Treatment and outcome of serious structural congenital heart disease

Semin Neonatol 2001; 6: 37–47 doi:10.1053/siny.2000.0033, available online at http://www.idealibrary.com on

Treatment and outcome of serious structural congenital heart disease Nick Manning and Nick Archer Paediatric Cardiology, John Radcliffe Hospital, Oxford, UK

Key words: congenital heart disease, fetal and neonatal, treatment, natural history, outcome

Serious structural congenital heart disease usually presents to the neonatal paediatrician, although increasingly these conditions are being diagnosed before birth. It is, therefore, important that those dealing with these fetuses and infants have some knowledge of their natural and modified history. The vast majority of lesions can either be corrected or given symptomatic palliation and this review discusses treatment options and provides up-to-date outcome information to enable fetal and neonatal staff to anticipate and to complement information given to families by paediatric cardiologists.  2001 Harcourt Publishers Ltd

Introduction

Natural History

This chapter is a brief overview of the natural history and treatment options for cardiac lesions usually detected or causing problems in the newborn period. Increasingly, structural heart disease is diagnosed in the fetus (see Chapter 5). Some lesions thus detected will not affect the health of the young infant, for example small or moderate sized VSDs, but are an indication for follow-up. Nevertheless, for the time being, the majority of individuals with important structural heart disease will not be detected until after birth and the neonatologist will be involved in the initial task of helping parents through this process even if the precise diagnosis is made by a paediatric cardiologist. Follow-up will usually involve a paediatrician as well as a cardiologist, it is, therefore, important for the paediatrician to have knowledge about the natural and modified histories for these conditions. Natural history can be considered in general terms in categories of lesions; this is done first and then treatment options and outcomes are reviewed.

Shunt lesions

Correspondence to: Dr N. Archer, Consultant Paediatric Cardiologist, John Radcliffe Hospital, Oxford OX3 9DU, UK. Fax: +44 (0)1865 220323; E-mail: [email protected]

1084–2756/01/010037+11 $35.00/0

Generally speaking, uncomplicated or isolated lesions permitting left-to-right shunting of blood do not cause symptoms in the newborn period as excessive lung blood flow and heart failure do not develop until pulmonary vascular resistance has fallen sufficiently to allow this, isolated atrial septal defects (ASDs) almost never cause heart failure in infancy. The problem of patent ductus arteriosus (PDA) in the preterm infant will not be considered further here [1]. Heart failure will rarely be overlooked, but some infants with significant shunt lesions can develop pulmonary vascular disease without passing through a phase of definite heart failure. This can happen with a large ventricular septal defect (VSD), complete atrioventricular septal defect (AVSD) and large PDA; pulmonary vascular disease is rare in ostium secundum ASD many of which close spontaneously in the first 2 or 3 years of life [2]. Long-term risk of right heart failure, atrial dysrhythmias and paradoxical embolism are present in ASD, although difficult to quantify. The approaches to intervention for ASD and medium and small PDA in the last 3 or 4 decades make untreated natural history difficult to © 2001 Harcourt Publishers Ltd

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be confident about [3,4]. Partial AVSD (ostium primum ASD) may cause heart failure in infancy, particularly if marked atrioventricular valve regurgitation is present, and there is a risk of pulmonary vascular disease in the long term in a proportion of cases. Whatever the shunt lesion, it is generally considered that pulmonary vascular disease is more likely and will develop earlier in the context of Down syndrome, marked prematurity and when living at high altitude. Many VSDs will close or get smaller spontaneously, even some that are initially large [5,6]. Complete and partial AVSDs do not resolve, nor do sinus venosus type ASDs. Aortopulmonary window and arteriovenous fistulae (cranial or other) are much rarer shunt lesions but the same considerations apply, those that are detected in early life are usually large and have an unfavourable natural history [7]. Obstructive lesions Non-cyanotic obstructive lesions are hard to generalize about but if significant obstruction to aortic or pulmonary valves exists in early infancy it is likely that ventricular hypertrophy and symptoms will occur in childhood even if the circulation is not dependent on the ductus arteriosus (see below) and even if the infant is well. Coarctation detected in the fetus or in the newborn period will either be duct dependent or will produce heart failure in early infancy or cause important hypertension before the end of infancy. Many cases of coarctation will not be detected in infancy because symptoms are absent and signs subtle, however, even for this group the untreated long-term outlook is poor with 90% mortality by 50 years [8–10]. Mitral stenosis is a rare congenital lesion and is nearly always associated with other lesions in the left heart or septal defects which dominate the clinical picture initially. Subaortic stenosis is an acquired lesion very rarely met in infancy (hypertrophic cardiomyopathy being excepted), but may be encountered later in some who have repaired aortic arch abnormalities or VSD. Supravalvar aortic stenosis has a strong association with William’s syndrome and is rarely severe in infancy, although many cases will ultimately progress and require surgery but not necessarily in childhood. Regurgitant lesions Aortic regurgitation rarely causes symptoms in early childhood, if a symptomatic infant appears to

