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The aim of this study was to compare the size and geometry of the aorta in patients with 7q11.23 duplication (Dup7) to healthy controls. We retrospectively reviewed all echocardiograms in all patients with Dup7 evaluated at our institutions from June 2017 through September 2019. All standard aortic diameter measurements were made and recorded. Z-scores for the measurements were calculated. For comparison, a set of control echocardiograms was developed by randomly selecting 24 normal echocardiograms in age-matched patients who had undergone echocardiograms for an indication of either chest pain or syncope. In 58 echocardiograms from 21 Dup7 patients, all aortic measurements were increased compared to controls (p

A bovine arch is the most common aortic arch variant, characterized by a common origin of the innominate artery and the left common carotid artery. Data have shown that children with bovine arch anatomy and coarctation are at a significantly higher risk of recoarctation following coarctation repair. This study aims to explain the higher coarctation rates, assess the branching of the arch vessels, understand their embryologic origins, and delineate the patterns of displacement of the arch vessels in bovine versus normal anatomy. This retrospective study reviewed the medical records of 178 infants (

Progressive dilatation of the pulmonary autograft is one of the greatest concerns after the Ross procedure. Increased stress in the arterial wall may cause changes in the elastic properties of the pulmonary autograft, and thus lead to pathological dilatation. The present study aimed to investigate the changes in the autograft diameter and stiffness during follow-up after the Ross procedure. A total of ten patients underwent the Ross procedure at our institution between 2003 and 2011. Echocardiography was used to measure the diameters of the pulmonary autograft at the level of the annulus, sinus of Valsalva, and sinotubular junction. The stiffness index was calculated from the angiographic data, and compared with that of 16 age-matched control children. The diameters of the pulmonary autograft increased throughout the follow-up period, particularly at the level of the sinus of Valsalva and at the sinotubular junction. The aortic root was stiffer in Ross patients compared with control children (7.9 ± 1.8 vs. 3.9 ± 0.7 immediately postoperatively, p

We compared differences in the hemodynamic parameters of multiple surgical techniques for supravalvular aortic stenosis (SVAS). A three-dimensional model was reconstructed based on a patient’s CT scan. Virtual McGoon, Doty, and Brom repairs were completed using computer-aided design (CAD). Hemodynamic parameters were calculated through computational fluid dynamics (CFD). The velocity profile and wall shear stress (WSS) showed the blood flow pattern. Energy loss (EL) and energy efficiency (EE) were calculated to estimate the cardiac workload. The perioperative blood flow ratio (BFR) of brachiocephalic vessels and coronary arteries was calculated. The preoperative flow velocity was abnormally high (> 5.0 m/s). High WSS was detected at the sinotubular junction (STJ), and its preoperative distribution in the aorta was uneven. High-speed flow disappeared after each of the three operations. The WSS distribution at the aortic root was consistent with the postoperative STJ structure of each operation. EL in the systolic phase decreased postoperatively (Original: 634 mW, McGoon: 218 mW, Doty: 278 mW, Brom: 255 mW). No significant difference in brachiocephalic BFR was detected among the different techniques. A slightly increased coronary BFR (Original: 7.56%, McGoon: 7.99%, Doty: 8.55%, Brom: 8.89%) was detected. McGoon, Doty, and Brom repair each effectively restored stable blood flow and greatly improved EE. The best WSS distribution and coronary blood supply were achieved after Brom repair due to its ability to reconstruct the symmetrical aortic root structure. CFD combined with a virtual operation is a promising method in surgical planning and optimization for SVAS.

Few data exist regarding predictors of rapid aortic root dilation and referral for aortic surgery in Marfan syndrome (MFS). To identify independent predictors of the rate of aortic root (AoR) dilation and referral for aortic surgery, we investigated the data from the Pediatric Heart Network randomized trial of atenolol versus losartan in young patients with MFS. Data were analyzed from the echocardiograms at 0, 12, 24, and 36 months read in the core laboratory of 608 trial subjects, aged 6 months to 25 years, who met original Ghent criteria and had an AoR z-score (AoRz) > 3. Repeated measures linear and logistic regressions were used to determine multivariable predictors of AoR dilation. Receiver operator characteristic curves were used to determine cut-points in AoR dilation predicting referral for aortic surgery. Multivariable analysis showed rapid AoR dilation as defined by change in AoRz/year > 90th percentile was associated with older age, higher sinotubular junction z-score, and atenolol use (R2 = 0.01) or by change in AoR diameter (AoRd)/year > 90th percentile with higher sinotubular junction z-score and non-white race (R2 = 0.02). Referral for aortic root surgery was associated with higher AoRd, higher ascending aorta z-score, and higher sinotubular junction diameter:ascending aorta diameter ratio (R2 = 0.17). Change in AoRz of 0.72 SD units/year had 42% sensitivity and 92% specificity and change in AoRd of 0.34 cm/year had 38% sensitivity and 95% specificity for predicting referral for aortic surgery. In this cohort of young patients with MFS, no new robust predictors of rapid AoR dilation or referral for aortic root surgery were identified. Further investigation may determine whether generalized proximal aortic dilation and effacement of the sinotubular junction will allow for better risk stratification. Rate of AoR dilation cut-points had high specificity, but low sensitivity for predicting referral for aortic surgery, limiting their clinical use. Clinical Trial Number ClinicalTrials.gov number, NCT00429364.

