E. Demerouti, MD, MSc, PhDCardiologistOnassis Cardiac Surgery Center
A. Manginas, MD, PhD, FESC, FACCCardiologist, Director Cardiology and Interventional Cardiology DepartmentMediterraneo Hospital
ΕΛΛΗΝΙΚΗ ΚΑΡΔΙΟΛΟΓΙΚΗ ΕΤΑΙΡΕΙΑ
ΣΔΜΙΝΑΡΙΑ ΟΜΑΓΩΝ ΔΡΓΑΣΙΑΣΘΔΣΣΑΛΟΝΙΚΗ, 2012
CASE REPORT
1st patientMedical History
Male, 29 years old
• 2007: Pulmonary Embolism - 1st episode• 2008: Pulmonary Embolism – 2nd episode
Antiphospholipid syndrome Anticoagulation therapy with warfarin
• 2009: CTEPH diagnosis
1st patient
PERFUSION LUNG SCAN,
Chest CT
R.H.C. (2009)
RA mmHg 20
RV mmHg 110/20
PA mmHg 110/48/67
PCW mmHg 11
CI l/min/m² 2,6
PVR Wood U 12,4
1st patient
1st patient
PASP 110mmHg
Angiography1st PATIENT
„ L angiographie pulmonaire montre une
tres bonne forme d’HTPAP
post embolique
avec obstructions ostiales
au niveau des sous segmentaires des
deux cotes’.
Clinical assesement
WHO III II II
6MWD (m) 320 410 410
NT-proBNP 4200 3200 2900 (pg/ml, UL: )
Warfarin, Diuretics, Bosentan, Sildenafil & Inhaled Prostanoids
2009 2010 2011
1st PATIENT
1st Patient Successful PEA
SPECIMENJule 7th 2011
1st Patient before and after PEA
2011, March
WHO IV prostanoids, bosentan, sildenafil
acenocumarol
2011, August
WHO I
acenocumarol
1st PatientBefore and After PEA
BEFORE PEA
1 m AFTER PEA
6m AFTER PEA
1st Patient Before and After PEA
Before PEAPGmax 99 mmHg
PVR 12,4 WU
After PEAPGmax 36 mmHg
PVR 2 WU
2nd patient- History
Male, 47 years old
Smoker, Negative family history for cardiovascular disease
Αrterial Hypertension (ARB)
homozygosity for the variant of MTHFR (mutation 677C T)
CTEPH diagnosis
R.H.C. 2nd patient (Jan 2009)
RA: 17 mmHg
RV: 116/22 mmHg
PA: 116/53/79 mmHg
PCW: 14 mmHg
CI: 2,6 l/min/m²
PVR: 18,7 Wood U
2nd Case
2nd patient
Pulmonary Angiography(R)
2nd PATIENT
Pulmonary Angiography(L)
2nd PATIENT
2nd patient: PASP: 100mmHg
ECHOCARDIOGRAPHICSTUDY 2nd patient
Follow-up 2nd patient
02/2009 06/2009 01/2010
WHO II-III II II-III
PASP (mmHg) 90 100 110
NT-proBNP (pg/ml) 3818 2500 3500
6-MWT (m) 440 513 400
2nd patientPEA, Our pt expired 7 days after PEA
“Dear Dres. Demerouti and Manginas,
Mr. Χ unfortunately died 6 days after PEA due to right heart failure.
The operation was unsuccessful as there was only a very little amount of thromboembolic material in the PA segmental arteries.
We tried to stabilize his right heart function by venoarterial extracorporeal life support, but there was no chance to restore right heart function.
We believe that Mr. Χ had idiopathic PAHwith in situ PA thrombosis and not CTEPH.
We are all very sad about this poor outcome for his family”.
IPAHIn situ thrombosis
Well-recognized complication in patients with severe PH.
Circulation 1995; 91:741-745
Distinguishing pts with IPAH and in situ thrombosis from pts with a distal type of CTEPH or small-vessel arteriopathymay be impossible, and
There may be an overlap between both disorders.
