Echocardiographic Follow-up of Grown-ups with Congenital Heart Disease: Update 2013

ECHOCARDIOGRAPHY (T BUCK, SECTION EDITOR)

Echocardiographic Follow-up of Grown-ups with CongenitalHeart Disease: Update 2013

Doreen DeFaria Yeh & Mary Etta King

Published online: 30 October 2013# Springer Science+Business Media New York 2013

Abstract There is a burgeoning population of adults withcongenital heart disease (CHD) due to successful medical andsurgical intervention in childhood. This group of patientsrequires careful echocardiographic evaluation and follow-up ofthe residua and sequellae of their cardiac anomalies. This reviewaddresses the guidelines for evaluation and management of thegrown-up with CHD, the contributions of 3D echo in theirassessment, and the current status and new developments innoninvasive determination of ventricular volumes and function.The contribution of stress and contrast echocardiography for theadult with CHD is considered. The application of transcatheterinterventions for this population is included with particularregard for the role of echocardiography pre- and postprocedure.Particular problems presented by pregnancy for the adult CHDpatient are also reviewed.

Keywords Echocardiography . Adult congenital heartdisease . Interventional echocardiography . Stress echo .

Pregnancy and heart disease

Abbreviations2D Two-dimensional3D Three-dimensionalASD Atrial septal defectCHD Congenital heart diseaseCMR Cardiac magnetic resonance imaging

D-TGA D transposition of the great arteriesL-TGA L transposition (or congenitally

corrected) transposition of the great arteriesLV Left ventriclePA Pulmonary arteryPFO Patent foramen ovalePR Pulmonic regurgitationPW Pulsed waveRV Right ventricleTEE Transesophageal echocardiogramTOF Tetralogy of FallotVSD Ventricular septal defect

Introduction

As a result of medical and surgical advancements, there are anincreasing number of adults living with congenital heartdisease (CHD). The prevalence of CHD in the adultpopulation is now estimated at 3.0 per 1000 adults, basedupon data from several cross-sectional population databases[1–4]. This expanding demographic is exemplified in Fig. 1 asreported by Marelli and colleagues [2] from a Canadianadministrative database, with adults representing more than50 % of the cases recorded for each study point in time.Hence, the practicing cardiologist can expect to be called uponwith increasing frequency to evaluate and follow adultswith CHD, either unrepaired or modified by surgery ortranscatheter techniques. Echocardiography remains theprimary diagnostic imaging modality for assessing andfollowing these patients. This review will address theechocardiographic evaluation of the adult patient with CHD,considering the contributions of 3-dimensional (3D)echocardiography, functional assessment, interventionaltechniques for CHD, and specific issues relating to pregnancy.

D. DeFaria YehDivision of Cardiology, Adult Congenital Heart Disease Program,Echocardiography Section, Massachusetts General Hospital,55 Fruit St, Yawkey 5700, Boston, MA 02114, USA

M. E. King (*)Division of Pediatric Cardiology, Cardiac Ultrasound Laboratory,Massachusetts General Hospital for Children, 55 Fruit St,Boston, MA 02114, USAe-mail: [email protected]

Curr Cardiovasc Imaging Rep (2013) 6:454–466DOI 10.1007/s12410-013-9236-y

The American Society of Echocardiography has developedguidelines for appropriate use of echocardiography inevaluating and following patients with cardiac disorders[5••]. Their recommendations for appropriate use ofechocardiography for adult patients with CHD are shown inTable 1. In general, echocardiography is consideredappropriate for initial evaluation of suspected CHD, forfollow-up of known CHD with a change in clinical status orexam, for re-evaluation to guide therapy, and for routinesurveillance (>1 yr) of CHD following incomplete orpalliative repair. With these guidelines in mind, the practicingcardiologist needs to assess specific anatomic and functionalissues for the patient’s underlying congenital heart defect inorder to provide optimal supervision of care and timely

intervention for clinical problems. For a full review of clinicalevaluation and management guidelines, the reader is referredto the ACC/AHA 2008 Guidelines for the Management ofAdults with Congenital Heart Disease [6••], the CanadianCardiovascular Society Guidelines [7], and the EuropeanSociety of Cardiology Guidelines [8].

