Konjenital düzeltilmiş transpozisyonlu hastaların ekokardiyografik, kardiyak kateterizasyon ve cerrahi sonuçlarının orta dönem değerlendirilmesi: Tek merkez deneyimi

Midterm evaluation of echocardiographic examination, cardiac catheterization findings and surgical results of patients with congenitally corrected transposition of great arteries: A single center experience

Mustafa Orhan BULUT,İlker Kemal YÜCEL,Şevket BALLI,Emine HEKİM YILMAZ,Ayhan ÇEVİK,Ahmet ÇELEBİ

Özet

Kongenital düzeltilmiş büyük arter transpozisyonu, atriyoventriküler, ventriküloarteryal diskordans olarak tanımlanır. Aralık 1993-Ocak 2015 tarihleri arasında pediyatrik kardiyoloji bölümünde takip edilen 64 hasta retrospektif olarak değerlendirildi.Çalışmamız 79 hastadan oluştu. Hastalarımızın 46 (%58,2)'sı erkek, ortalama tanı yaşı 40±39,25 ay, ortalama takip süresi 56±39.4 ay idi. On üç (%16,5) hasta atriyal sitüs inversus idi. En sık eşlik eden anomali ventriküler septal defekt (%29,1) idi. Kırk beş hastada valvular ve/veya subpulmoner stenoz vardı. Hastaların %15,2'si pulmoner atrezik idi. Sistemik atriyoventriküler kapak yetersizliği 2 hastada ağır idi. Beş hastada (%6,3) Ebstein benzeri triküspid kapak yapısı belirlendi. Beş hastada ductal stent, 2 hastada sol ventrikül-pulmoner arter (PA) konduit anjiyoplasti, 1 hastada sağ ventrikül-PA konduit anjiyoplasti, 2 hastada pulmoner balon anjiyoplasti uygulandı. On altı hastaya Glenn anastomozu uygulandı. Hastaların %11,4'ünde kalıcı pil Kabul tarihi: 30.04.2017 implantasyonu gerektiren atriyoventriküler tam blok saptandı. Bu hastalarda farklı derecelerde anomalilerin eşlik etmesi nedeniyle ömür boyu takip ve tedavi prognostik önemlidir.

Abstract

Congenitally corrected transposition of the great arteries (cc-TGA), is also defined as double discordance and ventricular inversion. We conducted a retrospective evaluation of medical records of 79 patients diagnosed and followed-up with congenital transposition of great arteries between December 1993 and January 2014 at our pediatric cardiology department. Of 79 patients included in the study, 46 (58.2%) were male, mean age at diagnosis was 40±39.25 months and the mean follow-up time was 56±39.4 months. Thirteen (16.5%) patients had atrial situs inversus. The most common concomitant abnormality was found to be ventricular septal defet (29.1%). Fourty- five patients had pulmonary stenosis either in the form of valvular, subvalvular or both together. Twelve (15.2%) patients had pulmonary atresia. Systemic atrioventricular valve (AV) regurgitation was present in 51 (64.6%) patients, whereas 2 (2.5%) patients had severe systemic AV valve regurgitation. Ebstein-like AV valve was detected in 5 (6.3%) patients. Transcatheter interventions included ductal stent implantation (n=5; 6.3%), left ventriculopulmonary artery conduit angioplasty (n=2; 2.5%), RV-PA conduit angioplasty (n=1; 1.3%) and pulmonary balloon valvuloplasty (n=2; 2.5%). Eleven (13.9%) patients underwent interventional therapeutic cardiac catheterization. Glenn anastomosis was performed in 16 (20.3%) patients. Nine (11.4%) patients required a permanent pacemaker implantation.Individualization of treatments and managements for the patients is of prognostic importance for these patients needing lifelong follow-up due to the large number of accompanying anomalies and different degrees of abnormalities in cc-TGA patients.