Prognosis of Isolated Left Ventricular Non-compaction in Adults

Isolated Left Ventricular Non Compaction (LVNC) is a rare cause of cardiomyopathy supposed to result from the cessation of normal embryogenesis infarction, and characterized by persistent ventricular trabeculations prominent.

This is frequently a familial disease, but for which genetic characterization is still incomplete, and then requires the identification of new genes is desirable.

The prognosis of LVNC is uncertain, with a mortality rate reported in the literature ranging from 2 to 38%. Some series conclude that LVNC is a very severe heart disease, responsible for a high mortality, other that LVNC is frequently associated with a favorable prognosis. These series are however limited by the short duration of follow-up and the small number of patients included.

Between 2004 and 2006, a French registry LVNC, included 105 cases. It was found out that the LVNC was associated with a high rate of complications such as outbreaks of severe heart failure, need for heart transplantation, severe rhythm disorders, and embolic events. The prognosis of LVNC in France appears as pejorative:

• - there is no evidence that prognosis is different from other forms of cardiomyopathies.
• - the results of this register can be skewed by the inclusion of incident and prevalent cases (statistical survival bias).

Thus, a longer-term monitoring and the identification of relevant prognostic markers are imperative to better understand this rare disease and to improve the therapeutic management.

Inclusion criteria

• Male or female aged over 18 years
• Presenting with Left Ventricular Non Compaction (LVNC, Group 1) or Idiopathic Dilated Cardiomyopathy (DCM, Group 2)
• At distance of an acute heart failure thrust (> 1 month)
• Newly diagnosed (less than 6 months)
• Diagnosis confirmed by echocardiography associated or not with a Magnetic Resonance Imaging (MRI) confirmed after central review
• Having signed informed consent form

Exclusion criteria

• Age <18 years
• Patients who were diagnosed more than 6 months ago (prevalent cases)
• Presence of an associated cardiac disease, including valvular, ischemic, or congenital disease
• Refusal to sign the informed consent form