• Journal of Chest Surgery
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• v.26 no.2
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• pp.135-140
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• 1993

Supravalvular aortic stenosis is an uncommon, congenital narrowing of ascending aorta just above aortic valve. Eleven patients underwent an aortoplasty to relieve supravalvular aortic stenosis at Sejong General Hospital from July 1985 to December 1991. Age ranged from 5 to 14 years(median 9 years). There were 7 male and 4 female patients. Seven patients had characteristics of Williams' syndrome including elfin face and mental retardation. All patients had localized, hourglass type but 4 patients had atypical findings. Preoperative left ventricula-aortic pressure gradient ranged from 40 to 190 mmHg(mean 88 mmHg). To relieve severe supravalvular aortic stenosis, extended aortoplasty was used in 7 patients and standard aortoplasty in 4 patients. Postoperative pressure gradient ranged from 0 to 40 mmHg (mean 16.6 mmHg). Follow-up pressure gradient with Doppler ranged from 0 to 88mmHg(mean 32.5mmHg).

• Journal of Chest Surgery
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• v.33 no.8
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• pp.623-629
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• 2000

Background: There has been controversy over the prevalence of recoarctation in infants treated by subclavian flap aortoplasty(SFA) for coarctation of the aorta. To assess the rate of recurrence of coarctation after SFA, we reviewed the surgical results of SFA in infants with coarctation of the aorta. Material and method: Between 1986 and 1998, a total of 25 patients less than 1 year of age(12 neonates and 13 infants) underwent SFA for aortic coarctation. Age at operation was 3.0$\pm$3.0 months(mean $\pm$ standard deviation); mean weight was 5.0$\pm$1.4kg. Classic SFA was performed in 20 patients, reversed SFA in 2 patients, subclavian artery reimplantation in 2 patients and the combined resection-flap aortoplasty in one. Result: The aortic clamping time ranged from 20 to 88 minutes(mean 35.8 minutes). There were one operative death and two late deaths. There was no case of paraplegia or left arm ischemia in complications. Twenty-one(84%) of 24 hospital survivors were followed for 26.0$\pm$24.0 months. The risk of recoarctation in neonates(33.3%) was a little greater than infants(25.0%) without statistical significance. Conclusion: This study revealed that SFA resulted a relatively high incidence of recarctation in infants. It is desirable to select other methods of surgical treatment(combined resection-flap aortoplasty, extended end-to-end repair etc.) for severe isthmic coarctation or hypoplasia of the distal aortic arch in infants, instead of choosing SFA indiscriminately.

• Journal of Chest Surgery
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• v.30 no.11
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• pp.1069-1076
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• 1997

There remains controversy regarding the appropriate surgical treatment for coarctation of the aorta because of relatively high rate of recoartation and high mortality in the cases associated with complex anomalies. We evaluated 31 consecutive patients who underwent surgical repair of coarctation of the aorta from May 1992 through June 1996. Nineteen patients(61.3%) were neonates and 26(83.9%) were under three months. Nine patients did not have major associated anom lies(Group I), 15 patients had ventricular septal defect(Group II), and 7 patients had major complex anomalies(Group III). 35.5% of the patients had arch hypoplasia. Surgical procedures performed were as follows: extended end-to-end anastomosis in 17 patients, combined resection-flap procedure in 7 patients, and subclavian flap aortoplasty in 7 patients. Residual coarctation occurred in 7(25%) of 28 patients; 2 after subclavian (lap aortoplasty(2/6, 33.3%), none after combillrd resection-flap procedure(0/7, 0%), and 5 after extended end-to-end anastomosis(5/15, 33.3%). Higher incidence of residual coarctation was noticed in the group with arch hypoplasia. The incidence of postoperative coarctation at a mean follow-up of 20.5 months in survivals was 12.0%(3/25); 2 cases after subclavian flap aortoplasty(2/6, 33.3%), none after combined resection-flap procedure(017, 0%), and one after endtoend anastomosis(1/12, 8.3%). The mortality rate related to coarctation repair was 9.7%(3 patients all in Group III). This study revealed that isolated coarctation of aorta and coarctation with ventricular septal di3fect(groups I & ll) can be repaired with low mortality, but repair of coarctation with complex anomaly had a high operative mortality Also the patients with arch hypoplasia had higher incidence of post-operative residual coarctation.

• 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.28 no.3
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• pp.237-246
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• 1995

Between March 1989 and December 1994, one-stage repair was performed for correction of the intracardiac malformations associated with aortic coarctation in 34 patients or interrupted aortic arch in 8 patients via median sternotomy. There were 26 male and 16 female patients, and their body weight ranged from 1.8 to 8 kg [mean weight, 4.0 1.4 kg . The age at the operation ranged from 7 days to 18 months [mean age, 3.1 $\pm$ 3.8 months . The repair of aortic coarctation or interrupted aortic arch was performed using extended end-to-end anastomosis in most of the patients [86%, 36/42 , and six patients underwent ductal tissue excision and patch aortoplasty. Intracardiac defects were corrected concomitantly through the right atrium unless the anatomy dictated otherwise. Obstructive outlet septum was resected whenever necessary. There were seven early deaths [16.8 % , and three late deaths with a mean follow-up period of 25 months [range from 1 to 65 months . Three patients were reoperated upon residual subaortic stenosis, stenosis at the RPA origin, and subacute bacterial endocarditis respectively. None showed any significant residual or anastomotic stenosis postoperatively. One stage repair of the aortic coarctation and interrupted aortic arch associated with intracardiac defect leaves no native coarctation shelf tissue or residual hypoplasia in the repaired segment, has low incidence of recurrent or residual stenosis, minimizes reoperation and incisions, and manages arch hypoplasia easily. We concluded that surgical results of one-stage repair for the intracardiac malformation associated with aortic coarctation or interrupted aortic arch are reasonable.

