Anesthesia And Congenital Heart Disease

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REVIEW ARTICLEMartin J. London, MD

William C. Oliver, Jr, MDGregory A. Nuttall, MD

Section Editors

Anesthesia and Adult Congenital Heart Disease

Pierre-Guy Chassot, MD,* and Dominique A. Bettex, MD†

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ONGENITAL HEART DISEASES (CHDs) are commonpathologies because they occur in 0.5% to 1% of births;

mong them, complex malformations are less frequent (0.15%f births).1 The major advances made over the past 30 years inongenital cardiac surgery have resulted in an increased num-er of children born with heart disease who enjoy long-termurvival; 85% of these babies are expected to reach adulthood.2

he 15-year survival rate is 80% and 95% for complex andimple CHD, respectively; half of the patients with complexhysiology are over 25 years old.3 Moreover, this population isrowing at a rate of 5% each year.2 Any anesthesiologist mightherefore encounter one of these patients in his/her daily prac-ice of cardiac or noncardiac surgery. His/her role is pivotal inhe management of these cases, particularly during noncardiacurgery and obstetrics when his/her understanding of the patho-hysiology should lead the decisions of the operative team.4

In adulthood, patients with CHD can be allotted to 3 differentategories:

1. Patients with previous complete correction of theirdefect: even complete anatomic correction can leavehemodynamic sequelae, which are proportional to theremodeling of the cardiac chambers and to the age atwhich the operation was performed—the earlier thesurgery (�3 years), the less important the sequelae.Nevertheless, only ductus arteriosus, ostium secundumatrial septal defect (ASD), and isolated ventricular sep-tal defect (VSD) may be corrected without after effectsif surgery is in the first years of life.5

2. Patients with partial or palliative surgery: the hemody-namic behavior of these patients is frequently complexand far different from normal physiology (eg, after aFontan procedure). These “physiologic” repairs might

From the *Department of Anaesthesiology, University Hospital ofausanne, Lausanne, Switzerland; and †Department of Cardiac An-esthesia, Institute for Anaesthesiology, University Hospital of Zürich,ürich, Switzerland.Address reprint requests to Pierre-Guy Chassot, MD, Department of

naesthesiology, University Hospital of Lausanne, BH-10, CHUV,H-1011 Lausanne, Switzerland. E-mail: [email protected]© 2006 Elsevier Inc. All rights reserved.1053-0770/06/2003-0025$32.00/0doi:10.1053/j.jvca.2005.12.016Key words: congenital heart disease, adult anesthesia, intraopera-

ive transesophageal echocardiography

14 Journal of Cardiothor

correct cyanosis and relieve pulmonary hypertension;however, they have significant long-term complica-tions, particularly when the anatomic right ventricle(RV) must function as the systemic ventricle.

3. Nonoperated patients: some malformations are diag-nosed only in adulthood because they are benign (eg,ASD and bicuspid aortic valve) or because they pro-duce a progressive mismatch between systemic andpulmonary circulation and ventricular failure (eg, Eb-stein anomaly and congenitally corrected transpositionof great arteries). Spontaneous development of collat-eral circulation may obviate a restricted flow, likeaortopulmonary collaterals in the cases of tight pulmo-nary stenosis. Finally, some patients are immigrantsfrom countries without access to corrective cardiacsurgery.

Five different factors have an independent prognostic valueor perioperative complications: pulmonary hypertension, cya-osis, reoperation, arrhythmias, and ventricular dysfunction.rown-up patients with CHD may additionally develop pathol-gies that are characteristic of adulthood, such as arterial hy-ertension, diabetes,and coronary artery disease. These issuesecome more prominent because this population is growinglder.After an overview of the anatomic nomenclature of CHD,

his article describes the hemodynamic peculiarities of the mainongenital pathologies as they appear in adults, along with theathophysiology of their correction or palliation. Finally, someecommendations will be provided concerning their manage-ent by the anesthesiologist.

ANATOMIC NOMENCLATURE

The extreme diversity of CHDs calls for structural classifi-ation. The basic concept of this classification is a segmentalpproach6,7; the heart is considered in terms of 3 segmentsatria, ventricles, and arterial trunks) connected via 2 junctionsatrioventricular and ventriculoarterial) (Fig 1). The definitionf the segments is based on their intrinsic morphology becausehe usual criteria of size and position are not relevant inongenital diseases. In the sequential analysis, 5 criteria aresed to determine the anatomic structures of the heart:8

1. Situs: it can be solitus (normal) or inversus; because itis concordant with the abdominal status, the situs is
mainly defined by the position of the right atrium (RA).
acic and Vascular Anesthesia, Vol 20, No 3 (June), 2006: pp 414-437

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415ADULT CONGENITAL HEART DISEASE

2. Concordance or discordance of successive segments:instead of following each other normally (concor-dance), the different anatomic segments can be in aninappropriate relative position (discordance).

3. Segmental connections: the junctional parts betweensegments are the atrioventricular canal between atriaand ventricles and the infundibulum (or conus arterio-sus) between ventricles and arterial trunks.

4. Anatomic markers of each cardiac chamber: left ven-tricle is defined by 2 papillary muscles, a mitral valveand a partially fibrous outlet; the right ventricle isdefined by 3 papillary muscles, one of which is insertedon the septum; a tricuspid valve, of which the septalinsertion is more apical than the mitral annulus; and acompletely muscular outlet.

5. Associated abnormalities: dysmorphic chamber struc-tures, obstructive lesions, or septal defects.

The anatomic descriptions in this review are mainly based onransesophageal echocardiography (TEE) because it corre-ponds to the usual approach of the anesthesiologist in theperating room.Shunts may be located at the level of an ASD or a VSD.

hey may also be situated at the level of the central veinsanomalous pulmonary venous connections) or of the greatrteries (ductus arteriosus, aortopulmonary fistula, and coro-ary fistula). A shunt is defined by 3 characteristics.1. The direction of the flow: left-to-right (L-to-R), right-

to-left (R-to-L), or bidirectional. R-to-L shunts inducecyanosis. The flow through a shunt increases if theupstream pressure is increased or the downstream pres-sure decreased; in case of systemic arterial vasodila-tion, the flow of an L-to-R shunt declines, whereas theflow of an R-to-L shunt increases. To diminish a cya-notic shunt, the pulmonary artery resistances and theRV afterload must be decreased or the systemic arterial

Fig 1. Segmental analysis. The

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onnected through 2 junctions.

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and left ventricle (LV) afterload increased. t

2. The dimension of the defect: a shunt of small size(restrictive shunt) allows the passage of a small volumeof blood but generates a high-pressure gradient be-tween cavities or vessels; a large shunt does not impedethe blood flow, resulting in an absence of significantgradient and a large volume of blood.

3. The enlargement of the receiving chambers: isolateddefects situated upstream of the atrioventricular (AV)valves (ASD, anomalous pulmonary venous return)cause right-sided chamber dilatation, whereas lesionsdownstream of the AV junctions (VSD, ductus arteri-osus) induce early-on left-sided chamber dilatation; inboth cases, the pulmonary artery is dilated and its flowincreased.

The importance of a shunt is judged by the ratio between theulmonary flow and the systemic flow: Qp/Qs. It is calculatedy catheterization or by echocardiography. It is the ratio of theow or cardiac output measured in the pulmonary artery and in

he aorta (or left ventricular outflow tract). An L-to-R shunt,hich brings arterialized blood into the pulmonary circulation,

an also be calculated by the ratio of the O2 saturations asollows: Qp/Qs � (SaO2 � SvO2)/(SpvO2 � SpaO2), whereaO2 is arterial saturation (radial or femoral artery), SvO2 isentral venous saturation (mixed venous blood), SpvO2 is pul-onary venous saturation, and SpaO2 is pulmonary artery

aturation (enriched by the shunt). Because SpvO2 � SaO2 ifhe pulmonary gas exchange is normal, then Qp/Qs � (SaO2 �vO2)/(SaO2 � SpaO2).In the following example, the mixed venous blood (SvO2

4%) is enriched by an L-to-R VSD shunt to a pulmonaryrtery value of 90%: Qp/Qs � (98% � 74%)/(98% � 90%)nd Qp/Qs � 24/8 � 3:1. In this example, the ratio of pulmo-ary-to-systemic flow is 3:1. In case of an R-to-L shunt, theulmonary flow is decreased; a ratio of 0.5:1, for example,ndicates that the pulmonary flow is half the volume of sys-
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416 CHASSOT AND BETTEX

PATHOLOGIC APPROACH

Usually, congenital heart defects are classified according toheir impact on pulmonary blood flow, leading to cyanotic oroncyanotic pathologies. In this review, however, the authorsill consider the different pathologies following the segmental

pproach previously mentioned. Each description will be com-leted by comments concerning their surgical corrections, theirnesthesia management, and their intraoperative TEE imaging.

Persistence of a left superior vena cava (LSVC) is found in.5% of the general population and up to 10% of patients withHD.9 In 90% of the cases, the LSVC drains into the coronary

inus, which is strikingly enlarged on TEE images (Fig 2). Theight superior vena cava can be normal, absent, or hypoplastic.t is a harmless anomaly, except under the following situations:

1. In case of central vein placement, there is an increasedrisk of thrombosis of the coronary sinus; cathetersshould be removed as soon as possible. Pulmonaryartery catheters are contraindicated.

2. During cardiopulmonary bypass, the LSVC and coro-nary sinus flow must be drained by the venous cannu-lation.

3. If retrograde cardioplegia is used, the solution willescape into the LSVC, without protecting the heart.

4. In case of radiofrequency ablation, pacemaker, ortransjugular portosystemic shunt implantation, thetechnique must avoid damaging the coronary sinus.

