scholarly journals 85 Diuretics trajectories in dilated cardiomyopathy

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Vincenzo Nuzzi ◽  
Antonio Cannatà ◽  
Paolo Manca ◽  
Caterina Gregorio ◽  
Giulia Barbati ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Nyha Class ◽  
Diuretic Therapy ◽  
Left Ventricular ◽  
Equivalent Dose ◽  
Left Ventricular Ejection ◽  
Class Iii ◽  
Ventricular Ejection Fraction ◽  
Kaplan Meier

Abstract Aims Diuretics in heart failure (HF) are commended to relieve symptoms at lowest dosage effective. Dilated cardiomyopathy (DCM) is a particular HF setting with several variables that may influence disease trajectory. We aimed to assess the long-term use of diuretics in DCM, the possibility of withdrawal and to explore the prognostic correlations. Methods and results All consecutive DCM patients enrolled from 1990 to 2018 were considered eligible. All the patients had available the information about the furosemide-equivalent dose at baseline and at follow-up evaluation within 24 months. Patients were categorized in stable (diuretic dose variation <50%), increasers (diuretics dose increase ≥50% or initiation of diuretic therapy), and decreasers (diuretics dose decrease ≥50% or never prescribed diuretics in the 24-months observation period). The prognostic role of the diuretics trajectory group was assessed with Kaplan Meier analysis and with a time-dependent multivariable model. The outcome measure was a composite of all-cause death/heart transplantation/HF hospitalization (ACD/HTx/HFH). 908 patients were included [mean age 50 ± 16, 70% male sex, 24% NYHA class III or IV, mean left ventricular ejection fraction (LVEF) 31 ± 9%, 66% treated with diuretics at baseline]. The furosemide-equivalent dose at enrolment had a linear association with the risk of outcome. Compared to other groups, decreaser patients were younger, had less HF symptoms, higher LVEF and more dilated left atrium. Decreasers had a lower prescription rate of diuretics and less frequent indication to renin-angiotensin inhibitors and mineralocorticoid receptors antagonists. Over a median follow-up of 122 (62–195) months decreasers had the lowest incidence of outcome, followed by stable, while increasers had the worst outcome (P < 0.001). After adjustment for other prognosticators, compared to stable patients, decreasers had a reduced risk of ACD/HTx/HFH [HR: 0.497 (95% CI: 0.337–0.731)] while increasers had the highest risk of adverse outcome [HR: 2.027 (95% CI: 1.254–3.276)]. Similarly, amongst patients taking diuretics at baseline, the diuretics withdrawal was in independent outcome predictor. The only multivariable predictors of diuretics withdrawal were younger age and lower furosemide-equivalent dose at enrolment. Conclusions In DCM patients the diuretics dose at baseline is a strong prognosticator. Diuretics dose reduction or its withdrawal provides a prognostic benefit on hard outcome. Diuretics tapering in selected patients should be considered in the short-term follow-up to improve DCM prognosis.

Abstract 16141: Predictors of Death in Patients With Chagas Cardiomyopathy and Pacemaker - Preliminary Results of the PACINCHAGAS Study

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Giselle L Peixoto ◽  
Rodrigo O Madia ◽  
Sérgio F Siqueira ◽  
Mariana M Lensi ◽  
Silvana D Nishioka ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Nyha Class ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Class Iii ◽  
Ventricular Ejection Fraction ◽  
Chagas Cardiomyopathy ◽  
The Mean

