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

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

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

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.

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.