Chest pain during daily life in patients with hypertrophic cardiomyopathy: an ambulatory electrocardiographic study

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1 European Heart Journal (996) 7, 5664 Chest pan durng daly lfe n patents wth hypertrophc cardomyopathy an ambulatory electrocardographc study P Ellott, J C Kask, K Prasad, H Seo, A K Slade, J H Goldman and W J ckenna Department of Cardologcal Scences, St George's Hosptal edcal School, Cranmer Terrace, London SW7 ORE, UK Patents wth hypertrophc cardomyopathy frequently complan of chest pan durng daly actvtes STsegment depresson s descrbed n assocaton wth sudden death and pacng, but ts prevalence durng ambulatory electrocardographc montorng s unknown The am of ths study was to determne the relaton of ambulatory STsegment depresson to clncal characterstcs, rsk factors for sudden death and thallum perfuson n patents wth hypertrophc cardomyopathy Contnuous 48 h ambulatory electrocardographc montorng was performed n 3 patents (age 38 ± 4 years) wth hypertrophc cardomyopathy Nnetyfour (83%) recordngs were sutable for STsegment analyss A total of 9 epsodes of STsegment depresson (;> mm from baselne) were recorded n 5 (7%) patents (mean 4 ± 5) In patents < 3 years of age (but not >3) there was an assocaton between STsegment depresson and a hstory of exertonal chest pan (seven of vs one of ; P=), and dyspnoea NYHA class II/III Introducton Patents wth hypertrophc cardomyopathy frequently complan of typcal angna despte normal coronary arterograms ', suggestng that myocardal schaema may play a role n the pathophysology of the dsease Ischaemalke STsegment depresson s descrbed n patents wth hypertrophc cardomyopathy n assocaton wth syncope and cardac arrest' 3 and durng atral pacng' 4 However, the prevalence and clncal sgnfcance of STsegment depresson durng daly lfe n patents wth hypertrophc cardomyopathy and ts relaton to other nonnvasve markers of schaema has not Revson submtted September 995, and accepted 5 October 995 Ths study was supported by The Brtsh Heart oundaton Correspondence' Or Perry Ellott, Department of Cardologcal Scences, St George's Hosptal edcal School, Cranmer Terrace, London SW7 RE, UK (seven of 5 vs one of 7; /"=8) There was no assocaton between STsegment depresson and rsk markers for sudden death, e famly hstory of sudden death, syncope and noustaned ventrcular tachycarda, n any group Reversble thallum defects occurred n 7 (9%) of the 94 patents wth analysed recordngs but were not assocated wth symptoms, rsk factors for sudden death or ambulatory STsegment depresson In young patents wth hypertrophc cardomyopathy, schaemalke STsegment depresson s common and s assocated wth a hstory of typcal angna and dyspnoea Reversble thallum perfuson defects are assocated wth nether symptomatc status nor ambulatory STsegment depresson (Eur Heart J 996; ) Key Words Hypertrophc cardomyopathy, STsegment, yocardal schaema been systematcally analysed Ths study nvestgated the prevalence of STsegment depresson durng ambulatory electrocardographc montorng n a large seres of patents wth hypertrophc cardomyopathy and determned ts relatohp to clncal features and perfuson abnormaltes durng dpyrdamole stress thallum sngle photon computerzed tomography ethods Patents Nnetyfour coecutve patents wth hypertrophc cardomyopathy, evaluated between 99 and 99, were studed Cardoactve medcaton was dscontnued for at least fve halflves pror to electrocardographc evaluaton Nneteen addtonal patents who had been recevng amodarone (dose range 4 mg daly; serum levels 3 8 mg ~ ', mean mg ~ ') for at Downloaded from https//academcoupcom/eurheartj/artcleabstract/7/7/56/4534 by guest on 5 October X/96/756 9 $8/ 996 The European Socety of Cardology

