Recurrent Pericarditis: Modern Approach in 2016

Recurrent pericarditis is one of the most troublesome complications of pericarditis occurring in about one third of patients with a previous attack of pericarditis. The pathogenesis is presumed to be autoimmune and/or autoinflammatory in most cases. The mainstay of therapy for recurrences is physical restriction and anti-inflammatory therapy based on aspirin or NSAID plus colchicine. Corticosteroids at low to moderate doses (e.g., prednisone 0.2 to 0.5 mg/kg/day) should be considered only after failure of aspirin/NSAID (and more than one of these drugs) or for specific indications (e.g., pregnancy, systemic inflammatory diseases on steroids, renal failure, concomitant oral anticoagulant therapy). One of the most challenging issues is how to cope with patients who have recurrences despite colchicine. A small subset of patients (about 5 %) may develop corticosteroid-dependence and colchicine resistance. Among the emerging treatments, the three most common and evidence-based therapies are based on azathioprine, human intravenous immunoglobulin (IVIG), and anakinra. After failure of all options of medical therapy or for those patients who do not tolerate medical therapy or have serious adverse events related to medical therapy, the last possible option is the surgical removal of the pericardium. Total or radical pericardiectomy is recommended in these cases in experienced centers performing this surgery. A stepwise approach is recommended starting from NSAID and colchicine, corticosteroid and colchicine, a combination of the three options (NSAID, colchicine and corticosteroids), then azathioprine, IVIG, or anakinra as last medical options before pericardiectomy.     

Treatment with aspirin, NSAID, corticosteroids, and colchicine in acute and recurrent pericarditis

Empiric anti-inflammatory therapy for acute and recurrent pericarditis is warranted for viral and idiopathic cases that represent most cases of pericarditis in developed countries. For specific uncomplicated etiologies, such as systemic autoimmune diseases and postpericardiotomy syndromes, the same drugs are also indicated. Aspirin and non-steroidal anti-inflammatory drugs (NSAID) are mainstay of therapy with the possible adjunct of colchicine, especially for recurrences. Corticosteroids are a second choice for difficult cases requiring multi-drug therapies and specific medical conditions (i.e., specific cases with systemic autoimmune diseases, postpericardiotomy syndrome, and pregnancy). Medical therapy of pericarditis should be individualized as much as possible providing the attack dose every 8 h to ensure full daily control of symptoms and till remission and C-reactive protein normalization, and then tapering should be considered. The present paper will review current evidence for the treatment of acute and recurrent pericarditis with aspirin, NSAID, corticosteroids, and colchicine.     

Colchicine for large pericardial effusion

On the basis of our reported experience with colchicine for recurrent pericarditis, we administered colchicine to two patients with large pericardial effusions complicating idiopathic pericarditis. The first was a 26-year-old male who showed clinical deterioration following emergency pericardiocentesis and aspirin (3 g/day) for 10 days; the second was a 2-year-old girl who was unsuccessfully treated with aspirin (100 mg/kg/day) for 2 weeks, followed by corti-costeroids for 7 months. Administration of colchicine (1 mg/ day) instead of aspirin in the first case, and with a rapid tapering-off of the corticosteroids in the second case, led to complete regression of the pericardial effusion on echocardiography within 1 week and 1 month, respectively. Colchicine was discontinued after 1 month in the first patient and was continued for 6 months in the child. Neither has had a recurrence at 24 and 6 months of follow-up, respectively. No side effects of colchicine were observed. We conclude that colchicine may be effective in the treatment of large pericardial effusion when therapy with nonsteroidal anti-inflammatory drugs and/or corticosteroids fails.     

