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Ann Thorac Surg 2000;69:S83-S96
© 2000 The Society of Thoracic Surgeons

Congenital Heart Surgery Nomenclature and Database Project: right ventricular outflow tract obstruction–intact ventricular septum

^a Marie Lannelongue Hospital, Paris, France

Address reprint requests to Dr Lacour-Gayet, Marie Lannelongue Hospital, 133 Ave de la Résistance, 92350 Le Plessis Robinson, France
e-mail: flacourgayet{at}ccml.com

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

Abstract

The extant nomenclature for pulmonary atresia/stenosis with intact ventricular septum, pulmonary artery and/or pulmonary branch stenosis, double chambered right ventricle, absent pulmonary valve with intact ventricular septum, and ventricular to pulmonary artery conduit failure 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. Multiple risk factors were considered such as coronary artery fistulas/sinusoids, tricuspid annular size and location of peripheral pulmonary artery stenoses. 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. Potential diagnostic related risk factors for each lesion are presented.

This manuscript will cover the nomenclature for the following six lesions grouped together under the heading of RVOT Obstruction–Intact Ventricular Septum:

Pulmonary Atresia–Intact Ventricular Septum

Pulmonary Stenosis–Intact Ventricular Septum

Pulmonary Artery or Pulmonary Branch Stenosis

Double Chambered Right Ventricle

Absent Pulmonary Valve, Intact Ventricular Septum

Ventricular to PA Conduit Failure

I. Pulmonary atresia—intact ventricular septum (PA–IVS)

A. Background
Definition
Pulmonary atresia (PA) and intact ventricular septum (IVS) is a duct-dependent congenital malformation that forms a spectrum of lesions including atresia of the pulmonary valve, a various degree of right ventricle and tricuspid valve hypoplasia, and anomalies of the coronary circulation.

The favorable forms are treated by biventricular repair following satisfactory growth of the right ventricle which is effected by various palliative procedures including: percutaneous pulmonary valvotomy, open valvotomy, and transannular, all of which may or may not be associated with a systemic-pulmonary shunt.

The severe forms are treated by univentricular repair. The severely hypoplastic right ventricle is frequently associated with RV dependent coronary circulation that preclude RV decompression procedures.

The intermediate forms with coronary sinusoids without coronary stenosis or the forms with borderline growth of the RV cavity raise difficult issues and lead to challenging decision making; one and a half biventricular repair can offer a satisfactory solution.

B. Analysis: a unified nomenclature system for pulmonary atresia–intact ventricular septum (PA–IVS)

The initial classification by de Leval and colleagues [1] into tripartite, bipartite, and monopartite right ventricle has progressively evolved to a classification [2, 3] using only the diameter of the tricuspid valve and based on a Z score ranging from -5 to +5.

The presence of RV to coronary artery fistula is inversely related to the size of the tricuspid valve [2, 4]. A right ventricular dependent coronary artery circulation (RVDCC) is present when coronary artery fistulae (coronary sinusoids) [2] are associated with a proximal coronary artery stenosis; it represents the major surgical risk factor [2–5]. The presence of an associated coronary stenosis creates a true RV dependent coronary circulation and precludes RV decompression procedure.

The surgical management of patients with RV to coronary fistulae is complex and still under investigation [10]. The presence of a true RVDCC (10% of the patients) [2] precludes RV decompression procedures and is currently reated by total cavo-pulmonary artery connection. The quality of the final Fontan circulation can be impaired by LV endocardial fibrosis caused by hypoxic RV dependent coronary circulation and by potential subaortic septal bulging related to a residual high intracavitary RV pressure. These potential risks may lead to consideration of heart transplantation.

The presence of RV to coronary fistulae without RVDCC (no coronary stenosis) allows decompressing or excluding the RV [5, 6, 10] in order to close the fistulae.

When satisfactory growth of the tricuspid valve cannot be achieved, it has been proposed to perform a partial biventricular repair (one and a half) [7, 8].

More recently, RV endocardial fibrosis pealing [27] has been proposed to improve the RV growth.

Associated Ebstein’s anomaly of the tricuspid valve can be present; the tricuspid diameter is enlarged and the prognosis is poor [2, 9].

Hierarchy level 1

Pulmonary atresia—Intact Ventricular Septum (PA—IVS)

Hierarchy level 2

Pulmonary atresia—IVS, NOS

Pulmonary atresia—IVS, No coronary fistulas/sinusoids

Pulmonary atresia—IVS, Coronary fistulas/sinusoids-Non RV dependent coronary circulation

Pulmonary atresia—IVS, Coronary fistulas/sinusoids-RV dependent coronary circulation

Hierarchy level 3

Pulmonary atresia—IVS, NOS

Pulmonary atresia—IVS, No coronary fistulas/sinusoids, NOS

Pulmonary atresia—IVS, No coronary fistulas/sinusoids, TV Z-score 1 or larger

Pulmonary atresia—IVS, No coronary fistulas/sinusoids, TV Z-score 0

Pulmonary atresia—IVS, No coronary fistulas/sinusoids, TV Z-score -1

Pulmonary atresia—IVS, No coronary fistulas/sinusoids, TV Z-score -2

Pulmonary atresia—IVS, No coronary fistulas/sinusoids, TV Z-score -3

Pulmonary atresia—IVS, No coronary fistulas/sinusoids, TV Z-score -4

Pulmonary atresia—IVS, No coronary fistulas/sinusoids, TV Z-score -5 or smaller

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—Non RV dependent coronary circulation, NOS

