Personal View Future of paediatric cardiology and its patients

European Heart Journal (1997) 18, 198-207

Personal View

Future of paediatric cardiology and its patients

J. Somerville

Grown-Up Congenital Heart Unit, Royal Brompton Hospital, Sydney Street, London, U.K.

Introduction

A discussion about the future of anything — person,work, country, project — should start with an under-standing of how the present state has been reached bylooking at the past. Placed in the role of the Oracle ofDelphi, invited to prophesy on the future of paediatriccardiology, one is fortunate in having a shorter historyto survey, no payment for the prophesies and theinevitable criticism and rejection expected from themedical profession when change threatens! The chang-ing world of economics, technology and geopoliticsaffects something as small and unimportant as paediatriccardiology. Such fundamental changes profoundly influ-ence provision of health care, which should stimulate usurgently to examine our specialist work and future aims.Plans for our patients' care must be made withoutparochial attitudes and concern for our own success.The current period of superfluous bureaucracy in healthcare in the reformed (?deformed) National HealthService in the U.K. have meant that central funds forpatient care have been reduced by the introduction ofbusiness methods more appropriate for grocer's shopsthan for patients. Despite this, the necessary develop-ment of audit, analysis of outcomes and knowledge ofcosts are good and useful to the speciality. Unfortu-nately, use is made of 'Finished Consultant Episodes' asmeasures of success, promoted by politicians for theirown expediency, rather than true patient numbers andoutcomes which may not confirm the improvement ofservice. Paediatric cardiology, a small and expensivespecialty, with its few patients must feel threatened, so itmay make efforts to expand by 'taking' other contiguousspecialties without possessing the necessary expertise totreat the patients.

Key Words: Paediatric cardiology, economics, organisation.

Revision submitted 11 April 1996, and accepted 29 April 1996.

Based on invited lecture to First World Congress of PaediatricCardiology and Cardiac Surgery. Paris, 1993, and updated 1996.

Correspondence: Dr Jane Somerville, Grown-Up CongenitalHeart Unit. Royal Brompton Hospital. Sydney Street, LondonSW3 6NP, U.K.

Table 1 Topics discussed in the final plenary session ofthe World Congress of Paediatric Cardiology on 6th June1980

GeneticsC. CarterCellular/Genetic EngineeringB. Nadal-Ginard

Adolescent SurvivorsJ. Somerville

Cardiac TransplantationD. BaumDynamic Spatial ReconstructorE. Ritman

Final ThoughtsA. Castaneda

The specialty of paediatric cardiology would nothave been born in the 1960s without the challenge ofsuccessful cardiac surgery for congenital heart disease.When the present and future are viewed from theperspective where congenital heart disease accounts for90-95% of heart disease in children as in the 'West', it isless relevant to less affluent and less developed countrieswhere rheumatic fever is still more prevalent thancongenital heart disease. The courage, ingenuity and'macho' temperament of cardiac surgeons, when appliedto palliation and repair of congenital heart disease, hadto focus on new-borns and infants, where congenitalheart disease takes most toll. This stimulated the devel-opment of 'paediatric' including neonatal cardiology,the specialty separated from adult cardiology by defini-tion, department and practice with clear boundaries ofdemarcation. The coming of age of paediatric cardi-ology was the first World Congress of Paediatric Cardi-ology in London, 1980, probably the specialty's zenith.At the first plenary session on 'The Future' (Table 1) thesubjects showed the direction of the specialty and arerelevant 16 years later. Explosive technological develop-ments followed, which radically alter practice. Futureefforts should concentrate on preserving the achieve-ments of the past and the present high standards of

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practice and maintaining a human scientific specialtyguided by basic research.

Genetic engineering and molecular biology willmodify congenital heart disease, perhaps prevent it.Factors controlling myocardial hypertrophy and repairof cardiovascular damage are essential for therapy incongenital as well as acquired heart disease. Basic sci-ence research must, where financially possible, continuewithin paediatric cardiology. The major influences ofmolecular biology and genetic manipulation are morelikely to be on coronary artery disease — surely to beapplied in the paediatric period in those identified 'atrisk'. Who knows, in paediatric cardiology's need tosurvive the specialty might try to take this over as acongenital heart disease! This is not the only goodreason for encouraging closer development and integra-tion of paediatric and adult cardiology for its healthysurvival despite the restricted interests of most paediatriccardiologists.

The surgical treatment of congenital heart dis-ease in infants and neonates as early as possible, advo-cated and successfully practised by Castaneda et alP\should become every country's goal. It takes moreexpertise and resources to achieve this than may beavailable, but it should still remain the ideal even incountries where the specialty of paediatric cardiacsurgery is immature or unavailable. It may be better forpatients to have no service and a possibility of fairreferral outside than maintain an inadequate servicewith poor outcomes. It is sad to see now new paediatriccardiac surgical services not practising to up-to-datestandards, without audit of results or routine autopsy tolearn from.

