Arterial Switch Operation For D-transposition Research Articles

Cardiac Catheterization Interventions in the Right Ventricular Outflow Tract and Branch Pulmonary Arteries Following the Arterial Switch Operation.

The arterial switch operation for d-transposition of the great arteries achieves anatomic repair but creates the potential for right ventricular outflow tract obstruction as a result of the LeCompte maneuver. The resultant right ventricular hypertension is generally well tolerated but a select group are referred for cardiac catheterization. The outcomes of these catheterizations have not been well described. The objective of this study was to describe the degree and nature of right ventricular outflow tract obstruction found during cardiac catheterization among patients following the arterial switch operationas well as determine the rate of intervention and assess the acute impact of any catheter intervention undertaken. We conducted aretrospective study of patients after arterial switch operation with the LeCompte maneuver and subsequent right heart catheterization. Descriptive statistics were reported, and paired sample t tests were used for analysis. 544 children had an arterial switch operation, of which 110 children (20%) had a cardiac catheterization procedure after surgery and 11% had a right heart catheterization. Of the right heart catheterizations, 90% had an intervention (balloon and/or stent). In the interventional group, the right ventricletosystemic pressure ratio decreased modestly, from 2/3 to half systemic, after balloon dilationand/or stent placement(p < 0.01). No serious complications were observed.

Echocardiographic Midterm and Long-term Outcomes After Arterial Switch Operation for d-Transposition of the Great Arteries: A Single-Institution Experience

INTRODUCTION: Arterial switch operation is the preferred surgical management for d-transposition of the great arteries, but it still has long-term complications, which necessitate continued follow-up of patients. This procedure was first performed in this institution in 1991, and after three decades, there is a need to evaluate midterm and long-term outcomes in this population. METHODS: This is a descriptive study on arterial switch operation patients between January 2010 and March 2019 in this institution. The following parameters were collected through review of charts and online health records (Medtrack): preoperative clinical data, surgical technique and immediate postoperative course and complications, echocardiographic results, and current clinical status. The study was approved by the institutional ethics review board of the hospital. RESULTS: Among the 108 patients included in the study, 35 were long-term patients, and 73 were midterm. A total of 27 patients (25%) were lost to follow-up. Among the remaining patients (n = 81), 76 (94%) were alive. The most common postoperative complications were pulmonary stenosis (n = 11), aortic regurgitation (n = 24), and aortic dilatation (n = 2). One patient had reintervention after surgery for severe pulmonic stenosis. Overall survival function was 93.7% after 5 years (95% confidence interval, 0.81–0.98) and 79.4% after 9 years (95% confidence interval, 0.46–0.93). CONCLUSION: Complications seen in this population are similar to those seen in literature. The overall survival rate after 5 years compares to those of other institutions. However, long-term survival rates were lower, which may be due to poor patient follow-up and a high rate of patient attrition over time. To improve future outcomes, strategies should be implemented to promote continuity of care, and parents should be advised regarding the importance of follow-up. KEYWORDS: arterial switch operation, d-TGA, echocardiography, midterm complications, long term complications, survival rate

Diffuse myocardial fibrosis in adolescents operated with arterial switch for transposition of the great arteries - A CMR study

Diffuse myocardial fibrosis in adolescents operated with arterial switch for transposition of the great arteries - A CMR study

Gothic aortic arch and cardiac mechanics in young patients after arterial switch operation for d-transposition of the great arteries

