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CTEPH, Surgical and Medical Therapy. CTEPH, Terapia Chirurgica e Medica
 

CTEPH, Surgical and Medical Therapy. CTEPH, Terapia Chirurgica e Medica

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    CTEPH, Surgical and Medical Therapy. CTEPH, Terapia Chirurgica e Medica CTEPH, Surgical and Medical Therapy. CTEPH, Terapia Chirurgica e Medica Presentation Transcript

    • Andrea M D’Armini, MD, FCCP PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE “CTEPH” Cardiac Surgery University of Pavia School of Medicine Foundation I.R.C.C.S. “San Matteo” Hospital Pavia, Italy
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE BACKGROUND Chronic Thromboembolic Pulmonary Hypertension CTEPH Acute Pulmonary Embolism
      • Chronic thromboembolic pulmonary hypertension (CTEPH) represents a sequel of non-resolved venous thromboembolism with fatal natural history due to chronic right ventricular failure
      • Progress in surgical treatment over the past decade has considerably improved the outcome of CTEPH patients
      PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE BACKGROUND
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE INTRODUCTION
      • CTEPH represents the only type of pulmonary hypertension surgically treatable, in the majority of cases, without transplant
      • This life-saving conservative surgery is called pulmonary endarterectomy (PEA)
      • Vascular lung diseases suitable for transplantation are:
        • Idiopathic Pulmonary Hypertension
        • Eisenmenger’s Syndrome
        • CTEPH
      PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE VASCULAR LUNG DISEASES
      • Eisenmenger’s Syndrome
        • Idiophatic Pulmonary Hypertension
      1990 2011 Optimal Medical Therapy BEFORE HL/L Transplantation PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE VASCULAR LUNG DISEASES
      • 10089 HEART TRANSPLANTS
      • 309 LUNG TRANSPLANTS
      • 39 HEART-LUNG TRANSPLANTS
      PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE THORACIC TRANSPLANTATION 1357 TRANSPLANTS (17/11/1985 – 09/06/2011) 1357 INTRATHORACIC TRANSPLANTS
    • 348 LUNG AND HEART-LUNG TRANSPLANTS
      • 79 VASCULAR LUNG DISEASES
      • 269 PARENCHYMAL LUNG DISEASES
      PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE THORACIC TRANSPLANTATION 1357 TRANSPLANTS (17/11/1985 – 09/06/2011)
    • U. G. PRE DLTx U. G. 1° POST DLTx DLTx for FAMILIAL PULMONARY HYPERTENSION PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE THORACIC TRANSPLANTATION 1357 TRANSPLANTS (17/11/1985 – 09/06/2011)
    • DLTx for FAMILIAL PULMONARY HYPERTENSION RIGHT HEART CATHETERIZATION PRE-DLTx 1° POST-DLTx Right Atrium 13 9 Right Ventricle 118/0 25/0 Pulmonary Arterial Pressure 118/82/60 38/25/16 ( -70% ) Systemic Arterial Pressure 96/76/61 113/73/53 Pulmonary Capillary Wedge Pressure 5 10 Cardiac Output 2.9 6.2 ( +114% ) Cardiac index 1.6 4.0 Pulmonary Vascular Resistance 2134 155 ( -91% ) PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE THORACIC TRANSPLANTATION 1357 TRANSPLANTS (17/11/1985 – 09/06/2011)
    • HLTx for EISENMENGER’S SYNDROME M. P. PRE HLTx M. P. 1° POST HLTx PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE THORACIC TRANSPLANTATION 1357 TRANSPLANTS (17/11/1985 – 09/06/2011)
    • HLTx for EISENMENGER’S SYNDROME PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE THORACIC TRANSPLANTATION 1357 TRANSPLANTS (17/11/1985 – 09/06/2011) Systolic Pulmonary Arterial Pressure 105 mmHg Right Ventricular End-Diastolic Diameter 110 mm Inferior Vena Cava 34 mm PRE-OPERATIVE ECHOCARDIOGRAPHY Right Atrium 6 mmHg Right Ventricle 23/0 mmHg Pulmonary Arterial Pressure 23/11/6 mmHg Pulmonary Capillary Wedge Pressure 6 mmHg Cardiac Output 7.1 L/min Cardiac Index 4.3 L/min/m 2 Pulmonary Vascular Resistance 45 dyne*sec*cm -5 POST-OPERATIVE RIGHT HEART CATHETERIZATION
