CLINICAL STUDY: HYPERTROPHIC CARDIOMYOPATHY
Sudden death in hypertrophic cardiomyopathy: identification of high risk patients
Perry M. Elliott, MRCPa,
Jan Poloniecki, DPhila,
Shaughan Dickiea,
Sanjay Sharma, BSc, MRCPa,
Lorenzo Monserrat, MDa,
Amanda Varnava, MRCPa,
Niall G. Mahon, MD, MRCPIa and
William J. McKenna, MD, FRCP, FACC, FESCa
a Department of Cardiological Sciences, St. George’s Hospital Medical School, London, United Kingdom
Manuscript received February 4, 2000;
revised manuscript received July 10, 2000,
accepted August 18, 2000.
Reprint requests and correspondence: Dr. P. M. Elliott, Department of Cardiological Sciences, St. George’s Hospital Medical School, Cranmer Terrace, London SW17 0RE, United Kingdom
pelliott{at}sghms.ac.uk
 |
Abstract
|
|---|
OBJECTIVES
We sought to identify patients with hypertrophic cardiomyopathy (HCM) at high risk of sudden death (SD).
BACKGROUND
Relatively low mortality rates in HCM make conventional analysis of multiple clinical risk markers for SD problematic. This study used a referral center registry to investigate a smaller number of generally accepted noninvasive risk markers.
METHODS
We studied 368 patients (14 to 65 years old, 239 males) with HCM. There were five variables: nonsustained ventricular tachycardia (NSVT), syncope, exercise blood pressure response (BPR), family history of sudden death (FHSD) and left ventricular wall thickness (LVWT).
RESULTS
During follow-up (3.6 ± 2.5 years [range 2 days to 9.6 years]), 36 patients (9.8%) died, 22 of them suddenly. Two patients received heart transplants. The six-year SD-free survival rate was 91% (95% confidence interval [CI] 87% to 95%). In the Cox model, there was a significant pairwise interaction between FHSD and syncope (p = 0.01), and these were subsequently considered together. The multivariate SD risk ratios (with 95% CIs) were 1.8 for BPR (0.7 to 4.4) (p = 0.22); 5.3 for FHSD and syncope (1.9 to 14.9) (p = 0.002); 1.9 for NSVT (0.7 to 5.0) (p = 0.18) and 2.9 for LVWT (1.1 to 7.1) (p = 0.03). Patients with no risk factors (n = 203) had an estimated six-year SD-free survival rate of 95% (95% CI 91% to 99%). The corresponding six-year estimates (with 95% CIs) for one (n = 122), two (n = 36) and three (n = 7) risk factors were 93% (87% to 99%), 82% (67% to 96%) and 36% (0% to 75%), respectively. Patients with two or more risk factors had a lower six-year SD survival rate (95% CI) compared with patients with one or no risk factors (72% [56% to 88%] vs. 94% [91% to 98%]) (p = 0.0001).
CONCLUSIONS
This study demonstrates that patients with multiple risk factors have a substantially increased risk of SD sufficient to warrant consideration for prophylactic therapy.
|
Abbreviations and Acronyms
| | BPR | = blood pressure response | | CI | = confidence interval | | ECG | = electrocardiogram or electrocardiographic | | FHSD | = family history of sudden death | | HCM | = hypertrophic cardiomyopathy | | LVWT | = left ventricular wall thickness | | NSVT | = nonsustained ventricular tachycardia | | NYHA | = New York Heart Association | | SD | = sudden death |
|
The identification and treatment of patients with hypertrophic cardiomyopathy (HCM) who are at risk of dying suddenly are imperative in clinical management. Although some sarcomeric protein gene mutations are associated with a high risk of sudden death (SD) (1), the heterogeneity and variable expression of gene abnormalities in HCM have limited the use of clinical genetic testing for prognostic purposes. Many clinical features are associated with an increased risk of dying suddenly (2–8), but most are associated with only modest positive predictive accuracy. Furthermore, the relatively low annual mortality rates and small size of most patient groups make conventional analysis of the interaction between multiple risk factors problematic. To overcome this limitation, we hypothesized that a smaller number of generally accepted risk markers could be used to generate a practical clinical risk stratification algorithm.
|
Methods
|
|---|
Clinical characterization.
Between 1988 and 1998, 630 patients (37 ± 16 years old, 382 men) with HCM were assessed at St. George’s Hospital in London. The reasons for referral were symptom management (26%), family screening (19%), confirmation of diagnosis (10%) and risk assessment (17%). Fifteen percent were referred for miscellaneous reasons, including self-referral, second opinions and genetic counseling. The reason for referral was not recorded in 15% of patients. The diagnosis of HCM was based on the presence of unexplained left ventricular hypertrophy (>2 standard deviations from the normal ranges corrected for age and body size) or on the presence of unexplained electrocardiographic (ECG) and echocardiographic abnormalities in the relatives of patients with an unequivocal diagnosis of HCM, in accordance with recently proposed diagnostic criteria (9,10). Patients with Friedreich’s ataxia, Noonan’s syndrome and primary metabolic disorders known to cause HCM were not included.
During follow-up, six patients underwent myotomy-myectomy, three had percutaneous alcohol ablation of the interventricular septum and three had mitral valve replacement. Sixty-four patients had received pacemakers by the time of their last follow-up: 34 for refractory symptoms related to outflow tract obstruction, 28 for high degree atrioventricular block or symptomatic bifascicular or trifascicular block and 2 for chronotropic incompetence. Patients received the following cardioactive medications: beta-blockers (n = 137 [21.7%]), sotalol (n = 35 [5.6%]), calcium antagonists (n = 142 [22.5%]), diuretics (n = 66 [10.5%]), warfarin (n = 63 [10%]), angiotensin-converting enzyme inhibitors (n = 19 [3%]), disopyramide (n = 21 [3.3%]) and propafenone (n = 3 [0.5%]). One hundred and seventy-five patients (27.8%) were prescribed amiodarone for at least one day of their follow-up. The reasons for therapy were supraventricular tachycardia (including paroxysmal atrial fibrillation) (n = 34 [19.4%]), SD prophylaxis (n = 64 [36.6%]), both supraventricular tachycardia and SD prophylaxis (n = 36 [20.6%]) and rate control for atrial fibrillation (n = 5 [2.9%]). In the remaining 36 patients, amiodarone therapy had been started by referring physicians before the first assessment at St. George’s Hospital.
For the purposes of this study, patients were selected from the cohort of 630 patients using the following criteria: 1) age 14 to 65 years; 2) successful completion of all noninvasive tests (echocardiography, Holter monitoring and upright exercise testing); and 3) follow-up for more than one day. Patients with documented sustained ventricular arrhythmia or out-of-hospital cardiac arrest and those who had received amiodarone for an arbitrary period of >50% of their follow-up were also excluded. A detailed pedigree analysis and clinical history were obtained from all patients. A history of syncope was defined as one or more episodes of unexplained loss of consciousness within the 12 months preceding their first visit to St. George’s Hospital. Chest pain was classified as exertional or atypical if it lasted >30 min at rest in the absence of myocardial infarction. Dyspnea was coded according to the New York Heart Association (NYHA) classification (11). A family history of SD was defined as sudden cardiac death in two or more first-degree relatives <40 years old.
Echocardiography.
