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

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.

Study Overview

• Status: Completed
• Conditions: Left Ventricular Non Compaction
• Intervention / Treatment: Other: Clinical examination; Procedure: Echocardiography; Other: Blood sample

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.

• Study Type: Interventional
• Enrollment (Actual): 175
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Gilbert HABIB, Pr; Phone Number: +33 04 91 38 75 87; Email: gilbert.habib@free.fr
• Study Contact Backup: Name: Nathalie CHARVIN; Phone Number: +33 04 91 38 17 05; Email: nathalie.charvin@ap-hm.fr

Study Locations

• France
  • Marseille, France, 13005
    • Assistance Publique Hôpitaux de Marseille, Hôpital de la Timone

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

Description

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

Study Plan

How is the study designed?

Design Details

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

Number of Arms

2

Arms and Interventions

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

What is the study measuring?

Primary Outcome Measures

Outcome Measure	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

Collaborators and Investigators

• Sponsor: Assistance Publique Hopitaux De Marseille

Study record dates

Study Major Dates

• Study Start (Actual): December 21, 2011
• Primary Completion (Actual): October 23, 2015
• Study Completion (Actual): April 12, 2023

Study Registration Dates

• First Submitted: August 17, 2016
• First Submitted That Met QC Criteria: August 30, 2016
• First Posted (Estimate): August 31, 2016

Study Record Updates

• Last Update Posted (Actual): April 14, 2023
• Last Update Submitted That Met QC Criteria: April 12, 2023
• Last Verified: April 1, 2023

More Information

Terms related to this study

• Other Study ID Numbers: 2011-20; 2011-A00987-34 (Other Identifier: ANSM)

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED