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Diagnostic dilemmas and management of fulminant myocarditis.


Myocarditis is commonly a diagnosis of exclusion. We report a case of fulminant myocarditis in a patient with cardiogenic shock in whom the initial diagnosis was unclear. Early supportive care including extracorporeal membrane oxygenation and intra-aortic balloon pump were instituted. Complete recovery of cardiac function was achieved. This case highlights the difficulty in diagnosing myocarditis and the benefits of early intensive support.

Key Words: myocarditis, cardiogenic shock, intra-aortic balloon pump, extracorporeal membrane oxygenation


Fulminant myocarditis is an uncommon syndrome and in the initial phase of the illness clinical features are non-specific. As the myocarditis progresses the diagnosis is usually made after excluding other acute illnesses and/or an acute coronary syndrome. There may be a delay in making the correct diagnosis and therapeutic strategies directed at alternate diagnoses may be embarked upon. The outcome of fulminant myocarditis may be one of complete recovery if early supportive care including extracorporeal membrane oxygenation (ECMO) is instituted. This report highlights both the difficulty in making an early diagnosis of fulminant myocarditis in a patient with cardiogenic shock and the potential for complete recovery in cardiac function following intensive supportive measures.


A previously well 43-year-old male presented to a rural hospital with a four-day history of myalgia, cough with minor sputum production, fevers and headaches. He was treated as an outpatient with oral amoxycillin with the provisional diagnosis of an upper respiratory tract infection. He re-presented four days later to the emergency department following two episodes of witnessed brief syncope. The episodes lasted less than 30 seconds and were preceded by dizziness but not associated with palpitations, dyspnoea or chest pain. On arrival he again collapsed and was observed to have myoclonic jerking movements of the limbs with clenching of the jaw. There was no associated urinary incontinence. The seizure-like activity lasted approximately 40 seconds. Intravenous phenytoin was commenced and the Glasgow coma score recovered to 15/15 within three minutes.

The patient was a smoker but was not taking any prescribed medications or illicit drugs.

On examination the pulse rate was regular and 90 bpm. The blood pressure was 160/70 mmHg and the oxygen saturation 90% on room air by digital plethysmography. The initial cardiovascular examination was reported as unremarkable. Neurologic review demonstrated fluctuation in orientation to time and place, with no focal neurological deficit. The electrocardiogram (ECG) showed a first degree heart block with a widened ORS complex with O waves and ST elevation in leads I, 11, III, aVF and V1-4 (Figure 1), suggestive of possible acute myocardial infarction. A CT scan of the brain was performed to exclude intracranial haemorrhage or infarction. The patient became haemodynamically unstable prior to the CT imaging with complete heart block requiring atropine 3 mg and the insertion of a temporary pacing wire. The CT of the brain revealed no acute changes. Although the initial full blood count, creatinine, urea and electrolytes were normal, the cardiac enzymes were elevated: creatine kinase (CK) 1220 (<240 U/1) and troponin (Tn) 122.1 (<0.09, [micro]g/l).

Based on the ECG changes a provisional diagnosis of acute ST segment elevation myocardial infarction was made and thrombolytic therapy with retepluse (2 x 10 units) delivered. There was no subsequent ST segment resolution and the patient continued to deteriorate, developing hypoxia and hypotension. An adrenaline infusion was commenced and the patient intubated before being transferred to a tertiary referral centre.


On arrival the patient was in cardiogenic shock with acute pulmonary oedema and oliguria (creatinine 0.28 mmol/1; normal <0.10 mmol/1). Continuous arteriovenous haemofiltration was commenced. The patient was coagulopathic with an INR of 3.0 (0.8-1.3), APTT of 57 seconds (24-35) and thrombocytopenic --platelets 75,000 (140-400 x [10.sup.3]). Ten units of cryoprecipitate and 4 units of fresh frozen plasma were given. The liver function tests were deranged with a hepatitic picture and elevated transaminases. A Swann-Ganz pulmonary artery catheter was inserted. The cardiac index was 1.6 whilst on infusions of adrenaline (8 [micro]g/min), noradrenaline (20 [micro]g/min) and milrinone (0.1 [micro]g/kg/min).

A coronary angiogram was performed which

showed normal coronary arteries and a left ventriculogram demonstrated global hypokinesis with apical dyskinesis. Complete heart block was present. An intra-aortic balloon pump (IABP) was inserted and the temporary pacing wire advanced to the right ventricular apex.

A transthoracic echocardiogram demonstrated severe biventricular systolic dysfunction, with normal left and right ventricle sizes (left ventricular end diastolic diameter=4.8 cm). The pattern of left ventricular dysfunction was segmental rather than global. Severe tricuspid regurgitation was present with mild pulmonary hypertension (right ventricular systolic pressure 45 mmHg).

