The spectrum of pericardial tamponade in systemic lupus erythematosus: Report of ten patients

Objective. To describe the range of clinical manifestations and the outcome of pericardial tamponade in patients with systemic lupus erythematosus (SLE). Methods. Patients with pericarditis and with pericardial tamponade were identified from our computerized database of 395 SLE patients. Medical records were reviewed to establish activity of SLE at the time of tamponade, as well as clinical and laboratory features, treatment, and outcome of the tamponade. Results. Pericarditis occurred in 75 patients (19%), with 11 episodes of tamponade in 10 of them (13%; 2.5% of entire series). Tamponade was the initial manifestation of SLE in 4 patients. Seven episodes occurred during active lupus, with nephritis present in 6. Signs of venous congestion, including ascites and facial or peripheral edema, were the most common manifestation of tamponade. Pericardial fluid was exudative, and pericardial tissue demonstrated a range of findings including fibrinous and fibrotic changes, acute and chronic inflammatory infiltrates, and vascular proliferation. Tamponade was fatal in 1 patient, and 2 patients each had recurrent effusions and pericardial thickening. Conclusion. Pericardial tamponade may occur at any point in the course of SLE, and should be considered in patients with unexplained signs of venous congestion. The differential diagnosis includes active SLE, uremia, and infection. Treatment with high-dose steroids and either pericardiocentesis or placement of a pericardial window is indicated, but recurrent effusions or pericardial thickening may develop.     

Pericardial tamponade complicating systemic lupus erythematosus

We describe a case of pericardial tamponade as an initial manifestation of late onset systemic lupus erythematosus (SLE). Tamponade is uncommon in SLE, but when it occurs, it is usually during the initial presentation of the patient. The occurrence of tamponade in patients with SLE with pericarditis is unpredictable, but usually associated with radiographic evidence of cardiomegaly. Treatment consists of pericardiocentesis, administration of high dose glucocorticoids and drainage via pericardial catheter. Recognition of this rare manifestation of SLE may be life saving.     

Diagnosis and management (by subxiphoid pericardiotomy) of large pericardial effusions causing cardiac tamponade.

To determine the clinical features, course and outcome of patients with cardiac tamponade, 57 consecutive patients with new, large pericardial effusions were prospectively studied. Twenty-five patients (44%) developed cardiac tamponade with venous hypertension and a pulsus paradoxus greater than 10 mm Hg. Electrocardiography, radiographic studies and echocardiography did not differentiate patients with and without tamponade. All 57 patients underwent thorough diagnostic evaluation followed by subxiphoid pericardial biopsy and drainage. A diagnosis was obtained in 53 patients (93%). Collagen vascular disease was significantly more frequent in the 25 patients with than in the 32 without cardiac tamponade (24 vs 3%; p less than 0.05). The frequency of malignant and uremic effusions was equal in both groups, whereas radiation-induced effusions seldom produced tamponade. At 1-year follow-up, 3 patients (12%) with tamponade had recurrent effusions, and 1 needed reoperation. This was not significantly different from the 32 patients without tamponade. Twelve-month mortality was also similar in both groups (36 vs 44%). This prospective series disclosed several unexpected findings: (1) Cardiac tamponade occurred in almost 50% of patients with new large pericardial effusions; (2) both malignancy and collagen vascular disease occurred with equal frequency as etiologies, whereas radiation-induced tamponade was unusual; (3) thorough clinical evaluation resulted in few idiopathic etiologies; and (4) subxiphoid pericardiotomy was effective for both diagnosis and therapy of tamponade.     

