Background Sudden death is a possible consequence of hypertrophiccardiomyopathy. Quantification of the risk of sudden death,however, remains imprecise for most patients with this disease.
Methods We assessed the relation between the magnitude of leftventricular hypertrophy and mortality in 480 consecutive patientswith hypertrophic cardiomyopathy. The patients were categorizedinto five subgroups according to maximal wall thickness: 15mm or less, 16 to 19 mm, 20 to 24 mm, 25 to 29 mm, and 30 mmor more. Their ages ranged from 1 to 89 years (median, 47).
Results Over a mean follow-up period of 6.5 years, 65 of the480 patients (14 percent) died: 23 suddenly, 15 of heart failure,and 27 of noncardiac causes or stroke. The risk of sudden deathincreased progressively and in direct relation to wall thickness(P=0.001), ranging from 0 per 1000 person-years (95 percentconfidence interval, 0 to 14.4) for a wall thickness of 15 mmor less to 18.2 per 1000 person-years (95 percent confidenceinterval, 7.3 to 37.6) for a wall thickness of 30 mm or moreand almost doubling from each wall-thickness subgroup to thenext. The cumulative risk 20 years after the initial evaluationwas close to zero for patients with a wall thickness of 19 mmor less but almost 40 percent for wall thicknesses of 30 mmor more. As compared with the other subgroups, patients withextreme hypertrophy were the youngest (mean age, 31 years),and most (41 of 43) had mild symptoms or no symptoms; of the12 patients who were less than 18 years old at the initial evaluation,5 died suddenly.
Conclusions In hypertrophic cardiomyopathy, the magnitude ofhypertrophy is directly related to the risk of sudden deathand is a strong and independent predictor of prognosis. Youngpatients with extreme hypertrophy, even those with few or nosymptoms, appear to be at substantial long-term risk and deserveconsideration for interventions to prevent sudden death. Themajority of patients with mild hypertrophy are at low risk andcan be reassured regarding their prognosis.
Sudden death has been recognized as a possible consequence ofhypertrophic cardiomyopathy since the first modern descriptionsof the disease.1,2 However, despite 40 years of investigation,the search for a way to identify patients who have a high riskof dying suddenly has been frustrated by certain features ofthis disorder, including the frequent absence of symptoms beforesudden death,2,3,4,5 the great variation in clinical presentationand prognosis,3,4,5 the relatively low prevalence of the disease,6,7and the difficulty of enrolling large study populations withoutbias due to referral of patients at tertiary centers.8,9,10
Several factors are known to be strong indicators of a highrisk of sudden death in patients with hypertrophic cardiomyopathy,including a previous aborted cardiac arrest, one or more episodesof sustained ventricular tachycardia, and a history of suddendeath in two or more young family members.3,4,5 However, thesefeatures are uncommon in the overall population of patientswith hypertrophic cardiomyopathy. Other indicators of increasedrisk, such as the presence of nonsustained ventricular tachycardiaon ambulatory electrocardiographic monitoring or an abnormalblood-pressure response during exercise, have a low positivepredictive value.4,5,11,12,13,14 The limitations of risk stratificationhave recently acquired particular relevance because of the increasingavailability of implantable cardioverter–defibrillatorsand their proven efficacy in preventing sudden death in patientswith hypertrophic cardiomyopathy.15
Although there is circumstantial evidence that young patientswith extreme left ventricular hypertrophy may be at increasedrisk for sudden death,16,17,18 the importance of the magnitudeof hypertrophy as a risk factor remains unresolved. The purposeof our study was to assess the relation between the magnitudeof left ventricular hypertrophy and survival in a large andrelatively unselected series of consecutive patients with hypertrophiccardiomyopathy.
Methods
Study Population
Each of the 490 consecutively enrolled patients with hypertrophiccardiomyopathy who were evaluated at the Ente Ospedaliero OspedaliGalliera in Genoa, Italy, from January 1983 to December 1997(213 patients) or at the Minneapolis Heart Institute Foundationin Minneapolis from January 1981 to December 1996 (277 patients)were initially considered for inclusion in the study.
