Thoracic aortic diseases result in more than 40,000 deaths per year in the United States, but this estimate is likely low because many of these deaths will result from an undiagnosed aortic rupture or dissection and will be labeled as a heart attack. Most thoracic aortic diseases are asymptomatic until they present with catastrophic aortic dissection, aortic rupture, stroke, or other complications associated with a high likelihood of mortality or major morbidity. Acute aortic dissection may present with atypical symptoms and findings, making a diagnosis even more difficult. Awareness of the importance of thoracic aortic diseases and their varied presentations has been lacking.
In the April 2, 2010 issues of Circulation and the Journal of American College of Cardiology, my colleagues and I developed guidelines to provide physicians with recommendations for the early diagnosis and management of thoracic aortic disease. The guidelines represent a consensus of 10 co-sponsoring specialty organizations with an additional endorsing imaging organization; it also utilized input of two additional specialty organizations.
Scientific and clinical advances in medicine drove the development of the guidelines, significantly impacting the diagnosis and management of aortic dissection, aortic aneurysms, and other forms of thoracic aortic disease. For example, imaging techniques have improved the ability to make diagnoses. A diagnosis of thoracic aortic disease requires dedicated imaging (CT or MRI, and in some cases, echocardiography); standard chest radiography is not sufficient. Improved surgical and endovascular methods of treating thoracic aortic diseases and methods of preserving other organs during and after surgical or endovascular interventions have also improved the management of thoracic aortic disease.
Managing At-Risk Patients
Patients at risk for thoracic aortic disease must be screened with CT, MRI, or, in some cases, echocardiography to identify issues before a catastrophic presentation occurs. Treatment in stable, asymptomatic patients carries far less risk than treatment in catastrophic situations. Clinicians must recognize key risk factors, including:
History of uncontrolled high blood pressure.
Presence of a connective tissue disorder such as Marfan syndrome, Ehlers-Danlos syndrome (vascular type), Loeys-Dietz syndrome, and Turner syndrome, among others.
Family history of aortic aneurysm or aortic dissection (even without a history of connective tissue disorder).
Personal or family history of bicuspid aortic valve or recent aortic manipulation.
“Awareness of the importance of thoracic aortic diseases and their varied presentations has been lacking.”
Family history is a critical tool to identify undiagnosed cases. First-degree relatives of any patient who has a thoracic aortic aneurysm or aortic dissection should be screened for the presence of thoracic aortic disease. Patients at high risk should be screened for the presence of thoracic aortic dilatation. Patients who are identified with asymptomatic or symptomatic thoracic aortic diseases should be referred to cardiovascular surgeons or cardiologists with expertise in thoracic aortic disease.
Other Guideline Highlights
Aortic dissection involving the ascending aorta is considered a life-threatening emergency that should be treated surgically. Aortic dissection involving the descending thoracic aorta may be managed with medications that control the blood pressure and heart rate unless malperfusion or life-threatening complications develop. Additional medical therapy may include statins to lower elevated cholesterol levels. Furthermore, less invasive endovascular techniques may be an option in select patients.
The new guidelines also include a series of algorithms to improve the early diagnosis of aortic dissection. The hope is that these algorithms will be of use to emergency physicians. For further information on thoracic aortic disease, physicians can visit www.TADCoalition.org.
Readings & Resources (click to view)
Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis and management of patients with thoracic aortic disease. A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology,American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. J Am Coll Cardiol. 2010;55:e27-e129. Available at: http://circ.ahajournals.org/cgi/content/full/121/13/e266#BIBL.
Svensson LG, Rodriguez ER. Aortic organ disease epidemic, and why do balloons pop? Circulation. 2005;112:1082-1084.
Milewicz DM, Guo DC, Tran-Fadulu V, et al. Genetic basis of thoracic aortic aneurysms and dissections: focus on smooth muscle cell contractile dysfunction. Annu Rev Genomics Hum Genet. 2008;9:283-302.
Klompas M. Does this patient have an acute thoracic aortic dissection? JAMA. 2002;287:2262-2272.
Ellis JD, Mayo JR. Computed tomography evaluation of traumatic rupture of the thoracic aorta: an outcome study. Can Assoc Radiol J. 2007;58:22-26.
Coady MA, Ikonomidis JS, Cheung AT, et al. Surgical Management of Descending Thoracic Aortic Disease: Open and Endovascular Approaches. A Scientific Statement From the American Heart Association. Circulation. 2010 Jun 7. [Epub ahead of print].