What Is New in Dilatation of the Ascending Aorta?
2011; Lippincott Williams & Wilkins; Volume: 123; Issue: 8 Linguagem: Inglês
10.1161/circulationaha.110.949131
ISSN1524-4539
AutoresLuc Cozijnsen, Richard L. Braam, Reinier A. Waalewijn, Marc Schepens, Bart Loeys, Matthijs F.M. van Oosterhout, Daniela Q.C.M. Barge‐Schaapveld, Barbara J.M. Mulder,
Tópico(s)Coronary Artery Anomalies
ResumoHomeCirculationVol. 123, No. 8What Is New in Dilatation of the Ascending Aorta? Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessCase ReportPDF/EPUBWhat Is New in Dilatation of the Ascending Aorta?Review of Current Literature and Practical Advice for the Cardiologist Luc Cozijnsen, Richard L. Braam, Reinier A. Waalewijn, Marc A.A.M. Schepens, Bart L. Loeys, Matthijs F.M. van Oosterhout, Daniela Q.C.M. Barge-Schaapveld and Barbara J.M. Mulder Luc CozijnsenLuc Cozijnsen From the Department of Cardiology, Gelre Hospital, Apeldoorn, the Netherlands (L.C., R.L.B., R.A.W.); Department of Cardiothoracic Surgery, AZ Sint-Jan, Brugge, Belgium (M.A.A.M.S.); Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium (B.L.L.); Department of Pathology, St Antonius Hospital, Nieuwegein, the Netherlands (M.F.M.O.); and Departments of Clinical Genetics (D.Q.C.M.B.-S.) and Cardiology (B.J.M.M.), Academic Medical Centre, Amsterdam, the Netherlands. , Richard L. BraamRichard L. Braam From the Department of Cardiology, Gelre Hospital, Apeldoorn, the Netherlands (L.C., R.L.B., R.A.W.); Department of Cardiothoracic Surgery, AZ Sint-Jan, Brugge, Belgium (M.A.A.M.S.); Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium (B.L.L.); Department of Pathology, St Antonius Hospital, Nieuwegein, the Netherlands (M.F.M.O.); and Departments of Clinical Genetics (D.Q.C.M.B.-S.) and Cardiology (B.J.M.M.), Academic Medical Centre, Amsterdam, the Netherlands. , Reinier A. WaalewijnReinier A. Waalewijn From the Department of Cardiology, Gelre Hospital, Apeldoorn, the Netherlands (L.C., R.L.B., R.A.W.); Department of Cardiothoracic Surgery, AZ Sint-Jan, Brugge, Belgium (M.A.A.M.S.); Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium (B.L.L.); Department of Pathology, St Antonius Hospital, Nieuwegein, the Netherlands (M.F.M.O.); and Departments of Clinical Genetics (D.Q.C.M.B.-S.) and Cardiology (B.J.M.M.), Academic Medical Centre, Amsterdam, the Netherlands. , Marc A.A.M. SchepensMarc A.A.M. Schepens From the Department of Cardiology, Gelre Hospital, Apeldoorn, the Netherlands (L.C., R.L.B., R.A.W.); Department of Cardiothoracic Surgery, AZ Sint-Jan, Brugge, Belgium (M.A.A.M.S.); Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium (B.L.L.); Department of Pathology, St Antonius Hospital, Nieuwegein, the Netherlands (M.F.M.O.); and Departments of Clinical Genetics (D.Q.C.M.B.-S.) and Cardiology (B.J.M.M.), Academic Medical Centre, Amsterdam, the Netherlands. , Bart L. LoeysBart L. Loeys From the Department of Cardiology, Gelre Hospital, Apeldoorn, the Netherlands (L.C., R.L.B., R.A.W.); Department of Cardiothoracic Surgery, AZ Sint-Jan, Brugge, Belgium (M.A.A.M.S.); Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium (B.L.L.); Department of Pathology, St Antonius Hospital, Nieuwegein, the Netherlands (M.F.M.O.); and Departments of Clinical Genetics (D.Q.C.M.B.-S.) and Cardiology (B.J.M.M.), Academic Medical Centre, Amsterdam, the Netherlands. , Matthijs F.M. van OosterhoutMatthijs F.M. van Oosterhout From the Department of Cardiology, Gelre Hospital, Apeldoorn, the Netherlands (L.C., R.L.B., R.A.W.); Department of Cardiothoracic Surgery, AZ Sint-Jan, Brugge, Belgium (M.A.A.M.S.); Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium (B.L.L.); Department of Pathology, St Antonius Hospital, Nieuwegein, the Netherlands (M.F.M.O.); and Departments of Clinical Genetics (D.Q.C.M.B.-S.) and Cardiology (B.J.M.M.), Academic Medical Centre, Amsterdam, the Netherlands. , Daniela Q.C.M. Barge-SchaapveldDaniela Q.C.M. Barge-Schaapveld From the Department of Cardiology, Gelre Hospital, Apeldoorn, the Netherlands (L.C., R.L.B., R.A.W.); Department of Cardiothoracic Surgery, AZ Sint-Jan, Brugge, Belgium (M.A.A.M.S.); Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium (B.L.L.); Department of Pathology, St Antonius Hospital, Nieuwegein, the Netherlands (M.F.M.O.); and Departments of Clinical Genetics (D.Q.C.M.B.