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Adult congenital heart disease (grown-up congenital heart disease)

Andrea A Kelleher MB BS FRCA


Matrix reference 2A03, 3G00

Key points Ninety per cent of children now born with congenital heart disease survive until adulthood. There were an estimated 200 000 patients with adult congenital heart disease (ACHD) in the UK in 2010. These patients are increasingly presenting to their local hospitals for non-cardiac surgery. ACHD can be classied according to complexity into simple, moderate, and severe. A clear understanding of the anatomical conguration of the heart and great vessels and its associated pathophysiology is essential before contemplating anaesthesia in these patients.

Congenital heart disease (CHD) may be dened as a structural abnormality of the heart or intrathoracic great vessels that is present at birth and is, or may become, of functional signicance.1 This denition excludes congenital cardiomyopathies and arrhythmias such as Wolff Parkinson White syndrome even if they are due to abnormalities present at birth. CHD affects around 7.6 in every 1000 live births in the UK amounting to 5000 babies each year. Of these, 60% are diagnosed in infancy, 30% in childhood, and 10% as adults ( particularly secundum atrial septal defect, Ebsteins anomaly, coarctation of the aorta, and congenitally corrected transposition). The majority of patients with CHD are amenable to treatment by either device closure or surgery, and 90% of these children now survive to adulthood. Current estimates suggest that there are 1.2 million adults in Europe and a further 2 million in the USA with adult CHD (ACHD). In 2010, it was estimated that there were 200 000 people aged 16 and over with ACHD in the UK. Of these, 20 25% require life-long specialist care, 35 40% require access to expert consultation, and the remaining 40% need little ongoing support.

of patients. The relative frequencies of the most common lesions are shown in Table 2.

Simple ACHD
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Many of the simple forms of CHD such as small ventricular or atrial septal defects may close spontaneously or require relatively simple surgical or cardiological intervention. It is generally recommended that these patients are seen at least once in adulthood by a specialist centre as some may remain at risk of arrhythmias and require further follow-up. In the absence of such need, they may undergo non-cardiac surgery and anaesthesia in a general hospital without difculty.

Moderate complexity ACHD


Moderate complexity ACHD includes defects such as Fallots tetralogy (TOF) which is often accompanied by other non-cardiac abnormalities. The natural history of TOF (ventricular septal defect, over-riding aorta, pulmonary outow tract obstruction, and right ventricular hypertrophy) depends upon the severity of the right ventricular outow tract obstruction and surgery is typically scheduled around 6 months of age. If a pulmonary transannular patch is necessary, it will lead to pulmonary regurgitation with right ventricular dilatation and failure in adulthood. These patients require signicant further intervention often including major surgery, and although the 30 yr survival after TOF repair is 86%, the risk of sudden death due to pulmonary regurgitation or arrhythmias is signicant.3 Ebsteins anomaly involves atrialization of a portion of the right ventricle and apical displacement of a variably dysplastic tricuspid valve. Approximately 50% of patients have an associated atrial septal defect or patent foramen ovale, 50% have Wolff Parkinson White syndrome, and many have non-cardiac abnormalities. Tricuspid regurgitation may range from mild to severe and as a consequence, adult life may be complicated by right heart failure and arrhythmias.

Life expectancy and classication of ACHD


Patients with CHD have a shorter life expectancy than those with normal hearts. From the Dutch CONCOR National registry, 77% of patients with ACHD died due to a cardiovascular event, 26% of which were due to chronic heart failure (average age at death 51.0 yr) and 19% sudden death (average age at death 39.1 yr).2 Rather than using the structurally based classication of CHD commonly used in children, ACHD may be better classied according to complexity, that is, simple, moderate, and severe (Table 1). This allows analysis of the relationship between morbidity, mortality, and level of care required for these different groups

Andrea A Kelleher MB BS FRCA Consultant Cardiothoracic Anaesthetist Department of Anaesthesia and Intensive Care Royal Brompton Hospital London SW3 6NP UK Tel: 44 20 7351 8247/8526 Fax: 44 20 7351 8524 E-mail: aakelleher@rbht.nhs.uk (for correspondence)

