B enedito C arlos M aciel, O sw aldo C esar de A lm eida Filho, A ndre S chm idt, Jose A ntonio M arin-N eto
V e n tric u la r fu n c tio n in C h a g a s' h e a rt d ise a se
Division of Cardiology, Department of Internal Medicine, Faculdade de Medicina de
Ribeirlio Pre to, Universidade de Slio Paulo - Slio Paulo, Brazil
In va sive a n d n o n in va sive m e th o d s u se d to e va lu a te ve n tricu la r fu n ctio n in C h a g a s's d ise a se a re re vie w e d . T h e tra d itio n a l in d ice s o f o ve ra ll ve n tricu la r p e rfo rm a n ce re fle G t th e in te ra ctio n o f p re lo a d , co n tra ctility, a fte rlo a d a n d h e a rt ra te . T h e re fo re , th e y a re u n a b le to d istin g u ish ch a n g e s in co n tra ctility fro m m o d ifica tio n s o f lo a d in g co n d itio n s. T h e ro le o f ve n tricu la r fu n ctio n a s a p re d icto r
o f m o rta lity in ch ro n ic C h a g a s' h e a rt d ise a se is d iscu sse d . V e n tricu la r fu n ctio n a b n o rm a litie s in p a tie n ts w ith in d e te rm in a te a n d d ig e stive fo rm s o f C h a g a s' d ise a se a re e sp e cia lly e m p h a size d . F in a lly, th e e vid e n ce o f e a rly im p a irm e n t o f d ia sto lic p e rfo rm a n ce in
p a tie n ts w ith C h a g a s' d ise a se is p re se n te d .
U N IT E R M S : C h a g a s' d ise a se . V e n tricu la r fu n ctio n .
IN T R O D U C T IO N
C
h r o n icim p o r ta n tC h a g a s 'c lin ic a lh e a r t d is e a s ef o r m o f C h a g a s 'd is e a s e .is c e r ta in ly th e m o s tT h e c h r o n ic c a r d ia c in v o lv e m e n t o f th is d is e a s e is m o r e p r e v a le n t, a n d it is a ls o a s s o c ia te d to la r g e r m o r b id ity a n d m o r ta lity , w h e n c o m p a r e d to d ig e s tiv e d is e a s e ( m e g a e s o p h a g u s a n d m e g a c o lo n ) ( 1 4 ,1 8 ) . D ia s ( 1 9 8 5 ) , in a lo n g itu d in a l s tu d y , d e s c r ib e d , s in c e th e a c u te in f e c tio n , a p r o g r e s s iv e in c r e a s e in th e p r o p o r tio n o f p a tie n ts w ithA ddress for correspondence: B enedito C arlos M aciel
D ivisao de C ardiologia, D epartam ento de C lfnica M edica
Faculdade de M edicina de R ibeirao P reto
U niversidade de S ao P aulo
R ibeirao P reto -S P -B rasil - C E P 14049-900
8 1 4
c a r d ia c d is e a s e f r o m th e s e c o n d ( 3 3 .8 % ) , to th ir d ( 4 9 .3 % ) a n d f o u r th ( 5 8 .1 % ) d e c a d e s o f f o llo w u p . T h e p r o g r e s s io n o f c a r d ia c in v o lv e m e n t is , h o w e v e r , h e te r o g e n e o u s , a n d te n d to b e s lo w : a b o u t 7 0 % o f c a r d ia c s u b je c ts w h o h a v e m ild c a r d ia c in v o lv e m e n t, w ith o u t h e a r t f a ilu r e , r e m a in c lin ic a lly s ta b le f o r p e r io d s r a n g in g f r o m 1 2 to 2 7 y e a r s o f f o llo w u p ( l) . T h e b a s ic m e c h a n is m s d e te r m in in g th e p r o g r e s s io n o f h e a r t d is e a s e in e a c h c h a g a s ic p a tie n t r e m a in u n k n o w n . T h e r e a r e , h o w e v e r , e v id e n c e s s u g g e s tin g th a t th e p r o g n o s is o f p a tie n ts w ith c h r o n ic C h a g a s ' h e a r t d is e a s e d e p e n d s o n th e c lin ic a l e x p r e s s io n o f h e a r t in v o lv e m e n t. B a s e lin e e le c tr o c a r d io g r a p h ic a b n o r m a litie s ( 2 9 ) , c a r d ia c a r r h y th m ia s ( 1 4 ) a n d le f t v e n tr ic u la r f u n c tio n ( 4 ) a r e r e c o g n iz e d a s im p o r ta n t r is k f a c to r s f o r in c r e a s e d m o r ta lity in th o s e p a tie n ts .
