A 35-year-old male with no significant past medical history presents to the Emergency Department with one day of worsening chest pain. The chest pain was abrupt in onset, is described as “sharp” in nature, and worsens with inspiration. It is primarily centered in the center of his chest but occasionally radiates to the left upper back. He feels that the pain worsens when he lays down. Vital signs are unremarkable including equal bilateral upper extremity blood pressures and radial pulses. On physical examination, he is febrile to 38.2°C. A triphasic “scratching” sound in time with the cardiac cycle is auscultated at the left lower sternal border. Initial laboratory studies show a negative troponin T and mild elevations in white blood cell count and the C-reactive protein. His ECG is shown below:
Which of the following is the most appropriate next step:
Correct Answer: B
This patient’s presentation is consistent with acute pericarditis. The majority of cases of acute pericarditis in developed countries are either due to viral or idiopathic causes. However, in developing nations, tuberculosis is a leading cause of pericarditis. Typical symptoms include acute onset, severe, and often sharp chest pain that is usually retrosternal, but occasionally in the left chest. The classic pattern of pericarditis pain is radiation to the trapezius ridge in the back. The pain in pericarditis is often pleuritic in nature. Additionally, it can be positional, with worsening pain with reclining and palliation with leaning forward. A low-grade fever and sinus tachycardia may be present. On physical examination a pericardial friction rub may be present and is classically heard as a triphasic scratching sound in the precordium, best heard with the patient leaning forward. However, there may be only monophasic or biphasic rub. Muffled heart sounds or elevation of the jugular venous pressure should raise immediate suspicion for a large pericardial effusion and possible tamponade physiology.
Diagnosis of acute pericarditis is a clinical decision made based on having two or more of the following: chest pain; pericardial friction rub; ECG changes with typical concave ST segment elevation spanning multiple coronary territories and/or PR depression; pericardial effusion. In addition, other morbid conditions such as acute myocardial infarction, aortic dissection, and pulmonary embolism should be considered in the differential diagnosis and excluded by appropriate clinical judgment or targeted testing. This patient has three out of four of the listed criteria and several signs and symptoms consistent with acute pericarditis.
First line treatment of the first episode of acute pericarditis thought to be secondary to viral or idiopathic causes consists of high-dose NSAIDs and weight-based colchicine dosing. The usual NSAID regimen consists of aspirin 750 to 1,000 mg every 8 hours or ibuprofen 600 to 800 mg every 8 hours for 1 to 2 weeks, followed by tapering to discontinuation over 2 to 3 weeks. Colchicine (0.5-0.6 mg every 12 hours for patients weighing >70 kg, or 0.5-0.6 mg once daily for patients weighing <70 kg) should be continued for 3 months, and then discontinued without tapering. The use of colchicine improves response to NSAID and reduces the risk of recurrent pericarditis by half. The clinical history and ECG with concave ST-segment elevations spanning multiple coronary territories are highly suggestive of acute pericarditis, and unlikely to reflect acute coronary syndrome. Therefore, initiation of heparin in this patient is not the best next step, especially as there is a theoretical risk of hemorrhagic conversion in the setting of acute pericarditis. Corticosteroids are not the first line therapy for acute pericarditis (viral or idiopathic) unless the patient cannot tolerate NSAIDs or colchicine, or if there is another indication for corticosteroids, such as an autoimmune pericarditis. Imaging studies such as CTA and coronary angiography are not as necessary at this time because acute coronary syndrome and aortic dissection are less likely given the clinical picture of pericarditis, negative troponin, ECG, and equal bilateral blood pressures.
(Figure from Adler Y, Charron P, Imazio M, et al. 2015 ESC guidelines for the diagnosis and management of pericardial diseases: the task force for the diagnosis and management of pericardial diseases of the European Society of Cardiology (ESC). Eur Heart J. 2015;36:2921.)
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A 51-year-old male with a recent history of acute pericarditis 6 weeks ago now resents with new onset chest pain. He stopped high-dose ibuprofen 4 weeks ago and has been feeling well since then. The chest pain started 12 hours ago, is very sharp, substernal, and pleuritic in nature. The symptoms are similar to his prior episode of pericarditis. He denies palpitations, lightheadedness, orthopnea, shortness of breath, and lower extremity edema.
On physical examination:
He has an audible friction rub. Kussmaul sign and jugular venous distension are absent. Laboratory evaluation reveals two negative serial troponin values, normal white blood cell count and creatinine, and mildly elevated C-reactive protein. ECG has nonspecific changes without ST-segment elevation. Echocardiogram shows normal biventricular function, no wall motion abnormalities, and a trace pericardial effusion that was previously visualized as well.