The future will witness the principle of earlytreatment applied 'in utero', using a combination ofinterventional technology and surgical skills whichmodify structural congenital abnormalities and theensuing cascade of lesions in congenital cardiovascularanomalies in the live-born. The 1990s have witnessed thebeginning of this with a single 'success': an intra-uterineaortic valvotomy[2]. Subsequent World Congresses willcontain a plenary session on fetal cardiology andtherapy, that is if World Congresses survive — adebatable issue with regard to need, usefulness andcosts.

Since there is something potentially beneficial or'curative' to offer most congenital cardiac lesions, medi-cal concerns must address the effects on the function ofother systems, particularly the brain, minimizing adap-tive and destructive changes during bypass, therapy andhypoxia. The inseparability of paediatric cardiac surgeryand cardiology for research, clinical practice andtraining of junior staff and the importance of unifieddepartments'31 cannot be over-stressed. Following thefirst World Congress of Paediatric Cardiac Surgery(Bergamo, 1988) separate congresses were intended forthe future. Fortunately the foresight of a few sawpaediatric cardiology and cardiac surgery as equal part-ners, an important example for future practice inpatients and departments.

Cardiac transplantation in children is establishedand must remain a properly restricted practice. Wherefinancially and religiously possible (not in Japan) thisshould continue, controlled and audited to ensure, likeother management procedures, that it is cost effective. Itwill help to provide solutions to other immunologicaland donor problems of other system diseases, thuscontributing to medicine in general.

'Adolescent' cardiology, spoken of and estab-lished by 1980, has expanded to the new subspecialty ofGrown-Up Congenital Hearts (GUCH) with increasingnumbers of survivors and problems in adult life. Manyhave complex anomalies, once considered to be incom-patible with survival to normal adulthood, and there arenow grandparents with treated severe congenital cardiacdefects. The problems and management of GUCH aredifferent from those in children with congenital heartdisease. Keeping tadpoles does not give rights to ownand control frogs. The parent has to 'free' the child tohis/her own life once maturity is reached despite life-longinterest and concern. So there is the need for thepaediatric cardiologist to recognise the new medicaldemands of the adult congenital heart patient, notfulfillable by the paediatric doctor continuing care like aconcerned and over-protective parent.

Paediatric cardiology has flourished, but thereare storm clouds gathering warning us about the need tochange.

World paediatric cardiology

The 'developed' world concentrating on congenital heartdefects, is obsessively intrigued with the rare andcomplex, while other countries, less fortunate, ensurethe survival of a patient with an atrial septal defect.Examination of the work of units in different parts of theworld in the 1990s demonstrates the varying state ofdevelopment of paediatric cardiology.

Data from Boston — with the largest paediatriccardiac surgical practice (in 1990) — Bangkok andBuenos Aires (Figs l(a), (b) and (c)) show differences inthe lesions treated and incidence of anomalies. Thedifferences relate to referral patterns, experience andtechnology, resources, sophistication of medicine andnational priorities. Services for paediatric heart patientsvary from optimal 'up-to-date' fully financed to partlydeveloped, or absence of any specialized units. Onlyabout 15% of the world (Fig. 2) has the 'state-of-the-art'modern, ideal services to serve its population in part orwholly. As in the theory of chaos, the future for paedi-atric cardiology in the millennium will differ dependingon the starting state. Even in 'developed' countries withoptimal practice the service is not always uniformthroughout the country because of local factors, rivalry,variable professional expertise and personal interests. Itshould not be impossible for governments with organ-ised health services to correct this but to do so requiresguidelines of practice and careful audit. For a smallspecialty and easily identifiable patients it should be

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(a)AS

HLHSA-V canal

2: ASDCoarctationTGA switch

FontanFallotBDGVSD

ASD

VSD

Fallot

PDA

(c)

TGA switch

Homograft

Fontan

A-V canal

Fallot

ASD

VSD

I ]

20 40 60No. of procedures

80

0 20 40 60No. of patients

80

50 100No. of operations

150

Figure 1 (a) Surgery performed at the Boston Children'sHospital, Boston, U.S.A. in 1992. (Data provided by DrJim Lock.) (b) Surgery performed at the Children'sHospital, Bangkok, Thailand in 1992. (Data providedby Dr Choompol Vongprateep.) (c) Surgery performed atthe Hospital J.P. Garrahan, Buenos Aires, Argentina in1992. (Data provided by Dr Horacio Capelli.) AS=aorticstenosis; 2° ASD=secundum arrial septal defect; A-V=atrioventricular; BDG=bi-directional Glenn; HLHS =hypoplastic left heart syndrome; PDA=patent ductusarteriosus; PS=pulmonary stenosis; TGA=transpositionof the great arteries; VSD=ventricular septal defect.

urgently addressed and corrected in civilised countrieswith a national health service. This author believes thatthere should be centralization of expensive facilities, andconcentration of effort, expertise and patients. Europe,with an ability to invest in prospective audit of out-comes, already established by the CHSS of the U.S.A.,should make a concentrated effort and reduce thenumber of units treating congenital heart diseases.Agreement by doctors is always difficult to obtain and itwill be interesting to see if 'market forces' can rational-ize, restrict and organise specialist services for thebetterment of patients.

