Clinical Outcome of Benign Cardiac Tumors in Infants During A 13 Years' Experience: Impact of Prenatal Diagnosis

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LIVER STIFFNESS EVALUATION USING ACOUSTIC RADIATION FORCE IMPULSE ELASTOGRAPHY IN PEDIATRIC AND ADULT PATIENTS WITH CONGENITAL HEART DISEASE

Background: Hepatic complications are common in patients with congenital heart disease as a consequence of the primary cardiac defect or as a result of surgical palliation (e.g. Fontan procedure). Liver involvement represents a significant challenge and an adequate hepatic surveillance is fundamental. Liver biopsy represents the gold standard for diagnosis and staging of hepatic fibrosis but it’s an invasive procedure not suitable for a routine setting. Acoustic radiation force impulse (ARFI) elastography is a recently developed technique that allows to assess hepatic stiffness in a non-invasive and reproducible way. The usefulness of ARFI imaging has been described in adult Fontan patients but only few studies have been reported in the pediatric Fontan population and no one in CHD others than Fontan. Aim: The aims of this study were to assess liver stiffness, using ElastPQTM acoustic radiation force impulse elastography, in pediatric and adult patients with CHD, to compare liver stiffness values with healthy controls and to analyze possible associations between ARFI values and clinical, biochemical, cardiac and hepatic parameters. Materials and methods: Pediatric and adult patients that underwent heart surgery for CHD and were followed at the Cardiology Unit of the “Azienda Ospedaliera Universitaria Integrata” of Verona between October 2018 and October 2020 were prospectively enrolled. Controls subjects without any liver or cardiac disease matched for age and sex to the case group were also included. The latest laboratory tests and echocardiogram available were collected. Liver ultrasound and ARFI measurement of liver stiffness were performed by a specifically trained single expert radiologist using the Philips Healtcare® ultrasound with ElastPQTM software. Results: A total of 50 subjects were enrolled for the study: 20 Fontan patients (13 males, median age at ARFI 8.4 years), 13 non-Fontan (9 males, median age at ARFI 4.8 years) and 17 controls (6 males, median age at ARFI 10 years). The median values of ARFI elastography were significantly higher in patients with CHDs (Fontan and non-Fontan patients) compared to control subjects (p<0.01). Patients with morphological right ventricle overload showed significantly higher results (p=0.02). The cut-off of 5.7 kPa at elastography was used to discriminate between normal liver and liver with signs of congestion or fibrosis. All controls subjects showed ARFI values <5.7 kPa whereas only 25% of Fontan patients and 46% of non-Fontan were below that threshold. Liver stiffness values were positively correlated with time from surgery and age at liver evaluation (p<0.01). The number of platelets and white blood cells were inversely related to liver stiffness measurements (p=0.04 and p=0.05 respectively). The AST to platelet ratio index positively correlated with ARFI elastography results (p<0.03). No significant correlations between ARFI results and other biochemical or cardiac parameters were found. Conclusions: Our data showed that the median values of liver stiffness measured with ElastPQTM pSWE were significantly higher in patients with CHDs compared to control subjects and, in particular, in those with morphological right ventricle overload. Liver stiffness values were also correlated with time from surgery and age at liver evaluation. The number of platelets and white blood cells were inversely related to liver stiffness measurements supporting the need of a screening for portal hypertension and splenomegaly in these patients. The AST to platelet ratio index was also correlated to ARFI elastography results suggesting that liver stiffness may reflect the evolution of liver fibrosis. In conclusion, our study demonstrated, for the first time in literature, that acoustic radiation force impulse elastography (pSWE) with ElastPQTM software can be a useful tool to assess liver stiffness in patients with Fontan circulation and other congenital heart disease

Familial fetal-type rhabdomyoma of the tricuspid valve in the neonate: Malignant course for a benign disease

Familial fetal-type rhabdomyoma of the tricuspid valve in the neonate: malignant course for a benign disease

The Ross–Yacoub procedure for aneurysmal autograft roots: A strategy to preserve autologous pulmonary valves

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Repair of quadricuspid aortic valve by bicuspidization: a novel technique

Quadricuspid aortic valve (QAV) is a rare congenital lesion, generally manifesting with valve regurgitation. Standard treatment involves valve replacement, though anecdotal cases of successful repair by means of valve tricuspidization have been reported. Here, the successful application of a repair technique previously unreported in the setting of QAV is described

The ST2/IL-33 Pathway in Adult and Paediatric Heart Disease and Transplantation

ST2 is a member of interleukin 1 receptor family with soluble sST2 and transmembrane ST2L isoforms. The ligand of ST2 is IL-33, which determines the activation of numerous intracytoplasmic mediators following the binding with ST2L and IL-1RAcP, leading to nuclear signal and cardiovascular effect. Differently, sST2 is released in the blood and works as a decoy receptor, binding IL-33 and blocking IL-33/ST2L interaction. sST2 is mainly involved in maintaining homeostasis and/or alterations of different tissues, as counterbalance/activation of IL-33/ST2L axis is typically involved in the development of fibrosis, tissue damage, inflammation and remodeling. sST2 has been described in different clinical reports as a fundamental prognostic marker in patients with cardiovascular disease, as well as marker for the treatment monitoring of patients with heart failure; however, further studies are needed to better elucidate its role. In this review we reported the current knowledge about its role in coronary artery disease, heart failure, heart transplantation, heart valve disease, pulmonary arterial hypertension, and cardiovascular interventions

