Prognostic importance of cardiac arrhythmias in systemic sclerosis

doi:10.1016/0002-9343(88)90305-1

The American Journal of Medicine

Volume 84, Issue 6, June 1988, Pages 1007-1015

https://doi.org/10.1016/0002-9343(88)90305-1 Get rights and content

Abstract

Ambulatory electrocardiography was performed in 183 patients with systemic sclerosis recruited from five centers who were selected to reflect a balanced population with respect to disease extent and duration. Ventricular ectopy occurred in 67 percent of patients and was strongly correlated by both univariate and multivariate analyses with total mortality and with sudden death. By multivariate analysis, ventricular ectopy was strongly associated with increasing patient age and with other evidence of cardiac and pulmonary involvement but not with clinical and laboratory measures of duration and extent of systemic sclerosis. Evidence of myocardial fibrosis thought to be secondary to microvascular alteration is common in systemic sclerosis, but the clinical implications of myocardial involvement are less well appreciated. The present data suggest the need for ambulatory electrocardiography in the clinical assessment of selected patients with systemic sclerosis, especially those with cardiac or pulmonary involvement, as well as for studies of the effects of antiarrhythmic therapy.

References (45)

W.A. D'Angelo et al.
Pathologic observations in systemic sclerosis (scleroderma): a study of fifty-eight autopsy cases and fifty-eight matched controls
Am J Med
(1969)
M.A. Sackner et al.
The heart in scleroderma
Am J Cardiol
(1966)
J. Wynn et al.
Prediction of survival in progressive systemic sclerosis by multivariate analysis of clinical features
Am Heart J
(1985)
B.H. Bulkley
Progressive systemic sclerosis: cardiac involvement
Clin Rheum Dis
(1979)
W.P. Follansbee et al.
The electrocardiogram in systemic sclerosis (scleroderma)
P.J. Clements et al.
The relationship of arrhythmias and conduction disturbances to other manifestations of cardiopulmonary disease in progressive systemic sclerosis
Am J Med
(1981)
T.A. Medsger
Progressive systemic sclerosis
Clin Rheum Dis
(1983)
A.T. Masi et al.
Methods and preliminary results of the Scleroderma Criteria Cooperative Study of the American Rheumatism Association
Clin Rheum Dis
(1979)
M.J. Fritzler et al.
The CREST syndrome: a distinct serologic entity with anticentromere antibodies
Am J Med
(1980)
W.P. Follansbee et al.
Myocardial function and perfusion of the CREST syndrome variant of progressive systemic sclerosis

W.W. Ellis et al.

Left ventricular dysfunction induced by cold exposure in patients with systemic sclerosis

Am J Med

(1986)

G.R. Owens et al.

Cardiopulmonary manifestations of systemic sclerosis

Chest

(1987)

R.L. Ridolfi et al.

The cardiac conduction system in progressive systemic sclerosis: clinical and pathologic features of 35 patients

Am J Med

(1976)

J.B. Kostis et al.

The prognostic significance of ventricular ectopic activity in survivors of acute myocardial infarction

J. Mukharji et al.

Risk factors for sudden death after acute myocardial infarction: two-year follow-up

Am J Cardiol

(1984)

R.E. Brashear

Arrhythmias in patients with chronic obstructive pulmonary disease

Med Clin North Am

(1984)

R.H. Young et al.

Pulmonary vascular changes in scleroderma

Am J Med

(1978)

R.G. Ungerer et al.

Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis

Am J Med

(1983)

G.R. Owens et al.

Pulmonary function in progressive systemic sclerosis

T.A. Medsger et al.

Survival with systemic sclerosis (scleroderma)

W.P. Follansbee

The cardiovascular manifestations of systemic sclerosis (scleroderma)

Curr Probl Cardiol

(1986)

N.K. Roberts et al.

The prevalence of conduction defects and cardiac arrhythmias in progressive systemic sclerosis

Ann Intern Med

(1981)

Cited by (136)

