Ann Thorac Surg 2000;69:S147-S163
© 2000 The Society of Thoracic Surgeons
Congenital Heart Surgery Nomenclature and Database Project: aortic aneurysm, sinus of valsalva aneurysm, and aortic dissection
W. Steves Ring, MDa
a Division of Thoracic and Cardiovascular Surgery, Children’s Medical Center of Dallas, University of Texas Southwestern Medical Center, Dallas, Texas, USA
Address reprint requests to Dr Ring, Division of Thoracic and Cardiovascular Surgery, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75235-8879
e-mail: sring{at}mednet.swmed.edu
Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.
Abstract
The extant nomenclature for aortic aneurysms, sinus of valsalva aneurysms, and aortic dissections 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. Classification was based on morphology, histology, anatomic location, etiology, and acuity. 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 that would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.
I. Background
An aneurysm of the aorta is defined as a localized dilation or enlargement of the aorta. It can result from a wide variety of aortic pathology and involve any or all segments of the aorta from the aortic annulus to the aortoiliac bifurcation. Aortic aneurysms have been classified on the basis of morphology, histology, anatomic location, and etiology [1]. Morphologically, aortic aneurysms are classified as either fusiform or saccular. Histologically, they may be classified as true aneurysms involving all layers of the aorta, dissecting aneurysms splitting the layers of the aorta (usually medial separation), or false aneurysms contained only by the adventitia and surrounding connective or fibrous tissue. Anatomically, they may involve the sinus of Valsalva, aortic root, ascending aorta, aortic arch, descending aorta, thoracoabdominal aorta, or the abdominal aorta. Finally, aortic aneurysms can have numerous etiologies including congenital, degenerative, atherosclerotic, inflammatory, mycotic, traumatic, or iatrogenic. Aneurysms are generally considered significant if the diameter exceeds twice the diameter of the normal aorta or is more than two standard deviations greater than normal [1]. This level of significance is based on the natural history of most aortic aneurysms to expand and rupture according to the law of Laplace. Important predictors of survival include absolute diameter, shape, location, rate of expansion, and clinical symptoms [2], based on several natural history studies [3–6].
Aneurysms of the thoracic aorta (including dissections) are reported to occur with an incidence of 5.9 cases per 100,000 of population [4], most often in adults and more commonly in older age groups. They are most often caused by cystic medial degeneration or atherosclerosis, and frequently associated with hypertension. Other etiologies include aortitis (syphilitic, giant cell, granulomatous), trauma (acute or chronic transection), primary pyogenic infection (mycotic), and iatrogenic injury (false or pseudoaneurysm). Approximately 50% of thoracic aortic aneurysms involve the ascending aorta, while 40% involve the descending aorta, and 10% involve the aortic arch [4]. In adults, approximately 90% of descending aortic aneurysms are caused by atherosclerosis, while ascending aortic and arch aneurysms are more commonly due to cystic medial degeneration. In contrast to adults, thoracic aneurysms in children are quite rare and generally related to congenital abnormalities, inflammatory disease, infection, trauma, iatrogenic injury, or hereditary connective tissue disease (Marfan’s, Ehler-Danlos, etc) [7, 8]. Three of the aneurysm types more commonly encountered in children are discussed individually and in more detail.
Sinus of valsalva aneurysm
The sinus of Valsalva is defined as that portion of the aortic root between the aortic valve annulus and the sinotubular ridge. Aneurysmal dilation with rupture of the sinus of Valsalva was first described in 1939 by Hope [9]. It is a rare condition occurring in only 0.09% of a large older autopsy series [10], but in 0.14% to 0.23% of Western surgical series [11, 12] and 0.46% to 3.5% of surgical series from the Orient [13, 14]. In 1919, Abbott [15] postulated that these defects are congenital in origin, caused by a developmental defect in the distal bulbar septum. This was later confirmed by the histologic studies of Edwards and Burchell [16], demonstrating discontinuity between the aortic media and the annulus fibrosis. A congenital etiology is also supported by the frequent association of sinus of Valsalva aneurysms with ventricular septal defects [17]. However, it is clear that other disease processes involving the aortic root and sinuses can also be associated with aneurysms of the sinus of Valsalva. These include syphilis, endocarditis, cystic medial necrosis, atherosclerosis, and trauma. They more frequently involve multiple sinuses of Valsalva and are therefore more appropriately classified as aneurysms of the aortic root.
The only formal classification system for sinus of Valsalva aneurysms is that originally proposed by Sakakibara and Konno in 1962 [17, 18]. This anatomic classification describes only four types of congenital sinus of Valsalva aneurysms arising from either the right sinus or the anterior noncoronary sinus, and does not account for all the possible chambers of penetration. Most recent authors have chosen to classify them more descriptively in terms of the sinus of origin and the chamber of termination [19, 20]. From a surgical aspect, the most useful classification system includes both clinical (acquired vs congenital, ruptured vs nonruptured) and anatomic (sinus of origin, chamber of penetration) descriptors.
A congenital sinus of Valsalva aneurysm is a dilation usually of a single sinus of Valsalva caused by a separation between the aortic media and the annulus fibrosus, and often with a deficiency of the normal elastic tissue and abnormal development of the bulbus cordis. These most commonly originate from the right sinus (65% to 85%), less commonly from the noncoronary sinus (10% to 30%), and rarely from the left sinus (< 5%) [19–23]. Aneurysmal dilation involving multiple sinuses is best considered as aneurysmal dilation of the aortic root (eg, Marfans) because both the clinical presentation and the surgical management are usually quite different. A true sinus of Valsalva aneurysm presents above the aortic annulus. It must be distinguished from a prolapsing aortic cusp that occurs below the annulus, although both are commonly associated with a ventricular septal defect.
Associated conditions include ventricular septal defects in 30% to 60% of patients, aortic insufficiency in 20% to 30% of patients, bicuspid aortic valve in about 10% of patients, and less commonly pulmonary stenosis, coarctation, and atrial septal defect. Associated conditions are best classified and coded separately (eg, ventricular septal defect, aortic insufficiency, bicuspid aortic valve, etc). Congenital aneurysms have a 4:1 male predominance. The racial predominance in Far Eastern countries correlates with the higher incidence of supracrystal ventricular septal defects noted in Asians and the higher incidence of ventricular septal defects associated with sinus of Valsalva aneurysms (about 60%) [13].
A nonruptured congenital sinus of Valsalva aneurysm may vary from a mild dilation of a single aortic sinus to an extensive windsock deformity. Although it is usually asymptomatic and discovered on routine echocardiogram, symptoms may occur from compression of the coronary arteries, obstruction of the right ventricular outflow tract, or from an associated defect such as a ventricular septal defect or aortic insufficiency. Rupture of a congenital sinus of Valsalva aneurysm into an adjacent chamber occurs most commonly between the ages of 15 and 30 years. It may occur spontaneously, after trauma, after strenuous physical exertion, or from acute bacterial endocarditis. The resulting intracardiac fistula usually causes clinical decompensation due to congestive heart failure. It may rarely cause free perforation into the pericardium, a condition that is usually fatal.
