• 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.38 no.12 s.257
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• pp.856-859
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• 2005

A male neonate was referred to our hospital with facial cyanosis and tachypnea at 19 days of age. Two-dimensional echocardiography showed type B interrupted aortic arch, posterior malalignment ventricular septal defect and valvular aortic stenosis. A new surgical repair was done with biventricular repair and neo-aortic arch reconstruction. Left ventricular outflow track (LVOT) was consisted of aortic valve and pulmonic valve. Right ventricular outflow (RVOT) track was reconstructed with extracardiac conduit. Postoperative two-dimensional echocardiography showed no stenosis and turbulency flow on LVOT and RVOT.

• Journal of Chest Surgery
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• v.21 no.4
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• pp.665-671
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• 1988

Between 1981 and 1987, five patients with an interruption of the aortic arch were operated upon. All had a ventricular septal defect and a patent ductus arteriosus as associated anomalies. A two-stage procedure was employed in these cases, the initial procedure being repair of the interrupted arch, ligation of the patent ductus arteriosus, banding of the main pulmonary artery and a lung biopsy which was followed, 5 to 49 months later, by the repair of the ventricular septal defect. Four patients completed the two-stage procedure with one postoperative mortality. The remaining patient is yet to complete the second stage procedure.

• Journal of Chest Surgery
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• v.21 no.3
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• pp.588-593
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• 1988

We experienced a case of interrupted aortic arch[Type A] associated with PDA, VSD, mitral regurgitation and single coronary artery. The patient was 7 years old boy, who showed congestive heart failure[NYHA functional class III]. One stage total correction was performed under profound hypothermia with total circulatory arrest. Aortic continuity was established using PDA with anterior wall of main pulmonary artery flap. VSD was closed with Dacron patch and mitral regurgitation repaired by Reed`s annuloplasty method. The postoperative cardiac catheterization revealed no pressure gradient between ascending aorta and descending aorta, decreased pulmonary artery pressure and trivial residual shunt[Qp/Qs: 1.28]. The aortogram showed good continuity of the aorta without narrowing of the anastomotic site. During the period of 1 year follow up, heart failure symptoms were nearly subsided.

• Journal of Chest Surgery
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• v.38 no.12 s.257
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• pp.852-855
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• 2005

A 85-day-old infant was successfully operated on for truncus arteriosus (type I) with interrupted aortic arch (type A) using one-stage anterior approach without circulatory arrest. Aortic arch was reconstructed by direct anastomosis of ascending aorta and descending aorta with regional perfusion and continuity of right ventricle to pulmonary artery was established with $Shelhigh^{circledR}$ pulmonic conduit. The patient experienced left bronchus compression by descending aorta immediately postoperatively, which was improved with positional change and physiotherapy. The patient had reoperation due to stenosis of valved conduit at 13 months later. The patient is currently well under follow-up of 14 months from initial repair.

• Journal of Chest Surgery
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• v.28 no.6
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• pp.610-618
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• 1995

Three neonates with interrupted aortic arch with VSD underwent one stage repair using revised technique of cardiopulmonary bypass with short period of circulatory arrest. A left posterolateral thoracotomy was made to permit mobilization of the descending aorta and placement of polytetrafluoroethylene[PTFE graft for distal aortic perfusion. Then the patient was placed in the supine position and a median sternotomy was performed to permit the proximal dissection, VSD repair, and direct anastomosis between the ascending aorta and descending aorta. This technique has advantages to facilitate direct anastomosis between the ascending aorta and the descending aorta, to lessen circulatory arrest time, and to prevent dangerous laceration and post-operative narrowing of the thin small ascending aorta at cannulation site. There was no operative mortality but postoperative stenosis developed in one case which was relieved with balloon aortoplasty.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.360-370
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• 1985

A case of complete interruption of aortic arch with aortopulmonary window, patent ductus arteriosus, and aberrantly originated right subclavian artery from proximal descending aorta, in a four year old boy is reported in detail. This is the only reported case in Korea, who has had a successful one-stage total anatomical correction of this combination of defects. Under deep hypothermia and total circulatory arrest, aortic continuity was established using patent ductus arteriosus and anterior wall of pulmonary artery, which was anastomosed obliquely to anteromedial side of ascending aorta. Aortopulmonary window was closed using Impra patch via pulmonary arteriotomy. Then pulmonary arteriotomy was reconstructed primarily except at the junction of right pulmonary artery and main pulmonary artery, where a small piece of pericardium was used to close the defect to prevent kinking and narrowing of right pulmonary artery. Postoperative cardiac catheterization demonstrated a good reconstruction.

• 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.24 no.7
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• pp.693-700
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• 1991

A rare case of the association of distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and interrupted aortic isthmus in a 40-day-old infant is reported. The infant was suffered from two operations with an interval of nine days. At the first operation a 10mm polytetrafluoroethylene prosthesis was inserted instead of the interrupted aortic isthmus and ductus was ligated via the left posterolateral thoracotomy. But the patient could not be weaned from the respirator because of large amount of left-to-right shunt. So the total correction was subsequently performed after an interval of nine days. At the second operation, tunneling of the right pulmonary artery to the main pulmonary artery through the aortopulmonary septal defect was performed using the Dacron patch via a longitudinal transaortic approach and a separate autologous pericardial patch was applied to the longitudinally incised margins of the anterior wall of the ascending aorta. The second postoperative course was relatively uneventful except some respiratory distress and nutritional problems. Now he is at 6 months of age and thrives well without any symptom. Because the success of the surgical repair of this complex anomalies depends upon the accurate diagnosis and meticulous design of each step of procedure prior to operation these problems are also discussed.

• Journal of Chest Surgery
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• v.39 no.2 s.259
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• pp.99-105
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• 2006

Background: Interrupted aortic arch is a rare congenital heart anomaly which still shows high surgical mortality. In this study, we investigated the causes of and the risk factors for mortality to improve the surgical outcomes for this difficult disease entity. Material and Method: From 1984 to 2004, 42 patients diagnosed as IAA were reviewed retrospectively. Age, body weight at operation, preoperative diagnosis, preoperative PGE1 requirement, type of interrupted aortic arch, degree of left ventricular outflow stenosis, CPB time, and ACC time were the possible risk factors for mortality. Result: There were .14 hospital deaths. Preoperative use of PGE1, need for circulartory assist and aortic cross clamp time proved to be positive risk factors for mortality on univariate analysis. Preoperative left ventricular outflow stenosis was considered a risk factor for mortality but it did not show statistical significance (p-value=0.61). Causes of death included hypoxia due to pulmonary banding, left ventricular outtract stenosis, infection, mitral valve regurgitation, long cardiopulmonary bypass time and failure of coronary transfer failure in TGA patients. Conclusion: In this study, we demonstrated that surgical mortality is still high due to the risk factors including preoperative status and long operative time. However preoperative subaortic dimension was not related statistically to operative death statistically. Adequate preoperative management and short operation time are mandatory for better survival outcome.