    • 1990 2011 PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE SURGICAL TREATMENT OF CTEPH PAVIA EXPERIENCE - 357 PEAs
      • CTEPH
      • ( NO SPECIFIC DRUGS ARE CURRENTLY APPROVED FOR CTEPH )
      From Transplant to Conservative Surgery (PEA)
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE INTRODUCTION
      • Elective surgery, non donor-dependent
      • No “transplant window” to be considered
      • Age is not a contraindication
      • Lower post-operative complications
        • early (acute graft failure, acute rejection, infections)
        • late (BOS, neoplasms, infections)
      • Outcome
        • post-operative long term survival
        • quality of life (back to normal)
        • steady functional improvement
      PEA vs. LTx
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE EPIDEMIOLOGY
      • Epidemiologic data: in Italy ≈ 65.000 cases / year of acute symptomatic pulmonary embolism (PE)
      • Prevalence of CTEPH in pts surviving an acute PE ( ≈ 80 %) is calculated between 0.5% – 3.8%
      • -> up to 2.000 new cases / year
      • Considering asymptomatic pulmonary embolism and misdiagnosed pulmonary embolism, the true incidence of CTEPH may be even greater
      • Jamieson SW, Kapelanski DP. Pulmonary endarterectomy. Curr Probl Surg 2000; 37:165-252
      • Fedullo PF, Auger WR, Kerr KM, Rubin LJ. Chronic thromboembolic pulmonary hypertension. N Engl J Med 2001; 345:1465-72
      • Pengo V, Lensing AV, Prins MH, Marchiori A, Davidson BL, Tiozzo F et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004; 350:2257-64
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CURRENT SITUATION
      • CTEPH is still under-diagnosed and nowadays only few physicians are aware of the surgical procedure called PEA
      • For all these reasons about 8000 PEA have been performed worldwide so far with ≈ 30 % of all cases carried out by the San Diego Group
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE NATURAL HISTORY
      • Pulmonary embolism (symptomatic / asymptomatic)
      • “ Honeymoon ” period: months / years
      • Hypertensive remodeling of the patent pulmonary vascular bed (Eisenmenger-like)
      • Right ventricle hypertrophy with progressive right heart deterioration  right failure
      • Left ventricle compression with left heart functional impairment
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE GENERAL CONDITIONS
      • Low cardiac output with dyspnea, cough, cyanosis, hepatomegaly, ascites, lower limb edema, syncope, hemoptysis and interscapular olosystolic murmur
      • Hypoxemia with exercise, sometimes at rest also
      • Frequent positive anamnesis for deep venous thrombosis and / or coagulative and immunologic disorders
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE COAGULATIVE DISORDERS DISORDER % PTS MEAN ± SD RANGE HYPERHOMOCYSTEINEMIA ( μ mol/L) 72.6 % 21.7 ± 8.3 14.1 – 63.2 EXCESS FACTOR VIII ANTIGEN (%) 78.2 % 206.7 ± 33.9 161.1 – 392.9 EXCESS FACTOR VIII RISTOCETIN (%) 47.6 % 182.1 ± 46.6 150.0 – 334.0 EXCESS FACTOR VIII (%) 27.4 % 179.3 ± 25.8 153.4 – 220.0 PAI EXCESS (U/ml) 53.2 % 5.1 ± 1.2 3.6 – 7.9 FACTOR V LEIDEN 15.3 % 1.34 ± 0.55 0.50 – 1.99
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE IMMUNOLOGIC DISORDERS DISORDER % PTS MEAN ± SD RANGE Anti-Nuclear Antibodies (ANA) 23.4 % – – Lupus Anticoagulant (LAC) 19.4 % – – Anti-Cardiolipin Antibodies (ACA) IgG 20.2 % 56.3 ± 40.3 10.3 – 121.0 Anti-Cardiolipin Antibodies (ACA) IgM 13.7 % 30.8 ± 30.5 7.3 – 101.0 Anti-Phospholipid Antibodies (APA) IgG 14.5 % 63.2 ± 36.5 8.4 – 121.0 Anti-Phospholipid Antibodies (APA) IgM 12.9 % 28.0 ± 23.5 10.1 – 91.3 Positive Direct Coombs’ Test 8.9 % – –
