• 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.

• Journal of Veterinary Clinics
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• v.35 no.1
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• pp.26-29
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• 2018

A 6-month-old, female poodle presented with a three-month history of persistent regurgitation immediately after eating. On physical examination, the patient was emaciated and dehydrated. Thoracic radiography showed ventral displacement of the trachea and increased radiopacity in the mediastinum, cranial to the heart base. A severely dilated esophagus was identified cranial to the heart on esophagram. Computed tomography (CT) revealed the esophagus was filled with gas, fluid and a little of contrast and dilated from caudo-cervical to cranio-thoracic part. The esophageal diameter was markedly decreased at the heart base. In addition, the trachea was displaced to the left-ventral side of the right aortic trunk and an aberrant left subclavian artery originating from the aorta was identified. There was no evidence of abdominal vascular anomaly. Based on diagnostic imaging, persistent right aortic arch (PRAA) with an aberrant left subclavian artery was diagnosed. The patient did not undergo surgery and died at 15 days after diagnosis. This report describes imaging diagnosis, including CT and radiography in a weaned dog with regurgitation due to esophageal obstruction by PRAA. When PRAA is suspected and conventional radiography or contrast study is insufficient for diagnosis, CT may be helpful for diagnosing PRAA.

• Clinical and Experimental Pediatrics
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• v.55 no.1
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• pp.29-33
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• 2012

PHACE association is a rare neurocutaneous condition in which facial hemangiomas associate with a spectrum of posterior fossa malformations, arterial cerebrovascular anomalies, cardiovascular anomalies, and eye anomalies. We reported a case of PHACE association in a premature infant showing facial, intracranial, and oropharyngeal hemangiomas with evidence of the Dandy-Walker variant and complicated cardiovascular anomalies, including a right-sided aortic arch and an atypical patent ductus arteriosus arising from a tortuous left subclavian artery. To our knowledge, intracranial hemangiomas are rare in PHACE association, and a concomitant oropharyngeal hemangioma has not been previously reported in the PHACE association literature. In infants presenting with large, plaque-like facial hemangiomas, it is important to conduct active cardiovascular and neurological evaluations. Special attention should be given to the laryngoscopic examination to search for additional hemangiomas in the airway.

• Journal of Chest Surgery
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• v.17 no.3
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• pp.448-455
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• 1984

Coarctation of the Aorta is a congenital constriction of aorta of varying degree, usually located at or near the aortic ismuth with frequent associations of other cardiac anomalies. Various modes of surgical corrections, such as resection and end-to-end anastomosis, graft interposition, angioplasty using prosthetic patch or subclavian flap have been used according to the status of coarctation and age of the patient. We have experienced two cases of surgically treated coarctation of the aorta, one of which was preductal coarctation with hypoplastic aortic arch and ventricular septal defect in a 4 year old boy, and the other case was juxtaductal type with aortic regurgitation. Subclavian flap angioplasty with additional pulmonary artery banding procedure was done in the first case and wedge resection with end-to-end anastomosis and aortic valve replacement [St. Jude valve, 23mm] 20 days later of first operation in the other case. The first case developed massive tarry stool on 3rd POD, probably due to mesenteric arteritis with resultant bowl ecrosis, and expired the next day. Recovery was uneventful with the second case.

• Journal of Chest Surgery
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• v.26 no.1
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• pp.36-43
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• 1993

Sixty four children [aged 2 days to 9 years] , 58 with complete transposition of the great arteries, 5 with Taussig-Bing double outlet right ventricle, and 1 with double outlet left ventricle plus left ventricular type single ventricle, have undergone anatomic correction from November 1987 to August 1992. Eleven underwent previous operations: pulmonary artery banding[7], modified Blalock-Taussig shunt[2], coarctoplasty[2], aortic arch reconstruction[1] . Of 58 patients with TGA, Type A coronary arteries of Yacoub were seen in 50[86%]. U-shaped coroanry arterial flaps were transfered to the neoaorta using trap door technique, and neopulmonary arterial tract was constructed using glutaraldehyde fixed autopericardium with Lecompte maneuver. There were 18 hospital deaths [28.1%] with no late mortality. Mean follow-up of 20.4\ulcorner11.9 months were achieved in all survivors. Postoperative cardiac catheterizations were done in 14 cases. Mean pressure gradients of pulmonary and aortic outflow tract were 15.0 $\pm$2.6 and 4.2$\pm$1.4mmHg, mild aortic valve insufficiencies were found in 2, and mean cardiac index was 5.18$\pm$0.19 L/min/M2. We conclude that we should continue anatomic correction for the complex congenital heart anomalies with the malposition of the great arteries because myocardial function seems to be well preserved, though we are still on the learning curve.

• Journal of Chest Surgery
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• v.19 no.1
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• pp.165-173
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• 1986

During the 4 year period from 1982 through 1985, twelve patients have undergone operations for discrete subaortic stenosis with good short-term clinical result at Department of Thoracic and cardiovascular Surgery, S.N.U.H. According to the cineangiographic and operative findings, nine of the 12 patients were classified as Deutsch type I, the other 3 as type II, and eleven of the 12 had one or more associated anomalies of the cardiovascular system such as PDA[5], VSD[5], left SVC[2], MS[1], COA[1], supramitral membrane[1], DORY[1], right aortic arch[1], DCRV[1], and TOF[1] [one with Shone`s complex], and three of them had secondary cardiac disorders such as aortic regurgitation[3],mitral regurgitation[2], and tunnel shaped dynamic obstruction of left ventricular outflow tract[2]. We have performed membrane resection via oblique aortotomy with retraction of the aortic cusps in 7 cases and via VSD from right cardiac chamber in 5 cases with large VSD and have also performed the operations on the correctable associated anomalies. There was only one operative death in patient with associated TOF due to neurologic complication and no other postoperative difficulties except in one patient with transient heart block resolved spontaneously on postoperative 3rd day. To our knowledge, this article is the first report of operation for discrete subaortic stenosis in Korean literature.

