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Ann Thorac Surg 2000;69:S44-S49
© 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 Pediatric Cardiothoracic Surgery, Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
c Division of Cardiovascular Surgery, Miami Children’s Hospital, Miami, Florida, USA
d 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 aortopulmonary window (AP window) and pulmonary artery origin from ascending aorta (hemitruncus) 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
Aortopulmonary window (AP window) is the rarest of the four types of septal defects. Individual institutions rarely see more than one or two neonates with this problem in a given year [1]. Overall, the lesion accounts for approximately 0.1% of all congenital cardiac anomalies [2].
An AP window is a communication between the main pulmonary artery and the ascending aorta in the presence of two separate semilunar valves. The presence of two separate semilunar valves separates AP window from truncus arteriosus [3].
Mori and associates [4] classified AP window into three types. Proximal defects are the most common and are located just above the sinus of Valsalva, a few millimeters above the semilunar valves. Distal defects are located in the uppermost portion of the ascending aorta. Total defectsinvolve the majority of the ascending aorta.
Ho and associates [5] modified this classification in a way that is more useful to interventional cardiologists considering transcatheter device closure of AP windows. They keep the basic terminology from Mori and associates as defined above but add additional description. Proximal defects are noted to have little inferior rim separating the AP window from the semilunar valves. Distal defects are noted to have a well-formed inferior rim but little superior rim. Total defects are called confluent defects with little superior and inferior rims. Finally, intermediate defects are defects with adequate superior and inferior rims; this type is obviously the group most suited for device closure.
An alternative classification has been proposed by Richardson and associates [6] that includes both the AP window lesions described above and anomalous right pulmonary artery origin from ascending aorta. The Richardson system describes three types of anomalies of aortopulmonary septation: type I) typical aortopulmonary septal defect or window; type II) distal aortopulmonary septal defects in which the ascending aorta communicates with the origin of the right pulmonary artery; and type III) anomalous right pulmonary artery origin from ascending aorta, or hemitruncus arteriosus.
The term "hemitruncus arteriosus" merits special consideration. This term is described in the truncus arteriosus manuscript of this supplement as follows.
Hemitruncus has often been used to describe a situation in which one pulmonary artery arises from the ascending aorta and the other pulmonary artery arises from the right ventricle. This defect is usually not associated with a ventricular septal defect. Hemitruncus is not really a form of truncus arteriosus, and is best defined as ascending aortic origin of one pulmonary artery and right ventricular origin of the other pulmonary artery.
We agree with the above discussion; hemitruncus is actually not a type of truncus arteriosus. We feel that hemitruncus is actually an anomalous pulmonary artery originating from ascending aorta. We also feel that the Richardson classification is flawed because anomalous right pulmonary artery originating from ascending aorta (or hemitruncus arteriosus) is not actually an anomaly of aortopulmonary septation, as is aortopulmonary window [7]. Furthermore, and more importantly for our purposes, Richardson type III lesions are corrected with different surgical techniques than are those of Richardson type I and II [8]. Therefore, we would classify Richardson type I and II lesions as "AP window" (Richardson type II lesions would be likely classified in the "AP window, Type 2 Distal defect" in our hierarchical nomenclature system presented below). Meanwhile, we would classify Richardson type III lesions as "Pulmonary artery origin from ascending aorta (Hemitruncus), Right pulmonary artery" in our hierarchical nomenclature system presented below.
Lesions classified as "Pulmonary artery origin from ascending aorta (Hemitruncus)" should not include origin of the right pulmonary artery or left pulmonary artery from the innominate artery or the aortic arch via a patent ductus arteriosus (PDA) or collateral artery [7]. Cases where a pulmonary artery arises from the innominate artery (as a trifurcation with normal common carotid and subclavian arteries), or from the undersurface of the mid-aortic arch, often have ductal tissue forming the proximal part of the pulmonary artery origin [9]. These cases are therefore not part of the group of cases classified as "Pulmonary artery origin from ascending aorta (Hemitruncus)." Moreover, the term "hemitruncus" should be discouraged; hemitruncus is an inaccurate term because it implies that the anomalous vessel arises from a common persistent arterial trunk, although it actually arises from the aorta [10]. Although we include this term in our hierarchy below, we discourage its use. If used, hemitruncus should be synonymous with "pulmonary artery origin from ascending aorta, left pulmonary artery" or "pulmonary artery origin from ascending aorta, right pulmonary artery," and not as a subtype of truncus arteriosus or AP window.
