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Comprehensive Echocardiographic Assessment of Adults with Congenital Heart Disease

Echocardiography of Complex Congenital Heart Disease

Naser M. Ammash, M.D., FACC

Frank Cetta, M.D., FACC, FASE

Disclosure Summary To CME Participants

As a provider accredited by ACCME, College of Medicine, Mayo Clinic (Mayo School of CPD) must ensure balance, independence, objectivity and scientific rigor in its educational activities. Course Director(s), Planning Committee Members, Faculty, and all others who are in a position to control the content of this educational activity are required to disclose all relevant financial relationships with any commercial interest related to the subject matter of the educational activity. Safeguards against commercial bias have been put in place. Faculty also will disclose any off label and/or investigational use of pharmaceuticals or instruments discussed in their presentation. Disclosure of this information will be published in course materials so those participants in the activity may formulate their own judgments regarding the presentation.

Disclosure Summary

Listed below are individuals with control of the content of this program who have disclosed

Relevant financial relationship(s) with industry

Listed below are individuals with control of the content of this program who have disclosed

No relevant financial relationship(s) with industry

Gina A. Anderson, B.A

Naser M. Ammash, M.D., FACC

Frank Cetta, M.D., FACC, FASE

I do intend to discuss off-label/investigative uses(s) of the following commercial products(s)/device(s):

Objectives

• Identify the hemodynamic issues that impact adults with various complex congenital heart diseases

• Review long-term outcome of various complex congenital heart diseases after surgical repair

• Report the impact of novel valve repair techniques for patients with complex congenital heart diseases

Distribution of Age at Death in Patients With Congenital Heart Disease in 1987-1988 and 2004-2005

051015202530

Khairy et al: JACC 56:1149, 2010

Age at death (years)

Proportion of all deaths (%)

1987-1988

Distribution of Age at Death in Patients With Congenital Heart Disease in 1987-1988 and 2004-2005

051015202530

Khairy et al: JACC 56:1149, 2010

Age at death (years)

Proportion of all deaths (%)

1987-1988

0 5 10 15 20 25 30

2004-2005

Tetralogy of Fallot

Pink tetralogy Classic tetralogy Pulmonary atresia

Blalock-Taussig Shunt

Right common carotid artery

Right subclavian artery

Right pulmonary artery

Pulmonary trunk

Brachiocephalic trunk (innominate artery)

Surgical Repair of TOF

Residua and sequelae are common

Late morbidity and mortality

26-Year-Old Repaired TOF in 1981 Dyspnea, MR Noted Pre-Partum Evaluation in 2005

29 mm mosaic porcine valve

Survival Without Reoperation Following TOF Repair Mayo Clinic 1955-1960, n=163

0 5 10 15 20 25 30

Murphy, 1993

PR noted in 80% of patients

PR has detrimental long-term effect on RV size and function

PR is most common indication for re-operation

Pulmonary Regurgitation After TOR Repair Determinants

• Residual PV abnormality (bicuspid in 50%)

• Pulmonary valvotomy/valvectomy

• Extensive ventriculotomy/infundibulectomy

• Trans-annular patch repair

• Pulmonary annulus size

• Peripheral pulmonary artery stenosis

• Acquired CV/pulmonary diseases Pulmonary HTN

• Shunts, HTN, CAD, COPD/asthma, OSA

Ammash et al: CHD, 2007

Hemodynamic Substrate in for VT and SCD 465 Patients Following TOF Repair

Echo Within 12 Months

RVSP (>60 mm Hg) TR (≥ moderate) PR (≥ moderate)

