Angiocardiographic studies of valvular malformations in pulmonary stenosis: Relationship to...

Angiocardiographic Studies of Valvular

Malformations in Pulmonary Stenosis

Relationship to Physiologic Alterations*

EDUARDO COELHO, M.D., EDCARDO DE P.41v.4, ~L.u., A~ERICO NUNES, M.D. and \V~~:~~r~ TAVARES, M.D.

I&bon, Portugal

L IKE OTHERS,~ we have found that the clinical picture varies in pulmonary stenosis with

intact ventricular septum. When the pulmonary circulation is normal or almost normal, the condition is well tolerated; when the pulmonary circulation is very deficient, clinical complaints are clearly present. Between these two extremes are intermediate conditions de- pending on the degree and type of stenosis, but we have not found a close correlation between the right ventricular-pulmonary artery’ pressure gradient and the decrease in pulmonary circulation or the severity of the clinical signs.

On the other hand, the anatomic alterations of the valves demonstrated by angiocardiography also vary, although on the basis of angiocardiography some pathologists,’ radiologists+” and clinician@ have described only. one type of valvular stenosis -the dome form. Heim de Balsac et al.7 found only one anatomic form of valvular stenosis, the conic diaphragm form, but described various kinds of orificial stenosis produced by fusion of the cusps. Such fusion mav be complctc or partial. This small orifice varies in appearance, position and size and is generally central but sometimes eccentric. It may be circular, oval or, more rarely, star- shaped or a simple slit. The diaphragm may have the thinness and mobility of normal valves or may be thickened, fibrous or irregular. These authors presented no angiocardiographic data on these points.

Although the severity of the hcmody-namic changes depends on the degree of stenosis. each type of valvular deformity may affect the anatomic and physiologic disturbances at the level of the trunk of the pulmonary- artery and peripheral pulmonary circulation and, in turn, may

affect the severity of the clinical symptoms. It is this aspect of pulmonary stenosis with intact ventricular septum that we have studied.

MATERIAL AND METHODS

We performed angiocardiography in 29 patients who had pulmonary valvular stenosis with intact ventricular septum. In all of them we performed cardiac- catheterizations and selective angiocardiography as previously described.8 We also recorded phonocardiograms using the Elema Klinik calibrated phonocardiograph, 12 lead electrocardiograms with supplementary right precordial leads and vector- cardiograms.

RESULTS

W’e found five different angiocardiographic types of deformity of the pulmonary valves :

I. Dome-shaped with good mobility of the valvular plane (12 cases) ; 2 patients had atria1 septal defect ; 1, patent ductus arteriosus. In 5 patients there was a “corkscrew” or spiral movement of the contrast substance while passing through the infundibulum during sys- tolr. This dome form corresponds to fusion of the cusps with the orifice, either in the center or at the periphery.

II. Diaphragm-shaped with the valvular plane less elevated than in the previous form (4 cases).

III. Stenosis from valvular deformation caused by irregular fusion of the cusps, giving rise to a winding trajectory of the contrast medium (5 cases).

IV. Stenosis caused by incomplete opening of the cusps (6 cases); 1 was associated with coarctation of the aorta and 1 with aortic stenosis.

v. Subvalvular stenosis (2 cases).

* From the Center of Cardiology of the Instituto dr Alta Cultura, Faculty of Medicine, Lisbon, Portugal.

634 THE AMERICAN JOURNAL OF CARDIOLOGY

Pulmonary Stenosis

ILLUSTRATIVE CASES

T__JPP I. Dome-form Stenosis: The 5 cases showing corkscrew movement of the contrast substance through the infundibulum during systole have special interest angiocardiographi- tally.

CASE 1. Different phases of opacification of the infundibulum were seen in successive angiocardiographic exposures (Fig. 1A and B). The contrast substance showed how the blood stream is modified in overcoming the resistance offered by the dome- form stenosis with central orifice. The third and fourth exposures depicted clearly the jet, its direction and the poststenotic dilatation in the region of the pulmonary artery where the jet impinges (Fig. 1A). The contrast substance did not completely outline the ventricular cavity because it was partly occupied by the mass of papillary muscles and trabeculae resulting from the marked hypertrophy of the ventricular walls. The thickness of the cusps and good mobility of the valvular plane were evident. The eighth

exposure in the anteroposterior plane (Fig. 1C) showed the narrowing of the infundibulum, post-

NOVEMBER 1962

stenotic dilatation and paucity of pulmonary vascula- ture.

