Updated 2022 ACC/AHA Guideline Improves Concordance Between TTE and CT in Monitoring Marfan Syndrome and Related Disorders, but Relevant Measurement Differences Remain Frequent

Abstract

Background: Patients diagnosed with Marfan syndrome or a related syndrome require frequent aorta monitoring using imaging techniques like transthoracic echocardiography (TTE) and computed tomography (CT). Accurate aortic measurement is crucial, as even slight enlargement (>2 mm) often necessitates surgical intervention. The 2022 ACC/AHA guideline for Aortic Disease Diagnosis and Management includes updated imaging recommendations. We aimed to compare these with the 2010 guideline. Methods: This retrospective study involved 137 patients with Marfan syndrome or a related disorder, undergoing TTE and ECG-triggered CT. Aortic diameter measurements were taken based on the old 2010 guideline (TTE: inner edge to inner edge, CT: external diameter) and the new 2022 guideline (TTE: leading edge to leading edge, CT: internal diameter). Bland-Altman plots compared measurement differences. Results: Using the 2022 guideline significantly reduced differences outside the clinical agreement limit from 49% to 26% for the aortic sinus and from 41% to 29% for the ascending aorta. Mean differences were –0.30 mm for the aortic sinus and +1.12 mm for the ascending aorta using the 2022 guideline, compared to –2.66 mm and +1.21 mm using the 2010 guideline. Conclusion: This study demonstrates for the first time that the 2022 ACC/AHA guideline improves concordance between ECG-triggered CT and TTE measurements in Marfan syndrome patients, crucial for preventing life-threatening aortic complications. However, the frequency of differences >2 mm remains high. Clinical Relevance/Application: Accurate aortic diameter measurement is vital for patients at risk of fatal aortic complications. While the 2022 guideline enhances concordance between imaging modalities, frequent differences >2 mm persist, potentially impacting decisions on aortic repair. The risk of repeat radiation exposure from ECG-triggered CT, considered the ‘gold standard’, continues to be justified.

Keywords

• marfan syndrome and related disorders
• monitoring aortic enlargement
• computed tomography
• transthoracic echocardiography