2 ST segment depresson n hypertrophc cardomyopathy 57 least 3 months were also studed It was not feasble to dscontnue amodarone therapy because of ts long half lfe, but the patents were ncluded n order to avod selecton bas n favour of the 'low rsk' cohort Hypertrophc cardomyopathy was defned by characterstc clncal, echocardographc and haemodynamc features 5 ' 6 All 3 patents n ths study had left ventrcular hypertrophy (;> 5 cm) on twodmeonal echocardography, n the absence of any other cardac or systemc cause Patents wth a blood pressure of 56/9 were excluded Patents were aged 65 or less (range 965 years, mean 38 ± 4); 5 were under years of age, 9 aged 3, 9 aged 34, and 5 were older than 4 There were 7 males and 4 females A famly hstory of hypertrophc cardomyopathy and sudden death was present n 8 (5%) patents fty patents (44%) had dyspnoea, New York Heart Assocaton class II (n=45) and III (n = 5), and 8 (5%) patents had a hstory of syncope No patent had dyspnoea NYHA class IV A hstory of typcal angna pectors was present n 7 patents ourteen patents had atypcal chest pan (e pan at rest or of more than 3 mn duraton wthout evdence of nfarcton) fteen had both atypcal and exertonal pan Coronary arterography was performed only n those patents n whom there was a clncal ndcaton based upon age, symptoms and electrocardographc and/or radonuclde evdence suggestve of schaema Thrty patents (3 male, mean age 45 ± 3, range 965) were nvestgated; two (aged 6 and 65 years) had mnor coronary artery dsease (^4% dameter narrowng) n one vessel The remander had completely normal coronary angograms Baselne electrocardographc features Standard lead electrocardograms were performed n all patents usng a arquette system (arquette Electroncs Inc, Dagnostc Dvson, lwaukee, USA) Two patents had complete left bundle branch block and two had paced ventrcular rhythm Eghtyfour patents had abnormal baselne T wave nverson' 7 and/or ;> mm STsegment depresson n one or more standard leads Of these, 44 patents had T wave changes only, 3 had STsegment depresson wthout T wave nverson, and 7 had both sgnfcant T wave changes and <, mm STsegment depresson STsegment depresson was present n leads V5 6 n 33 patents and n one or more nferor leads (standard leads II, m, and av) n 4 Voltage crtera for left ventrcular hypertrophy, e sum of the S wave n lead VI plus the R wave n V5 equal to or greater than 35 mv 8 ', was present n 3 (7%) patents Noustaned ventrcular tachycarda, defned as three or more coecutve ventrcular extrasystoles wth a mean rate of ^ beats mn ~', lastng for less than 3 s, was detected durng 48 h ambulatory electrocardographc recordng n 39 (35%) patents Thallum magng Dpyrdamole thallum sngle photon emsson computerzed tomography was performed accordng to a prevously publshed protocol' 9 usng a wdefeld gamma camera (General Electrc 4T) Regonal perfuson was vsually assessed from a Bull'seye polar coordnate map, comparng the mmedate postdpyrdamole mage to the delayed mage at 3 h Echocardography Conventonal measurement technques on twodmeonal and mode echocardography ' were performed ean maxmal left ventrcular wall thckness was 3 ± 7 mm (range ^4); mean left ventrcular enddastolc dmeon 45 ± 6 mm (range 36) and mean left ventrcular endsystolc dmeon 6 ± 6 mm (range 5 45) ean left atral sze was 45 ± 9 mm (range 87) ortysx patents had left atral dameters greater than 45 mm (4%) Sxteen (4%) patents had complete systolc anteror moton of the mtral valve wth contact of the anteror mtral valve leaflet and the septum A calculated peak systolc left ventrcular outflow tract gradent of more than 3 mmhg was detected usng conventonal contnuous wave Doppler n 8 (6%) patents Contnuous ambulatory electrocardographc montorng All patents n the study underwent routne ambulatory electrocardographc montorng whlst performng unrestrcted actvtes of daly lfe Recordngs were made usng the arquette Holter recordng system on two channels, C5 on channel and V on channel Computer asssted analyss was performed usng the arquette Seres 8 Laser Holter and Laser Holter XP system (arquette Electroncs Inc, Dagnoss Dvson, lwaukee, USA) STsegment depresson was vsually dentfed from a computer generated STsegment trend Epsodes of horzontal or dowlopng STsegment depresson, mv or more from the baselne