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—Non RV dependent coronary circulation, TV Z-score 1 or larger

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—Non RV dependent coronary circulation, TV Z-score 0

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—Non RV dependent coronary circulation, TV Z-score -1

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—Non RV dependent coronary circulation, TV Z-score -2

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—Non RV dependent coronary circulation, TV Z-score -3

Pulmonary atresia—IVS, Coronary fistulas/sinuosoids-Non RV dependent coronary circulation, TV Z-score -4

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—Non RV dependent coronary circulation, TV Z-score -5 or smaller

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—RV dependent coronary circulation, NOS

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—RV dependent coronary circulation, TV Z-score 1 or larger

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—RV dependent coronary circulation, TV Z-score 0

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—RV dependent coronary circulation, TV Z-score -1

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—RV dependent coronary circulation, TV Z-score -2

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—RV dependent coronary circulation, TV Z-score -3

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—RV dependent coronary circulation, TV Z-score -4

Pulmonary atresia—IVS, Coronary fistulas/sinusoids—RV dependent coronary circulation, TV Z-score -5 or smaller

GUEST EDITORS’ NOTE: Ebstein’s anomaly and LVOT obstruction may exist with pulmonary atresia, IVS. These lesions will be coded in detail in the computer database as additional diagnoses.

C. Nomenclature for pulmonary atresia–intact ventricular septum (PA–IVS) treatment options

Procedures

ASD repair, secundum

ASD repair, PFO

ASD partial closure

Atrial septal fenestration

Ebstein’s anomaly repair

Endocardial fibrosis pealing, RV

RVOT,

Patch enlargement, transannular (TAP)

RV to PA conduit, NOS

RV to PA conduit, homograft reconstruction

RV to PA conduit, nonhomograft conduit re construction

Valvuloplasty,

Pulmonic

Pulmonic, Valvotomy, Percutaneous

Pulmonic, Valvotomy, Closed Heart, Transventricular

Pulmonic, Valvotomy, Closed Heart, Via pulmonary artery

Pulmonic, Valvotomy, Inflow occlusion

Pulmonic, Valvotomy, With CPB

Tricuspid

Tricuspid, Ebstein’s anomaly

Tricuspid, Valvectomy

Tricuspid, Tricuspid valve closure (thrombo exclusion)

Shunt,

Systemic to pulmonary, Modified Blalock-Taussig Shunt (MBTS)

Systemic to pulmonary, Modified Blalock-Taussig Shunt (MBTS), Left (LMBTS)

Systemic to pulmonary, Modified Blalock-Taussig Shunt (MBTS), Right (RMBTS)

Systemic to pulmonary, Central (From aorta or to main pulmonary artery)

Systemic to pulmonary, Other

Ligation and takedown

Bilateral bidirectional cavopulmonary anastomosis (BBDCPA) (bilateral bidirectional Glenn)

Glenn (unidirectional cavopulmonary anastomosis) (unidirectional Glenn)

Hemifontan

Fontan

NOS

Atrio–pulmonary connection

Atrio–ventricular connection

TCPC, Lateral tunnel, Fenestrated

TCPC, Lateral tunnel, Non-fenestrated

TCPC, External conduit, Fenestrated

TCPC, External conduit, Non-fenestrated

Other

1 ventricular repair (Partial biventricular repair)

Transplant, heart

D. Diagnosis and procedure short lists for pulmonary atresia–intact ventricular septum (PA–IVS)

Diagnosis Short List

Pulmonary atresia, IVS

Procedure Short List

ASD repair, primary closure

ASD repair, patch

PFO, Primary closure

ASD partial closure

Atrial septal fenestration

Ebstein’s anomaly repair

RVOT procedure

Valvuloplasty, pulmonic

Valvuloplasty, tricuspid

Valve closure, tricuspid

Shunt, systemic to pulmonary, modified Blalock-Taussig Shunt (MBTS)

Shunt, systemic to pulmonary, central (From aorta or to main pulmonary artery)

Shunt, systemic to pulmonary, Other

Shunt, ligation and takedown

Bilateral bidirectional cavopulmonary anastomosis (BBDCPA) (Bilateral bidirectional Glenn)

Glenn (Unidirectional cavopulmonary anastomosis) (Unidirectional Glenn)

Hemifontan

Fontan,

NOS

Atrio—pulmonary connection

Atrio—ventricular connection

TCPC, Lateral tunnel, Fenestrated

TCPC, Lateral tunnel, Non-fenestrated

TCPC, External conduit, Fenestrated

TCPC, External conduit, Non-fenestrated

Other

1 ventricular repair (Partial biventricular repair)

