British Journal of Anaesthesia 93 (1): 129±39 (2004)

DOI: 10.1093/bja/aeh172

Anaesthetic implications of grown-up congenital heart disease A. T. Lovell University Department of Anaesthesia, Level 7, Bristol Royal In®rmary, Marlborough Street, Bristol BS2 8HW, UK E-mail: [email protected] Br J Anaesth 2004; 93: 129±39 Keywords: complications, grown-up congenital heart disease; pathophysiology; surgery, cardiovascular

Congenital heart disease (CHD) is one of the most common inborn defects, occurring in approximately 0.8% of newborn infants.75 98 The frequency of the common congenital cardiac defects is shown in Table 1. In the era before the development of paediatric cardiac surgery, fewer than 20% of these children survived to adulthood.59 Now, more than 85% of children with CHD can expect to live into adulthood. This dramatic success has created an ever increasing population of young adults with grown-up congenital heart disease (GUCHD).22 It is expected that shortly there will be more adults with CHD than children.24 There are approximately 800 000 adults in the USA with CHD,98 and recent estimates suggest that 1600 new cases with moderate-to-severe complexity are referred to GUCHD units within the UK each year.103 The nature of the underlying cardiac problems that these GUCHD patients have, shown in Table 2, is substantially different from that in the paediatric population. The majority of these adults will require lifelong cardiological surveillance.22 This is in recognition of the fact that, in a substantial number of patients, surgery in childhood has been reparative rather than corrective. Perhaps the greatest example of this is the long-term management of a patient who has undergone correction of tetralogy of Fallot (ToF). GUCHD patients can broadly be divided into three categories: those who have undergone some sort of reparative operation; those who have undergone a palliative operation; and ®nally, there is a group of patients who have not previously undergone a corrective or palliative procedure. There are several reasons why patients may present with uncorrected lesions: late diagnosis is always a possibility, particularly in the case of atrial septal defects (ASD) and aortic coarctation. Patients with balanced pulmonary and systemic circulations, as commonly occurs in patients with complex lesions, may remain relatively asymptomatic until the balance between pulmonary and systemic circulations is disturbed. Some patients will have previously been considered to be inoperable, and some patients may

have come from overseas where facilities are unavailable. The uncorrected group of patients may present a severe challenge, in that issues related to the very long-term effects of chronic hypoxia and excessive or reduced pulmonary blood ¯ow may result in considerable modi®cation of the patient's physiology. Paediatric cardiac surgery has changed enormously over the last 50 years. As a consequence, the pathophysiology of the patients is changing. Some palliative procedures have been abandoned, and increasingly there is a move towards the use of complex, sometimes staged, procedures for the correction of complex lesions.

General considerations Traditionally patients with CHD requiring non-cardiac surgery have frequently been managed in their paediatric cardiac centre. As they become adults, this is no longer appropriate. With the steady increase in the numbers of GUCHD patients, they are increasingly presenting for coincident procedures to units not involved in their GUCHD management. Increasing numbers of women are reaching reproductive age and requiring anaesthetic management during labour and delivery. Management of these women is based on knowledge of the physiological changes that occur during pregnancy, assessment of the existing degree of cardiovascular impairment and a detailed knowledge of the underlying pathophysiology. These highrisk cases require regular assessment throughout pregnancy, and it is important that a plan is made to cope with delivery. Some lesions are extremely well tolerated throughout pregnancy; others can decompensate disastrously. The presence of cyanosis is associated with deterioration of more than half of the women during pregnancy, while only 15% of acyanotic patients deteriorate.86 The presence of congestive failure is associated with functional deterioration in a third of cases, compared with only 5% in its absence. The presence of pulmonary hypertension is particularly

Ó The Board of Management and Trustees of the British Journal of Anaesthesia 2004

Lovell Table 1 Relative frequency of common congenital heart defects47 Acyanotic lesions Ventricular septal defect Atrial septal defect Patent ductus arteriosus Pulmonary stenosis Coarctation of the aorta Aortic stenosis Atrioventricular septal defect Cyanotic lesions Tetralogy of Fallot Transposition of the great arteries

Table 3 Factors that are likely to require transfer to a regional grown-up congenital heart unit55 35% 9% 8% 8% 6% 6% 3% 5% 4%

Signi®cant intracardiac shunting More than mildly elevated pulmonary vascular resistance More than moderate left ventricular dysfunction or failure More than mild right ventricular dysfunction or failure A systemic right ventricle Functional univentricular heart More than mild obstructive valvular or vascular disease, excluding isolated aortic stenosis, and many cases of isolated mitral valve disease Signi®cant congenital coronary artery abnormalities Pregnancy in a patient with signi®cant congenital heart disease New onset of symptomatic tachy- or brady-arrhythmias

