Archives ofDisease in Childhood 1996;75:96-101

Annotations

Does it matter if atrial septal defects are not diagnosed inchildhood?

Does it matter if atrial septal defects are not diagnosed inchildhood? It is only important to diagnose atrial septaldefect (ASD) in childhood if the defect carries an adverseprognosis that can safely and effectively be improved bytreatment. As this defect is largely asymptomatic duringchildhood, it is necessary also to know whether delayeddiagnosis and therefore treatment adds to the risks orprejudices the long term result.The three types ofASD are the ostium secundum, which

is by far the most common and is a defect in the region ofthe fossa ovalis, sinus venosus ASD sited at the junction ofthe superior vena cave and usually associated withanomalous drainage of the right upper lobe pulmonaryveins, and ostium primum defects. Ostium secundum andsinus venosus ASDs behave similarly and are usuallyisolated. Ostium primum ASDs, more properly calledpartial atrioventricular canal or endocardial cushiondefects, are sited inferiorly, are frequently complicated,and are usually diagnosed and surgically treated ininfancy or childhood. They are not the subject of thisannotation.

Isolated ASD is rarely diagnosed in the neonate becauseshunting is established only gradually. The left to rightshunt is dependent on the greater distensibility of the rightheart chambers and lower resistance of the pulmonarycompared with the systemic circuit. Pulmonary bloodflow gradually increases as the right ventricle thins afterfall of the high fetal pulmonary vascular resistance butpulmonary artery pressure remains normal. In the majorityof cases the defect is large, does not restrict flow, andthere is only a potential gradient between the low pressureleft and right atria. In a minority the defect remains smalland restrictive. Because there is no communicationbetween the high pressure left ventricle or aorta and thepulmonary circuit, pulmonary hypertension is never'obligatory' as in non-restrictive ventricular septal defectand the pulmonary artery pressure falls normally afterbirth.

PrevalenceASD is said to account for 10% of congenital heart diseaseat birth and for about 25% of adult patients with congeni-tal heart disease in whom it is the commonest congenitalcardiac defect after bicuspid aortic valve. It is two or threetimes more common in women and although more andmore ASDs are being diagnosed and closed duringchildhood, many are still only first identified in adult life oreven in old age. This is because the clinical features ofASDin the adult can be subtle. The diagnosis is easier inchildren. Most children, but not all adults, have obvioussigns and any child with a murmur is usually referred forechocardiography. In adults both symptoms and obviousmurmurs may be lacking and the diagnosis may never bemade.

DiagnosisMost children have a pulmonary systolic murmur, fixedsplitting of the second heart sound, and inspiratorymid-diastolic tricuspid flow murmur. The electrocardio-gram shows sinus rhythm with normal or rightward frontalQRS axis and partial right bundle branch block. This maybe undramatic but should always raise the possibility ofASD. Left axis deviation suggests a partial atrioventricularcanal (ostium primum) defect. The chest radiograph mayshow a barely enlarged heart but there is usually slightprominence of the right atrium and main pulmonary arteryand suggestion of pulmonary plethora. Echocardiographyis diagnostic and permits calculation of the right ventricu-lar and pulmonary artery pressures and the size of theshunt as well as direct visualisation of the defect andpulmonary venous connections. Cardiac catheterisation isunnecessary in children.

Natural historyTextbooks of cardiology continue to teach that in theabsence of pulmonary vascular disease all ASDs should beclosed, ' or that closure should be advised for all patientswith uncomplicated ASDs in whom there is evidence ofsignificant shunting, that is pulmonary to systemic flowratio (Qp:Qs) exceeding about 1.5:1.' This advice is basedon early perception of the natural history of the untreateddefect in the presurgical era and before echocardiographyhad begun to reveal a whole new population of ASDs,which had formerly not been diagnosed at all. The patientsdescribed in the early studies of the natural history ofASDwere recognised because they had the florid clinical andradiological evidence of heart disease that were thenneeded to justify cardiac catheterisation. They representedonly the worst end of the ASD spectrum. Both childrenand adults with uncomplicated and asymptomatic ASDwere regarded, even if seen by a cardiologist, as having mildpulmonary valve stenosis, 'idiopathic dilatation of the pul-monary artery', or innocent murmurs.ASD was thought to be associated with progressive mor-

bidity and greatly reduced survival. These opinions, whichare still quoted, '1 were not based on long follow upbecause ASD had only slowly begun to be recognisedclinically after papers by Bedford et al in 1941 4 and byWood after his St Cyres lectures in 1950.5 The fixed split-ting of the second heart sound was only published in1956.6 Lacking personal follow up of only recentlydiagnosed patients, cardiologists compared the prevalenceof symptoms and complications in the different age groupsand came to the conclusion that ASD had a relatively poorprognosis. Based on this apparently dismal outlook theearliest open heart surgery was practised on patients withASD whose defects could be closed quickly at a time whenspeed was all important under conditions of hypothermiccardiac arrest. Surgical repair on diagnosis became the rule

