{"id":5714,"date":"2026-09-11T08:29:09","date_gmt":"2026-09-11T06:29:09","guid":{"rendered":"https:\/\/cvja.co.za\/journal\/?p=5714"},"modified":"2026-09-11T08:29:09","modified_gmt":"2026-09-11T06:29:09","slug":"cvja-2026-036","status":"publish","type":"post","link":"https:\/\/cvja.co.za\/journal\/cvja-2026-036\/","title":{"rendered":"Complex cardiac and porcelain aorta surgery in patients with untreated familial hypercholesterolaemia: a case report"},"content":{"rendered":"
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\r\n \r\n \r\nRahma Kallel, Hela Ben Jemaa, Faten Dhouib, Hela Fendri, Amal Samet, Imed Frikha, Hichem Denguir\r\n<\/div> \r\n<\/div> \r\n
\r\n Copyright:<\/strong> Clinics Cardive Publishing (Pty) Ltd. publisher\r\n of Cardiovascular Journal of Africa.<\/div>\r\n
<\/div> \r\n
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\r\n Abstract \r\n <\/div> \r\n \r\n
\r\n \r\n \r\n \r\n Familial hypercholesterolaemia (FH) is a genetic disorder characterised by elevated levels of circulating low-density lipoprotein cholesterol. Serious presentations are challenging to treat. We report a case of complex cardiovascular outcomes in a 28-year-old man with diabetes mellitus and untreated FH. The diagnostic workup was compatible with a homozygous phenotype. The patient presented with a non-ST-elevation myocardial infarction, which revealed severe premature three-vessel coronary artery disease with significant proximal blockages, including severe ostial left main stenosis, as well as severe valvular and supravalvular aortic stenosis. Aortic computed tomography angiography (CTA) confirmed the presence of a porcelain aorta and a guided repair. Despite the porcelain aorta, the patient underwent conventional cardiac and aortic surgery with an uneventful post-operative course. This case highlights a rare case of successful cardiac and aortic surgery in the context of a porcelain aorta, performed safely by experienced surgeons using current perfusion and operative techniques guided by CTA imaging. Ongoing efforts are required to improve early detection and prompt management of FH. \r\n\r\n
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\r\n \r\n Keywords:<\/strong> \r\n \r\n hyperlipoproteinaemia type II, aortic stenosis, supravalvular, porcelain aorta, cardiovascular surgical procedures.<\/strong> \r\n \r\n <\/div> \r\n \r\n
\r\n \r\n Submitted:<\/strong> October 2, 2025; \r\n Accepted:<\/strong> May 13, 2026; \r\n Published:<\/strong> September 11, 2026
\r\n Cardiovasc J Afr 2025; 37<\/b>: 381-385<\/em>\r\n
Volume 37, Issue 3<\/strong>
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\r\n DOI Citation Reference: dx.doi.org\/10.5830\/CVJA-2026-036 \r\n <\/div> \r\n \r\n
\r\n\r\n\r\nFaculty of Medicine of Sfax, University of Sfax, Sfax, Tunisia\r\n\r\n<\/strong>
\r\nRahma Kallel
\r\nHela Ben Jemaa
\r\nFaten Dhouib
\r\nHela Fendri
\r\nImed Frikha
\r\nHichem Denguir\r\n

\r\n\r\nDepartment of Cardiovascular Surgery, Habib Bourguiba University Hospital, Sfax, Tunisia\r\n\r\n<\/strong>
\r\nHela Ben Jemaa
\r\nFaten Dhouib
\r\nImed Frikha\r\n

\r\n\r\nDepartment of Radiology, Hedi Chaker University Hospital, Sfax, Tunisia\r\n\r\n<\/strong>
\r\nHela Fendri\r\n\r\n

\r\n\r\nDepartment of Emergency, Hospital of Mahres, University of Sfax, Sfax, Tunisia\r\n\r\n<\/strong>
\r\nAmal Samet\r\n

\r\n\r\n\r\n\r\n [soshsc hide=\"logged\"]\r\n \r\n You need to be logged in to view and download<\/a> the full text PDF version. Register for a free account?<\/a><\/strong>

[\/soshsc]\r\n \r\n \r\n \r\n <\/div>\r\n \r\n<\/div>","protected":false},"excerpt":{"rendered":"

Familial hypercholesterolaemia (FH) is a genetic disorder characterised by elevated levels of circulating low-density lipoprotein cholesterol. 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