Management
Since no guidelines for the management of patients with LVNC exist, we have suggested a strategy based on case series, registry data, previous reviews and local experience (figure 4). Patients who are asymptomatic and have normal LV systolic function may be followed every 2–3 years with clinical assessment and echocardiography, as their prognosis is usually good. Clinical visits should comprise history, physical examination, echocardiography as well as Holter monitoring, to identify silent arrhythmias. Patients who are asymptomatic, but have echocardiographic LV systolic and/or diastolic dysfunction should be treated with evidence-based HF therapy, and followed every 1–2 years, and undergo the original investigations at subsequent visits. Symptomatic patients should be managed based on their clinical presentation based on the respective consensus guidelines. Neurological referral at the time of diagnosis of LVNC is also appropriate. In the correct clinical context, when regional wall-motion abnormalities or LV dysfunction is present, an elective coronary angiogram could be considered to rule out obstructive coronary disease. There is, however, no evidence that LVNC is associated with increased risk of coronary artery disease.
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Figure 4.
Suggested management algorithm for patients with left ventricular non-compaction.
There is controversy regarding the routine use of anticoagulation in patients with LVNC. Some in the past have argued that all patients should be anticoagulated with warfarin, while others recommend anticoagulating only those with any of the following: LV dysfunction (fractional shortening <25% or EF <40%), AF, previous history of embolic events, or those with known ventricular thrombi. There is, however, no robust data to support either approach. We support the use of the second approach, which has been shown to reduce the incidence of thromboembolic events in one study.
The issue of ICD implantation in all LVNC patients has been proposed due to the high risk of sudden cardiac death, but is also highly controversial. Implantation of an ICD is indicated for secondary prophylaxis in patients with sustained ventricular arrhythmia with haemodynamic compromise, and those with aborted sudden cardiac death. However, for the purpose of primary prevention, current guidelines for non-ischaemic CM should be considered (EF≤35%, NYHA 2–3, optimal medial therapy, life expectancy with good functional status for >1 year). It is also important to realise that although the incidence of SCD (Table 2) in LVNC is high (1%–9% in recent studies), it is still significantly lower than that seen in ICD trials of patients with ischaemic and non-ischaemic CM.