Introduction: Chagas’ disease causes different clinical expressions in the heart and permanent pacing due to bradycardia is not uncommon. Objective: Predictors of death in patients with Chagas Cardiomyopathy requiring pacemaker (PM) are unknown. This is the aim of this study. Methods: We prospectively evaluated 529 patients included in the Pacinchagas Study - Risk Stratification in Pacemaker Patients with Chagas Cardiomyopathy, which primary objective is to create a risk score to predict death in this population. The patients are submitted to an extent questionnaire which included clinical (NYHA class, symptoms, comorbidities and medications) functional (electrocardiography, Holter and echocardiography) and electronic variables (burden of pacing and arrhythmias). Patients with at least 6 months of follow-up were included in this preliminar analysis. Results: The cohort included 337 (63.7%) females, the mean age was 62.3±11.9 years and 63.1% were in NYHA class I. Indication for PM implantation was atrioventricular block, sick sinus syndrome, atrial fibrillation with slow ventricular response and unknown in 72.0%; 20.4%; 5.1% and 2.5%, respectively. During a mean follow-up of 1.5±0.6 years, 62 (11.7%) patients died. The mean time of PM implantation was not different between the dead and the survivors (11.9±9.0years versus 11.1±8.6years, P=0.503). Twenty-five deaths (40.3%) were sudden, 22 (35.5%) were due to heart failure, 6 (9.7%) were due to other cardiovascular causes, and 7 (11.3%) were due to noncardiovascular causes. The cause of death could not be determined in two patients (3.2%). Cox proportional hazards identified three predictors of death: NYHA class III/IV (Hazard Ratio [HR] 5.661; 95% Confidence Interval [95%IC] 2.617-12.245; P<0.001); left ventricular ejection fraction (LVEF) ≤42% (HR 2.779; 95%IC 1.299-5.945; P=0.008) and chronic kidney disease (HR 2.635; 95%IC 1.167-5.948; P=0.020). Conclusions: This analysis of Pacinchagas study identified in a mean follow-up of one year and half, three predictors of death in PM users with Chagas Cardiomyopathy: NYHA class III/IV, LVEF≤42% and chronic kidney disease.

scholarly journals Incidence of Cardiotoxicity and Validation of the Heart Failure Association- International Cardio-Oncology Society risk stratification Tool in Patients Treated with Trastuzumab for HER2-Positive Early Breast Cancer.

2021 ◽  
Author(s):  
Nicolò Matteo Luca Battisti ◽  
Maria Sol Andres ◽  
Karla A Lee ◽  
Tharshini Ramalingam ◽  
Tamsin Nash ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Heart Failure ◽  
Early Breast Cancer ◽  
Nyha Class ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Class Iii ◽  
Her2 Positive ◽  
Ventricular Ejection Fraction ◽  
Very High

Abstract PurposeTrastuzumab improves survival in patients with HER2+ early breast cancer. However, cardiotoxicity remains a concern, particularly in the curative setting, and there are limited data on its incidence outside of clinical trials. We retrospectively evaluated the cardiotoxicity rates (left ventricular ejection fraction [LVEF] decline, congestive heart failure [CHF], cardiac death or trastuzumab discontinuation) and assessed the performance of a proposed model to predict cardiotoxicity in routine clinical practice.MethodsPatients receiving curative trastuzumab between 2011-2018 were identified. Demographics, treatments, assessments and toxicities were recorded. Fisher’s exact test, chi-squared and logistic regression were used.Results931 patients were included in the analysis. Median age was 54 years (range 24-83) and Charlson comorbidity index 0 (0-6), with 195 patients (20.9%) aged 65 or older. 228 (24.5%) were smokers. Anthracyclines were given in 608 (65.3%). Median number of trastuzumab doses was 18 (1-18). The HFA-ICOS cardiovascular risk was low in 401 patients (43.1%), medium in 454 (48.8%), high in 70 (7.5%) and very high in 6 (0.6%).Overall, 155 (16.6%) patients experienced cardiotoxicity: LVEF decline≥10% in 141 (15.1%), falling below 50% in 55 (5.9%), CHF NYHA class II in 42 (4.5%) and class III-IV in 5 (0.5%) and discontinuation due to cardiac reasons in 35 (3.8%). No deaths were observed.Cardiotoxicity rates increased with HFA-ICOS score (14.0% low, 16.7% medium, 30.3% high/very high; p=0.002). ConclusionsCardiotoxicity was relatively common (16.6%), but symptomatic heart failure on trastuzumab was rare in our cohort. The HFA-ICOS score identifies patients at high risk of cardiotoxicity

The use of peritoneal dialysis in heart failure: A systematic review

2020 ◽  
Vol 40 (6) ◽  
pp. 527-539 ◽  
Author(s):  
Chang Yin Chionh ◽  
Anna Clementi ◽  
Cheng Boon Poh ◽  
Fredric O Finkelstein ◽  
Dinna N Cruz
Keyword(s):  
Heart Failure ◽  
Systematic Review ◽  
Peritoneal Dialysis ◽  
Hospital Admissions ◽  
Diuretic Therapy ◽  
Heart Association ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction

Heart failure (HF) is a major cause of morbidity and mortality. Extracorporeal (EC) therapy, including ultrafiltration (UF) and haemodialysis (HD), peritoneal dialysis (PD) and peritoneal ultrafiltration (PUF) are potential therapeutic options in diuretic-resistant states. This systematic review assessed outcomes of PD and compared the effects of PD to EC. A comprehensive search of major databases from 1966 to 2017 for studies utilising PD (or PUF) in diuretic-resistant HF was conducted, excluding studies involving patients with end-stage kidney disease. Data were extracted and combined using a random-effects model, expressed as odds ratio (OR). Thirty-one studies ( n = 902) were identified from 3195 citations. None were randomised trials. Survival was variable (0–100%) with a wide follow-up duration (36 h–10 years). With follow-up > 1 year, the overall mortality was 48.3%. Only four studies compared PD with EC. Survival was 42.1% with PD and 45.0% with EC; the pooled effect did not favour either (OR 0.80; 95% confidence interval (CI): 0.24–2.69; p = 0.710). Studies on PD in patients with HF reported several benefits. Left ventricular ejection fraction (LVEF) improved after PD (OR 3.76, 95%CI: 2.24–5.27; p < 0.001). Seven of nine studies saw LVEF increase by > 10%. Twenty-one studies reported the New York Heart Association status and 40–100% of the patients improved by ≥ 1 grade. Nine of 10 studies reported reductions in hospitalisation frequency and/or duration. When treated with PD, HF patients had fewer symptoms, lower hospital admissions and duration compared to diuretic therapy. However, there is inadequate evidence comparing PD versus UF or HD. Further studies comparing these modalities in diuretic-resistant HF should be conducted.

Phase III trial of non-pegylated liposomal doxorubicin (M) in combination with trastuzumab (T) and paclitaxel (P) in HER2+ metastatic breast cancer (MBC).

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 517-517 ◽  
Author(s):  
Lorena De La Pena ◽  
Javier Cortes ◽  
Alexey Manikhas ◽  
Laslo Roman ◽  
Vladimir Semiglazov ◽  
...  
Keyword(s):  
Nyha Class ◽  
Metastatic Breast ◽  
Pegylated Liposomal Doxorubicin ◽  
Progression Free Survival ◽  
Left Ventricular ◽  
Phase Iii ◽  
Left Ventricular Ejection ◽  
Class Iii ◽  
Ventricular Ejection Fraction ◽  
Phase Iii Trial

517 Background: M in combination with T has shown promising activity and cardiac safety in MBC patients (pts). We conducted a randomized Phase III trial of first-line M plus T and P (MTP) versus T plus P (TP) in HER2+ MBC pts. Methods: Pts with HER2+ (by FISH) MBC ≥ 18 years and ECOG 0-1, who had received no prior chemotherapy for metastatic disease, were eligible. Left ventricular ejection fraction should be within normal institutional limits. Prior (neo-) adjuvant anthracyclines, T or P were permitted, if completed >1 year before the start of the study. Pts received M 50 mg/m2 q3w for 6 cycles, T 4 mg/kg loading dose followed by 2 mg/kg qw, and P 80 mg/m2qw, or T+P at the same doses until progression or toxicity. Primary outcome was progression-free survival (PFS). Enrollment of 332 pts would provide 80% power with a 5% significance to detect an improvement in PFS with MTP, assuming a median PFS of 8 months in TP and a hazard ratio (HR) of 0.70. Results: 363 pts enrolled (MTP 181, TP 183), 360 received treatment. The two groups were well balanced for demographics, pretreatment characteristics and extent of disease. One third of the pts had prior exposure to anthracyclines, but almost none to trastuzumab (1% and 2% in MTP and TP arms, respectively). Six cycles of M could be given to 72% of the pts. With a median follow-up of 31 months, median PFS was 16.1 and 14.5 months with MTP and TP, respectively (HR 0.84, P=0.174). In pts with ER and PR-negative tumors, PFS was 20.7 and 14.0 months, respectively (HR, 0.68; 95% CI 0.47–0.99). Median overall survival (OS) was 33.6 and 28.9 months, respectively (HR, 0.79, P=0.083). In ER and PR-negative tumors, OS was 38.2 and 27.9 months, respectively (HR, 0.63; 95% CI 0.42–0.93). The incidence of NYHA Class III/IV congestive heart failure was 3% with MTP and there were 2 cardiac deaths with TP. The frequency of adverse events was higher with MTP, especially myelosuppression, stomatitis and gastrointestinal intolerance. Conclusions: The trial failed to demonstrate a significant clinical improvement with the addition of M to TP. The clinical benefit observed in an exploratory analysis in the ER and PR-negative population deserves consideration for further clinical trials. Clinical trial information: NCT00294996.

