p.38 抽出テキスト
Otto et al 2020 ACC/AHA Guideline for the Management of Valvular Heart Disease Circulation. 2021;143:e72–e227. DOI: 10.1161/CIR.0000000000000923 February 2, 2021 e109 CLINICAL STATEMENTS AND GUIDELINES 5 BICUSPID AORTIC VALVE 5.1 BAV and Associated Aortopathy 5.1.1 Diagnosis and Follow-Up of BAV 5.1.1.1. Diagnostic Testing: Initial Diagnosis Recommendations for Diagnostic Testing: Initial Diagnosis of BAV Referenced studies that support the recommendations are summarized in Online Data Supplement 18. COR LOE Recommendations 1 B-NR 1. In patients with a known BAV, TTE is indicated to evaluate valve morphology, measure severity of AS and AR, assess the shape and diameter of the aortic sinuses and ascending aorta, and evaluate for the presence of aortic coarctation for prediction of clinical outcome and to determine timing of intervention.1–4 1 C-LD 2. In patients with BAV, CMR angiography or CT angiography is indicated when morphology of the aortic sinuses, sinotubular junction, or ascending aorta cannot be assessed accurately or fully by echocardiography.4,5 2b B-NR 3. In first-degree relatives of patients with a known BAV, a screening TTE might be considered to look for the presence of a BAV or asymptomatic dilation of the aortic sinuses and ascending aorta.6 Synopsis BAV is a common congenital anomaly that affects 0.5% to 2.0% of adults with a 3:1 male-to-female predominance.1 Patients with BAV may develop isolated aortic valve disease, including isolated AR, AS, or a combination of the two. Aortic aneurysms have been reported in 20% to 40% of patients with BAV.1 This aortopathy can occur independent of valve function and consists of dilation of the aortic sinuses, the ascending aorta, or the arch. Therefore, patients with BAV require careful evaluation of both the aortic valve and the aorta throughout their lifetimes (Figure 5). Recommendation-Specific Supportive Text 1. Many patients with BAV will develop AS or AR over their lifetimes. In a recent meta-analysis of natural history studies of patients with BAV, 13% to 30% of patients developed moderate or greater AR and 12% to 37% developed moderate or greater AS during follow-up.1 TTE usually is adequate for evaluation of aortic valve anatomy and hemodynamics. TEE provides improved 2D and 3D images if needed. Aortic enlargement at the level of the sinuses or proximal ascending aorta has been reported in 20% to 40% of patients with BAV,1 and some develop severe aneurysmal dilation and are at increased risk of aortic dissection.2,3,7–10 Aortic measurements are reported at the aortic annulus, sinuses of Valsalva, sinotubular junction, and mid-ascending aorta. Doppler interrogation of the proximal descending aorta and abdominal aorta should also be performed to evaluate for the presence of aortic coarctation, which is associated with BAV in a subset of patients, although a coarctation also can be detected by comparing arm and leg blood pressures. 2. CT angiography or CMR provides better images of the aortic sinuses, sinotubular junction, or ascending aorta when TTE does not adequately visualize the sinus and proximal 5 to 6 cm of the ascending aorta. The choice of CMR versus CT angiography depends on patient preference, insurance coverage, institutional expertise, and consideration of radiation exposure. 3. In about 20% to 30% of patients with a BAV, other family members also have a BAV and/or an associated aortopathy. A specific genetic cause has not been identified, and the patterns of inheritance are variable. Imaging can identify the presence of a BAV and aortic dilation, but there is a paucity of data on the cost-effectiveness of this approach and whether earlier diagnosis would improve long-term clinical outcomes.6,11 5.1.1.2 Diagnostic Testing: Routine Follow-Up Recommendations for Diagnostic Testing: Routine Follow-Up of Patients With a BAV Referenced studies that support the recommendations are summarized in Online Data Supplement 18. COR LOE Recommendations 2a C-LD 1. In patients with BAV and a diameter of the aortic sinuses or ascending aorta of ≥4.0 cm, lifelong serial evaluation of the size and morphology of the aortic sinuses and ascending aorta by echocardiography, CMR, or CT angiography is reasonable, with the examination interval determined by the degree and rate of progression of aortic dilation and by family history.1–5 2a B-NR 2. In patients with a BAV who have undergone AVR, continued lifelong serial interval imaging of the aorta is reasonable if the diameter of the aortic sinuses or ascending aorta is ≥4.0 cm.6,7 Synopsis Patients with BAV with and without associated aortic aortopathy require lifelong surveillance. Because progression of valve disease and growth of the aorta can occur in the absence of symptoms, diagnostic imaging plays an integral role in the surveillance process. Recommendation-Specific Supportive Text 1. Aortopathy is present in approximately 20% to 40% of patients with a BAV and is associated with dilation of the aortic sinuses, the ascending aorta, and/or the arch.1 In a retrospective case series of 918 patients with BAV followed for 2 to 12 years Downloaded from http://ahajournals.org by on October 7, 2026