• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.284-290
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• 2005

We evaluated the early and mid-term results for repair of Ebstein's anomaly. Material and Method: Between January 1989 and June 2004, 29 patients underwent repair of Ebstein's anomaly. The median age was 11.4 years (4 days-50 years). Tricuspid insufficiency equal to or greater than grade 3 was present in 21 $(72.4\%)$ patients. Surgical techniques included tricuspid valve repair with vertical plication of the atrialized ventricle (n=14), Carpentier's technique (n=7), tricuspid valve replacement (n=4), systemic-to-pulmonary arterial shunt (n=2), tricuspid valve repair (n=1), and Fontan operation (n=1). Bi-directional cavopulmonary shunt (BCPS) was required in 5 patients. Among the 2 neonates, one patient underwent successful biventricular repair, and the other patient underwent systemic-to-pulmonary arterial shunt. Follow-up was possible in 21 patients $(75\%)$, and the average follow-up was 37.6 months (3 months-11.3 years). Result: There were $1(3.4\%)$ early and 1 late deaths. Reoperation was required in 4 patients. Two patients underwent tricuspid valve re-replacement, and the other 2 tricuspid valve repair. At recent follow-up, only 2 patients showed tricuspid insufficiency equal to or greater than grade 3, and most patients showed clinical improvement. Excluding the patients who underwent tricuspid valve replacement, the actuarial rate of freedom from reoperation at 1 and 5 years were $94.7\%\;and\;79.0\%$, respectively. Conclusion: Tricuspid valve repair was possible in most patients with good mid-term outcome. Most patients showed clinical and hemodynamic improvement. Indications for the BCPS should be clarified.

• Journal of Chest Surgery
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• v.33 no.3
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• pp.262-264
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• 2000

We report a successful biventricular repair of D-transposition of great arteries, pulmonary stenosis and remote muscular inlet ventricular septal defect, after modifie Blalock-Taussing shunt early in infant. A long left ventricle-to-aorta intraventricular rerouting tunnel was created without stenosis by transferring the medial papillary muscle of the tricuspid valve to the tunnel, obliterating the trabeculation of right ventricle in the course of tunnel and excising the secondary chordae of the tricuspid valve.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.683-687
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• 1986

A direct communication between the left ventricle and right atrium is a relatively uncommon defect. Familiarity with this anomaly has become increasingly important, however, since the preoperative findings may be identical with those of an atrial septal defect. The left ventricle is directly related to the right atrium over. an area of the membranous ventricular septum which extends superior to the septal attachment of the right atrioventricular valve in the LV-RA communication. The clinical triad of a ventricular septal defect murmur, cardiac enlargement, and an arteriovenous shunt at atrial level is characteristic of the malformation. A 2-year-old boy with left ventriculo-right atrial communication has operated at the Maryknoll Hospital. Under the cardiopulmonary bypass, the atrium was opened, there was a jet-blood stream just above the atria-ventricular portion adjacent to the septal leaflet of the tricuspid valve.

• Journal of Chest Surgery
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• v.24 no.7
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• pp.719-724
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• 1991

A case of pulmonary atresia with intact ventricular septum was presented in a 10-month-old cyanotic female patient, which was congenitally rare. Infant with pulmonary atresia and intact ventricular septum usually require urgent surgical intervention. Angiogram showed the pulmonary atresia at the level of the pulmonary valve, the hypoplasia of tricuspid valve and atrial septal defect without patent ductus arteriosus. We performed the pericardial patch graft on the right ventricular outflow tract and pulmonary artery after ventriculotomy using pacemaker wire as electrical saw and main pulmonary arteriotomy and then modified Waterston shunt from the ascending aorta to patch on the right ventricular outflow tract without extracorporeal circulation. Patient was postoperatively good condition.

