- Email: [email protected]
A rare case of concomitant viral myocarditis and pericarditis in a 44-year-old patient
The Journal of Emergency Medicine, Vol. 27, No. 4, pp. 355–359, 2004 Copyright © 2004 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/04 $–see front matter
doi:10.1016/j.jemermed.2004.03.017
Clinical Communications
A RARE CASE OF CONCOMITANT VIRAL MYOCARDITIS AND PERICARDITIS IN A 44-YEAR-OLD PATIENT Aleksandr Gorenbeyn,
MD*
and Alan Jon Smally,
MD†
*Integrated Residency in Emergency Medicine and †Division of Emergency Medicine, The University of Connecticut School of Medicine, Hartford, Connecticut Reprint Address: Aleksandr Gorenbeyn, MD, Department of Emergency Medicine and Traumatology, John Dempsey Hospital, University of Connecticut Health Center, 263 Farmington Avenue, Farmington, CT 06030
e Abstract—Concomitant severe myocarditis and pericarditis are under-recognized or rare. We report a case of a 44-year-old woman who presented in shock after a week of a flu-like illness and several hours of severe back pain. Emergent echocardiography revealed cardiac tamponade for which pericardiocentesis achieved transient improvement. Unfortunately, the patient died 2 days later secondary to progressive cardiomyopathy and multi-system organ failure. Post-mortem studies of the heart were consistent with coxsackie virus myocarditis. Diagnosis and treatment of myocarditis and pericarditis are discussed, including clues to detecting concurrent disease processes. © 2004 Elsevier Inc.
and treatment of both disease entities are discussed after the case report.
CASE REPORT A 44-year-old woman presented to the ED in shock. She had been discharged 3 h previously with a diagnosis of back pain and Raynaud Syndrome. She had been sick for about a week with a flu-like illness manifesting as myalgias, diaphoresis, and non-productive cough. For the previous 3 days she had been feeling weak and had slept on the couch at home. The back pain had developed within the last 24 h. Her primary physician diagnosed her with viral syndrome 1 day before admission. The past medical history was significant for Raynaud Syndrome without evidence of generalized collagen vascular disease, and depression treated with nortriptylline. On the initial ED visit, she complained of severe low back pain without radiculopathy. She gave no history of similar pain before and had experienced no trauma. At triage she had vital signs that included a pulse of 112 beats/min and a blood pressure of 84/60 mm Hg. The respirations were 18 breaths/min and the temperature was 34.9°C (94.8°F) tympanically. She was placed in a
e Keywords—pericarditis; myocarditis; myopericarditis; viral illness; coxsackie virus
INTRODUCTION Concomitant severe myocarditis and pericarditis are rarely seen, but can result in a moribund patient presenting to the Emergency Department (ED). We discuss the case of a 44-year-old patient who came to the ED in shock, was treated for cardiac tamponade, and died 2 days later from progressive cardiomyopathy. Coxsackie virus myocarditis was confirmed post-mortem. Diagnosis
Clinical Communications (Adults) is coordinated by Ron Walls, University Medical School, Boston, Massachusetts
RECEIVED: 30 April 2003; FINAL ACCEPTED: 30 March 2004
SUBMISSION RECEIVED:
MD,
4 February 2004; 355
of Brigham and Women’s Hospital and Harvard
356
A. Gorenbeyn and A. J. Smally
Figure 1. Patient’s EKG on the second visit.
