Eisenmenger syndrome - Eisenmenger syndrome

Japanese: アイゼンメンジャー症候群 - アイゼンメンジャーしょうこうぐん（英語表記）Eisenmenger syndrome
Eisenmenger syndrome - Eisenmenger syndrome

What is the disease?

Cyanosis (purple discoloration of the skin and mucous membranes) Hemoptysis ( Cutting edge ) A 32-year-old Vienna patient died of Ventricular septal defect ( Evidence-based survey ) About 100 years ago, Dr. Eisenmenger reported in a medical journal that there was a condition in which venous blood flows into the arteries due to pulmonary hypertension, leading to the appearance of cyanosis, and this condition came to be called Eisenmenger syndrome after his name.

Pulmonary artery pressure changes with the contraction and expansion of the heart, but is expressed as the highest blood pressure, called systolic blood pressure, the lowest blood pressure, called diastolic blood pressure, and the average of these, called mean blood pressure. Pulmonary artery pressure can be measured by inserting a thin tube called a catheter into the pulmonary artery, or estimated from outside the body using an echocardiogram.

Pulmonary hypertension is defined as a condition in which the mean pulmonary artery pressure is 25 mmHg or higher. People with mild pulmonary hypertension are not called Eisenmenger syndrome. Also, even if the pulmonary artery pressure is high, a large amount of arterial blood flowing into the venous side is not called Eisenmenger syndrome.

Eisenmenger syndrome is Atrial septal defect ( Heart space exploration ) , ventricular septal defect, Patent ductus arteriosus ( Physiological analysis ) Pulmonary hypertension occurs when a person who has a disease in which arterial blood normally flows into the venous side, such as those mentioned above, develops gradually severe pulmonary hypertension, causing some of the venous blood to flow into the arterial side.

In Eisenmenger syndrome, if there is a ventricular septal defect or patent ductus arteriosus, the pulmonary artery systolic blood pressure and the aortic systolic blood pressure are almost the same.

Testing and diagnosis

Once you have Eisenmenger syndrome, surgery to correct the underlying heart disease becomes extremely risky. If you choose to undergo surgery, even if you survive, there is a high risk that it will actually shorten your lifespan.

Therefore, people who are on the borderline of having this syndrome and are considering undergoing surgery must undergo rigorous testing, including cardiac catheterization. Although catheterization does have risks, it is one of the most important tests for making a diagnosis.

Treatment methods

Because surgery involving general anesthesia is extremely risky, it is important to consult with your doctor and be completely aware of the risks before using any medication that may lower your systemic blood pressure.

There are reports that home oxygen therapy and oral and intravenous medications for pulmonary hypertension are effective. If you use these medications, be sure to consult with your doctor about which ones to use.

Pulmonary embolism ( Fast train ) Preventive medications such as aspirin and warfarin are not usually used, but they may be used for other purposes, so please discuss this with your doctor.

If you have a persistent fever or severe headache even though you have no cold symptoms, you may have endocarditis or a brain abscess, so consult your doctor.

Pregnancy and childbirth are contraindicated (things that should not be done) in people with Eisenmenger syndrome.

Toshio Nakanishi

Source: Houken “Sixth Edition Family Medicine Encyclopedia” Information about the Sixth Edition Family Medicine Encyclopedia

Japanese:

どんな病気か

　チアノーゼ（皮膚や粘膜が紫色になる）があり、喀血(かっけつ)で亡くなった32歳のウイーンの患者さんに大きな心室中隔欠損(しんしつちゅうかくけっそん)があったことを、アイゼンメンジャー医師が約100年前に医学雑誌に報告しました。その名前にちなんで、肺高血圧があるために静脈血が動脈側に流れ込み、チアノーゼが出現するに至った状態をアイゼンメンジャー症候群と呼ぶようになりました。

　肺動脈圧は、心臓の収縮と拡張に伴って変化しますが、収縮期血圧と呼ばれるいちばん高い血圧と、拡張期血圧と呼ばれるいちばん低い血圧、そしてその平均である平均血圧で表されます。肺動脈の圧力は、カテーテルという細い管を肺動脈まで入れて測定したり、心エコー装置で体外から推定したりします。

　肺動脈の平均血圧が25㎜Hg以上ある場合を、肺高血圧があると定義します。軽度の肺高血圧の人はアイゼンメンジャー症候群とは呼びません。また、肺動脈圧が高い状態でも、大量の動脈血が静脈側に流れ込んでいる状態はアイゼンメンジャー症候群とは呼びません。

　アイゼンメンジャー症候群は心房中隔欠損(しんぼうちゅうかくけっそん)、心室中隔欠損、動脈管開存(どうみゃくかんかいぞん)など、通常は動脈血が静脈側に流れ込む病気をもつ人が、肺高血圧がだんだん高度となって、静脈血の一部が動脈側に流れ込むようになって発症します。

　アイゼンメンジャー症候群になると、心室中隔欠損や動脈管開存がある場合には、肺動脈の収縮期血圧と大動脈の収縮期血圧とはほぼ同じになります。

検査と診断

　アイゼンメンジャー症候群になると、心臓のもともとの病気を治す手術は非常に危険が高くなります。手術をあえて行って、たとえ生存しえても、その後の寿命をかえって縮める危険性が高くなります。

　したがって、本症候群かどうかの境界にいる人で、手術を受けようとする場合には、心臓カテーテル検査を含めた厳密な検査を受ける必要があります。カテーテル検査はリスクはありますが、診断のために最も重要な検査のひとつです。

治療の方法

　全身麻酔を伴う手術は非常に危険なため、全身の血圧を下げる可能性のある薬は、医師と相談して納得のうえで使用することが重要です。

　在宅酸素や肺高血圧に対する経口薬、静脈投与薬に効果があるとする報告があります。使用するとしたら、どの薬を使用するかを、医師と相談して使用するようにしてください。

　肺塞栓(はいそくせん)予防のためのアスピリンやワルファリンという薬剤は、通常は使用しません。ただし、ほかの目的のために使用することがあるので、医師と相談してください。

　かぜ症状がないのに熱が続いたり、頭痛がひどい時には、心内膜炎や脳膿瘍の可能性もあるので、かかりつけ専門医に相談しましょう。

　アイゼンメンジャー症候群では、妊娠、出産は禁忌（やってはいけないこと）です。

中西　敏雄

出典　法研「六訂版 家庭医学大全科」六訂版 家庭医学大全科について　情報

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