認識肺動脈高壓

ᙯ‫ٺ‬ү۰ ͹በ ᆒܷᚈᗁर ੼ฯၷϔᓁᗁੰઘੰ‫ܜ‬ ઼֨ᗁጯੰᗁጯ̀ ઼࡚ࡗᎰᐒ঒‫ܛ‬೻ᗁጯ͕̚ซ࣒ ઼֨ᗁጯੰᗁጯրᓜԖି଱ ઼ϲว‫̂ځ‬ጯᗁጯրઘି଱

͹በ เઈߋᗁर ੼ฯၷϔᓁᗁੰ͕᝙̰ࡊ͹‫ڼ‬ᗁर ੼ฯၷϔᓁᗁੰࢦাΐ᜕̰ࡊ͹‫ڼ‬ᗁर ࡻ઼ο֧೻ћ̂ጯᗁጯ౾̀ ઼ϲว‫̂ځ‬ጯᗁጯրᗁጯ̀ ͟ώ઼ϲִౌ̂ጯ‫ܢ‬ᛳঽੰ࣒ຽ ࡻ઼ͱߺ̂ጯซ࣒ ઼ϲว‫̂ځ‬ጯᗁጯրӄநି଱ ઼֨̂ጯ઼֨ᗁጯੰᓜԖઘି଱ ᅃࡻࡊԫ̂ጯᗁጯᄃઉ૵ጯੰӄநି଱ ኎Ϡཌ੼‫ލ‬ᗁᒚડાᅃጱᄃᗁᒚྤ໚ፋЪࢍ൪ ડાგந؎ࣶົᏙྙ؎ࣶ ̚රϔ઼۱જਔ੼ҕᑅᙯ͕‫ົם‬நְ ̚රϔ઼۱જਔ‫ڼ‬ᒚ໤‫݋‬በᏭ؎ࣶ

͹በ ዑᐅ‫پ‬ᗁर ੼ฯၷϔᓁᗁੰ͕᝙̰ࡊ͹‫ڼ‬ᗁर ੼ฯၷϔᓁᗁੰࢦাΐ᜕̰ࡊ͹‫ڼ‬ᗁर ઼ϲว‫̂ځ‬ጯᗁጯրᗁጯ̀ ઼ϲว‫̂ځ‬ጯᗁጯրᓾर ઼֨̂ጯ઼֨ᗁጯੰ ᓜԖӄநି଱ ᅃࡻࡊԫ̂ጯᗁጯᄃઉ૵ጯੰᓾर ̚රϔ઼۱જਔ੼ҕᑅᙯ͕‫ົם‬நְ ̚රϔ઼۱જਔ‫ڼ‬ᒚ໤‫݋‬በᏭ؎ࣶ

iii

ᄮᙊ۱જਔ੼ᑅ

ᇤቇဥฤ ᔁ౶Ϡᗁर

ౘтຍᗁर

੼ฯၷϔᓁᗁੰ‫׊‬ආᗁጯొ͹Ї

੼ฯၷϔᓁᗁੰछलᗁጯొщှቤ‫׶‬ᗁጯ ࡊࡊ͹Ї ੼ฯᗁጯੰᗁጯրᗁጯ̀ έ៉щှቤ‫׶‬ᗁጯጯົႾְ

઼֨ᗁጯੰᗁጯրᗁጯ̀ ઼֨ᗁጯੰ‫׊‬ආᗁጯొ‫ିؠ‬଱ ‫ࡊ׊‬ᗁጯົ‫׊‬ආࢦা؎ࣶົ͹Ї؎ࣶ ੼৺͕᝙ା‫׻‬ఙᓑЪ؎ࣶົ͹Ї؎ࣶ ̚රϔ઼۱જਔ੼ҕᑅᙯ͕‫ົם‬நְ‫ܜ‬ ኎Ϡཌௐ˛ഇᗁᒚშࢍ൪૞छᏙྙ؎ࣶ ̚රϔ઼۱જਔ‫ڼ‬ᒚ໤‫݋‬በᏭ؎ࣶ

ѯ̯Ԡᗁर ੼ฯၷϔᓁᗁੰჟৠొ͹‫ڼ‬ᗁर ઼̚ᗁᘽጯੰᗁጯրᗁጯ̀ έ៉ჟৠᗁጯົ૞ࡊᗁर

ਃॲώᗁर

ᗞҳटᗁर

੼ฯၷϔᓁᗁੰ‫׊‬ආ͕᝙ࡊ͹Ї

੼ฯၷϔᓁᗁੰ࿅ୂҺࠪࢲᒅࡊ͹‫ڼ‬ᗁर ੼ฯᗁጯ̂ጯᗁጯրᗁጯ̀

઼ϲว‫̂ځ‬ጯᗁጯրᗁጯ̀ ઼࡚ᔙ‫ܠ‬૜̂ጯࡁտࣶ ઼ϲว‫̂ځ‬ጯઘି଱ ̚රϔ઼۱જਔ੼ҕᑅᙯ͕‫ົם‬৪३‫ܜ‬ ̚රϔ઼۱જਔ‫ڼ‬ᒚ໤‫݋‬በᏭ؎ࣶ

߸ϒ̚ᗁर

Ղ‫ޙ‬ϲᘽर ੼ฯၷϔᓁᗁੰᘽጯొᓜԖᘽጯࡊࡊ͹Ї ੼ฯᗁጯ̂ጯᘽጯੰ౾̀ ੼ฯௐ˘ࡊԫ̂ጯࡊԫ‫ࡁޠڱ‬տٙซ࣒ έ៉ᗁચგநጯົ੼৺ᗁგर ିֈొ​ొ‫ؠ‬ᓾर

