Transposition Of Great Arteries Research Articles

The Association of Prenatal Diagnoses with Mortality and Long-Term Morbidity in Children with Specific Isolated Congenital Anomalies: A European Register-Based Cohort Study

ObjectivesTo compare 5-year survival rate and morbidity in children with spina bifida, transposition of great arteries (TGA), congenital diaphragmatic hernia (CDH) or gastroschisis diagnosed prenatally with those diagnosed postnatally.MethodsPopulation-based registers’ data were linked to hospital and mortality databases.ResultsChildren whose anomaly was diagnosed prenatally (n = 1088) had a lower mean gestational age than those diagnosed postnatally (n = 1698) ranging from 8 days for CDH to 4 days for TGA. Children with CDH had the highest infant mortality rate with a significant difference (p < 0.001) between those prenatally (359/1,000 births) and postnatally (116/1,000) diagnosed. For all four anomalies, the median length of hospital stay was significantly greater in children with a prenatal diagnosis than those postnatally diagnosed. Children with prenatally diagnosed spina bifida (79% vs 60%; p = 0.002) were more likely to have surgery in the first week of life, with an indication that this also occurred in children with CDH (79% vs 69%; p = 0.06).ConclusionsOur findings do not show improved outcomes for prenatally diagnosed infants. For conditions where prenatal diagnoses were associated with greater mortality and morbidity, the findings might be attributed to increased detection of more severe anomalies. The increased mortality and morbidity in those diagnosed prenatally may be related to the lower mean gestational age (GA) at birth, leading to insufficient surfactant for respiratory effort. This is especially important for these four groups of children as they have to undergo anaesthesia and surgery shortly after birth. Appropriate prenatal counselling about the time and mode of delivery is needed.

Navigating the spectrum of double-outlet right ventricle presentations: Outcomes from a contemporary cohort based on subtypes.

Our aim in this study was to investigate the prenatal and postnatal prognosis of double outlet right ventricle (DORV) cases diagnosed prenatally by analyzing the outcomes based on the subtype. This study is a retrospective chart review. Cases diagnosed with fetal DORV by prenatal ultrasound in the maternal-fetal medicine department of our hospital between 2014 and 2022 were included. Data on maternal characteristics, fetal echocardiographic features (type of DORV), pregnancy and neonatal outcomes (termination of pregnancy [TOP], intrauterine fetal death [IUD], neonatal death [NND], death in infancy (IND), survival) were collected and analyzed. Ninety-nine cases of prenatally diagnosed cases of DORV were included. The prenatal diagnosis was right in 97% of the liveborn fetuses. The cases were classified into subtypes, including transposition of great arteries (TGA), Fallot, ventricular septal defect (VSD), remote, and heterotaxy types. The cohort consisted of 32.3% TGA type, 19.1% fallot type, 11.1% VSD type, 2% remotetype, and 35.3% heterotaxy type of DORV. An additional cardiac anomaly was observed in 87% and an extra-cardiac anomaly was observed in 54% of the cases. When we excluded the cases with heterotaxy type but without any chromosomal abnormality, additional genetic abnormalities were detected in 42% of the remaining cases. Outcome of pregnancy was livebirth in 68/99 (68.7%), IUFD in 5/99 (5.1%), and TOP in 26/99 (26.3%). Postnatal cardiac surgical repair was performed in 48 cases. Survival among livebirths was 39/68 (57.3%). Twenty-nine neonates or infants who had additional cardiac anomalies and/or genetic abnormalities died before any surgical intervention. The postoperative survival rate was 39/48 (81.2%). The prognosis in DORV depends on the anatomical subtype, the presence, and severity of associated anomalies. Survival increases in isolated cases without any additional structural or genetic anomalies.

