INFECTIVE ENDOCARDITIS IN CONGENITAL HEART DISEASE

747

o’clockI INFECTIVE

benefit. He did not regain consciousness -until 6 in the evening. Afterwards his convalescence was slow but continuous.

ENDOCARDITIS CONGENITAL HEART DISEASE

Commentary.

IN

INVOLVING THE PULMONARY ARTERY. this, unlike the first case, the heart was not felt the reasons the but for through diaphragm, believing BY H. E. A. BOLDERO, B.M.OXF., M.R.C.P. LOND., it had stopped were equally strong. There was the PHYSICIAN TO THE EVELINA HOSPITAL; MEDICAL REGISTRAR, cessation of pulse, apex beat, and respiration, the MIDDLESEX HOSPITAL; appearance of the patient’s face, the fact that when

In

the needle was pushed into the heart no movement was felt, the prompt appearance of oedema of the lung when the heart recovered, and the protracted interval before the return of consciousness. In such emergencies it is very difficult to estimate time exactly, but the duration of the intervals between the various events was noted immediately-in consultation with Drs. Archibald and Scrimger in the first case, and with Dr. Scrimger in the second. One of the most noticeable features in such cases is the length of time the nervous system takes to There is always recover as compared with the heart. This a long period of unconsciousness afterwards. is well shown in a case reported by W. M. Mollison.2 A boy of 6 was given a mixture of chloroform and ether

for the operation of removing tonsils and adenoids. Just as it had been completed pulse and respiration stopped. By means of direct injection of pituitrin into the heart and massage through an opening in the abdominal wall, the heart was started again after 13 minutes. The patient was unconscious for seven days. For ten days he had choreic movements and symptoms of brain irritation. He eventually recovered.

AND

D.

E.

BEDFORD, M.B., B.S. LOND.,

MEDICAL

THE

REGISTRAR,

occurrence

of

MIDDLESEX HOSPITAL.

infective

endocarditis

in

congenitally malformed hearts, although emphasised in text-books of medicine, is nevertheless infrequently met with in hospital practice. The association is. interesting, first because it is rare, and second because it throws light on the origin of infective endocarditis. in general. The recent

investigations

of Lewis and Grant have

demonstrated the importance of congenital defects of

the aortic valves in the causation of subacute bacterial endocarditis ; and we propose here to consider the relationship of the more common congenital malformations to infective endocarditis involving the

pulmonary artery. Endocarditis, simple or vegetative,

is less common at the pulmonary valves than in any other situation, pulmonary incompetence being the rarest valvular lesion. Statistics based on postmortem findings published by Osler (209 cases), Horder (118),3 Washbourn (100),4 Cowan and Ritchie