Table 2 Frequency of common diagnoses amongst attenders at a grown-up congenital heart clinic32 Atrial or ventricular septal defects Tetralogy of Fallot Complex lesions Obstructive lesions affecting the left ventricle out¯ow tract Complete transposition of the great arteries Obstructive lesions affecting the right ventricle out¯ow tract Coarctation of the aorta Marfan's syndrome Congenitally corrected transposition of the great arteries Atrioventricular septal defect Eisenmenger syndrome

22% 14% 13% 12% 10% 8% 7% 5% 4% 3% 3%

ominous, being associated with a maternal mortality of 30% in the presence of pulmonary vascular disease (PVD).78 Some congenital lesions are inheritable. However, the risk to the fetus is largely proportional to the degree of maternal hypoxia, with a 50% incidence of fetal death if maternal arterial saturation is either less than 85% or the haematocrit exceeds 65%.79 Surgery in children with CHD has long been recognized as posing an increased risk of morbidity and mortality, particularly if their cardiac disease is poorly compensated.37 Twenty percent of GUCHD patients who require emergency hospitalization, for any reason, will have died or require transplantation within 3 yr.48 Risk factors associated with perioperative complications include the presence of cyanosis, treatment for congestive cardiac failure, poor general health and younger age.97 Strati®cation into high- and lowrisk surgery is based on whether the operation is elective or urgent and major or minor. It has been suggested that optimal outcome for high-risk patients is best provided in a tertiary care facility.75 It is inevitable that this will require the transfer of some patients, but the numbers are small: 571 a year for the whole of Germany and Switzerland.48 Conditions that are likely to require transfer to a regional unit are shown in Table 3.55

Anaesthetic technique GUCHD patients represent a spectrum, from completely well patients with normal physiology to those with severely deranged physiology. The effect of damage to the central nervous system and the success of ventricular preservation

at the time of their previous procedures require careful consideration. In many patients in whom there has been a good outcome from cardiac surgery and in whom there is no evidence of late deterioration, conventional anaesthetic management is appropriate. In patients with functional limitation, the anaesthetic technique is modi®ed to take account of the main problems currently presented by the patient. The most important aspect of the perioperative care is the involvement of a team with a detailed understanding of the patient's cardiac defect, their functional status and anticipation of the perioperative stresses. Sedative premedication to reduce oxygen consumption is popular, although care must be taken in patients with cyanotic heart disease and PVD. The bene®ts of regional anaesthesia, alone or in combination with general anaesthesia, need to be weighed on an individual basis. The bene®ts from good postoperative analgesia, and avoidance of activation of the sympathetic nervous system with consequent catecholamine release, need to be weighed against the effects of a reduction in systemic vascular resistance (SVR). The circulatory effects of the i.v. induction agents are well known. All of the commonly used i.v. induction agents can be used safely ± the rate and dose administered are more important than the actual drug used. All are associated with a reduction in SVR to some degree, and if a right-to-left shunt is present, this will increase. The effect of right-to-left shunting on delaying the uptake of inhalational agents is rarely problematic, although inhalational induction of anaesthesia is prolonged.44 In patients with simple cardiac lesions, halothane, iso¯urane and sevo¯urane do not change the degree of left-to-right shunt in patients ventilated with 100% oxygen.54 However, this cannot be extrapolated to patients with complex CHD, or those with right-to-left shunts. The need for invasive monitoring depends upon the nature of the surgery as well as the underlying cardiac lesion. Previous Blalock±Tausig (B±T) shunt placement requires arterial pressure monitoring on the contralateral side. Central venous canulae pose a signi®cant thromboembolic risk in patients with a Fontan type of circulation, and yet the information they provide may be extremely valuable. Pulmonary artery catheterization can be dif®cult

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Anaesthetic implications of GUCHD patients Table 4 Factors affecting pulmonary vascular resistance (PVR)

Infective endocarditis

Decrease in PVR

CHD is an important risk factor for the development of infective endocarditis, being responsible for 9% of all cases recently reported in Japan.66 Virtually any congenital cardiac defect may predispose to the development of infective endocarditis; however, the risks vary dramatically with the underlying lesion (Table 5). It has been estimated that for an uncorrected ventricular septal defect (VSD), the risk of developing infective endocarditis by the age of 30 yr is 10%,35 with an overall lifetime risk of 13%.85 A clear cause of a preceding bacteraemia is present in two-thirds of cases, and apart from i.v. drug use, the mouth, especially dental procedures, remains the commonest portal of entry. Both the American Heart Association20 and the European Society of Cardiology41 clearly recommend that, for patients at high and moderate risk, antibiotic prophylaxis should be used when performing procedures associated with a risk of bacteraemia. Previously, labour and delivery have been regarded as high risk. Bacteraemia after uncomplicated vaginal delivery only occurs in 1±5% of cases, and endocarditis in this setting is uncommon.91 This has led both the American Heart Association20 and the American College of Obstetrics and Gynecology5 to recommend that antibiotic prophylaxis at delivery is con®ned to those patients in whom bacteraemia is likely.

