A case of familial hypercholesterolemia that necessitated coronary by pass surgery in childhood Case Report

Öz

Familial hypercholesterolemia is an autosomal dominant dyslipidemia causing premature coronary artery disease A 12 year old girl with complaints of chest pain and syncope during the last six months was referred for cardiac evaluation nbsp; Echocardiographic examination revealed a hyperechogenic crescentic mass in the ascending aorta wich was thought to be an atheroma just above the aortic valve narrowing both coronary ostia Coronary angiography showed 40 stenosis in the left main coronary artery 80 stenosis in the left anterior descending artery 95 stenosis in the ostium of the right coronary artery and 50 stenosis in the proximal part of the circumflex artery She underwent a coronary artery by pass grafting operation The risk of premature coronary artery disease is high in familial hypercholesterolemia Therefore coronary angiography is useful in those patients who have ıschemic symptoms Key words: hypercholesterolemia atherosclerosis coronary by pass child

Anahtar Kelimeler

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Çocukluk çağında koroner “by pass” cerrahisi gerektiren ailevi hiperkolesterolemi olgusu Olgu Sunumu

Öz

Ailevi hiperkolesterolemi otozomal baskın dominan geçişli bir dislipidemi olup erken koroner arter hastalığı nedenidir 12 yaşındaki ailesel hiperkolesterolemi olgusu son altı ayda ortaya çıkan göğüs ağrısı ve senkop nedeniyle başvurdu Ekokardiyografik incelemesinde çıkan aortada aort kapağının hemen üzerinde başlayan yarımay şeklinde her iki koroner arter ostiyumunu daralttığı izlenimi alınan ve aterom plağı olabileceği düşünülen hiperekojen bir yapı görüldü Koroner anjiyografide sol ana koroner arterde 40 sağ koroner arter ostiyumunda 95 sol ön inen arterde 80 sirkumfleks arterin proksimalinde 50 darlık saptandı Bu bulgularla koroner “by pass” yapıldı Ailesel hiperlipidemi olgularında erken koroner arter hastalığı riski yüksektir Bu nedenle iskemik yakınmaları olan bu hastalara koroner anjiyografi yapılması yararlıdır Anahtar kelimeler:hiperkolesterolemi ateroskleroz koroner by pass çocuk SummaryFamilial hypercholesterolemia is an autosomal dominant dyslipidemia causing premature coronary artery disease A 12 year old girl with complaints of chest pain and syncope during the last six months was referred for cardiac evaluation nbsp; Echocardiographic examination revealed a hyperechogenic crescentic mass in the ascending aorta wich was thought to be an atheroma just above the aortic valve narrowing both coronary ostia Coronary angiography showed 40 stenosis in the left main coronary artery 80 stenosis in the left anterior descending artery 95 stenosis in the ostium of the right coronary artery and 50 stenosis in the proximal part of the circumflex artery She underwent a coronary artery by pass grafting operation The risk of premature coronary artery disease is high in familial hypercholesterolemia Therefore coronary angiography is useful in those patients who have ıschemic symptoms Key words: hypercholesterolemia atherosclerosis coronary by pass child

Anahtar Kelimeler

• hiperkolesterolemi, ateroskleroz, koroner by-pass, çocuk

Kaynakça

1. Mehan VK, Salzmann C, Pfammatter JP, Stocker FP, Meier
2. B. Left main coronary angioplasty in a ten year old boy with
3. homozygous familial hypercholesterolaemia. Cathet Cardiovasc
4. Diagn 1993; 29: 24- 7. 8.
5. Pfammatter JP, Stocker FP, Oetliker O, Wiesmann U, Meier
6. B. Familial hypercholesterolaemia with severe cardiac
7. involvement in a boy: successful management and mid-term
8. Pfammatter JP, Stocker FP, Oetliker O, Wiesmann U, Meier

9. B. Familial hypercholesterolaemia with severe cardiac
10. involvement in a boy: successful management and mid-term
11. follow-up. Acta Paediatr 1996; 85: 254- 7.