Incomplete thrombus resolution in patients with venous thromboembolism (VTE) may increase the risk of recurrent thromboembolic events and other complications, such as post-thrombotic syndrome. Various options exist for thrombus resolution, including systemic thrombolytic agents, catheter-directed thrombolysis, and traditional anticoagulants such as heparins or vitamin K antagonists (VKAs). Data are accumulating on the use of non-VKA oral anticoagulants, such as rivaroxaban, and these may provide greater thrombus resolution compared with VKAs. Data from the phase III rivaroxaban studies presented here show that a 21-day intensive dosing regimen of rivaroxaban 15 mg twice daily is effective during the acute treatment phase for VTE, with similar recurrence rates and thrombus resolution to standard anticoagulation. Pooled analyses of phase III studies have also indicated that rivaroxaban 20 mg once daily monotherapy for up to 12 months after this initial intensive treatment period may provide effective prevention of recurrent VTE and a reduction in the risk of major bleeding, irrespective of clot burden. Four case studies from the Darmstadt Academic Teaching Hospital, Germany, and Gunma University Hospital, Japan, are also provided. Further clinical studies and real-world data may improve our understanding of initial intensive dose regimens, and assess the full significance of thrombus burden in VTE.

Specific signatures of culprit lesions detected on multidetector computed tomography (MDCT) were identified as predictors of periprocedural myocardial injury (PMI) after percutaneous coronary intervention (PCI) in patients with stable angina; PMI has been shown to be associated with a worse prognosis. We investigated the association between preprocedural culprit lesion characteristics, assessed by MDCT, and PMI after PCI for chronic total occlusion (CTO). From three medical centers, 81 patients who underwent pre-PCI MDCT and CTO PCI, and systematic cardiac troponin (cTn) sampling before and after PCI, were included. Patients were divided into two groups according to the presence or absence of post-PCI cTn elevation. Patient characteristics, MDCT findings, and procedural variables were compared between the two groups. Procedure success was observed in 65 patients (80.2%) and was not associated with PMI. The incidence of PMI was higher in patients treated with the retrograde versus the antegrade approach. On MDCT, lesion length and the presence of the napkin-ring sign were significantly associated with PMI. Multivariate analysis revealed that the lesion length (odds ratio [OR]: 1.04; 95% confidence interval [CI]: 1.01–1.08; P < 0.05), napkin-ring sign (OR: 5.41; 95% CI: 1.01–29.0; P < 0.05), and retrograde approach (OR: 4.78; 95% CI: 1.28–15.4; P < 0.05) were significant predictors of PMI. PMI is not uncommon in patients undergoing elective CTO PCI, regardless of procedure success or failure. Pre-PCI MDCT may help identify patients at high risk for PMI after CTO PCI.

The immune system may play important roles in the pathogenesis of cardiovascular disease. T-cell mediated immune responses in human progression of atherosclerotic disease and hypertension have recently been revealed, but the significance of T-cell specific chemokines in coronary artery heart disease has not been confirmed. In our study, we sought to examine the association between serum levels of the monokine induced by gamma interferon (MIG)/CXCL9 and the severity of coronary artery disease. We studied 117 patients with coronary heart disease and 80 patients with no coronary heart disease. The severity of coronary artery disease was assessed via coronary artery angiography and the Gensini score was calculated. Clinical and biochemical indices, including serum levels of MIG, CD40L, and IFN-γ were analyzed in all subjects. Finally, we found there was a significant correlation between serum MIG levels and the severity of coronary artery disease, quantified by the Gensini score (r = 0.122, P = 0.009). Furthermore, multivariate regression analysis revealed that serum MIG levels were independently associated with the severity of coronary artery disease, quantified by the Gensini score (β = 0.100, P = 0.021). Our findings could indicate the potential clinical implication of MIG with respect to early coronary artery atherosclerosis in humans.

