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cteph, terapia chirurgica e medica
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UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
Andrea M D’Armini, MD, FCCP
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE “CTEPH”
Cardiac SurgeryUniversity of Pavia School of Medicine
Foundation I.R.C.C.S. “San Matteo” HospitalPavia, Italy
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
BACKGROUND
Chronic Thromboembolic Pulmonary Hypertension
CTEPH
Acute Pulmonary Embolism
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
Vascular lung diseases suitable for transplantation are:
• Idiopathic Pulmonary Hypertension
• Eisenmenger’s Syndrome
• CTEPH
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
VASCULAR LUNG DISEASES
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• Eisenmenger’s Syndrome
• Idiophatic Pulmonary Hypertension
1990 2011
Optimal Medical Therapy
BEFORE
HL/L Transplantation
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
VASCULAR LUNG DISEASES
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• 10089 HEART TRANSPLANTS
• 309 LUNG TRANSPLANTS
• 39 HEART-LUNG TRANSPLANTS
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
THORACIC TRANSPLANTATION1357 TRANSPLANTS (17/11/1985 – 09/06/2011)
1357 INTRATHORACIC TRANSPLANTS
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
348 LUNG AND HEART-LUNG TRANSPLANTS• 79 VASCULAR LUNG DISEASES
• 269 PARENCHYMAL LUNG DISEASES
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
THORACIC TRANSPLANTATION1357 TRANSPLANTS (17/11/1985 – 09/06/2011)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
U. G. PRE DLTx U. G. 1° POST DLTx
DLTx for FAMILIAL PULMONARY HYPERTENSION
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
THORACIC TRANSPLANTATION1357 TRANSPLANTS (17/11/1985 – 09/06/2011)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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 TRANSPLANTATION1357 TRANSPLANTS (17/11/1985 – 09/06/2011)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
HLTx for EISENMENGER’S SYNDROME
M. P. PRE HLTx M. P. 1° POST HLTx
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
THORACIC TRANSPLANTATION1357 TRANSPLANTS (17/11/1985 – 09/06/2011)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
HLTx for EISENMENGER’S SYNDROME
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/m2
Pulmonary Vascular Resistance 45 dyne*sec*cm-5
POST-OPERATIVE RIGHT HEART CATHETERIZATION
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
THORACIC TRANSPLANTATION1357 TRANSPLANTS (17/11/1985 – 09/06/2011)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
1990 2011
From Transplant
to Conservative Surgery (PEA)
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
SURGICAL TREATMENT OF CTEPHPAVIA EXPERIENCE - 357 PEAs
• CTEPH(NO SPECIFIC DRUGS ARE CURRENTLY APPROVED FOR CTEPH)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
COAGULATIVE DISORDERS
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
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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 % – –
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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 %
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CLINIC
The clinical indication changes substantially
according to the different surgical treatment of CTEPH
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• 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
TRANSPLANT WINDOW
TOO LATETOO EARLY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CLINIC
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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 asPROXIMAL
DISTAL
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PROXIMAL LESIONS
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
M.B. – 62 yrs M – Jul 2001 – PEA #64
Perfusion and ventilation scan
Pulmonary angiogram
Hemodynamic
mPAP 67CI 1.6PVR 1766
