Naoki Kanemitsu

Coronary artery bypass graft in a patient with Fabry's disease

Fabrys disease is a lysosomal storage disease characterized by intracellular accumulation of ceramide trihexoside resulting from alpha-galactosidase A deficiency. While the heart is often involved, coronary artery disease and its management in Fabrys disease patients are extremely rare clinical entities. We report a case of a 72-year-old man with left main disease in Fabrys disease with special consideration of the arterial wall pathology.

Histopathological evaluation of aortic dissection: a comparison of congenital versus acquired aortic wall weakness

OBJECTIVES The aim of this study was to identify pathological changes of aortic dissection based on histopathological evaluation of aortic wall weakness by comparing patients with and without congenital abnormalities. METHODS We reviewed records of patients who underwent repair for dissection-related aortic disease between 2008 and 2015. Fifty patients (20 men and 30 women; mean age 66.9 ± 14.0 years) who underwent surgery with subsequent histopathological examination of the aortic wall were divided into 2 groups. Group 1 had congenital abnormalities, including Marfan syndrome and bicuspid aortic valve (n = 5), and Group 2 had no congenital abnormalities (n = 45). We compared the histopathological characteristics of the aortic wall in these patients. RESULTS There were significant differences in age and body surface area between the 2 groups. Although 80% of Group 1 patients developed dissection at the middle of the media, all Group 2 patients developed dissection at the outer one-third of the media, which is along the pathway of the vasa vasorum of the aortic wall. Both groups showed the same extent of degeneration of the vasa vasorum. Group 1 showed a severe score of mucoid extracellular matrix accumulation in the aortic media. CONCLUSIONS Although it may be multifactorial, congenital maldevelopment of the media tends to result in dissection of the centre of the media, and acquired aortic wall weakness is concentrated in the outer third of the media. Degeneration of the vasa vasorum may be an important emerging substrate for developing aortic dissection.

Impact of concomitant tricuspid regurgitation on long-term outcomes in severe aortic stenosis

AIMS Tricuspid regurgitation (TR) has been reported to be associated with worse survival in various heart diseases, but there are few data in aortic stenosis (AS). METHODS AND RESULTS In the Contemporary Outcomes after Surgery and Medical Treatment in Patients with Severe Aortic Stenosis (CURRENT AS) Registry enrolling 3815 consecutive patients with severe AS, there were 628 patients with moderate or severe TR (TR group) and 3187 patients with no or mild TR (no TR group). The study patients were subdivided into the initial aortic valve replacement (AVR) stratum (n = 1197) and the conservative stratum (n = 2618) according to treatment strategy. The primary outcome measure was a composite of aortic valve-related death or hospitalization due to heart failure. The 5-year freedom rate from the primary outcome measure was significantly lower in the TR group than in the no TR group (49.1% vs. 67.3%, P < 0.001). Even after adjusting for confounders, the excess risk of TR relative to no TR for the primary outcome measure remained significant [hazard ratio (HR): 1.25, 95% confidence interval (CI): 1.06-1.48; P = 0.008]. The trend for the excess adjusted risk in the TR group was consistent in the initial AVR and the conservative strata (HR 1.55, 95% CI: 0.97-2.48; P = 0.07; HR 1.22, 95% CI: 1.02-1.46; P = 0.03, respectively). In the initial AVR stratum, the 5-year freedom rate from the primary outcome measure was not different between the two groups with (n = 56) or without (n = 91) concomitant tricuspid annuloplasty (61.5% vs. 72.1%, P = 0.48). CONCLUSION The presence of clinically significant TR concomitant with severe AS is associated with a poor long-term outcome, regardless of the initial treatment strategy.

Coronary artery and supra-aortic vessel revascularization without aortic clamping for Takayasu arteritis

Takayasu arteritis (TA) is a rare idiopathic large-vessel vasculitis involving the aorta and its major branches. Coronary artery disease and its surgical management in patients with TA is also a rare clinical entity. We report the case of a 73-year-old woman with TA and 3-vessel coronary artery disease in whom we performed off-pump coronary artery bypass graft concomitant with supra-aortic vessel revascularization.

Giant atrial septal aneurysm originating from the right coronary artery

Giant coronary artery aneurysm is a rare clinical entity and its involvement in the interatrial space is extremely rare. We here report the rare surgical case of a 67-year old man with giant right coronary artery aneurysm located in the atrial septum with fistula formation to the right atrium, complicated with congestive heart failure, rapid atrial fibrillation and left atrial appendage thrombus. The patient eventually recovered fully without sequelae.

Surgical resection of two independent primary intimal sarcomas in the left atrium.

Primary intimal sarcoma of the heart is an extremely rare tumor that is known to have a very poor prognosis. We present a case of a 65-year-old man who suffered from deteriorating congestive heart failure due to a severe mitral stenosis caused by a large mobile left atrial tumor. The patient underwent an emergency operation of the tumor in the left atrium. The tumor was attached to the inferior wall of the left atrium. After the resection of the tumor, a second tumor on the interatrial septum, which had not been detected in the preoperative investigation, was discovered and resected. The patient developed acute respiratory failure soon after the operation and succumbed to his illness. The appearance of the main tumor was cauliflower-like, which strongly suggested the possibility of malignancy. Immunohistochemistry revealed that the neoplastic cells were positive for vimentin, desmin, p16, and especially murine double minute 2 (MDM-2). The first tumor was CD34 positive and cdk4 negative, but the second tumor was more anaplastic and CD34 negative and cdk4 positive, which suggests a different origin of the two tumors. The two tumors were diagnosed as intimal sarcomas by MDM-2, which is currently considered a conclusive marker. This is an exceptionally rare case of two simultaneous and possibly independent primary intimal sarcomas in the left atrium.