scholarly journals Electrocardiographic Characteristics and Their Correlation with Echocardiographic Alterations in Fabry Disease

2022 ◽  
Vol 9 (1) ◽  
pp. 11
Author(s):  
Matthew Zada ◽  
Queenie Lo ◽  
Siddharth J. Trivedi ◽  
Mehmet Harapoz ◽  
Anita C. Boyd ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Wall Thickness ◽  
Left Ventricular ◽  
P Wave ◽  
Cross Sectional ◽  
Qrs Duration ◽  
And Gender ◽  
Cardiac Manifestations ◽  
Ecg Abnormalities ◽  
Ventricular Depolarization

Fabry disease (FD) is an X-linked disorder with α-galactosidase A deficiency. Males (>30 years) and females (>40 years) often present with cardiac manifestations, predominantly left ventricular hypertrophy (LVH). The aim of this study was to evaluate electrocardiographic (ECG) characteristics within FD patients to identify gender related differences, and to additionally explore the association of ECG parameters with structural and functional alterations on transthoracic echocardiography (TTE). Retrospective cross-sectional analysis of 45 FD patients with contemporaneous ECG and TTE was performed and compared to age and gender matched healthy controls. FD patients demonstrated alterations in several ECG parameters particularly in males, including prolonged P-wave duration (91 vs. 81 ms, p = 0.022), prolonged QRS duration (96 vs. 84 ms, p < 0.001), increased R-wave amplitude in lead I (8.1 vs. 5.7 mV, p = 0.047), increased Sokolow–Lyon index (25 vs. 19 mV, p = 0.002) and were more likely to meet LVH criteria (31% vs. 7%, p = 0.006). FD patients with impaired basal longitudinal strain (LS) on TTE were more likely to meet LVH criteria (41% vs. 0%, p = 0.018). Those with more advanced FD (increased LV wall thickness on TTE) were more likely to meet LVH criteria but additionally demonstrated prolonged ventricular depolarization (QRS duration 101 vs. 88 ms, p = 0.044). Therefore, alterations on ECG demonstrating delayed atrial activation, delayed ventricular depolarization and evidence of LVH were more often seen in male FD patients. Impaired basal LS, a TTE marker of early cardiac involvement, correlated with ECG abnormalities. Increased LV wall thickness on TTE, a marker of more advanced FD, was associated with more severe ECG abnormalities.

scholarly journals Hypertrophy of unaffected cardiomyocytes correlates with severity of cardiomyopathy in female patients with Fabry disease

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Cristina Chimenti ◽  
Romina Verardo ◽  
Andrea Frustaci
Keyword(s):  
Fabry Disease ◽  
Wall Thickness ◽  
Left Ventricular ◽  
Female Patients ◽  
Maximal Wall Thickness ◽  
Group 4 ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Abstract Aim To investigate the contribution of unaffected cardiomyocytes in Fabry disease cardiomyopathy. Findings Left ventricular (LV) endomyocardial biopsies from twenty-four females (mean age 53 ± 11 ys) with Fabry disease cardiomyopathy were studied. Diagnosis of FD was based on the presence of pathogenic GLA mutation, Patients were divided in four groups according with LV maximal wall thickness (MWT): group 1 MWT ≤ 10.5 mm, group 2 MWT 10.5–15 mm, group 3 MWT 16–20 mm, group 4 MWT > 20 mm. At histology mosaic of affected and unaffected cardiomyocytes was documented. Unaffected myocytes’ size ranged from normal to severe hypertrophy. Hypertrophy of unaffected cardiomyocytes correlated with severity of MWT (p < 0.0001, Sperman r 0,95). Hypertrophy of unaffected myocytes appear to concur to progression and severity of FDCM. It is likely a paracrine role from neighboring affected myocytes.