at 6 ms from the J pont, persstng for at least mn were coded as postve Each epsode had to be separated from other epsodes by at least mn Channel (C5) was used to detect STsegment depresson Recordngs were excluded from the analyss f they showed persstent atral fbrllaton or flutter (n = 9), left bundle branch block (n=) or persstent rght ventrcular pacng (n=3) because of the dffculty n dennng the soelectrc pont and the presence of secondary effects on the STsegment Tapes were also rejected f abnormalty of the PQ and/or STsegments precluded defnton of the soelectrc and J 6 ms ponts respectvely (n = 5) Indvdual epsodes were dsregarded f Downloaded from https//academcoupcom/eurheartj/artcleabstract/7/7/56/4534 by guest on 5 October 8 Eur Heart J, Vol 7, July 996

3 58 P Ellott et al Table Relaton between STsegment depresson durng ambulatory euctrocardographtc montorng and clncal varables n 94 patents wth hypertrophc cardomyopathy Sex Age (years) H hypertrophc NYHA II/III Exertonal chest pan Syncope NSVT SVT PG >3mmHg LVEDD (mm) LVESD (mm) LA (mm) LA >45 mm ax LVWT ST depresson (n = 5) ±3 5 (%) 4 (56%) 3(5%) 3 ()% 9 (36%) 6 (4%) 6 (4%) 44±5 5 ±4 45 ±7 3 (5%) 4 ±7 p r No ST depresson (n = 69) ±5 (9%) 5 (36%) 4 (35%) 3 (33)% (3%) 3 (33%) (4%) 46±5 7 ±6 43 ±9 4 (35%) 3 ±7 H hypertrophc = amly hstory of hypertrophc cardomyopathy and sudden death, NYHA II/III = dyspnoea of New York Heart Assocaton unctonal Class II and III; NSVT=noustaned ventrcular tachycarda durng ambulatory ECG montorng; SVT=supra ventrcular tachycarda; PG = peak left ventrcular outflow gradent; LA = mean left atral sze; LA>45 = left atral dmeon above 45 mm n dameter; LVEDD = left ventrcular end dastolc dmeon; LVESD = left ventrcular end systolc dmeon; ax LVWT=maxmum left ventrcular wall thckness of sudden oet and offset' and f assocated wth excessve baselne wander or artfact All postve recordngs were revewed and valdated by a second experenced observer Nnetyfour (83%) recordngs were codered to be sutable for STsegment analyss (59 male, 35 female, mean age 37 ± 4 years, range 965) (Table ) ortyeght hour recordngs were avalable n 8 patents The remander were of 4 h duraton Of the 9 patents takng amodarone, sx were excluded as ther recordngs demotrated persstent atral fbrllaton or flutter The ndcaton for treatment n the remander was supraventrcular arrhythma n two; the presence of recognzed rsk factors for sudden death (famly hstory of sudden death, syncope and noustaned ventrcular tachycarda) n and one was a survvor of outofhosptal ventrcular fbrllaton Statstcal analyss All results are expressed as mean values ± standard devaton The Student's ttest and the x test were used as approprate A P value of less than 5 was codered sgnfcant Results Ambulatory electrocardography A total of 9 epsodes of schaemalke STsegment depresson were detected n 5 patents (7 male and eght female, mean age 38 ±3 years, range 563) (Table ) ortynne epsodes were > mm from baselne Seventeen patents had two or more epsodes durng ther recordngs The mean number of epsodes per patent was 4 ± 5 (range ) The mean maxmal heart rate durng ambulatory electrocardographc montorng was 39 ± beats, mn" n those patents wth STsegment depresson and 33 ±6 n patents wthout STsegment change (/ ) =) Nne patents had one or more epsodes of STsegment depresson at heart rates of less than beats mn ~ ' Of the 3 patents aged 3 years or less, eght had traent STsegment depresson (Table 3) Of these, seven (88%) had a hstory of exertonal angna Only fve (%) of those wth negatve recordngs had a hstory of exertonal chest pan ( > =) In the young, STsegment depresson was also assocated wth a hstory of dyspnoea NYHA class II or III (P=8) There were no sgnfcant dfferences n the mean left atral, left ventrcular cavty dmeo or mean left ventrcular wall thckness n patents wth or wthout STsegment depresson n any group (Tables and 3) STsegment analyss was performed n 6 of the 3 patents who underwent coronary arterography pror to ambulatory electrocardographc