Dextro-transposition Of The Great Arteries Research Articles

Relationship of white matter network topology and cognitive outcome in adolescents with d-transposition of the great arteries.

Patients with congenital heart disease (CHD) are at risk for neurocognitive impairments. Little is known about the impact of CHD on the organization of large-scale brain networks. We applied graph analysis techniques to diffusion tensor imaging (DTI) data obtained from 49 adolescents with dextro-transposition of the great arteries (d-TGA) repaired with the arterial switch operation in early infancy and 29 healthy referent adolescents. We examined whether differences in neurocognitive functioning were related to white matter network topology. We developed mediation models revealing the respective contributions of peri-operative variables and network topology on cognitive outcome. Adolescents with d-TGA had reduced global efficiency at a trend level (p = 0.061), increased modularity (p = 0.012), and increased small-worldness (p = 0.026) as compared to controls. Moreover, these network properties mediated neurocognitive differences between the d-TGA and referent adolescents across every domain assessed. Finally, structural network topology mediated the neuroprotective effect of longer duration of core cooling during reparative neonatal cardiac surgery, as well as the detrimental effects of prolonged hospitalization. Taken together, worse neurocognitive function in adolescents with d-TGA is mediated by global differences in white matter network topology, suggesting that disruption of this configuration of large-scale networks drives neurocognitive dysfunction. These data provide new insights into the interplay between perioperative factors, brain organization, and cognition in patients with complex CHD.

Executive Function in Children and Adolescents with Critical Cyanotic Congenital Heart Disease.

Children and adolescents with critical cyanotic congenital heart disease (CHD) are at risk for deficits in aspects of executive function (EF). The primary aim of this investigation was to compare EF outcomes in three groups of children/adolescents with severe CHD and controls (ages 10-19 years). Participants included 463 children/adolescents with CHD [dextro-transposition of the great arteries (TGA), n=139; tetralogy of Fallot (TOF), n=68; and, single-ventricle anatomy requiring Fontan procedure (SVF), n=145] and 111 controls, who underwent laboratory and informant-based evaluation of EF skills. Rates of EF impairment on D-KEFS measures were nearly twice as high for CHD groups (75-81%) than controls (43%). Distinct EF profiles were documented between CHD groups on D-KEFS tasks. Deficits in flexibility/problem-solving and verbally mediated EF skills were documented in all three CHD groups; visuo-spatially mediated EF abilities were impaired in TOF and SVF groups, but preserved in TGA. Parent, teacher, and self-report ratings on the BRIEF highlighted unique patterns of metacognitive and self-regulatory concerns across informants. CHD poses a serious threat to EF development. Greater severity of CHD is associated with worse EF outcomes. With increased understanding of the cognitive and self-regulatory vulnerabilities experienced by children and adolescents with CHD, it may be possible to identify risks early and provide individualized supports to promote optimal neurodevelopment.

White Matter Microstructure and Cognition in Adolescents with Congenital Heart Disease

To describe the relationship between altered white matter microstructure and neurodevelopment in children with dextro-transposition of the great arteries (d-TGA). We report correlations between regional white matter microstructure as measured by fractional anisotropy (FA) and cognitive outcome in a homogeneous group of adolescents with d-TGA. Subjects with d-TGA (n = 49) and controls (n = 29) underwent diffusion tensor imaging and neurocognitive testing. In the group with d-TGA, we correlated neurocognitive scores with FA in 14 composite regions of interest in which subjects with d-TGA had lower FA than controls. Among the patients with d-TGA, mathematics achievement correlated with left parietal FA (r = 0.39; P = .006), inattention/hyperactivity symptoms correlated with right precentral FA (r = -0.39; P = .006) and left parietal FA (r = -0.30; P = .04), executive function correlated with right precentral FA (r = -0.30; P = .04), and visual-spatial skills correlated with right frontal FA (r = 0.30; P = .04). We also found an unanticipated correlation between memory and right posterior limb of the internal capsule FA (r = 0.29; P = .047). Within the group with d-TGA, regions of reduced white matter microstructure are associated with cognitive performance in a pattern similar to that seen in healthy adolescents and adults. Diminished white matter microstructure may contribute to cognitive compromise in adolescents who underwent open-heart surgery in infancy.

