INTRAUTERINE MYOCARDIAL INFARCTIONBY

ERIC N. COLEMAN and ALISTAIR M. MACDONALDFrom the Royal Hospital for Sick Children, Glasgow, and the Department of Child Health,

University of Glasgow

(RECEIVED FOR PUBLICATION MARCH 23, 1962)

In infancy myocardial disease other than myo-carditis is undoubtedly rare, but infarction resultingfrom coronary artery occlusion and glycogenstorage disease of the heart occasionally occur.Still more unusual apparently is the lesion firstreported in 1952 by Uhl in which the right ventricularmyocardium is virtually absent, and this curiousdefect is likely to present serious diagnostic difficulty.Uhl (1952) regarded it as representing an intra-uterine failure in development of the primordiumof the right ventricular myocardium, but ourobservations of the case to be reported lead us totake a different view of its pathogenesis.

Case ReportA.MacL. was the third child of healthy parents. Her

two sibs were healthy. She was born after a normalpregnancy and weighed 2-95 kg. She cried immediatelyafter birth and was not then cyanosed. Aged 8 days ondismissal from the maternity hospital, she seemed wellexcept for a slight mucoid nasal discharge. Duringthe next 10 days she continued to feed hungrily butvomited occasionally. Persistent cyanosis was firstnoticed at the age of 19 days and this was increased byfeeding or crying. She became irritable and during thethree days preceding her admission to hospital at leasthalf of every feed was vomited. When admitted atthe age of 26 days she was well developed, vigorous andafebrile, but had marked central cyanosis and dyspnoeaat rest. The heart rate was 150 per minute. There wasno oedema but the liver edge was felt two and a halffingerbreadths below the costal margin. No cardiacpulsation was palpable. The heart sounds at all areaswere of poor quality but no murmur was heard. In theelectrocardiogram the waves and complexes were smallin all the recorded leads, particularly in V4R, and therewas no evidence of ventricular hypertrophy (Fig. 1).A radiograph of the chest disclosed a large heart shadow,some concavity of the pulmonary segment and clearlung fields (Fig. 2). No methaemoglobin was detectedin the blood.On clinical grounds transposition of the great arteries

was suspected but the radiological appearances raisedthe alternative possibility of Fallot's tetrad. Theelectrocardiographic features, however, were not inaccord with either diagnosis.

During the next five days the cyanosis and dyspnoeapersisted without substantial change except for accen-tuation of cyanosis during feeding and crying. Theaverage respiratory rate was 50 per minute. She tookfeeds eagerly but vomited occasionally. Oxygen wasadministered after feeds with beneficial effect on hercolour. Suddenly and quite unexpectedly she died onthe sixth day after admission at the age of 32 days.

Findings at Autopsy. The body was that of a normallydeveloped 5-week-old female child weighing 3,458 g.There was no external sign of disease. Neither lungsnor liver showed congestion.The only remarkable anatomical observation was made

in the heart which weighed 24 g. and was normal inshape. On routine examination a clot was found at theapex of the right ventricle adherent to the wall. Theventricular wall was thin but not excessively so. Theforamen ovale seemed competently closed by a valve.The ductus arteriosus was still patent but probablyclosing. There was no anomaly of the coronary vessels.

Several blocks of the heart were made for examination.Neither in the left atrium nor left ventricle was anymuscular lesion found. Nowhere was a coronaryartery lesion found. The striking feature was absenceof muscle from the right ventricular wall, which is wellshown in Fig. 3, and an attempt was made to determinewhether this was a developmental defect or the result ofsome catastrophe. The sharp demarcation between thenormal muscle of the left ventricle and its absence in theright is seen in Fig. 4. Furthermore, in the rightventricular wall (Fig. 4) a collection of deposited calciumand giant cells was recognized; in Fig. 5 they can be seenat a higher magnification. In spite of the presence ofcalcium these multinucleated giant cells are not of theusual foreign body reaction type; it was thought possiblethat they could be the remains of muscle cells, butrepeated attempts to demonstrate cross-striation failed.From a general examination of the apex of the rightventricle the fihth impression was gained that the tissueexamined was in fact granulation tissue, and Fig. 6 showsthe high vascularity of the tissue of a pattern typicalof granulation tissue. Here again after extensive exami-nation no cross-striation was recognized and thereforeno evidence of muscle cells. The apex of the rightventricle was proved to have an adherent clot showingextensive organization with the right ventricular wall;

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INTRAUTERINE MYOCARDIAL INFARCTION

FIG. 2.-The teleradiogram shows cardiomegaly, some concavity ofthe upper left heart border and clear lung fields.

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FIG. 1.-All waves and complexes are small and there is no evidenceof hypertrophy of either ventricle.

this granulation tissue reaction could be found for some3 cm. above the apex. Also throughout the rightventricular wall at the apex were small collections ofround cells, again suggesting an inflammatory basis forthe lesion.

Discussion

A myocardial defect closely similar if not identicalto that reported here has been described by Uhl(1952), Castleman (1952) and Taussig (1960) inpatients whose ages at death were 8 months, 24years and 6 years respectively. Congestive heartfailure preceded death in each instance. Taussig

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FIG. 3.-Apex of heart (P.A.H. x6 3). This shows absence ofmuscle in the right ventricle.

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446

FIG. 4.-Junction of right and leftThere is sharp demarcation betvcoronary vessel is patent. Calciurm

wall

FIG. 5.-Higher power of right v

is deposited and giant cells have ft(Haematoxylin an

FIG. 6.-Wall of right ventricle atA vascularized scar.