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have aortic regurgitation it is far more likely that there is an aortico ventricular tunnel than valvar incompetence [11]. Mitral regurgitation may be secondary to heart muscle dysfunction or marked volume overloading of the heart. If it is due to a structural mitral valve abnormality it is likely to part of an AVSD be it partial or complete and the natural history is discussed above. Severe tricuspid regurgitation is usually a manifestation of Ebstein’s anomaly of the valve in which case persistent cyanosis and heart failure in infancy are associated with a poor short-term outlook. Conversely if cyanosis resolves over a week or two as pulmonary artery pressure falls or if there are no symptoms, but only a loud murmur the outlook is much better and the patient may be well for many years [12]. Tricuspid regurgitation with a normal valve is usually only mild or at most moderate and is associated with birth asphyxia and persistent pulmonary hypertension of the newborn. Cyanotic lesions Very few untreated cyanotic lesions are associated with survival to the age of 1 year, the notable exception being tetralogy of Fallot [13] which often does not cause neonatal cyanosis. Some cases of total anomalous pulmonary venous drainage (TAPVD) will not be detected in the newborn period if lung venous drainage is non-obstructed allowing high pulmonary blood flow and mild or absent cyanosis. Pulmonary atresia with VSD and aorto-pulmonary communicating arteries is in a minority of cases associated with reasonable or even good quality of life for decades. Identification of these rare cases in order to give realistic advice to families about the relative benefits of conservative versus operative management can be difficult [14]. Duct dependent lesions In some structural cardiac abnormalities either the systemic (Table 1) or the pulmonary (Table 2) circulations can be critically dependent on patency of the arterial duct. These lesions are associated with dramatic deterioration when the ductus arteriosus closes and without intervention—initially in the form of prostaglandin administration—death will nearly always follow the development of symptoms. Very occasional long-term survivors of

Treatment and outcome of congenital heart disease

typical duct dependent lesions have been described if the ductus remains open [15].

Types of intervention Medical management of symptomatic structural congenital heart disease in the newborn period and infancy will usually only improve symptoms and stabilize the patient. Sometimes treatment of heart failure in VSD will allow time for the defect to get smaller and in the very rare case of symptomatic isolated ostium secundum, ASD medical management should be persevered with, as many ASDs close [2]—in this situation it is important to be sure that the only cardiac lesion is the ASD (concomitant PDA or mitral stenosis can easily be overlooked for example) and to be sure that there is not coexistent respiratory disease contributing to symptoms. In symptomatic newborn babies and in the majority of symptomatic older infants, it is appropriate to consider surgical or transcatheter intervention. Interventions can be anatomically corrective (as in the repair of TAPVD or the arterial switch operation for transposition), physiologically corrective (as in the now rarely used intra-atrial repairs for transposition) or palliative (as in pulmonary artery banding and pulmonary to systemic shunt procedures). The general trend is to aim at early correction whenever possible, but in many conditions this is not possible, for example when there are not two adequate ventricles or there is only one arterial exit from the heart. Heart surgery can be either closed or open. In closed surgery the heart is not stopped whilst the procedure is carried out, examples of such surgery include coarctation repair, pulmonary artery banding and creation of systemic to pulmonary artery shunts (such as Blalock-Taussig or modified Blalock shunts). These operations are most often performed through a postero-lateral thoracotomy incision. Open-heart surgery is performed through a median sternotomy and involves stopping the heart and using either or both hypothermia and cardiopulmonary bypass to permit cardiac standstill. Corrective surgical procedures with the exception of coarctation repair and clipping of the ductus arteriosus are open. Interventional catheter procedures can similarly be considered as either palliative (for example balloon atrial septostomy) or as attempts to restore anatomy to nearer normal (balloon valvuloplasty).