BackgroundIn patients who have undergone arterial switch operation (ASO) for d-transposition of the great arteries a gothic aortic arch (GA) morphology has been found and it has been associated with abnormal aortic bio-elastic properties. HypothesisGA is frequent in ASO patients and may have an impact on cardiac mechanics. Our study aims were to assess 1- the occurrence of GA in a large sample of patients after ASO; 2- the association between GA and aortic bio-elastic properties; and 3- the impact of GA on left ventricular (LV) function using speckle tracking echocardiography (STE). MethodsWe studied one hundred and five asymptomatic patients, who have undergone first stage ASO for d-TGA, with normal left ventricular ejection fraction (LVEF ≥53%). ResultsForty-six (44%) patients showed a GA (mean age 11.5±7.2years, 26 males) while fifty-nine (56%) patients (mean age 9.6±6.7years, 37 males) did not present GA. The two groups were comparable for age, sex, BSA, and office blood pressure values. In group GA aortic root was significantly dilated (27.4±7.5mm vs. 21.2±6.9mm, p<0.0001), aortic stiffness index (Group GA=1.8±1.2 vs. 1.4±0.7, p=0.025) was significantly increased, left atrial volume was larger (p=0.0145), global longitudinal strain (Group GA=−18.4±2.5% vs. −20.1±3.3%, p=0.012) and basal LV longitudinal strains (Group GA=−16.9±4.8% vs. −20.4±7.0%, p=0.013) were significantly reduced. ConclusionsAfter ASO the presence of a GA is associated with a significantly dilated aortic root, stiffer aortic wall, larger left atrial volume, and worse LV longitudinal systolic deformations, well known predictors of cardiovascular morbidity and mortality.

Five-year experience with immediate extubation after arterial switch operations for transposition of great arteries.

We sought to identify preoperative, intraoperative and anatomical factors associated with immediate extubation (IE) after arterial switch operation for d-transposition of great arteries (dTGA). This was a single-centre retrospective study performed from 1 January 2010 to 30 June 2015. IE was defined as successful extubation in the operating room (OR). Univariate/bivariate regression of preoperative, intraoperative and anatomical variables was used to determine associations with IE. Of 32 patients in the dTGA spectrum (age at operation 6 days), 18 (56%) underwent IE. Twelve (71%) of the 17 patients with an intact ventricular septum and 6 (43%) of the 14 patients with ventricular septal defect (VSD) underwent IE, whereas none of the patients with double outlet right ventricle or aortic arch obstruction ( n = 4) did. Patients who had cardiopulmonary bypass time (CPB) >173 min ( P = 0.01), lowest temperature on CPB (T min) ≤ 30.4°C ( P = 0.04) and aortic cross-clamp time >86 min ( P = 0.04) were more likely to be left intubated at the end of the procedure. There was no significant difference in patient's chronological age, gestational age, post-conceptual age, weight, coronary anatomy or prevalence of VSD between those who did and did not undergo IE. There was a median increase in intensive care unit (ICU) length of stay (LOS) by 1 day (33%, P = 0.03) and ICU costs by $12 338 (15%, P = 0.06) in non-IE patients. The OR turnover time ( P = 0.09) and reintubation rate ( P = 1) at 24 h post-extubation did not differ between those who did and did not have IE. There was no myocardial dysfunction evident on predismissal echocardiography in either group. In this cohort of infants, post repair for TGA, 56% were extubated immediately in the OR. Greater CPB and cross-clamp times and T min ≤ 30.4°C were associated with a lesser likelihood of IE. IE was associated with shorter ICU length of stay.

Children and Adolescents Show Altered Visual Working Memory Related Brain Activity More Than One Decade After Arterial Switch Operation for D-Transposition of the Great Arteries

ABSTRACTThis pilot study investigated neural correlates of visual working memory using functional magnetic resonance imaging (fMRI) in seven patients more than one decade after neonatal arterial switch operation for surgical correction of d-transposition of the great arteries (d-TGA, aged 10–18 years, 1 female). Compared with age and sex matched healthy controls patients showed similar visual working memory performance and a smaller increase in brain activity in the posterior parietal cortex with higher visual working memory load. These findings suggest that patients exhibit altered neural activity within a network that is known to support visuospatial memory and cognition.