room and, without any treatment, had a repeat blood pressure of 110 mm Hg palpable and a heart rate of 110 beats/min. Examination on the first visit revealed a woman who was noted to be in pain with any movement. She was noted to have tenderness over the lumbar spine. Examination of the chest, heart, abdomen, and extremities revealed only acrocyanosis of the hands that she stated was consistent with the Raynaud Syndrome. No mention was made of abnormalities of neck veins, heart sounds or pericardial sounds. During a stay of just over 4 h, she was given a liter of i.v. fluid, 3 mg of morphine, and two oxycodone and acetaminophen tablets and was discharged feeling better with a pulse of 110 beats/min and blood pressure of 118 mm Hg. About 3 h after arriving home, the patient’s husband noted her sensorium to be markedly depressed. He called 911. Evaluation by paramedics revealed a woman who was hypotensive with a systolic blood pressure of 70 mm Hg and decreased respirations. She was transported to the ED with bag valve mask-assisted respirations during part of the transport, but had return of adequate respirations by ED arrival. An unknown amount of intravenous fluid was administered. The patient was placed in a room in the ED and found to have a blood pressure of 55/43 mm Hg, pulse of 120 beats/min, and a respiratory rate of 16 breaths/min. A physician called to evaluate her found her to be again in respiratory arrest, now without palpable pulses. Chest
compressions and bag valve mask ventilation were begun. Shortly thereafter she was intubated and found to have weak femoral pulses. The examination was remarkable only for prominent neck veins in the supine position. The heart sounds were quite distant and no abnormal rubs, murmurs or gallops were apparent. Pulsus paradoxus was not measured. An electrocardiogram (EKG) showed low voltage without acute changes (Figure 1). The chest X-ray study is shown in Figure 2. A stat cardiac ultrasound revealed tamponade. Pericardiocentesis was performed by the Emergency Medicine Resident with 120 cc of yellow fluid removed, followed by rapid return of circulation and a blood pressure of 103/61 mm Hg. However, the patient’s mental status did not appreciably improve. The patient was admitted to the cardiology service with consultation by cardiac surgery. She had a stormy 2-day course. She was found to have a severe cardiomyopathy and went into multi-system organ failure. Initially an intra-aortic balloon pump and then a left ventricular assist device were used, but the course was one of progressive deterioration. She died on the second day of hospitalization. Autopsy revealed an enlarged, dilatated heart. Cardiac and pericardial fibrinous adhesions were present. Reactive mesothelial cells were seen in the pericardial fluid. Electron microscopy studies were consistent with coxsackie virus myocarditis. Death was secondary to progressive myocarditis and multi-system organ failure.
Concomitant Myocarditis and Pericarditis
Figure 2. Chest X-ray immediately after intubation. Chest pad has not been removed.
DISCUSSION Our patient’s illness began a week before her death as a simple flu-like syndrome that developed into pericarditis and progressive, fulminant myocarditis. The second ED presentation was precipitated by the development of pericardial tamponade and its relief temporarily alleviated her shock. The remaining course was one of rapidly deteriorating myocardial and multisystem failure. Pericarditis and myocarditis are relatively common. Their concurrence, however, is recognized infrequently. Incidence of concomitant disease is unknown, probably due to the mild and non-specific symptoms of myocarditis (in the presence of pericarditis) that do not prompt diagnostic studies (1). Non-specific flu-like symptoms may proceed and mimic both pericarditis and myocarditis. Carefully acquiring the history and searching thoroughly for signs that suggest a life-threatening disease process is the key to successful early diagnosis. Additional tests and ancillary studies may be helpful. Our patient’s initial presentation of weakness, diaphoresis, dry cough, and myalgias, most consistent with a viral illness, had a potentially vast differential diagnosis. The later development of restlessness and severe low back pain was more concerning. Considerations at that point included aortic dissection, retroperitoneal hemorrhage, pyelonephritis, spinal epidural abscess or hema-
357
toma, and occult traumatic injury perhaps secondary to domestic violence. In addition, the combination of back pain (though low back pain would be atypical) and hypotension suggested the possibilities of cholecystitis, pneumothorax, acute coronary syndrome, chest pain related to illicit drug use, pericarditis, and added the concern about cardiac tamponade. The history given by this patient excluded traumatic causes and made illicit drug abuse (denied using street drugs) and pneumothorax (slow symptom progression, pain location and no dyspnea) less likely. Abnormal physical examination findings may also suggest that a particular patient’s illness is more than a simple viral illness. Signs portending more serious disease include hypotension and tachycardia; significantly different blood pressures between two arms; pulsus paradoxus; elevated neck veins; muffled heart sounds, heart murmurs, rubs or gallops; absent breath sounds