PAH related to congenital heart disease: PAH- 2014-05-10¢  PAH related to congenital heart disease:

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  • PAH related to congenital heart disease: PAH-CHD

    Kostas Dimopoulos

    Royal Brompton Hospital

    Imperial College London

    London, UK

  • PAH-CHD: same prevalence as

    iPAH UK National Audit of Pulmonary Hypertension 2011

    ... and many more

    unidentified or lost to

    follow-up

  • Large ventricular septal defect

    Infancy: Lungs flooded

    PVR = TPG PBF

    PBF>>CO

    (Qp/Qs>>1)

    TPG increases

    PBF increases

    PVR normal/mildly increased

    PBF

  • I

    Shear stress & stretch Vascular Remodeling

    Bidirectional or RL

    shunt

    PAH associated

    with L-R

    shunt

    Eisenmenger

    syndrome

    Endothelial dysfunction

    L-R

    R-L

    IV-VII III

    Shunt

    Histology

    PVR PVR

    Lanigan MJ, Chaney MA, Tissot C, Beghetti M, Dimopoulos K. J Cardiothorac Vasc Anesth. 2013

  • Interactive Questions

    In which patients can you diagnose PH on

    echo with confidence?

    – 1 Only Eisenmenger patients with a post

    tricuspid shunt (VSD, PDA)

    – 2 All PAH-CHD patients

    – 3 Any patient who has a good TR trace

  • Interactive Questions

    In which patients can you diagnose PH on

    echo with confidence?

    – 1 Only Eisenmenger patients with a post

    tricuspid shunt (VSD, PDA)

    – 2 All PAH-CHD patients

    – 3 Any patient who has a good TR trace

  • No cath is required for the diagnosis of Eisenmenger

    syndrome in post-tricuspid defects

    Large VSD

    Low velocity bidirectional shunt

    +

    = RV pressure ≈ LV pressure

    No PS

    +

    = Near-systemic PA pressures

    + Desaturation at rest or min efforts

    = Eisenmenger syndrome

    Unlike

    iPAH

  • IN ALL OTHER PATIENTS, THE

    DIAGNOSIS OF PH/PAH REQUIRES

    CARDIAC CATHETERISATION

  • Baim DS and Grossman W. Cardiac Catheterization, Angiography, and Intervention.

    SVR =

    Catheterisation essential for pretricuspid

    Eisenmenger and non Eisenmenger pts

    Ao - RA

    Qs

    PVR = PA - LA

    Qp

    Systemic vascular resistance

    Pulmonary vascular resistance

    http://www.med.umn.edu/intcardio/curriculum/modules/Hemodynamic/Hemodynamic_files/Hemodynamic.ppt

    L-R shunt No PVD

    L-R shunt Mild PVD

    R-L shunt Severe PVD

    Qp↑↑ Qp↑ Qp ↓

    PAP↑↑ PAP↑↑ PAP↑↑↑

    PVR=N PVR=↑ PVR=↑↑↑

    Do not use thermodilution

    PAPresssure not sufficient to define pulmonary vascular disease:

    Need PVResistance

  • Interactive Questions

    How many types of PAH-CHD are there

    according to the ESC PH Guidelines?

    – 1 One

    – 2 Two

    – 3 Three

    – 4 Four

    – 5 Five

  • Interactive Questions

    How many types of PAH-CHD are there

    according to the ESC PH Guidelines?

    – 1 One

    – 2 Two

    – 3 Three

    – 4 Four

    – 5 Five

  • Clinical classification of PAH-CHD Four different classes of PAH-CHD

    A. Eisenmenger’s syndrome (ES): shunt reversal

    L-R-shunts due to large defects leading to a severe increase in PVR and resulting in a

    reversed R-L or bidirectional shunt, resulting in cyanosis, high haemoglobin and multiple

    organ involvement.

    B. PAH associated with relevant L-R shunt (systemic-to-pulmonary)

    L-R shunt through moderate to large defects, with mild to moderate increase in PVR, with

    no cyanosis.

    C. PAH with clinically not relevant L-R shunt

    L-R shunt through small defects (usually VSD

  • Interactive Questions

    Of these 3 types of PAH-CHD, which could be

    amenable to repair of the defect?

    – 1 Groups B+C

    – 2 Groups A+C

    – 3 Groups A+B

    – 4 Group B

    – 5 All groups

    A. Eisenmenger’s syndrome (ES):

    shunt reversal

    B. B. PAH associated with relevant L-

    R shunt (systemic-to-pulmonary)

    C. C. PAH with clinically not relevant

    L-R shunt

  • Interactive Questions

    Of these 3 types of PAH-CHD, which could be

    amenable to repair of the defect?

