Why the study?
Cardiac involvement in Sneddon syndrome is rare and its physiopathology remains unclear.
This case highlights the rare coexistence of myocardial injury and aortic valve disease in Sneddon syndrome, emphasizing the role of multimodality imaging for diagnosis and follow-up.
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Alerts clinicians to possible INOCA and valvular disease in antibody-negative Sneddon syndrome; leaves open need for systematic cardiac screening.
BACKGROUND: Cardiac involvement in Sneddon syndrome (SS) is rare, the physiopathology is still unclear. We report a first case of SS without antiphospholipid antibodies who had coexisting ischaemia with no obstructive coronary arteries and aortic valve diseases. CASE SUMMARY: A 34-year-old woman with SS without antiphospholipid antibodies, was admitted for aphasia, and paresthaesia with confirmed right opercular ischaemic lesions at brain magnetic resonance imaging. Transthoracic echocardiographic examination showed akinesis of apical segments, moderate aortic valve stenosis, and moderate aortic insufficiency. Coronary angiogram was normal. Cardiac magnetic resonance showed transmural necrosis in the territory of the left anterior descending artery. Seven years later, our patient had no change or progression of myocardial ischaemic lesions or valvular disease. CONCLUSION: We will discuss different hypothesis, diagnosis, treatment, and evolution of cardiac involvement in SS. Close follow-up should be regularly performed for early diagnosis, hence the importance of multimodality imaging, to guide treatment and prevent further complications.
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Scadi et al. (2021) studied this question.
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