Project description:Persistent fifth aortic arch (PFAA) is a rare congenital cardiovascular anomaly of the aortic arch, which can occur independently but is frequently associated with other cardiovascular malformations including patent ductus arteriosus (PDA), interrupted aortic arch (IAA), pulmonary atresia, tetralogy of Fallot, and transposition of the great vessels. PFAA can be classified into three different types according to different abnormal vascular connections (type A, B and C). We report an infant diagnosed with PFAA along with interrupted fourth aortic arch (type B) and PDA. Several surgical methods have been delivered to patients diagnosed with PFAA. In our case, the correction of coarctation PFAA and the ligation of PDA were performed without cardiopulmonary bypass through left lateral thoracotomy. We removed the coarctation part of the PFAA, and then performed the end-to-end anastomosis between the fifth aortic arch and the descending aorta. Despite the stenosis at the site of anastomosis was observed 10 months after the operation, the patient resulted in good recovery by once balloon dilation procedure. Thus, we recommend it is more appropriable to select this procedure in the surgical treatment of PFAA for those patients whose fourth aortic arches were interrupted with fifth aortic arches well developed. Our experience can provide a beneficial reference for future cases.
Project description:BackgroundPersistent fifth aortic arch (PFAA) is a rare anomaly often associated with aortic coarctation or interruption, and various surgical techniques for this anomaly have been reported. Herein, we show a case of an infant with PFAA and severe aortic coarctation.Case presentationA 41-day-old female infant was admitted for sustained fever. Initially, the patient was diagnosed with bacterial meningitis, and echocardiography showed PFAA with severe aortic coarctation. Because the patient presented progressive oliguria and metabolic acidosis, she was transferred for emergency cardiac surgical intervention. The aortic arch was reconstructed using end-to-side anastomosis between the fifth aortic arch and the descending aorta without any artificial conduit or patching material.ConclusionsPFAA with aortic coarctation can be repaired by various surgical methods. Among them, our surgical approach is easy and effective, has growth potential, and an additional surgery is not needed.
Project description:The double-barreled aorta connecting the ascending aorta and descending aorta caudal to the normal fourth aortic arch has fascinated the interests of cardiac morphologists for over a century. This condition is commonly associated with coarctation. While the controversies surrounding the embryology of the double-barreled aorta have settled down, we present a case-based illustration of the technical aspects of coarctation stenting peculiar to this condition.
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:ObjectiveSurgery for aortic coarctation repair provides excellent hemodynamic results but may be complicated by musculoskeletal issues. The purpose of the study was to determine the midterm results of a muscle-sparing surgical approach to aortic coarctation repair, with special emphasis on the repair and on the musculoskeletal changes associated with a posterior thoracotomy.MethodsWe included all children with aortic coarctation operated on with our minimally invasive approach between June 2002 and October 2004, with a follow-up of ≥4.5 years. Patients were assessed clinically and echocardiographically. The spine, left chest, and shoulder were assessed clinically and radiographically.ResultsThirty-one children were included. The age at operation ranged from 1 day to 15 months and weight ranged from 980 g to 10 kg. All patients underwent an extended end-to-end anastomosis coarctation repair through a minimal (n = 19) or total-muscle sparing (n = 12) or extrapleural (n = 18) approach. Five patients had an additional enlargement procedure on the aortic arch. 27 patients had no residual or recurrent gradient. Four patients exhibited restenosis, for which 1 underwent a percutaneous angioplasty and 2 underwent surgical reintervention. All patients were free of hypertension. One patient had borderline values. The musculoskeletal assessment was normal in all but 3 patients. Two patients who underwent other subsequent thoracic surgeries developed thoracogenic scoliosis of moderate severity. A third patient had a left winged scapula. No rib fusion or intercostal space enlargement was found.ConclusionsCompared with a conventional approach, our minimally invasive surgical approach led to excellent musculoskeletal outcomes without compromising the hemodynamic results.
Project description:Objectives(i) To monitor cerebral blood flow velocity (CBFv) throughout aortic arch repair surgery and during the recovery period. (ii) To examine the relationship between transcranial doppler ultrasound (TCD) and near-infrared spectroscopy (NIRS) during cardiac surgery. (iii) To examine CBFv in patients cooled to 20°C and 25°C.MethodsDuring aortic arch repair and after surgery, measurements of TCD, NIRS, blood pH, pO2, pCO2, HCO3, lactate, Hb, haematocrit (%) and temperature (core and rectal) were recorded in 24 neonates. General linear mixed models were used to examine differences over time and between two cooling temperatures. Repeated measures correlations were used to determine the relationship between TCD and NIRS.ResultsCBFv changed during arch repair (main effect of time: P = 0.001). During cooling, CBFv increased by 10.0 cm/s (5.97, 17.7) compared to normothermia (P = 0.019). Once recovering in paediatric intensive care unit (PICU), CBFv had increased from the preoperative measurement by 6.2 cm/s (0.21, 13.4; P = 0.045). CBFv changes were similar between patients cooled to 20°C and 25°C (main effect of temperature: P = 0.22). Repeated measures correlations (rmcorr) identified a statistically significant but weak positive correlation between CBFv and NIRS (r = 0.25, P≤0.001).ConclusionsOur data suggested that CBFv changed throughout aortic arch repair and was higher during the cooling period. A weak relationship was found between NIRS and TCD. Overall, these findings could provide clinicians with information on how to optimise long-term cerebrovascular health.
