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All of these ing and training aspect of the department buy cheap diltiazem line symptoms internal bleeding, and oversight of factors can lead to failure of a congenital heart program generic diltiazem 60 mg free shipping symptoms nausea. Caring for the Patient and Family with Congenital Heart Disease 5 rebUilding the fAiled congenitAl heArt progrAm There are challenges buy diltiazem 60 mg online medicine and science in sports and exercise, however discount diltiazem 180 mg on line medications like zoloft, in developing a heart institute structure. The chief of surgery and/or the chief of Once failure of a congenital cardiac program has occurred, pediatrics may be unwilling to cede the power or revenue there are three main directions in which the failed program involved, and this can be exacerbated by a fscal segregation can move in order to restructure (Box 1. Hospital adminis- First, the congenital cardiac surgical program can be con- tration may not be willing to change and break with tradition, solidated into a major regional center with maintenance of the the chief of cardiology may be unwilling to share prestige, local cardiology program and with total outsourcing of surgi- power, and dollars with a junior surgeon, and the political cal patients. Second, a heart institute structure can be devel- inexperience of a junior surgeon advocating within the heart oped with a horizontal political administration rather than a institute structure may lead to instability. Finally, administrator model, which has been pioneered by Frank there can be oversight by a traveling senior surgeon. The senior of a surgical program, include (1) the presence of a successful traveling surgeon recruits less experienced surgeons who regional center within 100 miles and within the same state, remain on site and perform simple and moderate complex- (2) that it is the preference of the cardiologists and intensivists ity cases alone. The senior surgeon acts as gram, (4) the lack of a strong medical school, and (5) specifc a surgical administrator and advocate for the pediatric pro- challenges, whether they be geographic, cultural, or fnancial, gram and performs more complex cases on site. The challenge outsourcing usually does not happen principally because of for the traveling surgeon scenario is the stigma of itinerant fnancial issues. In addition, there are the home and important factors of regional pride as well as the desires of family stresses of frequent travel. With this structure, the most senior adminis- AmericAn medicAl AssociAtion trators within the group advocate for cardiac surgery in a way that is not possible for a chief of surgery or chief of cardiac The stated mission of the American Medical Association is surgery, who have too many competing allegiances with sur- to “promote the art and science of medicine for the better- geons from other specialties. This is a particular problem if ment of the public health, to advance the interests of physi- the pediatric cardiac surgical program is administered with cians and their patients, to promote public health, to lobby for an adult cardiothoracic surgery program, where revenue and legislation favorable to physicians and patients, and to raise volume (but not income expectations) have declined substan- money for medical education. In the United States, the role of spe- cardiothoracic surgeons including congenital cardiac sur- cialty certifcation is taken by the American Board of Medical geons. The American Board of Thoracic Surgery over- stringent requirements including the need to have made a sees certifcation of general cardiothoracic surgery and the signifcant contribution to the specialty. Most members have recently developed subspecialty certifcation in congenital published at least 40 or 50 scientifc papers and are usually cardiac surgery. There a non-government nonproft organization separate from the is an annual scientifc meeting, usually in early May, at which Accreditation Council for Graduate Medical Education that some of the most important research breakthroughs are pre- oversees training positions in teaching hospitals. It member and supporting letters of recommendation by at least plays a strong political advocacy role including a perma- two additional surgeon members. The two major cardiology societies play important roles in advocating for patients with heart disease and increasing aware- ness of heart disease in the community. The American Heart congenitAl heArt sUrgeons’ society Association has an important focus on fundraising and support The Congenital Heart Surgeons’ Society began as a small of research. Both societies have annual scientifc meetings and club that met annually at a Chicago airport hotel. They senior surgeons in the emerging feld of congenital cardiac each have small subspecialty “councils” representing pediatric surgery to discuss challenging cases in an informal atmo- heart disease and cardiac surgeons, but they are overwhelm- sphere. The society has grown to more than 100 members ingly about acquired heart disease in adults and adult cardi- representing all of the major congenital cardiac surgical ologists. Membership requires nomination, letters of reference, programs in North America as well as a small number of and review by a membership committee. Membership requires a signifcant contribution to the feld through clinical activity, teaching of thorAcic sUrgery societies: AmericAn surgical