Background:Partial cor triatriatum sinistrum is a rare congenital heart disease and is usually considered for surgery in symptomatic patients. We describe three cases of partial cor triatriatum sinistrum, two cases of successful percutaneous balloon dilatation, and one case of conservative treatment in a patient without symptoms. Case summary:The first case describes a female patient with progressive dyspnoea on exertion in which the membrane was dilated with a balloon to relieve symptoms. The second case is about a young patient with chronic congestion of the right lung because of a partial cor triatriatum sinistrum which was also treated with dilatation. The third case describes a male patient with an incidental finding of a cor triatriatum sinistrum who did not experience any symptoms and was treated conservatively. Discussion:Percutaneous balloon dilatation is a safe and effective alternative to surgery in selected cases of (partial) cor triatriatum.

OBJECTIVES:In selected patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO), the arterial switch operation (ASO) may be the procedure of choice. This study reviews the clinical outcomes of TGA-VSD-LVOTO patients after ASO and compares mechanisms of LVOTO in this patient group to a historical series of cardiac specimens. METHODS:This retrospective analysis included all cases with TGA-VSD-LVOTO who underwent ASO between January 1977 and December 2023. Additionally, a series of non-operated cardiac specimens with TGA-VSD-LVOTO was selected and examined for morphological comparison. RESULTS:Eleven patients with TGA-VSD-LVOTO underwent ASO. Eight of them had TGA-VSD, and three had Taussig-Bing anomaly. LVOTO mechanisms were multifactorial, including posteriorly deviated infundibular septum and fibrous tissue masses. Median age at ASO was 0.4 (0.07-1.8) years. Ten patients underwent primary LVOTO relief during ASO; no in-hospital mortality occurred. Two patients died >30 days post-ASO at 3.1 months and 6.0 years. Median follow-up was 19.0 (11.1-26.8) years, all survivors in NYHA class I. The patient without initial LVOTO relief did require reoperation during follow-up for progressive LVOTO at 3.5 months post-ASO. Two patients had moderate residual LVOTO at latest follow-up (gradient 30-50 mmHg). No significant neoaortic valve regurgitation was observed. From the anatomical specimen series, 10 of 33 TGA-VSD-LVOTO specimen were deemed eligible for ASO, revealing similar LVOTO mechanisms as the clinical cases. CONCLUSIONS:ASO is feasible in selected patients with TGA-VSD-LVOTO showing good long-term outcomes with preserved neoaortic valve function and no reoperations for LVOTO after initial relief.

Abstract Introduction Diagnostic work-up of patients with an anomalous aortic origin of a coronary artery (AAOCA) includes an anatomical and functional assessment. The approach to functional evaluation currently varies widely and mainly includes noninvasive assessment. Intracoronary hemodynamic assessment can potentially enhance risk assessment for myocardial ischemia and sudden cardiac death and guide management of AAOCA patients. Hence, this study aims to evaluate invasive detection of ischemia on top of the standard of care diagnostic work-up for interarterial or intraseptal AAOCA and the impact on patient management. We report the first clinical outcomes. Methods In this multicenter prospective cohort study on AAOCA, all consecutive patients of 16 years and older with AAOCA with a newly diagnosed interarterial or intraseptal course in whom diagnostic work-up according to the protocol of the MuSCAT trial was performed between January 2021 and January 2024, were included for analysis. Exclusion comprised incomplete diagnostic evaluation, hemodynamically significant concomitant congenital heart disease or obstructive coronary artery disease in the AAOCA effluence. The work-up included invasive ischemia testing using fractional flow reserve (FFR) and or instantaneous wave-free ratio (iFR)/resting full-cycle ratio (RFR) at baseline and during pharmacological stress using adenosine, adrenaline and dobutamine. Results Fifty-eight patients (50% female, median age at AAOCA diagnosis 51.5 years (IQR 44.0-59.0)) were included, Table 1. The right coronary artery was anomalous in 88%, 93% patients had an interarterial and 69% an intramural course. Non-invasive ischemia detection was positive in 12%, negative in 84% and inconclusive in 4%. Invasive hemodynamic assessment was positive for ischemia in 22%, negative in 74% and inconclusive in 3%. Class of recommendation for surgery based on ESC guidelines was I or IIa in 28% patients and IIb, III or unclassified in 72%. Invasive hemodynamic assessment changed the treatment decision in 24% of the patients; 50% with initially recommended surgical treatment and 14% with initially a conservative treatment adjudication, Figure 1. Median follow-up after initial diagnosis was 20 [IQR 10-28] months and in 56% of all patients who completed 6 months follow-up the initial symptoms alleviated. One (2%) patient underwent a percutaneous re-intervention of the operated AAOCA. Conclusions This national prospective study of invasive hemodynamic assessment in patients with AAOCA showed clinically significant impact on the adjustment of initial management strategy in 24% of the patients. No cardiac death of myocardial infarction in the AAOCA supply area occurred during follow-up. Analysis of the complete follow-up of the MuSCAT trial can further substantiate the role of additional invasive functional imaging in AAOCA.