Influence of technology

The explosive development of technology in the 1980sbrought improved diagnosis and treatment to paediatriccardiology and profoundly altered practice. This willcontinue. Paediatric cardiologists and surgeons shouldmake plans for the metamorphosis. A beautiful butterflymay not emerge from the chrysalis of current paediatriccardiology, but if the needs of the patient are put beforethose of the specialist (unlikely), an improved, evencheaper service with better outcomes for children withheart disease could result.

Interventional therapy

When Bill Rashkind invented balloon atrial septos-tomy[41, the outlook for infants with transposition of thegreat arteries was revolutionized. Before his untimelydeath from malignant melanoma, he had produced adevice for closure of duct and defects. Since then inter-ventional therapy has nourished so that now manysimple cardiac lesions can be treated without surgery.The expansion and progress of interventional therapyeffects not only the practice but also the training ofpaediatric cardiac surgeons and cardiologists, as it haschanged adult cardiology and cardiac surgery.

Although the numbers of diagnostic invasivetests (cardiac catheterization) fell in the late 1970s andearly 1980s because of non-invasive diagnostic imaging,there has been an increase in therapeutic interventionoccupying longer time in the catheter laboratory. Non-surgical treatment for simple cardiac lesions reduces thenumber of cardiac operations and bed days in hospital,thus fewer beds for paediatric cardiology/surgery areneeded. This is further reduced by the day-case conceptfor investigation and treatment which will affect costs.As well as influencing manpower and training, fundingto pay for interventions necessitates transfer of moneyfrom surgery which will not be noticed if the depart-ments of paediatric cardiac surgery and medicine areone. Inevitably technology will increase the scope oftreatable lesions. Thus there is a likelihood that thepaediatric cardiac surgeon will treat only the complexanomalies and failed interventions, thus reducing thenation's need for paediatric cardiac surgeons andmaking their training difficult.


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Figure 2 Map of the world to show those countries with 'state of the art' services forpaediatric cardiology/cardiac surgery (•) and those with little or no specialized services in1995 (•).

The outcomes of interventions should be assessedin the way good cardiac surgeons look at their results ata national level. The national registry of proceduresshould be extended to include outcomes of all interven-tional and surgical procedures — so easily countablewith such small numbers. With few exceptions, the low-est mortality/morbidity in cardiac surgery, particularlywith complex anomalies and surgery, occurs in thecentres with the most experience, which is likely to be thesame with therapeutic catheter interventions. Experienceallows profit from mistakes and the establishment ofcorrect treatment protocols and advance of technology.'Occasionalists' in therapeutic intervention for congeni-tal heart disease should desist from 'having a go', i.e.every paediatric cardiologist should not expect to be aninterventionalist. Paediatric cardiac intervention, likecardiac surgery, is for highly trained super-specialistprofessionals and each country should ensure its practiceand design for paediatric cardiology encourages andeven enforces professionalism and centralization of theseactivities, dependent on the number of cases in the popu-lation. The scope of intervention will increase and theissue of training interventional paediatric cardiologistsand required manpower need to be looked at.

Several conditions, such as complex pulmonaryatresia, need an optimal combination of surgery andintervention. Interventional therapy should be consid-ered as an adjunct, not a rival, to cardiac surgery withmanagement strategies planned by both surgeon andphysician.

Imaging

Echocardiography, transthoracic and transoesophageal,are established diagnostic methods in paediatric and

adult cardiology. The scope of imaging has increasedwith new transoesophageal probes, both biplane andmultiplane and soon smaller probes for infants andchildren will be here with extension of routine use in theoperating theatre and intensive care. This will improverepairs of complex lesions and valves and thus theprognosis and lead to reduced complications and resi-dual lesions. Paediatric cardiologists need training intransoesophageal echocardiography. The mandatoryvisits to the operating room are vital for the paediatriccardiologist's knowledge of real anatomy rather thanimages, as well as witnessing the surgeon's problems,and should be part of paediatric cardiology training.Three-dimensional imaging will come and could, if nottoo expensive or demanding of time, become a usefulprocedure for complex lesions.