T h e m o s t im p o r ta n t h is to lo g ic a l f in d in g s in c h r o n ic C h a g a s ' h e a r t d is e a s e a r e : c h r o n ic d if f u s e m y o c a r d itis , in te r s titia l f ib r o s is , m y o c a r d ia l f ib e r h y p e r tr o p h y w ith o r w ith o u t f o c a l a tr o p h y a n d m y o c y to lis is ( 2 2 ,3 3 ) . A la r g e
n u m b e r o f p a tie n ts p re s e n t fo c a l th in n in g o f th e
m y o c a rd iu m o r a n e u ry s m a t th e le ft v e n tric u la r a p ic a l
re g io n . C lin ic a l m a n ife s ta tio n s o f th e c h ro n ic c a rd ia c fa s e
o f th e d is e a s e a re q u ite v a ria b le : p a tie n ts c a n b e
a s y m p to m a tic o r m a y p re s e n t d iffe re n t ty p e s o f a rrh y th m ia s
(in c lu d in g p o te n tia lly le th a l v e n tric u la r a rrh y th m ia s ),
a trio v e n tric u la r b lo c k , c o n d u c tio n a b n o rm a litie s o n
e le c tro c a rd io g ra m o r h e a rt fa ilu re . T h e c lin ic a l p ic tu re o f
h e a rt fa ilu re d u e to e x te n s iv e m y o c a rd ia l d a m a g e m im ic s
th a t o f id io p a th ic d ila te d c a rd io m y o p a th y (3 5 ). L e ft
v e n tric u la r d y s fu n c tio n , le ft v e n tric u la r a n e u ry s m a n d
c o n g e s tiv e h e a rt fa ilu re a re re c o g n iz e d a s im p o rta n t
p re d ic to rs o f m o rta lity in th o s e p a tie n ts . In a d d itio n , le ft
v e n tric u la r s y s to lic d y s fu n c tio n a p p e a rs to b e s ig n ific a n tly
c o rre la te d w ith c o m p le x v e n tric u la r a rrh y th m ia s (1 0 ) a n d
a ls o w ith c o n d u c tio n a b n o rm a litie s o n th e
e le c tro c a rd io g ra m (II).
M o s t o f th e in v a s iv e o r n o n in v a s iv e m e th o d s u s e d
s o fa r to e v a lu a te s y s to lic v e n tric u la r fu n c tio n in C h a g a s '
d is e a s e h a v e a n a ly s e d o v e ra ll p e rfo rm a n c e o f th e
v e n tric le s , re fle c tin g th e in te ra c tio n o f p re lo a d , a fte rlo a d ,
c o n tra c tility a n d h e a rt ra te (5 ). H e m o d y n a m ic s tu d ie s o f
p a tie n ts w ith th e c h ro n ic c a rd ia c fo rm h a v e s h o w n d a ta
c o m p a ra b le to th a t o b s e rv e d in d ila te d id io p a th ic
c a rd io m y o p a th y , in c lu d in g : in c re a s e d p u lm o n a ry a rte ry
a n d p u lm o n a ry w e d g e p re s s u re s a n d a ls o h ig h e n d -d ia s to lic
v e n tric u la r p re s s u re s ; in c re a s e d rig h t a n d /o r le ft v e n tric u la r
v o lu m e s a n d re d u c e d .v e n tric u la r e je c tio n fra c tio n (4 1 ).