੼ฯၷϔᓁᗁੰ‫ࡊ̰ੰ̶ڌލ‬͹‫ڼ‬ᗁर ઼̚ᗁᘽ̂ጯᗁጯրᗁጯ̀

ౘӻನᘽर

ͳۗፔᗁर

੼ฯၷϔᓁᗁੰᘽጯొᓜԖᘽर ੼ฯᗁጯ̂ጯᓜԖᘽጯٙჇ̀ ੼ฯᗁጯ̂ጯᘽጯրጯ̀

੼ฯၷϔᓁᗁੰ͕᝙̰ࡊ͹‫ڼ‬ᗁर හ‫ޠ‬ᄼ‫ڱ‬འႳᗁጯੰᗁጯրጯ̀

ᅃࡻࡊԫ̂ጯ‫ڼۏ‬րࣘЇӄநି଱

੼ฯၷϔᓁᗁੰჟৠొᓜԖ͕நर ੼ฯᗁጯੰҖࠎࡊጯࡁտٙ ੼ฯξᓜԖ͕நर̳ົ૱ચநְ ჟৠᗁᒚૻ‫ט‬ᝥ‫ؠ‬ᆶߤົ؎ࣶ ੼ฯξгᑭཌԦ߲ဥវ‫ڼ‬ᒚर ିֈొ​ొ‫ؠ‬ᓾर