Abstract Background In anomalous aortic origin of a coronary artery (AAOCA) an intramural segment is present if the proximal AAOCA courses through the aortic wall. This is defined as a shared tunica media of the aorta and AAOCA in the ‘intramural septum’. This definition is based on autopsy studies of selected AAOCA patients who suffered sudden cardiac death and is difficult to apprehend on imaging modalities. Therefore, the aim was to identify the histological features and variants of the interarterial vascular wall in living adult patients, and correlate this to CT-angiography (CTA). Methods This prospective multicenter study included consecutive adult AAOCA patients who underwent surgical unroofing between 2021 to 2024. The excised interatrial vascular wall tissue was embedded, sectioned, and immunohistochemically stained for physiological components, e.g. smooth muscle cells and connective tissue, and pathological components such as fibrosis. Microscopic examination and quantification was performed by two independent observers and findings were correlated with CTA. Results Fifteen patients (mean age 42.9±14.0 years, 60% female) and 1 postmortem specimen were included. Fourteen (93%) had a right-AAOCA and 1 (7%) a left-AAOCA, with a mean intramural length of 8.4±4.1mm. Histopathologically, disorganized elastic lamellae with fragmentation, presence of fibrosis and loss of nuclei were observed. No vasa vasorum or nervi vasorum were identified. Post-sectioning virtual 3D reconstructions enhanced the spatial insight in correlation of histological sections with CTA. Conclusion This first study of the intramural septum in adults with AAOCA reveals the histological structure of the shared vascular wall between the aorta and AAOCA. Marked vascular wall abnormalities such as lack of vasa and nervi vasorum, fragmentation of elastic lamellae and fibrotic changes were observed. These findings support a propensity to local vascular wall pathology in patients with AAOCA. 3D reconstruction supported the translation of histological data to clinical imaging techniques.Central Figure

Introduction: Increasing survival of adult congenital heart disease (ACHD) patients comes at the price of a range of late complications—arrhythmias, heart failure, and valvular dysfunction. Transcatheter valve interventions have become a legitimate alternative to conventional surgical treatment in selected acquired heart disease patients. However, literature on technical aspects, hemodynamic effects, and clinical outcomes of percutaneous atrioventricular (AV) valve interventions in ACHD patients is scarce. Method: This is a descriptive cohort from CAHAL (Center of Congenital Heart Disease Amsterdam-Leiden). ACHD patients with severe AV valve regurgitation who underwent a transcatheter intervention in the period 2020–2022 were included. Demographic, clinical, procedural, and follow-up data were collected from patient records. Results: Five ACHD patients with severe or torrential AV valve regurgitation are described. Two patients underwent a transcatheter edge-to-edge repair (TEER), one patient underwent a valve-in-valve procedure, one patient received a Cardioband system, and one patient received both a Cardioband system and TEER. No periprocedural complications occurred. Post-procedural AV valve regurgitation as well as NYHA functional class improved in all patients. The median post-procedural NYHA functional class improved from 3.0 (IQR [2.5–4.0]) to 2.0 (IQR [1.5–2.5]). One patient died 9 months after the procedure due to advanced heart failure with multiorgan dysfunction. Conclusion: Transcatheter valve repair is feasible and safe in selected complex ACHD patients. A dedicated heart team is essential for determining an individualized treatment strategy as well as pre- and periprocedural imaging to address the underlying mechanism(s) of AV regurgitation and guide the transcatheter intervention. Long-term follow-up is essential to evaluate the clinical outcomes of transcatheter AV valve repair in ACHD patients.