T h e m a g n itu d e o f th e s e c h a n g e s is q u ite v a ria b le
d e p e n d in g o n th e d e g re e o f m y o c a rd ia l in v o lv e m e n t in
e a c h p a tie n t. S e g m e n ta l le ft v e n tric u la r w a ll m o tio n
a n a ly s is b a s e d o n e c h o c a rd io g ra p h y a n d ra d io n u c lid e o r
c o n tra s t a n g io g ra p h y h a v e s h o w n a n u m b e r o f
a b n o rm a litie s in c h ro n ic c a rd ia c c h a g a s ic p a tie n ts
(1 ,2 ,4 ,9 ,4 1 ): a p ic a l d y s k in e s ia w a s d o c u m e n te d in 5 0 -6 5 %
o f s y m p to m a tic c a rd ia c p a tie n ts , w h ile a k in e s ia o r
h y p o k in e s ia w e re o b s e rv e d in th e s a m e re g io n in
a p ro x im a te ly 2 0 % o f p a tie n ts (F ig u re I); o n th e o th e r h a n d ,
w a ll m o tio n a b n o rm a litie s in v o lv in g p o s te ro -in fe rio r w a ll
a re re p o rte d in 1 5 -2 0 % o f p a tie n ts a n d ra re ly a t la te ra l
w a ll; fin a lly , d iffu s e le ft v e n tric u la r h y p o k in e s ia w a s
o b s e rv e d in 1 5 -4 0 % o f p a tie n ts . It is im p o rta n t to m e n tio n
th a t, in a s ig n ific a n t p ro p o rtio n (3 1 % ) o f c h ro n ic c a rd ia c
p a tie n ts , s e g m e n ta l w a ll m o tio n a b n o rm a litie s in m o re th a n
o n e re g io n h a s b e e n d o c u m e n te d (4 ). It is a ls o re le v a n t
th a t th is v a ria b le p a tte rn o f s e g m e n ta l in v o lv e m e n t o f th e
h e a rt is n o t re la te d to o b s tru c tiv e le s io n s a t e p ic a rd ia l
c o ro n a ry a rte rie s , w h ic h a re u s u a lly n o rm a l o r e v e n d ila te d
in c h a g a s ic p a tie n ts . T h e a n a ly s is o f rig h t v e n tric u la r
fu n c tio n in c h ro n ic C h a g a s ' h e a rt d is e a s e h a s b e e n
re la tiv e ly n e g le c te d s o fa r. M a rin -N e to e t a I. (3 0 ) h a v e
S a o P a u lo M e d ic a l J o u r n a l/R P M 1 1 3 ( 2 ) M a r /A p r 1 9 9 5
s h o w n a n im p o rta n t im p a irm e n t o f rig h t v e n tric u la r s y s to lic
fu n c tio n in th is c lin ic a l s e ttin g , o c u rrin g e v e n in th o s e
p a tie n ts w ith n o rm a l o v e ra ll le ft v e n tric u la r fu n c tio n a n d
w ith o u t a n y c lin ic a l e v id e n c e o f h e a rt d is e a s e (F ig u re s 2
a n d 3 ). R ig h t v e n tric u la r a n e u ry s m s h a v e b e e n re p o rte d
Figure
1 . E n d - d ia s to lic a n d e n d - s y s to lic le ft v e n tr ic u la r a n g io g r a p h ic fr a m e s in a p a tie n t w ith c h r o n ic C h a g a s ' h e a r t d is e a s e s h o w in g a p ic a l a n e u r y s m a n d in fe r io r d y s k in e s ia . In th e lo w e r h a lf o f th e fig u r e n o r m a l c o r o n a r y a r te r ie s a r e s h o w n .in 1 0 % o f c h ro n ic c h a g a s ic p a tie n ts e v a lu a te d b y
e c h o c a rd io g ra p h y (I).
T h e in d e te rm in a te fo rm o f C h a g a s ' d is e a s e h a s b e e n
e x te n s iv e ly in v e s tig a te d re c e n tly to d e te rm in e w h e th e r o r
n o t th e re a re e v id e n c e s , in th e s e p a tie n ts , o f a n y d e g re e o f
d ig e s tiv e o r m y o c a rd ia l in v o lv e m e n t. P a tie n ts a re in c lu d e d
in th is c lin ic a l fo rm w h e n th e y h a v e a p o s itiv e s e ro lo g ic a l
te s t fo r C h a g a s ' d is e a s e , n o s y m p to m s a n d n o rm a l
e le c tro c a rd io g ra m a n d x -ra y e x a m in a tio n o f h e a rt,
e s o p h a g u s a n d c o lo n (3 9 ). D iffe re n t d ia g n o s tic te c h n iq u e s
u s e d to s tu d y p a tie n ts w ith th e c h ro n ic in d e te rm in a te fo rm
o f C h a g a s ' d is e a s e h a v e s h o w n s o m e d e g re e o f im p a irm e n t
o f d ig e s tiv e o r h e a rt fu n c tio n , b u t th e re s u lts o f th o s e
in v e s tig a tio n s n e e d e d to b e c ritic a lly re v ie w e d : th e c rite ria
fo r in c lu d in g p a tie n ts in th e in d e te rm in a te fo rm a n d fo r
a n a ly s i s o f e le c tro c a rd io g ra m re s u I ts w e re n o t
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2 . E n d - d ia s to lic a n d e n d - s y s to lic im a g e s o f r a d io n u c lid e a n g io g r a m in a n a s y m p to m a tic c h a g a s ic p a tie n t s h o w in g is o la te dim p a ir m e n t o f r ig h t v e n tr ic u la r s y s to lic fu n c tio n .
