Noncardiac chest pain case file

Noncardiac chest pain case file

Eugene C. Toy, MD, Barry C. Simon, MD, Terrence H. Liu, MD, MHP, Katrin Y. Takenaka, MD, Adam J. Rosh, MD, MS

Case 51

A 28-year-old man arrives at the emergency department (ED) complaining of 3 days of chest pain that began after multiple episodes of retching and vomiting following a night of heavy drinking. He reported subjective fever and sweats the day following the initial vomiting. He has been unable to tolerate any swallowing and has urinated only twice since the onset of symptoms. He describes the lower anterior chest pain that is non-radiating and of moderately severe intensity. He has no history of prior chest pain. He also complains of an intermittent sore throat and painful swallowing. He does not appear short of breath but states that he has to control his breathing to avoid a “tight feeling in his throat”. He states that his voice sounds “different” and that he feels weak and faint. The review of systems is otherwise negative. His temperature is 3 7°C, HR 132, blood pressure 168/94 and RR 22 with an O2 saturation of 98% on room air. Physical examination shows an ill and uncomfortable appearing man who is tachycardic, hypertensive, and afebrile. He has dry mucous membranes and faint bibasilar crackles. He has no past medical history and is taking no medications.

⯈ What is the most likely diagnosis?

⯈ What are the next diagnostic steps?

⯈ What the rapies should be instituted immediately?

ANSWER TO CASE 51:

Noncardiac Chest Pain

Summary: This is a 28-year-old man presenting with acute severe chest pain, diaphoresis, and dyspnea after multiple episodes of vomiting.

• Most likely diagnosis: Spontaneous esophageal perforation or Boerhaave Syndrome
• Next diagnostic steps: Place the patient on a cardiac monitor, establish IV access and obtain an electrocardiogram (ECG) immediately. A chest x-ray (CXR) should be obtained as soon as possible.
• Immediate therapies: NPO, NGT, IV resuscitation with isotonic fluids, broad spectrum antibiotics, pain management and immediate surgical consultation

ANALYSIS

Objectives

1. Become familiar with the serious causes of noncardiac chest pain (NCCP).
2. Consider spontaneous esophageal perforation in the differential diagnosis of chest pain and recognize the key signs and symptoms of Boerhaave Syndrome.
3. Understand t he therapeutic approach to NCCP in general and Boerhaave Syndrome in particular.

Considerations

This case presents a young male ED patient with chest pain who is severely ill. Failure to recognize, diagnose and treat the acuity and severity of this patient illness would be catastrophic. While the diagnosis in this case is Boerhaave Syndrome, the differential diagnosis in this case should also include: esophagitis, gastroesophageal reflux disease (GERD), esophageal spasm, esophageal foreign body, peptic ulcer disease, hepatitis, pancreatitis, pneumonia, pneumomediastinum, pulmonary embolism, pericarditis, aortic dissection/aneurysm and musculoskeletal disorders. Coronary artery disease would be very rare in a 28-year-old individual but should remain in the differential, especially in young diabetics or those with unusual risk factors.

Approach To:

Noncardiac Chest Pain

DEFINITIONS

BOERHAAVE S YNDROME: Post-emetic barotrauma induced spontaneous rupture of the esophagus

GASTROGRAFIN: Water soluble iodinated radiographic contrast agent (each mL contains 660 mg diatrizoate meglumine and 100 mg diatrizoate sodium). Each 100 mL contains 37 g of elemental iodine.

BARIUM: Water insoluble particulate suspension of barium sulfate used as radiographic contrast agent

APPROACH T O SUSPECTED BOERHAAVE SYNDROME

Pathophysiology

Boerhaave syndrome results from an increased intraesophageal pressure alternating with a negative intrathoracic pressure due to straining or vomiting. In most cases, the tear occurs along the left posterolateral aspect of the distal esophagus, as it is the least supported portion of the esophagus. Localized cervical esophageal perforation may also occur and generally follows a benign course. The esophagus is especially vulnerable to rupture because it lacks a serosal layer, and therefore collagen and elastin fibers to provide support. It is most common among 40- to 60-year-old males.

Esophageal rupture from other causes is a related but different clinical entity. The majority of esophageal ruptures are iatrogenic due to endoscopy.

Morbidity a nd mortality associated with Boerhaave Syndrome are due to an overwhelming inflammatory response to mediastinal soilage caused by gastric contents and oropharyngeal bacteria deposited in the mediastinal and pleural spaces with subsequent development of pneumonia, mediastinitis, empyema, sepsis, and multiorgan failure. Untreated Boerhaave Syndrome has a 100% case fatality rate.

