Efficacy of Implantable CardioverterDefibrillators for the Prevention of Sudden Death in Patients with Hypertrophic Cardiomyopathy
Barry J. Maron, M.D., Win-Kuang Shen, M.D., Mark S. Link, M.D., Andrew E. Epstein, M.D., Adrian K. Almquist, M.D., James P. Daubert, M.D., Gust H. Bardy, M.D., Stefano Favale, M.D., Robert F. Rea, M.D., Giuseppe Boriani, M.D., N.A. Mark Estes, M.D., Paolo Spirito, M.D., Susan A. Casey, R.N., Marshall S. Stanton, M.D., and Sandro Betocchi, M.D.
Background Hypertrophic cardiomyopathy is a genetic diseaseassociated with a risk of ventricular tachyarrhythmias and suddendeath, especially in young patients.
Methods We conducted a retrospective multicenter study of theefficacy of implantable cardioverterdefibrillators inpreventing sudden death in 128 patients with hypertrophic cardiomyopathywho were judged to be at high risk for sudden death.
Results At the time of the implantation of the defibrillator,the patients were 8 to 82 years old (mean [±SD], 40±16),and 69 patients (54 percent) were less than 41 years old. Theaverage follow-up period was 3.1 years. Defibrillators wereactivated appropriately in 29 patients (23 percent), by providingdefibrillation shocks or antitachycardia pacing, with the restorationof sinus rhythm; the average age at the time of the interventionwas 41 years. The rate of appropriate defibrillator dischargewas 7 percent per year. A total of 32 patients (25 percent)had episodes of inappropriate discharges. In the group of 43patients who received defibrillators for secondary prevention(after cardiac arrest or sustained ventricular tachycardia),the devices were activated appropriately in 19 patients (11percent per year). Of 85 patients who had prophylactic implantsbecause of risk factors (i.e., for primary prevention), 10 hadappropriate interventions (5 percent per year). The intervalbetween implantation and the first appropriate discharge washighly variable but was substantially prolonged (four to nineyears) in six patients. In all 21 patients with stored electrographicdata and appropriate interventions, the interventions were triggeredby ventricular tachycardia or fibrillation.
Conclusions Ventricular tachycardia or fibrillation appearsto be the principal mechanism of sudden death in patients withhypertrophic cardiomyopathy. In high-risk patients with hypertrophiccardiomyopathy, implantable defibrillators are highly effectivein terminating such arrhythmias, indicating that these deviceshave a role in the primary and secondary prevention of suddendeath.
Hypertrophic cardiomyopathy is a genetically determined myocardialdisease with a diverse natural history.1,2,3,4,5,6,7 Since asubgroup of patients with hypertrophic cardiomyopathy are athigh risk for sudden death, there has been considerable interestin risk stratification1,3,8,9,10,11 and appropriate preventivemeasures.3,11,12 There are few data supporting the efficacyof prophylactic treatment with amiodarone in patients with hypertrophiccardiomyopathy,13 and the frequent adverse consequences of long-termuse of this drug limit its application for the prevention ofsudden death in young patients with this disease.
The implantable cardioverterdefibrillator14 is widelyaccepted as a definitive treatment for the prevention of suddendeath, principally in high-risk patients with ischemic heartdisease.15,16,17 The superiority of the implantable defibrillatorover antiarrhythmic-drug treatment has been documented in prospective,randomized trials.15,16 There are few data, however, on theefficacy of implantable cardioverterdefibrillators inpatients with hypertrophic cardiomyopathy.12,18,19,20 We conducteda retrospective, multicenter study to determine the efficacyof defibrillators in a group of patients with hypertrophic cardiomyopathywho were considered to be at high risk for sudden death.
Methods
Selection of Patients
Patients were recruited at 19 institutions in the United Statesand Italy. U.S. centers were invited to participate in the studybecause of their experience with the use of implantable defibrillatorsin patients with hypertrophic cardiomyopathy and because theyroutinely offered implantation of a defibrillator as a treatmentoption to patients judged to be at high risk (e.g., those withprior cardiac arrest or sustained ventricular tachycardia).In Italy, all major electrophysiology centers were invited toparticipate in the study. The participating centers enrolledan average of seven patients each, and eight institutions enrolledat least five patients.
The 128 patients enrolled in the study were the consecutivepatients at each institution who met the following enrollmentcriteria: an unequivocal diagnosis of hypertrophic cardiomyopathybased on two-dimensional echocardiographic evidence of a hypertrophiedand nondilated left ventricle in the absence of another cardiacor systemic disease that could have accounted for the hypertrophy7,21;successful implantation of a cardioverterdefibrillatorfor the prevention of sudden death; and a follow-up period ofat least three months after implantation of the defibrillator,with documentation of the clinical outcome as of September 1,1998 (for 124 patients), the time of death (2 patients), orthe removal of the device because of infection (2 patients).One patient with severe symptoms (who had survived cardiac arrestat the age of 16 years) died at the time of thoracotomy forimplantation and is not included in the analysis.
Defibrillators
The defibrillators were implanted between December 1984 andJune 1998; 101 patients (79 percent) underwent implantationafter January 1, 1994. Implantation was performed through athoracotomy with epicardial lead systems in 22 patients (17percent) or transvenously in 106 (83 percent). Most of the deviceswere third-generation defibrillators with the capacity to provideantitachycardia and antibradycardia pacing and the potentialfor pectoral implantation; 95 (74 percent) of the devices haddiagnostic memory and the ability to record and store electrocardiographicdata, including intracardiac electrograms, for subsequent review.22
Defibrillation thresholds were routinely tested to documentsuccessful termination of ventricular tachyarrhythmias. Programmedantitachycardia pacing23 was activated at the discretion ofthe investigator. Stored data were reviewed after all discharges.
Interpretation of Intracardiac Electrograms
Stored data were analyzed to classify the arrhythmias responsiblefor precipitating defibrillator discharges, according to thefollowing definitions. Ventricular fibrillation or flutter wasdefined as regular or irregular tachycardia with regard to QRSor electrographic polarity, amplitude, morphology, and sequence,with a mean cycle length of 240 msec or less. Ventricular tachycardiawas defined as regular (monomorphic) or irregular (polymorphic)tachycardia with regard to QRS or electrographic polarity, amplitude,and morphology, with a mean cycle length of more than 240 msec.Atrial flutter was defined as regular tachycardia with a ventricularcycle length of 350 to 450 msec and with no differences in electrographicmorphology and polarity as compared with sinus rhythm. Atrialfibrillation was defined as irregular tachycardia with morethan 60 msec between consecutive complexes and no differencesin QRS morphology as compared with sinus rhythm. Sinus tachycardiawas defined as regular tachycardia with a gradual accelerationidentical to that recorded during sinus rhythm and a mean rateexceeding the programmed cutoff rate.
Classification of Discharges as Appropriate or Inappropriate
Defibrillator discharges that were considered appropriate includedautomatic defibrillation shocks or programmed antitachycardiaoverdrive pacing triggered by ventricular tachycardia or fibrillationand documented by stored intracardiac electrographic or cycle-lengthdata. For events occurring in patients who had defibrillatorswithout the capacity to store electrographic data, dischargeswere judged to be appropriate on the basis of clinical findingsthat strongly suggested the presence of ventricular arrhythmia(i.e., symptoms such as presyncope or syncope immediately beforethe discharge and the absence of these symptoms immediatelyafterward).
For patients with defibrillators that stored electrographicdata, inappropriate discharges were defined as those triggeredby a rapid ventricular rate exceeding the programmed thresholdrate as a consequence of supraventricular tachycardia, exercise-relatedsinus tachycardia, or a malfunction of the device. For patientswith defibrillators that did not store electrographic data,discharges were defined as inappropriate if they were not precededby symptoms, if circumstances suggested the presence of sinustachycardia due to emotional or physical stress, or if therewas a malfunction of the device.