Aortic dissection
Aortic dissection is a separation of the layers of the aortic wall with variable proximal and distal extension. Although not clearly defined, it is thought to be caused either by abnormal connective tissues within the aortic wall (congenital or acquired disorders with medial degeneration), by excessive shear stresses within the intima and media from acute or chronic hypertension (hypertension, weight lifting, pregnancy), or by trauma (surgical or external). In over 95% of cases, it begins as a transverse tear through the intima and inner layers of the media, with only 2% to 4% not originating from an intimal tear alone. The plane of the dissection then extends within the aortic wall both longitudinally and circumferentially, propagated by the energy of the pulsatile aortic pressure waveform. Extension of the plane of the dissection may cause occlusion of major side branches in up to 30% of cases (coronary, carotid, subclavian, intercostal, renal, visceral, or femoral arteries) with corresponding ischemic syndromes. Extension may also progress to free rupture into the pericardium, mediastinum, or pleural space if not contained by the outer layers of the media and adventitia. This may present as either pericardial tamponade or hemothorax. Aortic dissection is rarely seen in pediatric patients except in those with an underlying connective tissue abnormality such as Marfan’s or Ehler-Danlos syndromes, iatrogenic injury, or in association with Turner’s syndrome, aortic coarctation, or congenitally bicuspid aortic stenosis. In adults, approximately two-thirds originate from a tear in the ascending aorta most commonly just above the sinotubular ridge. Another 20% originate in the descending aorta usually just distal to the left subclavian artery, with only 10% originating within the transverse aortic arch. Only isolated cases of aortic dissection in children have been reported, mostly over the age of 10 years.
Several classification systems for aortic dissection have been developed based on anatomic considerations, including the site of the tear and the extent of aortic involvement. DeBakey and associates [23] proposed the first classification for aortic dissection in 1965 based on both the site of the tear and the extent of the dissection. According to this classification system, type I aortic dissection involves both the ascending aorta and arch, type II is confined to the ascending aorta, while type III begins in the descending aorta. The Stanford system [24] classifies all dissections involving the ascending aorta as type A regardless of the site of the original tear. Those involving only the descending aorta are considered type B. Other authors classify aortic dissection simply as proximal or distal [1, 25, 26]. Clinically, aortic dissections are usually classified as either acute or chronic based on time from the onset of symptoms. This distinction has significant clinical relevance based on natural history studies of medically treated patients showing a 2-week mortality rate of 57% to 89% [27–29]. Based on these observations, it has become conventional to classify aortic dissections as acute during the first 2 weeks after the onset of symptoms and chronic beyond 2 weeks.
A complex classification system for arterial aneurysms in children has been proposed by Sarkar and associates [8]. This system is generally based on etiology and includes aneurysms of either the aorta or its major branch arteries but does not incorporate aneurysms of the sinus of Valsalva, dissecting aneurysms, or any distinction based on anatomic location. As noted previously, sinus of Valsalva aneurysms [17, 18], aortic dissections [23–26], and thoracoabdominal aneurysms [30] each have their own separate classifications. While each of these classification systems is widely utilized, none encompass the entire spectrum of aortic aneurysmal disease. A comprehensive diagnostic nomenclature for aortic aneurysmal disease should incorporate all factors of major clinical relevance to the surgical treatment and outcomes. Because anatomic location, acuity, etiology, and histology (pathology) have each been shown to significantly impact outcomes after aortic aneurysm surgery, any comprehensive diagnostic nomenclature system should include each of these factors in either a hierarchical or matrix classification system. Etiologies other than congenital have been included in the proposed nomenclature hierarchy because the typical pediatric congenital heart surgeon is likely to be faced with these cases within the scope of his/her practice.
Nomenclature for diagnosis
Hierarchy level 1
Aortic aneurysm is any dilation or enlargement of the aorta including all pathologies, all etiologies, and involving any location within the aorta (from the aortic annulus to the aortic bifurcation).
SVA is defined as a dilation or enlargement of one sinus of the aortic root between the aortic valve annulus and the sinotubular ridge. Aneurysms of the sinus of Valsalva involve only a single sinus. Those involving multiple sinuses are classified below as aneurysms of the aortic root.
Aortic dissection is defined as a partial tear of the aortic wall leading to a separation of the layers of the wall with variable proximal and distal extension.
Hierarchy level 2: location/anatomy
Aortic root aneurysm is defined as dilation of multiple sinuses of Valsalva with or without dilation of the annulus and sinotubular ridge (annuloaortic ectasia).
Ascending aorta is defined as that segment of the aorta extending from the sinotubular ridge to a line perpendicular to the proximal origin of the first arch vessel. Those involving both the ascending aorta and either the aortic root or arch (ie, multiple segments) should be coded for both diagnoses.
Aortic arch is defined as the segment of the aorta between the proximal origin of the first head branch (usually innominate or carotid artery) and a line from the distal origin of the last branch (usually the subclavian artery).
Descending aorta is defined as extending from the most distal subclavian artery to the diaphragm.
Abdominal aorta extends from the diaphragm to the aortic bifurcation, and the thoracoabdominal aorta involves both the descending thoracic aorta and some portion of the abdominal aorta.
Multiple segment involvement should list each segment involved as a separate diagnosis within the anatomic location hierarchy. For dissections, each segment can be listed with the principal segment (containing the tear) receiving the primary diagnosis and additional segments listed as secondary diagnoses. Proximal, DeBakey type I and II, or Stanford type A dissections are therefore classified as aortic root, ascending aortic, and/or transverse aortic arch dissections according to the site of the tear and the extent of aortic involvement.
Left sinus of Valsalva is defined as the aortic sinus normally giving rise to the left coronary artery.
Right sinus of Valsalva is defined as the aortic sinus normally giving rise to the right coronary artery.
Noncoronary sinus of Valsalva is defined as the aortic sinus from which no coronary artery normally arises, usually facing opposite the pulmonary artery.
Hierarchy level 3: acuity
Acute or ruptured aortic aneurysm defines the urgency status of the aneurysm. By convention, aortic dissections and transections are classified as acute if symptoms have been present for less than 2 weeks. True aneurysms are classified as ruptured if they are leaking or freely ruptured into the surrounding tissues, cavities, or chambers. These include sinus of Valsalva aneurysms that have ruptured or created a fistulous communication into one of the adjacent cardiac chambers.
Chronic or nonruptured aortic aneurysms include true aneurysms that are not leaking or ruptured, including intact sinus of Valsalva aneurysms. Aortic dissections and transections are classified as chronic if they have been present for more than 14 days.
Hierarchy level 4: pathology and chamber of penetration
True aortic aneurysm is defined as a dilated segment of the aorta involving all layers of the aortic wall, but specifically lined by intima. This includes all aortic aneurysms except those with dissection of the aortic wall or those with disruption of all layers of the aortic wall except the adventitia.
False aortic aneurysm (or pseudoaneurysm) is defined as a dilated segment of the aorta not containing all layers of the aortic wall. This designation includes postoperative or postprocedure false aneurysms (eg, at anastomotic sites), traumatic aortic injuries or transections, and infectious processes leading to a contained rupture.
Because the techniques used for repair of sinus of Valsalva aneurysms frequently involve approaching the aneurysm through both the aorta and through the chamber or structure into which the aneurysm penetrates, protrudes, or ruptures, it is useful to include this information in any hierarchical structure. Although rare, any sinus may rupture freely into the pericardium. However, anatomically, neither the left sinus nor the noncoronary sinus rupture into the pulmonary artery, nor does the right sinus rupture into the left atrium.
Significant modifiers relating to etiology
The etiology of the aneurysm or dissection provides useful prognostic information. However, its inclusion in the primary diagnostic hierarchy classification system would make it cumbersome and is better represented as a modifier table.
Atherosclerotic aortic aneurysms are confined almost entirely to the elderly adult population. These aneurysms result from a weakening or degeneration of the aortic media often associated with typical atheromas. They are not seen in the pediatric age group but are included in this classification system for completeness.
Congenital aortic aneurysms include any aneurysmal dilation of a segment of aorta related to a congenital defect in the aorta. This includes aneurysms of the sinuses of Valsalva with or without associated ventricular septal defects or aortic valve abnormalities, ascending aortic aneurysms associated with congenital aortic valve stenosis or insufficiency, and aneurysms of the descending aorta associated with coarctation, a ductus diverticulum, or a Kommerell’s diverticulum.
