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Competing Interests: The authors report no financial relationships or conflicts of interest regarding the contents of the manuscript.

Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype-phenotype correlation in patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype-phenotype correlation. Detailed clinical information was available for 64 patients in our database who were diagnosed with OTOF-related hearing loss. As reported previously, most of the patients (90.6%) showed a "typical" phenotype; prelingual and severe-to-profound hearing loss. Forty-seven patients (73.4%) underwent cochlear implantation surgery and showed successful outcomes; approximately 85-90% of the patients showed a hearing level of 20-39 dB with cochlear implant and a Categories of Auditory Performance (CAP) scale level 6 or better. Although truncating mutations and p.Arg1939Gln were clearly related to severe phenotype, almost half of the patients with one or more non-truncating mutations showed mild-to-moderate hearing loss. Notably, patients with p.His513Arg, p.Ile1573Thr and p.Glu1910Lys showed "true" auditory neuropathy-like clinical characteristics. In this study, we have clarified genotype-phenotype correlation and efficacy of cochlear implantation for OTOF-related hearing loss patients in the biggest cohort studied to date. We believe that the clinical characteristics and genotype-phenotype correlation found in this study will support preoperative counseling and appropriate intervention for OTOF-related hearing loss patients., (© 2021. The Author(s).)

Downstream paclitaxel particle embolization for nonhealing ischemic ulcers and systemic adverse effects caused by a paclitaxel drug-coated balloon are of concern, and safety measures to prevent these adverse risks are needed. To reduce distal particle embolization and movement of the paclitaxel particles to systemic blood flow during drug-coated balloon inflation, proximal balloon occlusion using a sheathless temporary occlusion balloon-guiding catheter and extraction by manual aspiration of the paclitaxel-containing blood through the catheter are good treatment options to overcome these risks. Here, we introduce this method with tips and tricks, and demonstrate initial experience of this technique., (© 2021 The Author(s).)

Background: Several recent reports have shown that a stentless interventional procedure using rotational atherectomy followed by drug-coated balloon (DCB) treatment (RA/DCB) is a potent revascularization therapy for calcified de novo lesions even in the new-generation drug-eluting stent era; however, the role of the RA/DCB procedure for noncalcified de novo lesions remains unclear., Methods: A total of 47 consecutive patients (53 lesions) who underwent RA/DCB for coronary de novo lesions were enrolled. According to the presence or absence of severe calcification at target lesions on fluoroscopy, the 47 patients were divided into the noncalcified cases (n = 12) and the calcified cases (n = 35), and the 53 lesions were divided into the noncalcified lesions (n = 14) and the calcified lesions (n = 39)., Results: The noncalcified cases tended to have a higher frequency of bleeding risk and had a significantly lower prevalence of dual antiplatelet therapy compared with the calcified cases. The main lesion-specific factors for the RA/DCB procedure among the noncalcified lesions were presence of left circumflex coronary artery ostial lesion. The final burr size, DCB diameter used, and angiographic success rate did not significantly differ between the 2 groups. The noncalcified lesions had a larger reference diameter and a shorter lesion length than the calcified lesions, whereas acute gain and late lumen loss did not differ between the 2 groups. Nine-month clinical outcomes were comparable between the 2 groups., Conclusions: Under drug-eluting stent-unsuitable clinical or lesion conditions, acute and midterm outcomes of RA/DCB for noncalcified de novo lesions might be comparable with those for calcified de novo lesions., (© 2021 The Authors.)

MYO6 is known as a genetic cause of autosomal dominant and autosomal recessive inherited hearing loss. In this study, to clarify the frequency and clinical characteristics of hearing loss caused by MYO6 gene mutations, a large-scale genetic analysis of Japanese patients with hearing loss was performed. By means of massively parallel DNA sequencing (MPS) using next-generation sequencing for 8074 Japanese families, we found 27 MYO6 variants in 33 families, 22 of which are novel. In total, 2.40% of autosomal dominant sensorineural hearing loss (ADSNHL) in families in this study (32 out of 1336) was found to be caused by MYO6 mutations. The present study clarified that most cases showed juvenile-onset progressive hearing loss and their hearing deteriorated markedly after 40 years of age. The estimated hearing deterioration was found to be 0.57 dB per year; when restricted to change after 40 years of age, the deterioration speed was accelerated to 1.07 dB per year. To obtain supportive evidence for pathogenicity, variants identified in the patients were introduced to MYO6 cDNA by site-directed mutagenesis and overexpressed in epithelial cells. They were then assessed for their effects on espin1-induced microvilli formation. Cells with wildtype myosin 6 and espin1 co-expressed created long microvilli, while co-expression with mutant constructs resulted in severely shortened microvilli. In conclusion, the present data clearly showed that MYO6 is one of the genes to keep in mind with regard to ADSNHL, and the molecular characteristics of the identified gene variants suggest that a possible pathology seems to result from malformed stereocilia of the cochlear hair cells.

