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Bedside Clues to the Diagnosis ofHeart Disease*

JOHN F. GOODWIN, M.D .

London, England

A PART of bedside diagnosis, the moderncardiologist is entitled to an electrocardio-

gram and good chest x-ray film in order to makea clinical assessment of cardiovascular disease .

In this lecture I shall hope to show how, onthe basis of the personal experience of myself andof my colleagues at the Postgraduate MedicalSchool of London, bedside clues have helped toarrive at a final diagnosis in certain forms ofcongenital and acquired disease . Naturally,such a lecture can touch only superficially on afew disorders, but it is hoped that what I have tosay will be of some value in indicating the impor-tant principles of diagnosis at the bedside . Inparticular, I should like to stress that clinicalbedside diagnosis is essential in planning thetype of investigation needed to arrive at a finaland comprehensive picture of the patient's illness .

JUGULAR VENOUS PULSE

I shall begin by considering the jugular venouspressure, to the study of which the late PaulWood brought so much fruitful endeavor .' Itshould be realized that this pulse has much totell the physician before the external jugularveins become distended . Jugular venous dis-tension is often merely a sign of mediastinalobstruction or gross elevation of jugular venouspressure . The aim should be to recognize thenormal jugular venous pulse, and its abnormalcharacteristics in various forms of disease at astage when pulsation of the deep veins only canhe seen and there may be no filling of the ex-ternal jugular veins .

The normal venous pressure should just he visibleto a height of I to 3 cm. above the sternal anglewith the patient lying at 45° .

A normal venous pulse consists of three mainwaves, the a wave due to atrial contraction,

which is followed by a negative or x wave due toatrial relaxation .' This is followed by a secondpositive deflection, the v wave due to atrial fillingand obstruction to blood flow during ventricularsystole, which in turn is succeeded by a negativedeflection, the y wave, which is due to emptyingof the atrium as the tricuspid valve opens andthe blood enters the ventricle . There is some-times a small positive deflection on the down-stroke of the x wave, the c wave, which may bedue to ventricular contraction and tricuspidvalve closure, but often, at least on externaljugular phlebographic records, is due to carotidartefact .Figure 1 shows a very crude and purely

diagrammatic representation of the jugularvenous pulses and the auscultatory phenomenain four heart diseases which affect the jugularvenous pulse in a distinctive way . This diagramis not to scale .2

Pulmonary Hypertension : The upper of thefour diagrams represents the jugular venouspulse in pulmonary hypertension . As a resultof the right ventricular hypertension, right atrialhypertrophy occurs, and this produces an aug-mented a wave in the jugular venous pulse,which can be seen as a flicking pulsation abovethe sternal angle lateral to the carotid pulse andpreceeding it . In cases of extreme right atrialhypertrophy, the a wave may be palpable .The auscultatory phenomena in severe pul-monary hypertension consist of a short ejectionsystolic murmur, which is often soft, precededby a pulmonary ejection click . The pulmonarycomponent of the second heart sound is alwaysaccentuated and narrowly separated from aorticclosure . There may be a fourth heart sounddue to atrial hypertrophy and corresponding tothe a wave, and occasionally a soft high pitched

* From the Postgraduate Medical School and Hammersmith Hospital, London, England .Guest Lecture read at the 13th Annual Meeting of the American College of Cardiology, New Orleans, February 16,

1964.

VOLUME 15, JANUARY 1965 81

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FIG . 1 . Schematic representation of the jugular uenour pulse andauscultatory signs in pulmonary hypertension, lone pulmo-nary stenosis, tricuspid stenosis and cardiac constric-tion . (The drawings are not to scale .) (A = atrialsound . I = first heart sound . C = systolic c lick . SM =systolic murmur. A, = aortic valve closure . P, =pulmonary valve closure . PDM = pulmonary dia-stolic murmur . TSM = tricuspid systolic murmur .TMDM = tricuspid mid-diastolic murmur) . See textfor description of jugular venous pulse waves . (Repro-duced from Clinical Disorders ofthe Pulmonary Circula-don by permission of the publishers . J. & A . ChurchillLtd.%).

early diastolic murmur is heard at the left sternaledge, due to pulmonary valvar incompetence .

Pulmonary Stenosis : Although the jugularvenous pulse in pulmonary stenosis with closedventricular septum differs in no way from that ofpulmonary hypertension, because the cause isthe same, the auscultatory phenomena are en-tirely different . The systolic murmur is of adiamond shape, maximal in mid- and latesystole, extremely loud and accompanied by athrill . The murmur extends over the soundmade by aortic closure up to the soft and de-layed pulmonary closure . In both conditionsthe right ventricle is large and may be felt as atapping substernal impulse or sometimes maylift the sternum when dilatation is added tohypertrophy. When tricuspid incompetenceoccurs as a complication, the x descent of the

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jugular venous pulse is obliterated and replacedby a large systolic wave due to regurgitation ofblood from the ventricle into the atrium duringventricular systole .

Tricuspid Stenosis : In this condition, de-picted in the third diagram, the a wave is alsoaugmented, but the y descent due to atrialemptying, instead of being sharp, is slow andleisurely, reflecting the obstruction to inflow tothe ventricle . There is commonly an atrialsound, and since tricuspid incompetence mayaccompany tricuspid stenosis, there may be asystolic murmur in the tricuspid area . Usually,there is a low-pitched rumbling diastolic mur-mur heard in the same area, increasing on in-spiration .

Cardiac Constriction : Finally, when the heartis constricted by constrictive pericarditis, peri-cardial effusion or by endomyocardial disease,the a and v waves are augmented, and x and ydescents sharp, the y descent being particularlyobvious . The a and v waves reflect the highventricular end-diastolic pressure which causesan increased right atrial pressure ; the sharp xdescent indicates the absence of tricuspid in-competence while the y descent reflects rapidfilling of the ventricle due to absence of tricuspidvalve obstruction . The murmurs in cardiacconstriction are often trivial, as shown in thediagram, the second sound being commonlywidely split, and there is nearly always a thirdsound which coincides in time with the end-diastolic pressure in the right ventricle and withthe y descent . The third sound is due to thesudden arrest of ventricular filling by the thickpericardium . The cardiac impulse reveals adiastolic thrust coinciding with the early dia-stolic sound . 8

Thus, by attention to the jugular venous pulseand auscultation, considerable information ofgreat value may be obtained at the bedside inelucidating these four groups of disorder .There are, of course, many other abnormalitiesof thejugular venous pulse . These have merelybeen selected to illustrate the value of appreciat-ing the form of the venous pulse .

