Prognosis of Isolated Left Ventricular Non-compaction in Adults

Not Applicable

Completed

• Conditions: Left Ventricular Non Compaction
• Interventions: Other: Clinical examination; Other: Blood sample; Procedure: Echocardiography

• Registration Number: NCT02885363
• Lead Sponsor: Assistance Publique Hopitaux De Marseille

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.

Study Timeline

• Study Start: 2011-12-21
• Primary Completion: 2015-10-23
• Study First Submitted: 2016-08-17
• Study First Submitted QC: 2016-08-30
• Study First Posted: 2016-08-31
• Status Verified: 2023-04
• Study Completion: 2023-04-12
• Last Update Submitted: 2023-04-12
• Last Update Posted: 2023-04-14

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 175

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 & Design

• Study Type: INTERVENTIONAL
• Study Design: PARALLEL

Arm && Interventions

Group	Intervention	Description
Patients with Idiopathic Dilated Cardiomyopathy	Clinical examination	Patient newly diagnosed with Idiopathic Dilated Cardiomyopathy (diagnose \< 6 months), confirmed by echocardiography associated or not with MRI, after centralized review
Patients with newly diagnosed Left Ventricular Non Compaction	Echocardiography	Patient newly diagnosed with Left Ventricular Non Compaction (diagnose \< 6 months), confirmed by echocardiography associated or not with MRI, after centralized review
Patients with newly diagnosed Left Ventricular Non Compaction	Blood sample	Patient newly diagnosed with Left Ventricular Non Compaction (diagnose \< 6 months), confirmed by echocardiography associated or not with MRI, after centralized review
Patients with newly diagnosed Left Ventricular Non Compaction	Clinical examination	Patient newly diagnosed with Left Ventricular Non Compaction (diagnose \< 6 months), confirmed by echocardiography associated or not with MRI, after centralized review
Patients with Idiopathic Dilated Cardiomyopathy	Blood sample	Patient newly diagnosed with Idiopathic Dilated Cardiomyopathy (diagnose \< 6 months), confirmed by echocardiography associated or not with MRI, after centralized review
Patients with Idiopathic Dilated Cardiomyopathy	Echocardiography	Patient newly diagnosed with Idiopathic Dilated Cardiomyopathy (diagnose \< 6 months), confirmed by echocardiography associated or not with MRI, after centralized review

Primary Outcome Measures

Name	Time	Method
Occurrence of death of cardiac origin	Up to 2 years
Occurrence of hospitalization due to cardiac event	Up to 2 years
Occurrence of a cardiac transplantation	Up to 2 years

Trial Locations

Locations (1)

Assistance Publique Hôpitaux de Marseille, Hôpital de la Timone; 🇫🇷 Marseille, France