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Use of rapid- • Single-ventricle patients continue to pose a signif- deployment extracorporeal membrane oxygenation for the cant challenge best nizagara 50mg impotence may be caused from quizlet. Development of an impeller pump on 84 resuscitation of pediatric patients with heart disease after car- the Von Karman principle offers hope for mechan- diac arrest nizagara 50 mg visa impotence genetic. Extracorporeal children of all ages with a minimum of associated membrane oxygenation for bridge to heart transplanta- complications. Outcomes of and mortality of heart failure-related hospitalizations in chil- pediatric patients bridged to heart transplantation from dren in the United States: a population-based study. Preoperative extracorporeal membrane oxygenation as after extracorporeal membrane oxygenation use to aid pedi- a bridge to cardiac surgery in children with congenital heart atric cardiopulmonary resuscitation. Optimizing patient resuscitation outcomes children requiring repeat extracorporeal membrane oxy- with simulation. Outcomes of second- citation performance during simulated cardiac arrest in run extracorporeal life support in children: a single–institution nursing student teams. J Thorac Cardiovasc technique to prevent limb ischemia during veno-arterial Surg 2008;136:976–83. Pediatr Crit Care Med after common femoral artery cannulation for venoarterial 2012;14:428–34. Survival outcomes assist device support with a centrifugal pump for 2 months in after rescue extracorporeal cardiopulmonary resuscitation a 5-kg child. Post-cardiotomy (accessed September 2012) extracorporeal cardiopulmonary resuscitation in neonates 41. Outcomes of chil- after extracorporeal cardiopulmonary resuscitation in infants dren bridged to heart transplantation with ventricular assist and children with heart disease. J Heart Lung Transplant corporeal cardiopulmonary resuscitation for refractory 2000;19:121–6. Pediatric Extracorporeal Life Support: Extracorporeal Membrane Oxygenation and Mechanical Circulatory Support 119 44. Ann dren of all sizes to cardiac transplantation: the initial mul- Thorac Surg 2011;91:1256–60. J Heart Lung Transplant experience with the MicroMed DeBakey pediatric ventricu- 2011;30:1–8. High level lar and biventricular support with the Thoratec ventricular of cerebral microembolization in patients supported with the assist device as a bridge to cardiac transplantation. Improvement in survival support results in improved outcomes compared with delayed after mechanical circulatory support with pneumatic pulsatile conversion of a left ventricular assist device to a biventricular ventricular assist devices in pediatric patients. Berlin Heart as a Safety of long-term mechanical support with Berlin Heart bridge to recovery for a failing Fontan. Outcomes of ventricular transplant with the Berlin Heart after cavopulmonary shunt. J assist device support in young patients with small body sur- Heart Lung Transplant 2009;28:399–401. Outcomes with diac transplant after Berlin Heart bridge in a single ventri- ventricular assist device versus extracorporeal membrane cle heart: use of aortopulmonary shunt as a supplementary oxygenation as a bridge to pediatric heart transplantation. Use of an tricular assist devices in pediatric patients with univentricular allograft patch in repair of hypoplastic left heart syndrome hearts. The 1-year mortality before transplant was 3% for 24 typical donation after brain death recipients. Clearly, the patients with a score of 0, 8% for a score of 1, and 20% for a interval from withdrawal of life support to death remains a score of 2. While the warm ischemia time ranges from 30 or circulatory support and does not provide an advantage to minutes for lungs to nearly 60 minutes for abdominal organs, those who do not require this degree of support. Status 2 can- age has been noted to be a risk factor for higher recipient didates do not meet the 1A or 1B criteria and are not usually mortality. Patients in status 1A may be assigned organs hearts from older donors due to size considerations and have based on time on wait list instead of risk of death. Wait list mortality for heart trans- to fulfll traditional brain death criteria as herniation does not plant patients can range from 4% to 18% depending on age usually occur due to open fontanelle and skull sutures that group, with young infants having the highest risk, in large are not fused. One study initially listed as status 1B or 2 were likely upgraded to status limitation is that many participating centers did not routinely 1A. As expected, wait list had an equivalent 1-year survival and freedom from rejection mortality for highly sensitized patients was higher. Specifcally, mortality in list time, which means a possibility of increased risk of end- Fontan patients listed less than 6 months after surgery was organ malperfusion and complications from medications increased compared to patients listed more than 6 months used to support cardiac function. The authors noted that though the infant group has the high- Patients who have undergone Fontan completion have a est wait list mortality and highest early post-heart transplant varying degree of preserved ventricular function. This large multicenter study tabulated data from more served ventricular function prior to heart transplant, the other than 700 infants less than 6 months of age. Louis study, preserved function meant no pared with the previous era (71% versus 70%). Data for vascular accidents – particularly for small children – remains 