Cardiovascular Medicine Book

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Aortic Dissection

Aka: Aortic Dissection, Dissecting Aortic Aneurysm, Thoracic Aortic Dissection
  1. Epidemiology
    1. Gender: Most common in males by factor of 2-3 to 1
    2. Age: 40-80 years old
    3. Incidence: 6000-10,000 per year in US (rare)
  2. Pathophysiology
    1. Aortic wall is composed of three layers: Intima (inner), media and adventitia (outer)
    2. Aortic Dissection has a very different mechanism than Abdominal Aortic Aneurysm
      1. AAA is caused by atherosclerosis and involves all three layers of aorta wall
      2. Aortic Dissection is caused by Hypertension and involves only the innermost layer (intima)
    3. Intimal tear precedes dissection
  3. Precautions
    1. Keep Aortic Dissection in the Chest Pain differential diagnosis
    2. Aortic Dissection may present in similar fashion to Acute Coronary Syndrome, Pulmonary Embolism, Pericarditis
      1. However, empiric antiplatelets and Anticoagulants can result in worse outcomes for dissection
    3. Atypical presentations are very common with a wide variety of findings that mimic other conditions
      1. Transient global amnesia or Altered Mental Status
      2. Painless lower extremity weakness
      3. New onset CHF with ST Elevation MI
      4. Renal or Intestinal Ischemia
  4. Risk Factors
    1. Male gender (>2:1 ratio)
    2. Pregnancy
    3. Cocaine Abuse or other Sympathomimetics
    4. Chronic Hypertension (present in 70-90% of cases, esp uncontolled)
    5. Giant Cell Arteritis
    6. Family History of aortic disease
    7. Pre-existing aorta structural abnormalities
      1. Bicuspid aortic valve
      2. Aortic Coarctation
      3. Thoracic Aortic Aneurysm
      4. Prior Aortic Dissection history
      5. Cardiovascular procedures (especially recent)
        1. Cardiac or aorta surgery
        2. Cardiac catheterization
    8. Connective Tissue Disease (presentation at younger ages, <40 years old)
      1. Marfan's Syndrome
      2. Ehlers-Danlos Syndrome
    9. Other risk factors
      1. Hyperlipidemia
      2. Tobacco Abuse
      3. Weight Lifting
      4. Pheochromocytoma
      5. Polycystic renal disease
      6. Chronic Corticosteroid use
      7. Chronic immunosuppressant use
      8. Aortic wall infections
  5. Types: Standford Classification
    1. Type A (60-65%, Debakey Type I and II)
      1. Ascending Aorta and/or aortic arch (dissection may extend intracardiac)
      2. In a Debakey Type II, the Aortic Dissection is limited to the aortic arch
    2. Type B (30-35%, Debakey Type III)
      1. Descending Aorta (after origin of subclavian artery)
  6. Symptoms
    1. Chest Pain (94% of patients)
      1. Severe, sudden tearing sensation in the chest, back or Abdomen (may radiate into legs)
      2. Aortic Dissection pain radiates to back or Abdomen
        1. Myocardial Infarction rarely radiates like this
      3. Aortic Dissection pain is most severe at onset
        1. Myocardial Infarction pain is typically crescendo in nature
    2. Neurovascular symptoms
      1. Cerebrovascular Accident
        1. Visual deficit
        2. Hemiparesis
        3. Bilateral paresis
      2. Syncope
      3. Extremity Paresthesias
  7. Symptoms: Test Sensitivity at presentation with Aortic Dissection (based on IRAD Data)
    1. Classic Triad (100% Test Specificity if present, but most cases are atypical and do not have all 3 findings)
      1. Severe abrupt onset, ripping or tearing Chest Pain that radiates to back AND
      2. Pulse deficit or difference in upper extremity Blood Pressure >20 mmHg AND
      3. Mediastinal Widening or aortic knob widening on Chest XRay
    2. Timing
      1. Sudden onset: 85%
    3. Severity
      1. Severe pain: 90%
    4. Characteristics
      1. Pain: 95%
        1. Type A: 94%
        2. Type B: 98%
      2. Sharp pain: 64%
      3. Tearing/ripping: 50%
