Antiphospholipid antibodies as a cause of pulmonary capillaritis and diffuse alveolar hemorrhage: a case series and literature review

OBJECTIVES: To discuss the clinical manifestations and possible pathogenic mechanisms of the unusual syndrome of diffuse alveolar hemorrhage (DAH) and pulmonary capillaritis without thrombosis in the setting of the primary antiphospholipid antibody syndrome (PAPS). METHODS: Four men with DAH and capillaritis in the setting of PAPS are identified. Their clinical presentations, laboratory, radiographic, and pathologic findings are reviewed as is their clinical course and response to therapy. In addition, the literature regarding DAH and pulmonary capillaritis in the setting of PAPS is reviewed. RESULTS: The patients presented with dyspnea, hemoptysis, fever, hypoxia, and diffuse alveolar infiltrates; none had evidence of acute thromboembolic disease. All secondary causes of DAH were ruled out. All patients had positive testing for the lupus anticoagulant and high-titer anticardiolipin antibodies, including antibodies against the beta-2-glycoprotein I antigen. Three cases had lung biopsies that revealed pulmonary capillaritis and DAH with no evidence of thrombosis. All patients improved with high-dose corticosteroids. Recurrent disease in the setting of aggressive immunosuppression responded to intravenous immunoglobulin. Antiphospholipid antibody-mediated endothelial cell activation in the absence of thrombosis may induce capillaritis as seen in these cases. CONCLUSIONS: The syndrome of DAH and pulmonary capillaritis is further defined. Evidence supports a causative relationship between PAPS, pulmonary capillaritis, and DAH in the absence of thromboembolic disease. Further elucidation of a possible nonthrombotic mechanism of antiphospholipid antibody-mediated pathology is needed to guide future therapies for this unusual manifestation of PAPS.     

Transesophageal Echocardiographic Correlates of Clinical Risk of Thromboembolism in Nonvalvular Atrial Fibrillation

Objectives. This study explored the mechanisms linking clinical and precordial echocardiographic predictors to thromboembolism in atrial fibrillation (AF) by assessing transesophageal echocardiographic (TEE) correlations.

Background. Clinical predictors of thromboembolism in patients with nonvalvular AF have been identified, but their mechanistic links remain unclear. TEE provides imaging of the left atrium, its appendage and the proximal thoracic aorta, potentially clarifying stroke mechanisms in patients with AF.

Methods. Cross-sectional analysis of TEE features correlated with low, moderate and high thromboembolic risk during aspirin therapy among 786 participants undergoing TEE on entry into the Stroke Prevention in Atrial Fibrillation III trial.

Results. TEE features independently associated with increased thromboembolic risk were appendage thrombi (relative risk [RR] 2.5, p = 0.04), dense spontaneous echo contrast (RR 3.7, p < 0.001), left atrial appendage peak flow velocities ≤20 cm/s (RR 1.7, p = 0.008) and complex aortic plaque (RR 2.1, p < 0.001). Patients with AF with a history of hypertension (conferring moderate risk) more frequently had atrial appendage thrombi (RR 2.6, p < 0.001) and reduced flow velocity (RR 1.8, p = 0.003) than low risk patients. Among low risk patients, those with intermittent AF had similar TEE features to those with constant AF.

Conclusions. TEE findings indicative of atrial stasis or thrombosis and of aortic atheroma were independently associated with high thromboembolic risk in patients with AF. The increased stroke risk associated with a history of hypertension in AF appears to be mediated primarily through left atrial stasis and thrombi. The presence of complex aortic plaque distinguished patients with AF at high risk from those at moderate risk of thromboembolism.     