II. Analysis: a unified aortopulmonary window nomenclature system
AP window hierarchy level 1
AP window hierarchy level 1 definitions
AP window (aortopulmonary window)
An AP window is a communication between the main pulmonary artery and the ascending aorta in the presence of two separate semilunar valves. The presence of two separate semilunar valves distinguishes AP window from truncus arteriosus.
Pulmonary artery origin from ascending aorta (hemitruncus)
A situation in which one pulmonary artery arises from the ascending aorta and the other pulmonary artery arises from the right ventricle. This term does not include origin of the right pulmonary artery or left pulmonary artery from the innominate artery or the aortic arch via a PDA or collateral artery [7].
AP window hierarchy level 2
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AP window hierarchy level 2 definitions
AP window, NOS
An AP window not further described (not otherwise specified [NOS]).
AP window and interrupted aortic arch
An AP window in association with an interrupted aortic arch [11, 12]. The AP window and interrupted aortic arch may then be coded separately to further specify the individual AP window and interrupted aortic arch types.
AP window, type 1 proximal defect
An AP window located just above the sinus of Valsalva, a few millimeters above the semilunar valves, with a superior rim but little inferior rim separating the AP window from the semilunar valves.
AP window, type 2 distal defect
An AP window located in the uppermost portion of the ascending aorta, with a well-formed inferior rim but little superior rim.
AP window, type 3 total defect
An AP window involving the majority of the ascending aorta, with little superior and inferior rims.
AP window, intermediate type
An AP window similar to the total defect but with adequate superior and inferior rims.
III. Nomenclature for aortopulmonary window treatment options
Since the first report of successful surgical repair by Gross [13], surgical approaches to AP window have continued to evolve [14–16]. Recently, transcatheter closure of an AP window has been successfully accomplished several times [17–19]. The nomenclature system below will allow for coding of all of these treatment options. Over time, this system can be modified to provide more detail if necessary.
AP window treatment hierarchy level 1
AP window treatment hierarchy level 2
AP window treatment hierarchy level 3
Additional comments regarding therapeutics
In addition to the above basic treatment options for AP window, 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, glutaraldehyde-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 AP window include the following data fields.
Specific postoperative complications of AP window to be studied include:
1.5:1 VI. Database studies and outcome analysis
AP window: inclusion criteria and allowable concomitant diagnoses
A case is included for AP window analysis if the primary diagnosis is AP window and concomitant cardiac diagnoses are none, left superior vena cava, PDA, atrial septal defect (ASD), ventricular septal defect (VSD) [20], interrupted aortic arch [11, 12], or any combination of these.
If no AP window subtype is given, the AP window will be classified as AP window, NOS.
Outcome tables
AP window surgery type (by year)
This table will show the number and percentage of each major AP window type (according to AP window hierarchy level 2) for each year. (If no AP window subtype is given, the AP window will be classified as AP window, 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.)
AP window method of diagnosis for each AP window type (by year)
This table will show the method of diagnosis for each major AP window type for each year.
AP window age (years) at operation for each AP window type (by year)
This table will show the distribution of age at operation for each major AP window type for each year.
AP window gender distribution for each AP window type (by year)
This table will show the gender for each major AP window type for each year.
AP window features of repair: cardiopulmonary bypass (by year)
This table will show the number and percentage of each major AP window type treated with cardiopulmonary bypass for each year.
AP window features of repair: aortic cross-clamp (by year)
This table will show the number and percentage of each major AP window type treated with aortic cross-clamping for each year.