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE GENETIC MUTATIONS MUTATION % PTS C677T MTHFR (HOMOCYSTEINEMIA) HETEROZYGOSIS 50.8 % HOMOZYGOSIS 19.4 % TOTAL 70.2 % A1298C MTHFR (HOMOCYSTEINEMIA) HETEROZYGOSIS 44.4 % HOMOZYGOSIS 9.7 % TOTAL 54.1 % G20210A PROTHROMBIN (FACTOR II) 8.1 % G1691A FACTOR V (FACTOR V LEIDEN) 5.6 %
      • A PERMANENT INFERIOR VENA CAVA FILTER was placed before PEA in the majority (335/357) of patients
      • Lifelong oral anticoagulation was prescribed after PEA
      PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE MARKED THROMBOPHILIA
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE REFERENCE
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE REFERENCE
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE REFERENCE
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE REFERENCE
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE INDICATIONS FOR SURGERY
      • The indications for the surgical treatment of these patients are based on
      • CLINIC
      • HEMODYNAMIC
      • The indications for the type of surgery are based on
      • ANATOMY
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CLINIC The clinical indication changes substantially according to the different surgical treatment of CTEPH
      • Age < 60 yrs
      • WHO III or IV
      • Contraindications
      • Tx “window”
      • Age is not a contraindication
      • Symptomatic PH (WHO II-III-IV )
      • Absence of severe pulmonary parenchymal disease
      • Elective surgery
      Tx PEA PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CLINIC TRANSPLANT WINDOW TOO LATE TOO EARLY
      • Pulmonary hypertension (mPAP  25 mmHg)
      • Pulmonary wedge pressure < 15 mmHg
      • Causing low cardiac output
      • Resulting in calculated pulmonary vascular resistances (PVR) > 300 dyne*sec*cm -5
      PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE HEMODYNAMIC PRECAPILLARY PULMONARY HYPERTENSION
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE ANATOMY
      • The surgical treatment depends on the localization of the lesions in the pulmonary arterial branches
      • Lesions can be classified as PROXIMAL
      • DISTAL
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PROXIMAL LESIONS
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE M.B. – 62 yrs M – Jul 2001 – PEA #64 Perfusion and ventilation scan Pulmonary angiogram Hemodynamic mPAP 67 CI 1.6 PVR 1766
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE DISTAL LESIONS
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE S.S. – 31 yrs M – Sep 2002 Perfusion and ventilation scan Pulmonary angiogram Hemodynamic mPAP 50 CI 1.8 PVR 1120
      • Growing single surgeon’s experience due to learning curve
      PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE OPERABILITY ASSESSMENT Which lesions have to be considered as inoperable ?
      • Different operability assessments from different Centers
    • Concomitant severe parenchymal lung disease is the real absolute contraindication to PEA Such patients are not suitable for PEA and must be listed for DLTx (if indicated) PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CONTRAINDICATION TO PEA
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE P.B. – 60 yrs M – Jun 2002 Perfusion and ventilation scintigraphy Pulmonary angiography CT scan Hemodynamic mPAP 28 CI 1.9 PVR 645
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE BACKGROUND
      • PEA is the treatment of choice for patients with CTEPH
      • About only 10-15 Centers worldwide are performing PEA on a routine basis
      • In expert hands, mortality ranges between 5% and 12% and technical failure is below 8%
      • Early hemodynamic results
      • are known to be excellent in
      • case of successful operation
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE REFERENCE
      • National referral program
      • Begin: April 1994
      • To date: 357 PEAs performed
      PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE OUR PROGRAM
