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Transposition of the Great Arteries Eric Osborn January 27, 2010.

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Presentation on theme: "Transposition of the Great Arteries Eric Osborn January 27, 2010."— Presentation transcript:

1 Transposition of the Great Arteries Eric Osborn January 27, 2010

2 Outline Definitions Definitions Embryology Embryology Epidemiology Epidemiology Complete transposition (D-TGA) Complete transposition (D-TGA) Congenitally corrected transposition (L-TGA) Congenitally corrected transposition (L-TGA) Echocardiography Echocardiography

3 Definitions The key anatomic characteristic of transposition complexes is ventriculoarterial discordance. The key anatomic characteristic of transposition complexes is ventriculoarterial discordance. The aorta arises from the morphological RV The aorta arises from the morphological RV The PA arises from the morphological LV The PA arises from the morphological LV

4 Definitions Complete transposition (D-TGA) Complete transposition (D-TGA) Atrioventricular concordance Atrioventricular concordance

5 Definitions Congenitally corrected transposition (L-TGA) Congenitally corrected transposition (L-TGA) Atrioventricular discordance Atrioventricular discordance

6 Embryology 22 days gestation … 22 days gestation … the primitive straight cardiac tube is formed

7 Embryology 23 days gestation … the straight cardiac tube elongates and bends forming the cardiac loop. 23 days gestation … the straight cardiac tube elongates and bends forming the cardiac loop. Cephalic portion bends ventrally, caudally, and right-ward. Cephalic portion bends ventrally, caudally, and right-ward. Caudal portion moves dorsally, cranially, and left-ward. Caudal portion moves dorsally, cranially, and left-ward. The rotational motion folding over of the bulboventricular portion bringing the future ventricles side-by-side. The rotational motion folding over of the bulboventricular portion bringing the future ventricles side-by-side.

8 Embryology 4 th -7 th weeks gestation … the heart divides into 4 chambers via formation of swellings (cushions) of tissue that exhibit differential growth. 4 th -7 th weeks gestation … the heart divides into 4 chambers via formation of swellings (cushions) of tissue that exhibit differential growth. Endocardial cushions divide the AV canal forming the mitral and tricuspid valves. Endocardial cushions divide the AV canal forming the mitral and tricuspid valves. Conotruncal cushions form the outflow tracts, aortic and pulmonary roots. Conotruncal cushions form the outflow tracts, aortic and pulmonary roots.

9 Embryology 5 th week gestation … the conotruncal cushions. 5 th week gestation … the conotruncal cushions. Right superior truncal cushion grows distally and left-ward. Right superior truncal cushion grows distally and left-ward. Left inferior truncal cushion grows distally and right-ward. Left inferior truncal cushion grows distally and right-ward. The net effect is a twisting motion. The net effect is a twisting motion. The truncal cushions fuse to form the truncal septum. The truncal cushions fuse to form the truncal septum. Additional cushions develop in the conus which grow down and towards each other until they fuse with the truncal septum to form the RVOT and LVOT. Additional cushions develop in the conus which grow down and towards each other until they fuse with the truncal septum to form the RVOT and LVOT.

10 Embryology Mechanism of great artery transposition Mechanism of great artery transposition Conotruncal cushion defect Conotruncal cushion defect Leads to failure of the conotruncal septum to spiral and instead extends straight downward Leads to failure of the conotruncal septum to spiral and instead extends straight downward Aorta fuses with the RV and PA with the LV Aorta fuses with the RV and PA with the LV

11 Epidemiology ~0.8% of live births are complicated by a cardiovascular malformation *. ~0.8% of live births are complicated by a cardiovascular malformation *. >750,000 adult patients with congenital heart disease. >750,000 adult patients with congenital heart disease. Transposition of the great arteries occurs in approximately 1 per 5,000 live births. Transposition of the great arteries occurs in approximately 1 per 5,000 live births. More common in males More common in males Diagnosis possible in utero with fetal echocardiography Diagnosis possible in utero with fetal echocardiography Transvaginal ultrasound at weeks (limited views) Transvaginal ultrasound at weeks (limited views) Transabdominal ultrasound at 16 weeks Transabdominal ultrasound at 16 weeks *not including bicuspid aortic valve and mitral valve prolapse

12 Complete transposition (D-TGA) Pulmonary and systemic circulations are in parallel Pulmonary and systemic circulations are in parallel Lethal, if no mixing (ASD, PDA, VSD) Lethal, if no mixing (ASD, PDA, VSD) ¾ are simple with no major associated abnormalities ¾ are simple with no major associated abnormalities ¼ are complex ¼ are complex VSD (16%) VSD (16%) Pulmonary/subpulmonary stenosis (9%) Pulmonary/subpulmonary stenosis (9%) Coarctation of the aorta (4%) Coarctation of the aorta (4%)

