Switch Operation For Transposition Research Articles

Successful Management of a Very Unusual Coronary Pattern in Transposition of the Great Arteries.

Coronary artery anatomy is the key to a successful arterial switch operation in transposition of the great arteries. We came across an unusual coronary pattern in a child with transposition in which the three major coronary arteries were seen arising from all three aortic sinuses. This coronary pattern is the first of its kind in transposition, and this case report emphasizes the difficulty in translocating such a rare coronary pattern while performing an arterial switch operation.

EVAHEART 2 with double cuff tipless inflow cannula is suitable for long-term support atrial switch operation in transposition of great arteries.

Implantation of continuous-flow left ventricular assist device in a narrow lumen is technically challenging to secure an optimal support. We experienced a patient with the transposition of the great arteries after the Senning procedure who was initially implanted with Jarvik 2000®. She presented with worsening heart failure symptoms 2 years after implanting Jarvik 2000®. We assumed that the inflow cannula was stuck in the highly developed trabeculae on the interventricular septum, which disturbed the VAD to maintain an expected support. After converting to the EVAHEART® 2, we successfully obtained an adequate inflow. We consider that the tipless cannula of EVAHEART® 2 is the most suitable when there is no sufficient room to place a conventional inflow cannula in the systemic ventricle.

Common genetic variants improve risk stratification after atrial switch operation for transposition of the great arteries

Abstract Background Current clinical risk scores are able to predict late complication risk in adults after atrial switch operation (AtrSO) for transposition of the great arteries (TGA), but a large heterogeneity in clinical course remains. Purpose To study whether common genetic factors are predictive of outcome and provide added value to an existing clinical risk score in TGA-AtrSO patients. Methods This multicenter study examined the association of genome-wide single-nucleotide polymorphisms (SNPs) in TGA-AtrSO patients with a combined clinical endpoint: time to symptomatic ventricular arrhythmia, heart failure hospitalization, ventricular assist device implantation, heart transplantation, or mortality. Furthermore, we evaluated whether a polygenic risk score (PRS) constructed of independent single-nucleotide polymorphisms (SNPs) with a p<1x10–5 could be of added value to a recently published clinical risk score (included clinical factors: age >30 years, prior ventricular arrhythmia, age >1 year at repair, ≥moderate right ventricular dysfunction, severe tricuspid regurgitation, and ≥mild left ventricular dysfunction). Results We followed 133 patients (age at inclusion 28 [IQR 24–35] years, 59% male) for 13 [IQR 8–16] years. Thirty-two patients (24%) reached the endpoint. The genome-wide association study yielded one locus that reached genome-wide significance (p<1x10–8) and 18 loci marked by 20 SNPs that reached the suggestive threshold (p<1x10–5). The constructed PRS remained an independent predictor after correction for the clinical score (HR=1.21/point increase [95% CI 1.13–1.29], p=3x10–10). While the clinical risk score indicated intermediate (5–20%) 5-year risk of events in 52 patients (39%), the combined risk score (clinical score + PRS) reclassified 35 patients to low (<5%) and 6 to high (>20%) risk. Observed 5-year event-free survival based on the combined score remained 100% for low-risk patients, compared to 23% and 64% in intermediate and high-risk patients, respectively. This resulted in improved risk stratification with the combined risk score vs the clinical risk score alone (p=2x10–16, C-statistic 0.95 vs 0.85). Conclusions Genetic factors explain some of the variation in clinical course of TGA-AtrSO patients and improve risk stratification. In the heterogeneous group of patients with a clinical score indicating intermediate risk, the combined model could classify 67% of patients more accurately to <5% or >20% risk. These data argue for more research into the impact of genetics on clinical outcome in adult congenital heart disease. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Dutch Heart Foundation Figure 1. Risk stratification of predicted 5-year risk of events based on the clinical model versus the combined model (clinical risk score + polygenic risk score).

Arterial switch operation for transposition of great arteries: Is coronary artery circulation evaluation necessary?