Large pericardial effusions due to systemic lupus erythematosus: a report of eight cases

The aim of this study was to describe the clinical, echocardiographic and laboratory characteristics of large pericardial effusions and cardiac tamponade secondary to systemic lupus erythematosus (SLE). An ongoing prospective study was conducted at Tygerberg Academic Hospital, South Africa between 1996 and 2002. All patients older than 13 years presenting with large pericardial effusions (> 10 mm) requiring pericardiocentesis were included. Eight cases (out of 258) were diagnosed with SLE. The mean (SD) age was 29.5 (10.7) years. Common clinical features were Raynaud's phenomenon, arthralgia and lupus nephritis class III/IV. Echocardiography showed Libman-Sacks endocarditis (LSE) in all the mitral valves. Two patients developed transient left ventricular dysfunction; both these patients had pancarditis. Typical serological findings included antinuclear antibodies, anti-double stranded DNA antibodies, low complement C4 levels and low C3 levels. CRP was elevated in six cases. Treatment consisted of oral steroids and complete drainage of the pericardial effusions. No repeat pericardial effusions or constrictive pericarditis developed amongst the survivors (3.1 years follow up). This study concludes that large pericardial effusions due to SLE are rare, and associated with nephritis, LSE and myocardial dysfunction. Treatment with steroids and complete drainage is associated with a good cardiac outcome.     

Case Report: Cardiac Tamponade Resembling an Acute Myocardial Infarction as the Initial Manifestation of Metastatic Pericardial Adenocarcinoma

Pericardial malignancies are uncommon, usually metastatic, linked to terminal oncology patients, and rarely diagnosed premortem. A very small number of patients will develop signs and symptoms of malignant pericardial effusion as initial clinical manifestation of neoplastic disease. Among these patients, a minority will progress to a life-threatening cardiac tamponade. It is exceedingly rare for a cardiac tamponade to be the unveiling clinical manifestation of an unknown malignancy, either primary or metastatic to pericardium. We present the case of a 50-year-old male who was admitted to the emergency department with an acute myocardial infarction diagnosis that turned out to be a cardiac tamponade of unknown etiology. Further studies revealed a metastatic pericardial adenocarcinoma with secondary cardiac tamponade. We encourage considering malignancies metastatic to pericardium as probable etiology for large pericardial effusions and cardiac tamponade of unknown etiology.     

Cardiac tamponade as an initial manifestation of systemic lupus erythematosus in early childhood.

Cardiac tamponade is rare as an initial manifestation of systemic lupus erythematosus (SLE), and even more so in paediatric patients. This paper reports an 8 year old girl with SLE with several unusual features: unusual age of presentation, unusual initial organ manifestation and recurrent cardiac tamponade as a complication.     

Systemic lupus erythematosus: an unusual cause of cardiac tamponade in a young man.

Although pericarditis and pericardial effusion are common cardiac complications of systemic lupus erythematosus (SLE), cardiac tamponade is a very rare initial manifestation of this disease. We describe a case of a young male patient in whom cardiac tamponade secondary to a loculated pericardial effusion was the presenting symptom of SLE.     

Pericardial effusion and mitral valve involvement in systemic lupus erythematosus. Echocardiographic study.

Echocardiography was used in 30 women and 2 men with systemic lupus erythematosus (SLE) in order to determine the incidence and severity of pericardial effusion and mitral valve involvement. 31 patients showed normal thickness of the mitral valve leaflets, only one patient showed irregular thickening of the leaflets suggesting the presence of vegetations. Mitral valve motions were normal in all patients. These results indicate that myocardial and valvular involvement in SLE is usually not severe enough to result in haemodynamic abnormalities. Pericardial effusion was found in 2 patients who were symptom free, whereas 4 of the patients with a past history suggestive of pericarditis showed no echocardiographic evidence of pericardial effusion. These suggest the transient nature of pericarditis in SLE, and the value of echocardiography as a diagnostic tool in detecting clinically inapparent lupus pericarditis.     

Tamponade precedes diagnosis of systemic lupus erythematosus

The most common cardiovascular manifestation of Systemic Lupus Erythematosus is pericardial disease. Tamponade in SLE is rarely described. The patient discussed in this case report presented with symptoms of heart failure. Physical exam, laboratory testing, echocardiography, and right heart catheterization revealed multiple morbid conditions including tamponade. The diagnoses satisfied four criteria for the classification of SLE. This case emphasizes the importance of a thorough physical exam in guiding diagnostic and therapeutic measures.     