TECTA is well known as a causative gene for autosomal dominant mid-frequency hearing loss observed in various populations. In this study, we performed next-generation sequencing analysis of a large Japanese hearing loss cohort, including eight hundred and twelve (812) subjects from unrelated autosomal dominant hearing loss families, to estimate the prevalence and phenotype-genotype correlations in patients with TECTA mutations. The prevalence of TECTA mutations in Japanese autosomal dominant sensorineural hearing loss families was found to be 3.2%. With regard to the type of hearing loss, the patients with mutations in the nidogen-like domain or ZA domain of TECTA showed varied audiograms. However, most of the patients with mutations in the ZP domain showed mid-frequency hearing loss. The rate of hearing deterioration in TECTA -associated hearing loss patients and in the normal hearing Japanese control population were the same and regression lines for each group were parallel. We carried out haplotype analysis for four families which had one recurring missense variant, c.5597C>T (p.Thr1866Met). Our results revealed four different haplotypes, suggesting that this mutation occurred independently in each family. In conclusion, TECTA variants represent the second largest cause of autosomal dominant sensorineural hearing loss in Japan. The hearing loss progression observed in the patients with TECTA mutations might reflect presbycusis. The c.5597C>T mutation occurred in a mutational hot spot and is observed in many ethnic populations.

Objective The purpose of the present study was to examine the relationship between thrombocytopenia at baseline and in-hospital outcomes in unselected patients undergoing elective percutaneous coronary intervention (PCI) in Japan. Methods Among a total of 1,247 consecutive elective PCI-treated patients, patients with a baseline platelet count 150,000-449,000/μL and 50,000-149,000/μL were assigned to the normal platelet (n=1,009) and thrombocytopenia (n=226) groups, respectively. The thrombocytopenia group was further divided into the mild thrombocytopenia (100,000-149,000/μL, n=187) and moderate thrombocytopenia (50,000-99,000/μL, n=39) groups. Results The angiographic success rate of PCI and in-hospital mortality rate did not differ to a statistically significant extent between the normal platelet and thrombocytopenia groups or between the mild thrombocytopenia and moderate thrombocytopenia groups, whereas the moderate thrombocytopenia group had a significantly higher rate of access site-related bleeding complications than the normal platelet group. According to a multivariate analysis, moderate thrombocytopenia was an independent predictor of access site-related bleeding complications. Conclusion Among patients with mild to moderate thrombocytopenia, elective PCI might be feasible and effective in the short term; however, more attention should be paid to access site-related bleeding complications, particularly in patients with moderate thrombocytopenia.

A 61-year-old man with essential thrombocythemia (ET) presented with acute myocardial infarction (AMI) and underwent primary percutaneous coronary intervention. After stent deployment from the left main (LM) to the left anterior descending artery, intravascular ultrasound revealed thrombi formation in the whole stent. Two days later, optical frequency domain imaging confirmed stent malapposition and thrombi remaining in only the LM. The stent malapposition and ET might have contributed to this phenomenon. He underwent an additional stent expansion and aggressive anti-thrombotic regimen. AMI complicated with ET carries increased risks of in-stent thrombi formation and requires careful revascularization and aggressive pharmacotherapy.

A 77-year-old man was referred to our hospital for angina on effort. Coronary angiography and computed tomography demonstrated a single coronary artery arising from the right sinus of Valsalva. The left circumflex coronary artery (LCx) anomalously deriving near from the ostium of right coronary artery exhibited severe stenosis in the bifurcation of the obtuse marginal branch. Although the bifurcation lesion still remains a therapeutic challenge for guide extension catheter (GEC)-based percutaneous coronary intervention, under the guidance of intravascular ultrasound imaging, we successfully implanted an everolimus-eluting stent at the bifurcated LCx lesion and performed kissing balloon inflation using 0.014- and 0.010-inch systems through GECs.

An 83-year-old man presented with recurrent acute coronary syndrome (ACS) at the left main coronary artery (LMCA) complicated with ostial chronic total occlusion (CTO) in the right coronary artery (RCA) (RCA-CTO). At the first LMCA-ACS approximately 1 year earlier, he had undergone LMCA-crossover stenting with a biolimus-eluting stent in the presence of RCA-CTO. At the second LMCA-ACS, we angiographically confirmed severe in-stent restenosis in the distal LMCA, in addition to angled severe stenosis in the just proximal LCx, and performed primary PCI for the LMCA bifurcation lesion under intra-aortic balloon pumping support. Because of difficulty in crossing a guidewire through the just proximal LCx lesion, we first performed rotational atherectomy against the LMCA in-stent eccentric lesion. After successfully crossing the guidewire into the LCx, we added balloon dilation with kissing balloon inflation followed by alternate drug-coated balloon dilation. An eight-month follow-up coronary angiography revealed no further vessel narrowing in the LMCA bifurcation lesion.

