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PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                   JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH

                    P. F.       #255

                 XX-XX-1949


                      ITALIAN
                     VARESE


                      HOUSE PAINTER

                   150 cm


                           48 Kg
            Systemic arterial hypertension
            Gastroesophageal reflux disease




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                   JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH
                                       JUNE 2004
                             Onset of mild dyspnea (WHO II)

                                       JUNE 2005
                             Worsening of dyspnea (WHO III)

                                    JULY 2005
                 Admission to the Cardiology ward of a local hospital

         • ECG: right ventricle overload
         • Echocardiogram:      dilation and hypokinesia of the right chambers
                                severe tricuspid regurgitation
                                    CTEPH
                                severe pulmonary hypertension (sPAP 85 mmHg)
         • Lung V/Q scan:       bilateral mismatches with multiple perfusion defects
         • HRCT scan:           multiple bilateral segmental perfusion defects
         • Venous echocolordoppler of lower limbs :     negative



UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                 JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH
                               SEPTEMBER 2005
                Admission to our Division for operability assessment

       • COMPLETE DIAGNOSTIC WORKUP:           CTEPH CONFIRMED
       • Right Heart Catheterization: RA             1 mmHg
                                    RV            82 / 0 mmHg
                                    PA         82 / 39 / 13 mmHg
                                    PCWP               3 mmHg
                                    CO                3.3 L/min
                                    CI                2.2 L/min
                                    RVEF              25 %
                                  PVRP         873 dyn*s*cm-5
       • OPERABILITY:     INOPERABLE FOR EXCLUSIVELY DISTAL DISEASE


                 DOUBLE LUNG TRANSPLANT WAITING LIST


UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                 JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH
                             LUNG V/Q SCAN


           VENTILATION                            PERFUSION




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                 JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH
                       PULMONARY ANGIOGRAM




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                 JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH
                               HRCT SCAN




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                    JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH
                                    DECEMBER 2005
                           Enrollment in the BENEFIT study
                  (bosentan vs. placebo in inoperable forms of CTEPH)
    INOPERABILITY CONFIRMED BY THE INTERNATIONAL COMMITTEE

                      Enrollment    End of study (16 weeks)   Open label extension
                     27-DEC-2005        28-APR-2006             26-OCT-2006
      RA                  4                   4                        6             mmHg
      RV                 82/2                82/0                    85/3            mmHg
      PA               82/46/24            82/45/22                85/49/28          mmHg
      PCWP                5                   5                        5             mmHg
      CO                 3.1                  2.4                     3.0            L/min
      CI                 2.1                  1.6                     2.0            L/min/m2
      RVEF               34                   11                      12             %
      PVR               1057                 1343                    1164            dyn*s*cm-5
      Serum-BNP          360                 324                      151            pg/ml
      WHO                III                  III                      II



UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                            JAMIESON TYPE III
           A peculiar case: a “seasoned veteran” in CTEPH
                  BENEFIT and BENEFIT-OPEN LABEL EXTENSION
                                 Clinical course

                                                   6mWT
                                                                              15-FEB-2008 Withdrawal from DLTx waiting list


         600
                                             480
                                                        456             462
         500                                                                              442
                                                                                                         418

         400
meters




                                   290
                270
         300

         200
                                  WHO III                              WHO II
         100
                        BENEFIT                          BENEFIT-Open Label
           0
               Dec-05             Apr-06    Oct-06    May-07          Oct-07            May-08         Jan-09

                                                     Follow-up


UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                 JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH
                                OCTOBER 2009
                     Worsening of dyspnea (back to WHO III)

                               NOVEMBER 2009
                   Admission to our Division for therapy update

        NEW OPERABILITY ASSESSMENT → NOW TECHNICALLY OPERABLE
     (JUST ALIKE THE PREVIOUS FINDINGS)


            BILATERAL PULMONARY ENDARTERECTOMY
                          Right: upper, middle and lower lobe
                         Left: upper lobe, lingula and lower lobe
                             Moderate hypothermia (23 C)
                 Intermittent circulatory arrests    right side: 91 min
                                                     left side: 47 min
                                                     total time: 138 min




