The Great Arteries Research Articles

Long-term results after the réparation à l'étage ventriculaire procedure for transposition of the great arteries and double-outlet right ventricle with pulmonary stenosis.

The purpose of this study is to describe the long-term results of the 'réparation à l'étage ventriculaire' (REV) technique for double-outlet right ventricle and transposition of the great arteries (TGA) with pulmonary stenosis (PS). Between 1980 and 2021, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, ventricular septal defect and PS (n = 116, 73.9%). Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 109 patients (69.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89.3%. Thirty-seven patients (23.6%) required 68 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months to 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6%, respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years. The REV procedure is a good alternative for TGA and double-outlet right ventricle with PS patients. Only a quarter of the patients required redo surgery on the RVOT. Reoperations for left ventricular outflow tract obstruction are scarce.

Intracardiac Three-Dimensional Image as Surgical Decision-Making Tool of Congenital Heart Disease.

This study aimed to evaluate the anatomical reproducibility of a preoperative intracardiac 3D image (IC image) created using computed tomography, and to investigate its usefulness as a surgical decision-making tool. Between 2012 and 2022, ventricular septal defect (VSD) patients, and double outlet right ventricle (DORV) or transposition of the great arteries (TGA) with pulmonary stenosis (PS) patients who underwent cardiac surgery and had preoperative computed tomography were enrolled. SYNAPSE VINCENT® (Fujifilm) was used to create an IC image which was analyzed retrospectively. In 14 VSD patients, the diagnostic consistency rate in the Soto classification with intraoperative findings was 100% (14/14) for IC image versus 64% (9/14) for transthoracic echocardiography (P = 0.04). The defect size showed a higher correlation coefficient with IC image (0.837, P = 0.001) than with transthoracic echocardiography (0.567, P = 0.034). In 11 DORV/TGA with PS patients, the diagnostic consistency rate in the Lev classification was 100% (9/9) for IC image versus 77% (7/9) for transthoracic echocardiography (P = 0.47). The secondary interventricular foramen (SVF)/left ventricular outflow tract (LVOT) ratio by IC image was significantly smaller in the biventricular-repair group (median 0.71, IQR 0.67-1.06) than in the univentricular-repair group (median 1.79, IQR 1.53-2.42) (P = 0.006). An IC image is useful as a surgical decision-making tool for simple VSDs and complex congenital heart diseases such as DORV or TGA with pulmonary stenosis. The SVF/LVOT ratio determined from the IC image may be a useful indicator for avoiding LVOT obstruction.

Dapaglifozin in adults with a systemic right ventricle:initial results from the DAPA-SERVE trial

Abstract Background Sodium-glucose Cotransporter-2(SGLT2) inhibitors are currently recommended as first line therapy for all patients with heart failure with reduced ejection fraction (HFrEF).Adults with a systemic right ventricle (sRV) may develop progressive systolic dysfunction during follow-up. Optimal medical therapy for HFrEF remains still to be determined in this complex population. Purpose We aimed to assess safety and evaluate potential clinical benefit of dapagliflozin in patients with a sRV compared to standard medical treatment for HF. Methods Inclusion criteria were:age ≥ 18 years; transposition of the great arteries (TGA) following Senning/Mustard repair or congenitally corrected TGA, sRV EF ≤ 40%, already on optimized medical therapy including sacubitril/valsartan for at least 3 months. Exclusion criteria were: systolic blood pressure (SBP) <90 mmHg, glomerular filtration rate (GFR)<30ml/min and univentricular physiology. All eligible patients attending our tertiary center for adult congenital heart disease were randomly assigned to the treatment or control group. Patients in the treatment arm were prescribed 10 mg dapagliflozin o.d. on top of their medical therapy, while control group continued standard therapy. Patients were scheduled for a structured follow-up including clinical evaluation, blood test, echocardiography, and 6 minute-walking at 6 months from study inclusion for both groups. Results Fifty patients (41±11 years, 54% male, 64% TGA) met the inclusion criteria: 25(50%) were started on 10 mg dapagliflozin. There was no significant difference in terms of age (p=0.4), sex(p=0.1), anatomy(p=0.2) and baseline sRV EF (p=0.2). Up to February 2023, 14 patients in the treatment group and 13 controls completed the 6-month follow-up. Clinical and echocardiographic findings at baseline and at 6-month in the treatment group are summarized in Table1: dapagliflozin was well tolerated and no major adverse events were reported. Treatment did not impact on the systolic blood pressure and renal function. However a significant improvement in the sRV freewall global longitudinal strain(GLS) was found (-15.8±3 Vs -18.3±3; p=0.03). Differences in main echocardiographic indices of sRV systolic function between baseline and follow-up in the two arms are shown in Table2: treatment resulted in significant positive changes of sRV FAC, GLS, and free wall GLS compared to the group on standard therapy. Conclusions Initial results from the ongoing single-centre DAPA-SERVE trial provided reassuring data on the safety profile of dapagliflozin in patients with a sRV. Furthermore, significant improvement of echocardiographic indices of sRV function in patients on dapagliflozin compared to those on standard medical therapy was demonstrated at 6-month. Longer follow-up is warranted to determine the clinical impact of those positive changes and to shed light on the potential benefit of SGLT2 inhibitors use on top of standard therapy in this complex population.