Two-dimensional and M-mode echocardiography were performed using conventional methods (12,13). In summary, end-diastolic left ventricular wall thickness (LVWT) was recorded at the mitral valve and papillary muscle level in the anterior and posterior septum, as well as in the lateral and posterior left ventricular wall using short-axis two-dimensional images. Anterior and posterior septal thickness at the apex was assessed from the apical four-chamber and the parasternal short-axis views. Left ventricular outflow tract velocities were determined using continuous wave Doppler echocardiography, and left ventricular outflow tract gradients were calculated using the modified Bernoulli equation.
Electrocardiography.
All patients underwent 48-h ambulatory ECG monitoring while performing unrestricted daily activities, using the Marquette (Marquette Electronics Inc., Diagnostic Division, Milwaukee, Wisconsin) Holter recording system on two channels. Computer-assisted analysis was performed using the Marquette series 8000 laser Holter and laser Holter XP system. Nonsustained ventricular tachycardia (NSVT) was defined as a run of three or more consecutive ventricular beats at a rate of  120 beats/min, lasting <30 s.
Exercise testing.
Patients exercised to exhaustion on a treadmill, using the Bruce or modified Bruce protocols (1988 to 1994), or on an upright bicycle ergometer (Sensormedics Ergometrics 800S, Bitz, Germany), using an incremental ramp protocol from 1994 onward. Blood pressure was measured using a mercury sphygmomanometer and auscultation of the Korotkoff sounds over the brachial artery at rest, every minute during exercise and for the first 5 min of recovery (5).
Survival analysis.
Survival data were collected between July 1997 and January 1998, using a questionnaire sent to all patients’ general practitioners. Additional information was obtained at clinic visits and by direct communication with patients and their physicians.
|
Definitions
|
|---|
Initial investigations.
The initial investigations are defined as the first echocardiogram, 48-h ambulatory ECG and upright exercise test performed at our institution. Data from subsequent investigations at St. George’s Hospital were not examined in this study.
Start of follow-up.
Follow-up started with the date of completion of the initial investigations, as defined previously.
End point classification.
Sudden cardiac death
Witnessed sudden cardiac death with or without documented ventricular fibrillation or death within 1 h of new symptoms. Nocturnal deaths with no antecedent history of worsening symptoms were included in this category.
Progressive heart failure
Death preceded by signs and/or symptoms of heart failure, including cardiogenic shock.
Heart transplantation
Orthotopic heart transplantation performed for end-stage disease with systolic impairment and refractory congestive symptoms.
Other cardiovascular death
Stroke, pulmonary or systemic embolism and myocardial infarction.
Noncardiovascular death
Death secondary to noncardiovascular events and of unknown cause.
Risk factors.
Five clinical risk factors were assessed: a family history of sudden cardiac death (FHSD), unexplained syncope, NSVT on ambulatory electrocardiography, abnormal blood pressure response (BPR) during upright exercise testing and maximal LVWT. Blood pressure response and LVWT were examined in a dichotomous fashion to determine the most predictive cut-off values for SD.
An abnormal BPR was defined as a failure of blood pressure to rise, or a fall in blood pressure during exercise (5). According to previously published data, BPRs were only classified as a risk factor in patients  40 years of age (5). The relation between SD and the change in blood pressure during exercise was determined using the Cox proportional hazards model. The statistical significance of blood pressure thresholds for a flat or hypotensive BPR in increments of 5 mm Hg (range 0 to 30) was determined, and the most significant pairing of threshold values for a rise and fall was used in subsequent analyses. The relation between maximal LVWT and time to SD was also studied in the Cox proportional hazards model. The statistical significance of wall thickness in 1-mm increments was determined, and the most significant cut-off value was used in the subsequent survival analysis.
Statistical analysis.
All data are presented as the mean value ± standard deviation. A p value <0.05 was considered statistically significant. All statistical analyses were performed using SAS software, release 6.12 (SAS Institute Inc.; Cary, North Carolina). Comparison between groups was performed using the chi-square test for proportional data. The probability of survival was estimated as a function of the length of follow-up, age and the five risk factors, using the Cox proportional hazards model. All risk factors were tested for pairwise interactions.
|
Results
|
|---|
From a total of 630 patients assessed between 1988 and 1998, 368 (239 men) fulfilled the selection criteria. Sixty-eight patients were excluded because they were older than 65 years or younger than 14 years. The remaining patients were excluded because of a history of ventricular fibrillation before their first assessment (n = 11), no verifiable Holter monitor recording (n = 30), inability or unwillingness to perform exercise testing (n = 25), incomplete blood pressure recordings at baseline, peak exercise and/or recovery (n = 38) and less than one day of follow-up (n = 14). Eleven additional patients were disqualified because they had two or more of these exclusions. When patients were excluded for these reasons, an additional 76 patients who had taken amiodarone for at least 50% of their follow-up were excluded.
The mean age at diagnosis of HCM in the 368 study patients was 33 ± 14 years (range 1 to 65). Their mean age at initial evaluation at St. George’s Hospital was 37 ± 13 years. All patients completed their initial investigations without complications. Symptoms at initial assessment and the results of noninvasive testing in the study cohort of 368 patients are shown in Table 1. Five patients were in paced rhythm at their initial assessment. Forty-two patients (11%) were <20 years old; 84 (23%) were 20 to 29 years old; 85 (23%) were 30 to 39 years old; 90 (25%) were 40 to 49 years old; 45 (12%) were 50 to 59 years old; and 22 (6%) were 60 to 65 years old.
Blood pressure response.
Using the Cox proportional hazards model for SD, the most predictive cut-off values for a flat or hypotensive BPR during upright exercise in patients 40 years old were <25 mm Hg and >15 mm Hg, respectively (p = 0.04). The model confirmed that these blood pressure criteria were not significant at the age cut-off of >40 years (p > 0.05), and abnormal BPR during exercise was subsequently considered a risk factor only in patients 40 years old. A total of 138 (38%) of the 368 patients had an abnormal BPR by these criteria. Flat or hypotensive responses were more prevalent in patients 40 years old than in older patients (91 [43%] of 214 vs. 47 [31%] of 154, p = 0.02). Hypotensive but not flat responses (i.e., a fall of >15 mm Hg from baseline) were more prevalent in patients 40 years old than in older patients (38 [18%] of 214 vs. 13 [8%] of 154, p = 0.01).
Maximal wall thickness.
Using the Cox proportional hazards model for SD, the most predictive cut-off value for LVWT was 30 mm (p = 0.001). Forty-four patients (12%) had a wall thickness 30 mm. Of the 368 patients studied, 229 (62%) had asymmetric septal hypertrophy (ratio >1.5:1 [12]), 120 (33%) had concentric hypertrophy and 14 (4%) had hypertrophy confined to the ventricular apex. Five patients (2%) had other eccentric patterns of hypertrophy. There was no relation between the pattern of hypertrophy (asymmetric, concentric, apical or eccentric) and survival from all-cause death (p = 0.32) or SD (p = 1.0).
Survival.
Mean follow-up was 3.6 ± 2.5 years (range 2 days to 9.6 years). Thirty-six of the 368 patients (9.8%) died during follow-up. Twenty-two deaths (61%) were sudden, 5 (14%) were from progressive heart failure, 4 (11%) were from other cardiovascular causes and 5 (14%) were from noncardiac causes. Two patients received orthotopic heart transplants. The mean age at death or transplantation was 39 ± 16 years (range 15 to 65). Of the 22 patients who died suddenly, 15 were <40 years old. Sudden death occurred at rest in three patients (14%), after mild exertion in eight patients (36%) and after moderate to severe exertion in four patients (18%) and was nocturnal in three patients (14%). The circumstances preceding SD were unknown in four patients. The Kaplan-Meier estimate for six-year survival from all-cause death and transplantation for the whole group was 84% (95% confidence interval [CI] 78% to 89%). The Kaplan-Meier estimate for six-year survival from cardiovascular death (including transplantation) for the whole group was 87% (95% CI 82% to 92%). The Kaplan-Meier estimate for six-year survival from SD for the whole group was 91% (95% CI 87% to 95%).
