ﺑﺎﺯﮔﺸﺖ ﺑﻪ ﺻﻔﺤﻪ ﻗﺒﻠﯽ
خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
نسخه الکترونیک
medimedia.ir

Bicuspid aortic valve: Intervention for valve disease or aortopathy in adults

Bicuspid aortic valve: Intervention for valve disease or aortopathy in adults
Literature review current through: Jan 2024.
This topic last updated: Dec 18, 2023.

INTRODUCTION — The bicuspid aortic valve (BAV) condition is a valvulo-aortopathy (affecting both the aortic valve and the thoracic aorta); therefore, its management in adults includes surveillance of aortic valve function and the thoracic aorta (the aortic root and ascending aorta), timely intervention, treatment of hypertension, measures to address the risk of infective endocarditis, counseling patients on physical activity, and management prior to and during pregnancy [1]. Interventional management of valve disease and aortopathy in adults with BAV will be discussed here.

Other aspects of management and diagnosis of BAV in adults are discussed separately:

(See "Bicuspid aortic valve: General management in adults".)

(See "Bicuspid aortic valve: Management during pregnancy".)

(See "Clinical manifestations and diagnosis of bicuspid aortic valve in adults".)

BAV in children is discussed separately:

(See "Valvar aortic stenosis in children" and "Subvalvar aortic stenosis (subaortic stenosis)".)

RATIONALE FOR INTERVENTION — Indications for intervention for valvulopathy and for aortopathy in adults with BAV have been developed to provide timely treatment to reduce the risk of adverse outcomes from uncorrected progressive disease while managing the risk associated with intervention. In patients with BAV, decisions regarding the timing of BAV intervention and aortic root or ascending aorta surgery are complex, requiring consideration of the aortic valve function, aortic size, and other patient characteristics.

The indications for intervention are based upon natural history and intervention outcomes data for aortic stenosis (AS), aortic regurgitation, and aortic disease (algorithm 1), as discussed below. (See 'Procedures for aortic stenosis' below and 'Procedures for aortic regurgitation' below and 'Rationale for aortic size thresholds' below.)

Recommendations for intervention presented here are similar to recommendations in the 2020 American College of Cardiology/American Heart Association (ACC/AHA) guideline for valvular heart disease, the 2021 European Society of Cardiology/European Association for Cardio-Thoracic Surgery guidelines for valvular heart disease, and the 2022 ACC/AHA guideline for aortic disease [2-4].

TYPES OF INTERVENTION — Options for intervention depend upon whether the patient with BAV requires aortic valve intervention, repair of the aorta, or both.

Surgical repair of the aorta (most commonly involving replacement of a dilated aortic segment with a synthetic polyethylene terephthalate [eg, Dacron] graft) may involve one or more of four anatomic areas: the aortic valve, the aortic root (from the valve annulus to the sinotubular junction), the tubular ascending aorta (from the sinotubular junction to the innominate artery), and, occasionally, the aortic arch [5]. BAV patients with aortopathy exhibit predominant ascending aorta dilation (70 percent), and some exhibit predominant root dilation (20 percent). However, it is common for root-predominant patients to also exhibit dilation of the ascending aorta.

In some patients, dilation of the ascending aorta extends to the aortic arch [5]. In such cases, concomitant aortic arch repair (hemiarch or total arch) may be required, depending upon the extent of arch dilation, surgical expertise, and technique.

Isolated aortic valve intervention – A patient with an indication for intervention for AS or aortic regurgitation (AR) without an indication for repair of the aorta is a candidate for aortic valve intervention without surgery on the aorta. This scenario is more frequent than an isolated indication for intervention for the aorta without valve intervention [6]. Options for aortic valve intervention are discussed below. (See 'For aortic stenosis' below and 'For aortic regurgitation' below.)

Valve-sparing aortic surgery – A patient with an indication for surgery on the aortic root and/or ascending aorta without an indication for aortic valve replacement is a candidate for "valve-sparing aortic repair" (the patient retains their native BAV) (figure 1). The majority of patients with BAV have predominant ascending aortic dilation and do not require root replacement for aneurysm disease; in these patients, a valve-sparing ascending aorta repair (from the sinotubular junction to the innominate artery) is performed and may include BAV repair for AR. Valve-sparing aortic root surgery involves surgical repair of the aortic root and ascending aorta while retaining the native valve and includes the David (reimplantation technique), Yacoub (remodeling technique), and partial Yacoub procedures [7]. These procedures may include concomitant repair of BAV AR; there is growing experience with surgical aortic valve repair, rather than replacement, for selected cases of AR complicating BAV [8-10].

Combined aortic valve replacement and aortic surgery – Patients with indications for both aortic valve replacement and repair of the aorta are candidates for combined aortic valve and aortic surgery. This is generally accomplished by surgical aortic valve replacement with concurrent aortic repair.

With aortic root sparing For a patient who requires aortic valve replacement and has ascending aortic dilation only above the sinotubular junction, a prosthetic aortic valve and a "supracoronary" (from the sinotubular junction to the innominate artery) aortic Dacron graft are placed.

With aortic root replacement – For a patient who requires aortic valve replacement and has a dilated aortic root and ascending aorta, a composite prosthetic aortic valve and aortic root and ascending aorta graft replacement (modified Bentall procedure) may be performed. Alternatively, a homograft that includes valve, root, and ascending aorta is infrequently used in selected cases (eg, in the setting of infective endocarditis). The Bentall procedure or homograft procedure includes reimplantation of the coronary arteries into the ascending aortic graft (figure 2). The aortic valve prosthesis may be mechanical or biologic ("bio-Bentall").

FOR AORTIC STENOSIS — Aortic valve replacement for AS is the most common intervention required in patients with BAV [6]. Timely intervention is indicated for patients with BAV patients with severe AS to improve clinical outcomes.

Indications for intervention for AS — Standard recommendations for aortic valve intervention for high-gradient severe AS and low-gradient severe AS (table 1) apply to patients with BAV. (See "Indications for valve replacement for high gradient aortic stenosis in adults", section on 'Indications for valve replacement' and "Management and prognosis of low gradient aortic stenosis".)

Procedures for aortic stenosis — For patients with severe AS requiring valve intervention, the main options are surgical aortic valve replacement (SAVR; mechanical or bioprosthetic) and transcatheter aortic valve implantation (TAVI). Compared with tricuspid aortic valve (TAV) patients, patients with BAV attain severe AS at 60s and 70s years of age, versus 70s and 80s years of age for patients with TAV. [11]. Patient age is an important consideration when choosing the type of SAVR (mechanical versus biologic) and when considering TAVI. (See "Choice of intervention for severe calcific aortic stenosis".)

The choice between SAVR and TAVI is discussed separately. (See "Indications for valve replacement for high gradient aortic stenosis in adults" and "Choice of intervention for severe calcific aortic stenosis".)

For patients undergoing SAVR, the choice between a mechanical or bioprosthetic valve is discussed separately. (See "Choice of prosthetic heart valve for surgical aortic or mitral valve replacement".)

For selected younger patients – The Ross procedure (pulmonary autograft) may be an option for selected young and middle-aged patients requiring surgical valve replacement (see 'Ross procedure' below). For selected patients (generally <25 years of age) with severe AS but noncalcified valves and no more than mild aortic regurgitation (AR), balloon aortic valvotomy is an alternative option. (See 'Balloon valvotomy' below.)