Figure
3 . R a d io n u c lid e le ft a n d r ig h t v e n tr ic u la r e je c tio n fr a c tio n in g r o u p 1 ( n o n c a r d ia c ) a n d g r o u p 2 ( c a r d ia c ) c h a g a s ic p a tie n ts .H o r iz o n ta l b a r r e p r e s e n t a v e r a g e v a lu e s in e a c h g r o u p . T h e
h a tc h e d a r e a s in d ic a te th e n o r m a l r a n g e ( 2 S O o f m e a n ) .
h o m o g e n e o u s ; in s o m e s tu d ie s , a c o n tro l g ro u p w a s n o t
in c lu d e d (1 6 ,3 6 ,4 2 ). D e p e n d in g o n th e d ia g n o s tic m e th o d
u tiliz e d , a v a ria b le p ro p o rtio n o f a b n o rm a litie s o f h e a rt
. fu n c tio n h a s b e e n re p o rte d . T h e re fo re , a b n o rm a litie s w e re
d e s c rib e d in 6 8 -8 8 % o f p a tie n ts (2 6 ,3 8 ) s tu d ie d b y
v e c to c a rd io g ra p h y ; 7 3 % o f p a tie n ts h a d a n e x e rc is e s tre s s
te s t c o n s id e re d a b n o rm a l (3 8 ); a b n o rm a l m y o c a rd ia l 9 9 m
te c h n e tiu m -p y ro p h o s p h a te u p ta k e w a s fo u n d in 8 2 % o f
p a tie n ts ; H o lte r m o n ito rin g w a s re p o rte d a b n o rm a l in 4 2
-5 0 % o f c a s e s (2 7 ,3 8 ); a tria l d ro m o tro p ic a b n o rm a litie s
d u rin g e le c tro p h y s io lo g ic s tu d ie s w e re o b s e rv e d in 5 0 %
o f p a tie n ts (1 7 ) a n d in 6 1 % o f c a s e s s tu d ie d b y
e n d o m y o c a rd ia l b io p s y a n a b n o rm a l h y s to lo g ic a l p a tte rn
w a s d o c u m e n te d . P e re ira -B a rre to e t a l. (3 8 ) h a v e s h o w n
in a g ro u p o f 2 2 p a tie n ts w ith th e c h ro n ic in d e te rm in a te
fo rm o f C h a g a s ' d is e a s e th a t a ll p a tie n ts h a d a t le a s t o n e
o u t o f s ix d ia g n o s tic s m e th o d s a p p lie d (v e c to c a rd io g ra p h y ,
e c h o c a rd io g ra p h y , ra d io n u c lid e v e n tric u lo g ra p h y ,
m y o c a rd ia l u p ta k e o f 9 9 m -te c h n e tiu m p y ro p h o s p h a te ,
e x e rc is e s tre s s te s t, H o lte r m o n ito rin g ) c o n s id e re d a s
a b n o rm a l. T h e p ro g n o s tic im p lic a tio n s o f th e s e
a b n o rm a litie s to in flu e n c e p ro g re s s o f th e d is e a s e to th e
s y m p to m a tic c lin ic a l fo rm s o r re g a rd in g m o rta lity a re
c u rre n tly u n k n o w n .
L e ft v e n tric u la r s y s to lic fu n c tio n h a s b e e n e v a lu a te d
in p a tie n ts in c lu d e d in th e in d e te rm in a te fo rm o f C h a g a s '
d is e a s e u s in g d iffe re n t m e th o d s . S tu d ie s w ith c o n tra s t
v e n tric u lo g ra p h y re p o rte d s e g m e n ta l le ft v e n tric u la r w a ll
m o tio n a b n o rm a litie s in u p to 5 0 % o f p a tie n ts , in c lu d in g
2 5 -3 7 % o f c a s e s w ith a p ic a l a k in e s ia o r d y s k in e s ia , 1 5
-2 0 % o f p a tie n ts w ith d iffu s e h y p o k in e s ia , 3 .3 % o f c a s e s
w ith a k in e s ia o f a n te rio r w a ll a n d 1 0 % o f p a tie n ts w ith
m o re th a n o n e s e g m e n ta l a b n o rm a lity d o c u m e n te d
(2 3 ,2 8 ,4 0 ). T h o s e in v a s iv e s tu d ie s h a v e a ls o s h o w n a n
in c re a s e d le ft v e n tric u la r e n d -d ia s to lic p re s s u re o c c u rrin g
in 4 0 to 1 0 0 % o f p a tie n ts e v a lu a te d (2 3 ,2 8 ,4 0 ). H o w e v e r,
w h e n c o m p a rin g 2 3 p a tie n ts w ith th e in d e te rm in a te fo rm
a n d n o rm a l s u b je c ts , M a d y e t a l. (2 4 ) d e s c rib e d a m in o r
b u t s ig n ific a n t in c re a s e o f rig h t v e n tric u la r d ia s to lic
p re s s u re in th e c h a g a s ic g ro u p , a s th e o n ly h e m o d y n a m IC
a b n o rm a lity th a t d is tin g u is h e d n o rm a l s u b je c ts fro m th e
c h a g a s ic g ro u p . In a d d itio n , in th is in v e s tig a tio n (2 4 ) le ft
v e n tric u la r a p ic a l d y s k in e s ia w a s n o t d o c u m e n te d b y
c o n tra s t v e n tric u lo g ra p h y .
