High-Throughput Genomics Identify Novel FBN1/2 Variants in Severe Neonatal Marfan Syndrome and Congenital Heart Defects.

Fibrillin-1 and fibrillin-2, encoded by FBN1 and FBN2, respectively, play significant roles in elastic fiber assembly, with pathogenic variants causing a diverse group of connective tissue disorders such as Marfan syndrome (MFS) and congenital contractural arachnodactyly (CCD). Different genomic variations may lead to heterogeneous phenotypic features and functional consequences. Recent high-throughput sequencing modalities have allowed detection of novel variants that may guide the care for patients and inform the genetic counseling for their families. We performed clinical phenotyping for two newborn infants with complex congenital heart defects. For genetic investigations, we employed next-generation sequencing strategies including whole-genome Single-Nucleotide Polymorphism (SNP) microarray for infant A with valvular insufficiency, aortic sinus dilatation, hydronephrosis, and dysmorphic features, and Trio whole-exome sequencing (WES) for infant B with dextro-transposition of the great arteries (D-TGA) and both parents. Infant A is a term male with neonatal marfanoid features, left-sided hydronephrosis, and complex congenital heart defects including tricuspid regurgitation, aortic sinus dilatation, patent foramen ovale, patent ductus arteriosus, mitral regurgitation, tricuspid regurgitation, aortic regurgitation, and pulmonary sinus dilatation. He developed severe persistent pulmonary hypertension and worsening acute hypercapnic hypoxemic respiratory failure, and subsequently expired on day of life (DOL) 10 after compassionate extubation. Cytogenomic whole-genome SNP microarray analysis revealed a deletion within the FBN1 gene spanning exons 7-30, which overlapped with the exon deletion hotspot region associated with neonatal Marfan syndrome. Infant B is a term male prenatally diagnosed with isolated D-TGA. He required balloon atrial septostomy on DOL 0 and subsequent atrial switch operation, atrial septal defect repair, and patent ductus arteriosus ligation on DOL 5. Trio-WES revealed compound heterozygous c.518C>T and c.8230T>G variants in the FBN2 gene. Zygosity analysis confirmed each of the variants was inherited from one of the parents who were healthy heterozygous carriers. Since his cardiac repair at birth, he has been growing and developing well without any further hospitalization. Our study highlights novel FBN1/FBN2 variants and signifies the phenotype-genotype association in two infants affected with complex congenital heart defects with and without dysmorphic features. These findings speak to the importance of next-generation high-throughput genomics for novel variant detection and the phenotypic variability associated with FBN1/FBN2 variants, particularly in the neonatal period, which may significantly impact clinical care and family counseling.

Left ventricle implantable cardioverter defibrillator: a dextro-transposition of the great arteries case.

We present a case of a patient with dextro-transposition of the great arteries palliated with a Senning procedure and a long-term arrhythmic complication that required an intervention, with an Implantable Cardioverter Defibrillator (ICD) implantation in the sub-pulmonary ventricle (morphologically left). This case highlights the need to perform off-label procedures to deal with the long-term complications of these complex patients.

Medical Therapy for Systemic Right Ventricles: A Systematic Review (Part 1) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Patients with systemic morphological right ventricles (RVs), including congenitally corrected transposition of the great arteries and dextro-transposition of the great arteries with a Mustard or Senning atrial baffle repair, have a high likelihood of developing systemic ventricular dysfunction. Unfortunately, there are a limited number of clinical studies on the efficacy of medical therapy for systemic RV dysfunction. We performed a systematic review and meta-analysis to assess the effect of angiotensin-converting enzyme (ACE) inhibitors, angiotensin-receptor blockers (ARBs), beta blockers, and aldosterone antagonists in adults with systemic RVs. The inclusion criteria included age ≥18 years, systemic RVs, and at least 3 months of treatment with ACE inhibitor, ARB, beta blocker, or aldosterone antagonist. The outcomes included RV end-diastolic and end-systolic dimensions, RV ejection fraction, functional class, and exercise capacity. EMBASE, PubMed, and Cochrane databases were searched. The selected data were pooled and analyzed with the DerSimonian-Laird random-effects meta-analysis model. Between-study heterogeneity was assessed with Cochran's Q test. A Bayesian meta-analysis model was also used in the event that heterogeneity was low. Bias assessment was performed with the Newcastle-Ottawa Scale and Cochrane Risk of Bias Tool, and statistical risk of bias was assessed with Begg and Mazumdar's test and Egger's test. Six studies met the inclusion criteria, contributing a total of 187 patients; treatment with beta blocker was the intervention that could not be analyzed because of the small number of patients and diversity of outcomes reported. After at least 3 months of treatment with ACE inhibitors, ARBs, or aldosterone antagonists, there was no statistically significant change in mean ejection fraction, ventricular dimensions, or peak ventilatory equivalent of oxygen. The methodological quality of the majority of included studies was low, mainly because of a lack of a randomized and controlled design, small sample size, and incomplete follow-up. In conclusion, pooled results across the limited available studies did not provide conclusive evidence with regard to a beneficial effect of medical therapy in adults with systemic RV dysfunction. Randomized controlled trials or comparative-effectiveness studies that are sufficiently powered to demonstrate effect are needed to elucidate the efficacy of ACE inhibitors, ARBs, beta blockers, and aldosterone antagonists in patients with systemic RVs.