4091Mechanical dispersion by speckle tracking echocardiography in fabry disease

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T F Cianciulli ◽  
M C Saccheri ◽  
A M Risolo ◽  
J A Lax ◽  
R J Mendez ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Speckle Tracking ◽  
Heart Diseases ◽  
Systolic Dysfunction ◽  
Cross Sectional Study ◽  
Left Ventricular ◽  
Peak Strain ◽  
Cross Sectional ◽  
Mechanical Dispersion ◽  
Group I

Abstract Background Fabry disease is a rare X-linked storage disorder caused by a deficiency of the lysosomal enzyme α-galactosidase A and generally causes multi-organ dysfunction. Heart disease is the main cause of death, due to severe left ventricular (LV) systolic dysfunction and sudden death. In several heart diseases, the LV systolic dysfunction and ventricular arrhythmias are associated with mechanical dispersion (MD). The presence of MD in patients with FD has not been studied yet. In this cross-sectional study, we investigated the prevalence of MD in patients with FD. Methods Complete echocardiographic and speckle tracking echocardiographic (STE) data were collected. MD is an index of inter-segmental discoordination of contraction which has been used to quantify LV dyssynchrony and was defined as the standard deviation (SD) of time to peak negative strain in 17 left ventricular segments. Patients were divided into two groups according to whether or not they had left ventricular hypertrophy (LVH). MD was defined as an SD >49 msec. Results We studied 108 patients with FD, 24 patients (22%) were excluded due to inadequate imaging quality or presence of comorbidities, so the final study population consisted of 84 patients (mean age 33.3±14.6 years, 60.7% women). LVH in FD appears at older ages than in patients without LVH (48±12.5 y/o vs 27.8±11.1 y/o, p<0.0001). Patients with FD without LVH (Group I) showed normal global longitudinal peak strain (GLPS) (21.2±2.5%) and no MD (32.7±8.8 msec). In Group II (n=23) patients with FD with LVH, 17 (73.9%) had MD >49 msec prolonged mechanical dispersion (73.3±20.7 msec) and reduced GLPS (13.6±4.0%). MD was more pronounced in Fabry patients with LVH than in patients without LVH (63.4±24.7 msec vs. 32.7±8.8 msec, p<0.0001). GLPS was lower in Fabry patients with LVH than in patients without LVH (15.3±4.7% vs 21.2±2.5%, p<0.0001). Figure 1 Conclusions To our knowledge, this is the first study to demonstrate the prevalence of mechanical dispersion in patients with FD. Mechanical dispersion was seen in 73.9% of patients with FD with LVH. This dyssynchrony should be taken into account in patients who develop heart failure or life-threatening ventricular tachyarrhythmias.

Proposed stages of Fabry disease: insights from multiparametric cardiac MRI and advanced ECG

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
JA Bicho Augusto ◽  
N Johner ◽  
D Shah ◽  
S Nordin ◽  
K Knott ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Cardiac Mri ◽  
Cardiac Involvement ◽  
T2 Mapping ◽  
Left Ventricular ◽  
P Wave ◽  
Stress Perfusion ◽  
Healthy Controls ◽  
T Wave ◽  
Advanced Analysis