Each investigator initially classified a discharge as appropriateor inappropriate. For the group of 40 patients with dischargesinitially classified as appropriate, the stored electrographicdata or clinical circumstances at the time of the dischargewere subsequently reviewed independently by two of the investigators,who were experienced electrophysiologists.
The two independent reviews were concordant for 38 of the 40patients (95 percent); for the other 2 patients, an agreementwas reached. On the basis of these reviews, discharges wereclassified as appropriate in 29 patients and as inappropriatein 11 patients.
Statistical Analysis
Continuous data are expressed as means ±SD. Cumulativerates of appropriate discharges were estimated by the KaplanMeiermethod.24
Results
Characteristics of the Patients
At the time of implantation, the 128 patients were 8 to 82 yearsold (mean age, 40±16); 69 patients (54 percent) wereless than 41 years old (Figure 1) and 88 patients (69 percent)were male (Table 1). A total of 126 patients survived to theend of the follow-up period. Two patients (2 percent) with end-stagehypertrophic cardiomyopathy, systolic dysfunction, and refractorycongestive heart failure died after defibrillator dischargesfailed to reverse ventricular tachyarrhythmias.25
Figure 1. Age at the Time of Implantation of a Defibrillator in 128 Patients with Hypertrophic Cardiomyopathy Who Were Judged to Be at High Risk for Sudden Death.
The age distribution is shown according to whether the defibrillator was implanted for primary or secondary prevention.
Table 1. Clinical and Echocardiographic Data on 128 Patients with Hypertrophic Cardiomyopathy Who Received Implantable CardioverterDefibrillators.
Two patients had an apical aneurysm associated with a midcavityobstruction,4 12 had undergone prior septal myotomymyectomy,1,2,3,4,7and 6 had end-stage disease with severe systolic dysfunctionand cavity dilatation.26 The ejection fraction, estimated onthe basis of echocardiography, was at least 55 percent in allthe patients except the eight with end-stage disease or an aneurysm.
Reasons for Implantation of Defibrillators
In 43 patients (34 percent), with a mean age of 40±19years, defibrillators were implanted for secondary preventionafter either resuscitation from cardiac arrest (with documentedventricular fibrillation)27 or sustained, spontaneous ventriculartachycardia. In the remaining 85 patients (66 percent), witha mean age of 40±15 years, defibrillators were implantedprophylactically for primary prevention of sudden death. Thepredominant clinical reasons for these prophylactic implantations,either alone or in combination, were syncope (in 41 patients),a family history of one or more sudden deaths due to hypertrophiccardiomyopathy (in 39), nonsustained ventricular tachycardiaon Holter electrocardiographic monitoring (in 32), and a left-ventricular-wallthickness of at least 30 mm (in 10). In addition, 56 patientshad inducible ventricular tachycardia or fibrillation duringelectrophysiologic testing.
Inappropriate Discharges
Of the 128 patients, 32 (25 percent) had one or more episodesof inappropriate discharges due to sinus tachycardia (in 13patients), atrial fibrillation with a rapid ventricular rate(in 10), or lead dislodgment, disruption, or oversensing (in9). Of these 32 patients, 13 had only 1 inappropriate discharge,and 19 had more than 1, including 5 patients who had 6 or moredischarges (average, 2.6). In 7 of the 32 patients with inappropriatedischarges, there was also at least one appropriate discharge.
Appropriate Discharges
Twenty-nine of the 128 patients (23 percent) had one or moreappropriate discharges. Of these 29 patients, 19 were in thegroup of 43 patients who had received defibrillators for secondaryprevention (44 percent of the group), and 10 were in the groupof 85 patients who had received defibrillators for primary prevention(12 percent of the group). Twenty-one of the 29 patients withappropriate discharges had defibrillators that stored electrographicdata. The interventions were defibrillation shocks in 18 ofthese 21 patients and antitachycardia pacing in 3. Dischargesin the other eight patients were considered to be appropriateon the basis of the clinical findings.
The age at which the first appropriate discharge occurred rangedfrom 11 to 83 years (mean, 41). Patients with appropriate dischargeswere most likely to be less than 31 years old or more than 55years old (Figure 2).
Figure 2. Age at the Time of the First Appropriate Defibrillator Discharge in 29 Patients.
The bar for each age category shows the number of patients with appropriate discharges expressed as a proportion of the patients who were in the same age category at the time that the defibrillator was implanted.
In the group of 10 patients who had appropriate discharges afterimplantation of defibrillators for primary prevention, the clinicalreasons for implantation were as follows: syncope unassociatedwith sustained ventricular tachycardia or hemodynamic compromisein 5 patients (syncope on exertion in 2 and at rest in 3, withrecurrent syncope in 2 of the 5), each with inducible ventriculartachycardia or fibrillation on electrophysiologic testing; nonsustainedventricular tachycardia in 2; massive left ventricular hypertrophyin 2; and a family history of sudden death in 1, with inducibleventricular arrhythmias in the patient. However, the 5 patientswith syncope represented only 12 percent of the 41 patientsin whom syncope was a major justification for prophylactic implantationof a defibrillator.
At the time of implantation, 26 of the 29 patients with appropriatedischarges were asymptomatic or had mild functional limitation(New York Heart Association class I or II), and 3 had severesymptoms (class III). Information about activity at the timeof the first appropriate discharge was available for 28 patients;25 were sedentary, asleep, or engaged in mild physical activity(e.g., walking or eating), and 3 were performing activitiesthat involved physical exertion. Only 3 of 29 patients (10 percent)had left ventricular outflow obstruction (i.e., a gradient ofat least 30 mm Hg). At the time of implantation, the cardiacrhythm was normal sinus in all the patients, but three had ahistory of atrial fibrillation.
Fifteen of the 29 patients with appropriate discharges (52 percent)received antiarrhythmic drugs (11 received amiodarone, 4 sotalol,and 2 disopyramide, alone or in combination) after implantation,as compared with 21 of the 99 patients (21 percent) with inappropriatedischarges or none.
The interval between implantation of the defibrillator and theinitial appropriate discharge28 ranged from 2 weeks to 9 years(mean, 23 months) (Figure 3). The interval was four or moreyears in six patients. Conversely, 12 patients had an initialappropriate discharge less than six months after implantation;in 9 of these 12 patients, there was at least one additionaldischarge (more than one year later in 6 patients).
Figure 3. Interval between Implantation of the Defibrillator and the First Appropriate Discharge in 29 Patients.
Discharge Rates
With an average follow-up of 3.1 years after implantation, therate of appropriate discharges for the overall study group was7 percent per year. In the group of 43 patients with defibrillatorsimplanted for secondary prevention, the rate of appropriatedischarges was 11 percent per year (mean follow-up period, 4.0years). In the group of 85 patients with devices implanted forprimary prevention, the estimated rate of appropriate dischargeswas 5 percent per year (mean follow-up period, 2.6 years). Cumulativerates of first appropriate discharges (Figure 4) were significantlyhigher in the secondary-prevention group than in the primary-preventiongroup (P=0.004).
Figure 4. Estimated Cumulative Rates of First Appropriate Discharges, Calculated Separately for the 85 Patients with Defibrillators for Primary Prevention and the 43 Patients with Defibrillators for Secondary Prevention.
Multiple Discharges
Of the 29 patients with appropriate discharges, 8 had a singledefibrillation shock or cardioversion, and 12 had two to fiveinterventions. Nine patients had more than five interventions,including six with clusters of multiple and consecutive appropriatedischarges (three or more within 24 hours).29
Triggering Events
Analysis of the stored electrographic data, which were availablefor 21 of the patients with appropriate discharges, showed thatthe intervention was triggered by ventricular tachycardia in10 patients and by ventricular fibrillation in 9 patients, precededby ventricular tachycardia in 3 of the 9 (Figure 5). In twoother patients, multiple discharges were preceded by eitherventricular tachycardia or fibrillation.
