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Sudden Non Traumatic CV Death Case Case Case Case Case

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					                                                                 Case 3
         CV Pathology Specialty Conference         Sudden Non-Traumatic CV Death
               USCAP, Denver, CO
                   April 4, 2008                • The circumstances
                                                  59F in Rochester, MN, with husband (p/o)
                                                  09:30 pm: last seen alive by her family
  Sudden Non-Traumatic CV Death                   12:30 am: found dead in bed by daughter
             Case 3                             • The co-morbidities
                                                  Obesity, polycythemia vera, poliomyelitis
                                                  Recent nausea, exhaustion, foot swelling
             William D. Edwards, M.D.           • The county coroner
          Dept of Lab Medicine & Pathology        Scene: no accident, suicide, or homicide
            Mayo Clinic, Rochester, MN            Autopsy: no pulm emboli; neg toxicology




                      Case 3                                     Case 3
     Sudden Non-Traumatic CV Death                 Sudden Non-Traumatic CV Death



Ht wt:
 480 g
Exp:
 265 g




         Heart, RVH & Dilatation (Short-Axis)          Heart, RV Hypertrophy (H&E)




                      Case 3                                     Case 3
     Sudden Non-Traumatic CV Death                 Sudden Non-Traumatic CV Death




          Lung, Medial Hypertrophy (VVG)               Lung, Intimal Fibrosis (H&E)




                                                                                              1
                Case 3                                         Case 3
  Sudden Non-Traumatic CV Death                 Sudden Non-Traumatic CV Death




   Lung, Recanalized Thrombi (H&E)              Lung, Fresh/Organizing Thrombi (H&E)




                Case 3                                         Case 3
  Sudden Non-Traumatic CV Death                 Sudden Non-Traumatic CV Death

               Diagnoses                                  Death Certificate
Sudden death, presumably arrhythmic                    Sudden arrhythmic death
Pulmonary heart disease (cor pulmonale)
                                                       Chronic cor pulmonale
Hypertensive pulmonary vascular disease,
secondary arterial thrombotic-fibrotic type            Pulmonary hypertension

History of long-standing polycythemia vera             Polycythemia vera




                 Case 3                                        Case 3
           Discussion Outline                 Overview of Pulmonary Hypertension
                                              • Definition
         • Overview of Pulm Htn
                                                Pressure: ↑ pulm art ± ↑ pulm venous
         • Cardiac Features                     Pulm art: >30/15 mm Hg (mean >25)
                                                Diagnosis: clinical (hemodynamic), but
         • Pulmonary Features
                                                           also can be by pathologist
         • Microscopic Features               • Categorization
         • Sudden Death                         Location: arterial or post-arterial type
                                                Duration: acute, subacute, or chronic
         • Summary of Topic
                                                Etiology:    primary or secondary type




                                                                                           2
               Case 3                                Case 3
        Discussion Outline                   Cardiac Features

     • Overview of Pulm Htn
                                     RA
     • Cardiac Features
                                                              RA

     • Pulmonary Features                       RV
                                                                      RV
     • Microscopic Features
                                                                            LV
     • Sudden Death
     • Summary of Topic                 Pulm Htn              Normal
                                      RVH & Dilatation (Anterior View)




               Case 3                                Case 3
        Cardiac Features                     Cardiac Features
                                                                   Regurgitation



                        RV
   RV
          LV
                               LV



                                                                Pulmonary
   Pulm Htn               Normal      Tricuspid Valve              Valve
RVH & Dilatation (Short-Axis View)   Annular Dilatation (Short-Axis View)




               Case 3                                Case 3
        Cardiac Features                     Cardiac Features

                                          • Right ventricle
                                            Hypertrophy & dilatation
                                            Straightened septum
                                            D-shaped ventricles
                        LA
          RA                              • Right-Sided Valves
                                            Pulmonary dilatation
                                            Tricuspid dilatation
                                          • Right atrium
                                            Dilatation & hypertrophy
RA Dilatation (L Ant Oblique View)




                                                                                   3
              Case 3                                      Case 3
        Discussion Outline                      Pulmonary Features

       • Overview of Pulm Htn
       • Cardiac Features
       • Pulmonary Features
       • Microscopic Features
       • Sudden Death
       • Summary of Topic                Pulm Htn (CXR)        Normal (CXR)
                                             Dilated Main Pulmonary Artery




                                                          Case 3
              Case 3                            Pulmonary Features
       Pulmonary Features

                            Ao
                                 PA
                                                Main
                       RA                     Pulmonary              Ascending
                                                Artery                 Aorta

                                 RV




Pulm Htn (CXR)      Pulm Htn (Autopsy)
    Dilated Main Pulmonary Artery           Dilated Main Pulmonary Artery