An 89-year-old woman appeared to have acute coronary syndrome at the left main trunk (LMT) complicated with severe aortic stenosis, moderate-severe mitral regurgitation, depressed left ventricular (LV) function, and multivessel disease. Because of sustained hypotension even under intra-aortic balloon pumping support during emergency coronary angiograhy, we performed primary percutaneous coronary intervention solely for the LMT lesion using a bare metal stent, leading to recovery from the shock state. On the second hospital day, based on our heart-team consensus, we performed aortic valve replacement and coronary artery bypass grafting surgery, and added edge-to-edge repair (Alfieri stitch) of the mitral valve, resulting in complete revascularization and dramatically improved LV function.

A J-shaped or U-shaped curve phenomenon might exist between systolic blood pressure (SBP) or pulse pressure (PP) at admission and in-hospital mortality in Japanese patients with acute myocardial infarction (AMI) undergoing primary percutaneous coronary intervention (PCI). However, data regarding a relationship between mean blood pressure (MBP) at admission and in-hospital outcome in AMI patients undergoing primary PCI are still lacking in Japan.A total of 1,413 primary PCI-treated AMI patients were classified into quintiles based on admission MBP (< 79 n = 283, 79-91 n = 285, 92-103 n = 285, 104-115 n = 279, and ≥ 116 mmHg n = 281). Patients with MBP < 79 mmHg had a significantly higher in-hospital mortality, while mortality was not significantly different among the other quintiles: 16.6% (< 79), 4.9% (79-91), 3.9% (92-103), 3.2% (104-115), and 5.0% (≥ 116 mmHg). On multivariate analysis, Killip class ≥ 3 at admission, LMT or multivessels as culprit lesions, admission MBP < 79 mmHg, and age were independent positive predictors of in-hospital mortality, whereas hypercholesterolemia and TIMI 3 flow before/after PCI were negative predictors, while the other MBP categories were not.These results suggest that admission MBP < 79 mmHg might be associated with in-hospital death, and the in-hospital prognostic effects of MBP, the steady component of blood pressure, at admission might be different from those of SBP or PP, the pulsatile component of blood pressure, at admission in Japanese AMI patients undergoing primary PCI.

Rotational atherectomy with/without low-pressure balloon dilation has been a mainstay of interventional treatment for stenosis due to the coronary sequelae of Kawasaki disease (KD). Here, we report a restenosis case of probable coronary sequelae of KD treated with rotational atherectomy with low-pressure 2.5-mm balloon dilation 6 months previously. Under the guidance of optical frequency domain imaging, we performed rotational atherectomy followed by 2.5-mm drug-coated balloon (DCB) dilation for an atherosclerotic restenosis at the inlet of a calcified aneurysm in the proximal left anterior descending coronary artery. Coronary angiography 6 months later showed no apparent progression of vessel narrowing, and we could defer repeat intervention. The present case suggests that rotational atherectomy followed by DCB dilation could be an alternative revascularization therapy of choice in coronary KD sequelae complicated with atherosclerosis.

Arteriovenous fistula (AVF) after trans-radial catheterization is an extremely rare complication. A 61-year-old man experienced a painful swelling in the left radial punctured site. The findings of vascular ultrasound and angiography led to a diagnosis of an iatrogenic radial AVF. We performed continuous compression using a hemostatic band for 24 h after which the radial AVF completely disappeared without vessel occlusion, and no relapse occurred. Eight cases of catheterization-induced radial AVF have been mentioned in the literature, but the treatment has not been noninvasive in any case. This is the first report of an iatrogenic radial AVF that was noninvasively repaired using continuous low-pressure compression with a hemostatic band. < Learning objective: Arteriovenous fistula (AVF) after trans-radial catheterization is a rare complication. The literature reports only 8 cases of catheterization-induced radial AVF and no patient has been cured noninvasively. This is the first report of an iatrogenic radial AVF that was repaired noninvasively. We believe that continuous low-pressure compression using a hemostatic band is an effective therapeutic option for this complication.>.

Objective: The predictors of in-hospital outcomes after primary percutaneous coronary intervention (PCI) for acute myocardial infarction (AMI) complicated with heart failure or cardiogenic shock at presentation remain unclear., Methods: Using the AMI-Kyoto Multi-Center Risk Study database, the clinical background characteristics, angiographic findings, primary PCI results, and in-hospital prognoses were retrospectively compared between primary PCI-treated AMI patients with a Killip class status of ≥2 (Killip 2-4 patients, n=390) and those with a Killip class 1 status (Killip 1 patients, n=1,057)., Results: The Killip 2-4 patients were more likely to have a higher age and proportion of women and exhibited a higher prevalence of previous myocardial infarction, diabetes mellitus and chronic kidney disease or anemia on admission, lower systolic blood pressure (SBP) values on admission, a higher rate of multivessels or left main trunk as the culprit artery, a larger number of diseased vessels, a lower Thrombolysis In Myocardial Infarction (TIMI) grade in the infarct-related artery (IRA) before/after primary PCI and a significantly higher in-hospital mortality rate than the Killip 1 patients. According to a multivariate analysis, age was found to be an independent positive predictor of in-hospital mortality, while admission SBP was an independent positive predictor of in-hospital survival in both groups. In contrast, anemia on admission was found to be an independent predictor of in-hospital death, while the TIMI 3 flow in the IRA after PCI was found to be an independent factor for survival in the Killip 2-4 patients, but not the Killip 1 patients., Conclusion: Anemia on admission and the final TIMI 3 flow in the IRA are critical determinants of in-hospital death in AMI patients with a Killip class status of ≥2 undergoing primary PCI.

