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ACUTE AORTIC SYNDROMES. RAJESH K F. Acute aortic syndromes consist of 3 interrelated conditions with similar clinical characteristics Aortic dissection Intramural hematoma Penetrating aortic ulcer.
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ACUTE AORTIC SYNDROMES RAJESH K F
Acute aortic syndromes consist of 3 interrelated conditions with similar clinical characteristics • Aortic dissection • Intramural hematoma • Penetrating aortic ulcer
Erbel R, Alfonso F, Boileau C, Dirsch O, Eber B, Haverich A, et al. Diagnosis and management of aortic dissection. Eur Heart J 2001;22:1642-81.
AORTIC DISSECTION • Most common aortic catastrophe • Incidence - 5 to 30 per 1 million people/year • Primary tear in aortic intima with bleed into diseased media • Rupture of vasavasorum - Hemorrhage in aortic wall with subsequent intimal disruption
Most ascending aortic dissections begin within a few centimeters of aortic valve • Most descending aortic dissections have their origin just distal to left subclavianartery
DeBakey classification • I: Ascending aorta -> arch +/- descending aorta • II: Ascending aorta only • III:Descending aorta • IIIa: Limited to descending thoracic aorta • IIIb: Extending below diaphragm
Stanford classification Type A • Affect ascending aorta, regardless of site of origin Type B • Do not affect ascending aorta
Classification • Based on time of onset of initial symptoms to time of presentation • Acute dissection < 2 weeks • Subacute- between 2 and 6 weeks • Chronic > 6 weeks Behave like aneurysm Rupture is the risk Malperfusionis rare
RISK FACTORS Hypertension (75%) Genetically triggered • Marfansyndrome • Bicuspid aortic valve (5 to 10 times risk) • Loeys-Dietz syndrome • Hereditary thoracic AA or dissection • Vascular Ehlers-Danlossyndrome Congenital diseases or syndromes • Coarctation of the aorta • Turner syndrome(dissection at small aortic dimensions) • Tetralogy of Fallot Atherosclerosis • Penetrating atherosclerotic ulcer Trauma, blunt or iatrogenic • Catheter or stent • Intra-aortic balloon pump • Aortic or vascular surgery • Motor vehicle accident • CABG or AVR Cocaine use Inflammatory or infectious disease • Giant cell arteritis • Takayasu arteritis • Behcetdisease • Aortitis • Syphilis Pregnancy (typically in third trimester)
Patients <40years are less likely to have HTN(34%) and more likely to have Marfan’s syndrome, bicuspid aortic valve, or prior aortic surgery IRAD registery
Marfan syndrome -Ghent criteria CARDIOVASCULAR SYSTEM MAJOR CRITERIA •Dilation of ascending aorta •Aortic dissection MINOR CRITERIA •MVP •Dilation of MPA •Premature mitral annular calcification (<40yrs) •Descending thoracic or Abdominal aortic aneurysm(< 50 yrs) CVS involvement:one minor criterion OCULAR SYSTEM-MAJOR CRITERION • Ectopialentis SKELETAL SYSTEM MAJOR CRITERIA Presence of at least four of the following • Pectuscarinatum • Pectusexcavatum requiring surgery • Reduced US to LS ratio (.85to 0.95) or arm span to height ratio > 1.05 • Wrist and thumb signs •Scoliosis > 20° or spondylolisthesis •Reduced extension at elbows (<170°) • Medial displacement of medial malleolus causing pesplanus • Protusioacetabulae of any degree
Marfan syndrome -Ghent criteria • INDEX CASE: Major criteria in two systems and involvement of a third system • FAMILY MEMBER: One major criterion in an organ system and involvement of a second organ system
Revised Ghent criteria J Med Genet 2010 47: 476-485
Loeys-Dietz aneurysm syndrome • Autosomal dominant • Mutations in TGFBR1 or TGFBR2 • Arterial tortuosity, hypertelorism, bifid or broad uvula,cleft palate • Soft, velvety skin and easily visible veins • Aortic dissection and aneurysm involving branch vessels
Familial thoracic aortic aneurysm and dissection • Autosomal dominant disorder • Mutations in various genes including ACTA 2, MYH11, TGFBR1 and TGFBR2, FBN1 • Thoracic aortic aneurysm and dissection • May be associated with PDA, cerebral aneurysm, BAV or livedoreticularis
VascularEhlers-Danlos syndrome (type 4) • Autosomal dominant • Mutation in gene COL3A1 • Abnormal type III procollagen synthesis • Hyperflexible fingers, hyperlucent skin with visible veins, easy bruisability and varicose veins • Hisk for spontaneous arterial dissection and rupture, often in medium sized arteries
Aortic dissection clinical presentation • Usual age is sixth or seventh decades of life • Chestpain or backpain or both • Most severe at its onset • Migratory Pain • Ripping, tearing, stabbing, or sharp quality • Patients on steroids and Marfan syndrome prone for painless presentation (6.4%)