Univariate survival analysis.
The univariate SD risk ratios for individual risk factors were 2.4 for an abnormal exercise blood pressure (95% CI 1 to 5.5, p = 0.04); 2.0 for syncope 2.0 (95% CI 0.8 to 4.9, p = 0.13); 1.8 for NSVT (95% CI 0.7 to 4.7, p = 0.21); 1.9 for FHSD (95% CI 0.8 to 4.5, p = 0.15); and 4.1 for maximal LVWT (95% CI 1.7 to 9.5, p = 0.001).
All risk factors were tested for lack of additivity in the Cox model for SD. There was no statistical significance for the interaction term when any pair of factors was considered, except for FHSD and syncope (p = 0.01). In the absence of the other factor, each of these two factors had a relative risk <1 (p = NS). Thus, in the subsequent multivariate analyses, these two factors were considered as a single factor (i.e., FHSD and syncope). This successfully removed all pairwise interactions and reduced the number of risk factors in the survival model to four. The univariate risk ratio for this combined risk factor was 8.2 (95% CI 3.0 to 22.4, p < 0.0001).
Multivariate survival analysis.
The multivariate SD risk ratios for these four risk factors were 1.8 for an abnormal exercise blood pressure (95% CI 0.7 to 4.4, p = 0.22); 5.3 for FHSD and syncope (95% CI 1.9 to 14.9, p = 0.002); 1.9 for NSVT (95% CI 0.7 to 5.0, p = 0.18); and 2.9 for maximal LVWT (95% CI 1.1 to 7.1, p = 0.03) (Fig. 1). The SD risk ratios in patients who were in NYHA functional class I were 3.3 for an abnormal exercise blood pressure (p = 0.06); 5.2 for FHSD and syncope (p = 0.07); 3.8 for NSVT (p = 0.04); and 3.9 for maximal LVWT (p = 0.03).
View larger version (29K):
[in this window]
[in a new window]
|
Figure 1 Multivariate risk ratios for each of the four risk factors and age. The bars represent the upper and lower 95% confidence intervals. Ratios >1 indicate a positive association with SD. Abnormal exercise blood pressure pertains only to patients <40 years old. MLVWT = maximal left ventricular wall thickness; FH = family history of SD; Abn BP = abnormal exercise blood pressure response in patients <40 years old; RF = risk factors.
|
|
Relation between number of risk factors and SD.
When FHSD and syncope were considered as a single risk factor, 203 patients (55%) had no risk factors. The corresponding numbers of patients with one, two and three risk factors were 122 (33%), 36 (10%) and 7 (2%), respectively. No patient had four risk factors. The numbers of SDs in patients with zero, one, two and three risk factors were 6 (3%), 6 (5%), 6 (17%) and 4 (57%), respectively. The total numbers of deaths in patients with zero, one, two and three risk factors were 13 (6%), 12 (10%), 8 (22%) and 5 (71%), respectively (Fig. 2). Patients with no risk factors had an estimated six-year survival rate from SD of 95% (95% CI 91% to 99%). The corresponding SD survival estimates (with 95% CIs) for one, two and three risk factors were 93% (87% to 99%), 82% (67% to 96%) and 36% (0% to 75%), respectively. Of the 122 patients with only a single risk factor, only four had FHSD and syncope. Patients with two or more risk factors had a significantly lower estimate of six-year survival from SD compared with patients with one or no risk factors (72% [95% CI 56% to 88%] vs. 94% [95% CI 91% to 98%], p = 0.0001), with a risk ratio of 5.6 (95% CI 2.43 to 13.1, p = 0.002) (Fig. 1).
View larger version (15K):
[in this window]
[in a new window]
|
Figure 2 Bar graph showing the percentage of each risk factor group (zero, one, two and three risk factors) in which patients died during follow-up (black bars = sudden death; hatched bars = congesitve cardiac failure or transplant; white = all deaths). The majority of deaths were sudden, and the greatest proportion occurred in patients with multiple risk factors. CCF = congestive cardiac failure; Tx = cardiac transplant.
|
|
Thirteen patients (8%) with none of the four risk factors, as defined earlier, died during follow-up, six of them suddenly. Of the six patients who died suddenly, two (ages 52 and 70 years, maximal wall thickness of 17 and 20 mm, respectively) had significant coronary artery disease with no resting left ventricular outflow obstruction. One patient (age 49 years) had a maximal wall thickness of 23 mm, no obstruction, refractory chest pain with angiographically normal coronary arteries and ST segment depression during ambulatory ECG monitoring. Two patients (ages 35 and 22 years) had FHSD, but no history of syncope, NSVT or an abnormal exercise BPR. Both had asymmetric septal hypertrophy with a maximal LVWT of 23 and 27 mm, respectively, with no rest outflow tract obstruction. One patient (age 48 years, maximal wall thickness of 24 mm) had an outflow tract gradient of 121 mm Hg and left atrial dilation.
|
Discussion
|
|---|
Many clinical features associated with an increased risk of SD have been reported in the published data. However, the relatively low annual mortality rates in most patient groups (14–24) make the study of their interaction problematic, as conventional multiple logistic regression analysis of large numbers of risk factors jeopardize the reproducibility of survival estimates. This study shows that a simpler model based on a smaller number of generally accepted risk factors can be used to identify a cohort of patients who are at a substantially increased risk of SD.
Patient selection criteria.
The patient selection criteria in this study were designed to allow examination of a specific question—namely, can noninvasive markers be used to identify high risk patients who might benefit from prophylactic antiarrhythmic treatment? Patients with a history of sustained ventricular arrhythmia were excluded, as the risk of SD in this cohort was sufficiently high to warrant prophylactic therapy, irrespective of their other risk factors (25,26). Similarly, many patients in our group were treated with amiodarone because they were considered to be at risk of SD or for suppression of supraventricular arrhythmias, or both, and the exclusion criterion of amiodarone therapy for >50% of total follow-up was a pragmatic attempt to minimize any confounding effect that the drug may have on survival (27,28). Patients >65 years were excluded to reduce the confounding effects of comorbidity on survival and the inability to perform all aspects of risk stratification. The five risk factors analyzed in this study were selected because they reflect the published data and are easily determined in routine clinical practice. However, they are not exclusive, and other clinical features, such as left ventricular outflow tract obstruction, atrial fibrillation, diastolic dysfunction and myocardial ischemia, could potentially be incorporated into a survival model. In several patients, there was circumstantial evidence that myocardial ischemia may have played a role in triggering a fatal rhythm disturbance, but routine screening for myocardial ischemia in patients with HCM remains problematic.
Significance of individual risk factors.
In the univariate analysis, only BPR and maximal LVWT achieved a statistically significant association with SD. In the light of evidence describing "high risk" cardiac sarcomeric protein mutations (1), the low risk associated with FHSD alone seems counterintuitive. However, the small size of most family pedigrees, as well as the uncertain cause of SD in some individuals, can make it difficult to define a "malignant" family history and masks the undoubted importance of a family history in individuals with troponin T and high risk beta-myosin heavy-chain gene mutations. Isolated syncope is also problematic, as its multifactorial nature reduces its predictive accuracy in individual patients. An important finding in this study was the statistical interaction between FHSD and syncope. In the absence of the other, neither of these two factors was associated with an increased risk. In combination, however, they were a powerful predictor of risk. This observation may reflect a particular clinical phenotype, perhaps associated with specific high risk gene mutations.