Neo-aortic dimensions and coronary take-off angle in adolescent and young adult patients with transposition of the great arteries after the arterial switch operation

Abstract Objective Aim of this study was (1) to assess the relation between neo-aortic dimensions and coronary angle take-off in adult patients with transposition of the great arteries (TGA) late after the arterial switch operation (ASO) and (2) to evaluate if temporal increase in the neo-aortic dimensions is associated with a decrease of the coronary angle take off. Methods In this retrospective two-center cohort study, all patients with TGA who underwent ASO and had ≥1 CT-angiography (CTA) of the coronary arteries at the age of ≥ 16 years old were included. Neo-aortic dimensions and coronary angle take-off were measured with CTA, Figure 1. Results Eighty-one patients, 69% male and median age 21.0 (18.5-22.8) years, were included. Maximum neo-aortic diameter was 39.2±5.3mm (corrected for BSA 20.9±2.7mm/m2). Sixty-one (76%) patients had a coronary take-off angle of <45° at baseline. No correlation between the maximum neo-aortic diameter and coronary take-off angle of the right coronary artery (RCA) (R=0.161, p=0.150) and left coronary artery (LCA) (R=0.089, p=0.428) was found, Figure 2A-B. During a mean follow up of 7.9±3.1 years in patients with 2 CTAs (n=26), the average increase of maximum neo-aortic diameter at the level of sinus of Valsalva was 0.16±0.20mm/year and a poor correlation between the change in maximum aortic diameter and change in coronary take-off angle of the RCA (R=0.126, p=0.541) and LCA (R=0.311, p=0.158) was observed, Figure 2C-D. Conclusions This study reports slightly increased neo-aortic dimensions and high prevalence of acute coronary angle take-off in young adults late after ASO. The average neo-aortic growth rate of 0.16 mm/year at the level of the sinus of Valsalva is twice as fast as reported for healthy adults. However, no significant correlation was found between the neo-aortic dimensions and coronary take-off angle at baseline or during follow-up of almost 8 years in this group.Figure 1Figure 2

Abstract 17366: Effects of Acute Maternal Supplemental Oxygen on the Fetal Circulation in Transposition of the Great Arteries

Introduction: The presence of a restrictive atrial septum in the setting of transposition of the great arteries (TGA) is associated with a high risk of hypoxic ischemic injury upon transition to postnatal circulation. Fetal cardiovascular magnetic resonance (CMR) measures of vessel flow and oxygen saturation provide a unique opportunity to study fetal TGA hemodynamics at baseline and during acute maternal hyperoxygenation. Methods: Thirty-one pregnant mothers with fetuses diagnosed with TGA and an intact ventricular septum (n=23) or small ventricular septal defect (n=8) were recruited and underwent fetal CMR in room air and during administration of 10L/min of oxygen by face mask for up to 45 minutes. Blood flow and oxygen saturations were measured in the major fetal vessels using cine phase contrast MRI and MR relaxometry at baseline and during hyperoxygenation. Results: Two patients exhibited severe restriction at the atrial septum, one undergoing fetal atrial septostomy at 37 weeks and one requiring cannulation onto ECMO shortly after birth. Both revealed reversal of the usual relationship of oxygen saturation between the aorta and the main pulmonary artery at baseline and during hyperoxygenation, i.e. in TGA with restrictive atrial septum the saturations are higher in the aorta than pulmonary. Acute maternal supplemental oxygen was associated with increased pulmonary blood flow (p=0.008) and reduced foramen ovale (p=0.01) and ductus arteriosus shunting (p=0.01). There were no significant changes in umbilical vein, descending aorta and superior vena cava flow. Fetal oxygen saturations increased throughout fetal circulation (p<0.0001) with an associated increase in fetal oxygen delivery (p=0.007), but no increase in fetal cerebral oxygen delivery (p=0.58). Conclusions: This study characterized the effects of acute maternal supplemental oxygen on the fetal circulation in patients with TGA and confirmed the redistribution of flow toward the fetal pulmonary circulation. We observed higher oxygen saturations in the aorta than main pulmonary artery in fetuses with TGA with an intact or highly restrictive atrial septal communication. This finding could be helpful in selecting patients for prenatal or emergency neonatal balloon atrial septostomy.