Case-control analysis of maternal prenatal analgesic use and cardiovascular malformations: Baltimore–Washington Infant Study

We sought to assess maternal prenatal use of analgesics and risk of cardiovascular malformations (CVM) in the offspring. Data from the Baltimore-Washington Infant Study, a population-based case-control investigation of CVM, were used to examine selected isolated CVM diagnoses and maternal analgesic use during the periconceptional period (3 months before and after conception). We compared case and control infants on frequency of maternal use of analgesics and estimated adjusted odds ratios (adjORs) and 95% confidence intervals (CI) with logistic regression models for specific CVM phenotypes. Frequency of periconceptional use of any analgesic was 52% among control mothers and 53% among case mothers. Analyses by CVM diagnoses identified an association of tetralogy of Fallot with maternal acetaminophen use (adjOR, 1.6; 95% CI, 1.1-2.3) and dextrotransposition of the great arteries with intact ventricular septum with maternal nonsteroidal antiinflammatory drug use (adjOR, 3.2; 95% CI, 1.2-8.7). Analgesic use during the periconceptional period was not associated with CVM in the aggregate or with most phenotypes of CVM examined. Associations with 2 phenotypes of CVM may have occurred by chance. These findings warrant corroboration and further study, including further evaluation of the observed associations, the dose of analgesic taken, more specific timing of analgesic use, and indications for use.

Repair of early left main stem stenosis after switch operation using an autologous innominate vein patch

We describe the repair of the postoperative left main stem stenosis of a 24-day old male patient who initially underwent arterial switch operation for dextro-transposition of the great arteries. The repair was facilitated by using an autologous left innominate vein patch, while there was a shortage of suitable repair material in this neonate. Postoperative echocardiogram prior to discharge confirmed a satisfactory flow through the left main stem and improved left ventricle function.

Echocardiographic parameters of systemic right ventricular size and function compared to cardiac MRI

Background Patients with dextro-Transposition of the Great Arteries (d-TGA) who have undergone atrial switch palliation and patients with congenitally corrected Transposition of the Great Arteries (ccTGA) have systemic right ventricles which are prone to long term dysfunction and failure. Assessment of right ventricular function by echocardiography has been challenging because of anatomical location and shape. Quantitative echocardiographic measurements have been validated in sub-pulmonary right ventricles, but are understudied in systemic right ventricles. Cardiac MRI (cMRI) is the current gold standard in RV assessment but is time consuming and expensive. Methods 27 patients with either d-TGA (n = 15) or ccTGA (n = 12) who had both a cMRI and echocardiogram within 6 months of each other were evaluated. Echocardiographic parameters of RV size including measurements at the base, mid cavity, longitudinal axis, and end diastolic area were compared to volume measurements made by cMRI. Echocardiographic parameters of function including myocardial performance index (MPI), dP/dt and fractional area change (FAC) were compared to cMRI measurements of ejection fraction. Statistical analysis was done using linear regression analysis comparing echocardiographic parameters to cMRI results in all patients in the study and in the subgroups of d-TGA and ccTGA.

Earlier Arterial Switch Operation Improves Outcomes and Reduces Costs for Neonates With Transposition of the Great Arteries

This study sought to examine the impact of surgical timing on major morbidity and hospital reimbursement for late preterm and term infants with dextrotransposition of the great arteries (d-TGA). Neonatal arterial switch operation is the standard of care for d-TGA. Little is known about the effects of age at operation on clinical outcomes or costs for these neonates. We conducted a retrospective cohort study of infants at≥36 weeks' gestation, with d-TGA, with or without ventricular septal defects, admitted to our institution at 5 days of age or younger, between January 1, 2003 and October 1, 2012. Children with other cardiac abnormalities or other major comorbid conditions were excluded. Univariable and multivariable analyses were performed to determine the effects of age at operation on major morbidity and hospital reimbursement. A total of 140 infants met inclusion criteria. Reimbursement data were available for them through January 1, 2012 (n= 128). The mortality rate was 1.4% (n= 2). Twenty percent (n= 28) experienced a major morbidity. The median costs were $60,000, in 2012 dollars (range: $25,000 to $549,000). The median age at operation was 5 days (range: 1 to 12days). For every day later that surgery was performed, beyond day of life 3, the odds of major morbidity increased by 47% (range: 23% to 66%, p< 0.001) and costs increased by 8% (range: 5% to 11%, p<0.001), after considering the effects of sex, birth weight, gestational age, year at which surgery was performed, transfer, weekend admission, insurance, surgeon, septostomy, bypass and cross-clamp times, and the presence of ventricular septal defects or abnormal coronary anatomy. Delay of neonatal arterial switch operation beyond 3 days is significantly associated with increased morbidity and healthcare costs.

