scholarly journals Apical hypertrophic cardiomyopathy, are low-risk patients really at low risk? A case report

2020 ◽  
Vol 4 (5) ◽  
pp. 1-4
Author(s):  
Rui Files Flores ◽  
Fernando Mané ◽  
Nuno Antunes ◽  
Vítor Hugo Pereira
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular ◽  
Low Risk ◽  
Bundle Branch Block ◽  
Case Summary ◽  
Risk Patients ◽  
Apical Hypertrophy ◽  
Genetically Determined ◽  
New Onset

Abstract Background Hypertrophic cardiomyopathy (HCM) is a genetically determined myocardial disease that constitutes the main cause of sudden cardiac death (SCD) in young athletes. Apical HCM (ApHCM) represents a complex subset of patients, whose risk of SCD seems not negligible. Most applied scores likely underestimate the risk of heart events in this subset of patients. Case summary We report the case of a 55-year-old man who was admitted in the emergency department after an episode of aborted sudden death due to ventricular fibrillation. The electrocardiogram made at admission was noted for atrial fibrillation and a new-onset left bundle branch block. Emergency coronary angiography was normal. The electrocardiogram was repeated and showed symmetrical and profound inversion of T waves in the lateral leads. Transthoracic echocardiogram and cardiac magnetic resonance revealed left ventricular apical hypertrophy suggestive of apical variant of HCM. A cardiac defibrillator was implanted for secondary prevention of SCD. After 6 months of follow-up no further rhythmic events were noted. Discussion Although low, the risk of SCD of ApHCM patients is not negligible. This case illustrates the need for searching of new predictors of rhythmic risk in patients with ApHCM.

scholarly journals Prognostic impact of atrial fibrillation in patients with hypertrophic cardiomyopathy in a community-based Japanese cohort: results from Kochi RYOMA study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Kubo ◽  
K Sugiura ◽  
Y Ochi ◽  
A Takahashi ◽  
Y Baba ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Hypertrophic Cardiomyopathy ◽  
Adverse Events ◽  
Left Ventricular ◽  
Disease Stage ◽  
Prognostic Impact ◽  
Class Iii ◽  
Community Based ◽  
New Onset

Abstract Background The prognostic impact of atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy (HCM) is not fully elucidated. Purpose The aim of this study was to examine the prevalence and prognostic impact of AF in a prospectively assembled community-based HCM patient cohort in an aged Japanese community. Methods In 2004, we established a cardiomyopathy registration network in Kochi Prefecture, Japan, consisting of 9 hospitals, and finally 293 patients with HCM were followed. Results The ages at registration and at diagnosis were 63±14 and 56±16 years, respectively, and 197 patients (67%) were men. 86 patients (29%) showed AF. During follow-up period of 6.1±3.2 years, 44 patients died. In those patients, HCM-related deaths occurred in 23 patients with an annual mortality rate of 1.3%. Regarding HCM-related adverse events including HCM-related deaths, appropriate ICD discharge, heart failure admission and hospitalization for embolic events, a total of 77 cardiovascular events in 70 patients occurred. Multivariate analysis revealed that presence of AF, left ventricular (LV) outflow obstruction, NYHA functional class III, and lower LV fractional shortening at registration were significant predictors of these adverse events. During the follow-up period, additional 31 patients (11%) developed new-onset AF. Importantly, the incidence of HCM-related adverse events was significantly higher in patients with new AF observed from its onset compared with those with AF at registration (log-rank p=0.029) (Figure 1). Conclusions In an unselected HCM registry in an aged Japanese community, presence of AF, particularly new-onset AF, was associated with unfavorable clinical outcomes. AF is not just a marker of the disease stage but an important trigger of HCM-related adverse events. Figure 1 Funding Acknowledgement Type of funding source: None

Prospective 12-Year Follow-up Study of Clinical and Hemodynamic Sequelae After Deep Vein Thrombosis in Low-Risk Patients (Zürich Study)

Circulation ◽  
1996 ◽  
Vol 93 (1) ◽  
pp. 74-79 ◽  
Author(s):  
Ulrich K. Franzeck ◽  
Ilse Schalch ◽  
Kurt A. Jäger ◽  
Ernst Schneider ◽  
Jörg Grimm ◽  
...  
Keyword(s):  
Deep Vein Thrombosis ◽  
Low Risk ◽  
Vein Thrombosis ◽  
Follow Up Study ◽  
Risk Patients ◽  
Deep Vein

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 Echocardiography and EuroSCORE II for the stratification of low-gradient severe aortic stenosis and preserved left ventricular ejection fraction

2021 ◽  
Author(s):  
Yan Fan ◽  
Hong Shen ◽  
Brandon Stacey ◽  
David Zhao ◽  
Robert J. Applegate ◽  
...  
Keyword(s):  
High Risk ◽  
Left Ventricular Ejection Fraction ◽  
Risk Group ◽  
Severe Aortic Stenosis ◽  
Left Ventricular ◽  
High Risk Group ◽  
Low Risk ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Risk Patients