-S.) and Cardiology (B.J.M.M.), Academic Medical Centre, Amsterdam, the Netherlands. and Barbara J.M. MulderBarbara J.M. Mulder From the Department of Cardiology, Gelre Hospital, Apeldoorn, the Netherlands (L.C., R.L.B., R.A.W.); Department of Cardiothoracic Surgery, AZ Sint-Jan, Brugge, Belgium (M.A.A.M.S.); Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium (B.L.L.); Department of Pathology, St Antonius Hospital, Nieuwegein, the Netherlands (M.F.M.O.); and Departments of Clinical Genetics (D.Q.C.M.B.-S.) and Cardiology (B.J.M.M.), Academic Medical Centre, Amsterdam, the Netherlands. Originally published1 Mar 2011https://doi.org/10.1161/CIRCULATIONAHA.110.949131Circulation. 2011;123:924–928A 61-year-old woman presented with atypical chest complaints. Her sister and brother suffered from type A aortic dissection at 47 and 39 years of age, respectively.1 The diameter of the ascending aorta was 4.6 cm as measured with magnetic resonance imaging (Figure 1).Download figureDownload PowerPointFigure 1. Magnetic resonance angiography of the ascending aorta. The diameter at the level of the ascending aorta measures 4.6 cm. The diameter of the aortic root is normal.How Would You Manage This Patient?Acute dissection of the ascending aorta is often lethal. Even when emergency surgery can be performed, associated morbidity and mortality are high. To prevent dissection of the aorta, timely operation on a patient with a known dilatation of the ascending aorta (for normal values, see Table 1) is advised, along with other supportive measures. The guideline for the management of aortic dissection published by the European Society of Cardiology (ESC) task force in 20012 recommends the following for the prevention of aortic dissection in inherited diseases (Marfan syndrome [MFS], Ehlers-Danlos syndrome, annuloaortic ectasia): lifelong β-adrenergic blockade (Class IB), moderate restriction of physical activity (Class IC), periodic routine imaging of the aorta (Class IC), prophylactic replacement of the aortic root before diameter exceeds 5.5 cm (Class 2A), and prophylactic replacement of the aortic root before diameter exceeds 5.0 cm in patients with a family history of dissection (Class 2A). These ESC recommendations received endorsement by the American College of Cardiology (ACC) in 2001. In recent years, insights into the diagnosis and management of these patients have changed. The purpose of the present article is to summarize these changes, referring to this recent case presented at our clinic.Table 1. Normal Ascending Aortic Dimensions in AdultsDiameterValueStudyAortic annulus, cm Men2.6±0.3TTE Women2.3±0.2TTESinus of Valsalva, cm Men3.4±0.3TTE Women3.0±0.3TTEAortic root<3.7TTEProximal ascending aorta, cm Men2.9±0.3TTE Women2.6±0.3TTEAscending aorta, cm/m21.4–2.1 cm/m2TEE cm<3.8 (2.5–3.8)CT cm<3.7TTEReprinted with permission (modified) from Erbel et al2 for the ESC task force. Note that computed tomography or magnetic resonance measures the external diameter and is expected to be 0.2 to 0.4 cm larger than the TTE/transesophageal echocardiography (TEE) internal diameter.3 For younger patients, we refer to the nomograms of normal values with SDs related to body surface area in the same article.2Causes of Dilatation of the Ascending AortaIn patients with aortic dilatation, the aortic wall can be weakened by cystic media degeneration. The media displays loss of smooth muscle cells and fragmentation of elastic fibers with the appearance of cystic spaces filled with mucoid material.4 This normally occurs with aging. The normal growth rate of the aorta is about 0.07 to 0.2 cm/y5 but can be accelerated by hypertension. At younger ages, cystic media necrosis is associated with various connective tissue diseases such as MFS caused by a mutation in the gene for fibrillin-1, Ehlers-Danlos syndrome type IV with a deficiency in type III collagen, and