61–63 recipients between 18 and 54 years of age from 1990 to 2008 signifcant. In part this may be due to the less mature 64,65 (era 1: 1990–1998; era 2: 1999–2008) were assessed. Next, the aorta is transected close to the innominate Linda, California; although the child expired 3 weeks after artery, and the cardiectomy is completed by transecting the the procedure, the operative intervention attracted consider- main pulmonary artery just proximal to the takeoff of the able controversy, as well as worldwide attention on both the right pulmonary artery. The heart is carefully moved away need for heart transplants in young children as well as the from the operative feld and onto a sterile back table. Typically, other surgical teams are time from the moment of donor recovery to recipient implan- present on site to participate in the multiorgan recovery pro- tation. It is particularly important for the cardiac donor recov- patients compared to other groups, there is often a clinical ery surgeon to establish a clear line of communication with necessity to expand the donor pool by accepting organs from the lung and liver recovery surgeons with respect to key ele- far away. The primary endpoint was graft loss within 6 mobilized free from surrounding tissue and the azygos vein months, and the secondary endpoint was long-term graft loss is ligated and divided (Fig. Although data were not available in artery to enable future aortic cross-clamp placement. A car- this study to support this conclusion, it is likely that increased dioplegia catheter is placed in the aortic root and connected graft ischemia time leads to graft loss due to a higher likeli- to the tubing lines to allow for the cardioplegia/preservation hood of primary graft failure secondary to myocyte damage solution (our preference is Belzer–University of Wisconsin and endothelial activation after donor brain death. The left heart is vented thorough an incision arteries to the recipient’s native pulmonary vasculature. Topical cooling is performed with the recent report from two experienced centers (Michigan and use of sterile ice–saline slush. There was a downtrend in fstula size over the ostia of the individual pulmonary veins are not compro- time, and no patient required interventions; of note, there was mised for a possible lung recipient surgical procedure (Fig. The lines of division are carefully assessed circumferentially before transection is undertaken. It is preferable to use two monoflament sutures (anterior suture line/posterior suture line) for the pulmonary artery connection to minimize risk of supravalvar pulmonary artery stenosis. Careful attention to assessing the size mismatch between the donor and recipient aorta can help avoid using patch material in most cases to complete the reconstruction. The biatrial technique of implanta- the key elements of the procedure involves ensuring that tion (which requires two atrial anastomosis) was initially 110,111 the four pulmonary veins are kept intact during the donor described in the early 1960s by the Stanford group. This technique, while preserving the physi- nique employed by surgeons worldwide for many years.

Key Issues Raised from the Case Study Although viruses can be spread in numerous ways buy cheapest nizagara erectile dysfunction 30 years old, mosquitoes have been a source of infectious disease since the beginning of time (e order 100 mg nizagara mastercard erectile dysfunction treatment dallas. It is critically important to have an efective plan in place to control the mosquito population and prevent a massive outbreak of infection in densely populated areas. In this case study, health ofcials appeared to be caught of guard on the spread of West Nile virus. Tey were unable to have a plan in place that prevented a high number of infected cases from occurring. Items of Note North America West Nile virus was frst diagnosed in Uganda in 1937 (Lane County of Oregon, 2008). Killer Bee Attacks, United States, 2008 Stage 1 of the Disaster You are the director for a state agency contending with agriculture in the southwest United States. On March 25, you receive a report out of San Antonio that a family was attacked by bees inside their home (Sting Shield Insect Veil, 2008). It was later confrmed that the bees were “killer bees” or Africanized bees that are beginning to migrate through the United States from Mexico (Sting Shield Insect Veil, 2008). In addition, you know that these types of bees can cause damage to your state’s local honeybee population, which is essential for producing commercial honey and pollinating crops. The director should deter- mine the current location of the killer bees and attempt to contain them until a plan is formulated to terminate the bees in the state. A second priority would be to put a plan in place to assist residents who have a killer bees’ nest located on their property and are at risk for a bee attack. What should be your communication plan for government ofcials and residents of your state? The director should keep in contact with county and city of- cials and animal control divisions that could alert the director to the presence of killer bees in their areas. In addition, the director could communicate with the agricultural stations that are run by university and college systems throughout the state to give them an alert of killer bee migration. The director needs to formulate a plan to quarantine the killer bees where they have been sighted and then needs the resources to poison the bees before they can cause harm to humans or agriculture. What other agencies do