઼ϲว‫̂ځ‬ጯᗁጯրᓜԖઘି଱

੼ၷᓜԖ͕நरିֈ੊ቚࢍ൪͹޺ˠ

‫ڒ‬ҹษᗁर ੼ฯၷϔᓁᗁੰೇઉొ͹‫ڼ‬ᗁर ઼ϲว‫̂ځ‬ጯᗁጯրᗁጯ̀ ፘˠᗁ૞‫ࡊڼۏ‬/᜕நࡊࣘЇӄநି଱

iv

ૺ൦ᓜԖ͕நर

Ъ੃ဦ३΍‫ ۤۍ‬൴Җ

ᇤቇဥฤ

Ꮒս‫آ‬᜕ந‫ܜ‬

ᅀϜර᜕நर

੼ฯၷϔᓁᗁੰ͕᝙̰ࡊঽ᜕ٗந‫ܜ‬

੼ฯၷϔᓁᗁੰ͕᝙̰ࡊঽ᜕ٗநर

ᅃࡻࡊԫ̂ጯ᜕நր

̮ૈࡊԫ̂ጯ᜕நր

ᔨժ᱕᜕நर

ᛗ࿭೎‫ۏ‬ந‫ڼ‬ᒚर

੼ฯၷϔᓁᗁੰ͕᝙̰ࡊঽ᜕ٗநर

੼ฯၷϔᓁᗁੰ‫ۏ‬ந‫ڼ‬ᒚर

઼ϲέΔ᜕நઉ૵̂ጯ᜕நր

੼ฯᗁጯੰ੼᛬‫ܜ‬ഇ໰᜕ࡁտٙซ࣒

ధฮ↳᜕நर

੼ฯ̂ጯྻજЃยᄃઉ૵გநրጯ̀

੼ฯၷϔᓁᗁੰ͕᝙̰ࡊঽ᜕ٗநर ઼ϲέΔ᜕நઉ૵̂ጯ᜕நր

‫ܜ‬ഇ໰᜕‫ا‬छ‫ۏ‬ந‫ڼ‬ᒚर ‫ا‬छ‫ۏ‬ந‫ڼ‬ᒚ‫ڇ‬ચဥฤ

ᆒ‫۾‬Ӗ᜕நर

Ꮒം͛ۤ̍र

੼ฯၷϔᓁᗁੰ͕᝙̰ࡊΐ᜕ঽ᜕ٗநर

੼ฯၷϔᓁᗁੰۤ̍र

࡚‫ࡊ׶‬ԫ̂ጯ᜕நրጯ̀

઼ϲέΔ̂ጯۤົ̍үጯր

໅⟳ನ᜕நर

ም‫ݡ‬Ꮮ᜕நर

੼ฯၷϔᓁᗁੰ͕᝙̰ࡊঽ᜕ٗநर

੼ฯၷϔᓁᗁੰ᜕நर

‫طܜ‬ጯ७ੑဥ‫ڱ‬ˠ‫ࡊطܜ‬ԫ̂ጯ᜕நր

ᅃࡻࡊԫ̂ጯ᜕நր

Ъ੃ဦ३΍‫ ۤۍ‬൴Җ

v

ᆒԔů੼ฯၷᓁ۱੼ᑅ૞ຽ‫ڼ‬ᒚဥฤᖎ̬ ώੰ͕᝙̰ࡊҋ౹ੰͽֽĂ᎕ໂซҖ۱੼ᑅ়ঽ̝෧ᕝ̈́‫ڼ‬ᒚĄ˩ѐ݈ ӈฟؕӀϡ͕ጱგซҖҕგᕖૺീྏĂӀϡถᗓ̄‫ܡ‬ᕝ጗ඈ็௚‫ڼ‬ᒚ೩ֻঽ ̓‫ڼ‬ᒚĂٕᖼ̬ঽ̓ซҖ۱ொങ‫ڼ‬ᒚĄᐌᘽ‫ࡊۏ‬ԫ۞൴णĂ 93 ѐ੓੼ฯၷᓁ ۱੼ᑅ૞ຽ‫ڼ‬ᒚဥฤ͔ซາ˘΃۞۱੼ᑅᇾ࿯ᘽ‫ۏ‬Ăฟؕ੫၆۱੼ᑅঽ̓೩ ֻ۱੼ᑅᇾ࿯‫ڼ‬ᒚĂࠎέ៉۱੼ᑅᇾ࿯‫ڼ‬ᒚᗁጯ͕̝̚ᅳАဥฤ̝˘Ăࠎฟ णέ៉۱੼ᑅ‫ڼ‬ᒚ۞າ˘ࢱԷႊࢦࢋ֎ҒĄώੰ۱੼ᑅဥฤ௢᎕˩ѐ۞‫ڼ‬ᒚ གྷរĂ၆ঽˠ௟͕‫ר‬᜕۞࡝͕̈́ᙯ͕Ăࠎέ៉۱੼ᑅ‫ڼ‬ᒚགྷរ౵ᖳಱ۞ᅳА ‫ڼ‬ᒚဥฤ̝˘Ą ੼ฯၷᓁ۱੼ᑅ૞ຽ‫ڼ‬ᒚဥฤፋЪЧ̙Т૞ࡊᗁरĂͽΐૻ၆ঽˠ۞‫ڼ‬ ᒚ̈́Б͞Ҝ‫ڇ‬ચĂੵ˞͕᝙̰ࡊ૞ࡊᗁर͕̈́᝙γࡊ૞ࡊᗁरγĂᔘΒ߁਒ ටγࡊ૞ࡊᗁर۞۱᝙ொങ‫ڼ‬ᒚć࿅ୂҺࠪࢲᒅࡊ૞ࡊᗁर੫၆ඕ቙௡ᖐঽ ត۞෧ᕝ̈́‫ڼ‬ᒚćࢦা૞ࡊᗁर੫၆۱੼ᑅ̝ࢦা‫ڼ‬ᒚć਒ට̰ࡊ૞ࡊᗁर ੫၆۱Αਕᑭߤ̈́۱়ঽ͔੓۱੼ᑅ̝෧ᕝ̈́‫ڼ‬ᒚćབࡤࡊ૞ࡊᗁर੫၆‫ܝ‬ ਔ੼ᑅ͔੓۱੼ᑅ̝෧ᕝ̈́‫ڼ‬ᒚć‫ײ‬ӛೈᒖ‫ٸ‬डࡊ૞ࡊᗁर‫ם‬ӄঽˠ̝෧ ᕝć‫׊‬ආ͕᝙ࡊ૞ࡊᗁरซҖ‫׊‬ආ۱੼ᑅ۞෧ᕝ̈́‫ڼ‬ᒚćჟৠࡊ૞ࡊᗁर‫ם‬ ӄঽˠ͕நᆸࢬ̝‫ڼ‬ᒚćछᗁࡊ૞ࡊᗁर‫ם‬ӄщှ໰ᜪćೇઉࡊ૞ࡊᗁर‫ם‬ ӄঽˠ̝ೇઉ‫ڼ‬ᒚĄࠎᜪ̈́ঽ͕̓֗ី۞БˠᗁᒚĂώဥฤፋЪ͕۱ೇઉ रăᘽरă᜕நरĞΒ߁ΐ᜕ঽٗ̈́૞ࡊঽٗğ ăۤ̍̈́ᒉዳरซҖБˠ໰ ᜪĄᖣϤဥវ۞˧ณ೩̿۱੼ᑅঽˠ̝‫ڼ‬ᒚ‫ݡ‬ኳĄώဥฤ˵ፋЪঽˠଂާ ෧ăۤડ‫ڼ‬ᒚăঽٗ‫ڼ‬ᒚă‫ܝ‬෧‫ڼ‬ᒚ̈́΍ੰ‫ڇ‬ચĂ೩ֻ۱੼ᑅঽ̓˘࣎Бࢬ ۞ᗁᒚშĄ

vii

ᄮᙊ۱જਔ੼ᑅ

ώဥฤࠎέ៉͌ᇴણΐ઼ᅫ̂‫ݭ‬۱੼ᑅࡁտ̝ᗁጯ͕̝̚˘Ăΐˢ઼ᅫ ̂‫ࡁݭ‬տᅮགྷ࿅઼γ፟ၹЧ͞ࢬкѨᆶߤĂ௑Ъ͵ࠧ‫ڼ‬ᒚ෧ᕝ̈́‫ڼ‬ᒚͪπĂ ̖଀ͽΐˢࡁտဥฤĂТॡ˵೩ֻ۱੼ᑅঽˠ଀ͽତ‫צ‬౵າ‫ڼ‬ᒚ۞፟ົĄώ ဥฤࠎ̚ᄂ៉̈́‫ݑ‬ᄂ៉ௐ˘࣎ᒔ଀઼ᅫᄮΞࠎ઼ᅫ̂‫ݭ‬۱੼ᑅࡁտ̝ᗁጯ̚ ͕Ąҋ 97 ѐ੓Ăώဥฤણᄃкีௐˬഇ۞઼ᅫ̂‫ݭ‬۱੼ᑅᘽ‫ࡁۏ‬տĂϫ݈ѣ кีࡁտ̪дซҖ̚Ąϫ݈̏Ԇј۞ PATENT ࡁտĂߏ੫၆ࢵ࣎۱જਔ੼ᑅ ‫ڼ‬ᒚ۞າ‫ݭ‬Ξ໘ّ౧ A ᅕᒖ̼㤲 (sGC) ܳซ጗Ăඕ‫̏ڍ‬གྷдາࡻॾᜋᗁጯᗔ ᄫ̚൴ܑĂώဥฤࠎέ៉ќঽּ౵к̝ဥฤĂ‫ם‬ӄঽ̓ΐˢࡁտ֭଀ͽᒔ଀ ౵າ۞۱੼ᑅ‫ڼ‬ᒚĄॲፂ൴ܑдາࡻॾᜋᗁጯᗔᄫ۞ 12 ฉ઼ᅫᓜԖࡁտ (PATENT)ĂВৼˢ 380 ּ۱જਔ੼ᑅଈ۰̚Ă Riociguat ΞԼච 6 ̶ᛗՎҖ෼ ᗓă WHO Αਕ̶৺‫ؼ׶‬ቤᓜԖೋ̼Ąϫ݈ Riociguat ̏‫ ٺ‬2013 ѐ 10 ͡ 8 ͟ᒔ ઼࡚ࢴ‫ݡ‬ᘽ‫ۏ‬გ‫ט‬Ԋ఼࿅ග̟۱੼ᑅঽˠ‫ڼ‬ᒚĂϏֽѣ፟ົдέ៉೩ֻ࠹ᙯ ۱੼ᑅঽˠ‫ڼ‬ᒚֹϡĄ ώဥฤҌೀ 95 ѐ੓ՏѐӮᓝᏱ۱੼ᑅࢦাᗁጯኝ඀Ă೩ֻ౵າ۱੼ᑅ‫ڼ‬ ᒚາۢĄТॡώဥฤ˵੫၆᜕ந໰᜕ᓝᏱ࠹ᙯ᜕ந໰᜕ኝ඀Ąώဥฤ‫ٺ‬ώੰ ̰ࡊ Chief Round ă Grand Round ̈́ѐᅅᗁरିጯĂӮ᎕ໂ۞ିֈາ˘΃ᗁर ۱੼ᑅ෧ᕝ‫ڼ‬ᒚԫμĂឰ۱੼ᑅ‫ڼ‬ᒚΞШ˭͙ॲĄώဥฤᄃۤဥ‫ڱ‬ˠ̚රϔ ઼۱જਔ੼ҕᑅᙯ͕‫ົם‬ЪүซҖঽ̓̈́࠹ᙯ̝኎ି߿જĄ ώੰ۱੼ᑅ૞ຽ‫ڼ‬ᒚဥฤӀϡ௢᎕˩ѐ۞‫ڼ‬ᒚགྷរĂЪ˧በᆷ఺ώ३Ă ‫ۍ‬ඁ˵БᗝॕගĶۤဥ‫ڱ‬ˠ̚රϔ઼۱જਔ੼ҕᑅᙯ͕‫ົם‬ķ Ąበ३ߏࠎ˞ឰ ۱੼ᑅঽ̓ăछˠͽ̈́ᗁ᜕ˠࣶਕՀᄮᙊ۱੼ᑅĂ࠻֍۱੼ᑅঽ̓Ѝ‫۞ځ‬ ϏֽĄ

