• Journal of Chest Surgery
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• v.34 no.10
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• pp.775-780
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• 2001

We report a case of the successful anatomical correction of the Taussig-Bing anomaly associated with the interrupted aortic arch and intramural left coronary artery for an 38 day-old infant Aortic arch and neoaortic reconstructions were conducted without any prosthetic or pericardial patch. Intramural left coronary was separated from right one after partial detachment of aortic commissure and both coronary artery buttons were transferred separately to the proximal main pulmonary artery(nee-aorta). Delayed sternal closure was done 3 days after the operation and hospital discharge was delayed for a month because of postoperative pneumonia. Now he is 5 months old and free of symptoms and cardiac drugs.

• Journal of Chest Surgery
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• v.37 no.10
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• pp.856-860
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• 2004

Interrupted aortic arch with concomitant intracardiac defects is a rare congenital anomaly that has an unfavorable natural course. We report a successful staged operation of interrupted aortic arch with apical muscular ventricular septal defect associating esophageal atresia with tracheoesophageal fistula in a 3-day-old neonate weighing 2.6 kg. We repaired esophageal atresia through the right thoracotomy and subsequently performed extended end-to-end anastomosis of the aortic arch with pulmonary artery banding through the left thoracotomy at same operation. The apical muscular VSD was repaired 87 day after first operation. The patient required multiple additional interventions before closure of the apical muscular ventricular septal defect, such as pyloromyotomy for idiopathic hypertrophic pyloric stenosis, anterior aortopexy for airway obstruction, and balloon aortoplasty for residual coarctation. She is now doing well.

• 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.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.30 no.1
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• pp.83-87
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• 1997

Taussig-Bing anomaly is infrequently associated with interrupted aortic arch and size discrepancy of great arteries makes it difficult to undergo arch reconstruction and arterial switch operation. A 20-day old male infant was admitted with the diagnosis of Taussig-Bing anomaly with type B Interrupted aortic arch. Multi-organ failure, due to the diminution of ductal flow, was stabilized after 3 weeks of prostaglandin El and controlled ventilatory support. The surgical correction consisted of VSD closure, arterial swtich and extended aortic arch reconstruction. The marked disparity between the hypoplastic ascending aorta and the dilated main pulmonary artery was overcome by constructing distal neoaorta using both native ascending and descending aortic tissue. The patient was extubated on postoperative 2nd day Postoperative catheterization showed no left ventricular outflow obstruction, no intracardiac shunt, and no incompetence of neoaortic valve.

• 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.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.

• Journal of Chest Surgery
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• v.39 no.6 s.263
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• pp.434-439
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• 2006

Background: Deep hypothermic circulatory arrest during repair of aortic arch anomalies may induce neurological complications or myocardial injury. So we surveyed if the regional cerebral and myocardial perfusion might eliminate those potential side effects. Material and Method: From March 2000 to December 2004, 62 neonates or infants with aortic arch anomaly underwent one stage biventricular repair using the regional perfusion technique by single surgeon. Preoperative diagnosis of the arch anomaly consisted of coarctation (n=46), interruption of the aorta (n=12), hypoplastic left heart syndrome (n=2) and truncus areteriosus (n=2). Combined anomalies were ventricular septal defect (n=51), TAPVR (n=1), PAPVR (n=1) and atrioventricular septal defect (n=2). Arterial cannula was inserted at the innominate artery. Result: The mean regional perfusion time of brain was $28{\pm}10min$. Operative mortality rates was 0 (0/62). Late death was 1 (1/62) during $11{\pm}7$ months of follow-up. Neurologic complications consisted of transient chorea in 1 case. There was no reoperation associated with arch anolamy. Pulmonary complication associated with arch repair occurred in f case which was managed by aortopexy. Conclusion: One-tage rch repair using the regional profusion is safe and effective in minimizing the neurologic and myocardial complications.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.547-551
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• 2001

The effects of deep hypothermia and circulatory arrest during aortic arch reconstruction are associated with potential neurologic and myocardial injury. We describe a surgical technique that two patients underwent a modified Norwood procedure without circulatory arrest and myocardial ischemia. One was 13-day-old female patient, weighing 3.1kg, having a variant of hypoplastic left heart syndrome and another was 38-day-old male patient, weighing 3.4 kg, diagnosed Taussig-Bing anomaly with severe aortic arch hypoplasia, coarctation of the aorta, and subaortic stenosis. The arterial cannula was inserted in innominate artery directly. During Norwood reconstruction, regional high-flow perfusion into the inominate artery and coronary perfusion were maintained and there were no neurologic, cardiac, and renal complications in two patients. This technique may help protect the brain and myocardium from ischemic injury in patients with hypoplastic left heart syndrome or other arch anomalies including coarctation or interruption.

• 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.