Treatment Strategy for Fatal Arrhythmias in Ebstein's Anomaly Combined With Leadless Pacemaker and S-ICD Implantations.

The management of heart rhythm disorders in patients with adult congenital heart disease and limited vascular access is challenging. We present the case of a 38-year-old woman with Ebstein's anomaly who underwent implantation of a combination of a leadless pacemaker and a subcutaneous implantable cardioverter-defibrillator to manage fatal arrhythmias. (Level of Difficulty: Intermediate.).

Impact of corticosteroid use on the clinical response and prognosis in patients with cardiac sarcoidosis who underwent an upgrade to cardiac resynchronization therapy.

Background: Corticosteroids are widely used in patients with cardiac sarcoidosis (CS). In addition, upgrading to cardiac resynchronization therapy (CRT) is sometimes needed. This study aimed to investigate the impact of corticosteroid use on the clinical outcomes following CRT upgrades. Methods: A total of 48 consecutive patients with non-ischemic cardiomyopathies who underwent CRT upgrades were retrospectively reviewed and divided into three groups: group 1 included CS patients taking corticosteroids before the CRT upgrade (n = 7), group 2, CS patients not taking corticosteroids before the CRT upgrade (n = 10), and group 3, non-CS patients (n = 31). The echocardiographic response, heart failure hospitalizations, and cardiovascular deaths were evaluated. Results: The baseline characteristics during CRT upgrades exhibited no significant differences in the echocardiographic data between the three groups. After the CRT upgrade, responses regarding the ejection fraction (EF) and end-systolic volume (ESV) were significantly lower in CS patients than non-CS patients (ΔEF: group 1, 6.7% vs. group 2, 7.7% vs. group 3, 13.6%; p = .039, ΔESV: 3.0 ml vs. -12.7 ml vs. -37.2 ml; p = .008). The rate of an echocardiographic response was lowest in group 1 (29%). There were, however, no significant differences in the cumulative freedom from a composite outcome among the three groups (p = .19). No cardiovascular deaths occurred in group 1. Conclusion: The echocardiographic response to an upgrade to CRT and the long-term prognosis in patients with CS should be carefully evaluated because of the complex etiologies and impact of immunosuppressive therapy.

Factors related to the skin thickness of cardiovascular implantable electronic device pockets.

INTRODUCTION: The skin overlying cardiovascular implantable electronic devices (CIEDs) sometimes becomes very thin after implantations, which could cause a device erosion. The factors related to the skin thickness of device pockets have not been elucidated. This study aimed to evaluate the skin thickness of CIED pockets and search for the factors associated with the thickness. METHODS: Seventeen skin thickness points around the CIED pocket were measured through ultrasonography in each patient. RESULTS: A total of 101 patients (76 ± 11 years, 26 female) were enrolled. The median duration from the implantation to the examination was 95 months (quartile: 52.5-147.5). The median skin thickness overlying the device was 4.1 mm (3.3-5.9). Patients with heart failure and malignancy had thinner skin overlying the CIED than those without. A significant correlation existed between skin thickness and body mass index (BMI), hemoglobin, serum creatinine, estimated glomerular filtration rate (eGFR), and left ventricular ejection fraction. In contrast, age, gender, and device size did not exhibit a significant correlation with skin thickness. A multivariate logistic regression analysis revealed that chronic heart failure and a decrease in the eGFR and BMI were independent predictive factors of "very thin (≦3.3 mm)" skin of the CIED pocket late after an implantation. CONCLUSION: Aside from a low BMI, the comorbidities (low hemoglobin, heart failure, and renal dysfunction) had a stronger impact on the skin thickness overlying the device than the device size. A careful observation of the device pocket should be performed in patients with those risk factors.

Successful catheter ablation of postoperative atrial tachycardia with conduction disturbances: Assessment by late-gadolinium enhancement magnetic resonance imaging and high-resolution electro-anatomical mapping.

