TGA atrial vs arterial switch what do we need to look for and how to react

Transcription

1 TGA atrial vs arterial switch what do we need to look for and how to react Folkert Meijboom, MD, PhD, FES Dept ardiology University Medical entre Utrecht The Netherlands

2 TGA + atrial switch: Follow-up All patients should be seen at least annually in a specialized GUH centre. Frequent complications to look for: RV dysfunction Dilatation of the RV resulting in TR Baffle related problems Stenosis leakage

3 TGA atrial switch routine check-up: Once a year History Physical examination EG Echo On indication Holter, MR/T, cardiac cath

4 Physical examination TGA atrial switch Physical examination Signs of venous congestion Either heart failure Or obstruction systemic venous return baffle obstruction

5 Physical examination TGA atrial switch Physical examination Signs of venous congestion Either heart failure Or obstruction systemic venous return baffle obstruction Specific imaging: TTE, TEE, MR, T

6 TGA atrial switch Physical examination RV impuls precordial heave Auscultation: Normal first heart sound Single loud second heart sound aorta anterior Usually no murmur f murmur appears: development of TR; sign of worsening systemic ventricle =RV

7 TGA atrial switch Physical examination RV impuls precordial heave Auscultation: Normal first heart sound Single loud second heart sound aorta anterior Usually no murmur f murmur appears: development of TR; sign of worsening systemic ventricle =RV maging echo, MR -consider treatment

8 ndications for ntervention in Transposition of the Great Arteries After Atrial Switch (1) ndications 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 n 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. lass a Level b 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. a

9 TGA atrial switch EG 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

10 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 AA pacing often good enough AV conduction not compromised

11 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 AA pacing often good enough AV conduction not compromised Beware baffle narrowing, possibly compromised by PM lead

12 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 AA pacing often good enough AV conduction not compromised Visualization baffles before PM placement MR or TEE

13 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 AA pacing often good enough AV conduction not compromised n case of severe narrowing baffle?

14 ndications for ntervention in Transposition of the Great Arteries After Atrial Switch (2) ndications for catheter intervention lass a Level b Stenting should be performed in symptomatic patients with baffle stenosis Stenting (covered) or device closure should be performed in symptomatic patients with baffle leaks and substantial cyanosis at rest or during exercice Stenting (covered) or device closure should be performed in patients with baffle leaks and symptoms due to L-R shunt Stenting (covered) or device closure should be considered in asymptomatic patients with baffle leaks with substantial ventricular volume overload due to L- R shunt Stenting should be considered in asymptomatic patients with baffle stenosis who require a PM treatment a Stenting may be considered in other asymptomatic patients with baffle stenosis b 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.

15 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!!

16 TGA atrial switch: echo ventricular function

17 Parasternal long axis: RV and LV function

18 Parasternal long axis: RV and LV function

19 Parasternal long axis: M-mode

20 TGA atrial switch: echo ventricular function L R

21 ndications for ntervention in Transposition of the Great Arteries After Atrial Switch (1) ndications 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 n 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. lass a Level b 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. a

22 TGA atrial switch: echo ventricular function Baffles: Patency Leakages ndication for intervention?

23 ndications for ntervention in Transposition of the Great Arteries After Atrial Switch (2) ndications for catheter intervention lass a Level b Stenting should be performed in symptomatic patients with baffle stenosis Stenting (covered) or device closure should be performed in symptomatic patients with baffle leaks and substantial cyanosis at rest or during exercice Stenting (covered) or device closure should be performed in patients with baffle leaks and symptoms due to L-R shunt Stenting (covered) or device closure should be considered in asymptomatic patients with baffle leaks with substantial ventricular volume overload due to L- R shunt Stenting should be considered in asymptomatic patients with baffle stenosis who require a PM treatment a Stenting may be considered in other asymptomatic patients with baffle stenosis b 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.

