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ABSTRACT: Although patients with abdominal aortic aneurysm (AAA) often have other cardiovascular diseases (CVDs), the exact prevalence remains unclear. We aimed to determine the prevalence and predictors of coexistent silent atherosclerotic cardiovascular diseases (SACVDs) in patients with AAA without a history of CVD. Consecutive 157 patients with AAA (mean age, 73 years), without any previous history of CVD, were included. Silent myocardial ischemia (SMI), cerebrovascular disease (CeVD), peripheral artery disease (PAD), and thoracic aortic aneurysm (TAA) without symptoms coexisted in 29.3%, 25.5%, 15.9%, and 8.3%, respectively. The significant predictors of SMI were diabetes mellitus (P = .025) and male sex (P = .048). The significant predictor of silent CeVD was older age (P = .039). The borderline predictors of asymptomatic PAD and TAA were diabetes mellitus (P = .056) and AAA size (P = .053), respectively. Even with no previous symptomatic CVD, patients with AAA have high prevalence of coexistent SACVD.
Angiology 09/2011; 63(5):380-5. · 1.51 Impact Factor
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ABSTRACT: Loeys-Dietz syndrome (LDS) is a connective tissue disease caused by mutations in the genes encoding the transforming growth factor-beta receptor (TGFBR). LDS is associated with aneurysms or dissections of the aorta similar to Marfan syndrome (MFS) as well as arterial tortuosity and aneurysms in the peripheral arteries. The purpose of this study was to evaluate the arterial diseases of LDS to differentiate it from MFS.
A total of 10 LDS patients with an identified mutation in TGFBR (6 male, 4 female; mean age 36.3 years) and 20 MFS patients with an identified mutation in fibrilin-1 who were age- and sex-matched to the LDS subjects (12 male, 8 female; mean age 37.1 years) were reviewed. The prevalence of vertebral arterial tortuosity (VAT) and peripheral aneurysm (PAN) was studied using computed tomography angiography.
In all, 9 of the 10 LDS patients had VAT, and five PANs were observed in 3 patients. In contrast, 8 (40%) of the MFS patients had VAT, and 1 patient had a PAN. LDS had a higher prevalence of VAT (P = 0.017) by Fisher's exact test.
The VAT was highly prevalent among LDS patients. Thus, the presence of VAT has the potential to differentiate LDS from MFS.
Japanese journal of radiology 05/2010; 28(4):273-7. · 0.65 Impact Factor
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ABSTRACT: Although the existence of the young patients with aortic disease not fulfilling the diagnostic criteria for Marfan syndrome (MFS) has been known, the etiology of their disease has not yet been elucidated. The purpose of the present study was to elucidate the genetic and clinical features of the young patients with aortic disease not having MFS.
Eighty young adult patients with aortic disease were examined. They were divided into a definite MFS (n=51) and a non-definite MFS group (n=29) according to the Ghent nosology. Clinical and genetic characteristics were compared between the 2 groups. Among 29 non-definite MFS probands, 1 (3%) FBN1, 2 (7%) TGFBR1, and 3 (10%) TGFBR2 mutations were found, and 4 ACTA2 mutations were found in the 23 probands examined without FBN1, TGFBR1, or TGFBR2 mutations. In total, more than 10 out of 29 (34%) probands in the non-definite MFS group were associated with genetic mutations. Skeletal involvement was less frequent in the non-definite than in the definite MFS group (7% vs 82%, P<0.01).
In the probands with aortic diseases in young who cannot be diagnosed with MFS, mutations other than FBN1 mutations accounted for at least one-third of all causes of aortic disease.
Circulation Journal 03/2010; 74(5):990-7. · 3.77 Impact Factor
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Hiroko Morisaki, Koichi Akutsu,
Hitoshi Ogino,
Norihiro Kondo,
Itaru Yamanaka,
Yoshiaki Tsutsumi,
Tsuyoshi Yoshimuta,
Toshiya Okajima,
Hitoshi Matsuda,
Kenji Minatoya,
Hiroaki Sasaki,
Hiroshi Tanaka,
Hatsue Ishibashi-Ueda,
Takayuki Morisaki
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ABSTRACT: Approximately 20% of aortic aneurysm and/or dissection (AAD) cases result from inherited disorders, including several systemic and syndromatic connective-tissue disorders, such as Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome, which are caused by mutations in the FBN1, COL3A1, and TGFBR1 and TGFBR2 genes, respectively. Nonsyndromatic AAD also has a familial background, and mutations of the ACTA2 gene were recently shown to cause familial AAD. In the present study, we conducted sequence analyses of the ACTA2 gene in 14 unrelated Japanese patients with familial thoracic AAD (TAAD), and in 26 with sporadic and young-onset TAAD. Our results identified three mutations of ACTA2, two novel [p.G152_T205del (c.616+1G>T), p.R212Q] and one reported (p.R149C), in the 14 patients with familial TAAD, and a novel mutation (p.Y145C) of ACTA2 in the 26 sporadic and young-onset TAAD patients, each of which are considered to be causative for TAAD. Some of the clinical features of these patients were the same as previously reported, whereas others were different. These findings confirm that ACTA2 mutations are important in familial TAAD, while the first sporadic and young-onset TAAD case with an ACTA2 mutation was also identified.