Pericarditis and Pericardial Effusion: Management Update

Prompt recognition of the signs and symptoms of pericardial disease is critical so that appropriate treatments can be initiated. Acute pericarditis has a classical presentation, including symptoms, physical examination findings, and electrocardiography abnormalities. Early recognition of acute pericarditis will avoid unnecessary invasive testing and prompt therapies that provide rapid symptom relief. Non-steroidal anti-inflammatory drugs (NSAIDs) remain first-line therapy for uncomplicated acute pericarditis, although colchicine can be used concomitantly with NSAIDS as the first-line approach, particularly in severely symptomatic cases. Colchicine should be used in all refractory cases and as initial therapy in all recurrences. Aspirin should replace NSAIDS in pericarditis complicating acute myocardial infarction. Systemic corticosteroids can be used in refractory cases or in those with immune-mediated etiologies, although generally should be avoided due to a higher risk of recurrence. Pericardial effusions have many etiologies and the approach to diagnosis and therapy depends on clinical presentation. Pericardial tamponade is a life-threatening clinical diagnosis made on physical examination and supported by characteristic findings on diagnostic testing. Prompt diagnosis and management is critical. Treatment consists of urgent pericardial fluid drainage with a pericardial drain left in place for several days to help prevent acute recurrence. Analysis of pericardial fluid should be performed in all cases as it may provide clues to etiology. Consultation of cardiac surgery for pericardial window should be considered in recurrent cases and may be the first-line approach to malignant effusions, although acute relief of hemodynamic compromise must not be delayed. Constrictive pericarditis is associated with symptoms that mimic many other cardiac conditions. Thus, correct diagnosis is critical and involves identification of pericardial thickening or calcification in association with characteristic hemodynamic alterations using noninvasive and invasive diagnostic approaches. Constrictive physiology may occur transiently and resolve with medical therapy. In chronic cases, definitive therapy requires referral to an experienced surgeon for pericardiectomy.     

Use of colchicine in chronic pericardial effusion. Presentation of 2 clinical cases and review of the literature

Colchicine was introduced in 1987 for the treatment of recurrent pericarditis. Up to the present, papers have been published on a total of 117 patients treated with colchicine after the failure of treatment with FANS, corticosteroids and repeated pericardiocentesis. Here two cases of chronic pericardial effusion, one secondary to pericardiotomy, the second idiopathic, are reported. Both were recalcitrant to conventional therapy. Both patients were treated with 2 mg/die colchicine for 1 month followed by 1 mg/die for a further 5 months, without recurrence of the effusion after follow-up of 12 and 24 months respectively. No side-effects were observed. Colchicine is an anti-inflammatory drug which, by inhibiting various leukocyte functions, depresses the action of the leukocytes and of the fibroblasts at the site of the inflammation. We conclude that colchicine is effective in post-pericardiotomic and idiopathic chronic pericardial effusion as already reported in cases of recurrent pericarditis. Given the lack of side-effects, it could be considered as a drug of choice alternatively to FANS and corticosteroids.     

Prevention of Recurrent Pericarditis With Colchicine in 2012

The most troublesome complication of acute pericarditis is recurrent pericardial inflammation, which occurs in 15%–32% of cases. The optimal method for prevention has not been fully established; accepted modalities include nonsteroidal anti‐inflammatory drugs, corticosteroids, immunosuppressive agents, and pericardiectomy. Over the last years, objective clinical evidence has matured and clearly indicates the important role and beneficial clinical effect of colchicine therapy in preventing recurrent pericarditis caused by various etiologies. Colchicine‐treated patients consistently display significantly fewer recurrences and longer symptom‐free periods, and even when attacks occur, they are weaker and shorter in nature. Notably, pretreatment with corticosteroids substantially attenuates the efficacy of colchicine, causing significantly more recurrences and longer therapy periods. The safety profile seems superior to other drugs, such as corticosteroids and immunosuppressive drugs. Colchicine is a safe and effective modality for the treatment and prevention of recurrent pericarditis, especially as an adjunct to other modalities, because it provides a sustained benefit, superior to all current modalities. The authors have no funding, financial relationships, or conflicts of interest to disclose.     

