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Ann Thorac Surg 2000;69:S18-S24
© 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, Division of Thoracic and Cardiovascular Surgery, All Children’s Hospital, 603 Seventh Str 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 atrial septal defect (ASD) 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. 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
An atrial septal defect (ASD) is defind as an opening or hole in the interatrial septum. ASD nomenclature is less controversial than ventricular septal defect (VSD) nomenclature. ASD nomenclature is based upon knowledge of the developmental anatomy of the atrial septum [1].
In utero, systemic veins drain into the sinus venosus and then the primitive atrium [2]. As the atrium develops, the thin septum primum forms the initial atrial septum and obliterates the foramen primum. The foramen secundum simultaneously forms in the superior portion of the septum primum. The atrial roof then folds inward and leads to the formation of the thickened septum secundum, which lies to the right and superior to septum primum and ends in the limbus, its curved, oval-shaped inferior free edge. The foramen ovale is formed when the septum secundum incompletely closes the foramen secundum. The mobile septum primum fuses with septum secundum at the limbus at birth when left atrial pressures exceed right atrial pressures. The remnant of the right horn of the sinus venosus extends from the superior vena cava (SVC) orifice to the inferior vena cava (IVC) orifice overlying the entrance of the right pulmonary veins into the left atrium [1]. The remnant of the left horn of the sinus venosus contributes to the formation of the wall of the coronary sinus [2]. Defective development of any of these atrial components can lead to ASDs.
II. Analysis: a unified ASD nomenclature system
ASD hierarchy level 1
ASD hierarchy level 1 definitions
ASD: An opening or hole in the interatrial septum
ASD hierarchy level 2
ASD hierarchy level 2 definitions (Fig 1)
ASD, NOS (not otherwise stated)
This designation allows for patient entries from other database schemes if no specific subtype is characterized or assigned.
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ASD, coronary sinus (synonym: unroofed coronary sinus)
A deficiency of the wall separating the left atrium from the coronary sinus, often allowing blood to shunt from the left atrium to the right atrium via the coronary sinus [5]. A deficiency of the remnant of the left horn of the sinus venosus leads to this defect [1].
ASD, PFO
A small interatrial communication in the region of the foramen ovale, which is characterized by no deficiency of septum primum and a normal limbus with no deficiency of septum secundum.
This definition was debated extensively at the First International Nomenclature Conference for Pediatric Cardiac Surgery, Chicago, Illinois, September 19–20, 1998. While some present argued for echocardiographic criteria to separate "ASD, PFO" from "ASD, Secundum," it remains crucial that these entities have definitions that will be viable without relying on one given diagnostic test (diagnostic tests will continue to evolve over time [6]). Some also argued for a strict measurement criteria to make a distinction between "ASD, PFO" and "ASD, Secundum," but this would not be feasible without indexing the measurement to patient size, and therefore, would be cumbersome and not practical.
A large amount of anatomic variability exists between the various ASDs present in the fossa ovalis [7]. Most surgeons will be able to distinguish between "ASD, PFO" from "ASD, Secundum" based on the above definition.
This definition was again debated extensively at the Second International Nomenclature Conference for Pediatric Cardiac Surgery, San Antonio, Texas, January 23, 1999. At the Second International Nomenclature Conference for Pediatric Cardiac Surgery, the decision was made that "PFO" would not be considered a subtype of ASD on the short diagnosis list (see "Diagnosis and Procedure Short Lists"); "PFO" would thus be a separate diagnostic listing on the short diagnosis list. Although "PFO" is accessed via the ASD hierarchy in the hierarchical nomenclature system, it can be analyzed separately from the remaining types of ASD.
ASD, primum
A usually crescent-shaped ASD in the inferior portion of the atrial septum just above the AV valve.
The proper location in the nomenclature system for lesions classified as "ASD, Primum" or any subtypes of "ASD, Primum" was also debated extensively at the First International Nomenclature Conference for Pediatric Cardiac Surgery, Chicago, Illinois, September 19–20, 1998. Some felt strongly that these lesions should be grouped with ASD [8], and others feel strongly that these lesions should be grouped with atrioventricular canal defects (AV canal) [4].