Another interesting feature is the prompt appearof oedema of the lung, apparently due to the arrest of circulation. In looking over the records of (70),5 Glynn (4=7),s Abrahams (39),’ Libman (19),8 such cases in the literature of the last few years one comprising 602 cases of infective endocarditis, show finds the condition of the lung mentioned very rarely. involvement of the pulmonary valves in 30 cases only, Prus noticed it in his experiments on dogs, two out an incidence of 5 per cent. From an analysis of 198 of 12 dying from this cause during the first hour after consecutive cases of infective endocarditis from the revival. post-mortem records of the Middlesex Hospital, we The chief lesson to be learned is the necessity for found the pulmonary valves affected in nine instances, immediate injection of adrenalin into the heart. or 4-5 per cent. Three of the nine cases had conWhen breathing stops during anaesthesia the first genitally deformed hearts. thing is to ascertain the condition of the heart, for Apart from congenital malformations, the pulno amount of artificial respiration will oxygenate monary valves are most frequently affected in blood which is not circulating. Time spent in giving gonococcal endocarditis, and in pudrperal and trauis matic septicaemias. Rheumatic endocarditis of the hypodermic injections, hot applications, &c., merely compromising the chances of recovery. The pulmonary valves is almost unknown, and thereactual injection is apparently harmless. Mamy post- fore one is able to consider the development of the mortems have been done on patients in whom cardiac infective process in this situation, as it were, in injections have been given, but in none has damage virgin soil. to the heart been recorded, whereas damage to the With regard to the relative frequency of the various heart muscle which was inconsistent with life has malformations associated with pulmonary infective been revealed at post-mortems after cardiac massage. endocarditis, Newton Pitt" found among 21 collected The needle should be inserted into the fourth inter- cases : (1) patency of the ductus arteriosus in threecostal space at the upper edge of the fifth rib, close cases ; (2) defects of the interventricular septum in ten to the sternal border, or else one finger’s breadth cases ; and (3) pulmonary stenosis in eight cases. Weinside the left border of relative heart dullness. By have found descriptions of 36 cases in the literature.,the first route, the right, and by the second, the left patent ductus arteriosus was present in 13 cases, ventricle is reached. By either the internal mammary patent interventricular septum in 12, pulmonary artery is avoided. The needle is known to be in one stenosis in 7, patent auricular septum in 6, defects of the cavities of the heart by the escape of blood of the pulmonary valves in 2, and stenosis of the when the stylet is withdrawn. If the heart is much conus arteriosus in 2. distended with blood it may be of advantage to let It would seem most rational to make some escape. 1. Patent Ductus Arteriosus. the injection into the heart muscle itself. One French to Libman this is the most important According writer advises injection into the cavity of the heart, defect connected with infective endocarditis. The believing that absorption through the lymphatics of following interesting case has recently occurred which the endocardium, can take place in time.3 The injecunder our observation for over two years, and in tion can be conveniently made by means of a spinal was which an autopsy was performed. puncture needle. Other substances than adrenalin A male of 29 years was admitted to the Middlesex Hospital have been used, but none have proved so satisthe care of Dr. C. E. Lakin in June, 1921, complaining factory. The best method of artificial respiration is under pain in the legs and of feeling unwell. According to his by means of the intra-tracheal insufflation of air. of mother he had had " rheumatism in childhood," and Sylvester’s method has the drawback of hampering diphtheria at 7 years of age. He had always been delicate the heart by means of pressure on the lower thorax. and " suffered with his heart,"and had never been able to doMy thanks are due to Dr. Archibald and to Dr. ordinary work. He was of average physical development, but Scrimger for permission to report these cases and mentally backward. There was no cyanosis and no clubbing for the help they gave me in recollecting the course of the fingers or toes, but the complexion was highly coloured. of events. Physical examination revealed a greatlv enlarged heart with some bulging of the prsecordium, and a diffuse pulsation ance

3

2 British Journal of Children’s Diseases, January, 1917. D. Petit-Dutaillis, Journal de Chirurgie, Juli-Décembre,

1923, p. 519.

most

marked in the second left interspace. A pronounced thrill with maximum intensity over the pulmonary area was palpable throughout the cardiac cycle. On auscultation a.

748 diseased, in addition to the

mass of vegetations on the wall of the pulmonary artery which started at interspace near the sternum ; the bruit continued throughout the ductus but did not extend to the pulmonary systole and diastole with systolic intensification. In addition valves. a loud " bang " was audible over the pulmonary area during The fact that the pulmonary valves so frequently systole. X ray examination showed the heart to be consider- escape make it quite clear that the process originates aorta to as as to the The the right, well left. ably enlarged was dilated, but there was no evidence of aneurysm. The in the artery wall, most probably at the site of the electrocardiogram showed great increase in amplitude of the ductus. This extensive endarteritis would appear to ventricular deflections, with an inverted T wave in lead 1, be peculiar to the pulmonary artery. In this connexion but no other abnormality. The systolic blood pressure was the case described by Weber and Fiirth," of a man, 200 mm. Hg, and the diastolic 90. The blood picture aged 27, who had had gonorrhoea, is of interest, in that showed a polycythaemia of 8,375,000 red cells per c.mm., the process occurred without there being a congenital with a colour index of 0-58. The Wassermann reaction was defect or valvular lesion. In several of the above first the fever for The had negative. patient slight irregular three weeks only, and he was discharged after two months, cases the artery was involved as far as the mediumin considerably improved general condition. A tentative sized branches, and in four case mycotic aneurysms diagnosis was made of patent ductus arteriosus with super- were also present.

very loud harsh bruit was heard over the praecordium and at the back of the chest, most marked in the second left

,

added endocarditis. He was readmitted in June, 1923, after being under observation in the out-patient department periodically. His general condition had deteriorated, and symptoms of cardiac failure were now present. There was cedema of both legs and moderate enlargement of the liver. The spleen On examination of the heart much the same was palpable. physical signs were found, the lungs, however, showing bubbling r&les and rhonchi all over the chest. The temperature ranged from 99° to 102° F. ; the pulse from 100 to 120 per minute, and the respirations were increased to 40 per minute. The blood examination now showed marked anaemia, the red cells being 2,700,000 per c.mm., and the leucocytes 9700. No tubercle bacilli were present in the The patient’s condition did not sputum. improve under treatment and he died one month after admission.