Human Mutation 07/2009; 30(10):1406-11. · 5.69 Impact Factor
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The Canadian journal of cardiology 07/2009; 25(6):365. · 3.36 Impact Factor
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Yuiichi Tamori, Koichi Akutsu,
Satoshi Kasai,
Shingo Sakamoto,
Toshiya Okajima,
Tsuyoshi Yoshimuta,
Naoyuki Yokoyama,
Hitoshi Ogino,
Masahiro Higashi,
Hiroshi Nonogi,
Satoshi Takeshita
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ABSTRACT: Aortic aneurysms are found in 5-20% of patients with acute aortic dissection (AAD). Coexisting aortic aneurysms might potentially influence the incidence of AAD. The purpose of this study was to elucidate the role of coexistent aortic aneurysms in AAD.
A total of 140 patients with AAD were enrolled in the present study. Clinical characteristics of the patients were evaluated in relation to the locations of aortic segments affected by the dissection as well as of the coexistent aortic aneurysm. Among the 140 study patients, 34 (24%) had true aortic aneurysms. Patients with coexistent aortic aneurysm were significantly older than those without (72 +/- 11 years vs 65 +/- 14 years, P=0.012) and had higher incidence of thrombosed false lumen (62% vs 38%, P=0.017), and coronary artery disease (26% vs 8%, P=0.006). Twenty-two of these 34 (65%) patients had a thoracic aortic aneurysm (TAA), and this frequency of TAA was much higher than that observed in the general population. Furthermore, among all patients with AAD, 12 patients (9%) might be associated with development of AAD.
The current study showed that nearly one-quarter of AAD patients had coexisting true aortic aneurysms, and suggests that TAA are likely to be associated with development of AAD.
Circulation Journal 04/2009; 73(5):822-5. · 3.77 Impact Factor
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ABSTRACT: Diagnosis of Marfan syndrome (MS) is made according to the Ghent nosology, which is based on data from European and American populations. The validity of applying the Ghent nosology to other than Western populations is an ongoing discussion because there may be racial differences in basic physical features. The validity of applying the Ghent nosology to patients other than Westerners suspected of having MS was examined. One hundred thirteen Japanese patients who were suspected of having MS and underwent genetic analysis were examined to see whether they fulfilled the Ghent nosology. Of 113 patients, MS was diagnosed in 58 patients/51 probands. Of these 51 probands, 46 (90%) showed mutations in the Fibrillin-1 gene(FBN1) and were enrolled in this study. The frequency of each manifestation of Ghent nosology in the Japanese population was compared with those reported in the FBN1 Universal Mutation Database that was mainly obtained from the Western population (n = 1,013 probands). Frequencies were lower in the Japanese population than the Western population of the manifestations of arm span to height ratio >1.05 (20% vs 55%; p <0.01), scoliosis (40% vs 53%; p <0.05), reduced extension at elbows (2% vs 16%; p <0.05), and joint hypermobility (46% vs 63%; p <0.05). In conclusion, we found a lower frequency of skeletal manifestations of MS in Japanese patients than reported in the database for Western patients with MS. It was possible that the diagnosis of MS according to the Ghent nosology for Japanese patients was underestimated, especially for skeletal involvements.
The American journal of cardiology 04/2009; 103(8):1146-8. · 3.58 Impact Factor
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The Canadian journal of cardiology 01/2009; · 3.36 Impact Factor
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ABSTRACT: Exercise training improves walking ability in patients with peripheral arterial disease (PAD), but whether exercise training improves the long-term outcome of these patients remains unknown.
