Search Results (39)

Objective: This multicenter study compares outcomes of open thoracoabdominal aortic aneurysm (TAAA) repair in patients < 60 years with (n = 106), versus without (n = 167), hereditary aortopathy. Methods: We conducted a retrospective analysis of 273 consecutive open TAAA repairs (2000–2024) at two European centers. The primary endpoint was early outcome. We used a Kaplan–Meier curve to assess survival, and logistic regression to identify predictors. Results: Operative death rates were similar (hereditary: 13/106 [12.3%] vs. non-hereditary: 22/167 [13.2%], p = 0.83). Hereditary aortopathy patients were younger (median 42 vs. 54 years, p < 0.001) with lower BMI (24.1 vs. 28.4 kg/m², p < 0.001). Non-genetic patients had higher rates of chronic kidney insufficiency (58/167 (34.7%) vs. 14/106 (13.2%), p < 0.001), coronary artery disease (43/167 (25.7%) vs. 9/106 (8.5%), p < 0.001), and prior myocardial infarction (31/167 (18.6%) vs. 4/106 (3.8%), p < 0.001). Hereditary aortopathy patients suffered more often from post-dissection TAAA (68/106 [64.2%] vs. 44/167 [26.3%], p < 0.001) and prior aortic surgery (81/106 (76.4%) vs. 79/167 (47.3%), p < 0.001). Pulmonary complications (67.0% vs. 61.1%, p = 0.32), acute kidney injury (25.5% vs. 22.8%, p = 0.61), and spinal cord ischemia (6.6% vs. 10.2%, p = 0.31) were comparable between groups. Overall 5-year survival was 65.7%; the rate of any reintervention during follow up was 21.2%. Logistic regression identified no predictors for perioperative mortality. Conclusions: Open TAAA repair in patients < 60 years carries relevant perioperative mortality, which is comparable between hereditary and non-hereditary groups; non-hereditary patients had impaired preoperative cardiopulmonary status. Full article

Background/Objectives: Kissing aneurysms, a rare and intriguing cerebrovascular anomaly, challenge even the most advanced neurosurgical techniques. These lesions, characterized by two intimately apposed aneurysms with shared arterial walls, often masquerade as single, irregular aneurysms. This report documents a case of ruptured kissing aneurysms in the M1 segment of the right middle cerebral artery (MCA), complicated by ischemic stroke and pulmonary thromboembolism (PTE)—a convergence of severe complications rarely encountered. The case underscores the importance of precise diagnostics, innovative surgical strategies, and multidisciplinary care. Methods: A 55-year-old female presented with subarachnoid hemorrhage, confirmed by advanced imaging to arise from ruptured kissing aneurysms in the M1 segment of the right MCA. Surgical intervention via a right frontotemporal craniotomy and microsurgical clipping achieved definitive aneurysmal exclusion. Postoperatively, the patient experienced ischemic stroke and PTE, necessitating dynamic adjustments in anticoagulation therapy, intensive care, and rehabilitation protocols. Results: The dual aneurysms were successfully clipped, as confirmed by intraoperative and postoperative imaging. Despite developing significant complications, including left-sided motor deficits and PTE, a carefully orchestrated treatment strategy enabled the patient’s full recovery, with marked neurological and systemic improvement by her three-month follow-up. This favorable outcome highlights the resilience of a multidisciplinary approach in navigating such high-risk scenarios. Conclusions: This case showcases the formidable challenges of managing kissing aneurysms, particularly when compounded by stroke and PTE. It emphasizes the transformative role of cutting-edge imaging and surgical techniques in achieving successful outcomes. By illustrating how precision medicine and collaborative care can overcome rare and complex cases, this report contributes valuable insights to the evolving field of cerebrovascular surgery and postoperative management. Full article