Transplant, heart

E. Potential diagnostic related risk factors for pulmonary atresia, intact ventricular septum (PA—IVS)

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 Pulmonary Atresia—Intact Ventricular Septum (PA—IVS) include the following data fields:

Coronary artery anomalies, RV to coronary artery fistulae

Ebstein’s anomaly

Specific postoperative complications of pulmonary atresia–intact ventricular septum (PA–IVS) to be studied include:

Complications: RV failure

Lesion specific risk factors include:

Risk: Coronary artery anomalies, RV to coronary artery fistulae

II. Pulmonary stenosis–intact ventricular septum (PS–IVS)

A. Background
Pulmonary stenosis (PS) with intact ventricular septum (IVS) defines a spectrum of lesions ranging from critical neonatal pulmonic valve stenosis with hypoplasia of the right ventricle to valvar pulmonary stenosis in infant, child, or adult, usually better tolerated but potentially associated with infundibular stenosis.

This field has considerably benefited from the development of interventional cardiology [11, 12] and only a minority of patients are currently treated by surgery.

B. Analysis: a unified nomenclature system for pulmonary stenosis–intact ventricular septum (PS–IVS)
Neonatal critical PVS usually requires ductal dependency for early survival. In 50% of neonates with PVS, the tricuspid valve diameter is normal (within 2 standard deviation; 0 < Z > -2); in less than 10%, the tricuspid valve is severely hypoplastic (Z = -4 or -5). Only 10% of neonates have RV to coronary artery fistulae, and only 2% have a RV dependent coronary circulation [13]. The RV cavity dimension is rarely reduced and the reduction, if present, is more in relation with a concentric muscular hypertrophy [14]. Marked endocardial fibrosis can be seen in the most severe cases of hypoplasia of the RV cavity and is probably related to an imbalance in the myocardial oxygen supply to demand ratio [14]. Pulmonary branch hypoplasia can be associated [13]. The size of the RV cavity is not highly correlated with the diameter of the tricuspid valve [13]; the reduction in size being secondary to RV hypertrophy and not to an abnormal structural development as it is observed in PA-IVS. Critical PVS is rarely seen in patients with enlarged RV cavity; this condition has a poor prognosis and is usually associated with cardiomyopathy [13].

Neonatal surgical repair is performed by open pulmonary valvotomy or by transannular patching in the most severe form. Rarely, a systemic to pulmonary shunt is added.

Beyond the neonatal period, in infancy and childhood, the leaflet tissue may become thickened and myxomatous with retraction of the commissure attachment frequently creating an associated supravalvar stenosis. Following RV concentric hypertrophy, severe infundibular stenosis is frequently present. The rare surgical procedures are performed in patients with severe infundibular stenosis, where an open pulmonary valvotomy requires an associated infundibular resection and/or patch enlargement.

Hierarchy level 1

Pulmonary stenosis—Intact Ventricular Septum (PS—IVS)

Hierarchy level 2

Pulmonary stenosis—IVS, NOS

Pulmonary stenosis—IVS, Subvalvar

Pulmonary stenosis—IVS, Supravalvar

Pulmonary stenosis—IVS, Valvar

Hierarchy level 3

Pulmonary stenosis—IVS, NOS

Pulmonary stenosis—IVS, Subvalvar

Pulmonary stenosis—IVS, Supravalvar

Pulmonary stenosis—IVS, Valvar, NOS

Pulmonary stenosis—IVS, Valvar, No coronary fistulas/sinusoids

Pulmonary stenosis–IVS, Valvar, Coronary fistulas/sinusoids–Non-RV dependent coronary circulation

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-RV dependent coronary circulation

Hierarchy level 4

Pulmonary stenosis—IVS, NOS

Pulmonary stenosis—IVS, Subvalvar

Pulmonary stenosis—IVS, Supravalvar

Pulmonary stenosis—IVS, Valvar, NOS

Pulmonary stenosis—IVS, Valvar, No coronary fistulas/sinusids, NOS

Pulmonary stenosis—IVS, Valvar, No coronary fistulas/sinusoids, TV Z-score 1 or larger

Pulmonary stenosis—IVS, Valvar, No coronary fistulas/sinusoids, TV Z-score 0

Pulmonary stenosis—IVS, Valvar, No coronary fistulas/sinusoids, TV Z-score -1

Pulmonary stenosis—IVS, Valvar, No coronary fistulas/sinusoids, TV Z-score -2

Pulmonary stenosis—IVS, Valvar, No coronary fistulas/sinusoids, TV Z-score -3

Pulmonary stenosis—IVS, Valvar, No coronary fistulas/sinusoids, TV Z-score -4

Pulmonary stenosis—IVS, Valvar, No coronary fistulas/sinusoids, TV Z-score -5 or smaller

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-Non RV dependent coronary circulation, NOS

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-Non RV dependent coronary circulation, TV Z-score 1 or larger

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-Non RV dependent coronary circulation, TV Z-score 0

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-Non RV dependent coronary circulation, TV Z-score -1

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-Non RV dependent coronary circulation, TV Z-score -2

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-Non RV dependent coronary circulation, TV Z-score -3

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-Non RV dependent coronary circulation, TV Z-score -4

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-Non RV dependent coronary circulation, TV Z-score -5 or smaller

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-RV dependent coronary circulation, NOS

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-RV dependent coronary circulation, TV Z-score 1 or larger

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-RV dependent coronary circulation, TV Z-score 0

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-RV dependent coronary circulation, TV Z-score -1

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-RV dependent coronary circulation, TV Z-score -2

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-RV dependent coronary circulation, TV Z-score -3

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-RV dependent coronary circulation, TV Z-score -4

Pulmonary stenosis—IVS, Valvar, Coronary fistulas/sinusoids-RV dependent coronary circulation, TV Z-score -5 or smaller

C. Nomenclature for pulmonary stenosis–intact ventricular septum (PS–IVS) treatment options
Procedures

ASD repair, secundum

ASD repair, PFO

Endocardial fibrosis pealing, RV

RVOT

Infundibular muscle resection (Sub PS resection)

Patch enlargement, NOS

Patch enlargement, Nontransannular

Patch enlargement, Transannular (TAP)

Valvuloplasty,

Pulmonic

Pulmonic, Valvotomy, Percutaneous

Pulmonic, Valvotomy, Closed heart, Trans ventricular

Pulmonic, Valvotomy, Closed heart, Via pulmonary artery

Pulmonic, Valvotomy, Inflow occlusion

Pulmonic, Valvotomy, With CPB

Shunt, Systemic to pulmonary,

Modified Blalock-Taussig Shunt (MBTS)

Modified Blalock-Taussig Shunt (MBTS), Left (LMBTS)

Modified Blalock-Taussig Shunt (MBTS), Right (RMBTS)

Central (From aorta or to main pulmonary artery)

Other

Shunt, Ligation and takedown

Bilateral bidirectional cavopulmonary anastomosis (BBDCPA) (Bilateral bidirectional Glenn)

Glenn (Unidirectional cavopulmonary anastomosis) (Unidirectional Glenn)

Hemifontan

Fontan, NOS

Atrio—pulmonary connection

Atrio—ventricular connection

TCPC, Lateral tunnel, Fenestrated

TCPC, Lateral tunnel, Non-fenestrated

TCPC, External conduit, Fenestrated

TCPC, External conduit, Non-fenestrated

Other

1 ventricular repair (Partial biventricular repair)

Transplant, heart

PA reconstruction (plasty), NOS

PA reconstruction (plasty), Main (Trunk)

Note: The various types of PA plasty are defined in detail in the next section—Pulmonary Artery and/or Pulmonary Branches Stenoses.

D. Diagnosis and procedure short lists for pulmonary stenosis–intact ventricular septum (PS–IVS)

Diagnosis Short List

Pulmonary stenosis, valvar

Pulmonary valve, other

Procedure Short List

ASD repair,

Primary closure

Patch

PFO, Primary closure

RVOT procedure

Valvuloplasty, pulmonic

Shunt, Systemic to pulmonary,

Modified Blalock-Taussig Shunt (MBTS)

Central (From aorta or to main pulmonary artery)

Other

Shunt, Ligation and takedown

Bilateral bidirectional cavopulmonary anastomosis (BBDCPA) (Bilateral bidirectional Glenn)

Glenn (Unidirectional cavopulmonary anastomosis) (Unidirectional Glenn)

Hemifontan

Fontan, NOS

Atrio–pulmonary connection

Atrio–ventricular connection

TCPC, Lateral tunnel, Fenestrated

TCPC, Lateral tunnel, Non-fenestrated

TCPC, External conduit, Fenestrated

TCPC, External conduit, Non-fenestrated

Other

1 ventricular repair (Partial biventricular repair)

Transplant, Heart

PA reconstruction (plasty),

Main

NOS

Main (trunk)

E. Potential diagnostic related risk factors for pulmonary stenosis–intact ventricular septum (PS–IVS)

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 Pulmonary Stenosis—Intact Ventricular Septum (PS—IVS) include the following data fields:

Coronary artery anomalies, RV to coronary artery fistulae

Specific postoperative complications of Pulmonary Stenosis—Intact Ventricular Septum (PS—IVS) to be studied include:

Complications: RV failure

Lesion specific risk factors include:

Risk: Coronary artery anomalies, RV to coronary artery fistulae

III. Pulmonary artery and/or pulmonary branches stenoses

A. Background
The terms pulmonary artery and/or pulmonary branch stenosis define a congenital anomaly presenting with pulmonary trunk and/or branch narrowing. The pulmonary trunk and/or branch stenoses may be an isolated lesion or may be associated with other lesions such as valvar PS or TOF. The pulmonary trunk and/or branch stenoses may be congenital or acquired.

B. Analysis: a unified nomenclature system for pulmonary artery and/or pulmonary branches stenoses
The native forms of pulmonary artery and/or branches stenosis are very frequently associated with extracardiac anomalies in comorbid conditions like the Williams and Beuren syndromes [15], the Alagille syndrome (intrahepatic biliary duct agenesis) [16], and the 22q11 deletion [21].

The lesions can be unilateral or bilateral, with or without bifurcation stenosis; moreover the lesions frequently extend towards the periphery with segmental branch stenoses [15].

