Left Ventricle Non-compaction Cardiomyopathy
Conditions
Brief summary
Objective: To evaluate clinical characteristics, cardiac magnetic resonance imaging features, and outcomes of patients with left ventricle non-compaction.
Detailed description
Left Ventricle Non-Compaction Cardiomyopathy (LVNCC) is a type of myocardial disease characterized by prominent myocardial trabeculae and recesses resulting in two distinct layers of myocardium: compacted layer and the non-compacted layer. It arises due to the failure of left ventricle (LV) maturation and compaction during intra-uterine life (1). As per the position statement from the European Society of Cardiology, LVNCC has been labelled as 'unclassified' type of cardiomyopathy. Clinical course can be complicated by heart failure, thromboembolism, or arrhythmia. Phenotypic presentation of LVNC can range from extremely thickened layer of non-compacted myocardium to mere presence of prominent trabeculae and recesses albeit a compacted myocardium. Transthoracic echocardiography is the first tool to diagnose LVNC. However, Cardiac Magnetic Resonance (CMR) imaging has emerged as a strong tool to differentiate between LVNCC from mere hyper-trabeculated LV myocardium when echocardiogram is inconclusive (Figure 1 and Video 1). Various criteria have evolved to diagnose LVNC by CMR. Peterson et al defined the end-diastole non-compacted to compacted myocardium ratio of \>2.3 to have good sensitivity, specificity, and negative predictive value for differentiating pathological non-compaction from hyper-trabeculation. With an increase in the use of diagnostic cardiovascular imaging modalities in South-Asian (SA) belt, LVNC is being increasingly diagnosed. The exact prevalence of LVNC in SA is not known and phenotypic CMR characteristics, clinical features, and outcomes of LVNC remain unknown for SA population. This brought us to the need of analysing CMR data of LVNC at our centre.
Interventions
OTHERPatients were retrospectively enrolled. This was a retrospective study and no intervention was performed.
Patients were retrospectively enrolled. This was a retrospective study and no intervention was performed.
Sponsors
Aga Khan University Hospital, Pakistan
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Patients \> 18 years, referred for CMR for further evaluation of cardiomyopathy.
Exclusion criteria
* patients with intracardiac masses, pericardial diseases and congenital heart diseases were excluded from the study.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| To evaluate clinical characteristics, cardiac magnetic resonance imaging features, and outcomes of patients with left ventricle non-compaction. | 37 months | Evaluating CMR features and looking at outcomes such as all cause mortality, major heart failure or arrhythmia hospitalizations. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| All cause-mortality, heart failure hospitalization, and arrhythmia hospitalization. | 37 months | All cause-mortality, heart failure hospitalization, and arrhythmia hospitalization. |
Outcome results
None listed