Magnetic resonance imaging (MRI) gives a beau-tiful display of anatomy as does spiral computer tomog-raphy, techniques particularly useful in older congenitalheart patients who can stay quietly and safely for thelength of time needed to acquire good images. Theseshould become more widely available for 'above Region'centres serving large populations. They obviate the needfor angiography in extracardiac vascular problems suchas coarctation, aneurysm and pulmonary artery distor-tions on still films or in cine mode. However, just becausethere is a machine available does not mean a 'generalist'imager can produce good diagnoses in such a specializedfield as congenital heart disease. The topical specialistmust be with the machine in the specialist centre.

For infants and small children where trans-thoracic echocardiography is so good, acquisition ofMRI is more difficult and anaesthesia may be necessary.The addition of velocity mapping for quantification ofobstruction and cine MRI for anatomy and physiologyare valuable. Technology improves daily, its use will be


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expanded, bring shorter acquisition times, better defini-tion and delineation of coronary arteries with the abilityto recognise early atheroma in children — probably itsgreatest value for the future. Cost will be the limitation.Different MRI spectroscopy for tissue characterizationshould be available in major centres, giving help inrecognition of obscure myocardial diseases, in changesduring bypass, in endocardial and endothelial reactionsand in effects of drugs and changing haemodynamics.Will the future imaging help identify and thus predictthose who might die suddenly?

Angioscopy and intra-arterial Doppler, whenadapted for use in small vessels, will be established inpaediatric cardiology, contributing knowledge of earlycoronary disease, enabling inspection of defects, andwill be useful for assessing interventional as well assurgical therapy and for looking for pulmonary arterythrombosis etc.

The technology which has done so much toadvance the specialty, namely echocardiography, mayprovide the instrument of its shrinking importance,almost to annihilation. This is so fundamental thatthe influence of fetal echocardiography is separatelyconsidered.

Fetal echocardiography

Fetal echocardiography can identify severe and complexcongenital heart lesions by 15-16 weeks' gestation, per-mitting the option of termination in many but not allcountries. Many lesions recognised early are the com-plex abnormalities which consume the most resource,time and expertise in paediatric cardiology. As technol-ogy and experience increase, earlier intra-uterine diag-nosis will be made, perhaps before 12 weeks. Once thedecision about termination can be made before oraround 3 months, it will be easier and less traumatic forthe mother and family to accept and choose termination.Expansion of this field is certain and paediatric cardi-ologists should become involved in several ways sincenew avenues of research, management and changes willopen. Blood tests may become available for detection ofcardiac abnormalities as they have for Down's syn-drome, which would be less demanding for the paediat-ric cardiologist. Fetal cardiology will affect practice andorganisation of paediatric cardiology, not only by reduc-ing the size and influence of the specialty, but byresearch and by providing an opportunity to understandhow and why lesions develop. Mothers who have chosennot to have termination will elect to be delivered close tothe centre where the baby can be treated early. Thisvaluable source of material for a unit will expandperinatal cardiology and encourage closer integration bythe paediatric cardiologist with obstetricians and neo-natologists. Intra-natal cardiology will develop the artand science of therapy for the fetus introduced throughthe mother or through interventional procedures. Ideallythe paediatric cardiologist should be involved, embrac-ing fetal cardiology and ensuring that there are a limited

number of properly supported and audited centres.Thus, a period in the obstetric department, and practiceinvolved in fetal echocardiography could be part ofthe training of paediatric cardiologists, otherwise thisintriguing field of research and practice may be lost tothem. In many systems this may not be practical.Obstetric screening should provide referral to a paediat-ric cardiologist who should assist in the training ofthe technicians. Intra-uterine interventions and surgerymay become feasible using the placental circulation forbypass.

The emerging specialty of fetal cardiology willteach us about cause and prevention of anomalies,cardiac muscle formation and more in as yet unchartedwaters. With the predictable reduction in paediatriccardiology patients, this new field should be absorbedand influenced by paediatric cardiology without delay.New departments and chairs should be created incombined fetal and paediatric cardiology.

There are relatively few fetal cardiac lesions inthe pregnant population. The need to scan about 500to detect one major cardiac abnormality means it islaborious. Special attention by the paediatric cardiolo-gist should be given to the mother with high risk, betterexpressed as 'higher than normal' risk of producing afetus with congenital anomalies. Despite the routine useof the four-chambered view in current obstetric fetalscreening, many serious cardiac lesions are missed byradiologists and technicians in the U.K.. Litigation inthis field is likely. Mothers already are aggrieved thatthey were not informed earlier of abnormality and/orgiven the choice of abortion or having therapy for theaffected fetus. The paediatric cardiologist should beavailable for the suspicious case and the at-risk mother,in whom diabetes, drug therapy and abuse, early virusinfection, lithium epilepsy, CHD in mother or father(less at risk) or sibling, may be a cause. Nothing canbe done for the fetus or mother if the lesion is notrecognised early.