Management Strategies in Pericardial Emergencies

BACKGROUND: The most frequent pericardial emergency is cardiac tamponade, but complications of an acute coronary syndrome and aortic dissection may also involve the pericardium. Acute pericarditis can also represent a medical emergency due to chest pain of upsetting intensity. Decompensations in chronic advanced constriction and in the clinical course of purulent pericarditis necessitate critical care as well. DIAGNOSIS AND MANAGEMENT: The diagnosis of cardiac tamponade is based on clinical presentation and physical findings, confirmed by echocardiography and cardiac catheterization. Tamponade is an absolute indication for urgent drainage, either by pericardiocentesis or surgical pericardiotomy. The approach for pericardiocentesis can be subxiphoid or intercostal using echocardiographic or fluoroscopic guidance. Urgent drainage, combined with intravenous antibiotics, is also mandatory in suspected purulent pericarditis. If confirmed, it should be combined with intrapericardial rinsing (best by a surgical drainage). Pericardiocentesis is contraindicated in cardiac tamponade complicating aortic dissection. This condition should immediately lead to cardiac surgery. Although pericardiectomy is the only treatment for permanent constriction, this procedure is contraindicated when extensive myocardial fibrosis and/or atrophy are demonstrated. CASE STUDY: Iatrogenic tamponade may occur during percutaneous mitral valvuloplasty, implantation of pacemakers, electrophysiology and radiofrequency ablation procedures, right ventricular endomyocardial biopsy, percutaneous coronary interventions, and rarely during Swan-Ganz catheterization. The authors report on a 79-year-old who suffered coronary perforation and cardiac tamponade during elective stent implantation. Tamponade was successfully treated with pericardiocentesis and implantation of a membrane-covered graft stent. Subsequent recurrent pericarditis/postpericardial injury syndrome with moderate pericardial effusion was initially treated with aspirin and then with aspirin and colchicine. At 6 months, the patient is in stable remission even after withdrawal of colchicine. CONCLUSION: Natural history of pericardial diseases can be complicated with pericardial emergencies requiring prompt diagnosis, intensive care with hemodynamic monitoring, and early aggressive management. Medical supportive measures, drainage of pericardial effusion, surgical pericardiotomy, and pericardiectomy should be applied when needed with no delay. This procedural approach also applies to iatrogenic interventions leading to tamponade.     

Transient Constrictive Pericarditis: Current Diagnostic and Therapeutic Strategies

Transient constrictive pericarditis is increasingly recognized as a distinct sub-type of constrictive pericarditis. The underlying pathophysiology typically relates to impaired pericardial distensibility, associated with acute or sub-acute inflammation, rather than the fibrosis or calcification often seen in chronic pericardial constriction. Accordingly, patients may present clinically with concomitant features of pericarditis and constrictive physiology. Non-invasive multimodality imaging is advocated for diagnosis of transient constrictive pericarditis. Echocardiography remains the mainstay for initial evaluation of the dynamic features of constriction. However, cardiac magnetic resonance imaging can provide complimentary functional information, with the addition of dedicated sequences to assess for active pericardial edema and inflammation. Although transient pericardial constriction can spontaneously resolve, institution of anti-inflammatory therapy may hasten resolution or even prevent progression to chronic pericardial constriction. Non-steroidal anti-inflammatory agents remain the initial treatment of choice, with subsequent consideration of colchicine, steroids, and other immune-modulating agents in more refractory cases.     

Colchicine in Treatment of Intractable Postpericardiotomy Syndrome in an Elderly Patient

Postpericardiotomy syndrome is a specific type of acute pericarditis because of a delayed pericardial and/or pleural reaction after thoracic surgery. Relapse after aspirin, nonsteroidal anti-inflammatory drug, and/or steroid treatment or intractable to this conventional therapy causes a troublesome situation. Colchicine was first proposed for treatment of recurrent pericarditis in 1987. A number of investigators have reported the efficacy and safety of colchicine in combination therapy for recurrent pericarditis. Recently, Colchicine for Recurrent Pericarditis and Colchicine for Acute Pericarditis studies suggested that colchicine is useful in the first attack of acute pericarditis, and corticosteroid therapy given in the first attack favors the recurrence of pericarditis. In this report, we present an 82-year-old woman with severe tricuspid regurgitation and moderate-to-severe mitral regurgitation because of rheumatic heart disease, postpericardiotomy syndrome with severe pleural and pericardial effusion developed after the open-heart surgery. Both pleural and pericardial effusion was intractable to steroid therapy. Colchicine and steroid combination therapy made the syndrome remission rapidly. The total course of colchicines therapy was 2.5 months. There was no recurrence after 1 year of clinic follow-up.     

Diagnostik und Therapie der Perikarditis und des Perikardergusses

This article describes the diagnostics, differential diagnostics, multimodal imaging, medicinal and invasive diagnostic therapy of acute and chronic pericarditis, constrictive pericarditis, pericardial effusion and cardiac tamponade under etiological aspects and on the basis of the guidelines of the European Society of Cardiology (ESC). The starting point of the decision tree is the symptomatic patient with echocardiographic evidence of pericardial effusion. The principle feature of the diagnostics is the etiopathogenetic allocation of the pericardial disease which influences the clinical picture, course therapy and prognosis. Infectious pericarditis (e.g. viral, bacterial and tuberculous) is differentiated from sterile autoreactive pericarditis and from neoplastic pericardial effusion by the cytology of the effusion and immunohistological and molecular investigations of the pericardial and epicardial biopsies. Pericardioscopy plays an important role in the recognition of suspicious areas. In many cases intrapericardial administration of cisplatin for neoplastic pericardial effusion and instillation of triamcinolone for autoreactive pericarditis prevent recurrence just as a treatment of several months with colchicine.     

