Conduction system in systemic lupus erythematosus with atrioventricular block

Bharati, Saroja; Fuente, Domingo J. de la; Kallen, Ronald J.; Freij, Yaser; Lev, Maurice

doi:10.1016/0002-9149(75)90017-x

Cited by 43 publications

(13 citation statements)

References 15 publications

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“…Three patients died suddenly. An autopsy of 1 of these patients, a 12-year-old girl, revealed conduction system replacement by fibroblastic and mononuclear cells, with degenerative changes present in the remaining cells, and in the myocardium, moderate focal necrosis was found in the hypertrophied ventricle (7). In a series of 8 SLE patients with cardiac conduction system disease, James et al (2) described pathologic findings of pericarditis and small vessel arteriopathy, and in all patients there were scattered inflammatory cells at the interface between the atrioventricular node and the central fibrous body.…”

Section: Case Reportmentioning

confidence: 98%

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High‐grade atrioventricular heart block in an adult with systemic lupus erythematosus: The association of nuclear RNP (U1 RNP) antibodies, a case report, and review of the literature

Bilazarian

Taylor

Brezinski

et al. 1989

Arthritis & Rheumatism

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High-grade atrioventricular heart block (HGAVB) occurring as a manifestation of systemic lupus erythematosus is an exceedingly rare event. We describe a patient with HGAVB who had myositis and antibodies to nuclear RNP (previously associated with myocarditis). A review of the literature on HGAVB associated with systemic lupus erythematosus is also presented.Cardiovascular involvement occurs in the majority of patients with systemic lupus erythematosus (SLE). It is manifested most commonly by pericarditis, which has been reported in 12-100% of patients, and myocarditis, which is clinically apparent in < 10% of patients in most series. It has been recognized in autopsy series in >40% of subjects (1). Although the documented incidence of arrhythmias or conduction disturbance in SLE patients is seldom reported to be >lo%, one can reasonably suspect that the undocumented paroxysmal disturbances in cardiac rhythms are considerably more common (2).High-grade atrioventricular block (HGAVB) as a manifestation of SLE is an extremely rare event, being previously described in only 7 patients (2-7). Several investigators have reported first-or second- degree atrioventricular block in association with SLE (2,8,9). We describe an adult with SLE who developed HGAVB, which occurred in the setting of a putative drug reaction to trimethoprim/sulfamethoxazole (T/S); this patient also had myositis and antibodies to nuclear ribonucleoprotein (nRNP) (U1 RNP), which have been previously associated with myocarditis (10). CASE REPORTThe patient, a 42-year-old black woman with SLE, was referred to The Johns Hopkins Hospital because of intermittent palpitations and dizziness. She had been well until 7 days before admission, at which time she began taking T/S for dysuria. Two days later, palpitations and dizziness began, and these symptoms continued to worsen until the time of admission. On the day prior to admission, the patient discontinued the T/S on her own.Seven years earlier, the patient had been diagnosed as having SLE. Her symptoms at that time were fatigue, periungual erythema, migratory polyarthritis, pleurisy, weakness, and Raynaud's phenomenon. There had been no evidence of sclerodactyly, puffy fingers, or telangiectasias. She was then leukopenic, antinuclear antibody (ANA) positive, and had myositis proven by biopsy. When corticosteroids were administered, her symptoms had resolved and her creatine kinase (CK) level had returned to normal. Prednisone therapy given at that time was gradually completely tapered and withdrawn. For the 2 years prior to the symptoms that brought her to our care, she enjoyed good health and was taking no medications. She denied having cardiac or pericardial symptoms, and previous results of electrocardiograms (EKGs) were

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Section: Case Reportmentioning

confidence: 98%

“…Independently, James et al (2) and Moffitt (4) first reported cases of HGAVB due to SLE in 1965. Only 7 other patients, including children and adults, with HGAVB and SLE (Table 1) have been previously described in the literature (2)(3)(4)(5)(6)(7). The details of their case histories and their outcomes are incomplete.…”

Section: Case Reportmentioning

confidence: 99%

High‐grade atrioventricular heart block in an adult with systemic lupus erythematosus: The association of nuclear RNP (U1 RNP) antibodies, a case report, and review of the literature

Bilazarian

Taylor

Brezinski

et al. 1989

Arthritis & Rheumatism

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“…5 The patient's myocardial infarct was subsequently shown by angiographic examination to have occurred in the presence of normal coronary arteries and was possibly due to compression of the right coronary artery by the aortic mass, which was not demonstrable on the coronary arteriographic study; arteritis, such as that complicating systemic lupus erythematosus; or embolism from either verrucous or infective endocarditis. 6 Finally, it is possible that this patient's heart block was related to the extension of the aortic mass into the interventricular septum, 7 metastatic myocardial calcification in the presence of severe azotemia, 8,9 involvement of the conduction system by systemic lupus erythematosus, 10,11 or a septal abscess complicating infective endocarditis of the aortic valve.…”

Section: P Resentation Of C Asementioning

confidence: 99%

“…17,18 Heart block is uncommon, however, and in the reported cases the diagnosis of lupus was obvious. 10,11 Moreover, verrucous endocarditis rarely causes coronary-artery embolism. Finally, the negative test for antinuclear antibodies in this case makes the diagnosis of systemic lupus erythematosus unlikely.…”