Cardiorenal anemia syndrome has recently been receiving greater attention; however, data regarding the relationship between chronic kidney disease (CKD)/anemia on presentation and in-hospital outcome in patients with acute myocardial infarction (AMI) undergoing primary percutaneous coronary intervention (PCI) are still limited in Japan.A total of 1,447 primary PCI-treated AMI patients were classified into 4 groups according to the presence of CKD and/or anemia on hospital admission (with CKD/with anemia n = 222, with CKD/without anemia n = 299, without CKD/with anemia n = 151, without CKD/without anemia n = 775). Angiographic acute results of primary PCI were similar among the 4 groups. The patients with CKD had a significantly higher in-hospital overall mortality rate than the patients without CKD, and in the presence or absence of CKD, patients with anemia tended to have a higher in-hospital mortality rate than the patients without anemia. According to a multivariate analysis, anemia on admission was found to be an independent predictor of in-hospital mortality, whereas admission CKD and admission eGFR were statistically not independent predictors. Moreover, the multivariable adjusted odds ratio of in-hospital death in AMI patients with CKD alone was 1.855 (95% CI 0.929-3.706), and that in AMI patients with CKD/with anemia was 3.384 (95% CI 1.697-6.748).These results suggest that among real-world, unselected Japanese AMI patients undergoing primary PCI, the combination of CKD and anemia on admission confers significant adverse effects on in-hospital mortality.

Background: Downsized devices for less invasive endovascular treatment are gaining more attention., Case Report: An 82-year-old woman was admitted to our hospital with pain and swelling in left lower extremity. Ultrasonography showed much thrombus at the left common femoral, superficial femoral, and popliteal veins, confirming a diagnosis of acute deep vein thrombosis (DVT). For lack of therapeutic effect of systemically administered anticoagulative and thrombolytic agents, we performed catheter intervention against DVT. After insertion of a 4.5 French guiding sheath (Parent Plus, Medikit, Tokyo, Japan) into left popliteal vein, venography showed a total occlusion of the common femoral vein. Even after thrombectomy by aspiration catheter, balloon dilation, and catheter-induced thrombolysis, severe stenosis at the proximal site of the left common iliac vein remained. After confirming the presence of iliac vein compression syndrome (IVCS) by intravascular ultrasound imaging, we successfully implanted a Zilver ® stent (Cook Inc., Bloomington, IN, USA) in the common-external iliac vein through a 4.5 French guiding sheath, leading to an optimal venous-return flow., Conclusions: The present case suggests that in addition to thrombus aspiration, Zilver stent implantation using a 4.5 French guiding sheath has the potential to serve as a minimally invasive strategy for the treatment of IVCS complicated with iliofemoral DVT.< Learning objective: During the interventional treatment of deep vein thrombosis, we should pay attention to procedure-related venous valve dysfunction in addition to bleeding complications and pulmonary embolism. From the point of view, downsized catheter devices might have some advantage with lesser prevalence of those complications.>.

Background: Data regarding the relationship between systolic blood pressure (SBP) at admission and in-hospital outcome in patients with acute myocardial infarction (AMI) undergoing primary percutaneous coronary intervention (PCI) are still lacking in Japan., Methods and Results: A total of 1475 primary PCI-treated AMI patients were classified into quintiles based on admission SBP (<105 mmHg, n=300; 105-125 mmHg, n=294; 126-140 mmHg, n=306; 141-158 mmHg, n=286; and ≥159 mmHg n=289). The patients with SBP<105 mmHg tended to have higher age, previous myocardial infarction, chronic kidney disease (CKD), Killip class≥3 at admission, right coronary artery, left main trunk (LMT), or multivessels as culprit lesions, larger number of diseased vessels, lower Thrombolysis In Myocardial Infarction (TIMI) grade in the infarct-related artery before primary PCI, and higher value of peak creatine phosphokinase concentration. Patients with SBP<105 mmHg had a significantly higher mortality, while mortality was not significantly different among the other quintiles: 24.3% (<105 mmHg), 4.8% (105-125 mmHg), 4.9% (126-140 mmHg), 2.8% (141-158 mmHg), and 5.2% (≥159 mmHg) (p<0.001). On multivariate analysis, Killip class≥3 at admission, LMT or multivessels as culprit lesions, admission SBP<105 mmHg, CKD, and age were the independent positive predictors of in-hospital mortality, whereas admission SBP 141-158 mmHg and TIMI 3 flow after PCI were the negative ones, but admission SBP 105-125 mmHg, admission SBP 126-140 mmHg, and admission SBP≥159 mmHg were not., Conclusions: These results suggest that admission SBP 141-158 mmHg might be correlated with better in-hospital prognosis, whereas admission SBP<105 mmHg was associated with in-hospital death in Japanese AMI patients undergoing primary PCI., (Copyright © 2012 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

