scholarly journals Compound Mutation in Cardiac Sarcomere Proteins Is Associated with Increased Risk for Major Arrhythmic Events in Pediatric Onset Hypertrophic Cardiomyopathy

2021 ◽  
Vol 10 (22) ◽  
pp. 5256
Author(s):  
Kathrin Pollmann ◽  
Emanuel Kaltenecker ◽  
Julia Schleihauf ◽  
Peter Ewert ◽  
Agnes Görlach ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Disease Onset ◽  
Cardiopulmonary Exercise Test ◽  
Left Ventricular ◽  
Single Mutation ◽  
Increased Risk ◽  
Systolic And Diastolic Function ◽  
Cardiac Medication ◽  
Pediatric Onset

Hypertrophic cardiomyopathy (HCM) is associated with adverse left ventricular (LV) remodeling causing dysfunction and malignant arrhythmias. Severely affected patients present with disease onset during childhood and sudden cardiac death risk (SCD) stratification is of the highest importance in this cohort. This study aimed to investigate genotype–phenotype association regarding clinical outcome and disease progression in pediatric onset HCM. Medical charts from forty-nine patients with pediatric HCM who had undergone genetic testing were reviewed for retrospective analysis. Demographic, clinical, transthoracic echocardiographic, electrocardiographic, long-term electrocardiogram, cardiopulmonary exercise test, cardiac magnetic resonance, and medication data were recorded. Childhood onset HCM was diagnosed in 29 males and 20 females. Median age at last follow-up was 18.7 years (range 2.6–51.7 years) with a median follow-up time since diagnosis of 8.5 years (range 0.2–38.0 years). Comparison of patients carrying mutations in distinct genes and comparison of genotype-negative with genotype-positive individuals, revealed no differences in functional classification, LV morphology, hypertrophy, systolic and diastolic function, fibrosis and cardiac medication. Patients with compound mutations had a significantly higher risk for major arrhythmic events than a single-mutation carrier. No association between affected genes and disease severity or progression was identified in this cohort.

Abstract 13943: Hypertrophic Cardiomyopathy with Left Ventricular Apical Aneurysm Represents a High-Risk Subgroup Necessitating Aggressive Preventive Therapy: A Long-term Follow-Up Study

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Ethan J Rowin ◽  
Barry J Maron ◽  
Tammy S Haas ◽  
John R Lesser ◽  
Mark S Link ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Death ◽  
Left Ventricular ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Apical Aneurysm

Background: Increasing penetration of high spatial resolution cardiovascular magnetic resonance (CMR) imaging into routine cardiovascular practice has resulted in more frequent identification of a subset of hypertrophic cardiomyopathy (HCM) patients with thin-walled, scarred left ventricular (LV) apical aneurysms. Prior experience involved relatively small numbers of patients with short follow-up and therefore the risk associated with this subgroup remains incompletely defined. Therefore, we assembled a large HCM cohort with LV apical aneurysms and long-term follow-up in order to clarify clinical course and prognosis. Methods and Results: Of 2,400 HCM patients, 60 (2.5%) were identified by CMR with LV apical aneurysm, 24 to 86 years of age, including 19 (32%) <45 years old; 70% male, and followed for 5.6 ± 3.5 years. Over the follow-up period, 24 patients experienced 31 adverse disease-related complications including: appropriate implantable cardioverter-defibrillator discharge for VT/VF (n=11), received or listed for heart transplant (n=6), heart failure death (n=5), nonfatal thromboembolic events (n=4), resuscitated out-of-hospital cardiac arrest (n=3), and sudden death (n=2). In addition, an intracavitary thrombus was identified in the apical aneurysm in 9 patients without a thromboembolic history. Combined HCM-related death and aborted life threatening event rate was 8.6% per year, nearly 6-fold greater than the 1.5% annual mortality rate reported in the general HCM population. Conclusions: Patients with LV apical aneurysms represent a high-risk subgroup within the diverse HCM spectrum, associated with substantial increased risk for disease-related morbidity and mortality, including advanced heart failure, thromboembolic stroke and sudden death. Identification of this unique HCM phenotype should prompt consideration for primary prevention ICD, and anticoagulation for stroke prophylaxis.