The initial evaluation (base line) at the center in Genoa wasdefined as taking place at the time of the first visit and atthe center in Minneapolis as taking place at the initial diagnosisof hypertrophic cardiomyopathy (for a minority of patients asearly as 1966). Eight patients had only a single evaluationand were excluded from subsequent analysis. Two patients hada history of aborted cardiac arrest before their initial evaluationand were also excluded from the analysis. Therefore, the finalstudy population comprised a total of 480 patients (210 in Genoaand 270 in Minneapolis). Six of these 480 patients had an abortedcardiac arrest or had ventricular tachycardia or fibrillationinterrupted by the discharge of an implantable cardioverter–defibrillatorafter enrollment; follow-up of these patients was terminatedat the time of their most recent evaluation. Six other patientsunderwent heart transplantation, and follow-up was terminatedat that time.
Echocardiography
The magnitude of left ventricular hypertrophy was assessed withtwo-dimensional echocardiography according to previously publishedcriteria.17,18 The greatest thickness measured at any site inthe left ventricular wall was considered to represent the maximalwall thickness.17,18,19 In the analysis of data, the maximalwall thickness was not corrected for body size, either in adultsor in children. The study population was divided into five subgroupsaccording to maximal wall thickness: 15 mm or less, 16 to 19mm, 20 to 24 mm, 25 to 29 mm, and 30 mm or more. These arbitrarycutoff points are consistent with criteria used in previousstudies.4,5,16,17,18,19
The echocardiographic measurements in each of the 210 patientsevaluated at the center in Genoa were made by a single investigator,and in each of the 270 patients evaluated in Minneapolis thesemeasurements were made by a single, different investigator.The correlation between these two investigators' measurementsof left ventricular wall thickness in patients with hypertrophiccardiomyopathy has been reported.20,21
The same investigators repeated their measurements of maximalwall thickness independently and without knowledge of the identityof the patients or of the previous findings in 48 echocardiogramsselected at random from the echocardiograms of the 480 patients(24 were from the Italian cohort and 24 from the U.S. cohort,and 18 others were from the 43 patients with a wall thicknessof 30 mm or more). The correlation between the two sets of measurementswas high (Pearson correlation coefficient, 0.94), and therewas good concordance between the two sets with respect to classificationinto the five wall-thickness subgroups (weighted kappa for orderedcategories, 0.91).
Left ventricular outflow obstruction under basal conditionswas considered present when a peak outflow gradient of 30 mmHg or more was identified by Doppler echocardiography.22 Theleft atrial and left ventricular end-diastolic cavity dimensionswere assessed by M-mode echocardiography in a standard fashion.
Definitions
Hypertrophic cardiomyopathy was diagnosed when there was echocardiographicevidence of a nondilated and hypertrophied left ventricle (definedas a wall thickness of 15 mm in adult index patients or theequivalent wall thickness relative to body-surface area in children)in the absence of another cardiac or systemic disease that couldproduce a similar degree of hypertrophy.10,13,19 In adult relativesof the patients with hypertrophic cardiomyopathy, a wall thicknessof 13 mm or more was considered a criterion for diagnosis.23
Sudden death of cardiac origin was defined as instantaneousand unexpected death within minutes after a witnessed collapsein patients previously in stable clinical condition. Death wasalso classified as sudden if it occurred unexpectedly but wasunwitnessed, such as death occurring in bed overnight.13
Death due to congestive heart failure was defined as death occurringin the context of long-standing cardiac decompensation withprogression of disease during the previous year, with the developmentof pulmonary edema or evolution to end-stage disease.
A history of sudden death in the family was considered presentif one or more family members with hypertrophic cardiomyopathyhad died suddenly, according to the definition of sudden deathgiven above, or if one or more close relatives without a documenteddiagnosis of hypertrophic cardiomyopathy had died suddenly atless than 50 years of age.