When aortic valve intervention is indicated, the need for concomitant prophylactic repair of the aorta must be assessed. (See 'Approach to identifying candidates for aortic surgery' below.)

Transcatheter aortic valve implantation — Although BAV was previously considered a contraindication for TAVI due to technical concerns about elliptical deployment or underdeployment, TAVI has been successfully performed in many patients with calcific bicuspid stenosis [12-26]. While patients with BAV were excluded from the early landmark randomized trials for TAVI using balloon-expandable or self-expanding prostheses, observational data are available on TAVI in patients with BAV [20-26]. In addition, it is frequently difficult to ascertain the number of valve leaflets in the presence of severe calcific stenosis, so a significant number of patients with an indeterminate leaflet number may have congenital BAV.

Anatomical variability of the valve, annulus, and left ventricular outflow tract, distribution and deposition of valvular and annular calcification, and the presence of aortic disease present complexities for TAVI in BAV disease [21]. Data are emerging on the effect of various BAV phenotypes on TAVI outcome. The choice between SAVR and TAVI for patients with severe AS is discussed separately. (See "Indications for valve replacement for high gradient aortic stenosis in adults" and "Choice of intervention for severe calcific aortic stenosis".)

Comparison of outcomes for BAV versus TAV — Contemporary observational studies of patients undergoing TAVI have generally found similar one-year mortality rates in patients with BAV compared with patients with TAV [20-26]. The following studies are based on data from the Society of Thoracic Surgeons/American College of Cardiology (STS/ACC) Transcatheter Valve Therapy Registry [20,23,24]:

A study analyzed data on 2691 propensity-matched pairs of patients with bicuspid and tricuspid AS undergoing TAVI during 2015 to 2018 with a mean STS score of 5 percent [20]. Mortality rates were similar in the two groups at 30 days (2.6 versus 2.5 percent) and one year (10.5 versus 12 percent). However, patients with bicuspid AS had higher 30-day stroke rate (2.5 versus 1.6 percent) and procedural complications requiring open heart surgery (0.9 versus 0.4 percent) than those with tricuspid AS. Given the limited data on patient selection for TAVI in this study, the clinical significance of these findings is uncertain. Similar rates of post-TAVI paravalvular AR were identified in patients with bicuspid AS compared with patients with tricuspid disease (2 versus 2.4 percent at 30 days and 3.2 versus 2.5 percent at one year) [20].

A study compared outcomes in individuals with bicuspid and tricuspid AS undergoing TAVI from 2011 through 2018 [24]. Of 170,959 procedures, 5412 (3.2 percent) were performed for individuals with BAV. BAV patients were significantly younger and had lower STS scores than those with TAV.

With current-generation TAVI devices compared with older devices, device success rate was higher (96.3 versus 93.5 percent) and the incidence of moderate or more AR was lower (2.7 versus 14 percent).

With current devices, device success rate was nominally but not significantly lower in BAV patients compared with those with TAV (96.3 versus 97.4 percent) with a slightly higher incidence of moderate or severe AR among patients with BAV (2.7 versus 2.1 percent). A lower one-year adjusted risk of mortality (hazard ratio 0.88, 95% CI 0.78-0.99) was observed for patients with BAV versus TAV, and there was no difference in one-year risk of stroke.

A study analyzed data on 3168 propensity-matched pairs of patients with bicuspid and tricuspid AS with low surgical risk (STS score 1.7 percent) undergoing TAVI during 2015 to 2020 [23]. Mortality rates were similar in the two groups at 30 days (0.9 versus 0.8 percent) and at one year (4.6 versus 6.6 percent). Stroke rates were also similar at 30 days (1.4 versus 1.2 percent) and at one year (2 versus 2.1 percent). There were similar rates of procedural complications, valve hemodynamics, and moderate or severe paravalvular leak (3.4 versus 2.1 percent).

Studies have reported TAVI outcomes in highly selected patients with BAV and low surgical risk.

A prospective single-arm study evaluated early outcomes following self-expandable supra-annular TAVI in 150 patients (mean age 70.3) with BAV and low surgical risk (mean STS score of 1.4) [26]. The incidence of mortality or disabling stroke was 1.3 percent (one death and one stroke) at 30 days. The device success rate was 95.3 percent. A permanent pacemaker was implanted in 15.1 percent. No patient had greater than mild paravalvular regurgitation.

For comparison of one-year outcomes following self-expandable supra-annular TAVI, 145 of these patients with BAV and low surgical risk [26] were propensity-matched with 145 patients with TAV and low surgical risk [21]. Device success rates were similar in the two groups (95.8 in the bicuspid group and 96.5 percent in the tricuspid group). At one year, all-cause mortality rates (0.7 percent for bicuspid and 2.1 percent for tricuspid) and stroke rates (4.8 percent for bicuspid and 2.8 percent for tricuspid) were similar in the two groups, and there were similar rates of permanent pacemaker implantation (16.6 percent for bicuspid and 17.9 percent for tricuspid). There was a lower incidence of mild or worse paravalvular regurgitation among the patients with BAV (21.3 versus 42.6 percent). It is uncertain whether this difference in paravalvular regurgitation rate was caused by differences in procedures (eg, more frequent use of pre-TAVI balloon aortic valvuloplasty in the bicuspid group).

The PARTNER 3 Bicuspid Registry examined the one-year safety and efficacy of TAVI using the balloon-expandable SAPIEN 3 valve in low-risk patients with severe BAV stenosis [22]. The careful patient selection process excluded patients with severe raphe or subvalvular calcification or aortic dilation. Among a total of 320 submitted bicuspid patients, 169 (53 percent) were treated with TAVI. In a propensity-matched analysis comparing TAVI in BAV with the PARTNER 3 TAVI cohort, there were no differences in the one-year composite rate (or its individual components) of death, stroke, and cardiovascular rehospitalization between patients with bicuspid and tricuspid aortic valve stenosis [22].

Effect of BAV morphology — The effect of BAV morphology on outcomes was assessed in a study of 1034 patients with computed tomography (CT)-confirmed BAV (mean age 74.7 and STS score 3.7 percent) undergoing TAVI with contemporary devices [25]. Mortality at 30 days, one year, and two years was 2, 6.7, and 12.5 percent. Two-year mortality was significantly higher in patients with both calcified raphe (moderate or greater) and excess leaflet calcification compared with those with one or none of these features (25.7 versus 9.5 versus 5.9 percent). Patients with both of these morphologic features also had higher rates of aortic root injury, moderate or greater paravalvular regurgitation, and 30-day mortality.

Ross procedure — The Ross procedure is a surgical option at centers of excellence for selected young and middle-aged patients (generally <50 years old) with nonrepairable bicuspid valves who prefer to avoid the long-term anticoagulation required with a mechanical aortic valve prosthesis and favor a procedure that may have more durability than surgical bioprosthetic aortic valve replacement. In addition, candidates for this procedure are generally required to have at least a 20-year expected survival.