During operation the surgeon confirmed the absence of infundibular stenosis. The pressure in the right ventricle was 150/O, the pressure in the pulmonary artery 13/7 and the gradient 137 mm. Hg.

CASE 2. In the third angiocardiographic exposure, the opacified blood stream was seen taking on a corkscrew form as a result of the resistance presented by the dome-form vavular stenosis. The jet was central and impinged on the wall of the pulmonary artery, provoking the poststenotic dilatation. The tenth exposure (lateral plane) and eleventh (posteroanterior) showed the direction of the jet. The eleventh exposure showed clearly the valvular stenosis and poststenotic dilatation. The ventricular cavity appeared incompletely filled, as a result of inward bulging of the papillary muscles and chordae tendi- nosae in consequence of the marked hypertrophy of the walls. In the eleventh exposure (posteroanterior plane), the valvular stenosis was superimposed on the poststenotic dilatation.

The pressure in the right ventricle was 78,‘5 and in the pulmonary artery 18/12 mm. Hg (gradient 60), and the pulmonary circulation was slightly diminished. After valvulotomy the surgeon established by digital exploraiion that there was no infundibular stenosis.

FIG. 2. ‘ryp“ 1. Chsc 3. .Tct passing through central ori lice- in dome form and “corkscrewing” of infundibulum (lateral position i.

CASE 3. .lhis case had an associated patent ductus arteriosus opening into the trunk of the pulmonary artery. There was no poststenotic dilatation, pos- sibly because the How of the jet was neutralized by the stream from the patent ductus arteriosus (left-to- rightshunt). ‘l’hc patent ductus arteriosus was shown very clearly by the increase and decrease in opacity of the trunk of the pulmonary artery according to the phase of the cardiac cycle, a sign previously described.’ We also noted the second indirect sign of patent ductus-the reopacification of the trunk of the pulmonary artcry during the Ievocardiogram. In tht fourth exposure (lateral plane) the dome form of the stenosis, the central orifice and corkscrewing in the infundibulum were clearly seen (Fig. 2). Pressure in the right ventricle was 4818 and in the pulmonary artery 16/10, with a gradient of 32 mm. Hg. In spite of a low pulmonary systolic pressure, the patent ductus arteriosus permitted good pulmonary circulation.

CASE 4. ‘l’he eleventh exposure (lateral plane) during systole demonstrated clearly the dome-shaped stenosis with the jet a little eccentric anteriorly, causing poststenotic dilatation (Fig. 3, top). This exposure also clearly defined the narrowed infundibulum and supraventricular crest and the corkscrewing in the infundibulum during ejection of blood. The four-

Fro. 3. Type I, Case 4. Top, anterior position of jet and therefore of valvular orifice causing poststenotic dilatation (lateral position). Rotlom, valvular plane in systole (lateral position).

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Pulmonary Stenosis 637

tetnth exposure clearly showed the valvular plane in diastole (Fig. 3, bottom).

When we compared the two exposures, systolic and diastolic, we concluded that there was relatively good mobility of the valvular plane (about 3 mm.). This fact was manifested in the phonocardiograms by an earlv svstolic click.gV*O Pressure in the right ventricle , was 114 ‘4 and in the pulmonary artery, 18 ‘11 mm.

Hg.

C.&E 5. ‘The pulmonary valvular stenosis was associated with an atria1 septal defect. During systole corkscrewing was seen only in the initial part of the infundibulum. The sixth and seventh exposures (lateral plane) clearly showed the dome appearance of the stenosis and the direction of the jet giving rise to the poststenotic dilatation.

‘The hemodynamic study yielded the following pressures: right ventricle 48/8, pulmonary artery 16110, systolic gradient 32 mm. Hg. The pulmonary circulation was almost normal. Hypervolemia from the atria1 septal defect with left-to-right shunt was com- pensated for by the stenosis.

In the remaining 7 cases of type I deformity

there was no corkscrewing in the infundibulum

during systole, but there was poststenotic dilata-

tion because the jet was directed against the

anterior wall of the pulmonary artery. Pulmo-

nary pressure was low and pulmonary circula-

tion poor.