              Case 3                                      Case 3
       Pulmonary Features                       Pulmonary Features

  Bronchus




                   Artery




Dilated Elastic Pulmonary Artery (VVG)     Atherosclerosis of Elastic Arteries




                                                                                 4
                       Case 3                                             Case 3
              Pulmonary Features                                Discussion Outline

              • Elastic arteries                               • Overview of Pulm Htn
                Dilatation (stretch)
                Medial hypertrophy                             • Cardiac Features
                Atherosclerosis                                • Pulmonary Features
              • Muscular arteries
                Medial hypertrophy                             • Microscopic Features
                Intimal proliferation                          • Sudden Death
              • Arterioles
                Muscularization                                • Summary of Topic




                       Case 3                                             Case 3
             Microscopic Features                              Microscopic Features
                                                                  Normal Artery
                                                                  and Arteriole


 <10%        Normal               Mild           10-20%




20-25%      Moderate              Sev            >25%



            Medial Hypertrophy (VVG)                      Arterial Medial Lesions (HE, VVG)




                       Case 3                                             Case 3
             Microscopic Features                              Microscopic Features




                                         Glomerulus

         Arterial Intimal Lesions (HE, VVG)               Arterial Luminal Lesions (HE, VVG)




                                                                                               5
                   Case 3                                         Case 3
           Microscopic Features                            Microscopic Features
• Arterial medial lesions
  Hypertrophy of muscular & elastic arteries
  Muscularization of arterioles (intra-acinar)
  Necrotizing arteritis and micro-aneurysms
• Arterial intimal lesions
  Proliferation, fibrosis (eccentric, concentric)
  Plexiform lesion (vs organizing thrombus)
• Arterial luminal lesions
  Thrombus, embolus, fibrous webs or plugs
                                                           Venous Lesions (VVG)




                   Case 3                                         Case 3
           Microscopic Features                            Microscopic Features
• Venous medial lesions
  Medial hypertrophy of veins and venules
  Arterialization of veins (ext elastic memb)
  Active phlebitis (may be granulomatous)
• Venous intimal lesions
  Proliferation, fibrosis (eccentric, concentric)
                                                                         V
• Venous luminal lesions
                                                                          Lymphatic     V
  Thrombus (organized and recanalized)
  Fibrous webs or occlusive luminal plugs
                                                    Capillary, Alveolar, and Septal Lesions




                   Case 3                                         Case 3
           Microscopic Features                             Discussion Outline
• Capillary lesions
                                                           • Overview of Pulm Htn
  Engorgement and dilatation (congestion)
  Proliferation (pulm cap hemangiomatosis)                 • Cardiac Features
• Lymphatic lesions                                        • Pulmonary Features
   Dilatation (in pleura and interlobular septa)
                                                           • Microscopic Features
• Other lesions
  Alveolar edema and micro-hemorrhages                     • Sudden Death
  Alveolar siderophages (rbc phagocytosis)
  Pleural and septal edema (Kerley B lines)                • Summary of Topic




                                                                                              6
           Case 3                            Case 3
        Sudden Death                      Sudden Death
 • Causes of death        %
   Progressive R-HF       50
   Sudden death           25
   Other causes           25
 • Patterns with SD       %
   Plexogenic             50
   Thrombotic, fibrotic   30
   Veno-occlusive         10
   Other (not PVH)        10
                                     Plexogenic Type (HE, VVG)




           Case 3                            Case 3
        Sudden Death                      Sudden Death

                                 Venule
                                                            Venule




                                                            Septum




Thrombotic/Fibrotic Type (VVG)       Veno-Occlusive Type (VVG)




           Case 3                            Case 3
     Discussion Outline                      Summary

   • Overview of Pulm Htn        • Pulmonary hypertension
                                   Clinical hemodynamic diagnosis
   • Cardiac Features
                                   May be diagnosed at autopsy,
   • Pulmonary Features              by cardiopulmonary lesions
   • Microscopic Features        • Cardiac features
   • Sudden Death                  RV hypertrophy and dilatation
                                   Straight VS, with D-shaped LV
   • Summary of Topic
                                   RA, TV, and PV dilatation




                                                                     7
             Case 3
            Summary                      CV Pathology Specialty Conference
                                               USCAP, Denver, CO
                                                   April 4, 2008
• Pulmonary features
  Medial hypertrophy of arteries
  Obstructive vascular lesions
                                                  Case 3
  Specific combinations of lesions    Sudden Death as First Manifestation
                                         of Pulmonary Hypertension
• Sudden death
  May be the first manifestation             William D. Edwards, M.D.
  Plexogenic or thrombotic/fibrotic       Dept of Lab Medicine & Pathology
  Not pulm venous hypertension              Mayo Clinic, Rochester, MN




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