Background: Several clinical studies have demonstrated an inverse relationship between systolic blood pressure (SBP) at admission and in-hospital mortality in patients hospitalized for acute myocardial infarction (AMI). However, data on the relation between admission SBP and in-hospital prognosis in AMI patients are still lacking in Japan., Methods and Results: A total of 1211 AMI patients were classified into quintiles based on SBP at hospital admission (<106 mmHg, n = 241; 106-125 mmHg, n = 239; 126-140 mmHg, n = 244; 141-159 mmHg, n = 238; and ≥ 160 mmHg, n = 249). The patients with SBP < 106 mmHg tended to have higher age, Killip class ≥ 3 at admission, right coronary artery, left main trunk, or multivessels as culprit lesions, larger number of diseased vessels, lower Thrombolysis In Myocardial Infarction grade in the infarct-related artery before primary percutaneous coronary intervention (PCI), and higher value of peak creatine phosphokinase concentration. Patients with SBP <106 mmHg had a significantly higher mortality, while mortality was not significantly different among the other quintiles: 25.7% (<106 mmHg), 5.4% (106-125 mmHg), 5.7% (126-140 mmHg), 2.5% (141-159 mmHg), and 5.6% (≥ 160 mmHg) (p<0.001). On multivariate analysis, Killip class ≥ 3 at admission, admission SBP <106 mmHg, and age were the independent positive predictors of in-hospital mortality, whereas admission SBP 141-159 mmHg and primary PCI were the negative ones, but admission SBP 106-125 mmHg, admission SBP 126-140 mmHg, and admission SBP ≥ 160 mmHg were not., Conclusions: These results suggest that admission SBP 141-159 mmHg might be correlated with better in-hospital prognosis, whereas admission SBP <106 mmHg was associated with in-hospital death in Japanese patients hospitalized for AMI., (Copyright © 2011 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

Despite otological surgical progress improving clinical congenital ossicular malformation management, some cases remain inadequately treated. We report 27 cases of congenital ossicular malformation, focusing on reasons for remaining or delayed postoperative hearing loss evaluated in 27 congenital ossicular malformation cases in Kyoto Prefecture from 2002 to 2008. Overall success was 93% (25/27) 6 months postoperatively. Two ears had no hearing improvement and three delayed hearing loss 8 to 48 months postoperatively. The first two ears underwent small fenestration stapedotomy with malleus attachment piston, and the other three tympanoplasty type III using an autologous ossicle or total ossicular replacement prosthesis (TORP) as a columella. We discuss problems and solutions using a malleus attachment piston or prosthesis, preoperative audio-and radiological findings, and operative findings including facial nerve anomaly and congenital cholesteatoma.

Background: Predictors of in-hospital outcome after primary percutaneous coronary intervention (PCI) for acute myocardial infarction (AMI) requiring mechanical support devices such as intra-aortic balloon pumping (IABP) and/or percutaneous cardiopulmonary support (PCPS) remain unclear., Methods and Results: Using the AMI-Kyoto Multi-Center Risk Study database, clinical background, angiographic findings, results of primary PCI, and in-hospital prognosis were retrospectively compared between primary PCI-treated AMI patients requiring mechanical assist devices (with-IABP/PCPS patients, n=275) and those without (without-IABP/PCPS patients, n=1,510). The with-IABP/PCPS patients were more likely to have a larger number of diseased vessels, lower Thrombolysis In Myocardial Infarction (TIMI) grade in the infarct-related artery (IRA) before/after primary PCI, and a significantly higher in-hospital mortality rate than the without-IABP/PCPS patients. On multivariate analysis, the number of diseased vessels > or =2 or diseased left main trunk (LMT) at initial coronary angiography (CAG) was the independent positive predictor of the in-hospital mortality in the with-IABP/PCPS patients, not in the without-IABP/PCPS patients, whereas acquisition of TIMI 3 flow in the IRA immediately after primary PCI was the negative predictor in the without-IABP/PCPS patients, not in the with-IABP/PCPS patients., Conclusions: The number of diseased vessels > or =2 or diseased LMT at initial CAG is an independent risk factor of in-hospital death in primary PCI-treated AMI patients requiring mechanical support devices.