Clinical outcomes in adult athletes with hypertrophic cardiomyopathy: a 7-year follow-up study

2020 ◽  
Vol 54 (16) ◽  
pp. 1008-1012 ◽  
Author(s):  
Antonio Pelliccia ◽  
Stefano Caselli ◽  
Matteo Pelliccia ◽  
Maria Beatrice Musumeci ◽  
Erika Lemme ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Hypertrophic Cardiomyopathy ◽  
Sport Participation ◽  
Sudden Cardiac Arrest ◽  
Left Ventricular ◽  
Rank Test ◽  
Competitive Sport ◽  
Increased Risk ◽  
Exercise Programmes

ObjectiveCurrent guidelines recommend precautionary disqualification from competitive sports in patients with hypertrophic cardiomyopathy (HCM). We assessed the incidence of cardiovascular events in a cohort of patients with HCM engaged in long-term exercise programmes and competitive sport.MethodsWe reviewed data on 88 consecutive athletes diagnosed with HCM, from 1997 to 2017; 92% male, 98% Caucasian, median age 31 (IQR: 19–44) years. All participated in regular exercise programmes and competitive sport at study entry.We performed follow-up evaluation after 7±5 (1–21) years. 61 (69%) of the athletes had substantially reduced or stopped exercise and sport (ie, HCM-detrained), and 27 had continued with regular training and sport competitions (HCM-trained). At baseline evaluation, both groups were similar for age, gender balance, symptoms, ECG abnormalities, extent of left ventricular hypertrophy, arrhythmias and risk profile for sudden cardiac death/arrest.ResultsDuring the follow-up period, two participants suffered sudden cardiac arrest or death (0.3% per year) both outside of sport participation. In addition, 19 (22%) reported symptoms (syncope in 3, palpitations in 10, chest pain in 4 and dyspnoea in 2). The Kaplan-Meier analyses of freedom from combined sudden cardiac arrest/death and symptoms (log-rank test p=0.264) showed no differences between HCM-trained and detrained patients.ConclusionIn this adult cohort of low-risk HCM athletes, voluntary decision to pursue in participation in competitive sport events was not associated with increased risk for major cardiac events or clinical worsening compared with decision to reduce or withdraw from exercise programmes and sport. Similar results may not be seen in younger or racially diverse athlete populations, or in patients with more severe HCM phenotypes.

scholarly journals 382 Clinical manifestation and prognosis of different cardiomyopathies on the base of genetic background (GEN-PHEN)

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Alessia Paldino ◽  
Davide Stolfo ◽  
Matteo Dal Ferro ◽  
Marta Gigli ◽  
Kristen Medo ◽  
...  
Keyword(s):  
Heart Disease ◽  
Heart Transplant ◽  
Total Population ◽  
Multivariable Analysis ◽  
Disease Onset ◽  
Left Ventricular ◽  
Prognostic Impact ◽  
Increased Risk

Abstract Aims Cardiomyopathies (CMP) are a heterogeneous group of heart disease characterized by structural and electrical abnormalities with no other secondary causative etiology and frequently related to mutations in disease related genes. Clinical evaluation and phenotype definition are the key factors for clinical classification of CMPs. Recent studies in this field have showed important phenotype overlaps between Dilated Cardiomyopathy (DCM) and Arrhythmogenic Cardiomyopathy (ACM), making the diagnosis a challenging task. The aim of this study is to assess whether a classification of CMP patients (not hypertrophic) based on genetic characterization outperforms in diagnostic and prognostic accuracy the classical, phenotype-driven, diagnostic approach. Methods and results We analysed a population of patients affected by genetically determined DCM and ACM, carriers of ‘pathogenic’ or ‘likely pathogenic’ (P/LP) variants, registered into the Heart Disease Centers of Trieste and Denver hospitals. First, we described the phenotype distribution in our population with a clinical and echocardiographic evaluation based on the different disease-related mutated genes. Then, we examined the prognostic impact of the single gene/genetic cluster in assessing these composite outcomes: (1) all-cause mortality and heart transplant; (2) heart failure-related death, heart transplant or destination left ventricular assist device implantation (DHF/HTx/VAD); and (3) sudden cardiac death, sustained ventricular tachycardia/ventricular fibrillation or appropriate defibrillator shock (SCD/VT/VF/shock). 281 patients carrying P/LP variants (82% DCM) were included in the study. Titin (TTN) and sarcomeric genes (SARC) variants were the most prevalent (TTN: 95 patients, 34% of total population; SARC: 63 patients, 22% of total population) and almost completely related to DCM phenotype (TTN: 100% DCM, SARC: 95% DCM), such as lamin (LMNA) patients (29 patients, 10% of total population, 96% DCM). A more heterogeneous phenotypic distribution between DCM and ACM were detected for desmoplakin (DSP), plakoglobin (PKP2), and filamin (FLNC) variants. Patients with not isolated DCM phenotype and patients’ carriers of DSP, PKP2, FLNC and LMNA variants (arrhythmic genes) experienced more frequent SCD/VT/VF/shock events (P-value = 0.002 and P = 0.023), compared respectively to patients with DCM phenotype and to patients’ carriers of TTNtv and SARC variants, during follow-up (median time 132 months). The multivariable analysis shown that only P/LP variants of arrhythmic genes, younger age of disease onset and male gender, were associated with an increased risk of SCD/VT/VF/shock events during follow-up. No difference in terms of HF events was significantly related to genotype. Conclusions Our study demonstrated that the classification based on the presence of a specific genotype outperforms phenotypic classification in terms of arrhythmic risk in a large DCM and ACM population with a positive genetic test for P/LP variants. These findings support the need of extensive genetic testing to support CMP diagnosis and prognosis.

