What are the next steps for a patient with a dilated aortic (Aorta) root, ascending aorta, and pulmonary artery?

From the Guidelines

For a patient with a dilated aortic root, ascending aorta, and pulmonary artery, immediate referral to a cardiologist or cardiothoracic surgeon for specialized assessment is crucial, and surgical intervention should be considered when the aortic diameter reaches 5.0cm or greater, as recommended by the 2022 ACC/AHA guideline for the diagnosis and management of aortic disease 1. The next steps should include comprehensive cardiovascular evaluation and management, with a focus on reducing hemodynamic stress on the vessel walls. Medical management should begin with blood pressure control using beta-blockers (such as metoprolol 25-100mg twice daily or propranolol 10-40mg three times daily) to reduce the risk of aortic dissection or rupture, as supported by the 2022 aortic disease guideline-at-a-glance 1. Some key considerations for management include:

• Regular imaging surveillance with echocardiography every 6-12 months, depending on the degree of dilation and rate of progression
• CT or MRI angiography to provide more detailed assessment, performed annually or biannually
• Lifestyle modifications, including strict blood pressure control, avoidance of strenuous isometric exercise, smoking cessation, and weight management
• Consideration of surgical intervention when the aortic diameter reaches 5.0-5.5cm for most patients, or 4.5-5.0cm for those with genetic aortopathies, as recommended by the 2022 ACC/AHA guideline 1. It is essential to note that the size threshold for elective surgery to replace the dilated aortic root in Marfan syndrome is dependent on many factors, including the patient’s age, height and weight, family history, rate of aortic growth, and other patient-specific factors, as highlighted in the 2022 ACC/AHA guideline 1. The patient should be managed by a Multidisciplinary Aortic Team, with shared decision-making involving the patient and the team to determine the optimal medical, endovascular, and open surgical therapies, as recommended by the 2022 aortic disease guideline-at-a-glance 1.

From the Research

Next Steps for Patients with Dilated Aortic Root, Ascending Aorta, and Pulmonary Artery

• The patient's condition requires careful evaluation and consideration of various factors, including the size of the aorta, underlying pathology, and the patient's overall health 2, 3.
• Surgical intervention is often necessary to prevent complications such as dissection or rupture, and the choice of procedure depends on the patient's specific condition and the surgeon's experience 2, 3.
• For patients with a dilated aortic root, intervention is typically recommended when the diameter exceeds 5.5 cm, although some studies suggest that intervention may be necessary at a smaller diameter, especially in patients with connective tissue disorders such as Marfan syndrome 2, 4.
• The natural histories of the aortic root and mid-ascending aorta are uniquely different, and dilation of the aortic root imparts a significant higher risk of adverse events 4.
• A diameter shift for intervention to 5.0 cm for the aortic root and to 5.25 cm for the mid-ascending aorta should be considered at expert centers 4.
• In pediatric patients, repair of the aortic root and/or ascending aorta can be performed with acceptable early and late results, although the presence of severe comorbidity may adversely affect early outcome 5.
• The use of angiotensin receptor blockers (ARBs) such as irbesartan may be beneficial in reducing the rate of aortic dilatation in patients with Marfan syndrome, although further research is needed to confirm this 6.

Surgical Options

• Composite replacement of the aortic valve and the ascending aorta, as originally described by Bentall, DeBono and Edwards, or modified by Kouchoukos, remains a widely applied method 3.
• Valve-sparing root reconstructive options are also available and may be considered in certain cases 2.
• The choice of surgical procedure depends on various factors, including the patient's age, underlying aortic pathology, and the surgeon's experience 2, 3.

Monitoring and Follow-up

• Regular monitoring of the patient's condition, including echocardiography and other imaging studies, is necessary to assess the progression of the disease and the effectiveness of treatment 2, 4.
• Patients with a dilated aortic root, ascending aorta, and pulmonary artery require close follow-up and may need to undergo repeated surgical interventions to prevent complications 5, 3.