Prognosis of Isolated Left Ventricular Non-compaction in Adults
Overview
- Phase
- Not Applicable
- Intervention
- Not specified
- Conditions
- Left Ventricular Non Compaction
- Sponsor
- Assistance Publique Hopitaux De Marseille
- Enrollment
- 175
- Locations
- 1
- Primary Endpoint
- Occurrence of death of cardiac origin
- Status
- Completed
- Last Updated
- 3 years ago
Overview
Brief Summary
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
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: 1. - there is no evidence that prognosis is different from other forms of cardiomyopathies. 2. - 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.
Investigators
Eligibility Criteria
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
Outcomes
Primary Outcomes
Occurrence of death of cardiac origin
Time Frame: Up to 2 years
Occurrence of hospitalization due to cardiac event
Time Frame: Up to 2 years
Occurrence of a cardiac transplantation
Time Frame: Up to 2 years