Jenna M. Dahl and Mosi K. Bennett (2017) Fulminant Lymphocytic Myocarditis. Journal of the Minneapolis Heart Institute Foundation: Fall/Winter 2017, Vol. 1, No. 2, pp. 138-141.
Jenna M. Dahl, APRN, CNP
Mosi K. Bennett, MD, PhD
Minneapolis Heart Institute®, Abbott Northwestern Hospital, Minneapolis, MN
Address for correspondence:
Mosi K. Bennett, MD, PhD
Minneapolis Heart Institute® at Abbott Northwestern Hospital
800 E 28th St.
Minneapolis, MN 55407
Fulminant lymphocytic myocarditis is rare, and affects approximately 200 people per year in the US. It is most frequently diagnosed in males between the ages of 20 and 40 years. Most patients with fulminant lymphocytic myocarditis present with severe heart failure symptoms and arrhythmias and require intensive care. The thirty day mortality rate of fulminant lymphocytic myocarditis is greater than 40%. We describe a case of fulminant lymphocytic myocarditis in a previously healthy young male. The clinical presentation highlights the aggressive nature of this inflammatory cardiomyopathy. The subsequent clinical course demonstrates an integrated medical and surgical approach to the management of cardiogenic shock as well as timely utilization of appropriate mechanical circulatory support.
Keywords: lymphocytic myocarditis, cardiogenic shock
Myocarditis is inflammation of cardiac myocytes with clinical and histopathological manifestations. This immune process can cause decreased myocardial function and is estimated to be associated with more than 25% of all cases of dilated cardiomyopathies. A diagnosis of myocarditis is confirmed by endomyocardial biopsy and is based on established histological, immunological, and immunohistochemical criteria. Myocarditis can be caused by a variety of infectious and noninfectious illnesses. Lymphocytic myocarditis accounts for 54% of cases of myocarditis, and viral infection is the most commonly identified cause. Clinical presentation can vary from a chronic indolent disease that can progress to a dilated cardiomyopathy, to an acute fulminant disease that presents with cardiogenic shock as we describe in this case report. We review this patient’s presentation, differential diagnoses to be considered, and outline the evidence based treatment guidelines for management of fulminant lymphocytic myocarditis, which includes endomyocardial biopsy and early mechanical circulatory support.
A 44-year old man with a history of a recent pneumonia presented to the emergency department complaining of chest and upper back pain. On presentation, his troponin was elevated to 20.5 ng/mL and his electrocardiogram (EKG) showed ST elevation in the inferior leads (Figure 1). An echocardiogram revealed a severely reduced ejection fraction of 10%, a nondilated left ventricle (LV), and a small pericardial effusion. Right ventricle size and function were normal. The patient was emergently taken to the cardiac catheterization lab where a coronary angiogram showed minimal coronary artery disease. A right heart catheterization revealed a severely low cardiac output and elevated filling pressures. Over the next 24 hours, the patient developed ventricular arrhythmias requiring defibrillation, and he was started on amiodarone and lidocaine infusions. He also developed progressive multiorgan system failure and required mechanical ventilation, as well as continuous renal replacement therapy.
Presenting EKG showing inferior ST-segment elevation.
On hospital day 2, cardiogenic shock and hypotension remained refractory to escalating doses of inotropes and vasopressors. He ultimately was placed on venoarterial extracorporeal membrane oxygenation (VA-ECMO) with the addition of an intra-aortic balloon pump. A repeat echocardiogram while on VA-ECMO showed persistently poor left ventricle function and thrombus formation within the left ventricle cavity (Figure 2). The patient was taken to the operating room for placement of a left ventricle apical vent in order to improve left ventricle decompression. The patient also underwent left ventricle endomyocardial biopsy at the time of the left ventricle vent procedure. Endomyocardial biopsy revealed fulminant lymphocytic myocarditis (Figure 3).
Echocardiogram image while on VA-ECMO support shows a dilated LV with marked spontaneous echo contrast in the LV cavity and probable laminar mural thrombus in the apical lateral and inferolateral segments.
Histologic sections of the LV show lymphocytic myocarditis under a. low and b. high magnification. The myocardium is extensively infiltrated by numerous inflammatory cells. Extensive myocyte necrosis is present. The lymphocytic infiltration consists predominantly of c. T cells (CD3+, CD20-) and d. histiocytes (CD68+).