Statistical Analysis
To estimate mortality rates, the number of patients who diedduring follow-up was divided by the total number of person-yearsaccumulated during follow-up in the study population or in eachwall-thickness subgroup. For the calculation of overall ratesof death, follow-up time was considered to be the interval fromthe date of the initial evaluation to the time of death or (amongsurviving patients) to the time of the most recent evaluation.For the calculation of rates of sudden death, follow-up datafor patients who died from congestive heart failure or fromnoncardiac causes were censored at the time of death. For thecalculation of rates of death due to heart failure or noncardiaccauses, follow-up data for patients who died suddenly were censoredat the time of death.
Ninety-five percent confidence intervals for mortality rateswere calculated with the assumption of an underlying Poissondistribution of rare events. Rates were compared among subgroupsof patients by means of the chi-square test for heterogeneity,Fisher's test, or the chi-square test for trend, as appropriate.Survival curves were constructed according to the Kaplan–Meiermethod.
To assess the role of wall thickness as an independent predictorof death, three multivariate Cox proportional-hazards modelswere fitted to the data. In each model, patients were stratifiedaccording to age (into one of four categories), and variablessignificantly associated with outcome were entered in a stepwiseprocedure based on the likelihood-ratio test. The results ofthe multivariate analyses should be interpreted cautiously inview of the relatively small number of events and the largenumber of strata. All P values are two-sided. SPSS statisticalsoftware (SPSS, Chicago) was used for most calculations.
Results
Base-Line Clinical Characteristics
The clinical features of the overall study population and ofthe five wall-thickness subgroups at base line (the initialevaluation) are summarized in Table 1. The duration of follow-upranged from 1 month to 31 years (mean, 6.5 years; median, 4.6).The 480 study patients ranged in age from 1 to 89 years (mean,47; median, 47); 288 of the patients (60 percent) were male.Of the 480 patients, 446 (93 percent) were asymptomatic or hadonly mild symptoms (New York Heart Association functional classI or II), and 34 (7 percent) had severe symptoms (class IIIor IV); 133 patients (28 percent) had left ventricular outflowobstruction (an outflow gradient of 30 mm Hg under basal conditions).
Table 1. Base-Line Characteristics of the Study Patients According to the Magnitude of Left Ventricular Hypertrophy.
At the time of the first visit, the maximal left-ventricular-wallthickness ranged from 7 to 40 mm (mean, 21±5); 448 patientshad a left-ventricular-wall thickness of 15 mm or more, and32 had a wall thickness of less than 15 mm. Of these 32 patients,10 were children 15 years of age or younger; 3 were patientswith progression to end-stage disease with wall thinning, cavitydilatation, and systolic dysfunction21; and 19 were adult membersof families affected by hypertrophic cardiomyopathy.
Among the five wall-thickness subgroups, patients with wallthicknesses at the two extremes of the morphologic spectrum(maximal wall thickness, 15 or 30 mm) were the youngest (P<0.001)and had the lowest frequency of outflow obstruction (P=0.001)(Table 1). Sex distribution and New York Heart Association functionalclass were similar among the five subgroups (P=0.77 and P=0.79,respectively), and the proportion of patients with moderateor severe symptoms in each subgroup was low (4 to 9 percent).
During follow-up, 65 of the 480 study patients (14 percent)died: 23 suddenly, 15 of heart failure, and 27 of noncardiaccauses or stroke. The overall incidence of death from any causewas 20.9 per 1000 person-years (95 percent confidence interval,16.1 to 26.6), that of sudden death was 7.4 per 1000 person-years(95 percent confidence interval, 4.7 to 11.1), that of deathdue to heart failure was 4.8 per 1000 person-years (95 percentconfidence interval, 2.7 to 8.0), and that of death from noncardiaccauses or stroke was 8.7 per 1000 person-years (95 percent confidenceinterval, 5.7 to 12.6). The mean age at the time of death was44±24 years among those who died suddenly, 63±21years among those who died of heart failure, and 74±12years among those who died of noncardiac causes or stroke. Ofthe 23 patients who died suddenly, 21 had no symptoms or mildsymptoms (New York Heart Association class I or II) and 2 hadsevere symptoms (class III) at the time of death.