Post-mortem Examination.-The heart was greatly enlarged and both ventricles were hypertrophied, the left ventricular muscle being 9/10 of an inch thick, and the right 4/10. A massive warty vegetation, measuring 11 by 1 inch, was found completely filling the lumen of the pulmonary artery, extending from the valves to slightly beyond the bifurcation, and firmly adherent to the anterior wall of the vessel. The ductus arteriosus was patent, measuring 3/5 inch (1’5 cm.) in length, its lumen being partly filled with small warty vegetations which protruded from the aortic orifice of the canal. Two small calcified areas were present in the wall of the aorta ; one just below the ductus opening, the other on the 4onvexityof the aorta directly opposite the ductus opening. On passing a probe along the canal from the aortic aspect, it was found to be buried in the mass of vegetations in the pulmonary artery previously described. The pulmonary cusps were the seat of extensive vegetations, growing mainly from the ventricular surfaces. Between the right anterior and posterior cusps was a mass 1 inch loxig extending upwards into the pulmonary artery and downwards into the ventricle, causing great distortion of the posterior cusp. The aortic cusps were covered on their ventricular surfaces with luxuriant vegetations; in addition, both the mitral and tricuspid valves showed small recent deposits, situated mostly on their auricular surfaces. The lungs showed brown induration and great cedema, but no infarction. The liver weighed 40 oz., and on section many small inflammatory areas were seen, which microscopically proved to be foci of round-celled infiltration ai3ound branches of the hepatic artery. Sections of the kidneys revealed a mild degree of embolic glomerular nephritis.

Defects of the I nter- Ventricular Septum. The post-mortem records of the Middlesex Hospital 2.

since 1860 contain seven cases of infective endocarditis in which this lesion was present. In one case a congenital aperture in the undefended spot was the seat of a mass of vegetations which projected into the right ventricle and involved the septal cusp of the tricuspid valve. In another case stenosis of the conus arteriosus was also present, the constricted portion being surrounded with fibrinous vegetations. In a third case the stenosed orifice of an aberrant pulmonary artery was fringed with vegetations, while the valves Yet another case of especial interest were healthy. was one described by Dr. Thompson in 1877, in which two perforations of the ventricular septum at the site of an aneurysmal bulging were fringed with vegetations, which had spread into the conus arteriosus and involved the pulmonary valves, causing incompetence. These perforations were considered as acquired, but from their situation in the membranous part of the septum it seems possible that a congenital defect was originally present. Five saccular aneurysms occurred The specion branches of the pulmonary artery. men is preserved in the museum of the Middlesex

Hospital. cases reported in the literature, vegetations commonly found around the margin of the aperture, spreading to the ventricles, either contiguously or forming deposits on the wall opposite. The pulmonary, and frequently the aortic, cusps

In the

were

were

also involved.

Where

one

side of the heart alone

is

affected, paradoxical embolism may occur. 3. Pulmonary Stenosis. In our analysis of 198 cases of infective endocarditis there is no instance of its occurrence in this congenital lesion. This relative infrequence is probably due to the fact that pulmonary stenosis has a more serious prognosis than the other two lesions we have discussed ; and therefore subjects are less likely to reach

