This poll asked readers to consider the best approach to managing complex issues surrounding patients with heritable thoracic aortic disease (HTAD), specifically Marfan syndrome, in this relatively young woman who presented with acute aortic dissection of a heretofore unknown aortic root aneurysm. 

Most respondents (63.3%) chose "a multidisciplinary team specializing in the care of patients with aortic disease," while 18.4% chose "a cardiologist who specializes in the treatment of patients with aortic disease." Interestingly, not many chose either a cardiac or vascular surgeon for follow-up, perhaps recognizing that while aortic dissection is a cardiovascular problem, even cardiac and vascular surgeons may not all have expertise in the management of patients with HTAD status post aortic dissection repair.

The 2010 multi-society guidelines on the treatment of thoracic aortic disease have recommendations for imaging surveillance for aortic disease in previously repaired patients, with Class 2a, Level of Evidence C data that suggest that this patient should have computed tomographic (CT) or magnetic resonance imaging (MRI) of the chest plus abdomen with transthoracic echocardiography before discharge, at 1 month, at 6 months, and yearly in the post-repair period.¹ The guidelines also recommend optimal blood pressure control and particular medication regimens that may be beneficial in HTAD.¹

However, in addition to aortic follow-up, this patient, with a new mechanical valve and newly diagnosed Marfan syndrome, may develop multi-system complications associated with her HTAD, including prosthetic or mitral valvular dysfunction, myopia or lens dislocation, scoliosis, pneumothorax, or hernias.

Different centers have varying approaches to the post-operative care of these patients. The practice at our institution is to follow a "team" approach, with the patients seen life-long by their cardiac surgeon at defined time intervals with CT angiography surveillance in an aortic clinic, and additional follow-up with their cardiologist and a geneticist who specializes in HTAD. A vascular surgeon would also be involved depending on the location of complications of the residually dissected aorta.

References

1. 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: executive summary. 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.

Clinical Topics: Cardiac Surgery, Cardiovascular Care Team, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Vascular Medicine, Aortic Surgery, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Computed Tomography, Echocardiography/Ultrasound, Magnetic Resonance Imaging, Nuclear Imaging

Keywords: Follow-Up Studies, Blood Pressure, Aftercare, Marfan Syndrome, Computed Tomography Angiography, Patient Discharge, Pneumothorax, Scoliosis, Aneurysm, Dissecting, Aorta, Aortic Diseases, Echocardiography, Magnetic Resonance Imaging, Patient Care Team, Lens Subluxation, Surveys and Questionnaires, Abdomen, Surgeons, Hernia, Myopia, Cardiac Surgical Procedures

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