First report from the European registry for anomalous aortic origin of coronary artery (EURO-AAOCA).

OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) is a group of rare congenital heart defects with various clinical presentations. The lifetime-risk of an individual living with AAOCA is unknown, and data from multicentre registries are urgently needed to adapt current recommendations and guide optimal patient management. The European AAOCA Registry (EURO-AAOCA) aims to assess differences with regard to AAOCA management between centres. METHODS: EURO-AAOCA is a prospective, multicentre registry including 13 European centres. Herein, we evaluated differences in clinical presentations and management, treatment decisions and surgical outcomes across centres from January 2019 to June 2023. RESULTS: A total of 262 AAOCA patients were included, with a median age of 33 years (12-53) with a bimodal distribution. One hundred thirty-nine (53.1%) were symptomatic, whereas chest pain (n = 74, 53.2%) was the most common complaint, followed by syncope (n = 21, 15.1%). Seven (5%) patients presented with a myocardial infarction, 2 (1.4%) with aborted sudden cardiac death. Right-AAOCA was most frequent (150, 57.5%), followed by left-AAOCA in 51 (19.5%), and circumflex AAOCA in 20 (7.7%). There were significant differences regarding diagnostics between age groups and across centres. Seventy-four (28.2%) patients underwent surgery with no operative deaths; minor postoperative complications occurred in 10 (3.8%) cases. CONCLUSIONS: Currently, no uniform agreement exists among European centres with regard to diagnostic protocols and clinical management for AAOCA variants. Although surgery is a safe procedure in AAOCA, future longitudinal outcome data will hopefully shed light on how to best decide towards optimal selection of patients undergoing revascularization versus conservative treatment.

Automatic 3D Segmentation and Identification of Anomalous Aortic Origin of the Coronary Arteries Combining Multi-view 2D Convolutional Neural Networks.

This work aimed to automatically segment and classify the coronary arteries with either normal or anomalous origin from the aorta (AAOCA) using convolutional neural networks (CNNs), seeking to enhance and fasten clinician diagnosis. We implemented three single-view 2D Attention U-Nets with 3D view integration and trained them to automatically segment the aortic root and coronary arteries of 124 computed tomography angiographies (CTAs), with normal coronaries or AAOCA. Furthermore, we automatically classified the segmented geometries as normal or AAOCA using a decision tree model. For CTAs in the test set (n = 13), we obtained median Dice score coefficients of 0.95 and 0.84 for the aortic root and the coronary arteries, respectively. Moreover, the classification between normal and AAOCA showed excellent performance with accuracy, precision, and recall all equal to 1 in the test set. We developed a deep learning-based method to automatically segment and classify normal coronary and AAOCA. Our results represent a step towards an automatic screening and risk profiling of patients with AAOCA, based on CTA.

Unusual Aneurysm of a Cervical Aortic Arch: Surgical Repair Improves Fluid Dynamics.

The cervical aortic arch is a rare congenital vascular abnormality related to the anomalous development of the aortic arch. We present the case of a 6-year-old patient with a large aneurysmal cervical aortic arch who underwent surgical correction and arch reconstruction. Surgical repair was indicated based on the risk of progressive dilation and rupture, aiming to restore correct geometry and hemodynamics. We evaluated preoperative and postoperative hemodynamics using computational fluid dynamics simulations, and we also identified, within the repaired region, an area that remains affected by greater turbulent flow, requiring follow-up surveillance.

Minimally Invasive Surgery for Simple Congenital Heart Defects: Preserving Aesthetics without Jeopardizing Patient Safety.

Minimally invasive surgeries for pediatric patients have been proposed for decades, with different approaches in mind. Minimal right axillary thoracotomy (MRAT), proposed two decades ago, allows the preservation of patients' safety alongside faster aesthetic and functional recovery. The MRAT did not become widely adopted due to the prejudice that to follow a minimally invasive approach, safety and efficacy must be compromised. With this study, we aim to compare MRAT to the standard median sternotomy approach with a focus on safety and clinical outcomes. Between January 2017 and April 2021, 216 patients diagnosed with ASD, pAVSD, or PAPVD underwent surgical repair with different approaches in the same period. MRAT was used for 78 patients, and median sternotomy was used for 138 patients. In this last group, standard median sternotomy (SMS) was used for 116 patients, while a minimal skin incision (SMS mini) was used for 22 patients. There were no major complications overall nor in each specific approach. MRAT enabled the successful repair of simple heart defects, providing similar post-operative and cardiological recovery. MRAT does not compromise patients' safety and does not prolong the duration of surgery once the learning curve is overcome, which is generally after 15-20 consecutive operations.

