Mitral valve prolapse - it"s possible to stratify prognosis in these patients?

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Mitral valve prolapse (MVP) is one of the most frequent causes of mitral valve disease in developed countries, traditionally with a benign prognosis, however some patients develop arrythmias and significant mitral regurgitation (MR) with need of intervention. Herein our purpose was to establish clinical, electrocardiographic and echocardiographic predictors of arrythmias, mitral valve intervention (MVI) and hospitalization in MVP patients to better characterize the prognosis in these patients. Methods Single-center retrospective study of consecutive patients with MVP documented in transthoracic echocardiogram between January 2014 and October 2019. MVP was defined as systolic displacement of the mitral leaflet into the left atrium ≥ 2 mm from the mitral annular plane. Demographic, clinical, echocardiographic, electrocardiographic data were collected as well as adverse events at follow-up. The results were obtained using Chi-square and Student-t tests; predictors were found with logistic regression. Results 247 patients were included (mean age 62.9 ± 18 years, 61% males), most with MVP involving the posterior leaflet (48.6%). 40% were symptomatic, 47.4% had more than moderate MR, and 25% had interventricular conduction delay in the ECG. During a mean follow-up of 30 ± 19 months, 38% had arrythmias, 27.1% needed mitral valve intervention (95% surgery and 5% percutaneous), 27.1% had atrial fibrillation (AF), 3.4% had ventricular arrythmias, 19.2% had ventricular premature beats, 13.3% had hospital admission for cardiovascular cause and 8.5% (n = 21) died. 9.3% of the patients had mitral annulus disjunction (MAD). Palpitations (p = 0.018), AF (p < 0.001), significant MR (p < 0.001), higher NYHA class (p = 0.016), systolic pulmonary artery pressure (SPAP) (p < 0.001), LV mass (p < 0.001), QTc (p = 0.01) and MAD maximum distance (p = 0.02) associated with MVI. MAD maximum distance value presented an excellent capacity to predict the MVI (AUC 0.85 p = 0.019); the best cut-off was 11,5 mm (Sens = 80%; Spec = 83%). AF was a predictor of hospitalization in univariate analysis (OR = 2.57, CI95% 1.15-5.75, p = 0.022). Regarding arrhythmic events, we found association with aortic root dilatation (p = 0.032), NYHA III-IV (p = 0.013), age and LV mass (both with p < 0.001). In multivariate analysis, LV mass (OR = 1.02, CI95% 1.005-1.027, p = 0.005) and age (OR = 1.038, CI95% 1.004-1.053, p = 0.021) were independent predictors of arrythmias. In this sample, MAD was not associated with arrythmias. Conclusion Opposing to previous studies in our population, MAD was not associated with arrythmias but had an excellent capacity to predict MVI. Age and LV hypertrophy were independent predictors of arrythmias in our patients. Larger studies are needed to better stratify patients with MVP, as its association with arrhythmias, hospitalization and the need for intervention is not negligible.

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P302 Forgotten and neglected: LV hypertrophy and LV remodeling as important predictors in patients with mitral valve prolapse

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.155 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

D Trifunovic ◽

O Petrovic ◽

M Tomic-Dragovic ◽

I Paunovic ◽

V Tutus ◽

...

Keyword(s):

