Spontaneous Remission Without Steroid Therapy in Isolated Cardiac Sarcoidosis with Severe Left Ventricular Systolic Dysfunction.

Spontaneous remission is often observed in extracardiac cases of sarcoidosis, such as skin sarcoidosis. However, for cardiac sarcoidosis (CS), the prognosis is unfavorable. Although corticosteroids are the first-line treatment for CS, data regarding the natural history of isolated CS are limited. We describe a rare case of isolated CS with severe left ventricular systolic dysfunction that improved without steroid therapy.

Coexistence of Spontaneous Coronary Artery Dissection, Takotsubo Cardiomyopathy, and Myocardial Bridge.

We report a case of spontaneous coronary artery dissection located next to a myocardial bridge in a patient with concomitant takotsubo cardiomyopathy. A fusion image with multidetector-row computed tomography and single-photon emission computed tomography played an important role in the diagnosis of these lesions. (Level of Difficulty: Advanced.).

Real-World Safety Profile of Atrial Fibrillation Ablation Using a Second-Generation Cryoballoon in Japan: Insight From a Large Multicenter Observational Study.

OBJECTIVES: This study sought to investigate the incidence and characteristics of the real-world safety profile of second-generation cryoballoon ablation (2nd-CBA) in Japan. BACKGROUND: Pulmonary vein isolation using second-generation cryoballoons is an accepted atrial fibrillation ablation strategy. METHODS: This multicenter observational study included 4,173 patients with atrial fibrillation (3,807 paroxysmal) who underwent a 2nd-CBA in 18 participating centers. The baseline data and details of all procedure-related complications within 3 months post-procedure in consecutive patients from the first case at each center were retrospectively collected. RESULTS: Adjunctive ablation after the pulmonary vein isolation was performed in 2,745 (65.8%) patients. Complications associated with the entire procedure were observed in 206 (4.9%) total patients, and in the multivariate analysis, the age (odds ratio: 1.015; 95% confidence interval: 1.001 to 1.030; p = 0.035) and study period were predictors. Air embolisms manifesting as ST-segment elevation and cardiac tamponade requiring drainage occurred in 63 (1.5%) and 15 (0.36%) patients, respectively. Six (0.14%) patients had strokes/transient ischemic attacks, among whom 5 underwent ablation under an interrupted anticoagulation regimen. No atrioesophageal fistulae occurred; however, 10 (0.24%) patients had symptomatic gastric hypomotility. Esophageal temperature monitoring did not reduce the incidence, and the incidence was significantly higher in patients with adjunctive posterior wall isolations or mitral isthmus ablation than those without (p = 0.004). Phrenic nerve injury occurred during the 2nd-CBA in 58 (1.4%) patients; however, all were asymptomatic and recovered within 13 months. One patient died of aspiration pneumonia. CONCLUSIONS: This study had a high safety profile of 2nd-CBA despite including the early experience and high rate of adjunctive ablation. Care should be taken for air embolisms during 2nd-CBA.

Remote Management of Pacemaker Patients With Biennial In-Clinic Evaluation: Continuous Home Monitoring in the Japanese At-Home Study: A Randomized Clinical Trial.

BACKGROUND: Current expert consensus recommends remote monitoring for cardiac implantable electronic devices, with at least annual in-office follow-up. We studied safety and resource consumption of exclusive remote follow-up (RFU) in pacemaker patients for 2 years. METHODS: In Japan, consecutive pacemaker patients committed to remote monitoring were randomized to either RFU or conventional in-office follow-up (conventional follow-up) at twice yearly intervals. RFU patients were only seen if indicated by remote monitoring. All returned to hospital after 2 years. The primary end point was a composite of death, stroke, or cardiovascular events requiring surgery, and the primary hypothesis was noninferiority with 5% margin. RESULTS: Of 1274 randomized patients (50.4% female, age 77±10 years), 558 (RFU) and 550 (Conventional follow-up) patients reached either the primary end point or 24 months follow-up. The primary end point occurred in 10.9% and 11.8%, respectively (P=0.0012 for noninferiority). The median (interquartile range) number of in-office follow-ups was 0.50 (0.50-0.63) in RFU and 2.01 (1.93-2.05) in conventional follow-up per patient-year (P<0.001). Insurance claims for follow-ups and directly related diagnostic procedures were 18 800 Yen (16 500-20 700 Yen) in RFU and 21 400 Yen (16 700-25 900 Yen) in conventional follow-up (P<0.001). Only 1.4% of remote follow-ups triggered an unscheduled in-office follow-up, and only 1.5% of scheduled in-office follow-ups were considered actionable. CONCLUSIONS: Replacing periodic in-office follow-ups with remote follow-ups for 2 years in pacemaker patients committed to remote monitoring does not increase the occurrence of major cardiovascular events and reduces resource consumption. Registration: URL: https://clinicaltrials.gov; Unique identifier: NCT01523704.

