What does a tortuous aorta indicate?

From the Guidelines

A tortuous aorta indicates an elongated and twisted aorta, which is often a normal age-related change rather than a disease, and may be associated with various genetic syndromes, such as Loeys-Dietz syndrome, which is characterized by arterial tortuosity, aneurysms, and dissections, as noted in the 2022 ACC/AHA guideline for the diagnosis and management of aortic disease 1. This condition typically develops as people age due to the natural loss of elasticity in the aortic wall, causing the vessel to become more elongated and curved. While a tortuous aorta is generally benign and requires no specific treatment, it may occasionally cause symptoms such as chest discomfort, difficulty swallowing, or shortness of breath if it compresses surrounding structures. The condition is more common in elderly individuals and those with hypertension, atherosclerosis, or connective tissue disorders. Regular monitoring through imaging studies may be recommended if the tortuosity is significant, but intervention is rarely needed unless complications develop. Some key points to consider in the management of a tortuous aorta include:

• The importance of regular imaging surveillance to monitor the size and shape of the aorta, as well as the presence of any associated aneurysms or dissections, as recommended in the 2022 ACC/AHA guideline for the diagnosis and management of aortic disease 1.
• The need for a comprehensive evaluation of patients with a tortuous aorta, including a thorough medical history, physical examination, and laboratory tests, to identify any underlying genetic syndromes or other conditions that may be contributing to the development of the tortuosity, as noted in the 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease 1.
• The potential benefits and risks of various treatment options, including surgical repair, endovascular repair, and medical management, and the importance of individualizing treatment decisions based on the specific needs and circumstances of each patient, as discussed in the 2022 ACC/AHA guideline for the diagnosis and management of aortic disease 1. The underlying mechanism involves gradual structural changes in the aortic wall, including fragmentation of elastic fibers and increased collagen deposition, which reduce the vessel's ability to maintain its normal straight configuration against the pressure of blood flow, as noted in the example answer. In addition to these factors, it is also important to consider the potential role of genetic syndromes, such as Loeys-Dietz syndrome, in the development of a tortuous aorta, and to provide appropriate counseling and management for patients with these conditions, as recommended in the 2022 ACC/AHA guideline for the diagnosis and management of aortic disease 1. Overall, the management of a tortuous aorta requires a comprehensive and individualized approach, taking into account the specific needs and circumstances of each patient, and incorporating the latest evidence and guidelines from the medical literature, including the 2022 ACC/AHA guideline for the diagnosis and management of aortic disease 1.

From the Research

Indications of a Tortuous Aorta

A tortuous aorta can indicate several conditions, including:

• Genetic arteriopathies, such as Loeys-Dietz syndrome and arterial tortuosity syndrome 2
• Turner syndrome, which is associated with aortic coarctation, dissection, and dilation/aneurysm 3
• Increased risk of adverse cardiovascular events, including aortic surgery, aortic dissection, and death 2, 4
• Aortic dilation or aneurysm, as tortuosity is often present in patients with these conditions 2, 4

Association with Aortic Dissection

A tortuous aorta has been linked to an increased risk of aortic dissection, particularly in patients with:

• Type A aortic dissection (ATAAD), where tortuosity is strongly correlated with maximum diameter of the ascending aorta 4
• Type B aortic dissection (TBAD), where endovascular therapies may be used to prevent late aneurysmal degeneration and improve outcomes 5

Clinical Implications

The presence of a tortuous aorta can have significant clinical implications, including:

• Increased risk of endoleaks and stroke in patients undergoing thoracic endovascular aortic repair (TEVAR) 6
• Lower survival rates in patients with high tortuosity of the thoracic aorta 6
• Need for closer monitoring and follow-up examinations in patients with highly tortuous aortas 6