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.

Regression of Cardiac Rhabdomyomas Producing a Severe Aortic Stenosis: Case Report and Discussion of the Literature.

We describe a case of a voluminous rhabdomyoma (R) detected by fetal echocardiography at 32 weeks' gestation (w.g.) obstructing the left ventricular inflow and aortic outflow tract, with a moderate aortic gradient at birth, not needing immediate surgery. At follow-up, the mass progressively regressed, leaving the aortic valve partly damaged, with a gradient that increased to a maximum of 100 mmHg at 9 years. The girl was then operated on successfully by a plasty of the aortic valve. The literature regarding R is discussed.

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.

Aortic root enlargement in patients undergoing mitral and aortic replacement: early outcomes in a sub-Saharan population.

Introduction: Aortic root enlargement (ARE) is often required to avoid patient-prosthesis mismatch (PPM) in young patients undergoing aortic surgery, including those undergoing combined mitral and aortic valve replacement (double valve replacement, DVR). Adding ARE to DVR may increase the operative risk by extending the surgical time. Herein, we review our experience with ARE in patients who underwent DVR. Materials and methods: The medical records of 69 patients who underwent DVR at our institution between February 2008 and November 2021 were retrospectively reviewed. The patients were divided into two groups according to the ARE procedure (ARE-DVR: 25 patients; DVR: 44 patients). Descriptive and comparative analyses of demographic, clinical, and surgical data were performed. Results: Among the 69 patients who underwent DVR, 35 were women (sex ratio, 0.97). The mean age at surgery was 26.7 ± 13.9 years (range: 7-62 years). Among the 47 patients aged ≤30 years, 40.4% (19/47) were aged between 10 and 20 years, and 6.3% (3/47) were aged <10 years. Patients in the ARE-DVR group were younger (23.3 ± 12.9 years vs. 28.5 ± 14.2 years, p < 0.05). The New York Heart Association Class ≥III dyspnea was the most common symptom (89.9%), with no differences between the two groups. Of all the patients, 84.1% had sinus rhythm. Rheumatic disease was the most common etiology in the entire cohort (91.3%). The mean aortic annulus diameter was 20.54 mm, with smaller sizes found in the ARE-DVR group (18.00 ± 1.47 mm vs. 22.50 ± 2.35 mm, p < 0.05). The aortic cross-clamping duration was greater in the ARE-DVR group (177.6 ± 37.9 min vs. 148.3 ± 66.3 min, p = 0.047). The operative mortality rate was 5.6% for the entire cohort (ARE-DVR: 8% vs. DVR: 4.5%, p = 0.46). Among the patients who underwent echocardiographic control at follow-up, the mean aortic gradient was 19.6 ± 7.2 mmHg (range: 6.14-33 mmHg), with no differences among the groups. Conclusion: The association between ARE and DVR did not significantly affect operative mortality. ARE can be safely used whenever indications arise to reduce the occurrence of PPM, especially in young patients with growth potential.

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.

The importance of being the morning case in adult cardiac surgery: a propensity-matched analysis.

OBJECTIVES: The quality of the outcome after cardiac surgery with cardiopulmonary bypass depends on the patient demographics, co-morbidities, complexity of the surgical procedure and expertise of surgeons and the whole staff. The purpose of the present study is to analyse the timing of surgery (morning versus afternoon) with respect to morbidity and mortality in adult cardiac surgery. METHODS: The primary end point was the incidence of major morbidity defined according to a modified Society of Thoracic Surgeon criterion. We consecutively included all the adult (>18 years) patients receiving a cardiac surgery operation at our Institution. RESULTS: From 2017 through 2019, a total of 4003 cardiac surgery patients were operated. With a propensity-matching technique a final patient population of 1600 patients was selected, with 800 patients in the first-case surgery group and 800 in the second-case surgery group. Patients in the second-case group had a major morbidity rate of 13% vs 8.8% in the first-case group (P = 0.006), and a higher rate of 30-day mortality (4.1% vs 2.3%, P = 0.033). After correction for EuroSCORE and operating surgeon, the second-case group confirmed a higher rate of major morbidity (odds ratio 1.610, 95% confidence interval 1.16-2.23, P = 0.004). CONCLUSION: Our study suggests that patients operated as second cases are exposed to an increased morbidity and mortality probably due to fatigue, loss of attention and hurriedness in the operating room and decreased human resources in the intensive care unit.