or evidence of rales on lung auscultation. Findings such as these would prompt the clinician to obtain a chest X-ray study, EKG, cardiac enzymes, and cardiac ultrasonography to search for pericardial effusion or tamponade (2). On the first visit, our patient’s blood pressure normalized before any interventions, masking the underlying pathophysiology. A viral etiology is the most common cause of myocarditis with coxsackievirus B virus identified specifically in up to 50% of viral myocarditis patients (3). Pericarditis is less commonly viral (4). When viral pericarditis occurs, there is commonly myocardial involvement too, producing myopericarditis. Both diseases have many other etiologies (Table 1). Tamponade is infrequent with myocarditis or viral pericarditis; it occurs most commonly with malignant pericardial disease (5). Clinically, patients with myocarditis present with non-specific symptoms, including fatigue, myalgias, low-grade fever and mild dyspnea. Up to 60% of patients may have a documented antecedent viral syndrome. When symptoms are more severe, myocarditis mimics acute myocardial infarction or congestive heart failure (CHF). If syncope has occurred, it should be viewed as an ominous sign of impending dysrhythmia (3). Acute pericarditis is characterized by sharp or stabbing precordial or retrosternal chest pain that may radiate to the back, neck or left shoulder. It can be of sudden or gradual onset and characteristically is relieved with sitting and leaning forward. With the development of tamponade, patients are anxious, dyspneic, fatigued, and can have altered mental status. Onset may be slow or rapid (6). In mild myocarditis, abnormal findings on the physical examination are minimal or even absent. With progression, tachypnea and tachycardia are seen and with
358
A. Gorenbeyn and A. J. Smally
Table 1. Common Causes Of Myocarditis And Pericarditis Myocarditis Infectious Viruses Coxsackie B virus Echovirus Influenza virus Parainfluenza virus Epstein-Bar virus Hepatitis B virus Human Immunodeficiency virus Bacteria Corineum diphtheriae Neiserria meningitides Mycoplasma pneumoniae B-hemolytic streptococcus Borrelia burgdorferi Fungi Immune mediated Allergens Medications Autoantingens Chagas’ disease Sarcoidosis Scleroderma Systemic lupus erythematosus Alloantingens Heart transplant Toxic/miscellaneous/idiopathic Drugs Anthracyclines, cyclophosphamide, ethanol, lithium Heavy metals Physical agents Electric shock, radiation
Pericarditis
Coxsackie B virus Echovirus Human Immunodeficiency virus
Staphylococcus species Streptococcus species Mycobacterium tuberculosis Histoplasma Capsulatum
Medications (procainamide and hydralazine) Systemic lupus erythematosus
Radiation Malignancy Uremia Dressler Syndrome Myxedema
ventricular impairment, jugular venous distention, bibasilar crackles and peripheral edema develop. Progression to ventricular dilatation leads to mitral and tricuspid regurgitation murmurs, signs of peripheral or pulmonary embolism, or frank cardiogenic shock. The latter has a high mortality (3). In pericarditis, auscultation of a pericardial friction rub is classic but can be a transient finding. It is the most common manifestation of pericarditis, occurring in about 50% of cases. Low grade fever, frequent premature ventricular contractions (PVCs) and atrial premature contractions (APCs), tachypnea and dyspnea may be present. Pericardial effusion progressing to tamponade is characterized by Beck’s triad (jugular venous distension, hypotension, muffled heart sounds), pulsus paradoxus, and diminishing level of consciousness (6). Laboratory findings in myocarditis include leukocytosis in 25% of patients and an elevated erythrocyte
sedimentation rate (ESR) in up to 60% of patients. In a minority of patients, cardiac enzyme elevations may mistakenly lead to the diagnosis of myocardial infarction (3,5). Leukocytosis and an elevated ESR are also seen in patients with pericarditis. Chest radiography is generally normal in mild myocarditis but with progression, cardiomegaly, vascular redistribution, interstitial and alveolar edema develop. In uncomplicated pericarditis, the chest film is normal. With progression to a moderate to large pericardial effusion, a characteristic bottle-shaped heart may be seen. Echocardiography demonstrates globally impaired ventricular function in myocarditis. In early pericarditis there is often no detectable effusion seen with echocardiography and ventricular function is normal. With progression, pericardial effusion becomes evident. With tamponade, additional findings include right ventricular collapse during diastole, a “swinging heart” (beat-to beat counterclockwise rotational movement), and a dilatated inferior vena cava without an inspiratory collapse (2). Both computed tomography (CT) and magnetic resonance imaging (MRI) can be helpful with subacute presentations of either disease, but are not used routinely in the ED (7). The EKG findings may be useful in the diagnosis of both processes. In myocarditis, sinus tachycardia, diffuse ST-segment elevation, decreased QRS amplitude, AV block, and bundle branch block (especially when new) may be present. The EKG changes in acute pericarditis evolve through four stages: initial diffuse ST-segment elevation is followed by return of ST segments to the baseline accompanied by T-wave flattening. Next, Twave inversion without