Embolic events in patients with atrial fibrillation and effective anticoagulation: value of transesophageal echocardiography to guide direct-current cardioversion. Final results of the Ludwigshafen Observational Cardioversion Study

OBJECTIVES: The primary objective was to evaluate the usefulness of transesophageal echocardiography (TEE)-guided cardioversion to prevent thromboembolic complications in patients with atrial fibrillation (AF) and effective anticoagulation (International Normalized Ratio of 2 or 3) at least three weeks before cardioversion. BACKGROUND: Transesophageal echocardiography has been proposed as a method of screening patients for left atrial thrombi before direct-current cardioversion of AF. The usefulness of TEE as a screening tool has always been evaluated in patients without long-term anticoagulation before cardioversion. METHODS: This prospective, single-center, observational study, performed on an intention-to-cardiovert basis, comprised 1,076 consecutive, unselected patients with AF. The initial two years were designed to be the control phase, during which the conventional approach was used. After that, cardioversion guided by TEE was performed in consecutive patients. RESULTS: The prevalence of left atrial thrombi was 7.7% in patients with persistent AF and effective anticoagulation. During the first four weeks after electrical cardioversion, six thromboembolic complications were observed in patients in whom the TEE-guided approach was employed (6 [0.8%] of 719 patients), compared with three thromboembolic complications in patients in whom the conventional approach was used (3 [0.8%] of 357 patients). None of the patients in whom electrical cardioversion was not performed experienced an embolic event. CONCLUSIONS: There were no differences in the rate of embolic events between the two treatment groups. In patients with AF and effective anticoagulation, TEE-guided electrical cardioversion does not reduce the embolic risk. However, TEE revealed left atrial thrombi in 7.7% of patients with AF and effective anticoagulation, before direct-current cardioversion.     

Cardiological diagnosis in arterial embolism]

Potential cardiac sources of arterial embolism are in particular thrombi within the left atrium or ventricle, or attached to a prosthetic valve, intracardiac tumors, and vegetations due to endocarditis. Patent foramen ovale and atrial septal defect may lead to paradoxical embolism, and spontaneous echo contrast within the heart has to be considered as a parameter of increased thromboembolic risk. In rare cases, atrial septal aneurysm, mitral valve prolapse or annulus calcification and calcified aortic stenosis has to be taken into consideration. Current method of choice for diagnosis of these abnormalities is echocardiography. When the transthoracic approach fails, transesophageal echocardiography (TEE) leads to a definite diagnosis in most cases. Precordial echocardiography allows the detection of left ventricular thrombi with a sensitivity ranging between 72 and 95%, and monoplane TEE does usually not increase these numbers. In contrast, thrombi within the left atrium and particularly in the left atrial appendage can be detected with a significantly higher detection rate when TEE is used. The same is true for spontaneous echo contrast in the left atrium, a phenomenon which is almost exclusively diagnosed by TEE, as well as for endocarditis associated vegetations that can be identified by TEE with a sensitivity higher than 90%. Patient foramen ovale is usually diagnosed by precordial contrast echocardiography combined with a Valsalva maneuver; color Doppler or contrast TEE allows to increase the detection rate. In the diagnosis of prosthetic valve attached thrombi and vegetations, TEE is clearly superior compared to the precordial examination, at least concerning prosthetic devices in mitral position. If echocardiography fails to identify a potential cardiac source of embolism, other techniques don't add significant information in most cases. Detection of a potential source of embolism, however, does not necessarily prove that the particular finding represents the true etiology of an embolic event; results of all clinical and technical examinations have to be evaluated in a critical synopsis. In addition, proper therapeutic consequences in quite a number of abnormalities considered as potential cardiac sources of embolism are not yet defined.     