Connective tissue aortic aneurysms are among the more commonly encountered aortic aneurysms found in children. These include aneurysmal dilation of a segment of the aorta caused by cystic medial necrosis (Erdheim’s disease, annuloaortic ectasia), Marfan’s syndrome, Ehlers-Danlos syndrome, Turner’s syndrome, Noonan’s syndrome, Klippel-Feil syndrome, and pseudoxanthoma elasticum.
Infection aortic aneurysms include those aneurysms caused either primarily by infection (primary mycotic aneurysms, endocarditis), secondarily by weakening of the aortic wall from a periaortic abscess (tuberculosis, pancreatitis, etc), or syphilis (Leutic aortitis).
Inflammatory aortic aneurysms include those demonstrating some degree of inflammation within the aortic wall. This may result from autoimmune disorders (scleroderma, periarteritis nodosa, Reiter’s syndrome, lupus, ankylosing spondylitis, juvenile rheumatoid arthritis, etc), Takayasu’s aortitis, giant cell aortitis, Kawasaki’s disease, Beçhet’s disease, and others. The aortic valve and branch arteries may be involved with some of these processes. Any associated diagnoses are listed separately in addition to the appropriate classification of the aneurysm.
Traumatic aortic aneurysms include aneurysms resulting from penetrating, blunt, surgical, or procedural trauma. They tend to be more saccular in morphology and localized in their extent. Classification may become difficult in the patient who presents very late without a clear history of remote trauma, but with other findings compatible with a chronic transection.
II. Proposed diagnostic hierarchy: levels 1 to 4
Hierarchy level 1
- Aortic aneurysm
- Sinus of Valsalva aneurysm
- Aortic dissection
Hierarchy level 2: location
- Aortic aneurysm, Not otherwise specified (NOS)
- Aortic aneurysm, Aortic root
- Aortic aneurysm, Ascending aorta
- Aortic aneurysm, Transverse arch
- Aortic aneurysm, Descending aorta
- Aortic aneurysm, Thoracoabdominal aorta
- Aortic aneurysm, Abdominal aorta
- Sinus of Valsalva aneurysm, NOS
- Sinus of Valsalva aneurysm, Left sinus
- Sinus of Valsalva aneurysm, Right sinus
- Sinus of Valsalva aneurysm, Noncoronary sinus
- Aortic dissection, NOS
- Aortic dissection, Aortic root
- Aortic dissection, Ascending aorta
- Aortic dissection, Transverse arch
- Aortic dissection, Descending aorta
- Aortic dissection, Thoracoabdominal aorta
- Aortic dissection, Abdominal aorta
Hierarchy level 3: acuity
- Aortic aneurysm, NOS
- Aortic aneurysm, aortic root, NOS
- Aortic aneurysm, Aortic root, Ruptured
- Aortic aneurysm, Aortic root, Nonruptured
- Aortic aneurysm, Ascending aorta, NOS
- Aortic aneurysm, Ascending aorta, Ruptured
- Aortic aneurysm, Ascending aorta, Nonruptured
- Aortic aneurysm, Transverse arch, NOS
- Aortic aneurysm, Transverse arch, Ruptured
- Aortic aneurysm, Transverse arch, Nonruptured
- Aortic aneurysm, Descending aorta, NOS
- Aortic aneurysm, Descending aorta, Ruptured
- Aortic aneurysm, Descending aorta, Nonruptured
- Aortic aneurysm, Thoracoabdominal aorta, NOS
- Aortic aneurysm, Thoracoabdominal aorta, Ruptured
- Aortic aneurysm, Thoracoabdominal aorta, Nonruptured
- Aortic aneurysm, Abdominal aorta, NOS
- Aortic aneurysm, Abdominal aorta, Ruptured
- Aortic aneurysm, Abdominal aorta, Nonruptured
- Sinus of Valsalva aneurysm, NOS
- Sinus of Valsalva aneurysm, Left sinus, NOS
- Sinus of Valsalva aneurysm, Left sinus, Ruptured
- Sinus of Valsalva aneurysm, Left sinus, Nonruptured
- Sinus of Valsalva aneurysm, Right sinus, NOS
- Sinus of Valsalva aneurysm, Right sinus, Ruptured
- Sinus of Valsalva aneurysm, Right sinus, Nonruptured
- Sinus of Valsalva aneurysm, Noncoronary sinus, NOS
- Sinus of Valsalva aneurysm, Noncoronary sinus, Ruptured
- Sinus of Valsalva aneurysm, Noncoronary sinus, Nonruptured
- Aortic dissection, NOS
- Aortic dissection, Aortic root, NOS
- Aortic dissection, Aortic root, Acute
- Aortic dissection, Aortic root, Chronic
- Aortic dissection, Ascending aorta, NOS
- Aortic dissection, Ascending aorta, Acute
- Aortic dissection, Ascending aorta, Chronic
- Aortic dissection, Transverse arch, NOS
- Aortic dissection, Transverse arch, Acute
- Aortic dissection, Transverse arch, Chronic
- Aortic dissection, Descending aorta, NOS
- Aortic dissection, Descending aorta, Acute
- Aortic dissection, Descending aorta, Chronic
- Aortic dissection, Thoracoabdominal aorta, NOS
- Aortic dissection, Thoracoabdominal aorta, Acute
- Aortic dissection, Thoracoabdominal aorta, Chronic
- Aortic dissection, Abdominal aorta, NOS
- Aortic dissection, Abdominal aorta, Acute
- Aortic dissection, Abdominal aorta, Chronic
Hierarchy level 4: pathology and chamber of penetration
- Aortic aneurysm, NOS
- Aortic aneurysm, Aortic root, NOS
- Aortic aneurysm, Aortic root, Ruptured, NOS
- Aortic aneurysm, Aortic root, Ruptured, True aneurysm
- Aortic aneurysm, Aortic root, Ruptured, False aneurysm
- Aortic aneurysm, Aortic root, Nonruptured, NOS
- Aortic aneurysm, Aortic root, Nonruptured, True aneurysm
- Aortic aneurysm, Aortic root, Nonruptured, False aneurysm
- Aortic aneurysm, Ascending aorta, Ruptured, NOS
- Aortic aneurysm, Ascending aorta, Ruptured, True aneurysm
- Aortic aneurysm, Ascending aorta, Ruptured, False aneurysm
- Aortic aneurysm, Ascending aorta, Nonruptured, NOS
- Aortic aneurysm, Ascending aorta, Nonruptured, True aneurysm
- Aortic aneurysm, Ascending aorta, Nonruptured, False aneurysm
- Aortic aneurysm, Transverse arch, Ruptured, NOS
- Aortic aneurysm, Transverse arch, Ruptured, True aneurysm
- Aortic aneurysm, Transverse arch, Ruptured, False aneurysm
- Aortic aneurysm, Transverse arch, Nonruptured, NOS
- Aortic aneurysm, Transverse arch, Nonruptured, True aneurysm
- Aortic aneurysm, Transverse arch, Nonruptured, False aneurysm
- Aortic aneurysm, Descending aorta, Ruptured, NOS
- Aortic aneurysm, Descending aorta, Ruptured, True aneurysm
- Aortic aneurysm, Descending aorta, Ruptured, False aneurysm
- Aortic aneurysm, Descending aorta, Nonruptured, NOS
- Aortic aneurysm, Descending aorta, Nonruptured, True aneurysm
- Aortic aneurysm, Descending aorta, Nonruptured, False aneurysm
- Aortic aneurysm, Thoracoabdominal aorta, Ruptured, NOS
- Aortic aneurysm, Thoracoabdominal aorta, Ruptured, True aneurysm
- Aortic aneurysm, Thoracoabdominal aorta, Ruptured, False aneurysm
- Aortic aneurysm, Thoracoabdominal aorta, Nonruptured, NOS
- Aortic aneurysm, Thoracoabdominal aorta, Nonruptured, True aneurysm
- Aortic aneurysm, Thoracoabdominal aorta, Nonruptured, False aneurysm
- Aortic aneurysm, Abdominal aorta, Ruptured, NOS
- Aortic aneurysm, Abdominal aorta, Ruptured, True aneurysm
- Aortic aneurysm, Abdominal aorta, Ruptured, False aneurysm
