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Ann Thorac Surg 2000;69:S25-S35
© 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 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 ventricular septal defect (VSD) 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. Four basic VSD types are described: Subarterial, Perimembranous, Inlet, and Muscular. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analysis. A minimum database set is also presented which 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
A ventricular septal defect (VSD) is defined as an opening or hole in the interventricular septum [1]. Isolated VSD occurs in approximately 2 out of every 1000 live births and constitutes over 20% of all congenital heart disease [2]. Perhaps because isolated VSD is the most commonly recognized form of congenital heart disease [3], numerous nomenclature schemes have been utilized to describe and classify this lesion. Three of the more commonly utilized VSD nomenclature systems will be presented.
Historically, VSDs have been assigned to one of four anatomic types [2]. Figure 1 represents a computer-generated diagram depicting this classic anatomic nomenclature. Type I VSDs are termed supracristal, infundibular, juxtaarterial, or conal. This defect lies caudad to the pulmonary valve in the infundibular portion of the right ventricular outflow tract. Type II VSDs are termed perimembranous or paramembranous and are located adjacent to the membranous portion of the ventricular septum and the septal leaflet of the tricuspid valve. Type III VSDs are termed inlet or atrioventricular canal VSDs and are located posteriorly at the inlet portion of the right ventricular septum (corresponding to the outlet portion of the left ventricular septum). Type IV VSDs are muscular defects and include a variety of single and multiple defects in the muscular septum.
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Obviously, numerous variations on these three VSD nomenclature proposals exist. A picture is worth a thousand words, so computer generated images can be utilized to demonstrate how the various previously described nomenclature systems can all coexist and be interrelated. Figure 4 represents a computer-generated depiction of the ventricular septum. Figures 5, 6, and 7 epict each of the three VSD nomenclature systems described above mapped onto this computer-generated depiction of the ventricular septum. This exercise allows for the creation and understanding of the unified nomenclature system proposed below.
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VSD hierarchy level 1
VSD hierarchy level 1 definitions
Ventricular septal defect (VSD)
An opening or hole in the interventricular septum.
VSD hierarchy level 2
VSD hierarchy level 2 definitions
VSD, NOS
A VSD not further described (NOS = not otherwise specified). This designation allows for patient entries from other database schemes if no specific subtype is characterized or assigned.
VSD, multiple
More than one VSD exists. Each individual VSD may then be coded separately to specify the individual VSD types. (In this hierarchical system, if a VSD is coded and VSD, Multiple is not selected, the patient is coded as having a single VSD.)
VSD, type 1
(Synonyms: subarterial VSD, supracristal VSD, conal VSD, infundibular VSD). A VSD that lies beneath the semilunar valve(s) in the conal or outlet septum [9].
VSD, type 2
(Synonyms: perimembranous, paramembranous, conoventricular). A VSD that is confluent with and involves the membranous septum and is bordered by an atrioventricular valve, not including the type 3 VSDs (see below).
VSD, type 3
(Synonyms: inlet, AV canal type). A VSD that involves the inlet of the right ventricular septum immediately inferior to the AV valve apparatus.
VSD, type 4
(Synonyms: Muscular). A VSD completely surrounded by muscle.
VSD, type: Gerbode type
(Synonym: Left ventricle-right atrium (LV-RA) fistula). A rare form of VSD in which the defect is at the membranous septum; the communication is between the LV and RA.
VSD hierarchy level 3
VSD hierarchy level 3 definitions
VSD, type 1 (subarterial) (supracristal) (conal septal defect) (infundibular)
VSD, type 1 (subarterial) (supracristal) (conal septal defect) (infundibular), NOS
A subarterial VSD not further described.
VSD, type 1 (subarterial) (supracristal) (conal septal defect) (infundibular), conal muscular
A subarterial VSD completely surrounded by muscle. This VSD lies in the conal or infundibular septum with muscle between the VSD and the semilunar valves.
VSD, type 1 (subarterial) (supracristal) (conal septal defect) (infundibular), juxtaarterial
A subarterial VSD that lies immediately beneath the semilunar valve(s) in the conal or outlet septum. This VSD is limited upstream by the semilunar valve(s), but is otherwise surrounded by muscle. Muscle does not separate the VSD from the semilunar valve(s).
VSD, type 2 (perimembranous), (paramembranous), (conoventricular)
VSD, type 2 (perimembranous), (paramembranous), (conoventricular), NOS
A perimembranous VSD not further described.
