Exploring CMR Imaging in Cardiac Conditions Related to Systemic Disease

In this episode, CardioNerds Dr. Gurleen Kaur, Dr. Richard Ferraro, and Dr. Jake Roberts are joined by Cardio-Rheumatology expert, Dr. Monica Mukherjee, to discuss the role of utilizing multimodal imaging for cardiovascular disease risk stratification, monitoring, and management in patients with chronic systemic inflammation. The team delves into the contexts for utilizing advanced imaging to assess systemic inflammation with cardiac involvement, as well as the role of imaging in monitoring various specific cardiovascular complications that may develop due to inflammatory diseases. Audio editing by CardioNerds academy intern, Christiana Dangas.

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Pearls – Cardiovascular Multimodality Imaging & Systemic Inflammation

1. Systemic inflammatory diseases are associated with an elevated CVD risk that has significant implications for early detection, risk stratification, and implementation of therapeutic strategies to address these risks and disease-specific complications. As an example, patients with SLE have a 48-fold increased risk for developing ASCVD compared to the general population. They may also develop disease-specific complications, such as pericarditis, that require focused imaging approaches to detect.
2. In addition to increasing the risk for CAD, systemic inflammatory diseases can also result in cardiac complications, including myocardial, pericardial, and valvular involvement. Assessment of these complications requires the use of different imaging techniques, with the modality and serial studies selected based on the suspected disease process involved.
3. In most contexts, echocardiography remains the starting point for evaluating cardiac involvement in systemic inflammatory diseases and can inform the next steps in terms of diagnostic study selection for the assessment of specific cardiac processes. For example, if echocardiography is completed in an SLE patient and demonstrates potential myocardial or pericardial inflammation, the next steps in evaluation may include completing a cardiac MRI for better characterization.
4. While no current guidelines or standards of care directly guide our selection of advanced imaging studies for screening and management of CVD in patients with systemic inflammatory diseases, our understanding of cardiac involvement in these patients continues to improve and will likely lead to future guideline development.
5. Due to the vast heterogeneity of cardiac involvement both across and within different systemic inflammatory diseases, a personalized approach to caring for each individual patient remains central to CVD evaluation and management in these patients. For example, patients with systemic sclerosis and symptoms of shortness of breath may experience these symptoms due to a range of causes. Echocardiography can be a central guiding tool in assessing these patients for potential concerns related to pulmonary hypertension or diastolic dysfunction. Based on the initial echocardiogram, the next steps in evaluation may involve further ischemic evaluation or right heart catheterization, depending on the pathology of concern.

Show notes – Cardiovascular Multimodality Imaging & Systemic Inflammation

Episode notes drafted by Dr. Jake Roberts.

What are the contexts in which we should consider pursuing multimodal cardiac imaging, and are there certain inflammatory disorders associated with systemic inflammation and higher associated CVD risk for which advanced imaging can help guide early intervention?

• Systemic inflammatory diseases are associated with elevated CVD risk, which has significant implications for early detection, risk stratification, prognostication, and implementation of therapeutic strategies to address CVD risk and complications in these patient populations.
  • The most well-characterized autoimmune diseases with an association between systemic inflammation and CVD risk are inflammatory arthritic conditions such as rheumatoid arthritis. Additional inflammatory diseases with elevated CVD risk include spondyloarthropathies and psoriatic arthritis.
  • Patients with rheumatoid arthritis have a 1.5- 2x risk of developing coronary artery disease compared to the general population.
  • The mechanism of elevated CVD risk in inflammatory disease patients is likely related to a combination of abnormalities in lipid metabolism, endothelial dysfunction, and vascular inflammation.
  • Conditions including systemic lupus erythematosus (SLE), myositis, vasculitis disorders, and systemic sclerosis may have additional cardiovascular complications beyond CAD, including pericarditis, myocarditis, electrical, and valvular complications.

Are there any established or emerging technologies to help with improving early detection or characterization of cardiac involvement in systemic inflammatory diseases?

• Echocardiography remains the most common and useful starting point for screening and early detection of cardiac involvement in systemic inflammatory diseases due to its widespread availability, real-time interpretation, low cost, and noninvasive nature. Furthermore, echocardiography remains a crucial tool in serial monitoring for disease progression and the detection of therapeutic effects. This modality additionally provides significant utility for early detection and screening of pericardial and valvular involvement.
  • Given that patients with inflammatory disorders have an elevated risk for developing CAD, utilizing CAC scores and CCTA are often additionally helpful for CAD detection in these patient populations.

Are there different imaging techniques that should be used to assess complications specific to different systemic inflammatory diseases?