Figure 5. Stored Ventricular Electrogram from an Asymptomatic 35-Year-Old Man Who Received a Defibrillator Prophylactically Because of a Family History of Sudden Death Related to Hypertrophic Cardiomyopathy and Marked Ventricular Septal Thickness (31 mm).
The electrogram was obtained four years eight months after implantation of the defibrillator. The data were recorded at 1:20 a.m. while the patient was asleep. A continuous recording, at 25 mm per second, is shown in four panels, with the tracing recorded from left to right in each. After four beats of sinus rhythm, ventricular tachycardia begins abruptly, at a rate of 200 beats per minute (Panel A). The defibrillator senses ventricular tachycardia and charges (Panel B). Ventricular tachycardia deteriorates into ventricular fibrillation (Panel C). The defibrillator discharges appropriately (a 20-J shock denoted by the bar, Panel D) during ventricular fibrillation and restores sinus rhythm. Adapted from Maron et al.30
Complications
Complications of defibrillator therapy occurred in 18 patients.These included lead malfunctions with fracture or disruption(in 12 patients, including 9 with inappropriate discharges),infection requiring removal of the defibrillator (in 2), andsubclavian thrombus, hemorrhage requiring thoracotomy, hematoma,and clinical depression in 1 patient each.
Discussion
Sudden death has been recognized as a devastating consequenceof hypertrophic cardiomyopathy since the initial descriptionof the disease in 1958.31 Many authors have emphasized thatsudden deaths frequently occur in young, asymptomatic patients,with reported annual mortality rates as high as 4 to 6 percent.32,33,34,35,36Prevention of sudden death in patients with hypertrophic cardiomyopathycontinues to be a major challenge.
The implantable cardioverterdefibrillator is clearlyeffective in terminating life-threatening ventricular arrhythmiasin patients with coronary artery disease.14,15,16,17 Furthermore,the evolution of the implantable defibrillator from a devicewith epicardial leads that requires thoracotomy for implantationto a transvenous endocardial electrode system with pectoralimplantation of the pulse generator has facilitated its clinicaluse. However, studies of defibrillators in small numbers ofpatients with hypertrophic cardiomyopathy have had mixed results,12,18,19,20leaving the role of the implantable defibrillator in the managementof this disease unresolved.37 Indeed, in the joint recommendationsof the American College of Cardiology and the American HeartAssociation, hypertrophic cardiomyopathy is not a standard indicationfor implantation of a defibrillator.38
The results of our multicenter study establish an importantrole for implantable-defibrillator therapy in the preventionof sudden death in high-risk patients with hypertrophic cardiomyopathy.The defibrillator proved reliable in sensing and terminatinglife-threatening ventricular tachyarrhythmias. Appropriate dischargesoccurred in almost 25 percent of the 128 patients during anaverage follow-up period of three years. The annual dischargerate was 7 percent per year; furthermore, in about 70 percentof the patients with appropriate discharges, there were multipleappropriate interventions. It should be emphasized, however,that the discharge rates in this study may have been influencedby the selection of patients and are most appropriately regardedas estimates. More than one third of the patients were takingamiodarone at the time of an appropriate discharge afinding that highlights the superiority of the defibrillatorin preventing sudden death.15
Discharges were most frequent in patients who had received defibrillatorsfor secondary prevention of sudden death (i.e., those with priorcardiac arrest or spontaneous, sustained ventricular tachycardia).In over 40 percent of these patients, the defibrillator wasactivated on one or more occasions during a relatively shortfollow-up period. The frequent recurrence of potentially lethalventricular tachyarrhythmias after cardiac arrest is consistentwith reports on the clinical course of hypertrophic cardiomyopathyin the era before defibrillators were available.27 Nevertheless,in our study, the implantable defibrillator did not providecomplete protection against sudden death. The device failedto prevent death in two patients who had end-stage hypertrophiccardiomyopathy with severe systolic dysfunction and heart failure.26
The rate of appropriate discharges was about 5 percent per yearin the group of patients who had received defibrillators solelyfor primary prevention (i.e., those with one or more risk factorsfor sudden death). By extrapolating from this discharge rate,one could predict that within 10 years, almost 50 percent ofthe defibrillators prophylactically implanted in young patientswould discharge and prevent sudden death. Also, the 5 percentannual discharge rate in our study is remarkably similar tothe rates reported in selected high-risk patients with hypertrophiccardiomyopathy at tertiary referral centers.32,33,34,35,36
In the primary-prevention subgroup, the patients with appropriatedischarges were most likely to have received a defibrillatorbecause of syncope. However, our investigation was not designedto establish firm guidelines for prophylactic implantation ofdefibrillators in patients with hypertrophic cardiomyopathy,and one must be cautious in interpreting these findings, givenour study design. The use of defibrillators for primary preventionin this retrospective study, although based on the generallyaccepted risk factors for sudden death in patients with hypertrophiccardiomyopathy,1,3 was not systematic or controlled and reliedlargely on the participating electrophysiologists' evaluationof the level of risk in their patients.
Since some patients with hypertrophic cardiomyopathy are atrisk for sudden death over a long period, which may extend throughoutmidlife and thereafter,39 the implantable defibrillator hasthe potential to prolong life substantially in such patients.In patients with coronary artery disease, defibrillators areimplanted at a relatively advanced age (average, about 65 years),whereas patients with hypertrophic cardiomyopathy who are athigh risk for sudden death are often much younger and have fewor no symptoms. Indeed, the average age of our patients at thetime of implantation was 40 years (more than 25 percent wereunder the age of 31 years), and the average age at the timeof the first appropriate defibrillator discharge was only 41years. Despite our relatively short period of follow-up, insome cases, there was a particularly long interval between implantationand the first appropriate discharge, with a maximal intervalof nine years; in 21 percent of the patients with appropriatedischarges, the interval was at least four years. Thus, in patientswith hypertrophic cardiomyopathy, the implanted defibrillatormay remain dormant for a long period but eventually dischargeappropriately. As a result, the decision to implant a cardioverterdefibrillatorin a high-risk patient is likely to represent a lifelong preventivemeasure.
It has been difficult to identify the arrhythmias responsiblefor sudden death in patients with hypertrophic cardiomyopathy,largely because of the paucity of electrocardiographic recordingsat the time of such events.40 Considerable attention has beenfocused on primary ventricular tachyarrhythmias,33,34,40 butalternative mechanisms have been proposed.19 In our study, defibrillatorsin 21 patients recorded and stored electrographic data duringappropriate discharges. In each instance, ventricular tachycardiaor fibrillation was the rhythm that activated the device. Thesefindings support the hypothesis that ventricular tachycardiaor fibrillation is often the primary cause of sudden death inpatients with hypertrophic cardiomyopathy, with the arrhythmiaemanating largely from a substrate of electrical instabilityand distorted electrophysiologic transmission as a result ofthe characteristically disorganized arrangement of cardiac-musclecells.41 The occurrence of bradycardia-mediated events couldnot be ruled out in our study population because of the backuppacing capacity in many of the devices.
Our data support the view that the implantable defibrillatorcan be a lifesaving device in patients with hypertrophic cardiomyopathyand support its use for both secondary and primary prevention.The defibrillator proved effective in terminating ventriculartachycardia or fibrillation despite the substantial increasesin heart mass and wall thickness that are characteristic ofthis disease.1,2,3,4,6,21 However, it is also important to recognizethat the implantable defibrillator is expensive, that its availabilityis still limited in many countries, that it may be associatedwith complications and may be activated inappropriately, andthat it is not invariably effective. Although the rates of appropriatedischarges in our patients with hypertrophic cardiomyopathywere lower than those reported in patients with ischemic heartdisease,15,16 they are nevertheless substantial when viewedin the context of a disease that frequently affects young patientswithout symptoms of congestive heart failure and with preservedsystolic function. Given the protection afforded by an implantabledefibrillator, such patients may survive for many decades, withnormal or nearly normal life expectancy.
Since our study was retrospective and uncontrolled, with possiblelimitations related to the selection of patients, we wish tobe cautious in drawing conclusions about the role of implantabledefibrillators in the management of hypertrophic cardiomyopathy.We can reasonably conclude that the defibrillator was effectivein preventing sudden death in a group of patients with hypertrophiccardiomyopathy who were considered to be at high risk. However,we did not compare the outcome in this group with that in agroup of patients with hypertrophic cardiomyopathy and a similarclinical profile who were evaluated at the same centers butwho did not receive defibrillators. It would be difficult toconduct prospective, randomized trials of the efficacy of defibrillatorsas compared with that of drug therapy in patients with hypertrophiccardiomyopathy because of ethical considerations, the relativelylow prevalence of the disease in the general population,1 andthe relatively low event rate,1,3 as well as major practicallimitations (e.g., the frequently long dormant period betweenimplantation of the defibrillator and a first discharge).
Although selection bias could have influenced the precisionof the estimated rates of appropriate discharges reported here,this factor does not obscure the basic message that the implantablecardioverterdefibrillator provides lifesaving protectionby effectively terminating ventricular tachycardia or fibrillationin patients with hypertrophic cardiomyopathy who have few orno symptoms, many of whom are young. The use of a defibrillatoris therefore warranted, for both primary and secondary preventionof sudden death, in high-risk patients with hypertrophic cardiomyopathy.
Funded in part by a grant from Medtronic, which manufacturesimplantable cardioverterdefibrillators.
We are indebted to Drs. Michel Mirowski and Morton M. Mowerfor their pioneering efforts, without which the clinical useof the implantable cardioverterdefibrillator would nothave been possible, and to Dr. Cynthia De Souza, of Medtronic,Minneapolis, for statistical support.
* Participating centers and investigators are listed in the Appendix.
Source Information
From the Minneapolis Heart Institute Foundation, Minneapolis (B.J.M., A.K.A.); the Mayo Clinic, Rochester, Minn. (W.-K.S., R.F.R.); New England Medical Center and Tufts University School of Medicine, Boston (M.S.L., N.A.M.E.); the University of Alabama at Birmingham, Birmingham (A.E.E.); University of Rochester Medical Center, Rochester, N.Y. (J.P.D.); University of Washington Medical Center, Seattle (G.H.B.); Università degli Studi di Bari, Bari, Italy (S.F.); Università di Bologna, Bologna, Italy (G.B.); and Ente Ospedaliero Ospedale Galliera, Genoa, Italy (P.S.).
Address reprint requests to Dr. Maron at the Minneapolis Heart Institute Foundation, 920 E. 28th St., Suite 40, Minneapolis, MN 55407, or at gencvres{at}skypoint.com.
Wigle ED, Rakowski H, Kimball BP, Williams WG. Hypertrophic cardiomyopathy: clinical spectrum and treatment. Circulation 1995;92:1680-1692. [Free Full Text]
Spirito P, Seidman CE, McKenna WJ, Maron BJ. The management of hypertrophic cardiomyopathy. N Engl J Med 1997;336:775-785. [Free Full Text]
Wigle ED, Sasson Z, Henderson MA, et al. Hypertrophic cardiomyopathy: the importance of the site and the extent of hypertrophy: a review. Prog Cardiovasc Dis 1985;28:1-83. [CrossRef][Medline]
Maron BJ, Moller JH, Seidman CE, et al. Impact of laboratory molecular diagnosis on contemporary diagnostic criteria for genetically transmitted cardiovascular diseases: hypertrophic cardiomyopathy, long-QT syndrome, and Marfan syndrome: a statement for healthcare professionals from the councils on clinical cardiology, cardiovascular disease in the young, and basic science, American Heart Association. Circulation 1998;98:1460-1471. [Free Full Text]
Maron BJ, Bonow RO, Cannon RO III, Leon MB, Epstein SE. Hypertrophic cardiomyopathy: interrelations of clinical manifestations, pathophysiology, and therapy. N Engl J Med 1987;316:780-9, 844. [Medline]
Sadoul N, Prasad K, Elliott PM, Bannerjee S, Frenneaux MP, McKenna WJ. Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy. Circulation 1997;96:2987-2991. [Free Full Text]
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 patient population with hypertrophic cardiomyopathy. J Am Coll Cardiol 1999;33:2044-2051. [Free Full Text]
Spirito P, Rapezzi C, Autore C, et al. Prognosis of asymptomatic patients with hypertrophic cardiomyopathy and nonsustained ventricular tachycardia. Circulation 1994;90:2743-2747. [Free Full Text]
McKenna WJ, Camm AJ. Sudden death in hypertrophic cardiomyopathy: assessment of patients at high risk. Circulation 1989;80:1489-1492. [Free Full Text]
Primo J, Geelen P, Brugada J, et al. Hypertrophic cardiomyopathy: role of the implantable cardioverter-defibrillator. J Am Coll Cardiol 1998;31:1081-1085. [Free Full Text]
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. [Free Full Text]
Mirowski M, Reid PR, Mower MM, et al. Termination of malignant ventricular arrhythmias with an implanted automatic defibrillator in human beings. N Engl J Med 1980;303:322-324. [Medline]
The Antiarrhythmics versus Implantable Defibrillators (AVID) Investigators. A comparison of antiarrhythmic-drug therapy with implantable defibrillators in patients resuscitated from near-fatal ventricular arrhythmias. N Engl J Med 1997;337:1576-1583. [Free Full Text]
Moss AJ, Hall WJ, Cannom DS, et al. Improved survival with an implanted defibrillator in patients with coronary disease at high risk for ventricular arrhythmia. N Engl J Med 1996;335:1933-1940. [Free Full Text]
Zipes DP, Roberts D. Results of the international study of the implantable pacemaker cardioverter-defibrillator: a comparison of epicardial and endocardial lead systems. Circulation 1995;92:59-65. [Free Full Text]
Silka MJ, Kron J, Dunnigan A, Dick M II. Sudden cardiac death and the use of implantable cardioverter-defibrillators in pediatric patients. Circulation 1993;87:800-807. [Free Full Text]
Elliott PM, Sharma S, Varnava A, Poloniecki J, Rowland E, McKenna WJ. Survival after cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 1999;33:1596-1601. [Free Full Text]
Zhu D, Sun H, Hill R, Roberts R. The value of electrophysiology study and prophylactic implantation of cardioverter defibrillator in patients with hypertrophic cardiomyopathy. Pacing Clin Electrophysiol 1998;21:299-302. [CrossRef][Medline]
Klues HG, Schiffers A, Maron BJ. Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy: morphologic observations and significance as assessed by two-dimensional echocardiography in 600 patients. J Am Coll Cardiol 1995;26:1699-1708. [Abstract]
Rüppel R, Schlüter CA, Boczor S, et al. Ventricular tachycardia during follow-up in patients resuscitated from ventricular fibrillation: experience from stored electrograms of implantable cardioverter-defibrillators. J Am Coll Cardiol 1998;32:1724-1730. [Free Full Text]
Schaumann A, von zur Mühlen F, Herse B, Gonska B-D, Kreuzer H. Empirical versus tested antitachycardia pacing in implantable cardioverter defibrillators: a prospective study including 200 patients. Circulation 1998;97:66-74. [Free Full Text]
Kaplan EL, Meier P. Nonparametric estimation from incomplete observations. J Am Stat Assoc 1958;53:457-81.
