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【略 語】AASM:American Academy of Sleep MedicineA-aDO2:alveolar-arterial O2 tension difference/gradientAHA:American Heart AssociationACC:American College of CardiologyACCP:American College of Chest PhysicianALK1:activin receptor-like kinase-1ACVRL1:activin A receptor type II-like 1AHI:apnea hypopnea indexARB:angiotensin receptor blockerASD:atrial septal defectBMPR2:bone morphogenetic protein receptor type IIBNP:brain natriuretic peptideBPA:balloon pulmonary angioplastyCAV1:caveolin-1CCB:calcium channel blockercGMP:cyclic guanosine monophosphateCHD-PAH:congenital hear disease-pulmonary arterial
されるようになった.現時点で最新の肺高血圧症に関するガイドラインとしては,2008年に米国・ダナポイントで開催された第4回肺高血圧症ワールド・シンポジウム(ダナポイント会議)での議事録を集約した米国心臓病学会誌のガイドライン1),2)や欧州の心臓病学会(European Society of Cardiology: ESC)/呼吸器病学会(European Respiratory Society: ERS)作成の肺高血圧症診断・治療ガイドラインが存在する3),4).そこで本肺高血圧症治療ガイドライン2012年度版は,基本的にはこれら最新の欧米ガイドラインに準拠しつつ,我が国に固有の事情も加味して作成する方針とした.なお,2013年2月には第5回肺高血圧症ワールド・シンポジウム(ニース会議)が開催されたので,その発表内容の一部や,また現時点ではエビデンスとしては確立していないが,専門家間では有効/またはその可能性が高いと認識されている治療薬や方法についても,注釈を加えた上で記載した.このため本ガイドラインの内容はすべてがランダム化比較試験やこれに準じる客観的なエビデンスを根拠として記載されている訳ではなく,「治療の参考」とするべき程度の内容も多く含まれている.したがってその限界については理解し,本「ガイドライン」を利用していただきたい. 治療法の文献エビデンスレベルや推奨グレードについては,近年では「Minds診療ガイドライン作成の手引き2007」に準拠して作成される傾向にある.ただ,本ガイドラインでは従来の日本循環器病学会作成のガイドラインと統一性を保つ形式とし,AHA(American Heart Association)/ACC(American College of Cardiology)ガイドライン,ESC/ERSのガイドラインに準拠した表1
2008年のダナポイント会議では,安静時に右心カテーテル検査を用いて実測した肺動脈平均圧(mean PAP)が25mmHg以上の場合が肺高血圧と定義された3),5).以前の肺高圧の定義では運動時の mean PAP が30mmHg以上も肺高血圧に含まれていたが,ダナポイント会議の定義では本基準は採用されていない.さらに肺高血圧症例中で特に肺動脈楔入圧(pulmonary capillary wedged pressure: PCWP)が15mmHg以下の場合を肺動脈性肺高血圧症(pulmonary arterial hypertension: PAH)と定義した.安静時,健常人の mean PAP は14±3mmHg,正常上限は20mmHgと報告されており6), mean PAP が20~25mmHgの例の臨床的意義は今後の検討課題とされている.近年心エコー法の発達により,右室と左室の形態や心ドプラ法より,肺動脈圧や心拍出量の推定が可能となった.本法は容易,かつ非侵襲的に肺高血圧の存在を推定することが可能なため,本症に関して極めて有用な診断手段といえる.しかし少なくとも初診時や治療法の変更時には,正確な病態評価を行うため,右心カテーテル検査を用いた肺血行動態の直接測定が必要であるとの考え方がダナポイント会議では提唱され,現在の世界標準となっている.
③ 心拍出量(cardiac output: CO)または肺血流量(Qp: pulmonary blood flow)
基本的にはCOとQpは等しいが,シャント疾患ではCOとQpは異なる.したがって小児科領域で肺循環系の血行動態諸量を扱う場合にはQpが用いられる場合が多い.肺高血圧では有意な三尖弁逆流を伴うことが多く,熱希釈法により得られた CO は誤差が大きいことが知られている.三尖弁逆流に影響されないFick法による測定が勧められる.
右心カテーテル検査中に,短時間作用性の肺血管拡張薬を投与し,肺血行動態の変化の有無を観察し治療方針を決定する目的で行われる.急性肺血管反応性試験に使用する薬剤としてエポプロステノール持続静注,アデノシン静注,一酸化窒素(NO)吸入が欧米のガイドラインでは取り上げられている3).急性肺血管反応性試験が陽性の定義は mean PAP が10mmHg以上減少し40mmHg以下になることで,その際にCOが低下しないことが前提である37).ESC/ERSガイドラインでは,急性肺血管反応性試験の適応は IPAHかHPAH,または食欲抑制剤に伴うPAHに限られると記載されていることに注意していただきたい3).日本では急性肺血管反応性試験の有用性に関して専門家の間で意見の相違があり,本試験を実施しない場合も多い.この理由は,陽性判定のケースが極めて少ないこと,陽性例では欧米のガイドラインではカルシウムチャンネル拮抗薬が治療の選択となるが,このような症例ではそれ以外のPAH治療薬に対する反
病歴,主訴,身体所見,胸部単純X線写真,心電図などから肺高血圧の存在が疑われれば,現在最も簡便な肺高血圧の診断法である心エコー・ドプラ法を用いて肺高血圧の有無を検査する.肺高血圧症の存在が強く疑われた場合には,再改訂版肺高血圧臨床分類2~5群の他の肺高血圧症の鑑別診断を行いつつ,右心カテーテル法を用いて肺血行動態諸量の直接計測を行い確定診断とする.定義上,安静時の mean PAP ≧ 25mmHg が肺高血圧の確定に必須であり,加えてPAHの診断はPCWP ≦ 15mmHgを満たすことが必要となる.
血行動態を示しつつ,PVOD/PCHでは胸水やKerley B line,小葉間隔壁の肥厚やスリガラス様陰影などの所見が存在することが目安となる.またPVOD/PCHではPAH治療で用いられる肺血管拡張療薬により肺水腫が生じ,病態が悪化する可能性が報告されており,本所見も鑑別診断に有用である. 再改訂版肺高血圧症臨床分類第2群の肺高血圧症は,さらに収縮不全,拡張不全,弁膜症,左心流入路/流出路閉塞の4種の亜分類を設定し,心エコー・ドプラ法や右心カテーテルを組み合わせて詳細な病態決定を行う.
5 第4群:CTEPH CTEPHでは基礎疾患として①深部静脈血栓症②凝固能異常(抗リン脂質抗体陽性,Protein C や Protein Sなどの欠損など)が存在し,肺に血栓塞栓症を反復することで肺動脈が慢性閉塞し,閉塞した血管数が多くなるにつれ肺高血圧を呈するようになる. CTEPHの肺動脈病変は主肺動脈から連続して内膜肥厚が起こっている場合と,主に区域枝から内膜肥厚が始まっている症例がある.また,さらに末梢の肺動脈にのみ血栓形成が認められ,臨床的に IPAHとの鑑別が困難となる症例もある.末梢枝のみの閉塞の場合は手術による内膜剥離が困難となる.近年,我が国ではカテーテルを用いた肺動脈拡張術が行われるようになり,比較的末梢の血栓性閉塞も開大できるようになった.閉塞した部位の肺動脈には器質化した血栓塞栓や血栓内の再疎通血管像が存在する場合がある.非閉塞部での肺動脈でも肺高血圧の二次的変化としてびまん性内膜肥厚がみられる.拡張した肺動脈主幹部は瘤状となり,しばしば壁在血栓形成を来たす.閉塞部領域の陳旧性肺梗塞を認める.これは IPAHにはあまりみられない所見である53),54).
デンスを集約したPAH治療ガイドラインの作成が試みられ,2009年にはこれを成文化したPAH治療ガイドライン が Journal of the American College of Cardiology誌(JACC)1)に発表された.またこの内容をより具体化したESC/ERS肺高血圧症治療ガイドライン3)がEuropean Heart Journal誌に掲載され,ほぼ同内容のERSガイドライン4)もEuropean Respiratory Journal 誌に掲載されている.しかし我が国では肺高血圧症治療薬の大規模な臨床試験はなく,日本人に関するエビデンスにより独自のPAH治療ガイドラインを作成することは困難である.このため本日本循環器学会ガイドラインは JACCおよびESCやERSのPAHガイドライン,および2013年2月のニース会議の発表内容を基本とし,我が国では発売されていない治療薬は除いて作成した.1)IPAH/HPAHの治療指針概略(図5) IPAH/HPAHの治療指針は,まず IPAH/HPAHの確定診断と重症度評価を行いつつ,以下の一般的対応/支持療法を開始する(表9,表10).PAHでは基本的にはワルファリンを用いて抗凝固療法を行う.低酸素血症があれば酸素投与の適応と考えられ,我が国では肺高血圧症の診断で在宅酸素療法の健康保険の適応が得られている.右心不全症状があれば利尿薬の投与や,心房性頻拍症があればジゴキシンを用いる場合もある.妊娠は禁忌とされ,感染症予防の目的でインフルエンザや肺炎球菌の予防接種が勧められている. これらの支持療法を行いつつ,IPAH/HPAHについてはまずNO吸入かエポプロステノール静注法を用いた急性肺血管反応性試験を行うことが推奨される.急性肺血管反応性試験の詳細は「肺高血圧症の診断」の項を参照されたい.肺血管反応性が陽性の症例についてはカルシウムチャンネル拮抗薬(CCB)療法が推奨される.CCB治療開始後にNYHA/WHO機能分類のⅡ度またはⅠ度に改善すれば本治療を続行する. 急性肺血管反応性試験の陰性例,またはCCB治療を行っても症状の改善がみられない例については特異的PAH治療薬を速やかに開始する.特異的PAH治療薬には前述のように3系統の治療薬が存在し,そのいずれかを用いて治療を開始するが,NYHA/WHO機能分類の観点からⅡ度・Ⅲ度例に対しては,ERA,PDE5-Iの薬剤は適応とするエビデンスがある.またNYHA/WHO機能分類Ⅲ度例の中でも特に「症状の進行速度など」他の重症度指標で予後不良の項目が多い重症例ではエポプロステノールの適応を考慮する.NYHA/WHO機能分類Ⅳ度例はエポプロステノールの適応である. 単剤で十分な治療効果が得られない場合,現在は2剤,
POPHは予後不良で,最近の報告によると,POPHで治療を行わなかった場合の5年生存率は14%であり,54%の患者が診断から1年以内に死亡する166).肝移植を行わずPOPHの加療のみを行った場合の5年生存率は45%で,肝移植が行える場合,5年生存率は67%と改善する167).肝移植前に肺血管拡張薬を併用し,肺血行動態を改善した上で肝移植を行うことによってPOPHの予後が改善する可能性があるが,今後の検討が待たれる. 肝移植を行うためのPOPHの治療目標は mean PAPが
32
循環器病の診断と診療に関するガイドライン(2011 年度合同研究班報告)
35mmHg以下,かつPVRが250 dynes・sec・cm-5以下である3).これは肝移植前の mean PAPが35mmHg以上の患者は,35mmHg以下の患者に比べ有意に死亡率が高いため167),肺血管拡張療法により mean PAP が35mmHg以下にならなければ肝移植は相対的に禁忌と考えられる. 右心カテーテル検査はPOPHの重症度判定に非常に重要で168),疾患の重症度に応じて治療オプションを考慮する169)(表13).