TYJ)Y II. Diaphragm Form: This type differs

morphologically from type I in that the valvular

membrane is not dome-shaped but flattened,

and during systole slightly convex. There is

still some mobility of the membrane, but less

than in type I. In all cases we noted post-

stenotic dilatation. The more anterior the jet, and, therefore, the more anterior the valvular orifice, the more striking was the poststenotic dilatation. In this diaphragm type, as in type I, the orifice may be central or peripheral and anterior, and the form of the poststenotic dilatation varies accordingly. It is either diffuse or in the form of a dromedary’s hump, according to the position of the orifice and whether the jet is central or anterior. Of the 4 cases in this

group, 2 had pulmonary pressures of 18 and 22 mm. Hg, respectively, and only slightly reduced pulmonary circulation. The second exposure (lateral and posterior plane) of one case showed the typical angiocardiographic morphology of this type of anomaly (Fig. 4).

‘~J~IP III. I’alvular Deformation: In some cases, although the valvular membrane is neither dome- nor diaphragm-shaped, the deformation still causes the jet to impinge against the wall of the pulmonary artery and give rise to dilatation

NOVEMBER 1962

FIG. 4. Type II. Diaphragm form, with central jet (lateral position).

during systole. This dilatation may be fusi- form. During diastole the dilatation of the trunk of the artery is not so noticeable (Fig. 5A).

The valvular deformation in this case was very clear in these exposures (Fig. 5A and B). The ventricular pressure was 4017, pulmonary pressure 17/7 and systolic gradient 23 mm. Hg. In the clinical his- tory there was readily induced fatigue. The fifth and sixth exposures of another case, the first taken in systole, the second in diastole (lateral plane:, showed the strange form taken by the valvular plane through the deformation of the cusps (Fig. 6.4 and B). In one case there was an increase in the caliber of the branches of bifurcation of the pulmonary artery as far as the second order branches. Hypercirculation was present in the peripheral portion of the lungs; the gradient was 160 mm. Hg (175 ‘10 to 15/‘2 mm. Hg). In another case the gradient was 14 mm. Hg (3213 to 18/11 mm. Hg). This patient had normal pulmonary circulation and did not complain of fatigue.

Ty#e Iv. Incomplete Opening of tt11 17alves:

Comparison of the exposures in systole and

diastole indicated that there was good mobility

of the valvular plane. In 5 of the 6 cases there

(Ioelho et al.

FIG

B, (

FIG. 6. I’ypc 11). .I, strange form of the valvular plant tiu~ to drformation of the valves (lateral position). B

asPe :ct of the valvular plane (lateral position).

wa: ; a certain degree of dilatation of the pulmo-

nar y artery but not poststenotic dilatation,

sine :e the anatomic alterations of the L-alvcs do

not alter the form and direction of the jet in

sucl 1 a way as to give rise to this. The sixth

cast : did show poststenotic dilatation.

In this case (Case 6) the stenosis was clearly evident

in exposures 7 (systole) and 9 (diastole) by inc omplete

opening of the valves, the direction of the jet and the

poststenotic dilatation; in exposure 12 (an .teropos-

terior plane) normal pulmonary circulation I ,4as seen

(Fig. 7A and B). ‘I-his agreed with the hemoc lynamic

. T -ing

xxition).

, another

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FIG. 7. Type tv, Case 6. A, incomplete opening of the valves during systole (lateral position). of the valves during diastole.

B, incomplete opening The poststenotic dilatation is more evident (lateral position).

A B C

FIG. 8. Type v, Case 2. A, double valvular and subvalvular stenosis (lateral position). B, close proximity of calvular and subvalvular stenosis (lateral position). C, the direction of the jet is parallel to the walls of the pulmonary artery. There is no poststenotic dilatation (lateral position).

examination. Pressures were right ventricle 44/4, pulmonary artery 25/l 5, and systolic gradient 19 mm. Hg. In all 6 cases of this group except one with a gradient of 52 mm. Hg, the pulmonary pressure was 20 mm. Hg or higher, the pulmonary circulation almost normal and the clinical signs very slight.

Type v. Suhcalvular Form: There were two

cases in this group.

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CASE 1. In the first set of angiocardiograms the catheter was introduced into the saphenous vein and entered the left atrium via a patent foramen ovale. The exposures showed the left side of the heart very clearly. The second set of exposures was not so clear, but in the seventh and ninth (lateral plane), a break in the contrast substance below the valvular plane was seen. This seemed to be due to a subvalvular diaphragm. In this type of malformation there is

640 C:oelllo et al.

usually no pulmonary artery dilatation. ‘l‘he gradient

of 13 mm. Hq (26 10 to 13 7 mm. Hg) also shows

that this is a mild case.

