151 Full

Adult congenital heart disease: a 2008 overview
dard1,2, Darryl F. Shore1,2, and Michael A. Gatzoulis1,2* Elisabeth Be

Adult Congenital Heart Center and Center for Pulmonary Arterial Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK, and 2National Heart and Lung Institute, Imperial College, London, UK
1
Introduction: 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 benet of physicians who are not specialized in this eld. Areas timely for developing research: Research efforts and education strategies are greatly needed in order to optimize the care of patients with ACHD.
Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013
Keywords: Congenital/heart/adult
Introduction
Accepted: January 21, 2008 *Correspondence to: Michael A. Gatzoulis, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. E-mail: m.gatzoulis@rbht.nhs.uk
Congenital heart disease (CHD) is defined as a cardiovascular malformation that is present from birth. Approximately 4 to 10 liveborn 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,
& The Author 2008. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oxfordjournals.org
British Medical Bulletin 2008; 85: 151180 DOI:10.1093/bmb/ldn005
dard et al. E. Be
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.
Overview of common CHD lesions

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.
Contraception and pregnancy

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
152
British Medical Bulletin 2008;85
Table 1: Overview common congenital heart lesions

Lesion ASD8 14 Anatomical ndings Secundum: Within the oval fossa 75% of ASDs Primum: Part of an AVSD with a common atrioventricular junction and trileaet left AVV 10 15% of ASDs Sinus venosus: Deciency of infolding of the atrial wall usually in the region of the superior vena cava 5 10% of ASDs Coronary sinus: Deciency of the wall between the coronary sinus and the left atrium VSD15 19 Perimembranous: Part of its border is formed by brous continuity between the tricuspid and aortic valves (membranous septum) 80% of VSDs Muscular: The defect is completely surrounded by a muscular rim 5 20% of VSDs Double committed subarterial: Part of its border is formed by brous continuity between the aortic and pulmonary valves 6% of VSDs Clinical presentation Dyspnea on exertion Palpitations (arrhythmias) Cardiomegaly on chest radiography Cerebrovascular events from paradoxical embolism (uncommon) Right heart failure Cyanosis Examination: Right ventricular lift Wide and xed splitting of the second heart sound Pulmonary ejection systolic murmur at the ULSE Increase in pulmonary component of the second sound if elevated PAP Dyspnea on exertion Palpitations (arrhythmias) Heart failure Endocarditis Eisenmenger syndrome if VSD not restrictive Examination: Holosystolic murmur (smaller defects are louder) Widely split second sound, varies normally with respiration Diastolic rumble of increased mitral ow if signicant shunt Laterally displaced apical impulse if signicant shunt Increase in pulmonary component of the second sound if elevated PAP Management Indications for closure: 1. RA and RV enlargement in the absence of advanced pulmonary hypertension with one or more of the following: Left to right shunt (Qp:Qs) . 1:5:1 ASD . 10 mm 2. Paradoxical embolism Options: 1. Surgery - mortality , 1% - or 2. Device closure of secundum defects only (if stretched diameter , 38 mm) complication , 1% Outcome Complications ASD closure before age 25 years: normal long-term survival If . 25 years or elevated PAP before closure: lower long term survival. However, benets are expected even if . 40 years, but risk of atrial arrhythmia remains high Complications: Arrhythmias (atrial tachyarrhythmia, bradyarrhythmia) Right heart failure Impaired functional capacity Pulmonary arterial hypertension Embolic cerebrovascular events Unoperated patients: Small restrictive VSD with normal pulmonary vascular resistance: Excellent prognosis17 25-year survival rate decreases with increasing size of the VSD16
Small (Qp/Qs less than 1.4, no LV volume overload): Observe
Moderate or large (Qp/Qs . 1.5) Closure is recommended in the absence of advanced pulmonary hypertension if 1 of the following are present: 1. Evidence of LV volume overload (LV Operated patients: Without PAH: normal life expectancy enlargement, + reduced LV EF) Late repair with degree of PAH: 2. Symptoms decreased survival Other indications for closure (regardless Complications: of the size): Endocarditis 1. Endocarditis (if recurrent) Arrhythmias (atrial or ventricular) 2. Aortic regurgitation in cases of Aortic regurgitation subarterial VSD PAH and Eisenmenger syndrome Heart failure Transcatheter closure: In the investigational stage. Consider for selected cases. Management: Surgical correction including repair of the AVV within the rst few months of life for complete AVSD to avoid pulmonary vascular disease 5 10% will require surgical revision for left AVV repair or replacement during Unoperated patients: Complete AVSD: 4% survival beyond 5 years old Ostium primum: 50% mortality before 20 years of age Operated patients: Complete AVSD: 10 year survival of 83%
AVSD20 24
Common atrioventricular junction guarded by a common AVV that consists of 5 leaets. Partial or Primum ASD: The common valve is divided in 2 orices
Relate to the morphology of the defect and may include: Unrepaired: Cyanosis Eisenmenger syndrome if large VSD
153
Continued
Table 1:. Continued