P2457Left atrial strain parameters predict clinical outcomes in patients with HFmrEF and HFpEF

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Vrettos ◽  
L Al Saikhan ◽  
L Matiti ◽  
C Plymen ◽  
B Low ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Diastolic Function ◽  
Left Atrial ◽  
Left Ventricular ◽  
Baseline Characteristic ◽  
Operating Characteristics ◽  
Increased Risk ◽  
Echocardiographic Parameters ◽  
Systolic And Diastolic Function

Abstract Background Heart failure with preserved ejection fraction (HFpEF) and with mid-range ejection fraction (HFmrEF) are conditions sharing similar diagnostic criteria and symptoms. Left ventricular (LV) diastolic dysfunction has often been considered central to the pathophysiology of both, as determined by current conventional echocardiographic measures of elevated filling pressure and/or an increased in left atrial (LA) volume. However, the independent prognostic importance of LA function, beyond clinical predictors and LV features, in those groups remains unclear. Aims To evaluate the prognostic value of LA function measured by speckle tracking echocardiography in patients with new diagnosis of HFpEF and HFmrEF. Methods A total of 171 consecutive patients diagnosed with HFpEF with an Ejection Fraction (EF) ≥50%, or HFmrEF with an EF of 40–49% were prospectively enrolled and followed up. Follow-up was completed in 134 patients with HFpEF (n=100) and HFmrEF (n=34) for a median period of 57 months. Baseline characteristic and comorbidities were recorded, as well as conventional resting echocardiographic parameters of systolic and diastolic function. Left atrial phasic function was analysed using 2D-Specle Tracking. Peak-atrial longitudinal strain (PALS) and peak-atrial contraction strain (PACS) were measured, reflecting LA reservoir and pump function respectively. We looked at the relationship of traditional parameters of systolic and diastolic function and LA strain, with the following cardiovascular (CV) events: acute coronary syndromes, new atrial fibrillation, cerebrovascular events, HF-related admissions and cardiac deaths. Results During a median follow up period of 57 months (range 11.10), of 134 patients, 42 (31%) had at least one CV event, 10 (8%) out of whom had at least one HF-related hospitalization and 5 (4%) had cardiac death. 92 patients (69%) remained event-free. In univariate analysis, lower PALS and PACS were associated with increased risk for the composite endpoint of all CV-events (PALS OR=0.92 p<0.01 and PACS OR=0.92 p<0.05), but only PALS predicted HF admissions (PALS OR=0.86 p<0.05). In multivariate analysis after adjusting for LV EF, E/e' and LAVi, PALS remained significant at predicting CV-events (OR=0.91 p<0.05) and HF admissions (OR=0.85 p<0.05), but PACS did not. Amongst all echocardiographic parameters measured, PALS had the greatest Area Under the Curve at Receiver Operating Characteristics analysis (AUC=0.78 p<0.05) and a PALS value of <23% best predicted future HF-related admissions (sensitivity 0.9, specificity 0.6). Conclusion LA dysfunction in HFpEF and HFmrEF is associated with a higher risk of CV events and HF hospitalizations. The association of LA strain with HF hospitalization remained significant after adjustment for systolic and diastolic parameters.

scholarly journals POSTERS (2)96CONTINUOUS VERSUS INTERMITTENT MONITORING FOR DETECTION OF SUBCLINICAL ATRIAL FIBRILLATION IN HIGH-RISK PATIENTS97HIGH DAY-TO-DAY INTRA-INDIVIDUAL REPRODUCIBILITY OF THE HEART RATE RESPONSE TO EXERCISE IN THE UK BIOBANK DATA98USE OF NOVEL GLOBAL ULTRASOUND IMAGING AND CONTINUEOUS DIPOLE DENSITY MAPPING TO GUIDE ABLATION IN MACRO-REENTRANT TACHYCARDIAS99ANTICOAGULATION AND THE RISK OF COMPLICATIONS IN PATIENTS UNDERGOING VT AND PVC ABLATION100NON-SUSTAINED VENTRICULAR TACHYCARDIA FREQUENTLY PRECEDES CARDIAC ARREST IN PATIENTS WITH BRUGADA SYNDROME101USING HIGH PRECISION HAEMODYNAMIC MEASUREMENTS TO ASSESS DIFFERENCES IN AV OPTIMUM BETWEEN DIFFERENT LEFT VENTRICULAR LEAD POSITIONS IN BIVENTRICULAR PACING102CAN WE PREDICT MEDIUM TERM MORTALITY FROM TRANSVENOUS LEAD EXTRACTION PRE-OPERATIVELY?103PREVENTION OF UNECESSARY ADMISSIONS IN ATRIAL FIBRILLATION104EPICARDIAL CATHETER ABLATION FOR VENTRICULAR TACHYCARDIA ON UNINTERRUPTED WARFARIN: A SAFE APPROACH?105HOW WELL DOES THE NATIONAL INSTITUTE OF CLINICAL EXCELLENCE (NICE) GUIDENCE ON TRANSIENT LOSS OF CONSCIOUSNESS (T-LoC) WORK IN A REAL WORLD? AN AUDIT OF THE SECOND STAGE SPECIALIST CARDIOVASCULAT ASSESSMENT AND DIAGNOSIS106DETECTION OF ATRIAL FIBRILLATION IN COMMUNITY LOCATIONS USING NOVEL TECHNOLOGY'S AS A METHOD OF STROKE PREVENTION IN THE OVER 65'S ASYMPTOMATIC POPULATION - SHOULD IT BECOME STANDARD PRACTISE?107HIGH-DOSE ISOPRENALINE INFUSION AS A METHOD OF INDUCTION OF ATRIAL FIBRILLATION: A MULTI-CENTRE, PLACEBO CONTROLLED CLINICAL TRIAL IN PATIENTS WITH VARYING ARRHYTHMIC RISK108PACEMAKER COMPLICATIONS IN A DISTRICT GENERAL HOSPITAL109CARDIAC RESYNCHRONISATION THERAPY: A TRADE-OFF BETWEEN LEFT VENTRICULAR VOLTAGE OUTPUT AND EJECTION FRACTION?110RAPID DETERIORATION IN LEFT VENTRICULAR FUNCTION AND ACUTE HEART FAILURE AFTER DUAL CHAMBER PACEMAKER INSERTION WITH RESOLUTION FOLLOWING BIVENTRICULAR PACING111LOCALLY PERSONALISED ATRIAL ELECTROPHYSIOLOGY MODELS FROM PENTARAY CATHETER MEASUREMENTS112EVALUATION OF SUBCUTANEOUS ICD VERSUS