Acutemyopericardial Syndromes: Ali Farzad,, Jeffrey M. Schussler
Acutemyopericardial Syndromes: Ali Farzad,, Jeffrey M. Schussler
Acutemyopericardial Syndromes: Ali Farzad,, Jeffrey M. Schussler
Sy ndromes
Ali Farzad, MDa,b,*, Jeffrey M. Schussler, MDc,d
KEYWORDS
Pericarditis Myocarditis Myopericarditis Perimyocarditis Pericardium Pericardial effusion
Tamponade
KEY POINTS
Myocarditis and pericarditis often present with symptoms and findings that overlap with or mimic
acute coronary syndromes.
Because the treatment of myocarditis and pericarditis is different from that of acute coronary syn-
dromes or myocardial infarction, identification of these and differentiation of them from acute cor-
onary syndromes or myocardial infarction is important.
Cardiac tamponade, which may occur as a sequela of pericarditis, is a potential emergency.
Although it can often quickly be treated, this is predicated on early and accurate diagnosis.
a
Department of Emergency Medicine, Baylor University Medical Center, 3500 Gaston Avenue, Dallas, TX
75246, USA; b Department of Emergency Medicine, Texas A&M College of Medicine, 3302 Gaston Avenue, Dal-
las, TX 75246, USA; c Department of Cardiology, Baylor University Medical Center, Baylor Jack and Jane Ham-
ilton Heart and Vascular Hospital, 621 Hall Street, Dallas, TX 75226, USA; d Division of Cardiology, Department
of Medicine, Texas A&M College of Medicine, 3302 Gaston Avenue, Dallas, TX 75246, USA
* Corresponding author. Department of Emergency Medicine, Baylor University Medical Center, 3500 Gaston
Avenue, Dallas, TX 75246.
E-mail address: [email protected]
serosal surface that contacts pericardial fluid in metabolic functions of the pericardium are sum-
the pericardial cavity and an opposite surface, marized in Box 1.
which lines the fibrous pericardium (Fig. 1).
The pericardium separates the epicardium from PATHOPHYSIOLOGY OF THE PERICARDIUM
other mediastinal structures and attaches to the
diaphragm, sternum, and other structures to limit Pericardial heart disease comprises mainly peri-
displacement of the heart within the chest during carditis, which may be an acute, subacute, or
respiration or movement.2 The pericardium also chronic fibrinous, noneffusive, or exudative pro-
protects the heart by serving as a physical barrier cess.2 Response to injury is limited to exudation
to prevent the spread of infection or malignancy.3 of fluid, fibrin, or inflammatory cells. Adhesions
The normal pericardial space contains a small may result during healing that may cause oblitera-
amount of fluid (w25–50 mL of plasma ultrafil- tion of the pericardial space, and later calcifica-
trate), which lubricates the serosal surface of the tion.2 Complications include tamponade and
parietal and visceral layers to reduce friction dur- its variants, and constriction, which may be acute,
ing cardiac activity. This fluid equalizes gravita- subacute, or chronic fibrinous.2 Despite a
tional, hydrostatic, and inertial forces over the limited number of clinical syndromes, the pericar-
heart to maintain transmural cardiac pressures.2 dium is affected by virtually every category of
The pericardium is also metabolically active and disease, including infectious, neoplastic, immune-
known to produce hormonal modulators of neuro- inflammatory, metabolic, iatrogenic, traumatic,
transmission and myocardial contractility. The and congenital causes.