Heart Failure ◽

Mitral Valve ◽

Mitral Valve Prolapse ◽

Left Ventricular ◽

Secondary Outcome ◽

Multivariable Model ◽

Chi Square ◽

Increased Risk ◽

Lv Mass

Abstract Current ESC guidelines recommends left ventricular (LV) end-systolic diameter (ESD), LV ejection fraction (LV EF), systolic pulmonary arterial pressure (SPAP) as key parameters in a multifactorial treatment algorithm for chronic severe primary MR. However, LV hypertrophy (LVH) and LV remodelling during the process of adaptation to chronic MR can influence further clinical course. Aim of this study was to test whether LVH and distinctive LV geometry are coupled with increased risk for heart failure (HF) development and occurrence of major adverse cardiac event (MACE) among patients with MVP and can they improve power of statistical models for HF and MACE prediction based on parameters supported by the current guidelines. Methods 376 pts diagnosed with mitral valve prolapse (MVP) between 1. January 2014. and 31. December 2017 and with complete medical chart and follow-up data from central echo laboratory in the tertiary health center were enrolled in the study. Four types of LV geometry were identified: Type 1 (normal LV mass with normal geometry), Type 2 (normal LV mass with concentric remodeling), Type 3 (eccentric hypertrophy) and Type 4 (concentric hypertrophy). The primary outcome was HF and secondary outcome was MACE (HF development, myocardial infarction, myocardial revascularisation (both PCI and/or ACBG) and cardiac death). Results The distribution of patients was as follow: 51.2% (Group 1) vs 3.3% (Group 2) vs 41.4 % (Group 3) vs 4.1% (Group 4). In multivariable model the highest OR for HF development after adjustment for age, ESD and LVH, had concentric LVH (OR= 5.361, p= 0.004, 95% CI 1.696-16.648), then EF < 60% (OR= 3.025, p = 0.004, 95% CI 1.427-6.411) and the lowest OR had SPAP > 40 mmHg (OR = 2.274, p = 0.039, 95% 1.43-4.958). Adding LVH significantly increased model’s power to predict HF above traditional parameters (Chi-square from 19.386 to 23.640, p < 0.001; Nagelkerke R square from 0.090 to 0.110), whereas addition of LV geometry increased it even more (Chi-square from 23.640 to 28.729, p < 0.001; Negelkerke R square from 0.110 to 0.132). Independent MACE predictors in multivariable model were: EF < 60% (OR 3.645, p < 0.001, 95% CI 1.808- 7.50), new onset atrial fibrillation during the follow-up (OR =3.327, p = 0.012, 95% CI 0.305-8.484), concentric LVH (OR= 4.241, p = 0.015, 95% CI 1.327-13.550) and normal LV geometry without LVH (OR= 0.514, p = 0.002, 95% CI 0.288-0.918), even after adjustment for MV surgery. Adding LVH significantly improved model’s power (Chi-square from 29.026 to 35.112, p < 0.001; Nagelkerke R square 0.121 to 0.146) to predict MACE and addition of type of LV geometry provided additional strength (Chi-square from 35.112 to 39.707, p < 0.001; Nagelkerke R square from 0.146 to 0.164). Conclusion LVH and especially concentric LVH are independent predictors of heart failure development and MACE in mitral valve prolapse and significantly improves predictive powers of the models based on traditional parameters.

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Influence of Atrial Fibrillation on Outcome Following Mitral Valve Repair

Circulation ◽

10.1161/circ.104.suppl_1.i-59 ◽

2001 ◽

Vol 104 (suppl_1) ◽

Author(s):

Eric Lim ◽

Clifford W. Barlow ◽

A. Reza Hosseinpour ◽

Christopher Wisbey ◽

Kate Wilson ◽

...

Keyword(s):

Atrial Fibrillation ◽

Mitral Valve ◽

Sinus Rhythm ◽

Ventricular Function ◽

Mitral Valve Repair ◽

Nyha Class ◽

Univariate Analysis ◽

Left Ventricular ◽

Valve Repair

Background To investigate the outcome of patients in atrial fibrillation (AF) following mitral valve repair, clinical and echocardiographic follow-up was undertaken in 400 consecutive patients who underwent mitral valvuloplasty from 1987 to 1999. Methods and Results The main indications for surgery were degenerative (81.4%), endocarditis (7.1%), rheumatic (6.6%), ischemic (4.6%), and traumatic (0.3%) mitral valve disease. After excluding 6 paced patients and 1 patient in nodal rhythm, we compared the outcomes of 152 patients in AF against 241 patients in sinus rhythm. For patients in AF versus those in sinus rhythm, more AF patients were older (mean age 67.2±8.8 versus 61.9±11.8 years, respectively; P <0.001), more were assigned to a poorer New York Heart Association (NYHA) class (77.6% versus 66.0% in NYHA III/IV, respectively; P =0.01), and more demonstrated impaired ventricular function (78.9% versus 46.2% with moderate or severe impairment, respectively; P <0.001). For patients in AF versus those in sinus rhythm, there was no difference in 30-day mortality (2.0% versus 2.1%, respectively; P =0.95), repair failure (5.4% versus 3.6%, respectively; P =0.41), stroke (5.4% versus 2.2%, respectively; P =0.11), or endocarditis (2.3% versus 0.9%, respectively; P =0.27) on follow-up at a median of 2.8 years (interquartile range 1.1 to 6.0). On echocardiography, the proportion of patients with mild regurgitation or worse was 13.3% (AF patients) versus 10.8% (patients in sinus rhythm) ( P =0.70). Patients in AF versus those in sinus rhythm had lower survival at 3 years (83% versus 93%, respectively) and 5 years (73% versus 88%, respectively). Univariate analysis identified factors affecting survival as AF ( P =0.002), age >70 years ( P =0.041), and poor ventricular function ( P <0.001). However, by use of a multivariate model, only poor ventricular function remained significant ( P =0.01). Conclusions AF does not affect early outcome or durability of mitral repair. The onset of AF may be indicative of disease progression because of its association with poor left ventricular function.

