Vous êtes sur la page 1sur 17

216

JDMS 21:216231 May/June 2005

ARTICLE 10.1177/8756479305276801 JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 PEDIATRIC ECHOCARDIOGRAPHY / Witt JDMS 21:216231 JDMS 21:216231 May/June 2005 May/June 2005 VOL. 21, NO. 3

Pediatric Echocardiography
SANDRA A. WITT, RDCS, FASE
When evaluating congenital heart disease, echocardiography is a powerful diagnostic tool. The availability, mobility, diagnostic accuracy, and noninvasive nature of echocardiography provide distinct advantages over other diagnostic modalities such as cardiac catheterization. Pediatric echocardiography has distinct differences from echocardiography performed on adults. First, the scanning techniques employed are tailored to the smaller size and younger age of the pediatric age group. Second, the types of diseases for which echocardiography is employed are necessarily different and usually consist of complex congenital lesions. Key words: segmental approach, atrial and ventricular septal defect, complex congenital heart disease

In accordance with ACCME Standards, authors are required to disclose any commercial affiliations or financial interests that might be perceived as a real or apparent conflict of interest related to the content of their JDMS CME article. The author, Sandra A. Witt, RDCS, FASE, did not disclose any real or apparent conflict(s) of interest.

Scanning Techniques
Patient comfort. The comfort of the pediatric patient is of utmost priority because it is only through a comfortable, cooperative patient that an accurate examination is obtained. The staff must establish rapport with the patient and family as soon as possible, starting with the initial introduction. A child may have limited capacity to understand what the test entails so an ageappropriate explanation of the examination is paramount. A child-friendly examination room consists of toys, colorful decorations, and a television equipped with a video recorder. Toys, videotapes, and television keep the childs attention away from the echocardiographic study and alleviate boredom. Toddlers, infants, and newborns have unique requirements. These patients frequently require sedation. The patient should have vital signs and oxymeter monitoring while sedated. Some laboratories use a nurse to help with these duties. In addition, the ultrasound gel can be warmed, and a

Correspondence: Society for Diagnostic Medical Sonography, c/o Dawn Sanchez, 2745 North Dallas Parkway, Suite 350, Plano, TX 75093. E-mail: dsanchez@sdms.org. Article originally published by the SDMS Educational Foundation within the Integrated Reference Guide, volume 2. JDMS expresses appreciation to Diana Ketchum, BS, RDCS, and S. Michelle Bierig, MPH, RDCS, RDMS, for editing and updating. The author expresses gratitude to Thomas R. Kimball, MD, for his assistance in helping in the preparation of this article. DOI: 10.1177/8756479305276801

PEDIATRIC ECHOCARDIOGRAPHY / Witt

217

radiant warmer may make infants more comfortable, thereby facilitating the examination. Transducer selection. Since the field of pediatric echocardiography necessitates the imaging of patients from birth to adulthood, a wide variety of transducers are employed. The higher frequency transducers (e.g., 7.5-12 MHz) are employed for newborns and infants, and the lower frequency transducers (2-2.5 MHz) are employed for adults. The mid-frequency transducers (3.5-5.0 MHz) are used for toddlers, children, and preteens. The transducer frequency should be chosen to optimize resolution and penetration. In most patients, this may require employing multiple transducers.
TWO-DIMENSIONAL EXAMINATION

Segmental approach to the heart. The twodimensional examination is most critical because it entails a complete delineation of the entire cardiac anatomy (segmental approach). This approach is best described by envisioning a red blood cell coursing through the heart while describing each detail of anatomy. In this way, a systematic and complete evaluation of anatomy can be achieved. In a segmental approach, the following must be defined: (1) cardiac position (levocardia, mesocardia, or dextrocardia); (2) systemic venous return; (3) atrial situs (solitus, inversus, or ambiguous); (4) right atrial anatomy, atrial septum, and tricuspid valve anatomy; (5) right ventricle and ventricular septum; (6) right ventricular outflow tract, pulmonary valve, main pulmonary artery, and branch pulmonary arteries; (7) pulmonary venous return; (8) left atrial anatomy and mitral valve; and (9) left ventricle, left ventricular outflow tract, aortic valve, coronary arteries, and aortic arch.1 Many laboratories do not actually identify these structures in the chronology outlined above because patient cooperation may not be amenable to doing so, but all laboratories take this approach in theory so that by the end of the examination, all of the cardiac segments have been identified. In this way, cardiac position, atrial situs, venous return, atrioventricular relationships, ventricular morphology, ventriculoarterial relationships, and great vessel anatomy are completely evaluated.

Imaging windows. There are four basic imaging windows: the parasternal, apical, subcostal, and suprasternal windows. Many pediatric laboratories begin the examination in the subcostal window since this is the best window to define cardiac position and situs, which is the first step in a segmental approach to the heart. However, other laboratories prefer to start in the parasternal window since this is the most comfortable for the patient. From there, the examination continues to the apical and subcostal windows and ends with the suprasternal window so that the examination progresses from most to least comfortable windows. Imaging planes. When imaging the body in general, there are three basic imaging planes: sagittal, transverse, and coronal. When imaging the heart, there are also three imaging planes corresponding to the bodys imaging planes: long axis (or sagittal), short axis (or transverse), and coronal. Whereas in the body, these planes run parallel and perpendicular to the spine, the longitudinal and short axis cardiac planes are skewed slightly to the left because the hearts axis is skewed slightly from the spine. From the parasternal window, the heart is imaged in its sagittal (long axis) and transverse (short axis) planes. From the apical window, the heart is imaged in its coronal (apical four-chamber) and sagittal (apical two-chamber) planes. The subcostal view provides images in the coronal and sagittal planes. From the suprasternal view, sagittal and coronal images of the base of the heart are obtained. M-mode examination. M-mode echocardiography can provide valuable quantitative information such as chamber dimensions and indices of left ventricular performance.
DOPPLER EXAMINATION

Color Doppler. The color Doppler examination allows for rapid determination of the presence of pathologic flow (e.g., shunts). Vascular pathology such as total anomalous pulmonary venous return, arteriovenous malformation, anomalous origin of the left coronary artery, and coronary artery fistulae is usually easily demonstrated. Color Doppler also demonstrates normal blood flow

218

JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 VOL. 21, NO. 3

TABLE 1. Prevalence of Specific Congenital Heart Defects Defect Prevalence Per 1000 Live Births 0.2 0.3 0.06 0.06 0.9 0.3 0.4 0.09 0.04 0.08 0.3 0.2 0.1 0.2 0.08

Conotruncal abnormalities Transposition of the great vessels Tetralogy of Fallot Double-outlet right ventricle Truncus arteriosus Septal defects Ventricular septal defect Atrial septal defect Endocardial cushion defect Patent ductus arteriosus Valve and vessel lesions Tricuspid atresia Pulmonary atresia Hypoplastic left heart syndrome Pulmonary stenosis Aortic stenosis Coarctation of the aorta Total anomalous pulmonary venous return

tricular pressure is the systemic arterial systolic pressure obtained from a blood pressure cuff. Therefore, the right ventricular pressure can be obtained by subtracting the ventricular septal defect Doppler gradient from the systolic blood pressure. Even in the absence of a shunt, it is possible to estimate right heart pressures. For example, in a child with suspected primary pulmonary hypertension and tricuspid insufficiency, the right heart pressure can be estimated by measuring the tricuspid insufficiency Doppler gradient (the difference between right ventricular and right atrial pressures) and adding an assumed right atrial pressure (usually taken as 10 mmHg). Furthermore, the tricuspid insufficiency jet can be interrogated while oxygen (a potent vasodilator) is administered in the echocardiography laboratory to determine if right heart pressures can be therapeutically lowered.