trainees, and research. The society maintains a data center, formerly at the University of Alabama where it was maintained by Dr. John Kirklin (a) not only pioneered many cardiac surgical procedures, but was also responsible for establishing the data center of the Congenital Heart Surgeons Society currently located in Toronto, Canada. He was very ably assisted by his long-term colleague at the University of Alabama, Dr. Eugene Blackstone (b), who has been instrumental in developing rigorous statistical analysis of cardiac surgical outcomes. Constantine “Gus” Mavroudis, formerly from Chicago and now from Orlando, Florida, and (c) Dr. Bove (front row center), Austin (back row right), and the author are pictured with their wives at the gala dinner in Warsaw, Poland, of the combined meeting in 2008 organized by Dr. The society meets every fourth year with the European Congenital Heart The World Society brings together congenital cardiac sur- Surgeons Association (Fig. The society publishes the only journal exclu- pital’s administrative structure as well as advocating for the sively devoted to surgery for congenital heart disease, the care of patients with heart disease both in the local commu- World Journal for Pediatric and Congenital Heart Surgery, nity and globally. The print journal is available through membership, while the online journal is presently available for no charge. Prevalence of congeni- that cares for the child or adult with congenital heart disease. The preoperative procedure in the adult may take many hours and demand physical stamina and Training for congenital heart surgery is probably longer endurance as well as strength. Muscle strength and stamina and more demanding than that for any other surgical sub- are not things that just happen. The intensity of training refects the many chal- geon’s schedule, there must be time to maintain physical ft- lenges of the specialty. There is no question Many congenital surgical procedures must be performed that certain individuals can master the necessary skills more under time pressure. Application of the aortic cross-clamp easily than others, but fundamentally these are skills that can starts the clock ticking and requires that the procedure be be taught and learned. Thus, the surgeon must cialty requires considerable depth of knowledge and a wide carefully plan and sequence the procedure so that it is com- range of personal strengths and stamina. The surgeon must aim for a There are emotional and psychological challenges in all meticulously correct procedure and be self-critical of the surgical specialties, but congenital heart surgery presents its result. It is heartbreaking to witness the shock and may require that reasonable compromises be accepted. But to be a part of the same experience with a child- of good” must be understood and practiced by every congeni- less couple who may have tried for years to achieve pregnancy, tal cardiac surgeon. It is said that While there are many others in the room who are responsi- the death of a child is the single greatest tragedy that anyone ble for critically important tasks, it is ultimately the surgeon can face in a lifetime. The congenital heart surgeon bears wit- who must tell the family if the child has not survived, and ness to the truth of these words many times in a career. On the it is the surgeon to whom the family has directly entrusted other hand, there is no greater reward than to be able to save a their child. Thus, the surgeon must constantly monitor the child from the certain death threatened by a serious congeni- performance of all team members, particularly those who are tal cardiac malformation. The surgeon must monitor the status of the knowledge that one has made every possible effort to do one’s patient and the perfusion conditions, keep track of ischemic best for every child, that allows the congenital cardiac surgeon time, plan the next steps in the procedure, as well as focus on to carry the weight of those who do not survive. A wide hearing what one is not listening to and seeing what one is range of patient size is encountered.

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Continuous wave Doppler assessment during several consecutive cardiac cycles generates a signal from which the mean gradient through the fenestration can be calculated (Fig cheap 180mg diltiazem amex medicine lodge treaty. The normal gradient should be 5 to 8 mm Hg and is primarily determined by pulmonary vascular resistance cheap 60mg diltiazem fast delivery medicine 5658. An elevated gradient should raise concern about Fontan pathway stenosis purchase discount diltiazem on-line medicine bow, intrinsic pulmonary resistance issues discount 60mg diltiazem overnight delivery medicine quiz, lung disease, or pulmonary venous obstruction. Large flow reversals that are “cannon A waves” are present in the pulmonary arteries and hepatic veins. Obstruction in the Fontan pathway and branch pulmonary arteries can be identified with two-dimensional and Doppler echocardiography. The importance of defining the size of the pulmonary artery/Fontan lumen cannot be overstated. Given the low pressure, nonpulsatile nature of “Fontan flow” very narrow stenoses may generate relatively small pressure differences. Evaluation of pulmonary artery flow with color Doppler requires reducing the Nyquist limit to 30 to 40 cm/s or less. In order to define a Doppler derived pressure gradient in these patients, one must account for respiratory