- Email: [email protected]
Congenital malformations of the heart
CONGENITAL MALFORMATIONS OF T H E H E A R T T H E C L I N I C I A N ' S I~ESPONSIBILITY IN THE ~ELECTION OF t)ATIENTS FOR OPERATION
tIEr.EN B. TAUSSm, M.D. B ALTI1VIORE, MD.
tient. To do so wisely, the physician must not only be able to diagnose the nature of the malformation, but also know the risks, the dangers, and the amount of benefit to be derived from operation and balance these against the prognosis without operation. This is not always easy because the prognosis varies with the nature of the malformation and with the severity of the abnormality. For this reason it is a grave mistake to tell a patient or a parent that the child must have an operation or he will die. If operation is not indicated, it is difficult for the consultant to persuade the parents of the fact. If operation is not possible, irrevocable harm may have been done. The prognosis, without operation, is usually better than the doctor anticipates. Although some infants die from cardiac failure and some die from anoxemia, many infants survive innumerable attacks of paroxysmal dyspnea. Most infants survive respiratory infections and pneumonia. Children with mMformed hearts usually survive the common contagious diseases of childhood as well as do other children. Operation is only justified in infancy if life is precarious without operation. In general, as long as the infant is growing and gaining norreally there is tittle or no cause for concern. Failure to gain is often F r o m the H a r r i e t Lane H o m e of The J o h n s Hopkins Hospital and The D e p a r t m e n t of the first manifestation of difficulty. Pediatrics of The Johns Hopkins Hospital. 853
T
t i E advent of cardiac surgery brings new responsibilities to the clinician, not only because of the necessity for accurate diagnosis as to the nature of the malformation, but also in the selection of patients for surgery and in the prevention of needless operations. If the patient is but slightly incapacitated or entirely asymptomatic, in order to justify operation the risk must be minimal and the degree of benefit great. On the other hand, if the patient is "severely incapacitated and the prognosis without operation is bad, considerably greater operative risk, for even moderate benefit, is justified. Careful evaluation and sound advice to the patient are especially important, because in the last ten years there has been a complete change in the philosophy of patients and parents. Formerly there was grave hesitation concerning operations and there was widespread fear of " e x p e r i m e n t i n g " on a patient. Today patients frequently demand operation. They hear of the extraordinary successes. The dark sorrow of the background which makes the sueeess stand out brilliantly is frequently entirely overlooked or but poorly understood. T h e r e f o r e the physician has a great responsibility in the proper evaluation of his pa-
85~
THE JOURNAL OF PEDIATRICS
Cyanosis due to a malformation of the h e a r t is usually indicative of a serious, but not necessarily of a fatal, malformation. B r o a d l y s2eaking, the main causes of death in patients with congenital m a l f o r m a t i o n s of the h e a r t are cardiac failure, anoxemia, and occasionally cerebral thrombosis secondary to severe po]ycythemia. Form e r l y subacute bacterial endoearditis t o o k a h e a v y toll. This disease can f r e q u e n t l y be p r e v e n t e d by the administration of antibiotics or penicillin prior to and immediately following dental e x t r a c t i o n or tonsillect o m y ; furthermore, if the disease does occur, it is now eurabIe. Pulm o n a r y h y p e r t e n s i o n and abnormalities of the lungs m a y cause severe or even fatal cardiac strain, but discussion of these conditions is beyond the scope of this paper. Cardiac failure is almost invariably p r e c e d e d b y cardiac enlargement. F o r this reason in the noneyanotic group, where anoxemia and thrombosis p l a y no part, the size of the heart and the r a t e of cardiac enlargement are the two most i m p o r t a n t factors. Moreover it is i m p o r t a n t to r e m e m b e r that months are to an inf a n t ' s life as y e a r s are to a child of 10; t h e r e f o r e in i n f a n c y it requires close observation to determine the rate of enlargement or at least to be certain t h a t the heart is not rapidly increasing in size. F o r t u n a t e l y in m a n y instances, although immediately after birth the heart undergoes rapid enlargement due to the load placed on it by the e x t r a u t e r i n e circulation, once the heart has a d j u s t e d to the altered circulation the load placed oft it is a constant one, and enlargement comes
to an end. The heart of an infant is larger in proportion to the ehest t h a n is t h a t of a child or an adult; consequently growth is in the inf a n t ' s favor. E v e n though the heart m a y be enormously enlarged in infancy, the baby is not necessarily doomed to die at an early age. If the h e a r t does adjust to its load, slight Cardiac enlargement in child r e n need eause no great concern. B y the time a person has reached adult life, if he is free from symptoms and without any restriction of activity, with a h e a r t of normal size or even slight cardiac enlargement, he has only a slightly greater chance of death from heart disease t h a n does an individual with a normal heart. He is more subject to subacute bacterial endocarditis, but t h a t danger is seldom completely eradicated by operation and, as previously stated, the disease is now curable. Patients who show persistent eyanosis may die from anoxemia without cardiac failure and even with no cardiac enlargement. Therefore in such a patient the oxygen saturation
of the arterial blood is quite as imp o r t a n t as is the h e a r t size. In a n u m b e r of malformations, such as a t e t r a l o g y of Fallot, tricuspid atresia, and a single ventricle with pulmon a r y atresia, the malformation does not cause cardiac enlargement. Under such circumstances if death oc-
curs, it is ahnost invariably due to anoxemia. Moreover, f r e q u e n t l y an i n f a n t who has barely enough oxygen to sustain life, fails to eat and develops a severe anemia. Such an infant may show little or no eyanosis and yet suffer from a severe degree of oxygen unsaturation of the arterial blood. For this reason the
TAUSSIG:
CONGENITAL MALFORMATIONS OF HEART
i m p o r t a n t consideration is not the intensity of the eyanosis, but the sev e r i t y of the anoxemia. The oxygen s a t u r a t i o n of the arterial blood is of great prognostic importance provided it is p r o p e r l y evaluated. An o x y g e n saturation of the arterial blood of 30 per cent obtained u n d e r basal conditions is far more serious than if it is obtained when the i n f a n t is crying. Unfortunately there is an almost universal t e n d e n c y to r e g a r d an e x t r e m e l y low arterial o x y g e n s a t u r a t i o n as a technical error or to consider t h a t the sample must be that of venous blood, and consequently not significant. The findings of an o x y g e n saturation of 10 p e r cent or less in any blood sample t a k e n from an e x t r e m i t y should always be r e g a r d e d as a danger signal. I f it is a sample erroneously taken from a femoral or braehial vein, it is seriously low; if it is from arterial blood it is ominously low. The failure to realize how low the arterial oxygen saturation can be and failure to appreciate the d a n g e r of anoxemia is the reason w h y some infants with malformations of the heart die unexpectedly, In m y experience, most infants during an attack of loss of consciousness have an arterial oxygen s a t u r a t i o n of less t h a n 10 per cent. The corollary of this is also true, namely, if the i n f a n t suffers from attacks of paroxysmal dyspnea which progress to loss of consciousness, the arterial oxygen s a t u r a t i o n is usually e x t r e m e l y low; the condition is always serious. W h e n it is impossible to determine the arterial oxygen saturation, the best clinical guide to the infant's condition is weight gain, and whether or not he suffers from epi-
855
sodes of loss of consciousness. So long as the i n f a n t gains in weight at the normal rate there is usually no cause for concern. As previously mentioned, with loss of appetite and failure to gain, the infant f r e q u e n t l y develops an anemia and this in t u r n decreases the amount of available oxygen. T h e r e f o r e in the presence of persistent eyanosis or definite oxygen u n s a t u r a t i o n of the arterial blood, every effort should be made to maintain the hemoglobin at a level of 15 grams per 100 e.c. of circulating blood. W h e n the hemoglobin falls to 10 grams per 100 e.e. or lower, repeated small transfusions are usually indicated and m a y be life saving'. A n x-ray of the chest also offers i m p o r t a n t information. In the presence of persistent eyanosis, with m a r k e d r e d u c t i o n in the p u l m o n a r y blood flow, the lungs are excessively elear. P o l y c y t h e m i a and the development of eollateral circulation go hand in hand. Hence in the absence of p o l y e y t h e m i a i n e r e a s e d hilar markings are n e v e r due to collateral circulation. I f the hilar markings are increased and the red blood cell count is normal, the patient does not suffer from decreased p u l m o n a r y blood flow. U n d e r such circumstances a n y operation to increase the circulation to the lungs is contraindicated. Not i n f r e q u e n t l y an i n f a n t with a t e t r a l o g y of FMtot will show no cyanosis and have a normal weight gain for the first few months. Then between 3 to 6 months of age the inf a n t suffers front attacks of eyanosis or p a r o x y s m a l dyspnea. I f in spite of the attacks of cyanosis the infant
856
THE
JOURNAL