Gatzoulis et al: Lancet 356:975, 2000

Patients

Arrhythmia-free

Pulmonary Regurgitation Severity Color Doppler

• Mild: PR noted in RVOT not PA

• Moderate: PR noted in the main PA

• Severe: PR noted in the branch PAs

Pulmonary Regurgitation Severity PW or CW Doppler

• Rapid decrease throughout diastole

• Return to baseline before end of diastole if low PA diastolic pressure

• High EDPR suggests pulmonary HTN

Pulmonary Regurgitation Severity PW or CW Doppler

Pulmonary Regurgitation Severity PR Index

Li et al: AHJ 147:165, 2004

PR index <0.77 identified severe PR

Predictive accuracy 95%

Pulmonary Regurgitation Severity Pressure Half Time

Silversides et al: JASE 16:1057, 2003

P ½t <100 msec identified severe PR:

Sensitivity 76%, specificity 94%

What to do?

When to intervene?

Pulmonary Regurgitation After TOF Repair

• PR tolerant for many years – subtle on PR

• Lack of symptoms doesn’t reflect RV dysfunction

• Degree of adaption

• Long standing PR

• ↑ RVEDV

• TV annular dilation

• TV regurgitation

• ↓ RV dysfunction

• ↓ FAC

Optimal Timing of Intervention in PR

0 10 20

Severe PR RV enlargement

RV dysfunction

Symptoms

Time ?

Pulmonary Regurgitation After TOF Repair Indications for Intervention

• Symptoms, decline in exercise capacity

• Moderate-Severe or progressive RVE or dysfunction

• Symptomatic or sustained atrial arrhythmias

• Spontaneous VT or inducible VT

• QRS prolongation (>180 msec)

• LV dysfunction (usually after RV dysfunction)

• RVOT obstruction: MIG >50 mm Hg or RVSP 70% systole

• Residual shunt, significant TR

• Early LV dysfunction Ammash et al: CHD, 2007

TOF repair

RVOT complications

Arrhythmias

Residual VSD

AV and TV regurgiation

RV, LV dysfunction

Pulmonary hypertension

Endocarditis

Echo Examination Post TOF Repair Comprehensive

• Level and severity of pulmonary stenosis

• Severity of pulmonary and TV regurgitation

• RV and PA systolic pressures

• RV size and function

• Residual atrial or ventricular septal defect

• LV size and function

• Ascending aorta dilation/AR

RV Dysfunction After TOF Repair Visual Estimate

Selected Indices of RV Contractility Functional parameters Normal value Load dependence

RVEF (%) 61±7 (45-76)

>40-45 +++

RVFAC (%) >32 +++

TAPSE (mm ) >15 +++

Small annular (cm/s) >12 +++

Strain

Basal: 19±6

Mid: 27±6

Apical: 32±6

Strain rate (s-1)

Basal: 1.50±0.41

Mid: 1.72±0.27

Apical: 2.04±0.41

RVMPI 0.28±0.04 ++

dP/dt max (mm Hg/s) 100-250 ++

PR Post TOF Repair MRI

Normal RVEDV ≤115 mL/m2

Normal RVESV ≤47 mL/m2

RVEDV Before and After PVR in TOF MRI Study in 17 Patients

• Presence of either

• RVEDV >170 cc/m2 or

• RVESV >85 cc/m2

Therrien: AJC, 2005

No normalization

of RV volumes

150 < RVEDV <170

RV volume ↓ a mean of 28% RVEF does not change Oosterhof: Circ 116, 2007

RV Dysfunction After TOF Repair Diastolic Dysfunction

43-Year-Old Lady Status-Post TOF Repair at Age 14 VT Status-Post RFA 8 Years Ago, Now DOE

RVSP = 56 + 10

= 66 mmHg

44-Year-Old Repaired TOF With Dyspnea

44-Year-Old Repaired TOF With Dypnea

PASP = 66 – Peak PV grad

= 66 – 10 = 56 mm Hg

Pulmonary HTN in r-TOF

• Prior systemic to pulmonary shunt

• Distal pulmonary stenosis

• Residual shunt

• LV dysfunction (systolic, diastolic)

