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Ann Thorac Surg 2000;69:S106-S117
© 2000 The Society of Thoracic Surgeons
a Division of Cardiovascular and Thoracic Surgery, Mayo Clinic, Rochester, Minnesota, USA
Address reprint requests to Dr Dearani, Division of Cardiovascular Surgery, Mayo Clinic, 200 First St, SW, Rochester, MN 55902
e-mail: jdearani{at}mayo.edu
Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.
Abstract
Ebstein’s anomaly is a rare congenital heart defect that is characterized by a spectrum of anatomical abnormalities of the tricuspid valve that also involve the right atrium and right ventricle. The extant nomenclature for Ebstein’s anomaly and our approach to the description of the severity of Ebstein’s anomaly are reviewed with the objective of establishing a unified reporting system. Although there are common features in Ebstein’s anomaly, there is a wide spectrum of pathology with an infinite variety of combinations of severity of the involved structures. An effort was made to develop a classification system that would take into consideration the anatomic abnormalities that help direct the surgical management, particularly in regard to tricuspid valve repair or valve replacement. Isolated congenital tricuspid stenosis and regurgitation are also rare, and a simple classification system is presented. Acquired causes of tricuspid regurgitation and stenosis are more common and are included in the classification system. A comprehensive database set for these malformations is presented so that a comprehensive risk stratification analysis can be performed. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and risk factors are presented.
I. Background
Ebstein’s anomaly [1] is a rare cardiac anomaly that occurs in 1 per 210,000 live births and accounts for less than 1% of all congenital heart disease [2]. It is a malformation of the tricuspid valve and right ventricle that is characterized by a spectrum of several features: 1) adherence of the tricuspid leaflets to the underlying myocardium (failure of delamination); 2) downward (apical) displacement of the functional annulus (septal > posterior > anterior leaflet); 3) dilation of the "atrialized" portion of the right ventricle with variable degrees of hypertrophy and thinning of the wall; 4) redundancy, fenestrations, and tethering of the anterior leaflet; and 5) dilation of the right atrioventricular junction (the true tricuspid annulus). These anatomical and functional abnormalities cause important tricuspid regurgitation that results in right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. In addition, approximately 14% of patients will have one or more accessory conduction pathways with Wolff-Parkinson-White syndrome [3, 4].
Isolated congenital tricuspid stenosis or regurgitation occurs even more rarely than Ebstein’s anomaly. Acquired tricuspid stenosis or regurgitation includes multiple causes ranging from rheumatic valvular disease, to pacemaker lead entanglement, to late sequelae after other cardiac surgical procedures.
Review of extant nomenclature
The morphologic spectrum of the anomaly [5–8] as well as the frequently associated anomalies [4] have been described extensively. Perhaps because Ebstein’s anomaly is such a rare form of congenital heart disease and there is such a wide spectrum of abnormalities, there has been, to our knowledge, only one attempt to classify the anomaly, that of Alain Carpentier and associates in 1988 [9]. The authors proposed four grades of Ebstein’s anomaly: type A, the volume of the true right ventricle is adequate; type B, there is a large atrialized component of the right ventricle but the anterior leaflet moves freely; type C, the anterior leaflet is severely restricted in its movement and may cause significant obstruction of the right ventricular outflow tract; and type D, almost complete atrialization of the ventricle with the exception of a small infundibular component.
This classification has the advantage of simplicity in which all degrees and combinations of anatomical severity are divided into only four categories. In actuality, however, there may be little correlation between the degrees of severity of the various components of the anomaly as listed above (degree of leaflet adherence, degree of apical displacement, degree of dilation of the atrialized right ventricle, etc). For example, one heart may have little apical displacement of the valve yet severe dilation of the atrioventricular junction and severe tricuspid regurgitation, whereas another may have the reverse (significant apical displacement of the valve but well-formed leaflets and only mild tricuspid regurgitation and mild dilation of the atrioventricular junction). Similarly, the presence of any tricuspid stenosis in our experience is variable and may actually be more common in Carpentier type D than in type C.
