the praecordial honk w · the'praecordial honk'or 'systolic whoop'is...

9
British Heart Journal, I97I, 33, 707-7I5. The praecordial honk Paolo Rizzon', Giuseppina Biasco, and Giuseppe Maselli-Campagna From the Division of Cardiology, Department of Internal Medicine, and Division of Cardiac Surgery, Department of Surgery, University of Bari, Italy The 'praecordial honk' or 'systolic whoop' is an intermittent, loud, musical, systolic sound which has been considered until a few years ago an extracardiac noise, probably pleuropericardial in origin, and therefore of no clinical significance. Only recently has it been suggested that this sound can represent a sign of mitral valve involvement. The pertinent findings of 9 cases are presented. Three were young women with minor congenital skeletal abnormalities, no history of acute rheumatic fever, and no abnormal auscultatory findings except a late systolic apical murmur; left ventricular cineangiocardiograms showed a systolic mitral valve ballooning and mild late systolic mitral insufficiency. The other 6 cases had evident heart disease of known aetiology: rheumatic in 3 cases and ischaemic in 3. All of them presented mitral insufficiency. No definite ballooning of the mitral valve was seen. Some different features of the 'praecordial honk' in the two groups are pointed out. The 'praecordial honk' or 'systolic whoop' is an intermittent, loud, musical, systolic sound which can occur with or without evidence of significant heart disease. The first description was probably given by Osler (i88o). Until a few years ago the 'praecordial honk' or 'systolic whoop' had been considered an extracardiac noise, prob- ably pleuropericardial in origin, and therefore of no clinical significance (McKusick, 1958; Received 30 November 1970. 1 Address for reprints: P. Rizzon, Clinica Medica, Universita, 70I24, Bari, Italy. Levine and Harvey, I959). Only recently Rackley et al. (I966) suggested that in some cases the 'praecordial honk' could be intra- cardiac in origin and caused by mitral regur- gitation. The same year, Leon et al. (I966) showed by means of intracardiac phonocardio- graphy that the late systolic murmurs and systolic 'whoops' arose from the mitral valve and represented a sign of mitral regurgitation. One year later, Behar, Whalen, and McIntosh (I967) presented 2 new cases and a re-evalua- tion of the cineangiocardiograms of 6 cases of the previous group of Rackley et al. (I966). FIG. I Phonocardiogram of Case i (A), Case 2 (B), and Case 3 (C) showing a crescendo- decrescendo late systolic murmur. PEIX ~ ~ ~~ .A 6C ECG BCG ~ ~ ~ * C A flOG __ .............. w_ a AR :::-: EA .; . ;- 80-160 Hzs APEPX 160-320 Hz t t L .. , N 80-160 Hz N ~~ CARO'!'. O~~~AROT, C It 5. i I 5, on February 15, 2020 by guest. Protected by copyright. http://heart.bmj.com/ Br Heart J: first published as 10.1136/hrt.33.5.707 on 1 September 1971. Downloaded from

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Page 1: The praecordial honk w · The'praecordial honk'or 'systolic whoop'is anintermittent, loud, musical, systolic sound which can occur with or without evidence of significant heart disease

British Heart Journal, I97I, 33, 707-7I5.

The praecordial honk

Paolo Rizzon', Giuseppina Biasco, and Giuseppe Maselli-CampagnaFrom the Division of Cardiology, Department of Internal Medicine,and Division of Cardiac Surgery, Department of Surgery, University of Bari, Italy

The 'praecordial honk' or 'systolic whoop' is an intermittent, loud, musical, systolic sound whichhas been considered until a few years ago an extracardiac noise, probably pleuropericardial inorigin, and therefore of no clinical significance. Only recently has it been suggested that this soundcan represent a sign of mitral valve involvement.

