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Page 1: Case Report - Livemedia.grstatic.livemedia.gr/HCS/cfiles/OE_RGNC_180212_022_Demerouti.pdf · CASE REPORT. 1st patient Medical History Male, 29 years old ... 6MWD (m) 320 410 410 NT-proBNP

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

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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

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1st patient

PERFUSION LUNG SCAN,

Chest CT

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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

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1st patient

PASP 110mmHg

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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’.

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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

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1st Patient Successful PEA

SPECIMENJule 7th 2011

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1st Patient before and after PEA

2011, March

WHO IV prostanoids, bosentan, sildenafil

acenocumarol

2011, August

WHO I

acenocumarol

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1st PatientBefore and After PEA

BEFORE PEA

1 m AFTER PEA

6m AFTER PEA

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1st Patient Before and After PEA

Before PEAPGmax 99 mmHg

PVR 12,4 WU

After PEAPGmax 36 mmHg

PVR 2 WU

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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

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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

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2nd Case

2nd patient

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Pulmonary Angiography(R)

2nd PATIENT

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Pulmonary Angiography(L)

2nd PATIENT

Page 17: Case Report - Livemedia.grstatic.livemedia.gr/HCS/cfiles/OE_RGNC_180212_022_Demerouti.pdf · CASE REPORT. 1st patient Medical History Male, 29 years old ... 6MWD (m) 320 410 410 NT-proBNP

2nd patient: PASP: 100mmHg

ECHOCARDIOGRAPHICSTUDY 2nd patient

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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

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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”.

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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

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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

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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

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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

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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

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PROPOSED ALGORITHM- THERAPEUTIC APPROACH

Circulation 2006; 113: 2011-2010

MULTIDISCIPLINARYDISCUSSIONBETWEEN CHEST PHYSICIANS, REDIOLOGISTSSURGEONS

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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

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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

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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

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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

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Chest CTIPAH

Focal Perivascular HyperattenuatingAreasIn a peripheral orPerihilar distribution

Radiographics 2010; 30: 1753-1777

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1st patient, succesful PEAPVR 12,4 WU

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2nd PATIENT

PVR 18,7 Wood U

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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

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384 PEA

18 died in hospital (4.7%)

Comparison between the operable and nonoperable pt group after

3-year follow up data

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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

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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

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Thank you for your attention

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RHC – PA occlusion waveform analysis

Experimental

Partition of PVR into upstream and downstream components

Circulation 2004; 109: 18-22

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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.

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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

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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

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ANGIOGRAPHY MRI CT

SENSITIVITY

Thrombi 71%

Webs, bands 50%

Obstruction 83%

Radiology 2005; 236: 694

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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

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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.

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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.

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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

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Radiographics 2010; 30: 1753-1777

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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