Only partial anomalous pulmonary venous drainage is en-ountered in adults; 1 or 2 pulmonary veins drain into theystemic venous return. It is frequently associated with a sinusenosus ASD. The most common form is the anomalous drain-ge of the right upper pulmonary vein into the RA or into thease of the SVC. The right cavities are dilated because of

Fig 2. The LSVC. (A) In basal view, the LSVC (arrow) appears as a

eft atrial appendage (LAA). (B) In 4-chamber view of the right cavitie

n a left forearm vein appear in the coronary sinus.

olume overload. t

After correction of caval or pulmonary venous return, theow must present a normal biphasic systolic-diastolic patternn TEE imaging, with a maximal velocity below 1 m/s. Aontinuous nonphasic pattern and a peak velocity of �2 m/s arendicative of restriction.10

The ASD represents 7% of all CHD and 30% of the adultHD population; it is the second most common congenitalefect in adulthood after aortic bicuspid valve.11 The interatrialeptum may present 4 types of defects: ostium secundumdefect at the level of the fossa ovalis, representing 75% of theases), ostium primum (defect in the AV septum, 15% of theases), sinus venosus (frequently associated with an abnormalaval or pulmonary venous connection, 10%), and coronaryinus defect (unroofing of the coronary sinus into the left atriumLA], rare) (Fig 3). The volume overload secondary to the-to-R shunt is proportional to the dimensions of the defect; it

nduces a dilation of the RA, a dilated hypertrophy of the RV,nd an increase in size of the PA (Fig 4); the diameter of the PAs larger than the diameter of the aorta, and the flow velocity isncreased in the pulmonary artery and veins. The normal ratiof 0.6 between right- and left-heart dimensions and velocitiesay be more than doubled. The diagnosis is made at echocar-

iography; there is an echo dropout area in the interatrialeptum and a bright continuous systolic-diastolic flow from theA to RA (Fig 5).The L-to-R flow presents a typical biphasic cyclic pattern

Fig 5). Variations are related to the cardiac cycle and toespiration. One peak of flow occurs during late systole andarly diastole (synchronous with “v” wave), and one peakuring the atrial contraction (synchronous with “a” wave). Ahort period of R-to-L shunt can usually be recorded duringarly systole and mid-diastole.12,13 This flow pattern is consis-

ementary vessel between the left upper pulmonary vein (LUPV) and

coronary sinus (CS) looks very enlarged; the microbubbles injected

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he left and right atria14 (Fig 6). The most important shunteversal is observed in protosystole when the mitral annulusescent abruptly increases the LA volume and therefore de-reases its pressure.15 The pressure gradient between atria isodified by changes in the compliance and afterload of the

Superior sinusvenosus defect

Inferior sinusvenosus defect

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Fig 4. ASDs. (A) Schematic drawing of the shunt, with RA and RV e

ricuspid annulus; the RV is enlarged. (C) Ostium secundum defect (arrow

entricles, by systemic and pulmonary venous capacitance, andy AV regurgitation.The R-to-L component of shunt flow is increased during

udden intrathoracic pressure drops (increased venous return toA and decreased venous return to LA) like during spontane-

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Foramenovale (PFO)

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us inspiration, the relaxing phase of a Valsalva maneuver, orueller maneuver.16 It also increases when the afterload of RV

s elevated or during intermittent positive-pressure ventilationIPPV) or positive end-expiratory pressure (PEEP) (�15

Fig 5. ASD flow. (A) Color flow through an ASD (left-to-right shun

ow is predominantly left-to-right (below baseline), there are 2 right

iastole.

Fig 6. ASD flow. (A) Simultaneous recordings of the RAP and the

he RA. LAP is higher than RAP during “a” and “v” peaks of pressur

ight-to-left components of the shunt. (Modified with permission.16) (

5 cmH2O of PEEP to the same patient modifies the pattern of the flow

ecreased.

mH2O) (Fig 6). Therefore, paradoxic emboli may happen evenn cases of predominant L-to-R shunt.

A small ASD may remain asymptomatic for years; the onlyign is a 3/6 systolic ejection murmur heard at the left upper

) Pulse wave Doppler spectral display of the ASD flow; although the

ft components (above baseline), one during systole and one during

the amplitude of the pressure variations is larger in the LA than in

lower during nadir of pressure “x” and “y”; this fact explains the 2

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order of the sternum because of the high pulmonary flow andsplit fixed second heart sound. With time, adults may presentrogressively with shortness of breath, suprajunctional arrhyth-ias, and episodes of RV failure. The right ventricle is dilated

y the volume overload, and tricuspid regurgitation appears.bove the age of 40, the mortality of nonoperated patients

ncreases by 6% per year, whereas there are no long-termfter-effects if the surgical correction is performed before thege of 5 years.17 A large shunt may lead to pulmonary hyper-ension (rarely �500 dynes/s/cm5) and RV failure because ofongstanding pulmonary overflow. The right atrial pressureRAP) increases, the flow through the shunt decreases, and the-to-L component becomes major as cyanosis appears. Anisenmenger reaction occurs in less than 10% of patients older

hen 40 years. When the shunt is reversed, a surgical corrections no longer possible.

The importance of the shunt and the presence of pulmonaryypertension (PHT) are determining criteria for the manage-ent of the patient during anesthesia. Hemodynamic mainte-

ance will be achieved through a reduction of the L-to-R shunt.s long as PHT is absent, this is obtained by a slight increasef preload, normal-to-elevated pulmonary resistances, and lowystemic resistances. High ventilation pressure and PEEP mightnduce a pronounced R-to-L shunt and lead to hypoxemia;herefore, low-pressure IPPV is recommended. Hypovolemia isoorly tolerated because a high circulating volume is needed toaintain the shunt volume through the pulmonary tree in

arallel with the normal systemic blood volume. The low-esistance pulmonary circuit tends to steal volume from theigh-resistance systemic circulation. The risk of paradoxic em-oli through venous accesses (air bubbles, microaggregates) islways present, particularly during IPPV. Neuraxial blockadend halogenated agents like isoflurane are good choices be-ause systemic vasodilation diminishes the L-to-R shunt;

Fig 7. PFO. (A) A 90° view of the fossa ovalis membrane, the dista

ow shows the L-to-R shunt. (B) Microbubble test; microbubbles hav

trium, and many bubbles are visible in the LA.

ropofol, which decreases the preload, is theoretically less c

dapted. The central venous pressure (CVP) measurement isot a good marker of the circulating volume because it isncreased due to the shunt and the possible tricuspid regurgi-ation. If the shunt is bidirectional and the pulmonary arteryressure elevated, systemic vasodilation increases the R-to-Lomponent and decreases arterial saturation. The right ventric-lar function may be improved by increasing heart rate andontractility.

If a residual shunt is observed after surgical repair of anSD, it raises 2 critical questions: how large is the shunt and isreoperation indicated? Minimal residual shunting across the

uture line, appearing as a little flame-like jet at TEE, is withoutignificance; it frequently disappears after protamine injection.

large dehiscence is an indication for immediate surgicalevision. An increase of more than 20% in pulmonary arteryxygen saturation compared with the values in the SVC andnferior vena cava (IVC) will confirm the indication for surgicalevision.

Patent foramen ovale (PFO) is a common finding in the adultopulation: its incidence varies from 5% with 2D-echocardio-raphic examination to 27% at direct intraoperative visualiza-ion.18-20 The flap occluding the foramen ovale usually closesuring the first years of life by fusion with the interatrial wall.hen it only overlaps the septum on the LA side, the orificeay reopen if the RAP becomes higher than the left atrial

ressure (LAP). The PFO is frequently associated with anneurysm of the interatrial septum. The PFO is diagnosed atntraoperative TEE by color flow and contrast study (Fig 7).he injection of microbubbles, best performed into a centralatheter, must be synchronous with the release of endothoracicressure after a short period of high PEEP21; the normal pres-ure gradient between the atria is reversed, and the bubbles,ven in small numbers, appear in the LA and LV during theext four systoles.22 If their appearance is delayed by 5 cardiac

t is not attached to the interatrial septal wall; the oblique blue color

d the right atrium, the interatrial septum is bulging toward the left

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assage. The test is positive when more than 5 bubbles crosshe septum during a single cardiac cycle. It detects 92% ofFOs.20

The incidence of paradoxic emboli is much lower than thencidence of the anatomic lesion itself because the risk oferebral vascular accident is 1% to 2% per year in patients withPFO.23 A PFO, normally asymptomatic, may cause paradoxicmboli during a Valsalva maneuver or PEEP; it is a cause ofefractory hypoxemia in patients on IPPV. Systemic arterial airmboli may also occur after deep-sea diving, during laparo-copic procedures, or during the sitting position in neurosur-ery.24,25

Endocardial cushion defect or AV canal is a lack of centraleptation of the heart. In the complete form, the AV canalresents a large ostium primum ASD, a large VSD extendingnto the membranous septum, and a common AV valve sur-ounding an orifice that creates clefts in the septal leaflets of thericuspid and mitral valves26 (Fig 8). The tricuspid and mitralalves appear inserted at the same level on the septum in the-chamber view. The shunt flow is composite and may take theollowing configurations: shunt between LA and RA, LV andV, or LV and RA and mitral or tricuspid regurgitation. TheV canal is frequently associated with Down’s syndrome (tri-

omy 21). The clinical presentation is determined by the dom-

LARA

RV LV

Central cleft in septal leaflets of tricuspid and mitral valves

Mitral cleft regurgitation

Membranous VSD

:Ostium primum ASD

A B

Fig 8. Atrioventricular canal defect (AV canal or endocardial cush

ous ventricular septal defect and a cleft in the septal leaflets of the m

egurgitant flow through the mitral cleft. (B) Normally, the septal leafl

f the mitral valve; the distance between both insertions is less than

he same level (arrow).

nant lesion, which is usually the VSD. The RA is dilated, and fi

he RV and LV are hypertrophied and dilated. PHT appearsarly if the VSD is large but is unusual in the case of isolatedSD. AV canal in its complete form is seldom encountered in

dults. Partial AV canal (absence of VSD), however, may beiagnosed late. Long-term complications of previous surgicalorrection may be present, such as progressive mitral regurgi-ation, subaortic stenosis, or AV block; they represent 10% ofhe cardiac operations performed in adults with CHD.27

The surgical correction of AV canal consists of ASD andSD closure, with or without patch, and AV valves repair. Theostoperative echocardiographic assessment should address theompetence of the AV valve and search for residual septalefects or left ventricular outflow tract (LVOT) obstruction.mall jets of AV regurgitation or residual shunts are frequentlyncountered after correction of AV canal; most of them disap-ear after protamine administration and hemodynamic stabili-ation. Hemodynamic management of these patients is mostlyhe same as with ASD or VSD, depending on which lesionredominates.Ebstein’s anomaly is characterized by an apical displacement

f more than 10 mm of the insertion of the septal and some-imes posterior tricuspid leaflets; the anterior leaflet is usuallyarge and dysplastic. Tricuspid leaflets progressively displaympaired mobility because of chordae shortening, tethering, or