Based on the above findings a clinical diagnosis of myocarditis was made. Serology tests for common infectious agents and inflammatory conditions were performed. Despite the insertion of the IABP and continued inotropic supports the patient did not improve. A decision was made to give pulse intravenous methylprednisolone (3 x 500 mg) over the next 24 hours. The cardiac index temporarily increased to 2.6 but deteriorated over the subsequent 12 hours.

ECMO was instituted and a right ventricular myocardial biopsy was taken. ECMO was performed centrally due to the high cardiac output requirements (Abiomed 10 mm aortic cannula with a flow rate of 7.5 1/min). The myocardial biopsy identified changes consistent with fulminant lymphocytic myocarditis (Figure 2).

The clinical condition slowly improved and the patient was successfully weaned off ECMO six days after insertion. A further myocardial biopsy performed three weeks after presentation showed resolving myocardial inflammation with no features of active myocarditis (Figure 2). At this time repeat transthoracic echocardiography identified normal biventricular size and function with mild tricuspid regurgitation. He was discharged home approximately three weeks after initial presentation. Autoimmune, virology and rheumatology investigations were nondiagnostic.



Two forms of myocarditis exist, fulminant which represents 10% of cases of myocarditis and is defined as the development of shock following admission that requires the use of an intra-aortic pump or cardiopulmonary support, and secondly the acute form (1,2). The incidence of myocarditis is reported to be between 0.15 to 0.6% in the community and in people under the age of 40 it has been reported to account for up to 20% of sudden cardiac death (3,4).

This case highlights the difficulty of identifying myocarditis that is often a diagnosis of exclusion based on both clinical history and investigations. The most common reported presenting symptoms of myocarditis include flu-like symptoms (62%), chest pain (35%) and fever (18%) (5). These symptoms are relatively non-specific which explains the initial difficulty in diagnosing myocarditis. Similarly, the investigational findings can also be non-specific, masquerading as other medical pathologies. Typically coronary angiography demonstrates normal coronary arteries and consequently it is the myocardial biopsy that is diagnostic (6). The ECG changes can vary from sinus tachycardia, arrhythmias (complete heart block, AV conduction abnormalities to supraventricular tachycardia, ventricular tachycardia), fascicular blocks, ST segment changes that may be diffuse or generalised making it difficult to differentiate from an acute ischaemic event (5). Similarly the echocardiographic findings may be diffuse or generalised and not uncommonly pericardial effusion can be found (5). Segmental LV dysfunction with preservation of LV size is commons.

Risk factors for the progression to fulminant myocarditis include elevated C-reactive protein (CRP) and creatine kinase (CK), lower left ventricular ejection fraction and intraventricular conduction delay (7). Those with the fulminant course tend to have abnormal Q waves, ST segment elevation and prolonged QRS duration indicating widespread pancarditis (7). Shock may be a presenting feature but it generally develops within the first few days with malignant arrhythmias such as complete heart block or ventricular tachycardia as possible precipitating causes. Patients with acute myocardial infarction generally develop shock on or during the initial presentation.

The patient in this case report was administered thrombolytic therapy based on the ECG changes, though these were not typical for ST segment elevation myocardial infarction and there was no history of chest pain. In the initial rural setting there was no access to acute diagnostic coronary angiography to confirm an acute myocardial infarct highlighting the diagnostic difficulties in presentations of myocarditis.

Management of myocarditis is principally supportive measures in addition to appropriate management of cardiac failure. Supportive measures include IABP, inotropes, ECMO and ventricular pacing. The use of immunosuppressive agents is still controversial. The myocarditis treatment trial involved 111 patients and demonstrated improvement in left ventricular ejection fraction regardless of treatment (8). Furthermore, a recent meta-analysis of five randomised controlled trials found no evidence for the use of immunosuppressive agents in the treatment of inflammatory myocarditis, but there appears to be a trend to favour short-term improvement in left ventricular ejection fraction (9).

Exceptions where immunosuppressive agents have been shown to be beneficial include eosinophilic, granulomatous and lymphocytic myocarditis associated with connective tissue disease (10). In this case the administration of 1.5g of methylprednisolone over 24 hours may have contributed to the improvement in the cardiac index in the early stage of his management. The use of supportive measures, initially the IABP and subsequently ECMO, may have had a greater impact on the patients' final outcome compared to the administration of steroids. These supportive measures have been previously found to be effective in management of fulminant myocarditis (11-14). The use of percutaneous ECMO in the management of patients with fulminant myocarditis and cardiogenic shock has been shown to lead to more favourable clinical recovery and outcome (15).