- Aortic aneurysm, Abdominal aorta, Nonruptured, NOS
- Aortic aneurysm, Abdominal aorta, Nonruptured, True aneurysm
- Aortic aneurysm, Abdominal aorta, Nonruptured, False aneurysm
- Sinus of Valsalva aneurysm, NOS
- Sinus of Valsalva aneurysm, Left sinus, NOS
- Sinus of Valsalva aneurysm, Left sinus, Ruptured, NOS
- Sinus of Valsalva aneurysm, Left sinus, Ruptured, To right atrium
- Sinus of Valsalva aneurysm, Left sinus, Ruptured, To right ventricle
- Sinus of Valsalva aneurysm, Left sinus, Ruptured, To left atrium
- Sinus of Valsalva aneurysm, Left sinus, Ruptured, To left ventricle
- Sinus of Valsalva aneurysm, Left sinus, Ruptured, To pericardium
- Sinus of Valsalva aneurysm, Left sinus, Nonruptured, NOS
- Sinus of Valsalva aneurysm, Left sinus, Nonruptured, Protruding onto right atrium
- Sinus of Valsalva aneurysm, Left sinus, Nonruptured, Protruding onto right ventricle
- Sinus of Valsalva aneurysm, Left sinus, Nonruptured, Protruding onto left atrium
- Sinus of Valsalva aneurysm, Left sinus, Nonruptured, Protruding onto left ventricle
- Sinus of Valsalva aneurysm, Left sinus, Nonruptured, Protruding into pericardial cavity
- Sinus of Valsalva aneurysm, Right sinus, NOS
- Sinus of Valsalva aneurysm, Right sinus, Ruptured, NOS
- Sinus of Valsalva aneurysm, Right sinus, Ruptured, To right atrium
- Sinus of Valsalva aneurysm, Right sinus, Ruptured, To right ventricle
- Sinus of Valsalva aneurysm, Right sinus, Ruptured, To pulmonary artery
- Sinus of Valsalva aneurysm, Right sinus, Ruptured, To left ventricle
- Sinus of Valsalva aneurysm, Right sinus, Ruptured, To pericardium
- Sinus of Valsalva aneurysm, Right sinus, Nonruptured, NOS
- Sinus of Valsalva aneurysm, Right sinus, Nonruptured, Protruding onto right atrium
- Sinus of Valsalva aneurysm, Right sinus, Nonruptured, Protruding onto right ventricle
- Sinus of Valsalva aneurysm, Right sinus, Nonruptured, Protruding onto left ventricle
- Sinus of Valsalva aneurysm, Right sinus, Nonruptured, Protruding onto pulmonary artery
- Sinus of Valsalva aneurysm, Right sinus, Nonruptured, Protruding into pericardial cavity
- Sinus of Valsalva aneurysm, Noncoronary sinus, NOS
- Sinus of Valsalva aneurysm, Noncoronary sinus, Ruptured, NOS
- Sinus of Valsalva aneurysm, Noncoronary sinus, Ruptured, To right atrium
- Sinus of Valsalva aneurysm, Noncoronary sinus, Ruptured, To right ventricle
- Sinus of Valsalva aneurysm, Noncoronary sinus, Ruptured, To left atrium
- Sinus of Valsalva aneurysm, Noncoronary sinus, Ruptured, To left ventricle
- Sinus of Valsalva aneurysm, Noncoronary sinus, Ruptured, To pericardium
- Sinus of Valsalva aneurysm, Noncoronary sinus, Nonruptured, NOS
- Sinus of Valsalva aneurysm, Noncoronary, Nonruptured, Protruding onto right atrium
- Sinus of Valsalva aneurysm, Noncoronary, Nonruptured, Protruding onto right ventricle
- Sinus of Valsalva aneurysm, Noncoronary, Nonruptured, Protruding onto left atrium
- Sinus of Valsalva aneurysm, Noncoronary, Nonruptured, Protruding onto left ventricle
- Sinus of Valsalva aneurysm, Noncoronary, Nonruptured, Protruding into pericardial cavity
- Aortic dissection, NOS
- Aortic dissection, Aortic root, NOS
- Aortic dissection, Aortic root, Acute
- Aortic dissection, Aortic root, Chronic
- Aortic dissection, Ascending aorta, NOS
- Aortic dissection, Ascending aorta, Acute
- Aortic dissection, Ascending aorta, Chronic
- Aortic dissection, Transverse arch, NOS
- Aortic dissection, Transverse arch, Acute
- Aortic dissection, Transverse arch, Chronic
- Aortic dissection, Descending aorta, NOS
- Aortic dissection, Descending aorta, Acute
- Aortic dissection, Descending aorta, Chronic
- Aortic dissection, Thoracoabdominal aorta, NOS
- Aortic dissection, Thoracoabdominal aorta, Acute
- Aortic dissection, Thoracoabdominal aorta, Chronic
- Aortic dissection, Abdominal aorta, NOS
- Aortic dissection, Abdominal aorta, Acute
- Aortic dissection, Abdominal aorta, Chronic
Aortic aneurysm etiologic diagnostic modifiers
Aortic aneurysm etiologic diagnostic modifiers hierarchy level 1
- Aortic aneurysm etiology: atherosclerotic
- Aortic aneurysm etiology: congenital
- Aortic aneurysm etiology: connective tissue disease
- Aortic aneurysm etiology: infectious
- Aortic aneurysm etiology: inflammatory
- Aortic aneurysm etiology: traumatic
Aortic aneurysm etiologic diagnostic modifiers hierarchy level 2
- Aortic aneurysm etiology: atherosclerotic
- Aortic aneurysm etiology: congenital, NOS
- Aortic aneurysm etiology: congenital, Kommerell’s diverticulum
- Aortic aneurysm etiology: connective tissue disease, NOS
- Aortic aneurysm etiology: connective tissue disease, Cystic medial necrosis
- Aortic aneurysm etiology: connective tissue disease, Erdheim’s disease (annuloaortic ectasia)
- Aortic aneurysm etiology: connective tissue disease, Marfan syndrome
- Aortic aneurysm etiology: connective tissue disease, Ehlers-Danlos syndrome
- Aortic aneurysm etiology: connective tissue disease, Turner’s syndrome
- Aortic aneurysm etiology: connective tissue disease, Noonan’s syndrome
- Aortic aneurysm etiology: connective tissue disease, Klipper-Feil syndrome
- Aortic aneurysm etiology: connective tissue disease, pseudoxanthoma elasticum
- Aortic aneurysm etiology: connective tissue disease, other
- Aortic aneurysm etiology: infectious, NOS
- Aortic aneurysm etiology: infectious, Primary mycotic aneurysm
- Aortic aneurysm etiology: infectious, Endocarditis
- Aortic aneurysm etiology: infectious, Secondary: tuberculous mycotic aneurysm
- Aortic aneurysm etiology: infectious, Secondary: pancreatitis
- Aortic aneurysm etiology: infectious, Secondary: other
- Aortic aneurysm etiology: infectious, Syphilitic aneurysm (Leutic aortitis)
- Aortic aneurysm etiology: inflammatory, NOS
- Aortic aneurysm etiology: inflammatory, Autoimmune disorders
- Aortic aneurysm etiology: inflammatory, Takayasu’s aortitis
- Aortic aneurysm etiology: inflammatory, Giant cell aortitis
- Aortic aneurysm etiology: inflammatory, Kawasaki disease
- Aortic aneurysm etiology: inflammatory, Beçhet’s disease
- Aortic aneurysm etiology: inflammatory, Other
- Aortic aneurysm etiology: traumatic, NOS
- Aortic aneurysm etiology: traumatic, Penetrating trauma
- Aortic aneurysm etiology: traumatic, Blunt trauma
- Aortic aneurysm etiology: traumatic, Iatrogenic, postsurgical trauma
- Aortic aneurysm etiology: traumatic, Iatrogenic, postprocedural trauma
Aortic aneurysm etiologic diagnostic modifiers hierarchy level 3
- Aortic aneurysm etiology—atherosclerotic
- Aortic aneurysm etiology—congenital, NOS
- Aortic aneurysm etiology—congenital, Kommerell’s diverticulum
- Aortic aneurysm etiology—connective tissue disease, NOS