RA LA

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efect). (A) The ostium primum defect is accompanied by a membra-

and tricuspid valves; there are shunts through the ASD and VSD and

the tricuspid valve is inserted more apically then the anterior leaflet

m. (C) In AV canal, both mitral and tricuspid leaflets are inserted at

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s atrialized; the RA is enlarged, and an interatrial shunt is oftenssociated. The tricuspid valve may be regurgitant, stenotic, oroth. The auscultation is characterized by a widely split firsteart sound at the lower left sternal border and an early mildystolic murmur of tricuspid regurgitation. There is a poororrelation between the morphologic findings and the resultanteverity of the lesion.28 The clinical manifestation is linked tohe restriction of the RV cavity including venous stasis, hepa-omegaly, and congestive right-heart failure. The patient mayecome cyanotic in cases of associated ASD or PFO. In 30% ofhe cases, severe arrhythmias are present including AV heartlock, or Wolff-Parkinson-White syndrome. They might be therst manifestation of the disease.4 Prognostic factors are theunctional status, the size of the heart, the severity of arrhyth-ias, and the cyanosis if present.For the anesthesiologist, the hemodynamic pattern is domi-

ated by 2 symptoms: the arrhythmias and the restriction to RVlling. After surgical repair, the tricuspid anterograde flowust be unrestricted (acceptable mean gradient �5 mmHg); a

mall residual regurgitation (grade �II) is acceptable. Postop-rative AV block is particularly frequent after tricuspid valveepair. Central venous cannulation might be dangerous becausetrial stimulation with a catheter produces malignant ventricu-ar arrhythmias because of the atrialization of the RV.

VSDs represent 10% of adult congenital cases. The inter-entricular septum is mainly muscular, with the exception of amall membranous segment located at its superior border justeneath the right and noncoronary cusps of the aortic valve.he defect can appear in 4 different parts of the interventriculareptum (Fig 10):

1. Membranous or perimembranous VSD: 68% of all

Fig 9. Ebstein’s anomaly. (A) Schematic drawing of the lowered in

eaflet of the tricuspid valve (arrow) is inserted low inside the right v

ecause of the atrialization of the RV inlet. MV, mitral valve.

cases (Fig 11A); it can be progressively closed by

marked septal hypertrophy surrounding the defect or byredundancy of the TV septal leaflet.29

2. Inlet VSD is part of an endocardial cushion defect; itappears between the mitral and tricuspid leaflets.

3. Supracristal or infundibular VSD: immediately belowthe pulmonary and aortic valves; it is frequently asso-ciated with aortic regurgitation because of a prolapse ofthe right coronary cusp.

4. Muscular VSD: frequently multiple, it is less frequentamong congenitals than in acquired cases (Fig 11B).

Up to 40% of congenital VSDs close spontaneously duringhe first years of life.30 The VSD creates an L-to-R shunt, whichverloads the pulmonary circulation and the left ventricle.natomically, most of the congenital defects are situated close

o the RV admission chamber and outflow tract, in such a wayhat the shunted blood bypasses the right ventricular cavity. Theressure and volume work are performed by the left ventricle,hich is chronically overloaded by the excess volume shunted

hrough the lungs (Fig 11C); left ventricular failure may ensue.he right ventricle hypertrophies only when PHT supervenes.uscultation reveals a holosystolic harsh murmur at the lower

eft sternal border, radiating in a circle, with a prominentystolic thrill. Adult patients with VSD can be divided in 4ifferent groups:1. Spontaneously or surgically closed VSD and no shunt.

There is no or minimal residual shunt; the pulmonarypressures are normal. The long-term outcome of thesepatients is excellent if the surgical closure has beenperformed before the age of 2 and if there is no sig-nificant remodeling of the ventricles.5

2. Small VSD, small shunts, and normal pulmonary pres-

n of the tricuspid valve into the RV. (B) A 4-chamber view: the septal

ular cavity; the RV is decreased in size, whereas the RA is very large

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sures. The pulmonary flow is slightly increased, and the

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pulmonary pressures are at the upper normal limit.31

The life expectancy is good, even if the risk of endo-carditis is elevated.32

3. Moderate shunt and elevated pulmonary pressure. TheRV is hypertrophied; PHT is still reactive to hypocar-bia, hyperoxia, and NO. The life expectancy is 86% at25 years old.33

4. Large shunts and Eisenmenger syndrome. Eisenmengersyndrome is defined as a severe nonreactive PHT (pul-monary vascular resistance [PVR] �800 dynes/s/cm5)and a progressive equalization of right and left ventric-ular pressures. The shunt becomes bidirectional; theRV dilates and fails, with venous stasis and tricuspidregurgitation. Forty percent of patients die before theage of 25.33,34 A ratio of PVR/systemic vascular resis-tance (SVR) larger than 0.7 speaks for a prohibitivesurgical risk.

At the echocardiographic examination, the left-sided cavitiesre enlarged because of volume overload; the PA is dilatedecause it is the receiving chamber of the shunted flow. The RVs hypertrophied and dilated proportionally to the degree ofHT. Color-flow mapping will reliably identify the defect by

maging the characteristic flow jet within the RV and by visu-lizing the flow convergence area on the LV side; the dimen-ion of this proximal converging flow field is a good estimatef the size of the VSD.35 With high-volume L-to-R shunts,hese color images appear both during systole and diastole,hereas the shunt flow is recorded only during early systole in

mall VSDs. The systolic pressure difference between right andeft ventricles might be calculated from the shunt Vmax using theimplified Bernoulli equation (�P � 4 Vmax

2). In the absence ofortic or subaortic pathology, the subtraction of the gradient�P) from the peripheral systolic blood pressure gives anccurate assessment of the RV systolic pressure. It is usuallyecommended to not calculate the RV systolic pressure fromhe velocity of tricuspid regurgitation because the RV pressures contaminated by the shunt flow, which is determined by the

embranousefect

Atrio-ventriculardefect

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V pressure. However, it is possible to calculate the RV l

ystolic pressure if the alignment of the echocardiographicnterrogating beam is exactly superposed with the main axis ofhe tricuspid regurgitation jet and if the VSD is restrictive.

The surgical correction of a VSD is mandatory if the shunt isoderate or major (Qp/Qs �1.5), if the defect is part of a

omplex disease (AV canal or tetralogy of Fallot [TOF]), or ifhe defect is situated in the LVOT because of the risk of aorticegurgitation.36 A correction performed during the first 2 yearsuarantees normal long-term ventricular performance and pul-onary pressure. Bundle-branch blocks or complete AV blocks

re frequent postoperatively because of the close vicinity of theonduction fibers; many patients are dependent on a permanentacemaker.4

The aim of the anesthesiologist is to reduce the amount oflood shunted through the defect. This can be achieved by aecrease in the systemic pressure (arterial vasodilation) and anncrease in RV pressure (pulmonary vasoconstriction). Generalnesthesia with isoflurane or neuraxial blockade is well de-igned for this purpose. As long as there is no pulmonaryypertension, the anesthesia is conducted under normocarbia orlight hypercarbia, low oxygen inspired concentration (FIO2 �.3), and PEEP. If PHT is present, on the contrary, the hemo-ynamic priority is to decrease pulmonary artery pressure.Patients with a VSD are very sensitive to hypovolemia

espite their increase in circulating blood volume because theolume shunted through the lungs depends on the diameter ofhe defect and on the PVR. Because the dimension of the defects fixed and the PVR is lower than the systemic resistances, thelood volume is “stolen” through the shunt, and any loss oflood will aggravate this behavior. This is further increased byhe reflex systemic arterial vasoconstriction of hypovolemia.

Minimal residual shunting across the suture line of the patchs frequently found after surgical repair of a VSD and is withoutignificance; only large dehiscence (�3 mm) of the patch, withsignificant flow convergence on the LV side, is an indication

o perform immediate surgical revision.37 An assessment of thericuspid valve is important because tethering of the septal

Infundibularsupracristaldefect

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Fig 10. VSDs. The view of the

interventricular septum is taken

from the right ventricle. The most

common congenital VSDs are sit-

uated close to the right ventricular

outflow tract. (Adapted with per-

mission from Chassot and Bet-

tex.116)

eaflet is possible during perimembranous VSD closure. The

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ight coronary cusp of the aortic valve should be checked forhe same reason. The technique of closure of the VSD throughhe RA requires detachment of the septal tricuspid leaflet; afteruture of the patch, this leaflet is reconstructed. The grade ofhe residual regurgitation must be evaluated.

The ventricular segment may present variable degrees ofypoplasia with marked asymmetry up to the absence of oneentricle (univentricular heart). The main chamber, which re-eives blood from both atria and ejects through both greatrteries, becomes more or less circular because of the volumeverload; the degree of its remodeling is a useful predictor ofostoperative ventricular dysfunction.38,39 The flow repartitionetween the aorta and pulmonary artery is linked to the equi-ibrium between systemic and pulmonary resistances. Shuntingt the atrial level is crucial for survival. Children with hypo-lastic ventricles generally do not survive into adulthood with-ut palliative surgery. Only anecdotal cases have reached adultge because they presented a nonrestrictive ASD and a pulmo-ary stenosis to limit the pulmonary flow. Different operationsave been designed to correct these anomalies, like the Fontannd Norwood procedures. They lead to very special hemody-amic situations.