What is the most appropriate next therapy?
Correct Answer: D
Approximately 15% to 30% of patients with idiopathic acute pericarditis will have a recurrence. Recurrent pericarditis is diagnosed with new symptoms of pericarditis after resolution of prior symptoms, signs of persistent pericardial inflammation (ECG changes, elevation of CRP, friction rib, new or worse pericardial effusion) after a symptom-free period of 4 weeks or more from the prior episode of pericarditis. This patient has a history of pericarditis and presents with acute onset chest pain similar to his prior episode of pericarditis. A nonspecific ECG and negative troponins with 12 hours of chest pain decreases the likelihood of acute coronary syndrome, and thus heparin not indicated. The similarity of symptoms to prior pericarditis, friction rub, and elevated CRP occurring 4 weeks after stopping ibuprofen all point toward recurrent pericarditis as the cause of his chest pain. First line therapy for recurrent pericarditis consists of reinitiating highdose NSAIDs along with colchicine. NSAID therapy should be continued until complete resolution of symptoms and objective signs of pericarditis (CRP normalization, etc.), and then tapered. Colchicine should be continued for the duration of NSAID therapy. It improves response to NSAID and reduces the risk of recurrence by half. Proton pump inhibitor should be prescribed for gastric protection from NSAIDs. Exercise restriction is also reinstated until resolution of symptoms and normalization of CRP.
In the presence of contraindications to NSAIDs and colchicine, or recurrence of pericarditis during NSAID/colchicine therapy, low-dose corticosteroids can be considered as second line therapy, after exclusion of infectious causes. Prednisone at doses of 0.2 to 0.5 mg/kg/d (or equivalent other steroid dosing) may be considered for at least 2 to 4 weeks, and until symptoms and signs resolve. Once remission has been achieved, the steroids should be gradually tapered over 2 to 4 weeks. Next line therapies include immunosuppressive medications such as azathioprine, anakinra (a recombinant anti-interleukin-1 receptor antagonists), and intravenous immunoglobulin infusions.
A 62-year-old woman with a history of acute pericarditis 4 years ago that was treated with nonsteroidal anti-inflammatory drug (NSAID) to complete resolution presents with 8 weeks of insidiously worsening fatigue, bilateral lower extremity edema, breathlessness, and a feeling of abdominal fullness. She denies any fevers, chest pain, palpitations, or light-headedness. Physical examination is remarkable for 2+ pitting bilateral lower extremity edema, elevated jugular venous pressure with a rapid y descent, and an early diastolic sound best heard at the apex. Echocardiography shows normal right and left ventricular systolic function, a thickened pericardium without pericardial effusion, moderate left-sided pleural effusion, inspiratory ventricular septal motion toward the left ventricle, along with marked dilatation and absent respirophasic collapse of the inferior vena cava and hepatic veins.
The therapy most likely to yield relief of the patient’s symptoms is:
This patient’s clinical picture, along with signs on physical examination and diagnostic testing, are most consistent with constrictive pericarditis. The definitive treatment of constrictive pericarditis is surgical pericardiectomy. Constrictive pericarditis is the result of inflammatory injury to the pericardium arising from a plethora of causes. The risk of progressing to constrictive pericarditis is very low with most etiologies of pericardial disease, ranging from 1% in viral and idiopathic pericarditis, but up to 20% to 30% in bacterial and purulent pericarditis. Tuberculosis is a major cause of constrictive pericarditis in the developing world.
The clinical presentation of constrictive pericarditis is due to manifestations of impaired diastolic filling of both ventricles. The rigid pericardium lacks compliance, and as a result, leads to diastolic constraint on the heart with preserved ventricular function; accordingly, constrictive pericarditis is on the differential diagnosis for a heart failure with preserved ejection fraction syndrome. Signs and symptoms of right heart failure are the most prominent manifestations of constrictive pericarditis. This includes venous and hepatic congestion, hepatomegaly, and ascites. There can be significant tricuspid regurgitation present. Pleural effusions may also be seen.
A 44-year-old woman with a past medical history of asthma presents with acute onset chest pain of 2 hours duration. She reports being in her usual state of health until the morning, when she had acute onset of sharp substernal chest pain. The pain is sharp, does not radiate, and has an intensity of 8/10. It is worse when she leans forward. She does not have any associated shortness of breath, nausea, palpitations, or light-headedness. She denies any recent long car rides or airplane trips. Her son is recovering from an upper respiratory infection.