Real-time three-dimensional trans-oesophageal echocardiography-guided balloon dilation of pulmonary venous pathway obstruction in a patient with dextro-transposition of the great arteries after atrial switch surgery: a case report.

A percutaneous transcatheter balloon dilation of a pulmonary venous pathway obstruction was successfully performed in a 40-year-old patient after a Mustard procedure. During the procedure, real-time three-dimensional trans-oesophageal echocardiography demonstrated the morphology of the obstruction. Our case highlights the usefulness of real-time three-dimensional trans-oesophageal echocardiography as a guide for transcatheter intervention in the increasing number of adults with CHD.

Relationship Between Cavum Septi Pellucidi Measurements and Fetal Hypoplastic Left Heart Syndrome or Dextro-Transposition of the Great Arteries.

OBJECTIVES: The aim of this study was to compare the size and position of the cavum septi pellucidi (CSP) in fetuses with hypoplastic left heart syndrome (HLHS) or dextro-transposition of the great arteries (d-TGA) with healthy fetuses. METHODS: The CSP length, CSP width, and frontal lobe length were measured in 185 healthy fetuses (404 scans), 11 fetuses with HLHS (16 scans), and 11 fetuses with d-TGA (12 scans) between January 2005 and April 2016. Each measurement was compared between healthy fetuses and those with HLHS or d-TGA, controlling for the biparietal diameter. RESULTS: Positive correlations were noted between biparietal diameter and CSP length, CSP width, and frontal lobe length (adjusted R2 = 0.811, 0.821, and 0.878, respectively). An increased CSP length was found in both fetuses with HLHS and those with d-TGA (P < .0001). The CSP width was only increased in fetuses with d-TGA (P = .0466). No difference in the frontal lobe length was noted. CONCLUSIONS: In fetuses with HLHS, the CSP is increased in length. In fetuses with d-TGA, the CSP is increased in both length and width.

No difference found in safety or efficacy of balloon atrial septostomy performed at the bedside versus the catheterisation laboratory.

BACKGROUND: Balloon atrial septostomy is performed in infants with dextro-transposition of the great arteries to improve oxygenation before surgery. It is performed in the catheterisation laboratory with fluoroscopy or at the bedside using echocardiography. It is unclear whether procedural safety and efficacy is superior in one location versus the other, although the bedside procedure may improve resource utilisation and present an opportunity for reducing cost. This study compares safety and efficacy of atrial septostomy performed at the patient's bedside versus the catheterisation laboratory. METHODS: Neonates with dextro-transposition of the great arteries who underwent balloon atrial septostomy from October, 2000 to January, 2014 were included. Medical and procedural records, echocardiograms, and catheterisation data were reviewed. Comparisons between the two procedural locations included patient demographics, pre- and post-procedure oxygen saturations, and outcomes. Complications reviewed included bleeding, arrhythmia, cardiac trauma, stroke, and death. Coronary artery evaluations were recorded. T-tests were used for continuous variables, and Fisher's exact tests were used for all categorical variables. Wilcoxon rank sum and analysis of covariance modelling were used for time variables and oxygen saturation, respectively. RESULTS: A total of 88 infants met the inclusion criteria. Among them, 53 underwent septostomy at the bedside and 35 underwent septostomy in the catheterisation laboratory. No safety or outcome benefit was identified between the two procedural locations. CONCLUSION: Septostomy performed at the bedside and in the catheterisation laboratory had similar outcomes and efficacy. Further, bedside septostomy has the advantage of no radiation exposure, and obviating risks with patient transfer from the ICU to the catheterisation laboratory.

