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    Acute aortic syndrome

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    Aug.11.2023

    Acute Aortic Syndrome

    Synopsis

    Key Points

    • Acute aortic syndrome comprises several life-threatening aortic conditions that present similarly but have differing pathologic, demographic, and survival characteristics; it includes aortic dissection, intramural hemorrhage, and penetrating atherosclerotic ulcer
    • Male individuals older than 40 years with hypertension and individuals younger than 40 years with Marfan syndrome or bicuspid aortic valve are at highest risk
    • Characteristic symptoms include very abrupt onset of chest pain, usually described as sharp, tearing, or ripping. Pain may radiate to neck, back, or abdomen, depending on location of aortic damage
    • CT angiography is usually the diagnostic test of choice, although transesophageal echocardiography or MRI can be used depending on patient characteristics and facility capabilities
    • Initial management includes immediate surgical consultation and urgent reduction of heart rate to 60 to 80 beats per minute or less and reduction of blood pressure to 120 mm Hg or less (systolic) with IV β-blockers
    • Type A dissection (and intramural hemorrhage and penetrating atherosclerotic ulcer of ascending aorta) is typically managed surgically
    • Type B dissection (and intramural hemorrhage and penetrating atherosclerotic ulcer of descending aorta) can be managed conservatively (treatment of hypertension) or surgically, depending on clinical findings
    • Patients with acute aortic syndrome need lifelong antihypertensive therapy and surveillance aortic imaging

    Urgent Action

    • Obtain emergent surgical consultation for type A dissection; expedite transfer to higher-level facility if indicated r1
    • Decrease stress on aortic wall by decreasing heart rate and blood pressure
      • Begin IV β-blocker to decrease heart rate to 60 beats per minute or less; add nondihydropyridine calcium channel blocker if needed to reach this goal
      • After heart rate is controlled, begin vasodilators to decrease systolic blood pressure to 120 mm Hg or less; nitroprusside is commonly used
      • Administer IV narcotic analgesic for pain

    Pitfalls

    • Always consider aortic dissection as a cause of chest pain, particularly for pain that radiates to upper back or abdomen or that is described as tearing or ripping
    • Missed or delayed diagnosis is usually due to an incorrect working diagnosis of acute coronary syndrome r2
      • Patients with an intermediate-risk profile for acute aortic syndrome who are being evaluated for a possible acute coronary syndrome (but without clear-cut ST-elevation myocardial infarction) should have aortic imaging before being given antiplatelet and antithrombin agents r3
      • Myocardial infarction can coexist with aortic dissection
    • CT, MRI, and transesophageal echocardiography are all accurate diagnostic modalities, but false-negative results do occur
      • Diagnosis cannot be excluded definitively on the basis of results from a single imaging study. If first aortic imaging findings are negative, strongly consider obtaining a second imaging study if there is high clinical suspicion

    Terminology

    Clinical Clarification

    • Acute aortic syndrome comprises several life-threatening aortic conditions that present similarly but have differing pathologic, demographic, and survival characteristics r4r5
    • Includes aortic dissection, intramural hematoma, and penetrating atherosclerotic ulcer r4r6
      • Aortic dissection: disruption of medial layer provoked by intramural bleeding, usually from an intimal tear. This results in separation of aortic wall layers and subsequent formation of true lumen and false lumen with or without communication r7
        • Most common type of acute aortic syndrome r4
      • Aortic intramural hematoma: hematoma develops in media of aortic wall in absence of false lumen and intimal tearr7(definition is controversial; intramural hematoma may be aortic dissection with thrombosis of false lumen)r4
        • Accounts for up to 25% of cases of acute aortic syndrome
      • Penetrating atherosclerotic ulcer: ulceration of an aortic atherosclerotic plaque penetrating through internal elastic lamina into media;r7localized to descending thoracic aorta in more than 90% of casesr3
        • Accounts for 2% to 7% of cases of acute aortic syndrome r7

    Classification

    • Aortic dissection: classification based on anatomy
      • Stanford classification r5
        • Type A r8
          • Dissection originates in root of aorta or upper ascending aorta; may extend into abdominal aorta
          • Accounts for approximately two-thirds of acute dissections
          • Virtually all require surgical correction, and in-hospital mortality ranges from 22% to 47%
        • Type B r8
          • Dissection is confined to descending aorta
          • Accounts for approximately one-third of acute dissections
          • Most are uncomplicated type B dissections, which can be managed medically with very low in-hospital mortality; almost all progress to chronic phase
          • Complicated type B dissections require urgent endovascular or surgical repair for aortic rupture, rapid expansion, or branch vessel malperfusion
      • DeBakey classification r4
        • Type I
          • Dissection originates in ascending aorta and propagates distally at least to aortic arch and typically to descending aorta
          • 90% of type A dissections r8
        • Type II
          • Dissection originates in and is confined to ascending aorta
          • 10% of type A dissections r8
        • Type III
          • Dissection originates in descending aorta and propagates distally

