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Shone's anomaly: a report of one case in sub-Saharan Africa
Clovis Nkoke1, Alain Lekoubou1, Edvine Wawo Yonta1
11 Faculty of Medicine and Biomedical Sciences, Department of Internal medicine, University of Yaounde1, Cameroon ; 2 Department of neurosciences, Division of Neurology, Medical University of South Carolina, 96 Jonathan Street, Ste 301 CSB, Charleston, South Carolina, USA ; 3 Department of Internal Medicine, Douala General Hospital, Cameroon ; 4 Non-Communicable Diseases Research Unit, South African Medical Research Council 7505 Cape Town, South Africa ; 5 Department of Medicine, Groote Schuur Hospital and University of Cape Town, Cape Town, South Africa.
Insights
This case report details a rare Shone anomaly variant in a Cameroonian teen, emphasizing early diagnosis and management of congenital heart defects in resource-limited areas.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Shone's anomaly is a rare congenital cardiac malformation involving multiple left heart obstructive lesions.
- It commonly presents in an incomplete form, affecting the mitral valve and aortic arch.
- Diagnosis can be challenging, particularly in resource-limited settings.
Abstract:
Shone's anomaly is a rare congenital cardiac malformation consisting of four obstructive lesions of the left heart and the aortic arch: a supramitral membrane; parachute mitral valve; subaortic stenosis; and coarctation of the aorta which occurs most commonly in its incomplete form. We report on a 17-year-old male Cameroonian with a variant of shone anomaly consisting of sub-valvular aortic stenosis, coarctation of the aorta and parachute mitral valve. He was referred for the investigation of a cardiac murmur after he presented with shortness of breath preceded by flu like syndrome. He underwent successful management abroad consisting of aortic coarctation treatment by percutaneous angioplasty and resection of the subaortic ring by traditional surgical approach without any mitral valve repair. Shone's anomaly is a rare congenital heart disease that can be missed in low resource settings. This case raises awareness about this syndrome, highlights the importance of cardiac ultrasonography in resource limited settings and illustrates the need for comprehensive service frameworks to improve access and level of care and services for patients in the African context.
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