Increase in PVR

Sympathetic stimulation Increasing PaO2 Hypocarbia Light anaesthesia Alkalaemia Pain Minimizing intra-thoracic pressure Acidaemia Spontaneous ventilation Hypoxia Normal lung volumes Hypercarbia High frequency and jet ventilation Hypothermia Avoidance of sympathetic stimulation Increased intrathoracic pressure Deep anaesthesia Controlled ventilation Pharmacological methods PEEP Isoprenaline Atelectasis Phosphodiesterase III inhibitors Prostaglandin (Pg) infusion (PgE1 and PgI2) Inhaled nitric oxide

because of anatomical abnormalities, and is not without risk in the patient with reactive PVD. It must be remembered that, in the presence of a right-to-left shunt, measurement of the cardiac output by thermodilution on the right side of the circulation can be dangerously misleading. In a patient who has undergone a Glenn or Fontan type of repair, superior vena cava (SVC) pressure should be the same as pulmonary artery pressure (PAP). Transoesophageal echocardiography can provide real-time assessment of ventricular function, preload and intracardiac shunting, as well as valve function. In the case of a patient undergoing cardiac surgery, its utility is beyond question; in the patient undergoing non-cardiac surgery it may be just as valuable. Capnography signi®cantly under-reads PaCO2 in the presence of right-to-left shunting, and the degree of discrepancy will change as the extent of shunting changes. Postoperative care of these patients is in either a high dependency unit or intensive care unit (ICU). There has been a trend for over 10 yr in many ICUs to `optimize the cardiovascular system' in high-risk surgical patients by ¯uid loading and other measures to increase systemic oxygen delivery. This approach may precipitate catastrophic cardiac failure in GUCHD patients with poor ventricular function. Experience in managing pulmonary vascular resistance (PVR) problems in the perioperative period in children with CHD can be generalized to the GUCHD patient (see Table 4). Control of blood gases is fundamental to control of PVR and hence pulmonary blood ¯ow in GUCHD patients. During IPPV, the duration of inspiration has a greater effect than the peak inspiratory pressure. Consequently, decreasing the duration of inspiration, and consequently increasing peak inspiratory pressure, is usually the best strategy to maximize pulmonary blood ¯ow. Probably the greatest difference in management of GUCHD patients with complete mixing compared with normal patients is in the interpretation of a high saturation. Whilst this is a normal goal for the care of patients in the ICU, in patients with central mixing there is a hyperbolic relationship between arterial saturation and the ratio of pulmonary and systemic ¯ows.

Hypoxaemia Cyanosis can arise either because of inadequate pulmonary blood ¯ow, usually associated with right-to-left shunting, or because of pulmonary hyperperfusion. In patients with limited pulmonary perfusion, avoidance of perioperative dehydration, maintenance of SVR, control of PVR, and minimizing increases in oxygen consumption are central to a successful outcome. In patients with high pulmonary blood ¯ow (PBF) secondary to excessive left-to-right shunting, any further increase in PBF is likely to be accompanied by an increase in cardiac work, or if ventricular function is unable to increase further, a decrease in systemic perfusion. Regardless of the cause of hypoxaemia, it has profound haematological effects that affect many other organ systems. Polycythaemia is a compensatory response that improves oxygen transport at the expense of an increase in viscosity. Under most circumstances, the increase in viscosity is well tolerated, although undoubtedly these patients are at an increased risk of thrombosis and stroke.7 Despite the risk of thrombosis, the platelet count is frequently reduced, and abnormalities of platelet function are common. Multiple coagulation factor de®ciencies are also common in patients with cyanotic CHD, producing a patient who is at risk of both spontaneous and excessive perioperative bleeding. The effect of chronic hypoxia on the heart is to induce myocardial dysfunction that usually manifests as a

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Lovell Table 5 Reported risk of underlying congenital heart disease defect on the risk of endocarditis.20 41 ASD, atrial septal defect; VSD, ventricular septal defect; PDA, patent ductus arteriosus High risk Previous infective endocarditis Prosthetic heart valves Complex congenital cyanotic heart disease Surgically constructed systemic or pulmonary conduits Moderate risk Non-cyanotic heart disease not mentioned elsewhere Mitral valve prolapse with valvular regurgitation Negligible risk Isolated secundum ASD 6 months after surgical repair of ASD, VSD or ligation of PDA Mitral valve prolapse without regurgitation Physiological, functional or `innocent' heart murmurs Cardiac pacemakers

reduction in ventricular diastolic compliance and a reduction in myocardial reserve. The limitation of cardiac output may not be apparent at rest, but frequently there is a marked limitation in exercise tolerance. Chronic renal hypoxia produces marked glomerular abnormalities27 that are associated with a reduction in glomerular ®ltration rate and result in a rise in plasma creatinine and urate. The latter is aggravated by the increase in red cell turnover. The hyperuricaemia is well tolerated and does not require intervention.73

