Last edited: 10/25/2021
AORTIC DISSECTION
         Aortic Dissection                                       Medical Editor: Donya Moslemzadeh
                         OUTLINE                                               I) OVERVIEW
   I) OVERVIEW                                            Blood vessel Layers from the lumen outward:
   II) ETIOLOGY                                              o Tunica Intima (Interna)
   III) PATHOPHYSIOLOGY                                      o Tunica Media
   IV) CLINICAL FEATURES                                     o Tunica Externa (Adventitia)
   V) DIAGNOSIS
   VI) TREATMENT                                          What is an Aortic Dissection?
   VII) APPENDIX                                             o A tear through the Tunica Intima (Between Tunica
   VIII) REVIEW QUESTIONS                                      Intima and Tunica Media)
   IX) REFRENCES
                                                                  Most common site is Aorta
                                                             o Tear in the intima
                                   Figure 1. Artery Structure (AMBOSS).
                                    Figure 2 Aortic Dissection (AMBOSS)
Aortic Dissection                                      CARDIOVASCULAR PATHOLOGY: Note #6.                 1 of 8
                                                               Giant Cell Arthritis
                        II) ETIOLOGY
                                                                 =Temporal Arthritis
                 (A) ACQUIRED CAUSES                             Autoimmune Vasculitis
                                                                 chronic inflammatory disease involving large- and
(1) Hypertension                                                 medium-sized arteries
                                                                   o Most Common→ Involvement of the cranial
 Most Common cause
                                                                     branches of the carotid arteries
 High risk Hypertensive patients for Aortic Dissection are
 patients with:                                                  Age >50
  o Age > 40-60
                                                               Table 1. Takayasu vs Giant Cell Arthritis.
  o Tobacco Use
  o Hyperlipidemia                                                                    Takayasu              Giant Cell
                                                                                       Arthritis            Arthritis
 Secondary Hypertension
  o Transient elevation in blood pressure                       Age            <50                   >50
       Cocaine or Amphetamines Use                             Affected       Mostly Aorta and      Mostly Temporal
       High intensity weightlifting→ Heavy Valsalva            Vessels        its branches          Vessels
        maneuver → Brief rise of Blood Pressure
                                                                Clinical          Constitutional
 Mechanism:                                                     Features          symptoms:
  o ↑Sheer Forces on the vessel wall → Tear in the                                                     Constitutional
                                                                                  Weight loss          symptoms:
    Tunica Intima→ Blood tracks in between Tunica                                 Fever,               Weight loss
    Intima and Tunica Media Layer                                                 Fatigue.             Fever, Fatigue.
                                                                                  Absent or            Headache
   o Arteriosclerosis of the Vasa Vasorum                                         weak                 Ocular
       In HTN patients → Atherosclerosis of the Vasa                             Peripheral           Involvement
         Vasorum → ↓Blood Flow to the Tunica Media→                               Pulse                Jaw Claudication
         necrotic processes → Weak Vessel walls → ↑                               Discrepant
         Risk of having a tear → ↑ Risk of Dissection                             blood
                                                                                  pressure
                                                                                  between arms
   Blood Supply of the Blood Vessels
     Tunica Intima→ directly from blood in the lumen
     Tunica Media and Tunica Externa → Vasa Vasorum
   Vasa vasorum
      small blood vessels supply oxygen and nutrients to
     the outer layers of the arterial wall
(2) Trauma
  Motor Vehicle Collision
   o Secondary to Deceleration type of Injury
  Iatrogenic Injuries
   o Catheterization
   o Valve Repairs
(3) Vasculitis
 = Inflammation of the blood Vessel wall
 Triggers :
   o Tertiary Syphilis
        Inflammation of the Vasa vasorum caused by
          Treponema pallidum→↓Blood Supply to the
          arterial walls → Cystic Medial Degeneration of the
          Tunica Media → Weak wall →↑ Risk of Dissection
   o Takayasu Arthritis
       Inflammatory granulomas deposit in the vessel
         walls → Medial Degeneration of the Tunica Media
         → Weak wall →↑ Risk of Dissection
       Age < 50
       Usually, Aorta is Involved
       Less Facial and Ocular involvement compared to
         Giant cell. Arthritis
                                                                      Figure 3. Acquired Causes of Aortic Dissection