Lifestyle Modification or Medication to Improve Condition of Patients With Asymptomatic Heart Failure　- Monitoring and Management of OptiVol Alert to Reduce Heart Failure Hospitalization II (MOMOTARO II) Study.

BACKGROUND: In heart failure (HF) patients implanted with high-energy devices, worsening of HF can be diagnosed from intrathoracic impedance (ITI) before symptoms appear. Early therapeutic intervention can prevent HF worsening, but the optimal intervention remains unknown. This study aimed to examine which lifestyle modifications or medications can improve HF indicators in asymptomatic HF patients diagnosed from ITI.Methods and Results:This multicenter, prospective, randomized study included patients with high-energy devices, left ventricular ejection fraction <40%, or with a history of HF hospitalization. After the OptiVol alert was evoked by decreased ITI, patients underwent examinations. If they were diagnosed with HF, they were randomly assigned to 3 groups: lifestyle modification, diuretic, or nitrate. After 1 week, they underwent the same examinations. The primary endpoint was change in ITI and serum B-type natriuretic peptide (BNP). Totally, 57 patients were randomized. In all 3 groups, ITI was significantly increased post-intervention compared with pre-intervention. In the diuretic and nitrate groups, logBNP post-intervention was significantly lower than pre-intervention, but not in the lifestyle modification group. CONCLUSIONS: Compared with lifestyle modifications, diuretic and nitrate therapy for 1 week may be more effective management of HF detected by decreased ITI. However, lifestyle modification may have the additional benefits of reducing the workload or cost.

The Role of Saline and Sodium Bicarbonate Preprocedural Hydration to Prevent Mid-term Renal Insufficiency in Patients with Chronic Kidney Disease Undergoing Percutaneous Coronary Intervention.

Objective Persistent renal damage (RD) three months after exposure to contrast media is associated with contrast-induced acute kidney injury (CI-AKI) and poor clinical outcomes. Little is known about the role of preprocedural hydration on persistent RD in patients with chronic kidney disease [CKD; estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2] undergoing percutaneous coronary intervention (PCI). We therefore examined the use of preprocedural hydration to decrease the incidence of persistent RD. Methods Between 2012 and 2015, 1,230 consecutive patients undergoing PCI, except for patients with an eGFR ≥60 mL/min/1.73 m2, on dialysis, having acute myocardial infarction, or recently having started renin-angiotensin inhibitors, were screened (n=333). Before their index PCI, the 12-h saline group (n=103) received 1 mL/kg/h 0.9% sodium chloride for 12 hours, and the 1-h bicarbonate group (n=63) received 3 mL/kg 154 mEq/L sodium bicarbonate for 1 hour. The control group (n=167) received no pre-procedural hydration. The study outcome of kidney function decline was investigated using the percent-change (%-change) of the calculated creatinine clearance between the baseline value and the lowest value recorded three to six months after index PCI. Results There was less renal function deterioration in the saline group than in the control group, and the bicarbonate group showed deterioration similar to the other groups (%-change; 12-h saline 2.0±11.3% vs. control -5.6±12.6%, p<0.001; vs. 1-h bicarbonate -1.8±14.1%, p=0.18; 1-h bicarbonate vs. control, p=0.14 ANOVA). A multiple regression analysis adjusted for risk factors for persistent RD showed that saline hydration correlated independently with a higher %-change (r=0.262, p<0.001). Conclusion Preprocedural 12-h saline may be better than no preprocedural hydration in preventing mid-term renal insufficiency in CKD patients undergoing PCI.

