EDITORIALS
Overview of Pediatric Cardiology With a Critique of Congenital Heart Disease in the 1970's FORREST H. ADAMS, MD, FACC
Los Angeles, Cafifornia
The impetus for this critique is based upon several recent publications 1-3 as well as my own experiences and concerns in the field of cardiology. Its purpose is to bring into sharper focus some problems in pediatric cardiology and congenital heart disease that are not commonly discussed at scientific meetings. First, I wish to stress that a pediatric cardiologist should be trained in, and competent to care for, all cardiovascular problems that occur in infants, children and adolescents including congenital, acquired, metabolic and endocrine disorders. N a t u r e of the P r o b l e m
The incidence rate of congenital heart disease is known to be approximately 1 percent of all birthsJ From this, one can calculate that 30,000 infants with congenital heart disease are born in the United States each year. Such data permit ready comparison with data on other serious diseases affecting our population. The studies of Mitchell et al. 4 have shown that ventricular septal detect is by far the most common congenital heart lesion, accounting for 30 percent of cases; the remaining lesions each account for less than 10 percent. Such data permit assignment of priorities. Present knowledge of the natural history (without surgery) of the various congenital heart lesions is incomplete. For some of the severe but less common forms (hypoplastic left heart, tricuspid atresia) death during the neonatal period is expected. For some of the more common forms (ventricular and atrial septal defect, patent ductus arteriosus), neonatal deaths are rare, and From the Division of Cardiology, Department of Pediatrics, University of California, Los Angeles, California. This work was supported in part by funds from the U. S. Public Health Service, Bethesda, Maryland, Children's Bureau, Department of Health, Education and Welfare, Washington, D. C. and the Los Angeles Affiliate, American Heart Association, Los Angeles, California, Address for reprints: Forrest H. Adams, MD, the Division Of Cardiology, Department of Pediatrics, University of California, Los Angeles, California 90024.
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the condition of a sizable percentage of patients improves with time. Spontaneous closures of these defects have been described, but the mechanism of closure is only poorly understood. 5,6 Clinically, I find it difficult if not impossible to predict which patient's lesion will close, get smaller or become enlarged with time. R e l a t i o n of C o n g e n i t a l H e a r t D i s e a s e to Acquired Heart Disease
The incidence of congenital heart disease seems to be the same throughout the world. On the other hand, in developed countries, the incidence of rheumatic heart disease has been decreasing for many decades; the reduction began even before the discovery of antibiotic agents, thus suggesting the importance of social factors. 7 The developed countries now face two much larger problems in terms of the number of persons involved: atherosclerosis and hypertension. These two interrelated diseases, which account for more than 50 percent of all deaths in the United States, s clearly have their onset in the pediatric age range in many cases. 9 Thus, I ask: Should not pediatric cardiologists and others concerned with the heart in young children give more interest, time and energy to these two important problems? Diagnosis and T r e a t m e n t of Congenital Heart Diseases
Diagnosis and treatment of congenital heart disease be complex and difficult problems. They require a team effort by well qualified physicians, technicians, nurses, social workers and others in an institution that has sophisticated expensive equipmentJ TM All of these persons must be available 24 hours a day, 7 days a week, and it is expected that they and the facilities they use must meet certain minimal standards. 1°,11 Although many words have been written regarding the "development of an optimal plan" for the diagnosis and treatment of patients with heart disease, 1°,12-14 the can