Different is the coarctation of pulmonary artery [17] that is related to an abnormal extension of the ductus arteriosus into a pulmonary branch and more frequently the left branch [18].

The PA artery stenosis following PA banding is part of this diagnostic group. Patients with pulmonary artery and/or branches stenosis are frequently treated by percutaneous balloon dilation and stent angioplasty. The surgical repairs include patch enlargement and possibly intra-operative balloon angioplasty [20].

Hierarchy level 1

Pulmonary artery coarctation

Pulmonary artery stenosis (Hypoplasia)

Hierarchy level 2

Pulmonary artery coarctation, NOS

Pulmonary artery coarctation, Left

Pulmonary artery coarctation, Right

Pulmonary artery stenosis (Hypoplasia), NOS

Pulmonary artery stenosis (Hypoplasia), Main (trunk)

Pulmonary artery stenosis (Hypoplasia), Branch

Hierarchy level 3

Pulmonary artery coarctation, NOS

Pulmonary artery coarctation, Left

Pulmonary artery coarctation, Right

Pulmonary artery stenosis (Hypoplasia), NOS

Pulmonary artery stenosis (Hypoplasia), Main (Trunk)

Pulmonary artery stenosis (Hypoplasia), Branch, NOS

Pulmonary artery stenosis (Hypoplasia), Branch, Central (Within the hilar bifurcation)

Pulmonary artery stenosis (Hypoplasia), Branch, Peripheral (At or beyond the hilar bifurcation)

Hierarchy level 4

Pulmonary artery coarctation, NOS

Pulmonary artery coarctation, Left

Pulmonary artery coarctation, Right

Pulmonary artery stenosis (Hypoplasia), NOS

Pulmonary artery stenosis (Hypoplasia), Main (Trunk)

Pulmonary artery stenosis (Hypoplasia), Branch, NOS

Pulmonary artery stenosis (Hypoplasia), Branch, Central (within the hilar bifurcation), NOS

Pulmonary artery stenosis (Hypoplasia), Branch, Central (within the hilar bifurcation), Left

Pulmonary artery stenosis (Hypoplasia), Branch, Central (within the hilar bifurcation), Right

Pulmonary artery stenosis (Hypoplasia), Branch, Central (within the hilar bifurcation), Left and right

Pulmonary artery stenosis (Hypoplasia), Branch, Central (within the hilar bifurcation), Left and right including PA carinal stenosis

Pulmonary artery stenosis (Hypoplasia), Branch, Peripheral (at or beyond the hilar bifurcation), NOS

Pulmonary artery stenosis (Hypoplasia), Branch, Peripheral (at or beyond the hilar bifurcation), Left

Pulmonary artery stenosis (Hypoplasia), Branch, Peripheral (at or beyond the hilar bifurcation), Right

Pulmonary artery stenosis (Hypoplasia), Branch, Peripheral (at or beyond the hilar bifurcation), Left and right

Additional modifiers for pulmonary artery and/or pulmonary branch stenosis

Hierarchy level 1

Pulmonary artery stenosis, Congenital

Pulmonary artery stenosis, Acquired

Pulmonary artery stenosis, Congenital

Pulmonary artery stenosis, Acquired, NOS

Pulmonary artery stenosis, Acquired, Not postoperative

Pulmonary artery stenosis, Acquired, Postoperative

Non-cardiac abnormalities for pulmonary artery and/or pulmonary branches stenoses
The native forms of pulmonary artery and/or branches stenosis are very frequently associated with extracardiac anomalies in comorbid conditions. The database will allow coding of these preexisting anomalies. In the Minimum Database, a field exists called "Non-cardiac Abnormalities." Entries in this field will come from a Noncardiac Abnormalities Short List. Noncardiac Abnormalities field is especially important for the diagnosis of Pulmonary Artery and/or Pulmonary Branches Stenoses. Relevant possible entries from the Noncardiac Abnormalities Short List for pulmonary artery or branches stenosis are given below.

Noncardiac abnormalities for pulmonary artery and/or pulmonary branches stenoses

Williams Beuren syndrome

Alagille syndrome (Intra-hepatic biliary duct agenesis)

22q11 deletion

Rubella

C. Nomenclature for pulmonary artery and/or pulmonary branches stenoses treatment options

Hierarchy level 1

PA reconstruction (plasty)

Hierarchy level 2

PA reconstruction (plasty), NOS

PA reconstruction (plasty), Main

PA reconstruction (plasty), Branch

Hierarchy level 3

PA reconstruction (plasty), NOS

PA reconstruction (plasty), Main, NOS

PA reconstruction (plasty), Main, MPA

PA reconstruction (plasty), Main, MPA and LPA

PA reconstruction (plasty), Main, MPA and RPA

PA reconstruction (plasty), Main, MPA/LPA/RPA

PA reconstruction (plasty), Branch, NOS

PA reconstruction (plasty), Branch, Central (Within the hilar bifurcation)

PA reconstruction (plasty), Branch, Peripheral (At or beyond the hilar bifurcation)