Abstract Funding Acknowledgements Type of funding sources: None. Background Staging of Fabry disease (FD) cardiomyopathy uses multiparametric cardiac MRI. Advanced disease is characterized by left ventricular hypertrophy (LVH), myocardial inflammation/oedema (high native T2 mapping) and/or fibrosis (late gadolinium enhancement, LGE). Pre-LVH involvement has been described and includes myocardial sphingolipid storage (low native T1 mapping), impaired LV global longitudinal strain (GLS) and microvascular disease/dysfunction (low stress myocardial blood flow, MBF, in perfusion mapping). We aimed to define (1) the early myocardial phenotype prior to T1 lowering/pre-storage and (2) the stages of cardiac involvement in FD.   Methods FD patients and age, sex and heart rate matched healthy controls underwent same-day ECG with advanced analysis and multiparametric CMR (cines, GLS, pre-contrast T1 and T2 mapping, adenosine stress perfusion mapping [for MBF] and LGE). Results 114 Fabry patients (46 ± 13 years, 61% female, 37% [n = 72] had LVH) and 76 controls (49 ± 15 years, 50% female) were included. FD with vs without LVH in brief and as expected, FD with LVH had significantly (p &lt; 0.05) lower MBF, GLS and T1, and higher T2 and %LGE. FD pre-LVH low T1 vs pre-LVH normal T1: low T1 patients (32/72, 44%) had higher LV mass index (67 ± 14 vs 59 ± 10g/m2, P = 0.011), maximum Q wave amplitude (2[1-2] vs 1[1-2]mm, P &lt; 0.001), Sokolow-Lyon index (22[16-28] vs 17[13-23]mm, P = 0.031) and more fractionated QRS complexes (44 vs 18%, P = 0.020). FD pre-LVH normal T1 vs healthy controls: normal T1 pre-LVH Fabry patients (40/72, 56%) had reduced GLS (-18 ± 2 vs -20 ± 2%, P &lt; 0.001), microvascular impairment (lower MBF 2.5 ± 0.7 vs 3.0 ± 0.8mL/g/min, P = 0.028), subtle T2 elevation (50 ± 4 vs 48 ± 2ms, p = 0.027) and limited LGE (%LGE 0.3 ± 1.1 vs 0%, P = 0.004) when compared to healthy controls; ECG abnormalities included shorter P wave duration (88 ± 12 vs 94 ± 15ms, P = 0.010) and T wave peak time (Tonset–Tpeak; 104 ± 28 vs 115 ± 20ms, P = 0.015), resulting in a more symmetric T wave with lower T wave time ratio (Tonset–Tpeak)/(Tpeak–Tend) (1.5 ± 0.4 vs 1.8 ± 0.4, P &lt; 0.001) compared to controls. Conclusion Prior staging of Fabry cardiomyopathy included a pre-LVH stage (accumulation/storage) and two LVH stages (hypertrophy and inflammation; fibrosis and impairment). Here we define an even earlier stage, pre-LVH pre-detectable storage, defined by microvascular dysfunction, impaired GLS and altered atrial depolarization and ventricular repolarization intervals (see Figure). Abstract Figure. Proposed stages of cardiac involvement

scholarly journals “A CROSS SECTIONAL, DESCRIPTIVE STUDY ON SYSTEMIC ILLNESSES AND CARDIAC FUNCTION ABNORMALITIES IN CHILDREN”

2021 ◽  
pp. 75-79
Author(s):  
Munesh Tomar ◽  
Tanvi goel ◽  
Raza Faizan ◽  
Vijay Jaiswal
Keyword(s):  
Cardiac Output ◽  
Cardiac Function ◽  
Ventricular Dysfunction ◽  
Significant Proportion ◽  
Descriptive Study ◽  
Left Ventricular ◽  
The Body ◽  
Cross Sectional ◽  
Pr Interval ◽  
Ecg Abnormalities

Background:There are wide number of diseases of almost every system in the body which can affect heart in a number of different ways including increasing demands on the heart ,ventricular dysfunction ,rhythm abnormalities ,valve abnormalities ,pulmonary pressures and lot more.Cardiac involvement in systemic diseases is usually silent or oligosymptomatic and includes different pathophysiological mechanisms such as myocardial inflammation, infarction ,subendocardial vasculitis,valvular disease and different patterns of fibrosis. Objective : To study association between systemic illnesses (hematological, endocrinal , renal) and cardiac function abnormalities as ventricular function,cardiac dimensions ,pulmonary artery pressure and pericardial effusion,for early diagnosis and treatment to decrease morbidity and mortality in patient with systemic illness. Design/Method:It was a cross sectional,descriptive study The present study was conducted in the Department of Pediatrics, LLRM Medical College,Meerut,Uttar Pradesh over a period of 1 year (June 2019-June 2020) Results: Cardiac findings in all three groups show ECG abnormalities and echocardiographic changes compared to general population. ECG abnormalities were prolonged PR interval and sinus tachycardia while echocardiographic changes mainly left ventricular(LV) dilatation and hypertrophy ,increased cardiac output ,ventricular dysfunction and pulmonary arterial hypertension(PAH),were noted in a significant proportion of patients. Conclusion:Systemic illnesses affect cardiac parameters in various ways including prolonged PR interval,cardiac dilatation,chamber hypertrophy ,high cardiac output,high cardiac index ,PAH and ventricular dysfunction.