The vasopressin receptor 2 (V2) receptor antagonist tolvaptan is an aquaretic agent that has been approved for heart failure patients with volume overload in Japan. In this study (SMILE study), we investigated patient characteristics and effectiveness in both a 14 days and shorter treated group (14DS) and 15 days and longer treated group (15DL). The results showed that the patients in the 15DL group had low cardiac output with intensive diuretic administration (ie, diuretic resistance). The congestive symptoms were greatly improved within 14 days of treatment in both the 14DS and 15DL groups. Further improvements in lower limb edema, pulmonary congestion, dyspnea, third sound, and rales after 2 weeks were statistically significant in the 15DL group, but the amount of improvement was subtle and the 15DL group might have consisted of a considerable number of “non-responders”. Therefore, identifying “responders” by biomarkers and conducting a prospective randomized study is required to validate our findings.

The J-wave has been reported to be associated with life-threatening ventricular arrhythmia. However, the clinical implication of the J-wave is still unclear in patients with an implantable cardioverter defibrillator (ICD).

The study population consisted of 170 ICD patients (age, 56 ± 16 years, 79.4% male) treated at Kitasato University Hospital between 2003 and 2014. Ventricular fibrillation (VF) and ventricular tachycardia (VT) events were documented via ICD interrogation, and the patients were divided into 3 groups: 1) VF event group, 2) VT event group, and 3) No-event group. To predict VT or VF events, univariate and multivariate analysis of clinical data including ECG findings were performed. A J-wave was defined as the presence of notching or slurring of the QRS complex (≥ 0.1 mV) in inferior/lateral leads. Among the 170 patients examined, 23 experienced VF and 38 experienced VT during 54 ± 39 months follow-up. In the multivariate Cox proportional hazards model, the J-wave was identified as an independent predictor for a VF event (HR: 3.886, 95% CI: 1.313-10.568, P = 0.012). In contrast, BNP (HR: 1.002, 95% CI: 1.000-1.003, P = 0.043) and left ventricular diastolic diameter (HR: 1.039, 95% CI: 1.002-1.081, P = 0.049) were independent predictors for a VT event.

The results suggest J-waves in the stable phase in an ECG may be a useful predictor for a VF event in ICD patients.

Atrial tachyarrhythmias (ATAs) occur in a significant proportion of Brugada syndrome (BrS) patients and are often an important cause of inappropriate shocks. The aim of this retrospective study was to evaluate the incidence of ATAs and ATA-induced inappropriate shocks in early repolarization syndrome (ERS) patients as compared to BrS patients.

We analyzed data from 20 consecutive patients who were diagnosed with ERS and compared them with patients diagnosed with BrS (n = 31). Clinical and ICD interrogation data were collected and analyzed for all events with ICD shocks.

Three patients had a history of atrial fibrillation (AF) prior to ICD implantation. One patient had AV reentrant tachycardia and was successfully ablated before ICD implantation. ATAs were newly diagnosed in 4 patients with no prior history of AF. There were no significant differences in gender, age, or left atrial diameter between ATA development. Four (20%) of 20 consecutive patients received inappropriate ICD shocks for ATAs. One suffered from repeat inappropriate shocks triggered by paroxysmal AF and received catheter ablation for AF.

ATAs were not infrequent in patients with ERS and seemed to be related to inappropriate ICD therapy. Careful ICD programming is required to reduce ATA-related inappropriate ICD shock in patients with ERS.

Blood transfusions are considered as an important predictor of adverse outcome in patients with severe aortic (AS) undergoing transcatheter aortic valve implantation (TAVI). We sought to investigate the association between blood transfusions and mortality after TAVI. We enrolled 101 consecutive patients with severe AS undergoing TAVI. Patients who required transfusion were defined as patients in whom at least one unit of packed red blood cells (PRBCs) was transfused in the perioperative period. Twelve-month outcomes were assessed based on Valve Academic Research Consortium definitions. A total of 28 (27.7%) patients required blood transfusion after TAVI. Baseline characteristics of the patients with and without a transfusion were similar. Median amount of PRBCs was 2 (interquartile range, 2-4). Twelvemonth all-cause mortality was higher in patients with than without a blood transfusion (39.3% versus 9.6%; P = 0.001). Importantly, the need for a blood transfusion after TAVI was an independent predictor of higher mortality rates after 12 months (hazard ratio (HR) 2.84 95%CI (1.06-7.63); P = 0.039; (HR for incomplete coronary revascularization 10.86, 95%CI 3.72-31.73; P < 0.001; HR for a history of stroke/TIA 3.93, 95%CI 1.39-11.07; P < 0.001). The duration of inhospital stay was longer in patients requiring transfusion (16.0 (14.0-22.0) versus 7.0 (7.0-11.5) days; P = 0.014). In conclusion, blood transfusions after TAVI were associated with higher mortality rates after 12 months, longer in-hospital stay, and were identified as an independent predictor of impaired clinical outcome.