Assessment of left ventricle geometry and function after arterial switch operation for D-transposition of the great arteries with intact ventricular septum using two-dimensional echocardiography

Assessment of left ventricle geometry and function after arterial switch operation for D-transposition of the great arteries with intact ventricular septum using two-dimensional echocardiography

Circulation: Clinical Summaries

<i>Circulation:</i> Clinical Summaries

Cardiovascular Outcomes After the Arterial Switch Operation for D-Transposition of the Great Arteries

Data regarding long-term outcomes after the arterial switch operation for D-transposition of the great arteries are scarce. A single-institution retrospective cohort study was conducted to assess cardiovascular outcomes after an arterial switch operation between 1983 and 1999. Patients without follow-up visits within 3 years were contacted and secondary sources of information obtained. Overall, 400 patients, 154 (38.3%) with a ventricular septal defect, 238 (59.5%) with an intact septum, and 9 (2.3%) with a Taussig-Bing anomaly, were followed for a median of 18.7 years. In perioperative survivors, overall and arrhythmia-free survival rates at 25 years were 96.7±1.8% and 96.6±0.1%, respectively. Late mortality was predominantly a result of sudden deaths and myocardial infarction. At 25 years, 75.5±2.5% remained free from surgical or catheter-based reintervention. Freedom from an adverse cardiovascular event was 92.9±1.9% at 25 years. Independent predictors were a single right coronary artery (hazard ratio, 4.58; 95% confidence interval, 1.32-15.90), P=0.0166) and postoperative heart failure (hazard ratio, 6.93; 95% confidence interval, 1.57-30.62; P=0.0107). At last follow-up, the left ventricular ejection fraction was 60.3±8.9%, 97.3% had class I symptoms, and 5.2% obstructive coronary artery disease. Peak oxygen uptake was 35.1±7.6 mL/kg/min (86.1±15.1% predicted), with a chronotropic index <80% in 34.2%. At least moderate neoaortic and pulmonary regurgitation were present in 3.4% and 6.6%, respectively, and more than mild neoaortic and pulmonary stenosis in 3.2% and 10.3%. Long-term and arrhythmia-free survival is excellent after arterial switch operation. Although sequelae include chronotropic incompetence and neoaortic, pulmonary, and coronary artery complications, most patients maintain normal systolic function and exercise capacity.

Late Manifestation of Coarctation of the Aorta After Arterial Switch Operation for D-Transposition of the Great Arteries With Intact Ventricular Septum

We describe an infant with D-transposition of the great arteries with an intact ventricular septum who developed coarctation of the aorta 13days after arterial switch operation. A mildly hypoplastic aortic isthmus was present on echocardiogram at the time of the arterial switch operation, but there was no echocardiographic or clinical evidence of coarctation of the aorta.

Electrophysiology Issues In Adult Congenital Heart Disease

Improved surgical outcomes in children have led to a growing population of adults with congenital heart disease. Rhythm disturbances in the adult congenital heart disease (ACHD) patient can be intrinsic to the anomaly or acquired after palliation. Tachyarrhythmias, either supraventricular or ventricular, and bradyarrhythmias, either sinus node dysfunction or atrioventricular block, may occur frequently. Technological advances in intervention and surgical approaches have led to prophylactic and therapeutic reduction in arrhythmias. In order of escalation, this article addresses medical management, catheter ablation, device therapy for antitachycardia pacing and defibrillation, and surgical intervention. There are now an estimated one million-plus ACHD patients living in the United States. An estimated 45% of those have simple defects (e.g., atrial septal defects), 40% have moderately complex defects (e.g., tetralogy of Fallot, or ToF), and 15% have severely complex defects (e.g., single ventricle anatomy or surgical palliations for transposition of the great arteries [TGA]). The moderate and severe categories have a high incidence of arrhythmia. Of older repaired ToF patients, 34% develop symptomatic atrial or supraventricular tachycardias, 8.5% develop high-grade ventricular tachycardia (VT), and there are an increasing number of implantable defibrillators due to a sudden-death estimate of 2% per decade. Thus, an estimated 50,000 adults with repaired ToF will require electrophysiology follow-up with 100 sudden deaths per year nationally. Roughly 3% of all congenital heart surgeries requiring cardiopulmonary bypass have post-operative AV block, and 1% will require permanent pacing. Older atriopulmonary Fontans have up to an estimated 50% incidence of atrial tachycardia within a decade of palliation due to suture lines and elevated atrial pressures. Patients who have undergone an atrial switch operation (e.g., Mustard or Senning operations) are rarely exclusively in sinus rhythm a decade after repair, thus posing problems for rhythm control and anticoagulation. Catheter ablation in congenital heart disease is often more challenging than structurally normal hearts because of abnormal anatomy (congenital and/or post-surgical) and thicker chamber walls due to unfavorable hemodynamics. In preparation, review of noninvasive imaging, previous catheterization angiography, and surgical palliation reports are paramount prior to a procedure. Fortunately, to meet the growing challenge, newer software with 3-D packages allow for improved mapping. In addition, advances with larger-tipped and irrigated-tip catheters allow for deeper, more effective lesions to be placed. Several advances in surgical approach have led to a reduction in arrhythmias. For example, the arterial switch operation for d-transposition of the great arteries instead of the aforementioned atrial switch operation eliminates the use of a systemic right ventricle that can later develop intra-atrial reentrant tachycardia and sinus node dysfunction. Other advances include paying careful attention to minimizing ventriculotomies in ToF and ventricular septal defect (VSD) repairs, earlier complete repairs, and valve sparing to reduce pulmonary insufficiency. Finally, completion of the extra-cardiac Fontan procedure (e.g., total cavopulmonary connection) for single ventricles avoids extensive suture lines in the right atrium, thereby reducing scarring and higher pressures that lead to IART and sinus node dysfunction. Extracardiac (EC) conduits and lateral tunnel (LT) Fontans are preferred today, and the Fontan conversion procedure (converting prior atriopulmonary Fontans to the EC or LT type) can be performed to reduce arrhythmia and thromboembolic events.

