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Outcome in Adult Patients after Arterial Switch
Operation for Transposition of the Great Arteries
Aleksander Kempny, MD
Adult Congenital Heart Center and Center for Pulmonary Hypertension, Royal Brompton Hospital
London, UK
No disclosures.
Background
- Transposition of the great arteries (TGA) is one of the
most common neonatal cyanotic heart defects.
- Most patients require palliative or reparative
interventions shortly after birth.
- Definitive surgery, allowing the majority of patients to
survive to adulthood, can either consist of a physiologic
(atrial switch) or anatomic “correction” (arterial switch).
Background
Atrial switch
- Introduced in 1958 by Senning and later modified by Mustard
- Although early survival prospects are excellent, this procedure leaves
the right ventricle (RV) supporting the systemic circulation
Background
Arterial switch
- In 1975, Jatene reported a method for switching the great arteries and
re-implanting the coronary arteries.
- This method, with subsequent modifications, has nowadays become
the method of choice for repairing TGA.
- The pediatric outlook for patients surviving the operation has been
excellent, but uncertainty and some concerns about long-term
complications in adulthood remain, including:
1.
Aortic root dilatation
2.
Neo-aortic valve regurgitation
3.
Coronary lesions
4.
RVOT or PA lesions
Aortic root dilatation and aortic regurgitation
ML Schwartz et al, Long-Term Predictors of Aortic Root Dilatation and Aortic Regurgitation After
Arterial Switch Operation
Coronary complications
Legendre A, Losay J, Touchot-Kone A, et al. Coronary events after arterial switch operation for transposition of the
great arteries. Circulation 2003;108 Suppl 1:II186-90.
Coronary complications
Excentric intimal thickening in
most vessels
FU approx 10 years
? early atherosclerosis
Coronary complications
Coronary complications
Hauser M. Circulation 2001
Methods and Results
(n=145)
Methods and Results
Functional status
PVO2:
Entire cohort:
30.0±9.4mL/kg/min
70.8±17.7% of predicted PVO2, P<0.0001
Asymptomatic patients (NYHA I)
30.8±9.1mL/kg/min
75.1±16.4% of predicted PVO2, P<0.0001
Lower in patients with RVOT obstruction
24.4±10.8 vs. 31.1±8.7 mL/kg/min, P=0.047
Methods and Results
Coronary circulation
No patient required any percutaneous coronary intervention in childhood.
No acute coronary events in adulthood
In adulthood:
13 patients (9%) were suspected to have coronary disease, they underwent:
•
exercise test, CT
•
•
myocardial perfusion scintigraphy (4 pts.)
coronary angiography (5pts., including 2 patients who had both)
•
Myocardial ischemia confirmed in 3 patients
Methods and Results
Ventricular function
Left ventricle:
Ejection fraction (EF):
• 131 – normal (90%)
• 11 – mildly ↓
• 0 – moderate ↓
• 3 – severe ↓
MAPSE – 13.0±3.4mm, reduced (<10mm) in 15% of pts.
Right ventricle:
Longitudinal function was impaired in the majority of patients.
• TAPSE impaired (<16mm) in 67% of patients
• TDI – S impaired (<10cm/s) in 88% of patients.
Modest, but statistically significant correlation between TAPSE measurements and the
number of previous cardiac surgeries (r=-0.39, P=0.006).
Methods and Results
Aortic root and neo-aortic valve
Aortic root:
•
•
•
•
•
84% pts. abnormal dimensions of aortic root
54% dilated aortic sinus
Significant dilatation was rare (2.1%, maximal diameter 49mm)
No patient required aortic root surgery
Direct comparison of this data to those reported in previous
studies is not straightforward, most of all due to different normvalues for z-score estimation used in children and adults
Neo-aortic valve:
• Bicuspid 2 pts. (1.4%)
• Regurgitation:
• Severe
• Moderate
• Mild/trivial
1
7
34
• Patients with moderate or severe AoV regurgitation had a
significantly larger aortic sinus diameter compared to patients
with mild or no AoV regurgitation
(39.5±5.5 vs. 35.0±5.2mm, P=0.049)
• Only 1 of the 8 patients with more than mild AoV regurgitation
had aortic sinus diameter within normal limits
Roman MJ, et al. Am J Cardiol 1989;64:507–12.
Methods and Results
Right ventricular outflow tract (RVOT)
RVOT obstruction:
• Mild – 49 pts. (34.5%)
• Moderate/severe – 36 pts. (24.8%)
RVOT stenosis localisation:
• PA suture line
64
• PA branches
8
• Valvular
3
• Combined
10
Pulmonary regurgitation
• Severe
1
• Moderate
2
Methods and Results
Surgical and percutaneous interventions
Methods and Results
Predictors of outcome
Methods and Results
Predictors of outcome
Conclusions
• Mortality in adult patients after ASO is low
• Many patients (41%), however, require cardiac interventions or present with relevant
hemodynamic lesions.
• The most common lesion and intervention site is right ventricular outflow tract and
the pulmonary arteries.
• Clinically relevant coronary complications are infrequent and appear to be mainly
related to previous intraoperative complications.
• Our data do not support the need for routine invasive coronary assessment in this
young population.
• Due to the high prevalence of interventions and residual lesions regular follow up in
specialized centres is, however, warranted.
Thank you