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    Interventional Catheterization

    to treat Adults with

    Congenital Heart DiseaseDanyal Khan, M.D, FACC, FAAP, FSCAI

    Pediatric Interventional Cardiologist

    Miami Children's Hospital

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    Congenital Heart Disease (CHD)

    Occurs in 1 in 120 newborns

    Commonest congenital problem

    Operative mortality is about 2% at themajor centers

    Approximately 800,000 children in USA

    with CHD

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    Adults with Congenital Heart

    Disease (ACHD)

    10% of CHD is diagnosed in adulthood

    In USA, there are more adults than

    children with CHD

    1 million adults in USA with CHD

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    Historical Perspective

    Heart surgery-1940s

    Open heart surgery-1960s

    First therapeutic catheterization-1953

    Last Decade

    Advanced fetal diagnosis

    Explosion of interventional cath procedures

    Introduction of minimally invasive surgical techniques Near extinction of the inoperable patient

    Cardiac transplantation and immunosuppression

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    Surgery

    Interventional Catheterization

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    Types of CHD

    ACYANOTIC HEART

    DISEASE

    Pulse oximetry

    normal Left to Right Shunt

    Usually not

    symptomatic at birth

    CYANOTIC HEART

    DISEASE

    Pulse Oximetry may

    be low Right-to-Left Shunt

    Usually presents at

    young age

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    ACYANOTIC

    HEART DISEASE

    ASD

    VSD

    PDA

    AV Canal

    PS

    Coarctation of the Aorta

    LVOT Obstruction

    CYANOTIC

    HEART DISEASE

    Tetralogy of Fallot

    Transposition of the greatarteries

    Tricuspid Atresia

    Total anomalouspulmonary venous return

    Tricuspid Valve, Ebsteinsanomaly

    Pulmonary Atresia/VSD

    Pulmonary Atresia/ IntactSeptum

    Hypoplastic Left HeartSyndrome

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    Adult CHD

    Atrial Septal Defect (ASD)

    Patent Foramen Ovale (PFO)

    Coarctation Pulmonary Regurgitation

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    Atrial Septal Defect

    5-10 % of Congenital Heart Disease

    Male : Female ratio = 1:2

    Types Secundum (50-70%)

    Primum

    Sinus Venosus Coronary Sinus ASD

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    Atrial Septal Defect

    Left to Right shunt

    Asymptomatic in childhood

    Causes dilation of Right Atrium & Ventricle Causes symptoms in adulthood

    Chest pain

    Palpitations Decreased Exercise tolerance

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    Diagram

    S d ASD

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    AORTA

    Deficient antero-

    superior rim

    Central defect

    Deficient inferior

    rim

    Secundum ASD

    Anatomic Sub-types

    Multi-fenestrated

    Deficient posterior

    rim

    Multiple

    Multiple

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    Case - ASD

    52 year old lady with c/o SOB & Palpitations

    EKG incomplete RBBB

    CXR Cardiomegaly

    Echo- Severe Pulm HTN, Marked RA & RV

    dilation, Severe TR

    O/E- Sats 94%, decreased to 84% with exertion

    Fixed split & loud S2, Flow Murmur Early clubbing & cyanosis

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    Cardiac MRI

    Pulmonary artery dilation

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    Heterotaxy

    Stomach

    Transverse

    Liver

    No IVC

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    Management

    Diagnostic Cath

    Treated with Oxygen via nasal cannula &

    Revatio (sildenafil)

    After 3 months repeat cath

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    Amplatzer Device Closure

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    Cardiac Cath

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    Interrupted Inferior Vena Cava

    IVC

    IVC

    SVC

    RA

    Azygous

    Vein

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    IntraCardiac Echo

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    Hepatic Access

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    ICE Catheter

    Wire

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    ASD - Echo

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    ASD IntraCardiac Echo

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    ASD Device

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    Left disc deployment

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    Left Disc

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    Right Disc deployment

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    Right Disc

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    Device released

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    18mm Amplatzer ASD Device

