Defect	What to Look for on 2D	CF and Spectral Doppler
All ASDs	• Measure ASD size. • Examine MV structure and function. Look for prolapse/cleft. • Examine TV for prolapse. Measure annulus. • Examine PV drainage especially right-sided PVs. • Assess for RAE, RVE. • Assess biventricular function. • Examine morphology/function of PulmV. Look for number of leaflets, doming leaflets, commissural fusion, restricted motion (ME AV SAX, ME asc aortic SAX, ME RV inflow-outflow, UE aortic arch, SAX). • Elevated PAP, RVH, R→L shunt, flat or leftward orientation of IAS in systole may indicate Eisenmenger’s. • Look for an LSVC, located between the LUPV and the LAA (see Figure 8-5) (ME 2C, ME 4C).	• CF/PW Doppler for direction of flow (L→R in uncomplicated ASDs) (Figure 8-6). • CF/CW Doppler to grade MR, TR. • PW of PVs may show higher baseline velocities second to high PBF but pulsatility is preserved and peak and mean velocities are not significantly elevated. • Estimate PAP if TR is present RVP = PAP = 4V_TR² + RAP (ME inflow-outflow, ME 4C). • CF/CW Doppler to assess for PS. Velocities up to 2.5 m/s may result from ASD flow alone.

asc, ascending; ASD, atrial septal defect; AV aortic valve; 4C, four-chamber; CF, color flow; IAS, interatrial septum; L, left; LAA, left atrial appendage; LSVC, left superior vena cava; LUPV, left upper pulmonary vein; ME, midesophageal; MR, mitral regurgitation; MV, mitral valve; PAP, pulmonary artery pressure; PBF, pulmonary blood flow; PS, pulmonic stenosis; PulmV, pulmonary valve; PV, pulmonary vein; PW, pulsed wave; R, right; RAE, right atrial enlargement; RV, right ventricular; RVE, right ventricular enlargement; RVH, right ventricular hypertrophy; RVP, right ventricular pressure; SAX, short axis; TR, tricuspid regurgitation; TV, tricuspid valve; 2C, two-chamber; 2D, two-dimensional; UE, upper esophageal.

TABLE 8-2 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING SPECIFIC ATRIAL SEPTAL DEFECTS

Primum Atrial Septal Defects (Figure 8-1A)

• Cleft mitral valve (MV) is almost always present (see Figure 8-1B).

• Secondary changes to the MV from prolonged mitral regurgitation (MR) can result in annular dilatation, thickened leaflets, and restricted motion and complicate the repair.

• Left ventricular outflow tract obstruction (LVOTO) can develop because of:

• A deficient LV inlet compared with the LV outlet causing long tunnel-like LVOTO (“gooseneck deformity”).

• A discrete membrane causing stenosis (Figure 8-2A).

• Aberrant chords inserting into the interventricular septum (IVS) obstructing outflow (see Figure 8-2B).

• Reoperations for new/recurrent MR/LVOTO.

Figure 8-1 A, Primum ASD. ME 4C view with CF Doppler demonstrates a defect in the inferior part of the IAS and two jets of MR. More imaging suggested that the medial jet originated from the medial aspect of the cleft and the second, more central jet of MR was likely a coaptation defect from annular dilatation. Note also that both AVVs originate at the same level from the center (crux) of the heart. B, TG basal SAX view showing a large cleft (asterisk) in the anterior mitral leaflet (AML). C, Secundum ASD. Deep TG view at 90 degrees with rightward rotation (deep TG bicaval equivalent) shows a defect in the area of the fossa ovalis (FO).

Figure 8-2 A, ME AV LAX view in a patient with a primum ASD shows a chord inserting into the ventricular septum. B, ME AV LAX in a patient 2 years post primum ASD repair shows a long segment tunnel-like LVOTO that required surgical repair.

Secundum Atrial Septal Defects (see Figure 8-1C)

• Associated defects include MV prolapse, left superior vena cava (LSVC), pulmonic stenosis (PS), and partial anomalous pulmonary venous drainage (PAPVD).

• If left untreated, approximately 5% develop Eisenmenger’s syndrome (irreversible pulmonary vascular disease).

Sinus Venosus Atrial Septal Defects (Figure 8-3)

• Most are superior sinus venosus (SV) ASDs and are associated with anomalous right pulmonary vein drainage into the right atrium (RA) or superior vena cava (SVC).

• Inferior defects are very rare.

• Scimitar syndrome is anomalous pulmonary vein (PV) drainage of part or all of the right lung to the inferior vena cava (IVC). Associated defects include secundum ASD, right lung hypoplasia, and aortopulmonary collaterals supplying the right lung.

• Reoperations for obstruction of the SVC/PVs/ASD baffle.

Figure 8-3 A, Superior SV ASD. 2D and CF Doppler ME 4C view with the probe withdrawn slightly demonstrates a defect in the superior aspect of the IAS. The RUPV is seen to drain into the RA and flow is left to right through the ASD. B, Inferior SV ASD. 2D ME bicaval view shows a defect in the inferior part of the IAS. Note the enlarged IVC. C, Schematic drawing with the SV ASD straddling the right upper and lower pulmonary veins (arrows). In the right hand frame, the defect was closed using a double-patch repair technique, while assuring that the right pulmonary veins drained into the left atrium.

Courtesy of Starr Kaplan.

Coronary Sinus Atrial Septal Defects (Figure 8-4)

• The coronary sinus (CS) opens into the floor of the left atrium (LA) causing a L→R shunt. The defect may involve part or the whole CS.

• There is a high association with an LSVC and it alters the surgical repair (Figure 8-5).

• Associated defects: PAPVD, LSVC.

Figure 8-4 A, CS ASD. ME AV LAX shows the CS opening into the LA (asterisk). The extent of the defect cannot be determined in this view. B, Flow is going left to right through the defect.

Figure 8-5 A, ME modified 2C view shows an enlarged CS in the atrioventricular groove (enlarged CS marked with an asterisk). B, ME modified 5C view shows the LSVC. Always rule out an LSVC in the presence of a dilated CS (>1 cm).

Principles of Surgical/Interventional Management

• Concomitant tricuspid valve (TV) repair may be necessary if right ventricular (RV) dilatation/hypertension has caused moderate to severe tricuspid regurgitation (TR).

Ventricular Septal Defects

Key Points

All Ventricular Septal Defects (Tables 8-3 to 8-5)

• Adult presentation is rare unless the ventricular septal defect (VSD) is small or there are coexisting defects that restrict pulmonary blood flow (PBF). A high incidence of Eisenmenger’s occurs in unrepaired moderate to large VSDs.

• Cardiac catheterization must be performed when there is PHTN to evaluate pulmonary vascular resistance (PVR) and suitability for VSD closure.

• Associated lesions: ASDs, double-chamber right ventricle (DCRV), coarctation, subaortic obstruction, other complex CHDs (see Video 8-2 on the Expert Consult website).

• PHTN may occasionally progress even in those with early repair.

TABLE 8-3 SURGICAL APPROACH TO VENTRICULAR SEPTAL DEFECT CLOSURE

Most Common Surgical Approach	Defect	Additional Facts
RA	• Inlet, perimembranous, anterior malaligned VSD.

• Preferable because it is the most innocuous approach.

• Retraction of TV necessary. Occasionally partial removal and then resuspension of TV is employed.

• Anterior malaligned (TOF)

• Perimembranous/inlet if cannot be closed via RA approach.

• Division/resection of muscle bundles easily performed from RV.

• Risk of RV dysfunction or dysrhythmias.

Transpulmonary

• Outlet

Transaortic

• VSD associated with DORV or when concomitant aortic valve repair (valvuloplasty) or subaortic resection is needed.

• Higher incidence of heart block when VSD closed via this approach.

• Rarely muscular

• Risk of LV dysfunction or dysrhythmias.

DORV, double-outlet right ventricle; LV, left ventricle; LV, left ventricular; RA, right atrium; RA, right atrial; RV, right ventricle; RV, right ventricular; TOF, tetralogy of Fallot; TV, tricuspid valve; VSD, ventricular septal defect.

TABLE 8-4 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING PATIENTS WITH A VENTRICULAR SEPTAL DEFECT

TABLE 8-5 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING SPECIFIC VENTRICULAR SEPTAL DEFECTS

Perimembranous Defects (Figure 8-9A)

• Also known as infracristal, subaortic, paramembraneous, conoventricular.

• Owing to the Venturi effect from VSD flow, the right coronary cusp (RCC) and noncoronary cusp (NCC) are at risk of prolapse with the development of aortic insufficiency (AI).

• Defect may be partially/completely closed with TV tissue that appears aneurysmal (see Video 8-3 on the Expert Consult website).

• Reoperations for residual VSD, AI.

Figure 8-9 A, Perimembranous VSD. 2D and CF Doppler ME AV LAX with the probe turned rightward. There is a high-velocity restrictive VSD adjacent to the RCC in the LV and the TV in the RV. Note that the RCC is prolapsing into the defect. Ao, aorta. B, Outlet VSD. 2D and CF Doppler modified ME AV SAX demonstrates a VSD immediately underneath the PulmV. The RCC is prolapsing into the defect, although there is no AI seen (clip taken in diastole).

Inlet Defects (Figure 8-10)

• Also known as AV canal type, inlet muscular.

• Often part of an atrioventricular canal (AVC) defect or a component of heterotaxy syndrome.

• Adult presentation is rare unless PS is present.

Figure 8-10 A, Inlet VSD. ME 4C view shows a defect immediately underneath the AVV. There is an overriding TV with more than 50% of the TV opening into the LV (the stippled yellow line marks where the crest of the septum would line up with the AVV). There are also straddling chords that are not well seen in this image (see text). B, Inlet VSD as part of an AVC defect. 2D and CF Doppler ME 4C views show an inlet VSD and the associated primum ASD and common AVV. The direction of flow through the VSD is away from the LV (L→R).

Outlet Defects (see Figure 8-9B)

• Also known as subpulmonary, supracristal, doubly committed subarterial, infundibular, conal.

• Owing to the Venturi effect from VSD flow, the RCC is at risk of prolapse with the development of AI.

• Reoperations for AI.

Muscular Defects (Figure 8-11)

• May be difficult to locate especially if there are multiple defects in the trabeculated RV apex.

• Defect may partially/completely close with surrounding muscle.

• Reoperations for residual VSD.

Figure 8-11 A, Muscular VSD. CF Doppler ME AV LAX view shows a muscular VSD in a heavily trabeculated part of the mid septum. There is a left to right shunt and it is likely large given the low-velocity flow. Note also there is LV enlargement. B, CF Doppler ME 4C view shows a high-velocity restrictive VSD in the mid septum.

Malalignment Type (Figure 8-12)

• Outlet (infundibular) septum is deviated anteriorly or posteriorly and fails to join the IVS, resulting in a VSD and outflow tract obstruction.

• Anterior deviation, typical in TOF, causes VSD and right ventricular outflow tract obstruction (RVOTO).

• Posterior deviation, typical of interrupted aortic arch, causes VSD and LVOTO.

Figure 8-12 A, Anterior malaligned VSD. ME AV LAX shows the outlet septum deviated anteriorly resulting in a VSD and RVOTO. This patient had TOF. B, Posterior malaligned VSD. ME AV LAX view shows the outlet septum deviated posteriorly into the LVOT resulting in a VSD and LVOTO. This patient had an interrupted aortic arch.

Principles of Surgical Management

• TV repair/annuloplasty ring may be necessary if RV dilation/hypertension has caused moderate to severe TR.

• Aortic prolapse can usually be repaired but occasionally requires valve replacement.

• Surgical closure of VSD is with a synthetic patch via a right atrial (RA), RV, infundibular (transpulmonary), transaortic, and rarely, LV approach (see Table 8-3).

• Devices are not yet approved for routine closure of VSDs, although devices for closure of muscular and perimembranous defects are available. Hybrid approach with catheter access through a right ventriculotomy with TEE guidance is useful in some cases of apical muscular VSD.

Figure 8-13 CW Doppler of ME AV LAX view in a patient with a perimembranous VSD. CW is aligned as parallel as possible to the direction of the jet and the peak velocity is used to calculate RVP = LVP – 4V_vsd². SBP is used as LVP, assuming there is no LVOTO. As RVP nears LVP, the defect becomes unrestrictive and the velocity decreases.

Postoperative TEE Assessment after Primary or Revision Ventricular Septal Defect Repair

Step 1: Look for evidence of a residual VSD

• Residual VSD flow should be high velocity with right ventricular pressure (RVP)/left ventricular pressure (LVP) less than 0.5 to 0.6 and a size less than 25% of the diameter of the aortic annulus are ideal. Reexamine the entire IVS because the decrease in RVP can “unmask” another VSD or DCRV.

Step 2: Examine the TV apparatus, PulmV and aortic valve (AV) for evidence of injury or inadequate repair

• Look for prolapse, perforation of a cusp, or poor resuspension of TV around annulus. Less than mild AI, TR is ideal after repair.

Step 3: Examine biventricular function especially after ventriculotomies

• New or worsening ventricular dysfunction can be secondary to the ventriculotomy or injury to a coronary artery.

Step 4: Measure pulmonary artery pressure (PAP)

Atrioventricular Canal Defects (Table 8-6)

TABLE 8-6 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING PATIENTS WITH REPAIRED OR UNREPAIRED ATRIOVENTRICULAR CANAL DEFECTS

Key Points

Primum ASD

• Also called a partial AVC (see “Atrial Septal Defects” section)

Transitional Canal

• Adult presentation possible.

• Includes primum ASD, cleft MV, restrictive VSD (Figure 8-14).

• Reoperations for atrioventricular valve repair (AVVR), LVOTO

Figure 8-14 Schematic of the AVVs. A, A normal heart. B, CAVC defects. In the most common configuration, there is a common AVV with five leaflets including two right-sided lateral leaflets, one left-sided lateral leaflet, and superior and inferior leaflets that bridge the IVS. During surgical repair, the atrial and ventricular defects are closed and are fashioned to separate the valves. In addition, the cleft is closed. C, Primum and transitional AVC defects. There is a cleft in the left-sided AVV, which is usually closed during repair. IVS, interventricular septum; Lt AVV, left atrioventricular valve; Rt AVV, right atrioventricular valve; PL, posterior leaflet (of the MV).

A and B, Modified and reprinted with permission from Seale A, Shinebourne EA. Cardiac problems in Down’s syndrome. Curr Paediatr. 2004;14:33-38.

Common Atrioventricular Canal

• Includes a primum ASD, a large inlet VSD, and a common atrioventricular valve (AVV).

• Common in trisomy 21. Surgery between 4 and 6 months of age. Early development of Eisenmenger’s in nonoperated patients. Adult presentation only if patient has concomitant RVOTO.

• Single AVV has multiple leaflets: an anterior (superior) and posterior (inferior) leaflet that bridge the IVS and several lateral leaflets (see Figure 8-14; see Video 8-4 on the Expert Consult website).

• AVV is often described as right- or left-sided AVV.

• SV repair is necessary in patients with straddling chords or in patients with an unbalanced AVC (the VSD separates the heart unevenly so that one ventricle, usually the LV, is too small).

• Associated lesions: tetralogy of Fallot (TOF), heterotaxy syndrome.

• Reoperations for residual VSD, AVVR, LVOTO.

Principles of Surgical Management

• A one- or two-patch technique or the so-called Australian technique (stitch closure of VSD with patch closure of ASD) is used to close the septal defects and bridge the anterior and posterior leaflets.

• The atrial patch is usually placed so that the CS drains into the LA. An LSVC draining to the CS would preclude that.

• MV clefts are usually closed unless it would result in significant mitral stenosis (MS).

• When repaired in infancy, may have to accept even moderate residual regurgitation in order to avoid AVV stenosis or early valve replacement because morbidity/mortality is significantly increased when MVR is performed at less than 2 years. Approximately 15% of patients require reoperations for AVVR. Complex techniques often required for revision MVR (see section on “Primum Atrial Septal Defect”). MVR may be necessary in older patients.

