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Adult congenital heart disease: a 2008 overview
1 Adult Congenital Heart Center and Center for Pulmonary Arterial Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK
2 National Heart and Lung Institute, Imperial College, London, UK
* Correspondence to: Michael A. Gatzoulis, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. E-mail: m.gatzoulis{at}rbht.nhs.uk
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Abstract |
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Abstract
IntroductionIntroduction: During the past decades, health care of patients born with congenital heart disease (CHD) has improved substantially, leading to a growing population of adult survivors.
Source of data: Using the recently published and relevant data on adult CHD (ACHD), we reviewed the most common congenital heart defects and discussed important related issues.
Areas of agreement: Adults with CHD most often require specialized medical or surgical care in a tertiary centre. However, this population also need local follow-up; general practitioners and other specialists therefore have to face the complexity of their disease.
Areas of controversies: Management of pregnancy, non-cardiac surgery, arrhythmias and endocarditis prophylaxis may be challenging in patients with CHD and should be adapted to their condition.
Growing points: The present article summarizes key clinical information on ACHD for the benefit of physicians who are not specialized in this field.
Areas timely for developing research: Research efforts and education strategies are greatly needed in order to optimize the care of patients with ACHD.
Keywords: Congenital • heart • adult
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Introduction |
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Congenital heart disease (CHD) is defined as a cardiovascular malformation that is present from birth. Approximately 4 to 10 live-born infants per 1000 are affected.1,2 The dramatic success of paediatric cardiology and surgery in the past 25 years has led to a marked increase in adult patients with CHD. In the UK, there are currently approximately 250 000 adults with CHD,3 and this number is growing. The most recent epidemiological study conducted in Quebec, Canada, revealed that the prevalence of adults with CHD was 4.09 per 1000 for the year 2000, representing an increase of 85% compared with 1985, while this increase was only 22% in children for the same period.4 The authors extrapolated their results to a US population and estimated that about 900 000 adults and 900 000 children had CHD in the year 2000 in the USA. Although 85% of children with CHD are now expected to survive into adulthood,5 a significant proportion of them will need further surgery or experience various complications such as arrhythmia and heart failure. The complexity of their disease poses a real challenge and requires expert cardiological care. Indeed, initial assessment of suspected CHD, follow-up of patients with CHD of moderate severity and complex lesions as well as risk assessment for non-cardiac surgery and pregnancy should ideally take place in a tertiary adult-care centre. However, primary-care physicians, general adult cardiologists, obstetricians, surgeons and anaesthesiologists should also be aware of the various issues in the general medical management of CHD because most patients will also need local follow-up for economic, social and geographic reasons.6,7 The present article provides simple and concise key clinical information concerning the adult with CHD. It should help physicians with different specialist backgrounds and the general practitioner to know when to refer these patients to a centre with the necessary expertise to help them.
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Overview of common CHD lesions |
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Table 1 describes in brief the anatomical findings, clinical features, management and complications of the most common CHD lesions, namely atrial septal defect (ASD), ventricular septal defect (VSD), atrioventricular septal defect (AVSD), tetralogy of Fallot (TOF), transposition of the great arteries (TGAs), single ventricle and Fontan circulation, aortic valve stenosis (AS)/bicuspid aortic valve, coarctation of the aorta, Ebstein anomaly of the tricuspid valve, pulmonary stenosis and patent ductus arteriosus. It also describes the Eisenmenger syndrome.
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Contraception and pregnancy |
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Half of the adult patients with CHD are women (most of them are of reproductive age) and cardiac disease is the leading cause of maternal death after suicide.93,94
Pregnancy represents a major stress for the cardiovascular system due to
- a 50% expansion in blood volume,
- an increase in heart rate from the 6th week of gestation, peaking at the 3rd trimester,
- a decrease in systemic vascular resistance (SVR) and
- a 50% increase in cardiac output.
Preconception counselling To avoid an unplanned and potentially dangerous pregnancy, women should be counselled in early adolescence about the risk of pregnancy, both to themselves and to the baby. Advice about contraception is essential. Ideally, both a cardiologist with training in CHD and an interested obstetrician/gynaecologist should be involved.
Information should include94
- contraception,
- maternal and foetal morbidity and mortality associated with pregnancy,
- risk of recurrence of CHD in the offspring,
- maternal (and paternal, when the partner has CHD) life expectancy; to avoid inaccurate beliefs and to make informed life choices95 and
- level of surveillance, need for treatment and anticipated hospitalisation required during pregnancy.
Contraception Unplanned pregnancies in women with CHD may carry significant risks; timely contraception counselling is therefore important and recommended. Even though there is no perfect contraceptive method, highly effective methods with a lower rate of side effects have been developed in recent years (Table 2).93,96
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Pregnancy: risks for the mother The risk of adverse cardiovascular events during pregnancy in woman with CHD depends on the underlying congenital condition with or without previous repair, current haemodynamic status and the functional capacity of the patient. Potential complications include arrhythmias, thromboembolic events, heart failure or pulmonary oedema, aortic dissection (for patients with coarctation, bicuspid aortic valve and Marfan syndrome), endocarditis and even death.97,98 Conditions associated with a higher maternal mortality rate and a significant increased risk in cardiovascular events during pregnancy are listed in Box 1.93,94,97,98
Box 1 Conditions associated with higher maternal mortality during pregnancy and the peripartum.
Other conditions with significant increased maternal risk of cardiovascular events
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*Pregnancy should be avoided and termination should be discussed if such a patient presents in early pregnancy.
Pregnancy: risks for the foetus In general, women with CHD have an approximate risk of transmission of 2–50% CHD to their offspring, depending on the underlying defect and the family history of CHD. For example, the recurrence risk has been reported to be99
- VSD and ASD: 6%,
- aortic stenosis: 8%,
- Marfan syndrome: 50% and
- TOF: 2% (50% if the patient has DiGeorge syndrome).
- women <20 or >35 years who had obstetric risk factors or multiple gestation,
- women who receive anticoagulants,
- left heart obstructive lesions,
- poor functional class (NYHA > II) prior to pregnancy and
- cyanosis.
Antenatal care While most of the low-risk patients can safely be cared for in their local hospital, moderate and high-risk patients should be looked after in a tertiary centre with experienced cardiologists, obstetricians, anaesthetists and neonatologists. Pregnant women with CHD will need more frequent visits, and some may benefit from hospitalization during the third trimester. Foetal echocardiography should be offered at 18–22 weeks.101,102 If intrauterine growth restriction is suspected (i.e. in cyanotic patients), close ultrasound surveillance is warranted.
Labour, delivery and postpartum period A detailed plan including timing and mode of delivery should be discussed in advance and the patient should be informed of the recommendations. Management of each case should be individualized, but there are some general principles:
- Vaginal delivery carries approximately half the risk of a caesarean section and is therefore preferable for the majority of patients, unless obstetric indications determine otherwise.
- Early epidural analgesia should be recommended to avoid increase in cardiac output associated with contraction and pain.
- Assisted delivery either by ventouse or forceps should be performed if the second stage of labour is prolonged.
- Endocarditis prophylaxis should be considered for patients with CHD undergoing instrumented delivery.
- The early postpartum is a potentially high-risk period and should be monitored closely. Volume overload can occur after uterine retraction associated with transfusion of extra blood into the maternal circulation. Oxytocic drugs (improving uterine contraction) should be used cautiously because of their major haemodynamic effects.
- Puerperal thromboprophylaxis with low-molecular-weight heparin should be routinely administered until the mother is fully mobilized.
Pregnancy in women with CHD: when to refer?
- In early adolescence, for counselling about risks of pregnancy and advice on contraception.
- When the patient wants to become pregnant, to reassess her haemodynamic status before conception and therefore update her current risk.
- Early in pregnancy to discuss plans and decide about management.
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Non-cardiac surgery |
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Assessment of adult patients with CHD undergoing non-cardiac surgery can represent a real challenge. Published guidelines to assist physicians in the cardiovascular evaluation for non-cardiac surgery103 were developed for acquired heart disease, but many of the specific issues regarding CHD are not addressed. Several principles proposed in the ACC/AHA guidelines apply in an adult CHD (ACHD) population and this systematic approach with an 8-step algorithm should be considered.103 However, a strategy specifically targeting patients with CHD has been proposed and is summarized in Box 2.104
Box 2 Strategy for assessment and management of the ACHD patient through non-cardiac surgery.
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Modified from Colman.104
The first step in the preoperative evaluation of patients with CHD is to determine the patient's cardiac anatomy by finding details about the primary defect and previous palliative or corrective surgeries. These important data should be explained to the surgeon and the anaesthesiologist before the surgery. Global assessment including patient's clinical predictors as described in the ACC/AHA guidelines (e.g. presence of heart failure, renal dysfunction, diabetes, etc.) and the risk of the proposed surgery itself should then be determined.
Risk factors specific to ACHD population (Table 3) need to be considered and added to the patient's clinical predictors. It is mandatory to identify these risk factors because their presence can increase the overall risk of the surgery and they may require specific perioperative management strategies (see Table 3).
Perioperative management Low-risk patients undergoing a low-risk procedure may have their surgery in a community hospital after preoperative assessment in a tertiary centre, but patients with moderate or complex CHD ideally should be operated in an ACHD centre. Perioperative management of conditions specific to an ACHD population is summarized in Table 3. Management of chronic anticoagulation needs to be individualized according to each patient's risk of thromboembolism associated with their anatomy and the presence of additional risk factors (atrial arrhythmias, previous thromboembolic events). Patients with mechanical valves can, in general, be managed following the 2006 ACC/AHA guidelines for management of patients with valvular heart disease.59 Postoperative atrial arrhythmias are more likely to occur in patients with late repair ASD or unrepaired ASD in older patients, history of atrial surgery, Fontan circulation, systemic ventricular dysfunction and Ebstein anomaly with pre-excitation. There are no current guidelines for prophylactic treatment of arrhythmias in non-cardiac surgery. If they occur, standard antiarrhythmic management should be considered for the symptomatic and/or haemodynamically compromised patients. Regarding endocarditis prophylaxis, please see the relevant section.
Anaesthetic management Anaesthetic management should ideally be provided by a senior anaesthesiologist with experience in ACHD. The anaesthesiologist should consider the potentially harmful haemodynamic effects related to each anaesthetic technique and agent used.105 For example, fall in SVR induced by spinal/epidural anaesthesia can increase a pulmonary to systemic shunt and, consequently, enhance hypoxemia. Also, a reduction in preload occurring after spinal/epidural anaesthesia can significantly decrease pulmonary blood flow. For induction, ‘haemodynamically stable’ agents (e.g. narcotics) should be selected to avoid an abrupt drop in SVRs.
