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Sep.15.2021

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
  • Men older than 40 years with hypertension, and those 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 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 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-negatives 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 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 acute aortic syndrome r7

Classification

  • Aortic dissection: classification based on anatomy
    • Stanford classification r5
      • Type A
        • Dissection originates in root of aorta or upper ascending aorta; may extend into abdominal aorta
      • Type B
        • Dissection is confined to descending aorta
    • DeBakey classification r4
      • Type I
        • Dissection originates in ascending aorta and propagates distally at least to aortic arch and typically to descending aorta
      • Type II
        • Dissection originates in and is confined to ascending aorta
      • 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 increases likelihood of acute aortic syndrome c1c2c3c4c5
  • Seek history of stimulant drug use (eg, cocaine, methylenedioxymethamphetamine, methamphetamine) c6c7c8
  • 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 are 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 c9
        • Most patients describe pain as sharp and knife-like; others describe it as tearing or ripping r3r8c10
        • Abruptness of onset is a specific characteristic (84%) r8
        • 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 r3r9c11c12
          • Begins as back pain in type B (64% of cases), but presentation with chest and/or abdominal pain also occurs r3c13c14c15
          • Pain may be migratory as dissection extends c16
      • May present with syncope (15% with type A; less than 5% with type B) r7c17c18
      • 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 r3c19c20
        • Flank pain and decreased urine output are likely if renal artery is involved in dissection c21c22
        • Dyspnea and orthopnea are likely if ischemic congestive heart failure is present or if left ventricular failure results from aortic rupture into left atrium c23c24c25c26
        • 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) c27c28c29
        • Rarely, hemoptysis is caused by rupture into tracheobronchial tree c30
        • Rarely, hematemesis is caused by rupture into esophagus c31
    • Intramural hematoma
      • Patient is more likely to be older and more likely to be female than in aortic dissection r4
      • Presents with pain similar to that of aortic dissection r4c32c33
      • Less likely to present with neurologic symptoms
    • Penetrating atherosclerotic ulcer r10
      • Patient is more likely to be an older man and more likely to have known coronary artery disease or risk factors for atherosclerotic disease, than in aortic dissection r7c34c35
      • Presents with pain similar to that of aortic dissection r3r4c36c37
      • Rarely presents with symptoms of dissection-related lack of organ perfusion

Physical examination

  • May appear anxious and diaphoretic c38c39
  • Blood pressure may be high, normal, or low r11
    • Measure blood pressure in both arms; highest reading will be used to determine blood pressure management plan r3
    • Type A dissection
      • Hypotension in about 25% r8c40
      • Hypertension in about 35% r8c41
    • Type B dissection
      • Hypertension in about 70% r8c42
  • Cardiopulmonary examination
    • Pulse deficit (apical versus radial) is present in about 18% of type A cases and about 9% of type B cases r8c43c44
    • Pressure differential (interarm blood pressure difference) greater than 20 mm Hg is suggestive (although studies differ on its significance) r9c45
      • 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 r8c46c47
    • 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 r8c48c49c50c51
    • Cool, pale lower extremities with decreased or absent pulses are likely if iliac and femoral arteries are involved c52c53c54c55c56c57c58c59
  • Neurologic examination
    • Signs of stroke are present in about 6% of type A cases and about 2% of type B cases r8
    • Hemiparesis is likely if spinal artery malperfusion is present c60
    • Hoarseness (from laryngeal nerve compression) may occur c61

Causes and Risk Factors

Causes

  • Often multifactorial; most common factors include uncontrolled hypertension and inherited susceptibility r5
    • Hypertension c62
      • Elevated systolic pressure can propagate an aortic dissection c63
      • Physical exertion or emotional stress is direct precursor of pain in two-thirds of aortic dissection events; both involve acute changes in blood pressure r11c64c65
    • Genetic syndromes c66
      • Congenital connective tissue diseases (associated with abnormalities of aortic media) c67
        • Marfan syndrome r12c68
          • 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 r11
        • Ehlers-Danlos syndrome type IV r13c69
          • Autosomal dominant genetic disorder characterized by skin hyperextensibility, joint hypermobility, and tissue fragility (which endangers heart and blood vessels)
          • Arterial complications are the leading cause of death
        • Loeys-Dietz syndrome c70
          • 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 c71
        • Familial Marfan-like habitus c72
        • Familial ectopia lentis c73
        • Familial aortic dissection c74
        • Erdheim cystic medial necrosis c75
        • Congenital contractural arachnodactyly c76
      • Turner syndrome c77
        • Phenotypic female with 45,X karyotype
        • May have associated bicuspid aortic valve and/or aortic coarctation