Q-wave formation is seen. Finally, the EKG normalizes. PR-segment depression occurs in up to 80% of patients with viral pericarditis (5). Electric alternans is suggestive of tamponade. Endomyocardial biopsy is the gold standard of diagnosis in myocarditis. Typical findings are lymphocytic infiltrates with myocyte necrosis (8). The diagnosis of pericarditis is usually by clinical criteria. Pericardiocentesis is performed to relieve cardiac tamponade and, non-emergently, to aid in diagnosis in chronic cases. The ED treatment and necessity for admission of patients with myocarditis is prompted by degree of symptomotology. Although patients with mild symptoms, normal laboratory studies, and no evidence of dysrhythmia can be treated with symptomatic therapy as outpatients, most others will require admission for telemetry monitoring, possible anticoagulation, and treatment of CHF. Patients in cardiogenic shock may require ionotropic and vasodilatator agents and intra-aortic balloon counterpulsation. Pacemaker placement may be needed in Mobitz II or complete heart block (9). Immunosuppression has not been shown to be beneficial (10). Prog-
Concomitant Myocarditis and Pericarditis
nosis is related to the degree of CHF and dilatated cardiomyopathy at presentation. Only a quarter of patients who present in CHF recover partially or completely. The elderly are at higher risk for an adverse outcome (9). Viral pericarditis is treated initially with NSAIDs (6). Corticosteroids are added in persistently symptomatic patients. A pericardial effusion that is rapidly enlarging necessitates admission. Signs of impending or actual tamponade, such as hypotension or echocardiographic indicators (as described above) suggest consideration of emergent pericardiocentesis. Emergent pericardiocentesis is warranted in the patient who develops hemodynamic compromise unresponsive to fluid resuscitation or experiences cardiac arrest. On the contrary, in patients without hemodynamic impairment, with both myocardial and pericardial disease, drainage of the pericardial effusion may result in dilatation of the heart and worsening contractile function (11). Although an occasional patient develops tamponade, the great majority of patients with viral pericarditis have complete recovery. The key to preventing an adverse outcome lies in a careful assessment and appropriate discharge instructions. Our patient’s initial presentation was nonspecific. At both the primary doctor’s office and the first ED visit, the predominant signs and symptoms were those of the generalized coxsackie virus infection. The disease process progressed and the next symptomatic manifestation was pericarditis with cardiac tamponade. With relief of tamponade the patient had initial recovery of cardiac function before progressive myocarditis proved fatal. This fulminant course was not predicted by the early symptoms, and emphasizes the importance of considering a broad differential diagnosis in an ill patient with a seemingly benign, but rapidly progressive, disease process. Although she had maximum therapeutic effort, including
359
emergent pericardiocentesis, our patient’s myocarditis and multi-system organ failure could not be reversed. The diagnosis of concomitant myocarditis and pericarditis can be difficult (11). Findings consistent with both processes in the history and physical examination should prompt the physician to obtain ancillary studies. Combinations such as positional pain or a pericardial friction rub in the presence of congestive heart failure or ST segment elevations with atypical evolution would suggest combined pericardial and myocardial disease. REFERENCES 1. Lie JT. Myocarditis and endomyocardial biopsy in unexplained heart failure: a diagnosis in search of disease. Ann Intern Med 1988;109:525– 8. 2. Hauser AM. The emerging role of echocardiography in the emergency department. Ann Emerg Med 1989;18:1298 –303. 3. Feldman AM, McNamara D. Myocarditis. N Engl J Med 2000; 343:1388 –98. 4. Spodick DH. Differential diagnosis of acute pericarditis. Prog Cardiovasc Dis 1971;14:192. 5. Oakley CM. Myocarditis, pericarditis and other pericardial diseases. Heart 2000;84:449 –54. 6. Aikat S, Ghaffari S. A review of pericardial diseases: clinical, ECG and hemodynamic features and management. Cleve Clin J Med 2000;67:903–14. 7. Sechtem U, Tscholakoff D, Higgins CB. MRI of the abnormal pericardium. AJR Am J Roentgenol 1986;147:245–52. 8. Billingham ME. The diagnostic criteria of myocarditis by endomyocardial biopsy. In: Sekiguchi M, Olsen EGJ, Goddwin JF, eds. Myocarditis and related disorders: proceedings of the International Symposium on Cardiomyopathy and Myocarditis. New York: Springer-Verlag; 1985:133–7. 9. O’Conell JB, Costanzo-Nordin MR, Engelmeier RS, et al. Prognosis and treatment of cardiomyopathy and miocarditis. In: Sekiguchi M, Olsen EGJ, Goddwin JF, eds. Myocarditis and related disorders: proceedings of the International Symposium on Cardiomyopathy and Myocarditis. New York: Springer-Verlag; 1985:175–9. 10. Mason JW, O’Conell JB, Herskowitz A, et al. A clinical trial of immunosuppressive therapy for myocarditis. The Myocarditis Treatment Trial Investigators. N Engl J Med 1995;333:269 –75. 11. Spodick DH. Pericardial diseases. In: Braunwald E, ed. Heart disease: A textbook of cardiovascular medicine, 6th edn. Philadelphia, PA: WB Saunders Co; 2001:1836 – 8.
