Br HeartJ_ 1993;70:266-271

Study of left and right ventricular function inRomano-Ward syndrome

Stephen J Cross, John C S Dean, Hai Shiang Lee, Murdoch Y Norton, Noel T S Evans,Francis Smith, Kevin Jennings, Stephen Walton

AbstractObjective-To examine left and rightventricular contraction in Romano-Wardsyndrome: does abnormal myocardiumaffect the predisposition to arrhythmia?Design-Tomographic radionuclide ven-triculography was performed after thered blood cells were labelled convention-ally with stannous pyrophosphate andtechnetium-99m.Setting-Department ofNuclear Medicine,Aberdeen Royal Infirmary.Patients-Eight subjects from twofamilies known to have Romano-Wardsyndrome, four of whom (two from eachfamily) had had symptoms.Results-The five subjects from family 1had normal left ventricular contraction;two had subtle abnormalities of rightventricular phase. In family 2 all threesubjects had abnormal left ventricularcontraction (reduced amplitude in three,abnormal phase in two). All had subtleabnormalities of right ventricular phase.Conclusion-Abnormal right or left ven-tricular myocardium may be partly orwholly responsible for the repolarisationchanges seen on the electrocardiogram ofthese families or may act as an ectopicfocus to start ventricular tachycardias ina susceptible heart.

(Br Heart J7 1993;70:266-27 1)

Romano-Ward syndrome is characterised bya prolonged QT interval and the propensityto recurrent ventricular arrhythmias that mayprecipitate syncope or sudden death.' 2 It is an

Pedigree 1

11

III

IV

1 2 3 4 5 6 7 8 9 10 11 12 13 14 15

o Symptom free, normal ECGO Symptom free, long QTco Symptomatic, ECG not available* Symptomatic, long QTc

Figure 1 Pedigree offamily 1. No electrocardiograms were available for . 1, 1.2 and II.4as they died before the start of the study.

inherited condition with autosomal dominanttransmission.3

It is proposed that there is an imbalance ofthe sympathetic innervation to the heart witha relative left sided over activity.4

Postmortem reports in some of thesepatients have found evidence of neuritis andmyocarditis.56 This has prompted us toexamine, in vivo, left and right ventricularfunction in members of two families with thiscondition.

Patients and methodsThe study was approved by the local ethicscommittee. Informed consent was obtainedfrom all patients.

ELECTROCARDIOGRAMSThe electrocardiograms taken for screening ofsymptom free relatives were recorded whenthe subjects were not receiving any treatmentknown to affect the QT interval. The QTcintervals were calculated with Bazett's for-mula.7 The QTc intervals quoted in the tablesare the longest that have been recorded onany occasion for that patient, and the valuesof QT and resting heart rates quoted corre-spond to these electrocardiograms. A value ofQTc above 440 ms is considered to be pro-longed. The U waves were differentiatedfrom T waves by the method described byLepeschkin.5

STUDY PATIENTSFamily 1This family first came to medical attentionwhen subject III.4 presented with a pseu-doepileptiform seizure. A biochemical profile,electroencephalogram, computed tomogra-phy scan of the brain, and echocardiographywere all normal. A resting electrocardiogram,however, showed a prolonged QTc interval of0.47 s. Holter monitoring showed episodes ofventricular bigeminy. Cardiac catheterisation(performed because of exertional chest pain)showed normal coronary arteries. The leftventriculogram was normal. Treatment withpropranolol abolished the pseudoseizures.

Subject III.10 presented independently atthe age of 32 with an epileptiform seizureduring a severe exacerbation of duodenalulcer disease complicated by duodenal steno-sis. Biochemical profile at presentationshowed hypokalaemia (2.4 mmol/l). Her QTcwas prolonged (0-54 s) and remained so aftercorrection of the electrolyte disturbance.

Family screening showed 15 other subjectswith abnormal electrocardiograms (fig 1).