montorng Seven of the 6 had postve recordngs The two patents wth mnor coronary artery leso had negatve ambulatory electrocardographc studes Relaton of ambulatory STsegment change to the baselne electrocardogram Twentyfve (7%) patents had baselne electrocardograms that fulflled the voltage crtera for left ventrcular hypertrophy There was no assocaton between the presence of hypertrophy by voltage crtera and STsegment depresson on ambulatory electrocardographc montorng n any group Twentynne of the 94 patents wth recordngs sutable for ST analyss had STsegment depresson of ;> mm n leads V5 and/or V6 on ther baselne lead electrocardogram Twelve (48%) of the 5 patents wth postve ambulatory recordngs had STsegment depresson n the lateral leads of the baselne electrocardogram compared wth 7 (5%) of those wth negatve tests (P=3) In those subjects aged less than 3, fve of the eght patents wth postve recordngs had ST depresson n leads V5 and/or V6 compared to fve of 4 wth negatve tests (/"=3) ourteen (48%) patents wth baselne STsegment depresson had a hstory of chest pan (/"=) and 6 a hstory of dyspnoea NYHA class II/III (/ > =) In patents under 3, sx had chest pan (/=) and seven had dyspnoea (P=) Downloaded from https//academcoupcom/eurheartj/artcleabstract/7/7/56/4534 by guest on 5 October 8 Eur Heart J, Vol 7, July 996

4 ST segment depresson n hypertrophc cardomyopathy 59 Table Electrocardographc characterstcs n 4 patents wth epsodes of STsegment depresson durng contnuous ambulatory electrocardographc montorng Age (years) Sex ax HR No eps 5 mm STdep < beats mn ' LVH Baselne ECG ST dep V5/ ax HR = maxmum heart rate durng the perod of recordng; No eps = number of epsodes of STsegment depresson ;>l mm from baselne; ^ mm = number of epsodes ^ mm from baselne; ST dep < beats mn ~ = epsodes of STsegment depresson at heart rates of < beats per mnute; LVH = left ventrcular hypertrophy (SV RV5^35 mv) on baselne lead electrocardogram, ST dep V5/6= ;> mm STsegment depresson n leads V5 and V6 of baselne lead electrocardogram Baselne STsegment depresson n leads V5/6 was assocated wth a greater mean left ventrcular wall thckness (5 ± 7 mm, vs ± 7 mm, P<) and a left atral dameter n excess of 45 mm (6 (55%) wth ST depresson vs (3%) wthout, / ) <4) In the cohort aged less than 3 years, there was an assocaton between baselne STsegment depresson and maxmal left ventrcular wall thckness (7 ± 5 mm vs ± 9 mm wthout ST depresson, / > <) but not left atral sze Left ventrcular enddastolc, endsystolc dmeo and outflow tract gradents were unrelated to the presence of baselne ST depresson n any group Thallum magng 9 _ 9 _ depresson, fve (%) had reversble defects only (/ > =), fve (5%) had fxed defects only (P=), three (%) had both types of perfuson abnormalty and had nether fxed nor reversble defects There was no assocaton between the presence of any type of perfuson defect and symptomatc status at the tme of magng (g 3) In the 7 patents wth at least one reversble defect, seven (6%) had a famly hstory of premature sudden death and hypertrophc cardomyopathy (/"=) nne (33%) had noustaned ventrcular tachycarda durng ambulatory electrocardography {P) and sx (%) had a hstory of syncope (Z'=) There was no assocaton between the presence of reversble thallum perfuson defects, syncope and/or a hgh rsk famly hstory n patents under 3 Downloaded from https//academcoupcom/eurheartj/artcleabstract/7/7/56/4534 by guest on 5 October 8 Of the 94 patents wth ambulatory recordngs sutable for STsegment analyss, (%) had one or more reversble perfuson defects as ther only abnormalty durng thallum sngle photon emsson computerzed tomography, 9 (%) had fxed defects only and seven (7%) had both fxed and traent perfuson abnormaltes Of the 5 patents wth STsegment Effect of amodarone on STsegment depresson STsegment analyss was performed n 3 (nne male, mean age 39 ± 4) of the 9 patents takng amodarone Two had STsegment depresson durng ambulatory Eur Heart J, Vol 7, July 996

