Takayasu's arteritis: What should the dentist know?

Takayasu's arteritis is a chronic inflammatory disease that affects large blood vessels, especially the aorta and/or its major branches. The condition presents with segmental lesions adjacent to normal, apparently unaffected, areas. The lesions include stenosis, occlusion, dilatations or aneurysm formations along the path of the affected artery. Because of the severity of the disease and the possibility of cardiovascular complications, patients with Takayasu's arteritis require medical treatment based on immunosuppressive and antihypertensive drugs, as well as regular follow up and surgical intervention in many instances. The aim of this paper was to describe the characteristics of Takayasu's arteritis, to report dental treatment carried out on an affected patient, and to discuss the main implications and care required during routine treatment for children in the dental office. © 2005 BSPD and IAPD.

Ascending thoracic aorta dimension and outcomes in acute type B dissection (from the International Registry of Acute Aortic Dissection [IRAD])

It is not well known if the size of the ascending thoracic aorta at presentation predicts features of presentation, management, and outcomes in patients with acute type B aortic dissection. The International Registry of Acute Aortic Dissection (IRAD) database was queried for all patients with acute type B dissection who had documentation of ascending thoracic aortic size at time of presentation. Patients were categorized according to ascending thoracic aortic diameters ≤4.0, 4.1 to 4.5, and ≥4.6 cm. Four hundred eighteen patients met inclusion criteria; 291 patients (69.6%) were men with a mean age of 63.2 ± 13.5 years. Ascending thoracic aortic diameter ≤4.0 cm was noted in 250 patients (59.8%), 4.1 to 4.5 cm in 105 patients (25.1%), and ≥4.6 cm in 63 patients (15.1%). Patients with an ascending thoracic aortic diameter ≥4.6 cm were more likely to be men (p = 0.01) and have Marfan syndrome (p <0.001) and known bicuspid aortic valve disease (p = 0.003). In patients with an ascending thoracic aorta ≥4.1 cm, there was an increased incidence of surgical intervention (p = 0.013). In those with an ascending thoracic aorta ≥4.6 cm, the root, ascending aorta, arch, and aortic valve were more often involved in surgical repair. Patients with an ascending thoracic aorta ≤4.0 were more likely to have endovascular therapy than those with larger ascending thoracic aortas (p = 0.009). There was no difference in overall mortality or cause of death. In conclusion, ascending thoracic aortic enlargement in patients with acute type B aortic dissection is common. Although its presence does not appear to predict an increased risk of mortality, it is associated with more frequent open surgical intervention that often involves replacement of the proximal aorta. Those with smaller proximal aortas are more likely to receive endovascular therapy.

Role of brain nitric oxide in the cardiovascular control of bullfrogs

The goal of the present study was to determine if nitric oxide (NO) acting on the brain of bullfrog (Lithobates catesbeianus) is involved in arterial pressure and heart rate (HR) control by influencing sympathetic activity. We investigated the effect of intracerebroventricular injections of l-NMMA (a nonselective NO synthase inhibitor) on mean arterial blood pressure (MAP), HR and cutaneous vascular conductance (CVC) of pelvic skin after intravenous injection of α or β adrenergic blockers, prazosin or sotalol, respectively. Arterial pressure was directly measured by a telemetry sensor inserted in the aortic arch of animals. l-NMMA increased MAP, but did not change HR. This hypertensive response was inhibited by the pre-treatment with prazosin, but accentuated by sotalol. The effect of l-NMMA on MAP was also inhibited by i.v. injections of the ganglionic blocker, hexamethonium. Thus, NO acting on the brain of bullfrog seems to present a hypotensive effect influencing the sympathetic activity dependent on α and β adrenergic receptors in the periphery. © 2013 Elsevier Inc.

Should aortic arch replacement be performed during initial surgery for aortic root aneurysm in patients with Marfan syndrome?

