Paradoxical embolism associated with ebsteins anomaly in an adult. Tricuspid valve replacement and various types of plastic reconstruction are the two methods of treating ebsteins anomaly. Pdf anesthetic management in a patient with ebstein anomaly. In addition to the hemodynamic burden of the actual valve defect, ebsteins patients must also contend with an extraordinarily high incidence of tachyarrhythmias, most of which can be attributed to accessory. Ebsteins anomaly is characterized by a downward displacement of the septal and posterior leaflet attachments at the junction of the inlet and trabecular portions of the right ventricle. Echocardiography in the diagnosis of ebsteins anomaly. The aim of this investigation is to demonstrate that in ebsteins anomaly ea the right ventricle rv is affected in its three portions and to establish an anatomoechocardiographic correlation between the anatomic features and the equivalent echocardiographic images. Sep 11, 2009 ebstein s anomaly of the tricuspid valve is a congenital malformation with apical displacement and tethering of the valve resulting in atrialization of a portion of the ventricle. The goals of surgery are to treat the valvular disease and, if necessary, the dysfunction of the right ventricle. Ebsteins anomaly is a complex malformation that has been treated by various surgical techniques, with variable results, since 1958. Replacement of the tricuspid valve has been more successful in older children and adults than in infants, but overall, less successful than. Someone with ebsteins anomaly has a malformed not formed correctly and improperly positioned tricuspid valve. The tricuspid valve separates the right lower heart chamber right ventricle from the right upper heart chamber right atrium.
The tricuspid valve separates the right atrium the chamber that receives blood from the body from the right ventricle the chamber that pumps blood to the lungs. Ebstein s anomaly is a malformation of the tricuspid valve. The most common involves a repair of the tricuspid valve. In ebstein anomaly, the something different is the position of the. The aim of the present study was to define the ecg characteristics before and after ablation of an accessory a. Ebsteins anomaly johns hopkins textbook of cardiothoracic.
Ebsteins anomaly can result in severe tricuspid valve regurgitation reverse blood flow, heart rhythm disorders and associated atrial septal defects. In ebstein s anomaly, one or two of the three leaflets are stuck to the wall of the heart and dont move normally. The abnormality causes the tricuspid valve to leak blood backwards into the right atrium. The first case was that of an 18yearold girl with minimal cyanosis and minimal exercise intolerance. Ebsteins anomaly is the association of tricuspid valve insufficiency with a right ventricle anomaly. Cases with severe deformity can lead to congestive heart failure during the neonatal period or even intrauterine death. Management and longterm outcome of neonatal ebstein anomaly. To our knowledge, this observation has not been described previously. The severity of the morphology of the lesion and ventricular function determine the spectrum of surgical techniques that.
Ebstein anomaly is a rare heart defect in which the tricuspid valve the valve between the upper right chamber right atrium and the lower right chamber right ventricle of the heart isnt formed properly. We present the case of an infant with prenatal diagnosis, at 32 weeks gestation, of ebsteins anomaly without anterograde flow from right ventricular to pulmonary atresia pafunctional pa with flow reversal in the ductus arteriosus. Ebstein anomaly is a congenital malformation that is characterized primarily by abnormalities of the tricuspid valve and right ventricle. As a result, blood leaks back through the valve and into the right atrium. Tricuspid valve replacement and various types of plastic reconstruction are the two methods of treating ebstein s anomaly. Ebsteins anomaly is the most common congenital malformation of the tricuspid valve. Ebstein s anomaly is a congenital malformation of the right ventricle and tricuspid valve that is characterized by multiple features that can exhibit an infinite spectrum of malformation. The purpose of this study was to examine our experience with cone repair. Some people with ebsteins anomaly require heart valve replacement, while others may only develop a.
It is our purpose to add one more diagnostic feature to the present physiologic findings characterizing ebsteins malformation, an abnormality which has been well documented in the last few years. A 26yearold mixedrace pregnant woman with a history of apparent health evaluated in a community genetic counseling clinic with increased risk due to exposure to teratogens and mutagens was presented. Ebsteins anomaly is a rare type of congenital heart defect characterized by a malformation of the tricuspid valve and the right side of the heart. Some people with ebsteins anomaly require heart valve replacement, while others may only develop a mild heart rhythm disorder. Ebsteins anomaly may present at any age and has a highly variable clinical. In patients with ebstein s anomaly, the valve between the chambers on the right side of the heart the tricuspid valve does not close correctly.
Ebstein s anomaly, also called ebstein s malformation, is a rare, congenital present at birth heart defect. It happens because of a problem with how the valve forms before a baby s birth. Anatomical description and types there is significant variation in the anatomy of the valve but ebstein s anomaly always involves, at least in part, the septal and posterior leaflets which are deformed and displaced toward the. The backup of blood flow can lead to heart swelling and fluid buildup in the lungs or liver. The defect usually causes the valve to work poorly, often resulting in blood. In ebstein anomaly, two leaflets of the tricuspid valve are displaced downward into the pumping chamber. The medical records of 40 neonates with a diagnosis of ebstein anomaly who were admitted to our.
