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Ann Thorac Surg 2000;69:S298-S307
© 2000 The Society of Thoracic Surgeons

Congenital Heart Surgery Nomenclature and Database Project: patent ductus arteriosus, coarctation of the aorta, interrupted aortic arch

^a Department of Surgery, Northwestern University Medical School, Division of Cardiovascular-Thoracic Surgery, Children’s Memorial Hospital, Chicago, Illinois, USA

Address reprint requests to Dr Backer, Division of Cardiovascular-Thoracic Surgery, Children’s Memorial Hospital, 2300 Children’s Plaza, m/c 22, Chicago, IL 60614
e-mail: c-backer{at}nwu.edu

Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.

Abstract

The extant nomenclature for patent ductus arteriosus (PDA), coarctation of the aorta (CoAo), and interrupted aortic arch (IAA) 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. PDA is subclassified by origin, insertion, and patient weight. CoAo is subclassified into isolated CoAo, CoAo with ventricular septal defect, and CoAo with complex intracardiac anomalies. IAA is subclassified into anatomic types A, B, and C based on the location of the interruption. 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 analyses. 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

Apatent ductus arteriosus (PDA) is defined as a vascular arterial connection between the thoracic aorta and pulmonary artery. Depending on the patient’s age, the size of the ductus, and concomitant cardiac diagnoses, a PDA can be asymptomatic, can cause severe congestive heart failure, or can be the child’s only source of pulmonary or systemic blood flow. Most infants with a PDA in fact have spontaneous closure in the first few hours to days of life and the PDA becomes a ligamentum arteriosum.

The word coarctation comes from the Latin, coarctere, to contract. Coarctation of the aorta is defined as a congenital narrowing of the descending thoracic aorta usually (but not always) just distal to the left subclavian artery adjacent to the site of insertion of the ductus arteriosus (ligamentum arteriosum). Almost all patients with a coarctation will require intervention (surgical or catheter) to alleviate the pressure gradient across the coarctation. A significant number of patients with a coarctation will also have either a ventricular septal defect (VSD) or other complex intracardiac anomalies.

Interrupted aortic arch (IAA) is defined as a loss of luminal continuity between the ascending and descending thoracic aorta. Some of these patients may have a cord of tissue connecting the ascending and descending thoracic aorta, but if loss of luminal continuity occurs this should be considered an IAA. Associated anomalies are very common in patients with IAA and they include ventricular septal defect, bicuspid aortic valve, subaortic stenosis, and the DiGeorge syndrome (hypocalcemia, T-cell defects). Other frequently described associated cardiac anomalies include aortopulmonary window, transposition of the great arteries, single ventricle, and truncus arteriosus.

II. Patent ductus arteriosus

A PDA is defined as a vascular arterial connection between the thoracic aorta and the pulmonary artery. Most commonly a PDA has its origin from the descending thoracic aorta, just distal and opposite the origin of the left subclavian artery. The insertion of the ductus is most commonly into the very proximal left pulmonary artery (LPA) at its junction with the main pulmonary artery (MPA). However, this can be highly variable, and the ductus can originate from the ascending aorta, from any of the brachiocephalic branches, or from the distal descending thoracic aorta. Similarly, the PDA can insert onto the right pulmonary artery (RPA), LPA, or MPA. In some cases, the PDA can be the only source of blood flow for the RPA, LPA, or MPA.

The ligation of a PDA by Robert Gross in 1938 is considered by many to be the first successful operation for congenital heart disease [1]. The indications for closing a PDA are several and depend on the patient’s age and the size of the ductus. In premature infants with poorly developed pulmonary artery smooth muscle, a PDA can cause severe congestive heart failure (CHF) and chronic lung disease. In older children and adults, even an asymptomatic PDA places the patient at risk for subacute bacterial endocarditis. Other reported complications from a PDA include aneurysm formation, aortic dissection, pulmonary hypertension, and CHF. The diagnosis of a PDA is usually suspected by auscultation (machinery murmur) and is confirmed by color doppler echocardiogram, or less frequently by cardiac catheterization.