Severe device landing zone calcification (DLZ-CA) predicted paravalvular leak (PVL) and post-dilatation (PD) after transcatheter aortic valve implantation (TAVI). The aim of this study was to determine the influence of DLZ-CA on PVL or PD rates after SAPIEN XT (XT) versus CoreValve (CV).

We analyzed patients undergoing TAVI who had severe DLZ-CA. Severe DLZ-CA defined the upper left ventricular outflow tract calcification; the cross-sectional region 2 mm inferior to the annular plane. PVL was evaluated at 30days using transthoracic echocardiography. Overall, 133 patients had XT-TAVI and the remaining 41 patients had CV-TAVI. Two patients had annulus injury in the XT group (oversizing 20.2% and 20.5% for two XT cases). PD was less frequently performed in the XT group (34.1% versus 12.8%; P = 0.002), but PVL rates were similar between both groups (42.1% versus 41.5% for the XT and CV groups, respectively; P = 0.94). Importantly, excessive oversizing or the degree of filling volume was not associated with decreased PVL after XT-TAVI (P = non-significant for all). On multivariate analysis, CV-TAVI was found to be one of the independent predictors of need for PD (Odds ratio 3.63, 95% confidence interval 1.55 to 8.53, P = 0.003).

In the setting of severe DLZ-CA, XT and CV have similar rates of PVL but XT had less need for PD. Excessive oversizing with XT carries a risk of root injury which could be further increased by DLZ-CA.

Although a subtype of hypertrophic cardiomyopathy (HCM), dilated phase of HCM (D-HCM) characterized by left ventricular (LV) systolic dysfunction, has been reported to have a poor prognosis, some patients with D-HCM survive for a relatively long period. The degree of LV dilatation and functional mitral regurgitation (MR) are generally thought to be important predictors of poor prognosis in patients with LV systolic dysfunction. However, there is little information available on the relations among LV size, presence of significant MR, and prognosis in D-HCM patients.

We retrospectively studied 31 patients with D-HCM to determine whether echocardiographic assessment of LV size and MR provides incremental prognostic information.

During a follow-up period of 5.6 ± 4.2 years, there were 13 cardiovascular deaths. When the patients were divided into two groups by LV size at diagnosis of D-HCM, a non-dilated LV group (LV end-diastolic diameter (LVEDD) < 50 mm, n = 9) and a dilated LV group (LVEDD ≥ 50 mm, n = 22), the clinical course in the non-dilated LV group was significantly worse. As for the clinical impact of MR, no patient in the non-dilated LV group showed significant MR and 7 of the patients with dilated LV size showed significant MR during follow-up. Once significant MR was reached, cardiovascular deaths were significantly more frequent in patients with MR.

Patients with D-HCM, particularly those with less LV dilatation at diagnosis of dilated phase and with significant MR during follow-up, have a poor prognosis.