Although several cross-sectional studies have shown that NSVT is associated with an increased incidence of SD, in this study NSVT as a single risk factor was not significantly associated with SD. A recent study in a selected "low risk" cohort has suggested that NSVT is prognostically significant only when repetitive or associated with symptoms (29). However, even in this latter report, NSVT in asymptomatic patients was associated with a relative SD risk of 2.4, with a very wide 95% CI (0.5 to 11.9). Although this ratio did not attain conventional levels of statistical significance, it was higher than that observed in the present study, and the wide confidence interval means that an association between SD and NSVT cannot be excluded.
Although abnormal exercise BPRs occurred at all ages, this study confirms the previous observation that abnormal BPRs are of prognostic significance only in patients younger than 40 years of age. Hypotensive responses were more common in the younger cohort, supporting the suggestion that the predominant mechanism of abnormal BPRs in young patients is inappropriate vasodilation in nonexercising muscles (30). In older patients, factors related to cardiac output during exercise and impaired exercise capacity due to comorbidity may be more important.
This study confirms the findings from previous cross-sectional studies that severe left ventricular hypertrophy is associated with an increased incidence of SD (8). This relation should not, however, obscure the fact that only 12% of patients had a wall thickness >30 mm, and that the majority of SDs occurred in patients with only mild to moderate hypertrophy.
Clinical implications.
Although the absence of risk factors identified individuals at low risk of SD, the presence of two or more risk factors (present in 12% of patients) was associated with a 4% to 5% estimated annual SD risk. This observation suggests that the estimated risk of SD associated with multiple risk factors may be high enough to justify prophylactic antiarrhythmic therapy. There is evidence to suggest that both amiodarone and implantable cardioverter-defibrillators can prevent SD in high risk patients with HCM, but their relative merits in different patient subgroups remains to be determined (26,27,31–34). Therapeutic recommendations for patients with single risk factors remain speculative, and further work on individual risk factors is necessary to determine if and when prophylactic therapy is indicated.
Study limitations.
The overall mortality rate in this study was higher than that observed in some recently reported community studies. This may be explained by the fact that the study was performed at a tertiary referral center for the disease, but factors such as end point classification and statistical methods are probably of equal importance. Furthermore, the lack of comparable systematic risk stratification data in community-based populations makes it difficult to determine the importance, if any, of referral center bias with respect to clinical risk assessment.
The mean age of the overall study group was lower than that in many community studies. As with survival, this may be explained by referral patterns, but the routine practice of screening first-degree family members at our institution undoubtedly results in the identification of younger individuals with the disease.
The rates of surgical intervention for left ventricular outflow tract obstruction in our group are lower than those in some North American and European centers, reflecting historic practice and our participation in multicenter pacing studies. Similarly, the relatively low use of beta-blockers and calcium antagonists reflects the fact that 65% of our patients were in NYHA functional class I at their initial evaluation.
Conclusions.
Although overall SD rates are relatively low in patients with HCM, this study shows that relatively simple, noninvasive tests can be used to identify a cohort of patients who have a substantial risk of SD and who warrant consideration for prophylactic antiarrhythmic therapy.
|
Footnotes
|
|---|
Dr. Sharma was supported by a Junior Fellowship Grant from the British Heart Foundation. Dr. Varnava was supported by a grant from the Medical Research Council.
|
References
|
|---|
1. Watkins H, McKenna WJ, Thierfelder L, et al. Mutations in the genes for cardiac troponin T and alpha-tropomyosin in hypertrophic cardiomyopathy. N Engl J Med. 1995;332:1058–1064[Abstract/Free Full Text]
2. McKenna WJ, England D, Doi Y, Deanfield JE, Oakley CM, Goodwin JF. Arrhythmia in hypertrophic cardiomyopathy. 1. Influence on prognosis. Br Heart J. 1981;46:168–172[Abstract/Free Full Text]
3. Maron BJ, Savage DD, Wolfson JK, Epstein SE. Prognostic significance of 24 hour ambulatory electrocardiographic monitoring in patients with hypertrophic cardiomyopathy: a prospective study. Am J Cardiol. 1981;48:252–257[CrossRef][Medline]
4. McKenna WJ, Deanfield J, Faruqui A, England D, Oakley C, Goodwin J. Prognosis in hypertrophic cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features. Am J Cardiol. 1981;47:532–538[CrossRef][Medline]
5. Sadoul N, Prasad K, Elliott PM, Banerjee S, Frenneaux MP, McKenna WJ. Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy. Circulation. 1997;96:2987–2991[Abstract/Free Full Text]
6. Maki S, Ikeda H, Muro A, et al. Predictors of sudden cardiac death in hypertrophic cardiomyopathy. Am J Cardiol. 1998;82:774–778[CrossRef][Medline]
7. Olivotto I, Maron BJ, Montereggi A, Mazzuoli F, Dolara A, Cecchi F. Prognostic value of systemic blood pressure response during exercise in a community based population with hypertrophic cardiomyopathy. J Am Coll Cardiol. 1999;33:2044–2051[Abstract/Free Full Text]
8. Spirito P, Maron BJ. Relation between extent of left ventricular hypertrophy and occurrence of sudden cardiac death in hypertrophic cardiomyopathy. J Am Coll Cardiol. 1990;15:1521–1526[Abstract]
9. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of Cardiomyopathies. Circulation. 1996;93:841–842[Free Full Text]
10. McKenna WJ, Spirito P, Desnos M, Dubourg O, Komajda M. Experience from clinical genetics in hypertrophic cardiomyopathy: proposal for new diagnostic criteria in adult members of affected families. Heart. 1997;77:130–132[Abstract/Free Full Text]
11. Criteria Committee of the New York Heart Association. Nomenclature and Criteria for Diagnosis. 9th ed. Boston: Little, Brown & Company; 1994.