Described therapeutic interventions for PDA include the administration of indocin [2], surgical ligation (single, double, triple) [1], surgical division and oversewing [3], surgical placement of a clip [4], coiling or placement of an occluder device with transcatheter techniques in the cardiac catheterization lab [5–7], and clipping with video-assisted thoracoscopic surgery (VATS) [8, 9]. The potential intraoperative risks of a procedure for PDA include injury to the recurrent laryngeal nerve, the thoracic duct, phrenic nerve, hemorrhage, and ligation of the wrong vascular structure; examples include the LPA and the descending thoracic aorta. The diagnostic and therapeutic hierarchies described below are based on our institutional experience with over 1,000 ductus operations [3].

IIA. PDA diagnostic hierarchy levels
PDA hierarchy level 1

PDA

PDA hierarchy level 2

PDA, NOS

PDA, Normal origin and insertion

PDA, Abnormal origin and insertion

PDA hierarchy level 3

PDA, NOS

PDA, Normal origin and insertion, NOS

PDA, Normal origin and insertion, Patients less than or equal to 2,500 g

PDA, Normal origin and insertion, Patients greater than 2,500 g

PDA, Abnormal origin and insertion, NOS

PDA, Abnormal origin and insertion, Patients less than or equal to 2,500 g

PDA, Abnormal origin and insertion, Patients greater than 2,500 g

PDA diagnostic modifiers
Abnormal PDA origin and insertion choices for the first or only PDA:

PDA abnormal origins

Ascending aorta

Right innominate artery

Left innominate artery

Right carotid artery

Left carotid artery

Right subclavian artery

Left subclavian artery

Descending aorta

PDA abnormal insertions

RPA

LPA

MPA

Abnormal PDA origin and insertion choices for the second PDA:

PDA abnormal origins

Ascending aorta

Right innominate artery

Left innominate artery

Right carotid artery

Left carotid artery

Right subclavian artery

Left subclavian artery

Descending aorta

PDA abnormal insertions

RPA

LPA

MPA

If there is only one PDA with an abnormal origin and/or insertion, the first PDA diagnostic modifier is completed. In the rare cases of two would be PDAs, the clinician must distinguish between a major aortopulmonary collateral artery (MAPCA), anomalous origin of the right pulmonary artery from the ascending aorta, and a second PDA. By convention, the left sided PDA is coded as the first PDA, and the right sided PDA is coded as the second PDA.

Additional diagnostic modifiers for PDA

PDA, Persistent postoperative (S/P ligation only)

PDA, Persistent postoperative (S/P ligation and clip)

PDA, Persistent postoperative (S/P ligation and division)

PDA, Persistent postoperative (S/P clip by thoracotomy)

PDA, Persistent postoperative (S/P clip by sternotomy)

PDA, Persistent postoperative (S/P clip by thoracoscopy)

PDA, Persistent postcoil

PDA, Persistent postdevice

PDA hierarchy level 1 definitions
Patent ductus arteriosus (PDA)
A PDA is a vascular connection between the aorta and pulmonary artery.

PDA hierarchy level 2 definitions

A normal origin and normal insertion of a PDA is defined as a vascular connection between the descending thoracic aorta (opposite and distal to the left subclavian artery) and the LPA at the junction with the MPA. An abnormal origin or insertion of a patent ductus arteriosus is present when there is a deviation from the normal origin or insertion.

PDA hierarchy level 3 definitions

A premature infant is defined as a baby born before 37 weeks of gestation. Sometimes this definition can be difficult. As a result more authors have defined a premature infant as a newborn whose weight is less than or equal to 2,500 g (low birth weight is also defined as 2,500 grams). The indication for PDA closure in such a patient is mostly to prevent chronic lung disease and occasionally for congestive heart failure (CHF).