③POPHに特異的な診断基準と治療アルゴリズム
1)診断 POPHの診断基準を表14に示す3).肝硬変では全身血管抵抗の減少とそれに伴う心拍出量の増加を認めることがあり,その場合は心拍出量の増加に伴い肺動脈圧が上昇し,PVRが低下していることになる(肺肝症候群).肺肝症候群では一般的にPVRが低下することから,PVRの上昇がPOPHの特徴といえる. 診断のためには他の肺高血圧症と同様に,経胸壁心エコーでスクリーニングを行い,右心カテーテルで mean PAP,PAWP,CO(CI),PVRを測定し,POPHを診断する.2)治療(表15) POPHに特異的な治療は肝移植である.移植前に mean PAP 35mmHg以上の場合は予後不良であるため,PHの治療目標を, mean PAPが35mmHg未満,PVRが250 dynes/sec-cm-5未満を目標とする3),170). その他の治療は他のPAHと同様であるが,IPAHに使用する薬剤に関してPOPHで使用するにあたり注意が必要な場合がある. まず利尿薬に関しては慎重に用いる必要がある.右心
不全と慢性肝疾患に伴い血管内の血液量は増加しており,利尿薬はそれを減少させることで効果を認めるが,血液量が減少することで前負荷が減少し CO が減少する可能性がある171).抗凝固療法は IPAHや慢性肺塞栓症で有効であるが,肝硬変患者では易出血性であり,特に消化管出血の可能性が高くなるためPOPHには勧められない. 前述の通り,PDE5-Iも有効で,PVRを低下させるが,シルデナフィルにより6分間歩行を1年後に改善するものの, mean PAP,PVRに関して投与1年後には改善を維持できなかった症例も報告されており172),今後検討が必要である. プロスタサイクリン(エポプロステノール)はPOPHにも効果のある治療である.中等度から重症のPOPHではエポプロステノールの静脈注射が急性期も慢性期にも肺血行動態および心負荷所見を改善する. 小規模試験ではあるが,ERAのボセンタンでPOPHの生存期間の延長と右心不全の再発減少を認め173),アンブリセンタンで血行動態を改善した試験が報告されている174). これらの血管拡張薬の併用療法は IPAHではその有効性が示されているが,POPHでの併用に関しての報告は少なく167),これも今後の検討課題である.
初発症状は労作時息切れ,呼吸困難が多い.PAHに比較して安静時の酸素飽和度の低下や軽労作時の著明な低下が目立ち,拡散能が低値であることは診断に有用である.胸部単純X線では間質影の増強,Kerley B lineがみられる.HRCTでの胸膜直下の小葉間隔壁肥厚,小葉中心性のground glass-opacity,縦隔リンパ節腫大の3徴候が診断に有用である191).肺換気─血流シンチグラムでは,亜区域性の血流欠損を認める.
2)相対的除外条件 a)肝臓,腎臓の可逆的機能障害 b)活動性消化性潰瘍 c )合併症を伴ったインスリン依存性糖尿病 d)高度胸郭変形や胸膜に広範な癒着や瘢痕 e )高度筋・神経疾患 f )極端な低栄養または肥満 g) リハビリテーションが行えない,またはその能力が
期待できない症例 h)本人および家族の理解と協力が得られない I )精神社会生活上に重要な障害
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循環器病の診断と診療に関するガイドライン(2011 年度合同研究班報告)
肺高血圧の作用機序を図10に示した216). まず,左心不全は左室収縮機能の低下に基づく心不全と左室収縮機能の保持された心不全に分けられるが,原因にかかわらず左室充満圧は上昇し,その結果左房圧も上昇する.僧房弁狭窄症や左房粘液種では,左室への血液流入障害があるため左室充満圧の上昇はないが,やはり左房圧は上昇する.上昇した左房圧は肺静脈から肺毛細血管を介して肺動脈系に伝搬され,肺動脈圧は上昇する.この段階では肺高血圧は可逆性で,治療により左房圧が下がれば肺動脈圧も低下する.しかしこの状態が長期にわたり持続すると,肺動脈の反応性収縮が起こり肺高血圧が悪化する.さらには肺動脈のリモデリングが起こり解剖学的変化を来たすと,肺高血圧はさらに進行し,肺高血圧はもはや不可逆性となってしまう.このような状態は以前は“out of proportion”,つまり,肺静脈圧の上昇では説明のできないほどの肺高血圧を来たした状態と言われ,原疾患の治療を行っても肺高血圧は改善せず,弁置換後,心臓移植後の右心不全につながってゆく. 左房圧の上昇を来たす左心疾患のうち,収縮性心膜炎では例外的に肺動脈圧の上昇は肺静脈圧を反映しない.収縮性心膜炎では右心系の充満圧も左心系と同様に上昇するため,左心系充満圧の肺動脈への伝搬は起こらない.また1回拍出量の低下に伴い右室の発生圧は低いため,右室拡張期圧+右室発生圧で決定される肺動脈収縮期圧は上昇しない.