Hierarchy level 4

PA reconstruction (plasty), NOS

PA reconstruction (plasty), Main, NOS PA reconstruction (plasty), Main, MPA

PA reconstruction (plasty), Main, MPA and LPA, NOS

PA reconstruction (plasty), Main, MPA and LPA, 1 Patch

PA reconstruction (plasty), Main, MPA and LPA, 2 Patches

PA reconstruction (plasty), Main, MPA and RPA, NOS

PA reconstruction (plasty), Main, MPA and RPA, 1 Patch

PA reconstruction (plasty), Main, MPA and RPA, 2 Patches

PA reconstruction (plasty) Main, MPA/LPA/RPA, NOS

PA reconstruction (plasty) Main, MPA/LPA/RPA, 1 patch

PA reconstruction (plasty), Main, MPA/LPA/RPA, 2 Patches: MPA to LPA/separate RPA

PA reconstruction (plasty), Main, MPA/LPA/RPA, 2 Patches: MPA to RPA/separate LPA

PA reconstruction (plasty), Main, MPA/LPA/RPA, 2 Patches: RPA to LPA/separate MPA

PA reconstruction (plasty), Main, MPA/LPA/RPA, 3 Patches

PA reconstruction (plasty), Branch, NOS

PA reconstruction (plasty), Branch, Left

PA reconstruction (plasty), Branch, Right

PA reconstruction (plasty), Branch, Bilateral

PA reconstruction (plasty), Branch, Central (within the hilar bifurcation), NOS

PA reconstruction (plasty), Branch, Central (within the hilar bifurcation), Left

PA reconstruction (plasty), Branch, Central (within the hilar bifurcation), Right

PA reconstruction (plasty), Branch, Central (within the hilar bifurcation), Bilateral

PA reconstruction (plasty) Branch, Central (within the hilar bifurcation), Bilateral with PA carinal reconstruction

PA reconstruction (plasty), Branch, Peripheral (at or beyond the hilar bifurcation), NOS

PA reconstruction (plasty), Branch, Peripheral (at or beyond the hilar bifurcation), Left

PA reconstruction (plasty), Branch, Peripheral (at or beyond the hilar bifurcation), Right

PA reconstruction (plasty), Branch, Peripheral (at or beyond the hilar bifurcation), Bilateral

Additional modifiers for treatment options for pulmonary artery and/or pulmonary branches stenoses

Hierarchy level 1

PA reconstruction (plasty), Debanding

PA reconstruction (plasty), Intraoperative balloon angioplasty

PA reconstruction (plasty), Intraoperative stent placement

Hierarchy level 2

PA reconstruction (plasty), Debanding

PA reconstruction (plasty), Intraoperative balloon angioplasty, NOS

PA reconstruction (plasty), Intraoperative balloon angioplasty, Main

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch

PA reconstruction (plasty), Intraoperative stent placement, NOS

PA reconstruction (plasty), Intraoperative stent placement, Main

PA reconstruction (plasty), Intraoperative stent placement, Branch

Hierarchy level 3

PA reconstruction (plasty), Debanding

PA reconstruction (plasty), Intraoperative balloon angioplasty, NOS

PA reconstruction (plasty), Intraoperative balloon angioplasty, Main

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, NOS

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Central (Within the hilar bifurcation)

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Peripheral (At or beyond the hilar bifurcation)

PA reconstruction (plasty), Intraoperative stent placement, NOS

PA reconstruction (plasty), Intraoperative stent placement, Main

PA reconstruction (plasty), Intraoperative stent placement, Branch, NOS

PA reconstruction (plasty), Intraoperative stent placement, Branch, Central (Within the hilar bifurcation)

PA reconstruction (plasty), Intraoperative stent placement, Branch, Peripheral (At or beyond the hilar bifurcation)

Hierarchy level 4

PA recostruction (plasty), Debanding

PA reconstruction (plasty), Intraoperative balloon angioplasty, NOS

PA reconstruction (plasty), Intraoperative balloon angioplasty, Main

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, NOS

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Left

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Right

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Bilateral

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Central (within the hilar bifurcation), NOS

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Central (within the hilar bifurcation, Left

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Central (within the hilar bifurcation), Right

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Central (within the hilar bifurcation), Bilateral

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Peripheral (at or beyond the hilar bifurcation), NOS

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Peripheral (at or beyond the hilar bifurcation), Left

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Peripheral (at or beyond the hilar bifurcation), Right

PA reconstruction (plasty), Intraoperative balloon angioplasty, Branch, Peripheral (at or beyond the hilar bifurcation), Bilateral

PA reconstruction (plasty), Intraoperative stent placement, NOS

PA reconstruction (plasty), Intraoperative stent placement, Main

PA reconstruction (plasty), Intraoperative stent placement, Branch, NOS

PA reconstruction (plasty), Intraoperative stent placement, Branch, Left

PA reconstruction (plasty), Intraoperative stent placement, Branch, Right

PA reconstruction (plasty), Intraoperative stent placement, Branch, Bilateral

PA reconstruction (plasty), Intraoperative stent placement, Branch, Central (within the hilar bifurcation), NOS

PA reconstruction (plasty), Intraoperative stent placement, Branch, Central (within the hilar bifurcation), Left

PA reconstruction (plasty), Intraoperative stent placement, Branch, Central (within the hilar bifurcation), Right

PA reconstruction (plasty), Intraoperative stent placement, Branch, Central (within the hilar bifurcation), Bilateral

PA reconstruction (plasty), Intraoperative stent placement, Branch, Peripheral (at or beyond the hilar bifurcation), NOS

PA reconstruction (plasty), Intraoperative stent placement, Branch, Peripheral (at or beyond the hilar bifurcation), Left

PA reconstruction (plasty), Intraoperative stent placement, Branch, Peripheral (at or beyond the hilar bifurcation), Right

PA reconstruction (plasty), Intraoperative stent placement, Branch, Peripheral (at or beyond the hilar bifurcation), Bilateral

D. Diagnosis and procedure short lists for pulmonary artery and/or pulmonary branches stenoses

Diagnosis Short List

Pulmonary artery stenosis (Hypoplasia), NOS

Pulmonary artery stenosis (Hypoplasia), Main Trunk

Pulmonary artery stenosis (Hypoplasia), Branch, Central (within the hilar bifurcation)

Pulmonary artery stenosis (Hypoplasia), Branch, Peripheral (at or beyond the hilar bifurcation)

Procedure Short List

PA reconstruction (plasty), NOS

PA reconstruction (plasty), Main (trunk)

PA reconstruction (plasty), Central (within the hilar bifurcation)

PA reconstruction (plasty), Peripheral (at or beyond the hilar bifurcation)

E. Potential diagnostic related risk factors for pulmonary artery and/or pulmonary branches stenoses
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 Pulmonary Artery or Pulmonary Branches Stenoses include the following data fields:

Pulmonary artery stenosis (Hypoplasia), Branch, Peripheral (At or beyond the hilar bifurcation)

Specific postoperative complications of Pulmonary Artery or Pulmonary Branches Stenoses to be studied include:

Complications: RV failure

Lesion specific risk factors include:

Risks: Pulmonary artery stenosis (Hypoplasia), Branch, Peripheral (At or beyond the hilar bifurcation)

IV. Double chambered right ventricle

A. Background
The term double chambered right ventricle (DCRV) defines a congenital anomaly characterized by a low infundibular stenosis frequently associated with one or several "closing" VSDs. In other cases, the VSD is already closed. The DCRV without VSD is similar to the rare isolated infundibular stenosis that develops more superiorly in the infundibulum [13].

B. Analysis: a unified nomenclature system for double chambered right ventricle
The RV intracavitary stenosis is produced by muscular band hypertrophy and is located between the trabecula septomarginalis posteriorly and the parietal band anteriorly [22]. The orifice can be extremely reduced, less than 5 mm, and the endocardial muscle is covered by a localized white fibrosis that encircles the stenosed area. The stenosis creates two chambers in the RV; one inferior including the inlet and trabecular portions of the right ventricle and one superior reduced to the infundibulum.

In two thirds of the cases, an associated closing VSD is present [22, 23]. Frequently perimembranous, it can also be infundibular. Multiple VSDs can be present in either chamber [24].

The surgical repair combines the closure of the VSD when present with the relief of the infundibular stenosis. The operation is currently performed through the tricuspid valve with muscle resection. A ventriculotomy may be required and is repaired by patch enlargement of the infundibulum [23, 24].

Hierarchy level 1

Double chambered right ventricle (DCRV)

Hierarchy level 2

DCRV, NOS

DCRV, No VSD

DCRV, VSD

Hierarchy level 3

DCRV, NOS

DCRV, No VSD

DCRV, VSD, NOS

DCRV, VSD, To lower RV chamber

DCRV, VSD, To upper RV chamber

DCRV, VSD, To lower and upper RV chamber

C. Nomenclature for double chambered right ventricle treatment options
Hierarchy level 1

DCRV repair

Hierarchy level 2

DCRV repair, NOS

DCRV, repair, No ventriculotomy

DCRV, repair, Ventriculotomy

D. Diagnosis and procedure short lists for double chambered right ventricle

Diagnosis Short List

DCRV

Procedure Short List

DCRV repair

E. Potential diagnostic related risk factors for double chambered right ventricle
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 Double Chambered Right Ventricle include the following data fields:

None

Specific postoperative complications of Double Chambered Right Ventricle to be studied include:

Complication: none

Lesion specific risk factors include:

Risk: none

V. Absent pulmonary valve, intact ventricular septum

A. Background
Absent pulmonary valve syndrome is usually associated with tetralogy of Fallot. Rarely, absent pulmonary valve can be seen with intact ventricular septum [25]. Patients present in infancy with respiratory failure with various degrees of bronchial compression. When not responding to postural methods, patients may require reduction pulmonary artery arterioplasty with pulmonic valvuloplasty or pulmonary valve insertion.