Pulmonary hypertension Pulmonary hypertension is de®ned as a mean PAP greater than 25 mm Hg. Unrestricted left-to-right shunts result in abnormally high PBF. These lesions result in pulmonary hypertension and right ventricular (RV) hypertrophy early in life. If this situation is allowed to persist, the changes in the pulmonary vascular tree become irreversible. At this stage, correction of the underlying lesion is not accompanied by a return of PAP to normal. The right ventricle is not designed to function against a high afterload, and ultimately will fail. One of the driving forces in paediatric cardiac surgery over the last 20 yr has been to avoid the long-term sequelae of pulmonary hypertension. Although many of the lesions likely to produce pulmonary hypertension are now corrected early in childhood, there still exists a sizable cohort of patients who underwent surgery in an era when de®nitive surgery was performed late. In patients who have had pulmonary hypertension, signi®cant problems can develop in the perioperative period even if they are undergoing non-cardiac surgery. The aim of the management of these patients is to avoid factors that predispose to pulmonary hypertension and to reverse any reversible factors (Table 4). Where practical, regional anaesthetic techniques are preferred. If general anaesthesia is required, controlled ventilation is almost mandatory.

Eisenmenger syndrome The presence of a non-restrictive communication at any level of the heart, with a consequent increase in PBF, along with transmission of a high, sometime systemic, pressure to the pulmonary arteries, is the driving force for the development of irreversible PVD. The Eisenmenger complex is characterised by a PVR greater than 800 dynes s cm±5, with a reversed or bidirectional shunt ¯ow.102 The Eisenmenger syndrome is the commonest cause of congenital cyanotic heart disease in adults,21 unlike children where ToF predominates. The structural changes to the pulmonary vascular bed progress relentlessly, starting in childhood.36 80 102 Various methods of grading the pathological changes have been described; the simplest that shows good correlation between haemodynamic behaviour and pathology is that of Rabinovitch and colleagues.80 Early on there is extension of muscle into normally non-muscular peripheral arteries. This is associated with an increase in PBF and PAP, but a normal PVR. Later there is medial hypertrophy of the more proximal muscular pulmonary arteries, associated with a rise in mean PAP. Finally there is a reduction in the number of distal pulmonary vessels accompanied by an increase in PVR. The rate of progression of Eisenmenger's syndrome is greatly in¯uenced by the level of the shunt. In Wood's classic series, 80% of cases that were secondary to a VSD presented in infancy, with fewer than 2% presenting in adulthood.102 In contrast, 92% of cases secondary to an ASD present in adult life.71 De®nitive treatment, if the change in PVR is not irreversible, is to close the defect. If this is not possible, then the survival of patients with Eisenmenger's syndrome is often thought to be poor. In fact many of them do surprisingly well: the median survival is to the mid-thirties, although patients in their sixties have been described. The actuarial survival is substantially better than that of patients with primary pulmonary hypertension.39 Patients with simple underlying cardiac lesions present later than those with complex lesions, and have a substantially better survival rate.21 Decreases in SVR are likely to result in increases in rightto-left shunting, a worsening in cyanosis, and have been associated with cardiovascular collapse and death. Sudden increases in SVR may lead to a decrease in ventricular function, especially if associated with a rise in PVR. Ventricular function is usually well maintained,40 dysfunction occurring most commonly in the setting of complex congenital heart disease. The development of symptomatic RV failure carries a poor prognosis.21 Unsurprisingly, arrhythmias are usually poorly tolerated. Fluid shifts and hypovolaemia, as can occur with haemorrhage, can result in a marked fall in cardiac output in a heart that is highly preload dependent. As a consequence of these physiological limitations, surgical procedures are associated with a high morbidity and mortality.6 21 81 There has been considerable debate as to the

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advisability of the use of regional, particularly neuraxial, techniques. This has been fuelled by the knowledge that maintenance of the SVR is vital. In the past, general anaesthesia has frequently been preferred.6 81 More recently, it has been shown that regional techniques can be used safely.62 Martin and colleagues62 concluded that the perioperative mortality was related to the surgical procedure, rather than the anaesthetic. In addition to the type of anaesthetic, there are other equally important considerations. The risk of systemic embolization is not insigni®cant; all i.v. lines should be ®tted with an air ®lter. The goal of intra-operative monitoring is to detect sudden changes in haemodynamics very early in order to reverse them and prevent further complications. There are no outcome studies to guide the use of invasive cardiac monitoring. Continuous intra-arterial pressure monitoring and pulse oximetry, as a means of determining changes in right-to-left shunt, have been very widely used. Pregnancy is generally considered to be contraindicated3 79 with maternal mortality quoted as 30± 70%.3 101 104 More recent reviews have suggested that the outcome after regional anaesthesia may be better,62 88 although mortality is still markedly increased. Caesarean section carries a very high mortality that reaches 80% in the presence of preeclampsia.76