PDA additional diagnostic modifier definitions

IIB. PDA therapeutic hierarchy levels
Hierarchy level 1

PDA closure, Surgical

PDA closure, Device

PDA closure, NOS

Hierarchy level 2

PDA closure, Surgical, NOS

PDA closure, Surgical therapy, Ligation

PDA closure, Surgical therapy, Ligation and clip

PDA closure, Surgical therapy, Ligation and division

PDA closure, Surgical therapy, Clip by thoracotomy

PDA closure, Surgical therapy, Clip by sternotomy

PDA closure, Surgical therapy, Clip by thoracoscopy (VATS)

PDA closure, Device, NOS

PDA closure, Device, Transcatheter techniques (See article on Interventional Cardiology)

PDA closure, NOS

Hierarchy level 3

PDA closure, Surgical, NOS

PDA closure, Surgical therapy, Ligation, NOS

PDA closure, Surgical therapy, Ligation, Single suture technique

PDA closure, Surgical therapy, Ligation, Double suture technique

PDA closure, Surgical therapy, Ligation, Triple suture technique

PDA closure, Surgical therapy, Ligation and clip

PDA closure, Surgical therapy, Ligation and division

PDA closure, Surgical therapy, Clip by thoracotomy, NOS

PDA closure, Surgical therapy, Clip by thoracotomy, Single clip

PDA closure, Surgical therapy, Clip by thoracotomy, Double clip

PDA closure, Surgical therapy, Clip by sternotomy, NOS

PDA closure, Surgical therapy, Clip by sternotomy, Single clip

PDA closure, Surgical therapy, Clip by sternotomy, Double clip

PDA closure, Surgical therapy, Clip by thoracoscopy (VATS), NOS

PDA closure, Surgical therapy, Clip by thoracoscopy (VATS), Single clip

PDA closure, Surgical therapy, Clip by thoracoscopy (VATS), Double clip

PDA closure, Device, NOS

PDA closure, Device, Transcatheter techniques (see article on interventional cardiology)

PDA closure, NOS

IIC. PDA diagnosis and procedure short lists

Diagnosis Short List

PDA

Procedure Short List

PDA closure, Surgical

PDA closure, Device

PDA closure, NOS

IID. PDA potential diagnostic related risk factors

Recurrent laryngeal nerve injury—Diagnosis by laryngoscopy

Lymphatic duct injury—Chyle draining from chest tube

Phrenic nerve injury—Diagnosis by echocardiography or fluoroscopy

Ligation of the wrong vascular structure such as

LPA

Descending aorta

Hemorrhage requiring transfusion

Residual PDA after repair

III. Coarctation of the aorta

CoAo is defined as a hemodynamically significant narrowing of the descending thoracic aorta, usually just distal to the left subclavian artery where the ligamentum arteriosum originates. In 1903, Bonnet classified coarctation into two groups: infantile which later became known as preductal, and adult which later became known as postductal [10]. Van Praagh pointed out that the site of coarctation is almost always juxtaductal so the preductal and postductal classification scheme is really inaccurate [11]. The infantile and adult classifications reflect the different times of presentation of the patients and the typical appearance in an infant versus an adult. There are significant pathophysiologic differences between an adult with a chronic coarctation and an infant with a ductal dependent circulation.

A commonly used surgical classification is to group coarctation into three categories [12–14]: I. isolated coarctation; II. coarctation and VSD; and III. coarctation and complex intracardiac anomaly.

Amato proposed a slightly different classification system of coarctation [15]: I. primary coarctation; II. coarctation with isthmus hypoplasia (portion of the aorta between the left subclavian artery and the patent ductus or ligamentum arteriosum); and III. coarctation with tubular hypoplasia involving the isthmus and segment between the left carotid and left subclavian arteries.

Each of these groups had subtypes: A, with VSD; and B, with other major cardiac defects.