OBJECTIVES: The aim of this study was to investigate whether total arch replacement (TAR) during initial surgery for root aneurysm should be routinely performed in patients with Marfan syndrome (MFS). METHODS: Retrospective analysis of 94 consecutive MFS patients fulfilling Ghent criteria who underwent 148 aortic surgeries and were followed at this institution during the past 16 years. RESULTS: The mean follow-up interval was 8.8 ± 7 years. Initial presentation was acute aortic dissection (AAD) in 35% of patients (76% Type A and 24% Type B) and aneurismal disease in 65%. TAR was performed in 8% of patients during initial surgery for AAD (otherwise a hemi-arch replacement was performed) and 1.6% in elective root repair. Secondary TAR had to be performed in only 3% of patients without, but in 33% following AAD (33% Type A and 33% Type B; P = 0.0001). Thirty-day, 6-month, 1-year and overall mortalities were 3.2, 5.3, 6.4 and 11.7%, respectively. Operative and 30-day mortalities in secondary aortic arch replacement were zero. Secondary TAR after AAD did not increase the need for the replacement of the entire thoracoabdominal aorta during follow-up compared with patients without secondary TAR (37 vs 40%, P = 1.0). CONCLUSIONS: MFS patients undergoing elective root repair have small risk of reinterventions on the aortic arch, and primary prophylactic replacement does not seem to be justified. In patients with AAD, the need for reinterventions is precipitated by the dissection itself and not by limiting the procedure to the hemi-arch replacement in the emergency setting. Limiting surgery to the aortic root, ascending aorta and proximal aortic arch is associated with low mortality in MFS patients presenting with AAD.

Transposition of the supra-aortic vessels before stent grafting the aortic arch and descending aorta

Thoracic endovascular aortic repair has broadened the spectrum of treatment options for various acute and chronic thoracic aortic diseases. In clinical practice, aneurysms of the descending aorta are rarely limited to 1 segment. Thus, various surgical and endovascular options have been developed to offer treatment to those patients with more extended descending thoracic aortic disease. We have summarized the most common methods of arch rerouting, depending on the aortic involvement, emphasizing that these techniques should be used very selectively by experienced cardiovascular surgery teams.

Huge ruptured and infected pseudoaneurysm of the ascending aorta and aortic arch with erosion of sternum after previous cardiac surgery

A 77-year-old woman underwent aortic valve replacement and coronary bypass grafting in 2007 in the Emirates. Evolution was uneventful until December 2011. After repeated episodes of unspecific infections, a computed tomographic scan showed a large pseudoaneurysm of the distal ascending aorta. The site of aortic rupture was closed with a Gore-Tex patch and a Staphylococcus aureus infection treated appropriately. Two months later, a small cutaneous lesion on the cranial part of the sternotomy started bleeding. Computed tomographic scan demonstrated recurrence of a false aneurysm with erosion of the sternum and a large subcutaneous hematoma caused by the fistula. The patient was transferred to our institution. The challenges of this case included safe surgical approach (sternotomy, cannulation, perfusion, cerebral protection) as well as complete removal and extensive debridement of the infected material and reconstruction of the aortic arch. Using fully biological material, reconstruction of the ascending aorta and proximal arch was successfully performed.

Technical advances improved outcome in patients undergoing surgery of the ascending aorta and/or aortic arch: ten years experience

BACKGROUND: Several technical advances in thoracic aortic surgery, such as the use of antegrade cerebral perfusion, avoidance of cross-clamping and the application of glue, have beneficially influenced postoperative outcome. The aim of the present study was to analyse the impact of these developments on outcome of patients undergoing surgery of the thoracic aorta. METHODS AND RESULTS: Between January 1996 and December 2005, 835 patients (37.6%) out of 2215 aortic patients underwent surgery on the thoracic ascending aorta or the aortic arch at our institution. All in-hospital data were assessed. Two hundred and forty-one patients (28.8%) suffered from acute type A dissection (AADA). Overall aortic caseload increased from 41 patients in 1996 to 141 in 2005 (+339%). The increase was more pronounced for thoracic aortic aneurysms (TAA) (+367.9%), than for acute type A aortic dissections (+276.9%). Especially in TAA, combined procedures increased and the amount of patients with impaired left ventricular function (EF <50%) raised up from 14% in 1996 to 24% in 2005. Average age remained stable. Logistic regression curve revealed a significant decrease in mortality (AADA) and in the overall incidence of neurological deficits. CONCLUSIONS: Technical advances in the field of thoracic aortic surgery lead to a decrease of mortality and morbidity, especially in the incidence of adverse neurological events, in a large collective of patients. Long-term outcome and quality of life are better, since antegrade cerebral perfusion has been introduced.

