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Mental retardation associated with congenital heart disease
MENTAL RETARDATION ASSOCIATED WITH CONGENITAL HEART DISEASE A STUDY
01,'TWENTY-Two CASES
IV[AB~L ROSS, M.D. BALTINIORE, ~{D. NCE congenital anomalies are known to occur in combinations rather ~J than singly, the question of possible association of mental retardation with congenital cardiac defects has been raised. I t is a well recognized fact that congenital heart disease is often found in mongolism. Cassel 1 reported eight cases in sixty (13.3 per cent), yon Hole 2 fourteen in 150 (9.3 per cent), and Hi!l, ~ between 10 and 20 per cent of mongolian idiots as showing congenital heart disease. A persistent defect of the atrial septum is the one most commonly reported. Cook and Millman ~ r e p o r t e d congenital anomalies in two low-grade imbeciles, who died at 3 years of age, and stated: " T h e coexistence of bicuspid valves and mental deficiency might reasonably be expected, for the occurrence of almost every congenital malformation has been noted in eases of amentia. Nevertheless, we have been unable to find any record of this association unless the ease of a hydroeephalic 8-month fetus be taken as a potential imbecile." The association of physical and mental retardation in school children has been repeatedly studied; but, oddly, tonsils and teeth received most of the attention, and heart conditions, either acquired or congenital, were rarely mentioned. Pope, ~ in a study of " P h y s i c a l Causes of Slighter Forms of Mental D e f e c t " in children in Leicester and London, found four out of seventy-six and six out of 151, respectively, with cardiac disease, and said, " I n a few cases the condition was congenital; in others it was due to r h e u m a t i s m . " Josephine Baker 6 investigated the physical condition of_ r e t a r d e d school children whose grade placement, was definitely behind the average. She found cardiac disease in 0.5 per cent of all pupils and in 2.3 per cent of the retarded ones. No attempt was made to separate congenital and acquired conditions. Beggs 7 mentioned "defects of the heart and b l o o d " in 5.1 per cent of 272 children studied in an investigation of conditions associated with retardation in elementary school children. Carter s found four cardiac lesions in 300 "problem children," and Eisler, 9 in an article concerning the relation of physical disease to behavior problems, mentioned two out of a hundred as showing mitral murmurs. In neither study was there mention of intellectual endowment or differentiation of congenital and acquired F r o m t h e C h i l d r e n ' s : P s y c h i a t r i c Clinic, H a r r i e t L a n e t I o m e f o r I n v a l i d Children, the Johns Hopkins Hospital. 21
22
~Hn JOURNAL OF PEDIATRICS
cardiac lesions. Smith ~~ felt that cyanosis at birth is a mark of delayed development and a sign of eventual mental retardation but gave no follow-up statistics to bear out this statement. Bostock ~ stated that in mental deficiency " p u l m o n a r y stenosis and congenital heart conditions are sometimes serious complications . . . . (and) circulation is p o o r . " H e gave no figures on the incidence of these findings. Stevenson, TM in a s t u d y of cardiovascular physiology in mental defectives, spoke of a "less stable circulatory response in lower feebleminded," but made no comment as to the actual anatomic findings in the circulatory system. Weber ~3 stated, "Dr. H. Morley Fletcher, however, tells me that congenital heart disease is not rare in imbecile children other than of the mongolian g r o u p . " U n f o r t u n a t e l y no paper of Fletcher's could be found in which he elaborated upon this statement. In the H a r r i e t Lane Home those working in the cardiac clinic noted that m a n y of the children being followed as having congenital heart lesions had marked difficulty in school and were often placed in special classes. This observation led to the question as to the incidence of ~nental retardation in patients with congenital cardiac 'disease. The cardiac and psychiatric clinics of the H a r r i e t Lane Home collaborated in an attempt to answer this question. The cases studied were limited according to age (not