Fig 11. VSDs. (A) Perimembranous VSD, partially obstructed by t

f the interventricular septum. (B) Muscular VSD, situated in the dist

hunt, which flows into the pulmonary artery and returns to the left

The Fontan operation consists of bypassing the tricuspid f

alve and right ventricle and rerouting the systemic venousow directly into the pulmonary artery; this can be performedy a wide range of procedures. The simplest is an end-to-sidenastomosis of the SVC to the right PA (Glenn procedure); thisperation is performed in children because the SVC flow isore important in babies than it is in adults. Nowadays, it is

onsidered as the first step of a complete Fontan derivation.ince its description in 1971, the Fontan procedure has contin-ously evolved including atriopulmonary connection, internalunnel from the IVC to the PA, interposition of a conduitetween systemic venous return (IVC) and the pulmonaryirculation40 (Fig 12). Maintaining the RA in the circuit leads tots massive dilation, with an increased risk of tachyarrhythmiasnd thrombosis.41 Critical issues to these reconstructions are thedequacy of the pulmonary bed, the low PA resistances, the AValvular competency, good systemic ventricular function, andhe sinus rhythm.42 The pulmonary flow drive relies entirely onhe pressure gradient between the CVP and LAP; a gradient of

to 10 mmHg is necessary to provide this flow, and LAPhould be in the range of 5 to 8 mmHg.42 Any elevation of theulmonary resistances (hypoxia, atelectasis), of the intratho-acic pressure (Valsalva, IPPV, PEEP), or of the LAP (LV

ptal leaflet of the tricuspid valve; it is situated in the upper portion

t of the interventricular septum. (C) Schematic drawing of the L-to-R

m and left ventricle, which is overloaded.

he se

al par

ailure, mitral regurgitation) dramatically decreases the pulmo-

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ary flow. Patients with well-functioning Fontan repairs haven arterial saturation of 95% at room air; an SpO2 below 90%s the sign of an insufficient flow.42 Arrhythmias are common40% of the cases) and are poorly tolerated.41 Systemic venoustasis is frequent; 28% of the patients present ascites and somerotein-losing enteropathy. Hepatic dysfunction may lead tooagulation disorders.43 The incidence of thromboembolicvents is 18%.44 The survival rate at 15 years is 75%.45 Theontractile function of the single ventricle is reduced; theunctional reserve is very limited because of the inability toodify the heart rate (arrhythmias and conduction abnormali-

ies) and the preload (pulmonary driving pressure).46 The he-odynamic performance is less depressed when the single

entricle is the anatomically left ventricle (tricuspid atresia).The flow profile through the venopulmonary anastomosis or

n the conduit shows a biphasic forward pattern of moderateelocity (0.2-0.5 m/s).9 A higher flow velocity (�1.5 m/s),ithout cyclic variations, and not reaching baseline during

ardiac and/or respiratory cycle, is suggestive of a significantbstruction.47 The flow pattern is highly dependent on respira-ion; it is maximally increased during spontaneous inspiration.low attenuation or even reversal has been shown during the

Fig 12. Fontan procedures. (A) Schematic drawing of the RV byp

econstructions (anastomoses or conduits between the RA or the IVC

s similar to a central vein flow; it is highly dependent on the system

ressure rises with each mechanical inspiration. (C) In spontaneous

ntrathoracic pressure aspirates blood into the pulmonary artery. Th

VC-PA conduit with the TEE probe in the transgastric position; the b

ight pulmonary artery; SV, single ventricle. (Adapted with permissi

ositive-pressure phase of IPPV48 (Fig 12). Under those cir-

umstances, TEE might be used to adequately manage theentilatory pattern of the patient.The entirely passive pulmonary flow determines the hemo-

ynamic management of these patients when they are undernesthesia.49 During management, the following should be keptn mind:

1. Hypovolemia is dangerous: a fall in preload immedi-ately decreases the pulmonary flow.

2. CVP should be at least 15 to 18 mmHg because it is theactual pulmonary arterial pressure. Monitoring theCVP is extremely useful, but the risk of thrombosis isvery high; the catheter must therefore be removed assoon as possible. TEE is a less invasive method formonitoring the preload.

3. Pulmonary vascular resistance must be low; acidosis,hypoxia, and hypercarbia must be avoided. Slight hy-pocarbia is necessary (hyperventilation with low respi-ratory pressure).

4. Any increase in LAP is deleterious: LV dysfunction,mitral insufficiency, or loss of sinus rhythm with canonwaves are poorly tolerated.

5. The subatmospheric intrathoracic pressure during

y the Glenn procedure (SVC to RPA anastomosis) and the Fontan

the main pulmonary artery). (B) The flow through a Fontan conduit

nous pressure; during IPPV, it is interrupted when the intrathoracic

iration, the flow is increased during inspiration when the negative

w is situated under the baseline because it is recorded through an

is flowing away from the transducer. SVC, superior vena cava; RPA,

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pulmonary flow. On the contrary, IPPV might interruptthe flow during inspiration. Therefore, spontaneousventilation is the best choice, as long as hypercapniaand hypoxia do not supervene. If paralysis and IPPVare necessary, the airway pressures should be kept aslow as possible, without PEEP, but with slight hyper-ventilation to induce hypocarbia.

6. The ventricular function is mostly limited and mightrequire inotrope stimulation; any further decreasethrough negative inotropic substances should beavoided.

7. Epidural anesthesia is a good solution, as long as thepreload is stable. A spinal block might induce an acuteand dangerous drop in venous return.50

8. In the postoperative period, spontaneous ventilationshould be resumed as soon as possible; prophylacticanticoagulation should be started very early.

TOF is the third most common lesion encountered in adults.he basic abnormality is the underdevelopment of the rightulmonary infundibulum; malalignement and deviation of thearietal band result in an obstruction to pulmonary outflow andn a large subaortic VSD. TOF comprises 4 anomalies: mem-ranous VSD, overriding aorta, right ventricular outflow tractRVOT) and/or pulmonary valve stenosis, and RV hypertrophyFig 13). The RV hypertrophy is secondary to the increased

Fig 13. TOF. (A) Schematic drawing of the 4 components of the TO

ypertrophied right ventricle. (C) Right ventricular hypertrophy with m

fterload because of its outflow obstruction and its connection a

o the systemic circulation through the VSD. The subvalvularuscular narrowing has an important dynamic component and

ehaves like an obstructive cardiomyopathy; any sympathetictimulation or loss of preload increases the systolic narrowingf the outflow tract and deepens the cyanosis. This obstructions associated with variable degrees of pulmonary valvular ste-osis and thickening and hypoplastic pulmonary arteries. Theulmonary arterial flow and pressure are low. The aorta over-iding the VSD receives blood from both ventricles; the cya-osis is proportional to the degree of mixing. If the systemicrterial resistance is increased, the proportion of flow comingrom the RV (R-to-L shunt) decreases, and the arterial oxygenaturation increases. The aortic valve is enlarged and competentn children, but progressive aortic dilation may occur with age,ostly in unoperated patients, leading to mild aortic regurgi-

ation (grade I-II) in 75% of adult cases.51 In 25% of cases, aecundum ASD creates a pentalogy. Coronary anomalies areound in 10% of cases.

Patients are cyanosed and show drumstick finger deforma-ion, but blue spells do not occur in adults. They present with

4/6 systolic ejection murmur and a thrill at the left sternalorder in the second or third intercostal space. A medium-requency mid-diastolic murmur in the third left intercostalpace is typical of pulmonary regurgitation. Nonoperated pa-ients have a survival rate of 30% at 10 years and less than 3%

Aorta overriding the VSD at 120° view, large membranous VSD and

lar stenosis of the RVOT (arrow). RVH, right ventricular hypertrophy.

F. (B)

t 40 years.52 Those who survive into adulthood have either a

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ild degree of pulmonary flow restriction or a pulmonarylood flow maintained through multiple aortopulmonary col-aterals originating anywhere in the thoracic arterial system; inhese cases, the diastolic arterial pressure is low because thelood flows from the thoracic aorta into the low-pressure pul-onary tree in diastole. Some degree of pulmonary stenosis is

ound in 10% of adult patients with CHD.1 Anatomically, themage is close to that of a TOF but without a VSD andverriding aorta. They are not cyanosed at rest and are fre-uently described as “pink Fallot.” If major collateral arteriesan preserve sufficient pulmonary flow, the children with se-ere pulmonary stenosis or atresia can survive until adoles-ence or adulthood.

On echocardiographic imaging, the large aortic valve is atriking feature. It opens to both ventricles by a large subaorticefect located between the right and noncoronary cusps (Fig4). The blood flows through the VSD from both ventricularhambers toward the aorta; there is no clear-cut shunt from theV to the RV. An intense turbulent flow is shown at the RVOTtenotic area; it might be at the subvalvular level (dynamic orembranous stenosis), at the valvular level (fibrosis and ste-

osis of the pulmonary valve), or in the pulmonary tree (com-ined stenoses and membranes). Usually, the maximal velocity3 m/s) peaks in early systole in case of fibrous stenosis,hereas it appears later in systole in case of dynamic muscularbstruction. Shunts and collateral circulation appear as contin-ous systolic-diastolic turbulent flow images in the posteriorediastinum; they can sometimes be traced from the aorta to a

ulmonary vessel.In the 1960s and 1970s, surgical correction was not per-

ormed before the age of 5 to 10 years. Therefore, to maintainnough pulmonary flow, many of these patients have hadurgically implemented shunts in infancy (Blalock-Taussighunt between subclavian and R or L pulmonary artery or

Fig 14. Subaortic and aortic disease. (A) A fibromuscular ridge is

n the septum (double arrow). AoV: aortic valve. (B) Short-axis view o

�) is smaller than the anterior leaflet (��), which corresponds to the

entral aortopulmonary shunts). The burden of this strategy was I

permanent cyanosis, pressure overload on the RV, volumeverload on the LV, and distortion of the pulmonary arteries.owadays, the complete correction is performed in infancy or

arly childhood (�2 years), which relieves the cyanosis andV remodeling and allows the pulmonary tree to grow.53,54

urgical correction of the TOF consists of closing the VSD andnlarging the RVOT and PA. When necessary, a valved conduits placed between the RV to the main PA; unfortunately, theseonduits have a restricted lifespan and generally require furthereplacement after a variable lapse of time. Patching the pulmo-ary valve annulus induces regurgitation proportional to theelief of the obstruction. The surgical result is therefore aompromise between minimal regurgitation and some residualradient; a peak gradient up to 20 mmHg is considered normal.max higher than 3 m/s and gradients above 40 mmHg indicate

ignificant stenosis.55 The maximal velocity may overestimatehe real gradient because of the phenomenon of pressure re-overy in the case of a tubular narrowing or due to an excessiveystolic volume associated with pulmonary regurgitation. Theong-term survival after early complete correction is 85% at 36ears,56 but numerous patients need a subsequent reoperation.

