Right Heart Function in Scleroderma: Insights from Myocardial Doppler Tissue Imaging

doi:10.1016/j.echo.2005.12.003

Journal of the American Society of Echocardiography

Volume 19, Issue 5, May 2006, Pages 507-514

https://doi.org/10.1016/j.echo.2005.12.003 Get rights and content

To use Doppler tissue imaging to evaluate heart function and to predict rehospitalization rate in progressive systemic sclerosis, we studied 40 patients (limited in 24 patients, diffuse in 16 patients) with chest roentgenography, pulmonary function test, routine echocardiography, and myocardial Doppler tissue. Another 45 volunteers without any sign of heart failure served as the control group. Significant difference of echocardiographic parameters was found in peak transmitral early diastolic velocity, right ventricular (RV) ejection fraction (EF) (RVEF), pulmonary artery systolic pressure, and Doppler tissue parameters of the RV and septum (peak transmitral early diastolic velocity, P = .012; RVEF, P < .0001; pulmonary artery systolic pressure, P < .0001). The parameters derived by pulsed wave Doppler tissue decreased in RV, including peak systolic myocardial velocity (Sm), early diastolic velocity, late diastolic velocity, and myocardial performance index. RVEF and left ventricular EF were estimated by Simpson’s method. RV-Sm could be used to identify RV failure. Receiver operating characteristic area under the curve for RV-Sm was 0.935. RV-Sm less than 11 cm/s indicted RVEF less than 40% with sensitivity of 87% and specificity of 86%. Contrary to expectation, pulmonary artery systolic pressure was not so well correlated with RV function. The frequency of admission was reverse correlated with decrement of RV-Sm in patients with RV-Sm less than 12 cm/s. We conclude that in progressive systemic sclerosis, RV systolic dysfunction is common and appears to be a result of pulmonary hypertension, disturbance of myocardial microcirculation, and myocardial fibrosis. Pulmonary hypertension was not well correlated with RV dysfunction; it suggested pulmonary hypertension was not the only cause of RV failure. Primary right heart involvement was the other possible cause. By myocardial Doppler tissue imaging, we can predict the frequency of hospitalization; it suggests simultaneous involvement of heart, skin, lung, and other organs. RV-Sm more than 12 cm/s predicted a decreased likelihood of readmission to the hospital.

Section snippets

Study Population

From April to July 2004, 40 patients classified as having either diffuse (16 patients) or limited (24 patients) forms of SSc and 45 age- and sex-matched healthy volunteers were enrolled into the study with informed consent. Each individual in the study population was confirmed as having SSc using the criteria for SSc defined by the American College of Rheumatology.³³ All patients were selected after exclusion of (1) moderate or severe left-sided valvular disease, (2) dilated cardiomyopathy, (3)

Results

The study and control groups were comparable regarding age, sex, and heart rate. Using standard echocardiographic analysis, we determined that the differences in chamber size and LVEF between the two groups were statistically insignificant. The early diastolic peak velocity in patients with SSc was significantly higher than in the control group. PASP was also higher in the SSc group than in the control group (P < .0001). RVEF was lower in patients with SSc than in control subjects (P < .0001).

Discussion

The prognosis of SSc has been reported to mainly correlate with pulmonary hypertension,27, 28, 29, 30 but heart involvement is another prognostic factor for poor outcome.1, 2, 3, 31 This is the reason many investigators have looked for heart involvement in patients with SSc. However, almost all of the previous studies focused on the LV and overlooked RV function. Recent advances in echocardiography now allow us to easily assess LV and RV function using DTI parameters.

Radionuclide studies

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Cited by (58)

