Case report: airway and concurrent hemodynamic management in a neonate with oculo-auriculo-vertebral (Goldenhar) syndrome, severe cervical scoliosis, interrupted aortic arch, multiple ventricular septal defects, and an unstable cervical spine.

We report the challenging case of a 1-week-old, term, 2.4 kg neonate with Goldenhar syndrome (including microcephaly, left microtia, left facial palsy, dextro-scoliosis of the cervical spine, and cervico-thoracic levoscoliosis), multiple ventricular septal defects, a type B interrupted aortic arch, a large patent ductus arteriosis, and radiographic and clinical signs concerning for an unstable cervical spine. Our anesthesia team was consulted for perioperative management of this patient during her surgical repair. This case report describes the use of the Air-Q size 1 laryngeal airway (LA) to assist fiberoptic intubation in an ASA 4 neonate with cardiac disease, an anticipated difficult airway with the addition of an unstable cervical spine, as well as the anesthetic techniques used to maintain hemodynamic stability while the airway was secured.

[CT and MR imaging follow-up of medically treated acute aortic syndrome]. / Comment je surveille un syndrome aortique aigu traité médicalement au scanner ou en IRM?

The follow-up of medically treated acute aortic syndromes relies on CT and MR imaging. Comparison with prior examinations is essential. For aortic dissections, progressive enlargement of the false lumen, visceral hypoperfusion, and extension should be excluded. Mural hematomas and ulcers also undergo close follow-up to detect progression and recanalization. It is important to be familiar with the risk factors of disease progression for medically treated acute aortic syndromes and their management. It is also important to be familiar with the imaging features of disease progression. Acute aortic syndromes managed medically should undergo routine follow-up with CT or MR because these lesions may evolve silently over time and present with complications.

Aortic Arch Calcification as a Predictor of Repeated Arteriovenous Fistula Failure within 1-Year in Hemodialysis Patients.

OBJECTIVES: The aim of the study was to identify the factors associated with repeated arteriovenous fistula (AVF) failure within 1-year, especially the impact of aortic arch calcification (AAC) on patency of AVF. MATERIALS AND METHODS: We retrospectively assessed chest radiography in hemodialysis patients who had undergone initial AVF. The extent of AAC was categorized into four grades (0-3). The association between AAC grade, other clinical variables, and repeated failure of AVF was then analyzed by binary logistic regression analysis. RESULTS: This study included 284 patients (158 males, mean age 61.7 ± 13.1 years). Patients with higher AAC grade were older, had more frequently diabetes mellitus and cardiovascular disease, had lower diastolic blood pressure, and had higher corrected calcium and lower intact parathyroid hormone levels. In multivariate analysis, the presence of higher AAC grade (odds ratio (95% confidence interval): 2.98 (1.43-6.23); p = 0.004), lower mean corrected calcium (p = 0.017), and mean serum albumin level (p = 0.008) were associated with repeated failure of AVF. CONCLUSIONS: The presence of higher AAC grade, lower mean corrected calcium and mean serum albumin level were independently associated with repeated AVF failure within 1 year in hemodialysis patients.

Paraparesis after Primary Percutaneous Coronary Intervention for ST-segment Elevation Myocardial Infarction: Combined Uncommon Complications of Acute Aortic Syndrome in a Patient.

Acute aortic syndrome complicated by both ST-segment elevation myocardial infarction (STEMI) and spinal ischemia is exceedingly rare. We herein report the case of a 66-year-old man who presented with paraparesis after primary percutaneous coronary intervention for STEMI. He was found to have an intramural hematoma of the ascending aorta and a severe dissection in the descending aorta, which led to both STEMI and paraparesis.

Takayasu arteritis. A study of 32 North American patients.

