, lactate 2 mmol/l, base excess -5 mmol/l) was observed on arterial blood gas. Echocardiographic findings revealed a nondilated, severely hypocontractile left ventricle (LV) (ejection fraction 30%), with increased diffuse wall thickness (septal and posterior wall, 12 mm). Right ventricular (RV) dysfunction was present. Synchronized electrical cardioversion was attempted twice but was not effective in restoring sinus rhythm. An important release of cardiac biomarkers [high sensitivity-cardiac troponin I (hs-cTnI) 24535 ng/l, normal value (nv) <11 ng/l; creatinine kinase-MB 109 ng/ml, nv <6 ng/ml; myoglobin 339 ng/ml, nv <65 ng/ml] was observed with mild elevation of inflammatory markers (leukocytes 12.7 × 103/μl; C-reactive protein 2.1 mg/dl, nv <0.5 mg/dl) (Supplemental Figure 1, Lactate and cardiac troponin T level variations, http://links.lww.com/JCM/A265). Emergency coronary angiography ruled out coronary artery disease, and she was thus admitted to the ICU for suspected fulminant myocarditis. In the first few hours, despite treatment escalation with mechanical ventilation and high-dose vasopressors and inotropes, the patient progressively deteriorated developing a profound cardiogenic shock with severe metabolic acidosis (pH 7.1, lactate 8.1 mmol/l, base excess -12 mmol/l), signs of organ dysfunction and LVEF deterioration (10%). Mechanical circulatory support with intraaortic balloon counterpulsation (IABP) and veno-arterial extracorporeal membrane oxygenator (va-ECMO) was initiated. She was transferred to our heart transplant (HTx) center on day 1 after initial admission to complete diagnostic workup. RV endomyocardial biopsy (EMB) was performed a few hours after transfer (Supplemental video 1, Fluoroscopy-guided right-ventricle endomyocardial biopsy performed on day 1 on mechanical circulatory support with intraaortic balloon counterpulsation and veno-arterial extracorporeal membrane oxygenator, http://links.lww.com/JCM/A262) showing diffuse lymphocytic inflammatory infiltrates (Fig. 1b) and confirming the diagnosis of lymphocytic acute myocarditis. High-dose intravenous pulse steroid therapy (methylprednisolone 1 g once daily for 3 days from days 1 to 3, followed by 1 mg/kg once daily as maintenance dose) was administered, determining a progressive hs-cTnT normalization and functional recovery (LVEF 52%) [Supplemental video 2, Transesophageal echocardiogram (apical 3-chamber view) performed on day 1 compared with transthoracic echocardiogram (apical 4-chamber view) performed on day 7 demonstrating a complete biventricular functional recovery occurring after treatment with intravenous pulse high-dose steroid therapy (methylprednisolone 1 g od for 3 days from day 1 to day 3), http://links.lww.com/JCM/A263]. A right bundle branch block persisted on ECG. Autoimmune disorders were ruled out. Multiplex real-time-PCR for respiratory virus detection on nasopharyngeal swab tested positive for coronavirus-OC43 subtype. She was successfully weaned from va-ECMO on day 6, IABP and inotropes on day 7, extubated on day 8. Immunosuppression was discontinued on day 8. Cardiac MRI (CMRI), performed 14 days after admission, confirmed a complete biventricular functional recovery. Short tau inversion recovery (STIR) sequences revealed diffuse increased signal intensity suggestive of diffuse edema (Fig. 1d); transmural late gadolinium enhancement (LGE) involving LV basal-lateral and basal-inferior walls was observed (Fig. 1e). She was discharged home on day 18 on low-dose betablocker and ace-inhibitor. At 3-month follow-up, the patient was a NYHA-1 class, ECG normalized, hs-cTnT was within normal limits, and CMRI [Supplemental video 3, A comparison of cardiac magnetic resonance imaging performed on day 14 and at 3-month follow-up confirming biventricular functional recovery and showing resolution of left ventricular wall thickening, initially present due to inflammatory edema (left ventricular myocardial mass index 95 vs. 62 g/m2), http://links.lww.com/JCM/A264] showed normal wall thickness, normal biventricular dimension and function, and absence of residual edema (negative STIR sequences) or fibrosis (no LGE; Fig. 1f).
Fulminant myocarditis defines an acute condition because of myocardial inflammation characterized by a rapidly progressive severe hemodynamic compromise and the need for circulatory support.1–3 Once believed to be marked by a benign course, fulminant myocarditis is now recognized to be burdened by a high mortality and HTx rate.4–7 As in the setting of other acute diseases (e.g. ST-elevation myocardial infarction) whose prognosis depends on a correct acute management, fulminant myocarditis deserves bundles of care to promote recovery and survival.2 Correct and rapid fulminant myocarditis identification is essential. Early circulatory support should be promoted, as well as transfer to hub centers, given the rapid clinical deterioration and the potential need for mechanical circulatory support or HTx. Acute phase EMB in fulminant myocarditis should be pursued to confirm diagnosis and rule out giant cell myocarditis, a form requiring an aggressive multimodal immunosuppression regimen because of its dismal outcome.4 The present case further suggests that in virus-triggered lymphocytic myocarditis, high-dose corticosteroids may hamper the severe inflammatory process, favoring rapid functional recovery. Whether a routine viral genome search in myocardial tissue improves patient management guiding immunosuppression therapy in patients with fulminant myocarditis still remains to be proven. Rapid initiation of immunosuppressive treatment (e.g. pulse steroid therapy) may be considered in acute fulminant myocarditis, where early immunosuppression may be crucial. Nevertheless, its role in the acute phase of lymphocytic fulminant myocarditis should be urgently evaluated in clinical trials. We believe that this case serves as an example of a properly managed fulminant myocarditis in the modern era. Finally, this case highlights that different coronavirus subtypes can trigger acute myocarditis, as recently observed also in another case triggered by severe respiratory distress syndrome coronavirus (SARS-CoV)-2 subtype.8
All authors had access to the data and a role in writing this manuscript.
Conflicts of interest
There are no conflicts of interest.
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