Related Experiment Video
Updated: Jan 19, 2026
Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Cardiac devices in patients with transthyretin amyloidosis: Impact on functional class, left ventricular function,
Eoin Donnellan1, Oussama M Wazni1, Walid I Saliba1
1Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio.
Insights
High right ventricular pacing burden worsens heart failure in transthyretin amyloid cardiomyopathy (ATTR CA) patients. Biventricular pacing improves cardiac function and reduces mortality in these patients.
Area of Science:
- Cardiology
- Electrophysiology
- Cardiomyopathy Research
Background:
- Transthyretin amyloid cardiomyopathy (ATTR CA) is a progressive disease affecting the heart.
- Patients with ATTR CA often require implantable cardiac devices for rhythm management.
- The impact of pacing strategies on cardiac function in ATTR CA is not fully understood.
Purpose of the Study:
- To investigate the outcomes of patients with ATTR CA and implantable devices.
- To assess the effects of right ventricular (RV) pacing burden and biventricular (BiV) pacing on left ventricular ejection fraction (LVEF), mitral regurgitation (MR), New York Heart Association (NYHA) functional class, and mortality.
Main Methods:
- Retrospective observational cohort study of 78 patients with ATTR CA and implantable devices.
- Mean follow-up of 42 months.
- Analysis of RV pacing burden and BiV pacing impact on LVEF, MR severity, NYHA class, and mortality.
Main Results:
- Higher RV pacing burden (>40%) was associated with worsening MR (62%), LVEF (89%), and NYHA class (89%) compared to lower RV pacing (<40%).
- Biventricular (BiV) pacing led to improvements in LVEF (78%), NYHA class (67%), and MR severity (67%).
- Mortality was high across groups but lowest in the cardiac resynchronization therapy (CRT) group (67%) compared to RV pacing >40% (92%).
Conclusions:
- Increased RV pacing burden is linked to adverse cardiac remodeling and heart failure in ATTR CA.
- BiV pacing demonstrates significant benefits for cardiac function and clinical status in ATTR CA patients.
- BiV pacing should be strongly considered for ATTR CA patients requiring pacing, pending further prospective research.
Background:
The aim of our study was to investigate outcomes of patients with ATTR (amyloidosis and transthyretin) CA (cardiac amyloidosis) and implantable devices with respect to left ventricular ejection fraction (LVEF), mitral regurgitation (MR), New York Heart Association (NYHA) functional class, and mortality.
Methods:
This was a retrospective observational cohort study of 78 patients with ATTR CA and implantable devices. During a mean follow-up of 42 months we investigated the impact of right ventricular (RV) pacing burden and biventricular (BiV) pacing on LVEF, MR severity, NYHA functional class, and mortality.
Results:
Worsening MR occurred in 11% of patients with a RV pacing % <40% compared to 62% of those with a RV pacing burden >40% (P = .002). Similarly, worsening LVEF occurred in 26% of patients who were RV paced <40% and 89% of those who were RV paced >40% of the time (P < .0001) and worsening in NYHA functional class occurred in 22% and 89%, respectively (P < .0001). Improvement in LVEF, NYHA functional class, and MR severity occurred in 78%, 67%, and 67%, respectively, in those with BiV devices. Death occurred in 67% of patients in the cardiac resynchronization therapy group compared to 68% of those with a RV pacing burden <40% and 92% of those with a RV pacing burden >40%.
Conclusion:
A higher RV pacing burden is associated with deleterious remodeling and congestive heart failure in patients with ATTR CA, whereas BiV pacing is associated with improvements in LVEF, NYHA class, and degree of MR. BiV pacing should be considered in patients with ATTR CA and an indication for pacing. However, further larger prospective studies will need to be performed.
More Related Videos
Related Concept Videos
06:33Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
08:43Chronic Ovine Model of Right Ventricular Failure and Functional Tricuspid Regurgitation
07:42An Image Guided Transapical Mitral Valve Leaflet Puncture Model of Controlled Volume Overload from Mitral Regurgitation in the Rat
06:34Evaluation of Left Ventricular Structure and Function using 3D Echocardiography
07:55Echocardiographic Characterization of Left Ventricular Structure, Function, and Coronary Flow in Neonate Mice
08:09Echocardiographic Assessment of Cardiac Anatomy and Function in Adult Rats