Vascular access may be difficult in patients with CHD for various reasons104:
- Blalock-Taussig shunt: pressure measurement may be inaccurate distal to the shunt even if a subclavian artery was not interrupted.
- Classical Glenn shunt (see Table 1): no venous access to the right heart from the arm.
- Fontan circuit: catheters placed into the circuit may provoke venous thrombosis.
- Pulmonary artery catheterization is often more difficult (e.g. atrial baffle) and more dangerous (e.g. pulmonary hypertension).
Postoperative period Complications such as thromboembolic events, haemorrhage or infection are more likely to occur in the postoperative period; close monitoring is thus mandatory, especially for high-risk patients who usually benefit from management in an intensive care unit. Special care should be taken to ensure that pain is adequately relieved and thromboprophylaxis is provided.
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Arrhythmias in ACHD |
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Arrhythmias now represent one of the most common long-term complications in this population, posing new challenges in terms of management. Rhythm anomalies can be part of the natural history of the underlying defect itself, but most of them arise from consequences of surgical interventions or from longstanding haemodynamic abnormalities. Specific arrhythmias associated with CHD and their management106 are summarized below and in Table 4.
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Intra-atrial re-entrant tachycardia Intra-atrial re-entrant tachycardia (IART) is the most common arrhythmia diagnosed in the ACHD population. The mechanism is a macroreentry within the right atrium, usually occurring years after surgical intervention involving the right atrial tissue. This ‘atypical’ atrial flutter is more frequent in patients with previous Mustard and Senning procedures, or classic Fontan operations (severe dilation and scarring of the right atrium), but it can also occur even after simple ASD closure. Atrial rates are usually between 150 and 250 min–1, and 1:1 A:V conduction can lead to severe symptoms such as syncope and even cardiac arrest. Therapeutic options include the following106:
- Acute episode can be terminated with electrical cardioversion, antiarrhythmic drugs or overdrive pacing.
- Long-term antiarrhythmic drug therapy: often ineffective.
- Catheter ablation107: now often used as an early intervention. Good short- to mid-term success reduces the frequency of episodes, but high recurrence rates have been reported.
- Pacemaker implantation: used for patients with tachy–brady syndrome; decrease in IART frequency by correcting bradycardia and by atrial antitachycardia pacing capability.
- Surgical ablation with a modified right atrial maze operation: low rates of recurrence but associated with surgical risks. Usually performed in patients who already need a cardiac surgery for other indications.
Re-entrant tachyarrhythmias secondary to accessory pathways
Wolff–Parkinson–White syndrome occurs in 
20% of patients with Ebstein's anomaly. About half of them have multiple accessory pathways. Even if the success rate is lower than in the general population, catheter ablation remains the procedure of choice for Wolff–Parkinson–White syndrome in Ebstein's patients.106
Atrial fibrillation CHD conditions associated with left atrial pressure and/or volume overload predispose to atrial fibrillation. Mitral valve abnormalities (or left AV valve in the setting of an AVSD), aortic stenosis, left ventricular dysfunction (or systemic RV dysfunction in the setting of a ccTGA) with elevated end-diastolic pressures and unrepaired single ventricle are such defects. General management of this arrhythmia in patients with CHD does not differ from its treatment in other cardiac diseases. However, early intervention to improve any compromising haemodynamic abnormality should be considered.
Ventricular tachycardia VT and sudden cardiac death have become a major issue in selected patients with CHD reaching adulthood. Macroreentrant circuits in the regions of a previous surgical scar can occur following a ventriculotomy or patching of a VSD. Other mechanisms that might be involved in arrhythmogenesis are severe ventricular dysfunction and/or hypertrophy with fibrosis. TOF, aortic valve disease, cc-TGA with systemic RV dysfunction, Eisenmenger's syndrome and severe Ebstein's anomaly are lesions with an increased risk of VT.
Sinus node dysfunction Sinus node dysfunction occurring late after surgical repair is the most common cause of sinus bradycardia in patients with CHD. Mustard, Senning, Glenn and Fontan operations can all be associated with sinus node dysfunction. Pacemaker implantation is indicated if the bradycardia is associated with symptoms. Left atrial isomerism is associated with absence of the sinus node and with atrial or junctional escape rhythm with bradycardia.
AV block Both congenitally corrected TGAs and AVSD carry an intrinsic lifelong risk of developing AV block. Complete AV block can also result from surgical trauma following VSD closure, resection of left ventricular outflow tract obstruction or replacement/repair of an AV valve. Postoperative AV block is often transient, but pacemaker implantation is indicated if it does not recover within 7–10 days of surgery.