Massive intractable pericardial effusion in a patient with systemic lupus erythematosus treated successfully with pericardial fenestration alone

Systemic lupus erythematosus (SLE) is often complicated by pericarditis with effusion, which generally responds well to glucocorticoid. We report herein a Japanese patient with SLE who showed a sign of cardiac tamponade and severe chest and back pain because of massive intractable pericardial effusion. Pulse glucocorticoid and pulse cyclophosphamide gained marginal effects. Pericardial effusion accumulated again soon after ultrasound-guided pericardiocentesis and drainage. Pericardial fenestration performed surgically as a last resort, for draining pericardial fluid into the pleural space, was very effective, and only a much smaller amount of fluid was observed in the space thereafter in comparison with the volume before the surgery. Pathological examination of the retrieved pericardium unfolded intense hyperplasia of small vessels and capillaries. Levels of IL-6 and TNF-alpha in pericardial effusion were extremely higher than those in serum. Pericardial effusion with extensive capillary hyperplasia in SLE would be resistant to medical treatment and require surgical fenestration.     

Acute myocarditis and ventricular fibrillation as initial presentation of pediatric systemic lupus erythematosus

Acute myocarditis and ventricular arrhythmia are rarely seen as the initial presentation of systemic lupus erythematosus (SLE) in children. We reported the case of a 12-year-old girl with congestive heart failure, acute myocarditis and pericardial effusion as a primary manifestation of SLE. Sudden cardiovascular collapse due to ventricular fibrillation (VF), ventricular tachycardia (VT) and cardiac tamponade occurred. After resuscitation and pericardiocentesis, frequent VF/VT refractory to anti-arrhythmic therapy was supported by venoarterial extracorporeal membrane oxygenation. Early diagnosis and a combination treatment for heart failure, arrhythmias and immunosuppression may result in a favorable outcome.     

Echocardiographic evaluation of pericardial effusion in myxedema. Incidence and biochemical and clinical correlations.

Pericardial effusion is a recognized consequences of myxedema. Its incidence is unknown, primarily because of past difficulties in establishing the diagnosis. We studied 33 hypothyroid patients by echocardiography. Ten of the 33 patients (30%) had positive echoes for pericardial effusion. Seven of these ten patients had enlarged hearts on chest X-ray. Five patients had cardiac enlargement but no echo evidence of pericardial effusion. Serum concentrations of thyroxine, 1.8+/-0.3 vs 1.5+/-0.1 mcg/dl and of thyroid stimulating hormone, 34+/-4 vs 38+/-5 muU/ml did not differ in the groups with and without pericardial effusion, respectively. However, the pericardial effusion group had significantly slower heart rates on ECG than those without pericardial effusion: 53+/-8 vs 68+/-2 beats/min, P less than 0.05. Low voltage was present in five of the ten patients with pericardial effusion and five of the 23 nonpericardial effusion patients. None of the patients with pericardial effusion developed tamponade. Seven patients with pericardial effusion were restudied after periods of thyroxine replacement therapy ranging from six months to two years. All were euthyroid and had negative echoes on follow-up, but two still showed cardiomegaly on chest X-ray (both had associated coronary artery disease). We conclude that pericardial effusion occurs frequently in patients with myxedema. Tamponade is uncommon and the effusions disappear with thyroid replacement therapy. Cardiomegaly on chest X-ray and low voltage on ECG are not reliable indicators of pericardial effusion.     

Pericardial tamponade after left posterolateral thoracotomy for left upper lobectomy for pulmonary aspergilloma

Pericardial tamponade limits diastolic filling of the heart; therefore, a high venous pressure is required to fill the ventricle. In presence of cardiac tamponade, therapeutic agents and manoeuvres that results in venodilation or vasodilation can severely compromise diastolic filling of the heart and might result in rapid cardiac decompensation. Equalization of central venous pressure and pulmonary artery diastolic pressure or equalization of pressures in all four chambers during diastole confirms cardiac tamponade. Transthoracic echocardiography can detect the site of tamponade and assist in pericardiocentesis. We describe acute pericardial tamponade in a young man who underwent left posterolateral thoracotomy for left upper lobectomy. Intraoperatively, mobilization of the left upper lobe was frequently associated with hypotension. Postoperatively, the patient suffered two more episodes of hypotension. The episodes of hypotension were attributed to surgical manipulation and epidural blockade. Hemodynamics normalized after discontinuing epidural infusion, volume resuscitation and lobectomy. On third postoperative day, the patient developed cardiovascular collapse; arterial blood pressure and central venous pressure were 70/50 and 12 mmHg. Investigations showed haziness of left lung, and severe respiratory acidosis. On opening of the left thoracotomy wound, pericardial tamponade was diagnosed. A pericardial window was created and tamponade was released with that the hemodynamics normalized. Episodes of unexplained hypotension after left upper lobectomy suggest a cardiac etiology and acute pericardial tamponade is a possibility which should be released immediately otherwise it can result in fatal outcome.     