Lesion Anatomical ndings Complete: The common valve guard a common orice About 70 80% of patients with complete AVSD have Down syndrome (surgical series) Aorta displaced anteriorly and to the right in an unwedged position (goose-neck deformity) Clinical presentation Congestive heart failure Increased pulmonary component of the second heart sound Ejection systolic murmur (variable) Apical mid-diastolic murmur (in large left to right shunt) Pansystolic murmur (with AVV regurgitation) Adult with repaired AVSD may present with clinical features of: Complete heart block AVV regurgitation LVOT obstruction Pulmonary hypertension TOF
25 38
154
dard et al. E. Be
Management the follow-up Benets also reported from surgical repair of partial AVSDs in later life ( . 40 years old)
Outcome Complications Ostium primum: 40 year survival of 76% Long term complications Left AVV regurgitation Left ventricular outow tract obstruction Late onset complete heart block Pulmonary vascular disease Atrial or ventricular dysrhythmias Left atrioventricular valve stenosis Right atrioventricular valve stenosis/ regurgitation Residual ventricular septal defect Aortic incompetence Unoperated patients: 25% mortality in the rst year of life, 70% before 10 years Operated patients: 85% survival rate at 36 years (older age at repair decreases late survival) Long term complications: Residual pulmonary regurgitation Residual RVOT obstruction RV dysfunction/RVOT aneurysm LV dysfunction AR + aortic root dilatation Endocarditis Atrial tachyarrhythmia Sustained VT/Sudden death Heart block
Morphologic marker: Anterocephalad deviation of the outlet septum with hypertrophy of the septoparietal trabeculations creating obstruction of the RVOT 4 components: 1. Ventricular septal defect 2. Aorta overriding the ventricular septum 3. Obstruction of the RVOT (subvalvar, valvar, supravalvar) 4. Right ventricular hypertrophy Associated with: Right aortic arch (25%), ASD (10%), coronary arterial anomalies (10%) Can be associated with a genetic anomaly (example: 15,9% have chromosome 22q11 microdeletion, DiGeorge syndrome)
Early presentation (Depends on the degree of RVOTO): Cyanosis and squatting Dyspnoea on exertion Palpitations (arrhythmias) Heart failure Endocarditis Adult with previous repair-late symptoms: Exertional dyspnoea Palpitations (atrial or ventricular arrhythmias) Syncope
Management: Elective primary repair before 18 months of age Symptomatic neonates with cyanosis: Palliative shunt to augment pulmonary blood ow before reparative surgery Adults who present without previous palliative surgery: Surgical repair recommended unless contraindications Post repair in adulthood - Indications for reintervention: PR with RV enlargement/dysfunction New onset TR Deterioration in exercise capacity PS with RV/LV pressures . 0.67 (percutaneous PV implantation in selected patients) Arrhythmias (atrial or ventricular) Residual VSD with Qp:Qs . 1.5:1 Residual patent arterial shunt with LV volume overload Signicant AR with LV enlargement/ dysfunction Aortic root enlargement ( . 55 mm) Percutaneous PVR: May be considered in selected patients
TGA39 44
Complete TGA (D-TGA): The aorta Early presentation: arises from the morphological RV and Blue baby often rst day of life the PA arises from the morphological Single and loud second heart sound LV (ventriculoarterial discordance) Adult-unoperated: Associated with other cardiovascular Almost all have had prior repair, but lesions in 50%: VSD (40 45%), LVOT some with a large VSD survive with obstruction (25%), coarctation of the Eisenmenger physiology aorta (5%) Adult-operated: see complications
Before denitive surgery (to increase systemic oxygenation): Prostaglandin E1 to maintain PDA Balloon atrial septostomy
Unoperated patients: 90% mortality in infancy
Atrial switch repair: 75 90% 25-year survival rate (less good Arterial switch: if VSD present and with Mustard Procedure of choice when the anatomy is procedure)40 appropriate, usually performed in the Long term complications (atrial switch): rst month of life Tachyarrhythmias (atrial reentry tachycardia) Atrial switch (Mustard or Senning): Bradyarrhythmias (sinus node Blood from the vena cava is directed to dysfunction with junctional escape the MV and LV and into the PA, and pulmonary venous blood is directed into rhythm) the morphological RV and into the aorta. Sudden death (7 15%) Usually performed between 1 month and Systemic ventricular dysfunction Tricuspid regurgitation 1 year of age Bafe obstruction or leak Pulmonary arterial hypertension Rastelli operation: For TGA with PS VSD. Patch placed to direct blood from LV through the VSD to Arterial switch operation: 88% 15-year survival rate (less good if the aorta. Pulmonary valve is oversewn, associated lesions are present) and a conduit is inserted between RV and PA Long term complications(arterial switch): PA stenosis Treatment of complications in adults Dilatation of the neoaortic root + AR Atrial bafe obstruction: Percutaneous Coronary ostia stenosis balloon and stent or surgery Bradyarrhythmias: Pacemaker TR: TVR in selected patients Severe heart failure: Transplantation Large VSD: Early VSD closure Severe LAVV (tricuspid) regurgitation: TVR TR reduced systemic RV function: Consider double-switch procedure: combines an atrial switch with either an arterial switch or Rastelli repair (when PS VSD present) End-stage heart failure: Cardiac transplantation Unoperated patients: 25% have developed CHF by 45 years of age (isolated ccTGA), vs 67% if associated lesions Long term complications: RV dysfunction CHF TR Heart block Arrhythmias (atrial, ventricular) Sudden cardiac death Conventional repair: 54 83% 10-year survival rate Double-switch repair: Long term survival rates not available
ccTGA45 50
Congenitally corrected TGA (L-TGA): The RA connects to the morphological LV, which gives rise to the PA, and the LA connects to the morphological RV, which gives rise to the aorta (double discordance) Associated with: Dextrocardia (20%), VSD (70%), pulmonary stenosis (40%), abnormalities of the systemic AVV Ebstein like (90%), abnormalities of the conduction system, i.e. complete heart block
Clinical presentation: Can be asymptomatic well into adulthood if no associated lesions Congestive heart failure Palpitations Complete heart block Cyanosis (if LVOTO VSD)
155
Continued
Table 1:. Continued 156