the age at which infective endocarditis is most common. Dickinsonl2 described an interesting -case The above case is a good example oflsubacute of its occurrence in a male of 4 years, in which bacterial endocarditis engrafted on a congenital thrombosis of the pulmonary artery resulted. lesion. The infective process appears to have comConsiderations. Tnenced at the pulmonary end of the ductus arteriosus There is as yet, we feel, no wholly satisfactory and to have spread along the wall of the pdhnonary ’ explanation of the fact that infective endocarditis of artery, finally involving the valves.*lit Hamilton and Abbott’have described a similar the left side of the heart is far more common than that The study of the inception of the case, and have collected ten others from the literature. of the right. In their own case a large oval mass of vegetations infective process is simplified by considering it as it was present on the wall of the pulmonary artery, but occurs in congenital lesions, more especially of the the valves had escaped. From a careful histological pulmonary valves, for thus can rheumatic damage be study they conclude that the process originated in the excluded. The common lesions-namely, defects of the septa pulmonary end of the ductus as a result of invasion of the intima at the point of maximum, strain, and and patency of the ductus arteriosus-might prenot as an embolic infection via the vasa vasorum. dispose to infection of the pulmonary valves in the Of their 11 cases the wall of the pulmonary artery following ways :was involved in all, the wall of the ductus in nane, the 1. The rise of blood pressure produced in the lesser wall of the aorta in seven, while the pulmonary circulation exposes the pulmonary valvular apparatus valves were affected in two cases only. In a similar to increased strain. That this does occur is certain, as case described by Horder3 the aortic valves were evidenced by the enlargement of the right ventricle and dilatation of the pulmonary artery so often * The case, and subsequently the specimen, were shown by In cases of patent ductus arteriosus AbbottlS Dr. C. E. Lakin at the Medical Society of London. (Med. Soc. present. has found atheromatous changes in the pulmonary Trans., vol. xlv., p. 29.)

749

artery, and even aneurysm of this vessel has been described (Durno and Brown).14 There is, however, little reason to suppose that this increased pressure is a factor in the predisposition to infective endocarditis. In other conditions, where a high pulmonary pressure occurs, there is no special liability to infective lesions. Stokesl5 observed that in cases of atheroma and dilatation of the pulmonary artery, secondary to emphysema, the valves were usually unaffected. Again, in the cases of atheroma and dilatation of the pulmonary artery described by Rogers16 as occurring in India, and which he attributes to syphilis, the valves were normal. Furthermore, in the parallel instance of the aortic valves in cases of hyperpiesis with raised systemic pressure, infective endocarditis

A PLEA FOR THE PRESERVATION OF THE

PREMAXILLARY BONES IN CONGENITAL CLEFT PALATE. BY ALBERT D. DAVIS,

(From the Department

B.SC., M.D. NEB., D.D.S.

of Oral Surgery, University of

Nebraska, College of Medicine.)

IT has been my good fortune to assist a noted oral surgeon in the compilation of data from all parts of the world on cleft palate and cleft lip. In going over the literature thus assembled, it seems to me that there is probably no other field of surgery in which there is such a variance of opinion as to the best time for operation, the objective aimed at, the technique to be employed, and the final results of operation. Most authors in this field seem to agree that the clefts of the lip should be closed, and some agree that clefts of the palate should be closed too. Others seem to be satisfied to approximate the hemispheres of the hard palate without effecting union. The more indi vidual the operation and the easier its performance, the greater the tendency becomes to forget the primary objective where deformity exists-the restoration of normality as far as possible.

