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Ann Thorac Surg 2000;69:S36-S43
© 2000 The Society of Thoracic Surgeons
a Division of Thoracic and Cardiovascular Surgery, All Children’s Hospital, University of South Florida School of Medicine, St. Petersburg, Florida, USA
b Division of Cardiovascular Surgery, Miami Children’s Hospital, Miami, Florida, USA
c Division of Thoracic and Cardiovascular Surgery, Northwestern University Medical School, Chicago, Illinois, USA
Address reprint requests to Dr Jacobs, Divison of Thoracic and Cardiovascular Surgery, All Children’s Hospital, 603 Seventh St S, Suite 450, St. Petersburg, FL 33701
e-mail: jjacobs1{at}compuserve.com
Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.
Abstract
The extant nomenclature for atrioventricular (AV) canal/atrioventricular septal defect is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The three general categories are: partial AV canal (ostium primum defect), transitional (intermediate) AV canal, and complete AV canal. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. 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 various risk factors are presented.
I. Background
Atrioventricular canal defect (AVC) has also been called endocardial cushion defect and atrioventricular septal defect (AVSD). These defects are characterized by varying degrees of incomplete development of the septal tissue surrounding the atrioventricular (AV) valves along with varying degrees of abnormalities of the AV valves themselves. Consequently, AVC may include defects in the inferior (posterior) portion of the atrial septum, defects in the inflow portion of the ventricular septum, and defects in the tissue forming the left and right AV valves (LAVV and RAVV).
AVC represents a spectrum of cardiac anomalies subdivided into partial AVC, transitional AVC, and complete AVC [1]. Partial AVC (also known as incomplete AVC or partial AVSD [PAVSD]) has an ostium primum atrial septal defect (ASD) (a crescent-shaped ASD in the inferior portion of the atrial septum just above the AV valve) and may have varying degrees of malformation of the LAVV leading to varying degrees of LAVV regurgitation. Complete AVC (also known as complete AVSD [CAVSD]) has both defects in the atrial septum just above the AV valves (ostium primum ASD) and defects in the ventricular septum just below the AV valves. In CAVSD, the AV valve is one valve that bridges both the right and left sides of the heart, creating superior (anterior) and inferior (posterior) bridging leaflets. Transitional AVC (also known as intermediate AVC) is in the middle of this spectrum and has two distinct LAVV and RAVV orifices, but also has both an ASD just above and a VSD just below the AV valves. Although these AV valves in the intermediate form do form two separate orifices, they remain abnormal valves. The VSD in this lesion is often restrictive.
The Rastelli classification, originally described in 1966 [2], describes three types of complete AVC based on the morphology of the superior (anterior) bridging leaflet, its degree of bridging, and its chordal attachments. The Rastelli classification does not relate to the anatomy of the inferior (posterior) bridging leaflet because this leaflet displays greater anatomic variation, and no consistent relationship exists between the morphology of the superior (anterior) bridging leaflet and that of the inferior (posterior) bridging leaflet. In a Rastelli type A defect, the common superior (anterior) bridging leaflet is effectively split in two at the septum: the left superior (anterior) leaflet is entirely over the left ventricle, and the right superior (anterior) leaflet is similarly entirely over the right ventricle. The division of the common superior (anterior) bridging leaflet into left and right components is caused by extensive attachment of the superior (anterior) bridging leaflet to the crest of the ventricular septum by chordae tendineae. In many cases, this chordal attachment pulls the plane of the AV valve down into the ventricular septal defect (VSD) below the plane of the annulus. Rastelli type B is rare and involves anomalous papillary muscle attachment from the right side of the ventricular septum to the left side of the common superior (anterior) bridging leaflet. In Rastelli type C defects, there is marked bridging of the ventricular septum by the superior (anterior) bridging leaflet. The superior (anterior) bridging leaflet is generally not divided and floats freely over the ventricular septum without chordal attachment to the crest of the ventricular septum (Fig 1).
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AVC hierarchy level 1
AVC hierarchy level 1 definitions
AVC (synonyms: atrioventricular canal defect (AVC), atrioventricular septal defect (AVSD), endocardial cushion defect): A spectrum of lesions characterized by incomplete formation of the inferior (posterior) portion of the atrial septum along with varying degrees of incomplete formation of the inflow portion of the ventricular septum and the atrioventricular valves.