Thirty-two patients (26 female, 6 male) with angiographically diagnosed Takayasu arteritis were seen at the Mayo Clinic between 1971 and 1982. Racial composition of this group was 23 North American Caucasians, 4 Mexicans, 3 Orientals, 1 Native American, and 1 patient of Middle Eastern origin. Incidence of the disease in Olmsted County, Minnesota, was 2.6/million/year. Diagnosis was often delayed for long periods of time, with a median delay of 18 months. Patients had both non-vascular symptoms (arthralgias in 56%, fever in 44%, weight loss in 38%) and symptoms of vascular stenosis such as arm claudication (47%) and hypertension due to renal artery stenosis (41%). All patients had either multiple vascular bruits (94%) or absent pulses (50%). Laboratory findings included anemia (44%) and elevations of erythrocyte sedimentation rate (78%). Almost all patients had multiple sites of arterial involvement documented by angiogram with various combinations of stenosis, luminal irregularity and aneurysm formation. Response to corticosteroid treatment was usually very good, with dramatic improvement in non-vascular symptoms and return of pulses in 8 of the 16 patients with absent pulses prior to treatment. Five-year survival rate from time of diagnosis was 94%. Twelve patients underwent surgical procedures involving the carotid arteries (5 cases), subclavian artery (4 cases) and renal arteries (3 cases). Three aneurysms were resected, one had aortic valve replacement for severe aortic regurgitation, and two patients underwent transluminal angioplasty. Pathologic changes were restricted to the media and adventitial layers of the vessel wall and were indistinguishable from those of giant-cell or temporal arteritis. Takayasu arteritis is more common than previously suspected in North America, is not restricted to any one racial group, and is readily treatable with corticosteroids and surgical vascular reconstruction.

Takayasu's arteritis of the aortic arch: endovascular treatment and correlation with positron emission tomography.

A case is presented in which three arterial stenoses and one arterial occlusion due to Takayasu's disease were treated by an endovascular approach. The endovascular technique is discussed, and the clinical and angiographic findings after treatment are correlated with cerebral hemodynamic and metabolic parameters as measured by positron emission tomography.

Radiology of aortoarteritis.

Based mainly on the practice and experience in China, the radiology of aortoarteritis, including plain film and angiographic manifestations and its correlation to clinical syndromes, is reviewed in this article. The preliminary results of percutaneous transluminal angioplasty in the management of renovascular hypertension due to aortoarteritis are described briefly and discussed.

Aortoarteritis: a report of 480 cases.

We report 480 patients with aorto-arteritis studied in our group. Among 320 patients, who were diagnosed by aortography the female to male ratio was 2.8:1, and the age of onset ranged from 5-45 years. The patients were followed for 1-27 years (average 6.8 years). Two hundred and eighty-six (59.6%) patients with renovascular hypertension were found in the group. All patients were treated with medical or surgical procedures. One hundred and thirty patients with renovascular hypertension were operated upon by revascularisation or uninephrectomy and 11 patients with renal artery stenosis were subjected to percutaneous transluminal renal angioplasty. There were 55 deaths (11.5%) in the group, cerebral haemorrhage being the major cause of death. Five year and ten year survival rates were 93.1% and 91.1% respectively.

The management of stenotic and obstructive lesions of the aortic arch branches.

The stenotic internal carotid can be managed in a variety of ways and number of tests can be utilized for assessing the collateral blood flow. Except in unusual situations, carotid thromboendarterectomy with or without a patch graft is generally employed. Although some surgeons use no protective shunt at all, or only upon specific indications, intraluminal shunting is utilized extensively. Our preference is to employ the customary Javid shunt technic except in unusual circumstances that suggest that added safety may be assured by shortening to a matter of seconds the period of interruption of carotid flow. In such cases, we believe the temporary axillary-internal carotid intraluminal shunt is of considerable value. Although mediastinal and thoracic procedures and bypass grafts delivering blood from the ascending aorta are not needed nearly as often as they were formerly, they are essential in certain cases. They yield excellent results and carry small risk. Carotid-subclavian grafts have proved quite valuable in restoring pulsatile flow to the subclavian and carotid systems. Our preference, however, because of technical simplicity, is the carotid-axillary bypass procedure. Subclavian-subclavian and axillary-axillary grafts have been employed successfully. When a carotid-axillary bypass is feasible, we would choose this method instead and reserve the others for unusual anatomic-pathologic situations.

