This is one case report of the extremely rare congenital cardiac malformation, Double-outlet of left ventricle in corrected transposition of great arteries. 11-year-old boy complained acrocyanosis and exertional dyspnea, the parents noticed cyanosis since birth. Physical examination revealed acrocyanosis, clubbed fingers and toes, G-III pansystolic murmur on 2nd and 3rd ICS, LSB. Right heart catheterization revealed significant $O_2$ jump in ventricular level. Right and left ventriculography showed the both catheters arriving in the same ventricle i.e. anterior chamber, morphological left ventricle was in right and anterior position, simultaneous visualization of aorta and pulmonary artery and aorta locating anterior and right side of pulmonary artery. Echo cardiogram surely disclosed interventricular septum. Conclusively it was clarified that the patient has Double-outlet of left ventricle and corrected transposition of great arteries [S.L.D.]. Operation was performed to correct the anomalies under extracorporeal circulation with intermittent moderate hypothermia. Right-sided ventriculotomy disclosed the following findings. 1. Right-sided ventricle was morphological left ventricle. 2. Left-sided ventricle was morphological right ventricle. 3. Right side atrioventricular valve was bicuspid. 4. Left side atrioventricular valve was tricuspid. 5. Aortic valve was superior, anterior and right side of pulmonary valve. 6. Subpulmonary membranous stenosis. 7. Non-committed ventricular septal defect. We made a tunnel between VSD and aorta with Teflon patch so that arterial blood comes through VSD and the tunnel into aorta. After correction the patient needed assisted circulation for 135 min. to have adequate blood pressure. Postoperatively by any means, adequate blood pressure could not be maintained and expired in the evening of operation day.
Recently, the arterial switch operation has received more interest and increased application for many patients with TGA [Transposition of the Great Arteries]. Not all patients, however, are suitable candidates for this approach, and its success will be measured against the Senning procedure. From June 1982 through June 1989, 48 consecutive patients underwent correction of TGA by a modification of the Senning procedure in our hospital. 34 were males and 14 females, with ages ranging from 3 months to 99 months [mean age 14 months]. The patients were divided into two groups, group I [TGA with intact ventricular septum or without significant pulmonary hypertension] and group II [TGA with significant pulmonary hypertension]. The hospital mortality was 9.5 % in group I and 55.6 % in group lI. 31 patients were discharged from the hospital after a Senning operation and have been followed a mean of 31.2 months. There were 5 late deaths, 3 in group I and 2 in group II. All patients were followed up with EKG, echocardiogram, and in several cases, Holter monitor and cardiac catheterization and angiography were also performed. There have been arrhythmias in 7, superior vena caval obstruction in 5, pulmonary venous obstruction in 2 and tricuspid insufficiency and/or right ventricular dysfunction in 16 patients. The Senning operation for Simple TGA can be performed with a low operative mortality and morbidity, but tricuspid insufficiency and/or depressed right ventricular function can be a problem with prolonged follow up.
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.
We analysed 60 consecutive patients who got Senning operation for transposition of the great arteries [TGA] with or without ventricular septal defects [VSD]. There were 41 simple TGA [group I] and 19 TGA with VSD [Group II], the operative mortality was 20 % [in group I 4.9 %, group II 52.6 %]. Among the survivors [n=48], the mean follow-up period was 7 years [range, 1 year to 13.5 years] and the actuarial survival rate at 13 years were 95 % in group I and 42 % in group II. Preoperative high left ventricular pressure and high pulmonary arterial pressure affected the surviving [p<0.01]. There occurred various type of arrhythmia like junctional rhythm, first degree atrioventricular [AV] block, sick sinus syndrome and complete AV block, and we inserted 2 permanent pacemakers for these patients. The incidence of arrhythmia were 28.2 % [11/39] in group I and 55.6 % [5/9] in group II, and the actuarial freedom from arrhythmia at 13 years after operation was 66 % [71 % in group I, 44 % in group II]. Increased aortic cross clamping time had affected the development of arrhythmia [p<0.05] which meant the complexity of the operation. The total incidence of left ventricular outflow tract obstruction [LVOTO] was 31.3 % [15/48], but only 3 patients [6.25 %] showed the significant gradient requiring reoperation. The pulmonary venous pathway obstruction [PVO] were found in 3 patients, all in group I, and among them only one required the reoperation. The estimated freedom from PVO was 89 % at 13 years [87 % in group I, 100 % in group II], but we couldn`t find any significant systemic venous obstruction in our series. There occurred 27.1 % [13/48] mild degree tricuspid valve regurgitation without necessary surgical correction. We experienced 14.6 % [7/48] reoperation rate: 3 residual VSD, 3 LVOTO, 1 PVO, 3 atrial baffle leakage. For this high incidence of complication rate after Senning operation and high mortality in TGA with VSD, We do not use this kind of surgical modality any more and do the Jatene operation for all the TGA patients since several years ago.
Corrected transposition of the great arteries [C-TGA] is one of the rare congenital heart disease in which there is both a discordant atrioventricular relationship and transposition of the great vessels. With this arrangement, systemic venous blood passes through the right atrium into the morphologic left ventricle and out the pulmonary artery. Pulmonary venous blood returns to the left atrium, flows into the morphologic right ventricle and out the aorta. Thus, in the rare case when no additional cardiac anomaly is present, a hemodynamically normal heart exists. But more often they are symptomatic as a result of one or several of the commonly associated defects. This paper describes 13 patients who underwent repair of one or more cardiac anomalies associated with corrected transposition at SN UH, from June 1976 through June 1984. 1.8 were males and 5 females, with ages ranging from 3 years to 27 years. 2. Segmental anatomy was {S,L,L} in 12, or {I,D,D} in 1. 3.Associated anomalies were ventricular septal defect in 10, pulmonary outflow tract obstruction in 6, tricuspid insufficiency in. 4, atrial septal defect in 3, subaortic stenosis in 1, mitral insufficiency in 1, and patent ductus arteriosus in 1. 4.None had complete heart block preoperatively, and 3 developed complete heart block intraoperatively. But one of them recovered sinus rhythm on the postoperative 7th day spontaneously. 5.There were 3 cases of hospital morality. But there was no morality since Dec. 1980. 6.Patients with single ventricle, hypoplastic ventricle or those who had palliative surgery alone are not included in this review.
This 3-year-old girl was observed frequent exertional dyspnea and cyanosis at crying since birth. She was not premature baby and delivered at full term normally. On physical examination, she was underdeveloped-body weight 13.5 kg, height 99 cm.- and cyanotic. There was severe clubbing on fingers. There was grade II/VI ejection systolic murmur on left lateral border of the sternum. The preoperative examinations [EKG, echocardiogram, cardiac catheterization and biventriculogram] showed that complicated T.G.A. combined vena cava[S.D.D.]. Preoperatively, we decided the corrective surgery of Rastelli operation using a. pulmonary valved conduit. The operation was performed under total circulatory arrest using deep profound hypothermia combining with extracorporeal circulation. On operation, the anatomy of the heart showed that, 1. The subaortic conus was seen and subaortic muscles were hypertrophied. 2. The VSD[type II], behind the subaortic conus-about 1 cm. in diameter, was visible only through LV cavity and, 3. The pulmonary valve ring was hypoplastic and pulmonary valvular stenosis was seen also. The subpulmonic area [LV outflow tract] was obstructed with hypertrophied muscle and mitral valve. 4. Left superior vena cava was drained to RA via coronary sinus. 5. LAD coronary artery was originated from right coronary artery and ran anterior to the pulmonary artery. According to above anatomy, we performed the VSD closure with Teflon patch, and Mustard operation combined with LV-to-pulmonary artery bypass graft using the valve contained [Hancock 16 mm] conduit. Postoperatively, adequate blood pressure could be maintained under the state of using inotropic agent [epinephrine]. On the second postoperative day, the patient died of cardiac arrest due to low cardiac output syndrome, acute renal failure and pulmonary edema.
This is a report of a successful, rapid two-stage arterial switch operation in an infant with dextrotransposition of great arteries with an intact ventricular septum beyond the appropriate time for arterial switch operation.'A 4-month-old female infant was admitted due to severe cyanosis, respiratory insufficiency, and frequent dia rhea secondary to ischemic colitis. Echocardiographic examination presented marked leftward deviation of the interventricular septum, and left and right ventricular pressures as measured by cardiac catheterization were 40/4 mmHg and 85/2 mmHg, respectively. Fifteen days after a preparatory operation (pulmonary artery band with modified right Blalock-Taussig shunt), left ventricular-right ventricular systolic pressure ratio increased to 105/90. Arterial switch operation associated with the division of the right Blalock-Taussig shunt and the removal of pulmonary artery band was performed. A(leer the operation, the infant showed normal cardiac function with no postoperative complication.
One of the most widely recognized problems in patients with TGA [transposition of the great arteries] after the Senning operation is the occurrence of arrhythmias. From June 1982 through October 1984, 22 patients, 11 with "simple" and 11 with "complex" TGA, underwent correction of TGA by a modification of the Senning operation designed to avoid dysrhythmias. 16 were males and 6 females, with ages ranging from 3 months to 27 months. [mean age 10.8 months] 7 patients [31.8%], 1 with simple TCA and 6 with complex TGA, died in the early post-operative period. The operative mortality was 9.1% in "simple" TGA, and 54.9% in "complex" TGA. There were 4 late deaths but only one of these patients died of a dysrhythmia as a possible factor. The standard ECG [Electrocardiography] checked preoperatively showed sinus rhythm in 17 [77.3%], 1 AV block in 5 [22.7%]. In 22 patients studied postoperative 1st day, the incidence of arrhythmias was 63.6%, with significant incidence compared with preoperative study. [P<0.05]. But, in 15 patients studied postoperatively at intervals from 1 month to 26 months, the incidence of arrhythmias was 26.7%, with no significant incidence compared with preoperative study [P>0.1]. Our results indicate that a modified Senning operation may reduce the frequency of surgically induced arrhythmias. The mean frontal plane P-wave axis also revealed significant deviation to the right side [P<0.05], compared with preoperative study. These findings raise the possibility that what has been called sinus rhythm postoperatively may have a different origin and conduction sequence from normal. But the rhythm seems to be functionally similar.rhythm seems to be functionally similar.
We investigated changes of the size of neoaortic annulus, root, and aortic anastomosis after arterial switch operation for complete transposition of the great arteries performed in infancy. A total of 23 patients were included in this study. Age ranged from 6 to 153 days. Body weight averaged 3.9$\pm$0.8kg and 17 patients were male. The preoperative angiocardiographic dimensions of the pulmonary annulus, the pulmonary root, and the sinotubular junction, standardized to the diameter of descending aorta at the level of diaphragm, were compared to the size of postoperative measurements of the neoaortic annulus, the neoaortic root, and the aortic an stomosis at a mean interval of 17.2$\pm$ 9.4 months. Mean dimensions of the neoaortic annulus and the neoaortic root were significantly increased postoperatively(n=23, annulus; p<0.01, root; p<0.01), however, those of the aortic anastomosis did not reveal significant change(n=23, p=0.06). There were no significant differences in changes of diameters of the neoaortic annulus, the root, and the aortic anastomosis between patients with(n=8) and without(n=15) postoperative neoaortic regurgitation(annulus; p=0.32, root; p=0.29, anastomosis; p=0.86). Postoperative dimensions of the neoaortic root and annulus between patients with ventricular septal defect(n: 10) and without ventricular septal defect(ni 13) were not significantly changed compared to the preoperative measurements(annulus; p=0.09, root; p=0.07) but mean diameters of the aortic anastomosis decreased significantly after operation in patients with ventricular septal defect(p=0.04). This study revealed that the site of the aortic an stomosis grows in proportion to patient's somatic growth after arterial switch operation. Although we could not demonstrate the relation between the aortic root dilatation and the postoperative neoaortic regurgitation in this study, a continuous close follow-up might be necessary to detect a possible progression of the aortic root dilatation and the resulting significant aortic valve regurgitation.
Background: Homografts and bioprostheses are most commonly used for Rastelli operation in congenital heart disease, but the limited durability is responsible for multiple reoperations associated with increased morbidity This study evaluated long-term results after Rastelli operation with a mechanical valved conduit. Material and Method: A total of 20 patients underwent Rastelli operation with mechanical valved conduit from January 1990 to July 1992. Operative mortality was 1 of 20 patients, and a retrospective review of 19 patients(10 males, 9 females) was done. Initial diagnosis was congenitally corrected transposition of great arteries(cc-TGA, n=4), complete TGA (n=2), ventricular septal defect with pulmonary atresia(VSD with PA, n=9), truncus arteriosus(n=2), double outlet right ventricle with pulmonary stenosis(DORV with PS, n=2). The mean age at Rastelli operation was $4.6{\pm}3.4$ years, and mean follow-up period was $12.8{\pm}2.7$ years. Patients underwent Rastelli opearation using 16 CarboMedics mechanical valve, and 3 Bjork-Shiley mechanical valve($17{\pm}2$ mm). Result: There were 15 reoperations for failed mechanical valved conduit. The freedom from reoperation at 5 and 10 years was 53% and 37%. Most patients were received oral anticoagulation with warfarin, and maintained the international normalized ratio(INR) of 1.5 to 2.0. There was no anticoagulation or thromboembolism related complication. There was a significant difference in the causes of a conduit failure between early(within 3 years) and late(after 3 years) failure groups. The six patients reported early prosthetic valve failure, mainly due to valvular dysfunction by thrombosis or pannus formation. The other nine patients reported late prosthetic valve failure, mainly due to dacron conduit stenosis at anastomosis sites, whereas their valvar motion was normal except 1 patient. Conclusion: To avoid early prosthetic valve failure, strict anticoagulation therapy would be helpful. About the late development of obstructive intimal fibrocalcific peels within the Dacron conduit, an improvement of conduit material is necessary to reduce late prosthetic valve failure. In selected patients, the long term results were satisfactory.
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