There has been no formal classification system proposed for isolated congenital tricuspid valve stenosis or regurgitation. Acquired causes of tricuspid valve stenosis and regurgitation include many different etiologies. These are reviewed below and can be incorporated into a nomenclature system without difficulty.
Recommendations for nomenclature
Ebstein’s anomaly
Our experience with Ebstein’s anomaly, which includes more than 400 surgical patients and others who are being managed conservatively without operation, forms the basis of these recommendations. We have employed two approaches in describing the severity of the anatomical deformity of hearts with Ebstein’s anomaly. The first is based on the echocardiographic appearance in which the pathology is described as anatomically mild, moderate, or severe. This is derived primarily from an impression formed by summation of the amount of displacement of the leaflets, the degree of tethering of the anterior leaflet, and the degree of right ventricular dilation. The three categories of mild, moderate, or severe are similar to that used for echocardiographic quantitation of mitral regurgitation. This classification is imprecise and subjective, and in essence resembles that of Carpentier and associates [9]; it also has the same limitations.
Our second approach is to describe the exact anatomy of each of the involved structures, ie, all three leaflets of the tricuspid valve, the right ventricle, and the right atrium. Additional associated anomalies are noted together with any left-sided cardiac abnormalities that are present. These descriptions are made at the time of operation and are detailed in the operative note. Although there are common features in Ebstein’s anomaly, no two hearts have exactly the same deformities. Because of the wide spectrum of pathology and the nearly infinite variety of combinations of severity of the involved structures, a comprehensive classification or grading system would be necessarily complex and, although detailed and accurate, probably not clinically practical.
The challenge is to find a middle course of classification in which the anatomy is described in a practical way without being too complex or too generalized to be useful. Additionally, a nomenclature system should emphasize those characteristics that surgeons have found to be important when considering valve repair vis-a-vis valve replacement.
The diagnosis of nearly all cases of Ebstein’s anomaly can be made with confidence from the two-dimensional transthoracic echocardiogram. The principle echocardiographic characteristic that differentiates Ebstein’s anomaly from other forms of congenital tricuspid regurgitation is the degree of apical displacement of the septal leaflet at the crux of the heart (
0.8 cm/m2) [10]. Angiography is generally reserved for evaluation of associated anomalies not well displayed by echocardiography, eg, evaluation of the pulmonary arteries in a patient who has had a previous shunt procedure performed.
In the surgical literature, the terminology for the three tricuspid valve leaflets has generally been "anterior, posterior, and septal." Other terms have been suggested in the past and more recently, such as the terms "anterosuperior, inferior, and medial (septal)" by Lamers and associates [11].
With increasing degrees of anatomic severity of malformation, the fibrous transformation of leaflets from their muscular precursors remains incomplete, with the septal leaflet being most severely involved, the posterior leaflet less severely involved, and the anterior leaflet least severely involved (Fig 1). These changes result in a downward displacement of the hinge point of the posterior and septal leaflets in a spiral fashion below the true annulus. In some patients, the right side of the anterior leaflet can also show failure of delamination and apical displacement. The tricuspid leaflets are typically bizarre and dysplastic, and are tethered by short chordae and papillary muscles or attached to the underlying myocardium directly or by muscular bands. Chordae may be few in number or absent. Fenestrations of the leaflets are common; these characteristically are comprised of an opening in the leaflet guarded by a single papillary muscle giving origin to chordae that attach around the periphery of the opening.
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In less severe cases, the anterior leaflet may form a large sail-like intracavitary curtain; this structure forms the basis of tricuspid valve repairs. The anterior leaflet forms most of the true orifice that directs blood to the pulmonary valve, and it is likely to contain accessory fenestrations. In contrast, if valvular tissue obstructs the flow pathway from the atrialized right ventricle to the pulmonary valve, functional tricuspid stenosis is created. This is most likely to occur when the displaced anterior leaflet is severely tethered or linearly attached to the right ventricular endocardium with or without additional fibrous attachments to the septal leaflet so that the only exit from the right ventricle is through the commissure between the anterior and septal leaflets [6].