At the time of the initial evaluation or shortly thereafter,151 (31 percent) of the study patients were not taking any cardioactivemedications, 300 (62 percent) were taking beta-blockers or calciumantagonists, and 29 (6 percent) were taking amiodarone eitherbecause of ambulatory electrocardiographic evidence of nonsustainedventricular tachycardia or because of paroxysmal atrial fibrillation.Of the 29 patients taking amiodarone, 5 (17 percent) died suddenlyduring follow-up; each was taking amiodarone at the time ofdeath.
Of the 480 study patients, 26 underwent a septal myotomy–myectomyoperation, and 8 were given a pacemaker in an attempt to reducethe left ventricular outflow gradient and to improve symptoms.24Thirteen patients had a cardioverter–defibrillator; threeof them had an appropriate discharge for ventricular tachycardiaor fibrillation during follow-up.
Relation between Maximal Left-Ventricular-Wall Thickness and Mortality
Mortality in the five wall-thickness subgroups is reported inTable 2. The risk of sudden death increased significantly andprogressively in direct relation to wall thickness (P=0.001)(Figure 1). The risk of sudden death was 0 (95 percent confidenceinterval, 0 to 14.4) per 1000 person-years in patients witha wall thickness of 15 mm or less, 2.6 per 1000 person-years(95 percent confidence interval, 0.3 to 9.6) in those with awall thickness of 16 to 19 mm, 7.4 per 1000 person-years (95percent confidence interval, 3.5 to 13.6) in those with a wallthickness of 20 to 24 mm, 11.0 per 1000 person-years (95 percentconfidence interval, 3.0 to 28.2) in those with a wall thicknessof 25 to 29 mm, and 18.2 per 1000 person-years (95 percent confidenceinterval, 7.3 to 37.6) in those with a wall thickness of 30mm or more.
Figure 1. Relation between Maximal Left-Ventricular-Wall Thickness and the Risk of Sudden Death in 480 Patients with Hypertrophic Cardiomyopathy.
The incidence of sudden death increased progressively and in direct relation to maximal wall thickness (P=0.001 by the chi-square test for trend).
Kaplan–Meier estimates of the proportion of patients withoutsudden death in each of the five wall-thickness subgroups areshown in Figure 2. Twenty years after the initial evaluation,patients with a wall thickness of 19 mm or less had a cumulativerisk close to zero, whereas those with a wall thickness of 30mm or more had a risk of close to 40 percent.
Figure 2. Kaplan–Meier Estimates of the Proportions of Patients without Sudden Death among the 480 Patients with Hypertrophic Cardiomyopathy, According to the Magnitude of Left Ventricular Hypertrophy.
The highest rate of sudden death was observed in the youngestpatients with a wall thickness of 30 mm or more; of the 12 patientswho were younger than 18 years of age at base line 5 died suddenly(incidence of sudden death, 37.9 per 1000 person-years; 95 percentconfidence interval, 12.8 to 88.5). Of the five patients youngerthan 13 years of age at base line, three died suddenly.
Univariate Analysis
The results of univariate analyses of the relation between clinicalvariables (other than wall thickness) and mortality are reportedin Table 3. A significant univariate association with deathdue to heart failure was identified for several variables: ageat base line (P=0.003), New York Heart Association functionalclass (P=0.001), the presence or absence of outflow obstruction(P=0.006), and left atrial cavity dimension (P=0.03). A univariateassociation with sudden death was identified for left ventricularend-diastolic cavity dimension (P=0.002). No relation was identifiedbetween the incidence of sudden death or of death due to heartfailure and a history of sudden death in the family (P=0.23and P=0.48), respectively).
Table 3. Results of Univariate Analysis of the Relation between Base-Line Clinical Variables Other Than Left-Ventricular-Wall Thickness and the Risk of Death.