Systemic lupus erythematosus presenting as cardiac tamponade-a case report

Although pericarditis and pericardial effusion (PE) are some of the common manifestations of systemic lupus erythematosus (SLE), the occurrence of cardiac tamponade is quite rare. We present herewith a young girl with cardiac tamponade presenting as initial manifestation of SLE.     

Pericardial effusion in patients with cancer: outcome with contemporary management strategies.

OBJECTIVE--To investigate the clinical presentation and current management strategies of pericardial effusion in patients with malignancy. DESIGN--Retrospective single centre, consecutive observational study. SETTING--University hospital. PATIENTS--93 consecutive patients with a past or present diagnosis of cancer and a pericardial effusion, including 50 with a pericardial effusion > 1 cm. RESULTS--Of the 50 patients with pericardial effusions > 1 cm, most had stage 4 cancer (64%), were symptomatic at the time of presentation (74%), and had right atrial collapse (74%). Twenty patients were treated conservatively (without pericardiocentesis) and were less symptomatic (55% v 87%, P = 0.012), had smaller pericardial effusions (1.5 (0.4) v 1.8 (0.5), P = 0.02), and less frequent clinical (10% v 40%, P = 0.02) and echocardiographic evidence of tamponade (40% v 97%, P < 0.001) than the 30 patients treated invasively with initial pericardiocentesis (n = 29) or pericardial window placement (n = 1). Pericardial tamponade requiring repeat pericardiocentesis occurred in 18 (62%) of 29 patients after a median of 7 days. In contrast, only four (20%) of 20 patients in the conservative group progressed to frank clinical tamponade and required pericardiocentesis (P = 0.005 v invasive group). The overall median survival was 2 months with a survival rate at 48 months of 26%. Survival, duration of hospital stay, and hospital charges were similar with both strategies. By multivariable analysis, the absence of symptoms was the only independent predictor of long-term survival (relative hazards ratio = 3.2, P = 0.05). Survival was similar in the 43 patients with cancer and pericardial effusions of < or = 1 cm. CONCLUSION--Asymptomatic patients with cancer and pericardial effusion can be managed conservatively with close follow up. In patients with symptoms or clinical cardiac tamponade, pericardiocentesis provides relief of symptoms but does not improve survival and has a high recurrence rate. Surgical pericardial windows or possibly percutaneous balloon pericardiotomy should be used for recurrences and should be considered for initial treatment.     

Subxiphoid pericardiotomy in the diagnosis and management of large pericardial effusions associated with malignancy.

To determine the safety, diagnostic value, and clinical outcome of patients with malignancy undergoing subxiphoid pericardiotomy for large pericardial effusions, we prospectively studied 25 consecutive patients with malignancy and new, large pericardial effusions diagnosed by echocardiography. Twenty-two of the 25 operations were done under local anesthesia, and no patient died at surgery. Pericardial fluid cytology revealed malignant cells in 11 patients (44 percent), while tumor was seen in only five (45 percent) of these 11 patients on pathologic examination. The remaining 14 patients showed no evidence of pericardial invasion with tumor. Evidence of intrathoracic disease by CT or MRI scanning, tamponade, a sanguineous pericardial fluid character, and an elevated serum and pericardial fluid lactate dehydrogenase level all were suggestive of malignant invasion of the pericardium. All 25 patients were followed at least 12 months postoperatively. Effusions recurred in three patients (12 percent), and one patient required reoperation. Overall mortality was 72 percent with a 91 percent (10 of 11) mortality for those with malignant effusions and a 57 percent (8 of 14) mortality for those with nonmalignant effusions. Diagnostically, subxiphoid pericardiotomy has little advantage over examination of pericardial fluid alone in this group of patients. Therapeutically, however, it is a low morbidity procedure which is safe and effective in treating patients with malignancy and large pericardial effusions.     