The role of integrated backscatter intravascular ultrasound in characterizing bare metal and drug-eluting stent restenotic neointima as compared to optical coherence tomography.

BACKGROUND: To evaluate the role of integrated backscatter intravascular ultrasound (IB-IVUS) in assessing the morphology of neointima in bare-metal stent (BMS) and drug-eluting stent (DES) restenosis as compared to the gold-standard, optical coherence tomography (OCT). METHODS: A total of 120 cross-sections were evaluated by IB-IVUS and OCT at five cross-sections from 24 patients (24 lesions): at the minimal lumen area (MLA) and at 1 and 2mm proximal and distal to the MLA site in 24 lesions (9 treated with DES and 15 treated with BMS). IB-IVUS and OCT findings were analyzed according to the time at which restenosis was identified (early <12 months and late ≥12 months) and the stent type. RESULTS: IB-IVUS was found to correctly characterize the neointima of both BMS and DES in-stent restenosis (ISR) as compared to OCT. The overall agreement between the pattern of ISR neointima by IB-IVUS and that by OCT was excellent (kappa=0.85, 95% CI 0.76-0.94). Late DES ISR was characterized by more non-homogeneous, low backscatter and lipid-laden neointima, as compared to the BMS equivalent (BMS vs. DES, 45.0% vs. 80.0%, p<0.01; 51.7% vs. 85.0%, p=0.008; 33.3% vs. 65.0%, p<0.01, respectively). CONCLUSIONS: IB-IVUS assessment of the ISR neointima pattern appears to provide similar information as the gold-standard OCT in patients with stable angina. Both modalities suggested that late DES restenosis is characterized by a non-homogeneous lipid-laden neointima.

Impact of combined supine and prone myocardial perfusion imaging using an ultrafast cardiac gamma camera for detection of inferolateral coronary artery disease.

BACKGROUND: Although combined supine and prone acquisitions improve the detection of inferolateral obstructive coronary artery disease (CAD), the predictors of inaccurate detection of inferolateral ischemia have not been reported by using cadmium zinc telluride (CZT) myocardial perfusion imaging (MPI). METHODS AND RESULTS: Vasodilator stress (99m)Tc tetrofosmin MPI using CZT camera and coronary angiography was performed in 322 patients within an interval of 2 months. Prone MPI was performed immediately after supine MPI. Narrowing of the luminal diameter ≥ 75% was considered significant. The presence of an abnormality on both supine and prone images was considered significant. Combined supine and prone imaging, compared with supine-only quantification, was more specific (93% vs. 72%, respectively, p<0.0001) and accurate (88% vs. 74%, p<0.0001) without compromising sensitivity (82% vs. 68%, p=0.10). The area under the curve for detecting inferolateral ischemia was 0.769 (95% CI 0.705-0.833) for supine imaging and 0.802 (95% CI 0.730-0.875) for combined supine and prone imaging (p<0.05). Multivariable analysis revealed that previous inferolateral myocardial infarction was an independent predictor of a false diagnosis (odds ratio=3.45, 95% confidence interval [CI] 1.62-7.37, p<0.001). CONCLUSIONS: Combined supine and prone quantitative CZT MPI enhances the detection of inferolateral CAD without adversely affecting its sensitivity. However, we recommend inferolateral ischemia be monitored in patients with a history of previous inferolateral MI because previous inferolateral MI is a predictor of inaccurate diagnosis.

Calcification analysis by intravascular ultrasound to define a predictor of left circumflex narrowing after cross-over stenting for unprotected left main bifurcation lesions.