Pre- and post-cardioversion transesophageal echocardiography for brief anticoagulation therapy with enoxaparin in atrial fibrillation patients: a prospective study with a 1-year follow-up

BACKGROUND: In patients with atrial fibrillation (AF) eligible for electrical cardioversion (C), the guided approach with transesophageal echocardiography (TEE) allows to avoid the 3 weeks of recommended precardioversion anticoagulation therapy. However, after sinus rhythm restoration, at least other 4 weeks of oral anticoagulation therapy are indicated, due to the postcardioversion thromboembolic risk related to left atrial (LA) and left atrial appendage (LAA) stunning. The aim of this study was to prospectively assess the effectiveness and the safety of anticoagulation therapy discontinuation 7 days after C using low-molecular-weight heparins (LMWH) in a selected group of patients who underwent a pre-C and 7 days post-C TEE evaluation. METHODS: One hundred one patients (74 patients with nonvalvular AF and 27 patients with atrial flutter lasting >48 h and history of AF) were enrolled into the study. Two patients refused the TEE, therefore, in 99/101, we performed a first TEE and, within 24 h, a C if there were no LAA thrombi, complex aortic plaques or severe spontaneous echocontrast. After C and 7 days of home-administered enoxaparin, a second TEE was carried out. In the absence of any new thrombi, severe spontaneous echocontrast and/or low emptying velocity of LAA, the therapy with enoxaparin was stopped; otherwise, anticoagulation therapy with enoxaparin was overlapped with oral anticoagulation and continued for at least 3 weeks. All patients were clinically followed at 1, 6 and 12 months after C. RESULTS: Sinus rhythm was restored in 68/99 patients after successful C. The second TEE was carried out in 53 patients. At 1 month follow-up, no thromboembolic events were recorded either in patients at risk who had continued the oral anticoagulant therapy for at least 3 weeks or in those who suspended LMWH after 7 days post-C TEE. Between the 2nd and 12th month, three ischemic strokes occurred, all in the group of patients who had anticoagulation therapy for at least 3 weeks and had shown LAA velocity <25 cm/s at first or second TEE. No thromboembolic events were recorded in patients with normal LAA velocity; conversely, among the patients who had shown low LAA velocity at either TEE, three suffered from ischemic stroke. In two of these three patients, low LAA velocity was detected only at post-C TEE. CONCLUSIONS: A brief anticoagulation therapy using LMWH appears to be safe and feasible. The 7 days post-C TEE can well-define patients without LAA stunning at low thromboembolic risk, who may take advantage of an early interruption of enoxaparin as an alternative to long oral anticoagulation. The LAA stunning, even in the absence of other thromboembolic risk factors, could select a group of patients at high risk who should continue oral anticoagulation indefinitely or until signs of LAA dysfunction disappear.     

TEE-geführte Kardioversion bei Patienten mit Vorhofflimmern ohne vorherige Antikoagulation

A total of 332 patients (mean age 65+/-10 years, 86 female) with nonvalvular atrial fibrillation (AF) of more than 48 hours duration and lack of a sufficient anticoagulation were included. After exclusion of thrombotic material in the left atrium using transesophageal echocardiography (TEE) cardioversion (CV) was performed within 24 hours. At the same time oral anticoagulation (AC) (overlapping with PTT-affecting heparinisation) was started. If thrombi were found by TEE, the examination was repeated after at least four weeks of anticoagulation. If thrombi were absent at this time, CV was performed. Periprocedural embolism was defined as primary endpoint, whereas the detection of atrial thrombi before CV was defined as secondary endpoint. In 33 of the 332 Patients (9.9%) the TEE showed a thrombus in the left atrium respectively the left atrial appendage (n=22) or thrombi could not be excluded (n=11). 383 TEEs were performed without complications in an overall of 332 patients.A total of 305 CV were performed (electrical n=300, pharmacological n=5) and during periprocedural monitoring and in the time of four weeks after CV no thromboembolic complications were observed.TEE-guided CV in patients with AF persisting for more than 48 hours and without previous AC can be considered as a method that is both safe and effective.     

