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Shone syndrome revealed by treatment-resistant hypertension
Soumia Boulouiz1, Amine Kossir1, Fadoua Mouedder1
1Department of Cardiology, Mohammed VI University Hospital of Oujda, Mohammed First University of Oujda, Morocco.
Insights
Shone complex, a rare congenital heart defect, involves four left heart obstructions. Early diagnosis and coordinated specialist treatment, including surgical or transcatheter interventions, are crucial for managing this condition.
Area of Science:
- Cardiology
- Congenital Heart Disease
Background:
- Shone complex is a rare congenital heart defect characterized by four left heart obstructive lesions.
- These include mitral supravalvular ring, sub-aortic stenosis, parachute mitral valve, and coarctation of the aorta (CoA).
Observation:
- A 25-year-old woman presented with severe hypertension (190/80 mmHg).
- Transthoracic echocardiography revealed sub-mitral membrane, single sub-papillary muscle, and coarctation of the aorta.
- CT scan showed a narrowed aortic arch and a left superior vena cava, confirming Shone syndrome.
Findings:
- The case highlights the diagnostic challenges and clinical presentation of Shone complex in an adult.
- Multimodality imaging (TTE and CT) was essential for accurate diagnosis.
Implications:
- This review emphasizes the importance of early diagnosis and comprehensive management strategies for Shone complex.
- Multidisciplinary collaboration is vital for successful treatment, which may involve surgery or transcatheter procedures.
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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