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Some conditions may be treated either surgically or by catheter intervention (neonatal critical aortic stenosis for example), for some catheter intervention is generally first choice (pulmonary stenosis) and for some surgery is appropriate (repair of transposition, truncus arteriosus, TAPVD, AVSD and VSD). There are some local and international differences in approach so it is important to know local practice. It is to be hoped that multicentre data collection will continue to improve and allow answers to be obtained about preferred methods as well as to allow monitoring of individual operators’ performance be they surgeons or cardiologists.

Treatment of particular conditions In this section details of treatment type, timing, success rates and references are given in tabular form under diagnostic groups with additional comments in the text below.

Duct dependent systemic circulation (Table 1) Information on survival numbers and quality in hypoplastic left heart syndrome is evolving rapidly and will vary from place to place. The figures given in the table are representative. The ultimate goal in this condition is to establish a Fontan-type circulation where systemic veins drain directly into the pulmonary circulation without passing through a ventricle. This is usually achieved in three stages, the first of which is in the early newborn period and carries the greatest risk of death (25–35%), the subsequent stages have a survival rate of 90% or better and a 5-year survival as good as of 70–75% has been reported from some centres but this is by no means universally achieved. Many of these infants are fragile and there is an attrition rate between surgical stages. The initial high incidence of major long-term neurological sequelae is improving, but some families still opt for no active surgical treatment of this condition. The outcome for interventions for critical aortic stenosis is influenced very much by the size and condition of the left ventricle. All individuals who are successfully treated for critical aortic stenosis in infancy will eventually need further intervention, in some this may mean further balloon dilatation in infancy, in a few it means surgery for severe aortic regurgitation

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Table 1. Conditions in which there is duct dependent systemic circulation Condition

Intervention

Timing

Survival%

References

HLHS critical AS

Open Norwood stage 1 Open or catheter

Early Early

65–75 50–95+ 80+early 50 at 1 month

16, 17 18

Closed repair Closed repair PA band Closed repair PA band Open repair usually

Early Early

98 94

19–21

Early

Maybe 60

Early

80–90

Coarctation isolated with VSD with complex lesion interrupted aortic arch

22

Table 2. Lesions associated with duct dependent pulmonary circulation Condition Pulmonary atresia+IVS

Intervention

(a) Open (b) Laser assisted balloon (c) Palliative closed shunt Critical PS (a) Balloon (b) Surgery Pulmonary atresia+VSD (no collaterals) (a) Palliative shunt (b) Open repair

in infancy, but in some cases reintervention may not be for many years. Representative published figures are not easy to find. This makes comparing surgery with balloon catheter dilatation difficult and local practices vary a great deal. Some of the more extreme cases are treated as for hypoplastic left heart syndrome (HLHS). Surgical repair of coarctation is the preferred approach, recurrence may occur within a few months (particularly in preterm infants) and then balloon dilatation is the treatment of choice. In other cases return of narrowing takes some years, recurrence rates quoted for repair in early life are up to 20% [23]. There is less tendency to place a pulmonary artery band at coarctation repair in those with a VSD than previously unless there are strong contraindications to performing VSD closure within a matter of weeks or months if necessary. Pulmonary artery banding is usually reserved for those with more complex associated lesions than a VSD. Interrupted aortic arch repair is usually performed at the same open heart procedure as the almost invariable VSD is closed. If the cardiac abnormality is more complex a staged

Timing Survival% References Early Early Early Early Early Early Early

See text See text See text 90+ 90 80–85

24 25

approach with pulmonary artery banding may be employed. Duct dependent pulmonary circulation (Table 2) Pulmonary atresia with intact ventricular septum can be repaired by open heart surgery or if laser catheter technology [26] is available the valve can be perforated with this and then balloon dilated as for critical pulmonary stenosis. A trans catheter approach fails in up to 25% of cases [27], both balloon and surgery have significant reintervention rates [26,28], although in the case of surgery this may not be for some years. Some cases with this condition have such a small right ventricle that they are not suitable for a biventricular circulation and the ultimate aim then is a Fontan type circulation which is established in two or three stages. The first stage is a systemic to pulmonary arterial shunt. Survival figures for procedures vary, but it is to be hoped that 5-year survival figures are

Treatment and outcome of congenital heart disease

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Table 3. Other cyanotic lesions Condition Pulmonary atresia+VSD and MAPCAs Transposition no VSD large VSD VSD+PS Truncus arteriosus Tetralogy of Fallot TAPVD Tricuspid atresia DILV DORV