12. Shapiro LM, McKenna WJ. Distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy: a two-dimensional echocardiographic study. J Am Coll Cardiol. 1983;2:437–444[Abstract]
13. Maron BJ, Gottdiener JS, Epstein SE. Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy: a wide-angle, two-dimensional echocardiographic study of 125 patients. Am J Cardiol. 1981;48:418–428[CrossRef][Medline]
14. Shah PM, Adelman AG, Wigle ED, et al. The natural and unnatural history of hypertrophic obstructive cardiomyopathy. Circ Res. 1974;35(Suppl II):II179–II195
15. Loogen F, Kuhn H, Gietzen F, Losse B, Schulte HD, Bircks W. Clinical course and prognosis of patients with typical and atypical hypertrophic obstructive and with hypertrophic non-obstructive cardiomyopathy. Eur Heart J. 1983;4(Suppl F):145–153
16. Koga Y, Itaya K, Toshima H. Prognosis in hypertrophic cardiomyopathy. Am Heart J. 1984;108:351–359[CrossRef][Medline]
17. Romeo F, Pelliccia F, Cristofani R, Martuscelli E, Reale A. Hypertrophic cardiomyopathy: is a left ventricular outflow tract gradient a major prognostic determinant? Eur Heart J. 1990;11:233–240[Abstract/Free Full Text]
18. McKenna WJ, Deanfield JE. Hypertrophic cardiomyopathy: an important cause of sudden death. Arch Dis Child. 1984;59:971–975[Abstract/Free Full Text]
19. Spirito P, Chiarella F, Carratino L, Zoni-Berisso M, Bellotti P, Vecchio C. Clinical course and prognosis of hypertrophic cardiomyopathy in an outpatients population. N Engl J Med. 1989;320:749–755[Abstract]
20. Cecchi F, Olivotto I, Montereggi A, Santoro G, Dolara A, Maron BJ. Hypertrophic cardiomyopathy in Tuscany: clinical course and outcome in an unselected regional population. J Am Coll Cardiol. 1995;26:1529–1536[Abstract]
21. Cannan CR, Reeder GS, Bailey KR, Melton LJ III, Gersh BJ. Natural history of hypertrophic cardiomyopathy: a population based study, 1976 through 1990. Circulation. 1995;92:2488–2495[Abstract/Free Full Text]
22. Shapiro LM, Zezulka A. Hypertrophic cardiomyopathy: a common disease with a good prognosis—five-year experience of a district general hospital. Br Heart J. 1983;50:530–533[Abstract/Free Full Text]
23. Kofflard MJ, Waldstein DJ, Vos J, ten Cate F. Prognosis in hypertrophic cardiomyopathy observed in a large clinic population. Am J Cardiol. 1993;72:939–943[CrossRef][Medline]
24. Maron BJ, Casey SA, Poliac LC, Gohman TE, Almquist AK, Aeppli DM. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA. 1999;281:650–655[Abstract/Free Full Text]
25. Cecchi F, Maron BJ, Epstein SE. Long-term outcome of patients with hypertrophic cardiomyopathy successfully resuscitated after cardiac arrest. J Am Coll Cardiol. 1989;13:1283–1288[Abstract]
26. Elliott PM, Sharma S, Varnava A, Poloniecki J, Rowland E, McKenna WJ. Survival following cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 1999;33:1596–1601[Abstract/Free Full Text]
27. McKenna WJ, Oakley CM, Krikler DM, Goodwin JF. Improved survival with amiodarone in patients with hypertrophic cardiomyopathy and ventricular tachycardia. Br Heart J. 1985;53:412–416[Abstract/Free Full Text]
28. Cecchi F, Olivotto I, Montereggi A, Squillatini G, Dolara A, Maron BJ. Prognostic value of non-sustained ventricular tachycardia and the potential role of amiodarone treatment in hypertrophic cardiomyopathy: assessment in an unselected non-referral based patient population. Heart. 1998;79:331–336[Abstract/Free Full Text]
29. Spirito P, Rapezzi C, Autore C, et al. Prognosis of asymptomatic patients with hypertrophic cardiomyopathy and nonsustained ventricular tachycardia. Circulation. 1994;90:2743–2747[Abstract/Free Full Text]
30. Counihan PJ, Frenneaux MP, Webb DJ, McKenna WJ. Abnormal vascular responses to supine exercise in hypertrophic cardiomyopathy. Circulation. 1991;84:686–696[Abstract/Free Full Text]
31. Silka MJ, Kron J, Dunnigan A, Dick M. Sudden cardiac death and the use of implantable cardioverter-defibrillators in pediatric patients. Circulation. 1993;87:800–807[Abstract/Free Full Text]
32. Primo J, Geelen P, Brugada J, et al. Hypertrophic cardiomyopathy: role of the implantable cardioveter defibrillator. J Am Coll Cardiol. 1998;31:1081–1085[Abstract/Free Full Text]
33. Kron J, Oliver RP, Norsted S, Silka MJ. The automatic implantable cardioverter-defibrillator in young patients. J Am Coll Cardiol. 1990;16:896–902[Abstract]
34. Maron BJ, Shen W-K, Link MS, et al. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden cardiac death in patients with hypertrophic cardiomyopathy. N Engl J Med. 2000;342:365–373[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
I. Ostman-Smith, A. Wisten, E. Nylander, E.-L. Bratt, A. de-Wahl Granelli, A. Oulhaj, and E. Ljungstrom
Electrocardiographic amplitudes: a new risk factor for sudden death in hypertrophic cardiomyopathy
Eur. Heart J.,
November 5, 2009;
(2009)
ehp443v1.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Gupta, T. Baman, and S. M. Day
Cardiovascular Health, Part 1: Preparticipation Cardiovascular Screening
Sports Health: A Multidisciplinary Approach,
November 1, 2009;
1(6):
500 - 507.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Michels, O. I.I. Soliman, J. Phefferkorn, Y. M. Hoedemaekers, M. J. Kofflard, D. Dooijes, D. Majoor-Krakauer, and F. J. Ten Cate
Disease penetrance and risk stratification for sudden cardiac death in asymptomatic hypertrophic cardiomyopathy mutation carriers
Eur. Heart J.,
November 1, 2009;
30(21):
2593 - 2598.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. R. Gimeno, M. Tome-Esteban, C. Lofiego, J. Hurtado, A. Pantazis, B. Mist, P. Lambiase, W. J. McKenna, and P. M. Elliott
Exercise-induced ventricular arrhythmias and risk of sudden cardiac death in patients with hypertrophic cardiomyopathy
Eur. Heart J.,
November 1, 2009;
30(21):
2599 - 2605.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. R. Ommen and B. J. Gersh
Sudden cardiac death risk in hypertrophic cardiomyopathy
Eur. Heart J.,
November 1, 2009;
30(21):
2558 - 2559.
[Full Text]
[PDF]
|
|
|
|
|
|
|
O. Bruder, S. Schneider, D. Nothnagel, T. Dill, V. Hombach, J. Schulz-Menger, E. Nagel, M. Lombardi, A. C. van Rossum, A. Wagner, et al.
EuroCMR (European Cardiovascular Magnetic Resonance) Registry: Results of the German Pilot Phase
J. Am. Coll. Cardiol.,
October 6, 2009;
54(15):
1457 - 1466.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. S. Maron, I. Olivotto, B. J. Maron, S. K. Prasad, F. Cecchi, J. E. Udelson, and P. G. Camici
The case for myocardial ischemia in hypertrophic cardiomyopathy.
J. Am. Coll. Cardiol.,
August 25, 2009;
54(9):
866 - 875.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Unno, S. Isobe, H. Izawa, X. W. Cheng, M. Kobayashi, A. Hirashiki, T. Yamada, K. Harada, S. Ohshima, A. Noda, et al.
Relation of functional and morphological changes in mitochondria to myocardial contractile and relaxation reserves in asymptomatic to mildly symptomatic patients with hypertrophic cardiomyopathy
Eur. Heart J.,
August 1, 2009;
30(15):
1853 - 1862.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. M. Bos, J. A. Towbin, and M. J. Ackerman
Diagnostic, prognostic, and therapeutic implications of genetic testing for hypertrophic cardiomyopathy.
J. Am. Coll. Cardiol.,
July 14, 2009;
54(3):
201 - 211.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Basso, G. Thiene, S. Mackey-Bojack, A. C. Frigo, D. Corrado, and B. J. Maron
Myocardial bridging, a frequent component of the hypertrophic cardiomyopathy phenotype, lacks systematic association with sudden cardiac death
Eur. Heart J.,
July 1, 2009;
30(13):
1627 - 1634.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Haghjoo, S. Mohammadzadeh, M. Taherpour, B. Faghfurian, A. F. Fazelifar, A. Alizadeh, M. A. Rad, and M. A. Sadr-Ameli
ST-segment depression as a risk factor in hypertrophic cardiomyopathy
Europace,
May 1, 2009;
11(5):
643 - 649.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G Lin, R A Nishimura, B J Gersh, D Phil, S R Ommen, M J Ackerman, and P A Brady
Device complications and inappropriate implantable cardioverter defibrillator shocks in patients with hypertrophic cardiomyopathy
Heart,
May 1, 2009;
95(9):
709 - 714.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Grace
Implantable defibrillators in hypertrophic cardiomyopathy
Heart,
May 1, 2009;
95(9):
695 - 696.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Baggish and P. D Thompson
Thick hearts, high stakes, great uncertainties: screening athletes for hypertrophic cardiomyopathy
Heart,
March 1, 2009;
95(5):
345 - 347.