D is c re p a n t o b s e rv a tio n s h a v e a ls o b e e n re p o rte d in
s tu d ie s u s in g n o n in v a s iv e m e th o d s o f le ft v e n tric u la r
fu n c tio n a n a ly s is . L e ft v e n tric u la r a p ic a l d y s k in e s ia o r
a k in e s ia h a v e b e e n o b s e rv e d in 1 0 -4 0 % o f p a tie n ts w ith
in d e te rm in a te fo rm e v a lu a te d b y tw o -d im e n s io n a l
e c h o c a rd io g ra p h y (1 ,2 ,3 8 ), w h ile le ft v e n tric u la r p o s te ro
-a p ic -a l h y p o k in e s i-a w a s th e m a in a b n o rm a lity re p o rte d b y
O rtiz e t a l. (3 4 ) (2 2 % o f c a s e s ), w h o a ls o fo u n d d iffu s e
h y p o k in e s ia in 4 % o f th e in d e te rm in a te c h a g a s ic p a tie n ts .
In s tu d ie s u s in g M -m o d e e c h o c a rd io g ra p h y , s o m e
in v e s tig a to rs (3 ,1 9 ,3 7 ) h a v e re p o rte d m ild in c re a s e o f le ft
v e n tric u la r d ia s to lic d im e n s io n s a n d /o r a m in o r im p a irm e " n t
o f le ft v e n tric u la r s y s to lic fu n c tio n in in d e te rm in a te
c h a g a s ic p a tie n ts w h ile o th e r s tu d ie s d id n o t s h o w a n y
d iffe re n c e s in th o s e v a ria b le s w h e n c o m p a rin g c h a g a s ic
a n d n o rm a l s u b je c ts (2 ,3 8 ,4 2 ). L e ft v e n tric u la r e je c tio n
fra c tio n u n d e r re s tin g c o n d itio n s , a s e v a lu a te d b y
ra d io n u c lid e a n g io g ra p h y , w a s n o t d iffe re n t w h e n
c o m p a rin g n o rm a l a n d in d e te rm in a te p a tie n ts (2 1 ,3 1 ,3 8 ).
O n th e o th e r h a n d , le ft v e n tric u la r s e g m e n ta l w a ll m o tio n
a n a ly s is in th e s e s tu d ie s h a v e s h o w n a p ic a l h y p o k in e s ia
in u p to 6 8 % o f c a s e s (3 1 ,3 8 ) o r e v e n a b s e n c e o f a n y
s e g m e n ta l w a ll m o tio n a b n o rm a lity (2 1 ). In a re c e n t s tu d y
(3 0 ), M a rin -N e to e t a l. d o c u m e n te d , in a g ro u p o f 5 p a tie n ts
w ith th e in d e te rm in a te fo rm o f C h a g a s ' d is e a s e , th a t w h ile
th e le ft v e n tric u la r e je c tio n fra c tio n w a s n o rm a l a s
e v a lu a te d b y ra d io n u c lid e v e n tric u lo g ra p h y , rig h t
v e n tric u la r s y s to lic fu n c tio n w a s s ig n ific a n tly d e p re s s e d ,
in d ic a tin g th a t rig h t v e n tric u la r d y s fu n c tio n c a n b e th e o n li
a b n o rm a lity in th is g ro u p o f p a tie n ts .