Delayed Arterial Switch Operation for D-Transposition of the Great Arteries and Glucocorticoid Remediable Aldosteronism

The Jatene arterial switch operation (ASO) for dextro-transposition of the great arteries is ideally performed within the first 2 weeks of life. Clinical circumstances, however, may dictate a delayed ASO and left ventricle "retraining" prior to the procedure. Glucocorticoid remediable aldosteronism (GRA) accounts for 0.5% to 1% of primary aldosteronism. It presents as severe hypertension in infants and children, with poor response to standard antihypertensive medications. To the authors' knowledge, this is the first reported case of GRA in the context of transposition physiology. The management of GRA and a rationale for delayed ASO are discussed.

An Approach To Imaging Adult Congenital Heart Disease : Pitfals And Pearls

Noninvasive cardiovascular evaluation of an adult congenital heart disease (ACHD) patient is a multimodality endeavor that can include chest roentgenograms, electrocardiograms, echocardiography (including stress, three-dimensional, intravascular and intracardiac ultrasound, and transesophageal), cardiac magnetic resonance imaging (CMR), computed tomography angiography (CTA), single photon emission computed tomographic (SPECT) perfusion imaging, and positron emission tomography (PET). The cost-benefit ratio and limitations of each modality should always be considered (Table 1). Adults with ACHD often need repetitive imaging, making them vulnerable to radiation-induced cancer; hence, modalities using ionizing radiation should be minimized. A transthoracic echocardiogram remains the initial noninvasive modality of choice due to its real-time imaging capability with excellent temporal resolution and its ability to quickly assess hemodynamics along with anatomic delineation. A transesophageal echocardiogram is extremely helpful in further defining intracardiac anatomy and guiding surgical and interventional procedures. Cardiac magnetic resonance imaging is an adjunctive modality to an echocardiogram because ACHD patients frequently have intra-thoracic abnormalities (e.g., adhesions from multiple cardiac operations and lung disease) that can limit the resolution of the ultrasound beam. A CMR is especially helpful in periodic evaluation of the right ventricle, visceral situs, extracardiac anatomy, lesions of the pericardium, pulmonary veins, systemic veins, pulmonary arteries, and aorta. For real-time CMR, special techniques such as echo planar can be used; for hemodynamic evaluation by CMR, techniques like velocity-vector mapping, myocardial tagging, echo planar, and fast gradient echo can be used. Besides excellent spatial resolution, the advantage of CTA over other techniques is an extremely fast acquisition time. Furthermore, CTA can replace an invasive procedure such as catheterization and angiography for anatomical diagnosis where hemodynamics is not required, such as the diagnosis of arch or coronary artery anomalies. Nuclear scans such as lung perfusion scans and splenic scans are very helpful in determining differential pulmonary blood flow and splenic function, respectively. Both SPECT and PET are valuable in assessing tissue perfusion and metabolism and help to determine viable versus nonviable myocardium after surgery for hypoplastic left heart syndrome and coronary artery anomaly, after arterial switch operation for d-transposition of great arteries, and in acquired illness such as Kawasaki disease. When considering imaging a patient with ACHD, and overall evaluation strategy should include the aspects found in Table 2.