Congenital Heart Disease


through the heart, which can be particularly helpful when the two-dimensional examination incompletely defines the anatomy (e.g., normal pulmonary venous return). Doppler quantitation. Pulsed and continuouswave Doppler have revolutionized the pediatric examination because these modalities provide data previously only available by cardiac catheterization. Since the Doppler velocity reflects the instantaneous pressure gradient across a vascular space, it is possible to accurately and, most important, noninvasively calculate valve areas and gradients and estimate intracardiac pressures. In fact, under certain conditions, a noninvasive catheterization can be performed by extensive Doppler evaluation of the heart. In pediatrics, the right heart pressures are critical pieces of information in patient management. For example, in a child with a large ventricular septal defect, the right heart pressures may become elevated because of the large left-to-right shunt, usually an indication for surgical repair. This pressure can be obtained noninvasively using the Doppler gradient across the ventricular septal defect, which is the difference between left ventricular and right ventricular systolic pressures. In the absence of left ventricular outflow tract obstruction, the left venPrevalence. The birth prevalence of congenital heart disease has been estimated to range between 2 to 10 cases per 1000 live births. When using only cases confirmed by autopsy, catheterization, or echocardiography, the prevalence is most accurately assessed at 8.8 per 1000 live births. The prevalence of specific defects is outlined in Table 1. It is evident that the most common congenital heart defect is ventricular septal defect. The most common cyanotic lesions are the tetralogy of Fallot and transposition of the great vessels.

Acyanotic Heart Lesions


VENTRICULAR SEPTAL DEFECT

General considerations. Ventricular septal defect (VSD) is a hole of varying size in the interventricular septum allowing left-to-right shunting of oxygenated blood. As the increased volume of blood returns from the lungs to the left heart, left atrial and ventricular dilatation may result. VSDs may also result in dilatation of the right atrium and right ventricle. Small holes are of no hemodynamic significance, can be left untreated, and frequently close spontaneously. Larger defects allow large shunts, producing

PEDIATRIC ECHOCARDIOGRAPHY / Witt

219

pulmonary overcirculation, and sometimes require anticongestive medications. These large defects usually require surgical closure because of symptoms and to avoid the complication of irreversible pulmonary hypertension associated with a chronic, large left-to-right shunt. Ventricular septal defects can be located in five locations within the septum: (1) apical, (2) muscular or trabecular, (3) posterior or inlet, (4) perimembranous or subaortic, and (5) supracristal or subpulmonic. Patients may have multiple defects in different locations or a single defect extending across regions. Anatomic examination. The location and size of a VSD are established using multiple views. In a parasternal short-axis view, swept the transducer from the apex of the heart to the base of the heart with the simultaneous use of color Doppler. The apical four-chamber and subcostal views are excellent for visualizing VSDs. In general, a VSD less than the diameter of the aortic root is considered small to medium, and a VSD greater than this diameter is considered medium to large. Associated pathology (e.g., aortic coarctation, pulmonary stenosis) should be sought. Physiologic examination. The size of the left-toright shunt can be ascertained by measuring the left atrial and ventricular sizes, which will become large in the face of a significant shunt. In addition, the pulmonary-to-systemic flow ratio can be obtained by measuring the pulmonary flow (the flow across either the pulmonary or tricuspid valve) and the systemic flow (the flow across either the aortic or mitral valve). Flow is calculated by first measuring the stroke volume across the valve. Stroke volume is measured as valve area (()(r)2), and second, the velocity across the valve is measured with Doppler echocardiography. The flow (stroke volume) is then taken as (valve area) (mean velocity). A pulmonary-to-systemic flow ratio greater than 2 is considered significant. An estimate of the pulmonary artery or right ventricular pressure should also be performed by measuring the VSD or tricuspid insufficiency gradients.2 Postoperative examination. Postoperatively, the echocardiographic evaluation should assess for residual leaks and the right ventricular or pulmonary artery pressure.

ATRIAL SEPTAL DEFECT

General considerations. Atrial septal defect (ASD) is a hole in the atrial septum of varying size producing a left-to-right shunt, which results in right atrial and ventricular dilatation. Surgical closure has been the standard of care; however, transcatheter closure is becoming an attractive alternative. This procedure involves delivering a closure device across the defect with a catheter threaded to the right atrium from the femoral vein.3 Atrial septal defects are located in three basic locations within the septum: (1) secundum (midportion of the septum), (2) primum (inferior portion of the septum), and (3) sinus venosus (at the superior vena cavaright atrial junction or superior portion of the septum). In early infancy, it may be difficult to distinguish a patent foramen ovale (PFO) from an ASD. The PFO is a residual phenomenon from intrauterine life and is distinguished from a true defect because it usually closes in the first weeks of life and has a flap of tissue over it. Anatomic examination. The location and size of the defect should be determined from subcostal coronal imaging. Secundum ASDs will be seen in the mid-portion of the atrial septum. A slight inferior tilt of the transducer will demonstrate the ostium primum defect, which lies immediately adjacent to the atrioventricular valves hinge points. A slight superior and clockwise tilt will reveal the sinus venosus defect. Currently, closure by transcatheter devices is offered only for secundum ASDs. In these instances, the superior and inferior rims of the atrial septum should be measured so that the physician knows how much tissue is available to anchor the device. The examination should continue by investigating for associated pathology. Sinus venosus ASDs are frequently associated with partial anomalous pulmonary return. Ostium primum defects will frequently have anomalies of the atrioventricular valves (e.g., cleft mitral valve, atrioventricular [AV] canal). Secundum ASDs are associated with pulmonary stenosis. Physiologic examination. Although less critical than in a VSD, the pulmonary-to-systemic flow ratio can be calculated. The pulmonary flow is the flow across the pulmonary or tricuspid valves, and

220

JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 VOL. 21, NO. 3

the systemic flow is the flow across the mitral or aortic valves. Postintervention examination. Postoperatively, the evaluation should assess for residual leaks. In the case of a transcatheter closure, the device should be checked for proper placement.
ATRIOVENTRICULAR SEPTAL DEFECT

General considerations. Atrioventricular septal defect (AVSD), endocardial cushion defect, or AV canal is a common congenital heart lesion and is most often associated with patients with trisomy 21 syndrome (Down syndrome). This lesion is a large defect in the crux of the heart that affects the inferior portion of the atrial septum, the AV valves, and the posterior portion of the ventricular septum.4 In some instances, the shunting patterns are such that one ventricle may be more dominant than the other. There is usually a common AV valve that has superior (anterior) and inferior (posterior) leaflets that bridge the defect from left to right. This valve may have a common orifice or two separate orifices. In a common orifice, the valve may also be classified into a Rastelli category based on the amount of bridging and chordal attachments of the superior leaflet. A Rastelli type A valve has almost total commitment of the superior leaflet to the left ventricle and chordal attachments to the septum. A Rastelli type B valve has more bridging, and the chordal attachments are into the papillary muscles of the right ventricle. A Rastelli type C valve has maximal bridging, and the chordal attachments are into the right ventricular free wall. Patients with AVSD (especially with Down syndrome) are at risk for pulmonary hypertension, and AVSDs always require surgical closure in the first year of life. Anatomic examination. The defect and ventricular dominance (if any) are best visualized in an apical four-chamber or subcostal view with slight posterior angulation of the transducer. The morphology (orifice and Rastelli type) of the AV valve can be determined by parasternal short-axis or subcostal sagittal imaging. In a common orifice AV valve, there may also be associated left ventricular outflow tract narrowing, which is best assessed in the subcostal views. The examination should continue by focusing on associated defects,

for example, the tetralogy of Fallot, coarctation of the aorta, and double-outlet right ventricle. Physiologic examination. The physiologic evaluation should assess the intracardiac shunting and the degree of valvular insufficiency by color Doppler. Importantly, the degree of preoperative valvular insufficiency is predictive of the need for reoperation. In addition, a pulmonary-to-systemic flow ratio can be calculated by calculating the flow across the pulmonary and aortic valves. Finally, the degree of pulmonary hypertension can be calculated by interrogating a tricuspid insufficiency jet only when a restrictive VSD is present. If the VSD is not restrictive, then the rightsided pressure is systemic. Postoperative examination. Postoperatively, the echocardiographic evaluation should assess for residual atrial or ventricular shunts. Most important, however, is assessment of the mitral valve for residual mitral insufficiency. The AV valves should also be assessed for the presence of stenosis. Right ventricular pressure should be measured. Finally, the left ventricular outflow tract needs to be interrogated for residual narrowing.
PATENT DUCTUS ARTERIOSUS