A little-mentioned characteristic of the anterior leaflet that is critical to most tricuspid valve repairs is the presence of a free leading edge. The leading edge of the anterior leaflet can be free and mobile, have hyphenated attachments (focal, segmental direct attachments to the underlying endocardium), or linear direct attachment (entire leading edge is attached to the endocardium) [12]. In each case, there can be partial or complete delamination of the remaining portion of the leaflet. In the absence of a free leading edge, suboptimal repairs have been achieved. Perhaps the experience gained with the use of artificial chordae in mitral and tricuspid valve repairs will permit an occasional successful repair in some borderline cases of Ebstein’s anomaly in which the leading edge of the valve has direct attachments to the right ventricular endocardium. We believe that any useful classification of Ebstein’s anomaly should include the status of the leading edge of the anterior leaflet.
There are varying degrees of downward displacement of the functional annulus from the true annulus. The true annulus occupies its normal right atrioventricular junction position; it may be poorly defined, especially posterolaterally, and it is usually dilated. The tissue separating the true from the functional annulus is referred to as the "atrialized" right ventricle. The free wall portion of the atrialized right ventricle is dilated and often thinner than normal; the most extreme thinning occurs posterolaterally. Dilation of the right ventricle may be massive. It involves not only the right ventricular wall proximal to the tricuspid valve but also the right ventricle distal to the valve (functional right ventricle), including the right ventricular infundibulum. These findings suggest that cardiac dilation in Ebstein’s anomaly is due not only to the hemodynamic abnormalities of the anomaly but also to a generalized right ventricular myocardial dysfunction. Morphometric histopathologic studies have demonstrated that right ventricular dilation is associated not only with thinning of the wall but also with an absolute decrease in the number of myocardial fibers counted through the thickness of the wall from endocardium to epicardium [13]. Right ventricular mural thrombus is usually not a finding even in the most severe cases.
Variable degrees of right atrial dilation are commonly seen in Ebstein’s anomaly. In infancy, the atrial wall may be very thin and massively dilated. Marked hypertrophy of the atrial wall may be associated with atrial dilation in older patients. Right atrial mural thrombus is rarely present. There is usually an interatrial communication that may be either a secundum atrial septal defect or a stretched patent foramen ovale.
Marked dilation of the right ventricle significantly affects the structure and function of the ventricular septum and left ventricle. The left ventricle is compressed, displaced posteriorly, and rotated toward the spine. In the more severe cases, the septum is flattened (D shaped) or has leftward bowing associated with paradoxical septal motion. Although measurements of fractional shortening and ejection fraction by echocardiography are less reliable in the presence of paradoxical motion of the septum, many such patients clearly have depressed left ventricular systolic function and a reduced ejection fraction. Interstitial fibrosis may develop in chronic cases.
Mitral valve prolapse is commonly seen, usually because of compression and flattening of the left ventricle. With satisfactory repair of Ebstein’s anomaly in which the right atrium and right ventricle are reduced in size, the left ventricle returns to a more normal configuration and the mitral valve prolapse may disappear. Congenital abnormalities of the mitral valve may also be associated with Ebstein’s anomaly; these include cleft anterior leaflet and parachute mitral valve.
So-called "left-sided" Ebstein’s anomaly is a common finding in patients with atrioventricular discordance and ventriculoarterial discordance (corrected transposition) [14, 29]. In this condition, the systemic (morphologically tricuspid) atrioventricular valve is located on the left side in patients with situs solitus. The nature of the displacement of the septal and posterior leaflets is similar to that in right-sided Ebstein’s anomaly in patients with situs solitus. However, the anterior leaflet is smaller and anatomically quite different. There is less thinning of the wall of the atrialized ventricle, and rarely is the functional portion of the morphologically right ventricle dilated. Moderate to severe dilation of the annulus is rarely seen. The atrioventricular conduction tissue in corrected transposition is right sided and anterior, well removed from the left-sided tricuspid valve.
