Sie sind auf Seite 1von 38




Anatomie aorta ascendens

Diagram of the aortic root as seen at echocardiography. The aortic diameter should be measured at the aortic annulus (1), the sinuses of Valsalva (2), the supra-aortic ridge (3), and the proximal ascending aorta (4). In Marfan syndrome, dilatation usually starts at the sinuses of Valsalva, so this measurement is critical in monitoring the early evolution of the condition. Diameters must be related to normal values for age and body surface area.

Aorta dimensies-TEE
Cohen et al made various cardiac measurements using transoesophageal echocardiography in a group of 60 normal adults. These authors found the following aortic dimensions:

Aortic Annulus Tran-sinus Sino-tubular junction Ascending aorta Aortic arch Area

1.4 - 2.6 cms 2.1 - 3.5 cms 1.7 - 3.4 cms 2.1 - 3.4 cms 2.0 - 3.6 cms 2.4 - 3.5 cms^2

Aorta dimensies-Ctscan
Hager et al who have reported the diameter of the thoracic aorta at various sites as measured by helical computed tomography. In a group of 70 normal adults, these authors reported the following aortic dimensions: Tran-sinus Ascending aorta Distal Aortic arch Diaphragm 2.98 +/- 0.46 cms 3.09 +/- 0.41 cms 2.47 +/- 0.40 cms 2.43 +/- 0.35 cms

Ascending Aortic Aneurysm

ascending aortic aneurysm

aneurysm was associated with marked dilatation of the aortic annulus such that the annular diameter was 3.8 cms (normal range:1.4 - 2.6 cms). This dilatation was associated with severe aortic incompetence. Further 2D examination of the valve demonstrated completely normal leaflet morphology. Note also, the complete effacement of the sinotubular junction.

ascending aortic aneurysm

This patient was a young man. In such cases, Marfan's and EhlersDanlos' syndrome and the presence of a Bicuspid Aortic Valve (BAV) are recognised as specific risk factors for aneurysm formation. The genetic basis for Marfan syndrome is now well-accepted as being a mutation in the gene for fibrillin-1 and the Ehlers-Danlos syndrome is believed to result from mutations in the gene for type III procollagen (COL3A1). There is a strong relationship between the presence of a BAV and the development of ascending aortic aneurysm - even in the absence of aortic stenosis. This has been attributed to the presence of significant histological abnormalities in the aortic wall of patients with BAV (Matthias Bechtel et al).

Ziekte van marfan

* Variable autosomal dominant disorder, characteristically with cardiovascular, eye and skeletal, features. * The minimal birth incidence is 1 in 9800 * 27% of cases arise from new mutation * Mutation in fibrillin-1 on chromosome 15 is detected in 6691% of cases * Some cases may be due to mutation in TGFbetaRl or TGFbetaR2 * TGFbetaR1 or TGFbetaR2 are also associated with Loeys-Dietz syndrome, and TGFbetaR2 with familial thoracic aortic aneurysm * The clinical diagnosis in adults should be made using the Ghent criteria * The Ghent criteria are unreliable in children * Prophylactic medical treatment to protect the aorta with regular follow-up helps prevent or delay serious complications * Prophylactic aortic surgery should be considered when the aortic root at the Sinus of Valsalva exceeds 5 cm

Marfaan syndrome
Table 1. Ghent diagnostic nosology System Major criterion Involvement Skeletal At least 4 of the following features: 2 of the major features, or 1 major feature and 2 of the following: filled circle Pectus carinatum filled circle Pectus excavatum filled circle Pectus excavatum requiring surgery filled circle Joint hypermobility filled circle ULSR <0.86 or span:height >1.05 filled circle High palate with dental filled circle Wrist and thumb signs filled circle Crowding filled circle Scoliosis >20 or spondylolisthesis filled circle Characteristic face filled circle Reduced elbow extension (<170) filled circle Pes plenus filled circle Protrusio acetabulae Ocular Lens dislocation (ectopia lentis) Flat cornea Increased axial length of globe (causing myopia) Hypoplastic iris or ciliary muscle (causing decreased miosis) Cardiovascular Dilatation of the aortic root Mitral valve prolapse Dissection of the ascending aorta Dilatation of the pulmonary artery, below age 40 Calcified mitral annulus, below age 40 Other dilatation or dissection of the aorta Pulmonary None Spontaneous pneumothorax Apical blebs Skin/Integument None Striae atrophicae Recurrent or incisional hernia Dura Lumbosacral dural ectasia None Genetic findings Parent, child or sibling meets these criteria independently None Fibrillin 1 mutation known to cause Marfan syndrome Inheritance of DNA marker haplotype linked to Marfan syndrome in the family Abbreviations: ULSR, Upper:lower segment ratio. Having one of the features listed constitutes a major criterion or system involvement for all systems except the skeletal system, where more than one feature is needed. Next table | Figure and tables index