The pertinent findings of 9 cases are presented. Three were young women with minor congenitalskeletal abnormalities, no history of acute rheumaticfever, and no abnormal auscultatory findingsexcept a late systolic apical murmur; left ventricular cineangiocardiograms showed a systolicmitral valve ballooning and mild late systolic mitral insufficiency. The other 6 cases had evidentheart disease of known aetiology: rheumatic in 3 cases and ischaemic in 3. All of them presentedmitral insufficiency. No definite ballooning of the mitral valve was seen.Some different features of the 'praecordial honk' in the two groups are pointed out.

The 'praecordial honk' or 'systolic whoop' isan intermittent, loud, musical, systolic soundwhich can occur with or without evidence ofsignificant heart disease.The first description was probably given

by Osler (i88o). Until a few years ago the'praecordial honk' or 'systolic whoop' hadbeen considered an extracardiac noise, prob-ably pleuropericardial in origin, and thereforeof no clinical significance (McKusick, 1958;Received 30 November 1970.1 Address for reprints: P. Rizzon, Clinica Medica,Universita, 70I24, Bari, Italy.

Levine and Harvey, I959). Only recentlyRackley et al. (I966) suggested that in somecases the 'praecordial honk' could be intra-cardiac in origin and caused by mitral regur-gitation. The same year, Leon et al. (I966)showed by means of intracardiac phonocardio-graphy that the late systolic murmurs andsystolic 'whoops' arose from the mitral valveand represented a sign of mitral regurgitation.One year later, Behar, Whalen, and McIntosh(I967) presented 2 new cases and a re-evalua-tion of the cineangiocardiograms of 6 cases ofthe previous group of Rackley et al. (I966).

FIG. I Phonocardiogram of Case i (A), Case 2 (B), and Case 3 (C) showing a crescendo-decrescendo late systolic murmur.

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708 Rizzon, Biasco, and Maselli-Campagna

TABLE Auscultatory and phonocardiographic features of 'praecordial honk'

Case Age Sex Diagnosis Point of maximum intensity Timing IntensityNo. (yr) and transmission

I i8 F Ballooning mitral valve; Apex and midpraecordium, Constantly late Conspicuously variable (grademild mitral insufficiency radiating all over chest systolic 3-6/6), absent for long

periods (days or weeks)2 23 F Ballooning mitral valve; Apex and midpraecordium, Constantly late Conspicuously variable (grade

mild mitral insufficiency radiating all over chest systolic 3-6/6, absent for longperiods

3 i8 F Ballooning mitral valve; Apex and midpraecordium, Constantly late Conspicuously variable (grademild mitral insufficiency radiating all over chest systolic 3-6/6), absent for long

periods4 37 F Severe mitral stenosis; mild Midpraecordium Early, mid-, or Grade 3/6, absent for long

mitral insufficiency late systolic periods5 66 M Severe aortic stenosis; mild Apex, radiating into left Mid- or late Slightly variable (grade 3-4/6),

mitral insufficiency axilla systolic absent only for shortperiods (minutes or hours)

6 24 M Severe aortic stenosis and Apex and midpraecordium, Early or mid- Slightly variable (grade 4-5/6),insufficiency; mild mitral radiating into left axilla systolic absent only for shortinsufficiency and back periods

7 62 M Ischaemic heart disease; Apex, radiating into left Early or mid- Slightly variable (grade 3-4/6),moderate mitral insuffici- axilla systolic absent only for shortency periods

8 54 F Ischaemic heart disease; Apex, and midpraecordium, Early, mid-, or Slightly variable (grade 3-4/6),mild mitral insufficiency radiating into left axilla late systolic absent only for short

periods9 70 M Ischaemic heart disease; Apex, radiating into left Early, mid-, or Slightly variable (grade 3-4/6),

moderate mitral insuffici- axilla late systolic absent only for shortency periods

FIG. 2. Phonocardiogram of Case 2 (A) and Case 3 (B) showing the midsystolic clicks.

