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Interruption of the ascending aorta is an extremely rare anomaly defined by a point of interruption between the intrapericardial and extrapericardial aorta and can be explained by developmental errors proximal to the embryologic right aortic sac. Herein, we present a case of interruption of the ascending aorta and describe a successful biventricular surgical repair of this unique anomaly.
The optimal surgical management of patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO) is debatable. This is our initial experience with pulmonary root translocation (PRT), a technique that aims to preserve the pulmonary valve function.
From July 2012 to October 2019, 16 patients underwent anatomical repair for TGA, VSD, and LVOTO. PT2977 nmr The median age was 12 months (range 7 months to 13 years), and the median weight was 7.75 kg (range 5.6-29.5 kg). Thirteen patients had a diagnosis of d-TGA and three had congenitally corrected transposition of the great arteries (cc-TGA). The surgical technique involved PRT from the left ventricle (LV) to the right ventricle and routing the LV to the aorta. The left ventricular outflow tract orifice resulting from the pulmonary root extraction was closed with a pericardial patch. In patients with cc-TGA, an atrial switch operation was added. A bidirectional Glenn was necessary in four patients with a long LV to aorta tunnel. One patient required a transannular patch to reconstruct the right ventricular outflow tract (RVOT).
The median follow-up was 27 months. There was one hospital death due to residual mitral regurgitation. One patient died at home four months after hospital discharge. The remaining patients are doing well with adequate RVOT function and no valve regurgitation.
Complete correction of TGA, VSD, and LVOTO using PRT was achieved with acceptable risk in patients with pliable and nondysplastic pulmonary valve. The translocated pulmonary root performed well in this short follow-up.
Complete correction of TGA, VSD, and LVOTO using PRT was achieved with acceptable risk in patients with pliable and nondysplastic pulmonary valve. The translocated pulmonary root performed well in this short follow-up.
In pediatric cardiac surgery, an application of three-dimensional (3D) modeling to develop custom-made prostheses is limited, and currently surgeons use their intraoperative visual estimation to develop 3D complex structures from 2D patch materials. Contemporary 3D designers are developing complex surfaces using surface modeling in other industries, which can be applied to pediatric cardiac surgery. However, its free-form nature may lead to intradesigner variability.
A patient with a body weight of 4 kg with partial anomalous pulmonary venous connection and preoperative computed tomography data was selected, and a patient-specific 3D heart model was obtained. Through collaboration with a pediatric cardiologist and a pediatric cardiac surgeon, a 3D designer developed two patient-specific 3D patches for an intra-atrial rerouting procedure (IAR) for the patient using different methods of surface modeling. The shape and size of two flattened patches were analyzed using a geometric morphometrics (GM) approach. Computational fluid dynamics (CFD) analysis was also performed to calculate pressure drop across streamlines and flow energy loss in the right atrium for both patches.
The GM analysis showed that the size and shape of the two patches around the systemic vein orifice, crucial to prevent systemic venous obstruction, were almost equivalent. However, the CFD analysis showed that the pressure drop and flow energy loss were almost twice for one patch compared with the other.
Our platform of developing a patient-specific 3D patch for an IAR procedure using surface modeling seemed promising, although intradesigner patch variability was not neglectable in our small-sized patient.
Our platform of developing a patient-specific 3D patch for an IAR procedure using surface modeling seemed promising, although intradesigner patch variability was not neglectable in our small-sized patient.
STAT Mortality Categories (developed 2009) stratify congenital heart surgery procedures into groups of increasing mortality risk to characterize case mix of congenital heart surgery providers. This update of the STAT Mortality Score and Categories is empirically based for all procedures and reflects contemporary outcomes.
Cardiovascular surgical operations in the Society of Thoracic Surgeons Congenital Heart Surgery Database (January 1, 2010 - June 30, 2017) were analyzed. In this STAT 2020 Update of the STAT Mortality Score and Categories, the risk associated with a specific combination of procedures was estimated under the assumption that risk is determined by the highest risk individual component procedure. Operations composed of multiple component procedures were eligible for unique STAT Scores when the statistically estimated mortality risk differed from that of the highest risk component procedure. Bayesian modeling accounted for small denominators. Risk estimates were rescaled to STAT 2020 Scores base mix.Surgical repair of Scimitar syndrome is challenging, especially in small patients. Our images demonstrate that the pericardial tunnel technique is feasible even in low-weight patient and that it provides a good growth potential.Introduction Biliary tract cancers (BTCs) [including cholangiocarcinoma and gallbladder cancer] are rare cancers associated with poor survival; most patients have advanced disease at diagnosis. Current chemotherapy reference regimens include cisplatin and gemcitabine as first-line; and oxaliplatin and 5-fluorouracil (FOLFOX) in second-line. Molecular profiling has identified several actionable therapeutic targets including isocitrate dehydrogenase (IDH)1 mutations. Ivosidenib is a reversible inhibitor of mutant IDH1; it is currently approved for the treatment of acute myeloid leukemia and has been studied in patients with advanced cholangiocarcinoma.Areas covered This article introduces current treatments for BTC and sheds light on the mechanism of action, pharmacodynamics, pharmacokinetics, clinical efficacy, and safety of ivosidenib in advanced cholangiocarcinoma. The authors conclude with insights on the changing treatment paradigm created by emerging drugs and precision approaches.Expert opinion Ivosidenib is well tolerated, with good oral exposure and long half-life as shown by phase I data.
Read More: https://www.selleckchem.com/products/pt2977.html
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