A Retrospective Observational Study in Neonates with Transposition of Great Arteries Undergoing Balloon Atrial Septostomy in a Tertiary Care Hospital

Objectives: Transposition of great arteries (TGA) is a common cause of cyanotic newborns. There is an atrioventricular concordance with ventriculoarterial discordance. This parallel circulation is incompatible with life unless communication exists for the inter-circulatory mixing of blood. Balloon atrial septostomy (BAS) is a percutaneously performed interventional procedure in catheterization laboratory, usually in patients with TGA-intact ventricular septum (IVS) to ensure admixture of oxygenated and deoxygenated blood thus improving systemic oxygen delivery, to achieve hemodynamic stability before the definitive procedure (Arterial switch operation), and to determine the risk of intraprocedural complications in neonates undergoing balloon atrial septostomy. Material and Methods: This is a retrospective observational study, which included neonatal patients during a single year. All the data were collected from the medical record section of the hospital. Results: We included 17 neonates with TGA transferred to our center for definite treatment. Six cases were done under sedation and 11 under general anesthesia. The mean age at the time of BAS was 4.8 days. Procedure-related complications occurred in 41% of patients. In one of the cases, difficult airway management made the periprocedural course daunting. Complications included intraprocedural balloon rupture (1 case), transient atrial arrhythmia (4 cases), hypotension (1 case), and pericardial tamponade due to left atrial wall puncture (1 case). Conclusion: BAS is a safe and effective palliative procedure for TGA-IVS, with good immediate results in our institution. Maintaining cardiorespiratory stability, prevention of respiratory depression in a spontaneously breathing neonate, and maintenance of normothermia in the cold temperature of the catheterization laboratory, with eternal vigilance, forms the cornerstone of a successful neonatal outcome.

Perioperative Factors and Radiographic Severity Scores for Predicting the Duration of Mechanical Ventilation After Arterial Switch Surgery

ObjectivesCardiac surgery on cardiopulmonary bypass (CPB) during the neonatal period can cause perioperative organ injuries. The primary aim of this study was to determine the incidence and risk factors associated with postoperative mechanical ventilation duration and acute lung injury following arterial switch operation (ASO). The secondary aim was to examine the utility of the Brixia score for characterizing postoperative acute lung injury (ALI).Design: A retrospective study. SettingA single-center university hospital. ParticipantsA total of 93 neonates with transposition of great arteries with intact ventricular septum (dTGA/IVS) underwent ASO. InterventionsNone Measurements and Main ResultsFrom January 2015 to December 2022, 93 neonates with dTGA/IVS were included in the study. The cohort had a median age of 4.0 (3.0-5.0) days and a mean weight of 3.3 ± 0.5 kg. About 63% of patients had ≥ 48 hours postoperative mechanical ventilation after ASO. Risk factors included prematurity, post-CPB transfusion of salvaged red cells, platelets and cryoprecipitate, and postoperative fluid balance by univariate analysis. The larger transfused platelet volume was associated with the risk of ALI by multivariate analysis. The median baseline Brixia scores were 11.0 (9.0-12.0) and increased significantly in the postoperative day 1 in patients who developed moderate ALI 24 hours after admission to the intensive care unit (15.0 (13.0-16.0) vs. 12.0 (10.0-14.0), p = 0.046). ConclusionsArterial switch operation results in a high incidence of ≥ 48-hour postoperative mechanical ventilation. Blood component transfusion is a potentially modifiable risk factor. The Brixia scores may also be used to characterize postoperative acute lung injury.

Neuroimaging and Neurodevelopmental Outcomes Among Individuals With Complex Congenital Heart Disease: JACC State-of-the-Art Review.

Although neuroimaging advances have deepened our understanding of brain health in individuals with congenital heart disease (CHD), it is less clear how neuroimaging findings relate to neurodevelopmental and mental health outcomes across the lifespan. We systematically synthesized and critically evaluated evidence on associations between neuroimaging and neurodevelopmental, neurocognitive, psychiatric, or behavioral outcomes among individuals with transposition of great arteries or single-ventricle CHD (Protocol CRD42021229617). Six databases were searched and 45 papers from 25 unique studies were identified. Structural brain injury was generally linked to poorer neurodevelopment in infancy. Brain volumes and microstructural and functional brain changes appear linked to neurocognitive outcomes, including deficits in attention, learning, memory, and executive function in children and adolescents. Fetal neuroimaging studies were limited. Four papers investigated psychiatric outcomes; none found associations with neuroimaging. Multicenter, longitudinal studies incorporating functional neuroimaging and mental health outcomes are much-needed to inform early neuroprotective and therapeutic strategies in CHD.

