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%.

Examining the relationship between exercise tolerance and isoproterenol-based cardiac reserve in murine models of heart failure

2013 ◽  
Vol 114 (9) ◽  
pp. 1202-1210 ◽  
Author(s):  
Daniel A. Richards ◽  
Weike Bao ◽  
Mary V. Rambo ◽  
Mark Burgert ◽  
Beat M. Jucker ◽  
...  
Keyword(s):  
Heart Failure ◽  
Oxygen Consumption ◽  
Ejection Fraction ◽  
Exercise Tolerance ◽  
Exercise Intolerance ◽  
Peak Oxygen Consumption ◽  
Cardiac Reserve ◽  
Magnetic Resonance Imaging Mri ◽  
The Relationship

The loss of cardiac reserve is, in part, responsible for exercise intolerance in late-stage heart failure (HF). Exercise tolerance testing (ETT) has been performed in mouse models of HF; however, treadmill performance and at-rest cardiac indexes determined by magnetic resonance imaging (MRI) rarely correlate. The present study adopted a stress-MRI technique for comparison with ETT in HF models, using isoproterenol (ISO) to evoke cardiac reserve responses. Male C57BL/6J mice were randomly subjected to myocardial infarction (MI), transverse aortic constriction (TAC), or sham surgery under general anesthesia. Mice underwent serial ETT on a graded treadmill with follow-up ISO stress-MRI. TAC mice showed consistent exercise intolerance, with a 16.2% reduction in peak oxygen consumption vs. sham at 15-wk postsurgery (WPS). MI and sham mice had similar peak oxygen consumption from 7 WPS onward. Time to a respiratory exchange ratio of 1.0 correlated with ETT distance ( r = 0.64; P < 0.001). The change in ejection fraction under ISO stress was reduced in HF mice at 4 WPS [10.1 ± 3.9% change (Δ) and 8.9 ± 3.5%Δ in MI and TAC, respectively, compared with 32.0 ± 3.5%Δ in sham; P < 0.001]. However, cardiac reserve differences between surgery groups were not observed at 16 WPS in terms of ejection fraction or cardiac output. In addition, ETT did not correlate with cardiac indexes under ISO stress. In conclusion, ISO stress was unable to reflect consistent differences in ETT between HF and healthy mice, suggesting cardiac-specific indexes are not the sole factors in defining exercise intolerance in mouse HF models.

Estimation of the right atrial pressure by ultrasound-assessed internal jugular vein in patients with advanced chronic heart failure

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
E Ammirati ◽  
D Marchetti ◽  
G Colombo ◽  
A Garascia ◽  
F Macera ◽  
...  
Keyword(s):  
Heart Failure ◽  
Chronic Heart Failure ◽  
Internal Jugular Vein ◽  
Validation Cohort ◽  
Jugular Vein ◽  
Nyha Class ◽  
Left Ventricular ◽  
Right Atrial ◽  
Class Iii

Abstract Background In patients with systolic chronic heart failure (CHF) clinical signs of congestion cannot always be evident at clinical examination. Right atrial pressure (RAP) measured by right heart catheterization (RHC) is an accurate and reproducible marker of blood volume. A non-invasive accurate tool to identify CHF patients with normal RAP would be desirable to tailor therapy. Purpose To validate an ultrasound (US)-assessed internal jugular vein distensibility (JVD) ratio to identify patients with normal mean RAP (defined as 7 mmHg or less) measured by RHC. Methods We first identify the JVD ratio that allows the most accurate identification of patients with normal RAP in a prospective calibration cohort of 100 patients with systolic CHF. Then, we tested the JVD ratio threshold to identify patients with normal RAP in a validation cohort of 101 consecutive patients with systolic CHF. All patients had a left ventricular ejection fraction (LVEF)&lt;50% and underwent RHC in the setting of heart transplant work-up. At the time of jugular vein puncture, we recorded the internal jugular vein diameter by conventional linear probes. JVD ratio was calculated as the ratio between maximum diameter (during Valsalva maneuver) and rest diameter of the vein (FIGURE). Finally, we assessed the prognostic value of the JVD ratio in the follow up of the first 100 patients. Results In the calibration cohort (mean age 53 years, 13% female; median LVEF 25%, 81% in NYHA class III/IV) we define the best threshold of the JVD ratio to identify patients with normal RAP that has 1.6 with an area under the curve (AUC of 0.74; p&lt;0.0001). Based on this JVD ratio threshold we defined patients with low JVD ratio (≤1.6; n=58; median RAP 8 mmHg) and patients with high JVD ratio (&gt;1.6, n=42; median RAP 4 mmHg). High JVD ratio and low JVD ratio groups had similar clinical and laboratory characteristics. In the validation cohort (mean age 55 years, 13% female; median LVEF 25%; 56% in NYHA class III/IV) using the previously defined 1.6 JVD ratio threshold, we identified 51 patients with low JVD ratio (median RAP 8 mmHg) and 50 patients with high JVD ratio (median RAP 3 mmHg; p&lt;0.0001) The JVD ratio threshold has an accuracy to identify patients with a normal RAP with an AUC of 0.82 (p&lt;0.0001); a predictive positive value of 0.94, negative predictive value of 0.51, specificity of 0.90, and sensitivity of 0.65. Finally, in the calibration cohort, the CHF patients with low JVD ratio (≤1.6) had a higher cumulative incidence of overall death, heart transplant, or left ventricular assist device (42.7% vs. 16.1% in the high JVD ratio group, p log-rank 0.006) at a median of 13-month follow-up. Conclusions We found that US-assessed JVD ratio is a convenient and accurate diagnostic tool to identify patients with advanced systolic CHF with normal vs. increased RAP. This tool could be tested in the ambulatory setting to modulate therapies, particularly diuretics and vasodilators. Figure 1 Funding Acknowledgement Type of funding source: None

