2014 ESC Guidelines on the diagnosis and treatment of aortic diseases

Erbel, Raimund; Aboyans, Victor; Boileau, Cathérine; Bossone, Eduardo; Rd, Bartolomeo; Eggebrecht, Holger; Evangelista, Arturo; Falk, Volkmar; Frank, Herbert; Gaemperli, Oliver; Grabenwöger, Martin; Haverich, Axel; Iung, Bernard; Aj, Manolis; Meijboom, Folkert J.; Ca, Nienaber; Roffi, Marco; Rousseau, Hervé; Sechtem, Udo; Pa, Sirnes; Rs, Allmen; Cj, Vrints

doi:10.1093/eurheartj/ehu281

Cited by 3,669 publications

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“…Diseases of the thoracic aorta are increasing in prevalence, accounting for 1% to 2% of all deaths in Western countries 1, 2, 3, 4, 5. In the United States, diseases of the aorta account for more than 40 000 deaths per year 1, 4.…”

Section: Introductionmentioning

confidence: 99%

“…Therefore, early diagnosis and treatment are likely to improve long‐term survival. TADs may be syndromic, associated with disorders involving other organs such as Marfan syndrome, or more commonly nonsyndromic, with manifestations restricted to the thoracic aorta 4, 5. Nonsyndromic TADs (NS‐TADs) may be familial, characterized by the presence of a family history and an autosomal dominant inheritance, or sporadic 4, 5, 6, 7.…”

Section: Introductionmentioning

confidence: 99%

“…TADs may be syndromic, associated with disorders involving other organs such as Marfan syndrome, or more commonly nonsyndromic, with manifestations restricted to the thoracic aorta 4, 5. Nonsyndromic TADs (NS‐TADs) may be familial, characterized by the presence of a family history and an autosomal dominant inheritance, or sporadic 4, 5, 6, 7. Unlike syndromic TADs, NS‐TADs are not evident from external physical features and abnormalities of other organ systems and are characterized by silent aneurysm formation and dissection 4, 5.…”

Section: Introductionmentioning

confidence: 99%

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Systematic Review of Studies That Have Evaluated Screening Tests in Relatives of Patients Affected by Nonsyndromic Thoracic Aortic Disease

Mariscalco

Debiec

Elefteriades

et al. 2018

JAHA

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BackgroundNonsyndromic thoracic aortic diseases (NS‐TADs) are often silent entities until they present as life‐threatening emergencies. Despite familial inheritance being common, screening is not the current standard of care in NS‐TADs. We sought to determine the incidence of aortic diseases, the predictive accuracy of available screening tests, and the effectiveness of screening programs in relatives of patients affected by NS‐TADs.Methods and ResultsA systematic literature search on PubMed/MEDLINE, Embase, and the Cochrane Library was conducted from inception to the end of December 2017. The search was supplemented with the Online Mendelian Inheritance in Man database. A total of 53 studies were included, and a total of 2696 NS‐TAD relatives were screened. Screening was genetic in 49% of studies, followed by imaging techniques in 11% and a combination of the 2 in 40%. Newly affected individuals were identified in 33%, 24%, and 15% of first‐, second‐, and third‐degree relatives, respectively. Familial NS‐TADs were primarily attributed to single‐gene mutations, expressed in an autosomal dominant pattern with incomplete penetrance. Specific gene mutations were observed in 25% of the screened families. Disease subtype and genetic mutations stratified patients with respect to age of presentation, aneurysmal location, and aortic diameter before dissection. Relatives of patients with sporadic NS‐TADs were also found to be affected. No studies evaluated the predictive accuracy of imaging or genetic screening tests, or the clinical or cost‐effectiveness of an NS‐TAD screening program.ConclusionsFirst‐ and second‐degree relatives of patients affected by both familial and sporadic NS‐TADs may benefit from personalized screening programs.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Systematic Review of Studies That Have Evaluated Screening Tests in Relatives of Patients Affected by Nonsyndromic Thoracic Aortic Disease

Mariscalco

Debiec

Elefteriades

et al. 2018

JAHA

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“…The utility of D‐dimer assessment in the diagnosis of acute aortic syndrome, including AAD, is well recognized 6, 7, 8, 9, 10. The European Society of Cardiology Guidelines recommends D‐dimer elevation as a IIa indication for a diagnostic workup of acute aortic syndrome,11 and previously reported studies have shown correlation between D‐dimer concentrations and the extent of aortic dissection 12, 13, 14. In addition, the D‐dimer concentration is reported to be lower in patients with a thrombosed false lumen (FL) or intramural hematoma than in patients with patent‐type AAD (ie, AAD with a patent FL) 12, 15.…”

