J Cardiol 2006 Dec; 48（6）: 345 – 352

Fulminant Myocarditis Survivor
After 56 Hours of Non-Responsive
Cardiac Arrest Successfully Returned to Normal Life by Cardiac
Resynchronization Therapy : A
Case Report
Koichi SUGAMURA, MD
Seigo

SUGIYAMA, MD

Hiroaki

KAWANO, MD＊

Eiji

HORIO, MD

Shunichi

ONO, MD＊

Sunao

KOJIMA, MD

Koichi

KAIKITA, MD

Katsuyuki

SAGISHIMA, MD＊

Tomohiro

SAKAMOTO, MD

Michihiro

YOSHIMURA, MD

Yoshihiro

KINOSHITA, MD＊

Hisao

OGAWA, MD, FJCC

Abstract ─────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────────
A 20-year-old female survived fulminant myocarditis with 56 hr of non-responsive cardiac arrest andwas able to resume a normal life with cardiac resynchronization therapy（CRT）. On admission, she had
developed cardiogenic shock refractory to pharmacological intervention. Percutaneous cardiopulmonary
support was initiated with intraaortic balloon pumping. She developed complete cardiac standstill unresponsive to ventricular pacing. After 56 hr of cardiac arrest, ventricular fibrillation occurred and her ventricle started to respond to pacing therapy. She could leave the intensive care unit, although she continued tohave severe heart failure refractory to medical intervention. She presented with paradoxical ventricularmotion with a low cardiac output, so CRT was performed. After the initiation of CRT, her heart failure
symptoms improved and she could return home.
─────────────────────────────────────────────────────────────────────────────────────────────────────────────────────J Cardiol 2006 Dec ; 48
（6）
: 345−352

Key Words
Myocarditis fulminant
Cardiac arrest standstill

Cardiac surgery resynchronization therapy

INTRODUCTION
Fulminant myocarditis is commonly recognized

as acute myocarditis with cardiogenic shock requiring artificial mechanical support systems. 1,2）
Fulminant myocarditis still causes substantial mor-──────────────────────────────────────────────
熊本大学大学院医学薬学研究部 循環器病態学講座，＊侵襲制御医学講座 : 〒 860−8556 熊本県熊本市本荘 1−1−1
Department of Cardiovascular Medicine, ＊ Department of Aggressology and Critical Care Medicine, Graduate School of Medical
Sciences, Kumamoto University, Kumamoto
Address for correspondence : SUGIYAMA S, MD, Department of Cardiovascular Medicine, Graduate School of Medical Sciences,
Kumamoto University, Honjo 1−1−1, Kumamoto, Kumamoto 860−8556 ; E-mail : ssugiyam@gpo.kumamoto-u.ac.jp
Manuscript received July 5, 2006 ; revised August 2, 2006 ; accepted August 17, 2006

345

346

Sugamura, Sugiyama, Kawano et albidity and mortality, especially in children andyoung adults. Before the development of mechanical assist devices, most patients with fulminant
myocarditis died in the acute phase because of
rapidly progressive cardiac decompensation.3）
Acute stage survival of fulminant myocarditis
has been improved by the use of mechanical circulatory assist devices, such as intraaortic balloon
pumping（IABP）, percutaneous cardiopulmonary
support（PCPS）, and left ventricular assist devices. 4−8） Mechanical circulatory support has
become the standard method to bridge patients withintractable heart failure to either transplantation or
myocardial recovery.
Survivors of the acute phase of fulminant
myocarditis have a good long-term prognosis withcomplete reversal of cardiac dysfunction. 9−13）
However, some fulminant myocarditis patients
have recovered from the critical clinical condition
with severe cardiac dysfunction.
We treated a young female who survived theacute phase of fulminant myocarditis with the useof circulatory assist devices and was able to resume
a normal life after cardiac resynchronization therapy（CRT）.
CASE REPORT
A 20-year-old healthy female developed generalfatigue and was admitted to a local hospital.
Electrocardiography showed complete atrioventricular block（AV block）. She was treated with temporary pacing, but atrioventricular conduction did notrecover over 7 days. A permanent pacemaker was
implanted at the primary hospital, but then pacing
failure occurred because of rising threshold andworsening heart failure. Fulminant myocarditis was
suspected, and she was transferred to our university
hospital by ambulance because of cardiogenic
shock.
On arrival, she was alert and responsive with respiratory distress and signs of central cyanosis.
Auscultation detected coarse crackles in the lower
bilateral lung fields, and weak cardiac sounds. Her
temperature was 38.1 °C, systolic blood pressure
was 72 mmHg（under 5μg/kg/min dopamine infusion）, her pulse rate had an irregular rhythm at
105/min, and her respiratory rate was 28/min. Chest
radiography indicated cardiomegaly（cardiothoracic
ratio 52%）, obvious pulmonary edema and nopleural effusion.
Electrocardiography showed idioventricular

