Systemic Sclerosis and the Heart

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Echocardiography. 2007 Apr;24(4):374-84.
The impact of hypertension and hypertension-related left ventricle hypertrophy on right ventricle function.
Tumuklu MM, Erkorkmaz U, Ocal A.
Department of Cardiology, Faculty of Medicine, University of Gaziomanspasa, Tokat, Turkey.

AIM: The aim of our study is to determine the effect of hypertension and hypertension-related left ventricle hypertrophy on right ventricle (RV) morphology and function by using RV standard Doppler echocardiographic indices, myocardial Doppler imaging, and strain/strain rate imaging indices. METHODS: We studied 35 patients with arterial hypertension and 30 age- and sex-adjusted control subjects who had no other pathological conditions. Standard transthoracic Doppler echocardiographical measurements, pulsed-wave tissue Doppler from tricuspid anulus (Peak systolic-st, peak early diastolic-et, peak late diastolic velocity-at), reconstructed spectral pulsed-wave tissue Doppler velocities (peak systolic-S, peak early-E, peak late diastolic velocity-A), and strain/strain rate imaging of RV free wall mid region (peak systolic strain-in, peak systolic strain rate-SR) were obtained. RESULTS: Age, body surface area, blood pressure, and heart rate were comparable between two groups. Hypertensive subjects had significantly increased LV end-diastolic septal and posterior wall thickness, left atrial diameter, LV mass, LV mass index, and relative wall thickness during diastole. At the level of right ventricular lateral tricuspid annulus without systolic changes, the majority of diastolic measurements were altered in hypertensives (early diastolic velocity et; 13 +/- 2 vs. 18 +/- 4 m/sec, P < 0.0001, late diastolic velocity at; 20 +/- 4 vs. 14 +/- 3 m/sec, P < 0.0001, early to late diastolic velocity ratio; 0.69 +/- 0.14 vs. 1.32 +/- 0.38, P < 0.0001). The velocity data from two-dimensional color myocardial imaging at the level of RV free wall mid region again showed altered diastolic measurements in hypertensives (E; 8.01 +/- 2.6 vs. 10.4 +/- 3.14 m/sec, P < 0.001, A; 11.5 +/- 2.6 vs. 9.12 +/- 3.7 m/sec, P < 0.0001, E/A ratio; 0.75 +/- 0.41 vs. 1.87 +/- 0.48, P < 0.00). The peak systolic strain of RV free wall mid region was significantly lower in hypertensive individuals than controls (25.666 +/- 5.64 vs. 30.03 +/- 6.78%, P < 0.05). No significant differences were found in other parameters of RV function between hypertensive and control subjects. CONCLUSIONS: The present study demonstrates that besides the manifest morphologic LV adaptations, significant RV functional alterations can be determined by TDI and strain/strain rate imaging in patients arterial hypertension. Both tissue velocities by TDI and strain imaging may be new tools to define and quantitate subtle change in systolic and diastolic function of right ventricular function in arterial hypertension that cannot be determined in standard echocardiographic parameters.

Chest. 2007 Apr;131(4):988-92.
Pericardial abnormalities predict the presence of echocardiographically defined pulmonary arterial hypertension in systemic sclerosis-related interstitial lung disease.
Fischer A, Misumi S, Curran-Everett D, Meehan RT, Ulrich SK, Swigris JJ, Frankel SK, Cosgrove GP, Lynch DA, Brown KK.
Division of Rheumatology, National Jewish Medical and Research Center, 1400 Jackson St, Denver, CO 80206.

OBJECTIVES: To determine the prevalence and significance of pericardial abnormalities in systemic sclerosis (SSc)-related interstitial lung disease (ILD). METHODS: Retrospective study of 41 subjects with SSc-related ILD who underwent evaluation including thoracic high-resolution CT (HRCT) imaging, transthoracic echocardiography (TTE), and pulmonary function testing. HRCT review evaluated the pericardium for the presence of pericardial effusion (PEf), thickness of the anterior pericardial recess (APR) [abnormal defined as > 10 mm], and pericardial thickening as calculated by total pericardial score (TPS) [abnormal defined as > 8 mm]. Pulmonary arterial hypertension (PAH) was defined as a pulmonary artery pressure > 35 mm Hg estimated by TTE. RESULTS: Fifty-nine percent had an abnormal pericardium, 49% had a PEf, 56% had an abnormal APR, and 49% had an abnormal TPS. An abnormal pericardium was more common in men than women. Subjects with and without pericardial abnormalities were otherwise similar with respect to age, SSc classification, autoantibodies, ILD radiographic pattern, and presence of esophageal dilation. Both groups had similar median percentage of predicted total lung capacity, percentage of predicted FVC, percentage of predicted FEV(1), and percentage of predicted diffusion capacity of the lung for carbon monoxide. Subjects with pericardial abnormalities were more likely to have coexistent PAH (35% vs 75%; p = 0.02) and a higher median right ventricular systolic pressure (31 mm Hg vs 44 mm Hg; p = 0.03). Multiple logistic regression revealed that TPS was the best individual predictor of the presence of TTE-defined PAH. CONCLUSIONS: In patients with SSc-related ILD, pericardial abnormalities are commonly seen on HRCT, and their presence is strongly associated with echocardiographically defined PAH, with abnormal TPS as the best individual predictor.