• Significant LVOTO may require a simple resection or more aggressive intervention (see section on “Left Ventricular Outflow Tract Obstruction”).

Tetralogy of Fallot

Key Points

All Tetralogy of Fallot (Tables 8-7 and 8-8)

• Consists of RVOTO, an anterior malaligned VSD, right ventricular hypertrophy (RVH), and overriding aorta (<50% overrides the VSD). The VSD is almost always large and unrestrictive. There may be additional muscular VSDs (Figure 8-15).

• RVOTO may be multifactorial including a subpulmonary component, infundibular narrowing, PulmV annular hypoplasia, PS, and PA or branch PA hypoplasia. Rarely, there is also DCRV.

• Associated defects: PFO, ASD, additional muscular VSDs, aortopulmonary collateral arteries (APCs).

• There is a 25% incidence of right aortic arch (the arch goes over the right bronchus, but this is not seen by TEE).

• Coronary artery anomalies are common. In approximately 3%, the left anterior descending (LAD) comes from the right coronary artery (RCA) and crosses the RVOTO.

• Patients with conotruncal abnormalities (TOF, transposition of the great arteries [TGA], truncus arteriosus) can develop dilatation of the ascending aorta, which may progress and become a risk for rupture or dissection. It may also cause AI.

TABLE 8-7 PRINCIPLES OF SURGICAL/INTERVENTIONAL MANAGEMENT

Defect	Most Common Surgical Approach
All TOF (except TOF-PA)	• Complete repair consists of VSD closure, division (occasionally resection) of obstructing RV muscle bundles, pulmonary valvotomy and commissurotomy, a transannular patch or PA plasty if indicated. RA or RV approach for VSD. May require a separate infundibular incision for obstructing outlet components. • An RV-PA conduit may be necessary if aberrant coronary anatomy interferes with the ability to relieve RVOTO. • After repair, variable degree of PI results from valvotomy/transannular patch (mild to 4+) • A small atrial fenestration/PFO often left as “pop off” because of decreased RV diastolic function. • Reoperations/reinterventions for residual VSDs, persistent/recurrent RVOTO, PA stenosis, PV/AV and RV-PA conduit replacements, ASD/PFO closure, dilated ascending aorta. • Long-term RV function can be compromised by residual volume/pressure loads/ventriculotomy as a result of the disease and/or repair. Many patients returning for reoperation have decreased RV function. • The natural history of isolated root dilatation (without AI) is not known in this disease; some recommend surgery when the ascending aorta is greater than 50 to 55 cm. The optimal procedure can include aortic reduction, a valve-sparing root replacement, or a Bentall.

Defect

Most Common Surgical Approach

All TOF (except TOF-PA)

• Complete repair consists of VSD closure, division (occasionally resection) of obstructing RV muscle bundles, pulmonary valvotomy and commissurotomy, a transannular patch or PA plasty if indicated. RA or RV approach for VSD. May require a separate infundibular incision for obstructing outlet components.

• An RV-PA conduit may be necessary if aberrant coronary anatomy interferes with the ability to relieve RVOTO.

• After repair, variable degree of PI results from valvotomy/transannular patch (mild to 4+)

• A small atrial fenestration/PFO often left as “pop off” because of decreased RV diastolic function.

• Reoperations/reinterventions for residual VSDs, persistent/recurrent RVOTO, PA stenosis, PV/AV and RV-PA conduit replacements, ASD/PFO closure, dilated ascending aorta.

• Long-term RV function can be compromised by residual volume/pressure loads/ventriculotomy as a result of the disease and/or repair. Many patients returning for reoperation have decreased RV function.

• The natural history of isolated root dilatation (without AI) is not known in this disease; some recommend surgery when the ascending aorta is greater than 50 to 55 cm. The optimal procedure can include aortic reduction, a valve-sparing root replacement, or a Bentall.

TOF-PS

• The norm is complete repair as an infant. This may be preceded by an aortopulmonary shunt in some patients.

• Adult patients having primary repair would benefit from PulmV placement (or valved RV-PA conduit) at the time of the repair in order to reduce the volume load on the RV.

TOF-PA

• Surgical strategy is complex and often requires staged procedures.

• Cardiac catheterization is necessary for adequate assessment of distal PAs and MAPCAs.

• Reoperations/reinterventions. Patients require life-long interventions including repeat PA dilatations/stent placements/late VSD/ASD closure because of persistent RVOTO secondary to hypoplastic PAs. They may also have PHTN in some lung segments.

TOF-AVC

• Both lesions are usually repaired simultaneously as an infant.

• Reoperations/reinterventions for R- or L-sided AVVR/stenosis/PulmV replacement, residual VSD/RVOTO obstruction.

TOF-APV

• In addition to standard TOF repair, may require translocation and/or plication of PAs or placement of a valved RV-PA conduit.

• Reoperation/reintervention: see TOF.

DORV

• The spectrum of repairs for DORV varies from a TOF-like repair to an arterial switch procedure and occasionally other options.

• In the TOF spectrum, the VSD closure involves placing the patch in such a way that left ventricular outflow is baffled out the aorta.

• Reoperation/reintervention: see TOF. The intracardiac baffle may also result in subaortic obstruction.

AI, aortic insufficiency; APV, absent pulmonary valve; ASD, atrial septal defect; AV, aortic valve; AVC, atrioventricular canal; AVVR, atrioventricular valve regurgitation; DORV, double-outlet right ventricle; L, left; MAPCAs, multiple aortopulmonary collateral arteries; PA, pulmonary artery; PFO, patent foramen ovale; PI, pulmonic insufficiency; PS, pulmonic stenosis; PulmV, pulmonary valve; R, right; RA, right atrial; RV, right ventricle; RV, right ventricular; RVOTO, right ventricular outflow tract obstruction; TOF, tetralogy of Fallot; VSD, ventricular septal defect.

TABLE 8-8 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING REPAIRED/UNREPAIRED TETRALOGY OF FALLOT

Figure 8-15 Primary TOF repair in a 40-year-old patient. A, ME 4C view shows RVH and an additional muscular VSD. B, ME AV LAX with slight rightward rotation shows the anterior malaligned VSD with an overriding aorta.

Tetralogy of Fallot with Pulmonic Stenosis

• The most common location of PA hypoplasia is the left pulmonary artery (LPA), which is not well seen by TEE.

• APCs very rare.

• Patients rarely present with unrepaired disease as an adult.

Tetralogy of Fallot with Pulmonary Atresia

• Atresia may be limited to the valve or may involve the subpulmonary infundibulum.

• Usually variable degrees of hypoplasia of the main or branch PAs often with multiple aortopulmonary collateral arteries (MAPCAs) supplying lung segments.

• Adult presentation of unrepaired TOF-pulmonary atresia possible if MAPCAs supply enough PBF.

Tetralogy of Fallot with Atrioventricular Canal

• Patients have components of both diseases.

Tetralogy of Fallot with Absent Pulmonary Valve

• The pulmonary annulus is usually hypoplastic and there are no real PV leaflets, resulting in severe pulmonic insufficiency (PI). PAs are large and frequently aneurysmal. Often severe RV dilatation.

• The degree of airway compromise in these patients is the overriding determinant of prognosis.

Double-Outlet Right Ventricle–Tetralogy of Fallot Spectrum

• When the aorta overrides the ventricular septum by more than 50%, the patient is said to have double-outlet right ventricle (DORV).

• When associated with a subaortic VSD and subpulmonary obstruction, the patient has physiology that resembles TOF. The intracardiac anatomy resembles TOF except that the aorta is more rightward and there is no aorta-MV continuity.

Figure 8-16 ME longitudinal (A) and transverse (B) views at the level of the liver show the descending aorta and IVC on the right side. Abnormalities in the course of the descending aorta should increase the suspicion of a right aortic arch. A left aortic arch can exist with a right descending aorta, but it is unusual. HV, hepatic vein.

Figure 8-17 CW Doppler of RVOT/PulmV after TOF repair in ME AV SAX view. There is no significant RVOTO (peak gradient < 10 mm Hg) and wide-open PI lasting throughout diastole.