AbstractThe purpose of this study was to explore the utility of echocardiography and the EuroSCORE II in stratifying patients with low-gradient severe aortic stenosis (LG SAS) and preserved left ventricular ejection fraction (LVEF ≥ 50%) with or without aortic valve intervention (AVI). The study included 323 patients with LG SAS (aortic valve area ≤ 1.0 cm2 and mean pressure gradient < 40 mmHg). Patients were divided into two groups: a high-risk group (EuroSCORE II ≥ 4%, n = 115) and a low-risk group (EuroSCORE II < 4%, n = 208). Echocardiographic and clinical characteristics were analyzed. All-cause mortality was used as a clinical outcome during mean follow-up of 2 ± 1.3 years. Two-year cumulative survival was significantly lower in the high-risk group than the low-risk patients (62.3% vs. 81.7%, p = 0.001). AVI tended to reduce mortality in the high-risk patients (70% vs. 59%; p = 0.065). It did not significantly reduce mortality in the low-risk patients (82.8% with AVI vs. 81.2%, p = 0.68). Multivariable analysis identified heart failure, renal dysfunction and stroke volume index (SVi) as independent predictors for mortality. The study suggested that individualization of AVI based on risk stratification could be considered in a patient with LG SAS and preserved LVEF.

scholarly journals Accuracy of upper endoscopies with random biopsies to identify patients with gastric premalignant lesions who can safely be exempt from surveillance

2021 ◽  
Vol 24 (3) ◽  
pp. 680-690
Author(s):  
Michiel C. Mommersteeg ◽  
Stella A. V. Nieuwenburg ◽  
Wouter J. den Hollander ◽  
Lisanne Holster ◽  
Caroline M. den Hoed ◽  
...  
Keyword(s):  
Gastric Cancer ◽  
High Risk ◽  
Low Risk ◽  
Premalignant Lesions ◽  
High Risk Patients ◽  
European Guidelines ◽  
Progression Of Disease ◽  
Risk Patients ◽  
Progression Risk

Abstract Introduction Guidelines recommend endoscopy with biopsies to stratify patients with gastric premalignant lesions (GPL) to high and low progression risk. High-risk patients are recommended to undergo surveillance. We aimed to assess the accuracy of guideline recommendations to identify low-risk patients, who can safely be discharged from surveillance. Methods This study includes patients with GPL. Patients underwent at least two endoscopies with an interval of 1–6 years. Patients were defined ‘low risk’ if they fulfilled requirements for discharge, and ‘high risk’ if they fulfilled requirements for surveillance, according to European guidelines (MAPS-2012, updated MAPS-2019, BSG). Patients defined ‘low risk’ with progression of disease during follow-up (FU) were considered ‘misclassified’ as low risk. Results 334 patients (median age 60 years IQR11; 48.7% male) were included and followed for a median of 48 months. At baseline, 181/334 (54%) patients were defined low risk. Of these, 32.6% were ‘misclassified’, showing progression of disease during FU. If MAPS-2019 were followed, 169/334 (51%) patients were defined low risk, of which 32.5% were ‘misclassified’. If BSG were followed, 174/334 (51%) patients were defined low risk, of which 32.2% were ‘misclassified’. Seven patients developed gastric cancer (GC) or dysplasia, four patients were ‘misclassified’ based on MAPS-2012 and three on MAPS-2019 and BSG. By performing one additional endoscopy 72.9% (95% CI 62.4–83.3) of high-risk patients and all patients who developed GC or dysplasia were identified. Conclusion One-third of patients that would have been discharged from GC surveillance, appeared to be ‘misclassified’ as low risk. One additional endoscopy will reduce this risk by 70%.

scholarly journals SAT0500 HOW THE ADULT CRISS WORKS IN PEDIATRIC jSSc PATIENTS - RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1206.2-1206
Author(s):  
J. Klotsche ◽  
I. Foeldvari ◽  
O. Kasapcopur ◽  
A. Adrovic ◽  
K. Torok ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Area Under Curve ◽  
Health Assessment ◽  
Organ Damage ◽  
Left Ventricular ◽  
Inception Cohort ◽  
Research Support ◽  
Predicted Probability ◽  
New Onset

