Intimal sarcomas (ISarc) of the cardiac chambers (CC) of the heart and great vessels (GV): a comprehensive genomic profiling (CGP) study.

Abstract Background Sodium-glucose cotransporter 2 inhibitors (SGLT-2i) have proven benefits in patients with biventricular heart failure, however, their role in patients with univentricular physiology and failing Fontan Circulation (FC) remains largely unexplored. Clinical Presentation A 21-year-old male with failing FC, preserved ejection fraction, and Protein Loosing Enteropathy (PLE) presented to our Adult Congenital Heart Disease unit for evaluation. Born with a L-transposition of the great vessels with interventricular septal defect and antero-superior rudimentary chamber, he underwent a Fontan completion at age 5. Since he was 16 years old, he had multiple admissions for FC failure complicated by PLE and ascitis, which did not respond to conventional treatments including diuretics, corticosteroids, albumin and immunoglobulin infusions. Despite the stenosis of the extracardiac conduit, treated by the placement of a covered stent and a further conduit balloon dilation and stenting in stent procedure performed after 3 years, the patient continued to experience ascites and hypoalbuminemia. To better understand the extent of his lymphatic dysfunction, Magnetic Resonance (MR) lymphoscintigraphy was performed. The study showed significant lymphatic congestion, with leakage into the duodenal lumen and prominent stasis in the thoracic duct. Based on these findings, therapy with SGLT-2i was initiated. Over the following 18 months, there was marked clinical improvement with a reduction in ascites, and stabilization of serum protein and albumin levels. Conclusions This case highlights the potential benefit of SGLT-2i in managing Fontan failure complicated by PLE. Multicenter studies are needed to further investigate the efficacy, safety and mechanism of action of SGLT-2i in this unique patient population.

Background Idiopathic retroperitoneal fibrosis (IRPF) is a rare fibroinflammatory condition that is challenging to distinguish from retroperitoneal malignancies based solely on imaging. We evaluated laparoscopic ultrasound-guided biopsy for diagnosing IRPF. Methods We retrospectively analyzed 12 patients with suspected retroperitoneal fibrosis who underwent laparoscopic ultrasound-guided biopsy. The surgical technique involved systematic laparoscopic exploration, intraoperative ultrasound to delineate the mass and its relation to great vessels, and targeted biopsy. Results Preoperative imaging suggested retroperitoneal masses in all 12 patients but failed to provide a definitive diagnosis in any case. Laparoscopic biopsy was successfully performed in all patients without conversion to laparotomy. Pathological examination confirmed IRPF in 11 patients and retroperitoneal adenocarcinoma in one. The procedure was safe, with minimal blood loss (20.4 ± 14.8 mL) and a short operative time (68.3 ± 19.0 min). All IRPF patients were treated with a combination of prednisone, tamoxifen, and mycophenolate mofetil, leading to significant lesion regression on follow-up imaging (median 37.9 months). Conclusions Laparoscopic ultrasound-guided biopsy is a safe and highly effective diagnostic procedure for IRPF. It provides definitive pathological diagnosis to guide appropriate therapy and should be considered when percutaneous biopsy is not feasible.

Introduction: Vascular morphometry plays a key role in diagnosing congenital pathologies and supporting clinical management. Although the diameters of great vessels in adults are well-established, with influences from factors such as age and sex, there remains a gap in the literature regarding their development in fetuses. In particular, the relationship between the diameters of the ascending aorta (AA), pulmonary trunk (PT), and brachiocephalic trunk (BCT) with gestational age and sex, as well as the onset of sexual dimorphism throughout development, remains underexplored. Objective: To investigate the correlation between the diameters of the AA, PT, and BCT with gestational age and sex in human fetuses. Methodology: Thirty-five fetal cadavers (18 male, 17 female), collected between 2012 and 2020 at the Human Anatomy Laboratory of UFS, were analyzed following ethical approval and exclusion of cardiovascular malformations. Gestational age was determined using the hallux-to-calcaneus length method. External diameters of the AA, PT, and BCT were measured with a digital caliper. Data were analyzed using Spearman’s correlation coefficient to assess associations with gestational age and Student’s t test to compare differences between sexes. Results: Strong positive correlations were found between gestational age and the diameters of the AA (ρ = 0.710), PT (ρ = 0.701), and BCT (ρ = 0.828) (p < 0.001 for all), confirming consistent growth throughout fetal development. No significant differences were found between males and females (p > 0.05). Conclusion: This study provides normative fetal data for the AA, PT, and BCT, demonstrating that vessel diameters increase proportionally with gestational age. The absence of sexual dimorphism in the fetal period suggests that postnatal hormonal and environmental factors may contribute to vascular size differences observed later in life. These findings provide clinically relevant reference values for prenatal diagnosis of vascular abnormalities. In addition, enhance understanding of vascular development.