Associated cardiac anomalies include an interatrial communication, which is present in 94% of patients who come to operation [4]. This communication is usually associated with a right-to-left shunt, although a left-to-right shunt may be present in some young patients with mild forms of Ebstein’s anomaly. The persistent right-to-left shunt found in most patients with Ebstein’s anomaly carries the risk of paradoxical embolism and stroke, a complication that is more likely to occur in older patients or during cardiac catheterization or electrophysiologic procedures. More uncommon cardiac lesions include pulmonary atresia or stenosis, ventricular septal defect, and partial atrioventricular canal. The combination of Ebstein’s anomaly with hypoplastic pulmonary arteries is associated with significant early mortality and poses difficult therapeutic options. A patent ductus arteriosus may be present in the severe forms of neonatal Ebstein’s anomaly. The presence of one or more accessory conduction pathways, often associated with Wolff-Parkinson-White syndrome, is found in approximately 14% of patients [3, 4], and atrioventricular nodal reentry tachycardia is found in 1% to 2% of patients [15]. In addition to these arrhythmias, other serious atrial and ventricular arrhythmias resulting from anatomical changes of right atrial and right ventricular dilation and from right ventricular ischemia are important causes of morbidity and mortality.
Extracardiac structures can also be involved. Extreme dilation of the right atrium and right ventricle in patients with severe Ebstein’s anomaly may compress the lungs bilaterally, especially in infants. Reduction of the size of the right atrium and right ventricle at the time of repair is especially beneficial for infants who are ventilator dependent, as it then allows successful weaning from the ventilator. Congestive hepatosplenomegaly is commonly observed, and microscopic hepatic fibrosis may eventually occur in chronic cases.
An Ebstein’s-like displacement of one or more tricuspid leaflets may be seen in pulmonary atresia with intact ventricular septum or other forms of hypoplastic right heart syndrome. The tricuspid valve in these malformations is hypoplastic and bears little resemblance to the classical form of Ebstein’s anomaly [16, 17].
Congenital tricuspid valve malformations
Isolated congenital tricuspid valve stenosis is very rare. This may result from valvar hypoplasia or shortening, thickening, or fusion of the subvalvar apparatus. In addition, the stenosis may result from a double-orifice tricuspid valve or a parachute deformity with all chordae arising from a single papillary muscle. This malformation is almost always accompanied by a patent foramen ovale or atrial septal defect. In addition, there may be concomitant right ventricular hypoplasia and pulmonary valve stenosis or atresia.
Isolated tricuspid regurgitation is similarly uncommon. Organic causes from anatomical malformations include tricuspid valve prolapse or regurgitation from the underdevelopment of one or more leaflets, absence of the papillary muscles or chordae, or annular dilation. In addition, tricuspid regurgitation can occur after previous cardiac operations that may include the atrial switch procedure (Mustard and Senning procedure), atrioventricular canal defect, ventricular septal defect, corrected transposition of the great arteries, and hypoplastic left heart syndrome. Functional causes are usually because of right ventricular outflow obstruction or right ventricular dysfunction. Functional tricuspid regurgitation can also occur with recurrent right ventricular outflow tract obstruction, especially when combined with pulmonary regurgitation, after repair of lesions such as tetralogy of Fallot, pulmonary atresia, or prior right ventricle-to-pulmonary artery conduits.
Acquired tricuspid valve malformations
Acquired causes of tricuspid stenosis can include carcinoid heart disease, rheumatic valvular heart disease, fibroelastosis, systemic lupus erythematosis, right atrial tumors, prior radiation therapy, pericardial disease, catheter entanglements, and pacemaker leads. Rheumatic valvular heart disease represents the most common acquired cause of tricuspid stenosis. Acquired causes of tricuspid regurgitation include rheumatic valvular heart disease, annular dilation, infective endocarditis, right ventricular hypertension, trauma, right ventricular infarction, cardiomyopathy, prior radiation therapy, drug exposure (ergot alkaloids and anorexigens), chronic heart failure, or catheter/lead entanglement. In addition, tricuspid regurgitation can occur after previous cardiac operations such as orthotopic heart transplantation. Rheumatic valvular heart disease also represents the most common acquired cause of tricuspid regurgitation.