Palliative shunts Palliative shunts are used to increase PBF when de®nitive correction is either impractical or impossible. The most widely used shunt has been the B±T (Blalock-Taussig) shunt or its modi®cation in which the subclavian artery is connected to the pulmonary artery. When a B±T shunt is inadequate or impossible, then a central shunt between the aorta and pulmonary artery may be the only method of providing tolerable pulmonary perfusion. It is dif®cult to regulate the blood ¯ow through a central shunt, frequently it is excessive, and may give rise to pulmonary hypertension. The ¯ow through a palliative shunt is largely determined by the size and length of the shunt, which are ®xed, and the systemic driving pressure and PVR, which are under control of the anaesthetist. Measures that lower systemic arterial pressure in patients with a palliative arterial shunt can have disastrous effects on pulmonary perfusion and should be used with extreme caution.

Arrhythmias Arrhythmias are the main reason for hospitalization of adults with GUCHD,89 and account for over half of all emergency admissions.48 All arrhythmias that can occur in the normal population can occur in GUCHD patients, but much more common are the acquired arrhythmias rarely seen in young adults that are a consequence of the underlying pathophysiology and the scarring induced by surgery.93 Supraventricular arrhythmias are common in

patients who have undergone atrial surgery and in the presence of atrial distension such as can occur following the Fontan procedure. Most commonly they take the form of an intra-atrial re-entrant tachycardia (IART). Risk factors for development of IART are older age at surgery and longer follow up. Changes in surgical technique, such as the development of the total cavopulmonary connection (TCPC) instead of the classical Fontan have been associated in a reduction in its incidence.90 Although the most frequent arrhythmia has long been considered to be ventricular tachycardia (VT), more recent studies have shown that IART is more common.84 Pharmacological treatment of IART is generally unsuccessful, and current management aims at curative radiofrequency ablation.49 If this is unsuccessful, surgical intervention is warranted.23 Late-onset VT is a major problem in patients who have undergone an otherwise successful repair of ToF. Major risk factors for the development of VT are duration of follow up69 and QRS prolongation to greater than 180 ms, especially if associated with pulmonary regurgitation and RV dilatation.29 33 Antiarrhythmic therapy may be effective in suppressing ventricular arrhythmias, but has not been associated with improved survival.93 Therrien and colleagues92 have shown that replacement of an incompetent pulmonary valve is associated with stabilization in the QRS duration and a reduction in arrhythmias, especially if combined with cryoablation. Sudden late cardiac death remains a problem in GUCHD patients, occurring at a much greater incidence than in an aged matched population. More than 90% of these cases can be attributed to ToF, transposition of the great arteries, aortic coarctation or aortic stenosis.

Speci®c conditions It is impossible in the space available for this review to comprehensively cover the congenital lesions that are likely to be met in clinical practice. Detailed guidelines for the management of patients with CHD have been published following the Canadian Consensus Conference in 199618 and by the European Society of Cardiology.24 The detailed pathophysiology of the common, and rare lesions, in both the corrected and uncorrected states have been described by Gatzoulis and colleagues34 and by Brickner and colleagues.12 13 In all cases, it is vital that there is a thorough understanding of the patient's pathophysiology, and a decision will need to be made by the multidisciplinary team caring for the patient as to where the optimal location is for surgery to occur. In many cases, particularly those with complex lesions, referral to a regional centre is the best option.

Atrial septal defects ASDs are subclassi®ed according to their exact location; however, their physiological consequences essentially are

