Background/Purpose

Heart failure and cardiac dysfunctions both of intrinsic or secondary origin and targeting LV (left ventricule) and/or RV (right ventricule) are critical complications promoting mortality in systemic sclerosis (SSc). While several studies reported possible right ventricule (RV) alterations in SSc patients having pulmonary hypertension, only few and small series investigated RV function in unselected SSc patients. Therefore, the aim of the present study is to investigate LV and RV systolic and diastolic function in a large SSc cohort of unselected patients compared to a control group using comprehensive echocardiographic parameters.

Methods

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 two institutions (Paris, France and Los Angeles, USA) and 50 healthy controls.

Results

Patients’ characteristics from the two institutions were very similar allowing combined analyses. 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 (p<0.001) (Table 1). Patients also exhibited alterations in LV contractility and diastolic function (p<0.001 each) (Table 1). Looking at associated parameters, 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). In a subset of 27 patients with proven pre-capillary PAH, comparison between SSc-PAH versus SSc free of PAH patients, revealed reduced LV diastolic function (measured by transmitral E/A ratio (p=0.045) and E_A <10cm/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).

Conclusion:

Whereas most previous studies focused on the LV, we report in the present controlled study that not only systolic but also diastolic RV dysfunction is common in SSc and that several cardiopulmonary factors seem to influence RV function in this multifaceted disease. Given that RV dysfunction and fibrosis are poor prognosticator, possibly associated with lethal ventricular arrhythmias, sudden death, exercise limitation, and impaired RV cardiac output, we assume that RV function should be closely investigated in SSc patients and that the impact on RV diastolic function of future therapies targeting PAH and/or primary myocardial involvement is to be assessed.

Table 1: