• Journal of Chest Surgery
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• v.36 no.6
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• pp.384-390
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• 2003

Background: Since Ross and Sormeville first reported the use of aortic homograft valve for correction of pulmonary atresia in 1966, homograft valves are widely used in the repair of congenital anomalies as conduits between the pulmonary ventricle and pulmonary arteries. On the basis of these results, we have used it actively. In this report, we describe our experience with the use of cryopreserved valved homograft conduits for infants and children requiring right ventricle to pulmonary artery connection in various congenital cardiac anomalies. Material and Method: Between January, 1996 and December 2001, 27 infants or children with a median age of 16 months(range 9days to 18years) underwent repair of RVOTO using homograft valved conduit by two surgeons. We studied 22 patients who have been followed up at least more than one year. The diagnosis at operation included pulmonary atresia with ventricular septal defect (n=13), truncus arteriosus (n=3), TGA or corrected TGA with RVOTO (n=6). Homograft valved conduits varied in size from 15 to 26 mm (mean, 183.82 mm). The follow-up period ranged from 12 to 80.4 months (median, 48.4 months). Result: There was no re-operation due to graft failure itself. However, early progressive pulmonary homograft valve insufficiency developed in one patient, that was caused by dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia after repair of pulmonary atresia with ventricular septal defect. This patient was required reoperation (conduit replacement). During follow-up period, there were significant pulmonary stenosis in one, and pulmonary regurgitation more than moderate degree in 3. And there were mild calcifications at distal anastomotic site in 2 patients. All the calcified homografts were aortic in origin. Conclusion: We observed that cryopreserved homograft conduits used in infant and children functioned satisfactorily in the pulmonic position at mid-term follow-up. To enhance the homograft function, ongoing investigation is required to re-establish the optimal strategy for the harvest, preservation and the use of it.

• Journal of Chest Surgery
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• v.34 no.11
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• pp.854-857
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• 2001

Double-outlet left ventricle(DOLV) is a rare congenital cardiac malformation, defined as the origin of both the aorta and the pulmonary artery being entirely or predominantly above the morphologically left ventricle, which is difficult to diagnose accurately. A 3-year old male was admitted for cyanosis and dyspnea. At the age of 2 months, he had undergone pulmonary artery banding and coarctoplasty. He was diagnosed as DOLV with subaortic ventricular septal defect(VSD). Biventricular repair was achieved by patch closure of VSD, primary closure of PFO, and pulmonary trunk translocation from left ventricle to right ventricle. The advantages of this procedure using native tissue for right ventricular outflow tract reconstruction are growth potential and preserved valve function, which contribute to a decreased likelihood of reoperation related to the right ventricular dysfunction related to pulmonary insufficiency.

• Journal of Chest Surgery
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• v.36 no.10
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• pp.759-765
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• 2003

Truncus arteriosus with interrupted aortic arch is a very rare congenital cardiac anomaly that has an unfavorable natural course. We report a successful one-stage repair of truncus arteriosus with interrupted aortic arch through median sternotomy in a 25-day-old neonate weighing 3.1 kg. We reconstructed the aortic arch with direct side-to-end anastomosis between ascending and descending aortas. The right ventricular outflow reconstruction was performed with untreated autologous pericardial conduit without valve following Lecompte maneuver. The patient has been grown-up in good condition (25 ∼ 50 percentile of body weight) and shows the right ventricular outflow tract wide 1 year after the operation.

• Journal of Chest Surgery
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• v.37 no.10
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• pp.833-838
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• 2004