more than 14 years) by the admitting rules of the Dispensary, and, in order to have a reasonable expectation of accurate results by the Binet-Simon intelligence tests, no child less than 5 years of age was included. Otherwise no attempt was made to select the cases as to age, race, sex, or degree of cardiac defect. Twenty-two eases were seen, eleven boys and eleven girls ranging from 5 to 14 years of age. Of these, fifteen had been followed in the cardiac clinic, but the diagnosis was well checked in all. In these cases, patent interventricular septmn and patent duetus arteriosus were most common, occurring eight and six times, respectively. F o u r were diagnosed as patent interauricular septum, two as pulmonary stenosis, one as valvular disease, and in one the pathology was undetermined. In this group of twenty-two eases none fell in the idiot class but two had I.Q.'s below fifty, thus belonging to the imbecile group; seven fell in the moron group with I.Q.'s of fifty-one to seventy, and eight were in the dull normal group with I.Q.'s of seventy-one to ninety. Only four were within the normal range of ninety-one to 110, and the highest of the group had an I.Q. of 117. There is no relation to the type of lesion, as the two lowest and the one highest in the whole group had been diagnosed as having patent interventricular septum. This group was compared with a cross section of the H a r r i e t Lane Psychiatric Dispensary patients. One thousand eases within the same age range seen in the H a r r i e t Lane psychiatric clinic were felt to be fairly representative of the genera! outpatient population. F r o m the
ROSS:
MENTAL
RETARDATION ASSOCIATED WITH
HEART
DISEASE
23
findings in this thousand, one would expect that in twenty4wo there would be two with I.Q.'s below fifty, four in the moron group, nine in the dull normal, eight in the normal (91 to 110) and two between 111 and 120. Thus the extremes are approximately the same and the greatest percentage is in the dull normal group in both the cardiac and the general outpatient groups. The difference lies in the number to be found in the moron and the normal groups. This is best shown in Table I. A superimposed graph of the two groups shows this even more clearly (Fig. 1). It is thus seen that the dull normal range contains the same percentage in each category, but among those with congenital cardiac lesions //
8
Out-patient Cardiac
2
I
o50
51-
70
71-
91IIIII0 120
90 ~.Q. ' s Fig. 1.
the moron group was much larger and the normal group much smaller t h a n i s t r u e of a c r o s s s e c t i o n of t h e o u t p a t i e n t p o p u l a t i o n . In fact, e a c h is a l m o s t a m i r r o r i m a g e of t h e o t h e r . TABLE I I.
Q.
Under 50 51- 70 71- 90 91-110 111-120
OUTPATIENT
CARDIAC NO,
PER CENT
NO.
PER CENT
9 32 36.3 18.2 4.5
2 3 8 7 2
9 13.6 36.3 32 9
24
THE JOURNAl, OF PEDIATRICS
Two difficulties arise in attempting to reach any decisions on the basis of this investigation. First, the number studied is entirely too small to allow one to draw definite conclusions ; and, second, the difference is not large enough to permit one to discard the suggestion that it is primarily due to a chance selection of cases. Even so, one feels justified in saying that f r o m a comparison of the findings in twenty-two cases of eongenital heart disease with those in a cross section of the psychiatric outpatient population it appears that the intellectual endowment, as measured by the Bluet-Simon intelligence tests, tends to be somewhat lower in the group suffering from congenital heart disease. REFEREiNCES 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13.
Cassel: Berl. klin. Welmsehr. 54: 159, 1917. Yon tIofe, F. It.: Arch. Pediat. 39: 737, 1922. ttill, W. B.: Quart. J. 1Ked. 11: 49, 1908.. Cook, L. C., and Millman, C. G.: Lancet 1: 1018, 193g. Pope, 1~. M.: Lancet 2: 11, 1901. Baker, Josephine: Meal. Ree. 85: 64, t914. t~eggs, S. T.:. Practitioner 112: 380, 1924. Carter, W. E.: 1Vfent. I-Iyg. 10: 75, 1926. Eisler, E. R.: 1Vient. }Iyg. 10: 85, 1926. Smith, G. B.: Illinois l~I. J. 60: 346, 193]. ]gostock, J.: M. J. Australia 1: 255, 1937. Stevenson, G. S.: Arch. Neurot. & Psyehiat. 12: 507, 1924. Weber, }~. P.: B r i t . J. Child. ]Dis. 14: 269, 1917.
