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Intraventricular dyssynchrony in light chain amyloidosis: a new mechanism of systolic dysfunction assessed by 3-dimensional echocardiography.

ABSTRACT: Light chain amyloidosis (AL) is a rare but often fatal disease due to intractable heart failure. Amyloid deposition leads to diastolic dysfunction and often preserved ejection fraction. We hypothesize that AL is associated with regional systolic dyssynchrony. The aim is to compare left ventricular (LV) regional synchrony in AL subjects versus healthy controls using 16-segment dyssynchrony index measured from 3-dimension-al (3D) echocardiography.Cardiac 3D echocardiography full volumes were acquired in 10 biopsy-proven AL subjects (60 +/- 3 years, 5 females) and 10 healthy controls (52 +/- 1 years, 5 females). The LV was subdivided into 16 segments and the time from end-diastole to the minimal systolic volume for each of the 16 segments was expressed as a percent of the cycle length. The standard deviations of these times provided a 16-segment dyssynchrony index (16-SD%). 16-SD% was compared between healthy and AL subjects.Left ventricular ejection fraction was comparable (control vs. AL: 62.4 +/- 0.6 vs. 58.6 +/- 2.8%, p = NS). 16-SD% was significantly higher in AL versus healthy subjects (5.93 +/- 4.4 vs. 1.67 +/- 0.87%, p = 0.003). 16-SD% correlated with left ventricular mass index (R 0.45, p = 0.04) but not to left ventricular ejection fraction.Light chain amyloidosis is associated with left ventricular regional systolic dyssynchrony. Regional dyssynchrony may be an unrecognized mechanism of heart failure in AL subjects.

SUBMITTER: Migrino RQ

PROVIDER: S-EPMC2525629 | biostudies-literature | 2008 Aug

REPOSITORIES: biostudies-literature

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Intraventricular dyssynchrony in light chain amyloidosis: a new mechanism of systolic dysfunction assessed by 3-dimensional echocardiography.

Migrino Raymond Q RQ Harmann Leanne L Woods Timothy T Bright Megan M Truran Seth S Hari Parameswaran P

Cardiovascular ultrasound 20080807

<h4>Background</h4>Light chain amyloidosis (AL) is a rare but often fatal disease due to intractable heart failure. Amyloid deposition leads to diastolic dysfunction and often preserved ejection fraction. We hypothesize that AL is associated with regional systolic dyssynchrony. The aim is to compare left ventricular (LV) regional synchrony in AL subjects versus healthy controls using 16-segment dyssynchrony index measured from 3-dimension-al (3D) echocardiography.<h4>Methods</h4>Cardiac 3D echoc ...[more]

PMID: 18687125

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Project description:BackgroundLong-term RV pacing leads to ventricular dyssynchrony, in the form of LBBB-like morphology, with subsequent detrimental effects on LV structure and function. Three-dimensional echocardiography allowed early detection of volumetric changes associated with PICMP and provided more accurate assessment of mechanical dyssynchrony. Speckle tracking strain is able to identify LV dysfunction even before any reduction in LVEF. Our aim was to study pacing effects on LV function and hemodynamics using 3D echo and speckle tracking strain.ResultsThis was a prospective study of 175 consecutive patients without structural heart disease (LVEF > 50%) presented for permanent pacing. Full-volume 3D echocardiography done before implantation, 1 week, and 6 months together with GLS. Patients were followed for 6 months to detect incidence of PIVD (defined as reduction in LVEF > 10% but still above 50%) and PICMP (defined as decrease in LVEF by 10% from baseline in absence of other known causes of cardiomyopathy resulting in EF< 50%). PIVD and PICMP predictors and risk factors were analyzed. Only 50 patients met study criteria. Twenty-five (50%) patients developed LV systolic dysfunction; of these, 19 (38%) developed PIVD and 6 (12%) developed PICMP. Pre-implantation GLS was significantly lower in the 6 patients who subsequently developed PICMP, compared to those who developed PIVD and the preserved EF group (mean GLS - 15.50 vs. - 21.0, - 20.0 respectively; p = 0.005, 0.033, respectively). At 1 week, GLS was significantly lower in the 25 patients who subsequently developed PIVD, compared to those who did not (GLS - 13.0 vs. - 18.0, respectively; p = 0.002). A reduction of baseline GLS by 15% or more at 1 week was associated with the development of PIVD and PICMP (p = < 0.001). A wider native QRS complex was associated with PIVD and PICMP (p = 0.008, 0.018, respectively). The other predictors were found non-significant.ConclusionPICMP may be more common than previously reported and it may occur shortly after implantation. Pre-implantation GLS is a sensitive parameter for PICMP. One-week GLS, pre-implantation QRS complex width are early predictors for PICMP and PIVD before any reduction in EF.

Dataset Information

Intraventricular dyssynchrony in light chain amyloidosis: a new mechanism of systolic dysfunction assessed by 3-dimensional echocardiography.

Publications

Intraventricular dyssynchrony in light chain amyloidosis: a new mechanism of systolic dysfunction assessed by 3-dimensional echocardiography.

Similar Datasets

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