Risk factors and/or associations r14

Age
  • Incidence rises with age; mean age at presentation is 63 years r8c78
  • Age at presentation is often lower in patients with Marfan syndrome, bicuspid aortic valve, or history of previous aortic surgery r3c79
  • Case reports of aortic dissection in children as young as 3 years r3
Sex
  • Over two-thirds of aortic dissection events occur in men r11c80c81
  • Women have higher mortality rates from aortic dissection than men r15c82c83
Genetics
  • Marfan syndrome: heterozygous mutation in the FBN1 gene (fibrillin 1; OMIM *134797)r16 on chromosome band 15q21 (phenotype is OMIM #154700)r17c84
  • Ehlers-Danlos type IV syndrome: heterozygous mutation in the COL3A1 gene (collagen type III alpha 1 chain; OMIM *120180)r18 on chromosome band 2q32 (phenotype is OMIM #130050)r19c85
  • Loeys-Dietz syndrome type 2: heterozygous mutation in the TGFBR2 gene (transforming growth factor beta receptor 2; OMIM *190182)r20 on chromosome band 3p22 (phenotype is OMIM #610168)r21c86
Other risk factors/associations
  • Congenital cardiovascular malformations c87c88
    • Bicuspid aortic valve c89c90
      • 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 r22r23
    • Aortic coarctation c91c92
      • Significantly increases risk of aortic dissection, particularly when in association with bicuspid aortic valve (odds ratio, 4.7) r11
  • Preexisting thoracic aortic aneurysm r3c93
    • Aortic dissection is frequently superimposed on thoracic aortic aneurysms
  • Previous cardiac interventions c94
    • Aortic dissection is a rare but dangerous complication of cardiac surgery and cardiac catheterization r24
    • Data from International Registry of Acute Aortic Dissection show that about 4% of cases were caused by these interventions r8
  • Blunt chest trauma r3c95
    • High-energy deceleration injury is common mechanism
  • Stimulant use (eg, cocaine, methamphetamine) r3c96c97c98c99c100c101
    • Associated with hypertension and hyperdynamic state
  • Pregnancy c102
    • Marfan syndrome is present in up to half of pregnancy-associated cases of aortic dissection r12c103
    • Aortic dissection usually occurs in third trimester r12c104
  • Acquired inflammatory vasculitides r3c105
    • May cause weakening of aortic wall
      • Giant cell arteritis c106
      • Takayasu arteritis c107
      • Behçet disease c108

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 r5c109
    • 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 r25
      • 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelinesr3 recommend this strategy, but a 2015 clinical policy from the American College of Emergency Physiciansr9 recommends against using this score to direct testing
        • 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, or 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 r3
      • CT angiography is diagnostic test of choice for most patients with high-risk features r3r26
      • Bedside transesophageal echocardiography can be used for patients with suspected type A disease when CT is not feasible owing to hemodynamic instability r3
        • Transthoracic echocardiography has poor sensitivity and cannot be considered definitive imaging
      • MRI is an additional option if immediately available r3
    • 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 r3
      • 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
      • For patients at intermediate risk, proceed to definitive aortic imaging after chest radiograph if no alternative cause of symptoms has been identified
      • For patients at low risk, consider definitive aortic imaging if clinical scenario is suggestive and no alternative explanation for symptoms has been identified
  • Consider D-dimer testing, although do not use a specific result to direct further testing or treatment r27
    • European Society of Cardiology guidelinesr7 recommend obtaining D-dimer level; a negative result in a patient with pretest low-risk status decreases likelihood of aortic dissection r28
    • American College of Emergency Physicians guidelinesr9 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 r3
    • 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
    • Obtain presurgical laboratory testing for all high-risk patients, including CBC, coagulation tests, serum chemistry, and blood type and screen r3