Quantitative extracellular volume fraction (ECV) mapping with MRI is commonly used to investigate in vivo diffuse myocardial fibrosis. This study aimed to validate ECV measurements against ex vivo histology of myocardial tissue samples from patients with aortic valve stenosis or hypertrophic cardiomyopathy. Sixteen patients underwent MRI examination at 3 T to acquire native T1 maps and post-contrast T1 maps after gadobutrol administration, from which hematocrit-corrected ECV maps were estimated. Intra-operatively obtained myocardial tissue samples from the same patients were stained with picrosirius red for quantitative histology of myocardial interstitial fibrosis. Correlations between in vivo ECV and ex vivo myocardial collagen content were evaluated with regression analyses. Septal ECV was 30.3

OBJECTIVES:To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch (RAA). METHODS:Fetuses diagnosed with isolated RAA, defined as the absence of intra- or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR. RESULTS:In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false-positive; therefore, the follow-up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17 months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor. CONCLUSIONS:Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.

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

Marfan syndrome (MFS) is a connective tissue disorder causing aortic aneurysm formation. Currently, only prophylactic aortic surgery and blood pressure-lowering drugs are available to reduce the risk of aortic rupture. Upon whole genome sequencing of a Marfan family, we identified a complement gene C1R variant (p.Ser152Leu), which is associated with severe aortic patients. Therefore, we assessed the role of complement activation in MFS aortic tissue. Expression of various complement genes and proteins was detected in human and murine MFS aneurysm tissue, which prompted us to study complement inhibition in MFS mice. Treatment of the Fbn1C1041G/+ MFS mice with human plasma-derived C1-esterase inhibitor Cetor® resulted in reduced complement deposition, decreased macrophage influx in the aorta, and lower circulating TNFα levels. However, in line with previous anti-inflammatory treatments, complement inhibition did not change the aortic dilatation rate in this MFS mouse model. Thus, while complement factors/component 3 activation were detected in human/murine MFS aorta, Cetor® had no effect on aortic dilatation in MFS mice, indicating that complement inhibition is not a suitable treatment strategy in MFS.

Building on the experience of long-term mechanical circulatory support in systemic left ventricles with acquired heart disease, ventricular assist device (VAD) therapy for the failing systemic right ventricle has been explored over the past 2 decades by several groups [1, 2]. However, the number of patients treated in this way is still limited, around 50 reported in the literature, and patient selection and fighting the burden of complications related to VAD therapy remain a considerable challenge. Gonzalez-Fernandez et al. [3] should be congratulated with their more than 10 years’ experience with VAD therapy in patients with atrial switch correction for transposition of the great arteries and congenitally corrected transposition of the great arteries. Their series of 18 patients can be confirmed to be the largest reported so far. Although not fully understood, they observed a high incidence of subpulmonary left ventricular failure, which also seemed to be associated with adverse outcomes. Of 7 patients developing subpulmonary LV failure on support, only 2 were transplanted just in time, whereas the other 5 died while being on mechanical support, thereby abrogating the purpose of VAD therapy as a bridge to candidacy or bridge to transplant.

BackgroundPatients with transposition of the great arteries (TGA) after an atrial switch or congenitally corrected TGA (ccTGA) are prone to systemic right ventricular (sRV) failure. Tricuspid valve (TV) regurgitation aggravates sRV dysfunction. Timely TV surgery stabilizes sRV function, yet the development of atrioventricular (AV)-conduction disturbances in the course of sRV failure can contribute to sRV dysfunction through pacing-induced dyssynchrony. This study aims to explore the incidence, timing, and functional consequences of AV-block requiring ventricular pacing after TV surgery in patients with sRV.MethodsConsecutive adolescent and adult patients with an sRV who underwent TV surgery between 1989 and 2020 and followed-up at our center were included in this observational cohort study.ResultsThe data of 28 patients (53% female, 57% ccTGA, and a mean age at surgery 38 ± 13 years) were analyzed. The mean follow-up was 9.7 ± 6.8 years. Of the remaining 22 patients at the risk of developing high degree AV-block after TV surgery, 9 (41%) developed an indication for chronic ventricular pacing during follow-up, of which 5 (56%) within 24 months postoperatively (3 prior to hospital discharge). The QRS duration, a surrogate marker for dyssynchrony, was significantly higher in patients with chronic left ventricular pacing than in patients with native AV-conduction (217 ± 24 vs. 116 ± 23 ms, p = 0.000), as was the heart failure biomarker NT-pro-BNP [2,746 (1,242–6,879) vs. 495 (355–690) ng/L, p = 0.004] and the percentage of patients with ≥1 echocardiographic class of deterioration of systolic sRV function (27 vs. 83%, p = 0.001). Of the patients receiving chronic subpulmonary ventricular pacing (n = 12), 9 (75%) reached the composite endpoint of progressive heart failure [death, ventricular assist device implantation, or upgrade to cardiac resynchronization therapy (CRT)]. Only 4 (31%) patients with native AV-conduction (n = 13) reached this composite endpoint (p = 0.027).ConclusionPatients with a failing sRV who undergo TV surgery are prone to develop AV-conduction abnormalities, with 41% developing an indication for chronic ventricular pacing during 10 years of follow-up. Patients with chronic subpulmonary ventricular pacing have a significantly longer QRS complex duration, have higher levels of the heart failure biomarker NT-pro-BNP, and are at a higher risk of deterioration of systolic sRV function and progressive heart failure.