• Associated MV disease

• Sleep apnea

• Chronic lung disease

43-Year-Old Lady Status-Post TOP Repair at Age 14 VT Status-Post RFA 8 Years Ago, Now DOE

Moderate – Severe left PS from prior Lt BT

43-Year-Old Lady Status-Post TOP Repair at Age 14

Hybrid repair: Lt PA stent + Pulmonary Valve Replacement

Pulmonary Regurgitation After TOF Repair

• Important determinant of morbidity/mortality

• Dyspnea, RVE/D, arrhythmia, LVD, SCD

• Modified approach of repairing TOF

• Early identification/evaluation by expert

• Timing of intervention can be challenging

• PR assessment is only 1 component of comprehensive Echo examination

Tetralogy of Fallot Anomalous CA

Anomalous CAD in 3-7%

LAD from RCA in TOF

Pulmonary Regurgitation After TOF Repair Valve Replacement Options

Heterograft

Percutaneous

Homograft

Mechanical

Melody Valve

• Bovine jugular venous valve segment

• Platinum-iridium stent

Indications Melody Valve FDA Approved Under HDE – 2010

• Circumference RVOT conduit with ≥16 mm diameter at time of original implantation

• Inner diameter ≤22 mm

• Regurgitation and/or stenosis

• ≥ moderate PR

• RVOT mean gradient ≥35-40 mm Hg

Post-Implant Intracardiac Echo

Extending the “lifespan” of RV to PA conduits

Safety Considerations

Procedural Risks

• Coronary artery compression

• Conduit rupture

• Device embolization

Device Risks

• Stent fracture

• Pulmonary emboli

• Endocarditis

Single Ventricle Univentricular Hearts

Double inlet Single inlet Common inlet

Mitral valve atresia

Hypoplastic LHS

Hypoplatic RHS

Pulmonary atresia/IVS

Heterotaxia

24-Year-Old Tricuspid Atresia

31-Year-Old Astronomer With DILV

Single Ventricle Adult Presentation

• Non-operated

• Unobstructed pulmonary circulation

• Eisenmenger syndrome

• Pulmonary stenosis (at or below PV)

• Balanced circulation with cyanosis

• Normal pulmonary pressure

• Palliated if normal PAP and ventricular fracture

• Systemic to pulmonary shunt

• Fontan operation

• Non-operated

31-Year-Old Astronomer With DILV

Eisenmenger Syndrome

VSD Eisenmenger

Eisenmenger

Cardiovascular

Glomerular sclerosis

Infection

Gall stones

Pulmonary

Hypertrophic osteoarthopathy

Neurologic

Hematologic

Syndrome in Eisenmenger Syndrome

0 2 4 6 8 10 12 14

Diller et al: EHJ 27:1737, 2006

Age (years)

171 patients Age 32±12 years

Eisenmenger patient P=0.02

Predicted survival

Simple lesion Complex

lesion

• Non-operated

26-Year-Old With DILV and Sub PS

• Non-operated

Blalock-Taussig Shunt

Right common carotid artery

Right subclavian artery

Right pulmonary artery

Pulmonary trunk

Brachiocephalic trunk (innominate artery)