Abstract 17341: Obesity is Associated With Progressive Heart Failure in Hypertrophic Cardiomyopathy

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Aadhavi Sridharan ◽  
Martin S Maron ◽  
Dou Huang ◽  
Craig Cooper ◽  
Jennifer Drummond ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Disease Progression ◽  
Nyha Class ◽  
Class I ◽  
Class Iii ◽  
Progressive Heart Failure ◽  
The Impact ◽  
New Onset

Background: The impact of comorbid disease states and lifestyle on the natural history of patients with hypertrophic cardiomyopathy (HCM) remains unknown. Objective: Evaluate the association of non-HCM comorbidities including obesity, hypertension, diabetes, obstructive sleep apnea, kidney disease, tobacco use, alcohol use, and lung disease with disease progression in a large cohort of HCM patients. Methods: 2269 patients evaluated at the Tufts HCM Institute between 2004 to 2019, ≥ 18 years of age (54 ± 15 years; 1392 male), and followed for an average of 4 ± 3.4 years for disease progression including progressive heart failure (HF) symptoms (from NYHA class I/II to NYHA class III/IV), new-onset atrial fibrillation (AF), or sudden death (SD) event (including appropriate defibrillation for ventricular arrhythmias, resuscitated cardiac arrest, or SD). Results: Of 1376 patients with NYHA class I/II symptoms at initial clinical evaluation, 252 (18%) developed progressive HF symptoms to NYHA class III/IV over follow-up (5%/year). Obesity (BMI ≥ 30) was significantly more prevalent in patients who had progressive HF during follow-up (43%) compared to those who remained without HF (34%, p = 0.014). In contrast, other comorbidities were not significantly associated with progressive HF symptoms (p > 0.10 for all other comorbidities). Of the 1823 patients without AF history at initial clinical visit, 198 (11%) developed new-onset AF over follow-up (3%/year). No comorbidities were significantly associated with new-onset AF in HCM (p > 0.10), although obesity was more common in patients who developed new-onset AF (48%) compared to those who had no AF (41%, p = 0.08). Notably, SD events were not associated with non-HCM comorbidities (p > 0.10 for all comorbidities), and patients with SD events were less likely to have comorbidities than patients without SD events. Conclusions: In adult HCM patients, obesity is associated with progressive symptoms and outcomes supporting weight loss as an important modifier in obese HCM patients to potentially help prevent HCM complications. In contrast, other non-HCM comorbidities do not appear to impact disease course, and SD events are not associated with comorbidities in HCM.

Peak Oxygen Consumption and Resting Left Ventricular Ejection Fraction Changes After Cardiomyoplasty at 6-Month Follow-up

Circulation ◽  
1995 ◽  
Vol 92 (9) ◽  
pp. 216-222 ◽  
Author(s):  
Edimar Alcides Bocchi ◽  
Guilherme Veiga Guimarães ◽  
Luiz Felipe P. Moreira ◽  
Fernando Bacal ◽  
Alvaro Vilela de Moraes ◽  
...  
Keyword(s):  
Oxygen Consumption ◽  
Left Ventricular Ejection Fraction ◽  
Ejection Fraction ◽  
Left Ventricular ◽  
Ventricular Ejection ◽  
Peak Oxygen Consumption ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction

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 P-162 Transesophageal left ventricular pacing with different pacing mode in heart failure patients with NYHA class III

EP Europace ◽  
2003 ◽  
Vol 4 (Supplement_2) ◽  
pp. B105-B105
Author(s):  
M. Heinke ◽  
H. Kuhnert ◽  
R. Surber ◽  
G. Dannberg ◽  
H.R. Figulla ◽  
...  
Keyword(s):  
Heart Failure ◽  
Nyha Class ◽  
Left Ventricular ◽  
Ventricular Pacing ◽  
Class Iii ◽  
Heart Failure Patients ◽  
Pacing Mode ◽  
Left Ventricular Pacing