Pires LA, Lehmann MH, Steinman RT, Baga JJ, Schuger CD. Sudden death in implantable cardioverter-defibrillator recipients: clinical context, arrhythmic events and device responses. J Am Coll Cardiol 1999;33:24-32. [Free Full Text]
Maron BJ, Spirito P. Implications of left ventricular remodeling in hypertrophic cardiomyopathy. Am J Cardiol 1998;81:1339-1344. [CrossRef][Medline]
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]
Myerburg RJ, Luceri RM, Thurer R, et al. Time to first shock and clinical outcome in patients receiving an automatic implantable cardioverter-defibrillator. J Am Coll Cardiol 1989;14:508-514. [Abstract]
Credner SC, Klingenheben T, Mauss O, Sticherling C, Hohnloser SH. Electrical storm in patients with transvenous implantable cardioverter-defibrillators: incidence, management and prognostic implications. J Am Coll Cardiol 1998;32:1909-1915. [Free Full Text]
Maron BJ, Casey SA, Almquist AK. Aborted sudden cardiac death in hypertrophic cardiomyopathy. J Cardiovasc Electrophysiol 1999;10:263-263. [CrossRef][Medline]
Teare D. Asymmetrical hypertrophy of the heart in young adults. Br Heart J 1958;20:1-8.
Shah PM, Adelman AG, Wigle ED, et al. The natural (and unnatural) history of hypertrophic obstructive cardiomyopathy. Circ Res 1974;35:Suppl II:II-179.
McKenna WJ, Deanfield JE. Hypertrophic cardiomyopathy: an important cause of sudden death. Arch Dis Child 1984;59:971-975. [Free Full Text]
McKenna W, 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]
Fiddler GI, Tajik AJ, Weidman WH, McGoon DC, Ritter DG, Giuliani ER. Idiopathic hypertrophic subaortic stenosis in the young. Am J Cardiol 1978;42:793-799. [CrossRef][Medline]
Maron BJ, Henry WL, Clark CE, Redwood DR, Roberts WC, Epstein SE. Asymmetric septal hypertrophy in childhood. Circulation 1976;53:9-19. [Free Full Text]
Borggrefe M, Breithardt G. Is the implantable defibrillator indicated in patients with hypertrophic cardiomyopathy and aborted sudden death? J Am Coll Cardiol 1998;31:1086-1088. [Free Full Text]
Gregoratos G, Cheitlin MD, Conill A, et al. ACC/AHA guidelines for implantation of cardiac pacemakers and antiarrhythmia devices: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Pacemaker Implantation). J Am Coll Cardiol 1998;31:1175-1209. [Free Full Text]
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. [Erratum, JAMA 1999;281:2288.] [Free Full Text]
Nicod P, Polikar R, Peterson KL. Hypertrophic cardiomyopathy and sudden death. N Engl J Med 1988;318:1255-1257. [Medline]
Maron BJ, Roberts WC. Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum of patients with hypertrophic cardiomyopathy. Circulation 1979;59:689-706. [Free Full Text]
Appendix
The following centers and investigators participated in thestudy: Mayo Clinic, Rochester, Minn. W.-K. Shen, M.S.Stanton, and R.F. Rea; Azienda Ospedaliero S. Maria della Misericordia,Udine, Italy A. Proclemer; Georgetown University MedicalCenter, Washington, D.C. A.J. Solomon; Minneapolis HeartInstitute Foundation, Minneapolis A.K. Almquist, S.A.Casey, and B.J. Maron; New England Medical Center, Boston M.S. Link and N.A.M. Estes III; North Shore University Hospital,Manhasset, N.Y. M. Ovadia; Ospedale Civile, Asti, Italy R. Massa; Ente Ospedaliero Ospedale Galliera, Genoa,Italy P. Spirito and M. Berisso; Ospedale Maggiore dellaCarità, Novara, Italy E. Occhetta; Ospedale Niguarda-CáGranda, Milan, Italy M. Lunati; Ospedale S. FilippoNeri, Rome M. Santini and R. Ricci; Ospedale San Gerardo,Monza, Italy A. Vincenti; St. Luke'sRooseveltHospital Center, New York M.V. Sherrid and F. Ehlert;Università degli Studi di Bari, Bari, Italy S.Favale; Ospedale S. Orsola, Università di Bologna, Bologna,Italy G. Boriani and C. Rapezzi; Università FedericoII, Naples, Italy S. Betocchi; Cattedra di Cardiologia,Università di Milano, Milan, Italy P. Della Bella;University of Alabama at Birmingham, Birmingham A.E.Epstein; De Paul Hospital, Norfolk, Va. J.M. Herre;University of Rochester Medical Center, Rochester, N.Y. J.P. Daubert; and the University of Washington Medical Center,Seattle G.H. Bardy and J. Anderson.
Myerburg, R. J., Reddy, V., Castellanos, A.
(2009). Indications for implantable cardioverter-defibrillators based on evidence and judgment.. J Am Coll Cardiol
54: 747-763
[Abstract][Full Text]
Leonardi, S., Raineri, C., De Ferrari, G. M., Ghio, S., Scelsi, L., Pasotti, M., Tagliani, M., Valentini, A., Dore, R., Raisaro, A., Arbustini, E.
(2009). Usefulness of cardiac magnetic resonance in assessing the risk of ventricular arrhythmias and sudden death in patients with hypertrophic cardiomyopathy. Eur Heart J
30: 2003-2010
[Abstract][Full Text]
Basso, C., Thiene, G., Mackey-Bojack, S., Frigo, A. C., Corrado, D., Maron, B. J.
(2009). Myocardial bridging, a frequent component of the hypertrophic cardiomyopathy phenotype, lacks systematic association with sudden cardiac death. Eur Heart J
30: 1627-1634
[Abstract][Full Text]
Haghjoo, M., Mohammadzadeh, S., Taherpour, M., Faghfurian, B., Fazelifar, A. F., Alizadeh, A., Rad, M. A., Sadr-Ameli, M. A.
(2009). ST-segment depression as a risk factor in hypertrophic cardiomyopathy. Europace
11: 643-649
[Abstract][Full Text]
Spirito, P., Autore, C., Rapezzi, C., Bernabo, P., Badagliacca, R., Maron, M. S., Bongioanni, S., Coccolo, F., Estes, N.A. M., Barilla, C. S., Biagini, E., Quarta, G., Conte, M. R., Bruzzi, P., Maron, B. J.
(2009). Syncope and Risk of Sudden Death in Hypertrophic Cardiomyopathy. Circulation
119: 1703-1710
[Abstract][Full Text]
Shephard, R., Semsarian, C.
(2009). Advances in the prevention of sudden cardiac death in the young. Ther Adv Cardiovasc Dis
3: 145-155
[Abstract]
Proclemer, A., Ghidina, M., Gregori, D., Facchin, D., Rebellato, L., Fioretti, P., Brignole, M.
(2009). Impact of the main implantable cardioverter-defibrillator trials in clinical practice: data from the Italian ICD Registry for the years 2005-07. Europace
11: 465-475
[Abstract][Full Text]
Soor, G S, Luk, A, Ahn, E, Abraham, J R, Woo, A, Ralph-Edwards, A, Butany, J
(2009). Hypertrophic cardiomyopathy: current understanding and treatment objectives. J. Clin. Pathol.
62: 226-235
[Abstract][Full Text]
Attili, A., Mueller, G. C., Day, S. M.
(2009). AJR Teaching File: Asymptomatic Man with Giant Negative T Waves on ECG. Am. J. Roentgenol.
192: S57-S61
[Full Text]
Hess, O. M., McKenna, W., Schultheiss, H.-P.
(2009). CHAPTER 18 Myocardial Disease. ESC Textbook of Cardiovascular Medicine
2: med-9780199566990-chapter-med-9780199566990-chapter
[Abstract][Full Text]
Brignole, M., Blanc, J.-J., Sutton, R., Moya, A.
(2009). CHAPTER 26 Syncope. ESC Textbook of Cardiovascular Medicine
2: med-9780199566990-chapter-med-9780199566990-chapter
[Abstract][Full Text]
Eckardt, L., Breithardt, G.;n., Hohnloser, S.