Single Ventricle

• Glenn anastomosis: SVC to right PA

Classic bi-directional

Normal LVEF

LVEDP <12 mm Hg

TPG <10 mm Hg

MPAP <14-18 mm Hg

Glenn Anastomosis Isolated or TCP Connection

• Suprasternal short axis view

• Use low Nyquist limit and filter

• Low velocity biphasic forward flow

• Laminar color flow

Glenn Anastomosis Isolated or TCP Connection

Need to exclude obstruction by color/spectral

If high velocity or Mosaic color Doppler

Fontan Operation

• Fontan is not a specific procedure, but rather a concept

• Diverting all systemic venous return into the pulmonary artery bypassing RV

• Combination of different surgeries

• Palliation for the functionally Univentricular heart or ‘single’ ventricle

Unique Fontan Physiology

• Pulmonary flow does not have a ventricular pump

• Systemic ventricular function provides energy for transpulmonary flow

• Intrathoracic pressure changes also help

• Positive pressure ventilation poorly tolerated

• Pulmonary arterial flow obstructions will behave like venous stenosis

• Even small gradients can be bad

Optimal Candidates for Fontan Revised Criteria

1. Normal PA size and distribution

2. Low mean PA pressure <15 mm Hg

3. Normal pulmonary vascular resistance

4. Normal LV systolic and diastolic function

5. ± no AV valve regurgitation

Optimal results in those with optimum selection Any deviation can result in Fontan failure

Allow smooth stream-lined BF from veins to lungs

Echocardiographic Essentials Comprehensive Assessment

1. Fontan pathway

2. Ventricular size and function

3. Atrioventricular valve

4. Ventriculoarterial connection

5. Pulmonary veins and ASD

6. Right Atrium

7. Hepatic veins and inferior vena cava

8. Ascites and pleural effusion

Fontan Operation Historical Perspective

Kreutzer, 1973 Fontan, 1971

Fontan Operation Modifications

Post-Fontan Examination

• Acyanotic unless residual shunt

• Normal JVP except

• Fontan obstruction/failure

• Atrial arrhythmia, flutter waves ?

• Single S2 (palpable A2 if anterior aorta)

• No murmur except

• AVV regurgitation or sub AS

• Right sided CHF if complicated

Fontan

Fontan obstruction

Cyanosis

Ventricular dysfunction

Liver cirrhosis

Protein losing enteropathy

RA thrombi embolism

Arrhythmia

Pul V Obst; Sub AS; Co A

AV valve regurgitation

1. Fontan pathway

6. Right Atrium

Echocardiographic Essentials Fontan Pathway

• Rely on surgical record to identify type of connection

• Cavopulmonary anastomosis: Classic Glenn

• Bidirectional cavopulmonary connection

• Atriopulmonary connection

• Extracardiac or intracardiac conduit from IVC to main or right PA with bidirectional cavopulmonary connection

• Intracardiac tunnel from IVC to right PA with bidirectional cavopulmonary connection

Challenging anatomic and surgical variations

Use all windows in Fontan assessment

24-Year-Old SV With RAA to RVOT Fontan

24-Year-Old SV With RAA to RVOT

Pulsatile Fontan flow

Peaks in late systole and diastole

Both increase with inspiration

RA Appendage to PA Connection

• Horizontal plane

• Modified parasternal short axis

• Subcostal window

RA Appendage to PA Connection

25-Year-Old With PA/IVS, Status-Post Fontan Intracardiac Conduit

Lateral Tunnel Fontan

Lateral Right

Atrial Tunnel

21-Year-Old With TA, Status-Post Fontan Lateral Tunnel

Lateral Right

Atrial Tunnel

Tricuspid Atresia, Normally Related GA Extracardiac Fontan

‘Fenestrated’ Fontan

• Purposeful placement of small (3-4 mm) ASD

• Provides constant preload to ventricle

• Decreases in systemic venous pressure (pop off)

• Causes mild cyanosis (R to L shunt)

• Potential for paradoxical embolism

• Echo assessment

• Presence of gradient through

• AP4C, subcoastal

• Location and size

• Agitated saline (optional)

Fenestration Physiology

• Any ASD in Fontan will always show R L shunt

• Mean RAP is always > LAP

• Mean gradient of fenestration flow reflects trans-pulmonary gradient (5-8 mm Hg)

Any TPG of >8 mm Hg requires an explanation

Echocardiographic Essentials Ventricular Size and Function

• Systolic function

• 2D qualitative assessment of volume and global function evaluation

• Diastolic function

• AV valve, annulus and pulmonary veins

• Spectral and tissue Doppler

Echocardiographic Option

Myocardial performance index

Systolic rates of ventricular pressure change (Dp/Dt)