Efficacy of colchicine in recurrent acute idiopathic pericarditis

Acute idiopathic pericarditis is complicated by recurrence in 15 to 30% of cases. The preventive treatment of recurrences is not well codified. Aspirin, non-steroidal anti-inflammatory drugs and corticoids are the commonest prescribed treatments. The objective of this study was to assess the value of colchicine in the prevention of recurrences of acute idiopathic pericarditis. Twenty-eight cases of recurrent acute pericarditis (2 episodes, 1 month between each episode) admitted to a department of internal medicine between 1989 and 1999 were reviewed. Analysis was concentrated on the 13 idiopathic forms. The subjects were 7 women and 6 men with an average age of 41 years (10-62) at the time of the first episode of acute pericarditis. These 13 patients were treated with colchicine (1 to 2 mg/day) after failure of conventional treatment (aspirin 13/13, NSAID 13/13, steroids 9/13 and pericardiocentesis 3/13). Ten patients were followed up regularly (6 months after starting colchicine) and were improved with respect to number, duration and intensity of their recurrences. The average duration of colchicine therapy was 17 +/- 14 months (6-48). Progressive withdrawal of NSAID and steroids was obtained in 8/10 cases. The authors conclude that colchicine is useful in the prevention of recurrence of acute pericarditis. It may be proposed as treatment of choice, especially in the idiopathic forms.     

New Developments in the Management of Recurrent Pericarditis

Recurrent pericarditis is a common and troublesome complication that affects 15%-30% of patients with a previous episode of pericarditis. However, the pathogenesis of these recurrences is not well understood, and most cases remain idiopathic. Recent advances in medical therapy, including the use of colchicine and anti-interleukin-1 agents like anakinra and rilonacept, have suggested an autoinflammatory rather than an autoimmune mechanism for recurrences with an inflammatory phenotype. As a result, a more personalized approach to treatment is now recommended. Patients with an inflammatory phenotype (fever and elevated C-reactive protein level) should receive colchicine and anti-interleukin-1 agents as first-line therapy, whereas those without systemic inflammation should receive low to moderate doses of corticosteroids (eg, prednisone 0.2-0.5 mg/kg/d as an initial dose) and consider azathioprine and intravenous human immunoglobulins in the case of corticosteroid failure. Tapering of corticosteroids should be slow after achieving clinical remission. In this article, we review the new developments in the management of recurrent pericarditis.     

New directions in diagnosis and treatment of pericardial disease. A project of the Taskforce on Pericardial Disease of the World Heart Federation

New directions in the diagnosis and treatment of pericardial diseases synthesize the achievements of modern imaging with molecular biology and immunology techniques. Comprehensive and systematic implementation of new techniques of pericardiocentesis, pericardial fluid analysis, pericardioscopy, epicardial and pericardial biopsy, as well the application of comprehensive molecular biology and immunology techniques for pericardial fluid and biopsy analyses have opened new windows to the pericardial diseases, permitting early specific diagnosis and creating foundations for etiologic treatment in many cases. In patients with recurrent pericarditis, resistant to conventional treatments, as well as in patients with neoplastic pericarditis an alternative intrapericardial treatment regimen was suggested by the Taskforce on Pericardial Diseases of the World Heart Federation. Intrapericardial application of medication avoids systemic side effects with increased local efficacy. The following protocols are proposed: CIRP (colchicine in recurrent pericarditis)--colchicine vs placebo in chronic/recurring pericarditis without pericardiocentesis; TRIPE (triamcinolone in pericardial effusion)--intrapericardial instillation of triamcinolone + 6 months colchicine vs pericardial puncture without instillation + 6 months colchicine; NEPIN (neoplastic effusion and pericardial instillation)--pericardiocentesis and drainage + intrapericardial instillation of cisplatin or thiotepa.     

Pretreatment with corticosteroids attenuates the efficacy of colchicine in preventing recurrent pericarditis: a multi-centre all-case analysis.