C:AsK 2. ‘I’his case was of greater inleresl. ‘l‘he

sixth and sew nth exposmw (lateral plane) in diastole

and syatole, respectively, showed thv aspects ol’ a

double valvular and subvalvular stenosis (Fig. 8.A).

‘Thr region bc.t\vcac n the two formations did not l’unc-

lion as a third vcntriclc brcausc thr subvalvular

sttmosis was very close to the valws (l!ig. SB). ‘rhe

fourth rxposuw showrd that the orifice: \~as wwntric-

and the jrt diwctcd parallel to the walls of the> artcr)

so that thcrc \vas no poststcnotir dilatation (I;ig. SC:). Hemodynamic txamination gave thr follwving

pressurrs : right ventrick 51 2, pulmonar! artty

18 8 and systolic gradirnt 33 mm. Hg. I’hr, pul-

monary circ~kttion \~as almoat normal. ‘lhr stud\ of

thr pulmon;uy and ventricular prcssurc curve did

not Icad to the diagnosis of the rubvalvular corn-

poncnt oC the stcnoais since thrrc was a sharp tmnsi-

tion a~ soon as thy cathf>ter crowd the valvular plant.

(:o\lhlEl\i I‘S

The anatomic form uf the valvular malfor-

mation infiuenccs the dilatation of thr pulmo-

nary artq and the dcgrec of stenosis. .An+o- cardiography (and plain radiography) in 14 cases showed pronounced poststenotic dilatation of the pulmonary artery. This occurred in the first four groups and was predominant in the second and third group : type I, 5 cases out of 12 : type II, 4 out of 4: type III, 4 out of 5: and type Iv, 1 out of 6,

The systolic pressure in the right \-cntriclc varied between 200 and 32 mm. Hg, and systolic pressure in the pulmonary artery varied between 29 and 10 mm. Hg. The gradient between systolic ventricular and systolic pulmonary pressure varied between 178 ;nd 14 mm.

Hg. The pressures were highest in t!-pes I, II and III. In 3 cases in the first two groups, however, the systolic pressure in the right ventricle \cas only- 43, 48 alid 40 mm. f-17 and in 2 cases in the third group. 40 and 32 mm. Hq. Slight pulmonary stenosis appeared more frr- quently in the last two groups. The 5).stolic prcssurc graclicnt l)ct\vceIl right ventricle and pulmonar)- artcry t)! itself clot not qivc com- plete information as to Ihr se\.c.ril). of stenosis or extent of the ph)-sioloqic altcration~. The study of thcscl 19 casts sho\vs that patients \vith a high gradient mav have almost normal pulmonar) artcry prexsurr and litrlc fatigue. whereas those with louver qraclicnth may have poor pulmonary circulation and marked fatigue.

WC found that the pulmonar>- circulation was

normal or only slightly diminished in 11 cases distributed among all the groups. Pulmonar) s>;stolic pressure was 18 mm. Hg or more. The thrcct estimation of the pulmonary blood flow, so important in this condition, was not con- siclcrcd in this stud\-. The level of the pulmonary arterial pressure is an important index of the deqrec of the pulmonary circulation. Its \;illu~~ corrclatcs better with the latter than wit11 rhc qradicnl, \Vhen it is normal or onl\, sliqhtl!- diminished (between 26 and 1X mm. Hai, a high ,gradient corresponds to slight circulatory alterations and clinical si,qns. 011

tllc other Iland, a lower graclicnt with low pres- slmx in lhc pulmonary artcq- (below 18 mm. Hq) ma)- correspond to more marked physiologic alterations and clinical repercussions poor pulmonary circulation and fatigue. \t’c do not include those cases with patent ductus artcriosus or atria1 septal defect and left-to-ri,Tht shunt which do not show diminished pulmonar!, circulation.

Rrlation c!/ Corksmwing Phmomenon, Arssurrs

nncl LS~.c~cdi~~ Mztrmur: The corkscrewing aspect of the infundibulum during systole only appeared with the dome type. How can this alteration in the blood stream as it passes through the infundibulum in some cases of dome-shaped valvular stenosis be explained? On the basis of studies of functional anatom! I))- other authorsgs10 we explain the phenomenon as follows : In the right ventricle the spiral muscltx fibers predominate over the constrictors. The corkscrewing aspect of the infundibulum results from a selective hypertrophy of the spiral fibers (both internal and external) at this level provoked by the blood Jet. The obstacle at the outlrt c,f the ,jet in the dome-form valvular stenosis ma)- cause hypertrophy of the spiral fibers both anterior and exterior to the in- fmlndibulum and give rise to the corkscrew effect.