Pulmonary vasculitis identifies a heterogeneous group of diseases characterized by inflammation, damage and necrosis of the wall of pulmonary vessels. The most common approach to classify vasculitis is according to etiology, therefore dividing them into primary and secondary, with a further sub-classification of primary vasculitis based on the size of the affected vessels (large, medium, and small). Pulmonary involvement is frequently observed in patients with systemic vasculitis and radiological presentation is not pathognomonic, but may vary between diseases. The main findings using high-resolution computed tomography (HRCT) include small vessel wall thickening, nodular lesions, cavitary lesions, reticular opacities, ground-glass opacities (GGO), consolidations, interlobular septal thickening, tracheobronchial stenosis, and aneurysmal dilatation of pulmonary arteries, with or without pleural effusion. Radiological diagnosis alone is difficult since signs and symptoms of lung vessel involvement are often non-specific and might overlap with other conditions such as infections, connective tissue diseases and neoplasms. Therefore, the aim of this review is to describe the most common radiological features of lung involvement in pulmonary vasculitis so that, alongside detailed clinical history and laboratory tests, a prompt diagnosis can be performed. Full article

Background: Takayasu’s arteritis is a rare type of vasculitis with severe complications like stroke, ischemic heart disease, pulmonary hypertension, secondary hypertension, and aneurysms. Diagnosis is achieved using clinical and angiographic criteria. Treatment is medical and surgical, but unfortunately, the outcome is limited. Case presentation: A 34-year-old Caucasian woman had an ischemic stroke (2009). She was diagnosed with Takayasu’s arteritis and received treatment with methotrexate, prednisolone, and antiplatelet agents, with a mild improvement in clinical state. After 6 years (2015), she experienced an ascending aorta aneurysm, pulmonary hypertension, and mild aortic regurgitation. Surgical treatment solved both the ascending aorta aneurysm and left carotid artery stenosis (ultrasound in 2009 and computed tomography angiogram in 2014). Morphopathology revealed a typical case of Takayasu’s arteritis. Tumor necrosis factor inhibitors (TNF inhibitors) were prescribed with methotrexate. At 48 years old (2023), she developed coronary heart disease (angina, electrocardiogram); echocardiography revealed severe pulmonary hypertension, and angiography revealed normal coronary arteries, abdominal aorta pseudoaneurysm, and arterial–venous fistula originating in the right coronary artery with drainage in the medium pulmonary artery. The patient refused surgical/interventional treatment. She again received TNF inhibitors, methotrexate, antiplatelet agents, and statins. Conclusions: This case report presented a severe form of Takayasu’s arteritis. Our patient had multiple arterial complications, as previously mentioned. She received immunosuppressive treatment, medication targeted to coronary heart disease, and surgical therapy. Full article

Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy and is associated with considerable early mortality. Heart transplantation is often the only viable life-saving option. Pulmonary artery banding (PAB) has been recently proposed as a bridge or alternative to transplantation for DCM. In our cohort, PAB was selectively addressed to heritable DCM or DCM with congenital left ventricle aneurysm (CLVA). This study aimed to describe the clinical evolution and left ventricle reverse remodeling (LVRR) over time (6 months and 1 year after surgery). Ten patients with severe DCM received PAB between 2016 and 2021 and underwent clinical and postoperative echocardiography follow-ups. The median age at PAB was <1 year. The in-hospital mortality was zero. Two patients died two months after PAB of end-stage heart failure. The modified Ross class was improved in the eight survivors with DCM and remained stable in the two patients with CLVA. We observed a positive LVRR (LV end-diastolic diameter Z-score: 8.4 ± 3.7 vs. 2.8 ± 3; p < 0.05; LV ejection fraction: 23.8 ± 5.8 to 44.5 ± 13.1 (p < 0.05)). PAB might be useful as part of the armamentarium available in infants and toddlers with severe DCM not sufficiently responding to medical treatment with limited probability of spontaneous recovery. Full article