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The praecordial honk 709

Body position and respiratory Muller's manoeuvre Valsalva's manoeuvre Effort Amyl nitritechanges

Constantly initiated or accentuated Constantly decreased Constantly decreased Constantly increased Constantly elimin-in upright position and by or eliminated or eliminated atedexpiration

Constantly initiated or accentuated Constantly decreased Constantly decreased Constantly increased Decreasedin upright position and by or eliminated or eliminatedexpiration

Constantly initiated or accentuated Constantly decreased Constantly decreased Constantly increased Decreasedin upright position and by or eliminated or eliminatedexpiration

No change in upright position, initi- Increased Eliminated No evident changes Modified but slightlyated or accentuated by inspiration and unpredictably

Initiated or moderately accentuated Decreased Decreased Slightly increased Slightly decreasedin upright position and byexpiration

Initiated or accentuated in upright Eliminated or no Slightly decreased Conspicuously increased Slightly increasedposition; moderately accentuated evident changeby expiration

Initiated or accentuated in upright Moderately increased Decreased No evident changes Modified but slightlyposition; moderately accentuated and unpredictablyby expiration

No evident changes Decreased Slightly decreased No evident changes Modified but slightlyand unpredictably

Initiated or accentuated in upright No evident changes Slightly decreased No evident changes No evident changesposition; moderately accentuatedby expiration

FIG. 3 Phonocardiogram of Case I showinga loud 'honk' which occupies the second halfof the systole and extends into the second heartsound.

ECG

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APEX X320-640 lHz

CAROT'.

Three of them had obvious heart disease. Theother 5 had no significant heart disease, butpresented cineangiocardiographic evidence ofa ballooning of the mitral valve leaflets intothe left atrium during early ventricular systole.In 4 of them this mitral valve dysfunction wasassociated with late systolic mitral insuffici-ency.Nine cases with 'praecordial honk' will be

the subject of this report. Three of them hadballooning of the mitral valve and 6 an obviousheart disease of known aetiology. The clinicaland phonocardiographic features of these twogroups will be discussed.

Patients and methodsNine patients were seen in our department be-tween April I969 and April I970. Each had acomplete history, physical examination, electro-cardiogram, chest x-ray, and right heart catheter-ization. The left ventricle was reached throughretrograde aortic catheterization in all but 2(Cases 8 and 9). Left ventricular cineangiocardio-grams were performed by using an Arriflex 35 mmcamera at a speed of 34 frames/min. The contrastmedium (Angio Conray 8o%) was injected bymeans of a Gidlund apparatus at a pressure of7 kg/cm2. Phonocardiograms were obtained on afour channel Md. R. io8 r. Galileo Direct Recor-der with a frequency range of 6o to 6oo Hz at aspeed of 50 mm/sec in recumbent and uprightposition, during and after the Miuller and Val-salva manoeuvres, amyl nitrite inhalation, effort,

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7IO Rizzon, Biasco, and Maselli-Campagna

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FIG. 4 Phonocardiograms of Case I showing a

assumes the upright position.

and in some patients (Cases 1-3) after intravenousadministration of isoprenaline (o0o5 mg) and pro-pranolol (5 mg). All patients had been followed upclinically and phonocardiographically for a periodvarying from 3 to I2 months.

ResultsThe cases were divided into two groups ac-

cording to the presence of either 'ballooningmitral valve syndrome' (Group i), or a sig-nificant heart disease of known aetiology(Group 2).