Initial experience with percutaneous transluminal angioplasty in the management of Takayasu's arteritis.

We have performed percutaneous transluminal angioplasty (PTA) for 15 arterial stenoses in 11 patients with Takayasu's arteritis. The lesions included tight, proximally located renal artery stenosis (12 stenoses; nine patients), localized abdominal aortic stenosis (two patients) and occluded left common iliac artery (one patient). Clinically successful dilatation was achieved in seven patients (10 stenoses) with renal artery stenosis, in both the patients with abdominal aortic stenosis and in the only patient with an occluded left common iliac artery. No complications related to the procedure were encountered. The follow-up period (n = 7) ranged between 1 and 16 months, mean follow-up period after renal angioplasty was 5 months and after abdominal aortic angioplasty was 12.5 months. Initial success has been maintained in both the patients with abdominal aortic stenoses and in four out of five patients with renal artery stenosis. Percutaneous transluminal angioplasty offers an attractive alternative for the management of stenosing lesions in Takayasu's arteritis with good short term results.

Successful outcome of pregnancy in a subfertile patient with severe aortoarteritis (Takayasu's disease).

This report describes the successful outcome of pregnancy in a 27-yr-old subfertile Chinese nullipara with severe Takayasu's disease. Pregnancy was achieved following ovulation induction with clomiphene. Blood pressure was controlled with propranalol and fetal growth was monitored by serial ultrasound fetal anthropometry. Pregnancy was terminated at 38 weeks of pregnancy by elective caesarean section. A live female infant weighing 2420 g was delivered.

[Transluminal dilatation of innominate stenosis in aortic arch syndrome]. / Transluminale Dilatation einer Anonymastenose bei Aortenbogensyndrom.

Successful percutaneous transluminal dilatation of an anonyma stenosis is reported. The patient was a 40 year-old male with the aortic arch syndrome. Five years before an occlusion of the central carotid artery, as well as anonyma and left subclavian stenoses were treated by means of vascular grafts. On recurrence of the neurological symptoms, reocclusion of the right graft to the anonyma artery and subtotal stenosis of the left carotid bifurcation was noted. The anonyma stenosis was dilated by means of PTD. Haemodynamic success was demonstrated by Doppler sonography.

[Mid-aortic syndrome in 18 year-old woman with arterial hypertension and bilateral renal artery stenosis]. / Zespól aorty brzusznej u 18-letniej chorej z nadcisnieniem tetniczym i obustronnym zwezeniem tetnic nerkowych.

Mid-aortic syndrome (MAS) is characterised by narrowing of the abdominal aorta, often with involvement of the renal and splanchnic arterial branches. Although uncommon, MAS is an important cause of renovascular hypertension in children and adolescents and should be considered in the differential diagnosis of hypertension . Hypertension is typically severe and often difficult to manage. The management of MAS should always be individualised and may include percutaneous transluminal renal angioplasty, stent implantation or surgical revascularisation. We present a 18 year-old woman with hypertension and MAS coexisting with bilateral renal artery stenosis who underwent left renal artery angioplasty and than was followed-up for one year.

Embryology and imaging review of aortic arch anomalies.

Congenital malformations of the thoracic aorta can be discovered on chest radiographs when associated with symptoms or found incidentally. We review the imaging anatomy and associations of many of the aortic arch malformations that can be encountered in adults and highlight key points with regard to their treatment and prognoses. An understanding of the normal and abnormal embryologic development of the aortic arch, with knowledge of their imaging features, may be important for improving diagnostic accuracy and patient care.