Anaesthetic Management during Balloon Atrial Septostomy in Transposition of Great Arteries

&lt;strong&gt;Background: &lt;/strong&gt;Transposition of the great arteries (TGA) is a congenital heart defect that can cause death in 30% of the first week of birth, 50% in the first month, 70% in 6 months, and 90% in the first year, thus requiring immediate corrective action in neonates with rapid early detection. The purpose of this case study is to highlight management considerations in TGA cases in the Balloon Atrial septostomy (BAS) procedure.&lt;p&gt;&lt;strong&gt;Discussion:&lt;/strong&gt; A 1-day-old newborn girl was brought to Dr. Moewardi General Hospital in February 2022. The patient had a history of cyanosis when she cried and was born with an APGAR score of 6.7.8. The lips were cyanotic on physical examination, with a SpO2 of 77% with a nasal O2 of 2 lpm. The echocardiography results obtained TGA, ASD II, PFO, and PDA. The patient then underwent a BAS procedure. Anesthesia management was performed using ketamine for induction, air bar, O2, and sevoflurane for maintenance of anesthesia. The operation was successful, and postoperative care was carried out. Anesthesia management aims to keep SVR and PVR to a minimum, with a PVR lower than SVR, to prevent desaturation in the patient. A decrease in PVR also can increase pulmonary blood flow, allowing more blood to be mixed and higher oxygen saturation in the blood for the patient.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion:&lt;/strong&gt; The principle of anesthesia management in TGA cases is to avoid a reduction in cardiac output and SVR and keep the PVR lower than the SVR.&lt;/p&gt;

Potential benefits of prenatal diagnosis of TGA in Australia may be outweighed by the adverse effects of earlier delivery: likely causation and potential solutions

ObjectivePrenatal diagnosis of transposition of great arteries (TGA) is expected to improve postoperative outcomes after neonatal arterial switch operation (ASO); however, published reports give conflicting results. We aimed to determine...

Fetal congenital heart diseases: Diagnosis by anatomical scans, echocardiography and genetic tests

Objective: To determine the distribution of major fetal congenital heart diseases (CHDs) diagnosed antenatally during routine second-trimester obstetric anatomical scans in an unselected population at a single tertiary centre and to characterise and stratify risk factors, genetic diagnosis and long-term health at 4 years old. Method: A single-centre cohort study of all major fetal CHDs detected on routine obstetric fetal anatomical ultrasound scans between January 2014 and December 2017 was performed in an unselected population. Demographic details, fetal echocardiogram reports, genetic test results, delivery outcomes and postnatal progress were stratified by CHD subtype. Results: Of 20,031 screened pregnancies, 109 pregnancies (0.53%) had major fetal CHDs. The most common subtypes were coarctation of aorta (17.4%), transposition of great arteries (16.5%), and tetralogy of Fallot and univentricular hearts (13.8% each). Of the 60.5% that underwent confirmatory genetic testing—mostly conventional karyotyping and testing for 22q11 microdeletion—about a quarter had abnormalities, of which 22q microdeletion was the most common. We had complete obstetric data in 85 pregnancies (78%), of which 76.5% progressed to live birth. Among these, 92.1% of postnatal echocardiograms concurred with antenatal ones. At 4 years old, 43.2% of offspring had no medical or developmental issues, 20.0% had mild medical or developmental issues, 21.5% had major medical or developmental issues, and 12.3% had deceased. Conclusion: Fetal echocardiograms accurately diagnose CHDs. Future studies should evaluate the roles of chromosomal microarray and next-generation sequencing in diagnosing CHD.