        1. Type A: 49%
        2. Type B: 52%
    5. Distribution: Typically involves both above and below the diaphragm
      1. Anterior Chest Pain: 61%
        1. Type A: 71%
        2. Type B: 44%
      2. Back pain: 53%
        1. Type A: 46%
        2. Type B: 64%
      3. Abdominal Pain: 35%
        1. Type A: 22%
        2. Type B: 42%
      4. Migrating pain: 17%
        1. Type A: 15%
        2. Type B: 19%
    6. Associated Findings
      1. Syncope: 9%
        1. Type A: 13%
        2. Type B: 4%
  8. Signs
    1. Blood Pressure at presentation (based on IRADS results)
      1. Hypertensive SBP>150: 49%
        1. Type A: 36%
        2. Type B: 70%
      2. Normotensive SBP 100-150: 35%
      3. Hypotensive or shock SBP: 16%
        1. Type A: 25%
        2. Type B: 4%
      4. Blood Pressure differential between sides
        1. Poor sensitivity and Specificity for Aortic Dissection
        2. Up to 20% of normal patients have a Blood Pressure differential of at least 20 mmHg
    2. Pulse on presentation
      1. Pulse deficit: 15-30%
        1. Type A Dissection: Two thirds of those with pulse deficit
        2. Type B Dissection: One third of those with pulse deficit
        3. Positive Likelihood Ratio when associated with acute Chest Pain or back pain: 5.3 to 5.7
          1. Von Kodolitsch (2000) Arch Intern Med 160(19): 2977-82 [PubMed]
      2. Palpable pulse differential
        1. Less prominent pulse (e.g. radial pulse) on one side compared with the other
    3. Aortic Murmur: 30%
      1. Aortic Regurgitation murmur suggests a Type A dissection with intracardiac involvement
      2. Overall, new murmurs are found in 50% of Aortic Dissection patients
    4. Cardiac Tamponade (5% of Type A Dissections)
    5. Findings associated with dissection of hematoma
      1. Altered Mental Status (12% of Type A Dissections)
      2. Cerebrovascular Accident (8% of Type A Dissections)
      3. Focal neurologic deficit (e.g. Hemiplegia)
      4. Pulse deficits
      5. Aortic Insufficiency
      6. Mesenteric Ischemia
      7. Acute Kidney Injury
      8. Paraplegia (spinal artery Occlusion)
  9. Labs
    1. Basic metabolic panel
      1. Acute Renal Failure may occur depending on level of dissection
    2. D-Dimer
      1. Consider when evaluating differential diagnosis
      2. Normal D-Dimer (up to 10% False Negative Rate) does not exclude thoracic dissection
    3. Serum Troponin
      1. Elevated in Acute Coronary Syndrome, Aortic Regurgitation
  10. Diagnostics
    1. Electrocardiogram
      1. Test Sensitivity: 69%
      2. Test Specificity: Low (non-diagnostic)
      3. Left Ventricular Hypertrophy
      4. Myocardial Ischemia
      5. Myocardial Infarction
    2. Emergency Echocardiography (bedside)
      1. Evaluate for Pericardial Effusion
  11. Imaging
    1. Aortic Angiography (gold standard)
      1. Test Sensitivity: 90-98%
      2. Test Specificity: 95-98%
    2. CT Angiography Chest (Chest CTA) - preferred first line study
      1. Similar efficacy to Transesophageal Echocardiogram (TEE) or MRA
      2. Test Sensitivity: 100% with new generation CT (older studies quoted 94%)
      3. Test Specificity: 98% with new generation CT (older studies quoted 90%)
      4. Risk of cardiac motion artifacts near the aortic root
        1. EKG Gating can reduce this artifact
    3. Transesophageal Echocardiogram
      1. Limited availability at non-tertiary hospitals
      2. Test Sensitivity: 97%
      3. Test Specificity: 75-90%
    4. Transthoracic Echocardiogram
      1. Test Sensitivity: 77-80%
      2. Test Specificity: 74-96%
      3. Not recommended to rule-out Aortic Dissection (low Test Sensitivity)
      4. Does identify Aortic Dissection complications (Cardiac Tamponade)
      5. Bedside Ultrasound evaluation on Parasternal Long-Axis Echocardiogram View (PLAX View)
        1. Aorta Diameter Measurement on PLAX View (for Aortic Dissection)