The surgical correction might have major long-term se-uelae: arrhythmias in more than 50% of the cases (right orifascicular bundle-branch block, ventricular ectopy, and atrialbrillation or flutter), residual VSD (10%-20%), RV outflow

ract obstruction, and pulmonary valve/conduit stenosis or re-urgitation.57 Advanced age at the time of operation, incom-lete correction, RV overload, and dysfunction are predispos-ng factors for arrhythmias and sudden death.58 Some degree ofulmonary regurgitation is present in the majority of patients; its the single most important factor determining the level of RVilation and performance with time.59 In adult cases correcteduring childhood, the pulmonary regurgitation (PR) might be-ome the main pathology; when PR becomes severe (grade

e in the LVOT beneath the anterior mitral leaflet (single arrow) and

elliptical opening of a bicuspid aortic valve (Ao); the posterior leaflet

n of the right and left coronary cusps.

visibl

f the

II-IV), the RV sustains a volume overload, dilates, and finally

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ails. On the other hand, a residual pulmonary stenosis reduceshe pulmonary flow, further increases the RV pressure andypertrophy (RV:LV pressure ratio �0.5), and may eventuallyead to RV failure. The correction is performed by interposing

valved conduit between the RV and the PA, which protectshe ventricle from arrhythmias and volume or pressure over-oad.60,61

Anesthesia for noncorrected patients is governed by 2 he-odynamic goals: decrease the R-to-L shunt and increase the

ulmonary flow (Fig 15). Systemic arterial vasoconstrictionecreases the R-to-L component of the shunt; however, ithould not be such that the afterload becomes excessive foroth ventricles. The SpO2 is an efficient and immediate meansf monitoring the degree of shunting. For increasing pulmonaryow, �-blockade is necessary to relieve the systolic narrowingf the RVOT in case of dynamic obstruction; if the obstructions fixed (subvalvular membrane, pulmonary valve stenosis),-stimulation decreases the shunted component of the RVystolic volume and increases the flow through the pulmonaryree. It is mandatory to keep the patient normovolemic, sinceypovolemia increases the R-to-L component of the shuntecause of the low systemic arterial pressure.If a palliative aortopulmonary shunt is present (Blalock-

aussig or Waterston), the pulmonary perfusion is dependentn the systemic arterial pressure; systemic hypotension de-

Fig 15. Anatomically corrected transposition of the great arteries.

he anatomic LV, with the mitral valve, is on the right side and conn

apillary muscle, is on the left side and connected to the aorta. (B) A 4

arrow) show that the mitral valve (MV) is connected to the RA and t

ide, and the small triangular LV is on the right side. (Adapted with

reases the pulmonary flow and induces cyanosis. The same p

ituation happens in patients who survive only because anmportant collateral circulation supports the pulmonary flow.he diastolic blood pressure is usually low because of theontinuous flow from the aorta to the low-pressure pulmonaryrteries. Moreover, the burden of systemic and pulmonaryirculation is on the LV, which is chronically overloaded, anday ultimately fail.The function of both ventricles is better preserved if cyanosis

s absent and if the surgical correction has been made in earlyife. However, the disease is not completely cured by surgery.he major risk is defined by the right ventricular function. Inases of residual pulmonary valve regurgitation, the RV isolume overloaded; the goal is to decrease the PVR, whichnclude hyperventilation, hypocarbia, hyperoxia, low ventila-ory pressure. In case of pressure overload because of residualulmonary stenosis, the risk of RV subendocardial ischemiaequires maintaining an adequate systemic arterial pressure andnormal heart rate. The use of inotropes is frequently neces-

ary. The LV sustains a volume overload that is proportional tohe flow through the collateral circulation. Arrhythmias arerequent; there is a 60% incidence of bundle-branch block,ost often right; and ventricular tachycardia happens in 5% of

he cases.62,63

Stenosis of the LVOT obstruction may be static or dynamic.n the first case, a more or less circular fibrous thickening is

e atrioventricular and the ventriculoarterial junctions are discordant;

d to a PA. The anatomical RV, with the tricuspid valve and a septal

ber view: the septal insertions of the mitral and the tricuspid valves

icuspid valve (TV) to the LA; the enlarged anatomic RV is on the left

ission from Chassot and Bettex.116)

(A) Th

ecte

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resent in the middle of the outflow tract; this ridge or dia-

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hragm of fibromuscular tissue extends from the interventric-lar septum to the anterior leaflet of the mitral valve (Fig 14A).he aortic valve is frequently hypoplastic. The symptoms are

he same as those of an aortic stenosis. The second case isnalogous to the LVOT dynamic obstruction of hypertrophicdiopathic subaortic stenosis. The blood flow velocity exceeds.5 m/s in the LVOT (normal: 1 m/s) and the dynamic gradients greater than 25 mmHg. It is further aggravated by sympa-hetic stimulation, hypovolemia, and arterial vasodilation. Bothathologies may accompany a stenosis or regurgitation of thealve itself. Recurrence of subaortic stenosis is frequent be-ause the development of the LVOT is abnormal.

The surgical treatment consists of resection of the membraner of a myectomy of the LVOT; the major complications are anccidental VSD because of excessive resection or a residualtenosis because of incomplete resection. At postoperative TEExamination, care must be taken to exclude an iatrogenic VSDnd an injury of the mitral or aortic valve. The velocity can stille increased at the septal bulge according to hemodynamiconditions and may affect the incidence of reoperation.64 Thempact of TEE examination on surgical results of LVOT re-onstruction is very high: in 12% to 55% of the cases, anmmediate surgical revision is indicated by the postbypassxamination.65

Bicuspid aortic valve is the most common congenital cardiacnomaly; its incidence is 2% in the global population.66 It isdentified by 2 cusps of unequal size, the larger of which mayave a fibrous raphe at the site of fusion. It presents a fish-outh opening (Fig 14B). Rarely, the valve may be unicuspid,ith a central hole simultaneously generating stenosis and

ncompetence. The bicuspid valve is fragile; the stress on theeaflets is more pronounced than in a normal valve becauseheir edges cannot touch each other anatomically in diastolend cannot reach the walls of the sinus of Valsalva in systole;hey vibrate during ejection and give rise to a systolic murmur.

ith continuous stress over the years, the valve may becomealcified and stenotic, usually after the age of 40; by annularilation, it can also lose tensile strength and become incompe-ent. The restriction to flow is characterized by a mean gradientbove 50 mmHg in cases of severe stenosis and normal LVunction. The auscultation of aortic stenosis is typical, includ-ng 5/6 systolic ejection murmur with thrill, paradoxic split ofhe second heart sound, and ejection click at the apex. Aorticicuspid valve is frequently associated with aortic coarctationnd perimembranous VSD. A prolapse of the right coronaryusp may supervene in infundibular VSD and aggravate theathology.In complete transposition of the great arteries (TGA), the

orta arises from the anatomic RV (subaortic ventricle) and theA originates from the anatomic LV (subpulmonic ventricle);

here is a ventriculoarterial discordance. The great arteries arisen parallel at the base of the heart. The aortic valve is anterior,o the right, and slightly superior to the pulmonary valveD-transposition). The PA originates directly above the LVOT.ulmonary and systemic circulations are running in parallel.urvival is possible if an ASD allows mixing between theulmonary and systemic circuits. A VSD is present in 30% to0% of the patients. Three main therapeutic approaches govern
he surgical treatment. A
1. Rashkind procedure: the interatrial septum is torn bycatheterization to create or enlarge an ASD; this is anemergency palliative operation performed during thefirst hours of life.

2. Mustard or Senning procedures: at the atrial level, thesystemic venous return is redirected to the subpulmonicventricle and the pulmonary venous blood to the sub-aortic ventricle by a complex interatrial baffle. Unfor-tunately, the systemic ventricle remains in the RV; itfails after 20 to 25 years because of pressure over-load.67

3. Jatene operation: the great arteries are switched duringthe first 2 to 3 weeks of life, before the remodeling ofthe ventricles takes place. The PA is connected to theRV and the aorta to the LV; the coronary arteries arereimplanted. Beyond the postoperative period and itscomplications, the life expectancy of these children isalmost normal (97% 10-year survival).68,69

Children with TGA do not survive into adulthood withouturgical correction, but, when grown up, they do not haveormal hemodynamic status. After atrial switch (Mustard orenning procedures), tricuspid regurgitation and progressiveV dilation and dysfunction are common because the RV takesver the systemic work. The LV is small and squashed by theV because of septum encroachment on the LV cavity. Aynamic LVOT obstruction, most frequently encountered byatients with intact septum, may occur progressively with ad-ancing age because of the low-resistance PA bed being con-ected to an anatomic LV chamber. LV dilation and failureay supervene in cases of increased pulmonary vascular resis-

ance. A restriction to systemic or pulmonary venous returnecause of baffle obstruction may supervene. Atrial tachyar-hythmias and AV blocks are present in more than half of theatients after 10 years and are very resistant to pharmacologicreatment.70

After arterial switch (Jatene operation), the hemodynamichysiology is close to normal. However, 2 complications dom-nate the long-term survival: regurgitation of the neoaorticalve (25% of the cases) and myocardial ischemia caused byoronary ostial lesions.71,72 RVOT or LVOT obstruction, pos-ible residual shunt, and LV dysfunction might also be found.73

hese patients are particularly sensitive to hypovolemia and-stimulation.74

If an atrioventricular discordance is added to the ventriculo-rterial discordance, the circulation is physiologically correctedecause both the great arteries and the ventricles are inverted;ystemic and pulmonary circulation is in continuity. The bloodows from the RA to the LV and then to the PA and to the

ungs; it returns to the LA and then to the RV and to the aorta.he great arteries are positioned like in TGA and run parallel

Fig 15). The position of the infundibulum is such that the aortarises anteriorly and to the left of the PA. The anatomic RV isn a left-sided position, and the anatomic LV stays on the rightide of the heart. Nearly 80% of the patients have a perimem-ranous VSD, 50% mitral abnormalities, and 30% tricuspidegurgitation.75 The tricuspid valve might be abnormally dis-laced toward the apex of the ventricle (Ebstein-like anomaly).

pulmonary outflow tract obstruction is frequent because it

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ies in an oblique position because of the malalignment of thenterventricular septum.

