Possibility of steroid therapy without pacemaker implantation in patients with sarcoidosis presenting atrioventricular block.

Atrioventricular Block (AVB) is one of the common manifestations in cardiac sarcoidosis (CS). Although pacemaker implantation is generally recommended in patients with CS complicated by symptomatic AVB, some case reports have shown that they can be managed by steroid therapy without pacemaker implantation. The aim of this study was to evaluate the feasibility and effectiveness of steroid therapy without pacemaker implantation in patients with CS complicated by symptomatic AVB. We performed medical record review of consecutive ten CS patients who admitted Nippon Medical School Hospital for symptomatic second or third degree AVB between April 2015 and March 2021. Of the studied population, steroid therapy before pacemaker implantation was feasible in three patients with second degree AVB. Two of them showed subsequent recovery of atrioventricular conduction to 1:1, and they were managed by steroid therapy without pacemaker. The remaining one patient showed no improvement of atrioventricular conduction and required pacemaker implantation. Seven patients with third degree AVB required device implantation (pacemaker; n = 7, cardiac resynchronization therapy defibrillator; n = 1) before steroid therapy mainly because of hemodynamic instability. Steroid therapy without pacemaker implantation might be feasible, and possibly be effective in patients with CS presenting second degree AVB. However, the feasibility is limited in patients with third degree AVB.

Characteristics of patients with atrial flutter and spontaneous 1:1 atrioventricular conduction with and without anti-arrhythmic drug treatment.

Atrial flutter (AFL) is a large reentrant circuit located in the right atrium. Anti-arrhythmic drugs (AADs) can provoke AFL with 1:1 atrioventricular conduction (AVC) to cause hemodynamic collapse. We elucidated the characteristics of patients with AFL exhibiting spontaneous 1:1 AVC. Fifteen patients (1:1 AFL group; 11 males, 52.4 ± 13.7 years old) who documented AFL with 1:1 AVC were enrolled and compared to 153 patients without 1:1 AVC (Control group; 137 males, 68.9 ± 11.2 years old). AFL cycle length during maximum AVC was significantly longer in the 1:1 AFL group than in the control group (274.7 ± 37.0 vs. 216.2 ± 25.6 ms, p < 0.001). Among 1:1 AVC group, 9 patients had AADs, and AFL cycle length was significantly longer during 1:1 AVC as compared with 2:1 AVC documented the other day (284.4 ± 41.3 vs. 233.3 ± 26.0 ms, p < 0.001), suggesting enhancement effect of the AADs during 1:1 AVC. Remaining 6 patients who did not take AADs, 2 patients showed enlargement of the tricuspid annulus and 3 patients developed 1:1 AVC during exercise. Multivariate analysis revealed that younger age and the use of AADs was independent risk factors for the development of 1:1 AFL group. Prolonged AFL cycle length associated with the class Ia/Ic AAD use, slower heart rate during sinus rhythm and younger age were important risk factors for the development of 1:1 AVC during AFL.

Localization of Late Gadolinium Enhancement and Its Association with Ventricular Tachycardia in Patients with Cardiac Sarcoidosis.

Sustained ventricular tachycardia (sVT), leading to sudden cardiac death, is one of the common manifestations in cardiac sarcoidosis (CS). Although late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) has been reported to be associated with sVT, the relationships of its localization to sVT have not been fully evaluated.To evaluate the localization of LGE and its relationships to sVT in patients with CS, we reviewed medical record of consecutive 31 patients with CS who underwent CMR. The localization of LGE was divided into four categories: Left ventricular (LV) septum, LV free wall, right ventricular (RV) septum, and RV free wall. We investigated the association of sVT with localization of LGE and other parameters including serum biomarkers LV ejection fraction on echocardiography and Fluorine-18-fluorodeoxyglucose (FDG) accumulation on positron emission tomography (PET) -CT.Of the studied population, 8 patients (25.8%) were known to present with sVT among 31 CS patients. LGE was observed in the RV free wall in 6 patients with sVT, whereas it was in 5 patients without sVT (75.0% versus 21.7%, P = 0.022). Univariate analysis showed that only LGE in the RV free wall was associated with sVT (odds ratio [OR]: 10.80; 95% confidence interval [CI]: 1.64-70.93, P = 0.013).LGE in the RV free wall was associated with sVT in patients with CS.