pericardium fixes the heart to the mediastinum
and maintains ventricular compliance to limit dila- BACKGROUND/DEFINITIONS/CRITERIA
tation of cardiac chambers. Moreover, the pres-
sure in the pericardial cavity is subatmospheric, Clinically, acute pericarditis and myocarditis
facilitating atrial filling and maintenance of cardiac commonly coexist. The degree of their respective
pressure.2 involvement in disease is variable, giving rise to
Nevertheless, the pericardium is not essential terminology that attempts to accurately describe
for life and few adverse consequences follow clinical presentations. Myopericarditis is defined
congenital absence or surgical removal of the peri- as a primarily pericarditic syndrome with concom-
cardium.2 Presence of an intact pericardium re- itant myocardial involvement and inflammation.4
sults in a potential space that can accommodate Perimyocarditis specifies a primarily myocarditic
only a small reserve volume. The pressure- syndrome with pericardial involvement.4 In prac-
volume relation of the pericardium is nonlinear; tice, the two terms are often used interchangeably
hence, small acute pericardial effusions may and a precise and uniformly adopted definition is
cause cardiac tamponade, whereas slow chronic lacking. Typically, inflammation of the pericardium
effusions may stretch the pericardium and not pro- without involvement of the myocardium or
duce tamponade physiology (Fig. 2). The pericar- depressed ejection fraction is referred to as
dium has limited elasticity and, once its limit pericarditis.
is reached, the heart must compete with the Acute pericarditis refers to pericardial inflamma-
intrapericardial fluid for a fixed intrapericardial tion with acute onset of symptoms. Without treat-
volume.2 The mechanical, membranous, and ment, these symptoms can last for 4 to 6 weeks
before resolution. The term incessant pericarditis pericarditic chest pain (>85%–90% of cases),
is used when symptoms last for greater than 4 to which is typically sharp and pleuritic, and
6 weeks but less than 3 months without remission. improved by sitting up and leaning forward; (2)
Pericarditis is said to be recurrent or relapsing if pericardial friction rub (rare); (3) characteristic
symptoms recur after a previous episode that ECG abnormalities; (4) new or worsening pericar-
completely resolved with treatment. Chronic peri- dial effusion. Acute pericarditis with known
carditis lasts more than 3 months (Box 2).3 (increased levels of biomarkers of myocardial
Acute pericarditis is a clinical diagnosis, made
with at least 2 of the following 4 criteria: (1)
Box 2
Myopericardial syndromes: terminology
Table 1
Causes of acute pericarditis
Infectious Causes
Viral (common) Coxsackie viruses; echoviruses; adenovirus; influenza A and B viruses; enterovirus;
mumps virus; Epstein-Barr virus; HIV; herpes simplex virus; type I varicella zoster
virus; measles; parainfluenza viruses type II; respiratory syncytial virus,
cytomegalovirus ; hepatitis A, B, and C, parvovirus B19
Bacterial Gram-positive and gram-negative species (streptococci, staphylococci,
pneumococci), Mycobacterium tuberculosis
Less common: Legionella, Nocardia, Actinobacillus, Rickettsia, Borrelia burgdorferi
(Lyme disease), Listeria, Leptospira, Chlamydophila psittaci, Treponema
pallidum (syphilis), Coxiella burnetii, Meningococcus spp, Haemophilus spp,
Mycoplasma spp
Fungal (rare) Histoplasma, Blastomyces, coccidiosis, Aspergillus, Candida
Parasitic (rare) Toxoplasma, Entamoeba, Echinococcus
Noninfectious Causes
Autoimmune Systemic lupus erythematous, Sjögrensyndrome, rheumatoid arthritis,
(common) scleroderma, vasculitides–eosinophilic granulomatosis (Churg-Strauss
syndrome), Takayasu disease, Behçet syndrome, sarcoidosis, familial
Mediterranean fever, inflammatory bowel disease, Still disease, mixed
connective tissue disorder, Reiter syndrome, Wegener granulomatosis,
ankylosing spondylitis, giant cell arteritis, dermatomyositis, serum sickness
Neoplastic Primary (mesothelioma), secondary (lung, breast, and so forth)
Metabolic Uremia, myxedema, anorexia nervosa
Drugs Daunorubicin, doxorubicin, cyclophosphamide, 5-fluorouracil, amiodarone,
cyclosporine, mesalazine, clozapine, methysergide, anti–tumor necrosis factor,
hydralazine, procainamide, methyldopa, phenytoin, isoniazid, reserpine
Trauma/ Coronary interventions, permanent pacemaker/implantable cardioverter-
iatrogenic defibrillator implantation, radiofrequency ablation, penetrating,
nonpenetrating trauma, esophageal perforation, rupture
Miscellaneous Amyloidosis, aortic dissection, radiation induced (indirect injury)
Adapted from Doctor NS, Shah AB, Coplan N, et al. Acute pericarditis. Prog Cardiovasc Dis 2017;59(4):349–59; with
permission.