• Journal of Chest Surgery
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• v.24 no.3
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• pp.280-286
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• 1991

Supravalvular aortic stenosis may be defined as an obstructive congenital deformity of the ascending aorta which originates just distal to the level of the origins of the coronary arteries It may be localized or diffuse. Enlargement of the aorta with a diamond-shaped patch of the noncoronary sinus of Valsalva was reported in 1961 by McGoon and associates But this reconstruction is asymmetric and the aortic obstruction may remain. In 1977, Dotty and associates reported the extended aortoplasty, the supravalvular ring was incised at two points in the noncoronary and in the right coronary sinuses of Valsalva closed with a tubular Dacron prosthesis of inverted Y-shape tailored to reconstruct the aorta We experienced three cases of the supravalvular aortic stenosis. The 11-year-old female and 4-year-old male with localized supravalvular aortic stenosis in William`s syndrome were operated with an inverted Y-shaped aortotomy toward the non-coronary sinus and the right coronary sinus and closed with "Hemashield`s collagen impregnated Dacron" tube graft, fashioned into "pantaloon" form patch. The 12-year-old male with localized supravalvular aortic stenosis and mitral insufficiency in William`s syndrome were operated with same procedure as two other patient above-mentioned for relief of supravalvular aortic stenosis and with mitral valve replacement. Postoperative course has been good.ourse has been good.

• Journal of Chest Surgery
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• v.31 no.8
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• pp.763-769
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• 1998

Background: Supravalvular aortic stenosis is a rare form of congenital cardiac anomaly involving ascending aorta distal to coronary orifice. Materials and methods: We operated 12 cases of supravalvular aortic stenosis between July 1986 and March 1997. Age ranged from 4 to 17(mean 10.2) years and 11 of them were male. Nine patients had clinical features of Williams syndrome. We experienced two types of supravalvular aortic stenosis, including 10 hour glass type and 2 diffuse type. Results: Preoperative transaortic pressure gradient ranged from 40 to 180(mean 92) mmHg by cardiac catheterization. Pulmonary stenosis was associated in 5 and 2 of them required angioplasty. Operative techniques included 6 standard aortoplasty with elliptical patch, 4 extended aortoplasty with inverted Y shaped patch, and 2 modified Brom's repair. There were no operative deaths. Postoperative echocardiographic evaluation was done at a mean interval of 12 months. Grade I or II aortic regurgitation was found in 3 cases. Postoperative cardiac catheterization revealed a mean transaortic pressure gradient of 26 (range 0 to 75) mmHg. A mean pressure drop was 78(range 30 to 114) mmHg. All patients were followed up for a mean of 40(range 1 to 67) months with uneventful clinical course. Conclusions: Our data proved the low mortality and excellent hemodynamic improvement after surgical relief of supravalvular aortic stenosis in children.

• 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.28 no.7
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• pp.658-665
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• 1995

Between March 1989 and December 1994, one-stage repair was performed for correction of the intracardiac malformations associated with aortic coarctation in 34 patients or interrupted aortic arch in 8 patients via median sternotomy. There were 26 male and 16 female patients, and their body weight ranged from 1.8 to 8kg[mean weight, 4.0$\pm$l.4kg . The age at the operation ranged from 7 days to 18 months [mean age, 3.1$\pm$3.8 months . The repair of aortic coarctation or interrupted aortic arch was performed using extended end-to-end anastomosis in most of the patients[86%, 36/42 , and six patients underwent ductal tissue excision and patch aortoplasty. Intracardiac defects were corrected concomitantly through the right atrium unless the anatomy dictated otherwise. Obstructive outlet septurn was resected whenever necessary. There were seven early deaths[16.8% , and three late deaths with a mean follow-up period of 25 months [range from 1 to 65 months . Three patients were reoperated upon residual subaortic stenosis,stenosis at the RPA origin, and subacute bacterial endocarditis respectively. None showed any significant residual or anastomotic stenosis postoperatively. One stage repair of the aortic coarctation and interrupted aortic arch associated with intracardiac defect leaves no native coarctation shelf tissue or residual hypoplasia in the repaired segment, has low incidence of recurrent or residual stenosis, minimizes reoperation and incisions, and manages arch hypoplasia easily.We conclude that surgical results of one-stage repair for the intracardiac malformation associated with aortic coarctation or interrupted aortic arch are resonable.

• Journal of Chest Surgery
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• v.29 no.3
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• pp.285-291
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• 1996

We have reviewed 157 cases of coarctation of aorta in the age of infancy and children from March 1973 to december 1994. Patients were 9) males and 64 females, and their age ranged from one month to fifteen years (23.8 $\pm$ 41.6months) with 113 infant cases. In the infantile age, congestive heart failure was the most common chief complaint (781113), and above that age, asymptom was most common (15/44). Isolated coarctation was present in 31 patients, and 9 patients had additional atrial septal defect (group I), 73 patients had associated ventricular septal defect (group II), and 44 patients had associated complex intracardiac lesions(group III). Aortic arch hypoplasia was present in 17 patients. , The operations perf'ormed were subclavian flap angioplasty in 71 patients, resection and anastomosis in 32 patients, patch aortoplasty in 26 patients, resd extended end-!o-end anastomosis in 27 patients, and direct angioplasty with resection of web in 1 patient. The early mortality was 17.2% (27/157) and re-coarctation rate was 18.0% (281157). The most common complication was respiratory problem in infants and postoperative hypertension in children.