CHRONIC CARDITIS IN A CHILD OFNINETEEN MONTHS

BY

B. E. McCONNELL, M.D., M.R.C.P.From the Department of Pathology, Bristol University

(RECEIVED FOR PUBLICATION, JuNE 22, 1949)

Chronic carditis in infancy is so rare that thefollowing case, which presented both clinical andpathological problems, is considered worthreporting.

Case HistoryA.N., a boy and the only child, was stated to

have been a fortnight premature and to haveweighed 5j lb. at birth. Breast fed for one month,he was then given bottle feeds as his mother hadinsufficient milk. On this he thrived, and sub-sequent progress was normal with no history of anyillness until his last. At 16 months he weighed21 lb., and at 18 months he had 12 teeth and couldwalk and speak a few words. Both parents werehealthy, and there was no history of illness duringthe mother's pregnancy.Three weeks before admission, at the age of

19 months, the child became fractious and appearedunwell. The only abnormal physical sign was stiffand painful middle fingers, but the general conditiondeteriorated and he was eventually sent to hospital.

Clinical Examination. On admission the childwas lying quietly, but resented disturbances. Thecheeks and mucous membranes were pale, thelimbs hypotonic, and the nails spoon-shaped. Therewas sweating and apparent recent loss of weight,the actual weight being 18 lb. 3 oz. The skullcircumference was 191 in. (normal average at thisage, 18 in.), and the anterior fontanelle admitteda finger tip, indicating delay in closing, which isnormally complete at 18 months. The middlefinger of each hand was semi-flexed and painful andslight oedema of the feet and lumbar region wasnoticed.The temperature was 990 F., respiration 40,

and pulse 135 per minute. The blood pressure was75/40. Scattered r'ales were noted.The apex beat was not localized on palpation,

but on percussion the area of cardiac dullnessextended three finger breadths to the left of thesternum in the fourth intercostal space and onefinger breadth to the right in the third space. Onauscultation a blowing apical systolic murmur,

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conducted into the axilla, was heard. A systolicmurmur was also audible to the right of the sternum.The liver was palpable two finger breadths below

the costal margin and the spleen one finger breadth.Special Investigations. Radiographs showed an

enlarged heart and some opacity of the left upperand lower zones in the chest. The fingers appearednormal.The Mantoux reaction (1: 10,000) and the

tuberculin patch and jelly tests were negative.BLOOD EXAMINATION. R.B.C. 4,200,000/cu. mm.;

Hb 72% (Haldane). The film showed mildanisocytosis and slight hypochromia. W.B.C.12,200 (neutrophils, 52%; eosinophils, 1%; lympho-cytes, 42%; monocytes, 5%).

Progress. For the first three days after admissionthere was little change in the child's condition,but the fourth day brought the onset of markeddyspnoea and cyanosis. An oxygen tent gaveslight relief of symptoms, but penicillin and sulpha-thiazole in ordinary doses were without effect, andthe clinical course was rapidly downhill withincreasing dyspnoea, cyanosis, and sweating. Deathoccurred on the ninth day with great respiratorydistress.No clinical diagnosis had been made. The

possibility of rickets, tuberculosis, or a pyogenicinfection had been dismissed after the specialinvestigations.

Post-mortem FindingsNecropsy was performed 9j hours after death.The body showed little subcutaneous fat. Each

pleural cavity contained about 100 ml. of straw-coloured fluid with no clot. The pericardiumappeared normal.The heart (Fig. 1) weighed 65 g. (normal at this

age is 52 g.). On section the right ventriclemeasured 4- 5 mm. in thickness and the left ventricle11 mm. The striking feature was the great thicken-ing of the endocardium. Both mitral and tricuspidvalves felt like cartilage, and the chordae wereshort, thick and fused. At the bases of these valvesfibrosis could be seen extending into the

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CHRONIC CARDITIS IN A CHILD

myocardium, and in other areas of the muscle smallpatches of fibrosis were evident, particularly at thebases of the papillary muscles.The contiguous margins of the cusps of the

aortic valve were fused, while the free margins werethick and hard. The adjacent aortic wall was alsothickened, and at a distance of 2 cm. above thevalve measured approximately 2 mm. in thickness.Above the valve the'lumen of the aorta appearednormal throughout, while the intimal surface wasquite smooth.Both lungs showed marked chronic venous

congestion. The costo-chondral junctions and'middle fingers were normal on section and noabnormality was found

Discussion

The problem here was to ascertain the natureand age of the lesion, which for practical purposeswas confined to the heart and aorta. The picture ofmyocardial fibrosis, valvular distortion and thicken-ing with fusion of chordae tendineae, is so highlysuggestive of a chronic rheumatic process thatit is hard to see what alternative diagnosiscould be made. The only logical possibilityis an- endocarditis, not rheumatic, of unknownaetiology, and as yet not clearly defined.Verrucose endocarditis due to disseminated lupus

erythematosis does not111 dlly U 611'lu1 1gu11 U1

tissue.Histology. Sections

were taken from bothright and left sides ofthe heart to show theauriculo -vent ricularjunction and theattachment and cusp ofthe appropriate valve.The section from theleft side included thecircumflex branch ofthe left coronary artery.The histological picturein each section wassimilar. The epi-cardium appearednormal. The myo-cardium showed areasof fibrosis adjacent tothe blood vessels.There was also markedfibrosis at the base ofthe valve, the fibroustissue interlacing with.&h- - 1-ol e & &h;r ;+-:A

The circumflex branchof the left coronaryartery showed subin- CENTIMETREStimal fibrosis, very jJj jJmarked replacementfibrosis of its medial FIG.. 1.-Heart showingcoat, and also para-vascular fibrosis. The valves were both thick andconsisted of hyaline fibrous tissue. No vegetationand no signs of acute or subacute inflammationwere evident.The intima of the aorta was normal (Fig. 2).