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    Hepatic Vein- occluded with

    Vascular Plug

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    PFO

    In Fetal circulation, presence of a PFO is

    essential

    PFO usually closes within the first two weeks

    of life Persistant PFO is present in more than 25%

    of adults

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    No. 1 cause of serious disability and morbidity

    Sacco RL, et al. Stroke1997

    0

    5

    10

    15

    20

    25

    30

    Heart Cancer Stroke

    Causes of Death

    STROKE3rd Leading Cause of Death

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    Ann Neurol. 1989;25:382-90

    STROKE VICTIMS

    43%

    PREVALENCE OF PFO IN CRYPTOGENIC STROKE

    Prevalence

    ofP

    FO(%)

    Prevalence

    ofP

    FO(%)

    Cryptogenic Stroke

    Cryptogenic Stroke

    Cruz-Gonzalez I, 2009

    Expert Rev Cardiovasc Ther

    PFO Rates

    Cryptogenic Stroke

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    2.50.451.10000

    26

    7.1

    3.25.4

    0.601.90

    0

    10

    20

    30

    Lausanne

    (1996)

    Mas2

    001:(PFO

    only)

    DeCa

    stro2

    000

    Mas2

    001:(PFO

    andA

    SA)

    PICS

    S(2002)

    Sievert(

    2002)

    Cujec

    (surgery)

    Devuyst1

    966(

    surgery)

    Lock

    (1998)

    Meier

    (2000)

    USMultice

    nter(2002)

    Palac

    ios(2002)

    Sievert(

    2002)

    %o

    fRecurre

    ntStokeRates

    MEDICAL THERAPY DEVICE CLOSURE

    PFO Closure vs. Medical Therapy

    REGISTRY DATA

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    7.07

    2.71

    0

    2

    4

    6

    8

    Medical

    Therapy

    Device

    Closure

    %A

    djusted1YrStroke-TIA

    PFO Closure vs. Medical Therapy

    Landzberg Heart 2004

    20 studies n=2250

    5.55

    4.86

    2.95

    0

    2

    4

    6

    8

    Surgery Medical

    therapy

    Device

    26 studies n=2534

    Homma Circulation 2005

    5.2

    1.3

    0

    2

    4

    6

    8

    Medical

    Therapy

    Device

    Closure

    Whrle Lancet 2006

    20 studies n=3014

    Events/100patientyears

    %1YearRecurrentStrokeorTIA

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    PFO & Stroke

    PFO are more common in patients who

    have strokes or TIAs

    No RCTrial has found that closure of

    PFOs reduces the incidence of stroke or

    death

    AAN, AHA, ACC state that the benefit of

    device closure therapy remains unknown

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    Right-to-Left Shunt (PFO)

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    To close or not to close ?

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    (My) Indications for PFO Closure

    Cryptogenic stroke

    Rule out other obvious cause (Lipid profile,

    BP, CRP, Carotid Doppler US,

    HyperCoagulability w-up)

    PFO with bidirectional or Right-to-Left

    Shunt (on bubble study)

    Age less than 55 yrs

    Look for Atrial Septal aneurysm

    ASD/PFO O l i D i t

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    ASD/PFO Occlusion Devices at

    MCH

    CardioSEAL

    Amplatzer

    HELEX

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    Case # 1

    14 year old girl, who was in a car accident

    and suffered a left tibial fracture

    Patient was neurologically normal after the

    accident

    36 hours later had a stroke

    TTE PFO. Bubble study positive

    Confirmed with transcranial doppler

    PFO closed with Sideris Button device

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    Case # 2 (AS)

    17 year old girl with c/o palpitations and

    has had two episodes of TIA (right sided

    hemiparesis + dysarthria)

