Shone syndrome revealed by treatment-resistant hypertension.

2021 
Introduction and importance: Shone complex is a congenital heart defect consisting of four obstructive defects in the left heart: a mitral supravalvular ring, sub-aortic stenosis, parachute mitral valve, and coarctation of the aorta (CoA), which affects only a small minority of people. Case presentation We report the case of a 25-year-old woman with a past medical history of moderate mitral stenosis, since she was 10-year-old with uncontrolled high blood pressure, treated with nicardipine. admitted to our emergency department with high blood pressure: 190/80 mmhg, in whom The transthoracic echocardiography (TTE) revealed: sub-mitral membrane, with a single sub-papillary muscle, and coarctation of the aorta and the CT scan showed narrowed aortic arch and a left superior vena cava allowing to retain shone syndrome as the main diagnosis. The patient was treated with an antihypertensive treatment combining (perindopril/indapamide/amlodipine) while waiting for surgery. Clinical discussion In this mini-review, we aim to describe this rare pathological condition its pathophysiological thoughts, and the way to diagnosis this complex early. Conclusion Treatment required the coordinated efforts of a team of specialists. It could be either surgical with different method or by Trans catheter treatments.
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