Autologous Stem Cell Transplantation in Relapsing Polychondritis
Richard K. Burt, Alberto M. Marmont in Stem Cell Therapy for Autoimmune Disease, 2019
Although relapsing polychondritis is a separate clinical entity, up to 30% of patients also suffer from another inflammatory disease (Table 1). The most common associated disease is vasculitis, which can affect vessels of all sizes. Five to 14% of all patients with relapsing polychondritis have biopsy-confirmed leukocytoclastic vasculitis, ranging from isolated cutaneous leukocytoclastic vasculitis to aortitis. Although vasculitis most commonly leads to dilatation of the aortic ring and ascending aorta, aneurysm of the descending thoracic or abdominal aorta can also occur. Aortic rupture has also been reported. Dermatological and renal manifestations, neuropathies, audiovestibular abnormalities, and episcleritis are most likely due to microscopic angiitis. Vasculitis seems to worsen the prognosis of relapsing polychondritis.17 Relapsing polychondritis is infrequently accompanied by defined vasculitides like Wegener’s granulomatosis and polyarteritis nodosa.18 The co-occurrence of relapsing polychondritis and Behcet’s disease has also been reported; this constellation was named “MAGIC syndrome” (mouth and genital ulcers with inflamed cartilage syndrome).19 Roughly 25% of patients with relapsing polychondritis have associated autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, mixed connective tissue disease, Sjogren’s syndrome, and hypothyroidism.
Injury to the Thoracic Great Vessels
Stephen M. Cohn, Matthew O. Dolich, Kenji Inaba in Acute Care Surgery and Trauma, 2016
Cardarelli et al. looked at the University of Maryland’s 30 years of experience with traumatic aortic rupture [23]. There were 219 patients with a diagnosis of traumatic aortic rupture between 1971 and 2001. Patients were divided according to surgical technique. There were 82 patients in the clamp-and-sew technique group (Group A), 64 patients in the passive shunt group (Group B), and 73 patients in the heparin-less partial cardiopulmonary bypass (Group C). Mortality was 18 patients for Group A (21.9%), 23 patients for Group B (35.9%), and 13 patients for Group C (17.8%) (p = 0.03). Paraplegia occurred in 15 of the 64 survivors in Group A (23.4%), 7 of the 41 survivors in Group B (17%), and 0 of the 60 survivors in Group C (p = 0.0005). Aortic occlusion without lower body perfusion for longer than 30 min (p = 0.004) and surgical technique without lower body bypass support (p = 0.0005) were associated with paraplegia. They concluded that the use of heparin-less distal cardiopulmonary bypass in the authors’ hands is safe and is associated with a reduced incidence of paraplegia.
Predicting the Biomechanics of the Aorta Using Ultrasound
Ayman El-Baz, Jasjit S. Suri in Cardiovascular Imaging and Image Analysis, 2018
Thoracic aortic disease continues to be associated with a significant burden of morbidity and mortality in the general population. Disease of the thoracic aorta is due to aneurysm and/or dissection. An aneurysm is by definition an aortic diameter twice the normal size. This can lead to frank rupture or dissection then rupture. A dissection is a tearing of the inner lumen of the aorta such that the layers of the media separate and blood flows into a false lumen as well as the true lumen. An aortic rupture and an ascending aortic dissection (Type A) are considered surgical emergencies. The mortality is high and generally over 50% are dead without surgical treatment within two weeks. Despite improvement in diagnostics and advanced surgical techniques, mortality rates following surgery for acute aortic syndromes such as a rupture or type A aortic dissection continue to be associated with an overall mortality of 20–25% and significant morbidity such as stroke [1–5]. This high mortality following acute life-saving surgery is contrasted by the much lower risk of mortality (1.5–2.5%) when the ascending aortic aneurysm is repaired electively [3, 4, 6, 7]. This comparison illustrates the critical importance of early detection of individuals at risk for acute aortic syndromes such as dissection and rupture. Currently, most aortic aneurysms are detected incidentally when undergoing imaging for an unrelated issue, as aortic disease is generally asymptomatic until a first presentation of catastrophic dissection or even sudden death [9].