Prognostic impact of newly detected atrial fibrillation in patients with hypertrophic cardiomyopathy following cardiac implantable electronic device implantation.

Atrial fibrillation (AF) is the most common arrhythmia in patients with hypertrophic cardiomyopathy (HCM). The present study aimed to investigate the incidence and prognostic impact of newly detected AF after cardiac implantable electronic device (CIED) implantation with HCM patients. Fifty-six patients (33 men, age 57 ± 17 years) with HCM who underwent CIED implantations with no previous history of AF at the time of implantation (ICD n = 46, Pacemaker n = 10) were retrospectively enrolled. During 5.7 ± 3.6 years of follow-up, AF was newly detected in 20 (36%) of 56 patients after the CIED implantation (AF group) and the rest of the patients had no newly detected AF (non-AF group). The presence of mitral regurgitation (HR 8.49; 95% CI 2.29-30.6 P < 0.01) and concomitant NYHA II-IV (HR 3.37; 95% CI 1.30-8.86 P = 0.01) were the independent predictors of newly detected AF. During the follow-up, all patients in the AF group started anticoagulation mean 21 days after detection of AF, and none had a stroke during the follow-up period. The rate of appropriate ICD therapy (log-rank P = 0.95), inappropriate ICD therapy (log-rank P = 0.78), and all-cause death (log-rank P = 0.23) were similar between the two groups. However, the incidence of hospitalizations due to heart failure was higher in the AF group (55% vs. 6% log-rank P < 0.01). In conclusion, the incidence of newly detected AF after CIED implantations in HCM patients was high. The newly detected AF was associated with worsening heart failure and careful follow-up is recommended.

Differential Effectiveness of Landiolol Between Atrial Fibrillation and Atrial Flutter/Atrial Tachycardia Patients With Left Ventricular Dysfunction.

BACKGROUND: Landiolol, an ultra-short acting ß1-selective blocker, is more effective for controlling the heart rate (HR) than digoxin in patients with atrial tachyarrhythmias and left ventricular (LV) dysfunction. The impact of the type of atrial tachyarrhythmias on the effectiveness of landiolol is uncertain. We evaluated the efficacy and safety of landiolol on tachycardiac atrial fibrillation (AF) and tachycardiac atrial flutter/atrial tachycardia (AFl/AT) in patients with reduced LV function. Methods and Results: Seventy-seven patients treated with landiolol were retrospectively analyzed. There were no significant differences in the baseline characteristics between the AF group (n=65) and AFl/AT group (n=12). Despite a higher dosage, the %change in HR from baseline to 12 and 24 h was only -10.2±12.7% and -16.1±19.4% in the AFl/AT group, while it was -28.3±13.2% and -31.3±11.3% in the AF group (P<0.02), respectively. The prevalence of the responders to landiolol treatment was much greater in the AF group than in the AFl/AT group (P<0.001). Alternative treatments such as i.v. amiodarone and electrical cardioversion were required in 83% of the AFl/AT patients. CONCLUSIONS: Landiolol was ineffective in the majority of AFl/AT patients. An alternative management to prevent any worsening of heart failure might be considered in those patients.

Electrocardiographic characteristics in the patients with a persistent left superior vena cava.

A persistent left superior vena cava (PLSVC) is a congenital venous abnormality and is usually asymptomatic and does not cause hemodynamic disturbances. Therefore, it is difficult to identify it by routine examinations in clinical practice. This study aimed to elucidate the electrocardiographic characteristics for the prediction of a PLSVC. Twelve patients (9 males, 56.2 ± 18.3 years) who were diagnosed with a PLSVC were enrolled. The electrocardiographic parameters, including the P-wave duration, axis, and morphology of the P waves, were automatically measured and compared to 150 controls (77 males, 57.3 ± 14.6 years). There were no significant differences in the P-wave duration. Negative or positive/negative P waves in lead III predicted a PLSVC with a sensitivity of 100% and specificity of 81%. The P-wave axis in PLSVC exhibited a significant leftward deviation as compared to the controls (14.8 ± 21.1 vs. 54.0 ± 17.4°, p < 0.001). A receiver operating characteristic curve analysis of the P-wave axis for predicting a PLSVC exhibited an area under curve of 0.93 [CI 95% (0.87-0.98), p < 0.001), and identified a P-wave axis of less than 37.5° to have a 92% sensitivity and 83% specificity in predicting a PLSVC. A negative or positive/negative P-wave morphology in lead III was a useful finding for suggesting the presence of a PLSVC.