Note that many surgeons might utilize this choice as the term used to describe their most common type of VSD and consider further description into other hierarchical choices as irrelevant. The advantage of this hierarchical nomenclature system is that these surgeons can stop at this level and still share data with other surgeons who choose to code in more detail. Clearly, many surgeons might question the relevance of this additional detail, but other programs, especially some in Europe, will want to code using the terms trabecular, outlet, and confluent as defined below.
VSD, type 2 (perimembranous), (paramembranous), (conoventricular), inlet
A type 2 VSD which extends towards the inlet portion of the septum.
VSD, type 2 (perimembranous), (paramembranous), (conoventricular), trabecular
A type 2 VSD which extends towards the trabecular or apical septum.
VSD, type 2 (perimembranous), (paramembranous), (conoventricular), outlet
A type 2 VSD which extends toward the outlet portion of the septum.
VSD, type 2 (perimembranous), (paramembranous), (conoventricular), confluent
A type 2 VSD that extends towards multiple parts of the septum, including the inlet, trabecular, and outlet portions.
VSD, type 3 (inlet) (AV canal type)
VSD, type 3 (inlet) (AV canal type), NOS
An inlet VSD not further described.
VSD, type 3 (inlet) (AV canal type), associated with AV canal defect
An inlet VSD that is associated with an AV canal defect.
VSD, type 3 (inlet) (AV canal type), not associated with AV canal defect
An inlet VSD that is not associated with an AV canal defect.
VSD, type 4 (muscular)
VSD, type 4 (muscular), NOS
A muscular VSD not further described.
VSD, type 4 (muscular), multiple
More than one muscular VSD exists. Each individual muscular VSD may then be coded separately to specify the individual muscular VSD types.
VSD, type 4 (muscular), multiple—"Swiss-cheese"
More than three muscular VSDs exist. A variety of definitions have been used to define this entity [10–12] including uncountable multiple VSDs [12]. Nevertheless, for the purpose of this database, this term can be utilized if four or more muscular VSDs exist, as defined by Serraf and colleagues [10].
VSD, type 4 (muscular), inlet: (synonym: posterior)
A muscular VSD in the inlet portion of the ventricular septum. This term is used to describe an AV canal type VSD with muscle between the defect and the AV valve(s).
VSD, type 4 (muscular), trabecular
A muscular VSD in the trabecular portion of the septum.
VSD, type 4 (muscular), outlet
A muscular VSD extending towards the outlet portion of the septum.
VSD, type 4 (muscular), confluent
A muscular VSD that extends towards multiple parts of the septum, including the inlet, trabecular, and outlet portions.
VSD hierarchy level 4
VSD hierarchy level 4 definitions
VSD, type 1 (subarterial) (supracristal) (conal septal defect) (infundibular), juxtaarterial
(Subdivisions: NOS; juxtaarterial, doubly-committed). A type 1 subarterial VSD immediately beneath the semilunar valve(s) in the conal or outlet septum in which the superior rim of the defect is made up of the facing leaflets of both the aortic and pulmonary valves. (These facing leaflets are in fibrous continuity with each other in the superior rim of the defect [5]).
VSD, type 2 (perimembranous), (paramembranous), (conoventricular) (infundibular), outlet
(Subdivisions: NOS; conal septal malalignment). Type 2 VSD extending towards the outlet septum with malalignment of the conal septum.
VSD, type 4 (muscular), trabecular
Difficulties closing muscular VSDs are most common in the VSD, Type 4 (Muscular), Trabecular subgroup; and consequently, the trabecular septum and this subgroup have been analyzed in more detail, leading to the following subdivisions [10, 13]: NOS, anterior, apical, and midventricular.
VSD, type 4 (muscular), trabecular, anterior
A muscular trabecular VSD anterior to the septal or moderator band, in the high trabecular septum.
VSD, type 4 (muscular), trabecular, apical
A muscular trabecular VSD near the cardiac apex, in the low trabecular septum.
VSD, type 4 (muscular), trabecular, midventricular
A muscular trabecular VSD in the midseptum just inferior to the septal or moderator band, in the midtrabecular septum.
VSD hierarchy level 5
VSD hierarchy level 5 definitions
VSD, type 2 (perimembranous), (paramembranous), (conoventricular), outlet, conal septal malalignment
Subdivisions: NOS, TOF type, and IAA type.
VSD, type 2 (perimembranous) (paramembranous) (conoventricular), outlet, conal septal malalignment, TOF type
A type 2 VSD with anterior malalignment of the infundibular septum.
