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Ann Thorac Surg 2000;69:S70-S76
© 2000 The Society of Thoracic Surgeons

Congenital Heart Surgery Nomenclature and Database Project: systemic venous anomalies

^a Division of Pediatric Cardiothoracic Surgery, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
^b Division of Pediatric Cardiology, Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

Address reprint requests to Dr Gaynor, Division of Pediatric Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, 34th & Civic Center Blvd, Suite 8527, Philadelphia, PA 19104
e-mail: gaynor{at}email.chop.edu

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

Abstract

The extant nomenclature for systemic venous anomalies 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. This nomenclature system classifies systemic venous anomalies into two primary groups by venous segment: (1) systemic venous anomalies, superior vena cava; and (2) systemic venous anomalies, inferior vena cava. Subsets are clearly defined and categorized. 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

Anomalies of the systemic venous channels that drain to the heart may occur in isolation or in association with other congenital heart defects [1–9]. Many of the common defects are of little clinical significance when found in isolation, and there are many rare anomalies, which may or may not be clinically significant. Systemic venous anomalies are, however, of great importance in patients with various forms of functional single ventricle and may complicate creation of a superior cavopulmonary connection as well as the later Fontan operation. The presence of a systemic venous anomaly may necessitate modification of the implantation techniques for heart and combined heart/lung transplantation [10]. The various anomalies may also complicate cannulation for cardiopulmonary bypass even if repair of the venous anomaly itself is not required. However, this diagnostic category will be utilized primarily as a modifier or secondary diagnosis, particularly in patients with single ventricle.

The classification of these anomalies is primarily descriptive. Previous authors have classified anomalies of the systemic veins in several different ways. Some authors grouped the anomalies according to embryological origin [1, 2]. Others grouped the anomalies according to the venous segment (ie, superior vena cava [SVC] vs inferior vena cava [IVC]) or according to the connection of the heart (ie, systemic venous atrium vs pulmonary venous atrium) [3, 4]. For the purposes of this chapter, the anomalies will be classified into two primary groups by venous segment: 1) systemic venous anomalies, SVC; and 2) systemic venous anomalies, IVC.

The primary difficulty in classifying these anomalies arises from description of the atria. In the normal heart (situs solitus), the SVC and IVC connect to a right-sided atrium, which is morphologically a right atrium as defined by the presence of a large pyramidal appendage, septum secundum (superior limbic band) covering attachments of septum primum (flap valve of foramen ovale), and usually the orifice of the inferior vena cava. The pulmonary veins drain to a left-sided atrium, which is morphologically a left atrium as defined by a small finger-like appendage and absence of a crista terminalis. Difficulties arise in patients with either situs inversus, where the left-sided atrium is morphologically a right atrium, or with atrial isomerism (heterotaxy syndrome, either asplenia or polysplenia), where there may be either bilateral right or bilateral left atrial appendages [1–8]. Certain types of venous anomalies are more common in patients with atrial isomerism. Persistent left SVC and bilateral SVC may be seen in both right and left atrial isomerism [8]. Interruption of the IVC with azygos continuation is commonly seen in patients with left atrial isomerism [8]. For the purposes of this discussion, the term "right-sided atrium" refers to location, not morphology, and does not imply a morphologic right atrium; similarly, the term "left-sided atrium" does not imply left atrial morphology. The hierarchical structure, therefore, will provide classification of the systemic venous anatomy but does not include consideration of segmental anatomy or the presence of atrial isomerism. It will be necessary to specify the segmental anatomy and situs elsewhere in the database.

II. Analysis: a unified systemic venous anomalies nomenclature system

Definitions
Superior vena cava (SVC)
A venous trunk that drains blood from the head, neck, and upper extremities. It is usually formed by the two brachiocephalic veins and normally empties into the morphologic right atrium.

Inferior vena cava (IVC)
A venous trunk that drains the lower extremities, pelvis, and abdomen. It is formed by the common iliac veins, passes upward, and normally empties into the inferior aspect of the morphologic right atrium.

Brachiocephalic veins
Veins that drain blood from the head, neck, and upper extremities. Each is formed by the joining of the internal jugular and subclavian veins. Normally, the two brachiocephalic veins combine to form the SVC. The left brachiocephalic vein is frequently termed the innominate vein.