The Ross procedure (pulmonary autograft replacement of the aortic valve-and-root plus implantation of a homograft valve-and-root in the pulmonic position) is a complex procedure to replace the diseased aortic valve with a living substitute (pulmonary autograft) [27]. Although its use declined due to its technically demanding nature and late reoperation rates, the Ross procedure can be performed safely and reproducibly at centers of excellence in appropriately selected patients [28]; late dilation of the pulmonary autograft root and sinotubular junction (which may lead to late aortic regurgitation and root aneurysms) can be avoided with autograft-reinforcing/replacement techniques [27-29], and annuli can be reduced and reinforced, thereby allowing long-term durability of the procedure [27,28,30-33]. When performed at experienced and high-volume centers, operative mortality is reported to be 0.3 to 1.1 percent [28].

A growing body of evidence suggests that the Ross procedure is durable and associated with better long-term outcomes compared with conventional aortic valve replacement in young and middle-aged adults [27,28,30-34].

A meta-analysis including 18 studies (17 observational and 1 randomized trial) with a total of 3516 patients with median 5.8 year follow-up found that the Ross procedure was associated with a lower rate of all-cause mortality than mechanical aortic valve replacement (incidence rate ratio [IRR] 0.54, 95% CI 0.35-0.82) [34]. The Ross procedure was also associated with lower rates of stroke (IRR 0.26, 95% CI 0.09-0.80) and major bleeding (IRR 0.17, 95% CI 0.07-0.40), but rates of reintervention were higher (IRR 1.76, 95% CI 1.16-2.65).

In a retrospective multicenter cohort study of 1431 patients (median age 48.5 years, range 18 to 65), including 778 with BAV, who underwent the Ross procedure at five experienced centers from 1991 to 2018, the in-hospital mortality rate was 0.7 percent [32]. Freedom from autograft and homograft reintervention at 15 years was 92 and 97 percent, respectively.

In a retrospective analysis of mandatory California and New York databases from 1997 to 2014, in adults aged 18 to 50 years, propensity matching (1:1:1) produced three groups of 434 patients each with bioprosthetic aortic valve replacement, mechanical aortic valve replacement, and the Ross procedure [33].

At 15 years follow-up, the Ross procedure group survival was similar to that of the sex- and age-matched United States population, while survival for bioprosthetic and mechanical aortic valve replacements was inferior (protective hazard ratios of Ross versus bioprosthetic and mechanical replacements 0.42 and 0.45, respectively).

At 15 years, cumulative incidence of stroke was highest with mechanical aortic valve replacement (4.8 percent), and lower with bioprosthetic replacement (3.3 percent) and the Ross procedure (2.1 percent). Likewise, major bleeding was highest with mechanical replacement (5.2 percent) versus bioprosthetic replacement (3.3 percent) and Ross (1.9 percent).

In contrast, reintervention was the least common with mechanical aortic valve replacement (7.2 percent), intermediate with Ross (17.2 percent), and highest with bioprosthetic replacement (29.8 percent). Interestingly, endocarditis was the highest with bioprosthetic replacement (8.5 percent) and low with mechanical replacement (3.7 percent) and Ross (2.3 percent).

Specific considerations apply for patients with BAV [28,29]:

For patients with BAV who undergo the Ross procedure to treat AR with a dilated aortic valve annulus and dilated root, reinforcement and reduction of the annulus must be performed together with reinforcement of the sinotubular junction to prevent future pulmonary autograft failure resulting in AR.

Patients with BAV with a dilated ascending aorta without evidence of connective tissue disease or heritable thoracic aortic disease may be candidates for the Ross procedure but should undergo prophylactic ascending aorta repair and other surgical modifications at the time of the Ross procedure to stabilize the sinotubular junction and reduce the risk of late pulmonary autograft failure and AR.

Balloon valvotomy — While percutaneous aortic valvotomy is not recommended in the setting of calcific AS in older adults, there is a role for valvuloplasty in selected younger adults. For adults (generally <25 years old) with bicuspid AS with noncalcified valves and no more than mild AR, balloon aortic valvotomy is an alternative option [2,35]. (See "Percutaneous balloon aortic valvotomy for native aortic stenosis in adults" and "Subvalvar aortic stenosis (subaortic stenosis)".)

FOR AORTIC REGURGITATION — Timely aortic valve intervention is indicated for patients with BAV with severe aortic regurgitation (AR) to improve clinical outcomes.

Indications for intervention for AR — Standard recommendations for aortic valve surgery for severe AR (table 2) apply to patients with BAV. (See "Natural history and management of chronic aortic regurgitation in adults", section on 'Indications for aortic valve surgery'.)

Procedures for aortic regurgitation — For patients with BAV requiring surgery for AR, options include surgical aortic valve replacement (SAVR; mechanical or bioprosthetic) and, for selected patients with AR, surgical aortic repair, as described below. (See 'Aortic valve repair' below.)

Among patients with BAV disease, AR is more common in males than females, and affects males at a younger age than females [6]. Patients with BAV are referred with severe AR at a mean age of 46 years versus a mean age of 67 years for patients with tricuspid valve. Therefore, the heart team must plan a lifetime management strategy for these patients, given their young age [36].

Aortic valve replacement — For patients undergoing SAVR, the choice of valve prosthesis (bioprosthetic versus mechanical) is discussed separately. (See "Choice of prosthetic heart valve for surgical aortic or mitral valve replacement".)

When performed at experienced centers, the Ross procedure (pulmonic valve autograft replacing the aortic valve) is an option for carefully selected young and middle-aged patients (generally <50 years old) with BAV AR who have nonrepairable valves, as discussed above. (See 'Ross procedure' above.)

Aortic valve repair — Aortic valve repair is an option for selected cases identified by careful echocardiographic anatomic and functional bicuspid valve assessment at centers where surgical expertise with this procedure is available [10,37-43]. The success and durability of BAV repair for pure AR are good in selected patients with the appropriate anatomy treated at experienced centers with standardized surgical principles and techniques.

The conditions for a successful bicuspid repair for pure AR are:

Valvular conditions – No valvular stenosis, good mobility and systolic excursion of the cusps, sufficient length of cusps, no or minimal calcification of the cusps and or raphe, a symmetric or only mildly asymmetric bicuspid valve, no retraction or perforation of the cusps [5,10].

Surgical conditions – Standardized anatomic surgical principles include correction of prolapse, correction of bicuspid asymmetry, stabilization of enlarged valvular annuli (with suture or ring annuloplasty), stabilization of dilated sinotubular junction (with ascending aorta repair if needed), and no use of pericardial patches [5,10]. Bicuspid valve repair can be performed in isolation but also concomitantly with root and or ascending aortic surgery (see 'For the ascending aorta' below); regardless of whether isolated or with concomitant aorta repair, the surgical principles are the same.

Associations between BAV repair surgical procedures and incidence of reoperation are illustrated by the following studies [44-47]:

With or without aorta repair or replacement The largest BAV repair series included 1024 patients (median age 47 years; 90 percent males) undergoing bicuspid repair with or without concomitant aortic replacement, from a single center, between 1995 and 2018 [45]. Surgical mortality was 0.4 percent. At 10 years, the incidence of reoperation was lower in patients treated according to standardized anatomic surgical principles (8.8 versus 24.6 percent). In addition, 10-year survival was superior in patients who were treated according to standardized anatomic surgical principles (98.7 versus 87.6 percent).