ARCHIVES OF DISEASE IN CHILDHOOD

(1960) described another example in which at 17months of age the diagnosis was made by means

of cardiac catheterization and angiocardiography.Ebstein's anomaly of the tricuspid valve waslater proved to be present in addition. In theother cases the correct diagnosis was not suspectedduring life and the diagnostic possibilities enter-tained included Fiedler's myocarditis or congenitalheart disease (Jhl, 1952), Fallot's tetrad (Taussig,1960), idiopathic cardiac hypertrophy or myocarditisof unknown aetiology (Castleman, 1952).

In the present case the diagnosis which seemedmost probable was Fallot's tetrad or transpositionof the great arteries, but the absence of a heartmurmur and, in particular, the electrocardiographicfindings were considered to be exceptional features.

ventricle at apex (P.A.H. x26). Uhl's patient (1952) had no murmur and althoughween right and left ventricle;n deposition in right ventricular Taussig (1960) heard an apical diastolic murmur and

Castleman (1952) described a pulmonary systolicmurmur, it is difficult to attach any diagnostic

li0i; Z significance to these. As in the electrocardiogramr

"

^* tof the present patient, QRS complexes of low voltage:g were observed by Uhl (1952) and by Castleman

55!.'i ..g (1952), but tall P waves were also described by theformer author and by Taussig (1960). In the presentcase the concave pulmonary segment of the heart on

if I;,f the radiograph, together with the clear lung fieldsI i led to the conclusion that infundibular stenosis was

likely and lent support to the erroneous diagnosis oft; ~Fallot's tetrad. The cardiomegaly was, however,

a suspicious feature. In the description of hersecond patient Taussig (1960) referred to a largeheart shadow and clear lung fields on the radiograph.Her first patient diagnosed incorrectly as Fallot'stetrad seems likely also to have shown clear lung

ventricular lesion where calcium fields. Uhl (1952) described the heart shadow of6rmed. No evidence of muscle. his patient as being grossly enlarged and globular.id eosin x 124) It is clear, therefore, that this defect of the right

ventricular myocardium is yet another cyanotictype of congenital heart disease in which the electro-cardiogram may not show right ventricular domi-nance and that this taken in conjunction with clinicalevidence of right ventricular enlargement, cardio-megaly and clear lung fields should suggest thepossibility of the correct diagnosis.The cause of cyanosis in our patient is not certain,

but although at autopsy the foramen ovale seemedsoundly covered by a valve it is likely that in liferight atrial hypertension could have caused thevalve to open so permitting venous blood to passinto the left atrium. It is probable that peripheral

.l > 6.> stagnation of blood in a failing circulation con-W S t .tributed to the cyanosis. The ductus arteriosus can

apex. No evidence of muscle scarcely be incriminated as the site of a veno-. (P.A.H. x 68.) arterial shunt, because it seems impossible that the* \ * . * ....... * * * . copyright.

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INTRAUTERINE MYOCARDIAL INFARCTION 447

pulmonary artery blood pressure could haveexceeded that in the aorta.

In her account of this lesion Taussig (1960) hasdescribed it as parchment right ventricle and by sodoing recalls its naked-eye resemblance to themyocardial lesion of unknown pathogenesis whichaffected both ventricles of the heart of a middle-agedman in Osler's collection and which was referred toby Segall (1950) as parchment heart. Uhl (1952)thought that the extensive defect of the right ventri-cular myocardium which he described representedinjury during intrauterine life and was most probablythe result of a failure of growth of the right cardio-genic fold. He considered but rejected coronaryartery occlusion and foetal endomyocarditis ascausal. The present patient died at the age of32 days the opportunity thus being given for thelesion to be studied at an earlier stage in its develop-ment. There can be no doubt that it originatedduring intrauterine life, but because of the replace-ment of the right ventricular myocardium by granu-lation tissue, the case for a defect in the developmentof the primordium of the right ventricle does notseem tenable. Nevertheless, the cause for an inflam-matory lesion is difficult to produce. The possibilityof foetal endomyocarditis is ruled out because therewas little evidence of an active infection at the timeof examination, the cellular reaction expected of amyocarditis was lacking and the lesion was sharplylimited to the right ventricular myocardium. No

occlusion or aberration of coronary vessels wasfound, but we are of the opinion that the onlytenable theory is that this clearly demarcated lesionin which granulation tissue was so abundantrepresents myocardial infarction as the result ofsevere ischaemia due to occlusion of the rightcoronary artery at some time during the course ofintrauterine life.

Summary

An infant with cyanotic congenital heart diseasewho died at the age of 32 days is described. Theright ventricular myocardium was found to bereplaced by granulation tissue. The difficulty ofclinical diagnosis is discussed. The lesion mayreflect intrauterine infarction of the right ventricularmyocardium.

We are indebted to Professor J. H. Hutchison forpermission to report this case and for his criticism. Wethank Dr. S. P. Rawson for his X-ray report, and Messrs.J. Devlin and W. Mason for the illustrations.

REFERENCES

Castleman, B. (1952). Case records of the Massachusetts GeneralHospital. Case 38201. New Engl. J. Med., 246, 785.

Segall, H. N. (1950). Parchment heart (Osler). Amer. Heart J.,40, 948.

Taussig, Helen B. (1960). Congenital Malformations of the Heart,2nd ed., Vol. 2, p. 138. Harvard University Press, Cambridge,Massachusetts.

Uhl, H. S. M. (1952). Previously undescribed congenital malforma-tion of the heart: almost total absence of the myocardium of theright ventricle. Bull. Johns Hopk. Hosp., 91, 197.

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