Three-Dimensional Echocardiography in AdultCongenital Heart Disease

Progress and advances in the realm of adult CHD can bepartially attributed to more accurate understanding of theunderlying anatomy and physiology, which allows optimalclinical management. Much of the recent literature in the fieldhas involved the application of 3D echocardiography forimproved diagnostic detail, functional assessment, and forguidance during surgical procedures. Real time 3Dechocardiography has made 3D echo imaging more readilyaccessible and time efficient, and the superior resolution oftransesophageal imaging provides an exquisite view of certainaspects of cardiac anatomy.

Much attention has been focused on 3D imaging of atrialseptal defects (ASD) [9–11] documenting the ability todetermine defect size, shape, and number, and comparingthese assessments with 2D echo measures, stretched balloondiameters, and intraoperative inspection. The 3D approachreliably detected and classified all ASDs, correlated wellquantitatively with balloon defect size, and was shown to besuperior to 2Dwith respect to irregularly shaped defects and indetection of multiple fenestrations (Fig. 2).

Fig. 1 This figure depicts the increase in the number and proportion ofadults with congenital heart disease between 1985 and 2000 obtainedfrom an administrative database in Quebec, Canada. Modified withpermission from Marelli AJ, et al. Circulation. 2007;115:167

Table 1 Appropriate use criteria for transthoracic echo for adult congenital heart disease

Indication Appropriate use score (1–9)

• Initial evaluation of known or suspected adult congenital heart disease A (9)

• Known adult congenital heart disease with a change in clinical status or cardiac exam A (9)

• Re-evaluation to guide therapy in known adult congenital heart disease A (9)

• Routine surveillance (<2 y) of adult congenital heart disease following complete repair I (3)○ Without a residual structure or hemodynamic abnormality

○ Without a change in clinical status or cardiac exam

• Routine surveillance (≥2 y) of adult congenital heart disease following complete repair U (6)○ Without a residual structure or hemodynamic abnormality


• Routine surveillance (<1 y) of adult congenital heart disease following incomplete or palliative repair U (5)○ Without a residual structure or hemodynamic abnormality


• Routine surveillance (≥1 y) of adult congenital heart disease following incomplete or palliative repair A (8)○ Without a residual structure or hemodynamic abnormality


A appropriate, I inappropriate, U uncertain, score 7–9 generally appropriate, score 4–6 may be appropriate, score 1–3 generally inappropriate

Modified with permission from Douglas et al. J Am Soc Echocardiogr. 2011;24–238

Curr Cardiovasc Imaging Rep (2013) 6:454–466 455

Transthoracic real-time 3D echo has also been applied tothe detection and sizing of ventricular septal defects [12] withsuccessful detection and localization in 88 % of cases, andgood correlation of defect size with surgical measures.Problems remain with respect to motion artifacts, spatialresolution and limited field of view with the real-time 3Dprobe, requiring full-volume data acquisition and off-linereconstruction.

Another particularly helpful application of 3D echo has beenin the accurate visualization of atrioventricular valve anatomyand function in patients with atrioventricular septal defects. The

complexity of valve anatomy, chordal attachments, andlocalization of mechanism of valvular regurgitation are betterdetermined with the 3D technique than is possible with 2D echo(Fig. 3) [13, 14].

Real-time 3D echo has also proven to be a powerful new toolfor guidance of selected percutaneous catheter-based proceduresas will be discussed later in this review [15]. Advantages of 3DTEE include the ability to visualize the entire length ofintracardiac catheters, stents, and balloons and determine theirposition relative to other cardiac structures, and the unique abilityto demonstrate structures in an “en face” view improving visualunderstanding of intracardiac relationships for the operator.

