# The emerging role of cardiovascular magnetic resonance in the evaluation of cardiac involvement in systemic sclerosis

**Authors:** Sophie I. Mavrogeni, Alessia Pepe

PMC · DOI: 10.1515/rir-2024-0012 · Rheumatology and Immunology Research · 2024-07-15

## TL;DR

Cardiovascular magnetic resonance (CMR) is a promising tool for evaluating heart involvement in systemic sclerosis, offering detailed tissue characterization and function assessment.

## Contribution

The paper highlights CMR's ability to detect myocardial inflammation, fibrosis, and ischemia in systemic sclerosis, which echocardiography cannot achieve.

## Key findings

- CMR provides tissue characterization and function assessment without radiation.
- Modified Lake Louise criteria using T2, native T1 mapping, and ECV can diagnose inflammatory cardiomyopathy.
- Transmural/subendocardial LGE patterns indicate epicardial and microvascular coronary artery disease.

## Abstract

Systemic sclerosis (SSc) is an autoimmune rheumatic disease, characterized by vascular, inflammatory and fibrotic alterations. Cardiac involvement is the « fatal tip of the iceberg» in SSc, as it leads to high morbidity/mortality. Cardiovascular imaging modalities play an important role in the early diagnosis and treatment assessment of cardiac involvement. Echocardiography is the corner stone for evaluation of cardiac involvement, providing information about function, wall motion, pulmonary pressure, pericardium and valvular disease. It is a low-cost modality, widely available, without radiation and with great experience among cardiologists. However, it is a window and operator dependent modality and cannot provide tissue characterization information, absolutely necessary for diagnosis and treatment of cardiac involvement in SSc. Cardiovascular magnetic resonance (CMR) can perform myocardial function and tissue characterization in the same examination without radiation, has excellent reproducibility and is window and operator independent. The great advantage of CMR is the capability to assess peri- myo-vascular inflammation, myocardial ischemia and presence of replacement and diffuse myocardial fibrosis in parallel with ventricular function assessment. The modified Lake Louise criteria including T2, native T1 mapping and extracellular volume fraction (ECV) has been recently used to diagnose inflammatory cardiomyopathy. According to expert recommendations, myocardial inflammation should be considered if at least 2 indices, one T2 and one T1 parameter are positive, whereas native T1 mapping and ECV assess diffuse fibrosis or oedema, even in the absence of late gadolinium enhancement (LGE). Moreover, transmural/subendocardial LGE following the distribution of coronary arteries and diffuse subendocardial fibrosis not related with epicardial coronary arteries are indicative of epicardial and micro-vascular coronary artery disease, respectively. To conclude, CMR can overcome the limitations of echocardiography by identifying acute/active or chronic myocardial inflammation/fibrosis, ischemia and myocardial infarction using classic and parametric indices in parallel with biventricular function assessment

## Linked entities

- **Diseases:** systemic sclerosis (MONDO:0005100)

## Full-text entities

- **Genes:** DLD (dihydrolipoamide dehydrogenase) [NCBI Gene 1738] {aka DLDD, DLDH, E3, GCSL, LAD, OGDC-E3}
- **Diseases:** autoimmune rheumatic disease (MESH:D012216), myocardial disease (MESH:D004194), CV (MESH:D002318), Myocardial Ischemia (MESH:D017202), vascular dysfunction (MESH:D002561), myocardial involvement (MESH:C564676), Lipomatous (MESH:D008080), ischemic (MESH:D002545), myocardial infarction (MESH:D009203), ECAD (MESH:D003324), death (MESH:D003643), myocardial alterations (MESH:D004408), amyloid (MESH:C000718787), peri (MESH:D057873), HF (MESH:D006333), Diffuse Myocardial Fibrosis (MESH:D005355), ischaemia (MESH:D007511), Myocardial Inflammation (MESH:D007249), myo-vascular (MESH:D057772), chronic renal failure (MESH:D007676), valvular disease (MESH:D006349), Cardiac involvement (MESH:D006331), thrombus (MESH:D013927), inflammatory cardiomyopathy (MESH:D009202), myocardial oedema (MESH:C536897), Reumatism Scleroderma (MESH:D012595), LGE (MESH:C564835), arrhythmias (MESH:D001145)
- **Chemicals:** regadenoson (MESH:C430916), adenosine (MESH:D000241), STIRT2 (-), Gadolinium (MESH:D005682)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

_Full body text omitted from this summary view._ Fetch the complete paper as Markdown: https://tomesphere.com/paper/PMC12308729/full.md

## Figures

6 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12308729/full.md

## References

28 references — full list in the complete paper: https://tomesphere.com/paper/PMC12308729/full.md

---
Source: https://tomesphere.com/paper/PMC12308729