Download Transposition of the great arteries with atrial switch versus arterial

TGA – atrial vs arterial switch
what do we need to look for
and
how to react
Folkert Meijboom, MD, PhD, FESC
Dept Cardiology
University Medical Centre Utrecht
The Netherlands
www.escardio.org/guidelines
TGA + atrial switch: Follow-up
● All patients should be seen at least annually in a
specialized GUCH centre.
Frequent complications to look for:
● RV dysfunction
● Dilatation of the RV resulting in TR
● Baffle – related problems
● Stenosis
● leakage
www.escardio.org/guidelines
TGA atrial switch
● routine check-up:
– Once a year
● History
● Physical examination
● ECG
● Echo
– On indication
● Holter, CMR/CT, cardiac cath
www.escardio.org/guidelines
TGA atrial switch
Physical examination
● Physical examination
– Signs of venous congestion
● Either heart failure
● Or obstruction systemic venous return
● baffle obstruction
www.escardio.org/guidelines
TGA atrial switch
Physical examination
● Physical examination
– Signs of venous congestion
● Either heart failure
● Or obstruction systemic venous return
● baffle obstruction
● Specific imaging: TTE, TEE, CMR, CT
www.escardio.org/guidelines
TGA atrial switch
Physical examination
● RV impuls – precordial heave
● Auscultation:
– Normal first heart sound
– Single loud second heart sound – aorta anterior
– Usually no murmur
● If murmur appears: development of TR;
– sign of worsening systemic ventricle =RV
www.escardio.org/guidelines
TGA atrial switch
Physical examination
● RV impuls – precordial heave
● Auscultation:
– Normal first heart sound
– Single loud second heart sound – aorta anterior
– Usually no murmur
● If murmur appears: development of TR;
– sign of worsening systemic ventricle =RV
– Imaging – echo, CMR -consider treatment
www.escardio.org/guidelines
Indications for Intervention in Transposition
of the Great Arteries After Atrial Switch (1)
Classa Levelb
Indications for surgical intervention
● Valve repair or replacement should be performed in patients with severe
symptomatic systemic (tricuspid) AV valve regurgitation without significant
ventricular dysfunction (RVEF ≥ 45%)
● Significant systemic ventricular dysfunction, with or without TR, should be
treated conservatively or eventually with cardiac transplantation
● LVOTO if symptomatic or if LV function deteriorates should be treated
surgically
● In symptomatic pulmonary venous obstruction surgical repair (catheter
intervention rarely possible) should be performed.
● Symptomatic patients with baffle stenosis not amenable for catheter
intervention should be treated surgically
● Symptomatic patients with baffle leaks not amenable for catheter
intervention should be treated surgically.
● Valve repair or replacement should be considered for severe asymptomatic
systemic (tricuspid) AV valve regurgitation without significant ventricular
dysfunction (RVEF ≥ 45%)
● Pulmonary artery banding in adult patients, to create septal shift, or a left
ventricular training with subsequent arterial switch, is currently experimental
and should be avoided.
I
C
I
C
I
C
I
C
I
C
I
C
IIa
C
III
C
a = class of recommendation. b = level of evidence. AV = atrioventricular; L-R shunt = left-to-right shunt; LV = Left ventricle;
LVOTO = left ventricular outflow tract obstruction; RVEF = right ventricular ejection fraction; TR = tricuspid regurgitation.
www.escardio.org/guidelines
TGA atrial switch
ECG
● sinus rhythm or narrow-QRS escape rhythm and
RVH.