Transesophageal Echocardiography for the Prevention of Embolic Complications After Catheter Ablation for Atrial Fibrillation

Background: Thromboembolic complications during left-sided ablations range between 1.5 and 5.4%. Preprocedural TEE has been used to exclude the presence of left atrial thrombi in order to minimize risk. The use of TEE is empiric and it has not been evaluated in contemporary practice.
Methods and Results: A multicenter national survey describing the practice at 11 Canadian teaching hospitals. A total of 2,225 patients underwent elective catheter ablation for symptomatic AF. Transesophageal echocardiography (TEE) was used either routinely or selectively as a preablative strategy in patients. There were 996 patients in a routine preprocedure TEE strategy and 1,190 in a selected TEE strategy; 1 center (n = 39 patients) did not perform TEE. Twelve of 996 (1.2%) in the routine unselected cohort had thrombi identified. TEEs were performed in 200 of 1,190 in the selected cohort; 4 (2.0%) left atrial thrombi were observed; there was no significant difference in the prevalence of thrombi (P = 0.34). A total of 11 embolic events occurred inclusive of all groups. There was no difference in event rates between the 2 strategies (0.6% and 0.4%, P = 0.54). Events were unrelated to AF duration (persistent vs paroxysmal, r = 0.03, N = 2,225, P = 0.9).
Conclusion: The selection criteria employed to perform TEEs did not increase the chance of identifying LA thrombi in a patient cohort with primarily nondilated left atria and paroxysmal AF. The overall thromboembolic event rate was low (0.49%) and was not significantly different between the 2 TEE strategies.     

Detection of left atrial auricular thrombus in patients with atrial fibrillation and risk factors of thromboembolic complications: the role of transesophageal echocardiography and multispiral computer tomography

Transesophageal echocardiography (TEE) is routinely used for visualization of left auricular thrombi in patients with atrial fibrillation (AF). Multispiral computed tomography (MSCT) with the use of contrast preparations is a novel method of visualization of intracardiac structures. Forty three patients (27 men, 16 women aged 46 - 81 years) with duration of atrial fibrillation > 48 hours and scheduled for sinus rhytm restoration were included in this study. TEE and MSCT were carried out in all patients during first 3 days of hospitalization. If according to both methods there were no thrombi in the left auricle cardioversion was performed. If data of one of the methods were suspicious of left auricular thrombus cardioversion was not performed. In these patients both investigations were repeated after 8 weeks of therapy with warfarin. If initially detected mass decreased or disappeared at the background of indirect anticoagulants, it was considered to be a thrombus. In 1/3 of patients repeat examination allowed to reject initial diagnosis of left auricular thrombosis. Simultaneous application of TEE and MSCT detected more thrombi than the use of either of these methods. Rate of detection of thrombi in left atrial auricle with the use of both methods was 32%.     

Management of Nonobstructive Thrombosis of Prosthetic Mitral Valve in Asymptomatic Patients in the Early Postoperative Period: A Study in 20 Patients

The purpose of the present study was to determine the optimal management of nonobstructive thrombi in the early postoperative period after mitral valve replacement. Twenty cases of early thrombi were revealed by systematic transesophageal echocardiography (TEE) performed 9 days after surgical implantation of 229 St. Jude prostheses. Patients were prospectively randomized into two groups: the first group received oral anticoagulants (fluindione), and the second group was treated with a combination of oral anticoagulants and aspirin. The impact of the different therapies on prosthetic thrombi was assessed by systematic TEE at 5 months. All thrombi were nonobstructive and disappeared without fibrinolytic agents or surgery. Seven large (≥ 5 mm) thrombi disappeared: in two patients after reintroduction of intravenous heparin and in five patients with an optimized oral treatment (anticoagulants alone in one patient, anticoagulants associated with aspirin in four patients). In the 13 small (< 5 mm) thrombi, 11 disappeared with an optimized oral treatment: anticoagulants alone in nine patients and anticoagulants associated with aspirin in two patients. The other two small thrombi treated with heparin alone initially persisted and finally disappeared with heparin associated with aspirin. In the group with large thrombi, we recorded a 43% rate of thromboembolic events, with more deaths and severe cerebral embolic events than in the patients with small thrombi. Embolic events were associated with mobile thrombi. An optimized oral anticoagulation therapy with correction of the parameters of Virchow et al. is effective in the treatment of small prosthetic thrombi. For large thrombi, the high rate of thromboembolic events would appear to require a more aggressive approach such as an association of heparin with aspirin and TEE follow-up.     

