Morsolini Marco Torino 13° Convegno Patologia Immune E Malattie Orfane 21 23 Gennaio 2010
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Transcript of Morsolini Marco Torino 13° Convegno Patologia Immune E Malattie Orfane 21 23 Gennaio 2010
Andrea M D’Armini, MD, FCCPMarco Morsolini, MD, PhD
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Division of Cardiac SurgeryUniversity of Pavia School of MedicineSt. Matteo HospitalPavia - Italy
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
INTRODUCTION
• Chronic thromboembolic pulmonary hypertension (CTEPH)represents the only type of pulmonary hypertension surgically treatable, in the majority of cases, without transplant
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• This life-saving conservative surgery is called pulmonary endarterectomy (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
PEA vs. LTx
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• 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
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
EPIDEMIOLOGY
• Epidemiologic data: in Italy ≈ 65.000 cases / yearof acute symptomatic pulmonary embolism (PE)
• Prevalence of CTEPHin pts surviving an acute PE(≈ 80 %) is calculated between 0.5% – 3.8%
→ up to 2.000 new cases / year
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
→ 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 Surg2000; 37:165-252
• Fedullo PF, Auger WR, Kerr KM, Rubin LJ. Chronic thromboembolic pulmonary hypertension. N Engl J Med2001; 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 Med2004; 350:2257-64
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CURRENT SITUATION
• CTEPH is still under-diagnosedand nowadays only few physicians are aware of the surgical procedurecalled PEA
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• For all these reasons about 6000 PEAhave been performed worldwide so far with ≈ 40 %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)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
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 andinterscapular olosystolic murmur
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• 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 DISORDERSOF LAST 185 PEAs
DISORDER % PTS MEAN ± SD RANGE
HYPERHOMOCYSTEINEMIA (µmol/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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
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 DISORDERSOF LAST 185 PEAs
DISORDER % PTS MEAN ± SD RANGE
Anti-Nuclear Antibodies (ANA) 23.4 % – –
Lupus Anticoagulans (LAC) 19.4 % – –
Anti-Cardiolipin Antibodies (ACA) IgG 20.2 % 56.3 ± 40.3 10.3 – 121.0
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
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 MUTATIONSOF LAST 185 PEAs
MUTATION % PTS
C677T MTHFR (HOMOCYSTEINEMIA) HETEROZYGOSIS 50.8 %
HOMOZYGOSIS 19.4 %
TOTAL 70.2 %
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
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 %
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
INDICATIONS FOR SURGERY
• The indications for the surgical treatmentof these patients are based on
CLINICHEMODYNAMIC
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• The indications for the type of surgeryare based on
ANATOMY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CLINIC
• Patients must be in NYHA functional class III or IV
• Full anticoagulation for at least 3 months
• Some Authors (we too) recently have performed PEA
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• Some Authors (we too) recently have performed PEA even in NYHA class IIpatients, given the natural history of the disease
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
HEMODYNAMIC
• Pulmonary hypertension (mPAP > 25 mmHg)