Abstract Funding Acknowledgements Type of funding sources: None. Background Patients with transposition of the great arteries(TGA) after atrial switch or congenitally corrected TGA(ccTGA) are prone to systemic right ventricular(sRV) failure. Atrioventricular(AV)-conduction disturbances requiring chronic ventricular pacing and tricuspid valve(TV) regurgitation aggravate sRV dysfunction. Timely TV surgery stabilizes sRV function, yet is a risk factor for AV-block, potentially contributing to sRV failure due to pacing-induced dyssynchrony. The aim of this study is to explore the incidence, timing and functional consequences of AV-block requiring ventricular pacing after TV surgery in sRV patients. Methods Consecutive adolescent and adult patients with a sRV who underwent TV surgery in the period 1989-2020 and follow-up at our centre were included in this observational cohort study. Demographic and clinical data was collected from patient records. Results Data of 28 patients(54% female, 57% ccTGA, mean age at surgery 38±13 years) was analysed. Mean follow-up duration was 9.7±6.8 years. Five patients(18%) already had chronic(>40%) subpulmonary left ventricular pacing preoperatively, of which 2 received cardiac resynchronization therapy(CRT) upgrade prior to surgery. One patient received CRT during TV surgery. Of the remaining 22 patients at risk for AV-block after surgery, 9(41%) developed an indication for chronic pacing during follow-up, of which 3(33%) before hospital discharge and a total of 5(56%) within 24 months postoperatively, Figure 1. Five(20%) patients received CRT during follow-up due to progressive heart failure(HF). In one patient with transvenous upgrade, effective resynchronization was not attained due to suboptimal lead position. Of the patients receiving chronic pacing, 9(75%) died, underwent ventricular assist device(VAD) implantation or required CRT due to progressive HF. Only 4(31%) patients with native AV-conduction reached this composite endpoint(p=0.027). QRS duration, a surrogate marker for dyssynchrony, was significantly higher in patients with chronic pacing than with native AV-conduction(217±24 vs 116±23msec, p=0.000), as was NT-pro-BNP(2746[1242–6879] vs 495[355–690]ng/L, p=0.004) and the percentage of patients with ≥1 class of deterioration of systolic sRV function(p=0.001), Figure 2. Conclusions Patients with a failing sRV who undergo TV surgery are prone to AV-conduction abnormalities with 41% developing an indication for chronic ventricular pacing during follow-up. The patient group with chronic pacing has significantly more events of the composite endpoint of death, VAD implantation or upgrade to CRT, higher percentage of ≥1 grade deterioration of systolic sRV function and higher levels of HF biomarker NT-pro-BNP. Implantation of an epicardial sRV lead at the time of TV surgery for future CRT may be considered to attenuate the detrimental effects of subpulmonary ventricular pacing in this HF prone patient group with complex anatomy that limits transvenous possibilities.

