Pathology of Congenital Heart Disease

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					            Congenital Heart Disease:
            Challenges and Opportunities
            for Pathologists    USCAP
            March 7, 2009    Boston, MA


 Postoperative Pathology of
 Congenital Heart Disease
(No Disclosures; No Conflicts of Interest)

        William D. Edwards, M.D.
     Dept of Lab Medicine & Pathology
     Mayo Clinic, Rochester, MN, USA
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
  Introduction to Post-Op CHD
    Scenarios at Time of Autopsy
• Previous complete repair of CHD
   PDA ligation, ASD or VSD closure
• Previous and future interventions
   Complex congenital heart disease
• Inoperable (except transplantation)
   Irrev pulm htn or severe heart failure
• Inoperable (unrelated to CHD)
   Cancer or other end-stage disease
• Source of specimens with CHD
   Autopsy, surgical, & rarely biopsy
  Introduction to Post-Op CHD
     Extracardiac Complications
                 (20F)                       (20F)


                         R Lung            L Lung




                                   Heart

        Chest
        Plate


Sternal Osteomyelitis        Mediastinitis

    Post-Operative Wound Infection
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
     Closure of Congenital Shunts
                  Types of Anomalies


        LA                 LA                 LA
                                                             Ao
RA                  RA                 RA
             LV                  LV
                                                   LV       PA
      RV                  RV
                                            RV



   Atrial            Ventricular      Atrioventricular     Patent
Septal Defect       Septal Defect     Septal Defect      Ductal Artery
     (ASD)               (VSD)           (AVSD)             (PDA)

      Diagram of Four Common Shunts
     Closure of Congenital Shunts
                  Types of Anomalies


        LA                 LA                 LA
                                                             Ao
RA                  RA                 RA
             LV                  LV
                                                   LV       PA
      RV                  RV
                                            RV



   Atrial            Ventricular      Atrioventricular     Patent
Septal Defect       Septal Defect     Septal Defect      Ductal Artery
     (ASD)               (VSD)           (AVSD)             (PDA)

      Diagram of Four Common Shunts
   Closure of Congenital Shunts
           Atrial Septal Defect
• Methods of closure
                                        LA
  Surgical: patch or suture
                                  RA
  Cath: clamshell occluder                   LV

• Complications (uncommon)             RV

  RA arrhythmia, mural thr
   & emb, pulm htn, RV dil      Patch Closure

• Post-operative mortality (<1% overall)
  Early: pulm htn, coexistent heart disease
  Late: heart failure, arrhythmia, or stroke
Closure of Congenital Shunts
             Atrial Septal Defect
                                  Isolated ASD
     SVC                          with Pulm Htn



       Limbus               RVH
        ASD

             Valve
                            RV


       IVC

Autopsy Specimen (40F)

     Pathology of Secundum ASD
Closure of Congenital Shunts
             Atrial Septal Defect
                                        Surgical
     SVC                               Specimen
                                         (36F)


       Limbus

        ASD                 Valve of
                             Fossa
             Valve           Ovalis




       IVC

Autopsy Specimen (40F)

     Pathology of Secundum ASD
Closure of Congenital Shunts
       Atrial Septal Defect
                  (19mF)




   Thrombus




   RA View                 LA View

 Patch Closure of ASD (Autopsy)
Closure of Congenital Shunts
     Atrial Septal Defect
                (4F)


             Left Atrium




                  Thrombus



        MV



Thrombus on ASD Patch (Autopsy)
Closure of Congenital Shunts
         Atrial Septal Defect
                                    Secundum
                                   ASD Closure
                    LA                (57M)



               RA

                              LV



                         RV
                                         Four
  Persistent                           Chamber
  RVH & Dil                             View

 Post-Op Fatal Acute MI (Autopsy)
Closure of Congenital Shunts
           Atrial Septal Defect
 Atrial
Septum
          Catheter
 ASD