Group I This includes 3 young women,aged i8 (Case I), 23 (Case 2), and i8 (Case 3)years. None of them had an illness whichcould have been pleuritis or pericarditis; inCase 3 an atrial septal defect had been previ-ously diagnosed by means of cardiaccatheterization.They were referred to our department be-

cause of the following symptoms: occasionalpalpitation and irregular heart beat, especiallywhen assuming the upright position, aftereffort or excitement; mild exertionaldyspnoea; vague pain over the anterior chest,not typical of angina; an intermittent,squeaking noise in the chest (Case i) on

assuming the upright position, after effort or

excitement.Physical examination revealed red hair and

fair complexion (unusual findings in SouthernItaly); minor skeletal abnormalities (higharched palate, short antero-posterior diameterof the chest, moderate kyphoscoliosis, andhollow foot in Case I; incurving little fingersin Case 2; moderate scoliosis and hollow footin Case 3); extrasystoles; slight (Cases i and3) or no (Case 2) enlargement of the heart; an

'honk' that becomes louder when the patient

intermittent, prominent systolic thrill overthe praecordium simultaneously with the'honk' (Cases i and 2); a soft apical late sys-tolic murmur (Fig. i) accompanied (Cases 2and 3) by a midsystolic click (Fig. 2); a latesystolic 'honk'.The electrocardiograms showed in all cases

ventricular ectopic beats, which were muchincreased by the slow intravenous injection ofa small amount of isoprenaline (o0os mg), by

FIG. 5 Phonocardiogram of Case I showinga 'honk' that develops during expiration fromlate systole, masking the late systolic murmur.

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The praecordial honk 711

physical effort, and when in the upright posi-

tion; an incomplete right bundle-branch

block was present in Case 3; the QT interval

and the T and U waves were normal. The

x-rays showed no left atrial enlargement; mnCase i a slight prominence of the pulmonary

artery and left ventricle was seen; Case 3

presented slight enlargement of the right ven-

tricle and a prominent pulmonary artery. At

cardiac catheterization the pressures were

found to be normal in the right heart, pul-

monary tree, and left heart; the pulmonary

capillary pulse showed no abnormalities; in

Case 3 the previous diagnosis of atrial septaldefect was confirmed. Left ventricular cine-

angiocardiograms showed in all cases a sys-

tolic ballooning of the mitral valve into the

left atrium and a mild late systolic regurgita-tion.

At surgical correction of the atrial septaldefect in Case 3 no abnormalities were ob-

served, except for conspicuously redundant

mitral leaflets. Mitral incompetence could

not be assessed at palpation. No pleuro-

pericardial adhesions were found.

The main clinical and phonocardiographiccharacteristics of the 'honk' (Table and Fig.

3-7) were the following: constantly situated

in the second half of the systole; at times end-

ing just before the second sound, at times

extending into it; intermittency with long

periods of absence; conspicuous spontaneous

changes in intensity; at times extremely loud

(grade 6/6) and associated with a prominent

apical thrill; on these occasions it could be

heard by the physician all over the chest;

almost constantly initiated or accentuated on

expiration, in upright position, by effort, and

ECGG

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80-160 Hz

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160-320 Hz

FIG. 6 Phonocardiogram of Case i showinga 'honk' that becomes smaller on inspiration.

isoprenaline; almost constantly reduced or

eliminated for a few seconds by the Miillerand Valsalva manoeuvres, by amyl nitrite

inhalation, and for longer periods by pro-

pranolol.

GroUp 2 This includes 6 patients (Cases

4-9) with heart disease of known aetiology.In the 4 patients (Cases 4-7) who underwent

left ventricular cineangiocardiography, mild

(Cases 4-6) or moderate (Case 7) mitral in-

FIG.- 7 Phonocardiogram of Case 2 (A) and Case 3 (B) showing a loud, late systolic 'honk'which envelops the second heart sound.

