Transposition of the Great Arteries

Eric Osborn
January 27, 2010
 Outline
 Definitions
 Embryology
 Epidemiology
 Complete transposition (D-TGA)
 Congenitally corrected transposition (L-TGA)
 Echocardiography
 Definitions
 The key anatomic characteristic of transposition
complexes is ventriculoarterial discordance.
 The aorta arises from the morphological RV
 The PA arises from the morphological LV
 Definitions
 Complete transposition (D-TGA)
 Atrioventricular concordance
 Definitions
 Congenitally corrected transposition (L-TGA)
 Atrioventricular discordance
 Embryology
 22 days gestation …
the primitive straight cardiac tube is formed
 Embryology
 23 days gestation … the straight cardiac tube elongates
and bends forming the cardiac loop.
 Cephalic portion bends ventrally, caudally, and right-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.
 Embryology
 4th-7th 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.
 Conotruncal cushions form the outflow tracts, aortic and
pulmonary roots.
 Embryology
 5th week gestation … the conotruncal cushions.
 Right superior truncal cushion grows distally and left-ward.
 Left inferior truncal cushion grows distally and right-ward.
 The net effect is a twisting motion.
 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.
 Embryology
 Mechanism of great artery transposition
 Conotruncal cushion defect
 Leads to failure of the conotruncal septum to spiral and
instead extends straight downward
 Aorta fuses with the RV and PA with the LV
 Epidemiology
 ~0.8% of live births are complicated by a
cardiovascular malformation*.
 >750,000 adult patients with congenital heart disease.
 Transposition of the great arteries occurs in
approximately 1 per 5,000 live births.
 More common in males
 Diagnosis possible in utero with fetal echocardiography
 Transvaginal ultrasound at 13-14 weeks (limited views)
 Transabdominal ultrasound at 16 weeks

*not including bicuspid aortic valve and mitral valve prolapse

Complete transposition (D-TGA)
 Pulmonary and systemic circulations are in parallel
 Lethal, if no mixing (ASD, PDA, VSD)
 ¾ are simple with no major associated abnormalities
 ¼ are complex
 VSD (16%)
 Pulmonary/subpulmonary stenosis (9%)
 Coarctation of the aorta (4%)
 Complete transposition (D-TGA)
Clinical Presentation and Outcomes
 Larger size and weight at birth
 Dyspnea and cyanosis
 Progressive hypoxemia
 Congestive heart failure

 Without treatment, the outlook is dismal

 30% mortality within the 1st week
 90% mortality within the 1st year
 Complete transposition (D-TGA)
Management
 Prostaglandin E1 to maintain the PDA
 Atrial septostomy (balloon or surgical)
 Palliative prior to corrective surgery
 Repair within the first days to weeks of life
 2-4% mortality with 90% 1 year survival
 Atrial switch
 Mustard or Senning
 Arterial switch
 Rastelli procedure
 Complete transposition (D-TGA)
Atrial switch (Mustard/Senning)
 Developed in the 1950s
 Baffle directs venous return to contralateral ventricle
 Complete transposition (D-TGA)
Atrial switch (Mustard/Senning)
 Disadvantages
 RV functions as the systemic ventricle
 Several significant long term complications
 Congestive heart failure
 Arrhythmias
 Baffle leaks and obstruction
 Pulmonary hypertension
 Paradoxial embolus
 Endocarditis

 Overall survival 75% at 25 years

 Senning may be better than Mustard [Moons et al, Heart 2004]
 340 patients (~⅔ Senning) compared
 Less obstruction (1 vs. 15%) and better functional class with Senning
 No significant mortality benefit
 Complete transposition (D-TGA)
Atrial switch (Mustard/Senning)
Arrhythmias
 Palpitations, presyncope, and syncope are not uncommon
 Both brady and tachyarrythmias frequently seen

 50% develop sinus node dysfunction

 Physical damage during surgery and baffle construction
 Disruption of blood supply leading to ischemia

 20% develop atrial flutter

 Sensitive to nodal agents due to conduction system disease

 11% required pacemakers at 20 years [Gelatt et al, J Am Coll Cardiol 1997]

 Pacemakers are difficult to place due to distorted anatomy

 Should be avoided if residual intracardiac communications due to risk of paradoxical
embolus and stroke
 Complete transposition (D-TGA)
Atrial switch (Mustard/Senning)
Congestive heart failure
 Most adult patients develop congestive heart failure
 By 20 years most are NYHA Class I or II

 RV filling compromised due to defects in baffle construction

 Baffle leaks (Mustard>Senning)
 Left-to-right shunts with pulmonary hypertension (7%)
 Risk of paradoxical embolus and stroke
 Indications for intervention include >1.5:1 left-to-right shunt or any right-to-left shunt
 Baffle obstruction (5-15%, Mustard>Senning)
 SVC>IVC manifesting as SVC syndrome or hepatic congestion/cirrhosis
 Often undetected due to collateral venous drainage (e.g. azygous vein)