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Mitral valve prolapse: American versus European guidelines - which one is better

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa356.072 ◽

2021 ◽

Vol 22 (Supplement_1) ◽

Author(s):

J Brito ◽

J Rigueira ◽

T Rodrigues ◽

I Aguiar-Ricardo ◽

R Santos ◽

...

Keyword(s):

Mitral Valve ◽

Mitral Valve Prolapse ◽

Clinical Presentation ◽

Nyha Class ◽

Daily Practice ◽

Posterior Wall ◽

Systolic Pulmonary Artery Pressure ◽

Chi Square ◽

Funding Sources ◽

European Guidelines

Abstract Funding Acknowledgements Type of funding sources: None. Introduction According to the most recent recommendations of AHA, mitral valve prolapse (MVP) is defined as systolic displacement of the mitral leaflet into the left atrium (LA) of at least 2 mm from the mitral annular plane. The ESC recommendations define MVP, flail and billowing, according to the location of the leaflet tips in relation to the coaptation plan. Differences in outcomes considering these classifications are not established. Purpose To evaluate the differences in clinical presentation and outcomes of MVP considering AHA and ESC classifications. Methods Single-center retrospective study of consecutive patients with MVP (defined according to the AHA classification) documented in transthoracic echocardiogram between January 2014 and October 2019. Demographic, clinical, echocardiographic and electrocardiographic data were collected. The results were obtained using Chi-square and ANOVA tests. Results We included 247 patients (mean age 62.9 ± 18 years, 61% males) according to AHA classification; considering the ESC classification: 147 (59%) had prolapse, 30 (12%) flail and 67 (39%) billowing. In comparison to patients with flail and billowing, patients with MVP had less cordae rupture (p = 0.02). Prolapse was associated with better survival (p = 0.037) and was an independent predictor of survival (OR = 0.372, CI95% [0.148-0.935], p = 0.035) Patients with flail were older in comparison to the ones with prolapse and billowing (71 ± 14 vs 63 ± 17 vs 60 ± 21 years, respectively, p = 0.022). Patients with flail were mostly men (80%, p = 0.028), with more significant mitral regurgitation (p = 0.003) and higher NYHA class (p = 0.018). They also had higher systolic pulmonary artery pressure (SPAP) (48 ± 23 vs 38 ± 18 vs 36 ± 12mmHg, p = 0.015) and higher values of LV mass and posterior wall thickness (144 ±32 vs 125 ± 44 vs 114 ± 37g/m2, p = 0.005 and 11 ± 1,5 vs 10 ± 1,7 vs 9 ± 1.9 mm, p = 0.009, respectively). Women had more billowing (p = 0.04) than prolapse and flail. Conclusion The ESC classification adds information to the AHA classification in what concerns to clinical presentation and prognosis of mitral valve prolapse, so both classifications should be used in daily practice.

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Features of degenerative mitral valve prolapse in the North East of China: repair characteristics, and short-term follow-up results

Minerva Cardioangiologica ◽

10.23736/s0026-4725.20.05248-2 ◽

2020 ◽

Vol 68 (5) ◽

Author(s):

Silvia Corona ◽

Paolo Barbier ◽

Guangyu Liu ◽

Osafo A. Annoh ◽

Marcio Scorsin ◽

...

Keyword(s):

Mitral Valve ◽

Mitral Valve Prolapse ◽

Short Term ◽

North East ◽

The North

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Favorable course of tricuspid regurgitation after percutaneous mitral valve repair is associated with improved survival and clinical outcome

European Heart Journal ◽

10.1093/ehjci/ehaa946.1945 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

F Meijerink ◽

J Baan ◽

B.J Bouma

Keyword(s):