Background: Despite the excellent early results after the repair of congenital bicuspid aortic valve (BAV) disease, the mid-term durability of the repaired valve has still controversies. Material and Method: To evaluate the mid-term results of BAV repair, retrospective review of medical records and echocardiographic data were done. Between 1994 and 2003, twenty-two patients underwent reparative procedure for either regurgitant or stenotic congenital bicuspid aortic valve (BAV). Result: Mean age was $41\pm14$ years with male predominance (Male=17, Female=5). The pathophysiologies of the BAV were regurgitation-dominant in 20 (91%) and stenosis-dominant in 2 (9%) cases. Various repair techniques were used for raphe, prolapsed leaflet, thickened leaflet, and commissures; 1) release of raphe in 19 (86%), 2) wedge resection and primary repair in 11 (50%), pericardial patch reinforcement after plication of the leaflet in 6 (27%), and plication of the leaflet in 3 (14%), 3) slicing of thickened leaflet was used in 12 (55%) cases, 4) commissuroplasty in 8 (36%), and commissurotomy in 6 (27%) cases. There was no in-hospital mortality. During the mean follow-up of $38\pm17$ months, one patient underwent aortic valve replacement after developing acute severe regurgitation from dehiscence of the suture on postoperative 2 months. New York Heart Association functional class was improved from $1.9\pm0.6$ to $1.2\pm0.5$ (p<0.01). Left ventricular end-systolic and diastolic dimension (LVESD/LVEDD) were also improved from $45\pm9$ and $67\pm10$ to $37\pm10$ and $56\pm10,$ respectively (p<0.01). The grade of aortic regurgitation (AR) was improved from preoperative $(3.1\pm1.2)$ to post-bypass $(0.9\pm0.7).$ However, the grade at last follow-up $(1.7\pm1.1)$ was deteriorated during the follow-up period (p<0.01). Freedom from grade III and more AR at one, three, and four year were 89.7%, 89.7%, and 39.9% respectively. Conclusion: Midterm clinical result of the BAV repair was favorable. But, the durability of the repaired valve was not satisfactory.

• 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.41 no.1
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• pp.102-105
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• 2008

An anomalous origin of the coronary artery with subsequent coursing between the great vessels is a rare congenital heart defect that may cause myocardial ischemia and sudden death. Several surgical techniques have been described to address this defect. An extended unroofing procedure to create an alternative ostium for the right coronary artery was successfully carried out in a patient having an anomalous origin of the right coronary artery. The newly constructed orifice was widely patent 3 months later, without any episodes of myocardial ischemia or aortic regurgitation.

• Journal of Chest Surgery
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• v.42 no.5
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• pp.597-603
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• 2009

Background: Atrioventricular valve regurgitation in pediatric patients with a functional single ventricles (FSV) - has been known as one of the important risk factors for death and unfavorable long-term results after a Fontan operation. We evaluated early and mid-term results of bivalvation valvuloplasty in FSV patients. Material and Method: We retrospectively evaluated 11 patients with a functional single ventricle who underwent bivalvationvalvuloplasty between 1999 and 2007. The degree of common atrioventricular valve regurgitation (CAVVR) was determined by color Doppler echocardiography (regurgitation grade scoring, trivial; 1, mild; 2, moderate; 3, severe; 4). Mean age at valve surgery was $6.9{\pm}7.0$ months (median 4 months, 24 days$\sim$21 months)and mean body weight was $6.2{\pm}2.8\;kg$ ($3.1{\sim}11.3\;kg$). Nine patients had isomerism heart and two of them had TAPVC. The concomitant procedures were performed in all but one patient. Additional commissural closure was performed in 3 patients and commissural annuloplasty in another 3 patients. Result: There was one hospital death after. surgery. A 32-day old patient who had been preoperatively dependent on a ventilator died of air way and lung problems 4.3 months after pulmonary artery banding and bivalvation valvuloplasty. Mean follow-up duration was 40 months ($4.3{\sim}114$ months). Mean preoperative CAVVR score was $3.3{\pm}0.6$, which decreased to $1.9{\pm}0.7$ postoperatively (p<0.0001). This residual regurgitation slightly increased to $2.2{\pm}0.4$ (no statistical significance) after a mean follow-up of 1.4.3 months. Six patients (60%) required re-operations for residual regurgitation at a subsequent bidirectionalcavopulmonary shunt or Fontan operation. One patient with Ebsteinoid malformation of the right sided atrioventricular valve required valve replacement due to stenoinsufficiency. Another patient required edge-to-edge repair at the right sided AV valve (between the right mural leaflet and the bridging leaflets). The remaining 4 patients required additional suture placements between bridging leaflets with or without commissural annuloplasty. All survivor had trivial or mild CAVVR at the latest follow-up. Conclusion: Bivalvation valvuloplasty for CAVVR in FSV patients is. an effective and safe procedure. However, significant numbers of the patients have small residual regurgitation and require additional valve procedures at subsequent operations. Long-term observations to monitor progression of the CAVVR is mandatory.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.270-274
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• 1997

