PowerPoint Presentation (PDF) - Perfusion.com

A CASE REPORT:
ECMO SUPPORT FOR FULMINANT
MYOCARDITIS
Dafne Andrea Chianella
Division of Cardiovascular Perfusion
College of Health Professions
Medical University of South Carolina

HISTORY OF MYOCARDITIS
• Diagnostic Dilemma:
• Shifting diagnostic criteria
• Complex classification
• Changing pattern of infectious disease
• Consensus definition in 1984: “Dallas Criteria”
• Defined by the American College of Cardiology
• Used for the multicenter NIH myocarditis treatment
trials
• Remain standards histological classification

MYOCARDITIS: DEFINITION
• Myocardial process characterized by the presence of an
inflammatory infiltrate and myocyte damage or
necrosis not typical of the myocardial damage of
ischemic heart disease.
Ventricular
Dysfunction
Cardiogenic Shock
MYOCARDITIS
Acute-Onset Heart
Failure
MI/Stroke
Myocarditis is implicated in sudden cardiac death of
young adults (8.6% to 12%)

• Chest pain
• Arrhythmia
SYMPTOMS
• Shortness of breath, at rest or during physical
activity
• Fluid retention with swelling of legs, ankles and
feet
• Fatigue

CLASSIFICATION
• Etiology
• Ex: viral, bacterial, fungal, protozoal,
parasitic, toxic, hypersensitive,
immunologic syndrome
• Histologically
• Clinical Presentation
• Acute vs. Fulminant

PATHOLOGY OF MYOCARDITIS
• Phase I: Viral Infection
• Phase II: Autoimmunity
• Phase III/IV: Dilated
Cardiomyopathy
Enterovirus
(gut or respiratory tract)
Coxsackieviruse
Adenoviruses
(respiratory tract)
Encephalomyocarditis
virus

Phase I: Viral Infection
• Cardiotropic RNA virus enters host
• Transported to target organ sites (ex. heart)
• Co-receptors aid membrane binding (DAF and CD5 complex)
• Viral genome enters host cell via CAR (coxsackie-adenoviral
receptor)
• Protein
translation

PHASE II: INFLAMMATION
• Inflammatory cells infiltrate the myocardium
• Cytokine release
• (Ex. interferons, interleukins, tumor necrosis factor)
• Macrophages, lymphocytes, and endothelial cell activation
• Inflammatory mediators = myocarditis
• Vascular endothelium disrupted:
• Endothelial dysfunction
• Vasoconstriction
• Necrosis of myocardial tissues

PHASE III: CELL-MEDIATED IMMUNITY
• Recruitment of immune cells to infected myocardium
• T helper cells + cytotoxic T clear the virally infected cells
• Activated T lymphocytes lysis virus-infected cardiomyocytes
• Viral clearing
• Detrimental effects:
• Myocyte signal transduction
• Calcium homeostasis
• Cardiac bioenergetics

RESOLUTION:
PHASE 4: RESOLUTION OR DILATED
CARDIOMYOPATHY
• Virus effectively cleared
• Myocardial healing and
recovery
CARDIOMYOPATHY:
• Persistent activation of T cells
• Continued antibody-mediated
myocyte destruction
• Ventricular dysfunction
• Fulminant myocarditis or
Chronic persistent myocarditis
• Fibrosis/ necrosis
• Ventricular dilatation
• End-stage heart failure.

FIGURE 1 PATHOGENESIS OF VIRAL MYOCARDITIS CAN BE DIVIDED INTO FOUR PHASE S

Case Report

HISTORY & PHYSICAL
• 24 year old African American Female
• Ht: 167.59 cm
BSA: 1.75m²
• Wt: 68.14 kg
• Presented to ER
• Chest pain X3 days
• Fever, diaphoresis, nausea, vomiting
• ST elevation
• Troponin of 92.5 ng/mL on admission

LEFT HEART CATHETERIZATION
• Normal coronary arteries
• Diffuse left ventricular hypokinesis
• EF = 25%
• LVEDP 30mmHg

ICU AND RIGHT HEART CATH
• VTach with loss of consciousness
• Cardioversion with 200 joules and return to sinus rhythm
• Amiodarone and Norepinephrine administered
• Intermittent complete heart block
Right Heart Catheterization:
• Placement precordial pacemaker and IABP
• PCWP= 22 mmHg
• PAP mean= 23 mmHg
Echocardiogram:
•Hypokinesis of the LV
•EF= 25% to 40% depending on the heart rate