24 ndications for ntervention in Transposition of the Great Arteries After Atrial Switch (2) ndications for catheter intervention lass a Level b Stenting should be performed in symptomatic patients with baffle stenosis Stenting (covered) or device closure should be performed in symptomatic patients with baffle leaks and substantial cyanosis at rest or during exercice Stenting (covered) or device closure should be performed in patients with baffle leaks and symptoms due to L-R shunt Stenting (covered) or device closure should be considered in asymptomatic patients with baffle leaks with substantial ventricular volume overload due to L- R shunt Stenting should be considered in asymptomatic patients with baffle stenosis who require a PM treatment a Stenting may be considered in other asymptomatic patients with baffle stenosis b 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.

25 TGA atrial switch routine check-up: Once a year History Physical examination EG Echo On indication Holter, MR/T, cardiac cath

26 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. SV stenosis is, however, mostly difficult to assess and may require TEE. ontrast echo is helpful for baffle leakage or stenosis. MR (T): 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) ardiac catheterization: indicated when non-invasive assessment is inconclusive or PAH requires evaluation. f something comes out; possible candidate for EP or ablation??

27 TGA + atrial switch (Mustard/Senning): EP Testing, Ablation and D 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 SD. Atrial tachyarrhythmia, impaired systemic RV function and QRS duration 140msec have been reported to be risk factors. See general recommendations for D implantation.

28 TGA atrial switch Did not cover everything Just some soundbites

29 TGA arterial switch

30 TGA + arterial switch: Follow-up All patients should be seen at least annually in a specialized GUH 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).

31 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. MR: evaluation of the aorta, pulmonary branch stenosis and flow distribution between left and right lung. T: might be used for non-invasive imaging of coronary arteries, including the ostia, in case of suspicion of stenosis and as an alternative for MR.

32 PA branches after Lecomte R L

33 Lecomte: pulmonary bifurcation anterior from ascending aorta

34 Echo Doppler often adequate to diagnose presence of elevated RV pressure, often not the exact substrate

35 MR or T often necessary for imaging of pulmonary trunk and branches

36 ndications for ntervention in Transposition of the Great Arteries After Arterial Switch Operation lass a Level b Stenting or surgery (depending on substrate) should be performed for coronary artery stenosis causing ischaemia Surgical repair of RVOTO should be performed in symptomatic patients with RV systolic pressure > 60 mmhg (TR velocity > 3.5 m/sec) Surgical repair of RVOTO should be performed regardless of symptoms when RV dysfunction develops (RVP may then be lower) Surgical repair should be considered in asymptomatic patients with RVOTO and systolic RVP >80 mmhg (TR velocity > 4.3 m/sec) 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) 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 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. a a a

37 ndications for ntervention in Transposition of the Great Arteries After Arterial Switch Operation lass a Level b Stenting or surgery (depending on substrate) should be performed for coronary artery stenosis causing ischaemia Surgical repair of RVOTO should be performed in symptomatic patients with RV systolic pressure > 60 mmhg (TR velocity > 3.5 m/sec) Surgical repair of RVOTO should be performed regardless of symptoms when RV dysfunction develops (RVP may then be lower) Surgical repair should be considered in asymptomatic patients with RVOTO and systolic RVP >80 mmhg (TR velocity > 4.3 m/sec) 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) 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 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. a a a

38 TGA + arterial switch: Follow-up All patients should be seen at least annually in a specialized GUH 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).

39 ndications for ntervention in Transposition of the Great Arteries After Arterial Switch Operation lass a Level b Stenting or surgery (depending on substrate) should be performed for coronary artery stenosis causing ischaemia Surgical repair of RVOTO should be performed in symptomatic patients with RV systolic pressure > 60 mmhg (TR velocity > 3.5 m/sec) Surgical repair of RVOTO should be performed regardless of symptoms when RV dysfunction develops (RVP may then be lower) Surgical repair should be considered in asymptomatic patients with RVOTO and systolic RVP >80 mmhg (TR velocity > 4.3 m/sec) 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) 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 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. a a a

40 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 use the guidelines do not know the guidelines by heart

41 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 use the guidelines do not know the guidelines by heart Thank you