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Endocarditis prophylaxis |
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Endocarditis is a life-threatening disease with an in-hospital mortality rate of approximately 16–20% despite improvement in medical care.108–110 Turbulent blood flow across a narrowed orifice from a high- to a low-pressure chamber or vessel (as found in many CHD lesions) promotes endothelial damage, which may predispose to the development of endocarditis. Prevention of endocarditis involves identification of high-risk CHD conditions (Table 5) and of procedures carrying significant risk of bacteraemia (Table 6). However, prospective, randomized, placebo-controlled studies are lacking, and recommendations have changed substantially over the past 10 years.111,112 Current ACC/AHA and British Society for Antimicrobial Chemothrapy guidelines are somewhat controversial and much more restrictive concerning the use of prophylactic antibiotics.111,113 However, we advocate a prudent approach for endocarditis prophylaxis in patients with CHD based on previous British Cardiac Society recommendations, as suggested in Tables 5 and 6.114–116
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Dental procedures carry a significant risk of bacteraemia, but poor oral hygiene and dental disease might represent an even more important cause of endocarditis.111,117 Emphasis should therefore focus on good oral hygiene including daily brushing and flossing as well as regular visits to the dentist to decrease the frequency of bacteraemia in daily life.
Even if it is not clear which dental, genitourinary tract, skin, gastrointestinal or respiratory tract, procedures are more likely to result in more extensive bacteraemia, it is prudent to recommend endocarditis prophylaxis in moderate- and high-risk patients undergoing the following procedures (Table 6).
Tables 7 and 8 summarize the prophylactic antibiotic regimens for dental, oral, respiratory tract, oesophageal, genitourinary and gastrointestinal procedures.116
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Late repair and re-operation |
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Most early interventions performed in patients with CHD are not curative. A significant proportion of patients will require further surgery in adult life, either for re-operations after ‘correction’ in childhood or for repair of residual defects.118 Some patients never had previous repair of their defect and would benefit from primary correction even if they have reached adulthood. The next section will help health-care givers to understand the major indications for late repair and for re-operation of patients with CHD and will also indicate how to assess these patients preoperatively.119
Late correction Principal reasons why patients with CHD may not have had early correction are (1) they had a late diagnosis, (2) they grew up where there was no local surgical facility, (3) they were considered inoperable when first seen and (4) they have complex lesions with balanced systemic and pulmonary blood flow. In general, primary correction in adult life should be considered in non-cyanotic defect without pulmonary vascular disease (e.g. ASD, VSD, aortic stenosis, etc.; see Table 1). Cyanotic heart diseases including VSD with right ventricular outflow tract obstruction, TOF and possibly pulmonary atresia with VSD should also be considered for repair after exclusion of pulmonary vascular disease (uncommon in this setting).
Re-operation Most common conditions that require re-operation include the following:
- Pulmonary valve or right ventricle-to-pulmonary artery conduit stenosis, for example, after pulmonary valvotomy or after repair of TOF or pulmonary atresia with VSD. This may require replacement of conduit or pulmonary valve replacement.
- Severe pulmonary regurgitation following TOF: pulmonary valve insertion.
- Aortic restenosis or regurgitation following previous aortic valve surgery or balloon valvuloplasty for AS: redo aortic valve replacement.
- Recoarctation of the aorta: redo-repair (surgical or transcatheter with intravascular stenting).
Pre-operative assessment Most re-operations will need re-sternotomy, with associated risks of damage to cardiac structures, great vessels or extra-cardiac conduits. Late correction demands perfect knowledge of the underlying anatomy. A complete preoperative evaluation can reduce the risks of complications and should include
- magnetic resonance imaging to establish the relationship of the structures to the sternum in patients with previous repair;
- assessment of biventricular function;
- assessment of the pulmonary vasculature (eliminate pulmonary vascular disease and peripheral pulmonary artery stenosis);
- assessment of possible aortopulmonary collaterals;
- evaluation of any aortic regurgitation and aortic root dilatation and
- selective coronary angiography in patients older than 40 years referred for CHD surgery, or following re-implantation of coronaries, i.e. aortic root replacement (exclude presence of atheromatous coronary artery disease and/or anomalous coronary artery or iatrogenic damage to coronary arteries).
Special considerations Cyanosis increases the risk of postoperative bleeding due to abnormal platelet and coagulation pathways and friable collateral vessels that are difficult to coagulate (see Table 3). Furthermore, patients with cyanosis often have limited cardiac reserve and are prone to renal dysfunction and to perioperative myocardial injury; cardiac output and fluid balance must be carefully monitored and managed in the post-operative period.
Post-operative arrhythmias (mostly atrial) increase morbidity after cardiac surgery. Arrhythmia targeting surgery as an adjunct to the haemodynamic component of surgery in patients with pre-operative atrial fibrillation or flutter should be seriously considered.
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Conclusion |
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Increasing numbers of CHD patients are now surviving to adulthood. Over the past few decades, there has been major progress in the understanding and management of CHD, including identification of genetic causes for some defects. Although early interventions have transformed the outcome for these patients, many of them have ongoing problems that require tertiary cardiac care in adult life. Furthermore, most adults with CHD require additional non-tertiary care for issues such as pregnancy, non-cardiac surgery, endocarditis and other problems. Our purpose in writing this article was to give some guidance to the general physicians and surgeons managing this difficult group of patients.
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Funding |
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We thank the Cardiology Institute of Quebec, Laval University and the Cardiologists Association of the province of Quebec for their financial support. MAG and the Royal Brompton Adult Congenital Heart Programme have received support from the British Heart Foundation.
Accepted for publication January 21, 2008.
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References |
|---|
- Pierpont ME, Basson CT, Benson DW, et al. Genetic basis for congenital heart defects: current knowledge. A scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. Circulation (2007) 115:3015–3038.
[Abstract/Free Full Text] - Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol (2002) 39:1890–1900.