(2009). CHAPTER 30 Ventricular Tachycardia and Sudden Cardiac Death. ESC Textbook of Cardiovascular Medicine
2: med-9780199566990-chapter-med-9780199566990-chapter
[Abstract][Full Text]
Cuoco, F. A., Spencer, W. H. III, Fernandes, V. L., Nielsen, C. D., Nagueh, S., Sturdivant, J. L., Leman, R. B., Wharton, J. M., Gold, M. R.
(2008). Implantable Cardioverter-Defibrillator Therapy for Primary Prevention of Sudden Death After Alcohol Septal Ablation of Hypertrophic Cardiomyopathy. J Am Coll Cardiol
52: 1718-1723
[Abstract][Full Text]
Elliott, P, Spirito, P
(2008). Prevention of hypertrophic cardiomyopathy-related deaths: theory and practice. Heart
94: 1269-1275
[Abstract][Full Text]
Coats, C J, Hollman, A
(2008). Hypertrophic cardiomyopathy: lessons from history. Heart
94: 1258-1263
[Abstract][Full Text]
Sen-Chowdhry, S., McKenna, W. J.
(2008). Non-invasive risk stratification in hypertrophic cardiomyopathy: don't throw out the baby with the bathwater. Eur Heart J
29: 1600-1602
[Full Text]
Epstein, A. E., DiMarco, J. P., Ellenbogen, K. A., Estes, N.A. M. III, Freedman, R. A., Gettes, L. S., Gillinov, A. M., Gregoratos, G., Hammill, S. C., Hayes, D. L., Hlatky, M. A., Newby, L. K., Page, R. L., Schoenfeld, M. H., Silka, M. J., Stevenson, L. W., Sweeney, M. O.
(2008). ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons. J Am Coll Cardiol
51: e1-e62
[Full Text]
Writing Committee Members, , Epstein, A. E., DiMarco, J. P., Ellenbogen, K. A., Estes, N.A. M. III, Freedman, R. A., Gettes, L. S., Gillinov, A. M., Gregoratos, G., Hammill, S. C., Hayes, D. L., Hlatky, M. A., Newby, L. K., Page, R. L., Schoenfeld, M. H., Silka, M. J., Stevenson, L. W., Sweeney, M. O.
(2008). ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons. Circulation
117: e350-e408
[Full Text]
Berul, C. I., Van Hare, G. F., Kertesz, N. J., Dubin, A. M., Cecchin, F., Collins, K. K., Cannon, B. C., Alexander, M. E., Triedman, J. K., Walsh, E. P., Friedman, R. A.
(2008). Results of a Multicenter Retrospective Implantable Cardioverter-Defibrillator Registry of Pediatric and Congenital Heart Disease Patients. J Am Coll Cardiol
51: 1685-1691
[Abstract][Full Text]
Adabag, A. S., Maron, B. J., Appelbaum, E., Harrigan, C. J., Buros, J. L., Gibson, C. M., Lesser, J. R., Hanna, C. A., Udelson, J. E., Manning, W. J., Maron, M. S.
(2008). Occurrence and Frequency of Arrhythmias in Hypertrophic Cardiomyopathy in Relation to Delayed Enhancement on Cardiovascular Magnetic Resonance. J Am Coll Cardiol
51: 1369-1374
[Abstract][Full Text]
Mittnacht, A. J., Fanshawe, M., Konstadt, S.
(2008). Anesthetic Considerations in the Patient With Valvular Heart Disease Undergoing Noncardiac Surgery. SEMIN CARDIOTHORAC VASC ANESTH
12: 33-59
[Abstract]
De Mozzi, P., Longo, U. G., Galanti, G., Maffulli, N.
(2008). Bicuspid aortic valve: a literature review and its impact on sport activity. Br Med Bull
85: 63-85
[Abstract][Full Text]
Khairy, P., Harris, L., Landzberg, M. J., Viswanathan, S., Barlow, A., Gatzoulis, M. A., Fernandes, S. M., Beauchesne, L., Therrien, J., Chetaille, P., Gordon, E., Vonder Muhll, I., Cecchin, F.
(2008). Implantable Cardioverter-Defibrillators in Tetralogy of Fallot. Circulation
117: 363-370
[Abstract][Full Text]
Rossenbacker, T., Priori, S. G., Zipes, D. P.
(2007). The fight against sudden cardiac death: consensus guidelines as a reference. Eur Heart J Suppl
9: I50-I58
[Abstract][Full Text]
Boriani, G., Ricci, R., Toselli, T., Ferrari, R., Branzi, A., Santini, M.
(2007). Implantable cardioverter defibrillators: from evidence of trials to clinical practice. Eur Heart J Suppl
9: I66-I73
[Abstract][Full Text]
Ripplinger, C. M., Li, W., Hadley, J., Chen, J., Rothenberg, F., Lombardi, R., Wickline, S. A., Marian, A. J., Efimov, I. R.
(2007). Enhanced Transmural Fiber Rotation and Connexin 43 Heterogeneity Are Associated With an Increased Upper Limit of Vulnerability in a Transgenic Rabbit Model of Human Hypertrophic Cardiomyopathy. Circ. Res.
101: 1049-1057
[Abstract][Full Text]
McLeod, C. J., Ommen, S. R., Ackerman, M. J., Weivoda, P. L., Shen, W. K., Dearani, J. A., Schaff, H. V., Tajik, A. J., Gersh, B. J.
(2007). Surgical septal myectomy decreases the risk for appropriate implantable cardioverter defibrillator discharge in obstructive hypertrophic cardiomyopathy. Eur Heart J
28: 2583-2588
[Abstract][Full Text]
Woo, A., Monakier, D., Harris, L., Hill, A., Shah, P., Wigle, E D., Rakowski, H., Rozenblyum, E., Cameron, D. A
(2007). Determinants of implantable defibrillator discharges in high-risk patients with hypertrophic cardiomyopathy. Heart
93: 1044-1045
[Abstract][Full Text]
Stephenson, E. A., Berul, C. I.
(2007). Electrophysiological Interventions for Inherited Arrhythmia Syndromes. Circulation
116: 1062-1080
[Full Text]
Yap, S.-C., Roos-Hesselink, J. W., Hoendermis, E. S., Budts, W., Vliegen, H. W., Mulder, B. J.M., van Dijk, A. P.J., Schalij, M. J., Drenthen, W.
(2007). Outcome of implantable cardioverter defibrillators in adults with congenital heart disease: a multi-centre study. Eur Heart J
28: 1854-1861
[Abstract][Full Text]
Passman, R., Kadish, A.
(2007). Sudden Death Prevention With Implantable Devices. Circulation
116: 561-571
[Full Text]
Maron, B. J., Spirito, P., Shen, W.-K., Haas, T. S., Formisano, F., Link, M. S., Epstein, A. E., Almquist, A. K., Daubert, J. P., Lawrenz, T., Boriani, G., Estes, N. A. M. III, Favale, S., Piccininno, M., Winters, S. L., Santini, M., Betocchi, S., Arribas, F., Sherrid, M. V., Buja, G., Semsarian, C., Bruzzi, P.
(2007). Implantable Cardioverter-Defibrillators and Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy. JAMA
298: 405-412
[Abstract][Full Text]
Nishimura, R. A., Ommen, S. R.
(2007). Hypertrophic Cardiomyopathy, Sudden Death, and Implantable Cardiac Defibrillators: How Low the Bar?. JAMA
298: 452-454
[Full Text]
Maron, B. J.
(2007). Surgical Myectomy Remains the Primary Treatment Option for Severely Symptomatic Patients With Obstructive Hypertrophic Cardiomyopathy. Circulation
116: 196-206
[Full Text]
Fifer, M. A.