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
DISTAL LESIONS
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
S.S. – 31 yrs M – Sep 2002
Perfusion and ventilation scan
Pulmonary angiogram
Hemodynamic
mPAP 50CI 1.8PVR 1120
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
P.B. – 60 yrs M – Jun 2002
Perfusion and ventilation scintigraphy
Pulmonary angiography
CT scan
Hemodynamic
mPAP 28CI 1.9PVR 645
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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 resultsare known to be excellent incase of successful operation
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REFERENCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• National referral program
• Begin: April 1994
• To date: 357 PEAs performed
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
OUR PROGRAM
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
55101
124
15
1916
28
7
47
215
18 153
6
14
Pts coming from outside Italy- Greece 1- Kosovo 1- Uganda 1
2
2
Pavia
≤ 10 pts
11 – 20 pts
≥ 21 pts
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PATIENTS’ REFERRALOF 357 PEAs
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
AMOUNT OF PATIENTS
0
10
20
30
40
50
60
70
1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011
Patients
65 PEAs in almost 8 yrs
65 PEAs in 1 yr
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
AMOUNT OF PATIENTSNEW EVALUATIONS
54
108
0
20
40
60
80
100
120
2004 2010
Pat
ien
ts
+ 100%
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
AMOUNT OF PATIENTSDIAGNOSTIC ACCURACY
34
82
20 260
10
20
30
40
50
60
70
80
90
2004 2010
Pat
ien
ts
IPCTE
Other
63%
76%
+ 13%
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
AMOUNT OF PATIENTSOPERABILITY RATE
25
73
9 90
10
20
30
40
50
60
70
80
2004 2010
Pat
ien
ts
Operable
Inoperable
74%
89%
+ 15%
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
AMOUNT OF PATIENTSPEAs PERFORMED
22
65
0
10
20
30
40
50
60
70
2004 2010
PE
As
+ 195 %
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
• 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PAVIA CTEPH PROGRAMJanuary, 1st – December, 31st 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PAVIA CTEPH PROGRAMJanuary, 1st – June, 9th 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
08-MAR-1991
First HLTx for CTEPH
11-APR-1994
First PEA
28-JUL-2003
First PEA in patient listed for DLTx
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
SURGICAL TREATMENT OF CTEPH
25-DEC-1995
First DLTx for CTEPH
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
Tx for CTEPH
0
1
2
3
4
5
6
7
8
1991-1995 1996-2000 2001-2005 2006-2010
MO
RE DIA
GN
OSI
SM
ORE DISTAL PEAs
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
TRANSPLANT FOR CTEPH 18 / 79
PATIENTS DIAGNOSED WITH CTEPH
0
10
20
30
40
50
60
70
1991
1992
1993
1994
1995
1996
1997
1998
1999
2000
2001
2002
2003
2004
2005
2006
2007
2008
2009
2010
PEAs PERFORMED
0
10
20
30
40
50
60
1991
1992
1993
1994
1995
1996
1997
1998
1999
2000
2001
2002
2003
2004
2005
2006
2007
2008
2009
2010
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PEA POPULATIONOF 357 PEAs
Age 56 16 (11 84) years
Gender 171 M – 186 F
NYHA class 33 II – 165 III – 159 IV
Length III / IV 19 23 months
Urgent / Emergent 75 / 357
Oxygen therapy 174 / 357
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
RIGHT HEART CATHETERIZATIONOF 357 PEAs
Mean SD Range
mPAP 47 13 17 88 mmHg
CI 1.9 0.6 1.1 4.1 l/min/m2
PVR 1149 535 191 3938 dynes*sec*cm-5
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDIOGRAPHYOF 357 PEAs
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDIOGRAPHYOF 357 PEAs
• Tricuspid regurgitation Absent 2.7%
Mild18.9%
Moderate29.7%
Severe48.7%
• Abnormal interventricular septal motion Yes91.9%
No 8.1%
• Inferior vena cava collapsibility Yes40.5%
No59.5%
78.4%
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDIOGRAPHYOF 357 PEAs
Mean SD Range
LV-EDD 41 8 23 56 mm
LV-EDV 71 31 14 157 ml
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDIOGRAPHY
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIAC MAGNETIC RESONANCEOF 244 PEA
Mean SD Range
RV-EDV 113 40 45 200 ml
RV-ESV 80 33 28 160 ml
RV-EF 33 11 10 57 %
RV-FWT 8.4 ± 1.3 6.5 – 11 mm
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIAC MAGNETIC RESONANCEOF 244 PEA
Mean SD Range
LV-EDV 88 29 38 156 ml
LV-ESV 50 21 20 109 ml
LV-EF 43 13 11 72 %
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIAC MAGNETIC RESONANCE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
LUNG FUNCTIONOF 357 PEAs
Mean SD Range
FVC 91 18 39 148 %
FEV-1 84 18 40 131 %
DLCO 65 15 26 100 %
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ARTERIAL BLOOD GASESOF 357 PEAs
Mean SD Range
Pa O2 65 10 43 97 mmHg
Pa CO2 31 7 24 43 mmHg
O2-sat 93 3 84 98 %
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
MODIFIED BRUCE TESTOF 357 PEAs
Steps Walking distance
No (Pa O2 < 60) 36.9% 103 ± 160 (0 – 852) meters
Step 0 - ½ 56.8%
Step 1 - 2 4.5%
Step 3 - 4 1.8%
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
Peak exercise
No (ECG, advanced NYHA IV, other) 16.3%
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 200 PEAs
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIOPULMONARY EXERCISE TESTINGOF 200 PEAs
Mean SD Range
Peak-DP 16037 4822 5600 30600 mmHg*FC
Peak-VO2 9.9 3.6 3.0 29.4 ml/min/kg
Peak-Exe 50 22 15 160 watt
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• Intra-wall dissection• Peripheral extension• Explore all branches
J Thorac Cardiovasc Surg 1993;106:116-27
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PULMONARY ENDARTERECTOMY
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
SURGICAL INSTRUMENT
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PULMONARY ENDARTERECTOMY
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN 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%)
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
TYPICAL SURGICAL SPECIMENS
P.A. – 66 yrs M – Jun 2001 – PEA #60mPAP 50 25 (-50%)CO 2.6 4.4 (+69%)PVR 1385 364 (-74%)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
DISTAL LESIONS
JAMIESON TYPE IIILEARNING CURVE
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
JAMIESON TYPE I vs. TYPE II vs. TYPE III
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%)
B.A. - 43 yrs F - May 2009 - PEA #233mPAP 49 19 (-61%)CO 3.3 5.0 (+52%)PVR 1067 224 (-79%)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
JAMIESON TYPE III
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%)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN 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
JAMIESON TYPE III
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
OUT OF PROPORTION PH ?
Pre-operative Pulmonary Angiogram
Pre-operative 64-HRCT
B.R.A. - 72 yrs FmPAP 44CO 2.9RVEF 28PVR 1159
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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 #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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN 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
OUT OF PROPORTION PH ?
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
JAMIESON TYPE III
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN 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%
60%
70%
80%
90%
100%P
erc
ent
age
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CUMULATIVE PROPORTION SURVIVINGOF 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%)
89.21.9 87.12.
2 86.52.2
85.62.4 84.52.
6 83.12.9 81.63.
2 79.23.9
79.23.9
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
0 12 24 36 48 60 72 84 96 108 120 132 144 156
M onths after PEA
0.0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0
Percentage
1 3 3
8 6 .8 ± 2 .6
8 4 .7 ± 2 .8
8 3 .8 ± 2 .9 8 3 .8 ± 2 .9 8 3 .8 ± 2 .9 8 3 .8 ± 2 .9
1 1 3 9 3 5 4 3 4 1 4
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CUMULATIVE PROPORTION SURVIVINGSURGERY vs. MEDICAL THERAPY
Riedel M. Chest 1982;81(2):151-8.
D’Armini A.M. Ital Heart J 2005;6(10):861-8.