• Causing low cardiac output
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• Resulting in calculated pulmonary vascular resistances (PVR) > 300 dyne*sec*cm-5
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ANATOMY
• The surgical treatment depends on the localization of the lesions in the pulmonary arterial branches
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• Lesions can be classified as PROXIMALDISTAL
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PROXIMAL LESIONS
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
M.B. – 62 yrs M – Jul 2001 – PEA #64
Perfusion and ventilation scan
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Pulmonary angiogram
Hemodynamic
mPAP 67CI 1.6PVR 1766
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
DISTAL LESIONS
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
S.S. – 31 yrs M – Sep 2002
Perfusion and ventilation scan
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Pulmonary angiogram
Hemodynamic
mPAP 50CI 1.8PVR 1120
• National referral program
• Begin: April 1994
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
OUR PROGRAM
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• Begin: April 1994
• To date: 266 PEAs performed
4079
103
12
1412
19 26
4
Pts coming from outside Italy- Greece 1- Uganda 1
2
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PATIENTS’ REFERRALOF 266 PEAs
Pavia
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
6
164
11 101
5
11
1
≤ 10 pts
11 – 20 pts
≥ 21 pts
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PATIENTS’ AMOUNT
• Apr ‘94 → Jan ‘10
• 266 PEAs(5 redo: 26, 40, 56, 86 and 174 months after the first PEA)
• In the majority of
40
50
60
Pa
tient
s
54 PEAs in 1 yr
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• In the majority of pts (250/266) an ICF was placed before PEA
• Lifelong anticoagulation therapy was prescribed 0
10
20
30
1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010
Pa
tient
s
54 PEAs in 7 yrs
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PAVIA CTEPH PROGRAMJanuary, 1st - December, 31st 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
• PROXIMAL LESIONS (25 pts)- 22 PEAs- 2 pts refused
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
- 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 %
- 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 PROGRAMJanuary, 1st - December, 31st 2009 →→→→ 121 pts
NEW EVALUATIONS (78 pts) PEAs FOLLOW-UP (43 pts)
CONFIRMED (70 pts - 90%) OTHER DIAGNOSIS (8 pts - 10%)
• PROXIMAL LESIONS (62 pts)- 54 PEAs- 1 pt waiting for PEA
• RECENT EMBOLIZATION (4 pts)
• MINIMAL CTE LESIONS WITHOUT PH (4 pts)- 4 medical therapy
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
OPERABILITY RATE 89 %
- 1 pt waiting for PEA- 4 pts refused PEA- 2 pts with “too old” lesions (pulmonary artery
retraction)- 1 pt general condition too compromised
• DISTAL LESIONS (8 pts)- 8 medical therapy: 5 too old for DLTx
3 too early for DLTx
• RECENT EMBOLIZATION (4 pts)- 3 medical therapy- 1 surgical embolectomy
CHEST STUDIES: THE PAVIA EXPERIENCE
MAIN WORLD PEA CENTERS
Bad Nauheim, Germany≈60 PEAs / year
MORE THAN ONE PROGRAM
Cambridge, UK≈80 PEAs / year
NATIONAL REFERRAL PROGRAM BY LAW
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
San Diego, California, USA≈120 PEAs / year
NATIONAL REFERRAL PROGRAM FOR EXCELLENCE Paris, France
≈100 PEAs / yearNATIONAL REFERRAL PROGRAM
FOR EXCELLENCE
Pavia, Italy≈50 PEAs / year
MORE THAN ONE PROGRAM
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PEA POPULATIONOF 266 PEAs
Age 56 ± 16 (11 − 84) years
Gender 134 M – 132 F
NYHA class 15 II – 122 III – 129 IV
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
NYHA class 15 II – 122 III – 129 IV
Length III / IV 19 ± 23 months