5 6 P Ellott et al Table 3 Relaton between STsegment depresson and clncal varables n 3 patents aged less than 3 years Sex Age (years) H hypertrophc NYHA II/III Exertonal chest pan Syncope NSVT SVT PG >3mmHg LVEDD (mm) LVESD (mm) LA (mm) LA >45 mm ax LVWT ST depresson (n = 8) 7 4 ±5 3 (38%) 7 (88%) 7 (88%) (3%) (3%) (5%) (3%) 45 ±5 4 ±5 44±6 4 (5%) 5 ±6 8 No ST depresson (n = 4) 3 ±5 (4%) 8 (33%) 5 (%) (4%) 5 (%) 5 (%) 4(7%) 44±6 6 ±6 4 ± 6 (5%) 4 ±9 H hypertrophc=famly hstory of hypertrophc cardomyopathy and sudden death; NYHA II/III =dyspnoea of New York Heart Assocaton unctonal Class II and III; NSVT=noustaned ventrcular tachycarda dunng ambulatory ECG montorng; SVT=supra ventrcular tachycarda; PG=peak left ventrcular outflow gradent; LA = mean left atral sze; LA >45 = left atral dmeon above 45 mm n dameter, LVEDD = left ventrcular end dastolc dmeon; LVESD = left ventrcular end systolc dmeon; ax LVWT = maxmum left ventrcular wall thckness electrocardographc montorng ve of the 3 were less than 3 years old, one of whom had a postve recordng There was no assocaton between STsegment depresson durng ambulatory electrocardography and chest pan or dyspnoea n the remanng 8 patents not takng amodarone In the cohort aged less than 3 years, 7 were not takng amodarone In ths group STsegment depresson was sgnfcantly assocated wth chest pan (sx of wth pan vs one of 7 wthout, P<) and dyspnoea NYHA class II/III (sx of wth dyspnoea vs one of 5 wthout, P=) Dscusson STsegment depresson n patents wth hypertrophc cardomyopathy s descrbed durng rapd atral pacng n assocaton wth metabolc evdence for myocardal schaema' 4 and n solated case reports precedng sudden death and/or cardac arrest ' 3 ore recently, Camc et a/' 3 ' have demotrated that patents wth hypertrophc cardomyopathy who develop STsegment depresson durng dpyrdamole stress have lower coronary flow reserve than those wthout electrocardographc changes, suggestng that schaemalke electrocardographc changes may reflect abnormal myocardal metabolsm n some ndvduals wth the dsease The demotraton, n the present study, that STsegment depresson s relatvely common and s sgnfcantly assocated wth a hstory of typcal Y y (a) j; ;;H r A 4 A P (b) _ "/I I ; (c) ; ;[ 'r ll / L Ilk ^IHUIlll ST trend Heart rate (bpm) Hours gure ST segment and heart rate trends n a 3yearold female wth a hstory of dyspnoea and exertonal chest pan There was no hstory of syncope or premature sudden death n her famly One fourbeat epsode of noustaned ventrcular tachycarda was detected durng ambulatory electrocardographc montorng, but detaled electrophyscal assessment was normal The baselne electrocardogram (b) demotrates repolarzaton abnormalty of the ST segment In ths patent, epsodes of STsegment depresson were detected n 48 h Two patter were observed (a) bref epsodes assocated wth supraventrcular tachycarda; and (c) more prolonged epsodes assocated wth only a modest ncrease n heart rate The patent was treated wth verapaml resultng n the relef of both pan and dyspnoea She ded suddenly, however, at the age of 35 exertonal angna and dyspnoea n young patents wth hypertrophc cardomyopathy, provdes some qualfed support of ths hypothess A recent report has also suggested that myocardal schaema may be mportant as a trgger for sudden death n young patents wth hypertrophc cardomyopathy 4 In the present study, however, there was no sgnfcant assocaton between recognzed rsk factors for sudden death (e famly hstory of sudden death, syncope or noustaned ventrcular tachycarda) and STsegment depresson n Downloaded from https//academcoupcom/eurheartj/artcleabstract/7/7/56/4534 by guest on 5 October 8 Eur Heart J, Vol 7, July 996