[Abstract/Free Full Text] - Gatzoulis MA. Adult congenital heart disease: education, education, education. Nat Clin Pract Cardiovasc Med (2006) 3:2–3.[CrossRef][Web of Science][Medline]
- Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation (2007) 115:163–172.
[Abstract/Free Full Text] - Nieminen HP, Jokinen EV, Sairanen HI. Late results of pediatric cardiac surgery in Finland: a population-based study with 96% follow-up. Circulation (2001) 104:570–575.
[Abstract/Free Full Text] - Mackie AS, Pilote L, Ionescu-Ittu R, Rahme E, Marelli AJ. Health care resource utilization in adults with congenital heart disease. Am J Cardiol (2007) 99:839–843.[CrossRef][Web of Science][Medline]
- Gurvitz MZ, Inkelas M, Lee M, et al. Changes in hospitalization patterns among patients with congenital heart disease during the transition from adolescence to adulthood. J Am Coll Cardiol (2007) 49:875–882.
[Abstract/Free Full Text] - Gatzoulis MA, Freeman MA, Siu SC, Webb GD, Harris L. Atrial arrhythmia after surgical closure of atrial septal defects in adults. N Engl J Med (1999) 340:839–846.
[Abstract/Free Full Text] - Webb G, Gatzoulis MA. Atrial septal defects in the adult: recent progress and overview. Circulation (2006) 114:1645–1653.
[Free Full Text] - Inglessis I, Landzberg MJ. Interventional catheterization in adult congenital heart disease. Circulation (2007) 115:1622–1633.
[Free Full Text] - Murphy JG, Gersh BJ, McGoon MD, et al. Long-term outcome after surgical repair of isolated atrial septal defect: follow-up at 27 to 32 years. N Engl J Med (1990) 323:1645–1650.[Abstract]
- Attie F, Rosas M, Granados N, Zabal C, Buendia A, Calderon J. Surgical treatment for secundum atrial septal defects in patients >40 years old: a randomized clinical trial. J Am Coll Cardiol (2001) 38:2035–2042.
[Abstract/Free Full Text] - Lindsey JB, Hillis LD. Clinical update: atrial septal defect in adults. Lancet (2007) 369:1244–1246.[CrossRef][Web of Science][Medline]
- Konstantinides S, Geibel A, Olschewski M, et al. A comparison of surgical and medical therapy for atrial septal defect in adults. N Engl J Med (1995) 333:469–473.
[Abstract/Free Full Text] - Thanopoulos BV, Rigby ML, Karanasios E, et al. Transcatheter closure of perimembranous ventricular septal defects in infants and children using the Amplatzer perimembranous ventricular septal defect occluder. Am J Cardiol (2007) 99:984–989.[CrossRef][Web of Science][Medline]
- Kidd L, Driscoll DJ, Gersony WM, et al. Second natural history study of congenital heart defects: results of treatment of patients with ventricular septal defects. Circulation (1993) 87:I38–I51.[Medline]
- Gabriel HM, Heger M, Innerhofer P, et al. Long-term outcome of patients with ventricular septal defect considered not to require surgical closure during childhood. J Am Coll Cardiol (2002) 39:1066–1071.
[Abstract/Free Full Text] - Minette MS, Sahn DJ. Ventricular septal defects. Circulation (2006) 114:2190–2197.
[Abstract/Free Full Text] - Fu YC, Bass J, Amin Z, et al. Transcatheter closure of perimembranous ventricular septal defects using the new Amplatzer membranous VSD occluder: results of the U.S. phase I trial. J Am Coll Cardiol (2006) 47:319–325.
[Abstract/Free Full Text] - El-Najdawi EK, Driscoll DJ, Puga FJ, et al. Operation for partial atrioventricular septal defect: a forty-year review. J Thorac Cardiovasc Surg (2000) 119:880–889. discussion 889–890.
[Abstract/Free Full Text] - Craig B. Atrioventricular septal defect: from fetus to adult. Heart (2006) 92:1879–1885.
[Free Full Text] - Shinebourne EA, HS. Atrioventricular septal defect: complete and partial (ostium primum atrial septal defect). In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 179–187.
- Najm HK, Coles JG, Endo M, et al. Complete atrioventricular septal defects: results of repair, risk factors, and freedom from reoperation. Circulation (1997) 96.
- Bergin ML, Warnes CA, Tajik AJ, Danielson GK. Partial atrioventricular canal defect: long-term follow-up after initial repair in patients > or = 40 years old. J Am Coll Cardiol (1995) 25:1189–1194.[Abstract]
- Goldmuntz E, Clark BJ, Mitchell LE, et al. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol (1998) 32:492–498.
[Abstract/Free Full Text] - Gatzoulis MA, Balaji S, Webber SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet (2000) 356:975–981.[CrossRef][Web of Science][Medline]
- Bashore TM. Adult congenital heart disease: right ventricular outflow tract lesions. Circulation (2007) 115:1933–1947.
[Free Full Text] - Therrien J, Siu SC, Harris L, et al. Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot. Circulation (2001) 103:2489–2494.
[Abstract/Free Full Text] - Gatzoulis MA, Till JA, Somerville J, Redington AN. Mechanoelectrical interaction in tetralogy of Fallot: QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation (1995) 92:231–237.
[Abstract/Free Full Text] - Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med (1993) 329:593–599.
[Abstract/Free Full Text] - Gatzoulis MA. Tetralogy of Fallot. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone, 315–326.