you need to contact and coordinate with in contending with the killer bees? The federal government can provide resources to combat such an invasive and dangerous insect. The federal government has an inter- est in making sure that the killer bees do not proceed any further into the United States to damage agriculture in other parts of the country. The direc- tor will need to coordinate eforts with county and city governments as well as any organization contending with beekeeping and agriculture. In addition, the agriculture programs in state universities and colleges may be able to assist the director in combating the killer bee threat. Stage 2 of the Disaster On April 20, a second killer bee attack took place in San Antonio against a man who accidently set his house on fre when attempting to drive the bees away (Sting Shield Insect Veil, 2008). The director should make sure to take action on killer bee nests when they are discovered. The direc- tor should send any type of resource to the area that can be used to destroy any killer bee nests that are found. In addition, the director may also want to send research scientists to the area to collect data in an efort to analyze what would be the best approach to eliminate killer bees. The director should make an efort to inform the public on how to act around killer bees and who to notify if killer bee nests are found. By informing the public on what not to do to killer bees, the director could potentially save some lives. Stage 3 of the Disaster The presence of killer bees has been verifed in 151 counties of your state, and they show no sign of containment. The killer bees have now attacked a family Case Studies: Other Natural Disasters ◾ 117 in Abilene and killed their two dogs. On April 29, you received a report that a Corpus Christi retirement home had literally thousands of bees swarming inside it (Sting Shield Insect Veil, 2008). If the director is unable to stop the fow of killer bees throughout the state, then the federal government should be contacted and requested to provide assistance. The director needs to verify that killer bees are indeed at populated areas, and if so, take action on eliminating killer bee nests. The director needs to communicate efec- tively with federal, county, and city ofcials. The population needs to be kept apprised of the situation as well as anyone involved with beekeeping in the agriculture business. Stage 4 of the Disaster It turns out that the attack on the retirement home was caused by ordinary honey- bees. However, on May 26, the killer bees claim a 41-year-old victim in Palestine, Texas, who was attacked by hundreds of bees (Sting Shield Insect Veil, 2008). The director needs to be aggressive about going after killer bee nests to prevent the insects from encroaching on populated areas. The director’s eforts need to be coordinated with county and local ofcials and agencies. In addition, medical supplies to contend with killer bee attacks on people should be kept on hand where killer bees are now known to reside. More research should be done on killer bees to get an understanding of what their weak- nesses may be in an efort to eliminate them from the state without damaging honeybees, which contribute to the agriculture business. The director should make a very large efort to continue any public announcement on the dangers of killer bees and edu- cate the public on how to recognize that particular type of bee. Key Issues Raised from the Case Study Now that the killer bee colony has been seen in the United States, there is no efec- tive choke point to stop the bees from entering in other states, cities, or counties. Terefore, administrators that face the possibility of killer bees being in their area should have a plan on hand to assist individuals that have been attacked by the killer bees and to protect any industry that may be impacted by the killer bees’ presence. The inability to contain the killer bee colony led to people being attacked as well as inficting harm on the honeybee population, which produces honey for the agricultural industry. In counties where killer bee attacks have occurred, numbers of honeybee colonies have been quarantined, impacting the honey industry (Sting Shield Insect Veil, 2008). At 10:15 in the morning on December 6, you receive a dispatch that there has been an explosion at the local mining operation near your city (Boise State University, 2008). The fre chief should alert all frst responders that there has been an accident at the mine and then locate any type of resource that can assist frst responders with digging (e. The fre chief should contact the owners of the mining operation, local government ofcials, and any other entity that may be able to provide resources for search and rescue operations. It turns out that none of your frst responders have the appropri- ate breathing apparatuses to contend with the poisonous gases, and therefore the frst responders must work in shifts. In addition, the main entrances to the tunnels are completely blocked with two strings of iron ore cars, rock, and twisted metal debris caused by the blast that induced the cave-in (Boise State University, 2008). The fre chief needs to acquire appropriate breathing apparatuses from other organiza- tions if they are not available to him currently. The fre chief should also obtain heavy machinery to dig tunnels into the mine to retrieve the miners. Tis activity should be conducted under the supervision of an engineer to ensure the safety of the frst responders and to advise how best to dig the victims out of the rubble.