Another active application of 3D echo involves rightventricular (RV) and left ventricular (LV) volumetric assessmentas correlated with cardiac magnetic resonance imaging (CMR)volumetric assessment [16, 17] for long term monitoring ofventricular size and function to determine timing of intervention.For example, among patients with tetralogy of Fallot (TOF),pulmonic regurgitation (PR) is a common complication postcomplete repair, and timing for pulmonic valve replacementdepends on exercise capacity and RV size and function. CMRhas been the gold standard for assessment of RV size andfunction for this indication, however, recent literature suggests3D echocardiography may rival CMR for serial RV monitoring[17]. Three-D echocardiography provides more accurateassessment of RV volumes compared with 2D echo [18••],and closely correlates with CMR derived RV volumes in bothchildren and adults with CHD [15–17].

Ventricular Assessment: Size, Function,and Hemodynamics

Although surgical repairs of simple and complex congenitalheart lesions have been quite successful, there are frequentlyresidual anatomic and hemodynamic issues, whichcompromise long term survival and quality of life for the adult

Table 2 Summary of reference limits for recommended measures ofright heart structure and function

Variable Unit Abnormal

Chamber dimensions

RV basal diameter cm >4.2

RV subcostal wall thickness cm >0.5

RVOT PSAX distal diameter cm >2.7

RVOT PLAX proximal diameter cm >3.3

RA major dimension cm >5.3

RA minor dimension cm >4.4

RA end-systolic area cm2 >18

Systolic function

TAPSE cm <1.5

Pulsed Doppler peak velocity at the annulus cm/s <10

Pulsed Doppler MPI − >0.40

Tissue Doppler MPI − >0.55

FAC (%) % <35

Diastolic function

E/A ratio − <0.8 or >2.1

E/E ratio − >6

Deceleration time (ms) ms <120

Adapted with permission from Rudski, et al. JASE. 2010;23:685–713

Fig. 2 Transesophageal 3-Dechocardiographic en face imagesof the atrial septum. In Panel A,there is a crescentic defect(arrows) with a catheter passingthrough the defect (star). In PanelB, there is a large secundumdefect but a smaller adjacentfenestration (arrows) is alsodetected

456 Curr Cardiovasc Imaging Rep (2013) 6:454–466

with CHD. In many cases, these issues involve pressure orvolume loading of the left or right ventricle, and accuratenoninvasive assessment of ventricular function has been acontinuing area of study in this patient population.

Assessment of the Systemic Left Ventricle

Congenital heart defects that result in a chronic volume load tothe LV include moderate or large VSDs, PDAs, or congenitalmitral or aortic insufficiency. Lesions that cause LV outflowtract obstruction (discrete subaortic membranes, congenitalaortic stenosis, supravalvular aortic stenosis, and coarctationof the aorta) result in chronic pressure overload, LVhypertrophy, myocardial fibrosis, and diastolic dysfunction.Assessment of systemic LV size, function and wall thicknessfor most CHD patients may be performed in a variety of waysand are similar to those used for acquired heart disease [19]:

(1) 2D linear measures of LV end diastolic and end systolicdimensions; single dimension (modified Quinones)estimation of LV ejection fraction

(2) Volumetric assessment of LV at end systole and enddiastole; may be indexed for body surface area (biplanemethod of discs)

(3) 3D LVejection fraction(4) LV wall thickness (normal <12 mm), LV mass calculation

In addition, the assessment of LV diastolic parameters hasbecome increasingly important in the adult CHD population,and patients who have sustained left heart obstructiveprocesses over years often demonstrate delayed relaxation ormore advanced restrictive diastolic filling patterns [20•].

Finally, LV systolic dysfunction may lead to heart failure,which is a major cause of death among patients with complex

CHD [21]. Cardiac resynchronization therapy has beenevaluated among patients with CHD with promising results,however, efficacy varies widely with the underlying anatomicand pathophysiologic substrate [22]. Recent data demonstratesechocardiographic assessment of cardiac mechanicaldyssynchrony in the CHD patient plays a role in predictingresponse to resynchronization therapy [23, 24].