Arterial switch operation for transposition of great arteries: Is coronary artery circulation evaluation necessary?

Abstract 14443: Aortic Root Rotational Position Associates With Aortic Valve Incompetence and Aortic Dilation After Arterial Switch Operation for Transposition of the Great Arteries

Introduction: Variation in aortic root (AoR) rotational position affects flow dynamics in normal patients. Complications of arterial switch operation (ASO) for transposition of the great arteries (TGA) include aortic dilation and neo-aortic regurgitation (AR). We sought to assess the association of neo-AoR rotational position with neo-AoR and ascending aorta (AA) dilation and neo-AR in TGA after ASO. Methods: Patients after ASO for TGA who underwent cardiac magnetic resonance (CMR) from 2005-2020 were retrospectively reviewed. Neo-AoR rotational angle, indexed (to height) neo-AoR and AA dimensions, indexed left ventricular end diastolic volume (LVEDVi), and neo-aortic regurgitant fraction (RF) were obtained from CMR. Multivariable regression analysis for neo-AoR and AA dilation, RF, and LVEDVi were performed. Results: A total of 36 patients (78% male) were included. Median age at ASO was 5 days (3, 7) and at CMR was 17.1 years (12.3, 21.9). Rotational angle was 25% counterclockwise (<-9°), 25% central (-9 to +14°), and 50% clockwise (>+15°). There was no material association of neo-AoR and AA size with sex, age, and coronary artery anatomy. A quadratic term for neo-AoR rotational angle - increasing extremes of counterclockwise and clockwise angles - was significantly associated with neo-AoR dilation (p=0.03), AA dilation (p<0.02), and LVEDVi (p<0.01) (Figure 1) and remained significant in multivariable model of neo-AoR dilation (p<0.02), AA dilation (p<0.01), and LVEDVi (p<0.02). Rotational angle was negatively associated with neo-aortic RF (p<0.05) and remained significant in multivariable model (p<0.02). Conclusions: In conclusion, both clockwise and counterclockwise neo-AoR rotational position in TGA after ASO are associated with neo-AoR and AA dilation and LVEDVi. Counterclockwise rotational angle was associated with RF. Neo-AoR rotational position likely affects valve function and hemodynamics, leading to risk of neo-AR and aortic dilation.

Clinical course long after atrial switch: a novel risk score for serious clinical events

Abstract Background Patients after atrial switch surgery for transposition of the great arteries (TGA-AtrSO) experience serious clinical events during adulthood, mainly heart failure and arrhythmias, but data on the emerging risks remain scarce. Purpose To assess the risk for events during the clinical course in adulthood of TGA-AtrSO patients and provide a novel risk score for event-free survival. Methods We reviewed medical records of TGA-AtrSO patients from five hospitals. Endpoints were all-cause mortality, heart failure (HF), defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF-related death, and symptomatic ventricular arrhythmias (VA). Predictors for event-free survival were examined to construct a prediction model using bootstrapping techniques. Results We followed 169 TGA-AtrSO patients (60% Mustard, age 28 [IQR 24–36] years) for 13 [IQR 9–16] years, during which 17 (10%) died, 34 (20%) had HF events, and 15 (9%) had VA events. Five-year risk of mortality, first HF event, and first VA increased from 1% each at age 25, to 7% (95% CI 4–10%), 17% (95% CI 10–25%), and 4% (95% CI 2–8%), respectively, at age 50. A prediction model combining age >30, prior VA, age >1 year at repair surgery, QRS duration >120ms, ≥mild LV dysfunction, and severe tricuspid regurgitation discriminated well between patients at low (<5%), medium (5–20%) and high (>20%) 5-year risk (optimism corrected C-statistic=0.84). Observed 5- and 10-year survival in low-risk patients were 100% and 99%, compared to only 45% and 19% in high-risk patients. Conclusion The clinical course of atrial switch patients increasingly consists of serious clinical events, especially heart failure. A novel risk score stratifying patients as low, medium, and high risk for event-free survival is presented, providing information on absolute individual risks which may support decisions for pharmacological and interventional management. Figure 1. Observed event-free survival of patients with predicted low risk (<5% in 5 years), medium risk (5–20% in 5 years) and high risk (>20% in 5 years). Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): Dutch Heart Foundation; Amsterdam University Fund

