International Registry Of Aortic Dissection Research Articles

Long-term outcomes after recurrent acute thoracic aortic dissection: Insights from the International Registry of Aortic Dissection

ObjectiveWith an aging population and advancements in imaging, recurrence of thoracic aortic dissection is becoming more common. MethodsAll patients enrolled in the International Registry of Aortic Dissection from 1996 to 2023 with type A and type B acute aortic dissection were identified. Among them, initial dissection and recurrent dissection were discerned. The study period was categorized into 3 eras: historic era, 1996 to 2005; middle era, 2006 to 2015; most recent era, 2016 to 2023. Propensity score matching was applied between initial dissection and recurrent dissection. Outcome of interests included long-term survival and cumulative incidence of major aortic events defined by the composite of reintervention, aortic rupture, and new dissection. ResultsThe proportion of recurrent dissection increased from 5.9% in the historic era to 8.0% in the most recent era in the entire dissection cohort. In patients with type A dissection, propensity score matching between initial dissection and recurrent dissection yielded 326 matched pairs. Kaplan–Meier curves showed similar long-term survival between the 2 groups. However, the cumulative incidence of major aortic events was significantly higher in the recurrent dissection group (40.3% ± 6.2% vs 17.8% ± 5.1% at 4 years in the initial dissection group, P = .02). For type B dissection, 316 matched pairs were observed after propensity score matching. Long-term survival and the incidence of major aortic events were equivalent between the 2 groups. ConclusionsThe case volume of recurrent dissection or the ability to detect recurrent dissection has increased over time. Acute type A recurrent dissection was associated with a higher risk of major aortic events than initial dissection. Further judicious follow-up may be crucial after type A recurrent dissection.

The Past, Present, and Future: 25 years of the International Registry of Aortic Dissection

The Past, Present, and Future: 25 years of the International Registry of Aortic Dissection

Editor's Choice – Trends in Management and Outcomes of Type B Aortic Dissection: A Report From the International Registry of Aortic Dissection

To describe the trends in management and outcomes of patients with acute type B aortic dissection in the International Registry of Acute Aortic Dissection. From 1996 - 2022, 3 908 patients were divided into similar sized quartiles (T1, T2, T3, and T4). In hospital outcomes were analysed for each quartile. Survival rates following admission were compared using Kaplan-Meier analyses with Mantel-Cox Log rank tests. Endovascular treatment increased from 19.1% in T1 to 37.2% in T4 (ptrend < .001). Correspondingly, medical therapy decreased from 65.7% in T1 to 54.0% in T4 (ptrend < .001), and open surgery from 14.8% in T1 to 7.0% in T4 (ptrend < .001). In hospital mortality decreased in the overall cohort from 10.7% in T1 to 6.1% in T4 (ptrend < .001), as well as in medically, endovascularly and surgically treated patients (ptrend= .017, .033, and .011, respectively). Overall post-admission survival at three years increased (T1: 74.8% vs. T4: 77.3%; p= .006). Considerable changes in the management of acute type B aortic dissection were observed over time, with a significant increase in the use of endovascular treatment and a corresponding reduction in open surgery and medical management. These changes were associated with a decreased overall in hospital and three year post-admission mortality rate among quartiles.

Rapid echocardiography in the primary angioplasty era for timely detection and management of acute Stanford type A aortic dissection: a case illustration

Acute Stanford type A aortic dissection (ATAD), comprising two-thirds of all captured aortic dissections (AD) in the International Registry of Aortic Dissection (IRAD) series, is a life-threatening cardiothoracic emergency.[1][1] Inpatient mortality without surgical intervention is approximately 50

P5608Acute respiratory failure after type A aortic dissection repair: data from the International Registry of Aortic Dissection (IRAD)