VA-ECMO REPORT:
• Preoperative diagnosis:
• Cardiogenic shock and myocardial stunning
• Myocarditis with biventricular failure
• Coagulopathy
• Acute renal failure
• 8,000 IU Heparin to pt
• Pump primed with 2 units of PRBC, 1 unit FFP
• Cannulation:
• 23-French femoral venous cannula (right femoral vein)
• 17-French arterial cannula (left femoral artery)

MUSC-ECMO
•Equipment:
•Stockert roller pump
•Jostra Quadrox-D oxygenator w/ Safeline® coating
•Gish biomedical circuit w/ GBS coating
•Safety Devices:
•Super Tygon tubing
•Bubble detector
•Transonic flow probe
•Anti Factor Xa levels measured
•Servoregulation:
•Venous line pressure -40 mmHg
•Arterial line pressure +350 mmHg

MUSC ECMO Circuit :
Quadrox D
Membrane Oxygenator

• Cardiac Stun:
ECHO: DAY 1 OF ECMO
• Third-degree heart block with ventricular
paced rhythm.
• Marked global LV hypokinesis
• Paradoxical septal motion and some minor
regional variability.
• EF < 10%.
• RV hypokinesis, mild hypertrophy.

ECHO: DAY 3 OF ECMO
• Concentric left ventricular hypertrophy
• Increased wall thickness
• Increased myocardial mass index
• Severe global hypokinesis of the LV
• EF= 26%.

ECHO: DAY 6 OF ECMO
• Trial Off- evaluate LV function after clamping
ECMO.
• Hypokinesis of the anterior wall, anterior
septum, and inferior septum
• EF increased to 52%.
• RV function appeared recovered
• After 60 minutes of clamping, there was no
significant change in ventricular performance

ECHO: DAY 7 OF ECMO
• EF= 55%
• RV systolic pressure =14mmHg
• Intraoperative removal of the left femoral
ECMO cannula
• Postoperative Diagnosis:
• Fulminant Myocarditis

6
14
20
34
38
46
54
62
70
82
90
102
110
118
126
134
142
150
Anti-Xa Level (IU/mL)
1.2
Anti-Factor Xa Level on ECMO
1
0.8
0.6
0.4
0.2
0
Hours on ECMO

Cardiac Index (l/min/m²)
1
9
17
25
33
41
49
57
65
73
81
89
97
105
113
121
129
137
145
153
2.5
CARDIAC INDEX ON ECMO
2
1.5
1
0.5
0
Hours on ECMO

fib (mg/dL) 215 191 208 229 233 306 377
Plasma Free HGB
(mg/dL) 40 40 90 100 40 30
Lab Test Initiation Day 1 Day 2 Day 3 Day 4 Day 5 Day 6 Day 7
PRBC units
administered 2 1 2 2 2 0 4 2
PLT units administered 1 0 0 0 0 1 2 1
BUN (mg/dL) 41 81 92 67 51 53 47
Creatinite (mg/dL) 2.4 4.8 5.3 3.5 3.3 3.5 3.5
Average ACT (sec ) 200 210 195 175 160 175 180 185
D-Dimer (mg/L) >20 >20 9.1 9.86 5.26
AT3 % 69
PT (sec) 30.3 26 18.3 18.4 17.3
PTT (sec) 150 118 90 66 108 89 88
INR 2.9 2.4 1.43 1.49 1.38

DISCHARGE
• 8 days S/P ECMO ECHO:
• EF= 69% with improved LV systolic
function
• Peak right ventricular systolic pressure is
34 mmHg

EF (%)
80
Patient EF Through Hospital Course
70
69
60
50
40
30
20
25
mean 33
(25-40)
26
52
55
10
0
8

Day 1 on ECMO
Discharge

CLINICAL PRESENTATION OF
MYOCARDITIS
Acute vs. Fulminant

FULMINANT: DEFINITION
• Fulminant :
• Any event or process that occurs suddenly and
quickly
• With intensity and severity to the point of
lethality.
The word comes from Latin fulmināre,
to strike with lightning.

METHODS OF FM DIAGNOSIS
• Echocardiography and EKG
• Cardiac MRI
• 67 Ga and 111 In antimyosin antibody
scintigraphy,
• Angiography
• Biopsy

• FM:
FM VS ACUTE
• Fever and symptoms of heart failure
• Distinct Prodrome (usually viral)
• Severe cardiovascular compromise
• Ventricular dysfunction that either resolves
spontaneously or results in death.
• Acute:
• Patients present with established ventricular
dysfunction
• May respond to immunosuppressive therapy
• May progress to dilated cardiomyopathy.