After 10 days on ECMO support, his pulmonary and renal function improved, but he showed no signs of myocardial recovery. The advanced heart failure team emergently evaluated him for advanced therapies and made the decision to transition him to durable mechanical support. He underwent removal of VA-ECMO and implantation of a left ventricular assist device (LVAD; Heartmate II; Thoratec, Pleasanton, CA). He did well postoperatively, and he was discharged from the hospital to a rehabilitation facility 1 month later. After 6 months on LVAD support, he now has minimal heart failure symptoms and his left ventricle ejection fraction has improved to 50%.
Our patient was a previously healthy 44-year old man who presented with chest pain, EKG changes, elevated cardiac enzymes, and cardiogenic shock. Acute coronary syndrome was suspected initially but ruled out by coronary angiogram. Fulminant lymphocytic myocarditis can in fact present as an ST-segment elevation myocardial infarction, either due to coronary vasospasm7–9 or genuine coronary artery disease. A recent study found that 32% of patients diagnosed with lymphocytic myocarditis on autopsy also had evidence of a recent myocardial infarction.10
Mechanical circulatory support, either as bridge to recovery, or as a bridge for possible cardiac transplantation, remains the most effective treatment for cardiogenic shock caused by acute fulminant myocarditis.
Fulminant lymphocytic myocarditis may be self-limiting with an overall good prognosis and resolution of ventricular dysfunction as long as supportive therapy is initiated in a timely fashion.11 Predictors of adverse outcomes in acute lymphocytic myocarditis include the severity of left ventricular dysfunction, as well as the presence of an intraventricular conduction delay.12 Right ventricular systolic function has also been found to be an independent predictor of death or need for myocardial transplant in patients with acute myocarditis.13 Patients with smaller left ventricle dimensions, lack of intraventricular conduction delay, and without cardiogenic shock are more likely to have complete recovery.14
The role for antiviral or immunosuppressive therapies in patients with lymphocytic myocarditis remains unclear. Immunosuppressive therapy may be effective for giant cell myocarditis, sarcoidosis, eosinophilic myocarditis, and myocarditis associated with known autoimmune disease. Current guidelines recommend consideration for immunosuppression only on an individual case basis in infection-negative lymphocytic myocarditis refractory to standard therapy.15
Mechanical support, either as a bridge to recovery or as a bridge for possible cardiac transplantation, remains the most effective treatment for cardiogenic shock caused by acute fulminant myocarditis.16 When evaluating mechanical support options, two important factors for consideration are the anticipated duration of support, as well as need for left ventricular alone, or biventricular support. Our patient did not have right ventricular failure that necessitated biventricular mechanical support. In predominant left ventricular failure scenarios, VA-ECMO has been utilized as the initial method of circulatory support with favorable outcomes.17,18 If needed, temporary right ventricle support can be provided by a paracorporeal right ventricular assist device connected to the right atrium and the pulmonary artery. When long-term durable support is required, options include an implantable left ventricular assist device, or biventricular support with an implantable LVAD on the left and right ventricle, or a total artificial heart.
We decided to use VA-ECMO as our first line temporary mechanical support for refractory cardiogenic shock. An intra-aortic balloon pump was placed to help unload the left ventricle. However, an echocardiogram while on ECMO support demonstrated a severely distended left ventricle and early thrombus formation. Contemporary case series data suggest that left ventricle distention on ECMO can lead to right ventricle failure, and an increased need for biventricular support at time of durable implant, increased risk of pulmonary dysfunction, and increased risk of stroke.19 We elected for a surgically placed left ventricle drainage cannula to decompress the left ventricle. This procedure also provided access to the myocardial tissue for histological analysis that confirmed the diagnosis of lymphocytic myocarditis.
In our case, there was a strong indication for endomyocardial biopsy, with the acute onset of severe left ventricular dysfunction, severe hemodynamic compromise, and ventricular arrhythmias.15,20 Endomyocardial biopsy has a relatively high diagnostic yield in this type of clinical scenario, and can also guide therapy by identifying cases of giant cell myocarditis, which are more likely to respond to immunosuppressive therapy.21–23 Cardiac magnetic resonance imaging (MRI) can also be helpful in diagnosing myocarditis, but MRI imaging requires clinical stability as well as MRI compatible hardware.
In conclusion, we report a case of fulminant lymphocytic myocarditis with severe cardiogenic shock, with the successful utilization of both temporary and durable mechanical support with the intent to bridge to myocardial recovery or cardiac transplantation.
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