    2 of 8                CARDIOVASCULAR PATHOLOGY: Note #6.                                                   Aortic Dissection
                (B) CONGENITAL CAUSES                               (3) Coarctation of Aorta
 (1) Connective Tissue Diseases                                       A narrowing of the descending aorta (after aortic arch), at
                                                                      the level of Left Subclavian artery , near Ductus
   Defect in the connective tissues of the blood vessels              Arteriosus
   →Weak vessel wall → ↑ Risk of having a tear, ↑ Risk                Mechanism: Narrowing cause a lot of resistance →
   Aneurysm → ↑ Risk of Dissection                                    ↑Blood pressure proximal to the affected area → HTN →
                                                                      →↑ Risk Aortic Dissection , ↑ Risk Aneurysm
   Connective tissues of the blood vessels Figure 1                   Patients with Coarctation of Aorta probably have
    o Subendothelial Layer                                              o History of Turner Syndrome
    o Elastic Laminas                                                   o Associated Bicuspid aortic Valve
    o Tunica Externa
   Aneurysm
    True Aneurysm
      o abnormal dilation of an artery due to a weakened
        vessel wall, ≥50% dilation of all 3 layers of aorta
     False Aneurysm
      o external hematomas with a persistent
         communication to a leaking artery, rupture within
         adventitia
   Aneurysm → ↑ Risk of Dissection
   Dissection → ↑Risk of Aneurysm
 Marfan Syndrome
   Etiology
    o Mutation of Fibrillin Gene → ↓Elasticity of the vessel
       walls→ ↑ Risk Aneurysm and ↑ Risk of Dissection
   Clinical Features
    o Cardiovascular Disorders: mitral valve prolapse, aortic
       aneurysm, and dissection
    o Musculoskeletal Disorders: tall stature with
       disproportionately long extremities, joint hypermobility
    o Eyes Disorders: subluxation of the lens of the eye
   Fibrillin= A glycoprotein that forms a supportive sheath
     around Elastin
 Ehlers-Danlos Syndrome(EDS)
   Etiology
    o Mutation of Collagen Gene→ ↓Resilience of the blood
       vessels →↑ Risk Aneurysm and ↑ Risk of Dissection
   Clinical Features
    o Cardiovascular Disorders: mitral valve prolapse, aortic
       aneurysm, and dissection
    o Musculoskeletal Disorders: Joint Hypermobility
    o Skin Disorders : Skin hyperextensibility, Easy
       Bleeding , Tendency to bruise easily
 (2) Bicuspid Aortic Valve
   One of the most common types of congenital heart
   disease
   Genetic Disorder→ 2 valve cusps fuse together →
   Bicuspid Aortic Valve
   ↑ Risk of
     o Aortic Stenosis (Most Common)
     o Aortic Regurgitation
     o Hypertension
     o Aortic Aneurysms (unknown Mechanism)
     o Aortic Dissections( unknown Mechanism)
   (Potential) Mechanism                                                   Figure 4. Congenital Causes of Aortic Dissection
     o Bicuspid aortic Valve → Left Ventricle Hypertrophy →
       HTN → Work harder to push blood out→↑ Risk Aortic
       Dissection , ↑ Risk Aneurysm
Aortic Dissection                                                 CARDIOVASCULAR PATHOLOGY: Note #6.                   3 of 8
                         III) PATHOPHYSIOLOGY                                                 Narrowing / Occlusion of a branching vessel
                                                                                           (due to forming Hematoma) → Malperfusion
      Tear in the aortic intima → blood enters the media of the                            Syndromes
        aorta and forms a false lumen in the intima-media                                     Intramural (Intraluminal) Thrombosis →
        space→ 4 Possible Scenarios:                                                       Forming hematoma at the tear site
                 Reentry of the blood into the True lumen
                 ↑ pressure within the aortic wall → Rupture
                                                        Figure 5. Pathophysiology of Aortic Dissection
                                                                IV) CLINICAL FEATURES
      Table 2. Clinical Features of Aortic Dissection
   Affected Structure                  Mechanism                                                Clinical Features
                              Blood moves between the              New Aortic Regurgitation murmur
Aortic Valve                  intima and media into the
                              valve leaflet
                              Malperfusion of Coronary               Acute Coronary Syndrome
Coronary Arteries
                              arteries (mostly right)                MI (STEMI, NSTEMI)
                              Coronary arteries rupture              Cardiac Tamponade
                              into the pericardium
Pericardium                                                        (Classic signs of Beck's triad: low blood pressure, distension of the
                                                                   jugular veins and decreased or muffled heart sounds)
Brachiocephalic,                                                     Syncope
                              Occlusion →↓ blood flow to
common carotid,                                                      Cerebral Vascular incidents (Stroke)
                              the brain
or left subclavian arteries
                                                                     Upper extremity pulselessness