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Does it matter if atrial septal defects are not diagnosed in childhood?

in both children and adults. Because this practice has beenconscientiously followed since the late 1950s, all that isknown of the natural history of the untreated defect isbased on that early glimpse of a fragment of the ASDpopulation.The early writers believed that patients with ASDs were

likely to have a poor outcome. For example, Campbell et alcalculated an annual rate of attrition of4.5% for the fourthdecade, 5.4% for the fifth, and 7.5% for the sixth.7 8 Thatmeant that the average patient with ASD would not beexpected to live beyond the age of 50. Using the samemethods, Craig and Seltzer found that three quarters oftheir patients with ASD who were between the ages of 18and 56 were symptomatic. Pulmonary hypertension wasthe commonest adverse predictive factor (although only15% of their patients had a pulmonary vascular resistanceof over 5 Wood units).' Markman et al attributeddeterioration to the development of atrial fibrillation,recurrent bronchitis, pulmonary hypertension, and pulmo-nary infarction. They observed that the development ofheart failure was often associated with the onset of atrialfibrillation. Only 7% of patients in the third decadecompared with 30% in the fifth and sixth decades had apulmonary artery systolic pressure of over 50 mm Hg and60% of their patients aged 40 or over were in New YorkHealth Association (NYHA) classes III and IV, 30% by theage of 50 or over and 49% by 60 years.'0 The pulmonaryartery mean pressure was raised in only 14% of patientsunder 20 years compared with 53% of patients over 40years in the series of Hamilton et al."

Dyspnoea, fatigue, and palpitations are the mostcommon symptoms. Only 8% of children aged 1 to 10years in the study of Hamilton et al were symptomaticcompared with 35% of patients aged over 40 years. Theprogressive increase in prevalence of symptomatic limita-tion as age advances has been emphasised in most studies.Markman et al regarded pulmonary infection as a factorpredicting clinical deterioration, while Adams blamed pul-monary infection for the development of pulmonaryhypertension.'2 Unlike atrial arrhythmias, the developmentof pulmonary hypertension has not been related to shuntsize and it does not seem that a raised pulmonary bloodflow is the cause as this is not more than the cardiac outputon modest exercise in a normal person. Unlike other writ-ers, Craig and Seltzer concluded that younger adults aremore prone to develop this complication. They had prob-ably observed patients with anatomical ASDs andEisenmenger's syndrome who are frequently young, withsmall hearts, and no evidence that they have everdeveloped a high pulmonary blood flow. Wood's theorywas that there had been congenital persistence of the highfetal pulmonary vascular resistance after birth. Whateverthe reason for the association of a primary type pulmonaryhypertension with an anatomical ASD, the number of suchpatients included in the reports on the natural history ofASD will strongly bias the figures. These young patientsare quite different from the 5% of older patients with ASDwhose raised pulmonary vascular disease develops later inlife usually in association with atrial arrhythmia andprogressive tricuspid regurgitation. This tricuspid regur-gitant volume usurps shunted volume and right ventricularforward flow diminishes. The reduced flow velocity in thedilated pulmonary arteries leads to laminar thrombosisand repeated peripheral platelet emboli with gradually ris-ing pressure. This accounts for the observations ofMarkman et al of pulmonary infarction as a feature ofdeterioration in his pulmonary hypertensive older patients.In patients with severe disability pulmonary hypertensionis an important contributor. It is associated with clinicaldeterioration and poor life expectancy and was a feature in

