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The effects of medications on the outcome of aortic dissection remain poorly understood. We sought to address this by analyzing the International Registry of Acute Aortic Dissection (IRAD) global registry database. A total of 1,301 patients with acute aortic dissection (722 with type A and 579 with type B) with information on their medications at discharge and followed for ≤5 years were analyzed for the effects of the medications on mortality. The initial univariate analysis showed that use of β blockers was associated with improved survival in all patients (p = 0.03), in patients with type A overall (p = 0.02), and in patients with type A who received surgery (p = 0.006). The analysis also showed that use of calcium channel blockers was associated with improved survival in patients with type B overall (p = 0.02) and in patients with type B receiving medical management (p = 0.03). Multivariate models also showed that the use of β blockers was associated with improved survival in those with type A undergoing surgery (odds ratio 0.47, 95% confidence interval 0.25 to 0.90, p = 0.02) and the use of calcium channel blockers was associated with improved survival in patients with type B medically treated patients (odds ratio 0.55, 95% confidence interval 0.35 to 0.88, p = 0.01). In conclusion, the present study showed that use of β blockers was associated with improved outcome in all patients and in type A patients (overall as well as in those managed surgically). In contrast, use of calcium channel blockers was associated with improved survival selectively in those with type B (overall and in those treated medically). The use of angiotensin-converting enzyme inhibitors did not show association with mortality.

Acute aortic syndromes are highly morbid conditions that require prompt diagnosis and management. Aortic dissections have rhythmic patterns, with notable peaks at certain points in every 24 hours as well as weekly and seasonal variations. Several retrospective studies have assessed the chronobiology of acute aortic dissections and there seems to be a winter seasonal peak and morning daily peak in incidence. Although the pathophysiology of this chronobiology is unclear, there are several environmental and physiologic possibilities. This article reviews the major studies examining the chronobiology of acute aortic dissection, and summarizes some theories on the pathophysiology of this phenomenon.

Capillary malformation-arteriovenous malformation (CM-AVM) is an autosomal-dominant disorder, caused by heterozygous RASA1 mutations, and manifesting multifocal CMs and high risk for fast-flow lesions. A limited number of patients have been reported, raising the question of the phenotypic borders. We identified new patients with a clinical diagnosis of CM-AVM, and patients with overlapping phenotypes. RASA1 was screened in 261 index patients with: CM-AVM (n=100), common CM(s) (port-wine stain; n=100), Sturge-Weber syndrome (n=37), or isolated AVM(s) (n=24). Fifty-eight distinct RASA1 mutations (43 novel) were identified in 68 index patients with CM-AVM and none in patients with other phenotypes. A novel clinical feature was identified: cutaneous zones of numerous small white pale halos with a central red spot. An additional question addressed in this study was the second-hit hypothesis as a pathophysiological mechanism for CM-AVM. One tissue from a patient with a germline RASA1 mutation was available. The analysis of the tissue showed loss of the wild-type RASA1 allele. In conclusion, mutations in RASA1 underscore the specific CM-AVM phenotype and the clinical diagnosis is based on identifying the characteristic CMs. The high incidence of fast-flow lesions warrants careful clinical and radiologic examination, and regular follow-up. (C) 2013 Wiley Periodicals, Inc.

Few data exist on race-related differences in acute aortic dissection patients.We evaluated black (n = 189, 14%) or white (n = 1165, 86%) patients (mean age 62.8 ± 15.3 years; 36.4% women) enrolled in 13 US centers participating in the International Registry of Acute Aortic Dissection. We excluded patients of other racial descent.Type B acute aortic dissection was more frequent in the black cohort (52.4% vs 39.3%, P = .001). Black patients were younger (mean age 54.6 ± 12.8 years vs 64.2 ± 15.2 years, P.001) and more likely to have a history of cocaine abuse (12% vs 1.6%, P.001), hypertension (89.7% vs 73.9%, P .001), and diabetes (13.2% vs 6.4%, P = .001). Conversely, they were less likely to have bicuspid aortic valve (1.8% vs 5.8%, P = .029), iatrogenic dissection (0.5% vs 4.5%, P = .010), and prior aortic dissection repair (7.7% vs 12.8%, P = .047). Presenting features were similar except for more abdominal pain (44.6% vs 30.6%, P.001) and left ventricular hypertrophy on echocardiogram (44.2% vs 20.1%, P .001) in blacks. Management was similar. Hypotension/shock/tamponade was less common (7.6% vs 20.1%, P.001), whereas acute kidney failure was more common (41.0% vs 21.7%, P.001) in blacks. Mortality was similar in-hospital (14.3% vs 19.1%, P = .110, odds ratio 0.704, 95% confidence interval 0.457-1.085) and at 3 years postdischarge, as evaluated by Kaplan-Meier survival analysis (22.0% vs 14.3%, P = .224, SE = 0.062 and 0.018).Our study shows differences in type, etiology, and presentation of blacks and whites with acute aortic dissection, yet similar mortality for these cohorts.