Congenital heart diseases: Pattern of clinical presentations in children less than 2-years of age in a pediatric practice in southsouth Nigeria

The increase in the prevalence of congenital heart disease remains a major contributing factor tochildhood morbidity and mortality in Africa. The study describes the clinical features of children withCHD that presented to a general pediatric hospital, to improve on early recognition and management ofthe diseases. The prevalence of CHD from the study was 2 per 100 (30 per 1577 or 1 in 52) i.e. (2/100 or20/1000). A higher incidence in females (53.3%) with M: F = 1:1.14, the majority presented withdifficulty / fast breathing (70%), cough(40%), other presenting symptoms are poor weight gain (26.7%),delayed milestones(13.3%). On examination, 60% had dyspnea, 56.7% had murmur. Packed cell volumefor acyanotic heart disease ranged 28-30%, and 50-61% for cyanotic heart disease. The majority hadcomorbid bronchopneumonia (46.6%), and heart failure (23.3%). Echocardiographic findings revealedVSD in 26.7%, 20.0% had PDA and tetralogy of Fallot in 6.7%, Transposition of Great Arteries in 3.3%,and Dextrocardia in 3.3%. The outcome showed that 30% had no symptoms on follow-up, 23.3% werereferred for surgery, 10% dropped from follow-up and 3.3% died. These findings which are comparable tothe findings of other researchers showed that congenital heart disease has a place in childhood morbidityand mortality, therefore appropriate attention should be directed to improve on early recognition andmanagement of the diseases.

Prognostic significance of size and function of the subpulmonary left ventricle in patients with a systemic right ventricle

Abstract Funding Acknowledgements Type of funding sources: None. Background Systolic dysfunction of the systemic right ventricle (SRV) is a well-recognized predictor of adverse events in adults with transposition of great arteries (TGA) after atrial switch repair or with congenitally corrected TGA (ccTGA). The prognostic role of subpulmonary left ventricle (LV), however, remains unclear. Purpose To assess the prognostic significance of echocardiographic parameters of subpulmonary LV size and systolic function. Methods All adult patients with the SRV who underwent transthoracic echocardiography in 2010–2018 at a large tertiary referral centre were identified from a digitally recorded echocardiographic database. Biventricular size and function were assessed at the most recent exam. The study endpoint was all-cause mortality and heart or heart-lung transplantation. Results We included 180 patients, 100(55.6%) male, mean age 42.4±12.3 years, of whom 103(57.2%) had undergone Mustard/Senning operations and 77(42.8%) had ccTGA. One half of patients were in NYHA class ≥2 and 113 (63.5%) were receiving heart failure medication(s). During a follow-up period of 4.9[3.8–5.7] years, 28(15.6%) patients died and 4(2.2%) underwent heart or heart-lung transplantation. Univariable predictors of death or transplantation included older age, NYHA class III/IV, history of atrial arrhythmias, presence of pacemaker or cardioverter-defibrillator, high BNP, and echocardiographic markers of both SRV and subpulmonary LV size and function (Figure 1). On multivariable Cox analysis, indexed LV end-systolic diameter (ESDi; HR 2.77 [95%CI 1.35–5.68], p = 0.01), LV fractional area change (FAC; HR 0.7 [95%CI 0.57–0.85], p = 0.002), SRV basal diameter (HR 1.66 [95%CI 1.21–2.29], p = 0.005), and SRV FAC (HR 0.65 [95%CI 0.49–0.87], p = 0.008) remained predictive of mortality or transplantation. On ROC analysis, subpulmonary LV parameters performed better than SRV markers in predicting death or transplantation (Figure 2). Conclusions A raised LV ESDi and reduced LV FAC were strongly associated with mortality and heart or heart-lung transplantation independent of SRV size and function. Accurate echocardiographic assessment of the subpulmonary LV is therefore essential for risk stratification and management of patients with the SRV and should be a part of routine echocardiographic protocols.

