Right Bundle Branch Block: A Predictor of Mortality in Early Systemic Sclerosis

Abstract

ObjectiveTo evaluate the prognostic significance of baseline electrocardiogram (ECG) abnormalities in a multiethnic cohort of patients with early systemic sclerosis (SSc) and to determine the serological, clinical, and echocardiogram correlates of ECG findings.MethodsSSc patients with disease duration of≤5 years were enrolled in the GENISOS (Genetics versus Environment in Scleroderma Outcome Study) cohort. At the first visit, a standard 12 lead ECG was obtained along with demographic information, clinical data, and autoantibodies. The results of echocardiograms were also recorded. All ECGs were interpreted by a cardiologist unaware of the patients' clinical data.ResultsOf 265 SSc patients with average disease duration at enrollment of 2.5 years, 140 (52.8%) had abnormal ECG findings. These findings were not associated with SSc disease type or autoantibody profile but were associated with more severe heart and lung involvement. A total of 75 patients (28.3%) died over a follow up time of 9.9 years. Complete right bundle branch block (± left anterior hemiblock) on ECG, present in 7 (2.6%) patients, predicted a higher risk of mortality (HR: 5.3; 95% CI: 2.1 to 13.4; p<0.001). The predictive significance of right bundle branch block was independent of age at enrollment, gender, ethnicity and risk factors for coronary artery disease.ConclusionECG abnormalities are common in patients with early SSc and are associated with the severity of lung and heart involvement. Right bundle branch block is an independent predictor of mortality, and should be considered a marker of disease severity in SSc.

Differences in Right Ventricular Functional Changes during Treatment between Systemic Sclerosis–associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension

“…Some plausible mechanisms that could explain the poor survival in patients with SSc-PAH include (1) a more aggressive phenotype of pulmonary vascular disease with or without a significant presence of pulmonary venoocclusive disease (8) and (2) poor RV adaptation to the worsening PVR. Possible causes of poor RV adaptation include myocardial fibrosis (9), intracardiac conduction delays (10), or accelerated coronary atherosclerosis (11,12). Because progressive RV dysfunction usually precedes death in patients with PAH, we sought to compare the progression of RV dysfunction in patients with idiopathic pulmonary arterial hypertension (IPAH) with that of patients with SSc-PAH, as well as to delineate the differences in RV function change over time between these two groups.…”

Pharmacological stress, rest perfusion and delayed enhancement cardiac magnetic resonance identifies very early cardiac involvement in systemic sclerosis patients of recent onset

“…SSc‐HD is often asymptomatic for a long time and, when recognized, is associated with a poor prognosis. In the last years, new techniques have been shown to be more sensitive methods than conventional echocardiography to allow us a very early diagnosis, possibly in the preclinical stage, when therapeutic strategies could be more effective …”

“…Cardiac magnetic resonance (CMR) is recognized as a useful tool for the diagnosis of myocardial diseases. Stress and rest perfusion CMR technique is largely used to assess myocardial structural damages, and in SSc patients, some specific CMR patterns may be detected …”

“…In the last years, new techniques have been shown to be more sensitive methods than conventional echocardiography to allow us a very early diagnosis, possibly in the preclinical stage, when therapeutic strategies could be more effective. [1][2][3][4][5] Cardiac magnetic resonance (CMR) is recognized as a useful tool for the diagnosis of myocardial diseases. Stress and rest perfusion CMR technique is largely used to assess myocardial structural damages, 4,5 and in SSc patients, some specific CMR patterns may be detected.…”

“…[1][2][3][4][5] Cardiac magnetic resonance (CMR) is recognized as a useful tool for the diagnosis of myocardial diseases. Stress and rest perfusion CMR technique is largely used to assess myocardial structural damages, 4,5 and in SSc patients, some specific CMR patterns may be detected. 6,7 In a previous study, we used contrast enhanced-CMR in SSc patients with long-standing disease to assess the presence and the distribution of myocardial fibrosis by the late enhancement (LGE) technique.…”