|FOCUS ISSUE - CONGENITAL HEART DISEASE
|Year : 2020 | Volume
| Issue : 3 | Page : 304-311
Echocardiographic Evaluation in Complete Transposition of Great Arteries
Bharti Sharma1, Shreepal Jain2
1 Department of Pediatric Cardiology, Queensland Children's Hospital, Brisbane, Australia
2 Department of Pediatric Cardiology, Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India
|Date of Submission||09-Aug-2020|
|Date of Acceptance||02-Oct-2020|
|Date of Web Publication||18-Dec-2020|
Dr. Shreepal Jain
Tender Hearts Clinic, 16, 1st floor, Avighna 9, Dr. Babasaheb Ambedkar Road, Lalbaug, Mumbai - 400 012, Maharashtra
Source of Support: None, Conflict of Interest: None
Complete transposition of great arteries (TGA) is a common and nearly fatal cyanotic congenital cardiac malformation if not treated in time. Without any intervention, nearly 50% succumb in neonatal period and 90% before their first birthday. Advancements in diagnostic and surgical modalities have led to significant improvement in the survival rate of these children. This article discusses comprehensive echocardiographic evaluation in TGA and its variants before and after surgical correction.
Keywords: Complete transposition of great arteries, cyanotic, echocardiography
|How to cite this article:|
Sharma B, Jain S. Echocardiographic Evaluation in Complete Transposition of Great Arteries. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:304-11
|How to cite this URL:|
Sharma B, Jain S. Echocardiographic Evaluation in Complete Transposition of Great Arteries. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2020 [cited 2021 Apr 13];4:304-11. Available from: https://www.jiaecho.org/text.asp?2020/4/3/304/303937
| Introduction|| |
Complete transposition of great arteries (TGA) denotes discordant ventriculoarterial connection with concordant atrioventricular (AV) connection. This entity was first described by Matthew Baillie in 1797 as a singular malformation in which pulmonary artery (PA) arises from left ventricle and aorta arises from right ventricle. And in 1814, John Farre coined the term “transposition.” TGA represents about 5%–8% of all congenital heart malformations and was responsible for up to one-fifth cardiac deaths in 1st year of life prior to the era of surgical correction.,
Surgical management of TGA has seen significant development from physiological repair of 1950s, to anatomical repair in mid 1970s. Arterial switch operation (ASO) (Jatene procedure) in 1975 is considered as a major breakthrough in surgical correction in children with TGAs.
About 50% of neonates with TGA do not have any significant associated cardiac anomaly other than patent foramen ovale or an atrial septal defect (ASD). Ventricular septal defect (VSD) can be seen in 40% of cases; and about 5%–10% can have left ventricular outflow tract obstruction (LVOTO).,
The clinical manifestations are predominantly influenced by amount of intercirculatory mixing. Cyanosis is the most common presentation in about 90% infants and can be seen as early as day one of life in patients with intact ventricular septum (IVS). In TGA with unrestrictive VSD cyanosis can be mild, however, these patients can have early signs of heart failure. Patients with TGA and significant LVOTO can present with severe cyanosis and occasionally with hypercyanotic spells and extreme irritability.
Echocardiography is the primary diagnostic modality for confirmation of TGA. It not only gives the anatomical confirmation of the lesion but also provides the hemodynamic information about intracardiac and ductal mixing. In present times, a significant progress has been made in antenatal detection of this malformation using fetal echocardiography.
As the assessment of TGA will be predominantly in a neonate or an infant admitted to a nursery or intensive care unit, sedation is not required and child can be calmed with noninvasive measures like feeding. The role of echocardiography in TGA is not only limited to diagnosis but also in assisting in intervention such as balloon atrial septostomy (BAS) and in the evaluation of postoperative cases.
The detailed preoperative assessment of TGA by Echocardiography should include:
- Assessment of AV and ventriculo-arterial relationship
- Systemic and pulmonary venous connections
- Adequacy of inter ASD and position of atrial appendages
- Size and abnormalities of AV valves (tricuspid and mitral valves)
- Assessment of VSD type, location, routability, size, flow direction, and numbers
- The right and left ventricle (RV and LV) function and adequacy of LV
- Obstruction across RV outflow tract (RVOT) or LV outflow tract (LVOT), location, and severity
- Spatial relationship of great vessels
- Size and morphology of aortic and pulmonary valves
- Proximal coronary anatomy
- Confluence of pulmonary arteries and their dimensions; and any flow abnormality, if present
- Patent ductus arteriosus (PDA) size, flow direction, and restriction if present
- Aortic arch sidedness, branches, adequacy, and patency.
Atrio-ventricular and ventriculoarterial relationship
The diagnosis of TGA from two-dimensional transthoracic echocardiography (2D-TTE) can be established in a single sweep from subcostal long-axis view, which shows concordant AV connections, origin of a bifurcating great vessel (PA) arising from LV and other great vessel (Aorta) originating from RV, on continuing sweeping anteriorly., In TGA, both great arteries are parallel to each other in contrast to normal hearts where they cross each other [Figure 1].
|Figure 1: Subcostal coronal view showing VA discordance along with parallel arrangement of great arteries. LV: Left ventricle, RV: Right ventricle, PA: Pulmonary artery, Ao: Aorta, VA: Ventriculoarterial|
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Systemic and pulmonary venous connections
Further segmental analysis includes evaluation of systemic venous and pulmonary venous connections. Subcostal sagittal (short axis) and frontal (long axis) both view provide assessment of systemic venous drainage to right atrium (RA) and pulmonary venous drainage to left atrium (LA). Dilated coronary sinus in subcostal long axis can be an indirect marker of persistent left superior vena cava (LSVC), which, if present, should be mentioned as it can impact cannulation during cardiopulmonary bypass. The incidence of persistent LSVC is nearly 4% in TGA.
Interatrial septum can be assessed in subcostal long-axis and bicaval view. The adequacy of the interatrial communication (ASD) is assessed by the size of the defect and the mean pressure gradient across it [Figure 2]. In postnatal life, there will be increase in blood flow from PVs which results in obligatory left to right shunt across the ASD. Shunt at atrial level is largely considered as pivotal for mixing of deoxygenated and oxygenated blood especially in TGA with IVS and also in TGA with small/restrictive VSD. Oxygen saturations and TTE will guide the clinician about the adequacy of ASD and need for BAS.
|Figure 2: Subcostal coronal view showing a restrictive patent foramen ovale (arrow) with the interatrial septum bulging toward the RA. RA: Right atrium, LA: Left atrium|
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When both atrial appendages are adjacent to each other, they are termed as juxtaposed appendages. The left side juxtaposition is six times more common than the right side. Juxtaposed atrial appendages are strongly associated with TGA. About 2% of TGAs have leftward juxtaposed appendages., Juxtaposition decreases the size and volume of RA and mimics moderate to large ASD. Furthermore, the orientation of atrial septum appears perpendicular to the diaphragm with left juxtaposition in subcostal long-axis view which should be parallel in normal hearts. Juxtaposed right atrial appendage lying posterior to PA can also be seen in apical 4 chamber view and parasternal short axis view focusing on atrial septum from posterior to anterior. In the parasternal short axis view, the interatrial septum appears more horizontal rather than vertical behind the posterior great vessel due to its posterior and inferior alignment.
Atrioventricular valve abnormalities
Although functional tricuspid valve (TV) abnormalities are seen in only 4% of patients with TGA, autopsy series show a higher frequency of 31%. Structural mitral valve abnormalities can occur in 20%–30% of hearts with TGA., The abnormalities of AV valves are usually associated with TGA with VSDs especially those of conoventricular type. The abnormalities include hypoplasia, straddling or overriding, cleft mitral valve, and common AV valve. The abnormal chordal attachment of the AV valves in TGA with VSD can be well visualized from subcostal and apical for chamber views and plays a crucial role in planning surgical strategies., Occasionally, redundant TV tissue can prolapse through VSD and lead to subpulmonic obstruction. Similarly, attachment from anterior mitral valve leaflet can result in LVOTO.
Ventricular septal defect
VSD is the most common association with TGA and occurs in about 20%–40% cases.,, It can be small, large, or (rarely) multiple and can be located anywhere in septum. Types and location of VSDs include perimembranous, inlet (AV canal type), muscular and outlet (infundibular/conoventricular type) with or without malalignment. In most of the patients with TGA there is persistence of subaortic conus and regression of subpulmonary conus. Bilateral conus can be present in 3%–7% of cases. The assessment of VSD by TTE through subcostal, apical, and parasternal views will provide comprehensive details of the defect and its extent in different planes which will be decisive in choosing the different options for corrective surgeries.