PULMONARY HYPERTENSION

Returning to pulmonary hypertension, it mustbe noted that there are many causes and degreesof severity. The physical signs which havebeen mentioned represent extreme pulmonaryhypertension such as is met with in conditions inwhich the pulmonary vascular bed at arteriolarlevel is obliterated by extreme vasoconstriction

THE AMERICAN JOURNAL OF CARDIOLOGY

or obliterative disease such as thromboembolism .The facies in such pulmonary hypertension maybe characteristic, consisting of bright red cheeks,with marked circumoral pallor . The color ofthe cheeks differs slightly from the characteristicmitral facies in that the color tends to be red orscarlet rather than purple, but the difference onoccasions may only be slight . These facies arenot usually seen in patients with milder forms ofpulmonary hypertension such as that due toexcessive pulmonary blood flow or moderatevasoconstriction .

The cause of pulmonary hypertension may often besubstantially elucidated at the bedside . One ofthe commonest causes is a left-sided cardiaclesion which produces pulmonary venous hyper-tension, which in turn excites pulmonary ar-teriolar vasoconstriction . The commonest le-sion is, of course, mitral stenosis, and the charac-teristic murmurs can usually be detected byauscultation, although when an extremely highpulmonary vascular resistance is present, mitralmurmurs may be faint . Other left-sided lesionswhich may also produce pulmonary hyperten-sion are left heart failure from any cause, leftatrial tumors, congenital abnormalities of theleft atrium and pulmonary venous disease .Extreme pulmonary hypertension, however, asalready outlined, only occasionally occurs withmitral valve disease and is usually found in con-ditions which involve primarily the pulmonaryarteriolar bed . It is unusual for asphyxial corpulmonale to produce such severe degrees ofpulmonary hypertension ; and when the physi-cal signs are striking, the diseases which shouldparticularly be considered are obliterative dis-orders, principally pulmonary thromboembo-lism and the Eisenmenger reaction,* in whichpatients with a large intracardiac communicationhave obliteration of the pulmonary arteriolarbed and reversal of the left to right shunt .

In the Eisenmenger syndrome, cyanosis is com-monly present although it may be slight ; andwhen the cause is a patent ductus arteriosuswith reversed flow, the lower limbs may showcyanosis with clubbing which is not present inthe hands (Fig . 2) . Careful inspection, there-fore, of the whole body, not merely of the upperlimbs, may yield a valuable clue to the cause ofthe pulmonary hypertension .

An atrial septal defect may be considered whenthe second heart sound is widely split . Theradiograph is of value in this regard . Forexample, in patent ductus the aorta will be en-larged, and in atrial septal defect it will be small

VOLUME 15, JANUARY 1965

Bedside Diagnosis of Heart Disease 83

F,c . 2 . Clubbing of toes, but not of the hands, in a patientwith patent ductus arteriosus and reversed shunt . (Re-produced from Clinical Disorders of the Pulmonary Cir-culation by permission of the publishers, J. & A. Church-ill Ltd .%)

and the main pulmonary arteries larger . Re-markably enough, in ventricular septal defect ofthe Eisenmenger type, the heart and lungs mayappear almost normal on the chest radiograph .

TYPES OF PULMONARY STENOSIS

Pulmonary Stenosis with Closed Ventricular Septum :When severe, this lesion gives the physical signsalready outlined . Except in the most extremecases, the sound of pulmonary closure can beheard faintly and late . This is often a valuableclue to the fact that the ventricular septum isclosed (Fig . 3A), though this is not invariable ;when pulmonary stenosis coexists with a ven-tricular septal defect with left to right shunt,pulmonary closure is often quite well heard, al-though delayed. Unless there is a patentforamen ovale or atrial septal defect permittinga right to left shunt, central cyanosis and club-bing are absent, and the patient will not exhibitcentral cyanosis on effort . The electrocardio-gram commonly shows very severe right ven-tricular hypertrophy with inverted T waves inright and central precordial leads . Often thereis a small q wave preceding the large R wave inleads V,R and V, (Fig . 3B) . The radiographusually shows enlargement of the right atrium

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and of the main pulmonary artery with little orno generalized cardiac enlargement unless thecondition is exceptionally severe (Fig. 4) . Alateral view shows enlargement of the rightventricle, however. The lung vasculature isunderfilled only in veryy severe cases ; cases ofmoderate severity often show an apparentlynormal vascular pattern . The arch of the aortais almost invariably left-sided and if right-sided,should strongly suggest the presence of a ven-tricular septal defect .

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Fm. 4 . Posteroanterior teleoradiograph in pulmonary stenosiswith closed ventricular septum . There is enlargementof the right ventricle and right atrium . The mainpulmonary artery is prominent, and the long fieldsoligemic .

Angina pectoris is a symptom of pulmonarystenosis of only the most extreme form, and itsoccurrence in a patient with mild or moderatestenosis should at once arouse the suspicion ofsome other lesion .

Figure 5 shows the electrocardiogram of a boy withsigns of pulmonary stenosis . The ejection murmurwas of unusual intensity in the aortic area and up theright side of the neck. He complained of unequivocalangina of effort . The electrocardiogram shows com-bined right and left ventricular hyperu'ophy, and thiscombination of symptoms and signs suggested thepresence of associated aortic and pulmonary stenosis .

Fmf3B. Electrocardiogram of severepulmonary stenosis with closed ven-tricular septum showing invertedT waves in right and centralprecordial leads, dominant Rwaves preceded by small q wavesin V,R and Vt, dominant R wavein VR and right atrial P wave .



Bedside Diagnosis of Heart Disease

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FIG . 5 . Electrocardiogram of a boy of 12 years with combineddiscrete suboalaar aortic and pulmonary stenosis, showingsigns of right ventricular hypertrophy (dominant R wavein V,R and V I ) and left ventricular hypertrophy (inver-sion of T in V,-V, and normal axis).

Figure 6 shows the results of right and left heart cathe-terization in this patient and indicates a gradient be-tween pulmonary artery and right ventricle, and be-tween aorta and left ventricle . The gradient on theright side is at infundibular level. The phonocardio-gram in the pulmonary area shows that the systolicmurmur starts early and extends up to and past aorticclosure, suggesting that it was produced by the right-sided obstruction . The aortic murmur is shorter .Figure 7 shows right and left ventricular angiocardio-grams indicating the subvalvar pulmonary and aorticstenoses with normal pulmonary and aortic valves .A small ventricular septal defect was present and isindicated in the lateral view of the right ventricularstudy . The contrast medium has passed across theventricular septal defect to outline the aortic valvecusps. It will be seen later that the physical .l signs .and the angiographic appearances are entirely dif-ferent from another condition which may produceoutflow tract gradients in both ventricles-that ofhypertrophic obstructive cardiomyopathy,5 otherwiseknown as idiopathic hypertrophic subaortic stenosis

Attention to the history and the bedsidephysical signs and particularly to the electro-cardiogram in this patient allowed the diagnosisof left ventricular flow tract obstruction, whichwas largely masked by the pulmonary stenosis .