Lesion Single ventricle and the Fontan circulation51 55 Anatomical ndings Single ventricle: Hearts receiving systemic and pulmonary venous blood in one functional ventricle that pumps blood into the PA and aorta. Fontan circulation: Systemic venous return is connected to the PAs without the interposition of a subpulmonary ventricle. Performed in patients having a functionally single ventricle. Classic Fontan repair (no longer used): RA to PA direct anastomosis TCPC (modied Fontan): Combines bidirectional Glenn (see in Management) with connection of IVC to PA (by a lateral tunnel or an extracardiac conduit), possibly with a fenestration created between the tunnel conduit and the pulmonary atrium Congenital AS/ Bicuspid aortic valve10, 35, 56 60 Subvalvar AS: Range from discrete brous membrane (90%) to tunnel-like bromuscular band. Valvar AS: Abnormal development of the valvar commissures with a bicuspid AV in 95%. Bicuspid AV 1-2% of general population. Associated with abnormal aortic root tissue structure. Supravalvar AS: Rare. Aortic narrowing at the level of sinotubular junction. Associated with William syndrome. Clinical presentation Early presentation: Depends on initial underlying lesion After Fontan procedure: 90% FC I or II at 5 years See complications Examination (after Fontan procedure): Elevated nonpulsatile JVP with prominent A wave Single second sound, often accentuated Fontan patients should usually not have a heart murmur. Management Surgery is always palliative Neonatal period: Palliative procedure to prepare PAs for the Fontan operation: Severe PS: Systemic to PA shunt Unrestricted pulmonary ow: PA band Balloon septostomy if required 4-12 months: Second- stage palliation: Bidirectional Glenn: end-to-side SVC to RPA anastomosis 1-5 years of age: Fontan (TCPC) Failing Fontan (decreased exercise tolerance, recurrent atrial tachycardia): Revision of the circuit to an extracardiac conduit in combination with maze procedure or consideration for heart transplantation Outcome Complications After Fontan operation: 81% survival at 10 years (ideal candidates) Late complications: Atrial reentry tachycardia Thromboembolic events RA dilatation hepatic dysfunction Protein losing enteropathy Ventricular outow obstruction Pulmonary arteriovenous malformations Obstruction of pulmonary veins Narrowing + leaks in Fontan pathway Myocardial dysfunction and failure Systemic venous collateralization Sinus node dysfunction Plastic bronchitis Cyanosis Symptoms may develop in childhood or adulthood: Exertional angina Syncope Dyspnea CHF Endocarditis Examination Slow-rising, diminished pulse Apex-to-carotid delay (severe) Sustained apical impulse Ejection click (if valve pliable) Diminished aortic component of S2, paradoxical splitting if severe S4, S3 if ventricular dysfunction Crescendo-decrescendo systolic murmur, late peaking if severe Childhood: Balloon or surgical valvotomy indicated if: 1. Symptoms are present 2. Severe AS (peak gradient . 80 mmHg or valve area , 0.5 cm2/m2) 3. Abnormal ECG response to exercise Adolescents and young adults: Balloon valvotomy may be considered for pliable valve with fusion of commissures : 1. Presence of symptoms or 2. Peak echo Doppler gradient . 70 80 mmHg or peak to peak catheter gradient . 60 mmHg or 3. Ischemic ECG changes at rest or on exercise with peak to peak catheter gradient . 50 mmHg or 4. Peak to peak catheter gradient . 50 mmHg in patients who wish to do competitive sports All other adults: Aortic valve replacement Unoperated patients: Symptomatic: 5-year survival rates of 15 50% Asymptomatic: good, average risk of sudden death: 0.4%/year Patients who underwent intervention: In childhood: 25-year survival rate 85% Post surgical valvotomy: 40% will require reoperation within 25 years In adult post AVR: survival . 80% at 3 years Complications Endocarditis Recurrent AS after surgical or balloon intervention/AR Aortic root dilatation or dissection Left ventricular dysfunction Sudden cardiac death If prosthesis has been used, embolic events, valve thrombosis (mechanical), prosthetic valve degeneration (biologic)
dard et al. E. Be
59
Coarctation of the aorta10, 56, 61 66
Localized stenosis (shelf-like infolding of the posterior aortic wall) almost always located at the junction of the distal aortic arch and the descending aorta, just below the origin of the LSCA. Diffuse form: tubular hypoplasia involving the aortic arch or the aorta distal to the LSCA. Associated with: Bicuspid aortic valve (70%), VSD, mitral valve abnormalities, intracranial aneurysms, Turner syndrome
Symptoms (Depend on the degree of stenosis and associated defects): Often asymptomatic Headache/nose-bleeds Dizziness/tinnitus Cold feet/ Exertional leg fatigue Abdominal angina Intracranial haemorrhage Examination Right arm systemic hypertension BP drop between upper-lower extremities Weak, delayed femoral pulses Palpable arteries over the scapulae, lateral chest wall, between the ribs Thrill in suprasternal notch or neck vessels Heaving apex, not displaced Loud aortic component of S2 Ejection click if bicuspid aortic valve + ejection systolic murmur Continuous vascular murmur between the scapulae or over the thorax Symptoms (depend on anatomic severity and associated defects): Right to left shunt with cyanosis (severe) Right heart failure with dyspnoea (severe) May be asymptomatic if mild Arrhythmia and sudden cardiac death Examination: Cyanosis + digital clubbing Large V wave is rare despite severe TR (the large RA is very compliant) Widely split S1 and S2 added sounds Holosystolic murmur of TR
Early surgical repair: Best age between 2 5 years Angioplasty + stent alternative treatment at some centers (higher incidence of aneurysm) Indications for intervention or reintervention (surgery or angioplasty + stent) in adults: 1. Symptoms peak to peak catheter gradient . 30 mmHg across coarctation 2. No symptoms, gradient . 30 mmHg systemic hypertension or LVH 3. Signicant AS/AR 4. Paraaortic aneurysm Angioplasty + stent: Treatment of choice for recoarctation
Unoperated patient: 60 90% mortality during the rst year of life Operated patients: 20-year survival rate: 84%66 Complications following surgical repair: Persistent high blood pressure Aneurysm of the aorta Recoarctation or residual stenosis Coronary artery disease AS /AR (if bicuspid aortic valve present) Mitral valve defects Endocarditis/ endarteritis Rupture of aortic or cerebral aneurysm
Ebstein anomaly of the tricuspid valve67 73
Abnormality of the tricuspid valve with: 1. Downward (apical) displacement of the septal and often the mural leaet 2. Adherence of the septal and mural leaets to the underlying myocardium 3. Dilation of the atrialized portion of the right ventricle 4. Redundant, malformed anterior leaet Abnormal tricuspid valve leads to severe tricuspid regurgitation Associated with: ASD or PFO ( . 1/3 of cases), RBBB, multiple accessory pathways, pulmonary stenosis or atresia, bicuspid aortic valve, VSD, ccTGA.
Tachyarrhythmias-accessory pathways: Severe cases detected in foetus or Catheter ablation. Success 80% (vs 95% if neonates: High mortality rates (1/3 die no Ebstein) and 25% risk of recurrence. before age 10) Indications for TV surgery in adults: 1. NYHA III despite medical treatment 2. Right heart enlargement/Reduced systolic function/Early right heart failure 3. Cyanosis 4. Paradoxical emboli 5. Recurrent supraventricular arrhythmias despite treatment (relative indication) Surgery may be combined with antiarrhythmic intervention High risk patients with severe TR inadequate functional RV: Glenn shunt or modied Fontan procedure has been proposed. Transplantation is an alternative. Actuarial survival for all live-born patients: 59% at 10 years. Complications Arrhythmias Heart failure Cyanosis Paradoxical embolus Endocarditis Sudden cardiac death
Pulmonary Stenosis10, 27,

74 77
59,
Dome-shaped PV: Narrow central orice but mobile valve (most common), usually with dilated main
Symptoms (depend on the severity of PS): Mild to moderate: asymptomatic
Indications for balloon valvuloplasty: 1. Symptoms and/or 2. RV pressure . 2/3 of systemic
Good prognosis, normal life expectancy after repair
157
Continued
Table 1:. Continued