(2007). Most Fully Informed Patients Choose Septal Ablation Over Septal Myectomy. Circulation
116: 207-216
[Full Text]
Melacini, P, Maron, B J, Bobbo, F, Basso, C, Tokajuk, B, Zucchetto, M, Thiene, G, Iliceto, S
(2007). Evidence that pharmacological strategies lack efficacy for the prevention of sudden death in hypertrophic cardiomyopathy. Heart
93: 708-710
[Abstract][Full Text]
Maron, B. J., Thompson, P. D., Ackerman, M. J., Balady, G., Berger, S., Cohen, D., Dimeff, R., Douglas, P. S., Glover, D. W., Hutter, A. M. Jr, Krauss, M. D., Maron, M. S., Mitten, M. J., Roberts, W. O., Puffer, J. C.
(2007). Recommendations and Considerations Related to Preparticipation Screening for Cardiovascular Abnormalities in Competitive Athletes: 2007 Update: A Scientific Statement From the American Heart Association Council on Nutrition, Physical Activity, and Metabolism: Endorsed by the American College of Cardiology Foundation. Circulation
115: 1643-1655
[Full Text]
Bayes-Genis, A.
(2007). Hypertrophy and inflammation: too much for one heart. Eur Heart J
0: ehm008v1-3
[Full Text]
Pablo Kaski, J., Teresa Tome Esteban, M., Lowe, M., Sporton, S., Rees, P., Deanfield, J. E, McKenna, W. J, Elliott, P. M
(2007). Outcomes after implantable cardioverter-defibrillator treatment in children with hypertrophic cardiomyopathy. Heart
93: 372-374
[Full Text]
Sarkozy, A., Boussy, T., Kourgiannides, G., Chierchia, G.-B., Richter, S., De Potter, T., Geelen, P., Wellens, F., Dingena Spreeuwenberg, M., Brugada, P.
(2007). Long-term follow-up of primary prophylactic implantable cardioverter-defibrillator therapy in Brugada syndrome. Eur Heart J
28: 334-344
[Abstract][Full Text]
Heldman, A. W., Wu, K. C., Abraham, T. P., Cameron, D. E.
(2007). Myectomy or Alcohol Septal Ablation: Surgery and Percutaneous Intervention Go Another Round. J Am Coll Cardiol
49: 358-360
[Full Text]
Maron, B. J., Pelliccia, A.
(2006). The Heart of Trained Athletes: Cardiac Remodeling and the Risks of Sports, Including Sudden Death. Circulation
114: 1633-1644
[Full Text]
Developed in Collaboration With the European Heart, , Zipes, D. P., Camm, A. J., Borggrefe, M., Buxton, A. E., Chaitman, B., Fromer, M., Gregoratos, G., Klein, G., Moss, A. J., Myerburg, R. J., Priori, S. G., Quinones, M. A., Roden, D. M., Silka, M. J., Tracy, C., Smith, S. C. Jr, Jacobs, A. K., Adams, C. D., Antman, E. M., Anderson, J. L., Hunt, S. A., Halperin, J. L., Nishimura, R., Ornato, J. P., Page, R. L., Riegel, B., Priori, S. G., Blanc, J.-J., Budaj, A., Camm, A. J., Dean, V., Deckers, J. W., Despres, C., Dickstein, K., Lekakis, J., McGregor, K., Metra, M., Morais, J., Osterspey, A., Tamargo, J. L., Zamorano, J. L.
(2006). ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death--Executive Summary: 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
48: 1064-1108
[Full Text]
Developed in Collaboration With the European Heart, , Zipes, D. P., Camm, A. J., Borggrefe, M., Buxton, A. E., Chaitman, B., Fromer, M., Gregoratos, G., Klein, G., Moss, A. J., Myerburg, R. J., Priori, S. G., Quinones, M. A., Roden, D. M., Silka, M. J., Tracy, C., Smith, S. C. Jr, Jacobs, A. K., Adams, C. D., Antman, E. M., Anderson, J. L., Hunt, S. A., Halperin, J. L., Nishimura, R., Ornato, J. P., Page, R. L., Riegel, B., Priori, S. G., Blanc, J.-J., Budaj, A., Camm, A. J., Dean, V., Deckers, J. W., Despres, C., Dickstein, K., Lekakis, J., McGregor, K., Metra, M., Morais, J., Osterspey, A., Tamargo, J. L., Zamorano, J. L.
(2006). 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
48: e247-e346
[Full Text]
Zipes, D. P., Zipes, D. P., Camm, A. J., Borggrefe, M., Buxton, A. E., Chaitman, B., Fromer, M., Gregoratos, G., Klein, G., Moss, A. J., Myerburg, R. J., Priori, S. G., Quinones, M. A., Roden, D. M., Silka, M. J., Tracy, C., ESC Committee for Practice Guidelines, , Priori, S. G., Blanc, J.-J., Budaj, A., Camm, A. J., Dean, V., Deckers, J. W., Despres, C., Dickstein, K., Lekakis, J., McGregor, K., Metra, M., Morais, J., Osterspey, A., Tamargo, J. L., Zamorano, J. L., ACC/AHA (Practice Guidelines) Task Force Members, , Smith, S. C. Jr, Jacobs, A. K., Adams, C. D., Antman, E. M., Anderson, J. L., Hunt, S. A., Halperin, J. L., Nishimura, R., Ornato, J. P., Page, R. L., Riegel, B.
(2006). ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death--executive summary: 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.. Eur Heart J
27: 2099-2140
[Full Text]
Writing Committee Members, , Zipes, D. P., Camm, A. J., Borggrefe, M., Buxton, A. E., Chaitman, B., Fromer, M., Gregoratos, G., Klein, G., Moss, A. J., Myerburg, R. J., Priori, S. G., Quinones, M. A., Roden, D. M., Silka, M. J., Tracy, C., ESC Committee for Practice Guidelines, , Priori, S. G., Blanc, J.-J., Budaj, A., Camm, A. J., Dean, V., Deckers, J. W., Despres, C., Dickstein, K., Lekakis, J., McGregor, K., Metra, M., Morais, J., Osterspey, A., Tamargo, J. L., Zamorano, J. L., ACC/AHA Task Force Members, , Smith, S. C. Jr, Jacobs, A. K., Adams, C. D., Antman, E. M., Anderson, J. L., Hunt, S. A., Halperin, J. L., Nishimura, R., Ornato, J. P., Page, R. L., Riegel, B.
(2006). 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
8: 746-837
[Full Text]
Maron, B. J., Olivotto, I., Maron, M. S.
(2006). 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
27: 1895-1897
[Full Text]
Harris, K. M., Spirito, P., Maron, M. S., Zenovich, A. G., Formisano, F., Lesser, J. R., Mackey-Bojack, S., Manning, W. J., Udelson, J. E., Maron, B. J.
(2006). Prevalence, Clinical Profile, and Significance of Left Ventricular Remodeling in the End-Stage Phase of Hypertrophic Cardiomyopathy. Circulation
114: 216-225
[Abstract][Full Text]
Ho, C. Y., Seidman, C. E.
(2006). A Contemporary Approach to Hypertrophic Cardiomyopathy. Circulation
113: e858-e862
[Full Text]
Pedrote, A., Morales, F. J., Garcia-Riesco, L., Errazquin, F.
(2006). Documented exercise-induced cardiac arrest in a paediatric patient with hypertrophic cardiomyopathy. Europace
8: 430-433
[Abstract][Full Text]
Spirito, P., Autore, C.
(2006). Management of hypertrophic cardiomyopathy.. BMJ
332: 1251-1255
[Full Text]
Cesario, D. A., Dec, G. W.
(2006). Implantable Cardioverter- Defibrillator Therapy in Clinical Practice. J Am Coll Cardiol
47: 1507-1517
[Abstract][Full Text]
Strickberger, S. A., Benson, D. W., Biaggioni, I., Callans, D. J., Cohen, M. I., Ellenbogen, K. A., Epstein, A. E., Friedman, P., Goldberger, J., Heidenreich, P. A., Klein, G. J., Knight, B. P., Morillo, C. A., Myerburg, R. J., Sila, C. A.