Fro. 6. Same case . Cardiac catheterization data in combined suboalaar aortic and pulmonary stenosis . Top : Withdrawaltrace from pulmonary artery (PA) through infundibulum (inf) to right ventricle (RV). There are two systolic gra-dients below the pulmonary valve . Bottom left: Simultaneous left ventricular (LV) and femoral artery (FA) pressuresshowing systolic gradient . Bottom right: Phonocardiogram at pulmonary area (PA) showing a long systolic ejectionmurmur due to pulmonary stenosis extending past aortic closure in the pulmonary area and a shorter murmur due toaortic stenosis in the aortic area (AA) . Aortic closure is soft and pulmonary closure delayed and soft .

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Failure to appreciate the presence of aorticstenosis at the time of pulmonary valvotomy forpulmonary stenosis can he disastrous .'

Tetralogy of Fallot : The bedside differentia-tion of the tetralogy of Fallot from pulmonarystenosis with closed ventricular septum maypresent difficulties when patients with thetetralogy are without cyanosis at rest or club-bing . The differential clues are the absence ofa markedly augmented a wave in the jugularvenous pulse in the tetralogy, the quieter cardiacimpulse, which usually taps rather than lifts thesternum, and the difference in the murmur .This may be full length but often achieves itsmaximal intensity early in systole and dies awayat aortic closure (Fig . 8) . It never passes theaortic closure sound which is loud, and pul-monary closure is often inaudible. Aorticclosure is well heard, possibly because of theanterior position of the aortic root .

When considerable cyanosis and clubbing arcpresent, the differentiation between severetetralogy of Fallot and pulmonary stenosis withclosed ventricular septum and patent foramenovale may also be difficult. In the closed sep-tum type of pulmonary stenosis, the a wave in

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Fie. 7 . Same case. Right and leftventricular angiocardiograms . (1)Right ventricular injection : systolicfilm . Subvalvar pulmonary stenosisis considerable (arrow) . (2) Rightventricular injection : diastolic film .The aortic valve cusps (arrow) are out-lined by contrast medium . (3) Leftventricular injection : systolic film .Localized subaortic stenosis (arrow) .(4) Left ventricular injection : diastolicfilm .

the jugular venous pulse under these circum-stances is commonly considerably augmented,which is not the case in the tetralogy of Fallot .The auscultalory difference may be very markedwhen the tetralogy is associated with a diminu-tive right ventricular outflow tract and a con-siderable right to left shunt through a large ven-tricular septal defect. The murmur may thenbe soft and short and preceded by an ejectionclick arising from the large aorta, which receivesall or most of the right ventricular flow (Fig . 9) .In patients with the tetralogy and only moderatepulmonary stenosis and little cyanosis, the pul-monary blood flow is reasonably good, andtherefore, the systolic murmur is often loud andlong . Thus a paradoxic situation exists, forwhen the ventricular septum is closed, thelength and intensity of the murmur commonlyparallels the severity of the stenosis . But whenthe ventricular septum is open, the greater theoutflow tract obstruction and the greater theseverity of the pulmonary stenosis, the larger theright to left shunt and the less blood will passthrough the right ventricular obstruction, andtherefore, the shorter and softer will be the mur-mur .


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The electrocardiogram in the tetralogy of Fallotdiffers from that in the severe closed septumpulmonary stenosis. In the tetralogy the degreeof right ventricular hypertrophy is usually lessbecause the right ventricular systolic pressure,which is a major factor governing the electro-cardiographic appearances,' seldom rises aboveleft ventricular pressure . In severe closed-


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septum pulmonary stenosis much higher levelsmay obtain in systole in the right ventricle .Thus the electrocardiogram in the tetralogyusually shows dominant R waves in right pre-cordial leads, often with a slightly slurred up-stroke to the R wave, and no T wave inversionin right or central precordial leads (Fig . 10) .Occasionally the electrocardiographic changesmay be less severe, especially in patients withlittle cyanosis and mild or moderate pulmonarystenosis (Fig . 11) .

These clinical and cardiographic rules may occasionally bebroken. Figure 12A shows the electrocardiogram of apatient who presented the history and clinical signsof tetralogy of Fallot with considerable cyanosis andclubbing . The a wave in the jugular venous pulse,however, was rather larger than usual, although themurmur was quite typical. The very tall R waves inright precordial leads with sharp T wave inversionsuggested that the ventricular septum was closed .At cardiac catheterization (Fig . 12B), the right ven-tricular pressure was higher than the systemic arterialpressure measured at the same time . There was noevidence of aortic stenosis . The ability of the rightventricular pressure to rise higher than the left insystole suggested that the ventricular septum wasimperforate . Angiocardiography, however, showeda classic appearance of the tetralogy with a shuntfrom right ventricle into left ventricle and aorta (Fig .13). A diagnosis of the tetralogy in which the ven-tricular septal defect was closed by the septal cusp ofthe tricuspid valve in late systole'-thus mimickingclosed-septum pulmonary stenosis was made ; itwas thought that the intense cyanosis was partly dueto a right to left shunt through a patent foramenovale. At eardiotomy by Mr . W . P . Cleland thediagnosis was confirmed, but the ventricular septaldefect was found to be smaller than that usually seenin the tetralogy and quite capable of being closed inthe way mentioned during systole. There was also apatent foramen ovale .

Thus, physical signs aided by the results of special

Fte. 9 . Phonacardiogram in tetralogy of Fallot of extreme severity with hypoplastic right ven-tricular outflow tract, approaching pulmonary atresia . Aortic closure (2) alone is seen and thesystolic click (cl) is well marked . There is only a soft, early systolic murmur (SM) .

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investigations indicated that the anatomic and physio-logic situations in this patient were different fromthose usually found in the tetralogy .

Figure 14 shows a typical selective right ventricularangiocardiogram in the tetralogy of Fallot . It in-dicates the anteriorly placed pulmonary artery witha posterior aorta and a well marked crista supraven-tricularis lying above the large ventricular septaldefect . By contrast, Figure 15 shows the angiocar-diogram in a patient with pulmonary stenosis, a largeventricular septal defect and both great vessels arisingfrom the right ventricle (double outlet right ven-tricle) . 10 4 11 Although the clinical syndrome wassimilar, the appearances were quite different from thetetralogy, the pulmonary artery and aorta beingsuperimposed in the lateral view and the aorta arisinganteriorly . No crista supraventricularis can be seen,and in the anteroposterior view, the aortic valve liesmuch higher than usual, a characteristic finding inthis condition .