Participants were 118 PAD patients who were enrolled in a 12-week supervised exercise program. The long-term outcomes of 64 patients who completed the training were compared with the outcomes of 54 patients who did not. The primary endpoint was cardiovascular mortality, and the secondary endpoint was cardiovascular morbidity. Mean follow-up was 5.7+/-3.9 years. The cardiovascular death-free rate was higher in patients who completed the training program than in those who did not (P=0.022). Multivariate analysis showed independent predictors of cardiovascular death were age over 70 years, diabetes mellitus, maximum walking distance, history of coronary revascularization, and completion of training program. The cardiovascular event-free rate was also higher in patients who completed the training program (P=0.048).
Supervised exercise training improved cardiovascular mortality and morbidity in patients with PAD, which suggests that exercise training should be considered as a secondary prevention strategy for these patients.
Circulation Journal 12/2008; 73(1):167-73. · 3.77 Impact Factor
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ABSTRACT: Medical therapy gives excellent short-term outcomes for patients with Stanford type B acute aortic dissection. However, the affected aorta is often compromised by aneurysmal dilatation and rupture, resulting in poor long-term outcome. The present study investigated which antihypertensive treatment may prevent long-term aortic events in these patients.
The study group comprised 78 consecutive patients with Stanford type B acute aortic dissection who were treated medically and followed-up for an average of 873+/-548 days. The optimal hypertensive drug regimen to reduce aortic events was determined by multivariate analyses. Of the 78 patients, 73 (94%) were discharged from hospital with medical therapy, and aortic events occurred in 13 (18%) of them (aortic rupture/recurrent dissection in 2 (3%); aortic expansion >or=60 mm in 7 (10%), rapid aortic expansion >or=10 mm/year in 3 (4%), and development of visceral/limb ischemia in 1 (1%)) during follow-up. By multivariate analysis, patients given angiotensin-converting enzyme inhibitor (ACEI) were less likely to have long-term aortic events than those without (odds ratio: 0.18, 95% confidence interval: 0.04-0.85).
Use of ACEI is associated with a reduced risk of long-term aortic events in patients with medically treated type B aortic dissection.
Circulation Journal 10/2008; 72(11):1758-61. · 3.77 Impact Factor
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The Canadian journal of cardiology 08/2008; 24(7):568. · 3.36 Impact Factor
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Circulation 06/2008; 117(19):2542-3. · 14.74 Impact Factor
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ABSTRACT: Percutaneous peripheral intervention (PPI) for superficial femoral artery (SFA) stenosis is associated with a high restenosis rate. Whether PPI improves the long-term outcome of patients with SFA occlusive disease remains to be determined.
A review was done of 107 patients with SFA occlusive disease. Fifty-five patients received PPI for SFA (ie, PPI group) and 52 patients received conservative medical therapy (ie, control group). Clinical records were searched for adverse events (eg, death, limb amputation, re-hospitalization, new onset of coronary artery disease and cerebrovascular disease) for an average of 30.6+/-17.7 months. At follow-up, only 5 patients (9.1%) in the PPI group experienced improved limb symptoms compared with baseline, and 6 patients (10.9%) showed ischemic skin ulcer or gangrene. In addition, 2 of these 6 patients were unsuccessful PPI cases complicated with distal embolization and perforation. In the control group, 3 patients (5.8%) presented with improved limb symptoms, and an equal number of patients had worsening of symptoms. Although 2 patients showed ischemic skin ulcers at follow-up, both patients had these lesions at baseline. Adverse events were observed more frequently in the PPI group than the control group (69.1% vs 46.2%, p<0.05). This was mainly due to a higher frequency of re-hospitalization in the PPI group than in controls (52.7% vs 15.4%, p<0.001).
The current study demonstrates that PPI for patients with SFA occlusive disease does not provide superior long-term benefits compared with conservative medical therapy, and that medical therapy will continue to remain the primary treatment strategy for this group of patients.
Circulation Journal 06/2008; 72(5):734-9. · 3.77 Impact Factor
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ABSTRACT: Antiphospholipid syndrome is characterized with arterial and venous thrombosis. In this article, a 38-year-old man presented with headache and swelling of his left limb, which had lasted for 2 months. Duplex ultrasonography showed thrombosis of the left femoral vein. Cerebral magnetic resonance imaging also showed cerebral sinus thrombosis. Serological examination showed that antiphospholipid syndrome was the underlying disease condition in this patient. Despite adequate anticoagulant therapy, deep vein thrombosis and cerebral sinus thrombosis exacerbated, resulting in inferior vena cava occlusion, papilloedema, and abducent nerve paralysis. Optic canal decompression and cistern-peritoneal shunt operation were performed, following which his neurological symptoms were relieved. The occurrence of cerebral sinus thrombosis as initial presentation of antiphospholipid syndrome is extremely rare and remains a diagnostic challenge. Although the clinical presentation is highly variable, the diagnosis should be considered in patients with antiphospholipid syndrome presenting with recent unusual headaches. Improved diagnosis and treatment strategy may ultimately improve the clinical outcome of these patients.
