Circulatory Dynamics in Osteitis Deformans

By J. LEQUTIME, M.D. AND H. DENOLIN, M.D.

In Paget's disease the peripheral blood flow is increased in the bones affected by the disease.Nevertheless the circulatory dynamics of the patients studied at rest are usually not modifiedsignificantly. But during exercise, an abnormal augmentation of the cardiac output in regard to themetabolic needs is observed. These findings are similar to those the authors have observed inpatients suffering from systemic arteriovenous aneurysms.

IN 1945, Edholm, Howarth and McMi-chael4 showed for the first time that theperipheral blood flow was considerably in-

creased in limbs, the bones of which mere af-fected by active osteitis deformans. On thisoccasion, they reported the findings in a pa-tient suffering from both congestive heartfailure and Paget's disease, in whom theyfound a very high cardiac output: 13.3 litersper minute. They suggested that the increasein the blood flow in the bones was responsiblefor the high-output state. Since then, Paget'sdisease has been placed among the clinicalconditions which lead to congestive heartfailure accompanied by high cardiac output,the other conditions being systemic arterio-venous aneurysm, beri-beri, severe anemia,hyperthyroidism and emphysema. 8 11

Edholm, Howarth and McMichael believedthat osteitis deformans could give rise to con-ditions similar to those created by systemicarteriovenous aneurysms and that it is prob-able that direct, communications between ar-teries and veins exist in the bones of patientswith Paget's disease. Though the existence ofsuch fistulas have not been certainly demon-strated,2 Rutishauser, Veyrat and Rouiller10have clearly shown histologically that thereis an extremely important increase in the vas-cularity of the diseased bones. This consider-able augmentation in periosteal vascularity hasbeen also demonstrated in vivo and post-mor-tem by the injection of radio-opaque ma-terial.3' 9, 12 Moreover, an important increase in

From the Department of Internal Medicine andthe Department of Cardiology of St. Peter's Hos-pital, University of Brussels.

Presented before the Second World Congress ofCardiology, Washington, D. C., September 1954.

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the size of the vessels forming the periostealplexus has been demonstrated by dissection.3Recently, Howarth5 has called attention to thepresence of a high cardiac output in 5 out of13 patients with Paget's disease. With the ex-ception of one case in which the cardiac out-put reached 13.3 liters, the increase of outputobserved was slight, in particular if one keepsthe fact in mind that the observations on thesepatients were not made under basal conditions.Howarth stated that involvement of 35 percent of the skeleton by Paget's disease isnecessary before an augmentation of cardiacoutput can be observed; moreover she thoughtthat the disease must be in an active stage,accompanied by a high phosphatase level.For several years, we have studied the cir-

culatory modifications occurring in Paget'sdisease. We have observed seven cases. Inone patient, the disease affected only the rightleg; in another, it involved all bones exceptthose of the right arm; in the last five patients,the disease was generalized. The importance ofthe alterations varied according to the bonesaffected. In these patients, we studied thehemodynamic modifications that were presentat rest and also during exercise.

RESULTS

We have been able to confirm the existenceof a considerable increase in the peripheralblood flow in the limbs affected by the dis-ease. This phenomenon is clearly demon-strated in the patient in whom the diseaseis localized in the right leg (fig.1). This pa-tient, 70 years old, suffered from congestiveheart failure of coronary origin and from em-physema. The arterial oxygen saturation wasdecreased because of the lung condition (table

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CIRCULATORY DYNAMICS IN OSTEITIS DEFORMANS

FIG. 2. Skull in Paget's disease affecting the entireskeleton except the right arm.

FIG. 1. Osteitis deformans localized in the rightleg (A). The left leg is unaffected. (B).

TABLE 1.-Osteitis Deformans of the Right LegDE CON, Jean, age 70. Congestive heart

failure and emphysema

Oxygen content (vol. %)Right femoral artery ....................... 14.07Left femoral artery ........................ 14.08Right femoral vein ......... ............... 6.20Left femoral vein .......................... 4.13

Capacity (vol. %) ........................... 17.70Saturation (%) .............................. 81Arteriovenous difference (vol. %)

Right leg .................................. 7.87Left leg ................................... 10.94

TABLE 2.-Osteitis Defornmans. Arteriovenous OxygenDifference in a Normal Arm and in a Diseased Leg

Oxygen content (vol. %)Arterial ...................................

Left arm vein.............................Left, leg vein..............................

Oxygen arteriovenous difference (vol. %)Left arm ..................................

Left leg ..................................