AIMS: Effective prevention of recurrent pericarditis remains an important yet elusive goal. Corticosteroid therapy often needs to be continued for a prolonged period and causes severe side effects. We performed a multi-centre all-case analysis to investigate the efficacy of colchicine in preventing subsequent relapses of pericarditis, and addressed the hypothesis that pretreatment with corticosteroids may attenuate the beneficial effect of colchicine. METHODS AND RESULTS: One hundred and forty published and unpublished cases of patients treated with colchicine after at least two relapses of pericarditis were aggregated from European centres. Of those, 119 were included in the study group. Only 18% of the patients had relapses under colchicine therapy, and 30% after its discontinuation. There were significantly more relapses among male patients after colchicine treatment (36 vs. 17%, P=0.046), and those with previous corticosteroid treatment (43 vs. 13%, P=0.02). Multivariate logistic regression analysis identified previous corticosteroid therapy (OR 6.68, 95% CI: 1.65-27.02) and male gender (OR 4.20, 95% CI: 1.16-15.21) as independent risk factors for recurrence following colchicine therapy. CONCLUSION: Treatment with colchicine is highly effective in preventing recurrent pericarditis, while pretreatment with corticosteroids exacerbates and extends the course of recurrent pericarditis.     

Constrictive pericarditis as a complication of cardiac surgery: recognition of an entity

Among 5,207 adult patients who underwent cardiac surgery, postoperative constrictive pericarditis was recognized in 11 patients (0.2% incidence rate). Seven patients had coronary arterial bypass grafting and 4 had valve replacement; the pericardium was left open in all cases. The average interval between surgery and presentation of pericardial constriction was 82 days (range 14 to 186). M mode echocardiography revealed epicardial and pericardial thickening in 7 cases and variable degrees of posterior pericardial effusion in 5 cases. Cardiac catheterization demonstrated uniformity of diastolic pressures with a characteristic early diastolic dip and late plateau pattern. Two patients responded to medical therapy for chronic pericarditis. One patient had a limited parietal pericardiectomy followed by recurrent constrictive pericarditis that eventually stabilized with medical therapy. The other 8 patients required radical pericardiectomy. The pathophysiology of constriction after surgery is unclear. Its clinical expression involves a wide spectrum of presentation and therapeutic response. Constrictive pericarditis may be a complication of cardiac surgery in spite of an open pericardium and should be considered in postoperative patients who present with deteriorating cardiac function.     

Chronic Pericarditis and Pericardial Tamponade Associated with Ulcerative Colitis

Unlike other extraintestinal inflammatory manifestations of ulcerative colitis, cardiac involvement is infrequently reported and inadequately characterized, with only 9 previously reported cases of pericardial tamponade associated with inflammatory bowel disease. A 32 year old male with ulcerative colitis, treated with orally administered mesalamine for ten years, developed chronic pericarditis. Extensive clinical and laboratory evaluation failed to find any cause of the pericarditis other than the ulcerative colitis. Although the pericarditis remitted with indomethacin therapy, this medicine had to be discontinued because of a reactivation of ulcerative colitis attributed to this nosteroidal antiinflammatory drug (NSAID). The pericarditis then responded well to high-dose corticosteroid therapy, but the patient represented with chest pain, dyspnea, tachypnea, and engorged neck veins after tapering the corticosteroid therapy. Angiography revealed near equalization of end diastolic pressures in both ventricles, a finding consistent with pericardial tamponade. The patient underwent subtotal pericardiectomy. Thoracotomy revealed a thickened pericardial wall and a large pericardial effusion. The patient's symptoms resolved postpericardiectomy. This case extends the clinical spectrum of pericarditis associated with ulcerative colitis, by describing a case of pericarditis that was chronic, refractory to maintenance medical therapy, caused pericardial tamponade, and was successfully treated by pericardiectomy.     

Post-myocardial infarction pericarditis

Opinion statement Post-myocardial infarction pericarditis occurs in approximately 5% to 6% of patients who receive thrombolytic agents. It should be suspected in any patient with pleuropericardial pain. A pericardial friction rub may or may not be present. Differentiation of pericarditis from recurrent angina may be difficult, but a careful history and evaluation of serial electrocardiograms can help distinguish the two entities. Dressler’s syndrome, pericarditis that occurs at least 1 week following myocardial infarction, is now exceedingly rare. Most cases of pericarditis have a benign course; however, because pericarditis is associ-ated with larger infarcts, overall long-term mortality rate is increased. Rare complications include hemopericardium, cardiac tamponade, and constrictive pericarditis. Therapy is directed toward relief of pain, which usually responds well to nonsteroidal anti-inflammatory agents (eg, aspirin or ibuprofen).     