Elective use of left ventricular assist after delayed arterial switch operation for D-transposition of the great vessels with intact ventricular septum

Elective use of left ventricular assist after delayed arterial switch operation for D-transposition of the great vessels with intact ventricular septum

Late Results After PTCA for Coronary Stenosis After the Arterial Switch Procedure for Transposition of the Great Arteries

The arterial switch operation has become the surgical approach of choice for d-transposition of the great arteries, but there is an increased awareness of adverse sequelae in some survivors. Long-term patency and normal function of the translocated coronary arteries must be achieved. It is reported that dependent of the prior coronary status, 3% to 11% of all survivors have proximal coronary stenosis or complete occlusion develop after arterial switch operations. However, treatment of these stenoses is still a matter of debate. Late results after percutaneous transluminal coronary angioplasty (PTCA) for coronary stenosis after the arterial switch operation for d-transposition of the great arteries are reported. Seven children after arterial switch operation for d-transposition of the great arteries who had subsequently undergone PTCA for coronary stenosis were angiographically re-evaluated 3 to 15 months after the initial PTCA and again after 3 to 5 years. All children survived the initial PTCA procedure. There were no late deaths. The degree of stenosis before PTCA ranged from 74% to 97%; immediately after PTCA from 5% to 10%; at 3 to 15 months after PTCA from zero to 6%; and at 3 to 5 years after PTCA from zero to 3%. Three to 5 years after PTCA all children showed normal development of the treated coronary artery. Primary PTCA of stenotic proximal coronary arteries after the arterial switch procedure for d-transposition of the great arteries seems to be an effective treatment with excellent long-term results.

Stress echocardiography following the arterial switch operation for D-transposition of the great arteries

Stress echocardiography following the arterial switch operation for D-transposition of the great arteries

Growth after neonatal arterial switch operation for D-transposition of the great arteries.

The objective of this study was to evaluate growth in infants with d-transposition of the great arteries (d-TGA) who underwent switch operation (anatomical correction) in the early neonatal period. Growth data (at birth and 1, 3, 6, 12, and 24 months of age) were retrieved in 23 infants with d-TGA who survived the operation without major complications. Measures were transformed into z scores and compared with normative data for age. Following surgery, infants showed low z scores for weight, height, and head circumference. Weight and height showed a trend to reach normal ranges between 6 and 12 months of age, and the z scores were positive at 24 months. z scores for head circumference were still negative at 24 months of age. No dietary interventions were implemented during this period, and the infants were fed their own mothers' milk or, before discharge, high-calorie, preterm formula. Following anatomical correction for d-TGA in the early neonatal period, infant growth shows a characteristic pattern, with parameters normalized by 12 months of age in most cases. Head growth shows a different pattern, although gross motor development seems unaffected.

Exercise performance after the arterial switch operation for d-transposition of the great arteries

Early- and intermediate-term results of the arterial switch operation for D-transposition of the great arteries (D-TGA) are encouraging. However, questions remain about the long-term outcome for these patients, especially with regard to exercise performance. Preliminary studies have demonstrated normal endurance time on treadmills. However, data regarding aerobic capacity and cardiopulmonary function are lacking. We report the cardiopulmonary performance of 22 school-age patients with D-TGA who underwent the arterial switch operation. Outcome variables included maximal oxygen consumption (VO2), maximal cardiac index, and peak heart rate. Patient and procedure-related variables were assessed for their association with outcome variables using linear and logistic regression. The mean values of maximal VO2 (113 ± 19% predicted) and maximal cardiac index (96 ± 18% predicted) were within normal limits for the pediatric population. Although the mean peak heart rate for the entire group (184 ± 14 beats/min) was within normal limits, there were 7 subjects (32%) with chronotropic impairment. Significant ST-segment depression was seen in 2 subjects (9%). In regression analysis, surgery subsequent to the arterial switch was associated with lower maximal cardiac index (p = 0.01). Other variables were not significantly associated with maximal VO2, maximal cardiac index, and peak heart rate. In particular, chronotropic impairment was not significantly associated with maximal VO2 or maximal cardiac index. These findings demonstrate that cardiopulmonary performance during exercise is excellent after the arterial switch operation. The finding of ST-segment depression in some subjects supports the role of formal exercise testing in those patients participating in vigorous athletic activities.