13 Complete transposition (D-TGA) Clinical Presentation and Outcomes Larger size and weight at birth Larger size and weight at birth Dyspnea and cyanosis Dyspnea and cyanosis Progressive hypoxemia Progressive hypoxemia Congestive heart failure Congestive heart failure Without treatment, the outlook is dismal Without treatment, the outlook is dismal 30% mortality within the 1 st week 30% mortality within the 1 st week 90% mortality within the 1 st year 90% mortality within the 1 st year

14 Complete transposition (D-TGA) Management Prostaglandin E1 to maintain the PDA Prostaglandin E1 to maintain the PDA Atrial septostomy (balloon or surgical) Atrial septostomy (balloon or surgical) Palliative prior to corrective surgery Palliative prior to corrective surgery Repair within the first days to weeks of life Repair within the first days to weeks of life 2-4% mortality with 90% 1 year survival 2-4% mortality with 90% 1 year survival Atrial switch Atrial switch Mustard or Senning Mustard or Senning Arterial switch Arterial switch Rastelli procedure Rastelli procedure

15 Complete transposition (D-TGA) Atrial switch (Mustard/Senning) Developed in the 1950s Developed in the 1950s Baffle directs venous return to contralateral ventricle Baffle directs venous return to contralateral ventricle

16 Complete transposition (D-TGA) Atrial switch (Mustard/Senning) Disadvantages Disadvantages RV functions as the systemic ventricle RV functions as the systemic ventricle Several significant long term complications Several significant long term complications Congestive heart failure Congestive heart failure Arrhythmias Arrhythmias Baffle leaks and obstruction Baffle leaks and obstruction Pulmonary hypertension Pulmonary hypertension Paradoxial embolus Paradoxial embolus Endocarditis Endocarditis Overall survival 75% at 25 years Overall survival 75% at 25 years Senning may be better than Mustard [Moons et al, Heart 2004] Senning may be better than Mustard [Moons et al, Heart 2004] 340 patients (~ ⅔ Senning) compared 340 patients (~ ⅔ Senning) compared Less obstruction (1 vs. 15%) and better functional class with Senning Less obstruction (1 vs. 15%) and better functional class with Senning No significant mortality benefit No significant mortality benefit

17 Complete transposition (D-TGA) Atrial switch (Mustard/Senning) Arrhythmias Palpitations, presyncope, and syncope are not uncommon Palpitations, presyncope, and syncope are not uncommon Both brady and tachyarrythmias frequently seen Both brady and tachyarrythmias frequently seen 50% develop sinus node dysfunction 50% develop sinus node dysfunction Physical damage during surgery and baffle construction Physical damage during surgery and baffle construction Disruption of blood supply leading to ischemia Disruption of blood supply leading to ischemia 20% develop atrial flutter 20% develop atrial flutter Sensitive to nodal agents due to conduction system disease Sensitive to nodal agents due to conduction system disease 11% required pacemakers at 20 years [Gelatt et al, J Am Coll Cardiol 1997] 11% required pacemakers at 20 years [Gelatt et al, J Am Coll Cardiol 1997] Pacemakers are difficult to place due to distorted anatomy Pacemakers are difficult to place due to distorted anatomy Should be avoided if residual intracardiac communications due to risk of paradoxical embolus and stroke Should be avoided if residual intracardiac communications due to risk of paradoxical embolus and stroke

18 Complete transposition (D-TGA) Atrial switch (Mustard/Senning) Congestive heart failure Most adult patients develop congestive heart failure Most adult patients develop congestive heart failure By 20 years most are NYHA Class I or II By 20 years most are NYHA Class I or II RV filling compromised due to defects in baffle construction RV filling compromised due to defects in baffle construction Baffle leaks (Mustard>Senning) Baffle leaks (Mustard>Senning) Left-to-right shunts with pulmonary hypertension (7%) Left-to-right shunts with pulmonary hypertension (7%) Risk of paradoxical embolus and stroke Risk of paradoxical embolus and stroke Indications for intervention include >1.5:1 left-to-right shunt or any right-to-left shunt Indications for intervention include >1.5:1 left-to-right shunt or any right-to-left shunt Baffle obstruction (5-15%, Mustard>Senning) Baffle obstruction (5-15%, Mustard>Senning) SVC>IVC manifesting as SVC syndrome or hepatic congestion/cirrhosis SVC>IVC manifesting as SVC syndrome or hepatic congestion/cirrhosis Often undetected due to collateral venous drainage (e.g. azygous vein) Often undetected due to collateral venous drainage (e.g. azygous vein) 40% develop right ventricular dysfunction 40% develop right ventricular dysfunction 10-40% develop 2 + or greater tricuspid (systemic AV valve) regurgitation 10-40% develop 2 + or greater tricuspid (systemic AV valve) regurgitation Annular dilatation from RV failure Annular dilatation from RV failure Damage from surgery or endocarditis Damage from surgery or endocarditis