General considerations. Patent ductus arteriosus is a residual intrauterine blood vessel that communicates between the underside of the aortic arch and the roof of the main pulmonary artery and usually closes in the first weeks of life. A persistent patent ductus arteriosus results in a leftto-right shunt from the aorta to the pulmonary artery, producing left atrial and ventricular dilatation. In the first weeks of life, a patent ductus arteriosus can be closed with indomethacin. Later in life, a small- or moderate-sized patent ductus arteriosus is usually closed by the transcatheter placement of coils, delivered directly into the patent ductus arteriosus by a catheter threaded to the ductal ostium via the femoral artery. A larger patent ductus arteriosus (> 4-5 mm) will require surgical ligation. Anatomic examination. The presence of a patent ductus arteriosus is established by color Doppler interrogation of the main pulmonary artery from the parasternal short-axis view with anterior angulation of the transducer. Other views include

PEDIATRIC ECHOCARDIOGRAPHY / Witt

221

the suprasternal notch window, in which the transducer can be rotated clockwise between the aortic arch and the left pulmonary artery, or the subcostal window imaging the pulmonary artery. The examination should continue by ensuring that the patient does not have a ductal-dependent (i.e., the systemic or pulmonary circulation is dependent on shunting through the ductus) lesion such as severe pulmonary stenosis or coarctation of the aorta. Physiologic examination. A ductal gradient is helpful in that, if high, the ductus is more amenable to coil closure. The gradient also yields an estimate of pulmonary artery pressure. The pulmonary-tosystemic flow ratio can be calculated by measuring flow across the mitral or aortic valves (pulmonary flow) and the tricuspid or pulmonary valves (systemic flow). Postintervention examination. After closure (either medically, surgically, or by coil), the examination should assess for residual shunting. In the case of a surgical closure, the descending aorta and the left pulmonary artery should be clearly identified since, in rare instances, these have been mistakenly ligated rather than the patent ductus arteriosus. In the case of a coil closure, the examination should assess the coil placement, with specific emphasis on the development of left pulmonary artery stenosis or descending aorta narrowing associated with improper coil placement. A ductal arch view would be especially helpful for further interrogation.

Obstructive Lesions
AORTIC STENOSIS

General Considerations. Aortic stenosis is an obstruction of left ventricular outflow. It may exist at three levels: subvalvar, valvar, or supravalvar. Subvalvar aortic stenosis is produced by a fibrous ridge of tissue or a membrane immediately below the aortic valve in the left ventricular outflow tract. Because this produces a high-velocity jet, which can strike the aortic valve, damage to the valve may occur and aortic insufficiency can develop. Valvar aortic stenosis is produced when the aortic valve leaflets are thickened and dysplastic and sometimes bicuspid. Supravalvar aortic stenosis is

a narrowing of the ascending aorta immediately above the aortic sinus. In all instances, the lesion results in obstruction of left ventricular outflow, which, when chronic, results in left ventricular hypertrophy. Concomitant aortic insufficiency may produce left ventricular dilatation as well. Severe aortic stenosis is treated surgically in the case of subvalvar or supravalvar narrowing and by balloon valvuloplasty in the case of valvar narrowing without significant aortic insufficiency. Anatomic examination. The first priority is to determine the level of stenosis. Subvalvar aortic stenosis is best diagnosed in the parasternal longaxis and apical five-chamber views. Valvar aortic stenosis is best seen in the parasternal long axis, with the aortic valve leaflets appearing thick and doming, or the parasternal short axis, where the diagnosis of a bicuspid aortic valve can be made. Supravalvar aortic stenosis is best diagnosed in a parasternal long axis, with the transducer positioned more superior on the chest, or in the suprasternal notch or subcostal views. At times, a patient may have multiple levels of stenosis; therefore, Doppler interrogation from the left ventricular outflow tract, through the aortic valve and into the distal ascending aorta, should be performed. If a patient is being considered for a balloon valvuloplasty, then the size of the aortic annulus diameter should be obtained for proper balloon sizing. The examination should also determine the presence of associated lesions, in particular, coarctation of the aorta and mitral valve anomalies. Physiologic examination. The pressure gradient across the stenosis is obtained. The peak instantaneous gradient can be obtained by placing the sample volume distal to the obstruction, measuring the peak velocity, and utilizing the Bernoulli equation. However, it should be born in mind that the aortic peak instantaneous gradient derived from Doppler is higher than the aortic peak-to-peak gradient derived from catheterization. This problem occurs because the aortic pressure curve is significantly delayed relative to the left ventricular pressure curve. When the left ventricular pressure is reaching its maximal value, the aortic pressure has not yet peaked and is still on the upstroke. Therefore, in every instance, the peak instantaneous gradient will be measuring the

222

JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 VOL. 21, NO. 3

difference between a near-peak left ventricular pressure and a subpeak aortic pressure. This gradient will be necessarily higher than the peakto-peak gradient. Therefore, echocardiographic measurements have been found that correlate better to peak-to-peak gradient than the peak instantaneous gradient.5 The mean Doppler gradient is the most useful. Color Doppler in the parasternal long-axis, apical, and subcostal views can determine the presence of associated aortic insufficiency. An estimate of the hemodynamic impact of aortic stenosis on the left ventricle can be obtained by measuring left ventricular size, mass, and function. Postintervention examination. After intervention, the gradient is again calculated by the mean Doppler gradient. The degree of aortic insufficiency is also assessed. Aortic valve replacement may involve a homograft valve or a mechanical valve. In the latter case, the valve should be inspected for paravalvar leaks. In addition, thrombi can develop on the mechanical valve, prohibiting full motion of the leaflets and resulting in obstruction. As in native aortic stenosis, the Doppler velocity across the valve can be used to obtain the gradient. However, the interpretation of these Doppler velocities must proceed with the knowledge that a mechanical valve normally has higher (up to 200-220 cm/s) velocities coursing through it. The Ross procedure is an operation for patients with severe aortic disease in which the pulmonary root is dissected off of the right ventricle and switched into the aortic position. The advantage of this operation is that native valve tissue is placed in the aortic position, making restenosis less likely. A pulmonary homograft or conduit is then used to connect the right ventricle and the distal pulmonary artery. In addition, a coronary transfer onto the neo-aorta (old pulmonary root) is necessary. When evaluating a patient with a Ross procedure, the examination should focus on neo-aortic insufficiency and dilatation of the neo-aortic sinuses. In addition, regional wall motion needs to be assessed to rule out the possibility of ischemia associated with coronary artery transfer. The pulmonary homograft/conduit needs to be assessed for pulmonary stenosis/insufficiency.