Laboratory

  • D-dimer level c110
    • Elevated D-dimer level, especially in the first hour, increases suspicion of aortic dissection, but it cannot be used as diagnostic biomarker r7r29
      • 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 toolr9r3
      • With a cutoff of 0.5 mcg/mL r25r27r29
      • Intramural hematoma and penetrating atherosclerotic aortic ulcer may still be present even with D-dimer levels below this cutoff r7

Imaging

  • CT angiography r30c111
    • Diagnostic test of choice for most patients r30
      • Test sensitivity and specificity are both 100% r3
    • 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,r31 and inclusion of entire length of aorta plus iliac and femoral vessels
  • Transesophageal echocardiography c112
    • Useful in ascending and descending thoracic aortic dissection r32
      • Often cannot clearly visualize the proximal aortic arch owing to tracheal interference r32
    • Able to determine presence of hemopericardium and presence/severity of aortic regurgitation
    • Mid and distal abdominal aorta and branch vessels are often poorly visualized r32
    • Performed under conscious sedation or general anesthesia
    • Can be performed at bedside in hemodynamically unstable patients who would not tolerate transport
  • Magnetic resonance angiography r26c113
    • Sensitivity and specificity are similar to those of CT, but the procedure is more time-intensive
      • Use is not appropriate in urgent situations
    • Gadolinium contrast material is less nephrotoxic than the iodinated contrast material used in CT scanning
  • Chest radiography r26c114c115
    • 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

Other diagnostic tools

  • Can be used in addition to risk category scores
  • Aortic Dissection Risk Score (ADD-RS)
    • 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 with D-dimer may be a useful screening test to determine whether to proceed with imaging
  • AORTA score r25
    • 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 r14

  • Acute coronary syndrome c116d1
    • 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 c117d2
    • 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 c118
    • 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 c119d3
    • 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 branch arteries off of abdominal aorta
    • Differentiate the 2 conditions with CT angiography, imaging the complete abdominal aorta and abdominal branch arteries
  • Perforated peptic ulcer c120d4
    • 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 r33r34
    • Reduce heart rate to goal of 60 beats per minute or less (using β-blocker or calcium channel blocker) r3
    • After reducing heart rate, reduce blood pressure to goal of 120 mm Hg or less (systolic) using vasodilators (to lowest blood pressure that maintains adequate end-organ perfusion) r3
    • 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: r33
    • 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 higher than goal (more than 60 beats per minute or more than 120 mm Hg, respectively):
      • Use medical therapy, first to decrease heart rate to 60 beats per minute or less and then to reduce and maintain systolic blood pressure at 120 mm Hg or less r3
        • Begin therapy with IV β-blocker (ie, esmolol, labetalol, propranolol, metoprolol) r3
          • Use cautiously in the setting of acute aortic insufficiency; compensatory tachycardia will be blocked r3
        • Use verapamil or diltiazem if β-blockers are ineffective (or patient is intolerant of β-blockade) r3
        • Add sodium nitroprusside (most commonly used), ACE inhibitor, nicardipine, or other vasodilator to β-blocker if blood pressure goal is not achieved r3
    • 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 r3
      • Begin resuscitation as follows:
        • Administer 1 or more fluid boluses titrated to improvement of blood pressure, aiming for euvolemia r3
        • If condition is unresponsive, begin IV vasopressors (eg, norepinephrine, phenylephrine); 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
  • Determine definitive therapy (surgical versus medical) r2r33
    • For type A dissection, proceed to surgery or expedite transfer to more appropriate facility; mortality risk increases 1% to 2% every hour of delay until surgery r3r35
    • For type B dissection, definitive treatment is less clear r3r36r37
      • Medical management of hypertension is appropriate in most cases, with surgical intervention (usually an endovascular procedure) indicated for the following complications:
        • Uncontrolled hypertension
        • Refractory pain
        • Aortic rupture
        • Hypotension or shock
        • Malperfusion of limbs or viscera
      • Although it is controversial, some surgeons recommend endovascular aortic repair for cases with type B dissection that they consider uncomplicated r38r39
        • Aim is prophylaxis against later dissection-related complications
    • For intramural hemorrhage and penetrating atherosclerotic ulcer
      • If the damage involves the ascending aorta, high mortality occurs with medical treatment alone; treatment is usually surgical r3
      • If the damage involves the descending aorta, blood pressure control is the primary treatment in many cases, but surgery (especially endovascular repair) is increasingly being used r40
      • Penetrating atherosclerotic ulcer diameter more than 15 mm is associated with high risk for disease progression; consider early surgical intervention r41
      • Persistent pain, hemodynamic instability, or increase in ulcer/hematoma size requires early surgical intervention r40
  • Maintain effective blood pressure and heart rate control after discharge
    • Convert to oral formulations of antihypertensives and ensure adequate follow-up r7
      • Goal for chronic hypertension
        • Less than 140/90 mm Hg (European aortic diseases guideline) r7
        • Less than 140/90 mm Hg (patients without diabetes) or less than 130/80 mm Hg (patients with diabetes or chronic renal disease) (American aortic diseases guideline) r3
        • Lower blood pressure targets may be beneficial
    • Optimal outpatient medical regimen for type B dissection managed conservatively needs further study; aim is to prevent aortic enlargement and aneurysm r7
      • Statins may inhibit expansion of aneurysms r7
      • For patients with Marfan syndrome, β-blockers are recommended; angiotensin receptor blockers and ACE inhibitors reduce progression of aortic dilation r7
    • Advise all patients with enlarged aorta to avoid sports and other strenuous activities (eg, shoveling snow, chopping wood) r7