familial/thoracic aortic aneurysm and dissection,4 in which genetic heterogeneity is observed. The Loeys-Dietz syndrome (LDS)6–8 is a recently recognized syndrome with a difficult phenotype characterized by the triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula or cleft palate. Two types can be distinguished. LDS type 1 has many overlapping features with MFS, whereas LDS type 2 has features that overlap with Ehlers-Danlos syndrome. Transforming growth factor-β receptor genes (TGFBR1 and TGFBR2) are the only genes known to be associated with LDS. However, no differences in phenotype are found between individuals with TGFBR1 or TGFBR2 mutations, and mutations in these 2 genes are also described in patients with MFS (diagnosed by the Ghent criteria) or with familial/thoracic aortic aneurysm and dissection. LDS is more aggressive than MFS or familial/thoracic aortic aneurysm and dissection with a propensity toward rupture and dissection of the ascending aorta at a younger age and at smaller aortic diameters.7–9 On the other hand, LDS patients appear to tolerate surgical intervention well, better than patients with Ehlers-Danlos syndrome IV, because they do not have friable vascular tissue.9 With respect to histology, the changes in the ascending aorta cannot be distinguished from the cystic media degeneration occurring during aging. However, in individuals with MFS, a marked excess of aortic wall collagen is observed compared with age-matched control subjects. In individuals with TGFBR2 mutation, for instance, this collagen excess is even more accentuated.6There is growing evidence that many patients with bicuspid aortic valve (BAV) or aortic coarctation have disorders of vascular connective tissue as well, involving loss of elastic tissue.10 The aortic expansion rate is higher in patients with BAV than in patients with tricuspid aortic valve: 0.19 versus 0.13 cm/y.11,12 This may result in dilatation of the aortic root or ascending aorta, even in the absence of hemodynamically significant aortic valve stenosis or regurgitation.11,13 Patients with aortic dilatation and BAV have a greater risk of rupture, dissection, or death than those without BAV.12 Aortic dilatation and aortic dissection may still occur in this circumstance, even after aortic valve replacement.13 This confirms a common pathogenetic mechanism for BAV and aortic dilatation that is independent of hemodynamic alterations.Besides connective tissue disease, hypertension is strongly correlated to aortic dilatation by elevating wall stress. Atherosclerosis, on the contrary, more often causes aneurysms of the descending aorta, particularly aneurysms of the abdominal aorta, not the ascending aorta.11,14 Aneurysm formation can also be caused by chronic dissection, trauma, aortic surgery (false aneurysm), and cardiopulmonary resuscitation. Lastly, aortic dilatation can be caused by inflammatory diseases as bacterial or fungal aortitis, Takayasu arteritis, and giant cell arteritis.When Can Aortic Dissection Occur?Aortic dilatation may lead to aortic dissection or aortic rupture. The chance of aortic dissection is related to the aortic diameter. In 2002, Davies et al15 identified that the median aortic diameter at the time of rupture for the ascending or aortic arch was 6.0 cm. There is, however, growing evidence that a substantial number of patients have aortic diameters <5.5 cm at the time of dissection. Fifteen percent of MFS patients have aortic dissection at a size <5.0 cm.16 In the work by Svensson et al,16 12.5% (5 of 40) of BAV patients had also diameters <5 cm at the time of operation for aortic dissection. In the analysis by Parish et al, 17 42% (74 of 177) of the non-MFS tricuspid aortic valve patients had a diameter <5 cm at moment of type A dissection. In the International Registry of Acute Aortic Dissections, nearly 60% of the 591 type A dissection patients had diameters <5.5 cm, and 40% had diameters 5.0 cm. These ESC guidelines received ACC endorsement in 2001.Between 2006 and 2008, 3 guidelines were published, not focused on aortic dissection/dilatation but with additional advice