doi:10.1016/j.jemermed.2004.03.017
Clinical Communications
A RARE CASE OF CONCOMITANT VIRAL MYOCARDITIS AND PERICARDITIS IN A 44-YEAR-OLD PATIENT Aleksandr Gorenbeyn,
MD*
and Alan Jon Smally,
MD†
*Integrated Residency in Emergency Medicine and †Division of Emergency Medicine, The University of Connecticut School of Medicine, Hartford, Connecticut Reprint Address: Aleksandr Gorenbeyn, MD, Department of Emergency Medicine and Traumatology, John Dempsey Hospital, University of Connecticut Health Center, 263 Farmington Avenue, Farmington, CT 06030
e Abstract—Concomitant severe myocarditis and pericarditis are under-recognized or rare. We report a case of a 44-year-old woman who presented in shock after a week of a flu-like illness and several hours of severe back pain. Emergent echocardiography revealed cardiac tamponade for which pericardiocentesis achieved transient improvement. Unfortunately, the patient died 2 days later secondary to progressive cardiomyopathy and multi-system organ failure. Post-mortem studies of the heart were consistent with coxsackie virus myocarditis. Diagnosis and treatment of myocarditis and pericarditis are discussed, including clues to detecting concurrent disease processes. © 2004 Elsevier Inc.
and treatment of both disease entities are discussed after the case report.
CASE REPORT A 44-year-old woman presented to the ED in shock. She had been discharged 3 h previously with a diagnosis of back pain and Raynaud Syndrome. She had been sick for about a week with a flu-like illness manifesting as myalgias, diaphoresis, and non-productive cough. For the previous 3 days she had been feeling weak and had slept on the couch at home. The back pain had developed within the last 24 h. Her primary physician diagnosed her with viral syndrome 1 day before admission. The past medical history was significant for Raynaud Syndrome without evidence of generalized collagen vascular disease, and depression treated with nortriptylline. On the initial ED visit, she complained of severe low back pain without radiculopathy. She gave no history of similar pain before and had experienced no trauma. At triage she had vital signs that included a pulse of 112 beats/min and a blood pressure of 84/60 mm Hg. The respirations were 18 breaths/min and the temperature was 34.9°C (94.8°F) tympanically. She was placed in a
e Keywords—pericarditis; myocarditis; myopericarditis; viral illness; coxsackie virus
INTRODUCTION Concomitant severe myocarditis and pericarditis are rarely seen, but can result in a moribund patient presenting to the Emergency Department (ED). We discuss the case of a 44-year-old patient who came to the ED in shock, was treated for cardiac tamponade, and died 2 days later from progressive cardiomyopathy. Coxsackie virus myocarditis was confirmed post-mortem. Diagnosis
Clinical Communications (Adults) is coordinated by Ron Walls, University Medical School, Boston, Massachusetts
RECEIVED: 30 April 2003; FINAL ACCEPTED: 30 March 2004
SUBMISSION RECEIVED:
MD,
4 February 2004; 355
of Brigham and Women’s Hospital and Harvard
356
A. Gorenbeyn and A. J. Smally
Figure 1. Patient’s EKG on the second visit.