PULMONARY STENOSIS

General considerations. Pulmonary stenosis is an obstruction of right ventricular outflow, resulting in right ventricular hypertrophy. Like aortic stenosis, it can exist at three levels: subvalvar, valvar, and supravalvar.6 Subvalvar stenosis is produced from infundibular septal hypertrophy and is seen most often in patients with a VSD. In patients with valvar stenosis, the valve is thickened and dysplastic. Supravalvar stenosis occurs when there is a narrowing just distal to the pulmonary valve. In all three conditions, the right and left branch pulmonary arteries may be hypoplastic. Associated pulmonary insufficiency results in right ventricular dilatation as well. Severe subvalvar or supravalvar stenosis requires surgery. Valvar stenosis is amenable to pulmonary balloon valvuloplasty. Narrowing of the branch pulmonary arteries can be treated with balloon angioplasty with possible stent placement or surgery. Anatomic examination. Infundibular narrowing can be seen in the parasternal short-axis, the subcostal coronal, and the subcostal sagittal views. Valvar pulmonary stenosis is best seen in a parasternal short-axis view. In fact, the transducer can be turned clockwise slightly so that the number, thickness, and morphology of the leaflets can be seen. Movement of the transducer slightly cranially may also be helpful in obtaining this view. Valvar stenosis is also seen in the subcostal sagittal view. Supravalvar pulmonary stenosis is best seen in the parasternal short-axis view, but the subcostal views are complementary. The origin of the branch vessels is usually best seen in a parasternal short-axis view. A long segment of the right pulmonary artery can be easily visualized in the suprasternal coronal view. The left pulmonary artery can be seen from the suprasternal aortic arch view by rotating the transducer slightly clockwise. As in aortic stenosis, a patient may have multiple levels of stenosis, so a 2D and Doppler sweep needs to be performed from the right ventricular outflow tract, through the pulmonary valve and out into the main and branch pulmonary arteries. This is best performed in a subcostal sagittal or parasternal short-axis view. If a patient is being considered for a balloon valvuloplasty, then the pulmonary annulus diameter needs to be measured.

PEDIATRIC ECHOCARDIOGRAPHY / Witt

223

Finally, associated lesions, such as an ASD, need to be investigated. Physiologic examination. Unlike aortic stenosis, the upstrokes of the (right) ventricular and (pulmonary) arterial pressure curves occur simultaneously. Therefore, the peak instantaneous pressure derived by Doppler correlates nicely with the peak gradient by catheterization. Color Doppler in parasternal short-axis and subcostal coronal and sagittal views is best for visualizing associated pulmonary insufficiency. The hemodynamic significance of pulmonary stenosis can be assessed by determining right ventricular size and the degree of hypertrophy. Postintervention examination. In patients having undergone balloon valvuloplasty, the echocardiogram should focus on obtaining a new gradient and assessing the degree of pulmonary insufficiency from the parasternal short-axis view. Surgical results on infundibular stenosis or supravalvar stenosis should be obtained by Doppler gradient. Some repairs may involve the placement of conduits or homografts, which are frequently very difficult to image. For conduit or homograft stenosis, the sonographer places his or her hand on the chest to feel a thrill. If one is felt, then the transducer is immediately placed over the thrill. This usually defines the point for obtaining the best gradient. Since the conduits and homografts are so anterior in the chest, the transducer may need to be tilted far anterior in the parasternal short-axis and subcostal sagittal views. Color Doppler can be used to delineate the anatomy and determine where to proceed. A color jet will often identify a jet in a conduit or homograft, which can then be examined in more detail by 2D echocardiography. If the branch pulmonary arteries have undergone balloon angioplasty with stent placement, then the stents must be investigated. This is best done in the parasternal short and suprasternal notch views.
COARCTATION OF THE AORTA

General considerations. Coarctation of the aorta is a narrowing of the aorta usually just distal to the origin of the left subclavian artery and adjacent to the ductal insertion. There are generally two modes of presentation. In the newborn period, the

narrowing can be severe and the disease can be life threatening. These babies maintain systemic blood flow through a right-to-left ductal shunt. However, symptoms of cardiogenic shock develop as systemic blood flow becomes compromised when the patent ductus arteriosus begins to close. This usually constitutes a medical emergency, and a rapid echocardi- ographic diagnosis must be made. Milder forms of coarctation may be present during childhood. These patients usually have upper extremity hypertension and diminished femoral pulses. Turner syndrome is a genetic condition with a 45XO genotype, resulting in webbing of the neck and short stature and having a high incidence of coarctation of the aorta. Surgery is usually the treatment of choice. This consists of resecting the narrowed segment and (1) reanastomosing the two ends of the aorta, (2) placing an interposition graft, or (3) sacrificing the left subclavian artery and using it as a flap to augment the repair (Waldhausen repair). Alternatively, coarctation of the aorta may be treated by performing a balloon angioplasty in the catheterization laboratory. However, this is most often performed on patients who have developed renarrowing after surgery. Anatomic examination. Coarctation is best diagnosed from the suprasternal notch window. In the newborn, one can also demonstrate the ductal arch, slightly leftward from the aortic arch and consisting of the main pulmonary artery, large patent ductus arteriosus, and descending aorta distal to the narrowed segment. Although this is a valuable view because it can demonstrate the highvelocity jet produced from the narrowed segment entering the distal descending aorta, it can be mistakenly interpreted as a normal-appearing, widely patent aortic arch. The true aortic arch is identified by the vessels arising from it. The examination should also determine the presence of associated lesions such as aortic and mitral anomalies. Physiologic examination. Usually, there is a very characteristic Doppler profile in the descending aorta distal to the coarctation consisting of a high-velocity jet with a long, protracted slope.7 This long slope is due to the presence of continuous flow throughout the cardiac cycle either from a patent ductus arteriosus or collateral vessels. When present, it is so specific

224

JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 VOL. 21, NO. 3

that a diagnosis of coarctation can often be made on its presence combined with the physical findings. The coarctation gradient is obtained by subtracting the distal gradient from the proximal gradients. The left ventricular performance, size, and mass should be carefully assessed. Postintervention examination. Following surgery or balloon angioplasty, the aortic arch should be inspected for patency and residual gradient. In addition, aneurysms of the aorta may develop following balloon angioplasty. Frequently, it is only after the coarctation is repaired that the gradient of associated aortic stenosis becomes apparent. Therefore, the aortic valve and Doppler velocity should be carefully assessed.
HYPOPLASTIC LEFT HEART SYNDROME

General considerations. Hypoplastic left heart syndrome consists of varying degrees of hypoplasia of the left heart structures. Some degree of mitral and/or aortic (usually both) stenosis is often present. When clear atresia of the aortic and/ or mitral valve is present, the left ventricle is severely hypoplastic.8 However, with lesser degrees of narrowing, the left ventricle undergoes some fetal development, and the diagnosis can be difficult. Recently, there has been much discussion of how small is too small for left heart structures. Although this continues to evolve, an aortic annulus less than 5 to 6 mm, a mitral annulus less than 6 to 7 mm, and a nonapex-forming left ventricle would certainly be considered findings indicative of the syndrome. Patients with hypoplastic left heart syndrome usually present in the first 2 weeks of life but can present later (e.g., 1 month old) if there continues to be a wide patent ductus arteriosus. The presentation is often one of cardiogenic shock, as in newborns with coarctation of the aorta. However, sometimes the presentation may merely be increased precordial activity on the physical examination. These babies are usually totally dependent on a right-to-left ductal shunt to maintain systemic blood flow. The right ventricle is dilated to compensate. The condition is universally fatal unless there is medical and surgical intervention. The acute medical intervention consists of prostaglandins to maintain ductal patency, positive inotropic therapy to assist