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                 JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH
                 BILATERAL PEA – SURGICAL SPECIMEN




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                  JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH

                                  BILATERAL PEA
                             Early postoperative outcome



        • Invasive mechanical ventilation < 24 h

        • Assisted normal breathing with continuous positive pressure (cPAP)
          (to avoid reperfusion lung edema)

        • ICU stay: 2 days (fast-track recovery program)

        • Total postoperative hospital stay: 16 days




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                   JAMIESON TYPE III
      A peculiar case: a “seasoned veteran” in CTEPH
                                     BILATERAL PEA
                                    Hemodynamic results

                     Preoperative           At discharge   3 months FUP
                    27-NOV-2009            15-DEC-2009     26-FEB-2010

       RA                7                      3               3                mmHg

       RV              120/0                   53/0            35/0              mmHg
       PA            120/65/36               53/22/8         35/19/12            mmHg

       PCWP              5                      5               5                mmHg

       CO                3.5                    3.9            4.2               L/min

       CI                2.4                    2.7            2.9               L/min/m2

       RVEF              6                      18             21                %
       PVR              1371                   308             267      – 81 %   dyn*s*cm-5

       Serum-BNP        996                    742             106               pg/ml

       WHO               III                     I              I



UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                   JAMIESON TYPE III
                         The “seasoned veteran”



                                     SPECIFIC
                                     MEDICAL
                                     THERAPY                   PULMONARY
                                                            ENDARTERECTOMY
                      TRANSPLANT                              (Gold Standard)
                      WAITING LIST




                                                            SPECIFIC PAH-DRUG
                                                             DISCONTINUATION
DIAGNOSIS



  UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

             PEA IN THE ELDERLY
                             A peculiar case

                  P. B.   #250

                XX-XX-1934 (75 y)


                   ITALIAN
                  TURIN


                   PENSIONER

                 178 cm


                       72 Kg




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

             PEA IN THE ELDERLY
                             A peculiar case

                              MEDICAL HISTORY

                        • Congenital single kidney (right)
                        • Sigmoid diverticulosis
                        • Bilateral cataract
                        • 2000: colonscopy polyp removal


                                SEPTEMBER 2009
              Onset of mild dyspnea (WHO II) and right thoracic pain


                               OCTOBER 2009
           Acute dyspnea (WHO IV) → admission to the Emergency Ward




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

               PEA IN THE ELDERLY
                                  A peculiar case

                                   OCTOBER 2009
                           Emergency ward diagnostic workup

         • ECG: right ventricle overload
         • Echocardiogram:
                                     CTEPH
                                dilation and hypokinesia of the right chambers
                                paradoxical movement of the interventricular septum
                                severe tricuspid regurgitation
                                severe pulmonary hypertension (sPAP 90 mmHg)
         • HRCT scan:           multiple bilateral segmental perfusion defects




                                  NOVEMBER 2009
                  Admission to our Division for operability assessment




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

             PEA IN THE ELDERLY
                               A peculiar case

              THROMBOPHILIA AND HYPERCOAGULABILITY

                                    Laboratory findings

             • Hyperhomocysteinemia

             • Excess Factor VIII

             • Prothrombin gene mutation (G20210A – Factor II)

             • Homocysteine methabolism gene mutation (C677T – MTHFR)

             • Homocysteine methabolism gene mutation (A1298C – MTHFR)


                         Echocolordoppler of the lower limbs

             • Previous bilateral deep venous thrombosis




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

             PEA IN THE ELDERLY
                                A peculiar case

                            OPERABILITY ASSESSMENT

                                      Lung V/Q scan

               • Massive bilateral perfusion defects
                 (mainly at left upper lobe, lingula and right upper lobe)
               • Quantitative perfusion map: left 38% – right 62%


                                 Pulmonary angiogram

               • Right upper lobe occlusion
               • Right inferior segmental middle lobe occlusion
               • Right posterior segmental lower lobe occlusion
               • Left anterior and posterior segmental upper lobe occlusion
               • Left lower lobe occlusion




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

             PEA IN THE ELDERLY
                           A peculiar case
                        OPERABILITY ASSESSMENT