J Natl Med Assoc. 2007 Mar;99(3):232-7.
Cardiac repolarization abnormalities and increased sympathetic activity in scleroderma.
Ciftci O, Onat AM, Yavuz B, Akdogan A, Aytemir K, Tokgozoglu L, Sahiner L, Deniz A, Ureten K, Kizilca G, Calguneri M, Oto A.
Department of Cardiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

BACKGROUND: Cardiac involvement in scleroderma is a poor prognostic sign and is usually underdiagnosed, particularly in asymptomatic patient. This paper focuses on QT dynamicity and heart rate variability (HRV) in patients with scleroderma and controls in an attempt to investigate the cardiac autonomic system and ventricular repolarization. METHODS: Sixty patients with scleroderma and 30 age- and sex-matched healthy controls who had no cardiovascular risk factors were included in this study. All patients and the controls underwent a 24-hour holter recording as well as a transthoracic echocardiography. HRV and QT dynamicity parameters were calculated. RESULTS: In HRV analysis, autonomic balance was changed in favor of the sympathetic system in patients with diffuse scleroderma. In QT dynamicity analysis, QT/RR slopes were significantly steeper in patients with diffuse scleroderma compared to patients with limited scleroderma and controls (QTapex/RR: 0.24 +/- 0.16, 0.15 +/- 0.03, 0.14 +/- 0.03 respectively p < 0.001; QTend/RR: 0.26 +/- 0.17, 0.14 +/- 0.04, 0.13 +/- 0.05, respectively p < 0.001). CONCLUSIONS: Patients with diffuse scleroderma may have asymptomatic cardiac repolarization abnormalities and autonomic dysfunction. Our results may indicate that QT dynamicity and HRV can be useful noninvasive methods that may detect impaired state of autonomic balance and cardiac repolarization in patients with diffuse scleroderma.

Echocardiography. 2007 Feb;24(2):118-25.
Right heart function and scleroderma: insights from tricuspid annular plane systolic excursion.
Lee CY, Chang SM, Hsiao SH, Tseng JC, Lin SK, Liu CP.
Cardiovascular Center, Department of Internal Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan, R.O.C.

OBJECTIVE: The purpose of this study was to evaluate the use of echocardiographic parameters as predictors of rehospitalization in scleroderma patients. METHODS: Echocardiographic studies were conducted in 38 patients with systolic scleroderma (SSc) to assess cardiopulmonary function. Forty-five age-matched volunteers without any sign of heart failure served as the control group. Transmitral flow pattern, tricuspid annular plane systolic excursion (TAPSE), left ventricular ejection fraction (LVEF), and right ventricular ejection fraction (RVEF) were evaluated. All patients were subsequently followed for one year. RESULTS: Peak transmitral early-diastolic velocity (mitral E) and TAPSE measurements were significantly different between SSc and control patients (mitral E: 74.1 +/- 16.3 vs. 83.5 +/- 17.0 cm/s with P = 0.012; TAPSE: 2.4 +/- 0.43 vs. 1.9 +/- 0.39 cm with P < 0.0001). LVEF was similar, but RVEF was lower in the SSc group (LVEF: 61.7 +/- 9.7 vs. 61.7 +/- 5.8% with P = 0.962; RVEF: 49.6 +/- 6.8 vs. 39.2 +/- 6.7% with P < 0.0001). A strong correlation was found between TAPSE and RVEF. A TAPSE less than 1.96 cm indicted a RVEF less than 40% with a sensitivity of 81% and specificity of 78%. Contrary to expectation, pulmonary artery systolic pressure (PASP) did not correlate well with RV function (r = 0.261, r2= 0.068, P = 0.016). Finally, the frequency of rehospitalization was inversely correlated with RVEF and TAPSE in SSc patients. CONCLUSIONS: We can predict the rehospitalization rate of SSc patients by TAPSE and RVEF, suggesting the involvement of heart, skin, lung, and other organs in scleroderma patients.

Cardiology. 2007 Feb 12;108(4):317-321
Assessment of Atrial Conduction in Patients with Scleroderma by Tissue Doppler Echocardiography and P Wave Dispersion.
Can I, Onat AM, Aytemir K, Akdogan A, Ureten K, Kiraz S, Ertenli I, Ozer N, Tokgozoglu L, Oto A.
Department of Cardiology, Hacettepe University, Ankara, Turkey.