Description of the straddle AV valve in inlet type of VSDs and additional chordae attachment near the VSD margins is important for surgical decisions. Conoventricular or outlet type of VSDs may be associated with malalignment of the conal septum. Anterior malalignment of the conal septum will result in varying degree of pulmonary valve override on RV and varying degree of subaortic (RV outflow) obstruction (Taussig–Bing anomaly variant). The severity of subaortic stenosis can be associated with arch hypoplasia, coarctation, or even complete interruption. Posterior malalignment will cause varying degree of subpulmonic (LV outflow) obstruction [Figure 3]. The evaluation of VSD with respect to tunneling LV to aorta on TTE is the key for biventricular repair like Rastelli or Réparation à l'Etage Ventriculaire (REV) procedure., If the size of the defect is large, a rough estimate about routability can be provided by 2D TTE based on the distance from the superior margin of the VSD to the center of arterial valve. If the distance is larger than the aortic valve diameter then routing the LV to aorta will be difficult and will result in failure of biventricular strategy using Rastelli or REV procedure.,, In such cases, aortic root translocation (Nikaidoh procedure) can be considered.
|Figure 3: Subcostal coronal 5 chamber view demonstrating a moderate sized conoventricular septal defect along with posteriorly deviated CS. The left circumflex coronary artery (arrow) can be visualised in this view coursing towards the left atrioventricular groove suggestive of its origin from sinus and coursing posterior to the pulmonary artery. LA: Left atrium, LV: Left ventricle, RV: Right ventricle, PA: Pulmonary artery, CS: Conal septum|
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A large VSD may provide good circulatory mixing in TGA in absence of interatrial communication. TGAs with large VSDs have predisposition to early pulmonary vascular disease evident in early infancy., Small VSDs are usually hemodynamically insignificant and may close spontaneously. Nevertheless, the assessment of multiple small VSDs on TTE can be challenging when pressures are equal in both ventricles. Direction of flow across the VSD is determined by the pressure difference in both systemic and pulmonary circuit in the absence of any outflow obstruction.
Ventricular wall thickness, cavity shape, and function
In TGA with intact septum, RV wall is thick compared to the LV at birth and wall thickness increases with time as RV is the systemic ventricle. LV wall thickness however decreases after few weeks as a result of fall in the pulmonary vascular resistance (PVR). In neonates with TGA and IVS, LV cavity is ellipsoid at birth but soon becomes banana shaped corresponding with a decrease in PVR., The assessment of adequacy of LV to support systemic circulation is one of the major evaluation criteria for arterial switch.
LV end-diastolic volume calculated using area length method (Bullet method) on 2D-TEE in subcostal or parasternal short axis and subcostal or apical long axis view can be used in calculating LV mass. LV mass of <60% of predicted or, when indexed to the surface area of the body, of <35 g/m2 suggests a regressed LV. End-diastolic LV posterior wall thickness measured at the mid-cavity level of <4 mm is another indicator of regressed LV [Figure 4]. Moreover, LV cavity shape assessment from subcostal short axis views of the heart at end-systole [Figure 5] can provide additional information on favorable LV for single-stage repair. LV geometry was classified as “favorable” or type I if the superoinferior/anteroposterior dimension ratio was <2; “acceptable” or type II if the ratio was between 2 and 3 [Figure 6]; and “unfavorable” or type III if the ratio was >3. Patients with the higher ratios had crescent-shaped or banana-shaped LV cavities.
|Figure 4: M-mode taken through the left ventricle in the parasternal long axis view. The left ventricular posterior wall dimension (LVPWD) being measured at end-diastole|
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|Figure 5: LV shape assessment from subcostal short axis view at end-systole. V: Vertical axis, h: Horizontal axis, LV: Left ventricle|
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|Figure 6: Subcostal short axis view at end-systole showing a D-shaped left ventricle (acceptable/Type II LV). LV: Left ventricle|
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LV wall thickness is usually preserved in the presence of a large VSD. Similarly, the LV wall thickness is preserved in the presence of LVOTO with intact interventricular septum. LV wall thickness reflects ventricle's functional capacity.
In an uncorrected TGA, RV end-diastolic volume increases and ejection fraction decreases with time. This is due to relative myocardial hypoxia or geometry of the chamber as well as secondary to increase in afterload.
Left and right ventricle outflow tract obstruction
Obstruction to pulmonary blood flow in TGA can be dynamic or anatomic. About 10% patients with TGA VSD present with LVOTO and 5% of TGA IVS can have hemodynamically significant LVOTO.,, The mechanism of obstruction includes; septal hypertrophy, subvalvar fibromuscular ridge, aneurysmal membranous septum billowing in subpulmonary area, abnormal attachment of mitral valve apparatus, posterior malalignment of the conal septum and rarely pulmonary valve stenosis., Dynamic obstruction is common in TGA IVS due to leftward bulging of the basal muscular ventricular septum toward the lower-pressure LV, which narrows the LVOT during systole but opens widely during diastole. This type of dynamic LVOTO usually resolves after arterial switch.
