Clinical significance of right ventricular dysfunction in left ventricular non-compaction cardiomyopathy

Abstract

Left ventricular non-compaction (LVNC) is described as the persistence of trabeculated myocardium in the left ventricle (LV) and is optimally assessed by cardiac magnetic resonance (CMR). Right ventricular (RV) involvement in LVNC remains poorly studied. Consecutive patients (N = 14) diagnosed with LVNC by CMR were studied. Their clinical data were analyzed. In addition, CMR assessment included quantification of LV and RV volumes, mass, ejection fraction (EF), LV wall motion score, LV non-compacted segments and non-compacted to compacted myocardium ratios. Average age of presentation was 33.1 ± 17.6 years old, with 9 males (64%). Of these patients, 7 (50%) presented with acute heart failure and 3 (21%) with syncope, including 1 documented ventricular tachycardia. RV EF < 35% was identified in 7 (50%) of these patients. Patients with RV EF < 35% presented at a higher median New York Heart Association class (1 [IQR 1-2] vs. 3 [IQR 2-4], P = 0.021) and had significantly lower LV EF (50.7% ± 15.4 vs. 21.8% ± 19.9, P = 0.029), higher LV end diastolic (100.9 ml/m(2) ± 22.3 vs. 159.1 ml/m(2) ± 36.0, P = 0.002) and systolic volume indices (52.0 ml/m(2) ± 25.8 vs. 129.1 ml/m(2) ± 48.4, P = 0.002), higher LV wall motion score index (1.3 ± 0.5 vs. 2.2 ± 0.6, P = 0.004) and higher ratio of LV non-compacted to compacted myocardium (3.3 ± 0.6 vs. 4.1 ± 0.8, P = 0.026). All 4 patients that had ventricular tachycardia also had RV dysfunction. RV dysfunction was present in half of patients with LVNC. Significant RV dysfunction seems to be a marker of advanced LVNC and may carry a worse prognosis. Further studies in a larger sample of patients are needed to confirm those observations.

Right Ventricular Morphology and Systolic Function in Left Ventricular Noncompaction Cardiomyopathy

“…3 Similarly, a small case series of 14 patients evaluated with cardiac MRI have noted severe RV systolic dysfunction among those diagnosed with LVNC. 4 Our study extends these observations and demonstrates increased RV EDV, decreased RV EF, and increased RV apical trabecular thickness among those who meet ES criteria for LVNC (see figure 5 for example). Further, after separating this population into those with LV ES noncompacted:compacted ratio ≥ 2 vs. < 2, those with lower RV EF had a higher incidence of HF and traditional LVNC events.…”

“…3,4 To determine whether RV structure or function is related to potential LVNC, we reviewed 105 cardiac MRI cases where left ventricular trabeculation was noted. We assessed RV size and ejection fraction (EF), as well as ES measures of LVNC.…”

Cardiac magnetic resonance markers of left-ventricular non-compaction in patients with suspicious echocardiographic study

“…Leung et al reported that RV dysfunction was present in half of patients with LVNC. Significant RV dysfunction seemed to be a marker of advanced LVNC [4] . In a study by Nucifora et al LV systolic function was the only variable independently related to RV systolic function, with a correlation coefficient similar to ours [5] .…”

“…The main finding of our study is RV involvement in LVNC, demonstrating that the disease is more likely a biventricular entity and not just an isolated LV disease. There are only a limited number of reports on RV involvement in LVNC in the literature [4][5][6] . Leung et al reported that RV dysfunction was present in half of patients with LVNC.…”

“…Other results of our study are in line with previous research confirming that the disease usually comprises more than five LV segments and that despite similar LV mass in both studied groups the thickness of the compacted layer is lower in patients with LVNC [3,7] . The main limitation of this analysis is its small character, but most other studies on this subject have included similar groups of patients [1][2][3][4][5][6][7] . Furthermore, for practical purposes we decided to use only one CMR definition of LVNC (Petersen's definition) and not the LV trabeculation mass [1,2] .…”