        2. Measure the maximal distance between anterior and posterior walls of aorta
          1. Probe should be perpendicular to the two aorta walls
          2. Distance >4 cm is concerning for Aortic Dissection
          3. Other suggestive findings: Pericardial Effusion, flap within the aorta
    5. MRA Chest
      1. Not recommended as an emergency evaluation (may be indicated in some stable patients)
      2. May be considered when iodinated contrast or CTA is contraindicated
      3. Test Sensitivity: 98%
      4. Test Specificity: 98%
    6. Chest XRay
      1. Test Sensitivity: 64-71% (up to 90% for a completely otherwise normal Chest XRay)
      2. Test Specificity: Low (non-diagnostic)
      3. Unlikely to demonstrate anything more than intrathoracic catastrophe
      4. Mediastinal Widening (progressive), aortic knob widening
      5. Double density aorta (lines define margins of true and false lumens)
      6. Tracheal, Bronchial or esophageal deviation to the right
      7. Pleural Effusion
  12. Complications
    1. Neurologic deficits
      1. Cerebrovascular Accident
    2. Unequal perfusion
      1. Unequal pulses
      2. Unequal extremity Blood Pressures
    3. Myocardial Ischemia or Myocardial Infarction
      1. Proximal Aortic Dissection involves the coronary arteries in 3% of cases
    4. Aortic Regurgitation (with Cardiogenic Shock)
    5. Aortic valve rupture
    6. Cardiac Tamponade
  13. Management: Acute Management
    1. Consult Vascular Surgeon early on suspicion of Aortic Dissection
      1. See Surgical Management as below
    2. Lower Blood Pressure (in addition to Heart Rate lowering)
      1. Goals (confirm goal levels with accepting vascular surgeon)
        1. Blood Pressure goal: <120 mmHg (based on consensus expert opinion)
        2. Heart Rate goal: <60 bpm (based on consensus expert opinion)
      2. First-Line Agents: Beta Blockers
        1. Esmolol
        2. Labetalol 20-40 mg incremental boluses IV
          1. Consider while awaiting CT imaging and diagnosis
        3. Metoprolol
      3. Second-Line Agents: Calcium Channel Blockers
        1. Nicardipine
        2. Clevidipine
      4. Third-line Agents (Refractory Hypertension after rate control)
        1. ACE Inhibitors (e.g. IV enaprilat)
        2. Vasodilators (e.g. Nitroprusside 0.5-10 ug/kg/min IV)
          1. Contraindicated before Heart Rate is controlled (risk of reflex Tachycardia)
      5. Adjunctive measures
        1. Decreasing pain will decrease Blood Pressure
      6. Older agents that have largely been replaced
        1. Trimethaphan 1-4 mg/min IV
    3. Pain control
      1. IV Opioids
      2. Avoid IV NSAIDs
    4. Definitive Management
      1. Proximal Aortic Dissection (Type A)
        1. Mortality 1-2% per hour
        2. Emergent surgical management
      2. Distal Aortic Dissection (Type B)
        1. Initial medical management (including Blood Pressure control as above)
        2. Surgery will be needed in 20-33% of cases
          1. Acute renal artery Occlusion
          2. Superior Mesenteric Artery Occlusion
          3. Acute iliac Occlusion
    5. Hypotension
      1. Type and crossmatch and consider transfusion
      2. Consider Aortic Dissection-related causes (Bedside Ultrasound)
        1. Cardiac Tamponade
        2. Acute valvulopathy
        3. Myocardial Infarction with Cardiogenic Shock
        4. Ruptured aorta with Hemothorax
        5. Falsely depressed Blood Pressure (dissection causes decreased perfusion to the arm with BP cuff)
  14. Management: Surgical
    1. Indications for immediate, emergent surgical repair (even if neurologic deficits, coma, shock, advanced age)
      1. All Stanford Type A Aortic Dissection
      2. Aortic Dissection with Hypotension
      3. Complicated Stanford Type B Aortic Dissection
        1. Aortic Dissection with Acute Limb Ischemia, Mesenteric Ischemia, spinal ischemia