Atrial tachycardia (AT) in the right atrium often occurs following open-heart surgery. Catheter ablation for these AT is challenging and can lead to unintended conduction block. We performed late-gadolinium enhancement magnetic resonance imaging (LGE-MRI) prior to catheter ablation and predicted wavefront propagation during SR as well as the slow conduction zone during tachycardia. LGE-MRI may assist predicting the conduction disturbance and reducing the risk of unexpected sinus exit block.

Electrophysiological and Pathological Impact of Medium-Dose External Carbon Ion and Proton Beam Radiation on the Left Ventricle in an Animal Model.

Background Medium-dose (25 gray) x-ray radiation therapy has recently been performed on patients with refractory ventricular tachyarrhythmias. Unlike x-ray, carbon ion and proton beam radiation can deliver most of their energy to the target tissues. This study investigated the electrophysiological and pathological changes caused by medium-dose carbon ion and proton beam radiation in the left ventricle (LV). Methods and Results External beam radiation in the whole LV was performed in 32 rabbits. A total of 9 rabbits were not irradiated (control). At the 3-month or 6-month follow-up, the animals underwent an open-chest electrophysiological study and were euthanized for histological analyses. No acute death occurred. Significant LV dysfunction was not seen. The surface ECG revealed a significant reduction in the P and QRS wave voltages in the radiation groups. The electrophysiological study showed that the local conduction times in each LV site were significantly longer and that the local LV bipolar voltages were significantly lower in the radiation groups than in the control rabbits. Histologically, apoptosis, fibrotic changes, and a decrease in the expression of the connexin 43 protein were seen in the LV myocardium. These changes were obvious at 3 months, and the effects were sustained 6 months after radiation. No histological changes were seen in the coronary artery and esophagus, but partial radiation pneumonitis was observed. Conclusions Medium-dose carbon ion and proton beam radiation in the whole LV resulted in a significant electrophysiological disturbance and pathological changes in the myocardium. Radiation of the arrhythmogenic substrate would modify the electrical status and potentially induce the antiarrhythmic effect.

Lesion characteristics between cryoballoon ablation and radiofrequency ablation with a contact force-sensing catheter: Late-gadolinium enhancement magnetic resonance imaging assessment.

BACKGROUND: Pulmonary vein isolation (PVI) lesions after cryoballoon ablation (CBA) are characterized as a wider and more continuous than that after conventional radiofrequency catheter ablation (RFCA) without the contact force (CF)-sensing technology. However, the impact on the lesion characteristics of ablation with a CF-sensing catheter has not been well discussed. We sought to assess the lesions using late-gadolinium enhancement magnetic resonance imaging (LGE-MRI) and to compare the differences between the two groups (CB group vs. RF group). METHODS: A total of 30 consecutive patients who underwent PVI were enrolled (CB group, 18; RF group, 12). The RF applications were delivered with a target lesion size index (LSI) of 5. The PVI lesions were assessed by LGE-MRI 3 months after the PVI. The region around the PV was divided into eight segments: roof, anterior-superior, anterior carina, anterior inferior, bottom, posterior inferior, posterior carina, and posterior superior segment. The lesion width and visual gap of each segment were compared between the two groups. The visual gaps were defined as no-enhancement site of >4 mm. RESULTS: The mean LSI was 4.7 ± 0.7. The lesion width was significantly wider but the visual gaps were more frequently documented at the bottom segment of right PV in the CBA group (lesion width: 8.1 ± 2.2 vs. 6.3 ± 2.2 mm; p = .032; visual gap at the bottom segment or right PV: 39% vs. 0%; p = .016). CONCLUSIONS: The PVI lesion was wider after CBA, while the visual gaps were fewer after RFCA with a CF-sensing catheter.

Lesion distribution after cryoballoon ablation and hotballoon ablation: Late-gadolinium enhancement magnetic resonance imaging analysis.