ᆒܷᚈᗁर

viii

Ъ੃ဦ३΍‫ ۤۍ‬൴Җ

เԔů˩ѐ ˩ѐֽĂౖҡ‫܉‬ᖡă͵ฯă͵սăᚊࡻă݄ҳăፐ‫׶܅‬ధктТᏐˠ۞ ঽ̓‫ޘ‬࿅˘࣎​࣎۞ߋआࡌ΋Ąಶည࢕̚۞ᑗᏐົĂᓁߏഇ࠽඾Տ͡۞ົࢬĂ ࠻඾ঽଐ۞ుՎᘦ‫ؠ‬Ă͕̚˵ी​ी۞ࢳ᜙੓ֽĄՏ༊ѣࢲәਨજĂ͕ଐ˵ᐌ ඾੓Є̙‫ؠ‬Ăᓁߏፉ͕Η؆ତ‫ז‬࿪ྖ۞ঽଐត̼Ąಶညछˠٕр̓ਠ˘੓д facebook ޷៙Ą˘੓ࢬ၆Ϡ‫۞׻‬ᕇᕇႍ​ႍĄ͕̚ᓁߏ఺ᆃ۞ഇ࠽඾Ą˘੓ᛸ ͘Ш඾·႕ԓ୕۞Ϗֽ݈ซĄ ˩ѐֽĂ͕̚·႕˞ຏᔁĄຏᔁᆒઘੰ‫ܜ‬ă‫ڒ‬͹Їă‫ܘ‬͹Їă੺͹Ї۞ ۢ࿃̝ाĄຏᔁ‫ܘ‬ᆵᛝᗁर็଱໰ᜪঽ̓۞͕థĄຏᔁᅀछကᗁरᛸ͘޽ጱ ۱੼ᑅ͕ጱგҕგᑭߤԫμĄຏᔁዑᐅ‫پ‬ᗁर၆ঽˠ۞൑ռ؇ᚥͽౖ̈́ҡঽ ̓‫ޘ‬࿅ԧࡻ઼ซ࣒۞ˬѐĄຏᔁဥฤᗁरă͕۱ೇઉरăᘽरă᜕நरăۤ ̍̈́ᒉዳरᆷ˭၆ঽˠ۞ᙯ͕Ą͍‫ߏ׎‬᜕நर͟ͽᚶ؆۞໰ᜪঽ̓Ą༊൒͌ ̙˞‫ݡ‬Ꮮ၆ঽ̓۞௟͕੠ᖸĄপҾĄপҾຏᔁ͉ळ̄า۞វ෭̈́ᓽᓽ‫ ׶‬Lulu ۞ౖҡĄ ˩ѐ΃ܑ˘֧࣎඀༫Ă˵΃ܑ˘࣎າ۞੓ᕇĂԧࣇഇధౖҡ఺ཏр̓‫ޘ‬ ࿅Հк࣎Ғ૾ᚵ৾۞˩ѐĂ˵ԓ୕૟ԧࣇ˩ѐ۞གྷរᄃଋ˘੓̶ֳĄ˘੓࠻ ֍ϏֽĄ