III. Analysis: a unified tricuspid valve disease and Ebstein’s anomaly diagnostic hierarchical nomenclature system
Tricuspid valve disease and Ebstein’s anomaly hierarchy level 1
Tricuspid valve disease and Ebstein’s anomaly hierarchy level 1 definitions
A spectrum of lesions characterized by the presence of tricuspid regurgitation, tricuspid stenosis, or a combination of both. The lesion may or may not result from Ebstein’s anomaly.
Tricuspid valve disease and Ebstein’s anomaly hierarchy level 2
Tricuspid valve disease and Ebstein’s anomaly hierarchy level 2 definitions
Tricuspid valve disease, Ebstein’s anomaly
Ebstein’s anomaly is a malformation of the tricuspid valve that is characterized by a spectrum of multiple features described above. These anatomical and functional abnormalities related to Ebstein’s anomaly usually cause important tricuspid regurgitation, but uncommonly may cause tricuspid stenosis.
Tricuspid valve disease, tricuspid regurgitation
Tricuspid regurgitation can be functional or anatomic and can be caused by a number of congenital and acquired lesions. For the purposes of classification, patients in this category are those with tricuspid regurgitation not because of Ebstein’s anomaly.
Tricuspid valve disease, tricuspid stenosis
Tricuspid stenosis can be caused by congenital or acquired lesions that can result in right atrial hypertension or a right to left atrial shunt in the presence of an atrial septal defect.
Tricuspid valve disease, tricuspid stenosis and regurgitation
Tricuspid stenosis and regurgitation can occur simultaneously and be due to congenital and/or acquired lesions.
Tricuspid valve disease and Ebstein’s anomaly hierarchy level 3
Tricuspid valve disease and Ebstein’s anomaly hierarchy level 3 definitions
Tricuspid valve disease, Ebstein’s anomaly, type I
The anterior leaflet is large and mobile with complete delamination of 50% or more of its surface area. The leading edge can be freely mobile, have hyphenated attachments, or linear attachment. The posterior and septal leaflets may be present and displaced, dysplastic and displaced, or absent. Fenestrations are common. The atrialized ventricular chamber size varies from relatively small to large.
Tricuspid valve disease, Ebstein’s anomaly, type II
In this unusual variant, the anterior, posterior, and often septal leaflets are present but relatively small and displaced in a spiral fashion toward the apex. There is mobility of the anterior, posterior, and often septal leaflets that are supported by small, short papillary muscles and chordae. The leading edge of the anterior leaflet is most often freely mobile. The atrialized ventricular chamber is moderately large.
Tricuspid valve disease, Ebstein’s anomaly, type III
The anterior leaflet has restricted motion with shortened, fused, and tethered chordae. Direct insertion of papillary muscles into the anterior leaflet are frequently present. The anterior leaflet may be large or small, and the leading edge usually has hyphenated or linear attachments. The posterior and septal leaflets are displaced, dysplastic, and usually not reconstructable. Fenestrations are common. The atrialized chamber is large.
Tricuspid valve disease, Ebstein’s anomaly, type IV
The anterior leaflet is severely deformed and displaced into the right ventricular outflow tract. There may be few or no chordae, and direct insertions of papillary muscles into the leading edge of the valve are common. The leading edge usually has hyphenated or linear attachments. The posterior leaflet is typically dysplastic or absent, and the septal leaflet is represented by a ridge of fibrous tissue descending apically from the membranous septum. Tricuspid valve tissue displaced into the right ventricular outflow tract may cause obstruction of blood flow (functional tricuspid stenosis). Nearly all of the right ventricular cavity is atrialized ventricle.
Tricuspid valve disease, Ebstein’s anomaly, "left-sided" and atypical types
"Left-sided" Ebstein’s anomaly associated with atrioventricular discordance and ventriculoarterial discordance (corrected transposition) and atypical Ebstein-like anomalies associated with pulmonary atresia with intact ventricular septum and other hypoplastic right heart syndromes.