Significant debate in the literature remains as to whether the best term for these lesions is AVC or AVSD [3–5]. The purpose of this nomenclature system is not to validate either term but instead to allow surgeons and centers using either terminology to share data meaningfully.
AVC hierarchy level 2
AVC hierarchy level 2 definitions
AVC (AVSD), partial (incomplete) (PAVSD) (ASD, primum)
An AVC with an ostium primum ASD (a usually crescent-shaped ASD in the inferior (posterior) portion of the atrial septum just above the AV valve) and varying degrees of malformation of the LAVV leading to varying degrees of LAVV regurgitation. No VSD is present. (At the Second International Nomenclature Conference for Pediatric Cardiac Surgery, San Antonio, Texas, January 23, 1999, the consensus was that this lesion did not exist without malformation of the LAVV.)
The proper location in the nomenclature system for lesions classified as "ASD, primum" or any subtypes of "ASD, primum" was debated extensively at the First International Nomenclature Conference for Pediatric Cardiac Surgery, Chicago, Illinois, September 19–20, 1998. Some felt that these lesions should be grouped with ASD [6] and others felt that these lesions should be grouped with atrioventricular canal defects (AV canal) because of the long-term outcome issues regarding reoperation, etc [7].
These lesions may be entered into the database either under ASD or AV canal. Thus, any given center or surgeon may decide to group these lesions under either ASD or AV canal. The database will then equate identical terms such as "ASD, Primum, cleft mitral valve" and "AVC (AVSD), partial (Incomplete) (PAVSD), (ASD, primum)."
Regardless of where in the database the lesion is entered, for our purposes of analysis, it was agreed that "lesions coded as "ASD, primum" or any subtypes of "ASD, primum" will not be analyzed with ASD but instead will be analyzed with atrioventricular canal defects."
This definition of atrioventricular canal defects was also debated extensively at the Second International Nomenclature Conference for Pediatric Cardiac Surgery, San Antonio, Texas, January 23, 1999. The decision was made that "ASD, primum" would not be considered a subtype of ASD on the short diagnosis list (see "Diagnosis and Procedure Short Lists"); "ASD, primum" would thus not be a choice on the short diagnosis list. "ASD, primum" would instead be coded under "AVC (AVSD), partial (incomplete) (PAVSD) (ASD, primum)" on the short diagnosis list (see "Diagnosis and Procedure Short Lists"). Although "ASD, primum" can be accessed via the ASD hierarchy in the hierarchical nomenclature system, it will not be analyzed with ASD but instead will be analyzed with atrioventricular canal defects.
AVC (AVSD), intermediate (transitional)
An AVC with two distinct LAVV and RAVV orifices but also with both an ASD just above and a VSD just below the AV valves. While these AV valves in the intermediate form do form two separate orifices, they remain abnormal valves. The VSD in this lesion is often restrictive.
AVC (AVSD), complete (CAVSD)
An AVC with both a defect in the atrial septum just above the AV valves (ostium primum ASD [a crescent-shaped ASD in the inferior (posterior) portion of the atrial septum just above the AV valve]) and a nonrestrictive defect in the ventricular septum just below the AV valves. In CAVSD, the AV valve is one valve that bridges both the right and left sides of the heart.
AVC hierarchy level 3
AVC hierarchy level 3 definitions
Balanced
An AVC with two essentially appropriately sized ventricles [6].
Unbalanced
An AVC with two ventricles in which one ventricle is inappropriately small but is thought to be a candidate for a biventricular repair [6]. AVC lesions with unbalanced ventricles so severe as to preclude biventricular repair should be classified as single ventricles.
GUEST EDITORS’ NOTE: The term "biventricular unbalanced AV canal" is difficult to pin down in light of a biventricular repair. It also leaves too much room for "gaming" the database, that is, making the patient more complex than he/she actually is. Although some favor removing this term from the hierarchy, others feel strongly to keep it in. We will keep it in place so as to remain inclusive. However, this term may not play a meaningful role in multiinstitutional analysis.