● Sinus node dysfunction with slow heart rate can
occur
● Atrial flutter with a 2:1 or 3:1 conduction is
freequently seen
● all types of arrhythmia can occur
www.escardio.org/guidelines
TGA atrial switch
arrhythmia: how to react
● Sinus node dysfunction with slow heart rate can
occur
– PM indication as in normal hearts
– Transvenous pacing:
● PM lead through baffle in remnant LA with left
auricle
● PM lead in left auricle
– Good and stable position; easy to reach
– AAI pacing often good enough
– AV conduction not compromised
www.escardio.org/guidelines
TGA atrial switch
arrhythmia: how to react
● Sinus node dysfunction with slow heart rate can
occur
– PM indication as in normal hearts
– Transvenous pacing:
● PM lead through baffle in remnant LA with left
auricle
● PM lead in left auricle
– Good and stable psoition; easy to reach
– AAI pacing often good enough
– AV conduction not compromised
Beware baffle narrowing, possibly compromised by PM lead
www.escardio.org/guidelines
TGA atrial switch
arrhythmia: how to react
● Sinus node dysfunction with slow heart rate can
occur
– PM indication as in normal hearts
– Transvenous pacing:
● PM lead through baffle in remnant LA with left
auricle
● PM lead in left auricle
– Good and stable psoition; easy to reach
– AAI pacing often good enough
– AV conduction not compromised
Visualization
baffles before PM placement – CMR or TEE
www.escardio.org/guidelines
TGA atrial switch
arrhythmia: how to react
● Sinus node dysfunction with slow heart rate can
occur
– PM indication as in normal hearts
– Transvenous pacing:
● PM lead through baffle in remnant LA with left
auricle
● PM lead in left auricle
– Good and stable psoition; easy to reach
– AAI pacing often good enough
– AV conduction not compromised
In case of severe narrowing baffle?
www.escardio.org/guidelines
Indications for Intervention in Transposition
of the Great Arteries After Atrial Switch (2)
Classa Levelb
Indications for catheter intervention
● Stenting should be performed in symptomatic patients with baffle stenosis
I
C
● Stenting (covered) or device closure should be performed in symptomatic
patients with baffle leaks and substantial cyanosis at rest or during exercice
I
C
● Stenting (covered) or device closure should be performed in patients with baffle
leaks and symptoms due to L-R shunt
I
C
● Stenting (covered) or device closure should be considered in asymptomatic
patients with baffle leaks with substantial ventricular volume overload due to LR shunt
I
C
● Stenting should be considered in asymptomatic patients with baffle stenosis
who require a PM treatment
IIa
C
● Stenting may be considered in other asymptomatic patients with baffle stenosis
IIb
C
a = class of recommendation. b = level of evidence.
AV = atrioventricular; L-R shunt = left-to-right shunt; LV = Left ventricle; LVOTO = left ventricular outflow tract obstruction;
PM = pacemaker; RVEF = right ventricular ejection fraction; TR = tricuspid regurgitation.
www.escardio.org/guidelines
TGA atrial switch
PM & conduction disturbances
● AV conduction
compromised/questionnable?
– Also PM wire in ventricle
● Smooth-walled LV – screw-in electrode
● 2 wires in baffles – beware of narrowing!!
www.escardio.org/guidelines
TGA atrial switch: echo
● ventricular function
www.escardio.org/guidelines
Parasternal long axis: RV and LV function
www.escardio.org/guidelines
Parasternal long axis: RV and LV function
www.escardio.org/guidelines
Parasternal long axis: M-mode
www.escardio.org/guidelines
TGA atrial switch: echo – ventricular function
L
www.escardio.org/guidelines
R
Indications for Intervention in Transposition
of the Great Arteries After Atrial Switch (1)
Classa Levelb
Indications for surgical intervention
● Valve repair or replacement should be performed in patients with severe
symptomatic systemic (tricuspid) AV valve regurgitation without significant
ventricular dysfunction (RVEF ≥ 45%)
● Significant systemic ventricular dysfunction, with or without TR, should be
treated conservatively or eventually with cardiac transplantation
● LVOTO if symptomatic or if LV function deteriorates should be treated
surgically
● In symptomatic pulmonary venous obstruction surgical repair (catheter
intervention rarely possible) should be performed.
