Case Report - CASE REPORT. 1st patient Medical History Male, 29 years old ... 6MWD (m) 320 410 410...

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Transcript of Case Report - CASE REPORT. 1st patient Medical History Male, 29 years old ... 6MWD (m) 320 410 410...

  • E. Demerouti, MD, MSc, PhD Cardiologist Onassis Cardiac Surgery Center

    A. Manginas, MD, PhD, FESC, FACC Cardiologist, Director Cardiology and Interventional Cardiology Department Mediterraneo Hospital

    ΕΛΛΗΝΙΚΗ ΚΑΡΔΙΟΛΟΓΙΚΗ ΕΤΑΙΡΕΙΑ

    ΣΔΜΙΝΑΡΙΑ ΟΜΑΓΩΝ ΔΡΓΑΣΙΑΣ ΘΔΣΣΑΛΟΝΙΚΗ, 2012

    CASE REPORT

  • 1st patient Medical 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

  • Angiography 1st 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

    SPECIMEN Jule 7th 2011

  • 1st Patient before and after PEA

    2011, March

    WHO IV prostanoids, bosentan, sildenafil

    acenocumarol

    2011, August

    WHO I

    acenocumarol

  • 1st Patient Before and After PEA

    BEFORE PEA

    1 m AFTER PEA

    6m AFTER PEA

  • 1st Patient Before and After PEA

    Before PEA PGmax 99 mmHg

    PVR 12,4 WU

    After PEA PGmax 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

    ECHOCARDIOGRAPHIC STUDY 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 patient PEA, 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 PAH with in situ PA thrombosis and not CTEPH.

    We are all very sad about this poor outcome for his family”.

  • IPAH In 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 arteriopathy may be impossible, and

     There may be an overlap between both disorders.

    Circulation 2006; 113: 2011-2020

  • PEA: Pulmonary Thromboendarterectomy

    ESC GUIDELINES, 2009

    PERIOPERATIVE MORTALITY- FACTORS MORTALITY 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 vessels Not perfused, Histologically normal

    High Intravascular pressures

    PLEXIFORM LESIONS

  • Curr Probl Surg 2000; 37:165-252

    ON THE BASIS OF PATHOLOGIC EVIDENCE, IT IS NOT CLEAR IF THOSE PATIENTS REPRESENT AN EXTREME OF SPECTRUM OF CTEPH OR IF THEY SHOULD BE CONSIDERED AS 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 PH Semin Respir Crit Care Med 2009; 30: 2257-2264

  • PROPOSED ALGORITHM- THERAPEUTIC APPROACH

    Circulation 2006; 113: 2011-2010

    MULTIDISCIPLINARY DISCUSSION BETWEEN CHEST PHYSICIANS, REDIOLOGISTS SURGEONS

  • AJR 2006; 144: 27-30

    IPAH:Normal or patchy defects

    CTEPH: Segmental, LARGE defects

    Perfusion Lung SCAN: DD IPAH-CTEPH

    May underestimate The extent of Obstruction

    Proc Am Thorac Soc 2006; 3: 571-576

    DD: Tumors, PVOD, Arteritis Am J Resp Crit Care 2000;162:1974-8

    Normal or low probability V/Q scan, Excludes CTEPH Sensitivity: 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 ANGIOGRAPHY DD IPAH-CTEPH

    Pouch defect

    thrombus

    Obstruction of flow

    THE GOLD STANDARD

    Abrupt narrowing

  • Pitton M B et al. AJR 2006;187:128-134

    BANDS

    WEBS

    PULMONARY ANGIOGRAPHY

    Conventional MRI

    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 BUT IN PAH may exist CENTRAL thrombi

    No pulmonary Artery branches

    Mosaic Attenuation Hypo-hyper Attenuating areas

    JACC 2003; 42: 1982-1987,

    CHEST CT CTEPH

    Bronchial Artery Collateral Vessels

    Thrombotic mass

    Web

    Radiographics 2010; 30: 1753-1777

  • Chest CT IPAH

    Focal Perivascular Hyperattenuating Areas In a peripheral or Perihilar distribution

    Radiographics 2010; 30: 1753-1777

  • 1st patient, succesful PEA PVR 12,4 WU

  • 2nd PATIENT

    PVR 18,7 Wood U

  • CTEPH International Prospective Registry

    Circulation 2011; 124: 1973-1981

    Nonoperable: 118: Inaccessibility of

    the occlusions 25: Imbalance PVR-

    occlusions 6: PVR > 18.75 WU

    Thromboemb olic 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 cli