Fractional area shortening

Annular systolic DTI EF with 2D or 3D imaging

Echocardiographic Essentials

• Pulmonary veins

• 2D assessment with color and spectral Doppler

• Assess for obstruction of right pulmonary veins

• Best views: AP4C, subcoastal

• AV valve connection

• 2D and Doppler assessment of AV valve regurgitation presence and severity (PLAX, PSAX, AP4C)

• PISA assessment of RV/ERO, vena contracta

• Systolic flow reversal in pulmonary veins

11-Year-Old: TA, TGA, Sub AS and Coarction Fontan at Age 4, Sub AS x2, AVR

Fontan

11-Year-Old: TA, TGA, Sub AS and Coarction Fontan at Age 4, Sub AS x2, AVR

Fontan conversion to intra-atrial conduit

Cryoablation for atrial flutter

R P vein

RA L P vein

23-Year-Old With CHF

• Hypoplastic right HS

• TV hypoplasia, near pulmonary atresia

• Right BT at 3 days old

• Pulmonary vavlotomy at 2 years old

• Modified Fontan at 15 years old (RA to right PA)

• Edema, ascites, DOE in August 2007

• Atrial fibrillation

• New loud systolic murmur

23-Year-Old With CHF and PLE

1. Conversion to IA conduit BDCPA

2. Patch closure of TV

3. Lt AVV annuloplasty ring

4. Bi-atrial maze

5. Fenestration in atrial septum

1AT: 304 to 27 mg/dL

T. protein: 5.5 to 6.5 g/dL

Albumin: 3-3 to 6 g/dL

35-Year-Old With Tricuspid Atresia Fontan RAA to PA With PLA

Not suitable for Fontan conversion

LVEDP 16 mm Hg, MR, Cr = 2.8

35-Year-Old With Tricuspid Atresia RAA to PA Fontan

1. Fontan pathway

6. Right Atrium

Indications for TEE in Fontan

• Atrial arrhythmia necessitating cardioversion

• Severe spontaneous Echo contrast

• Systemic/pulmonary embolization

• Unexplained cyanosis

• Uncertainty about ventricular function or AV valve regurgitation

• Uncertainty about pulmonary venous obstruction

Adult Complex CHD Survivor

• No longer rare or odd

• Natural survivors

• Post-repair

• Challenging problems

• Repair or palliation

• Residua and sequelae

• Many need re-intervention

• Require specialized lifelong care

Wilhelm Ebstein

1836-1912

Ebstein Anomaly

• June 28, 1864

• 19-year-old laborer admitted to Allerheiligen Hospital in Breslau with shortness of breath and palpitations

• Died on July 6th

• Ebstein performed an autopsy the next day

Ebstein: Arch Anat Physiol 238, 1866

Thin, Translucent, Redundant, Sail-like Anterior Leaflet

Courtesy: William Edwards Collection

Anterior TV leaflet in Ebstein

Normal Delamination (Separation) of TV from RV Myocardium

RCA RA

Failed TV Delamination in Ebstein Anomaly

Atrialized

Ebstein Anomaly Genetics

• 1/210,000 live births

• MYH7 mutation + LVNC

• NKX 2.5 mutation

Ebstein Presentation Varies

• Asymptomatic

• Cyanosis (ASD/PFO = 30-78%)

• Arrhythmia (WPW = 15%)

• Dyspnea on exertion

• Edema

• Cardiomegaly

• Intrauterine death

Clinical Evaluation Pearls

• 60% diagnosed age <1 year

• 38% cyanotic

• 66% of neonates & infants

• 10% diagnosed prenatally

• Murmur most common in older children

• JVP frequently normal

• 10% not diagnosed until adulthood

Spectrum of Presentation

Severe Mild

The Child With This CXR…

… Had This Echo

Typical Ebstein ECG

Courtesy: Bryan Cannon, M.D.