[Full Text]
[PDF]
|
|
|
|
|
|
|
G S Soor, A Luk, E Ahn, J R Abraham, A Woo, A Ralph-Edwards, and J Butany
Hypertrophic cardiomyopathy: current understanding and treatment objectives
J. Clin. Pathol.,
March 1, 2009;
62(3):
226 - 235.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Attili, G. C. Mueller, and S. M. Day
AJR Teaching File: Asymptomatic Man with Giant Negative T Waves on ECG
Am. J. Roentgenol.,
March 1, 2009;
192(3_Supplement):
S57 - S61.
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Brignole, J.-J. Blanc, R. Sutton, and A. Moya
CHAPTER 26 Syncope
ESC Textbook of Cardiovascular Medicine,
January 1, 2009;
2(1):
med-9780199566990-chapter - med-9780199566990-chapter.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. C. Afonso, J. Bernal, J. J. Bax, and T. P. Abraham
Echocardiography in hypertrophic cardiomyopathy: the role of conventional and emerging technologies.
J. Am. Coll. Cardiol. Img.,
November 1, 2008;
1(6):
787 - 800.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. A. Noseworthy and C. Newton-Cheh
Genetic Determinants of Sudden Cardiac Death
Circulation,
October 28, 2008;
118(18):
1854 - 1863.
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. S. Maron, J. J. Finley, J. M. Bos, T. H. Hauser, W. J. Manning, T. S. Haas, J. R. Lesser, J. E. Udelson, M. J. Ackerman, and B. J. Maron
Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy
Circulation,
October 7, 2008;
118(15):
1541 - 1549.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P Elliott and P Spirito
Prevention of hypertrophic cardiomyopathy-related deaths: theory and practice
Heart,
October 1, 2008;
94(10):
1269 - 1275.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K Prasad, L Williams, R Campbell, P M Elliott, W J McKenna, and M Frenneaux
Episodic syncope in hypertrophic cardiomyopathy: evidence for inappropriate vasodilation
Heart,
October 1, 2008;
94(10):
1312 - 1317.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. J. Goldberger, M. E. Cain, S. H. Hohnloser, A. H. Kadish, B. P. Knight, M. S. Lauer, B. J. Maron, R. L. Page, R. S. Passman, D. Siscovick, et al.
American Heart Association/American College of Cardiology Foundation/Heart Rhythm Society Scientific Statement on Noninvasive Risk Stratification Techniques for Identifying Patients at Risk for Sudden Cardiac Death: A Scientific Statement From the American Heart Association Council on Clinical Cardiology Committee on Electrocardiography and Arrhythmias and Council on Epidemiology and Prevention
J. Am. Coll. Cardiol.,
September 30, 2008;
52(14):
1179 - 1199.
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. J. Goldberger, M. E. Cain, S. H. Hohnloser, A. H. Kadish, B. P. Knight, M. S. Lauer, B. J. Maron, R. L. Page, R. S. Passman, D. Siscovick, et al.
American Heart Association/American College of Cardiology Foundation/Heart Rhythm Society Scientific Statement on Noninvasive Risk Stratification Techniques for Identifying Patients at Risk for Sudden Cardiac Death: A Scientific Statement From the American Heart Association Council on Clinical Cardiology Committee on Electrocardiography and Arrhythmias and Council on Epidemiology and Prevention
Circulation,
September 30, 2008;
118(14):
1497 - 1518.
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. S. Maron, E. Appelbaum, C. J. Harrigan, J. Buros, C. M. Gibson, C. Hanna, J. R. Lesser, J. E. Udelson, W. J. Manning, and B. J. Maron
Clinical Profile and Significance of Delayed Enhancement in Hypertrophic Cardiomyopathy
Circ Heart Fail,
September 1, 2008;
1(3):
184 - 191.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. C. Saumarez, M. Pytkowski, M. Sterlinski, J. P. Bourke, J. R. Clague, S. M. Cobbe, D. T. Connelly, M. J. Griffith, P. P. McKeown, K. McLeod, et al.
Paced ventricular electrogram fractionation predicts sudden cardiac death in hypertrophic cardiomyopathy
Eur. Heart J.,
July 1, 2008;
29(13):
1653 - 1661.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Sen-Chowdhry and W. J. McKenna
Non-invasive risk stratification in hypertrophic cardiomyopathy: don't throw out the baby with the bathwater
Eur. Heart J.,
July 1, 2008;
29(13):
1600 - 1602.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Basavarajaiah, A. Boraita, G. Whyte, M. Wilson, L. Carby, A. Shah, and S. Sharma
Ethnic differences in left ventricular remodeling in highly-trained athletes relevance to differentiating physiologic left ventricular hypertrophy from hypertrophic cardiomyopathy.
J. Am. Coll. Cardiol.,
June 10, 2008;
51(23):
2256 - 2262.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. S. Adabag, B. J. Maron, E. Appelbaum, C. J. Harrigan, J. L. Buros, C. M. Gibson, J. R. Lesser, C. A. Hanna, J. E. Udelson, W. J. Manning, et al.
Occurrence and Frequency of Arrhythmias in Hypertrophic Cardiomyopathy in Relation to Delayed Enhancement on Cardiovascular Magnetic Resonance
J. Am. Coll. Cardiol.,
April 8, 2008;
51(14):
1369 - 1374.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Basavarajaiah, M. Wilson, G. Whyte, A. Shah, W. McKenna, and S. Sharma
Prevalence of hypertrophic cardiomyopathy in highly trained athletes: relevance to pre-participation screening.
J. Am. Coll. Cardiol.,
March 11, 2008;
51(10):
1033 - 1039.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. F. Padera Jr. and F. J. Schoen
Pathology of Cardiac Surgery
Card. Surg. Adult,
January 1, 2008;
3(2008):
111 - 178.
[Full Text]
|
|
|
|
|
|
|
L. Williams and M. Frenneaux
Syncope in hypertrophic cardiomyopathy: mechanisms and consequences for treatment
Europace,
September 1, 2007;
9(9):
817 - 822.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. A. Stephenson and C. I. Berul
Electrophysiological Interventions for Inherited Arrhythmia Syndromes
Circulation,
August 28, 2007;
116(9):
1062 - 1080.
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Passman and A. Kadish
Sudden Death Prevention With Implantable Devices
Circulation,
July 31, 2007;
116(5):
561 - 571.
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. A. Nishimura and S. R. Ommen
Hypertrophic Cardiomyopathy, Sudden Death, and Implantable Cardiac Defibrillators: How Low the Bar?
JAMA,
July 25, 2007;
298(4):
452 - 454.
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Kubo, J. R. Gimeno, A. Bahl, U. Steffensen, M. Steffensen, E. Osman, R. Thaman, J. Mogensen, P. M. Elliott, Y. Doi, et al.