COPDに合併する肺高血圧の治療は慢性安定期と増悪期とで異なる.前者では酸素療法,後者では酸素療法に加え抗菌薬など増悪因子への対処が中心となる.なお,COPD自体に対する適切かつ最善の気管支拡張薬の投与は肺高血圧治療に対しても重要である(表29).1)慢性安定期①酸素療法(表30) COPDに合併する肺高血圧に対して唯一有効性が証明されている治療法である.在宅酸素療法の施行によって肺高血圧ないし肺性心の進行の阻止,および生命予後の改善が期待され264),265),さらにうつ傾向の軽減など精神神経機能への効果も期待できる260).ただし本治療による肺高血圧の部分的進展抑制効果が示されているものの,肺動脈圧の正常化は期待できない267).酸素の投与にあたっては低流量から開始しCO2ナルコーシスを避ける注意が必要である.運動時のみ,および睡眠中のみの低酸素血症に対する在宅酸素療法の長期効果についてはエビデンスに乏しく意見が一致していない268).②血管拡張薬 血管拡張薬は直接的な肺動脈圧降下作用を持つ一方,低酸素性肺血管攣縮を解除することで低酸素血症の増悪や体血圧の低下をもたらす可能性がある269).これまで種々の血管拡張薬の全身投与が試みられたが,酸素療法に匹敵する効果は証明されていない.一酸化窒素(NO)の吸入療法も明らかな有用性は示されていないが270),271),酸素吸入との併用が有効であるとの報告がある272). 一方ASPIRE(Assessing the Spectrum of Pulmonary hypertension Identified at a Referral centre)登録研究のサブグループ解析では,肺血管拡張療法に対する治療反応
CPFEやそれ以外の肺疾患であっても,肺高血圧の重症度が背景となる肺疾患の重症度と比較して明らかに重篤であると判断される症例が存在し,これはout of proportion PHと称される3).このような症例は,他の通常の肺疾患合併肺高血圧症と病態が異なる可能性があり,血管拡張療法の治療効果も別個に検証される必要がある.今後はこのようなout of proportion PHを呈する3群肺高血圧においては,薬物治療介入の意義が無作為前向き試験によって検証されるべきである.
③ 肺動脈造影所見慢性化した血栓による変化として,1.pouch defects,2.webs and bands,3.intimal irregularities,4.abrupt narrowing,5.complete obstructonの5つのうち少なくとも1つが証明される.
④ 胸部造影CT所見造影CTにて,慢性化した血栓による変化として,1.mural de fec t s,2.webs and bands,3. in t imal irregularities,4.abrupt narrowing,5.complete obstructionの5つのうち少なくとも1つが証明される.
などの投与を行い,肺血行動態や運動体用能の評価で有効との研究報告も存在する.さらに血管拡張療法が使用されて以後,本症の予後が改善したとする報告もみられる335),340).また現在,グアニルシクラーゼ活性化薬や,PGI2受容体アゴニストの臨床試験も行われている. 血管拡張療法の適応としては,NYHA/WHO Class Ⅱ以上の症例で,末梢型CTEPHや合併症を有し手術困難例,本人が手術を希望しない例,手術後に肺高血圧症残存例などで,その可能性がある4),338).また重症の手術適応例では術前の肺血行動態を改善し,手術成績の向上を期待した術前の使用も試みられているが,その評価は定まっていない344),345),349),350).
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