Gradients measured preoperatively across the LVOT by Doppler echocardiography may overestimate the degree of anatomic obstruction as a result of the greatly increased pulmonary blood flow in children with TGA, especially with an associated VSD.
RV outflow obstruction occur less frequently and can be at subvalvar (more common due to anterior malalignment of conal septum) or valvar level. Coarctation, arch hypoplasia, and interruption can be seen in 5% of cases.
Subcostal, apical, and parasternal views help provide comprehensive information about the mechanisms resulting in RV or LV outflow tract obstruction.
Spatial relation of great vessels
In TGA, aorta is anterior and to the right of PA [Figure 7]. This can be visualized well in parasternal short axis view. Occasionally, aorta and PA can be either side by side to each other or exactly anteroposterior.
|Figure 7: Parasternal short axis view showing great arterial relationship in TGA with the Ao being right and anterior to the PA. Posteriorly placed PA can be seen bifurcating into right and left pulmonary artery. PA: Pulmonary artery, Ao: Aorta, TGA: Transposition of great arteries|
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Coronary artery anatomy
In TGA, coronary anatomy is highly variable. Contemplation of arterial switch surgery makes the assessment of coronary arterial anatomy an important aspect in echocardiogram assessment in TGAs. Usually in >99% of cases, coronary artery arises from the aortic sinuses facing the pulmonary trunk [Figure 8]. Few nomenclature suggests naming facing sinuses as sinus 1 and 2 for left facing sinus and right facing sinus, respectively., The coronary arteries appear to take the shortest route to a sinus in the aortic root. Most often the left anterior descending (LAD) and circumflex (Cx) coronary arteries arise as a single trunk (left main coronary artery) from aortic sinus 1 and distribute in a normal manner. The right coronary artery arises from sinus 2 and follows its usual course.
|Figure 8: Parasternal short axis view in TGA demonstrating coronary arteries arising from the respective sinuses facing the pulmonary artery. LMCA: Left main coronary artery, RCA: Right coronary artery, TGA: Transposition of great arteries|
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The different variations of the coronary anatomy are summarized in [Table 1] and [Figure 9].,
|Figure 9: Coronary artery variations in TGA as visualised in parasternal short axis view on echocardiography. RCA: Right coronary artery, LCA: Left coronary artery, LAD: Left anterior descending coronary artery, LCx: Left circumflex artery, TGA: Transposition of great arteries|
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|Figure 10: Parasternal short axis view in TGA demonstrating the LCx coronary artery arising as a branch from RCA from Sinus 2 and coursing posterior to the pulmonary artery to reach the left atrioventricular groove. Note the LAD coronary artery arising from its respective sinus (Sinus 1). RCA: Right coronary artery, LAD: Left anterior descending, TGA: Transposition of great arteries, LCx: Left circumflex artery|
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|Figure 11: Parasternal short axis view in TGA demonstrating inverted origins of RCA and the LCx. RCA: Right coronary artery, TGA: Transposition of great arteries, LCx: Left circumflex artery|
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The challenging coronary anatomy for arterial switch is intramural coronary artery and presence of single ostia for coronary arteries which is associated with poor early outcomes and early mortality.
Parasternal short axis view is routinely used for coronary artery evaluation; furthermore, it shows commissural alignment between aortic and pulmonary valves. The variation in coronary anatomy can be evaluated by sweeps from apical 4 chamber view and subcostal views. For example, the left Cx coronary artery arising from sinus 2 and coursing posterior to the PA can be easily detected in an apical 4 chamber view sweep [Figure 3].
Arch and branch pulmonary artery anomalies
The evaluation of arch and branch PAs is done from high parasternal, right parasternal, and suprasternal views. Abnormalities of arch and branch PA are usually associated with RV or LV outflow tract obstruction, respectively.
Patent ductus arteriosus
The assessment of PDA is important for mixing in TGA. It is assessed from high left parasternal view [Figure 12] or suprasternal sagittal view due to parallel relationship of aorta and PA.
|Figure 12: High parasternal view in a case of TGA showing a large PDA originating from the aortic isthmus. Corresponding color Doppler image demonstrates left to right shunt across the PDA. TGA: Transposition of great arteries, PDA: Patent ductus arteriosus|
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| Role of Echocardiography During Balloon Atrial Septostomy|| |
This procedure can be lifesaving in neonates with TGA IVS with significant desaturation. The procedure can be done in the catheterization laboratory or at the bed side under echocardiographic guidance [Figure 13]. Both umbilical and femoral access can be used for BAS. Umbilical access is preferred when there is already an umbilical venous catheter placed and positioned in RA prior to the procedure. Furthermore, it is preferred over the femoral access to avoid risk of femoral or iliac vein occlusion in patients who might require cardiac catheterization in future (e.g., patients needing a RV-PA conduit and who might require a diagnostic or therapeutic transcatheter procedure in future). Femoral access is preferred when ductus venosus is closed or is tortuous.