High-risk examination features • Loss of peripheral pulse • SBP limb differential greater than 20 mm Hg • Focal neurologic deficit • New AR murmur International Registry of Acute Aortic Dissection (IRAD) Physical Findings of 591 Patients With Type A Aortic Dissection
Most type B dissection are hypertensive on presentation • Type A dissection may present with normal BP or hypotension • Loss of peripheral pulse • are reported in 10% to 30% of acute dissections • May be intermittent -Dynamic movement of dissection flap Dynamic obstruction
Aortic regurgitation • In 40% with acute type A dissection • Mechnisms of AR • Malcoaptation of aortic leaflets - dilation of aortic root and annulus • Distortion of alignment • Aortic leaflet prolapse • Prolapse of intimal flap across aortic valve
Neurologic manifestations • Most common in dissection type A • May dominate clinical presentation • Neurologic syndromes include • Persistent or transient ischemic stroke • Spinal cord ischemia • Ischemic neuropathy • Hypoxic encephalopathy
Syncope • Reported in 13% of patients in IRAD • Indicate development of dangerous complications • Acute hypotension - Cardiac tamponade (10% of acute type A dissections) or aortic rupture • Cerebral vessel obstruction or activation of cerebral baroreceptors
Vascular insufficiency • Renal artery - 5% to 10% • Renal ischemia,infarction, renal insufficiency or refractory hypertension • Mesenteric ischemia or infarction in 5% • Extension to iliac arteries -acute limb ischemia
Acute myocardial infarction • Flap causing malperfusionof coronary artery • Occurs in 1% to 7% of acute type A dissections • RCA is most commonly involved
Left-sided pleural effusion • Usually related to inflammatory response • Acute hemothorax
Chest radiography • Widening mediastinum (80% to 90% of cases (83%, type A; 72%, type B) • Obliteration of aortic knob • Displaced intimal calcification (>5 mm) -calcium sign 20% • Displacement of trachea to right • Distortion of left main-stem bronchus • Pleural effusion (more common left sided) • Cardiomegaly • Normal in 12% to 15% of cases
D-dimer levels • Rise in acute aortic dissection as in pulmonary embolism • Level >1,600 ng/mL within first 6 hours - positive likelihood ratio of 12.8 for dissection • In first 24 hours after symptom onset - D-dimer level < 500 ng/Ml has negative predictive value of 95%
IMAGING 1 Establish presence of AD or variant (IMH,PAU) 2 Location of the dissection (Type A, Type B) 3 Anatomical features a Extent of dissection b Sites of entry and reentry c False lumen patency, partial thrombosis, thrombosis 4 Complications of dissection a Type A i Aortic regurgitation ii Coronary artery involvement iii Pericardial effusion/hemopericardium b Aortic rupture or leaking c Branch vessel involvement d Malperfusion
Contrast-enhanced CT • Most commonly used • Sensitivity and specificity of 95% to 98% • ECG gating or multi detector scanning eliminate pulsation artifacts • Intravenous contrast is necessary to visualize true and false channels • Visualize hemopericardium, aortic rupture, and branch vessel involvement
MRI • Sensitivity of 98% and specificity of 98% with diagnostic odds ratio of 6.8 • Capable of multiplanar imaging with 3D reconstruction • Cine MRI visualize blood flow, differentiating slow flow and clot and AR • MRA -detect and quantify AR & branch vessel morphology
TTE • 78.3% sensitivity and 83.0% specificity for diagnosing proximal dissection • 2 lumens separated by flap
TEE • Accurately visualise entire thoracic aorta (sensitivity 98.0%, specificity 95.0%, diagnostic odds ratio 6.1) • 2 lumens separated by flap • Visualize coronary ostia • AR • Pericardial effusion • LV & RV function • May not adequately visualize distal ascending aorta and aortic arch
Aortography • Identify intimal flap, true and false lumen • Thickened wall (thrombosed false lumen) • AR, branch vessel involvement • Diagnostic accuracy 90-95% • 5-10% false negative rate – thrombosed false lumen – simultaneous opacification of both lumens – misses IMH • Risks of procedure
Comparative study with nonhelical CT, 0.5 Tesla MR and TEE showed • 100% sensitivity for all modalities, • Better specificity of CT (100%) than for TEE and MR • False-negative studies can and do occur Shiga T, Wajima Z, Apfel CC, Inoue T, Ohe Y. Diagnostic accuracy of transesohageal echocardiography, helical computed tomography,andmagnetic resonance imaging for suspected thoracic aortic dissection. Arch Intern Med. 2006;166:1350-6.