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
SURGICAL TREATMENT OF CTEPH: FROM TRANSPLANT TO CONSERVATIVE SURGERY
CUMULATIVE PROPORTION SURVIVING45 PTS ON WAITING LIST FOR TRANSPLANT IN CTEPH
SURVIVALWAITING LIST FOR TRANSPLANT IN CTEPH
0 365 730 1095 1460 1825 2190 2555 2920 3285 3650 4015 4380
Time (days)
0.0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0
Cum
ulat
ive
Prop
ortio
n Su
rviv
al
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
SURGICAL TREATMENT OF CTEPH: FROM TRANSPLANT TO CONSERVATIVE SURGERY
CUMULATIVE PROPORTION SURVIVING18 TRANSPLANTS IN CTEPH
SURVIVALTRANSPLANT IN CTEPH
0 365 730 1095 1460 1825 2190 2555 2920 3285 3650 4015 4380 4745 5110 5475
Time (days)
0.0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0
Cum
ulat
ive
Prop
ortio
n Su
rviv
al
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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)
– 3th month
– yearly for 5 years
– 7th, 10th and 15th year (10 controls)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
NYHA FUNCTIONAL CLASS
NYHA Functional Class
0
10
20
30
40
50
60
70
80
90
100
Pre-op 3 mesi 1 anno 3 anni 5 anni 7 anni 10 anni
Follow-up
% p
ati
ents
I -I I
I I I -IV
Pre-op 3m 1y 3y 5y 7y 10y
pp < 0.01 < 0.01
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
mean PULMONARY ARTERY PRESSURE
mean Pulmonary Arterial Pressure
0
10
20
30
40
50
60
Pre-op Dimiss 3 mesi 1 anno 3 anni 5 anni 7 anni 10 anni
Follow-up
mm
Hg
pp < 0.01 < 0.01
Pre-op disch 3m 1y 3y 5y 7y 10y
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PULMONARY VASCULAR RESISTANCES
Pulmonary Vascular Resistances
0
200
400
600
800
1000
1200
Pre-op Dimiss 3 mesi 1 anno 3 anni 5 anni 7 anni 10 anni
Follow-up
dyne*se
c*cm
-5
pp < 0.01 < 0.01
Pre-op disch 3m 1y 3y 5y 7y 10y
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDIOGRAPHYBefore PEA
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDOGRAPHYFirst echo after PEA – POD #9
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ECHOCARDIOGRAPHY3-months FUP after PEA
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIAC MAGNETIC RESONANCEBefore PEA First CMR after PEA – POD #6
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CARDIAC MAGNETIC RESONANCE4-years FUP after PEAFirst CMR after PEA – POD #6
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
REVERSE RIGHT VENTRICULAR REMODELING
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
ARTERIAL OXYGEN PARTIAL PRESSURE
Arterial Oxygen Partial Pressure
0
10
20
30
40
50
60
70
80
90
100
Pre-op 3 mesi 1 anno 3 anni 5 anni 7 anni 10 anni
Follow-up
mm
Hg
pp < 0.01 < 0.01
Pre-op 3m 1y 3y 5y 7y 10y
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
MODIFIED BRUCE TEST
Modified Bruce Test
0
100
200
300
400
500
600
700
800
900
1000
Pre-op 3 mesi 1 anno 3 anni 5 anni 7 anni 10 anni
Follow-up
met
ers
pp < 0.01 < 0.01
Pre-op 3m 1y 3y 5y 7y 10y
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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)
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PULMONARY ENDARTERECTOMYIN THE ELDERLY
EXTENSION OFSURGICAL CRITERIA
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
AGE
0%
20%
40%
60%
80%
100%
1994-1998 1999-2003 2004-2008 2009-2011
>= 70 yrs
< 70 yrs
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
CUMULATIVE PROPORTION SURVIVING
OF 261 vs. 96 PEAs
< 70 yrs >= 70 yrs0 12 24 36 48 60 72 84 96 108 120 132 144 156 168 180
Months after PEA
0.0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0P
erc
en
tag
e
90.92.2
86.93.088.52.689.32.5
85.03.5 85.03.581.25.7
78.06.3 78.06.3
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
• 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
UNIVERSITY OF PAVIA SCHOOL OF MEDICINE - SAN MATTEO HOSPITAL - PAVIA - ITALY
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCE
PAVIA PULMONARY ENDARTERECTOMY GROUPCTEPH
• 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• Critical 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