    Diagnosis

    Clinical Presentation

    History

    • Earlier medical history is significant; known aortic aneurysm, congenital heart disease, connective tissue disease, hypertension, and previous surgical or catheter-based cardiac procedure increase likelihood of acute aortic syndrome c1c2c3c4c5
    • Seek history of stimulant drug use (eg, cocaine, methylenedioxymethamphetamine, methamphetamine) c6
    • Symptom onset is sudden and rapid
    • Clinical symptoms do not allow distinction between acute aortic syndrome types and other acute pathologic conditions
    • For all types of acute aortic syndrome, broad range of nonspecific symptoms is possible, depending on location of dissection, degree of extension, presence of vascular branch (and end-organ) involvement, and presence of rupture
      • Aortic dissection
        • Symptomatic in 80% to 90% of cases r4
        • Sudden-onset severe chest pain is first symptom c7
          • Most patients describe pain as sharp and knife-like; others describe it as tearing or ripping r3r9c8
          • Abruptness of onset is a specific characteristic (84%) r9
        • Location of pain may reflect site of origin of intimal tear
          • Typically anterior chest pain in type A (80% of cases), but 47% of patients also report back pain r3r10c9c10
          • Begins as back pain in type B (64% of cases), but presentation with chest and/or abdominal pain also occurs r3c11c12c13
          • Pain may be migratory as dissection extends c14
        • May present with syncope (15% with type A; less than 5% with type B) r7c15
        • Other symptoms reflect ischemic end-organ dysfunction caused by extension of dissection to branch arteries
          • Diffuse abdominal pain is present in about 21% of type A cases and about 43% of type B cases r3c16
          • Flank pain and decreased urine output are likely if renal artery is involved in dissection c17c18
          • Dyspnea and orthopnea are likely if ischemic congestive heart failure is present or if left ventricular failure results from aortic rupture into left atrium c19c20
          • Symptoms of stroke are likely if carotid artery is involved
          • Lower extremity numbness and pain are likely if dissection extends to iliac arteries (with paraplegia if spinal arteries are not perfused) c21c22c23
          • Rarely, hemoptysis is caused by rupture into tracheobronchial tree c24
          • Rarely, hematemesis is caused by rupture into esophagus c25
      • Intramural hematoma
        • Patient is more likely to be older and more likely to be female than a patient with aortic dissection r4
        • Presents with pain similar to that of aortic dissection r4c26c27
        • Less likely to present with neurologic symptoms
      • Penetrating atherosclerotic ulcer r11c28
        • Patient is more likely to be an older male individual and more likely to have known coronary artery disease or risk factors for atherosclerotic disease than a patient with aortic dissection r7c29c30
        • Presents with pain similar to that of aortic dissection r3r4c31c32
        • Rarely presents with symptoms of dissection-related lack of organ perfusion

    Physical examination

    • May appear anxious and diaphoretic c33c34
    • Blood pressure may be high, normal, or low r12
      • Measure blood pressure in both arms; higher reading will be used to determine blood pressure management plan r3
      • Type A dissection
        • Hypotension in about 25% r9c35
        • Hypertension in about 35% r9c36
      • Type B dissection
        • Hypertension in about 70% r9c37
    • Cardiopulmonary examination
      • Pulse deficit (apical versus radial) is present in about 18% of type A cases and about 9% of type B cases r9c38
      • Pressure differential (interarm blood pressure difference) greater than 20 mm Hg is suggestive (although studies differ on its significance) r10c39
        • Compare carotid and femoral arteries
      • Aortic insufficiency murmur (early diastolic decrescendo murmur heard best in the left third intercostal space) is present in about 44% of type A cases and about 12% of type B cases r9c40
      • Signs of congestive heart failure (eg, rales, S₃ gallop) are present in about 9% of type A cases and about 3% of type B cases r9c41c42
      • Cool, pale lower extremities with decreased or absent pulses are likely if iliac and femoral arteries are involved c43c44c45c46
    • Neurologic examination
      • Signs of stroke are present in about 6% of type A cases and about 2% of type B cases r9
      • Hemiparesis is likely if spinal artery malperfusion is present c47
      • Hoarseness (from laryngeal nerve compression) may occur c48

    Causes and Risk Factors

    Causes

    • Often multifactorial; most common factors include uncontrolled hypertension and inherited susceptibility r5
      • Hypertension c49
        • Elevated systolic pressure can propagate an aortic dissection c50
        • Physical exertion or emotional stress is direct precursor of pain in two-thirds of aortic dissection events; both involve acute changes in blood pressure r12c51c52
      • Genetic syndromes c53
        • Congenital connective tissue diseases (associated with abnormalities of aortic media) c54c55
          • Marfan syndrome r13c56
            • Autosomal dominant genetic disorder characterized by weakness of connective tissues (which endangers heart and blood vessels)
            • Aortic dissection occurs in 15% to 50% of patients with Marfan syndrome who are younger than 40 years r12
          • Ehlers-Danlos syndrome type IV r14c57
            • Autosomal dominant genetic disorder characterized by skin hyperextensibility, joint hypermobility, and tissue fragility (which endangers heart and blood vessels) c58
            • Arterial complications are the leading cause of death
          • Loeys-Dietz syndrome c59
            • Autosomal dominant genetic disorder characterized by arterial tortuosity, wide-set eyes, cleft palate, and aortic dissection and aneurysm
        • Other familial thoracic aneurysm and dissection syndromes c60
          • Familial Marfan-like habitus c61
          • Familial ectopia lentis c62
          • Familial thoracic aortic aneurysm and dissection c63
          • Erdheim cystic medial necrosis c64
          • Congenital contractural arachnodactyly c65
        • Turner syndrome c66
          • Phenotypic female with 45,X karyotype
          • May have associated bicuspid aortic valve and/or aortic coarctation