Department ofCardiologyS J CrossH S LeeK JenningsS WaltonDepartment ofMedical GeneticsJ C S DeanDepartment ofMedical PhysicsMY NortonN T S EvansDepartment ofNuclear Medicine,Aberdeen RoyalInfirmaryF SmithCorrespondence to:Dr S J Cross, Department ofCardiology, Aberdeen RoyalInfirmary, Foresterhill,Aberdeen AB9 2ZD.Accepted for publication18 January 1993

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Table 1 Demographic data of members offamily 1 aged over 18

Patient Age (yr) Sex Heart rate QT (s) QTc (s) Symptoms RNV

II.2 69 F 64 0-60 0-62 - -II.6 56 F 48 0 54 0 57 - -III.1 47 F 64 0-60 0-62 + -III.3 45 F 53 0-64 0-60 - +III.4 44 F 73 0-58 0-64 + +III.7 36 M 80 0-54 0-62 - +III.10 33 F 75 0-56 0-63 + +I1.15 33 M 70 0-58 0-63 + -III.16 30 M 48 0-62 0-55 - -III.17 27 F 103 0-38 0 50 - +IV.6 27 F 60 0-64 0-64 - -IV.7 25 F 64 0-62 0-64 - -IV.10 22 F 83 0 40 0 47 - -

RNV, radionuclide ventriculography; QTc is maximum recorded at any time for each subject.

Table 1 shows the demographic details of the13 affected adults assessed.

Subject III. 1 died suddenly at home shortlyafter diagnosis. She was aged 46, and hadbeen receiving diuretic treatment for mildhypertension, but her biochemical profile wasnormal. One night she felt unwell, rose fromher bed, and collapsed and died. A detailedpostmortem examination found no abnormal-ities. It was concluded that an arrhythmia hadbeen responsible for the death.

Subject III.15 had one episode of syncopeafter two episodes of near syncope at the ageof 18, while under severe physical stress.

Other family members have been symptom

aVR vi V4

aVL V2 V5

III aVF '13 '16

Figure 2 Electrocardiogram from111.7 (family1) showing prolonged QTc at -0S0s,andabnormal ST segment configuration. Patient was not receiving treatment.

aVR vi '14

aVL '12 '16

In ~~~aVF '13 V6

Figure 3 Electrocardiogram from 111.17 (family 1) showing prolonged QTc at O-47 s,and inverted or biphasic T waves in the chest leads. Patient was not receiving treatment.

free, with the exception of II.4 who isreported to have had one seizure after beingstartled at the age of 6. Unfortunately, noelectrocardiogram is available as he diedsome years ago from malignancy.The electrocardiographic findings in this

family take one of three forms: ST changeswith QT prolongation (fig 2), inverted orbifid T waves, or both, with prolongation ofthe QT interval (fig 3), or normal ST seg-ment T wave morphology with prolongationof the QT interval. There have been norecorded episodes of T wave alternans.Invasive electrophysiology has not beenundertaken in any member of this family.Of the 12 living members who are over 18

years of age, five agreed to take part in theresearch studies: two of these have beensymptomatic.

Family 2This family first came to medical attentionwhen IV.2 presented with focal convulsionsat the age of 31 years. A computed tomogra-phy scan of the brain was performed, whichwas reported to be normal. Treatment withphenytoin for presumed epilepsy was begun.

Five years later, at a routine school medicalexamination, V.1 (daughter of IV.2) wasfound to have an irregular pulse. On directquestioning she complained of some short-ness of breath on cross country running,episodes of dizziness, and presyncope. Therehad not been any episodes of loss of con-sciousness. An electrocardiogram showedunderlying sinus rhythm and ventricular extrasystoles with runs of non-sustained polymor-phic ventricular tachycardia, which were alsoseen on a 24 hour Holter recording (fig 4).The QTc interval at this stage was normal,but there were prominent U waves, especiallyin the right chest leads.An invasive electrophysiology study, per-

formed at another centre, showed normalsinus node function, functional duality of theatrioventricular node conduction, a normalHV interval, and normal ventricular refrac-tory periods. Aggressive stimulation in multi-ple right ventricular sites failed to provoke asustained arrhythmia. A Valsalva manoeuvreproduced a flat heart rate response, did notcause any elecrocardiographic changes, anddid not precipitate an arrhythmia. Holtermonitoring showed a break in the arrhyth-mias overnight, with an early morning resur-gence.An electrocardiogram taken from IV.2 at

this time showed considerable repolarisationchanges (similar to those of her daughter)and a period of non-sustained ventriculartachycardia. The clinical and electrophysio-logical findings were considered consistentwith Romano-Ward syndrome.