                                 HRCT scan




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

             PEA IN THE ELDERLY
                           A peculiar case

                        OPERABILITY ASSESSMENT

                                  Hemodynamic

                                         Preoperative
                                        06-NOV-2009
                           RA                2
                           RV               78/0
                           PA             78/38/15
                           PCWP              5
                           CO                4.4
                           CI                2.3
                           RVEF              36
                           PVR              600
                           Serum-BNP         70
                           WHO               III




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

             PEA IN THE ELDERLY
                              A peculiar case
                            COLLATERAL FINDINGS

                       Echocolordoppler of the neck vessels

                • 55% right common – internal carotid artery stenosis
                • 65% left common – internal carotid artery stenosis


                                Coronary angiogram

                • Left main coronary artery ostial stenosis (80%)
                • LAD, 1st Diagonal, 2nd Diagonal and Circumflex stenosis
                • Normal right coronary artery


                                   Echocardiogram

                • Moderate aortic regurgitation




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

             PEA IN THE ELDERLY
                                 A peculiar case
                                        OPERATION

                  BILATERAL PULMONARY ENDARTERECTOMY
                              Right: upper, middle and lower lobe
                             Left: upper lobe, lingula and lower lobe
                                  Moderate hypothermia (24 C)
                 Intermittent circulatory arrests         right side: 50 min
                                                          left side: 30 min
                                                          total time: 80 min



                      CORONARY ARTERY BYPASS GRAFTING
                       Right great saphenous vein on LAD and Circumflex



                            AORTIC VALVE REPLACEMENT
                        Carpentier-Edwards® Aortic Porcine Bioprosthesis




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

             PEA IN THE ELDERLY
                           A peculiar case
                 BILATERAL PEA – SURGICAL SPECIMEN




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              PEA IN THE ELDERLY
                               A peculiar case

                                  BILATERAL PEA
                             Early postoperative outcome



        • Invasive mechanical ventilation < 24 h

        • Assisted normal breathing with continuous positive pressure (cPAP)
          (to avoid reperfusion lung edema)

        • ICU stay: 2 days (fast-track recovery program)

        • Total postoperative hospital stay: 10 days




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                  PEA IN THE ELDERLY
                                   A peculiar case
                                    BILATERAL PEA
                                   Hemodynamic results

                    Preoperative         At discharge    3 months FUP
                   06-NOV-2009          18-NOV-2009      18-FEB-2010

      RA                2                    3                5            mmHg
      RV               78/0                 26/0             30/0          mmHg

      PA             78/38/15              26/11/5         30/18/9 – 53 % mmHg

      PCWP              5                    5                5            mmHg

      CO                4.4                  4.2             4.9    + 11 % L/min

      CI                2.3                  2.2             2.7           L/min/m2
      RVEF              36                   40              36            %

      PVR              600                  114              212    – 65 % dyn*s*cm-5

      Serum-BNP         70                  186              66            pg/ml

      WHO               III                   I               I




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

               WHO FUNCTIONAL CLASS II
                                    A peculiar case

                     T. S.         #108

                  XX-XX-1983


                      ITALIAN
                     GENOA


                      STUDENT

                    162 cm


                            79 Kg
            Thrombosis risk factors
             Sedentary lifestyle




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                WHO FUNCTIONAL CLASS II
                               A peculiar case
                                MEDICAL HISTORY

• Obesity (BMI > 30 kg/m2) – Hormonal contraception – Active smoker ( 20 cig / day)
• Recent car accident injuries without adequate venous thromboembolism prophylaxis
• Coagulative disorders (hyperhomocysteinemia with C677T and A1298C gene mutation,
  excess plasminogen activator inhibitor)

                                   NOVEMBER 2003
                              Acute pulmonary embolism
                        THROMBOLYSIS
            Right: partial embolic obstruction of the main pulmonary artery
              Left: total embolic occlusion of the main pulmonary artery




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

               WHO FUNCTIONAL CLASS II
                              A peculiar case
                                    APRIL 2004
                                    HRCT scan

        Despite thrombolysis and 6 months of full oral anticoagulation therapy

                            Right: patent pulmonary artery
      Left: persisting subtotal embolic obstruction of the main pulmonary artery