Urgent / Emergent 64 / 266
Oxygen therapy 131 / 266
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
RIGHT HEART CATHETERIZATIONOF 266 PEAs
Mean ± SD Range
mPAP 47 ± 13 17 − 88 mmHg
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
CI 1.9 ± 0.6 1.1 − 4.1 l/min/m2
PVR 1149 ± 535 191 − 3938 dynes*sec*cm-5
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDIOGRAPHY
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIAC MAGNETIC RESONANCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ARTERIAL BLOOD GASESOF 266 PEAs
Mean ± SD Range
Pa O2 65 ± 10 43 − 97 mmHg
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Pa CO2 31 ± 7 24 − 43 mmHg
O2-sat 93 ± 3 84 − 98 %
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
MODIFIED BRUCE TESTOF 266 PEAs
Steps Walking distance
No (Pa O2 < 60) 36.9% 103 ± 160 (0 – 852) meters
Step 0 - ½ 56.8%
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Step 0 - ½ 56.8%
Step 1 - 2 4.5%
Step 3 - 4 1.8%
Peak exercise
No (∆ECG, advanced NYHA IV, other) 16.3%
Watts ≤25 12.0%
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIOPULMONARY EXERCISE TESTINGOF 157 PEAs
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Watts ≤25 12.0%
Watts >25 / ≤50 50.0%
Watts >50 / ≤75 15.2%
Watts >75 6.5%
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIOPULMONARY EXERCISE TESTINGOF 157 PEAs
Mean ± SD Range
Peak-DP 16037 ± 4822 5600 − 30600 mmHg*FC
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Peak-VO2 9.9 ± 3.6 3.0 − 29.4 ml/min/kg
Peak-Exe 50 ± 22 15 − 160 watt
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PULMONARY ENDARTERECTOMY
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
TYPICAL SURGICAL SPECIMENS
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
E.L. – 38 yrs M – Dec 1999 – PEA #42mPAP 43 →→→→ 20 (-53%)CO 3.3 →→→→ 6.9 (+109%)PVR 994 →→→→ 220 (-78%)
P.A. – 66 yrs M – Jun 2001 – PEA #60mPAP 50 →→→→ 25 (-50%)CO 2.6 →→→→ 4.4 (+69%)PVR 1385 →→→→ 364 (-74%)
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
JAMIESON TYPE I vs. TYPE II vs. TYPE III
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
L.M.E.L. - 65 yrs M - Oct 2004 - PEA #119mPAP 39 →→→→ 19 (-51%)CO 4.4 →→→→ 5.4 (+23%)PVR 665 →→→→ 222 (-66%)
G.A.C. - 52 yrs F - Jul 2003 - PEA #96mPAP 48 →→→→ 27 (-44%)CO 2.1 →→→→ 4.2 (+100%)PVR 1638 →→→→ 381 (-77%)
S.F. - 48 yrs F - Mar 2009 - PEA #221mPAP 44 →→→→ 29 (-34%)CO 2.9 →→→→ 3.5 (+21%)PVR 1131 →→→→ 549 (-51%)
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
JAMIESON TYPE III
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
B.A. - 43 yrs F - May 2009 - PEA #233mPAP 49 →→→→ 19 (-61%)CO 3.3 →→→→ 5.0 (+52%)RVEF 16 →→→→ 35 (+119%)PVR 1067 →→→→ 224 (-79%)
Pre-operative Pulmonary Angiogram
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
JAMIESON TYPE III
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Pre-operative Pulmonary Angiogram
Pre-operative 64-HRCT
F.C. - 33 yrs F - Apr 2009 - PEA #225mPAP 52 →→→→ 20 (-62%)CO 4.6 →→→→ 4.7 (+2%)RVEF 32 →→→→ 41 (+28%)PVR 870 →→→→ 255 (-71%)
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
OUT OF PROPORTION PH ?
Pre-operative Pulmonary Angiogram
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Pre-operative Pulmonary Angiogram
Pre-operative 64-HRCT
B.R.A. - 72 yrs FmPAP 44CO 2.9RVEF 28PVR 1159
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
JAMIESON TYPE III
Pre-operative Pulmonary Angiogram
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Pre-operative Pulmonary Angiogram
Pre-operative 64-HRCT
B.R.A. - 72 yrs F - Mar 2009 - PEA #222mPAP 44 →→→→ 33 (-25%)CO 2.9 →→→→ 4.9 (+69%)RVEF 28 →→→→ 34 (+21%)PVR 1159 →→→→ 457 (-61%)
B.R.A. - 72 yrs FmPAP 44CO 2.9RVEF 28PVR 1159
Pre-operative Pulmonary Angiogram
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
OUT OF PROPORTION PH ?