room and, without any treatment, had a repeat blood pressure of 110 mm Hg palpable and a heart rate of 110 beats/min. Examination on the first visit revealed a woman who was noted to be in pain with any movement. She was noted to have tenderness over the lumbar spine. Examination of the chest, heart, abdomen, and extremities revealed only acrocyanosis of the hands that she stated was consistent with the Raynaud Syndrome. No mention was made of abnormalities of neck veins, heart sounds or pericardial sounds. During a stay of just over 4 h, she was given a liter of i.v. fluid, 3 mg of morphine, and two oxycodone and acetaminophen tablets and was discharged feeling better with a pulse of 110 beats/min and blood pressure of 118 mm Hg. About 3 h after arriving home, the patient’s husband noted her sensorium to be markedly depressed. He called 911. Evaluation by paramedics revealed a woman who was hypotensive with a systolic blood pressure of 70 mm Hg and decreased respirations. She was transported to the ED with bag valve mask-assisted respirations during part of the transport, but had return of adequate respirations by ED arrival. An unknown amount of intravenous fluid was administered. The patient was placed in a room in the ED and found to have a blood pressure of 55/43 mm Hg, pulse of 120 beats/min, and a respiratory rate of 16 breaths/min. A physician called to evaluate her found her to be again in respiratory arrest, now without palpable pulses. Chest
compressions and bag valve mask ventilation were begun. Shortly thereafter she was intubated and found to have weak femoral pulses. The examination was remarkable only for prominent neck veins in the supine position. The heart sounds were quite distant and no abnormal rubs, murmurs or gallops were apparent. Pulsus paradoxus was not measured. An electrocardiogram (EKG) showed low voltage without acute changes (Figure 1). The chest X-ray study is shown in Figure 2. A stat cardiac ultrasound revealed tamponade. Pericardiocentesis was performed by the Emergency Medicine Resident with 120 cc of yellow fluid removed, followed by rapid return of circulation and a blood pressure of 103/61 mm Hg. However, the patient’s mental status did not appreciably improve. The patient was admitted to the cardiology service with consultation by cardiac surgery. She had a stormy 2-day course. She was found to have a severe cardiomyopathy and went into multi-system organ failure. Initially an intra-aortic balloon pump and then a left ventricular assist device were used, but the course was one of progressive deterioration. She died on the second day of hospitalization. Autopsy revealed an enlarged, dilatated heart. Cardiac and pericardial fibrinous adhesions were present. Reactive mesothelial cells were seen in the pericardial fluid. Electron microscopy studies were consistent with coxsackie virus myocarditis. Death was secondary to progressive myocarditis and multi-system organ failure.
Concomitant Myocarditis and Pericarditis
Figure 2. Chest X-ray immediately after intubation. Chest pad has not been removed.
DISCUSSION Our patient’s illness began a week before her death as a simple flu-like syndrome that developed into pericarditis and progressive, fulminant myocarditis. The second ED presentation was precipitated by the development of pericardial tamponade and its relief temporarily alleviated her shock. The remaining course was one of rapidly deteriorating myocardial and multisystem failure. Pericarditis and myocarditis are relatively common. Their concurrence, however, is recognized infrequently. Incidence of concomitant disease is unknown, probably due to the mild and non-specific symptoms of myocarditis (in the presence of pericarditis) that do not prompt diagnostic studies (1). Non-specific flu-like symptoms may proceed and mimic both pericarditis and myocarditis. Carefully acquiring the history and searching thoroughly for signs that suggest a life-threatening disease process is the key to successful early diagnosis. Additional tests and ancillary studies may be helpful. Our patient’s initial presentation of weakness, diaphoresis, dry cough, and myalgias, most consistent with a viral illness, had a potentially vast differential diagnosis. The later development of restlessness and severe low back pain was more concerning. Considerations at that point included aortic dissection, retroperitoneal hemorrhage, pyelonephritis, spinal epidural abscess or hema-
357
toma, and occult traumatic injury perhaps secondary to domestic violence. In addition, the combination of back pain (though low back pain would be atypical) and hypotension suggested the possibilities of cholecystitis, pneumothorax, acute coronary syndrome, chest pain related to illicit drug use, pericarditis, and added the concern about cardiac tamponade. The history given by this patient excluded traumatic causes and made illicit drug abuse (denied using street drugs) and pneumothorax (slow symptom progression, pain location and no dyspnea) less likely. Abnormal physical examination findings may also suggest that a particular patient’s illness is more than a simple viral illness. Signs portending more serious disease include hypotension and tachycardia; significantly different blood pressures between two arms; pulsus paradoxus; elevated neck veins; muffled heart sounds, heart murmurs, rubs or gallops; absent breath sounds