01,'TWENTY-Two CASES
IV[AB~L ROSS, M.D. BALTINIORE, ~{D. NCE congenital anomalies are known to occur in combinations rather ~J than singly, the question of possible association of mental retardation with congenital cardiac defects has been raised. I t is a well recognized fact that congenital heart disease is often found in mongolism. Cassel 1 reported eight cases in sixty (13.3 per cent), yon Hole 2 fourteen in 150 (9.3 per cent), and Hi!l, ~ between 10 and 20 per cent of mongolian idiots as showing congenital heart disease. A persistent defect of the atrial septum is the one most commonly reported. Cook and Millman ~ r e p o r t e d congenital anomalies in two low-grade imbeciles, who died at 3 years of age, and stated: " T h e coexistence of bicuspid valves and mental deficiency might reasonably be expected, for the occurrence of almost every congenital malformation has been noted in eases of amentia. Nevertheless, we have been unable to find any record of this association unless the ease of a hydroeephalic 8-month fetus be taken as a potential imbecile." The association of physical and mental retardation in school children has been repeatedly studied; but, oddly, tonsils and teeth received most of the attention, and heart conditions, either acquired or congenital, were rarely mentioned. Pope, ~ in a study of " P h y s i c a l Causes of Slighter Forms of Mental D e f e c t " in children in Leicester and London, found four out of seventy-six and six out of 151, respectively, with cardiac disease, and said, " I n a few cases the condition was congenital; in others it was due to r h e u m a t i s m . " Josephine Baker 6 investigated the physical condition of_ r e t a r d e d school children whose grade placement, was definitely behind the average. She found cardiac disease in 0.5 per cent of all pupils and in 2.3 per cent of the retarded ones. No attempt was made to separate congenital and acquired conditions. Beggs 7 mentioned "defects of the heart and b l o o d " in 5.1 per cent of 272 children studied in an investigation of conditions associated with retardation in elementary school children. Carter s found four cardiac lesions in 300 "problem children," and Eisler, 9 in an article concerning the relation of physical disease to behavior problems, mentioned two out of a hundred as showing mitral murmurs. In neither study was there mention of intellectual endowment or differentiation of congenital and acquired F r o m t h e C h i l d r e n ' s : P s y c h i a t r i c Clinic, H a r r i e t L a n e t I o m e f o r I n v a l i d Children, the Johns Hopkins Hospital. 21
22
~Hn JOURNAL OF PEDIATRICS
cardiac lesions. Smith ~~ felt that cyanosis at birth is a mark of delayed development and a sign of eventual mental retardation but gave no follow-up statistics to bear out this statement. Bostock ~ stated that in mental deficiency " p u l m o n a r y stenosis and congenital heart conditions are sometimes serious complications . . . . (and) circulation is p o o r . " H e gave no figures on the incidence of these findings. Stevenson, TM in a s t u d y of cardiovascular physiology in mental defectives, spoke of a "less stable circulatory response in lower feebleminded," but made no comment as to the actual anatomic findings in the circulatory system. Weber ~3 stated, "Dr. H. Morley Fletcher, however, tells me that congenital heart disease is not rare in imbecile children other than of the mongolian g r o u p . " U n f o r t u n a t e l y no paper of Fletcher's could be found in which he elaborated upon this statement. In the H a r r i e t Lane Home those working in the cardiac clinic noted that m a n y of the children being followed as having congenital heart lesions had marked difficulty in school and were often placed in special classes. This observation led to the question as to the incidence of ~nental retardation in patients with congenital cardiac 'disease. The cardiac and psychiatric clinics of the H a r r i e t Lane Home collaborated in an attempt to answer this question. The cases studied were limited according to age (not more than 14 years) by the admitting rules of the Dispensary, and, in order to have a