variation. A: An intra-atrial conduit (C) diverted inferior vena caval flow to the pulmonary artery. The mean gradient between the conduit and the pulmonary venous atrium was 6 mm Hg, representing a relatively normal transpulmonary gradient after Fontan completion. These signals were obtained from the right pulmonary artery (A) and the hepatic vein (B) of a patient after an extracardiac Fontan operation. These flow patterns are not normal, primarily because of the abnormal cardiac rhythm that is present. Since atrial contractions are dyssynchronous in this rhythm, they will produce significant elevations of pulmonary venous pressure (cannon A waves). These pressure increases are reflected not only in the pulmonary arterial flow signal, but are also transmitted all the way back to the hepatic veins (even though there is no “atrium” within the systemic venous pathway). A: yellow arrows: Reductions in forward flow velocity caused by atrial contraction. One of the atrial contractions during this recording occurred so early (white arrow) that it actually caused flow reversal in the pulmonary artery. This phenomenon was much more evident when the hepatic venous flows were recorded (B). Cannon A waves with large flow reversals (white arrows) could be seen during nearly every cardiac cycle (yellow arrow). The one cardiac cycle in this recording in which atrial contraction occurred at approximately the “correct” time are relative to ventricular contraction. In this cardiac cycle, there was a slight decrease in forward velocity after atrial contraction, but no reversals were observed. The best method of determining the presence of or absence of stenosis within a Fontan pathway is to directly visualize the vessels involved. Analysis of venous Doppler flow patterns can provide clues regarding the status of downstream venous connections to the pulmonary circulation. Even respiratory variation is absent; this is extremely abnormal in the venous circulation. The middle (2) and bottom (3) signals were obtained from patients with less obstruction. There is some respiratory variation and augmentation of forward flow during ventricular diastole. There are no reversals seen, but there is respiratory variation and appropriate reductions and augmentations to forward flow not only during ventricular diastole, but also with left atrial contraction and relaxation. Evidence of atrial activity in the vena caval flow pattern again confirms the patency of the connection in this case. Elevated systemic venous pressure in the Fontan circuit will be identified by reduced expiratory forward flow in the hepatic veins. Complete imaging of the pulmonary circulation after Fontan operation requires more than echocardiography especially in the older child, adolescent, and adult. However, even when image quality is suboptimal, there are clues to Fontan failure that can be assessed: 1. Reduction in ventricular contractility and/or worsening diastolic function Volumetric analysis, fractional area change, tissue Doppler, and myocardial strain data have been utilized in a quantitative manner 3. Assessment of anatomic pathway stenosis Small gradients in the Fontan circuit and branch pulmonary arteries are important Altered flow patterns can reflect diastolic dysfunction or dysrhythmia 4. Calculation of the transpulmonary gradient by measuring the “average” mean fenestration gradient over several cardiac cycles 5. Middiastolic deceleration time was relatively normal (185 ms), but there was a prominent middiastolic L wave (white arrow), indicating significantly abnormal/delayed left ventricular relaxation. It shows extremely low- velocity diastolic forward flow into a prominent, prolonged atrial reversal (yellow arrows). In fact, the reversal duration was 60 ms longer than the atrial forward flow duration. These findings are consistent with reduced diastolic ventricular compliance and significantly elevated ventricular end-diastolic and mean left atrial pressures. This type of bradycardia may be poorly tolerated in the face of such severe ventricular diastolic dysfunction. There is severe compression of the right lower pulmonary veins (arrow) by the massive right atrium. No adverse effect from prior pulmonary artery operation Outcomes after Fontan Operation Survival Reports of outcomes after Fontan operation include all patients with functional single ventricle and may not be representative of only patients with tricuspid atresia. In general, results of the Fontan operation for patients with tricuspid atresia are superior to those for other forms of functional single ventricle. Operative survival (<30 days) was 80% to 85% in early series and has increased to 95% in more recent studies (Fig. For patients with tricuspid atresia operated between 1988 and 1997, the operative mortality was only 2%. Early and late mortality were higher for patients operated in the 1970s and 1980s than for patients operated more recently (24,25,26,27,28,29,30,31,32,33,34,35). Also, some of the risk factors for poor outcome, such as hypoplastic left heart syndrome (26) are now apparent. Decremental survival is demonstrated regardless of lesion, era, or institution(s) during 5-, 10-, and 15-year follow-up. Choussat and Fontan (21) recommended 10 criteria for a successful low-risk Fontan operation (Table 38. These are relative criteria and it is clear that some are more important than others. For example many investigators have studied the effect of young age (<2 years or <4 years) upon survival.