or evidence of rales on lung auscultation. Findings such as these would prompt the clinician to obtain a chest X-ray study, EKG, cardiac enzymes, and cardiac ultrasonography to search for pericardial effusion or tamponade (2). On the first visit, our patient’s blood pressure normalized before any interventions, masking the underlying pathophysiology. A viral etiology is the most common cause of myocarditis with coxsackievirus B virus identified specifically in up to 50% of viral myocarditis patients (3). Pericarditis is less commonly viral (4). When viral pericarditis occurs, there is commonly myocardial involvement too, producing myopericarditis. Both diseases have many other etiologies (Table 1). Tamponade is infrequent with myocarditis or viral pericarditis; it occurs most commonly with malignant pericardial disease (5). Clinically, patients with myocarditis present with non-specific symptoms, including fatigue, myalgias, low-grade fever and mild dyspnea. Up to 60% of patients may have a documented antecedent viral syndrome. When symptoms are more severe, myocarditis mimics acute myocardial infarction or congestive heart failure (CHF). If syncope has occurred, it should be viewed as an ominous sign of impending dysrhythmia (3). Acute pericarditis is characterized by sharp or stabbing precordial or retrosternal chest pain that may radiate to the back, neck or left shoulder. It can be of sudden or gradual onset and characteristically is relieved with sitting and leaning forward. With the development of tamponade, patients are anxious, dyspneic, fatigued, and can have altered mental status. Onset may be slow or rapid (6). In mild myocarditis, abnormal findings on the physical examination are minimal or even absent. With progression, tachypnea and tachycardia are seen and with
358
A. Gorenbeyn and A. J. Smally
Table 1. Common Causes Of Myocarditis And Pericarditis Myocarditis Infectious Viruses Coxsackie B virus Echovirus Influenza virus Parainfluenza virus Epstein-Bar virus Hepatitis B virus Human Immunodeficiency virus Bacteria Corineum diphtheriae Neiserria meningitides Mycoplasma pneumoniae B-hemolytic streptococcus Borrelia burgdorferi Fungi Immune mediated Allergens Medications Autoantingens Chagas’ disease Sarcoidosis Scleroderma Systemic lupus erythematosus Alloantingens Heart transplant Toxic/miscellaneous/idiopathic Drugs Anthracyclines, cyclophosphamide, ethanol, lithium Heavy metals Physical agents Electric shock, radiation
Pericarditis
Coxsackie B virus Echovirus Human Immunodeficiency virus
Staphylococcus species Streptococcus species Mycobacterium tuberculosis Histoplasma Capsulatum
Medications (procainamide and hydralazine) Systemic lupus erythematosus
Radiation Malignancy Uremia Dressler Syndrome Myxedema
ventricular impairment, jugular venous distention, bibasilar crackles and peripheral edema develop. Progression to ventricular dilatation leads to mitral and tricuspid regurgitation murmurs, signs of peripheral or pulmonary embolism, or frank cardiogenic shock. The latter has a high mortality (3). In pericarditis, auscultation of a pericardial friction rub is classic but can be a transient finding. It is the most common manifestation of pericarditis, occurring in about 50% of cases. Low grade fever, frequent premature ventricular contractions (PVCs) and atrial premature contractions (APCs), tachypnea and dyspnea may be present. Pericardial effusion progressing to tamponade is characterized by Beck’s triad (jugular venous distension, hypotension, muffled heart sounds), pulsus paradoxus, and diminishing level of consciousness (6). Laboratory findings in myocarditis include leukocytosis in 25% of patients and an elevated erythrocyte
sedimentation rate (ESR) in up to 60% of patients. In a minority of patients, cardiac enzyme elevations may mistakenly lead to the diagnosis of myocardial infarction (3,5). Leukocytosis and an elevated ESR are also seen in patients with pericarditis. Chest radiography is generally normal in mild myocarditis but with progression, cardiomegaly, vascular redistribution, interstitial and alveolar edema develop. In uncomplicated pericarditis, the chest film is normal. With progression to a moderate to large pericardial effusion, a characteristic bottle-shaped heart may be seen. Echocardiography demonstrates globally impaired ventricular function in myocarditis. In early pericarditis there is often no detectable effusion seen with echocardiography and ventricular function is normal. With progression, pericardial effusion becomes evident. With tamponade, additional findings include right ventricular collapse during diastole, a “swinging heart” (beat-to beat counterclockwise rotational movement), and a dilatated inferior vena cava without an inspiratory collapse (2). Both computed tomography (CT) and magnetic resonance imaging (MRI) can be helpful with subacute presentations of either disease, but are not used routinely in the ED (7). The EKG findings may be useful in the diagnosis of both processes. In myocarditis, sinus tachycardia, diffuse ST-segment elevation, decreased QRS amplitude, AV block, and bundle branch block (especially when new) may be present. The EKG changes in acute pericarditis evolve through four stages: initial diffuse ST-segment elevation is followed by return of ST segments to the baseline accompanied by T-wave flattening. Next, Twave inversion without