GUEST EDITORS’ NOTE: Type I hearts are ideal for tricuspid valve repair if the leading edge is not severely hyphenated or linearly attached. Type II hearts are also ideal for valve repair, particularly with an annuloplasty technique performed at the level of the displaced annulus. Type III hearts are borderline for repair. A reasonable surgical option is to attempt a repair and then evaluate the result by intraoperative echocardiography. If the repair is good to excellent, it can be accepted, and if not, tricuspid valve replacement can be performed. Type IV hearts uniformly require tricuspid valve replacement for a satisfactory result. Numerous valvuloplasty techniques have been described since the first report of Hunter and Lillehei [18–26].
Tricuspid valve disease, tricuspid regurgitation, congenital
Tricuspid regurgitation can be functional or anatomic. Functional tricuspid regurgitation is usually because of right ventricular outflow tract obstruction or right ventricular dysfunction. Organic causes from anatomical malformations include tricuspid valve prolapse or tricuspid regurgitation from the underdevelopment of one or more leaflets, absence of the papillary muscles or chordae, or annular dilatation.
Tricuspid valve disease, tricuspid regurgitation, acquired
Acquired causes of tricuspid regurgitation include annular dilatation, infective endocarditis, right ventricular hypertension, trauma, right ventricular infarction, cardiomyopathy, or chronic heart failure.
Tricuspid valve disease, tricuspid regurgitation, status post-cardiac surgery
Tricuspid regurgitation can occur after previous cardiac operations that may include the atrial switch procedure, atrioventricular canal defect, ventricular septal defect, corrected transposition of the great arteries, hypoplastic left heart syndrome, previous valve repair or valve replacement, to name a few. Significant tricuspid regurgitation can also occur after orthotopic cardiac transplantation.
Tricuspid valve disease, tricuspid stenosis, congenital
Tricuspid stenosis may result from valvular hypoplasia or shortening, thickening, or fusion of the subvalvar apparatus. In addition, the stenosis can be from a double-orifice tricuspid valve or a parachute deformity with all chordae arising from a single papillary muscle.
Tricuspid valve disease, tricuspid stenosis, acquired
Acquired causes of tricuspid stenosis can include carcinoid heart disease, rheumatic valvular heart disease, fibroelastosis, systemic lupus erythematosis, cardiac tumor (eg, right atrial myxoma), noncardiac tumor (eg, renal cell carcinoma), pericardial disease, catheter entanglements, and pacemaker leads.
Tricuspid valve disease, tricuspid stenosis, status post-cardiac surgery
Tricuspid stenosis after cardiac surgery can result from valvuloplasty techniques or complications stemming from operations involving the tricuspid valve.
Tricuspid valve disease, tricuspid stenosis and regurgitation (congenital)
A combination of tricuspid stenosis and tricuspid regurgitation can be from a congenital malformation described above.
Tricuspid valve disease, tricuspid stenosis and regurgitation (acquired)
A combination of tricuspid stenosis and tricuspid regurgitation from an acquired cause as described above.
