• Advances in pediatric surgery
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• v.8 no.2
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• pp.161-165
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• 2002

This is a case of tracheomalacia associated with esophageal atresia. An 11-month-old- male boy presented with a life-threatening apneic spell after correction of esophageal atresia (Gross type C). After complete exclusion of the other possible causes of the apneic spell, the presumptive diagnosis of tracheomalacia was made with fluoroscopy and 3-dimensional chest CT. The final diagnosis was made with rigid bronchoscopy under spontaneous respiration. The aortopexy was performed with intraoperative bronchoscopic examination. The postoperative period was unremarkably uneventful. The patient was discharged 9 days after the aortopexy and has remained well to date (5 months after the aortopexy).

• Journal of Chest Surgery
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• v.48 no.3
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• pp.217-219
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• 2015

Postpneumonectomy syndrome (PPS) is a rare late complication of pneumonectomy. It occurs more often in children than in adults, and is characterized by respiratory failure resulting from bronchial compression caused by severe mediastinal shift. Various methods have been used to treat PPS, including aortopexy and the insertion of plastic balls, silastic implants, and saline-filled breast prostheses. We describe two cases of PPS corrected with tissue expanders after right pneumonectomy in patients with esophageal atresia.

• Korean Journal of Bronchoesophagology
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• v.10 no.2
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• pp.63-67
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• 2004

Tracheomalacia can be a life threatening upper air way obstructive disease in an infant and vascular rings can be also a major rare cause of tracheoesophageal obstruction. These two rare entities can be combined in one patient because the vascular ring can cause secondary tracheomalacia during development of fetus. The diagnosis of this combination and adequate surgical correction is occasionally difficult. This is a report of an infant who had not diagnosed tracheomalacia associated with vascular ring until 5 months of age because of the prolonged tracheal intubation. The rigid bronchoscopic examination performed under impression of tracheomalacia revealed a concentric tracheal collapse, an unusual bronchoscopic findings of tracheomalacia, which raised a suspicion of the tracheal compression by vascular rings. The 3-D reconstructive DT aortography clearly demonstrated the double aortic arch. The patient was treated surgically by simple division of the left aortic arch and aortopexy with good result. The vascular ring such as double aortic arch should be considered during the diagnosis of tracheomalacia in infants. If the tracheomalacia is associated with vascular ring, simultaneous surgical correction should be performed.

• 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.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.

• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.737-744
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• 2005

Purpose : Infants and children with cardiovascular diseases often present with respiratory symptoms. However, missed or delayed evaluation for potential airway problem may complicate overall prognosis. The aim of this study is to determine the clinical characteristics of these patients and explore the cause of airway problem. Methods : We reviewed the medical records of 64 patients(M : F=33:31, mean age : $6.3{\pm}7.5$ months) whose airway problems were proven by computed tomography or bronchoscopy in perioperative periods at the Asan Medical Center from January 1997 to June 2004. Patients were divided into two groups based on the duration of ventilator care : ${\leq}7$ days(group 1 : 23 cases, M : F=10 : 13) and >7 days(group 2 : 41 cases, M : F=23 : 18). Results : The patients in group 2 significantly developed more post-operative respiratory symptoms than group 1(P<0.001) and had more airway problems including extrinsic obstruction, intrinsic anomaly, and combined problem than group 1 although not significantly different(P=0.082). Among underlying diseases, the most common diseases were vascular anomaly(26.2 percent) and aortic arch anomaly(26.2 percent) in group 1 and pulmonary atresia with ventricular septal defect(22.4 percent) in group 2. The most frequent respiratory symptoms were recurrent wheezing pre-operatively and failure of ventilator weaning post-operatively. The major types of airway anomaly were tracheomalacia and tracheal stenosis(in each case 18.2 percent). Nineteen patients with persistent airway problems underwent aortopexy or other vascular correction. Of the 19 patients, 13(68.4 percent) were improved, but 2 failed in weaning ventilator and 4 died of non-airway problems. Conclusion : Early evaluation and treatment for potential airway problems may affect natural or surgical prognosis in patients with cardiovascular diseases presenting with respiratory symptoms.

• Journal of Chest Surgery
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• v.40 no.3 s.272
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• pp.193-199
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• 2007

Background: Reoperation is usually required for a right ventricle to pulmonary artery conduit obstruction caused by valve degeneration, conduit peel formation or somatic growth of the patient. An autologous tissue reconstruction (peel operation), where a prosthetic roof is placed over the fibrotic tissue bed of the explanted conduit, has been used to manage conduit obstructions at our institute since May 2002. Herein, the early and midterm results are evaluated. Material and Method: Between May 2002 and July 2006, 9 patients underwent obstructed extracardiac conduit replacement with an autologous tissue reconstruction, at a mean of 5.1 years after a Rastelli operation. The mean age at reoperation was $7.5{\pm}2.4$ years, ranging from 2.9 to 10.1 years. The diagnoses included 6 pulmonary atresia with VSD, 2 truncus arteriosus and 1 transposition of the great arteries. The preoperative mean systolic gradient was $88.3{\pm}22.2mmHg$, ranging from 58 to 125 mmHg. The explanted conduits were all Polystan valved pulmonary conduit (Polystan, Denmark). A bioprosthetic valve was inserted in 8 patients, and a monocusp ventricular outflow patch (MVOP) was used in 1 patient. The anterior wall was constructed with a Gore-Tex patch (n=7), MVOP (n=1) and bovine pericardium (n=1). Pulmonary artery angioplasty was required in 5 patients and anterior aortopexy in 2. The mean cardiopulmonary bypass time . was 154 minutes, ranging from 133 to 181 minutes; an aortic crossclamp was not performed in all patients. The mean follow-up duration was 20 months, ranging from 1 to 51 months. All patients were evaluated for their right ventricular outflow pathway using a 3-D CT scan. Resuit: There was no operative mortality or late death. The mean pressure gradient, assessed by echocardiography through the right ventricular outflow tract, was 20.4 mmHg, ranging from 0 to 29.6 mmMg, at discharge and 26 mmHg, ranging from 13 to 36 mmHg, at the latest follow-up (n=7, follow-up duration >1 year). There were no pseudoaneurysms, strictures or thrombotic occlusions. Conclusion: A peel operation was concluded to be a safe and effective re-operative option for an obstructed extracardiac conduit following a Rastelli operation.