Blanking Period Phenomenon After a Second Atrial Fibrillation Ablation Session: The Application and Factors Related to It.

INTRODUCTION: The concept of a 3-month blanking period is widely accepted after the first radiofrequency catheter ablation (RFCA) session for atrial fibrillation (AF). We sought to investigate whether this phenomenon was also observed after a 2nd session, and which factors were related to it. METHODS AND RESULTS: We conducted a prospective observational study including all AF patients who underwent RFCA since 2010. The patients who underwent a second RFCA were followed without any antiarrhythmic drugs. Their clinical background, laboratory data, echocardiographic parameters, ablation procedures, and arrhythmia recurrences were analyzed. Recurrences were classified into early period recurrences (EPRs) and late period recurrences (LPRs) recorded within and after the first 3 months postablation, respectively. Among 925 patients who underwent an initial AF ablation, 2nd sessions were performed in 250 patients, and EPRs and LPRs occurred in 53 (21.2%) and 54 (21.6%) patients, respectively. Although EPRs were an independent predictor of LPRs (hazard ratio [HR], 8.01; 95% confidence interval [CI] 4.03-15.93, P < 0.001), 20 of the patients with EPRs (37.7%) did not experience LPRs, supporting the concept of a blanking period. Among 53 patients with EPRs, the E/E' ratio on echocardiography (HR, 1.156; 95% CI 1.00-1.33, P = 0.04) was an independent predictor of LPRs, while other parameters including the maximum serum C-reactive protein level after the session and the ablation procedure details were not. CONCLUSION: A 3-month blanking period was also applicable after the 2nd AF ablation session. This phenomenon was related to a lower left atrial pressure demonstrated by the E/E' ratio.

Protective Effect of Catheter Ablation of Atrial Fibrillation on the Renal Function in Patients With Hypertrophic Cardiomyopathy.

Atrial fibrillation (AF) is a common arrhythmia in patients with hypertrophic cardiomyopathy (HCM) and is associated with renal function deterioration. The protective effects of catheter ablation (CA) of AF on renal function in patients with HCM remain unsolved. From 2009 to 2020, a total of 169 consecutive patients with HCM and AF (age 70 ± 12, 87 males) were retrospectively evaluated. The estimated glomerular filtration rate (eGFR) was evaluated at the study enrollment or 1 month before the CA and reevaluated 3 and 12 months later. In the 169 patients, 63 underwent CA of AF (ablation group), and the remaining 106 did not (control group). After propensity score matching, 45 pairs were matched. The baseline eGFR was similar between the 2 groups (p = 0.83). During a mean follow-up period of 34 ± 27 months, sinus rhythm was maintained in 36 patients (80%) after 1.7 ± 0.8 ablation procedures. The eGFR significantly decreased from baseline to 3 months (p <0.01) and from baseline to 1 year (p <0.01) in the control group, whereas the eGFR in the ablation group was maintained both from baseline to 3 months (p = 0.94) and from baseline to 1 year (p = 1.00) after the CA. The change in the eGFR between baseline and 12 months was significantly smaller in the ablation group than in the control group (p <0.01). After logistic regression analysis, CA of AF was the independent predictor of an improvement of eGFR (odds ratio 2.81, 95% confidence interval 1.08 to 7.36, p = 0.04). In conclusion, CA of AF had a protective effect on renal function in patients with HCM.

Perioperative coronary artery spasms in patients undergoing catheter ablation of atrial fibrillation.