Intervention

Timing

Survival%

References

Multi-staged closed Single stage open

Infancy Infancy

See text See text

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Open repair Open repair Closed shunt Open repair Open repair Closed shunt Open repair Closed palliative Closed palliative

Early By 2 months Neonate or infant Neonate or early infant Infancy Infancy Neonate or infant Neonate or infant Neonate or infant

>95 92 See text 70–85 95+ 90+ 90 See text See text

31 32, 33 34 35 36–38

improving from about 50% in the late 1980s and early 1990s [29] as management becomes more individually tailored. Critical pulmonary stenosis is most often treated by balloon dilatation although local approaches and expertize will vary. Reintervention rates following balloon dilatation appear to vary greatly. Pulmonary atresia with VSD but without aorto-pulmonary collaterals is often treated by an early systemic to pulmonary anastomosis with later open repair. Early open repair has the disadvantage that the reconstruction of the right ventricular outflow tract will very probably need revision within a matter of only a few years. Other cyanotic lesions (Table 3) Pulmonary atresia with VSD and sources of pulmonary blood flow coming from the aorta directly or indirectly (major aorto pulmonary communicating arteries (MAPCAs)) produces a wide variety of clinical pictures depending on the size of lung blood flow. Surgical approaches are determined by detailed knowledge of the number and position of MAPCAs and this information is usually obtained by cardiac catheterization, ultrasound is rarely sufficient and magnetic resonance imaging is sometimes needed in addition or instead of angiography, particularly in older children. The choices include: (1) no intervention if the surgical challenge looks very high risk and the natural history might be one of years of reasonable quality of life (a minority of cases); (2) initial closed palliative shunt procedures with disconnection of MAPCAs and joining the

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pulmonary arterial branches together (staged unifocalization) with the ultimate possible goal of open corrective surgery; or (3) radical early open corrective surgery. Survival figures are hard to quote in a way that can be generalized, but approach 2 is likely to have the lower mortality in early life, though it defers higher risk procedures until later. The radical corrective approach has a number of centres producing impressive results, but this experience is not universal. Transposition is usually palliated by a balloon septostomy with a less than 1% risk of death. If there is no VSD corrective arterial switch surgery needs to be within 2 weeks or so of birth. If there is a large VSD delay for rather longer is acceptable; indeed, some of these patients will not present until after the newborn period. It is essential to perform surgery before pulmonary arterial pressures have fallen allowing the left ventricle to decondition as far as having to sustain high (systemic) pressure postoperatively. Cases presenting late need careful evaluation to be sure about the ability of the left ventricle to support the systemic circulation. A minority of arterial switch survivors develop important right ventricular outflow obstruction most usually supravalvar and about 0.5% per year require reintervention for this [31]. If there is important pulmonary outflow obstruction this is usually both valvar and subvalvar and often makes an arterial switch operation inappropriate as it would result in severe systemic outflow obstruction. These infants will need one or more shunt procedures before a corrective open operation in older childhood [34], involving refashioning of the

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Table 4. Simple left to right shunt lesions Condition AVSD Complete Partial ASD secundum VSD large PDA

Intervention

Timing

Survival%

References

Open repair Open repair Catheter Surgery Open repair Surgery rarely Catheter occlusion

Under 6 months Infancy or preschool if well Preschool and upwards

95 99 99+ 99 97+ 99+ 99+

42–44

Infancy Early infancy

pulmonary outflow tract and closing the VSD in such a way as to channel left ventricular blood into the aorta (procedures include the Rastelli operation and the Lecompte or Rev operations). Truncus arteriosus is repaired in early infancy, all cases will require at least one further operation before the end of childhood to replace the right ventricular outflow whatever technique is used initially, some will eventually also need aortic valve replacement for regurgitation. If aortic valve surgery is needed in infancy the risks are high. Tetralogy of Fallot is in the majority of cases suitable for primary open repair in infancy, a minority need palliative shunt surgery first if there are contraindications to repair. Contraindications include pulmonary atresia, abnormal coronary artery anatomy, multiple VSDs, severe uncontrollable hypercyanotic spells and other major medical problems. Total anomalous pulmonary venous drainage may need repair urgently at presentation if the pulmonary veins are obstructed, most are done shortly after diagnosis, but if heart failure is mild and controlled, and if pulmonary veins are not obstructed, there is less urgency. Long-term outlook for survivors is very good except for the minority with pulmonary venous hypoplasia or those in whom obstruction cannot be relieved surgically. If TAPVD is part of a more complex cardiac abnormality surgical survival is much worse and the long-term outlook not so good. These infants are likely to have isomerism states and may be asplenic. Tricuspid atresia is the condition with the best results from the Fontan procedure. The aim in early life is to ensure that the pulmonary vasculature grows well (using a shunt if pulmonary blood flow is duct dependent or severely diminished by pulmonary stenosis and/or a small VSD), but is protected from excessive flow or pressure (with a pulmonary artery band if there is no restriction to pulmonary flow by virtue of there being a large VSD or transposition). Some cases of

