Chronic Dermatomyositis with Intermittent Trifascicular Block: An Electrophysiologic-Conduction System Correlation

doi:10.1378/chest.71.3.413

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Volume 71, Issue 3, March 1977, Pages 413-416

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A case of dermatomyositis associated with recurrent syncope and complete heart block is described. Right bundle branch block and left anterior hemiblock with intact atrioventricular conduction then persisted over a five-year period. His bundle studies demonstrated a prolonged His-ventricle (H-V) interval. Postmortem examination confirmed the diagnosis of dermatomyositis as well as a fibrotic cardiomyopathy. The pathologic findings of marked fibrosis of both bundle branches, especially the right bundle branch and the anterior portion of the main left bundle branch, correlated well with the electrocardiographic and electrophysiologic findings.

Section snippets

Case Report

In November 1967, a 47-year-old black man initially had severe pain and weakness of both thighs as well as discoloration of his skin. The concentrations of serum glutamic oxalacetic transaminase (SGOT), creatinine phosphokinase, and aldolase were markedly elevated. A skin and muscle biopsy was diagnostic of chronic dermatomyositis and demonstrated chronic dermatitis and skin atrophy, as well as severe focal necrosis and degeneration of skeletal muscles. The patient responded to steroid therapy.

His Bundle Studies

His Bundle studies were performed on March 3, 1972. Two bipolar catheters were inserted percutaneously from the right femoral vein, with one placed at the tricuspid valve to obtain His bundle electrograms by standard techniques,⁹ and the other catheter was placed in the superior right atrium for recording an atrial electrogram and for atrial pacing.

During sinus rhythm, the P-R interval was 160 msec with a P-A interval of 20 msec, an atrio-His (A-H) interval of 80 msec, and a His-ventricle (H-V)

Postmortem Examination

General. Large nonseptate fungi were present in both orbits, the anterior sinuses, most cranial nerves, and the basal leptomeninges. A Rhizopus species grew out of cultures.

The skin showed epidermal atrophy, dermal sclerosis, and edema. There was generalized marked atrophy of muscles, with local areas of necrosis and inflammation. There was no evidence of malignant disease.

Heart. On gross examination, the heart was enlarged, weighed 500 gm, and exhibited hypertrophy of all chambers. There was

Discussion

Chronic dermatomyositis is an inflammatory and degenerative myopathy associated with dermatitis.1, 2 This patient demonstrated the strictest criteria for chronic dermatomyositis with (1) clinical features of severe muscle pain, weakness, and atrophy, as well as dermatitis; (2) elevation of serum concentrations of skeletal muscular enzymes; and (3) demonstration of varying stages of muscular necrosis, inflammation, fibrosis, and degeneration, along with epidermal atrophy and dermal infiltration,

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Dermatomyosite compliquee de bloc auriculo-ventriculaire complet avec syndrome d'Adams-Stokes: Implanation d'un stimulateur interne
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Cited by (34)

Systemic Diseases and Heart Block
2023, Cardiology Clinics
Systemic Diseases and Heart Block
2021, Cardiac Electrophysiology Clinics
Citation Excerpt :
Similar inflammatory changes and eventual contraction-band necrosis have been observed involving the cardiac conduction system on autopsy studies.37,38 In other post mortem studies of patients with heart block, extensive fibrous replacement of the AV node, His bundle, and bundle branches have been reported.38–40 Cardiac involvement is often subclinical, and presentation delayed to a few years after the initial diagnosis of the rheumatologic condition.
Dermatomyositis and Polymyositis
2017, The Heart in Rheumatic, Autoimmune and Inflammatory Diseases: Pathophysiology, Clinical Aspects and Therapeutic Approaches
Idiopathic inflammatory myopathies (IIMs), collectively called myositis, are a heterogeneous group of diseases affecting adults and children. The IIMs are characterized by symmetrical, proximal muscle weakness and by inflammatory infiltrates in skeletal muscles. Based on differences in clinical and histopathological features, they can be divided into different subgroups, the most common forms being dermatomyositis (DM), polymyositis (PM), sporadic inclusion body myositis (sIBM), and in children juvenile dermatomyositis (JDM). Other organs are frequently involved in patients with IIM eg, lungs, gastrointestinal tract, and the heart, emphasizing that these are systemic inflammatory diseases. IIMs are associated with increased mortality, and cardiovascular involvement has been recognized as the main prognostic factor for death. Processes affecting the heart in IIM include coronary artery disease and cardiac affection caused by inflammation. In this chapter, we focus on adult PM and DM and JDM as little is known about cardiac involvement in the other subsets.
Electrocardiographic changes in dermatomyositis and polymyositis
2016, Revista Brasileira de Reumatologia
Citation Excerpt :
And the pathogenesis of DM and PM itself also may have contributed to left chamber hypertrophy. There are indications of presence of infiltration of mononuclear inflammatory cells in the endomysium and in perivascular areas of infarction, as well as degeneration of cardiac myocytes and areas of myocardial fibrosis.12,13,18,19 This inflammatory process in the myocardium can lead to remodeling, with anatomical and functional changes of the myocardium and, ultimately, possible left ventricular dysfunction and restrictive cardiomyopathy and heart failure.
Cardiac involvement is frequent in inflammatory myopathies. Electrocardiogram (ECG) may show evidence of this involvement and its changes should be well-known and described.
Due to the lack of studies in the literature, we conducted an analysis of the ECG findings in patients with dermatomyositis (DM) and polymyositis (PM), comparing them with a control group.
This cross-sectional study compared the ECG of 86 individuals with no rheumatic disorders (controls) with 112 patients (78 DM and 34 PM), during 2010–2013. The ECG findings between DM and PM were also compared.
Demographic characteristics, comorbidities and ECG abnormalities were similar between controls and patients (p > 0.05), except for a higher frequency of left ventricular hypertrophy (LVH) in patients (10.7% vs. 1.2%, p = 0.008). Demographic characteristics, comorbidities, clinical and laboratory manifestations, were also similar between the groups PM and DM, except for the presence of cutaneous lesions only in DM. One-third of the patients had ECG abnormalities, which were more prevalent in PM than DM (50% vs. 24.4%, p = 0.008). LVH, left atrial enlargement, rhythm and conduction abnormalities were more frequent in PM than DM (p < 0.05 for all), especially the left anterior fascicular block.
We showed distinct ECG changes between DM and PM and a higher frequency of LVH in patients compared to controls. Investigation of cardiac involvement should be considered even in asymptomatic patients, especially PM. Further studies are necessary in order to determine the correlation of ECG findings with other complementary tests, clinical manifestations, disease activity and progression to other cardiac diseases.
Acometimento cardíaco nas miopatias inflamatórias é frequente. Eletrocardiograma (ECG) pode mostrar indícios desse acometimento e suas alterações devem ser bem conhecidas e descritas.
Devido à escassez de trabalhos na literatura, analisamos as alterações de ECG em pacientes com dermatomiosite (DM) e polimiosite (PM) e as comparamos com um grupo controle.
Este estudo transversal comparou ECGs de 86 indivíduos sem doenças reumatológicas (controles) com 112 pacientes (78 DM e 34 PM), de 2010 a 2013. Também comparamos os ECGs entre DM e PM.
Características demográficas, comorbidades e alterações de ECG foram semelhantes entre controles e pacientes (p > 0,05), exceto pela maior frequência de sobrecarga de ventrículo esquerdo (SVE) nos pacientes (10,7% vs. 1,2%; p = 0,008). Características demográficas, comorbidades, manifestações clínicas e laboratoriais também foram semelhantes entre os grupos PM e DM, exceto por lesões cutâneas apenas em pacientes com DM. Um terço dos pacientes apresentou alterações de ECG, que foram mais prevalentes em PM do que em DM (50% vs. 24,4%, p = 0,008). Sobrecarga de câmaras esquerdas (SCE), distúrbios do ritmo e da condução foram mais encontrados em PM do que em DM (p < 0,05 para todos), sobretudo o bloqueio divisional do ramo anterossuperior.