- Aortic aneurysm etiology—connective tissue disease, cystic medial necrosis
- Aortic aneurysm etiology—connective tissue disease, Erdheim’s disease (annuloaortic ectasia)
- Aortic aneurysm etiology—connective tissue disease, Marfan syndrome
- Aortic aneurysm etiology—connective tissue disease, Ehlers-Danlos syndrome
- Aortic aneurysm etiology—connective tissue disease, Turner’s syndrome
- Aortic aneurysm etiology—connective tissue disease, Noonan’s syndrome
- Aortic aneurysm etiology—connective tissue disease, Klipper-Feil syndrome
- Aortic aneurysm etiology—connective tissue disease, Pseudoxanthoma elasticum
- Aortic aneurysm etiology—connective tissue disease, Other
- Aortic aneurysm etiology—infectious, NOS
- Aortic aneurysm etiology—infectious, Primary mycotic aneurysm
- Aortic aneurysm etiology—infectious, Endocarditis
- Aortic aneurysm etiology—infectious, Secondary: tuberculous mycotic aneurysm
- Aortic aneurysm etiology—infectious, Secondary: pancreatitis
- Aortic aneurysm etiology—infectious, Secondary: other
- Aortic aneurysm etiology—infectious, Syphilitic aneurysm (Leutic aortitis)
- Aortic aneurysm etiology—inflammatory, NOS
- Aortic aneurysm etiology—inflammatory, Autoimmune disorders, NOS
- Aortic aneurysm etiology—inflammatory, Autoimmune disorders, Scleroderma
- Aortic aneurysm etiology—inflammatory, Autoimmune disorders, Periarteritis nodosa
- Aortic aneurysm etiology—inflammatory, Autoimmune disorders, Reiter’s syndrome
- Aortic aneurysm etiology—inflammatory, Autoimmune disorders, Lupus
- Aortic aneurysm etiology—inflammatory, Autoimmune disorders, Ankylosing spondylitis
- Aortic aneurysm etiology—inflammatory, Autoimmune disorders, Juvenile rheumatoid arthritis
- Aortic aneurysm etiology—inflammatory, Autoimmune disorders, Other
- Aortic aneurysm etiology—inflammatory, Takayasu’s aortitis
- Aortic aneurysm etiology—inflammatory, Giant cell aortitis
- Aortic aneurysm etiology—inflammatory, Kawasaki disease
- Aortic aneurysm etiology—inflammatory, Beçhet’s disease
- Aortic aneurysm etiology—inflammatory, Other
- Aortic aneurysm etiology—traumatic, NOS
- Aortic aneurysm etiology—traumatic, Penetrating trauma
- Aortic aneurysm etiology—traumatic, Blunt trauma
- Aortic aneurysm etiology—traumatic, Iatrogenic, Postsurgical trauma
- Aortic aneurysm etiology—traumatic, Iatrogenic, Postprocedural trauma
III. Nomenclature for treatment options
Aortic aneurysms
The surgical treatment of descending thoracic aortic aneurysms dates to the early 1950s when descending thoracic aortic aneurysms were first repaired using homografts [31, 32]. In 1953, Bahnson [33] reported a series of saccular aneurysms repaired using a technique of lateral resection with primary suture repair. That same year, DeBakey and Cooley [34] reported the first successful replacement of the descending thoracic aorta with a prosthetic graft. The "elephant trunk" technique for repair of descending thoracic aneurysms after aortic arch reconstruction was introduced by Borst and associates [35] in 1983 and later extended by others with multiple variations [36, 37]. Endovascular stenting for the repair of descending thoracic aortic aneurysms was introduced by Dake and associates at Stanford in 1992 [38].
Surgical repair of ascending aortic aneurysms dates to the pioneering work of Cooley and DeBakey in 1956 [39], when they first replaced the ascending aorta with a prosthetic graft using cardiopulmonary bypass. The combination of a supracoronary tube graft with prosthetic aortic valve replacement was introduced by Starr and associates [40] in 1963. Wheat and associates [41] contributed the technique of creating coronary buttons in 1964. This was extended by Bentall and DeBono [42] in 1968, when they reported the technique of replacing the entire aortic root with a composite valved conduit, suturing the aortic wall around the coronary ostia directly to the conduit, and creating an inclusion wrap using the surrounding aortic wall. However, the high incidence of pseudoaneurysm formation found with the inclusion wrap technique prompted Kouchoukas and associates [43] to advocate an open valved conduit repair with avoidance of the inclusion wrap. Cabrol and associates [44] contributed the technique of attaching the coronaries to the ascending aortic graft using a separate tube graft. Sommerville and Ross [45] reported their long-term results with aortic root replacement using a homograft in 1982. Yacoub and associates [46] and David [47] have introduced the valve-sparing aortic root replacement techniques.
Successful repair of an aortic arch aneurysm was first accomplished by DeBakey and colleagues in 1957 [48] using direct brachiocephalic perfusion and replacement of the arch with a homograft. The use of a single Carrel patch anastomosis of the cerebral vessels was introduced by Bloodwell and associates [49] in 1968. The modern era of arch reconstruction using deep hypothermic circulatory arrest was pioneered by Griepp and colleagues at Stanford and outlined in their 1975 report [50].
The first successful surgical correction of a sinus of Valsalva fistula was performed under deep hypothermia without cardiopulmonary bypass and reported by Brown and associates in 1955 [51]. In 1956, Lillehei and associates [52] and McGoon and associates [53] were the first to use cardiopulmonary bypass to resect a sinus of Valsalva aneurysm and close the defect with a simple suture repair. Interestingly, Lillehei and associates also described the use of potassium citrate cold blood cardioplegia administered retrograde through the coronary sinus to initiate cardiac arrest in their first patient.
In general, the surgical repairs for sinus of Valsalva aneurysms are divided into repairs accomplished through the chamber of origin (aorta), through the chamber of penetration (right atrium, right ventricle, left atrium, left ventricle, pulmonary artery, or pericardium), or through both chambers. They may also be classified into primary suture closure (with or without pledgets), patch closure (single or double), or aortic root replacement (usually for associated uncorrectable aortic insufficiency, or multiple sinus involvement). Additional procedures commonly performed at the time of sinus of Valsalva repair include closure of an associated ventricular septal defect, repair or replacement of the aortic valve, and coronary reconstruction. These additional procedures are coded separately as outlined in the appropriate chapters discussing these topics.
The procedures developed for treatment of false aneurysms have paralleled those for true aneurysms of the aorta. Therefore, coding for repair of both false and true aneurysms of the aorta are the same.