PURPOSE: Catheter ablation (CA) is an established treatment for atrial fibrillation (AF). Although coronary artery spasms (CAS) during or after ablation procedures have been described as a rare complication in some case reports, the incidence and characteristics of this complication have not been fully elucidated. The present observational study aimed to clarify the CAS in a large number of patients experiencing AF ablation. METHODS: A total of 2913 consecutive patients (male: 78%, mean 66 ± 10 years) who underwent catheter ablation of AF were enrolled. RESULTS: Nine patients (0.31%, mean 66 ± 10 years, 7 males) had transient ST-T elevation (STE). Eight out of the 9 patients had STE in the inferior leads. STE occurred after the transseptal puncture in 7 patients, after the sheath was pulled out of the left atrium in 1, and 2 h after the ablation procedure in 1. Six patients had definite angiographic CAS without any sign of an air embolization on the emergent coronary angiography. In the3 other patients, the STE improved either directly after an infusion of nitroglycerin or spontaneously before the CAG. The patients with CAS had a higher frequency of a smoking habit (89% vs. 53%; P = .04), smaller left atrial diameter (36 ± 6 vs. 40 ± 7; P = .07), and lower CHADS2 score (0.6 ± 0.5 vs. 1.3 ± 1.1; P = .004) than those without. CONCLUSIONS: Although the incidence was rare (0.31%), CAS should be kept in mind as a potentially life-threatening complication throughout an AF ablation procedure especially performed under conscious sedation.

Feasibility and safety of CT-aided pericardiocentesis from a subxiphoid anterior approach by using fluoroscopy in patients with chronic pericardial effusions.

BACKGROUND: Pericardiocentesis is an essential procedure for the diagnosis and treatment of pericardial effusions. The purpose of this study was to evaluate the feasibility and safety of a subxiphoid anterior approach using fluoroscopy aided by a sagittal axis chest computed tomography (CT) view in comparison with an ultrasound-guided apical approach in patients with chronic pericardial effusion. METHODS: Among 72 consecutive patients (68.8 ± 14.4 years old, 52 males) with hemodynamically stable chronic pericardial effusions, a total of 85 procedures were retrospectively analyzed. We divided them into two groups according to the site of the approach for the pericardiocentesis. RESULTS: A subxiphoid anterior approach (n = 53) was performed guided by fluoroscopy. The sagittal axis view of the chest CT was constructed to determine the puncture angle and direction for the subxiphoid anterior approach. An apical approach (n = 32) was performed by ultrasound guidance. The success rates of the anterior and apical approaches were 98.1% and 93.8%, respectively. There were two cases with cardiac perforations in the apical approach group, while no cases developed perforations in the subxiphoid anterior approach group. CONCLUSION: The subxiphoid anterior approach for pericardiocentesis was feasible and safe for managing chronic pericardial effusions. A reconstruction of the sagittal axis view of the chest CT imaging was helpful to identify the direction and depth to access the pericardial space from the subxiphoid puncture site before the pericardiocentesis using the lateral fluoroscopic view.

Improvement in Quality of Life via Catheter Ablation for Atrial Fibrillation in Patients Undergoing Hemodialysis Therapy.

Background: Atrial fibrillation (AF) is the most common arrhythmia in patients undergoing hemodialysis (HD); AF lowers quality of life (QoL) and increases the risk of dialysis-related complications. The present study aimed to evaluate the effectiveness of AF ablation on the QoL in patients undergoing HD. Methods: Nineteen patients undergoing HD (14 men, age 68 ± 8 years; 15 with paroxysmal AF) who underwent catheter ablation (CA) of AF were enrolled in the study. The Kidney Disease Quality of Life Short Form (KDQOL-SF) was assessed to evaluate the QoL of the HD patients at baseline and 6 months after the ablation. Ablation outcomes and procedural complications were evaluated and compared to those of 1053 consecutive non-HD patients who underwent AF ablation. Results: The KDQOL-SF of the HD patients 6 months after the ablation showed an improvement in physical functioning (54 ± 23 to 68 ± 28, P < 0.01), general health perceptions (38 ± 17 to 48 ± 15, P < 0.01), and symptoms/problems (75 ± 21 to 84 ± 13, P = 0.02), compared to baseline. For intradialytic symptoms, dyspnea during HD significantly improved after the CA in the HD patients without AF recurrence (43% to 7%, P = 0.04), whereas the atrial tachyarrhythmias and hypotension during HD remained unchanged. During the follow-up period of 17 ± 13 months after the last procedure, the incidence of being arrhythmia-free was similar (HD patients, 79% vs non-HD patients, 86%, log-rank P = 0.82). No life-threatening complications occurred in any of the patients. Conclusions: CA of AF improves QoL in patients undergoing chronic HD therapy.