Drug therapy

  • Blood pressure and heart rate management
    • β-blockers c121
      • Labetalol c122
        • Labetalol Hydrochloride Solution for injection; Children and Adolescents: 0.2 to 1 mg/kg IV bolus (Max: 40 mg), then 0.25 to 3 mg/kg/hour continuous IV infusion.
        • 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.
      • Esmolol c123
        • 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.
      • Propranolol c124
        • Propranolol Hydrochloride Solution for injection; Neonates†: 0.01 mg/kg/dose IV push over 10 minutes, repeated every 6 to 8 hours as needed. Titrate gradually as needed; heart rate may be dose-limiting. Max: 0.15 mg/kg/dose.
          • Off-label use in this age group
        • Propranolol Hydrochloride Solution for injection; Children and Adolescents: 0.01 mg/kg/dose IV push over 10 minutes, repeat every 6 to 8 hours as needed. May titrate dosage gradually as needed. Max: 0.15 mg/kg/dose or 3 mg/dose.
        • Propranolol Hydrochloride Solution for injection; Adults: Limited data suggest continuous IV at a rate of 2 to 3 mg/hour may be effective in post-surgical patients who cannot tolerate oral therapy; infusions were given for up to 9 days.
      • Metoprolol r3c125
        • Metoprolol Tartrate Solution for injection; Adults: 2.5 to 5 mg IV bolus over 1 to 2 minutes; may repeat every 5 minutes to a maximum of 3 doses.
    • Calcium channel blockers c126
      • For β-blocker–intolerant patients, verapamil or diltiazem may be used to reduce heart rate or to reduce blood pressure without causing reflex tachycardia r42
      • Verapamil r3r42c127
        • Verapamil Hydrochloride Solution for injection; Children and Adolescents: Initially, 0.1 to 0.3 mg/kg (ie, 2 to 5 mg) IV bolus, given over at least 2 minutes. Max single dose: 5 mg. If no response, repeat dose in 30 minutes. Max total dose: 10 mg.
        • Verapamil Hydrochloride Solution for injection; Adults: Initially, 5 to 10 mg (0.075-0.15 mg/kg) IV over at least 2 minutes. If no adequate response after 30 minutes, may give an additional 10 mg (0.15 mg/kg). An optimal interval for subsequent doses has not been determined and should be individualized for each patient. Clinical practice guidelines recommend 2.5 to 5 mg IV over 2 minutes. If no therapeutic response or adverse reaction is seen, may administer repeated doses of 5 to 10 mg every 15 to 30 minutes up to a total dose of 20 mg. Alternative dosing is 5 mg IV every 15 minutes, up to a total dose of 30 mg.
      • Diltiazem r3r42c128
        • Diltiazem Hydrochloride Solution for injection; Infants older than 7 months†, Children†, and Adolescents†: 0.25 mg/kg IV over 5 minutes followed by 0.05 to 0.15 mg/kg/hour continuous IV infusion (mean 0.11 mg/kg/hour) has been reported.
        • Diltiazem Hydrochloride Solution for injection; Adults: Initially, 0.25 mg/kg IV bolus over 2 minutes. After 15 minutes, 0.35 mg/kg over 2 minutes may be given. Individualize as needed. A 10 mg/hour IV infusion is started immediately after IV bolus. Some patients respond to lower doses (eg, 5 mg/hour). Max: 15 mg/hour.
      • Nicardipine (indicated as vasodilator for hypertension) c129
        • Nicardipine Hydrochloride Solution for injection; Neonates: 0.5 to 1 mcg/kg/minute continuous IV infusion, initially. Titrate every 15 to 30 minutes until goal blood pressure is attained. Usual dose: 0.5 to 2 mcg/kg/minute. Max: 6 mcg/kg/minute.
