Medical Treatment of Thoracic Aortic Pathologies
Thoracic aortic aneurysm disease is an important cause of morbidity and mortality. Recognizing the specific disorder responsible for the condition informs management, including screening of first-degree relatives, imaging strategies, and determining the t
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Alan C. Braverman
14.1 Introduction Thoracic aortic aneurysm (TAA) is due to multiple distinct disorders (Table 14.1) and has an estimated incidence of approximately 10 per 100,000 person-years [1]. Aneurysms may involve the aortic root, ascending aorta, arch, or descending aorta. The natural history, treatment, and timing of surgical or endovascular therapy is related to the location of the aneurysm and its underlying cause [1]. Because the natural history of TAA disease may be potentially altered by hypertension, cigarette smoking, pregnancy, exercise, and physical activity, pharmacological therapy and lifestyle modification are important in the “medical” management of thoracic aortic aneurysm disease. When evaluating the patient with TAA disease, it is essential to make the correct diagnosis of the underlying condition. Genetically triggered diseases such as Marfan syndrome, LoeysDietz syndrome, familial thoracic aortic aneurysm (FTAA) and dissection syndromes, and vascular Ehlers-Danlos syndrome may have A. C. Braverman (*) Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA Marfan Syndrome and Aortopathy Center, St. Louis, MO, USA Inpatient Cardiology Firm, St. Louis, MO, USA e-mail: [email protected]
overlapping phenotypes, and accurate diagnosis is important for surgical decision-making and for identifying affected relatives [1, 2]. Pharmacological therapy may have a role in the management of patients with TAAs. Much of the data regarding pharmacologic therapy for TAA disease is derived from mouse model studies in genetically triggered disease, such fibrillin-1-deficient (Marfan) mice [3]. However, there are few studies on pharmacologic treatment of TAA disease in humans. Trials of beta blockers and angiotensin receptor blockers have been completed or are underway in Marfan syndrome. Thoracic aortic aneurysm disease often has a long period of observation before requiring elective open surgery or endovascular repair. Lifestyle modification is essential in the management of patients with TAA disease. Physical activity, exercise, weight lifting, pregnancy, and job- related physical demands must be considered to lessen stress on the dilated thoracic aorta and maintain adequate safety. Finally, long-term surveillance of the aorta is required both before and after surgery for patients with TAA disease.
14.2 Diagnosis of Underlying Disease-Causing TAA The medical history, family history, and physical examination may assist in the diagnosis of the correct underlying disease responsible for TAA
© Springer-Verlag GmbH Austria, part of Springer Nature 2019 O. H. Stanger et al. (eds.), Surgical Management of Aortic Pathology, https://doi.org/10.1007/978-3-7091-4874-7_14
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208 Table 14.1 Etiology of thoracic aortic aneurysms Genetically triggered diseases and congenital heart defects Marfan syndrome Bicuspid aortic valve aortopathy Familial thoracic aortic aneurysm syndromes Loeys-Dietz syndrome Vascular Ehlers-Danlos syndrome Turner s
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