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Background: Fulminant myocarditis presentation (FMP) is a rare and severe presentation of myocarditis. The natural history of FMP and its clinical features associated with poor outcomes are incompletely understood because there is a lack of generalizable evidence. Methods: This multicenter retrospective cohort study included patients hospitalized with histologically proven myocarditis who underwent catecholamine or mechanical support from 235 cardiovascular training hospitals across Japan between April 2012 and March 2017. Clinical features and the prognostic predictors of death or heart transplantation within 90 days on the basis of clinical and pathologic findings were determined using the Kaplan-Meier method, log-rank test, and Cox regression analysis. Results: This study included 344 patients with histologically proven FMP (median age, 54 years; 40% female). The median follow-up was 600 days (interquartile range, 36 to 1599 days) and the cumulative risk of death or heart transplantation at 90 days was 29% (n=98). Results from multivariable Cox regression analysis showed that older age, nonsinus rhythm, low left ventricular wall motion ( Conclusions: The results from analyses of data from this multicenter registry demonstrated that patients with FMP are at a higher risk of death or heart transplantation in real-world settings. These observations inform which clinical and pathologic findings may be useful for prognostication in FMP. Registration: URL: https://www.umin.ac.jp/ctr ; Unique identifier: UMIN000039763.

Surgical valve replacement improves the symptoms and prognosis of patients with valvular heart diseases. Aortic regurgitation elicits volume overload that causes enlargement of the left ventricle (LV), while the LV size often shrinks to near normal after aortic valve replacement (AVR), which is referred to as "reverse remodeling". We experienced a case in which LV outflow tract (LVOT) obstruction became apparent after AVR, resulting in worsening of heart failure. A 65-year-old man who had undergone surgical AVR for aortic valve regurgitation 15 months previously exhibited dyspnea on effort accompanied with severe LVOT obstruction. With double pressure catheters, we directly recorded an augmented pressure gradient in the LVOT and rapid relief of the obstruction by intravenous administration of the anti-arrhythmic drug cibenzoline. Since the considerable LV hypertrophy had been indicated by an electrocardiogram and echocardiography before AVR, we suspected that dilation of the LV chamber due to aortic valve regurgitation could have masked the subclinical LVOT obstruction, which became clinically evident after LV size reduction due to reverse remodeling after AVR.

A pulmonary artery (PA) aneurysm is an extremely rare condition that can be idiopathic or secondary. Only a few reports on giant PA aneurysms associated with chronic thromboembolic pulmonary hypertension (CTEPH) are available in the literature. Here, we present a case of CTEPH associated with a secondary giant PA aneurysm detected by autopsy. A 68-year-old woman was diagnosed with pulmonary hypertension (PH) and a PA aneurysm with a diameter of 7.5 cm 7 years before admission. CTEPH was suspected as the cause of PH; however, she refused to undergo surgical treatment. Although her condition improved temporarily with pulmonary vasodilators, she had recurrence of heart failure and died because of the deterioration of her general condition. An autopsy revealed a giant PA aneurysm without medial degeneration, suggesting a secondary PA aneurysm associated with PH. Histological findings indicated multiple organized thrombi with recanalization in the PA bilaterally, and CTEPH was diagnosed as the cause of PH. Although rare, when a PA aneurysm is detected, it is important to consider that CTEPH might be associated with a giant PA aneurysm. A better understanding of this condition is necessary to improve the therapeutic strategy.

The "on-therapy range" of direct oral anticoagulants is the 90% interval of drug concentration. Previously, we reported the on-therapy range of rivaroxaban in a single-center cohort. The present study aimed to confirm the range and intraindividual reproducibility in a multicenter cohort.Eligible patients with non-valvular atrial fibrillation under rivaroxaban treatment for prevention of ischemic stroke were enrolled from nine institutes in Tokyo, Japan, between June 2016 and May 2017 (n = 324). The first and second (three months later) blood samples both taken within 1-5 hours after rivaroxaban intake were analyzed (n = 219). Plasma concentration of rivaroxaban (PC-Riv) and prothrombin time (PT) with five reagents were measured.The 90% interval of PC-Riv was 47.3-532.9 ng/mL. The 90% interval of PT measured with RecombiPlasTin 2G was 11.8-22.3 seconds, the widest range among the five reagents examined. PC-Riv reproducibility within a 90% interval was evaluated bidirectionally (first-to-second and second-to-first), and 92.4% of samples were reproducible. The change rate (CR) of PC-Riv between two samplings ranged widely, and high CR (≥54.3%, cutoff for predicting non-reproducibility) was predicted by concomitant drugs (non-dihydropyridine calcium antagonist and thiazide) and mitral regurgitation.We reported the on-therapy range of rivaroxaban in a multicenter cohort. This range was consistent with that of a single-center cohort and was highly reproducible within three months in daily clinical practice. However, caution is necessary regarding several factors that may affect the intraindividual variation of PC-Riv.