Defining primary systemic sclerosis heart involvement: A scoping literature review
2019, Seminars in Arthritis and Rheumatism
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.
Systemic Sclerosis
2017, The Heart in Rheumatic, Autoimmune and Inflammatory Diseases: Pathophysiology, Clinical Aspects and Therapeutic Approaches
Cardiac involvement in systemic sclerosis (SSc) may be either primary or secondary to the impairment of other organs such as the lungs and kidneys. Primary cardiac involvement, which develops as a direct consequence of the disease, may affect all cardiac tunics, endocardium, myocardium, and pericardium. This may conduct to pericardial effusion, arrhythmias, conduction system defects, myocardial ischemia, myocardial hypertrophy, and heart failure. The prevalence of primary cardiac involvement in SSc varies depending on the sensitivity of the diagnostic tools used, but it is ascertained that overt cardiac manifestations are associated with poor prognosis. Microvascular impairment is a pathognomonic feature of SSc, and together with focal fibrosis may involve all cardiac structures. In contrast, the frequency of atherosclerotic coronary artery disease in SSc seems to be comparable to that of the general population. Because cardiac involvement in SSc can be often clinically obscure, a complete routine evaluation should be adopted in every patient, including standard 12-lead electrocardiogram (ECG) and Doppler echocardiography (especially tissue Doppler imaging), while magnetic resonance imaging and cardiac computed tomography are sensitive techniques for earlier detection of both structural and functional scleroderma-related cardiac pathologies, although they still represent a second level examination, not available everywhere. Additional investigations can be required in suspicion of other manifestations such as conduction and rhythm disorders (24-h Holter monitoring, invasive electrophysiological studies), or myocarditis (cardiac magnetic resonance). Routine screening for subclinical cardiac involvement with more sensitive tools leads to early diagnosis, in order to treat patients in a “window of opportunity” with drugs that may improve myocardial perfusion and function, and possibly limit the progression of the most life-threatening complications.
Pseudo-infarction pattern in diffuse systemic sclerosis. Evaluation using cardiovascular magnetic resonance
2016, International Journal of Cardiology
Diffuse systemic sclerosis (dSSc) is characterized by vascular lesions and fibrosis. Cardiac involvement, although silent, accounts for 36% of deaths. We hypothesized that cardiovascular magnetic resonance (CMR) can clarify the pathophysiology of Q waves in dSSc patients.
105 dSSc, aged 48 ± 2 years, with atypical symptoms and normal routine assessment, were evaluated by ECG and CMR using a 1.5 T system. Biventricular function was assessed by steady-state free-precession sequence (SSFP). To identify fibrosis, late gadolinium enhanced areas (LGE) were evaluated 15 min after injection of 0.2 mmol/kg gadolinium-DTPA and expressed as % of LV mass.
Q waves in V1–V5 (Group A), II, III, AVF (Group B) and I, AVL, II, III, AVF, V1–V5 (Group C) were found in 25/105, 8/105 and 5/105 dSSc, respectively. In 25 dSSc with Q in V1–V6, patchy intramyocardial LGE was detected in 24/25 and involved 8 ± 2% of LV mass. LGE involved the intraventricular septum (IVS) in 11/24 and the lateral wall (LAT) in 5/24 dSSc. Only in 1/25 dSSc, an anterior, transmural LGE, due to LAD occlusion, was identified. In 8 dSSc with Q in II, III, AVF, patchy intramyocardial LGE was detected in the inferior wall and involved 5 ± 2% of LV mass. In 5 dSSc with Q in V1–V5, II, III, AVF, patchy intramyocardial LGE was detected in anterior and inferolateral wall and involved 9 ± 2% of LV mass.
CMR unveiled that the pattern of myocardial fibrosis in dSSc with Q waves is due to the systemic disease and not to CAD.
A right ventricular diastolic impairment is common in systemic sclerosis and is associated with other target-organ damage
2016, Seminars in Arthritis and Rheumatism
Citation Excerpt :
In addition, secondary heart disease involvement due to severe internal organ damage, such as pulmonary arterial hypertension (PAH), severe interstitial lung disease or renal crisis, can occur [1,4,7–9]. Theoretically, the right ventricle (RV) might be affected very early in the course of the disease [10] as it might be involved in both primary myocardial involvement and/or lung vascular or lung interstitial disease, which are common in SSc [10–12]. While several studies reported possible RV alterations in SSc patients PAH [13–16], only a few, small series investigated RV function in unselected SSc patients [4,17–21].
