Abstract
Background
Systemic sclerosis (SSc) is a progressive autoimmune disease which has been proven to affect the heart. While it is widely known that the disease can cause pulmonary artery hypertension and therefore secondary right heart impairment, new studies have detected a subclinical heart involvement of both the left and right ventricles. The similar changes in myocardial deformation of both chambers assessed by speckle tracking imaging are consistent with the definition of SSc-related cardiomyopathy as a standalone entity with peculiar characteristics.
Purpose
The aim of the present study is to describe the progression of myocardial deformation as assessed through speckle tracking in patients with SSc and no pulmonary hypertension.
Methods
Prospective, longitudinal study on 48 patients affected by SSc. Patients with a history of heart failure, known structural heart disease, atrial fibrillation, and pulmonary hypertension were excluded. For every patient, standard echocardiographical and speckle-tracking derived variables for the systolic and diastolic function of the left ventricle (LV) and right ventricle (RV) were acquired at baseline and after 15±6 months.
Results
While common parameters of systolic function (Simpons's left ventricular ejection fraction, right ventricular fractional area change, TAPSE, tricuspidal S') did not change during follow-up, mean global longitudinal strain (GLS) significantly worsened for both left (from −19.1%±4.2% to −17.2%±5.0%) and right ventricle (from −17.9%±5.2% to −15.9%±4.8%) over 15 months. The increased impairment seen in SSc patients was homogeneous across endocardial layers (LV: from −21.8%±4.8% to −18.8%±5.2%; RV: from −20.6%±4.5% to −19.4%±4.9%), midventricular layers (LV: from −19.2%±4.5% to −17.7%±4.9%; RV: from −17.7%±4.7% to −16.7%±4.6%) and epicardial layers (LV: from −16.3%±4.7% to −16.0%±4.3%; RV: from −15.4%±5.0% to −14.6%±4.1%), as well as across myocardial segments (Figure 1). No difference in progression rate was seen between the diffuse and limited version of SSc, nor between different serotypes.
Figure 1
Conclusions
While traditional echocardiographical parameters are useless in order to follow the natural history of SSc-related cardiomyopathy during its early stages, GLS impairment progresses during a 15-month follow-up and involves similarly both the left and right ventricle. Whether, how, and how much the altered myocardial deformation contributes to the incidence of pulmonary arterial hypertension in these patients is still to be assessed.
Acknowledgement/Funding
Marche Polytechnic University
Molecular normalization of dystrophin in the failing left and right ventricle of patients treated with either pulsatile or continuous flow-type ventricular assist devices