心室肌致密化不全(NVM)产妇的麻醉处理？

gaojingui

昨天，我们遇到一例罕见的剖宫产的病例，患者妊娠30周，因心慌、气短，不能平卧急诊入院，诊断为NVM（noncompaction ventricular of myocardium）。经我院心脏内科抢救、纠正心衰后，急诊行剖宫产术，不知同行们哪位遇到过此类患者？麻醉选择？

lirui238

心室肌致密化不全常有心律失常，以快速室性心律失常最常见，还有预激综合症，发生阵发性的室上速时容易诱发室速或室颤，心室内易有血栓形成，所以最好做个超声心动图，利多，胺碘酮等治疗心律失常的药物准备好以及除颤器，
我觉得我会选择腰麻，控制麻醉平面，维持血压心率平稳，如果用全麻诱导时如何用药，万一镇痛不全，插管刺激更大，用药过多对婴儿有影响，所以我觉得我会选择腰麻，个人愚见，错误之处希望前辈们给予批评指出

shenxiu2

Review article on Noncompaction of Left Ventricle ( LVNC)
DEFINITION — LVNC is a rare disorder, classified as a primary genetic cardiomyopathy by the American Heart Association .
LVNC is characterized by the following features:
·        An altered myocardial wall with prominent trabeculae and deep intertrabecular recesses resulting in thickened myocardium with two layers consisting of compacted and noncompacted myocardium .
·        Continuity between the left ventricular cavity and the deep intratrabecular recesses, which are filled with blood from the ventricular cavity without evidence of communication to the epicardial coronary artery system.
·        LVNC may be due to intrauterine arrest of compaction of the loose interwoven meshwork that makes up the fetal myocardial primordium.

GENETICS — LVNC can be either sporadic or familial. Rarely LVNC can occur as a transient phenomenon during myocarditis .  12 to 50 percent of  LVNC had a family history  .

CLINICAL MANIFESTATIONS — The major clinical manifestations of LVNC are heart failure, atrial and ventricular arrhythmias, and thromboembolic events, including stroke..
At diagnosis, the clinical manifestations included:
·        Dyspnea
·        New York Heart Association class III or IV heart failure  
·        Chest pain  
·        Chronic atrial fibrillation
The electrocardiogram is usually abnormal , but there are no characteristic changes . The abnormalities that can be seen include left or right bundle branch block, fascicular block, atrial fibrillation, and ventricular tachycardia.

DIAGNOSIS — The diagnosis of LVNC is usually established by echocardiography.
Echocardiography —Criteria for diagnosis:
·         A thickened left ventricular wall consisting of two layers: a thin compacted epicardial layer; and a markedly thickened endocardial layer with numerous prominent trabeculations and deep recesses with a maximum ratio of noncompacted to compacted myocardium > 2:1 at end-systole in the parasternal short-axis view .
·        - Color Doppler evidence of flow within the deep intertrabecular recesses.
·        - Prominent trabecular meshwork in the LV apex or midventricular segments of the inferior and lateral wall.
All three echocardiographic criteria were required for diagnosis. The criteria are assessed in the parasternal short-axis view at base, mid and apical levels.Other nonspecific findings that can be seen on echocardiography include reduced global left ventricular systolic function, diastolic dysfunction, left ventricular thrombi, and abnormal papillary muscle structure .

PROGNOSIS —  LVNC is associated with high rates of morbidity and mortality in adults. Patients with or without atrial fibrillation are at high risk for arterial thromboembolism .Atrial and ventricular arrhythmias are common causes of morbidity and mortality .

MANAGEMENT — Data on treatment of LVNC are limited, and there is no specific therapy for LVNC. Medical management varies with the clinical manifestations, left ventricular ejection fraction (LVEF), the presence or absence of arrhythmias, and perceived risk of thromboembolism.
SUMMARY on Management:
·        For patients with LVNC with reduced LVEF and HF( Heart failure),  use standard medical therapy for HF due to systolic dysfunction.
·        For patients with LVNC with reduced LVEF without HF,  use standard medical therapy for asymptomatic systolic dysfunction.
·        Patients with LVNC and atrial fibrillation who meet standard criteria for anticoagulation should be anticoagulated according to standard guidelines.
·        We recommend anticoagulation with warfarin in patients with LVNC without atrial fibrillation with LVEF <40 percent .
·        For all patients with LVNC, we suggest annual Holter monitoring to detect asymptomatic arrhythmias.
·        Patients with LVNC should receive ICD (implantable cardioverter-defibrillator) therapy according to standard indications for secondary and primary prevention of SCA( Sudden cardiac arrest).
·        - Patients with LVNC who survive an episode of sustained ventricular tachycardia or SCA should receive implantable cardioverter-defibrillator (ICD) therapy for secondary prevention of SCA(Sudden cardiac arrest).
·        - Patients with LVNC with an LVEF ≤35 percent and NYHA class II to III HF should receive ICD therapy for primary prevention of SCA.
·        Until further data are available, we suggest implantable cardioverter-defibrillator (ICD) therapy for primary prevention of SCD if one or more of the following features are present: syncope, non-sustained ventricular tachycardia, severe left ventricular dysfunction (LVEF ≤35 percent) OR family history of SCA. .
·        Patients with LVNC who have end-stage heart failure are candidates for cardiac transplantation evaluation.

节录自"与时并进"综述文章2009年九月.

gaojingui

谢谢两位同道的积极回帖，我们选择的是连续硬膜外麻醉，麻醉、手术很成功，婴儿也安全出生，稍后把麻醉记录单贴上与大家讨论，请继续批评指正！