Abstract Background/Introduction An anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an interarterial course can be assessed using Computed Tomography Angiography (CTA) for the presence of high-risk characteristics associated with sudden cardiac death. These features include a slit-like ostium, acute angle take-off, and degree of proximal luminal narrowing. However, no robust CTA criteria currently exist to determine the presence of an intramural segment. Purpose The aim of this study is to deduct a method to accurately identify an intramural course of interarterial ACAOS on CTA imaging. Methods All consecutive adult patients with an interarterial ACAOS that were evaluated at the two academic hospitals between January 2010 and July 2019 were screened for inclusion. Inclusion criteria were availability of a preoperative CTA-scan (0.5–1mm slice-thickness) and peroperative confirmation of the intramural segment. Using multiplanar reconstruction of the CTA, the distance between the lumen of the aorta and the lumen of the ACAOS (defined as “interluminal space” (ILS)) was assessed at 2mm intervals along the intramural segment (Figure 1). Results Twenty-five patients (64% female, mean age 46 years, 88% right ACAOS) were included. Analysis showed a mean ILS of 0.69mm±0.15mm at 2mm from the ostium. At the end of the intramural segment where the ACAOS becomes non-intramural, the mean ILS was significantly larger (1.27±0.29mm, p<0.001) (Figure 2). Interobserver agreement evaluation showed good reproducibility of ILS (intraclass correlation coefficient 0.77, p<0.001). ROC-analysis demonstrated that at a cut-off ILS of ≤0.95mm, an intramural segment can be diagnosed with 100% sensitivity and 84% specificity. Conclusion(s) The ILS is introduced as novel and robust CTA parameter to identify an intramural course of interarterial ACAOS. An ILS of ≤0.95mm is indicative of an intramural segment with 100% sensitivity and 84% specificity. Funding Acknowledgement Type of funding sources: None.

Background Anomalous coronary artery from the pulmonary artery is a rare congenital disorder with high mortality rates in infancy. Adult cases can present with life-threatening arrhythmias and sudden cardiac death. Case summary We report three cases of adults with anomalous pulmonary origin of each of the main coronary branches. The first patient with an anomalous left coronary artery from the pulmonary artery presented with an out-of-hospital cardiac arrest. The second patient with an anomalous circumflex artery from the pulmonary artery was evaluated for a bicuspid aortic valve and a suspected coronary fistula but was otherwise asymptomatic. The third patient with an anomalous right coronary artery from the pulmonary artery presented with anginal symptoms. In all cases, the diagnosis was made by cardiac computed tomography or coronary angiography. Cardiac magnetic resonance imaging was performed in all patients to guide clinical decision making on surgical or non-invasive management. All patients underwent surgical repair. In two patients, a dual coronary artery system was restored by aortic reimplantation of the anomalous coronary artery. In one patient, aortic reimplantation was unsuccessful due to poor vessel quality and the anomalous coronary artery was ligated. Clinical follow-up during 1.8-9.7 years did not show any cardiovascular complications and all patients are currently alive and asymptomatic. Discussion Anomalous coronary artery from the pulmonary artery can have various clinical presentations in adulthood. Cardiac magnetic resonance imaging is a useful modality to guide selection of patients who might have symptomatic or prognostic benefit from surgical repair.

This report describes the case of a symptomatic patient with a right coronary artery fistula draining into the coronary sinus who underwent transcatheter closure, which was deployed in the drainage site to seal off the exit of the fistula. (Level of Difficulty: Advanced.)

Background: Prolonged or excessive bleeding after cardiac surgery can lead to a broad spectrum of secondary complications. One of the underlying causes is incomplete wound drainage, with subsequent accumulation of blood and clots in the pericardium. We developed the continuous postoperative pericardial flushing (CPPF) therapy to improve wound drainage and reduce postoperative blood loss and bleeding-related complications after cardiac surgery. This study compared CPPF to standard care in patients after coronary artery bypass grafting (CABG). Methods: This is a single center, open label, randomized trial that enrolled patients at the Amsterdam UMC, location AMC, Amsterdam, the Netherlands. The study was registered at the 'Netherlands Trial Register', study identifier NTR5200 [1]. Adults undergoing CABG were randomly assigned to receive CPPF therapy or standard care, participants and investigators were not masked to group assignment. The primary end point was postoperative blood loss in the first 12-hours after surgery. Findings: Between the January 15, 2014 and the March 13, 2017, 169 patients were enrolled and assigned to CPPF therapy (study group; n = 83) or standard care (control group; n = 86). CPPF reduced postoperative blood loss when compared to standard care (median differences -385 ml, reduction 76% p=<= 0.001), with the remark that these results are overestimated due to a measurement error in part of the study group. None of patients in the study group required reoperation for non-surgical bleeding versus 3 (4%, 95% CI -0.4% to 7.0%) in the control group. None of the patients in the study group suffered from cardiac tamponade, versus 3 (4%, 95% CI -0,4% to 7.0%) in the control group. The incremental cost-effectiveness ratio was (sic)116.513 (95% bootstrap CI (sic)-882.068 to (sic)+897.278). Interpretation: The use of CPPF therapy after CABG seems to reduce bleeding and bleeding related complications. With comparable costs and no improvement in Qualty of Life (QoL), cost consideration for the implementation of CPPF is not relevant. None of the patients in the study group required re-interventions for nonsurgical bleeding or acute cardiac tamponade, which underlines the proof of concept of this novel therapy. (c) 2020 The Authors. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)