Background:The Composite Response Index in Systemic Sclerosis (CRISS) was developed by Dinesh Khanna as a response measure in patients with adult systemic sclerosis. CRISS aims to capture the complexity of systemic sclerosis and to provide a sensitive measure for change in disease activity. The CRISS score is based on a two-step approach. First, significant disease worsening or new-onset organ damage is defined as non-responsiveness. In patients who did not fulfill the criteria of part one, a probability of improvement is calculated for each patient based the Rodnan Skin Score (mRSS), percent predicted forced vital capacity (FVC%), patient and physician global assessments (PGA), and the Health Assessment Questionnaire Disability Index (HAQ-DI). A probability of 0.6 or higher indicates improvement.Objectives:The objective of this study was to validate the CRISS in a prospectively followed cohort of patients with juvenile systemic sclerosis (jSSc).Methods:Data from the prospective international inception cohort for jSSc was used to validate the CRISS. Patients with an available 12-months follow-up were included in the analyses. Clinically improvement was defined by the anchor question about improvement (much better or little better versus almost the same, little worse or much worse) in patients overall health due to scleroderma since the last visit provided by the treating physician.Results:Forty seven jSSc patients were included in the analysis. 74.2% had diffuse subtype. The physician rated the disease as improved in 34 patients (72.3%) since the last visit. No patient had a renal crisis or new onset of left ventricular failure during the 12-months follow-up. Three patients (3.4%) each had a new onset or worsening of lung fibrosis and new onset of pulmonary arterial hypertension. In total, 6 patients resulted in a rating of not improved based on the CRISS in part I. The mRSSS, FVC%, CHAQ and PGA significantly improved during the 12-months follow-up in patients who were rated as improved. The predicted probability based on the CRISS algorithm resulted in an area under curve of 0.77 predicting the anchor question of improvement. In summary, 33 (70.0%) patients were correctly classified by the adult CRISS score resulting in an overall area under curve of 0.7.Conclusion:The CRISS score was evaluated in a pediatric jSSc cohort for the first time. It showed a good performance. However, it seems that the formula of part II of the CRISS score needs a calibration to pediatric jSSc patients.Disclosure of Interests:Jens Klotsche: None declared, Ivan Foeldvari Consultant of: Novartis, Ozgur Kasapcopur: None declared, Amra Adrovic: None declared, Kathryn Torok: None declared, Valda Stanevicha: None declared, Jordi Anton Grant/research support from: grants from Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Grant/research support from: Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Consultant of: Novartis, Sobi, Pfizer, abbvie, Consultant of: Novartis, Sobi, Pfizer, abbvie, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, edoardo marrani: None declared, Maria T. Terreri: None declared, Flávio R. Sztajnbok: None declared, Cristina Battagliotti: None declared, Lillemor Berntson Consultant of: paid by Abbvie as a consultant, Speakers bureau: paid by Abbvie for giving speaches about JIA, Despina Eleftheriou: None declared, Gerd Horneff Grant/research support from: AbbVie, Chugai, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Speakers bureau: AbbVie, Bayer, Chugai, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Farzana Nuruzzaman: None declared, Nicola Helmus: None declared

New onset left bundle branch block after transcatheter aortic valve implantation and the effect on long-term survival – a UK wide experience

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S.S Arri ◽  
A Myat ◽  
I Malik ◽  
N Curzen ◽  
A Baumbach ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Transcatheter Aortic Valve Implantation ◽  
Left Bundle Branch Block ◽  
Left Ventricular ◽  
Bundle Branch Block ◽  
Transcatheter Aortic Valve ◽  
All Cause Mortality ◽  
Aortic Valve Implantation ◽  
Valve Implantation ◽  
New Onset

Abstract Introduction New onset left bundle branch block (LBBB) is the most common conduction disturbance associated with transcatheter aortic valve implantation (TAVI). It has been shown to adversely affect cardiac function and increase re-hospitalisation, although its impact on mortality remains contentious. Methods We conducted an observational cohort analysis of all TAVI procedures performed by 13 heart teams in the United Kingdom from inception of their structural programmes until 31st July 2013. The primary outcome was 1-year all-cause mortality. Secondary outcomes included left ventricular ejection fraction (LVEF) at 30 days and need for a post-TAVI permanent pacemaker (PPM). Results 1785 patients were eligible for inclusion to the study. The primary analysis cohort was composed of 1409 patients with complete electrocardiographic (ECG) data pre- and post-TAVI. Pre-existing LBBB was present in 200 (14.2%) patients. New LBBB occurred in 323 (22.9%) patients post TAVI, which resolved in 99 (7%) patients prior to discharge. A balloon-expandable device was implanted in 968 (69%) patients, whilst 421 (30%) patients received a self-expandable valve. New LBBB was observed in 120 (12.4%) and 192 (45.6%) patients receiving a balloon- or self-expandable prosthesis respectively. Overall 1-year all-cause mortality post TAVI was 18.7%. New onset LBBB was not associated with an increase in 1-year all-cause mortality (p=0.416). Factors that were associated with mortality included an increasing logistic EuroScore (p=0.05), history of previous balloon aortic valvuloplasty (p=0.001), renal impairment (p=0.003), previous myocardial infarction with pre-existing LBBB (p=0.028) and atrial fibrillation (p=0.039). Lower baseline peak and mean AV gradients were also associated with greater mortality at 1 year (p=0.001), likely reflecting underlying left ventricular dysfunction. In the majority of patients, LVEF remained unchanged following TAVI. Interestingly, the presence or absence of new onset LBBB did not affect LVEF improvement at 30 days. 10% of patients required a PPM post TAVI. Predictors of PPM included new LBBB (OR 2.6, p&lt;0.001), pre-TAVI left ventricular systolic impairment (OR 1.2, p=0.037), a self-expandable device (p&lt;0.001), and pre-existing RBBB (OR 4.0, p&lt;0.001). Conclusions These findings suggest that new onset LBBB post TAVI does not increase mortality at 1 year or adversely affect LVEF at 30 days. Funding Acknowledgement Type of funding source: None

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 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.

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