Abstract Background Acute Respiratory Failure (ARF) has been noted in up to 20% of patients undergoing cardiac surgery and is associated with increased mortality. Cardiopulmonary bypass (CPB) is often followed by pulmonary dysfunction, although literature on the subject in the setting of Type A acute aortic dissection (TAAAD) is limited. Methods This study identified the incidence of ARF after TAAAD, associated risk factors, and the impact of ARF on early and late outcomes. All data have been derived from the International Registry of Acute Aortic Dissection (IRAD). Results Postoperative ARF (defined as ventilator support for ≥3 days, tracheostomy, and/or pneumonia) occurred in 434 (24.6%) of 1764 surgically managed TAAAD patients (mean age 60.1±14.2 years) from November 2001 until November 2017. Peripheral vessel procedures (6.4% v 2.8%, p=0.002), cerebral perfusion (89.2% v 82.3%, p&lt;0.001), use of hypothermic circulatory arrest (93% v 87.7%), longer arrest time (median 39 (Q1-Q3 27–128 minutes) v 31 (Q1-Q3 22.0–52.9 minutes)), and lower extremity ischemia (18.8% v 6.7%, p&lt;0.001) were more common in ARF patients. On multivariable logistic regression analysis, age ≥70 years (OR 1.019, 95% CI 1.005–1.034, p=0.008), current smoking (OR 1.744, 95% CI 1.184–2.570, p=0.005), peripheral vessel procedures (OR 2.457, 95% CI 1.132–5.334, p=0.023), presenting hypotension/shock (OR 2.036, 95% CI 1.336–3.102, p=0.001), lower extremity ischemia at surgery (OR 2.77, 95% CI 1.574–4.875, p&lt;0.001), concomitant coronary artery bypass graft (CABG) (OR 2.982, 95% CI 1.597–5.568, p=0.001), pre-operative acute renal failure (OR 2.532, 95% CI 1.350–4.749, p=0.004), and prolonged circulatory arrest time in minutes (OR 1.005, 95% CI 1.003–1.007, p&lt;0.001) were independently associated with ARF development. Patients with aortic valve replacement (AVR) were less likely to develop ARF (OR 0.497, 95% CI 0.308–0.802, p=0.004). Post-operative complications were more common in ARF patients. In-hospital mortality was higher in the ARF cohort (16.4% v 4.7%, p&lt;0.001). Multivariable logistic regression identified ARF (OR 2.686, 95% CI 1.647–4.381, p&lt;0.001) as well as pre-operative hypotension (OR 1.89, 95% CI 1.130–3.159, p=0.015), lower extremity ischemia (OR 2.77, 95% CI 1.545–4.998, p=0.001), pre-operative myocardial infarction (OR 3.141, 95% CI 1.058–9.33, p=0.039), and CABG (OR 1.988, 95% CI 1.011–3.909, p-value 0.047) as independent predictors of death. Conclusions Post-operative ARF is common after TAAAD repair; in-hospital complications and death are higher in this cohort. Acknowledgement/Funding W.L. Gore &amp; Associates, Inc.; Medtronic; Varbedian Aortic Fund; Hewlett Foundation; Mardigian Foundation; UM Faculty Group Practice; Ann &amp; Bob Aikens

Recurrent Aortic Dissection: Observations From the International Registry of Aortic Dissection

Recurrent Aortic Dissection: Observations From the International Registry of Aortic Dissection

Marfan syndrome is the main independent predictor of recurrent aortic dissection in patients enrolled in the International Registry of Aortic Dissection (IRAD).

In the October 2016 issue of Circulation (1), Isselbacher and colleagues published their observations regarding patients with recurrent aortic dissection enrolled in the International Registry of Aortic Dissection (IRAD).