for patients with dilated ascending aorta in combination with BAV or aortic regurgitation. The 2006 ACC/American Heart Association guidelines for the management of valvular heart disease,22 the 2007 ESC guidelines on the management of valvular heart disease,23 and the 2008 ACC/AHA guidelines for managing adults with congenital heart disease24 all give practically the same recommendations: (1) Repair of the aortic root or replacement of the ascending aorta is indicated if the diameter of the aortic root or the ascending aorta exceeds 5.0 cm or if the rate of increase is ≥0.5 cm/y; (2) in patients undergoing aortic valve replacement, repair of the aortic root or replacement of the ascending aorta is indicated if the diameter is >4.5 cm; (3) lower thresholds may be considered for patients of small stature22; and (4) prepregnancy counseling is advised at 4.0/4.5 cm.Very recently, the 2010 ACC Foundation/AHA guidelines for the management of thoracic aortic disease were published.3 They recommend similar thresholds for surgery. What is new is the statement that for patients with LDS or confirmed TGFBR1 or TGFBR2 mutation, it is reasonable to undergo aortic repair when the aorta diameter exceeds 4.5 cm.Given the recent data on type A dissection at diameters 4.0 cm: Advice for the Clinician Caring for Patients With Aortic DilatationDiameter >4.0 cmSearch for connective tissue disorder, initiate β-blocker therapy, maintain strict blood pressure control, moderately restrict physical activity, provide prepregnancy counseling, perform yearly follow-up by TTE and/or computed tomography/magnetic resonance imagingDiameter >4.5 cm and aortic valve surgeryProvide operative treatmentDiameter >4.5 cm in cases of connective tissue disorderConsider operative treatment in cases of desired pregnancy, family history of aortic dissection, LDS, or TGFBR1/TGFBR2 mutation or progressive aortic growth >0.5 cm/yDiameter >5.0 cm in cases of connective tissue disorderProvide operative treatmentDiameter >5.5 cm in other casesProvide operative treatmentManagement and Follow-Up in Patients With Aortic DilatationIn case of dilatation of the ascending aorta >4.0 cm, evaluation of a possible connective tissue disease should be performed by a multidisciplinary team (cardiologist, geneticist, and ophthalmologist). Patient and family history should be investigated; physical examination should be undertaken; and eventually DNA testing should be carried out. Transthoracic echocardiography (TTE) should be performed to examine the aortic valve and to quantify aortic regurgitation. The diameter of the ascending aorta should be measured at 4 levels: at the level of the annulus, the aortic sinuses, the sinotubular junction, and the proximal ascending aorta. In addition, magnetic resonance imaging or computed tomography should be performed to visualize the entire aorta.26,27 Especially in BAV patients, aneurysms tend to occur more frequently in the proximal ascending aorta, which may be difficult to visualize by TTE. Risk factors such as hypertension, cigarette smoking, and hypercholesterolemia should be managed. Blood pressure should be lowered to ≤120/80 mm Hg. Currently, β-blockers are still the preferred medical therapy,5,11,28 but large prospective multicenter trials of the effects of angiotensin II receptor antagonists or angiotensin-converting enzyme inhibitors on aortic growth in patients with MFS are being performed worldwide.5,11 Moderate restriction of physical activity should be advised.2,5 Patients should avoid exertion at maximal capacity and specifically should not engage in competitive, contact, or isometric sports.29,30 Yearly follow-up of the aortic diameter by TTE and/or magnetic resonance imaging or computed tomography should be performed. In case of LDS, magnetic resonance imaging of the thoracic and cervical vessels should be performed more frequently. Patients with LDS often have a more widespread and malignant course of aneurysmal disease.Operative TreatmentThe aortic valve and ascending aorta are replaced simultaneously by use of a so-called composite valve graft with