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doi:10.1093/bjaceaccp/mkr045 Advance Access publication 22 December, 2011 Continuing Education in Anaesthesia, Critical Care & Pain | Volume 12 Number 1 2012 & The Author [2011]. Published by Oxford University Press on behalf of the British Journal of Anaesthesia. All rights reserved. For Permissions, please email: journals.permissions@oup.com

Adult congenital heart disease

Table 1 Classication of ACHD according to complexity Simple ACHD Unrepaired lesion Isolated aortic valve disease (13/1000 live births) Isolated mitral valve disease (excluding mitral cleft or parachute valve) Isolated patent foramen ovale, small atrioseptal defect, or ventricular septal defect Mild pulmonary stenosis Repaired lesion Previously ligated or occluded ductus arteriosus Repaired sinus venosus or secundum atrioseptal defect without residual defect Repaired ventricular septal defect without residual defect Moderate complexity ACHD Aorta to left ventricular stula Partial or total anomalous pulmonary venous drainage Atrioventricular canal defects partial or complete Coarctation of the aorta Ebsteins anomaly Signicant infundibular right ventricular outow tract obstruction Ostium primum or sinus venosus atrioseptal defect Unrepaired ductus arteriosus Moderate-to-severe pulmonary stenosis or regurgitation Sinus of valsalva stula or aneurysm Subvalvular or supravalvular aortic stenosis Tetralogy of Fallot Ventricular septal defect with associated anomaly, e.g. aortic regurgitation, absent valve, subaortic stenosis, mitral valve disease, right ventricular outow tract obstruction, straddling atrioventricular valve Severe complexity ACHD Conduits valved or non-valved All types of cyanotic heart disease Double-outlet ventricle Eisenmenger syndrome Fontan procedure or TCPC Mitral, tricuspid, or pulmonary atresia Pulmonary hypertension Any single-ventricle circulation Transposition of the great vessels Truncus arteriosus Very rare complex anomalies, e.g. crisscross heart, isomerism, ventricular inversion, heterotaxy syndromes

A combination of delayed femoral pulses, hypertension, and rib notching on the chest X-ray of an adult is pathognomonic of coarctation of the aorta. Despite corrective surgery, often in infancy, many patients have considerable ongoing morbidity; 70% develop hypertension, up to 54% require re-repair, and 85% have a bicuspid aortic valve. Regular follow-up is essential to treat the hypertension, screen for and treat premature coronary artery disease and aortic valve disease, and to check for recurrence or aneurysm formation. Despite regular follow-up, particularly if repair is delayed to late childhood, life expectancy is markedly reduced with a mean age at death of 38 yr. If patients with moderate complexity ACHD require noncardiac surgery in a non-specialist centre, it may be appropriate to discuss the details of their history and ongoing management with their specialist centre before undertaking anaesthesia.

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Severe complexity ACHD


Severe complexity ACHD includes all adult patients with cyanotic CHD or non-cyanotic single-ventricle physiology. The former may arise from an uncorrected anatomically balanced lesion and the latter the repair of a univentricular heart with the Fontan procedure or total cavopulmonary connection (TCPC). This is used to repair hypoplastic left heart, tricuspid atresia, and pulmonary atresia and involves the connection of the superior and inferior vena cavae to the right pulmonary artery either via the right atrium (Fontan) or directly (TCPC). Without a subpulmonary pumping chamber, blood ow through the lungs is passive and the cardiac output depends upon a relatively high central venous pressure, lower pulmonary vascular resistance, and low systemic atrial and ventricular diastolic pressures. Sinus rhythm is essential to maintain this gradient and its loss may be accompanied by a catastrophic reduction in cardiac output. Patients who rely on a high venous pressure to maintain their cardiac output tolerate hypovolaemia very poorly and considerable care should be devoted to maintaining perioperative euvolaemia, if surgery is undertaken. The Fontan or TCPC procedure is palliative and almost 20% of patients do not survive beyond their 20th birthday. Deterioration as an adult may present with cyanosis, ventricular failure ( particularly if the right ventricle is the systemic ventricle), thromboembolism, protein-losing enteropathy, and arrhythmias. Transposition of the great vessels is treated with a switch procedure either at the atrial (Senning or Mustard) or, more recently, arterial level. A switch at the atrial level involves the construction of an atrial bafe which redirects blood to the contralateral ventricle. Late complications of this procedure include right ventricular dysfunction (as this remains the systemic ventricle), atrial arrhythmias, tricuspid regurgitation, or an obstructed or leaky atrial bafe. The more recent arterial switch procedure involves transecting the great vessels, attaching them to the correct valve, and re-implanting the coronary arteries. This operation is usually performed in the neonatal period but may be complicated many years later by supra-aortic or pulmonary stenosis, impaired coronary