19 Complete transposition (D-TGA) Suggested Follow-up

20 Complete transposition (D-TGA) Arterial switch Developed in the 1980s Developed in the 1980s Great arteries and coronaries are transected and re- anastamosed Great arteries and coronaries are transected and re- anastamosed

21 Complete transposition (D-TGA) Arterial switch Advantages Advantages LV is the systemic pump LV is the systemic pump No disruption of atrial conduction (sinus rhythm) No disruption of atrial conduction (sinus rhythm) Fewer long term complications compared to atrial switch Fewer long term complications compared to atrial switch Coronary ostial stenosis Coronary ostial stenosis Supravalvular pulmonary/aortic stenosis Supravalvular pulmonary/aortic stenosis Intervention indicated for RVOT gradient >50 mmHg Intervention indicated for RVOT gradient >50 mmHg Neoaortic regurgitation Neoaortic regurgitation Arrhythmias Arrhythmias Follow up with normal LV function and good exercise capacity Follow up with normal LV function and good exercise capacity

22 Complete transposition (D-TGA) Rastelli procedure TGA with VSD and LVOT obstruction TGA with VSD and LVOT obstruction Outcomes Outcomes RV-PA conduit obstruction RV-PA conduit obstruction Exercise intolerance/angina Exercise intolerance/angina RV failure RV failure Intervention for RV-PA Intervention for RV-PA gradient >50 mmHg gradient >50 mmHg LV-Ao patch obstruction LV-Ao patch obstruction Dyspnea or syncope Dyspnea or syncope

23 Complete transposition (D-TGA) RV Failure after Atrial Switch Standard heart failure therapies are unproven Standard heart failure therapies are unproven The two-stage arterial switch The two-stage arterial switch Stage 1 – the PA is banded to ‘re-train’ the LV to handle systemic pressures Stage 1 – the PA is banded to ‘re-train’ the LV to handle systemic pressures Stage 2 – the atrial baffles and pulmonary band are taken down and an arterial switch is performed Stage 2 – the atrial baffles and pulmonary band are taken down and an arterial switch is performed 50% survival at 8 years in early results 50% survival at 8 years in early results Appears to be more successful in patients under 12 Appears to be more successful in patients under 12

24 Congenitally corrected transposition (L-TGA) A rare disorder that may present in adulthood. A rare disorder that may present in adulthood. Associated anomalies (95% of patients) Associated anomalies (95% of patients) VSD (75%, commonly perimembranous) VSD (75%, commonly perimembranous) Pulmonary stenosis (75%, commonly subvalvular) Pulmonary stenosis (75%, commonly subvalvular) Tricuspid valve anomalies (>75%) Tricuspid valve anomalies (>75%) Congenital complete heart block (5%) Congenital complete heart block (5%)

25 Congenitally corrected transposition (L-TGA) Outcomes Arrhythmias Arrhythmias Abnormal AV node and His positions Abnormal AV node and His positions Dual AV nodes Dual AV nodes 2% per year incidence of complete heart block 2% per year incidence of complete heart block Susceptible to fibrosis of conduction system Susceptible to fibrosis of conduction system Median survival 40 years Median survival 40 years Mortality from progressive RV failure or arrhythmias Mortality from progressive RV failure or arrhythmias Tricuspid regurgitation is major predictor Tricuspid regurgitation is major predictor

26 Congenitally corrected transposition (L-TGA) Double Switch Procedure

27 Echocardiography Segmental approach to congenital heart disease 1.Position of the apex 2.Situs of the atria Morphological atria based on anatomic appearance of their appendages Morphological atria based on anatomic appearance of their appendages 75% concordance with abdominal situs (aorta and IVC positions) 75% concordance with abdominal situs (aorta and IVC positions) 3.Atrioventricular relationship Differentiate the morphological RV from LV: Differentiate the morphological RV from LV: 1. Trabeculated apex 2. Moderator band 3. Septal attachment of the tricuspid valve 4. Lower (apical) insertion of the tricuspid valve 4.Ventriculoarterial relationship Pulmonary artery is distinguished by its early branching pattern Pulmonary artery is distinguished by its early branching pattern Curved contour of the aortic arch with three major branches Curved contour of the aortic arch with three major branches

28 Echocardiography Complete Transposition with Atrial Switch Hallmark is parallel great arteries (parasternal long axis) Hallmark is parallel great arteries (parasternal long axis) Aorta is anterior to PA Aorta is anterior to PA