6 ST segment depresson n hypertrophc cardomyopathy Reversble thallum defects (a) 6 Angna ;?t ; ; ; Ah r'x K ' ' ^ ^ II / Jl, I ;;! h, ^> J ' ; ( [; III V ^ ' 4 P= Reversble thallum defects (bj,,, I r ( ' f 'A L ;, jlf { l; f J ) T IIn A r t T ", < ' < f IB K,!! Dyspnoea (NYHA/3), J l ' ' ll l gure Ambulatory electrocardogram n a 45yearold male wth hypertrophc cardomyopathy and normal coronary arterography (a) Baselne electrocardogram demotratng normal QRS/ST morphology, (b) Electrocardogram demotratng STsegment depresson assocated wth central chest pan radatng to the left arm patents less than 3 years old Nevertheless, the lmtato of these adverse prognostc markers n young ndvduals wth the dsease are well recognzed and myocardal schaema rema an mportant possble stmulus for fatal ventrcular arrhythma n ths patent subgroup'5 Potental mechansms of myocardal schaema n hypertrophc cardomyopathy The pathologcal examnaton of hearts for patents wth hypertrophc cardomyopathy often reveals the presence of medal thckenng and lumenal narrowng n the small P= gure 3 Relaton between symptoms and dpyrdamole nduced reversble thallum defects n 94 patents wth hypertrophc cardomyopathy The presence of reversble regonal thallum perfuson defects was assocated wth nether chest pan nor dyspnoea n any group ntramural myocardal vessels'67 Ths may, together wth rased left ventrcular ntracavty dastolc pressures'8, an ncrease n the extra vascular component of mcrovascular resstance'9 and the ncreased metabolc demands of the hypertrophed myocardum', predspose to subendocardal hypoperfuson and schaema' Ths hypothess s supported by several studes that have shown that coronary flow reserve n patents wth hypertrophc cardomyopathy s reduced durng pharmacologcal and pacng nduced stress'8' ore recently, studes usng Doppler echocardographc technques have demotrated both mpared dastolc coronary blood flow and dramatc systolc flow reversal n the left anteror descendng artery3'4 Eur Heart J, Vol 7, July 996 Downloaded from https//academcoupcom/eurheartj/artcleabstract/7/7/56/4534 by guest on 5 October 8 y >

7 6 P Ellott et al Relatohp of thallum scntgraphy to myocardal schaema n hypertrophc cardomyopa thy Based on exteve experence n patents wth epcardal coronary artery dsease, a number of recent studes have used thallum sngle photon emsson computerzed tomography (SPECT) as a marker of myocardal schaema n young patents wth hypertrophc cardomyopathy In our vew the valdty of ths approach requres careful reexamnaton as, n spte of the frequency of reversble thallum perfuson defects n patents wth hypertrophc cardomyopathy, the presence of 'typcal' regonal hypoperfuson correlates poorly wth symptomatc status' 5 " 9 Wth the sngle excepton of a study by Ptcher et alp 9 all reports (ncludng the present study) have faled to demotrate an assocaton between reversble thallum abnormaltes and a hstory of typcal exertonal angna Ths fndng s mportant n vew of a recent study usng 3 Nammona and postron emsson tomography that demotrated sgnfcantly lower coronary flow reserve n patents wth hypertrophc cardomyopathy and chest pan Several workers' 5 ' 3 have nvestgated the relatohp between regonal thallum perfuson abnormaltes and metabolc markers of schaema In a study of 5 selected patents, Cannon et al [S] found that reversble thallum perfuson defects (defned as both regonal perfuson abnormalty and 'apparent cavty dlataton') were sgnfcantly more frequent n patents wth net lactate producton durng rapd atral pacng Ths s n contrast to an earler study by Hanrath et a/' 3 that found no relatohp between the presence of reversble thallum defects and pathologcal lactate extracton rates n patents The fact that coronary snus lactate concentraton s determned by a number of factors, some of whch are unrelated to myocardal schaema (eg substrate avalablty and adrenergc stmulaton' 3 ), mea that ts use as a 'gold standard' measure of myocardal schaema s lmted In addton, the tmng of venous samplng s crtcal as the maxmal 'washout' of acdc metaboltes after a perod of myocardal schaema may occur mmedately after the cessaton of stress and last for only a matter of seconds' 333 The recent demotraton of 'msmatch' between the uptake of thallum and regonal fatty acd metabolsm assessed by the radolabelled fatty acd analogue BIPP* 34 n patents wth hypertrophc cardomyopathy, suggests that further study of