Right ventricular function and N-terminal pro-brain natriuretic peptide levels in adult patients with simple dextro-transposition of the great arteries.

INTRODUCTION: Dextro-transposition of the great arteries (d-TGA) patients is at high risk of developing right ventricular dysfunction and tricuspid regurgitation in adulthood. Determining the relation between echocardiographic parameters, N-terminal pro-brain natriuretic peptide (NT-pro-BNP) levels and the New York Heart Association (NYHA) functional class may help determining the best time to operate them. METHODS: Patients with simple d-TGA operated in infancy with an atrial switch procedure (Mustard or Senning operation) were followed up in our Adult Congenital Heart Disease Unit. Analytical, echocardiographic, and clinical parameters were determined to evaluate the correlation between right echocardiographic ventricular function, NT-pro-BNP levels, and NYHA functional class. RESULTS: Twenty-four patients with d-TGA were operated in infancy of whom 17 alive patients had simple d-TGA. Nine patients had NT-pro-BNP levels lower than 200 pg/mL and eight patients were above 200 pg/mL. Patients with lower hemoglobin concentration, higher right ventricular diameter or under diuretic treatment showed significant higher NT-pro-BNP levels (above 200 pg/dL). The Spearman test showed a positive correlation between basal right ventricular diameter and tricuspid regurgitation with pro NT BNP levels (correlation coefficient of .624; P=.017 and .490; P=.046, respectively) and a negative correlation with the right ventricle fractional area change (-.508, P=.045). No correlation was seen between NT-pro-BNP levels and the rest of echocardiographic parameters or the NYHA functional class. CONCLUSION: NT-pro-BNP levels showed a positive correlation with basal right ventricular diameter and tricuspid regurgitation but not with NYHA association functional class in d-TGA patients.

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.

Lead Extraction and Baffle Stenting in a Patient with Transposition of the Great Arteries.

This case report discusses a 42-year-old male with dextro-transposition of the great arteries (D-TGA) status post Mustard repair and sick sinus syndrome status post dual-chamber pacemaker implant, who developed symptomatic superior vena cava (SVC) baffle stenosis. He was treated with a combined pacemaker extraction and subsequent SVC baffle stenting. The case highlights the complexities of treating SVC baffle stenosis in the presence of cardiac implantable devices and demonstrates the efficacy of this combined approach. Furthermore, the authors delve into the intricacies of D-TGA, its surgical history, and the long-term complications associated with atrial switch procedures.

Prenatal echocardiography diagnosis of a novel combination of bilateral ductus arteriosus and cardiovascular anomalies: a case report and literature review.

Background: Bilateral ductus arteriosus (BDA) is a relatively rare vascular malformation. According to the double arch theory, BDA is formed when the distal ends of the sixth pairs of primitive arches on the left and right sides have not regressed. We describe a fetus with prenatal echocardiographic findings of BDA and right aortic arch mirror-image branching (RAA-MIB) combined with congenital heart disease. Furthermore, to gain a deeper understanding of the embryological mechanism of BDA, we review the literature on all combinations of BDA present in 40 fetuses/infants. Case summary: A 22-year-old female patient underwent fetal echocardiography at 23 weeks of gestation. Both the two-dimensional (2D) grayscale image and color Doppler flow imaging (CDFI) revealed dextro-transposition of the great arteries combined with a ventricular septal defect and RAA-MIB. The following scan revealed a rare vascular ring, which was identified as BDA extending from the confluent of the left pulmonary artery and right pulmonary artery, completely encircling the trachea to form an "O"-shaped vascular ring before finally converging into the descending aorta. A persistent left superior vena cava was also observed. We subsequently used four-dimensional (4D) color Doppler imaging with the spatiotemporal image correlation (STIC) HD live flow and STIC HD live flow silhouette mode to clearly display ventricular arterial connectivity and the direction of vessel travel. Adjusting the image quality and display angle is very important when applying STIC. The 4D images confirmed our diagnosis. After multidisciplinary counseling and discussion with her family, this female patient decided to terminate the pregnancy. Conclusion: Our review of the literature summarized nine combinations classified into three types of BDA and aortic arch pathology. However, our case differs because it is a novel combination of intracardiac structural abnormalities and vascular rings in a fetus. Prenatal ultrasound diagnosis of BDA is important and requires a combination of 2D grayscale, CDFI, and STIC images to assist in scanning.