Novel uses for implanted haemodynamic monitoring in adults with subaortic right ventricles

BackgroundPulmonary hypertension (PH) is a common complication in patients with complete dextro-transposition of the great arteries (TGA) after atrial switch (D-TGA/AS) and congenitally corrected TGA (ccTGA). In this population with...

Anaesthetic Management during Balloon Atrial Septostomy in Transposition of Great Arteries

<strong>Background: </strong>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.<p><strong>Discussion:</strong> 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.</p><p><strong>Conclusion:</strong> 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.</p>

Late follow-up of neo-aortic dimensions and coronary arteries in adult patients after the arterial switch operation

BackgroundAfter the arterial switch operation (ASO) for transposition of the great arteries (TGA), neo-aortic dilatation and coronary arterial anomalies, especially an interarterial course and acute coronary artery take-off angle, are commonly found. Long-term follow-up data after ASO is scarce. Aim of this study was to determine the prevalence of neo-aortic dilatation and coronary abnormalities, with special emphasis on acute coronary take-off angle, in adult TGA-ASO patients. MethodsIn this retrospective cohort study, all adult TGA-ASO patients with ≥1 CT-angiography (CTA) at the age of ≥16 years were included. ResultsEighty-one patients, 69 % male and median age 21.0 [18.5–22.8] years, were included. At baseline, maximum neo-aortic diameter was 39.2 ± 5.3 mm; 35 (43 %) patients had neo-aortic dilatation (neo-aortic diameter of >40 mm), 22 (27 %) patients had an acute coronary take-off angle (<30°), and 5 (6 %) patients had an interarterial course of the RCA (2 %) or LCA (4 %). Neo-aortic or coronary artery re-intervention occurred in 10 (12 %) patients. All 10 patients had neo-aortic dilatation or coronary take-off angle of <30° on baseline CTA. ConclusionThis study reports a prevalence of 43 % of neo-aortic dilatation, 6 % of interarterial coronary course and 27 % for acute coronary take-off angle (<30°) at a median term of 21.0 years post ASO. All patients with a neo-aortic re-intervention or coronary artery re-intervention during follow-up had a maximum neo-aortic diameter of >40 mm or a coronary take-off angle of <30° at baseline CTA. This hypothesis generating study suggests that an active surveillance in patients with neo-aortic dilation and/or an acute angulation of < 30° post ASO might be considered and requires prospective evaluation.

Fetal circulatory physiology and brain development in complex congenital heart disease: A multi-modal imaging study.

Fetuses with complex congenital heart disease have altered physiology, contributing to abnormal neurodevelopment. The effects of altered physiology on brain development have not been well studied. We used multi-modal imaging to study fetal circulatory physiology and brain development in hypoplastic left heart syndrome (HLHS) and d-transposition of the great arteries (TGA). This prospective, cross-sectional study investigated individuals with fetal congenital heart disease and controls undergoing fetal echocardiography and fetal brain MRI. MRI measured total brain volume and cerebral oxygenation by the MRI quantification method T2*. Indexed cardiac outputs (CCOi) and vascular impedances were calculated by fetal echocardiography. Descriptive statistics assessed MRI and echocardiogram measurement relationships by physiology. Sixty-six participants enrolled (control=20; HLHS=25; TGA=21), mean gestational age 33.8weeks (95% CI: 33.3-34.2). Total brain volume and T2* were significantly lower in fetuses with cardiac disease. CCOi was lower in HLHS, correlating with total brain volume - for every 10% CCOi increase, volume increased 8mm3 (95% CI: 1.78-14.1; p=0.012). Echocardiography parameters and cerebral oxygenation showed no correlation. TGA showed no CCOi or aortic output correlation with MRI measures. In HLHS, lower cardiac output is deleterious to brain development. Our findings provide insight into the role of fetal cardiovascular physiology in brain health.