Background The pathological diagnosis of mediastinal lesions is crucial for precision oncology. While endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is the preferred minimally invasive method for visceral mediastinal lesions, its diagnostic yield can be limited for high-risk lesions surrounded by great vessels. This case demonstrates that under such complex anatomical constraints, a meticulously planned CT-guided percutaneous biopsy serves as a safe and effective alternative. Case presentation A middle-aged male patient was highly suspected of having lung cancer with mediastinal metastasis based on clinical and radiological findings. Two successive bronchoscopic biopsies of the pulmonary lesion failed to yield a diagnosis. Although the medical team recommended EBUS-TBNA at a tertiary hospital, the patient opted for a CT-guided biopsy at our institution after considering personal convenience and economic factors. The target was a high-risk mediastinal lymph node located within the vascular “core area” between the aorta and superior vena cava. Intervention and outcome Preprocedural planning with contrast-enhanced CT simulated three potential trajectories (transcostochondral, transsternal, transpulmonary). The transsternal approach was prioritized to avoid lung parenchyma, thereby eliminating the risk of pneumothorax—a critical consideration given the patient’s comorbid emphysema and bullae. The initial transcostochondral approach was abandoned due to pain upon vascular contact and restricted maneuverability. The subsequent transsternal approach was successfully performed using a coaxial biopsy system to navigate the narrow vascular space, followed by tract embolization upon needle withdrawal. The procedure was safe, with only minimal, self-resolving mediastinal emphysema. Adequate tissue cores were obtained, enabling a definitive diagnosis of metastatic lung adenocarcinoma. Conclusion For complex mediastinal lesions where standard approaches are unsuitable or declined by the patient, a meticulously planned CT-guided percutaneous biopsy based on three-dimensional anatomical assessment is a feasible and valuable diagnostic strategy.

Outcomes of Ascending Aorta-Based Trans-sternal Great Vessel Reconstruction.

Comparative analysis of artificial intelligence-based contouring of cardiac substructures on computed tomography scans for radiation therapy.

VATS resection of a large mediastinal teratoma abutting great vessels.

Rationale:This report describes a rare case of dextrocardia concurrent with atrial fibrillation (AF) and ischemic bowel disease, to inform clinical management of such complex comorbidities.Patient concerns:A 70-year-old male presented with hematochezia, left lower abdominal pain, and AF confirmed by electrocardiogram.Diagnoses:Dextrocardia (complete transposition of great vessels), AF with atrial enlargement and left ventricular dysfunction, and colonic ischemic bowel disease (mucosal edema/ulceration) were confirmed.Interventions:Conservative therapy was given, including vasodilators, fluid resuscitation, anticoagulants, cardiac rate control, and antibiotics.Outcomes:Symptoms improved significantly posttreatment: abdominal pain resolved, stool color normalized, and cardiac status stabilized.Lessons:Comprehensive diagnosis and regular follow-up are essential for dextrocardia patients. Targeted intervention improves prognosis in complex comorbid cases.