Double Outlet Right Ventricle with PulmonaryStenosis: Clinical clues to this diagnosis arevery difficult but it may be suggested if aorticclosure is louder in the second than in the fourthintercostal space, which is the reverse of thefindings in the tetralogy . This physical sign,although more impressive in corrected trans-position, was first pointed out to me by Dr .Alexander Nadas of Boston . The diagnosismay also be suspected if the heart is larger than

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would be considered typical of the tetralogy andif the ejection murmur is well conducted into theneck although, as already seen, this may lead oneto be suspicious of aortic stenosis . Cardiaccatheterization in patients with a double outletright ventricle and pulmonary stenosis usuallyshows a large left to right shunt in a cyanoticpatient, which is a combination unusual in thesevere tetralogy .

Pulmonary Stenosis with Endocardial CushionDefect or Undeveloped Right Ventricle : Figure 16shows an electrocardiogram with signs of markedright ventricular hypertrophy in the right pre-cordial leads, but an unusual cardiac axis withdeep negative deflections in the three standardleads . The appearances in leads it, in and VFwould suggest an endocardial cushion type ofseptal defect, and the signs of right ventricularhypertrophy and deep S wave in standard leadi indicate that this might be combined withpulmonary hypertension or pulmonary stenosis .My colleagues and I have occasionally notedthis type of electrocardiogram in patients pre-senting clinically as the tetralogy of Fallot ; wehave found that it may be produced by anendocardial cushion type of ventricular septaldefect with pulmonary stenosis and right to leftinterventricular shunt, or by pulmonary stenosis

Fmc . 10. Electrocardiogramintypi-cal tetralogy of Fallot . There isright axis deviation with domi-nant R waves in right precordialleads and in VR, and deep Swaves in left precordial leads . Twaves are positive in right pre-cordial leads other than V 4R andVt .

FIG . 11 . Electrocardiogram in tetral-ogy of Fallot with moderate pulmo-nary stenosis and cyanosis only oneffort . There is evidence ofonlyslight right ventricular hyper-trophy.


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Fm. 12. A, atypical electrocardiogram in tetralogy of Fallot. The greatly augmented R waves inright precordial leads preceded by small q waves and with inversion of T waves suggest thatthe ventricular septum is closed . In fact, a small ventricular septal defect was found at opera-tion (see text). B, Same case . Cardiac catheterization in tetralogy of Fallot with small ven-tricular septal defect . Top left shows the right atrial (RA) pressure pulse, and top right, thephonocardiogram showing the long systolic ejection murmur . Bottom left shows the gradientbetween right ventricle (RV) and femoral artery (FA), and bottom right, the gradient betweenpulmonary artery (PA) and infundibulum (inf) and body of the right ventricle . IIF = highfrequence. LSE = left sternal edge . MF = medium frequency .

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should be considered . The diagnosis can usu-ally be made by angiocardiography .

ATRIAL SEPTAL DEFECTS

Figure 17 shows two phonocardiograms .That on the left shows an ejection murmur witha widely split second heart sound in the put-

90

FIG . 13 . Same case . Right ventricular angiocardio,grano intetralogy of Fallot and small ventricular septal defectwhich was probably occluded in the latter half of systole(see text)- A, frontal projection shows early filling of theaorta (AO) from the right ventricle (RV) through theventricular septal defect and extreme narrowing of theinfundio i m (1) of the right ventricle, with good sizedpulmonary Nalve (PV) and pulmonary arteries (PA) .

FIG. 14. Typical tetralogy of Fallot . Right ventricularangiocardiogram (lateral view) showing the aorta (A(D)lying behind the pulmonary artery (PA).

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Fm . 13B. Lateral projection shows relative positions of thegreat arteries, early filling of the aorta from the rightventricle, and the narrowed infundibulum . The ven-tricular septal defect is not exactly delineated .

monary area due to a secundum type of atrial septaldefect . That on the right shows the pansystolicmurmur heard at the apex and conducted to theaxilla in mitral incompetence due to a cleftmitral valve in the ostium primurn or endocardialcushion (atrioventricular canal) type of atrialseptal defect . In any patient with the signs ofatrial septal defect careful auscultation in theregion of the apex and the axillaa is essential todetect a murmur of mitral incompetence . Ifthis is present the diagnosis of cndocardial cush-ion or ostium primum type of atrial septaldefect becomes probable . Additional signs arethe presence of left ventricular hypertrophy andpulmonary hypertension at a young age . Thecharacteristic electrocardiogram (Fig . 18) showsleft axis deviation with deep S waves in leads n,in and VF with partial bundle branch block['and sometimes a long P-K interval.'' My col-league, Dr . Celia Oakley," has produced evi-dence that the presence of a small q wave in leftprecordial leads with left ventricular hyper-trophy in this type of atrial septal defect mayindicate the presence of substantial mitral in-



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Fm. 16 . Endocardial cushion defectand pulmonary stenosis . Electro-cardiogram showing signs ofright ventricular hypertrophywith deep S waves in the threestandard leads and left precordialleads consistent with an atrio-ventricular cushion type of septaldefect and pulmonary stenosis .

Fta. 17. Phonocardingram in atrialseptal defect . Left : Secundum typeof atrial septal defect showingpulmonary ejection murmur andwidely split second sound at leftsternal edge (LSE) . A = aorticvalve closure. P = pulmonaryvalve closure. Right : Atrio-ventricular canal type of defect show-ing pansystolic murmur due tomitral incompetence at the apex .


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BFIG . 19 . Left ventricular-right atrial shunt . A, posteroanteior teleoradiograph of a patient with a ventricular septal de-fect shunting into the right atrium (preoperative film) . The heart is very large and the lung fields slightly pleonemic .B, left ventricular angiocardiogram (frontal projection) . The left ventricle (IN) and aorta (AO) are filled with con-trast medium which has passed across the ventricular septal defect into the right atrium (RA) .

competence or a ventricular component to thedefect . There is a tendency for mongolism tobe associated with atrioventricular canal of thistype and for bacterial endocarditis to occur,which, of course, is exceptionally rare or un-known in the secundum type of atrial septaldefect .

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FIG. 18 . Electrocardiogram of enda-cardial cushion type of atrial septaldefect showing left axis deviation,partial right bundle branchblock, and a long P-R interval(0 .24 sec .) .