   LA        RA




   ASD Occluder Device (Diagram)
   Closure of Congenital Shunts
            Atrial Septal Defect
(65M)                                    (65M)




        ASD Occluder Device (Surgical)
   Closure of Congenital Shunts
            Atrial Septal Defect
(65M)                       At Autopsy, Assess
                            Injury by ASD/PFO
                            Occluder Device to:
                                    Aortic Valve,
                                      Asc Aorta,
                                       and SVC




        ASD Occluder Device (Autopsy)
   Closure of Congenital Shunts
       Ventricular Septal Defect
• Methods of closure
  Surgical: patch or suture            LA

                                 RA
  Cath: clamshell device                    LV

• Complications (uncommon)            RV


  Residual VSD, conduction
   injury, arrhythmia, AI, TR   Patch Closure

• Post-op mortality rate (<1% in infants)
  Early: acute heart failure (usually LV)
  Late: sudden (arrhythmic or pulm htn)
Closure of Congenital Shunts
   Ventricular Septal Defect
                               Persistent
                             Truncal Artery
                   RV-PA         (4mF)
                   Conduit



                Patch



           TV           RV




 Residual Post-Op VSD (Autopsy)
Closure of Congenital Shunts
     Ventricular Septal Defect
                      Ao




                           MV



                 LV




Persistent
Truncal Artery

 Residual Post-Op VSD (Autopsy)
Closure of Congenital Shunts
    Ventricular Septal Defect
          Isolated VSD (11M)




      Cusp Trauma by Patch Suture

Sev AI, Post-VSD Closure (Surgical)
   Closure of Congenital Shunts
     Atrioventricular Septal Defect
• Methods of repair
  Patch closure of AVSD              LA

                               RA
  Repair of MV component
                                          LV
• Complications                     RV

  MR/MS, subao stenosis,
   arrhythmias, & pulm htn
• Mortality (<3% partial, <5% complete)
  Risk: sev pre-op symptoms, sev regurg
  Early: acute HF (esp in ToF & Down’s)
 Closure of Congenital Shunts
  Atrioventricular Septal Defect




        RA   IAS     LA




             AVS


        RV   IVS     LV

Simulated AVSD (Four-Chamber View)
 Closure of Congenital Shunts
  Atrioventricular Septal Defect




        RA           LA




        RV           LV

Simulated AVSD (Four-Chamber View)
Closure of Congenital Shunts
  Atrioventricular Septal Defect
              Classic Partial AVSD

                                           LA
     RA
                                      D
FO        D
                   RV
                             LV




 Primum ASD                Cleft MV with MR
(Anterior to FO)           (Anterior Leaflet)
Autopsy Heart (Opened R & L Sides)
  Closure of Congenital Shunts
     Atrioventricular Septal Defect
             Complete AVSD

                                   Ao


                                        LA
RA     D                       D
                RV
                         LV



Large Septal Defect    Elongated LVOT
  (Two-Chamber)          (Long-Axis)
 Autopsy Heart (Free Walls Removed)
Closure of Congenital Shunts
  Atrioventricular Septal Defect
          Complete AVSD

                                Ao
           Ao

                     +25%            LA
                LA          D
  LV                  LV



 Normal Heart        Elongated LVOT
  (Long-Axis)          (Long-Axis)
Autopsy Heart (Free Walls Removed)
Closure of Congenital Shunts
  Atrioventricular Septal Defect
     Repair of Complete AVSD
                                AVSD
    RVOT                  LA     (2M)

                     RA
           LVOT

       CAVV                    LV

                          RV




Simulated Repair       Patch Repair
(Short-Axis View)   (4-Chamber View)
 Patch Closure of Defect (Autopsy)
   Closure of Congenital Shunts
           Patent Ductal Artery
• Methods of closure
  Premature: vascular clip          Ao
   Non-surg: indomethacin
  Others: ligation or division     PA

• Complications (uncommon)
  Nerve injury, residual shunt,
   aorto-bronchial fistula, false aneurysm
• Death (<1% pre, ~0% infant, <2% adult)
  Related to cardiomegaly or pulm htn
Closure of Congenital Shunts
     Patent Ductal Artery