depression (Figs. 3 and 4). However, these classic but not limited to large-territory ST-segment eleva-
findings are insensitive and are reported to be tion myocardial infarction (STEMI), (also called
seen in less than 60% of patients.5 It is estimated wraparound left anterior descending artery STEMI,
that approximately 90% of patients with acute or inferolateral STEMI, in which ST elevation is
pericarditis manifest some type of ECG abnormal- seen in multiple leads), ventricular aneurysm, cor-
ity.11 In the past, ECG changes in acute pericar- onary vasospasm, acute perimyocarditis, early
ditis have been thought to present in 4 stages. repolarization, left bundle branch block, and
First, diffuse ST-segment elevation that occurs in paced rhythms. A method to assist in clinical dif-
all leads (except for V1 and aVR) with diffuse PR- ferentiation of these distinct disease processes
segment depression; second, the ST segment has value, because treatment varies based on
returns to baseline and the T wave flattens; third, the underlying cause. This differentiation can
the T wave inverts and there is potential prove challenging because several different clin-
ST-segment depression; and fourth, the ECG ical entities can have similar and atypical symp-
returns to normal over the course of weeks or toms with comparable ECG abnormalities. Thus,
months.11–13 emergency physicians must focus on the life-
Clinicians are commonly tasked with distin- threatening causes of chest pain to avoid misdiag-
guishing the differences between the ECG nosis and failure to treat these conditions in a
changes seen in acute myopericardial syndromes timely manner.
and other differential diagnoses that can cause Although there are differentiating ECG patterns,
diffuse ST-segment elevations (Box 3). Several accurate assessment is imperfect using the
important causes must be considered, including ECG alone because many of the features in
108 Farzad & Schussler
Fig. 3. Twelve-lead ECG of a 47-year-old woman with acute perimyocarditis. The ECG shows sinus bradycardia with
diffuse concave morphology ST-segment elevation compared with the isoelectric T-P segment. ST-segment elevations
are most marked in leads I, II, aVF, and V3 to V5. Note the absence of reciprocal ST-segment depression (excluding
leads aVR and V1) as seen in ST-elevation MI. PR-segment depressions are also seen. (Courtesy of Amal Mattu, MD,
Baltimore, MD.)
perimyocarditis are temporal.10 The focus should STEMI should be considered when the degree of ST
be on identifying ACSs that require urgent or emer- elevation is greater in lead III than lead II, conversely
gent angiography. Reciprocal ST-segment the ST-increase in pericarditis is typically greater in
changes favor STEMI but are not always present. lead II than lead III.12–15 Recent literature by Bischof
In contrast with ACS, which may have several and colleagues16 suggests that, when there is ST-
different ST-segment morphologies, concave ST- segment elevation in inferior leads, the presence
segment elevation is typical of acute perimyocardi- of any ST depression in lead aVL is highly specific
tis. When ST elevation is seen in both leads II and III, for coronary occlusion from inferior STEMI and
Fig. 4. Another ECG of acute pericarditis showing sinus tachycardia, diffuse (mild) ST elevation, and marked PR-
segment depression. (Courtesy of Jeffrey Schussler, MD, Baltimore, MD.)
