Endovascular Therapy for Vascular Graft Infection After Multiple Bypass Surgeries for Aorto-Iliac Occlusive Disease.

Background: Vascular graft infection is a very complex disease. Although complete excision of the infected grafts with extra-anatomic bypass or in situ reconstruction is a general treatment strategy, some concerns including reinfection in the new graft remain.

Case Report: An 88 year-old man presented to the hospital with abdominal swelling and bleeding.

Rapid Formation of an Infected Coronary Artery Aneurysm With Stent Fracture.

Infected coronary artery aneurysm (ICAA) is a rare but fatal disease. We describe a case of rapid formation of ICAA with fracture of an intracoronary stent observed on coronary angiography and cardiac computed tomography. Surgery with resection of the aneurysm and coronary artery bypass grafting was performed successfully.

Hokuriku-plus familial hypercholesterolaemia registry study: rationale and study design.

Introduction: Familial hypercholesterolaemia (FH) is an autosomal-dominant inherited genetic disease. It carries an extremely high cardiovascular risk associated with significantly elevated low-density lipoprotein (LDL) cholesterol. The diagnostic rate of this disease in some European nations is quite high, due to the presence of multiple prospective registries.

Huge right ventricular mass lesion associated with genital malignant tumor: a case report.

Background: Primary heart tumors are rare, whereas metastatic heart tumors occur more frequently.

Case Presentation: We report a case of a 75-year-old Japanese woman who had metastatic heart tumors of the right ventricle. Although she initially received antibiotic therapy following a diagnosis of pneumonia and pleuritis, her symptoms worsened, and she developed dyspnea and bilateral lower limb edema.

Impact of combined lipid lowering and blood pressure control on coronary plaque: myocardial ischemia treated by percutaneous coronary intervention and plaque regression by lipid lowering and blood pressure controlling assessed by intravascular ultrasonography (MILLION) study.

The aim of the study was to elucidate the aggressive reduction of both low-density lipoprotein cholesterol (LDL-C) and blood pressure (BP) reduced coronary atherosclerotic plaque volume compared with a standard treatment of LDL-C and BP in Japanese patients with coronary artery disease (CAD). This study is a prospective, randomized, and open-labelled with a blind-endpoint evaluation study. A total of 97 patients (81 men, mean age 62.

Impact of combined lipid lowering with blood pressure control on coronary plaque regression: rationale and design of MILLION study.

A line of epidemiological studies suggests that the accumulation of coronary risk factors promotes the progression of coronary atherosclerosis. Recent clinical studies showed that aggressive low-density lipoprotein (LDL) cholesterol-lowering therapy using statins could regress coronary atheroma and reduce major cardiovascular events. Additionally, therapy that controlled amlodipine-based blood pressure reduced major cardiovascular events in patients with hypertension compared with an atenolol-based regimen.

A novel mutation in the transmembrane nonpore region of the KCNH2 gene causes severe clinical manifestations of long QT syndrome.

Background: Long QT syndrome (LQTS) is characterized by prolonged ventricular repolarization and variable clinical course with arrhythmia-related syncope and sudden death. Mutations in the nonpore region of the LQTS-associated KCNH2 gene (also known as hERG) are mostly associated with coassembly or trafficking abnormalities, resulting in haplotype insufficiency and milder clinical phenotypes compared with mutations in the pore domain.

Objective: To investigate the effect of a nonpore mutation on the channel current, which was identified from an LQTS family with severe clinical phenotypes.

Polymorphic ventricular tachycardia in a patient with hypertrophic cardiomyopathy and digitalis intoxication.

We report the case of a 74-year-old woman who presented with recurrent episodes of polymorphic ventricular tachycardia (PVT) with a normal QT interval due to digitalis intoxication (serum digoxin concentration, 5.0 ng/mL) and severe hyperkalemia (serum potassium level, 8.3 mEq/L).

Pulmonary hypertension associated with veno-occlusive disease in systemic sclerosis: Insight into the mechanism of resistance to vasodilator.

We report a case with pulmonary veno-occlusive disease (PVOD) associated with systemic sclerosis which exhibits strong resistance to pulmonary vasodilator. A 55-year-old female with severe pulmonary hypertension was admitted to our hospital to be introduced to epoprostenol infusion therapy. She was diagnosed as having pulmonary arterial hypertension (PAH) associated with systemic sclerosis at the age of 51.

Coronary arteriovenous fistulas complicated by complete atrioventricular block: A case report.

We report the case of a patient with bilateral coronary arteriovenous fistulas (CAVFs) connecting the right coronary artery and left circumflex coronary artery with the right atrium who had progression of first-degree atrioventricular (AV) block to complete AV block during a 4-year period. The His bundle electrogram revealed that the complete AV block was the result of a block at the level of the AV node. Dipyridamole stress thallium-201 myocardial imaging showed decreased perfusion in the inferoapical wall.

Multiple noncoding exons 1 of nuclear receptors NR4A family (nerve growth factor-induced clone B, Nur-related factor 1 and neuron-derived orphan receptor 1) and NR5A1 (steroidogenic factor 1) in human cardiovascular and adrenal tissues.

Objective: Nuclear receptors are involved in a wide variety of functions, including aldosteronogenesis. Nuclear receptor families NR4A [nerve growth factor-induced clone B (NGFIB), Nur-related factor 1 (NURR1) and neuron-derived orphan receptor 1 (NOR1)] and NR2F [chicken ovalbumin upstream promoter-transcription factor 1 (COUP-TFI), COUP-TFII and NR2F6) activate, whereas NR5A1 [steroidogenic factor 1 (SF1)] represses CYP11B2 (aldosterone synthase) gene transcription. The present study was undertaken to elucidate the mechanism of differential regulation of nuclear receptors between cardiovascular and adrenal tissues.