82% of the patients of Gault et al who were in NYHAclasses III and IV compared with only 24% of those inclasses I and II.'Although children and young adults with ASD may have

considerable athletic ability and endurance, it has longbeen felt that left ventricular dysfunction contributes to thedevelopment of symptomatology.'4 Gault et al observedthat major right to left shunting is rare even in patients withpulmonary hypertension and suggested that this is becauseof increases in left ventricular diastolic stiffness with age orthat the development of left ventricular systolic dysfunc-tion might act to maintain left to right shunting. Right ven-tricular dilatation deforms the geometry of the left ventri-cle reducing its size, impairing diastolic compliance, anddiminishing left ventricular volume reserve, which is alsodiminished by inability to invoke the Starling mechanism.This may activate the renin-angiotensin system but anincreased left ventricular end diastolic pressure cannot begiven as evidence of left ventricular dysfunction because indiastole all four cardiac chambers are in open communica-tion and their pressures are similar. Recent studies haveshown normal left ventricular function after closure ofASD and that preoperative limitation of stroke volume isdue to reversible mechanical factors.'1'7

Small right to left shunts can be demonstrated even inuncomplicated ASDs and transient right to left shuntingwith paradoxical embolism has been blamed for otherwiseunexplained stroke in healthy adults with a patent foramenovale 18 or atrial septal aneurysm."The onset of atrial flutter or less commonly fibrillation,

nearly always marks a step down in the course of patientswith ASD and the incidence increases with age, beingfound in half of the surgical series of Sutton et al whosepatients were aged 60 years or over.20 Craig and Seltzerfound it to be a more important adverse prognostic featureeven than pulmonary hypertension. Atrial fibrillation ismost likely to develop in patients with both volume andpressure overloaded right ventricles. Its onset brings a riskofboth pulmonary and systemic embolism and these com-plications would explain Campbell's observation that mostpatients died within 5 or 10 years of its onset. Somepatients with ASD achieve considerable longevity even intotheir 90s.2' It would be helpful to recognise patients withASD programmed to reach old age without major disabil-ity despite this malformation.The value of surgical intervention in ASD has been

exhorted after comparison of the subsequent progress ofpatients after ASD closure with that of historic controlswith ASD described from a presurgery, pre-echocardiographic era.20 These were skewed populationsand such comparison is clearly invalid. More realisticallyMurphy et al studied 123 patients whose defects had beenclosed between 27 and 32 years earlier at the Mayo Clinicand compared them with age matched normal controls.22The actuarial survival rate among young patients aged 11to 24 years at the time of surgery was found to be similar tothe controls but the survival rate in patients whose defectswere closed when they were over age 25 years was lessgood. The survival of patients operated on when over age41 was considerably worse, 40% compared with 59% inthe controls. The excess mortality was due to anunchanged risk of onset of arrhythmias and continuedthroughout the follow up period due to the associationwith stroke and heart failure. The administration ofanticoagulants to patients who have started to developatrial arrhythmias is obviously very important.

People with ASD may remain asymptomatic or mini-mally symptomatic and undiagnosed until middle or laterage. At Hammersmith Hospital routine surgical closurehas not been the practice in such patients. A comparison of

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98 Oakley

the long term health of medically compared with surgicallytreated patients showed no difference between 34 patientsmanaged medically and 48 who had undergone surgicalrepair. 3 The two groups were similar with respect to age,functional class, and haemodynamic characteristics at thetime of entry which was before the echocardiographic era.There were no operative or perioperative deaths and aftera mean follow up of 25 years overall survival was more than90% in both groups whose mean age was 63 years in each.Symptomatic status, prevalence of atrial fibrillation, anduse of diuretics was also similar. Clearly the prognosis ofthe medically treated patients was much better than that ofthe historical controls described previously, although it wasnot normal but then nor was that of the group who hadundergone late surgical closure. This study was quitedifferent from that of Konstantinides et al who comparedthe long term course of 179 patients with isolated ASDdiagnosed after the age of 40 years, 84 of whom had theirdefects closed and 95 were followed up medically.24 Twentyper cent of the patients had initially been followed upmedically for up to 16 years before a decision was made tooperate on them. The mean follow up ranged from one to26 years (mean 8.9 years). The surgical mortality was2.8%. Surgical repair was found to increase survival andlimit functional deterioration but this favourable effect ofsurgical treatment was only prominent in the subgroup ofpatients with severe preoperative heart failure (NYHAclasses III or IV) among whom 69% had long termimprovement. This is not in contention. Such patients hadnot been included in the Hammersmith study of largelyasymptomatic ASDs.