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                           A peculiar case
                                 APRIL 2004
                          Complete diagnostic workup

                             LUNG V/Q SCAN

           VENTILATION                                 PERFUSION




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                           A peculiar case
                                 APRIL 2004
                          Complete diagnostic workup

                                HEMODYNAMIC
                                         Preoperative
                                        07-APR-2004
                           RA                6
                           RV               33/0
                           PA             29/20/12
                           PCWP              4
                           CO                6.7
                           CI                3.6
                           RVEF              55
                           PVR              191
                           Serum-BNP         20
                           WHO               II




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                            A peculiar case
                                 APRIL 2004
                          Complete diagnostic workup

                       PULMONARY ANGIOGRAM

                      • Radiologic features

                      • Failure of anticoagulation on the left obstruction

                      • Normal hemodynamic despite severe perfusion defect




                                     DIFFERENTIAL DIAGNOSIS

                          LEFT PULMONARY ARTERY ANGIOSARCOMA ?


UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                           A peculiar case
                                 APRIL 2004
                          Complete diagnostic workup

                        DIFFERENTIAL DIAGNOSIS

               LEFT PULMONARY ARTERY ANGIOSARCOMA ?




                                           INTRAVASCULAR BIOPSY

                                          • Unfortunately unsuccessful

                                          • Undetermined diagnosis




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                           A peculiar case
          LEFT UNILATERAL PEA – INTRAOPERATIVE DIAGNOSIS



                              • At surgical inspection the endoluminal material
                                appeared to be thromboembolic
                              • A left unilateral PEA was performed
                              • Diagnosis confirmed by histological examination
                              • Uncomplicated postoperative recovery
                                Invasive Mechanical Ventilation        < 24 h
                                ICU stay                               2 days
                                Total postoperative hospital stay      8 days




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                           A peculiar case
                          LEFT UNILATERAL PEA
                            Hemodynamic results

                           Preoperative    At discharge
                          07-APR-2004     26-APR-2004

              RA               6               3            mmHg

              RV              33/0            29/0          mmHg

              PA            29/20/12        29/13/2 – 35 % mmHg

              PCWP             4               5            mmHg

              CO               6.7             7.5   + 12 % L/min

              CI               3.6             4.1          L/min/m2

              RVEF             55              53           %

              PVR             191              85    – 55 % dyn*s*cm-5

              Serum-BNP        20              91           pg/ml

              WHO              II               I



UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

               WHO FUNCTIONAL CLASS II
                              A peculiar case
                                   DISCUSSION


    • The hospitalization due to car accident injuries may have been complicated
      by a left pulmonary thromboembolism

    • The patient could have been asymptomatic because of her sedentary lifestyle

    • A second acute pulmonary thromboembolism on the right side could have
      revealed the previously asymptomatic disease

    • Even if oral anticoagulation relieved most of symptoms and hemodynamic
      was normal, we deemed the patient a potential candidate for PEA

    • The aim was to prevent the inexorable evolution of chronic thromboembolic
      lesions into CTEPH with vascular remodeling of the patent branches




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                             A peculiar case

                  P. G.   #146

                XX-XX-1944


                   ITALIAN
                  CREMONA


                   SPORTSMAN

                 180 cm


                       83 Kg




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                 WHO FUNCTIONAL CLASS II
                                A peculiar case
                                 MEDICAL HISTORY

• 1996: onset of exertional angina (negative cardiopulmonary exercise test)
• 2001: acute pulmonary embolism → oral anticoagulation therapy
• 2002 → 2006: regular echocardiographic controls showing the progressive onset of PH
• 2006: onset of exertional dyspnea (WHO II)




                                      MAY 2006
                  Admission to our Division for operability assessment




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                           A peculiar case
                                  MAY 2006
                          Complete diagnostic workup

                             LUNG V/Q SCAN

           VENTILATION                                 PERFUSION




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                           A peculiar case
                                  MAY 2006
                          Complete diagnostic workup

