小林 欣夫

Skin abnormalities are often indicative of cardiovascular diseases. Such a disease entity is called cardiocutaneous syndrome; however, the details regarding the involvement of bulla and nails remain largely unclear. A 49-year-old man with systemic bulla was admitted for heart failure. His bulla had previously been diagnosed as epidermolysis bullosa, but no known gene mutations for it had been identified. He had a triad of palmoplantar keratosis, curly and fine hair, and cardiomyopathy, which are characteristic of NAXOS-Carvajal syndrome. This case highlights the fact that bulla and brittle nails can accompany NAXOS-Carvajal syndrome, showing that these extra-cardiac findings can help identify otherwise overlooked serious cardiac conditions.

Near-infrared spectroscopy-intravascular ultrasound (NIRS-IVUS) studies have demonstrated that lipid core plaque (LCP) is frequently observed in the culprit segment of myocardial infarction (MI). However, little is known about the impact of clinical presentations such as chronic coronary syndrome (CCS) and acute coronary syndrome (ACS) including unstable angina (UA), non ST-segment elevation MI (NSTEMI), and ST-segment elevation MI (STEMI) on LCP. The present prospective single-center registry included a total of 178 patients who underwent percutaneous coronary intervention under NIRS-IVUS guidance. Patients were divided into CCS and ACS groups, and ACS patients were further sub-divided into the 3 groups according to the clinical presentation. The primary endpoint was coronary LCP in the target lesion assessed by NIRS-IVUS with maximal lipid core burden index over any 4 mm segment (maxLCBI4mm). The study population included 124 and 54 patients with CCS and ACS. MaxLCBI4mm in the target lesion was significantly higher in the ACS group than in the CCS group (503 [284-672] vs. 406 [250-557], p = 0.046). Among ACS patients, MaxLCBI4mm in the target lesion was also significantly different in those with UA (n = 18), NSTEMI (n = 21), and STEMI (n = 15) (288 [162-524] vs. 518 [358-745] vs. 646 [394-848], p = 0.021). In conclusion, LCP assessed by NIRS-IVUS, a surrogate of coronary plaque vulnerability, was significantly different according to the clinical presentations such as CCS, UA, NSTEMI, and STEMI.

Percutaneous coronary intervention (PCI) for complex lesions is still technically demanding and is associated with less favorable procedural parameters such as lower success rate, longer procedural time, higher contrast volume and unexpected complications. Because the conventional angiographic analysis is limited by the inability to visualize the plaque information and the occluded segment, cardiac computed tomography has evolved as an adjunct to invasive angiography to better characterize coronary lesions to improve success rates of PCI. Adding to routine image reconstructions by coronary computed tomography angiography, the thin-slab maximum intensity projection method, which is a handy reconstruction technique on an ordinary workstation, could provide easy-to-understand images to reveal the anatomical characteristics and the lumen and plaque information simultaneously, and then assist to build an in-depth strategy for PCI. Especially in the treatment of chronic total occlusion lesion, these informations have big advantages in the visualization of the morphologies of entry and exit, the occluded segment and the distribution of calcium compared to invasive coronary angiography. Despite of the additional radiation exposure, contrast use and cost for cardiac computed tomography, the precise analysis of lesion characteristics would consequently improve the procedural success and prevent the complication in complex PCI.

Over the past 40 years, the safety and efficacy of percutaneous coronary intervention has dramatically improved by overcoming several challenges. The introduction of drug-eluting stent (DES) in particular was a major breakthrough in interventional cardiology. Compared to bare-metal stents, first-generation DES (G1-DES) has dramatically reduced the rates of in-stent restenosis and subsequent target lesion revascularization. However, major safety concerns surrounding stent thrombosis (ST) emerged with G1-DES in clinical practice as a result of the high incidences of death, myocardial infarction, and repeat revascularization associated with ST. To overcome these limitations, second-generation DES (G2-DES) has been developed with an improved stent platform with thinner strut and biocompatible durable or biodegradable polymers. Indeed, G2-DES, when compared with G1-DES, has improved clinical outcomes by reducing the risk of late thrombotic events while maintaining anti-restenotic efficacy, whereas ST still occurs, even with the use of G2-DES. This review gives an overview of pathophysiology, risk factors, and outcomes of ST after DES implantation. Additionally, we discuss the management and prevention of ST.