Electrocardiographic Predictors of Cognitive Decline and Dementia: A Systematic Review

2021 ◽  
pp. 1-20
Author(s):  
Yume Imahori ◽  
Davide L. Vetrano ◽  
Petter Ljungman ◽  
Chengxuan Qiu
Keyword(s):  
Systematic Review ◽  
Heart Rate ◽  
Cognitive Decline ◽  
Prospective Studies ◽  
Left Ventricular ◽  
Qt Prolongation ◽  
P Wave ◽  
Cross Sectional ◽  
Incident Dementia ◽  
Electrocardiogram Ecg

Background: Markers of altered cardiac function might predict cognitive decline and dementia. Objective: This systematic review aims to review the literature that examines the associations of various electrocardiogram (ECG) markers with cognitive decline and dementia in middle-aged and elderly populations. Methods: We searched PubMed, Embase, and Web of Science through 1 July 2020 for literature and conducted a systematic literature review. We included studies examining the associations of ECG markers (e.g., left ventricular hypertrophy [LVH], spatial QRS-T angle, and QT prolongation) with cognitive function and dementia in adult populations regardless of study setting and design, but excluded studies examining atrial fibrillation and heart rate variability. Results: Fourteen community-based cross-sectional and longitudinal studies were identified. ECG markers were investigated in association with dementia in four prospective studies, and with cognitive decline in ten prospective studies. ECG-assessed LVH was associated with dementia in one study while five heterogeneous prospective studies yielded inconsistent associations with cognitive decline. Regarding ventricular repolarization markers, spatial QRS-T angle was associated with cognitive decline in one study while another study found no association between QT prolongation and cognitive decline. High resting heart rate was associated with both dementia and cognitive decline in one study but not associated with dementia in another study. P-wave abnormality was significantly associated with incident dementia and cognitive decline in one prospective study. Conclusion: Some ECG markers were associated with incident dementia and cognitive decline. However, limited number of heterogeneous studies did not allow us to make firm conclusions. Further studies are needed.

P0076HOMOARGININE ASSOCIATES WITH RENAL AND CARDIAC FUNCTION IN FABRY DISEASE

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Christiane Drechsler ◽  
Andreas Menitzer ◽  
Winfried Maerz ◽  
Lena Gutjahr-Lengsfeld ◽  
Daniel Oder ◽  
...  
Keyword(s):  
Renal Function ◽  
Fabry Disease ◽  
Clinical Symptoms ◽  
Controlled Trial ◽  
Cardiac Contractility ◽  
Left Ventricular ◽  
Amino Acid Derivative ◽  
Cerebral Stroke ◽  
Cross Sectional ◽  
Lv Mass