Mitral Valve ◽

Clinical Outcome ◽

Mitral Valve Repair ◽

Tricuspid Regurgitation ◽

Nyha Class ◽

Funding Source ◽

Valve Repair ◽

Percutaneous Mitral Valve Repair ◽

All Cause Mortality

Abstract Background Tricuspid Regurgitation (TR) is often present in patients with mitral regurgitation (MR) and is associated with increased mortality and morbidity after percutaneous mitral valve repair (PMVR) using the MitraClip (Abbott Vascular). It is unclear to what extent TR is reduced after PMVR and whether the reduction of TR is related to survival and functional outcome. Purpose The aim of this study was to determine (1) the TR course after PMVR and (2) if this was related to survival and clinical outcome. Methods Patients who underwent PMVR and had complete echocardiographic data at baseline and follow-up were included. TR severity was graded as none, mild, moderate or severe (according to current guidelines) and was determined before treatment and at 6-months of follow up. Favorable TR course was defined as improvement of ≥1 grade or ≤ mild TR at 6-months. Clinical endpoints were all-cause mortality during 1-year of follow-up and improvement in New York Heart Association (NYHA) functional class after 6 months. Results A total of 67 patients were included (mean age 76 years, 57% male, 81% NYHA class ≥3 and 69% baseline TR ≥ moderate). Favorable TR course was achieved in 31 patients (46%) (figure 1A). All-cause mortality at 1 year was 7.5%, and was lower in the favorable TR course group (0% vs. 13.9%, p=0.057) (figure 1B). Improvement in NYHA class at 6-months was seen in 45% of patients without vs. 81% of patients with favorable TR course (p=0.01) (figure 1C). Conclusion A favorable TR course is achieved in 46% of PMVR patients and is associated with improved survival and improvement of NYHA class. The relatively high rate of an unfavorable TR course at 6-months, indicates that interventional treatment of the tricuspid valve might benefit these patients. TR course (A) and NYHA improvement (B) Funding Acknowledgement Type of funding source: Other. Main funding source(s): Abbott

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Valvular Heart Disease - Part II

10.2310/fm.1712 ◽

2021 ◽

Author(s):

Miriam S. Jacob ◽

Brian P Griffin

Keyword(s):

Heart Disease ◽

Mitral Valve ◽

Mitral Regurgitation ◽

Aortic Stenosis ◽

Aortic Regurgitation ◽

Valvular Heart Disease ◽

Mitral Valve Prolapse ◽

Developed Countries ◽

Cardiac Morbidity ◽

Mechanical Prostheses

Valvular heart disease is an important cause of cardiac morbidity in developed countries despite a decline in the prevalence of rheumatic disease in those countries. This chapter discusses the many etiologies of valvular heart disease and presents methods for assessment and management. Specific valvular lesions discussed include mitral stenosis, mitral regurgitation, mitral valve prolapse, aortic stenosis, aortic regurgitation, and tricuspid and pulmonary disease. The section on tricuspid disease includes a discussion of mechanical prostheses (ball-in-cage and tilting-disk) and biologic prostheses (xenografts, allografts, and autografts) and their complications. This review contains 5 figures, 9 tables, and 53 references. Keywords: Valvular heart disease, stenosis, regurgitation, mitral regurgitation, mitral valve prolapse (MVP), aortic stenosis, congenital bicuspid valve, senile valvular calcification, aortic regurgitation, chordae or papillary muscles

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Abstract 2068: Systematic Undersized Rigid Ring Decreases Recurrence Rate Following Repair of Ischemic Mitral Valve

Circulation ◽

10.1161/circ.116.suppl_16.ii_448-a ◽

2007 ◽

Vol 116 (suppl_16) ◽

Author(s):

Raphaël Fontaine ◽

Denis Bouchard ◽

Philippe Demers ◽

Raymond Cartier ◽

Michel Carrier ◽

...

Keyword(s):