AVC hierarchy level 4
AVC hierarchy level 4 definitions
Rastelli type A
In a Rastelli type A defect, the common superior (anterior) bridging leaflet is effectively split in two at the septum: the left superior (anterior) leaflet is entirely over the left ventricle, and the right superior (anterior) leaflet is similarly entirely over the right ventricle. The division of the common superior (anterior) bridging leaflet into left and right components is caused by extensive attachment of the superior (anterior) bridging leaflet to the crest of the ventricular septum by chordae tendineae.
Rastelli type B
Rastelli type B is rare and involves anomalous papillary muscle attachment from the right side of the ventricular septum to the left side of the common superior (anterior) bridging leaflet.
Rastelli type C
In Rastelli type C defects, there is marked bridging of the ventricular septum by the superior (anterior) bridging leaflet. The superior (anterior) bridging leaflet floats freely over the ventricular septum without chordal attachment to the crest of the ventricular septum. The anterior bridging leaflet in this subgroup has been termed a "free-floater" [6].
AVC hierarchy level 5
III. Nomenclature for AVC defect treatment options
AVC treatment hierarchy level 1
AVC treatment hierarchy level 2
AVC treatment hierarchy level 3
AVC treatment hierarchy level 3 definitions
Debate in the literature continues concerning whether to name the left atrioventricular valve in this lesion a mitral valve or LAVV, and whether to call the "cleft" a cleft or commissure. Strong arguments have been made that "LAVV" and "commissure" are better terms [4, 8, 9]. The purpose of this database is not to settle these arguments, but to foster international sharing of data.
AVC treatment hierarchy level 4
Additional comments regarding therapeutics
In addition to the above basic treatment options for AVC, several other therapeutic issues must be addressed and coded in other areas of the database. First, separate areas of coding must cover palliative treatment of AVC (pulmonary artery banding and debanding). Second, a separate part of the database must allow for coding of incisions for this and all other diagnoses (median sternotomy, submammary incision, right thoracotomy, left thoracotomy, minimally invasive incisions [partial sternotomy, parasternal incision, "mini-thoracotomy"], etc). Third, a separate part of the database must allow for coding of cardiac incisions for this and all other diagnoses (aortotomy, pulmonary arteriotomy, right atriotomy, right ventriculotomy, left ventriculotomy, etc). Finally, a separate module of the database must permit coding of patch materials (Dacron, Gore-Tex [W.L. Gore & Associates, Flagstaff, AZ], bovine pericardium, autologous pericardium, gluteraldehyde fixated autologous pericardium, etc).
IV. Diagnois and procedure short lists
V. Potential diagnostic-related risk factors
In addition to the common data fields utilized in the minimal data set and comprehensive data set applicable to all lesions, specific data fields to be tracked for AVC include the following data fields: outcome analysis comparing cleft closure versus no cleft closure in "AVC (AVSD) repair, partial (incomplete) (PAVSD);" and outcome analysis comparing cleft closure versus no cleft closure, one-patch technique versus two-patch technique, and leaving the coronary sinus on the left versus the right in "AVC (AVSD) repair, complete (CAVSD)".
Specific postoperative complications of AVC to be studied include:
1.5:1 1.5:1 VI. Database studies and outcome analysis
AVC: inclusion criteria and allowable concomitant diagnoses
A case is included for AVC analysis if the primary diagnosis is AVC and concomitant cardiac diagnoses are none, left superior vena cava, PDA, ASD, VSD, or any combination of these. If no AVC subtype is given, the AVC will be classified as "AVC, NOS." Tetralogy of Fallot (TOF) combined with AVC will be coded and studied as a subtype of TOF. AVC lesions with unbalanced ventricles so severe as to preclude biventricular repair will be coded and studied as a subtype of single ventricles. Lesions coded as "ASD, primum" or any subtypes of "ASD, primum" will not be analyzed with ASD but instead will be analyzed with atrioventricular canal defects. These lesions will be analyzed as "AVC (AVSD), partial (incomplete) (PAVSD)."
Outcome tables
AVC surgery type (by year)
This table will show the number and percentage of each major AVC type (according to AVC hierarchy level 2) for each year. (If no AVC subtype is given, the AVC will be classified as AVC, NOS.) (All tables below will break down the data for each given year of data collection and also will provide the total data of the cumulative experience.)