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independent of their site, depending on their size and the extent of any shunt. The behaviour of the shunt depends on the compliance of the two ventricles and the size of the defect. A small defect (<5 mm) is associated with a small shunt that is without haemodynamic consequences. A 20 mm defect on the other hand is associated with a large shunt that can have considerable haemodynamic effects. The majority of ASDs are detected in childhood, although a signi®cant minority are only diagnosed in adult life. Initially RV compliance is substantially greater than the LV and this is associated with the development of a left-to-right shunt. PBF is increased and pulmonary hypertension develops with increasing age. A consequence of these physiological changes is dilatation of both left and right atria, the RV and the pulmonary arteries in order to accommodate the increased blood volume. Ultimately either the RV fails, or RV compliance falls, resulting in a reduction in the magnitude of shunting, or even ¯ow reversal. Although pulmonary hypertension is quite common with increasing age, it is rare for the PVR to exceed 500 dyn s cm±5. A few ASDs present in childhood with breathlessness, or even heart failure, but most are detected by echocardiography after a child has been referred for other reasons. In adult life, presentation commonly occurs because of breathlessness, atrial arrhythmias or heart failure, although some individuals remain essentially asymptomatic until shunt reversal occurs. Although substantially less common than in the case of VSD, some ASDs close early in childhood.17 In the absence of signs of ventricular dilatation, with only a modest increase in PBF, closure has often been thought to be unnecessary. ASDs associated with a greater than 50% increase in PBF should be closed, however, in order to prevent the development of RV dysfunction51 65 and atrial arrhythmias.31 51 In patients who have undergone closure before 25 yr of age, life expectancy and functional outcome are usually normal. Unfortunately later closure remains a risk for premature late death. Although small ASDs are generally considered benign, like a patent foramen ovale (PFO) they can allow paradoxical embolization to occur. Some 25±30% of the adult population have a probe-patent PFO; in the vast majority of cases this will cause no long-term sequelae for the individual. There is, however, no doubt that in rare individuals, spontaneous cerebrovascular events related to a PFO can occur.56 This has led the American Heart Association4 to recommend that echocardiography is performed on all stroke victims aged under 45 yr. Unlike an ASD, PFOs do not permit a left-to-right shunt, but will permit a right-to-left shunt to occur if pressure in the right atrium exceeds that on the left, such as can occur during sneezing. Concern has been raised that venous air embolization during neurosurgical procedures may lead to paradoxical embolization in patients with a PFO. Whilst this event is undoubtedly possible, and venous air embolization can occur in up to 50% of neurosurgical cases,96 paradoxical embolization is in fact very rare.19 61

Traditionally, the closure of ASDs has required surgery. The era of transcatheter closure has now arrived, and today most ASDs can be closed percutaneously.14 15 The longterm results of the devices used to close these defects are currently unknown, although the short-term results are encouraging.

Cavopulmonary anastomoses Both tricuspid atresia and univentricular double-inlet ventricle are characterized by hypoplasia, or even absence, of one of the ventricular chambers, thereby precluding an anatomical biventricular repair. Additionally, an ultimate conversion to a univentricular circulation, as opposed to transplantation, may be chosen for children with hypoplastic left heart syndrome.28 Early attempts at palliation in these patients relied on the use of systemic-to-pulmonary arterial anastomoses. A univentricular circulation is inherently inef®cient because of the recirculation of the pulmonary and systemic venous return. Over time, the PVR falls, resulting in an increase in PBF, with a reduction in systemic ¯ow. Since the late 1950s, these patients have undergone palliative procedures in which the systemic venous blood is diverted to the pulmonary arteries, the single ventricle functioning as a systemic ventricle. Two classes of shunt have been developed. The ®rst was the Glenn shunt in which the SVC was connected to the right pulmonary artery. This only supplied blood to the right lung, and has largely been superseded by the bidirectional Glenn shunt in which the SVC is anastomosed to the main pulmonary artery. The effect of these shunts is to improve arterial saturation and exercise tolerance, although cyanosis remains because blood draining from the inferior vena cava (IVC) still mixes at atrial level. Long-term survival is possible, 60% of patients surviving for 25 yr.52 Its bene®t frequently reduces after 5±15 yr owing to the development of pulmonary arteriovenous malformations (AVM). It is thought that these develop as a consequence of hepatic venous blood bypassing the lungs; restoration of IVC ¯ow through the lungs has been associated with long-term improvement in arterial saturation.87 Because of long-term problems with the Glenn shunt, in most cases it is used as a staging procedure to a Fontan type of circulation. As originally described, the Fontan operation consisted of a classical Glenn shunt, insertion of a homograft valve in the IVC, closure of the ASD and connection of the right atrium and pulmonary artery by a valved homograft conduit. Late problems with the valved conduits rapidly led to their abandonment and a direct anastomosis between the right atrium and pulmonary artery. Intermediate and long-term follow up has shown that incorporation of the whole right atrium in the Fontan circulation is associated with a high incidence of atrial arrhythmias and thromboembolic complications.10 Turbulent blood ¯ow within the atrium is inef®cient, and the large right atrium permitted stasis, encouraging thrombus formation. The ®nding that the right