There is also the fundamental question aboutwho should counsel the mother once the abnormality isdetected? The paediatric cardiologist should take on thisresponsibility if only to increase his/her referral base! Itcan be done well by the few GUCH doctors. Theoutcome of detected fetal cardiac abnormalities will beinfluenced by this advisor. In two similar populations ofmothers with a similar spectrum of lesions, Fermont'sgroup in Paris and Lindsey Allen's in London (Fig. 3),there were fewer terminations in France compared toLondon. This results from differences in the structure ofsociety and attitudes related to ethics, religion, finance,intelligence and education. It is interesting that in theUnited Kingdom now, babies with hypoplastic lefthearts are born almost exclusively to young (under 17)single mothers and to those from ethnic minorities whomay not receive advice or be allowed the option oftermination. The advice which a prospective mother ofan abnormal fetus receives depends so much on threeEs — the £yes, Experience and Emotions of the coun-sellor. Different professionals look at the same object

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(a) (b)

Figure 3 (a) Data from Dr Lindsey Allen, Guy's Hospital, London, for1991 to show the outcome of 201 fetal echocardiograms. (b) Data fromProfessor Fermont, Paris showing the outcome of 1008 fetal echocardio-grams. D=termination; H = intra-uterine death; • = neonatal death;S = alive.

with professed open-mindedness yet see something dif-ferent. This obvious fact of life relates to perceptionsabout success of therapy in congenital heart disease as itdoes the same about everything else. For instance, aninternationally renowned paediatric cardiac surgeonwhen asked how he would advise a mother carrying afetus with double inlet ventricle, stated that he would be'optimistic' as he was pleased with the results of totalcaval-pulmonary connection with 'staged' operations!This author, when asked, is gloomy, mentioning abnor-mal adulthood, never a normal heart, multiple surgicalprocedures and uncertainties and would encourage ter-mination, having established religious background andfather's views. Strenuous efforts to remove or reducethe mother's guilt should she choose the course oftermination must be made. These could be regarded asextremes of view about the same subject but they comefrom professionals with similar views on humanity anda large, long experience of congenital heart disease.The different perspectives of counsellors suggest thatalthough the paediatric cardiologist has an importantrole in counselling, someone from another disciplinemay be needed. The female physician without childrenmay have different perspectives from the male or femalewith children. The distress of rearing a chronicallyabnormal child is harrowing for the whole family andmay precipitate marital break-up. It is worse for thefemale who is the one generally accepting the long-termburden of the disabled child and coping with the dis-tressing loss in adolescence or young adulthood when itis too late to breed again. If allowed to choose, andmothers with emerging emancipation increasinglychoose abortion for serious lesions, the effect on thespecialty of paediatric cardiology and surgery will bedevastating by reducing the number of patients withcongenital heart diseases. A look at the cardiac surgicalwork from a major unit (Fig. 4) shows how muchactivity would be lost if mothers knew about the cardiacproblem in their unborn child and chose abortion.Already in the London area, Lindsey Allen's advice hasled to nearly 200 abortions for major cardiac anomaliesin 1991/1992. This could have kept one active supra-

regional unit in London occupied for a year, not tomention the recurrent procedures and care requiredduring the subsequent decades and into adult life.Since it is the severe lesions which are recognised, infetuses which abort both spontaneously and by choice,fewer paediatric cardiac units are and will be needed.Already there is reduction or absence of procedurewaiting lists — not always admitted. Not all womenwill choose abortion and many will not be given theopportunity.

Fetal echocardiography and cardiology providemany intriguing facets for paediatric cardiology. Thereluctance to accept fetal cardiac scanning into paedi-atric cardiology, particularly for the high-risk mother, isshort-sighted because its influence on the specialty andits importance in research should be enjoyed by paedi-atric cardiology, which has a large research potential.The impact of fetal cardiology must influence theplanning of a nation's future paediatric cardiologyor the effects on paediatric cardiac surgery could bedevastating.

Outcomes

Assessment of the efficacy of treatment for a child'scardiac anomaly too often is based on short-term, nowextended to 'mid-term' statistics, encouraged by a fre-quent belief in new surgical strategies. For instance, it isnot known whether Fontan type operations or caval-pulmonary connections are better in the long-term forcomplex cyanotic anomalies than a well constructedBlalock-Taussig shunt. There are theoretical argumentsto support the view that the former solution supersedesthe latter, but no evidence in the long-term, as judged bysurvival to adulthood with good quality of life followingoperation in infancy. This doubting author considersdeath with the late complications of Fontan circulationis more unpleasant and difficult to manage, indeed areal misery, than the consequences of chronic heartfailure from shunts. The aorto-pulmonary shunt volumeloads the ventricle so that it fails in a dilated state