Spontaneous healing of life-threatening iatrogenic type A aortic dissection

Acute Stanford type A aortic dissection is a life threatening disease associatedwith a 1 to 4% hourly death rate if left untreated [1]. Although relatively rare, aortic dissection may be caused by left cardiac catheterization. Usually such iatrogenic dissection of the ascending aorta occurs as a consequence of retrograde extension of coronary artery dissection. Here is reported the favourable outcome with medical management of a patient with catheter-induced acute aortic dissection originating from the abdominal aorta and extending to the right coronary artery. A 61-year-old woman was referred to the intensive care unit for the management of a guide catheter-induced aortic dissection. Elective coronary angiography via right femoral artery access was motivated by exercise-induced angina.While it revealed normal left coronary arteries and right coronary dissection, occlusion was documented. Contrast opacification of false lumen was also seen within the abdominal aortic wall. Admission blood cuff pressure was asymmetric (105/50 mm Hg in the right arm and 85/45 mm Hg in the left one). Jugular vein distension was found on physical examination. EKG showed inferior and apical ST segment elevation and reciprocal changes in lateral leads (Fig. 1). Bedside transthoracic echocardiogram showed inferior wall akinesia and left ventricular ejection fraction at 0.45. Neither aortic regurgitation nor pericardial effusion was found. The right ventricle was enlarged and hypokinetic. CT angiography confirmed the retrograde Stanford type A aortic dissection extending proximally from the abdominal aorta below the renal arteries, to the right coronary artery (Fig. 2 a, b, c). No additional entry site for blood flow into the false lumen was found. Conservative treatment strategy was decided due to both high operative risk associated with ongoing acute myocardial infarction and absence of entry tear in the ascending aorta. Peak cardiac troponin I was 132 ng/ml (N b 0.5) at day 2. The patient received morphine, angiotensin converting enzyme inhibition, preventive heparin regimen and later on, beta-adrenergic blockade. Repeat CT scan at day 7 showed healing of the ascending aortic dissection (Fig. 2 d, e, f). The patient was discharged at day 9. A third CT scan at 1 month confirmed complete healing of the dissection, showing a normalized aortic wall (Fig. 2 g, h, i). 3D reconstructive CT scan (Fig. 2 j) documented severe aortic calcifications that likely promoted the entry intimal tear. The patient is doing well at three-year follow-up. Acute aortic dissection is a challenging clinical emergency requiring urgent surgical treatment. Iatrogenic aortic dissection due to catheterization was reported in 5 patients (1.7%) amongst the 289 patients presenting with a Stanford type A aortic dissection and in 5 (2.8%) amongst the 175 presenting with type B, in the International Registry of Aortic Dissection [1]. Overall in-hospital mortality of iatrogenic type A aortic dissection is reported to be up to 86% without surgical repair [2]. The majority of type A aortic dissections are related to retrograde extension of coronary dissection to the adjacent aortic wall and often linked with coronary angiography or percutaneous coronary intervention [3]. The incidence of guide catheter induced coronary dissection involving the aortic root is reported to be of 0.02% [4]. Treatment of the primary entry intimal tear by coronary stenting in patients with limited aortic extension [5] and surgical treatment [6] for those with greater aortic involvement have been reported with variable outcomes. In this case the patient had clinical evidence of poor prognosis including acute myocardial infarction and the associated risk of multiple organ failure due to unstable hemodynamic condition and abdominal aortic dissection. Furthermore, she was denied surgical treatment in spite of the “theoretical” Stanford type A aortic dissection presentation. The primary entry tear occurred in the calcified abdominal aorta and the International Journal of Cardiology 177 (2014) e78–e80

Reply to Hajj-Chahine

We would like to thank J. Hajj-Chahine [1] for his insightful comments on our paper, specifically regarding the discrepancy in the outcomes of patients with postsurgical iatrogenic acute aortic dissection type A (iAADA) presented in our study [2] vs results others have reported [3–5]. We reported on 100 patients with iAADA enrolled in the German Registry for Acute Aortic Dissection Type A (GERAADA) between 2006 and 2010. Postsurgical iatrogenic dissection was diagnosed in 43 patients, and their 30-day mortality was 12% (5/ 43). We agree that this low mortality stands in contrast to previous studies reporting rather dramatic in-hospital mortality of up to 60%. There are several reasons for the discrepancy between our data and other reports. First, in our report, patients underwent surgery between 2006 and 2010. Individual centres usually report results covering the last two or more decades. The investigation of iAADA according to the International Registry of Aortic Dissection was undertaken a decade ago and included patients operated on between 1996 and 2000 [3]. The fact that our study represents contemporary experience may be essential, as we have already observed and reported on significant outcome differences between patients with acute aortic dissection type A treated a few decades ago and those treated very recently [6]. Secondly, patients with postsurgical iAADA underwent a less aggressive surgical approach than those presenting spontaneous dissection. Ascending aortic replacement without aortic root and/or arch repair (performed most likely due to surgeons’ awareness of poor iatrogenicdissection results) might lead to anecdotally better contemporary early outcomes. However, those patients’ long-term survival and reintervention rates remain unknown. Finally, it is more than conceivable that if an iatrogenic dissection occurs in the operating room and is detected in time, prolonging surgery to permit ascending aortic replacement is feasible with an acceptable outcome. Therefore, an intraoperative iAADA does not necessarily need to increase the perioperative risk dramatically. Iatrogenic aortic dissection remains a devastating and often lethal complication necessitating emergency corrective surgery. The GERAADA registry’s contemporary results describing outcomes in patients with iAADA are simply more encouraging than those reported previously, and for good reason.