เઈߋᗁर

ix

ዑԔůԓ୕ дҘ̮ 1995 ѐͽ݈Ă۱જਔ੼ҕᑅߏ˘࣎൑‫ڼڱ‬ᒚ۞়ঽĂѣх߿ॡม ൺ̈́੼Ѫ˸த۞পّĄҭߏҋ 1990 ѐ΃‫ޢ‬ഇĂᐖਔ‫ڦ‬डᘽ‫ڇ˾̈́ۏ‬ᘽ‫ۏ‬ౙᜈ ‫઼ٺ‬γય͵Ă၆۱જਔ੼ҕᑅঽଈߏ˘̂჊ࢰĄᔵ൒έ៉͔ซ۱જਔ੼ᑅ۞ ᘽ‫઼ٺ୎ۏ‬γᇴѐĂҭ੼ฯၷϔᓁᗁੰ‫ٺૄࣇءܜ‬ଯજާᙱࢦা‫ڼ‬ᒚ۞ֹ‫׻‬ ຏĂ‫ٺ‬ϔ઼ 93 ѐಶฟؕ᎕ໂ͚޺۱જਔ੼ᑅঽଈ۞෧‫ڼ‬Ă༊ॡдέ៉ზߏА ᜭĄགྷ࿅˩ѐ۞൴णĂ੼ฯၷϔᓁᗁੰ۞۱જਔ੼ᑅ‫ڼ‬ᒚဥฤѝ̏ѣஎ‫۞ݓ‬ ‫ڼ‬ᒚགྷរĄ ‫ڼ‬ᒚ۱જਔ੼ᑅ۞͞‫̙ڱ‬γͼᘽ‫ڼۏ‬ᒚ̈́͘ఙொങĂҭᘽ‫ڼۏ‬ᒚ̙ည‫׎‬ι ૱֍۞়ঽĂт੼ҕᑅٕᎤԌঽĂѣкᇹّ۞ᏴፄĂٙͽ̪ѣ‫ޝ‬к൴ण۞۩ มĄҭߏ۱જਔ੼ᑅ۞‫ڼ‬ᒚ̙ਕΪѣ‫ڼ‬ᒚ൒۱જਔ੼ᑅ఺়࣎ঽĂ‫ڼ‬ᒚ˯ᔘᅮ ੨Ъ‫ڼ‬ᒚ͔൴۱જਔ੼ᑅ۞јЯĂтҺࠪࢲᒅ়ঽٕАّ͇͕᝙ঽඈĄд఺˘ ͞ࢬĂ੼ฯၷϔᓁᗁੰ۞ဥฤߏј‫ڍ‬೺൒۞ĂЯࠎ఺࣎ဥฤ่̙‫ڼ‬ᒚց֍۞ࣧ ൴ّ۱જਔ੼ᑅঽଈĂТॡ˵‫ڼ‬ᒚЧё়ঽ͔ٙ൴۞۱જਔ੼ᑅĄΐ˯Ă۱જ ਔ੼ᑅ۞ଈ۰่̙ࢬᓜ়ঽ۞‫ރ‬਎ĂѣՀк͕ந̈́ۤགྷ۞ᑅ˧Ăͽ̈́Հк۞ᓜ ௣યᗟĄͽ‫ـ‬ᙯ‫ٺ‬۱જਔ੼ᑅ۞३ᚱĂ̂ౌะ̚дᓾࢗᘽ‫ڼۏ‬ᒚĂ‫׎‬ιొ̶‫݋‬ ᗒтĄ ఺ώ۱જਔ੼ᑅ۞‫ڼ‬ᒚ͘ΊĂ͹ࢋߏ̶ֳ੼ฯၷϔᓁᗁੰ࿅Ν˩ѐֽ۞ ෧‫ڼ‬གྷរĂଂ෧ᕝă‫ڼ‬ᒚăೇઉă͕நᏙથ̈́ᓜ௣໰᜕Ă̙˘֖҃Ąԓ୕ᖣ Ϥ఺ώ३۞΍‫ۍ‬ĂΞ೩̿ঽଈٕ‫׎‬छᛳ၆়ঽᄮۢĂՀԓ୕၆ᗁᒚ૞ຽˠ̀ ѣՀк۞ୁ൴Ą

ዑᐅ‫پ‬ᗁर x

ϫᐂ 1 ౢ ۱જਔ੼ᑅᖎ̬ ································································ 1 œ

ௐ

1-1 ̦ᆃߏ۱જਔ੼ᑅ .................... 1 Ɵ߸ϒ̚ᗁरăเઈߋᗁर 1-2 ۱જਔ੼ᑅ۞া‫ ې‬.................... 3 Ɵᗞҳटᗁरăዑᐅ‫پ‬ᗁर 1-3 ۱જਔ੼ᑅјЯ ....................... 5

Ɵਃॲώᗁरăᔁ౶Ϡ͹Ї 1-4 ۱જਔ੼ᑅПᐍЯ̄ ................ 6 Ɵ߸ϒ̚ᗁरăเઈߋᗁर 1-5 ۱જਔ੼ᑅ‫ڼ‬ᒚ۞࿰‫ ޢ‬............ 7 Ɵ߸ϒ̚ᗁरăเઈߋᗁर

2 ౢ ۱જਔ੼ᑅ෧ᕝƟዑᐅ‫پ‬ᗁर ··········································· 9 œ

ௐ

2-1 ঽΫ ......................................... 9

2-11 ८ᗁ఼ঈ᛿‫ڦ‬ବೡ (V/Q scan) .. 16

2-2 ֗វᑭߤ .................................. 9

2-12 ۱Αਕᑭߤ ............................ 17

2-3 ͕࿪ဦ .................................... 10

2-13 ˘উ̼჆ᕖ೸ീរ (DLCO) ......18

2-4 ҕ୵ᑭߤ ................................ 11 2-5 ҕউ࿸‫ ޘ׶‬............................ 12

2-14 ̶̱ᛗҖ֕ീྏ Ɵᛗ࿭೎ೇઉर ......................... 19

2-6 ਒ొ X Ѝͯ ............................. 13

2-15 ྻજ͕۱Αਕീྏ .................. 22

2-7 གྷ਒݈͕᝙෹ࢰ‫ گ‬.................. 13 2-8 གྷࢴ྽͕᝙෹ࢰ‫ گ‬.................. 13 2-9 ਒ొ࿪ཝᕝᆸ ......................... 14 2-10 ८ჃВॎ ................................ 15

(cardiopulmonary exercise testing)

Ɵᛗ࿭೎ೇઉर 2-16 Ⴢ্ീྏ ................................ 26 2-17 Π͕ጱგ ................................ 27 2-18 ҕგᕖૺྏរ ......................... 28

xi

ᄮᙊ۱જਔ੼ᑅ

3 ౢ ۱જਔ੼ᑅ‫ڼ‬ᒚ ····························································· 29 œ

ௐ

3-1 ᘽ‫ڼۏ‬ᒚƟเઈߋᗁर .......... 29 3-2 ‫ڼ‬ᒚᒚड़੠ᖸƟዑᐅ‫پ‬ᗁर ... 52

3-4 ᘽ‫ֹۏ‬ϡ̝Ϲ̢үϡ .............. 59 ƟౘӻನᘽरăՂ‫ޙ‬ϲ͹Ї

3-3 ซҖ͘ఙ఍ཉƟዑᐅ‫پ‬ᗁर ... 56

4 ౢ ۱જਔ੼ᑅೇઉ‫ڼ‬ᒚƟᛗ࿭೎ೇઉरă‫ڒ‬ҹษᗁर ········· 75 œ

ௐ

4-1 ۱੼ᑅଈ۰۞ྻજᒚ‫ ڱ‬.......... 75

4-3 ۱ొ୻ሒ ................................ 88

4-2 ‫ײ‬ӛ੊ቚ ................................ 86

5 ౢ ۱જਔ੼ᑅ໰᜕ ····························································· 91 œ

ௐ

5-1 ฼ࢴ͞ࢬ ................................ 91 Ɵᔨժ᱕᜕நरăዑᐅ‫پ‬ᗁर 5-2 ॠҖ͞ࢬ .............................. 101 Ɵᔨժ᱕᜕நरăเઈߋᗁर