Postoperative pulmonary and aortic 3D haemodynamics in patients after repair of transposition of the great arteries

To characterise aortic and pulmonary haemodynamics and investigate the correlation with post-surgical anatomy in patients with dextro-transposition of the great arteries (d-TGA). Four-dimensional (4D) MRI was performed in 17 patients after switch repair of TGA and 12 healthy controls (age, 11.9 ± 5.4 vs 23.3 ± 1.6years). Patients were divided according to the pulmonary trunk (TP) position in relation to the ascending aorta (AAo): anterior (n = 10) and right/left anterior position (n = 7). Analysis included visual grading (ranking 0-2) of pulmonary and aortic vortical and helical flow, flow velocity quantification, blood-flow distribution to the right and left pulmonary arteries (flow ratio rPA:lPA), and vessel lumen areas. Anterior TP position was associated with increased vortices in six out of ten patients compared with right anterior TP position (one out of seven) and controls (none). Reduced systolic lPA and TP lumina in patients resulted in significantly increased peak systolic velocities (P < 0.001). Flow ratio rPA:lPA was more heterogeneous in patients (rPA:lPA = 1.56 ± 0.78 vs volunteers 1.09 ± 0.15; P < 0.05) with predominant flow to the rPA. Eleven patients presented increased helices in the AAo (grade 1.6). Evaluation of post-surgical haemodynamics in TGA patients revealed increased vortical flow for anterior TP position, asymmetric flow and increased systolic flow velocity in the pulmonary arteries owing to reduced vascular lumina. • 3D phase contrast MRI with velocity encoding (4D MRI) has numerous cardiovascular applications • 4D MRI demonstrates postoperative haemodynamics following surgery for transposition of the great arteries • Flow visualisation depicted enhanced pulmonary vortices in the anterior pulmonary trunk • Narrow pulmonary arterial systolic lumina resulted in increased peak systolic velocities.

Strategic and operational aspects of a transfusion-free neonatal arterial switch operation

Blood transfusion-free complex congenital cardiac surgery in a neonate remains a challenge for multidisciplinary cardiac teams. At our institution, a 3.5 kg neonate, born to a family of Jehovah's Witnesses and postnatally diagnosed with dextro-transposition of the great arteries (d-TGA) and a small muscular ventricular septal defect, underwent a successful arterial switch operation without blood or platelet transfusion. Key points that contributed to success were optimal preoperative haematopoetic conditioning using erythropoietin and iron, a miniaturized cardiopulmonary bypass circuit including a low prime volume oxygenator and crystalloid cardioplegia, and a well-coordinated multidisciplinary team. We report an overview of the literature regarding blood transfusion-free complex congenital cardiac surgery.

Modified Nikaidoh Operation in a Patient with Dextro-Transposition of the Great Arteries, Ventricular Septal Defect, and Left Ventricular Outflow Tract Obstruction

The optimal surgical management for patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) remains controversial. We describe the case of a patient with TGA, a remote restrictive VSD, and LVOTO who underwent a successful modified Nikaidoh operation.

Ablation of atrial tachycardia after Mustard and Senning surgeries for d-transposition of the great arteries

Various types of cardiac arrhythmias can be encountered in patients with previous Mustard or Senning palliation for dextro transposition of the great arteries. This review focuses on the most frequent presentations with supraventricular tachycardias which are amenable to catheter ablation using advanced technologies.In most of the cases, success can be achieved with ablation from the neo-pulmonary venous site, a region no longer directly accessible. Different techniques can be employed to reach the pulmonary side of the circulation, depending on the personal experience and the available equipment including remote magnetic navigation. Careful pre- and peri-procedural assessment of the underlying anatomy, possible baffle obstructions or residual leaks is essential.

Dextro-Transposition of the Great Arteries in a Neonate with Ventricular Septal Defect and Pulmonary Stenosis Complicated by Premature Closure of the Ductus Arteriosus and Possible Pathophysiology of Both Defects

Premature closure of the ductus arteriosus (PCDA) and transposition of the great arteries (TGA) cause persistent pulmonary hypertension of the newborn (PPHN). We present a case of a newborn who demonstrated d-TGA with ventricular septal defect (VSD) and pulmonary stenosis (PS) complicated by PCDA. The neonate showed severe cyanosis resistant to resuscitation soon after birth, and was diagnosed with d-TGA with VSD by echocardiography. PPHN was also suspected based on physical symptoms and results of echocardiography. The neonate was given inhaled nitric oxide, prostaglandin E1, and catecholamines under sedation, and underwent a balloon atrial septostomy (BAS). His condition gradually improved, and he was extubated on day 7, but his pulmonary subvalvular stenosis gradually worsened and pulmonary blood flow was markedly decreased. A second BAS was performed on day 27 and he showed no improvement. Blalock-Taussig shunt surgery was performed on day 34, which markedly improved his condition. The co-existence of d-TGA and PCDA is generally a lethal state. In our patient, an increase in pulmonary blood flow during the fetal period was restricted because of PS and outlet flow from the left ventricle to the right ventricle via the VSD. This restricted blood flow through the ductus arteriosus, which led to narrowing of the DA. At the same time, damage to and constrictive changes of the pulmonary vessels were prevented. The ductus arteriosus should be carefully evaluated to exclude PCDA in cases of d-TGA. The presence of both VSD and PS may be a prognostic factor in such cases.

