Prognosis of Isolated Left Ventricular Non-compaction in Adults (NCVG)

Public Assistance-Hospitals of Marseille (AP-HM)

Status: Completed

Conditions: Left Ventricular Non Compaction

Treatments: Procedure: Echocardiography; Other: Blood sample; Other: Clinical examination

Study type: Interventional

Funder types: Other

Identifiers

NCT02885363

2011-20

2011-A00987-34 (Other Identifier)

Details and patient eligibility

About

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.

Full 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.

Enrollment: 175 patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

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

Trial design

Primary purpose: Diagnostic

Allocation: Non-Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

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175 participants in 2 patient groups

Patients with newly diagnosed Left Ventricular Non Compaction

Experimental group

Description:

Patient newly diagnosed with Left Ventricular Non Compaction (diagnose \< 6 months), confirmed by echocardiography associated or not with MRI, after centralized review

Treatment:

Other: Clinical examination

Procedure: Echocardiography

Other: Blood sample

Patients with Idiopathic Dilated Cardiomyopathy

Active Comparator group

Description:

Patient newly diagnosed with Idiopathic Dilated Cardiomyopathy (diagnose \< 6 months), confirmed by echocardiography associated or not with MRI, after centralized review

Treatment:

Other: Clinical examination

Procedure: Echocardiography

Other: Blood sample