Comparison of Doppler and two-dimensional echocardiography for assessment of pericardial effusion.

Respiratory changes in left ventricular inflow velocities by Doppler echocardiography have been used to assess cardiac tamponade; however, Doppler echocardiography has not been compared to right atrial or right ventricular collapse. Pulsed Doppler echocardiography of left ventricular inflow velocities was performed with respiratory monitoring in 28 patients with small to large pericardial effusions. Ten of the 17 patients (59%) with large effusions had equalization of right-sided diastolic pressures before pericardial drainage. The measurements performed included percent change in left ventricular inflow peak early velocity, isovolumic relaxation time, change in inferior vena cava diameter from apnea to inspiration, and the presence of right atrial and right ventricular collapse. Percent change in early left ventricular inflow velocities significantly correlated with pericardial effusion size (p = 0.001) and right ventricular collapse (p = 0.007), and showed a trend with right atrial collapse (p = 0.10). Pericardial effusions with a left ventricular inflow velocity change > 22% were found to have right-sided equalization at a 95% confidence interval. Our data indicate that the respiratory changes in Doppler echocardiographic parameters are useful in the assessment of pericardial effusion and tamponade. This study concurs with the hypothesis that there is a continuum of hemodynamic compromise in pericardial effusion that is easily detected by Doppler echocardiography.     

How do the clinical findings in patients with pericardial effusions influence the success of aspiration?

OBJECTIVE--To identify features associated with success or failure of aspiration of pericardial effusion. METHOD--A retrospective analysis of 36 drainage procedures in 30 patients with pericardial effusion was performed using patient records and echocardiograms. RESULTS--Unsuccessful aspiration was associated with pericardial loculation but not with the seniority of the operator or the size and position of the effusion. Pericardiocentesis relieved symptoms of breathlessness in 21 of 26 patients who had a pericardial effusion suspected of causing dyspnoea. These 21 patients had few clinical or echocardiographic signs of classic tamponade. CONCLUSION--The paucity of abnormal physical or echocardiographic signs of tamponade in breathless patients with pericardial effusion does not exclude symptomatic benefit being derived from pericardiocentesis. Pericardial aspiration is safe in appropriate hands, although aspiration of loculated effusions may not be as successful as aspiration of non-loculated effusions.     

Pericardial effusion after cardiac surgery in children and effects of aspirin for prevention

Seventy-four children aged 0.3 to 21.4 years (median 4.0) were followed echocardiographically on days 4, 7, 14 and 28 (+/- 2 days) after cardiac surgery to evaluate the incidence of postoperative pericardial effusion, to identify the patients at greatest risk of developing an effusion and to evaluate the use of aspirin as prophylaxis against pericardial effusion. Pericardial effusion was graded relative to the size of the aortic root from grade 0 (no effusion) to grade 5 (larger than the aortic root dimension). Patients were randomly divided into 2 groups: group 1 (32 patients) received aspirin 60 mg/kg/day for 7 days starting on the third postoperative day; group 2 (42 patients) received no aspirin. Forty-eight patients (65%) developed an effusion during the study period, 3 required pericardiocentesis and 1 died of tamponade. All patients with tamponade had a grade 4 effusion. Age or type of operation did not alter the cumulative incidence of significant effusion. No patient with a grade 0 effusion on the first echocardiogram developed a grade 4 or 5 effusion. Results in groups 1 and 2 were similar. Pericardial effusions are common in the first month after cardiac surgery. Patients with no effusion in the immediate postoperative period appear to be at lesser risk of developing a grade 4 effusion in the first month after operation. Finally, aspirin prophylaxis against postoperative pericardial effusions did not significantly alter the outcome in this small series of patients.