Dressler syndrome (or postmyocardial infarction syndrome, post cardiac injury syndrome, and post pericardiotomy syndrome) is a complication of pericardial surgery or injury and myocardial infarction, presenting as a form of acute pericarditis. Dressler syndrome presents with low grade fever, pleuritic chest pain, and pericardial friction rub.
History
The most constant symptom of dressler syndrome is pleuritic chest pain, typically located retrosternally or on the left precordial region and which is charasteristically relieved by leaning forward and worsened by lying flat [10] [11]. Another common feature of dressler syndrome is fever, which is often low grade [11]. Pneumonitis also occurs sequel to pericardial inflammation.
A history of myocardial infarction, pericardial surgery or injury is required to make a diagnosis of dressler syndrome. A history of risk factors of myocardial infarction should also be sought, these risk factors include obesity, hypertension, family history of ischaemic heart diseases, smoking, and dyslipedemia [10] [11].
Physical examination
Characteristically, on physical examination in dressler syndrome, a pericardial rub is heard on auscultation. This sound results from friction between the inflamed visceral and parietal pericardial layers. The sound is best heard near the end of expiration over the left lower sternal border with the patient leaning forward. Pleural effusion is also a typical finding in dressler syndrome. Another typical finding on physical examination is a dull percussion note in the left upper lung zone and scapular area, this is due to the collapse of the left lung sequel to compression by the enlarged pericardium which results from massive effusion into the pericardial space. This is called Ewart's sign. It is also common to find tachycardia and tachypnea in dressler syndrome.
Work up approach considrations in dressler syndrome includes initial baseline laboratory investigations, cardiac imaging, and pericardiocentesis.
Complete blood count reveals leukocytosis with left shift. However, blood cultures are typically negative to rule out a bacterial cause of acute pericarditis. Blood cultures are, therefore, very necessary as part of the initial laboratory studies. Serology reveals a high titer of antimyocardial antibodies. Typically, erythrocyte sedimentation rate (ESR) and C-reactive protein level are evelated. Cardiac enzyme levels are not necessary in the diagnosis of dressler syndrome as results remain unchanged as in patients without dressler syndrome who have had pericardial injury or myocardial infarction. As part of baseline investigations, pericardial fluid should be sent for microscopic analysis, biochemistry, and cultures.
Cardiac imaging includes electrocardiography (ECG), chest radiography, echocardiography, and cardiac magnetic imaging resonance (MRI) which are all indicated in dressler syndrome. However, echocardiography is the diagnostic modality of choice in dressler syndrome.
Chest radiographs typically show effacement of the costophrenic angles from pleural effusion, and an enlarged cardiac silhouette due to pericardial effusion.
Findings on the ECG include T-wave inversion, low QRS amplitude, and ST segment elevation. A low QRS amplitude is most commonly seen if the pericardial effusion is massive.
The echocardiography is more sensitive than chest radiography [12]. Echocardiography helps exclude other differentials such as congestive heart failure with the characteristic finding of fluid collection posterior to the left ventricle during systole. Cardiac MRI is indicated to clearly reveal pericardial fluid collection which has not been clearly delineated with echocardiography.
Pericardiocentesis is both a diagnostic and therapeutic procedure. Pericardial inflammation causes effusion and collection of fluid in the pericardial potential space, overtime, the fluid accumulated makes the pericardium massively enlarged compressing the heart, thereby restricting outflow and cardiac output. This is called cardiac tamponade and it requires urgent management. Echocardiography-guided pericardiocentesis is the recommended initial management method for significant pericardial effusion and cardiac tamponade. The drainage is usually done over 24-48 hours.
Initial evaluation and treatment of dresser syndrome in a hemodynamically stable patient is usually on an outpatient basis. Initial care is largely conservative with nonsteroidal anti-inflammatory drugs prescribed for up to 6 weeks, doses of which are tapered as the collected fluid reduces. Corticosteroids are indicated if treatment with NSAIDs fail to resolve the inflammatory process. Certain reports have revealed successful treatment of recurrent cases of dressler syndrome with a single high dose of intravenous immunoglobulin in a patient and a weekly administration of low dose methotrexate in another patient [13].