Permanent pacing, with additional ,B block-ade in the case of V.1, has been a successfultreatment in these two patients, stopping allpalpitations and blackouts.

Electrocardiograms were obtained from theother members of the family, none of whomhave any impairment of their hearing. A total

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VT X04:24 Chl....................... .. .:.

~~~~~~~~~~~~~-

.~~~~ ~ ~ ~ ~ ~ ~ ~ ~

Figure 4 Recordingfrom a 2 channel 24 hour electrocardiogram from indiv:(family 2) showing a selflimiting run of multiform ventricular tachycardia.

of five members of this family are

to have Romano-Ward syndromThese five have prominent U wavelectrocardiograms (fig 6), some

longed QT and QTc intervals, anda sinus bradycardia (table 2). No e

11

III

IV

V

Pedigree 2

2

3

2

1 22

o Symptom free, normal ECGo Symptom free, long QTc and/o

abnormal U wave* Symptomatic, long QTc and/or

abnormal U wave

Figure 5 Pedigree offamily 2. No electrocarwere available for . 1, .2, II. 1, and II. 3 as tbefore the start of the study.

avR

II aVL

Tl aVF

VI V4

V2 V5

V3 V6

Figure 6 Electrocardiogram from affected member (V.2) offamily 2 showinubradycardia, a QTc of 0-42 s, and abnormally prominent U waves. Patientreceiving medication.

T wave alternans has been recorded. In thisfamily there have not been any sudden unex-plained deaths.

TOMOGRAPHIC RADIONUCLIDE VENTR,ICULOG-RAPHYThe blood pool was labelled conventionallywith sequential injections of 7T5 mg stannouspyrophosphate and 800 MBq of technetium-99m. Tomography of the cardiac blood poolwas then undertaken with the Aberdeen sec-tion scanner.

This scanner has been describedidual V.1 elsewhere,9 10 but in summary it is a gated,

focused tomographic scanner that uses sixtransaxial slices, measured sequentially, toimage the heart in less than 20 minutes.

considered These individual slices are interpolatedLe (fig 5). together to produce a three dimensional dataes on their image of the ventricular blood pool, at eachhave pro- of eight phases through the cardiac cycle.some have Amplitude, ejection fraction, and phase mapsepisodes of can be superimposed on this image, which

can be orientated to view any aspect of thecardiac blood pool.

At the time of image reconstruction themean RR interval during acquisition of the

-;e2 data is calculated; only data from those cycleswith RR intervals between 60% and 140% ofthis value are included in the final construc-

vCJ3 tion of the image. Hence, extrasystoles arenot included in the final analysis.The left ventricle, because it is cone shaped

472 is particularly suited to be represented by a

bull's eye display. The apex of the left ventri-cle corresponds to the centre of the image,the anterior wall at the top, the septum on theleft, the inferior surface at the bottom, andthe free lateral wall on the right (fig 7).Routinely we produce separate bull's eyeimages for ejection (or amplitude) and phase.

For objective interpretation of the left ven-tricular images, data from 50 normal controls

r has been incorporated into the analysis pro-gramme. The ejection and phase bull's eyesfrom a patient study are compared with thesecontrols, and abnormal areas (outside 2 SDs)

rdiograms are displayed on separate corresponding errorthey died maps (fig 8). Normal regions of left ventricle

are depicted as white areas on these errormaps.The right ventricle, because of its crescent

shape, is not suited to display in a bull's eyeformat. It is therefore necessary to view phase

<- and amplitude on the more anatomically cor-rect three dimensional image. Because wehave found a wide variation in normalregional wall amplitude of the right ventricle,only the phases of this structure have beenstudied in our subjects." This was done bysuperimposing the phase values on to a threedimensional image (in an approximate rightanterior oblique projection).To recognise abnormal areas of phase we

view the phase map as a two tone image:> 9 areas of the phase within the normal range

(which we have previously defined) appearingzg sinus as red, and areas outside this range blue.'2was not This, once again, allowed objective assess-

ment of the images.

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Table 2 Demographic data of affected members offamily 2

Patient Age (yr) Sex Heart rate QT (s) QTc (s) Symptoms RNV EF (%)II.2 86 F 61 0-45 0 45 - - -

HII.3 64 F 58 0-48 0 49 + - -

IV.2 42 F 38 0 34 0 43 + + 27V.1 19 F 45 0-41 0.47 + + 26V.2 18 F 71 0-46 0-42 - + 38

