A 4-day-old girl with Posterior fossa anomalies, Haemangiomas of the head and neck, Arterial, Cardiovascular, and Eye anomalies and ventral developmental defect syndrome comprising a facial haemangioma, aortic coarctation at the aortic arch, torturous aortic aneurysm distal to coarctation, and ductus arteriosus originating proximal to the coarctation is presented. The aortic arch was successfully reconstructed without cardiopulmonary bypass, and she is currently doing well after 4 years and 8 months.

We present a 5-year-old child with pulmonary atresia palliated at infancy with midline shunt now for complete repair with aneurysmally dilated aorta. Patient underwent a combined Rastelli procedure with supra-coronary ascending aorta replacement with hemi-arch repair. Patient was discharged on post-operative day 10 and was asymptomatic on follow-up.

We reported a successful case of thoracic endovascular aortic repair of a late aortic aneurysm after patch repair of an interrupted aortic arch and favourable outcome 4 years after the procedure.

We report a case of subarachnoid haemorrhage resulting from a mycotic aortic aneurysm in a child with CHD. The patient previously underwent operations for CHD and had a subarachnoid haemorrhage of unknown cause before the scheduled re-operation. During the re-operation, a sealed rupture of an undiagnosed mycotic ascending aortic aneurysm was identified, and the causative organism was later identified as Streptococcus. A postoperative MRI indicated a partially thrombosed cerebral aneurysm. This case demonstrates that a mycotic aortic aneurysm can be a cause of intracranial haemorrhage in children.

Sudden death from aortic dissection of an ascending aortic aneurysm is an uncommon but important finding in all series of sudden death in young, apparently healthy athletes. Individuals at risk include those having any of a variety of conditions in which structural weakness of the ascending aorta predisposes to pathological dilation under prolonged periods of increased wall stress. These conditions include Marfan syndrome, Loeys–Dietz syndrome, bicuspid aortic valve, and the vascular form of Ehlers–Danlos syndrome. Diagnostic criteria, surveillance strategies, medical management, and surgical indications are discussed. Finally, the current recommendations for sports participation are provided.

Arterial diseases such as aneurysm and stenosis may result from the mechanical and/or morphological change of an arterial wall structure and correspondingly altered hemodynamics. The development of a 3D computational model of blood flow can be useful to study the hemodynamics in major blood vessels and may provide an insight into the noninvasive technique to detect arterial diseases in early stage. In this paper, we present a three-dimensional model of blood flow in the aorta, which is based on the immersed boundary method to describe the interaction of blood flow with the aortic wall. Our simulation results show that the hysteresis loop is evident in the pressure-diameter relationship of the normal aorta when the arterial wall is considered to be viscoelastic. In addition, it is shown that flow patterns and pressure distributions are altered in response to the change of aortic morphology.

Loeys–Dietz syndrome is a newly described entity characterised by a constellation of arterial tortuosity, cranial malformations, and hypertelorism. We report a case of a 7-year old boy with confirmed Loeys–Dietz syndrome and discuss magnetic resonance imaging as a complete technique for assessment and follow-up of aggressive vascular pathology in the brain, thorax, and abdomen, which may dictate early surgical intervention.

Marfan's syndrome is an inherited disorder of connective tissue, caused by mutations in the fibrillin-1 gene located on chromosome 15. Diagnosis is still based on a combination of major and minor clinical features. Prognosis is mainly determined by the cardiovascular complications. Advances in surgical and medical treatment for these complications have dramatically improved the prognosis of the syndrome.

An hydropic fetus seen at 28-weeks gestation had a saccular aortic aneurysm in the descending thoracic aorta. Histology disclosed marked fibrointimal hyperplasia, thrombus, and attenuation of the tunica media. The remainder of the descending thoracic aorta showed fibrointimal hyperplasia. We speculate that the narrowed lumen and rigid aortic wall resulting from this vasculopathy provided an increased afterload leading to cardiac failure.

Objectives: A systematic review of the cost-effectiveness of abdominal aortic aneurysm (AAA) repair was conducted. Although open surgery has been considered the gold standard for prevention of AAA rupture, emerging less-invasive endovascular treatments have led to increased interest in evaluating the cost and cost-effectiveness of treatment options.

Methods: A systematic review of studies published in MEDLINE between 1999 and 2005 reporting the cost and/or cost-effectiveness of endovascular and/or open surgical repair of nonruptured AAAs was conducted. Case series studies with less than fifty patients per treatment were excluded.

Results: Of twenty eligible articles, three were randomized controlled trials, twelve case series, four Markov models, and one systematic review. Regardless of time frame, all studies found that endovascular repair costs more than open surgery. Although the high cost of the endovascular prosthesis was partially offset by reduced intensive care, hospital length of stay, operating time, blood transfusions, and perioperative complications, hospital costs were still greater for endovascular than open surgical repair. For patients medically fit for open surgery, mid-term costs were greater for endovascular repair with no difference in overall survival or quality of life. For patients medically unfit for open surgery, endovascular repair costs more than no intervention with no difference in survival.