Asymptomatic intracardiac thrombi and primary antiphospholipid syndrome

Intracardiac thrombi have rarely been reported in patients with the antiphospholipid syndrome. We describe a new case revealed by systematic echocardiography in an asymptomatic woman who consulted for mild thrombocytopenia. Our case is characterized by a past history of migraine, fetal loss and psychiatric disturbances and by a high level of antiphospholipid antibodies (anticardiolipin, anti-beta(2)-glycoprotein I and lupus anticoagulant). Echography, in the absence of any cardiovascular symptom, showed bulky right intra-atrial thrombi requiring surgical excision. Histopathological analysis revealed the fibrino-cruoric nature of the lesion without myxoma. This case shows that, when faced with a mild thrombocytopenia associated with antiphospholipid antibodies, echocardiography may reveal a life-threatening and completely asymptomatic intracardiac thrombosis.     

Rapid resolution of left ventricular thrombus in antiphospholipid syndrome

A 43-year-old male with recurrent cerebral infarctions and high titers of antiphospholipid antibodies was found to have a thrombus in his left ventricular outflow tract by transthoracic echocardiography. This thrombus disappeared after less than 24 hours of intravenous heparin therapy due to resolution or asymptomatic embolism. Left ventricular thrombi in patients with antiphospholipid syndrome and rapid disappearance of left ventricular thrombi with heparin therapy are infrequently reported occurrences.     

Usefulness of transesophageal echocardiography for the detection of left atrial thrombi in patients with rheumatic heart disease

Transesophageal (TEE) and transthoracic (TTE) echocardiograms were performed in 110 patients with rheumatic heart disease to evaluate the usefulness of these methods for the detection of left atrial thrombi. TEE was better than TTE for detecting left atrial thrombi (21 vs 9). The thrombi not detected by TTE were in the left atrial appendage in ten and over the left atrial posterior wall in two. Patients with left atrial thrombi had significantly smaller mitral valve area (P less than 0.01) and greater left atrial dimension (P less than 0.05) than those without. All patients with left atrial thrombi had atrial fibrillation. Thirty-one patients underwent surgical intervention and 13 were found to have left atrial thrombi. TEE detected left atrial thrombi in all 13 patients with a sensitivity of 100%, specificity of 100%, and accuracy of 100%, while TTE detected left atrial thrombi in only nine of these 13 patients with a sensitivity of 69.2%, specificity of 100%, and accuracy of 87.1%. Thus, TEE is superior to TTE for the detection of left atrial thrombi, especially for those thrombi located in the left atrial appendage and along the left atrial posterior wall.     

Should Transesophageal Echocardiography Become a Routine Test in Patients with Suspected Pulmonary Thromboembolism?

Pulmonary thromboembolism presents in two clinical subsets: acute pulmonary embolism (PE) with or without right heart thrombi or paradoxical embolism and chronic thromboembolic pulmonary hypertension (CTEPH). Both PE and CTEPH have been underdiagnosed and carry high mortality rates. Acute massive PE is a hemodynamic entity leading to right ventricular overload readily identified with the use of transthoracic echocardiography. Transesophageal echocardiography (TEE) is a noninvasive bedside technique that has high diagnostic accuracy for the detection of central pulmonary thromboembolism. Due to the high prevalence of central pulmonary thromboembolism in acute PE, TEE is a useful method to provide the necessary proof for the institution of thrombolytic therapy. In the subset of patients with acute PE combined with right heart thrombi or paradoxical embolism, TEE is the technique of choice to guide surgery. CTEPH presents as primary pulmonary hypertension, but it has become a surgically curable disease. TEE is a fast, fairly sensitive, and highly specific diagnostic bedside modality to select surgical candidates with CTEPH. TEE should become a routine test in patients with suspected massive acute PE, suspected right heart thrombi, or paradoxical embolism associated with acute pulmonary embolism and in patients with primary pulmonary hypertension to select those having CTEPH who are suitable for surgery.     