 40% develop right ventricular dysfunction

 10-40% develop 2+ or greater tricuspid (systemic AV valve) regurgitation

 Annular dilatation from RV failure
 Damage from surgery or endocarditis
Complete transposition (D-TGA)
Suggested Follow-up
 Complete transposition (D-TGA)
Arterial switch
 Developed in the 1980s
 Great arteries and coronaries are transected and re-
anastamosed
 Complete transposition (D-TGA)
Arterial switch
 Advantages
 LV is the systemic pump
 No disruption of atrial conduction (sinus rhythm)

 Fewer long term complications compared to atrial switch

 Coronary ostial stenosis
 Supravalvular pulmonary/aortic stenosis
 Intervention indicated for RVOT gradient >50 mmHg
 Neoaortic regurgitation
 Arrhythmias

 Follow up with normal LV function and good exercise capacity

 Complete transposition (D-TGA)
Rastelli procedure
 TGA with VSD and LVOT obstruction

 Outcomes
 RV-PA conduit obstruction
 Exercise intolerance/angina
 RV failure
 Intervention for RV-PA
gradient >50 mmHg
 LV-Ao patch obstruction
 Dyspnea or syncope
 Complete transposition (D-TGA)
RV Failure after Atrial Switch
 Standard heart failure therapies are unproven

 The two-stage arterial switch

 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

 50% survival at 8 years in early results

 Appears to be more successful in patients under 12
Congenitally corrected transposition (L-TGA)

 A rare disorder that may present in adulthood.

 Associated anomalies (95% of patients)
 VSD (75%, commonly perimembranous)
 Pulmonary stenosis (75%, commonly subvalvular)
 Tricuspid valve anomalies (>75%)
 Congenital complete heart block (5%)
Congenitally corrected transposition (L-TGA)
Outcomes
 Arrhythmias
 Abnormal AV node and His positions
 Dual AV nodes
 2% per year incidence of complete heart block
 Susceptible to fibrosis of conduction system

 Median survival 40 years

 Mortality from progressive RV failure or arrhythmias

 Tricuspid regurgitation is major predictor
Congenitally corrected transposition (L-TGA)
Double Switch Procedure
 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
 75% concordance with abdominal situs (aorta and IVC positions)
3. Atrioventricular relationship
 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
 Curved contour of the aortic arch with three major branches
 Echocardiography
Complete Transposition with Atrial Switch
 Hallmark is parallel great arteries (parasternal long axis)
 Aorta is anterior to PA
 Echocardiography
Complete Transposition with Atrial Switch
 Systemic hypertrophied RV septum bows into LV
 May impact TR and enhance subpulmonary stenosis
 Echocardiography
Complete Transposition with Atrial Switch
 Aortic and pulmonic valves lie in the same plane
 Aorta is anterior and to the right (parasternal short axis)
 Echocardiography
Congenitally Corrected Transposition
 Hallmark is reversed offsetting of the AV valves
 Aorta is anterior and to the left (parasternal short axis)
 Echocardiography
Special Considerations
 Atrial switch
 RV function
 Tricuspid regurgitation
 Subpulmonary obstruction
 Baffle leak or obstruction (color Doppler)
 Normal baffle flow is phasic with peak velocity <1 m/sec
 Arterial switch
 Neoaortic valve regurgitation
 Supraneopulmonary valve stenosis
 Wall motion abnormalities due to coronary artery ostial stenosis
 Rastelli procedure
 LV-Ao tunnel patch obstruction
 RV-PA conduit degeneration (stenosis/regurgitation)
 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
 Prior endocarditis
 Congenital heart disease
 Unrepaired cyanotic, including palliative shunts and conduits
 Completely repaired with prosthetic material or device (6 months)
 Repaired with defects at or near a prosthetic device
 Post-cardiac transplant with valvular disease
Endocarditis Prophylaxis
 References
 Webb et al., Congenital Heart Disease in Braunwald’s Heart Disease, 8th ed., Chapter 61, 1561-1624.
 Sadler, Cardiovascular System in Langman’s Medical Embryology, 8th ed., Chapter 11, 208-259.
 Otto, The Adult with Congenital Heart Disease in Clinical Echocardiography, 4th ed., Chapter 17,
418-447.
 Warnes, Transposition of the Great Arteries, Circulation 2006 114:2699-2709.
 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
2008 5:454-67.
 Verhuegt et al., Long-term Prognosis of Congenital Heart Defects: A Systematic Review, Int J
Cardiol 2008 131:25-32.
 Skinner et al., Transposition of the Great Arteries: from Fetus to Adult, Heart 2008 94:1227-35
 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease, J Am Coll
Cardiol 2008 52:e1-121.