The media was normal at its inner part, but near theadventitial junction had been involved in a severeinflammatory process. Fibrosis was markedadjacent to the vasa vasorum and in many placesislets of elastic tissue were surrounded by fibroustissue, as if cut off by an advancing tide. Theadventitia was irregularly thickened and consistedof hyaline scar tissue.

IT

meet tne case asno abnormality wasfound in any otherorgan, nor did theclinical picture in anyway suggest thedisease. Fiedler'smyocarditis spares theendocardium, which inthis case was the mostheavily damaged part.So-called idiopathiccardiac hypertrophy isan improperly under-stood condition. Theliterature on the sub-ject was reviewed byKugel and Stoloff in1932 and again byKugel in 1939. Whilethe clinical picturethey describe is similarto that in this case, theendocardium is onlyslightly, if at all,involved. Moreover, a

|42 51 congenital lesion can-WL. not be considered as

iitral valve and aorta. the picture is soobviously that of a

chronic healed inflammatory process.If rheumatic carditis is to be considered, then an

acute rheumatic process is implied, which is veryrare in infants.

Paul White (1944) states that ' rheumatic heartdisease . . . is especially-rare before the age of twoyears'. Various writers have described the diseasehowever. In 1896 Abrahams reported six cases innew-born infants whose mothers were sufferingfrom rheumatic fever while in labour. The infantsall had arthritic signs or cardiac murmurs, the

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ARCHIVES OF DISEASE IN CHILDHOODsigns and symptoms responding to salicylates (butno follow-up of the patients is reported). Cathalaand others (1936) described a child of 22 monthswith limb pains, an apical systolic murmur, andenlargement of the heart. The symptoms res-ponded to salicylates, but the murmur remainedand was considered to indicate mitral incom-petence. Saubidet (1936) described three infantsof 8, 18, and 21 months who showed joint painsand fever, but noabnormal cardiacsigns; the symp- etoms responded tosalicylates. Thesecases cannot beregarded asproven, althoughCathala's patientshowed a clinicalcourse stronglyresembling that ofrheumatic carditis.Denzer, however(1924), reportedthree cases ofinfants under twoyears in whichthe diagnosis ofrheumatic carditiswas proven atnecropsy. Two ofthese cases wereacute, while thethird was esti-mated at sixmonths' duration.Kissane and

Koons (1933) des-cribed a case inwhich a womanwas ill during ..pregnancy withrheumatic fever.The child was Xborn with arthritis FIG. 2.-Transverse section ofand cardiac mur- elastic tissue.murs and remained

when rheumatic carditis, affecting mainly the mitralvalve, was found.McIntosh and Wood (1935) reviewed 24 cases in

infants or young children in the New York Babies'Hospital. In six of these the diagnosis was con-firmed at necropsy, the youngest child being 20months old at death and suffering from acuterheumatism at the time.There is, therefore, sufficient precedent to con-

torta x 75. Verhoff's stain for

under the observation of the one physician until rheumatism early in its firsthe was nine years old when he died in an attack ofacute rheumatic fever. Nepropsy showed chronic Summaryrheumatic carditis with a recent acute exacerba- A case is reported of a childtion. 19 months in which necropsy

Fischer (1934) described an infant one year of lesion suggestive of chronic rhage dying with pneumonia. A clinical diagnosis of I wish to express my thanks to Dicongenital heart disease was disproved at necropsy whose care this child was at th

sider a diagnosisof rheumaticcarditis in a childof 19 months.Having made thisdecision the nextproblem is toattempt to assessthe age of thelesion. The recentworkof McKeown(1945) on theevolution of theAschoff nodulehas shown thathealing takes somenine months andthat before thisthe lesions havea characteristicarchitecture andcan be recognizedas specific entities.When healing iscomplete there issimply scar tissuewith no specificarch it e c ture,although the para-vascular andsubendocardialdistributiorn ishighly suggestive,as it was in thiscase. On thesegrounds it is sug-gested that thischild had acuteyear of life.

dying at the age ofrevealed a cardiaceumatic carditis.r. Beryl Corner, underie Bristol Children's

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CHRONIC CARDITIS IN A CHILD 189Hospital; also to Professors T. F. Hewer and C. BrucePerry for helpful criticism and advice. My thanks arealso due to Mr. G. W. Griffin and Mr. G. Rogers of theDepartment of Pathology, Bristol University, for thehistology and photographs.

REFERENCESAbrahams, R. (1896). Med. Rec., N.Y., 50, 547.Cathala, J., Sustendal, J., and Patey, G. (1936). Bull.

Soc. Pediat., Paris, 34, 187.Denzer, B. S. (1924). J. Amer. med. Ass., 82, 1243.

Fischer, V. E. (1934). Amer. J. Dis. Child., 48, 590.Kissane, R. W., and Koons, R. A. (1933). Arch. intern.

Med., 52, 905.Kugel, M. A., and Stoloff, E. G. (1932). Amer. J. Dis.

Child., 45, 828.Kugel, M. A. (1939). Amer. Heart J., 17, 602.McIntosh, R., and Wood, C. L. (1935). Amer. J. Dis.

Child., 49, 844.McKeown, F. (1945). Ulster med. J., 14 97.Saubidet, E. (1936). Sem. mdd., 2, 1077.White, P. (1944). 'Heart Disease,' 3rd Ed., 327.

Macmillan. New York.

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