Table 1

Arterial blood gas analysis, complete bloodcount and blood chemistry on admission

Arterial blood gas analysis（at O2 15 l/min）
pH

7.329

Hb

13.1 g/dl

Hct

38%

Plt

12.5×104μl

PaO257.7 mmHg

Blood chemistry

PaCO2

42.1 mmHg

AST

1,067 IU/l

HCO3−

21.6 mmol/l

ALT

604 IU/l

BE−4.1 mmol/l

LDH

2,147 IU/l

CK2,866 IU/l

Complete blood countWBC

14,800/μl

Neu

83.9%

TnT

16.3 ng/ml

Lym

8.9%

CRP

5.55 mg/dl

Mon

7.0%

BNP

406.4 pg/ml

Eos

0.1%

CK-MB

123 IU/ltachycardia with widened QRS complexes, left axis
deviation, and a QS pattern in leads Ⅱ, Ⅲ, aⅤF and
Ⅴ4−Ⅴ（Fig.
1−A）. Echocardiography showed a6
thickened left ventricular wall, particularly theanterior wall with reduced systolic contraction, andpericardial effusion without left ventricular cavity
enlargement（Fig. 2−A）. The results of arterial
blood gas analysis, a complete blood count andblood chemistry on admission are shown in Table
1. The arterial blood gas analysis indicated hypoxia
and metabolic acidosis under O2 therapy. The whiteblood cell count and other biochemical markers
were all elevated, especially the creatine kinase（CK）
level.
Mechanical ventilatory support, IABP, and PCPS
were started promptly according to standard operating procedures. Corticosteroid（ 125 mg/day）and
immunoglobulin（5,000 mg/day）therapy were also
started. PCPS was initiated at 3.5 l/min in addition
to the conventional treatments（Fig. 3）. We suspended the pacemaker because pacing stimulation
was not effective due to the rising threshold.
Her myocarditis was resistant to therapy, and her
bradycardia progressed to ventricular standstill（Fig. 1−B）. The day after ventricular standstill
occurred, P waves disappeared and the electrocardiography showed complete cardiac standstill（Fig.
1−C）. Effective systole was not seen on echocardiography and her heart became non-responsive（Fig. 2−B）. At this time, CK and CK in the
myocardial band（CK-MB）reached their peak levels. The value of CK was 9,472 U/l and CK-MB
was 169 U/l. Because of cardiac standstill, her circulatory function completely depended on the
J Cardiol 2006 Dec; 48（6）: 345 – 352