    Risk factors and/or associations r15

    Age
    • Incidence rises with age; mean age at presentation is 63 years r9c67
    • Age at presentation is often lower in patients with Marfan syndrome, bicuspid aortic valve, or history of previous aortic surgery r3c68
    • Case reports of aortic dissection in children as young as 3 years r3
    Sex
    • More than two-thirds of aortic dissection events occur in male individuals r12c69c70
    • Female individuals have higher mortality rates from aortic dissection than male individuals r16c71c72
    Genetics
    • Marfan syndrome: heterozygous mutation in the FBN1 gene (fibrillin 1; OMIM *134797)r17 on chromosome band 15q21 (phenotype is OMIM #154700)r18c73
    • Ehlers-Danlos syndrome type IV: heterozygous mutation in the COL3A1 gene (collagen type III alpha 1 chain; OMIM *120180)r19 on chromosome band 2q32 (phenotype is OMIM #130050)r20c74
    • Loeys-Dietz syndrome type 2: heterozygous mutation in the TGFBR2 gene (transforming growth factor beta receptor 2; OMIM *190182)r21 on chromosome band 3p22 (phenotype is OMIM #610168)r22c75
    Other risk factors/associations
    • Congenital cardiovascular malformations c76c77
      • Bicuspid aortic valve c78c79
        • Affects 1% to 2% of general population r7
        • Confers increased risk of aortic dissection; has been described in the range of 5- to 10-fold increased risk, although the true multiple is uncertain r23r24
      • Aortic coarctation c80c81
        • Significantly increases risk of aortic dissection, particularly when in association with bicuspid aortic valve (odds ratio, 4.7) r12
    • Preexisting thoracic aortic aneurysm r3c82
      • Aortic dissection is frequently superimposed on thoracic aortic aneurysms
    • Previous cardiac interventions c83
      • Aortic dissection is a rare but dangerous complication of cardiac surgery and cardiac catheterization r25
      • Data from International Registry of Acute Aortic Dissection show that about 4% of cases were a complication of these interventions r9
    • Blunt chest trauma r3c84
      • High-energy deceleration injury is common mechanism
    • Stimulant use (eg, cocaine, methamphetamine) r3c85c86c87
      • Associated with hypertension and hyperdynamic state
    • Pregnancy c88
      • Marfan syndrome is present in up to half of pregnancy-associated cases of aortic dissection r13c89
      • Aortic dissection usually occurs in third trimester r13c90
    • Acquired inflammatory vasculitides r3c91
      • May cause weakening of aortic wall
        • Giant cell arteritis c92
        • Takayasu arteritis c93
        • Behçet disease c94

    Diagnostic Procedures

    Primary diagnostic tools

    • History and physical examination findings suggest the diagnosis, but a high index of suspicion is necessary, given overlap with more common conditions r5c95
      • Best predictors are:
        • Abruptness of chest pain
        • Tearing or ripping quality of pain with migration
        • History of hypertension
        • Pulse deficit
        • Focal neurologic deficits
      • Consider risk scoring to determine pretest probability of acute aortic syndrome to direct testing strategy r26
        • 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelinesr3 recommend the use of an algorithm that includes initial evaluation using ADD-RS (aortic dissection detection risk score); however, a 2015 clinical policy from the American College of Emergency Physiciansr10 notes several limitations to this score, in particular, that it was found to be insufficient at excluding the diagnosis of aortic dissection
          • High-risk history includes known aortic aneurysm or aortic valve disease, connective tissue diseases (eg, Marfan syndrome), familial or genetic aortic aneurysm/dissection syndromes, and recent surgical manipulation of aorta (surgical or catheter-based)
          • High-risk pain features and physical signs include:
            • Abrupt onset of severe sharp or ripping chest/back/abdominal pain
            • Presence of pulse deficit
            • Blood pressure differential of more than 20 mm Hg between limbs
            • New murmur of aortic insufficiency
            • New focal neurologic deficit
          • Risk category: r3
            • 2 or more high-risk features: high risk
            • 1 high-risk feature: intermediate risk
            • 0 high-risk features: low risk
    • Obtain imaging based on risk group r3r7
      • Obtain urgent definitive aortic imaging (without waiting for chest radiograph) for all high-risk patients; if first aortic image finding is negative, obtain second imaging study for all high-risk patients or when suspicion is otherwise high r27
        • CT angiography is diagnostic test of choice for most patients with high-risk features r3r28c96
        • Transesophageal echocardiography can be performed at bedside for hemodynamically unstable patients with suspected type A disease for whom CT is not feasible r27c97
          • Transthoracic echocardiography has lower sensitivity for identifying acute aortic syndromes than other modalities and is not considered definitive imaging
        • Magnetic resonance angiography is an additional option, if immediately available r27c98
      • Order chest radiograph for all patients who are at low or intermediate risk, primarily to identify other causes of chest pain, which may obviate the need for definitive aortic imaging r27c99
        • If dilated aorta or widened mediastinum is seen, proceed to definitive aortic imaging; chest radiograph alone is not sufficiently sensitive or specific for diagnosis of acute aortic syndrome
    • Consider D-dimer testing, although do not use a specific result to direct further testing or treatment r29c100
      • European Society of Cardiology guidelinesr7 recommend obtaining D-dimer level; a negative result for a patient with pretest low-risk status decreases likelihood of aortic dissection r30
      • American College of Emergency Physicians guidelinesr10 and 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelinesr3 do not recommend routine D-dimer testing
    • Additional testing is not diagnostic for acute aortic syndrome, but it is directed at differential diagnosis, identification of complications, and expediting time to surgery (for high-risk patients)
      • ECG and troponin levels r3c101c102
      • European Society of Cardiology guidelinesr7 suggest the following be obtained routinely: CBC and levels of creatinine, AST, ALT, C-reactive protein, procalcitonin, lactate, and blood gases c103c104c105c106c107c108c109c110c111
      • Obtain presurgical laboratory testing for all high-risk patients, including CBC, coagulation tests, serum chemistry, and blood type and screen r3c112c113c114c115