A 69-year-old woman without any past disease history was hospitalized for heart failure. After hospitalization, she showed myocardial infarction, atrioventricular dissociation, and cardiac dysfunction, and finally she passed away despite intensive care. Autopsy revealed that the cardiac abnormalities were due to bacterial myocarditis possibly resulting from urinary tract infection by E. coli. Although bacterial myocarditis is rare in developed countries, we should consider its possibility when patients show various cardiac abnormalities with bacterial infection.

A 63-year-old woman who had hypopituitarism was re-admitted to our hospital because of fever, diarrhea and disturbance of consciousness with life-threatening arrhythmia due to prolongation of the QT interval. She has been treated with hydrocortisone consequently, and has shown few ventricular arrhythmias with normalization of the QT interval. There have been several reports showing the case of prolonged QT interval with adrenal insufficiency, but there are few reports of isolated adrenocorticotropic hormone deficiency without any electrolytes imbalance that showed polymorphic ventricular tachycardia associated with QT prolongation. We discuss some possible mechanisms of how adrenal insufficiency causes life-threatening arrhythmia. Since lack of glucocorticoid hormone might induce prolongation of the QT interval, patients with adrenal insufficiency should be paid attention as candidates of lethal arrhythmias particularly when exposed to excessive stresses.

Background The Cornell product (CP) improved identification of left ventricular hypertrophy (LVH) in the LIFE study, although its clinical significance is still unknown in Japanese hypertensive (HT) patients. Methods and Results A standard 12-lead ECG was recorded in 265 HT and 363 normotensive cases (N). All ECGs were digitized, and a simple product was calculated by multiplying the Cornell voltage (CV) by the QRS duration. In 147 of the 265 HT cases, the standard 12-lead ECG and transthoracic Doppler echocardiography were examined in the same period. The mean value of CP increased in the following order: 1,426±673 mm · ms in N, 1,989±900 mm · ms in HT with treatment, 2,137±976 mm · ms in HT without treatment. The correlation with left ventricular mass index (LVMI) measured by echocardiography was improved by CP with the simple CV. With use of a partition of 2,440 mm · ms in CP, LVMI and relative wall thickness were significantly higher in HT with ≥2,440 mm · ms of CP compared with

Background Fulminant type 1 diabetes is a non-autoimmune disorder characterized by sudden onset. This complication is rarely associated with myocarditis, suggesting an involvement of viral infection. We report a patient with myocarditis who was admitted for fulminant type 1 diabetes and diagnosed using a combination of non-invasive techniques. Case presentation We describe the case of a 25-year-old Japanese man with fulminant type 1 diabetes complicated by myocarditis. The patient was admitted with flu-like symptoms and diabetic ketoacidosis, followed by chest pain the next day. Myocardial damage was suspected based on ST-segment elevation on electrocardiogram and elevation of cardiac enzymes. However, coronary angiography revealed no abnormality in the coronary arteries. We diagnosed myocarditis by a combination of echocardiography, cardiovascular magnetic resonance imaging (CMR), as well as Thallium-201 and Iodine-123 beta-methyl iodophenyl pentadecanoic acid (Tl-201 BMIPP and I-123 BMIPP) and myocardial imaging. More importantly, CMR revealed diffuse enhancement in the subepicardium of the left ventricle with late gadolinium enhancement, consistent with myocardial edema. The patient was successfully treated, received a two-week education program on diabetes and discharged without complication. Conclusions The rapid onset and flu-like symptoms strongly suggest the involvement of viral infection in the pathogenesis of fulminant type 1 diabetes and myocarditis. While cardiac muscle biopsy is routinely performed, this case demonstrates that a combination of non-invasive techniques, especially CMR, may successfully diagnose myocarditis in patients with fulminant type 1 diabetes.