INTRODUCTION: Pulmonary vein isolation (PVI) lesions after cryoballoon ablation (CBA) are wide and continuous, however, the distribution can depend on the pulmonary vein (PV) size. We sought to assess the relationship between the lesion distribution and PV size after CBA and hotballoon ablation (HBA). METHODS AND RESULTS: A total of 80 consecutive patients who underwent PVI were enrolled (40 with CBA). The lesions were visualized by late-gadolinium enhancement magnetic resonance imaging. The lesion width, lesion gaps, and distance from the PV ostium (PVos) to distal lesion edge (DLE) were assessed. If the DLE extended inside the PV, the value was expressed as a negative value. Although the lesion width was significantly wider in the CB group (7.8 ± 2.0 vs 4.9 ± 1.0 mm, P < .001), the number of lesion gaps was significantly less in the HB group (2.9 ± 2.4 vs 1.3 ± 1.4 gaps, P = .001). The distance from the PVos to DLE was a negative value in both groups, but the impact was significantly greater (-1.5 ± 1.8 vs -0.2 ± 1.2 mm, P < .001) and negatively correlated with PV size in the CB group, but not in HB group (r = -0.27, P = .007). The AF recurrence 12 months after the procedure did not differ (5 [12.5%] of 40 in the CB group vs 4 [10%] of 40 in the HB group, P = .695). CONCLUSIONS: The PVI lesions after HBA were characterized by (a) narrower, but (b) more continuous, (c) smaller lesion inside the PV, and (d) irrespective of PV size as compared to that after CBA.

Techniques for reducing air bubble intrusion into the left atrium during radiofrequency catheter and cryoballoon ablation procedures: An ex vivo study with a high-resolution camera.

BACKGROUND: Air embolisms are serious complications during catheter ablation procedures. OBJECTIVES: The aims of the present study were to determine when air bubbles enter the left atrium (LA) during catheter ablation procedures and to identify techniques that reduce air bubble intrusion. METHODS: An ex vivo study was performed to monitor air bubbles using a silicone heart model and a high-resolution camera. In total, 280 radiofrequency catheter and cryoballoon ablation processes were tested. RESULTS: Small and large air bubbles were often observed during catheter ablation processes. Many small air bubbles arose during sheath flushing at fast speeds (15 mL/2 s) (median bubble number [quartiles]: 35 [20-53] for SL0, 35 [23-44] for Agilis, and 98 [91-100] for FlexCath) and during initial cryoballoon inflation/freezing/deflation (34 [22-47]). Large (≥1.5 mm) air bubbles were observed during Lasso catheter insertion (1 [0-1]), cryoballoon insertion (2 [1-2]), and initial inflation/freezing/deflation (1 [1-3]). Massive air bubbles were observed during Optima catheter insertion into the sheath using an inserter (10 [2-15]). Sheath flushing at slow speeds (15 mL/5 s) significantly reduced the number of air bubbles. Before cryoballoon insertion, temporary balloon inflation and air bubble removal from the inflated surface were most effective in reducing air bubble intrusions. Optima catheter insertion without an inserter significantly reduced large air bubble intrusion. CONCLUSION: Air bubbles entered the LA at specific times. Techniques such as sheath flushing at slow speeds, temporary cryoballoon inflation before insertion, inserting the Optima catheter without an inserter, and avoidance of negative pressure in the LA could reduce air bubble intrusion.

ECG-based 4D-dose reconstruction of cardiac arrhythmia ablation with carbon ion beams: application in a porcine model.

Noninvasive ablation of cardiac arrhythmia by scanned particle radiotherapy is highly promising, but especially challenging due to cardiac and respiratory motion. Irradiations for catheter-free ablation in intact pigs were carried out at the GSI Helmholtz Center in Darmstadt using scanned carbon ions. Here, we present real-time electrocardiogram (ECG) data to estimate time-resolved (4D) delivered dose. For 11 animals, surface ECGs and temporal structure of beam delivery were acquired during irradiation. R waves were automatically detected from surface ECGs. Pre-treatment ECG-triggered 4D-CT phases were synchronized to the R-R interval. 4D-dose calculation was performed using GSI's in-house 4D treatment planning system. Resulting dose distributions were assessed with respect to coverage (D95 and V95), heterogeneity (HI = D5-D95) and normal tissue exposure. Final results shown here were performed offline, but first calculations were started shortly after irradiation The D95 for TV and PTV was above 95% for 10 and 8 out of 11 animals, respectively. HI was reduced for PTV versus TV volumes, especially for some of the animals targeted at the atrioventricular junction, indicating residual interplay effects due to cardiac motion. Risk structure exposure was comparable to static and 4D treatment planning simulations. ECG-based 4D-dose reconstruction is technically feasible in a patient treatment-like setting. Further development of the presented approach, such as real-time dose calculation, may contribute to safe, successful treatments using scanned ion beams for cardiac arrhythmia ablation.