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.

Cardiac Surgery Development in Cameroon: Unexpected Challenges From a Socio-Political Crisis.

Despite the alarming and growing burden of cardiovascular diseases in sub-Saharan Africa (SSA), there is still a huge lack of specialised institutions in the region with a mean of one cardio-surgical unit for 33 million inhabitants. Despite the numerous efforts from humanitarian organisations made in recent years, the setting up of cardio-surgical units in the region remains challenging with regards to long-term sustainability. Indeed, besides the lack of financial resources, the insufficient local expertise in addition to the inadequate health infrastructure, unpredictable threats from external factors such as recurrent conflicts and humanitarian crises are still major concerns in an environment characterised by endemic socio-political instability. In Cameroon, located in the North West Anglophone region at 500 km from the capital, the cardiac centre of Shisong (CCS) is currently the lone cardio-surgical institution of the country. Fruit of a joint initiative of two Italian Non-governmental organisations namely, Bambini Cardiopatici nel Mondo (ABCnM) and Cuore Fratello (CF), and a local religious partner, the Tertiary Sisters of Saint Francis (TSSF), the CCS was faced with in the middle of a socio-political crisis that led to the urgent need of revision of the cardio-surgical project. The current paper reviews the impact of the ongoing socio-political crisis on the CCS over the past 3 years, in terms of clinical activities, staff perspectives, and long-term sustainability.

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.

Autograft Wrapping Reinforcement in Adolescents Undergoing Ross Operation: A Tailored Coat.

BACKGROUND: The Ross operation is a good surgical option for the pediatric population with aortic valve disease. However, the need for reoperation due to aortic root dilatation remains the principal limitation of this procedure. We report a small series of adolescents who underwent the Ross operation with Gore-Tex (W. L. Gore & Associates, Flagstaff, AZ) membrane reinforcement to avoid the progressive dilatation of the neoaortic root. METHODS: Between March 2002 and March 2010, 15 adolescent patients underwent a modified Ross procedure with the autograft wrapping with 0.1-mm Gore Preclude (W. L. Gore & Associates) pericardial membrane. Follow-up was performed by clinical and echocardiographic controls. RESULTS: These patients were a mean age of 15 ± 1.4 years. The mean aortic cross-clamp time was 130 ± 17 minutes. The mean cardiopulmonary bypass time was 187 ± 27 minutes. There was no hospital mortality. The mean follow-up was 15 ± 2.5 years (range, 9.7-17.7 years). During the follow-up, 1 patient required aortic valve reoperation for cusps prolapse. The mean diameters of annulus (22.8 ± 1.8 mm vs 23.3 ± 1.5 mm, P = .12), aortic root (27.4 ± 1.4 mm vs 28.2 ± 0.8 mm, P = .09), and sinotubular junction (24.3 ± 1.1 mm vs 25.1 ± 0.7 mm, P = .11) were not statistically different between discharge and follow-up. CONCLUSIONS: The wrapping reinforcement autograft in the Ross procedure with Gore-Tex membrane is simple, safe, and does not require significant additional time. Our results showed good early and long-term outcomes for reoperation, aortic root dilatation, and aortic valve degeneration.

Impact of COVID-19 Pandemic on the Italian Humanitarian Congenital Cardiac Surgery Activity: What No One Tells You.

