Sait AŞLAMACI, İlkay ERDOĞAN, Murat ÖZKAN, Kahraman YAKUT, N. Kürşat TOKEL, Birgül VARAN

Aortopulmoner pencerenin tanı ve cerrahi tedavisi: Tek merkez deneyimimiz

Amaç: Bu çalışmada aortopulmoner pencereye ilişkin tek merkezli deneyimimiz sunuldu ve klinik bulgular, belirtiler, cerrahi düzeltme teknikleri ve uzun dönem sonuçlar incelendi. Çalışma planı: Hastanemizde Mayıs 1998 - Haziran 2016 tarihleri arasında aortopulmoner pencere tanısı ile izlenen toplam 30 hastanın tıbbi kayıtları retrospektif olarak incelendi. Hastaların klinik özellikleri, ekokardiyografik ve anjiyografik bulguları, cerrahi girişim sonuçları ve takip sırasında tıbbi sorunlar değerlendirildi. Bulgular: En sık görülen bulgu ve belirtiler üfürüm, dispne, taşipne, gelişim geriliği ve konjestif kalp yetmezliği bulguları idi. Cerrahi sırasındaki ortalama yaş 8.2±14.4 ay (dağılım 7 gün - 60 ay) idi. On sekiz (%60) hastaya ek kardiyak anomali eşlik etmekteydi. On bir hastada basit doğuştan kalp hastalığı, yedi hastada kompleks doğuştan kalp hastalığı vardı. Dört hasta Eisenmenger sendromu (n=3) ve kompleks doğumsal kalp hastalığı (n=1) nedeniyle ameliyat edilemedi. Cerrahi sonrası erken veya geç dönemde ölüm gözlenmedi. Ortalama takip süresi 6.4±4.8 yıl (dağılım 5 ay-16 yıl) idi. Aortopulmoner pencere onarımının yanı sıra, dokuz hastada (%34.6) cerrahi girişimi etkileyen ek kardiyak anomali düzeltildi. Üç yıl sonra bir hasta rezidüel aortopulmoner pencere ve bir diğer hasta pulmoner darlık (valvüler, supravalvüler) nedeniyle yeniden ameliyat edildi. Bu hastalardan birine iki yıl sonra pulmoner balon valvüloplasti uygulandı. Takip sırasında tekrar ameliyat oranı %7.7 (n=2) idi. Sonuç: Aortopulmoner pencere ekokardiyografik çalışma sırasında atlanabilen ve düşük cerrahi risk ile onarımı olanaklı olan nadir bir kardiyak anomalidir. Bu nedenle, pulmoner vasküler hastalık gelişmeden önce tanı konması ve tedavi edilmesi önemlidir.

Diagnosis and surgical treatment of aortopulmonary window: Our single-center experience

Background: In this study, we aimed to report our single-center experience in aortopulmonary window and review clinical signs, symptoms, surgical correction techniques, and long-term outcomes. Methods: We retrospectively reviewed the medical records of a total of 30 patients who were followed with the diagnosis of aortopulmonary window in our hospital between May 1998 and June 2016. The clinical characteristics of the patients, echocardiographic and angiographic findings, surgical treatment outcomes, and medical problems during follow-up were reviewed.Results: The most common signs and symptoms were murmur, dyspnea, tachypnea, growth retardation, and signs of congestive heart failure. The mean age at the time of surgery was 8.2±14.4 months (7 days to 60 months). Eighteen patients (60%) had additional congenital cardiac anomalies. Eleven patients had simple congenital heart diseases, and seven patients had complex congenital heart diseases. Four patients were unable to be operated due to Eisenmenger syndrome (n=3) and complex congenital heart disease (n=1). No early or late postoperative death was observed. The mean follow-up was 6.4±4.8 years (range, 5 months to 16 years). In addition to aortopulmonary window repair, an additional cardiac anomaly modifying surgical intervention was corrected in nine patients (34.6%). One patient was reoperated for residual aortopulmonary window and another patient for pulmonary stenosis (valvular, supravalvar) after three years. One of these patients underwent pulmonary balloon valvuloplasty after two years. The reoperation rate was 7.7% (n=2) during follow-up.Conclusion: Aortopulmonary window is a rare cardiac anomaly which may be overlooked by echocardiographic study, and which is amenable for repair with low-surgical risk. It is, therefore, imperative to diagnose and treat this condition, before pulmonary vascular disease develops.

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