Evaluation

Mackler triad: vomiting, lower chest pain, and subcutaneous cervical emphysema are classically associated with Boerhaave Syndrome, but is seen in a minority of cases on early presentation. Important potential signs and symptoms include fever, chest pain, back pain, tachypnea, tachycardia, dyspnea, cervical subcutaneous emphysema and Hamman sign, a “mediastinal crunch” heard as the heart beats surrounded by mediastinal air. Breath sounds may be decreased on the side of perforation due to pleural effusion.

If esophageal perforation is suspected, a CT scan of the chest should be ordered promptly. A CXR will likely be the initial study performed and may show pleural effusion, pneumomediastinum, widened mediastinum or pneumothorax. It should be noted that on early presentation, the CXR can be negative. CT scan is far more sensitive and may show esophageal wall thickening, periesophageal fluid, extraesophageal air, and/or air and fluid in pleural spaces. Contrast radiographic studies of the esophagus may be necessary to localize the perforation. Although barium is superior to gastrografin when attempting to locate a small perforation, it causes a severe inflammatory reaction in the mediastinum or peritoneum. Small esophageal perforations are frequently missed by both contrast modalities. If a thoracentesis is performed, fluid should be evaluated for food particles, pH <6 and elevated amylase. Endoscopy has no role in the evaluation of Boerhaave Syndrome and may exacerbate the perforation due to insufflation during the procedure.

Treatment

An immediate surgical consult should be obtained. Definitive treatment will depend on the size and location of the perforation, whether preexisting disease is present, and whether or not it is contained. If the CT scan shows containment, treatment consists of NPO status, NGT placement with suction to remove gastric fluids and prevent further contamination, broad spectrum IV antibiotics and parenteral nutrition. If the perforation is not contained surgical repair is indicated. Liberal narcotic analgesia and anti-emetics to prevent additional valsalva induced barotrauma should be given as early as possible in the course of treatment.

Complications

Any delay in diagnosis or treatment will result in increased morbidity and mortality. Death due to spontaneous esophageal rupture is generally reported in 20% to 40% of treated cases. Complications of surgical repair include persistent esophageal leaks, mediastinitis, and sepsis. Despite adequate repair, continued leakage may occur in up to 30% of patients.

CLINICAL APPROACH

Chest pain (CP) is responsible for more than 6 million visits per year to the ED in the US. The evaluation of acute CP should always begin with a complete history focusing on quality, radiation of pain, context of onset, duration, risk factors, and exacerbating factors.

Due to the high morbidity and mortality of myocardial infarction, an ECG should be obtained immediately upon presentation to help assess the possibility of acute ischemia. Even when NCCP is suspected, reevaluation may be necessary if traditional cardiac risk factors suggest a high pretest probability of cardiac disease. One recent study showed that 2.8% of a study population diagnosed with NCCP had adverse cardiac events within 30 days. This correlates well with the reported missed diagnosis rate of 1% to 5% for patients presenting to the emergency department with acute myocardial infarction. The pretest probability of a cardiac etiology for an episode of CP increases with the patient age and positive risk factor profile.

How sensitive is ECG in predicting cardiac disease? That depends on how you ask the question. A meta-analysis of exercise tolerance test results reported a sensitivity of 68% and a specificity of 77% for cardiac ischemia. A single ECG cannot logically be any better than this result. Another study reported ECG abnormalities in 50% to 90% of patients who ultimately died of MI. In short, a work up including ECG with and without pain and serial cardiac enzymes is probably indicated at initial presentation for any patient with a moderate or greater pretest probability of cardiac disease before diagnosing them with NCCP.

NCCP is generally defined as CP occurring in the presence of normal large epicardial coronary artery anatomy and thus, not related to myocardial ischemia. A small percentage of these patients may have ischemia due to vasospasm or isolated distal arterial disease, but this does not change the general approach or differential diagnosis. The global differential diagnosis of NCCP for any patient should include the following etiologies in decreasing order of prevalence: musculoskeletal, esophageal/GI, psychiatric, pulmonary, other cardiac causes (pericarditis, etc), and other/nonspecific. Despite the preceding statement, the most common cause of NCCP seen in the ED is GERD.

