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: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:We report stenting of coarctation of an angulated right circumflex aortic arch (RCAA) using four Cheatham Platinum stents in a child with Goldenhar syndrome. Difficulties in measuring the accurate length of the curved and narrowed transverse aortic arch marked discrepancy between the luminal diameters of the long narrow transverse arch and wide descending thoracic aorta, increased displacement force caused by the 90° bend between the two parts resulted in repeated stent migrations. We discuss the tips to avoid distal stent migration in the setting of an angulated RCAA.
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:Distal aortic complications from acute DeBakey I dissection repair are an important source of late morbidity and mortality. We present an early experience of using a novel single-branched thoracic aortic endograft in conjunction with open techniques to treat acute DeBakey I aortic dissection.The patients in this series include five hyperacute dissections managed with a combined zone 2 partial arch replacement and placement of a zone 2 single subclavian branch endograft.There were no perioperative mortalities, strokes, or spinal cord ischemia in any patients at either stage of the procedure. At follow-up imaging, no patients had anterograde flow into the false lumen. All patients experienced false lumen thrombosis in the stented portion of the aorta.This combination of open repair techniques and the use of a novel branched endograft resulted in excellent early outcomes in this pioneer series. Further investigation of these techniques in a prospective fashion is warranted.