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If the child return with obstruction this must be repaired as early in life is duct dependent purchase 60mg diltiazem symptoms breast cancer, prostaglandin E1 should be continued and as possible buy diltiazem 180 mg with amex treatment junctional tachycardia. If there is restriction at the level of the atrial sep- the distal anastomosis of the shunt should be placed on the tum in the setting of obligatory left to right fow at the atrial branch pulmonary artery contralateral to the site of ductal septal level then the atrial septum must be surgically excised insertion (Fig 180mg diltiazem free shipping medicine 013. Balloon atrial septostomy is not nosed safe 180mg diltiazem k-9 medications, for example an arterial oxygen saturation of less than adequate in this setting. It is important that the approach is through a Surgery for Inadequate Pulmonary Blood Flow median sternotomy and not through the more traditional tho- Caused by Pulmonary Outfow Obstruction 48 racotomy approach though there is a greater risk of exces- By far the commonest cause of inadequate pulmonary blood sive pulmonary blood fow when using this approach. The fow in the neonate or young infant with single-ventricle most important advantage of the sternotomy approach for the physiology is obstruction to pulmonary outfow. If the ductus patient with a single ventricle is that dissection of the pul- has closed and if the arterial oxygen saturation is consistently monary arteries occurs very centrally so that distortion of less than 70–75%, then the neonate should undergo place- the lobar branches is highly unlikely to result from the sur- ment of a systemic to pulmonary arterial shunt. The sternotomy approach has the cosmetic advantage of a single incision for all three stages of surgical palliation. Potential Disadvantages of a Systemic There may be a lower risk of thoracotomy-induced scoliosis. Pulmonary Arterial Shunt In our experience, the incidence of Horner’s syndrome is also The most important risk of a systemic to pulmonary arte- decreased with the sternotomy approach. The growth potential, such as the classic Blalock shunt, Waterston mechanism is most likely a positive feedback loop in which shunt, or Potts shunt. All of these shunts can result in a pro- the low diastolic pressure resulting from pulmonary blood gressive increase in pulmonary blood fow over time. They fow throughout the cardiac cycle results in a fall in cardiac also have a higher risk of distortion of the pulmonary arteries output which further reduces diastolic pressure and coronary relative to the modifed Blalock shunt which uses a Gore-Tex perfusion. Typically, The shunt should be anastomosed to a central pulmonary artery where it is anticipated that the subsequent Fontan the child will have an oxygen saturation of greater than 83 anastomosis will take place. It can be very diffcult to resuscitate a neonate in this pulmonary artery which results from the shunt can be incor- setting with cardiopulmonary resuscitation alone. Excessive shunt size can also result over time in an the site selected for the proximal anastomosis are critically increase in pulmonary vascular resistance. A shunt can also important in avoiding a low diastolic pressure in the early result in scarring and distortion of the pulmonary arteries, postoperative period with a risk of acute cardiac arrest as and if performed through a thoracotomy is likely to cause well as longer-term excessive volume loading of the single distortion of the lobar branches. For the average-sized neonate weighing between 3 488 Comprehensive Surgical Management of Congenital Heart Disease, Second Edition L. The proximal anastomosis is placed to a longitudinal arteriotomy in the origin of the right subcla- vian artery, which is controlled with a side-biting clamp. After construction of the proximal anastomosis the shunt is threaded posterior to the left innominate vein. This will also serve to lengthen the shunt which also to the left or right side so that at a subsequent procedure a helps to reduce the volume loading of the ventricle as origi- patch of anterior pericardium can be harvested if necessary. However, a child presenting at a weight of 5 dissected free being careful to avoid injury to the right recur- or 6 kg is likely to be several months old. If there is a suff- rent laryngeal nerve which can be seen passing around the cient degree of cyanosis that a systemic to pulmonary arterial distal right subclavian artery. A side-biting clamp is applied shunt has to be inserted then it is probable that the child has across the origin of the right subclavian artery and the distal suffciently low pulmonary resistance by that age, for exam- innominate artery. A longitudinal arteriotomy is made either ple beyond 2–3 months, that a more appropriate management partially or completely on the right subclavian artery depend- strategy would be to proceed directly to a bidirectional Glenn ing on the size of the child. A The child