Improvement in the Severity of Mitral Regurgitation after Direct Intramyocardial Injection of Angiogenic Cell Precursor

2009 ◽  
Vol 4 (5) ◽  
pp. 291-293
Author(s):  
Kitipan V. Arom ◽  
Permyos Ruengsakulrach ◽  
Lertlak Chaothawee
Keyword(s):  
Mitral Regurgitation ◽  
Dilated Cardiomyopathy ◽  
Myocardial Fibrosis ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Severe Mitral Regurgitation ◽  
Class Iii ◽  
Intramyocardial Injection ◽  
Ventricular Ejection Fraction ◽  
Cell Implantation

We reported a case of dilated cardiomyopathy and moderate-severe mitral regurgitation (MR) who we treated by surgical direct intramyocardial angiogenic cell precursors injection. The patient was a New York Heart Association functional class III-IV, 56 year old man, who presented with end-stage congestive heart failure, moderate/severe mitral regurgitation, and myocardial fibrosis with the left ventricular ejection fraction of 13%. After he underwent direct surgical intramyocardial cell implantation, the myocardial fibrosis was resolved at 3 months follow-up. The severity of MR reduced to moderate and mild at 3 and 9 months, respectively. The left ventricular function gradually improved up to 53% at 19 months. To our knowledge, this is one of the only reports of successful direct surgical intramyocardial peripheral blood stem cell implantation to treat MR in dilated cardiomyopathy patient.

scholarly journals Comparison of ventricular tachyarrhythmia recurrence between ischemic cardiomyopathy and dilated cardiomyopathy: a retrospective study

PeerJ ◽  
2018 ◽  
Vol 6 ◽  
pp. e5312 ◽  
Author(s):  
Chih-Yuan Fang ◽  
Huang-Chung Chen ◽  
Yung-Lung Chen ◽  
Tzu-Hsien Tsai ◽  
Kuo-Li Pan ◽  
...  
Keyword(s):  
Secondary Prevention ◽  
Dilated Cardiomyopathy ◽  
Cardiovascular Mortality ◽  
Ischemic Cardiomyopathy ◽  
Ventricular Tachyarrhythmia ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
All Cause Mortality

BackgroundThe use of an implantable cardioverter-defibrillator (ICD) has been established as an effective secondary prevention strategy for ventricular tachycardia (VT)/ventricular fibrillation (VF). However, few reports discuss the difference in clinical predictors for recurrent VT/VF between patients with ischemic cardiomyopathy (ICM) and patients with dilated cardiomyopathy (DCM).MethodsFrom May 2004 to December 2015, 132 consecutive patients who had ICM (n= 94) or DCM (n= 38) and had received ICD implantation for secondary prevention were enrolled in this study. All anti-tachycardia events during follow-up were validated. The clinical characteristics and echocardiographic parameters were obtained for comparison. The incidence of recurrence of VT/VF, cardiovascular mortality, all-cause mortality, the change of left ventricular ejection fraction (LVEF) and LV volume were analyzed.ResultsAt a mean follow-up of 3.62 ± 2.93 years, 34 patients (36.2%) in the ICM group and 22 patients (57.9%) in the DCM group had a recurrence of VT/VF episodes (p= 0.032). The DCM group had a lower LVEF (p= 0.019), a larger LV end-diastolic volume (LVEDV) (p= 0.001), a higher prevalence of LVEDV >158 mL (p= 0.010), and a larger LV end-systolic volume (p= 0.010) than the ICM group. LVEDV >158 mL and no use of angiotensin-converting-enzyme inhibitor/angiotensin receptor blocker were independent predictors of recurrences of VT/VF in ICM patients but not in DCM patients. There were no difference in cardiovascular mortality and all-cause mortality between the ICM and DCM patients.ConclusionThe DCM patients had a higher recurrence rate of VT/VF than did the ICM patients during long-term follow-up. An enlarged LV is an independent predictor of the recurrence of VT/VF in ICM patients receiving ICD for secondary prevention.

scholarly journals 84 Left atrial reverse remodelling in dilated cardiomyopathy

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Vincenzo Nuzzi ◽  
Anne Raafs ◽  
Paolo Manca ◽  
Michiel T.h.M. Henkens ◽  
Caterina Gregorio ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Left Atrial ◽  
Tertiary Care ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Linear Association ◽  
Lower Baseline ◽  
Reverse Remodelling