OBJECTIVES: The aim of this study was to identify predictors of significant LCx-ostium compromise after distal unprotected left main coronary artery (ULMCA) stenting on the basis of baseline intravascular ultrasound (IVUS). BACKGROUND: Provisional single-stenting is considered as the default strategy for non-true bifurcation lesions in ULMCA. However, in certain cases, left circumflex artery (LCx)-ostium stenting is necessary. METHODS: A total of 77 patients underwent percutaneous coronary intervention with drug-eluting stents for non-true bifurcation lesions in ULMCA and had IVUS evaluation. Pre-procedural IVUS was performed to measure cross-sectional areas at the following segments: left main trunk, left anterior descending artery (LAD)-ostium. Post-stenting-narrowing at the circumflex ostium (PSN-LCx) was defined as the presence of more than 50% diameter stenosis at the LCx-ostium as determined by quantitative coronary angiography analysis. RESULTS: PSN-LCx occurred in 27 (35%) patients. The presence of calcified plaque at the culprit lesion as identified by IVUS was more frequently observed in the PSN-LCx group as compared to the non-PSN-LCx group (81.5% vs. 22.0%, p<0.001). Calcium arc in the PSN-LCx group was significantly greater than that in the non-PSN-LCx group (118.1°±69.9° vs. 36.9°±63.0°, p<0.001). On multivariable analysis, a calcium arc>60° was an independent predictor of PSN-LCx (odds ratio: 5.12, 95% confidence interval: 1.21-25.01, p=0.03). CONCLUSIONS: The presence of calcified plaque at the culprit lesion appears to be one of the factors involved in LCx-ostial compromise in non-true bifurcation ULMCA lesions, especially when the calcium arc is >60°.

Appearance of neointima according to stent type and restenotic phase: analysis by optical coherence tomography.

AIMS: The features of neointima after bare metal stent (BMS) or drug-eluting stent (DES) implantation have not yet been fully characterised. The aim of this study was to investigate in-stent neointima characteristics according to stent type and restenotic phase. METHODS AND RESULTS: The study included 59 consecutive patients undergoing target lesion revascularisation for in-stent restenosis (ISR) evaluated by optical coherence tomography (OCT) during the early phase (≤1 year, n=30) and late phase (>1 year, n=29) after either BMS (n=37) or DES (n=22) implantation. The OCT signal patterns of tissues at the minimal lumen area were categorised into three patterns: (1) homogeneous high-signal band, (2) heterogeneous mixed-signal band, and (3) lipid-laden intima. The predominant OCT pattern was homogeneous high-signal band in the BMS early phase (19/21 [91%]), lipid-laden intima in the BMS late phase (12/16 [76%]), and heterogeneous mixed-signal band in the DES late phase (9/13 [69%]). Heterogeneous mixed-signal band was seen more frequently in the DES early phase compared with BMS early phase (44% vs. 9%, p<0.05). CONCLUSIONS: There were differences of neointima according to stent type and restenotic phase, and this may lead to a better understanding of the different mechanisms of ISR.

SCN5A mutation is associated with early and frequent recurrence of ventricular fibrillation in patients with Brugada syndrome.

BACKGROUND: Mutations in SCN5A are reportedly linked to Brugada syndrome (BS), but recent observations suggest that they are not necessarily associated with ventricular fibrillation (VF) in BS patients. Therefore, the clinical importance of SCN5A mutations in BS patients was examined in the present study. METHODS AND RESULTS: The 108 BS patients were examined for SCN5A mutations and various parameters were compared between patients with and without mutations. An implantable cardioverter defibrillator (ICD) was implanted in 49 patients and a predictor of appropriate ICD shock was investigated. The existence of a SCN5A mutation was not associated with initial VF episodes (21.7% vs 20.0%, P=0.373). In the secondary prevention group, appropriate shock-free survival rate was significantly lower in patients with spontaneous type 1 ECG than in those without (41.1% vs 85.7% at 2 years, P=0.014). The appropriate shock-free survival rate was also significantly lower in patients with SCN5A mutations than in those without (28.6% vs 83.3% at 1 year, P=0.040). Appropriate shock was more frequent in patients with SCN5A mutations than in those without (6.6±6.2 vs 1.7±3.0, P=0.007). CONCLUSIONS: SCN5A mutations are associated with early and frequent VF recurrence, but not with initial VF episodes. This is the first report on the genotype-phenotype interaction and clinical significance of this mutation.

Abnormal restitution property of action potential duration and conduction delay in Brugada syndrome: both repolarization and depolarization abnormalities.