Postoperative TEE Assessment after Primary Repair/Reoperation of Tetralogy of Fallot

Step 1: Look for a residual VSD and reexamine the entire IVS because a drop in RVP may unmask additional VSDs

Step 2: Look for RVOTO and identify etiology

• RVP/LVP should be less than 0.6 (lower is ideal).

Step 3: Assess PulmV/RV-PA valved conduits

• Gradients should be within manufacturer’s expected estimate and PI should be minimal. Conduits may be hard to visualize but flow is usually visible.

Step 4: Assess biventricular systolic and diastolic dysfunction

• Circumflex coronary at risk of injury during PulmV replacement.

Step 5: Note the predominant direction of flow through the atrial defect

• Flow that is R→L provides a crude indication of the degree of restrictive RV dysfunction and also provides an explanation for systemic desaturation.

Step 6: Assess AV/ascending aorta after AVR/ascending aorta repair or replacement/Bentall

Transposition of the Great Arteries

Key Points

All Transposition of the Great Arteries (Table 8-9)

• Repair as a neonate or young infant with an arterial switch operation (ASO), Rastelli, or Nikaidoh (aortic translocation) procedure. Atrial switches may be seen in older adult patients.

• Adult presentation of unrepaired TGA is very rare because of pulmonary vascular disease or insufficient intracardiac mixing.

TABLE 8-9 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING REPAIRED D-TRANSPOSITION OF THE GREAT ARTERIES

D-Transposition of the Great Arteries

• The aorta originates rightward and anteriorly from the RV. The PA originates from the LV (see Video 8-5 on the Expert Consult website).

• A VSD is present in 20% of patients and 20% of those have PS.

• Patients without a VSD must have a source of intracardiac mixing, usually a PDA, and PFO, or ASD.

• A small number have an interrupted aortic arch or coarctation.

• Reoperations/reinterventions for coronary insufficiency (may be asymptomatic), supravalvular PS, conduit stenosis/insufficiency, LVOTO, RVOTO, and AI with or without a dilated aorta.

Double-Outlet Right Ventricle–Transposition of the Great Arteries Spectrum

• When the aorta arises from the RV and the PA originates from the LV but overrides the RV by more than 50%, DORV is said to exist. When associated with a subpulmonary VSD, the patient has physiology that resembles TGA and the surgical management is similar.

Congenitally Corrected Transposition of the Great Arteries (CTGA)

• Often called L (levo) TGA.

• Atrioventricular and ventriculoarterial discordance. “Physiologically correct” but anatomically “inverted.” Aorta exits anteriorly and to the left from the right-sided LV.

• Often associated with VSD (perimembranous), PS, Ebstein’s anomaly of the systemic TV.

• Reoperations/reinterventions: systemic ventricular failure and TV insufficiency, baffle obstruction/leaks, heart transplantation.

Surgical Management

• Contemporary management for d-transposition of the great arteries (d-TGA) is the ASO in which the PA and branches are brought anterior and connected to the RV (Lecompte’s maneuver) and the aorta is moved posteriorly to the LV. The coronary arteries are translocated to the aorta. Any intracardiac shunt is closed and the arch is repaired if necessary.

• Coronary abnormalities are very common in TGA (Figure 8-18). Typically, the coronaries originate from the aortic sinuses that face the PA. The most common arrangement is the anterior sinus gives rise to the right coronary artery (RCA) and the posterior sinus to the left coronary artery (LCA). Intramural and interarterial segments can also occur (see “Coronary Anomalies”). During the ASO, the coronaries are translocated to the closest area on the PulmV (neoaorta), usually the anterior/posterior right side above the respective sinus.

• Coronary insufficiency may be a result of ostial stenosis or may involve more distal stenosis. Ostial stenosis generally requires surgical intervention and balloon dilatations. Stent placement are options for more distal disease.

• Supravalvar PS or aortic stenosis (AS) may be treated with balloon dilatation/surgical repair.

• An atrial switch (Mustard’s or Senning’s procedure), which involves intra-atrial baffling of systemic and PV flow to the opposite atrium, is no longer a standard treatment for d-TGA because of early RV failure and arrhythmias.

• Baffle obstruction and leaks or PS from septal shift causing RVOTO as a result of elevated RVP may occur after an atrial switch. Baffle issues can usually be treated in the catheterization laboratory.

• A Rastelli, Nikaidoh, or ASO with left ventricular outflow tract (LVOT) enlargement are surgical options for patients with d-TGA, VSD, and PS.

• The Rastelli procedure has been the standard treatment and involves baffling the VSD so that LV outflow is routed to the anterior aorta. The PA is disconnected from the RV and an RV-PA conduit is usually used. Long-term survival is poor as a result of LVOTO/RVOTO from the intracardiac baffle and from repeated reoperations on the conduit/baffle.

• A Nikaidoh procedure involves switching the aorta along with the valve so that the aortic root, coronaries, and aorta are moved to the LV. An RV-PA conduit is also required but it is in a more favorable position than in a Rastelli. The procedure has components of a Ross, Konno, and ASO (Figure 8-19).

• The treatment of CTGA is controversial:

1 Perform repair of associated defects and “disregard” the CTGA (Figure 8-20A). This is not optimal because the RV/TV remain in the systemic ventricle, and there is a high risk of heart block, systemic ventricular failure, and TR. TR should be managed very aggressively with early valve repair/replacement.

2 Perform a double switch in early life (see Figure 8-20B). An atrial switch and ASO are performed after a preliminary PA band.

3 Heart transplant.

Figure 8-18 ME ascending aortic SAX view before an ASO shows a single coronary giving rise to the RCA and the LCA. Not well seen is the LCA dividing into the LAD and the Cx. Coronary anomalies are common and include intramural and interarterial segments.

Figure 8-19 ME AV LAX view post Nikaidoh procedure. This patient had TGA with a VSD and PS. The anterior aortic root was translocated posteriorly and is now the LV outflow vessel. The LVOT was enlarged (Konno procedure, see “Left Ventricular Outflow Tract Abnormalities”) and the VSD patch is clearly seen. The stippled arrow is where a patch is placed had a Rastelli been performed (in which case, the great vessels are not switched). The VSD patch is oriented in a more anatomic position with a Nikaidoh. The RV-PA conduit is not visible in this view (see text).

Figure 8-20 A, ME 4C view in a patient with CTGA treated with “disregard” for the CTGA. There was a VSD, which was closed, and the VSD patch is clearly visible. Note the IVS bows toward the LV because the RV is the systemic ventricle. Also note that the patient is in heart block and is paced. B, ME 4C view shows a patient who has been managed with a double switch. The IVS bows toward the RV because the LV is the systemic ventricle. There is an interatrial baffle. In this patient, there is significant apical displacement of the TV consistent with Ebstein’s anomaly of the left-sided TV.

Total Anomalous Pulmonary Venous Drainage (Table 8-10)

TABLE 8-10 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING PRIMARY/REVISION TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE

Key Points

• All PVs drain to a location other than the LA.

• PV usually drains to a horizontal confluence behind the LA and then to a vertical vein that drains to a systemic vein, most commonly the innominate vein. However, drainage may be to the SVC, azygos, CS, IVC, or other veins (Figure 8-21).

• Supracardiac total anomalous pulmonary venous drainage (TAPVD). The most common type. Drainage is usually to the innominate vein.

• Cardiac TAPVD. Drainage is usually to the CS. CS can be so large that it obstructs mitral inflow.

• Infracardiac TAPVD. Confluence is more vertically oriented. Drainage is usually to a vertical vein that descends to join the ductus venosus. Usually associated with pulmonary venous obstruction requiring emergent surgery as a newborn.

• Mixed TAPVD. A combination of these.

• A PFO/ASD is necessary for survival.

• TAPVD usually an isolated finding except when associated with heterotaxy syndrome.

• Adult presentation can occur with unobstructed TAPVD. Patients present with symptoms/signs of a large L→R shunt, cyanosis, and often PHTN.

• Reoperation/reintervention for PV stenosis, closure of ASD.

Figure 8-21 A, ME bicaval view shows an enlarged SVC from TAPVD. B, CF Doppler UE aortic arch (AA) LAX view shows an enlarged innominate vein in a patient with TAPVD.