the right ventricle, and careful management of the pulmonary vasculature with the ventilator to prohibit excessive pulmonary blood flow. From a surgical standpoint, cardiac transplant is recommended by some centers. However, lack of donor hearts is problematic. Most often, surgical reconstruction involves three stages of surgery over the first 2 years of life. This first stage (the Norwood procedure) consists of creating a neoaorta by transecting the main pulmonary artery and sewing the main pulmonary artery stump to the side of the ascending aorta. In this fashion, the right ventricle is converted to the systemic pumping chamber, and the aortic narrowing is corrected. Also part of the first stage is the placement of a Blalock-Taussig shunt for pulmonary blood flow and an atrial septectomy. The second stage, at about 6 months old, is a bidirectional cavopulmonary anastomosis (the superior vena cava is transected, and the stump is sewn to the side of the right pulmonary artery). In this manner, the deoxygenated blood from the upper half of the body is diverted directly to the pulmonary arteries. The third stage, at 12 to 18 months old, is a lateral tunnel from the mouth of the inferior vena cava through the right atrium and connecting to the superior vena cavaright pulmonary artery anastomosis. In this way, the deoxygenated blood from the lower half of the body is diverted directly to the pulmonary arteries. Sometimes, a fenestration is placed in the lateral tunnel so that the circuit can decompress into the right atrium if the pulmonary resistance becomes too high. Anatomic examination. In the presence of aortic and/or mitral atresia, the diagnosis of hypoplastic left heart syndrome is not difficult by echocardiography. Frequently, the findings will be evident in the parasternal long-axis view. The echocardiographic examination is essential in determining appropriate medical management and suitability for surgical reconstruction. Therefore, the anatomy of the pulmonary valve (which will become the neo-aortic valve), the tricuspid valve (which will become the systemic AV), and the right ventricle must be assessed. In addition, the size of the PFO should be established. A slightly small PFO is preferred since this will keep left atrial and, therefore, pulmonary artery pressures high, thereby promoting right-to-left ductal shunting.

PEDIATRIC ECHOCARDIOGRAPHY / Witt

225

The entire aortic arch must be clearly imaged so that the surgeon knows the degree of reconstruction necessary. The presence of a patent ductus arteriosus is important. The size of the pulmonary arteries should be assessed because in the second and third stages, they must be of normal caliber. The presence of a left superior vena cava needs to be established since this will need to be dealt with at the time of cavopulmonary anastomosis. The echocardiographic diagnosis of hypoplastic left heart syndrome becomes more difficult when there is stenosis (rather than atresia) of the aortic or mitral valves. In these instances, the left ventricle will have some growth and may be almost normal size. In the presence of such a variant, the aortic and mitral dimensions must be accurately measured. In addition, left ventricular volume should be measured, and contribution to the apex of the heart (apex forming) should be determined. Physiologic examination. The degree of restriction across the PFO is of critical importance in the medical management of these patients. Color Doppler will aid in determining the severity of pulmonary (neo-aortic) or tricuspid insufficiency. If there is a tricuspid insufficiency jet, right ventricular function can be assessed by calculating the peak rate of pressure generation (dP/dtmax). Right ventricular function may also be qualitatively evaluated or quantitated using tissue Doppler imaging. This is done by obtaining a clean Doppler envelope of the tricuspid insufficiency jet. The time in seconds it takes the pressure curve to course from the 1-m/s calibration mark to the 3-m/s calibration mark (t) is measured. The dP/dtmax is calculated as the difference in pressure (36 4) divided by the time or 32/t (normal > 1000 mmHg/s). Finally, the direction and degree of shunting across the patent ductus arteriosus should be assessed. The medical management of these patients focuses on optimizing the right-to-left shunt through the ductus so that systemic blood flow is enhanced. Postintervention examination. After the Norwood procedure, the echocardiographic examination is used to assess right ventricular function, the degree of tricuspid insufficiency, the degree of aortic insufficiency, and aortic arch

patency. The suture line of the old pulmonary artery to the ascending aorta should be assessed for stenosis. The atrial septum should be examined to ensure that it is no longer restrictive. The BlalockTaussig shunt should be assessed for patency. In preparation for the second stage (bidirectional cavopulmonary anastomosis), the most critical issue is the size of the pulmonary arteries, which need to be of normal size. This allows the pulmonary artery pressure to be low enough to allow flow from the superior vena cava to the pulmonary arteries. In addition, the features examined following the first stage (above) should be reevaluated at this time. Following the second stage, in preparation for the third stage, the patency of the cavopulmonary anastomosis needs to be assessed. This is best performed from a suprasternal coronal view. In this view, the superior vena cava is seen draining into the right pulmonary artery. This connection should be assessed for thrombus. Following the third stage, the pulmonary artery pressures can be directly measured by adding the lateral tunnel fenestration gradient to an assumed right atrial pressure of 10 mmHg from subcostal coronal views. The connection of the lateral tunnel to the pulmonary artery and its patency are examined by subcostal sagittal imaging. In the long term, children who have progressed through all three surgical stages should be assessed for right ventricular dysfunction. The dP/dtmax can be followed serially. Chronically, these patients can also develop pulmonary arterial to pulmonary venous collaterals, which may be evident on the echocardiographic examination. Patency of the lateral tunnel and cavopulmonary artery anastomosis are always a concern. Finally, the fenestration gradient can be followed as an index of pulmonary artery pressures.

Cyanotic Lesions
TRICUSPID ATRESIA

General considerations. Tricuspid atresia is a rare lesion in which the tricuspid valve fails to develop, and instead there is a musculofibrous plate across the tricuspid annulus, which prohibits blood passage from the right atrium to the right

226

JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 VOL. 21, NO. 3

ventricle. A large ASD is almost always present, essentially resulting in a single atrium. The amount of pulmonary blood flow is dependent on the size of the associated VSD and the pulmonary artery. If these structures are large, then pulmonary blood flow may be sufficient. If, however, these structures are small, then pulmonary blood flow will be insufficient, and there must be an auxiliary source of pulmonary blood flow. These patients may be dependent on a left-to-right ductal shunt as newborns and may require a Blalock-Taussig shunt. Tricuspid atresia is also associated with transposition of the great vessels such that the transposed aorta is supplied through the VSD. If the VSD is small, then there may be aortic hypoplasia and coarctation. The patient may then be dependent on a right-to-left ductal shunt to maintain systemic blood flow. In these instances, the patient usually requires a Damus-Kaye-Stansel operation in which the pulmonary artery is transected and anastomosed to the side of the aorta. This eliminates the need for antegrade flow through the VSD and small aorta. Pulmonary blood flow is established through a Blalock-Taussig shunt. In either instance, the patient is destined for a single ventricle repair (i.e., bidirectional cavopulmonary anastomosis and lateral tunnel). The timing of these surgeries is the same as in the hypoplastic left heart syndrome. Anatomic evaluation. The echocardiographic diagnosis of tricuspid atresia is established in the apical four-chamber and subcostal views where the tricuspid plate is obvious. The great vessel relationship should be established in the parasternal short-axis view. The size of the ASD should be assessed from the subcostal view. This defect is usually in the superior atrial septum, so a suprasternal notch view is often useful. The presence of a patent ductus arteriosus is established in the parasternal short and suprasternal notch views. The superior vena cava needs to be demonstrated because of the future bidirectional cavopulmonary anastomosis these patients will undergo. In the case of normally related great vessels, the main and branch pulmonary artery sizes should be measured. In the case of transposition of the great vessels, the aorta and aortic arch should be carefully imaged. The