Abstract Aims Left atrial (LA) dilation is associated a with worse prognosis in several cardiovascular settings but therapies can promote LA reverse remodelling. Characterizing and defining the prognostic implications of LA volume (LAVI) reduction in dilated cardiomyopathy (DCM). Methods and results Consecutive DCM patients from two tertiary care centres, with available echocardiography at baseline and at 1 year follow-up, were analysed. LA dilation was defined as LAVI &gt;34 ml/m2, Delta (Δ)LAVI was defined as the 1 year relative LAVI reduction. The outcome was a composite of death/heart transplantation/heart failure hospitalization (D/HTx/HFH). Five hundred sixty patients were included [age 52 ± 13 years; left ventricular ejection fraction (LVEF) 31 ± 10%, LAVI 45 ± 18 ml/m2]. Baseline LAVI had a non-linear association with the risk of D/HTx/HFH, independently from LVEF (P &lt; 0.001). At 1 year follow-up, LAVI decreased in 374 patients (67%, median ΔLAVI 24%, interquartile range 37% 11%). Factors independently associated with ΔLAVI were higher baseline LAVI and lower baseline LVEF. After adjustment for confounders, ΔLAVI showed a linear association with the risk of D/HTx/HHF (HR: 0.92, 95% CI: 0.86 0.99 per 5% decrease, P &lt; 0.001). At 1 year Follow-up, patients with a ≥ 15% reduction in ΔLAVI or LAVI normalization (i.e. Follow-up LAVI ≤34ml/m2) (42% of the cohort) were at lower risk of D/HTx/HFH (HR: 0.49, 95% CI: 0.33 0.74, P &lt; 0.001). Conclusions In a large cohort of DCM, 1 year reduction in LAVI is observed in the majority of patients. The association between reduction in LAVI and D/HTx/HHF candidates LA reverse remodelling as complementary early therapeutic goal in DCM.

scholarly journals The central arterial stiffness parameters in decompensated versus compensated states of heart failure: a paired comparative cohort study

2022 ◽  
Vol 74 (1) ◽  
Author(s):  
Ahmed El Fol ◽  
Waleed Ammar ◽  
Yasser Sharaf ◽  
Ghada Youssef
Keyword(s):  
Heart Failure ◽  
Arterial Stiffness ◽  
Ejection Fraction ◽  
Pulse Wave ◽  
Nyha Class ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Class Iii ◽  
Ventricular Ejection Fraction ◽  
Reduced Ejection Fraction

Abstract Background Arterial stiffness is strongly linked to the pathogenesis of heart failure and the development of acute decompensation in patients with stable chronic heart failure. This study aimed to compare arterial stiffness indices in patients with heart failure with reduced ejection fraction (HFrEF) during the acute decompensated state, and three months later after hospital discharge during the compensated state. Results One hundred patients with acute decompensated HFrEF (NYHA class III and IV) and left ventricular ejection fraction ≤ 35% were included in the study. During the initial and follow-up visits, all patients underwent full medical history taking, clinical examination, transthoracic echocardiography, and non-invasive pulse wave analysis by the Mobil-O-Graph 24-h device for measurement of arterial stiffness. The mean age was 51.6 ± 6.1 years and 80% of the participants were males. There was a significant reduction of the central arterial stiffness indices in patients with HFrEF during the compensated state compared to the decompensated state. During the decompensated state, patients presented with NYHA FC IV (n = 64) showed higher AI (24.5 ± 10.0 vs. 16.8 ± 8.6, p < 0.001) and pulse wave velocity (9.2 ± 1.3 vs. 8.5 ± 1.2, p = 0.021) than patients with NYHA FC III, and despite the relatively smaller number of females, they showed higher stiffness indices than males. Conclusions Central arterial stiffness indices in patients with HFrEF were significantly lower in the compensated state than in the decompensated state. Patients with NYHA FC IV and female patients showed higher stiffness indices in their decompensated state of heart failure.