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712 Rizzon, Biasco, and Maselli-Campagna

competence, but no definite ballooning of themitral leaflets was shown. Cases 4, 5, and 6had a history of rheumatic fever; mitral in-sufficiency was associated in Case 4 (a womanaged 37) with tight mitral stenosis, in Case 5(a man aged 66) with severe aortic stenosis,and in Case 6 (a man aged 24) with severeaortic stenosis and insufficiency. All of thempresented with symptoms of cardiac failure:classes I, III, and IV, respectively. Case 4underwent closed mitral valvotomy. At opera-tion no pleuropericardial or pericardial adhe-sions were found; mitral incompetence couldnot be assessed at palpation. The cusps weremobile and free from calcium deposits. Bothcommissures could be easily split with thefinger. No relevant abnormalities of the chor-dae tendineae or papillary muscles were found.Ten days after the operation a decrease of theapical regurgitant murmur from grade 2/6 toi/6 was observed. The 'honk' disappearedand has not been seen afterwards during athree-months' follow-up. Case 6 underwentaortic valve replacement with a Starr-Edwards prosthesis. At operation the mitralvalve was found to be normal, except for therupture of a small group of chordae tendineae,attached to the posterior leaflet. The patientdied a few hours after operation. Permissionfor necropsy was not obtained.

Case 7 (a man aged 62), Case 8 (a womanaged 54), and Case 9 (a man aged 70) had nohistory of rheumatic fever. The history andthe electrocardiographic changes were typicalofischaemic heart disease; mitral insufficiencywas considered of moderate degree in Cases7 and 9, of mild degree in Case 8, and attri-buted to papillary muscle dysfunction. Therewas no cardiac failure.

While in 4 patients in this group (Cases 4,and 7-9), the diagnosis of mitral regurgitationcould be based on the typical auscultatoryfindings, in the 2 with aortic stenosis (Cases 5and 6) mitral insufficiency could be suspectedonly by the presence of the 'praecordialhonk'.The main features of the 'praecordial

honk' (Table and Fig. 8-I5) in this groupwere the following: variable in timing, movingin the same patient from the early to themiddle or late systole; at times absent, butonly for short periods; generally of moderateand slightly variable intensity; often difficultto diagnose because of its association with themurmurs of mitral insufficiency or of aorticstenosis (in some cases its presence could besuspected only by an intermittent change inthe quality of the murmur which acquired avibratory, honking quality), not associatedwith a thrill; inconstantly and less evidently

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FIG. 8 Phonocardiogram of Case 5 showingan ejection murmur and a late systolic 'honk'.

accentuated in the upright position and byeffort; less evidently and unpredictably modi-fied by respiration, the Muiller and Valsalvamanoeuvres, and by amyl nitrite inhalation.

DiscussionThe sex incidence, history, symptoms, andthe clinical findings of Group I closely re-semble those of the syndrome of 'mid-late

FIG. 9 Phonocardiogram of Case 5 showingthe variations in the intensity of the 'honk'from beat to beat; the 'honk' disappears inthe third systole.

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The praecordial honk 713

has often been found to be familial (Barlowand Bosman, I966; Linhart and Taylor, I966;Hancock and Cohn, I966; Stannard et al.,I967; Barlow et al., I968; Shell et al., I969),as well as in our cases of Group i, a peculiarmitral valve dysfunction characterized by thesystolic ballooning of the mitral leaflets intothe left atrium, often associated with mildlate-systolic mitral insufficiency, has been ob-served by cineangiocardiographic studies(Barlow and Bosman, I966; Linhart andTaylor, I966; Criley et al., I966; Hancockand Cohn, I966; Stannard et al., I967;Kesteloot and Van Houte, I965).

In a group of patients with this syndromestudied by Hancock and Cohn (I966)'several patients stated that their own mur-mur had been audible to them on occasion'.It can be presumed that on these occasions a

FIG. 10 Phonocardiogram of Case 5 showingthe variable intensity of the 'honk'; theassociation with a loud ejection murmur makesthe identification of the 'honk' difficult in thefirst systole, almost impossible in the secondone. 60G

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FIG. II Phonocardiogram of Case 6 showing:(A) decreased heart sounds, a loud ejectionmurmur, and a soft decrescendo diastolic mur-mur; (B) an early systolic 'honk' in the firstbeat and two 'honks' in the second beat: thefirst occupies early systole, the second latesystole.