45 5, 46 4, 47

tricuspid atresia with transposition will have coarctation. Survival figures for every possible combination are hard to quote. Double inlet left ventricle (DILV) has similar considerations as for tricuspid atresia and will require the appropriate palliative intervention as newborns or in early infancy. Double outlet right ventricle (DORV) describes a variety of anatomical and haemodynamic arrangements and may have physiology like a simple VSD or like transposition. In a few cases of DORV, however, there is effectively only one ventricle and for these the same considerations apply as in tricspid atresia and DILV. These cases of DORV usually have severe mitral valve hypoplasia or atresia. Simple left to right shunt lesions (Table 4) These lesions generally are repaired if indicated in infancy with high success rates and a good longterm outlook. Atroventricular septal defects if only partial (ostium primum ASD) may not require surgery until a few years of age but those with heart failure and failure to thrive will do, these cases often have marked atrioventricular valve regurgutation. All complete AVSDs will need intervention preferably in early infancy if pulmonary vascular disease is to be avoided and even then occasionally it will still occur. Corrective open surgery is the normal approach for AVSD, pulmonary artery banding is only rarely appropriate. Some AVSDs, both partial and complete will require later atrioventricular valve repairs or even replacement. Ostium secundum atrial septal defects are not repaired as isolated lesions in infancy as many close. Transcatheter occlusion is the preferred approach in most centres now and is usually deferred until nearly school age or older. Sinus venosus type ASDs are

Treatment and outcome of congenital heart disease

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Table 5. Other obstructive lesions Condition

Intervention

Timing

Survival%

References

Aortic stenosis (non critical) Pulmonary stenosis Mitral stenosis

balloon dilatation

when indicated

99

47

balloon dilatation open valvotomy

99+ see text

47

Subaortic stenosis

open surgery

when indicated depends on symptoms and other lesions childhood

99

48

not currently suitable for transcatheter occlusion because of the proximity of the defect to either the superior vena cava and pulmonary veins or to the inferior vena cava. Symptomatic ventricular septal defect and those with pulmonary artery pressures that are significantly elevated are repaired by open surgery in infancy. Some defects are associated with the development of right or left ventricular outflow obstruction or aortic regurgitation so that surgery becomes indicated in later childhood for one of these complications even if the VSD has by then closed spontaneously. Patent ductus arteriosus is rarely closed by surgery as transcatheter techniques are applicable to all but the smallest infants.

Other obstructive lesions (Table 5) Balloon dilatation is the treatment of choice for non critical aortic stenosis (AS). It is likely that AS requiring intervention in childhood will require further treatment but not necessarily for many years. Pulmonary valve stenosis if not critical or symptomatic in early infancy is very successfully treated by balloon dilatation and most will not require further intervention, those with thick dysplastic valves may do. This type of valve is particularly seen in association with Noonan’s syndrome. Mitral stenosis is rare in early life and associated lesions usually mask it, dominate the clinical picture and determine treatment plans. Treatment can be difficult, valvotomy or replacement are occasionally needed, the outlook is often poor because of other lesions. Subaortic stenosis is usually an acquired or progressive lesion and intervention in infancy is rarely needed, although in cases of interrupted aortic arch the development of this problem can sometimes be predicted and occasionally needs addressing at initial repair.

Other lesions Aortic and mitral regurgitation are both very rarely severe enough to require intervention in early life (see above).