thallum knetcs n patents wth the dsease are requred before t can be safely assumed to be a setve clncal marker of myocardal schaema Ambulatory electrocardography, STsegment depresson and myocardal schaema n hypertrophc cardomyopathy The crtera for STsegment depresson used n ths study are dentcal to those employed for the detecton of Eur Heart J, Vol 7, July 996 myocardal schaema n patents wth chronc stable angna' 35 " 37 and have been valdated agat varous measures of myocardal perfuson' 38 "^ Ambulatory STsegment montorng s an establshed technque for the detecton of myocardal schaema n patents wth large vessel coronary artery dsease, but ts role n patents wthout coronary artheroma rema controversal or example, although Cannon et alp 5^ demotrated abnormal lactate extracton n 7% of patents wth hypertrophc cardomyopathy and exercse nduced STsegment changes, the prevalence n patents wthout STsegment change was 33% The poor overall correlaton between symptomatc status and ambulatory STsegment depresson n the present study s, therefore, costent wth the publshed lterature Nevertheless, the assocaton between STsegment changes and symptoms n young patents s noteworthy, as t supports a growng clncal suspcon that ths subgroup s partcularly vulnerable to myocardal schaema' 4 The patents n ths study represent a crosssecton of the populaton seen at a referral centre, usually sent for assessment because they have progressve symptoms, markers of adverse prognoss or requre specalst nvestgaton and/or treatment Ths s reflected n the present study by the hgh proporton wth a famly hstory of sudden death (5%) and the percentage of patents takng amodarone The latter were ncluded n order that the sample of patents remaned representatve Amodarone may have reduced both the prevalence of chest pan and STsegment depresson n the 3 patents takng t at the tme of electrocardogram montorng 4 ' 43 but t s dffcult to evaluate ths potental effect n the small cohort recevng treatment at the tme of ambulatory montorng Nevertheless, even when those patents takng amodarone are excluded from the analyss, the assocaton between symptoms and ST depresson perssts n the young cohort Exaggerated atral repolarzaton may nfluence the STsegment, partcularly durng tachycarda, and t s possble that morphologcal analyss of the PQ and STsegments can reduce the ncdence of falsepostve tests' In ths study, there was no sgnfcant dfference n the mean maxmal heart rate durng ambulatory electrocardogram montorng n patents wth and wthout STsegment depresson ean left atral dmeo were also smlar n both groups Data from postron emsson tomography ndcates that chest pan n patents wth hypertrophc cardomyopathy s perhaps the best nonnvasve clncal marker of reduced coronary flow reserve In keepng wth other publshed data, ths study of a large coecutve populaton wth hypertrophc cardomyopathy ndcates that there s a poor correlaton between clncal status and conventonal nonnvasve markers of myocardal schaema Therefore, the relable dagnoss of myocardal schaema n patents wth hypertrophc cardomyopathy requres a new dagnostc strategy, based on more setve measures of myocardal blood flow and metabolsm Downloaded from https//academcoupcom/eurheartj/artcleabstract/7/7/56/4534 by guest on 5 October 8

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9 64 P Ellott et al [4] Chercha S, Lazzar, reedman B, Brunell C, aser A [43] renneaux P, Counhan PJ, Porter A, Lpkn DP, Imparment of myocardal perfuson and functon durng ckenna WJ Effects of amodrone on erect and supne panless myocardal schaema J Am Coll Cardol 983; exercse haemodynamcs and exercse capacty n patents wth 943 hypertrophc cardomyopathy Eur Heart J 99; [4] Kask JC, Grott LA, Elzar V et al Effcacy of amodarone durng longterm treatment of potentally dangerous ventrcular arrhythmas n patents wth chronc stable schaemc heart dsease Am Heart J 984; Downloaded from https//academcoupcom/eurheartj/artcleabstract/7/7/56/4534 by guest on 5 October 8 Eur Heart J, Vol 7, July 996