Utku Arman ÖRÜN, Selmin KARADEMİR, Burhan ÖCAL, Filiz ŞENOCAK, Özben CEYLAN

Büyük arterlerin anatomik düzeltilmiş malpozisyonu

Büyük arterlerin anatomik düzeltilmiş malpozisyonu (BAADM), atriyoventriküler konkordans veya diskordans ve ventriküloarteriyel konkordans ile arteriyel köklerin birbirine paralel çıkması ile karakterizedir. Anormal ilişkili aort sol ventrikülden, anormal ilişkili pulmoner arter sağ ventrikülden çıkar. Bu yazıda, BAADM tanısı konulan beş aylık ve iki yaşında iki erkek olgu sunuldu. Ekokardiyografik incelemede atriyal situs solitus (S), ventrikülerin d-ilmiği (D) ve büyük damarların l-malpozisyonu (L) [Van Praaghın sembolik terminolojisi (S.D.L)] olduğu saptandı. Tanı, kardiyak kateterizasyon ve anjiyografi ile kesinleştirildi. Her iki hasta da ameliyat edildi. Ameliyat sırasında BAADMnin morfolojik bulguları teyit edildi. Bu yazıda anatomik düzeltilmiş malpozisyonun nadir görülmesi nedeniyle akla gelmediği zaman büyük arter transpozisyonu ve doğuştan düzeltilmiş transpozisyon ile karıştırılabileceği vurgulandı.

Anatomically corrected malposition of the great arteries

Anatomically corrected malposition of the great arteries (ACMGA) is characterized by atrioventricular concordance or discordance and ventriculoarterial concordances with arterial roots arising parallel to each other. Abnormally related aorta arises from the left ventricle, while abnormally related pulmonary artery arises from the right ventricle. In this article, we report two male cases who were five-months-old and two-years-old diagnosed with ACMGA. Echocardiographic examination revealed atrial situs solitus (S), ventricular d-loop (D) and l-malposition (L) of great arteries [Van Praagh s symbolic nomenclature (S.D.L)]. The diagnosis was confirmed by cardiac catheterization and angiography. Both patients were operated. During the operation, morphologic findings of ACMGA were confirmed. In this article, we emphasize that anatomically corrected malposition is a rare condition and it can be confused with conditions such as transposition of great arteries and congenitally corrected transposition of great arteries, when it is not considered.

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1. Van Praagh R, Durnin RE, Jockin H, Wagner HR, Korns M, Garabedian H, et al. Anatomically corrected malposition of the great arteries (S, D, L). Circulation 1975;51:20-31.
2. Van Praagh R, Perez-Trevino C, Lõpez-Cuellar M, Baker FW, Zuberbuhler JR, Quero M, et al. Transposition of the great arteries with posterior aorta, anterior pulmonary artery, subpulmonary conus and fibrous continuity between aortic and atrioventricular valves. Am J Cardiol 1971;28:621-31.
3. Van Praagh R, Van Praagh S. Anatomically corrected transposition of the great arteries. Br Heart J 1967;29:112-9.
4. Anderson RH, Becker AE, Losekoot TG, Gerlis LM. Anatomically corrected malposition of great arteries. Br Heart J 1975;37:993-1013.
5. Kirklin JW, Pacifico AD, Bargeron LM Jr, Soto B. Cardiac repair in anatomically corrected malposition of the great arteries. Circulation 1973;48:153-9.
6. Freedom RM, Harrington DP. Anatomically corrected malposition of the great arteries. Report of 2 cases, one with congenital asplenia; frequent association with juxtaposition of atrial appendages. Br Heart J 1974;36:207-15.
7. Oku H, Shirotani H, Yokoyama T, Kawai J, Nishioka T, Noritake S, et al. Anatomically corrected malposition of the great arteries--case reports and a review. Jpn Circ J 1982;46:583-94.
8. Blume ED, Chung T, Hoffer FA, Geva T. Images in cardiovascular medicine. Anatomically corrected malposition of the great arteries [S,D,L]. Circulation 1998;97:1207.
9. Rittenhouse EA, Tenckhoff L, Kawabori I, Mansfield PB, Hall DG, Brown JW, et al. Surgical repair of anatomically corrected malposition of the great arteries. Ann Thorac Surg 1986;42:220-8.
10. Chen MR. Anatomically corrected malposition of the great arteries. Pediatr Cardiol 2008;29:467-8.