TRANSVENOUS ICD- A PROPENSITY MATCHED COST-EFFICACY ANALYSIS OF COMPLICATIONS & OUTCOMES113LOCALISING DRIVERS USING ORGANISATIONAL INDEX IN CONTACT MAPPING OF HUMAN PERSISTENT ATRIAL FIBRILLATION114RISK FACTORS FOR SUDDEN CARDIAC DEATH IN PAEDIATRIC HYPERTROPHIC CARDIOMYOPATHY: A SYSTEMATIC REVIEW AND META-ANALYSIS115EFFECT OF CATHETER STABILITY AND CONTACT FORCE ON VISITAG DENSITY DURING PULMONARY VEIN ISOLATION116HEPATIC CAPSULE ENHANCEMENT IS COMMONLY SEEN DURING MR-GUIDED ABLATION OF ATRIAL FLUTTER: A MECHANISTIC INSIGHT INTO PROCEDURAL PAIN117DOES HIGHER CONTACT FORCE IMPAIR LESION FORMATION AT THE CAVOTRICUSPID ISTHMUS? INSIGHTS FROM MR-GUIDED ABLATION OF ATRIAL FLUTTER118CLINICAL CHARACTERISATION OF A MALIGNANT SCN5A MUTATION IN CHILDHOOD119RADIOFREQUENCY ASSOCIATED VENTRICULAR FIBRILLATION120CONTRACTILE RESERVE EXPRESSED AS SYSTOLIC VELOCITY DOES NOT PREDICT RESPONSE TO CRT121DAY-CASE DEVICES - A RETROSPECTIVE STUDY USING PATIENT CODING DATA122PATIENTS UNDERGOING SVT ABLATION HAVE A HIGH INCIDENCE OF SECONDARY ARRHYTHMIA ON FOLLOW UP: IMPLICATIONS FOR PRE-PROCEDURE COUNSELLING123PROGNOSTIC ROLE OF HAEMOGLOBINN AND RED BLOOD CELL DITRIBUTION WIDTH IN PATIENTS WITH HEART FAILURE UNDERGOING CARDIAC RESYNCHRONIZATION THERAPY124REMOTE MONITORING AND FOLLOW UP DEVICES125A 20-YEAR, SINGLE-CENTRE EXPERIENCE OF IMPLANTABLE CARDIOVERTER DEFIBRILLATORS (ICD) IN CHILDREN: TIME TO CONSIDER THE SUBCUTANEOUS ICD?126EXPERIENCE OF MAGNETIC REASONANCE IMAGING (MEI) IN PATIENTS WITH MRI CONDITIONAL DEVICES127THE SINUS BRADYCARDIA SEEN IN ATHLETES IS NOT CAUSED BY ENHANCED VAGAL TONE BUT INSTEAD REFLECTS INTRINSIC CHANGES IN THE SINUS NODE REVEALED BY I (F) BLOCKADE128SUCCESSFUL DAY-CASE PACEMAKER IMPLANTATION - AN EIGHT YEAR SINGLE-CENTRE EXPERIENCE129LEFT VENTRICULAR INDEX MASS ASSOCIATED WITH ESC HYPERTROPHIC CARDIOMYOPATHY RISK SCORE IN PATIENTS WITH ICDs: A TERTIARY CENTRE HCM REGISTRY130A DGH EXPERIENCE OF DAY-CASE CARDIAC PACEMAKER IMPLANTATION131IS PRE-PROCEDURAL FASTING A NECESSITY FOR SAFE PACEMAKER IMPLANTATION?