Valve-sparing root replacement with valve reimplantation In a series of 76 patients undergoing valve-sparing root replacement with reimplantation (David) for regurgitant BAV, 10-year freedom from reoperation was 88 percent [46].

Aortic root remodeling In a series of 472 patients who underwent BAV repair and aortic root remodeling (modified Yacoub) from 1995 to 2021, the primary indication for surgery was AR in 67 percent and aortic aneurysm in 30 percent [47]. Freedom from reoperation was 91 percent at 10 years and 77 percent after 20 years. The use of a pericardial patch in cusp repair and cusp calcifications were predictors for reoperation.

FOR THE ASCENDING AORTA

Approach to identifying candidates for aortic surgery — The decision to proceed with aortic surgery (to repair the root [aortic sinuses] or repair the tubular ascending aorta) in a patient with a BAV involves weighing the estimated risk of surgery and the risk of aortic complications without surgery (such as aortic dissection and rupture) based upon root or ascending aorta size and the presence of high-risk features. A shared decision-making approach is required incorporating many relevant clinical features such as the patient's age, BAV dysfunction (AS and/or aortic regurgitation [AR]), aortic size in relation to body size, associated comorbidities, operative risk, overall prognosis, and patient preferences [48-50]. Aortic surgery should be performed by an experienced multidisciplinary aortic team with established expertise in these procedures [4]. (See 'Rationale for aortic size thresholds' below and "Overview of open surgical repair of the thoracic aorta", section on 'Indications for open repair'.)

Indications for aortic surgery — The threshold ascending aortic (root or tubular ascending aorta) diameter for prophylactic surgery depends on whether the patient has high-risk features (see 'High-risk features' below), the patient's operative risk, and whether the patient has a concurrent indication for aortic valve surgery, as shown in the table (table 3). Aortic surgery should be performed by an experienced multidisciplinary aortic team with established expertise in these procedures. (See "Management of thoracic aortic aneurysm in adults", section on 'Summary of indications'.)

The approach to identifying individuals with BAV who are candidates for prophylactic ascending aortic surgery (algorithm 1) is similar to that in major society guidelines [49-55], including the 2016 statement of clarification from the American College of Cardiology/American Heart Association (ACC/AHA) [52], the 2018 American Association for Thoracic Surgery (AATS) consensus guidelines [53], and the 2022 ACC/AHA guidelines for the diagnosis and management of aortic disease [4]. Limited evidence is available to support these recommendations, as discussed below. (See 'Rationale for aortic size thresholds' below.)

As discussed separately, patients with aortic dissection involving the ascending aorta or symptoms suggestive of expansion of a thoracic aneurysm should be evaluated for prompt surgical intervention. (See "Management of acute type B aortic dissection" and "Management of thoracic aortic aneurysm in adults".)

Recommendations for measuring the ascending aorta are discussed separately (see "Bicuspid aortic valve: General management in adults", section on 'Surveillance'). The role for adjustment of aortic size for body size is discussed below. (See 'Role of adjustment for body size' below.)

High-risk features — High-risk features associated with risk of aortic dissection include family history of aortic dissection, aortic growth rate ≥0.3 cm/year, aortic coarctation, and "root phenotype" aortopathy (prominent dilation of the aortic root) [4]. Presence of predominant AR and refractory uncontrolled hypertension may also be considered high-risk features [53]. When one or more high-risk features is present, a lower threshold (smaller ascending aortic size) may be used to trigger prophylactic aortic surgery (table 3).

Rationale for aortic size thresholds

Without high-risk features — Thresholds for prophylactic surgery in patients with nonsyndromic BAV (eg, ≥5.5 cm for patients without high-risk features) are based upon natural history data on the risk of aortic complications (aortic dissection or rupture) in patients with ascending aortic aneurysms regardless of aortic valve morphology, the relatively low risk of aortic surgery in observational studies, and generally good postoperative outcomes [56-60] (see 'Indications for aortic surgery' above). These studies are discussed separately. (See "Management of thoracic aortic aneurysm in adults", section on 'Diameter criteria'.)

Data on the risk of aortic complications in patients with BAV represent the clinical history of the disease (as opposed to the natural history) since most published studies of aortic complications in patients with BAV have included patients treated with elective aortic surgery for aortic dilation (if indicated) [57,58,61-67]. Collectively, these data have demonstrated that the BAV aortopathy has a more benign course than syndromic aortopathy (eg, Marfan, Turner, Loeys Dietz syndromes). Although the incidence of aortic dissection with BAV is higher than in the general population, it remains low in absolute terms when studying the clinical history. This notion has been corroborated by two natural history studies assessing aortic dissection risk before aortic surgery:

An observational study including 554 bicuspid patients and 1874 tricuspid aortic valve (TAV) patients with aneurysms assessed the rates of type A dissection and ascending aortic death before elective repair of the aorta [57]. Incident type A dissection was significantly more common in TAV patients (10 percent) versus BAV patients (1.4 percent). Likewise, ascending aortic death was significantly more common in TAV patients (2.1 percent) versus BAV patients (0.2 percent).

An observational study of 407 naturally occurring acute ascending or descending aortic dissections demonstrated that female sex, hypertension, and smoking were associated with type A dissections at diameters <5.5 cm, while BAV was protective for type A dissections at diameters <5.5 cm (odds ratio 0.3) [60].

Nonetheless, whether to intervene electively with aortic repair in BAV patients with ascending aortas with diameters of 5 to 5.4 cm and no high-risk features requires shared decision making based upon individual characteristics and risk-benefit analysis [4,67-71].

A retrospective multicenter cohort study of 875 consecutive BAV patients (mean age 60, 86 percent men) reported rates of surgical mortality and aortic dissection in 496 patients with ascending aorta diameters of 5 to 5.4 cm during a median of seven years follow-up [67]. During surveillance, 266 of 496 patients (54 percent) underwent elective aortic repair. Aortic dissection occurred in 1.8 percent of unoperated patients for an incidence of 0.4 cases per 100 person-years, and surgical mortality for elective aortic repair was 1.9 percent, suggesting similar rates of these adverse outcomes for surveillance and surgical strategies for BAV patients with ascending diameters 5 to 5.4 cm. Moderate or greater AS was associated with all-cause mortality, highlighting the importance of the valvulo-aortopathy in patients with BAV.

The randomized controlled trial Treatment in Thoracic Aortic Aneurysm: Surgery versus Surveillance (TITAN: SvS [NCT03536312]) is designed to compare mortality rates with early surgery and surveillance in asymptomatic adults (including patients with BAV or TAV) with ascending thoracic aorta diameters of 5 to 5.4 cm [72].

With high risk features and/or low surgical risk — Since aortic dissection can occur at ascending aortic diameters <5.5 cm [73], we suggest prophylactic surgery at lower thresholds (ie, >5 cm) in patients with high-risk features (see 'High-risk features' above) and/or low surgical risk by an experienced surgical team at a center with experience in these procedures. As mentioned above, the aortic root phenotype is a high-risk feature, as it is associated with more rapid growth and higher risk of aortic events, especially after isolated aortic valve replacement [7,74,75]. (See 'Postoperative outcomes' below.)