Post-cardiotomy type A aortic dissection

I read with great interest the article by Rylski et al. [1] investigating the management and outcomes of iatrogenic acute type A aortic dissection (AAAD) in the current era by extracting data from an international registry. AAAD after non-aortic cardiac surgery is considered an extremely dreadful complication, converting a low-risk procedure into a high-risk rescue situation. In the present report, the authors found that 30-day mortality after surgical correction of postsurgical-induced AAAD (n = 43 patients) is 12%. This relatively low mortality rate contradicts the findings of previous reports. A report from the International Registry of Aortic Dissection published in 2002 [2] establishes that the mortality of iatrogenic AAAD is higher than that of spontaneous aortic dissection. They identified in this observational registry 18 patients with postsurgical-induced AAAD with a mortality rate of 32%. Recently, Leontyev et al. [3] reported the outcomes of 36 patients with postsurgical-induced AAAD in a single high-volume cardiac surgery centre over a 14-year period. Once again, the mortality rate was high in this subset of patients at 35.5% for those diagnosed perioperatively and exceedingly high at 60% for those detected in the early postoperative period. More recently, the largest post-cardiotomy AAAD report [4] has been published with similarly high in-hospital mortality rate. Of note, the overall operative mortality observed was 27% in a cohort of 103 patients. This paper was the collaborative result between nine heart centres in four European countries. Although this study excluded patients with previous aortic surgery for aneurysm or dissection, the mortality rate was somewhat higher than that in the current published series. The discrepancy between data can be attributed to ambiguous definitions and varying inclusion and exclusion criteria. Therefore, for accurate comparison of data for future reporting, Stanger et al. [4] advocated using the following definitions. AAAD should be categorized into four different entities depending on the time interval between the primary cardiac surgery and the diagnosis of the dissection. AAAD is considered intraoperative when the dissection is recognized and treated during the initial cardiac surgery. Dissection within 2–4 weeks of index cardiac surgery is classified as early postoperative AAAD and this occurrence is associated with a woefully high risk of mortality. Late dissection is defined as occurring >30 days after primary heart surgery. Symptomatic patients are considered having late acute dissection, however late chronic dissection is a radiological incidental finding without acute symptoms. In the latest report [4], the mortality rates for intraoperative, early postoperative, late acute and chronic AAAD were 17, 42, 32 and 22%, respectively. Another important finding is the valuable effect of preoperative coronary angiography implementation to assess coronary artery and grafts status especially in patients with previous coronary artery bypass grafting surgery. With the increasing number of patients undergoing heart surgery and because previous cardiac surgery is increasingly recognized as an additional risk factor for aortic dissection [5], the incidence of post-cardiotomy aortic dissection will continue to increase. With careful planning by using preoperative coronary angiography and prompt execution, the outcomes in redo sternotomy operations for aortic dissection will parallel the results of spontaneous aortic dissection.

The IRAD Classification System for Characterizing Survival after Aortic Dissection

BackgroundThe classification of aortic dissection into acute (<14 days from symptom onset) versus chronic (≥14 days) is based on survival estimates of patients treated decades before modern diagnostic and treatment modalities were available. A new classification of aortic dissection in the current era may provide clinicians with a more precise method of characterizing the interaction of time, dissection location, and treatment type with survival. MethodsWe developed separate Kaplan-Meier survival curves for Type A and Type B aortic dissection using data from the International Registry of Aortic Dissection (IRAD). Daily survival was stratified based on type of therapy provided: medical therapy alone (medical), nonsurgical intervention plus medical therapy (endovascular), and open surgery plus medical therapy (surgical). The log-rank statistic was used to compare the survival curves of each management type within Type A and Type B aortic dissection. ResultsThere were 1815 patients included, 67.3% male with mean age 62.0 ± 14.2 years. When survival curves were constructed, 4 distinct time periods were noted: hyperacute (symptom onset to 24 hours), acute (2-7 days), subacute (8-30 days), and chronic (>30 days). Overall survival was progressively lower through the 4 time periods. ConclusionsThis 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.