Aortic dissection
The first attempts at surgical control of aortic dissection involved distal fenestration [54] and cellophane wrapping of the aorta [55]. DeBakey and associates [56] reported the first direct surgical repair of a descending thoracic aortic dissection utilizing a prosthetic graft in 1955. This was followed by Cooley and associates’ report of ascending aortic aneurysm repair with a graft on cardiopulmonary bypass in 1957 [57]. Morris and colleagues first reported successful repair of an acute ascending aortic dissection in 1963 [58]. The principles of replacing of the entire segment involving the proximal tear, obliterating the false lumen proximally, and reconstituting antegrade flow through the true lumen of the aorta to adequately perfuse distal branches are advocated by most centers. Tube graft replacement of the ascending aorta with resuspension of the aortic valve is preferred by the Stanford group [59] for proximal dissections. Full aortic root replacement with a composite graft (Bentall type procedure) is generally reserved for cases involving aortic root dilation such as annuloaortic ectasia or Marfan’s syndrome. The role of valve-sparing aortic root replacement for acute proximal aortic dissection is unclear. The first successful repair of an acute aortic arch dissection was not reported until 1980 [60], indicative of the very poor historical results with this lesion. However, the poor late results of arch dissections that have not been repaired, along with refinements in operative technique, have recently prompted a more aggressive approach to repair at the time of presentation [61].
The difficulty with performing a hemostatic anastomosis when dealing with the friable tissues of the dissected aorta prompted Cooley and Livesay [62] to advocate using an open distal anastomotic technique. Under deep hypothermic circulatory arrest with retrograde "trickle flow" in the descending aorta, all clamping of the aortic arch and descending aorta is avoided while the distal aortic anastomosis is accomplished. The sutureless prosthesis for repair of acute aortic dissections was introduced in 1976 by several surgical teams, but has been criticized due to the complications of false aneurysm formation and erosion of the rigid ring [63].
Procedural hierarchy for aortic aneurysms and dissections
Hierarchy level 1
- Aortic aneurysm repair
- Sinus of Valsalva aneurysm repair
- Aortic dissection repair
Hierarchy level 2: location/anatomy
- Aortic aneurysm repair, NOS
- Aortic aneurysm repair, Root
- Aortic aneurysm repair, Ascending
- Aortic aneurysm repair, Arch
- Aortic aneurysm repair, Descending
- Aortic aneurysm repair, Thoracoabdominal
- Aortic aneurysm repair, Abdominal
- Sinus of Valsalva aneurysm repair, NOS
- Sinus of Valsalva aneurysm repair, Left sinus
- Sinus of Valsalva aneurysm repair, Right sinus
- Sinus of Valsalva aneurysm repair, Noncoronary sinus
- Aortic dissection repair, NOS
- Aortic dissection repair, Proximal (ascending only, type A, type II)
- Aortic dissection repair, Proximal (ascending and arch, type A, type I)
- Aortic dissection repair, Distal (type B, III)
Hierarchy level 3: technique
- Aortic aneurysm repair, NOS
- Aortic aneurysm repair, Root, NOS
- Aortic aneurysm repair, Root, External wrap
- Aortic aneurysm repair, Root, Primary (suture)
- Aortic aneurysm repair, Root, Patch graft
- Aortic aneurysm repair, Root, Tube graft (Yacoub/David valve sparing)
- Aortic aneurysm repair, Root, Valved conduit
- Aortic aneurysm repair, Ascending, NOS
- Aortic aneurysm repair, Ascending, External wrap
- Aortic aneurysm repair, Ascending, Primary (suture)
- Aortic aneurysm repair, Ascending, Patch graft
- Aortic aneurysm repair, Ascending, Tube graft
- Aortic aneurysm repair, Ascending, Valved conduit
- Aortic aneurysm repair, Arch, NOS
- Aortic aneurysm repair, Arch, External wrap
- Aortic aneurysm repair, Arch, Primary (suture)
- Aortic aneurysm repair, Arch, Patch graft
- Aortic aneurysm repair, Arch, Tube graft
- Aortic aneurysm repair, Descending, NOS
- Aortic aneurysm repair, Descending, External wrap
- Aortic aneurysm repair, Descending, Primary (suture)
- Aortic aneurysm repair, Descending, Patch graft
- Aortic aneurysm repair, Descending, Tube graft
- Aortic aneurysm repair, Thoracoabdominal, NOS
- Aortic aneurysm repair, Thoracoabdominal, External wrap
- Aortic aneurysm repair, Thoracoabdominal, primary (suture)
- Aortic aneurysm repair, Thoracoabdominal, Patch graft
- Aortic aneurysm repair, Thoracoabdominal, Tube graft
- Aortic aneurysm repair, Abdominal, NOS
- Aortic aneurysm repair, Abdominal, External wrap
- Aortic aneurysm repair, Abdominal, Primary (suture)
- Aortic aneurysm repair, Abdominal, Patch graft
- Aortic aneurysm repair, Abdominal, Tube graft
- Aortic aneurysm repair, Abdominal, Bifurcated graft
- Sinus of Valsalva aneurysm repair, NOS
- Sinus of Valsalva aneurysm repair, Left sinus, NOS
- Sinus of Valsalva aneurysm repair, Left sinus, Primary (suture)
- Sinus of Valsalva aneurysm repair, Left sinus, Patch graft
- Sinus of Valsalva aneurysm repair, Left sinus, Tube graft
- Sinus of Valsalva aneurysm repair, Left sinus, Valved conduit
- Sinus of Valsalva aneurysm repair, Right sinus, NOS
- Sinus of Valsalva aneurysm repair, Right sinus, Primary (suture)
- Sinus of Valsalva aneurysm repair, Right sinus, Patch graft
- Sinus of Valsalva aneurysm repair, Right sinus, Tube graft
- Sinus of Valsalva aneurysm repair, Right sinus, Valved conduit
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, NOS
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, primary (suture)
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Patch graft
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Tube graft
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Valved conduit
- Aortic dissection repair, NOS
- Aortic dissection repair, Proximal (ascending only, type A, type II), NOS
- Aortic dissection repair, Proximal (ascending only, type A, type II), Primary (suture)
- Aortic dissection repair, Proximal (ascending only, type A, type II), Patch graft
- Aortic dissection repair, Proximal (ascending only, type A, type II), Tube graft
- Aortic dissection repair, Proximal (ascending only, type A, type II), Valved conduit
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), NOS
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Primary (suture)
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Patch graft
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Tube graft
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Valved conduit
- Aortic dissection repair, Distal (descending and/or distal), NOS
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Fenestration
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Primary (suture)
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Patch graft
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Tube graft
Hierarchy level 4: technique
- Aortic aneurysm repair, NOS
- Aortic aneurysm repair, Root, NOS
- Aortic aneurysm repair, Root, External wrap
- Aortic aneurysm repair, Root, Primary (suture)
- Aortic aneurysm repair, Root, Patch graft
- Aortic aneurysm repair, Root, Tube graft (Yacoub/David valve sparing)
- Aortic aneurysm repair, Root, Valved conduit
- Aortic aneurysm repair, Ascending, NOS
- Aortic aneurysm repair, Ascending, External wrap
- Aortic aneurysm repair, Ascending, Primary (suture)
- Aortic aneurysm repair, Ascending, Patch graft
- Aortic aneurysm repair, Ascending, Tube graft
- Aortic aneurysm repair, Ascending, Valved conduit
- Aortic aneurysm repair, Arch, NOS
- Aortic aneurysm repair, Arch, External wrap
- Aortic aneurysm repair, Arch, Primary (suture)
- Aortic aneurysm repair, Arch, Patch graft
- Aortic aneurysm repair, Arch, Tube graft, NOS
- Aortic aneurysm repair, Arch, Tube graft, Carrel patch arch vessels