                                                                     ↓ BP on the Left upper Extremities (A considerable variation (>20
                                                                     mmHg) in systolic blood pressure may be seen when comparing the
                              Occlusion of the Left                  blood pressure in the arms.)
                              Subclavian Artery and no               Acute limb Ischemia
Left Subclavian Artery
                              occlusion of the Right                   o Pulselessness
                              Subclavian Artery                        o Pain
                                                                       o Losing color (Pallor)
                                                                       o Weak muscle → Paralysis
                                                                       o Cold (Poikilothermia)
                                                                     ↓Urine output
                              Dynamic Obstruction and                Acute kidney Injury
Renal artery                  Malperfusion of the Renal               o ↑Creatinine
                              Artery                                  o ↑BUN
                                                                      o ↓GFR
                              Occlusion                              Anterior Spinal Artery Syndrome
                                                                      o Paraplegia of lower extremities
Artery of Adamkiewicz                                                 o Loss of Sensations
                                                                      o Incontinence
                              Dynamic Obstruction                    Acute Mesenteric Ischemia
Celiac or mesenteric                                                 Abdominal pain
arteries                                                             Bloody Diarrhea
                                                                     Nausea and vomiting
                                                                     Pulselessness of LLE
                                                                     ↓BP compared to RLE
                              If Occlusion of the Left               Weakness
                              Common Iliac artery                    Acute Limb Ischemia
Common iliac artery                                                   o Pulselessness
                              →↓Blood flow to the Left
                              Lower Extremities LLE                   o Pain
                                                                      o Losing color (Pallor)
                                                                      o Weak muscle →Paralysis
                                                                      o Cold (Poikilothermia
           4 of 8                 CARDIOVASCULAR PATHOLOGY: Note #6.                                                             Aortic Dissection
                                                                          (2) EKG
                        V) DIAGNOSIS                                          o Indication:
 (1) Aortic Involvement                                                            Initial evaluation of patients with chest pain
                                                                                   Rule Out → Acute MI
   Tearing Ripping pain →                                                             • less helpful in dissection leads to coronary
    o Anterior chest pain →Dissections involving the                                      ischemia
      Ascending Aorta                                                         o Findings may include:
    o Neck and Jaw Pain → Dissections involving the                                Normal
      Aortic Arch and its branches→ e.g., Carotid arteries                         ST Elevation (Coronary Artery Occlusion)
    o Interscapular Pain → Dissections involving the                               ST Depression
      Descending Aorta (Beyond Left Subclavian artery)                        o Troponin may be POSITIVE
   Most Common Location for Aortic Dissection =
   Ascending Aorta (2.2cm of Aortic Valve)
                                                                             Figure 7 ST-Elevation [from the 12 leads of EKG lecture]
          Figure 6. Aortic Involved site Associated Pain
                                                           Figure 8. EKG of STEMI
 (3) Chest Xray
                                                                          (4) TEE, Trans Esophageal Echocardiography
   Advantage → Rapid
                                                                            TEE
   To Rule Out
                                                                            Can be Performed at the bedside
    o Pneumothorax
                                                                            Indications:
    o pleural effusion
                                                                              o Hemodynamically unstable patients
    o Pneumonia
                                                                              o Severely low GFR
   Finding:                                                                        Contrast Induced Nephropathy
     o Mediastinal widening→ ≥ 8cm → Suspicious for
                                                                            Rule out → Cardiac Tamponade
       Aortic Dissection (Suspicious not Diagnostic!)