Prevalence, Clinical Significance, and Genetic Basis of Hypertrophic Cardiomyopathy With Restrictive Phenotype
J. Am. Coll. Cardiol.,
June 26, 2007;
49(25):
2419 - 2426.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P Melacini, B J Maron, F Bobbo, C Basso, B Tokajuk, M Zucchetto, G Thiene, and S Iliceto
Evidence that pharmacological strategies lack efficacy for the prevention of sudden death in hypertrophic cardiomyopathy
Heart,
June 1, 2007;
93(6):
708 - 710.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. E. Petersen, M. Jerosch-Herold, L. E. Hudsmith, M. D. Robson, J. M. Francis, H. A. Doll, J. B. Selvanayagam, S. Neubauer, and H. Watkins
Evidence for Microvascular Dysfunction in Hypertrophic Cardiomyopathy: New Insights From Multiparametric Magnetic Resonance Imaging
Circulation,
May 8, 2007;
115(18):
2418 - 2425.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. Ashrafian and H. Watkins
Reviews of Translational Medicine and Genomics in Cardiovascular Disease: New Disease Taxonomy and Therapeutic Implications: Cardiomyopathies: Therapeutics Based on Molecular Phenotype
J. Am. Coll. Cardiol.,
March 27, 2007;
49(12):
1251 - 1264.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Bayes-Genis
Hypertrophy and inflammation: too much for one heart
Eur. Heart J.,
March 5, 2007;
(2007)
ehm008v1.
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. Pablo Kaski, M. Teresa Tome Esteban, M. Lowe, S. Sporton, P. Rees, J. E Deanfield, W. J McKenna, and P. M Elliott
Outcomes after implantable cardioverter-defibrillator treatment in children with hypertrophic cardiomyopathy
Heart,
March 1, 2007;
93(3):
372 - 374.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. W. Heldman, K. C. Wu, T. P. Abraham, and D. E. Cameron
Myectomy or Alcohol Septal Ablation: Surgery and Percutaneous Intervention Go Another Round
J. Am. Coll. Cardiol.,
January 23, 2007;
49(3):
358 - 360.
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. Elliott, J. Gimeno, M. Tome, and W. McKenna
Left ventricular outflow tract obstruction and sudden death in hypertrophic cardiomyopathy
Eur. Heart J.,
December 2, 2006;
27(24):
3073 - 3073.
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. S. Maron, I. Olivotto, A. G. Zenovich, M. S. Link, N. G. Pandian, J. T. Kuvin, S. Nistri, F. Cecchi, J. E. Udelson, and B. J. Maron
Hypertrophic Cardiomyopathy Is Predominantly a Disease of Left Ventricular Outflow Tract Obstruction
Circulation,
November 21, 2006;
114(21):
2232 - 2239.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
Developed in Collaboration With the European Heart, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, et al.
ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death)
J. Am. Coll. Cardiol.,
September 5, 2006;
48(5):
e247 - e346.
[Full Text]
[PDF]
|
|
|
|
|
|
|
Writing Committee Members, D. P. Zipes, A. J. Camm, M. Borggrefe, A. E. Buxton, B. Chaitman, M. Fromer, G. Gregoratos, G. Klein, A. J. Moss, et al.
ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: A report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death) Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society
Europace,
September 1, 2006;
8(9):
746 - 837.
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. M. Elliott, J. R. Gimeno, M. T. Tome, J. Shah, D. Ward, R. Thaman, J. Mogensen, and W. J. McKenna
Left ventricular outflow tract obstruction and sudden death risk in patients with hypertrophic cardiomyopathy
Eur. Heart J.,
August 2, 2006;
27(16):
1933 - 1941.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. J. Maron, I. Olivotto, and M. S. Maron
The dilemma of left ventricular outflow tract obstruction and sudden death in hypertrophic cardiomyopathy: do patients with gradients really deserve prophylactic defibrillators?
Eur. Heart J.,
August 2, 2006;
27(16):
1895 - 1897.
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. M. Harris, P. Spirito, M. S. Maron, A. G. Zenovich, F. Formisano, J. R. Lesser, S. Mackey-Bojack, W. J. Manning, J. E. Udelson, and B. J. Maron
Prevalence, Clinical Profile, and Significance of Left Ventricular Remodeling in the End-Stage Phase of Hypertrophic Cardiomyopathy
Circulation,
July 18, 2006;
114(3):
216 - 225.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Y. Ho and C. E. Seidman
A Contemporary Approach to Hypertrophic Cardiomyopathy
Circulation,
June 20, 2006;
113(24):
e858 - e862.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. D'Andrea, P. Caso, S. Severino, S. Cuomo, G. Capozzi, P. Calabro, G. Cice, L. Ascione, M. Scherillo, and R. Calabro
Prognostic value of intra-left ventricular electromechanical asynchrony in patients with hypertrophic cardiomyopathy
Eur. Heart J.,
June 1, 2006;
27(11):
1311 - 1318.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Pedrote, F. J. Morales, L. Garcia-Riesco, and F. Errazquin
Documented exercise-induced cardiac arrest in a paediatric patient with hypertrophic cardiomyopathy
Europace,
June 1, 2006;
8(6):
430 - 433.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P M Elliott, J R Gimeno, R Thaman, J Shah, D Ward, S Dickie, M T Tome Esteban, and W J McKenna
Historical trends in reported survival rates in patients with hypertrophic cardiomyopathy
Heart,
June 1, 2006;
92(6):
785 - 791.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. Spirito and C. Autore
Management of hypertrophic cardiomyopathy.
BMJ,
May 27, 2006;
332(7552):
1251 - 1255.
[Full Text]
[PDF]
|
|
|
|
|
|
|
K.A.M. Majamaa-Voltti, S. Winqvist, A. M. Remes, U. Tolonen, J. Pyhtinen, S. Uimonen, M. Karppa, M. Sorri, K. Peuhkurinen, and K. Majamaa
A 3-year clinical follow-up of adult patients with 3243A>G in mitochondrial DNA
Neurology,
May 23, 2006;
66(10):
1470 - 1475.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. A. Cesario and G. W. Dec
Implantable Cardioverter- Defibrillator Therapy in Clinical Practice
J. Am. Coll. Cardiol.,
April 18, 2006;
47(8):
1507 - 1517.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. A. Strickberger, D. W. Benson, I. Biaggioni, D. J. Callans, M. I. Cohen, K. A. Ellenbogen, A. E. Epstein, P. Friedman, J. Goldberger, P. A. Heidenreich, et al.
AHA/ACCF Scientific Statement on the Evaluation of Syncope: From the American Heart Association Councils on Clinical Cardiology, Cardiovascular Nursing, Cardiovascular Disease in the Young, and Stroke, and the Quality of Care and Outcomes Research Interdisciplinary Working Group; and the American College of Cardiology Foundation In Collaboration With the Heart Rhythm Society
J. Am. Coll. Cardiol.,
January 17, 2006;
47(2):
473 - 484.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. A. Strickberger, D. W. Benson, I. Biaggioni, D. J. Callans, M. I. Cohen, K. A. Ellenbogen, A. E. Epstein, P. Friedman, J. Goldberger, P. A. Heidenreich, et al.
AHA/ACCF Scientific Statement on the Evaluation of Syncope: From the American Heart Association Councils on Clinical Cardiology, Cardiovascular Nursing, Cardiovascular Disease in the Young, and Stroke, and the Quality of Care and Outcomes Research Interdisciplinary Working Group; and the American College of Cardiology Foundation: In Collaboration With the Heart Rhythm Society: Endorsed by the American Autonomic Society
Circulation,
January 17, 2006;
113(2):
316 - 327.
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. M. Wolf, I. P. G. Moskowitz, S. Arno, D. M. Branco, C. Semsarian, S. A. Bernstein, M. Peterson, M. Maida, G. E. Morley, G. Fishman, et al.