The ISCHEMIA was eagerly awaited study in the field of ischemic heart disease. Following the presentation and publication of ISCHEMIA, multiple opinions and viewpoints get complicated. The ongoing debates have been including the relevance of coronary revascularization, non-invasive diagnostic methods, and invasive ischemic testing in patients with stable ischemic heart disease (SIHD). Prior to ISCHEMIA, observational studies indicated the potential of coronary revascularization for improving clinical outcomes, while the randomized COURAGE trial did not support the plausible concept. Although the FAME 2 trial implied the superiority of percutaneous coronary intervention over medical therapy alone, the clinical relevance of coronary revascularization to improve outcomes and quality of life has been questioned. As a consequence, the ISCHEMIA trial did not demonstrate clear benefits in reducing clinical events but showed antianginal effects of revascularization. This landmark trial also suggested the difficulties of non-invasive ischemia testing rather than computed tomography angiography. Despite the complex results, the ISCHEMIA trial may simplify the clinical indications of coronary revascularization in patients with SIHD. Future publications from the ISCHEMIA trial and debates on the results will sharpen our thinking and understanding.

BACKGROUND: High platelet reactivity (HPR) is associated with subsequent thrombotic events in patients undergoing percutaneous coronary intervention (PCI). Recently, the ABCD-GENE score was developed to identify patients at risk for HPR, incorporating both clinical and genetic factors. However, this score was derived and validated in mostly Caucasian subjects and it has not been validated in an East Asian population. METHOD: Individual patient data from 4 prospective studies were pooled, in which platelet reactivity was measured using the VerifyNow assay on clopidogrel and genotyping of CYP2C19 was performed after PCI. Study populations included patients with general stable coronary artery disease, hemodialysis, age ≥75 and/or body weight <50 kg, and acute coronary syndrome. VerifyNow P2Y12 reactivity units >208 was defined as HPR. RESULTS: Of 184 patients, 111 (60%) had HPR on clopidogrel. In the receiver operating characteristics curve analyses, the ABCD-GENE score significantly predicted HPR on clopidogrel (AUC 0.78, best cut-off value 9, p < 0.001). Across the 4 studies and their combinations, the diagnostic ability and cut-off values of ABCD-GENE score for HPR on clopidogrel were consistent. CONCLUSIONS: The ABCD-GENE score had significant and moderate diagnostic ability for HPR on clopidogrel in Japanese patients undergoing PCI. The predictivity was consistent across a broad spectrum of patient populations, suggesting the applicability of this novel scoring system in clinical practice worldwide.

【目的】訪問看護における心不全管理に関する現状を学習方法とニーズの観点から明らかにすることである。【方法】千葉県内にある訪問看護ステーションへ郵送でアンケート用紙を送付した。【結果】最終的に返送のあった80通(回収率37%)を分析した。多くの訪問看護師が自己学習と外部研修に参加をして心不全管理の学習をしていたが、心不全の基礎的な知識に加え、地域連携や事例の共有、活動量や食事の具体的な目安と指標のような研修のニーズ、在宅生活を見える化するツールの使用などのニーズが挙げられた。【結論】多くの訪問看護師が外部の研修に参加しているものの、ニーズを十分に満たす研修が開催されていない可能性がある。訪問看護における心不全管理の質向上に向けて、多忙な職場環境においても参加しやすい形式の検討および迅速に現場に活用できるニーズに則した研修内容の検討の必要性が示唆された。(著者抄録)