Lesion Anatomical ndings pulmonary artery Dysplastic: Thickened, poorly mobile leaets Rarely: Secondary to rheumatic heart disease or carcinoid involvement Associated with: Noonan, Williams and Alagille syndromes Clinical presentation Exertional dyspnoea/Fatigue Exertional chest pain or syncope Palpitations Mild cyanosis (severe right to left shunt) Examination (if severe): Cyanosis clubbing Increased a wave RV lift, thrill along the left sternal edge Widely split S2, reduced or absent P2 Short S1-ejection click interval Long systolic ejection murmur, late peaking Symptoms (depends on the size of PDA): Small: Asymptomatic, often detected incidentally (murmur, echocardiogram) Moderate-large: Exercise intolerance, CHF, Atrial brillation, Eisenmenger (large) Examination (if signicant shunt): Bouncy pulses, wide pulse pressure, low diastolic pressure Prominent ventricular impulse Continuous murmur in the left infraclavicular area, radiates to the back Eisenmenger: Differential cyanosis (cyanosis clubbing of toes more than ngers), usually no murmurs Management pressure with RVH and 3. No signicant calcication or dysplasia and 4. 2 PR Dysplastic valve: Surgical valvotomy or PV replacement Presence of signicant PR: PV replacement (bioprosthesis or homograft) Outcome Complications Complications (unoperated): RVH and RV heart failure Atrial brillation or utter Endocarditis (rare) After pulmonary valvotomy: PR + RV dilatation Residual or recurrent PS Supraventricular or ventricular arrhythmias Sudden cardiac death (rare)
158
dard et al. E. Be
Patent Ductus Arteriosus78 83
Vital structure in foetal life, normally closes after birth. When remains patent, leads to left to right shunting with increased pulmonary ow and left heart volume overload.
PDA closure recommended: 1. Signicant left-to-right shunt LV dilatation 2. To eliminate the risk of endarteritis 3. Not if silent tiny duct or irreversible PAH Device closure: Is the preferred method, and should be considered even in presence of severe pulmonary hypertension, if reversible Surgical closure: Reserved for: PDA too large, too distorted
Complication: Congestive heart failure Pulmonary vascular disease Endarteritis Aneurysm of the ductus arteriosus
Eisenmenger syndrome84 92
Pulmonary hypertension with reversed (right-to-left) or bidirectional shunting (intra- or extra-cardiac). Due to a large communication with consequent increased pulmonary blood ow, causing development of irreversible pulmonary vascular disease.
Symptoms: Exertional dyspnoea or fatigue Palpitations Oedema Syncope Examination: Depends on the underlying defect Cyanosis Clubbing
Goal: Avoid complications 1. Phlebotomy: Restricted to patients with moderate to severe hyperviscosity symptoms in the absence of dehydration and iron deciency 2. Avoid and treat anaemia 3. Iron supplements if iron deciency present 4. Avoid and treat dehydration
Actuarial survival: 75% at the age of 30 years, 70% at 40 years, 55% at 50 years92 Complications Hyperviscosity symptoms (headache, altered mentation, blurred vision, paresthesia, myalgias) Bleeding complications Iron deciency Thromboembolic events
Causes: VSD, PDA, ASD, AVSD, truncus arteriosus.
Loud P2 May have holosystolic murmur of TR
5. Anticoagulation if additional indications (e.g. atrial brillation/utter, mechanical valve, recurrent TE events) 6. Oxygen therapy may occasionally be benecial (controversial) 7. Vaccination: Flu shot annually Pneumovax once 8. Consider non-selective endothelin receptor antagonist or Sildenal if WHO functional class III 9. Consider transplantation for end-stage heart and lung disease.
Arrhythmias (supraventricular or ventricular) Sudden cardiac death Congestive heart failure Viral and bacterial infections (endocarditis, cerebral abscess, pneumonia) Hyperuricemia and gout Gallstones and cholecystitis Renal dysfunction Hypertrophic osteoarthropathy
AR: Aortic regurgitation /AS: Aortic stenosis/ ASD: Atrial septal defect / AVSD: Atrioventricular septal defect / AVV: atrioventricular valve / BP: Blood pressure / ccTGA: Congenitally corrected transposition of great arteries / CHF: Congestive heart failure / EF: Ejection Fraction/ FC: Functional class / IVC: Inferior vena cava / JVP: Jugular venous pressure / LA: Left atrium / LAVV: Left atrioventricular valve/ LSCA: Left subclavian artery / LV: Left ventricle / LVH: Left ventricular hypertrophy / LVOT: Left ventricular outow tract / MV: Mitral valve / PA: Pulmonary artery / PAH: Pulmonary arterial hypertension / PAP: Pulmonary arterial pressure / PDA: Patent Ductus Arteriosus / PFO: Patent Foramen Ovale / PR: Pulmonary regurgitation / PS: Pulmonary stenosis / PV: Pulmonary valve / PVR: Pulmonary valve replacement / RA: Right atrium / RBBB: Right bundle branch block / RV: Right ventricle / RVH: Right ventricular hypertrophy/ RVOT: Right ventricular outow tract / RVOTO: Right ventricular outow tract obstruction / TCPC: Total cavopulmonary connection / TGA: Transposition of great arteries / TOF: Tetralogy of Fallot / TR: Tricuspid regurgitation / TVR: Tricuspid valve replacement / ULSE: Upper left sternal edge / VSD: Ventricular septal defect / VT: Ventricular tachycardia
159
dard et al. E. Be
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.
These effects on the cardiovascular system explain why patients with CHD may experience cardiac decompensation during pregnancy. Another major issue is the activation of the coagulation system during pregnancy, which is associated with an increased risk of thromboembolic events six times greater than in other patients with CHD.
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
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
160
Table 2 Contraceptive methods for women with CHD.

Contraceptive Natural methods (abstinence, withdrawal, safe period) Barrier methods (condoms, diaphragm) Comments Very high failure rate Not recommended Signicant failure rate ( 15% with typical use) Not recommended, although condoms are recommended to prevent sexually transmitted disease Approximately 92% effectiveness with typical use Risk of venous thromboembolism increased 3 4 times because of oestrogen Should not be used in patients at risk of thromboembolism (cyanosis, impaired cardiac function, atrial arrhythmias, Fontan-type repair, prosthetic heart valves) Approximately 92% effectiveness with typical use but less if not taken at the same time every day No increased risk of venous thromboembolism Irregular vaginal bleeding relatively common Highly effective: 97% effectiveness with typical use (99.7% with optimal use) Depot injection lasts 3 months, and Implanon up to 3 years Good when compliance is a concern No increased risk of venous thromboembolism Highly effective: 99.9% effectiveness; lasts for 5 years Decrease menorrhagia and dysmenorrhea (80% amenorrhea) Very low risk of infection and ectopic pregnancy No increased risk of venous thromboembolism Risk of vagal reaction during insertion; should be inserted in operating room with anaesthesiologist available Approximately 99.5% effectiveness; Mirena coil is even safer Surgical risks associated with the procedure
Combined oral contraceptive
Progestin-only pill (Mini pill)
Depot injection of progestogen and subcutaneous implants with slow release of progesterone (Implanonw and Norplantw)
Hormonal intrauterine system (Mirenaw coil)
Sterilization (laparoscopic tubal occlusion)
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
161
dard et al. E. Be
Box 1 Conditions associated with higher maternal mortality during pregnancy and the peripartum.
Pulmonary arterial hypertension including the Eisenmenger syndrome* Marfan syndrome with aortic root . 40 mm* Severe left heart obstruction (aortic stenosis, mitral stenosis, hypertrophic obstructive cardiomyopathy)* Poor functional class before pregnancy (NYHA III IV)* Systemic ventricular dysfunction with ejection fraction , 40%
Other conditions with significant increased maternal risk of cardiovascular events

Cyanotic congenital heart disease Patients with prosthetic valves Impaired function of the subpulmonary ventricle Severe pulmonary regurgitation Preconception history of adverse cardiac events such as sustained arrhythmia, stroke, transient ischaemic attack and pulmonary oedema
*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).
Compared with the general population, there is an increased risk of intrauterine growth restriction, premature birth, intracranial haemorrhage, spontaneous abortion, respiratory distress syndrome and neonatal death.94,97,98 Maternal risk factors for such complications include100
women , 20 or . 35 years who had obstetric risk factors or multiple gestation,
162
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.
163
dard et al. E. Be
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.
Non-cardiac surgery
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.
1. Define the condition Primary lesion Previous palliative and corrective surgeries Residua and sequelae 2. Assess the surgical risk Predictors and risks of the proposed surgical procedure itself Specific ACHD risk factors Co-morbid conditions 3. Complete preoperative investigation as necessary 4. Develop a management plan (written and available for all caregivers) Explanation of anatomy and physiology Anaesthetic considerations (involvement of anaesthesiologist) Nature of postoperative monitoring (continuous ECG monitoring, pulse oximetry, invasive blood pressure recording, blood tests required) Specific perioperative management treatment to be administered (including thromboprophylaxis) see Table 3 5. Consider management in regional ACHD centre when appropriate
Modified from Colman.104