RNV, radionuclide ventriculography; EF, ejection fraction; QTc is maximum recorded at any time for each subject.

Figure 7 Regions of the left ventricle depicted as a bull'seye image.

Figure 8 Bull's eye image of left ventricular ejection and phase with corresponding errormaps for patient IV. 2 (family 2). There is reduced ejection in the anterior, inferior, andlateral regions. The ejection fraction is reduced (normal >40%). The phase is abnormal inthe inferior and lateral regions. Normal areas on the error maps are coloured white.

Alternatively, the phase of either ventriclecan be studied by superimposing a dynamicphase wave on to the three dimensionalimage, and viewing the changes in phase inreal time. Although this technique is subjec-tive, we have found excellent betweenobserver agreement (unpublished data).When the data images are reanalysed, the val-ues of ejection and phase are consistent(unpublished data).During acquisition of the scans, patients

IV.2 and V.1 had the rates of their pacemak-ers reduced so that their underlying (sinus)rhythm was present.

ResultsLEFT VENTRICULAR FUNCTIONAll three members of family 2 had abnormalleft ventricular function. The mother (IV.2)had a large area, covering most of the leftventricle, of reduced ejection with a matchingphase abnormality (fig 8). The ejection frac-tion was reduced at 27% (normal >40%).Ejection is linearly related to amplitude, butis not dependent on the number of counts foran absolute value.The symptomatic daughter (V.1) had a

reduced area of ejection in the inferolateraland apical regions of the left ventricle, butwithout any phase abnormality. The ejectionfraction was reduced at 26%. The symptomfree daughter (V.2) had a small matching areaof reduced amplitude and abnormal phase inthe inferolateral region of the left ventricle.The ejection fraction was minimally reduced(38%).The scans of family 1 did not show any

areas of abnormal left ventricular contraction:they all had normal ejection fractions.

RIGHT VENTRICULAR FUNCTIONThe findings of the right ventricular phaseswere not uniform. Whereas all subjects hadvalues of right ventricular phases within thenormal range, the dynamic phase wave wassometimes abnormal.

In family 2, V.1 and V.2 had abnormalphase waves in that they started in the middlepart of the anterior free wall of the right ven-tricle instead of towards the right ventricularapex. In IV.2 the phase wave started in thenormal region but its progress was delayedand persistent at a small area towards theright ventricular outflow tract.

In family 1, III.17 and III.7 the phase wavestarted in the normal area but progress wasdelayed and persistent in a small area at the

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posterior aspect of the right ventricular apex.The location of these abnormal areas wasidentical for these two subjects.The other members of family 1 had normal

right ventricular scans.The results of the radionuclide angiogra-

phy prompted us to perform echocardiogra-phy on the two symptomatic patients fromfamily 2. Both of these subjects had reducedmotion in the apical region of their left ventri-cles. The ejection fraction, from a single viewin the parastemal short axis orientation, was,however, in the normal range. Right ventricu-lar function seemed normal.

DiscussionRomano-Ward syndrome is a rare hereditarydisorder characterised by a prolonged QTinterval and a propensity to ventricular tachy-cardias that may precipitate sudden death.' 2The prolonged QT interval reflects delayedventricular repolarisation. This state may pre-dispose to arrhythmias after ventricularextrasystoles.Autonomic neuropathy in diabetic patients

can produce QT prolongation,'3 as can rightstellate ganglionectomy or left stellate stimu-lation in animal models. 14 It has thereforebeen inferred that autonomic dysfunction isthe most likely mechanism of QT prolonga-tion in Romano-Ward syndrome.

Postmortem studies performed in somepatients with Romano-Ward syndrome haveshown focal areas of neuritis andmyocarditis.5 6

It has been proposed that an intracardiacabnormality, instead of a neural defect, coulddecrease electrical stability and make themyocardium more vulnerable to sympatheticdischarges.'5 A cellular mechanism has beensuggested to explain such a susceptibility.'6

There has been a single case report of thefindings of electron microscopy in the heartof a Romano-Ward patient; this suggested amyocardial metabolic disturbance as theunderlying defect.'7The two families we have studied both