Tricuspid valve disease and Ebstein’s anomaly hierarchy level 4
Tricuspid valve disease and Ebstein’s anomaly hierarchy level 4 definitions
Anterior leaflet leading edge
Freely mobile: no attachment of the leading edge to underlying endocardium
Hyphenated attachment
A portion of the leading edge is free and other portions of the leading edge are directly attached to the underlying endocardium (ie, focal, segmental direct attachments)
Linear attachment
The entire leading edge of the anterior leaflet is directly attached to the underlying endocardium
Tricuspid valve disease and Ebstein’s anomaly therapeutic hierarchy
Hierarchy level 1
Hierarchy level 2
Additional treatment options specific to Ebstein’s anomaly
Additional comments regarding therapeutics
In addition to the above basic treatment components for Ebstein’s anomaly, other important therapeutic issues must be addressed and coded in other areas of the database. Although tricuspid valve repair or replacement is the principle element in the surgical treatment for Ebstein’s anomaly, there are other important adjuncts to the operation that need to be considered. First, there is frequently an associated atrial septal defect or patent foramen ovale. Because the defect is usually large or the atrial septum attenuated, thus requiring excision, the defect frequently requires a patch to repair (autologous pericardium, bovine pericardium, gluteraldehyde-treated autologous pericardium, etc). Second, electrophysiologic mapping for localization and ablation of accessory conduction pathways in patients with ventricular preexcitation may be necessary [3]. Third, the incidence of supraventricular arrhythmias such as atrial flutter and fibrillation (paroxysmal and chronic) occurs with an increased frequency as the right atrium becomes enlarged. Under these circumstances, a right-sided maze procedure is often performed [27]. Fourth, in unusual cases of Ebstein’s anomaly during the neonatal and infant periods, an approach toward a univentricular repair may be considered [28]. This can involve the creation of either a central or systemic-to-pulmonary artery shunt, a bidirectional cavopulmonary anastomosis, and a Fontan type of connection. In severe cases of Ebstein’s anomaly, especially during infancy, orthotopic cardiac transplantation may be a treatment option. In the rare case of Ebstein’s anomaly of the left-sided tricuspid valve, a code will be required. Finally, the correction of associated anomalies such as relief of pulmonic stenosis, closure of ventricular septal defect, and others will need to be coded. In rare circumstances, the operative procedure may include surgical correction of an associated anomaly without definitive treatment of the tricuspid valve.
In the setting of pericardial constriction (constrictive pericarditis) as a cause for acquired tricuspid regurgitation, radical or partial pericardiectomy may be the treatment of choice and thus would need to be coded.
IV. Diagnosis and procedure short lists
V. Diagnosis-related potential risk factors
VI. Outcome tables
Ebstein’s anomaly surgery type (by year)
This table will show the number and percentage of each major Ebstein’s type (according to Ebstein’s anomaly [EA] hierarchy level 3) for each year. (If no EA subtype is given, the EA will be classified as EA, NOS.) (All tables below will break down the date for each given year of data collection and also will provide the total date of the cumulative experience.)
Ebstein’s anomaly age (years) at operation for each EA type (by year)
This table will show the distribution of age at operation for each major EA type for each year.
Ebstein’s anomaly gender distribution for each EA type (by year)
This table will show the gender for each major EA type for each year.
Ebstein’s features of repair—cardiopulmonary bypass (by year)
This table will show the number and percentage of each major EA type treated with cardiopulmonary bypass for each year.
Ebstein’s anomaly features of repair—aortic cross-clamp (by year)
This table will show the number and percentage of each major EA type treated with aortic cross-clamping for each year.
Ebstein’s anomaly features of repair—myocardial preservation (cardioplegia type) (by year)
For patients treated with cross clamping, this table will show the number and percentage of each major EA type treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other.
Ebstein’s anomaly features of repair (by year)
This table will show the number and percentage of each repair technique for each major EA type for each year.
Ebstein’s anomaly features of replacement (by year)
This table will show the number and percentage of tricuspid valve replacement done for each major EA type for each year.
Ebstein’s anomaly complication incidence (including operative death) (by year)
This table will show the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system [cardiac, pulmonary, renal, infectious, neurologic]) for each major EA type for each year.
Ebstein’s anomaly complication incidence (including operative death) patients less than 1 year of age (by year)
For patients less than 1 year of age, this table will show the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system [cardiac, pulmonary, renal, infectious, neurologic]) for each major EA type for each year.
Ebstein’s anomaly complication incidence (including operative death) patients more than 1 year of age (by year)
For patients 1 year of age or older, this table will show the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system [cardiac, pulmonary, renal, infectious, neurologic]) for each major EA type for each year.
Ebstein’s anomaly postoperative length of ventilation (hours) (by year)
This table will show the postoperative length of ventilation for each major EA type for each year.
Ebstein’s anomaly total length of ventilation (hours) (by year)
This table will show the total length of ventilation for each major EA type for each year.
Ebstein’s anomaly postoperative length of stay (days) (by year)
This table will show the postoperative length of stay for each major EA type for each year.
Ebstein’s anomaly total length of stay (days) (by year)
This table will show the total length of stay for each EA type for each year.