VENTRICULAR SEPTAL DEFECTSLeft Ventricular-Right Atrial Shunt . The type

of ventricular septal defect which shunts directlyfrom left ventricle to right atrium 15 may presentsigns somewhat similar to those of an endocardialcushion defect with mitral incompetence .Thus, the pansystolic murmur of the ventricular


FIG . 20. A boy 11 years of age with a large ventricularseptal defect . Note the poor physique and bulgingsternum .

septal defect may be heard outside the leftsternal edge and be conducted toward the apex .The second heart sound is widely split, and thex-ray film usually indicates a prominent rightatrium and may show a larger cardiac contourthan would he expected from the clinical signsor assessment by catheter of the size of the left toright shunt . Figure 19A shows a posteroan-terior radiograph of a boy with this type ofventricular septal defect, the size of the heartbeing larger than a ratio of pulmonary to sys-temic flow of only 2 :1 would suggest . Figure19B shows the left ventricular angiocardiogramindicating the passage of contrast mediumthrough the ventricular septal defect into theright atrium .

In patients with a large defect in the ven-tricular septum, the history in the first year oflife is well known . 1 e Infants tend to be difficultto feed, fail to thrive and to gain weight, andmay be the victims of congestive heart failureand severe respiratory infections . Some ofthese infants die rapidly in the first days or



FIG. 21 . Systolic murmurs in patients with ventricularsepta] defects and varying degrees of pulmonary vasculardisorder (see text) . Numbers indicate pulmonaryarteriolar resistance in units . (Courtesy of Dr . A .Hollman . )

weeks of life from heart failure and pulmonaryinfection.

Ventricular Septal Defect with Pulmonary Hyper-tension : When babies with large ventricularseptal defects reach childhood, they often havesevere pulmonary hypertension due largely toexcessive pulmonary blood flow and also oftento an increased pulmonary vascular resistance .Such a child has a characteristic physical habitus .He is undersized, gains weight slowly, is skinnyand appears to be malnourished ." He oftenhas a bulging sternum thought to be due to stifflungs as a result of the excessive pulmonary bloodflow and the increased arterial pressure ."

Figure 20 shows the characteristic physicalhabitus in a boy of 11 years with a large ven-tricular septal defect and an elevated pulmonaryvascular resistance . Such patients with ven-tricular septal defect seldom have cyanosis, andthe presence of a definite right to left shuntclinically may suggest that transposition of thegreat arteries is also present.

The detection of this type of sternal bulge is of con-siderable importance in diagnosis . It does notoccur in the Eisenmenger type of ventricularseptal defect, where pulmonary hypertension isextreme and a left to right shunt small or absent .Neither does it occur in patients with small ven-

94

Fie. 22 . Phonocardiograms and diagrammatic representa-tions of early diastolic murmur which may be heard tothe left of the sternum in ventricular septal defect (VSD)(see text). 1 = first heart sond . 2 = second heartsound . (Courtesy of Dr . A .Hollman .)

tricular septal defects who have little or nopulmonary hypertension and a modest left toright shunt. It is unusual in uncomplicatedatrial septal defect, although it may occur in theatrioventricular canal type of defect with con-siderable pulmonary hypertension at an earlyage. A bulging sternum is exceptional in thetetralogy of Fallot, in other forms of pulmonarystenosis and in aortic stenosis ; so its detectionis of considerable diagnostic value .

The presence of an elevated pulmonary vascularresistance in ventricular septal defect may besuspected from the character of the accompany-ing murmur, which tends to become short andejection in type as the pulmonary vascular resist-ance rises and the left to right shunt is cur-tailed ." These signs are found in conjunctionwith accentuation of pulmonary valve closure,and enlargement of the right ventricle bothclinically and electrocardiographically . Thelength of the murmur and thrill, however, is one

Goodwin

of the best guides to the presence of severe pul-monary vascular disease in ventricular septaldefect, as my colleague Dr . Arthur Hollman,has shown. Figure 21 shows a series of phono-cardiograms in patients with ventricular septaldefect and different levels of pulmonary vascularresistance . The inverse relation of the lengthof the murmur to the pulmonary vascular resist-ance is well demonstrated .

Associated Lesions : Many associated lesionsare found in conjunction with ventricular septaldefect: patent ductus arteriosus, pulmonaryincompetence, aortic incompetence, variousforms of transposition, mitral incompetence,common ventricle, and multiple ventricularseptal defects being the most important condi-tions 20 Pulmonary stenosis due either to in-fundibular obstruction or to organic obstructionof the pulmonary valve is also not uncommon,and occasionally stenosis of a pulmonary arterybranch may occur . When an early diastolicmurmur is heard to the left of the sternum in apatient with ventricular septal defect, patentductus arteriosus, pulmonary incompetence, oraortic incompetence should be considered .When a patent ductus is present, it carries littleleft to right shunt, and the classic continuousmurmur is invariably absent, although it may berepresented by a soft short high-pitched diastolicmurmur at the left sternal edge . An earlydiastolic murmur which is also heard loudly inthe aortic area suggests that aortic incompetenceis present, and a quick rising, large volume pulseharmonizes with this diagnosis . This type ofpulse does not tend to occur if the ductus ispatent, because the ductus is carrying littleshunt, and there is therefore no 'run off' fromthe aorta to pulmonary artery which wouldproduce the characteristic pulse. Presence ofan early diastolic murmur in the pulmonaryarea, increasing on inspiration, is suggestive ofpulmonary incompetence and usually indicatesthe presence of severe pulmonary hypertension,although previous bacterial endocarditis mayalso he a cause in ventricular septal defect .Figure 22 shows various types of early diastolicmurmurs in ventricular septal defect .

Corrected transposition of the great arteries maypose a considerable problem when associatedwith ventricular septal defect ." ,21 Clinically itmay be difficult to diagnose, but the presence ofestablished or paroxysmal complete heart blockstrongly suggests the condition. If the ven-tricular rate is found to be unusually slow in apatient with the signs of ventricular septal de-


VOLUME. 15, JANUARY 1965

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feet, and if in addition there are cannon wavesin the jugular venous pulse, a diagnosis of com-plete heart block should be made, and correctedtransposition should be strongly suspected .Radiologically the heart may show a narrowvascular pedicle with convex left border . Theelectrocardiogram, however, is usually moresuggestive, since there are large Q waves in rightprecordial leads and no q waves in left precordialleads and often complete heart block (Fig . 23) .The diagnosis is definitively made by angiocar-diography, which shows a characteristic mor-phology of the left ventricle which resemblesthe right, and vice versa, a centrally placedpulmonary artery arising from the right ven-tricle (Fig . 24) and a laterally placed aortaarising from the left ventricle. In the lateralprojection the aorta lies anterior to the pul-monary artery .