The first successful surgical repair of coarctation was by Craaford in 1945 using a technique of coarctation resection with simple end-to-end anastomosis of the aorta [16]. This operation had a high incidence of recoarctation in many series leading to surgical modifications and new techniques for coarctation repair [17]. These techniques include the patch aortoplasty [13, 18], subclavian flap aortoplasty [19], and resection with extended end-to-end anastomosis [12, 14, 20]. There are many other techniques reported for surgical repair of aortic coarctation including subclavian flap aortoplasty with preservation of arterial blood flow to the left arm [21], subclavian free flap [22], subclavian flap with resection of ductal tissue [23], radically extended end-to-end anastomosis [24], end-to-side anastomosis of the descending aorta to the proximal aortic arch [25], and pulmonary homograft patch aortoplasty [26].

IIIA. Coarctation diagnostic hierarchy levels
Coarctation hierarchy level 1

Coarctation of the Aorta (CoAo)

Coarctation hierarchy level 1 definitions
Coarctation of the aorta (CoAo)
A hemodynamically significant narrowing of the descending thoracic aorta usually distal to the left subclavian artery. However, it may occur anywhere in the thoracic or abdominal aorta.

Coarctation hierarchy level 2

CoAo, NOS

Coarctation of the Aorta (CoAo), Isolated

Coarctation of the Aorta (CoAo), With VSD

Coarctation of the Aorta (CoAo), With complex intracardiac anomaly

CoAo hierarchy level 3

CoAo, NOS

CoAo, Isolated, NOS

Coarctation of the Aorta (CoAo), Isolated, Isthmus hypoplasia

Coarctation of the Aorta (CoAo), Isolated, Arch hypoplasia

Coarctation of the Aorta (CoAo), Isolated, Isthmus hypoplasia + arch hypoplasia

Coarctation of the Aorta (CoAo), With VSD, NOS

Coarctation of the Aorta (CoAo), With VSD, Isthmus hypoplasia

Coarctation of the Aorta (CoAo), With VSD, Arch hypoplasia

Coarctation of the Aorta (CoAo), With VSD, Isthmus hypoplasia + arch hypoplasia

Coarctation of the Aorta (CoAo), With complex intracardiac anomaly, NOS

Coarctation of the Aorta (CoAo), With complex intracardiac anomaly, Isthmus hypoplasia

Coarctation of the Aorta (CoAo), With complex intracardiac anomaly, Arch hypoplasia

Coarctation of the Aorta (CoAo), With complex intracardiac anomaly, Isthmus hypoplasia + arch hypoplasia

Definitions: arch hypoplasia [27, 28]

Aortic arch
Can be divided into 3 parts, the proximal transverse arch (arch between the innominate and left carotid arteries), the distal transverse arch (arch between the left carotid and left subclavian arteries), and the aortic isthmus (arch between the left subclavian and insertion of the patent ductus arteriosus).

Isthmus hypoplasia
The isthmus is less than 40% of the diameter of the ascending aorta.

Arch hypoplasia
Proximal or distal transverse arch are less than 60% or 50% respectively of the diameter of the ascending aorta.