continues to gain weight, the outlook is usually good. If he ceases to gain weight, the condition is much more serious; early operation may be necessary. Loud murmurs are not usually of serious prognostic importance. Indeed, in the cyanotic infant a harsh systolic m u r m u r commonly carries a good prognosis, whereas the absence of a murmur carries a guarded prognosis. It is the cyanotic infant with a small heart, no murmur, and excessively clear lung fields who is in grave danger of dying from anoxemia. If he develops attacks of'paroxysmM dyspnea which progress to loss of consciousness, early operation is almost always indicated. Indeed, the condition is virtually a surgical emergency. So long as an attack of paroxysmal dyspnea is readily relieved by placing the infant in the knee-chest position, there is little danger of death from anoxemia. If the infant does not respond to this treatment, the inhalation of oxygen may help. If Oxygen does not give prompt relief, or if it is not available, morphine (1 rag. per 10 pounds of body weight) should be given. Most infants in whom the attacks of paroxysmal dyspnea and loss of consciousness are due to pulmonary stenosis respond to this treatment. If the infant requires daily administration of morphine, operation is indicated. In children with a tetralogy of Fallot, the degree of incapacity and the degree of polycythemia are the two most important factors in the consideration of operation, i f the patient shows only minimal cyanosis but can walk only a short distance without squatting, the probability is
OF
PEDIATRICS
that he suffers from a sharp reduction in the oxygen saturation of the arterial blood upon exercise. If such is the ease, he can usually be greatly helped by operation. An extreme polyeythemia, although it develops to compensate for anoxemia, increases the danger of thrombosis. Thromboses may occur anywhere; many are fatal and others cause severe incapacity, hemiplegia, or blindness. Therefore every precaution possible should be taken to prevent thrombosis. The danger may be eliminated by successful Surgery. Furthermore the results of operation are better for a child or youth than for an adult with longstanding polyeythemia. Moreover, the long-time results are far better for a patient who has obtained a good result from operation than for one who has only obtained a fair result. For this reason marked or increasing polyeythemia is an indication for operation. When operation is not possible, every effort should be made to main~ tain adequate fluid intake and to prevent dehydration, because this lessens the danger of hemoeoneentration and thereby lessens the danger of thrombosis. Because of this danger a child or adult with a red blood cell count of over 7 million should receive 2,500 to 4,000 c.e. of fluid every twenty-four hours and never be without fluid for more than twelve hours. This simple precaution does much to prevent cerebral thrombosis. Indeed, I do not think it is chance that the only two cyanotic patients whom I have known to become blind were French ehiMren. French children, by and large, receive a low fluid intake as they do not drink water and
TAUSSIG:
CONGENITAL MALFORMATIONS OF HEART
children are not given much wine, and they are rarely given milk. Pure pulmonary stenosis is another malformation which can be helped by surgery. Although a mild to moderate degree of pulmonary stenosis may be compatible with a long and active life, severe pulmon a r y stenosis may lead to cardiac failure and death within the first year. Infants with severe pulmonary stenosis generally show cyanosis at birth due to the physiological patency of the foramen ovale. The persistence or disappearance of cyanosis depends upon the extent to which the valve covering the foramen ovale becomes sealed. Patency of the foramen ovale acts as an escape valve for the high pressure in the right ventricle. Therefore there is greater danger of cardiac enlargement if the cyanosis is markedly reduced or disappears than if it persists. It is important to remember that this malformation may lead to, progressive cardiac enlargement. Hence, an infant suspected of this malformation should be kept under close observation for the first two years of life. Since cardiac enlargement precedes symptoms, even though the infant may appear healthy and gain weight normally, his heart may be undergoing progressive enlargement. Serial x-rays at monthly intervals are of great help in the evaluation of the infant's condition. If the heart becomes greatly enlarged or if the infant develops pulsations at the margin of the liver, early operation is indicated. I f the heart remains normal in size and the infant's color is good, it is wise to postpone operation. Such a patient
857
should be observed at regular intervals and special care be taken during the spurt of growth which occurs at puberty and during adolescence. In children and young adults, evidence of increase in heart size is an indication for operation. There are, however, all grades of pulmonary stenosis. N o t infrequently the condition is so mild that there is no evidence of cardiac enlargement or cardiac strain and the diagnosis is established by catheterization. If the pressure in the right ventricle is under 100 r a m . of mercury and the patient has a heart of normal size and is asymptomatic on full activity, operation is not indicated. Indeed, under such circumstances cutting the pulmonary valve may lead to pulmonary insufficiency or even to subsequent scarring of the pulmonary valves and eventually to an increase in the pulmonary stenosis; therefore, in my opinion operation for these patients is contraindicated. In the group of malformations which do not cause cyanosis, there are three; namely, a patent ductus arteriosus, coarctation of the aorta, and a vascular ring, which are now amenable to surgery. In each of these, operation may be of great benefit. An uncomplicated patent ductus arteriosus is virtually the only malformation in which operation is usually indicated. Even in this malformation if the heart is completely normal in size and there is no evidence of cardiac strain, I doubt that ligation or division of the ductus arteriosus is indicated in an infant under 2 years of age, or in an adult over 45 years. In the former in-