Accuracy of pulmonary venous flow quantification by 2D phase contrast cardiac magnetic resonance

Abstract Funding Acknowledgements Type of funding sources: None. Background Determination of pulmonary venous flow by cardiac magnetic resonance (CMR) is frequently required in patients with complex congenital heart disease (CHD) lesions. Although new advanced imaging techniques like 4D flow are increasingly available, standard 2D phase contrast (2DPC) sequences remain the most frequently used method for quantification of flow. However, little is known on the accuracy of 2DPC flow measurements in the pulmonary veins. Purpose This study therefore aims to investigate the accuracy of pulmonary venous flow quantification by 2DPC. Methods Patients with CHD underwent a CMR on a 1.5T scanner. 2DPC flow sequences (typical temporal resolution 35–50ms, inplane spatial resolution 1.3–1.8 mm2, VENC 80–100) were acquired in the ascending aorta (AA), main, right and left pulmonary arteries (MPA, RPA, LPA), as well as left (LPV) and right (RPV) pulmonary veins. Pulmonary venous flow was compared to pulmonary arterial flow in all patients and to aortic flow in patients without left to right (L-R) shunt using Bland-Altman plots and Pearson’s correlations. Results Twenty CHD patients (age 27±3, range 7; 53 years, 7 women, tetralogy of Fallot N=7, Ross operation N=1; Fontan N=4, transposition of great arteries N=2, atrial septal defect N=2, Turner syndrome 1, Ebstein N=1, valvulopathies N=2) were included. Table 1 shows absolute flow values of the different vessels. Bland-Altman analyses and correlations (Table 2) showed a good agreement of total pulmonary venous (PV) to flow in the ascending aorta (group without shunt) and the sum of flow of both branch pulmonary arteries. However, agreement of total PV with MPA flow as well as of LPV and RPV with the respective branch pulmonary artery flow was reduced. Conclusions Total PV flow determined by 2DPC shows a good agreement with flow in the ascending aorta and the sum of the branch pulmonary arteries. Flow turbulences due to the pathologies of the right ventricular outflow tract probably contribute to the reduced agreement of total PV and MPA flow. However, the reduced agreement of the flow in left/right pulmonary veins and their respective branch pulmonary arteries underlines the need for newer advanced flow quantification techniques to improve accuracy of pulmonary venous flow measurements.

Situs inversus with dextrocardia and transposition of great arteries.

Transposition of the great arteries with left ventricle outflow tract obstruction in combination with situs inversus totalis and dextrocardia is a very rare anomaly and carries high morbidity and mortality. Only few cases have been reported with this anomaly. We describe a 21-day of infant girl with transposition of the great arteries and mirror image dextrocardia and pulmonary stenosis who underwent successful neonatal arterial switch operation and left ventricle outflow tract obstruction resection following PDA stent implantation.

Report of the successful Senning procedure from Nepal

A child born with transposition of great arteries should undergo arterial switch, preferably within 2 to 3 weeks of life. Sometimes, this can be extended even up to 2 months of life, if left ventricular mass is adequate. When child presents very late left ventricle may have regressed, so the options left is either left ventricular training and arterial switch or atrial switch. We present here, a 3 years old child presenting with transposition of great arteries, who underwent successful Senning procedure.

A Rare Combination of Transposition of Great Arteries and Total Anomalous Pulmonary Venous Connection

The association of total anomalous pulmonary venous connection (TAPVC) with transposition of great arteries (TGA) is a very rare combination of 2 congenital cyanotic heart diseases. TAPVC, in the case of TGA with the intact interventricular septum (IVS), is a cause of early regression of the left ventricle (LV). Very few cases of this rare combination (TAPVC and TGA with or without VSD) have been reported in the world of literature. We found 10 case reports in the literature. The minimum age was 6 days and the maximum age of 16 years. 5 of them, which were operated before 1990, underwent atrial switch with TAPVC repair. Other 4 of them, operated after 1995, underwent arterial switch with TAPVC repair. In this case report we are reporting 2 such rare cases. In both cases, large VSD prevented LV regression.