Surgical Management

• The surgical management of TAPVD involves ligating the vertical vein and opening the pulmonary vein confluence (PVC) into the LA (incising the floor of the LA, which is the roof of the PVC).

• In newborns at risk of PHTN, a PFO may be left open to serve as a “pop-off.”

• In the case of cardiac TAPVD to the CS, the procedure involves “unroofing” the CS into the LA and closing the ostium of the CS with a patch (Figure 8-22). Unroofing is performed by incising the floor of the LA (roof of the CS) so that the CS (and anomalous PVs) drain into the LA.

Figure 8-22 ME 4C view with probe advancement in a patient with TAPVD to the CS shows a patch over the CS. The PVs and CS now drain into the LA.

Reoperations/Reinterventions

• Patients may develop symptoms of recurrent PV obstruction. They usually present in childhood but may occur later.

• Obstruction can occur:

1 At the anastomosis of the PVC and the LA (Figure 8-23).

2 Involving the PV itself. It may be extensive and involve multiple veins. The etiology is thought to be an inflammatory reaction related to the surgery/intrinsic vasculopathy. Catheterization is usually necessary to delineate the extent. Revision surgery may involve enlarging the anastomosis, alone or in combination with a “sutureless” technique of PV repair. This involves filleting the PV as distally as necessary and the pericardium is folded over and sutured to the atrium and used to “capture” PV flow. Extensive involvement may not respond well to therapy.

Figure 8-23 A, ME modified 5C view shows the LUPV drain to the PVC. A widely patent anastomosis with low-velocity flow is seen. B, ME 4C view with the probe turned to the LA. The PVC is seen entering the LA. There is turbulence at the anastomotic site (arrow) that required surgical revision. Note there is also mild TR.

Truncus Arteriosus (Table 8-11)

TABLE 8-11 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING PATIENTS AFTER TRUNCUS ARTERIOSUS REPAIR

Key Points

• Truncus arteriosus is classified according to the location of the origin of the main PA or (if absent) the separate PAs off the truncus (common great vessel exiting the heart) (see Video 8-6 on the companion website).

• Abnormal truncal valve (neoaortic valve) very common with more than three leaflets. Can be regurgitant, rarely stenotic.

• Associated defects include a subaortic VSD, interrupted aortic arch (10-15%), right aortic arch (25%), and aberrant right subclavian artery (5-10%).

• Abnormal coronary anatomy is common.

• There is very early development of pulmonary vascular disease in unrepaired patients. Adult presentation is very unlikely unless there is PS or Eisenmenger’s.

• Reoperations and reinterventions for truncal valve disease, residual VSD, RV-PA conduit replacement, and dilated ascending aorta.

Surgical and Interventional Management

Complete early repair consists of removing the PAs from the truncus, VSD closure, and placement of an RV-PA conduit. When possible, a valved conduit is used. A PFO or fenestrated atrial communication is left.

• The management of moderate to severe truncal valve regurgitation is usually attempted with a valve repair with the understanding that residual regurgitation is likely and so is future prosthetic valve replacement. When repair is not possible, a homograft AV replacement is necessary in the neonatal period.

• The placement of PA bands as palliation before complete repair was performed in the past but results were poor. Adequate PA band placement was rare and patients develop early PVD, or PA stenosis.

• Percutaneous balloon dilatation/stent placement/valve replacement (Melody valve) may be possible in some cases of conduit obstruction/regurgitation. Otherwise, reoperations are necessary for RV-PA conduit replacements and truncal (aortic) valve repair/replacement. Dilatation of the ascending aorta may require root replacement, with or without a Bental procedure.

Single Ventricle

Key Points

• Includes hypoplastic left heart syndrome (HLHS) (Figure 8-24A), single ventricle (SV; double-inlet left ventricle [DILV] most common variant), tricuspid atresia, unbalanced AVC, pulmonary atresia with intact septum (PAIVS), heterotaxy syndrome (Table 8-12).

• In double-inlet ventricle, both AVVs empty into the ventricle. A VSD (also referred to by its embryologic name, the bulboventricular foramen) leads to the outlet chamber (see Figure 8-24B). The ventricles are often transposed, as are the great vessels. There is often obstruction to one or, rarely, both great vessels.

• In tricuspid atresia, there is no effective TV orifice. The RV is frequently very hypoplastic. A VSD may be present and the vessels are often transposed.

• In PAIVS, the TV and RV are often hypoplastic. RVP may be suprasystemic. In severe cases, there are coronary artery sinusoids/fistulae and nonatherosclerotic proximal coronary artery stenosis so that myocardial perfusion becomes dependent on the right ventricle (RV) providing coronary perfusion via the fistulae. RV decompression may result in acute ischemia/death.

• Heterotaxy syndrome occurs when there is ambiguous differentiation of sidedness of the heart/lungs/visceral organs. Associated with bilateral SVC, PS, unbalanced AVC defects, interrupted IVC with azygos continuation, TAPVD, and unroofed CS. This constellation usually results in SV physiology/management (Figure 8-25).

Figure 8-24 A, ME 4C view in a patient with HLHS. The mitral annulus and MV are hypoplastic, as are the LV and ascending aorta. In some patients, there is atresia of the MV and aorta so that the ascending aorta and coronary arteries are perfused in a retrograde fashion by the PDA. B, ME 4C view in a patient with DILV. The smooth-walled LV is on the right and both inlet valves open into the LV. This patient also has transposed great vessels, which are not seen in this view. The bulboventricular foramen is large and extends from the lower arrow, which marks the crest of the IVS, to the upper arrow, which marks the crux of the heart.

TABLE 8-12 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING PATIENTS WITH SINGLE VENTRICLES

Figure 8-25 A modified ascending aortic view shows a right-sided appendage with a short narrow opening and a “Snoopy ear” appearance characteristic of an LAA.

• A three-stage approach leading to a Fontan procedure is usually necessary (Table 8-13). A stage 1 procedure is usually performed within the first couple of weeks of life unless there is a perfectly “balanced” circulation with an adequate degree of restriction to PBF and no restriction to systemic outflow. Stage 2—bidirectional cavopulmonary anastomosis (Glenn or hemi-Fontan procedure) is usually performed between 4 and 6 months. A stage 3—Fontan procedure—is performed between 18 months and 4 years.

• Occasionally, an adult will present having had only a stage 1 or 2 palliation or rarely no previous surgery.

• The principles of long-term management of patients with SV are to preserve systolic and diastolic ventricular function, and AVV competence and to keep PVR as low as possible.

• Conversion from an atriopulmonary Fontan to an extracardiac Fontan is performed in some older patients with arrhythmias/recurrent thrombus and massively dilated RAs. They must have preserved systemic ventricular function and low PVR (Figure 8-26).

• Patients with complications related to end-stage heart failure or other SV-specific diseases (e.g., protein-losing enteropathy) may be candidates for cardiac transplantation.

TABLE 8-13 SURGICAL AND INTERVENTIONAL MANAGEMENT FOR STAGE 1

Defect	Surgical Options for Stage 1	Additional Points
HLHS	• Norwood procedure—includes arch augmentation, DKS type of proximal anastomosis, atrial septectomy, and Sano (RV to PA shunt) or BT shunt (Figures 8-27A and 8-28A).	• DKS—the PulmV and proximal main PA, which is the effective systemic outflow vessel, is anastomosed end to side to the aorta.

SV-DILV

1 Norwood/DKS

2 Enlargement of BVF, PA band, arch or coarctation repair if needed (see Figure 8-26B).

• In option 2, the size of the BVF is critical because systemic flow must cross the BVF to reach the aorta. Obstruction at any time adversely affects ventricular function.

Tricuspid atresia

Unbalanced AVC

Pulmonary atresia/intact septum

• SV, one and one-half-, two-ventricle repair

• Must not decompress the RV in patients with RVDCA circulation.

• Natural history of coronary perfusion in patients with RVDCA is not known.

Heterotaxy

• Usually, a SV approach is necessary. Procedure(s) depend on associated defects.

• See below for cases with interrupted IVC and azygos continuation.