• Based on the specific disease involved, the choice of imaging technique may vary depending on the clinical context and the cardiovascular complication requiring further investigation.
• As an example, in systemic sclerosis, there can be a wide range of variable cardiac manifestations that emerge depending on the subtype of the disease, with the cardiac complications developing either because of the fibrotic disease process or from other secondary effects of the disease. Specifically, if the patient’s phenotype involves interstitial lung disease, the right ventricle of the heart will encounter chronic increased afterload, which can lead to adverse adaptive responses and remodeling over time. As a result, screening tools such as echocardiography can be very useful in this patient population, with these patients often requiring regular annual screening echocardiograms coupled with pulmonary function testing to screen for coupled changes in individual patients’ physiology. When these patients develop complications of their disease, including pulmonary hypertension, echocardiography can help evaluate the underlying cause of this complication and inform subsequent diagnostic steps.
• In terms of assessing myocardial disease and inflammation in myocardial tissue, cardiac MRI remains a valuable tool in detecting subclinical myocardial disease and can identify areas of low-grade myocardial inflammation. One of the advantages of cardiac MRI over other imaging techniques involves its ability to allow for noninvasive tissue characterization.
• For disease complications such as pericarditis, which can commonly develop in SLE, 2D echocardiography remains the first-line imaging modality of choice to detect pericardial disease involvement. In SLE patients who have long-standing pericardial disease with progression, they can also develop constrictive symptoms resulting from this process. In those cases, either CT or cardiac MRI can assist in defining the pericardial or myocardial anatomy.

As an example, what would be the approach to utilizing advanced imaging to assess for CVD detection and monitoring in a patient with SLE with relatively well-controlled symptoms on chronic immunosuppressive agents and no prior history of heart failure or CVD?

• As a starting point, all patients with systemic inflammatory diseases should undergo comprehensive ASCVD risk assessment. Initial stratification involves completing a laboratory assessment with a standard lipid panel and diabetes screening studies. Further evaluation of any symptoms that a patient may describe, which could indicate potential early cardiovascular disease processes, should also be thoroughly assessed and may influence the next steps in screening.
• In the context of SLE, pericardial disease is common, and therefore, obtaining a baseline echocardiogram to assess for any early pericardial involvement should be the initial step in evaluation. If the patient also has an elevated ASCVD risk, they should also undergo assessment for coronary artery disease.

What should be the approach to the sequence of imaging technique selection, serial imaging, monitoring, and follow-up in patients with systemic inflammatory disorders undergoing evaluation of CVD screening and monitoring?

• The initial selection of imaging modality should be based on what is suspected to be the primary driver of the patient’s symptoms or as the primary underlying process of concern that requires further evaluation.
• As an important consideration in the context of systemic inflammatory diseases such as SLE, ischemic disease may involve atypical presentations due to underlying myocardial dysfunction and microvascular disease. Therefore, imaging and other diagnostic studies may be warranted to assess for reversible ischemia. There is emerging evidence that cardiac PET perfusion and cardiac MRI may be particularly useful in this patient population to assess coronary flow reserve to evaluate for coronary microvascular disease.

What evidence currently exists to demonstrate the impact on cardiovascular outcomes resulting from the utilization of advanced multimodality imaging for CVD detection and monitoring in patients with systemic inflammatory disorders?

• While there is limited evidence that has directly measured the impact of advanced imaging utilization on CVD outcomes in this patient population, there is growing recognition of the increased risk of cardiac complications in patients with systemic inflammatory diseases. With increasing recognition of the commonality of cardiac involvement in these diseases, we are now more often utilizing appropriate testing in these patients.
• Directly measuring outcomes in these patient populations is somewhat challenging in large part due to the wide heterogeneity of phenotypes both across and within specific inflammatory diseases. Much of the approach in cardio-rheumatology should emphasize personalized medicine specific to each patient, given the wide range of cardiovascular complications and unique presentations of cardiac involvement in individual patients. Providing care for patients with systemic inflammatory diseases further requires a collaborative approach across disciplines and subspecialties within medicine to provide appropriate comprehensive care.

Is there a need for more standardized approaches for utilizing imaging in patients with systemic inflammatory diseases?

• Currently, there are no formal guidelines or standards of care to direct the use of multimodality imaging to assess CVD risk and direct management in patients with systemic inflammatory diseases.
• Many of the current standardized approaches are institution-dependent and often informed by clinical observations at individual medical centers.
• As we work to better understand the role of cardiac involvement in systemic inflammatory diseases and gain more experience in the evaluation of CVD and specific cardiovascular complications in these disorders, we will likely have ongoing development of standards of care and guidelines for management of CVD in these patients.

References – Cardiovascular Multimodality Imaging & Systemic Inflammation

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2. Mortensen MB, Jensen JM, Sand NP, et al. Association of Autoimmune Diseases with Coronary Atherosclerosis Severity and Ischemic Events. Journal of the American College of Cardiology. 2024;83(25):2643-2654. doi:https://doi.org/10.1016/j.jacc.2024.04.030
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6. Lu J, Jani V, Mercurio V, et al. Stress Echocardiographic Prediction of Emerging Pulmonary Vascular Disease in Systemic Sclerosis. Journal of the American Society of Echocardiography. 2023;36(2):259-261. https://doi.org/10.1016/j.echo.2022.10.006
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