858
THE JOURNAL OF PEDIATRICS
stance the ductus arteriosus may yet close spontaneously; in the latter the danger of subacute bacterial endocarditis is virtually the only reason for operation. Although the danger of such an infection is markedly reduced by operation, we have not yet p~oved that with ligation or division of the ductus arteriosus both the aorta and the pulmonary artery heal so smoothly that the danger of subacute bacterial endocarditis is entirely eliminated. Indeed, even after successful ligation of the ductus arteriosus, I still advise a patient to receive prophylactic penicillin or other antibiotics prior to dental extraction or tonsillectomy. When we have a means of prevention, why take the slightest chance! Coarctation of the aorta is another malformation in which operation may restore the circulation to normal. Operation is only indicated in infancy if the infant develops cardiac failure which does not respond to digitalis and other medical treatment. In older patients severe hypertension, left ventricular strain, or aortic insufficiency are definite indications for operation. Although operation is easier and. the chance of success is better in childhood than in adult life, if the blood pressure is normal and there is no evidence of left ventricular strain, and the condition is picked up on routine examination, the risk of operation must be balanced against the possible future benefit. A vascular ring which causes symptoms in early infancy ~s virtually a surgical emergency, whereas if the condition is asymptomatie and is picked up on x-ray or fluoroscopy, there is no need for operation and no
benefit to be deriyed therefrom. The condition is compatible with a long and active life. It is important to remember that a cardiac operation is of benefit only to the heart and circulation. Lack of oxygen to the brain does not cause mental retardation. The converse of this is also true--improvement in the oxygen supply to the brain does not improve mentality. Furthermore, the physically strong, mentally weak child ean get into far more trouble in this world than the mentally deficient child who is also physically handicapped. The proper handling of the infant or child is important. If the mother is given a hopeless prognosis, she naturally and inevitably spoils the child and even though he may be rehabilitated at a later age, the psychologieal damage done is well nigh impossible to overcome. My social worker, Mrs. Mildred White Solomon, made a study of "how blue babies adjust to operation." She found the adjustment after operation varied with the adjustment before operation. My own experience is entirely in agreement with this observation; the well-adjusted, wisely handled, severely handicapped child blossoms after operation, whereas the spoiled child will seek other ways of demanding attention. Therefore, the more normal the child's life can be, the better it is for the child and the parent. Whenever possible the c h i l d should attend a regular school. He should be encouraged to play and develop his physical strength, i t is in eases of severe pulmonary stenosis or aortic stenosis that exercise may place a strain on the heart. In a
TAUSSIG:
CONGENITAL MALFORMATIONS OF HEART
patient with p u l m o n a r y stenosis, evidence of progressive cardiac enlargement is an indication for operation. In patients with aortic stenosis, since operation is not y e t possible, restriction of activity m a y be necessary. W i t h other malformations the patient usually limits his own activity; it is seldom necessary for the doctor or parent to do so. Furthermore, we should all remember that "we know not what is to come." Even though a malformation is not operable today, it may be amenable to surgery within the lifetime of that individual. For this reason the existence of a cardiac
malformation is seldom, if ever, justification for sterilization. Sad is the situation when a y o u n g woman is r e f e r r e d to the doctor with an operable malformation and the doctors say, " W e are glad to be able to restore the heart to n o r m a l , " and she
859
replies, " W h a t is the u s e ~ I can no longer have a c h i l d . " Finally, a word concerning the new operations which are col~stantly appearing on the horizon. If the patient is asymptomatic and the heart is not greatly enlarged, there is no need to urge a new operation. Such a patient is in no immediate danger. F u r t h e r m o r e , experience has shown that with any new operation the risk of s u r g e r y decreases sharply during the first few years. Therefore it is only the seriously incapacitated patient who should be submitted to a new operation. Let ~s point out the hope of the future. W h e n hope is near, wait its safe development; when hope is distant, make the mother realize that in spite of a physical h a n d i c a p m a n y individuals can lead happy, significant lives and take their place as respected, useful citizens in the world.