AP window features of repair: induced fibrillation (by year)
This table will show the number and percentage of each major AP window type treated with induced fibrillation for each year.
AP window 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 AP window type treated with circulatory arrest for each year.
AP window 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 AP window type treated with circulatory arrest for each year.
AP window 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 AP window type treated with circulatory arrest for each year.
AP window 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 AP window type treated with various cardioplegia types including blood, crystalloid, substrate enriched, and other.
AP window 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 AP window type for each year.
AP window 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, etc) for each major AP window type for each year.
AP window 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 AP window type for each year.
AP window 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 AP window type for each year.
AP window complication incidence (including operative death) 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 AP window type for each year.
AP window complication incidence (including operative death) 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 AP window type for each year.
AP window preoperative length of ventilation (hours) (by year)
This table will show the preoperative length of ventilation for each major AP window type for each year.
AP window postoperative length of ventilation (hours) (by year)
This table will show the postoperative length of ventilation for each major AP window type for each year.
AP window total length of ventilation (hours) (by year)
This table will show the total length of ventilation for each major AP window type for each year.
AP window preoperative length of stay (days) (by year)
This table will show the preoperative length of stay for each major AP window type for each year.
AP window same day surgery (by year)
This table will show the number and percentage of day of surgery admissions for each major AP window type for each year.
AP window postoperative length of stay (days) (by year)
This table will show the postoperative length of stay for each major AP window type for each year.
AP window total length of stay (days) (by year)
This table will show the total length of stay for each major AP window type for each year.
AP window preoperative length of stay (days) by patient age (by year)
This table will show the preoperative length of stay for each major AP window type for each year, comparing patients <6 months to those 6 months.
AP window same-day surgery by patient age (by year)
This table will show the number and percentage of day of surgery admissions for each major AP window type for each year, comparing patients < 6 months with those 6 months.
AP window postoperative length of stay (days) by patient age (by year)
This table will show the postoperative length of stay for each major AP window type for each year, comparing patients < 6 months with those 6 months.
AP window total length of stay (days) by patient age (by year)
This table will show the total length of stay for each major AP window 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 AP window type for each year, comparing the total AP window cohort with patients < 6 months with those 6 months.
AP window 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 AP window type for each year.
AP window 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 AP window type for each year.
AP window 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 AP window type for each year.
AP window 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 AP window type for each year.
AP window 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 [cardiac, pulmonary, renal, infectious, neurologic]), in patients treated with each major cardiac incision type (aortotomy, pulmonary arteriotomy, etc), for each major AP window type for each year.
AP window 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 one-patch and two-patch closure, for each major AP window type for each year.
AP window 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 AP window type for each year.
AP window 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 AP window type for each year.
AP window 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 AP window type for each year.
AP window 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 AP window type for each year.
AP window 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, etc), for each major AP window type for each year.
AP window postoperative length of ventilation (hours) versus closure technique (by year)
This table will compare the postoperative length of ventilation, in patients treated with one-patch and two-patch closure, for each major AP window type for each year.
AP window 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 AP window type for each year.
AP window 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 AP window type for each year.
AP window 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 AP window type for each year.
AP window 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 AP window type for each year.
AP window 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, etc), for each major AP window type for each year.
AP window postoperative length of stay (days) versus closure technique (by year)
This table will compare the postoperative length of stay, in patients treated with one-patch and two-patch closure, for each major AP window type for each year.
References
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 M. Nathan, D. Rimmer, G. Piercey, P. J. del Nido, J. E. Mayer, E. A. Bacha, and F. A. Pigula Early repair of hemitruncus: Excellent early and late outcomes J. Thorac. Cardiovasc. Surg., May 1, 2007; 133(5): 1329 - 1335. [Abstract] [Full Text] [PDF]
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 C. L. Backer and C. Mavroudis Surgical management of aortopulmonary window: a 40-year experience Eur J Cardiothorac Surg, May 1, 2002; 21(5): 773 - 779. [Abstract] [Full Text] [PDF]
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