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Senning operation for correction of the transposition of the great arteries, results, long-term outcome and quality of life  [PDF]
Miguel Angel Maluf
World Journal of Cardiovascular Diseases (WJCD) , 2012, DOI: 10.4236/wjcd.2012.23036
Abstract: Objective: Long-term results after the Senning operation for transposition of the great arteries are little known. Sinus node dysfunction and systemic ventricular dysfunction are crucial in patient survival. We evaluated the results, long term outcome and quality of life in a group of 39 patients. Methods: The study was a retrospective analysis, of 39 (39/40 = 97.5%) surgical surviving patients, submitted to Senning operation, with a mean follow-up time of 14.7 +/– 3.1 years. Electrocardiogram, echocardiogram, and chest radiograph series were performed every 6 months. Thirty six patients of this series underwent Holter study and ergometric test, to evaluate the physical capacity. Three patients living overseas were excluded. Results: There was only one late death (1/39) (late mortality = 2.5%): a 16 year-old patient had a no cardiac death. The actuarial survival was 95.0% (38/40) (simple or with little VSD, TGA). The probability of staying in sinus rhythm, in 39 surviving patients was 77.1% (30) or normal right ventricular function was 76.5% (29), 10 to 20 years after operation. The incidence of sinus node and right ventricular dysfunction increased gradually over time. No re-operations and pacemaker implantation, was performed. Functional class: I = 30 (85.7%) cases and functional class II = 5 (14.3%) cases. Conclusions: Patients with simple TGA submitted to Senning procedure in our experience, presented during late follow-up: 1—Low incidence of right ventricular dysfunction and active arrhythmias; 2—Low mortality and no sudden death recorded; 3—Good quality of life and 4—Satisfactory surgical results (free of re-operation or definitive pacemaker implantation).
Serial exercise testing in children, adolescents and young adults with Senning repair for transposition of the great arteries  [cached]
Buys Roselien,Budts Werner,Reybrouck Tony,Gewillig Marc
BMC Cardiovascular Disorders , 2012, DOI: 10.1186/1471-2261-12-88
Abstract: Background Patients with Senning repair for complete transposition of the great arteries (d-TGA) show an impaired exercise tolerance. Our aim was to investigate changes in exercise capacity in children, adolescents and adults with Senning operation. Methods Peak oxygen uptake (peak VO2), oxygen pulse and heart rate were assessed by cardiopulmonary exercise tests (CPET) and compared to normal values. Rates of change were calculated by linear regression analysis. Right ventricular (RV) function was assessed by echocardiography. Results Thirty-four patients (22 male) performed 3.5 (range 3–6) CPET with an interval of ≥ 6 months. Mean age at first assessment was 16.4 ± 4.27 years. Follow-up period averaged 6.8 ± 2 years. Exercise capacity was reduced (p<0.0005) and the decline of peak VO2 ( 1.3 ± 3.7 %/year; p=0.015) and peak oxygen pulse ( 1.4 ± 3.0 %/year; p=0.011) was larger than normal, especially before adulthood and in female patients (p<0.01). During adulthood, RV contractility changes were significantly correlated with the decline of peak oxygen pulse (r= 0.504; p=0.047). Conclusions In patients with Senning operation for d-TGA, peak VO2 and peak oxygen pulse decrease faster with age compared to healthy controls. This decline is most obvious during childhood and adolescence, and suggests the inability to increase stroke volume to the same extent as healthy peers during growth. Peak VO2 and peak oxygen pulse remain relatively stable during early adulthood. However, when RV contractility decreases, a faster decline in peak oxygen pulse is observed.
Ventricular assist device outflow graft in congenitally corrected transposition of great arteries - a surgical challenge  [cached]
Mohite Prashant N,Popov Aron F,Garcia Diana,Hards Rachel
Journal of Cardiothoracic Surgery , 2012, DOI: 10.1186/1749-8090-7-93
Abstract: Congenitally corrected transposition of the great arteries is a complex congenital cardiac anomaly with a wide spectrum of morphologic features and clinical profiles. Most patients are diagnosed late in their life, undergoes surgical repairs, eventually leading to systemic ventricular failure needing heart transplant or mechanical circulatory assistance. As, aorta is located anterior to and left of the PA (Transposition of great arteries), the outflow graft of ventricular assist device traverse across pulmonary artery to reach aorta which poses challenge during further surgical explorations.