Background: Atrial conduction abnormalities in patients with scleroderma have not been evaluated in terms of P wave duration, P wave dispersion (P(d)) and electromechanical coupling measured by tissue Doppler echocardiography. Methods: Twenty-four patients with scleroderma and 24 control subjects underwent resting electrocardiogram (ECG), M mode and tissue Doppler echocardiography. The P wave duration was calculated in all leads of the surface ECG. The difference between the maximum (P(max)) and minimum P wave duration was calculated and defined as P(d). Interatrial and intraatrial electromechanical delays were measured with tissue Doppler tissue echocardiography. Results: The left ventricular dimensions, fractional shortening, and left atrial diameter did not differ between the patients and the controls. P(d) and P(max) were significantly higher in patients with scleroderma compared with controls: 51 +/- 17 versus 28 +/- 7 ms (p < 0.01) and 109 +/- 10 versus 93 +/- 6 ms (p < 0.01), respectively. There was a delay between the onset of the P wave on surface ECG and the onset of the late diastolic wave (A wave; PA) obtained by tissue Doppler echocardiography in patients with scleroderma compared with controls measured at lateral septal annulus (lateral PA; 122 +/- 8 vs. 105 +/- 7 ms, p = 0.001), septal mitral annulus (104 +/- 11 vs. 93 +/- 10 ms, p = 0.01) and tricuspid annulus (right ventricular PA; 71 +/- 9 vs. 64 +/- 7 ms, p = 0.05). Interatrial conduction time (lateral PA - right ventricular PA) was delayed in patients with scleroderma compared with controls (88 +/- 13 vs. 76 +/- 11 ms, p = 0.01). A positive correlation was detected between interatrial electromechanical delay (lateral PA - right ventricular PA) and P(d) (r = 0.5, p = 0.03). Conclusion: Atrial conduction abnormalities as estimated with P(d) and P(max) are significantly higher in patients with scleroderma compared with controls. There is a delay in both intraatrial and interatrial electromechanical coupling intervals in patients with scleroderma. Copyright (c) 2007 S. Karger AG, Basel.

Presse Med. 2006 Dec;35(12 Pt 2):1938-42.
Heart involvement in systemic sclerosis
Allanore Y, Kahan A.
UniversitÚ RenÚ Descartes, FacultÚ de MÚdicine, Service de Rhumatologie A, H˘pital Cochin, AP-HP, Paris.

Primary myocardial involvement is common in systemic sclerosis. Increasing evidence strongly suggests that this involvement is related to repeated focal ischemic injury causing irreversible myocardial fibrosis. The underlying mechanism appears to be microcirculatory impairment with abnormal vasoreactivity, with or without structural vascular abnormalities. Clinically evident cardiac involvement is recognized to be a poor prognostic factor. Pericardial involvement is frequent but usually asymptomatic. Conduction system abnormalities appear common but not serious, while arrhythmias may be life-threatening. No significant valvular involvement appears to be associated with systemic sclerosis. Treatment for myocardial involvement includes long-term systematic administration of calcium channel blockers and possibly angiotensin-converting enzyme inhibitors, frequently given at high dosage.

Eur J Echocardiogr. 2005 Dec;6(6):407-18. Epub 2005 Apr 7.
Early impairment of myocardial function in systemic sclerosis: non-invasive assessment by Doppler myocardial and strain rate imaging.
D'Andrea A, Stisi S, Bellissimo S, Vigorito F, Scotto di Uccio F, Tozzi N, Moscato F, Pezzullo E, Calabr˛ R, Scherillo M.
Department of Interventional Cardiology, G. Rummo Hospital, Benevento, Italy.

BACKGROUND: Aim of the present study was to analyze both left (LV) and right ventricular (RV) myocardial function in patients with Systemic Sclerosis (SSc), and their relation to other instrumental features of the disease. METHODS AND RESULTS: Twenty-five healthy subjects and 23 age- and sex-comparable asymptomatic patients classified as having either diffuse (11 patients) or limited form (12 patients) of SSc underwent clinical examination, serological tests, high-resolution chest-CT, standard Doppler echo, pulsed Doppler myocardial imaging (DMI) and strain rate imaging (SRI) of both LV and RV lateral walls. By chest-CT, 11 patients showed interstitial pulmonary fibrosis. Serological antibodies analysis detected anti-centromere pattern in 8 patients, and anti Scl-70 in 15 patients. LV diameters and ejection fraction were comparable between the two groups, while RV end-diastolic diameter was increased in SSc (p<0.01). Tricuspid inflow E/A ratio was slightly decreased in SSc (p<0.01), while systolic pulmonary pressure was increased (p<0.001). Pulsed DMI detected in SSc impaired myocardial RV early-diastolic (Em) peak velocity (p<0.0001), and prolonged myocardial time intervals at tricuspid annulus level. In SSc, peak systolic RV SR and strain were both reduced in basal, middle and apical RV lateral free walls, and in basal and middle LV lateral walls. By multivariate analysis, independent inverse association of RV peak Em velocity with both Rodnan Skin Score (p<0.0005) and pulmonary systolic pressure (p<0.0001), as well as independent inverse correlation of the same RV peak Em velocity with pulmonary fibrosis (<0.0005) in SSc patients were observed. In addition, RV Em was an independent predictor of the anti Scl-70 antibody pattern (p<0.001). CONCLUSIONS: Pulsed DMI and SRI are valuable non-invasive and easy-repeatable tools for detecting RV and LV myocardial involvement caused by SSc, and may therefore be useful to early identify patients with more diffused and severe form of SSc.

