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

Miyairi, Takeshi; Matsubara, Hiroaki; Taketani, Tsuyoshi; Sawaki, Daigo; Suzuki, Tohru; Hirata, Yasunobu; Shimizu, Hideyuki; Motomura, Noboru; Takamoto, Shinichi

doi:10.1536/ihj.54.401

Cited by 3 publications

(1 citation statement)

References 17 publications

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“…Even after successful aortic root replacement, however, other aortic segments remain a source of late aortic aneurysm/dissection formation. According to the Japan Cardiovascular Surgery Database (JCVSD), the 30 day operative mortality rate in MFS patients was 4.4% (37/845), and renal insufficiency and respiratory disorder had great impact . Stent‐graft repair is not indicated, because stent deployment imposes high stresses and vascular injury on the aortic wall.…”

Section: Prevention and Therapymentioning

confidence: 99%

Pathophysiology and Japanese clinical characteristics in Marfan syndrome

Fujita

Takeda

Imai

et al. 2014

Pediatrics International

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Marfan syndrome is an autosomal dominant heritable disorder of the connective tissue, caused by mutations of the gene FBN1, which encodes fibrillin-1, a major component of the microfibrils of the extracellular matrix. Fibrillin-1 interacts with transforming growth factor-β (TGF-β), and dysregulated TGF-β signaling plays a major role in the development of connective tissue disease and familial aortic aneurysm and dissection, including Marfan syndrome. Losartan, an angiotensin II blocker, has the potential to reduce TGF-β signaling and is expected to be an additional therapeutic option. Clinical diagnosis is made using the Ghent nosology, which requires comprehensive patient assessment and has been proven to work well, but evaluation of some of the diagnostic criteria by a single physician is difficult and timeconsuming. A Marfan clinic was established at the University of Tokyo Hospital in 2005, together with cardiologists, cardiac surgeons, pediatricians, orthopedists, and ophthalmologists in one place, for the purpose of speedy and accurate evaluation and diagnosis of Marfan syndrome. In this review, we discuss the recent progress in diagnosis and treatment of Marfan syndrome, and the characteristics of Japanese patients with Marfan syndrome.

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Section: Prevention and Therapymentioning

confidence: 99%

Pathophysiology and Japanese clinical characteristics in Marfan syndrome

Fujita

Takeda

Imai

et al. 2014

Pediatrics International

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Effect of Periodontitis on Cardiovascular Manifestations in Marfan Syndrome

et al. 2015

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SummaryMarfan syndrome (MFS) is a systemic connective tissue disorder that is caused by mutations in the extracellular matrix protein fibrillin-1. While MFS patients are considered to be at high risk of dental disorders and cardiovascular complications, little causal relationship has been provided to date. It is well known that an elevated level of active TGF-β in the plasma is a major manifestation of MFS. TGF-β is known to play a critical role in the development of cardiovascular diseases and its levels were also elevated in the serum and saliva of periodontitis patients. These findings may suggest an association between periodontitis and the cardiovascular complications of MFS. In this article, we review the influence of periodontitis in MFS patients with cardiovascular complications in order to identify critical therapeutic targets of TGF-β. (Int Heart J 2015; 56: 121-124)

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Circadian Variation of Acute Aortic Dissection

et al. 2015

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Acute aortic dissection (AAD) is a life-threatening cardiovascular disease with high mortality. Hypertension is a well known risk factor of AAD. There have been previous reports about the association between circadian variation of blood pressure (BP) and cardiovascular events. However, little is known about the association between the onset-time of AAD and circadian variation of BP. The purpose of this study was to clarify the characteristics of circadian variation of BP in AAD and its relation to the onset-time of this disease. This study included type B spontaneous AAD patients who were referred to our institution and treated conservatively between January 2008 and June 2013. Patients with type A AAD, secondary to trauma, and type B AAD which preceded surgical intervention were excluded. Data were retrospectively collected from the hospital medical records. Sixty-eight patients with type B AAD were enrolled. The distribution of the circadian pattern in the study patients was as follows: extreme-dipper, 0% (none); dipper, 20.6% (n = 14); nondipper, 50% (n = 34); riser, 29.4% (n = 20). Non-dipper and riser patterns were more frequently observed compared with other population studies reported previously. Moreover, no patient in the dipper group had night-time onset while 31.5% of the patients in the absence of nocturnal BP fall group (non-dipper and riser) did (P = 0.01). Absence of a nocturnal BP fall was frequently seen in AAD patients. Absence of a nocturnal BP fall may be a risk factor of AAD. Circadian variation of BP may also affect the onset-time of type B AAD.

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

Cited by 3 publications

References 17 publications

Pathophysiology and Japanese clinical characteristics in Marfan syndrome

Pathophysiology and Japanese clinical characteristics in Marfan syndrome

Effect of Periodontitis on Cardiovascular Manifestations in Marfan Syndrome

Circadian Variation of Acute Aortic Dissection

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