For elective ascending aorta surgery, some clinicians use <2 percent as a threshold for low operative risk. In the above-cited multicenter study including 496 patients with ascending aorta diameters of 5 to 5.4 cm, aortic dissection occurred in 1.8 percent of unoperated patients (incidence of 0.4 cases per 100 person-years) and surgical mortality was 1.9 percent for elective aortic repair, lending some support to an operative risk threshold of <2 percent [67]. A study of Society of Thoracic Surgeons data for elective aortic root replacement for 2011 to 2016 identified a mean operative mortality of 2.2 percent [76]. Surgical risk is estimated based upon institutional experience as well as patient-specific clinical characteristics and comorbidities (the latter of which may be estimated by risk scores such as the EuroSCORE II).

Indications for prophylactic aortic surgery in patients with thoracic aortic aneurysm without BAV or heritable thoracic aortic disease are discussed separately. (See "Management of thoracic aortic aneurysm in adults".)

Role of adjustment for body size — As discussed above, we suggest including aortic cross-sectional area adjusted for height as a factor to consider in the timing of prophylactic aortic aneurysm surgery for selected individuals with BAV and short stature (see 'Indications for aortic surgery' above). This approach is consistent with a recommendation in the 2022 ACC/AHA aortic disease guidelines [4] and consistent with the international BAV consensus on classification and nomenclature [5].

The risk of thoracic aortic aneurysm rupture and dissection is related not only to aortic size but also to aortic size relative to the patient's body size [77-80]. Correcting absolute aortic diameters for body size may help clarify the need for surgery in patients with BAV, although further verification of the value of aortic diameter indexing is needed.

Some studies in patients with dilated ascending aortas and BAV [77], TAV [79], or unspecified valve morphologies [78] have found that ascending aortic diameter indexed for body size better predicted adverse aortic events than absolute aortic diameter, and indexing methods based on height (as opposed to weight or body surface area) seem to be the most promising [77,78,80]. As an example, a series from the Cleveland Clinic reported a 4.1 percent risk-adjusted probability of acute aortic dissection when the BAV aortic aneurysm was 5 cm at the root and a 3.8 percent risk-adjusted probability of acute aortic dissection when the aneurysm size was 5.3 cm in the ascending aorta [77], corresponding to aortic root or ascending aorta cross-sectional area adjusted for the patient's height of ≥10 cm2/m for the root and ≥13 cm2/m at the level of the tubular ascending aorta. In this study, aortic cross-sectional area to height ratio was a better predictor of aortic dissection than aortic diameter. However, other observational studies in patients with thoracic aortic aneurysms found no additional predictive value from indexing aortic size to body size [58].

Postoperative outcomes — Expected mortality rates and the potential for continued aortic dilation over time are important considerations in management of the ascending aorta. Late complications depend upon the surgical procedure performed: aortic valve intervention without surgery on the aorta, valve-sparing aortic operations, or combined aortic valve replacement and ascending aorta surgery.

A systematic review of 32 observational studies reported outcomes for 9441 adults with BAV (median age 50 years) and 1605 control subjects [68]. The 30-day mortality rate after aortic valve replacement and/or aortic surgery was 0 to 2.5 percent. There were slightly higher rates of bleeding and reexploration in patients undergoing both aortic valve replacement and proximal aortic surgery compared with isolated aortic surgery. Reported long-term outcomes included all-cause mortality and acute aortic event rate (need for subsequent proximal aortic surgery, aortic dissection/rupture, or sudden death not attributable to any other cause) for each of three groups of patients:

For nonoperated BAV patients (ages 30 to 40 years old):

The annualized mortality rate was 0.56 percent (95% CI 0.13-0.99).

The annualized acute aortic event rate was 0.29 percent (95% CI 0.23-0.35), which was not significantly different from the rate for patients with TAV.

For patients who had undergone aortic surgery (generally at ages 40 to 60 years old):

The annualized mortality rate was 0.78 percent (95% CI 0.20-1.36).

The annualized acute aortic event rate was 0.16 percent (95% CI 0-0.32).

For patients who had undergone isolated aortic valve replacement (>60 years old):

The annualized mortality rate was 2.39 percent (95% CI 1.61-3.16).

The annualized acute aortic event rate was 0.68 percent (95% CI 0.42-0.94).

Among patients with BAV undergoing isolated aortic valve replacement, the risk of late aortic complications is greater in patients with larger baseline aortic size, with the aortic root phenotype, or undergoing aortic valve replacement for predominant AR [74,75,81,82], as illustrated by the following studies:

A series of 201 patients (mean age 56 years) with BAV (63 percent had AS, 22 percent had AR, and 15 percent had mixed disease) underwent isolated aortic valve replacement between 1979 and 1993 at Toronto General Hospital [81]. Late aortic aneurysm formation depended upon the aortic size at the time of valve replacement. During mean 10.3-year follow-up, 18 patients (9 percent) required late aortic replacement for aneurysm at mean diameter of 5.8 cm, one had aortic dissection, and three experienced sudden cardiac death. Survival was 67 percent at 15 years, and patients with ascending aorta diameters of 4.5 to 4.9 cm had worse survival. Freedom from ascending aortic complications at 15 years (aneurysm repair, dissection, or sudden death) was 78 percent for initial aortic size <4 cm, 81 percent for aortic size 4 to 4.4 cm, and 43 percent for aortic size 4.5 to 4.9 cm.

A study of 1286 patients (mean age 58 years) with BAV (92 percent with AS, 7 percent with AR) who underwent isolated aortic valve replacement between 1960 and 1995 at the Mayo Clinic reported 12-year median follow-up [82]. Survival was 52 percent at 15 years, which was lower than expected for the age-matched Minnesota population. Freedom from ascending aortic complications was 89 percent at 15 years. Aortic dissection occurred in 13 patients (1 percent), while aortic dilation (aortic diameter >5 cm or >1 cm growth after valve replacement) occurred in 127 (10 percent).

Long-term survival was higher in a later study of 153 BAV patients (mean age 54 years) with AS and ascending aortic dilation of 4 to 5 cm who underwent isolated aortic valve replacement between 1995 and 2000 at the Central Clinic Bad Berka [75]. Actuarial survival was 78 percent for the AS group at 15 years. Freedom from adverse aortic events at 10 and 15 years was 95 and 93 percent, respectively. This study also included outcomes for a separate group of 21 BAV patients (mean age 41 years) with aortic valve regurgitation and predominant dilation of the aortic root (root phenotype) who underwent isolated aortic valve replacement. In this group, the freedom from adverse aortic events at 10 and 15 years was 88 and 70 percent, respectively. Thus, patients with the root phenotype were at increased risk for adverse aortic events, as has also been suggested by other observations.

MONITORING AFTER AORTIC VALVE OR AORTIC INTERVENTION

After aortic valve intervention — In patients with BAV who have undergone aortic valve intervention (repair or replacement) and have a diameter of the aortic root, ascending aorta, or both of ≥4 cm, lifelong surveillance of the aortic root and ascending aorta by transthoracic echocardiography, CT, or cardiovascular magnetic resonance (CMR) imaging is recommended at an interval dependent upon aortic diameter and rate of growth [4] (see "Bicuspid aortic valve: General management in adults", section on 'Surveillance'). Patients with BAV who have undergone aortic valve intervention continue to be at risk for aortic complications including aortic dissection/rupture and ascending aortic aneurysm formation and thus may require subsequent aortic surgery.