Multivariate Regression Analysis
In a Cox regression model that included age as a stratificationfactor (Table 4), left-ventricular-wall thickness was foundto be independently and directly related to the incidence ofsudden death (P= 0.003), and left ventricular end-diastoliccavity dimension was, as a consequence, inversely related tothis end point (P=0.04). Wall thickness was also independentlyrelated to the incidence of death due to heart failure (P=0.04).The New York Heart Association functional class and the presenceor absence of outflow obstruction at base line showed no relationto the incidence of sudden death (P=0.47 and P=0.76, respectively),but they were independently related to the incidence of deathdue to heart failure (P=0.001 and P=0.005, respectively).
Table 4. Results of Multivariate Cox Proportional-Hazards Analysis of the Relation between Base-Line Clinical Variables and the Risk of Death, Adjusted for Age.
To assess whether the associations between wall thickness andmortality were similar in the populations of patients at theItalian and U.S. institutions, a term to represent interactionbetween the institution and the wall-thickness subgroup wasintroduced into the multivariate models. The interaction termwas not significantly different from zero with regard to suddendeath (P=0.54), death due to heart failure (P=0.14), or deathfrom any cause (P=0.48), indicating no difference between thetwo populations in the relation between wall thickness and mortality.
Discussion
Our results show that the magnitude of left ventricular hypertrophy,as measured by echocardiography and expressed in terms of maximalwall thickness, is a strong and independent predictor of therisk of sudden death in patients with hypertrophic cardiomyopathy.Though relatively low overall, the rate of sudden death in ourstudy population increased progressively and significantly indirect relation to left-ventricular-wall thickness, almost doublingfrom each wall-thickness subgroup to the next.
About 10 percent of our patients were at the high end of themorphologic spectrum of disease (maximal wall thickness, 30mm). In these patients, the cumulative risk of sudden deathwas almost 20 percent 10 years after the initial evaluationand was almost 40 percent at 20 years. Most of these patientswere young, had no outflow obstruction under basal conditions,and had few or no symptoms. The possibility that life expectancyin this subgroup is substantially reduced is supported by previousfindings that patients with extreme hypertrophy who are olderthan 50 are rarely encountered.16,17,18
The rate of sudden death that we identified in patients withextreme left ventricular hypertrophy should be considered inthe context of their youth, particularly in an era in whichthe implantable cardioverter–defibrillator is availableand can provide effective protection from lethal ventriculartachyarrhythmias in patients with hypertrophic cardiomyopathy.15
Admittedly, we cannot predict the future clinical course ofyoung patients with extreme phenotypic expressions of this conditionafter sudden death has been prevented by a cardioverter–defibrillator.Other unfavorable patterns of disease could emerge later inlife, such as progression to heart failure or development ofatrial fibrillation.3,4,5,10 Nevertheless, since sudden deathoften occurs in the absence of clinically important symptomsand systolic dysfunction in hypertrophic cardiomyopathy, itis reasonable to expect that prevention of sudden death in patientswith this disease will prolong life substantially. Therefore,we believe that young patients with extreme hypertrophy (includingthose with no other risk factors) should be informed about theoption of the implantable cardioverter–defibrillator.
Treatment with amiodarone has been considered an effective methodfor the prevention of sudden death in patients with hypertrophiccardiomyopathy.3,4,5,25 This conclusion, however, is based principallyon a single retrospective study that included historical controlsubjects who were treated with potentially proarrhythmic medications.25The finding that almost 20 percent of our patients who diedsuddenly were taking amiodarone at the time of death casts doubton the efficacy of this drug in preventing sudden death in patientswith hypertrophic cardiomyopathy. Furthermore, the toxic effectsoften associated with long-term amiodarone treatment limit itsuse in young patients.