Impact of out-stent plaque volume on in-stent intimal hyperplasia: results from serial volumetric analysis with high-gain intravascular ultrasound.

Background: Changes in out-stent plaque volume can be related to in-stent intimal hyperplasia. However, few data exist regarding the impact of out-stent plaque volume on in-stent intimal hyperplasia.

Methods: We prospectively performed volumetric intravascular ultrasound in 46 stable coronary patients (34 males, mean age of 66 years) immediately as well as 18 months after stenting.

A KCR1 variant implicated in susceptibility to the long QT syndrome.

The acquired long QT syndrome (aLQTS) is frequently associated with extrinsic and intrinsic risk factors including therapeutic agents that inadvertently inhibit the KCNH2 K(+) channel that underlies the repolarizing I(Kr) current in the heart. Previous reports demonstrated that K(+) channel regulator 1 (KCR1) diminishes KCNH2 drug sensitivity and may protect susceptible patients from developing aLQTS. Here, we describe a novel variant of KCR1 (E33D) isolated from a patient with ventricular fibrillation and significant QT prolongation.

An enhanced device for transluminal retrieval of vascular stents without surgical procedures: experimental studies.

Background: Although efforts have been focused on developing endovascular procedures by which intravascular devices such as stents could be effectively deployed, few data exist regarding devices for the nonsurgical retrieval of deployed stents. Therefore, we designed to enable retrieval of deployed stents without a surgical procedure.

Methods: The device consisted of four components: ultra-low profile forceps with 2.

Idiopathic dissection from left subclavian artery to brachial artery: Spontaneous repair with conservative management.

We report an unusual case of a 58-year-old female with idiopathic dissection of the left subclavian artery to the brachial artery which provoked vessel narrowing in the acute phase and was spontaneously repaired without surgical procedures in the chronic phase. We describe the serial imaging findings of the angiography and ultrasonography which demonstrate restoration of the dissection. In carefully selected patients, conservative management could be an alternative treatment to surgery or stenting with an excellent outcome.

Long QT syndrome and associated gene mutation carriers in Japanese children: results from ECG screening examinations.

LQTS (long QT syndrome) is caused by mutations in cardiac ion channel genes; however, the prevalence of LQTS in the general population is not well known. In the present study, we prospectively estimated the prevalence of LQTS and analysed the associated mutation carriers in Japanese children. ECGs were recorded from 7961 Japanese school children (4044 males; mean age, 9.

Impact of QT variables on clinical outcome of genotyped hypertrophic cardiomyopathy.

Background: Although QT variables such as its interval and/or dispersion can be clinical markers of ventricular tachyarrhythmia, few data exist regarding the role of QT variables in genotyped hypertrophic cardiomyopathy (HCM). Therefore, we analyzed QT variables in genotyped subjects with or without left ventricular hypertrophy (LVH).

Methods: QT variables were analyzed in 111 mutation and 43 non-mutation carriers who were divided into three groups: A, those without ECG abnormalities and echocardiographically determined LVH (wall thickness > or =13 mm); B, those with ECG abnormalities but LVH; and C, those with ECG abnormalities and LVH.

Assessment of QT intervals and prevalence of short QT syndrome in Japan.

Background: Long QT syndrome causes ventricular tachyarrhythmias and sudden death. Recently, a short QT interval has also been shown to be associated with an increased risk of tachyarrhythmia and sudden death. However, the prevalence of short QT syndrome is not well-known.

Differences in the diagnostic value of various criteria of negative T waves for hypertrophic cardiomyopathy based on a molecular genetic diagnosis.

Differences in the diagnostic value of a variety of definitions of negative T waves for HCM (hypertrophic cardiomyopathy) have not yet been clarified, resulting in a number of definitions being applied in previous studies. The aim of the present study was to determine the most accurate diagnostic definition of negative T waves for HCM in genotyped populations. Electrocardiographic and echocardiographic findings were analysed in 161 genotyped subjects (97 carriers and 64 non-carriers).

Analysis of ankyrin-B gene mutations in patients with long QT syndrome.

Objective: To identify the ankyrin-B gene mutations that cause long QT syndrome (LQTS) and determine the prevalence of such mutations in Japanese patients with LQTS.

Methods: We conducted a search for ankyrin-B gene mutation in 78 unrelated patients with LQTS (28 males and 50 females, aged 2 to 89 years). With informed consent from all the subjects and/or their parents, genomic DNA was purified from the white blood cells of the patients and amplified using polymerase chain reaction (PCR).

Differences in diagnostic value of four electrocardiographic voltage criteria for hypertrophic cardiomyopathy in a genotyped population.

The diagnostic value of various classic electrocardiographic (ECG) voltage criteria for hypertrophic cardiomyopathy (HC) has not been established in a genotyped population. This study aimed to determine the most accurate diagnostic definition of classic ECG voltage criteria for detecting carriers of HC. ECG and echocardiographic findings were analyzed in 161 genotyped subjects (97 genetically affected, 64 unaffected) from 20 families with disease-causing mutations in 4 genes.

A novel mutation in the cardiac myosin-binding protein C gene is responsible for hypertrophic cardiomyopathy with severe ventricular hypertrophy and sudden death.

It has been demonstrated previously that clinical phenotypes of HCM (hypertrophic cardiomyopathy) caused by mutations in the cardiac MyBP-C (myosin-binding protein C) gene show late onset, low penetrance and favourable clinical course. However, we have encountered severe phenotypes in several carriers of the MyBP-C gene mutations. The aim of the present study was to screen novel MyBP-C gene mutations in patients with HCM and to investigate the genetic differences in affected subjects with severe phenotypes.