Hideaki Takai

Successful surgical repair for Emery–Dreifuss muscular dystrophy valvular disease with long-term follow-up

Emery-Dreifuss muscular dystrophy (EDMD) is an X-linked muscular dystrophy in which cardiac involvement can be serious. The disease progresses rapidly and the prognosis is strongly associated with cardiac involvement. We present the case of a 49-year-old man who was admitted with shortness of breath. Echocardiography revealed a huge right atrium and severe tricuspid regurgitation caused by annular dilatation and destruction of leaflets and chordae by pacemaker leads. Tricuspid valve replacement, right atrial plication and implantation of epicardial pacemaker leads were performed. The patient is in a good condition seven years after surgery. Cardiac transplantation is the desired therapy for valvular disease in EDMD cardiomyopathy, but this case indicates that valve replacement is also acceptable if performed with the appropriate timing.

Carotid artery stenting via a femoral bypass graft: technical note.

BACKGROUND We described a case of endoluminal stent placement for a cervical internal carotid artery stenosis in which access was obtained via the femoral bypass graft. CASE DESCRIPTION A 70-year-old man with known disease of the carotid, peripheral, and coronary arteries as well as congestive heart failure presented for endoluminal revascularization of a severe right internal carotid artery stenosis. Transradial access was complicated by the left subclavian artery occlusion and hypercalcified aortic arch. Bilateral femoral artery was replaced with bypass graft because of atherosclerosis obliterans. An alternative approach was attempted via the exposed left femoral bypass graft. The left inguinal region was incised, and the left common femoral-popliteal bypass graft was exposed. After placement of a purse string suture at the puncture site, the guiding sheath was introduced into the graft and positioned into the right common carotid artery. Stenting was successfully performed, and the suture was tied after withdrawing the sheath. CONCLUSIONS This novel approach should be considered for endovascular procedures for which access to the carotid artery is limited.

Consideration of prosthesis-patient mismatch and left ventricular mass regression after implantation of the Carpentier-Edwards pericardial valve in elderly Japanese patients: body surface area may be irrelevant.

The assessment of prosthesis patient mismatch (PPM) for small aortic annulus is important for prognosis after aortic valve replacement (AVR). Recent investigations have demonstrated that PPM occurs in AVR patients with an indexed effective orifice area (iEOA) of less than 0.85 cm2/m2. We investigated hemodynamic performance and left ventricular mass (LVM) regression after AVR. Eighteen patients who underwent AVR using a 19-mm Carpentier-Edwards pericardial (CEP) valve without annular enlargement were studied by echocardiography and Doppler examination 4 months after AVR. Patients were divided into two groups on the basis of their body surface area (BSA); the smaller BSA (group S, 1.14–1.36 m2, nine patients) and the larger BSA (group L, 1.40–1.83 m2, nine patients). Of these 18 patients, ten underwent isolated AVR, and five underwent AVR with coronary artery bypass graft; (i.e., double valvular replacement, AVR with maze procedure, and AVR with mitral valvulophasty. There were no statistically significant differences between the two groups, except for age (group S, 78.3 ± 2.5 years; group L, 73.6 ± 2.4 years). There was no significant difference for the iEOA during the late phase at rest (group S, 1.10 ± 0.26 cm2; group L, 1.02 ± 0.28 cm2). However, there was a significant difference for the LVM regression between the preoperative and postoperative values (group S, 243 ± 23.6 mg/cm2 [pre], 190 ± 16.9 mg/cm2 [post]; group L, 302 ± 13.7 mg/cm2 [pre], 199 ± 16.7 mg/cm2 [post]). In elderly Japanese patients with a BSA of less than 18 m2, we demonstrated LVM regression and avoidance of PPM after implantation of the aortic 19-mm CEP valve.

Demonstration and Operative Influence of Low Prime Volume Closed Pump

Various improvements have been made in cardiopulmonary bypass (CPB) in the past few decades. We designed a new type of CPB to reduce the secretion of systemic inflammatory markers. We used a low prime volume pump (LPVP), completely closed CPB circuit and examined coagulant factors and inflammatory cytokines. In this study, we demonstrate the efficacy of LPVP using molecular biological data. Fourteen patients were randomized prospectively into two groups: Group L patients underwent LPVP (n = 8) and Group N patients underwent normal prime volume CPB (n = 6). We measured thrombin-antithrombin III complex (TAT), complement factor (C3a), and interleukin (IL)-10 levels at four time points. TAT (66.1 ± 15.1 ng·mL−1), C3a (1895 ± 282 ng·mL−1) and IL-10 (486 ± 114 pg·mL−1) levels in Group N were significantly higher than in Group L (TAT, 19.5 ± 4.4 ng·mL−1; IL-10, 105 ± 24.6 pg·mL−1; C3a, 1349 ± 369 ng·mL−1) immediately following CPB. LPVP demonstrated a lower systemic inflammatory response compared to normal prime volume CPB, as assessed using a molecular biological approach.