The presence of a continuous murmur in ventricularseptal defect suggests a fistula between the aortaand pulmonary artery or right side of the heart(such as a ruptured sinus of Valsalva), or ifheard high up to the right of the sternum andaxilla or back of them, stenosis of the branchesor main branch of the right pulmonary artery .

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M.R . Post-op .Dexlrocardio-limb s chest leads reversed .

Fto. 23 . Electrocardiograms in 8 cases of corrected transposition of the great arteriesshowing q waves in right precordial leads (case MR shows the most strikingchanges).

FIG . 24 Right ventricular ongiocardiogram in corrected trans-position of the great vessels . Frontal projection showing,smooth walled "right" ventricle (RV) and central pul-monary artery (PA).

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APEX

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Fta. 25 . Phonoeardiogram illustrating the systolic mummr incongenital valvar aortic stenosis . The murmur is maximalin mid-systole and terminates before the second sound,which is single . Note that the first sound is soft and theclick is audible at the apex . 1 = first heart sound, 2 =second heart sound, cl = systolic click.)

When this is combined with severe pulmonaryhypertension, flow occurs across the stenosisthroughout the entire cardiac cycle, giving riseto the continuous murmur .

AORTIC STENOSIs

In congenital aortic stenosis it is well knownthat assessment of severity may be exceedinglydifficult R2 A good history of anginal pain orfainting attacks is evidence of severe and danger-ous stenosis and should be regarded as an emer-gency requiring early surgical relief to preventthe tragedy of sudden death. The absence ofsymptoms does not, however, exclude severe

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stenosis. The classic ejection murmur is heardat the aortic area and at the apex and is con-ducted up the right side of the neck . Themurmur never extends beyond aortic closure,often dies away before aortic closure, and itslength is a rough guide to severity, for the longerthe murmur the more severe is the stenosis .The intensity of the murmur, however, is oflittle value, and a loud murmur with thrill maybe present with trivial stenosis . The severity ofstenosis is judged on auscultation by the pres-ence of a fourth heart sound heard at the apexand by late maximal intensity of the murmur .The presence of a systolic click indicates that thestenosis is valvar in site, whereas its absence inchildren (but not in adults with a calcifiedvalve) makes subvalvar stenosis very likely . Asoft, early diastolic murmur of aortic incom-petence is quite consistent with valvar stenosisbut when loud, usually indicates subvalvarstenosis . Thus, a loud diastolic murmur with-out a click almost certainly indicates the pres-ence of localized subvalvar stenosis .23 Aorticclosure is usually soft . 23 a As will be seen later,the diffuse type of subaortic stenosis or hypertro-phic obstructive cardiomyopathy rarely if everproduces an early diastolic murmur .

Figure 25 shows the murinurs in a patientwith valvar aortic stenosis of congenital origin .It is important to realize that in severe aorticstenosis in children, although the left ventricle is

Fm. 26 . Physical signs in hypertrophic obstructive cardiomyopathy. P = percussion wave, T ="tidal wave," D = "dicrotic wave," a = a wave, and AA = aortic area. (Reproduced bypermission of the Editor, British Medical Journal .")


often enlarged clinically and radiologically,this may not be readily apparent . Neither isthe electrocardiogram necessarily of value, forsevere stenosis can exist in the presence of anormal electrocardiogram- Therefore, eventrivial signs of left ventricular hypertrophy are ofvalue in assessing severity, and the presence ofmarked left ventricular preponderance of pres-sure-load pattern, with deeply inverted T wavesin left precordial leads, invariably indicatesvery severe disease .

CARDIOMYOPATHY

In hypertrophic obstructive cardiomyopatlty thehistory commonly reveals a familial incidence .'The symptoms are very similar to those of aorticstenosis : syncope, angina and often dyspneaon exertion . Likewise, sudden death is notuncommon . The physical signs, however, areentirely different from aortic valve stenosis ordiscrete subvalvar stenosis ; the characteristicjerky arterial pulse of hypertrophic obstructivecardiomyopathy is particularly important . Thecardiac impulse commonly has a double. form,the first of the two impulses being due often to afourth heart sound, but occasionally a truedouble systolic thrust may be felt . The ejectionmurmur is late in onset and is heard maximallyat the left sternal edge rather than in the aorticarea, neck or apex. The second heart sound isusually single, and an early diastolic murmur isabsent . There may, in addition, be signs ofright ventricular and right atrial involvement,consisting of an exaggerated a wave in thejugular venous pulse, a third heart sound overthe right ventricle and sometimes a tricuspiddiastolic murmur .s. 24 Figure 26 shows thecharacteristic physical signs in this condition .

The electrocardiogram shows enlargement of theleft ventricle, and sometimes of the right also,and often of both atria . Ventricular hyper-trophy, especially of the left side of the heart, ismassive in this disease, and this is reflected by atendency to deep T wave inversion in left pre-cordial leads . The appearances are thus similarto those of severe discrete aortic stenosis, but thetendency to right ventricular, septal and atrialinvolvement is much greater . Occasionally ashort P-R interval may be seen, and sometimesq waves in central chest leads suggesting septalinfarction but due in fact to septal hypertrophymay occur . 5. 25

The angiographic appearances are quite differentfrom those in discrete pulmonary or aorticstenosis . They show the massive ventricular



Fm. 27 . Left ventricular angiocardiogram in hypertrophic ob-structive cardiomyopathy (lateral view) . Left: Systolic film,showing narrowed ventricular cavity (LV) and refluxinto left atrium (LA). Right : Diastolic film showing per-sistent narrowing below the aortic valve .

FIG . 28 . Right ventricular angiocardiagram in hypertrophieobstructive cardiomyopathy . Systolic film showing the out-flow tract of the right ventricle (RV) narrowed by themassive ventricular septum (S) . (Reproduced by per-mission of the Editor, British Medical Journal .)

hypertrophy, the bulging of the ventricularseptum into the right ventricle, and the irregularmasses of muscle encroaching on the cavity ofthe left ventricle and pinching it off well belowthe aortic valve .° Mitral incompetence ispresent . These appearances are illustrated inFigures 27 and 28 .

CONSTRICTIVE PERICARDITIS

Figure 29 illustrates a patient with severeconstrictive pericarditis in a very advancedstage who had refused surgical relief . Themarked ascites can be seen, and the jugularvenous pressure grossly elevated as evidenced bythe prominent external jugular veins . Con-strictive pericarditis should, of course, never beallowed to reach this stage ; this picture of

98

a patient who refused surgery emphasizes theimportance of early diagnosis . Table I lists theimportant clinical signs . The diagnosis even inthe early stages, can be made readily at the bed-side by the characteristic jugular venous pulse,the increase in jugular venous pressure on in-spiration and the pulsus paradoxus . The car-diac impulse is often quiet as are the heartsounds, a third sound being almost invariableand the second sound being usually widely split .