Abstract Background and Aims Patients with Fabry disease frequently develop progressive Fabry nephropathy, hypertrophic cardiomyopathy with arrhythmias and subsequent death, transient ischemic attacks and early cerebral stroke. Homoarginine is an amino acid derivative, mainly produced in the kidney from its precursor lysine. Homoarginine may increase nitric oxide availability, decrease the release of cytokines, modulate the renin-angiotensin-aldosterone system and improve cardiac contractility. We hypothesize that high homoarginine levels associate with less clinical symptoms and better renal function in patients with Fabry disease. Method This study investigated the homoarginin status and its association with renal function, left ventricular (LV) mass and adverse clinical symptoms in patients with Fabry disease. Homoarginine was measured by high-performance liquid chromatography in 162 patients who were genetically proven to have Fabry disease. GFR was determined by DTPA clearance. LV mass and cardiomyopathy were assessed by magnetic resonance imaging and echocardiography. In cross-sectional analyses, associations with adverse clinical outcomes were determined by linear and binary logistic regression analyses, respectively, and were adjusted for age, sex, and BMI. Results Patients had a mean age of 39±14 years and 41% were male. The mean homoarginine concentration was 2.0±1.0 µmol/l. Patients had a mean BMI of 23.7±4.5 kg/m2 and a mean GFR of 93±37 ml/min. Homoarginine was significantly correlated with GFR and proteinuria. The better the homoarginine status of the patients, the higher was their GFR (r=0.20, p=0.04) and the lower proteinuria (r=-0.21, p=0.03). Furthermore, LV mass was significantly higher with lower homoarginine levels (r=-0.30, p&lt;0.01). Patients of the lowest homoarginine tertile had a 4-fold higher risk of myocardial hypertrophy (OR 4.17, 95% CI 1.44-12.02), especially with septal and posterior hypertrophy, compared to those of the upper tertiles. Similarly, patients of the lowest homoarginine tertile had a higher rate of angina pectoris (OR 4.2, 95% CI 1.3-13.3) and chronic pain (OR 4.1, 95% CI 1.7-10.1), compared to patients of the upper tertiles. At lower homoarginine status, the median levels of proteinuria increased, as well as the prevalence rates of heart failure and the need for analgesic therapy. Conclusion In conclusion, low homoarginine status was strongly associated with cardiomyopathy, renal function and adverse clinical symptoms in patients with Fabry disease. Whether homoarginine supplementation improves complications of Fabry disease, requires a randomized controlled trial.

scholarly journals Left ventricular global strains by linear measurements in three dimensions: interrelations and relations to age, gender and body size in the HUNT Study

Open Heart ◽  
2019 ◽  
Vol 6 (2) ◽  
pp. e001050 ◽  
Author(s):  
Asbjørn Støylen ◽  
Harald Edvard Mølmen ◽  
Håvard Dalen
Keyword(s):  
Body Size ◽  
Normal Values ◽  
Left Ventricular ◽  
Three Dimensions ◽  
Health Study ◽  
Relative Deformation ◽  
Linear Measurements ◽  
Cross Sectional ◽  
Strain Components ◽  
And Gender

BackgroundStrain is a relative deformation and has three dimensions, in the left ventricle (LV) usually longitudinal (εL), transmural (εT) and circumferential (εC) strain. All three components can be measured generically by the basic systolic and diastolic dimension measures of LV wall length, wall thickness and diameter. In this observational study we aimed to study the relations of normal generic strains to age, body size and gender, as well as the interrelations between the three strain components.MethodsGeneric strains derived from dimension measures by longitudinal and cross-sectional M-mode in all three dimensions were measured in 1266 individuals without heart disease from the Nord-Trøndelag Health Study.ResultsThe mean εL was −16.3%, εC was −22.7% and εT was 56.5%. Normal values by age and gender are provided. There was a gradient of εC from the endocardial, via the midwall to the external level, lowest at the external. All strains decreased in absolute values by increasing body surface area (BSA) and age, relations were strongest for εL. Gender differences were mainly a function of BSA differences. The three strain components were strongly interrelated through myocardial incompressibility.ConclusionsGlobal systolic strain is the total deformation of the myocardium; the three strain components are the spatial coordinates of this deformation, irrespective of the technology used for measurement. Normal values are method-dependent and not normative across methods. Interrelation of strains indicates a high degree of myocardial incompressibility and that longitudinal strain carries most of the total information.

scholarly journals Sequential left ventricular electro-mechanical changes in presence of left bundle branch block

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
J Duchenne ◽  
S Calle ◽  
A Puvrez ◽  
F Rega ◽  
F Timmermans ◽  
...  
Keyword(s):  
Animal Model ◽  
Wall Thickness ◽  
Lateral Wall ◽  
Mechanical Dyssynchrony ◽  
Left Ventricular ◽  
Conduction Delay ◽  
Bundle Branch Block ◽  
Septal Wall ◽  
Qrs Duration ◽  
Over Time