EP Europace ◽  
2016 ◽  
Vol 18 (suppl 2) ◽  
pp. ii36-ii47
Author(s):  
T. Philippsen ◽  
M. Orini ◽  
C.A. Martin ◽  
E. Volkova ◽  
J.O.M. Ormerod ◽  
...  
Keyword(s):  
Heart Failure ◽  
Atrial Fibrillation ◽  
Ventricular Tachycardia ◽  
Hypertrophic Cardiomyopathy ◽  
Pacemaker Implantation ◽  
Left Ventricular ◽  
Day Case ◽  
Single Centre ◽  
Subcutaneous Icd

scholarly journals Subclinical Cardiac Organ Damage in Patients with Moderate to Severe Psoriasis

2021 ◽  
Vol 10 (11) ◽  
pp. 2440
Author(s):  
Anja Linde ◽  
Eva Gerdts ◽  
Kåre Steinar Tveit ◽  
Ester Kringeland ◽  
Helga Midtbø
Keyword(s):  
Body Mass Index ◽  
Body Mass ◽  
Treatment Initiation ◽  
Severity Index ◽  
Organ Damage ◽  
Left Ventricular ◽  
Infliximab Treatment ◽  
Increased Risk ◽  
Psoriasis Severity

We explored the association between subclinical cardiac organ damage (OD) with comorbidities and psoriasis severity in 53 psoriasis patients on infliximab treatment (age 47 ± 15 years, 30% women) and 99 controls without psoriasis (age 47 ± 11 years, 28% women). Cardiac OD was assessed by echocardiography as the presence of increased left ventricular (LV) relative wall thickness (RWT), LV hypertrophy or dilated left atrium. Psoriasis severity was graded using the psoriasis area and severity index (PASI). The prevalence of hypertension was 66% in psoriasis vs. 61% in controls (p = 0.54) and cardiac OD seen in 51 and 73%, respectively (p = 0.007). Psoriasis was associated with a lower prevalence of cardiac OD (odds ratio (OR) 0.32, 95% confidence interval (CI) 0.13–0.77, p = 0.01) independent of age, sex, smoking, body mass index, and hypertension. Among psoriasis patients, hypertension was associated with increased risk of subclinical cardiac OD (OR 6.88, 95% CI 1.32–35.98, p = 0.02) independent of age, sex, and body mass index. PASI at treatment initiation was associated with a higher RWT at follow-up, independent of sex, age, and hypertension (β 0.36, p = 0.006) while no association with current PASI was found. In conclusion, cardiac OD was less prevalent in psoriasis patients on infliximab treatment than controls. Hypertension was the major covariable for subclinical cardiac OD in psoriasis.

Global longitudinal strain predicts outcome in chronic heart failure across American Heart Association stages: results from the MyoVasc study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S.O Troebs ◽  
A Zitz ◽  
S Schwuchow-Thonke ◽  
A Schulz ◽  
M.W Heidorn ◽  
...  
Keyword(s):  
American Heart Association ◽  
Prognostic Value ◽  
Longitudinal Strain ◽  
American Heart ◽  
Cox Regression ◽  
Global Longitudinal Strain ◽  
Heart Association ◽  
Increased Risk ◽  
Systolic And Diastolic Function

Abstract Background Global longitudinal strain (GLS) demonstrated a superior prognostic value over left ventricular ejection fraction (LVEF) in acute heart failure (HF). Its prognostic value across American Heart Association (AHA) stages of HF – especially under considering of conventional echocardiographic measures of systolic and diastolic function – has not yet been comprehensively evaluated. Purpose To evaluate the prognostic value of GLS for HF-specific outcome across AHA HF stages A to D. Methods Data from the MyoVasc-Study (n=3,289) were analysed. Comprehensive clinical phenotyping was performed during a five-hour investigation in a dedicated study centre. GLS was measured offline utilizing QLab 9.0.1 (PHILIPS, Germany) in participants presenting with sinus rhythm during echocardiography. Worsening of HF (comprising transition from asymptomatic to symptomatic HF, HF hospitalization, and cardiac death) was assessed during a structured follow-up with subsequent validation and adjudication of endpoints. AHA stages were defined according to current guidelines. Results Complete information on GLS was available in 2,400 participants of whom 2,186 categorized to AHA stage A to D were available for analysis. Overall, 434 individuals were classified as AHA stage A, 629 as stage B and 1,123 as stage C/D. Mean GLS increased across AHA stages of HF: it was lowest in stage A (−19.44±3.15%), −18.01±3.46% in stage B and highest in AHA stage C/D (−15.52±4.64%, P for trend &lt;0.0001). During a follow-up period of 3.0 [1.3/4.0] years, GLS denoted an increased risk for worsening of HF after adjustment for age and sex (hazard ratio, HRGLS [per standard deviation (SD)] 1.97 [95% confidence interval 1.73/2.23], P&lt;0.0001) in multivariable Cox regression analysis. After additional adjustment for cardiovascular risk factors, clinical profile, LVEF and E/E' ratio, GLS was the strongest echocardiographic predictor of worsening of HF (HRGLS [per SD] 1.47 [1.20/1.80], P=0.0002) in comparison to LVEF (HRLVEF [per SD] 1.23 [1.02/1.48], P=0.031) and E/E' ratio (HRE/E' [per SD] 1.12 [0.99/1.26], P=0.083). Interestingly, when stratifying for AHA stages, GLS denoted a similar increased risk for worsening of HF in individuals classified as AHA stage A/B (HRGLS [per SD] 1.63 [1.02/2.61], P=0.039) and in those classified as AHA stage C/D (HRGLS [per SD] 1.95 [1.65/2.29], P&lt;0.0001) after adjustment for age and sex. For further evaluation, Cox regression models with interaction analysis indicated no significant interaction for (i) AHA stage A/B vs C/D (P=0.83) and (ii) NYHA functional class &lt;II vs ≥II in individuals classified as AHA stage C/D (P=0.12). Conclusions GLS demonstrated a higher predictive value for worsening of HF than conventional echocardiographic measures of systolic and diastolic function. Interestingly, GLS indicated an increased risk for worsening of HF across AHA stages highlighting its potential value to advance risk prediction in chronic HF. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): German Center for Cardiovascular Research (DZHK), Center for Translational Vascular Biology (CTVB) of the University Medical Center of the Johannes Gutenberg-University Mainz