Coarctation: additional modifiers

Coarctation of the Aorta (CoAo), Low thoracic aorta

Coarctation of the Aorta (CoAo), Abdominal aorta, Suprarenal

Coarctation of the Aorta (CoAo), Abdominal aorta, Infrarenal

Coarctation of the Aorta (CoAo), Recurrent, NOS

Coarctation of the Aorta (CoAo), Recurrent, S/P surgical repair

Coarctation of the Aorta (CoAo), Recurrent S/P transcatheter repair

Coarctation of the Aorta (CoAo), Residual, NOS

Coarctation of the Aorta (CoAo), Residual, S/P surgical repair

Coarctation of the Aorta (CoAo), Residual S/P transcatheter repair

IIIB. Nomenclature for CoAo treatment options

CoAo treatment hierarchy level 1

Coarctation repair, NOS

Coarctation repair, Left thoracotomy

Coarctation repair, Right thoracotomy

Coarctation repair, Median sternotomy

Coarctation repair, Transcatheter technique

Coarctation repair, Other

CoAo treatment hierarchy level 2

Coarctation repair, NOS

Coarctation repair, Left thoracotomy, NOS

Coarctation repair, Left thoracotomy, Resection with end-to-end anastomosis

Coarctation repair, Left thoracotomy, Prosthetic patch aortoplasty

Coarctation repair, Left thoracotomy, Prosthetic interposition graft

Coarctation repair, Left thoracotomy, Subclavian flap aortoplasty

Coarctation repair, Left thoracotomy, Subclavian flap aortoplasty—reverse flap

Coarctation repair, Left thoracotomy, Resection with extended end-to-end anastomosis

Coarctation repair, Left thoracotomy, Extraanatomic bypass graft

Coarctation repair, Left thoracotomy, Pulmonary artery banding

Coarctation repair, Left thoracotomy, Other

Coarctation repair, Right thoracotomy, NOS

Coarctation repair, Right thoracotomy, Resection with end-to-end anastomosis

Coarctation repair, Right thoracotomy, Prosthetic patch aortoplasty

Coarctation repair, Right thoracotomy, Prosthetic interposition graft

Coarctation repair, Right thoracotomy, Subclavian flap aortoplasty

Coarctation repair, Right thoracotomy, Subclavian flap aortoplasty—reverse flap

Coarctation repair, Right thoracotomy, Resection with extended end-to-end anastomosis

Coarctation repair, Right thoracotomy, Extraanatomic bypass graft

Coarctation repair, Right thoracotomy, Other

Coarctation repair, Median sternotomy, NOS

Coarctation repair, Median sternotomy, Resection with end-to-end anastomosis

Coarctation repair, Median sternotomy, Prosthetic patch aortoplasty

Coarctation repair, Median sternotomy, Prosthetic interposition graft

Coarctation repair, Median sternotomy, Subclavian flap aortoplasty

Coarctation repair, Median sternotomy, Subclavian flap aortoplasty—reverse flap

Coarctation repair, Median sternotomy, Resection with extended end-to-end anastomosis

Coarctation repair, Median sternotomy, Extraanatomic bypass graft

Coarctation repair, Median sternotomy, Pulmonary artery banding

Coarctation repair, Median sternotomy, Other

Coarctation repair, Transcatheter technique, NOS

Coarctation repair, Transcatheter technique, Balloon dilation

Coarctation repair, Transcatheter technique, Balloon dilation with stent placement

Coarctation repair, Transcatheter technique, Other

Coarctation repair, Other

Coarctation of the aorta modifiers

Partial Cardiopulmonary Bypass (CPB)

Full CPB

Intraoperative Shunt

Aortic cross-clamp time

Number of collaterals ligated

Patch

Polytetrafluoroethylene (PTFE)—0.4 mm thickness

PTFE—0.5 mm thickness

PTFE—0.6 mm thickness

Dacron

Hemashield

Homograft

Gluteraldehyde treated pericardium

IIIC. Coarctation of the aorta diagnosis and procedure short lists

Diagnosis Short List

Coarctation of aorta

Aortic arch hypoplasia

Procedure Short List

Coarctation repair, End-to-end

Coarctation repair, End-to-end, Extended

Coarctation repair, Subclavian flap

Coarctation repair, Patch aortoplasty

Coarctation repair, Interposition graft

Coarctation repair, Other

Coarctation repair, NOS

Aortic arch repair

IIID. Coarctation of the aorta potential diagnostic related risk factors

Hemorrhage

Intraoperative, postoperative

Recurrent laryngeal nerve injury

Phrenic nerve injury

Horner’s syndrome

Chylothorax

Paradoxical postoperative hypertension

Mesenteric ischemia

Paraplegia

Stroke

Aneurysm formation

Recoarctation (> 20 mm Hg gradient by catheter ization or blood pressure measurement)

Left arm ischemia

Short left arm

IV. Interrupted aortic arch

IAA is defined as loss of luminal continuity between the ascending and descending aorta. In nearly all cases, the blood flow to the descending thoracic aorta is from a PDA, and there is an associated VSD. The nearly universally used classification system is that of Celoria and Patton [29].