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 was also debated extensively at the Second International Nomenclature Conference for Pediatric Cardiac Surgery, San Antonio, Texas, January 23, 1999. At the Second International Nomenclature Conference for Pediatric Cardiac Surgery, 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. 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.
ASD, secundum
An ASD confined to the region of the fossa ovalis. The most common etiology is a deficiency of septum primum, but deficiency of the limbus or septum secundum may also contribute [1].
ASD, sinus venosus
An ASD in the region of the remnant right horn of the sinus venosus, usually in the region of the superior sinoatrial junction near the SVC orifice, but rarely posterior to the fossa ovalis not near either caval orifice or in the region of the inferior sinoatrial junction near the IVC orifice [1].
Numerous anatomists and pathologists have stressed that the "ASD, sinus venosus" would more properly be termed a sinus venosus defect rather than a sinus venosus ASD because the lesion represents an interatrial communication rather than a defect of atrial septal tissue [9–11]. While this does indeed appear to be true, most surgeons still group this lesion as a type of ASD.
ASD hierarchy level 3
ASD hierarchy level 3 definitions
ASD, secundum, fenestrated; ASD, secundum, nonfenestrated
These variables are important as we record data on the closure of secundum ASDs by both surgical and interventional cardiology approaches.
ASD hierarchy level 4
ASD hierarchy level 4 definitions
ASD, sinus venosus, SVC type, NOS; ASD, sinus venosus, SVC type, No PAPVR; ASD, sinus venosus, SVC type, PAPVR; ASD, sinus venosus, IVC type, NOS; ASD, sinus venosus, IVC type, No PAPVR; ASD, sinus venosus, IVC type, PAPVR
Obviously, sinus venosus ASD is associated with PAPVR. This level 4 hierarchy allows this association to be coded directly. The specific type of PAPVR can then be coded and described in detail separately under PAPVR. The PAPVR section of this coding system will allow for detailed coding of the specific PAPVR type, including PAPVR seen with sinus venosus ASD, other types of ASD, and without associated ASD, including the Scimitar syndrome.
Additional modifiers for ASD: hierarchy level 1
Additional modifiers for ASD: hierarchy level 1 definitions
ASD, persistent (postoperative intentional)
Any ASD that was intentionally left open at a previous operation.
ASD, previously created (iatrogenic)
Any ASD that was surgically created at a previous operation or intervention.
ASD, residual
Any ASD that had attempted closure or was missed at the time of prior surgery or intervention.
Additional modifiers for ASD: hierarchy level 2
III. Nomenclature for ASD treatment options
ASD treatment hierarchy level 1
ASD treatment hierarchy level 2
ASD treatment hierarchy level 3
Additional comments regarding therapeutics
In addition to the above basic treatment options for ASD, several other therapeutic issues must be addressed and coded in other areas of the database. First, 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). Second, 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. Diagnosis 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 ASD include the following data fields.
Atrial arrhythmias after "ASD repair, sinus venosus," comparing the incidence of atrial arrhythmias such as sinus node dysfunction with the various repair types including
Specific postoperative complications of ASD to be studied include:
1.5:1 VI. Database studies and outcome analysis
ASD: inclusion criteria and allowable concomitant diagnoses
A case is included for ASD analysis if the primary diagnosis is ASD and concomitant cardiac diagnoses are none, left superior vena cava, PDA, or any combination of these. If no ASD subtype is given, the ASD will be classified as ASD, NOS.
Outcome tables
ASD surgery type (by year)
This table will show the number and percentage of each major ASD type (according to ASD hierarchy level 2) for each year. (If no ASD subtype is given, the ASD will be classified as ASD, NOS.) (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.) (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.)
ASD method of diagnosis for each ASD type (by year)
This table will show the method of diagnosis for each major ASD type for each year.
ASD age (years) at operation for each ASD type (by year)
This table will show the distribution of age at operation for each major ASD type for each year.
ASD gender distribution for each ASD type (by year)
This table will show the gender for each major ASD type for each year.
ASD features of repair: cardiopulmonary bypass (by year)
This table will show the number and percentage of each major ASD type treated with cardiopulmonary bypass for each year.