Incidence and predictors of epilepsy in children with congenital heart disease

2021 ◽  
pp. 1-7
Author(s):  
Carlos Castillo-Pinto ◽  
Jessica L. Carpenter ◽  
Mary T. Donofrio ◽  
Anqing Zhang ◽  
Gil Wernovsky ◽  
...  
Keyword(s):  
Hospital Length ◽  
Left Ventricular ◽  
Hospital Length Of Stay ◽  
Patient Specific ◽  
Ventricular Assist ◽  
Acute Seizures ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Arterial Ischaemic Stroke

Abstract Objective: Children with CHD may be at increased risk for epilepsy. While the incidence of perioperative seizures after surgical repair of CHD has been well-described, the incidence of epilepsy is less well-defined. We aim to determine the incidence and predictors of epilepsy in patients with CHD. Methods: Retrospective cohort study of patients with CHD who underwent cardiopulmonary bypass at <2 years of age between January, 2012 and December, 2013 and had at least 2 years of follow-up. Clinical variables were extracted from a cardiac surgery database and hospital records. Seizures were defined as acute if they occurred within 7 days after an inciting event. Epilepsy was defined based on the International League Against Epilepsy criteria. Results: Two-hundred and twenty-one patients were identified, 157 of whom were included in our analysis. Five patients (3.2%) developed epilepsy. Acute seizures occurred in 12 (7.7%) patients, only one of whom developed epilepsy. Predictors of epilepsy included an earlier gestational age, a lower birth weight, a greater number of cardiac surgeries, a need for extracorporeal membrane oxygenation or a left ventricular assist device, arterial ischaemic stroke, and a longer hospital length of stay. Conclusions: Epilepsy in children with CHD is rare. The mechanism of epileptogenesis in these patients may be the result of a complex interaction of patient-specific factors, some of which may be present even before surgery. Larger long-term follow-up studies are needed to identify risk factors associated with epilepsy in these patients.

Abstract 19346: Peak Oxygen Consumption Predicts Outcome in Hypertrophic Cardiomyopathy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Carla Contaldi ◽  
Raffaella Lombardi ◽  
Alessandra Giamundo ◽  
Sandro Betocchi
Keyword(s):  
Heart Failure ◽  
Oxygen Consumption ◽  
Hypertrophic Cardiomyopathy ◽  
Nyha Class ◽  
Left Ventricular ◽  
Exercise Intolerance ◽  
Peak Oxygen Consumption ◽  
Class Iii ◽  
Asymptomatic Patients