Antiphospholipid syndrome plus rheumatic fever: a higher risk factor for stroke?

To compare clinical and laboratory findings between patients with primary antiphospholipid syndrome (PAPS) versus secondary APS due to rheumatic fever (APS-RF) (according to Jones criteria). Seventy-three APS patients (Sapporo criteria) were enrolled, and demographic, clinical, and laboratory data were collected. Exclusion criteria were heart congenital abnormalities and previous infectious endocarditis. Patients were divided into two groups: PAPS (n?=?68) and APS-RF (n?=?5). The mean current age, disease duration, frequencies of female gender, and Caucasian race were similar in APS-RF and PAPS patients (P?>?0.05). Remarkably, the frequency of stroke was significantly higher in APS-RF compared to PAPS patients (80% vs. 25%, P?=?0.02). Of note, echocardiogram of these patients did not show intracardiac thrombus. No significant differences were found in peripheral thromboembolic events (P?=?1.0), pulmonary thromboembolism (P?=?1.0), miscarriage (P?=?0.16), thrombocytopenia (P?=?0.36), arterial events (P?=?0.58), and thrombosis of small vessels (P?=?1.0). There were no differences in the frequencies of comorbidities such as diabetes mellitus, hypertension, smoking, and hyperlipidemia in both groups (P?>?0.05). The frequencies of lupus anticoagulant, IgG, and IgM anticardiolipin were similar in two groups. APS patients associated with rheumatic fever without infective endocarditis may imply a high stroke risk as compared with PAPS, and future studies are needed to confirm this finding.     

The role of transesophageal echocardiography in the management of patients with acute and chronic pulmonary thromboembolism

Acute and chronic pulmonary thromboembolism carry high mortality. The role of transesophageal echocardiography (TEE), however, has not been well delineated in patients with suspected pulmonary thromboembolism. The aim of the present study was to demonstrate the value of Tee in patients with various clinical manifestations of pulmonary thromboembolic disease. Twelve patients--ten males and two females, age 47-85 years--are presented in whom central pulmonary thromboembolism was found by TEE. Six patients were referred for breathlessness and had moderate to severe pulmonary hypertension (PH) with (3) or without (1) right atrial thrombus or had right heart dilatation (1) or right ventricular myxoma (1) on transthoracic echocardiography (TTE). Thrombolysis (2), surgery (2), and heparin (2) treatment was performed without angiography. All but one patient recovered. Six patients had severe PH by TTE, one of them had a right atrial thrombus. Angiography was done in five patients in whom surgery was considered. Pulmonary thromboendarterectomy was successfully performed in two patients, it was contraindicated in two patients for advanced age or severe left ventricular dysfunction, both patients died during follow-up, and two patients were waiting for surgery. In conclusion: TEE has a definite role in the management of patients with acute pulmonary thromboembolism or in pulmonary embolism associated with right-sided intracardiac masses and in the selection of patients with PH for pulmonary thromboendarterectomy.     

Transthoracic echocardiography for precardioversion screening during atrial flutter/fibrillation in young patients