P2742Right ventricular stroke volume during dobutamine stress magnetic resonance compared to lung perfusion and peak oxygen uptake after arterial switch operation for transposition of the great arteries

Abstract Objectives Abnormal pulmonary perfusion due to stenosis of the central pulmonary arteries is common after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA). We conducted a monocentric prospective study in young adults after neonatal ASO for TGA to evaluate the effects of abnormal pulmonary perfusion on the increase of the right ventricular stroke volume (RVSV) during dobutamine stress magnetic resonance (DSMR) and on cardiopulmonary exercise capacity. Methods 68 unselected patients (age 18–29 years) underwent CMR at rest and under dobutamine stress (10 to 40 μg/kg/min). RVSV, pulmonary blood flow distribution (PBFD) and peak flow velocity were derived from phase contrast mapping in the main, right and left pulmonary artery (PA) at rest and each stress level. A cardiopulmonary exercise test (CPET) was performed at the same day. All patients reached maximal exercise effort according to heart rate and respiratory exchange rate. Results PBFD at rest: 9/68 patients (13%, ASO-S) had abnormal pulmonary perfusion at rest, defined as PBFD >2:1 (right/left or left/right) and/or relevant stenosis of the main PA (Vmax >2.5 m/s). 59/68 patients (87%, ASO-N) had normal pulmonary perfusion. PBFD under DSMR: (1) In the whole patient group, there was no increase of PBFD under stress compared to PBFD at rest. On an individual patient level, no relevant worsening of abnormal PBFD was found. (2) Under low dose dobutamine, ASO-S had a significantly lower RVSV-increase (RVSVstress/RVSVrest) compared to ASO-N (see figure). However, under high dose dobutamine, this effect was no longer significant (see figure). (3) The RVSV-increase under low and high dose dobutamine did not correlate with peak oxygen uptake during CPET, neither in the total group nor in the subgroups (see table). Peak oxygen uptake was not significantly different between ASO-N and ASO-S (p=0,72). RVSV-increase compared to CPET peak VO2% ASO-total ASO-S ASO-N RVSV-increase/peak VO2% RVSV-increase/peak VO2% RVSV-increase/peak VO2% low dose dobu high dose dobu low dose dobu high dose dobu low dose dobu high dose dobu Pearson correlation coefficient −0.02 −0.05 0.01 −0.22 −0.05 −0.04 p-value 0.90 0.71 0.98 0.56 0.71 0.78 Figure 1 Conclusion (1) Patients with relevant stenosis of main PA and/or abnormal peripheral blood flow distribution (ASO-S) exhibit a reduced RVSV-increase under low dose dobutamine compared to patients without stenosis (ASO-N). This effect was not present under high dose dobutamine stress. (2) These findings did not correlate with peak oxygen uptake during CPET, an objective parameter of cardiopulmonary exercise capacity. (3) Therefore, a conservative proceeding rather than surgery or catheter intervention should be considered, especially in asymptomatic adult patients. Acknowledgement/Funding Supported by Kinderherzen, Fördergemeinschaft Deutsche Kinderherzzentren e.V.

P4692Pronostic factors after arterial switch operation for transposition of the great arteries in children