Assessment of the Subpulmonary Right Ventricle

In many patients with CHD the right heart is most affected.Despite life-saving palliation, the RV may experiencepersistent pressure or volume overload, and there may beintrinsic RV myopathy. It has been demonstrated that patientswith RV enlargement, dysfunction, and fibrosis have greaterexercise limitation and are at risk for RV failure and lethalventricular arrhythmias [25]. There is a trend to recommendearlier re-intervention for correction of tricuspid or pulmonicregurgitation in order to preserve RV function and decreaserisk of chronic right heart failure [26]. Careful quantificationof RV size and function is critical in determining optimaltiming for reducing pulmonic or tricuspid valve dysfunctionby catheter-based intervention or surgical correction.

Traditionally, echocardiographic assessment of RV systolicfunction has been largely qualitative, relying on visualassessment of the longitudinal motion of the tricuspid annulusin the four-chamber view and inward motion of the RV freewall and apex. The RV geometry is more complex than that ofthe LV; thus this qualitative assessment is clearly limited whencompared with quantitative assessment of systolic functionwith CMR [25]. In 2010, guidelines were developed forquantitative echocardiographic assessment of the right heartin adults [18••]. These guidelines provide standards for 2Dvolumetric and nonvolumetric assessment of RV function(Table 2). They have revolutionized the echocardiographer’sability to provide standardized quantitative RV measures tomonitor change over time. The contribution of 3D echo-cardiography to estimates of RV volume and function isdiscussed in this document above.

There are multiple quantitative echocardiographic techniquesto follow RV size and function (Figs. 4 and 5):

(1) 2D linear measures at the base, RVOT, and annulus toapex

(2) 3D volumetric RV assessment (known to have excellentcorrelation with CMR)

(3) Fractional area change (FAC)(4) Myocardial performance Index (MPI)(5) Tissue Doppler Index (TDI)(6) Tricuspid Annular Plane Systolic Excursion (TAPSE)

The reader is referred to the guidelines document fordetailed description of each of these methodologies.

Fig. 3 Parasternal short-axis three-dimensional image from a patientwith a transitional atrioventricular canal defect and a double-orifice mitralvalve with a number marking each orifice


Assessment of the Systemic Right Ventricle and FunctionalUniventricular Heart

Patients with transposition of the great arteries (D-TGA) whohave undergone atrial switch procedure or patients withcongenitally corrected transposition (or L-TGA) have an RVfunctioning as the systemic or subaortic ventricle. In othercases of complex CHD (eg, hypoplastic left heart, congenitalmitral atresia, unbalanced AV canal), the RV is the onlyfunctional ventricle and serves as the systemic pump. Amongthese adults with a systemic RV, ventricular systolicdysfunction is common. Current echocardiographicguidelines for RV quantification do not apply to the systemicRV [18••] and accurate measurement of ventricular functionremains a challenge. In a recent study, echocardiography andCMR were directly compared in assessing function of thesystemic RV in a cohort of adults with D- TGA post atrialswitch. Fractional area change and the rate of systolic RVpressure increase (dP/dt) correlated best with CMR RVEF.Global longitudinal strain based on speckle tracking has alsobeen used to assess early systemic RV dysfunction. Reducedlongitudinal strain of the systemic RV was associated withincreased risk for clinical events [27].

Right Ventricular Diastolic Assessment

Among patients with RV outflow obstruction or pulmonaryvascular disease, right ventricular compliance may bereduced. In Ebstein’s anomaly, the effective RV size may bevery reduced and therefore overall chamber compliance isreduced. Current guidelines suggest evaluating RV diastolicfunction via transtricuspid E/A ratio, E/E’ ratio, and right atrial

size, and these methods have been validated in acquired heartdisease. Another Doppler indicator of poor RV compliance isthe presence of antegrade forward flow into the pulmonaryartery during atrial contraction [28] (Fig. 6). The myocardialperformance index (MPI) is a measure that includes bothsystolic and diastolic assessment of RV function.

Echocardiography with Interventional Proceduresin Adult CHD

Percutaneous approaches have gained increasing acceptanceas a treatment option for patients with uncorrected or palliatedCHD, particularly for those who have undergone multipleprior surgical interventions when redo sternotomy orthoracotomy comes at increased risk.