                                                                            Findings:
                                                                              o Intimal Flap
                                                                              o False Lumen
        Figure 9. Widened Mediastinum on a Chest Xray
                                                                                         Figure 10. Aortic Dissection TEE.
Aortic Dissection                                                      CARDIOVASCULAR PATHOLOGY: Note #6.                    5 of 8
(5) CTA; CT Angiography                                            (6) MRA; Magnetic Resonance Angiography
 CTA → GOLD STANDARD                                                 Time Consuming
 Indications:                                                        Indications:
   o Hemodynamically Stable patients                                   o Hemodynamically Stable patients
   o Surgical Planning                                                 o Contradiction to CTA
 Findings:                                                           Findings:
   o Intimal Flap                                                      o Similar to CTA
   o False lumen                                                   Table 3. Imaging Techniques Comparison
   o Leak / rupture                                                   Imaging
                                                                                          Advantages
   o Aortic Hematoma                                                 Modalities                              Disadvantages
                                                                                                              Poor
                                                                     CXR            Rapid, Bedside            Sensitivity
                                                                                    Rapid, Bedside            Invasive
                                                                                    No radiation exposure     May not be
                                                                     TEE                                      readily
                                                                                    Sensitive and Specific
                                                                                                              available
                                                                                                              Cannot be
                                                                                                              performed at
                                                                                    Gold standard: Very       the bedside
                                                                     CTA            high sensitivity and      Radiation
                                                                                    specificity               exposure
                                                                                    Operative Planning        Contrast
              Figure 11. Aortic Dissection CTA.
                                                                                                              exposure
                                                                                                              Cannot be
                                                                                    Very high sensitivity     performed at
                                                                     MRA            and specificity           the bedside
                                                                                    No radiation exposure     Time
                                                                                                              Consuming
                                                          VI) TREATMENT
Type of Dissection Determination
                                                                                  (B) MEDICAL TREATMENT
 Aortic dissection classification systems
  o The DeBakey System                                             (1) Hypotensive Patients
  o The Stanford System (Preferred)                                  Goal → Mean Arterial Pressure ≥70mmhg
 Stanford Classification                                             IV Fluid
  o Stanford A                                                         o IV Fluid →↑ Blood Volume →↑Blood Pressure
       Involves Ascending Aorta                                     Vasopressor
         Emergency → Surgical Therapy                                 o →↑SVR →↑Blood Pressure
  o Stanford B                                                        o Norepinephrine
       Beyond Left Subclavian Artery → Involves                      o Phenylephrine (Pure Alpha-1 vasoconstrictor)
         Descending Aorta
       Medical Therapy BUT you may need Surgical                    Blood Transfusion
         Intervention                                                  o →↑ Blood Volume →↑Blood Pressure