Congenital Heart Disease

Congenital diseases of the heart and vasculature are the most common birth defects, occurring in approximately eight per 1,000 live births. Some patients with congenital heart defects (CHDs) have a high quality of life for many years due to progress in medical and surgical management made in recent decades. This relatively high incidence, coupled with improved management, has resulted in a large adult population of patients with congenital heart disease. This chapter reviews the CHDs most likely to be encountered in adult patients, including atrial septal defects, atrioventricular septal defects, ventricular septal defects, patent ductus arteriosus, bicuspid aortic valve, pulmonic stenosis, coarctation of the aorta, persistent left superior vena cava, tetralogy of Fallot, dextro-transposition of the great arteries, the univentricular heart, Ebstein anomaly of the tricuspid valve, and Eisenmenger syndrome. Also discussed are women’s health issues, such as contraception, risk of pregnancy to the mother with CHD and risk to their offspring. Tables list recommendations for pulmonary valve replacement in repaired tetralogy of Fallot, conditions in which pregnancy risk is contraindicated, and cardiac indications for fetal echocardiography. Figures include illustrations of atrial septal defects, the atrioventricular septum, ventricular septal defects, systemic artery–to–pulmonary artery shunts, and stages in the repair of functional single ventricles; an angiogram of a persistent left superior vena cava; and computed tomographic images of atrial switch palliation of transposition of the great arteries and multiple baffle obstructions, a magnetic resonance image of a repaired tetralogy of Fallot and long-standing pulmonary valve insufficiency, and echocardiograms of a patient with Ebstein anomaly. This review contains 9 highly rendered figures, 3 tables, and 44 references.

Left Main Coronary Artery Compression Long Term After Repair of Conotruncal Lesions: The Bow String Conduit

We report 4 cases of left main coronary artery (LMCA) compression after remote repair of conotruncal lesions and their successful surgical management.

Short- and Mid-Term Outcomes of Total Correction of Taussig–Bing Anomaly

Double-outlet right ventricle (DORV)/Taussig-Bing (TB) anomaly is the second most common type of DORV. This study evaluates our experience and outcomes of total correction of DORV-TB anomaly at King Abdulaziz Cardiac Center. We conducted a retrospective study for all cases of TB anomaly repaired between June 2001 and April 2009. Patients were divided into two groups: Group A included patients repaired with arterial switch operation, and group (B) included patients repaired with Rastelli procedure. Thirteen patients with TB anomaly underwent total correction. There were 5 male (38%) and 8 female (62%) patients. Mean age and weight at surgery were 6.8 ± 6 weeks and 3.6 ± 0.7 kg, respectively. Of the 13 patients, 9 (69%) were in group A, and 4 (31%) were in group B. Aortic arch abnormalities were present in 9 patients (69%); abnormal coronary artery patterns were present in 7 patients (54%); side-by-side great arteries were present in 5 patients (38%); dextrotransposition of the great arteries was present in 7 patients (54%); and levo-malposition of the great arteries was present in 1 patient (8%). At postoperative follow-up, 4 patients (31%) had developed either left- or right-ventricular outflow tract (VOT) obstruction requiring surgical and/or catheter intervention. There was no early mortality, but there was 1 late mortality caused by left-ventricle dysfunction. DORV-TB is often associated with other congenital cardiac anomalies. In general, total repair is feasible in the majority of patients with satisfactory results and improved outcome. Residual lesion and development of VOT obstruction can occur, requiring close follow-up and intervention for residual lesion.