The aim of the present study was to evaluate whether the baseline thiol/disulfide state can predict the occurrence of anthracycline induced cardiac toxicity. A total of 186 cancer patients receiving anthracycline (doxorubicin)-based chemotherapy were enrolled. All patients underwent 2-dimensional (2D) speckle tracking echocardiography (STE) to determine their left ventricular ejection fraction (LVEF) and blood samples for measuring thiol forms were obtained before treatment and 4 weeks after completion of the chemotherapy. The mean dose of doxorubicin exposure was 255 ± 39.2 mg/m². Baseline native thiol was found to be lower whereas baseline disulfide and the disulfide/total thiol ratio were found to be higher in patients who had a decrease in LVEF after anthracycline therapy. Also, the amount of decrease in LVEF was well correlated with the delta value of the thiol forms. Logistic regression analysis revealed that changes in BNP and global longitudinal strain (GLS), baseline level of native thiol, disulfide, and the disulfide/total thiol ratio were strong predictors for a decrease in LVEF.

The thiol/disulfide pathway may be a factor for predicting chemotherapy-induced cardiac toxicity as one of the oxidative stress mechanisms.

The pathophysiology of congenital heart disease includes aortic dilation and increased stiffness. However, the clinical determinants and significance remain unclear in patients after the Fontan operation.

Size and stiffness index (SI) of the ascending and descending aorta (aAO and dAO, respectively) were assessed using angiography in 130 consecutive Fontan patients and 30 age-matched controls. Compared with controls, Fontan patients showed a dilated aAO and smaller dAO (P < 0.0001) with greater SI (3.2 ± 0.7 versus 2.2 ± 0.3 for aAO and 2.7 ± 0.6 versus 2.2 ± 0.3 for dAO, P < 0.0001 for both). aAO was stiffer than dAO (P < 0.0001) and the greater aAO size was independently determined by the presence of pulmonary atresia, older age at Fontan operation, and low arterial oxygen saturation (P < 0.05-0.01). High plasma levels of brain natriuretic peptide (BNP) and glucose were independently associated with aAO SI (P < 0.05-0.01) and the SI ratio of aAO to dAO SI, whereas body mass index, plasma levels of highsensitivity C-reactive protein, and dAO size were independently associated with dAO SI (P < 0.05-0.01). A greater aAO and aAO/dAO ratio predicted an impaired exercise blood pressure response (P < 0.0001). Furthermore, in addition to age at Fontan operation and BNP level, the aAO SI independently predicted a lower peak oxygen uptake (P < 0.05).

Fontan patients have a stiffer dilated aAO with rapidly tapering smaller dAO that predicts exercise pathophysiology. In addition to intrinsic aortic structural abnormalities, heart failure severities as well as traditional cardiovascular risk factors are also involved in the aortic structural and functional abnormalities.

Long QT syndrome (LQTS) has great genetic heterogeneity: more than 500 mutations have been described in several genes. Despite many advances, a genetic diagnosis still cannot be established in 25-30% of patients. The aim of the present study was to perform genetic evaluation in 9 Russian families with LQTS; here we report the results of 4 positive probands and their relatives (a total of 16 individuals). All subjects underwent clinical examination, 12-lead ECG, and Holter monitoring. Genetic analysis of the 14 genes mainly involved in LQTS was performed using a next-generation sequencing approach. We identified two new mutations (KCNQ1 gene) and 6 known mutations (AKAP9, ANK2, KCNE1 and KCNJ2 genes) in 4 out of 9 probands, some of which have already been described in association with LQTS. Segregation studies suggest a possible causative role for KCNQ1 p.(Leu342Pro), AKAP9 p.(Arg1609Lys), KCNE1 p.(Asp85Asn), and KCNJ2 p.(Arg82Gln) variations. Our study confirmed the high genetic heterogeneity of this disease and highlights the difficulties to reveal clear pathogenic genotypes also in large pedigrees. To the best of our knowledge, this is the first genetic study of LQTS patients from Russian families.

Heterozygous familial hypercholesterolemia (FH) represents a strong risk for development of premature coronary artery disease (CAD). However, the majority of patients with FH are undiagnosed and the prevalence likely represents an underestimate in most countries. In Japan, the possible contribution of FH to the development of CAD may be higher because of the low incidence of CAD among the general population. We estimated the prevalence of heterozygous FH by measuring Achilles tendon thickness (ATT) in patients with acute coronary syndrome (ACS).