Pattern of congenital heart defects among children in Jazan Region, Saudi Arabia: A five-year hospital-based study

ABSTRACT Background: Congenital heart defects (CHDs) are the leading cause of death and morbidity in children under one year around the world. This study explores the prevalence, patterns, and interventions of congenital heart defects in children in the Jazan region, Saudi Arabia. Methods: This was a descriptive cross-sectional study, conducted in the Jazan region, Saudi Arabia. We looked back at all of the CHD patients who went to the two tertiary hospitals in the Jazan region between January 2017 and December 2021 and collected and analyzed their background information and diagnosis. Results: During the study period, 1,275 CHD patients aged under 15 years old were identified. A total of 717 (56.24%) were males, while 558 (43.76%) were females. Acyanotic CHDs were the most common, accounting for 79.21% of all cases, while cyanotic CHDs accounted for only 20.79%. Ventricular septal defect (VSD), atrial septal defect (ASD), and patent ductus arteriosus (PDA) were the most common acyanotic CHDs, accounting for 35.69%, 24.31%, and 8% of total cases, respectively. The most common cyanotic CHDs were Tetralogy of Fallot (7.14%) and Total Anomalies of Pulmonary Venous Connection (TAPVC) (1.57%). The most common Cath intervention was patent ductus arteriosus (PDA). Conclusions: This study’s results are similar to those described in previous studies from around the world, except for the unreported Transposition of the Great Arteries (TGA) cases. This kind of information is very important for setting priorities in the Saudi Arabian health sector in light of the National Transformation Program.

Optimizing Surgical Selection for Transposition With Left Ventricular Outflow Tract Obstruction

BackgroundStudies that have assessed the Rastelli and Nikaidoh operations for transposition of the great arteries (TGA) with obstructed left ventricular outflow tract obstruction (LVOTO) have not fully evaluated the anatomic drivers that may contribute to surgical selection. We present our procedural selection process for optimizing outcomes of complex TGA in the modern era. MethodsThis is a single-center, retrospective study that included pediatric patients who underwent either a Nikaidoh or Rastelli operation for the treatment of TGA-LVOTO, congenitally corrected TGA–LVOTO, or double-outlet right ventricle TGA type–LVOTO from June 2004 to June 2021. ResultsThere were 34 patients stratified by Nikaidoh (n = 16) or Rastelli (n = 18) operation. The incidence of all postoperative complications and mortality was low, and the incidence of complications between the groups was similar. Patients were more likely to have undergone a Nikaidoh than a Rastelli if they had a pulmonary annulus >5 mm (87.5% vs 11.1%), anteriorly/posteriorly oriented great vessels (88% vs 8%), remote (80% vs 11%) or restrictive (75% vs 6%) ventricular septal defect, and right ventricular hypoplasia (50% vs 0%; all, P < .05). The resulting rates of reoperation were similar between the groups (44.0% vs 37.5%; P = .24) and largely composed of conduit replacements in the Rastelli patients and valvular repairs or replacements in the Nikaidoh group. Rates of catheter-based interventions were also similar. ConclusionsThese findings suggest that for the optimal treatment of conotruncal anomalies with discordant ventriculoarterial connections, procedural selection should be based on pathoanatomic criteria that can ensure patients undergo the operation most suited to their anatomy.

Can We Safely Decrease Early-Term Delivery and Cesarean Section Rate in Pregnancies Complicated by Fetal Transposition of Great Arteries?

Background: Transposition of the great arteries (TGA) is a common critical neonatal congenital heart defect. After birth, physiological shunts close rapidly, necessitating early treatment with prostaglandin infusion and balloon-atrial septostomy. Timing of delivery is challenging, balancing the risks and advantages of early-term delivery and specialized care. The aim of this study is to assess the safety of a full-term delivery policy in fetuses diagnosed with TGA. Methods: A retrospective chart review was conducted of 17 women with a prenatal diagnosis of fetal TGA at Virgen del Rocío University Hospital between 2015 and 2021. Primary outcomes included: incidence of preterm, early-term, full-term, and late-term delivery, and rate of cesarean section. Secondary outcomes included: Saturday to Sunday admission and birth, and delivery between 0:00 a.m. and 8:00 a.m. Results: Full-term birth was achieved in 94.1%, reaching a low cesarean delivery rate (17.6%). A total of 82.4% of infants were born on weekdays, and only in three of the cases (17.6%) did delivery occur between 0 a.m. and 8 a.m. The median birth weight was 3300 g. Intravenous prostaglandins were administered in all cases, and 94.1% required balloon-atrial septostomy. Conclusions: In our study favoring full-term delivery, we reduce early-term deliveries and the cesarean section rate in prenatally diagnosed TGA.

Comparison of Four-Dimensional Flow MRI, Two-Dimensional Phase-Contrast MRI and Echocardiography in Transposition of the Great Arteries

Pulmonary artery (PA) stenosis is a common complication after the arterial switch operation (ASO) for transposition of the great arteries (TGA). Four-dimensional flow (4D flow) CMR provides the ability to quantify flow within an entire volume instead of a single plane. The aim of this study was to compare PA maximum velocities and stroke volumes between 4D flow CMR, two-dimensional phase-contrast (2D PCMR) and echocardiography. A prospective study including TGA patients after ASO was performed between December 2018 and October 2020. All patients underwent echocardiography and CMR, including 2D PCMR and 4D flow CMR. Maximum velocities and stroke volumes were measured in the main, right, and left PA (MPA, LPA, and RPA, respectively). A total of 39 patients aged 20 ± 8 years were included. Maximum velocities in the MPA, LPA, and RPA measured by 4D flow CMR were significantly higher compared to 2D PCMR (p < 0.001 for all). PA assessment by echocardiography was not possible in the majority of patients. 4D flow CMR maximum velocity measurements were consistently higher than those by 2D PCMR with a mean difference of 65 cm/s for the MPA, and 77 cm/s for both the RPA and LPA. Stroke volumes showed good agreement between 4D flow CMR and 2D PCMR. Maximum velocities in the PAs after ASO for TGA are consistently lower by 2D PCMR, while echocardiography only allows for PA assessment in a minority of cases. Stroke volumes showed good agreement between 4D flow CMR and 2D PCMR.

Graph theory applications in congenital heart disease

Graph theory can be used to address problems with complex network structures. Congenital heart diseases (CHDs) involve complex abnormal connections between chambers, vessels, and organs. We proposed a new method to represent CHDs based on graph theory, wherein vertices were defined as the spaces through which blood flows and edges were defined by the blood flow between the spaces and direction of the blood flow. The CHDs of tetralogy of Fallot (TOF) and transposition of the great arteries (TGA) were selected as examples for constructing directed graphs and binary adjacency matrices. Patients with totally repaired TOF, surgically corrected d-TGA, and Fontan circulation undergoing four-dimensional (4D) flow magnetic resonance imaging (MRI) were included as examples for constructing the weighted adjacency matrices. The directed graphs and binary adjacency matrices of the normal heart, extreme TOF undergoing a right modified Blalock–Taussig shunt, and d-TGA with a ventricular septal defect were constructed. The weighted adjacency matrix of totally repaired TOF was constructed using the peak velocities obtained from 4D flow MRI. The developed method is promising for representing CHDs and may be helpful in developing artificial intelligence and conducting future research on CHD.

Long-term outcome of patients with transposition of the great arteries and a systemic right ventricle: A systematic review and meta-analysis

