Shone syndrome revealed by treatment-resistant hypertension

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. We report the case of a 25...

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Veröffentlicht in:Annals of medicine and surgery 2021-11, Vol.71, p.102955, Article 102955
Hauptverfasser: Boulouiz, Soumia, Kossir, Amine, Mouedder, Fadoua, Miri, Chaimae, Ismaili, Nabila, El Ouafi, Noha
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Sprache:eng
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Zusammenfassung: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. 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. In this mini-review, we aim to describe this rare pathological condition its pathophysiological thoughts, and the way to diagnosis this complex early. Treatment required the coordinated efforts of a team of specialists. It could be either surgical with different method or by Trans catheter treatments. •The shone complex is a very rare congenital heart disease.•The long-term prognosis with this complex is poor, with a mortality rate of 24%–27%.•Minor mitral valve pathology patients should undergo a thorough diagnostic examination since they may have other obstructive lesions of the left outflow tract.•Individual lesions differ in clinical presentation and severity, making correct diagnosis complex.•Surgical interventions and outcomes in adult patients have not been reviewed as well as in children.
ISSN:2049-0801
2049-0801
DOI:10.1016/j.amsu.2021.102955