AIMS: This study sought to examine the action potential duration restitution (APDR) property and conduction delay in Brugada syndrome (BrS) patients. A steeply sloped APDR curve and conduction delay are known to be important determinants for the occurrence of ventricular fibrillation (VF). METHODS AND RESULTS: Endocardial monophasic action potential was obtained from 39 BrS patients and 9 control subjects using the contact electrode method. Maximum slopes of the APDR curve were obtained at both the right ventricular outflow tract (RVOT) and the right ventricular apex (RVA). The onset of activation delay (OAD) after premature stimulation was examined as a marker of conduction delay. Maximum slope of the APDR curve in BrS patients was significantly steeper than that in control subjects at both the RVOT and the RVA (0.77 +/- 0.21 vs. 058 +/- 0.14 at RVOT, P = 0.009; 0.98 +/- 0.23 vs. 0.62 +/- 0.16 at RVA, P = 0.001). The dispersion of maximum slope of the APDR curve between the RVOT and the RVA was also larger in BrS patients than in control subjects. The OAD was significantly longer in BrS patients than in control subjects from the RVOT to RVA and from the RVA to RVOT (from RVOT to RVA: 256 +/- 12 vs. 243 +/- 7 ms, P = 0.003; from RVA to RVOT: 252 +/- 11 vs. 241 +/- 9 ms, P = 0.01). CONCLUSIONS: Abnormal APDR properties and conduction delay were observed in BrS patients. Both repolarization and depolarization abnormalities are thought to be related to the development of VF in BrS patients.

Abnormal transmural repolarization process in patients with Brugada syndrome.

BACKGROUND: Repolarization abnormality, especially during bradycardia, might be critical for initiation of ventricular fibrillation (VF) in patients with Brugada syndrome (BrS), but the contribution of the rate-dependent repolarization dynamics to the occurrence of VF is still unknown. OBJECTIVE: The aim of our study was to determine the differences in rate-dependent repolarization dynamics between BrS with and without spontaneous VF and between BrS with and without SCN5A mutation. METHODS: The subjects were 37 BrS patients with VF (VF(+) group: 10 male subjects) and without VF (VF(-) group: 27 male subjects) and 20 control subjects. Genetic analysis of SCN5A was performed in all 37 BrS patients. The relationships between QT, QTp, Tp-e, and RR intervals were obtained from Holter recordings as first linear regression lines, and the slopes of QT/RR, QTp/RR, and Tp-e/RR linear regression lines as the sensitivity of rate-dependent repolarization dynamics were compared. RESULTS: QT/RR and Tp-e/RR slopes showed loss of a rate-dependent property in the VF(+) group compared with those in the VF(-) and control groups. There was no significant difference in QTp/RR slope among the VF(+), VF(-) and control groups. The Tp-e interval had a negative correlation with the RR interval in the VF(+) group and a positive correlation with the RR interval in the VF(-) and control groups. There was no significant difference in QT/RR, QTp/RR, and Tp-e/RR slopes between BrS patients with SCN5A mutation and those without SCN5A mutation. CONCLUSIONS: Loss of rate-dependent QT dynamics may be associated with occurrence of VF in BrS.

Quantitative analysis and characteristics of the electrograms recorded within the non-coronary aortic sinus of Valsalva.

BACKGROUND: Some supraventricular tachycardias could be ablated from the non-coronary sinus of Valsalva (NSV). However, the characteristics of the NSV electrograms have not been clarified. METHODS AND RESULTS: A quantitative analysis of the NSV electrograms was performed in 5 patients with tachycardias arising from near the atrioventricular node (AVN) and the His-bundle region, and in 20 control subjects. In another 7 control subjects, the NSV electrograms were compared with those recorded at the left and right sinus of Valsalva (LSV and RSV). The NSV electrograms during sinus rhythm had a larger atrial amplitude than ventricular amplitude, and the ratio of the atrial amplitude to the ventricular amplitude was usually >1, which was apparently different from the LSV and RSV electrograms. A tiny but distinct His-bundle deflection was sometimes recorded at the NSV during sinus rhythm while it was not during the tachycardia. The distance to the His-bundle region in the anteroseptal right atrium was shorter from the NSV than from the RSV or LSV. CONCLUSIONS: The precise identification of the catheter position at the NSV is possible using the characteristics of the electrograms. Much attention should be paid during ablation to the NSV because of its vicinity to the AVN and His-bundle region.