Impact of Maternal Obesity and Maternal Overweight on the Detection Rate of Fetal Heart Defects and the Image Quality of Prenatal Echocardiography

The purpose of this study was to compare the prenatal detection of four congenital heart defects (CHDs) and the image quality of five corresponding ultrasound planes among obese, overweight and normal-weight women. This was a retrospective cohort study of 54,846 pregnancies undergoing fetal echocardiography between 18 and 37 weeks of gestation in the years from 2000 to 2007. The women were categorized according to pre-pregnancy body mass index (BMI) as normal-weight (BMI < 25), overweight (BMI 25 - 29.9) and obese (BMI ≥ 30). Image quality and prenatal detection of atrioventricular septal defect (AVSD), double outlet right ventricle (DORV), tetralogy of fallot (TOF) and dextro transposition of the great arteries (D-TGA) were evaluated in the BMI strata. 108 cases with one of the considered CHDs were identified. The prevalence was significantly higher (relative risk = 2.04) in overweight or obese women (57/19,404 vs. 51/35,442, p < 0.0002) than in normal-weight women. In total 86.1% of CHDs were correctly identified prenatally (93/108, CI: 79.6%-92.6%), 84.3% (43/51) in the normal weight group, 88.6% (39/44) in the overweight group and 84.6% (11/13) in the obese group. The rate of insufficient ultrasound images increased from 6.4% in normal-weight patients to 17.4% in obese women within the 108 CHD cases. The prenatal detection of fetal AVSD, DORV, TOF and D-TGA was also satisfactory in overweight and obese patients, but image quality substantially decreases with an increasing maternal BMI. If there is a BMI-associated difference in the detection rate, it probably will not exceed 20%.

Typical Atrial Flutter in an Atypical Patient

Arrhythmias in adult congenital heart disease (ACHD) pose unique procedural challenges, especially with intravascular access. We report a unique case of ablation via a left-sided hepatic vein approach in a patient with situs inversus totalis. A 28-year-old woman with situs inversus totalis, ventriculoseptal defect, and dextro-transposition of the great arteries underwent ablation for documented narrow-complex tachycardia. Because of bilateral iliac venous occlusions, the coronary sinus (CS) was accessed through the left internal jugular vein. Rapid atrial pacing resulted in a tachycardia with an atrial cycle length of 225 msec and 2:1 atrioventricular association. Entrainment from the proximal and distal CS was consistent with typical atrial flutter around the left-sided tricuspid valve. Because of the iliac vein occlusions, access for ablation was obtained via a left-sided hepatic vein (Figure 1). Resetting from the cavotricuspid isthmus and three-dimensional electroanatomic mapping (Figure 2) confirmed typical atrial flutter, which, given the dextrocardia, occurred in a clockwise fashion around the tricuspid valve. Ablation was performed at the cavotricuspid isthmus resulting in arrhythmia termination and isthmus block. This case highlights the many unusual challenges that patients with ACHD can pose to the proceduralist, including atypical cardiac anatomy and difficult intravascular access. Unusual and creative approaches are often necessary to treat these patients successfully.

Reverse Differential Cyanosis: A Treatable Newborn Cardiac Emergency

Reverse differential cyanosis (upper extremity oxygen saturation less than lower extremity oxygen saturation) is a rare, formerly fatal condition. Usually seen with dextrotransposition of the great arteries (with persistent pulmonary hypertension or aortic interruption/coarctation), it can be treated successfully with oxygen, prostaglandin E1, atrial septotomy, nitric oxide, bosentan, or extracorporeal membrane oxygenation, followed by the arterial switch operation.

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.

Epidemiology of nonsyndromic conotruncal heart defects in Texas, 1999–2004

Congenital heart defects (CHDs) are the most common structural birth defects, yet their etiology is poorly understood. As there is heterogeneity within the group of CHDs, epidemiologic studies often focus on subgroups, of conditions, such as conotruncal heart defects (CTDs). However, even within these subgroups there may be etiologic heterogeneity. The aim of the present study was to identify and compare maternal and infant characteristics associated with three CTDs: truncus arteriosus (TA), dextro-transposition of the great arteries (d-TGA), and tetralogy of Fallot (TOF). Data for cases with nonsyndromic TA (n = 78), d-TGA (n = 438), and TOF (n = 529) from the Texas Birth Defects Registry, 1999-2004, were used to estimate crude and adjusted prevalence ratios, separately for each condition, using Poisson regression. Polytomous logistic regression was used to determine whether the observed associations were similar across the two largest case groups (d-TGA and TOF). In Texas, 1999-2004, the prevalence of nonsyndromic TA, d-TGA, and TOF was 0.35, 1.98, and 2.40 per 10,000 live births, respectively. There was evidence of a significant linear increase in the risk of each condition with advancing maternal age (p < 0.01). Significant associations were observed for TA and maternal residence on the Texas-Mexico border; d-TGA and infant sex, maternal race/ethnicity, history of previous live birth, and birth year; and TOF and maternal race/ethnicity and education. Further, the associations with some, but not all, of the study variables were significantly different for d-TGA and TOF. These findings add to our limited understanding of the epidemiology of CTDs.