Thoracic Aortic Aneurysmal Disease: Comprehensive Recommendations for the Primary Care Physician.

Thoracic aortic aneurysm (TAA) is a commonly encountered disease that is defined as aortic dilation with an increase in diameter of at least 50% greater than the expected age- and sex-adjusted size. Thoracic aortic aneurysms are described by their size, location, morphology, and cause. Primary care clinicians and other noncardiologists are often the first point of contact for patients with TAA. This review is intended to provide them with basic information on the differential diagnosis, diagnostic evaluation, and medical and surgical management of TAAs. Management decisions depend on having as precise a diagnosis as possible. Fortunately, this can often be achieved with a stepwise diagnostic approach that incorporates imaging and targeted genetic testing. Our review includes recommendations. In this review, we discuss these issues at a basic level and include recommendations for patients considering pregnancy.

What Is Marfan Syndrome?

This JAMA Patient Page describes the autosomal dominant genetic disorder of Marfan syndrome and its diagnosis and treatment.

Audit of Gastrointestinal Manifestations in Patients with Loeys-Dietz Syndrome and Vascular Ehlers-Danlos Syndrome.

OBJECTIVES: Loeys-Dietz syndrome (LDS) and vascular Ehlers-Danlos Syndrome (vEDS) are genetically heterogeneous heritable disorders of connective tissue. Both are multi-system disorders with dominant vascular pathology and associated gastrointestinal manifestations. AIM: To summarize the abdominal manifestations found in these two disorders in a cohort of patients seen at Mayo Clinic during a period of 25 years. METHODS: Data were collected via the advanced cohort explorer (ACE) of Mayo Clinic records from 1994 to 2018 in patients with vEDS or LDS confirmed by genetic testing and/or medical genetics consultation. We extracted information concerning gastrointestinal symptoms, abdominal hernias, and vascular manifestations or complications. RESULTS: We identified and reviewed records of 68 vEDS and 13 LDS patients. Patients were similar in age at diagnosis and gender distribution. Gastrointestinal symptoms were frequently reported in both disorders and largely similar, although altered bowel function was more prevalent in LDS patients. Hernias were present in similar proportions of patients with vEDS and LDS; however, ventral hernias were more frequent and more likely to be postoperative in vEDS than LDS. LDS patients had more arterial aneurysms overall (76.9% LDS vs. 58% vEDS, p = 0.02) and a higher proportion required arterial repair (69.2% LDS vs. 32.7% vEDS S, p = 0.03). Co-morbidities of autonomic dysfunction, psychopathology (most commonly anxiety, depression, adjustment disorder), and allergy were more prevalent in LDS than vEDS. CONCLUSION: Patients with vEDS and LDS had a propensity for gastrointestinal symptoms, abdominal hernias, and aneurysm formation, but repair for arterial rupture was more prevalent in LDS than EDS.

Spectrum of Aortic Disease in the Giant Cell Arteritis Population.

We report the spectrum of aortic involvement in patients with giant cell arteritis (GCA) following review of medical records of 4,006 patients including those with imaging studies. A total of 1,450 patients (36%) had a confirmed diagnosis of GCA. Of these, 974 had aortic imaging. Of the 974 patients with imaging, 435 (45%) had an identified aortopathy. The most common aortopathy was aneurysm/dilation (69%). Overall, an annual aneurysmal growth rate of 1.5 mm/y was calculated. In patients with aneurysm/dilation, aortic dissection occurred in 18 patients (6%), and these patients had a significantly higher aneurysmal growth rate compared with those without dissection (4.5 vs 1.4 mm/y, p = 0.005). The median size of the aorta at the time of dissection was 51 mm, with 7 (39%) occurring with a maximal aortic aneurysm/dilation <50 mm. In conclusion, our findings indicate higher aneurysmal growth rate in GCA compared with that reported for degenerative aortic disease. Moreover, patients who develop dissection had a significantly higher growth rate than those without dissection with over a third of these patients suffering dissection at a caliber <50 mm.

Aortic Root Surgery in Marfan Syndrome: Medium-Term Outcome in a Single-Center Experience.

BACKGROUND: The study aim was to analyze the authors' experience with aortic root surgery in Marfan syndrome (MFS), and to expand the surgical outcome data of patients meeting the Ghent criteria (Marfan registry). METHODS: Analyses were performed of data acquired from MFS patients (who met the Ghent criteria), including an aortic root surgery and Kaplan-Meier survival. RESULTS: Between April 2004 and February 2012, a total of 59 MFS patients (mean age at surgery 36 ± 13 years) underwent 67 operations for aortic root aneurysm (n = 52), aortic valve (AV) regurgitation (n = 15), acute aortic dissection (n = 2), and/or mitral valve (MV) regurgitation resulting from MV prolapse (n = 7). Of 59 initial operations, 21 (36%) involved AV-replacing root surgery, 38 (64%) AV-sparing root surgery, seven (12%) aortic arch or hemi-arch repair, and five (8%) simultaneous MV surgery. There were no early mortalities. The mean follow up was 6.8 ± 1.2 years, with five deaths (8%) and a relatively low reoperation rate (10 reoperations in nine patients; 14%). Seven reoperations involved AV or aortic root surgery (including four for AV regurgitation following failed AV-sparing surgery), two MV repair/replacements, and one coronary artery bypass graft. Eight patients (21%) with AV-sparing surgery had moderate/severe AV regurgitation at the last follow up before re-intervention. The mean five-year freedom from postoperative death was 91.2 ± 8.8%, from cardiac reoperation 86.3 ± 4.5%, and more-than-moderate AV regurgitation 90.3 ± 4.8%. CONCLUSIONS: Prophylactic aortic surgery in MFS patients with AV-replacing root or AV-sparing root surgery carries a low risk of operative morbidity and death when performed at an experienced center. AV-sparing root surgery increases the risk of AV regurgitation and, possibly, of re-intervention. Regular clinical follow up is important after any aortic root surgery in MFS patients, with a delineation of risk factors for AV regurgitation after AV rootsparing surgery.