Abstract Introduction Recent cross-sectional studies suggest a relationship between persisting left bundle branch block (LBBB) and the extent of left ventricular (LV) electro-mechanical alterations over time. When patients are referred for cardiac resynchronization therapy (CRT), temporal data during the sub-clinical phase of disease is often missing. A longitudinal study using an animal model would provide a better understanding of the relationship between the onset of LBBB and the electro-mechanical changes. Purpose To investigate the sequential alterations in LV structure and function that develop over time in an animal model of LBBB. Methods Thirteen sheep were subjected to rapid DDD pacing (180 bpm; leads on right atrium and right ventricular free wall) in order to induce a LBBB-like conduction delay. All animals underwent an 8-week pacing protocol, whereas 4 of them were subjected to 16 weeks of pacing in total. Echocardiographic speckle tracking was used to assess circumferential strain of the septal and lateral wall. Septal and lateral wall thickness were measured at end-diastole. Cardiac magnetic resonance imaging was used to determine LV volumes and ejection fraction (LVEF). Examinations took place at baseline (before and after start of pacing), and after 8 and 16 weeks of pacing. All examinations were performed at a physiologic heart rate of 110 bpm. Results At baseline, DDD pacing induced an increase in QRS duration (+85%, p&lt;0.0001) and LBBB-like mechanical dyssynchrony, with mild early-systolic notching and preserved systolic shortening of the septal wall. The lateral wall demonstrated early pre-stretch followed by increasing systolic shortening. No acute changes in LV end-diastolic volume, LVEF or septal or lateral wall thickness were observed (all p&gt;0.05). After 8 weeks of DDD pacing, mechanical dyssynchrony worsened: septal notching increased, followed by reduced systolic shortening. After 16 weeks, the initial septal shortening was followed by profound stretching throughout systole. Lateral wall systolic shortening was reduced compared to baseline. QRS duration increased further by +12% (week 8) and +20% (week 16) (all p&lt;0.001). End-diastolic volumes had increased by +39% (week 8) and +72% (week 16), whereas LVEF had decreased by −48% (week 8) and −56% (week 16) (all p&lt;0.001). Septal wall thickness had reduced by −24% (week 8) and −33% (week 16), while lateral wall thickness had increased by +21% (week 8) and +30% (week 16) (all p&lt;0.05). Conclusion A persisting LBBB-like conduction delay induces sequential changes in LV deformation patterns, and triggers morphological and electrical remodelling. These changes are similar to those observed in patients with LBBB and different degrees of LV dysfunction. Our data suggest a continuum due to the progression of LBBB-induced LV disease. In the clinic, patients with mild dysfunction should be closely monitored in order to treat dyssynchrony as soon as guideline indications are reached. FUNDunding Acknowledgement Type of funding sources: Other. Main funding source(s): This work was supported by a KU Leuven research grant

scholarly journals Correlation between P-Wave Terminal Force V1 (PTFV1) from 12-Lead ECG and Left Ventricular Diastolic Dysfunction in Patients Diagnosed With Hypertension

2020 ◽  
Vol 6 (2) ◽  
pp. 97-103
Author(s):  
Bambang Arie Hidayat Dalimunthe ◽  
Nizam Akbar ◽  
Refli Hasan ◽  
Harris Hasan ◽  
Andika Sitepu ◽  
...  
Keyword(s):  
Diastolic Dysfunction ◽  
Systolic Dysfunction ◽  
Hypertensive Heart Disease ◽  
Left Ventricular Diastolic Dysfunction ◽  
Left Ventricular ◽  
P Wave ◽  
P Value ◽  
Cross Sectional ◽  
Ventricular Diastolic Dysfunction ◽  
Grade Iii