Characteristics of Children with Acute Rheumatic Carditis from a High-Incidence Region: Importance of Unexplained Worsening of Functional Class

Cardiology ◽  
2020 ◽  
Vol 145 (8) ◽  
pp. 522-528
Author(s):  
Rajiv Narang ◽  
Anita Saxena ◽  
Sivasubramanian Ramakrishnan ◽  
Saurabh K. Gupta ◽  
Rajnish Juneja ◽  
...  
Keyword(s):  
Nyha Class ◽  
Public Health Problem ◽  
Left Ventricular ◽  
Functional Class ◽  
Left Ventricular Ejection ◽  
First Episode ◽  
Major Public Health Problem ◽  
Class Iii ◽  
Rheumatic Carditis ◽  
Ventricular Ejection Fraction

Background: Acute rheumatic fever (ARF) and acute rheumatic carditis (ARC) continue to be a major public health problem in developing countries. Objective: To study the characteristics of children with ARC being treated at a tertiary centre. Methods and Results: We studied 126 children (mean age 10.4 ± 2.3 years, range 5–15 years, 60% males) diagnosed with ARC by treating cardiologists. Most had lower socio-economic status. Fifty of 126 (40%) presented with a first episode of ARC. Joint symptoms were present in 29% and fever in 25%. Only 2.4% had subcutaneous nodules and none had erythema marginatum or chorea. Fifty-one percent presented in NYHA class II and 29% in NYHA class III or IV. Tachycardia and heart failure were present in 53% and 21%, respectively. Recent worsening of NYHA class (dyspnoea) was the commonest feature (48%). Laboratory investigations showed raised antistreptolysin O titres (>333 units) in only 36.7% of patients. Raised C-reactive protein (CRP) was present in 70%, while raised erythrocyte sedimentation rate was found in only 37% of patients. On the basis of above findings, the modified Jones criteria (2015) for the diagnosis of ARF were satisfied only in 46% of children. Echocardiography showed mitral valve thickening in 77% and small nodules on the tip of the leaflets in 43% (27 and 8%, respectively for aortic valve). Left ventricular ejection fraction was <50% in only 3 patients. The dominant valve lesion was mitral regurgitation (MR) (present in 95% of patients; severe in 78%, moderate in 15%), while aortic regurgitation was present in 44% (severe in 14%). Conclusions: The criteria are often not satisfied by patients being treated for ARC. Recent unexplained worsening of dyspnoea, young age, significant MR, echocardiographic nodules, and elevated CRP are important indicators.

Long-term outcomes and left ventricular diastolic function of sarcomere mutation-positive and mutation-negative patients with hypertrophic cardiomyopathy: a prospective cohort study

2020 ◽  
Author(s):  
Ching-Yu Julius Chen ◽  
Mao-Yuan Marine Su ◽  
Ying-Chieh Liao ◽  
Fu-Lan Chang ◽  
Cho-Kai Wu ◽  
...  
Keyword(s):  
New York ◽  
Hypertrophic Cardiomyopathy ◽  
Nyha Class ◽  
Global Longitudinal Strain ◽  
Heart Association ◽  
Left Ventricular Diastolic Function ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Class Iii ◽  
Ventricular Ejection Fraction

Abstract Aims Hypertrophic cardiomyopathy (HCM) is an inheritable disease that leads to sudden cardiac death and heart failure (HF). Sarcomere mutations (SMs) have been associated with HF. However, the differences in ventricular function between SM-positive and SM-negative HCM patients are poorly characterized. Methods and results  Of the prospectively enrolled 374 unrelated HCM patients in Taiwan, 115 patients underwent both 91 cardiomyopathy-related gene screening and cardiovascular magnetic resonance (45.6 ± 10.6 years old, 76.5% were male). Forty pathogenic/likely pathogenic mutations were identified in 52 patients by next-generation sequencing. The SM-positive group were younger at first cardiovascular event (P = 0.04) and progression to diastolic HF (P = 0.02) with higher N-terminal pro-brain natriuretic peptide (NT-proBNP) [New York Heart Association (NYHA) Class III/IV symptoms with left ventricular ejection fraction &gt; 55%] than the SM-negative group (P &lt; 0.001). SM-positive patients had a greater extent of late gadolinium enhancement (P = 0.01), larger left atrial diameter (P = 0.03), higher normalized peak filling rate (PFR) and PFR ratio, and a greater reduction in global longitudinal strain than SM-negative patients (all P ≤ 0.01). During mean lifelong follow-up time (49.2 ± 15.6 years), SM-positive was a predictor of earlier HF (NYHA Class III/IV symptoms) after multivariate adjustment (hazard ratio 3.5; 95% confidence interval 1.3–9.7; P = 0.015). Conclusion SM-positive HCM patients had a higher extent of myocardial fibrosis and more severe ventricular diastolic dysfunction than those without, which may contribute to earlier onset of advanced HF, suggesting the importance of close surveillance and early treatment throughout life.

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