Project description:Treatment of multiple penetrating aortic ulcers becomes troublesome if they involve the aortic arch. We report a percutaneous aortic repair using a unibody design endoprosthesis with a precannulated side component for the left subclavian artery to manage a large aortic arch ulcer and preserve the patency of previous coronary artery bypass graft. (Level of Difficulty: Advanced.).
Project description:Interstitial deletion of 6q21-22 has been previously reported in 11 individuals, who presented with intellectual disability, facial dysmorphism, cardiac abnormality, cerebellar hypoplasia and dysplasia of the corpus callosum. Here, we report the first instance of a patient with 6q21-22 deletion presenting with interrupted aortic arch in addition to the previously described clinical signs. Array analysis using Agilent Human genome CGH 180K identified a 13.3-Mb deletion at 6q21-q22.31 (nt. 109885195-123209593).
Project description:Persistent 5th aortic arch is a rare cardiac anomaly that is usually surgically corrected during infancy or early childhood if it is associated with coarctation of the aorta. Here, we report an adult with coarctation of the 5th aortic arch who was successfully treated by stent implantation. An asymptomatic 32-year-old woman presented with hypertension and a significant arm-leg difference in pressure. On suspicion of coarctation of the aorta, a chest computed tomography was performed, leading to a diagnosis of an interrupted 4th aortic arch with coarctation of a persistent 5th aortic arch. Percutaneous catheter intervention using a PALMAZ large stent dilated to 12 mm resulted in a minimal peak-to-peak pressure gradient. The patient was discharged home after a 2-day monitoring without hypertension and arm-leg blood pressure difference. She remained normotensive with a patent aortic arch on echocardiography performed 10 months after treatment. As for simple coarctation of the aorta, stent implantation was feasible and effective in an adult patient with coarctation of the 5th aortic arch.
Project description:We report a patient with cat eye syndrome and interrupted aortic arch type B, a typical finding in the 22q11.2 deletion syndrome. Chromosomal analysis and fluorescent in situ hybridization (FISH) showed a supernumerary bisatellited isodicentric marker chromosome derived from chromosome 22. The segment from 22pter to 22q11.2 in the supernumerary chromosome found in our patient does not overlap with the region deleted in patients with the 22q11.2 deletion syndrome. However, the finding of an interrupted aortic arch type B is unusual in CES, although it is a frequent heart defect in the 22q11 deletion syndrome.
Project description:Background:The purpose of this study was to investigate the correlation of the extent of aortic arch calcification (AAC) detectable on chest X-rays with the severity of coronary artery disease (CAD) as evaluated by the SYNTAX score (SS) in patients with acute coronary syndrome (ACS). Methods:A total of 1,418 patients (344 women; 59 ± 10 years) who underwent coronary angiography for ACS and were treated with coronary revascularization were included in the present study; chest X-rays were performed on admission. The AAC extent was divided into four grades (0-3). SS was calculated based on each patient's coronary angiographic findings. The relationship between the AAC extent and SS was assessed. Results:The AAC extent was positively correlated with SS (? = 0.639, P < 0.001). In the multivariate analysis, compared with grade 0, odds ratios (ORs) of AAC grades 1, 2, and 3 in predicting SS >22 were 12.95 (95% CI, 7.85-21.36), 191.76 (95% CI, 103.17-356.43), and 527.81 (95% CI, 198.24-1405.28), respectively. Receiver operating characteristic curve analysis yielded a strong predictive ability of the AAC extent for SS >22 (area under curve = 0.840, P < 0.001). Absence of AAC had a sensitivity, specificity, positive prognostic value, negative prognostic value, and accuracy of 46.7%, 95.9%, 94.1%, 56.4%, and 67.3%, respectively, for SS ?22. AAC grades ?2 had a sensitivity of 66.3%, specificity of 89.2%, positive prognostic value of 81.5%, negative prognostic value of 78.6%, and accuracy of 79.6% for the correct identification of SS >22. Conclusions:The extent of AAC detectable on chest X-rays might provide valuable information in predicting CAD severity in ACS patients.
Project description:Left ventricular outflow tract obstruction is an important complication after interrupted aortic arch repair and subsequent interventions may adversely affect survival. Identification of patients at risk for obstruction is important to facilitate clinical decision-making and monitoring during follow-up. The aim of this review is to summarize reported risk factors for left ventricular outflow tract obstruction after corrective surgery for interrupted aortic arch. A systematic search of the literature was performed across the PubMed and EMBASE databases. Studies that reported echocardiographic and/or clinical predictors for left ventricular outflow tract obstruction in infants that underwent biventricular repair of interrupted aortic arch were included. From the 44 potentially relevant studies, eight studies met the inclusion criteria. Postoperative left ventricular outflow tract obstruction requiring an intervention was common, with an incidence ranging between 14 and 38%. Manifestation of postoperative left ventricular outflow tract obstruction was associated with a smaller pre-operative size of the aortic root (sinus of Valsalva), sinotubular junction, and aortic annulus. Anatomic and surgical risk factors for left ventricular outflow tract obstruction were the presence of an aberrant right subclavian artery, use of a pulmonary homograft or polytetrafluoroethylene interposition graft for aortic arch repair, and the presence of a small- or medium-sized ventricular septal defect. In patients with a borderline left ventricular outflow tract that undergo a primary repair, these (pre-) operative predictors can provide guidance for optimal surgical decision-making and for close monitoring during follow-up of patients at increased risk for developing left ventricular outflow tract obstruction after corrective surgery.
Project description:Here, we report a fetus with a rare aortic arch anomaly with left aortic arch and right ductus arteriosus, which has not been reported so far. In this condition, the aorta extends to the left of the trachea as in normal, while the ductus arteriosus extends to the right of the trachea and joins the descending aorta posterior to the trachea, with a cross-ribbon sign.
Project description:We report a 6-month-old female infant with deletion of chromosome 22q11.2 (DiGeorge/VFS TUPLE 1), normal atrial arrangement with concordant atrioventricular connection, pulmonary atresia, large subaortic ventricular septal defect, diminutive native pulmonary arteries, a characteristic weird-shape right aortic arch with arch-on-arch appearance and figure of 8 configuration. We presented the cardiac computed tomographic angiographic and cardiac angiographic features. Using Autodesk 3ds Max 2018 software, we explained and illustrated the speculative embryologic etiology of this bizarre aortic archanomaly with the extensive abnormal remodeling of the left brachiocephalic artery, based on a "five-embryonic aortic arches" concept. As to the best of the authors' knowledge, this is the first report of a genetically confirmed case of DiGeorge syndrome and an exceedingly rare type of right aortic arch anomaly with embryologic explanation according to the "five-embryonic-aortic-arches" concept. It seems that the constellation of pulmonary atresia, bizarreshaped right aortic arch due to abnormal development of the aortic sac, and abnormal remodeling of the left brachiocephalic artery may be strongly suggestive of DiGeorge syndrome.