Abstract The objective of this study was to assess long-term outcome and factors associated wih mortality in children after arterial switch operation (ASO) for transposition of the great arteries (TGA). Material and methods Retrospective analysis of all ASO performed from 2000 to 2016 in patients with TGA. Demographics, clinical and biological data, surgical techniques, echocardiographic measurements, and outcomes were collected. Pronostic factors for mortality were assessed. Results 364 patients were included (263 males). Antenatal diagnosis was made in 60,4%. Birth weight was 3207,5±511.2g, gestational age was 39.1±1.6weeks. Intramural coronary artery was present in 24 cases (6.6%). Long term follow-up was 9.2±4.8 years. Rashkind procedure was performed in 88.7% of the cases. Ventilatory support was needed in 56% and prostaglandins in 85% preoperatively. ASO occurred at the age of 7.44±6.26 days (1 to 56) and 5.6±4.6 days after admission. Bypass duration was 123.1±33.3mn. Overall and postoperative hospital stay were respectively 19.9±8.6 and 14.4±7.3 days. Survival rates were 95.3% at 1 month, 94.8% at 3, 6 and 12 months and 94.5% at 2.5 years and up to 18 years after surgery. Twenty patients died (5.5%): 17 early postoperatively (4.7%). Time from surgery to death was 64.2±207.9 days (0 to 919). Long-term events occurred in 49.6% of the patients: pulmonary stenosis, aortic or mitral regurgitation, residual shunt, coronary lesions (1.7%), arrhythmias (5.5%), cardiac failure. Freedom from late reintervention was 99.7%, 97.4%, 96.2% at 1, 3 and 6 months, 95.9%, 95%, 92.3% and 90.3% at respectively 1, 2, 5 and beyond 8 years after ASO. No Rashkind, postoperative ECMO support, delayed chest closure, bypass duration, troponine level and small birth weight were significant predictive factors of mortality (respectively p=0.006, p<0.0001, p=0.0007, p<0.0001, p=0.001 and p=0.044). Antenatal diagnosis, gender, gestational age, preoperative ventilatory support, prostaglandin infusion, and SpO2, coronary arteries anatomy or age at surgery were not associated with death. Conclusion This study showed that Rashkind procedure may have a favourable impact on prognosis of neonates with TGA. Postoperative ECMO support and troponine level, and small birth weight were associated with worse outcome.

Long-Term Anatomical Outcomes of the Arterial Switch Operation for Transposition of the Great Arteries Characterised with Cardiac Magnetic Resonance Imaging

Long-Term Anatomical Outcomes of the Arterial Switch Operation for Transposition of the Great Arteries Characterised with Cardiac Magnetic Resonance Imaging

P739Cardiac Magnetic Resonance at rest and under dobutamine stress in young adults after arterial switch operation for transposition of the great arteries

P739Cardiac Magnetic Resonance at rest and under dobutamine stress in young adults after arterial switch operation for transposition of the great arteries

Fate of Neoaortic Valve in Patients undergoing Arterial Switch Operation for Transposition of Great Arteries

Fate of Neoaortic Valve in Patients undergoing Arterial Switch Operation for Transposition of Great Arteries

Успешная операция артериального переключения при транспозиции магистральных артерий с отхождением коронарных артерий единым устьем

Успешная операция артериального переключения при транспозиции магистральных артерий с отхождением коронарных артерий единым устьем

Towards standardization: Analysis of specific modifiable perioperative process measures among neonates undergoing arterial switch operation for transposition of the great arteries with intact ventricular septum – A mini-pilot for the California Congenital Cardiac Consortium

Towards standardization: Analysis of specific modifiable perioperative process measures among neonates undergoing arterial switch operation for transposition of the great arteries with intact ventricular septum – A mini-pilot for the California Congenital Cardiac Consortium

Noninvasive Assessment of Autonomic Cardiovascular Function in Patients after Arterial Switch Operation for Transposition of the Great Arteries