- Goldmuntz E, Geiger E, Benson DW. NKX2.5 mutations in patients with tetralogy of Fallot. Circulation (2001) 104:2565–2568.
[Abstract/Free Full Text] - Ghai A, Silversides C, Harris L, Webb GD, Siu SC, Therrien J. Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of Fallot. J Am Coll Cardiol (2002) 40:1675–1680.
[Abstract/Free Full Text] - Roos-Hesselink J, Perlroth MG, McGhie J, Spitaels S. Atrial arrhythmias in adults after repair of tetralogy of Fallot: correlations with clinical, exercise, and echocardiographic findings. Circulation (1995) 91:2214–2219.
[Abstract/Free Full Text] - Niwa K, Siu SC, Webb GD, Gatzoulis MA. Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot. Circulation (2002) 106:1374–1378.
[Abstract/Free Full Text] - Nollert G, Fischlein T, Bouterwek S, Bohmer C, Klinner W, Reichart B. Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair. J Am Coll Cardiol (1997) 30:1374–1383.[Abstract]
- Coats L, Khambadkone S, Derrick G, et al. Physiological and clinical consequences of relief of right ventricular outflow tract obstruction late after repair of congenital heart defects. Circulation (2006) 113:2037–2044.
[Abstract/Free Full Text] - Khambadkone S, Coats L, Taylor A, et al. Percutaneous pulmonary valve implantation in humans: results in 59 consecutive patients. Circulation (2005) 112:1189–1197.
[Abstract/Free Full Text] - Gatzoulis MA, Walters J, McLaughlin PR, Merchant N, Webb GD, Liu P. Late arrhythmia in adults with the mustard procedure for transposition of great arteries: a surrogate marker for right ventricular dysfunction? Heart (2000) 84:409–415.
[Abstract/Free Full Text] - Lange R, Horer J, Kostolny M, et al. Presence of a ventricular septal defect and the Mustard operation are risk factors for late mortality after the atrial switch operation: thirty years of follow-up in 417 patients at a single center. Circulation (2006) 114:1905–1913.
[Abstract/Free Full Text] - Hornung T. Transposition of the great arteries. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 349–362.
- Gelatt M, Hamilton RM, McCrindle BW, et al. Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience. J Am Coll Cardiol (1997) 29:194–201.[Abstract]
- Kammeraad JA, van Deurzen CH, Sreeram N, et al. Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries. J Am Coll Cardiol (2004) 44:1095–1102.
[Abstract/Free Full Text] - Legendre A, Losay J, Touchot-Kone A, et al. Coronary events after arterial switch operation for transposition of the great arteries. Circulation (2003) 108((Suppl 1)).
- Warnes CA. Transposition of the great arteries. Circulation (2006) 114:2699–2709.
[Abstract/Free Full Text] - Graham TP. Congenitally Corrected Transposition. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 379–387.
- Graham TP Jr, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol (2000) 36:255–261.
[Abstract/Free Full Text] - Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults: second of two parts. N Engl J Med (2000) 342:334–342.
[Free Full Text] - Prieto LR, Hordof AJ, Secic M, Rosenbaum MS, Gersony WM. Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries. Circulation (1998) 98:997–1005.
[Abstract/Free Full Text] - Langley SM, Winlaw DS, Stumper O, et al. Midterm results after restoration of the morphologically left ventricle to the systemic circulation in patients with congenitally corrected transposition of the great arteries. J Thorac Cardiovasc Surg (2003) 125:1229–1241.
[Abstract/Free Full Text] - Khairy P, Poirier N, Mercier LA. Univentricular heart. Circulation (2007) 115:800–812.
[Abstract/Free Full Text] - Veldtman GR, Nishimoto A, Siu S, et al. The Fontan procedure in adults. Heart (2001) 86:330–335.
[Abstract/Free Full Text] - Gatzoulis MA, Munk MD, Williams WG, Webb GD. Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology. Heart (2000) 83:51–57.
[Abstract/Free Full Text] - Mavroudis C, Deal BJ, Backer CL. The beneficial effects of total cavopulmonary conversion and arrhythmia surgery for the failed Fontan. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu (2002) 5:12–24.[CrossRef][Medline]
- Freedom RM, Li J, Yoo S-J. Late complications following the Fontan operation. Diagnosis and Management of Adult Congenital Heart Disease (2003) 85–91.
- Aboulhosn J, Child JS. Left ventricular outflow obstruction: subaortic stenosis, bicuspid aortic valve, supravalvar aortic stenosis, and coarctation of the aorta. Circulation (2006) 114:2412–2422.
[Free Full Text] - Fedak PW, Verma S, David TE, Leask RL, Weisel RD, Butany J. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation (2002) 106:900–904.
[Free Full Text] - Pellikka PA, Sarano ME, Nishimura RA, et al. Outcome of 622 adults with asymptomatic, hemodynamically significant aortic stenosis during prolonged follow-up. Circulation (2005) 111:3290–3295.
[Abstract/Free Full Text] - Bonow RO, Carabello BA, Kanu C, et al. ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): developed in collaboration with the Society of Cardiovascular Anesthesiologists: endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. Circulation (2006) 114:e84–e231.
[Free Full Text] - Carabello BA. Evaluation and management of patients with aortic stenosis. Circulation (2002) 105:1746–1750.