External Arrhythmia Ablation Using Photon Beams: Ablation of the Atrioventricular Junction in an Intact Animal Model.

BACKGROUND: This study sought to investigate external photon beam radiation for catheter-free ablation of the atrioventricular junction in intact pigs. METHODS AND RESULTS: Ten pigs were randomized to either sham irradiation or irradiation of the atrioventricular junction (55, 50, 40, and 25 Gy). Animals underwent baseline electrophysiological evaluation, cardiac gated multi-row computed tomographic imaging for beam delivery planning, and intensity-modulated radiation therapy. Doses to the coronary arteries were optimized. Invasive follow-up was conducted ≤4 months after the irradiation. A mean volume of 2.5±0.5 mL was irradiated with target dose. The mean follow-up length after irradiation was 124.8±30.8 days. Out of 7 irradiated animals, complete atrioventricular block was achieved in 6 animals of all 4 dose groups (86%). Using the same targeting margins, ablation lesion size notably increased with the delivered dose because of volumetric effects of isodose lines around the target volume. The mean macroscopically calculated atrial lesion volume for all 4 dose groups was 3.8±1.1 mL, lesions extended anteriorly into the interventricular septum. No short-term side effects were observed. No damage was observed in the tissues of the esophagus, phrenic nerves, or trachea. However, histology revealed in-field beam effects outside of the target volume. CONCLUSIONS: Single-fraction doses as low as 25 Gy caused a lesion with interruption of cardiac impulse propagation using this respective target volume. With doses of ≤55 Gy, maximal point-doses to coronary arteries could be kept <7Gy, but target conformity of lesions was not fully achieved using this approach.

Feasibility Study on Cardiac Arrhythmia Ablation Using High-Energy Heavy Ion Beams.

High-energy ion beams are successfully used in cancer therapy and precisely deliver high doses of ionizing radiation to small deep-seated target volumes. A similar noninvasive treatment modality for cardiac arrhythmias was tested here. This study used high-energy carbon ions for ablation of cardiac tissue in pigs. Doses of 25, 40, and 55 Gy were applied in forced-breath-hold to the atrioventricular junction, left atrial pulmonary vein junction, and freewall left ventricle of intact animals. Procedural success was tracked by (1.) in-beam positron-emission tomography (PET) imaging; (2.) intracardiac voltage mapping with visible lesion on ultrasound; (3.) lesion outcomes in pathohistolgy. High doses (40-55 Gy) caused slowing and interruption of cardiac impulse propagation. Target fibrosis was the main mediator of the ablation effect. In irradiated tissue, apoptosis was present after 3, but not 6 months. Our study shows feasibility to use high-energy ion beams for creation of cardiac lesions that chronically interrupt cardiac conduction.

Impact of freezing time and balloon size on the thermodynamics and isolation efficacy during pulmonary vein isolation using the second generation cryoballoon.

