Debate in
Congenital Heart disease
• 6세
세 여아
여아,
VSD SA type, small,
mild
ild RCC prolapsed
l
d with
ith trivial
t i i l AR
( no progression
p g
for several years)
y
)
• Observation
b
i
Points of issue
• Natural course of SA VSD
• Post
Post-operative
operative course
– In patients with < slight AR
– In
I patients
ti t with
ith ≥ moderate
d t AR
• Risk factors for p
progression
g
of aortic
valve prolapse / aortic regurgitation
– Size of defect?
– Morphology / position of defect?
Natural course
Sim EKW et al. Am J Cardiol 1999
Tomita H et al. Circ J 2004
Post operative course
Post-operative
Tomita H et al. Am J Cardiol 2000
Cheung Y et al. Ann Thorac Surg 2002
Tomita H et al. Circ J 2004
Risk factors for progression of
aortic valve prolapse / aortic regurgitation
Small sized defect is risky?
Sim EKW et al. Ann Thorac Surg 1999
Sim EKW et al. Am J Cardiol 1999
T it H ett al.
Tomita
l Am
A J Cardiol
C di l 2000
Large sized
defect is risky?
•
Group I 75 patients. CHF (+), prolapse (-), AR (-)
Group II 102 patients. CHF (-), prolapse (+), AR (±)
Group III 37 patients
patients. CHF ((-),
) prolapse (-),
( ) AR ((-))
(Lun K et al. Am J Cardiol 2001)
Subclassification of SA VSD
• Synonyms
Conal, infundibular, subpulmonary, subpulmonic, supracrystal, subarterial,
doubly committed
committed, etc
①
②
③
④
Absence of conal septum
High muscular VSD with a muscular rim
Ci l defect
Circular
d f t underneath
d
th partt off the
th RCC
Crescentic defect underneath the RCC
(Brizard C. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2006)
Severity indices of
RCC prolapse
p
p
• RCC deformity index = A/B
• RCC imbalance index = R/L
T it H ett al.l Circ
Tomita
Ci J 2004
(< 0.3)
(< 1.3)