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goal is far from implemented. Regional heart centers are nonexistent in most areas, and the maldistribution of cardiologists has been well documented. 13 On the other hand, The New England Regional Infant Cardiac Program for Congenital Heart Disease 15 has already demonstrated that a regional cooperative undertaking can work and is an effective and satisfactory way of caring for infants with heart disease. Regionalization of facilities and personnel is imperative now that care is so costly and a high level of expertise required. Nadas 16 has said that "for every 3 million population, there should be ohe major center that would see at least 100 infants a year." I agree with this general concept provided that geographic factors are also considered. If such a ratio is applied, a rough calculation suggests that in the United States, with a population of 210 million, 70 centers are needed. There may be two to three times that number! In California, for instance, the population is about 21 million, which suggests a need for 7 centers, but the California State Health Department has granted approval to 23 centers, more than three times the number needed. This has resulted in dilution of care at many centers with a concomitant increase in the unit cost for diagnosis. In addition to a plethora of heart centers in California, there is also a plethora of pediatric cardiologists; in 1974, of the 300 certified pediatric cardiologists in the United States, 56 were in California--twice the national average. I believe it is possible that an excess of doctors and facilities can lead to an abuse of the system in which the patient is seen more often than is indicated and unnecessary tests and procedures are performed. 2,3 Surgical considerations: Much of the effort of most pediatric cardiologists is spent in establishing the correct diagnosis of the patient with heart disease and then waiting for what is thought to be the best time for surgical palliation or total correction. Yet our ideas regarding the indications for surgery, the best time for surgery and the best technical procedure seem to chai~ge almost yearly. This reflects our changing concepts regarding the natural history of the various lesions, plus recent advances in surgical techniques, plus more extensive evaluation of the operative results. I am concerned about what we cardiologists say, imply or promise the patient with congenital heart disease or his or her parents. Except for the patient with patent ductus arteriosus, an implied promise of"curative surgery" is frequently a misstatement of the facts. The recent review of the surgical results in Houston by Morriss and McNamara 17 confirms my point. They have looked at their surgical results for some of the more common congenital heart lesions and have analyzed them in terms of residua, sequelae and complications. The need for improvement in surgical results should be obvious. Atrial septal defect: For this lesion the residua include a persistent shunt, cardiac enlargement, pulmonary valve gradient and mitral valve prolapse (6 percent); the sequelae include atrial arrhythmias. V e n t r i c u l a r septal defect: For this lesion the residuum is a persistent shunt in 30 to 40 percent. The main
sequela is a ventriculotomy scar with right bundle branch block; complications include complete heart block (4 percent) and aortic or tricuspid insufficiency. Aortic valve stenosis: For this lesion the residua are a persistent valve gradient in 60 percent and a tendency to valve calcification. The main sequela is aortic valve insufficiency (50 percent). Coarctation of the aorta: For this lesion the residua are several--a persistent gradient (10 percent), persistent systemic hypertension (9 percent), an aneurysm distal to the repair and in the brain, a bicuspid aortic valve (85 percent) and mitral valve lesions (6 percent). Sequelae include stenosis of the left subclavian artery and recoarctation; the main complication is paraplegia. T e t r a l o g y of Fallot: For this lesion residua are a persistent pulmonary arterial gradient (45 percent), a persistent shunt (20 to 40 percent) and obstruction distal to the pulmonary valve. Sequelae include a ventriculotomy scar, right bundle branch block, left anterior hemiblock, pulmonary valve insufficiency (70 to 100 percent) and aneurysm of the outflow patch. The main complication is complete heart block. P u l m o n a r y valve stenosis: For this lesion the residua are a persistent valve gradient and a persistent atrial shunt. The main sequela is pulmonary valve insufficiency (50 percent). Transposition of the great arteries: For this lesion the main residuum is the reverse function of the two ventricles; complications include arrhythmias (90 percent), venae caval obstruction (30 percent), pulmonary venous obstruction (30 percent) and tricuspid insufficiency. Another perspective is obtained if, to the postoperative evaluation, one adds studies of ventricular function with angiocardiography.18,19 Adding this bit of information to what is already known about operative mortality and residua brings us a step closer to an appraisal of the early postoperative results for the different heart lesions. My colleague Jay Jarmakani and I have attempted to include these data in our analysis of two common lesions, ventricular septal defect and tetralogy of Fallot (Table I). When this is done, only 60 percent of patients with ventricular septal defect and 20 percent of patients with tetralogy of Fallot have no residua!