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Tricuspid atresia

DORV

Pulm. atr. intact IVS

Pulm. atresia + VSD

20 40 60No. of operations

80

Figure 4 The surgical workload of the Hospital for Sick Children, Great OrmondStreet, London, in one year. Excluding VSD, if fetal echocardiography recognisedthe lesion and the mother chose abortion for all the serious lesions (shown in theshaded areas) there would be a considerable reduction in surgical workload. Thecross-hatched areas represent the number of patients/operations which would not beperformed had the pregnancy been terminated. Closed = operations performedwithout cardiopulmonary bypass, i.e. shunts, coarctation, banding of the pulmonaryartery; open=operations using cardiopulmonary bypass. Atr. = atresia; A-V =atrioventricular; DORV=double outlet right ventricle; IVS=interventricularseptum; Pulm. = pulmonary; TGA=transposition of the great arteries; VSD =ventricular septal defect.

but the Fontan/caval-pulmonary artery connectionschronically underfill and cause diastolic dysfunction inthe long-term with complications such as protein loss,thrombosis and extraordinarily high risk for cardiactransplantation.

Paediatric cardiologists/cardiac surgeons need tothink beyond the near and restrictive horizon of child-hood and consider the outcomes in adolescence andadulthood. There should be a reluctance to pronounceon excellence of therapy until these are established. Thenew medical community — the Grown-Up CongenitalHearts (GUCH) — ideally should be part of a con-tinuum of care for congenital heart disease, with liaisonbetween paediatric and adult cardiologists. Of coursetechniques have changed over three decades and today'sGUCH problems will not be tomorrow's. Actuarialsurvival curves give numbers but no idea of the state ofpatients and their problems. A focus on their ability tolead a normal life rather than on disability is important,hence the value of the Ability Index'51 for assessmentrather than NYHA classification.

An idea of the most at-risk lesions can be gainedfrom the study of incidence of problems occurring inolder patients (Figs 5(a) and (b)). Although improve-

ments are likely, today's problems must be noted andserve as a guide to counselling the mother with anabnormal fetus, the young adult with operated and un-operated complex disease, and long-term care arrange-ments for the adult patient with congenital heartdisease.

Paediatric cardiology needs to be familiar withoutcomes in the GUCH population and be prepared tochange management of the infant and child. The long-term and real outcomes of the interventions of the1980s-1990s are obviously unknown, but it is safe topredict that there will be a GUCH community in the21st century and provision must be made with a fewtrained to care with specialist expertise.

The medical profession, stimulated by paediatriccardiology, should organise the care structure for long-term supervision of grown-up patients with childhooddisease across the board. Government departments needto recognise and provide the continued funds for main-tenance once they are no longer paediatric patients anddeemed unattractive propositions for support by chari-ties and Departments of Health. Agencies will pay forthe sweet publicity-appealing blue baby but not whenhe grows up, still having major, expensive, medical

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(a)60

50

g 403

30

g 20

10

(b)100

80

"S 6 0

II 40E

20

ASD Ostium A-Vprimum canal

PVS TAPVD

Subaortic Aorticstenosis valvotomv

Fallot TGAMustard

Fontan

Figure 5 (a) and (b) Incidence of important problems age12-30 years in different congenital cardiac lesions oper-ated on under age 10 years. Major problems referredto are death, reoperation, endocarditis, symptomaticarrhythmias or intervention. ASD = atrial septal defect;A-V = atrioventricular; PVS = pulmonary valve stenosis;TAPVD=total anomalous pulmonary venous drainage;TGA = transposition of the great arteries.

problems. Paediatric cardiology and adult cardiologytogether should follow the 'ROCK' for the GUCHprinciple'6'; 'R' for Recognition is occurring but goodOrganisation is not. Paediatric cardiology in its shrink-ing importance considers it is almost its divine right tocontinue being responsible for adult congenital heartpatients, a view encouraged by the lack of interest orenthusiasm of adult cardiologists. The paediatric cardi-ologist is weak, without knowledge or training in man-agement of different problems in the adult, no trainingin adult medicine or cardiology or beds and skills tocope with the adult. Adult patients are not happy in theChildren's Hospital nor are there the personnel andfacilities available. The adult patient needs an adult

Table 2 Percentage of population with access to healthcare, safe water, adequate sanitation in countries ofvarious stages of development

% Population access to

Health careSafe water

Adequatesanitation

Least developedDevelopingDeveloped

4671

795-100

293998

Source. UNICEF.

cardiologist to be within that specialty and to have useof the 'tools and toys' of adult cardiology, just as thechild needs the paediatric environment with a trainedpaediatric cardiologist. Paediatrics have made such afuss and special pleading for so long about the specialneeds of the paediatric patient, that there is no justifica-tion for the paediatric cardiologist, having built thebarriers and boundaries, to ignore them in order toexpand this specialty.