Simultaneous assessment of myocardial glucose metabolism and contractile function by gated F-18-deoxyglucose positron emission tomography in infants after arterial switch operation for d-transposition of the great arteries

The arterial switch operation is now the treatment of choice for anatomic correction of dextro-transposition of the great arteries (d-TGA) in infancy. Early and late morbidity and mortality are related in part to impaired coronary perfusion due to stenosis, compression, kinking, or occlusion of the transferred coronary arteries. To guide further therapy in these patients, evaluation of myocardial viability in akinetic or hypokinetic regions is required. F-18-deoxyglucose positron emission tomography (FDG-PET) is well-established for the investigation of myocardial viability in adults. However, there are no reports about the impact of FDG-PET in infants. We applied ECG-triggered (7–12 gates) PET using a 30-min protocol for complete assessment of the metabolic and functional status of the myocardium in five infants with suspected or confirmed myocardial injury after the arterial switch operation. Left ventricular (LV) dysfunction was present in four infants. Coronary angiography revealed stenosis or occlusion of one or more coronary vessels in three patients. F-18-FDG (5–7 Mbq/kg) was injected after stimulation of glycolysis. Gated data acquisition was started 30 minutes after injection for a 20-min emission period, followed by a 10-min transmission. Glucose metabolism, wall motion, and wall thickening were evaluated visually and quantitatively from parametric 3-D images generated by a new software for wall delineation. In one infant with severely impaired LV function, PET demonstrated viable myocardium in hypokinetic regions except for a small non-transmural infarction in the apex, providing an objective indication for surgical revascularization. Another infant with severe stenosis of the left coronary artery showed no metabolic abnormality and was scheduled for coronary angioplasty. A third patient with kinking of the left and right coronary arteries had a large transmural defect corresponding to an apical and anterior infarction. These preliminary results suggest that myocardial viability and contractile function can be assessed simultaneously by gated FDG-PET in order to guide further therapy in infants with coronary stenosis or occlusion.

Usefulness of magnetic resonance imaging for evaluating great-vessel anatomy after arterial switch operation for D-transposition of the great arteries

Magnetic resonance imaging (MRI) produces high-resolution images of great-vessel anatomy in pediatric patients. In this study seven patients, aged 6 to 27 months were evaluated by using gated MRI and two-dimensional echocardiography 4 to 26 months after arterial switch operation for D-transposition of the great arteries. Measurements were taken at the right and left ventricular outflow tracts, beneath the semilunar valves, at the midaortic sinuses, at the anastomotic sites of the main pulmonary artery and the aorta, at the origin of the branch pulmonary arteries, and at the distal pulmonary arteries 1 cm beyond the bifurcation. Concordant results were obtained with both imaging techniques from all sides with the exception of the left pulmonary artery and the right pulmonary artery. With MRI, four patients had significant narrowing at the right pulmonary artery origin and six patients had narrowing at the left pulmonary artery origin. With two-dimensional echocardiogram, two patients had narrowing at the right pulmonary artery origin and four patients had narrowing at the left pulmonary artery origin. The measured pulmonary artery intraluminal diameters in these patients were consistently smaller when assessed by MRI versus two-dimensional echocardiography. To verify these results, five of seven patients underwent cardiac catheterization to provide physiologic correlation before reoperation; the MRI results were found to be significantly closer to the actual catheterization measurements. We conclude that MRI is a sensitive imaging technique for evaluation of great-vessel anatomy in patients after arterial switch operation for D-transposition of the great arteries. It is particularly useful in the evaluation of the branch pulmonary artery anatomy.