• Journal of Chest Surgery
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• v.29 no.9
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• pp.945-950
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• 1996

Twelve patients with left ventricular-right atrial shunt (LV-RA shunt) underwent surgical correction be- tween April 1982 and March 1995. Seven patients were male and five patients were female. Age ranged from 3 to 26 years with mean age of 8.5 years. On the preoperative chest PA views, increased pulmonary vascularity was noted in 3 cases and enlargement of right atrium in 4 cases. The mean preoperative cardiothoracic ratio was 0.59. Echocardiographic studies were obtained in 9 patients and the preoperative echocardiographic diagnoses were LV-RA shunt in 2 cases, ventricular septal defect (VSD) in 6 casei, and atrial septal defect (ASD) in 1 case. The preoperative ngiographic diagnoses which were obtained in all patients were LV-RA shunt in 5 cases, VSD in 5 cases, ASD in 1 case, and VSD with ASD in 1 case. The descriptions of defect of LV-RA shunt according to intraoperative findings were supravalvular defect in 5 cases(42%), infravalvular defect in 4 cases (33%), and combined defect in 3 cases (25%). Associated anomalies of tricuspid valve in 4 cases of infravalvular defect were perforation (3 cases) and cleft (1 case). Primary closure of the septal defect was performed through the right atriotomy in all but one patient. There was no operative death. One patient underwent reoperation because of the residual interventricular shunt. All patients have been in good condition.

• Journal of Chest Surgery
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• v.17 no.2
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• pp.244-249
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• 1984

Surgical treatment of partial endocardial cushion defect was accomplished in Feb. 1984 in this department. The 5 year old male patient had history of frequent upper respiratory tract infection and since his age of 3 years dyspnea on exertion and palpitation were noted but there were no cyanosis and clubbing. A thrill was palpable on the apex and grade IV/IV harsh systolic ejection murmur and diastolic murmur was audible on it. Liver was palpable about 3 finger breadths and no ascites. Chest X-ray revealed increased pulmonary vascularity, moderate cardiomegaly [C-T ratio; 0.69], and enlarged left atrium. EKG showed first degree heart block, RVH, LVH, and LAD. Echocardiogram showed paradoxical ventricular septal movement and abnormal diastolic movement of the anterior leaflet of mitral valve. Right heart catheterization resulted left to right shunt [Qp:Qs:2.1:1 ] and moderate pulmonary hypertension [60/40 mmHg]. Left ventriculogram showed mitral regurgitation [Grade III/IV] and filling of left atrium and right atrium nearly same time. Operative findings were: 1.Primum type atrial septal defect [3x2 cm] 2.Cleft on the anterior leaflet of mitral valve. 3.No interventricular communication and cleft of tricuspid valve leaflet. The mitral cleft was repaired with 4 interrupted sutures. The primum type atrial septal defect was closed with Dacron patch intermittently at endocardial cushion and continuously remainder. The post operative course was uneventful and discharged on 22nd postoperative day in good general conditions.

• Journal of Chest Surgery
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• v.40 no.10
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• pp.696-700
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• 2007

We report here on the midterm results after a Starnes operation for a severely symptomatic neonate with Ebstein's anomaly. A one-day-old baby presented with cyanosis and severe cardiomegaly. We peformed patch closure of the tricuspid valve with a central shunt after failure of tricuspid valve repair with vertical plication of the atrialized ventricle at her age of 19 days. The coronary sinus was drained into the right ventricle. She underwent bidirectional cavopulmonary shunt and extracardiac conduit Fontan operation at her age of 16 and 30 months, respectively. She is now 56 months old and is doing very well. The recent follow-up study revealed that she was in normal sinus rhythm and had a normal sized left ventricle with good function and the small right ventricle without thrombus formation.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.280-287
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• 1986