Conclusions: Although conclusions regarding the cost-effectiveness of AAA treatment options are time dependent and vary by institutional perspective, from a societal perspective, endovascular repair is not currently cost-effective for patients with large AAA regardless of medical fitness.

Dilation of the ascending aorta is rare in childhood. When seen, it is usually associated with some form of connective tissue disease or predisposing cardiac malformations, especially an aortic valve with two leaflets. We describe four children in whom significant dilation of the ascending aorta was encountered as an incidental finding. No patient had any sign of an associated connective tissue disease, nor did we detect any predisposing cardiac anomalies. One patient had undergone surgical ligation of the arterial duct in infancy, whilst another had undergone repair of aortic coarctation, also in infancy. A third child has had repair of an atrioventricular septal defect with exclusively atrial shunting, whereas the fourth patient had a structurally normal heart. The aortic valve had three leaflets, and was functionally normal in all. The dilation of the ascending aorta was progressive in all patients, and finally surgical treatment was recommended, relying on the guidelines established for the management of patients affected with the Marfan syndrome.

Objective: To heighten the awareness of pediatricians and pediatric cardiologists to aortic dissection, a potentially dangerous medical condition. Methods: We reviewed the charts of 13 patients, seen in four medical centers, who suffered acute or chronic aortic dissection over the period 1970 through 2000 whilst under the age of 25 years. Results: There were seven male and six female patients, with the mean age at diagnosis being 12.1 years, with a range from one day to 25 years. Congenital cardiac defects were present in five patients, and Marfan syndrome in four. In three of the patients with congenital cardiac defects, aortic dissection developed as a complication of medical procedures. In three patients, dissection followed blunt trauma to the chest. We could not identify any risk factors in one patient. The presenting symptoms included chest pain in four patients, abdominal pain and signs of ischemic bowel in two, non-palpable femoral pulses in one, and obstruction of the superior caval vein in one. Angiography and magnetic resonance imaging were the main diagnostic tools. Overall mortality was 38%. Only six patients had successful surgical outcomes. Conclusion: Due to the rarity of aortic dissection a high index of suspicion is required to reach the diagnosis in a timely manner. It should be considered in young patients complaining of chest pain in association with Marfan syndrome, anomalies of the aortic valve and arch, and chest trauma.

We present the anaesthetic management of a patient for stenting of a thoracic aortic aneurysm at the site of an aortic coarctation. The specific challenges to the anaesthetist for this case are outlined. These include the specific problems of placing the graft, the obvious risk of aortic rupture and the unfamiliar environment of the separate radiological theatre. The advantages and disadvantages to the anaesthetist of the treatment of thoracic aortic aneurysms with stents are briefly discussed.

Background and objective Ischaemic colitis can be a serious complication after aortic surgery. The paucity of clinical symptoms makes its diagnosis particularly difficult and often delayed. Automated on-line tonometry is now proposed to monitor intestinal perfusion. This study was designed to assess the use of semi-continuous sigmoid-to-arterial [P(r-a)CO2] PCO2 gap monitoring in aortic surgery to detect colonic ischaemia.

Methods This prospective clinical study was realized at the University Hospital of Lille, France, including eight males scheduled for abdominal aortic aneurysm surgery. Intraoperative and postoperative P(r-a)CO2 values were compared with conventional monitoring and colonic mucosa aspect performed by sigmoidoscopy 48 h after surgery. Haemodynamic variables, O2 delivery (DO2), O2 consumption (VO2), O2 extraction (ERO2), lactate, P(v-a)CO2, P(r-a)CO2 were measured peroperatively and every 4 h during a 48-h postoperative period.

Results Intraoperative P(r-a)CO2 values increased significantly with the highest value (4.36 ± 3.42 kPa) observed during aortic clamping when DO2 was the most altered. P(r-a)CO2 continued to deteriorate after surgery with the maximal values between 8 (4.79 ± 3.85 kPa) and 12 (4.68 ± 3.26 kPa) h after surgery. This peak was associated with a significant ERO2 increase counterbalancing an increase of VO2 whereas DO2 tended to decrease. P(r-a)CO2 values began to decrease only at the end of the study. The highest values of P(r-a)CO2 were registered in patients with the most altered haemodynamic variables, severe ischaemic colitis along with higher hospital lengths of stay.

Conclusion Taken together, these data suggest that regional and automated capnometry may be easily used non-invasively to detect peroperative intestinal ischaemia in aortic surgery.

Serious neurological complications of abdominal aortic vascular surgery are rare but devastating for all involved. When epidural blockade is part of the anaesthetic technique such compliscations may be attributed to needles, catheters or drugs. We present a patient who developed paraplegia following an elective abdominal aortic aneurysm repair. Continuous epidural blockade was part of the anaesthetic technique and postoperative analgesia. In this case the spinal cord damage was explained by ischaemia caused by the aortic surgery. This event has made us aware of a rare complication associated with abdominal aortic surgery and highlighted safety aspects of epidural anaesthesia in such patients.