This classification system is based on the site of the aortic arch interruption: A. interruption distal to the left subclavian artery; B. interruption between the left carotid and left subclavian arteries; and C. interruption between the innominate and left carotid arteries.

The most common is type B, next most common is type A, least common is type C. Associated anomalies are quite common.

Surgical approaches to IAA take one of two main approaches. The first approach reported was a staged approach beginning with an initial palliative operation through a left thoracotomy to address the interrupted aortic arch followed by later complete repair of the intracardiac anomaly from a median sternotomy. The other approach is complete primary repair in the neonate. A staged approach usually has as its initial operation (through a left thoracotomy) placement of a pulmonary artery band to control pulmonary blood flow and then repair of the interrupted aortic arch either with a graft (Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ), Dacron (C. R. Bard, Haverhill, PA), etc.) from the ascending to descending thoracic aorta [30, 31] or a direct anastomosis of a mobilized descending thoracic aorta to the ascending aorta [32]. This is followed in 2 to 6 months with an operation performed from the median sternotomy approach for removal of the pulmonary band, reconstruction of the pulmonary artery, and closure of the VSD [33]. Almost all of these patients will then require as a third stage either replacement of the conduit, additional conduits from the ascending to the descending thoracic aorta, or in some cases balloon dilation of a direct anastomosis. Complete repair in the neonatal period utilizes an approach through a median sternotomy with CPB and circulatory arrest [34, 35]. The VSD is closed and in most cases a direct anastomosis of a mobilized descending thoracic aorta to the ascending aorta is performed. These patients may require reoperation for subaortic stenosis, and often require balloon dilation of the anastomosis of the ascending and descending thoracic aorta [36].

IVA. IAA diagnostic hierarchy levels
IAA hierarchy level 1

Interrupted aortic arch (IAA)

IAA level 1
Complex arch anomaly where there is loss of luminal continuity between the ascending and descending thoracic aorta. In most cases blood flow to the descending thoracic aorta is through a PDA, and there is a large VSD.

IAA hierarchy level 2

IAA, NOS

IAA, Type A (Interruption distal to the left subclavian artery)

IAA, Type B (Interruption between the left carotid and left subclavian arteries)

IAA, Type C (Interruption between the innominate and left carotid arteries)

IAA modifiers

With PDA

Without PDA

With VSD

Without VSD

With bicuspid aortic valve

Without bicuspid aortic valve

With left ventricular outflow tract (LVOT) obstruction

Without LVOT obstruction

With DiGeorge syndrome

Without DiGeorge syndrome

With aortopulmonary window

Without aortopulmonary window

With transposition of the great arteries

Without transposition of the great arteries

With single ventricle

Without single ventricle

With truncus arteriosus

Without truncus arteriosus

IVB. Nomenclature for IAA treatment options

IAA treatment hierarchy level 1

IAA repair, NOS

IAA repair, Staged approach

IAA repair, Complete repair

IAA treatment hierarchy level 2

IAA repair, NOS

IAA repair, Staged approach, NOS

IAA repair, Staged approach, Stage I (Arch repair with or without PAB)

IAA repair, Staged approach, Stage II (VSD closure and PAB removal)

IAA repair, Staged approach, Stage III (Graft change or arch augmentation)

IAA repair, Complete repair, NOS

IAA repair, Complete repair, VSD closure with prosthetic graft for arch repair

IAA repair, Complete repair, VSD closure with direct arch anastomosis

IAA treatment hierarchy level 3

IAA repair, NOS

IAA repair, Staged approach, NOS

IAA repair, Staged approach, Stage I (Arch repair with or without PAB), NOS

IAA repair, Staged approach, Stage I (Arch repair with or without PAB), With prosthetic graft

IAA repair, Staged approach, Stage I (Arch repair with or without PAB), With direct anastomosis