Lumped-parameter model as a non-invasive tool to assess coronary blood flow in AAOCA patients.

Anomalous aortic origin of the coronary artery (AAOCA) is a rare disease associated with sudden cardiac death, usually related to physical effort in young people. Clinical routine tests fail to assess the ischemic risk, calling for novel diagnostic approaches. To this aim, some recent studies propose to assess the coronary blood flow (CBF) in AAOCA by computational simulations but they are limited by the use of data from literature retrieved from normal subjects. To overcome this limitation and obtain a reliable assessment of CBF, we developed a fully patient-specific lumped parameter model based on clinical imaging and in-vivo data retrieved during invasive coronary functional assessment of subjects with AAOCA. In such a way, we can estimate the CBF replicating the two hemodynamic conditions in-vivo analyzed. The model can mimic the effective coronary behavior with high accuracy and could be a valuable tool to quantify CBF in AAOCA. It represents the first step required to move toward a future clinical application with the aim of improving patient care. The study was registered at Clinicaltrial.gov with (ID: NCT05159791, date 2021-12-16).

Cervical aortic arch in the pediatric population: a meta-analysis of individual patient's data.

Background: This is the first meta-analysis to analyze all reports of published pediatric cases of cervical aortic arch (CAA) by highlighting the clinical characteristics and treatment outcomes using the reported individual data of the patients. The aim of the study is to investigate the clinical features and surgical outcomes of such a rare disease in the pediatric population. Methods: A comprehensive search was conducted in various academic databases, including PubMed, ScienceDirect, SciELO, DOAJ, and Cochrane Library, until June 2022 for case reports describing the presence of cervical aortic arch in the pediatric age. Case reports and series were included if the following criteria were met: (1) description of the cervical aortic arch; (2) patient of pediatric age; and (3) published in the English language. All other types of publications that lacked patient-specific information were excluded from the analysis. This systematic review was conducted in accordance with the PRISMA guidelines. The primary outcome measure of the analysis was early and late mortality. Results: The literature search identified 2,272 potentially eligible articles, 72 of which met our inclusion criteria with 96 patients including the author's institutional case. At a median of 365 (90-730) days, the overall cohort registered a 7.3% (7/96) mortality rate. In the subset of patients who underwent surgery, the mortality rate was also 7.3% (4/55), and the mortality rate following surgery to treat only CAA was 2.4% (1/42). Dyspnea was identified as an independent determinant of mortality by employing the univariable Firth bias-reduced logistic regression method. Conclusion: Cervical aortic arch is a rare congenital heart disease that poses treatment challenges due to the high anatomical variability, diverse clinical presentations, and the presence of other concomitant diseases. The surgical treatment appears to be a safe and effective approach for resolving the symptoms, although it needs to be tailored individually for each patient. Systematic Review Registration: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=346826, Identifier: CRD42022346826.

Morphological Changes of Anomalous Coronary Arteries From the Aorta During the Cardiac Cycle Assessed by IVUS in Resting Conditions.

