Cardiomyopathie hypertrophique

Mise à jour de la cardiomyopathie hypertrophique selon les
recommandations ACC/AHA 2011 par François Boustani
Diagnostic*de*la*CMH*:*
!
•"#$! %&&! '()*+#,$! -! .#$! +#/$'0+(#! +#0123$+#$.4$! 5$! 61(5.+0! 5$! '(#01240$! $40!
12+4(##2*7$! $#! '24! 5$! 8(1)$! 26+'27$! (.! 59:;<! 26+'27! 4+! 79:=<! #9$40! 624! 1,27+42*7$!
>?@@A?B?!::2CD!
•E9,'F(5(667$1! 59$88(10! $40! 12+4(##2*7$! -! 72! 1$'F$1'F$! 59.#$! (*401.'0+(#! >?@@A?B?!
::2CG! 27(14! H.$! 7$! #+01+0$! 592)I7$! $0! 79+4.61$7! 4(#0! 52#J$1$.K! $0! 72! )2#L.31$! 5$!
M2742732!6$.!4$#4+*7$D!!
•E9,'F(5(*.02)+#$!$40!'(#01$N+#5+H.,$!'21!6$.0!(''24+(##$1!5$4!01(.*7$4!5$!1I0F)$!
$0!$77$!#$!1$61(5.+0!624!79$88(10!6FI4+(7(J+H.$D!
•E9:=<! $40! 12+4(##2*7$! 6(.1! 1$'F$1'F$1! 79FI6$101(6F+$! 26+'27$! (.! 1$'F$1'F$1! .#!
2#,31I4)$!26+'27!>?@@A?B?!::2CD!
•E9:=<!-!72!1$'F$1'F$!59.#!1,F2.44$)$#0!0215+8!6$.0!O01$!$#3+42J,$!4+!72!40120+8+'20+(#!
5.!1+4H.$!5$!)(10!4.*+0$!#9$40!624!'(#'7.2#0$!>?@@A?B?!::*CD!
•E9:=<!6$.0!O01$!$#3+42J,$!$#!'24!59BMP!23$'!4.46+'+(#!5$!5+2J#(40+'!5+88,1$#0+$7!5$!72!
@<B!>2)I7(4$!'215+2H.$G!)2725+$!5$!Q2*1IRC!>?@@A?B?!::*CD!
!!!!!
!
!
!
*
*
*
!
*
*
*
*
*
*
*
*
*
*
*
*
*
*
*
*
*
*
*
*
*
Recherche*d’une*ischémie*en*cas*de*CMH!S!
!
L’echo*d’effort!S!$40!.0+7$!6(.1!(*/$'0+3$1!.#$! (*401.'0+(#G! )2+4! +7! $40! )(+#4! 8+2*7$!6(.1!
5,0$'0$1!.#$!'(1(#21(620F+$!'21!792#27I4$!5$!72!'+#,0+H.$!4$J)$#02+1$!$40!5+88+'+7$!$0!$77$!
$40! 6$10.1*,$! 621! 792.J)$#020+(#! 5$4! '(#5+0+(#4! 5$! 'F21J$! 7+,$! -! 79(*401.'0+(#D! @$0!
$K2)$#!$40!'(#01$N+#5+H.,!4+!7$!J125+$#0!+#012!MP!T!UV!))BJD!!
!
La*scintigraphie!myocardique*:*J12#5$*81,H.$#'$!5$4!82.K!6(4+0+84!42#4!200$+#0$!5$4!J1(4!
01(#'4G!'$4!2#()27+$4!,02#0!5.$4!-!.#$!200$+#0$!5$!72!)+'1('+1'.720+(#D!!
E2!Q%MP!6$.0!O01$!4(.4N$40+),$!621!72!5+88+'.70,!5$!5,0$'0+(#!5$!72!*(15.1$!$#5('215+H.$!
5.!82+0!59.#$!FI6$18+K20+(#!52#4!72!@<BD!
!
L’IRM* de* stress*:* $#! 61,4$#'$! 59.#! J125+$#0! 5$! *24$! TUV! ))BJG! 7W:=<! 5$! 401$44! $40!
'(#01$N+#5+H.,$! >2.44+! *+$#! 5+6I1+52)(7$G! H.$! 25,#(4+#$! (.! 5(*.02)+#$CD! %#! 7W2*4$#'$!