    Laboratory

    • D-dimer level c116
      • Elevated D-dimer level, especially in the first hour, increases suspicion of aortic dissection but should not be considered diagnostic for aortic dissection r7r31
        • D-dimer level increases precipitously in aortic dissection compared with other disorders in which the D-dimer level increases gradually r7
      • Low D-dimer level is associated with decreased probability, but more research is needed; not recommended as screening toolr10r3
        • Cutoff is 0.5 mcg/mL r26r29r31
        • Intramural hematoma and penetrating atherosclerotic aortic ulcer may still be present even with D-dimer levels below this cutoff r7

    Imaging

    • CT chest with IV contrast, CT chest without and with IV contrast, or CT angiography of chest with IV contrast (equivalent alternatives) r32r33c117
      • Diagnostic test of choice for most patients r32
      • Highly accurate r27
      • Requires hemodynamic stability sufficient for transport to radiology department
      • Quickly and reliably detects aortic dissection, intramural hemorrhage, and penetrating atherosclerotic ulcer and allows for interventional planning
      • Best images are obtained with arterial phase imaging, ECG gating,r34 and inclusion of entire length of aorta plus iliac and femoral vessels
    • Transesophageal echocardiography r27r33c118
      • Useful in detecting ascending and descending thoracic aortic dissection r35
        • Often cannot clearly visualize the proximal aortic arch owing to tracheal interference r35
      • Able to determine presence of hemopericardium and presence/severity of aortic regurgitation
      • Mid and distal abdominal aorta and branch vessels are often poorly visualized r35
      • Performed with patient under conscious sedation or general anesthesia
      • Can be performed at bedside for hemodynamically unstable patients who would not tolerate transport
    • Magnetic resonance angiography r28r33c119
      • Sensitivity and specificity are similar to those of CT, but the procedure is more time-intensive
        • Use is not appropriate in urgent situations or for unstable patients
      • Gadolinium contrast material is less nephrotoxic than the iodinated contrast material used in CT scanning
    • Chest radiography r28c120
      • Order chest radiograph for all patients who are at low or intermediate risk, primarily to identify other causes of chest pain, which may obviate the need for definitive aortic imaging
      • The following chest x-ray findings are suggestive of aortic dissection r27
        • Mediastinal widening
        • Disruption of the normally distinct contour of the aortic knob
        • Separation of more than 5 mm between intimal calcification and aortic wall (known as the "calcium sign")
        • Double density appearance within the aorta
        • Tracheal deviation to the right
      • Normal chest radiograph does not exclude acute aortic syndrome, and further imaging should be undertaken if acute aortic syndrome is suspected r33

    Other diagnostic tools

    • Can be used in addition to risk category scores
    • ADD-RS (aortic dissection detection risk score)
      • 1 point for risk factor (Marfan syndrome, family history of aortic disease, recent aortic condition or procedure)
      • 1 point for pain feature
      • 1 point for clinical exam feature
      • ADD-RS alone or when combined with D-dimer testing may be useful to determine whether to proceed with imaging
    • AORTA score r26
      • 1 point each for thoracic aortic aneurysm, severe pain, sudden pain, pulse deficit, or neurologic deficit
      • 2 points for hypotension
      • AORTA score over 2 combined with elevated D-dimer may help guide imaging decisions