    – 1 Groups B+C

    – 2 Groups A+C

    – 3 Groups A+B

    – 4 Group B

    – 5 All groups

    A. Eisenmenger’s syndrome (ES):

    shunt reversal

    B. B. PAH associated with relevant L-

    R shunt (systemic-to-pulmonary)

    C. C. PAH with clinically not relevant

    L-R shunt

  • “Treat and repair”

    IN 2014: THERE IS STILL NO EVIDENCE FOR TREATING WITH PAH THERAPIES

    AND REPAIRING PREVIOUSLY INOPERABLE DEFECTS.

  • NEVER CLOSE DEFECTS IN

    EISENMENGER PATIENTS!!!!

    Or any patient with established

    pulmonary vascular disease

  • The remarkable right ventricle of

    Eisenmenger syndrome

    Eisenmenger iPAH

  • However, not all CHD-PAH have

    preserved RV function

    Pretricuspid defects Complex defect

  • Clinical classification of PAH-CHD Four different classes of PAH-CHD

    A. Eisenmenger’s syndrome (ES): shunt reversal

    L-R-shunts due to large defects leading to a severe increase in PVR and resulting in a

    reversed R-L or bidirectional shunt, resulting in cyanosis, high haemoglobin and multiple

    organ involvement.

    B. PAH associated with relevant L-R shunt (systemic-to-pulmonary)

    L-R shunt through moderate to large defects, with mild to moderate increase in PVR, with

    no cyanosis.

    C. PAH with clinically not relevant L-R shunt

    L-R shunt through small defects (usually VSD

  • Repaired defects

    Why not to close defects in established pulmonary

    vascular disease

  • The extreme end of PAH-CHD

    Eisenmenger syndrome

  • Eisenmenger syndrome

    Victor Eisenmenger, 1897

    … The patient was a powerfully built man of 32 who gave

    a history of cyanosis and moderate breathlessness since

    infancy. He managed well until January 1894 when

    dyspnoea increase and oedema set in. Seven moths later he

    was admitted to the hospital in a state of heart failure.

    He improved with rest and digitalis, but collapsed and died

    suddenly on November 13 following a large haemoptysis”.

    Paul Wood, 1958

  • Scoliosis

    ↑ ↑ Perioperative risk

    Exercise intolerance Arrhythmias

    Thrombosis

    Heart failureBleeding

    Organ failure

    Hyperviscosity

    ↑ ↑ Pregnancy risk

    Disability

    ↓ QoL Sudden death

    Hepatic dysfunction

    Renal failure

    HyponatremiaTIA/CVA

    Syncope

    Gout

    Cholelithiasis

    Endocarditis

  • Dimopoulos et al. Circulation 2006

    Dimopoulos. In Crawford et al. Cardiology, 2009

    Exercise capacity in Eisenmenger

    patients

  • Kempny A et al. Eur Heart J 2011;eurheartj.ehr461

    cooking

  • Kempny A et al. Eur Heart J 2011;eurheartj.ehr461

    cooking

    Kempny A et al. Eur Heart J 2011;eurheartj.ehr461

    cooking

    Kempny A et al. Eur Heart J 2011;eurheartj.ehr461

    cooking

  • Perception of functional capacity in ES

    patients

    Early onset of symptoms

    Chronic adaptation of everyday

    activities to a lower intensity

    Patients underestimate their exercise

    limitation compared with objective

    measures of exercise tolerance

    Dimopoulos K, Giannakoulas et al. Curr Opin Cardiol 2008; 23:545-54.

    Who is functional

    class III? ATs

  • Interactive Questions

    What is differential cyanosis?

    – 1 Cyanosis at effort but not at rest

    – 2 Cyanosis in severe efforts only

    – 3 Cyanosis that occurs in women but not men

    – 4 Cyanosis that affects the lower body only

  • Interactive Questions

    What is differential cyanosis?

    – 1 Cyanosis at effort but not at rest

    – 2 Cyanosis in severe efforts only

    – 3 Cyanosis that occurs in women but not men

    – 4 Cyanosis that affects the lower body only

  • Differential cyanosis: Patent Ductus Arteriosus with severe PAH

    • Shunt: Bidirectional/R-L

    • PBF: Reduced

    • CO: Normal/reduced

    • Cyanosis: ++ differential

    I

    PVRPVR

    Bidirectional/RL shunt

    PAH associated

    with L-R shunt

    Eisenmenger

    syndrome

    L-R

    IV-VII III

    Easy Δ iPAH vs Eis PDA

    Always look at toes and measures sats

  • Interactive Questions

    What is secondary erythrocytosis?

    – 1 A compensatory increase in red cell count

    aimed at increasing oxygen delivery

    – 2 A malignant, extreme rise on RCC,

    inevitably leading to thromboembolic events

    – 3 An increase in RCC resulting in cyanosis

    and iron overload

  • Interactive Questions

    What is secondary erythrocytosis?