[Free Full Text] - Kaemmerer H. Aortic coarctation and interrupted aortic arch. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 253–264.
- Campbell M. Natural history of coarctation of the aorta. Br Heart J (1970) 32:633–640.
[Abstract/Free Full Text] - Cowley CG, Orsmond GS, Feola P, McQuillan L, Shaddy RE. Long-term, randomized comparison of balloon angioplasty and surgery for native coarctation of the aorta in childhood. Circulation (2005) 111:3453–3456.
[Abstract/Free Full Text] - de Divitiis M, Pilla C, Kattenhorn M, et al. Ambulatory blood pressure, left ventricular mass, and conduit artery function late after successful repair of coarctation of the aorta. J Am Coll Cardiol (2003) 41:2259–2265.
[Abstract/Free Full Text] - McCrindle BW, Jones TK, Morrow WR, et al. Acute results of balloon angioplasty of native coarctation versus recurrent aortic obstruction are equivalent: valvuloplasty and angioplasty of congenital anomalies (VACA) registry investigators. J Am Coll Cardiol (1996) 28:1810–1817.[Abstract]
- Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of the aorta: long-term follow-up and prediction of outcome after surgical correction. Circulation (1989) 80:840–845.
[Abstract/Free Full Text] - Sondergaard L, Cullen S. Ebstein anomaly. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 283–287.
- Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's anomaly. Circulation (2007) 115:277–285.
[Free Full Text] - 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.[Abstract]
- MacLellan-Tobert SG, Driscoll DJ, Mottram CD, Mahoney DW, Wollan PC, Danielson GK. Exercise tolerance in patients with Ebstein's anomaly. J Am Coll Cardiol (1997) 29:1615–1622.[Abstract]
- Cappato R, Schluter M, Weiss C, et al. Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein's anomaly. Circulation (1996) 94:376–383.
[Abstract/Free Full Text] - Quinonez LG, Dearani JA, Puga FJ, et al. Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. J Thorac Cardiovasc Surg (2007) 133:1303–1310.
[Abstract/Free Full Text] - Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC Jr. Operative treatment of Ebstein's anomaly. J Thorac Cardiovasc Surg (1992) 104:1195–1202.[Abstract]
- Dore A. Pulmonary stenosis. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 299–303.
- Hayes CJ, Gersony WM, Driscoll DJ, et al. Second natural history study of congenital heart defects: results of treatment of patients with pulmonary valvar stenosis. Circulation. 87:I28–I37.
- Kanter KR, Budde JM, Parks WJ, et al. One hundred pulmonary valve replacements in children after relief of right ventricular outflow tract obstruction. Ann Thorac Surg (2002) 73:1801–1806. discussion 1806–1807.
[Abstract/Free Full Text] - Jarrar M, Betbout F, Farhat MB, et al. Long-term invasive and noninvasive results of percutaneous balloon pulmonary valvuloplasty in children, adolescents, and adults. Am Heart J (1999) 138:950–954.[CrossRef][Web of Science][Medline]
- Schneider DJ, Moore JW. Patent ductus arteriosus. Circulation (2006) 114:1873–1882.
[Free Full Text] - Brili SV, Toutouzas P. Patent duct and aortopulmonary window. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 247–252.
- Cambier PA, Kirby WC, Wortham DC, Moore JW. Percutaneous closure of the small (less than 2.5 mm) patent ductus arteriosus using coil embolization. Am J Cardiol (1992) 69:815–816.[CrossRef][Web of Science][Medline]
- Yan C, Zhao S, Jiang S, et al. Transcatheter closure of patent ductus arteriosus with severe pulmonary arterial hypertension in adults. Heart (2007) 93:514–518.
[Abstract/Free Full Text] - Fisher RG, Moodie DS, Sterba R, Gill CC. Patent ductus arteriosus in adults—long-term follow-up: nonsurgical versus surgical treatment. J Am Coll Cardiol (1986) 8:280–284.[Abstract]
- Pass RH, Hijazi Z, Hsu DT, Lewis V, Hellenbrand WE. Multicenter USA Amplatzer patent ductus arteriosus occlusion device trial: initial and one-year results. J Am Coll Cardiol (2004) 44:513–519.
[Abstract/Free Full Text] - Singh TP, Rohit M, Grover A, Malhotra S, Vijayvergiya R. A randomized, placebo-controlled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am Heart J (2006) 151:851–855.
- Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation (2007) 115:1039–1050.
[Abstract/Free Full Text] - Levy M, Maurey C, Celermajer DS, et al. Impaired apoptosis of pulmonary endothelial cells is associated with intimal proliferation and irreversibility of pulmonary hypertension in congenital heart disease. J Am Coll Cardiol (2007) 49:803–810.
[Abstract/Free Full Text] - D'Alto M, Vizza CD, Romeo E, et al. Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisenmenger physiology): safety, tolerability, clinical, and haemodynamic effect. Heart (2007) 93:621–625.
[Abstract/Free Full Text] - Galie N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation (2006) 114:48–54.
[Abstract/Free Full Text] - Broberg CS, Bax BE, Okonko DO, et al. Blood viscosity and its relationship to iron deficiency, symptoms, and exercise capacity in adults with cyanotic congenital heart disease. J Am Coll Cardiol (2006) 48:356–365.