Serial monitoring of prosthetic valves is discussed separately. (See "Overview of the management of patients with prosthetic heart valves", section on 'Serial follow-up'.)

Patients who have undergone aortic valve repair should be followed postoperatively with yearly echocardiograms as recommended in the 2013 Society of Thoracic Surgeons guidelines [37].

Management and complications after transcatheter aortic valve implantation are discussed separately. (See "Transcatheter aortic valve implantation: Complications" and "Transcatheter aortic valve implantation: Periprocedural and postprocedural management".)

After ascending aorta repair — Surgical repairs of the ascending aorta are generally durable, with most anastomotic problems occurring early in the postoperative period. Thus, postoperative imaging is generally required only if a problem is suspected. If there is residual aortic dilation or other pathology, imaging of the thoracic aorta every three to five years after aortic surgery by CMR or CT is suggested [53]. However, many clinicians will routinely obtain CMR or CT scans pre-hospital discharge, at six months follow-up, and then at three- to five-year intervals to look for proximal or distal anastomotic pseudoaneurysms. (See "Management of thoracic aortic aneurysm in adults".)

Serial imaging following surgical or endovascular intervention for aortic dissection is discussed separately. (See "Management of acute type A aortic dissection", section on 'Serial imaging' and "Management of acute type B aortic dissection", section on 'Surveillance imaging'.)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Bicuspid aortic valve" and "Society guideline links: Cardiac valve disease" and "Society guideline links: Aortic dissection and other acute aortic syndromes" and "Society guideline links: Congenital heart disease in adults".)

SUMMARY AND RECOMMENDATIONS

Rationale for intervention – Indications for intervention for valvulopathy and aortopathy in adults with bicuspid aortic valve (BAV) have been developed to provide timely treatment to reduce the risk of adverse outcomes from uncorrected progressive disease while managing the risk associated with intervention. (See 'Rationale for intervention' above.)

Types of intervention – The choice of intervention is based on whether the patient with BAV requires aortic valve intervention, repair of the aorta, or both. (See 'Types of intervention' above.)

Aortic valve interventions - Standard recommendations for aortic valve intervention for severe aortic stenosis (AS) and for severe aortic regurgitation (AR) apply to patients with BAV. (See 'For aortic stenosis' above and 'For aortic regurgitation' above.)

Procedures for AS – For patients with severe AS requiring valve intervention, the main options are surgical aortic valve replacement (SAVR; mechanical or bioprosthetic) and transcatheter aortic valve intervention (TAVI). At experienced centers, the Ross procedure is an option for selected young and middle-aged patients with BAV. For selected patients (generally <25 years of age) with noncalcified valves and no more than mild AR, balloon aortic valvotomy is an alternative option. (See 'Procedures for aortic stenosis' above.)

Procedures for AR – For patients with BAV requiring surgery for AR, options include SAVR (mechanical or bioprosthetic) and, for selected patients, surgical aortic repair by experienced surgical teams. The Ross procedure is also an option for selected young and middle-aged patients with BAV AR with a nonrepairable valve. (See 'Procedures for aortic regurgitation' above.)

Aortic interventions – The decision on whether to proceed with prophylactic ascending aortic surgery in a patient with BAV is based upon weighing the estimated risk of surgery and the risk of progressive aortic disease. The threshold ascending aortic (root or tubular ascending aorta) diameter for prophylactic surgery depends on whether the patient has high-risk features, the patient's operative risk, and whether the patient has a concurrent indication for aortic valve surgery, as shown in the table (table 3). Aortic surgery should be performed by an experienced multidisciplinary aortic team with established expertise in these procedures. (See "Management of thoracic aortic aneurysm in adults", section on 'Summary of indications'.)

Monitoring after surgery

After aortic valve intervention – The patient with BAV who has undergone isolated aortic valve intervention should continue routine imaging surveillance of the aortic root and ascending aorta after surgery. (See 'After aortic valve intervention' above and "Bicuspid aortic valve: General management in adults", section on 'Surveillance'.)

After aorta repair – For patients who have undergone ascending aorta repair, routine postoperative imaging is indicated if there is residual aortic dilation or other aortic pathology (eg, every three to five years). Some clinicians obtain routine imaging after all aortic repairs to identify anastomotic aneurysms. (See 'After ascending aorta repair' above.)

ACKNOWLEDGMENTS — The editorial staff at UpToDate acknowledges Martin G Keane, MD, FACC, FAHA, FASE, Thomas P Graham, MD, Catherine M Otto, MD, and Candice Silversides, MD, MS, FRCPC, who contributed to earlier versions of this topic review.