                                HEMODYNAMIC
                                         Preoperative
                                        11-MAY-2006
                           RA                4
                           RV               84/3
                           PA             84/45/20
                           PCWP              5
                           CO                2.9
                           CI                1.4
                           RVEF              18
                           PVR              1103
                           Serum-BNP        140
                           WHO               II




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                           A peculiar case
                                  MAY 2006
                          Complete diagnostic workup

                       PULMONARY ANGIOGRAM




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                           A peculiar case
                                  MAY 2006
                          Complete diagnostic workup

                                HRCT SCAN




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

              WHO FUNCTIONAL CLASS II
                           A peculiar case
                 BILATERAL PEA – SURGICAL SPECIMEN




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

                  WHO FUNCTIONAL CLASS II
                                   A peculiar case
                                    BILATERAL PEA
                                   Hemodynamic results

                    Preoperative          At discharge    4 years FUP
                   11-MAY-2006           26-MAY-2006     12-MAR-2010

      RA                4                     0               5            mmHg

      RV               84/3                  15/0            29/0          mmHg

      PA             84/45/20               15/12/6        29/18/9 – 60 % mmHg

      PCWP              5                     3               5            mmHg

      CO                2.9                   3.9            4.5    – 55 % L/min

      CI                1.4                   2.0            2.3           L/min/m2

      RVEF              18                    25             39            %

      PVR              1103                  185             231    – 79 % dyn*s*cm-5

      Serum-BNP        140                    82             12            pg/ml
      WHO               II                     I              I




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE

               WHO FUNCTIONAL CLASS II
                              A peculiar case
                                   DISCUSSION


        • Anticoagulation after the acute pulmonary embolism was probably
          not completely effective

        • The incomplete resolution of organized pulmonary emboli resulted
          in progressive right heart failure

        • Due to his high exercise tolerance, the patient became symptomatic
          only when the severe pulmonary hemodynamic jeopardized the left
          heart function (cardiac index of 1.4 L/min/m2)

        • Hemodynamic and radiologic features of the disease were both
          impressive despite paucity of symptoms




UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY

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CTEPH CASI CLINICI

  • 1. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH P. F. #255 XX-XX-1949 ITALIAN VARESE HOUSE PAINTER 150 cm 48 Kg Systemic arterial hypertension Gastroesophageal reflux disease UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 2. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH JUNE 2004 Onset of mild dyspnea (WHO II) JUNE 2005 Worsening of dyspnea (WHO III) JULY 2005 Admission to the Cardiology ward of a local hospital • ECG: right ventricle overload • Echocardiogram: dilation and hypokinesia of the right chambers severe tricuspid regurgitation CTEPH severe pulmonary hypertension (sPAP 85 mmHg) • Lung V/Q scan: bilateral mismatches with multiple perfusion defects • HRCT scan: multiple bilateral segmental perfusion defects • Venous echocolordoppler of lower limbs : negative UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 3. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH SEPTEMBER 2005 Admission to our Division for operability assessment • COMPLETE DIAGNOSTIC WORKUP: CTEPH CONFIRMED • Right Heart Catheterization: RA 1 mmHg RV 82 / 0 mmHg PA 82 / 39 / 13 mmHg PCWP 3 mmHg CO 3.3 L/min CI 2.2 L/min RVEF 25 % PVRP 873 dyn*s*cm-5 • OPERABILITY: INOPERABLE FOR EXCLUSIVELY DISTAL DISEASE DOUBLE LUNG TRANSPLANT WAITING LIST UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 4. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH LUNG V/Q SCAN VENTILATION PERFUSION UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 5. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH PULMONARY ANGIOGRAM UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 6. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH HRCT SCAN UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 7. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH DECEMBER 2005 Enrollment in the BENEFIT study (bosentan vs. placebo in inoperable forms of CTEPH) INOPERABILITY CONFIRMED BY THE INTERNATIONAL COMMITTEE Enrollment End of study (16 weeks) Open label extension 27-DEC-2005 28-APR-2006 26-OCT-2006 RA 4 4 6 mmHg RV 82/2 82/0 85/3 mmHg PA 82/46/24 82/45/22 85/49/28 mmHg PCWP 5 5 5 mmHg CO 3.1 2.4 3.0 L/min CI 2.1 1.6 2.0 L/min/m2 RVEF 34 11 12 % PVR 1057 1343 1164 dyn*s*cm-5 Serum-BNP 360 324 151 pg/ml WHO III III II UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 8. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH BENEFIT and BENEFIT-OPEN LABEL EXTENSION Clinical course 6mWT 15-FEB-2008 Withdrawal from DLTx waiting list 600 480 456 462 500 442 418 400 meters 290 270 300 200 WHO III WHO II 100 BENEFIT BENEFIT-Open Label 0 Dec-05 Apr-06 Oct-06 May-07 Oct-07 May-08 Jan-09 Follow-up UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 9. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH OCTOBER 2009 Worsening of dyspnea (back to WHO III) NOVEMBER 2009 Admission to our Division for therapy update NEW OPERABILITY ASSESSMENT → NOW TECHNICALLY OPERABLE (JUST ALIKE THE PREVIOUS FINDINGS) BILATERAL PULMONARY ENDARTERECTOMY Right: upper, middle and lower lobe Left: upper lobe, lingula and lower lobe Moderate hypothermia (23 C) Intermittent circulatory arrests right side: 91 min left side: 47 min total time: 138 min UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 10. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH BILATERAL PEA – SURGICAL SPECIMEN UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 11. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH BILATERAL PEA Early postoperative outcome • Invasive mechanical ventilation < 24 h • Assisted normal breathing with continuous positive pressure (cPAP) (to avoid reperfusion lung edema) • ICU stay: 2 days (fast-track recovery program) • Total postoperative hospital stay: 16 days UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 12. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III A peculiar case: a “seasoned veteran” in CTEPH BILATERAL PEA Hemodynamic results Preoperative At discharge 3 months FUP 27-NOV-2009 15-DEC-2009 26-FEB-2010 RA 7 3 3 mmHg RV 120/0 53/0 35/0 mmHg PA 120/65/36 53/22/8 35/19/12 mmHg PCWP 5 5 5 mmHg CO 3.5 3.9 4.2 L/min CI 2.4 2.7 2.9 L/min/m2 RVEF 6 18 21 % PVR 1371 308 267 – 81 % dyn*s*cm-5 Serum-BNP 996 742 106 pg/ml WHO III I I UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 13. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III The “seasoned veteran” SPECIFIC MEDICAL THERAPY PULMONARY ENDARTERECTOMY TRANSPLANT (Gold Standard) WAITING LIST SPECIFIC PAH-DRUG DISCONTINUATION DIAGNOSIS UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 14. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case P. B. #250 XX-XX-1934 (75 y) ITALIAN TURIN PENSIONER 178 cm 72 Kg UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 15. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case MEDICAL HISTORY • Congenital single kidney (right) • Sigmoid diverticulosis • Bilateral cataract • 2000: colonscopy polyp removal SEPTEMBER 2009 Onset of mild dyspnea (WHO II) and right thoracic pain OCTOBER 2009 Acute dyspnea (WHO IV) → admission to the Emergency Ward UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 16. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case OCTOBER 2009 Emergency ward diagnostic workup • ECG: right ventricle overload • Echocardiogram: CTEPH dilation and hypokinesia of the right chambers paradoxical movement of the interventricular septum severe tricuspid regurgitation severe pulmonary hypertension (sPAP 90 mmHg) • HRCT scan: multiple bilateral segmental perfusion defects NOVEMBER 2009 Admission to our Division for operability assessment UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 17. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case THROMBOPHILIA AND HYPERCOAGULABILITY Laboratory findings • Hyperhomocysteinemia • Excess Factor VIII • Prothrombin gene mutation (G20210A – Factor II) • Homocysteine methabolism gene mutation (C677T – MTHFR) • Homocysteine methabolism gene mutation (A1298C – MTHFR) Echocolordoppler of the lower limbs • Previous bilateral deep venous thrombosis UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 18. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case OPERABILITY ASSESSMENT Lung V/Q scan • Massive bilateral perfusion defects (mainly at left upper lobe, lingula and right upper lobe) • Quantitative perfusion map: left 38% – right 62% Pulmonary angiogram • Right upper lobe occlusion • Right inferior segmental middle lobe occlusion • Right posterior segmental lower lobe occlusion • Left anterior and posterior segmental upper lobe occlusion • Left lower lobe occlusion UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 19. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case OPERABILITY ASSESSMENT HRCT scan UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 20. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case OPERABILITY ASSESSMENT Hemodynamic Preoperative 06-NOV-2009 RA 2 RV 78/0 PA 78/38/15 PCWP 5 CO 4.4 CI 2.3 RVEF 36 PVR 600 Serum-BNP 70 WHO III UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 21. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case COLLATERAL FINDINGS Echocolordoppler of the neck vessels • 55% right common – internal carotid artery stenosis • 65% left common – internal carotid artery stenosis Coronary angiogram • Left main coronary artery ostial stenosis (80%) • LAD, 1st Diagonal, 2nd Diagonal and Circumflex stenosis • Normal right coronary artery Echocardiogram • Moderate aortic regurgitation UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 22. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case OPERATION BILATERAL PULMONARY ENDARTERECTOMY Right: upper, middle and lower lobe Left: upper lobe, lingula and lower lobe Moderate hypothermia (24 C) Intermittent circulatory arrests right side: 50 min left side: 30 min total time: 80 min CORONARY ARTERY BYPASS GRAFTING Right great saphenous vein on LAD and Circumflex AORTIC VALVE REPLACEMENT Carpentier-Edwards® Aortic Porcine Bioprosthesis UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 23. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case BILATERAL PEA – SURGICAL SPECIMEN UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 24. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case BILATERAL PEA Early postoperative outcome • Invasive mechanical ventilation < 24 h • Assisted normal breathing with continuous positive pressure (cPAP) (to avoid reperfusion lung edema) • ICU stay: 2 days (fast-track recovery program) • Total postoperative hospital stay: 10 days UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 25. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA IN THE ELDERLY A peculiar case BILATERAL PEA Hemodynamic results Preoperative At discharge 3 months FUP 06-NOV-2009 18-NOV-2009 18-FEB-2010 RA 2 3 5 mmHg RV 78/0 26/0 30/0 mmHg PA 78/38/15 26/11/5 30/18/9 – 53 % mmHg PCWP 5 5 5 mmHg CO 4.4 4.2 4.9 + 11 % L/min CI 2.3 2.2 2.7 L/min/m2 RVEF 36 40 36 % PVR 600 114 212 – 65 % dyn*s*cm-5 Serum-BNP 70 186 66 pg/ml WHO III I I UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 26. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case T. S. #108 XX-XX-1983 ITALIAN GENOA STUDENT 162 cm 79 Kg Thrombosis risk factors Sedentary lifestyle UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 27. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case MEDICAL HISTORY • Obesity (BMI > 30 kg/m2) – Hormonal contraception – Active smoker ( 20 cig / day) • Recent car accident injuries without adequate venous thromboembolism prophylaxis • Coagulative disorders (hyperhomocysteinemia with C677T and A1298C gene mutation, excess plasminogen activator inhibitor) NOVEMBER 2003 Acute pulmonary embolism THROMBOLYSIS Right: partial embolic obstruction of the main pulmonary artery Left: total embolic occlusion of the main pulmonary artery UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 28. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case APRIL 2004 HRCT scan Despite thrombolysis and 6 months of full oral anticoagulation therapy Right: patent pulmonary artery Left: persisting subtotal embolic obstruction of the main pulmonary artery UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 29. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case APRIL 2004 Complete diagnostic workup LUNG V/Q SCAN VENTILATION PERFUSION UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 30. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case APRIL 2004 Complete diagnostic workup HEMODYNAMIC Preoperative 07-APR-2004 RA 6 RV 33/0 PA 29/20/12 PCWP 4 CO 6.7 CI 3.6 RVEF 55 PVR 191 Serum-BNP 20 WHO II UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 31. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case APRIL 2004 Complete diagnostic workup PULMONARY ANGIOGRAM • Radiologic features • Failure of anticoagulation on the left obstruction • Normal hemodynamic despite severe perfusion defect DIFFERENTIAL DIAGNOSIS LEFT PULMONARY ARTERY ANGIOSARCOMA ? UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 32. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case APRIL 2004 Complete diagnostic workup DIFFERENTIAL DIAGNOSIS LEFT PULMONARY ARTERY ANGIOSARCOMA ? INTRAVASCULAR BIOPSY • Unfortunately unsuccessful • Undetermined diagnosis UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 33. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case LEFT UNILATERAL PEA – INTRAOPERATIVE DIAGNOSIS • At surgical inspection the endoluminal material appeared to be thromboembolic • A left unilateral PEA was performed • Diagnosis confirmed by histological examination • Uncomplicated postoperative recovery Invasive Mechanical Ventilation < 24 h ICU stay 2 days Total postoperative hospital stay 8 days UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 34. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case LEFT UNILATERAL PEA Hemodynamic results Preoperative At discharge 07-APR-2004 26-APR-2004 RA 6 3 mmHg RV 33/0 29/0 mmHg PA 29/20/12 29/13/2 – 35 % mmHg PCWP 4 5 mmHg CO 6.7 7.5 + 12 % L/min CI 3.6 4.1 L/min/m2 RVEF 55 53 % PVR 191 85 – 55 % dyn*s*cm-5 Serum-BNP 20 91 pg/ml WHO II I UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 35. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case DISCUSSION • The hospitalization due to car accident injuries may have been complicated by a left pulmonary thromboembolism • The patient could have been asymptomatic because of her sedentary lifestyle • A second acute pulmonary thromboembolism on the right side could have revealed the previously asymptomatic disease • Even if oral anticoagulation relieved most of symptoms and hemodynamic was normal, we deemed the patient a potential candidate for PEA • The aim was to prevent the inexorable evolution of chronic thromboembolic lesions into CTEPH with vascular remodeling of the patent branches UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 36. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case P. G. #146 XX-XX-1944 ITALIAN CREMONA SPORTSMAN 180 cm 83 Kg UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 37. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case MEDICAL HISTORY • 1996: onset of exertional angina (negative cardiopulmonary exercise test) • 2001: acute pulmonary embolism → oral anticoagulation therapy • 2002 → 2006: regular echocardiographic controls showing the progressive onset of PH • 2006: onset of exertional dyspnea (WHO II) MAY 2006 Admission to our Division for operability assessment UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 38. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case MAY 2006 Complete diagnostic workup LUNG V/Q SCAN VENTILATION PERFUSION UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 39. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case MAY 2006 Complete diagnostic workup HEMODYNAMIC Preoperative 11-MAY-2006 RA 4 RV 84/3 PA 84/45/20 PCWP 5 CO 2.9 CI 1.4 RVEF 18 PVR 1103 Serum-BNP 140 WHO II UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 40. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case MAY 2006 Complete diagnostic workup PULMONARY ANGIOGRAM UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 41. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case MAY 2006 Complete diagnostic workup HRCT SCAN UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 42. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case BILATERAL PEA – SURGICAL SPECIMEN UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 43. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case BILATERAL PEA Hemodynamic results Preoperative At discharge 4 years FUP 11-MAY-2006 26-MAY-2006 12-MAR-2010 RA 4 0 5 mmHg RV 84/3 15/0 29/0 mmHg PA 84/45/20 15/12/6 29/18/9 – 60 % mmHg PCWP 5 3 5 mmHg CO 2.9 3.9 4.5 – 55 % L/min CI 1.4 2.0 2.3 L/min/m2 RVEF 18 25 39 % PVR 1103 185 231 – 79 % dyn*s*cm-5 Serum-BNP 140 82 12 pg/ml WHO II I I UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
  • 44. PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE WHO FUNCTIONAL CLASS II A peculiar case DISCUSSION • Anticoagulation after the acute pulmonary embolism was probably not completely effective • The incomplete resolution of organized pulmonary emboli resulted in progressive right heart failure • Due to his high exercise tolerance, the patient became symptomatic only when the severe pulmonary hemodynamic jeopardized the left heart function (cardiac index of 1.4 L/min/m2) • Hemodynamic and radiologic features of the disease were both impressive despite paucity of symptoms UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY