Interventional Catheterization to treat Adults with Congenital Heart Disease

Danyal Khan, M.D, FACC, FAAP, FSCAI Pediatric Interventional Cardiologist Miami Children's Hospital

Congenital Heart Disease (CHD)

Occurs in 1 in 120 newborns Commonest congenital problem Operative mortality is about 2% at the major centers Approximately 800,000 children in USA with CHD

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

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

Surgery

Interventional Catheterization

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

ACYANOTIC HEART DISEASE

ASD VSD PDA AV Canal PS Coarctation of the Aorta LVOT Obstruction

CYANOTIC HEART DISEASE

Tetralogy of Fallot Transposition of the great arteries Tricuspid Atresia Total anomalous pulmonary venous return Tricuspid Valve, Ebsteins anomaly Pulmonary Atresia/VSD Pulmonary Atresia/ Intact Septum Hypoplastic Left Heart Syndrome

Adult CHD
Atrial Septal Defect (ASD) Patent Foramen Ovale (PFO) Coarctation Pulmonary Regurgitation

Atrial Septal Defect

5-10 % of Congenital Heart Disease Male : Female ratio = 1:2 Types
Secundum (50-70%) Primum Sinus Venosus Coronary Sinus ASD

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

Diagram

Secundum ASD Anatomic Sub-types

AORTA

Deficient anterosuperior rim

Multiple Deficient posterior rim Central defect Multi-fenestrated Multiple Deficient inferior rim

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

Cardiac MRI
Pulmonary artery dilation

Heterotaxy

No IVC

Transverse Liver Stomach

Management
Diagnostic Cath Treated with Oxygen via nasal cannula & Revatio (sildenafil) After 3 months repeat cath

Amplatzer Device Closure

Cardiac Cath

Interrupted Inferior Vena Cava

SVC
Azygous Vein

IVC

RA IVC

IntraCardiac Echo

Hepatic Access

ICE Catheter

Wire

ASD - Echo

ASD IntraCardiac Echo

ASD Device

Left disc deployment

Left Disc

Right Disc deployment

Right Disc

Device released

18mm Amplatzer ASD Device

Hepatic Vein- occluded with Vascular Plug

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

STROKE 3rd Leading Cause of Death

30 25 20 15 10 5 0 Heart Cancer Stroke

Causes of Death

No. 1 cause of serious disability and morbidity

Sacco RL, et al. Stroke 1997

PREVALENCE OF PFO IN CRYPTOGENIC STROKE

PFO Rates STROKE VICTIMS Cryptogenic Stroke
Prevalence of PFO (%)
Prevalence of PFO (%)

Cryptogenic Stroke Non- Cryptogenic Stroke Control

Cryptogen

Non- Cryp Control

43%

Ann Neurol. 1989;25:382-90

Cruz-Gonzalez POSITIVE RESULTS I, 2009 Expert Rev Cardiovasc Ther

POSITIVE RESULTS

NEGATIVE RESULTS

M as 20
10 20 30 0

La us a nn e (1 9 96 )
1.90 0.60

M as 20 D 01 :
5.4

01 : (P FO

% of Recurrent Stoke Rates

MEDICAL THERAPY

3.2 7.1 26 0 0 0 0 1.1 0.45

PFO Closure vs. Medical Therapy

DEVICE CLOSURE

eC o as nly) tro (P FO 20 an 00 d PI AS C SS A) (2 Si 00 ev 2) er t( C D 20 u ev 02 uy jec (s st ) ur 19 66 ger y) (s ur ge Lo ry ck ) (1 U 99 M S ei 8) M er ul (2 tic 00 en 0) te r( Pa 20 la 02 ci os ) (2 Si 00 ev 2) er t( 20 02 )

2.5

REGISTRY DATA

PFO Closure vs. Medical Therapy

Landzberg Heart 2004
20 studies n=2250
8
7.07
8

Homma Circulation 2005

26 studies n=2534

Whrle Lancet 2006

20 studies n=3014
8

% Adjusted 1 Yr Stroke-TIA

Events /100 patient years

%1 Year Recurrent Stroke or TIA

5.55 4.86

5.2

4
2.71

2.95
2

1.3

0 Medical Therapy Device Closure

Surgery

Medical therapy

Device

Medical Device Therapy Closure

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

Right-to-Left Shunt (PFO)

To close or not to close ?

(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 Occlusion Devices at MCH

CardioSEAL

Amplatzer

HELEX

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

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

Helex

Balloon Sizing

Balloon Sizing

Helex implanted

Helex released

ICE Image - Helex

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)

PFO

Wire across PFO

Balloon Sizing 14-16mm

CardioSEAL septal occluder 28mm

CardioSeal snared

Amplatzer Septal Occluder 16mm

Amplatzer Septal Occluder 20mm

Both disks deployed

Final Result

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

Coarctation of Aorta (CoAo)

First described 1760 by Morgagni Currently 3 modes of treatment
Surgery infants Balloon angioplasty children Stent implantation - adults

Coarctation in Adults
Presentation
Hypertension resistant to therapy CXR rib notching due to collaterals

Diagnosis
BP difference between arm & leg Echo MRI/CT

Case - Coarctation
21 year old with h/o Coarctaton Repaired in infancy c/o Hypertension 140/90 Echo coarctation with 30 mm Hg gradient

Cath Angiogram

Cath Angiogram

Cath Angiogram

Cath Angiogram

Cath Angiograms

Cath Angiograms

Cath Angiograms

Cath Angiograms
Aneurysm

Covered Stent

Final Result

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

Pulmonary Insufficiency The Problem

Pulmonary insufficiency is common after right ventricular outflow tract reconstruction
Tetralogy Fallot, Pulmonary atresia, Truncus arteriosus

Results in RV volume load

Exercise intolerance Arrhythmia Heart failure

Traditional Rx
Surgical valve placement

The Solution

Pulmonary Valve Implantation

The Solution
Bovine (cow) jugular venous valve CP stent mounted 18-22 mm balloon Femoral vein access Currently 22Fr system

Bonhoeffer P, et al, England

Transcatheter Pulmonary Valve Implantation

PA

RV

Melody Valve

Ensemble Delivery System

Melody Valve Deployment

Echo Pulmonary Regurgitation

Cath Angiograms

Cath Angiograms

Sizing

Cath Angiograms

Balloon Angioplasty

Stenting the Pulmonary Valve

Stent

Melody Valve

Melody Valve inner balloon

Melody Valve outer balloon

Melody Valve in place

No Pulmonary Regurgitation

Echo post Melody

Thank you