Treatment of Aortic Disease in Patients With Marfan Syndrome

Milewicz, Dianna M.; Dietz, Harry C.; Miller, D. Craig

doi:10.1161/01.cir.0000155243.70456.f4

Cited by 356 publications

(221 citation statements)

References 46 publications

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“…14) As shown in our study, because the morbidity and mortality rates in MFS patients undergoing elective root surgery is low, and besides, emergency operation for aneurysmal rupure or acute dissection worsens the clinical results, early recognition of the disorder, identifi cation of presymptomatic patients, and subsequent institution of surgical therapy is mandatory to reduce the frequency of catastrophic aortic events.…”

Section: Discussionmentioning

confidence: 57%

Risk Model of Cardiovascular Surgery in 845 Marfan Patients Using the Japan Adult Cardiovascular Surgery Database

Miyairi

Matsubara

Taketani³

et al. 2013

Int. Heart J.

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SummaryThe aim of this study was to evaluate the short-term operative results of patients with Marfan syndrome who underwent thoracic or abdominal aortic surgery in a 4-year period in Japan. Data were collected from the Japan Cardiovascular Surgery Database (JCVSD). We retrospectively analyzed the data of 845 patients with Marfan syndrome who underwent cardiovascular surgery between January 2008 and January 2011. Logistic regression was used to generate risk models. The early mortality rate was 4.4% (37/845). Odds ratios (OR), 95% confi dence intervals (CI), and P values for structures and processes in the mortality prediction model were as follows: renal insuffi ciency (OR, 11.37; CI,; P < 0.001); respiratory disorder (OR, 11.12; CI,; P < 0.001); aortic dissection (OR, 13.02; CI, 2.80-60.60; P = 0.001); pseudoaneurysm (OR, 11.23; CI, 1.38-91.66; P = 0.024); thoracoabdominal aneurysm (OR, 2.67; CI, 1.22-5.84; P = 0.014); and aortic rupure (OR, 4.23; CI,; P = 0.002). The mortality prediction model had a Cindex of 0.82 and a Hosmer-Lemeshow P value of 0.56. In conclusion, this study demonstrated that renal insuffi ciency and respiratory disorder had great impact on the operative mortality of Marfan patients undergoing cardiovascular surgery. Because patients with aortic dissection or aortic rupture showed high operative mortality, close follow-up to avoid emergency operation is mandatory to improve the operative results. Achieving good results from surgery of the thoracoabdominal aorta was quite challenging, also in Marfan patients. (Int Heart J 2013; 54: 401-404)

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Section: Discussionmentioning

confidence: 57%

Risk Model of Cardiovascular Surgery in 845 Marfan Patients Using the Japan Adult Cardiovascular Surgery Database

Miyairi

Matsubara

Taketani³

et al. 2013

Int. Heart J.

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“…The patient should undergo careful evaluation for all cardiovascular abnormalities including valvular pathology, left ventricular function, diameter of the aortic root and the ascending and descending aorta using transthoracic and when needed transesophageal echocardiogram (8,9,59). Evaluation of the distal aorta is especially important in patients with dilated proximal aorta and those with the history of surgical repair of the proximal aorta, who have been shown to be at increased risk of distal AoD (71,72). Either computed tomography (CT) or magnetic resonance imaging (MRI) can be used for a precise assessment of the aortic size and anatomy before pregnancy (59,72,73).…”

Section: Preconception Counselingmentioning

confidence: 99%

Pregnancy and Marfan syndrome

Goland¹,

Elkayam²

2017

Ann. Cardiothorac. Surg.

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Pregnancy in women with Marfan syndrome (MFS) presents challenges to the clinician and the patient due to the increased incidence of maternal complications and involvement of the fetus, and deserves special consideration. The leading cause of morbidity and mortality in MFS is aortic dissection. This article presents an extensive review of available clinical information and provides recommendations for the management of patients with MFS during pregnancy.

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“…(17,18) Prophylactic surgery is recommended when the diameter of the aortic sinuses of Valsalva reaches 5.0 cm. (19) Other factors such as family history of aortic dissection, severe aortic valve regurgitation with associated symptoms or progressive ventricular dilatation or dysfunction, the possibility of a valvesparing operation, and the rate of aortic dilatation (aortic root growth >2 mm/yr), may indicate the need for surgery at a smaller aortic sinus…”

Section: Surgical Managementmentioning

confidence: 99%