Section: P Resentation Of C Asementioning

confidence: 99%

Case 11-1995

Abraham¹,

Hansen²

1995

N Engl J Med

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A 39-year-old man was admitted to the hospital because of calcifications around the aortic root, a recent myocardial infarction, and subsequent congestive heart failure, with increasing aortic regurgitation.The patient had been well until six years earlier, when progressive renal insufficiency with hypertension was found. A low-protein diet and enalapril were begun. He was subsequently well until four months before admission, when chest pain developed and he entered another hospital. The urea nitrogen concentration was 143 mg per deciliter (51.1 mmol per liter), and the creatinine concentration was 9.4 mg per deciliter (830 m mol per liter). An electrocardiogram showed an acute myocardial infarct. A transthoracic cardiac ultrasonographic examination revealed a mass around the aortic root that was suspected to represent an aneurysm of the sinus of Valsalva. A transesophageal cardiac ultrasonographic examination at another hospital showed a multilobulated mass with a highly reflective surface and a sonolucent center encircling the anterior aspect of the aortic root and right coronary artery. Dense echogenic material extended onto the base of the anterior mitral leaflet. The aortic valve was tricuspid, with mild stenosis and regurgitation. The peak systolic aortic gradient by continuous-wave Doppler study was 40 mm Hg. The left ventricular size and ejection fraction were normal; the inferior segments of the left ventricle appeared slightly hypokinetic. A magnetic resonance imaging (MRI) scan of the heart also disclosed an extensive mass that involved the aortic root and the myocardial surface of the right ventricle, without obvious invasion of the ventricle. Serologic tests for brucella and coxiella microorganisms were negative. The patient was afebrile, and multiple blood-culture specimens were ster-ile. It was decided not to perform a surgical procedure. The urea nitrogen concentration declined to 88 mg per deciliter (31 mmol per liter), and the creatinine concentration to 8.8 mg per deciliter (780 m mol per liter). The patient was discharged, with arrangements made for extracorporeal hemodialysis.The patient felt well until two weeks before admission, when he began to have bouts of dyspnea, usually after dialysis. Four days before admission he was treated for congestive heart failure at another hospital. There was evidence of increasing aortic regurgitation. Episodes of intermittent atrioventricular dissociation, with a ventricular rate of 40 to 50, were observed, and on the fourth hospital day a sequential dual-chamber electronic pacemaker was implanted. The patient was transferred to this hospital.The patient was unmarried and lived alone. He was a veterinarian who treated dogs and cats predominantly. He traveled to England in the summer and frequently took cruises to the Caribbean and South America. His father had died at the age of 41 years of a ruptured intracranial mycotic aneurysm due to bacterial endocarditis on a bicuspid aortic valve. There was no family history of renal disease. The patient's medicatio...

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“…1,2 While electrical abnormalities can occur, isolated atrioventricular (AV) conduction disease, ranging from first degree to complete AV block (AVB), is rarely seen in older children and adults with SLE such that pacemakers are infrequently required. [3][4][5][6][7][8] A few reports have described abnormal atrial electrical activity in the form of atrial standstill, which was speculated to be secondary to recurrent flares of SLE, pericarditis, myocarditis, and/or myocardial arteritis. 9 In contrast, complete AVB and ventricular cardiomyopathy are well-established immunologic complications in neonates of mothers with anti-Ro/SSA antibodies, such that pacemakers are frequently indicated.…”

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confidence: 99%

Left Atrial Inexcitability in Children With Congenital Lupus‐Induced Complete Atrioventricular Block

Abadir

Fournier

Vobecky

et al. 2015

JAHA

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BackgroundCongenital atrioventricular block is a well‐established immunologic complication of maternal systemic lupus erythematosus. We sought to further characterize the electrophysiological manifestations of maternal systemic lupus erythematosus on neonatal atria.Methods and ResultsCases of isolated congenital atrioventricular block treated at our center over the past 41 years were identified. Data were extracted from clinical charts, pacemaker interrogations, ECGs, echocardiograms, and histopathological reports, when available. Of 31 patients with isolated congenital atrioventricular block, 18 were negative for maternal antibodies and had normal epicardial atrial sensing and pacing thresholds. In contrast, 12 of 13 patients with positive maternal antibodies had epicardial pacemakers, 5 (42%) of whom had left atrial (LA) inexcitability and/or atrial conduction delay. In 3 patients, the LA could not be captured despite high‐output pacing. The fourth patient had acutely successful LA appendage and left ventricular lead placement. At early follow‐up, an increased delay between the surface P‐wave and intracardiac atrial depolarization was observed, indicative of atrial conduction delay. The fifth patient exhibited LA lead dysfunction, with atrial under‐sensing and an increased capture threshold, 2 weeks after implantation. Biopsies of LA appendages performed in 2 patients showed no evidence of atrial fibrosis or loss of atrial myocytes.ConclusionsHerein, we report previously undescribed yet prevalent electrophysiological ramifications of maternal systemic lupus erythematosus, which extend beyond congenital atrioventricular block to encompass alterations in LA conduction, including LA inexcitability. These manifestations can complicate epicardial pacemaker implantation in newborns. In the absence of histological evidence of extensive atrial fibrosis, immune‐mediated functional impairment of electrical activity is suspected.

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Conduction system in systemic lupus erythematosus with atrioventricular block

Cited by 43 publications

References 15 publications

High‐grade atrioventricular heart block in an adult with systemic lupus erythematosus: The association of nuclear RNP (U1 RNP) antibodies, a case report, and review of the literature

High‐grade atrioventricular heart block in an adult with systemic lupus erythematosus: The association of nuclear RNP (U1 RNP) antibodies, a case report, and review of the literature

Case 11-1995

Left Atrial Inexcitability in Children With Congenital Lupus‐Induced Complete Atrioventricular Block

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