If we consider the 5 cases of the first group \vith corkscrewing of the infundibulum and examine the pressures and systolic murmur, we find that in 2 of them where the ventricular and pulmonaq- pressures were rcspcctivcl>- 48 i8, 16 ‘10 mm. Hg (gradient 32) and 43 ‘5, 16,‘ll mm. Hq (gradient 37), the murmur was in crcscrndo until mesosystolc and terminated in the aortic component. In one case where the ventricular and pulmonary pressures were 78,,‘5, 18 ‘12 mm. Hg (gradient 60), the crescendo lasted for two thirds of the qtolc and did not go tzvoncl the aortic component. In the remain- ins_ 2 casts lvherc the pressures were respec-

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tively 11414, 18/10 mm. Hg (gradient 96) and 150,/O, 13/7 mm. Hg (gradient 137), the murmur terminated in the pulmonary component. In the first of these cases, however, the crescendo lasted for four fifths of the systole and extended to the pulmonary component without masking the aortic component; in the other, the crescendo lasted until the aortic component which was completely masked and the murmur terminated in decrescendo in the pulmonary component.“~‘” These 5 cases, therefore, correspond to no particular type of phonocardiogram or intracardiac pressures. The ventricular pressure ranged from 43 to 150 mm. Hg, the pulmonary systolic pressures from 13 to 20 mm. H,g and the gradient from 27 to 137 mm.

Hg . Like other authors,‘3-*” we have found no

relationship between the ventricular pressures and the poststenotic dilatation, nor between the pulmonary pressures or the pressure gradient and the poststenotic dilatation.

Corrrlation of Phonocardiogram and Angiocardio-

gram: Whenever these cases had fairly good mobility of the valves (notably in group I), this was shown on the phonocardiogram by the appearance of an early systolic click. This results from the impact of the blood stream against the valves when these still have sufficient mobility rather than against the pulmonary artery.

If we compare the phonocardiogram and the anatomic type of the malformation, we find that it is in groups I, II and III that we encounter the highest ventricular pressures. As the in- tensity and duration of the murmur increase with the rising pressure, these phonocardiographic characteristics predominate in the first three groups. However, we cannot establish any strict correlation between the phonocardiogram and the anatomic type of the malformation.

S~JMMARY

Selective angiocardiographic study of the pulmonary valves in 28 cases of pulmonary stenosis with normal ventricular septum established five anatomic types of stenosis: dome, diaphragm, deformation of valves, incomplete opening of valves and subvalvular.

The anatomic type may influence the degree of dilatation of the pulmonary artery. The poststenotic dilatation of the pulmonary artery is not related to the severity of the stenosis but is related to the direction of the blood jet and,

NOVEMBER J 962

therefore, to the topography of the valvular orifice.

In the angiocardiograms of the first type of valvular deformation, the corkscrew effect during ejection is attributed to hypertrophy- of the spiral muscles of the infundibulum caused by the strong resistance opposed by the valves to the blood flow. This phenomenon occurs onlv in the type lvhere the valves still have fair mobility.

In all the anatomic types, the pulmonary circulation depends more on the le\:el of the systolic pulmonary pressure than on the systolic pressure gradient between the right \rentricle and the pulmonary artery.

The morphology of the valvular mall’orma- tions is a factor to consider in evaluating the hemodynamic changes of pulmonary stenosis.

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12.

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BROCK, R. The Anatomy of Congenital Pulmonary

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GOULD, S. E. Pathology of the Heart, p. 391.

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HEIM DE BALSAC, R., METIAN, C., DURAP, M. and

DUBOST, C. Trait6 des Cardiopathies Congtni-

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13. GREENE, I?. G., BALDM.I.\I, E. DI: F., HIMMELSTEIN, .\., artery in pulmonary stenosis. Circulnlion, 13 : 719, ROH, C. and COURN.~D, A. Pure congenital 1956. pulmonary stenosis and idiopathic dilatation of 16. Voca~.eo~~., I,., SISRIRE, V., NELLEN, M. and GOETZ, thr pulmonary artrry. .,im. .f. M?d.. 6 : 24, 1949. K. .2. ‘l‘he differentiation of tetralogy of Fallot

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