Differential Diagnosis of Noncardiac Chest Pain

Musculoskeletal Chest Pain: Musculoskeletal CP is usually associated with a history of trauma, specific injury or repetitive use. It should be reproducible on careful examination by palpation or through specific motions or movements of the involved anatomic structures. The pain may also be pleuritic, or associated with deep breathing. Patients are frequently extremely anxious, making the diagnosis more difficult. Associated symptoms should be minimal once the effect of anxiety has been discounted. A detailed knowledge of the muscular and boney anatomy of the neck, thorax, and upper abdomen is necessary to fully evaluate this cause of NCCP. Frequently the only evaluation needed will be a careful and complete history and physical examination. Nonetheless, chest radiography (CXR) is appropriate if boney or pulmonary disease is being considered. Bedside ultrasound or formal echocardiography may be used to rule out pneumothorax, pericarditis, structural heart disease or aortic pathology. Liberal utilization of EKG is indicated and appropriate. Once the diagnosis has been made, appropriate care includes reassurance, analgesics, nonsteroidal anti-inflammatory drugs (NSAIDs), and temporary behavioral restrictions to minimize re-injury. Follow-up examination in 1 to 2 weeks is recommended with their PMD.

Esophageal and Gastrointestinal Causes of Chest Pain: Multiple pathologies of the esophagus may result in CP. These include reflux and chemical esophagitis, esophageal ulceration, foreign body, perforation and spasm. Esophageal perforation is discussed elsewhere in this chapter. Esophageal foreign body is usually obvious from the patient history except in young children and the elderly. In the case presented above, aspirated foreign body is certainly a possibility. CXR and neck films will identify radiopaque foreign bodies other objects require upper endoscopy for further evaluation. The remaining pathologic entities may be grouped under the label of GERD. The history in patients with GERD may include epigastric pain at night when lying supine and a correlation with large meals near bedtime. Caffeine, alcohol and tobacco use all decrease lower esophageal sphincter pressure, and thus increase the likelihood of reflux. NSAIDs and alcohol use both injure the esophageal mucosa. Long term reflux and esophagitis induce typical histologic changes in the distal esophageal mucosa and Barrett esophagus, that are diagnostic on biopsy. Physical examination is nonspecific and may show only subxiphoid or epigastric tenderness. Acute treatments are not helpful from the standpoint of narrowing the differential possibilities. Nitroglycerin relieves GERD associated esophageal spasm but also relieves cardiac CP. The classic “GI cocktail” of antacid, lidocaine and donnatal relieves GERD pain, but also anesthetizes nerves carrying pain sensations from other potentially lethal mediastinal pathologies. Thus, neither of these treatments should ever be used to rule out or rule in any single diagnosis. Long-term management of esophageal disorders includes behavior modification to prevent nocturnal reflux and various medications (PPI, H2 blocker, mucosal protectants) to allow healing of the esophageal mucosa. Peptic ulcer disease (PUD) is frequently associated with GERD. Triple therapy with PPI, H2 blockers, and antibiotics for eradication of Helicobacter pylori have resulted in >90% healing rate. Referral to a gastroenterologist for ongoing management is appropriate in more complicated cases.

Diseases of the upper abdominal organs may cause NCCP. Hepatitis can cause pain that radiates into the upper right chest due inflammation of the right hemidiaphragm. Cholecystitis can result in substernal CP and subxiphoid epigastric pain. Pancreatitis can cause pain that radiates from the epigastrum into the back and may radiate into the left chest in association with transudative pulmonary effusion. Splenic hematoma or infarction can result in CP that radiates to the left upper chest due to inflammation of the left hemidiaphragm.

Psychiatric Causes of Chest Pain: Depression frequently results in complaints of chest or abdominal pain. The patient may or may not be aware of the depression. Patients complaining of CP should be questioned about symptoms of depression such as anhedonia; early morning awakening, insomnia, and loss of interest or pleasure in normal activities. Physical examination in the organically depressed may reveal emotional lability, flattened affect, suicidal ideation, or psychomotor retardation but is generally negative for specific findings associated with other physical causes of CP. Somatization is the term applied to a specific type of conversion disorder where the patient depressive symptoms find focus in a specific physical complaint such as CP or abdominal pain. The patient may be fixated on the presenting physical complaint and unaware of the depressive component to their complaint. Diagnostic work up of this type of patient should be guided by a careful history and physical. A reasonably extensive initial diagnostic work up is indicated. Diagnosis of a psychiatric etiology for CP must be a diagnosis of exclusion. Recent meta-analysis has revealed a potential association between the diagnoses of panic attack with anxiety induced CP and CAD in the primary care and ED settings. The care provider should also keep in mind that depression may coexist with other organic causes of CP and that this risk increases with age. Also to be kept in mind is that depression alone may have lethal consequences and requires accurate diagnosis and aggressive therapy by a mental health care professional.