Vital signs include:
On physical examination, she has normal heart sounds without any murmurs, friction rub, or S3 or S4. There is no jugular venous distension. Laboratory findings are remarkable for a troponin T of 0.03 ng/dL (normal <0.01), d-dimer <500 ng/mL, leukocytosis to 14,000/ µL, creatinine 0.78 mg/dL, and CRP 3.5 mg/L. ECG shows nonspecific changes without evidence of active ischemia. Echocardiography shows a small pericardial effusion and normal biventricular function without wall motion abnormalities.
What is the best next step in the management of this patient?
Correct Answer: C
The patient presents with symptoms of positional chest pain after being exposed to a family member with a viral illness. She has evidence of mild myocardial injury with a very mildly elevated troponin, and echocardiogram shows no evidence of wall motion abnormalities but reveals a small pericardial effusion. The best unifying diagnosis here is myopericarditis, and the patient should be admitted to the hospital for observation and treatment with anti-inflammatory medications. The term myopericarditis or perimyocarditis refers to inflammatory injury that involves both the pericardium and myocardium. Myopericarditis indicates a primarily pericarditic syndrome, with symptoms of pericarditis (chest pain, often pleuritic and positional, nonspecific or widespread ECG changes, CRP elevations) along with elevation in cardiac injury biomarkers (troponin, CK-MB) without new global or focal left ventricular dysfunction. In contrast, perimyocarditis indicates a predominantly myocarditic syndrome with minor pericardial involvement and presents with new focal or diffuse left ventricular dysfunction along with some symptoms of pericarditis. Etiologies for both are similar and include cardiotropic viral illnesses, connective tissue diseases, inflammatory bowel diseases, radiation-induced myocardial injury, or drug-induced myocardial injury. The most common presentation of myopericarditis involves a preceding viral gastrointestinal or respiratory illness.
Although myocarditis may require an endomyocardial biopsy for confirmation, most patients with myopericarditis have a benign prognosis and self-limited illness, and therefore biopsy is not necessary in the absence of ventricular dysfunction or heart failure. Mainstay for treatment is similar to pericarditis when ventricular function is preserved and consists of high-dose anti-inflammatory agents and exercise restriction.
A 67-year-old male with history of former smoking, hypertension, diabetes, and stage III small cell lung cancer (on chemotherapy) presents to the Emergency Department with 3 days of progressive shortness of breath and an episode of syncope.
Vital signs show:
Blood pressure at rest is 96/54 mm Hg at end inspiration and drops to 82/50 mm Hg with inspiration. Physical examination reveals a pale cachectic and uncomfortable appearing male with cool extremities, muffled heart sounds, and jugular venous pressure of 14 cm H2O.
Laboratory studies are remarkable for:
Precordial leads from his ECG are shown in the figure that follows:
What is the appropriate next diagnostic step?
Correct Answer: A
This patient is presenting with evolving cardiogenic shock (hypotension, cool extremities, syncope, and evidence of organ dysfunction with acute kidney injury), likely due to pericardial tamponade. The next appropriate step is emergent limited echocardiography, to accompany emergent pericardiocentesis. Pericardial tamponade is a life-threatening emergency in which pressure in the pericardial space rises due to a variety of possible reasons, leading to compression of the heart, ultimately impairing cardiac output and leading to shock or death. Causes of pericardial tamponade are similar to those that cause pericardial effusions and are listed in the table that follows. In the case of this patient, his history of progressive lung cancer makes a malignant effusion most likely.
Pulsus paradoxus is an important physical finding in cardiac tamponade. Pulsus paradoxus refers to an abnormally large reduction in both the stroke volume and the systemic blood pressure with inspiration, which in pericardial tamponade is due to the extrinsic constraint on cardiac chamber expansion leading to enhanced ventricular interdependence. Transthoracic echocardiogram is the imaging modality of choice in diagnosing and guiding management of cardiac tamponade. Cardiac CT and MRI can be utilized to better understand the pericardium and help understand the cause of tamponade, but are rarely indicated in the acute setting.
Here, the patient is in cardiogenic shock and needs urgent imaging of the pericardium with a limited echocardiogram to guide treatment. Cardiac MRI, CTA chest, and CT chest would not add to the immediate management of this patient, as aortic dissection and pulmonary embolism are less likely based on clinical presentation. Coronary angiography is not warranted because there is no evidence of acute coronary syndrome. The troponin elevation, in this case, is explained by damage from cardiogenic shock rather than acute plaque rupture.
Causes of cardiac tamponade: Table from Adler Y, Charron P, Imazio M, et al. 2015 ESC guidelines for the diagnosis and management of pericardial diseases: the task force for the diagnosis and management of pericardial diseases of the European Society of Cardiology (ESC). Eur Heart J. 2015;36:2921.
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