Azygos vein
A vein draining the right intercostal veins that arises from the lumbar vein and empties into the SVC. It may serve as a connecting branch between the venous beds drained by the SVC and IVC. A similar vein may be present in the left chest.

Coronary sinus (CS)
The terminal portion of the great cardiac vein that lies in the atrioventricular groove and normally empties into the morphologic right atrium.

Levo-atrial cardinal vein
An anomalous pulmonary to systemic venous connection allowing drainage of pulmonary venous blood to a systemic vein in the setting of normal pulmonary venous connection to the left atrium. It usually occurs in conjunction with left atrial outlet obstructive lesions but can also be seen with cor triatriatum. It arises from the left atrium or more commonly from a pulmonary vein and drains to the SVC or innominate vein.

Cor triatriatum Dexter
Presence of prominent venous valve producing obstruction of the IVC and tricuspid valve (failure of involution of the eustachian valve)

Hierarchical classification

Anomalies of the SVC are shown in Figure 1. The hierarchical scheme that follows allows classification of all of the common venous anomalies and many of the rare anomalies but does not include all theoretical possibilities. Some rare anomalies must be classified as other.

View larger version (24K): [in this window] [in a new window]   Fig 1. Anomalies of SVC. (Top) (Left) RSVC to right-sided atrium; (center) RSVC to CS to left-sided atrium; (right) RSVC to left-sided atrium. (Middle) (Left) LSVC to left-sided atrium (completely unroofed CS); (center) LSVC to right-sided atrium via CS; (right) LSVC to right-sided atrium. (Bottom) (Left) Bilateral SVC, RSVC to right-sided atrium, LSVC to left-sided atrium (completely unroofed CS); (center) bilateral SVC, RSVC to right-sided atrium, LSVC to CS to right-sided atrium; (right) bilateral SVC, RSVC to CS to left-sided atrium, LSVC to left-sided atrium.

Figure 2 details anomalies of the IVC. The most common anomaly of the IVC is interruption of the IVC with azygos continuation to the SVC [8]. As with persistent LSVC, in isolation, this anomaly produces no physiological abnormality and does not require correction. The hepatic veins normally join the IVC before its entry into the atrium; however, they may enter the heart separately. Rarely, the IVC may drain to the left atrium (LA) [18]. Figure 3 details possible anomalies of the hepatic veins.

View larger version (32K): [in this window] [in a new window]   Fig 2. Anomalies of IVC. (Top) (Left) RIVC to right-sided atrium; (center) LIVC to left-sided atrium; (right) IVC to right-sided atrium and left-sided atrium (biatrial drainage). (Middle) (Left) LIVC to right-sided atrium; (right) RIVC to left-sided atrium. (Bottom) (Left) interrupted RIVC with azygos continuation to RSVC; (left center) interrupted LIVC with azygos continuation to LSVC; (right center) interrupted RIVC with azygos continuation to LSVC; (right) interrupted LIVC with azygos continuation to RSVC.

View larger version (40K): [in this window] [in a new window]   Fig 3. Anomalies of hepatic veins. (Top) (Left) RIVC, separate entry of hepatic veins to left-sided atrium; (center) RIVC, separate entry of hepatic veins to right-sided atrium; (right) RIVC, separate entry of hepatic veins to CS. (Middle) (Left) LIVC, separate entry of hepatic veins to right-sided atrium; (center) LIVC, separate entry of hepatic veins to left-sided atrium; (right) LIVC, separate entry of hepatic veins to CS. (Bottom) (Left) Interrupted RIVC with azygos continuation to RSVC, separate entry of hepatic veins to right-sided atrium; (left center) interrupted RIVC with azygos continuation to RSVC, separate entry of hepatic veins to left-sided atrium; (right center) interrupted LIVC with azygos continuation to LSVC, separate entry of hepatic veins to left-sided atrium; (right) interrupted LIVC with azygos continuation to LSVC, separate entry of hepatic veins to right-sided atrium.