VSD, type 2 (perimembranous) (paramembranous) (conoventricular), outlet, conal septal malalignment, IAA type
A type 2 VSD with posterior malalignment of the infundibular septum.
Additional modifiers for VSD hierarchy level 1
Additional modifiers for VSD: hierarchy level 1 definitions
VSD, persistent (postoperative intentional)
Any VSD that was intentionally left open at a previous operation such as in the case of Tetralogy with pulmonary atresia and severely hypoplastic pulmonary arteries (PA) where RV to PA continuity was established, but the VSD was left alone.
VSD, previously created (iatrogenic)
Any VSD that was surgically created at a previous operation or intervention.
VSD, postinfarct
Any VSD caused by a myocardial infarction.
VSD, residual
Any VSD that had attempted closure or was missed at the time of prior operation or intervention.
VSD, restrictive
Any VSD that is small enough to restrict flow across it such that a pressure gradient exists between the two sides of the VSD.
Additional modifiers for VSD: hierarchy level 2
Additional modifiers for VSD: hierarchy level 3
1.5:1
1.5:1
III. Nomenclature for VSD treatment options
VSD treatment hierarchy level 1
VSD treatment hierarchy level 1 definitions
VSD creation
Surgical creation of a VSD
VSD treatment hierarchy level 2
VSD treatment hierarchy level 3
Additional comments regarding therapeutics
In addition to the above basic treatment options for VSD, 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 VSD (PA banding and debanding). Second, a separate part of the database must allow for coding for incisions for this and all other diagnoses (median sternotomy, submammary incision, right thoractomy, left thoracotomy, minimally invasive incisions, including 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 (aortomy, 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 VSD include the following data fields.
1.5:1 Lesion specific risk factors include: VSD, Multiple.
VI. Database studies and outcome analysis
VSD: inclusion criteria and allowable concomitant diagnoses
A case is included for VSD analysis if the primary diagnosis is VSD and concomitant cardiac diagnoses are none, left superior vena cava, patent ductus arteriosus, atrial septal defect, or any combination of these.
If no VSD subtype is given, the VSD will be classified as VSD, NOS.
Outcome tables
VSD surgery type (by year)
This table will show the number and percentage of each major VSD type (according to VSD hierarchy level 2) for each year. (If no VSD subtype is given, the VSD will be classified as VSD, 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).
VSD method of diagnosis for each VSD type (by year)
This table will show the method of diagnosis for each major VSD type for each year.
VSD age [years] at operation for each VSD type (by year)
This table will show the distribution of age at operation for each major VSD type for each year.
VSD gender distribution for each VSD type (by year)
This table will show the gender for each major VSD type for each year.
VSD—number and percentage with prior PA band for each VSD type (by year)
This table will show the number and percentage of each major VSD type treated with prior PA band for each year.
VSD features of repair—cardiopulmonary bypass (by year)
This table will show the number and percentage of each major VSD type treated with cardiopulmonary bypass for each year.
VSD features of repair—aortic cross-clamp (by year)
This table will show the number and percentage of each major VSD type treated with aortic cross-clamping for each year.
VSD features of repair—induced fibrillation (by year)
This table will show the number and percentage of each major VSD type treated with induced fibrillation for each year.
VSD 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 VSD type treated with circulatory arrest for each year.
VSD 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 VSD type treated with circulatory arrest for each year.
VSD features of repair—percent of patients greater than or equal to 6 months having deep hypothermia and circulatory arrest (by year)
For patients greater than or equal to 6 months, this table will show the number and percentage of each major VSD type treated with circulatory arrest for each year.
VSD 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 VSD type treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other.
VSD 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, including partial sternotomy, parasternal incision, mini-thoracotomy, etc) for each major VSD type for each year.
VSD features of repair—cardiac incision type (by year)
This table will show the number and percentage of each major cardiac incision type (aortotomy, pulmonary arteriotomy, right atriotomy, right ventriculotomy, left ventriculotomy, etc) for each major VSD type for each year.
VSD 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 VSD type for each year.
VSD 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, including cardiac, pulmonary, renal, infectious, and neurologic) for each major VSD type for each year.
VSD complication incidence (including operative death) patients less than 6 months of age (by year)
For patients less than 6 months, this table will show the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system, including cardiac, pulmonary, renal, infectious, and neurologic) for each major VSD type for each year.