(2006). 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
47: 473-484
[Full Text]
Strickberger, S. A., Benson, D. W., Biaggioni, I., Callans, D. J., Cohen, M. I., Ellenbogen, K. A., Epstein, A. E., Friedman, P., Goldberger, J., Heidenreich, P. A., Klein, G. J., Knight, B. P., Morillo, C. A., Myerburg, R. J., Sila, C. A.
(2006). 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
113: 316-327
[Full Text]
Paterick, T. E., Paterick, T. J., Fletcher, G. F., Maron, B. J.
(2005). Medical and Legal Issues in the Cardiovascular Evaluation of Competitive Athletes. JAMA
294: 3011-3018
[Abstract][Full Text]
Wolf, C. M., Moskowitz, I. P. G., Arno, S., Branco, D. M., Semsarian, C., Bernstein, S. A., Peterson, M., Maida, M., Morley, G. E., Fishman, G., Berul, C. I., Seidman, C. E., Seidman, J. G.
(2005). Somatic events modify hypertrophic cardiomyopathy pathology and link hypertrophy to arrhythmia. Proc. Natl. Acad. Sci. USA
102: 18123-18128
[Abstract][Full Text]
Maron, B J
(2005). Distinguishing hypertrophic cardiomyopathy from athlete's heart: a clinical problem of increasing magnitude and significance. Heart
91: 1380-1382
[Abstract][Full Text]
Schumacher, B., Gietzen, F. H., Neuser, H., Schummelfeder, J., Schneider, M., Kerber, S., Schimpf, R., Wolpert, C., Borggrefe, M.
(2005). Electrophysiological Characteristics of Septal Hypertrophy in Patients With Hypertrophic Obstructive Cardiomyopathy and Moderate to Severe Symptoms. Circulation
112: 2096-2101
[Abstract][Full Text]
Hauser, R. G., Maron, B. J.
(2005). Lessons From the Failure and Recall of an Implantable Cardioverter-Defibrillator. Circulation
112: 2040-2042
[Full Text]
Binder, W. D., Fifer, M. A., King, M. E., Stone, J. R.
(2005). Case 26-2005 - A 48-Year-Old Man with Sudden Loss of Consciousness while Jogging. NEJM
353: 824-832
[Full Text]
Rickers, C., Wilke, N. M., Jerosch-Herold, M., Casey, S. A., Panse, P., Panse, N., Weil, J., Zenovich, A. G., Maron, B. J.
(2005). Utility of Cardiac Magnetic Resonance Imaging in the Diagnosis of Hypertrophic Cardiomyopathy. Circulation
112: 855-861
[Abstract][Full Text]
Olivotto, I., Maron, M. S., Adabag, A. S., Casey, S. A., Vargiu, D., Link, M. S., Udelson, J. E., Cecchi, F., Maron, B. J.
(2005). Gender-Related Differences in the Clinical Presentation and Outcome of Hypertrophic Cardiomyopathy. J Am Coll Cardiol
46: 480-487
[Abstract][Full Text]
Steinbrook, R.
(2005). The Controversy over Guidant's Implantable Defibrillators. NEJM
353: 221-224
[Full Text]
Kadish, A., Mehra, M.
(2005). Heart Failure Devices: Implantable Cardioverter-Defibrillators and Biventricular Pacing Therapy. Circulation
111: 3327-3335
[Full Text]
Woo, A., Williams, W. G., Choi, R., Wigle, E. D., Rozenblyum, E., Fedwick, K., Siu, S., Ralph-Edwards, A., Rakowski, H.
(2005). Clinical and Echocardiographic Determinants of Long-Term Survival After Surgical Myectomy in Obstructive Hypertrophic Cardiomyopathy. Circulation
111: 2033-2041
[Abstract][Full Text]
Maron, B. J., Zipes, D. P.
(2005). Introduction: Eligibility recommendations for competitive athletes with cardiovascular abnormalities--general considerations. J Am Coll Cardiol
45: 1318-1321
[Full Text]
Maron, B. J., Douglas, P. S., Graham, T. P., Nishimura, R. A., Thompson, P. D.
(2005). Task Force 1: Preparticipation screening and diagnosis of cardiovascular disease in athletes. J Am Coll Cardiol
45: 1322-1326
[Full Text]
Maron, B. J., Ackerman, M. J., Nishimura, R. A., Pyeritz, R. E., Towbin, J. A., Udelson, J. E.
(2005). Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. J Am Coll Cardiol
45: 1340-1345
[Full Text]
Adabag, A. S., Casey, S. A., Kuskowski, M. A., Zenovich, A. G., Maron, B. J.
(2005). Spectrum and prognostic significance of arrhythmias on ambulatory Holter electrocardiogram in hypertrophic cardiomyopathy. J Am Coll Cardiol
45: 697-704
[Abstract][Full Text]
Maron, B. J.
(2005). How should we screen competitive athletes for cardiovascular disease?. Eur Heart J
26: 428-430
[Full Text]
Kaufmann, P. A., Camici, P. G.
(2005). Myocardial Blood Flow Measurement by PET: Technical Aspects and Clinical Applications. JNM
46: 75-88
[Full Text]
Maron, B. J., Seidman, J.G., Seidman, C. E.
(2004). Proposal for contemporary screening strategies in families with hypertrophic cardiomyopathy. J Am Coll Cardiol
44: 2125-2132
[Abstract][Full Text]
Maron, B. J., Dearani, J. A., Ommen, S. R., Maron, M. S., Schaff, H. V., Gersh, B. J., Nishimura, R. A.
(2004). The case for surgery in obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol
44: 2044-2053
[Abstract][Full Text]
Hess, O. M., Sigwart, U.
(2004). New treatment strategies for hypertrophic obstructive cardiomyopathy: Alcohol ablation of the septum: the new gold standard?. J Am Coll Cardiol
44: 2054-2055
[Abstract][Full Text]
Boriani, G., Maron, B. J., Shen, W.-K., Spirito, P.
(2004). Prevention of Sudden Death in Hypertrophic Cardiomyopathy: But Which Defibrillator for Which Patient?. Circulation
110: e438-e442
[Full Text]
Cohen, M. I., Rhodes, L. A., Spray, T. L., Gaynor, J. W.
(2004). Efficacy of prophylactic epicardial pacing leads in children and young adults. Ann. Thorac. Surg.
78: 197-202
[Abstract][Full Text]
Katritsis, D. G, Camm, A.J.
(2004). Nonsustained ventricular tachycardia: where do we stand?. Eur Heart J
25: 1093-1099
[Abstract][Full Text]
Maron, B. J., Chaitman, B. R., Ackerman, M. J., Bayes de Luna, A., Corrado, D., Crosson, J. E., Deal, B. J., Driscoll, D. J., Estes, N.A. M. III, Araujo, C. G. S., Liang, D. H., Mitten, M. J., Myerburg, R. J., Pelliccia, A., Thompson, P. D., Towbin, J. A., Van Camp, S. P., for the Working Groups of the American Heart Assoc,
(2004). Recommendations for Physical Activity and Recreational Sports Participation for Young Patients With Genetic Cardiovascular Diseases. Circulation
109: 2807-2816
[Abstract][Full Text]
Roguin, A., Bomma, C. S., Nasir, K., Tandri, H., Tichnell, C., James, C., Rutberg, J., Crosson, J., Spevak, P. J., Berger, R. D., Halperin, H. R., Calkins, H.
(2004). Implantable Cardioverter-Defibrillators in patients with arrhythmogenic right ventricular Dysplasia/Cardiomyopathy. J Am Coll Cardiol
43: 1843-1852
[Abstract][Full Text]
Frenneaux, M. P
(2004). Assessing the risk of sudden cardiac death in a patient with hypertrophic cardiomyopathy. Heart
90: 570-575
[Full Text]
Nishimura, R. A., Holmes, D. R. Jr.
(2004). Hypertrophic Obstructive Cardiomyopathy. NEJM
350: 1320-1327
[Full Text]