A 64-year-old Caucasian woman with past medical history of dyslipidemia and Parkinson's disease was diagnosed with atrial fibrillation and started on treatment with apixaban and metoprolol. She later had a dual chamber pacemaker placement for sick sinus syndrome and her anticoagulation was held for 3 days prior to procedure. She was discharged home in a stable condition without any immediate perioperative complications and the next day she was started back on her anticoagulation. After about 3 weeks, she developed left sided chest pain radiating to her throat, worse with inspiration, shortness of breath, and passed out for a few seconds. She recovered spontaneously but her chest pain and shortness of breath persisted so she presented to the emergency room (ER).
On presentation, her blood pressure was 70/40 mm Hg with pulse of 110 beats per minute, respiratory rate of 24 per minute, and oxygen saturation of 94% on room air. She did not have any pericardial rub or knock but had elevated jugular venous distention with mild pedal edema. Chest X-ray immediately after pacemaker implantation and ER presentation are as shown in Figure 1. An electrocardiogram did not show any clear ST elevation or ST depressions. Troponin was 0.02 (normal 0.000-0.029 ng/ml), WBC count was 14 K/μL (normal 3.7-11 K/μL), US-CRP of 7.9 (normal <1 mg/L), and ESR 15 mm/hr (normal 0-15 mm/hr). A bedside transthoracic echocardiogram showed a moderate sized pericardial effusion with diastolic collapse of right sided chambers and she underwent urgent pericardiocentesis. The pericardial fluid was clear and negative for infections or neoplastic process. Interrogation of pacemaker showed normal lead thresholds and impedance. Follow up electrocardiogram did not show any evolving changes and troponins were stable.
Figure 1: Chest X-Ray
Which of the following is the most likely etiology of her presentation?
The correct answer is: D. Post-cardiac injury syndrome.
This patient has developed cardiac tamponade due to post-cardiac injury syndrome (PCIS) after her permanent pacemaker placement. The chest X-rays show increase in size of cardiac silhouette after the procedure and transthoracic echocardiogram showed signs of tamponade for which she underwent urgent pericardiocentesis. CT thorax did not show any evidence of perforation or pulmonary embolism but showed trace pleural effusions. Acute coronary syndromes, aortic dissection, acute pulmonary embolism, and perforation of pacemaker leads can present similarly with chest pain, shortness of breath, possibly hypotension, and syncope, and need to be evaluated as appropriate. The latent period after the procedure and the increase in inflammatory markers point to an immune-mediated process for the inflammatory process and the pericardial effusion after cardiac injury. Delayed micro perforation from the pacemaker leads can be the initiating factor in some cases. Patients may have pleural effusions, pulmonary infiltrative changes, fever, and EKG changes of pericarditis. A pericardial rub may only be heard in about 30-60% of patients.1 Anticoagulants, female sex, and even minor bleeding during cardiac procedures are some of the predictors that have been associated with the development of this syndrome.
PCIS is an inflammatory process which represents an umbrella diagnosis and includes post-myocardial infarction syndrome, post-pericardiotomy syndrome (PPS) or post-cardiotomy syndrome and post-traumatic pericarditis which may be iatrogenic or not.1 The PCIS syndrome is differentiated from acute or delayed perforation after procedures which is a localized mechanical complication. In the early days this was felt to be a rheumatologic condition and was noted after mitral valve surgery.2,3 PPS or post-cardiotomy syndrome is due to surgical procedures and is the commonest form with an incidence of 10-40% of patients who underwent surgery.4,5 Dressler's syndrome, first described in 1956, is the inflammatory syndrome that is seen typically as a late complication after acute myocardial infarction but its incidence has decreased to <0.5% in this age of early percutaneous intervention. There has however been a steady increase in cardiac procedures, such as such as percutaneous and electrophysiological procedures, over the years.
Early and late complications after pacemaker implantation range from 3.2 to 7.5%.6,7 Pericardial complications including pericardial effusions after pacemaker have been reported around 1-5%.1,8,9 One of the first reports of PCIS after pacemaker implantation has been reported by Dressler in 1962 in two out of 11 patients.10 In a CT-based study by Hirschl et al.,11 about 15% of patients who received implantable pacemakers and defibrillators has asymptomatic lead perforation, more so with atrial leads and active fixation leads.
The pathogenesis of PCIS is presumed to be immune-mediated from release of auto-antigens with any cardiac injury leading to antibodies in predisposed individuals. Imazio et al. have proposed diagnostic criteria for PPS with at least two out of five being required:12 1) unexplained fever; 2) pericarditic or pleuritic chest pain; 3) friction rub; 4) new or worsening pericardial effusion; or 5) new or worsening pleural effusion with elevated CRP. However, there is a need for further studies to evaluate the applicability of these criteria to all post-cardiac injury syndromes. ESC 2015 guidelines on pericardial diseases recommend anti-inflammatory therapy (Class IB),13 which has shown to reduce incidence of pericarditis by 50% compared to placebo,14 and colchicine (Class IIa B), which reduced the incidence for prevention as well as recurrence rate by half for PPS.5,15 While data is somewhat lacking, the prognosis does not appear to be worse as shown by the reported recurrence rate of 10-15% with PPS compared to 15%-30% with pericarditis as a whole.16,17
In conclusion, this patient has PCIS, which is a diagnosis of exclusion based on history of prior cardiac or pleural injury,1 with a latent period which may range from days to months; presenting with signs and symptoms of pleuro-pericardial inflammation and elevated inflammatory markers. Major differentials like acute myocardial infarction, pulmonary embolism, dissection, and lead perforations should be evaluated as appropriate.
Imazio M, Hoit BD. Post-cardiac injury syndromes. An emerging cause of pericardial diseases. Int J Cardiol 2013;168:648-52.
Janton O, Golver R, O'Neill T, Gregory J, Froid G. Results of the surgical treatment for mitral stenosis: analysis of 100 cases. Circulation 1952;6:321-33.
Soloff LA, Zatuchni J, Janton OH, O'Neill TJ, Glover RP. Reactivation of rheumatic fever following mitral commissurotomy. Circulation 1953;8:481-97.
Finkelstein Y, Shemesh J, Mahlab K, et al. Colchicine for the prevention of postpericardiotomy syndrome. Herz 2002;27:791-4.
Imazio M, Trinchero R, Brucato A, et al. Colchicine for the Prevention of the Post-pericardiotomy Syndrome (COPPS): a multicentre, randomized, double-blind, placebo-controlled trial. Eur Heart J 2010;31:2749-54.
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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) Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J 2015;36:2921-64.
Horneffer PJ, Miller RH, Pearson TA, Rykiel MF, Reitz BA, Gardner TJ. The effective treatment of postpericardiotomy syndrome after cardiac operations. A randomized placebo-controlled trial. J Thorac Cardiovasc Surg 1990;100:292-6.
Verma S, Eikelboom JW, Nidorf SM, et al. Colchicine in cardiac disease: a systematic review and meta-analysis of randomized controlled trials. BMC Cardiovasc Disord 2015;15:96.
Imazio M, Brucato A, Rovere ME, et al. Contemporary features, risk factors, and prognosis of the post-pericardiotomy syndrome. Am J Cardiol 2011;108:1183-7.
Cremer PC, Kumar A, Kontzias A, et al. Complicated pericarditis: understanding risk factors and pathophysiology to inform imaging and treatment. J Am Coll Cardiol 2016;68:2311-28.