Patients with severe Ebstein's anomaly showing in the neonatal period, represent progressive cardiac enlargement with pulmonary hypoplasia and functional pulmonary atresia with patent ductus alteriosus-dependent pulmonary circulation. Biventricular repair in these patients had been mostly unsuccessful except for Starnes' procedure that converts the anatomy to single ventricle physiology for Fontan procedure. A 4-days old male was admitted with the diagnosis of severe Ebstein's anomaly with anatomic pulmonary atresia and severe cardiac enlargement. He successfully underwent biventricular repair with vertical plication method of atrialized right ventricle, tricupid annuloplasty, transannular right ventricular outflow tract reconstrulltion, atrial septal defect patch closure with fenestration, and right atrial reduction angioplasty Postoperatively, cardiothoracic ratio was significantly reduced and mild tricuspid regurgitation was remnant in echocardiography. The patient is currently 10 months old and is fully active without restrictions.
Twenty two patients underwent total anatomic correction of complete atrioventricular septal defect associated with other cardiac anomalies between July 1986 and December 1994. Age ranged from 6 months to 11 years(mean 49.6 $\pm$ 35.8 months), and they were composed of 7 males and 15 females. Combined major cardiac anomalies were tetralogy of Fallot(TOF) in 11 cases, double outlet of right ventricle (DORV) in 6 ca es, and transposition of great arteries (TGA) in 5 cases. Down's syndrome was associated in 5 patients with TOF and 1 patient with DORV. They were classified as Rastelli type A in 3 patients, B in 2 patients, and C in 17 patients. Modified Blalock-Taussig shunt was performed.in 5 patients and Waterston shunt in 1 patient as a palliative procedure. There were 7 perioperative deaths(31.8%) and the causes were pump weaning failure, low cardiac output, acute renal failure, persistant pulmonary hypertension and hypertensive crisis, and sepsis. Reoperations were performed in 4 cases to repair atrioventricular valvular regurgitation or to relieve the right ventricular outflow tract (RVOT) or pulmonary arterial stenosis. One late death was due to aspiration pneumonia. Second reoperation was necessary for progressive worsening of left atrioventricular regurgitation and RVOT stenosis in one patient. Fourteen survived patients were followed up for a mean of 66.0 $\pm$ 26.7months and all of them w re NYHA functional class I or II.
In candidates for aortic valve replacement [AVR]it is our primary intention to implant the largest possible vale prosthesis of at least 23 mm in diameter in patients with severe valvular aortic stenosis. However, in many patients there is an additional subvalvular asymmetric septal hypertrophy which in some cases may cause an postextrasystolic increase of the LV-aortic gradient. Another component of the aortic stenosis syndrome is a narrow valvular ring, or a combination of both. After complete removal of the diseased valve and decalcification the narrow aortic ring [< 23 mm] can be widened firstly by transaortic subvalvular septal myectomy- [TSM] thus unfolding the left ventricular outflow tract[LVOT]and secondly by extending the oblique aortic incision into the aortic valve ring or further down into the anterior leaflet of the mitral valve. The sub-and supra-valvular defect will be closed by patch enlargement of the aortic root [PEAR] using autologous pericardium. These techniques allow a considerable enlargement of the valvular ring of about 4 to 10 mm in circumference. In a retrospective study using a computerized program, 847 patients with AVR [1980-1984]were reviewed to evaluate the intraoperative hemodynamic results mainly concerning relief of the transvalvular gradient. In 626 patients AVR was performed, 151 patients had double valve replacement [AVR+MVR], and 70 patients had AVR plus additional surgical procedures. Concentrating on the AVR-group [n=626] there were 103 patients with TSM, 24 patients with PEAR and 20 patients with TSM+PEAR which demonstrated that in a total, of 147 patients of this groups [23.5%] an additional procedure was necessary. The Statistical evaluation of the intraoperative pressure measurements before and after AVR in relation to the size of the implanted prostheses indicated the lowest preoperative mean gradient in patients with AVR alone, the highest in patients who afforded TSM plus PEAR. However, after AVR the mean gradients in all three groups were very low [mean 5 to 10 mmHg]. These data indicate that in patients with a narrow aortic ring and additional considerable ASH, TSM and PEAR are suitable techniques to enlarge the aortic root to enable the implantation of an adequate aortic valve prosthesis. Long-term controls have shown that autologous pericardium is a qualified graft material for the ascending aorta.
Backgound: It has been shown that the endothelium of cardiac valves and adjacent great vessels have a reduced immune reaction compared to other vessels. We investigated the clinical feasibility of using immunologically untreated xenogenic valves, in a pig-to-goat pulmonary valve conduit implantation model. Material and Method: Porcine pulmonary valve conduits were prepared without specific immunologic treatment and implanted into the right ventricular outflow tract of goats while undergoing cardiopulmonary bypass. Two goats each were assigned to the following observation time intervals: one day, one week, three months, six months and twelve months. Echo-cardiographic examinations were performed prior to sacrifice of the goat to evaluate pulmonary valve function. After the xenograft specimens were retrieved, histological changes were evaluated microscopically. Result: Ten of the twelve animals survived the predetermined observation time intervals. Aneurysmal dilatations, of the anterior wall of the implanted pulmonary artery, were observed at each of three and twelve month-survival animals. A variable degree of pulmonary valve regurgitation was observed on echocardiography. However, valve stenosis, thrombotic occlusion and vegetation were not seen. Microscopically, the nuclei of the donor tissue disappeared as a result of pyknosis and karyolysis; however the three components of the implanted xenografts (the pulmonary artery, the valve and the infundibulum) were gradually replaced by host cells over time, while maintaining their structural integrity. Conclusion: Immunologically untreated xenogenic pulmonary valve conduits were replaced by host cells with few observed clinical problems in a pig to goat pulmonary valve implantation model. Therefore, they might be an alternative bioprosthesis option.
Background: The aim of this study is to evaluate the long term results of creating various right ventricle to pulmonary artery conduits for treating complex congenital heart disease. Material and Method: Between June 1986 and July 2006, we retrospectively reviewed 245 patients who underwent reconstruction of the right ventricular outflow tract with various kinds of conduits. 410 operations were done in 245 patients, the mean age at operation was $3.2{\pm}4.9$ years (range: 7 days$\sim$45 years) and the mean body weight was $12.5{\pm}8.7\;kg$ (range: $2.4\sim76.3\;kg$). Result: We used the following conduits: Polystan conduit, Shelhigh conduit, Carpenter-Edward conduit, Dacron graft with an artificial valve, valveless Gore Tex vascular graft, homograft and hand-made bovine or autologous pericardial conduit. The mean follow up duration was $6.3{\pm}5.2$ years. Redo operation for RV-PA conduit dysfunction was performed in 131 patients, a second redo was done in 31 and a third redo was done in 3. The reoperation free rates were 67.3%, 48.5% and 39.4% for 5 years, 10 years and 15 years, respectively. The homograft showed the best durability, followed by the Dacron graft with artificial valve and the Carpentier-Edward conduit. The larger sized conduit showed better durability. Conclusion: The homograft showed lowest reoperation rate and a smaller size of conduit showed the highest reoperation rate. The reoperation rate for the RV-PA conduit was about 35% at 5 years, so it is mandatory to develop the more durable conduit for RV outflow.
Arterial oxygen saturation $(SaO_2)$ instability frequently takes place after systemic-pulmonary shunt without shunt occlusion. We analyzed actual incidence and risk factors for $SaO_2$ instability after shunt operations, and possible mechanisms were speculated on. Material and Method: Ninety three patients, who underwent modified Blalock-Taussig shunt from January 1996 to December 2000, were enrolled in this study. Adequacy of shunt was verified in all patients, either by ensuing one ventricle or biventricular repair later on or by appropriate pulmonary artery growth on postoperative angiogram. Age, body weight, hemoglobin level at operation were 3 day to 36 years (median: 1.8 months), 2.5kg to 51kg (median: 4.1kg) and $10.7\~24.3$ gm/dL (median: 15.2 gm/dL) respectively. Preoperative diagnoses were functional single ventricle with pulmonary stenosis or atresia in 39, tetralogy of Fallot in 38 and pulmonary atresia with intact ventricular septum in 16. Pulmonary blood flow (PBF) was maintained pre-operatively by patent ductus or previous shunt in 64 and by forward flow through stenotic right ventricular outflow tract (RVOT) in 29. $SaO_2$ instability was defined as $SaO_2$ less than $50\%$ for more than 1 hour with neither anatomic obstruction of shunt nor respiratory problem. Result: 10 patients $(10.7\%)$ showed $SaO_2$ instability after shunt operation. After shunt occlusion was ruled out by echocardiogram, they received measures to lower pulmonary vascular resistance (PVR), which worked within a few hours in all patients. Risk factors for $SaO_2$ instability included older age at operation (p=0.039), lower preoperative $SaO_2$ (p=0.0001) and emergency operation (p=0.001). PBF through stenotic RVOT showed marginal statistical significance (p=0.065). Conclusion: $SaO_2$ instability occurs frequently after shunt operation, especially in patients with severe hypoxia pre-operatively or unstable clinical condition necessitating emergency operation. Temporary elevation of pulmonary vascular resistance is a possible mechanism in this specific clinical setting.
Excellent clinical results of the arterial switch operation and the limited availablity of the intraventricular rerouting has recently made an arterial switch operation to become the therapeutic method of choice for the repair of double-outlet right ventricle (DORV) with subpulmonary ventricular septal defect (VSD). The early and midterm outcomes of arterial switch operation for this anomaly were evaluated. Material and Method: Between August 1994 and July 2002, 13 patients underwent an arterial switch operation for the correction of double-outlet right ventricle with subpulmonary VSD at Dong-A university hospital.. The 50% rule was used to define DORV. Median age and mean body weight were 27 days (range, 3-120 days) and 3.8$\pm$0.7kg (range, 2.92-5.3kg) respectively. Aortic arch anomalies were associated in 6 cases (46.2%), which were all repaired through one-stage operation. The relationship of the great arteries were side-by-side in 8 cases (61.5%) and anteroposterior in 5 (38.5%). Coronary artery patterns were 1 LCx-2R in 6 cases, retropulmonary left coronary artery (LCA) in 6, and intramural LCA in 1 respectively. The enlargement of VSD was required in 1 patient and the patch enlargement of right ventricular outflow tract was performed in another one patient. The Lecompte maneuver was used in all but 3 patients with a side by side relationship of the great arteries. Result: Overall postoperative hospital mortality was 23.1 % (3/13). All operative deaths were occurred in the patients with aortic arch anomalies. There was one late death related to the postoperative complication of the central nerve system during the mean follow-up of 41.3$\pm$30.7 months. Pulmonary valvar stenosis (>30mmHg of pressure gradient) developed in 1 patient (10%) and left pulmonary artery stenosis in 2 (20%), among them, one required reoperation 52 months after repair. There was an asymptomatic patient with moderate aortic regurgitation. 5-year survival rate including operative deaths was 68.3%. Conclusion: Although the operative mortality is high in the patients with aortic arch anomaly, the arterial switch operation for DORV with supbpulmonary VSD can be performed with low operative mortality and low reoperation rate in the patients Without arch anomaly. The arterial switch operation can be considered a good option for this complex anomaly.
Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.
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