Long-term outlook Until recently, the emphasis on survival rates for interventions meant that there was a paucity of information on long-term outlook after treatment in infancy, particularly relating to quality of life rather than mortality rates. Some long-term information relates to conditions not originally treated in infancy so that the patient population being generated now is very different. Thus 30 to 40 year follow-up of repaired tetralogy of Fallot [49] or coarctation [50] may not accurately reflect what may be expected for those treated at a younger age. This inaccuracy would also apply to conditions in which the surgical approach is now completely different such as repair of transposition for which intra-atrial repairs (Mustard and Senning procedures) are now very rarely used, although there are many young adults who had such surgery for whom atrial dysrhythmias and right ventricular failure are ongoing problems [51]. There is much more follow-up information on cardiovascular aspects of long-term health than there is on neurological and emotional aspects. In view of the nature of information available it is often not possible to give precise figures for various long-term sequelae, but the following considerations apply to all survivors of intervention for structural congenital heart disease and need to be addressed when talking to families. Imparting of relevant information is the responsibility of the cardiac team but other groups

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dealing with the fetus and infant should be aware of the issues in principle. Endocarditis risk It is generally considered that all uncorrected structural heart lesions except isolated uncomplicated ostium secundum and sinus venosus type ASDs constitute an increased risk for infective endocarditis and that preventative measures are indicated including good dental health, treatment of bacterial skin sepsis and prophylactic antibiotic administration for potentially bacteraemia producing surgical procedures. The risk is increased in the 3 months after any cardiovascular surgical intervention or transcatheter device implantation, but ceases thereafter in the case of isolated ASD repair and complete occlusion of a patent ductus arteriosus. Whether or not there is an increased risk after successful repair of total anomalous pulmonary venous drainage is not clear and advice given may vary, similarly after VSD repair with no residual leak, although most centres continue to advise preventative measures in that circumstance. Spontaneous closure of an isolated VSD is a reason to rescind preventative advice. Re-operations/re-interventions Any cardiac surgical procedure may require early re-operation for either bleeding or an unsatisfactory repair, although this is uncommon in modern practice. In the long term a number of procedures have significant and well-documented re-intervention rates. For example, recoarctation has been reported in up to 20% of neonatal repairs [23], the rate is lower for those operated on after the newborn period [23], but it is not usually possible to defer surgery in those presenting in early life. The re-intervention of choice is most often balloon dilatation. It is clear that a proportion of arterial switch survivors will develop significant right ventricular outflow obstruction that may be amenable to balloon dilatation or may require surgery [52]. If followed for several decades about 5–10% of tetralogy repairs need further surgery [49] for either recurrent right ventricular outflow obstruction or pulmonary regurgitation. All cases of truncus arteriosus repair will require further intervention in later childhood for replacement of right ventricular to pulmonary artery conduit,

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whatever technique was used initially. Successful repair of AVSD is associated with a long-term risk of further surgery for atrioventricular valve regurgitation of about 15% [53]. Critical aortic stenosis in the newborn will be associated with the need for further intervention in all cases, usually before adult life regardless of whether surgery or interventional catheterization was the initial therapy [54,55]. As well as revision of the original surgery there may be associated lesions for which the natural history is of progression so that, although the original procedure remains adequate intervention for a different problem becomes indicated. Examples of this include aortic stenosis or regurgitation in repaired coarctation and subaortic stenosis in repaired aortic arch interruption with VSD. In cases where the initial treatment is a transcatheter procedure, the incidence of further intervention is generally higher than for an initial surgical approach to the same condition, but the risk of further catheter interventions may be expected to be less than for repeat surgery [18,55].

Dysrhythmias All types of structural congenital heart disease have an increased incidence of rhythm disturbances compared to the general population whether or not the abnormality has been corrected or will ever need correction for haemodynamic considerations. Ten years after a Mustard or Senning procedure for transposition the majority of patients will not be in normal sinus rhythm all the time [51] and in some there is atrial flutter or severe sinoatrial dysfunction [51]. The incidence of significant dysrhythmias is much lower after the arterial switch operation and this is one of the long-term benefits of this approach to transposition [51]. Heart block can occur acutely after open heart surgery, but often recovers over a period of a few days. However, in some heart block is permanent and requires a permanent pacemaker system. The incidence of heart block is poorly documented in modern times, but the more complex the diagnosis and surgery the greater the risk. Any scar on the myocardium can in the long term be a focus for rhythm disturbance arising from the scarred region. Ventricular arrhythmias can occur years after tetralogy of Fallot repair in about 5% with a good haemodynamic status, the rate is considerably higher if haemodynamics are poor [56] and assessing risk of