Sudden cardiac death, arrhythmias and abnormal electrocardiography in systemic sclerosis: A systematic review and meta-analysis
2023, Seminars in Arthritis and Rheumatism
To calculate the frequency of sudden cardiac death(SCD), arrhythmia and conduction defects in SSc.
MEDLINE/EMBASE were searched to January 2023. English-language studies reporting the incidence/frequency of SCD, arrhythmia and electrocardiography(ECG) abnormalities in SSc were included. Odds ratios(OR), estimations of annual incidence or pooled frequencies were calculated.
Seventy-nine studies(n = 13,609 participants with SSc) were included in the meta-analysis. Methodology and outcomes were heterogeneous.
Ten studies included cohorts with known/suspected SSc-associated heart involvement(SHI), generally defined as clinically-manifest cardiac disease/abnormal cardiac investigations. The incidence of SCD in SHI was estimated to be 3.3% annually(n = 4 studies, 301PY follow-up). On ambulatory ECG, 18% of SHI cohorts had non-sustained ventricular tachycardia(NSVT; n = 4, 95%CI3.2–39.3%), 70% frequent premature ventricular complexes (PVCs; n = 1, 95%CI34.8–93.3%), and 8% atrial fibrillation (AF; n = 1, 95%CI4.2–13.6%).
Nineteen studies included participants without SHI, defined as normal cardiac investigations/absence of cardiac disease. The estimated incidence of SCD was approximately 2.9% annually (n = 1, 67.5PY). Compared to healthy controls, individuals without SHI demonstrated NSVT 13.3-times more frequently (n = 2, 95%CI2–102), and paroxysmal supraventricular tachycardia 7-times more frequently (n = 4, 95%CI3–15). Other ambulatory ECG abnormalities included NSVT in 9% (n = 7, 95%CI6–14%), >1000 PVCs/24 h in 6% (n = 2, 95%CI1–13%), and AF in 7% (n = 5, 0–21%).
Fifty studies included general SSc cohorts unselected for cardiac disease. The incidence of SCD was estimated to be 2.0% annually(n = 4 studies, 1646PY). Unselected SSc cohorts were 10.5-times more likely to demonstrate frequent PVCs (n = 2, 95%CI 2–59) and 2.5-times more likely to have an abnormal electrocardiography (n = 2, 95%CI1–4).
The incidence of SCD in SSc is estimated to be 1.0–3.3% annually, at least 10-fold higher than general population estimates. Arrhythmias including NSVT and frequent PVCs appear common, including amongst those without known/suspected SHI.
Cardiac involvement in systemic sclerosis: Getting to the heart of the matter
2021, Best Practice and Research: Clinical Rheumatology
Citation Excerpt :
Both supraventricular and ventricular rhythm disturbances are frequently detected in 45%–61% of patients with SSc, including premature ventricular contractions, couplet and triplet beats, bigeminy, trigeminy, quadrigeminy, ventricular tachycardia, and atrial fibrillation, or flutter [32,33,35]. Different groups have reported a significant association between the presence of arrhythmias, in particular ventricular arrhythmias, with both inflammatory and fibrotic changes measured on CMR, and ventricular arrhythmias have been associated with increased mortality [33,35,36]. Autonomic dysfunction in SSc can also be detected through electrophysiological studies, with reports of higher heart rate, reduced heart rate variability, and impaired heart rate recovery in patients [37,38].
Primary systemic sclerosis heart involvement (pSHI) is an important disease manifestation that accounts for a significant proportion of systemic sclerosis (SSc)-associated mortality. A broad clinical spectrum of pSHI exists, which ranges from asymptomatic perfusion abnormalities to diastolic dysfunction or acute myocarditis and congestive heart failure. With improving sensitivity of cardiac investigations, it is increasingly recognized that there is a large burden of subclinical cardiac disease in patients with SSc. Early signs of pSHI can be subtle and determining the etiology of cardiac abnormalities from other causes of cardiomyopathy such as hypertension, ischemic heart disease (IHD), and pulmonary vascular disease remain challenging. Early identification of pSHI potentially provides clinicians with a window of opportunity for intervention to avert progression to heart failure. However, optimal screening and treatment guidelines are lacking, and it is an area of much needed further clinical research.
CardioRheumatology
2021, Sex Differences in Cardiac Diseases: Pathophysiology, Presentation, Diagnosis and Management
A 41-year-old female patient with a history of systemic lupus erythematosus presents to the emergency room with symptoms of chest pain. The pain is described as sharp and midsternal, with radiation to the left shoulder. The pain started while at rest and has been constant, although worsens with activity and deep breaths. There is associated shortness of breath but no diaphoresis, nausea, or lightheadedness. No additional symptoms of syncope or edema. The week prior she had experienced symptoms of a cough, malaise, and subjective fevers/chills. The patient was diagnosed with lupus in her late 20s when she presented with profound fatigue and joint pain as well as a malar rash. She is maintained on mycophenolate mofetil and prednisone 5 mg daily. Currently, she is experiencing some increase in the pain in her hands and feet, along with the aforementioned recent chest pain.