This case report presents a 34-year-old male with Ehlers–Danlos syndrome, type 2 diabetes mellitus, aortic valve regurgitation, and aortic bulb aneurysm. Following spine surgery for thoracic–lumbar stabilization, the patient underwent assessment for aortic bulb aneurysm and aortic valve replacement surgeries. Five months post spinal surgery, a coronary computed tomography angiography was performed. The coronary computed tomography angiography revealed unique findings, including the absence of the left main coronary artery, right coronary artery dominance, ectopic origin of the left circumflex artery from the right sinus of the valsalva, a coronary–pulmonary arterial fistula originating from the right sinus of the valsalva, and an additional right pulmonary vein. The patient was qualified for surgical treatment for an aortic bulb aneurysm, was informed about the high surgical risk, and is awaiting surgery. This case underscores the rarity of Ehlers–Danlos syndrome coexisting with multiple coronary artery anomalies. The presence of a coronary–pulmonary arterial fistula further emphasizes the need for specialized patient monitoring when Ehlers–Danlos syndrome and coronary anomalies converge. Full article

Background: There is significant debate regarding the existence of sex-related differences in the presentation, treatment, and outcomes of men versus women affected by abdominal aortic aneurysm (AAA). The purpose of this study is to compare endovascular aneurysm repair (EVAR) of infrarenal AAAs with the current sex-neutral 5.0–5.5 cm-diameter threshold for intervention between the two sexes. Methods: Retrospective review of consecutive cases from a single teaching institution over a period of five years of patients who had undergone elective EVAR for AAAs between 5.0 and 5.5 cm in diameter. Outcomes of interest were compared according to sex. Results: Ninety-four patients were included in the analysis, with a higher prevalence of men (53%). Females were older at the time of repair, 78 ± 5.1 years, versus 71.7 ± 7 years (p < 0.01), and had higher incidence of underlying comorbidities, namely, arrhythmia, chronic kidney disease, and previous carotid revascularization. Women had higher incidence of immediate systemic complications (p = 0.021), post-operative AMI (p = 0.001), arrhythmia (p = 0.006), pulmonary oedema (p < 0.001), and persistent renal dysfunction (p = 0.029). Multivariate analysis for post-operative factors associated to mortality and adjusted for sex confirmed that AMI (p = 0.015), arrhythmia (p = 0.049), pulmonary oedema (p = 0.015), persistent renal dysfunction (p < 0.001), cerebral ischemia (p < 0.001), arterial embolism of lower limbs (p < 0.001), and deep-vein thrombosis of lower limbs (p < 0.001) were associated to higher EVAR-related mortality; a higher incidence of post-operative AMI (p = 0.014), pulmonary edema (p = 0.034), and arterial embolism of lower limbs (p = 0.046) were associated to higher 30-days mortality. In females there was also a higher rate of suprarenal fixation (p = 0.026), insertion outside the instruction for use (p = 0.035), and a more hostile neck anatomy with different proximal aortic diameter (p < 0.001) and angle (p = 0.003). Conclusions: A similar threshold of size of AAA for elective surgery for both males and females might not be appropriate for surgical intervention, as females tend to have worse outcomes. Further population-based studies are needed to guide on sex-related differences and intervention on AAA. Full article