An Image-Based Computational Fluid Dynamics Study of Mitral Regurgitation in Presence of Prolapse

Purpose: In this work we performed an imaged-based computational study of the systolic fluid dynamics in presence of mitral valve regurgitation (MVR). In particular, we compared healthy and different regurgitant scenarios with the aim of quantifying different hemodynamic quantities. Methods: We performed computational fluid dynamic (CFD) simulations in the left ventricle, left atrium and aortic root, with a resistive immersed method, a turbulence model, and with imposed systolic wall motion reconstructed from Cine-MRI images, which allowed us to segment also the mitral valve. For the regurgitant scenarios we considered an increase of the heart rate and a dilation of the left ventricle. Results: Our results highlighted that MVR gave rise to regurgitant jets through the mitral orifice impinging against the atrial walls and scratching against the mitral valve leading to high values of wall shear stresses (WSSs) with respect to the healthy case. Conclusion: CFD with prescribed wall motion and immersed mitral valve revealed to be an effective tool to quantitatively describe hemodynamics in case of MVR and to compare different regurgitant scenarios. Our findings highlighted in particular the presence of transition to turbulence in the atrium and allowed us to quantify some important cardiac indices such as cardiac output and WSS

Ten-Year Experience with Endomyocardial Biopsy after Orthotopic Heart Transplantation: Comparison between Trans-Jugular and Trans-Femoral Approach

Background: Endomyocardial biopsy (EMB) is considered the gold standard for monitoring allograft rejection after heart transplantation. EMB is an invasive procedure that may be performed via a trans-jugular or a trans-femoral approach with a complication rate reported as less than 6%. The aim of this study was to evaluate the complication rate after EMBs in heart recipients and to compare the results of EMBs performed via a trans-jugular or a trans-femoral approach. Methods: Medical records of heart recipients undergoing EMBs between January 2012 and December 2022 were retrospectively reviewed. EMB-related complications were classified as major (death, pericardial effusion, hemopericardium, cardiac tamponade requiring a pericardiocentesis or an urgent cardiac surgery, ventricular arrythmias, permanent atrio-ventricular block requiring permanent pacing, hemothorax, pneumothorax and retroperitoneal bleeding) and minor (de novo tricuspid regurgitation, arrhythmias, coronary artery fistula, vascular access site complications). Results: A total of 1698 EMBs were performed during the study period at our institution in 212 heart recipients. There were 927 (55%) EMBs performed through a trans-jugular approach (TJ group) and 771 (45%) EMBs performed through a trans-femoral approach (TF group). A total of 60 (3.5%) complications were recorded, including nine (0.5%) major complications (six cardiac tamponades, two pneumothorax and one retroperitoneal bleeding) and 51 (3%) minor complications (seven coronary fistulae, five de novo tricuspid regurgitation, four supraventricular arrythmias and thirty-five vascular access site complications). No difference was found in total (38 [4%] vs. 22 [3%]; p = 0.16) and major (6 [1%} vs. 3 [0.4%]; p = 0.65) complications (32 [3%] vs. 19 [2%]; p = 0.23) between the TJ group and the TF group. No difference was found in male sex, age at time of EMB and time from HT between complicated and not complicated EMBs. Conclusions: EMBs represent a safe procedure with a low risk of complications. In our experience, EMBs performed via a trans-jugular approach are as safe as the trans-femoral approach

Turbulent blood dynamics in the left heart in the presence of mitral regurgitation: a computational study based on multi-series cine-MRI

In this work, we performed a computational image-based study of blood dynamics in the whole left heart, both in a healthy subject and in a patient with mitral valve regurgitation. We elaborated multi-series cine-MRI with the aim of reconstructing the geometry and the corresponding motion of left ventricle, left atrium, mitral and aortic valves, and aortic root of the subjects. This allowed us to prescribe such motion to computational blood dynamics simulations where, for the frst time, the whole left heart motion of the subject is considered, allowing us to obtain reliable subject-specifc information. The fnal aim is to investigate and compare between the subjects the occurrence of turbulence and the risk of hemolysis and of thrombi formation. In particular, we modeled blood with the Navier–Stokes equations in the arbitrary Lagrangian–Eulerian framework, with a large eddy simulation model to describe the transition to turbulence and a resistive method to manage the valve dynamics, and we used a fnite element discretization implemented in an in-house code for the numerical solution

An alternative method for neonatal cerebro-myocardial perfusion.

Several techniques have already been described for selective cerebral perfusion during repair of aortic arch pathology in children. One method combining cerebral with myocardial perfusion has also been proposed. A novel technique is reported here for selective and independent cerebro-myocardial perfusion for neonatal and infant arch surgery. Technical aspects and potential advantages are discussed