These patients become symptomatic after adolescence; theyre not cyanotic, but their systemic ventricle is an anatomicallyight ventricle; therefore, it dilates and fails in about 20 to 25ears because of pressure overload.76 The diagnosis is fre-uently overlooked until the signs of progressive ventricularailure appear. AV blocks are frequent.77

The prevalence of coarctation of the aorta is 0.2% of births.78

ridge of dense tissue narrows the lumen of the aorta at theevel of the isthmus. The aortic arch above the lesion is dilatednd highly pulsatile, whereas the descending aorta distal to theoarctation is much less expansive in systole. Aortic coarcta-ion is frequently associated with bicuspid aortic valve (50% ofhe cases) and ductus arteriosus (20% of the cases). The pres-ure overload on the LV induces a concentric hypertrophy; theystolic function is preserved, but diastolic dysfunction issual. The disease is characterized by arterial hypertension inhe arms and hypotension in inferior limbs; the gradient ofressure is at least 20 mmHg. There is a 3/6 systolic murmurudible on the left midthoracic area in the back. If the coarc-ation is very narrowed, an important collateral network main-ains the distal perfusion through periscapular, internal mam-ary, cervical, and intercostal arteries. Dilation and increased

ulsatility of intercostal arteries cause a notching at the inferiordge of the ribs, which is typical on chest x-rays. The majorityf nonoperated patients die between 30 and 40 years from LVailure, endocarditis, aortic rupture, or cerebral hemorrhage.78

The operative risk is inversely proportional to the length ofhe stenosis and to the degree of collateralization. When col-aterals are well developed, clamping of the aorta does notncrease upstream pressure more than 25 to 30 mmHg.79 Usu-lly, the stenosis is short, and the resected area is small; theurgeon can perform a direct end-to-end anastomosis. A patchith the left subclavian artery or with prosthetic material is

ometimes necessary to prevent a residual stenosis. The clamp-ng time is 15 to 30 minutes. Meanwhile, the downstreamressure must be kept �60 mmHg with an arterial vasocon-trictor (phenylephrine and norepinephrine). The operation iserformed through a left thoracotomy and requires a double-umen endotracheal tube for exclusion of the left lung. After therocedure, the immediate decrease of the diastolic arterialressure is a good marker of the absence of significant residualradient. Postoperative paraplegia because of medullary isch-mia occurs in 0.1% to 0.4% of the patients.80

Postoperative hypertension is frequent and peaks at 12 to 24ours because of an acute firing of the baroreceptors adjusted tohe previous high upstream pressure; there is a second peakfter 2 to 3 days linked to an excessive level of renin andngiotensin. Hypertension persists in 20% to 50% of the pa-ients.81 In the first postoperative days, a mesenteric arteritis isrequent because of sympathetic hyperactivity; it produces se-ere abdominal pain.82 Even if the lesion has been corrected inhildhood, it may recur in the young adult.83 Systemic hyper-ension and left ventricular hypertrophy may persist after cor-ection in up to one third of the cases.

Many arterial anomalies can happen at the level of the aorticrch including hypoplasia, ductus arteriosus, aortopulmonary
stulae, and coronary anomalies. A small ductus arteriosus may p
ersist in an adult; it is responsible for an L-to-R shunt, whichay induce an LV dilatation and failure. It is diagnosed by a

/6 continuous or systolic crescendo murmur in the upper leftternal edge, radiating to the back. At echocardiography, theres a persistent diastolic turbulent flow in the distal main pul-onary artery. Patent ductus arteriosus should be closed unless

t is tiny. In case of a large patent ductus arteriosus withradient �60 mmHg between the aorta and pulmonary artery,he closure is recommended only if the associated PHT iseversible.

Coronary arteries may present anomalous origin from differ-nt aortic coronary sinuses or from a single common trunk orrom the pulmonary artery. They may also present anomalousermination into the RV or the PA. This situation leads to astula or a shunt between LV and RV. The effective shuntQp/Qs) is usually around 1.6.84 A biventricular dysfunction isrequently present.

GENERAL ISSUES IN ANESTHESIA

Adolescent or adult patients suffering from CHD share manyommon features.85,86 They present 5 major independent riskactors: PHT, cyanosis, reoperation, arrhythmias, and ventric-lar dysfunction. RV and/or LV failure is linked to the etiologynd to the degree of remodeling; it is directly associated withhe age and with the degree of cyanosis. Their average opera-ive mortality of 7% is higher than the mortality of childrenith similar pathologies. They show an elevated incidence of

rrhythmias (�50%) compared with children (15%). The pul-onary circulation is usually abnormal; there is PHT because

f volume and/or pressure overload, or the flow is limited andnsufficient at exercise. They suffer from the systemic effects ofypoxia and increased hematocrit on peripheral organs: theidneys, the lungs, the brain, and the hematologic system showigns of more or less severe dysfunction. Finally, they haveoagulation disorders and an increased hemorrhagic riskTable 1).

The flow through a shunt is proportional to the diameter ofhe defect or the conduit and to the ratio of the impedancesetween the upstream and the downstream cavities. An R-to-Lhunt (TOF, TGA, VSD, and pulmonary atresia) increaseshen systemic arterial resistances decrease or pulmonary re-

istances increase (Table 2). The SpO2 varies with the Qp/Qsatio and monitors very precisely the degree of mixing ofrterial and venous blood. Increasing the inspired oxygen con-entration has minimal effect on cyanosis because of an R-to-Lhunt, whereas arterial vasoconstriction (phenylephrine andorepinephrine) increases arterial oxygen saturation. The pres-nce of a shunt modifies the speed of induction of intravenousnd inhaled agents.87,88

An L-to-R shunt (ASD, VSD, and AV canal) decreases withdrop in SVR (Table 3). The major problem is usually the

resence of PHT. An elevated SpO2 is no guarantee that oxygenransport is satisfactory; with a Qp/Qs �3/1, the oxygen trans-ort decreases even if the arterial blood is correctly oxygen-ted.89 The purpose of a peripheral L-to-R shunt (Blalock-aussig, Waterston, and aortopulmonary collateralization) is toake up for insufficient pulmonary flow; because its dimension

s fixed, its output is proportional to the systemic arterial
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ension). The SpO2 decreases with the SVR; arterial vasocon-triction (phenylephrine and norepinephrine) increases arterialxygen saturation. These patients show a chronic low diastolicressure that might endanger coronary perfusion.Cyanosis is caused by an insufficient pulmonary flow (pul-onary atresia) or a contamination of arterial blood by venous

lood (R-to-L shunt). In an R-to-L shunt, the blood bypassinghe lungs corresponds to an increase in nonoxygenated blood inhe lungs (deadspace effect). In this case, the measured end-idal carbon dioxide (PETCO2) underestimates the actualaCO2. These patients maintain a normal ventilatory response

o hypercapnia but show a blunted response to hypoxemia.90

hey hyperventilate chronically to compensate for the poorO2 disposal. Because of the low arterial oxygen saturation, theematocrit increases up to 65% to 70% to match the oxygenransport. The hyperviscosity of a high hematocrit increases theardiac ejection work and the risk of spontaneous thromboses,articularly when these patients are fasting or dehydrated. Theyanosis of these patients is less obvious when they are anemicecause the degree of cyanosis depends on the concentration ofnsaturated hemoglobin.

Table 1. Risk Factors in Anesthesia in Case of Adult Congenital

Heart Disease

Pulmonary hypertension (PVR �500 dynes/s/cm5)Cyanosis (SaO2 �85%, Hb �150 g/L, Ht �55%)ReoperationArrhythmias (�50% of the cases)Ventricular dysfunction

Table 2. Congenital Heart Diseases With R-to-L Shunt and

Decreased Pulmonary Flow (Qp +)

Pathologies: single ventricle (univentricular AV connection,tricuspid atresia), tetralogy of Fallot, palliated Fallot,transposition of great arteries, pulmonary atresia

Cyanosis present, increased hematocrit (Ht �55%).To reduce the shunt and increase SaO2: * SVR, + PVR.Qp/Qs monitoring: SpO2

PETCO2 underestimates PaCO2

Anesthesia management aims at:SVR increase* (�1-stimulation); neuraxial blockade poorly

toleratedPVR decrease;* general anesthesia with normobaric

hyperventilation (hypocarbia, alcalosis) is preferedKeep Ht �40% in patients with important R-to-L shunt or

decreased pulmonary flow (SaO2 �75%)After Fontan procedures: spontaneous ventilation as possibleDecrease dynamic RV outflow tract obstruction by �-blockerTo increase SaO2: * SVR (�1-stimulation); * FIO2 without effect;

ideal SaO2: 80%-85%Induction with IV substances is accelerated; halogenated gas

uptake is slowed

Abbreviations: SVR, systemic vascular resistances; PVR, pulmonaryascular resistances; SaO2, arterial oxygen saturation; SpO2, pulseximetry; Qp, pulmonary blood flow; Qs, systemic blood flow;ETCO2, end-tidal carbon dioxide.*In case of noncorrected transposition of great arteries or single

entricle (rare in adults), Qp should be balanced with Qs (1:1) by an
ldequate balance of SVR and PVR.
Cyanotic diseases have the following effects on organs andystems:

1. Effects on myocardium: chronic systolic and diastolicventricular dysfunction and increased ischemic risk.63

2. Hematologic effects: increase of the mass and stiffnessof erythrocytes, hyperviscosity, and gallstones becauseof the excess of heme ring.91

3. Effects on coagulation: decrease in von Willebrandfactor and platelet function and increased fibrinolysis;92

the measured thrombocytopenia is usually artifactualbecause of the increase in erythrocyte volume. Whenhematocrit is above 55%, the values of PT and PTT aremisleading because of the relative excess of citrate inthe samples compared with the reduced plasma vol-ume93; false hypoglycemia might also occur because ofthe glycolysis from the excess number of erythrocytes.

4. Effects on kidneys: the chronic hypoxemia leads toproliferative lesions in the glomeruli and to a thicken-ing of the basal membrane, resulting in proteinuria anduric acid increase; the level of uric acid in the plasmais a good marker of the renal hemodynamics of cya-notic patients.94

5. Neurologic effects: in adults with R-to-L shunts, theincidence of cerebral abscesses is increased but not theincidence of cerebral vascular accidents.5,95,96

Fifty percent of the emergency hospitalizations are justifiedy threatening arrhythmias.97 Atrial tachyarrhythmias are fre-uent after correction of an ASD, Senning procedure, or whentria are enlarged; AV blocks are usual after surgery at the levelf the AV junction (perimembranous VSD). Ventricular tachy-ardia may supervene in any congenital disease but is particu-

Table 3. Congenital Heart Diseases With L-to-R Shunt and

Increased Pulmonary Flow (Qp *)

Pathologies: atrial septal defect (ASD), atrioventricular canal (AVcanal), ventricular septal defect (VSD), anomalous venousconnections, ductus arteriosus.