AVC, atrioventricular canal; BT, Blalock-Taussig; BVF, biventricular function; DILV, double-inlet left ventricle; DKS, Damus-Kaye-Stansel; HLHS, hypertrophic left heart syndrome; IVC, inferior vena cava; PA, pulmonary artery; PulmV, pulmonary valve; RV, right ventricle; RVDCA, right ventricle-dependent coronary artery; SV, single ventricle.

Figure 8-26 A, 2D ME bicaval view in a patient with an atriopulmonary Fontan. The RA is massively enlarged. It is important to look for thrombus. Note that the atrial septum must be intact in an atriopulmonary connection. B, Modified ME 4C view with the probe turned rightward in a patient with an extracardiac Fontan. External compression of the common atrium is seen and CF/PW Doppler images confirmed flow obstruction. Revision of the extracardiac conduit was required.

Figure 8-27 A, A patient with HLHS after a Norwood procedure. Deep TG LAX shows the RV leading to the PulmV. A DKS anastomosis is used to connect the PA and aorta. Note that there is retrograde flow in the proximal aorta (and coronary arteries). Turbulent flow through the Sano shunt is also seen exiting the RV. A velocity of up to about 3 m/s is often seen in the Sano. B, CF Doppler ME 4C view in a patient with DILV and transposed great vessels (not seen). The PA comes off the LV and the aorta off the small RV. Blood MUST pass through the BVF (VSD) in order to get to the aorta. The size of the BVF is borderline and could become restrictive. Surgical options include a DKS type of anastomosis or placement of PA bands and enlargement of the BVF.

Figure 8-28 A, CW Doppler in a ME 4C view with the probe turned toward the RV in a patient with HLHS and a Sano shunt demonstrates a peak velocity of 1.5 m/s. There is also free PI through the valveless conduit. B, PW Doppler in a ME bicaval view in a patient with a Glenn. Note the variation in PBF with the respiratory cycle while on a ventilator.

Stage 2

• Glenn/hemi-Fontan: The SVC(s) are connected to the PA so that there is passive flow to the lungs (see Video 8-7 on the Expert Consult website). IVC return is left to enter the heart. The volume work of the ventricle is decreased compared with stage 1.

Stage 3

• The Fontan procedure has undergone significant modifications since the 1970s. Original versions incorporated an atriopulmonary connection that became significantly dilated over time. Contemporary management reroutes IVC flow to the PAs via an extracardiac conduit or a lateral tunnel Fontan.

• Extracardiac Fontan: The cardiac end of the IVC is suture closed and the IVC is anastomosed to the PA via a conduit.

• Lateral tunnel Fontan: An intra-atrial baffle is fashioned from Gore-Tex and directs IVC flow to the PAs within the atrium.

• A small 3- to 4-mm fenestration may be placed between the conduit and the atrium in order to serve as a “pop-off” in the event of high SVC/PAP.

• A Kawashima Glenn (K Glenn) is a Glenn performed in a patient with an interrupted IVC and azygos continuation resulting in all lower extremity venous blood returning to the Glenn circuit via the azygos. The stage 3 procedure in those patients reroutes hepatic venous blood to the PAs via a conduit.

Coronary Artery Abnormalities

Key Points

• The left main coronary artery (LMCA) originates from the left sinus of Valsalva and bifurcates into the anteriorly directed LAD and the posteriorly directed circumflex coronary artery (Cx) just behind the main PA.

• The Cx continues laterally and posteriorly in the AV groove. In patients with a left dominant circulation, the Cx becomes the posterior descending coronary artery.

• The RCA originates anteriorly and slightly superior to the origin of the LCA from the right sinus of Valsalva and courses rightward around the atrioventricular groove to the posterior aspect of the heart. In patients with a right dominant circulation (75%), the RCA becomes the posterior descending coronary artery.

• Both the LAD and the RCA should be similar in size. A large size discrepancy can occur as a result of aneurysms, coronary fistulae, or compensatory enlargement of one coronary secondary to increased flow when it is providing collateral circulation.

• Coronary abnormalities often exist in patients with CHD (e.g., TOF, TGA, PAIVS).

• Isolated congenital coronary artery abnormalities occur in less than 1.5% of angiograms and 0.3% of autopsies.

• Despite a variety of abnormalities, most are not associated with an increased risk of sudden death. High-risk lesions include anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) or an abnormal origin or course of the coronary artery from the aorta, most commonly from the opposite aortic sinus of Valsalva with an intramural or interarterial course (especially LMCA/LAD and perhaps RCA) (Figures 8-29 and 8-30).

Figure 8-29 Schematics. A, A normal coronary pattern. B, The LCA originated aberrantly from the RCC with an interarterial course. C, The LCA with ostial stenosis and an intramural segment.

A-C, Modified and reprinted with permission from Eisses M, Verma S, Gurvitz M, Joffe DC. Echo rounds: Intramural left coronary artery. Anesth Analg. 2010;111:354-357.

Figure 8-30 A, 2D and CF Doppler ME modified AV SAX view in a newborn with ALCAPA shows the LMCA origin from the posterior PA. The vessel is seen to bifurcate into the LAD and the Cx. Retrograde systolic flow is seen in this clip and diastolic anterograde flow was seen in another. B, ME AV SAX view shows an aberrant origin of the RCA from the LCC. The RCA has an intramural segment and an interarterial course. The patient was symptomatic and had the coronary unroofed. C and D, Abnormal coronaries with no known risk: C, ME AV SAX view shows an incidental finding of both coronaries originating very closely from the appropriate cusp. D, CF Doppler ME AV LAX view shows an incidental finding of a high origin of the RCA above the sinus.

Anomalous Origin of the Coronary Artery from the Aorta

• Commonly have abnormally shaped ostia with stenosis, intramural coronary segments, or an interarterial course (see Figures 8-29 and 8-30B).

• An intramural coronary segment is one in which the coronary courses within the wall of the aorta for some distance before exiting, unlike the normal coronary artery, which exits the aortic wall perpendicularly.

• A coronary with an interarterial segment originates from the opposite aortic sinus and travels in between the great vessels to supply the appropriate territory.

• Presenting symptoms include sudden death, chest pain, and syncope.

• Presentation most commonly in the adolescent/early adult years.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

• LMCA or, very rarely, both coronary arteries arise from the left or right posterior sinus of the PA, the posterior main PA, or the PA branches (see Figure 8-30A).

• Early in life when the PVR is increased, there is anterograde flow of (deoxygenated) blood from the PA.

• As PVR decreases, reversal of flow in the left coronary system develops and there is retrograde flow into the PA. This results in coronary steal and myocardial ischemia.

• There are often some collateral vessels between the right and left coronary systems with significant enlargement of the RCA, which provides essentially all of the coronary perfusion, including retrograde flow into the PA.

• Patients often present as neonates in cardiogenic shock. Rarely, if enough collateral circulation has developed, they can present later in life with symptoms of a L→R shunt (aorta to coronary artery to PA) or symptoms of myocardial ischemia, including sudden death.

Coronary Artery Fistula

• Originates from either coronary artery but typically empties into the right side of the heart (RA, RV, or less commonly, the PA). Usually solitary fistula.

• Often accompanied by enlargement of the involved coronary because of increased flow and occasionally by aneurysm formation. In addition, it can result in steal of coronary flow from the distal segment of the involved coronary. Also, when large, the fistula results in volume overload of a right-sided structure. There is a small risk of endocarditis, aneurysm formation, and rupture of the fistula.

• Patients present in young adulthood with a murmur or symptoms of myocardial ischemia or right-sided volume overload.

Basic Echocardiographic Principles When Imaging Coronary Arteries (Table 8-14)

• The small size of coronary arteries, cardiac movement, echo drop-out, and other artifacts can make visualization of the coronaries difficult. Despite these limitations, transthoracic echocardiography (TTE) or TEE are often the only diagnostic tools necessary to identify congenital coronary abnormalities. However, cardiac catheterization is often used to confirm the diagnosis and perform a detailed examination of coronary anatomy.

• Decreasing the color scale and using spectral Doppler to time signals can be helpful.