A total of 359 patients suffering from ACS were enrolled in this multicenter registration study. Heterozygous FH was defined according to the diagnostic criteria proposed by the Japan Atherosclerosis Society. After excluding 63 patients because of missing ATT data or plasma triglyceride levels that were 4.5 mmol/L or more, 296 patients were eligible for inclusion in the study. The number of patients with ATT of 9 mm or more was 53 (17.9%). They were significantly younger and had significantly higher LDL cholesterol levels than patients with an ATT less than 9 mm. The prevalence of heterozygous FH was 5.7% (1/17.5) and more prominent in younger patients who were less than 60 years old (7.8%). In patients with ATT of 9 mm or more, approximately 1 in 3.5 fulfilled the criteria for heterozygous FH.

We demonstrated the usefulness of measuring ATT by radiography and the high prevalence of heterozygous FH in patients with ACS in Japan, especially in younger patients who were less than 60 years old.

Mutations in mitochondrial DNA are associated with the pathogenesis of essential hypertension. We report here the clinical, genetic, and molecular characterization of a three-generation Han Chinese family with essential hypertension. Most strikingly, this family exhibited a high penetrance of essential hypertension. Sequence analysis of the mitochondrial genome showed the presence of a homoplasmic T5655C mutation in tRNA^Ala, together with the A4401G mutation in the adjacent region between tRNA^Met and tRNA^Gln. Notably, the T5655C mutation was localized at the acceptor arm of tRNA^Ala, disrupted the high conserved base-pairing (1A-72T), and may impair the tRNA function. Moreover, the A4401G mutation was reported to decrease the steady-state level of tRNA^Met and tRNA^Gln, and consequently caused the mitochondrial dysfunction responsible for hypertension. Taken together, the combination of T5655C and A4401G mutations in mitochondrial tRNA genes may account for the high penetrance and expressivity of hypertension in this Chinese family. Thus, our findings may provide new insight into the pathogenesis of this disorder.

The mechanism of cardiac hypertrophy involving microRNAs (miRNAs) is attracting increasing attention. Our study aimed to investigate the role of miR-10a in cardiac hypertrophy development and the underlying regulatory mechanism.

Transverse abdominal aortic constriction (TAAC) surgery was performed to establish a cardiac hypertrophy rat model, and angiotensin II (AngII) was used to induce cardiac hypertrophy in cultured neonatal rat cardiomyocytes. Expression of T-box 5 (TBX5) and miR-10a was altered by cell transfection of siRNA or miRNA mimic/inhibitor. Leucine incorporation assay, histological and cytological examination, quantitative real-time PCR (qRT-PCR), and Western blot were performed to detect the effects of miR-10a and TBX5 on cardiac hypertrophy. Dual-luciferase reporter assay was conducted to verify the regulation of TBX5 by miR-10a.

miR-10a was down-regulated, and TBX5 was up-regulated in the rat model and AngII-stimulated cardiomyocytes. miR-10a inhibited TBX5 expression by directly targeting the binding site in Tbx5 3’UTR. Overexpression of miR-10a in AngII-treated cardiomyocytes decreased relative cell area, and significantly reduced the mRNA levels of natriuretic peptide A (Nppa), myosin heavy chain 7 cardiac muscle beta (Myh7), and leucine incorporation (P < 0.01 or P < 0.001). Knockdown of Tbx5 had similar effects on AngII-induced cardiomyocytes.

Our findings indicate that miR-10a may inhibit cardiac hypertrophy via targeting Tbx5. Thus, miR-10a provides promising therapeutic strategies for the treatment of cardiac hypertrophy.

Cardiac fibrosis is a pathological feature of myocardium of failing heart and plays causative roles in arrhythmia and cardiac dysfunction, but its regulatory mechanisms remain largely elusive. In this study, we investigated the effects of the novel EP4 receptor agonist ONO-0260164 on cardiac fibrosis in hypertrophied heart and explored the regulatory mechanisms in cardiac fibroblasts.