Background: Predictors of suboptimal coronary flow in the infarct-related artery (IRA) after stent-based primary percutaneous coronary intervention (PCI) in patients with acute myocardial infarction (AMI) have not been fully investigated., Methods and Results: Using the AMI-Kyoto Multi-Center Risk Study database, we retrospectively compared clinical manifestations and in-hospital prognosis between AMI patients undergoing stent-based primary PCI with final Thrombolysis In Myocardial Infarction (TIMI) grade < or = 2 in the IRA (nonoptimal group, n=69) and those with final TIMI grade 3 (optimal group, n=1200). The nonoptimal group had higher prevalence of Killip class > or = 3 at admission, higher frequency of mechanical support devices during procedures, larger value of maximal creatine phosphokinase, and a significantly higher in-hospital mortality rate (27.5% for nonoptimal vs. 9.0% for optimal, P<0.001), compared with the optimal group. On multivariate analysis, Killip class > or = 3 at admission was the independent predictor of the final nonoptimal flow (odds ratio 2.33, 95% confidence intervals 1.27-4.26 P=0.006), but TIMI 3 flow before primary PCI and elapsed time (symptom onset-to-admission time)<24h were not., Conclusions: Killip class > or = 3 at admission is an independent predictor of the final nonoptimal flow in AMI patients undergoing primary PCI with stent implantation., (Copyright 2009 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

Background: Recurrent acute myocardial infarction (AMI) is a disastrous condition with high in-hospital morbidity and mortality. However, the relation between location of previous myocardial infarction (MI) and in-hospital outcome in repeat-AMI patients undergoing primary percutaneous coronary intervention (PCI) remains unclear., Methods and Results: Using the AMI-Kyoto Multi-Center Risk Study database, clinical background, angiographic findings, results of primary PCI, and in-hospital prognosis were retrospectively compared between primary PCI-treated AMI patients with previous anterior MI (anterior group, n=151) and those with previous non-anterior MI (non-anterior group, n=157). Clinical backgrounds, angiographic findings, results of primary PCI, and in-hospital outcome did not differ significantly between the two groups. On multivariate analysis, Killip class > or =3 at admission, number of diseased vessels > or =2 or diseased left main trunk at initial coronary angiography, and age were the independent predictors of in-hospital mortality in the recurrent-AMI patients, but not the anterior location of previous MI., Conclusions: These results suggest that among recurrent-AMI patients undergoing primary PCI, in-hospital prognosis mostly depends on the severity of acute heart failure at the onset and the residual myocardial ischemia rather than previous MI sites., (2009 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

Background: Organic coronary artery stenosis is a significant prognostic factor in patients with coronary spastic angina (CSA), so the present study was focused on assessing the impact of intermediate fixed stenosis at sites of provoked spasm on the long-term outcomes of CSA patients., Methods and Results: CSA patients diagnosed on the basis of ergonovine-provoked spasm were enrolled and the clinical background and long-term prognosis of CSA patients with intermediate fixed stenosis at the site of provoked spasm (with-fixed-stenosis group, n=37) and those without fixed stenosis (without-fixed-stenosis group, n=126) were retrospectively compared. During the follow-up period (average 4.01 years for with-fixed-stenosis, 4.47 years for without-fixed-stenosis), the with-fixed-stenosis group had a significantly lower event-free survival rate, as well as a higher frequency of admission for unstable angina and percutaneous coronary intervention than the without-fixed-stenosis group, whereas the survival rate did not differ significantly between the 2 groups. In the multivariate analysis, intermediate fixed stenosis at the site of provoked spasm was a predictor of long-term major adverse cardiac events (MACE)., Conclusions: Intermediate fixed stenosis at the site of ergonovine-provoked spasm is an independent risk factor for MACE during the long-term period in CSA patients.

Background: Recurrent acute myocardial infarction (AMI) is a deteriorated condition with high in-hospital morbidity and mortality, but the predictors of in-hospital outcome after primary percutaneous coronary intervention (PCI) for repeat AMI remain unclear., Methods and Results: Using the AMI-Kyoto Multi-Center Risk Study database, clinical background, angiographic findings, results of primary PCI, and in-hospital prognosis were retrospectively compared between primary PCI-treated AMI patients with previous myocardial infarction (MI) (repeat-MI patients, n=235) and those without previous MI (first-MI patients, n=1,550). The repeat-MI patients had higher prevalence of Killip class>or=3 at admission, larger number of diseased vessels, and a significantly higher in-hospital mortality rate than the first-MI patients. On multivariate analysis, number of diseased vessels>or=2 or diseased left main trunk (LMT) on initial coronary angiography was the independent positive predictor of in-hospital mortality in the repeat-MI patients, not in the first-MI patients, whereas acquisition of Thrombolysis In Myocardial Infarction 3 flow in the infarct-related artery immediately after primary PCI and elapsed time<24 h were the negative predictors in the first-MI patients, not in the repeat-MI patients., Conclusions: Number of diseased vessels>or=2 or diseased LMT on initial coronary angiography is an independent risk factor of in-hospital death in recurrent-AMI patients undergoing primary PCI.