Angiology 05/2008; 59(6):765-8. · 1.51 Impact Factor
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ABSTRACT: Previous studies have indicated that medical therapy provides excellent outcomes for patients with uncomplicated Stanford type B acute aortic dissection. However, affected aortas are often compromised by aneurysmal dilatation and rupture, resulting in poor outcomes. The purpose of this study was to determine predictors of aortic events in patients with Stanford type B acute aortic dissection receiving conservative medical therapy. The study group consisted of 78 consecutive patients with Stanford type B acute aortic dissection who were admitted to the hospital within 48 hours of onset. These patients were treated medically and followed up for 1 year. Aortic events were defined as rupture, recurrent dissection, aortic expansion with diameter>or=60 mm, rapid aortic expansion at a rate of >or=10 mm/yr, and the development of visceral or limb ischemia. Predictors of these events were determined using multivariate analyses. During 1-year follow-up, aortic events were observed in 13 (17%) patients, including aortic rupture in 3 (4%), aortic diameter>or=60 mm in 4 (5%), rapid expansion of the aorta in 3 (4%), and the development of visceral or limb ischemia in 3 (4%). On multivariate analysis, fibrinogen-fibrin degradation product level>or=20 microg/ml (odds ratio 7.802, 95% confidence interval 1.405 to 43.335) on admission was the only independent predictor of aortic events at 1 year. In conclusion, careful monitoring is required for patients with medically treated Stanford type B acute aortic dissection associated with fibrinogen-fibrin degradation product level>or=20 microg/ml on admission.
The American Journal of Cardiology 05/2008; 101(9):1341-4. · 3.37 Impact Factor
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ABSTRACT: Differences in atherosclerotic profiles between patients with thoracic aortic aneurysm (TAA) and patients with abdominal aortic aneurysm (AAA) have not been studied. We retrospectively studied the clinical records of 343 consecutive patients (132 TAA and 211 AAA) who were admitted to our hospital for elective repair of aortic aneurysms between July 2001 and December 2004. Clinical variables were compared between patients with TAA and those with AAA by using a univariate analysis, and those achieving statistical significance were subsequently assessed in a multivariate analysis. The incidence of coronary artery disease (CAD) (53% vs 23%, p <0.0001), 3-vessel coronary disease (41% vs 10%, p <0.0001), male gender (86% vs 74%, p <0.01), smoker (88% vs 76%, p <0.01), chronic obstructive pulmonary disease (COPD) (30% vs 15%, p <0.01), and diabetes mellitus (39% vs 23%, p <0.01) were significantly higher in patients with AAA than in those with TAA. In contrast, the incidence of hypertension (91% vs 81%, p <0.05), saccular-type aneurysm (61% vs 7%, p <0.0001), and body mass index (24.1 +/- 3.1 vs 23.2 +/- 3.5, p <0.05) were significantly higher in patients with TAA than in those with AAA. Multivariate stepwise logistic analysis revealed that CAD (odds ratio [OR] 3.65; 95% confidence interval [CI] 2.12 to 6.42; p <0.0001), COPD (OR 2.05; 95% CI 1.11 to 3.89; p <0.05), and diabetes mellitus (OR 1.85; 95% CI 1.06 to 3.27; p <0.05) were associated with AAA, and that body mass index (OR 9.39; 95% CI 2.0 to 46.8; p <0.01), hypertension (OR 3.09; 95% CI 1.48 to 6.87; p <0.01), and cerebral infarction (OR 2.83; 95% CI 1.25 to 6.50; p <0.05) were associated with TAA. In conclusion, atherosclerotic profiles are significantly different between patients with TAA and patients with AAA. This result suggests the possibility that mechanisms underlying the development of aortic aneurysms may differ between TAA and AAA, and, from the perspective of prevention, provides further stimulus for the modification of key risk factors for atherosclerosis.