5$! J125+$#0G! 7$4! +)2J$4! 6$1)$00$#0! 5$! *+$#! 5+40+#J.$1! $#01$! 5$4! 2#()27+$4! 5$! 72!
)+'1('+1'.720+(#! >5$8$'04! 5$! 6$18.4+(#! 7('27+4,4! .#+H.$)$#0! 2.! 4$+#! 5$4! 4$J)$#04!
FI6$101(6F+,4G! $0! #(#! 52#4! .#! 0$11+0(+1$! '(1(#2+1$C! $0! .#$! +4'F,)+$! 5W(1+J+#$! '(1(#2+1$!
>5$8$'0!5W.#!4$.7!0$#2#0G!4(.4!$#5('215+H.$G!$#!0$11+0(+1$!'(1(#2+1$CD!
!
Le* scanner* coronaire* et* la* coronarographie*(#0! 72! )O)$! 327$.1! 61,5+'0+3$! 6(4+0+3$! $0!
#,J20+3$!H.$!'F$X!7$4!620+$#04!#92I2#0!624!5$!@<B.**!!

Mise à jour de la cardiomyopathie hypertrophique selon les
recommandations ACC/AHA 2011 par François Boustani
*
*
Le*patient*asymptomatique*:*
*
•E9+#0,1O0!5.!012+0$)$#0!*O02*7(H.2#0!$0!5.!M,1262)+7!-!0+01$!61(#(40+'!#9$40!624!*+$#!
5,)(#01,!$0!79+#5+'20+(#!5$!'$!012+0$)$#0!52#4!'$!'24!$40!5$!J125$!::*D!
•"#$! 1,5.'0+(#! 4$6027$! #$! 5(+0! 624! O01$! 1,27+4,$! $0! '$G! H.$7!H.$! 4(+0! 7$! 5$J1,! 5$!
79(*401.'0+(#!>?@@A?B?!:::CD!
•E$! 1$'(.14! 2.K! 324(5+72020$.14! 6.14! $0A(.! -! 5$! 8(10$4! 5(4$4! 5$! 5+.1,0+H.$! $40!
6(0$#0+$77$)$#0!#('+8!>?@@A?B?!:::CD!
•"#$! 2'0+3+0,! 46(10+3$! $#! 2,1(*+$! 5$! 82+*7$! +#0$#4+0,! $40! -! '(#4$+77$1! $0! 6$.0! O01$!
2.0(1+4,$!>?@@A?B?!::2CD!
!
*
La*prise*en*charge*médicamenteuse*:*
*
•E$4! *O02*7(H.2#04! 4(#0! 1$'())2#5,4! $#! 61$)+Y1$! +#0$#0+(#! $0! 4+! 7$4! 4I)60Z)$4!
6$14+40$#0!+7!82.0!0+01$1!72!5(4$!6(.1!'+*7$1!.#$!81,H.$#'$!'215+2H.$![!\VN\]!621!)+#!
>?@@A?B?!:CD!
•%#!'24!5$!'(#01$N+#5+'20+(#4!2.K!*O02*7(H.2#04!(.!$88$04!4$'(#52+1$4!(.!6$14+402#'$4!
5$!4I)60Z)$4!4(.4!'$4!5$1#+$14G!7$!3,1262)+7!$40!1$'())2#5,!$#!5,*.02#0!-!5(4$!
82+*7$!$0!$#!'+*72#0!.#$!5(4$!5$!^_V!)J>?@@A?B?!:CD!
•`1,'2.0+(#!23$'!7$!3,1262)+7!$#!'24!5$!S!
oP125+$#04!,7$3,4D!
oa125I'215+$!4+#.427$D!
o:#4.88+42#'$!'215+2H.$D!
o:7! 6$.0! )O)$! O01$! 52#J$1$.K! $#! '24! 5$!5I46#,$! 4,3Y1$! 2.! 1$6(4! (.! $#! '24!
59FI6(0$#4+(#!>?@@A?B?!:::CD!
•%#! '24! 59+#0(7,12#'$! 2.! 3,1262)+7! (.! $#! '24! 5$! '(#01$N+#5+'20+(#! -! '$! 5$1#+$1G! 7$!
5+70+2X$)!6$.0!O01$!'(#4+5,1,!>?@@A?B?!::*CD!!
•%#!'24!5$!6$14+402#'$!5$!4I)60Z)$4!4(.4!*O02*7(H.2#04!(.!3,1262)+7G!+7!$40!6(44+*7$!