Invited commentary

This is a well-written article by an experienced group with a relatively large number of patients treated for complicated acute type B aortic dissections. The International Registry of Acute Dissection published data noting an in-hospital mortality of 13% for all patients with acute type B dissection, with branch vessel involvement or malperfusion being an independent predictor of early death. An open surgical repair carries a mortality risk of at least 30%1Suzuki T. Mehta R.H. Ince H. Nagai R. Sakomura Y. Weber F. et al.Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD).Circulation. 2003; 108: II312-II317PubMed Google Scholar; consequently, endovascular repair has become a very attractive option. Feezor et al2Feezor R.J. Martin T.D. Hess Jr, P.J. Beaver T.M. Klodell C.T. Lee W.A. Early outcomes after endovascular management of acute, complicated type B aortic dissection.J Vasc Surg. 2009; 49 (discussion:6-7): 561-566Abstract Full Text Full Text PDF PubMed Scopus (67) Google Scholar reported their experience with thoracic endovascular aortic repair for 33 patients with acute complicated type B dissection. The 30-day in-hospital mortality for their group was 21%, with 15% having permanent spinal cord complications, 12% having renal failure requiring dialysis, and a 12% incidence of stroke. Clearly, most published results are far better than this,3Eggebrecht H. Nienaber C.A. Neuhauser M. Baumgart D. Kische S. Schmermund A. et al.Endovascular stent-graft placement in aortic dissection: a meta-analysis.Eur Heart J. 2006; 27: 489-498Crossref PubMed Scopus (435) Google Scholar and the results of this manuscript reflect this as well. Nevertheless, I find very surprising and not in line with published results, that with the authors' technique of extensive aortic coverage to the celiac artery, lack of spinal protection, and 78% of patients being American Society of Anesthesiologists IV or V (an independent risk factor for poor outcome), that they would essentially have 0% paraplegia. Persistent false lumen patency is associated with continued aortic expansion and seen in about 50% of proximally stented patients. Computed tomography (CT) imaging does not reliably identify re-entry tears and, as such, extensive coverage may not necessarily provide better long-term results if there are distal tears. The provisional extension to induce complete attachment (PETTICOAT) concept may perform better if extensive apposition of the dissection flap is required. Finally, the authors conclude that the volumetric analysis of true and false lumens using 3D CT is effective; however, there may be a potential flaw in their imaging technique. They perform CT images that are not electrocardiographically gated or dynamic; consequently, they can only get a time-averaged volume of the true and false lumens. We have published on dynamic aspects of aortic dissections4Karmonik C. Bismuth J. Davies M.G. Lumsden A.B. Computational fluid dynamics as a tool for visualizing hemodynamic flow patterns.Methodist Debakey Cardiovasc J. 2009; 5: 26-33PubMed Google Scholar, 5Karmonik C. Bismuth J. Davies M.G. Shah D.J. Younes H.K. Lumsden A.B. A computational fluid dynamics study pre- and post-stent graft placement in an acute type B aortic dissection.Vasc Endovasc Surg. 2010 Dec 13; ([Epublication ahead of print])PubMed Google Scholar, 6Karmonik C. Bismuth J. Davies M.G. Younes H.K. Lumsden A.B. An image processing algorithm for the in-vivo quantification and visualization of septum motion in type III B − aortic dissections with cine magnetic resonance imaging.Conf Proc IEEE Eng Med Biol Soc. 2009; 2009: 4391-4394PubMed Google Scholar, 7Karmonik C. Bismuth J. Redel T. Anaya-Ayala J.E. Davies M.G. Shah D.J. et al.Impact of tear location on hemodynamics in a type B aortic dissection investigated with computational fluid dynamics.Conf Proc IEEE Eng Med Biol Soc. 2010; 1: 3138-3141Google Scholar, 8Karmonik C. Bismuth J.X. Davies M.G. Lumsden A.B. Computational hemodynamics in the human aorta: a computational fluid dynamics study of three cases with patient-specific geometries and inflow rates.Technol Health Care. 2008; 16: 343-354PubMed Google Scholar and find a variation in the true/false lumen of up to 20% to 50% throughout the cardiac cycle. Additionally, the benefit of dynamic imaging goes beyond simple appreciation of a septum, but rather fundamentally how that septum acts (mobility). We have found that a septum may by age be categorized as acute, but act more chronic with a rigid septum. This has potential implications for the therapy. Finally, I think knowing the volumetric analysis for a case control group being managed medically would potentially provide many answers, and could affect the management paradigm. Aortic remodeling, volumetric analysis, and clinical outcomes of endoluminal exclusion of acute complicated type B thoracic aortic dissectionsJournal of Vascular SurgeryVol. 54Issue 2PreviewStructural changes within the aorta after thoracic endovascular aortic repair (TEVAR) for acute complicated type B thoracic aortic dissections (ABAD) remain unknown. This study reviewed and analyzed morphologic changes, volumetric data, and clinical outcomes of patients with ABAD. Full-Text PDF Open Archive