    • 55 101 12 4 15 19 16 28 7 4 7 21 5 18 15 3 6 14
      • Pts coming from outside Italy
      • Greece 1
      • Kosovo 1
      • - Uganda 1
      2 2 Pavia PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PATIENTS’ REFERRAL OF 357 PEAs ≤ 10 pts 11 – 20 pts ≥ 21 pts
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE AMOUNT OF PATIENTS 65 PEAs in almost 8 yrs 65 PEAs in 1 yr
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE AMOUNT OF PATIENTS NEW EVALUATIONS + 100%
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE AMOUNT OF PATIENTS DIAGNOSTIC ACCURACY 63% 76% + 13%
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE AMOUNT OF PATIENTS OPERABILITY RATE 74% 89% + 15%
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE AMOUNT OF PATIENTS PEAs PERFORMED + 195 %
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PAVIA CTEPH PROGRAM January, 1 st - December, 31 st 2004  130 pts EVALUATION (54 pts) FOLLOW-UP (72 pts) DLTx (4 pts) CONFIRMED (34 pts - 63%) OTHER DIAGNOSIS (20 pts - 37%)
      • RECENT EMBOLIZATION (3 pts)
      • - 2 medical therapy
      • - 1 surgical embolectomy
      • TUMORS (5 pts)
      • - 3 pulmonary angiosarcoma
      • - 1 adenocarcinoma with pulmonary artery thrombosis
      • - 1 intestinal tumor with liver metastases
      • MISCELLANEOUS (12 pts)
      OPERABILITY RATE 74 %
      • PROXIMAL LESIONS (25 pts)
      • - 22 PEAs
      • - 2 pts refused
      • - 1 pt died on evaluation
      • DISTAL LESIONS (7 pts)
      • - 5 DLTx waiting-list
      • - 2 medical therapy (too old for DLTx)
      • ASSOCIATION WITH SEVERE EMPHYSEMA (2 pts)
      • - 2 DLTx waiting-list
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PAVIA CTEPH PROGRAM January, 1 st – December, 31 st 2010  231 pts New Evaluations (108 pts) PEAs FUP (99 pts) Clinical Trials (23 pts) HLTx (1 pt) CONFIRMED (82 pts - 76%) OTHER DIAGNOSIS (26 pts - 24%) OPERABILITY RATE 89 %
      • PROXIMAL LESIONS (73 pts)
      • - 65 PEAs
      • - 4 pts waiting for PEA
      • - 3 pts refused PEA
      • - 1 pt with severe co-morbidities
      • DISTAL LESIONS (8 pts)
      • - 8 medical therapy: 5 too old for DLTx
      • 3 too early for DLTx
      • RECENT EMBOLIZATION (6 pts)
      • - 3 medical therapy
      • - 3 surgical embolectomy
      • MINIMAL CTE LESIONS WITHOUT PH (2 pts)
      • - 2 medical therapy
      • MISCELLANEOUS (15 pts)
      • TUMOR (3 pts)
      • - 3 pulmonary angiosarcoma
      • ASSOCIATION WITH SEVERE EMPHYSEMA (1 pt)
      • - 1 DLTx waiting-list
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PAVIA CTEPH PROGRAM January, 1 st – June, 9 th 2011  145 pts New Evaluations (57 pts) PEAs FUP (69 pts) Clinical Trials (18 pts) DL/HLTx (1 pt) CONFIRMED (31 pts - 54%) OTHER DIAGNOSIS (26 pts - 46%) OPERABILITY RATE 94 %
      • PROXIMAL LESIONS (29 pts)
      • - 29 PEAs (4 pts evaluated in 2010)
      • - 4 pts waiting for PEA
      • DISTAL LESIONS (2 pts)
      • - 2 medical therapy: 1 too old for DLTx
      • 1 too early for DLTx
      • MISCELLANEOUS (25 pts)
      • TUMOR (1 pt)
      • - 1 pulmonary angiosarcoma
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE MAIN WORLD PEA CENTERS Paris, France ≈ 100 PEAs / year NATIONAL REFERRAL PROGRAM FOR EXCELLENCE Cambridge, UK ≈ 80 PEAs / year NATIONAL REFERRAL PROGRAM BY LAW Pavia, Italy ≈ 60 PEAs / year MORE THAN ONE PROGRAM Bad Nauheim, Germany ≈ 50 PEAs / year MORE THAN ONE PROGRAM San Diego, California, USA ≈ 130 PEAs / year NATIONAL REFERRAL PROGRAM FOR EXCELLENCE
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE SURGICAL TREATMENT OF CTEPH 08-MAR-1991 First HLTx for CTEPH 11-APR-1994 First PEA 28-JUL-2003 First PEA in patient listed for DLTx 25-DEC-1995 First DLTx for CTEPH
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE TRANSPLANT FOR CTEPH 18 / 79 MORE DIAGNOSIS MORE DISTAL PEAs