should be positioned supine with as much exten- direct anastomosis is fashioned. It is not dard median sternotomy incision is performed with the skin essential to administer heparin during the clamping period as incision extending at least to the top of the sternal notch. Even though it is possible to perform a strict policy regarding heparin administration and often Three-Stage Management of Single Ventricle 489 administer 1 mg/kg before clamp application if the neonate’s right atrial line to maintain a systolic pressure of at least coagulation appears normal. This will help to maintain shunt fow and reduce Following completion of the anastomosis the shunt is con- the risk of platelet deposition, particularly at the proximal trolled with a bulldog clamp that is applied close to the anas- anastomosis. A side-biting clamp is tionally smaller than expected, that is the oxygen saturation applied to the right pulmonary artery with appropriate rota- is less than 75% and there is little change in blood pressure tion of the vessel so that an incision can be made on the supe- or heart rate at clamp release. The anastomosis is fashioned once again with ing of the anastomoses and the shunt itself is often helpful. Upon release of the clamps there should If heparin was not administered before clamp application it be a decrease in diastolic pressure and an increase in arterial can be given now, usually in a dose of 1 mg/kg. A chest tube, both anastomoses have been done as well as possible and usually a 15 Fr soft silicone four-channel drain (e. The sternotomy incision is closed tiny incision is made in the middle of the shunt. A 3 mm Fogarty embolectomy catheter is passed Shunt Complications: Excessive Pulmonary Blood Flow proximally and infated and withdrawn. There should be an Opening the clamp on a systemic to pulmonary arterial shunt abrupt increase in fow out of the incision in the shunt and an results in an acute increase in volume work for the single increase in oxygen saturation. Balloon dilation of platelet ration is high following clamp release, for example greater or fbrin accumulation is usually helpful. Sometimes it is than 85% and if a metabolic acidosis begins to build up, con- necessary to place a small stent to optimize shunt function. Cardiac catheterization may also diagnose an unanticipated Often the simplest way to do this is to ligate the ductus rather problem such as discontinuity of the pulmonary arteries that than waiting for the duct to close following prostaglandin has been missed by the echocardiographers. Ligation of the duct also avoids competitive consequence of ductal closure that has occurred after with- fow which probably increases the risk of shunt thrombosis. However, if the duct is not ligated and if the shunt does sub- sequently thrombose, there is a possibility of resuscitating Surgery for Inadequate Pulmonary Blood the child through administration of prostaglandin. Although Flow Caused by Obstructed Total Anomalous there is no clear answer to this conundrum it is our usual Pulmonary Venous Connection practice to ligate the ductus when it is clearly large or if there is an additional source of pulmonary blood fow, for example Neonates with heterotaxy may present with a profound degree the child with forward pulmonary outfow through a stenotic of cyanosis because of obstructed total anomalous pulmo- pulmonary outfow. We have children also tend to have branch pulmonary artery stenoses used various techniques including hemoclips and Gore-Tex at the origin of either the right or left pulmonary artery or suture. Probably a good general principle to bear in mind is even discontinuity of the right and left pulmonary arteries. In that a gentle taper should be achieved rather than an abrupt order to optimize pulmonary artery development and to min- diameter change. There is no question that narrowing the imize pulmonary vascular resistance it is important that the shunt by any technique will introduce an increased risk of total anomalous pulmonary connection be repaired shortly acute shunt thrombosis at a later time. Pulmonary blood must be Shunt Complications: Shunt Thrombosis established with a systemic to pulmonary arterial shunt when Following clamp release there should be an acute rise in there is associated obstruction to pulmonary outfow. The oxygen saturation as well as a decrease in systolic and par- procedure for repair of total anomalous pulmonary venous ticularly diastolic pressure. Generally the repair, that is anastomosis tourniquet (similar to a cardioplegia infusion site) is created of the pulmonary venous confuence to the posterior wall of at the highest point of the mid-ascending aorta. An appro- the common atrium, will be performed under deep hypo- priate side-biting clamp (nested clamp is ideal) is placed thermic circulatory arrest. An incision is made systemic to pulmonary arterial shunt can be performed with in the controlled segment of atrium. A suture line is begun at the application of side-biting clamps as described above.