5-5 ͕֗ࡊ͞ࢬ .......................... 106 ƟᅀϜර᜕நरăૺ൦͕நरă ѯ̯Ԡᗁर

5-3 Ϡֈ͞ࢬƟዑᐅ‫پ‬ᗁर ........ 102

5-6 ‫ا‬छ໰᜕͞ࢬ ....................... 112 Ɵధฮ↳᜕நरăᏂս‫آ‬᜕ந‫ܜ‬

5-4 щှ͞ࢬ .............................. 102 Ɵ໅⟳ನ᜕நरăౘтຍᗁर

5-7 ࠪࡺ͞ࢬ .............................. 115 Ɵᆒ‫۾‬Ӗ᜕நरăเઈߋᗁर

6 ౢ ྻϡۤົྤ໚ƟᏂം͛ۤ̍रăเઈߋᗁर···················· 121 œ

ௐ

6-1 ۤ̍‫ڇ‬ચ .............................. 121 6-2 ᖼ̬̝ۤົྤ໚ ................... 135

xii

Ъ੃ဦ३΍‫ ۤۍ‬൴Җ

6-3 ጡ‫ء‬ॕᙑྤੈ ....................... 136

ϫᐂ

7 ౢ ঽ̶ֳ̓ ········································································ 141 œ

ௐ

Ɵ௑‫܉‬ᖡ̈ؓăౘ͵ฯАϠăౘ݄ҳ̈ؓă߸ፐ‫ؓ̈܅‬

7-1 ࠻֍ϏֽƟ௑‫܉‬ᖡ̈ؓ ........ 141

7-3 ᔙШԓ୕Ɵౘ݄ҳ̈ؓ ........ 144

7-2 ૾ҒϠ߿Ɵౘ͵ฯАϠ ........ 143

7-4 ͕ଐ͟੃Ɵ߸ፐ‫ ؓ̈܅‬........ 146

‫ܢ‬ᐂ˘ Warfarin ů‫ڇ‬ϡԩ጖ҕ጗ ‫ڦ‬ຍְี .............................. 149

‫ܢ‬ᐂα ӛˢ Iloprost ᆡᙳጡֹϡ ߹඀ ...................................... 176 ‫ܢ‬ᐂ̣ ˘ਠ߿જᛷ‫פ‬ณܑ .............. 190

‫ܢ‬ᐂ˟ ‫ڼ‬ᒚ۱જਔ੼ᑅᘽ‫ݡ‬໰ͯ .............................................. 150 ‫ܢ‬ᐂˬ Treprostinil ᘽ‫ۏ‬ϩ˭‫ڦ‬ड ߹඀ ...................................... 162

‫ܢ‬ᐂ̱ ྻજႾീࡔᐂܑ .................. 191 ‫ܢ‬ᐂ˛ ࢦ๋̂ঽ͕̈́֗ᅪᘣ̝Чᙷ Ϧኛܑॾ .............................. 192

ણ҂͛ᚥ ························································································· 203

Ъ੃ဦ३΍‫ ۤۍ‬൴Җ

xiii

۱જਔ੼ᑅᖎ̬

1-1 ̦ᆃߏ۱જਔ੼ᑅ ۱੼ᑅ۞‫ؠ‬ཌྷߏЃिॡĂགྷϤΠ͕ጱგീ଀۞πӮ۱જਔᑅ˧̂ ‫ٕٺ‬ඈ‫ ٺ‬25 ୮ѼՑߗĄϫ݈ᄮࠎЃिॡϒ૱۞πӮ۱જਔᑅ˧ࡗࠎ 14 Ų 3 ୮ѼՑߗĂ౵੼̙଀෹࿅ 20 ୮ѼՑߗĄπӮ۱જਔᑅ˧རд 21 Ҍ 24 ୮ѼՑߗ̝ม۞ຍཌྷ‫إ‬Ϗ‫ځ‬६Ă఺ᇹ۞ঽˠᅮࢋՀк۞ࡁտĄ

πӮ۱જਔᑅ˧

ϒ૱۱ᑅ

ள૱۱ᑅ

۱੼ᑅ

14 Ų 3 ୮ѼՑߗ

21-24 ୮ѼՑߗ

Ÿ 25 ୮ѼՑߗ

ϫֶ݈ፂ۱ొ຋ҕგແᑅă۱ҕგ‫˧ܡ‬ăͽ͕̈́Ꮾ΍ณ۞̙Т௡ ЪΞҿ‫̙ؠ‬Т۞۱੼ᑅјЯĄ۱જਔ੼ᑅߏ۱੼ᑅ۞‫˘̚׎‬჌Ăд࿅ Ν۞ᇴѐ̰၆‫ٺ‬ѩ়ঽ۞ᄮᙊᄃ‫ڼ‬ᒚѣՀк۞ࡎ৔Ą۱જਔ੼ᑅΒӣ ధкᓜԖ়ঽĂ΁ࣇВֳ࠹Т۞ᓜԖᄃҕҖજ˧ጯপᇈĂͽ̈́۱ొ຋ ௟ҕგ۞ঽநត̼Ąጐგ௟ࡪ̝มᄃ௡ᖐ̝ม۞ঽϠந፟ᖼ̏གྷజቁ ᄮĂтңᄵ൴ᄃซण۱જਔ੼ᑅ۞‫ځ‬ቁϹ̢үϡֶ൒Ϗ‫ځ‬Ą