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PEA POPULATION OF 357 PEAs
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE RIGHT HEART CATHETERIZATION OF 357 PEAs
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE ECHOCARDIOGRAPHY OF 357 PEAs
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE ECHOCARDIOGRAPHY OF 357 PEAs
      • Tricuspid regurgitation Absent 2.7%
      • Mild 18.9%
      • Moderate 29.7%
      • Severe 48.7%
      • Abnormal interventricular septal motion Yes 91.9%
      • No 8.1%
      • Inferior vena cava collapsibility Yes 40.5%
      • No 59.5%
      78.4%
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE ECHOCARDIOGRAPHY OF 357 PEAs
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE ECHOCARDIOGRAPHY
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CARDIAC MAGNETIC RESONANCE OF 244 PEA
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CARDIAC MAGNETIC RESONANCE OF 244 PEA
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CARDIAC MAGNETIC RESONANCE
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE LUNG FUNCTION OF 357 PEAs
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE ARTERIAL BLOOD GASES OF 357 PEAs
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE MODIFIED BRUCE TEST OF 357 PEAs
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CARDIOPULMONARY EXERCISE TESTING OF 200 PEAs
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CARDIOPULMONARY EXERCISE TESTING OF 200 PEAs
      • Median sternotomy
      • Cardio Pulmonary Bypass
      • Moderate hypotermia (24 °C)
      • Circulatory arrest (7 min)
      • Reperfusion period (5 min)
      • Bilateral
      J Thorac Cardiovasc Surg 1993;106:116-27 PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PULMONARY ENDARTERECTOMY
      • Intra-wall dissection
      • Peripheral extension
      • Explore all branches
      J Thorac Cardiovasc Surg 1993;106:116-27 PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PULMONARY ENDARTERECTOMY
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE SURGICAL INSTRUMENT
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PULMONARY ENDARTERECTOMY
    • E.L. – 38 yrs M – Dec 1999 – PEA #42 mPAP 43  20 (-53%) CO 3.3  6.9 (+109%) PVR 994  220 (-78%) PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE TYPICAL SURGICAL SPECIMENS P.A. – 66 yrs M – Jun 2001 – PEA #60 mPAP 50  25 (-50%) CO 2.6  4.4 (+69%) PVR 1385  364 (-74%)
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE DISTAL LESIONS JAMIESON TYPE III LEARNING CURVE
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE I vs. TYPE II vs. TYPE III L.M.E.L. - 65 yrs M - Oct 2004 - PEA #119 mPAP 39  19 (-51%) CO 4.4  5.4 (+23%) PVR 665  222 (-66%) G.A.C. - 52 yrs F - Jul 2003 - PEA #96 mPAP 48  27 (-44%) CO 2.1  4.2 (+100%) PVR 1638  381 (-77%) B.A. - 43 yrs F - May 2009 - PEA #233 mPAP 49  19 (-61%) CO 3.3  5.0 (+52%) PVR 1067  224 (-79%)
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III B.A. - 43 yrs F - May 2009 - PEA #233 mPAP 49  19 (-61%) CO 3.3  5.0 (+52%) RVEF 16  35 (+119%) PVR 1067  224 (-79%)
    • Pre-operative Pulmonary Angiogram Pre-operative 64-HRCT F.C. - 33 yrs F - Apr 2009 - PEA #225 mPAP 52  20 (-62%) CO 4.6  4.7 (+2%) RVEF 32  41 (+28%) PVR 870  255 (-71%) PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE OUT OF PROPORTION PH ? Pre-operative Pulmonary Angiogram Pre-operative 64-HRCT B.R.A. - 72 yrs F mPAP 44 CO 2.9 RVEF 28 PVR 1159
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III Pre-operative Pulmonary Angiogram Pre-operative 64-HRCT B.R.A. - 72 yrs F - Mar 2009 - PEA #222 mPAP 44  33 (-25%) CO 2.9  4.9 (+69%) RVEF 28  34 (+21%) PVR 1159  457 (-61%) B.R.A. - 72 yrs F mPAP 44 CO 2.9 RVEF 28 PVR 1159
    • Pre-operative Pulmonary Angiogram Pre-operative 64-HRCT G.G. - 62 yrs F mPAP 51 CO 2.6 RVEF 19 PVR 1415 PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE OUT OF PROPORTION PH ?