Our findings also have implications for the identification ofpatients at low risk for sudden death. In patients with mildhypertrophy (maximal wall thickness, 19 mm), the rate of suddendeath was close to zero 10 years after the initial evaluationand was less than 3 percent at 20 years. These findings areconsistent with previous hypotheses suggesting that the majorityof patients with mild hypertrophy and without strong predictorsof risk should be reassured that they have a favorable prognosis.4,5,8,10These recommendations have far-reaching implications, sincepatients with mild hypertrophy represent a large proportionof those with this disease8,9,10 and, indeed, constituted morethan 40 percent of our study population.
Because virtually all the data in the literature on hypertrophiccardiomyopathy rely on measurements of left-ventricular-wallthickness uncorrected for body-surface area, we were concernedthat results based on values that took body size into accountwould be inconsistent with all previous findings and would thereforebe meaningless in the clinical arena. Consequently, we usedabsolute wall thickness, a reliable and easily obtained measurementwith which there has been extensive experience in studies ofhypertrophic cardiomyopathy. Inevitably, this method raisesthe problem of how to relate our findings with precision tothe risk of death among children of various body sizes. Unfortunately,the number of children in our study population was insufficientto address this issue in detail. Nevertheless, our observationsclearly indicate that children with extreme hypertrophy in absoluteterms (which would be even greater if wall thickness were correctedfor body size) are at particularly high risk and require treatmentto prevent sudden death. Children whose hypertrophy can be classifiedat the high end of the morphologic spectrum with respect tobody size are likely to be at increased risk, but we currentlyrecommend that treatment be based on an evaluation of theiroverall risk profile.
The clinical implications of a marked outflow gradient in patientswith hypertrophic cardiomyopathy have been a subject of debatesince the early 1960s.26 However, for more than 40 years, thelack of systematic data from large populations of consecutivelyenrolled patients has precluded definitive conclusions on thisissue. A recent study of a regional cohort of patients withhypertrophic cardiomyopathy identified a relation between thepresence or absence of basal outflow obstruction and the riskof death from cardiovascular causes.10 Because of the smallnumber of events in that investigation, however, sudden deathand death related to heart failure could not be examined asseparate end points. In the current study, we found a relationbetween the presence of outflow obstruction and the risk ofdeath due to heart failure, but not the risk of sudden death.This finding substantiates the view that interventions designedto lower the gradient, such as septal myotomy–myectomy,pacing, or alcohol septal ablation,27 cannot be expected todecrease the risk of sudden death. Also, the relation we identifiedbetween the presence of an outflow gradient and the risk ofdeath related to heart failure should not be interpreted asa reason to abolish or reduce the gradient by invasive meansin the absence of severe symptoms, since there is no evidencethat such interventions improve survival.
A history of sudden death at a young age in affected familymembers has generally been regarded as an indicator of increasedrisk in patients with hypertrophic cardiomyopathy.3,4,5 In thecurrent study, this variable was not related to the risk ofsudden death. However, only a minority of our patients had ahistory of sudden death in two or more family members less than50 years old. Therefore, our findings should not obscure previousdata suggesting that affected persons from such families areat increased risk.4,5,28,29
Despite our previous findings that two-dimensional echocardiographicmeasurements of left-ventricular-wall thickness in patientswith hypertrophic cardiomyopathy are highly reliable,16,17,18,19,20,21as also supported by the analysis of reproducibility in thecurrent study, we wish to sound a note of caution regardingthe capability of echocardiography to measure wall thicknesswithin 1 to 2 mm.30 This limitation may be more relevant toleft ventricles with particularly marked hypertrophy. Therefore,we believe that for borderline measurements of extreme hypertrophy,clinical decisions regarding treatment to prevent sudden deathshould also be based on the evaluation of other potential riskfactors, as well as on the physician's overall clinical judgment.For measurements of wall thickness that are clearly at the highend of the morphologic spectrum of hypertrophic cardiomyopathy,this phenotype alone identifies high risk and justifies interventionfor the primary prevention of sudden death.15
Our results show that the magnitude of left ventricular hypertrophyis a strong and independent predictor of prognosis in patientswith hypertrophic cardiomyopathy, a finding that has importantimplications for patient care. In the absence of other generallyaccepted risk factors, patients at the low end of the morphologicspectrum of this disease (maximal left-ventricular-wall thickness,19 mm) are at low risk for sudden death. Conversely, young patientswith extreme hypertrophy (maximal wall thickness, 30 mm), althoughthey usually have few or no symptoms, appear to be at substantiallong-term risk. Such patients, in consideration of their youthand the potential for a near-normal life expectancy if suddendeath is prevented, should be informed about the lifesavingprotection afforded by the implantable cardioverter–defibrillator.