  1. Michelena HI, Prakash SK, Della Corte A, et al. Bicuspid aortic valve: identifying knowledge gaps and rising to the challenge from the International Bicuspid Aortic Valve Consortium (BAVCon). Circulation 2014; 129:2691.
  2. Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation 2021; 143:e72.
  3. Vahanian A, Praz F, Milojevic M, Beyersdorf F. The "ten commandments" for the 2021 ESC/EACTS Guidelines on valvular heart disease. Eur Heart J 2021; 42:4207.
  4. Isselbacher EM, Preventza O, Hamilton Black J 3rd, et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Circulation 2022; 146:e334.
  5. Michelena HI, Della Corte A, Evangelista A, et al. International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes. J Thorac Cardiovasc Surg 2021; 162:e383.
  6. Yang LT, Ye Z, Wajih Ullah M, et al. Bicuspid aortic valve: long-term morbidity and mortality. Eur Heart J 2023; 44:4549.
  7. Etz CD, Haunschild J, Girdauskas E, et al. Surgical management of the aorta in BAV patients. Prog Cardiovasc Dis 2020; 63:475.
  8. Ouzounian M, Feindel CM, Manlhiot C, et al. Valve-sparing root replacement in patients with bicuspid versus tricuspid aortic valves. J Thorac Cardiovasc Surg 2019; 158:1.
  9. Lansac E, Di Centa I, Sleilaty G, et al. Long-term results of external aortic ring annuloplasty for aortic valve repair. Eur J Cardiothorac Surg 2016; 50:350.
  10. Ehrlich T, de Kerchove L, Vojacek J, et al. State-of-the art bicuspid aortic valve repair in 2020. Prog Cardiovasc Dis 2020; 63:457.
  11. Yang LT, Boler A, Medina-Inojosa JR, et al. Aortic Stenosis Progression, Cardiac Damage, and Survival: Comparison Between Bicuspid and Tricuspid Aortic Valves. JACC Cardiovasc Imaging 2021; 14:1113.
  12. Bauer T, Linke A, Sievert H, et al. Comparison of the effectiveness of transcatheter aortic valve implantation in patients with stenotic bicuspid versus tricuspid aortic valves (from the German TAVI Registry). Am J Cardiol 2014; 113:518.
  13. Costopoulos C, Latib A, Maisano F, et al. Comparison of results of transcatheter aortic valve implantation in patients with severely stenotic bicuspid versus tricuspid or nonbicuspid valves. Am J Cardiol 2014; 113:1390.
  14. Kochman J, Huczek Z, Scisło P, et al. Comparison of one- and 12-month outcomes of transcatheter aortic valve replacement in patients with severely stenotic bicuspid versus tricuspid aortic valves (results from a multicenter registry). Am J Cardiol 2014; 114:757.
  15. Hayashida K, Bouvier E, Lefèvre T, et al. Transcatheter aortic valve implantation for patients with severe bicuspid aortic valve stenosis. Circ Cardiovasc Interv 2013; 6:284.
  16. Phan K, Wong S, Phan S, et al. Transcatheter Aortic Valve Implantation (TAVI) in Patients With Bicuspid Aortic Valve Stenosis--Systematic Review and Meta-Analysis. Heart Lung Circ 2015; 24:649.
  17. Mylotte D, Lefevre T, Søndergaard L, et al. Transcatheter aortic valve replacement in bicuspid aortic valve disease. J Am Coll Cardiol 2014; 64:2330.
  18. Yoon SH, Bleiziffer S, De Backer O, et al. Outcomes in Transcatheter Aortic Valve Replacement for Bicuspid Versus Tricuspid Aortic Valve Stenosis. J Am Coll Cardiol 2017; 69:2579.
  19. Yoon SH, Lefèvre T, Ahn JM, et al. Transcatheter Aortic Valve Replacement With Early- and New-Generation Devices in Bicuspid Aortic Valve Stenosis. J Am Coll Cardiol 2016; 68:1195.
  20. Makkar RR, Yoon SH, Leon MB, et al. Association Between Transcatheter Aortic Valve Replacement for Bicuspid vs Tricuspid Aortic Stenosis and Mortality or Stroke. JAMA 2019; 321:2193.
  21. Deeb GM, Reardon MJ, Ramlawi B, et al. Propensity-Matched 1-Year Outcomes Following Transcatheter Aortic Valve Replacement in Low-Risk Bicuspid and Tricuspid Patients. JACC Cardiovasc Interv 2022; 15:511.
  22. Williams MR, Jilaihawi H, Makkar R, et al. The PARTNER 3 Bicuspid Registry for Transcatheter Aortic Valve Replacement in Low-Surgical-Risk Patients. JACC Cardiovasc Interv 2022; 15:523.
  23. Makkar RR, Yoon SH, Chakravarty T, et al. Association Between Transcatheter Aortic Valve Replacement for Bicuspid vs Tricuspid Aortic Stenosis and Mortality or Stroke Among Patients at Low Surgical Risk. JAMA 2021; 326:1034.
  24. Halim SA, Edwards FH, Dai D, et al. Outcomes of Transcatheter Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Disease: A Report From the Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy Registry. Circulation 2020; 141:1071.
  25. Yoon SH, Kim WK, Dhoble A, et al. Bicuspid Aortic Valve Morphology and Outcomes After Transcatheter Aortic Valve Replacement. J Am Coll Cardiol 2020; 76:1018.
  26. Forrest JK, Ramlawi B, Deeb GM, et al. Transcatheter Aortic Valve Replacement in Low-risk Patients With Bicuspid Aortic Valve Stenosis. JAMA Cardiol 2021; 6:50.
  27. Skillington PD, Mokhles MM, Takkenberg JJ, et al. The Ross procedure using autologous support of the pulmonary autograft: techniques and late results. J Thorac Cardiovasc Surg 2015; 149:S46.
  28. Mazine A, El-Hamamsy I, Verma S, et al. Ross Procedure in Adults for Cardiologists and Cardiac Surgeons: JACC State-of-the-Art Review. J Am Coll Cardiol 2018; 72:2761.
  29. Mazine A, El-Hamamsy I. The Ross procedure is an excellent operation in non-repairable aortic regurgitation: insights and techniques. Ann Cardiothorac Surg 2021; 10:463.
  30. Mazine A, David TE, Rao V, et al. Long-Term Outcomes of the Ross Procedure Versus Mechanical Aortic Valve Replacement: Propensity-Matched Cohort Study. Circulation 2016; 134:576.
  31. Ouzounian M, Mazine A, David TE. The Ross procedure is the best operation to treat aortic stenosis in young and middle-aged adults. J Thorac Cardiovasc Surg 2017; 154:778.
  32. Romeo JLR, Papageorgiou G, da Costa FFD, et al. Long-term Clinical and Echocardiographic Outcomes in Young and Middle-aged Adults Undergoing the Ross Procedure. JAMA Cardiol 2021; 6:539.
  33. El-Hamamsy I, Toyoda N, Itagaki S, et al. Propensity-Matched Comparison of the Ross Procedure and Prosthetic Aortic Valve Replacement in Adults. J Am Coll Cardiol 2022; 79:805.
  34. Mazine A, Rocha RV, El-Hamamsy I, et al. Ross Procedure vs Mechanical Aortic Valve Replacement in Adults: A Systematic Review and Meta-analysis. JAMA Cardiol 2018; 3:978.
  35. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019; 73:e81.
  36. Yang LT, Benfari G, Eleid M, et al. Contemporary differences between bicuspid and tricuspid aortic valve in chronic aortic regurgitation. Heart 2021; 107:916.
  37. Svensson LG, Adams DH, Bonow RO, et al. Aortic valve and ascending aorta guidelines for management and quality measures. Ann Thorac Surg 2013; 95:S1.
  38. Kari FA, Siepe M, Sievers HH, Beyersdorf F. Repair of the regurgitant bicuspid or tricuspid aortic valve: background, principles, and outcomes. Circulation 2013; 128:854.
  39. Svensson LG, Al Kindi AH, Vivacqua A, et al. Long-term durability of bicuspid aortic valve repair. Ann Thorac Surg 2014; 97:1539.
  40. Bavaria JE, Desai N, Szeto WY, et al. Valve-sparing root reimplantation and leaflet repair in a bicuspid aortic valve: comparison with the 3-cusp David procedure. J Thorac Cardiovasc Surg 2015; 149:S22.