TABLE 8-14 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING FOR CONGENITAL CORONARY ARTERY ANOMALIES (see Figure 8-30)

Principles of Surgical/Interventional Management

• The treatment of coronaries with anomalous origins involves unroofing intramural segments, enlarging abnormal ostia, translocation of the coronary to the appropriate sinus, translocation of the PA, which can compress the aberrantly located coronary or coronary bypass with venous or arterial conduits.

• Unroofing a segment that crosses an aortic valve commissure may involve resuspension of the aortic leaflet.

• Contemporary surgical treatment of ALCAPA is to translocate the coronary artery to the aorta. The etiology of the MR is usually a combination of papillary muscle dysfunction from ischemia or annular dilatation from cardiomegaly and, if mild to moderate, may improve as ventricular remodeling occurs. However, moderate to severe MR may require primary repair of the valve, although in neonates, this is rarely necessary (see “Mitral Valve Repair in Ischemic Mitral Regurgitation”).

• Most coronary fistulae can be closed using interventional catheter techniques. Rarely, surgical closure is necessary for very large fistulae. The fistula may be closed at its origin or at its entry into the heart.

Postoperative TEE Assessment after Repair of Coronary Anomalies

Step 1: Depending on the repair, verify a suitable origin and course of the repaired coronary

• Verify low-velocity anterograde flow from the ostia without obvious stenosis. Coronary flow may be hard to visualize and indirect assessment of perfusion such as RWMA and MR may be necessary.

Step 2: Confirm there is no prolapse, perforation, or other injury to the PulmV or AV

Step 3: Myocardial function is usually improved but it can be gradual if there is stunned or hibernating myocardium

Step 4: Verify MV function and grade MR (see Chapter 2)

Congenital Valvular Heart Disease

Tricuspid Valve

Key Points

Ebstein’s Anomaly (Table 8-15)

• Ebstein’s anomaly is the most common isolated congenital abnormality of the TV.

• Abnormal development of the TV, especially the septal and posterior leaflets, which are dysplastic and apically displaced occupying the ventricle and creating an “atrialized ventricle.” There is tethering and shortening of the valve and subvalvar apparatus. The annulus is normally positioned (see Video 8-8 on the companion website).

• There are variable degrees of TR and effective RV hypoplasia. Massive right heart enlargement can lead to functional pulmonary atresia and LV dysfunction. Structural pulmonary atresia is rare.

• An atrial level shunt is usually present and may be necessary for survival.

• Presentation can vary from severe cardiac compromise in a newborn to mild TR in an adult.

TABLE 8-15 BASIC ECHOCARDIOGRAPHIC FEATURES WHEN IMAGING UNREPAIRED OR PREVIOUSLY REPAIRED EBSTEIN’S ANOMALY

Surgical/Interventional Management (Table 8-16)

• Patients with Ebstein’s can have a very variable presentation from severe cardiac disease in the newborn to mild asymptomatic TV disease in older patients.

• Newborn patients who do not improve as their PVR decreases may require TV reconstruction or an RV exclusion procedure leading to an SV strategy. A valved RV-PA conduit or division of the PA may also be required if there is associated PI.

• The older symptomatic patient may require a TV repair with or without a Glenn procedure (functionally a one- and one-half ventricle repair) or a Fontan procedure.

TABLE 8-16 DETAILS OF THE SURGICAL REPAIR IN PATIENTS WITH EBSTEIN’S ANOMALY

Procedure	Key Facts
Valve repair	• Two main types of TV repair techniques. 1 Anterior leaflet of the TV is used to form a competent valve 2 A trileaflet valve that originates at the true annulus is created (the cone technique). • A reduction atrioplasty and a fenestrated atrial communication are performed in both. • The valve repairs may involve a reduction annuloplasty at the anteroposterior commissure, fenestration of the subvalvar apparatus, and/or augmentation of the anterior leaflet with a pericardial patch. • In the older patient, TVR with a bioprosthetic valve is an option.

Procedure

Key Facts

Valve repair

• Two main types of TV repair techniques.

1 Anterior leaflet of the TV is used to form a competent valve

2 A trileaflet valve that originates at the true annulus is created (the cone technique).

• A reduction atrioplasty and a fenestrated atrial communication are performed in both.

• The valve repairs may involve a reduction annuloplasty at the anteroposterior commissure, fenestration of the subvalvar apparatus, and/or augmentation of the anterior leaflet with a pericardial patch.

• In the older patient, TVR with a bioprosthetic valve is an option.

RV exclusion or Starnes procedure

• The staged approach involves placing a fenestrated patch closure on the TV (a Starnes procedure) (Figure 8-31).

• The ASD is enlarged

• A reduction atrioplasty is performed.

• An aortopulmonary shunt is placed.

• If there is PI, the PV is oversewn or the main PA is disconnected. The goal is to decompress the RV so that it does not interfere with LV function.

• A Glenn and Fontan procedure are performed at the standard time (see “SV” section).

Glenn

• Must be old enough (usually 3 mo minimum).

• Usually done in combination with a valve repair.

• May need a subsequent Fontan.

One and one-half ventricle repair

• RV function/size cannot provide a full cardiac output (IVC and SVC return).

• A Glenn is used for SVC flow; the RV pumps IVC flow to the PA.

• ASD is closed.

ASD, atrial septal defect; IVC, inferior vena cava; LV, left ventricular; PA, pulmonary artery; PI, pulmonic insufficiency; PV, pulmonary vein; RV, right ventricle; RV, right ventricular; SV, single ventricle; SVC, superior vena cava; TV, tricuspid valve; TVR, tricuspid valve repair.

Figure 8-31 ME 4C view in a patient after RV exclusion (Starnes procedure). The TV is closed but a fenestration is left to decompress the RV.

Postoperative TEE Assessment after Primary or Revision Surgery for Ebstein’s Anomaly

Step 1: Assess TV after a valve repair

• Trace to minimal TR without inflow obstruction is ideal.

• After TV repair, ensure the valve is well seated and there are no paravalvular leaks.

Step 2: Assess biventricular size and function

• The RCA is at risk of injury especially during complex valve repairs.

Step 3: If a Glenn and TV repair/replacement is being performed, assess the Glenn anastomosis and PA flow and ensure there is only trace to mild TR and adequate RV function for IVC return

• A PFO/atrial communication can be left and closed at a later time as RV function recovers.

Step 4: If the patient had an RV exclusion procedure, there will be a fenestration in the pericardial patch portion of the TV patch and the RV should appear decompressed. Confirm that the ASD is unrestrictive

• Assess LV function.

Step 5: If an RV-PA conduit was replaced, assess RV function/PulmV function

Pulmonary Valve

Key Points

• Isolated PulmV stenosis (PS) is almost always congenital and other than critical PS is often well tolerated.

• PS is due to variable degrees of fusion of the valve leaflets and commissures. The valve may be trileaflet, bicuspid, or unicuspid. There is often post-stenotic dilatation of the main PA that does not regress after treatment and poses a negligible risk of rupture, although there is a small risk of compression of surrounding structures.

• PulmV abnormalities are also characteristic of syndromes such as Alagille’s, Noonan’s, and Williams’ syndromes. The valves are often thick and dysplastic and include annular hypoplasia and stenosis of the PAs and other vessels as well.

• PI usually occurs in patients who have had balloon dilatation of congenital PS, in those status post TOF repairs/other procedures requiring RV-PA conduits. RV function may be diminished as a result of years of PI and the effects of multiple surgical procedures.

• Adult presentation of PS/PI is common. Treatment of PS is generally indicated when peak velocity is greater than 3 m/s.

Surgical and Interventional Management

• Balloon valvuloplasty is the treatment of choice for nondysplastic PS. PulmV or conduit replacement is the treatment for PI.

• Balloon dilatation is usually attempted in patients with dysplastic valves but has a lower success rate. Patients may need surgical intervention and transannular patch placement for relief of annular obstruction.

• Over time, the effects of severe PI may result in RV dysfunction. The decision to replace the valve is mostly dependent on the degree of symptoms and the function and size of the RV. Usually, a bioprosthetic valve is placed in the pulmonary position.