suprasternal notch view will be helpful to determine the presence of coarctation of the aorta. Physiologic evaluation. The flow pattern through the ASD is established by subcostal imaging with color and pulsed Doppler. Bowing of the atrial septum into the left atrium may also be a sign of a restrictive ASD. Mitral insufficiency, if present, is apparent from the apical view with color Doppler. The gradient through the VSD is a critical part of the physiologic examination and is best assessed by apical and subcostal imaging. The parasternal long axis places the VSD flow directly parallel to the ultrasound beam, making this a good view as well. Doppler interrogation should be performed from the left ventricle, through the VSD, and into the great vessel arising from the rudimentary right ventricle to assess for all levels of stenosis. The amount and direction of ductal shunting are established with color Doppler in the parasternal short-axis and suprasternal notch views. Postintervention examination. After the neonatal palliation (Blalock-Taussig shunt or Damus-Kaye-Stansel operation), the outflow of the ventricles should be assessed for the degree of residual obstruction in the subcostal view. The ASD size and gradient should be assessed. The patency of the Blalock-Taussig shunt is evaluated. If coarctation of the aorta is present, the patency of the arch should be established. Most crucial is the size of the pulmonary arteries, which need to be of adequate size for the later stages of the cavopulmonary anastomosis and the lateral tunnel. The echocardiographic evaluation following these two operations is similar to that in the hypoplastic left heart syndrome.
TETRALOGY OF FALLOT

General considerations. Tetralogy of Fallot is the most common cyanotic congenital heart lesion. It consists of a VSD, aortic override, pulmonary stenosis, and right ventricular hypertrophy.9 The degree of pulmonary stenosis determines the amount of pulmonary blood flow and, therefore, the degree of cyanosis of the patient. Pulmonary stenosis may range from mild, resulting in normal pulmonary blood flow, to severe, resulting in very little pulmonary blood flow and the need for a left-

PEDIATRIC ECHOCARDIOGRAPHY / Witt

227

to-right ductal shunt. The extreme is pulmonary atresia, in which there is no antegrade pulmonary blood flow and the patient is totally dependent on the patent ductus arteriosus. Pulmonary stenosis is infundibular, valvar, and supravalvar. In addition, the pulmonary arteries are frequently hypoplastic. The treatment depends on the degree of pulmonary stenosis. If the patient is ductal dependent, prostaglandins are used to maintain ductal patency, and a Blalock-Taussig shunt is created. Surgical correction involves closing the VSD and relieving the pulmonary stenosis by placing a right ventricular outflow patch, resecting infundibular stenosis, placing a transannular patch, performing a pulmonary valvuloplasty, and/or performing a pulmonary arterioplasty. In the case of pulmonary atresia, a homograft or valved conduit is placed in the pulmonary position. The aim of the surgery is to relieve enough pulmonary stenosis so that the right ventricular pressure is much less than the systemic pressure when the VSD is closed. Anatomic evaluation. Aortic override is best assessed in the parasternal long-axis view. Pulmonary stenosis is best assessed in the parasternal short-axis and subcostal coronal and sagittal views. In addition, the branch pulmonary arteries can be imaged from the suprasternal notch view.10 Assessing the level of pulmonary stenosis throughout the right ventricular outflow tract is the most critical part of the echocardiographic examination. Many patients with tetralogy of Fallot have right-sided aortic arches, which can be assessed from the suprasternal notch view. In addition, some patients have an aberrant left anterior descending coronary artery and/or a prominent conal branch arising from the right coronary artery and coursing over the right ventricular outflow tract. It is critical to identify these coronary anomalies before surgical correction because these coronary vessels lie in close proximity to possible surgical incisions. These anomalies are best identified from the parasternal short-axis view. Physiologic evaluation. The degree of pulmonary stenosis should be assessed with Doppler interrogation starting in the right ventricular outflow tract and continuing through the pulmonary valve, main pulmonary artery, and

branch pulmonary arteries, from the parasternal short-axis or subcostal sagittal views. In the newborn period, the pulmonary stenosis gradient may be underestimated because of the physiologically elevated pulmonary artery pressures, which do not decrease to normal values until the second week of life. Often, the aorta is so large that aortic insufficiency is present. This can be semiquantitated by assessing its color Doppler jet from the parasternal long-axis, apical, and subcostal views. Postintervention examination. For patients having undergone a Blalock-Taussig procedure, shunt patency should be established from the suprasternal notch view. For patients having undergone complete repair, the most critical issue is imaging the right ventricular outflow tract and measuring right ventricular pressure and residual pulmonary stenosis gradient. This pressure can be compared to the systemic systolic blood pressure to obtain a percentage of systemic pressure. A right ventricular pressure of less than 50% of systemic pressure is generally an adequate repair. Children with homografts and conduits are at high risk for developing stenosis. The simultaneous use of color Doppler is beneficial in following a particularly difficult-to-image conduit. The VSD patch should be assessed for residual leaks in the parasternal short-axis and apical views.
TRUNCUS ARTERIOSUS

General considerations. Truncus arteriosus consists of a single great vessel and semilunar valve overriding the ventricular septum and a VSD. The pulmonary arteries arise as branches from the trunk. Frequently, the truncal valve will have multiple, thick, and stenotic cusps. These cusps may fail to coapt completely, leading to truncal valve insufficiency. Anomalies of the aortic arch are relatively common in patients with truncus arteriosus and include right-sided aortic arch and interrupted aortic arch. Some patients may have genetic syndromes such as trisomy 22 or DiGeorge syndrome. The latter also have abnormalities with the T cells of the immune system and thymic hypoplasia. Surgical correction involves closing the VSD so that the truncal valve becomes the neoaortic valve arising from the left ventricle. The

228

JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 VOL. 21, NO. 3

branch pulmonary arteries are removed from the trunk and sewn to the distal end of a homograft or conduit arising from the right ventricle. Anatomic evaluation. The overriding trunk and VSD can be visualized in the parasternal long-axis view. The absence of a pulmonary valve and the branching pattern of the pulmonary arteries from the trunk are best seen in the parasternal short-axis, subcostal sagittal, subcostal coronal, and suprasternal notch views. The presence of a thymus should be documented in the high parasternal short-axis view. The cusps of the truncal valve and their degree of coaptation are best seen in the parasternal short-axis view. The aortic arch sidedness and patency are assessed in the suprasternal notch view. Physiologic evaluation. In the presence of thickened truncal valve leaflets, a truncal valve gradient should be obtained in the suprasternal notch view. The degree of truncal insufficiency can be semi-quantitated by examining its color Doppler jet in the long-axis, subcostal, and apical four-chamber views. Postintervention examination. Following repair, patients are at risk for stenosing their conduit or pulmonary homograft. This anatomy must be carefully inspected, usually from a parasternal short-axis view. Right ventricular pressure should be calculated. The VSD patch should be assessed in the parasternal short-axis and apical views for a residual VSD. The arch patency should be assessed from the suprasternal notch.
COMPLETE TRANSPOSITION OF THE GREAT VESSELS