The GUCH cardiologist can be trained in eitherbasic paediatric or adult cardiology. He or she needsknowledge of the practice of both, together with knowl-edge of management, of complications in complex con-ditions in the adult, causes of death and deterioration,how to investigate and when to refer for surgery. Theworld's few most successful and active GUCH Units areall in adult cardiology despite the difficulties of estab-lishing them. Solving long-term care problems has notbeen helped by such bodies as the Subcouncil forPaediatric Cardiology of the International Society andFederation of Cardiology stating ' . . . it is most inappro-priate for the Paediatric Cardiology group to be associ-ated with the Grown-Up Congenital Heart Disease unitin view of the fact that perspectives are so different.'Correct, but the aims should be the same. Such limitedhorizons of an international organisation trying to dic-tate policy do not bode well for the future of paediatriccardiology or its patients.

Finance — economics

As technology flourishes and man's ingenuity and exper-tise increase, the cost per capita of treating congenitalcardiac anomalies could rise. The factor 'time' is notused to cost treatment and should be. Cost assessment isusually restricted to one incident or operation, whichgives false ideas of benefit and value. The assessmentshould cover costs from birth to 16 years for patientswith specific anomalies. In the developed countries withrefined paediatric cardiology and surgical practice, theissues of economics are now important as the limitationof funds must balance the increasing cost of health. Forthe less affluent, fundamental problems in relation tohealth such as sanitation and clean water require more


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Table 3 Costs of surgery at the Hospital for SickChildren, Great Ormond Street in 1993

Type of surgery Cost (S)

Open heartNeonateInfant> 1 year simple> 1 year complex

ClosedHeart transplant+1 year

10 450947554208505

3500-700070 000

attention (Table 2). Paediatric cardiology and surgerycannot flourish where such fundamental problems exist.In those countries where clean water is available onlyto 50-60% of the population, the exotic treatmentsfor single ventricle and atresias are irrelevant. Manycountries cannot spend such money on saving a childwith a hypoplastic left heart or in undertaking infantcardiac transplantation. Where the need exists, $10 percapita for clean water is, and will be, a better investmentthan cardiac surgery for an exotic lesion in infancy oreven a paediatric cardiology unit. This, of course, willnot prevent them from being attempted. Indeed, at thefirst World Congress of Paediatric Cardiology in 1980,the session on the single ventricle was packed withdelegates while the simultaneous sessions on coarctationand hypertension were empty! Surveying the costs ofvarious procedures at a busy effective unit (Table 3) inthe United Kingdom in the 1990s, where the cost ofliving is high, gives an idea of how much per annum willbe needed for paediatric cardiology/surgery out of abudget for paediatrics, cardiac surgery, health alloca-tion, regional funds etc. and this is to treat a relativelyfew special patients. Lock and Freed have estimated thatone billion dollars is spent on congenital heart diseaseper annum — excusable because it is only 0-2% of thehealth budget! Where the under age 5 years mortalityrate exceeds 5%, cardiac surgery for congenital heartdisease is unlikely to be a priority, if considered at all. Asthe gross national product of a nation shrinks in reces-sion, the absolute size of the portion for the health of anation diminishes, even if the percentage for health isgranted an increase.

With paediatric cardiology and surgery function-ing as one department, costs automatically fall. Costlyattempts to save irreparable lesions may have to becurtailed lest those with better outcomes lose theirchance of an improved, longer life. Indeed the cost ofmass screening for fetal congenital heart disease may notbe worthwhile unless the obstetric technician looking forother things can be reliably trained to scan the heart. Itis the profession i.e. paediatric cardiologists and sur-geons who must decide and not the manager bureaucratswho cannot understand the complexity of heart diseasesor humanities — perhaps that is their advantage. Edu-cation of the future paediatric cardiologist must keepbasic economics in mind.

Organisation

Paediatric cardiology should be regarded as a small,expensive hot air balloon between the giants of paediat-rics and cardiology. In a few countries, mostly in thedeveloped world, it is accepted as a mono-specialtywhich means it has its own budget and autonomy whichhas helped its prosperity and growth. Whether this canbe maintained will depend on the sense and vision ofpaediatric cardiologists, so often inward looking with aclose horizon. In many countries, including Europe,paediatric cardiology is more often part of paediatricswhere there is a natural 'homing' because of the specialtraining and expertise, perceived to be necessary foroptimal care of infants and children. It is interesting thatthese special mandatory requirements are considerednow by some paediatric cardiologists to be enough tocare for adults.