In a mouse model of cardiac hypertrophy generated by transverse aortic constriction (TAC), ONO-0260164 treatment significantly prevented systolic dysfunction and progression of myocardial fibrosis at 5 weeks after TAC. In cultured neonatal rat cardiac fibroblasts, transforming growth factor-β1 (TGF-β1) induced upregulation of collagen type 1, alpha 1 (Col1a1) and type 3, alpha 1 (Col3a1), which was inhibited by ONO-0260164 treatment. ONO-0260164 activated protein kinase A (PKA) in the presence of TGF-β1 in the cardiac fibroblasts. PKA activation suppressed an increase in collagen expression induced by TGF-β1, indicating the important inhibitory roles of PKA activation in TGF-β1mediated collagen induction.

We have demonstrated for the first time the antifibrotic effects of the novel EP4 agonist ONO-0260164 in vivo and in vitro, and the important role of PKA activation in the effects.

Atherosclerosis is a multifactorial vascular disease characterized by formation of inflammatory lesions. Secretory phospholipase A2, group IIA (sPLA2-IIA) is involved in this process and plays a critical role. However, the exact role of sPLA2-IIA in cardiovascular inflammation is more complicated and remains unclear. Furthermore, both statins and Xuezhikang (XZK) are widely used in the prevention and treatment of cardiovascular disease risk because of their pleiotropic effects on the cardiovascular system. However, their effects on sPLA2-IIA are still controversial. We investigated the regulation of sPLA2-IIA by rat thoracic aorta smooth muscle cells (VSMCs) in culture. Cells were first incubated with IL-1β alone to induce expression of sPLA2-IIA and then treated with several concentrations of statins or XZK for different times in the absence or presence of IL-1β. We tested the expression of sPLA2-IIA, including sPLA2-IIA mRNA, protein, as well as activity. We found that statins or IL-1β increase the expression of sPLA2-IIA in VSMCs and the effect is based on a synergetic relationship between them. However, for the first time, we observed that XZK effectively reduces sPLA2-IIA expression in IL-1β-treated VSMCs. Our findings may shine a new light on the clinical use of XZK and statins in the prevention and treatment of atherosclerosis-related thrombosis.

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 68-year-old female with acute coronary syndrome was transferred to our hospital. Emergency coronary angiography showed 90% stenosis with severe calcification in the proximal right coronary artery (RCA). Intravascular ultrasound (IVUS) images were obtained and showed circumferential heavy calcification without any evidence of plaque rupture. Optical frequency domain imaging (OFDI) images were obtained in the RCA lesion 3 days after the initial coronary angiography. A cavity of plaque rupture in the calcified plaque by using OFDI was observed in the lesion, which could not be recognized by IVUS. Necrotic tissue was observed frequently in heavy calcified lesions and was usually hidden behind calcification. Judging from the OFDI images in this case, the thin fibrous cap over the necrotic tissue even if surrounded by calcification was disrupted and might have caused the acute coronary syndrome. However, necrotic tissue surrounded by calcification is generally recognized as calcified plaque in OFDI images because discrimination between necrotic tissue and calcification is based on the border characteristics (low intensity with diffuse border: necrotic tissue, low intensity with sharp border: calcification). Superficial residual necrotic tissue not yet replaced completely by calcification might cause plaque rupture and thus, result in acute coronary syndrome. In fact, there is a variety of OFDI and optical coherence tomography (OCT) characteristics in calcified plaque, such as relatively high intensity without attenuation or very low intensity with attenuation. Residual necrotic tissue within calcification could pose a problem in OCT/OFDI plaque evaluation.

The drug-coated balloon (DCB) is a device that is used to reduce the risk of stent re-implantation in patients with in-stent restenosis (ISR). However, imaging findings of the drug covering the neointimal plaque immediately after treatment of ISR by DCB, and during follow-up, have only been discussed in a few reports. Herein, we describe the use of optical coherence tomography (OCT) and angioscopy to evaluate ISR before and after treatment with DCB, and during the follow-up period in 3 patients. The patients developed critical ISR during the follow-up period after drug-eluting stent (DES) implantation. The patients included a 70-year-old woman, a 70-year-old man, and an 80-year-old man. These imaging modalities provided data about the various etiologies of ISR, and about the efficacy of DCB angioplasty. Based on the findings of the intracoronary images in these 3 cases, we concluded that DCB might not only inhibit neointimal proliferation, but also reduce neointimal volume and lead to changes in in-stent neointimal morphology.