Ebsteins anomaly in xxy klinefelters syndrome jama. Its classified as a critical congenital heart defect 1 accounting for ebsteins anomaly can result in severe tricuspid valve regurgitation reverse blood flow, heart rhythm disorders and associated atrial septal defects. Environmental factors and drug use can also influence the development of the defect. Ebsteins anomalyhow to correct severe anatomical forms. Perioperative and anesthetic considerations in ebsteins. Ebsteins anomaly is a congenital malformation of the right ventricle and tricuspid valve that is characterized by multiple features that can exhibit an infinite spectrum of malformation. Chest xray showed severe cardiomegaly and echocardiogram confirmed ebsteins anomaly with a thickened non. Renewed interest in the natural history of ebstein s anomaly has been created by recent surgical innovations in its management. Ebstein anomaly is a congenital malformation involving the tricuspid valve and right ventricle rv.
In ebsteins anomaly, one or two of the three leaflets are stuck to the wall of the heart and dont move normally. Pdf ebsteins anomaly in prenatal diagnosis revista. Severe ebstein s anomaly may cause symptoms in newborns including cyanosis and heart failure. Additionally the presented method of ebstein s anomaly repair has its technical limitation in the presence of a small atl and ptl or in carpentier type d disease that prevents the creation of a monocuspid valve, necessitating prosthetic tv replacement in 5 out of 29 patients presenting with ebstein s anomaly at our institution. Apr 30, 2014 ebstein s anomaly is a rare heart defect in which parts of the tricuspid valve which separates the right ventricle from the right atrium are abnormal. Ebsteins anomaly is a malformation of the tricuspid valve and right ventricle. Ebsteins anomaly of the heart occurring in a boy with xxy klinefelters syndrome is a combination which has not been previously reported.
Preface ebsteins anomaly is a rare congenital heart disorder occurring in. Ebsteins anomaly of the tricuspid valve can also be associated with problems in the electrical system that controls the hearts pumping. Severe ebsteins anomaly may cause symptoms in newborns including cyanosis and heart failure. It should be noted that the valvar leaflets are also abnormal in ebsteins malformation.
Full text get a printable copy pdf file of the complete article 1. Ebsteins anomaly and extracardiac defects jama pediatrics. Renewed interest in the natural history of ebsteins anomaly has been created by recent surgical innovations in its management. Ebsteins anomaly of the tricuspid valve is a congenital malformation with apical displacement and tethering of the valve resulting in atrialization of a portion of the ventricle. Ebsteins anomaly with reversible functional pulmonary. Cone repair provides a near anatomic tricuspid valve repair. Ebsteins anomaly ea is a rare congenital malformation of the tricuspid valve, often associated with other cardiac malformations, especially atrial septal defect. In patients with ebsteins anomaly, the valve between the chambers on the right side of the heart the tricuspid valve does not close correctly. The alterations of each portions of the rv were described. Ebstein s anomaly is a complex malformation that has been treated by various surgical techniques, with variable results, since 1958. Electrocardiographic characteristics of patients with.
In 1972, the authors developed a repair that consisted of plication of the free wall of the atrialized portion of the right ventricle, posterior tricuspid anuloplasty, and right atrial reduction. Ebsteins a nomaly is a rare heart defect in which parts of the tricuspid valve which separates the right ventricle from the right atrium are abnormal. Ebsteins anomaly is a rare congenital meaning it is present at birth heart defect in which the hearts tricuspid valve is abnormal. Ebsteins anomaly of the tricuspid valve is a complex malformation. Ebsteins anomaly of the tricuspid valve is a relatively rare form of congenital heart disease that has long been a challenge to electrophysiologists and cardiac surgeons. Since many severe congenital heart diseases do not permit survival into puberty, it is possible that the association of klinefelters syndrome with congenital heart disease is being missed. Ebstein s anomaly has many different presentations depending upon the severity of the lesion. Ebsteins anomaly is the most common congenital heart disease affecting the tricuspid valve. Ebsteins anomaly embryology anatomy physiology natural history clinical findings laboratory findings management 3.
Because the tricuspid valve is malformed in ebsteins anomaly, it often doesnt. Ebsteins anomaly ea, a rare congenital heart disease, results from the failure of delami. Ebsteins anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle of the heart. Walsh, md abstract ebsteins anomaly of the tricuspid valve is a relatively rare form of congenital heart disease that has long been a challenge to electrophysiologists and cardiac surgeons. Mild ebstein s anomaly may have no symptoms during childhood and present later in life with shortness of breath, fatigue and decreasing exercise tolerance. Ebsteins anomaly is a rare congenital heart disorder occurring in. Ebsteins anomaly of the tricuspid valve a natural laboratory for reentrant tachycardias edward p. Ebsteins anomaly is a malformation of the tricuspid valve. Pdf on oct 3, 2018, sinem altunyuva usta and others published ebsteins anomaly find, read and cite all the research. Nov 23, 2007 ebstein s anomaly has a highly variable natural history that depends on the degree of abnormality of the tricuspid valve, with a spectrum from mild to severe forms 1, 4, 5, 18. But sometimes the tricuspid valve leaks severely enough to result in heart failure or cyanosis. Because so little is known about this latter group, we. Ebsteins anomaly is a complex and heterogeneous form of congenital heart disease characterized by malformation and apical displacement of the tricuspid valve leaflets. The cause of ebstein anomaly is heterogeneous with most cases thought to be a multifactorial trait.