Acute kidney injury usually assessed within 48 h after percutaneous coronary intervention (PCI) is associated with poor clinical outcomes, and persistent kidney damage is also strongly related to long-term mortality. However, little is known about longitudinal renal function change from a very early period to long-term follow-up after PCI. A total of 327 patients with stable coronary artery disease underwent elective PCI. Renal function was assessed with serum creatinine levels and estimated glomerular filtration rate (eGFR) at baseline, 1 day after PCI, at 1 year and at the latest follow-up. Kidney injury was defined as an increase in creatinine levels ≥ 0.3 mg/dl or ≥ 50% from baseline at each timepoint. Major adverse cardiovascular events (MACE) was defined as a composite of death, myocardial infarction, and stroke. eGFR was significantly increased 1 day after PCI, while it was progressively decreased at 1-year and long-term follow-up (median 28 months). Overall, eGFR was declined by - 2.3 ml/min/1.73 m2 per year. Only one (0.3%) patient developed kidney injury 1 day after PCI, whereas kidney injury at 1-year and long-term follow-up was observed in 15 (4.6%) and 27 (8.3%). During the follow-up period, 23 (7.0%) patients had MACE. The incidence of subsequent MACE was significantly higher in patients with kidney injury at 1 year than those without. In conclusion, kidney injury within 24 h after elective PCI was rarely observed. eGFR was progressively decreased over time, and mid-term kidney injury at 1 year was associated with future MACE.

BACKGROUND: Vasospastic angina (VSA) reportedly accounts for one form of sudden cardiac arrest (SCA). Intracoronary acetylcholine (ACh) testing is useful for diagnosing VSA although invasive provocation testing after SCA is a clinical challenge. In addition, even if the ACh test is positive, any causal relationship between VSA and SCA is often unclear because patients with VSA may have other underlying cardiac disorders. METHODS: A total of 20 patients without overt structural heart disease who had been fully resuscitated from SCA were included. All patients underwent the ACh provocation test and scrutiny such as cardiac computed tomography or magnetic resonance imaging. Patients were followed up for all-cause death or recurrent SCA including appropriate implantable cardioverter defibrillator therapy. RESULTS: An ACh provocation test was performed 20 ± 17 days after cardiac arrest. Fifteen out of 20 (75.0%) patients had a positive ACh test and 2 (10.0%) had adverse events such as ventricular tachycardia and transient cardiogenic shock during the test. In patients with a positive ACh test, 6 of 15 (40.0%) patients had other overlapping cardiac disorders such as long QT syndrome, Brugada syndrome, cardiac sarcoidosis, myocarditis, or cardiomyopathy. Long-term prognosis was not different regardless of a positive ACh test or the presence of other cardiac disorders overlapping with VSA. CONCLUSIONS: Three-quarters of the patients who had been resuscitated from SCA had a positive ACh test. Further examinations revealed other overlapping cardiac disorders in addition to VSA in 40% of patients with a positive ACh test.

BACKGROUND: Leadless pacemakers are an effective treatment for bradycardia. However, some cases exhibit pericardial effusions, presumably associated with device implantations on the right ventricular free-wall. The present study was carried out to find the ECG features during ventricular pacing with a Micra, which enabled distinguishing free-wall implantations from septal implantations without using imaging modalities. METHODS: Thirty-one consecutive patients who received Micra implantations in our facility were enrolled. The location of the device in the right ventricle was evaluated using echocardiography or computed tomography in order to determine whether the device was implanted on the septum (Sep group), apex (Apex group), or free-wall (FW group). The differences in the 12-lead ECG during ventricular pacing by the Micra were analyzed between the Sep and FW groups. RESULTS: The body of the Micra was clearly identifiable in 22 patients. The location of the device was classified into Sep in 12 patients, Apex in 4, and FW in 6. The mean age was highest in the FW and lowest in the Sep group (82.7 ± 6.6 vs. 72.8 ± 8.7 years, p = 0.027). The peak deflection index (PDI) was significantly larger in the FW group than Sep/Apex group in lead V1 (Sep: 0.505 ± 0.010, Apex: 0.402 ± 0.052, FW: 0.617 ± 0.043, p = 0.004) and lead V2 (Sep: 0.450 ± 0.066, Apex: 0.409 ± 0.037, FW: 0.521 ± 0.030, p = 0.011), whereas there was no difference in the QRS duration, transitional zone, and QRS notching. CONCLUSION: The PDI in V1 could be useful for predicting implantations of Micra devices on the free-wall and may potentially stratify the risk of postprocedural pericardial effusions.

Esaxerenone, a mineralocorticoid receptor blocker (MRB), is a new antihypertensive agent. However, esaxerenone-related data with respect to hypertension with heart failure are limited. We investigated the safety and efficacy of esaxerenone in hypertensive patients with heart failure with reduced ejection fraction (HFrEF). Hypertensive patients with HFrEF treated with esaxerenone were retrospectively analyzed at two timepoints (short-term: 35±15 days; mid-term: 167±45 days). Adverse events including hyperkalemia (K+ >5.5 mEq/L), worsening renal function (WRF; estimated glomerular filtration rate (eGFR) reduction by ≥20%), and hypotension (systolic blood pressure <90 mmHg) were evaluated. eGFR and K+, serum creatinine, and brain natriuretic peptide (BNP) levels at baseline, short-term, and mid-term assessments were compared. Patients administered esaxerenone as their first MRB (first-MRB cohort) and those who converted from another MRB (conversion cohort) were separately analyzed. There were 50 (56±10 years old, 26% female) patients. At the short-term assessment, hyperkalemia or hypotension was not observed at a dose of 2.0±0.9 mg/day. Seven patients (14%) showed WRF. K+ was slightly elevated (4.12±0.41 to 4.25±0.39 mmol/L, p = 0.07) and eGFR was significantly reduced (66.9±19.6 mL/min/1.73 m2 to 62.4±19.7 mL/min/1.73 m2, p = 0.006). In the conversion cohort, significant changes in K+ and eGFR from baseline were not observed at the short-term assessment. BNP levels were consistently improved regardless of the cohorts (first-MRB cohort, 310 [110-370] pg/mL to 137 [47-152] pg/mL, p = 0.001; conversion cohort, 181 [30-203] pg/mL to 108 [26-146] pg/mL, p = 0.028). At the mid-term assessment, there were no significant changes in K+ and eGFR compared with the short-term assessment. In conclusion, esaxerenone was safe for hypertensive patients with HFrEF. Hyperkalemia and hypotension were rarely noted, while eGFR was marginally reduced. Moreover, esaxerenone might be beneficial for HFrEF in terms of BNP level reduction.

Rotational atherectomy (RA) has been widely used for percutaneous coronary intervention (PCI) to severely calcified lesions. As compared to other countries, RA in Japan has uniquely developed with the aid of greater usage of intravascular imaging devices such as intravascular ultrasound (IVUS) or optical coherence tomography (OCT). IVUS has been used to understand the guidewire bias and to decide appropriate burr sizes during RA, whereas OCT can also provide the thickness of calcification. Owing to such abundant experiences, Japanese RA operators modified RA techniques and reported unique evidences regarding RA. The Task Force on Rotational Atherectomy of the J apanese Association of Cardiovascular Intervention and Therapeutics (CVIT) has now proposed the expert consensus document to summarize the contemporary techniques and evidences regarding RA.

Percutaneous coronary intervention has become a standard-of-care procedure in patients with acute and chronic coronary syndromes, in which coronary stent technology is commonly used. In this mini-review article, we summarize the characteristics of contemporary coronary drug-eluting and coated stents.

The management of acute decompensated heart failure often requires intensive care. However, the effects of early intensive care unit/coronary care unit admission on activities of daily living (ADL) in acute decompensated heart failure patients have not been precisely evaluated. Thus, we retrospectively assessed the association between early intensive care unit admission and post-discharge ADL performance in these patients. Acute decompensated heart failure patients (New York Heart Association I-III) admitted on emergency between April 1, 2014, and December 31, 2018, were selected from the Diagnosis Procedure Combination database and divided into intensive care unit/coronary care unit (ICU) and general ward (GW) groups according to the hospitalization type on admission day 1. The propensity score was calculated to create matched cohorts where admission style (intensive care unit/coronary care unit admission) was independent of measured baseline confounding factors, including ADL at admission. The primary outcome was ADL performance level at discharge (post-ADL) defined according to the Barthel index. Secondary outcomes included length of stay and total hospitalization cost (expense). Overall, 12231 patients were eligible, and propensity score matching created 2985 pairs. After matching, post-ADL was significantly higher in the ICU group than in the GW group [mean (standard deviation), GW vs. ICU: 71.5 (35.3) vs. 78.2 (31.2) points, P<0.001; mean difference: 6.7 (95% confidence interval, 5.1-8.4) points]. After matching, length of stay was significantly shorter and expenses were significantly higher in the ICU group than in the GW group. Stratified analysis showed that the patients with low ADL at admission (Barthel index score <60) were the most benefited from early intensive care unit/coronary care unit admission. Thus, early intensive care unit/coronary care unit admission was associated with improved post-ADL in patients with emergency acute decompensated heart failure admission.