Background. The aim of this study was to assess the incidence of two post-operative acute kidney injury (AKI) stages according to the Risk, Injury, Failure, Loss of function, End-stage (RIFLE) criteria in patients undergoing fenestrated endovascular aortic repair (FEVAR) for complex aortic aneurysms. Furthermore, we analyzed predictors of post-operative AKI and mid-term renal function deterioration and mortality. Methods. We included all patients who underwent elective FEVAR for abdominal and thoracoabdominal aortic aneurysms between January 2014 and September 2021, independently from their preoperative renal function. We registered cases of post-operative acute kidney injury (AKI) both at risk (R-AKI) and injury stage (I-AKI) according to the RIFLE criteria. Estimated glomerular filtration rate (eGFR) was noted preoperatively, at the 48th post-operative hour, at the maximum post-operative peak, at discharge, and then during follow-up approximately every six months. Predictors of AKI were analyzed with univariate and multivariate logistic regression models. Predictors of mid-term chronic kidney disease (CKD) (stage ≥ 3) onset and mortality were analyzed using univariate and multivariate Cox proportional hazard models. Results. Forty-five patients were included in the present study. Mean age was 73.9 ± 6.1 years and 91% of patients were males. Thirteen patients (29%) presented with a preoperative CKD (stage ≥ 3). Post-operative I-AKI was detected in five patients (11.1%). The aneurysm diameter, thoracoabdominal aneurysms and chronic obstructive pulmonary disease were identified as predictors of AKI in univariate analysis (OR 1.05, 95% CI [1.005–1.20], p = 0.030; OR 6.25, 95% CI [1.03–43.97], p = 0.046; OR 7.43, 95% CI [1.20–53.36], p = 0.031; respectively), yet none of these factors were significative on multivariate analysis. Predictors of CKD onset (stage ≥3) during follow-up on multivariate analysis were age (HR 1.16, 95% CI [1.02–1.34], p = 0.023), post-operative I-AKI (HR 26.82, 95% CI [4.18–218.10], p < 0.001) and renal artery occlusion (HR 29.87, 95% CI [2.33–309.05], p = 0.013), while aortic-related reinterventions where not significantly associated with this outcome in univariate analysis (HR 0.66, 95% CI [0.07–2.77], p = 0.615). Mortality was influenced by preoperative CKD (stage ≥3) (HR 5.68, 95% CI [1.63–21.80], p = 0.006) and post-operative AKI (HR 11.60, 95% CI [1.70–97.51], p = 0.012). R-AKI did not represent a risk factor for CKD (stage ≥ 3) onset (HR 1.35, 95% CI [0.45–3.84], p = 0.569) or for mortality (HR 1.60, 95% CI [0.59–4.19], p = 0.339) during follow-up. Conclusions. In-hospital post-operative I-AKI represented the main major adverse event in our cohort, influencing CKD (≥ stage 3) onset and mortality during follow-up, which were not influenced by post-operative R-AKI and aortic-related reinterventions. Full article

The purpose of this bicentric case series was to report the safety, efficacy, and clinical outcome of transcatheter embolization in pulmonary artery pseudoaneurysms (PAPAs). Between January 2016 and June 2021, eight patients with PAPA were subjected to transcatheter embolization. The total number of patients was eight, of which five were female, with a mean age of 62 ± 14 years (average ± standard deviation). Etiology was traumatic in 2/8 cases and iatrogenic in 6/8 cases (after positioning a Swan-Ganz catheter in 5/6 cases and a temporary pacemaker in the latter case). In a single case, the PAPA was incidentally discovered during a routine X-ray, in the remaining 7 cases, the procedure was performed in emergency settings. PAPA embolization was performed using detachable coils alone in 3 cases; coils and glue in 1 case; coils, glue, and vascular plug in 1 case; coils and non-adhesive liquid embolic agents (Onyx and Squid respectively) in 2 cases; and non-adhesive liquid embolic agent alone (Onyx) in 1 case. No peri-procedural or post-procedural complications were recorded. Both the technical and clinical success rates were 100.0%. In conclusion, endovascular embolization is a technically feasible and safe therapeutic option for patients with PAPAs. Full article

Cardiovascular disease (CVD) is the leading cause of morbidity and mortality worldwide. Although a cadre of therapeutic strategies have been made available for CVDs in the clinical setting, predominantly through medication and surgery, these do not fully address the clinical needs of patients with CVD. As a new technique for CVD treatment, nanocarriers are employed to modify and package medications to ease the targeting of tissues, cells and molecules within the cardiovascular system. Nanocarriers are made of biomaterials, metals, or a combination of these materials, with sizes similar to bioactive molecules such as proteins and DNA. Cardiovascular nanomedicine (CVN) has only surfaced in recent years and is still in its infancy. Ample studies have displayed promise for the clinical utility of nanomedicine techniques, courtesy of continued perfection in nanocarrier design to optimize drug delivery and treatment outcomes. Here in this review, we will summarize the research advances in the literature on nanoparticles in the management of CVDs, including ischemic and coronary heart disease (e.g., atherosclerosis, angina pectoris and myocardial infarction), myocardial ischemia-reperfusion injury, aortic aneurysm, myocarditis, hypertension, and pulmonary artery hypertension and thrombosis. Full article

