Isolated right ventricular dysfunction in systemic sclerosis: latent pulmonary hypertension? (original) (raw)
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Annals of the Rheumatic Diseases, 2000
Objectives-To investigate right ventricular diastolic function in systemic sclerosis (SSc) and its relation to clinical features of the disease. Methods-Seventy seven unselected SSc patients and 33 healthy subjects were submitted to echocardiography and echo Doppler study to assess left and right systolic as well diastolic function and to estimate maximal arterial systolic pulmonary pressure (PAP). In addition, the patients were investigated to define the SSc subset and the extent of skin and internal organ involvement. Results-An abnormal right ventricular filling, as expressed by an inverted tricuspidal (Tr) E/A ratio (Tr E/A ratio <1), was detected in 31 of the 77 SSc patients (40%) and in 0 of the 36 controls (p<0.001). All the 31 patients with an inverted Tr E/A ratio were found to have a PAP > 30 mm Hg. Twenty resulted to have an inverted mitral (Mit) E/A ratio (Mit E/A ratio <1), indicating an abnormal left ventricular filling. In multiple regression analysis, Tr E/A ratio resulted to be independently correlated to both PAP (r= −0.35;p<0.003) and Mit E/A ratio (r=0.39;p<0.001). Conclusions-This study points out an impaired right ventricular filling in a significant percentage of SSc patients whatever the subset. This alteration is independently correlated to both PAP and left ventricular filling abnormalities.
Disturbed Right Ventricular Diastolic Function in Patients With Systemic Sclerosis *
CHEST Journal, 2005
Background: Cardiopulmonary involvement in patients with systemic sclerosis (SSc) carries a poor prognosis, mainly due to pulmonary hypertension and right-heart failure. To date, right ventricular (RV) involvement has not been studied in detail. We therefore assessed RV function in patients with SSc and related the findings to the clinical features of the disease. Method: Twenty-six consecutive patients (21 women) with SSc (mean age, 56 ؎ 15 years [؎ SD]) and 25 healthy, age-matched control subjects (21 women) were studied. Doppler echocardiography including Doppler tissue imaging was used to evaluate cardiac function. Pulmonary function was also studied. Results: Compared with control subjects, RV free wall thickness (5.8 ؎ 1.7 mm vs 3.7 ؎ 1.1 mm, p < 0.001) and right atrial (RA) systolic area (15.9 ؎ 3.7 cm 2 vs 13.0 ؎ 2.3 cm 2 , p < 0.01) were increased in patients with SSc, while the global early diastolic/atrial component velocity ratio was reduced (1.2 ؎ 0.4 vs 1.7 ؎ 0.6, p < 0.01). The global isovolumic relaxation time (IVRT) [64 ؎ 23 ms vs 39 ؎ 13 ms, p < 0.001] and regional IVRT (83 ؎ 40 ms vs 46 ؎ 24 ms, p < 0.001) were prolonged in patients vs control subjects, whereas the RV global filling time was reduced (454 ؎ 122 ms vs 548 ؎ 104 ms, p < 0.01). RV systolic function and pulmonary pressures at rest were similar in the two groups, but the pulmonary artery acceleration time was reduced (119 ؎ 34 ms vs 141 ؎ 29 ms, p < 0.05) in patients compared to control subjects. Left ventricular function did not differ between the two groups. Conclusion: Patients with SSc exhibit altered RV diastolic function together with an increase in RV wall thickness and RA area. These findings appear to be early markers of RV disturbance, probably in response to intermittent pulmonary arterial hypertension.
European Respiratory Journal, 2008
Since systemic sclerosis (SSc) also involves the heart, the aim of the present study was to evaluate possible differences in right ventricular (RV) pump function between SScassociated pulmonary arterial hypertension (PAH; SScPAH) and idiopathic PAH (IPAH). In 13 limited cutaneous SScPAH and 17 IPAH patients, RV pump function was described using the pump function graph, which relates mean RV pressure (PRV) and stroke volume index (SVI). Differences in pump function result in shift or rotation of the pump function graph. PRV and SVI were measured using standard catheterisation. The hypothetical isovolumic PRV (PRV,iso) was estimated using a single-beat method. The pump function graph was approximated by a parabola: PRV5PRV,iso[1-(SVI/SVImax) 2 ], where SVImax is the hypothetical maximal SVI at zero PRV, enabling calculation of SVImax. There were no differences in SVI and SVImax. Both PRV and PRV,iso were significantly lower in SScPAH than in IPAH (PRV 30.7¡8.5 versus 41.2¡9.4 mmHg; PRV,iso 43.1¡12.4 versus 53.5¡10.0 mmHg). Since higher pressures were found at similar SVI, the difference in the pump function graph results from lower contractility in SScPAH than in IPAH. Right ventricular contractility is lower in systemic sclerosis-associated pulmonary arterial hypertension than in idiopathic pulmonary arterial hypertension.