164
Table 3. Risk factors specic to ACHD population undergoing noncardiac surgery 104
Risk factor Consequences Perioperative management Avoid hypothermia, acidosis, additional hypoxia, hypercarpnea, alpha-adrenergic stimulation, drop in SVR.
Reduced ability to increase pulmonary Pulmonary vascular disease/ blood ow in response to # in systemic vascular resistance (SVR): Pulmonary Cardiac output fails to increase arterial Increase in right to left shunt with " in hypertension hypoxemia Cyanosis Increased risk of bleeding secondary to: Abnormal platelets and coagulation pathways Increased vascularity aorto-pulmonary collaterals Hyperviscosity due to secondary erythrocytosis, mostly if dehydration occurs Thrombocytopenia: usually artefact of elevated red blood count mass blood volume Effect of anaemia on O2-carrying capacity is worse in cyanotic patients
Isovolumic phlebotomy immediately preoperatively to # haematocrit to , 0.65 may improve haemostatic function* Iron repletion when patient is iron-decient Adequate iv uid to avoid dehydration when oral intakes are restricted Careful haemoglobin follow-up; normal haemoglobin may represent signicant decit in oxygen carrying capacity Avoid air into the venous system during intravenous access technique (use air lters in intravenous lines) Avoid " in PVR and drop in SVR (see Pulmonary arterial hypertension)
Residual shunt
Possibility of paradoxical embolism
Left to right: Generally well tolerated Right to left: # in SVR and " in pulmonary vascular resistance (PVR) can " in right to left shunt with " in hypoxemia Systemic Right Ventricle (RV) May have systemic RV dysfunction and congestive heart failure (CHF) Risk of decompensation " with " in SVR
If associated with CHF, improve signs and symptoms and optimize therapy before surgery Avoid signicant " SVR (uncontrolled pain, alpha-adrenergic stimulation) Avoid/correct " in PVR triggered by hypoxia, some anaesthetic agents, thromboemboli, atelectasis, pneumonia Avoid/correct # in preload caused by haemorrhage, venodilatation, inadequate volume replacement, avoid " in intrathoracic pressure by positive pressure ventilation Need close monitoring of blood pressure perioperative treatment of hypertension Blood pressure should usually be measured in the right arm Need preoperative assessment for possible coronary artery disease
Fontan circulation
Pulmonary blood ow can substantially # with: Small " in PVR Small # in preload
Coarctation of the aorta
May have signicant proximal systemic hypertension even after repair Left subclavian artery may have been used in the repair or may be stenosed ! low blood pressure estimation usually in the left arm Increased risk of premature coronary artery disease if late repair
*Otherwise phlebotomy is not indicated unless symptomatic hyperviscosity occurs
165
dard et al. E. Be
The first step in the preoperative evaluation of patients with CHD is to determine the patients 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 patients 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 patients 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 patients 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 noncardiac 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
166
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.
Arrhythmias in ACHD
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.
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
167
dard et al. E. Be
Table 4 Most frequent arrhythmias in CHD lesions.

CHD/arrhythmia ASD AVSD TOF Mustard/Senning cc-TGA Fontan AS/coarctation Ebstein IART x x x x x x x WPW Atrial brillation x x x x x x x x VT Sinus node dysfunction x x x x x x x x x AV block
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 min21, and 1:1 A:V conduction can lead to severe symptoms such as syncope and even cardiac arrest. Therapeutic options include the following106:
1. Acute episode can be terminated with electrical cardioversion, antiarrhythmic drugs or overdrive pacing. 2. Long-term antiarrhythmic drug therapy: often ineffective. 3. 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. 4. Pacemaker implantation: used for patients with tachy brady syndrome; decrease in IART frequency by correcting bradycardia and by atrial antitachycardia pacing capability. 5. 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 Ebsteins 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 Ebsteins patients.106
Atrial fibrillation
CHD conditions associated with left atrial pressure and/or volume overload predispose to atrial fibrillation. Mitral valve abnormalities (or
168
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, Eisenmengers syndrome and severe Ebsteins 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.
Endocarditis prophylaxis
Endocarditis is a life-threatening disease with an in-hospital mortality rate of approximately 16 20% despite improvement in medical
169
dard et al. E. Be
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 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
Table 5. Relative risk of endocarditis associated with underlying CHD

No endocarditis prophylaxis Low or no risk Surgically-repaired atrial septal defect, Surgically repaired ventricular septal defect, Surgically repaired patent ductus arteriosus, Post Fontan or Mustard procedure without residual defect/murmur Isolated secundum atrial septal defect Mitral valve prolapse without regurgitation Pulmonary stenosis Endocarditis prophylaxis indicated Moderate risk Acquired valvular heart disease eg: rheumatic heart disease Aortic stenosis and regurgitation Mitral regurgitation Bicuspid aortic valve Primum atrial septal defect Patent Ductus Arteriosus Aortic root replacement Coarctation of aorta Atrial septal aneurysm/patent foramen ovale Ventricular septal defect Hypertrophic obstructive cardiomyopathy Subaortic membrane High risk Prosthetic heart valves Previous infective endocarditis Complex cyanotic CHD TGA TOF Surgically constructed systemic pulmonary shunts or conduits Mitral valve prolapse with signicant mitral regurgitation or thickened leaets
Modied with permission from Ramsdale DR, Elliott TSJ, Wright P, Roberts GJ, Wallace P, Fabri B, et al. Guidelines for the prophylaxis and treatment of infective endocarditis in adults. http://www.bcs.com, 2004:1-106 116
170
Table 6. Procedures for which endocarditis prophylaxis is recommended in high and moderate at-risk patients
Dental Tooth extraction Restoration of decayed teeth Periodontal procedures Dental implants Root canal procedures Intraligamentary local anaesthetic injection Cleaning when bleeding is anticipated Genitourinary Prostatic surgery Cystoscopy Urethral dilation Urethral catheterization if infection present or traumatic Uterine dilation and curettage, therapeutic abortion, sterilization procedure, insertion or removal of intrauterine device Gastrointestinal Oesophageal procedures Sclerotherapy for varices Biliary tract surgery or endoscopic procedure Surgery on stomach, small or large bowel Respiratory Tonsillectomy or adenoidectomy Surgical procedure involving the respiratory mucosa Bronchoscopy with rigid scope Other Body piercing
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
Late repair and re-operation