Ebstein’s anomaly postoperative length of stay (days) by patient age (by year)
This table will show the postoperative length of stay for each major EA type for each year, comparing patients less than 1 year to those 1 year of age or older.
Ebstein’s anomaly total length of stay (days) by patient age (by year)
This table will show the total length of stay for each major EA type for each year, comparing patients less than 1 year with those 1 year or older.
Kaplan-Meier curves
Kaplan-Meier survival curves should be generated for each major EA type for each year, comparing the total EA cohort with patients less than 1 year with those 1 year or older.
Ebstein’s anomaly complication incidence (including operative death) versus myocardial preservation [cardioplegia type] (by year)
This table will compare the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system [cardiac, pulmonary, renal, infectious, neurologic]), in patients treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other, for each major EA type for each year.
Ebstein’s anomaly complication incidence (including operative death) versus repair versus replacement
This table will compare the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system [cardiac, pulmonary, renal, infectious, and neurologic]), in patients treated with repair versus replacement, for each major EA type for each year.
Ebstein’s anomaly postoperative length of ventilation (hours) versus repair and replacement technique (by year)
This table will compare the postoperative length of ventilation, in patients treated with repair versus replacement, for each major EA type for each year.
Ebstein’s anomaly preoperative length of stay (days) (by year)
This table will show the preoperative length of stay for each major EA type for each year.
Ebstein’s anomaly same day surgery (by year)
This table will show the number and percentage of day of surgery admissions for each major EA type for each year.
Ebstein’s anomaly preoperative length of stay (days) by patient age (by year)
This table will show the preoperative length of stay for each major EA type for each year, comparing patients less than 1 year with those 1 year or older.
Ebstein’s anomaly same day surgery by patient age (by year)
This table will show the number and percentage of day of surgery admissions for each major EA type for each year, comparing patients less than 1 year with those 1 year or older.
Ebstein’s anomaly complication incidence (including operative death) versus preoperative ventilation (by year)
This table will compare the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system [cardiac, pulmonary, renal, infectious, and neurologic]), in patients treated with and without preoperative ventilation, for each major EA type for each year.
Ebstein’s anomaly postoperative length of ventilation (hours) versus preoperative ventilation (by year)
This table will compare the postoperative length of ventilation, in patients treated with and without preoperative ventilation, for each major EA type for each year.
Ebstein’s anomaly postoperative length of stay (days) versus preoperative ventilation (by year)
This table will compare the postoperative length of stay, in patients treated with and without preoperative ventilation for each major EA type for each year.
References
This article has been cited by other articles:
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 S. Paranon and P. Acar Ebstein's anomaly of the tricuspid valve: from fetus to adult Heart, February 1, 2008; 94(2): 237 - 243. [Full Text] [PDF]
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 C. H. Attenhofer Jost, H. M. Connolly, J. A. Dearani, W. D. Edwards, and G. K. Danielson Ebstein's Anomaly Circulation, January 16, 2007; 115(2): 277 - 285. [Full Text] [PDF]
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 G. E. Sarris, N. M. Giannopoulos, A. J. Tsoutsinos, A. K. Chatzis, G. Kirvassilis, W. J. Brawn, J. V. Comas, A. F. Corno, D. Di Carlo, J. Fragata, et al. Results of surgery for Ebstein anomaly: A multicenter study from the European Congenital Heart Surgeons Association J. Thorac. Cardiovasc. Surg., July 1, 2006; 132(1): 50 - 57. [Abstract] [Full Text] [PDF]
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H.-Y. Yu, Y.-S. Chen, W.-Y. Tseng, and F.-Y. Lin Combined right atrial and ventricular reduction operation: Case report of unrolling-rolling of the right ventricle to preserve ventricular muscle orientation J. Thorac. Cardiovasc. Surg., November 1, 2002; 124(5): 1045 - 1047. [Full Text] [PDF]
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 I. M. Vettraino, R. Huang, and C. H. Comstock The Normal Offset of the Tricuspid Septal Leaflet in the Fetus J. Ultrasound Med., October 1, 2002; 21(10): 1099 - 1104. [Abstract] [Full Text] [PDF]
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