Supported in part by grants from the Consiglio Nazionale delleRicerche and Telethon–Italy (to Dr. Spirito) and the MinneapolisHeart Institute Foundation and the Paul G. Allen Foundation(to Dr. Maron).
We are indebted to Enrica Bagnato for her secretarial assistance.
Source Information
From the Divisione di Cardiologia, Ente Ospedaliero Ospedali Galliera, Genoa (P.S.); the Divisione di Cardiologia, Ospedale Santa Corona, Pietra Ligure, Savona (P. Bellone); the Cattedra di Cardiologia, Università degli Studi, Genoa (P. Bernabò); the Servizio di Epidemiologia Clinica, Istituto Nazionale per la Ricerca sul Cancro, Genoa (P. Bruzzi) — all in Italy; and the Minneapolis Heart Institute Foundation, Minneapolis (K.M.H., B.J.M.).
Address reprint requests to Dr. Spirito at the Divisione di Cardiologia, Ente Ospedaliero Ospedali Galliera, Via Volta 8, 16128 Genoa, Italy, or at p.spirito{at}galliera.it.
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Maron, B. J., McKenna, W. J., Danielson, G. K., Kappenberger, L. J., Kuhn, H. J., Seidman, C. E., Shah, P. M., Spencer, W. H. III, Spirito, P., Ten Cate, F. J., Wigle, E. D., Vogel, R. A., Abrams, J., Bates, E. R., Brodie, B. R., Danias, P. G., Gregoratos, G., Hlatky, M. A., Hochman, J. S., Kaul, S., Lichtenberg, R. C., Lindner, J. R., O'rourke, R. A., Pohost, G. M., Schofield, R. S., Tracy, C. M., Winters, W. L. Jr, Klein, W. W., Priori, S. G., Alonso-Garcia, A., Blomstrom-Lundqvist, C., De Backer, G., Deckers, J., Flather, M., Hradec, J., Oto, A., Parkhomenko, A., Silber, S., Torbicki, A.
(2003). 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
42: 1687-1713
[Full Text]
Writing Committee Members, , Maron, B. J., McKenna, W. J., Danielson, G. K., Kappenberger, L. J., Kuhn, H. J., Seidman, C. E., Shah, P. M., Spencer, W. H. III, Spirito, P., Ten Cate, F. J., Wigle, E. D., ACCF Task Force on Clinical Expert Consensus Docum, , Vogel, R. A., Abrams, J., Bates, E. R., Brodie, B. R., Danias, P. G., Gregoratos, G., Hlatky, M. A., Hochman, J. S., Kaul, S., Lichtenberg, R. C., Lindner, J. R., O'Rourke, R. A., Pohost, G. M., Schofield, R. S., Tracy, C. M., Winters, W. L. Jr, ESC Committee for Practice Guidelines Members, , Klein, W. W., Priori, S. G., Alonso-Garcia, A., Blomstrom-Lundqvist, C., De Backer, G., Deckers, J., Flather, M., Hradec, J., Oto, A., Parkhomenko, A., Silber, S., Torbicki, A.
(2003). 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
24: 1965-1991
[Full Text]
15 mm in adult index patients or the equivalent wall thickness relative to body-surface area in children) in the absence of another cardiac or systemic disease that could produce a similar degree of hypertrophy.10,13,19 In adult relatives of the patients with hypertrophic cardiomyopathy, a wall thickness of 13 mm or more was considered a criterion for diagnosis.23 
15 or 
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