Introduction: Peak oxygen consumption (VO 2 ) has a strong and independent prognostic value in systolic heart failure; in contrast no data support its prognostic role in hypertrophic cardiomyopathy (HCM). Hypothesis: We assess if peak VO 2 is a long-term predictor of outcome in HCM. Methods: We studied 92 HCM patients (40±15 years). Peak VO 2 was expressed as percentage (%) of the predicted value. Follow up was 76±57 months. The primary composite endpoint (CE) was atrial fibrillation, progression to NYHA class III or IV, myotomy-myectomy (MM), heart transplantation (HT) and cardiac death. An ancillary endpoint (HFE) included markers of heart failure (progression to NYHA class III or IV, MM and HT). Results: At baseline, 62% of patients were asymptomatic, 35% NYHA class II and 3% NYHA class III; 26% had left ventricular outflow tract obstruction. During follow up, 30 patients met CE with 43 events. By multivariate Cox survival analysis, we analyzed 2 models, using the CE, and in turn HFE. For CE, maximal left atrial diameter (LAD) (HR: 1.12; 95% CI: 1.04 to 1.22), maximal wall thickness (MWT) (HR: 0.14; 95% CI: 1.04 to 1.23) and % predicted peak VO 2 (HR: -0.03; 95% CI: 0.95 to 0.99) independently predicted outcome (overall, p<0.0001). For HFE, maximal LAD (HR:0.31; 95% CI: 1.09 to 1.70), MWT (HR: 0.35; 95% CI: 1.08 to 1.84) and % predicted peak VO 2 (HR: -0.06; 95% CI: 0.89 to 0.98) independently predicted outcome (overall, p<0.0001). Only 19% of mildly symptomatic or asymptomatic patients with % predicted peak VO 2 >80% had events, as opposed to 53% of them with % predicted peak VO 2 < 55% (p= 0.04). Event-free survival for both endpoints was significantly lower in patients with % predicted peak VO 2 < 55% as compared to those with it between 55 and 80 and >80% , Figure. Conclusion: In mildly or asymptomatic patients severe exercise intolerance may precede clinical deterioration. In HCM, peak VO 2 provides excellent risk stratification with a high event rate in patients with % predicted value <55%.

Abstract 15775: The Impact of B-type Natriuretic Peptide Activation on the Association of Left Ventricular Longitudinal Strain With All-cause Mortality

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Zi Ye ◽  
Maurice Enriquez-Sarano ◽  
Joseph Malouf ◽  
Hector I Michelena ◽  
Allan S Jaffe ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Natriuretic Peptide ◽  
Longitudinal Strain ◽  
Left Ventricular ◽  
Aortic Valve Area ◽  
Increased Risk ◽  
Highly Correlated ◽  
The Impact ◽  
Normal Lvef

Introduction: Left ventricular longitudinal strain (LV-LS) 1) predicts mortality in patients with aortic stenosis (AS) and 2) is highly correlated to type-B natriuretic peptide (BNP) values. The BNP ratio (measured BNP/maximal expected BNP value specific for age and sex) is a powerful independent predictor of death in patients with AS. Hypothesis: we hypothesize that BNP activation (i.e. BNP ratio >1) affects the association between LV-LS and mortality in patients with asymptomatic AS and preserved LV ejection fraction (EF ≥50%). Methods: 315 patients (age 74±12 years, 56% men and mean aortic valve area = 1.02±0.15cm2) underwent simultaneous Doppler echocardiographic and BNP measurements. LV-LS was calculated as the average of 12 LV segments from apical 2- and 4-chamber views using Velocity Vector Imaging. Results: Mean LV-LS was -16.8±3.2%, LV EF 66±7%, median BNP level 121 (interquartile 48-320) pg/ml. 58% of patients had BNP activation. Better LV-LS was associated with lower log BNPratio (regression coefficient 0.10, p<0.001). After a median follow-up of 6.5 yrs (interquartile: 3.6-8.2), 119 deaths occurred. After adjustment for age, sex, Charlson score index, hemoglobin level, aortic valve replacement (as a time dependent variable), LV-LS and log BNPratio were separately associated with increased risk for death (all p<0.01). Further adjustment for predictors of mortality, LV-LS and log-BNP ratio remained associated with increased risk for death (hazard ratio HR [95%CI]: 1.09 [1.03-1.15]; p=0.003 and 1.82 [1.52-2.19]; p<0.0001 respectively). In patients without BNP activation (i.e. normal BNP), LV-LS was associated with mortality (HR: 1.22 [1.04-1.43]; p=0.01) while it was not in patients with BNP activation (p=0.22). Conclusions: In patients with asymptomatic AS, without clinically obvious myocardial impairment (i.e. normal LVEF), a notable proportion of patients present with myocardial alterations detected by an elevated BNPratio or reduced LV-LS. These signs of myocardial alterations were predictive of mortality after diagnosis. Thus both BNP and LV-LS should be assessed in the clinical setting to provide complementary information on prognosis in patients with asymptomatic AS and preserved LV EF.

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