Funda ÖZTUNÇ, Halil TÜRKOĞLU, Kaya SÜZER, Levent SALTIK, Gürkan ÇETİN, Tufan PAKER, Aydın AYTAÇ, İlhan GÜNAY, Ahmet ÖZKARA, Atıf AKÇEVİN, Tayyar SARIOĞLU, Ayşe SARIOĞLU, Murat MERT

Total anormal pulmoner venöz dönüş ve cerrahi yaklaşımlar: Altmış bir olguda deneyimimiz

Amaç: Total anormal pulmoner venöz dönüş tanısıyla ameliyata alınan hastalara yönelik cerrahi tedavi seçenekleri değerlendirildi. Çalışma planı: Çalışmaya total anormal pulmoner venöz dönüş tanısıyla düzeltme girişimi uygulanan 61 hasta (27 kız, 34 erkek; ort. yaş 10.7±17.9 ay; dağılım 6 gün-9 yaş) alındı. Olguların 27’si (%44.2) suprakardiyak, 24’ü (%39.3) kardiyak, dördü (%6.5) infrakardiyak, altısı da (%9.8) mikst tipteydi. Ameliyat sonrası takip süresi ortalama 38.8±28.1 aydı.Bulgular: Hastane mortalitesi tüm olgularda %22 (14 hasta) olarak hesaplandı. Son 19 olgudan sadece ikisi (%10.5) kaybedildi. En yüksek mortalite mikst tipte gözlendi (%33.3), bunu sırasıyla infrakardiyak (%25), kardiyak (%25) ve suprakardiyak (%18.5) tipler izlemekteydi. Yirmi sekiz hastada pulmoner venöz obstrüksiyon vardı. Pulmoner hipertansif kriz mortalite nedenleri içinde ilk sırada yer alıyordu. Yaşayan hastalar düzenli aralıklarla ekokardiyografik olarak kontrol edildi. Hastaların fonksiyonel kapasiteleri New York Heart Association (NYHA) sınıf I olarak saptandı.Sonuç: Bulgularımız, giderek artan cerrahi ve yoğun bakım deneyimleri ve hızla ilerleyen teknolojik gelişmelerle tanı olanaklarının artışına paralel olarak, total anormal pulmoner venöz dönüş patolojilerindeki korrektif cerrahi girişimlerde daha başarılı sonuçlar alındığı yönündedir.

Total anomalous pulmonary venous connection and surgical options: experience with 61 cases

Background: We evaluated the surgical options of repair in patients with total anomalous pulmonary venous connection (TAPVC). Methods: The study included 61 children (27 girls, 34 boys; mean age 10.7±17.9 months; range 6 days to 9 years) who underwent repair of TAPVC at our institution. The localization of the TAPVC was supracardiac in 27 patients (44.2%), cardiac in 24 patients (39.3%), infracardiac in four patients (6.5%), and mixed in six patients (9.8%). The mean followup period was 38.8±28.1 months.Results: The overall hospital mortality was 22% (n=14). Mortality rate for the last 19 patients was 10.5% (n=2). The highest incidence of mortality was seen in patients having the mixed type (33.3%) of TAPVC, followed by infracardiac (25%), cardiac (25%), and supracardiac (18.5%) types. Pulmonary venous obstruction was observed in 28 patients preoperatively. Pulmonary hypertensive crisis was the main reason for mortality. The surviving patients who were followed-up by regular echocardiographic examinations had a mean functional capacity of New York Heart Association (NYHA) class I.Conclusion: In parallel with enhanced experience with surgical and intensive care management and improved diagnostic methods, the success rate of corrective surgical procedures performed for TAPVC has considerably increased resulting in less mortality.

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