1

۱જਔ੼ᑅ෧ᕝ

۱੼ᑅ۞јЯ࠹༊кĂ߇ืࢋщଵкีᑭߤͽቁ‫׎ؠ‬јЯâਠ ҃֏Ă̂ரΞͽડ̶ࠎ֗វᑭߤăҕ୵ᑭߤăΑਕّᑭߤăᇆညᑭ ߤĂ‫͕׶‬ጱგᑭߤඈĄ

2-1 ঽΫ тТ‫׎‬ι়ঽĂͿ௟ྙયঽΫߏ‫ܧ‬૱ࢦࢋ۞Ą͍‫ߏ׎‬۱੼ᑅ۞‫ܐ‬ ഇা‫ޝې‬ᅅ຋ٕ൑૞˘ّĂҭ̂ొ̶ົᄃྻજ࠹ᙯĄᗕཙͪཚ఼૱ຍ Ꮬ۱੼ᑅЪ‫׀‬Π͕਽აĄΩγĂ‫˳ܜ‬۱੼ᑅົ͔൴Π͕‫ވ‬۲̂Ăͽ࡭ ݄‫ې‬જਔֻٙᑕ۞ҕ୵̙ᇳΠ͕‫ֹވ‬ϡĂЯ҃ጱ࡭͕ඔ൭ĄঽଈࠤҌ ົѣ‫ڃ‬౿ٕ‫ܕ‬Ҭ‫ڃ‬౿۞া‫ې‬Ăѣֱ‫ົ݋‬ѣბӱ‫ײ‬ӛඈা‫ې‬Ą

2-2 ֗វᑭߤ ֗វᑭߤืΒ߁ᐚᐖਔߏӎ‫ૺޤ‬ăߏӎѣ̙ϒ૱۞͕ࢰă˭۳ߏ ӎѣͪཚăքතߏӎཚ̂ăߏӎѣཛͪăߏӎѣเ৅ăߏӎѣ‫ېڙ‬ ޽ă̈́‫׎‬ι௡ᖐ়ঽপѣ۞ϩቲត̼ඈĄ

9

۱જਔ੼ᑅ‫ڼ‬ᒚ

3-1 ᘽ‫ڼۏ‬ᒚ NJ݈֏ ۱જਔ੼ҕᑅ۞‫ڼ‬ᒚ࠹༊ኑᗔĂͷ‫ــ‬ѣઘүϡٕПᐍّćЯѩ ᅮࢋᄃ૞ຽ۞ᗁर·̶੨ЪĂֶᏥᗁर۞གྷរֽᏴፄ౵ዋЪ۞‫ڼ‬ᒚᘽ ‫ۏ‬Ąϫ݈‫ڼ‬ᒚ۞ࣧ‫ߏ݋‬ԓ୕ቤྋঽ̓۞̙ዋ̈́‫ؼ‬ቤ়ঽ۞ซ඀Ă֭ͷ ೩੼ঽ̓۞Ϡ߿‫ݡ‬ኳ‫׶‬х߿தĄ‫ڼ‬ᒚ͞ࢬΞ̶ࠎ็௚ᅃӄᒚ‫̈́ڱ‬າ˘ ΃۱જਔ੼ҕᑅ̝ᇾ࿯‫ڼ‬ᒚᘽ‫ۏ‬Ą д 1980 ѐ΃۱જਔ੼ᑅ۞࿰‫̙֭ޢ‬рĂЯࠎ‫ڼ‬ᒚᏴፄΪѣ็௚ᒚ ‫ڱ‬ćۡ‫ ז‬1990 ѐ΃൴ܑ˞ Epoprostenol ۞ᐌ፟ଠ‫ྏט‬រĂฟୁ˞‫ڼ‬ᒚ ۞າࡔ̮Ą็௚ᒚ‫ڱ‬Яࠎ৿͌၁ᙋᙋፂĂЯѩ็௚ᒚ‫ڱ‬តјᅃӄ۞֎ ҒĄϫ݈‫ڼ‬ᒚ͹คࠎາ˘΃۱જਔ੼ҕᑅ̝ᇾ࿯‫ڼ‬ᒚᘽ‫ۏ‬Ăт‫ڍ‬ᘽ‫ۏ‬ ൑‫ڱ‬ԼචঽଐĂ‫ޙ݋‬ᛉ͘ఙ̈́۱᝙ொങĄ

29

۱જਔ੼ᑅೇઉ‫ڼ‬ᒚ

4-1 ۱੼ᑅଈ۰۞ྻજᒚ‫ڱ‬ ۱੼ᑅঽଈੵ˞ᅮࢋ૞ຽᗁᒚဥฤ໰᜕ĂՀᅮࢋঽˠᄃछᛳϒቁ ͷ᎕ໂ۞ણᄃೇઉྻજࢍ൪Ą҃ॲፂা‫ֽې‬Ᏼፄዋ༊۞ྻજ͞ёߏ‫ޝ‬ ࢦࢋ۞Ą

NJྻજड़‫ڍ‬ (1) ྻજਕ˧ԼචĈֹϠ߿˯ត଀ᅅᗫĄ (2) ҕგ̰ϩ௟ࡪΑਕԼචĈֹҕგᕖૺĂԼචα۳Дҽଐ‫ڶ‬Ą (3) ҋ‫ޠ‬ৠགྷΑਕԼචĈഴ͕͌‫̙ޠ‬ፋĂഴ͌ҕᑅ۞តજĄ (4) ͕ଐត଀ྵᅅᗫă೚ԣĄ

➠Ϡ߿‫ݡ‬ኳ۞ԼචĂ֭ܲ޺ઉ૵ͷఢ‫۞ޠ‬Ϡ߿‫ݭ‬ၗĂਕഴ়͌ঽ۞ೇ ൴ᄃೋ̼Ą

75

۱જਔ੼ᑅ໰᜕

5-1 ฼ࢴ͞ࢬ ੫၆۱੼ᑅঽˠֽᄲĂ฼ࢴΞͽд‫ڼ‬ᒚ۱੼ᑅ࿅඀͚̚ᇣঽˠ֗ វĂॲፂ௚ࢍĂ၆‫ٺ‬ઉ૵۞ᇆᜩ˧ĂᒉዳΚ 70% ĂϠ߿͞ёΚ 20% Ă ‫΁׎‬Я৵ࡗ 10% Ăග̟։р۞ᒉዳ͚޺Ăѣӄ‫ٺ‬ჯ޺͕᝙۞ϒ૱Α ਕăჯ޺நຐ۞វࢦ̈́ഴ͌ͪཚඈৈ఍Ą