INITIAL MANAGEMENT • IV beta blockade -Target HR of 60 /min or less(LOE : C) • Esmolol -Initial bolus of 500microg/kg and continuous infusion of 50 to 200 microg/kg/min • Labetolol-Initial dose of 20 mg IV over 2 minutes and 40 to 80 mg IV every 15 minutes (max 300 mg), continuous infusion 2 to 8 mg/min • Propranolol and metoprolol–IV or oral • BBs blockers- compensatory tachycardia in acute AR
CIs to BB-Nondihydropyridine CCB(LOE : C) • IV diltiazem0.25 mg/kg over 2 minutes > infusion 5 to 15 mg/hr • SBP > 120 mm Hg after adequate HR control- ACEI and/or other vasodilators IV (LOE : C) • Should not be initiated prior to rate control - reflex tachycardia increase aortic wall stress (LOE : C)
IV sodium nitroprusside - most established agent, rapidly titratable , 20 microg/min, with titration to 0.5 to 5 microg/kg/min • Renal insufficiency or prolonged use-cyanide toxicity • IV enalaprilat,nitroglycerin,nicardipine,nitroglycerin, fenoldopam • Refractory hypertension - consider renal artery hypertension due to dissection causing renal malperfusion • Appropriate analgesia – opiate
Surgical Therapy Acute type A aortic dissection Retrograde dissection into ascending aorta Surgical Therapy and/or Endovascular Therapy Acute type B aortic dissection complicated by • Visceral ischemia • Limb ischemia • Rupture or impending rupture • Aneurysmal dilation • Refractory pain Medical Therapy Uncomplicated type B aortic dissection Uncomplicated isolated arch dissection
GOALS OF SURGERY • Excise intimal tear • Obliterate false channel by oversewingaortic edges • Reconstitute aorta,usuallyby placing a dacron interposition graft
Type A aortic dissection • Replace affected ascending aorta with or without aortic arch with prosthetic graft • In-hospital mortality 15-35% • Proximal extension of dissection to aortic valve or ostia of coronary arteries may require replacement or resuspension of aortic valve (24% )or coronary artery bypass (15% ) Valve sparing -David or Yacoub Procedures
• AVR required if annular supports of leaflets damaged (composite graft or homograft) • AVR required if aortic root >5 cm Modified Bentall’s operation
Preoperative Prediction Model of Surgical Mortality Risk From Rampoldi V, Trimarchi S, Eagle KA, et al: Simple risk models to predict surgical mortality in acute type A aortic dissection: The International Registry of Acute Aortic Dissection score. Ann ThoracSurg 83:55, 2007.
Uncomplicated acute type B aortic dissection • Drug treatment alone can result in 78% three year survival • Medical management remains the gold standard • Endovascular treatment is increasingly possible with low mortality [IRAD]: New insights into an old disease. JAMA 283:897, 2000.)
ADSORB trial • Acute Dissection: Stent graft OR Best medical therapy • Acute uncomplicated type B aortic dissection • Stent graft OR Best medical therapy • 250 subjects ,125 test / 125 control • Follow-up – 3 years <55mm