is unusual. 2. The thickened endocardium usually present at the site of congenital apertures forms a suitable soil for bacterial invasion. Horder considers this factor of prime importance, and points out that in the case of septal defects vegetations occur round the margins of the aperture and on the wall of the ventricle at the point upon which the stream of blood impinges. Local thickenings of the intima occur opposite the opening of a patent ductus arteriosus, both in the aorta and in the pulmonary artery, but it appears probable that the infective process begins at the pulmonary orifice of the ductus rather than at these thickened areas which in our case bore no vegetations. Brophyl gives the following current definition of cleft 3. Admixture of venous and arterial blood. The palate : "A congenital deformity, characterised by a frequency of left-sided endocarditis and the immunity fissure or fissures of the palate due to arrested developof the right side to infection has been attributed to ment." In other words, congenital cleft palate is the fact that arterial blood is the more favourable considered to be the result of incomplete development medium for bacterial growth. It is certainly true that of the tissues necessary to enter into its normal formadextral endocarditis is more common in those con- tion. " The opinions of authors thus expressed," Brophy genital lesions which allow arterial blood to reach the goes on to say," have been based, no doubt, on their right side of the heart. observation of the open space between the oral and nasal cavities. The error is easily explained, for the open space Summary. It is evident that local thickening of the endocardium suggests an absence of tissue. But the deformity, the and increased local strain are factors in determining statements of many authors to the contrary notwiththe onset of the infective process in congenital standing, is not the result of congenital deficiencies heart disease, but we suggest that the access of of the parts in question, nor is it due to arrested arterial blood to the right heart may explain the growth of the palate. All children who have congenital unusual frequence of dextral endocarditis in these cleft palate, with rare exceptions, have in the palate at birth the normal amount of tissue. The palatine cases. In our opinion the preponderance of left-sided lesions plates, however, are frequently misplaced upwards, in endocarditis in general cannot be adequately and the bones are not united in the middle line. The palate is cleft. Later in life it may atrophy for want explained by the higher blood pressure in the systemic of use. Therefore, a cleft palate is a fissure, a separawith are and we circulation, impressed the possibility that it may be due to the changed composition of the tion of well-developed parts, not (with rare exceptions)’ the result of arrested development nor failure of a blood after passing through the lungs. normal quantity of tissue to enter into its structure." We are indebted to Dr. C. E. Lakin for his kindness in allowing us to publish the details of the case formerly Most authors are now agreed that this is so, and, with this fact before us, it seems that the only logical course under his care. to pursue would be to preserve, as far as possible, all References. of this normal amount of tissue, assemble it into 1. Lewis, T., and Grant, R. T. : Heart, 1923, x., 21. correct relationship, freshen its edges, unite and 2. Osier, W. : Brit. Med. Jour., 1885, i., 467, 522, 577. immobilise it until healing occurs. Nevertheless, 3. Horder, T. J. : Quart. Jour. Med., 1908-9, ii., 289. 4. Washbourn, J. W. : Brit. Med. Jour., 1899, ii., 1269. surgeons who agree with and support the definisomeitself 5. Cowan, J., and Ritchie, W. T. : Diseases of the Heart, tion do not follow this logical course. Either 1922. the definition is forgotten in the subsequent operative 6. Glynn, T. R. : THE LANCET, 1903, i., 1007. 7. Abrahams, B.: Brit. Med. Jour., 1899, ii., 1273. procedure advised, or it is totally disregarded. 8. Libman, E., and Celler, H. L.: Amer. Jour. Med. Sci., 1910, There are many minor offences, but my object is 516. cxl., 9. Pitt, G. Newton : Allbutt and Rolleston’s System of to condemn the practice advised by certain authors Medicine, 1910, vol. vi., 310. relative to the disposition of the premaxillary bones 10. Hamilton, W. F., and Abbott, M. E. : Trans. Assoc. Amer. in the more severe forms of cleft palate. In these Phys., 1914, xxix., 294. 11. Fürth, K., and Weber, F, P. : Edin. Med. Jour., 1905, N.S., cases the premaxillary bones are usually protruded xviii., 33. for some distance anterior to the maxillse, making a 12. Dickinson, W. L. : Trans. Path Soc. Lond., 1897, xlviii., (Figs. 1 and 2.) Although very ugly deformity. 57. 13. Abbott, M. E.: Osier and McCrae’s Modern Medicine, 1915, these bones are protruded, and have the prolabium or vol. iv. central portion of the lip attached to them in front, 14. Durno, L., and Brown, W. Langdon : THE LANCET, 1908, it must be remembered that they form a part of the i., 1693. normal ununited tissue spoken of in the definition 15. Stokes, W. : Diseases of Heart and Aorta, Dublin, 1854. 16. Rogers, L.: Quart. Jour. Med., 1908-9, ii., 1. Yet we find repeated instances in the literature where the author recommends that these bones be excised UNIVERSITY COLLEGE HOSPITAL MEDICAL SOCIETY. and the lip closed, in the expectation that the maxillas The opening meeting of this society, at which Prof. J. S. will be moved more nearly together. To anyone who has a well-grounded education in Haldane, Oxford, is to read a paper on the Physiology of Acclimatisation to High Altitudes, has been postponed this special field of surgery, the excision of the pre from Friday, Oct. 17th, till the following Wednesday, Oct. 22nd, at 8.15 P.M. 1 T. W. Brophy: Oral Surgery. P 3 ,