Thoracic stab injuries (TSI) are rare but potentially life-threatening emergencies. In Germany, their incidence in emergency departments remains low. The cardiac box (CB) concept has been proposed to identify cardiac involvement in penetrating thoracic trauma, although its clinical relevance remains uncertain. This study aimed to evaluate the in-hospital management of TSI and to assess the predictive value of the cardiac box for major intrathoracic injuries. A retrospective and exploratory analysis was conducted of all patients with TSI resulting from assault or self-harm who were admitted to a certified Level 1 trauma centre between January 2020 and June 2024. Prehospital and in-hospital variables were descriptively analysed. Fifty-six male patients were included (median age 28.5years). Sixteen patients sustained injuries within the cardiac box (CB), and thirty-nine outside this area (NCB). All CB patients (100%) and 95% of NCB patients were admitted via the emergency department. The annual proportion of thoracic stab injuries among all emergency presentations ranged from 1.4% to 2.4%. The median Injury Severity Score (ISS) was significantly higher in the CB group (9.5 vs 3; p = 0.045), whereas the distribution of intrathoracic injury types and initial haemodynamic parameters (MAP CB: 93mmHg vs NCB: 97mmHg; p = 0.925) did not differ significantly. Two patients in the CB group had a cardiac and/or great vessel injury. Two NCB patients received prehospital chest tubes. In the emergency department, chest tubes were placed in 23.2% of patients, with no significant group difference. Median intrahospital transfer time to the target department was shorter in CB patients (38 vs 67min). Video-assisted thoracic surgery (VATS) was performed in eight patients (CB: 25%; NCB: 10.3%; p = 0.241), and one open procedure was undertaken in each group. Major complications (Clavien-Dindo ≥ II) occurred more frequently among CB patients (50.1% vs 25.7%; p = 0.018). The overall mortality rate was 3.6% (two CB patients). TSI are rare but serious injuries requiring structured, multidisciplinary in-hospital management. Minimally invasive approaches are feasible in haemodynamically stable patients. The low rate of prehospital chest tube placement warrants further evaluation. The cardiac box concept appears overly simplistic, as clinically significant injuries may also occur outside this anatomical region.

Background: This study evaluated the diagnostic efficacy of transthoracic echocardiography (TTE) and 128-slice multislice computed tomography (MSCT) angiography in congenital heart disease (CHD). Methods: Between January 2018 and August 2022, 50 patients diagnosed with CHD underwent both TTE and ECG-gated 128-MSCT. The imaging findings were compared with intraoperative observations, categorizing pathologies into cardiac, heart–great vessel, and great vessel malformations. Results: The median age of the patients was 0.45 months, and the median weight was 5 kg. Echocardiography showed a sensitivity of 89.8% and specificity of 99.12%, with an overall accuracy of 98%. MSCT had a sensitivity of 87.9%, specificity of 98.95%, and accuracy of 97.62%. There was no significant difference in diagnostic accuracy between the two modalities (χ2 = 31.796, p = 0.215), with substantial agreement (kappa = 0.901). For surgically confirmed cardiac malformations (n = 69), echocardiography had a 100% sensitivity, whereas MSCT had an 88.41% sensitivity (χ2 = 20.633, p = 0.039), with high concordance (kappa = 0.931). For heart–great vessel connection anomalies (n = 27), both modalities had an 81.48% sensitivity (χ2 = 14.115, p = 0.481), with substantial agreement (kappa = 0.887). For great vessel malformations (n = 61), the echocardiography and MSCT sensitivities were 81.97% and 88.52%, respectively, with no significant difference in performance (χ2 = 30.303, p = 0.063), indicating substantial concordance (kappa = 0.863). Conclusions: Both TTE and MSCT are highly accurate for CHD diagnosis, each with unique advantages. Their complementary use, especially where one modality is limited, enables a more comprehensive assessment, supporting clinical decision-making and surgical planning.

Pathologic mineralization refers to the abnormal deposition of calcium salts in tissues. In dogs, metastatic calcification is an uncommon but serious complication of chronic kidney disease (CKD), resulting from disturbances in calcium/phosphorus metabolism or renal damage by infectious agents. A 5-year-old female Boerboel was presented because of one week of anorexia. Clinical assessment revealed severe emaciation, dehydration, azotemia, hyperphosphatemia, and concurrent ehrlichiosis. Despite oxytetracycline and doxycycline therapy for two days, the dog died. Necropsy and histopathological examination were performed. Grossly, there was extensive mineralization of the diaphragm, intercostal muscles, pleura, pharynx, and great vessels. Histopathology showed tubular epithelial degeneration, protein casts, tubular ectasia, and interstitial fibrosis, consistent with advanced CKD. Laboratory findings of azotemia, hyperphosphatemia, hypoalbuminemia, and hypercalcemia supported renal failure as the underlying process driving the soft tissue mineralization. Extra-renal lesions included hepatocellular atrophy with vacuolar change, Kupffer cell hyperplasia, bronchointerstitial pneumonia with pulmonary edema, and hemorrhagic follicular cystitis with mononuclear infiltration. The combined gross and histopathological findings demonstrate how renal failure-induced mineral imbalance can drive widespread soft tissue mineralization. Veterinary clinicians must monitor calcium/phosphorus status to anticipate life-threatening sequelae.