have members with prolonged QTc intervals.The QTc interval can vary with time in indi-vidual subjects.7 18 Therefore we have quotedthe highest values obtained for each subject.Propranolol can affect the QTc interval, typi-cally shortening it. 920

Multiform ventricular tachycardia has beendocumented in two members of family 2.Patients with a long QT syndrome, if proneto arrhythmias, typically have torsades depointes. Although we have not identified thisspecific tachycardia in any of our patients,ventricular tachycardia with a similar configu-ration to that seen in our subjects has beendescribed previously in Romano-Ward syn-drome.2' 22

The prominent U wave seen in family 2does not seem to be dependent on the heartrate: sinus bradycardia was not present inV.1.

In family 1 an apparently healthy member(III.1) died suddenly, presumably from an

arrhythmia. The presence of hypokalaemia inindividual III. 10 in association with her pseu-doseizure may be important, as it is knownthat hypokalaemia prolongs the QT interval,23and this is thought to increase the tendencyto ventricular arrhythmia in those with a con-genital prolongation of the QT interval.Precipitation of arrhythmias by physical exer-tion has been described previously inRomano-Ward syndrome,4 and this may berelevant to the experience of III. 15.

Both families have normal hearing, and thefamily trees suggest an autosomal dominantinheritance: the affected members of thesefamilies therefore have Romano-Wardsyndrome.Romano-Ward syndrome does not have

the same importance for each affected per-son: frequency of symptoms can vary frommany syncope attacks a month to a few eachyear, or only one or two episodes during thewhole of the subject's life.4The Aberdeen section scanner has been

shown to be a sensitive method of detectingregional abnormalities in wall motion. It ismore sensitive in this respect than conven-tional planar techniques, especially for imag-ing the inferior surface of the left ventricle.2425Although the images of our subjects were notinterpreted blinded, analysis of left ventricu-lar phase and amplitude is objective becauseof the use of error images. Similarly the threedimensional display of values of right ventric-ular phase is objective, whereas the dynamicdisplay of spread of left or right ventricularphase is subjective.The left ventricular wall motion as assessed

by echocardiography in IV.2 and V.1 in fam-ily 2 confirrn the findings from radionuclideangiography. The normal ejection fractionsmeasured with ultrasound were not surprisingas the wall motion abnormalities on echowere local and not global, and we only used asingle view for calculation of ejection fraction.The assessment of ejection fraction byechocardiography in these instances is verymuch dependent on the regions sampled.This is not a limitation of tomography, wherethe whole of the ventricle is included in thecalculation of ejection fraction. Anotheradvantage of nuclear imaging is that theimages obtained are not dependent on thesubject having a good acoustic window.

It was important to restrict our investiga-tions to the younger members of the two fam-ilies, so that any abnormality in wall motionwould be a result of the Romano-Ward syn-drome and not damage from ischaemic heartdisease. The only patient with exertionalchest pain was found to have normal coro-nary arteries at cardiac catheterisation.

Only members of family 2 had detectableleft ventricular defects. These patients alsohad reduced ejection fractions, more pro-nounced in the two with symptoms.The differences between the two families in

the electrocardiographic morphology, and inthe finding of left ventricular abnormalities infamily 2, but not in family 1, suggest thatRomano-Ward syndrome is a heterogeneous

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disorder. Indeed, within family 2, it may benecessary to have a specific degree of abnor-mality of the left ventricular muscle wallbefore the manifestation of symptoms. It willbe interesting to see if subject V.2 developssymptoms in the future, and if so, if there hasbeen any deterioration in left ventricularfunction.The subtle abnormalities of right ventricu-

lar phase are interesting but not uniformeither within or between families. The pres-

ence of an abnormal right ventricular phasedid not relate to the presence or frequency ofsymptoms.

Perhaps our understanding of this interest-ing syndrome is limited because it is a collec-tion of syndromes within a syndrome, all withslightly different pathology but similar clinicalpresentations.

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