ECHO 2013 / Cardiac Magnetic Resonance Imaging in the Adult Congenital Patient
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Case: RA-to-PA Fontan
•37 year-old man with double inlet left ventricle, status post RA-to-PA Fontan procedure
Case: Lateral Tunnel Fontan
•36 year-old man with double inlet left ventricle with L- transposition of the great arteries, s/p lateral tunnel Fontan procedure
Case: TGA/VSD
•37 year-old man with no prior cardiac surgery
–Murmur and cyanosis at birth
–Work-ups have shown “inoperable” heart disease
•Progressive SOB on exertion
•Occasional palpitations
Physical Examination
•Situs inversus in abdomen
•Levo/mesocardia: apical impulse at LLSB
•No significant JVD, hepatomegaly
•Grade 4/6 “continuous” murmur RUSB
•Clubbing, full pulses
•BP = 120/50 LUE and LLE
•Oxygen saturation = 83-85% at rest, low 70’s with exertion
Axial Bright Blood
4C and Short Axis Bright Blood
Gd-Enhanced MRA (3D)
Physiologically Corrected TGA {I, D, D}
with VSD, PS
Box Diagram
LA RA
AV Discordance
RV LV
VA Discordance
Catheterization
RPCW=9
75% |
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52% |
LSVC |
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23/16, |
82% |
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m= 19 |
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125/40, (95%) |
110/45, |
23/16, |
76% LPCW=9 |
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m= 70 |
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m= 70 |
MPA m= 19 |
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54% |
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LA |
Ao |
mean=12 |
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RA |
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LIVC |
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50% |
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110/10 |
110/10 |
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84% 59%
RV LV
•Bidirectional shunt with net Qp/Qs = 1.5:1
•Low CI = 2.4 l/min/m2
•Mildly incr. filling pressures
•No PFO by probing
•Non-restrictive VSD
•No aortic outflow obstruction
•Wide aortic pulse pressure
•Severe PS, normal PA pressure
•No branch PS
•PVRi = 2.8 Wood units
Surgical Options
•VSD closure with LV-to-PA conduit
•“Double switch” procedure with Mustard and Rastelli procedures (and possible pacemaker)
LA RA
LA RA
NRA NLA
RV |
LV |
RV |
LV |