reasonable expectation of accurate results by the Binet-Simon intelligence tests, no child less than 5 years of age was included. Otherwise no attempt was made to select the cases as to age, race, sex, or degree of cardiac defect. Twenty-two eases were seen, eleven boys and eleven girls ranging from 5 to 14 years of age. Of these, fifteen had been followed in the cardiac clinic, but the diagnosis was well checked in all. In these cases, patent interventricular septmn and patent duetus arteriosus were most common, occurring eight and six times, respectively. F o u r were diagnosed as patent interauricular septum, two as pulmonary stenosis, one as valvular disease, and in one the pathology was undetermined. In this group of twenty-two eases none fell in the idiot class but two had I.Q.'s below fifty, thus belonging to the imbecile group; seven fell in the moron group with I.Q.'s of fifty-one to seventy, and eight were in the dull normal group with I.Q.'s of seventy-one to ninety. Only four were within the normal range of ninety-one to 110, and the highest of the group had an I.Q. of 117. There is no relation to the type of lesion, as the two lowest and the one highest in the whole group had been diagnosed as having patent interventricular septum. This group was compared with a cross section of the H a r r i e t Lane Psychiatric Dispensary patients. One thousand eases within the same age range seen in the H a r r i e t Lane psychiatric clinic were felt to be fairly representative of the genera! outpatient population. F r o m the
ROSS:
MENTAL
RETARDATION ASSOCIATED WITH
HEART
DISEASE
23
findings in this thousand, one would expect that in twenty4wo there would be two with I.Q.'s below fifty, four in the moron group, nine in the dull normal, eight in the normal (91 to 110) and two between 111 and 120. Thus the extremes are approximately the same and the greatest percentage is in the dull normal group in both the cardiac and the general outpatient groups. The difference lies in the number to be found in the moron and the normal groups. This is best shown in Table I. A superimposed graph of the two groups shows this even more clearly (Fig. 1). It is thus seen that the dull normal range contains the same percentage in each category, but among those with congenital cardiac lesions //
8
Out-patient Cardiac
2
I
o50
51-
70
71-
91IIIII0 120
90 ~.Q. ' s Fig. 1.
the moron group was much larger and the normal group much smaller t h a n i s t r u e of a c r o s s s e c t i o n of t h e o u t p a t i e n t p o p u l a t i o n . In fact, e a c h is a l m o s t a m i r r o r i m a g e of t h e o t h e r . TABLE I I.
Q.
Under 50 51- 70 71- 90 91-110 111-120
OUTPATIENT
CARDIAC NO,
PER CENT
NO.
PER CENT
9 32 36.3 18.2 4.5
2 3 8 7 2
9 13.6 36.3 32 9
24
THE JOURNAl, OF PEDIATRICS
Two difficulties arise in attempting to reach any decisions on the basis of this investigation. First, the number studied is entirely too small to allow one to draw definite conclusions ; and, second, the difference is not large enough to permit one to discard the suggestion that it is primarily due to a chance selection of cases. Even so, one feels justified in saying that f r o m a comparison of the findings in twenty-two cases of eongenital heart disease with those in a cross section of the psychiatric outpatient population it appears that the intellectual endowment, as measured by the Bluet-Simon intelligence tests, tends to be somewhat lower in the group suffering from congenital heart disease. REFEREiNCES 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13.
Cassel: Berl. klin. Welmsehr. 54: 159, 1917. Yon tIofe, F. It.: Arch. Pediat. 39: 737, 1922. ttill, W. B.: Quart. J. 1Ked. 11: 49, 1908.. Cook, L. C., and Millman, C. G.: Lancet 1: 1018, 193g. Pope, 1~. M.: Lancet 2: 11, 1901. Baker, Josephine: Meal. Ree. 85: 64, t914. t~eggs, S. T.:. Practitioner 112: 380, 1924. Carter, W. E.: 1Vfent. I-Iyg. 10: 75, 1926. Eisler, E. R.: 1Vient. }Iyg. 10: 85, 1926. Smith, G. B.: Illinois l~I. J. 60: 346, 193]. ]gostock, J.: M. J. Australia 1: 255, 1937. Stevenson, G. S.: Arch. Neurot. & Psyehiat. 12: 507, 1924. Weber, }~. P.: B r i t . J. Child. ]Dis. 14: 269, 1917.