Differential effects of cardiac sodium channel mutations on initiation of ventricular arrhythmias in patients with Brugada syndrome.

BACKGROUND: Premature ventricular contractions (PVCs) do not occur frequently but can induce ventricular fibrillation (VF) in patients with Brugada syndrome. The effect of SCN5A mutation on the onset of ventricular arrhythmias is unknown. OBJECTIVE: The purpose of this study was to evaluate PVC morphology and onset of VF in patients with Brugada syndrome. METHODS: Morphology of PVCs was evaluated by 12-lead ECG in 32 patients with Brugada syndrome. Patients had spontaneous ventricular arrhythmia (n = 17) or sodium channel blocker-induced ventricular arrhythmia (n = 19). Patients were classified into two groups according to the existence of SCN5A mutation (22 mutation negative, 10 mutation positive). RESULTS: Patients without mutation often had PVCs of left bundle branch block (LBBB) morphology (82%), especially with inferior axis (77%). Patients with mutation had PVCs of both right bundle branch block (36%) and LBBB (64%) morphologies. Only two patients with mutation had PVCs of LBBB, inferior-axis morphology. CONCLUSION: Patients without SCN5A mutation often had PVCs of LBBB, inferior-axis morphology, suggesting a right ventricular outflow tract origin. Patients with SCN5A mutations had PVCs that originated from both the right and left ventricles.

The characteristics and distribution of the scar tissue predict ventricular tachycardia in patients with advanced heart failure.

BACKGROUND: Contrast-enhanced magnetic resonance imaging (CMR) identifies scar tissue as hyperenhanced areas. We sought to clarify the relationship between the scar characteristics and occurrence of sustained ventricular tachycardia (VT) in patients with advanced heart failure. METHODS: CMR was performed in 29 patients with dilated cardiomyopathy (DCM group) and 18 patients with ischemic cardiomyopathy (ICM group). The characteristics, volume, and distribution of the hyperenhanced areas were analyzed by CMR. The CMR parameters and clinical arrhythmic events were compared between the two groups. RESULTS: In the DCM group, almost all hyperenhanced areas were nontransmural, and presented frequently in the midwall layer. The volume of the hyperenhanced areas and total number of hyperenhanced segments were greater in patients with sustained VT than in those without. On the other hand, in the ICM group, transmural or subendocardial hyperenhanced areas were detected in the territory of the coronary arteries. The volume of the hyperenhanced areas and total number of transmural hyperenhanced segments in patients with sustained VT were unexpectedly smaller than in those without. However, the percentage of nontransmural hyperenhanced segments was greater in patients with sustained VT than in those without. CONCLUSIONS: The presence and magnitude of the nontransmural scar tissue may predict sustained VT in patients with advanced heart failure. There was the possibility that a recruitment bias was responsible for the finding of the smaller scars in the ICM patients with sustained VT.

Prevalence and characterization of pulmonary vein variants in patients with atrial fibrillation determined using 3-dimensional computed tomography.

Although several branching patterns of pulmonary veins (PVs) were reported, their prevalence and characterization were not sufficiently clarified. Multislice computed tomography was performed in 428 patients who underwent catheter ablation for drug-refractory atrial fibrillation. Size and branching pattern of PVs were analyzed. A typical pattern of 4 PVs with 4 separate ostia was found in 326 patients (76%). However, a common PV trunk, defined as a PV with coalescence of superior and inferior PVs > or =1.5 cm proximal to the junction with the left atrium, was found on the left side in 34 patients (8%) and right side in 3 patients (0.76%). A discrete middle PV was found on the right side in 54 patients (13%) and left side in 9 patients (2%). A right top PV, defined as an anomalous insertion of a branch of the right superior PV into the left atrial body, was also found in 16 patients (4%). In conclusion, 24% of our patients with atrial fibrillation had PV anomalies and 3% had coexistence of 2 PV variants, indicating that PV variants are not rare.