Prolonged PR interval, marked RBBB, very large P waves

Atypical Ebstein ECG Normal PR, No RBBB Accessory Pathway

Courtesy: Bryan Cannon, M.D.

Approach to Patient With Ebstein & WPW

• 15% of patients with Ebstein anomaly

• Usually right-sided pathway

• RBBB + right-sided accessory path = synchronous RV/LV contraction narrow QRS

• EPS/RFA prior to OR for TV repair

• Long-term surveillance, high recurrence of atrial arrhythmia

Failed Delamination Results in

• Adherence of leaflets to underlying RV myocardium

• Displacement of the anular hinge points

Displacement septal

(8 mm/m2) and posterior

Dilated TVA

Dilated RA

Enlarged

atrialized RV

functional RV

Apical Displacement Index Normal <8 mm/m2 Ebstein Anomaly

23 mm/1.5 m2 = 15.3 mm/m2

Spiral Displacement of TV Anterior/Apical Rotation of Functional Orifice

Modified from Schrieber C Anderson: J Thorac Cardiovasc Surg, 1999

Ebstein Anomaly

• Spectrum of abnormalities of TV leaflets and RV myocardium

• Abnormalities of all leaflets

• Anterior, inferior, septal leaflets

• Delamination issues

• Inferior displacement and anterior/apical rotation of the septal leaflet

• More than valve problem, but rather a cardiomyopathy

Repairs for Ebstein Anomaly

• Danielson

• da Silva

• Ross

• Hardy

• Carpentier

• Quaegebeur

• Schmid

• Vargas

• Starnes

• Hetzer

• Meisner

• Sebening

• Kaushal

• Knott-Craig

• Friesen

• Chauvaud

• Ullmann

• Kaneko

• Wu

• Sano

Gordon K. Danielson Joseph A Dearani

Ebstein Anomaly Indications for Operation

• exercise tolerance, cyanosis

• Progressive RV dilation

• Before significant RV dysfunction

• Onset, progression of atrial arrhythmias

• Earlier operation if TV repair is likely

• Prior to LV dysfunction

Danielson Repair 1974

Pre-Cone Era

Overall Survival

0 5 10 15 20

ival (%

539 332 200 121 54

10 year = 84.7%

20 year = 71.2%

Multivariate Predictors of Overall Mortality

Mild mitral regurgitation 6.84 0.01

Mild PS 3.20 0.01

Moderate ↓ RV function 2.76 0.01

Accessory tract ablation 0.20 0.01

Late Survival Free of Reoperation

0 5 10 15 20

reopera

tion (

506 307 166 91 37

10 year = 74.1%

20 year = 46.1%

Late Functional Status

NYHA class I or II 83%

NYHA class III or IV 16%

• RV dysfunction

• Mitral and RVOTO surgery

• Correlation with ↑ early mortality

LV dysfunction correlated with ↑ late mortality

Early referral

Anatomic Tricuspid Valve Repair

Surgical Delamination

Dearani et al, 2008

Cone Repair for Ebstein Anomaly

Cone Technique Repair at True Anatomic Annulus via Surgical Leaflet Delamination

Preop Postop

Cone Repair

Preop Postop

Tricuspid Valve Repair in Pediatric Patients With Ebstein Anomaly

The Mayo Clinic Experience in the Current Era

Mayo Clinic Cone Repair <21-Years-Old June 2007-December 2012

Excluded n=4 Cone repair attempted

Early reop n=3

Early death n=1

(19 d, ECMO) Cone success

Valve replaced n=1

Re-repair n=2

Mayo Clinic Cone Repair <21-years-old June 2007-December 2012

82/84 (98%) repaired by time of hospital discharge

Additional Surgical Procedures

ASD/PFO closure 70%

Surgical Maze 33%

Bidirectional Glenn 31%*

*3 prior

Cone Repair Outcomes

Early mortality 1/84 (1.2%)

Early reoperation for TV

(prior to hospital discharge)

3/83 (3.6%)

2 re-repair

1 replaced

Late mortality (MVA) 1/82 (1.2%)

Late re-re-repair 1/81 (1.2%)

Cone Repair Early Breakdowns

• Age, gender not associated with early Cone failure

• Muscularization of anterior leaflet and absence of septal leaflet – factors that may preclude Cone repair

• Steep learning curve

No early Cone

breakdowns since 2010

Echo Follow-Up Data

Follow-up data available 94%

Time from hospital discharge 0.8±0.2 years

(0-5.5 years)

Tricuspid stenosis (>5 mm Hg) 6/81 (7.4%)

Grade of TR on last Echo

None/mild TR* 83%

Moderate 13%

Severe 4% *Annuloplasty ring use associated with less short-term TR (P=0.01)

Conclusions/Speculations Ebstein Anomaly 2013

• 98% successful Cone repair in young

• Intervene prior to RV dysfunction

• Selective use of 1.5 ventricle strategy

• Cone anatomic repair for most valve morphologies

• Durable repair in short term follow-up

• Replacement still important in older adult +/- Maze

• Long-term surveillance of RV and TV

Complete TGA After Surgical Correction

Management After Atrial and Arterial Switch Operations

A Brief Review

Complete-TGA

• Complete TGA or (d-TGA)

• Atrial switch for TGA

• Mustard and Senning

• Arterial switch for TGA

• Jatene with LeCompte

• Rastelli for TGA + PS + VSD

• Congenitally-corrected TGA (L-TGA)

All patients should be seen periodically in a

center where expertise in the clinical evaluation,

imaging and hemodynamic assessment of adult

congenital heart disease is available

Complete-TGA Mustard Operation

Atrial Switch Operations Mustard or Senning

• 20-year-old survival = 80%

• Successful pregnancies

• Late attrition from

• RV failure

• Arrhythmia

• Reduced exercise capacity

• 61% late gadolinium enhancement (fibrosis)

Wilson et al: JACC, 1998

Complete-TGA Status-Post Mustard

Complete-TGA Status-Post Senning

Complete-TGA Imaging After Mustard/Senning

*Parasternal long-axis image demonstrating the superior vena cava portion of systemic venous baffle as it enters left atrium; portion of PV is also visualized in this image

Complete-TGA Imaging After Mustard/Senning

Apical 4-chamber 2-D and color Doppler assessment of patient after Senning operation with significant PV pathway obstruction (arrow); proximal SV is also visualized in this image

Complete-TGA Imaging After Mustard/Senning Echocardiography in Pediatric and Adult Congenital Heart Disease

IVC after atrial switch procedure in d-TGA

Tilting into true apical 4-chamber view from more posterior PV pathway Left: IVC

3-D images from apical 4-chamber projection *Left atrium #Right atrium

Evaluation of Adult With Transposition After Atrial Switch Operation

Stent placement after Mustard operation with history of IVC baffle obstruction

Long-Term Outcome After Mustard

• 45 patient cohort assessment in 1997

• Follow-up decade later

• Mean age: 25 years (19-37)

• Time from Mustard: 24 years (18-33)

• 87% alive without transplant

• 54% persistent rhythm abnormality

• 26% pacemaker

Ebenroth & Hurwitz: Congenit Heart Dis, 2007

Issues After Atrial Switch Mustard or Senning

• Rhythm problems

• 40% NSR at 20 years post-op

• Fate of the systemic RV

• Progressive TR

• RV systolic dysfunction

• Venous baffle obstruction

• Mustard: SVC > IVC

• Senning: Pulmonary veins > systemic veins

Complete-TGA Status-Post Mustard Operation

Systemic RV Dysfunction

“The management is the same as LV dysfunction in a ‘normal’ heart”

• Beta-blockade

• Usually avoid

• Patients prone to bradyarrhythmia

• Afterload reduction ?

