Right ventricular cardiomyopathy in systemic sclerosis

Belin, Rashad J.; Varga, John; Collins, Jeremy D.; Freed, Benjamin H.

doi:10.1093/rheumatology/kew494

Cited by 4 publications

(5 citation statements)

References 2 publications

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“…In patients with SSc, right ventricular (RV) dysfunction is more common than left ventricular (LV) dysfunction and may exist due to primary myocardial involvement, increased pulmonary vascular resistance (PVR), or a mixture of both ( 2 , 8 , 9 ). RV function may occur early in the disease course ( 2 , 9 , 10 ) and myocardial fibrosis may be responsible for both diastolic and systolic dysfunction ( 2 , 8 , 11 ). While RV dysfunction is often associated with abnormal pulmonary artery (PA) pressures and PAH, myocardial fibrosis may directly lead to RV dysfunction and elevated right atrial pressure (RAP) even in the absence of elevated PA pressures ( 11 ).…”

Section: Right Ventricular Dysfunction and Pulmonary Hypertensionmentioning

confidence: 99%

“…RV function may occur early in the disease course ( 2 , 9 , 10 ) and myocardial fibrosis may be responsible for both diastolic and systolic dysfunction ( 2 , 8 , 11 ). While RV dysfunction is often associated with abnormal pulmonary artery (PA) pressures and PAH, myocardial fibrosis may directly lead to RV dysfunction and elevated right atrial pressure (RAP) even in the absence of elevated PA pressures ( 11 ).…”

Section: Right Ventricular Dysfunction and Pulmonary Hypertensionmentioning

confidence: 99%

“…Therefore, high resolution T1 mapping and myocardial extracellular volume (ECV) have emerged as useful techniques in detecting diffuse RV fibrosis, even in the absence of PH. CMR measures of myocardial fibrosis, such as T1 mapping and ECV, are valuable to detect fibrosis early in the disease course, which can potentially alter therapy and prevent worsening RV dysfunction ( Figure 3 ) ( 2 , 11 , 31 – 36 ). A study by Chaosuwannakit et al found that 21% of patients with SSc who underwent CMR had RV dilation despite not meeting criteria for PH suggesting the presence of intrinsic RV dysfunction – perhaps due to fibrosis – in these patients ( 37 ).…”

Section: Right Ventricular Dysfunction and Pulmonary Hypertensionmentioning

confidence: 99%

“…RV, right ventricle; LV, left ventricle. Note: this image is reproduced with permission from Belin et al ( 11 ).…”

Section: Right Ventricular Dysfunction and Pulmonary Hypertensionmentioning

confidence: 99%

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Cardiovascular Imaging for Systemic Sclerosis Monitoring and Management

Glynn

Hale

Hussain

et al. 2022

Front. Cardiovasc. Med.

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Systemic sclerosis (SSc) is a complex connective tissue disease with multiple clinical and subclinical cardiac manifestations. SSc can affect most structural components of the heart, including the pericardium, myocardium, valves, and conduction system through a damaging cycle of inflammation, ischemia, and fibrosis. While cardiac involvement is the second leading SSc-related cause of death, it is frequently clinically silent in early disease and often missed with routine screening. To facilitate identification of cardiac disease in this susceptible population, we present here a review of cardiac imaging modalities and potential uses in the SSc patient population. We describe well-characterized techniques including electrocardiography and 2D echocardiography with Doppler, but also discuss more advanced imaging approaches, such as speckle-tracking echocardiography, cardiovascular magnetic resonance imaging (CMR), and stress imaging, among others. We also suggest an algorithm for the appropriate application of these modalities in the workup and management of patients with SSc. Finally, we discuss future opportunities for cardiac imaging in SSc research to achieve early detection and to optimize treatment.

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Section: Right Ventricular Dysfunction and Pulmonary Hypertensionmentioning

confidence: 99%

Section: Right Ventricular Dysfunction and Pulmonary Hypertensionmentioning

confidence: 99%

Section: Right Ventricular Dysfunction and Pulmonary Hypertensionmentioning

confidence: 99%

“…RV, right ventricle; LV, left ventricle. Note: this image is reproduced with permission from Belin et al ( 11 ).…”

Section: Right Ventricular Dysfunction and Pulmonary Hypertensionmentioning

confidence: 99%

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Cardiovascular Imaging for Systemic Sclerosis Monitoring and Management

Glynn

Hale

Hussain

et al. 2022

Front. Cardiovasc. Med.

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“…3 Isolated cases have been reported in Takotsubo cardiomyopathy, systemic sclerosis, autoimmune hypothyroidism, and isolated RV noncompaction cardiomyopathy. 4 5 6 7 Here, we highlight a very rare case of idiopathic isolated RV cardiomyopathy and approach to its diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Idiopathic Isolated Right Ventricular Cardiomyopathy: A Rare Case Report

et al. 2023

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Idiopathic isolated right ventricular cardiomyopathy is an extremely rare cause of right ventricular failure. Here, we report a case of 65-year-old male presented with chief complaints of dyspnea, fatigue, and bilateral pedal edema for the last 6 months. On clinical evaluation, grade II/III pansystolic murmur was present in left parasternal area. Investigations and imaging revealed dilated right atrium and ventricle with normal pulmonary artery pressure without any etiology. Magnetic resonance imaging ruled out other common causes of right ventricular cardiomyopathy; thus, the patient was diagnosed as a case of idiopathic isolated right ventricular cardiomyopathy that is a diagnosis of exclusion.

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