Radiofrequency catheter ablation of the slow pathway is commonly used to treat atrioventricular (AV) nodal reentrant tachycardia. However, there has been little study of the follow-up assessment of AV nodal physiology. We compared AV nodal electrophysiological characteristics before, immediately after, and again 3 months after successful catheter ablation in 17 patients (mean age 50 +/- 16 years). Sinus cycle length, Wenckebach cycle length, A-H interval at a paced cycle length of 600 ms, effective refractory period and functional refractory period of the fast pathway were significantly changed immediately after catheter ablation, but had recovered 3 months after the procedure. There were no significant differences between the electrophysiological parameters immediately after catheter ablation and those 3 months after the procedure under the intravenous injection of atropine sulfate. We conclude that, due to changes in autonomic nervous tone, AV nodal electrophysiological characteristics are influenced immediately after catheter ablation of the slow pathway in AV nodal reentrant tachycardia.

Although the effective refractory period (ERP) of the fast pathway is reported to be shortened after radiofrequency catheter ablation of the slow pathway in atrioventricular nodal reentrant tachycardia (AVNRT), whether the atrioventricular (AV) nodal physiology maintains the same condition over the long term is controversial. To assess the AV nodal physiology after catheter ablation of the slow pathway in AVNRT, the A-H interval and ERP of the fast pathway were measured before, immediately after and 3 months after catheter ablation in 12 patients. We compared these factors in 12 patients with left concealed accessory bypass tract to clarify the mechanism of the electrophysiological change in the AV node. Both the A-H interval and ERP of the fast pathway were significantly shortened from 111 +/- 18 to 96 +/- 17 ms and 365 +/- 68 to 261 +/- 52 ms immediately after catheter ablation (p0.01), and recovered to 128 +/- 44 and 372 +/- 80 ms 3 months after catheter ablation. There were no significant differences in the A-H interval of the fast pathway between the period immediately after and 3 months after catheter ablation following administration of atropine sulfate (99 +/- 52). There were no significant differences in the ERP of the fast pathway between the period immediately after and 3 months after catheter ablation following administration of atropine sulfate (248 +/- 33 ms). On the contrary, there were no significant changes in the A-H interval and ERP of the AV node at these two times in patients with Wolff-Parkinson-White (WPW) syndrome. The change in characteristics of AV nodal physiology was observed only immediately after catheter ablation in AVNRT, but not in WPW syndrome. The change in electrophysiology immediately after catheter ablation was similar to that following administration of atropine sulfate in AVNRT 3 months after catheter ablation. These findings suggest that catheter ablation does not cause a permanent change in AV nodal physiology and that the location of the ablated site plays an important role in AV nodal physiology.

A 58-year-old woman presented with palpitation. A resting electrocardiogram revealed sinus rhythm and first-degree atrioventricular (AV) block. Transthoracic echocardiography showed a 15 mm × 17 mm round tumour in the lower interatrial septum ( Panel A ) that appeared as high signal intensity on T1-weighted magnetic resonance …

房室結節回帰性頻拍 (AVNRT) のSlow pathwayに対する高周波力テーテルアブレーションの急性期, 慢性期の有効性, 安全性を検討した.Slow pathwayアブレーションを施行した15例を急性期の対象とし, 3カ月後に電気生理学的検査 (EPS) を受けた12例を慢性期の対象とした.急性期にSlow pathwayは全例で遮断され, アブレーション直後のEPSでfast Pathwayの有効不応期の有意な短縮を認めた.またアブレーション後に血清OK値の上昇を認めず, その他の合併症も認めなかった.3カ月後のEPSでもslow pathwayの消失が確認され, アブレーション前との比較で房室伝導能に有意な変化を認めなかった.アブレーション直後, 3カ月後のEPSと臨床成績の検討から, slow pathwayアブレーションはfast Pathwayの伝導性に一過1生の影響を与えるが恒久的変化を残さず, 安全にAVNRTの根治が可能な方法であることが示された.