       Complications → may need Surgery                             Evaluate 3 Scenarios and fix them
           • Medical Treatment Failure                                o Rupture→ repair the rupture
           • Rupture                                                  o Cardiac Tamponade → Pericardial Fluid Drainage
           • Propagation of dissection                                o Severe Aortic insufficiency → Valve Repair
           • Expanding intramural Hematoma
                                                                   (2) Hypertensive Patients
              (A) SURGICAL TREATMENT                                 Goal:
   Open Surgery → replacement of the dissection with a                o Systolic Blood Pressure 100-120mmhg
polyester graft implantation                                          o Heart Rate < 60bpm
    a. Type A Dissections
   Endovascular Stent Placement                                       Beta Blockers (Alpha and Beta blocker activity)
    a. Type B Dissections                                             o Esmolol
                                                                      o Labetalol
                                                                      o Mechanism:
                                                                           Blocking Beta receptors on AV/SA nodes→ ↓Heart
                                                                            Rate
                                                                           Blocking Alpha-1 → Vasodilation → ↓SVR → ↓BP
                                                                      o Beta Blockers are given first to prevent Reflex
                                                                        Tachycardia
                                                                      Vasodilators
                                                                      o Vasodilators → Low Blood Pressure → Stimulate
                                                                        Cardiac Acceleratory Center in the brain →↑Heart
                                                                        rate and contractility (Reflex tachycardia)
       Figure 12. Aortic Dissection Surgical Treatment.               o Sodium Nitroprusside
                                                                           Vasodilation → ↓SVR → ↓BP
    6 of 8                CARDIOVASCULAR PATHOLOGY: Note #6.                                                  Aortic Dissection
                                    Figure 13. Aortic Dissection Medical Treatment.
                                                   VII) APPENDIX
                    Figure 14. Aortic Dissection Etiology, Pathophysiology, Diagnosis and treatment.
Aortic Dissection                                             CARDIOVASCULAR PATHOLOGY: Note #6.       7 of 8
                VIII) REVIEW QUESTIONS
1) Best Diagnostic test in Hemodynamically stable
  patients with suspected aortic dissection:
    a) ECG
    b) CTA
    c) CXR
    d) TEE
2) Best Diagnostic test in Hemodynamically unstable
  patients with suspected aortic dissection:
    a) ECG
    b) CTA
    c) MRA
    d) TEE
3) Which Statement is True?
    a) Type B dissections involves the ascending aorta.
    b) Type A dissections can be treated medically.
    c) Type A dissections are surgical emergencies, Type
       B are still emergencies but can often be treated
       medically.
    d) All patients with Type B dissections should have
       surgery.
4) Which one is not a High-Risk condition associate
  with Aortic Dissection?
    a) Athletic Lifestyle
    b) Cocaine Use
    c) Hypertension
    d) Preexisting Aortic Aneurysm
5) Acute Mesenteric Ischemia may happen as a result
  of
     a) Dynamic Obstruction of common Iliac Artery
     b) Dynamic Obstruction of Celiac and mesenteric
        arteries
     c) Dynamic Obstruction of Anterior Spinal Artery
     d) Dynamic Obstruction of Renal Artery
6) Treatment of Type B Aortic Dissection in a patient
  with Hypertension:
     a) Sodium Nitroprusside → Esmolol
     b) Polyester Graft Implantation → Norepinephrine
     c) Labetalol → Sodium Nitroprusside
     d) IV Fluids → Phenylephrine
                      IX) REFRENCES
     ● AMBOSS: medical knowledge platform for doctors and students.
   (n.d.). Amboss. Retrieved 2021, from https://www.amboss.com/us/
     ● UpToDate: Evidence-based Clinical Decision Support. (n.d.).
   UpToDate.Com. Retrieved 2021, from
   https://www.wolterskluwer.com/en/solutions/uptodate
     ● Le, T., Bhushan, V., & Sochat, M. (2021). First Aid for the
   USMLE Step 1 2021, Thirty First Edition (31st ed.). McGraw-Hill
   Education / Medical.
     ● Gabriel, D. (2019). USMLE Step 2 CK: A Student-to-student
   Guide (Clinical Knowledge) (10th ed.). Independently published.
     ● Papadakis, M., McPhee, S., & Rabow, M. (2019). CURRENT
   Medical Diagnosis and Treatment 2020 (59th ed.). McGraw-Hill
   Education / Medical.
    8 of 8                  CARDIOVASCULAR PATHOLOGY: Note #6.        Aortic Dissection