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE JAMIESON TYPE III Pre-operative Pulmonary Angiogram Pre-operative 64-HRCT G.G. - 62 yrs F - Sep 2009 - PEA #240 mPAP 51  27 (-47%) CO 2.6  4.0 (+54%) RVEF 19  24 (+26%) PVR 1415  460 (-68%) G.G. - 62 yrs F mPAP 51 CO 2.6 RVEF 19 PVR 1415
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CUMULATIVE PROPORTION SURVIVING OF 357 PEAs Operative mortality Global 32/357 (9.0%) NYHA II 0/33 (0.0%) NYHA III 8/165 (4.8%) NYHA IV 24/159 (15.1%) Jan 08 – May 11 13/183 (7.1%) 89.2  1.9 87.1  2.2 86.5  2.2 85.6  2.4 84.5  2.6 83.1  2.9 81.6  3.2 79.2  3.9 79.2  3.9
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CUMULATIVE PROPORTION SURVIVING SURGERY vs. MEDICAL THERAPY Riedel M. Chest 1982;81(2):151-8. D ’ Armini A.M. Ital Heart J 2005;6(10):861-8.
    • SURGICAL TREATMENT OF CTEPH: FROM TRANSPLANT TO CONSERVATIVE SURGERY CUMULATIVE PROPORTION SURVIVING 45 PTS ON WAITING LIST FOR TRANSPLANT IN CTEPH
    • SURGICAL TREATMENT OF CTEPH: FROM TRANSPLANT TO CONSERVATIVE SURGERY CUMULATIVE PROPORTION SURVIVING 18 TRANSPLANTS IN CTEPH
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE FOLLOW-UP In literature few data are reported on mid- and long- term cardiopulmonary function, particularly on exertion, and on clinical outcomes after PEA
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE FOLLOW-UP STUDY
      • To monitor the natural history of these pts after PEA in terms of:
        • clinical status (by NYHA class)
        • hemodynamic (by right heart catheterization)
        • heart morphology and function (by ecocardiography and nuclear magnetic resonance)
        • lung function (by spirometry, carbon monoxide transfer factor, arterial blood gas)
        • exercise tolerance (by modified Bruce test and cardiopulmonary exercise testing)
        • anatomy of arterial pulmonary vascular branches (by perfusion scinti scan, pulmonary angiography, multi detector angio-CT scan)
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE FOLLOW-UP TIMING
      • All pts underwent follow-up evaluation at:
        • discharge (at this interval NYHA class, lung function, and exercise tolerance are excluded because pts are to close to the surgical procedure)
        • 3 th month
        • yearly for 5 years
        • 7 th , 10 th and 15 th year (10 controls)
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE NYHA FUNCTIONAL CLASS Pre-op 3m 1y 3y 5y 7y 10y p < 0.01
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE mean PULMONARY ARTERY PRESSURE p < 0.01 Pre-op disch 3m 1y 3y 5y 7y 10y
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PULMONARY VASCULAR RESISTANCES p < 0.01 Pre-op disch 3m 1y 3y 5y 7y 10y
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE ECHOCARDIOGRAPHY Before PEA
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE ECHOCARDOGRAPHY First echo after PEA – POD #9
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE ECHOCARDIOGRAPHY 3-months FUP after PEA
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CARDIAC MAGNETIC RESONANCE Before PEA First CMR after PEA – POD #6
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CARDIAC MAGNETIC RESONANCE 4-years FUP after PEA First CMR after PEA – POD #6
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE REVERSE RIGHT VENTRICULAR REMODELING
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE ARTERIAL OXYGEN PARTIAL PRESSURE p < 0.01 Pre-op 3m 1y 3y 5y 7y 10y
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE MODIFIED BRUCE TEST p < 0.01 Pre-op 3m 1y 3y 5y 7y 10y
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE RESULTS
      • The majority of pts experienced dramatic improvement in pulmonary hemodynamics after PEA
      • After PEA the decrease in pulmonary artery pressure is immediate (in O.R.) and associated with complete recovery of RV morphology (at discharge)
      • The functional results also show a progressive good recovery over a longer time (about years)