BACKGROUND: Transthoracic echocardiography (TTE) is reliable for detection of thrombi in the left ventricle and right atrium, but not in the left atrial appendage. Therefore, transesophageal echocardiography (TEE) is routinely performed in adults prior to electric cardioversion for atrial flutter/fibrillation (AFF). Whether young survivors of congenital heart disease repair with AFF need routine TEE prior to electric cardioversion is unknown. HYPOTHESIS: Electric cardioversion for AFF is safe in survivors of congenital heart disease repair/palliation if an intracardiac thrombus is not suspected on TTE imaging. METHODS: This study reports the outcome of patients in a pediatric tertiary care cardiac unit where electric cardioversion was performed if no intracardiac thrombus was suspected on TTE. We performed a retrospective chart review of all patients treated with electric cardioversion for AFF at Children's Hospital of Michigan during 1997-2002. RESULTS: Of 35 patients who presented with 110 episodes of AFF requiring electric cardioversion during the study duration, 32 (age 3 months-49 years, median age 20.5 years, 104 AFF episodes) had previously undergone palliative surgery or repair of their congenital heart disease. Of these 32 patients, 18 were survivors of a Fontan palliation (for a single-ventricle variant) and the remaining 14 were survivors of other defects and repairs (septal defects, valve replacements, and tetralogy of Fallot). During 81% of the episodes, patients were receiving aspirin, warfarin, or heparin for anticoagulation at presentation. Transthoracic echocardiography was performed in 74 AFF episodes; of these, 10 TTE studies were suspicious for atrial thrombi. Transesophageal echocardiography confirmed the presence of a thrombus in 3 of these 10 patients. These patients received warfarin for 2 weeks and then underwent electric cardioversion. No thromboembolic events occurred immediately after or on follow-up in any patient. CONCLUSIONS: These findings suggest that TTE may be an effective imaging tool for precardioversion screening in young patients with AFF.     

Transesophageal Echocardiography in Candidates for Percutaneous Balloon Mitral Valvuloplasty

Prior to percutaneous balloon mitral valvuloplasty (PBMV), mitral valve morphology and the presence of left atrial thrombi are usually evaluated by transthoracic two-dimensional and Doppler echocardiography (TTE). This study analyzes the impact of transesophageal echocardiography (TEE) in addition to TTE on the selection of candidates considered for PBMV for mitral stenosis. Seventy-five patients with severe mitral stenosis who were considered as appropriate candidates for PBMV based on TTE findings were studied. In 19 (25%) patients, TEE revealed findings that were essential for PBMV but were missed by TTE: left atrial thrombi (n = 14; including 13 in left atrial appendage), right atrial thrombus (n = 1), incomplete cor triatriatum (n = 1) and mitral valve vegetation (n = 1). In two other patients, a left atrial thrombus had been suspected by TTE but could be excluded by TEE. TEE and TTE revealed similar scores of thickening, calcification, and mobility of the mitral valve. Compared to TTE, thickening of the subvalvular apparatus was graded lower using horizontal plane TEE due to shadowing by the mitral valve (echo score 1.8 ± 0.8 vs 1.4 ± 0.7; P < 0.05) whereas results from longitudinal plane TEE were similar to TTE findings. The data show that due to the high prevalence of left atrial thrombi, TEE should be performed in addition to TTE in all patients prior to PBMV.     

Prevalence of left atrial chamber and appendage thrombi in patients with atrial flutter and its clinical significance

OBJECTIVES: The study was done to assess the prevalence of left atrial (LA) chamber and appendage thrombi in patients with atrial flutter (AFl) scheduled for electrophysiologic study (EPS), to evaluate the prevalence of thromboembolic complications after transesophageal echocardiographic (TEE)-guided restoration of sinus rhythm and to evaluate clinical risk factors for a thrombogenic milieu. BACKGROUND: Recent studies showed controversial results on the prevalence of atrial thrombi and the risk of thromboembolism after restoring sinus rhythm in patients with AFl. METHODS: Between 1995 and 1999, patients with AFl who were scheduled for EPS were included in the study. After transesophageal assessment of the left atrial appendage and exclusion of thrombi, an effective anticoagulation was initiated and patients underwent EPS within 24 h. RESULTS: We performed 202 EPSs (radiofrequency catheter ablation, n = 122; overdrive stimulation, n = 64; electrical cardioversion, n = 16) in 139 consecutive patients with AFl. Fifteen patients with a thrombogenic milieu were identified. All of them had paroxysmal atrial fibrillation (AF). Transesophageal echocardiography revealed LA thrombi in two cases (1%). After EPS no thromboembolic complications were observed. Diabetes mellitus, arterial hypertension and a decreased left ventricular ejection fraction were found to be independent risk factors associated with a thrombogenic milieu. CONCLUSIONS: The findings of a low prevalence of LA appendage thrombi (1%) in patients with AFl and a close correlation between a history of previous embolism and paroxysmal AF support the current guidelines that patients with pure AFl do not require anticoagulation therapy, whereas patients with AFl and paroxysmal AF should receive anticoagulation therapy. In addition, the presence of clinical risk factors should alert the physician to an increased likelihood for a thrombogenic milieu.     