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In addition purchase nizagara 25 mg line impotence causes and cures, pullback of the use of both two-dimensional imaging and Doppler analysis nizagara 25 mg otc valsartan causes erectile dysfunction. However, today, a pulmonary artery line is reserved for can be used to quantitate the pressure gradient. In the early postoperative period, the right gradient will occur if the Doppler beam cannot be aligned ventricle is likely to be the limiting factor for total cardiac parallel to the area of peak velocity within the outfow tract. There has also been considerable retraction of tion for a return on bypass to extend the outfow patch across the right ventricle during the period of myocardial ischemia, the annulus. A high will be characterized by an elevated left atrial pressure and right atrial pressure, for example, more than 10–12 mm, is systemic hypotension. Normally, right atrial pressure would poorly tolerated by the neonate and young infant and will be expected to be higher than left atrial pressure in the imme- result in a “leaky capillary syndrome. The diagnosis can be confrmed who is adapted to the low oxygen environment of the prena- by demonstrating a marked step up in the oxygen saturation tal circulation. Cardiac output is maintained, urine output is of blood taken from the right atrium (e. In the child between about 4 and were undetected may become detectable once the repair 10 kg, the annular diameter in millimeters needs to be at is completed and right ventricular pressure is subsystemic. The peripheral pulmonary arteries are thin walled and distensible and pulmonary vascular resistance is gener- early mortality ally not elevated. An acute volume load is particularly poorly All patients were less than 90 days of age, with a median tolerated in the setting of diastolic dysfunction. Of the 99 patients, 59 were prostaglandin Restrictive right ventricular diastolic physiology may dependent. Overall 91% of patients were considered symp- occur in older patients as the result of the concentric hyper- tomatic because of cyanosis with or without cyanotic spells. The results from Children’s Hospital Boston are simi- lar to those from several other groups. There were Coronary Obstruction and Rare Coronary Anomalies If two hospital deaths for a hospital mortality of 0. In an the outfow tract patch suture line has passed extremely close important study described by Kirklin et al. The authors concluded malities (hypokinesis, akinesis) will further confrm this sus- that there was a possible disadvantage for the two-stage picion. It may become necessary to return on bypass, take approach employing preliminary shunting and later repair. When this is lonG-term results after early primary repair undertaken, it is useful to use interrupted pledgetted sutures with the pledgets lying on the endocardial surface of the free In 2001, Bacha et al. Retraction of the main pulmonary artery to view the up was obtained for 45 of the 49 long-term survivors. Although there were eight early Retrograde fow in the left main, as well as evidence of papil- deaths in this early timeframe, there was only one late death lary muscle fbrosis, may alert the echocardiographer to the 24 years after the initial repair. There was no infuence of a transannular patch on Transatrial Approach to Repair of late survival (Fig. The majority of reintervention procedures which obstruction is secondary to moderately hypertrophied muscle 10 patients underwent were for recurrent right ventricular out- bundles rather than generalized hypoplasia of the infundibu- fow tract obstruction which was necessary in eight patients. There is a trend toward a higher rate of reintervention for patients who did not have transannular patch. Other reinterventions included one patient who had a homo- graft pulmonary valve replacement 20 years postoperatively primary versus two-staGe repair performed at another institution and one patient who required a defbrillator for inducible ventricular tachycardia. A small number of centers continue to support an approach Long-term follow-up studies from other centers have of initial palliation with a modifed Blalock shunt in the suggested that residual or recurrent right ventricular out- frst 6–12 months of life followed by subsequent repair3,4 fow tract obstruction is a more serious late problem and a and have been able to achieve excellent early results with more common cause of need for reoperation than pulmonary this strategy. Chen and Moller67 followed 144 patients for 10 underwent a right ventricular infundibular sparing strat- years. They found that patients with right ventricular