BACKGROUND: Anomalous aortic origin of coronary artery (AAOCA) with intramural segment is associated with risk of sudden cardiac death, probably related to a compressive mechanism exerted by the aorta. However, the intramural compression occurrence and magnitude during the cardiac cycle remain unknown. We hypothesized that (1) in end diastole, the intramural segment is narrower, more elliptic, and has greater resistance than extramural segment; (2) the intramural segment experiences a further compression in systole; and (3) morphometry and its systolic changes vary within different lumen cross-sections of the intramural segment. METHODS: Phasic changes of lumen cross-sectional coronary area, roundness (minimum/maximum lumen diameter), and hemodynamic resistance (Poiseuille law for noncircular sections) were derived from intravascular ultrasound pullbacks at rest for the ostial, distal intramural, and extramural segments. Data were obtained for 35 AAOCA (n=23 with intramural tract) after retrospective image-based gating and manual lumen segmentation. Differences between systolic and end-diastolic phases in each section, between sections of the same coronary, and between AAOCA with and without intramural tract were assessed by nonparametric statistical tests. RESULTS: In end diastole, both the ostial and distal intramural sections were more elliptical (P<0.001) than the reference extramural section and the correspondent sections in AAOCA without intramural segment. In systole, AAOCA with intramural segment showed a flattening at the ostium (-6.76% [10.82%]; P=0.024) and a flattening (-5.36% [16.56%]; P=0.011), a narrowing (-4.62% [11.38%]; P=0.020), and a resistance increase (15.61% [30.07%]; P=0.012) at the distal intramural section. No-intramural sections did not show morphological changes during the entire cardiac cycle. CONCLUSIONS: AAOCA with intramural segment has pathological segment-specific dynamic compression mainly in the systole under resting conditions. Studying AAOCA behavior with intravascular ultrasound during the cardiac cycle may help to evaluate and quantify the severity of the narrowing.

Ross Operation in Pediatric Population: Impact of the Surgical Timing and the Native Pulmonary Diameter on the Outcome.

Aortic valve replacement early in life may be inevitable. Ross operation, until present day, remains the favorite surgical option in pediatrics with irreparable aortic valve disease. Nonetheless, the necessity for re-operation was always its principal limitation due to aortic valve failure or homograft degeneration. We present our 25 years of experience in the pediatric population. From August 1994 until June 2018, 157 children below 18 years underwent the Ross operation. This retrospective review aims at assessing the long-term outcomes, as well as the risk factors for re-operation after Ross procedure. Median age was 10.9 years, of which seven patients were infants, 79 children, and 71 adolescents. The median follow-up time was 14 years. Hospital mortality was 0.6%. Freedom from autograft re-operation for children was 96.7% and 94.1% at 10 and 20 years, respectively; whereas for adolescents, it was 92.6% and 74.9% at 10 and 20 years. For children, freedom from homograft re-operation was 92.5%, 83.5%, and 56.2% at 10, 15, and 20 years; while for adolescents, it was 96.8%, 91.8%, and 86.7% at 10, 15, and 20 years. Homograft size (p = 0.008) and childhood (p = 0.05) were risk factors for homograft re-operation. Pulmonary valve diameter > 24 mm (p = 0.044) and adolescence (p = 0.032) were risk factors for autograft re-operation. Our experience demonstrated excellent early and late survival. While children have preferential outcomes concerning autograft re-operation, those who received a smaller homograft had a higher right-sided re-intervention incidence than adolescents. Pulmonary diameter > 24 mm at surgery was an indicator of future autograft failure.

Myocardial infarction with non-obstructive disease and anomalous coronary origin: look for the common in the uncommon.

Management of congenital coronary artery anomalies (CAA) is not standardized due to the variety of conditions included and their rare prevalence. Detection of CAA during myocardial infarction with non-obstructive coronary arteries (MINOCA) may induce clinicians to address the patient for surgery as CAA is not included in any algorithm1,2 for the management of MINOCA and American Association for Thoracic Surgery evidence-based guidelines suggest surgical repair for patients with anomalous aortic origin of a coronary artery and symptoms compatible with myocardial ischaemia.3 We present the case of a 35-year-old man with an anomalous origin of left coronary artery from right Valsalva sinus with pre-pulmonic course detected during urgent coronary angiography for suspected myocardial infarction. Stress cardiac magnetic resonance did not show signs of ischaemia at high-dose dobutamine but did reveal a recent myocarditis. This clinical case highlights the need for accurate risk stratification in CAA especially when confounding clinical scenarios co-exist.

Primary Arterial Switch Operation for Late Presentation of Transposition of the Great Arteries With Intact Ventricular Septum.