General considerations. Complete transposition of the great vessels is one of the more common cyanotic congenital heart lesions. It consists of the aorta arising from the right ventricle and the pulmonary artery arising from the left ventricle. This results in deoxygenated blood from the body being recirculated to the body without gaining oxygen. It also results in oxygenated blood being recirculated to the lungs rather than being delivered to the body. For life to be sustained, there must be mixing of blood at the atrial, ventricular, and/or ductal levels. Acute management of these newborns focuses on establishing a ductal and/or atrial shunt. The former is done by administering

prostaglandins intravenously. The latter is accomplished by a balloon atrial septostomy via catherization. Surgical correction includes the arterial switch operation, in which the aorta and pulmonary artery are transected and the aorta is anastomosed to the old pulmonary artery stump on the left ventricle. The pulmonary artery is anastomosed to the old aortic stump on the right ventricle, and the coronary arteries are transferred from the old aortic stump to the old pulmonary stump. Anatomic evaluation. The diagnosis of complete transposition is made in the parasternal long-axis view by determining that the great vessel originating from the left ventricle courses more posteriorly like a pulmonary artery than superiorly like an aorta. In the parasternal short-axis view, the aorta is anterior (and to the right) of the pulmonary artery. In addition, the great vessel axes are parallel. In the subcostal view, the great vessel originating from the left ventricle is seen to bifurcate into branch pulmonary arteries. The anatomy of the pulmonary valve is assessed carefully since this will become the neo-aortic valve. The presence of a patent ductus arteriosus is assessed in the parasternal short-axis and suprasternal notch views. The size of the PFO is assessed in the subcostal view. Finally, the coronary artery origins are evaluated in a high parasternal short-axis view. It is not unusual to have anomalous origins of the coronary arteries. An unusual coronary origin may cause kinking of the coronary artery when it is transferred, leading to myocardial ischemia. Identifying the coronary arteries by echocardiography is one of the most difficult tasks asked of a sonographer. Lowvelocity color Doppler may aid in identifying the coronary ostia. Physiologic evaluation. The physiologic evaluation focuses on establishing the gradient across the PFO to determine if there is restriction. Doppler velocities should also be obtained across the pulmonary and aortic valves to assess for possible stenosis. Postintervention examination. Before surgery to promote mixing, newborns usually require a balloon atrial septostomy, which is often done under echocardiography guidance. While the echo-

PEDIATRIC ECHOCARDIOGRAPHY / Witt

229

cardiographer images from the subcostal coronal and sagittal planes, the catheter is threaded up the inferior vena cava into the right atrium. While viewing the subcostal images, the catheter is placed across the PFO into the left atrium. The balloon can then be inflated in the left atrium and jerked back across the PFO into the right atrium. The size of the PFO and the gradient across it can be compared to preseptostomy values. After the arterial switch operation, the aorta and pulmonary artery need extensive Doppler interrogation to examine for the development of supravalvar aortic and pulmonary stenosis at the surgical suture lines. The pulmonary artery is often difficult to visualize by echocardiography postoperatively because it is pulled far anterior. However, it can usually be assessed in the parasternal short-axis and subcostal (with great anterior angulation) views. It is also important to visualize the pulmonary artery branches postoperatively, as they can become obstructed after surgery. The wall motion of the myocardium should be assessed for possible damage because of myocardial ischemia from the coronary artery transfer. Sometimes, patients have dilatation of the neo-aortic root, resulting in severe aortic insufficiency seen in the parasternal longaxis view.
TOTAL ANOMALOUS PULMONARY VENOUS RETURN

General considerations. Total anomalous pulmonary venous return is a condition in which the pulmonary veins do not drain into the left atrium but rather drain into a right-sided structure. The anomalous drainage pathway may take one of three routes: directly into the heart (e.g., the right atrium or coronary sinus), supracardiac (i.e., from a posterior confluence into a vertical vein, innominate vein, superior vena cava, and then right atrium), or infracardiac (i.e., from a posterior confluence into a vertical vein, inferior vena cava, and then right atrium). The patient must have an interatrial shunt in order to fill the left atrium and ventricle and provide systemic blood flow. The vertical veins of the supracardiac and infracardiac drainage patterns have a high likelihood of becoming compressed as they pass through the body, leading to obstruction of pulmonary venous blood return. The repair of total anomalous

pulmonary venous return involves creating an anastomosis between the confluence and the left atrium, which lies immediately anterior to it. The anomalous pathway is then ligated. Anatomic evaluation. Normal pulmonary venous return into the left atrium is best seen in the suprasternal notch coronal view (and parasternal short-axis and apical views) with color Doppler. Supporting evidence for anomalous drainage is a large right ventricle and small left ventricle. If normal drainage is not seen, then anomalous pulmonary venous return must be suspected, and a thorough search for possible pathways is undertaken. Demonstrating anomalous drainage can be difficult. The examination usually requires an aggressive use of color Doppler combined with good 2D imaging techniques. At times, the confluence behind the left atrium may be the first part of the pathway identified. This can be seen in parasternal long-axis, parasternal short-axis, apical, and subcostal views. At other times, the vertical vein is the first feature of the pathway to be demonstrated. Once identified, the suprasternal or subcostal views will demonstrate how the vertical vein courses. These views can also demonstrate how the pulmonary veins drain into the confluence. The size of the ASD should also be assessed since these patients need an adequate ASD to fill the left heart. Physiologic evaluation. The physiologic evaluation should focus on a thorough Doppler examination of the anomalous pathways. Infracardiac and supracardiac drainage can become obstructed, which is easily demonstrated by Doppler techniques. The gradient through the ASD should be assessed with Doppler. Right ventricular pressure should be measured. Postintervention examination. After repair, potential obstruction at the anastomosis of the confluence to the left atrium is best evaluated from the apical or subcostal windows with color and pulsed Doppler techniques. Potential obstruction at the pulmonary vein ostia is evaluated in the same views. A third complication is the development of pulmonary veno-occlusive disease. This is a diffuse narrowing of the pulmonary veins and is universally fatal. It cannot be diagnosed by echocardiography, but it can be suspected if the right ventricular pressure by tricuspid insuf-

230

JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 VOL. 21, NO. 3

ficiency jet is high due to pulmonary hypertension, in the setting of an unobstructed repair.
PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

General considerations. Pulmonary atresia with an intact ventricular septum is also referred to as hypoplastic right heart syndrome. In this lesion, the pulmonary valve has not formed. Instead, there is a fibrous plate prohibiting the passage of blood from the right ventricle to the pulmonary artery. Systemic venous blood entering the right atrium can course through the tricuspid valve into the right ventricle, but since there is no antegrade flow possible, severe tricuspid insufficiency is present. The right ventricle is a high-pressure, thick, small chamber. The right atrium is often dilated. Pulmonary blood flow is provided by a patent ductus arteriosus. Since the right ventricle is under such high pressure, it may develop coronary artery fistula for decompression. In this situation, blood in the right ventricle enters the coronary arterial system and courses retrograde through the coronary arteries, spilling out through the coronary ostia and proximal ascending aorta. The neonatal management is first to initiate prostaglandins to maintain ductal patency. Following this, the management decision focuses on whether the right ventricle is useful. Often, the right ventricle is so hypoplastic that it is not useful, and the patient is destined for a single ventricle repair. In this instance, a Blalock-Taussig shunt is placed. However, in some cases, there is some intrauterine right ventricular development so that it is suitable for a biventricular repair. In these instances, further right ventricular growth can be fostered by opening the pulmonary valve either through a balloon valvuloplasty or, more commonly, through a surgical valvotomy. Anatomic evaluation. Pulmonary atresia is evident in the parasternal short-axis view. Frequently, pulsed Doppler is necessary to confirm that there is no flow across the fibrous plate at the pulmonary annulus. In the apical four-chamber view, the size of the right ventricle can be assessed. Particularly important are the size of the tricuspid annulus diameter, whether the right ventricle is apex forming, and whether the right ventricle has

three parts (inflow, body, and outflow). In patients with very hypertensive right ventricles, color Doppler should be used in the apical four-chamber and subcostal views to investigate for coronary artery fistula. Physiologic evaluation. The tricuspid insufficiency jet can be used to obtain the right ventricular pressure. The ASD gradient can be assessed by Doppler. Right ventricular volume may need to be calculated to determine its suitability in a biventricular repair. Postintervention examination. If the patient undergoes a shunt and single ventricle repair, the issues during and after the surgical stages remain the same as those in the hypoplastic left heart syndrome. If the patient is undergoing a procedure to open the pulmonary valve, then the degree of antegrade flow and residual stenosis, as well as severity of pulmonary insufficiency, needs to be assessed. In addition, the growth of the right ventricle needs to be assessed serially.
EBSTEIN ANOMALY