Systemic venous anomalies hierarchy level 1

Systemic venous anomaly

Systemic venous anomalies hierarchy level 2

Systemic venous anomaly, Not otherwise specified (NOS)

Systemic venous anomaly, SVC

Systemic venous anomaly, IVC

Systemic venous anomalies hierarchy level 3

Systemic venous anomaly, NOS

Systemic venous anomaly, SVC, NOS

Systemic venous anomaly, SVC, Abnormal RSVC

Systemic venous anomaly, SVC, Absent RSVC

Systemic venous anomaly, SVC, Bilateral SVC

Systemic venous anomaly, SVC, SVC stenosis

Systemic venous anomaly, SVC, SVC occlusion

Systemic venous anomaly, SVC, Retro-aortic innominate vein

Systemic venous anomaly, SVC, Levo-atrial-cardinal vein

Systemic venous anomaly, SVC, CS ostial atresia or stenosis (CS draining cephalad via LSVC)

Systemic venous anomaly, SVC, Other (specify)

Systemic venous anomaly, IVC, NOS

Systemic venous anomaly, IVC, Abnormal RIVC

Systemic venous anomaly, IVC, Separate entry of hepatic veins (RIVC to right-sided atrium)

Systemic venous anomaly, IVC, LIVC

Systemic venous anomaly, IVC, Biatrial drainage of IVC

Systemic venous anomaly, IVC, IVC stenosis

Systemic venous anomaly, IVC, IVC occlusion

Systemic venous anomaly, IVC, Cor triatriatum dexter

Systemic venous anomaly, IVC, Other (specify)

Systemic venous anomalies hierarchy level 4

Systemic venous anomaly, NOS

Systemic venous anomaly, SVC, NOS

Systemic venous anomaly, SVC, Abnormal RSVC, NOS

Systemic venous anomaly, SVC, Abnormal RSVC, RSVC to CS to left-sided atrium

Systemic venous anomaly, SVC, Abnormal RSVC, RSVC to left-sided atrium

Systemic venous anomaly, SVC, Absent RSVC, NOS

Systemic venous anomaly, SVC, Absent RSVC, LSVC to CS (intact) to right-sided atrium

Systemic venous anomaly, SVC, Absent RSVC, LSVC to CS (partially unroofed)

Systemic venous anomaly, SVC, Absent RSVC, LSVC to left-sided atrium (completely unroofed CS)

Systemic venous anomaly, SVC, Absent RSVC, LSVC to right-sided atrium

Systemic venous anomaly, SVC, Bilateral SVC, NOS

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to CS (intact) to right-sided atrium

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to CS (partially unroofed)

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to left-sided atrium (completely unroofed CS)

Systemic venous anomaly, SVC, Bilateral SVC, RSVC to CS to left-sided atrium

Systemic venous anomaly, SVC, SVC Stenosis, NOS

Systemic venous anomaly, SVC, SVC Stenosis, Acquired

Systemic venous anomaly, SVC, SVC Stenosis, Congenital

Systemic venous anomaly, SVC, SVC Occlusion, NOS

Systemic venous anomaly, SVC, SVC Occlusion, Acquired

Systemic venous anomaly, SVC, SVC Occlusion, Congenital

Systemic venous anomaly, SVC, Retro-Aortic innominate vein

Systemic venous anomaly, SVC, Levoatrial-Cardinal vein

Systemic venous anomaly, SVC, CS Ostial atresia or stenosis (CS draining cephalad via LSVC)

Systemic venous anomaly, SVC, other (Specify)

Systemic venous anomaly, IVC, NOS

Systemic venous anomaly, IVC, Abnormal RIVC, NOS

Systemic venous anomaly, IVC, Abnormal RIVC, Interrupted RIVC, Azygos continuation to RSVC

Systemic venous anomaly, IVC, Abnormal RIVC, Interrupted RIVC, Azygos continuation to LSVC

Systemic venous anomaly, IVC, Abnormal RIVC, RIVC to LA

Systemic venous anomaly, IVC, Separate entry of hepatic veins (RIVC to right-sided atrium), NOS

Systemic venous anomaly, IVC, Separate entry of hepatic veins (RIVC to right-sided atrium), Hepatic veins to right-sided atrium

Systemic venous anomaly, IVC, Separate entry of hepatic veins (RIVC to right-sided atrium), Hepatic veins to left-sided atrium