59244('+$1!7$!5+4(6I12)+5$!>::2CD!?00$#0+(#!72!61$4'1+60+(#!5.!5+4(6I12)+5$!4$.7!42#4!
*O02*7(H.2#04!(.!3,1262)+7!$40!6(0$#0+$77$)$#0!52#J$1$.4$!>?@@A?B?!:::CD!!!!!!!
•E$4!),5+'2)$#04!4.+32#04!4(#0!S!
o;,'(#4$+77,4!S!!
E$4!:%@!$0!7$4!?=?Nb!5(+3$#0!O01$!.0+7+4,4!23$'!61,'2.0+(#!3(+1$!#$!624!
O01$! 61(6(4,4! 'F$X! 7$4! 620+$#04! 61,4$#02#0! .#$! (*401.'0+(#! 2.! 1$6(4!
(.!261Y4!+#5.'0+(#!>?@@A?B?!::*CD!!
o@(#01$N+#5+H.,4!S!!
E2!#+8,5+6+#$!(.!2.01$!5+FI51(6I1+5+#$!>?@@A?B?!:::CD!
E$4! 5+J+027+H.$4! 6$.3$#0! O01$! 52#J$1$.K! $0! #9(#0! 624! 5$! 672'$! $#!
792*4$#'$!59.#$!Q?!>?@@A?B?!:::CD!!
E$4! +#(01(6$4! 6(4+0+84!S! 5(62)+#$G! 5(*.02)+#$G! #(125,1#27+#$!
>?@@A?B?!:::CD!!
!

Mise à jour de la cardiomyopathie hypertrophique selon les
recommandations ACC/AHA 2011 par François Boustani
La*prise*en*charge*invasive*des*CMH*symptomatiques*:*
*
La* myomectomie*septale* chirurgicale* 5(+0! O01$! 61(6(4,$! $#! 61$)+Y1$! +#0$#0+(#! 'F$X! 7$4!
620+$#04! 2I2#0! .#$! @<B! 23$'! (*401.'0+(#! 5$! 72! @@MP! 4I)60()20+H.$! 1,812'02+1$! 2.!
012+0$)$#0! 6F21)2'(7(J+H.$! >?@@A?B?! ::2CD! %77$! 6$.0! O01$! *,#,8+H.$! 'F$X! 7$4! 620+$#04!
4I)60()20+H.$4! )27J1,! 7$! 012+0$)$#0! ),5+'27! $0! 2I2#0! .#$! (*401.'0+(#! T! ]V! ))BJ!
>?@@A?B?!::2CD!
!
L’alcoolisation* septale*#$*5(+0! O01$! 61(6(4,$! H.9$#! 5$.K+Y)$! +#0$#0+(#! 621! 1266(10! -! 72!
)I()$'0()+$G!261Y4!5+4'.44+(#!21J.)$#0,$!$0!4+!7$!620+$#0!61,8Y1$!'$00$!4(7.0+(#!>?@@A?B?!
::*CD!
E927'((7+420+(#!#$!5(+0!624!O01$!82+0$!4+!79cJ$!$40![!bd!2#4!$0!$77$!$40!5,'(#4$+77,$!232#0!^V!2#4!
>?@@A?B?!:::CD!
%77$!5(+0!O01$! 5,'(#4$+77,$!'F$X!7$4!620+$#04!2I2#0!.#!4$60.)!T!UV!))!'21!4$4!1,4.70204! #$!
4(#0!624!'(##.4!52#4!'$!'24!>?@@A?B?!::*CD!
!
*
Le*stimulateur*cardiaque*en*cas*de*CMH*symptomatique*réfractaire:*
*
E$!40+).720$.1!'215+2H.$!#9$40!624!+#5+H.,!$#!61$)+Y1$!7+J#$!'F$X!7$4!620+$#04!,7+J+*7$4!-!72!
1,5.'0+(#!4$6027$!H.+!5(+0!O01$!61,8,1,$!>?@@A?B?!:::CD!:7!#$!01(.3$!42!672'$!H.$!49+7!#9I!2!624!
59+#5+'20+(#!'72+1$!-!72!1,5.'0+(#!4$6027$!>?@@A?B?!::*CD!
%#!1$32#'F$!'F$X!7$4!620+$#04!6(10$.14!59.#!40+).720$.1!5(.*7$!'F2)*1$G!+7!$40!12+4(##2*7$!