NJᒉዳ৵ ᒉዳߏઉ૵۞ॲώĂ҃ࢴ‫ߏۏ‬ᒉዳֽ໚Ăԧࣇᅮࢋଂࢴ‫̚ۏ‬ᒔ଀ ᒉዳ৵ֽჯ޺Ϡ߿፟ਕĂᒉዳ৵Βӣ˞ᔝᙷă਌۹ăకϨኳăჯϠ৵ ‫׶‬ᚩ‫ۏ‬ኳĂ‫̚׎‬కϨኳĞ4 ̂ΙƟ̳ҹğ ăᔝᙷĞ4 ̂ΙƟ̳ҹğ ă਌۹ Ğ9 ̂ΙƟ̳ҹğ Ă఺ˬ۰˫Ⴭࠎˬ̂ᒉዳ৵Ą ᔝᙷ̈́਌۹ࠎயϠ͟૱߿જ۞ਕณֽ໚ćకϨኳΞͽ‫ޙ‬ၹăჯ޺̈́ ࣒ྃਈຫ۞௡ᖐćჯϠ৵‫׶‬ᚩ‫ۏ‬ኳߏአ༼΃ᔁᄃϠந፟ਕ۞ࢦࢋ֎ҒĄ

91

ྻϡۤົྤ໚

6-1 ۤ̍‫ڇ‬ચ NJ˘ਠᏙྙ Ğ˘ğ၆ੰγЧีఢ‫ؠ‬үຽ඀Ԕ̙ۢ྽ٕѣႷય 1. ࢦ๋̂ঽΙϦኛ͞ёĞώੰᗁरቁ෧‫ޢ‬ĂᗁੰӈΞ‫ם‬ӄϦኛğ (1) ࢦ๋̂ঽቑಛܼϤҖ߆ੰ኎Ϡ჊Ӏొ̳ӘĂϫ݈Β߁ 30 ̂ᙷĄ (2) ܲᐍ၆෪གྷপࡗᗁᒚੰٙᗁर෧ᕝቁ‫ؠ‬ٙᎮଈ۞๋ঽߏᛳ‫ٺ‬ ̳Ә̝ࢦ๋̂ঽॡĂΞᑭ‫˭׍‬Е͛Іฎ૙ٕᏐҋਖ਼ІĂШઉ ̶ܲડຽચ௡Ϧኛࢦ๋̂ঽᙋ‫ځ‬Ą (a) Бϔઉ૵ܲᐍࢦ๋̂ঽᙋ‫ځ‬Ϧኛ३Ğ֍‫ܢ‬ᐂ˛Ă p.192ğ Ą (b) পࡗᗁੰă෧ٙฟϲˬ˩̰̝͟෧ᕝᙋ‫ځ‬३ĄĞ෧ᕝঽЩ ᛼Ăᑕΐ๱઼ᅫ়ঽ̶ᙷቅğ Ą (c) ̶֗ᙋ‫͛ځ‬ІĞϒͅࢬᇆώĂ‫׊‬ආ଀ͽ͗˾Щᘨ΃ആğ Ą னಞᓜᕢϦኛ۰ĂኛႽณᛸ૲ઉܲΙ (d) ঽ።ၡࢋٕᑭߤಡӘඈ࠹ᙯྤफ़Ą

121

ঽ̶ֳ̓

7-1 ࠻֍Ϗֽů௑‫܉‬ᖡ ϔ઼ 90 ѐˬ͡Ăԧௐ˘Ѩຏᛇ֗វѣ˞ត̼Ăߋֽ͇ᓜ͕ଐ೚ ॆĂ̾Ѓॡมϒࢋа๽๽छϡᐠĂ֕˞ 20 ೀѐ۞੺ྮިᆃ̫͇ߏтѩ ۞ຏᛇ̙න‫ڇ‬Ă࿅੺ྮॡᛇ଀ᗕཙ൲హ൑˧ҡᐌ඾‫ײ‬ӛާܳĂ၆ࢬሢ ଉ۞ූ྽‫ߏݒ‬ᓝՎჯᓞᙱ̏෸࿅Ă఺ᇹ۞‫΄ڶې‬ԧ‫ܧ‬૱̙ྋĂಶЯт ѩ۞া‫ې‬ᐌ‫͈זޢ‬छ‫͕ܕܢ‬᝙̰ࡊಶ෧Ă໰࿅͕෹‫ޢ‬ĂᗁϠ੺˯ฟಏ ࢋԧ‫੼ז‬ฯၷᓁԱ‫͌ڒ‬൞ᗁरĂֹԧ͕̚෹৺᪜᪛̙щĄଭ˞ཱིĂԧ ‫੼ז‬ฯၷᓁ֍‫͌ڒז‬൞͹Їଵ˞ᑭߤĂంЩຏᛇ΄ԧ̙න‫ڇ‬Ă‫ޝ‬ԣ۞ ‫ڒ‬͹Ї੺˯ࢋԧԱՂ྽ᔕᗁरĂдঽଐᅮࢋ˭Ăԧҝซ˞੼ฯၷᓁᗁ ੰĂ఺˘ాҚ۞ᑭߤ‫ޝ‬ԣ۞జ෧΍ĺࣧ൴ّ۱જਔ੼ᑅĻ఺ߏԧѣ֗ ͽֽௐ˘Ѩତᛈ఺ࢪϠঽЩĄ ༊ॡۢ྽֭൑ᘽᗁĂ͕ଐ۞‫ڷ‬ಉᄃऻᛵ̶ࡋᜇ඾ԧĂࢬ၆АϠᄃ ‫ࢬ׶̄ޅ‬၆छलᄃ̍үĂ఺ంЩ۞൑˧ຏឰԧрປ‫۝‬рҋయĄ̄͟ᔘ ߏࢋ࿅ĂӘ෦ҋ̎ᓁ̙ਕঐՕ˭ΝĂ‫̄ޅ‬ᔘ̈ᅮࢋԧ۞ຑĂੵ˞િૻ ᔘߏિૻĄ

141