LABORATORY EVIDENCE FOR FM VS.
ACUTE
Lab Test
Fulminant
Non-Fulminant
(Acute)
Normal
Creatinine 10.0±5.7 mmmol/L 6.4±3.6 mmol/L
BUN 97.2±44.3 mol/L 79.6±26.5 mol/L
Creatinine Kinase 1708±1217 U/L 617±447 U/L
0.5 to 1.0 mg/dL
7–21 mg/dL
60 and 400
IU/L
Transaminases 1158±595 U/L 189±59 U/L 0.0-45 IU/L
C-reative Protein 590±350 mg/L 340±260 mg/L

FM VS ACUTE
Cardiac Test
Fulminant
Non-Fulminant
(Acute)
Inraventricular Conduction
Delays 73% 25%
Ventricular Arrhythmias 27% 4%
Depressed Left Ventricular
EF% 57% 41%
Further FM lab evidence:
• leukocytosis, eosinophilia, and erythrocyte
sedimentation rate
• troponins and creatinine kinase MB isoenzyme
• Prolonged QRS complex and depressed left ventricular EF

Fulminant
Chronic
HISTOLOGICAL CLASSIFICATION OF FM
Normal
Borderline

Fulminant
Normal Cardiac Muscle
Acute

LONG-TERM OUTCOME OF FULMINANT MYOCARDITIS AS
COMPARED WITH ACUTE (NONFULMINANT) MYOCARDITIS
ROBERTE, MCCARTHY (N ENGL J MED 2000;342: 690-5)
Conclusions
Fulminant myocarditis is a distinct clinical entity with an
excellent long term prognosis. Aggressive hemodynamic
support is warranted for patients with this condition.

MECHANICAL CIRCULATORY SUPPORT FOR
PATIENTS WITH ACUTE-FULMINANT
MYOCARDITIS
(ANN THORAC SURG 2001;71:S73–6) 2001
• An aggressive use of mechanical support is strongly justified.
•Survival (full, BTR, or BTT) approaches 70%.
•Transplantation can often be avoided.

EXPERIENCE AND RESULT OF ECMO IN
TREATING FM WITH SHOCK:
WHAT MECHANICAL SUPPORT SHOULD BE
Conclusion:
CONSIDERED FIRST?
YIH-SHARNG CHEN, MD
J HEART LUNG TRANSPLANT 2005;24: 81–7.
ECMO can be considered the first-line treatment of
mechanical support for FM when the IABP is inadequate or
infeasible.
• Fewer complications
• Easier application
• Biventricular support

ADVANTAGES OF ECMO VS. VAD
AS FIRST-LINE TREATMENT FOR FM
• Easy and rapid setup without sternotomy
• Suitable with CPR
• Weaning to be attempted over several hours to days.
• Bridge to long-term mechanical devices
• Flexibility in ECMO application.
• Cost

CONCLUSION
Teamwork and aggressive treatment lead to the
patients extraordinary recovery

QUESTIONS??

Initial Tn levels were elevated (21.8 11.9 ng/dl) in the patient group. The absolute level of Tn could
not differentiate patients who would survive or not. Nevertheless, daily Tn level over the first 72
hours clearly showed that patients with a steep decline in Tn (6.53 3.73, ranged from 2.494 to
14.8, n 13) were able to be weaned off mechanical support (Figure 1). This finding may suggest
that initially high Tn values do not necessarily indicate irreversible damage to the myocardium;
rather, rapid decline in Tn slope may reflect early recovery of the myocardium. Conversely, initial
Tn levels, even if not particularly elevated, might reflect irreversible damage of the myocardium
without good prospects for recovery if they remain persistently elevated.
Figure 1. Trend of daily troponin
(Tn) levels.
Patients 1 to 5 used Tn T, and the
others used Tn I. There are similarities
in the slope in reversible fulminant
myocarditis, 6.53 3.73 ng/ml/day (n
13;
patient 12 with failed weaning and
patient 10 with only one Tn datum
were excluded from analysis).