Marfan syndrome (MFS) is an autosomal dominant connective tissue disease associated with acute aortic dissection (AAD). We used 2 large registries that include patients with MFS to investigate possible trends in the chronobiology of AAD in MFS. We queried the International Registry of Acute Aortic Dissection (IRAD) and the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) registry to extract data on all patients with MFS who had suffered an AAD. The group included 257 patients with MFS who suffered an AAD from 1980 to 2012. The chi-square tests were used for statistical testing. Mean subject age at time of AAD was 38 years, and 61% of subjects were men. AAD was more likely in the winter/spring season (November to April) than the other half of the year (57% vs 43%, p = 0.05). Dissections were significantly more likely to occur during the daytime hours, with 65% of dissections occurring from 6 a.m. to 6 p.m. (p = 0.001). Men were more likely to dissect during the daytime hours (6 a.m. to 6 p.m.) than women (74% vs 51%, p = 0.01). These insights offer a glimpse of the times of greatest vulnerability for patients with MFS who suffer from this catastrophic event. In conclusion, the chronobiology of AAD in MFS reflects that of AAD in the general population.

Aims Shock is among the most dreaded and common complications of type A acute aortic dissection (TAAAD). However, clinical correlates, management, and short- and long-term outcomes of TAAAD patients presenting with shock in real-world clinical practice are not known. Methods and Results We evaluated 2,704 patients with TAAAD enrolled in the International Registry of Acute Aortic Dissection between January 1, 1996, and August 18, 2012. On admission, 407 (15.1%) TAAAD patients presented with shock. Most in-hospital complications (coma, myocardial or mesenteric ischemia or infarction, and cardiac tamponade) were more frequent in shock patients. In-hospital mortality was significantly higher in TAAAD patients with than without shock (30.2% vs 23.9%, P =.007), regardless of surgical or medical treatment. Most shock patients underwent surgical repair, with medically managed patients demonstrating older age and more complications at presentation. Estimates using Kaplan-Meier survival analysis indicated that most (89%) TAAAD patients with shock discharged alive from the hospital survived 5years, a rate similar to that of TAAAD patients without shock (82%, P =.609). Conclusions Shock occurred in 1 of 7 TAAAD patients and was associated with higher rates of in-hospital adverse events and mortality. However, TAAAD survivors with or without shock showed similar long-term mortality. Successful early and aggressive management of shock in TAAAD patients has the potential for improving long-term survival in this patient population.

Background Acute aortic dissection associated with cocaine use is rare and has been reported predominantly as single cases or in small patient cohorts.Methods Our study analyzed 3584 patients enrolled in the International Registry of Acute Aortic Dissection from 1996 to 2012. We divided the population on the basis of documented cocaine use (C+) versus noncocaine use (C-) and further stratified the cohorts into type A (33 C+/2332, 1.4%) and type B (30 C+/1252, 2.4%) dissection.Results C+ patients presented at a younger age and were more likely to be male and black. Type B dissections were more common among C+ patients than in C- patients. Cocaine-related acute aortic dissection was reported more often at US sites than at European sites (86.4%, 51/63 vs 13.6%, 8/63; P

Background— Stroke is a highly dreaded complication of type A acute aortic dissection (TAAAD). However, little data exist on its incidence and association with prognosis. Methods and Results— We evaluated 2202 patients with TAAAD (mean age 62±14 years, 1487 [67.5%] men) from the International Registry of Acute Aortic Dissection to determine the incidence and prognostic impact of stroke in TAAAD. Stroke was present at arrival in 132 (6.0%) patients with TAAAD. These patients were older (65±12 versus 62±15 years; P =0.002) and more likely to have hypertension (86% versus 71%; P =0.001) or atherosclerosis (29% versus 22%; P =0.04) than patients without stroke. Chest pain at arrival was less common in patients with stroke (70% versus 82%; P P P =0.005), or pulse deficit (51% versus 29%; P ≤0.001). Arch vessel involvement was more frequent among patients with stroke (68% versus 37%; P P P Conclusions— Stroke occurred in >1 of 20 patients with TAAAD and was associated with increased in-hospital morbidity but not long-term mortality. Whether aggressive early invasive interventions will reduce negative outcomes remains to be evaluated in future studies.