Cyanosis absent, normal hematocritIncreased pulmonary flow

ASD: RV volume overloadVSD: LV volume overload

Longstanding L-to-R shunt (VSD) leads to pulmonary hypertensionand RV pressure overload.

To decrease the shunt: + SVR, * PVR.Anesthesia management aims at

SVR decrease (isoflurane, intrathecal or epidural anesthesia)PVR increase (hypoventilation, slight hypercarbia, FIO2 0.3)Preload increase; hypovolemia poorly tolerated because of

volume sequestration in pulmonary bedInduction with intravenous substances is slowed; halogenated gas

uptake is increasedPalliative shunts (Blalock-Taussig) or aortopulmonary collaterals

Flow through the shunt is fixed and proportional to systemicblood pressure

SVR decrease leads to decrease in pulmonary flowHypotension leads to low SpO2

Abbreviations: SVR, systemic vascular resistances; PVR, pulmonaryascular resistances; SpO2, pulse oximetry; Qp, pulmonary blood flow.

arly frequent in corrected TOF with pulmonary valve insuffi-

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iency and RV dilatation.4,98 The incidence of sudden death isncreased in CHD patients.

The criterion of PHT is a mean pulmonary arterial pressure25 mmHg at rest and �30 mmHg during exercise or PVR300 dynes/s/cm5. The most frequent origin is a nonrestrictive-to-R shunt with a pressure overload; it already appears dur-

ng childhood in 50% of the patients with VSD or AV canal butn only 10% of the adult patients with an ASD.99 The hyper-ension is associated with a progressive muscular hypertrophyf the media layers of the arteries; it leads to a hypertension thats at first reactive for many years but becomes secondarilyxed. This last stage is the Eisenmenger syndrome, character-

zed by PVR �800 dynes/s/cm5. The flow through an L-to-Rhunt becomes first bidirectional and then reversed; it is theain origin of an outbreak of cyanosis in adults.34 The survival

f patients with severe PHT is more favorable in adults suffer-ng from CHD than from primary PHT.100,101

The limited compliance of the pulmonary vascular systemrecludes the possibility of adapting to blood volume varia-ions. A decrease in arterial pressure because of hypovolemia orasodilation worsens the R-to-L component of the shunt. Asong as the pulmonary arterial pressure is not fixed, it overre-cts to sympathetic stimuli and to variations in pulmonary H�

oncentration; hypothermia, stress, pain, acidosis, hypercarbia,nd hypoxia will aggravate the hypertension.102

Ventilating these patients is a compromise between activeyperventilation and the preservation of a low intrathoracicressure. The ideal tidal volume corresponds to the functionalesidual capacity103 (Fig 16). It is necessary to manipulate theespiratory rate, the tidal volume, and the ventilatory mode

VR

Pulmonary volume

Alveolar vessels

FRC

Extra-alveolar vessels

Total PVR

Fig 16. PVR and ventilation. At low tidal volume, hypercarbia and

telectasis increase vascular resistances in small alveolar vessels. AT

igh tidal volume (hyperinflation), large extra-alveolar vessels are

echanically compressed. Total PVR presents a “U”-shaped curve,

ith its lowest value at the functional residual capacity (FRC).

volume- or pressure-controlled ventilation) to obtain the low-

st PaCO2 with the lowest mean intrathoracic pressure, remem-ering that the duration of inspiration raises the mean intratho-acic pressure more than the peak value of the inspiratoryressure.104 Fearing the interferences of the IPPV, the anesthe-iologist frequently prefers an intrathecal or epidural blockadeo general anesthesia. This might not be a good choice for 3easons. First, the incremental pressure of IPPV represents aeak increase compared with the already elevated pulmonaryressure and the RV is well adapted to a high afterload; second,PPV allows hyperventilation and therefore a decrease in PVR;nd third, the arterial vasodilation secondary to neuraxiallockade will worsen the R-to-L component of the shunt andggravate the cyanosis of the patient. The tolerance to IPPV cane tested before induction by the observation of the arterialressure tracing during a Valsalva maneuver; if the ventilatoryressure variations are stabilized and if the mean arterial pres-ure decreases �20% of its baseline value, IPPV can be startedithout fear. The inhaled and intravenous anesthetic agentsave minimal effects on pulmonary circulation, except foretamine, nitrous oxide, and desflurane, which increaseAP.103,105 Ketamine is useful in all sick children with CHD but

s poorly adapted to the hemodynamic conditions of adultsecause it increases PVR and myocardial oxygen consump-ion.104

The therapeutic measures to treat PHT are described in Table. Except for hyperventilation, inhaled prostanoids, and NO, allhe substances used for reducing PHT have also some systemicasodilating effect. The arterial vasodilators (phentolamine,itroprusside) are useless for the treatment of congenital PHTecause they aggravate the R-to-L shunt and because the pul-onary vascular tree is almost devoid of �1 receptors.106 In the

perating room or intensive care unit, the nonclosure or theeopening of the pericardium and sternum after cardiac surgerymproves RV function and decreases ventricular interdepen-ence.Poor ventricular performance is frequent among adult pa-

ients suffering from CHD. This is secondary to the followinghenomena67:

1. Ventricular remodeling: concentric hypertrophy be-cause of pressure overload and spherical dilatation of asingle ventricle, for example; the LV ejection fractioncalculation is not valid when the shape of the ventricleis abnormal, and the dimensions in systole and diastoleare more adequate criteria. For the LV, the upper limitof short-axis diameter is 2.5 cm/m2 in end-systole and4.5 cm/m2 in end-diastole.

2. Duration without correction: the longer the period be-fore surgical correction, the more extensive the ven-tricular lesions.

3. Type of overload: a volume overload is better toleratedthan a pressure overload; the RV fails when the PAP ischronically above 50 mmHg.

4. Cyanosis: the myocardial oxygen consumption is be-yond the possibility of the oxygen transport at exertion,and the myocardium is chronically ischemic.

5. Ischemia: microvascular occlusions because of hyper-viscosity and low perfusion pressure because of a shuntinduce chronic myocardial ischemia; coronary athero-
matous disease may be superimposed in the adult.

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In the perioperative management of patients with CHD, theriority for the anesthesiologist is a correct understanding ofhe peculiar pathophysiology of his/her patient, as it appears inhe data of the echocardiography, the catheterization, and theecords of previous surgery. There is no general recipe; thehoice of the anesthesia technique is adapted to the hemody-amic peculiarities of every single case. In patients with an-to-L shunt or low pulmonary flow, it is particularly important

o know the SpO2 at room air without any medication.Hypoventilation and hypoxia have catastrophic effects on

atients with low pulmonary flow or an R-to-L shunt; therefore,light premedication without opiates is recommended before

peration. With the preoperative fasting, cyanotic patients withigh hematocrit may be in danger of dehydration, low periph-ral flow, acidosis, and thrombosis; these patients benefit fromn intravenous perfusion.

Previous surgical procedures modify the possible accesses forquipment and monitoring. For example, the blood pressure is notbtainable on the side of a Blalock-Taussig shunt; there is noccess to the RA or RV after a Fontan procedure. In patients withhunts, drastic precautions must be taken to prevent any accidentalnjection of air bubbles through venous lines; paradoxic emboli are

constant threat in patients with R-to-L shunts or during theeversal phases of the flow through an L-to-R shunt (Fig 5B).

The pulse oximeter is the single most important monitoringevice in CHD. It gives information on the effective pulmonaryow, the Qp/Qs ratio, and the reciprocal variations of pulmo-ary and systemic vascular resistances. However, it is of noelp in the case of a large L-to-R shunt (Qp/Qs �3:1). Whenhere is an R-to-L shunt, the PETCO2 underestimates the realaCO2 because of the deadspace effect of the blood volumevoiding the lungs.107 An invasive monitor of arterial pressure

Table 4. Summary of the Treatment of Pulmonary Hypertension

Deepening anesthesia (fentanyl, sufentanil), rewarming the patientAcute treatment without vasodilating effect on SVR

Hyperventilation: PaCO2 25-30 mmHg, pH �7.45Hyperoxia: FIO2 1.0Low intrathoracic pressure (mean: 6-10 mmHg)NO: 10-50 ppm in inspiratory limbAerosolized prostanoid (iloprost 20 �g over 15 min)Magnesium sulfate (5-10 mmol)

Treatment of RV failure�-Stimulating drugs: dobutamine, isoprenalineAnti-phosphodiesterases-3: milrinone (0.5 �g/kg/min)Norepinephrine (* coronary perfusion pressure)Adaptation of RV preload (according to CVP)Opening/nonclosure of pericardium and sternumIABP (* coronary perfusion pressure)

Acute and chronic treatment with vasodilating effect on SVREpoprostenol perfusion (2-5 ng/kg/min)Adenosine (6-12 mg intravenous)Antiphosphodiesterases-5: dipyridamole (0.5 mg/kg intravenous)

sildenafil (50-100 mg/d per os) � L-arginineAntiendothelin: bosentan (125-250 mg/d per os)Antiangiotensin: losartan (50-100 mg/d per os)Calcium entry blockers (nifedipine, 15% responders)

Abbreviations: SVR, systemic vascular resistances; CVP, centralenous pressure; IABP, intra-aortic balloon pump.

s the second most important device. It gives immediate data on t

VR, volemia, and systolic function and allows frequent arte-ial blood gases analyses. The usual central venous access maye problematic because of anatomic abnormality (left SVC) orrevious surgical reconstruction. After a Fontan procedure, forxample, the CVP measures the pulmonary arterial inflowressure (which must be at least 15 mmHg).The pulmonary artery catheter is not of paramount impor-

ance in CHD patients because filling pressures are not repre-entative of actual volemia or ventricular function in theseighly abnormal hemodynamics. The variations of pulmonaryow are better transcribed by the pulse oximetry. The place-ent of the pulmonary catheter is frequently problematic or

ven impossible, such as in cases of R-to-L shunt (risk of beingouted in LA) or tight pulmonary stenosis, and after Fontanrocedures or atrial switch (Mustard or Senning operations).oreover, the presence of a shunt or a tricuspid regurgitation

iases the calculations of cardiac output and derived data.ransesophageal echocardiography is certainly a more usefulonitor because it provides multiple information on anatomy,

emodeling, pathophysiology, volume status, and ventricularunction (see later). If necessary, catheters can be introducedurgically into the pulmonary artery or the left atrium duringardiac procedures.