• RV-PA conduits require revision with interventional/surgical procedures several times during the patient’s lifetime for conduit stenosis/regurgitation. Percutaneous balloon dilatation with stent placement and valve replacement procedures are possible in some patients.

Basic Echocardiographic Features When Imaging Pulmonary Valve or Conduit Abnormalities

• Given the anterior location of the PulmV and conduit, they may not be well seen by TEE. Color flow (CF) Doppler is helpful in identifying abnormal jets but localization may be difficult. The deep transgastric (TG) long axis (LAX) view is used to measure pressure gradients (Table 8-17).

TABLE 8-17 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING PATIENTS WITH PULMONARY VALVE DISEASE

Postoperative TEE Assessment after Pulmonary Valve or Conduit Replacement

Step 1: Verify that the valve is well positioned and look for paravalvular/transvalvular insufficiency

Step 2: Examine the conduit for obstruction and grade insufficiency

Step 3: Assess biventricular function (the Cx can be injured during the procedure)

Mitral Valve (Table 8-18)

TABLE 8-18 BASIC ECHOCARDIOGRAPHIC FEATURES WHEN IMAGING THE PATIENT WITH A REPAIRED/REPLACED OR UNREPAIRED MITRAL VALVE

Key Points

• Composed of the mitral annulus, leaflets, chordae, and papillary muscles all attached to the anterolateral and posteromedial LV. Abnormalities in any of these can result in MV disease.

• Congenital MS consists of a combination of abnormalities of the mitral apparatus including thick, short, webbed chordae with restricted interchordal space (arcade) that produces LV inflow obstruction and often LVOTO. The papillary muscles may connect the leaflets to the ventricle (papillary commissural fusion). There may be a double-orifice MV or a parachute MV (there is only one papillary muscle). The result is often restricted inflow and MR (see Videos 8-9 and 8-10 on the Expert Consult website).

• Supramitral rings and cor triatriatum are anomalous fibromuscular tissue membranes that are located in the LA and can cause obstructive symptoms that mimic MS. A ring is located below the atrial appendage and is thought to be an acquired lesion that results from turbulent flow at the orifice, whereas the membrane of cor triatriatum is above the appendage (Figure 8-32).

Figure 8-32 2D and CF Doppler ME 2C view shows the membrane in a patient with cor triatriatum. The atrium is separated into a superior and an inferior chamber. The inferior chamber communicates with the LAA. The ostium of the membrane is not seen in this view.

Surgical and Interventional Management on the Mitral Valve

• Balloon valvuloplasty is the treatment of choice for rheumatic MS in the young patient, but it has limited success in patients with congenital MS.

• Valve repair is preferable to replacement in any child, even if there is residual mild to moderate disease but especially in the youngest patients (<2 yr) because of a markedly increased morbidity/mortality with MVR. In older patients, less residual disease is acceptable post repair and the lower the threshold for valve replacement.

• Some techniques of valve repair include closure of clefts, chordae lengthening and transfers, triangular resections, commissurotomy, splitting of papillary muscles, fenestrating webbed chords, pericardial patch augmentation of deficient leaflets, and annuloplasty techniques.

• Supraannular placement of a mitral prosthesis may be necessary in young children in order to place an adequate-sized valve. The atrial appendage is left above the prosthesis. The atrium may need augmentation in some cases in order to decrease atrial hypertension.

• Rarely, the Ross II procedure is used. Use the pulmonary autograft in the mitral position and insert a valved conduit in the pulmonary position.

• The “membranes” from supramitral rings and cor triatriatum are resected when obstructive.

• PHTN may resolve over time with vascular remodeling and rarely falls acutely.

• Multiple reinterventions in the form of either repeat balloon valvuloplasties or valve repair/replacement are the norm for the treatment of MS.

• The reintervention rate for patients who have had an MVR for MR depends on the etiology of the MR and the type of repair but it is not insignificant.

• Reintervention may also be necessary for prosthetic valve endocarditis, thrombosis, dehiscence, leaflet dysfunction, and pannus formation.

Postoperative TEE Assessment after Mitral Valve Repair/Replacement

Step 1: Assess the repair or replacement using techniques described in Chapter 2

Step 2: In addition, in the younger patient:

• Assess for LVOTO and AV dysfunction from an oversized MV.

• Measure PAP, although it seldom falls acutely.

Aortic Valve/Left Ventricular Outflow Tract Abnormalities (Table 8-19)

TABLE 8-19 BASIC ECHOCARDIOGRAPHIC PRINCIPLES WHEN IMAGING THE LEFT VENTRICULAR OUTFLOW TRACT AND AORTIC VALVE

Key Points

Aortic Valve

• A bicuspid AV is the most common congenital abnormality with an incidence of 1% to 2%.

• About one third remain asymptomatic, one third develop stenosis, or one third develop insufficiency. Symptoms tend to occur after childhood.

• Critical AS in the newborn is a result of a deformed valve with one to four cusps. Often associated with variable degrees of hypoplastic left-sided structures including the MV, LV, LVOT, and aorta. In addition, there may be associated endocardial fibroelastosis. The latter occurs when fibrous tissue replaces myocardium compromising LV function. Various “grading” schemes have been developed in an attempt to determine whether a one- or two-ventricle repair is best.

Left Ventricular Outflow Tract

• Subaortic membranes are fibromuscular membranes with attachments to the septum and often to the AV and MV. They may be discrete or long and complex. AI occurs in approximately 50% of patients from injury to the valve as a result of turbulent flow and/or involvement of the AV in the fibromuscular process.

Aorta

• The aorta consists of the aortic root, the ascending aorta, the proximal and distal transverse arch, the isthmus, and the descending aorta.

• The aortic root consists of the aortic annulus, the aortic cusps, the aortic sinuses, and the sinotubular junction (STJ). These structures are part of the AV apparatus and, when diseased, each component should be measured and examined to help determine the etiology and guide the surgical repair.

• The size of the aorta decreases from ascending to descending so that the descending aorta is approximately 40% of the size of the ascending aorta.

• The combination of left heart obstructive lesions including coarctation, AS, subaortic stenosis, and a supramitral ring or other forms of congenital MS, is referred to as Shone’s complex.

• Reoperations/reinterventions for AV/LVOTO/aortic disease, for recurrent AV and MV dysfunction, prosthetic valve dysfunction, endocarditis, recurrent subaortic obstruction, RV-PA conduit revision, and aortic root dilatation (more common with bicuspid AVs).

Surgical/Interventional Management

• Balloon valvuloplasty is the procedure of choice for treating isolated, noncalcified AS in pediatric patients, adolescents, and young adults (<30 yr). Pediatric patients may return for a repeat balloon valvuloplasty as long as the AI is mild. AI resulting from balloon dilatation can often be treated with a valve repair rather than AV replacement; however, they may require replacement at an older age.

• Options for valve replacements in very small infants include autograft valves (Ross procedure), homografts, and metallic prosthetic valves. The latter has a lower profile (smaller sewing ring) than bioprosthetic valves.

• The Ross procedure can be performed in combination with the modified Konno to enlarge the LVOT as well as the annulus (Ross-Konno). Reinterventions on the neoaortic valve and replacement of the RV-PA conduit are necessary.

• A Konno procedure is performed in patients needing a prosthetic valve replacement and annular enlargement with or without subaortic enlargement. This allows the placement of a larger prosthetic valve (valve can be two or three sizes greater) and reduces LVOTO. The annulus is enlarged “into” the ventricular septum using a surgically created VSD. Much less commonly, the annulus can be enlarged but to a lesser degree posteriorly toward the MV using the Manougian’s or Nick’s procedure.

• Simple resection of subaortic membranes has been associated with a high rate of recurrence (10-30%) (Figure 8-33). A peak gradient greater than 50 mm Hg (mean > 30) is an indication to resect the membrane. The risk of AI increases with gradients greater than 50 mm Hg. Very aggressive resection of the membrane on all surfaces in combination with a myomectomy may be associated with a decreased rate of recurrence and a lower incidence of AI. Alternatively, a modified Konno (LVOT enlargement without prosthetic valve placement) can be performed if there is more tunnel-like subaortic obstruction or in patients who have had multiple recurrences of the subaortic membrane (Figure 8-34).