Post-operative troponin levels and left ventricular function in patients with d-transposition of the great arteries following the arterial switch operation.

The aim of this study was to assess the significance of post-operative troponin levels as a surrogate for left ventricular (LV) dysfunction measured by global longitudinal strain (GLS) in patients with dextro-transposition of the great arteries (d-TGA) who undergo an arterial switch operation (ASO), and to explore the LV GLS recovery in the mid-term follow-up period. Seventy-eight neonates were included, of whom 41 had troponin-I measurements and 37 had troponin-T measurements. The primary outcome of LV GLS was assessed and compared with healthy controls at the pre-operative stage and time of discharge, 3 months, 6 months and 12 months of age. Secondary outcomes included deaths or transplantations and other clinical markers such as length of hospital stay. D-TGA patients had worse LV GLS post-operatively compared to age-matched controls (p < 0.01) which improved by 12 months of age (p = 0.53). No association was found between changes in troponin-I or troponin-T levels and LV GLS at the time of discharge (r = 0.4, p = 0.64 and r = -0.5, p = 0.91, respectively). In addition, there were no deaths or transplantations in this cohort over a period of 12 months. LV GLS appears to worsen in the early post-operative period for d-TGA patients who undergo neonatal ASO but this recovers through the first post-operative year. Troponin levels have limited value in predicting early or midterm LV dysfunction and recovery.

Balloon Atrial Septostomy in a Premature Infant.

Balloon atrial septostomy (BAS) in premature and very low weight infants has not been described. We present a successful BAS in a 1.4-kg, 13-day-old ex-31 6/7-week infant with dextro-transposition of the great arteries and a restrictive atrial septum. (Level of Difficulty: Advanced.).

Symptomatic Myocardial Bridging in D-Transposition of the Great Arteries Post-Arterial Switch.

We present Stanford's experience with patients post-arterial switch operation presenting with chest pain found to have hemodynamically significant myocardial bridging. The evaluation of symptomatic patients post-arterial switch should not only include assessment for coronary ostial patency but also for nonobstructive coronary conditions such as myocardial bridging. (Level of Difficulty: Advanced.).

The mitral annulus in transposition of the great arteries late after Senning- and Mustard-procedures (Insights from the CSONGRAD Registry and MAGYAR-Path Study).

Background: Dextro-transposition of the great arteries (dTGA) is a rare condition comprising 5-7% of all congenital heart diseases (CHD). Until 1990s, atrial switch operations were the method of choice for the treatment of dTGA. The aim of our present study was to evaluate the abnormalities of the mitral annulus (MA) by three-dimensional speckle-tracking echocardiography (3DSTE). Potential differences in MA dimensions and functional properties late after Senning- and Mustard-procedures were analyzed as well. Methods: This retrospective cohort study comprised 19 dTGA patients late after atrial switch operation, from which 7 subjects were not involved due to inferior image quality. The remaining dTGA patient group comprised 12 subjects (age: 30.7±8.6 years, 7 males). For comparisons, 32 age- and gender-matched healthy controls were evaluated (age: 34.4±12.3 years, 18 males). Results: End-systolic and end-diastolic MA diameter (MAD), area and perimeter proved to be increased, while MA functional parameters [MA fractional area change (MAFAC) and fractional shortening (MAFS)] were reduced in all dTGA patients as compared to matched controls regardless of the atrial switch procedure used. However, MA morphological parameters were tendentiously lower, while MA functional parameters were tendentiously favorable in case of Senning-procedure compared to Mustard-procedure. Conclusions: dTGA is accompanied by MA dilation and its functional impairment late after Senning- and Mustard-procedures.

Dextro-transposition of the great arteries in one twin: case reports and literature review.