       Neointima        Thrombus




Stenosis of Blalock-Taussig Shunt
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
     Relief of Obstructive Lesions
                 Types of Anomalies

           Ao                   Ao                  Ao

           PA                   PA        RA        PA
RA                    RA



                LV                   LV        RV        LV
      RV                   RV




PV Stenosis          AV Stenosis          Coarctation

Diagram of Three Common Obstructions
  Relief of Obstructive Lesions
     Pulmonary Valve Stenosis
• Relief of obstruction                Ao
  Surgical valvuloplasty     RA
                                       PA

  Balloon valvuloplasty
                                            LV
• Critical PS in neonates         RV


  Death: 10% early & late
  Cause: acute LV failure
• Non-critical PS
  Death (balloon): near 0%
  Death (surgery): near 0%
  Relief of Obstructive Lesions
        Aortic Valve Stenosis
• Relief of obstruction                   Ao
  Surgical valvuloplasty        RA
                                          PA

  Balloon valvuloplasty
                                               LV
• Post-op mortality rate             RV


  15% (esp hypoplastic LV)
  Cause: acute LV failure
• Post-balloon mortality rate
  15% (esp hypoplastic AV)
  Cause: cusp tears with AI
Relief of Obstructive Lesions
      Aortic Valve Stenosis
             Acute Myocardial
                Infarction
                  (8mF)


 RV                       RV
        LV                      LV




RCA Compression by Aortotomy (Aut)
  Relief of Obstructive Lesions
         Coarctation of Aorta
• Relief of obstruction                  Ao
  Surg: LSA flap; resection    RA        PA
  Balloon: re-coarctation
                                              LV
• Complications                     RV


  Htn, re-coarc, chylothorax
• Post-op mortality rate
  5% infants, 1% in others
  Early: acute LV failure
  Late: ao aneur, diss, rupt
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
 Ventriculo-Pulmonary Conduits
                  Types of Anomalies

            Ao
                                  Ao

                                  PA                 Ao
 RA                                            RA         PA
             LA         RA



                 LV                    LV                      LV
       RV                    RV                     RV




Tetralogy of Fallot    Persistent Truncal    Transposition of the
  (also PA-VSD               Artery             Great Arteries
   and DORV)          (Truncus Arteriosus)        with VSD


Diagram of Three Conotruncal Anomalies
   Ventriculo-Pulmonary Conduits
   Conotruncal Anomalies with VSD
• Complications
  Conduit stenosis (valve),             Ao


    recur VSD, arrhythmia       RA
                                       PA


  Late: RV fibrosis & failure,
    asc ao dilatation with AI        RV
                                           LV


• Postoperative mortality
  5% in tetralogy of Fallot
  10% in PA-VSD, DORV, and TGA-VSD
  15% in persistent truncal artery (truncus)
  Cause of death: acute ventricular failure
   Ventriculo-Pulmonary Conduits
    Conotruncal Anomalies with VSD
• Complications                Rastelli
                               Repair
  Conduit stenosis (valve),                Ao


    recur VSD, arrhythmia        RA
                                          PA


  Late: RV fibrosis & failure,
    asc ao dilatation with AI           RV
                                              LV


• Postoperative mortality
  5% in tetralogy of Fallot
  10% in PA-VSD, DORV, and TGA-VSD
  15% in persistent truncal artery (truncus)
  Cause of death: acute ventricular failure
Ventriculo-Pulmonary Conduits
               Surgical Repair
          Persistent Truncal   Bioprosthetic
                 Artery (3F)   Valve
 Ao                                                  LPA
                                      Conduit

              Conduit
                                                    LA
RA
                                RV
                                           LV
             RV



      Anterior View                  Left Lateral View
         Conduit Repair (Autopsy)
Ventriculo-Pulmonary Conduits
Synthetic and Homograft Conduits
       Valve
                         PA
                        Anast