One-year outcome of Senning operation in children with transposition of the great vessels
Shahmohammadi A,Mortezaian H,Alipour MR
Tehran University Medical Journal , 2011,
Abstract: "nBackground: Nowadays, the treatment of choice for anatomical correction of transposition of great arteries is arterial switch but some children are not good candidates for this operation. In these cases atrial switch or Senning procedure is an accepted method, thus outcome of this procedure needs to be better delineated."n "nMethods: This prospective study included 65 patients that underwent Senning operation in Shahid Rajaee Hospital in Tehran, Iran from 2002 to 2009 and were followed-up for one year."n "nResults: The early and late mortality rates were 16.9% and 1.5%, respectively. 51.8% of early deaths were due to simple Senning procedure, 38.9% due to a Senning with the closure of ventricular septal defect, with or without the repair of pulmonary artery stenosis, 9.3% related to palliative Senning and one late death due to simple Senning. The most common arrhythmia was accelerated junctional rhythm (18.5%). 15% of cases had Senning pathway obstruction and 1.7% had baffle leaks. Regardless of the mortalities, three patients (5.7%) had significant tricuspid regurgitation. Right ventricular systolic dysfunction was found in 13.3% of the cases, primarily in Senning with ventricular septal defect closure (37.5%). One case (1.9%) had visible cyanosis and three (5.7%) had exertional dyspnea but 94.3% of cases remained in functional class I."n "nConclusion: Since the majority of deaths following Senning procedure occur during the first month, especially on the second day post-operatively, assiduity during early post-operative period is crucial. Tricuspid regurgitation or right ventricular systolic dysfunction was not detected in most patients, suggesting optimistic prognosis for these patients.
Left ventricular outflow tract obstruction by accessory mitral tissue. A review regarding a case.
Iovank González Artiles,Raymid García Fernández,Guillermo Mojenas Morfa,Benito Sainz González de la Pe?a
Revista Cubana de Cardiología y Cirugía Cardiovascular , 2011,
Abstract: The presence of accessory mitral valve tissue is a rare congenital abnormality that rarely,causes left ventricular outflow tract obstruction. Its presentation in adults is uncommon. Wepresent a 38-year old man diagnosed with this malformation in our hospital. Echocardiographyfacilitated the diagnosis of left ventricular outflow tract obstruction. The patient underwenta successful operation for the replacement of the abnormal mitral valve with a prostheticone. We also present a review of this subject.
Transposition of the great arteries
Paula Martins, Eduardo Castela
Orphanet Journal of Rare Diseases , 2008, DOI: 10.1186/1750-1172-3-27
Abstract: The diagnosis is confirmed by echocardiography, which also provides the morphological details required for future surgical management. Prenatal diagnosis by foetal echocardiography is possible and desirable, as it may improve the early neonatal management and reduce morbidity and mortality. Differential diagnosis includes other causes of central neonatal cyanosis. Palliative treatment with prostaglandin E1 and balloon atrial septostomy are usually required soon after birth. Surgical correction is performed at a later stage. Usually, the Jatene arterial switch operation is the procedure of choice. Whenever this operation is not feasible, adequate alternative surgical approach should be implemented. With the advent of newer and improved surgical techniques and post operative intensive care, the long-term survival is approximately 90% at 15 years of age. However, the exercise performance, cognitive function and quality of life may be impaired.Transposition of the great arteries; physiologically uncorrected transposition; complete transposition; atrioventricular concordance with ventriculoarterial discordance. The European paediatric cardiac code for this disease is 01.05.01.The transposition of the great arteries was first described by Mathew Baillie in 1797, in the second edition of the book "The Morbid Anatomy of Some of the Most Important Parts of the Human Body". However, the term transposition was only applied in 1814, by Farre, meaning that aorta and pulmonary trunk were placed (positio) across (trans) the ventricular septum.In fact, this congenital cardiac malformation is characterised by atrioventricular concordance and ventriculoarterial discordance. In other words, the morphological right atrium is connected to the morphological right ventricle which gives rise entirely to or most of the aorta; the morphological left atrium is connected to the morphological left ventricle from where the pulmonary trunk emerges [1].The term congenitally corrected transposition
