Clinical trial NCT02885363: Prognosis of Isolated Left Ventricular Non-compaction in Adults
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: NA
Conditions: Left Ventricular Non Compaction
Lead sponsor: Assistance Publique Hopitaux De Marseille
Sponsor class: OTHER
First posted: 2016-08-31
Enrollment: 175
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NON_RANDOMIZED
Intervention model: PARALLEL
Primary purpose: DIAGNOSTIC

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.

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

Primary outcomes:
measure: Occurrence of death of cardiac origin
timeFrame: Up to 2 years
measure: Occurrence of a cardiac transplantation
timeFrame: Up to 2 years
measure: Occurrence of hospitalization due to cardiac event
timeFrame: Up to 2 years
Secondary outcomes:
Other outcomes:
Locations:
city: Marseille
country: France
facility: Assistance Publique Hôpitaux de Marseille, Hôpital de la Timone
Study officials:
affiliation: Assistance Publique Hôpitaux de Marseille
name: Catherine GEINDRE
role: STUDY_DIRECTOR
References: