Clinical Feature of Japanese Patients with Fulminant Myocarditis—Myocarditis in Japanese Patients

doi:10.4236/ijcm.2011.23041

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International Journal of Clinical Medicine, 2011, 2, 260-263

doi:10.4236/ijcm.2011.23041 Published Online July 2011 (http://www.SciRP.org/journal/ijcm)

Clinical Feature of Japanese Patients with

Fulminant Myocarditis

—Myocarditis in Japanese Patients

Hiroaki Kawano1*, Yasuhiro Nagayoshi2, Sunao Kojima2, Nahoko Kakihara3, Satoshi Sakuma1,

Masumi Uchino1, Aiko Komatsu1, Machiko Asaka1, Mitsuhiro Shimomura1, Hiroshige Yamabe2,

Hisao Ogawa2, Koichi Node1

1Department of Cardiovascular Medicine, Saga University, Saga, Japan; 2Department of Cardiovascular Medicine, Graduate School

of Medical Sciences, Kumamoto University, Kumamoto, Japan; 3Department of Basic Science of Nursing, Division of Function and

Morphology for Nursing, Saga University, Saga, Japan.

Email: *[email protected]

Received April 20th, 2011; revised May 27th, 2011; accepted June 7th, 2011.

ABSTRACT

Fulminant myocarditis is commonly recognized as an acute myocarditis with cardiogenic shock requiring mechanical

support systems. Before the development of mechanical assist devices, previous study described that most of patients

with fulminant myocarditis die at acute phase because of rapidly progressive cardiac decompensation. To investigate

the feature of myocarditis, we checked consecutive 18 patients (10 men, 8 women, (12 yr - 80 yr)) from April in 2001 to

September 2008 in University. Of these patients 3 needed intra-aortic balloon pumping (IABP) (2 men, 1 woman), 6 did

both IABP and percutaneous card iopulmonary support (PCPS) (2 men, 4 women). Three patients who need both IABP

and PCPS died (2 men, 49 yr and 65 yr, 1 woman, 54 yr) because of uncontrolled bacterial infection . No patients whose

age was under 40 yr (5 men, 4 women) died although a man needed IABP and 3 women did both IABP and PCPS. Six-

teen patients were able to resume a normal life although 1 male patient needed permanent pacemaker, 1 female did

cardiac resynch ronizatio n therapy, and a male and a fema le had left ventricular systo lic dysfunction. In conclu sion, the

young patients had better prognosis than older even if mechanical assist is needed. Although half of patients needed

mechanical assist, the prevention of multi-organ complications including bacterial infection is one of the most impor-

tant keys in the treatment of fulminant myocarditis.

Keywords: Myocarditis, Mechanical Support, Immunosuppressive Therapy, Immunomodulation Therapy

1. Introduction

Myocarditis is one of the most challenging diagnoses for

cardiologists. The entity is rarely recognized, the patho-

physiology is poorly understood, there is no commonly

accepted diagnostic gold standard and all current treat-

ment is controversial. The prevalence of myocarditis has

been found to be up to 42% in case of unexplained death

in people aged 35 or younger [1,2].

Acute myocarditis, especially fulminant myocarditis,

still causes substantial morbidity and mortality, espe-

cially in children and young adults. Fulminant myocardi-

tis is commonly recognized as an acute myocarditis with

cardiogenic shock requiring artificial mechanical support

systems [3,4]. Recently, because of the use of steroids

and immunoglobulin as well as mechanical circulatory

assist devices, such as intra-aortic balloon pumping

(IABP), percutaneous cardiopulmonary support (PCPS),

and left ventricular assist devices, the acute stage sur-

vival of fulminant myocarditis have been improved [5-8].

Mechanical circulatory support has become the standard

therapy to bridge patients with intractable heart failure to

either transplantation or myocardial recovery.

In the present study, we report the feature of Japanese

patients with fulminant myocarditis who hospitalized be-

tween 2001 and 2008 in our institute.

2. Methods

Before the development of mechanical assist devices,

previous study described that a lot of patients with ful-

minant myocarditis die at acute phase because of rapidly

progressive cardiac decompensation [10]. From April in

2001 to September 2008, we had 18 patients with fulmi-

Clinical Feature of Japanese Patients with Fulminant Myocarditis261

nant myocarditis who had severe heart failure (New York

Heart Association IV) in our institute. All had cardiac

symptoms and clinical suspicion of myocarditis in the

absence of known non-inflammatory causes including

coronary artery disease. We did transthoracic Doppler

echocardiography, complete heart catheterization and

selective coronary angiography in all patients. Ejection

fraction (EF) was measured by Simpson methods with

echocardiography.

Endomyocardial biopsy is the critical test to confirm

the diagnosis. Unfortunately, we were not able to per-

form endomyocardial biopsy in all patients because of

the unstable circulation due to myocarditis. Thus, myo-

carditis had been diagnosed with clinical symptoms,

physical examinations, heart catheterization, electrocar-

diogram and echocardiography [9]. All subjects gave

written, informed consent, and the study was approved

by the ethics committee at our institution.

3. Results

The etiology of myocarditis had been suspected viral

infection. Of 18 patients, 3 needed intra-aortic balloon

pumping (IABP) (2 men, 1 woman), 6 did both IABP

and percutaneous cardiopulmonary support (PCPS) (2

men, 4 women) as shown in Table 1. Three patients who

need both IABP and PCPS died (2 men, 49 yr and 65 yr,

1 woman, 54 yr) because of uncontrolled bacterial infec-

tion. No patients whose age was under 40 yr (5 men, 4

women) died although a man needed IABP and 3 women

did both IABP and PCPS. Sixteen patients were able to

resume a normal life although 1 male patient needed

permanent pacemaker, 1 female did cardiac resynchroni-

zation therapy, and a male and a female had left ven-

tricular systolic dysfunction.

4. Discussion

Multiple infectious etiologies had been implicated as the

cause of myocarditis, the most common being viral [11].

In the majority of patients, active myocarditis remains

unsuspected because the cardiac dysfunction is subclini-

cal and self-limited. In the present study, the etiology of

myocarditis had not been able to defined, but been sus-

pected viral infection in all study patients.

The cornerstone of therapy for patients with acute

myocarditis is supportive care [11]. Serial echocardi-

ography, right heart catheterization, etc., are all em-

ployed determine native cardiac reserve and suitability

for device choose. We present the feature of 18 patients

with fulminant myocarditis including a 20-year old

woman with long term non-responsive cardiac arrest re-

turning to normal life. In many cases with fulminant

myocarditis, it is difficult to maintain good circulatory

function with mechanical support devices, including ven-

tilation, pacing, IABP, PCPS, etc., because of several

critical complications. Thus, it is important to maintain

good circulatory support to allow sufficient time for

resolution of cardiac inflammation and improvement in

ventricular function. We had to pay much attention to

prevent multi-organ complications and circulatory insuf-

ficiency, and to control bacterial infection control during

Table 1. Patients characteristics.

No. Age (yr) Mechanical support Immunosuppressive & Immunomodulation therapy EF(%)

man

1 18 23

2 22 22

3 24 Corticosteroid 23

4 34 IABP (for 4 days) Corticosteroid + IVIG 12

5 38 IABP (for 9 days) Corticosteroid + IVIG 11

6 49 PCPS + IABP (for 11 days) 12 (dead)

7 59 23

8 65 PCPS (for 8 days) 20 (dead)

9 68 Pacing (for 3 days) 31

10 80 Pacing → permanent pacemaker 23

woman

1 12 PCPS + IABP (for 5 days) Corticosteroid + IVIG (see Figure 1) 24

2 16 PCPS + IABP + Pacing (for 7 days) Corticosteroid + IVIG 12

3 20 PCPS + IABP + Pacing (for 9 days)

→ cardiac resynchronization therapy

Corticosteroid + IVIG 13

4 30 20

5 49 21

6 54 PCPS + IABP (for 9 days) Corticosteroid + IVIG 13 (dead)

7 56 14 (dead)

8 71 IABP (for 7 days) Corticosteroid + IVIG 19

EFejection fraction; IABP: intra-aortic balloon pumping; IVIG: High-dose intravenous immune globulin; PCPS: percutaneous cardiopulmonary support. :

Clinical Feature of Japanese Patients with Fulminant Myocarditis

262

the mechanical support in order to resume a normal life.

Thus, several specialists, such as cardiologists, anesthe-

siologists, respiratory doctors, infection control teams,

mechanical support technicians, and skilful nurses, were

essential to care patients with fulminant myocarditis in

intensive care unit.

The treatment of myocarditis is controversial, and no

specific therapeutic regimen has been established [11].

All patients with myocarditis should receive standard

heart failure therapy. Immunosuppressive therapy has

been proposed to treat myocarditis in both children and

adults. The results of an international trail, the Myocardi-

tis Treatment Trial in 1995, showed that there was no

difference in heart function in the treated with immuno-

suppressive agents or placebo [12]. Despite the negative

results of this trail, the authors indicated that patients

who responded to immunosuppressive therapy appeared

to have higher initial ejection fraction and shorter dura-

tion of illness. High-dose intravenous immune globulin

(IVIG) has both immune modulatory and antiviral effects

[13-15]. However, there was no difference between pla-

cebo and IVIG. Thus, there was no benefit of immuno-

modulation. In the present study, some patients had im-

munosuppressive therapy, some had IVIG and some had

both. The present small study cannot conclude the effects

of the immunosuppressive and the immunomodulation

therapy for fulminant myocarditis. Taken as a whole, the

present small study together with previous trials, do not

support the routine use of immunosuppressive and im-

munomodulation therapy for fulminant myocarditis [16].

These data suggest that subgroup with ongoing fulminant

myocarditis, especially in the patients who need me-

chanical cardiac support, can be more likely to benefit

from immunosuppressive and immunomodulation ther-

apy, although no uniform methodology yet exists to

identify them.

Figure 1. Endomyocardial tissue of 12 yr woman with myo-

carditis (case 1). (Hematoxylin and Eosin stain).

In conclusion, the young patients had better prognosis

than older even if mechanical assist is needed. Although

half of patients needed mechanical assist, the prevention

of multi-organ complications including bacterial infec-

tion is one of the most important keys in the treatment of

fulminant myocarditis.

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