Myocarditis Survivor After Long Cardiac Arrest

A
V1

Ⅰ

B
V2

Ⅱ

Day 3
C
Ⅲ

V3
Day 4

aVR

V4

D

aVL
V5

Day 5
E

aVF

V6
Day 5 post pacing

Day 1
F

G
Ⅰ

V1

Ⅰ

V1

Ⅱ

V2

Ⅱ

V2

Ⅲ

V3

Ⅲ

V3

V4
aVR

V4

aVR

aVL

V5

aVL

aVF

V6

aVF

V5

Pre CRT

V6
Post CRT

Fig. 1 Time course of the electrocardiographic findings
A : Electrocardiography showed idioventricular tachycardia with widened QRS complexes（160 msec）, left
axis deviation, and a QS pattern in leads Ⅱ, Ⅲ, aⅤF and Ⅴ4−Ⅴ6 on admission.
B : A wide QRS like complex was found in the limb leads and a P-wave like pattern in Ⅴ1−Ⅴ2.
C : Cardiac standstill occurred on day 4.
D : Ventricular fibrillation occurred on day 5.
E : Post VVI pacing on day 5.
F : Pre CRT. QRS width was 220 msec.
G : Post CRT. QRS width decreased to 160 msec.
CRT ＝ cardiac resynchronization therapy.

J Cardiol 2006 Dec; 48（6）: 345 – 352

347

348

Sugamura, Sugiyama, Kawano et al

A

B

C

D

Fig. 2 Time course of the echocardiographic findings
M-mode scans at the mid-ventricular level are shown.
A : On admission, echocardiography showed a thickened left ventricular wall, particularly the anterior（interventricular septum 14 mm and posterior wall 9 mm）with a slight reduction in systolic performance（ejection fraction 59%）. The left ventricular end-diastolic diameter was within normal limits and pericardial effusion was present.
B : After ventricular standstill occurred, effective systole was not seen.
C : The patient had severe left ventricular dysfunction（ejection fraction 28%）. The septal wall was thinned
and paradoxical motion was observed.
D : After cardiac resynchronization therapy was performed, the ejection fraction increased by 5% and theparadoxical motion disappeared.

mechanical assist devices. Although termination oflife support was considered at this time, when we
reduced her degree of sedation to check her consciousness level, she awoke and well-preserved
central nervous system function was confirmed. We
decided to continue the on-going treatment.
During this period, we used only mechanicalassist devices without inotropic agents（dopamine,
dobutamine or norepinephrine）
for the avoidance of
cardiac damage. Since 20 hr had passed after herheart stopped，ventricular fibrillation occurred
spontaneously and direct current defibrillation was
attempted（Fig. 1−D）. Although the ventricularfibrillation was converted back to cardiac standstill,ventricular fibrillation continued to recur, and direct
current defibrillation was attempted repeatedly withthe same outcome（cardiac standstill）. Since theoccurrence of ventricular fibrillation suggested therecovery of cardiac muscle excitability, we attempted ventricular pacing which was successful（Fig.
1−E）. Her heart restarted approximately 56 hr
after cardiac arrest.
Although cardiac pacing was restarted, left ventricular motion was still very weak, so mechanicalcardiac support was continued until there was morerecovery of left ventricular function. On day 9, she
was weaned from PCPS and IABP because she met
the weaning criteria 10）and pulmonary alveolar
hemorrhage occurred as a complication of anticoagulant therapy. However, after weaning, several
critical complications（sepsis, pulmonary bleeding,disseminated intravascular coagulation, and acute
J Cardiol 2006 Dec; 48（6）: 345 – 352

Myocarditis Survivor After Long Cardiac Arrest

349

Immunoglobulin 5,000mg/day
Methyl predonisolone 125mg/day
PCPS

Ventricular
standstill（l/min）

Cardiac arrest（56 hr）
PCPS exchange

3.5−4.0

VF

PCPS off

3.0−3.5
2.5−3.0
2.0−2.5DC

1.5−2.0

VVI pacing restart
IABP
（1：1）
Day

1

2

3

4

5

6

7

8

9
IABP off

Fig. 3 Clinical course, treatment and circulatory assist
PCPS was initiated at 3.5 l/min. After cardiac standstill occurred, effective systole was lost and we had toincrease the flow of PCPS. Ventricular fibrillation occurred intermittently（down arrows）and direct current
defibrillation was attempted（up arrows）. However, cardiac standstill was sustained, so we tried cardiac
pacing again on day 5, which was successful and cardiac beats were restarted approximately after 56 hr ofcardiac arrest. On day 9, PCPS and IABP were stopped.
PCPS ＝ percutaneous cardiopulmonary support ; VF ＝ ventricular fibrillation ; DC ＝ direct-current countershock ; IABP ＝ intraaortic balloon pumping. Other abbreviation as in Fig. 1.