TABLE I
Estimated Results After Surgery f o r Ventricular Septal Defect and Tetralogy of Fallot (% o f patients)
May 4, 1977
Operative mortality Significant residua Minor residua Abnormal function Normal function No residua
Ventricular Septal Defect
Tetralogy of Fallot
3 17
I0 25
10 10 60
20 25 20
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Long-Term Follow-Up of Patients With Congenital Heart Disease A statement needs to be made regarding the longterm follow-up of children and adults with congenital heart disease. Some general outlines have been published. 11 However, little specific information has been available until recently. 2° The group at Johns Hopkins Hospital studied the records of 1,924 adolescents and young adults with congenital heart disease seen at their institution over the past several decades. Their data give some notion of the types of lesion seen in the adult as well as of the distribution and types of long-term survivors. Of greater interest are some of the problems that arise in the young adult. 2° Mental retardation due to Down's syndrome or rubella was present in 6 percent. Other anomalies of the genitourinary tract or gastrointestinal tract or other organs were present in 10 percent. Problems related to psychologic adaptation or vocational planning, or both, were very important, as was counseling for marriage and pregnancy. Securing health and life insurance also remains a problem. Conclusions and Recommendations To offset the criticisms of the medical profession by Illich 2 and others, 1,31 believe we have to take a good look
at ourselves and "put our house in order." As a guide, I suggest that we pay attention to the analysis of Thomas, 1 who stated that medicine may be classified into three groups: high technology, halfway technology and nontechnology. 1 Examples of high technology include vaccines for poliomyelitis and diphtheria and hormones for endocrine disorders. Such high technologic advances have resulted from a general understanding of disease mechanisms, are not nearly as many as the public thinks and are inexpensive, simple and easy to administer. Halfway technology is what one does after the fact to postpone death--coronary bypass surgery, cancer chemotherapy and artificial or transplanted organs. Such technology is very expensive and requires extensive facilities. Nontechnology is what we generally call "supportive therapy" for far advanced disease such as multiple sclerosis, stroke, intractable cancer and severe rheumatoid disease. This type of technology also tends to be very expensive. Thus, my recommendations for the leaders in the field of congenital heart disease include (1) greater devotion to high technology research including research on etiology and prevention; (2) better understanding of the natural history and the consequences of intervention; (3) improvement in surgical treatment of some of the more common lesions; and (4) greater regionalization of manpower and facilities.
References 1. Thomas L: Guessing and knowing: reflections on the science and technology of medicine. Saturday Review 55:52-57, 1972 2. Illich I: Medical Nemesis: The Expropriation of Health. New York, Pantheon Books, 1976, New York 3. Knowles JH: The struggle to stay healthy. Time 108:60-62, 1976 4. Mitchell SC, Korones SB, Berendes HW: Congenital heart disease in 56,109 births: incidence and natural history. Circulation 43: 323-332, 1971 5. Evans JR, Rowe RD, Keith JD: Spontaneous closure of ventricular septal defect. Circulation 22:1044-1054, 1960 6. Cayler GC: Spontaneous functional closure of symptomatic atrial septal defects. N Engl J Med 276:65-73, 1967 7. Schwentker FF: The epidemiology of rheumatic fever. In, Rheumatic Fever (Thomas L, ed). Minneapolis, The University of Minnesota Press, 1952, p 17-27 8. Vital Statistics of the United States, 1969, Vol 2, Part A. Washington DC, US Department of Health, Education, and Welfare, 1969, p 6 9. Carter GA, Lauer RM: Atherosclerosis. In, Heart Disease in Infants, Children and Adolescents, second edition (Moss AJ, Adams FH, Emmanouilides GC, ed). Baltimore, Williams & Wilkins, in press 10. Engle MA, Adams FH, Betson C, et ah Resources for the optimal acute care of patients with congenital heart disease. Circulation 43:A 123-A133, 1971
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11. Engle MA, Adams FH, Betson C, et ah Resources for optimal long-term care of congenital heart disease. Circulation 44: A205-A-219, 1971 12. Rutstein D: Blueprint for Medical Care. Cambridge, Massachusetts, MIT Press, 1974 13. Adams FH, Mendenhall RC: Profile of the cardiologist: training and manpower requirements for the specialist in adult cardiovascular disease. Am J Cardiol 34:389-456, 1974 14. Adams FH: Who is a cardiologist? Am J Cardiol 35:761-762, 1975 15. Fyler DC, Parisi L, Berman M: The regionalization of infant cardiac care in New England. Cardiovasc Clin 4:340-356, 1972 16. Nadas AS: Leo M. Taran Memorial Lecture. Pediatric News 9:6, 1975 17. Morriss JH, McNamara DG: Residua, sequelae, and complications of surgery for congenital heart disease. Prog Cardiovasc Dis 18: 1-25, 1975 18. Jarmakani JMM, Graham TP Jr, Canent RV Jr, et al: The effect of corrective surgery on left heart volume and mass in children with ventricular septal defect. Am J Cardiol 27:254-258, 1971 19. Jarmakani JMM, Graham TP Jr, Canent RV Jr, el ah Left heart function in children with tetralogy of Faltot before and after palliative or corrective surgery. Circulation 46:478-490, 1972 20. Neill CA, Haroutunian LM: The adolescent and young adult with congenital heart disease. In Ref 9
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