S o u s a (4 2 ) in v e s tig a te d in o u r la b o ra to ry le ft
v e n tric u la r s y s to lic fu n c tio n b y tw o -d im e n s io n a l
e c h o c a rd io g ra p h y d u rin g s tre s s c o n d itio n s . F ifte e n p a tie n ts
rig o ro u s ly c la s s ifie d in th e in d e te rm in a te fo rm o f C h a g a s '
d is e a s e w e re e v a lu a te d d u rin g is o m e tric e x e rc is e a n d a fte r
a d m in is tra tio n o f is o s o rb id e d in itra te . E je c tio n p h a s e
in d ic e s o f o v e ra ll le ft v e n tric u la r s y s to lic p e rfo rm a n c e , a s
p e rc e n t fra c tio n a l s h o rte n in g o r v e lo c ity o f le ft v e n tric u la r
s h o rte n in g , w e re c o m p a ra b le in n o rm a ls a n d in d e te rm in a te
p a tie n ts u n d e r re s tin g a n d s tre s s c o n d itio n s , b u t s e g m e n ta l
q u a n tita tiv e w a ll m o tio n a n a ly s is h a v e s h o w n a m in o r
im p a irm e n t o f s y s to lic fu n tio n a t th e p o s te rio r w a ll in
c h a g a s ic p a tie n ts d u rin g is o m e tric e x e rc is e , s u g g e s tin g a
re d u c e d fu n c tio n a l c a rd io v a s c u la r re s e rv e in th e s e p a tie n ts .
F u rth e rm o re , th is s tu d y d e m o n s tra te d th a t p a tie n ts w ith
th e d ig e s tiv e fo rm o f C h a g a s ' d is e a s e h a v e a m o re
im p o rta n t im p a irm e n t o f s e g m e n ta l le ft v e n tric u la r fu n c tio n
d u rin g e x e rc is e a s c o m p a re d to in d e te rm in a te p a tie n ts ,
s u g g e s tin g th a t s im u lta n e o u s d ig e s tiv e a n d c a rd ia c
in v o lv e m e n t o f th is d is e a s e c o u ld b e a fre q u e n t a s s o c ia tio n .
S im ila r re s u lts s h o w in g s im u lta n e o u s d ig e s tiv e a n d c a rd ia c
in v o lv e m e n t w e re p u b lis h e d la te r b y M o ra e s F ilh o e t a l.
(3 2 ). P a tie n ts w ith d ig e s tiv e a n d in d e te rm in a te fo rm s o f
C h a g a s ' d is e a s e a ls o h a d a n im p ro v e m e n t o f le ft
v e n tric u la r fu n c tio n a fte r re c e iv e in g s u b lin g u a l is o s o rb id e
d in itra te (4 2 ). T h is p a tte rn o f re s p o n s e w a s n o t o b s e rv e d
in n o rm a l s u b je c ts , re in fo rc in g th e im p re s s io n th a t th e s e
g ro u p s o f c h a g a s ic p a tie n ts h a d a n im p a irm e n t o f
fu n c tio n a l c a rd ia c re s e rv e a n d , a ls o , a d v e rs e b a s a l
c o n d itio n s o f p re lo a d a n d a fte rlo a d .
D e s p ite th e d is c re p a n t o b s e rv a tio n s p u b li-s h e d
re g a rd in g m y o c a rd ia l in v o lv e m e n t in p a tie n ts w ith th e
in d e te rm in a te fo rm o f C h a g a s ' d is e a s e , a n d e v e n
c o n s id e rin g th e lim ita tio n s o f s o m e in v e s tig a tio n s re la te d
to in a d e q u a te c lin ic a l c h a ra c te riz a tio n o f th e in d e te rm in a te
fo rm , it s h o u ld b e re c o g n iz e d th a t a s u b s ta n tia l p ro p o rtio n
o f th e s e p a tie n ts c a n h a v e m in o r im p a irm e n t o f le ft
v e n tric u la r s y s to lic fu n c tio n . It is a ls o im p o rta n t to m e n tio n
th a t, a c c o rd in g to th e a v a ila b le d a ta , p a tie n ts w ith
in d e te rm in a te fo rm h a v e a n e x c e le n t lo n g te rm p ro g n o s is
(1 8 ).
