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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).

Marfan syndrome (MFS) is a rare inherited autosomic disorder, which encompasses a variety of systemic manifestations caused by mutations in the Fibrillin-1 encoding gene (FBN1). Cardinal clinical phenotypes of MFS are highly variable in terms of severity, and commonly involve cardiovascular, ocular, and musculoskeletal systems with a wide range of manifestations, such as ascending aorta aneurysms and dissection, mitral valve prolapse, ectopia lentis and long bone overgrowth, respectively. Of note, an accurate and prompt diagnosis is pivotal in order to provide the best treatment to the patients as early as possible. To date, the diagnosis of the syndrome has relied upon a systemic score calculation as well as DNA mutation identification. The aim of this review is to summarize the latest MFS evidence regarding the definition, differences and similarities with other connective tissue pathologies with severe systemic phenotypes (e.g., Autosomal dominant Weill–Marchesani syndrome, Loeys–Dietz syndrome, Ehlers–Danlos syndrome) and clinical assessment. In this regard, the management of MFS requires a multidisciplinary team in order to accurately control the evolution of the most severe and potentially life-threatening complications. Based on recent findings in the literature and our clinical experience, we propose a multidisciplinary approach involving specialists in different clinical fields (i.e., cardiologists, surgeons, ophthalmologists, orthopedics, pneumologists, neurologists, endocrinologists, geneticists, and psychologists) to comprehensively characterize, treat, and manage MFS patients with a personalized medicine approach.

Percutaneous closure of atrial septal defect (ASD) is a valid alternative to surgical approach. Current device has significantly improved the success rate also in complex cases. The aim of this study is to assess the impact of age, defect size, and morphologic features on successfully percutaneous ASD closure. Between January 2000 and September 2004, 1,013 consecutive patients underwent closure of an isolated type II ASD at our institution. The following outcomes have been evaluated: (1) role of percutaneous ASD closure as alternative to surgical repair, in current daily practice; (2) impact of age on the selected closure approach; (3) analysis of morphologic variety of ASD and its effect on the closure technique; (4) possible role of specific device selection according to ASD morphology to improve procedural success. During the study period, up to 80% of secundum ASDs were suitable for percutaneous closure with the currently available devices. Need for surgical ASD closure was more common in pediatric patients, likely reflecting the more frequent diagnosis of larger and complex defects at a young age. Accurate ADS morphology assessment and appropriate device selection are key elements to obtain procedural success. In particular, among all the ASD characteristics, the rim absence is the main limiting factor to a successful percutaneous ASD closure. A trend of reduction in peri-procedural adverse events was observed during the study period, with complications needing immediate cardiac surgery occurred only in 1% of cases. Percutaneous ASD closure is feasible and associated with low complication rate. A thorough analysis of morphologic aspects is mandatory in order to select the appropriate device and the optimal approach. Surgical closure remains the treatment of choice in selected patients.

BackgroundThis 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.MethodsA 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.ResultsThe 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.ConclusionCervical 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 Registrationhttps://www.crd.york.ac.uk/prospero/display_record.php?RecordID=346826, Identifier: CRD42022346826.

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.

The impact of congenital heart disease on brain aging has not been extensively investigated. We evaluated cerebral microbleeds and white matter hyperintensities on brain magnetic resonance imaging in adult patients with tetralogy of Fallot (ToF). Ten ToF patients (6 women, 4 men; aged 21-58 years; New York Heart Association [NYHA] class 1-2) were prospectively enrolled and underwent a T1-weighted, a T2-weighted dark fluid, and a T2*-weighted scans. Ten age- and sex-matched controls were prospectively recruited and subjected to the same acquisition protocol. Cerebral microbleeds (CMBs) were manually counted while white matter hyperintensities (WMHs) were segmented using ITK-Snap. Wilcoxon signed-rank test, Spearman correlation, and Bland-Altman statistics were used. The median (interquartile range [IQR]) age was 45.0 (30.5-49.5) years in ToF patients and 46.0 (30.5-49.8) years in controls. The median (IQR) of the number of CMBs was 6.0 (4.0-7.8) in ToF patients and 0 (0.0-0.0) in controls (p = 0.002). The WMHs burden was 2,506 (1,557-2,900) mm3 for ToF patients and 2,212 (1,860-2,586) mm3 for controls (p = 0.160). Moreover, a positive significant correlation was found between the WMHs burden and the NYHA class (ρ = 0.80, p = 0.005). Inter-operator concordance rate for the presence/absence of CMBs was 90%; the reproducibility for the WMHs burden was 77%. In conclusion, we found more cerebral microbleeds and a higher WMHs burden in adult ToF patients than in controls. This preliminary comparison supports the hypothesis of an early brain aging in ToF patients. Larger studies are warranted.

IntroductionAortic 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 methodsThe 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.ResultsAmong 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.ConclusionThe 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.

Despite advances in treatment of cardiogenic shock (CS), the incidence of this serious complication of acute ST-elevation myocardial infarction (STEMI) has stayed relatively constant, and rates of mortality, although somewhat improved in recent decades, remain dauntingly high. Although both percutaneous coronary intervention (PCI) and coronary artery bypass grafting (CABG) are used in patients with CS with multivessel coronary disease, the optimal revascularization strategy in this setting remains unknown. We conducted a literature search and review of English language publications on CS in multiple online medical databases. Studies were included if they were (1) randomized controlled trials or observational cohort studies, (2) single-center or multicenter reports, (3) prospective or retrospective studies, and (4) contained information on PCI and CABG. Non-English language studies were excluded. Our search retrieved no published findings from randomized clinical trials, and only 4 observational reports evaluating PCI versus CABG. Our review of the limited available data suggests similar mortality rates with CABG and PCI in patients with STEMI and multivessel coronary disease complicated by CS. Limited data from observational studies in patients with CS and multivessel disease suggest that CABG should be considered a complementary reperfusion strategy to PCI and may be preferred, especially when complete revascularization with PCI is not possible. Our data highlight the need for large randomized trials to further evaluate the relative benefit of PCI versus CABG in patients with multivessel coronary disease and CS using contemporary surgical and percutaneous techniques.

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.

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.