Treatment and outcome of congenital heart disease

this happening is currently not a precise science, although it has been much studied. Exercise and employment The majority of survivors with treated congenital heart lesions will not need to have any particular restrictions placed on them and when giving families a broad view many years in advance there are only a number of key points to remember. If the ultimate goal is a Fontan-type circulation there is likely to be a significantly reduced exercise capability compared to peers [57]. Individuals with lesions in which progressive systemic outflow tract obstruction is likely to occur may well be advised to avoid highly physical competitive sporting activity, such lesions include subvalvar aortic stenosis in the context of repaired coarctation or aortic arch interruption. There are a few high risk or high stress jobs that individuals with repaired structural heart disease would not be accepted for, including many branches of the armed services, commercial flying and diving. Lesions that cannot be physiologically corrected and, therefore, leave the patient desaturated will be associated with exercise limitation. Education and neurological sequelae It is important if possible to recognize syndromes at an early stage so that accurate prognostic information can be given if available (see chapter N). The incidence of neurological events following heart surgery in children has probably been under recorded, but some kind of acute episode may be seen in up to 17% of aortic arch abnormalities and 5–10% of right ventricular outflow repairs [58]; in approximately 50% of these the abnormalities are transient. This information, of necessity, relates to past practice [59] and current figures could be skewed either way by improvements in cerebral protective strategies or by the survival with handicap of those that previously would have died. Reproductive issues Pregnancy carries considerable risk to women who remain cyanosed both in terms of their own health and in terms of the likelihood of a healthy baby [60]. This is particularly true of those with Eisenmenger’s syndrome, but a modern approach

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to early diagnosis and management should make that situation much less common. Repairs resulting in a heart with a systemic and a pulmonary ventricle without cyanosis will usually allow a woman to cope with pregnancy, although important valvar or conduit stenosis or regurgitation should be corrected before pregnancy is embarked upon. Risk to offspring of congenital heart disease needs to be addressed in detail at the appropriate time, but is not a big factor for the parents of babies facing cardiac surgery. Contraception similarly has some special considerations which are not particularly relevant in discussions in the early years of an individual’s management. References 1 Archer N. Patent ductus arteriosus in the newborn. Arch Dis Child 1993; 69: 529–532. 2 Helgason H, Jonsdottir G. Spontaneous closure of atrial septal defects. Pediatr Cardiol 1999; 20: 195–199. 3 Oakley CM. Does it matter if atrial septal defects are not diagnosed in childhood? Arch Dis Child 1996; 75: 96–99. 4 Sullivan ID. Patent arterial duct: when should it be closed? Arch Dis Child 1998; 78: 285–287. 5 Frontera-Izquierdo P, Cabezuelo-Huerta G. Natural and modified history of isolated ventricular septal defect: a 17-Year Study. Pediatr Cardiol 1992; 13: 193–197. 6 Turner SW, Hunter S, Wyllie JP. The natural history of ventricular septal defects. Arch Dis Child 1999; 81: 413– 416. 7 McElhinney DB, Reddy VM, Tworetzky W, Silverman NH, Hanley FL. Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants <6 months of age. Am J Cardiol 1998; 81: 195–201. 8 Karnell J. Coarctation of the aorta. Circulation 1968; XXXVII–XXXVIII(suppl V): V35–V44. 9 Campbell M. Natural history of coartctation of the aorta. Br Heart J 1970; 32: 633–640. 10 Jenkins NP, Ward C. Coarctation of the aorta: natural history and outcome after surgical treatment. Q J Med 1999; 92: 365–371. 11 Westaby S, Archer N. Aortico-right ventricular tunnel. Ann Thorac Surg 1992; 53: 1107–1109. 12 Celermajer DS, Bull C, Till JA, et al. Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994; 23: 170–176. 13 Bertranou EG, Blackstone EH, Hazelrig JB, Turner ME, Kirklin JW. Life expectancy without surgery in tetralogy of Fallot. Am J Cardiol 1978; 42: 458–466. 14 Bull K, Somerville J, Ty E, Spiegelhalter D. Presentation and attrition in complex pulmonary atresia. J Am Coll Cardiol 1995; 25: 491–499. 15 Maxwell P, Somerville J. Aortic atresia: survival to adulthood without surgery. Br Heart J 1990; 64: 336–337. 16 Ishino K, Stumper O, De Giovanni JJ, et al. The modified Norwood procedure for hypoplastic left heart

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27

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