Abstract 14647: Stress Echocardiography Predicts Heart Failure Progression and Alters Management Strategies in Patients with Hypertrophic Cardiomyopathy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Ethan J Rowin ◽  
Barry J Maron ◽  
Iacopo Olivotto ◽  
Susan A Casey ◽  
Anna Arretini ◽  
...  
Keyword(s):  
Heart Failure ◽  
Nyha Class ◽  
Management Strategies ◽  
Left Ventricular ◽  
Advanced Heart Failure ◽  
Outflow Tract ◽  
Class I ◽  
Class Iii ◽  
Outflow Obstruction ◽  
Heart Failure Progression

Background: One-third of HCM patients without left ventricular outflow tract obstruction under resting conditions have the propensity to develop an outflow gradient with physiologic exercise. However, the natural history and management implications of exercise-induced (i.e., provocable) obstruction is unresolved. Methods: We prospectively studied 533 consecutive HCM patients without outflow obstruction at rest (<30mmHg) who underwent a symptom limiting stress (exercise) echocardiogram to assess development of outflow obstruction following physiologic provocation and followed for 6.5 ± 2.0 years. Of the 533 patients, obstruction ≥ 30 mmHg was present following exercise in 262 patients (49%; provocable obstruction), and was absent both at rest and with exercise in 271 (51%; nonobstructive). Results: Over the follow-up period, 43 out of 220 (20%) HCM patients with provocable obstruction and baseline NYHA class I/II symptoms developed progressive limiting heart failure symptoms to class III/IV, compared to 24 of 249 (10%) nonobstructive patients. Rate of heart failure progression was significantly greater in patients with provocable obstruction vs. nonobstructive patients (3.1%/year vs. 1.5%/year; RR=2.0, 95% CI of 1.3-3.2; p=0.003). However, the vast majority of patients with provocable obstruction who developed advanced heart failure symptoms achieved substantial improvement in symptoms to class I / II following relief of obstruction with invasive septal reduction therapy (n=30/32; 94%). In comparison, the majority of nonobstructive patients who developed advanced heart failure remained in class III/IV (16/24;67%), including 10 (42%) currently listed for heart transplant. Conclusions: Stress (exercise) echocardiogram identifies physiological provocable outflow tract obstruction in HCM, and is a predictor of future risk for progressive heart failure (3.1%/year), in patients who become candidates for invasive septal reduction therapy. Therefore, exercise echocardiography should be considered in all HCM patients without obstruction under resting conditions.

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

Abstract 4876: LAMP2 Cardiomyopathy is a Particularly Adverse Phenocopy of Sarcomeric Hypertrophic Cardiomyopathy

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Barry Maron ◽  
William C Roberts ◽  
Michael Arad ◽  
Carolyn Y Ho ◽  
Tammy S Haas ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Death ◽  
Heart Transplantation ◽  
Systolic Dysfunction ◽  
Ventricular Tachyarrhythmia ◽  
Young Patients ◽  
Disease Process ◽  
Left Ventricular ◽  
Asymptomatic Patients

Mutations in the X-linked lysosome-associated membrane protein gene (LAMP2; Danon disease) produce a morphologic phenocopy of sarcomeric hypertrophic cardiomyopathy (HCM) in young patients, characterized by extreme left ventricular (LV) hypertrophy and pre-excitation. However, the natural history of this newly recognized cardiomyopathy is incompletely resolved. Seven young asymptomatic patients with LAMP2 cardiomyopathy were identified at ages 8 to 15 years; 6 were male. LV hypertrophy was particularly marked (septal thickness 25– 65 mm; mean 42±17) in the presence of nondilated LV cavity. On each ECG, Wolff-Parkinson-White pre-excitation pattern was associated with markedly increased voltages (74±38mm for R- or S-wave). Over the 7±3 year follow-up from initial cardiac diagnosis, all 7 patients experienced particularly adverse disease consequences associated with progressive LV wall thinning and cavity dilatation and systolic dysfunction (ejection fraction, 29±7%) by the ages of 12 – 24 years (mean 20). Of the 7 patients, 5 either died of progressive heart failure, had heart transplantation or were considered for a donor heart; 2 others had sudden death events, including one fatal ventricular tachyarrhythmia refractory to defibrillator therapy and one appropriate defibrillator shock in an asymptomatic female survivor. Pathologic examination of hearts at autopsy showed histopathologic findings compatible with both HCM due to sarcomere protein mutations (i.e., extensive myocyte disarray, intramural small vessel disease, myocardial replacement scarring), and also evidence of a storage disease process (i.e., clusters of myocytes with vacuolated sarcoplasm within fibrotic areas). Heart weights, 1266 and 1425 grams, are the most substantial recorded for hypertrophic cardiomyopathies. LAMP2 cardiomyopathy is a uniformly profound, and particularly deleterious disease entity, causing refractory heart failure with systolic dysfunction as well as sudden death in young patients < 25 years of age. This novel phenocopy of sarcomeric HCM underscores the power of molecular diagnosis for predicting prognosis, and should also raise consideration for intervention with early heart transplantation.

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.

Sign in / Sign up

Export Citation Format

Share Document