29 Echocardiography Complete Transposition with Atrial Switch Systemic hypertrophied RV septum bows into LV Systemic hypertrophied RV septum bows into LV May impact TR and enhance subpulmonary stenosis May impact TR and enhance subpulmonary stenosis

30 Echocardiography Complete Transposition with Atrial Switch Aortic and pulmonic valves lie in the same plane Aortic and pulmonic valves lie in the same plane Aorta is anterior and to the right (parasternal short axis) Aorta is anterior and to the right (parasternal short axis)

31 Echocardiography Congenitally Corrected Transposition Hallmark is reversed offsetting of the AV valves Hallmark is reversed offsetting of the AV valves Aorta is anterior and to the left (parasternal short axis) Aorta is anterior and to the left (parasternal short axis)

32 Echocardiography Special Considerations Atrial switch Atrial switch RV function RV function Tricuspid regurgitation Tricuspid regurgitation Subpulmonary obstruction Subpulmonary obstruction Baffle leak or obstruction (color Doppler) Baffle leak or obstruction (color Doppler) Normal baffle flow is phasic with peak velocity <1 m/sec Normal baffle flow is phasic with peak velocity <1 m/sec Arterial switch Arterial switch Neoaortic valve regurgitation Neoaortic valve regurgitation Supraneopulmonary valve stenosis Supraneopulmonary valve stenosis Wall motion abnormalities due to coronary artery ostial stenosis Wall motion abnormalities due to coronary artery ostial stenosis Rastelli procedure Rastelli procedure LV-Ao tunnel patch obstruction LV-Ao tunnel patch obstruction RV-PA conduit degeneration (stenosis/regurgitation) RV-PA conduit degeneration (stenosis/regurgitation)

33 Echocardiography Special Techniques Index of myocardial performance Index of myocardial performance dP/dT from tricuspid regurgitant velocity dP/dT from tricuspid regurgitant velocity Isovolumic myocardial acceleration Isovolumic myocardial acceleration Tissue Doppler measurement of myocardial acceleration during isovolumic contraction Tissue Doppler measurement of myocardial acceleration during isovolumic contraction ?sensitive assessment of RV contractility that is less load dependent ?sensitive assessment of RV contractility that is less load dependent

34 Endocarditis Prophylaxis ACC/AHA 2008 Guidelines state that antibiotic prophylaxis is reasonable to consider for patients at the highest risk of adverse outcomes (Class IIa) Prosthetic valves Prosthetic valves Prior endocarditis Prior endocarditis Congenital heart disease Congenital heart disease Unrepaired cyanotic, including palliative shunts and conduits Unrepaired cyanotic, including palliative shunts and conduits Completely repaired with prosthetic material or device (6 months) Completely repaired with prosthetic material or device (6 months) Repaired with defects at or near a prosthetic device Repaired with defects at or near a prosthetic device Post-cardiac transplant with valvular disease Post-cardiac transplant with valvular disease

35 Endocarditis Prophylaxis

36 References Webb et al., Congenital Heart Disease in Braunwald’s Heart Disease, 8 th ed., Chapter 61, Webb et al., Congenital Heart Disease in Braunwald’s Heart Disease, 8 th ed., Chapter 61, Sadler, Cardiovascular System in Langman’s Medical Embryology, 8 th ed., Chapter 11, Sadler, Cardiovascular System in Langman’s Medical Embryology, 8 th ed., Chapter 11, Otto, The Adult with Congenital Heart Disease in Clinical Echocardiography, 4 th ed., Chapter 17, Otto, The Adult with Congenital Heart Disease in Clinical Echocardiography, 4 th ed., Chapter 17, Warnes, Transposition of the Great Arteries, Circulation : Warnes, Transposition of the Great Arteries, Circulation : Love et al., Evaluation and Management of the Adult Patient with Transposition of the Great Arteries Follow Atrial-level (Senning or Mustard) Repair, Nature Clinical Practice Cardiovasc Med : Love et al., Evaluation and Management of the Adult Patient with Transposition of the Great Arteries Follow Atrial-level (Senning or Mustard) Repair, Nature Clinical Practice Cardiovasc Med : Verhuegt et al., Long-term Prognosis of Congenital Heart Defects: A Systematic Review, Int J Cardiol : Verhuegt et al., Long-term Prognosis of Congenital Heart Defects: A Systematic Review, Int J Cardiol : Skinner et al., Transposition of the Great Arteries: from Fetus to Adult, Heart : Skinner et al., Transposition of the Great Arteries: from Fetus to Adult, Heart : ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease, J Am Coll Cardiol :e ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease, J Am Coll Cardiol :e1-121.


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