Table 2 Distribution of diagnosis of grown up congenital heart disease (GUCH) patients in the Dutch national registry (CONCOR programme)2 Diagnosis ASD VSD Tetralogy of Fallot Coarctation Aortic stenosis Pulmonary stenosis Bicuspid aortic valve Marfans syndrome Transposition of the great arteries Pulmonary atresia AVSD Ebsteins anomaly Other diagnosis Percentage 17 14 11 10 8 7 6 5 5 2 2 2 11

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perfusion, pulmonary branch stenosis, and aortic root dilatation with aortic regurgitation. Despite this, the outcomes are generally good and the oldest patients are now approaching 35 yr. When an abnormal communication exists between the outlet of the right and left sides of the heart such as in unrepaired truncus arteriosus or double-outlet ventricle, a balance is established in the blood ow to the pulmonary and systemic circulations. This is dependent upon the size of defect and the relative resistances of the two vascular beds. Although this situation rare in adults, it is important to be aware of it, as hyperventilation under anaesthesia in these patients can cause a dramatic reduction in pulmonary vascular resistance resulting in increased pulmonary blood ow, decreased systemic blood ow, and the rapid deterioration of a previously stable patient. Pulmonary hypertension occurs as a result of left-to-right shunts in which unrestricted pulmonary blood ow progresses to pulmonary vaso-occlusive disease. Eisenmengers syndrome represents end-stage pulmonary hypertension and may be dened as reversal of the original systemic to pulmonary shunt accompanied by cyanosis. Importantly for the anaesthetist, 20% of Eisenmenger patients die during a medical procedure, and extreme caution should be observed when contemplating anaesthesia. The mean age at death is around 45 yr; 6 yr on average from diagnosis. If patients with severe complexity heart disease need to undergo anaesthesia in a non-specialist centre, it is appropriate to discuss the details of each individual case with the physicians normally involved in their care. In many instances, it may be appropriate for all non-emergency surgery to be undertaken in the specialist centre. Even anaesthesia for minor non-cardiac surgery, particularly in patients with pulmonary hypertension, may carry a substantial risk of death.

If sinus rhythm is lost and cardioversion is contemplated in a patient with severe complexity ACHD, discussion with the specialist cardiac unit is advised and ideally unless moribund, the patient should be anticoagulated rst. If this is not possible, transoesophageal echocardiographic guidance should be sought to exclude intracardiac thrombus by an operator able to interpret complex congenital lesions.

Sudden cardiac death


Sudden cardiac death is associated with certain forms of ACHD, and unexplained syncope should be regarded as a grave sign. The conditions associated with the greatest risk include univentricular hearts, transposition, congenitally corrected transposition, coarctation of the aorta, TOF, and aortic stenosis. Treatment includes electrophysiological studies and catheter ablation. If this is unsuccessful, the implantation of a cardioverter debrillator is advised, although the benets in this patient population are not yet fully established.4

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Heart failure
Patients with cardiac failure related to ACHD are generally treated according to guidance developed for patients with acquired disease. There is no certainty that this is appropriate as the failing structurally normal heart may not be physiologically similar to the failing ACHD heart; however, cardiac resynchronization therapy is being successfully applied to ACHD patients in heart failure. Clearly, if a patient in heart failure presents for non-cardiac surgery, caution should be exercised when administering i.v. uids and perioperative inotropic support may be necessary. For many patients with complex ACHD, as heart failure progresses, transplantation may be their only survival option. Donor organs are, however, scarce and the surgery is complex in patients who have had previous thoracotomies and may have aortopulmonary collaterals. It is possible that ongoing research into long-term mechanical forms of support may show more promise for this group of patients.