Circulation 2006; 113: 2011-2020
PEA: Pulmonary
Thromboendarterectomy
ESC GUIDELINES, 2009
PERIOPERATIVE MORTALITY- FACTORSMORTALITY RATES: 4.4% - 24%•WHO IV class Ann Thorac Surg 2003; 76: 1457-64
•Age>70 y.o. Eur J Cardiothorac Surg 2000; 18:696-702
•Right Ventricular Failure-High RAP Ann thorac Cardiovasc Surg 2001; 7: 261-5
•Morbid obesity Clin Chest Med 2007; 255-269
•Duration of PH Circulation 1990; 81:1735-43
•Severity of PH: PVR> 13,75 WU, mPAP>50 mmHg Ann Thorac Surg 1996; 61:1788-92
•Comorbidities Clin Chest Med 2007; 255-269
Selection of patients for PEA: RHC & P. Angiography
PVR
Powerful prognostic indicator
PVR elevated disproportionately to the degree of Proximal disease as visualized by Pulmonary Angiography,
this should be considered in discussions of perioperative risks with the patient
PVR increased: arises from surgically accessible obstructions and from resistance arising from
small vessel arteriopathy
Clin Chest Med 2007; 255-269
Distal inoperable microvascular disease in CTEPH
Similarity/overlap with IPAH
Pulmonary Angiogram,Capillary phase
Circulation 2006;113: 2011-2020
Peripheral vesselsNot perfused, Histologically normal
High Intravascularpressures
PLEXIFORMLESIONS
Curr Probl Surg 2000; 37:165-252
ON THE BASIS OF PATHOLOGIC EVIDENCE, IT IS NOT CLEAR IF THOSE PATIENTSREPRESENT AN EXTREME OF SPECTRUM OF CTEPH OR IF THEY SHOULD BE CONSIDEREDAS HAVING IPAH WITH ADDITIONAL LOCAL SUBSEGMENTAL THROMBOSIS
Galie N, Kim N. Proc Ann Thorac Soc 2006; 3: 571-576
10-15% of operable pts suffer from persistent PHSemin Respir Crit Care Med 2009; 30: 2257-2264
PROPOSED ALGORITHM- THERAPEUTIC APPROACH
Circulation 2006; 113: 2011-2010
MULTIDISCIPLINARYDISCUSSIONBETWEEN CHEST PHYSICIANS, REDIOLOGISTSSURGEONS
AJR 2006; 144: 27-30
IPAH:Normal or patchy defects
CTEPH: Segmental, LARGE defects
Perfusion Lung SCAN:DD IPAH-CTEPH
May underestimateThe extent ofObstruction
Proc Am Thorac Soc 2006; 3: 571-576
DD: Tumors, PVOD, ArteritisAm J Resp Crit Care 2000;162:1974-8
Normal or low probability V/Q scan,Excludes CTEPHSensitivity: 90-100%Specificity: 94-100%
J Nucl Med 2007; 48: 1747-1757
Fedullo P et al. N Engl J Med 2001;345:1465-1472
PULMONARY ANGIOGRAPHYDD IPAH-CTEPH
Pouch defect
thrombus
Obstruction of flow
THEGOLDSTANDARD
Abrupt narrowing
Pitton M B et al. AJR 2006;187:128-134
BANDS
WEBS
PULMONARY ANGIOGRAPHY
ConventionalMRI
WEBS
WEBS
Berman M et al. MMCTS 2009;2009:mmcts.2008.003491
N Engl J Med 2001;344:644, JACC 2003; 42: 1982-1987
Thrombi eccentric,Endothelialized, central BUTIN PAH may existCENTRAL thrombi
No pulmonary Artery branches
Mosaic AttenuationHypo-hyperAttenuating areas
JACC 2003; 42: 1982-1987,
CHEST CTCTEPH
Bronchial ArteryCollateral Vessels
Thromboticmass
Web
Radiographics 2010; 30: 1753-1777
Chest CTIPAH
Focal Perivascular HyperattenuatingAreasIn a peripheral orPerihilar distribution
Radiographics 2010; 30: 1753-1777
1st patient, succesful PEAPVR 12,4 WU
2nd PATIENT
PVR 18,7 Wood U
CTEPHInternational Prospective Registry
Circulation 2011; 124: 1973-1981
Nonoperable:
118: Inaccessibility of the occlusions
25: Imbalance PVR-occlusions
6: PVR > 18.75 WU
Thromboembolic disorders more frequent in operable pts
16 countries, 26 European centers, 1 Canadian center
384 PEA
18 died in hospital (4.7%)
Comparison between the operable and nonoperable pt group after
3-year follow up data
Messages
CTEPH diagnosis needs careful evaluation
Evaluation for PEA is a difficult process
The indication is not clearly defined
PEA can only relieve the portion of PVR that is accessible and amenable to surgical intervention
The extent and type of microvascular disease in CTEPH have a strong influence in the likelihood of a successful outcome in PEA
A consensus among experts is needed to reassess the criteria for operability
CTEPH
Chronic ThromboEmbolic Pulmonary Hypertension is one of the important clinical variants of P.H., and
is the only curable form of the disease.