      Conduit

                       Eggshell
                      Calcification

        RV




  Synthetic (Aut) Homograft (Surg)
Ventriculo-Pulmonary Conduits
   Valvular Conduit Stenosis
                           (28M)




                         Radiograph
                          of Valve
Massive Valvular Calcification (Surg)
Ventriculo-Pulmonary Conduits
   Proximal Conduit Stenosis




Extrinsic Sternal Compression (Surg)
Ventriculo-Pulmonary Conduits
Proximal Conduit Anastomotic Leak
     PA-VSD


                     False
    Conduit        Aneurysm




              RV
                     LV




  Peri-Conduit False Aneurysm (Aut)
Ventriculo-Pulmonary Conduits
Distal Conduit Anastomotic Leak
    PA-VSD
     (2F)
                    False
                  Aneurysm




             RV        LV




 Peri-Conduit False Aneurysm (Aut)
Ventriculo-Pulmonary Conduits
  Collateral Arteries in PA-VSD
               PA-VSD


RUL
               Ao                 LUL
                          LPA
                    RPA

RML


                     MPA
                                  LLL
RLL




Complex Collateral Arteries (Autopsy)
           Ventriculo-Pulmonary Conduits
           RVOT Reconstruction in Tetralogy

 Endo-                                           Myocyte
cardial                                          Hyper-
Fibrosis                                          trophy




 Myo-
                                                 Myocyte
cardial
                                                 Disarray
Fibrosis




             Infundibular Resection (Surgical)
Ventriculo-Pulmonary Conduits
  Late Aortic Valve Regurgitation
                 PA-VSD
                  (17M)




AVR 12 Years After Conduit Repair of DORV

AV Dilatation due to Aortopathy (Surg)
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
      Arterial Switch Procedure
             Types of Anomalies


       Ao
 RA         PA
                      Ao   PA



      RV         LV
                                     LV

                                RV

Transposition
With Intact VS
 TGA and DORV with Subpulm VSD
      Arterial Switch Procedure
             Types of Anomalies
                                     TGA or
                                     DORV?
       Ao
 RA         PA
                      Ao   PA



      RV         LV
                                     LV

                                RV

Transposition
  With VSD
 TGA and DORV with Subpulm VSD
      Arterial Switch Procedure
   TGA or DORV with Subpulm VSD
• Method of repair (usually during 1st week)
  Jatene: arterial switch, including cor art
  Lecompte: PA bifurcation ant to asc aorta
• Complications
  Dynamic RVOTO, PA stenosis (re-op in
    15%); cor art kinking or ostial stenosis
• Post-op mortality rates
  Early <5% and late <5%, due to technical
    problems, cor injury, RVOTO, LV failure
      Arterial Switch Procedure
     Post-Operative Complications
                                  Aorta
 RPA

PA        Ao         Ao
                LA

     OS

                                VSD
               VS               Patch
     RV

                                   LV
 RVOTO (Aut)              Fatal Acute AI (Aut)
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
Atrio-Pulmonary Anastomoses
                Types of Anomalies


 RA        LA          RA        LA        RA        LA



      LV                    LV                  RV



DILV (Usually)      Valve Atresia & IVS   CIRV (Usually)

Double Inlet       Single Inlet      Common Inlet
   Univentricular Atrioventricular Connections

Diagram of Single Functional Ventricles
   Atrio-Pulmonary Anastomoses
      Single Functional Ventricles
• Method (Modified Fontan)
  RA or SVC to PA (with many variations)
• Complications
  Related to no pumping chamber or valves
  Ascites, chylothorax, hepatic dysfunction,
   protein-losing enteropathy, RA thr, VT
• Post-op mortality rate (10-25%)
  Chronic HF, excess fluids, reoperation,
    infection, arrhythmias (SCD), PLE
  Atrio-Pulmonary Anastomoses
Heart with Modified Fontan Procedure