Diabetes mellitus is not associated with worse vascular outcome following percutaneous transfemoral transcatheter aortic valve implantation
Published in Acta Cardiologica, 2019
Fabien Lareyre, Claude Mialhe, François Bourlon, Yacoub Habib, Carine Dommerc, Juliette Raffort
The 30-day post-operative outcomes following TAVI were compared between diabetics and non-diabetics (Table 3). The post-operative mortality was similar among the groups (2.6% vs. 1.9%, p = .6916). The mean number of days in hospital post-procedure was significantly higher in diabetic patients (10.3+/– 0.5 days vs. 9.5+/– 0.2, p = .0507). Major vascular outcomes included aortic rupture and access site or access related injury leading to death, life-threatening or major bleeding. The aortic rupture was observed in one patient who was not diabetic. All the major access site related vascular injuries were observed in 6 (1.9%) non-diabetic patients. Among them, 5 patients had haematoma in the inguinal region associated with major bleeding requiring the transfusion of at least 2 packed red blood cells. One patient had a retroperitoneal haematoma leading to major bleeding which required transfusion associated with a surgical re-intervention to treat it.
Conventional aortic root vs valve-sparing root replacement surgery in aortic dilatation syndromes: a comparison of mortality and postoperative complications
Published in Expert Review of Cardiovascular Therapy, 2023
Hashrul N Rashid, Omar Chehab, Harriet Hurrell, Vitaliy Androshchuk, Agata Sularz, Tiffany Patterson, Gianluca Lucchese, Simon Redwood
Aortic dilatation is associated with an increased risk of aortic rupture and dissection. Progressive enlargement of the aortic root may cause secondary aortic regurgitation (AR) resulting from inadequate leaflet coaptation of the aortic valve. Valve-sparing root replacement (VSRR) surgery has become an alternative surgical option to treat significant aortic dilatation. Contrary to conventional aortic root replacement, VSRR preserves the native aortic leaflet (with or without aortic cusp repair), potentially avoiding the inherent risk of prosthetic aortic valve, such as infection or anticoagulation therapy-related complications, due to mechanical aortic valve replacement (m-AVR). Since the introduction of VSRR surgery over thirty years ago, there has been further improvement and growth of evidence in both conventional aortic root replacement and VSRR.
Aortic valve-related aortopathy: assessing optimal timing of surgical intervention
Published in Expert Review of Cardiovascular Therapy, 2019
Johannes Petersen, Tatiana Sequeira-Gross, Shiho Naito, Hermann Reichenspurner, Evaldas Girdauskas
Bicuspid aortic valve (BAV) disease is the most common congenital valve disease (prevalence of 1 to 2%) described in multiple autopsy studies [11,12] as well as in neonatal echocardiography studies [13] and is associated with an aortic aneurysm in 40 to 60%, which occurs independently of age and body size [14]. This so-called ‘BAV-aortopathy’ is accompanied with a low, but significantly higher than in the general population, risk of aortic rupture and dissection [15]. However, a meta-analysis was able to show that the risk of aortic dissection after aortic valve replacement (AVR) is primarily associated with the type of BAV disease and not necessarily with the aortic diameter [16]. Due to the marked heterogeneity of BAV disease [17,18], different phenotypes have been described in order to better distinguish between the predominantly genetic versus hemodynamic origin of BAV-aortopathy [19,20]. Outlined in Figure 1 are different presentations (1a-c) of a sinus of valsalva aneurysm which is most often of genetic origin (S. Sakakibara, S. Konno Congenital aneurysm of the sinus of Valsalva. Anatomy and classification; Troupis; Sinus valsalva aneurysm on cardiac CT angiography: Assessment and detection). Another BAV-phenotype is an AA aneurysm (Figure 1(d–f)) with increasing evidence suggesting a hemodynamic origin resulting in eccentric flow with high helicity, leading, especially in AS, to greater WSS in the greater curvature of the ascending aorta (Youssefi; Quelle 46)
Related Knowledge Centers
- Abdominal Aortic Aneurysm
- Aorta
- Aortic Aneurysm
- Aortic Dissection
- Hypotension
- Hypovolemic Shock
- Tachycardia
- Heart
- Artery
- Traumatic Aortic Rupture