More severe cases, especially involving cardiac tamponade are managed as emergencies in an inpatient setting. Cardiac tamponade is treated with urgent pericardiocentesis, after which patients may be continued on outpatient care, if they show satisfactory improvement.
Surgery is indicated in cases of relapse or persistence of symptoms despite medical treatment. Surgical procedures indicated in these cases include open thoracotomy or video-assisted thoracoscopy and percutaneous gallon pericardiotomy [14]. These procedures basically serve the same purpose: creation of a pericardial window through which pericardial fluid drains. However, percutaneous balloon pericardiotomy is less invasive and involves flouroscopic-guided balloon catheterization [15]. In this procedure, the pericardial fluid is shunted through a surgical defect in the diaphragm into the abdominal cavity.
Generally, the prognosis of dressler syndrome is benign, with the outcome largely determined by the severity of the underlying cardiac disease. Most cases of dressler syndrome resolve within a few weeks, however, in a few cases, the symptoms may persist for a number of months. Relapses occur in up to 15% of patients [9]. Most relapses occur within 6 months of the inciting surgery or injury.
Complications of dressler syndrome include cardiac tamponade, constrictive pericarditis, hypoxaemia, and occlusion of a coronary bypass graft. Constrictive pericarditis is however, neither exclusively nor frequently caused by dressler syndrome.
Cardiac tamponade is the restriction of ventricular filling and outflow sequel to massive effusion and thickening of the pericardium. Cardiac tamponade, being a life-threatening condition, requires urgent pericardiocentesis. Cardiac tamponade occurs in about 1% of cases of dressler syndrome. In addition, occlusion of a coronary bypass graft is a rare complication of dressler syndrome.
Dressler syndrome has an unknown etiology, however autoimmunity has been associated with its pathogenesis [5]. This is suggested by serologic findings of antimyocardial antibodies. However, the presence of these antibodies are yet to be linked to either the cause or the result of this syndrome. Reports of pericardial surgeries showed that a high titer of the anti myocardial antibodies was associated with the development of dressler syndrome.
Dressler syndrome was first described by Dressler in 1956 in which he noted that the syndrome had an incidence rate of 3-4% of all cases of acute myocardial infarction [6]. However, the incidence rate is now much reduced because of better treatment methods for acute myocardial infarction. Dressler syndrome is rarely seen in children under 2 years.
The etiology and pathogenesis of dressler syndrome is not clearly understood, however, studies show that it most likely has an autoimmune basis [7]. Other hypotheses include viral infections and localized inflammatory processes. In over 60% of cases, viruses including cytomegalovirus, coxackie B and adenovirus, have been shown to be present in patients with this syndrome, thus suggesting that the syndrome may be an antibody response to a viral infection. Nonetheless, a study showed no presence of viruses in patients with the syndrome postpericardiotomy [8]. Dressler syndrome is most commonly associated with intramural myocardial infarction.
The incidence of Dressler's syndrome is much reduced by use of NSAIDs post-pericardial or cardiac surgery. However, prophylactic steroid use prior to cardiac surgery doesn't prevent this syndrome [16].
Dressler syndrome is an inflammatory reaction involving the pericardium, often occurring secondary to pericardial surgeries, injuries, and myocardial infarction, hence, Dressler syndrome is also referred to as post-myocardial infarction syndrome and postpericardiotomy syndrome [1]. Other common triggers of dressler syndrome include heart puncture, blunt trauma, pacemaker implantation, and coronary stenting [2].
The syndrome presents as an acute pericarditis with low grade fever, pleuritic chest pain, pericardial friction rubs and pericardial effusion which could progress into cardiac tamponade, a life-threatening condition [3] [4]. In a few recurrent cases of dressler syndrome, cardiac tamponade may also recur. Dressler syndrome, in few cases, may recur years after the inciting surgery and initial treatment.