Radiologically, calcium may be seen in thepericardium, and on fluoroscopy the heart com-monly pulsates irregularly, the right border be-ing almost immobile and the left border pulsat-ing well . This irregular pulsation is of muchgreater diagnostic value than the oft stated lackof generalized pulsation, which is in no wayspecific although always suggestive if the heart isnormal in size . Angiocardiography reveals a

TABLE IClinical Findings in Constrictive Pericarditis

RA + R in VAR . Short Q-TcX-rayHeart small or moderately enlargedIrregular pulsationCalcification

J .V .Y . = jugular venous pressure, R.A. = right atrium and L.A . _eh atrium .

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FIG. 29 . A patient with advancedconstrictive pericarditis . Gross as-cites and wasting are evident, andthe jugular venous pressure is sohigh that the external jugularveins are engorged (arrow) .(Courtesy of Dr. A . Hollman.)

thickened pericardium and helps to distinguishthe condition from myocardial or endocardialconstriction, occurring as part of a cardiomyo-pathy (Fig . 30) 26,27

The electrocardiogram (Fig. 31) usually showslow voltage with flat or inverted T waves, andthere is a tendency for a small monophasic Rwave in V,R and for left or right atrial hyper-trophy . The Q-T interval may be normal orshort, and this may be a useful way of differen-tiating constrictive pericarditis from constrictivecardiomyopathy 26

FIG . 30 . Venous angiocardiogram in constrictive pericarditis;lateral projection, showing thickened pericardium (P)and calcification (C) . Contrast medium outlines theinferior vcna cava (IVC), the right atrium (RA), theright ventricle (RV) and the pulmonary artery (PA) .

THE AMERICAN JOURNAL . OF CARDIOLOGY

Sympmists Signs

Dyspnea Small rapid pulse . Low pulse pressure, para-dox

Fatigue Raised LV.P . (may pulsate well) . Rises onDiastolicinspiration .

dipSlight o absent cardiac enlargement . Dia-Cough

stolic thrustFaintness oft heart soundsAbdominal swelling Widely split 2nd soundWasting Triple rhythm due to 3rd sound . (Atria[

fibrillation)HepatomegalyAscites (ankle edema)EGG

tachycardia, low voltage, flat T . LA or

Figure 32 illustrates the hemodynamics inconstrictive pericarditis and cardiac constriction .It shows the characteristic venous pulse oc-curring in both the left and right atria, the in-crease in atrial pressure, the fall of arterial pres-sure on inspiration, and the characteristic earlydiastolic dip in the ventricular pressure pulse .It must be stressed that this type of hemodyna-mic pattern, indicating appreciable constriction,can occur with few symptoms and with no signsof congestive cardiac failure .

LEFT ATRIAL MYXOMA

Finally, I should like to deal with the syn-drome of left atrial myxoma . This conditionshould always be suspected in a patient withatypical signs of mitral valve disease inwhom pulmonary hypertension, dyspnea andhemoptysis are combined with features suggest-ing a general systemic disorder, such as a raisedsedimentation rate, clubbing of the fingers, feverand anemia . Abnormal serum proteins, con-sisting of an increase in the gamma globulinfraction, may also be found, and patients com-monly have lost weight or appear to have somemalignant or toxic condition ." There is usuallyno history of rheumatic fever, and the history ofsymptoms may be relatively short . Syncope onchanging position may occur, and emboli arefrequent although not necessarily of a major



FIG . 32 . Hemodynamic changes in cardiac compression due to constrictive pericarditis . EDD = early diastolic dip(see text) .

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Fio,31 . Electrocardiogram in constrictivepericardicis showinglow voltage, inverted and flat T waves and sinus tachy-cardia with occasional blocked atrial beats . The Pwaves arc full and broad indicating right and left atrialenlargement .

order . Occasionally, however, the patient maypresent with peripheral or even coronary em-bolism, and the condition should always be sus-pected in a young person who presents with myo-cardial infarction but in whom occlusive coro-nary artery disease cannot he conclusivelyproved .

In a personal series of six patients, clubbing ofthe fingers was present in all and was marked in2 . One patient also had cerebral and peri-pheral emboli, and subsequently signs suggestiveof mitral valve disease developed . Contrary to

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TABLE DFeatures of 50 Unpublished Cases of Left Atrial Myxoma

(British Cardiac Society)

Hb. = hemoglobin and E .S.R. = erythrocyte sedimen-tation rate .

(Reproduced by kind permission of the Editor ; TheLancet. so)

the condition of other patients in this series, liedid not have appreciable pulmonary hyperten-sion. Table it shows the incidence of features ofgeneral systemic disease combined with those ofobstruction to the pulmonary circulation in aseries of patients provided by the British CardiacSociety . 21 It will be noted that atrial fibrilla-tion is uncommon . The similarity to bacterialendocarditis involving the mitral valve is ob-vious, and, indeed, many features are commonto both diseases . Personal experience suggests,however, that a patient may have a myxomaif bacterial endocarditis on the mitral valveis suspected and he has not responded to anti-biotics, has no history of rheumatic fever and nosplenomegaly . The finding of other valve le-sions makes the diagnosis of myxoma extremelyunlikely but not impossible . Calcification ofthe mitral valve does not occur, but calcificationin the myxoma may do so and may resemblecalcified thrombus in the left atrium .

The essence of diagnosis lies in suspecting the

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Fm. 33. Left atrial myroma. Pulmonary arteriogram(frontal view) showing large filling defect in left atriumdue to myxoma (M) . The pulmonary vein of the rightlower lobe has not filled with contrast, and the arteriesshow marked tortuosity and narrowing at the bases dueto pulmonary hypertension . (Reproduced by permissionof the Editor of Thorax .")

condition at the bedside, and definitive proofrests with the angiocardiogram . Figure 33shows a pulmonary arteriogram indicating themyxoma as a lobulated filling defect in the leftatrium moving down through the mitral valveto the left ventricle, in association with severepulmonary vascular changes similar to thosefound in mitral stenosis and due to pulmonaryvenous hypertension .

The angiocardiogram was diagnostic in thesepatients, but it was the clinical suspicion by bed-side methods which led to the angiocardiogrambeing performed and the diagnosis being con-firmed .