2週間に1回以上の頻度で生じる自覚症状の強い発作性心房細動/粗動116例のうちの薬剤抵抗性の33例 (28%) と, 反復性心房頻拍7例を対象にIc群抗不整脈薬の効果を検討した.フレカイニド200mg, ピルジカイニド200mg, プロパフェノン450mgのいずれかが, 重複例を含めた発作性心房細動/粗動の33例中14例 (42%) に有効であった.有効例と無効例で年齢, 性別, 左室駆出分画, 左房径に有意差はなく, 発症後1年未満の例は有効例で50%, 無効例で11% (P

A 32-year-old Japanese male visited our hospital on May 20, 2004 because of ventricular premature contractions detected at a regular medical check up. He had been asymptomatic with adequate exercise capacity. However, echocardiography findings and Holter monitoring showed impaired left ventricular systolic function associated with left ventricular dilation and non-sustained ventricular tachycardia on June 26. Coronary angiography did not show significant stenosis. Different types of non-sustained ventricular tachycardia could be induced by an electrophysiologic test, but not sustained ventricular tachycardia. The physical findings, such as enlargement of the nose, lips, hands and feet, suggested endocrinologic abnormalities, and growth hormone and somatomedin-C levels were elevated. Therefore, the final diagnosis was cardiomyopathy caused by acromegaly.

A 71-year old woman with schizophrenia was admitted to our hospital on November 26, 2002 under a diagnosis of acute myocardial infarction because of ST segment elevation in leads V2-V5 and positive serum cardiac troponin T level. Emergent coronary angiography showed no significant stenosis, but left ventriculography demonstrated apical ballooning and basal hyperkinesis. The final diagnosis was ampulla cardiomyopathy. Myocardial contrast echocardiography during the acute phase revealed an echocontrast defect consistent with the area of apical ballooning. The condition improved gradually as determined by disappearance of the abnormal wall motion. The coronary flow reserve in the left anterior descending artery measured by transthoracic Doppler echocardiography was decreased at 1.5 in the acute phase and gradually recovered over a period of 3 months (23 days, 1.9; 96 days, 2.9). Microvascular stunning defined as decreased coronary flow reserve was observed and recovery was delayed in this case compared with cases of myocardial stunning. These findings are of interest in evaluating the pathophysiology of ampulla cardiomyopathy.

The purpose of this study was to examine the effects of microwave catheter ablation of ventricular myocardium. Microwave energy with a frequency of 2450 MHz was delivered via a coaxial catheter with an electrode ball tip. Microwave energy was applied to canine isolated left ventricular endocardium in vitro and to 6 anesthetized dogs in vivo at 50 watts for 15-150 sec. Ventricular arrhythmia was not observed during ablation in any of the dogs when microwave energy was applied for less than 45 sec. When the duration of microwave ablation was greater than 45 sec, ventricular premature contractions were observed in all of the dogs. Nonsustained ventricular tachycardia developed when the duration of microwave delivery was greater than 90 sec. After the cessation of ablation, ventricular arrhythmias did not occur and ventricular programmed stimulation did not induce ventricular tachycardia in any of the dogs. Except for ventricular arrhythmia, no declines in the hemodynamic status were observed in any of the 6 dogs. The size of the ablated lesion was significantly greater as the duration of ablation was increased (p

Background: Although it is well known about the beat-to-beat variability of ventricular repolarization, the quantitative evaluation method has not been still established. To assess the beat-to-beat variability of ventricular repolarization, we investigated the beat-to-beat variability of QT interval (BVR). Methods: An electrocardiography at rest was obtained in 26 normal subjects and 25 patients with any heart diseases such as paroxysmal atrial fibrillation, hypertension, ischemic heart disease, or heart failure. The QT interval was measured automatically using wavelet transformation method, and the Poincare plot graph was constructed using QTn and QTn+1 during consecutive 50 normal sinus beats. The BVR was expressed as a long-term variability (LTV) and a short-term variability (STV) in the Poincare plot graph. Results: The Poincare plot graph could be constructed clearly in all cases. Both LTV and STV were significantly longer in patients with any heart diseases compared with normal subjects (LTV; 6.7±5.0 vs 3.8±1.9 msec, STV; 6.4±5.3 vs 2.8±1.3 msec). Conclusion: This study demonstrates that patients with heart diseases have a large BVR compared with normal subjects, since LTV and STV were significantly longer in those patients. This shows that the BVR using the Poincare plot graph may be useful to evaluate beat-to-beat variability of ventricular repolarization abnormality quantitatively.