Interestingly the long-term prognosis for patients who present with fulminant myocarditis is more favourable compared to that of the less severe form with the 11-year survival after biopsy without transplantation being 93% for the fulminant group compared to 45% in the less severe form (acute group) who generally developed cardiomyopathy requiring on-going treatment(16,17).

This case highlights the difficulties associated with the initial diagnosis of myocarditis, especially in nonspecialist centres where the definitive diagnostic tool of myocardial biopsy is not readily available. The potential for rapid clinical deterioration and need for intensive supportive measures also highlights the need to consider early specialist centre referral in these patients.


We would like to thank Professor Catriona McLean, The Alfred Hospital, Melbourne, for preparation of the myocardial biopsy slides for publication.

Accepted for publication on September 18, 2006.


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(4.) Kawai C. From myocarditis to cardiomyopathy: mechanisms of inflammation and cell death: learning from the past for the future. Circulation 1999; 99:1091-1100.

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(7.) Kato S, Morimoto S, Hiramitsu S, Uemura A, Ohtsuki M, Kato Y et al. Risk factors for patients developing a fulminant course with acute myocarditis. Cite J 2004; 68:734-739.

(8.) Mason JW, O'Connell JB, Herskowitz A, Rose NR, Mcmanus BM, Billingham ME et al. The myocarditis treatment trial investigators. A clinical trial of immunosuppressive therapy for myocarditis. N Engl J Med 1995; 333:269-275.

(9.) Liu C, Junmin C, Liu K. Immunosuppressive treatment for inflammatory cardiomyopathy. Int Heart J 2005; 46:113-122.

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(11.) Oakley CM. Myocarditis, pericarditis and other pericardial diseases. Heart 2000; 84:449-454.

(12.) Chen YS, Yu HY, Huang SC, Chin KM, Lin TY, Lai LP et al. Experience and result of extracorporeal membrane oxygenation in treating fulminant myocarditis with shock: what mechanical support should be considered first? J Heart Lung Transplant 2005; 24:81-87.

(13.) Grinda J-M, Chevalier P, D'Attellis N, Bricourt M-O, Berrebi A, Guibourt P et al. Fulminant myocarditis in adults and children: bi-ventricular assist devise for recovery. Eur J Cardiothorac Surg 2004; 26:1169-1173.

(14.) Chen YS, Wang MJ, Chou NK, Han YY, Chin IS, Lin FY et al. Rescue for acute myocarditis with shock by extracorporeal membrane oxygenation. Ann Thorac Surg 1999; 68:2220-2224.

(15.) Asaumi Y, Yasuda S, Morii I, Kakuchi H, Otsuka Y, Kawamura A et al. Favourable clinical outcome in patients with cardiogenic shock due to fulminant myocarditis supported by percutaneous extracorporeal membrane oxygenation. Eur Heart J 2005;26:2185-2192.

(16.) McCarthy RE 3rd, Boehmer JP, Hruban RH, Hutchins GM, Kasper EK, Baughman KL et al. Long-term outcome of fulminant myocarditis as compared with acute (nonfulminant) myocarditis. N Engl J Med 2000; 342:690-695.

(17.) D'Ambrosio A, Patti G, Manzoli A, Sinagra G, Di Lenarda A, Silvestri F et al. The fate of acute myocarditis between spontaneous improvement and evolution to dilated cardiomyopathy: a review. Heart 2001; 85:499-504.

W. AHMAR *, A. LEET ([dagger]), J. MORTON ([double dagger])

Departments of Cardiology, The Melbourne Heart Centre, The Royal Melbourne Hospital and The Alfred Hospital, Melbourne, Victoria, Australia

* B.Sc. (Hons), Ph.D., M.B., B.S., Cardiology Registrar, Departments of Cardiology, The Melbourne Heart Centre and The Royal Melbourne Hospital.

([dagger]) F.R.A.C.P, Cardiology Department, The Alfred Hospital.

([double dagger]) PhD FRACP, Department of Cardiology, The Melbourne Heart Centre, The Royal Melbourne Hospital.

Address for reprints: Dr Walid Ahmar, The Melbourne Heart Centre, Department of Cardiology, The Royal Melbourne Hospital, Grattan St, Parkville, Vic. 3050.
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Article Details
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Title Annotation:Case Report
Author:Ahmar, W.; Leet, A.; Morton, J.
Publication:Anaesthesia and Intensive Care
Article Type:Clinical report
Geographic Code:8AUST
Date:Feb 1, 2007
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