Common complications, residual defects, or sequelae after cardiac surgery


The repair of congenital cardiac lesions may result in a variety of complications, residual defects, and sequelae of which the anaesthetist should be aware. These include electrophysiological disturbances, including sudden death, valvular disease, persistent shunts, myocardial dysfunction, vascular disorders, infectious or thromboembolic complications, and associated congenital or acquired noncardiac disease.

Infective endocarditis
Although infective endocarditis is an ever-present risk in ACHD, the risk varies markedly with different lesions. Ideally, all patients with ACHD should avoid tattoos and maintain good oral hygiene with regular visits to the dentist. Owing to the small risk of endocarditis associated with most lesions, combined with the risk of anaphylaxis to antibiotic therapy and the development of resistant organisms, it is now recommended that antibiotic prophylaxis is reserved only for those patients at highest risk (ESC 2009).5 These include the following patients undergoing invasive dental treatment: cyanotic CHD without previous repair, with residual defects, palliative shunts, or conduits;

Electrophysiological disorders
Arrhythmias are a common complication of ACHD. The loss of sinus rhythm may lead to signicant haemodynamic decompensation and also stasis and thromboembolism. Patients particularly prone to arrhythmias include those with previous ventriculotomy scars or a dilated right ventricle or atrium. Antiarrhythmic drugs are often poorly tolerated due to their negative inotropic effects, and the results of catheter ablation, although improving, are currently worse than in patients with structurally normal hearts.4

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CHD up to 6 months after repair with a device or prosthetic material when endothelialization is likely to be incomplete, or indenitely if there is a residual defect at the site of the prosthetic material or device; previous history of infective endocarditis; patients with a prosthetic valve or prosthetic material used in valve repair. It is not currently advised that these patients or others require antibiotic therapy for instrumentation of the respiratory, gastrointestinal, or genitourinary tracts, or of the skin or musculoskeletal systems unless infection is present. Strict asepsis should clearly be observed when inserting i.v. catheters or performing any other invasive procedure, and some physicians feel strongly that more routine antibiotic prophylaxis is appropriate in individual cases. The European Society of Cardiology acknowledges these views and states that they should be respected.5 Heart failure and embolism are the most common and severe complications of infective endocarditis. They are both difcult to treat and may require high-risk surgical intervention in addition to prolonged antibiotic therapy.

functional implications of the individual lesion and any associated acquired heart disease or other co-morbidity (Table 3). Those lesions associated with raised pulmonary vascular resistance represent the highest risk and should always be discussed with the specialist team before undertaking anaesthesia for routine non-cardiac surgery.

Pregnancy and genetic counselling


Although 25% of maternal deaths in the last 30 yr have been due to CHD, most patients with ACHD can undertake pregnancy without difculty. Counselling should be lesion and patient-specic and for all but the simplest lesions care should be provided in a specialist centre by an experienced multidisciplinary team. Those conditions listed in Table 4 have a very high risk of maternal or fetal mortality and affected patients are usually advised against pregnancy.7 In all other cases, the impact of pregnancy (increased cardiac output and decreased systemic vascular resistance) should be evaluated against the specic lesion to inform the discussion of risk with individual patients.

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Bleeding and thromboembolic disease


Patients with ACHD may exhibit a mild bleeding tendency (easy bruising, epistaxis, and haemoptysis) which may become lifethreatening in the context of major surgery or trauma. Platelet counts tend to be in the low normal range and their function is often abnormal. In addition, patients may be taking anticoagulants or antiplatelet agents to maintain shunt patency and there may also be associated abnormalities of the intrinsic and extrinsic clotting systems. Conversely, patients with cyanosis may develop a compensated erythrocytosis and although symptoms related to hyperviscosity are usually absent or mild, if the haematocrit is , 65 70%, the risk of venous thromboembolism is generally increased. The use of low molecular weight heparin thromboprophylaxis, elastic stockings, and early mobilization after surgery is essential.