IAA repair, Staged approach, Stage II (VSD closure and PAB removal), NOS

IAA repair, Staged approach, Stage II (VSD closure and PAB removal), With pulmonary artery reconstruction

IAA repair, Staged approach, Stage II (VSD closure and PAB removal), Without pulmonary artery reconstruction

IAA repair, Staged approach, Stage III (Graft change or arch augmentation), NOS

IAA repair, Staged approach, Stage III (Graft change or arch augmentation), Additional prosthetic graft

IAA repair, Staged approach, Stage III (Graft change or arch augmentation), Replacement of prosthetic graft

IAA repair, Complete repair, NOS

IAA repair, Complete repair, VSD closure with prosthetic graft for arch repair

IAA repair, Complete repair, VSD closure with direct arch anastomosis

IVC. Interrupted aortic arch diagnosis and procedure short lists

Diagnosis Short List

IAA

Procedure Short List

IAA repair

IVD. Interrupted aortic arch potential diagnostic related risk factors

Recurrent laryngeal nerve injury Phrenic nerve injury Chylothorax Paraplegia Cardiovascular accident Intraventricular hemorrhage Recurrent arch obstruction Subaortic stenosis DiGeorge complications Hypocalcemia Sepsis Residual VSD Delayed sternal closure Right bronchial compression Left bronchial compression

V. Outcome reports

A. PDA

Incidence of PDA by year, by weight and age at presentation and method of diagnosis.

Operative approach and type of PDA repair by year: surgical closure (specify ligation, clip, ligation and clip, ligation and division, clip by thoracoscopy); device closure (transcatheter technique).

Operative approach and type of PDA repair by weight (> 2500 g, 2500 g): surgical closure (specify ligation, clip, ligation and clip, ligation and division, clip by thoracoscopy); device closure (transcatheter technique).

PDA mortality and complication rate by year. Length of hospital stay (preoperative and postoperative) by year.

PDA mortality and complication rate by patient weight. Length of hospital stay (preoperative and postoperative) by weight.

B. CoAo

Incidence of isolated CoAo by year, by age at presentation and method of diagnosis.

Incidence of CoAo with VSD by year, by age at presentation, and method of diagnosis.

Incidence of CoAo with complex intracardiac anomaly by year, by age at presentation, and method of diagnosis.

Isolated coarctation repair-operative approach (left thoracotomy, right thoracotomy, median sternotomy, transcatheter technique) and type of repair by year: resection with end-to-end anastomosis, prosthetic patch aortoplasty, prosthetic interposition graft, subclavian flap aortoplasty, resection with extended end-to-end anastomosis, extraanatomic bypass graft, pulmonary artery banding, balloon dilation, balloon dilation with stent placement.

Coarctation with VSD repair-operative approach (left thoracotomy, right thoracotomy, median sternotomy, transcatheter technique) and type of complete repair by year: resection with end-to-end anastomosis, prosthetic patch aortoplasty, prosthetic interposition graft, subclavian flap aortoplasty, resection with extended end-to-end anastomosis, extraanatomic bypass graft, pulmonary artery banding, balloon dilation, balloon dilation with stent placement.

Coarctation with VSD repair-operative approach (left thoracotomy, right thoracotomy, median sternotomy, transcatheter technique) and type of staged repair by year: with pulmonary artery banding/without pulmonary artery banding.

Features of repair of coarctation with VSD: CPB (yes or no), circulatory arrest (yes or no), circulatory arrest time (minutes).

Coarctation with complex intracardiac anomaly repair—operative approach (left thoracotomy, right thoracotomy, median sternotomy, transcatheter technique) and type of complete repair by year: resection with end-to-end anastomosis, prosthetic patch aortoplasty, prosthetic interposition graft, subclavian flap aortoplasty, resection with extended end-to-end anastomosis, extraanatomic bypass graft, pulmonary artery banding, balloon dilation, balloon dilation with stent placement.