Between March 1978 and August 1985, 29 cases at various congenital heart diseases were reoperated because of remnant shunt of residual anomalies at Seoul National University Hospital. They were consisted of 10 cases of Tetralogy, 4 simple VSD, 6 complicated VSD. 3 partial ECD, and 5 other rare congenital anomalies. The interval between the initial and the second procedure ranged from 1 day to 122 months [mean; 26.9 months]. In 4 cases of them, the second procedure was done during initial hospitalization within 3 weeks post-operatively. The primary operation intended to be corrective surgery except four whose primary operation was palliative or exploratory one even though it was done with extracorporeal circulation. The indication for second operation was mainly residual shunt or valvular obstruction due to patch detachment or inadequate relief of stenotic lesion. Others were paravalvular leak, valvuloplasty failure, prosthetic valve failure, and inadequate primary diagnosis. Four patients were dead [14.3%]; three complicated VSD`s and one Tetralogy. There were 7 cases of nonfatal complication with subsequent improvement except one [diffuse cerebral dysfunction].

• Journal of Chest Surgery
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• v.22 no.2
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• pp.348-356
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• 1989

Seventeen patients of pulmonary atresia with intact ventricular septum were underwent operation during 4.8years period from Jan. 1983 to Aug. 1988 at Seoul National university Hospital. The patients were composed of 8 males and 9 females, aging 1day to 2.5 years [mean 88 days]. We classified pulmonary atresia according to right ventricular morphology; those with tripartite ventricles in 12, those with no trabecular portion to the cavity in 0, and those with inlet portion only in 5. The tripartite approach to right ventricular morphology is helpful in selecting the type of initial palliative procedures. Palliative procedures were as follows; pulmonary valvotomy in 5 with 3 early survivors, mod B-T shunt in 4 with 3 early survivors, and palliative right ventricular outflow tract reconstruction in 4 with 1 early survivor. Effective preliminary palliation of pulmonary atresia are pulmonary valvotomy or palliative right ventricular outflow tract reconstruction in those with tripartite right ventricle, and modified Blalock-Taussig shunt in those with no infundibular portion. The approach to definitive repair is based primarily on the actual size of the tricuspid annulus and the right ventricular cavity. Definitive repair was as follows: definitive right ventricular outflow tract reconstruction in 4 with all survivors and mod. Fontan operation in 2 with one survivors. Right ventricular outflow tract reconstruction can be done as complete repair for patients who had adequate tricuspid annulus and right ventricular cavitary size and mod. Fontan operation for patients who severely hypoplastic tricuspid valve annulus or small right ventricular cavity.

• Journal of Chest Surgery
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• v.29 no.10
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• pp.1081-1089
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• 1996

Pulmonary atresia with intact ventricular septum has continued to have a high surgical mortality and morbidity. This mAy attribute to the non-uniformity of the anomaly. We reviewed a total of 34 infants with pulmonary atresla and intact ventricular septum managed in this hospital between 1987 and 1995. Mean age and body weight were 57.2 (range, 3-208) days and 4.1 (range, B.3∼6.8) kg. The preoperative Z-value of the diameter of the tricuspid valve was less than -2 in 85.2% of patients and less than -4 in 33. 3% . It is well correlated w th right ventricular cavity size (n=27. r10.68, p< 0.05). Coronary artery-right ventricular fistulas were identified in 3 patients, and right ventricular dependency was suspected in 1 Over All hospital mortality was 23.5%(8/34), although it decreased to 16.6%(4124) in 1990s. Subsequent procedures were performed in 6 patients between 3 days and 58 months after Initial palliation : one bidirectional cavopulmonary shunt and 1 Fontan operation after systemic-pulmonary shunt, 3 transannular patch + atrial septal defect closure and 1 additional systemic-pulmonary shunt after polmonary valvectomy or valvotomy. Changes of Z-values of the diameter of tricuspid valve have been followed up in 11 patients between 1 and 66 months postoperatively. Z-values were increased In 5 out of 8 transannular right ventriculAr outflow tract enlargement group and in 1 out of 3 pulmonary valvectomy or valvotomy group. Our data suggest that tailoring a treatment to right ventricular cavity size and coronary anom lies may improve the surgical outcome. A Z-value of the tricuspid valve diameter could be used.