General considerations. Ebstein anomaly is a condition in which the tricuspid valve is tethered to the septum and right ventricular free wall. The hinge points of the tricuspid valve leaflets are, therefore, displaced apically and do not coapt completely, resulting in (1) tricuspid insufficiency and (2) atrialization of the right ventricle. The severity of Ebstein anomaly varies from mild (with very little tethering and minimal tricuspid insufficiency) to severe (with marked tethering and severe tricuspid insufficiency). In these cases, the tricuspid valve may interfere with right ventricular outflow, and the atrialization of the right ventricle may be so extensive that there is insufficient right ventricular myocardium to pump blood to the pulmonary arteries. Marked atrial right-to-left shunting occurs. The treatment for severe Ebstein anomaly is somewhat controversial. In the newborn period, efforts are made to lower pulmonary vascular resistance so that antegrade pulmonary blood flow is enhanced. Surgically, some advocate oversewing the tricuspid valve and placing a shunt so that the physiology mimics tricuspid atresia. Anatomic examination. The diagnosis is established in the apical four-chamber and sub-

PEDIATRIC ECHOCARDIOGRAPHY / Witt

231

costal views, where the tricuspid valve displacement can be assessed. In the parasternal short-axis and subcostal views, the right ventricular outflow tract needs to be imaged to see if there is an obstruction from the displaced anterior leaflet. The size of the pulmonary arteries can be assessed in the parasternal short-axis view. Physiologic examination. The degree of tricuspid insufficiency should be assessed in the apical four-chamber and subcostal views. The degree of right ventricular outflow tract obstruction can be assessed in the parasternal short-axis and subcostal sagittal views. The direction and amount of shunting across the ASD can be evaluated in the subcostal coronal view. Postintervention examination. If the patient has severe Ebstein anomaly and is undergoing oversewing of the tricuspid valve and shunt placement, the postoperative evaluation should focus on residual leaks across the tricuspid valve, the presence of a gradient across the ASD, and shunt patency. These patients will need serial evaluations to assess pulmonary artery size in preparation for an eventual single ventricle repair.

proving patient care, lowering patient costs, and enhancing patient survival.

REFERENCES
1. 2.

3.

4.

5.

6.

7.

Conclusion
In the field of pediatric cardiology, echocardiography is the most powerful diagnostic tool. It has become the cardiac catheterization of the 21st century, giving exquisite anatomic detail and accurate physiologic information. This has obviated the need for some invasive procedures, thereby im-

8. 9.

10.

Perloff JK: Clinical Recognition of Congenital Heart Disease. Philadelphia, W. B. Saunders, 1987. Sabry AF, Reller MD, Silberbach GM, Rice MJ, Sahn DJ: Comparison of four Doppler echocardiographic methods for calculating pulmonary-to-systemic shunt flow ratios in patients with ventricular septal defect. Am J Cardiol 1995;75:611614. Ussia GP, Momenah TS, Ursell P, et al: Evaluation of the morphology of the oval fossa for placement of devices. Cardiol Young 2000;5:502509. Fesslova V, Villa L, Nava S, Boschetto C, Redaelli C, Mannarino S: Spectrum and outcome of atrioventricular septal defect in fetal life. Cardiol Young 2002;12:1826. Barker PC, Ensing G, Ludomirsky A, Bradley DJ, Lloyd TR, Rocchini AP: Comparison of simultaneous invasive and noninvasive measurements of pressure gradients in congenital aortic valve stenosis. J Am Soc Echocardiogr 2002;15:14961502. Allen HD: Moss and Adams Heart Disease in Infants, Children & Adolescents. Vols. 12. Baltimore, MD, Lippincott Williams & Wilkins, 2001. DeGroff CG, Orlando W, Shandas R: Insights into the effect of aortic compliance on Doppler diastolic flow patterns seen in coarctation of the aorta: a numeric study. J Am Soc Echocardiogr 2003;16:162. Snider AR: Echocardiography in Pediatric Heart Disease. St. Louis, MO, Mosby, 1997. Musewe NN, Smallhorn JF, Moes CA, Freedom RM, Trusler GA: Echocardiographic evaluation of obstructive mechanism of tetralogy of Fallot with restrictive ventricular septal defect. Am J Cardiol 1988;61:664668. Morris DC, Felner JM, Schlant RC, Franch RH: Echocardiographic diagnosis of tetralogy of Fallot. Am J Cardiol 1975;36:908913.

232

JDMS 21:232233 May/June 2005

OTHER JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 JDMS 21:232233 May/June 2005 VOL. 21, NO. 3

JDMS 21:232233 May/June 2005 10.1177/8756479305277576 OTHER JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 JDMS 21:000000 May/June 2005 10.1177/8756479305277577 OTHER JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY May/June 2005 JDMS 21:000000 May/June 2005 VOL. 21 NO. 3 VOL. 21 NO. 3

SDMS-JDMS CME TEST


Article: Pediatric Echocardiography Author: Sandra A. Witt, RDCS, FASE Category: Pediatric Echocardiography (PE) Credit: 2.0 Objectives: After studying the article titled Pediatric Echocardiography, you will be able to 1. Describe characteristics of multiple congenital anomalies, including Ebstein anomaly, septal defects, coarctation of the aorta, tetralogy of Fallot, hypoplastic left heart syndrome, valvular stenosis, and total anomalous pulmonary venous return. 2. Specify considerations for the use of apical, parasternal, subcostal, and suprasternal acoustic windows. 3. Analyze the rationale for Doppler quantification in pediatric echocardiography. 4. Describe measurement standards in pediatric echocardiography. 5. Provide an example of nonsurgical repair of congenital heart anomaly. 6. Analyze systemic blood flow patterns in selected congenital heart diseases. 1. The acoustic window that is the most uncomfortable for patients and typically imaged last during a pediatric echocardiography examination is the _______________ window. a. apical b. parasternal c. subcostal d. suprasternal 2. The acoustic window that is best for determining cardiac position and situs is the ________________ window. a. apical b. parasternal c. subcostal d. suprasternal 3. Doppler quantification may noninvasively provide critical information in pediatric patient management. The critical information includes all but which of the following? a. valve areas b. tricuspid insufficiency c. right heart pressures d. valve gradients 4. The size of a ventricular septal defect is measured relative to the size of the a. mitral valve annulus b. aortic root c. ductus arteriosus d. pulmonary artery 5. ______________ atrial septal defects may be closed with transcatheter (nonsurgical) methods. a. secundum b. primum c. stenotic d. sinus venosus 6. A pathology that does not require a patent ductus arteriosus (or left-to-right shunt) for systemic or pulmonary circulation is a. tricuspid atresia b. pulmonary stenosis c. coarctation of the aorta d. truncus arteriosus 7. One of the most common cyanotic lesions in the newborn is a. hypoplastic left heart b. transposition of the great vessels c. ventricular septal defect d. tricuspid atresia 8. Which congenital heart anomaly is characterized by an overriding aorta? a. ventricular septal defect b. tetralogy of Fallot c. tricuspid atresia d. hypoplastic left ventricle 9. Ebstein anomaly is characterized by a. pulmonary insufficiency b. atrialization of the right ventricle c. atrial left-to-right shunting d. tricuspid tethering to the left ventricular wall 10. Which of the following pathologies is not associated with right-sided heart enlargement? a. ventricular septal defect b. pulmonary stenosis c. coarctation of the aorta d. total anomalus pulmonary venous return