        2. Refractory hemodynamic instability
        3. Aortic Rupture
        4. Rapidly increasing aortic size
    2. Repair Types
      1. Open Repair
        1. Indicated in all Type A Aortic Dissections
        2. Indicated in Connective Tissue Disorders (Marfan Syndrome, Ehlers-Danlos Syndrome)
      2. Thoracic Endovascular Aortic Repair or TEVAR
        1. Indicated in Type B, descending Aortic Dissection repair
        2. Endovascular stent graft inserted over proximal intimal tear
        3. Redirects blood flow through the true lumen
    3. Efficacy
      1. Type A Dissection mortality reduced >30%
      2. Aortic root repair often preserve native aortic valve in acute Aortic Regurgitation
    4. Complications
      1. Open Repair
        1. Post-operative stroke: 15%
        2. Perioperative mortality: 25%
        3. Acute Renal Failure
        4. Mesenteric Ischemia
        5. Spinal cord ischemia
      2. Endovascular Repair (TEVAR)
        1. Overall Mortality: 6-8%
        2. Lower rates of spinal cord ischemia than open repair
  15. Prognosis
    1. High mortality: 27% even under ideal conditions
    2. Proximal Aortic Dissection (Type A)
      1. Mortality increases 1-3% per hour from onset (first 48 hours)
      2. Mortality with medical therapy: 50%
      3. Mortality with surgical management: 7-36%
    3. Distal Aortic Dissection (Type B)
      1. Mortality 10%
  16. References
    1. Mattu and Swaminathan in Herbert (2019) EM:Rap 19(1): 6-8
    2. Rooke (2017) Vascular Medicine, Mayo Clinical Reviews, Rochester, MN
    3. Dachs (2012) Board Review Express, San Jose
    4. Jhun, Grock and Weinstock in Herbert (2016) EM:Rap 16(11): 11-12
    5. Kostura (2019) Crit Dec Emerg Med 33(8):19-27
    6. Orman and Mattu in Herbert (2015) EM:Rap 15(8):2-3
    7. (2015) Ann Emerg Med 651(1): 32-42 [PubMed]
      1. http://www.acep.org/workarea/DownloadAsset.aspx?id=100814
    8. Bushnell (2005) Ann Emerg Med 46:90-92 [PubMed]
    9. Gupta (2009) Pharmaceuticals 2: 66-76 [PubMed]
    10. Hagan (2000) JAMA 283: 897-203 [PubMed]

Dissection of aorta (C0340643)

Definition (NCI) A progressive tear in the aorta characterized by a separation of the media layer from the outer-most layer.
Concepts Disease or Syndrome (T047)
ICD9 441.00
ICD10 I71.0, I71.00
SnomedCT 308546005
English Dissection of aorta [any part], Aortic Dissection, dissection of aorta, dissection of aorta (diagnosis), Dissection of aorta, Dissecting aortic aneurysm, unspecified site, Aortic dissection, Dsct of aorta unsp site, aortic dissection, Dissection of unspecified site of aorta, Dissection of aorta, unspecified site, dissection aorta, dissection aortic, aorta dissection, aorta dissections, aortic dissections, Dissecting aneurysm of aorta, unspecified site, Dissecting aneurysm of aorta, AORTIC DISSECTION, DISSECTION, AORTIC, Dissection of aorta (disorder), dissection; aorta, aorta; dissection
Italian Dissezione dell'aorta, Aneurisma dissecante dell'aorta, sede non specificata
Dutch aneurysma dissecans op niet-gespecificeerde plaats, aorta; dissectie, dissectie; aorta, Dissectie van aorta [elk deel], aorta dissecans
French Anévrysme aortique disséquant, site non précisé, Dissection aortique
German dissezierendes Aortenaneurysma, unspezifische Stelle, Dissektion der Aorta [jeder Abschnitt], Aortendissektion
Portuguese Aneurisma dissecante da aorta, local NE, Dissecção da aorta
Spanish Aneurisma disecante de aorta, localización no especificada, disección aórtica (trastorno), disección de aorta, disección aórtica, disección de aorta (trastorno), Disección aórtica
Japanese 大動脈解離, 解離性大動脈瘤、部位不明, カイリセイダイドウミャクリュウブイフメイ, ダイドウミャクカイリ
Czech Disekující aneurysma blíže neurčené části aorty, Aortální disekce
Korean 대동맥의 박리[모든 부분]
Hungarian Aorta dissectio, Aorta aneurysma repedés, nem meghatározott elhelyezkedésű
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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