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Fig 1 Arrangement of circulatory connections following construction of a lateral tunnel Fontan. Ao, aorta; ASD, atrial septal defect; IVC, inferior vena cava; LA, left atrium, LPA, left pulmonary artery; LV, left ventricle; PV, pulmonary vein; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle; SVC, superior vena cava

atrium made an insigni®cant contribution to blood ¯ow in a valveless Fontan circulation led to the lateral tunnel Fontan. Here a baf¯e is placed in the right atrium to link the IVC and SVC and exclude most of the right atrium (Fig. 1). An alternative approach at producing a TCPC is the extracardiac Fontan, in which instead of an internal tunnel, an external conduit is placed. The short-term outcome of these two variants is identical, although the extra-cardiac operation has been associated with a greater risk of sinus node dysfunction.53 The physiological implications of a Fontan type of circulation are extreme.42 Blood ¯ow from the systemic venous circulation through the lungs is entirely passive. Factors that increase PVR will reduce PBF, resulting in a rise in central venous pressure and inadequate ®lling of the systemic ventricle, leading to a reduction in cardiac output. Systemic venous hypertension is an inevitable consequence of the absence of a right-sided pumping chamber and is commonly associated with development of peripheral oedema. Similarly, dysfunction of the systemic ventricle, which may be as mild as loss of sinus rhythm, leads to an increase in left atrial pressure and a reduction in PBF. In an attempt to avoid these problems, fenestration of the Fontan conduit is commonly used in high-risk patients. This allows

cardiac output to be maintained in the face of a reduction in PBF, albeit at the price of desaturation consequent on the right-to-left-shunt. In some patients, the fenestration is only required in the immediate operative period; in more borderline cases, it may remain patent inde®nitely. With a passive pulmonary circulation, hypovolaemia is extremely poorly tolerated, resulting in dramatic reductions in PBF and cardiac output. In healthy individuals, venous return is in¯uenced by the cardiopulmonary interaction whereby spontaneous respiration provides additional energy for forward blood ¯ow.83 In asymptomatic patients following TCPC palliation, this effect on venous return is enhanced, predominantly by effects on hepatic venous ¯ow.43 It has been speculated that any disturbance in respiratory mechanics in these patients, such as obstructive lung disease, may have a substantially greater adverse effect on the splanchnic circulation than in healthy individuals. The advantages of spontaneous respiration in this population are well established and results in lower pulmonary artery pressures and a higher cardiac index.57 The need to avoid respiratory depression, because of the carbon dioxide effects on PVR, and at the same time provide effective analgesia in these patients can be a challenge. It is widely accepted that the Fontan type of circulation is associated with progressive derangement in hepatic function that re¯ects a mild cholestatic picture.94 These changes are accompanied by both anticoagulant and procoagulant changes in the coagulation system. The anticoagulant changes are manifested by reductions in Factors V and VII, and prolongation of the prothrombin time, whilst the procoagulant changes are manifested by increases in the levels of factors VIII and X and prothrombin fraction, accompanied by reduced levels of antithrombin III, protein C and protein S.45 94 Some patients present with a mixed picture. A consequence of the procoagulant state that some patients develop is that overt and covert thromboembolism occurs in 18% of individuals.46 95 As well as pulmonary embolism, late stroke is a signi®cant problem in this population.16 One of the most debilitating long-term complications of a Fontan type of circulation is the development of protein-losing enteropathy, resulting in hypoproteinaemia. The effect of a successful Fontan type of circulation, without a fenestration, is that arterial saturation should rise to 95% breathing air, and most patients have a good functional outcome. Any saturation less than 90% should prompt referral to a GUCHD unit for investigation. The development of pulmonary AVMs, whilst less common than after a Glenn shunt, is ominous, often heralding clinical deterioration. The Fontan with the inclusion of the right atrium within the circulation is prone to right atrial dilatation, which acts as a substrate for the development of atrial arrhythmias, particularly IART. For the patient with a failing Fontan, particularly with arrhythmias, conversion to a TCPC circulation in combination with arrhythmia ablation provides an improvement in ventricular function

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and exercise tolerance, coupled with elimination of, or dramatic reduction in, arrhythmias.1 63 The long-term results of the TCPC operation are substantially better than the Fontan operation. Late arrhythmias, although less common than after a Fontan procedure,10 90 remain a problem and are associated with signi®cant morbidity. Pharmacological and anti-tachycardia pacing are only poorly effective. Electrophysiological ablation therapy has a 3 yr freedom from arrhythmia rate of 81%.100