Diagnosis of dressler syndrome is largely clinical, with characteristic findings on physical examination including pericardial friction rub, and a history of myocardial infarction, cardiac surgeries or injuries. Although, dressler syndrome is somewhat similar to peri-infarction pericarditis which follows a myocardial infarction, dressler syndrome often presents several weeks to months after an infarction as opposed to the early onset of peri-infarction pericarditis.
Common surgical procedures associated with the development of dressler syndrome include surgical corrections of atrial septal defects, ventricular septal defects and fallot's tetralogy, and cardiac transplantation.
The pathophysiology of dressler syndrome is not clearly understood, however, it is theorized to be a result of an autoimmune process, as evidenced by the presence of antimyocardial antibodies, and its sensitivity to anti-inflammatory drugs.
Clinical evaluation is always supported by laboratory investigation to confirm a diagnosis of dressler syndrome. Cardiac imaging provides characteristic features for the diagnosis and could be employed therapeutically to treat this syndrome. Treatment involves drainage of the peicardial fluid and administration of anti-inflammatory drugs. Cardiac tamponade is, however, treated as an emergency with an urgent drainage of the collected pericardial fluid.
Dressler syndrome is an inflammatory condition of the covering of the heart, the pericardium, after a heart attack, or after an injury or surgical procedure involving the heart or the pericardium. It is a condition characterised by chest pain, and fever. This pericardium is made up of two layers.
Causes
Doctors are not so clear about how the disease results, although they have postulated a theory, based on findings in a lot of cases, that the disease may result from the body's antibodies attacking the heart and pericardium. The antibodies were originally meant to repair the damaged heart tissue, but end up damaging it by causing excessive inflammation.
Symptoms
Dressler syndrome typically presents with pain in the middle of the chest which gets worse with lying flat and is relieved by leaning forward. The pain is usually described as an intermittent squeezing, burning sensation as opposed to a heart attack in which the pain is continuous. Another common symptom of this disease is a mild fever.
Prognosis
The inflammation of the pericarditis leads to release of fluids from the blood vessels around the pericardium. This fluid collects gradually, and if the condition is untreated, may collect till it becomes massive compressing the heart and restricting the heart's ability to pump out blood. This phenomenon is called cardiac tamponade and is a medical emergency.
Diagnosis
Doctors can make a diagnosis of dressler syndrome from the history and examination findings. A history of the typical chest pain and fever which occur after heart surgery or a heart attack suggests dressler syndrome. Your doctor would listen for a typical sound called pericardial rub, on placing the stethoscope on your chest. This sound is due to the irritation of the two layers of the pericardium when you breathe.
To confirm this diagnosis, however, your doctor may require radiological films including Chest X-ray, CT scan, echocardiography, and magnetic resonance imaging (MRI) of the heart. An electrocardiogram (ECG) may also be necessary. The echocardiograph is a technique which uses very high frequency sound waves to create an image of the heart to reveal the characteristic fluid collection in the pericardium and exclude other diseases which could cause similar symptoms. The ECG uses electrical wires plugged to the chest to check for the changes in the electrical activity of the heart.
Blood tests would also be ordered to reveal features characteristic of this condition.
Treatment
Treatment of this condition basically includes administration of over-the-counter pain medications such as advil and aspirin. Steroids are powerful immune suppressants which also serve to reduce the inflammation and pain in this condition. Doctors use steroids in testing dressler syndrome when over-the-counter pain medications do not resolve the symptoms.
The complications of this condition would also be treated. Cardiac tamponade is treated with urgent drainage of the fluid using a catheter. In severe and recurrent cases, surgery may be necessary to drain the fluid from the pericardium. Such surgical procedures create a hole in the pericardium through which the fluid drain to another safe site, usually in the abdomen.