To evaluate the diagnostic performance of an artificial intelligence (AI) system for detecting eight abnormal fetal ultrasound findings across cephalic, thoracic, and abdominal regions in routine, unfiltered, multicenter images. We performed a multicenter, retrospective evaluation of an AI software that detects eight abnormal ultrasound findings on still images. Ground truth was established by a multidisciplinary panel (board-certified reviewers with 5 or more years of experience) using a three-step process (view identification, structure visibility, sign presence or absence) with majority consensus. The software evaluated eight findings on six standard views: absence of the cavum septum pellucidum, absence of the corpus callosum, malposition of the great vessels, absence or unusual size of one of the three vessels, disequilibrium or absence of at least one of the two ventricles, thoracic situs inversus, abdominal situs inversus, and nonvisibility of a single stomach bubble or abnormally big stomach. For thoracic and abdominal situs, an evaluability step preceded classification. Primary end points were sensitivity and specificity per finding on evaluable images, with subgroup analyses by geography, device manufacturer, trimester, body mass index (BMI), demographics, anatomy, indication, and finding status. Cluster bootstrap accounted for within-patient clustering; multiplicity was controlled with Bonferroni or Hochberg correction. We analyzed 6,452 images from 1,115 examinations (11-41 weeks of gestation) from approximately 1,000 pregnancies in 942 patients across 75 international sites over five countries; 6,094 images contributed to performance estimates. Mean sensitivity for AI detection was 93.2% (95% CI, 91.6-94.6%) and mean specificity was 90.8% (95% CI, 89.5-92.0%) across the eight findings. Sensitivity was superior to 87% and specificity was superior to 81% for all findings. Abdominal situs inversus had the highest performance (sensitivity 99.3%, 95% CI, 97.6-100%; specificity 99.3%, 95% CI, 98.4-100%). Among thoracic findings, sensitivity was lowest for malposition of the great vessels (87.7%), and specificity was lowest for absence or unusual size of at least one of the three vessels (81.5%). Subgroup performance was generally consistent across manufacturers, regions, BMI categories, and trimesters. In a heterogeneous, multicenter dataset, the software reliably identified predefined ultrasound findings suggestive of congenital malformations. These results support its potential as a real-time assistant to standardize interpretation and to flag suspicious findings.

Modality-AGnostic image Cascade (MAGIC) for multi-modality cardiac substructure segmentation.

Initial Clinical Experience Using 14-fold Accelerated Whole-Chest, Equilibrium Phase Contrast-Enhanced bT1RESS for Evaluation of the Heart and Great Vessels

AI and Virtual Reality for Visualization and Segmentation of Intracardiac Anatomy and Great Vessels from Cardiovascular Magnetic Resonance Images

Ischemic heart disease (IHD) remains the leading cause of morbidity and mortality worldwide, accounting for millions of victims annually. Since the mid-1950s, coronary artery bypass grafting (CABG) performed via a traditional full median sternotomy has served as the gold standard for myocardial revascularization because it provides unsurpassed exposure of the heart and great vessels. However, this conventional approach is frequently characterized by "surgical aggressiveness," which can lead to significant physiological stress, postoperative bleeding, wound infections, and thoracic wall instability. To address these challenges, minimally invasive cardiac surgery (MICS) has emerged as a patient-centered clinical philosophy. These techniques are designed to reduce tissue trauma and mitigate the systemic inflammatory response without compromising the long-term durability of the surgical repair. This review evaluates the clinical, functional, and postoperative outcomes of CABG performed via mini-sternotomy in patients with IHD, drawing on a synthesis of 14 primary research articles. A primary focus is placed on assessing myocardial recovery through intraoperative graft patency measurements and the analysis of cardiac markers. The review further investigates complication rates, with a specific examination of postoperative atrial fibrillation (POAF), bleeding volumes, and the incidence of wound infections compared with traditional surgery. Additionally, this analysis evaluates the efficacy of postoperative rehabilitation by examining mechanical ventilation duration, length of hospital stay, and health-related quality of life, including the management of sharp neuropathic pain associated with minimal-access incisions. By aggregating these results, the review aims to define the feasibility and safety of the mini-sternotomy approach in modern revascularization.