Currently there are multiple procedures that may be offeredfor CHD patients and echocardiography in many cases iscritical to assessment of structure and function pre-procedure,for guidance during the intervention, as well as monitoring postprocedure for complications.

Device Closure

Percutaneous device closures for shunt lesions includingsecundum ASDs, muscular VSDs, and patent ductus arteriosusare commonly employed. Echocardiography pre-procedure isused to determine shunt location, size and hemodynamicsignificance, shunt flow directionality, ventricular size andfunction, and pulmonary pressure estimation. Shunt fraction(ratio of pulmonary blood flow to systemic blood flow, Qp/Qs) can be estimated echocardiographically utilizing the RV

Fig. 4 Echocardiographicimages in the apex-down formatof a patient post repair ofTetralogy of Fallot to demonstratemethods of quantification of RVsize and function. The upper leftpanels demonstrate right atrialand right ventricular lineardimensions, the upper rightpanels show measurement of RVend diastolic and end systoliccavity areas to compute afractional area change. The lowerpanel demonstrates themeasurement of a 3D volume setto compute a 3-dimensional RVvolume. EF ejection fraction, RAright atrium, RV right ventricle


Fig. 5 Panel A depictscalculation of right ventricularmyocardial performance index(MPI) using tricuspid valveclosure-opening time (TCO) andejection time (ET). (Adapted fromRudski, JASE. 2010) Panel B isan example of tissue Dopplerimaging of the tricuspid annulus.Panel C demonstratesmeasurement (red line) oftricuspid annular plane systolicexcursion (TAPSE)


outflow tract diameter and RV outflow tract velocity timeintegral to estimate pulmonary flow, and the LV outflow tractdiameter and velocity time integral to estimate systemic flow.

For most isolated secundumASDs, percutaneous closure ispreferable to surgical closure. Primum or sinus venosus typeASDs should not be closed percutaneously. Indications forsecundum ASD closure include right heart enlargement,which is generally associated with a shunt fraction (Qp/Qs)

>1.5, arrhythmia, orthodeoxia-platypnea, or history ofparadoxical embolism in the presence of predominantly leftto right shunting and less than moderate pulmonaryhypertension [6••]. Among patients with moderate to severepulmonary hypertension, test balloon occlusion may beperformed to determine changes in hemodynamics andcardiac output prior to consideration of closure. Pre- or peri-procedural transesophageal echocardiography is generallyhelpful for identification of more precise defect size andlocation, estimation of largest diameter, evaluation formultiple intra-atrial defects, and assurance of adequate tissuerim to secure the closure device. Three-Dimensionalechocardiography has gained increasing support for optimalvisualization of defect size and location as well as deviceplacement post deployment (Fig. 7) [10, 11, 27, 28].

Follow-up echocardiographic assessment is required to assureappropriate device positioning, determine presence and degreeof residual shunting, and detect complications such as thrombusformation. Aortic erosion is rare, but device proximity to aorticroot must be assessed following intervention in all cases.

Ventricular septal defects can also be closed percutaneously(Fig. 8) and this may be amanagement option for moderate sizeor multiple muscular VSDs or residual defects after surgicalrepair. Percutaneous closure of perimembranous VSD is morechallenging due to proximity of the aortic and tricuspid valve,as well as the conduction system, with postprocedural completeheart block occasionally seen (Device closure is not yetapproved by the United States Federal Drug Administrationfor perimembranous VSD). Ventricular septal defect closure isgenerally recommended either when LV volume overload

Fig. 6 Continuous wave Doppler spectral tracing across the pulmonaryvalve from a patient with pulmonary stenosis and a hypertrophied,noncompliant right ventricle. Antegrade flow is noted (small arrow)following the A-wave on the electrocardiogram (large arrow) as a signof RV diastolic dysfunction