renal failure）occurred sequentially. Continuous
hemodialysis filtration was necessary because ofanuria, but she recovered from these complications
and her condition was stabilized.
She was moved from the intensive care unit. Her
consciousness was clear and there was no neurological disorder except for peripheral neuropathy in thelower left leg, which was caused by long-termrecumbency. At that time, her left ventricular ejection fraction was only 28%, plasma brain natriuretic peptide levels were elevated（1,740 pg/ml）, complete AV block was still present, and she was pacemaker dependent. Electrocardiography showed aleft bundle-branch block pattern due to the rightventricular pacing and the QRS width was 220
msec（Fig. 1−F）
. The septal wall was thinned and. Sheparadoxical motion was observed（Fig. 2−C）
had drug-refractory severe heart failure and New
York Heart Association（NYHA）class Ⅳ symptoms. Although all possible medications wereadministered, congestive heart failure was still present and she could not be discharged from the hospital.
After she left the intensive care unit, there wasno further elevation of troponin T or CK levels.
J Cardiol 2006 Dec; 48（6）: 345 – 352

Endomyocardial biopsy of the left ventricle
revealed extensive fibrosis and a few remaining
lymphocytes, which indicated that she was in thehealing phase of myocarditis. In this period, lethal
arrhythmia did not appear. The absence of any
signs of continuous myocardial inflammation suggested that the chronic myocarditis had resolved.
We examined all indicators of secondary myocarditis as far as possible, but the possibility of the secondary myocarditis including sarcoidosis could be
excluded. Repeated measurement of several virus
antibody titers disclosed no evidence of recent viral
infection.
Because of her wide QRS complex, we thoughtthat left ventricular dys-synchrony may have been acontributing factor to her severe heart failure.
Therefore, she was considered a good candidate for
CRT.
Since a permanent pacemaker had already been
implanted, a new coronary venous pacing lead wasadded from the coronary sinus and the generator
was exchanged to the Insync （Model 8040,
Medotronic, Inc ; mode DDD, Lower rate 70
beats/min, AV delay 200 msec）. After CRT was
initiated, the QRS width decreased to 160 msec

350

Sugamura, Sugiyama, Kawano et al

A

B

C

D

Pre CRT

Post CRT

Fig. 4 Chest radiographs and tissue Doppler echo images
Chest radiograph showed the cardiothoracic ratio decreased from 62%（A）to 51%（B）after CRT was initiated. Comparison of the tissue Doppler echo images before（C）and after（D）CRT shows synchronization of
the septal wall
（red arrows indicated）at the lateral wall after CRT.
CTR ＝ cardiothoracic ratio. Other abbreviation as in Fig. 1.

（Fig. 1−G）, left ventricular ejection fraction
increased by 5%, and paradoxical motion disappeared（Figs. 2−D, 4−C, D）. The cardiothoracic.
ratio decreased from 62% to 51%（Figs. 4−A, B）
Plasma brain natriuretic peptide levels also markedly decreased from 555 to 152 pg/ml. NYHA class
improved from Ⅳ to Ⅱ, and she recovered enoughto return to normal life. She has been in good healthwith normal activity of daily life for 10 months
since her discharge.
DISCUSSION
The present 20-year-old female survivor of fulminant myocarditis with 56 hr non-responsive cardiac arrest was able to return to normal life by CRT.
Cardiac arrest is one of the most fatal and severe

complications occurring in patients with fulminant
myocarditis. This case is remarkable for the recovery after a long-lasting（56 hr）period of asystolewith neurological function intact. In many caseswith fulminant myocarditis, good circulatory function was difficult to maintain with mechanical support devices because of several critical complications. In this case, although the 56 hr period of cardiac arrest was complicated, the patient was savedby maintaining suitable circulatory assistance andperiodically retrying cardiac pacing. CRT improved
the cardiac insufficiency and served as a bridge tohelp the patient return home and resume a normal
life.
Asystole, a complete rest period of the heart,
may be necessary to allow the damaged myocardiJ Cardiol 2006 Dec; 48（6）: 345 – 352