Upon presentation to the emergency room, her blood pressure was 112/78, heart rate 82. Her physical exam was remarkable for a malar rash, mild lower extremity edema, and a soft pericardial friction rub. An electrocardiogram was done in the emergency room (Figure 1) and did not show evidence of an acute myocardial infarction. Pertinent labs included a normal white blood cell count, mild anemia, a sedimentation rate of 95 mm/1 h (normal ≤ 29 mm/1 h), and C-reactive protein of 177 mg/L (normal ≤ 9 mg/L). Cardiac biomarkers revealed a troponin T of 0.17 ng/mL at 0 h, 0.19 ng/mL at 3 h, and 0.21 ng/mL at 6 h (normal ≤ 0.01 ng/mL). How would you manage this patient?
Women have an increased risk of certain chronic autoimmune inflammatory disorders (CIDs). All aspects of the heart’s structure and function may be affected by the autoimmune disease. Many of the cardiovascular (CV) complications may be clinically silent and subclinical. However, there is still a clear burden of increased CV morbidity and mortality in these patients. Despite the growing recognition of the increased CV risk in the inflammatory autoimmune patient population, at present there are no specific guidelines that address the risk. A heightened awareness of the CV risk is important as one evaluates and treats the CID patient. Although the CID patient may not report CV symptomatology, their ongoing inflammatory condition may be resulting in damage to the entire cardiac system.
Very early systemic sclerosis
2019, Best Practice and Research: Clinical Rheumatology
The early diagnosis of systemic sclerosis (SSc) can be very difficult, when most of the typical signs and symptoms are absent. For this reason, the approach to SSc has changed during the last decades because the importance of an early diagnosis and treatment has been widely understood. “Very early SSc” is identified as a condition characterized by Raynaud's phenomenon, puffy fingers, disease-specific autoantibodies, and microvascular alterations at capillaroscopy. However, reliable biomarkers able to predict the disease evolution are missing, and decision whether to treat or not to treat in the earliest phase of the disease remains a dilemma. Presently, the only feasible clinical strategy in very early SSc remains a tight follow-up program to detect in “real time” the onset of internal organ involvement, which may thus allow an aggressive therapeutic agenda.
Defining primary systemic sclerosis heart involvement: A scoping literature review
2019, Seminars in Arthritis and Rheumatism
Citation Excerpt :
A number of studies reported that ventricular dysfunction was associated with the development of arrhythmias, suggesting that SSc patients with ventricular dysfunction may be at a particularly high risk of clinically significant rhythm disturbance or sudden cardiac death [5,6,234,253,275]. Independent of ventricular function, a high number of ventricular ectopic beats or premature ventricular contractions have been associated with a greater risk of sudden cardiac death [8,245,261,275]. A greater degree of SSc-associated cardiac fibrosis, detected either at biopsy or by CMR has been shown to be associated with increased risk of clinically significant arrhythmia [8,80,186].
Background: Clinically evident primary heart involvement due to systemic sclerosis (SHI) is considered a poor prognostic factor and is a leading cause of systemic sclerosis (SSc) related death. Yet, there remains no consensus definition of SHI and poor understanding of the natural history and risk factors for the development of SHI.
Methods: We performed a scoping literature review of published articles with a primary focus of SHI to capture previously used definitions of SHI and items used to measure SHI. Any factors reported to be associated with an increased risk of SHI were recorded.
Results: Of the 2436 records identified in a search of MEDLINE, EMBASE and PubMed databases, 295 were included in the final scoping review. Analysis of the literature revealed studies of variable quality, generally low patient numbers and highly heterogeneous definitions of SHI within studies. There is no clear consensus from the literature as to the scope of SHI and the prognostic significance of sub-clinical investigation abnormalities commonly detected.
Conclusion: The lack of a standardised definition of SHI remains a significant unmet need in SSc. The results of this review will assist in the development of consensus classification criteria to enable more accurate quantification of the burden of SHI, identification of factors associated with increased risk of developing SHI, and evaluation of the efficacy of any novel therapeutic strategies.
QTc interval prolongation in Systemic Sclerosis: Correlations with clinical variables and arrhythmic risk
2017, International Journal of Cardiology

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^☆: This study was supported in part by Grants HL14985 and HL07171 from the National Institutes of Health, Bethesda, Maryland; the Scleroderma Society, Inc., New York, New York; Scleroderma Research Foundation, Columbus, New Jersey; and Janssen Pharmaceutica, Piscataway, New Jersey.

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Prognostic importance of cardiac arrhythmias in systemic sclerosis☆

Abstract

Am J Med

Am J Cardiol

Am Heart J

Clin Rheum Dis

Am J Med

Clin Rheum Dis

Clin Rheum Dis

Am J Med

Am J Med

Chest

Am J Med

Am J Cardiol

Med Clin North Am

Am J Med

Am J Med

Survival with systemic sclerosis (scleroderma)

The cardiovascular manifestations of systemic sclerosis (scleroderma)

Curr Probl Cardiol

The prevalence of conduction defects and cardiac arrhythmias in progressive systemic sclerosis

Ann Intern Med