VSD complication incidence (including operative death) patients greater than 6 months of age (by year)
For patients greater than 6 months, this table will show the number and percentage of operative deaths and complications (both transient and permanent, for each major organ system, including cardiac, pulmonary, renal, infectious, and neurologic) for each major VSD type for each year.
VSD preoperative length of ventilation (hours) (by year)
This table will show the preoperative length of ventilation for each major VSD type for each year.
VSD postoperative length of ventilation (hours) (by year)
This table will show the postoperative length of ventilation for each major VSD type for each year.
VSD total length of ventilation (hours) (by year)
This table will show the total length of ventilation for each major VSD type for each year.
VSD preoperative length of stay (days) (by year)
This table will show the preoperative length of stay for each major VSD type for each year.
VSD same day surgery (by year)
This table will show the number and percentage of day of surgery admissions for each major VSD type for each year.
VSD postoperative length of stay (days) (by year)
This table will show the postoperative length of stay for each major VSD type for each year.
VSD total length of stay (days) (by year)
This table will show the total length of stay for each major VSD type for each year.
VSD preoperative length of stay (days) by patient age (by year)
This table will show the preoperative length of stay for each major VSD type for each year, comparing patients less than 6 months to those greater than or equal to 6 months.
VSD same day surgery by patient age (by year)
This table will show the number and percentage of day of surgery admissions for each major VSD type for each year, comparing patients less than 6 months to those greater than or equal to 6 months.
VSD postoperative length of stay (days) by patient age (by year)
This table will show the postoperative length of stay for each major VSD type for each year, comparing patients less than 6 months to those greater than or equal to 6 months.
VSD total length of stay (days) by patient age (by year)
This table will show the total length of stay for each major VSD type for each year, comparing patients less than 6 months to those greater than or equal to 6 months.
Kaplan-Meier curves
Kaplan-Meier survival curves should be generated for each major VSD type for each year, comparing the total VSD cohort to patients less than 6 months to those greater than or equal to 6 months.
VSD 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, including cardiac, pulmonary, renal, infectious, and neurologic), in patients treated with and without preoperative ventilation, for each major VSD type for each year.
VSD 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, including cardiac, pulmonary, renal, infectious, and neurologic), in patients treated with and without circulatory arrest, for each major VSD type for each year.
VSD 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, and neurologic), in patients treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other, for each major VSD type for each year.
VSD 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, including cardiac, pulmonary, renal, infectious, and neurologic), in patients treated with each major incision type (median sternotomy, submammary incision, right thoracotomy, left thoracotomy, minimally invasive incisions, including partial sternotomy, parasternal incision, minithoracotomy, etc), for each major VSD type for each year.
VSD complication incidence (including operative death) versus cardiac 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, including cardiac, pulmonary, renal, infectious, and neurologic), in patients treated with each major cardiac incision type (aortotomy, pulmonary arteriotomy, right atriotomy, right ventriculotomy, left ventriculotomy, etc), for each major VSD type for each year.
VSD 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, including cardiac, pulmonary, renal, infectious, and neurologic), in patients treated with primary (suture), patch, and device closure, for each major VSD type for each year.
VSD 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 VSD type for each year.
VSD postoperative length of ventilation (hours) versus deep hypothermia and circulatory arrest (by year)
This table will comare the postoperative length of ventilation, in patients treated with and without circulatory arrest, for each major VSD type for each year.
VSD 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 VSD type for each year.
VSD 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 incision, including partial sternotomy, parasternal incision, minithoracotomy, etc), for each major VSD type for each year.
VSD postoperative length of ventilation (hours) versus cardiac incision type (by year)
This table will compare the postoperative length of ventilation, in patients treated with each major cardiac incision type (aortotomy, pulmonary arteriotomy, right atriotomy, right ventriculotomy, left ventriculotomy, etc), for each major VSD type for each year.
VSD 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 VSD type for each year.
VSD 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 VSD type for each year.
VSD 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 VSD type for each year.
VSD 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 VSD type for each year.
VSD 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 incision, including partial sternotomy, parasternal incision, minithoracotomy, etc), for each major VSD type for each year.
VSD postoperative length of stay (days) versus cardiac incision type (by year)
This table will compare the postoperative length of stay, in patients treated with each major cardiac incision type (aortotomy, pulmonary arteriotomy, right atriotomy, right ventriculotomy, left ventriculotomy, etc), for each major VSD type for each year.
VSD 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 VSD type for each year.
Acknowledgments
Figures 1 through 7 were prepared by Jeffrey A. White, MS.
References
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