Hughes-Stovin syndrome is a rare disease characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. The etiology and pathogenesis of HSS are incompletely known. The current consensus is that vasculitis underlies the pathogenic process, and pulmonary thrombosis follows arterial wall inflammation. As such, Hughes-Stovin syndrome may belong to the vascular cluster with lung involvement of Behçet syndrome, although oral aphtae, arthritis, and uveitis are rarely found. Behçet syndrome is a multifactorial polygenic disease with genetic, epigenetic, environmental, and mostly immunological contributors. The different Behçet syndrome phenotypes are presumably based upon different genetic determinants involving more than one pathogenic pathway. Hughes-Stovin syndrome may have common pathways with fibromuscular dysplasias and other diseases evolving with vascular aneurysms. We describe a Hughes-Stovin syndrome case fulfilling the Behçet syndrome criteria. A MYLK variant of unknown significance was detected, along with other heterozygous mutations in genes that may impact angiogenesis pathways. We discuss the possible involvement of these genetic findings, as well as other potential common determinants of Behçet/Hughes-Stovin syndrome and aneurysms in vascular Behçet syndrome. Recent advances in diagnostic techniques, including genetic testing, could help diagnose a specific Behçet syndrome subtype and other associated conditions to personalize the disease management. Full article

Although primarily a lung disease, extra-pulmonary tuberculosis (TB) can affect any organ or system. Of these, cardiovascular complications associated with disease or drug toxicity significantly worsen the prognosis. Approximately 60% of patients with TB have a cardiovascular disease, the most common associated pathological entities being pericarditis, myocarditis, and coronary artery disease. We searched the electronic databases PubMed, MEDLINE, and EMBASE for studies that evaluated the impact of TB on the cardiovascular system, from pathophysiological mechanisms to clinical and paraclinical diagnosis of cardiovascular involvement as well as the management of cardiotoxicity associated with antituberculosis medication. The occurrence of pericarditis in all its forms and the possibility of developing constrictive pericarditis, the association of concomitant myocarditis with severe systolic dysfunction and complication with acute heart failure phenomena, and the long-term development of aortic aneurysms with risk of complications, as well as drug-induced toxicity, pose complex additional problems in the management of patients with TB. In the era of multidisciplinarity and polymedication, evidence-based medicine provides various tools that facilitate an integrative management that allows early diagnosis and treatment of cardiac pathologies associated with TB. Full article

The objective was to investigate the prevalence of abdominal aortic aneurysms (AAAs) and the diameters of the aorta and common iliac arteries (CIAs) in a Korean cohort and secondly to analyze the differences in aortic diameter by comparison with a European cohort. The Korean cohort included participants ≥ 50 years who consented to AAA screening and data were analysed retrospectively. Aortic and common iliac diameters were measured using the outer-to-outer diameter method and prevalence rates were calculated. Common risk factors such as smoking, body mass index, pulmonary disease, hypertension, diabetes, hyperlipidaemia, ischaemic heart disease, and cerebrovascular disease were reported in association with AAA occurrence and AAA development. The aortic diameters were then compared with those in a Belgian cohort of 2487 participants identified in the Liège AAA Screening Program. An aortic size index (ASI) was also calculated to account for the potential size differences in the Belgian and Korean populations. A total of 3124 Korean participants were examined using ultrasound. The prevalence of AAAs in this cohort was 0.7%. The combined prevalence of subaneurysmal dilatation and AAA was 1.5%. The prevalence in male smokers older than 65 years was 2.7% (19/715). The mean infrarenal aortic diameter was 17.3 ± 3.1 mm in men and 15.7 ± 2.7 mm in women; the corresponding values in Belgian participants were 19.4 ± 3.0 mm in men and 17.9 ± 2.4 mm in women. The median aortic size index was 0.99 (interquartile range 0.88–1.12). The mean infrarenal aortic diameter was significantly smaller in the Korean cohort than in the Belgian cohort. Considering the observed prevalence of AAAs in different age groups, the age groups which would contribute to most cases was male persons above 66 years in both cohorts. Full article

