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Right ventricle and outcome in left ventricular non-compaction cardiomyopathy


Stämpfli, Simon F; Donati, Thierry G; Hellermann, Jens; Anwer, Shehab; Erhart, Ladina; Gruner, Christiane; Kaufmann, Beat A; Gencer, Baris; Haager, Philipp K; Müller, Hajo; Tanner, Felix C (2020). Right ventricle and outcome in left ventricular non-compaction cardiomyopathy. Journal of Cardiology, 75(1):20-26.

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 ± 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 [HR 1.48 (1.24-1.77) 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 [HR 1.41 (1.18-1.70) 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$^{2}$.

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 ± 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 [HR 1.48 (1.24-1.77) 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 [HR 1.41 (1.18-1.70) 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$^{2}$.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Cardiology
Dewey Decimal Classification:610 Medicine & health
Scopus Subject Areas:Health Sciences > Cardiology and Cardiovascular Medicine
Language:German
Date:January 2020
Deposited On:18 Feb 2020 09:22
Last Modified:29 Jul 2020 14:30
Publisher:Elsevier
ISSN:0914-5087
OA Status:Closed
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1016/j.jjcc.2019.09.003
PubMed ID:31587941

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