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The DeBakey classification divides type A acute aortic dissection (AAD) into type I and type II; the latter limited to the ascending aorta. We endeavored to examine differences in DeBakey groups in a contemporary registry. We divided 1,872 patients with noniatrogenic AAD from the International Registry of Acute Aortic Dissection into type I (n = 1691, 90.3%) and type II (n = 181, 9.7%). Patients with type II AAD were older. On presentation, patients with type I AAD reported more back and abdominal pain and were more likely to have pulse deficit. Intramural hematoma was more frequent in type II AAD. Most patients with both types were treated surgically. Lower rates of renal failure, coma, mesenteric and limb ischemia were noted in those with type II AAD. In-hospital death was less frequent (16.6% vs 22.5%) after type II AAD, a trend that did not reach significance. There was no difference in the incidence of new dissection, rapid aortic growth, late aortic intervention or survival at 5 years. In conclusion, AAD limited to the ascending aorta (DeBakey type II) appears to be associated with improved clinical outcomes compared with dissection that extend to the aortic arch or beyond. Although fewer dissection-related complications were noted in patients presenting with type II AAD, as was a trend toward reduced in-hospital mortality, 5-year survival and descending aortic sequelae are not reduced in this contemporary report from International Registry of Acute Aortic Dissection., (Copyright © 2018 Elsevier Inc. All rights reserved.)

The Pediatric Heart Network randomized trial of atenolol versus losartan in the Marfan syndrome showed no treatment differences in the rates of aortic-root growth or clinical outcomes. In this report we present treatment effects on aortic stiffness and determine whether baseline aortic stiffness predicts aortic-root growth and clinical outcomes. Echocardiograms at 0, 6, 12, 24, and 36 months from 608 subjects (6 months to 25 years) who met original Ghent criteria and had a maximum aortic-root z-score (ARz) >3 were centrally reviewed. Stiffness index (SI) and elastic modulus (EM) were calculated for aortic root and ascending aorta. Data were analyzed using multivariable mixed effects modeling and Cox regression. Heart rate-corrected aortic-root SI over 3 years decreased with atenolol but did not change with losartan (-0.298 ± 0.139 vs 0.141 ± 0.139/year, p = 0.01). In the entire cohort, above-median aortic-root SI (>9.1) and EM (>618 mm Hg) predicted a smaller annual decrease in ARz (p ≤0.001). Upper-quartile aortic-root EM (>914 mm Hg) predicted the composite outcome of aortic-root surgery, dissection, or death (hazard ratio 2.17, 95% confidence interval 1.02 to 4.63, p = 0.04). Crude 3-year event rates were 10.4% versus 3.2% for higher versus lower EM groups. In conclusion, atenolol was associated with a decrease in aortic-root SI, whereas losartan was not. Higher baseline aortic-root SI and EM were associated with a smaller decrease in ARz and increased risk for clinical outcomes. These data suggest that noninvasive aortic stiffness measures may identify patients at higher risk of progressive aortic enlargement and adverse clinical outcomes, potentially allowing for closer monitoring and more aggressive therapy., (Copyright © 2018 Elsevier Inc. All rights reserved.)