Two-dimensional echocardiograms from patients with
fulminant and acute myocarditis at presentation. The top
panels show the parasternal long axis (A) and short axis
(B) views of a 20-year-old man with fulminant myocarditis
who presented after five days of a viral syndrome
followed by acute hemodynamic collapse. Note the severe
ventricular thickening (septal thickness 2.1 cm) but small
ventricular cavity size (LVEDD 2.5 cm). After hemodynamic
support with intravenous inotropic agents and a left ventricular
assist device, this patient recovered near normal left
ventricular function.
Echo of normal heart –
Parasternal View

#3 JOURNAL OF THE AMERICAN
COLLEGE OF CARDIOLOGY VOL. 36,
NO. 1, 2000
ECHOCARDIOGRAPHIC FINDINGS IN
FULMINANT AND ACUTE MYOCARDITIS
G. MICHAEL FELKER, MD,*
CONCLUSIONS:
Fulminant myocarditis is distinguishable
from acute myocarditis by echocardiography.
Patients with fulminant myocarditis exhibit a
substantial improvement in ventricular function at six
months compared with those with acute myocarditis.
Echo has value in classifying patients with
myocarditis and may provide prognostic information.
Figure 1. Echocardiographic findings in patients with fulminant and acute
myocarditis at baseline and six months. (A) Fractional shortening, (B) left
ventricular end-diastolic dimension, (C) septal thickness. *p , 0.01 vs.
acute; †p , 0.01 for interaction between time and type of myocarditis.

This study demonstrated that 70% of patients with fulminant myocarditis supported
by percutaneous ECMO could be saved. Cardiac function was severely
depressed in the acute phase but improved markedly in the chronic phase. The
clinical course in the chronic phase in the patientswith fulminant myocarditis
who were weaned from ECMO was similar to that in patients with non-fulminant
myocarditis.

FAVOURABLE CLINICAL OUTCOME IN PATIENTS WITH CARDIOGENIC SHOCK
DUE TO FULMINANT MYOCARDITIS SUPPORTED BY PERCUTANEOUS
EXTRACORPOREAL MEMBRANE OXYGENATION
YASUHIDE ASAUMI
EUROPEAN HEART JOURNAL (2005) 26, 2185–2192
Figure 2:
Acute changes in left ventricular function
before and immediately
after the support and at weaning from
ECMO in patients with fulminant
myocarditis (F group). (A) Fractional
shortening (B) end-systolic dimentions.
Open circles indicate patients who
were weaned from ECMO and closed
diamonds indicate F patients who were
not weaned from ECMO and died.

• Myocardial Stunning and Hibernation
• Acute, coronary occlusion causes a rapid decline (within seconds) in ventricular
performance. If coronary flow is reestablished, ventricular function will slowly return to
normal. The duration of reduced performance (myocardial stunning), depends on the
duration of the preceding ischemia. Stunning is completely reversible, and therefore there
is no permanent damage to the myocardium. The stunned myocardium, however, is less
responsive to inotropic drugs and cardiogenic shock can result. Stunning is found
following coronary angioplasty, thrombolysis, coronary vasospasm, and cardiopulmonary
bypass.
• Myocardial hibernation is a term used to describe the condition in which regional cardiac
function is depressed due to chronic ischemia. Restoration of normal coronary flow (e.g.,
by coronary bypass) will restore normal function in the affected region.
• The mechanisms of stunning and hibernation are not completely understood, but there is
evidence that it is related to impaired Ca ++ handling by the sarcoplasmic reticulum as well
as due to damage caused by oxygen free radicals.

SPINALE IDEAS
• Myocardial stanning
• Dysfunction not death yet hybernating myocard

MAJOR ETIOLOGICAL CLASSIFICATIONS OF
MYOCARDITIS
Viral
Bacterial
Fungal
Protozoal
Adenovirus
Coxsackievirus
HCV
HIV
Mycobacterial
Streptococcal species
Mycoplasma pneumoniae
Treponema pallidum
Aspergillus
Candida
Coccidiodes
Cryptococcus
Histoplasma
Trypanosoma cruzi
Parasitic
Toxins
Hypersensitivity
Schistosomiasis
Larva migrans
Anthracyclines
Cocaine
Interleukin-2
Cocaine
Sulfonamides
Cephalosporins
Diuretics
Digoxin
Tricyclic antidepressants
Dobutamine
Immunologic syndromes
Churg-Strauss
Inflammatory bowel disease
Giant cell myocarditis
Diabetes mellitus
Sarcoidosis
Systemic lupus erythematosus
Thyrotoxicosis
Takayasu’s arteritis
Wegener’s granulomatosis

FIGURE 2.
A CASE OF FULMINANT
MYOCARDITIS, MANAGED
USING INTRA-AORTIC
BALLOON PUMPING AND
MECHANICAL VENTILATOR,
SHOWED THAT ON DAY 7
WHEN THE CPK LEVEL
PEAKED, TH1 DOMINANT
POLARITY WAS
DETECTED. IN THE
HEALING PHASE, AT DAY
14, T-CELLS WITH TH2
FUNCTION DRAMATICALLY
INCREASED AND
OVERCAME TH1 T-CELLS.
AT DAY 20, BOTH TH1 AND
TH2 T-CELLS DECREASED.