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
171
dard et al. E. Be
Table 7. Prophylactic antibiotic regimens for dental, oral, respiratory tract or oesophageal procedures in adults
Clinical situation High risk and moderate risk patients including patients with prosthetic heart valves If allergic to penicillin Drug Amoxicillin Regimen 3G oral 1 hr preprocedure or 2G IV , 30 min preprocedure 600 mg oral 1 hr preprocedure or 300 mg IV , 30 min preprocedure then oral or IV clindamycin 150 mg 6 hours later 500 mg oral 1 hr preprocedure 1G IV over 2 hours, 1-2hrs preprocedure 1.5 mg/kg IV , 30 min preprocedure 2G IV , 30 min preprocedure 1G IV or orally 6hrs post 1.5 mg/kg IV , 30 min preprocedure 1G IV over 2 hrs, 1-2hrs preprocedure 1.5 mg/kg IV , 30 min preprocedure 300 mg IV , 30 min preprocedure then IV clindamycin 150 mg 6 hours later
Clindamycin
Patients with previous infective endocarditise If allergic to penicillin
or Azithromycin or Vancomycin gentamicin Amoxicillin gentamicin Vancomycin gentamicin or Clindamycin
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
Table 8. Prophylactic antibiotic regimens for genitourinary or gastrointestinal procedures in adults
Clinical situation High risk and moderate risk patients including patients with prosthetic heart valves If allergic to penicillin Drug Ampicillin or amoxicillin gentamicin Vancomycin gentamicin Regimen 2G IV , 30 min preprocedure and 1G IV or orally 6 hr post 1.5 mg/kg IV , 30 min preprocedure 1G IV over 2 hours, 1-2hrs preprocedure 1.5 mg/kg IV , 30 min preprocedure
172
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 insertion. regurgitation following TOF: pulmonary valve
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).
173
dard et al. E. Be
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.
Conclusion
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.
Funding
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.
References
1 Pierpont ME, Basson CT, Benson DW et al. (2007) 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, 115, 30153038. 2 Hoffman JI, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol, 39, 18901900.
174
3 Gatzoulis MA (2006) Adult congenital heart disease: education, education, education. Nat Clin Pract Cardiovasc Med, 3, 23. 4 Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L (2007) Congenital heart disease in the general population: changing prevalence and age distribution. Circulation, 115, 163172. 5 Nieminen HP, Jokinen EV, Sairanen HI (2001) Late results of pediatric cardiac surgery in Finland: a population-based study with 96% follow-up. Circulation, 104, 570 575. 6 Mackie AS, Pilote L, Ionescu-Ittu R, Rahme E, Marelli AJ (2007) Health care resource utilization in adults with congenital heart disease. Am J Cardiol, 99, 839 843. 7 Gurvitz MZ, Inkelas M, Lee M et al. (2007) Changes in hospitalization patterns among patients with congenital heart disease during the transition from adolescence to adulthood. J Am Coll Cardiol, 49, 875 882. 8 Gatzoulis MA, Freeman MA, Siu SC, Webb GD, Harris L (1999) Atrial arrhythmia after surgical closure of atrial septal defects in adults. N Engl J Med, 340, 839 846. 9 Webb G, Gatzoulis MA (2006) Atrial septal defects in the adult: recent progress and overview. Circulation, 114, 16451653. 10 Inglessis I, Landzberg MJ (2007) Interventional catheterization in adult congenital heart disease. Circulation, 115, 16221633. 11 Murphy JG, Gersh BJ, McGoon MD et al. (1990) Long-term outcome after surgical repair of isolated atrial septal defect: follow-up at 27 to 32 years. N Engl J Med, 323, 16451650. 12 Attie F, Rosas M, Granados N, Zabal C, Buendia A, Calderon J (2001) Surgical treatment for secundum atrial septal defects in patients . 40 years old: a randomized clinical trial. J Am Coll Cardiol, 38, 2035 2042. 13 Lindsey JB, Hillis LD (2007) Clinical update: atrial septal defect in adults. Lancet, 369, 12441246. 14 Konstantinides S, Geibel A, Olschewski M et al. (1995) A comparison of surgical and medical therapy for atrial septal defect in adults. N Engl J Med, 333, 469473. 15 Thanopoulos BV, Rigby ML, Karanasios E et al. (2007) Transcatheter closure of perimembranous ventricular septal defects in infants and children using the Amplatzer perimembranous ventricular septal defect occluder. Am J Cardiol, 99, 984 989. 16 Kidd L, Driscoll DJ, Gersony WM et al. (1993) Second natural history study of congenital heart defects: results of treatment of patients with ventricular septal defects. Circulation, 87, I38 I51. 17 Gabriel HM, Heger M, Innerhofer P et al. (2002) Long-term outcome of patients with ventricular septal defect considered not to require surgical closure during childhood. J Am Coll Cardiol, 39, 1066 1071. 18 Minette MS, Sahn DJ (2006) Ventricular septal defects. Circulation, 114, 21902197. 19 Fu YC, Bass J, Amin Z et al. (2006) 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, 47, 319325. 20 El-Najdawi EK, Driscoll DJ, Puga FJ et al. (2000) Operation for partial atrioventricular septal defect: a forty-year review. J Thorac Cardiovasc Surg, 119, 880 889; discussion 889 890. 21 Craig B (2006) Atrioventricular septal defect: from fetus to adult. Heart, 92, 1879 1885. 22 Shinebourne EA, HS (2003) Atrioventricular septal defect: complete and partial (ostium primum atrial septal defect). In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 179 187. 23 Najm HK, Coles JG, Endo M et al. (1997) Complete atrioventricular septal defects: results of repair, risk factors, and freedom from reoperation. Circulation, 96, pII-311 II-315. 24 Bergin ML, Warnes CA, Tajik AJ, Danielson GK (1995) Partial atrioventricular canal defect: long-term follow-up after initial repair in patients . or 40 years old. J Am Coll Cardiol, 25, 1189 1194. 25 Goldmuntz E, Clark BJ, Mitchell LE et al. (1998) Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol, 32, 492 498.
175
dard et al. E. Be
26 Gatzoulis MA, Balaji S, Webber SA et al. (2000) Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet, 356, 975 981. 27 Bashore TM (2007) Adult congenital heart disease: right ventricular outflow tract lesions. Circulation, 115, 1933 1947. 28 Therrien J, Siu SC, Harris L et al. (2001) Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot. Circulation, 103, 24892494. 29 Gatzoulis MA, Till JA, Somerville J, Redington AN (1995) Mechanoelectrical interaction in tetralogy of Fallot: QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation, 92, 231 237. 30 Murphy JG, Gersh BJ, Mair DD et al. (1993) Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med, 329, 593599. 31 Gatzoulis MA (2003) Tetralogy of Fallot. In: Gatzoulis MA, Webb GD, Daubeney PEF(eds) Diagnosis and Management of Adult Congenital Heart Disease. Edinburgh: Churchill Livingstone, 315326. 32 Goldmuntz E, Geiger E, Benson DW (2001) NKX2.5 mutations in patients with tetralogy of Fallot. Circulation, 104, 25652568. 33 Ghai A, Silversides C, Harris L, Webb GD, Siu SC, Therrien J (2002) Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of Fallot. J Am Coll Cardiol, 40, 1675 1680. 34 Roos-Hesselink J, Perlroth MG, McGhie J, Spitaels S (1995) Atrial arrhythmias in adults after repair of tetralogy of Fallot: correlations with clinical, exercise, and echocardiographic findings. Circulation, 91, 22142219. 35 Niwa K, Siu SC, Webb GD, Gatzoulis MA (2002) Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot. Circulation, 106, 1374 1378. 36 Nollert G, Fischlein T, Bouterwek S, Bohmer C, Klinner W, Reichart B (1997) 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, 30, 13741383. 37 Coats L, Khambadkone S, Derrick G et al. (2006) Physiological and clinical consequences of relief of right ventricular outflow tract obstruction late after repair of congenital heart defects. Circulation, 113, 20372044. 38 Khambadkone S, Coats L, Taylor A et al. (2005) Percutaneous pulmonary valve implantation in humans: results in 59 consecutive patients. Circulation, 112, 11891197. 39 Gatzoulis MA, Walters J, McLaughlin PR, Merchant N, Webb GD, Liu P (2000) Late arrhythmia in adults with the mustard procedure for transposition of great arteries: a surrogate marker for right ventricular dysfunction? Heart, 84, 409415. 40 Lange R, Horer J, Kostolny M et al. (2006) 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, 114, 1905 1913. 41 Hornung T (2003) Transposition of the great arteries. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 349362. 42 Gelatt M, Hamilton RM, McCrindle BW et al. (1997) Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience. J Am Coll Cardiol, 29, 194 201. 43 Kammeraad JA, van Deurzen CH, Sreeram N et al. (2004) Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries. J Am Coll Cardiol, 44, 1095 1102. 44 Legendre A, Losay J, Touchot-Kone A et al. (2003) Coronary events after arterial switch operation for transposition of the great arteries. Circulation, 108 (Suppl 1), pII186II190. 45 Warnes CA (2006) Transposition of the great arteries. Circulation, 114, 26992709. 46 Graham TP (2003) Congenitally Corrected Transposition. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 379387. 47 Graham TP Jr, Bernard YD, Mellen BG et al. (2000) Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol, 36, 255 261.
176