This report describes a rare case of complete transposition of the great arteries (TGA) with an intramural right coronary artery arising well above the sinotubular junction. Additionally in this case, the left circumflex artery arose anomalously as a branch from the right coronary. Identification of this anatomic anomaly was made by transthoracic echocardiography before surgery. The patient underwent a successful arterial switch operation on day 6 of life. Due to the preoperative diagnosis of the unusual right intramural coronary artery, the coronary transfer technique during the arterial switch was altered, allowing preservation of the right coronary artery.

The objectives of this study were to investigate left ventricular (LV) function, aortic dilation, and atherosclerosis in children with mildly deteriorated isolated bicuspid aortic valve (BAV) function using echocardiographic studies and biochemical markers of atherosclerosis and to correlate results with normal children. Biochemical analyses indicating cardiovascular risk of atherosclerosis and vascular changes in the aorta in relation to BAV were performed in 41 children aged 5-15 years old with isolated BAV and in 25 children with tricuspid aortic valves. Evaluations of aortic valve structures and functions; examinations of the LV M-mode and ascending aorta Doppler; and measurements of the LV Tei index (MPI), propagation velocity, ascending aorta at four levels, and carotid intima-media thickness (CIMT) were performed. There were no statistically significant differences in CIMTs, plasma matrix metalloproteinase-9, tissue metalloproteinase inhibitor-1 levels, or other biochemical parameters indicating cardiovascular risk or atherosclerosis between study and control groups. Deterioration of LV function, which could not be seen with M-mode echocardiography, was evident by MPI. MPI values in the study versus control groups were 0.46 +/- A 0.080 versus 0.40 +/- A 0.086 (p < 0.05). Diameters of the aorta in the study and control groups were 19.7 +/- A 4.7 and 17.2 +/- A 2.8 mm (p < 0.05) at the sinotubular junction level and 20.6 (14.4-40.5) and 18.3 (12.4-24) mm at the ascending aorta level (p < 0.05). Increased aortic valve insufficiency was related to increased aortic diameter. No sign of atherosclerosis was detected in children with BAV. Deterioration of LV function was seen using MPI, and aortic dilation was related to the severity of aortic valve insufficiency.

Pulmonary valve autografts have proven to be valid aortic valve substitutes in children. The objective of this study is to evaluate the performance of the pulmonary autograft (PAG) in systemic position and its adjustment to growth in four infants who underwent a Ross operation. Between April 1998 and January 2000, four infants whose aortic valve anatomy and function were judged unsuitable for a valve-sparing operation underwent successful Ross operation. All patients were followed postoperatively with echocardiograms to evaluate the function and the dimensional changes of the PAG within the systemic circulation. The PAG adjustments to growth were compared to the growth of the pulmonary root of healthy patients matched for body surface area, with the purpose to evaluate the effects of the systemic pressure on the pulmonary vascular wall, as a passive dilatation process versus a true and active structural adaptation. Median follow-up was 53 months (range, 48-65). There were no early deaths or reoperations. All patients were asymptomatic and in stable hemodynamic conditions. The PAG annulus grew according to body surface area without dilatation. PAG valve regurgitation was present but mild in three patients. Trans-PAG peak gradients were not significant. The PAG diameter at the level of the sinuses of Valsalva and sinotubular junction increased rapidly and steadily in all patients during follow-up compared to controls. The Ross operation in infants provides acceptable immediate surgical and hemodynamic results. However, significant midterm increases in PAG sinuses and sinotubular junction diameters require close and continued observation.

To describe great-vessel dimensions in patients with D-loop transposition of the great arteries (TGA) who have undergone atrial switch operation (ATSO). Patients who have undergone arterial switch operation for TGA have a high incidence of dilation of the neoaortic root. The incidence and degree of great artery dilation in patients who have undergone ATSO for TGA has not previously been described. A retrospective database review identified patients with TGA and intact ventricular septum who underwent ATSO at