Systemic venous anomaly, IVC, Separate entry of hepatic veins (RIVC to right-sided atrium), Hepatic veins to CS

Systemic venous anomaly, IVC, LIVC, NOS

Systemic venous anomaly, IVC, LIVC, Interrupted LIVC, Azygos continuation to LSVC

Systemic venous anomaly, IVC, LIVC, Interrupted LIVC, Azygos continuation to RSVC

Systemic venous anomaly, IVC, LIVC, LIVC to right-sided atrium

Systemic venous anomaly, IVC, LIVC, LIVC to left-sided atrium

Systemic venous anomaly, IVC, Bi-atrial drainage of IVC

Systemic venous anomaly, IVC, IVC Stenosis, NOS

Systemic venous anomaly, IVC, IVC stenosis, Acquired

Systemic venous anomaly, IVC, IVC Stenosis, Congenital

Systemic venous anomaly, IVC, IVC Occlusion, NOS

Systemic venous anomaly, IVC, IVC Occlusion Acquired

Systemic venous anomaly, IVC, IVC Occlusion Congenital

Systemic venous anomaly, IVC, Cor Triatriatum Dexter

Systemic venous anomaly, IVC, Other (Specify)

Systemic venous anomalies: hierarchy level 5

Systemic venous anomaly, NOS

Systemic venous anomaly, SVC, NOS

Systemic venous anomaly, SVC, Abnormal RSVC, NOS

Systemic venous anomaly, SVC, Abnormal RSVC, RSVC to CS to left-sided atrium

Systemic venous anomaly, SVC, Abnormal RSVC, RSVC to left-sided atrium

Systemic venous anomaly, SVC, Absent RSVC, NOS

Systemic venous anomaly, SVC, Absent RSVC, LSVC to CS (intact) to right-sided atrium

Systemic venous anomaly, SVC, Absent RSVC, LSVC to CS (partially unroofed)

Systemic venous anomaly, SVC, Absent RSVC, LSVC to left-sided atrium (completely unroofed CS)

Systemic venous anomaly, SVC, Absent RSVC, LSVC to right-sided atrium

Systemic venous anomaly, SVC, Bilateral SVC, NOS

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to CS (intact) to right-sided atrium, NOS

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to CS (intact) to right-sided atrium, Innominate present

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to CS (intact) to right-sided atrium, Innominate absent

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to CS (partially unroofed), NOS

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to CS (partially unroofed), Innominate present

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to CS (partially unroofed), Innominate absent

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to left-sided atrium (completely unroofed CS), NOS

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to left-sided atrium (completely unroofed CS), Innominate present

Systemic venous anomaly, SVC, Bilateral SVC, LSVC to left-sided atrium (completely unroofed CS), Innominate absent

Systemic venous anomaly, SVC, Bilateral SVC, RSVC to CS to left-sided atrium, NOS

Systemic venous anomaly, SVC, Bilateral SVC, RSVC to CS to left-sided atrium, Innominate present

Systemic venous anomaly, SVC, Bilateral SVC, RSVC to CS to left-sided atrium, Innominate absent

Systemic venous anomaly, SVC, SVC stenosis, NOS

Systemic venous anomaly, SVC, SVC stenosis, Acquired

Systemic venous anomaly, SVC, SVC stenosis, Congenital

Systemic venous anomaly, SVC, SVC occlusion, NOS

Systemic venous anomaly, SVC, SVC occlusion, Acquired

Systemic venous anomaly, SVC, SVC occlusion, Congenital

Systemic venous anomaly, SVC, retro-aortic Innominate vein

Systemic venous anomaly, SVC, Levoatrial-cardinal vein

Systemic venous anomaly, SVC, CS ostial atresia or stenosis (CS draining cephalad via LSVC)

Systemic venous anomaly, SVC, Other (specify)

Systemic venous anomaly, IVC, NOS

Systemic venous anomaly, IVC, Abnormal RIVC, NOS

Systemic venous anomaly, IVC, Abnormal RIVC, Interrupted RIVC, Azygos continuation to RSVC, NOS

Systemic venous anomaly, IVC, Abnormal RIVC, Interrupted RIVC, Azygos continuation to RSVC, Hepatic veins to right-sided atrium