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Pre-operative Pulmonary Angiogram
Pre-operative 64-HRCT
G.G. - 62 yrs FmPAP 51CO 2.6RVEF 19PVR 1415
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
JAMIESON TYPE III
Pre-operative Pulmonary Angiogram
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
Pre-operative Pulmonary Angiogram
Pre-operative 64-HRCT
G.G. - 62 yrs F - Sep 2009 - PEA #240mPAP 51 →→→→ 27 (-47%)CO 2.6 →→→→ 4.0 (+54%)RVEF 19 →→→→ 24 (+26%)PVR 1415 →→→→ 460 (-68%)
G.G. - 62 yrs FmPAP 51CO 2.6RVEF 19PVR 1415
60%
70%
80%
90%
100%
Per
cent
age
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CUMULATIVE PROPORTION SURVIVINGOF 266 PEAs
187 144 125 108 91 71 54 42 33 30 22 14 7 3 2
89.2±1.987.1±2.2
86.5±2.2 85.6±2.484.5±2.6
83.1±2.981.6±3.2
79.2±3.9 79.2±3.9
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
0 12 24 36 48 60 72 84 96 108 120 132 144 156 168 180
Months after PEA
0%
10%
20%
30%
40%
50%
Per
cent
age
Operative mortalityGlobal 24/266 (9.0%)NYHA II 0/15 (0.0%)NYHA III 5/122 (4.1%)NYHA IV 19/129 (14.7%)
Jan 08 - Jan 10 5/92 (5.4%)
0.4
0.5
0.6
0.7
0.8
0.9
1.0
Per
cent
age
133
86 .8±2.6
84.7±2.8
83.8±2.9 83.8±2.9 83.8±2.9 83.8±2.9
113 93 54 34 14
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CUMULATIVE PROPORTION SURVIVINGSURGERY vs. MEDICAL THERAPY
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
0 12 24 36 48 60 72 84 96 108 120 132 144 156
Months after PEA
0.0
0.1
0.2
0.3
Riedel M. Chest 1982;81(2):151-8.
D’Armini A.M. Ital Heart J 2005;6(10):861-8.
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
FOLLOW -UP
• Very few data are available in the literature on mid and long term cardiopulmonary function, particularly on exertion, and on clinical benefits after PEA
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
D’Armini AM, Zanotti G, Ghio S, Magrini G, Pozzi M, Scelsi L, Meloni G, Klersy C, Viganò M. Reverse right ventricular remodelingafter pulmonary endarterectomy. J Thorac Cardiovasc Surg2007; 133:162-8
Corsico AG, D’Armini AM, Cerveri I, Klersy C, Ansaldo E, Niniano R, Gatto E, Monterosso C, Morsolini M, Nicolardi S, Tramontin C, Pozzi E, Viganò M. Long-term outcome after pulmonary endarterectomy. Am J Respir Crit Care Med2008;178(4):419-24
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
tolerance are excluded because pts are to close to the surgical procedure)
– 3th month
– yearly for 5 years
– 7th, 10th and 15th year (10 controls)
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
FOLLOW -UP DATA
• Eleven of 240 pts (4.6%) refused to participate to the study since the beginning (2/4 pts referred from outside Italy) or at various intervals
• For these pts we obtained only data on survival and NYHA
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• For these pts we obtained only data on survival and NYHA class on a phone-call basis
• All the other pts (95.4%) have actively participated to the FUP study
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
NYHA FUNCTIONAL CLASS
NYHA Functional Class
60
70
80
90
100
% patients
I-II
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
0
10
20
30
40
50
Pre-op 3 mesi 1 anno 3 anni 5 anni 7 anni 10 anni
Follow-up
% patients
I-II
III-IV
Pre-op 3m 1y 3y 5y 7y 10y
pp < 0.01< 0.01
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
mean PULMONARY ARTERY PRESSURE
mean Pulmonary Arterial Pressure
40
50
60
mmHg
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
0
10
20
30
Pre-op Dimiss 3 mesi 1 anno 3 anni 5 anni 7 anni 10 anni
Follow-up
mmHg
pp < 0.01< 0.01
Pre-op disch 3m 1y 3y 5y 7y 10y
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PULMONARY VASCULAR RESISTANCES
Pulmonary Vascular Resistances
800
1000
1200
dyne*sec*cm-5
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
0
200
400
600