The choice of the anesthetic agents is governed by theiremodynamic consequences including decrease in preload andystemic afterload (propofol, midazolam), sympathetic stimu-ation (ketamine), tachycardia, and negative inotropic effectthiopental); the least disturbing induction agent is etomidate.soflurane has arterial vasodilating properties useful to decreasehe SVR. Desflurane abruptly raises the PVR when the inspiredoncentration is increased;105 this might have dramatic conse-uences in patients with augmented pulmonary flow. The pres-nce of a shunt modifies the speed of induction of intravenousnd inhaled agents. The direct passage of blood from theenous return to the arterial tree in an R-to-L shunt speeds uphe effects of intravenous agents, whereas uptake and release ofnhaled agents are slowed because the pulmonary flow is min-mal.87 An L-to-R shunt has opposite but limited consequences;he induction of anesthesia with intravenous agents is mini-ally slowed, and the effect on uptake and release of inhaled

gents is negligible in clinical practice.88 Maintaining normo-hermia is mandatory as long as the patients are not on cardio-ulmonary bypass; hypothermia may induce pulmonary vaso-onstriction and coagulation disorders. It is an independentredictor of postoperative cardiac complications.108

It is frequently thought by clinical practitioners that regionalnesthesia is less disturbing than general anesthesia. This is cer-ainly true for peripheral limb blocks; however, epidural andntrathecal anesthesia have 3 major drawbacks: they induce arte-ial vasodilation, reduce preload, and do not allow hyperventila-ion. By decreasing the SVR, neuraxial blockade aggravates an-to-L shunt and a bidirectional shunt with PHT; it diminishes theow through a palliative aortopulmonary shunt.5 On the contrary,

t is helpful to decrease the effects of an L-to-R shunt (ASD andSD). High thoracic epidural anesthesia (C7-T6 level) induces

ardiac sympatholysis but does not decrease PHT because humanulmonary vessels are poorly innervated and lack �1-recep-ors.102,106 General anesthesia with controlled ventilation affords
he advantage of hyperventilating the patient and reducing the

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HT; this outweighs the risk of increasing the RV afterloadecause the PVR is already so elevated that the increase in in-rathoracic pressure has only minimal effect on the impedance tojection of the hypertrophied RV.

Major hemodynamic modifications happen in pregnantomen including increases in blood volume (�50%), cardiacutput (�30%-40%), and heart rate (�30%), and a decrease inVR.109 The pulmonary flow tends to be overloaded in cases of-to-R shunt, whereas an R-to-L shunt increases its flow and

he patient becomes more cyanotic. In the presence of PHT, theaternal death rate varies from 30% to 70% according to the

athology. Most deaths occur between 2 and 9 days postpar-um.110 In aortic coarctation, the increase in cardiac output is

ediated exclusively by an increase in heart rate because theystolic volume is restricted by the stenosis. The rate of cardiacomplications including ventricular failure, thrombosis, ar-hythmia, endocarditis, and aortic dissection during pregnancys 32%.111 Cyanosis is an important prognostic factor; in cya-otic women, the mortality and morbidity rates are close to0%, whereas it is only 15% in noncyanotic patients.112 AnpO2 �85%, an Hb level �15 g/dL, and PHT before preg-ancy are independent prognostic factors of increased cardiacisk.

The recommendations for anesthesia in the most commoniseases can be summarized as follows110,113,114:1. L-to-R shunt (ASD and VSD): avoid increase in SVR;

intrathecal and epidural blockade are indicated for vag-inal delivery and cesarean section.

2. Corrective or palliative L-to-R shunt (Blalock-Taussig,Waterston): avoid decrease in SVR, general anesthesiais recommended, and epidural anesthesia is possible ifthe sympathetic blockade is very progressive.

3. Coarctation of the aorta: maintain tachycardia andSVR, general anesthesia is preferred, and intrathecalanesthesia is contraindicated.

4. TOF: general anesthesia is preferred to neuraxialblockade, although epidural anesthesia is possible if thesympathetic blockade is very progressive; esmolol andphenylephrine should be used in case of hypoxic crisis;intrathecal anesthesia is contraindicated.

5. Eisenmenger syndrome, R-to-L shunt: general anesthesiawith normobaric hyperventilation, epidural anesthesia isexceptionally possible if the sympathetic blockade is veryprogressive and if the preload of the RV is well pre-served,115 and intrathecal anesthesia is contraindicated.

Patients with CHD are at increased risk of endocarditis andhould benefit from an appropriate prophylactic antibiotic forvery procedure with a probability of bacterial contamination,uch as surgical operations, dental treatment, and endoscopicrocedures. The risks of endocarditis differ according to theemodynamic characteristics of the disease. They are highest inatients with prosthetic material and in those with high-velocityets such as in VSD, TOF, AV canal, and valvular stenosis.hey are lower when the pathology produces low-velocitybnormal flows (ASD and PFO), when a VSD is spontaneouslyccluded, or after surgical correction with a patch of autologousericardium.In the management of adult CHD, TEE is useful for the

nesthesiologist in 3 circumstances116:

1. For hemodynamic monitoring during cardiac and non-cardiac surgery; because of the complex anatomy andpathophysiology of these cases, the classic measure-ments of pressure must be completed by an echocar-diographic study to correctly understand the peculiarhemodynamic behavior of these patients and evaluatetheir volume status and ventricular function.

2. For the diagnosis of unexpected intraoperative find-ings, which happen in 2% to 7% of cases.

3. For evaluation of the surgical corrections during car-diac surgery; immediate revision is prompted by TEEin up to 7% of the cases.

Although most of the outcome studies in CHD patients areirected at pediatric patients, it seems reasonable to assume thatheir conclusions are valid for adult surgery.73 In cardiac sur-ery, unexpected findings of the prebypass examination lead toodifications in surgical plans in up to 7% of cases.116,117 After

ypass, the rate of immediate surgical revision induced byostbypass TEE examination is 3% to 7%.118,119 Procedureshat benefit most are valvular repairs, outflow tract reconstruc-ions, and complex AV discordances.120 The sensitivity andpecificity of TEE to determine the necessity of reoperationeach 89% and 100%, respectively.64,65 Postbypass TEE mightlso reveal residual defects without clinical significance in 15%o 38%.117,119 Patients who leave the operating room withignificant residual defects tend to have a poorer outcome thanhose with immediate complete correction.118,119 Postbypassentricular performance is in itself an important predictor ofurvival; patients with severely diminished ventricular functionave a greater incidence of early death than those with pre-erved function (35% v 4%, respectively).121

The rates of new diagnoses (2%-7%) and immediate revision3%-7%) in cardiac surgery plead for a routine use of TEE inHD because unexpected findings cannot be foreseen in someategories of patients. In complex cases, routine TEE results inignificant decreases in hospital costs because it decreases theate of residual defects and therefore the rate of delayed reop-ration.122,123 Moreover, the perioperative hemodynamic man-gement of ventricular function and volemia are more accurateith TEE, which has a “medical” impact that reaches 25% in

ardiac as well as in noncardiac surgery.117,120 However, insti-utional surgical abilities and echocardiographic experienceirectly affect the clinical impact; the rate of return to bypassor incomplete repair decreases from 9.6% to 0% when anxperienced perioperative echocardiographer is replaced by annsufficiently trained anesthesiologist, and the rate of theissed residual problems after bypass increases from 21% to

4%.124

As already mentioned, TEE offers the opportunity to nonin-asively evaluate intracavitary pressures with the simplifiedernoulli equation (�P � 4 Vmax

2). The systolic pulmonaryrtery pressure (PAP) can be easily calculated if the patient hastricuspid regurgitation, which is usually the case when PHT

s present. In the absence of pathology in the RVOT or theulmonary valve, the systolic RV pressure is identical to theAPs, which equal the sum of the systolic pressure gradient�P) between the RV and RA and the right atrial pressureRAP): PAPs � 4 (Vmax

2)TR � RAP.
This assumption is incorrect in the presence of a nonrestric-

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ive VSD because the upstream pressure is then the left ven-ricular pressure. However, the Vmax of the LV-to-RV shunt isroportional to the pressure difference between both ventriclesn systole. If the aortic valve and aorta are normal, the systemicrterial pressure is roughly equivalent to the LV maximalressure. The right ventricular systolic pressure can be calcu-ated by the difference between systolic arterial pressure andhe pressure difference across the shunt (�P): right ventricularystolic pressure � systolic arterial pressure � 4 (Vmax

2)VSD.If pulmonary regurgitation is present, the pulmonary dia-

tolic pressure can be estimated by the pressure gradienthrough the pulmonary valve in diastole. If the RV diastolicressure is equal to the RAP, the formula becomes: pulmonaryiastolic pressure � 4 (Vmax

2)PR � RAP.

CONCLUSIONS

The perioperative management of adult patients with CHD ischallenge for the anesthesiologist. The wide variety of pa-

hologies, the different surgical palliations or corrections, and d

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he long evolution of the diseases make every case unique. Theriority for the anesthesiologist in charge is a thorough under-tanding of the pathophysiology and hemodynamics of eachatient. Even if these cases are not frequent, their number isuch that every anesthesiologist might encounter them in his/er practice, particularly for noncardiac surgery. Because thenowledge of CHD is mainly concentrated in hospitals that runprogram of congenital cardiac surgery, adult CHD patients

re best followed up in dedicated tertiary centers that have aultidisciplinary expertise in these particular patients.125,126

oderate-to-severe cases should be transferred to these spe-ialized teams even for noncardiac surgery. However, this maye difficult when the operation is urgent. A patient with CHDight need an emergency procedure because of a car accident

r an acute surgical/obstetric crisis precluding transportation.herefore, basic knowledge of CHD is of paramount impor-

ance for every anesthesiologist because he/she is in the crucialosition to manage the acute care of these patients and to
ecide who and when to transfer to the tertiary hospital.
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