Right ventricle and outcome in left ventricular non-compaction cardiomyopathy

Abstract

Background: The risk of adverse events in patients with left ventricular non-compaction cardiomyopathy (LVNC) is substantial. Information on prognostic factors, however, is limited. This study was designed to assess the prognostic value of right ventricular (RV) size and function in LVNC patients. Methods: Cox regression analyses were used to determine the association of indexed RV end-diastolic area (RV-EDAI), indexed end-diastolic diameter (RV-EDDI), fractional area change (FAC), and tricuspid annular systolic excursion (TAPSE) with the occurrence of death or heart transplantation (composite endpoint). Results: Out of 127 patients (53.2 AE 17.8 years; 61% males, median follow-up time was 7.7 years), 17 patients reached the endpoint. In a univariate analysis, RV-EDAI was the strongest predictor of outcome ) per cm 2 /m 2 ; p < 0.0001]. FAC was predictive as well [HR 1.44 (1.16-1.83) per 5% decrease; p = 0.0009], while TAPSE was not (p=ns). RV-EDAI remained an independent predictor in a bivariable analysis with indexed left ventricular ED volume ) per cm 2 /m 2 ; p = 0.0002], while analysis of FAC and left ventricular ejection fraction demonstrated that FAC was not independent [HR 1.20 (0.98-1.52); per 5% decrease; p = 0.0721]. RV-EDAI 11.5 cm 2 /m 2 was the best cut-off value for separating patients in terms of outcome. Patients with RV-EDAI >11.5 cm 2 /m 2 had a survival rate of 18.5% over 12 years as compared to 93.8% in patients with RV-EDAI <11.5 cm 2 /m 2 (p < 0.0001). Conclusion: Increased end-diastolic RV size and decreased systolic RV function are predictors of adverse outcome in patients with LVNC. Patients with RV-EDAI >11.5 cm 2 /m 2 exhibit a significantly lower survival than those <11.5 cm 2 /m 2 .

Right ventricular involvement in left ventricular non-compaction cardiomyopathy

“…RV apical trabecular thickness assessed by CMR correlated with the extent of LV involvement in LVNC, whereas RV end-diastolic (ED) non-compacted to compacted (NC/C) ratio in four--chamber view (4CH) did not differ from normal ventricles [22]. RV systolic function was decreased in patients with advanced LVNC and seemed to be associated with enhanced RV trabeculation and impaired outcome [20][21][22][23].…”

Influence of Right Ventricular Dysfunction on Outcomes of Left Ventricular Non-compaction Cardiomyopathy

“…The increased awareness of LVNC among cardiologists and improved imaging technologies have led to a better understanding of this condition, resulting to it being a widely recognized cardiomyopathy (4). Prospective risk assessment of LNVC is difficult because of the wide variation in its clinical outcomes (5)(6)(7). In addition, only a few studies have evaluated prognostic predictors (8).…”

Uhl’s Anomaly With Left Ventricular Noncompaction

“…Thorough evaluation of LV function and left-sided valvular hemodynamics is crucial before considering conversion to TCPC in patients with Uhl’s anomaly because this strategy transitions the hemodynamics from a 2-ventricle to single-ventricle circulation with its inherent limitations. Additionally, compromised RV function in the setting of LVNC was associated with increased mortality and the need for heart transplantation in biventricular patients ( 10 ). For all of these reasons, heart transplantation was thought to be the most appropriate management option in our patient.…”