● Symptomatic patients with baffle stenosis not amenable for catheter
intervention should be treated surgically
● Symptomatic patients with baffle leaks not amenable for catheter
intervention should be treated surgically.
● Valve repair or replacement should be considered for severe asymptomatic
systemic (tricuspid) AV valve regurgitation without significant ventricular
dysfunction (RVEF ≥ 45%)
● Pulmonary artery banding in adult patients, to create septal shift, or a left
ventricular training with subsequent arterial switch, is currently experimental
and should be avoided.
I
C
I
C
I
C
I
C
I
C
I
C
IIa
C
III
C
a = class of recommendation. b = level of evidence. AV = atrioventricular; L-R shunt = left-to-right shunt; LV = Left ventricle;
LVOTO = left ventricular outflow tract obstruction; RVEF = right ventricular ejection fraction; TR = tricuspid regurgitation.
www.escardio.org/guidelines
TGA atrial switch: echo
● ventricular function
● Baffles:
– Patency
– Leakages
– Indication for intervention?
www.escardio.org/guidelines
Indications for Intervention in Transposition
of the Great Arteries After Atrial Switch (2)
Classa Levelb
Indications for catheter intervention
● Stenting should be performed in symptomatic patients with baffle stenosis
I
C
● Stenting (covered) or device closure should be performed in symptomatic
patients with baffle leaks and substantial cyanosis at rest or during exercice
I
C
● Stenting (covered) or device closure should be performed in patients with baffle
leaks and symptoms due to L-R shunt
I
C
● Stenting (covered) or device closure should be considered in asymptomatic
patients with baffle leaks with substantial ventricular volume overload due to LR shunt
I
C
● Stenting should be considered in asymptomatic patients with baffle stenosis
who require a PM treatment
IIa
C
● Stenting may be considered in other asymptomatic patients with baffle stenosis
IIb
C
a = class of recommendation. b = level of evidence.
AV = atrioventricular; L-R shunt = left-to-right shunt; LV = Left ventricle; LVOTO = left ventricular outflow tract obstruction;
PM = pacemaker; RVEF = right ventricular ejection fraction; TR = tricuspid regurgitation.
www.escardio.org/guidelines
Indications for Intervention in Transposition
of the Great Arteries After Atrial Switch (2)
Classa Levelb
Indications for catheter intervention
● Stenting should be performed in symptomatic patients with baffle stenosis
I
C
● Stenting (covered) or device closure should be performed in symptomatic
patients with baffle leaks and substantial cyanosis at rest or during exercice
I
C
● Stenting (covered) or device closure should be performed in patients with baffle
leaks and symptoms due to L-R shunt
I
C
● Stenting (covered) or device closure should be considered in asymptomatic
patients with baffle leaks with substantial ventricular volume overload due to LR shunt
I
C
● Stenting should be considered in asymptomatic patients with baffle stenosis
who require a PM treatment
IIa
C
● Stenting may be considered in other asymptomatic patients with baffle stenosis
IIb
C
a = class of recommendation. b = level of evidence.
AV = atrioventricular; L-R shunt = left-to-right shunt; LV = Left ventricle; LVOTO = left ventricular outflow tract obstruction;
PM = pacemaker; RVEF = right ventricular ejection fraction; TR = tricuspid regurgitation.
www.escardio.org/guidelines
TGA atrial switch
● routine check-up:
– Once a year
● History
● Physical examination
● ECG
● Echo
– On indication
● Holter, CMR/CT, cardiac cath
www.escardio.org/guidelines
TGA + atrial switch (Mustard/Senning): Diagnostic Work-up
● Echocardiography: first line diagnostic technique providing
systemic and sub-pulmonary ventricular size and function, subpulmonary outflow tract obstruction, TR, leakage or obstruction of
the atrial baffles and assessment of pulmonary venous return. SVC
stenosis is, however, mostly difficult to assess and may require TEE.
Contrast echo is helpful for baffle leakage or stenosis.