The American Journal of Cardiology 03/2008; 101(5):696-9. · 3.37 Impact Factor
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ABSTRACT: A 30-year-old man with severe back and abdominal pain was referred to our hospital because of a recurrence of acute type B aortic dissection. A computed tomography scan showed a 3-channel dissection and a severe narrowing of the true lumen of the descending aorta to the abdominal aorta because of the expansion of the newly formed second false lumen. Although laboratory testing, including creatine phosphokinase, lactate dehydrogenase, and lactate levels, indicated no visceral ischemia, abdominal pain requiring narcotics treatment had to be continued for more than 1 week. Based on the symptoms and computed tomography findings, the patient finally underwent aortic replacement, fenestration, and a reconstruction of the inferior mesenteric artery, after which the abdominal pain disappeared. Operative findings confirmed a pale shrunken intestine, indicative of mesenteric ischemia. The present case is a good demonstration revealing that mesenteric ischemia still remains a diagnostic challenge, and suggests that currently available laboratory markers are not sensitive enough to detect the presence of ischemia. A strong clinical suspicion for mesenteric ischemia may be the only key to preventing a catastrophic outcome in this condition.
Annals of thoracic and cardiovascular surgery: official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia 11/2007; 13(5):360-4. · 0.69 Impact Factor
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ABSTRACT: Mutations in the genes for transforming growth factor-beta receptor (TGFBR) have been identified in patients with Marfan syndrome (MFS) and Marfan-like connective tissue disorders. There are several syndromes associated with mutations in TGFBR genes, including Loeys-Dietz syndrome (LDS), MFS2, Furlong syndrome, and Shprintzen-Goldberg syndrome. However, with the exception of the first report by Loeys et al, the phenotypic features of patients with TGFBR gene mutations have not been precisely reported.
A total of 18 patients suspected of having MFS were recruited and 7 were diagnosed with MFS and mutations in FBN1. Among the remaining 11 patients, 1 patient had mutations in TGFBR1, 2 had mutations in TGFBR2, and 1 had mutations in COL3A1. The clinical manifestations of the 3 patients with TGFBR gene mutations were examined according to the list of 36 clinical features described in the first report by Loeys et al. The clinical manifestations of these 3 patients differed from those previously observed in patients with MFS2, Furlong syndrome, and Shprintzen-Goldberg syndrome. Thus, the most probable diagnosis of these 3 patients was LDS, despite the fact that they presented with only a fraction of the 36 clinical features associated with LDS.
Although the number of the patients was limited, the findings support the notion that mutations in the TGFBR gene may be associated with greater phenotypic heterogeneity than previously reported.
Circulation Journal 09/2007; 71(8):1305-9. · 3.77 Impact Factor
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Koji Miyamoto,
Kazuhiro Nishigami,
Noritoshi Nagaya, Koichi Akutsu,
Masaaki Chiku,
Masataka Kamei,
Toshihiro Soma,
Shigeki Miyata,
Masahiro Higashi,
Ryoichi Tanaka,
Takeshi Nakatani,
Hiroshi Nonogi,
Satoshi Takeshita
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ABSTRACT: The short-term clinical benefits of bone marrow mononuclear cell transplantation have been shown in patients with critical limb ischemia. The purpose of this study was to assess the long-term safety and efficacy of bone marrow mononuclear cell transplantation in patients with thromboangiitis obliterans.
Eleven limbs (3 with rest pain and 8 with an ischemic ulcer) of 8 patients were treated by bone marrow mononuclear cell transplantation. The patients were followed up for clinical events for a mean of 684+/-549 days (range 103 to 1466 days). At 4 weeks, improvement in pain was observed in all 11 limbs, with complete relief in 4 (36%). Pain scale (visual analog scale) score decreased from 5.1+/-0.7 to 1.5+/-1.3. An improvement in skin ulcers was observed in all 8 limbs with an ischemic ulcer, with complete healing in 7 (88%). During the follow-up, however, clinical events occurred in 4 of the 8 patients. The first patient suffered sudden death at 20 months after transplantation at 30 years of age. The second patient with an incomplete healing of a skin ulcer showed worsening of the lesion at 4 months. The third patient showed worsening of rest pain at 8 months. The last patient developed an arteriovenous shunt in the foot at 7 months, which spontaneously regressed by 1 year.
In the present unblinded and uncontrolled pilot study, long-term adverse events, including death and unfavorable angiogenesis, were observed in half of the patients receiving bone marrow mononuclear cell transplantation. Given the current incomplete knowledge of the safety and efficacy of this strategy, careful long-term monitoring is required for future patients receiving this treatment.
Circulation 01/2007; 114(24):2679-84. · 14.74 Impact Factor