This study analyzed the impact of sex, hemodynamic profile, and valve fusion pattern on aortopathy associated with bicuspid aortic valve (BAV). The National Heart Lung and Blood Institute-sponsored National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) provided comprehensive information on a large population of well-characterized patients with BAV. Of 969 enrolled patients with BAV, 551 (57%, 77% male) had already undergone valvular and/or aortic surgery. Echocardiographic imaging data were available on 339 unoperated or preoperative participants who formed the basis of this study. BAV function was normal in 45 (14%), with a predominant aortic regurgitation (AR) in 127 (41%) and a predominant aortic stenosis (AS) in 76 (22%). Moderate-severe AR was associated with larger sinus of Valsalva (SOV) diameters compared with normal function and AS (all p <0.01). Moderate-severe AS was associated with a larger ascending aortic (AscAo) diameter compared with normal function (p = 0.003) but not with AR. The SOV diameter was larger in men than in women (3.7 ± 0.7 vs 3.3 ± 0.6 cm, p <0.0001), whereas AscAo diameters were comparable (3.9 ± 0.9 vs 3.7 ± 0.9 cm, p = 0.08). Right-left commissural fusion was associated with a larger SOV diameter (3.7 ± 0.7 vs 3.3 ± 0.6 cm, p <0.0001) compared with a right-noncoronary fusion pattern. Predominant AR was more common in men (45% vs 27%, p = 0.004), whereas AS was more common in women (29% vs 18%, p = 0.04). In conclusion, in the GenTAC Registry, AR was associated with diffuse (annular, SOV, and AscAo) enlargement, whereas moderate-severe AS was only associated with AscAo enlargement. Male sex and right-left cusp pattern of cusp fusion were associated with larger SOV diameters and a greater likelihood of AR, whereas women had a higher prevalence of AS., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Little is known about the relation between type A acute aortic dissection (TAAAD) and pulse pressure (PP), defined as the difference between systolic and diastolic blood pressure. In this study, we explored the association between PP and presentation, complications, and outcomes of patients with TAAAD. PP at hospital presentation was used to divide 1,960 patients with noniatrogenic TAAAD into quartiles: narrowed (≤39 mm Hg, n=430), normal (40 to 56 mm Hg, n=554), mildly elevated (57 to 75 mm Hg, n=490), and markedly elevated (≥76 mm Hg, n=486). Variables relating to index presentation and in-hospital outcomes were analyzed. Patients with TAAAD in the narrowed PP quartiles were frequently older and Caucasian, whereas patients with markedly elevated PPs tended to be male and have a history of hypertension. Patients who demonstrated abdominal vessel involvement more commonly demonstrated elevated PPs, whereas patients with narrowed PPs were more likely to have periaortic hematoma and/or pericardial effusion. Narrowed PPs were also correlated with greater incidences of hypotension, cardiac tamponade, and mortality. Patients with TAAAD who were managed with endovascular and hybrid procedures and those with renal failure tended to have markedly elevated PPs. No difference in aortic regurgitation at presentation was noted among groups. In conclusion, patients with TAAAD in the third PP quartile had better in-hospital outcomes than patients in the lowest quartile. Patients with narrowed PPs experienced more cardiac complications, particularly cardiac tamponade, whereas those with markedly elevated PPs were more likely to have abdominal aortic involvement. Presenting PP offers a clue to different manifestations of acute aortic dissection that may facilitate initial triage and care., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Hereditary hemorrhagic telangiectasia is an autosomal dominant disorder characterized by vascular malformations, and many clinical complications are related to pulmonary arteriovenous malformations (PAVMs) because they provide direct right-to-left shunts. Paradoxical emboli through these shunts are a well-recognized cause of transient ischemic attack, stroke, and cerebral abscess. The aim of this study was to determine whether paradoxical emboli through PAVMs could manifest as cardiac ischemic events. The study included a single-center population of 98 patients with PAVMs. Eighty-four had undergone PAVM embolotherapy, and the remaining 14 patients had PAVMs too small to require embolization. Patients were interviewed by telephone and surveyed regarding their cardiopulmonary symptoms and histories of cardiac diagnoses. We found that 6 patients (which is 18% of patients with symptomatic PAVMs, n = 33, and 6% of the total cohort, n = 98) reported that they had experienced typical angina pectoris-like chest pain or had a myocardial infarction before PAVM embolotherapy. Five patients had had a cardiac catheterization, 4 had normal coronary arteries, and 1 had a single artery occlusion. In conclusion, we suggest that in patients with untreated PAVMs, cardiac ischemia can occur because of a paradoxical embolus through PAVMs to a coronary artery., (Copyright © 2013 Elsevier Inc. All rights reserved.)

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., (Copyright © 2011 Elsevier Inc. All rights reserved.)

The clinical, cardiac and echocardiographic features were reviewed of 15 consecutive infants and children with the Marfan syndrome who presented at less than 4 years of age. The 10 females and 5 males were diagnosed at a mean age of 18 months (range 2 days to 3.5 years); 13 were followed up for a mean of 45 months (range 8 to 109 months) and 2 were lost to follow-up. On first evaluation, 5 patients had mitral regurgitation (MR) and none had aortic regurgitation (AR). Echocardiography showed aortic root dilatation in 9 and mitral valve prolapse (MVP) in 9; only 2 patients had a normal echocardiogram. At follow-up examination, all patients had aortic root dilatation; the rate of increase in aortic root diameter was generally greater than predicted on the basis of body surface area. One patient had AR at age 10 years. All 13 patients had MVP; 9 had progressive mitral valve dysfunction and 4 had mitral valve replacement. One patient died immediately postoperatively. Thus, aortic root dilatation is usually present in early childhood and serves as an objective indicator of the Marfan syndrome. MVP is common and MR, which may require valve replacement, is the leading cause of cardiovascular morbidity in childhood. Echocardiography is useful in the diagnosis and routine management of infants and children in whom the Marfan syndrome is suspected.

Sporadic recent reports suggest that mitral valve prolapse (MVP) disappears with progressive left ventricular (LV) dilatation. To test this hypothesis, we sought to determine if an inverse relation exists between MVP and LV cavity size on M-mode echocardiograms in 83 patients with Marfan's syndrome. LV end-diastolic dimensions and presence or absence of MVP were determined. Forty-six patients had MVP. Of patients with an LV end-diastolic dimension less than or equal to 5 cm, 90% had MVP; only 19% of the 32 patients with abnormally large (greater than 5.8 cm) end-diastolic dimension had MVP. The prevalence of MVP in patients with an LV end-diastolic dimension of 5.1 to 5.8 cm was 69%. Thus, the prevalence of MVP was inversely related to LV cavity size. To determine whether appearance or disappearance of MVP was associated with decrease or increase in LV cavity size, serial echocardiograms from 67 patients (mean follow-up 42 months, range 3 to 99) were examined. These patients were separated into 3 groups based on changes in the LV end-diastolic dimension of greater than 1 cm over time. Group 1 consisted of 9 patients, all of whom had MVP and normal LV cavity size on their initial study. With subsequent increase in LV end-diastolic dimension (mean 1.42 +/- 0.3), MVP disappeared in 6 of the 9. Conversely, group 2 consisted of 4 patients, all of whom had dilated left ventricles on their initial echocardiogram and no evidence of MVP. After aortic valve replacement, the LV cavity size decreased (mean 2.3 +/- 0.7) and MVP appeared on follow-up studies.(ABSTRACT TRUNCATED AT 250 WORDS)