Contexte: La fibrillation auriculaire (FA), la forme d'arythmie la plus fréquente chez les patients sous hémodialyse (HD), entraîne une diminution de la qualité de vie (QdV) et une augmentation des risques de complications liées à la dialyse. La présente étude visait à évaluer l'effet de l'ablation de la FA sur la QdV des patients sous HD. Méthodologie: Dix-neuf patients sous HD (âgés de 68 ± 8 ans, dont 14 étaient des hommes et 15 étaient atteints de FA paroxystique) ayant subi une ablation par cathéter de la FA ont été admis dans l'étude. Le questionnaire KDQOL-SF (Kidney Disease Quality of Life Short Form) a été utilisé pour évaluer la QdV des patients sous HD avant l'intervention et six mois après l'ablation. L'issue de l'ablation et les complications liées à l'intervention ont été évaluées et comparées à celles de 1 053 patients consécutifs n'étant pas hémodialysés et ayant subi une ablation de la FA. Résultats: La comparaison des résultats initiaux au KDQOL-SF des patients hémodialysés avec les résultats obtenus six mois après l'ablation a montré des améliorations de la fonction physique (de 54 ± 23 à 68 ± 28, p < 0,01), de la perception de l'état de santé global (de 38 ± 17 à 48 ± 15, p < 0,01), et des symptômes/problèmes de santé (de 75 ± 21 à 84 ± 13, p = 0,02). En ce qui concerne les symptômes survenant lors des séances d'HD, une amélioration significative de la dyspnée a été observée après l'ablation par cathéter chez les patients sous HD sans récurrence de la FA (de 43 % à 7 %, p = 0,04), alors qu'aucun changement n'a été constaté pour les tachyarythmies auriculaires et l'hypotension. Durant la période de suivi de 17 ± 13 mois après la dernière intervention, le nombre de patients sans arythmie était comparable dans les deux groupes (79 % chez les patients hémodialysés et 86 % chez les patients non hémodialysés, test du log-rank = 0,82). Aucun patient n'a subi de complication menaçant le pronostic vital. Conclusions: L'ablation par cathéter de la FA permet d'améliorer la QdV des patients qui subissent un traitement par HD de longue durée.

Metal interference alert guided septal approach with 3 catheter positions on intracardiac echocardiography for a near-zero fluoroscopy catheter ablation of atrial fibrillation.

BACKGROUND: Attempting to minimize radiation exposure during catheter ablation of atrial fibrillation (AF) for patients, operators and medical staffs should be performed. This study aimed to investigate the feasibility and safety of a metal interference alert guided septal approach using 3 intracardiac echocardiography viewing positions for near-zero fluoroscopy AF ablation procedures. METHODS/RESULTS: A total of 668 procedures among 608 consecutive patients with AF (67.2 ± 7.3 years, 408 males) who underwent catheter ablation were retrospectively evaluated and divided into 2 groups, near-zero group (n = 42) and conventional group (n = 595). In the near-zero group, a metal interference alert guided septal approach with 3 different catheter intracardiac echocardiography positions to minimize the fluoroscopy time was applied, and a left atrial access with 2 long sheaths from a single septal puncture without fluoroscopy was successfully achieved in 41 out of 42 cases. The total fluoroscopy time was significantly shorter in the near-zero group than that in the conventional group (0.5 ± 2.0 vs. 21.4 ± 12.9 min p < 0.0001). The total procedure time and time to the septal puncture were both significantly longer in the near-zero group than those in the conventional group (131.4 ± 40.2 vs. 116.6 ± 46.4p = 0.0453, 31.6 ± 9.2 vs. 19.9 ± 10.2 min, p < 0.0001), The ablation time did not differ between the 2 groups (Near-zero: 99.8 ± 41.0 vs. Conventional: 96.8 ± 44.3 min, p = 0.6663). There were no significant differences in the complication rate between the 2 groups (Near-zero: 0 vs. Conventional 14 case, p = 0.6151). CONCLUSION: A metal interference alert guided septal approach using 3 intracardiac echocardiography viewing positions was feasible and safe for a near-zero fluoroscopy catheter ablation of AF.