Q-wave formation is seen. Finally, the EKG normalizes. PR-segment depression occurs in up to 80% of patients with viral pericarditis (5). Electric alternans is suggestive of tamponade. Endomyocardial biopsy is the gold standard of diagnosis in myocarditis. Typical findings are lymphocytic infiltrates with myocyte necrosis (8). The diagnosis of pericarditis is usually by clinical criteria. Pericardiocentesis is performed to relieve cardiac tamponade and, non-emergently, to aid in diagnosis in chronic cases. The ED treatment and necessity for admission of patients with myocarditis is prompted by degree of symptomotology. Although patients with mild symptoms, normal laboratory studies, and no evidence of dysrhythmia can be treated with symptomatic therapy as outpatients, most others will require admission for telemetry monitoring, possible anticoagulation, and treatment of CHF. Patients in cardiogenic shock may require ionotropic and vasodilatator agents and intra-aortic balloon counterpulsation. Pacemaker placement may be needed in Mobitz II or complete heart block (9). Immunosuppression has not been shown to be beneficial (10). Prog-
Concomitant Myocarditis and Pericarditis
nosis is related to the degree of CHF and dilatated cardiomyopathy at presentation. Only a quarter of patients who present in CHF recover partially or completely. The elderly are at higher risk for an adverse outcome (9). Viral pericarditis is treated initially with NSAIDs (6). Corticosteroids are added in persistently symptomatic patients. A pericardial effusion that is rapidly enlarging necessitates admission. Signs of impending or actual tamponade, such as hypotension or echocardiographic indicators (as described above) suggest consideration of emergent pericardiocentesis. Emergent pericardiocentesis is warranted in the patient who develops hemodynamic compromise unresponsive to fluid resuscitation or experiences cardiac arrest. On the contrary, in patients without hemodynamic impairment, with both myocardial and pericardial disease, drainage of the pericardial effusion may result in dilatation of the heart and worsening contractile function (11). Although an occasional patient develops tamponade, the great majority of patients with viral pericarditis have complete recovery. The key to preventing an adverse outcome lies in a careful assessment and appropriate discharge instructions. Our patient’s initial presentation was nonspecific. At both the primary doctor’s office and the first ED visit, the predominant signs and symptoms were those of the generalized coxsackie virus infection. The disease process progressed and the next symptomatic manifestation was pericarditis with cardiac tamponade. With relief of tamponade the patient had initial recovery of cardiac function before progressive myocarditis proved fatal. This fulminant course was not predicted by the early symptoms, and emphasizes the importance of considering a broad differential diagnosis in an ill patient with a seemingly benign, but rapidly progressive, disease process. Although she had maximum therapeutic effort, including
359
emergent pericardiocentesis, our patient’s myocarditis and multi-system organ failure could not be reversed. The diagnosis of concomitant myocarditis and pericarditis can be difficult (11). Findings consistent with both processes in the history and physical examination should prompt the physician to obtain ancillary studies. Combinations such as positional pain or a pericardial friction rub in the presence of congestive heart failure or ST segment elevations with atypical evolution would suggest combined pericardial and myocardial disease. REFERENCES 1. Lie JT. Myocarditis and endomyocardial biopsy in unexplained heart failure: a diagnosis in search of disease. Ann Intern Med 1988;109:525– 8. 2. Hauser AM. The emerging role of echocardiography in the emergency department. Ann Emerg Med 1989;18:1298 –303. 3. Feldman AM, McNamara D. Myocarditis. N Engl J Med 2000; 343:1388 –98. 4. Spodick DH. Differential diagnosis of acute pericarditis. Prog Cardiovasc Dis 1971;14:192. 5. Oakley CM. Myocarditis, pericarditis and other pericardial diseases. Heart 2000;84:449 –54. 6. Aikat S, Ghaffari S. A review of pericardial diseases: clinical, ECG and hemodynamic features and management. Cleve Clin J Med 2000;67:903–14. 7. Sechtem U, Tscholakoff D, Higgins CB. MRI of the abnormal pericardium. AJR Am J Roentgenol 1986;147:245–52. 8. Billingham ME. The diagnostic criteria of myocarditis by endomyocardial biopsy. In: Sekiguchi M, Olsen EGJ, Goddwin JF, eds. Myocarditis and related disorders: proceedings of the International Symposium on Cardiomyopathy and Myocarditis. New York: Springer-Verlag; 1985:133–7. 9. O’Conell JB, Costanzo-Nordin MR, Engelmeier RS, et al. Prognosis and treatment of cardiomyopathy and miocarditis. In: Sekiguchi M, Olsen EGJ, Goddwin JF, eds. Myocarditis and related disorders: proceedings of the International Symposium on Cardiomyopathy and Myocarditis. New York: Springer-Verlag; 1985:175–9. 10. Mason JW, O’Conell JB, Herskowitz A, et al. A clinical trial of immunosuppressive therapy for myocarditis. The Myocarditis Treatment Trial Investigators. N Engl J Med 1995;333:269 –75. 11. Spodick DH. Pericardial diseases. In: Braunwald E, ed. Heart disease: A textbook of cardiovascular medicine, 6th edn. Philadelphia, PA: WB Saunders Co; 2001:1836 – 8.