        • Nicardipine Hydrochloride Solution for injection; Infants, Children, and Adolescents: 30 mcg/kg (Max: 2 mg) IV, then 0.5 to 4 mcg/kg/minute continuous IV infusion, initially. Titrate by 1 mcg/kg/minute every 15 to 30 minutes until goal blood pressure is attained. Max: 10 mcg/kg/minute.
        • 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.
    • Sodium nitroprusside c130
      • Used in conjunction with β-blocker to offset tachycardia caused by nitroprusside
        • Sodium Nitroprusside Solution for injection; Infants, Children, and Adolescents: 0.3 to 3 mcg/kg/minute continuous IV infusion, initially. Titrate 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.
        • 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.
    • ACE inhibitors c131
      • Enalaprilat c132
        • Enalaprilat Solution for injection; Neonates, Infants, Children, and Adolescents: 5 to 10 mcg/kg/dose IV every 8 to 24 hours (determined by blood pressure readings) (Max: 1.25 mg/dose).
        • Enalaprilat Solution for injection; Adults: 1.25 to 5 mg IV every 6 hours.

Nondrug and supportive care

Procedures
Surgery c133c134c135
General explanation
  • Gold standard intervention
  • Dissected aortic section is removed and is 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 r43r44
  • Early surgical treatment of patients with acute type A dissection involved mortality between 28% and 58%. Despite continuous advances in diagnostic methods, operative technique, and perioperative care, acute type A dissection remains a major unsolved cardiovascular surgical challenge, as illustrated by the most recent International Registry of Acute Aortic Dissection report showing an 18% operative mortality in a contemporary cohort (2010-2013) r43
Thoracic endovascular aortic aneurysm repair r7r33c136c137c138c139
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
  • Appropriate for selected patients with type B dissection, penetrating atherosclerotic ulcer of descending aorta, and 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 r45c140c141
General explanation
  • Aspiration of pericardial fluid, ideally under real-time ultrasonographic or fluoroscopic guidance. In the 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)

Comorbidities

  • Hypertension c142
    • Uncontrolled hypertension can propagate aortic dissection
    • Ability to control hypertension may influence treatment choice in type B aortic dissection
      • Conservative management is reasonable only if systolic blood pressure can be controlled and maintained at goal
        • Less than 140/90 mm Hg (European aortic diseases guidelines) r7
        • Less than 140/90 mm Hg (patients without diabetes) or less than 130/80 mm Hg (patients with diabetes or chronic renal disease) (American aortic diseases guidelines) r3

Special populations

  • Pregnancy r12
    • Survival rate for aortic dissection during pregnancy is 20% to 30%
    • Aortic dissection usually occurs in third trimester
    • Marfan syndrome is present in up to half of pregnancy-associated cases of aortic dissection
    • Beyond 30 weeks’ gestation, emergency cesarean delivery and subsequent cardiac surgery may be the best option to save the lives of both baby and mother