● CMR (CT): indicated for assessment of systemic RV function and
patency of the atrial baffles.
● Holter monitoring, event recorder: required for selected
patients (high-risk, investigated for suspected or clinical arrhythmia)
● Cardiac catheterization: indicated when non-invasive
assessment is inconclusive or PAH requires evaluation.
If something comes out; possible
candidate for EP or ablation??
www.escardio.org/guidelines
TGA + atrial switch (Mustard/Senning):
EP Testing, Ablation and ICD
● These procedures are complicated by the fact that the
atria are not normally accessible for catheters and
“normal” EP procedures because of the course of the
baffles and should only be done in specialised centres
with specific expertise.
● Patients
are
at
increased
risk
of
SCD.
Atrial tachyarrhythmia, impaired systemic RV function
and QRS duration ≥ 140msec have been reported to be
risk factors.
See general recommendations for ICD implantation.
www.escardio.org/guidelines
TGA atrial switch
● Did not cover everything
– Just some soundbites
www.escardio.org/guidelines
TGA arterial switch
www.escardio.org/guidelines
TGA + arterial switch: Follow-up
● All patients should be seen at least annually in a
specialized GUCH centre.
Frequent complications to look for:
● LV dysfunction and arrhythmias: both may be related
to coronary artery problems (re-implanted ostia)
● Dilatation of the proximal part of the ascending aorta
resulting in AR
● Supravalvular PS, pulmonary branch stenosis
(unilaterally or bilaterally).
www.escardio.org/guidelines
TGA + arterial switch: Diagnostic Work-up (1)
● Echocardiography: key diagnostic technique providing LV
function (global and regional), stenosis at the arterial anastomotic
sites, most commonly PS, neoaortic valve regurgitation, dimension
of the ascending aorta and the acute angulation of the aortic arch.
The pulmonary trunk, the bifurcation and both branches should be
evaluated for the presence, localisation and severity of stenoses. RV
function should be judged and systolic pressures should be
estimated (TR velocity). Stress echo can unmask LV dysfunction
and detect provocable myocardial ischemia.
● CMR: evaluation of the aorta, pulmonary branch stenosis and flow
distribution between left and right lung.
● CT: might be used for non-invasive imaging of coronary arteries,
including the ostia, in case of suspicion of stenosis and as an
alternative for CMR.
www.escardio.org/guidelines
PA branches after Lecomte
R
www.escardio.org/guidelines
L
Lecomte: pulmonary bifurcation
anterior from ascending aorta
www.escardio.org/guidelines
Echo Doppler often adequate to diagnose
presence of elevated RV pressure,
often not the exact substrate
www.escardio.org/guidelines
CMR or CT often necessary for imaging of
pulmonary trunk and branches
www.escardio.org/guidelines
Indications for Intervention in Transposition of
the Great Arteries After Arterial Switch Operation
Classa Levelb
• Stenting or surgery (depending on substrate) should be performed for coronary
artery stenosis causing ischaemia
I
C
• Surgical repair of RVOTO should be performed in symptomatic patients with
RV systolic pressure > 60 mmHg (TR velocity > 3.5 m/sec)
I
C
• Surgical repair of RVOTO should be performed regardless of symptoms when
RV dysfunction develops (RVP may then be lower)
I
C
• Surgical repair should be considered in asymptomatic patients with RVOTO and
systolic RVP >80 mmHg (TR velocity > 4.3 m/sec)
IIa
C
• Aortic root surgery should be considered when the (neo-)aortic root is larger than
55 mm, providing average adult stature (for aortic valve replacement for severe AR
see guidelines for AR)
IIa
C
• Stenting or surgery (depending on substrate) should be considered for peripheral
PS, regardless of symptoms, if > 50% diameter narrowing and RV systolic
pressure > 50 mmHg and/or lung perfusion abnormalities
IIa
C
a = class of recommendation. b = level of evidence.