More than 4 millions of children with congenital heart disease (CHD) are waiting for cardiac surgery around the world. Few of these patients are treated only thanks to the support of many non-governmental organizations (NGOs). Starting in December 2019, the so-called coronavirus disease 2019 (COVID-19) has rapidly become a worldwide pandemic and has dramatically impacted on all the international humanitarian activities for congenital heart disease. We analyzed data from all the Italian congenital cardiac surgery centers with the aim to quantify the impact of the pandemic on their charities. Fifteen Italian centers participated in the study and contributed to data collection. We analyzed and compared data regarding humanitarian activities carried out abroad and on site from two periods: year 2019 (pre-COVID-19) and year 2020 (COVID-19 pandemic). In 2019, 53 international missions were carried out by Italian congenital cardiac surgeons, resulting in the treatment of 471 CHD patients. In the same period 11 Italian cardiac centers operated on 251 foreign patients in Italy. In 2020, the pandemic led to a reduction of this activity by 96% for the surgery performed overseas and 86% for the interventions carried out in Italy. In conclusion our study shows the important quantitative impact of the pandemic on the Italian humanitarian cardiac surgical activity overseas and in Italy. This shocking result highlights the failure of the systems adopted so far to solve the problem of CHD in developing countries.

Perioperative GABA Blood Concentrations in Infants with Cyanotic and Non-Cyanotic Congenital Heart Diseases.

Perioperative stress detection in children with congenital heart disease (CHD), particularly in the brain, is still limited. Among biomarkers, γ-amino-aminobutyric acid (GABA) assessment in biological fluids appears to be promising for its regulatory action on the cardiovascular and cerebral systems. We aimed to investigate cyanotic (C) or non-cyanotic (N) CHD children for GABA blood level changes in the perioperative period. We conducted an observational study in 68 CHD infants (C: n = 33; N: n = 35) who underwent perioperative clinical, standard laboratory and monitoring parameter recordings and GABA assessment. Blood samples were drawn at five predetermined time-points before, during and after surgery. No significant perioperative differences were observed between groups in clinical and laboratory parameters. In C, perioperative GABA levels were significantly lower than N. Arterial oxygen saturation and blood concentration significantly differed between C and N children and correlated at cardiopulmonary by-pass (CPB) time-point with GABA levels. The present data showing higher hypoxia/hyperoxia-mediated GABA concentrations in C children suggest that they are more prone to perioperative cardiovascular and brain stress/damage. The findings suggest the usefulness of further investigations to detect the "optimal" oxygen concentration target in order to avoid the side effects associated with re-oxygenation during CPB.

Anomalous aortic origin of coronary artery biomechanical modeling: Toward clinical application.

OBJECTIVES: Anomalous aortic origin of the coronary artery can be associated with sudden cardiac death and ischemic events. Anatomic static characteristics mainly dictated surgical indications, although adverse events are usually related to dynamic physical effort. We developed a computational model able to simulate anomalous coronary behavior, and we aimed to assess its clinical applicability and to investigate coronary characteristics at increasing loading stress conditions. METHODS: We selected 5 patients with anomalous aortic origin of the coronary artery and 5 control subjects. For each of them, we construct a 3-dimensional model resembling the aortic root and coronary arteries based on 25 parameters obtained from computed tomography. Structural finite element analysis simulations were run to simulate pressure increasing in the aortic root during exercise (+40 mm Hg, +100 mm Hg with respect baseline condition, assumed at 80 mm Hg) and investigate coronary lumen characteristics. RESULTS: The 25 parameters were obtainable in all subjects with a consistent interobserver agreement. In control subjects, the right coronary artery had a more significant lumen expansion at loading conditions compared with anomalous aortic origin of coronary artery (6%-19.2% vs 1.8%-8.1%, P = .008), which also showed an inability to expand within the intramural segment. CONCLUSIONS: The proposed anomalous aortic origin of coronary artery model is able to represent the pathogenic disease mechanism after being populated with patient-specific data. It can assess the impaired expansion of anomalous right coronary at loading conditions, a process that cannot be quantified in any clinical set-up. This first clinical application showed promising results on quantifying pathological behavior, potentially helping in patient-specific risk stratification.

