Prognostic impact of coronary microcirculation abnormalities in systemic sclerosis: a prospective study to evaluate the role of non-invasive tests

Abstract

IntroductionMicrocirculation dysfunction is a typical feature of systemic sclerosis (SSc) and represents the earliest abnormality of primary myocardial involvement. We assessed coronary microcirculation status by combining two functional tests in SSc patients and estimating its impact on disease outcome.MethodsForty-one SSc patients, asymptomatic for coronary artery disease, were tested for coronary flow velocity reserve (CFR) by transthoracic-echo-Doppler with adenosine infusion (A-TTE) and for left ventricular wall motion abnormalities (WMA) by dobutamine stress echocardiography (DSE). Myocardial multi-detector computed tomography (MDCT) enabled the presence of epicardial stenosis, which could interfere with the accuracy of the tests, to be excluded. Patient survival rate was assessed over a 6.7- ± 3.5-year follow-up.ResultsNineteen out of 41 (46%) SSc patients had a reduced CFR (≤2.5) and in 16/41 (39%) a WMA was observed during DSE. Furthermore, 13/41 (32%) patients showed pathological CFR and WMA. An inverse correlation between wall motion score index (WMSI) during DSE and CFR value (r = -0.57, P <0.0001) was observed; in addition, CFR was significantly reduced (2.21 ± 0.38) in patients with WMA as compared to those without (2.94 ± 0.60) (P <0.0001). In 12 patients with abnormal DSE, MDCT was used to exclude macrovasculopathy. During a 6.7- ± 3.5-year follow-up seven patients with abnormal coronary functional tests died of disease-related causes, compared to only one patient with normal tests.ConclusionsA-TTE and DSE tests are useful tools to detect non-invasively pre-clinical microcirculation abnormalities in SSc patients; moreover, abnormal CFR and WMA might be related to a worse disease outcome suggesting a prognostic value of these tests, similar to other myocardial diseases.

Coronary Microvascular Dysfunction in Asymptomatic Patients Affected by Systemic Sclerosis – Limited vs. Diffuse Form –

“…We believe that these data are important because they highlight the benefit of periodic cardiac evaluation of CFR in this subgroup, particularly in view of the negative prognostic significance of a reduced CFR, as shown by Vacca et al 18 We confirm the findings of Vacca et al relative to the greater prevalence of reduced CFR in dcSSc than in lcSSc. Compared with the latter study, however, we used a more conservative cutoff value of CFR (ie, ≤2.0 instead of ≤2.5 previously used by Vacca et al).…”

“…18 Our results, however, highlight the importance of early evaluation of CFR in these patients for prognostic stratification. In fact, the dcSSc patients in our study had a shorter disease duration than in those with lcSSc (5.43±3.16 years vs. 15.83±12.99 years, P=0.02).…”

Subclinical Cardiovascular Involvement in Autoimmune Diseases: Role of Coronary Flow Reserve

“…The SSc patients had a significantly lower CFR than controls, Finally, Vacca et al [36] have very recently confirmed our data in a group of 41 patients with SSc who were asymptomatic for CAD using transthoracic Doppler echocardiography with adenosine infusion to study the microcirculation and dobutamine stress echocardiography to search for left ventricular wall motion abnormalities. CFR was reduced in 19 SSc patients, and 16 showed wall motion abnormalities.…”

Population-based analysis of hospitalizations for patients with systemic sclerosis in a West-European region over the period 2001–2012

“…Introduction associated with the worse survival, but, given present rarity of renal crisis, cardiopulmonary involvement is now accounting for much of the mortality in patients with SSc [5][6][7][8]. However, survival rate may vary across countries being influenced by several factors such as ethnicity, national income, and healthcare programs [9,10].…”