Fig. 7 Transesophageal echoimages depicting device closureof an atrial septal defect. In panelA, the closure device is present inthe left atrium (arrow). Panel Bshows the left atrial aspect of thedevice apposed to the left septalsurface (arrow). In panel C thedevice is fully deployed on bothsides of the septum resulting intwo parallel linear structures (dualarrows). Panel D is a real-time3D en face image of the atrialseptum with the closure device asviewed from the right atrium. Aoaorta, LA left atrium, RA rightatrium


(unexplained by alternative mechanism) is present, correlatingwith Qp/Qs >1.5, in the presence of multiple recurrences ofotherwise unexplained bacterial endocarditis, or whenaccompanied by progressive aortic regurgitation [6••]. Peri-procedural TEE can guide fluoroscopic assessment of devicelocation, and assess for residual shunt [29, 30].

In adult patients, closure of patent ductus arteriosus ofany size is reasonable to prevent left ventricular volumeoverload and endarteritis. Currently, for adults with apatent ductus arteriosus, the Amplatzer ductal occluder isthe most commonly used device, with coil embolizationused for smaller PDAs measuring <2 to 3 mm or forresidual leaks. Echocardiography post procedure assists

with determining device position, residual shunt, and candetermine improvement in ventricular size and function(Fig. 9).

Valvuloplasty

Percutaneous pulmonary balloon valvuloplasty has become thetreatment of choice for patients with isolated congenitalpulmonic stenosis. Indications for intervention include peaktranspulmonic valve gradient greater than 60 mmHg, or50 mmHg in the presence of symptoms, and absence ofsignificant pulmonary regurgitation [6••]. A significant reductionin transvalvular gradient is noted in the majority of patients [31].Pulmonic regurgitation may develop either early post procedure,or years later and serial echocardiography is indicated formonitoring of regurgitation and presence of RV dilation ordysfunction.

Percutaneous aortic valvuloplasty is the therapy of choicefor children and young adults with congenital aortic stenosis.After the fourth decade the aortic valve often becomesthickened and calcified and may be less amenable tovalvuloplasty. In adult patients with congenital aortic stenosis,current practice guidelines suggest that percutaneous balloonvalvuloplasty is indicated if symptoms of angina, syncope, ordyspnea are present, and peak to peak gradient bycatheterization ≥ to 50 mmHg, or asymptomatic patientswith ECG changes or peak to peak gradient of 60mmHg.Complications of aortic valvuloplasty include significantincrease in aortic insufficiency (10 %–30 %), stroke orother embolic complication if aortic calcification is present[6••, 32].

Percutaneous Valve Repair or Replacement

The ability to repair or replace dysfunctional valves is a newarea of application for interventional cardiology in adult CHD.

Fig. 8 Transthoracic echocardiogram in the parasternal long axis view ofa patient with repaired tetralogy of Fallot and percutaneously closedmuscular ventricular septal defect. Arrows demonstrate an Amplatzerclosure device placed to close a midanteroseptal muscular ventricularseptal defect

Fig. 9 Two dimensionaltransthoracic short axis images atthe base of the heart in a patientwith a patent ductusarteriosus.Panel A shows the left-to-rightshunt flow by color Dopplerthrough the ductus (small arrow).Panel B shows the presence of aductal occluder device within theductus (dual arrows). Ao aorta,MPA main pulmonary artery,PDA patent ductus arteriosus


Transcatheter pulmonary valve implantation with the MelodyValve (Medtronic Inc,MinneapolisMN) is currently approvedfor use among patients with a dysfunctional Rastelli conduitand recent data suggests very good short and medium termsuccess [33]. A growing number of patients with TOF havehad pulmonary valve replacements with bioprosthetic valvesand these patients may also be candidates for percutaneousreplacement when the bioprostheses begin to fail.Percutaneous tricuspid valve replacement with the MelodyValve has also been described in a small cohort of CHDpatients with failure of a bioprosthesis in the tricuspidposition, either due to stenosis or regurgitation [34].

The MitraClip (Abbott Vascular, Santa Clara, CA)procedure is currently approved for patients with acquiredfunctional mitral regurgitation as an alternative to surgicalmitral valve repair [35] (Fig. 10). This procedure haspromise for potential percutaneous repair of systemic TRamong patients with D-TGA post atrial switch or L-TGA[36].