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End-systolic stress-velocity and pressure-dimension relationships by transthoracic echocardiography in mice cheap 180mg diltiazem with visa symptoms bladder cancer. Comprehensive transthoracic cardiac imaging in mice using ultrasound biomicroscopy with anatomical confirmation by magnetic resonance imaging generic diltiazem 180mg with visa symptoms pneumonia. Differences between echocardiographic measurements of left ventricular dimensions and function by local investigators and a core laboratory in a 2-year follow-up study of patients with an acute myocardial infarction buy cheap diltiazem on line medications rights. Use of hand-carried ultrasound devices to augment the accuracy of medical student bedside cardiac diagnoses purchase genuine diltiazem online permatex rust treatment. Physician-performed point-of-care echocardiography using a laptop platform compared with physical examination in the cardiovascular patient. Usefulness of a hand-carried cardiac ultrasound device to detect clinically significant valvular regurgitation in hospitalized patients. Will a handheld ultrasound scanner be applicable for screening for heart abnormalities in newborns and children? Use of a hand-carried ultrasound device by critical care physicians for the diagnosis of pericardial effusions, decreased cardiac function, and left ventricular enlargement in pediatric patients. Impact of telemedicine on the practice of pediatric cardiology in community hospitals. Application of a low cost telemedicine link to the diagnosis of neonatal congenital heart defects by remote consultation. Effectiveness of echocardiographic imaging by nurses to identify left ventricular systolic dysfunction in high-risk patients. Hand-carried cardiac ultrasound enhances healthcare delivery in developing countries. This paper identifies indications for outpatient pediatric echocardiography and graded “appropriateness” level for echocardiography in each indication. The impact of procedural sedation on diagnostic errors in pediatric echocardiography. These two papers investigate the factors that lead to diagnostic errors in pediatric echocardiography. The authors conclude that sedation is associated with lower likelihood of errors and that overnight or weekend studies are risk factors for poor quality. Exercise echocardiography demonstrates biventricular systolic dysfunction and reveals decreased left ventricular contractile reserve in children after tetralogy of Fallot repair. Linking pediatric obesity to subclinical alterations in cardiac structure and function. Friedberg Quantification of Chamber Dimensions and Cardiac Structures Accurate measurements of valves, chambers, and vessels are essential to the diagnosis and management of patients with congenital and pediatric heart disease. The adult guidelines were recently revised to include three- dimensional (3-D) echocardiography and myocardial deformation imaging (4). One of the important differences between measurements in adult and pediatric patients is the effect of growth or body size on measurements. To overcome this limitation, the use of z-scores has been proposed as a practical alternative (5). Z-scores for different cardiovascular structures have been published (6,7) but the effect of gender and race on cardiovascular measurements may necessitate establishing normal values based on population mix seen in a specific laboratory. Detailing the measurement of each individual cardiovascular structure is beyond the scope of this chapter. In this chapter, the evaluation of cardiac function and chamber quantification are discussed in more detail. M-mode measurements have a very high temporal resolution albeit at the expense of low spatial resolution. If the measurements are not well standardized, M-mode measurements can be highly variable. This study showed poor agreement between the core laboratory and local laboratory measurements with relatively wide limits of agreement. Especially at high heart rates, the identification of the frame representing end systole and end diastole can be more variable. Another limitation is that currently the majority of published normal z-score data are based on M-mode measurements and not on 2-D imaging. M-mode measurement is obtained from a parasternal short-axis view of the left ventricle just inferior to the mitral valve leaflets. Three-dimensional echocardiography overcomes this problem as full 3-D volumetric data sets can be acquired including the entire ventricle. This results in improved reproducibility as it eliminates observer bias in determining endocardial borders. Hence, the intra- and interobserver variability of 3-D methods are much lower compared to 2-D-based volumetric calculations. In pediatrics, a limitation of 3-D echocardiography is the lower temporal resolution related to the relative low volume rates. Additionally not every vendor has a high-frequency 3-D probe available, which can limit temporal resolution especially in smaller children. The first two images from the left show the apical four-chamber view in end diastole and end systole, respectively. The next two images are obtained from the apical two-chamber view also in end diastole and end systole, respectively. The formula cannot be used in patients with asymmetric hypertrophic cardiomyopathy. Various measurements obtained from the apical four-chamber view have been proposed (Fig. A problem with these measurements is the lack of normal pediatric data precluding calculation of z-scores. Studies have shown that good quality 3-D data sets can only be obtained in 55% to 75% of patients after tetralogy of Fallot repair (20,21,22). Studies have suggested that the larger the ventricle, the more important the underestimation. This is probably related to difficult visualization of the endocardial borders related to the coarse trabeculations. Quantification of the Univentricular Heart Assessing chamber dimensions of the univentricular heart can be challenging due to the variable morphology. First, it is important to know whether the dominant ventricle has left, right, or indeterminate morphology. Moreover, the type of palliation and associated volume loading will influence ventricular size. A shunted single ventricle will generally be larger compared to the same ventricle after total cavopulmonary anastomosis. Three-dimensional volumes can also be calculated based on full volumetric data sets. Evaluation of Systolic Ventricular Function When evaluating systolic function, it is important to consider the different levels (fiber, segment, or ventricle) evaluated by the various functional indices.