Recent studies have revealed a new population ofpatients with ASD discovered through the growingapplication of echocardiography. Sixty nine patients aged70 years or older were described from the Mayo Clinicwith ages ranging between 70 and 93 years.25 Forty percent of the patients had not been suspected of having anASD before the echo study and over 40% were in NYHAclass I. Interestingly the strongest overall predictors of sur-vival included atrial fibrillation. The others were functionalclass, left ventricular ejection fraction, and pulmonaryartery systolic pressure. This observation makes sense.Although the onset of atrial arrhythmia often initiatestricuspid regurgitation, the development of congestive fea-tures and possibly pulmonary vascular disease, patientswith atrial fibrillation will continue to do well provided theonset of the arrhythmia has not been marked by the devel-opment of these features. An analysis of 289 cases ofASDaged 50 or over from Tokyo showed that nearly a third wereasymptomatic and a fifth had normal or nearly normalpulmonary artery pressures.26 A third of these patients hadshunt ratios of less than 2:1 and the authors concluded thatconservative management may be appropriate in asympto-matic older patients with normal pulmonary arterypressures and that ASD can be compatible with a normallife span.

Does it matter ifASDs are not diagnosed inchildhood?Closure of ASD in children and young adults recreates adependably normal or near normal prognosis with verylow mortality and morbidity (never 'negligible' or 'accept-able') for the price of a scar on the chest and in the knowl-edge that a number of defects will have been closedunnecessarily.The development of device closure by a pair of occlud-

ing discs may permit non-operative closure of smaller welldefined defects but these are probably the sort that cansafely be left alone. It is likely that smaller restrictive

defects with smaller shunts carry a better and even normalprognosis. There is no doubt that the more florid defectsdefined by heart size, (specifically right atrial and rightventricular volumes), defect and shunt size, should beclosed during childhood. Lesser defects need to be fullydocumented by defect size, chamber volume and shuntsize and then carefully followed so that the future need fortheir closure either by device or surgically can beascertained for future generations. A few adults mayhave more late morbidity with or without later closurethan they would have had if their defects had been closedearlier.The natural history ofASD is still known only in part.27

The defect does not invariably reduce health and activityor limit prognosis and routine closure is not ofproven ben-efit.28 Development of atrial arrhythmias is common inmiddle or later years and leads to increased morbidity butthe threat of thromboembolism can be reduced by use ofanticoagulants. The risk of late development of pulmonaryvascular disease is small but is a definite indication fordefect closure before it advances further.

Yes, it is better to diagnose ASD in childhood than tomiss it but there is no indication for closure ofasymptomatic ASDs first diagnosed in middle or oldage.

CELIA M OAKLEYDepartment of Cardiology,Hammersmith Hospital,Du Cane Road,London W12 ONN

1 Tynan M, Anderson RH. Congenital heart disease. In: Julian D, Camm J,Fox K, et al. Disease of the heart. 2nd Ed. London: Saunders, 1996:703.

2 Friedman WF. Congenital heart disease in infancy and childhood. In:Braunwald E, ed. Heart disease. A textbook of cardiovascular medicine. 4thEd. Philadelphia: Saunders, 1992: 908.

3 Perloff JK. Congenital heart disease in adults. In: Braunwald E, ed. Heartdisease. A textbook of cardiovascular medicine. 4th Ed. Philadelphia:Saunders, 1992: 968.

4 Bedford DE, Papp C, Parkinson J. Atrial septal defect. Br HeartJ 1941; 3:37-68.

5 Wood P. Congenital heart disease (St Cyres lectures). BMJ 1950; ii: 639and 693.

6 Leatham A, Gray I. Auscultatory and phonocardiographic signs of atrialseptal defect. BrHeartJ 1956; 18: 193-6.

7 Campbell M, Neill C, Suzman S. The prognosis of atrial septal defect. BMJ1957; i: 1375-83.

8 Campbell M. Natural history of atrial septal defect. Br Heart J 1970; 32:820-6.

9 Craig RJ, Selzer A. Natural history and prognosis of atrial septal defect. Cir-culation 1968; 37: 805-15.

10 Markman P, Howitt G, Wade EG. Atrial septal defect in the middle-agedand elderly. Q J Med 1965; 34: 409-26.

11 Hamilton WT, Haffajee CI, Dalen JE, Dexter L, Nadas AS. Atrial septaldefect secundum type: clinical profile with physiologic correlates inchildren and adults. In: Roberts WC, ed. Adult congenital heart disease.Philadelphia: Davis, 1987: 395-407.