Less frequently, paediatric cardiology is foundwithin cardiology or cardiac surgery, a situation whichhas advantages as well as disadvantages. The placing ofpaediatric cardiology raises important issues for itsfuture viabilty. Too often professorial departments ofpaediatrics are 'jealous' and resentful of the elitism andperceived extravagance of paediatric cardiology. Togeneral paediatricians, diarrhoea, infections, social prob-lems, neonatal care, allergy and 'mad' mothers are moreimportant. The tools of the paediatric cardiology tradeare expensive to buy and maintain and the overall cost ofpaediatric cardiology with a paediatric setting demandstoo large a part of the budget per capita. However,placed within cardiology and cardiac surgery, its share ofthe budget/resource is minute. Cardiology can maintainpaediatric cardiology, share its experience, tools and toyswith little pain and no envy — it barely notices. It isbetter to have enough to spend without being noticedthan being resented and made to beg. With adult cardi-ology containing the specialist trained for grown-upcongenital heart disease, this GUCH problem could beeasily solved. Such skills can be acquired with effort andtraining by paediatric or adult cardiologists. It is not anautomatic right for paediatric cardiologists.

The cost of staying within the limited horizons ofpaediatric departments and profession could be too highfor healthy survival of the specialty. Organisation ofpaediatric cardiology must integrate education, practiceand research with obstetrics, offering services to largematernity units. The changing emphasis of paediatriccardiology to the fetus and perinatal cardiology de-mands this for the healthy survival of the specialty. Mostvital is fusion with paediatric cardiac surgery, numeri-cally a diminishing need. The concept of a department offetal, paediatric cardiology and cardiac surgery could beattractive.

As the specialty of paediatric cardiology inevi-tably shrinks, it will be interesting to see who is attractedto it. In Douglas Moodie's study on 'Man'-power inpaediatric and adult cardiology in the U.S.A. (Fig. 6)the proportion of women in paediatric cardiologywas greater in paediatric cardiology than in the higher

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Adultcardiologyn = 15 998

Paediatriccardiology'n= 1017

Figure 6 The gender of adult and paediatric cardiologists in the U.S.A. in1991. D = males; •=females. (Data provided by Dr Douglas Moodie.)

earning, more influential specialty of adult cardiology.As paediatric cardiology becomes less financially re-warding, and thus less competitive, the proportion ofwomen in it will increase and perhaps more will beappointed as chiefs as well as worker bees!

Conclusions

Paediatric cardiology is an explosion started 30 yearsago by the fire of cardiac surgery. An explosion results inpieces (Table 4) and as such these parts now could be'annexed' by other hungry, interested specialties leavinglittle for the small hot air balloon of paediatric cardi-ology. This threat needs recognition. Small may bebeautiful but not necessarily strong or have an assuredsurvival. Whatever else happens, paediatric cardiology

Table 4 Components of paediatric cardiology work1995. Suggestions of who could do this in future

Paediatriccardiology work Done by?

CHD Infants. ChildrenEchoInterventionFetal echo/cardiology

(Including counselling)Acquired heart diseaseCHD adultsIntensive carePrevention degen. CVSBasic research

PCPC or Echo dept.PC or invasive card.PC

Card. + Paed. ?PCCard. ± PCPaed. Intensive or PC NeonateCard.Special

Card.=cardiologist; CHD=congenital heart disease; CVS =cardiovascular system; Dept. = Department; Paed. = paediatrician:PC = paediatric cardiologist.

and surgery must remain inextricably together, sharingnot only patients but also funds, training, educa-tion, research and ideals. Working closely with adultcardiology has advantages. Despite all the difficulties,there will be children with heart disease and motherswith heartache — both will continue to need a humaneservice, scientifically based, with available technologyand skill correctly applied and carefully centralized.

Fantastic advances serve our population. TheWorld Congresses focus on the current and latest tech-niques, out of reach for many countries. Many oftoday's solutions will be obsolete and thus can beignored by those trying to develop services. Not all thesteps of the flourishing units must be followed by thoseyoung in experience or not yet established. Paediatriccardiology take heed — change of views, forgettingvested interests, and sensible planning are urgentlyneeded, if paediatric cardiology is to survive.

References

[1] Castaneda AR, Mayer JE Jr, Jonas RA, Lock JE, Wessel DL,Hickey PR. The neonate with critical congenital heart disease:Repair — a surgical challenge. J Thorac Cardiovasc Surg 1989;98: 869-75.

[2] Maxwell D, Tynan M, Allen L. In utero valvotomy — amethod of prenatal treatment for critical aortic stenosis. BrHeart J 1991; 65: 256-8.

[3] Castaneda AR. The making of a cardiothoracic surgeon:An Appolonian quest. J Thorac Cardiovasc Surg 1994; 108:806-12

[4] Rashkind WJ, Miller WW. Creation of an atrial septal defectwithout thoracotomy A palliative approach to complete trans-position of the great arteries. JAMA 1966; 196: 991-2.

[5] Somerville J. Grown-up congenital heart disease. Cardiol Pract1991: 9: 4-5.

[6] Warnes CA, Somerville J. Tricuspid atresia in adolescents andadults: Current state and late complications. Br Heart J 1986;56: 535^3.


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