Comparison of the Results of Prenatal and Postnatal Echocardiography and Postnatal Cardiac MRI in Children with a Congenital Heart Defect.

In fetuses with suspicion of congenital heart disease (CHD), assessment by segmental fetal echocardiography is of great importance. This study sought to examine the concordance of expert fetal echocardiography and postnatal MRI of the heart at a high-volume paediatric heart centre. The data of two hundred forty-two fetuses have been gathered under the condition of full pre- and postnatal and the presence of a pre- and postnatal diagnosis of CHD. The haemodynamically leading diagnosis was determined for each test person and was then sorted into diagnostic groups. The diagnoses and diagnostic groups were used for the comparison of diagnostic accuracy in fetal echocardiography. All comparisons between the diagnostic methods for detection of congenital heart disease showed an "almost perfect" (Cohen's Kappa > 0.9) strength of agreement for the diagnostic groups. The diagnosis made by prenatal echocardiography showed a sensitivity of 90-100%, a specificity and a negative predictive value of 97-100%, and a positive predictive value of 85-100%. The diagnostic congruence resulted in an "almost perfect" strength of agreement for all evaluated diagnoses (transposition of great arteries, double outlet right ventricle, hypoplastic left heart, tetralogy of Fallot, atrioventricular septal defect). An agreement of Cohen's Kappa > 0.9 was achieved for all groups, with exception of the diagnosis of double outlet right ventricle (0.8) in prenatal echocardiography compared to postnatal echocardiography. This study came to the result of a sensitivity of 88-100%, a specificity and negative predictive value of 97-100%, and a positive predictive value of 84-100%. The performance of cardiac magnetic resonance imaging (MRI) as an additional measure to echocardiography had an added value in the description of the malposition of the great arteries when diagnosed with double outlet right ventricle and in the detailed description of the anatomy of the lung circulation. Prenatal echocardiography could be shown to be a reliable method for detection of congenital heart disease when regarding the slightly lower accuracy of diagnosis for double outlet right ventricle and right heart anomalies. Furthermore, the impact of examiner experience and the consideration of follow-up examinations for further improvement of diagnosis accuracy may not be underestimated. The main advantage of an additional MRI is the possibility to obtain a detailed anatomic description of the blood vessels of the lung and the outflow tract. The conduction of further studies that include false-negative and false-positive cases, and studies that are not set within the high-risk-group, as well as studies in a less specialized setting, would allow the completion and investigation of possible differences and discrepancies when comparing the results that have been obtained in this study.

Aortic valve-sparing procedure in the pediatric population.

Aortic valve-sparing procedures have been established as a mainstream treatment option for adult patients with aortic root aneurysms. However, data regarding their use in the pediatric population is limited. This study reports on our experience with aortic valve-sparing procedures in children. A retrospective review was conducted of all patients who underwent an aortic valve-sparing procedure at the Royal Children's Hospital, Melbourne, Australia between April 2006 and April 2016. Clinical and echocardiographic data were analyzed. The study included 17 patients, with a median age of 15.7 years and a majority of patients being male (82.4%). The most common diagnosis was transposition of great arteries after arterial switch operation, followed by Loeys-Dietz syndrome and Marfan syndrome. Preoperative echocardiography showed more than moderate aortic regurgitation in 94.1% of the patients. The David procedure was performed in all 17 patients, with no mortality during follow-up. Reoperation was required in 29.4% of patients, and 23.5% required aortic valve replacement. Freedom from reoperation for aortic valve replacement at 1, 5, and 10 years was 93.8%, 93.8% and 68.2% respectively. Aortic valve-sparing surgery can be successfully performed in the pediatric population. However, it requires a highly skilled surgeon due to the often dysplastic or distorted nature of these valves and the need for additional procedures on the aortic valve leaflets.

