• Journal of Chest Surgery
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• v.39 no.4 s.261
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• pp.275-280
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• 2006

Background: Aortopulmonary window (APW) is a very rare congenital heart anomaly, often associated with other cardiac anomalies. It causes a significant systemic to pulmonary artery shunt, which requires early surgical correction. Accurate diagnosis and surgical correction will bring good outcomes. The purpose of this study was to describe our 20-year experience of aortopulmonary window. Material and Method: Between March 1985 and January 2005, 16 patients with APW underwent surgical repair. Mean age at operation was $157.8{\pm}245.3$ ($15.0{\sim}994.0$) days and mean weight was $4.8{\pm}2.5$ ($1.7{\sim}10.7$) kg. Patent ductus arteriosus (8), atrial septal defect (7), interruptedaortic arch (5), ventricular septal defect (4), patent foramen ovate (3), tricuspid valve regurgitation (3), mitral valve regurgitation (2), aortic valve regurgitation (1), coarctation of aorta (1), left superior vena cavae (1), and dextrocardia (1) were associated. Repair methods included 1) division of the APW with primary closure or patch closure of aorta and pulmonary artery primary closure or patch closure (11) and 2) intra-arterial patch closure (3). 3) Division of the window and descending aorta to APW anastomosis (2) in the patients with interrupted aortic arch or coarctation. Result: There was one death. The patient had 2.5 cm long severe tracheal stenosis from carina with tracheal bronchus supplying right upper lobe. The patient died at 5th post operative day due to massive tracheal bleeding. Patients with complex aortopulmonary window had longer intensive care unit and hospital stay and showed more morbidities and higher reoperation rates. 5 patients had reoperations due to left pulmonary artery stenosis (4), right pulmonary artery stenosis (2), and main pulmonary artery stenosis (1). The mean follow-up period was $6.8{\pm}5.6$ (57.0 days$\sim$16.7 years)years and all patients belonged to NYHA class 1. Conclusion: With early and prompt correction of APW, excellent surgical outcome can be expected. However, optimal surgical method needs to be established to decrease the rate of stenosis of pulmonary arteries.

• Journal of Chest Surgery
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• v.35 no.5
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• pp.397-401
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• 2002

The combination of interrupted aortic arch and aortopulmonary window is a rare presentation of congenital heart disease, which requires early diagnosis and surgical treatment. We describe a successful one-stage repair of the anomaly through median sternotomy in a 10-day-old neonate weighing 2.46 kg.

• Journal of Chest Surgery
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• v.37 no.11
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• pp.918-921
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• 2004

Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.

• Journal of Chest Surgery
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• v.41 no.4
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• pp.499-503
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• 2008

Berry syndrome (a distal aortopulmonary window and a right pulmonary artery originating from the ascending aorta, an intact ventricular septum, a patent ductus arteriosus and an interrupted aortic arch) is a rare complex congenital cardiac malformation. We describe a case of one stage repair with using an autologous arterial flap in a 19-day-old neonate who was suffering with this malformation, and we report on the development of postoperative right pulmonary artery stenosis and its successful management with performing multiple balloon angioplasties.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.291-300
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• 2005

One-stage repair of aortic arch anomalies and intracardiac defects through median sternotomy has been recently adopted by many institutions since it is known to be safer than the staged repair. The early and midterm results of the one-stage repair of aortic arch anomalies and intracardiac defects were retrospectively evaluated. Material and Method: 45 patients who underwent one-stage repair of aortic arch anomalies and intracardiac defects performed by one surgeon from January 1996 to July 2003 were included in this retrospective study. The median age of repair was 16 days (range, 3 days$\~$23.7 months) and the mean weight was $3.62\;\pm\;1.30 kg$. Thirty one $(68.9\%)$ had coarctation and 14 $(31.1\%)$ had interrupted aortic arch. Associated intracardiac anomalies were VSD in 31 patients (VSD group), TGA or Taussig-Bing anomaly in 10 (TGA group), and others in 4 (ASD in 1, aortopulmonary window 1, truncus arteriosus 1, aortic and mitral stenoses 1, miscellaneous group). The arch obstruction was repaired with end-to-side anastomosis in 23 patients and end-to-end anastomosis in 22. Result: Overall postoperative hospital mortality was $22.2\%\;(10/45);\;16.1\%$ (5/31) in VSD group, $40\%$ (4/10) in TGA group, and$25\%$ (1/4) in miscellaneous group. There was no mortality in VSD group since 1998, and the mortality in TGA group has remarkably reduced since technical modification for coronary transfer was adopted $(75\%\;vs\;16.7\%)$. There was no postoperative seizure or other neurological complications. Postoperative aortic restenosis occurred in 5 patients $(5/35,\;14.3\%)$. Two patients underwent balloon aortoplasty with successful results. There was no reoperation. There was one late death caused by pneumonia 5 months after the operation. Two-year actuarial survival rate including operative death was $72.9\%$. Conclusion: The operative mortality of one-stage repair has been reduced with time and aortic restenosis rate was also acceptable. We concluded that this procedure is a reproducible procedure for aortic arch anomalies associated with intracardiac defects.