Massive intraoperative thrombus of the aortic arch and proximal descending aorta

A 49-year-old male patient presented with a paravalvular leakage 18 years after aortic valve replacement. The patient received a mechanical valve and was weaned uneventfully from cardiopulmonary bypass. After a drop in the pressure curve of the left radial artery an additional line was placed in the proximal ascending aorta showing normal systemic pressure. Transesophageal echocardiography showed a massive circular thrombosis of the distal aortic arch. The patient received a covered stent to expand the residual aortic lumen and left the hospital in good health.

The frozen elephant trunk technique for the treatment of complex aortic arch and descending thoracic aorta pathologies: long term outcomes and distal reintervention

Background: The frozen elephant trunk(FET) technique is one of the last evolution in the treatment of complex pathologies of the aortic arch and the descending thoracic aorta.Materials and methods: Between January 2007 and March 2021, a total of 396 patients underwent total aortic arch replacements with the FET technique in our centre.The main indications were thoracic aortic aneurysm(n=104,28.2%), chronic aortic dissection(n=224,53.4%) and acute aortic dissection(n=68, 18.4%). We divided the population in two groups according the position of the distal anastomosis (zone 2 vs zone 3) and the length of the stent graft (< 150 mm vs > 150 mm): conservative group (Zone 2 anastomosis + stent length < 150mm, n. 140 pts) and aggressive group (zone 3 anastomosis + stent length > 150mm, n. 141). Results: The overall 30-day mortality rate was 13%(48/369); the risk factor analysis showed that an aggressive approach was neither a risk factor for major complication (permanent dialysis, tracheostomy, bowel malperfusion and permanent paraplegia) neither for 30-day mortality. The survival rate at 1, 5,10 and 15 years was 87.7%,75%,61.3% and 58.4% respectively. During the follow up, an aortic reintervention was performed in 122 patients (38%), 5 patients received a non-aortic cardiac surgery. Freedom from aortic reintervention at 1-,5- and 10-year was 77%,54% and 44% respectively. The freedom from aortic reintervention was higher in the ‘aggressive’ group (62.5%vs40.0% at 5 years, log-rank=0.056). An aggressive approach was not protective for aortic reintervention at follow up and for death at follow up. Conclusions: The FET technique represents a feasible and efficient option in the treatment of complex thoracic aortic pathologies. An aortic reintervention after FET is very common and the decision-making approach should consider and balance the higher risk of an aggressive approach in terms of post-operative complication versus the higher risk of a second aortic reintervention at follow-up.

The Influence Of Body Weight On Sexual Function And Quality Of Life In Women With Polycystic Ovary Syndrome.

To assess sexual function (SF) and quality of life (QOL) in women with polycystic ovary syndrome (PCOS). A cross-sectional study was conducted to assess 56 women with PCOS and 102 control women with regular menstrual cycles. To assess SF and QOL in Brazilian women with PCOS with Female Sexual Function Index (FSFI) and the WHOQOL-bref questionnaires. Women with PCOS had a worse evaluation to arousal, lubrication, satisfaction, pain and total FSFI, and there was no difference in sexual desire and orgasm. Besides, they had a worse evaluation concerning health status than controls. The body mass index was inversely correlated to the QOL, especially to the physical, psychological, environment aspects and self-assessment of QOL, but it did not show correlation to the SF. Women with PCOS had a worse sexual function and self-assessment of health condition in comparison to controls. The body weight as isolated symptom was correlated to the worsening in quality of life, but not with the worsening of sexual function.