Systemic venous anomaly, IVC, Abnormal RIVC, Interrupted RIVC, Azygos continuation to RSVC, Hepatic veins to left-sided atrium

Systemic venous anomaly, IVC, Abnormal RIVC, Interrupted RIVC, Azygos continuation to RSVC, Hepatic veins to CS

Systemic venous anomaly, IVC, Abnormal RIVC, Interrupted RIVC, Azygos continuation to LSVC, NOS

Systemic venous anomaly, IVC, Abnormal RIVC, Interrupted RIVC, Azygos continuation to LSVC, Hepatic veins to right-sided atrium

Systemic venous anomaly, IVC, Abnormal RIVC, Interrupted RIVC, Azygos continuation to LSVC, Hepatic veins to left-sided atrium

Systemic venous anomaly, IVC, Abnormal RIVC, Interrupted RIVC, Azygos continuation to LSVC, Hepatic veins to CS

Systemic venous anomaly, IVC, Abnormal RIVC, RIVC to LA, NOS

Systemic venous anomaly, IVC, Abnormal RIVC, RIVC to LA, Hepatic veins to right-sided atrium

Systemic venous anomaly, IVC, Abnormal RIVC, RIVC to LA, Hepatic veins to left-sided atrium

Systemic venous anomaly, IVC, Abnormal RIVC, RIVC to LA, Hepatic veins to CS

Systemic venous anomaly, IVC, Separate entry of hepatic veins (RIVC to right-sided atrium), NOS

Systemic venous anomaly, IVC, Separate entry of hepatic veins (RIVC to right-sided atrium), Hepatic veins to right-sided atrium

Systemic venous anomaly, IVC, Separate entry of hepatic veins (RIVC to right-sided atrium), Hepatic veins to left-sided atrium

Systemic venous anomaly, IVC, Separate entry of hepatic veins (RIVC to right-sided atrium), Hepatic veins to CS

Systemic venous anomaly, IVC, LIVC, NOS

Systemic venous anomaly, IVC, LIVC, Interrupted LIVC, Azygos continuation to LSVC, NOS

Systemic venous anomaly, IVC, LIVC, Interrupted LIVC, Azygos continuation to LSVC, Hepatic veins to right-sided atrium

Systemic venous anomaly, IVC, LIVC, Interrupted LIVC, Azygos continuation to LSVC, Hepatic veins to left-sided atrium

Systemic venous anomaly, IVC, LIVC, Interrupted LIVC, Azygos continuation to LSVC, Hepatic veins to CS

Systemic venous anomaly, IVC, LIVC, Interrupted LIVC, Azygos continuation to RSVC, NOS

Systemic venous anomaly, IVC, LIVC, Interrupted LIVC, Azygos continuation to RSVC, Hepatic veins to right-sided atrium

Systemic venous anomaly, IVC, LIVC, Interrupted LIVC, Azygos continuation to RSVC, Hepatic veins to left-sided atrium

Systemic venous anomaly, IVC, LIVC, Interrupted LIVC, Azygos continuation to RSVC, Hepatic veins to CS

Systemic venous anomaly, IVC, LIVC, LIVC to right-sided atrium, NOS

Systemic venous anomaly, IVC, LIVC, LIVC to right-sided atrium, Hepatic veins to right-sided atrium

Systemic venous anomaly, IVC, LIVC, LIVC to right-sided atrium, Hepatic veins to left-sided atrium

Systemic venous anomaly, IVC, LIVC, LIVC to right-sided atrium, Hepatic veins to CS

Systemic venous anomaly, IVC, LIVC, LIVC to left-sided atrium, NOS

Systemic venous anomaly, IVC, LIVC, LIVC to left-sided atrium, Hepatic veins to right-sided atrium

Systemic venous anomaly, IVC, LIVC, LIVC to left-sided atrium, Hepatic veins to left-sided atrium

Systemic venous anomaly, IVC, LIVC, LIVC to left-sided atrium, Hepatic veins to CS