Transposition of the great arteries (TGA) with intact ventricular septum (IVS) are subject to neonatal arterial switch operation (ASO) to avoid deconditioning of the left ventricle (LV). Often an early repair is not feasible in developing countries where late referral is frequent. We aim to explore ASO outcomes in late TGA-IVS and compare "unfavorable candidates" (LV myocardial mass <35gr/m2 unfavorable geometry. banana-like LV shape) with other late TGA-IVS. Single-center retrospective study on late TGA-IVS who underwent primary ASO between 2015 and 2018. We divide patients into 2 groups: unfavorable candidates and favorable candidates TGA. We report categorical variables as number and percentage and continuous variables as median with interquartile range. Differences were assessed with Chi-squared or Fisher exact tests, Wilcoxon sign-rank, and Wilcoxon-rank sum tests. 45 TGA-IVS were referred with a median age of 35 days. Pre-operative echocardiography identified banana-like LV shape in 66.7%; unfavorable LV geometry in 47.6% and LV mass <35gr/m2 in 51%. Only 1 death occurred related to myocardial ischemia. Five patients (11%) required ECMO for LV dysfunction, with unfavorable candidates having a higher but not significant use (18.7% vs 6.9%, P = 0.33). At discharge, echocardiography demonstrated significant LV mass improvement compared to pre-operative (58.6 vs 33.8 gr/m2, P < 0.0001) with no significant echocardiographic difference between unfavorable and favorable late-TGA. Primary ASO in late presenter TGA-IVS can be performed safely with low mortality having a low threshold to ECMO. Significantly unconditioned LV are amenable with primary ASO with good LV mass recovery.

Tricuspid Valve Intervention at the Time of Pulmonary Valve Replacement in Adults With Congenital Heart Disease: A Systematic Review and Meta-Analysis.

Background Tricuspid regurgitation (TR) is a common finding in adults with congenital heart disease referred for pulmonary valve replacement (PVR). However, indications for combined valve surgery remain controversial. This study aimed to evaluate early results of concomitant tricuspid valve intervention (TVI) at the time of PVR. Methods and Results Observational studies comparing TVI+PVR and isolated PVR were identified by a systematic search of published research. Random-effects meta-analysis was performed, comparing outcomes between the 2 groups. Six studies involving 749 patients (TVI+PVR, 278 patients; PVR, 471 patients) met the eligibility criteria. In the pooled analysis, both TVI+PVR and PVR reduced TR grade, pulmonary regurgitation grade, right ventricular end-diastolic volume, and right ventricular end-systolic volumes. TVI+PVR, but not PVR, was associated with a decrease in tricuspid valve annulus size (mean difference, -6.43 mm, 95% CI, -10.59 to -2.27; P=0.010). Furthermore, TVI+PVR was associated with a larger reduction in TR grade compared with PVR (mean difference, -0.40; 95% CI, -0.75 to -0.05; P=0.031). No evidence could be established for an effect of either treatment on right ventricular ejection fraction or echocardiographic assessment of right ventricular dilatation and dysfunction. There was no evidence for a difference in hospital mortality or reoperation for TR. Conclusions While both strategies are effective in reducing TR and right ventricular volumes, routine TVI+PVR can reduce TR grade to a larger extent than isolated PVR. Further studies are needed to identify the subgroups of patients who might benefit most from combined valve surgery.

Study design and rationale of the pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA-R).

AIM: Due to improved therapy in childhood, many patients with congenital heart disease reach adulthood and are termed adults with congenital heart disease (ACHD). ACHD often develop heart failure (HF) as a consequence of initial palliative surgery or complex anatomy and subsequently require advanced HF therapy. ACHD are usually excluded from trials evaluating heart failure therapies, and in this context, more data about heart failure trajectories in ACHD are needed to guide the management of ACHD suffering from HF. METHODS AND RESULTS: The pAtients pResenTing with cOngenital heaRt dIseAse Register (ARTORIA-R) will collect data from ACHD evaluated or listed for heart or heart-combined organ transplantation from 16 countries in Europe and the Asia/Pacific region. We plan retrospective collection of data from 1989-2020 and will include patients prospectively. Additional organizations and hospitals in charge of transplantation of ACHD will be asked in the future to contribute data to the register. The primary outcome is the combined endpoint of delisting due to clinical worsening or death on the waiting list. The secondary outcome is delisting due to clinical improvement while on the waiting list. All-cause mortality following transplantation will also be assessed. The data will be entered into an electronic database with access to the investigators participating in the register. All variables of the register reflect key components important for listing of the patients or assessing current HF treatment. CONCLUSION: The ARTORIA-R will provide robust information on current management and outcomes of adults with congenital heart disease suffering from advanced heart failure.