Background: Dextro-transposition of the great arteries (D-TGA) is a rare congenital heart disease, as it affects only 0.02-0.05% of live births. It is the second most common cyanotic heart disease following Tetralogy of Fallot. It has a male predominance. Fetal echocardiography is an optimal method for prenatal diagnosis of fetal D-TGA. In twin pregnancies, fetal D-TGA in one twin is very rare, especially in monochorionic-diamniotic twin pregnancies. Herein, we report a case of D-TGA in one twin in two dichorionic-diamniotic twin pregnancies and one monochorionic-diamniotic twin pregnancy from January 2018 to June 2021. Case Description: One twin with D-TGA was diagnosed by fetal echocardiography in the second trimester, and the co-twin was normal in all three cases. A multidisciplinary team provided extensive counseling regarding the D-TGA twin and the co-twin, and adequate perinatal management was provided. In cases 1, 2, and 3, the mothers underwent cesarean sections at 37 weeks + 2 days, 34 weeks + 5 days, and 36 weeks + 1 day, respectively. In case 1, which involved a female D-TGA neonate with birth weight 2,410 g, an emergent atrial septostomy was performed at 20 h after birth, and the neonate underwent atrial switch operation (ASO) 24 days after birth. In case 2, involving a male D-TGA neonate with a birth weight of 2,380 g, ASO was performed 24 days after birth. In case 3, involving a female D-TGA neonate with birth weight 2,240 g, ASO was performed 19 days after birth and delayed sternal closure was performed 4 days later. All six infants showed normal development during follow-up. Conclusions: Early antenatal diagnosis of D-TGA in one fetus of a twin pregnancy is significantly important. A multidisciplinary team should carry individual evaluation and integrated management of the D-TGA twin and co-twin during the pregnancy and perinatal period. After birth, delayed ductus arteriosus closure in the D-TGA twins should be performed when necessary and individualized timings for arterial switch operation should be considered.

Heart failure in systemic right ventricle: Mechanisms and therapeutic options.

d-loop transposition of the great arteries (d-TGA) and congenitally corrected transposition of the great arteries (cc-TGA) feature a right ventricle attempting to sustain the systemic circulation. A systemic right ventricle (sRV) cannot support cardiac output in the long run, eventually decompensating and causing heart failure. The burden of d-TGA patients with previous atrial switch repair and cc-TGA patients with heart failure will only increase in the coming years due to the aging adult congenital heart disease population and improvements in the management of advanced heart failure. Clinical data still lags behind in developing evidence-based guidelines for risk stratification and management of sRV patients, and clinical trials for heart failure in these patients are underrepresented. Recent studies have provided foundational data for the commencement of robust clinical trials in d-TGA and cc-TGA patients. Further insights into the multifactorial nature of sRV failure can only be provided by the results of such studies. This review discusses the mechanisms of heart failure in sRV patients with biventricular circulation and how these mediators may be targeted clinically to alleviate sRV failure.

Coronary artery augmentation with the right subclavian artery for single coronary artery variants of dextro transposition of the great arteries treated by an arterial switch operation.

Objective: Single coronary artery variants in dextro transposition of the great arteries increase the technical demands of performing coronary translocations during the arterial switch operation (ASO). Coronary augmentation using the right subclavian artery as an interposition graft is a viable option in certain cases. The aim of this study is to describe this operative technique and review outcomes. Methods: Of 59 patients who underwent an ASO, from July 2015 to May 2021, 6 had single coronary variants in which the right subclavian artery was used as an interposition graft. Mean follow-up was 21.5 months. Results: Patients mean age and weight at the time of surgery were 7.1 ± 3.8 days and 2.84 ± 0.76 kg, respectively. Four patients had the left anterior descending coronary artery and right coronary artery coming from the left sinus and the circumflex coronary artery coming from the right sinus. One patient had a single ostium arising from the left sinus and another patient had a single ostium coming from the right sinus. All patients are alive and free of cardiac symptoms at follow-up. Conclusions: Single coronary artery variants in dextro transposition of the great arteries can pose technical challenges for coronary translocation during ASO. The augmentation of coronary buttons using a segment of the right subclavian artery is an option which should be considered in selective cases.

Modified pulmonary artery banding: A novel strategy for balancing pulmonary blood flow with transposed great arteries.