Somatic events modify hypertrophic cardiomyopathy pathology and link hypertrophy to arrhythmia
PNAS,
December 13, 2005;
102(50):
18123 - 18128.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Kubo, H. Kitaoka, M. Okawa, Y. Matsumura, N. Hitomi, N. Yamasaki, T. Furuno, J. Takata, M. Nishinaga, A. Kimura, et al.
Lifelong Left Ventricular Remodeling of Hypertrophic Cardiomyopathy Caused by a Founder Frameshift Deletion Mutation in the Cardiac Myosin-Binding Protein C Gene Among Japanese
J. Am. Coll. Cardiol.,
November 1, 2005;
46(9):
1737 - 1743.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Schumacher, F. H. Gietzen, H. Neuser, J. Schummelfeder, M. Schneider, S. Kerber, R. Schimpf, C. Wolpert, and M. Borggrefe
Electrophysiological Characteristics of Septal Hypertrophy in Patients With Hypertrophic Obstructive Cardiomyopathy and Moderate to Severe Symptoms
Circulation,
October 4, 2005;
112(14):
2096 - 2101.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Thaman, P. M. Elliott, J. S. Shah, B. Mist, L. Williams, R. T. Murphy, W. J. McKenna, and M. P. Frenneaux
Reversal of Inappropriate Peripheral Vascular Responses in Hypertrophic Cardiomyopathy
J. Am. Coll. Cardiol.,
September 6, 2005;
46(5):
883 - 892.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
W. D. Binder, M. A. Fifer, M. E. King, and J. R. Stone
Case 26-2005 - A 48-Year-Old Man with Sudden Loss of Consciousness while Jogging
N. Engl. J. Med.,
August 25, 2005;
353(8):
824 - 832.
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Rickers, N. M. Wilke, M. Jerosch-Herold, S. A. Casey, P. Panse, N. Panse, J. Weil, A. G. Zenovich, and B. J. Maron
Utility of Cardiac Magnetic Resonance Imaging in the Diagnosis of Hypertrophic Cardiomyopathy
Circulation,
August 9, 2005;
112(6):
855 - 861.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
I. Olivotto, M. S. Maron, A. S. Adabag, S. A. Casey, D. Vargiu, M. S. Link, J. E. Udelson, F. Cecchi, and B. J. Maron
Gender-Related Differences in the Clinical Presentation and Outcome of Hypertrophic Cardiomyopathy
J. Am. Coll. Cardiol.,
August 2, 2005;
46(3):
480 - 487.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R Thaman, J R Gimeno, R T Murphy, T Kubo, B Sachdev, J Mogensen, P M Elliott, and W J McKenna
Prevalence and clinical significance of systolic impairment in hypertrophic cardiomyopathy
Heart,
July 1, 2005;
91(7):
920 - 925.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Isobe, H. Izawa, M. Iwase, M. Nanasato, M. Nonokawa, A. Ando, S. Ohshima, K. Nagata, K. Kato, T. Nishizawa, et al.
Cardiac 123I-MIBG Reflects Left Ventricular Functional Reserve in Patients with Nonobstructive Hypertrophic Cardiomyopathy
J. Nucl. Med.,
June 1, 2005;
46(6):
909 - 916.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. S. Adabag, S. A. Casey, M. A. Kuskowski, A. G. Zenovich, and B. J. Maron
Spectrum and prognostic significance of arrhythmias on ambulatory Holter electrocardiogram in hypertrophic cardiomyopathy
J. Am. Coll. Cardiol.,
March 1, 2005;
45(5):
697 - 704.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. A. Kaufmann and P. G. Camici
Myocardial Blood Flow Measurement by PET: Technical Aspects and Clinical Applications
J. Nucl. Med.,
January 1, 2005;
46(1):
75 - 88.
[Full Text]
[PDF]
|
|
|
|
|
|
|
P P Dimitrow and J S Dubiel
Echocardiographic risk factors predisposing to sudden cardiac death in hypertrophic cardiomyopathy
Heart,
January 1, 2005;
91(1):
93 - 94.
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. Mogensen, R. T. Murphy, T. Kubo, A. Bahl, J. C. Moon, I. C. Klausen, P. M. Elliott, and W. J. McKenna
Frequency and clinical expression of cardiac troponin I mutations in 748 consecutive families with hypertrophic cardiomyopathy
J. Am. Coll. Cardiol.,
December 21, 2004;
44(12):
2315 - 2325.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. J. Maron, J. A. Dearani, S. R. Ommen, M. S. Maron, H. V. Schaff, B. J. Gersh, and R. A. Nishimura
The case for surgery in obstructive hypertrophic cardiomyopathy
J. Am. Coll. Cardiol.,
November 16, 2004;
44(10):
2044 - 2053.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Boriani, B. J. Maron, W.-K. Shen, and P. Spirito
Prevention of Sudden Death in Hypertrophic Cardiomyopathy: But Which Defibrillator for Which Patient?
Circulation,
October 12, 2004;
110(15):
e438 - e442.
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. Faber, H. Seggewiss, D. Welge, D. Fassbender, H. K. Schmidt, U. Gleichmann, and D. Horstkotte
Echo-guided percutaneous septal ablation for symptomatic hypertrophic obstructive cardiomyopathy: 7 years of experience
Eur J Echocardiogr,
October 1, 2004;
5(5):
347 - 355.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. Thaman, J. R. Gimeno, S. Reith, M. T. T. Esteban, G. Limongelli, R. T. Murphy, B. Mist, W. J. McKenna, and P. M. Elliott
Progressive left ventricular remodeling in patients with hypertrophic cardiomyopathy and severe left ventricular hypertrophy
J. Am. Coll. Cardiol.,
July 21, 2004;
44(2):
398 - 405.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. P Frenneaux
Assessing the risk of sudden cardiac death in a patient with hypertrophic cardiomyopathy
Heart,
May 1, 2004;
90(5):
570 - 575.
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. J. Maron, W. J. McKenna, G. K. Danielson, L. J. Kappenberger, H. J. Kuhn, C. E. Seidman, P. M. Shah, W. H. Spencer III, P. Spirito, F. J. Ten Cate, et al.
American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy: a report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines
J. Am. Coll. Cardiol.,
November 5, 2003;
42(9):
1687 - 1713.
[Full Text]
[PDF]
|
|
|
|
|
|
|
Writing Committee Members, B. J. Maron, W. J. McKenna, G. K. Danielson, L. J. Kappenberger, H. J. Kuhn, C. E. Seidman, P. M. Shah, W. H. Spencer III, P. Spirito, et al.
American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy: A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines
Eur. Heart J.,
November 1, 2003;
24(21):
1965 - 1991.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Isobe, H. Izawa, Y. Takeichi, M. Nonokawa, M. Nanasato, A. Ando, K. Kato, M. Ikeda, T. Murohara, and M. Yokota
Relationship Between Exercise-Induced Myocardial Ischemia and Reduced Left Ventricular Distensibility in Patients with Nonobstructive Hypertrophic Cardiomyopathy
J. Nucl. Med.,
November 1, 2003;
44(11):
1717 - 1724.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A Woo, H Rakowski, J C Liew, M-S Zhao, C-C Liew, T G Parker, M Zeller, E D Wigle, and M J Sole
Mutations of the {beta} myosin heavy chain gene in hypertrophic cardiomyopathy: critical functional sites determine prognosis
Heart,
October 1, 2003;
89(10):
1179 - 1185.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
Top
Abstract
Methods