Background: Several clinical studies have demonstrated an inverse relationship between hospital volume of primary percutaneous coronary interventions (PCI) and in-hospital mortality. However, the relationships among hospital primary PCI volume, angiographic results, and in-hospital prognosis in patients with acute myocardial infarction (AMI) have not been fully investigated in Japan., Methods and Results: Using the AMI-Kyoto Multi-Center Risk Study database between January 2000 and December 2005, hospitals were classified into quintiles based on their annual volume of primary PCI. The fifth quintile of hospitals was labeled as high-volume, and the other quintiles were combined and defined as low-volume. Although patients undergoing primary PCI in high-volume hospitals (high-volume group, n=764) had a larger number of diseased vessels at initial coronary angiography and lower Thrombolysis In Myocardial Infarction (TIMI) flow grade in the infarct-related artery before PCI, compared with those in low-volume hospitals (low-volume group, n=1,021), the rates of achieving TIMI flow grade 3 just after PCI in the high-volume group was significantly higher than that in the low-volume group. The overall in-hospital mortality did not differ between the 2 groups. On multivariate analysis, in AMI patients undergoing primary PCI, Killip class >or=3 at admission, multivessel disease or left main trunk (LMT) as culprit lesion, number of diseased vessels >or=2 or diseased LMT, and age were the independent positive predictors of in-hospital mortality, whereas the TIMI flow grade 3 after primary PCI and elapsed time <24 h were the negative ones, but not low-volume hospital., Conclusions: Angiographic results of primary PCI in high-volume hospitals were superior to those in low-volume hospitals, but there was no significant difference in the in-hospital mortality between AMI patients in high-volume hospitals and those in low-volume hospitals.

Background: Primary percutaneous coronary intervention (PCI) for acute myocardial infarction (AMI) is performed in hospitals without on-site coronary artery bypass graft surgery in the ;real world'. However, data on the in-hospital outcomes of primary PCI performed at hospitals with and without on-site cardiac surgery are still lacking in Japan., Methods and Results: In the present study, 2,230 AMI patients were enrolled in the AMI-Kyoto Multi-Center Risk Study between January 2000 and December 2005. Of these, 1,817 patients underwent primary PCI. Excluding patients without adequate data, we retrospectively compared clinical background, coronary risk factors, angiographic findings, acute results of primary PCI and in-hospital prognosis between patients undergoing primary PCI in hospitals without on-site cardiac surgery (without surgery group, n=792) and those in hospitals with (with surgery group, n=993). The without surgery group had higher prevalence of previous myocardial infarction, Killip class>or=3 at admission and multivessels as a culprit lesion than the with surgery group. The without surgery group was more likely to have lower frequency of stent usage and lower thrombolysis in myocardial infarction flow grade just after PCI, whereas it was more likely to have intra-aortic balloon pumping and temporary pacing during procedures. The overall in-hospital mortality did not differ between the 2 groups. On multivariate analysis, in AMI patients undergoing primary PCI, Killip class>or=3 at admission, multivessels or left main trunk (LMT) as culprit lesions, number of diseased vessels>or=2 or diseased LMT, and age were the independent predictors of the in-hospital mortality, but the presence of on-site cardiac surgery was not., Conclusions: These results suggest that in-hospital outcomes in AMI patients undergoing primary PCI at hospitals without on-site cardiac surgery are comparable to those at hospitals with on-site cardiac surgery in Japan.

Background: It remains uncertain whether obesity is an independent risk factor for coronary heart disease in young adults, as well as adults, in Japan., Methods and Results: In the present study, 1,260 cases of acute myocardial infarction (AMI) and 3,775 community controls were recruited from the AMI-Kyoto Multi-Center Risk Study and Kyoto Citizen's Health and Nutrition Study, respectively. Obesity and other risk factors were retrospectively examined between cases and controls in each subgroup of young males (20-40 years), middle-aged males or females (40-60 years), older males or females (60-80 years), and very old males or females (80-100 years). In young, middle-aged, and older males, as well as in older females, cases had a higher body mass index (BMI) than controls. In young males, as well as in middle-aged and older females, cases had a higher prevalence of smoking than controls. Except for very old males, the prevalences of hypercholesterolemia, hypertension, and diabetes mellitus were higher in each subgroup of cases than in controls. Multivariate logistic regression analysis revealed that obesity (BMI >or=25) was an independent risk for AMI in young and middle-aged males, but not in females, whereas smoking was an independent risk for AMI in middle-aged and older females as well as in older males., Conclusions: Obesity is significantly associated with AMI, independent of the classic coronary risk factors, in young and middle-aged males. These findings support the current emphasis on controlling obesity to prevent coronary events in young Japanese male adults.

Background: Data on the medium- and long-term prognosis of young Japanese patients with acute myocardial infarction (AMI) are still lacking., Methods and Results: In the present study, 1,458 AMI patients were enrolled in the AMI-Kyoto Multi-Center Risk Study between January 2000 and December 2003. Of these, clinical characteristics and medium-term prognosis were retrospectively compared in 21 young patients < 40 years (young group), and 190 non-young patients 60-70 years old (non-young group) who could be followed after hospital discharge. The young group was all male and had higher prevalence of current smoking and greater body mass index, but previous myocardial infarction (MI) and hypertension were more prevalent in the non-young group. The young group had a higher prevalence of single-vessel disease and a lesser incidence of left circumflex coronary artery as the culprit lesion. The acquisition rates of Thrombolysis In Myocardial Infarction 3 flow after primary percutaneous coronary intervention did not differ between the 2 groups, but the data of maximal creatine kinase was significantly higher in the young group. During the follow-up period (average 2.42 years for young, 2.37 years for non-young), survival and event-free survival rates and incidence of major adverse cardiac events (MACE) did not differ between the 2 groups. The predictor of MACE during follow-up period was the presence of multivessel disease in the young group, whereas the presence of multivessel disease, history of previous MI and longer hospitalization were the predictors of MACE in the non-young group., Conclusions: These results suggest that the medium-term prognosis in young AMI patients is comparable to that of non-young AMI patients in Japan.