Tetralogy of Fallot ToF is the commonest cause of cyanotic CHD in children, accounting for 10% of CHD. The condition is composed of four parts: a large non-restrictive VSD, RV out¯ow tract obstruction (RVOTO) (which is almost always infundibular but can also be valvular and supravalvular), RV hypertrophy and overriding of the aorta. Although four defects are present, the ToF embraces a spectrum of disease, from patients with a VSD, overriding of the aorta and minimal pulmonary stenosis to patients with a VSD and virtual pulmonary atresia. Uncorrected, 70% of children with ToF die before their tenth birthday, although survival into adulthood is possible.11 Patients with ToF almost invariably present with cyanosis, although in those with mild RVOTO it may be mild. This is due to right-to-left shunting through the large VSD. Unlike patients with isolated large VSDs, these patients do not go on to develop PVD because the lungs are protected by the out¯ow tract obstruction. The exceptions to this are children who have palliative arterial shunts to improve PBF, or those with abnormal pulmonary arterial connections. In children, the infundibular obstruction is very labile, giving risk to very characteristic hypercyanotic spells, a feature not seen in uncorrected adult patients. The traditional surgical approach for these patients has been to create a systemic-to-pulmonary arterial shunt where necessary in infancy, with de®nitive repair at the age of 2±5 yr. Whilst this strategy has been successful in many cases,64 it is associated with problems related to chronic hypoxia, progression of RV hypertrophy leading to RV dysfunction in later life, problems with the arterial shunt, pulmonary artery distortion and, rarely, pulmonary vascular disease. This has led to a dramatic change in surgical management, with complete correction being performed in early infancy.2 9 25 77 This can be performed with a low mortality, and has been associated with an excellent longterm outcome. It abolishes cyanosis early in life, normalizes PBF and promotes pulmonary arterial growth. However, many GUCHD patients will have undergone later operations. Some may have only received a palliative shunt. These latter patients should undergo repair as adults, although their long-term outcome is not as good as in those corrected in childhood.8 Early surgical correction techniques required a substantial ventriculotomy, the scarring from which has been associated with ventricular

arrhythmias in later life. More modern surgical repairs can sometimes close the VSD via a transatrial approach, obviating the need for ventriculotomy. A hallmark of the repair of ToF is the development of a right bundle branch block pattern, or even bifasicular block on the ECG. A consequence of the move to operation at ever earlier ages is that transannular patching of the pulmonary valve is frequently required,25 a procedure that is almost certain to be associated with pulmonary regurgitation. The majority of patients who have undergone repair of ToF are asymptomatic. Approximately 10% of patients may require late re-intervention. Long-term survival rates of 85% at 36 yr following repair are typical of those repaired as infants.69 LV function remains normal 24 yr after repair.68 Most problems encountered during late follow up relate to abnormalities of RV physiology, and include exercise intolerance, ventricular arrhythmias and sudden death secondary to chronic pulmonary regurgitation. Restrictive RV physiology, although hazardous at the time of surgery, confers long-term bene®ts following repair of ToF. It is associated with a reduction in pulmonary regurgitation, better exercise tolerance, less prolongation of the QRS duration and fewer symptomatic arrhythmias.30 More recent understanding of the effects of transannular patching have shown that whilst pulmonary regurgitation is an inevitable consequence, this is the only form of out¯ow repair that is associated with the development of restrictive physiology.70 For patients who develop haemodynamically signi®cant pulmonary regurgitation, late pulmonary valve replacement is associated with an improvement in exercise tolerance26 and a reduction in arrhythmias.92

Ventricular septal defects VSD is the commonest form of CHD. Unlike ASD, the majority will undergo spontaneous closure: 40% by the age of 2 yr and 90% by the age of 10 yr.74 VSD is subclassi®ed according to location; however, the physiological consequences of the defect depend on the size of defect and the extent of shunting. The extent of shunting depends on the relative resistance of the pulmonary and systemic vascular beds and the size of the defect. If the defect is small, there is only a minimal increase in PBF. Large defects on the other hand are associated with equalization of ventricular pressures and initially a marked increase in PBF consequent on the low resistance of the pulmonary circulation. With time, PVR starts to rise, accompanied by a reduction in shunt ¯ow and, if left untreated, Eisenmenger physiology will develop. The natural history of VSDs in adults depends largely on their size and associated shunt ¯ow. Patients with a VSD that has closed spontaneously and normal ventricular function have a normal life expectancy. Patients with a small restrictive VSD who have a normal PVR have an excellent prognosis,60 albeit with a considerable risk of endocarditis.67 With a small VSD, it is unlikely that PVD will develop in later life.50 99 Patients with a moderate or

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large VSD have a marked reduction in their 25-yr survival rate to only 86% and 61%, respectively.50 99 These patients are at risk of developing left ventricular dilatation and failure secondary to volume overload. Endocarditis and the development of Eisenmenger's syndrome remains a signi®cant risk. Patients with an outlet VSD, and some with a perimembranous VSD, have a tendency to develop progressive aortic regurgitation secondary to prolapse of one or more cusps of the aortic valve.82 These patients are at increased risk of endocarditis as well as aortic valve dysfunction, and require aortic valve repair if the aortic regurgitation is worse than mild in severity. Lun58 has recommended that a subarterial VSD 5 mm or larger should be closed as early as possible to prevent the secondary development of aortic valve prolapse. Traditionally, surgical closure of VSDs has been carried out using a RV approach. This has resulted in a very high incidence of inter-ventricular conduction disturbances owing to the proximity of the bundle of His. Interventional cardiologists have shown that device closure is certainly technically feasible,38 72 but so far long-term results are lacking. Even with a transcatheter method, conduction abnormalities have been reported, related to the proximity of critical conducting tissue to the defect.

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