- Aortic aneurysm repair, Arch, Tube graft, Bypass graft arch vessels
- Aortic aneurysm repair, Arch, Tube graft, Branch graft arch vessels
- Aortic aneurysm repair, Arch, Tube graft, Elephant trunk distal
- Aortic anerysm repair, Descending, NOS
- Aortic aneurysm repair, Descending, External wrap
- Aortic aneurysm repair, Descending, Primary (suture)
- Aortic aneurysm repair, Descending, Patch graft
- Aortic aneurysm repair, Descending, Tube graft, NOS
- Aortic aneurysm repair, Descending, Tube graft, Interposition
- Aortic aneurysm repair, Descending, Tube graft, Bypass
- Aortic aneurysm repair, Descending, Tube graft, Elephant trunk proximal
- Aortic aneurysm repair, Thoracoabdominal, NOS
- Aortic aneurysm repair, Thoracoabdominal, External wrap
- Aortic aneurysm repair, Thoracoabdominal, Primary (suture)
- Aortic aneurysm repair, Thoracoabdominal, Patch graft
- Aortic aneurysm repair, Thoracoabdominal, Tube graft, NOS
- Aortic aneurysm repair, Thoracoabdominal, Tube graft, Interposition
- Aortic aneurysm repair, Thoracoabdominal, Tube graft, Bypass
- Aortic aneurysm repair, Abdominal, NOS
- Aortic aneurysm repair, Abdominal, External wrap
- Aortic aneurysm repair, Abdominal, Primary (suture)
- Aortic aneurysm repair, Abdominal, Patch graft
- Aortic aneurysm repair, Abdominal, Tube graft, NOS
- Aortic aneurysm repair, Abdominal, Tube graft, Interposition
- Aortic aneurysm repair, Abdominal, Tube graft, Bypass
- Aortic aneurysm repair, Abdominal, Bifurcated graft, NOS
- Aortic aneurysm repair, Abdominal, Bifurcated graft, Interposition
- Aortic aneurysm repair, Abdominal, Bifurcated graft, Bypass
- Sinus of Valsalva aneurysm repair, NOS
- Sinus of Valsalva aneurysm repair, Left sinus, NOS
- Sinus of Valsalva aneurysm repair, Left sinus, Primary (suture), NOS
- Sinus of Vassalva aneurysm repair, Left sinus, Primary (suture), Via aorta
- Sinus of Valsalva aneurysm repair, Left sinus, Primary (suture), Via atrium
- Sinus of Valsalva aneurysm repair, Left sinus, Primary (suture), Via right ventricle
- Sinus of Valsalva aneurysm repair, Left sinus, Primary (suture), Via pulmonary artery
- Sinus of Valsalva aneurysm repair, Left sinus, Patch graft, NOS
- Sinus of Valsalva aneurysm repair, Left sinus, Patch graft, Via aorta
- Sinus of Valsalva aneurysm repair, Left sinus, Patch graft, Via atrium
- Sinus of Valsalva aneurysm repair, Left sinus, Patch graft, Via right ventricle
- Sinus of Valsalva aneurysm repair, Left sinus, Patch graft, Via pulmonary artery
- Sinus of Valsalva aneurysm repair, Left sinus, Tube graft
- Sinus of Valsalva aneurysm repair, Left sinus, Valved conduit
- Sinus of Valsalva aneurysm repair, Right sinus, NOS
- Sinus of Valsalva aneurysm repair, Right sinus, Primary (suture), NOS
- Sinus of Valsalva aneurysm repair, Right sinus, Primary (suture), Via aorta
- Sinus of Valsalva aneurysm repair, Right sinus, Primary (suture), Via atrium
- Sinus of Valsalva aneurysm repair, Right sinus, Primary (suture), Via right ventricle
- Sinus of Valsalva aneurysm repair, Right sinus, Primary (suture), Via pulmonary artery
- Sinus of Valsalva aneurysm repair, Right sinus, Patch graft, NOS
- Sinus of Valsalva aneurysm repair, Right sinus, Patch graft, Via aorta
- Sinus of Valsalva aneurysm repair, Right sinus, Patch graft, Via atrium
- Sinus of Valsalva aneurysm repair, Right sinus, Patch graft, Via right ventricle
- Sinus of Valsalva aneurysm repair, Right sinus, Patch graft, Via pulmonary artery
- Sinus of Valsalva aneurysm repair, Right sinus, Tube graft
- Sinus of Valsalva aneurysm repair, Right sinus, Valved conduit
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, NOS
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Primary (suture), NOS
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Primary (suture), Via aorta
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Primary (suture), Via atrium
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Primary (suture), Via right ventricle
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, primary (suture), Via pulmonary artery
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Patch Graft, NOS
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Patch graft, Via aorta
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Patch graft, Via atrium
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Patch graft, Via right ventricle
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Patch graft, Via pulmonary artery
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Tube graft
- Sinus of Valsalva aneurysm repair, Noncoronary sinus, Valved conduit
- Aortic dissection repair, NOS
- Aortic dissection repair, Proximal (ascending only, type A, type II), NOS
- Aortic dissection repair, Proximal (ascending only, type A, type II), Primary (suture)
- Aortic dissection repair, Proximal (ascending only, type A, type II), Patch graft
- Aortic dissection repair, Proximal (ascending only, type A, type II), Tube graft, NOS
- Aortic dissection repair, Proximal (ascending only, type A, type II), Tube graft, Inclusion wrap
- Aortic dissection repair, Proximal (ascending only, type A, type II), Tube graft, Exclusion/resect
- Aortic dissection repair, Proximal (ascending only, type A, type II), Valved conduit, NOS
- Aortic dissection repair, Proximal (ascending only, type A, type II), Valved conduit, Inclusion wrap (Bentall)
- Aortic dissection repair, Proximal (ascending only, type A, type II), Valved conduit, Exclusion/resection
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), NOS
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Primary (suture)
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Patch graft
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Tube graft, NOS
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Tube graft, Iinclusion wrap
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Tube graft, Exclusion/resection
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Tube graft, Elephant trunk distal
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Valved conduit, NOS
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Valved conduit, Inclusion wrap (Bentall)
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Valved conduit, Exclusion/resection
- Aortic dissection repair, Proximal (ascending and arch, type A, type I), Valved conduit, Elephant trunk distal
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), NOS
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Fenestration
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Primary (suture)
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Patch graft
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Tube graft, NOS
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Tube graft, Inclusion wrap
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Tube graft, Exclusion/resection
- Aortic dissection repair, Distal (descending and/or distal) (type B, III), Tube graft, Elephant trunk proximal
Additional coding guidelines
Because aortic aneurysms and pseudoaneurysms are repaired using similar procedures, the procedure codes for repair of these defects are the same. All associated procedures (eg, aortic valve procedures, ventricular septal defect surgery, coronary procedures, etc) should be separately coded using procedure codes from the appropriate sections (aortic valve disease, ventricular septal defects, coronary abnormalities, etc). This includes various techniques for handling the coronaries (bypass, Cabrol modification, direct reimplantation, etc), and aortic valve procedures (eg, resuspension, replacement, repair, etc). The use of any prosthetic material (patches, tube grafts [straight or bifurcated], sutureless grafts, homografts, endoluminal prostheses, conduits, valves, stents, biological glue, etc) should also be coded separately under the prosthetic material module.