CONCLUSION

I hope that these few random observationsbased on the personal experience of my col-leagues and me will illustrate the value of thebedside approach to diagnosis .

Perhaps I might venture to conclude by say-ing that the art of medicine possibly consists inthe ability to bring the science of medicine to thebedside .

ACKNOWLEDCMENTSI am indebted to my colleagues for their help and ad-

vice, especially Prof. R . E. Steiner and Drs. A . Holtman,C. M. Oakley, M. Dulake and D . R . Smith .

REFERENCES1 . WocD, P. Diseases of the Heart and Circulation,

p.47. London, 1956. Eyre and Spottiswoode.2. Goonwiw, S. F . In: Clinical Disorders of the

Pulmonary Circulation, p . 74 . Edited by Daley,


FeaturesNo . ofPatients

Obstructive featuresDyspnea on effort 34Dyspnea on effort and paroxysmal

dyspnea 12Pulmonary hypertension 28Mitral systolic murmur 22Mitral diastolic murmur 27Mitral opening snap 12Third heart sound 9Syncope or vertigo 8

Embolic featuresSystemic embolism 18"Pulmonary embolism (or thrombosis) lot

Constitutional featuresFever (99-102 F.) 19Loss of weight 11Anemia (less than 80% Hb .) 15Raised E .S.R. (greater than 6 mm. in

first hour) 26Clubbing of fingers 2Abnormal serum proteins 5 k

Atrial fibrillation 6

* Includes 2 with coronary embolism .t Includes 3 with angina .j Only 16 patients tested.

A., Goodwin, J. F. and Steiner, R . E. London,1960. Churchill.

3. MounsaY, J . P. D. The early diastolic sound ofconstrictive pericarditis . Brit . Heart J., 17 : 143,1955 .

4. WOOD, P. The Eisenmenger syndrome or pulmo-nary hypertension with reversed shunt. Brit.M. J., 2 : 701 and 755, 1958 .

5. COHEN, J ., EFFAT, H., GOODWIN, J . F., OAKLEY,C. M. and STEINER, R . E. Hypetrophic obstruc-tive cardiomyopathy. Brit . Heart J., 26 :16,1964 .

6. BRAUNWALD, E ., MORROW, A . G., CORNELL, W . P .,AYGEN, M. M. and HmntsH, T . F . Idiopathichypertrophic subaortic stenosis . Am . J. Med .,29 : 924, 1960 .

7. NADAB, A. S., VAN DER HAUWERT, L ., HAUCK, A . J .and GROSS, R. E . Combined aortic and pul-monic stenosis . Circulation, 25 : 346, 1962 .

8. GOODWIN, J. F . and ABDIN, Z . H. The cardiogramof congenital and acquired right ventricularhypertrophy . Brit . Heart J ., 21 : 523, 1959 .

9. HOFFMAN, J . I . E., RUDOLPH, A. M., NADAB, A . S .and GROSS, R . E. Pulmonic stenosis, ventricularseptal defect, and right ventricular pressureabove systemic level . Circulation, 22: 405, 1960-

10. MORGAN, J. . J., PITMAN, R., GOODWIN, J . F .,STEINER, R . E. and HOLLMAN, A . Anomalies ofthe pulmonary artery and aorta complicating ven-tricular septal defect. Brit. Heart J., 24 : 279,1962 .

11. NEUFELD, H . N . et al . Origin of both great vesselsfrom the right ventricle without pulmonarystenosis . Brit. Heart J., 24 : 393, 1962 .

12. NADAB, A . S. Pediatric Cardiology, p . 293 . Phila-delphia, 1963. W. B. Saunders Co .

13 GOODWIN, J . F . In Ref. 2, p. 18 .14. OMERI, M ., BISHOP, M . B., OAKLEY, C . M., BEN-

TALL, H . B. and CLELAND, W . P. The mitralvalve in endocardial cushion defects. Brit . HeartJ., (in press) .

15. GERBODE, F ., HULTGRFx, H., MELROSE, D. andOSBORN, J . Syndrome of left ventricular-rightatrial shunt . Ann. Surg ., 148 : 433, 1958 .



16. KEITH, J . D., Rows, R. D. and VLAD, P . HeartDisease in Infancy and Childhood, p . 238. NewYork, 1958. MacMillan.

17. CLELAND, W. P. et al . The treatment of ven-tricular septal defect . Brit. M. J., 2 : 1369, 1958 .

18. DAVIES, H., WILLIAMS, J. and WOOD, P . Lungstiffness in states of abnormal pulmonary bloodflow and pressure . Brit. Heart J., 24: 129, 1962.

19 . HOLLMAN, A., MORGAN, J. J., GOODWIN, J . F . andFIELDS, H . Auscultation and phonocardiogra-phic findings in ventricular septal defect . Circula-tion, 28 : 94, 1963 .

20. GOODWIN, J. F . Ventricular septal defect . Roc.Roy . Soc. Med., 54 : 783, 1961 .

21 . ANDERSON, R. E ., LILLIHEI, C. W. and LESTER, R . G.Corrected transposition of the great vessels of theheart . A review of 17 cases . Pediatrics, 20 : 626,1957 .

22. BRAUNWALD, E., GOLDBLATT, A ., AYGEN, M. M.,ROCKOFF, D. and MORROW, A. G. Congenitalaortic stenosis . I . Clinical and hemodynamicfindings in 100 patients . Circulation, 27 : 426, 1963 .

23. LEES, M. H., HAUCK, A. J., STARKEY, G . W. N .,NADAS, A. S. and GROSS, R. E . Congenitalaortic stenosis ; operative indications and surgicalresults . Brit. Heart J., 24 : 31, 1962 .

23a. OAKLEY, C. M. Personal communication .24. GOODWIN, J . F., HOLLMAN, A ., CLELAND, W . P . and

TEARS, D . Obstructive cardiomyopathy simu-lating aortic stenosis . Brit. Heart J., 22 : 403,1960 .

25. HOLLISTER, R. M. and GOODWIN, J . F. The elec-trocardiogram in cardiomyopathy . Brit . Heart J.,25 : 357, 1963 .

26. BRIODEN, W. The non-coronary cardiomyopathies .Lancet, 2 : 1179 and 1243, 1957 .

27. GOODWIN, J. F ., GORDON, H., HOLLMAN, A. andBISHOP, M . B . Clinical aspects of cardiomyo-pathy. Brit. M. J., 1 : 69, 1961 .

28. GOODWIN, J . F . et al . Clinical features of left atrialmyxoma . Thorax, 17 : 91, 1962.

29. GOODWIN, J. F . Diagnosis of left atrial myxoma .Lancet, 1 : 464, 1963.