Aim: To analyze early and mid-term outcomes of the Bentall operation. Methods: Two hundred and seventeen patients (mean age 65.6 ± 15.9 years, males/females 172/45) underwent Bentall operation in a 7-year period (January 2015–December 2021), on average, 30 Bentall operations occurred per year, using biological (n = 104) or mechanical (n = 113) valved conduits for the treatment of ascending aorta–aortic root aneurysms. Associate procedures were performed in 58 patients (26.7%); coronary artery bypass grafting (CABG) in 35 (16%). Mean follow-up was 55.2 ± 24 (median 60.2) months. Cox model analysis was used to assess risk factors, Kaplan–Meier and log-rank tests were used to assess different survival rates. Results: Operative mortality was 1.38%. At 7 years, survival, freedom from cardiac death, and event-free survival were 93% ± 2%, 99% ± 1%, and 81% ± 5%. NYHA class (p < 0.0001), trans-aortic valve mean (p < 0.0001) and maximum (p < 0.000) gradients, left ventricular hypertrophy (p < 0.05), and pulmonary arterial pressure (p = 0.002) significantly improved vs. preoperative values. Concomitant CABG during Bentall operation independently affected late outcomes (HR 1.9–2.3; p-values < 0.05). Late survival was affected by concomitant CABG (84% ± 8% vs. 95% ± 2%, p = 0.04), preoperative myocardial infarction (91% ± 9% vs. 97% ± 2%, p = 0.02), and biological vs. mechanical prostheses valved conduits (91% ± 9% vs. 95% ± 3%, p = 0.02). Event-free survival also was affected by concomitant CABG (62% ± 14% vs. 85% ± 5%, p = 0.005) and biological prostheses (78% ± 8% vs. 84% ± 6%, p = 0.06). Freedom from endocarditis–redo operation was 83% ± 9% for biological prostheses vs. 89% ± 6% for mechanical prostheses (p = 0.49). Conclusions: Low rates of operative mortality and late complications make Bentall operation the gold standard for the treatment of ascending aorta–aortic root aneurysms. Coronary ischemic disease affects late outcomes. Biological prostheses should be preferred for the elderly. Full article

The ADNP-gene-related neurodevelopmental disorder Helsmoortel–Van der Aa syndrome is a rare syndromic-intellectual disability—an autism spectrum disorder first described by Helsmoortel and Van der Aa in 2014. Recently, a large cohort including 78 patients and their detailed phenotypes were presented by Van Dijck et al., 2019, who reported developmental delay, speech delay and autism spectrum disorder as nearly constant findings with or without variable cardiological, gastroenterological, urogenital, endocrine and neurological manifestations. Among cardiac malformations, atrial septal defect, patent ductus arteriosus, patent foramen ovale and mitral valve prolapse were the most common findings, but other unspecified defects, such as mild pulmonary valve stenosis, were also described. We present two patients with pathogenic ADNP variants and unusual cardiothoracic manifestations—Bland–White–Garland syndrome, pectus carinatum superiorly along the costochondral junctions and pectus excavatum inferiorly in one patient, and Kawasaki syndrome with pericardiac effusion, coronary artery dilatation and aneurysm in the other—who were successfully treated with intravenous immunoglobulin, corticosteroid and aspirin. Both patients had ectodermal and/or skeletal features overlapping those seen in RASopathies, supporting the observations of Alkhunaizi et al. 2018. on the clinical overlap between Helsmoortel–Van der Aa syndrome and Noonan syndrome. We observed a morphological overlap with the Noonan-like disorder with anagen hair in our patients. Full article