13.855.6710.74

8.183.11

1). The arteriovenous oxygen difference was in-creased in both legs due to cardiac unsufficiency,but was definitely higher in the unaffected leg.In another patient, osteitis deformans involvedall bones of the skeleton, except those of the

FIG. 3. Pelvis in Paget's disease affecting the en-tire skeleton excelpt the right arm.

right arm (figs. 2 and 3). In this case, thearteriovenous oxygen difference was consider-ably lower in one of the diseased legs than inthe right arm (table 2). These findings clearlyindicate arterialization of the venous blood inthe involved limbs.

In the various cases of generalized Paget'sdisease which we have studied, the cardiac out-put remained wIithin the limits of normal whenthe patients were at rest. Two examples of thisare shown, in tables 3 and 4. Table 3 shows thehemodynaamic data obtained on a patient,,age 54, whose cardiac output was 3.72 litersand arteriovenous oxygen difference 4.38 percent. The pressures in the pulmnonary arteryand in the cardiac cavities were normal; soalso were the vascular resistances and the car-diac work. In another patient whose data are

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J. LEQUIME AND H. DENOLIN2

TABLE 3.-Payet's Disease. Circulatory DynamicsDEG, Jean, age 54

Oxygen consumption (cc. minute) ...... ..... 251Erythrocytes (cu.mm.) ................ 3,160,000Hemoglobin (%) .................... 62Oxygen content (vol. %)Femoral artery........................... 13.85Pulmonary artery .................... 9.47

Capacity (vol. %) .................... 14.9Saturation (%) ............................. 93Arteriovenous difference (vol. %.4.38Cardiac output (/m in.) .5.72Cardiac index (L,/min. M12) ................. 3.66Pressures (mm. Hg)

Capillary ......................... 8 (mean)Pulmonary artery .................. 25/8 (mean 12)Right, ventricle .................... 25/3Right auricle ...................... 3 (mean)Femoral artery .................... 120 (mean)

Total pulmonary resistances (dynessec. cm-5) ......................... 168

Vascular pulmonary resistances(dvnes sec. cm-5) ........ ......... 56

Right vent ricular work (j oules min.) 9Left ventricular work (joules min.) 91

TABLE 4.-Payet's Disease. Circulatory DynanicsDUR, ZMlie, age 55

Oxygen consumption (cc. minute) ........... 213Oxygen content (vol. %)Femoral artery ........................... 17.931Pulmonarv artery ......................... 13.80

Capacity (vol. %)........................ 19.88

Saturation (%). ............................ 92Arteriovenouis difference (vol. %) ........... 4.13Cardiac output (L/min.) . ............ 5.18Cardiac index (L/min. M2) ............ 3.27Pressures (mm. Hg)

Capillary . .7 (mean)1'ulmonary artery ............. 35/10 (mean 20)Right ventricle ................ 35/4 (meats 12)Right auricle .................. 4 (meats)Femoral artery................. 195/110 (meats 140)

P'eripheric vascular resistances (d)ynes sec.cm-5) ....................................... 2106

Total pulmonary resistances (dynes sec. cm-5). 310Vascular pulmonary resistances (dynes sec.em-5) ..................................... 201

Right ventricular work (joules min.) .......... 14Left ventricular work (joules min.) ........... 97

reported in table 4, the cardiac output was5.18 liters and the arteriovenous oxygen dif-ference was 4.13 per cent (fig. 4). The otherhemodyniamic characteristics were normal

FIG. 4. Skutll and psCevis in Paget's disease in-volving all the bonets.

if one takes into ac(ount, the fact that thepatient had an elevated blood pressure.

Briefly then, in patients in whom osteitisdeformanis was generalized and in whom ab-normal arterialization of the venous blood inthe limbs was present, circulatory dynamicswere practically unchanged at rest. It is thusprobable that in these patients a high cardiacoutput at rest is exceptional, and is to befound only its particularly active forms of thedisease. Generally, the increase of the circula-tion in the pagetoid bones will be insufficientto increase the cardiac output. These facts aresimilar to those that we have found in patientswith systemic arteriov-enous fistulas.f In man,the cardiac output varies considerably fromone patient to the other, in accordance withthe importance of the blood flow throughthe fistula. The same is true in the case ofdogs on which we have created fistulas be-tween the femoral vessels. In these animals,

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CIRCULATORY DYNAMICS IN OSTEITIS DEFORMANS

TABLE 5.-Paget's Disease. Circulatory Dynamics During an Exercise Test

Tota. pulm. Periph. vasc. CardiacCases consun02 Cardiac Cardiac Pulm. art. press. resist. resist. increaseCases ~~consumption output index

m g) (yesc. dnssc. irae(cc/min.) (L/min.) (L/min /M2) (mmllg) (dynes s) pe r 100cc.