Surgical Rescue After Failed Percutaneous Closure of an Aorto-Atrial Tunnel.

Congenital sinus of Valsalva aneurysm is a rare disorder, mostly involving the right and noncoronary sinuses, in which intracardiac rupture is more likely foreseen into the right chambers. Because of the unfavorable prognosis, which includes heart failure and sudden death, detection itself is an indication for treatment, which may be performed either surgically or percutaneously. We present a case of a four-year-old patient with aorto-right atrial tunnel, in which a transcatheter attempt of closure was performed, complicated by new onset of aortic valve regurgitation, requiring surgical intervention.

Outcomes and Quality of Life After Ross Reintervention: Would You Make the Same Choice Again?

BACKGROUND: The Ross procedure was introduced as a long-term if not definitive solution for aortic pathology. However, the rate of reoperation is not negligible. METHODS: This single-center prospective study assessed the general outcome of Ross reoperation and patients' perceived quality of life compared with 2 control groups (Ross non-reoperation and mechanical aortic valve replacement). Patient's preference regarding the choice between mechanical aortic valve and Ross procedure was investigated in a subgroup that could theoretically have been directed to either of the 2 procedures. RESULTS: Between 2005 and 2017, 64 consecutive patients underwent reoperation after Ross. Median age was 31 years. Median freedom from reoperation after the Ross procedure was 136 months. An autograft reoperation was required in 49, and 25 had homograft failure. No in-hospital death was recorded. Mean follow-up was 77 months (range, 6-164 months). Quality of life was assessed with the 36-Item Short Form Health Survey questionnaire. The Ross reoperation group showed a lower score involving psychological concerns compared with the other groups. In the reoperated-on patients group, 52 had adequate aortic annulus dimensions to receive a prosthetic valve instead of a Ross procedure. When asked whether they would make the same choice, only 31% confirmed the preference. CONCLUSIONS: Reoperations after Ross procedure have low mortality and morbidity. Long-term follow-up showed a high quality of life, even after reoperations. However, owing to psychological concerns after the redo operation, when choosing a Ross procedure, it is our duty to thoroughly explain to patients that a high level of disillusion is predictable in case of reoperations.

Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†.

OBJECTIVES: We sought to describe early and late outcomes in a large surgical series of patients with anomalous aortic origin of coronary arteries. METHODS: We performed a retrospective multicentre study including surgical patients with anomalous aortic origin of coronary arteries since 1991. Patients with isolated high coronary takeoff and associated major congenital heart disease were excluded. RESULTS: We collected 156 surgical patients (median age 39.5 years, interquartile range 15-53) affected by anomalous right (67.9%), anomalous left (22.4%) and other anatomical abnormalities (9.6%). An interarterial course occurred in 86.5%, an intramural course in 62.8% and symptoms in 85.9%. The operations included coronary unroofing (56.4%), reimplantation (19.2%), coronary bypass graft (15.4%) and other (9.0%). Two patients with preoperative cardiac failure died postoperatively (1.3%). All survivors were discharged home in good clinical condition. At a median follow-up of 2 years (interquartile range 1-5, 88.5% complete), there were 3 deaths (2.2%), 9 reinterventions in 8 patients (5 interventional, 3 surgical); 91.2% are in New York Heart Association functional class ≤ II, but symptoms persisted in 14.2%; 48.1% of them returned to sport activity. On Kaplan-Meier analysis, event-free survival at follow-up was 74.6%. Morbidity was not significantly different among age classes, anatomical variants and types of surgical procedures. Furthermore, return to sport activity was significantly higher in younger patients who participated in sports preoperatively. CONCLUSIONS: Surgical repair of anomalous aortic origin of coronary arteries is effective and has few complications. Unroofing and coronary reimplantation are safe and are the most common procedures. The occurrence of late adverse events is not negligible, and long-term surveillance is mandatory. Most young athletes can return to an unrestrained lifestyle.