Eur J Echocardiogr. 2005 Oct;6(5):351-7. Epub 2005 Feb 25.
Myocardial contractility is early affected in systemic sclerosis: a tissue Doppler echocardiography study.
Meune C, Allanore Y, Pascal O, Devaux JY, Dessault O, Duboc D, Weber S, Kahan A.
Department of Cardiology, Cochin Hospital, AP-HP, RenÚ Descartes University, 27 rue du Fg St-Jacques, 75014 Paris, France.

AIMS: Systemic sclerosis (SSc) is a connective tissue disorder characterized by frequent myocardial involvement. Alteration in left ventricular (LV) function is reported to be rare; however, it may be underestimated by conventional measurements. Our aim was to prospectively investigate LV function in SSc patients, using Tissue Doppler echocardiography (TDE), a modern and accurate method of assessing myocardial function. METHODS AND RESULTS: Seventeen consecutive SSc patients with normal cardiac examination, pulmonary artery pressure (PAP) and radionuclide LV ejection fraction (EF) were prospectively investigated. Myocardial perfusion was investigated using single-photon-emission computerized tomography (SPECT). Echocardiography (ECHO), systolic and diastolic strain-rate (SR) measured in the posterior wall by TDE were used to investigate myocardial function, and compared with results of 15 matched controls. All patients (53+/-8 years; 14 women; systolic PAP 33+/-6 mmHg; LVEF 67+/-8%) had myocardial SPECT perfusion abnormalities. Despite normal ECHO, they had lower systolic SR than controls (1.7+/-0.5 versus 3.8+/-1.7 cm-1, p<0.0001), and lower diastolic SR (3.7+/-1.5 versus 5.6+/-1.2 cm-1, p=0.0004). Ten SSc patients had reduced systolic SR<1.7 cm-1 and 11 reduced diastolic SR<3.5 cm-1. CONCLUSION: Frequent abnormal myocardial perfusion is confirmed in SSc patients. Reduced contractility is also frequent as detected by TDE, despite normal radionuclide LVEF.

Semin Arthritis Rheum. 2005 Apr;34(5):721-7.
Echocardiographic alterations in systemic sclerosis: a longitudinal study.
Maione S, Cuomo G, Giunta A, Tanturri de Horatio L, La Montagna G, Manguso F, Alagia I, Valentini G.
Department of Clinical Medicine, Cardiovascular and Immunological Science, University of Naples Federico II, Italy.

OBJECTIVE: To investigate the evolution of cardiac alterations in systemic sclerosis (SSc). METHODS: Echocardiographic and echo-Doppler findings from 77 unselected SSc patients were analyzed at the first clinical observation and after a follow-up period of 65 +/- 36 months. Data were compared with those obtained from 45 normal subjects matched for age and sex. RESULTS: Baseline left ventricular (LV) systolic function was normal in all patients and controls while LV diastolic dysfunction (expressed by an inverted E/A ratio which represents early and late filling of the LV during atrial contraction) was present in 23 patients and in 1 control ( P < 0.001). At the end of the follow-up period, while LV systolic function declined in 1 case alone, 6 further patients developed an inverted E/A ratio. Moreover, in the group of SSc patients mean A-wave values, E/A ratio, left atrial dimension, and LV wall thickness significantly changed, all indicating the progression of heart involvement. The alteration of LV diastolic function was independent of other known causes potentially affecting LV relaxation. Moreover, impairment of LV filling parameters was detected in the first phase of follow-up, while the anatomical changes occurred in the last phase. CONCLUSIONS: Our data confirm the significant prevalence of LV diastolic dysfunction in SSc patients and the role of primary myocardial involvement. The long-term follow-up demonstrates that LV filling dysfunction is progressive and precedes the occurrence of LV remodeling.

