A good working definition of FM is a sudden and severe inflammation of the myocardium resulting in myocyte necrosis, edema, and cardiogenic shock. It is important to distinguish FM from other forms of acute circulatory compromise, the most common being an acute coronary syndrome.
The fulminant presentation may be a marker of a more robust immunological/inflammatory response indicative of more effective viral clearance and thus predictive of eventual complete myocardial recovery, at least among those with an infectious myocarditis.
Children and women may be more susceptible to this dramatic presentation. Other medical disorders such as lupus and celiac sprue may be concomitantly present and likely play a direct role in the pathogenesis of the myocardial inflammation, either alone or in concert with a propensity toward specific viral insults.
Recognition and Initial Management of Fulminant Myocarditis: A Scientific Statement From the American Heart Association
Robb D. Kociol ,et al
Originally published ,6 Jan 2020
https://doi.org/10.1161/CIR.0000000000000745
Circulation. ;0:CIR.0000000000000745
激症型心筋炎：Fulminant myocarditis (FM) は、珍しい症候群だが、突然の重症の心筋炎症を特徴とし、しばしば心原性ショック、心室性不整脈、MOFに至る疾患。歴史的には剖検診断が主。
定義だと、終末臓器還流維持のためのサポートにinotropic or mechanicalな循環器サポートが必要で、移植・回復まで必要。
ある特定のサブタイプだと免疫抑制剤をガイドライン指示医療に加えることで反応する場合があるかもしれない
循環器サポート、 orthotropic 心移植、疾患特異的治療にかかわらず、重篤な合併症・死亡率を生じる、一部には診断や循環器サポートの遅れ、適切な訓練者の不足も一因となり得る。
診断：
Consider myocarditis in young patients with apparent cardiovascular conditions often presenting as more common conditions such as ACS or de novo AHF.
Young patients without typical cardiovascular risk factors and history of signs and symptoms of recent viral URI or enteroviral infection presenting with cardiovascular symptoms.
Recognize typical signs and symptoms of RHF (右心不全)such as RUQ pain, LFT: liver function test abnormalities, jaundice, elevated neck veins, peripheral edema, hepatomegaly with liver pulsatility. Distinguish RHF from primary hepatobiliary disease such as cholecystitis early before progressive cardiogenic shock.
Triage
Early recognition of circulatory compromise such as a narrow arterial pulse pressure, sinus tachycardia, cool or mottled extremities, or elevated lactate. Patients may present febrile secondary to severe inflammation. Although the more common diagnosis is infection and sepsis, this may also be severe myocarditis. Discriminating sepsis from early cardiogenic shock secondary to myocarditis is challenging during the early stages of workup and treatment. A high index of suspicion is warranted.
初期治療
Avoid treatment of sinus tachycardia with rate control agents (especially those with negative inotropic properties such metoprolol, diltiazem, or verapamil).
Among patients with systolic dysfunction, cardiac output may significantly depend on a compensatory increase in heart rate given a minimal ability to augment stroke volume in the acutely affected nondilated heart.
Consider hypersensitivity myocarditis, a subset of eosinophilic myocarditis, generally presenting as FM with peripheral eosinophilia (65% of patients), rash, or elevated LFTs.
Patients often will have a fever and high risk (43%) of death, transplantation, or VAD placement at 120 d.
An EMB(endomyocardial biopsy:心筋生検) is often necessary for definitive diagnosis. Common causative agents are antibiotics such as β-lactams and minocycline and certain central nervous system drugs such as clozapine and carbamazepine.Avoid NSAIDs because they may increase Na retention, cause myocardial harm, and exacerbate renal hypoperfusion.
急性喉頭蓋炎とならぶ、恐ろしい病気