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE RESULTS
      • About 10-15% of our pts showed no statistically significant differences compared to pre-op or a persistent PH after PEA
      • About 5-10% of our pts showed a new increase in pulmonary pressure after PEA over time
      • The reason could be a secondary small vessel arteriopathy (Eisenmenger-type syndrome) in the non-obstructed segments of the lungs already present at the time of PEA
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CONCLUSION
      • Poor survival rate of untreated pts (10% 5-yrs survival if mPAP  50 mmHg), low mortality rate after PEA and good mid- and long- term results confirm PEA as the procedure of choice for operable CTEPH pts
      • The improvement of functional capacity strictly depends on the hemodynamic changes after PEA
      • When CTEPH is diagnosed, given the natural history of the disease, patients should be referred for surgery even when in NYHA functional class II
      • CTEPH pts should be referred early to Centres experienced in both PEA and Tx , to offer the best treatment and to achieve the best results
      • This strategy maximizes the use of scarce donor organs by offering, when feasible, a non-transplant option
      SURGICAL TREATMENT OF CTEPH: FROM TRANSPLANT TO CONSERVATIVE SURGERY CONCLUSION
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PULMONARY ENDARTERECTOMY IN THE ELDERLY EXTENSION OF SURGICAL CRITERIA
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE AGE
      • Apr 1994 – Dec 2002 -> 10.8 % (9/83)
      • Jan 2003 – June 2011 -> 31.8 % (87/274)
      Apr 1994 – June 2011 -> 357 PEAs 261 (73.0%) PEAs performed in patients < 70 years old 96 (27.0%) PEAs performed in patients ≥ 70 years old
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE AGE
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CUMULATIVE PROPORTION SURVIVING OF 261 vs. 96 PEAs 90.9  2.2 86.9  3.0 88.5  2.6 89.3  2.5 85.0  3.5 85.0  3.5 81.2  5.7 78.0  6.3 78.0  6.3
      • Age did not affect the efficacy of PEA: PVR dramatically and equally decreased in both groups
      • We never considered age as a contraindication to PEA
      • However, operative mortality and perioperative morbidity were higher in patients age 70 and older : Gram negative infections and reperfusion edema were more severe in the elderly
      PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CONCLUSIONS
      • Compared to young subjects, elderly patients had a higher prevalence of major preoperative comorbidities , were sicker at the time of operation and had a reduced functional reserve of their vital organs
      • However, this has to be expected in such patients:
        • age is a well identified independent risk factor in cardiac surgery
        • in elderly patients the immune system response is less effective
        • antibiotic therapy may lead to renal and hepatic impairment, increasing morbility and mortality
      PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE CONCLUSIONS
    • PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE PAVIA PULMONARY ENDARTERECTOMY GROUP CTEPH
      • Cardiac Surgery M Viganò, AM D’Armini, G Silvaggio, S Nicolardi, M Morsolini, G Mattiucci
      • Anestesiology M Maurelli, T Bianchi, R Veronesi, M Toscani, C Dezza,
      • E Milanesi, B Lusona, B Rossini
      • Cri tica l Care A Braschi, V Emmi, G Rodi, G Sala Gallini
      • F Capra Marzani, M Zanierato, F Mojoli
      • Cardiology L Oltrona Visconti, S Ghio, A Raisaro, L Scelsi, C Raineri
      • Respiratory Disease M Luisetti, I Cerveri, A Corsico
      • Radiology I R Dore
      • Radiology II F Zappoli Thyrion, P Quaretti, A Azzaretti, G Rodolico
      • Nuclear Medicine C Aprile
      • Reumatology C Montecucco, R Caporali
      • Thromboembolism F Piovella, M Barone, C Beltrametti
      • Pathology E Arbustini, M Grasso
      • General Rehabilitation E Dalla Toffola, L Petrucci
      • Pulmonary Rehabilitation C Fracchia, G Callegari
      • Biostatistics C Klersy