Palliation for transposition of great arteries
VO Adegboye, SI Omokhodion, O Ogunkunle, AI Brimmo, OA Adepo
Nigerian Journal of Surgical Research , 2003,
Abstract: Background: At the University College Hospital Ibadan we have no facility for total surgical correction of transportation of the great arteries (TGA). This prospective study reviews the palliative procedures we have used in the management of TGA. Method: Patients with the diagnosis of TGA were evaluated for morphological type. The choice of palliative procedure was made in some of the patients with morphological type in mind. No fixed criteria were used for allocating patients to Blalock-Hanlon (B-H), atrial septectomy while pulmonary banding (PB) and Blalock-Taussig (B-T) shunt have definite indications. Results: Fourteen consecutive patients with TGA were palliated. The ages of these patients ranged between 3 to 11 months (6.8 ± 2.4 months), there were 8 males to 6 females (1.3:1). Six patients had B-H atrial septectomy and 2(33.3%) died within 48 hours, 4 patients had B-T shunt and there were no mortality, 4 patients had PB and 2 (50.0%) died within 72 hours. The overall operative mortality was 28.6%. All the 10 survivors had improvement of their clinical features and fall in packed cell volume during the period of follow-up, which lasted 5 to 13 months (mean 9.3 ± 1.2 months). All patients had delayed wound healing. Conclusion: Appropriate and timely palliative surgery has a place in patients with TGA as an interim care. Key Words: Palliative surgery, transposition, great arteries Nigerian Journal of Surgical Research Vol.5(1&2) 2003: 129-133
A Case of Congenitally Corrected Transposition of the Great Arteries Discovered on Coronary Computed Tomography  [PDF]
Adam T. Marler,Jennifer N. Slim,Travis Batts,James Watts,Ahmad M. Slim
Case Reports in Vascular Medicine , 2013, DOI: 10.1155/2013/420213
Abstract: Congenitally corrected transposition of the great arteries is a rare condition accounting for less than 1% of all congenital cardiac diseases. The fundamental nature of this condition involves a blend of atrioventricular as well as ventriculoarterial discordance. Congenitally corrected transposition of the great arteries is classically associated with three additional abnormalities, including ventricular septal defect, right ventricular outflow tract obstruction, and tricuspid valve abnormalities. Patients with this anomaly have been shown to exhibit reduced exercise tolerance as well as reduced health-related quality of life when compared to patients with normal cardiovascular anatomy. We present the case of a 33-year-old active duty lieutenant in the United States Air Force referred to the cardiology clinic for evaluation of valvular heart disease with subsequent discovery of congenitally corrected transposition of the great arteries on cardiac gated computed tomography. 1. Case Report A 33-year-old male with a history of secundum atrial septal defect percutaneously ended in May 2008 referred for further evaluation of valvular heart disease. Transthoracic echocardiogram completed for a heart murmur was noted to be technically difficult with suboptimal image quality due to mesocardia. Official report from this examination detailed grossly normal left ventricular wall motion with moderate, posteriorly directed, eccentric mitral regurgitation, mild prolapse of the anterior mitral valve leaflet, mild aortic insufficiency, and an echo bright structure contiguous to the lateral aspect of the tricuspid annulus. The right ventricle and right atrium were not well visualized. At initial visit he reported feeling well overall, but he felt fatigued at 200–400 meters. Exercise stress testing was completed with the patient exercising 12 minutes 26 seconds on a standard Bruce protocol achieving a workload of 14.2 metabolic equivalents (METS) achieving 96% of maximal age-predicted heart rate with exercise. Exercise testing terminated due to fatigue with the patient reporting no chest pain during evaluation. No ischemic changes were noted on electrocardiogram during the study. Patient’s chest X-ray was suggestive of possible RV on the left side of the chest with possible dextrocardia or ccTGA in the differential (Figure 1). Figure 1: X-ray changes anticipated in ccTGA with right ventricular border outlined with red arrow and Amplatzer occluder device outlined with dark arrow. Subsequently, the patient underwent imaging evaluation with cardiac computed tomography (CT)
Transposition of Great Arteries: New Insights into the Pathogenesis  [PDF]