Aorta dissectie

Aortic Dissection (Ascending)

Type B dissectie

DEBAKEY: 1. Dissection involving the ascending and descending aorta and aortic arch (10 %). 2. Dissection involving only the ascending aorta and aortic arch (60%). 3. Dissection involving only the descending aorta only (30 % ). STANFORD: 'A'. Debakey types 1 and 2: i.e. involves the ascending aorta. 'B'. Debakey type 3: i.e. involves only the descending aorta. Although anatomically more specific than the Stanford classification, the Debakey system seems to be less-widely used and less clinically useful than the Stanford classification.

Behandeling Aorta dissectie

Approximately 95% of dissections involve either ascending or descending aorta whereas less than 5% involve the arch or abdominal aorta. Both management and long term outcome are different for type A and B dissections. Given the high risk of spontaneous fatal rupture in type A (~90%), surgery forms the mainstay of treatment for these cases, whereas surgery confers no advantage over medical management in uncomplicated type B dissections, with a reported 5 year survival of 75% irrespective of medical or surgical management.

flow is occurring only in the true lumen and that spontaneous echo contrast is apparent in the false lumen. The true lumen also expands markedly during systole.

In this longitudinal view through the distal aortic arch, the flap of a Stanford type 'A' dissection is demonstrated

distal descending thoracic aorta, occlusion of the false lumen of a Stanford type 'A' dissection by fresh thrombus is demonstrated.

Aortic atheroma
distal aortic arch, a pedunculated plaque

the mid and distal ascending aorta and proximal arch are blind spots for TOE due to the interposition of the trachea between the oesophagus and aorta. Although images are frequently seen of these regions, the image quality is often too poor for accurate diagnostic information and epiaortic ultrasonography is necessary for proper examination of this area.

Atheroma scoring
Various atheroma scoring systems have been suggested. A system recently used by Wilson et al is: 1. Normal 2. Intimal thickening (> 2 mm) 3. Atheroma < 4 mm +/- Ca++ 4. Atheroma >= 4 mm +/- Ca++, 5. Any size mobile or ulcerated lesion +/- Ca++. This is very similar to the grading system advocated by Montgomery et al which is also widely used: 1. normal 2. intimal thickening 3. atheroma < 5 mm 4. atheroma > or = 5 mm 5. mobile lesion

Spontaneous Echo Contrast (Aortic)

Spontaneous Echo Contrast (Aortic

Spontaneous Echo Contrast (SEC) is most commonly seen in the atria in those in atrial fibrillation. However, it can also be found in other parts of the circulation including the ventricles and the aorta. In the aorta it is usually found in those with a low cardiac output or aortic pathology such as aneurysm, dissection or marked atherosclerosis and it was first described by de Filippi et al. The presence of SEC is generally attributed to erythrocytic rouleaux formation in the setting of stagnation of blood flow or the presence of rheological factors such as the presence of an anti-cardiolipin antibody, an elevation of the erythrocyte sedimentation rate, an increase in the level of plasma fibrinogen, or a high blood viscosity. Leitman et al have recently demonstrated improved detection of SEC in the aorta using Tissue Doppler Imaging (TDI). In a group of 70 patients with significant cardiovascular pathology, they were able to demonstrate aortic SEC using conventional Doppler in 24 patients whereas with TDI it was apparent in 53. The important clinical correlates of aortic SEC which these authors found in their study are outlined in the table above. Note, in particular, the association with atrial fibrillation, peripheral embolism, stroke and transient ischaemic attack.

Descending Aortic Flow (Aortic Incompetence