Mitral Valve ◽

Mitral Regurgitation ◽

Recurrence Rate ◽

Nyha Class ◽

High Recurrence Rate ◽

Class Iii ◽

Term Survival ◽

Rigid Ring

Introduction: Chronic ischemic mitral regurgitation (MR) has been associated with poor long-term survival. Suboptimal midterm results have been a growing concern in the surgical community. In recent years, our approach to repair those valves has evolved to a standardized technique using complete, rigid and small annuloplasty rings. This study aims to compare this systematic approach with our prior experience from 1996 –2001 where recurrent MR rate was high. Methods: 129 patients underwent repair for pure ischemic mitral valve regurgitation between 2002 and 2005 at our institution. Of these patients, 99 had clinical and echographic follow-up. These patients were compared to the 1996 –2001 cohort of 73 patients. Results: Preoperatively, 84% of patients were in NYHA class III or IV, 17% had moderate MR, 83% had moderate-severe to severe MR. Sixteen were redo operations, mostly of previous CABG. All patients except one were treated with a complete rigid ring (Annuloflo 46.5%, Physioring 34.9%, Etlogix 13.9%, others 3.8%). Ring size was: 24 (0.8%); 26 (55.8%); 28 (38%); or 30 (4.5%). Mortality was 8.5% at 30 days, 14.7% at 1 year and 17.8% at 2 years. Immediate postoperative regurgitation was absent or trace in all patients. Freedom from reoperation was 97%. Mean postoperative NYHA class was 1.15 at a mean follow-up of 28 months. Recurrent moderate mitral regurgitation (2+) was 15.34%, severe mitral regurgitation (3+ to 4+) was 13.4% at a mean follow-up of 16 months. In the 73 patients from the period 1996 –2001 at the same echo follow-up time, the moderate and severe recurrence were: 37% and 21%. The decrease in the recurrence rate was highly significant (p=0.001). Conclusion: A more standardized approach to ischemic mitral valve repair has improved the high recurrence rate previously reported by our group. Long-term follow-up is necessary to confirm these findings.

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Risk stratification and prognostic value of CMR in DCM; parametric mapping and GLS- value beyond EF and LGE?

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa356.315 ◽

2021 ◽

Vol 22 (Supplement_1) ◽

Author(s):

P Brown ◽

A Dimarco ◽

J Bradley ◽

G Nucifora ◽

C Miller ◽

...

Keyword(s):

Risk Stratification ◽

Prognostic Value ◽

Nyha Class ◽

Univariate Analysis ◽

Multivariable Analysis ◽

Youden Index ◽

Private Company ◽

Parametric Mapping ◽

Selection For

Abstract Funding Acknowledgements Type of funding sources: Private company. Main funding source(s): Dr Pamela Brown was suppoerted by funding from Alliance Medical. Background; Arrhythmia risk stratification and device implantation in dilated cardiomyopathy (DCM) poses significant challenges and as demonstrated by the DANISH trial appears to have reached the asymptote of clinical efficacy. A body of evidence now demonstrates that risk stratification of and device selection for DCM patients may be enhanced by inclusion of patients" LGE-status. Furthermore, it has been suggested that CMR based parametric mapping and strain analysis may further advance risk stratification. Methods; 703 patients with DCM undergoing clinically indicated CMR scans and prospectively enrolled into the UHSM-CMR study (NCT02326324) between 03/2015-12/2018 were analysed. Multivariable Cox proportional hazard models and Youden index driven C-statistics were used to assess additive prognostic value of GLS, T1 and ECV mapping on the combined endpoint of cardiovascular death, cardiac transplantation, LVAD insertion or hospitalisation for heart failure in models incorporating NHYA class, EF and LGE status. Additionally. the value of GLS, T1, and ECV on predicting significant arrhythmic events (SAV) (ventricular arrhythmia (VA), resuscitated cardiac arrest (rCA) or sudden cardiac death (SCD)) was assessed. Results; Patients (mean age 59, 66% male, 60% ≥NYHA II, mean EF 42%, mean GLS -12%, mean ECV 27%) were on good medical therapy (beta blocker 74%%, ACE 79%, MRA 38%, Entresto 5%, CRT 23%). Mean follow-up was 21 months; the combined endpoint occurred in 34 patients (5%). On univariate analysis NYHA class (HR 2.44 (1.67-3.57), p < 0.001), ECV (HR 1.14 (1.05-1.22), p < 0.001), GLS% (HR 1.14 (1.07-1.21) p < 0.001,) T1 (HR 1.06 (1.005-1.1), p = 0.03), RVEF (HR 0.95 (0.93-0.98), p < 0.001), LVEF (HR 0.92 (0.9-0.95), p < 0.001) were all significantly associated with outcome. On multivariate analysis only EF and NYHA class was associated with outcome. SAV occurred as the first manifestation of disease or during follow up in 27 patients (4%). At univariate analysis LGE, ECV, GLS, EF and NYHA class were all associated with SAV. However, on multivariable analysis only EF, LGE and ECV (HR 1.11 (1.01-1.22), p = 0.03) but not GLS remained independently predictive in a model already incorporating EF, NYHA and LGE. Conclusion Optimally treated DCM populations have very low event rates. CMR based assessment of fibrosis status/burden with both LGE and ECV assessment has the potential to enhance patient selection for ICD therapy. Whilst GLS is increasingly recognised as a sensitive imaging biomarker of early disease detection it provides no additive value, likely because of it’s high co-linearity with EF, in models already containing EF, NYHA class and LGE status.