Pulmonary Causes of Che st Pain: The lungs, air passages and pleura can all cause chest discomfort. Irritation of the pleura can be caused by peripheral pneumonic inflammation from infectious causes, such as pneumonia; ischemia due pulmonary embolism or infarction; or mechanical irritation due to disruption of normal anatomy of the visceral and/or parietal pleural due to pneumothorax, pneumomediastinum, exudative effusions and some cancers. Infectious causes of CP will often be suspected if fever and hypoxia are present. Pneumothorax or pulmonary embolism should be suspected when the patient has acute unilateral pleuritic pain. Unilateral pleuritic CP ipsilateral to blunt chest trauma strongly suggests pneumothorax, but pneumothorax may occur spontaneously. Tracheal deviation, hypoxia, hypotension and hyper-resonance of the hemithorax contralateral to the tracheal deviation indicate the presence of tension pneumothorax. This is an immediately life threatening condition that requires emergency needle decompression of the hyper-resonant hemithorax with a large gauge needle or angiocath. If available, bedside ultrasound (US) may rapidly diagnose pneumothorax in trained hands. If the patient is unstable, treatment of tension pneumothorax should never be delayed for radiographic confirmation. Occult hypoxia may be uncovered by obtaining ambulatory pulse oximetry. Physical examination of the chest may reveal rales, rhonchi, wheezing, decreased breath sounds, signs of consolidation or effusion, and/or abnormalities of the chest wall. These finding may occur together or in isolation and should be correlated with the history and two view CXR to accurately determine the cause and scope of the patient pathology. If an infectious etiology is suspected in a patient likely to require admission to the hospital, then antibiotics should be administered as early as possible. Current nationwide quality improvement efforts sponsored by Centers for Medicaid and Medicare Services (CMS) specify ≤6 hour window, from time of patient arrival, for initial antibiotic administration in pneumonia patients admitted for inpatient care.

Other Causes of Chest Pain: Pericarditis is a non-ischemic cause of cardiac CP that results from inflammation of the pericardium, the connective tissue sack which envelopes the hear t. The pain associated with this entity is worse when supine and improves with erect posture or sitting up. Auscultation of the heart may reveal a rough scratchy sound very different from a murmur associated with cardiac systole and diastole. This is known as a pericardial friction rub. ECG findings vary over the course of the disease but, in general, are characterized by diffuse ST segment elevations. Many causes of pericarditis exist. These include but are not limited to postinfarction (Dressler syndrome), autoimmune, infectious (viral, bacterial, fungal), and traumatic. Viral infection is the most common non-infarction cause. Evaluation should include ECG, serial cardiac enzymes, and echocardiography. Until proven otherwise, ischemic cardiac disease should remain high in the differential. Echocardiography can demonstrate a thickened pericardium or a pericardial effusion typical of the disease and rule out the potentially lethal complication of pericardial tamponade. Tamponade may develop if pressure from the effusion is great enough to significantly impair cardiac filling. Emergent treatment consists of immediate pericardial drainage by needle aspiration. Treatment of pericarditis is more subacute and consists of analgesics, NSAIDs and frequent follow up until improvement is achieved.

A high suspicion must be maintained for aneurysm/dissection and pulmonary embolism to avoid missed diagnosis and potentially catastrophic outcomes for the patients. In both diseases the onset of CP can be sudden and associated with syncope. In aortic aneurysm the patient complains of CP radiating to the back. If the dissection involves the root of the aorta, and thus the coronary arteries, the patient may also have CP due to cardiac ischemia. Early echocardiography and/ or CT imaging will result in timely diagnosis. It is imperative to not treat blindly as the treatment for pulmonary embolism can exacerbate aortic dissection and/or aneurysmal rupture with life threatening results.

Another consideration in a vomiting patient with CP is the less serious but still potentially life threatening Mallory-Weiss tear. This entity involves a traumatic mucosal disruption usually located either at the gastroesophageal junction or gastric cardia. These esophageal tears may result in significant GI hemorrhage and can perforate if intense vomiting or retching continues. Deeper tears of the esophageal mucosa may manifest as intramural esophageal hematomas which can develop into full esophageal perforations with delayed rupture during the inflammatory weakening that accompanies healing.

Spontaneous pneumomediastinum without evidence of gastrointestinal or pleural source has been reported. It is usually seen in patients who have suffered from a valsalva episode against a closed glottis such as may occur during powerful coughing. Chest pain is universal and on exam a loud crunching sound may be heard as mediastinal air is squeezed by the beating heart (Hamman crunch). It is hypothesized that the mediastinal air arises from pulmonary interstitial emphysema caused by bronchoalveolar barotrauma. Crack cocaine smokers appear to be at particular risk. Despite the ominous CXR findings, this entity has a generally benign course. Nonetheless, the diagnosis of pneumomediastinum requires a diligent and exhaustive search for both pulmonary and gastrointestinal sources.

Breast pathology should be considered in the differential of NCCP in women presenting to the ED. Causes of breast pain include infectious etiologies, mastitis or abscess, benign lumps, cysts, or inflammatory carcinoma. Physical examination may reveal an asymmetric breast mass with or without signs of significant local induration, erythema, and tenderness. The patient with more serious pathology is often febrile. Intravenous analgesics, antibiotics and early surgical consultation are appropriate.

The preceding list is not exhaustive and diff icult clinical cases may require evaluation for more esoteric causes of NCCP. In the geriatric population, many patients may have more than one operative diagnosis causing their NCCP. As such, it is imperative that early primary care follow-up be arranged for patients given this diagnosis.

Presentation of Boerhaave Syndrome (Pressure Ind uced Esophageal Rupture)

The classical presentation of Boerhaave Syndrom e follows an episode of forceful retching and vomiting and includes retrosternal CP and/or epigastric pain. Odynophagia, dyspnea, tachypnea, cyanosis, fever, and shock may all develop thereafter. In one review, 40% of patients had a history of heavy drinking, 41% suffered from PUD, 83% complained of pain, 79% had a history of vomiting, 32% presented with shock and 39% with dyspnea. The diagnosis of Boerhaave Syndrome may be delayed because the clinical presentation may not be typical and causes are broad: childbirth, defecation, seizures, and heavy lifting. Common misdiagnoses include: myocardial infarction, pancreati tis, lung abscess, pericarditis, an d spontaneous pneumothorax. Concurrent alcohol intoxication may also delay diagnosis.

COMPREHENSION QUESTIONS

51.1 I n the initial evaluation of most ED patient with CP, what is the most important diagnostic test?

A. Chest x-ray

B. ECG

C. Serum cardiac markers

D. Computed tomography

E. Cholesterol levels

51.2 Which of the following is the most common cause of NCCP?

A. Musculoskeletal

B. Gastrointestinal

C. Other–nonspecific

D. Psychiatric

E. Pulmonary

51.3 A 45-y ear-old man with a known history of Boerhaave syndrome and primary surgical repair 5 years ago presents to the ED complaining of 24 hours of increasing CP and shortness of breath, what diagnostic test should be ordered to rule out perforation?

A. ECG

B. CXR

C. CT thorax

D. Barium esophagram

ANSWERS

51.1 B. An ECG to evaluate the patient for evidence of acute ischemia is the first indicated test. The healthcare provider should always keep in mind that a negative ECG does not rule out cardiac pathology.

51.2 A. Musculoskeletal conditions is the most commo n cause. All the listed answers are causes of NCCP and are listed in decreasing order with respect to their prevalence in the general population. GERD is the most common cause of NCCP seen in ED patients.

51.3 C. CT of the thorax is by far the most sensitive and specific test in assessing for possible esophageal perforation, especially in this patient given the time course of the symptoms and the history of prior surgery.

CLINICAL PEARLS

⯈ Boerhaave syndrome should always be considered in the differential diagnosis of acute CP and especially if the patient has been vomiting or performing any activity where barotrauma may have been sustained due to valsalva maneuver.

⯈ Gastrografin should be used instead of barium to avoid severe mediastinal and intra-pleural inflammatory reactions when doing contrast studies to locate the site of esophageal perforation.

⯈ GI Cocktail can not be used to reliably rule out a cardiac etiology for an episode of CP.

⯈ Response to a trial of sublingual nitroglycerin does not distinguish

between coronary artery disease and GERD induced esophageal spasm.

⯈ A single normal ECG can not be used to make the diagnosis of NCCP.

⯈ A significant percentage of patients (2%-3%) labeled with a diagnosis of NCCP will have an adverse cardiac event within 30 days.

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