Laparoscopic supramassive splenectomy weighing 1710 gm in a 17-year-old boy with a history of open heart surgery, transposition of great arteries and pulmonary artery stenosis with ventricular septal defect: a case report

When a spleen weighs more than 1,600 grams or has a craniocaudal length larger than 21 cm, it is categorized as a supramassive spleen. Laparoscopic splenectomy for normal to moderately enlarged spleens has become the gold standard in elective splenectomy surgery. Its utility in large splenomegaly has not yet completely established, but has great advantage over open surgery. Here, we present a case of 17-year-old young male with a history of congenital heart disease who underwent a Rastelli open heart surgery in 2010 and pulmonary balloon valvulotomy in 2022 came with supramassive splenomegaly, hypersplenism and pancytopenia that was treated with laparoscopic splenectomy. Vaccinations were done two weeks prior to the surgery. Preoperative embolization of the splenic artery was performed 24 hours before splenectomy to reduce complications. The excised spleen measured about 24×15 cm.

Prenatal diagnosis of fetal conotruncal defects by using 2D ultrasound and HD live flow combined with spatiotemporal image correlation.

To explore the diagnostic value of spatiotemporal image correlation (STIC) for different types of fetal conotruncal defects (CTDs). The clinical data and STIC images of 174 fetuses with CTDs diagnosed via prenatal ultrasound were analyzed retrospectively. Among the 174 cases of CTDs, 58 were tetralogy of Fallot (TOF); 30, transposition of great arteries (TGA) (D-TGA, 23 cases; cc-TGA, 7 cases); 26, double outlet of the right ventricle (DORV); 32, persistent arterial trunk (PTA) (type A1, 15 cases; type A2, 11 cases; type A3, 5 cases; type A4, 1 case); and 28, pulmonary atresia (PA) (ventricular septal defect, 24 cases; ventricular septal integrity, 4 cases). Among the cases, 156 were complicated with complex congenital intracardiac and extracardiac malformations. The abnormal display rate of the four-chamber view of two-dimensional echocardiography was low. The display rate of the permanent arterial trunk was the highest (90.6%) in STIC imaging. STIC imaging can be used in the diagnosis of different types of CTDs, especially in persistent arterial trunks, and thus has great value for the clinical treatment and prognosis of these defects.

Overshoot of the Respiratory Exchange Ratio during Recovery from Maximal Exercise Testing in Young Patients with Congenital Heart Disease

The overshoot of the respiratory exchange ratio (RER) after exercise is reduced in patients with heart failure. The present study aimed to investigate the presence of this phenomenon in young patients with congenital heart disease (CHD), who generally present reduced cardiorespiratory fitness. In this retrospective study, patients with CHD underwent a maximal cardiopulmonary exercise testing (CPET) assessing the RER recovery parameters: the RER at peak exercise, the maximum RER value reached during recovery, the magnitude of the RER overshoot and the linear slope of the RER increase after the end of the exercise. In total, 117 patients were included in this study. Of these, there were 24 healthy age-matched control subjects and 93 young patients with CHD (transposition of great arteries, Fontan procedure, aortic coarctation and tetralogy of Fallot). All patients presented a RER overshoot during recovery. Patients with CHD showed reduced aerobic capacity and cardiorespiratory efficiency during exercise, as well as a lower RER overshoot when compared to controls. RER magnitude was higher in the controls and patients with aortic coarctation when compared to those with transposition of great arteries, previous Fontan procedure, and tetralogy of Fallot. The RER magnitude was found to be correlated with the most relevant cardiorespiratory fitness and efficiency indices. The present study proposes new recovery indices for functional evaluation in patients with CHD. Thus, the RER recovery overshoots analysis should be part of routine CPET evaluation to further improve prognostic risk stratifications in patients with CHD.

TGA + IVS + LVOTO: patterns of practice and outcomes.

Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD). All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected. These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following "Nikaidoh, Kawashima, or LV-PA conduit" procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8). TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.