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ORIGINAL ARTICLE

DIAGNOSIS DAN TATALAKSANA KARDIOMIOPATI HIPERTROFIK


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ABSTRACT NHMDGLDQ NHODLQDQ MDQWXQJ LQL PHQFDSDL


Hypertropic cardiomyopathy is a genetic heart  SRSXODVL GDQ PHUXSDNDQ SHQ\HEDE
disease commonly occured in adult. Diagnosis NHPDWLDQ PHQGDGDN SDOLQJ VHULQJ SDGD
and management of this condition become SDVLHQ XVLD PXGD \DQJ VHEHOXPQ\D WLGDN
dilema for clinicians. The targets of management PHPLOLNLNHOXKDQDSDSXQ
are to treat the signs and symptoms, prevent 8QWXN GDSDW PHQGLDJQRVLV SHQ\DNLW
sudden death, and also give the education to LQLWLGDNODKWHUODOXVXOLW7HPXDQSHQHEDODQ
the patient. GLQGLQJ YHQWULNHO NLUL GHQJDQ PRGDOLWDV
SHQFLWUDDQ DSDSXQ GDSDW GLDQGDONDQ
Keywords: diagnosis, management, 1DPXQ NDUHQD EHUVLIDW DVLPWRPDWLN
hypertrophic cardiomyopathy VHULQJNDOL WHUMDGL NHWHUODPEDWDQ
GLDJQRVLV SDGD SDVLHQ \DQJ PHQJDODPL
PENDAHULUAN NDUGLRPLRSDWL KLSHUWUR¿N WHUXWDPD SDGD
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VHPDNLQ FDQJJLK PHQ\HEDENDQ NODVL¿NDVL KDOWHUVHEXWVHEDJDLVXDWXVDULSXVWDND
GDULNDUGLRPLRSDWLNHUDSEHUXEDK1+DOODLQ
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WHUVHEXW DGDODK DGDQ\D SHQLQJNDWDQ %HUGDVDUNDQ (6& European Society
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.HODLQDQ JHQHWLN \DQJ EHUVLIDW DXWRVRPDO
GRPLQDQ GLDQJJDS PHPLOLNL SHUDQ SDOLQJ How to cite this article :
SHQWLQJ SDGD SDWR¿VLRORJL WHUMDGLQ\D DIAGNOSIS DAN TATALAKSANA
NHODLQDQLQL3DGDSRSXODVLXPXPDQJND KARDIOMIOPATI HIPERTROFIK

Indonesia Journal Chest Vol. 6, No. 1 Januari - Juni 2019 1


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2 Indonesia Journal Chest Vol. 6, No. 1 Januari - Juni 2019


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Indonesia Journal Chest Vol. 6, No. 1 Januari - Juni 2019 3


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4 Indonesia Journal Chest Vol. 6, No. 1 Januari - Juni 2019


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PHQ\HEDENDQ WHUMDGLQ\D KLSHUWUR¿ VHSHUWL SDGDDWOHW\DQJEHUODWLKNHUDVVHUWDGLVLSOLQ
DRUWD VWHQRVLV PDXSXQ KLSHUWHQVL \DQJ WHODK PDNVLPDO GDQ SHQDPEDKDQ NHWHEDODQ
EHUODQJVXQJ ODPD Isolated basal septal YHQWULNHONLUL\DLWXKLQJJDPPSDGD
hypertrophy GDSDW GLWHPXL SDGD SDVLHQ ZDQLWDGDQPPSDGDSULD
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GLGDSDWNDQ DGDQ\D ULZD\DW SHQLQJNDWDQ NHWHEDODQ YHQWULNHO NLUL SDGD DWOHW DNDQ
WHNDQDQ GDUDK VHODPD  WDKXQ SHUWDPD EHUNXUDQJ OHELK GDUL  PP VHPHQWDUD
VHUWD GLGDSDWNDQ DGDQ\D EXNWL NHUXVDNDQ SDGD SDVLHQ GHQJDQ NDUGLRPLRSDWL

Indonesia Journal Chest Vol. 6, No. 1 Januari - Juni 2019 5


KLSHUWUR¿N WLGDN DNDQ WHUOLKDW \DQJ GDSDW PHPSURYRNDVL WHUMDGLQ\D HGHPD
SHQJXUDQJDQ 6HODLQ LWX KDQ\D SDGD SXOPRQHU
NDUGLRPLRSDWL KLSHUWUR¿N WHUGDSDW $SDELOD SDVLHQ LQWROHUDQ WHUKDGDS
JDPEDUDQSHQ\HQJDWDQROHKJDGROLQLXP SHQ\HNDWEHWDGDQMXJDYHUDSDPLOSHPEHULDQ
e. Exercise training - 3DGD DWOHW 92 GLOWLD]HP GHQJDQ GRVLV DZDO  [  PJ
SXQFDN !PNJPHQLW DWDX 92 GDSDW GLSHUWLPEDQJNDQ 1LIHGLSLQ GDQ
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7DWDODNVDQD.DUGLRPLRSDWL+LSHUWUR¿N GLEHULNDQ XQWXN PHPSHUEDLNL NHOXKDQ
GLVSQHX \DQJ EHUNDLWDQ GHQJDQ /9272
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6 Indonesia Journal Chest Vol. 6, No. 1 Januari - Juni 2019


\DQJ PHQHPSHO SDGD VHSWDO KLQJJD NH GDQ NHPDWLDQ PHQGDGDN 6HNLWDU 
EDJLDQEDZDKGDQNDWXSDRUWD7LQGDNDQLQL GDUL SDVLHQ GHQJDQ NHDGDDQ REVWUXNVL
EHUWXMXDQXQXWNPHQXUXQNDQWHNDQDQSDGD PLGNDYLWDV PHPLOLNL DQHXULVPD DSLNDO
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PDND PLHNWRPL VHSWDO OHELK GLSLOLK WLGDN PHPHUOXNDQ SHQDWDODNVDQDDQ OHELK
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WHUSURYRNDVL 1DPXQ GHPLNLDQ PHWRGH LQL VWLPXODVL LQRWURSLN SRVLWLI 3HPEHULDQ ace
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ablation DWDXSXQ DGDQ\D ULVLNR NHMDGLDQ MDQWXQJGHQJDQ()XQWXNPHQJXUDQJL
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WLQJJL WHUKDGDS WHUMDGLQ\D JDJDO MDQWXQJ SDVLHQGHQJDQJDJDOMDQWXQJfunctional class

Indonesia Journal Chest Vol. 6, No. 1 Januari - Juni 2019 7


,,,,9 \DQJ GLDNLEDWNDQ ROHK GLVIXQJVL SHQLQJNDWDQDQJNDNHPDWLDQPHQGDGDNGDQ
GLDVWROLN GHQJDQ () ! 3DGD SDVLHQ UDVDWLGDNQ\DPDQSDGDSHPDVDQJDQ
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SHPDVDQJDQ,&'NHUDSGLKXEXQJNDQGHQJDQ GLODNXNDQVHWLDSWDKXQVHNDOLSDGDSDVLHQ

8 Indonesia Journal Chest Vol. 6, No. 1 Januari - Juni 2019


\DQJ VHFDUD NOLQLV VWDELO DWDX VHWLDS  PHQJHWDKXL NHEHUDGDDQ NDUGLRPLRSDWL
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10 Indonesia Journal Chest Vol. 6, No. 1 Januari - Juni 2019

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