12 Adams C. A reappraisal of life expectancy with atrial septal defect of thesecundum type. Diseases of the Chest 1965; 48: 357-75.

13 Gault JH, Morrow AG, Gay WA, Ross J. Arial septal defect over the age offorty years: clinical and hemodynamic studies and the effects of operation.Circulation 1968; 37: 261-72.

14 Dexter L. Atrial septal defect. Br HeartJ 1956; 18: 209-25.15 Bonow RO, Borer JS, Rosing DR, Bacharach SL, Green MV, Kent KM. Left

ventricular functional reserve in adult patients with atrial septal defect:pre- and post-operative studies. Circulation 1981; 63: 1315-22.

16 Carabello BA, Gash A, Mayers D, SpannJF. Normal left ventricular systolicfunction in adults with atrial septal defect and left heart failure. Am J Car-diol 1982; 49: 1868-73.

17 Booth KM, Wisenbaugh T, Smith M, De Maria AN. Left ventricular disten-sibility and passive elastic stiffness in atrial septal defect. JAm Coll Cardiol1988; 12: 1231-6.

18 Lynch J, Schuchard GH, Gross CM, Wann L. Prevalence of right to leftatrial shunting in a healthy population: detection by valsalva manoeuvrecontrast echocardiography. Am J Cardiol 1984; 53: 1478-80.

19 MarazanofM, Roudaut R, Cohen A, et al. Atrial septal aneurysm. Morpho-logical characteristics in a large population: pathological associations. AFrench multicenter study on 259 patients investigated by transoesophagealechocardiography. IntJ Cardiol 1995; 52: 59-65.

20 St John Sutton MG, Tajikr AG, McGoon DC. A septal defect in patientsaged 60 years or older: operative results and long-term follow-up. Circula-tion 1981; 64: 402-9.

21 Perloff JK. Ostiunm secundum atrial septal defect-survival for 87 and 94years.AmJffCardiol 1984; 53: 388-9.

22 Murphy JG, Gersh BJ, McGoon MD, et al. Long-term outcome aftersurgical repair of isolated atrial septal defect. N Engl J Med 1990; 323:1645-50.

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23 Shah D, Azhar M, Oakley CM, Cleland JGF, Nihoyannopoulos P. Naturalhistory of secundum atrial septal defect in adults after medical or surgicaltreatment: a historical prospective study. Br HeartJ 1994; 71: 224-8.

24 Konstantinides S, Geibel A, Olschewski M, et al. A comparison of surgicaland medical therapy for atrial septal defect in adults. N Engl J Med 1995;333: 469-73.

25 Foster-Smith KW, Murphy JG, Bailey KR, et al. Secundum atrial septaldefect (ASD): clinical profile and surgical experience in septuagenariansand older. Proceedings of the American CoUlege ofCardiology Meeting. Atlanta,Georgia, 1994; 465A: 826-32.

26 Ueda K. Clinical profiles, pathologic spectrum and management of atrialseptal defect in patients aged 50 or over: how should they be treated? JfCardiogr 1984; 14: 129-36.

27 Konstantinides S, Geibel A, Kasper W, et al. The natural course of atrialseptal defect in adults-a still unsettled issue. Kin Wochenschr 1991; 69:506-10.

28 Ward C. Secundum atrial septal defect: routine surgical treatment is not ofproven benefit. Br HeartJ 1994; 71: 219-23.

Cystic fibrosis in teenagers and young adults

The millennium will see over 6000 patients with cysticfibrosis in the UK, half of whom will be teenagers andadults, the over 15 age group increasing by about 120 peryear.' We are not providing sufficient resources for this lat-ter group now, nor are we making sufficient commitmentto their future needs. There are three reasons for this.Firstly, some fault rests with the apparent failure of theNHS hospital trusts' management to deploy incomederived from purchaser provision for cystic fibrosis careback into the cystic fibrosis service. There is also their per-sistent failure to meet the staffing levels recommended forcystic fibrosis centres by the British Paediatric Association(BPA), the British Thoracic Society, the Cystic FibrosisTrust,2 and the Royal College of Physicians' and theiralleged failure to have established a realistic cost for treat-ments. Secondly, there are a significant minority of profes-sional colleagues who refuse to refer older patients withthis multisystem disease to cystic fibrosis centres withappropriate multidisciplinary teams. Finally, some faultalso rests with those of us who have a major commitmentto cystic fibrosis care failing to initiate a proper trainingprogramme with recognised accreditation for juniordoctors wishing to care for patients with cystic fibrosis.