       RPA
                           LPA             SVC      LPA
SVC

               MPA
                          Ao
         RAA                                RPA

  RA
                                            SVC     MPA
                                 LV                 Patch
                     RV                     Patch


Ant-Sup View                              IVC

  RAA-to-MPA Anast                    RA Lateral Tunnel
Atrio-Pulmonary Anastomoses
 Pulmonary Vascular Changes




 Normal Artery   Septal Edema
Atrio-Pulmonary Anastomoses
 Post-Operative Complications


                  L



              L       L




          L   L



Protein-Losing Enteropathy (Autopsy)
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
Repair of Regurgitant Valves
        Ebstein Anomaly


                      LA

          RA
                               LV


                  Atrialized
                     RV
                               RV
    Right-Sided
   Cardiomegaly

    Diagram of Malformed TV
    Repair of Regurgitant Valves
             Ebstein Anomaly
• Methods of Repair
  Repair/replace TV, reduction R atrioplasty,
    plicate atrialized RV, close PFO or ASD
• Complications
  Prosthetic valve dysfunction or infection,
    persistent RV dysfunction (dilatation &
    fibrosis), kinked RCA with acute MI
• Postoperative mortality
  Causes: heart failure & fatal arrhythmias
   Repair of Regurgitant Valves
                Ebstein Anomaly
                                Ebstein, WPW, & SCD
           Ao                           (13M)
                                       LA
                PA
   RA
                                 RA            LV
            RV
                                        RV




Redundant & Fenestrated      Downward Displacement
Anterior Tricuspid Leaflet   of Septal & Post Leaflets

  Malformed TV & Cardiomegaly (Aut)
Repair of Regurgitant Valves
         Ebstein Anomaly




    Abnormal Cords & Papillary Muscles
      and Fenestrated Leaflet Tissue

Malformed Anterior TV Leaflet (Surg)
Repair of Regurgitant Valves
        Ebstein Anomaly




      Redundant Leaflet Tissue and
     Abnormal & Poorly-Formed Cords

Malformed Anterior TV Leaflet (Surg)
Repair of Regurgitant Valves
        Ebstein Anomaly




               RV

                         LV




TVR with Post-Op Heart Failure (Aut)
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
Postoperative Pathology of CHD
          Lecture Outline
  • Introduction to Post-Op CHD
  • Closure of Congenital Shunts
  • Relief of Obstructive Lesions
  • Ventriculo-Pulmonary Conduits
  • Arterial Switch Procedures
  • Atrio-Pulmonary Anastomoses
  • Repair of Regurgitant Valves
  • Summary of Post-Op CHD
  Introduction to Post-Op CHD
       Source of Specimens
• Autopsy
  Heart and lungs (may be separated)
  Dissection: Short-axis slices (apical
    and mid) and inflow-outflow (basal)
• Surgery
  Resected valve, myocardium, vessel
  Acquired heart disease (older adults)
  Explanted heart (at transplantation)
  Introduction to Post-Op CHD
 Evaluation of Autopsy Specimens
 Function as a “medical archeologist”
  Identify layers of change that have
  occurred over time, due to natural
  history and interventions for CHD

• Cardiovascular malformations
  Systematic evaluation: all anomalies
• Interventions (old and recent)
   Systematic evaluation: all procedures
    Summary of Post-Op CHD
            Autopsy Cases
• Positive postoperative effects
  Enlargement: hypoplastic structures
  Regression: hypertrophy & dilatation
  Regression: pulmonary hypertension
• Negative postoperative effects
  Complications: technical problems,
   acute heart failure, infection (cardiac
   & non-cardiac), progressive pulm htn
  Late effects: AI, fibrosis, heart failure
         Congenital Heart Disease:
         Challenges and Opportunities
         for Pathologists    USCAP
         March 7, 2009    Boston, MA


Postoperative Pathology of
Congenital Heart Disease

     William D. Edwards, M.D.
  Dept of Lab Medicine & Pathology
  Mayo Clinic, Rochester, MN, USA

				
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