FIG. 12 Phonocardiogram of Case 7 showinga systolic murmur and a midsystolic 'honk';the intensity of the 'honk' varies from beatto beat

systolic click and late systolic murmur' (Barlowand Pocock, I963; Barlow et al., I963; Barlow,I965; Barlow and Bosman, I966; Linhart andTaylor, I966; Criley et al., I966; Hancockand Cohn, I966; Stannard et al., I967;Barlow et al., I968; Shell et al., I969; Leach-man, De Francheschi, and Zamalloa, I969),also described as 'late systolic dysfunction ofthe mitral apparatus' (Engle, I969).

In many cases with this syndrome, which6

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714 Rizzon, Biasco. and Maselli-Campagna

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FIG. I3 Phonocardiogram of Case 7 showing an early and midsystolic 'honk' that presentsa changing shape; sometimes more than one 'honk' seems to be present

physical examination would very likely havedetected a praecordial honk.

Mid-late systolic clicks and/or late apicalsystolic murmurs have recently been seen inpatients with mitral insufficiency associatedwith the Marfan syndrome or other connectivetissue heritable disorders (Hancock and Cohn,I966; Barlow et al., I968; Segal, Kasparian,and Likoff, I962). Though none of thesediseases was present in our cases, they allpresented some minor congenital deformitiesof the skeleton. According to Shell et al.(I969), 'there would appear to be some rela-tionship between the mitral valve abnormalityof the Marfan syndrome, the floppy mitralvalve and mid-late systolic click and late-systolic murmur syndrome'.The nature of this valve dysfunction is still

obscure. A voluminous posterior mitral valveleaflet and thin elongated chordae tendineaehave been found at necropsy in one case ofBarlow et al.'s (I968) series with mid-late sys-

tolic click and late systolic murmur. Volu-minous mitral valve leaflets were also seen inour patient who underwent operation for thecorrection of an atrial septal defect.

In the patients of Group 2 with significantheart disease, the most important finding was

the constant association of the 'praecordialhonk' with mitral valve incompetence ofdifferent degree (mild to moderate) and differ-ent aetiology (rheumatic or ischaemic). Aballooning of the mitral valve was not present

in any of these patients who underwent a

cineangiocardiographic study.In conclusion, our observations indicate:

(i) the 'praecordial honk' is not an innocent

sound, being constantly associated with heartdisease; (2) it seems to present some distinc-tive features according to the nature of theheart disease: the still poorly recognized'ballooning mitral valve syndrome' on one

side, the acquired valvular diseases (rheu-matic or ischaemic) on the other side. The firsttype of lesions produces a 'praecordial honk'which is constantly late systolic, intermittent(when absent a midsystolic click and/or an

apical late systolic murmur can always be de-tected), at times very loud, presenting con-

spicuous spontaneous changes, often associ-

FIG. I4 Phonocardiogram of Case 7 showinga 'honk' that occupies the last part of thesystole and masks the second heart sound.

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The praecordial honk 7315

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FIG.15 Phonocardiograms of Case 9 showing a holosystolic apical murmur(A),...an.earlysystoli'honk that prtiall envelos the irst hert soud (B), ndtheintermitent.caracteof~~~~~~~~~~~~~~~.....the.'honk'.(C)

ated with a prominent apical thrill, almostconstantly initiated or modified in a predict-able way by a certain number ofmanoeuvres ordrugs. The second type of lesions produces a'honk' which is variable in timing in the samepatient (moving from the early systole to themiddle or late systole on different occasions),not associated with a thrill, more constant,with less evident spontaneous changes, diffi-cult to recognize because of its associationwith a systolic murmur, often scarcely orunpredictably modified by the already men-tioned manoeuvres; sometimes more thanone 'honk' is present in one systole.

ReferencesBarlow, J. B. (1965). Conjoint clinic on the clinical sig-

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