Acute Myopericardial Syndromes 109
Box 3
Differential diagnosis for diffuse ST-segment elevation seen on 12-lead electrocardiogram with a few
distinguishing factors highlighted
strongly weighs against pericarditis. The temporal The PR segment has been suggested to be as
evolution of ECG changes with both entities is highly important as the ST segment because inflammation
variable and changes with treatment and time. of the subepicardial myocardium causes exagger-
When attempting to distinguish between STEMI ated atrial repolarization of the thin-walled atrium
and myopericardial syndrome, providers are and results in PR-segment depression.11,13 Spo-
encouraged to know the differences in ECG findings dick12 published a study in 1974 that identified
and consider the factors that strongly favor STEMI PR-segment depression in more than 80% of the
first. A 2-step process that is designed to avoid ECGs of patients with diagnosed acute pericarditis.
misdiagnosis and delay in STEMI diagnosis is pre- It has been is suggested that the PR segment can
sented in Box 4. be used to support the diagnosis of acute
110 Farzad & Schussler
Table 2
Comparison of electrocardiogram findings to help in distinguishing between ST-segment elevation
myocardial infarction versus myopericardial syndromes, and early repolarization
Comparison of
ECG Findings Acute STEMI Perimyocarditis Early Repolarization
ST-segment Diffuse or localized STE, Usually diffuse STE, Usually precordial STE,
deviation frequently with without reciprocal STD without reciprocal STD
reciprocal STD (except V1 or aVR)
ST-segment Straight, horizontal, Concave upwards (smiley Concave upwards (smiley
morphology convex (frowny face), or face) face)
concave
STE in inferior STE III>II (usually), with STE II>III (almost always), STE II>III without
leads reciprocal STD in aVL without reciprocal STD reciprocal STD in aVL
in aVL
Evolving ECG ST segments and T-wave Unlikely to witness Compare with old ECGs;
changes abnormalities evolve evolving changes unlikely to have
with treatment and during course of ED visit evolving changes
time during course of ED visit
ST/T-wave ratio NA >0.25 <0.25
in V6
Table 3
Recommendations for the treatment of acute pericarditis
Table 4
Commonly prescribed antiinflammatory therapies for recurrent pericarditis
Fig. 5. Twelve-lead ECG showing sinus tachycardia, low-voltage QRS complexes, and electrical alternans in a pa-
tient with cardiac tamponade. (Courtesy of Amal Mattu, MD, Baltimore, MD; with permission.)
is sometimes the first test in an ED for the evaluation assistance from both fluoroscopy and echocardi-
of chest pain. It can evaluate for pericardial effusion, ography. In the emergency setting, this can be
aortic dissection, and coronary artery disease (as done at the bedside using sonographic guidance
well as other intrathoracic disorders such as pneu- or (in the case of a code) without imaging.21
monia and PE). In addition to the evaluation for the Tamponade physiology, when it occurs rapidly,
presence or absence of pericardial fluid, both CT can be caused by a small amount of pericardial fluid.
and MRI can evaluate the pericardium for thick- In these situations, the risk of percutaneous removal
ening, which is sometimes seen in the acute phase is higher, and so imaging is helpful in locating the
of pericarditis but is a concern when more chronic fluid and guiding its removal.21 In less critical or
pericarditis (or constriction) is suspected. time-sensitive settings, or in situations in which
there is recurrence of the effusion, surgical evacua-
Myocardial biopsy tion can be done. The creation of a pericardial win-
Biopsy is not typically used in the setting of peri- dow is as effective in removing acute collections of
carditis but can be used in the confirmation of fluid as well as preventing their recurrence.21
myocarditis. Biopsy of the myocardium has an
inherent risk of perforation but this is low in expe-
Symptomatic Treatment of Pericarditis
rienced hands. Typically, biopsy is reserved for in-
stances in which more myocardial dysfunction is Treatment of pericarditis is with nonsteroidal anti-
present and acute infectious myocarditis needs inflammatory medications. Historically, indometh-
to be proved so that acute treatments can be acin was used, but most common nonsteroidal
tailored to the patient. antiinflammatory drugs (NSAIDs) can be used,
such as ibuprofen (typically in full-strength doses
TREATMENT of 800–1600 mg daily) or even intravenous ketoro-
lac.22 Colchicine has recently been added as first-
After identification, the treatment of acute myoper- line therapy with NSAIDs. Colchicine should be
icarditic conditions is in 2 categories: treatment of continued for at least 6 months to prevent recur-
any hemodynamic issues (mainly pericardial effu- rence, because many recurrences are caused by
sion and tamponade) and symptomatic relief of inadequate dose and duration of initial therapy.6,8
the pericardial pain. Recurrent pericarditis is treated with a repeat
course of NSAIDs (Tables 3 and 4).