outfow egy between 1995 and 2008 at Texas Children’s Hospital. Postoperative morbidity reviewed 106 patients who underwent repair of tetralogy at included arrhythmias (3% [10]), postoperative bleeding the University of Minnesota between 1954 and 1960. Similar (2% [7]), temporary renal failure (1% [3]), and neurologic to the experience from Boston, the commonest cause for reop- injury (<1% [2]). Overall eration was recurrent right ventricular outfow tract obstruc- 1- and 7-year Kaplan–Meier survivals were 97 and 96%. For with the mandatory reintervention required with a two- example, this was true in the two institutional study reported stage approach. For exam- of Alabama with an approach of early primary repair as prac- ple, in a 1997 report by Gladman et al. In fact, a high postrepair patients who had a two-stage approach at the Hospital for Tetralogy of Fallot with Pulmonary Stenosis 363 Sick Children in Toronto was 90%, while in patients who had replacement. The latter when indexed to body surface area is currently con- sidered the best measure for proceeding with valve replace- transatrial versus transventricular repair 2 ment. Currently, an indexed volume of 150–165 mL/m or A number of studies have presented excellent results using a greater is considered an indication to operate. Reoperation for right ventricular outfow obstruction was more common in the latter phase of the study, presumably Although some centers have expressed enthusiasm for place- as the strategy of infundibular sparing was more aggres- ment of a monocusp valve at the time of initial tetralogy sively applied. These authors have focused attention on the postoperative echocardiographic fndings of 24 patients who length of the ventriculotomy as an important determinant of had undergone transannular patch repair with a monocusp late right ventricular function. However, it is important to valve with 17 patients who had undergone patch repair with- remember that centers which mainly use a ventriculotomy out a monocusp valve and 20 patients who had undergone approach to the repair of tetralogy focus attention on a num- repair without a transannular patch. The authors found no ber of other factors which determine late right ventricular signifcant differences in the degree of early postoperative function. These factors include: pulmonary regurgitation or in clinical outcomes, such as mortality, number of reoperations, or hospital stay. Although • avoidance of excessive division of right ventricu- 16 of 19 patients had competent monocusp valves immedi- lar muscle ately postoperatively by 24 months only one of seven patients • careful preservation of tricuspid valve function by had a competent valve. No patient had monocusp stenosis, avoidance of sutures snaring tricuspid chords or although this is likely to be an important late consequence of leafet tissue monocusp valve insertion. This can result in severe compres- fracture and the huge variability of the shape and size of the sion of the trachea, mainstem, and peripheral bronchi. In postoperative infundibulum following placement of a trans- addition, the small pulmonary arteries can be affected. We have also seen late left main coronary normal pattern of single segmental arteries is replaced by a obstruction and development of an aorto-pulmonary fstula network of intertwining arteries which compress intrapul- in the setting of a previous Ross-Konno procedure. At the severe end of the for determining optimal timing for late pulmonary valve spectrum, the outlook is dismal. The pulmonary arteries are 364 Comprehensive Surgical Management of Congenital Heart Disease, Second Edition dilated along their entire length to the periphery of the lung, pathophysioloGy anD clinical presentation and airway compression is severe occurring along the length Hypoxemia in this syndrome is the result of both intrapul- of the bronchial tree and into the small airways. Pulmonary venous hand, at the mild end of the spectrum, there is minimal or desaturation can result from ventilation-perfusion mismatch no tracheal bronchial involvement, the small airways are not and intrapulmonary shunting. Air-trapping is encountered leading right ventricular outfow tract obstruction, including pulmo- to overinfated lungs. Hypercarbia commonly ensues with a about the absent pulmonary valve syndrome is that the ductus resultant respiratory acidosis which can be severe enough to arteriosus is never present when the pulmonary arteries are in reduce pH below 7. It is felt that in the presence of a ductus arteriosus, 79 sion is less severe, the child may present later in infancy with this lesion is incompatible with fetal survival to birth.