    Differential Diagnosis

    Most common r15

    • Acute coronary syndrome c121d1
      • Chest pain is usually the primary symptom in both scenarios; it is more likely to be of very abrupt onset and described as knife-like or tearing with acute aortic syndrome
      • Pulse differential and interarm blood pressure differential are unlikely in myocardial infarction
      • ECG may not always be helpful. Dissection-related coronary artery malperfusion may present with ECG changes that are indistinguishable from those of primary myocardial ischemia r3
      • Differentiate with immediate definitive aortic imaging (usually CT angiography) if patient is at intermediate or high risk for acute aortic syndrome
      • Myocardial infarction can coexist with aortic dissection, often as a complication thereof
    • Pulmonary embolism c122d2
      • May present with similar symptoms (eg, chest pain, dyspnea, syncope), although character of pain is more likely to be pleuritic with pulmonary embolism. Dyspnea may be a prominent symptom with pulmonary embolism
      • D-dimer level is elevated in both conditions; it typically rises faster (within first few hours) with aortic dissection, but it is not diagnostically helpful for differentiation
      • Differentiate with CT angiography
    • Pneumothorax c123
      • Usually presents with dyspnea and sudden onset of ipsilateral chest pain described most commonly as pleuritic rather than tearing
      • Physical examination may suggest the diagnosis, with ipsilateral decreased breath sounds, absence of tactile fremitus, and hyperresonance to percussion; cyanosis, hypotension, and tracheal deviation are usually present in tension pneumothorax
      • Differentiate with posteroanterior chest radiograph, which shows loss of lung markings in periphery and a pleural line that runs parallel to chest wall
    • Acute mesenteric ischemia c124d3
      • Usually presents with acute onset of severe periumbilical pain disproportionate to physical examination findings, as opposed to pain of acute aortic dissection, which begins in chest and back and involves radiation of pain as dissection progresses
      • Patients with both conditions often have known cardiovascular disease or risk factors for cardiovascular disease
      • May occur as isolated event (usually due to thrombosis or emboli) or may occur secondary to aortic dissection extending to arteries branching off of the abdominal aorta
      • Differentiate the 2 conditions with CT angiography, imaging the entire abdominal aorta and all abdominal branch arteries
    • Perforated peptic ulcer c125d4
      • Presents with pain typically limited to abdomen in contrast to pain of acute aortic syndrome, which usually begins in chest and/or back
      • Patients with perforated peptic ulcer usually have rigid abdomen and remain motionless with pain; those with aortic dissection usually do not have rigid abdomen and often writhe in pain
      • Erect chest radiograph shows subdiaphragmatic air in perforated peptic ulcer
      • Differentiate acute aortic syndrome from perforated peptic ulcer with CT angiography if there is diagnostic uncertainty

    Treatment

    Goals

    • In acute phase, decrease stress on aortic wall by reducing heart rate and blood pressure r36r37
      • Reduce heart rate to 60 to 80 beats per minute or less (using β-blocker or nondihydropyridine calcium channel blocker) r27
      • Reduce blood pressure to below 120 mm Hg (systolic) or to lowest blood pressure that maintains adequate end-organ perfusion (using vasodilators) r27
      • Manage pain
    • Correctly categorize type of acute aortic syndrome and treat appropriately to prevent propagation of dissection and other life-threatening complications

    Disposition

    Admission criteria

    Criteria for ICU admission
    • If not taken directly to surgery from emergency department, patients with acute aortic syndrome are admitted to ICU for the following: r36
      • Intensive monitoring of vital signs
      • IV medication delivery
      • Preparatory staging for surgery or interventional radiology procedure

    Recommendations for specialist referral r1

    • Obtain immediate urgent consult with cardiovascular surgeon upon diagnosis of acute aortic syndrome

    Treatment Options

    For all patients (with any cause, whether type A or B dissection, intramural hemorrhage, or penetrating atherosclerotic ulcer)