AR = aortic regurgitation; AV = atrioventricular; RV = right ventricle; RVP = right ventricular pressure;
RVOTO = right ventricular outflow tract obstruction; TR = tricuspid regurgitation.
www.escardio.org/guidelines
Indications for Intervention in Transposition of
the Great Arteries After Arterial Switch Operation
Classa Levelb
• Stenting or surgery (depending on substrate) should be performed for coronary
artery stenosis causing ischaemia
I
C
• Surgical repair of RVOTO should be performed in symptomatic patients with
RV systolic pressure > 60 mmHg (TR velocity > 3.5 m/sec)
I
C
• Surgical repair of RVOTO should be performed regardless of symptoms when
RV dysfunction develops (RVP may then be lower)
I
C
• Surgical repair should be considered in asymptomatic patients with RVOTO and
systolic RVP >80 mmHg (TR velocity > 4.3 m/sec)
IIa
C
• Aortic root surgery should be considered when the (neo-)aortic root is larger than
55 mm, providing average adult stature (for aortic valve replacement for severe AR
see guidelines for AR)
IIa
C
• Stenting or surgery (depending on substrate) should be considered for peripheral
PS, regardless of symptoms, if > 50% diameter narrowing and RV systolic
pressure > 50 mmHg and/or lung perfusion abnormalities
IIa
C
a = class of recommendation. b = level of evidence.
AR = aortic regurgitation; AV = atrioventricular; RV = right ventricle; RVP = right ventricular pressure;
RVOTO = right ventricular outflow tract obstruction; TR = tricuspid regurgitation.
www.escardio.org/guidelines
TGA + arterial switch: Follow-up
● All patients should be seen at least annually in a
specialized GUCH centre.
Frequent complications to look for:
● LV dysfunction and arrhythmias: both may be related
to coronary artery problems (re-implanted ostia)
● Dilatation of the proximal part of the ascending aorta
resulting in AR
● Supravalvular PS, pulmonary branch stenosis
(unilaterally or bilaterally).
www.escardio.org/guidelines
Indications for Intervention in Transposition of
the Great Arteries After Arterial Switch Operation
Classa Levelb
• Stenting or surgery (depending on substrate) should be performed for coronary
artery stenosis causing ischaemia
I
C
• Surgical repair of RVOTO should be performed in symptomatic patients with
RV systolic pressure > 60 mmHg (TR velocity > 3.5 m/sec)
I
C
• Surgical repair of RVOTO should be performed regardless of symptoms when
RV dysfunction develops (RVP may then be lower)
I
C
• Surgical repair should be considered in asymptomatic patients with RVOTO and
systolic RVP >80 mmHg (TR velocity > 4.3 m/sec)
IIa
C
• Aortic root surgery should be considered when the (neo-)aortic root is larger than
55 mm, providing average adult stature (for aortic valve replacement for severe AR
see guidelines for AR)
IIa
C
• Stenting or surgery (depending on substrate) should be considered for peripheral
PS, regardless of symptoms, if > 50% diameter narrowing and RV systolic
pressure > 50 mmHg and/or lung perfusion abnormalities
IIa
C
a = class of recommendation. b = level of evidence.
AR = aortic regurgitation; AV = atrioventricular; RV = right ventricle; RVP = right ventricular pressure;
RVOTO = right ventricular outflow tract obstruction; TR = tricuspid regurgitation.
www.escardio.org/guidelines
TGA atrial and arterial switch
● We went through the normal routine of the annual
outpatient clinic visit
● Made “jumps” to the guidelines what to do when
something abnormal/deteriorated was found
● And that is how I use the guidelines
● I do not know the guidelines by heart
www.escardio.org/guidelines
TGA atrial and arterial switch
● We went through the normal routine of the annual
outpatient clinic visit
● Made “jumps” to the guidelines what to do when
something abnormal/deteriorated was found
● And that is how I use the guidelines
● I do not know the guidelines by heart
www.escardio.org/guidelines
Thank you