Ital Heart J. 2004 Nov;5(11):831-9.
Associations of right ventricular myocardial function with skin and pulmonary involvement in asymptomatic patients with systemic sclerosis. D'Andrea A, Bellissimo S, Scotto di Uccio F, Vigorito F, Moscato F, Tozzi N, Di Donato M, Citro R, Stisi S, Scherillo M.
Department of Interventional Cardiology and Coronary Care Unit, G. Rummo Hospital, Benevento, Italy.

BACKGROUND: Systemic sclerosis (SSc) is a multisystem disorder characterized by widespread vascular lesions and fibrosis of the skin and specific internal organs. Cardiac involvement is a common finding in SSc, but often clinically occult. The aim of the present study was to analyze both left and right ventricular (RV) myocardial function in patients with SSc, and their relation to other instrumental features of the disease. METHODS: Twenty-five healthy subjects and 23 age- and sex-comparable asymptomatic patients classified as having either diffuse (11 patients) or limited cutaneous (12 patients) SSc underwent clinical examination, serological analysis, high-resolution chest computed tomography, standard Doppler echocardiography and pulsed Doppler myocardial imaging (DMI) of both the mitral and tricuspid annuli. SSc was classified using the modified Rodnan skin score (mRSS) into high mRSS (score > or = 10) and low mRSS (score < 10). RESULTS: Serological antibody analysis revealed the presence of antinuclear antibody in all patients, an anticentromere pattern in 8 patients, and anti-Scl-70 antibodies in 15 patients. Eleven patients were diagnosed with interstitial pulmonary fibrosis at chest computed tomography. Standard Doppler echocardiography revealed that the left ventricular mass index and ejection fraction were comparable between the two groups, while the RV end-diastolic diameter was increased in SSc (p < 0.01). The tricuspid inflow peak E and E/A ratio were slightly decreased in SSc (p < 0.01), while the systolic pulmonary pressure was increased (p < 0.0001). DMI analysis revealed, in SSc, an impaired RV myocardial early-diastolic (Em) peak velocity (p < 0.001) as well as a prolonged myocardial relaxation time (RTm) (p < 0.001) only at the tricuspid annulus level, even after correction for age, sex, heart rate and left ventricular mass index. Independent inverse associations of the RV Em peak velocity with both the Rodnan skin score (beta coefficient = -0.62, p < 0.0005) and the pulmonary systolic pressure (beta coefficient = 0.71, p < 0.0001), as well as the independent inverse correlation of the same RV Em peak velocity with interstitial pulmonary fibrosis (odds ratio 0.68, 95% confidence interval 0.45-0.83, p < 0.0005) in SSc patients were assessed at multivariate analysis. In addition, the RV Em velocity was an independent predictor of the anti-Scl-70 antibody pattern (odds ratio 0.68, 95% confidence interval 0.45-0.83, p < 0.01). Of note, a RV Em peak velocity < 0.11 m/s well selected SSc patients with pulmonary artery pressure > 35 mmHg, pulmonary fibrosis, a high mRSS, and an anti-Scl-70 antibody pattern. CONCLUSIONS: The relationships of RV myocardial diastolic dysfunction with both skin and pulmonary involvement as well as with the serological antibody pattern emphasizes the ability of DMI to identify patients with a more diffuse and severe form of SSc. This issue may be critical for the early identification of those SSc patients who are at higher risk of cardiac impairment, ideally when they are still asymptomatic before developing severe vasculopathy.

Curr Rheumatol Rep. 2004 Apr;6(2):137-40.
The heart in systemic sclerosis.
Steen V.
Georgetown University, 3800 Reservoir Road, LL Gorman, Washington, DC 20007, USA.

The heart is one of the major organs involved in scleroderma. Cardiac involvement can be manifested by myocardial disease, conduction system abnormalities, arrhythmias, or pericardial disease. Additionally, scleroderma renal crisis and pulmonary hypertension lead to significant cardiac dysfunction secondary to damage in the kidney and lung. This report summarizes the recent advances to further understand the types and mechanism of abnormalities in the heart in scleroderma. New cardiac technology shows significant frequencies of asymptomatic cardiac abnormalities. Further long-term studies are necessary to determine the outcome and the best approach to treatment of such abnormalities. Diastolic dysfunction has been carefully evaluated in scleroderma in recent years and appears to be more common than once realized. There is controversy as to whether this is a significant finding independent to other cardiopulmonary problems. More extensive evaluation of the conduction and the arrhythmia ablative therapy has helped manage these life-threatening complications.

Przegl Lek. 2000;57(7-8):389-92. Evaluation of early cardiovascular involvement in patients with systemic sclerosis
Wranicz JK, Strzondala M, Zielinska M, Cygankiewicz I, Ruta J, Dziankowska-Bartkowiak B, Sysa-Jedrzejowska A.
Klinika Kardiologii Instytutu Kardiologii, Akademii Medycznej w Lodzi.

