Diagnosis, Treatment and Outcomes of Patients with Aortopulmonary Window

Abstract

Background: Aortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary artery in the presence of two separate semilunar valves and is the rarest of septal defects. Aims: To present our experience with the diagnosis and outcome of APW cases. Study Design: Retrospective cohort study. Methods: Between June 2003 and October 2011, thirteen patients were diagnosed with APW. Clinical features of patients, findings of echocardiographic and angiographic examination, results of surgical intervention and follow-up were reviewed retrospectively. Results: Eleven children (10 days to 16 years), underwent surgical correction of APW. In a 12-month-old boy, the defect was repaired by the transcatheter approach. In addition to APW repair, closure of VSD was performed in 2 patients. APW were associated with interruption in two patients; one also had a complex pathology. None of the patients died due to complications of surgical or transcatheter procedures. After a median follow-up period of 40 months, the patients were asymptomatic and none of them required additional medication, except for the patient with complex pathology including an interrupted aortic arch, who underwent balloon angioplasty for recoarctation. Conclusion: In any infant with the findings of congestive heart failure and failure to thrive, APW must be kept in mind as a differential diagnosis. In isolated APW cases before 6 months of age, echocardiography is often sufficient for diagnosis. In complex cases, cardiac catheterisation is performed for the comprehensive evaluation of associated defects. After 6 months, cardiac catheterisation could be utilised to perform vasoreactivity testing and, if possible, to close the defect. Turkish Başlık: Aortopulmoner Pencereli Olguların Tanı ve Tedavi Sonuçlarının Değerlendirilmesi Anahtar Kelimeler: Aortopulmoner pencere, pulmoner hipertansiyon Arka Plan: Aortopulmoner pencere; iki ayrı semilunar kapak varlığnda asendan aorta ile pulmoner arter arasında bağlantı olmasıdır ve septal defektlerin en nadir görülen tipidir. Amaç: Bu çalışmada kliniğimizde tanı almış aortopulmoner pencereli olgularımızın tanı ve tedavi sonuçlarını değerlendirmeyi amaçladık. Çalışma Tasarımı: Retrospektif kohort çalışma Yöntemler: Haziran 2003 ile Ekim 2011 tarihleri arasında 13 olgu aortopulmoner pencere tanısı aldı. Bu olguların dosyalarından başvuru sırasındaki klinik özellikleri, ekokardiyografik ve kalp kateterizasyonu bulguları ile cerrahisonuçları geriye dönük olarak gözden geçirildi. Bulgular: Yaşları 10 gün ile 16 yaş arasında değişen 11 olguya aortopulmoner pencere tanısıyla cerrahi girişim uygulandı. 12 aylık bir olguda defekt transkateter yoldan kapatıldı. Cerrahi tamir sırasında 8 olguda yapay, 3 olguda perikardiyal yama kullanıldı. Aortopulmoner pencere tamiri sırasında 2 hastada VSD, 2 olguda da kesintili arkus aorta tamiri yapıldı. Cerrahi ya da transkateter yoldan tedavi edilmiş hiçbir olgu kaybedilmedi. Postoperatif dönemde 3 olguda önemli pulmoner hipertansiyon nedeniyle nitrik oksit ve iloprost kullanımı gerekti. İzlemde kesintili arkus aorta nedeniyle cerrahi tamir uygulanmışolguya rekoarktasyon nedeniyle balon angioplasti yapıldı. Median 40 aylık süredetakip edilen diğer olgular semptomsuz ve iyi klinik durumda izlenmektedir. Sonuç: Büyüme gelişme geriliği ve kalp yetersizliği bulguları olan infantlarda aortopulmoner pencere ayırıcı tanıda düşünülmelidir. Altı aydan küçük izole defekte sahip olgularda ekokardiyografi tanı için yeterli bir yöntemdir. Kompleks olgularda kardiyak anatominin değerlendirilmesi, altı aydan büyük olgularda operabilitenin değerlendirilmesi ve uygun olgularda defektin transkateter yoldan kapatılması için kalp kateterizasyonu gerekebilir.

Keywords

• Aortopulmonary window, pulmonary hypertension, aortopulmonary septal defect

Diagnosis, Treatment and Outcomes of Patients with Aortopulmonary Window

Öz

References

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