N Engl J Med 2008, 359:20.
A CENTRE EXPERTIZED IN PEA IS REQUIRED IN GREECE
Thank you for your attention
RHC – PA occlusion waveform analysis
Experimental
Partition of PVR into upstream and downstream components
Circulation 2004; 109: 18-22
Upstream Resistance
Downstream resistance
80%
50%
Rup% = 100 x (mPpa - Poccl) / (mPpa - Ppao).
Circulation. 2004;109:18-22.
Pulmonary artery occlusion waveform analysis
Patients with Rup values <60% appearto have the highest postoperative risk.
ΠΡΟΕΓΥΕΙΡΗΣΙΚΟ ΤΣΗΜΑ ΣΑΞΙΝΟΜΗΗ
Α Β C
Pulmonary
Angiography
+ + +
PVR dyn·s·cm 1100 1100
Rup> 60%
1100
Rup< 60%
Treatment PEA PEA PEA (>risk)
Or drugsPEA= Pulmonary endarterectomy
Rup= upstream resistance
Proc Am Thorac Soc. 2006;3:584-588
LUNG CTDD IPAH and CTEPH
CTEPH IPAH
THROMBI Intraluminal, webs, bands in several cases
central, periphery central
BRONCHIALARTERIES dilatation (73%) uncommon (14%)
SEGMENTALVESSELS variation in the size uncommon
LUNG HYPO-, HYPER-ATTENUATING AREAS segmental, subsegmental patchy hererogeneity
ANGIOGRAPHY MRI CT
SENSITIVITY
Thrombi 71%
Webs, bands 50%
Obstruction 83%
Radiology 2005; 236: 694
CTEPH
Precapillary PH IC
Multiple chronic/organized occlusive thrombi/emboli in the elastic pulmonary arteries IC
Referral to a centre expertised in Pulmonary Endarterectomy IIa
lndication, LOE
ESC Guidelines, Dana Point 2009
PEA
True Endarterectomy : Removal of organized thrombi, not
embolectomy The material is fibrotic and incorporated into
the native vascular wall. Identification of the pseudointima and
creation a dissection plane to adequately free the thrombotic residua from the central pulmonary vascular bed.
Circumferential dissection of the residua from the lobar, segmental and subsegmental vessels.
CTEPH: Progression of PH would result from progressive P Vascular Remodeling
Small vessel Disease
Concept proposed by Drs
Kenneth Moser & Nina Braunwald after Dr Braunwald‟s first PEA in Chicago, in 1971.
The patient had a „two compartment pulmonary vascular bed‟.
The open p. arteries had marked structural changes of chronic PH.
CTEPHSelection of pts for PEA
Identification of those who are unlikely to benefit is a particular challenge
PVR increased: arises from surgically accessible obstructions and from resistance arising from
small vessel arteriopathy
PEA relieves only the portion of PH from the accessible component
EARLY SURGICAL INTERVENTION is more acceptable.
Persistent PH despite satisfactory PEA in 10% of pts
Eur Respir J 2004; 23: 637-648
Radiographics 2010; 30: 1753-1777
PEA- Poor outcome
IPAH with in situ thrombosis
CTEPH with distal vasculopathy
Persistent PH despite satisfactory thromboendarterectomy in
10% of pts
Eur Respir J 2004; 23: 637-648
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