48 Brickner ME, Hillis LD, Lange RA (2000) Congenital heart disease in adults: second of two parts. N Engl J Med, 342, 334 342. 49 Prieto LR, Hordof AJ, Secic M, Rosenbaum MS, Gersony WM (1998) Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries. Circulation, 98, 9971005. 50 Langley SM, Winlaw DS, Stumper O et al. (2003) 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, 125, 12291241. 51 Khairy P, Poirier N, Mercier LA (2007) Univentricular heart. Circulation, 115, 800 812. 52 Veldtman GR, Nishimoto A, Siu S et al. (2001) The Fontan procedure in adults. Heart, 86, 330 335. 53 Gatzoulis MA, Munk MD, Williams WG, Webb GD (2000) Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology. Heart, 83, 51 57. 54 Mavroudis C, Deal BJ, Backer CL (2002) The beneficial effects of total cavopulmonary conversion and arrhythmia surgery for the failed Fontan. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 5, 12 24. 55 Freedom RM, Li J, Yoo S-J (2003) Late complications following the Fontan operation. Diagnosis and Management of Adult Congenital Heart Disease, 85 91. 56 Aboulhosn J, Child JS (2006) Left ventricular outflow obstruction: subaortic stenosis, bicuspid aortic valve, supravalvar aortic stenosis, and coarctation of the aorta. Circulation, 114, 24122422. 57 Fedak PW, Verma S, David TE, Leask RL, Weisel RD, Butany J (2002) Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation, 106, 900904. 58 Pellikka PA, Sarano ME, Nishimura RA et al. (2005) Outcome of 622 adults with asymptomatic, hemodynamically significant aortic stenosis during prolonged follow-up. Circulation, 111, 32903295. 59 Bonow RO, Carabello BA, Kanu C et al. (2006) 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, 114, e84 e231. 60 Carabello BA (2002) Evaluation and management of patients with aortic stenosis. Circulation, 105, 1746 1750. 61 Kaemmerer H (2003) Aortic coarctation and interrupted aortic arch. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 253 264. 62 Campbell M (1970) Natural history of coarctation of the aorta. Br Heart J, 32, 633 640. 63 Cowley CG, Orsmond GS, Feola P, McQuillan L, Shaddy RE (2005) Long-term, randomized comparison of balloon angioplasty and surgery for native coarctation of the aorta in childhood. Circulation, 111, 3453 3456. 64 de Divitiis M, Pilla C, Kattenhorn M et al. (2003) Ambulatory blood pressure, left ventricular mass, and conduit artery function late after successful repair of coarctation of the aorta. J Am Coll Cardiol, 41, 22592265. 65 McCrindle BW, Jones TK, Morrow WR et al. (1996) 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, 28, 18101817. 66 Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC (1989) Coarctation of the aorta: long-term follow-up and prediction of outcome after surgical correction. Circulation, 80, 840 845. 67 Sondergaard L, Cullen S (2003) Ebstein anomaly. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 283287.
177
dard et al. E. Be
68 Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK (2007) Ebsteins anomaly. Circulation, 115, 277 285. 69 Celermajer DS, Bull C, Till JA et al. (1994) Ebsteins anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol, 23: 170 176. 70 MacLellan-Tobert SG, Driscoll DJ, Mottram CD, Mahoney DW, Wollan PC, Danielson GK (1997) Exercise tolerance in patients with Ebsteins anomaly. J Am Coll Cardiol, 29, 16151622. 71 Cappato R, Schluter M, Weiss C et al. (1996) Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebsteins anomaly. Circulation, 94, 376 383. 72 Quinonez LG, Dearani JA, Puga FJ et al. (2007) Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. J Thorac Cardiovasc Surg, 133, 1303 1310. 73 Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC Jr (1992) Operative treatment of Ebsteins anomaly. J Thorac Cardiovasc Surg, 104, 1195 1202. 74 Dore A (2003) Pulmonary stenosis. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 299303. 75 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. 76 Kanter KR, Budde JM, Parks WJ et al. (2002) One hundred pulmonary valve replacements in children after relief of right ventricular outflow tract obstruction. Ann Thorac Surg, 73, 18011806; discussion 1806 1807. 77 Jarrar M, Betbout F, Farhat MB et al. (1999) Long-term invasive and noninvasive results of percutaneous balloon pulmonary valvuloplasty in children, adolescents, and adults. Am Heart J, 138, 950 954. 78 Schneider DJ, Moore JW (2006) Patent ductus arteriosus. Circulation, 114, 1873 1882. 79 Brili SV, Toutouzas P (2003) Patent duct and aortopulmonary window. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 247 252. 80 Cambier PA, Kirby WC, Wortham DC, Moore JW (1992) Percutaneous closure of the small (less than 2.5 mm) patent ductus arteriosus using coil embolization. Am J Cardiol, 69, 815 816. 81 Yan C, Zhao S, Jiang S et al. (2007) Transcatheter closure of patent ductus arteriosus with severe pulmonary arterial hypertension in adults. Heart, 93, 514 518. 82 Fisher RG, Moodie DS, Sterba R, Gill CC (1986) Patent ductus arteriosus in adultslongterm follow-up: nonsurgical versus surgical treatment. J Am Coll Cardiol, 8, 280284. 83 Pass RH, Hijazi Z, Hsu DT, Lewis V, Hellenbrand WE (2004) Multicenter USA Amplatzer patent ductus arteriosus occlusion device trial: initial and one-year results. J Am Coll Cardiol, 44, 513 519. 84 Singh TP, Rohit M, Grover A, Malhotra S, Vijayvergiya R (2006) A randomized, placebocontrolled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am Heart J, 151, 851855. 85 Diller GP, Gatzoulis MA (2007) Pulmonary vascular disease in adults with congenital heart disease. Circulation, 115, 10391050. 86 Levy M, Maurey C, Celermajer DS et al. (2007) Impaired apoptosis of pulmonary endothelial cells is associated with intimal proliferation and irreversibility of pulmonary hypertension in congenital heart disease. J Am Coll Cardiol, 49, 803810. 87 DAlto M, Vizza CD, Romeo E et al. (2007) 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, 93, 621 625. 88 Galie N, Beghetti M, Gatzoulis MA et al. (2006) Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation, 114, 48 54. 89 Broberg CS, Bax BE, Okonko DO et al. (2006) Blood viscosity and its relationship to iron deficiency, symptoms, and exercise capacity in adults with cyanotic congenital heart disease. J Am Coll Cardiol, 48, 356365.
178
90 Benza RL, Rayburn BK, Tallaj JA et al. (2006) Efficacy of bosentan in a small cohort of adult patients with pulmonary arterial hypertension related to congenital heart disease. Chest, 129, 10091015. 91 Michelakis ED, Tymchak W, Noga M et al. (2003) Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension. Circulation, 108, 20662069. 92 Cantor WJ, Harrison DA, Moussadji JS et al. (1999) Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol, 84, 677 681. 93 Connolly HM, Warnes CA (2003) Pregnancy and contraception. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 135 144. 94 Uebing A, Steer PJ, Yentis SM, Gatzoulis MA (2006) Pregnancy and congenital heart disease. Br Med J, 332, 401406. 95 Reid GJ, Webb GD, Barzel M, McCrindle BW, Irvine MJ, Siu SC (2003) Estimates of life expectancy by adolescents and young adults with congenital heart disease. J Am Coll Cardiol, 48, 349 355. 96 Fisher WA, Black A (2007) Contraception in Canada: a review of method choices, characteristics, adherence and approaches to counselling. CMAJ, 176, 953 961. 97 Khairy P, Ouyang DW, Fernandes SM, Lee-Parritz A, Economy KE, Landzberg MJ (2006) Pregnancy outcomes in women with congenital heart disease. Circulation, 113, 517524. 98 Drenthen W, Pieper PG, Roos-Hesselink JW et al. (2007) Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol, 49, 23032311. 99 Nora JJ (1995) From generational studies to a multilevel geneticenvironmental interaction. J Am Coll Cardiol, 23, 14681471. 100 Siu SC, Colman JM, Sorensen S et al. (2002) Adverse neonatal and cardiac outcomes are more common in pregnant women with cardiac disease. Circulation, 105, 21792184. 101 Kovalchin JP, Silverman NH (2004) The impact of fetal echocardiography. Pediatr Cardiol, 25, 299306. 102 Small M, Copel JA (2004) Indications for fetal echocardiography. Pediatr Cardiol, 25, 210222. 103 Eagle KA, Berger PB, Calkins H et al. (2002) ACC/AHA guideline update for perioperative cardiovascular evaluation for noncardiac surgeryexecutive 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, 39, 542 553. 104 Colman JM (2003) Noncardiac Surgery in Adult Congenital Heart Disease. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 99 104. 105 Lovell AT (2004) Anaesthetic implications of grown-up congenital heart disease. Br J Anaesth, 93, 129339. 106 Walsh EP, Cecchin F (2007) Arrhythmias in adult patients with congenital heart disease. Circulation, 115, 534545. 107 Walsh EP (2007) Interventional electrophysiology in patients with congenital heart disease. Circulation, 115, 3224 3234. 108 Wang A, Athan E, Pappas PA et al. (2007) Contemporary clinical profile and outcome of prosthetic valve endocarditis. JAMA, 297, 1354 1361. 109 Wallace SM, Walton BI, Kharbanda RK, Hardy R, Wilson AP, Swanton RH (2002) Mortality from infective endocarditis: clinical predictors of outcome. Heart, 88, 53 60. 110 Moreillon P, Que YA (2004) Infective endocarditis. Lancet, 363, 139149. 111 Wilson W, Taubert KA, Gewitz M et al. (2007) 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. 112 Dajani AS, Taubert KA, Wilson W et al. (1997) Prevention of bacterial endocarditis: recommendations by the American Heart Association. Circulation, 96, 358366.
179
dard et al. E. Be
113 Gould FK, Elliott TS, Foweraker J et al. (2006) 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, 57, 1035 1042. 114 Li W (2003) Infective endocarditis. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds) Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 125133. 115 Ramsdale DR, Turner-Stokes L (2004) Prophylaxis and treatment of infective endocarditis in adults: a concise guide. Clin Med, 4, 545 550. 116 Ramsdale DR, ET, Wright P, Roberts GJ, Wallace P, Fabri B (2004) Guidelines for the prophylaxis and treatment of infective endocarditis in adults. http://www.bcs.com, 1 106. 117 Di Filippo S, Delahaye F, Semiond B et al. (2006) Current patterns of infective endocarditis in congenital heart disease. Heart, 92, 14901495. 118 Daebritz SH (2007) Update in adult congenital cardiac surgery. Pediatr Cardiol, 28, 96104. 119 Shore DF (2003) Late repair and reoperations in adults with congenital heart disease. In: Gatzoulis MA, Webb GD, Daubeney PEF (eds). Diagnosis and Management of Adult Congenital Heart Disease, Edinburgh: Churchill Livingstone, 7378.
180