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Mitral Valve Prolapse and Athletic Participation in Children and Adolescents

PEDIATRICS ◽

10.1542/peds.95.5.789 ◽

1995 ◽

Vol 95 (5) ◽

pp. 789-790

Author(s):

Keyword(s):

Mitral Valve ◽

Children And Adolescents ◽

Mitral Valve Prolapse ◽

Holter Monitoring ◽

Premature Ventricular Contractions ◽

Benign Condition ◽

Migraine Headaches ◽

Asymptomatic Patients ◽

Vascular Accidents

Mitral valve prolapse (MVP) is generally a benign condition characterized by the protrusion of the mitral valve leaflets into the left atrium during systole. The prevalence of MVP in individuals under the age of 18 years is estimated to be 5% but is higher in those with Marfan's syndrome and other collagen vascular disorders.1 A midsystolic nonejection click with or without a late systolic murmur is the auscultatory hallmark of this syndrome. The diagnosis of MVP in children and adolescents should be based primarily on auscultatory findings and not on minor echocardiographic findings.1 The prognosis in children and adolescents with isolated MVP appears to be excellent and complications are rare. In 553 children, aged 15 days to 18 years, who were involved in studies with a follow-up period of 6 to 9 years, the following were reported: subacute bacterial endocarditis (one case), cerebral vascular accidents (two cases), migraine headaches (four cases), and chest pain (12 cases).2,3 Only four cases of sudden death have been reported in patients younger than 20 years of age.1-4 In a study of 103 patients with MVP, 16% were found to have premature ventricular beats during exercise electrocardiography (ECG) (exercise test).3 Thirty-eight percent were found to have premature ventricular contractions (PVCs) on 24-hour ECG (Holter) monitoring. This study, however, does not report the true prevalence of dysrhythmias because all these subjects had been referred to a pediatric cardiologist for evaluation. It is likely that these reported numbers are high because asymptomatic patients are less often referred.

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Abstract 4355: Functional Status Is More Strongly Associated with Mortality in Cardiac Amyloid Compared to Echocardiographic Parameters

Circulation ◽

10.1161/circ.118.suppl_18.s_871-c ◽

2008 ◽

Vol 118 (suppl_18) ◽

Author(s):

Bethany A Austin ◽

Brendan Duffy ◽

Rene Rodriguez ◽

Carmela Tan ◽

Randall C Starling ◽

...

Keyword(s):

Survival Analysis ◽

Cardiac Amyloidosis ◽

Nyha Class ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Proportional Hazard ◽

Term Survival ◽

Cox Proportional Hazard ◽

Lv Mass

Cardiac amyloidosis (CA) is generally associated with a poor prognosis and significantly increased mortality. We sought to identify predictors of longer-term survival in patients with EMB documented CA. Forty-five consecutive EMB documented CA patients were studied from 1/98 –12/03. Age, gender, NYHA class, medications, presence of light chain amyloid and ECG voltage were recorded. Baseline left ventricular ejection fraction (LVEF), deceleration time (DT), diastology, LV mass, interventricular septal (IVS) thickness and myocardial performance index (MPI) [(isovolumic contraction time + isovolumic relaxation time)/ejection time] were recorded. Length of follow-up and all-cause mortality were recorded. Mean age was 66 ±10 years with 34 (76 %) men. NYHA class > 2 and low voltage on ECG [S (V1) + R (V5) ≤12] were noted in 26 (58 %) and 12 (27 %) patients, respectively. Mean LVEF, IVS thickness and LV mass were 46 % ±13, 1.7 cm ±0.42 and 303 grams ±114, respectively. DT <150 msec and MPI > 0.6 were found in 19 (42 %) and 15 (33 %) patients, respectively. At median follow-up of 1.7 years, there were 25 (56%) deaths. On univariate Kaplan-Meier survival analysis, NYHA class > 2, DT <150 msec and lack of beta-blocker use were associated with increased mortality (log-rank statistic p-values <0.001, <0.05 and 0.01, respectively). Cox Proportional Hazard Survival Analysis is shown in the table . In patients with EMB-documented CA, s urvival is more strongly associated with NYHA functional class compared to ECG and echocardiographic variables, including DT and MPI. Evaluation of advanced imaging techniques such as cardiac magnetic resonance in predicting prognosis in these patients is warranted. Table: Cox Proportional Hazard Analysis of Various Clinical, Electrocardiographic and Echocardiographic Predictors of Mortality in Patients with Biopsy Proven Cardiac Amyloidosis

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