Systemic venous anomaly, IVC, Biatrial drainage of IVC

Systemic venous anomaly, IVC, IVC stenosis, NOS

Systemic venous anomaly, IVC, IVC stenosis, Acquired

Systemic venous anomaly, IVC, IVC stenosis, Congenital

Systemic venous anomaly, IVC, IVC occlusion, NOS

Systemic venous anomaly, IVC, IVC occlusion, Acquired

Systemic venous anomaly, IVC, IVC occlusion, Congenital

Systemic venous anomaly, IVC, Cor triatriatum Dexter

Systemic venous anomaly, IVC, Other (specify)

III. Nomenclature for systemic venous anomalies treatment options

Most of the isolated systemic venous anomalies that require repair are rare. The most common defect that requires repair is LSVC to LA or a partially unroofed CS [11, 13]. The hierarchical classification for surgical procedures in isolated venous anomalies is below. However, presence of a venous anomaly may significantly alter the technique for superior cavopulmonary connection or Fontan procedure. These procedures should be coded separately.

Systemic venous anomalies treatment hierarchy level 1

Systemic venous anomaly procedure

Systemic venous anomalies treatment hierarchy level 2

Systemic venous anomaly procedure, NOS

Systemic venous anomaly procedure, Ligation of LSVC

Systemic venous anomaly procedure, Atrial baffle (LSVC to right-sided atrium)

Systemic venous anomaly procedure, Roofing of CS

Systemic venous anomaly procedure, Reimplantation of LSVC to right-sided atrium

Systemic venous anomaly procedure, Reimplantation of LSVC to RSVC

Systemic venous anomaly procedure, Bidirectional Glenn shunt (LSVC to LPA)

Systemic venous anomaly procedure, Bidirectional Glenn shunt (RSVC to RPA)

Systemic venous anomaly procedure, Repair of SVC stenosis

Systemic venous anomaly procedure, Repair of IVC stenosis

Systemic venous anomaly procedure, Other (specify)

Systemic venous anomalies treatment hierarchy level 3

Systemic venous anomaly procedure, NOS

Systemic venous anomaly procedure, Ligation of LSVC

Systemic venous anomaly procedure, Atrial baffle (LSVC to right-sided atrium)

Systemic venous anomaly procedure, Roofing of CS

Systemic venous anomaly procedure, Reimplantation of LSVC to right-sided atrium, NOS

Systemic venous anomaly procedure, Reimplantation of LSVC to right-sided atrium, Direct

Systemic venous anomaly procedure, Reimplantation of LSVC to right-sided atrium, Conduit

Systemic venous anomaly procedure, Reimplantation of LSVC to RSVC, NOS

Systemic venous anomaly procedure, Reimplantation of LSVC to RSVC, Direct

Systemic venous anomaly procedure, Reimplantation of LSVC to RSVC, Conduit

Systemic venous anomaly procedure, Bidirectional Glenn shunt (LSVC to LPA)

Systemic venous anomaly procedure, Bidirectional Glenn shunt (RSVC to RPA)

Systemic venous anomaly procedure, Repair of SVC stenosis, NOS

Systemic venous anomaly procedure, Repair of SVC stenosis, Patch

Systemic venous anomaly procedure, Repair of SVC stenosis, Conduit

Systemic venous anomaly procedure, Repair of SVC stenosis, Reimplantation

Systemic venous anomaly procedure, Repair of IVC stenosis, NOS

Systemic venous anomaly procedure, Repair of IVC stenosis, Patch

Systemic venous anomaly procedure, Repair of IVC stenosis, Excision of membrane

Systemic venous anomaly procedure, Repair of IVC stenosis, Conduit

Systemic venous anomaly procedure, Repair of IVC stenosis, Reimplantation

Systemic venous anomaly procedure, Other (specify)

IV. Diagnosis and procedure short lists

Diagnosis Short List

Systemic venous anomaly

Systemic venous obstruction

Procedure Short List

Atrial baffle procedure (non-Mustard, non-Senning)

Anomalous systemic venous connection repair

Systemic venous stenosis repair

V. Database studies and outcome analysis

In addition to the diagnosis and procedure classification, the minimal data set for these anomalies should include the patient and procedural variables that have been identified as essential data fields. Because of the rarity of isolated systemic venous anomalies that require surgical correction, it would be impractical to generate detailed outcome reports for each anomaly. However, it will be important to assess the impact of systemic venous anomalies on morbidity and mortality after superior cavopulmonary connection of Fontan procedure.

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