Systemic RV Dysfunction ACE Inhibition in Complete TGA

• 14 patients status-post Mustard

• Enalapril >6 months

• No improvement

• Exercise tolerance

• Hemodynamics

• Remodeling

• 9 (64%) increased their VO2max by 27%

Before ACEinhibitors

After ACEinhibitors

Hechter et al: Am J Cardiol, 2001

Systemic RV Dysfunction Limited Data

• Dore et al: Circulation, 2004

• Losarten vs placebo trial (n=29)

• No improvement in exercise capacity or reduction in NT-proBNP levels

• Giardini et al: Int J Cardiol, 2006

• Carvedilol in systemic RV (n=8)

• Safe and + RV modeling

• Severe case reports of CRT in systemic RV

• Shortening of the QRS

• Symptom improvement of CHF

Contraction of Systemic RV

• Normal RV free wall shortening

• Longitudinal > Circumferential

• Systemic RV (n=14) status-post Senning

• Circumferential > Longitudinal

• Adaptive effect, but no torsion ?

Pettersen et al: JACC, 2007

Assessing Function of Systemic RV RV Strain in Normal RV and LV and in Patients With d-TGA

Helle-Valle et al: J Am Coll Cardiol 49:2450, 2007

Normal RV Systemic RV Normal LV

200 ms

Longitudinal

strain (%)

Circumferential

strain (%)

Atrial Switch Operations MRI Evaluation

• 61% late gadolinium enhancement (fibrosis)

• Correlated with age

• Inversely with RVEF

• 44% of these with full-thickness anterior free wall involvement

Royal Brompton Group: Circulation, 2005

28-year-old with TGA

Status-Post Senning

Please submit movie file:

4senningPVobs.mpg

4senningPVobscolor.mpg

Pulmonary vein baffle mean gradient = 14 mm Hg

Intra-op TEE

prestnet.mpg

Mean Gradient = 12 mm Hg

Hybrid Procedure

wire.mpg

4senningPVobscolor.mpg

stentdeploy.mpg

postcolor.mpg

Post-Stent Gradient = 4 mm Hg

5 Months Later

fusenningPVstentcolor.mpg

Re-Intervention After Mustard or Senning

• Pacemaker

• Tricuspid valve repair/replace – rarely

• Venous baffle stent placement

• Late arterial switch – rare

• Cardiac transplantation

• But not too soon

Transposition of Great Arteries Complete-TGA

Arterial Switch

Status-Post Arterial Switch (ASO)

Issues After Arterial Switch

• Early

• Coronary insufficiency

• PA stenosis after LeCompte

• Late

• Progressive aortic regurgitation

• Generally

• Few long-term rhythm or ventricular function issues

AR After ASO

• 15% at 6 years post

• 30% at 15 years post

• Only 1.4% required reoperation

• Increased with

• VSD

• Older age at ASO

• Prior PA band

• PA – aorta size mismatch

Rastelli Operation TGA + VSD + PS

Giancarlo Rastelli

1933-1970

Rastelli Operation TGA + VSD + PS

Issues After Rastelli Repair

• 1964-2001

• 1,270 patients pulmonary ventricle pulmonary artery conduit

• 232 with TGA

• Freedom from reop worst in TGA

• TGA-PS-VSD: Shunt early

• Hold off on conduit until 2-3 years old

Dearani et al: Ann Thorac Surg, 2003

Surgical – Corrected TGA Summary

• Long-term outlook is good

• Atrial switch

• Long-term rhythm issues

• Surveillance for RV dysfunction, medical management?

• Arterial switch

• Progressive aortic regurgitation

• Rastelli

• RV PA conduit replacement

• Sub-AS surveillance

Thank you!

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HANDOUT - Comprehensive Echocardiographic Assessment of Adults with Congenital Heart Disease.pdf

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