Previous studies have shown that the sudden cardiac death (SCD) prediction model proposed by the 2014 European Society of Cardiology (ESC) guideline (5-Year Risk-SCD) was validated in European patients with hypertrophic cardiomyopathy (HCM). However, there are limited data on Asian patients with HCM. We assessed the validity of the estimated 5-Year Risk-SCD in Japanese HCM patients with an implantable cardioverter-defibrillator (ICD) using the2014 ESC guidelines. We retrospectively examined data of 492 consecutive Japanese patients with an ICD. Sixty-two Japanese HCM patients with an ICD were enrolled in this study, and 50 patients (81%) were followed up for ≥ 5 years. We analyzed the characteristics and outcomes of these 50 patients. We investigated the incidence of appropriate ICD therapy as categorized by the ESC guideline and compared the 5-Year Risk-SCD with the 5-year rate of appropriate shock therapies. Based on the 2012 Japanese Circulation Society guideline and the 2011guidelines of the American Heart Association and American College of Cardiology Foundation, 10 and 40 patients met classes I and IIa of the ICD recommendation, respectively. However, only 18 (36%) patients were classified into class I or IIa of the ESC guideline. Among 50 patients followed up for ≥ 5 years after ICD implantation, the incidences of appropriate ICD therapies for classes I, IIa, IIb, and III indications based on the 2014 ESC guideline were 50%, 38%, 17%, and 0%, respectively. Risk stratification for SCD using 5-Year Risk-SCD is valid in Japanese HCM patients with an ICD, and the 2014 ESC guideline might be useful for the indication of ICD implantation in Japan.

Coronary artery fistulas are abnormal vascular conduits, rarely related to atrioventricular conduction abnormalities. We report the case of a 52-year-old woman who presented with dyspnoea on exertion. Her ECG revealed advanced atrioventricular block and left bundle branch block. CT scans confirmed two fistulas, from the conus branch of right coronary artery and from the left anterior descending coronary artery, into the pulmonary artery. The patient underwent pacemaker implantation. To date, only nine patients with different degrees of heart blocks associated with coronary artery fistulas have been reported. Herein, we review and summarise previously reported cases of different degrees of heart blocks associated with coronary artery fistulas.

背景:肥大型心筋症(HCM)患者では心臓突然死のリスクが高く、植込み型除細動器(ICD)による治療が広く行われている。心尖部肥大型心筋症(Apical HCM)の予後を検討した報告は少ない。目的:Apical HCMとその他のHCM患者のICD植え込み症例において、予後に差があるかを調べることである。方法:当院のデータベースより2006年10月から2016年7月までにICD植え込み術を施行しているHCM症例を抽出した。Apical HCMとその他のHCM患者に分けて、患者背景、採血データ、診断時の左室駆出率、最大左室壁厚、ICDの適切作動率、心疾患による入院の有無、死亡等について解析を行い、比較した。結果:2006年10月〜2016年7月にICD植え込み術を施行したHCM患者は53例(平均年齢67±15歳、男性45例(85%)、一次予防46例(87%)、フォローアップ期間85±25ヵ月、診断時左室駆出率57±12%)であり、Apical HCMは13例、その他のHCMは40例であった。2群間で年齢や性別、左室駆出率など患者背景において、有意差は認めなかった。イベントについては、Apical HCM患者はICD適切作動を13症例中5例(38%)認め、40症例中4例(10%)であったその他のHCM患者より、ICD適切作動が有意に多かった。また、2群間で心不全入院や死亡率に有意差は認めなかった。結語:ICD植え込み術後のHCM症例において、Apical HCMは、その他のHCM症例と比較し、ICD適切作動が有意に多かった。しかし、心不全による入院や死亡については、Apical HCMとその他のHCMでは有意差を認めなかった。(著者抄録)