Project description:Coarctation of the aorta is a form of left ventricular outflow tract obstruction in paediatric patients that can be presented with either bicuspid (BAV) or normal tricuspid (TAV) aortic valve. The congenital BAV is associated with hemodynamic changes and can therefore trigger different molecular remodelling in the coarctation area. This study investigated the proteomic and phosphoproteomic changes associated with BAV for the first time in neonatal coarctation patients. Aortic tissue was collected just proximal to the coarctation site from 23 neonates (BAV; n = 10, TAV; n = 13) that were matched for age (age range 4-22 days). Tissue from half of the patients was frozen and used for proteomic and phosphoproteomic analysis whilst the remaining tissue was formalin fixed and used for analysis of elastin content using Elastic Van-Gieson (EVG) staining. A total of 1796 protein and 75 phosphoprotein accession numbers were detected, of which 34 proteins and one phosphoprotein (SSH3) were differentially expressed in BAV patients compared to TAV patients. Ingenuity Pathway Analysis identified the formation of elastin fibres as a significantly enriched function (p = 1.12 × 10-4) due to the upregulation of EMILIN-1 and the downregulation of TNXB. Analysis of paraffin sections stained with EVG demonstrated increased elastin content in BAV patients. The proteomic/phosphoproteomic analysis also suggested changes in inositol signalling pathways and reduced expression of the antioxidant SOD3. This work demonstrates for the first time that coarcted aortic tissue in neonatal BAV patients has an altered proteome/phosphoproteome consistent with observed structural vascular changes when compared to TAV patients.
Project description:BackgroundManagement of an adult patient with aortic coarctation and an associated cardiac pathology poses a great surgical challenge since there are no standard guidelines for the therapy of such complex pathology. Debate exists not only on which lesion should be corrected first, but also upon the type and timing of the procedure. Surgery can be one- or two-staged. Both of these strategies are accomplice with elevate morbidity and mortality.Case reportIn the face of such an extended surgical approach, balloon dilatation seems preferable for treatment of severe aortic coarctation.We present an adult male patient with aortic coarctation combined with ascending aorta aneurysm and concomitant aortic valve regurgitation. The aortic coarctation was corrected first, using percutaneous balloon dilatation; and in a second stage the aortic regurgitation and ascending aorta aneurysm was treated by Bentall procedure. The patients' postoperative period was uneventful. Three years after the operation he continues to do well.
Project description:ObjectivesThe surgical treatment of coarctation of aorta with hypoplastic aortic arch (CoA/HAA) was challenging to achieve long-lasting arch patency. We reviewed early and late outcomes in our centre and identified predictors for arch reobstruction.MethodsA retrospective analysis of medical records was performed to identify CoA/HAA patients who underwent primary arch reconstruction via median sternotomy between 2011 and 2020. Preoperative aortic arch geometry was analysed with cardiac computed tomographic angiography. Bedside flexible fibre-optic bronchoscopy was routinely performed after surgery in intensive care unit.ResultsThere were 104 consecutive patients (median age 39.5 days) who underwent extended end-to-end anastomosis, extended end-to-side anastomosis and autograft patch augmentation. Early mortality was 3.8% and overall survival was 94.1% [95% confidence interval (CI) 89.6-98.8%] at 1, 3 and 5 years. Reobstruction-free survival was 85.1% (95% CI 78.4-92.3%) at 1 year, 80.6% (95% CI 73.1-88.9%) at 3 years and 77.4% (95% CI 69.2-86.6%) at 5 years. Preoperative aortic arch geometric parameters were not important factors for reobstruction. Nineteen patients (18.3%) were detected with left main bronchus compression (LMBC) on flexible fibre-optic bronchoscopy. Cardiopulmonary bypass time [P < 0.001, hazard ratio (95% CI): 1.02 (1.01-1.03)] and postoperative LMBC [P = 0.034, hazard ratio (95% CI): 2.99 (1.09-8.23)] were independent predictive factors on multivariable Cox regression analysis of reobstruction-free survival.ConclusionsAortic arch can be satisfactorily repaired by extended end-to-end anastomosis, extended end-to-side anastomosis and autograft patch augmentation via median sternotomy in CoA/HAA. Cardiopulmonary bypass time and postoperative LMBC detected by flexible fibre-optic bronchoscopy are significant predictors for long-term arch reobstruction.