The aim of the study was to evaluate cardiac function in patients with systemic sclerosis by means of noninvasive methods in order to detect early dysfunction of cardiovascular system. MATERIAL AND METHODS: We studied a group of 19 patients (15 women, 4 men, aged 17-74 yrs, av. 51 +/- 11) with systemic sclerosis comparing the results with a group of 23 healthy volunteers (17 women, 6 men aged 21-69 yrs, av 53 +/- 15). All the patients with SSc were taking corticosteroids, immunosuppressants or vasodilators at the time of the study. In all the patients we performed 24-hour Holter monitoring for the evaluation of arrhythmias, conduction disturbances, ischaemia, heart rate variability (HRV) and late potentials (LP). The following parameters of HRV in time domain were analyzed: SDNN, SDANN, SDNNI, rMSSD, pNN50. Standard ECG was performed to assess QT interval (QT, QTc, QTd). In all the patients the echocardiography examination was performed (M-Mode, 2-D, Doppler echocardiography). The morphology of heart structures and haemodynamic function were analyzed. RESULTS: In patients with SSc Holter monitoring revealed tendency to tachycardia. The mean heart rate was 81 +/- 11 vs. 71 +/- 9 in controls. Conduction disturbances were observed in 3 pts. In 6 pts we found significant ventricular arrhythmia. Silent ischaemia episodes were detected in 6 pts. Concerning HRV analysis the significantly lower values were detected in pts with SSc vs. controls: SDNN 123 vs 170; SDNNI 51 vs 76; SDANN 110 vs 152; rMSSD 29.6 vs 54; pNN50 6.1 vs 21. Late potentials were present in one patient with SSc vs none in the control group. The mean values QT-371, QTc-419, QTd-40- did not exceed the ranges of normal values. No signs of systolic cardiac dysfunction were detected, while in 6 pts we recognized left ventricle diastolic dysfunction. Valvular lesions were observed in 8 pts, but only in 2 pts they were hemodynamically important. CONCLUSIONS: 24-hour Holter monitoring and ECHO examination are valuable methods, which allow to detect early dysfunction of cardiovascular system in patients with systemic scleroderma presenting no apparent cardiac impairment symptoms.

Ann Rheum Dis. 1998 May;57(5):296-302.
Heart involvement in systemic sclerosis: an ultrasonic tissue characterisation study.
Ferri C, Di Bello V, Martini A, Giorgi D, Storino FA, Bianchi M, Bertini A, Paterni M, Giusti C, Pasero G.
Department of Internal Medicine, University of Pisa, Italy.

BACKGROUND: Clinicoepidemiological findings indicate that symptomatic heart involvement in patients with systemic sclerosis (SSc) predicts a very poor prognosis. At necropsy studies, SSc heart involvement without significant coronary lesions is characterised by patchy myocyte necrosis and contraction band necrosis with collagen replacement leading to myocardial fibrosis. There is a discrepancy between the frequency of clinically evident myocardial disease (25%) and autoptical myocardial fibrosis (81%). OBJECTIVE: The aim of this study was to detect preclinical myocardial alterations in SSc patients by ultrasonic videodensitometric analysis. METHODS: Thirty five SSc patients (three male, aged 48.6 (11) SD years, range 22-65) with normal ventricular function and 25 age and sex matched healthy controls were studied. All patients had a negative maximal exercise stress; in all cases arterial hypertension, renal involvement, and diabetes were excluded. Echocardiographic images were digitised by a real time videodigitiser (Tomtec Imaging Systems). Quantitative texture analysis was performed on data from the septum and the posterior wall, obtaining mean gray level histogram (MGL) at both end-diastole (d) and end-systole (s). The cyclic variation index (CVI), was calculated according to the formula ((MGLd-MGLs)/MGLd) x 100. Left ventricular mass (LVM), body surface corrected, was calculated according to Penn convention. RESULTS: Comparable systolic and diastolic blood pressure, LVM, diastolic and systolic function were recorded in both SSc patients and controls. In contrast, in SSc patients the CVI, which is the expression of the intrinsic myocardial structural function, was significantly lower than in controls (septum: -18 (28)% v 35 (10)%, p < 0.0001; and posterior wall: -13 (32)% v 50 (20)%, p < 0.0001). Changes in cyclic echo amplitude, probably related to myocardial fibrosis, were detected in the large majority of SSc patients (88%). CONCLUSIONS: Ultrasonic videodensitometric analysis represents a non-invasive, feasible method that can detect early myocardial changes in SSc patients, which could be related to both fibrosis and microcirculatory abnormalities. Their potential evolution towards ventricular dysfunction and their link with cardiac sudden death, because of severe conduction system or rhythm disturbances, should be further investigated.