Urgent Treatment of Tamponade Physiology
Steroids are not recommended for either the
Pericardiocentesis is the rapid removal of fluid acute occurrence of pericarditis or recurrence,
from the pericardial space and is the gold standard although they have been used in rare instances
for treatment of tamponade from any cause, with intractable pain. The concern is that using
including pericarditis. This procedure is typically them early in the course of disease may instigate
done in the cardiac catheter laboratory, with early recurrence.23
Acute Myopericardial Syndromes 113
Many patients with acute pericarditis are diagnostic criteria are needed to make an accurate
admitted to the hospital, but this is more because diagnosis, exclude concomitant disease, and
a significant percentage of them are initially diag- properly disposition patients. Therapy for acute
nosed as having an acute MI. Most patients with pericarditis should be guided per the underlying
acute pericarditis do not need to be admitted to cause. For the most common causes (idiopathic
the hospital (if they are correctly identified, and and viral), NSAIDs or aspirin with the addition of
their pain is managed). Patients with pericardial ef- colchicine remains the mainstay of therapy. Pa-
fusions who are hemodynamically stable may be tients with hemodynamic compromise who are
observed for changes in their status. Hypotensive resistant to therapy or have high-risk features
patients are a medical emergency and should be should prompt hospitalization and initiation of
admitted.20 more aggressive therapy.
12. Spodick DH. Electrocardiogram in acute pericar- 18. Shakir DK, Arafa SOE. Right atrial infarction, atrial
ditis: distributions of morphologic and axial changes arrhythmia and inferior myocardial infarction form a
by stages. Am J Cardiol 1974;33:470–4. missed triad: a case report and review of the litera-
13. Spodick DH. Electrocardiographic abnormalities in ture. Can J Cardiol 2007;23(12):995–7.
pericardial disease. In: Spodick DH, editor. The peri- 19. Salisbury AC, Olalla-Gómez C, Rihal CS, et al. Fre-
cardium: a comprehensive textbook. New York: quency and predictors of urgent coronary angiog-
Marcel Dekker; 1997. p. 40–64. raphy in patients with acute pericarditis. Mayo Clin
Proc 2009;84(1):11–5.
14. Wang K, Asinger RW, Marriott HJL. ST-segment
20. Khandaker MH, Espinosa RE, Nishimura RA, et al.
elevation in conditions other than acute myocardial
Pericardial disease: diagnosis and management.
infarction. N Engl J Med 2003;349(22):2128–35.
Mayo Clin Proc 2010;85(6):572–93.
15. Brady WJ, Syverud SA, Beagle C, et al. Electrocar-
21. Schussler JM, Grayburn PA. Contrast guided two-
diographic ST-segment elevation: the diagnosis of
dimensional echocardiography for needle localiza-
acute myocardial infarction by morphologic analysis
tion during pericardiocentesis: a case report. J Am
of the ST segment. Acad Emerg Med 2001;8(10):
Soc Echocardiogr 2010;23(6):683.e1-2.
961–7.
22. Arunasalam S, Siegel RJ. Rapid resolution of symp-
16. Bischof JE, Worrall C, Thompson P, et al. ST depres- tomatic acute pericarditis with ketorolac trometh-
sion in lead aVL differentiates inferior ST-elevation amine: a parenteral nonsteroidal antiinflammatory
myocardial infarction from pericarditis. Am J Emerg agent. Am Heart J 1993;125(5 Pt 1):1455–8.
Med 2016;34(2):149–54. 23. Farand P, Bonenfant F, Belley-Côté EP, et al.
17. Dorfman TA, Aqel R. Regional pericarditis: a review Acute and recurring pericarditis: more colchicine,
of the pericardial manifestations of acute myocardial less corticosteroids. World J Cardiol 2010;2(12):
infarction. Clin Cardiol 2009;32(3):115–20. 403–7.