SUGGESTED GUIDELINES FOR CASE REPORT PRESENTATIONS
Speakers for each case report will be allotted a 30-minute period on the program. You should plan to spend 20 minutes for the
case report followed by a 10-minute question and answer period guided by the session moderators. This 10 -minute period
is an important part of the case report under discussion and will add to the overall quality of the information conveyed.
Content (at presenters discretion) may be posted on International Perfusion Association (IPA) web site. IPA is a non -profit
perfusion education organization. Please sign at bottom of abstract form to exercise this option. The following guidelines
are recommended from past experience to help you communicate most effectively.
FORMAT
• 1. Title and Introduction Each presenter should give a brief description of the case report and how it pertains to perfusion
practice.
• 2. Case Report The specific case should be described including: patient age, gender, and other pertinent demographics
such as height, weight and body surface area, brief medical history, extracorporeal equipment and circuitry, operative and
perfusion procedure, outcome and possible follow-up.
• 3. Discussion This part could include evolution of the technique, history and bibliographic references. Additional experience
or expansion of alternative techniques or results by other practitioners can be described. The discussion of problems,
speculations or future suggested approaches should be included here.
• 4. Conclusion A brief concluding statement summarizing the case with the major points should be described.
• GENERAL GUIDELINES
• Whenever possible, generic drug names should be used; doses of each drug should be stated when appropriate.
Laboratory values should be expressed in proper units of measure. Extracorporeal equipment should be described (model
number and manufacturer) to give the audience a frame of reference. Medical history of the patient should be brief and
contain only information pertinent to the noteworthy aspects of the case report. The year the case was performed should be

CALL FOR ABSTRACTS
ABSTRACTS FOR CASE REPORTS PRESENTATIONS SHOULD
CONTAIN THE FOLLOWING INFORMATION:
• TITLE: State the title of your presentation.
• INTRODUCTION: Briefly describe the case and how it pertains to perfusion practice.
• CASE REPORT: Include patient age, gender and other pertinent demographics such as
height, weight and body surface area, brief medical history, extracorporeal equipment and
circuitry, operative and perfusion procedure, outcome and follow-up (if available).
• DISCUSSION: This may include evolution of the technique, history and bibliographic
references, expansion of alternative techniques or results, problems, speculations or
future suggested approaches.
• CONCLUSION: The conclusion should summarize the main points.
• QUESTIONS: Submit five questions that can be asked about your presentation at the
Case Reports Meeting.

ABSTRACT
Title: A case report: ECMO support for Fulminant Myocarditis
Introduction:
This case report describes the use of ECMO for biventricular support for a patient diagnosed with fulminant myocarditis (FM).
Case Report:
In this case study, we discuss a patient diagnosed with FM. A 25 year old woman present to the ER at MUSC hospital complaining of three days of chest pain, nausea, and
vomiting. She had a height of 167 cm, weighed 68 kg, and had a BSA of 1.75m². Upon admission and a diagnostic cardiac catheterization, her EF was 69%. After 21 days in the hospital, she was released to go home.
Discussion:
Fulminant myocarditis (FM) is caused by the inflammation of the myocardium that results in ventricular systolic dysfunction and acute-onset heart failure. Symptoms progress
rapidly and are life threatening. Patients who are diagnosed with FM often die of sudden cardiac arrest, arrhythmias, or sever heart failure unless mechanical circulatory support is
introduced. When patients are aggressively treated with biventricular support devices, there is a 70-75% of full recovery from FM. Heart transplantation can often be avoided.
Conclusion:
This paper presents the diagnostic and clinical aspects of an FM patient. A detailed account follows the clinical presentation, methods of diagnosis, patient management,
treatment options, and the inevitable prognosis of FM. Lastly, the pathophysiological, histological, and electrophysical distinguishing features of FM are identified.
Overall, the aggressive treatment, rapid mechanical support, and extraordinary collaboration of the heart team lead to the patient’s excellent recovery.
Questions:
1. Do other ventricular support devices provide the same results as ECMO?
2. How many case of FM occur every year?
3. How did ECMO effect the patient’s other organs?
4. What are the
5. How is FM classified from other forms of myocarditis?