Eur Heart J. 1997 Dec;18(12):1995-2001.
Atrial conduction abnormalities in patients with systemic progressive sclerosis.
Mizuno R, Fujimoto S, Nakano H, Nakajima T, Kimura A, Nakagawa Y, Dohi K.
First Department of Internal Medicine, Nara Medical University, Japan.

BACKGROUND: Atrial abnormalities in patients with progressive systemic sclerosis have not been evaluated in terms of intra-atrial conduction. We hypothesized that a delay in atrial conduction in these patients might produce diastolic abnormalities as well as atrial arrhythmias. OBJECTIVE: To evaluate the atrial function of patients with progressive systemic sclerosis by using echocardiography to measure the intra-atrial electromechanical activation coupling interval. METHODS: Twenty patients with progressive systemic sclerosis were assessed by Doppler echocardiography. Twenty age-matched healthy controls were also evaluated. Two-dimensional guided M-modes of ventricular long axes were recorded using simultaneous phono- and electrocardiograms of the apical four chamber view at the right lateral, septal and left lateral sites of the atrioventricular rings. Transmitral and tricuspid pulsed Doppler flow velocities were also recorded. Filtered P wave duration was measured on the signal averaged ECG to determine the duration of atrial electrical activation. RESULTS: There was a delay in P on the electrocardiogram (P) at the onset of atrial contraction on long axis M-modes at all three atrioventricular ring sites in patients with progressive systemic sclerosis as compared with controls (P-right; 56 +/- 13 vs 47 +/- 10 ms, P-septal; 74 +/- 14 vs 55 +/- 10 ms, and P-lateral; 93 +/- 16 vs 72 +/- 11 ms, P < 0.01). Inter-atrial conduction time [(P-lateral)-(P-right)] was delayed in patients with progressive systemic sclerosis, compared with healthy controls (37 +/- 15 vs 25 +/- 6 ms, P < 0.01). Mitral A waves acceleration and deceleration times were also decreased in the patients. The interval was prolonged between P to the onset and the peak of the A wave in transmitral flow. Duration of the filtered P wave was significantly prolonged in progressive systemic sclerosis as compared with controls (124 +/- 12 ms vs 106 +/- 8 ms, P < 0.01). PQ intervals, E waves and acceleration and deceleration times did not differ significantly in progressive systemic sclerosis vs, controls. The A wave acceleration rate on transmitral flow (peak A wave velocity/acceleration time) showed a significant correlation with inter-atrial conduction delay (r = 0.55, P < 0.01). CONCLUSIONS: Intra-atrial electromechanical coupling intervals were delayed in patients with progressive systemic sclerosis. Thus, the mechanical late diastolic filling time due to atrial contraction in the total diastolic phase was severely limited, and this resulted in a restricted mitral A wave. We should therefore evaluate patients with progressive systemic sclerosis for significant atrial abnormalities.

Int J Cardiol. 1996 Dec 6;57(2):151-60.
Relationships between electrocardiographic and echocardiographic findings in systemic sclerosis (scleroderma).
Morelli S, Sgreccia A, Ferrante L, Barbieri C, Bernardo ML, Perrone C, De Marzio P.
Istituto di Clinica Medica I, University La Sapienza, Rome, Italy.

We assessed the prevalence of electrocardiographic abnormalities in patients with systemic sclerosis and evaluated their functional significance through a comparison with echocardiographic findings. Seventy-two patients with systemic sclerosis and 64 controls underwent resting electrocardiogram (ECG) and M-mode, two-dimensional, Doppler and color Doppler echocardiography. Electrocardiographic abnormalities were observed in 48.7% of patients. Conduction disturbances (27.7%) infarction pattern (13.8%), non-specific ST-T wave changes (13.8%) and right ventricular hypertrophy (11.1%) were the most frequent abnormalities. QTc interval was significantly longer in patients with systemic sclerosis than in controls. Significant differences between patients and controls were found in the prevalence of long QTc interval (p = 0.0016) infarction pattern (p = 0.0016), right ventricular hypertrophy (p = 0.007) and non-specific ST-T wave abnormalities (p = 0.0016). All patients with infarction pattern and 90% of patients with prolonged QTc interval had some echocardiographic abnormalities. Electrocardiographic signs of right ventricular hypertrophy were 16% sensitive and 93% specific for pulmonary hypertension; the sensitivity and specificity of the combination of right ventricular hypertrophy, right atrial enlargement and right bundle branch block were 35% and 90%, respectively. Standard ECG is useful to assess cardiac involvement in patients with systemic sclerosis. If infarction pattern, right ventricular hypertrophy or long QTc interval are present, a cardiac involvement is very likely.

Rheum Dis Clin North Am. 1996 Nov;22(4):841-60.
Cardiac involvement in scleroderma.
Deswal A, Follansbee WP.
University of Pittsburgh School of Medicine, Pennsylvania, USA.