Section: Introductionmentioning

confidence: 99%

Characteristics and Treatment Outcomes of Acute Type A Aortic Dissection With Elevated D‐Dimer Concentration

Itagaki

Kimura

Mieno

et al. 2018

JAHA

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BackgroundClinical characteristics and treatment outcomes of acute type A aortic dissection with D‐dimer elevation have not been clarified.Methods and ResultsD‐dimer was measured preoperatively within 24 hours of symptom onset in 262 patients with acute type A aortic dissection. The median (and interquartile range) admission D‐dimer concentration in our total patient group was 26.7 (8.3–85.9) μg/mL. Median (interquartile range) D‐dimer concentrations were 5.0 (2.6–18.0) μg/mL for complete false lumen thrombosis (n=33), 60.9 (19.4–160.4) μg/mL for partial thrombosis (n=81), 26.5 (10.0–70.6) μg/mL for a patent false lumen (n=131), and 8.7 (3.2–26.9) μg/mL for ulcerlike projection (n=17) (P<0.01). With a D‐dimer concentration of ≤8.3 μg/mL representing the lower quartile, we then investigated predictors of a low D‐dimer level. Multivariate analysis showed dissection limited to the ascending aorta (P<0.01; odds ratio, 9.81) or descending aorta (P<0.01; odds ratio, 7.68), a completely thrombosed false lumen (P<0.01; odds ratio, 4.02), and absence of brain ischemia (P=0.013; odds ratio, 4.74) to be predictors of the lower D‐dimer concentration. Compared with patients with a low D‐dimer concentration (≤8.3 μg/mL, n=66), patients with a D‐dimer concentration >8.3 μg/mL (n=196) had a reduced preoperative platelet count and increased operation time and transfusion volume. In‐hospital mortality was elevated in this group (1.5% versus 11.2%; P=0.031), although 7‐year survival did not differ for hospital survivors (lower versus higher, 93.1% versus 79.1%; P=0.21).ConclusionsD‐dimer concentrations are strongly influenced by the extent of dissection and false lumen status. Operative risks are increased in patients with a relatively high D‐dimer concentration.

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“…Acute type A aortic dissection is associated with a mortality rate of 50% within the first 48 h if not surgically treated,1 so early diagnosis and surgical treatment are important. If the type A dissection is circumferential, earlier repair is needed, because severe complications (e.g., massive aortic regurgitation [AR] and coronary ostial occlusion) can occur.…”

Section: Introductionmentioning

confidence: 99%

A case of circumferential type A aortic dissection with intimal intussusception diagnosed using repeat transthoracic echocardiography examination

Yamagishi

Kashiura

Nakata

et al. 2017

Acute Medicine & Surgery

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CaseSometimes it is difficult to diagnose circumferential aortic dissection with enhanced computed tomography alone. A 58‐year‐old woman presented with sudden‐onset chest discomfort and loss of consciousness. Transthoracic echocardiogram showed mild aortic regurgitation. Enhanced computed tomography scans showed no obvious intimal tear or flap at the proximal ascending aorta, but an intimal flap was observed from the aortic arch to both common iliac arteries. Stanford type B dissection was tentatively diagnosed. Repeat detailed transthoracic echocardiography examination showed an intimal tear and flap at the ascending aorta; prolapse into the left ventricle caused severe aortic regurgitation. Type A aortic dissection was definitively diagnosed; emergent operation showed a circumferential intimal tear originating from the ascending aorta.OutcomeThe ascending aorta was replaced; aortic regurgitation disappeared. The patient was discharged in a good condition 58 days postoperatively.ConclusionDynamic evaluations with transthoracic echocardiography should be carried out to diagnose circumferential aortic dissection.

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2014 ESC Guidelines on the diagnosis and treatment of aortic diseases

Cited by 3,669 publications

References 539 publications

Systematic Review of Studies That Have Evaluated Screening Tests in Relatives of Patients Affected by Nonsyndromic Thoracic Aortic Disease

Systematic Review of Studies That Have Evaluated Screening Tests in Relatives of Patients Affected by Nonsyndromic Thoracic Aortic Disease

Characteristics and Treatment Outcomes of Acute Type A Aortic Dissection With Elevated D‐Dimer Concentration

A case of circumferential type A aortic dissection with intimal intussusception diagnosed using repeat transthoracic echocardiography examination

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