Psychogenetics of Turner syndrome: an investigation of 28 subjects and respective controls using the Bender test and Piagetian scales

Piagetian scales and the Bender visual motor gestalt test (BT) were applied to 28 subjects with universal 45, X Turner syndrome (TS), and their respective controls, in order to investigate their cognitive performance. Dermatoglyphics were also analyzed to obtain clues concerning embryological changes that may have appeared during development of the nervous system and could be associated with cognitive performance of TS patients. Dermatoglyphic pattern distribution was similar to that reported in previous studies of TS individuals: ulnar loops in the digital patterns and finger ridge, a-b, and A'-d counts were more frequent, while arch and whorl patterns were less frequent compared to controls. However, we did not find higher frequencies of hypothenar pattern, maximum atd angle, and ulnarity index in our TS subjects, unlike other investigations. Furthermore, we found significant differences between TS and control T line index values. The BT scores were also lower in probands, as has been previously reported, revealing a neurocognitive deficit of visual motor perception in TS individuals, which could be due to an absence of, or deficiency in, cerebral hemispheric lateralization. However, TS subjects seemed to improve their performance on BT with age. Cognitive performance of the TS subjects was not significantly different from that of controls, confirming a previous study in which TS performance was found to be similar to that of the normal Brazilian population. There were significant correlations between BT scores and Piagetian scale levels with dermatoglyphic parameters. This association could be explained by changes in the common ectodermal origin of the epidermis and the central nervous system. TS subjects seem to succeed in compensating their spatial impairments in adapting their cognitive and social contacts. We concluded that genetic counseling should consider cognitive and psychosocial difficulties presented by TS subjects, providing appropriate treatment and orientation for them and their families.

Syndromic and non-syndromic aneurysms of the human ascending aorta share activation of the Smad2 pathway

Common features such as elastic fibre destruction, mucoid accumulation, and smooth muscle cell apoptosis are co-localized in aneurysms of the ascending aorta of various aetiologies. Recent experimental studies reported an activation of TGF-beta in aneurysms related to Marfan (and Loeys-Dietz) syndrome. Here we investigate TGF-beta signalling in normal and pathological human ascending aortic wall in syndromic and non-syndromic aneurysmal disease. Aneurysmal ascending aortic specimens, classified according to aetiology: syndromic MFS (n = 15, including two mutations in TGFBR2), associated with BAV (n = 15) or degenerative forms (n = 19), were examined. We show that the amounts of TGF-beta 1 protein retained within and released by aneurysmal tissue were greater than for control aortic tissue, whatever the aetiology, contrasting with an unchanged TGF-beta 1 mRNA level. The increase in stored TGF-beta 1 was associated with enhanced LTBP-I protein and mRNA levels. These dysiregulations of the extracellular ligand are associated with higher phosphorylated Smad2 and Smad2 mRNA levels in the ascending aortic wall from all types of aneurysm. This activation correlated with the degree of elastic fibre fragmentation. Surprisingly, there was no consistent association between the nuclear location of pSmad2 and extracellular TGF-beta 1 and LTBP-I staining and between their respective mRNA expressions. In parallel, decorin. was focally increased in aneurysmal media, whereas biglycan was globally decreased in aneurysmal aortas. In conclusion, this study highlights independent dysregulations of TGF-beta retention and Smad2 signalling in syndromic and non-syndromic aneurysms of the ascending aorta. Copyright (C) 2009 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Holt-Oram syndrome: novel TBX5 mutation and associated anomalous right coronary artery

The Holt-Oram syndrome was confirmed in an asymptomatic 36-year-old man by a novel TBX5-gene mutation (exon 8 acceptor splicing site, c.663-1G greater than A). Computed tomography showed an atrial septal defect and an anomalous right coronary artery crossing between the aorta and pulmonary arteries. Surgery corrected the septal defect and the initial segment of the anomalous vessel was unroofed and enlarged. Anomalous coronary arteries were not previously described in the Holt-Oram syndrome patients and should be added to the list of possible associated cardiac defects.