Successful Prophylactic Endovascular Therapy for a Rapidly Expanding Hepatic Arterial Aneurysm in a Patient with Vascular Ehlers-Danlos Syndrome.

Vascular Ehlers-Danlos syndrome (vEDS) causes fatal vascular complications due to vascular fragility. However, invasive therapeutic procedures are generally avoided except in emergencies. We report a case of vEDS presenting with rapid expansion of a hepatic arterial aneurysm successfully treated using prophylactic endovascular therapy. A 43-year-old woman with vEDS confirmed by genetic testing was hospitalized for a symptomatic hepatic arterial aneurysm that expanded rapidly within a week. Prophylactic coil embolization was then successfully performed. Although the general applicability of this approach cannot be determined, prophylactic endovascular therapy can clearly be an option for arterial aneurysms at high risk of rupture.

The characteristics and efficacy of catheter ablation of focal atrial tachycardia arising from an epicardial site.

BACKGROUND: Although epicardial structures around the atrium such as adipose tissue possess arrhythmogenicity, little is known about atrial tachycardias (ATs) originating from epicardial sites (Epi-ATs). This study aimed to elucidate the prevalence, characteristics, and outcome after radiofrequency catheter ablation (RFCA) of Epi-ATs and to reveal the association between Epi-ATs and the epicardial structures. METHODS: The electrocardiographic, electrophysiologic, and anatomical properties and results of RFCA were analyzed in 42 patients with a total of 49 ectopic ATs. RESULTS: Six Epi-ATs (12%) were observed in six patients (14%). Four of six were respiratory cycle-dependent ATs and one was a swallowing-induced AT. The Epi-AT origins were adjacent to a pulmonary vein (five cases) and vein of Marshall (one case). A Valsalva maneuver or atropine infusion to define the arrhythmia mechanism affected the appearance of the Epi-ATs. The congruity rate between epicardial adipose tissue and the AT origin was significantly higher (100% vs. 44%, p = .045), and the epicardial adipose tissue volume of the atrium was significantly larger (104.1 vs. 64.6 ml, p = .04) in the Epi-AT group. Endocardial RFCA targeting the AT foci resulted in acute success in five of five cases. However, electrical isolation including of the AT foci resulted in acute failures (two of three cases) or a recurrence (one of one case). CONCLUSIONS: Six Epi-ATs were associated with thoracic veins and epicardial arrhythmogenic structures. The main cause provoking the Epi-ATs was associated with autonomic nerve activity.

Impact of the COVID-19 Pandemic on ST-elevation Myocardial Infarction from a Single-center Experience in Tokyo.