B. Analysis: a unified nomenclature system for absent pulmonary valve, intact ventricular septum
Hierarchy level 1

Absent pulmonary valve, intact ventricular septum

C. Nomenclature for absent pulmonary valve, intact ventricular septum treatment options
Hierarchy level 1

Reduction pulmonary artery arterioplasty

Valve insertion, pulmonary

Hierarchy level 2

Reduction pulmonary artery arterioplasty

Valve insertion, pulmonary, NOS

Valve insertion, Pulmonary, Pulmonary homograft

Valve insertion, Pulmonary, Aortic homograft

Valve insertion, Pulmonary, Porcine prosthesis

Valve insertion, Pulmonary, Other

D. Diagnosis and procedure short lists for absent pulmonary valve, intact ventricular septum

Diagnosis Short List

Absent pulmonary valve, Intact ventricular septum

Procedure Short List

RVOT procedure.

E. Potential diagnostic related risk factors for absent pulmonary valve, intact ventricular septum
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 Absent Pulmonary Valve, Intact Ventricular Septum include the following data fields:

None

Specific postoperative complications of absent pulmonary valve, Intact ventricular septum to be studied include:

Complication: none

Lesion specific risk factors include:

Risk: none

VI. Ventricular to PA conduit failure

A. Background
Valved and nonvalved conduit insertion in pediatric cardiac surgery generates conduit-related complications that represent a growing source of reoperations [26]. This diagnostic subgroup includes ventricular to PA conduit failure as well as a variety of other types of conduits also covered in this section.

B. Analysis: a unified nomenclature system for conduit failure
Hierarchy level 1

Conduit failure

Hierarchy level 2

Conduit failure, NOS

Conduit failure, Conduit obstruction

Conduit failure, Conduit stenosis

Conduit failure, Conduit insufficiency

Conduit failure, Conduit insufficiency and stenosis

Hierarchy level 3

Conduit failure, Conduit obstruction, NOS

Conduit failure, Conduit obstruction, LV to aorta

Conduit failure, Conduit obstruction, LV to PA

Conduit failure, Conduit obstruction, RA to RV

Conduit failure, Conduit obstruction, RV to aorta

Conduit failure, Conduit obstruction, RV to PA

Conduit failure, Conduit obstruction, Other

Conduit failure, Conduit stenosis, NOS

Conduit failure, Conduit stenosis, LV to aorta

Conduit failure, Conduit stenosis, LV to PA

Conduit failure, Conduit stenosis, RA to RV

Conduit failure, Conduit stenosis, RV to aorta

Conduit failure, Conduit stenosis, RV to PA

Conduit failure, Conduit stenosis, Other

Conduit failure, Conduit insufficiency, NOS

Conduit failure, Conduit insufficiency, LV to aorta

Conduit failure, Conduit insufficiency, LV to PA

Conduit failure, Conduit insufficiency, RA to RV

Conduit failure, Conduit insufficiency, RV to aorta

Conduit failure, Conduit insufficiency, RV to PA

Conduit failure, Conduit insufficiency, Other

Conduit failure, Conduit insufficiency and stenosis, NOS

Conduit failure, Conduit insufficiency and stenosis, LV to aorta

Conduit failure, Conduit insufficiency and stenosis, LV to PA

Conduit failure, Conduit insufficiency and stenosis, RA to RV

Conduit failure, Conduit insufficiency and stenosis, RV to aorta

Conduit failure, Conduit insufficiency and stenosis, RV to PA

Conduit failure, Conduit insufficiency and stenosis, Other

C. Nomenclature for conduit treatment options
Hierarchy level 1

Conduit reoperation

Hierarchy level 2

Conduit reoperation, NOS

Conduit reoperation, Conduit repair

Conduit reoperation, Conduit replacement

Hierarchy level 3

Conduit reoperation, NOS

Conduit reoperation, Conduit repair, NOS

Conduit reoperation, Conduit repair, Conduit: LV to aorta

Conduit reoperation, Conduit repair, Conduit: LV to PA

Conduit reoperation, Conduit repair, Conduit: RA to RV

Conduit reoperation, Conduit repair, Conduit: RV to aorta

Conduit reoperation, Conduit repair, Conduit: RV to PA

Conduit reoperation, Conduit repair, Conduit: Other

Conduit reoperation, Conduit replacement, NOS

Conduit reoperation, Conduit replacement, Conduit: LV to aorta

Conduit reoperation, Conduit replacement, Conduit: LV to PA

Conduit reoperation, Conduit replacement, Conduit: RA to RV

Conduit reoperation, Conduit replacement, Conduit: RV to aorta

Conduit reoperation, Conduit replacement, Conduit: RV to PA

Conduit reoperation, Conduit replacement, Conduit: Other

D. Diagnosis and procedure short lists for conduit failure

Diagnosis Short List

Conduit failure

Procedure Short List

Conduit reoperation

E. Potential diagnostic related risk factors for conduit failure
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 Conduit Failure include the following data fields:

The initial diagnosis (Conduit failure is an associated diagnosis.)

Specific postoperative complications of Conduit Failure to be studied include [19]:

Complication: bleeding

Lesion specific risk factors include:

Risk: the initial diagnosis

Acknowledgments

The author thanks Dr Jaroslav Stark for his constructive remarks in reviewing this manuscript.

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