Transition of care from the paediatric to the adult unit


Transfer of care from a paediatric to adult unit represents a difcult and uncertain time for patients with CHD. The timing varies between units and may involve transferring the care of an emotionally vulnerable teenager to a completely different hospital with different clinicians. Furthermore, patients at this age often have
Table 3 Factors to consider before undertaking anaesthesia in a patient with ACHD A clear understanding of the anatomical conguration of the heart and great vessels and its associated pathophysiology is essential A clear understanding of any associated non-cardiac disease and the often fragile emotional state of these patients when they discover they need to undergo anaesthesia and surgery The risk of associated cardiac arrhythmias, including the management of pacemakers and implanted debrillators The possible negative inotropic, vasodilatory effects of anaesthesia, or both, particularly in the presence of heart failure The impact of the chosen ventilatory strategy on the distribution of blood ow Awareness of possible residual defects in patients who have undergone previous repair (paradoxical embolism, air embolism, pulmonary hypertension, Eisenmengers syndrome) The need for antibiotic and thromboprophylaxis The risk of bleeding In older patients, additional co-morbid disease should be considered, e.g. coronary artery disease, heart failure, diabetes mellitus, renal impairment

Non-cardiac issues associated with ACHD


Renal impairment and urate metabolism abnormalities are common in cyanotic ACHD. These patients also face a wide array of noncardiac challenges, including issues related to exercise, sport, social interaction, pregnancy, and non-cardiac surgery. They may have considerable non-cardiac co-morbidity and the incidence of anxiety and depression in ACHD is signicantly higher than in the general population.6 They may be angry or fearful when medical treatment is required, particularly if this is outside their usual cardiac centre, and they should always be treated with sympathy and patience.

Table 4 ACHD in which pregnancy is very high risk Pulmonary hypertension (maternal mortality 30 50%) Arterial oxygen saturation , 85% (risk of fetal loss . 88%) Ejection fraction , 40% (NYHA III IV) (maternal mortality 5 30%) Aortic stenosis with an aortic valve area of , 1.5 cm2 or a gradient across the valve . 30 mm Hg (maternal mortality 530%) Dilated aortic root in Marfans syndrome (maternal mortality 530%)

Non-cardiac surgery in adults with ACHD


The risks to patients undergoing non-cardiac surgery can be greatly reduced by gaining an insight into the anatomical and

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limited understanding of their condition, and although specialization in ACHD is becoming more common, many clinicians may still have limited exposure to these patients within their normal practice. Transition may be improved by ensuring regular joint review of patients by the ACHD and paediatric teams as the time for transfer of care approaches. Thus, due to progressive advances in treatment, the mortality from CHD in both in childhood and adulthood continues to decline. This makes it increasingly likely that these patients will present for both medical and surgical management of non-related disease as adults, and an understanding of the anatomical and physiological implications of their cardiac disease and any sequelae or complications of previous treatment are important to the anaesthetist. In all but simple ACHD, it is wise to liaise with the centre providing their specialist care before non-cardiac anaesthesia or surgery.

References
1. Hoffman JIE, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39: 1890900 2. Verheught CL, Uiterwaal CSPM, Van der Velde ET et al. Mortality in adult congenital heart disease. Eur Heart J 2010, doi:10.1093/eurheartj/ ehq032 3. Ruiz JMO. Congenital heart disease in adults: residua, sequelae, and complications of cardiac defects repaired at an early age. Rev Esp Cardiol 2003; 56: 73 88 4. Baumgartner H, Bonhoeffer P, De Groot NMS et al. ESC guidelines for the management of grown-up congenital heart disease. Eur Heart J 2010; 31: 291557 5. Habib G, Hoen B, Tornos P et al. ESC guidelines on the prevention, diagnosis, and treatment of infective endocarditis. Eur Heart J 2009, doi:10.1093/eurheartj/ehp285 6. Kovacs AH, Saidi AS, Kuhl EA et al. Depression and anxiety in adult congenital heart disease: predictors and prevalence. Int J Cardiol 2009; 137: 15864 7. Dob DP , Yentis SM. Practical management of the parturient with congenital heart disease. Int J Obstet Anaesth 2006; 15: 137 44

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Declaration of interest
None declared.

Please see multiple choice questions 21 24.

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