Cardiac involvement in systemic sarcoidosis sometimes provokes life-threatening ventricular tachyarrhythmia. Steroid administration is one of the fundamental anti-arrhythmia therapies. For an indication of steroid therapy, a definitive diagnosis of sarcoidosis is required.¹⁾ However, cases that are clearly suspected of cardiac sarcoidosis based on their clinical courses sometimes do not meet the current diagnostic criteria and result in the loss of an appropriate opportunity to perform steroid therapy.

Here we report a case that was diagnosed as sarcoidosis by incidental biopsy of an inguinal lymph node during cardiac resuscitation for cardiac tamponade.²⁾ While the inguinal lymph node was not swollen on computed tomography, a specimen obtained from an incidental biopsy during the exposure of a femoral vessel for the establishment of extracorporeal cardio-pulmonary resuscitation showed a non-caseating granuloma.

This findings suggest a non-swelling lymph node biopsy might be an alternative strategy for the diagnosis for sarcoidosis if other standard strategies do not result in a diagnosis of sarcoidosis.

We report progressive enlargement of the excluded left atrial appendage (LAA) with a thrombus in a patient who had undergone valve surgery and endocardial suture closure of the LAA previously. Echocardiography and CT detected no communication between the LAA and the left atrium. Magnetic resonance imaging showed the LAA was filled with fresh and old thrombi. Coronary arteriography demonstrated small left coronary artery-LAA fistulae. At surgery, successful exclusion of the LAA was confirmed after removal of the thrombi. Persistent inflow of blood through the coronary artery fistulae to the excluded LAA may be the primary mechanism of this pathology.

We report here the case of a 67-year-old man who was initially diagnosed with myocardial hypertrophy with progressive hypertensive heart disease. After 6 years a cardiac biopsy was conducted because of changes in the electrocardiogram and transthoracic echocardiogram results, revealing amyloid deposition. Additional genetic studies revealed no TTR gene mutations, leading to a definitive diagnosis of wild-type transthyretin-related amyloidosis (ATTR). The patient started taking diflunisal as a stabilizer which is one of the advanced therapies for ATTR, and then the heart failure symptoms and brain natriuretic peptide (BNP) level improved in short-term follow-up. We present an elderly patient with ATTR, which is believed to be rare. We also discuss the apparent efficacy of novel therapeutic agents that increase the incentive to diagnose ATTR at an early stage. Therefore, we should always consider the existence of cardiac amyloidosis when we initiate the management of an elderly patient with cardiac wall thickening.

Systemic right ventricular (RV) failure in patients with congenitally corrected transposition of the great arteries (ccTGA), a major cause of mortality in the long-term follow-up, is usually induced by concomitant severe morphologically tricuspid regurgitation (TR) with/without Ebstein’s anomaly or progressive conduction tissue disturbances. However, whether or not myocardial fibrosis is a common cause of systemic RV failure in patients with ccTGA remains unclear. Here, we describe an 82-year-old man who had been diagnosed previously as having uncomplicated ccTGA and situs inversus and recently developed systemic RV failure, which was neither associated with severe TR nor advanced conduction tissue abnormalities. Cardiovascular magnetic resonance (CMR) with delayed-enhancement imaging clearly detected extensive myocardial scars (presumably fibrosis) in the RV wall as well as prominent dilatation, hypertrophy, and systolic dysfunction of the systemic RV. These findings suggest that myocardial fibrosis can cause systemic RV failure in elderly patients with uncomplicated ccTGA despite the absence of severe TR or advanced conduction tissue abnormalities and that CMR may be a useful examination to accurately detect systemic RV failure associated with myocardial fibrosis and to subsequently clarify the prognosis in these patients.