Chorea in primary antiphospholipid syndrome is associated with rheumatic fever

The aim of the study is to evaluate the frequency of chorea in a cohort of primary antiphospholipid syndrome (PAPS) patients and their possible clinical and laboratory associations. The records of 88 PAPS patients, fulfilling Sapporo criteria, followed up at the rheumatology outpatient clinic, were analyzed in order to determine the frequency of chorea. Risk factors for chorea, clinical manifestations, associated comorbidities, serologic features and treatment strategies were analyzed. Eighty-eight PAPS patients were evaluated. Mean age was 40.6 ± 11.1 years, and 91% of them were Caucasian and 91% women. Four (4.5%) patients with chorea were identified: 2 of them (50%) had only one chorea episode and 2 (50%) had recurrent chorea. All patients had chorea onset before PAPS diagnosis. Mean age, gender and ethnical distribution were comparable in groups with or without seizures (P > 0.05). Interestingly, the comparison of the 4 PAPS patients with chorea with those without this abnormality (n = 84) demonstrated a lower BMI [21.1 (18-24.2) vs. 27.5 (17.5-40.9) kg/m(2), P = 0.049] and frequency of venous events (0 vs. 63.1%, P = 0.023) in the first group. A higher frequency of rheumatic fever (75% vs. 0, P < 0.001) and thrombocytopenia (75 vs. 21.4%, P = 0.041) was observed in PAPS individuals with chorea. Both groups were alike regarding the other clinical APS manifestations, disease duration, risk factors for cerebrovascular diseases, use of drugs and antiphospholipid antibodies (P > 0.05). This study demonstrated that 4.5% of PAPS patients had chorea, predominately before PAPS diagnosis, and this neurological abnormality was associated with rheumatic fever and thrombocytopenia. These data reinforce the need for RF diagnosis in those PAPS patients with chorea.     

ANTI-DNA ANTIBODIES IN THE PRIMARY ANTIPHOSPHOLIPID SYNDROME (PAPS)

Primary antiphospholipid syndrome (PAPS) is considered a distinctentity from SLE and patients with PAPS are generally regardedas being dsDNA antibody negative. Levels of IgG and IgM ss andds DNA antibodies were measured by ELISA in 30 patients whofulfilled the criteria for the diagnosis of PAPS. We comparedthese patients with 20 normal controls and seven patients withidiopathic SLE. We also examined all the sera for anti-nuclearantibodies by Hep-2 cells and for dsDNA antibodies by Crithidia. We found that 16 patients with PAPS had antibodies to ss and/ordsDNA. Only three of the 16 positive patients had both IgG andIgM anti-DNA antibodies. Twelve patients had anti-nuclear antibodies,but only two were weakly positive for dsDNA antibodies by Crithidiaimmunofluorescence. Eleven out of 30 patients with PAPS hadIgM anti-dsDNA antibodies compared to two out of the seven SLEpatients. The PAPS patients with anti-DNA antibodies were clinicallyindistinguishable from the PAPS patients without antibodiesagainst DNA. Our results show that 53% of patients with PAPS had antibodiesto DNA which supports the view that PAPS and SLE are probablyoverlapping disorders. KEY WORDS: ELISA, Primary antiphospholipid syndrome, Anti-DNA antibodies