OBJECTIVE: To study the outcomes of a novel modified pulmonary artery banding (mPAB) technique used for staged repair of a subset of patients with complex transposition physiology. METHODS: A total of 13 patients who underwent mPAB during their staged repair (biventricular repair [BVR], n = 6) or palliation (1-1/2 repair, n = 1; univentricular repair [UVR], n = 6) from 2004 to 2020 were studied retrospectively. A restrictive interposition graft was used to reconstruct the main PA between the pulmonary root and the distal pulmonary confluence, functioning as a mPAB. Twelve of the 13 patients (92.3%) underwent a concurrent arterial switch operation (ASO), of which 6 were palliative ASOs for 1-1/2 repair (n = 1) or UVR (n = 5). Patient weight and cardiac anatomy determined the size of interposition graft. RESULTS: The disease spectrum included dextro transposition of the great arteries (d-TGA) with multiple ventricular septal defects (VSDs) (n = 4), Taussig-Bing anomaly (n = 3), d-TGA with VSD and hypoplastic right ventricle (RV) (n = 3), double-inlet left ventricle with l-TGA (n = 2), and congenitally corrected TGA with double-outlet RV (n = 1). The Lecompte procedure was performed in 10 patients. Predischarge echocardiography revealed a band gradient of 61 mm Hg (interquartile range [IQR], 40-90 mm Hg) for BVR/1-1/2 ventricular repair (n = 7) and 49 mm Hg (IQR, 37-61 mm Hg) for UVR (n = 6). Survival was 100% at a median follow-up of 3.7 years (IQR, 2.6-4.0 years). CONCLUSIONS: The mPAB technique is effective and reproducible for staged BVR or UVR for patients with TGA. It effectively regulates pulmonary blood flow, may reduce neopulmonary root distortion, and eliminates complications associated with band migration in standard PAB.

Obstetric outcomes following atrial and arterial switch procedures for transposition of the great arteries (TGA) - A single, tertiary referral centre experience over 20 years.

Repair of transposition of the great arteries usually involves an atrial switch or arterial switch operation, which can complicate physiological adaptation to the demands of pregnancy and adversely affect the fetus. We retrospectively compared outcomes of 48 completed pregnancies in 23 women with surgically corrected transposition of the great arteries (38 atrial switch/10 arterial switch operation) under joint cardiac-obstetric care in our tertiary referral clinic between 1997 and 2017. Most women delivered vaginally (85%). The pre-term delivery rate was high (atrial switch 39%; arterial switch operation 40%). Small for gestational age occurred in 56% of babies, significantly more in the atrial switch group (66%) than arterial switch operation (20%), p = 0.013. Women with surgically corrected transposition of the great arteries wishing to become pregnant are at high risk of obstetric complications, primarily pre-term delivery and small for gestational age baby. They require more careful ultrasound surveillance beyond 36 weeks' gestation and/or may benefit from early induction of labour. Trial registration: Text/Not applicable.

Management of the Adult with Arterial Switch.

Dextro-transposition of the great arteries (d-TGA) is a lethal congenital heart defect in which the great arteries-the pulmonary artery and aorta-are transposed to create ventriculoarterial discordance. Corrective surgical interventions have resulted in significant improvements in morbidity and mortality for this once-fatal congenital heart defect. The initial palliative surgery for d-TGA was the atrial switch operation, which provided physiological correction. The Mustard and Senning "atrial switch" procedures, in which an atrial baffle is created to produce a discordant atrioventricular connection on the existing discordant ventriculoarterial connection, showed preliminary success for the correction of d-TGA. However, follow-up evaluations demonstrated increasing complications from the right ventricle utilized as a systemic ventricle, resulting in progressive right ventricular dysfunction. Thus, the search continued for an anatomical correction of d-TGA to return the great arteries to their normal ventricular connections. The arterial switch operation (ASO), though attempted and theorized by many, was first successfully performed by Dr. Jatene and colleagues in 1975. For ASO, the distal main pulmonary artery and the distal ascending aorta are transected and then anastomosed to their respective ventricles with relocation of the coronary arteries to the neoaorta. The ASO has replaced the atrial switch operation since the 1980s and is now the standard surgical correction for d-TGA. As more patients who have undergone ASO are living into adulthood, late complications of this procedure have become more evident. The most common late postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency, and neopulmonic stenosis. Adults who have undergone ASO in childhood will need follow-up with surveillance imaging and evaluation of new symptoms or declining function to prevent and manage late postoperative complications. This review describes the management strategies for common late complications in patients who have undergone ASO.