In recent years, the use of allograft conduits in repair of congenital cardiac disease is widely accepted. However, the supply of homograft. is currently limiting their increased clinical application, especially small cryopreserved homografts for use in neonates and inf'ants. We used a technique to surgically reduce the size of the more readily available large-diameter allografts, making them suitable for right ventricular outflow tract reconstruction in small infants and children. From December 1994 to March 1996, a total of 11 patients ranging in age from 10 months to 6 years (mean age, 27.3 months) and ranging in weight from 5.6 to 18.5 kg (mean 11.5 kg) underwent reconstruction of the right ventricular outflow tract using this surgical technique (pulmo ary atresia with ventricular septal defect, 9 cases ; tetralogy of Falloff, 2 cases). The diameter after downsizing ranged from 14 to 19 mm with a mean of 16.8 mm. There was one operative death due to rupture of the infected homograft. Evaluation of these patients between 2 and 15 months (mean 6.9 months) after homograft implantation reveals excellent clinical and echocardiographic results. There were no significant homograft insufficiency and RVOT obstructions. Although a longer follow-up is certainly required to evaluate the long term fate of the surgically modified bicuspid homografts, we believe that this technique may represent a valuable therapeutic alternative, at least in the short term, to the use of synthetic grafts when an appropriately sized homograft is not available.

• Journal of Chest Surgery
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• v.30 no.12
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• pp.1184-1189
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• 1997

Tetralogy of Falloff is the most common cyanotic congenital cardiac malformation. Between April 1984 and December 1993, we experinced with 39 cases of total correction for the tetralogy of Falloff at the department of Thoracic and Cardiovascular Surgery, Masan Samsung General Hospital. The results were as follows; There were 14 males and 25 females, and their ages ranged from 2 to 36 years, with an average age of 13.33 $\pm$7.40 years. There was type I VSD in 10 cases(25.6%), type II VSD in 19 cases(48.8%) and type 1+ II VSD in 10 cases(25.6%) Types of right ventricular outflow tract obstruction were valvar + infundibular stenosis in 19 cases(48.7%), valvar + infundibular + annular stenosis in 10 cases(25.6%), infundibular stenosis in 9 cases(23.1%)and pure valvar stenosis in 1 case(2.6%). Right ventricular outflow tract patch widening was necessary in 33 cases(84.6%) and a Iransannular patch including 2 cases of Monocusp-bearing outflow patch was used in 10 cases. n 36 hospital survivors, 23 patients were evaluated by 2 dimensional and doppler echocardiography. The most common complication was arrthymia, especially complete right bundle branch block, in 30 cases (76.9%). The operative mortality was 7.7%, and the cause of death was low cardiac output syndrome.

• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.529-537
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• 2005

Background: Historically the Fontan operation in patients with single ventricle and heterotaxy syndrome has been associated with high mortality because of systemic or pulmonary anomalous venous drainage, incompetent common atrioventricular valve, right ventricle type univentricular heart, and arrhythmia. Material and Method: A retrospective review of 62 patients $(age:\;54.79\pm33.97\;months)$ with heterotaxy syndrome who underwent a extracadiac Fontan operation between 1996 and 2005 was performed. Twenty one patients had left atrial isomerism, and 41 had right isomerism. The Fontan procedure was staged in all but 2 patients, and a fenestration was less placed in left isomerism. Result: Left isomerism was associated more with interrupted inferior vena cava and pulmonary arteriovenous fistula, and right isomerism was associated more with anomalous pulmonary venous drainage, common atrioventricular valve and morphologic right ventricle. There were 3 hospital deaths$(4.8\%)$ and 3 late deaths $(5.2\%)$ with a follow-up duration of $48.8\pm31.0$ months. Eight-year survivals were $90.5\pm6.4\%$ in left isomerism and $88.6\pm5.4\%$ in right isomerism (p=0.94). At 8 years, freedom from reoperation was $73.9\pm11.3\%$ in left isomerism, and $82.3\pm6.7\%$ in right isomerism (p=0.87). Atrioventricular valve regurgitation progressed after Fontan operation in heterotaxy syndrome, and reoperation for pulmonary arteriovenous fistula and permanent pacemaker implantation for sinus node dysfunction were required more in left isomerism. Conclusion: The extracardiac Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative atrioventricular valve regurgitation, arrhythmia, and pulmonary arteriovenous fistula remains significant.