Arterioplasty

Among patients with unoperated coarctation of the aorta,balloon dilation or percutaneous stent placement may beacceptable alternatives to surgical intervention based onanatomic features [37]. Percutaneous intervention is alsoemployed for patients with aortic coarctation with postsurgicalrestenosis at site of prior repair. Post procedure complicationsinclude aortic dissection, very rarely acute aortic rupture, andlate aneurysm formation. Intraprocedural intravascularultrasound may be helpful in determining optimal stentplacement and assessing arterial wall changes [38, 39]. Postprocedure echocardiography is useful to assess stent positionand residual gradient (Fig. 11).

Patients with TOF, pulmonary atresia, and those with a prioraortopulmonary shunt or pulmonary artery band often developisolated or multiple branch pulmonary stenoses. These can besuccessfully addressed in the catheterization laboratory byballoon dilation or stenting [40]. Echocardiography is usuallynot helpful in directly assessing these lesions unless they arevery proximal in the pulmonary arterial tree. Indirect echo-Doppler assessment of RV function and pressure before andafter treatment can be useful.

Baffle and Atrial Conduit Dysfunction

Among patients with D-TGA who have undergone an atrialswitch operation, up to 25 % will demonstrate late baffleleaks, and those with large defects may undergo percutaneousdevice closure. Transesophageal echocardiography andintracardiac echo have been useful to guide device placementand monitor residual shunting [41]. Baffle obstruction isobserved in up to 15 % of those with prior atrial switchprocedure, and balloon angioplasty occasionally results inlong term results; however, stent deployment is highlysuccessful in relieving obstruction with low complication rates(Fig. 12) [32].

Patients who have undergone a Fontan procedure may alsodevelop conduit leaks or conduit obstruction. Device closure,balloon angioplasty and stent placement are all employed topreserve conduit integrity (Fig. 13) [32].

Stress Echocardiography

Many “asymptomatic” adults with CHD demonstrate significantexercise intolerance when quantified with cardiopulmonaryexercise testing [42]. As bicycle stress echocardiography canassess hemodynamics, myocardial function, and myocardial

Fig. 10 Intraproceduraltransesophageal images of amitral valve with apercutaneously placed mitral clip.Panel A shows the centralechodensity from the clip(arrows) with two inflow streamsby color Doppler. Panel B is areal-time 3D image of the mitralvalve as viewed from the leftatrium. The arrow points to themitral clip, which has created adouble orifice mitral valve. LAleft atrium, LV left ventricle


perfusion in real time during physiologic exercise, it is a usefuland noninvasive modality to uncover subclinical pathology inadults with CHD. Supine bicycle echocardiography is able toquantify exercise endurance and hemodynamic response toexercise, resulting in a comprehensive functional assessment.At peak exercise, standard transthoracic echocardiography maybe challenging due to acoustic window limitations. Ultrasound

contrast has been demonstrated in the adult CHD population toimprove visualization of segmental wall motion of the LV, tobetter quantify function at peak stress, to enhance Dopplersignals for more accurate assessment of subpulmonaryventricular systolic pressure and transvalvular pressure gradientsduring exercise, and to provide information regardingmyocardial perfusion [43].

Fig. 11 Panel A shows thesuprasternal notch 2D echo imageof the aortic arch from a patientwith discrete coarctation of theaorta. Narrowing of the isthmus isapparent, with flow accelerationby color Doppler beginning at thepoint of discrete obstruction(arrow). Panel B shows thecontinuous wave Doppler spectraltracing from this patient, whichdemonstrates high peak systolicvelocity and a continued gradientinto diastole (arrows). Panel C isa suprasternal 2D echo image of apatient who has undergone stentrepair of coarctation with highlyreflective parallel walls of thestent denoted by the arrows. Thecolor Doppler flow pattern in thissame view, shows acceleration offlow at entry to the stented region.AscAo ascending aorta