tIEr.EN B. TAUSSm, M.D. B ALTI1VIORE, MD.
tient. To do so wisely, the physician must not only be able to diagnose the nature of the malformation, but also know the risks, the dangers, and the amount of benefit to be derived from operation and balance these against the prognosis without operation. This is not always easy because the prognosis varies with the nature of the malformation and with the severity of the abnormality. For this reason it is a grave mistake to tell a patient or a parent that the child must have an operation or he will die. If operation is not indicated, it is difficult for the consultant to persuade the parents of the fact. If operation is not possible, irrevocable harm may have been done. The prognosis, without operation, is usually better than the doctor anticipates. Although some infants die from cardiac failure and some die from anoxemia, many infants survive innumerable attacks of paroxysmal dyspnea. Most infants survive respiratory infections and pneumonia. Children with mMformed hearts usually survive the common contagious diseases of childhood as well as do other children. Operation is only justified in infancy if life is precarious without operation. In general, as long as the infant is growing and gaining norreally there is tittle or no cause for concern. Failure to gain is often F r o m the H a r r i e t Lane H o m e of The J o h n s Hopkins Hospital and The D e p a r t m e n t of the first manifestation of difficulty. Pediatrics of The Johns Hopkins Hospital. 853
T
t i E advent of cardiac surgery brings new responsibilities to the clinician, not only because of the necessity for accurate diagnosis as to the nature of the malformation, but also in the selection of patients for surgery and in the prevention of needless operations. If the patient is but slightly incapacitated or entirely asymptomatic, in order to justify operation the risk must be minimal and the degree of benefit great. On the other hand, if the patient is "severely incapacitated and the prognosis without operation is bad, considerably greater operative risk, for even moderate benefit, is justified. Careful evaluation and sound advice to the patient are especially important, because in the last ten years there has been a complete change in the philosophy of patients and parents. Formerly there was grave hesitation concerning operations and there was widespread fear of " e x p e r i m e n t i n g " on a patient. Today patients frequently demand operation. They hear of the extraordinary successes. The dark sorrow of the background which makes the sueeess stand out brilliantly is frequently entirely overlooked or but poorly understood. T h e r e f o r e the physician has a great responsibility in the proper evaluation of his pa-
85~
THE JOURNAL OF PEDIATRICS
Cyanosis due to a malformation of the h e a r t is usually indicative of a serious, but not necessarily of a fatal, malformation. B r o a d l y s2eaking, the main causes of death in patients with congenital m a l f o r m a t i o n s of the h e a r t are cardiac failure, anoxemia, and occasionally cerebral thrombosis secondary to severe po]ycythemia. Form e r l y subacute bacterial endoearditis t o o k a h e a v y toll. This disease can f r e q u e n t l y be p r e v e n t e d by the administration of antibiotics or penicillin prior to and immediately following dental e x t r a c t i o n or tonsillect o m y ; furthermore, if the disease does occur, it is now eurabIe. Pulm o n a r y h y p e r t e n s i o n and abnormalities of the lungs m a y cause severe or even fatal cardiac strain, but discussion of these conditions is beyond the scope of this paper. Cardiac failure is almost invariably p r e c e d e d b y cardiac enlargement. F o r this reason in the noneyanotic group, where anoxemia and thrombosis p l a y no part, the size of the heart and the r a t e of cardiac enlargement are the two most i m p o r t a n t factors. Moreover it is i m p o r t a n t to r e m e m b e r that months are to an inf a n t ' s life as y e a r s are to a child of 10; t h e r e f o r e in i n f a n c y it requires close observation to determine the rate of enlargement or at least to be certain t h a t the heart is not rapidly increasing in size. F o r t u n a t e l y in m a n y instances, although immediately after birth the heart undergoes rapid enlargement due to the load placed on it by the e x t r a u t e r i n e circulation, once the heart has a d j u s t e d to the altered circulation the load placed oft it is a constant one, and enlargement comes
to an end. The heart of an infant is larger in proportion to the ehest t h a n is t h a t of a child or an adult; consequently growth is in the inf a n t ' s favor. E v e n though the heart m a y be enormously enlarged in infancy, the baby is not necessarily doomed to die at an early age. If the h e a r t does adjust to its load, slight Cardiac enlargement in child r e n need eause no great concern. B y the time a person has reached adult life, if he is free from symptoms and without any restriction of activity, with a h e a r t of normal size or even slight cardiac enlargement, he has only a slightly greater chance of death from heart disease t h a n does an individual with a normal heart. He is more subject to subacute bacterial endocarditis, but t h a t danger is seldom completely eradicated by operation and, as previously stated, the disease is now curable. Patients who show persistent eyanosis may die from anoxemia without cardiac failure and even with no cardiac enlargement. Therefore in such a patient the oxygen saturation