• Journal of Chest Surgery
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• v.30 no.4
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• pp.363-372
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• 1997

One hundred forty-four patients underwent operation for coarctation of the aorta at Seoul National University Children's Hospital between June 1986 and Decembsr 1995. Age ranged 0.1 to 191 months. Of these 78.5%(113) were infants. We classified the patients in terms of the anatomic location of coarctatiln and the associatCd anomalies(I[401= primary coarctation, 11(741=isthmic hypoplasia, lIIf30)=tubular hypoplasia involving transverse arch, Ar63 =with ventricular septal defect, B(28)=with other major cardiac defects). Subcalvian flap coarctoplasty(60), resection & anastomosis(44), extended aortoplasty(26), and onlay patch(14) were used as surgical methods. Overall operative mortality was 16.0(23/144)%. The hospital mortality was signific'antly higher in patheints with type 111, subtype B, younger age(under 3 months), extended aortoplasty(p(0.01). However, one-stage total repair in patients with subtype A or B were not found to be a predictor of hospital death. Restenosis had occured in 18 patients among 121 survivals(14. 9%). The mean follow-up period was 29.1 $\pm$28.8(0~129.2) months. Preoperative, immediate postoperative(within 3 months after operation) and postoperative(later than 6 months after operation) echocardiographic data on the dimensions of ascending aorta(AA), transverse arch(TA), an4 aortic isthmus(Al) were available in 77 patients(I=20, ll=42, 111= 15). Preoperative and postoperative aortic isthmus(All) and tra sverse arch indices(TAI), defined as TAIAA & AIIAA respectively, were compared. Immediate postoperative All in type 1, II and TAI in type 111 were significantly smaller in stenotic than non-stenotic group suggesting incomplete relieves of stenotic segment Younger age, subclavian coarctoplasty in patient under 3 months of age were round to be the risk factors for restenosis in this series. In conclusion, We found that aortic arch index and transverse arch index can be a useful tool to figure out the anatomic and clinical characteristics of the patients with aortic coarctation, and that anatomy, associated anomalies, age, and surgical methods may influence the surgical outcome of the coarctation repair.

• 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.23 no.2
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• pp.253-259
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• 1990

Between January, 1970 and August, 1989, a total of 81 patients whose age were more than 20 years of life, received total correction for tetralogy of Fallot. This report analyzed 70 patients among them and excluded the remaining 11 patients whose clinical data could not be found. Their mean age was 25.750.39 years[range 20 \ulcorner50]. The clinical manifestations were cyanosis and clubbing [64 pts], frequent URI[40 pts], anoxic spell [19 pts], infective endo-carditis[4 pts], brain abscess[3 pts], pulmonary tuberculosis[3 pts] and CHF, chest tightness, nephrotic syndrome, left hemiplegia, and tamponade. The types of right ventricular outflow tract obstruction were combined[46 pts], pure infundibular [21 pts] and pure valvular[3 pts]. Associated cardiovascular anomalies were PFO [27 pts], ASDi8 pts], LSVC[8 pts], aortic regurgitation [5 pts], right aortic arch, coronary artery anomalies, PDA and dextrocardia. Hospital mortality was 5.7%. The causes of death ware low cardiac output [2 pts], aggravation of CRF[1 pts] and brain damage[1 pts]. There was one late death because of residual intracardiac shunt and congestive heart failure. During the follow-up period, 16 patients were lost and the remaining 49 patients were asymptomatic and leading normal lives. Residual intracardiac shunt was detected in 5 patients with radionuclide single pass study but all of them had Qp / Qs ratio less than 1.5.

• Journal of Chest Surgery
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• v.42 no.6
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• pp.749-756
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• 2009

Background: Vascular ring is a rare anomaly of the aortic arch. We did surgical repair procedures on 16 cases of vascular ring over the past 12 years. This article reviews our results. Material and Method: Between 1995 and 2007, 16 patients (5 with double aortic arch, 7 with right aortic arch-left ligamentum, 4 with pulmonary artery sling) underwent surgical repair. Mean age at the time of the operation were as follows: double aortic arch, $5.7{\pm}5.5$ years; right aortic arch-left ligamentum, $6.1{\pm}13.4$ years; pulmonary artery sling, $2.9{\pm}2.6$ years. Five patients (71%) with right aortic arch-left ligamentum had an associated Kommerell's diverticulum. Two patients (40%) with double aortic arch, 2 patients (28.6%) with right aortic arch-left ligament and 4 patients (100%) with pulmonary artery sling had associated airway stenosis. Cardiac anomalies were present in 8 of 16 patients. Result: There was no peri-operative or post-operative mortality. The mean hospital stay was $27.1{\pm}38.2$ days. None of our patients underwent reoperation. Conclusion: Vascular ring is rare, but, it needs surgical correction. It is important to suspect the diagnosis and to validate with echocardiography. Preoperative and postoperative computed tomography and bronchoscopy are useful to evaluate the airway and surrounding structures.