Visiting medical student elective and clerkship programs: a survey of US and Puerto Rico allopathic medical schools.

BACKGROUND: No published reports of studies have provided aggregate data on visiting medical student (VMS) programs at allopathic medical schools. METHODS: During 2006, a paper survey was mailed to all 129 allopathic medical schools in the United States and Puerto Rico using a list obtained from the Association of American Medical Colleges. Contents of the survey items were based on existing literature and expert opinion and addressed various topics related to VMS programs, including organizational aspects, program objectives, and practical issues. Responses to the survey items were yes-or-no, multiple-choice, fill-in-the-blank, and free-text responses. Data related to the survey responses were summarized using descriptive statistics. RESULTS: Representatives of 76 schools (59%) responded to the survey. Of these, 73 (96%) reported their schools had VMS programs. The most common reason for having a VMS program was "recruitment for residency programs" (90%). "Desire to do a residency at our institution" was ranked as the leading reason visiting medical students choose to do electives or clerkships. In descending order, the most popular rotations were in internal medicine, orthopedic surgery, emergency medicine, and pediatrics. All VMS programs allowed fourth-year medical students, and approximately half (58%) allowed international medical students. The most common eligibility requirements were documentation of immunizations (92%), previous clinical experience (85%), and successful completion of United States Medical Licensing Examination Step 1 (51%). Of the programs that required clinical experience, 82% required 33 weeks or more. Most institutions (96%) gave priority for electives and clerkships to their own students over visiting students, and a majority (78%) reported that visiting students were evaluated no differently than their own students. During academic year 2006-2007, the number of new resident physicians who were former visiting medical students ranged widely among the responding institutions (range, 0-76). CONCLUSIONS: Medical schools' leading reason for having VMS programs is recruitment into residency programs and the most commonly cited reason students participate in these programs is to secure residency positions. However, further research is needed regarding factors that determine the effectiveness of VMS programs in residency program recruitment and the development of more universal standards for VMS eligibility requirements and assessment.

Type IV thoracoabdominal aortic aneurysm with lymphoplasmacytic aortitis and cystic medial degeneration in a 32-year-old patient with Marfan syndrome.

Aortitis identified in approximately 12% of all thoracoabdominal aneurysms. The most common subtype of inflammatory aortitis is giant cell aortitis, followed by lymphoplasmacytic aortitis. Inflammatory aortitis may occur in isolation or as part of a systemic inflammatory disorder such as Takayasu arteritis, systemic lupus erythematosus, rheumatoid arthritis, and giant cell arteritis. Aortitis has not been described in patients with Marfan syndrome. We report the case of a 32-year-old man with Marfan syndrome and a strong family history of aneurysmal disease who presented with an asymptomatic Crawford type IV thoracoabdominal aneurysm. His aneurysm had no associated dissection, and surgical pathology revealed severe medial degeneration and lymphoplasmacytic aortitis. To our knowledge, this is the first report of such a finding in a patient with Marfan syndrome.

Provider satisfaction in clinical encounters with ethnic immigrant patients.

OBJECTIVE: To determine whether physicians' satisfaction in clinical encounters with ethnic immigrant patients differs from satisfaction in clinical encounters with white patients in the local community. PATIENTS AND METHODS: Postvisit assessments from primary care physicians were collected for matched pairs of ethnic and control patients at the Mayo Clinic in Rochester, Minn, during a 10-week study (April 2-June 9, 2001). Ethnic patients were defined as first-generation Somalian, Cambodian, and Hispanic immigrants. Control patients were American-born white patients who were seen by the same physician and matched to the ethnic patients in age, sex, and type of visit. T tests and Hotelling T2 tests were used to analyze differences in physician responses between groups; regression analysis was used to identify the relationship between physicians' satisfaction and ethnicity in the presence of covariates. RESULTS: Physicians were considerably less satisfied with ethnic patient visits compared with control patient visits. Larger differences in satisfaction were reported in the areas of patient efforts with disease prevention and management of chronic diseases. Smaller differences in satisfaction were reported for issues related to communication and cultural beliefs and practices. These differences persisted after controlling for patient demographics, physician, and visit characteristics. CONCLUSIONS: Patients' ethnicity affects physician satisfaction with clinical encounters, particularly in the delivery of preventive care and chronic disease management.