[Abstract/Free Full Text] - Benza RL, Rayburn BK, Tallaj JA, et al. Efficacy of bosentan in a small cohort of adult patients with pulmonary arterial hypertension related to congenital heart disease. Chest (2006) 129:1009–1015.[CrossRef][Web of Science][Medline]
- Michelakis ED, Tymchak W, Noga M, et al. Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension. Circulation (2003) 108:2066–2069.
[Abstract/Free Full Text] - Cantor WJ, Harrison DA, Moussadji JS, et al. Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol (1999) 84:677–681.[CrossRef][Web of Science][Medline]
- Connolly HM, Warnes CA. Pregnancy and contraception. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 135–144.
- Uebing A, Steer PJ, Yentis SM, Gatzoulis MA. Pregnancy and congenital heart disease. Br Med J (2006) 332:401–406.
[Free Full Text] - Reid GJ, Webb GD, Barzel M, McCrindle BW, Irvine MJ, Siu SC. Estimates of life expectancy by adolescents and young adults with congenital heart disease. J Am Coll Cardiol (2003) 48:349–355.[CrossRef]
- Fisher WA, Black A. Contraception in Canada: a review of method choices, characteristics, adherence and approaches to counselling. CMAJ (2007) 176:953–961.
[Abstract/Free Full Text] - Khairy P, Ouyang DW, Fernandes SM, Lee-Parritz A, Economy KE, Landzberg MJ. Pregnancy outcomes in women with congenital heart disease. Circulation (2006) 113:517–524.
[Abstract/Free Full Text] - Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol (2007) 49:2303–2311.
[Abstract/Free Full Text] - Nora JJ. From generational studies to a multilevel genetic–environmental interaction. J Am Coll Cardiol (1995) 23:1468–1471.[Web of Science]
- Siu SC, Colman JM, Sorensen S, et al. Adverse neonatal and cardiac outcomes are more common in pregnant women with cardiac disease. Circulation (2002) 105:2179–2184.
[Abstract/Free Full Text] - Kovalchin JP, Silverman NH. The impact of fetal echocardiography. Pediatr Cardiol (2004) 25:299–306.[Web of Science][Medline]
- Small M, Copel JA. Indications for fetal echocardiography. Pediatr Cardiol (2004) 25:210–222.[Web of Science][Medline]
- Eagle KA, Berger PB, Calkins H, et al. ACC/AHA guideline update for perioperative cardiovascular evaluation for noncardiac surgery—executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee to Update the 1996 Guidelines on Perioperative Cardiovascular Evaluation for Noncardiac Surgery). J Am Coll Cardiol (2002) 39:542–553.
[Free Full Text] - Colman JM. Noncardiac Surgery in Adult Congenital Heart Disease. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 99–104.
- Lovell AT. Anaesthetic implications of grown-up congenital heart disease. Br J Anaesth (2004) 93:129–339.
[Free Full Text] - Walsh EP, Cecchin F. Arrhythmias in adult patients with congenital heart disease. Circulation (2007) 115:534–545.
[Free Full Text] - Walsh EP. Interventional electrophysiology in patients with congenital heart disease. Circulation (2007) 115:3224–3234.
[Free Full Text] - Wang A, Athan E, Pappas PA, et al. Contemporary clinical profile and outcome of prosthetic valve endocarditis. JAMA (2007) 297:1354–1361.
[Abstract/Free Full Text] - Wallace SM, Walton BI, Kharbanda RK, Hardy R, Wilson AP, Swanton RH. Mortality from infective endocarditis: clinical predictors of outcome. Heart (2002) 88:53–60.
[Abstract/Free Full Text] - Moreillon P, Que YA. Infective endocarditis. Lancet (2004) 363:139–149.[CrossRef][Web of Science][Medline]
- Wilson W, Taubert KA, Gewitz M, et al. Guidelines from the American Heart Association. A Guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. (2007).
- Dajani AS, Taubert KA, Wilson W, et al. Prevention of bacterial endocarditis: recommendations by the American Heart Association. Circulation (1997) 96:358–366.
[Abstract/Free Full Text] - Gould FK, Elliott TS, Foweraker J, et al. Working Party of the British Society for Antimicrobial C. Guidelines for the prevention of endocarditis: report of the Working Party of the British Society for Antimicrobial Chemotherapy. J Antimicrob Chemother (2006) 57:1035–1042.
[Abstract/Free Full Text] - Li W. Infective endocarditis. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 125–133.
- Ramsdale DR, Turner-Stokes L. Prophylaxis and treatment of infective endocarditis in adults: a concise guide. Clin Med (2004) 4:545–550.[Web of Science][Medline]
- Ramsdale DR, ET, Wright P, Roberts GJ, Wallace P, Fabri B. Guidelines for the prophylaxis and treatment of infective endocarditis in adults. (2004) 1–106.
- Di Filippo S, Delahaye F, Semiond B, et al. Current patterns of infective endocarditis in congenital heart disease. Heart (2006) 92:1490–1495.
[Abstract/Free Full Text] - Daebritz SH. Update in adult congenital cardiac surgery. Pediatr Cardiol (2007) 28:96–104.[CrossRef][Web of Science][Medline]
- Shore DF. Late repair and reoperations in adults with congenital heart disease. In: Diagnosis and Management of Adult Congenital Heart Disease—Gatzoulis MA, Webb GD, Daubeney PEF, eds. (2003) Edinburgh: Churchill Livingstone. 73–78.
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