59$#3+42J$1!.#$!40+).720+(#!5$!7926$K!5.!M;!6(.1!1,5.+1$!7$4!4I)60Z)$4!>?@@A?B?!::2CD!
!
CMH*avec*dysfonction*systolique*(FEVG*<*50%)*:*
*
La*conduite*du*traitement*médical*:*
*
E$! 012+0$)$#0! $40! +5$#0+H.$! -! '$7.+! 5$! 79+#4.88+42#'$! '215+2H.$! 4I40(7+H.$! '()6(102#0G! :%@G!
*O02*7(H.2#04!(.!2.01$!),5+'2)$#04!+#5+H.,4!52#4!'$!'24!>?@@A?B?!:CD!
;$!67.4G!+7!82.0!211O0$1!7$4!+#(01(6$4!#,J20+84!+#5+H.,4!/.4H.$N7-G!0$74!7$!3,1262)+7G!7$!5+70+2X$)!
$0!7$!5+4(6I12)+5$!>?@@A?B?!::*CD!
E$!5,8+*1+7720$.1!6$.0!O01$!'(#4+5,1,!'F$X!7925.70$!$#!4025$!:::!(.!:M!5$!72!efB?!)27J1,!.#!
012+0$)$#0!),5+'27!$0!23$'!.#$!Q%MP![!]Vg!>?@@A?B?!::*CD!
;92.01$4! '2.4$4! H.$! 72! @<B! >0$77$! .#$! )2725+$! '(1(#2+1$C! 6$.3$#0! O01$! -! 79(1+J+#$! 5$! 72!
5I48(#'0+(#!4I40(7+H.$!$0!5(+3$#0!O01$!1$'F$1'F,$4!>?@@A?B?!:CD!
!
Indication*de*la*transplantation*:*
:#4.88+42#'$!'215+2H.$!0$1)+#27$!$0!.#$!@<B!#(#!(*401.'0+3$!#$!6(.32#0!624!*,#,8+'+$1!5$4!
2.01$4!012+0$)$#04D!
E2!012#4672#020+(#!#$!5(+0!624!O01$!1,27+4,$!$#!'24!5$!@<B!6$.!4I)60()20+H.$D!
!
La*pratique*sportive*:*
*

Mise à jour de la cardiomyopathie hypertrophique selon les
recommandations ACC/AHA 2011 par François Boustani
E$!46(10!5$!'()6,0+0+(#! $40! '(#01$N+#5+H.,! )O)$!$#!2*4$#'$!59.#$!(*401.'0+(#!(.!!261Y4!
.#$!1,5.'0+(#!4$6027$!(.!72!6(4$!59.#!;?:!>?@@A?B?!:::CD!%#!'()6,0+0+(#G!4$.74!7$4!46(104!5$!
82+*7$!+#0$#4+0,!>J(78C!4(#0!6(44+*7$!>?@@A?B?!::2C!$0!.#+H.$)$#0!'F$X!7$4!620+$#04!42#4!4+J#$4!
5$!J123+0,D!
!
!
!
!
La*grossesse*:*
*
•"#$!@<B!24I)60()20+H.$!#9$40!624!.#$!'(#01$N+#5+'20+(#!>?@@A?B?!:CD!!!
•%#! '24! 5$! @<B! (''24+(##2#0! 5$4! 4I)60Z)$4! )(5,1,4! -! )(I$#4G! '(#01Z7,4! 621! 7$!
012+0$)$#0G! .#$! J1(44$44$! $40! 6(44+*7$! )2+4! +7! $40! '(#4$+77,! 5$! 1,27+4$1! .#$! 4.13$+772#'$!
1,J.7+Y1$!>?@@A?B?!:CD!
•%#!'24!5$!@<B!23$'!(*401.'0+(#!T!]V!))BJ!!$0A(.!2I2#0!5$4!4I)60Z)$4!#(#!'(#01Z7,4!
621! 7$! 012+0$)$#0! ),5+'27G! 7$! 1+4H.$! 5$! 72! J1(44$44$! $40! )2/(1,! $0! 72! 620+$#0$! 5(+0! O01$!
251$44,$!-!.#!(*40,01+'+$#!61$#2#0!$#!'F21J$!7$4!J1(44$44$4!-!F2.0!1+4H.$!!>?@@A?B?!:CD!!!!!
•"#$!@<B!23$'!5$4!4+J#$4!59+#4.88+42#'$!'215+2H.$!232#',$!$40!.#$!'(#01$N+#5+'20+(#!-!72!
J1(44$44$!>?@@A?B?!:::CD!
!!
Stratification*du*risque*de*mort*subite*et*indication*du*DAI*:!
!
La*stratification*du*risque*de*mort*subite!5(+0!O01$!4I40,)20+H.$!7(14!5$!79,327.20+(#!+#+0+27$*
>?@@A?B?! :C! $0! 1,6,0,$! 0(.4! 7$4! d! -! b! 2#4! 'F$X! 7$4! 620+$#04! -! 1+4H.$! #92I2#0! 624! *,#,8+'+,!
59.#!;?:!>?@@A?B?!::2CD!
!
@$00$!40120+8+'20+(#!'()6(10$!$#!J125$!>?@@A?B?!:C!S!
dD ?#0,',5$#0!6$14(##$7!5$!)(10!4.*+0$G!5$!QM!(.!5$!&M!4(.0$#.$D!
bD ?#0,',5$#0! 82)+7+27! 5$! )(10! 4.*+0$! (.! 59+)672#020+(#! 59.#! ;?:! 6(.1! 21I0F)+$!
3$#01+'.72+1$D!
UD "#$!4I#'(6$!#(#!$K67+H.,$D!
^D "#$!&M!#(#!4(.0$#.$!$0!5('.)$#0,$!h!U!'()67$K$4!3$#01+'.72+1$G!T!dbVA)#D!
]D %62+44$.1!)2K+)27$!5$!72!621(+!5.!MP!T!UV!))D*
*
%#! J125$! >?@@A?B?! ::2C! S! ,327.20+(#! 5$! 72! 1,6(#4$! 0$#4+(##$77$! -! 79$88(10D! "#$! 1,6(#4$! $40!
620F(7(J+H.$!4+!`?i!)2KN`?i!1$6(4![!bV!(.!b]!))BJ!(.!FI6(0$#4+(#!-!79$88(10D!!
!
%#!J125$!>?@@A?B?!::*C!S!-!'(#4+5,1$1!52#4!7$4!'24!7+)+0$4D!*
•=$F2.44$)$#0!0215+8!2.!J25(7+#+.)!-!79:=<D!
•;(.*7$!).020+(#!(.!).020+(#!'()6(4,$D!
•j*401.'0+(#!+)6(102#0$!5$!72!'F2)*1$!5$!'F244$!5.!MPD!
!
Indication*du*DAI*:*
*
Critères*solides*suffisants*pour*porter*l’indication*du*DAI***

Mise à jour de la cardiomyopathie hypertrophique selon les
recommandations ACC/AHA 2011 par François Boustani
*
•@<B!23$'!)(10!4.*+0$G!QM!(.!&M!23$'!1$0$#0+44$)$#0!F,)(5I#2)+H.$!>?@@A?B?!:CD*
•<(10!4.*+0$!61(*2*7$)$#0!7+,$!-!.#$!@<B!'F$X!.#!)$)*1$!5$!72!82)+77$!621$#0!5.!dk!
5$J1,!>?@@A?B?!::2CD*
•%62+44$.1!621(+!T!UV!))!>?@@A?B?!::2CD*
•"#$!(.!67.4+$.14!4I#'(6$4!#(#!$K67+H.,$4!>?@@A?B?!::2CD!*
*
Critères* moins* solides* H.+! #$! 4(#0! 624! 4.88+42#04! 621! $.KN)O)$! $0! #,'$44+0$#0! 592.01$!
82'0$.14!5$!1+4H.$!5$!)(10!4.*+0$*:*
•&M!#(#!4(.0$#.$!S!+#5+'20+(#*$#!J125$!>?@@A?B?!::2C!49+7!$K+40$!592.01$4!82'0$.14!5$!
)(10!4.*+0$G!)2+4!6244$!$#!::*!$#!792*4$#'$!5$!'$4!82'0$.14D*
•=,6(#4$! 0$#4+(##$77$! 2#(1)27$! -! 79$88(10!S! +#5+'20+(#! ! $#! J125$! >?@@A?B?! ::2C! 49+7!
$K+40$! 592.01$4! 82'0$.14! 5$! )(10! 4.*+0$G! )2+4! 6244$! $#! ::*! $#! 792*4$#'$! 5$! '$4!
82'0$.14D*