Objective The coronavirus disease 2019 (COVID-19) pandemic has had a significant impact on global healthcare systems. Some studies have reported the negative impact of COVID-19 on ST-elevation myocardial infarction (STEMI) patients; however, the impact in Japan remains unclear. This study investigated the impact of the COVID-19 pandemic on STEMI patients admitted to an academic tertiary-care center in Tokyo, Japan. Methods In this retrospective, observational, cohort study, we included 398 consecutive patients who were admitted to our institute from January 1, 2018, to March 10, 2021, and compared the incidence of hospitalization, clinical characteristics, time course, management, and outcomes before and after March 11, 2020, the date when the World Health Organization declared COVID-19 a pandemic. Results There was a 10.7% reduction in hospitalization of STEMI patients during the COVID-19 pandemic compared with that in the previous year (117 vs. 131 cases). During the COVID-19 pandemic, the incidence of late presentation was significantly higher (26.5% vs. 12.1%, p<0.001), and the onset-to-door [241 (IQR: 70-926) vs. 128 (IQR: 66-493) minutes, p=0.028] and door-to-balloon [72 (IQR: 61-128) vs. 60 (IQR: 43-90) min, p<0.001] times were significantly longer than in the previous year. Furthermore, the in-hospital mortality was higher, but the difference was not significant (9.4% vs. 5.0%, p=0.098). Conclusion The COVID-19 pandemic significantly impacted STEMI patients in Tokyo and resulted in a slight decrease in hospitalization, a significant increase in late presentation and treatment delays, and a slight but nonsignificant increase in mortality. In the COVID-19 era, the acute management system for STEMI in Japan must be reviewed.

Myocardial ultrastructure can augment genetic testing for sporadic dilated cardiomyopathy with initial heart failure.

AIMS: The aim of the present study was to consider whether the ultrastructural features of cardiomyocytes in dilated cardiomyopathy can be used to guide genetic testing. METHODS AND RESULTS: Endomyocardial biopsy and whole-exome sequencing were performed in 32 consecutive sporadic dilated cardiomyopathy patients [51.0 (40.0-64.0) years, 75% men] in initial phases of decompensated heart failure. The predicted pathogenicity of ultrarare (minor allele frequency ≤0.0005), non-synonymous variants was determined using the American College of Medical Genetics guidelines. Focusing on 75 cardiomyopathy-susceptibility and 41 arrhythmia-susceptibility genes, we identified 404 gene variants, of which 15 were considered pathogenic or likely pathogenic in 14 patients (44% of 32). There were five sarcomeric gene variants (29% of 17 variants) found in five patients (16% of 32), involving a variant of MYBPC3 and four variants of TTN. A patient with an MYBPC3 variant showed disorganized sarcomeres, three patients with TTN variants located in the region encoding the A-band domain showed sparse sarcomeres, and a patient with a TTN variant in encoding the I-band domain showed disrupted sarcomeres. The distribution of diffuse myofilament lysis depended on the causal genes; three patients with the same TMEM43 variant had diffuse myofilament lysis near nuclei (P = 0.011), while two patients with different DSP variants had lysis in the peripheral areas of cardiomyocytes (P = 0.033). CONCLUSIONS: Derangement patterns of myofilament and subcellular distribution of myofilament lysis might implicate causal genes. Large-scale studies are required to confirm whether these ultrastructural findings are related to the causative genes.

Esophageal pressure monitoring for airway management during catheter ablation of atrial fibrillation.

BACKGROUND: Respiratory management during catheter ablation of atrial fibrillation (AF) is important for the efficacy and safety of the procedure. Obstructive apnea due to an upper airway obstruction might cause serious complications including air embolisms and cardiac tamponade. However, real time monitoring of upper airway obstructions during catheter ablation has not been established. The purpose of the present study was to evaluate esophageal pressure monitoring for respiratory management during catheter ablation of AF. METHODS AND RESULTS: Twenty-four consecutive patients (20 men and 4 women; mean age, 61 ± 13 years) with AF who underwent esophageal pressure monitoring during catheter ablation of AF were retrospectively analyzed. The patients were divided into 2 groups. One was the obstructive apnea (OA) group (n = 17), which required airway management tools including nasal airways and/or non-invasive positive airway pressure (NPPV) and the other was the control group (n = 7), which did not require airway management. Esophageal pressure measurements were obtained in all patients, and the OA group exhibited a substantial negative esophageal pressure as compared to the control group (-41.48 ± 19.58 vs. -12.42 ± 5.77 mmHg, p < 0.001). Airway management in the OA group immediately improved the negative esophageal pressure and returned to a normal range (-41.48 ± 19.58 vs. -16 ± 8.1 mmHg, 0 < 0.001) along with a recovery from desaturation. CONCLUSIONS: Esophageal pressure monitoring was a simple and effective method for the evaluation and management of obstructive apnea during AF catheter ablation.