Coarctation with complex intracardiac anomaly repair—operative approach (left thoracotomy, right thoracotomy, median sternotomy, transcatheter technique) and type of staged repair by year: resection with end-to-end anastomosis, prosthetic patch aortoplasty, prosthetic interposition graft, subclavian flap aortoplasty, resection with extended end-to-end anastomosis, extraanatomic bypass graft, pulmonary artery banding, balloon dilation, balloon dilation with stent placement; with pulmonary artery banding/without pulmonary artery banding.

Features of repair of coarctation with complex intracardiac anomaly: CPB (yes or no), circulatory arrest (yes or no), circulatory arrest time (minutes).

Isolated coarctation, mortality and complication rate by year. Length of hospital stay (preoperative and postoperative) by year.

Isolated coarctation, mortality and complication rate by patient weight. Length of hospital stay (preoperative and postoperative) by year.

Coarctation with VSD, mortality and complication rate by year. Length of hospital stay (preoperative and postoperative) by year.

Coarctation with VSD, mortality and complication rate by patient weight. Length of hospital stay (preoperative and postoperative) by year.

Coarctation with complex intracardiac anomaly, mortality and complication rate by year. Length of hospital stay (preoperative and postoperative) by year.

Coarctation with complex intracardiac anomaly, mortality and complication rate by patient weight. Length of hospital stay (preoperative and postoperative) by weight.

C. IAA

Incidence of IAA (type A) by year, by age at presentation, and method of diagnosis.

Incidence of IAA (type B) by year, by age at presentation and method of diagnosis.

Incidence of IAA (type C) by year, by age at presentation and method of diagnosis.

IAA (type A) staged repair type by year: stage I with pulmonary artery band; stage I without pulmonary artery band; stage II with pulmonary artery band takedown; stage II with VSD closure

IAA (type A) complete repair type by year: with VSD closure; conduit (Gore-Tex, Dacron, homograft); direct anastomosis; with subaortic resection, without subaortic resection, with PFO/ASD closure, without PFO/ASD, with PDA ligation/division, with PDA ligation; conduit change; conduit additions

Features of repair (type A) of interrupted aortic arch: CPB (yes or no), circulatory arrest (yes or no), circulatory arrest time (minutes).

IAA (type A), incidence of DiGeorge syndrome.

IAA (type B) staged repair type by year: stage I with pulmonary artery band; stage I without pulmonary artery band; stage II with pulmonary artery band takedown; stage II with VSD closure

Interrupted aortic arch (type B) complete repair type by year: with VSD closure; conduit (Gore-Tex, Dacron, homograft); direct anastomosis; with subaortic resection, without subaortic resection, with PFO/ASD closure, without PFO/ASD, with PDA ligation/division, with PDA ligation; conduit change; conduit additions

Features of repair (type B) of interrupted aortic arch: CPB (yes or no), circulatory arrest (yes or no), circulatory arrest time (minutes).

IAA (type B), incidence of DiGeorge syndrome.

IAA (type C) staged repair type by year: stage I with pulmonary artery band; stage I without pulmonary artery band; stage II with pulmonary artery band takedown; stage II with VSD closure

IAA (type C) complete repair type by year: with VSD closure; conduit (Gore-Tex, Dacron, homograft); direct anastomosis; with subaortic resection, without subaortic resection, with PFO/ASD closure, without PFO/ASD, with PDA ligation/division, with PDA ligation; conduit change; conduit additions

Features of repair (type C) of interrupted aortic arch: CPB (yes or no), circulatory arrest (yes or no), circulatory arrest time (minutes).

IAA (type C), incidence of DiGeorge syndrome.

IAA (type A), mortality and complication rate by year. Length of hospital stay (preoperative and postoperative) by year.

IAA (type B), mortality and complication rate by year. Length of hospital stay (preoperative and postoperative) by year.

IAA (type C), mortality and complication rate by year. Length of hospital stay (preoperative and postoperative) by year.

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