Background: Children born with transposition of the great arteries (TGA) must undergo corrective surgery for survival, arterial switch being the standard surgical procedure. The sympathetic in- nervation of the heart may be damaged during the operation. This study was designed to deter- mine whether adults who were born with TGA and who had arterial switch operation (ASO) in in- fancy exhibit denervation of the heart, measured as heart rate variability (HRV) with electrocar- diography (ECG). Methods: Nine patients with transposition of the great arteries (four men and five women; mean age 26 ± 1 years) who underwent the ASO at a mean age of 85 ± 35 days, and nine healthy adults (five men and five women; mean age 26 ± 2) were included in the study. Car- diac autonomic nerve function was determined by the variation in RR intervals during maximal deep breathing, monitored by continuous ECG. The mean values were calculated for each group from six inspirations (I) and expirations (E), and the E:I ratios were calculated. Results: The E:I ra- tio did not differ between patients with an arterial switch and healthy controls (P = 0.161). Two patients had signs of denervation of the heart up to 30 years after the arterial switch operation. Conclusions: Reinnervation of the heart may take place in patients who have undergone the ASO in infancy, and these patients would not necessarily suffer from autonomic dysfunction. The HRV, measured by ECG, has the potential to identify arterially switched patients at risk of developing silent myocardial ischemia. * Corresponding author.

Late Arterial Switch Operation for Transposition With Intact Septum

Late Arterial Switch Operation for Transposition With Intact Septum

Left Main Coronary Artery Stenting in a Neonate After Arterial Switch Operation for Transposition of the Great Arteries

Anatomical correction of transposition of the great arteries (TGA) with an intramural coronary artery is associated with high risk of coronary complications such as vessel injury and stenosis. Here, we report on a case of left main coronary artery stenting in a neonate with single coronary artery after repair for TGA.

Late coronary insufficiency after arterial switch operation for transposition of the great arteries

Background: Arterial switch operation (ASO) is the current treatment of choice for neonates with transposition of the great arteries. Operative morbidity and mortality are currently very low, with favorable early and mid-term clinical results. Long-term studies are still scarce, as most patients are only now reaching late adolescence and adulthood, but suggest that patency and function of the re-implanted coronary arteries may be a concern. Methods and results: Between 1994 and 2011, 103 consecutive patients who underwent neonatal ASO were routinely studied with single-photon emission computed tomography (SPECT) and cardiac catheterization with angiography; we identified two patients who presented with late coronary insufficiency at the age of 16. Both had Yacoub type A native coronary anatomy and an uneventful ASO, with routine SPECT and angiography study at the age of two showing normal post-operative coronary patency and function. One patient complained of chest discomfort with exertion while the other remained asymptomatic, with electrocardiographic baseline ST-T non-specific changes. The prompted investigation showed a positive exercise test for coronary ischemia and SPECT revealed moderate-to-severe anterior and anteroseptal left ventricular wall ischemia; further investigation with cardiac catheterization and multislice computed tomography with 3D reconstruction showed severe kinking at the ostium of the left main coronary artery. In the first patient an internal thoracic artery to coronary artery bypass was performed while in the other, due to the presence of an intramural coronary segment, a proximal coronary patch angioplasty was performed. Both surgeries and post-operative periods were uneventful and at 6-months follow-up both remain asymptomatic. Conclusion: Coronary lesions after ASO have an estimated incidence of 3-7% with most cases reported between the ages of 2 and 5, and have been related to complex native coronary anatomy (Yacoub type B – E). Nevertheless, late coronary insufficiency may develop in patients with Yacoub type A native anatomy in whom good post-operative results had been previously confirmed with functional and invasive anatomic studies and without associated symptoms, rendering routine and lifelong clinical evaluation and exercise or pharmacologic stress testing, with invasive angiography when needed, an essential component of the follow-up of these patients. Further studies are required to fully characterize the incidence of late coronary insufficiency after ASO.

Early Childhood Language Outcomes after Neonatal Arterial SwitcH Operation for Transposition of the Great Arteries

Early Childhood Language Outcomes after Neonatal Arterial SwitcH Operation for Transposition of the Great Arteries

Left ventricular torsion in patients after the arterial switch operation for transposition of the great arteries with intact ventricular septum

Left ventricular torsion in patients after the arterial switch operation for transposition of the great arteries with intact ventricular septum

Managing the posterior coronary loop in the arterial switch operation: the ‘inverse flap’ technique

Posterior coronary looping remains a risk factor when performing an arterial switch operation for transposition of the great arteries. In such a situation, to avoid the risk of overstretching, we use an 'inverse' flap technique, resulting in a tension-free relocation.