Q u a n tita tiv e e v a lu a tio n o f m y o c a rd ia l c o n tra c tility ,
a n in trin s ic p ro p e rty o f v e n tric u la r m u s c le fib e r, is a q u ite
c o m p le x a n d c o n tro v e rs ia l is s u e . O v e ra ll le ft v e n tric u la r
s y s to lic p e rfo rm a n c e d e p e n d s o n th e b e a t-to -b e a t
in te ra c tio n o f m y o c a rd ia l c o n tra c tility , a fte rlo a d , p re lo a d
a n d h e a rt ra te . T ra d itio n a l a p p ro a c h e s to s tu d y le ft
v e n tric u la r p e rfo rm a n c e a re u n a b le to d iffe re n tia te c h a n g e s
in c o n tra c tility fro m m o d ific a tio n s o f th e lo a d in g
c o n d itio n s o r h e a rt ra te (5 ). T h is is a n im p o rta n t lim ita tio n
o f e je c tio n p h a s e in d ic e s in c lu d in g p e rc e n t fra c tio n a l
s h o rte n in g , m e a n v e lo c ity o f le ft v e n tric u la r fib e r
s h o rte n in g , e je c tio n fra c tio n , s tro k e v o lu m e o r c a rd ia c
o u tp u t a n d a ls o o f le ft v e n tric u la r w a ll m o tio n a n a ly s is .
Is o v o lu m ic in d ic e s o f le ft v e n tric u la r s y s to lic p e rfo rm a n c e ,
o b ta in e d d u rin g th e p re s s u re v a ria tio n a t th e p re -e je c tio n
p e rio d , a s th e m a x im a l ra te o f le ft v e n tric u la r p re s s u re ris e
(dP/dt
m a x ), a re s e n s itiv e to m y o c a rd ia l c o n tra c tility b u t a ls o d e p e n d o n p re lo a d c o n d itio n s .R e c e n tly , s e v e ra l in d ic e s o f le ft v e n tric u la r
p e rfo rm a n c e re la tin g p h y s io lo g ic fo rc e s a c tin g o n th e
m y o c a rd iu m a t e n d -s y s to le h a v e b e e n s h o w n to b e s e n s itiv e
to m y o c a rd ia l c o n tra c tility a n d in d e p e n d e n t o f lo a d in g
c o n d itio n s . E x p e rim e n ta l s tu d ie s h a v e d e m o n s tra te d th a t,
a s a fte rlo a d in c re a s e s , th e e x te n t o f v e n tric u la r s y s to lic
fib e r s h o rte n in g fa lls , re s u ltin g in p ro g re s s iv e ly g re a te r
e n d -s y s to lic fib e r le n g th a n d v e n tric u la r d im e n s io n s . In
a d d itio n , o v e r th e p h y s io lo g ic ra n g e , a lin e a r re la tio n s h ip
h a s b e e n o b s e rv e d b e tw e e n le ft v e n tric u la r e n d -s y s to lic
d im e n s io n a n d a fte rlo a d (4 3 ). T h e s lo p e o f th e le ft
v e n tric u la r e n d -s y s to lic p re s s u re -d im e n s io n ( o r v o lu m e )
re la tio n h a s b e e n p ro p o s e d in th e c lin ic a l s e ttin g a s a
re lia b le in d e x o f c o n tra c tility , c o n s id e rin g th a t it is
in d e p e n d e n t o f p re lo a d , in c o rp o ra te s a fte rlo a d a n d v a rie s
d ire c tly w ith m o d ific a tio n s o f th e m y o c a rd ia l c o n tra c tile
s ta te (6 ,7 ,8 ). A n o th e r s e n s itiv e in d e x o f c o n tra c tility re la te d
to th e p h y s io lo g ic e v e n ts a t e n d -s y s to le is o b ta in e d th ro u g h
th e re la tio n s h ip b e tw e e n le ft v e n tric u la r e n d -s y s to lic
m e rid io n a l w a ll s tre s s a n d th e ra te -c o rre c te d v e lo c ity o f
fib e r s h o rte n in g (1 2 ). T h e s e in d ic e s h a v e a n a d d itio n a l
a d v a n ta g e : th e y c a n b e o b ta in e d n o n in v a s iv e ly b y c h a n g in g
a fte rlo a d w ith v a s o a c tiv e d ru g s a n d e s tim a tin g e n d -s y s to lic
p re s s u re s u s in g c a lib ra te d c a ro tid p u ls e tra c in g s w h ile le ft
v e n tric u la r e n d -s y s to lic d im e n s io n s (o r v o lu m e s ) a re
m e a s u re d .u s in g M -m o d e e c h o c a rd io g ra p h y .