Heart involvement in systemic sclerosis (SSc) is a strong prognostic factor. Our aim was to examine left ventricle (LV) and right ventricle (RV) involvement.
We examined LV and RV, systolic and diastolic functions, using echocardiography and Tissue-Doppler echocardiography (TDE) indexes, in a cohort of 212 consecutive SSc patients seen during a 9-month period at 2 institutions (Paris, France and Los Angeles, USA). They were compared to 50 healthy controls.
When compared to controls, SSc patients had consistently impaired RV indices that include reduced RV contractility (p < 0.001), larger right atrial area (p = 0.027) and overall RV diastolic dysfunction (25% of SSc patients versus 0% of controls; p < 0.001). Patients also exhibited alterations in LV contractility and diastolic function (p < 0.001 each). In multivariate analysis, RV contractility as expressed by the TDE S_T parameter was associated with _TDE LV contractility S_M (p = 0.030), DLCO (p = 0.013) whereas RV diastolic impairment was associated with systolic pulmonary artery pressure (p = 0.015). A subgroup of 27 patients had proven pulmonary arterial hypertension (PAH); comparison between SSc-PAH versus SSc free of PAH patients revealed reduced LV diastolic function (transmitral E/A ratio, p = 0.045 and E_A < 10 cm/s, p = 0.029), reduced overall RV contractility (21.5% versus 4.5%; p = 0.03) and reduced RV diastolic function (transtricuspid E/A ratio; p = 0.014 and 68% versus 29% with impaired function; p = 0.001).
Our data show that RV is commonly affected in SSc with predominant impaired diastolic function. Several factors, including primary heart, lung vascular disease and pulmonary hypertension, contribute to such impairment.
Right ventricular thickness as predictor of global myocardial performance in systemic sclerosis: A Doppler tissue imaging study
2015, Indian Heart Journal
Citation Excerpt :
RV systolic dysfunction occurs late in the natural course of disease only when disease progresses to involve circular muscle fibers of myocardium. Though RV systolic dysfunction is not very well documented in older literature,19–21 Hsiao et al.22 found significant RV systolic dysfunction (RVEF < 40%) in 55% of 40 patients studied. We could also demonstrate significant LV diastolic dysfunction in our patients.
Cardiopulmonary involvement in systemic sclerosis (SSc) is a poor prognostic factor, due to pulmonary hypertension and right ventricular dysfunction. We assessed the echocardiographic parameters of right ventricular (RV) function in SSc and correlated echocardiographic findings to clinical features of the disease.
Thirty patients with SSc (cases) and 30 healthy, age-matched subjects (controls) were studied. Echocardiography, including tissue Doppler imaging, was used to evaluate cardiac function.
Pulmonary hypertension could be documented in only 5 cases by Doppler echo, using Bernoulli principle. RV diastolic function was significantly deranged in cases. RV systolic function and left ventricle (LV) diastolic function were also significantly deranged in the cases. RV thickness was increased in patients with SSc. There were no significant differences in the echocardiographic variables between diffuse and limited subtypes of SSc. Myocardial performance index (MPI) of both ventricles were increased in cases. We could demonstrate RV thickness as the single most important predictor of MPI of both ventricles with sensitivity of 82% and specificity of 72% for RV-MPI and 63% for LV-MPI. Diastolic function was not found to be affected by disease duration or Rodnan skin score.
Patients with SSc exhibit abnormal RV and LV diastolic functions as well as abnormal RV systolic function. RV wall thickness was found to be simple and the single best predictor of global myocardial performance. RV dysfunction may be a response to intermittent pulmonary arterial hypertension, lung parenchymal involvement, or secondary to LV diastolic dysfunction in SSc.
Myocardial perfusion-fibrosis pattern in systemic sclerosis assessed by cardiac magnetic resonance
2012, International Journal of Cardiology

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Original articleRight Heart Function in Scleroderma: Insights from Myocardial Doppler Tissue Imaging

Section snippets

Study Population

Results

Discussion

Am J Med

Am J Cardiol

J Am Coll Cardiol

J Am Soc Echocardiogr

J Am Coll Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Soc Echocardiogr

J Am Soc Echocardiogr

J Am Soc Echocardiogr

J Am Soc Echocardiogr

J Am Soc Echocardiogr

J Am Soc Echocardiogr

J Am Soc Echocardiogr

Am J Cardiol

J Am Soc Echocardiogr

Am J Cardiol

J Am Soc Echocardiogr

Am Heart J

Original article
Right Heart Function in Scleroderma: Insights from Myocardial Doppler Tissue Imaging