Marta Unolt,Carolina Putotto,Lucia M. Silvestri,Dario Marino,Alessia Scarabotti,Valerio Massaccesi,Angela Caiaro,Paolo Versacci,Bruno Marino
Frontiers in Pediatrics , 2013, DOI: 10.3389/fped.2013.00011
Abstract: Transposition of great arteries (TGA) is one of the most common and severe congenital heart diseases (CHD). It is also one of the most mysterious CHD because it has no precedent in phylogenetic and ontogenetic development, it does not represent an alternative physiological model of blood circulation and its etiology and morphogenesis are still largely unknown. However, recent epidemiologic, experimental, and genetic data suggest new insights into the pathogenesis. TGA is very rarely associated with the most frequent genetic syndromes, such as Turner, Noonan, Williams or Marfan syndromes, and in Down syndrome, it is virtually absent. The only genetic syndrome with a strong relation with TGA is Heterotaxy. In lateralization defects TGA is frequently associated with asplenia syndrome. Moreover, TGA is rather frequent in cases of isolated dextrocardia with situs solitus, showing link with defect of visceral situs. Nowadays, the most reliable method to induce TGA consists in treating pregnant mice with retinoic acid or with retinoic acid inhibitors. Following such treatment not only cases of TGA with d-ventricular loop have been registered, but also some cases of congenitally corrected transposition of great arteries (CCTGA). In another experiment, the embryos of mice treated with retinoic acid in day 6.5 presented Heterotaxy, suggesting a relationship among these morphologically different CHD. In humans, some families, beside TGA cases, present first-degree relatives with CCTGA. This data suggest that monogenic inheritance with a variable phenotypic expression could explain the familial aggregation of TGA and CCTGA. In some of these families we previously found multiple mutations in laterality genes including Nodal and ZIC3, confirming a pathogenetic relation between TGA and Heterotaxy. These overall data suggest to include TGA in the pathogenetic group of laterality defects instead of conotruncal abnormalities due to ectomesenchymal tissue migration.
Response of the oxygen pulse during exercise in children with atrial repair for transposition of the great arteries  [cached]
Buys R,Dockx K,Gewillig M,Reybrouck T
Research Reports in Clinical Cardiology , 2012,
Abstract: Roselien Buys,1,2,* Kimberly Dockx,1,* Marc Gewillig,3 Tony Reybrouck1,21Department of Rehabilitation Sciences, 2Department of Cardiovascular Rehabilitation, University of Leuven, Leuven, Belgium; 3Department of Pediatric Cardiology, University Hospital Gasthuisberg, Leuven, Belgium*These authors contributed equally to this paperBackground: The oxygen pulse equals stroke volume times arterial–venous oxygen difference and is calculated by dividing oxygen uptake (VO2) by heart rate (HR). In children with a Senning repair for the transposition of the great arteries (TGA), the response of both HR and VO2 to exercise is impaired. Our aim was to assess the oxygen pulse response during exercise in patients who underwent a Senning operation, comparing it with healthy controls.Methods: Twenty-one children with a Senning repair (mean age 12.5 ± 1.7 years) and a control group of 31 healthy children (mean age 13.2 ± 2.0 years) performed a graded maximal exercise test on a treadmill, during which HR and VO2 were measured. Oxygen pulse was calculated by dividing VO2 by HR. Right ventricular function was quantitatively assessed by cardiac ultrasound.Results: Senning patients had a lower peak oxygen pulse than the control (P = 0.0024) (8.45 ± 1.90 mL beats-1 versus 11.7 ± 3.93 mL beats-1), as with the peak VO2 (P < 0.001) (35.8 ± 5.67 mL min-1 kg-1 versus 46.6 ± 8.02 mL min-1 kg-1) and peak HR (171 ± 14 beats min-1 versus 188 ± 11 beats min-1). During submaximal exercise, oxygen pulse and VO2 were also significantly lower in Senning patients when compared to the control group (P = 0.027). In seven Senning patients (33%), the oxygen pulse did not increase any further after the first exercise levels. These patients had lower right ventricular function compared to the control group (P = 0.04).Conclusion: Children with a Senning repair for TGA have a reduced peak VO2, peak HR, and peak oxygen pulse. Their oxygen pulse starts off at a lower level, reaches its plateau earlier, and is related to right ventricular function. This variable can be considered a complementary parameter to assess cardiovascular exercise performance.Keywords: transposition of the great arteries, Senning repair, oxygen pulse, cardiopulmonary exercise testing
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