Traumatic dissection and rupture of the abdominal aorta as a complication of the Heimlich maneuver

Although the Heimlich maneuver is considered the best intervention for relieving acute upper airway obstruction, several complications have been reported in the literature. These complications can occur as a result of an increase in abdominal pressure leading to a variety of well-documented visceral injuries, including the great vessels. Acute abdominal aortic thrombosis after the Heimlich maneuver is a rare but recognized event; however, to date no case of traumatic dissection and rupture of the abdominal aorta has been described. We report the first known case, to our knowledge, of a traumatic dissection and rupture of the abdominal aorta after a forcefully applied Heimlich maneuver.

Endovascular Management of Thoracic Dissections

The first surgical management of aortic dissection was reported in 1935 by Gurin et al,1 who created a distal reentry point in the iliac artery to decompress the false lumen. In 1949, Abbott2 reported the repair of a chronic dissection by wrapping cellophane around the descending aorta to reinforce it. Despite the efforts of these early pioneers and other investigators, it was not until 1955 that a major therapeutic advance was made; this was the year that DeBakey, Cooley, and Creech introduced a revolutionary surgical treatment that involved excision of the intimal tear, obliteration of the false lumen, and either direct reanastomosis or insertion of a prosthetic graft.3 The next great milestone in therapy was introduced by Wheat et al4 in 1965, when they described medical therapy directed toward lowering blood pressure and dP/dt. Since then, investigators have made significant advances in the detection, characterization, and treatment of aortic dissection; however, the morbidity and mortality of this debilitating disease remain alarmingly high, with an overall in-hospital mortality of 27.4% reported by the International Registry of Aortic Dissection (IRAD).5 The latest additions to the armamentarium to treat dissection have been based on percutaneous interventional techniques. The minimally invasive nature of these techniques makes them an attractive alternative to open surgical intervention; however, the exact role and long-term durability of these procedures remain to be proven. The earliest endovascular therapies were directed toward the complications of aortic dissections and included angioplasty of an obstructed aorta, stenting of obstructed branch vessels, and fenestration of the dissection flap to relieve mesenteric ischemia.6–8 More recently, the advent of the stent graft has led to a novel endovascular approach aimed at treating the inciting lesion of aortic dissection by obliterating the primary intimal tear.9,10 Since the first reports in …

Dynamic Aortic Obstruction in a Patient With Acute Type B Dissection

There are at least 7000 aortic dissections diagnosed in the United States each year. Type B dissections accounted for 38% of cases enrolled in the prospective International Registry of Aortic Dissection. We report a case of a 48-year-old hypertensive woman with an acute type B aortic dissection causing significant dynamic obstruction of the aorta. Intravascular ultrasound of her aorta revealed a mobile intimal flap nearly obliterating the true lumen with each systolic contraction. Simultaneous pressure tracings obtained from her ascending aorta and femoral artery demonstrated a systolic pressure gradient in excess of 100 mm Hg. The patient developed progressive renal failure and ultimately underwent successful operative replacement of the proximal descending thoracic aorta with a Dacron graft. In this case presentation, we highlight the unusual physiology exemplified by this case and explore contemporary management strategies for complicated type B aortic dissection, including surgery and catheter-based techniques.