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This distance can be quantitated by measuring the linear distance between the two septal hinges in the four-chamber view order nizagara toronto erectile dysfunction causes natural treatment. A: Modest displacement of the tricuspid valve apparatus and excellent mobility of the valve leaflets buy 50 mg nizagara visa erectile dysfunction drugs associated with increased melanoma risk. Both the anterior and septal leaflets remain visible in this plane because the degree of anterior rotation in this heart was minimal. The small white arrows highlight the separation between the septal insertions of the anterior mitral and septal tricuspid leaflets. The remnant of the septal leaflet (and its hinge point) was found near the right ventricular outflow tract, far anterior to the plane demonstrated in this image. The anterior leaflet is significantly tethered and was immobile in this plane; it remains parallel to the right ventricular free wall, even though this frame was taken at peak systole. In this situation, the apical displacement index is clearly large but cannot be accurately measured because no septal leaflet tissue is visualized in this plane. Nonetheless, the exaggerated separation between the septal insertion of the anterior mitral leaflet and the displaced tricuspid septal remnant clearly identifies this is a case of Ebstein malformation. Case-control studies suggest genetic, reproductive, and environmental risk factors (e. In utero cases of severe Ebstein anomaly may demonstrate increased heart size, fetal hydrops, or pulmonary parenchymal hypoplasia secondary to the marked cardiac enlargement. Postnatal arrhythmias are usually supraventricular and 10% to 30% of patients with Ebstein anomaly have pre-excitation. Beyond infancy, patients with Ebstein anomaly display dyspnea, fatigue, palpitations, exercise intolerance, or cyanosis with exercise. Echocardiography has dramatically influenced when patients with Ebstein anomaly have initial diagnosis. However, despite the increased availability of echocardiography in the modern era, 10% of patients with Ebstein anomaly remain undiagnosed into adulthood. In contrast, patients with more severe Ebstein anomaly present at an earlier age (75,76,77). Neonatal cyanosis typically is the first sign due to shunting at atrial level (74). In the most severe forms of Ebstein anomaly, symptoms of congestive heart failure (dyspnea, poor feeding, and poor weight gain) will be evident. Cardiac examination usually reveals no lifts or thrills and normal first and second heart sounds. Rarely, in patients with a large, hypermobile anterior leaflet multiple sounds may occur. This “multiplicity of sounds” is unusual (despite previous descriptions that this is a “classic” finding in patients with Ebstein anomaly). Severe tricuspid regurgitation causes a holosystolic murmur along the left lower sternal border. In patients with severe cyanosis, the pulmonic component may not be detected due to decreased outflow. The spectrum of physical examination findings observed in Ebstein anomaly is summarized in Table 38. Because the massively enlarged heart occupies much of the chest, variable degrees of lung hypoplasia may be present (59,65,73). Signs and symptoms of Ebstein anomaly later in life are usually threefold: (1) murmur, (2) exertional dyspnea or cyanosis, (3) palpitations from new-onset arrhythmia (74). The V wave can be seen in patients with severe tricuspid regurgitation without an interatrial shunt. Approximately 10% of patients with Ebstein anomaly are diagnosed in adolescence or adulthood (74). Adult patients may have been misdiagnosed, without imaging, as having “mitral valve prolapse” because a click was detected during auscultation. The severity of the right bundle branch disease is directly related to the abnormal formation of the septal P. Accessory pathways are present on the tricuspid valve annulus and may conduct in antegrade and/or retrograde directions. Ventricular pre-excitation is present in 10% to 30% of patients with Ebstein anomaly (81,82) (Fig. More than one accessory pathway can be present in 20% of those with pre-excitation (83). Often, slow conduction through the accessory pathway results in a minimal degree of pre-excitation (84) (Fig. These patients are especially prone to cavotricuspid isthmus flutter and intra-atrial reentrant tachycardia (84). B: Echocardiograph from the same patient, in apical four-chamber projection, demonstrating severe right ventricular dilation and dysfunction. Despite the reassuring appearance of the cardiac silhouette on the radiograph this child had severe tricuspid regurgitation and did well after cone reconstruction. C: The lateral chest radiograph demonstrates obliteration of the retrosternal airspace consistent with right ventricular dilation. Chest Radiography Due to the variability of Ebstein anomaly, the chest radiograph can be normal or demonstrate severe cardiomegaly and diminished vascular markings (65,85). The newborn with severe Ebstein anomaly classically has massive cardiomegaly (Fig. Echocardiography The gold standard for the diagnosis of Ebstein anomaly is echocardiography. One of the most sensitive, but not solely, diagnostic features of Ebstein anomaly is the displacement of the septal leaflet hinge point. In a normal heart the tricuspid septal leaflet hinge point is slightly apical to the anterior mitral valve leaflet hinge point (see Fig. The “Displacement Index” is measured in systole or diastole from the insertion point of the anterior mitral leaflet to the hinge point of the tricuspid septal leaflet (where it begins to delaminate). A 2 displacement index >8 mm/m , is one feature that supports a diagnosis of Ebstein anomaly (see Fig. This is difficult in the neonatal setting with elevated pulmonary vascular resistance (88,89). Approximately 10% of patients with Ebstein anomaly are diagnosed utilizing fetal echocardiography (75,76,77). Fetal tachyarrhythmias occur in Ebstein anomaly and can contribute to development of hydrops. This is likely secondary to the combination of the severity of the malformation and the attendant lung hypoplasia. Three-dimensional echocardiography may add incremental information in select patients with Ebstein anomaly. Nuances of Assessment of Tricuspid Valve Regurgitation in Ebstein Anomaly Tricuspid regurgitation may be difficult to accurately quantitate in patients with Ebstein anomaly (93,94).