Pre-op Dimiss 3 mesi 1 anno 3 anni 5 anni 7 anni 10 anni
Follow-up
dyne*sec*cm-5
pp < 0.01< 0.01
Pre-op disch 3m 1y 3y 5y 7y 10y
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDIOGRAPHYBefore
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDOGRAPHYFirst control – POD #9
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDIOGRAPHYThree-month control
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIAC MAGNETIC RESONANCEBefore First control – POD #6
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIAC MAGNETIC RESONANCEFirst control – POD #6 Four-year control
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: REVERSE RIGHT VENTRICULAR REMODELING
REVERSE RIGHT VENTRICULAR REMODELING
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
BRAIN-TYPE NATRIURETIC PEPTIDEBNP TIME COURSE AFTER PEA
Livelli plasmatici di BNP
350.0
400.0
450.0
SERUM BNP LEVELS
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
0.0
50.0
100.0
150.0
200.0
250.0
300.0
PRE-OP DIMISSIONE 3 MESI 1 ANNO 2 ANNI
pg/m
l
PRE-OP DISCHARGE 3 MONTHS 1 YEAR 2 YEARS
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ARTERIAL OXYGEN PARTIAL PRESSURE
Arterial Oxygen Partial Pressure
60
70
80
90
100
mmHg
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
0
10
20
30
40
50
Pre-op 3 mesi 1 anno 3 anni 5 anni 7 anni 10 anni
Follow-up
mmHg
pp < 0.01< 0.01
Pre-op 3m 1y 3y 5y 7y 10y
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
MODIFIED BRUCE TEST
Modified Bruce Test
600
700
800
900
1000
meters
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
0
100
200
300
400
500
600
Pre-op 3 mesi 1 anno 3 anni 5 anni 7 anni 10 anni
Follow-up
meters
pp < 0.01< 0.01
Pre-op 3m 1y 3y 5y 7y 10y
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
RESULTS
• In expert Centers mortality ranges between 5% and 12% and technical failure is below 8%
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• Early hemodynamic results are known to be excellent in case of successful operation
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)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
RV morphology (at discharge)
• The functional results also show a progressive good recovery over a longer time (about years)
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• April 1994 – October 2008
• 204 PEAs performed
• 184 patients received a complete pre-operative
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• 184 patients received a complete pre-operative screening for thrombofilia
• Group A (HAPT– high titre > 10 U/ml) 28 pts
• Group B (LAPT– low titre > 10 U/ml) 156 pts
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PATIENTS’ MANAGEMENT
• Accurate NIRS monitoring during circulatory arrests
• Attention even to minor neurological dysfunctions
• Careful anticoagulation with higher target INR
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• Careful anticoagulation with higher target INR
• Inferior vena cava filter positioning
• Accurate post-operative follow-up
RESULTS OF PULMONARY ENDARTERECTOMY: THE PAVIA EXPE RIENCE
PAVIA PULMONARY ENDARTERECTOMY GROUP
• Cardiac Surgery M Viganò, AM D’Armini, C Monterosso, G Silvaggio,
S Nicolardi, M Morsolini, D Berwick, G Mattiucci
• Anestesiology M Maurelli, T Bianchi, R Veronesi, M Toscani
MA Villani, E Milanesi, B Lusona, M Gerletti
• Cri tical Care A Braschi, V Emmi, G Rodi, G Sala Gallini
F Capra Marzani, F Mojoli
• Cardiology L Oltrona Visconti, S Ghio, A Raisaro, L Scelsi, C Raineri
• Respiratory Disease E Pozzi, I Cerveri, A Corsico
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - ST. MATTEO HOSPITAL - PAVIA - ITALY
• 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 U Magrini, E Arbustini, M Grasso
• General Rehabilitation E Dalla Toffola, L Petrucci
• Pulmonary Rehabilitation C Fracchia, G Callegari
• Biostatistics C Klersy