Special needs ofthe teenage patient with cysticfibrosis: the 'transitional' clinicInevitably close ties develop between the paediatric medi-cal and nursing teams and the patients and their families.Important though these are in giving the child and familyemotional security, they must be loosened and eventuallybroken to allow the patient to grow into independence, andrealise responsibility for self. Teenagers with cystic fibrosiscan only grow into adulthood in an environment designedto cultivate their independence. Close collaborationbetween paediatric and adult cystic fibrosis teams is essen-tial so that a system of care is established within which thepatient's transition through the turbulence of adolescenceis as smooth as possible. In Leeds we believe that this isbest achieved in a flexible 'transitional' clinic, run by stafffrom both the paediatric and adult teams. Patients usuallytransfer from paediatric to transitional clinic at about 14years of age, and from transitional to adult clinic,according to individual needs, between 16 and 18 years ofage. A survey of our own patients showed that knowledgeof the transitional clinic had reduced patients' anxietiesabout leaving the paediatric unit, and getting to know theadult carers, before moving on to the adult clinic proper,had reassured them.4

Cystic fibrosis centres for young adultsThe teenager with cystic fibrosis must be allowed to leavethe paediatric unit and the paediatrician behind. There isno justification for the paediatrician retaining the reins ofcare either because of the spurious excuse that no adultrespiratory specialist will give the level of commitmentrequired, or to maintain 'patient numbers' in the NHS

market economy. Nor should 'adult' clinics run by apaediatrician and chest physician be seen as fulfilling aneed outside of the transitional clinic referred to above.The paediatrician has to 'let go'. But responsibility forcontinuing care must involve an adult cystic fibrosis centre.If, as paediatricians, we accept the argument for centredcare for children with cystic fibrosis, then similarly we mustaccept centred care for adult patients.Some with responsibility for older patients with cystic

fibrosis believe that all of them should receive total care atthe cystic fibrosis centre. Why? Because cystic fibrosis is amultisystem, complex disease and, with present highstandards of care in paediatric centres, multisystemcomplications are often confined to adults. Hence, not onlymust a critical mass of experience be learned so that one isalerted to complications early, but colleagues in many dis-ciplines (for example obstetrics and gynaecology, endo-crinology, rheumatology, gastroenterology, surgery), mustbecome familiar with the problems presented in their ownfields by adult patients with cystic fibrosis. A sufficientpatient 'through-put' can take place only in a cystic fibrosiscentre. Access to specialist paramedical personnel and tobasic, not esoteric, investigations is significantly easierwithin specialist clinics.5 Patients cared for by cystic fibro-sis specialist staff receive more intensive treatment,especially with regard to nutrition, and are significantlymore likely to adhere to physiotherapy regimens and to beself reliant. Physicians who believe that they can offer opti-mal care by looking after a few patients with a physiothera-pist, dietitian, and social worker unschooled in the specialneeds of cystic fibrosis, are wrong. Units which believe thatthey can offer a safe and optimal home based intravenousantibiotic treatment programme without a cystic fibrosisliaison nurse are wrong. Young adult patients have voicedclearly their concerns about the fall in quality service theyhave experienced on transfer from specialist paediatriccystic fibrosis units to general adult chest clinics.5There has been a marked increase over the last five years

in the numbers of older patients attending specialistclinics. Seventy seven per cent now receive full or sharedcare from a cystic fibrosis centre. Ninety eight per centwant some input to their care from a specialist centre and96% of patients not currently receiving centre care wouldprefer it if they were.5 Existing centres must be properlyresourced to cope with these rapidly increasing numbers,and new centres set up in areas of need.

What an adult centre must offerFirst and foremost the centre must be adequately staffedand have access to adequate bed numbers, preferably on adedicated cystic fibrosis ward. It is unlikely that anyoneother than a full time cystic fibrosis physician will be ableto care properly for more than 100 patients. Thereforemost large units will require two consultants. Teenagersand adults should have easy access to significant amountsof their doctor's time. Issues such as career choices, life

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