    Holter non sustained wide QRS

    tachycardia

    EPStudy no inducible Vent Tachycardia

    TTE Bubble study positive

    Hypercoagulability w-up: negative

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    Helex

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    Balloon Sizing

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    Balloon Sizing

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    Helex implanted

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    Helex released

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    ICE Image - Helex

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    Case # 3 (CA)

    55 yr old gentleman.

    h/o two episodes of TIA

    Carotid u/s normal

    Hypercoag w-up Negative

    No HTN or HyperLipidemia

    TTE: Atrial Septal Aneurysm + PFO(positive bubble study)

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    PFO

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    Wire across PFO

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    Balloon Sizing 14-16mm

    CardioSEAL septal occluder

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    CardioSEAL septal occluder

    28mm

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    CardioSeal snared

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    Amplatzer Septal Occluder 16mm

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    Amplatzer Septal Occluder 20mm

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    Both disks deployed

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    Final Result

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    Coarctation of Aorta

    8-10% of Congenital Heart Disease

    Male:Female ratio = 2:1

    Turners syndrome 30% incidence

    85% incidence of Bicuspid Aortic Valve

    Most patients present in infancy &

    childhood

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    Coarctation of Aorta (CoAo)

    First described 1760 by Morgagni

    Currently 3 modes of treatment

    Surgery infants Balloon angioplasty children

    Stent implantation - adults

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    Coarctation in Adults

    Presentation

    Hypertension resistant to therapy

    CXR rib notching due to collaterals

    Diagnosis

    BP difference between arm & leg

    Echo

    MRI/CT

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    Case - Coarctation

    21 year old with h/o Coarctaton

    Repaired in infancy

    c/o Hypertension 140/90

    Echo coarctation with 30 mm Hg

    gradient

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    Cath Angiogram

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    Cath Angiogram

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    Cath Angiogram

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    Cath Angiogram

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    Cath Angiograms

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    Cath Angiograms

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    Cath Angiograms

    C

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    Cath Angiograms

    Aneurysm

    C d S

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    Covered Stent

    Fi l R lt

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    Final Result

    C

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    Case

    22 year old lady, h/o Tetralogy of Fallot

    VSD closure + RV to PA homograft

    Has had Aortic Valve replacement

    23mm St Jude Valve

    c/o decreased exercise tolerance

    Echo

    Severe pulmonary regurgitation

    Right ventricular dilation & dysfunction

    P l I ffi i

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    Pulmonary Insufficiency -

    The Problem Pulmonary insufficiency is common after rightventricular outflow tract reconstruction Tetralogy Fallot, Pulmonary atresia, Truncus

    arteriosus Results in RV volume load

    Exercise intolerance

    Arrhythmia

    Heart failure Traditional Rx

    Surgical valve placement

    Th S l ti

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    The Solution

    Pulmonary Valve Implantation

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    Pulmonary Valve ImplantationThe Solution

    Bonhoeffer P, et al, England

    Bovine (cow) jugular

    venous valve

    CP stent mounted 18-22 mm balloon

    Femoral vein access

    Currently 22Fr system

    Transcatheter Pulmonary Valve

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    Transcatheter Pulmonary Valve

    Implantation

    RV

    PA

    M l d V l

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    Melody Valve

    E bl D li S t

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    Ensemble Delivery System

    M l d V l D l t

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    Melody Valve Deployment

    E h P l R it ti

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    Echo Pulmonary Regurgitation

    C th A i

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    Cath Angiograms

    Cath Angiograms

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    Cath Angiograms

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    Cath Angiograms

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    Cath Angiograms

    Balloon Angioplasty

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    Balloon Angioplasty

    Stenting the Pulmonary Valve

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    Stenting the Pulmonary Valve

    Stent

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    Stent

    Melody Valve

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    Melody Valve

    Melody Valve inner balloon

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    Melody Valve inner balloon

    Melody Valve outer balloon

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    Melody Valve outer balloon

    Melody Valve in place

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    Melody Valve in place

    No Pulmonary Regurgitation

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    No Pulmonary Regurgitation

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    Thank you

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