AVC method of diagnosis for each AVC type (by year)
This table will show the method of diagnosis for each major AVC type for each year.
AVC age (years) at operation for each AVC type (by year)
This table will show the distribution of age at operation for each major AVC type for each year.
AVC gender distribution for each AVC type (by year)
This table will show the gender for each major AVC type for each year.
AVC number and percentage with prior pulmonary artery (PA) band for each AVC type (by year)
This table will show the number and percentage of each major AVC type treated with prior PA band for each year.
AVC features of repair: cardiopulmonary bypass (by year)
This table will show the number and percentage of each major AVC type treated with cardiopulmonary bypass for each year.
AVC features of repair: aortic cross-clamp (by year)
This table will show the number and percentage of each major AVC type treated with aortic cross-clamping for each year.
AVC features of repair: induced fibrillation (by year)
This table will show the number and percentage of each major AVC type treated with induced fibrillation for each year.
AVC features of repair: percent of patients having deep hypothermia and circulatory arrest (by year)
This table will show the number and percentage of each major AVC type treated with circulatory arrest for each year.
AVC features of repair: percent of patients < 6 months having deep hypothermia and circulatory arrest (by year)
For patients < 6 months, this table will show the number and percentage of each major AVC type treated with circulatory arrest for each year.
AVC features of repair: percent of patients 6 months having deep hypothermia and circulatory arrest (by year)
For patients 6 months, this table will show the number and percentage of each major AVC type treated with circulatory arrest for each year.
AVC 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 AVC type treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other.
AVC features of repair: incision type (by year)
This table will show the number and percentage of each major incision type (median sternotomy, submammary incision, right thoracotomy, left thoracotomy, minimally invasive incisions [partial sternotomy, parasternal incision, "mini-thoracotomy"], etc) for each major AVC type for each year.
AVC features of repair: closure technique (by year)
This table will show the number and percentage of single-patch and double-patch closure for each major AVC type for each year.
AVC features of repair: cleft closure technique (by year)
This table will show the number and percentage of AVC repairs done with cleft MV repair and without cleft MV repair, for each major AVC type for each year.
AVC 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 AVC type for each year.
AVC complication incidence (including operative death) for patients < 6 months of age (by year)
For patients < 6 months, 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 AVC type for each year.
AVC complication incidence (including operative death) for patients 6 months of age (by year)
For patients 6 months, 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 AVC type for each year.
AVC preoperative length of ventilation (hours) (by year)
This table will show the preoperative length of ventilation for each major AVC type for each year.
AVC postoperative length of ventilation (hours) (by year)
This table will show the postoperative length of ventilation for each major AVC type for each year.
AVC total length of ventilation (hours) (by year)
This table will show the total length of ventilation for each major AVC type for each year.
AVC preoperative length of stay (days) (by year)
This table will show the preoperative length of stay for each major AVC type for each year.
AVC same-day surgery (by year)
This table will show the number and percentage of day of surgery admissions for each major AVC type for each year.
AVC postoperative length of stay (days) (by year)
This table will show the postoperative length of stay for each major AVC type for each year.
AVC total length of stay (days) (by year)
This table will show the total length of stay for each major AVC type for each year.
AVC preoperative length of stay (days) by patient age (by year)
This table will show the preoperative length of stay for each major AVC type for each year, comparing patients < 6 months with those 6 months.
AVC same-day surgery by patient age (by year)
This table will show the number and percentage of day of surgery admissions for each major AVC type for each year, comparing patients < 6 months with those 6 months.
AVC postoperative length of stay (days) by patient age (by year)
This table will show the postoperative length of stay for each major AVC type for each year, comparing patients < 6 months with those 6 months.
AVC total length of stay (days) by patient age (by year)
This table will show the total length of stay for each major AVC type for each year, comparing patients < 6 months with those 6 months.
Kaplan-Meier curves
Kaplan-Meier survival curves should be generated for each major AVC type for each year, comparing the total AVC cohort with patients < 6 months with those 6 months.
AVC 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, neurologic]), in patients treated with and without preoperative ventilation, for each major AVC type for each year.