I. DEG, JeanRest ................... 260 5.72 3.66 25/8 AM 12 168 1640Exercise................ .490 10.90 6.46 28/10 MI 13 95 856 2252

II. DUR, ZelieRest ................... 213 5.18 3.27 35/10 A/I 20 310 2106Exercise .... 476 8.66 5.48 42/15 M 25 201 1258 1320

arterialization of the right auricular blood var-ied widely in proportion to the size of the an-astomosis: on release of a compressed fistula,the oxygen content of the right auricle variedfrom 0.54 to 3.26 volumes per cent.6We have also investigated the hemodynam-

ics of our patients during exercise. Two ex-amples are shown in table 5. It is interestingto see that in the course of the exercise, theincrease of the cardiac output is abnormallyhigh. While in a healthy person, the cardiacoutput increases usually from 0.600 to 0.800liters per 100 cc. of oxygen consumed, one cansee that in these two patients, the increaseof the cardiac output is respectively 1.230and of 2.252 liters per 100 cc. of oxygen con-sumed. Such facts have been established inour various patients suffering from general-ized Paget's disease. They suggest that the cir-culation in the bones affected by Paget's dis-ease increases considerably under effort, andthus enables an important amount of blood toreturn prematurely to the right heart. Thesefindings are similar to those which we have ob-served in patients suffering from systemicarteriovenous aneurysms.

SUMMARY

In osteitis deformans (Paget's disease), theperipheral blood flow is increased in the bonesaffected by the disease. Nevertheless, the in-creased circulation usually is insufficient tomodify significantly the circulatory dynamicsof patients studied at rest.On the other hand, during exercise an abnor-

mal increase of the cardiac output is observed.This suggests an augmentation of the pe-

ripheral circulation in the bones involved bythe disease.

It is probable, that important alterations ofthe hemodynamics are exceptional and willonly be found in very severe and active formsof the disease.

SUMMARIO IN INTERLINGUA

In osteitis deformante (morbo de Paget),le peripheric fluxo sanguinee se augmenta in leossos afficite per le morbo. Nonobstante, leaugmento del circulation non suffice usual-mente pro modificar de maniera significativele dynamica circulatori del patiente in statode reposo.

Del altere latere, durante exercitio un aug-mento anormal del rendimento cardiac esobservabile. Isto supporta le conclusion que iloccurre un augmento del circulation periphericin le ossos que es afficite per le morbo.

Il es probabile que considerabile alterationeshemodynamic es exceptional e se trova sol-mente in formas severissime e activissime delmorbo.

REFERENCES

COURNAND, A., LEQUIME, J. AND REGNIERS, P.:L'Insuffisance Cardiaque Chronique. EtudesPhy siopathologiques. Paris, Masson et Cie, 1952.

2 DE MARNEFFE, R.: Donnees actuelles concernantla vascularisation osseuse dans la maladie dePaget. Acta cardiol. 8: 181, 1953.

EDHOLAI, 0. G. AND HOWARTH, S.: Studies on theperipheral circulation in osteitis deformans.Clin. Sc. 12: 277, 1953.

4 EDHOLM, 0. G., HOWARTH, S. AND i\lCM\ICHAEL,J.: Heart failure and bone blood flow in osteitisdeformans. Clin. Sc. 5: 249, 1945.

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HowswrTH, S.: Cardi.aic output in osteitis de-formitans. Clin. Sc. 12: 271, 1953.

6 JONNART, L., LEQUU8IE, .1. AND 1)ENOLIN H.:Ilecherches expe1rinentales sur les .ineui'vXsmesa.ritei io-vreineux pe ii)lieriiques. Acta clar(liol.7: 76, 1952.

L,1QUIMF, .J., DENOLIN, H. AND VERNIORY, A.:

La cir(ulation au cours (le mala(lie (le Paget.Acta cardiol. 7: 318, 1952.

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insufficienca cardiacta en la osteitis deforimante(le Paget. Rev. Asoc. med. argent. 64: 90, 1950.

RER1BOUL, H. L'Arte'iogiaphie des M\Iemlres et de

1'Aorte Abdominale: Etude Critique. Paris,Masson et Cie, 1935.

10 RUTISHAUSIE1R, 1,., VEYRAT, R. AND ROUILLE1R,CH.: La vascularisation de 1'os pagetique.Presse me(d. 62: (654, 1954.

1 SORNBEIRGI R, C. F. AND SAIEDAL, M. I.: Themechaniism an(l inciidence of cairliovrascularchanges in l'aget's dlisease, (osteitis (lefor-mans). Circulation 6: 711, 1952.

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J. LEQUIME and H. DENOLINCirculatory Dynamics in Osteitis Deformans

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1955 American Heart Association, Inc. All rights reserved.

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