reimplantation of the coronary ostia into the prosthesis. The most frequent cause of late death is aneurysmal formation at the downstream aorta. Valve-sparing aortic root replacements are becoming more popular in view of the potential thromboembolic, infectious, and bleeding complications after composite graft replacement. In the remodeling technique described by Sarsam and Yacoub,31 the graft is sewn to the remaining aortic wall around the commissures. This creates neosinuses and leaves the annulus mobile but unsupported. In the reimplantation technique,32 the graft is fixed at the subannular level, and the valve and commissures are reimplanted inside the fabric. Both procedures offer a reasonable alternative to composite valve grafting with excellent short- and medium-term results, but long-term durability is not yet established.Genetic CounselingMost connective tissue disorders, including LDS, are inherited in an autosomal-dominant manner, which means that first-degree relatives are at a 50% risk of inheriting the disease, although the severity cannot be predicted. First-degree relatives should therefore be evaluated for manifestations of connective tissue disorder, including a comprehensive clinical examination and TTE. If a disease-causing mutation in the index patient is known, molecular genetic testing of family members is possible, and prenatal testing can be performed.ConclusionsThe clinician should be aware of the need for an aggressive preventive approach in patients with aortic dilatation, specifically in cases of a connective tissue disorder. As a result of recent insights, criteria for operative treatment of aortic dilatation have been liberalized in the relevant guidelines. Furthermore, the clinician should realize that first-degree relatives of patients with thoracic aortic dilatation are also at risk and should be evaluated for manifestations of connective tissue disorders.Follow-Up of Our PatientFor our patient, we decided to operate at 4.6 cm because of the clear family history of aortic dissection, even though ESC guidelines recommend waiting until 5.0 cm.2 We replaced the ascending aorta prophylactically. A supracoronary replacement of the ascending aorta and proximal aortic arch was performed. A free-floating extension of the arch prosthesis, a so-called elephant trunk, was left behind in the proximal descending aorta for an eventually needed operation of the descending aorta. Histopathological examination revealed extensive media degeneration with fragmentation of elastic fibers (Figure 2) without signs of aortitis. Physical examination at clinical genetic evaluation showed a bifid uvula (Figure 3) but no other outward clinical signs of LDS. A possible pathogenic mutation (c.1043G>A, p.Cys348Tyr) was found in the TGFBR1 gene. This missense mutation has not been reported previously but is likely pathogenic because it occurs in a strongly conserved amino acid sequence. Family screening and functional studies are underway to confirm the pathogenicity of the mutation.Download figureDownload PowerPointFigure 2. Histological section of the resected ascending aorta. Note the extensive fragmentation and loss of elastic fibers. There were no signs of aortitis. A indicates adventitia; M, media; I, intima (elastic van Gieson stain, ×50).Download figureDownload PowerPointFigure 3. Photograph of the mouth of our patient revealing a split uvula.DisclosuresNone.FootnotesCorrespondence to Luc Cozijnsen, MD, Gelre Hospital, Albert Schweitzerlaan 31, 7334 DZ Apeldoorn, Netherlands. E-mail l.cozijnsen@gelre.nlReferences1. Stroes ESC, Cozijnsen L, Jaarsma W, Hamerlynck RPHA, Beemer FA. Familiale acute aorta dissectie. Cardiologie. 1994; 1:68–71Google Scholar2. Erbel R, Alfonso F, Boileau C, Dirsch O, Eber B, Haverich A, Rakowski H, Struyven J, Radegran K, Sechtem U, Taylor J, Zollikofer C, Klein WW, Mulder B, Providencia LA. Diagnosis and management of aortic dissection: Task Force on Aortic Dissection, European Society of Cardiology. Eur Heart J. 2001; 22:1642–1681CrossrefMedlineGoogle Scholar3. 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