Ebsteins anomaly genetic and rare diseases information. Although the average survival with ebsteins anomaly, when isolated, except for a defect in the atrial septum is about 25 years, at least seven patients surviving for 70 years or longer have been reported. Echocardiography is the procedure of choice for diagnosis of ebsteins anomaly. Ebstein s anomaly is a rare defect that involves the tricuspid valve and right ventricle with an unknown embryology. It happens because of a problem with how the valve forms before a babys birth. Because the tricuspid valve is malformed in ebstein s anomaly, it often doesnt. The right side of the heart is where blood returns from the rest of the body and gets. The cone reconstruction of the tricuspid valve in ebstein. This hole is called an atrial septal defect or asd. Often there s also a hole in the wall between the atria, the heart s two upper chambers.
Cone reconstruction of the tricuspid valve for ebsteins. Ebstein s anomaly is mild in most adults who have it, so they dont need surgery. Its classified as a critical congenital heart defect accounting for pdf the surgical management of ebstein anomaly. Ebstein anomaly is a rare heart defect that affects the tricuspid valve one of the heart s four valves. There have been cases, though, where the condition develops by chance and with no apparent underlying cause according to an article by stanford health care. Ebstein anomaly is a rare heart defect that affects the tricuspid valve one of the hearts four valves. Ebsteins anomaly is a special form of tricuspid valve dysplasia, characterized by the.
Ebsteins anomaly is a complex, congenital heart defect characterized by a malformation of the tricuspid valve and right side of the heart. A series of 226 consecutive patients with ebsteins anomaly was studied. Prenatal sonographic detection of ebsteins anomaly mariah c. Ebsteins anomaly occurs in approximately 1 in 20,000 live births.
Ebsteins anomaly has many different presentations depending upon the severity of the lesion. Mild ebsteins anomaly may have no symptoms during childhood and present later in life with shortness of breath, fatigue and decreasing exercise tolerance. In this defect, there is a downward displacement of the tricuspid valve located between the upper and lower chambers on the right side of the heart into the right bottom chamber of the heart or right ventricle. Ebsteins anomaly of the tricuspid valve occurs as an isolated defect with other forms of congenital heart disease such as transposition of the great arteries or tetralogy of fallot or, rarely, in association with extracardiac malformations. Ebsteins anomaly occurs in 1 percent of all congenital heart defects and represents 0. Often theres also a hole in the wall between the atria, the hearts two upper chambers. The mean age of death is approximately 20 years, with 15% alive at 60 years. Ebsteins anomaly is a rare congenital present at birth defect in the right side of the heart. Ebsteins anomaly, also called ebsteins malformation, is a rare, congenital present at birth heart defect.
A variety of cardiac abnormalities are associated with ebsteins anomaly, including atrial septal defect, conduction system abnormalities, patent foramen ovale, pulmonary stenosis or atresia, and ventricular septal defect. The patient is monitored with a radial artery catheter, a. The clinical presentation of ebstein anomaly varies widely, ranging from the critically ill neonate to the asymptomatic adult, depending upon the degree of anatomic abnormality. Ebsteins anomaly is a rare heart defect in which parts of the tricuspid valve which separates the right ventricle from the right atrium are abnormal. Listen to the audio pronunciation in the cambridge english dictionary. Ebstein anomaly has heterogeneous anatomy and numerous operative techniques are described. This classification is particularly helpful withneonatal ebsteins anomaly. Ebstein anomaly ea is a rare congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets. It is classified as a critical congenital heart defect accounting for ebsteins anomaly of the leftsided tricuspid valve.
Perioperative and anesthetic considerations in ebsteins anomaly. A value of greater than 8 mmm 2 is diagnostic of ebsteins malformation. The indications for surgical intervention in patients with ebsteins anomaly remain controversial in asymptomatic patients, although the natural history of the disease is a relentless progression to congestive heart failure, arrhythmias, or both 16 x 16 giuliani, e. Ebstein s anomaly ea is a rare congenital malformation of the tricuspid valve and right ventricle characterized by fig 1. Several different operations have been used in patients with ebstein s anomaly. Some children with ebsteins anomaly have wolffparkinsonwhite syndrome, which means there is an extra electrical connection between the upper and the lower chambers of the heart.
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