OBJECTIVES: We investigated the medical or mechanical therapy, and the present knowledge of Japanese cardiologists about aborted sudden cardiac death (ASCD) due to coronary spasm. METHODS: A questionnaire was developed regarding the number of cases of ASCD, implantable cardioverter-defibrillator (ICD), and medical therapy in ASCD patients due to coronary spasm. The questionnaire was sent to the Japanese general institutions at random in 204 cardiology hospitals. RESULTS: The completed surveys were returned from 34 hospitals, giving a response rate of 16.7%. All SCD during the 5 years was observed in 5726 patients. SCD possibly due to coronary spasm was found in 808 patients (14.0%) and ASCD due to coronary spasm was observed in 169 patients (20.9%). In 169 patients with ASCD due to coronary spasm, one or two coronary vasodilators was administered in two-thirds of patients [113 patients (66.9%)], while more than 3 coronary vasodilators were found in 56 patients (33.1%). ICD was implanted in 117 patients with ASCD due to coronary spasm among these periods including 35 cases with subcutaneous ICD. Majority of cause of ASCD was ventricular fibrillation, whereas pulseless electrical activity was observed in 18 patients and complete atrioventricular block was recognized in 7 patients. Mean coronary vasodilator number in ASCD patients with ICD was significantly lower than that in those without ICD (2.1 ± 0.9 vs. 2.6 ± 1.0, p < 0.001). Although 16 institutions thought that the spasm provocation tests under the medications had some clinical usefulness of suppressing the next fatal arrhythmias, spasm provocation tests under the medication were performed in just 4 institutions. CONCLUSIONS: In the real world, there was no fundamental strategy for patients with ASCD due to coronary spasm. Each institution has each strategy for these patients. Cardiologists should have the same strategy and the same knowledge about ASCD patients due to coronary spasm in the future.

We treated a man with co-incident Marfan-like connective tissue disease with morphologic left ventricular non-compaction (LVNC). He underwent valve-sparing aortic root replacement because of aortic root dilation at 43 years old. Pathological findings of the aorta revealed cystic medio-necrosis, consistent with Marfan syndrome. He developed congestive heart failure caused by LVNC at 47 years old. His daughter had scoliosis, and he had several physical characteristics suggestive of Marfan syndrome. We herein report a rare case of a patient who had Marfan-like connective disease with an LVNC appearance.

Some cancers are related to atherosclerotic diseases; therefore, these two types of disease may share some antibody biomarkers in common. To investigate this, a first screening of sera was performed from patients with esophageal squamous cell carcinoma (ESCC) or acute ischemic stroke (AIS) for serological identification of antigens using recombinant cDNA expression cloning (SEREX). The amplified luminescent proximity homogeneous assay-linked immunosorbent assay (AlphaLISA) method, which incorporates glutathione donor beads and anti-human IgG acceptor beads, was used to evaluate serum antibody levels. SEREX screening identified low-density lipoprotein receptor-related protein-associated protein 1 (LRPAP1) as a target antigen of serum IgG antibodies in the sera of patients with ESCC or AIS. Antigens, including recombinant glutathione S-transferase-fused LRPAP1 protein, were prepared to examine serum antibody levels. AlphaLISA revealed significantly higher antibody levels against the LRPAP1 protein in patients with solid cancers such as ESCC and colorectal carcinoma and some atherosclerosis-related diseases such as AIS and diabetes mellitus compared with healthy donors. Correlation analysis revealed that the elevated serum antibody levels against LRPAP1 were associated with smoking, a well-known risk factor for both cancer and atherosclerosis. Serum LRPAP1 antibody is therefore a common marker for the early diagnosis of some cancers and atherosclerotic diseases and may reflect diseases caused by habitual smoking.