    • Manage blood pressure, heart rate, and pain
      • If heart rate and/or blood pressure are more than 60 to 80 beats per minute or 120 mm Hg or more, respectively), reduce and maintain below these thresholds r27
        • Begin therapy with IV β-blocker (eg, esmolol, labetalol, metoprolol) to reduce heart rate and blood pressure without causing reflex tachycardia r3
          • For β-blocker–intolerant patients, use nondihydropyridine calcium channel blockers (verapamil or diltiazem) r38
            • Nondihydropyridine calcium channel blockers are not recommended for patients with heart failure with reduced ejection fraction due to negative inotropic effects r39
          • Use these agents cautiously in the setting of acute aortic insufficiency, as compensatory tachycardia will be blocked r3
        • Add IV vasodilator if blood pressure goal is not achieved with β-blocker alone r27
          • Options include sodium nitroprusside (most commonly used), ACE inhibitor, or dihydropyridine calcium channel blocker (eg, nicardipine, clevidipine)
          • Avoid administering before starting β-blockers or calcium channel blockers, as it may cause compensatory tachycardia r27
      • If patient is hypotensive or in shock
        • Review imaging for causes (eg, contained rupture, pericardial tamponade, acute aortic insufficiency)
        • Shock usually mandates immediate operative intervention r27
        • Resuscitation with fluid boluses with or without IV vasopressors; avoid inotropes, if possible, because they increase stress on aortic wall r3
        • For patients with hemopericardium and cardiac tamponade who cannot survive until surgery, consider performing pericardiocentesis, withdrawing just enough fluid to restore perfusion r3
      • Manage pain with IV opioids r27
        • This will also help to control blood pressure and heart rate
    • Determine definitive therapy (surgical versus medical) r2r36
      • Type A aortic dissection r27
        • Surgical emergency
        • Proceed to surgery or expedite transfer to more appropriate facility; time intervals between symptom onset, diagnosis, and surgery can significantly affect survival r27r40
          • Mortality rate of acute type A aortic dissection is 1% per hour of delay until surgery r27
          • Patients presenting with clinical manifestations of shock, neurologic deficits, malperfusion, or myocardial ischemia require immediate consideration for surgical repair
          • Surgical intervention corrects aortic regurgitation and myocardial ischemia, reestablishes flow to malperfused vessels, and removes immediate risk of aortic rupture or tamponade r27
      • Type B dissection r27r41r42
        • Complicated type B dissection
          • Surgical or endovascular intervention indicated for type B dissections associated with any of the following:
            • Uncontrolled hypertension
            • Refractory pain
            • Aortic rupture
            • Hypotension or shock
            • Malperfusion of limbs or viscera
          • Thoracic endovascular aortic repair has largely replaced open repair, providing anatomy is favorable, owing to lower morbidity and mortality rates r27r43r44
        • Uncomplicated type B dissection
          • Most uncomplicated cases are managed medically to maintain heart rate and blood pressure goals r27
          • Some surgeons recommend early endovascular aortic repair for uncomplicated type B dissection, particularly for patients with high-risk features on imaging, as it may delay disease progression and reduce risk of subsequent complications r27r45r46
            • Optimal timing of intervention has not been established
          • Beyond the acute phase (ie, chronic aortic dissection), management consists of: r8
            • Lifelong antihypertensive therapy to maintain heart rate and systemic blood pressure goals
              • Transition from IV therapy to oral route
              • Regimens that include β-blockers, ACE inhibitors and angiotensin receptor blockers r47
            • Statin therapy may be beneficial
              • Associated with greater long-term survival for medically managed patients (reducing both cardiovascular mortality and aortic-related mortality) r48
            • Lifelong surveillance with aortic imaging
            • Aortic repair once the dissected aorta becomes significantly aneurysmal
              • Repair is indicated for chronic thoracic and thoracoabdominal aortic dissections with a maximal aortic diameter of 6.0 cm for patients without connective tissue disease r8
              • Patients with connective tissue disease and those with a family history may warrant earlier intervention at aortic diameter of 5.0 to 5.5 cm or even lower in some specific heritable thoracic aortic aneurysm cases r27
              • Rapid growth of the aortic root or ascending aortic aneurysm growth is also an indication for repair r8r27
                • Defined as 0.5 cm or more in 1 year or 0.3 cm or more per year for 2 consecutive years for those with sporadic aneurysms or 0.3 cm or more in 1 year for those with heritable thoracic aortic disease or bicuspid aortic valve
              • Open surgical repair is the main repair method; however, thoracic endovascular aortic repair is reasonable for patients with indications for intervention who are at high risk for complications of open repair due to comorbidities r8r44
      • Intramural hemorrhage
        • Intramural hemorrhage involving the ascending aorta (type A intramural hemorrhage) has high mortality with medical treatment alone; treatment is usually surgical r3
          • Timing of intervention varies: urgent surgery in the case of complicated type A intramural hemorrhage, planned surgery within 24 hours or within a few days, expectant medical management with surgical intervention only for complications or disease progression r27
        • Intramural hemorrhage involving the descending aorta (type B intramural hemorrhage) is managed medically (blood pressure control) in many cases, but surgery or endovascular repair is increasingly being used r49
      • Penetrating atherosclerotic ulcer
        • Generally requires early surgical intervention if associated with persistent pain, hemodynamic instability, or high-risk imaging features (large diameter or depth, significant growth on surveillance imaging, or increasing pleural effusion) r27r49r50

    Drug therapy

    • Blood pressure and heart rate management
      • β-Blockers c126
        • Esmolol c127
          • Esmolol Hydrochloride Solution for injection; Adults: 500 to 1,000 mcg/kg IV over 1 minute, then 50 mcg/kg/minute continuous IV infusion, initially. Repeat bolus and titrate by 50 mcg/kg/minute until goal blood pressure is attained. Max: 200 mcg/kg/minute.
        • Labetalol c128
          • Labetalol Hydrochloride Solution for injection; Adults: 10 to 20 mg IV, then 20 to 80 mg IV every 10 to 30 minutes until goal blood pressure is attained. Max cumulative dose: 300 mg.
        • Metoprolol c129
          • Metoprolol Tartrate Solution for injection; Adults: 2.5 to 5 mg IV every 5 minutes as needed up to 15 mg.
      • Sodium nitroprusside c130
        • Used in conjunction with β-blocker to offset tachycardia caused by nitroprusside
          • Sodium Nitroprusside Solution for injection; Adults: 0.3 to 0.5 mcg/kg/minute continuous IV infusion, initially. Titrate by 0.5 mcg/kg/minute every 5 minutes until desired effect or blood pressure cannot be further reduced without compromising organ perfusion. Max: 10 mcg/kg/minute for 10 minutes.
      • Dihydropyridine calcium channel blockers (indicated as vasodilator for hypertension) c131
        • Clevidipine
          • Clevidipine Emulsion for injection; Adults: 1 to 2 mg/hour continuous IV infusion, initially. Double dose every 90 seconds until the blood pressure approaches goal, then increase by less than double every 5 to 10 minutes as needed. Max: 32 mg/hour or 1,000 mL/24 hours due to lipid load restrictions. Max duration: 72 hours.
        • Nicardipine c132
          • Nicardipine Hydrochloride Solution for injection; Adults: 5 mg/hour continuous IV infusion, initially. Titrate by 2.5 mg/hour every 5 to 15 minutes until goal blood pressure is attained. Max: 15 mg/hour. Reduce to 3 mg/hour after response achieved.
      • ACE inhibitors c133
        • Enalaprilat c134
          • Enalaprilat Solution for injection; Adults: 0.625 to 1.25 mg IV every 6 hours, initially; may increase dose up to 5 mg IV every 6 hours.