Background— In patients with acute type B aortic dissection, presence of recurrent or refractory pain and/or refractory hypertension on medical therapy is sometimes used as an indication for invasive treatment. The International Registry of Acute Aortic Dissection (IRAD) was used to investigate the impact of refractory pain and/or refractory hypertension on the outcomes of acute type B aortic dissection. Methods and Results— Three hundred sixty-five patients affected by uncomplicated acute type B aortic dissection, enrolled in IRAD from 1996 to 2004, were categorized according to risk profile into 2 groups. Patients with recurrent and/or refractory pain or refractory hypertension (group I; n=69) and patients without clinical complications at presentation (group II; n=296) were compared. “High-risk” patients with classic complications were excluded from this analysis. The overall in-hospital mortality was 6.5% and was increased in group I compared with group II (17.4% versus 4.0%; P =0.0003). The in-hospital mortality after medical management was significantly increased in group I compared with group II (35.6% versus 1.5%; P =0.0003). Mortality rates after surgical (20% versus 28%; P =0.74) or endovascular management (3.7% versus 9.1%; P =0.50) did not differ significantly between group I and group II, respectively. A multivariable logistic regression model confirmed that recurrent and/or refractory pain or refractory hypertension was a predictor of in-hospital mortality (odds ratio, 3.31; 95% confidence interval, 1.04 to 10.45; P =0.041). Conclusions— Recurrent pain and refractory hypertension appeared as clinical signs associated with increased in-hospital mortality, particularly when managed medically. These observations suggest that aortic intervention, such as via an endovascular approach, may be indicated in this intermediate-risk group.

Background: The classification of aortic dissection into acute (30 days). Overall survival was progressively lower through the 4 time periods. Conclusions: This IRAD classification system can provide clinicians with a more robust method of characterizing survival after aortic dissection over time than previous methods. This system will be useful for treating patients, counseling patients and families, and studying new diagnostic and treatment methods.

BACKGROUND: Few data exist on clinical/imaging characteristics, management, and outcomes of patients with type A acute dissection and mesenteric malperfusion. METHODS: Patients with type A acute dissection enrolled in the International Registry for Acute Dissection (IRAD) were evaluated to assess differences in clinical features, management, and in-hospital outcomes according to the presence/absence of mesenteric malperfusion. A mortality model was used to identify predictors of in-hospital mortality in patients with mesenteric malperfusion. RESULTS: Mesenteric malperfusion was detected in 68 (3.7%) of 1809 patients with type A acute dissection. Patients with mesenteric malperfusion were more likely to be older and to have coma, cerebrovascular accident, spinal cord ischemia, acute renal failure, limb ischemia, and any pulse deficit. They were less likely to undergo surgical/hybrid treatment (52.9% vs 87.9%) and more likely to receive only medical (30.9% vs 11.6%) or endovascular (16.2% vs 0.5%) management (P < .001). Overall in-hospital mortality was 63.2% and 23.8% in patients with and without mesenteric malperfusion, respectively (P < .001). In-hospital mortality of patients with mesenteric malperfusion receiving medical, endovascular, and surgical/hybrid therapy was 95.2%, 72.7%, and 41.7%, respectively (P < .001). At multivariate analysis, male gender (odds ratio [OR], 1.7; P = .002), age (OR, 1.1/y; P = .002), and renal failure (OR, 5.9; P = .020) were predictors of mortality whereas surgical/hybrid management (OR, 0.1; P = .005) was associated with better outcome. CONCLUSIONS: Type A acute aortic dissection complicated by mesenteric malperfusion is a rare but ominous complication carrying a high risk of hospital mortality. Surgical/hybrid therapy, although associated with 2-fold hospital mortality, appears to be associated with better long-term outcomes in the management of type A acute aortic dissection in this setting.

Background Performing preoperative coronary angiography (CA) before surgical repair of a type A acute aortic dissection (TA-AAD) remains controversial. Although the information provided by CA may be useful in planning the surgical approach, the potential delay to surgery and complications of CA may confer added risk of death before definitive repair of the aorta. Methods We analyzed 1,343 patients from January 27, 1996, to May 3, 2010, with TA-AAD from the International Registry of Acute Aortic Dissection who underwent surgical or endovascular repair during the index hospitalization, with (n = 156) or without (n = 1,187) preoperative CA. The main outcomes measured were in-hospital complications and in-hospital and long-term mortality. Results Patients who underwent preoperative CA were more likely to have a history of atherosclerosis and present with electrocardiographic signs of myocardial ischemia/infarction. In the preoperative CA group, significant delays from the onset of symptoms to the time of surgery occurred. In-hospital postoperative complications and mortality rates were largely similar between the 2 groups. On multivariable logistic regression analysis, preoperative CA had no significant effect on in-hospital risk-adjusted mortality when compared to the validated International Registry of Acute Aortic Dissection risk score. Long-term mortality was similar between patients receiving preoperative CA and those who did not; long-term rehospitalization rates were higher, although largely insignificantly, among preoperative CA recipients through 5 years of follow-up. Conclusions Preoperative CA is infrequently performed on patients with TA-AAD, except, occasionally, on patients at high risk for myocardial ischemia. When performed, preoperative CA was not associated with any significant changes in in-hospital and long-term mortality.