p.60 抽出テキスト

included patients with CAD and DCM. The COMPANION study differed from the others as it compared CRT-defibrillator, CRT-pacemaker, and OMT. A meta-analysis of the five trials (1854 patients with DCM) demonstrated a 31% reduction in allcause mortality with ICD relative to medical therapy (RR 0.69 [95% CI 0.55, 0.87], P , 0.002).658 This effect persisted when COMPANION was excluded. More recently, the DANISH trial enrolled 1116 symptomatic patients (NYHA class II or III) with non-ischaemic systolic heart failure (LVEF ≤ 35%). Patients were randomized to ICD or no ICD after OMT.359 No reduction in the primary endpoint of all-cause death for patients randomized to ICD therapy (HR 0.87; 95% CI 0.68–1.12; P = 0.28) was observed, despite a significant reduction in SCD in the ICD group (HR 0.50; 95% CI 0.31–0.82; P = 0.005). Potential explanations are the low SCD rate in the trial (4.3% in the ICD group and 8.2% in the control group), the excellent medical treatment (.90% of patients on ACE inhibitors and beta-blockers, .50% on MRA), and the high prevalence (58%) of CRT. A meta-analysis of all six primary prevention trials still demonstrated a reduction, although lower, in overall mortality with ICD therapy (RR 0.76; 95% CI 0.65–0.91; P= 0.002).635 In a sensitivity analysis, the benefit of ICD therapy was maintained after the removal of any single study from the pooled analysis.

ECG sinus rhythm – Small amplitude P waves and first degree AV block

ECG VT – RBBB-like, inferior axis (LV summit origin)

CMR – Mid-wall septal LGE

4-chamber Short axis

I

II

III V1

V2

V3

aVR

aVL

aVF V4

V5

V6

I

II

III V1

V2

V3

aVR

aVL

aVF V4

V5

V6

Figure 20 Typical features of dilated cardiomyopathy associated with lamin A/C gene mutation with ventricular arrhythmias. AV, atrioventricular; CMR, cardiac magnetic resonance; ECG, electrocardiogram; LGE, late gadolinium enhancement; LV, left ventricle; RBBB, right bundle branch block; VT, ventricular tachycardia. Downloaded from academic.oup.com/eurheartj/article/43/40/3997/6675633 by guest on 07 October 2026