of the arterial blood is quite as imp o r t a n t as is the h e a r t size. In a n u m b e r of malformations, such as a t e t r a l o g y of Fallot, tricuspid atresia, and a single ventricle with pulmon a r y atresia, the malformation does not cause cardiac enlargement. Under such circumstances if death oc-
curs, it is ahnost invariably due to anoxemia. Moreover, f r e q u e n t l y an i n f a n t who has barely enough oxygen to sustain life, fails to eat and develops a severe anemia. Such an infant may show little or no eyanosis and yet suffer from a severe degree of oxygen unsaturation of the arterial blood. For this reason the
TAUSSIG:
CONGENITAL MALFORMATIONS OF HEART
i m p o r t a n t consideration is not the intensity of the eyanosis, but the sev e r i t y of the anoxemia. The oxygen s a t u r a t i o n of the arterial blood is of great prognostic importance provided it is p r o p e r l y evaluated. An o x y g e n saturation of the arterial blood of 30 per cent obtained u n d e r basal conditions is far more serious than if it is obtained when the i n f a n t is crying. Unfortunately there is an almost universal t e n d e n c y to r e g a r d an e x t r e m e l y low arterial o x y g e n s a t u r a t i o n as a technical error or to consider t h a t the sample must be that of venous blood, and consequently not significant. The findings of an o x y g e n saturation of 10 p e r cent or less in any blood sample t a k e n from an e x t r e m i t y should always be r e g a r d e d as a danger signal. I f it is a sample erroneously taken from a femoral or braehial vein, it is seriously low; if it is from arterial blood it is ominously low. The failure to realize how low the arterial oxygen saturation can be and failure to appreciate the d a n g e r of anoxemia is the reason w h y some infants with malformations of the heart die unexpectedly, In m y experience, most infants during an attack of loss of consciousness have an arterial oxygen s a t u r a t i o n of less t h a n 10 per cent. The corollary of this is also true, namely, if the i n f a n t suffers from attacks of paroxysmal dyspnea which progress to loss of consciousness, the arterial oxygen s a t u r a t i o n is usually e x t r e m e l y low; the condition is always serious. W h e n it is impossible to determine the arterial oxygen saturation, the best clinical guide to the infant's condition is weight gain, and whether or not he suffers from epi-
855
sodes of loss of consciousness. So long as the i n f a n t gains in weight at the normal rate there is usually no cause for concern. As previously mentioned, with loss of appetite and failure to gain, the infant f r e q u e n t l y develops an anemia and this in t u r n decreases the amount of available oxygen. T h e r e f o r e in the presence of persistent eyanosis or definite oxygen u n s a t u r a t i o n of the arterial blood, every effort should be made to maintain the hemoglobin at a level of 15 grams per 100 e.c. of circulating blood. W h e n the hemoglobin falls to 10 grams per 100 e.e. or lower, repeated small transfusions are usually indicated and m a y be life saving'. A n x-ray of the chest also offers i m p o r t a n t information. In the presence of persistent eyanosis, with m a r k e d r e d u c t i o n in the p u l m o n a r y blood flow, the lungs are excessively elear. P o l y c y t h e m i a and the development of eollateral circulation go hand in hand. Hence in the absence of p o l y e y t h e m i a i n e r e a s e d hilar markings are n e v e r due to collateral circulation. I f the hilar markings are increased and the red blood cell count is normal, the patient does not suffer from decreased p u l m o n a r y blood flow. U n d e r such circumstances a n y operation to increase the circulation to the lungs is contraindicated. Not i n f r e q u e n t l y an i n f a n t with a t e t r a l o g y of FMtot will show no cyanosis and have a normal weight gain for the first few months. Then between 3 to 6 months of age the inf a n t suffers front attacks of eyanosis or p a r o x y s m a l dyspnea. I f in spite of the attacks of cyanosis the infant
856
THE
JOURNAL