    Nondrug and supportive care

    Advise all patients with aortic disease to avoid competitive and body contact sports and strenuous activities that would require the Valsalva maneuver (eg, isometric heavy weightlifting, shoveling snow, chopping wood) r7

    • Low resistance exercise that avoids straining is generally considered acceptable except in cases of severe aortic dilatation
    Procedures
    Surgery c135
    General explanation
    • Gold standard intervention
    • Dissected aortic section is removed and replaced with synthetic graft
    • Some patients may require additional immediate reconstruction owing to nature of dissection
      • Aortic valve revision or replacement
      • Coronary artery bypass grafting
    • Techniques such as hypothermic circulatory arrest and perfusion of the head vessels are often used during surgery
    Indication
    • Type A dissection
    • Complicated type B dissection that cannot be managed medically and for which an endovascular approach is not appropriate
    Complications
    • Operative mortality rate is now less than 18% for type A dissection r51r52
    Thoracic endovascular aortic aneurysm repair r7r36c136
    General explanation
    • Stent is inserted through catheters inserted into femoral artery
    • Balloon is inserted, covering primary entry tear and restoring blood flow into true lumen
    • Prevents further enlargement and eventual aortic rupture
    Indication
    • Preferred approach for most patients with type B dissectionr43, penetrating atherosclerotic ulcer of descending aorta, or intramural hematoma of descending aorta
    Complications
    • Access site complications are uncommon r7
    • Paraparesis/paraplegia occurs in 0.8% to 1.9%, and stroke occurs in 2.1% to 3.5% (lower risk than for open surgery) r7
    Pericardiocentesis r53c137
    General explanation
    • Aspiration of pericardial fluid, ideally under real-time ultrasonographic or fluoroscopic guidance. In an emergency setting, performed without such guidance
    • Patient is placed in supine or semirecumbent position with continuous ECG monitoring; anesthesia is usually local
    • Spinal needle is advanced through subxiphoid skin toward left shoulder or suprasternal notch. Needle is further advanced blindly (in emergency) or with ECG connector attached to the hub while negative pressure is applied to syringe. A pop is usually felt when puncture of pericardium occurs
    • When blood or pericardial fluid is aspirated or cardiac pulsations are felt, a syringe is attached to the needle and pericardial fluid is withdrawn
      • ECG changes suggesting pericardial penetration (increased P-wave amplitude, ST-segment elevation, or ectopic beats) indicate the need to withdraw needle slightly in 1- to 2-mm increments until the changes disappear
    Indication
    • Cardiac tamponade
    Contraindications
    • Anticoagulation is relative contraindication in life-threatening situations
    Complications
    • Myocardial puncture with hemopericardium
    • Coronary artery laceration
    • Bradyarrhythmias and tachyarrhythmias
    • Pneumothorax or hemothorax
    • Hypovolemic hypotension from removing large volumes of fluid
    • Recurrence (up to 70% using blind technique)
    • Cardiac arrest
    Interpretation of results
    • When procedure is done without sonographic or fluoroscopic guidance, false-negative aspiration rates are as high as 80% (usually owing to clotted blood in pericardial space or failure to enter pericardial space)

    Special populations

    • Pregnancy r13
      • Survival rate for aortic dissection during pregnancy is 20% to 30%
      • Aortic dissection is most common in third trimester or postpartum period
      • Marfan syndrome is present in up to half of pregnancy-associated cases of aortic dissection
      • Beyond 30 weeks of gestation, emergency cesarean delivery and subsequent cardiac surgery may be the best option to save the lives of both infant and child bearer
      • Refer patients with known aortic disease or at risk for aortic disease, including those with associated genetic syndromes or disease, for preconception and genetics counseling

    Monitoring

    • After type A or B dissection and intramural hematoma r3
      • Follow-up aortic imaging should be obtained at 1, 3, 6, and 12 months and then annually thereafter r8r49
      • Use the same imaging modality (CT or MRI) at the same institution if possible, so that similar images of matching anatomical segments can be compared side by side
      • If stable over long term, consider switch from CT to MRI to decrease radiation exposure

    Complications and Prognosis

    Complications

    • Death c138
      • Untreated acute type A dissection has a mortality rate of about 1% to 2% per hour initially r40r54
      • For acute type B dissection, in-hospital mortality is 10.6% r55
    • Myocardial infarction r56c139d1
      • Can occur from aortic dissection involvement of aortic arch, secondary to massive blood loss, or as a complication of treatment
    • Aortic valve regurgitation or rupture r56c140c141
      • Can be a complication of aortic dissection itself or of surgical treatment
      • Physical signs include early diastolic decrescendo murmur, heard best in left third intercostal space and radiating to left sternal border
    • Cardiac tamponade r56c142
      • Aortic dissection can cause rupture into lining around heart
      • Physical signs include jugular venous distention, pulsus paradoxus, and distant heart sounds on auscultation
    • Stroke or other neurologic complications c143c144
      • Neurologic complications, including paraparesis, occur in about 14%r57 to 17%r3 of patients with dissection
    • Organ and peripheral damage r56
      • Kidney failure c145d5
        • Can be caused by arterial embolism, by hypotensive state, or directly by propagation of aortic dissection to renal artery
      • Mesenteric ischemia c146
        • Can be caused by arterial embolism, by hypotensive state, or directly by propagation of aortic dissection to visceral arteries
      • Limb ischemia c147c148
        • Can be caused by arterial embolism, by hypotensive state, or directly by propagation of aortic dissection to iliac or femoral arteries
    • Development of a secondary aortic dissection after surgical or endovascular repair
      • Typically develops from a site just distal to surgical or endovascular graft
    • Progressive aortic disease c149c150
      • Progressive disease, including aneurysmal degeneration, occurs in 30% of medically managed patients with uncomplicated type B dissection r58