  41. Schneider U, Feldner SK, Hofmann C, et al. Two decades of experience with root remodeling and valve repair for bicuspid aortic valves. J Thorac Cardiovasc Surg 2017; 153:S65.
  42. Kari FA, Kvitting JP, Stephens EH, et al. Tirone David procedure for bicuspid aortic valve disease: impact of root geometry and valve type on mid-term outcomes. Interact Cardiovasc Thorac Surg 2014; 19:375.
  43. Arnaoutakis GJ, Sultan I, Siki M, Bavaria JE. Bicuspid aortic valve repair: systematic review on long-term outcomes. Ann Cardiothorac Surg 2019; 8:302.
  44. Patlolla SH, Schaff HV, Stulak JM, et al. Bicuspid Aortic Valve Repair: Causes of Valve Failure and Long-Term Outcomes. Ann Thorac Surg 2021; 111:1225.
  45. Schneider U, Hofmann C, Schöpe J, et al. Long-term Results of Differentiated Anatomic Reconstruction of Bicuspid Aortic Valves. JAMA Cardiol 2020; 5:1366.
  46. Mastrobuoni S, de Kerchove L, Navarra E, et al. Long-term experience with valve-sparing reimplantation technique for the treatment of aortic aneurysm and aortic regurgitation. J Thorac Cardiovasc Surg 2019; 158:14.
  47. Froede L, Abeln KB, Ehrlich T, et al. Twenty-five years' experience with root remodeling and bicuspid aortic valve repair. Ann Cardiothorac Surg 2022; 11:418.
  48. Braverman AC. Guidelines for management of bicuspid aortic valve aneurysms: what's the clinician to do? Curr Opin Cardiol 2014; 29:489.
  49. Adamo L, Braverman AC. Surgical threshold for bicuspid aortic valve aneurysm: a case for individual decision-making. Heart 2015; 101:1361.
  50. Braverman AC. Aortic replacement for bicuspid aortic valve aortopathy: When and why? J Thorac Cardiovasc Surg 2019; 157:520.
  51. Erbel R, Aboyans V, Boileau C, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J 2014; 35:2873.
  52. Hiratzka LF, Creager MA, Isselbacher EM, et al. Surgery for Aortic Dilatation in Patients With Bicuspid Aortic Valves: A Statement of Clarification From the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2016; 67:724.
  53. Borger MA, Fedak PWM, Stephens EH, et al. The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy: Full online-only version. J Thorac Cardiovasc Surg 2018; 156:e41.
  54. Boodhwani M, Andelfinger G, Leipsic J, et al. Canadian Cardiovascular Society position statement on the management of thoracic aortic disease. Can J Cardiol 2014; 30:577.
  55. Baumgartner H, Falk V, Bax JJ, et al. 2017 ESC/EACTS Guidelines for the management of valvular heart disease. Eur Heart J 2017; 38:2739.
  56. Davies RR, Kaple RK, Mandapati D, et al. Natural history of ascending aortic aneurysms in the setting of an unreplaced bicuspid aortic valve. Ann Thorac Surg 2007; 83:1338.
  57. Zafar MA, Wu J, Vinholo TF, et al. Bicuspid aortopathy does not require earlier surgical intervention. J Thorac Cardiovasc Surg 2023.
  58. Solomon MD, Leong T, Sung SH, et al. Association of Thoracic Aortic Aneurysm Size With Long-term Patient Outcomes: The KP-TAA Study. JAMA Cardiol 2022; 7:1160.
  59. Wu J, Zafar MA, Liu Y, et al. Fate of the unoperated ascending thoracic aortic aneurysm: three-decade experience from the Aortic Institute at Yale University. Eur Heart J 2023; 44:4579.
  60. Perez ZG, Zafar MA, Velasco JJ, et al. Aortic Size at the Time of Type A and Type B Dissections. Ann Thorac Surg 2023; 116:262.
  61. Michelena HI, Desjardins VA, Avierinos JF, et al. Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community. Circulation 2008; 117:2776.
  62. Michelena HI, Khanna AD, Mahoney D, et al. Incidence of aortic complications in patients with bicuspid aortic valves. JAMA 2011; 306:1104.
  63. Weinsaft JW, Devereux RB, Preiss LR, et al. Aortic Dissection in Patients With Genetically Mediated Aneurysms: Incidence and Predictors in the GenTAC Registry. J Am Coll Cardiol 2016; 67:2744.
  64. Itagaki S, Chikwe JP, Chiang YP, et al. Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan Syndrome. J Am Coll Cardiol 2015; 65:2363.
  65. Yang LT, Tribouilloy C, Masri A, et al. Clinical presentation and outcomes of adults with bicuspid aortic valves: 2020 update. Prog Cardiovasc Dis 2020; 63:434.
  66. Masri A, Svensson LG, Griffin BP, Desai MY. Contemporary natural history of bicuspid aortic valve disease: a systematic review. Heart 2017; 103:1323.
  67. Ye Z, Lane CE, Beachey JD, et al. Clinical outcomes in patients with bicuspid aortic valves and ascending aorta ≥50 mm under surveillance. JACC Adv 2023; 2:100626.
  68. Hardikar AA, Marwick TH. Surgical thresholds for bicuspid aortic valve associated aortopathy. JACC Cardiovasc Imaging 2013; 6:1311.
  69. Girdauskas E, Disha K, Borger MA, Kuntze T. Risk of proximal aortic dissection in patients with bicuspid aortic valve: how to address this controversy? Interact Cardiovasc Thorac Surg 2014; 18:355.
  70. Kallenbach K, Sundt TM, Marwick TH. Aortic surgery for ascending aortic aneurysms under 5.0 cm in diameter in the presence of bicuspid aortic valve. JACC Cardiovasc Imaging 2013; 6:1321.
  71. Verma S, Siu SC. Aortic dilatation in patients with bicuspid aortic valve. N Engl J Med 2014; 370:1920.
  72. Guo MH, Appoo JJ, Wells GA, et al. Protocol for a randomised controlled trial for Treatment in Thoracic Aortic Aneurysm: Surgery versus Surveillance (TITAN: SvS). BMJ Open 2021; 11:e052070.
  73. Pape LA, Tsai TT, Isselbacher EM, et al. Aortic diameter >or = 5.5 cm is not a good predictor of type A aortic dissection: observations from the International Registry of Acute Aortic Dissection (IRAD). Circulation 2007; 116:1120.
  74. Della Corte A, Bancone C, Dialetto G, et al. The ascending aorta with bicuspid aortic valve: a phenotypic classification with potential prognostic significance. Eur J Cardiothorac Surg 2014; 46:240.
  75. Girdauskas E, Disha K, Raisin HH, et al. Risk of late aortic events after an isolated aortic valve replacement for bicuspid aortic valve stenosis with concomitant ascending aortic dilation. Eur J Cardiothorac Surg 2012; 42:832.
  76. Wallen T, Habertheuer A, Bavaria JE, et al. Elective Aortic Root Replacement in North America: Analysis of STS Adult Cardiac Surgery Database. Ann Thorac Surg 2019; 107:1307.
  77. Wojnarski CM, Svensson LG, Roselli EE, et al. Aortic Dissection in Patients With Bicuspid Aortic Valve-Associated Aneurysms. Ann Thorac Surg 2015; 100:1666.
  78. Davies RR, Gallo A, Coady MA, et al. Novel measurement of relative aortic size predicts rupture of thoracic aortic aneurysms. Ann Thorac Surg 2006; 81:169.
  79. Masri A, Kalahasti V, Svensson LG, et al. Aortic Cross-Sectional Area/Height Ratio and Outcomes in Patients With a Trileaflet Aortic Valve and a Dilated Aorta. Circulation 2016; 134:1724.
  80. Zafar MA, Li Y, Rizzo JA, et al. Height alone, rather than body surface area, suffices for risk estimation in ascending aortic aneurysm. J Thorac Cardiovasc Surg 2018; 155:1938.
  81. Borger MA, Preston M, Ivanov J, et al. Should the ascending aorta be replaced more frequently in patients with bicuspid aortic valve disease? J Thorac Cardiovasc Surg 2004; 128:677.
  82. McKellar SH, Michelena HI, Li Z, et al. Long-term risk of aortic events following aortic valve replacement in patients with bicuspid aortic valves. Am J Cardiol 2010; 106:1626.
Topic 129707 Version 8.0

References

آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