OBJECTIVES: The objective of this study was to assess our 43-year experience with arterial switch operation (ASO) for transposition of the great arteries (TGA) by analysing cardiac outcome measures (hospital and late mortality, reoperations and catheter interventions, significant coronary artery obstruction) and to identify risk factors for reoperation and catheter interventions. METHODS: A total of 490 patients who underwent ASO for TGA from 1977 to 2020 were included in this retrospective, single-centre study. Data on reoperation and catheter intervention of hospital survivors were estimated by the Kaplan-Meier method and compared using a long-rank test. Risk factors for reoperation and/or catheter intervention were assessed by multivariate Cox regression analysis. RESULTS: Hospital mortality occurred in 43 patients (8.8%), late death in 12 patients (2.9%) and 43 patients were lost to follow-up. Median follow-up time of 413 hospital survivors was 15.6 (interquartile range 7.0-22.4) years. Reoperations were performed in 83 patients (117 reoperations). Neoaortic valve regurgitation with root dilatation was the second most common indication for reoperation (15/83 patients, 18.1%) after right ventricular outflow tract obstruction (50/83 patients, 60.2%). Risk factors for any reoperation on multivariable analysis were: TGA morphological subtype [TGA with ventricular septal defect: hazard ratio (HR)=1.99, 95% confidence interval (CI) 1.18-3.36; P=0.010 and Taussig-Bing: HR=2.17, 95% CI 1.02-4.64; P=0.045], aortic arch repair associated with ASO (HR=3.03, 95% CI 1.62-5.69; P=0.001) and a non-usual coronary artery anatomy (HR=2.41, 95% CI 1.45-4.00; P=0.001). One hundred and one catheter interventions were performed in 54 patients, usually for relief of supravalvular pulmonary stenosis (44/54 patients, 81.5%) or arch obstruction (10/54 patients, 18.5%). Main risk factor for catheter intervention on multivariable analysis was aortic arch repair associated with ASO (HR=2.95, 95% CI 1.37-6.36; P=0.006). Significant coronary artery stenosis was relatively uncommon (9/413 patients, 2.2%) but may be underrepresented. CONCLUSIONS: Patients after ASO typically have good long-term clinical outcomes but reoperations and interventions remain necessary in some patients. Neoaortic valve regurgitation with root dilatation is the second most common indication for reoperation after right ventricular outflow tract obstruction and an increasing need for neoaortic valve and root redo surgery in future is to be expected.

OBJECTIVES:Tracheobronchomalacia (TBM) is characterized by collapse of trachea, bronchi or both, leading to dyspnoea, expiratory stridor, coughing or recurrent airway infections. Surgical treatment with aortopexy is warranted for severe TBM. We describe a modified aortopexy technique with aortic wall strap sutures that evenly distributes the traction force over the full width of the aortic arch. The aim of this study was to determine the outcomes of this modified anterior aortopexy technique.METHODS:Retrospective chart review of all patients undergoing aortopexy with aortic wall strap sutures for TBM between January 2010 and June 2020 in 2 tertiary hospitals in the Netherlands.RESULTS:Twenty-four patients [median age 9 months (interquartile range 2-117 months); 71% male] underwent aortopexy with the modified technique for TBM (52%), tracheomalacia (40%) or bonchomalacia (8%). Aortopexy was successful in 91.7%, defined as relief or decrease of respiratory symptoms and no need for respiratory support. Complications occurred in 8.3% and mortality was 4%.CONCLUSIONS:Aortopexy with non-absorbable strap sutures seems an effective and safe treatment for severe TBM. This study supports the hypothesis that strap sutures provide a solid and reliable traction force, but future comparative studies should confirm the benefit of strap sutures over conventional techniques.

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