AVC complication incidence (including operative death) versus deep hypothermia and circulatory arrest (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 and without circulatory arrest, for each major AVC type for each year.
AVC 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 AVC type for each year. hd5
AVC complication incidence (including operative death) versus incision 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 each major incision type (median sternotomy, submammary incision, right thoracotomy, left thoracotomy, minimally invasive incisions [partial sternotomy, parasternal incision, "mini-thoracotomy"], etc), for each major AVC type for each year.
AVC complication incidence (including operative death) versus closure technique (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 single patch and double patch closure, for each major AVC type for each year.
AVC complication incidence (including operative death) versus cleft closure technique (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 cleft MV repair and without cleft MV repair, for each major AVC type for each year.
AVC 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 AVC type for each year.
AVC postoperative length of ventilation (hours) versus deep hypothermia and circulatory arrest (by year)
This table will compare the postoperative length of ventilation, in patients treated with and without circulatory arrest, for each major AVC type for each year.
AVC postoperative length of ventilation (hours) versus myocardial preservation (cardioplegia type) (by year)
This table will compare the postoperative length of ventilation, in patients treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other, for each major AVC type for each year.
AVC postoperative length of ventilation (hours) versus incision type (by year)
This table will compare the postoperative length of ventilation, in patients treated with each major incision type (median sternotomy, submammary incision, right thoracotomy, left thoracotomy, minimally invasive incisions [partial sternotomy, parasternal incision, "mini-thoracotomy"], etc), for each major AVC type for each year.
AVC postoperative length of ventilation (hours) versus closure technique (by year)
This table will compare the postoperative length of ventilation, in patients treated with single-patch and double-patch closure, for each major AVC type for each year.
AVC postoperative length of ventilation (hours) versus cleft closure technique (by year)
This table will compare the postoperative length of ventilation, in patients treated with cleft MV repair and without cleft MV repair, for each major AVC type for each year.
AVC 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 AVC type for each year.
AVC postoperative length of stay (days) versus deep hypothermia and circulatory arrest (by year)
This table will compare the postoperative length of stay, in patients treated with and without circulatory arrest, for each major AVC type for each year.
AVC postoperative length of stay (days) versus myocardial preservation (cardioplegia type) (by year)
This table will compare the postoperative length of stay, in patients treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other, for each major AVC type for each year.
AVC postoperative length of stay (days) versus incision type (by year)
This table will compare the postoperative length of stay, in patients treated with each major incision type (median sternotomy, submammary incision, right thoracotomy, left thoracotomy, minimally invasive incisions [partial sternotomy, parasternal incision, "mini-thoracotomy"], etc.), for each major AVC type for each year.
AVC postoperative length of stay (days) versus closure technique (by year)
This table will compare the postoperative length of stay, in patients treated with single-patch and double-patch closure, for each major AVC type for each year.
AVC postoperative length of stay (days) versus cleft closure technique (by year)
This table will compare the postoperative length of stay, in patients treated with cleft MV repair and without cleft MV repair, for each major AVC type for each year.
References
This article has been cited by other articles:
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  C. L. Backer, R. D. Stewart, F. Bailliard, A. M. Kelle, C. L. Webb, and C. Mavroudis Complete Atrioventricular Canal: Comparison of Modified Single-Patch Technique With Two-Patch Technique Ann. Thorac. Surg., December 1, 2007; 84(6): 2038 - 2046. [Abstract] [Full Text] [PDF]
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 J. I. Aramendi, M. A. Rodriguez, T. Luis, and R. Voces No patch technique for complete atrioventricular canal repair Interactive CardioVascular and Thoracic Surgery, August 1, 2006; 5(4): 349 - 352. [Abstract] [Full Text] [PDF]
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 R. Pretre, H. Dave, A. Kadner, D. Bettex, and M. I. Turina Direct closure of the septum primum in atrioventricular canal defects J. Thorac. Cardiovasc. Surg., June 1, 2004; 127(6): 1678 - 1681. [Abstract] [Full Text] [PDF]
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 P. J. Gruber and J. A. Epstein Development Gone Awry: Congenital Heart Disease Circ. Res., February 20, 2004; 94(3): 273 - 283. [Abstract] [Full Text] [PDF]
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