A lm e id a -F ilh o e t a l. (1 9 9 3 , u n p u b fis h e d re s u lts )
m e a s u re d , in o u r la b o ra to ry , th e s lo p e o f th e le ft v e n tric u la r
e n d -s y s to lic p re s s u re -d im e n s io n re la tio n s h ip in p a tie n ts
w ith th e in d e te rm in a te , d ig e s tiv e a n d c a rd ia c fo rm s o f
C h a g a s ' d is e a s e c o m p a re d to a n o rm a l g ro u p o f s u b je c ts .
250
150 200
5 0 , 345
End-sistolie dimension (em) 450
400
100
en
I 350
E
E-O> 300
:5
en en~
0 .
.g (5
iii
'w
-6
c :
w
d isfu n c tio n ,
e v e n in p a tie n ts
w ith th e in d e te rm in a te
fo rm
o f th e d ise a se . In a d d itio n , th e re a re so m e d a ta su g g e stin g
th a t th e im p a irm e n t
o f d ia sto lic
fu n c tio n
o c c u rs p rio r to
le ft v e n tric u la r
sy sto lic d isfu n c tio n
in th e n a tu ra l h isto ry
o f C h a g a s'
d ise a se .
Figure
4. Linear regression analysis of left ventricular end-systolic pressure-dimension relationship in a normal subject and in patients with indeterminate, digestive and cardiac forms of Chagas' disease.A ll p a tie n ts
h a d a n o rm a l o v e ra ll le ft v e n tric u la r
sy sto lic
p e rfo rm a n c e
a s
e v a lu a te d
b y
tw o -d im e n sio n a l
e c h o c a rd io g ra p h y
u n d e r re stin g c o n d itio n s, d e sp ite th e fa c t
th a t so m e c h a g a sic
p a tie n ts
w ith d iffe re n t c lin ic a l fo rm s
h a d
a m in o r
se g m e n ta l
w a ll
m o tio n
a b n o rm a lity .
T ra d itio n a l in d ic e s o f le ft v e n tric u la r sy sto lic p e rfo rm a n c e ,
a s p e rc e n t fra c tio n a l
sh o rte n in g
a n d m e a n v e lo c ity o f fib e r
sh o rte n in g , w e re n o t a b le to d iffe re n tia te
th e th re e c h a g a sic
g ro u p s fro m n o rm a ls b o th in b a se lin e c o n d itio n s
a n d a fte r
in tra v e n o u s
p h e n y le p h rin e
a d m in istra tio n .
H o w e v e r,
th e
slo p e
o f p re ssu re -d im e n sio n
re la tio n
w a s sig n ific a n tly
lo w e r in th e th re e c h a g a sic g ro u p s c o m p a re d to th e n o rm a l
su b je c ts (F ig u re 4 ). In a d d itio n , c h a g a sic p a tie n ts w h o h a d
a b a se lin e
w a ll m o tio n
a b n o rm a lity
o n tw o -d im e n sio n a l
e c h o c a rd io g ra m
h a d th e lo w e r
in d ic e s
o f m y o c a rd ia l
c o n tra c tility
fo r e a c p c h a g a sic g ro u p . T h e se d a ta in d ic a te d
th a t th e le ft v e n tric u la r
e n d -sy sto lic
p re ssu re -d im e n sio n
re la tio n
is a se n sitiv e
in d e x
o f c o n tra c tility ,
u se fu l
fo r
in v e stig a tio n s
in th e c h a g a sic
p o p u la tio n .
T h e se
re su lts
a lso su g g e st th a t m in o r b a se lin e le ft v e n tric u la r w a ll m o tio n
a b n o rm a lity
o n e c h o c a rd io g ra p h ic
stu d y is a d e te rm in in g
fa c to r fo r lo w e r o v e ra ll
c o n tra c tility
in d e x , in d e p e n d e n t
o f th e c lin ic a l
fo rm
o f C h a g a s'
d ise a se .
T h e lo n g te rm
p ro g n o stic
v a lu e o f th is c h a n g e s,
h o w e v e r, re m a in s to b e
d e te rm in e d .
L e ft
v e n tric u la r
d ia sto lic
fu n c tio n
h a s n o t b e e n
e x te n siv e ly
e v a lu a te d
in C h a g a s'
d ise a se .
S tu d ie s
u sin g
c o m p u te riz e d
e c h o c a rd io g ra p h ic
(M -m o d e ) a n a ly sis o f le ft
v e n tric u la r
w a ll m o tio n
(1 3 ,4 2 )
o r sp e c tra l D o p p le r
(1 5 )
o b ta in e d
re su lts
in d ic a tin g
a le ft v e n tric u la r
d ia sto lic
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