    Prognosis

    • When aortic disease is detected early and treated promptly, chances of survival greatly improve r36r37
      • Aortic dissection
        • 10-year survival, independent of type of dissection, is 35% to 70% r59
        • Reoperation is required for up to one-third of patients r59
        • Type A dissection
          • Operative mortality has declined; it is now less than 18% r52
          • Lower operative mortality in hospitals with high case volume versus those with low case volume r7
          • Patients treated medically have mortality of 50% r59
        • Type B dissection
          • Patients with medically treated uncomplicated type B dissection have 10% mortality over 5 years r58
            • Morbidity, including aneurysmal degeneration, occurs in 30% r58
          • Use of thoracic endovascular aortic aneurysm repair for management of complicated type B dissection has greatly increased early survival for these patients r42
      • Intramural hematoma
        • May regress, expand, progress to classic dissection, or rupture
        • Reported mortality has varied by region
          • In 3 studies, early mortality with surgery (versus without surgery) was as follows: 0% (versus 80%), 8% (versus 55%), and 14% (versus 36%) r60
          • However, another study primarily from Asian centers showed little difference in outcomes between surgical and medical treatment r60
      • Penetrating atherosclerotic ulcer
        • 30-day mortality for surgical treatment is 21% versus 4% for medical treatment, but some case series have reported much higher mortality for medical management r60
        • Patients with acute penetrating atherosclerotic ulcers are at high risk for aortic-related adverse events and clinically related adverse events within 30 days after onset r11

    Screening and Prevention

    Screening

    At-risk populations r15

    • Patients with bicuspid aortic valve
    • Patients with known genetic conditions
      • Marfan syndrome
      • Loeys-Dietz syndrome
      • Ehlers-Danlos syndrome type IV
      • Familial thoracic aortic aneurysm or dissection
      • Turner syndrome
    • First-degree relatives of patients with aneurysms of the aortic root or ascending aorta or with aortic dissection r27
    • Recent aortic manipulation
    • Aortic aneurysm

    Screening tests

    • Genetic testing and counseling r3c151
      • Recommended for:
        • First-degree relatives of a patient with known thoracic aortic aneurysm or dissection c152
        • First-degree relatives of a patient with known genetic mutation associated with thoracic aortic aneurysm or dissection; after genetic testing and counseling, only the relatives with the genetic mutation should undergo aortic imaging c153c154
    • Aortic imaging and surveillance
      • Modalities include CT angiography of chest and abdomen with IV contrast, magnetic resonance angiography of chest and abdomen with IV contrast, magnetic resonance angiography of chest and abdomen without IV contrast, and chest radiography r61
      • Recommended for: r3c155c156c157
        • Patients with thoracic aortic aneurysm c158c159c160
        • Patients with bicuspid aortic valve c161c162c163
        • Patients with known genetic syndromes associated with thoracic aortic aneurysm and/or dissection
          • Marfan syndrome c164c165c166
            • Echocardiogram is recommended at time of diagnosis of Marfan syndrome to determine diameters of aortic root and ascending aorta r3
            • Repeat at 6-month intervals to determine rate of enlargement of aorta; if stable, can decrease to annual imaging r3
            • If maximal aortic diameter is 4.5 cm or more, or if aortic diameter shows significant growth from baseline, obtain additional imaging r3
          • Loeys-Dietz syndrome or another confirmed (non-Marfan) genetic mutation known to predispose to aortic aneurysms and aortic dissections c167c168c169
            • Complete aortic imaging at initial diagnosis r3
            • Repeat at 6-month intervals to determine if enlargement is occurring r3
            • Patients with Loeys-Dietz syndrome should have yearly imaging from cerebrovascular circulation to pelvis r3
          • Turner syndrome c170c171c172
            • Baseline imaging of heart and aorta for evidence of bicuspid aortic valve, coarctation of aorta, or dilation of ascending thoracic aorta r3
            • If initial imaging findings are normal and there are no risk factors for aortic dissection, obtain repeated imaging every 5 to 10 years or if otherwise clinically indicated r3
            • If abnormalities exist, obtain annual imaging or follow-up imaging r3
        • First-degree relatives of patients with thoracic aortic aneurysm and/or dissection to identify those with asymptomatic disease r3c173c174c175
          • Imaging of second-degree relatives is reasonable if 1 or more first-degree relatives of a patient with known thoracic aortic aneurysm and/or dissection are found to have thoracic aortic dilation, aneurysm, or dissection

    Prevention

    • Patients with known thoracic aortic disease: r8c176
      • Antihypertensive therapy
      • Restriction from contact sports and other strenuous activities
      • Lifelong surveillance with aortic imaging
      • Surgical intervention based on aortic caliber, growth rate, pathogenesis, and presence of symptoms r8
    • Patients with Marfan syndrome or Ehlers-Danlos syndrome type IV: restriction from contact sports and other strenuous activities r6r62c177c178
    • Patients with Marfan syndrome: prophylactic elective replacement of aortic root may be an option r6r63c179
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