肺高血圧症
[定義]
肺高血圧症とは「平均肺動脈圧(mean PAP:mPAP)25mmHg以上」と定義される。
肺高血圧症は「平均肺動脈圧が上昇する原因」によって分類され、様々な疾患が肺高血圧症を引き起こし得る。ここでは肺動脈性肺高血圧症と慢性血栓塞栓性肺高血圧症を扱う。
[病態]
肺動脈内の圧力が上昇することで右心負荷が増加するとともに心拍出量が減少する。
右心負荷に右室が耐えられなくなると右心不全となる。
[症状]
心拍出量が減少することで全身に十分に血液を送り出せなくなるとともに酸素の供給が不足する。
このため、労作時に息切れや動悸を感じるようになり脳への血流が不足して酸素が不足すると失神してしまうこともある。低酸素血症によるチアノーゼを認める。
悪化すると右心不全の症状(頚静脈怒張・肝腫大など)が出てくるようになり、最終的には心臓が止まって死亡する。
映画『余命10年』では特発性肺動脈性肺高血圧症の患者の余命10年が描かれており肺高血圧症の症状を理解するには良い映画であると思われる。それ関係なしに見ても良い映画であるのでオススメします。
[所見]
Ⅱ音の亢進
Ⅱ音は大動脈閉鎖音(ⅡA)と肺動脈閉鎖音(ⅡP)で構成されており、肺高血圧症ではⅡ音肺動脈成分の亢進を認める。
[肺高血圧症の鑑別]
①肺高血圧症を疑ったらまず心エコーによって推定肺動脈収縮期圧を算出することで肺高血圧症のスクリーニングを行う。このとき、心エコーでは右室腔の拡大を認める。
②次に肺血流シンチグラムで肺動脈性肺高血圧症と慢性血栓塞栓性肺高血圧症を鑑別する。
肺動脈性肺高血圧症(PAH)
肺動脈の血管内腔が狭くなったことで肺血管抵抗が上昇して生じる肺高血圧症を肺動脈性肺高血圧症と総称する。
特発性肺動脈性肺高血圧症は原因が不明のものであり、遺伝性肺動脈性肺高血圧症は原因が遺伝的なものである。また、各種疾患に伴う肺動脈性肺高血圧症には全身性強皮症・SLE・混合性結合組織病(MCTD)といった膠原病に伴うものとEisenmenger症候群といった先天性心疾患に伴うものがある。
肺動脈性肺高血圧症の所見
胸部CTで中枢側肺動脈の拡張と末梢側肺動脈の急激な狭小化を認める。
胸部X線で肺動脈主幹部の拡大(左第2弓突出)と末梢肺血管陰影の細小化を認める。
肺動脈性肺高血圧症の確定診断
右心カテーテル検査によって平均肺動脈圧：高値・肺動脈楔入圧：正常を確認する。
肺動脈性肺高血圧症の治療
エンドセリン受容体拮抗薬＋エポプロステロール(プロスタグランディンI2製剤)在宅持続静注療法＋在宅酸素療法
エンドセリンはエンドセリン受容体に結合して血管収縮作用を示す。
プロスタグランジンには血管拡張作用がある。
内科的治療に抵抗性の場合には肺移植の適応がある。
慢性血栓塞栓性肺高血圧症(CTEPH)
慢性的に血栓(血管内で生じた血の固まり)や塞栓(血栓の一部が剥離して遠くまで運ばれたもの)が肺動脈内で詰まることによって生じる肺高血圧症を慢性血栓塞栓性肺高血圧症と呼ぶ。主には急性肺血栓塞栓症からの移行が想定されている。
慢性血栓塞栓性肺高血圧症は肺血栓塞栓症のカテゴリーに属しており、肺動脈性肺高血圧症とは全く別の病態であることを押さえるのが重要である。医師国家試験では主に急性肺血栓塞栓症が問われるので、急性肺血栓塞栓症をよく勉強するべきである。慢性血栓塞栓性肺高血圧症は急性肺血栓塞栓症の一部が移行して、急性肺血栓塞栓症より緊急的ではないものの慢性的に肺血栓塞栓症が生じるようになってしまったことで肺高血圧症が引き起こされたものであると考えれば分かりやすい。
慢性血栓塞栓性肺高血圧症の検査
肺血流シンチグラムで血流欠損を認める。
慢性血栓塞栓性肺高血圧症の確定診断
胸部造影CTで血栓を描出して確定診断する。
慢性血栓塞栓性肺高血圧症の治療
抗凝固療法＋在宅酸素療法
内科的治療に抵抗性の場合には肺移植の適応がある。
映画「余命10年」と肺高血圧症の関連
映画「余命10年」では肺動脈性肺高血圧症患者の余命10年が題材とされている。
簡単にあらすじを書くと、
小松菜奈が演じる主人公：高林茉莉さんは肺動脈性肺高血圧症に罹り、現代医学では完治することがないため自らが余命10年ということを知りながら生きていくことになる。
余命10年というのは短いような長いような中途半端な余命であり、余命10年が始まった当初は治療によって症状を抑えることができているので普通の健常な人間と何も変わらない生活を送ることができている。
しかし、自分が余命10年というのは変わりようがない事実であり、この余命10年という重みが恋愛や生き方に大きく影響を与えるというようなあらすじである。
余命10年では映画中でかなりリアルに病気に関して描かれており、肺動脈性肺高血圧症の症状を理解するのにかなり役立つと思われる。
また、肺動脈性肺高血圧症という医師国家試験でよく問われる疾患が扱われているのでこの肺動脈性肺高血圧症という疾患自体に興味を持つためにも一度見ることをオススメします。
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