Encontramos alterações distintas de ECG entre PM e DM e frequência aumentada de SVE em pacientes quando comparados com controles. Investigação do acometimento cardíaco nessas doenças deve ser considerada mesmo em pacientes assintomáticos, especialmente em se tratando de PM. Mais estudos são necessários para correlacionar os achados de ECG com outros exames complementares, manifestações clínicas, atividade das miopatias e evolução para outras doenças cardíacas.
Clinical cardiac involvement in idiopathic inflammatory myopathies: A systematic review
2011, International Journal of Cardiology
Citation Excerpt :
Another longitudinal study by Kiely et al. had also revealed 6 out of 16 patients with IIM having elevated CKMB (median of 75 μg/L, range of 5.1–1183 μg/L) while only one patient had an elevated cTnI with CK-MB levels below the diagnostic range of MI [39]. EPS has been reported in seven patients from four articles [14,17,22,40]. Patients presented with symptoms of lightheadedness and dyspnea with or without palpitations.
Idiopathic inflammatory myopathies (IIM) presenting with diffuse skeletal muscular inflammation and cardiac involvement is one of the major causes of clinical deterioration. Our purpose was to observe the frequency of various reported clinical cardiac afflictions, cardiovascular mortality and its most important contributors in the IIM population.
MEDLINE database was searched from 1977 through 2009. Articles reporting objective evidence of cardiac involvement were assessed. Patients were included if they satisfied Bohan and Peter criteria for definite or probable diagnosis of IIM. The review conforms to the criteria of the new PRISMA statement of preferred reporting items for systematic reviews and meta-analyses.
Thirty-three out of 90 articles reported cardiac data. Twelve were case reports, while 21 articles were either cohort studies or case series. Angina was reported in 7% of cumulative prospective cohort. Dysrhythmias were seen in 31.8% of electrocardiograms. Diastolic dysfunction was the most common echocardiographic finding. Congestive heart failure was reported in 5.6% of the prospective cohort and was the most common cause of death accounting for 21% of total cardiac mortality. Myocarditis was the most common feature in reported pathology literature (38%), followed by focal myocardial fibrosis (22%).
Cardiac involvement is an important cause of morbidity and mortality in patients with IIM. Myocardial fibrosis puts the patient at risk for systolic or diastolic heart failure and dysrhythmias. This review, therefore, suggests the importance of early and comprehensive cardiac evaluation in IIM population. Confirmation of these findings will require prospective studies of consecutive patients with long-term follow-up.
Myocardial dysfunction in polymyositis
2006, Canadian Journal of Cardiology
Myocardial involvement in polymyositis is occasionally suspected, but symptomatic cardiac dysfunction is rarely reported. Described in the present report is a 48-year-old woman with a two-year history of polymyositis who suddenly developed near fatal ventricular arrhythmia, and a 56-year-old man with a relapsing polymyositis who developed severe systolic dysfunction. These two cases emphasize the importance of systematic cardiac evaluation when the diagnosis of polymyositis is initially made and the necessity of re-evaluating cardiac function, even in the presence of clinical remission and normalization of creatine phosphokinase with treatment.
Dans la polymyosite, on soupçonne parfois une atteinte myocardique, mais les cas de dysfonction cardiaque symptomatique sont rares. Le présent rapport décrit le cas d’une femme de 48 ans présentant des antécédents de polymyosite depuis deux ans qui a subitement présenté une arythmie ventriculaire quasi fatale et celui d’un homme de 56 ans victime d’une rechute de sa polymyosite qui a développé une dysfonction systolique sévère. Ces deux cas rappellent l’importance d’une évaluation cardiaque systématique lorsqu’un diagnostic de polymyosite a déjà été posé et la nécessité de réévaluer la fonction cardiaque, même si le patient semble en rémission clinique et même si un traitement a permis de normaliser les taux de créatine phosphokinase.

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This investigation was supported by grant HL 07605-14 from the National Heart and Lung Institute.

Reprint requests: Dr. Lightfoot, Kaiser Foundation Hospital, Fontana, California 92335

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SELECTED REPORTSChronic Dermatomyositis with Intermittent Trifascicular Block: An Electrophysiologic-Conduction System Correlation

Section snippets

Case Report

His Bundle Studies

Postmortem Examination

Discussion

Am J Med

Am J cardiol

Am J Cardiol

Am J Cardiol

Polymyositis and dermatomyositis

N Engl J Med

Rheumatic and collagen disorders of the heart

Dermatomyosite compliquee de bloc auriculo-ventriculaire complet avec syndrome d'Adams-Stokes: Implanation d'un stimulateur interne

Bull Soc Med Hop Paris

SELECTED REPORTS
Chronic Dermatomyositis with Intermittent Trifascicular Block: An Electrophysiologic-Conduction System Correlation