151 Full

Uploaded by

Document Information

Original Description:

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

151 Full

Uploaded by

Copyright:

Available Formats

Adult congenital heart disease: a 2008 overview

dard1,2, Darryl F. Shore1,2, and Michael A. Gatzoulis1,2* Elisabeth Be

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

British Medical Bulletin 2008; 85: 151180 DOI:10.1093/bmb/ldn005

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

Overview of common CHD lesions

Contraception and pregnancy

British Medical Bulletin 2008;85

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

British Medical Bulletin 2008;85

Table 1: Overview common congenital heart lesions

Small (Qp/Qs less than 1.4, no LV volume overload): Observe

Adult congenital heart disease: a 2008 overview

Table 1:. Continued

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

British Medical Bulletin 2008;85

Unoperated patients: 90% mortality in infancy

Adult congenital heart disease: a 2008 overview

Table 1:. Continued 156

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

British Medical Bulletin 2008;85

Coarctation of the aorta10, 56, 61 66

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

British Medical Bulletin 2008;85

Ebstein anomaly of the tricuspid valve67 73

Adult congenital heart disease: a 2008 overview

Pulmonary Stenosis10, 27,

Symptoms (depend on the severity of PS): Mild to moderate: asymptomatic

Indications for balloon valvuloplasty: 1. Symptoms and/or 2. RV pressure . 2/3 of systemic

Good prognosis, normal life expectancy after repair

Table 1:. Continued

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

Patent Ductus Arteriosus78 83

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

British Medical Bulletin 2008;85

Causes: VSD, PDA, ASD, AVSD, truncus arteriosus.

Loud P2 May have holosystolic murmur of TR

Adult congenital heart disease: a 2008 overview

Pregnancy represents a major stress for the cardiovascular system due to

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

Pregnancy: risks for the mother

Adult congenital heart disease: a 2008 overview

Table 2 Contraceptive methods for women with CHD.

Combined oral contraceptive

Progestin-only pill (Mini pill)

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

Hormonal intrauterine system (Mirenaw coil)

Sterilization (laparoscopic tubal occlusion)

British Medical Bulletin 2008;85

Other conditions with significant increased maternal risk of cardiovascular events

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

Adult congenital heart disease: a 2008 overview

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

Labour, delivery and postpartum period

Pregnancy in women with CHD: when to refer?

British Medical Bulletin 2008;85

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

Modified from Colman.104

Adult congenital heart disease: a 2008 overview

Downloaded from http://bmb.oxfordjournals.org/ by guest on January 26, 2013

Possibility of paradoxical embolism