IV. Diagnosis and procedure short lists
- Diagnosis Short List
- Aortic aneurysm (including pseudoaneurysm)
- Sinus of Valsalva aneurysm
- Aortic dissection
- Procedure Short List
- Aortic aneurysm repair
- Sinus of Valsalva, aneurysm repair
- Aortic dissection repair
V. Diagnosis specific risk factors
Preoperative variables
Demographic
Age
Gender
Diagnoses
Cardiac
Ruptured versus nonruptured aneurysms
Associated ventricular septal defect (VSD)
Coarctation
Aortic valve disease
Acute versus chronic dissection
Proximal versus distal
Aortic arch involvement
Aortic valve insufficiency
Pericardial effusion/tamponade
Noncardiac
Endocarditis
Ischemic syndromes (branch arteries)
Central neurologic
Myocardial
Visceral
Renal
Spinal cord
Upper extremity
Lower extremity
Erdheim’s cystic medial necrosis
Marfan’s syndrome
Ehlers-Danlos syndrome
Turner’s syndrome
Noonan’s syndrome
Klippel-Feil syndrome
Pseudoxanthoma elasticum
Osteogenesis imperfecta
Takayasu’s disease
Kawasaki’s disease
Autoimmune disease
Hemothorax
Hypertension
Family history of aneurysm
Diagnostic technique
Echo [Transthoracic echocardiogram (TTE), transesophageal echocardiogram (TEE)]
Computed tomography scan
Magnetic resonance imaging
Angiography (aortogram)
Intraoperative variables
Additional procedures
Aortic valve surgery
VSD surgery
Coronary artery surgery
Techniques
Retrograde cerebral perfusion
Selective antegrade cerebral perfusion
Atrial-femoral/aortic bypass
Femoral-femoral bypass
Postoperative variables
Residual defects
Residual sinus of Valsava aneurysm (SVA) fistula
Residual aortic insufficiency
Residual VSD
Heart block
Endocarditis
Neurological injury
Paraplegia
Recurrent nerve injury
Phrenic nerve injury
Horners syndrome
Confusion/delerium
Permanent stroke
Renal failure
Reoperation for bleeding
Ischemic syndromes
Myocardial
Upper extremity
Lower extremity
Visceral
Renal
VI. Database studies and outcomes analysis
Aortic aneurysm (excluding sinus of valsalva aneurysms)
- Number of aortic aneurysms by year (true vs false)
- Number of aortic aneurysms by anatomic location (true vs false)
- Number of aortic aneurysms by urgency (true vs false)
- Incidence of aortic aneurysm as primary diagnosis by year and total (true vs false)
- Incidence of aortic aneurysm repair as a procedure by year and total (true vs false)
- Type of aortic aneurysm repair (all hierarchies) by anatomic location (true vs false)
- Age distribution of aortic aneurysm (true vs false)
- Gender distribution of aortic aneurysm (true vs false)
- Diagnostic procedures for aortic aneurysm by year
- Number and percentage of additional procedures
- Distribution (type, number, and percentages) of procedures (all hierarchies)
- Distribution (type, number, and percentages) of prosthetic materials
- Perfusion technique used by location (none, shunt, cardiopulmonary bypass, deep hypothermic circulatory arrest (DHCA), atrial-femoral bypass, aorto-femoral bypass, femoral-femoral bypass, etc)
- Operative mortality for aortic aneurysm by year (true vs false)
- Operative mortality for aortic aneurysm by anatomic location (true vs false)
- Operative mortality for aortic aneurysm by diagnosis (all hierarchies)
- Operative mortality for aortic aneurysm by age (true vs false)
- Operative mortality for aortic aneurysm by urgency (ruptured vs nonruptured)
- Postop length of stay by year (true vs false)
- Postop length of stay by anatomic location (true vs false)
- Postop length of stay by urgency (ruptured vs nonruptured)
- Complications of aortic aneurysm repair (number and percentage) by year
- Complications of aortic aneurysm repair (number and percentage) by location
- Complications of aortic aneurysm repair (number and percentage) by urgency (ruptured vs nonruptured)
- Complications of aortic aneurysm repair (number and percentage) (true vs false)
Sinus of valsalva aneurysm
- Number and percentage of SVA by year and type (congenital vs acquired)
- Number and percentage of congenital SVA by year and type (nonruptured vs ruptured)
- Number and percentage of acquired SVA by year and type (nonruptured vs ruptured)
- Incidence of SVA as a primary diagnosis by year and total
- Incidence of SVA as a procedure by year and total
- Age distribution of congenital SVA
- Age distribution of congenital SVA (nonruptured vs ruptured)
- Gender distribution of congenital SVA
- Gender distribution of congenital SVA (nonruptured vs ruptured)
- Number and percentage of congenital SVA by sinus of origin
- Number and percentage of acquired SVA by sinus of origin
- Number and percentage of congenital SVA by sinus of origin and chamber of penetration
- Number and percentage of acquired SVA by sinus of origin and chamber of penetration
- Distribution (number and percentage of all congenital SVA) of associated cardiac diagnoses (VSD, aortic insufficiency (AI), atrial septal defect (ASD), etc)
- Distribution (number and percentage of all congenital SVA) of associated non-cardiac diagnoses (endocarditis)
- Diagnostic procedures for congenital SVA by year (nonruptured vs ruptured)
- Urgency status for congenital SVA by year (nonruptured vs ruptured)
- Urgency status for acquired SVA by year (nonruptured vs ruptured)
- Distribution (number and percentage) of type of SVA repair (all hierarchies) for congenital SVA
- Number and percentage of additional procedures (aortic valve repair/replacement, VSD closure, etc)
- Number and percentage of prosthetic materials (patches, valves, valved conduits, etc) for congenital SVA repair
- Number and percentage of prosthetic materials (patches, valves, valved conduits, etc) for acquired SVA repair
- Use of DHCA by year
- Myocardial preservation technique for congenital SVA repair by year (nonruptured vs ruptured)
- Times (mean ± SD) of CPB, aortic cross-clamp (AXC), and DHCA for congenital SVA by year (nonruptured vs ruptured)
- Operative mortality by year for congenital SVA by year (nonruptured vs ruptured)
- Operative mortality by year for acquired SVA by year (nonruptured vs ruptured)
- Operative mortality by year for congenital SVA by year and preoperative status
- Operative mortality by year for acquired SVA by year and preoperative status
- Operative mortality by year for congenital SVA by age
- Operative mortality by year for acquired SVA by age
- Complications for congenital SVA by year (nonruptured vs ruptured)
- Complications for acquired SVA by year (nonruptured vs ruptured)
- Postop length of stay for congenital SVA by year (mean ± SD)
- Intensive care unit length of stay for congenital SVA by year (mean ± SD)
Aortic dissection
- Number of aortic dissection procedures by year (acute vs chronic, proximal vs distal)
- Number of aortic dissection procedures by age (acute vs chronic, proximal vs distal)
- Distribution (number and percentage) of type of aortic dissection repair by year (all hierarchies)
- Distribution of associated cardiac diagnoses (diagnosis, number, and percentage of all aortic dissections)
- Distribution of associated noncardiac diagnoses (diagnosis, number, and percentage of all aortic dissections) (eg, Marfans, family history, etc)
- Diagnostic procedure for aortic dissection by year (percentages)
- Number of aortic dissection procedures by gender (acute vs chronic, proximal vs distal)
- Number and percentages of additional procedures (aortic valve surgery, VSD, coronary procedure, etc)
- Number and percentages of prosthetic materials by year for aortic dissection repair (proximal vs distal)
- Use of DHCA, retrograde vs antegrade cerebral perfusion, atrial-femoral vs femoral-femoral bypass by year (proximal vs distal)
- Duration and distribution of DHCA times by year (proximal vs distal)
- Operative mortality by year (acute vs chronic, proximal vs distal)
- Operative mortality by age and year
- Operative mortality by procedure (all hierarchies)
- Complications by year (number and percentage, proximal vs distal, acute vs chronic)
- Postoperative length of stay (mean ± SD) for aortic dissection by year (proximal vs distal, acute vs chronic)
- Intensive care unit length of stay (mean ± SD) for aortic dissection by year (proximal vs distal, acute vs chronic)
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