continues to gain weight, the outlook is usually good. If he ceases to gain weight, the condition is much more serious; early operation may be necessary. Loud murmurs are not usually of serious prognostic importance. Indeed, in the cyanotic infant a harsh systolic m u r m u r commonly carries a good prognosis, whereas the absence of a murmur carries a guarded prognosis. It is the cyanotic infant with a small heart, no murmur, and excessively clear lung fields who is in grave danger of dying from anoxemia. If he develops attacks of'paroxysmM dyspnea which progress to loss of consciousness, early operation is almost always indicated. Indeed, the condition is virtually a surgical emergency. So long as an attack of paroxysmal dyspnea is readily relieved by placing the infant in the knee-chest position, there is little danger of death from anoxemia. If the infant does not respond to this treatment, the inhalation of oxygen may help. If Oxygen does not give prompt relief, or if it is not available, morphine (1 rag. per 10 pounds of body weight) should be given. Most infants in whom the attacks of paroxysmal dyspnea and loss of consciousness are due to pulmonary stenosis respond to this treatment. If the infant requires daily administration of morphine, operation is indicated. In children with a tetralogy of Fallot, the degree of incapacity and the degree of polycythemia are the two most important factors in the consideration of operation, i f the patient shows only minimal cyanosis but can walk only a short distance without squatting, the probability is
OF
PEDIATRICS
that he suffers from a sharp reduction in the oxygen saturation of the arterial blood upon exercise. If such is the ease, he can usually be greatly helped by operation. An extreme polyeythemia, although it develops to compensate for anoxemia, increases the danger of thrombosis. Thromboses may occur anywhere; many are fatal and others cause severe incapacity, hemiplegia, or blindness. Therefore every precaution possible should be taken to prevent thrombosis. The danger may be eliminated by successful Surgery. Furthermore the results of operation are better for a child or youth than for an adult with longstanding polyeythemia. Moreover, the long-time results are far better for a patient who has obtained a good result from operation than for one who has only obtained a fair result. For this reason marked or increasing polyeythemia is an indication for operation. When operation is not possible, every effort should be made to main~ tain adequate fluid intake and to prevent dehydration, because this lessens the danger of hemoeoneentration and thereby lessens the danger of thrombosis. Because of this danger a child or adult with a red blood cell count of over 7 million should receive 2,500 to 4,000 c.e. of fluid every twenty-four hours and never be without fluid for more than twelve hours. This simple precaution does much to prevent cerebral thrombosis. Indeed, I do not think it is chance that the only two cyanotic patients whom I have known to become blind were French ehiMren. French children, by and large, receive a low fluid intake as they do not drink water and
TAUSSIG:
CONGENITAL MALFORMATIONS OF HEART
children are not given much wine, and they are rarely given milk. Pure pulmonary stenosis is another malformation which can be helped by surgery. Although a mild to moderate degree of pulmonary stenosis may be compatible with a long and active life, severe pulmon a r y stenosis may lead to cardiac failure and death within the first year. Infants with severe pulmonary stenosis generally show cyanosis at birth due to the physiological patency of the foramen ovale. The persistence or disappearance of cyanosis depends upon the extent to which the valve covering the foramen ovale becomes sealed. Patency of the foramen ovale acts as an escape valve for the high pressure in the right ventricle. Therefore there is greater danger of cardiac enlargement if the cyanosis is markedly reduced or disappears than if it persists. It is important to remember that this malformation may lead to, progressive cardiac enlargement. Hence, an infant suspected of this malformation should be kept under close observation for the first two years of life. Since cardiac enlargement precedes symptoms, even though the infant may appear healthy and gain weight normally, his heart may be undergoing progressive enlargement. Serial x-rays at monthly intervals are of great help in the evaluation of the infant's condition. If the heart becomes greatly enlarged or if the infant develops pulsations at the margin of the liver, early operation is indicated. I f the heart remains normal in size and the infant's color is good, it is wise to postpone operation. Such a patient
857
should be observed at regular intervals and special care be taken during the spurt of growth which occurs at puberty and during adolescence. In children and young adults, evidence of increase in heart size is an indication for operation. There are, however, all grades of pulmonary stenosis. N o t infrequently the condition is so mild that there is no evidence of cardiac enlargement or cardiac strain and the diagnosis is established by catheterization. If the pressure in the right ventricle is under 100 r a m . of mercury and the patient has a heart of normal size and is asymptomatic on full activity, operation is not indicated. Indeed, under such circumstances cutting the pulmonary valve may lead to pulmonary insufficiency or even to subsequent scarring of the pulmonary valves and eventually to an increase in the pulmonary stenosis; therefore, in my opinion operation for these patients is contraindicated. In the group of malformations which do not cause cyanosis, there are three; namely, a patent ductus arteriosus, coarctation of the aorta, and a vascular ring, which are now amenable to surgery. In each of these, operation may be of great benefit. An uncomplicated patent ductus arteriosus is virtually the only malformation in which operation is usually indicated. Even in this malformation if the heart is completely normal in size and there is no evidence of cardiac strain, I doubt that ligation or division of the ductus arteriosus is indicated in an infant under 2 years of age, or in an adult over 45 years. In the former in-
858
THE JOURNAL OF PEDIATRICS
stance the ductus arteriosus may yet close spontaneously; in the latter the danger of subacute bacterial endocarditis is virtually the only reason for operation. Although the danger of such an infection is markedly reduced by operation, we have not yet p~oved that with ligation or division of the ductus arteriosus both the aorta and the pulmonary artery heal so smoothly that the danger of subacute bacterial endocarditis is entirely eliminated. Indeed, even after successful ligation of the ductus arteriosus, I still advise a patient to receive prophylactic penicillin or other antibiotics prior to dental extraction or tonsillectomy. When we have a means of prevention, why take the slightest chance! Coarctation of the aorta is another malformation in which operation may restore the circulation to normal. Operation is only indicated in infancy if the infant develops cardiac failure which does not respond to digitalis and other medical treatment. In older patients severe hypertension, left ventricular strain, or aortic insufficiency are definite indications for operation. Although operation is easier and. the chance of success is better in childhood than in adult life, if the blood pressure is normal and there is no evidence of left ventricular strain, and the condition is picked up on routine examination, the risk of operation must be balanced against the possible future benefit. A vascular ring which causes symptoms in early infancy ~s virtually a surgical emergency, whereas if the condition is asymptomatie and is picked up on x-ray or fluoroscopy, there is no need for operation and no
benefit to be deriyed therefrom. The condition is compatible with a long and active life. It is important to remember that a cardiac operation is of benefit only to the heart and circulation. Lack of oxygen to the brain does not cause mental retardation. The converse of this is also true--improvement in the oxygen supply to the brain does not improve mentality. Furthermore, the physically strong, mentally weak child ean get into far more trouble in this world than the mentally deficient child who is also physically handicapped. The proper handling of the infant or child is important. If the mother is given a hopeless prognosis, she naturally and inevitably spoils the child and even though he may be rehabilitated at a later age, the psychologieal damage done is well nigh impossible to overcome. My social worker, Mrs. Mildred White Solomon, made a study of "how blue babies adjust to operation." She found the adjustment after operation varied with the adjustment before operation. My own experience is entirely in agreement with this observation; the well-adjusted, wisely handled, severely handicapped child blossoms after operation, whereas the spoiled child will seek other ways of demanding attention. Therefore, the more normal the child's life can be, the better it is for the child and the parent. Whenever possible the c h i l d should attend a regular school. He should be encouraged to play and develop his physical strength, i t is in eases of severe pulmonary stenosis or aortic stenosis that exercise may place a strain on the heart. In a
TAUSSIG:
CONGENITAL MALFORMATIONS OF HEART
patient with p u l m o n a r y stenosis, evidence of progressive cardiac enlargement is an indication for operation. In patients with aortic stenosis, since operation is not y e t possible, restriction of activity m a y be necessary. W i t h other malformations the patient usually limits his own activity; it is seldom necessary for the doctor or parent to do so. Furthermore, we should all remember that "we know not what is to come." Even though a malformation is not operable today, it may be amenable to surgery within the lifetime of that individual. For this reason the existence of a cardiac
malformation is seldom, if ever, justification for sterilization. Sad is the situation when a y o u n g woman is r e f e r r e d to the doctor with an operable malformation and the doctors say, " W e are glad to be able to restore the heart to n o r m a l , " and she
859
replies, " W h a t is the u s e ~ I can no longer have a c h i l d . " Finally, a word concerning the new operations which are col~stantly appearing on the horizon. If the patient is asymptomatic and the heart is not greatly enlarged, there is no need to urge a new operation. Such a patient is in no immediate danger. F u r t h e r m o r e , experience has shown that with any new operation the risk of s u r g e r y decreases sharply during the first few years. Therefore it is only the seriously incapacitated patient who should be submitted to a new operation. Let ~s point out the hope of the future. W h e n hope is near, wait its safe development; when hope is distant, make the mother realize that in spite of a physical h a n d i c a p m a n y individuals can lead happy, significant lives and take their place as respected, useful citizens in the world.