Prognosis of Isolated Left Ventricular Non-compaction in Adults

Study Overview
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Brief Summary
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The main objective of this study is to clarify prospectively prognosis of patients newly diagnosed as carriers of a LVNC (incident cases) (i.e. without the occurrence of a survival of the following events: death, heart transplantation or hospitalization for cardiovascular complications). In a second time, prognosis factors will be identify in these patients with LVNC.

Detailed Description
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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.

Conditions
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Left Ventricular Non Compaction

Intervention / Treatment
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* Other: Clinical examination
* Other: Blood sample
* Procedure: Echocardiography


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Diagnostic
Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Patients with newly diagnosed Left Ventricular Non Compaction<br>Patient newly diagnosed with Left Ventricular Non Compaction (diagnose < 6 months), confirmed by echocardiography associated or not with MRI, after centralized review | Other: Clinical examination<br> <br> Other: Blood sample<br> <br> Procedure: Echocardiography<br> <br> |
| Active Comparator: Patients with Idiopathic Dilated Cardiomyopathy<br>Patient newly diagnosed with Idiopathic Dilated Cardiomyopathy (diagnose < 6 months), confirmed by echocardiography associated or not with MRI, after centralized review | Other: Clinical examination<br> <br> Other: Blood sample<br> <br> Procedure: Echocardiography<br> <br> |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Occurrence of death of cardiac origin |  | Up to 2 years | 
| Occurrence of a cardiac transplantation |  | Up to 2 years | 
| Occurrence of hospitalization due to cardiac event |  | Up to 2 years |