• Journal of Chest Surgery
• /
• v.25 no.12
• /
• pp.1471-1475
• /
• 1992

Within the group of congenital cardiac anomalies manifesting atrioventricular discordance, there exists a subset of hearts characterized by the additional presence of double outlet of the morphological right ventricle. Most of these hearts have associated pulmonary stenosis and abnormal direction of the cardiac apex, and all have a ventricular septal defect. Recently, a 10-year old boy underwent successful surgical correction of double outlet right ventricle with atrioventricular discordance. Diagnostic confirmation was made by 2-D echocardiography, cardiac catheterization and cineangiocardiography. The operation consisted of patch closure of ventricular septal defect so as to leave both great arteries draining the systemic[morphologically right] ventricle, and insertion of an extracardiac valved conduit between the morphological left ventricle and the distal end of the proximally oversewn pulmonary artery. Postoperative course was uneventful.

• Journal of Chest Surgery
• /
• v.23 no.6
• /
• pp.1174-1179
• /
• 1990

Forty-five patients with double-outlet right ventricle[DORV] underwent complete intracardiac repair between July, 1983 and June, 1989. Patients with complete atrioventricular canal, atrioventricular discordance and uni-ventricular heart were excluded. The 32 male and 13 female patients ranged in age from 3 months to 15 years[mean 4 years]. Thirty-two patients had pulmonary stenosis. The early mortality was 11.ltd[5 /45] None of 27 died after a completely intraventricular repair. The mortality was 20%[1/5] for repair using transannular patch, 20% [1/5] for REV operation, 33.3%[1/3] for repair including extracardiac valved conduit, and 50% [1/2] for Jatene operation, respectively. Two modified Fontan procedures were performed without mortality. One died after Senning operation. Causes of early deaths included high residual right ventricular pressure[one patient] small left atrial and left ventricular volume[one patient], persisting severe pulmonary hypertension [one patient] and low cardiac output of unknown cause [two patients]. Complete heart block developed in one patient. Two late deaths occurred among the 40 operative survivors [5.0Po] from persisting severe pulmonary hypertension and bleeding at reoperation. Our results indicate that significant defects can be repaired with low mortality and morbidity.

• Journal of Chest Surgery
• /
• v.20 no.3
• /
• pp.603-609
• /
• 1987

The clinical, investigative, and surgical experiences were reviewed in four patients with congenitally corrected transposition of the great arteries who presented to the National Medical Center between August 1983 and August 1985. This condition is very rare congenital anomaly defined as the combination of atrioventricular discordance and transposition of the great arteries. Examples of primitive [single] ventricle inverted [that is, left sided in situs solitus] with outflow chamber were excluded in this paper. According to the sequential arrangement of the hearts there were two cases of [S,L,L] and two cases of [I,D,D]. The surgical approach should be focused on minimizing the risk of heart block and increasing the degree of relief of pulmonary outflow tract obstruction [POTO]. We experienced complete heart block in two cases of [S,L,L] and significant residual POTO in one case of [S,L,L] and one case of [I,D,D] postoperatively. There were one hospital mortality caused by complete heart block and residual POTO and two delayed mortalities caused by congestive heart failure and sepsis respectively.

• Journal of Chest Surgery
• /
• v.20 no.3
• /
• pp.635-641
• /
• 1987

Recently, direct atriopulmonary anastomosis was performed in 2 patients without using pericardium or prosthetic material. Case 1 A 13-year-old male patient whose diagnosis was tricuspid atresia [type lb] received direct atriopulmonary anastomosis successfully with good clinical and hemodynamic results. Nine months after operation, he has an increased exercise tolerance. Case 2 A 4-year-old female patient whose diagnosis was DORV with pulmonary atresia in A-V discordance had a formidable surgical challenge. A large-diameter direct atriopulmonary anastomosis was performed after the closure of the right atrioventricular valve. Her postoperative course has been even in 20th postoperative day.

• Journal of Chest Surgery
• /
• v.23 no.4
• /
• pp.769-775
• /
• 1990

Congenitally corrected transposition of the great arteries is defined as the anomaly of atrioventricular and ventriculoarterial discordance. Although this condition is compatible with longevity, it is usually associated with other congenital cardiac anomalies, which result in a greatly shortened life span. This report describes a 2 years old female patient who had congenitally corrected transposition of the great arteries associated with ASD, VSD and pulmonary stenosis. The ASD was closed directly through the right atriotomy and the VSD was closed using Dacron patch through right ventriculotomy and the pulmonary[valvular, supra and subvalvular] stenosis was repaired with the use of a valved conduit woven Dacron vessel graft between the pulmonary ventricle and the main pulmonary artery.

• Journal of Chest Surgery
• /
• v.33 no.12
• /
• pp.959-962
• /
• 2000

A 4-year and 11-month old child was diagnosed as having dextrocardia, pulmonary atresia, atrioventricular discordance, aorta from right ventricle, PDA, the interruption of right pulmonary artery and postoperative state of Blalock-Taussing shunt on right pulmonary artery. Anatomical repair so called \"double switch operation\" was performed; the Ratelli procedure on ventricular level and the Mustard procedure on atrial level. We report the successful anatomical repair of congenitally Physiologically Corrected Transposition even with Dextrocardia, Situs Inversus and the interruption of Right Pulmonary Artery.ry Artery.

• Clinical and Experimental Pediatrics
• /
• v.46 no.3
• /
• pp.259-264
• /
• 2003

Purpose : We have performed an analysis on patients who received Rastelli operation in our institute and reviewed their progress postoperatively. Various factors with suspected relationship to the outcome have been considered to help in future treatment and follow-up. Methods : We analyzed retrospectively 43 patients who either received Rastelli operation in Yonsei University Cardiovascular Center from March 1995 to April 1997 or who received post-procedural cardiac catheterization and follow-up echocardiography in the out-patient department after the procedure. Results : No statistically valid relationships were found between the age of the patient, their body weight, preoperative pulmonary arterial index and pressure, presence of pulmonary branchial stenosis and postoperative results. Cases with atrioventricular concordance showed lower age and body weight, and discordant cases exhibited lower ejection fraction 3 days postoperatively. Upon follow up, lower NYHA score was seen in patients with severe residual stenosis. In the group that received cardiac catheterization after the procedure, residual stenosis and right ventricular pressure measurement in echocardiography showed good correlation with the catheterization data. Conclusion : In cases where conduit insertions of the right ventricular outflow tract are required to achieve total correction in complex cardiac deformity, early operation does not seem to provide a clear risk to the patient. In patients with atrioventricular discordance, careful postoperative observation of the ventricular function seems to be needed. Also, echocardiography appears to be a sound method in follow-up of patients after the correctional procedure.

• Journal of Chest Surgery
• /
• v.41 no.1
• /
• pp.1-11
• /
• 2008

Background: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. Material and Method: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months ($13\;days{\sim}38.1\;months$) and body weight was 5.0 kg ($2.9{\sim}13.5\;kg$). Preoperative pressure gradients were $25.3{\pm}15.7\;mmHg$ ($10{\sim}60\;mmHg$). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6,. another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. Result: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of $62.7{\pm}38.9$ months ($3.3{\sim}128.1$ months). Kaplan-Meier estimated actuarial survival was $71.9%{\pm}9.3%$ at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. Conclusion: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.