Gallium-67 citrate scanning for noninvasive detection of inflammation in pericardial diseases

The treatment of acute nonspecific pericarditis is controversial. No study is available that confirms the efficacy of the administration of corticosteroids or nonsteroid anti-inflammatory agents in this condition. There is no reliable invasive marker for pericardial inflammation, because echocardigraphy demonstrates only the presence of fluid. In four patients with pericarditis, gallium-67 citrate scanning was performed, and the isotope was localized to the cardiac silhouette in all. In one patient with effusion the gallium scan was positive and then reverted to negative with corticosteroid therapy. In another, the gallium scan remained positive despite resolution of the pericardial effusion with corticosteroid therapy. This patient eventually required pericardial stripping. Pericardial localization of gallium was useful in detecting the cause of fever in a patient after aortocoronary bypass grafting and in detecting pericardial involvement in a patient with multisystem viral disease. Pericardial localization of gallium-67 may be diagnostically useful and may provide a proper control for the study of the efficacy of corticosteroids versus nonsteroid anti-inflammatory agents in pericarditis.     

Colchicine as first-choice therapy for recurrent pericarditis: results of the CORE (COlchicine for REcurrent pericarditis) trial

BACKGROUND: Colchicine seems to be a good drug for treating recurrences of pericarditis after conventional treatment failure, but no clinical trial has tested the effects of colchicine as first-line drug for the treatment of the first recurrence of pericarditis. METHODS: A prospective, randomized, open-label design was used to investigate the safety and efficacy of colchicine therapy as adjunct to conventional therapy for the first episode of recurrent pericarditis. Eighty-four consecutive patients with a first episode of recurrent pericarditis were randomly assigned to receive conventional treatment with aspirin alone or conventional treatment plus colchicine (1.0-2.0 mg the first day and then 0.5-1.0 mg/d for 6 months). When aspirin was contraindicated, prednisone (1.0-1.5 mg/kg daily) was given for 1 month and then was gradually tapered. The primary end point was the recurrence rate. Intention-to-treat analyses were performed by treatment group. RESULTS: During 1682 patient-months (mean follow-up, 20 months), treatment with colchicine significantly decreased the recurrence rate (actuarial rates at 18 months were 24.0% vs 50.6%; P = .02; number needed to treat = 4.0; 95% confidence interval 2.5-7.1) and symptom persistence at 72 hours (10% vs 31%; P = .03). In multivariate analysis, previous corticosteroid use was an independent risk factor for further recurrences (odds ratio, 2.89; 95% confidence interval, 1.10-8.26; P = .04). No serious adverse effects were observed. CONCLUSION: Colchicine therapy led to a clinically important and statistically significant benefit over conventional treatment, decreasing the recurrence rate in patients with a first episode of recurrent pericarditis.     

Cardiac tamponade due to low-volume effusive constrictive pericarditis in a patient with uncontrolled type II autoimmune polyglandular syndrome

Abstract

Type II autoimmune polyglandular syndrome (APS), a relatively common endocrine disorder, includes primary adrenal insufficiency coupled with type 1 diabetes mellitus and/or autoimmune primary hypothyroidism. Autoimmune serositis, an associated disease, may present as symptomatic pericardial effusion. We present a case of a 54-year old male with APS who developed pericarditis leading to cardiac tamponade with a subacute loculated effusion. After urgent pericardiocentesis intrapericardial pressure dropped to 0, while central venous pressures remain elevated, consistent with acute effusive constrictive pericarditis. Contrast computerized tomography confirmed increased pericardial contrast enhancement. The patient recovered after prolonged inotropic support and glucocorticoid administration. He re-accumulated the effusion 16 days later, requiring repeat pericardiocentesis. Effusive–constrictive pericarditis, an uncommon pericardial syndrome, is characterized by simultaneous pericardial inflammation and tamponade. Prior cases of APS associated with cardiac tamponade despite low volumes of effusion have been reported, albeit without good demonstration of hemodynamic findings. We report a case of APS with recurrent pericardial effusion due to pericarditis and marked hypotension with comprehensive clinical and hemodynamic assessment. These patients may require aggressive support with pericardiocentesis, inotropes, and hormone replacement therapy. They should be followed closely for recurrent tamponade.