Myocarditis Survivor After Long Cardiac Arrest

um to heal and recover adequate function.14）At the
beginning of asystole in our patient, the pacing
pulses failed to capture the ventricles because ofextensive myocardial inflammation. However,myocardial excitability recovered with the onset ofventricular fibrillation, and her ventricles could be
captured by the pacing pulses. It is important that
we do not overlook signs of recovery of cardiac
muscle excitability during cardiac arrest in patientswith fulminant myocarditis.
After becoming asystolic, her circulatory function completely depended on the assist devices, andwe continued circulatory support because her central nervous system function was well preserved.
Therefore, good circulatory support is important tomaintain to allow sufficient time for resolution ofcardiac inflammation and improvement in ventricular function in such cases. We should pay special
attention to prevent multi-organ complications andcirculatory insufficiency.
Although our patient survived the acute phase offulminant myocarditis, her severe cardiac insufficiency（left ventricular ejection fraction 28%）made要351

it difficult to return home. One-year, 5-year, andcumulative survivals after fulminant myocarditis
are dependent upon ejection fraction and pulmonary capillary wedge pressure. 15,16） CRT is
known to cause clinical improvement in patientswith moderate-to-severe heart failure.17）Moreover,
the Cardiac Resynchronization-Heart Failure（CARE-HF）trial showed that CRT reduces morbidity and mortality regardless of the cause of heart
failure.18）We determined that she fit the enrollment
criteria for CARE-HF. This is the first case inwhich CRT was used successfully in a patient withfulminant myocarditis. After the initiation of CRT,
her cardiac function improved and she was able toreturn home and resume a normal life.
Acknowledgements
This study was supported by a Research Grant for Cardiovascular
Disease（17C-2）from the Ministry of Health, Labor, and Welfare,Japan, a Grant-in-aid for Scientific Research（B-17390232 and C17590753）from the Ministry of Education, Science and Culture,
Japan, and the Smoking Research Foundation Grant for Biomedical
Research, Japan.

約

56 時間の心停止から回復し心臓再同期療法により社会復帰しえた
劇症型心筋炎の 1 例
菅村

公一

杉山

正悟

河野

宏明

堀尾

英治

小野

俊一

小 島

淳

海北

幸一

鷺島

克之

坂本

知浩

吉村

道博

木下

順弘

小川

久雄

今回我々は，急性期に 56 時間の心停止を合併しながらも救命でき，慢性期に残存した重度の心機能障害を心臓再同期療法によって改善，社会復帰可能となった劇症型心筋炎症例を経験した．症
例は 20 歳，女性．薬物治療抵抗性の心原性ショックとして当院集中治療部へ紹介入院となった．
臨床経過から劇症型心筋炎と診断し，直ちに大動脈内バルーンパンピング法と経皮的心肺補助法を開始したが，心室ペーシングに反応しない完全な心静止に陥った．56 時間の心停止期間中に心室細動が起こり，それを契機として右室ペーシングに反応がみられ心拍を再開しえた．しかし，慢性期に重度の心機能障害が残存し，薬物治療不応性の心不全が持続した．自己心拍は出現せずペーシ
ングに依存しており，ペーシングによる奇異性運動と心拍出量の低下を認め，心臓再同期療法の適応と考えられた．心臓再同期療法施行後，心不全症状は改善され，社会復帰可能となった．

J Cardiol 2006 Dec; 48（6）: 345−352

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