In summary, cardiac involvement in systemic sclerosis can be manifested as myocardial disease, pericardial disease, conduction system disease, or arrhythmias. Clinical cardiac involvement is a poor prognostic factor. Asymptomatic cardiac abnormalities are frequent, and all cardiac abnormalities are seen more often in diffuse scleroderma. Unlike other organs, the role of vascular involvement is unclear. At present, treatment of cardiac scleroderma is essentially symptomatic and empiric. The role of vasodilation and immunosuppression needs further exploration.

Recenti Prog Med. 1996 Nov;87(11):557-63.
Cardiopathy in systemic sclerosis
Valentini G, Maione S.
Cartedra di Reumatologia, Seconda UniversitÓ, Napoli.

Cardiac involvement is quite frequent in systemic sclerosis (SSc). From a pathophysiologic point of view, one must differentiate a primary scleroderma heart disease due to pericardial and/or myocardial and/or small coronary intramyocardial vessel involvement from heart disease secondary to either pulmonary interstitial or vascular involvement (pulmonal cor) or to kidney disease (hypertensive myocardial disease). A significant difference emerges when the prevalence of clinically and standard ECG detected cardiac involvement in SSc patients is compared with that registered at autopsy. In the last years, however, Holter ECG, echocardiography, perfusional scintigraphy and ventriculography have reduced such gap, a preclinical scleroderma heart disease being detected by such techniques in quite a high percentage of SSc patients. Asymptomatic SSc patients may be found to present small pericardial effusions and/or either fixed (fibrosis) or reversible (vascular disease) or both types thallium defects or a defective cardiac functional reserve. Both clinically evident scleroderma heart disease and ventricular arrhythmias have a poor prognostic significance. Therefore, a complete cardiological work-up must be periodically carried out in SSc patients. Scleroderma heart disease has long been considered a condition difficult to treat. The detection of diastolic abnormalities and of diastolic failure in SSc patients make us able to understand the therapeutic failure of inotropic agents. Recently, captopril has been shown to improve cardiac function in SSc. It might act either on vascular disease or on fibrosis by affecting the remodelling process of the myocardial wall.

Ann Rheum Dis. 1996 Jul;55(7):455-60.
Diastolic abnormalities in systemic sclerosis: evidence for associated defective cardiac functional reserve.
Valentini G, Vitale DF, Giunta A, Maione S, Gerundo G, Arnese M, Tirri E, Pelaggi N, Giacummo A, Tirri G, Condorelli M.
Institute of Clinical Medicine, Division of Rheumatology, Second University of Naples, Italy.

OBJECTIVE: To investigate the pattern of diastolic abnormalities in patients with systemic sclerosis (SSc) and the relationship between impaired ventricular filling and systolic function. METHODS: Twenty four patients with SSc underwent M-mode and two dimensional echocardiography using echo-Doppler and gated blood pool cardiac angiography, both at rest and after exercise. RESULTS: An impaired diastolic relaxation of the left ventricle was detected in 10 of the 24 patients with SSc. Left ventricular ejection fraction at rest in these 10 patients with impaired ventricular filling did not differ from that in the remaining 14 patients, but eight of the 10 failed to increase their ejection fraction during exercise, compared with two of the 14 with normal ventricular filling (p = 0.003). CONCLUSION: Impaired relaxation of the left ventricle is a recently described feature of scleroderma heart disease. Diastolic dysfunction in SSc could depend on myocardial fibrosis or myocardial ischaemia, or both. It was found to be associated with a defective cardiac functional reserve. However, its prognostic significance remains to be clarified.

Semin Arthritis Rheum. 1989 Dec;19(3):191-200.
Heart disease in systemic sclerosis.
Janosik DL, Osborn TG, Moore TL, Shah DG, Kenney RG, Zuckner J.
Division of Rheumatology, St. Louis University School of Medicine, MO 63104.

Primary cardiovascular manifestations of SSc include pericardial disease, myocardial disease, conduction abnormalities, and cardiac arrhythmias. Significant cardiac abnormalities are present in more than half of SSc patients at autopsy. As the frequency of subclinical cardiac involvement is now appreciated and noninvasive cardiac diagnostic modalities continue to improve, the ability to detect early asymptomatic involvement in SSc has improved. Two-dimensional echocardiography, radionucleotide imaging, and ambulatory ECG allow recurrent serial testing with virtually no morbidity. The current treatment of cardiac involvement in SSc is emperic and primarily directed at symptomatology. Large prospective randomized trials are needed to determine if preventive therapy is effective. With the advent of new immunological and cardiotropic agents and a better understanding of the primary disease process, our ability to alter the pathogenesis and final outcome of cardiac involvement in SSc should improve.

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