BackgroundPatients with a transposition of the great arteries (TGA) and a systemic right ventricle are at risk of heart failure (HF) development, arrhythmia and early mortality. Prognostic evaluations in clinical studies are hampered by small sample sizes and single-centred approaches. We aimed to investigate yearly rate of outcome and factors affecting it. MethodsA systematic literature search of four electronic databases (PubMed, EMBASE, Web of Science and Scopus) was conducted from inception to June 2022. Studies reporting the association of a systemic right ventricle with mortality with a minimal follow-up of 2 years during adulthood were selected. Incidence of HF hospitalization and/or arrhythmia were captured as additional endpoints. For each outcome, a summary effect estimate was calculated. ResultsFrom a total of 3891 identified records, 56 studies met the selection criteria. These studies described the follow-up (on average 7.27 years) of 5358 systemic right ventricle patients. The mortality incidence was 1.3 (1–1.7) per 100 patients/year. The incidence of HF hospitalization was 2.6 (1.9–3.7) per 100 patients/year. Predictors of poor outcome were a lower left ventricular (LV) and right ventricular ejection fraction (RVEF) (standardized mean differences (SMD) of −0.43 (−0.77 to −0.09) and − 0.85 (−1.35 to −0.35), respectively), higher plasma concentrations of NT-proBNP (SMD of 1.24 (0.49–1.99)), and NYHA class ≥2 (risk ratio of 2.17 (1.40–3.35)). ConclusionsTGA patients with a systemic right ventricle have increased incidence of mortality and HF hospitalization. A lower LVEF and RVEF, higher levels of NT-proBNP and NYHA class ≥2 are associated with poor outcome.

P147 THE WINNING FORMULA IN COMPLEX ADULT CONGENITAL REDO INTERVENTIONS: MULTIDIMENSIONAL PERSPECTIVE IN A HYBRID SETTING

Abstract Introduction Transposition of the great artery is one of the most common congenital heart anomalies [1]. One of the surgical techniques to correct this anomaly is the Rastelli procedure [2]. After surgical correction, right and left ventricular outflow obstruction are the most common late complications [3]. In grown–up congenital disease (GUCH) late surgery is a high risk procedure and, repeated sternotomy may be associated with adverse injuries to mediastinal structures.The aim of this work is to demonstrate how a preoperative planning may be helpful in identifying patients at risk of re–entry. Case presentationA 50–year–old woman, with primary diagnosis of transposition of the great arteries (TGA), ventricular septal defect (VSD) and pulmonary stenosis (PS) was referred to our Grown Up Congenital Heart (GUCH) center for progressive dyspnoea and reduced exercise tolerance. She underwent a Blalock–Taussig shunt palliation at the age of 3 and a Rastelli procedure after 2 years. Echocardiography revealed moderate–to–severe RV–PA conduit obstruction (122 mmHg) and a left Ventricle–Aorta (LV–Ao) tunnel obstruction (Mean gradient 32 mmHg, Max gradient 52 mmHg). Cardiac MRI and cardiac catheterization confirmed the echo findings and showed the proximity of the RV–PA conduit to the left anterior descending coronary artery, which was considered an exclusion criteria for trans–catheter therapy. A CT scan confirmed diagnosis and showed that mediastinal structures, in particular the aorta, were adherent to the sternum. (Fig 1). After careful examination of the clinical case, our Heart Team decided for high risk surgical treatment of the residual lesions.A percutaneous cannulation was performed, to minimize the re–entry injury risks. Two other percutaneous devices were used: the coronary sinus cannula and the percutaneous left heart venting catheter. The postoperative course was regular and one month after surgery a CT scan confirmed a good result of surgery (Fig 2). Conclusions This peculiar case is emblematic to understand how a multidimensional perspective is mandatory in redo GUCH interventions. Hybrid surgery room setting and a multidisciplinary team of surgeons, interventional cardiologists, anesthesiologists and radiologists are essential to reach the goal of successful outcomes for these kinds of complex procedures as a GUCH “big operation”.

Tolerability and beneficial effects of sacubitril/valsartan on systemic right ventricular failure

ObjectivePatients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are prone to sRV dysfunction....

The impact of COVID-19 on a South African pediatric cardiac service: implications and insights into service capacity.