Background: The prevalence and clinical manifestations of acute myocardial infarction (AMI) in young patients in Japan have not been fully investigated., Methods and Results: In the present study, 1651 AMI patients were enrolled in the AMI-Kyoto Multi-Center Risk Study between January 2000 and June 2004. Of these, the clinical background, risk factors, angiographic findings, acute results of primary percutaneous coronary intervention (PCI) and in-hospital outcomes for 27 young patients <40 (young group), and 338 non-young patients 60

A patient with idiopathic long QT syndrome had repeated syncopal episodes. The QTc interval on the electrocardiogram at rest was 530 ms and was prolonged by exercise up to 740 ms with T wave alternation. Intravenous epinephrine (0.1 microg/kg weight per min), but not isoproterenol (0.7 microg/min), produced early after depolarization of the monophasic action potential recorded at the right ventricular apex. Epinephrine prolonged the QTc interval to 710 ms. After the addition of propranolol to the epinephrine, the QTc (580 ms) was longer than at baseline. Methoxamine also prolonged the QTc to 580 ms. The QT interval in long QT syndrome is generally considered to be prolonged by a beta-adrenergic effect, but in the present case alpha-adrenergic stimulation had an additional effect on the prolongation of the QT interval.

Subepicardial aneurysm is a rare complication of acute myocardial infarction and the present case was associated with ventricular septal perforation. Echocardiography showed the subepicardial aneurysm adjoining the true apico-anteroseptal aneurysm, with the former being discontinuous with the myocardium at its neck, which was narrower than the diameter of the aneurysm. In addition, color Doppler imaging showed shunt flow from the aneurysm to the right ventricle. Coronary angiography revealed extension only of the anterior descending artery without any discernible stenosis. The apical aneurysm was excised and the defect closed with an epicardial patch. The myocardial infarction was probably caused by coronary spasm. Echocardiography was useful for diagnosing the anatomy and hemodynamic condition of the subepicardial aneurysm.

Tachycardia-induced cardiomyopathy is a well-known and reversible condition, but the left ventricular dysfunction caused by frequent isolated premature ventricular complexes (PVCs) has been rarely reported. Apparent dilated cardiomyopathy was resolved in a patient after the focal source of PVCs was eliminated by radiofrequency catheter ablation. Echocardiography showed progressive improvement of the abnormal wall motion. Frequent PVCs could be the cause of left ventricular dysfunction in a subset of patients with dilated cardiomyopathy and radiofrequency ablation should be the choice of therapy in those patients.

Verapamil is widely used for the termination of paroxysmal supraventricular tachycardia (PSVT) with little proarrhythmic effect. We describe two cases of PSVT that changed to non-sustained polymorphic ventricular tachycardia after administration of verapamil. Electrophysiological study revealed atrioventricular nodal reentrant tachycardia in the first case, and atrioventricular reentrant tachycardia due to a concealed left lateral accessory pathway in the second case. Catecholamine-induced automaticity was one of the possible mechanisms of VT in the first case, but the mechanism is unknown in the second case.

To analyze the recent change in the long-term survival of patients with dilated cardiomyopathy (DCM), the present study comprised consecutive 111 patients with ejection fraction <50% and left ventricular end-diastolic diameter >58 mm. who were admitted to hospital from January 1983 to December 1994. The patients were divided into 2 groups: group A who were diagnosed before 1989 and group B diagnosed after 1990. Basic characteristics at diagnosis, including age, NYHA functional class, left ventricular end-diastolic diameter and ejection fraction, were similar between these 2 groups. Calculated survival rate at 5 years was 90.0% in group B in contrast to 62.3% in group A. Event-free survival also improved in group B. In group B, beta-blockers and angiotensin converting enzyme inhibitors were more frequently used than in group A (p<0.0001) whereas digitalis and other positive inotropic agents were significantly less used. Left ventricular ejection fraction was significantly improved during the follow-up period in patients treated with beta-blockers compared with those not treated with beta-blockers. These data indicate a significant improvement in the survival of patients with dilated cardiomyopathy after 1990, which may be explained by the change of medical treatment, especially the use of beta-blockers.

Acute necrotizing eosinophilic myocarditis is characterized by acute onset, fulminant congestive heart failure, and extensive necrosis of myocytes with striking eosinophilic infiltration. However, multinucleated giant cells sometimes appear in the fulminant phase of severe myocarditis. This is the first case of a patient with a 1 year previous history of idiopathic thrombocytopenic purpura, who presented with acute necrotizing eosinophilic myocarditis with giant cell infiltration.