Acute intramural hematoma of the aorta: A mystery in evolution

Conclusion: There is a 5-7% prevalence of intramural hematoma (IMH) in patients with acute aortic syndromes with 16% of patients evolving to classic aortic dissection (AD) on subsequent imaging studies and a 40% mortality rate of ascending aorta IMH. Summary: Acute aortic syndromes are characterized by a sudden onset of thoracic pain and consist of both classic AD and intramural aortic hemorrhage. There is an incomplete knowledge of the natural history of IMH. The International Registry of Aortic Dissection (IRAD) involves eighteen referral centers in six countries. Potential patients for inclusion in IRAD are identified prospectively or retrospectively by searching hospital discharge records. Diagnosis of acute aortic syndrome is suspected on history and physical examination and confirmed by imaging study or surgical exploration or post mortem examination. The current study represents 1,1010 patients enrolled between January 1, 1996 and November 19, 2001. Fifty-eight (5.7%) of patients had IMH. Patients with IMH tended to be older (68.7 years) than those with classic AD (61.7 years, P < 0.001). Patients with IMH were also more likely to have distal aortic involvement than patients with classic AD (60.3% versus 35.3%; P < 0.001). Patients with IMH described more severe initial pain than those with classic AD. Patients with IMH, however, were less likely to have pulse deficits, ischemic leg pain, or aortic valvular insufficiency. Overall, mortality of IMH was similar to AD (20.7% versus 23.9%; P = 0.57). Mortality of patients with IMH of the descending aorta was 8.3% versus 13.1% of patients with AD of the descending aorta (P = 0.60). Mortality of patients with IMH of the ascending aorta was 39.1% versus 29.9% of patients with classic AD of the ascending aorta; P = 0.34. Sixteen percent of patients with an initial diagnosis of IMH progressed to AD on serial imaging studies. Comment: The natural history of acute IMH of the aorta as well as its prevalence is difficult to determine. The current study suggests that the prevalence of IMH in patients with non-traumatic acute aortic syndromes is about 6%. This is lower than previously reported. Overall, however, this study, and others suggest, that despite the more limited aortic involvement of IMH and the absence of true dissection flaps at initial presentation, the overall natural history of the disease is similar to that of classic AD. At this point the known natural history of IMH suggests treatment and follow up should be similar to AD.

Acute Intramural Hematoma of the Aorta

The definition, prevalence, outcomes, and appropriate treatment strategies for acute intramural hematoma (IMH) continue to be debated. We studied 1010 patients with acute aortic syndromes who were enrolled in the International Registry of Aortic Dissection (IRAD) to delineate the prevalence, presentation, management, and outcomes of acute IMH by comparing these patients with those with classic aortic dissection (AD). Fifty-eight (5.7%) patients had IMH, and this cohort tended to be older (68.7 versus 61.7 years; P<0.001) and more likely to have distal aortic involvement (60.3% versus 35.3%; P<0.001) compared with 952 patients with AD. Patients with IMH described more severe initial pain than did those with AD but were less likely to have ischemic leg pain, pulse deficits, or aortic valve insufficiency; moreover, they required a longer time to diagnosis and more diagnostic tests. Overall mortality of IMH was similar to that of classic AD (20.7% versus 23.9%; P=0.57), as was mortality in patients with IMH of the descending aorta (8.3% versus 13.1%; P=0.60) and the ascending aorta (39.1% versus 29.9%; P=0.34) compared with AD. IMH limited to the aortic arch was seen in 7 patients, with no deaths, despite medical therapy in only 6 of the 7 individuals. Among the 51 patients whose initial diagnostic study showed IMH only, 8 (16%) progressed to AD on a serial imaging study. The IRAD data demonstrate a 5.7% prevalence of IMH in patients with acute aortic syndromes. Like classic AD, IMH is a highly lethal condition when it involves the ascending aorta and surgical therapy should be considered, but this condition is less critical when limited to the arch or descending aorta. Fully 16% of patients have evidence of evolution to dissection on serial imaging.

Comparison of aortic dissection in patients with and without Marfan’s syndrome (results from the international registry of aortic dissection)

Comparison of aortic dissection in patients with and without Marfan’s syndrome (results from the international registry of aortic dissection)

Comparison of aortic dissection in patients with and without Marfan's syndrome (results from the International Registry of Aortic Dissection)

Among 1,049 patients diagnosed with aortic dissection in a multinational registry, patients with Marfan's syndrome were typically younger and had unique presentations. Despite their younger age at presentation, patients with Marfan's syndrome and aortic dissection had a high mortality rate, similar to patients without Marfan's syndrome, an older patient cohort. Our data support the importance of aneurysm surveillance and prophylactic surgical intervention for patients with Marfan's syndrome to potentially reduce the risk of mortality.

Characterizing the young patient with aortic dissection: Results from the international registry of aortic dissection (IRAD)

Characterizing the young patient with aortic dissection: Results from the international registry of aortic dissection (IRAD)