|Figure 13: Echocardiography guided BAS procedure steps demonstrated on subcostal short axis or bicaval view. (a) Fogarty catheter entering the RA through the IVC; arrow. (b) Fogarty catheter (arrow) seen entering the LA through the patent foramen ovale. (c) Inflated balloon (arrow) visualised within the LA. (d) Moderate sized atrial septal defect created after BAS demonstrating right to left shunt on color Doppler (arrow). BAS: Balloon atrial septostomy, RA: Right atrium, IVC: Inferior vena cava, LA: Left atrium|
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Balloon septostomy catheter can be passed directly form umbilical access or through a 6Fr sheath from femoral venous access. Catheter entering the IVC and then into the RA can be easily imaged on TTE in subcostal sagittal view [Figure 13]a. The catheter should go almost straight into the LA if angled correctly [Figure 13]b. The balloon is then gently inflated with the help of saline and again the position is confirmed on TTE [Figure 13]c. Caution should be ensured to avoid positioning the balloon too close to the mitral valve or the pulmonary vein. The balloon is brought close against the atrial septum and then “jerked” across the septum to tear open the restricted opening. The defect size and the shunt across it is then assessed on TTE [Figure 13]d. The procedure can be repeated if the defect size or shunt appears restricted and increase in saturation is suboptimal.
| Postoperative Assessment of Transposition of Great Arteries|| |
1. Postoperative assessment in a TGA post-ASO includes
- Myocardial dysfunction or regional wall motion abnormalities (RWMA)
- Any residual defects (VSDs)
- Pulmonary hypertension
- Neoaortic valve regurgitation, neoaortic root dilatation
- Supravalvar aortic stenosis
- Supravalvar and branch PA stenosis.
Immediate postoperative management can be complicated by myocardial dysfunction and RWMA secondary to low cardiac output state or kinking of the coronaries which can be evaluated by TTE. Indirect measure of pulmonary pressures from tricuspid regurgitation jet and inter-ventricular septal position can be useful in postoperative management in intensive care units. Similarly, significant residual defects and great arterial connections can be well delineated on TTE. Apical 4 chamber, parasternal short and long axis as well as subcostal views are good for the assessment of LV functions, arterial connections and residual defects.
ASO involves switching of great arteries by re-anastomosing them at the supravalvar level. Suture lines at supravalvar area after ASO surgery can result in supravalvar narrowing in RV and LV outflows. Progressive neoaortic root dilatation and neoaortic valve regurgitation can also occur post-ASO. In pediatric population, Z scores of the root dimensions are used to keep a follow-up of patients for long term. Parasternal long axis view gives good delineation of the neoaortic root. The measurements should be done in systole.
Supravalvar and branch PA stenosis is the most common short-term complication after ASO. Lecompte maneuver, which is a part of ASO results in stretch of branch PAs on ascending aorta, more commonly left PA (LPA). On TTE, branch PA post-Lecompte procedure can be demonstrated from high parasternal or suprasternal views. The high parasternal view is equally good to assess Doppler gradients across pulmonary arteries due to alignment.
2. Postoperative assessment in a TGA postatrial switch operation (mustard/seWnning surgery)
In TGA with IVS, when LV has regressed and is not suitable for arterial switch; physiological correction in the form of atrial switch is the surgery of choice. Atrial switch, comprises of baffling the systemic venous connections (superior and inferior vena cava) to LA and pulmonary venous connections to RA. Postoperative assessment of unobstructed flow across these baffles is important and assessed by TTE from apical 4 chamber view and subcostal frontal and bicaval view. The assessment and progression of TR and RV systolic function postatrial switch by TTE is part of evaluation in long term.
Certain patients with TGA VSD may undergo staged surgery like PA band for restriction of excessive pulmonary blood flow. On the other hand, patients with TGA VSD and LVOTO may undergo placement of systemic to pulmonary shunt (Blalock–Taussig shunt) for increasing the pulmonary blood flow. The assessment of these cases in postoperative period is along the lines of respective surgeries as in other cases.
| Conclusion|| |
To summarize, echocardiography is the primary diagnostic tool in cases with TGA. TTE can be further helpful in assisting during procedure as well for postoperative management in the ICU. This modality is easily available, cost-effective, and user friendly.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13]