Background: Patients diagnosed with hypertension will deteriorate into hypertensive heart disease which is characterized by diastolic dysfunction first followed by systolic dysfunction later in the course of the disease. Diastolic dysfunction of the left ventricle causes an increase in LVEDP as well as in the dimension of the left atrium. P-Wave Terminal Force V1 (PTFV1) which is derived from 12 lead ECG could help diagnose diastolic dysfunction in centers where echocardiography is not available. The purpose of this study was to determine the correlation of PTFV1 on the 12-lead Electrocardiography with diastolic dysfunction in patients diagnosed with hypertension in the outpatient clinic of Cardiac Center Adam Malik General Hospital in Medan. Methods: This is a cross-sectional study conducted from March 2019 until August 2019. Patients with hypertension who met the inclusion criteria were examined electrocardiographically to obtain PTFV1 value. Then echocardiography examination was then performed to assess the grades of diastolic dysfunction and other parameters. Analysis of correlation between PTFV1 values and diastolic dysfunction was then conducted. Results: From the clinical characteristics, there is no difference regarding age, sex , and risk factorsbetween the three diastolic dysfunction groups, while echocardiography characteristic shows more reduced EF in grade III diastolic dysfunction (36.5±7.7). Significant differences in PTFV1 are found among diastolic dysfunction groups. Grade I diastolic dysfunction has PTFV1 value of 23.8 mm.ms, grade II diastolic dysfunction has PTFV1 value of 34.1 mm.ms, and grade III diastolic dysfunction has PTFV1 value of 52.1 mm.ms, Significance of  p value is <0.001. There is a strong correlation between PTFV1 and diastolic dysfunction grade (r = 0.63 (P <0.001)). Cut off point of PTFV1 > 29.8 mm.ms can discriminate patients who have increased LAP with a sensitivity of 84% and specificity of 71%. Conclusions: PTFV1 is a simple screening tool which is widely available and correlate well with left ventricular diastolic dysfunction in patients with hypertension, which makes it a good alternative tool especially in areas where echocardiography is not readily available.

scholarly journals P.025 APOLLO, a phase 3 study of patisiran for the treatment of hereditary transthyretin amyloidosis (hATTR): 18-month safety and efficacy in subgroup with cardiac involvement

2019 ◽  
Vol 46 (s1) ◽  
pp. S20
Author(s):  
J Vest ◽  
S Solomon ◽  
D Adams ◽  
A Gonzalez-Duarte ◽  
W O’Riordan ◽  
...  
Keyword(s):  
Wall Thickness ◽  
Gait Speed ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Phase 3 ◽  
Transthyretin Amyloidosis ◽  
End Diastolic Volume ◽  
Life Threatening ◽  
And Function ◽  
Cardiac Manifestations

Background: Hereditary transthyretin-mediated (hATTR) amyloidosis a hereditary, multi-systemic and life-threatening disease resulting in neuropathy and cardiomyopathy. In the APOLLO study, patisiran, an investigational RNAi therapeutic targeting hepatic TTR production resulted in significant improvement in neuropathy and QoL compared to placebo and was generally well tolerated. Methods: APOLLO, a Phase 3 study of patisiran vs. placebo (NCT01960348) prespecified a cardiac subpopulation (n=126 of 225 total) that included patients with baseline left ventricular (LV) wall thickness ≥ 13mm and no medical history of aortic valve disease or hypertension. Cardiac measures included structure and function by electrocardiography, changes in NT-proBNP and 10-MWT gait speed. Results: At 18 months, patisiran treatment resulted in a mean reduction in LV wall thickness of 1 mm (p=0.017) compared to baseline, which was associated with significant improvements relative to placebo in LV end diastolic volume (+8.31 mL, p=0.036), global longitudinal strain (-1.37%, p=0.015) and NT-proBNP (55% reduction, p=7.7 x 10-8) (Figure 1). Gait speed was also improved relative to placebo (+0.35 m/sec, p=7.4 x 10-9). Rate of death or hospitalization was lower with patisiran. mNIS+7 results in the cardiac subpopulation will also be presented. Conclusions: These data suggest patisiran has the potential to halt or reverse cardiac manifestations of hATTR amyloidosis.

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