Monitoring

  • After type A or B dissection and intramural hematoma r3
    • Obtain follow-up aortic imaging at 1, 3, and 6 months and annually thereafter r40
    • Use the same imaging modality (CT or MRI) at the same institution if possible, so that similar images of matching anatomic 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 c143c144
    • Untreated acute type A dissection has a mortality rate of about 1% to 2% per hour initially r35r46
    • For acute type B dissection, in-hospital mortality is 10.6% r47
  • Myocardial infarction r48c145c146d1
    • 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 r48c147c148
    • 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 r48c149
    • 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 c150c151
    • Neurologic complications, including paraparesis, occur in about 14%r49 to 17%r3 of patients with dissection
  • Organ and peripheral damage r48
    • Kidney failure c152c153c154d5
      • Can be caused by arterial embolism, by hypotensive state, and directly by propagation of aortic dissection to renal artery
    • Mesenteric ischemia c155c156c157
      • Can be caused by arterial embolism, by hypotensive state, and directly by propagation of aortic dissection to visceral arteries
    • Limb ischemia c158c159c160c161
      • Can be caused by arterial embolism, by hypotensive state, and 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 c162c163
    • Progressive disease, including aneurysmal degeneration, occurs in 30% of medically managed patients with uncomplicated type B dissection r50

Prognosis

  • When aortic disease is detected early and treated promptly, chances of survival greatly improve r33r34
    • Aortic dissection
      • 10-year survival, independent of type of dissection, is 35% to 70% r51
      • Reoperation is required in up to one-third of patients r51
      • Type A dissection
        • Operative mortality has declined; it is now less than 18% r43
        • Lower operative mortality in hospitals with high case volume versus those with low case volume r7
        • Patients treated medically have mortality of 50% r51
      • Type B dissection
        • Medically treated uncomplicated type B cases have 10% mortality over 5 years r50
          • Morbidity, including aneurysmal degeneration, occurs in 30% r50
        • Use of thoracic endovascular aortic aneurysm repair for management of complicated type B dissection has greatly increased early survival of these patients r37
    • 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%) r52
        • However, another study primarily from Asian centers showed little difference in outcomes between surgical and medical treatment r52
    • 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 r52
      • Patients with acute PAUs are at high risk for aortic-related adverse events and clinically related adverse events within 30 days after onset. r10

Screening and Prevention

Screening

At-risk populations r14

  • Patients with bicuspid aortic valve
  • Patients with known genetic conditions
    • Marfan syndrome
    • Loeys-Dietz syndrome
    • Ehlers-Danlos syndrome
    • Familial aneurysmal and dissection syndromes
    • Turner syndrome
  • First-degree relatives of patients with thoracic aortic aneurysm or dissection
  • Recent aortic manipulation
  • Aortic aneurysm

Screening tests

  • Genetic testing and counseling is recommended for: r3c164
    • First-degree relatives of a patient with known thoracic aortic aneurysm or dissection c165c166
    • 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 c167c168c169c170
  • Aortic imaging (choice of test per recommendation of cardiologist or cardiothoracic surgeon consultant) and surveillance is recommended for: r3c171c172c173
    • Patients with thoracic aortic aneurysm c174c175c176
    • Patients with bicuspid aortic valve c177c178c179
    • Patients with known genetic syndromes associated with thoracic aortic aneurysm and/or dissection
      • Marfan syndrome c180c181c182
        • 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 c183c184c185
        • 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 c186c187c188
        • 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 r3c189c190c191c192c193c194
      • 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 r6

  • Manage blood pressure c195
  • In patients with preexisting thoracic aortic aneurysm, counsel on adherence to imaging surveillance protocol
  • In patients with Marfan syndrome or Ehlers-Danlos syndrome, counsel on restriction from contact sports and other strenuous activities r53c196c197c198c199
  • In patients with Marfan syndrome, counsel on the option of prophylactic elective replacement of aortic root r54c200
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