ASD features of repair: aortic cross-clamp (by year)
This table will show the number and percentage of each major ASD type treated with aortic cross-clamping for each year.
ASD features of repair: induced fibrillation (by year)
This table will show the number and percentage of each major ASD type treated with induced fibrillation for each year.
ASD 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 ASD type treated with circulatory arrest for each year.
ASD features of repair: percent of patients less than 6 months having deep hypothermia and circulatory arrest (by year)
For patients less than 6 months, this table will show the number and percentage of each major ASD type treated with circulatory arrest for each year.
ASD features of repair: percent of patients 6 months or older having deep hypothermia and circulatory arrest (by year)
For patients 6 months or older, this table will show the number and percentage of each major ASD type treated with circulatory arrest for each year.
ASD 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 ASD type treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other.
ASD 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 ASD type for each year.
ASD features of repair: closure technique (by year)
This table will show the number and percentage of primary (suture), patch, and device closure for each major ASD type for each year.
ASD 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 ASD type for each year.
ASD complication incidence (including operative death) for patients less than 1 year of age (by year)
For patients less than 1 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 ASD type for each year.
ASD complication incidence (including operative death) for patients 1 year or older of age (by year)
For patients 1 year 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 ASD type for each year.
ASD preoperative length of ventilation (hours) (by year)
This table will show the preoperative length of ventilation for each major ASD type for each year.
ASD postoperative length of ventilation (hours) (by year)
This table will show the postoperative length of ventilation for each major ASD type for each year.
ASD total length of ventilation (hours) (by year)
This table will show the total length of ventilation for each major ASD type for each year.
ASD preoperative length of stay (days) (by year)
This table will show the preoperative length of stay for each major ASD type for each year.
ASD same-day surgery (by year)
This table will show the number and percentage of day of surgery admissions for each major ASD type for each year.
ASD postoperative length of stay (days) (by year)
This table will show the postoperative length of stay for each major ASD type for each year.
ASD total length of stay (days) (by year)
This table will show the total length of stay for each major ASD type for each year.
ASD preoperative length of stay (days) by patient age (by year)
This table will show the preoperative length of stay for each major ASD type for each year, comparing patients less than 1 year with those 1 year or more.
ASD same-day surgery by patient age (by year)
This table will show the number and percentage of day of surgery admissions for each major ASD type for each year, comparing patients less than 1 year with those 1 year or more.
ASD postoperative length of stay (days) by patient age (by year)
This table will show the postoperative length of stay for each major ASD type for each year, comparing patients < 1 year with those 1 year.
ASD total length of stay (days) by patient age (by year)
This table will show the total length of stay for each major ASD type for each year, comparing patients less than 1 year with those 1 year or more.
Kaplan-Meier curves
Kaplan-Meier survival curves should be generated for each major ASD type for each year, comparing the total ASD cohort with patients less than 1 year with those 1 year or more.
ASD 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 ASD type for each year.
ASD 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 ASD type for each year.
ASD 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 ASD type for each year.
ASD 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 ASD type for each year.
ASD 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 primary (suture), patch, and device closure, for each major ASD type for each year.
ASD 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 ASD type for each year.
ASD 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 ASD type for each year.
ASD 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 ASD type for each year.
ASD 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 ASD type for each year.
ASD postoperative length of ventilation (hours) versus closure technique (by year)
This table will compare the postoperative length of ventilation, in patients treated with primary (suture), patch, and device closure, for each major ASD type for each year.
ASD 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 ASD type for each year.
ASD 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 ASD type for each year.
ASD 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 ASD type for each year.
ASD 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 ASD type for each year.
ASD postoperative length of stay (days) versus closure technique (by year)
This table will compare the postoperative length of stay, in patients treated with primary (suture), patch, and device closure, for each major ASD type for each year.
References
This article has been cited by other articles:
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  A Sarkozy, E Conti, C Neri, R D'Agostino, M C Digilio, G Esposito, A Toscano, B Marino, A Pizzuti, and B Dallapiccola Spectrum of atrial septal defects associated with mutations of NKX2.5 and GATA4 transcription factors J. Med. Genet., February 1, 2005; 42(2): e16 - e16. [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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