Kardiologia polska, 2011
In systemic sclerosis (SSc), changes in the lungs and pulmonary hypertension (PH) are complications most adversely affecting the prognosis. Given the availability of specific treatment, early diagnosis of PH is very important. Exercise echocardiography, by increasing the patient's cardiac output, makes it possible to identify patients with elevated pulmonary artery pressure (PAP) during exercise. The diagnostic role of exercise echocardiography is still unclear, mainly because of the lack of prospective studies. To identify SSc patients with abnormally elevated PAP at rest or with a significant increase PAP during exercise, subsequently verified by right heart catheterisation (RHC). A total of 71 consecutive patients (67 females and 4 males, mean age 56.9 ± 17.1 years) with SSc diagnosed according to the American College of Rheumatology criteria were enrolled in this prospective study. The patients underwent transthoracic echocardiography (Philips iE33) with the measurement of t...
The Journal of rheumatology, 2008
Pulmonary hypertension (PH) is an ominous complication in patients with scleroderma (systemic sclerosis, SSc). We compared noninvasive assessment of PH with pulmonary artery (PA) pressures obtained by right-heart catheterization (RHC). Forty-nine patients with SSc were evaluated for suspected PH based on clinical findings, progressive dyspnea, and pulmonary function tests (PFT). PH was defined as mean PA pressure > or = 25 mm Hg, or > or = 30 mm Hg after exercise, with normal pulmonary capillary wedge pressure (PCW). Doppler echocardiography (echo) and cardiac magnetic resonance imaging (MRI) were performed within 4 hours of RHC, and the predictive accuracy of the tests was compared. RHC identified 24/49 (49%) patients with PH. The noninvasive cutpoints were: estimated right ventricular systolic pressure > 47 mm Hg by echo; diameter of the main PA > 28 mm by MRI; and the ratio of forced vital capacity to diffusion capacity (%FVC/%DLCO) > 2.0 by PFT. Echo classified 38...
Arthritis research & therapy, 2016
Pulmonary arterial hypertension (PAH) complicates the course of systemic sclerosis (SSc) and is associated with poor prognosis. The elevation of systolic pulmonary arterial pressure (sPAP) during exercise in patients with SSc with normal resting haemodynamics may anticipate the development of PAH. Exercise echocardiography (ExEcho) has been proposed as a useful technique to identify exercise-induced increases in sPAP, but it is unclear how to clinically interpret these findings. In this systematic review, we summarize the available evidence on the role of exercise echocardiography to estimate exercise-induced elevations in pulmonary and left heart filling pressures in patients with systemic sclerosis. We conducted a systematic review of the literature using MEDLINE, Cochrane Library and Web of Knowledge, using the vocabulary terms: ('systemic sclerosis' OR 'scleroderma') AND ('exercise echocardiography') AND ('pulmonary hypertension'). Studies includi...
Annals of the American Thoracic Society, 2017
Patients with scleroderma associated pulmonary arterial hypertension (SSc-PAH) continue to have an unacceptably high mortality despite the progress achieved with pulmonary arterial vasodilator therapies. We sought to determine whether SSc-PAH is a clinically distinct pulmonary vascular disease phenotype when compared to IPAH, based on the progression of echocardiographic right ventricular (RV) dysfunction. Retrospective analysis of echocardiographic data in 13 SSc-PAH and 11 IPAH patients was used to delineate the progression of RV dysfunction during single or combination pulmonary arterial vasodilator therapy. All patients had right heart catheterization (RHC) confirmed pulmonary arterial hypertension, complete baseline (at the time of diagnosis) and follow up (most recent) echocardiograms. We excluded patients with significant scleroderma associated interstitial lung disease. Adjusting for time of follow up and disease duration, we performed mixed model regression analyses compari...
European journal of echocardiography : the journal of the Working Group on Echocardiography of the European Society of Cardiology, 2005
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. 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 p...