Cardiac manifestations in children with osteogenesis imperfecta: A single-center observational study.

Studies have confirmed that a RFOF (redundant foramen ovale flap) can mimic CoA (coarctation of the aorta) in terms of traditional indicators; however, there is currently no more comprehensive and feasible indicator to differentiate between the two. This study aims to investigate the effect of RFOF on cardiac output, and explore the ability of multiple left cardiac parameters to differentiate RFOF from CoA. This prospective diagnostic cohort study consecutively enrolled fetuses with suspected CoA and normal controls. Comprehensive echocardiographic measurements, including cardiac output and novel morphological parameters-including RCO/LCO (right cardiac output/left cardiac output), the FOFd/LAd (foramen ovale flap/left atrial diameter ratio), aortic arch angle, and LIOA (left ventricular inflow-outflow tract angle)-were performed by operators blinded to the postnatal outcome. ROC (Receiver operating characteristic) curve analysis was performed to evaluate the predictive ability of all indicators for pregnancy outcomes. Analysis revealed that in the RFOF Group, FOFd/LAd had the highest AUC (area under the curve), followed by RCO/LCO. In the CoA Group, the aortic isthmus/ductus arteriosus diameter (Aoi/DA) and aortic arch angle showed the highest AUC values. RFOF can mimic the ultrasonic manifestations of CoA in terms of traditional indicators. FOFd/LAd > 0.65 (AUC = 1.000) is identified as a specific marker for RFOF, while RCO/LCO > 1.60 (AUC = 0.966) provides strong supportive evidence. Conversely, an aortic arch angle > 75.84° receiver operating characteristic (AUC = 0.995) combined with Aoi/DA < 0.75 (AUC = 0.999), suggests a higher probability of CoA. FOFd/LAd, aortic arch angle, and LIOA can help distinguish between RFOF and CoA. What's already known about this topic? The established consensus indicates that RFOF can mimic the sonographic features of CoA prenatally by causing imbalances in traditional parameters of cardiac chamber dimensions and great vessel diameter ratios. However, reliable tools for prenatally distinguishing between these two conditions based on hemodynamic and left heart morphological characteristics remain unavailable. What does this study add? This study adds a new, multiparameter approach that combines hemynamic (RCO/LCO) and novel morphological (FOFd/LAd, Aortic Arch Angle, LIOA) assessments. This approach moves beyond the limitations of traditional ratios and provides clinicians with practical tools to reduce the false positive diagnosis of CoA in fetuses with RFOF.

Background: Neck trauma is a high-risk emergency presentation due to the concentration of vital vascular, aerodigestive, and neurologic structures within a confined anatomical space. Even minor external injuries can conceal life-threatening internal damage, making timely diagnosis and intervention critical. Aim: To review evidence-based strategies for assessment, airway protection, and cervical spine management in acute neck trauma, emphasizing zone-based anatomy and mechanism-specific considerations. Methods: A comprehensive literature synthesis was conducted, integrating current guidelines, epidemiologic data, and clinical decision frameworks such as ATLS principles and NEXUS criteria. The review addresses diagnostic imaging modalities, physical examination priorities, and multidisciplinary management pathways. Results: Neck trauma accounts for 5–10% of trauma cases but carries disproportionate morbidity and mortality. Zone I injuries exhibit the highest lethality due to great vessel involvement and limited surgical access, while Zone II injuries are most common and more accessible for operative intervention. Computed tomography angiography (CTA) has emerged as the preferred initial imaging modality for vascular assessment. Airway compromise remains the foremost threat, often evolving rapidly; early airway control and hemorrhage management are essential. Selective rather than mandatory surgical exploration is increasingly favored, guided by hard and soft signs, imaging, and hemodynamic stability. Conclusion: Optimal outcomes require early airway protection, vigilant reassessment, and multidisciplinary coordination. Advances in imaging and selective intervention strategies have improved safety, but delayed recognition of esophageal or vascular injury remains a major cause of morbidity and mortality.