Fig. 12 Two dimensional off-axis apical transthoracic images illustratingthe appearance of the interatrial baffle following a Mustard procedure forcomplete transposition of the great arteries. Panel A shows the pulmonaryvenous atrium with the baffle in the left atrium (arrows) which directsflow from the pulmonary veins across to the systemic right ventricle.Panel B depicts the systemic venous atrium with a portion of the baffle

(arrows) directing vena caval inflow across to the subpulmonic leftventricle. Panel C shows a stent placed within the systemic venouspathway of a patient with baffle stenosis (arrows). LV left ventricle,PVA pulmonary venous atrium, RV right ventricle, SVA systemic venousatrium


Pregnancy in the CHD Patient

An increasing number of women with CHD are surviving tochildbearing age. Maternal mortality is fortunately uncommon,with the exception of patients with Eisenmenger syndrome andMarfan’s syndrome with aortopathy. During pregnancy there is asignificant increase in stroke volume, heart rate, and cardiacoutput and simultaneous decrease in systemic vascular resistance.These adaptations reach a maximum during labor and delivery.As a consequence of these physiologic changes, women whowere asymptomatic prior to pregnancy may develop symptomsduring pregnancy and echocardiography can be useful in makinga new diagnosis of CHD [44•]. Simple or moderate congenitalheart defects may be uncovered, such as ASDs, patent ductusarteriosus, coarctation of the aorta, or L-TGA.

The vast majority of patients with complex CHD are alreadyknown prior to pregnancy and echocardiography is useful in pre-pregnancy evaluation to determine situations that may poseincreased maternal and fetal risk such as severe ventriculardysfunction, severe aortic stenosis, or pulmonary hypertension.It is important to note that increased stroke volume duringpregnancy will increase transvalvular gradient estimates byechocardiography. Therefore, for women with known CHD,pre-pregnancy echo assessment is most important. For womenwith ventricular dysfunction, repeat echocardiography isgenerally performed during the third trimester to evaluate forventricular function deterioration with the physiologic volumeload of pregnancy. For women with Marfan aortopathy withdilated aortic size, repeat echocardiography is recommendedevery 6–12 weeks during pregnancy to assess for diameterchange, as progressive root dilation during pregnancy is a knownrisk factor for aortic dissection [45].

Conclusions

Successful treatment of children with CHD has led to anincreasing number of adults with unoperated, palliated, andrepaired CHD entering the general adult cardiology practice.Echocardiography remains the mainstay for diagnosis andfollow-up for this population. Knowledge of the specificanatomic and functional issues for the wide variety ofcongenital anomalies is critical to insure appropriateassessment and timely intervention. Three-dimensionalechocardiography has added to the diagnostic understandingof congenital abnormalities and helps to guide interventionalprocedures. Echocardiographic measures of ventricularfunction, particularly for the RV, are of considerableimportance in this patient group. Interventional cardiologytechniques that have been developed for acquired heartdisease are also employed with good effect in the adult CHDpopulation, and echocardiography helps to evaluate thebenefits and complications of these interventions. Finally,patients with CHD are now reaching child-bearing age, andechocardiographic assessment and follow-up facilitatessuccessful maternal and fetal outcomes.

Compliance with Ethics Guidelines

Conflict of Interest Doreen DeFaria Yeh declares that she has noconflict of interest. Mary Etta King declares that she has no conflict ofinterest.

Human and Animal Rights and Informed Consent This article doesnot contain any studies with human or animal subjects performed by anyof the authors.

Fig. 13 Transthoracicechocardiographic images in theapex-down format from a patientwith tricuspid atresia following alateral tunnel Fontan procedure.Panel A shows the lateral tunnelwithin the right atrium (arrow).Panel B demonstrates a color flowjet through the Fontanfenestration (double arrows).Panel C depicts a closure device,which has been placed in thefenestration (large arrow). LAfunctional left atrium, LV leftventricle, RV right ventricle


References

Papers of particular interest, published recently, have beenhighlighted as:• Of importance•• Of major importance

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Echocardiographic Follow-up of Grown-ups with Congenital Heart Disease: Update 2013

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