The Western Cape public pediatric cardiac service is under-resourced. COVID-19 regulations are likely to have long-term effects on patient care but may provide insight into service capacity requirements. As such, we aimed to quantify the impact of COVID-19 regulations on this service. An uncontrolled retrospective pre-post study of all presenting patients over two, one-year periods; the pre-COVID-19 period (01/03/2019-29/02/2020) and the peri-COVID-19 period (01/03/2020-28/02/2021). Admissions decreased by 39% (624 to 378) and cardiac surgeries decreased by 29% (293 to 208) in the peri-COVID-19 period, with an increase in urgent cases (PR:5.99, 95%CI:3.58-10.02, p < 0.001). Age at surgery was lower in the peri-COVID-19 period, 7.2 (2.4-20.4) vs. 10.8 (4.8-49.2) months (p < 0.05), likewise, age at surgery for transposition of the great arteries (TGA) was lower peri-COVID-19, 15 (IQR:11.2-25.5) vs. 46 (IQR:11-62.5) days (p < 0.05). Length of stay 6 (IQR:2-14) vs. 3 days (IQR:1-9) (p < 0.001), complications (PR:1.21, 95%CI:1.01-1.43, p < 0.05), and age-adjusted delayed-sternal-closure rates (PR:3.20, 95%CI:1.09-9.33, p < 0.05) increased peri-COVID-19. Cardiac procedures were significantly reduced in the peri-COVID-19 period which will have implications on an overburdened service and ultimately, patient outcomes. COVID-19 restrictions on elective procedures freed capacity for urgent cases, demonstrated by the absolute increase in urgent cases and significant decrease in age at TGA-surgery. This facilitated intervention at the point of physiological need, albeit at the expense of elective procedures, and also revealed insights into capacity requirements of the Western Cape. These data emphasize the need for an informed strategy to increase capacity and reduce backlog whilst ensuring minimal morbidity and mortality.Graphical Abstract.

Cardiac Fibrosis and Innervation State in Uncorrected and Corrected Transposition of the Great Arteries: A Postmortem Histological Analysis and Systematic Review

In the transposition of the great arteries (TGA), alterations in hemodynamics and oxygen saturation could result in fibrotic remodeling, but histological studies are scarce. We aimed to investigate fibrosis and innervation state in the full spectrum of TGA and correlate findings to clinical literature. Twenty-two human postmortem TGA hearts, including TGA without surgical correction (n = 8), after Mustard/Senning (n = 6), and arterial switch operation (ASO, n = 8), were studied. In newborn uncorrected TGA specimens (1 day-1.5 months), significantly more interstitial fibrosis (8.6% ± 3.0) was observed compared to control hearts (5.4% ± 0.8, p = 0.016). After the Mustard/Senning procedure, the amount of interstitial fibrosis was significantly higher (19.8% ± 5.1, p = 0.002), remarkably more in the subpulmonary left ventricle (LV) than in the systemic right ventricle (RV). In TGA-ASO, an increased amount of fibrosis was found in one adult specimen. The amount of innervation was diminished from 3 days after ASO (0.034% ± 0.017) compared to uncorrected TGA (0.082% ± 0.026, p = 0.036). In conclusion, in these selected postmortem TGA specimens, diffuse interstitial fibrosis was already present in newborn hearts, suggesting that altered oxygen saturations may already impact myocardial structure in the fetal phase. TGA-Mustard/Senning specimens showed diffuse myocardial fibrosis in the systemic RV and, remarkably, in the LV. Post-ASO, decreased uptake of nerve staining was observed, implicating (partial) myocardial denervation after ASO.

Impact of Bedside Balloon Atrial Septostomy in Neonates with Transposition of the Great Arteries in a Neonatal Intensive Care Unit in Romania.

(1) Background: Transposition of the great arteries (TGA) is the most common congenital heart disease, accounting for 5-7% of all cardiac anomalies, with a prevalence of 0.2-0.3 per 1000 live births. (2) Aim: Our main objectives were to evaluate the clinical safety of balloon atrial septostomy in neonates and the possible complications. Furthermore, we tried to establish whether the procedure should be performed in all TGA patients with small atrial septal defects, regardless of oxygen saturation, within a center where corrective surgery cannot be performed on an emergency basis due to the lack of a permanent cardiac surgery team for arterial switch surgery. (2) Methods: We conducted an observational, retrospective, single tertiary-care center study between January 2008 and April 2022, which included 92 neonates with TGA transferred to our institution for specialized treatment. (3) Results: The median age at the time of the Rashkind procedure was four days. The rate of immediate complications after balloon atrial septostomy (BAS) was high (34.3%), but most were transient (metabolic acidosis and arterial hypotension-21.8%). Twenty patients with TGA managed in our hospital underwent definitive and corrective surgical intervention (arterial switch operation) at a median age of 13 days. Most patients (82.6%) were term neonates, but 16 were born preterm. (4) Conclusions: Urgent balloon atrial septostomy is often the only solution to restore adequate systemic perfusion. Bedside balloon atrial septostomy is a safe, effective, and initial palliative intervention in neonates with TGA, which can be performed in the neonatal unit.