BACKGROUND: The differences in ablation characteristics of freezing time and balloon size using second generation cryoballoon are still unknown. METHODS AND RESULTS: Twenty-six dogs underwent pulmonary vein (PV) isolation. Balloon and tissue temperatures (left atrial-PV junction, phrenic nerve, and internal esophagus) were monitored. The ablation duration was randomized to either 3 or 4 minutes, which did not show significant differences in temperature profiles, PV isolation success rate, complications, or histological changes. Twenty dogs underwent cryoablation using 28-mm cryoballoon, 6 dogs were done using the 23-mm cryoballoon. Positioning of the 23-mm cryoballoon was more distal in the PV, which resulted in better PV occlusion. Temperature profiles showed lower temperatures in the 23-mm cryoballoon than in the 28-mm cryoballoon (inner balloon, median [range]: -51.5 [-66.0 to -31.0] versus -43.0 [-64.0 to -26.0]°C, P<0.001; balloon surface: -43.0 [-60.0 to -15.8] versus -6.5 [-46.2 to 28.9]°C, P<0.001; left atrial-PV junction: -6.7 [-20.0 to 21.4] versus 15.8 [-14.4 to 35.1]°C, P<0.001), and trended toward a higher PV isolation success rate in the 23-mm cryoballoon. Histologically, deeper extensions of ablative lesions into the PV were seen with 23-mm cryoballoon, and larger ablative lesions were seen in the left atrial antrum using 28-mm cryoballoon. CONCLUSIONS: The efficacy of 3-minute ablation was not significantly different from 4-minute ablation in dogs. The 23-mm cryoballoon had a greater cooling effect than the 28-mm cryoballoon for small PVs, but showed narrower ablative lesions in the left atrial antrum.

Atrioventricular node ablation in Langendorff-perfused porcine hearts using carbon ion particle therapy: methods and an in vivo feasibility investigation for catheter-free ablation of cardiac arrhythmias.

BACKGROUND: Particle therapy, with heavy ions such as carbon-12 ((12)C), delivered to arrhythmogenic locations of the heart could be a promising new means for catheter-free ablation. As a first investigation, we tested the feasibility of in vivo atrioventricular node ablation, in Langendorff-perfused porcine hearts, using a scanned 12C beam. METHODS AND RESULTS: Intact hearts were explanted from 4 (30-40 kg) pigs and were perfused in a Langendorff organ bath. Computed tomographic scans (1 mm voxel and slice spacing) were acquired and (12)C ion beam treatment planning (optimal accelerator energies, beam positions, and particle numbers) for atrioventricular node ablation was conducted. Orthogonal x-rays with matching of 4 implanted clips were used for positioning. Ten Gray treatment plans were repeatedly administered, using pencil beam scanning. After delivery, positron emission tomography-computed tomographic scans for detection of ß(+) ((11)C) activity were obtained. A (12)C beam with a full width at half maximum of 10 mm was delivered to the atrioventricular node. Delivery of 130 Gy caused disturbance of atrioventricular conduction with transition into complete heart block after 160 Gy. Positron emission computed tomography demonstrated dose delivery into the intended area. Application did not induce arrhythmias. Macroscopic inspection did not reveal damage to myocardium. Immunostaining revealed strong γH2AX signals in the target region, whereas no γH2AX signals were detected in the unirradiated control heart. CONCLUSIONS: This is the first report of the application of a (12)C beam for ablation of cardiac tissue to treat arrhythmias. Catheter-free ablation using 12C beams is feasible and merits exploration in intact animal studies as an energy source for arrhythmia elimination.

Spatial and time-course thermodynamics during pulmonary vein isolation using the second-generation cryoballoon in a canine in vivo model.

BACKGROUND: Thermodynamics in the left atrium-pulmonary vein (PV) junction, phrenic nerve, and esophagus during PV isolation (PVI) using the second-generation cryoballoon are not known. METHODS AND RESULTS: Twenty dogs underwent PVI using second-generation cryoballoon. Ablations were performed for ≤2 deliveries based on PVI without a bonus freeze. Inner balloon, balloon surface, and tissue temperatures were monitored during cryoablation. The tissue thermocouples were placed on the epicardial surface of the left atrium-PV junction, as well as on the phrenic nerve and within the esophagus. A total of 259 cryoballoon and 229 tissue tissue thermocouples profiles during 53 cryoablations of 40 PVs were analyzed. Acutely, PVI was achieved in 36 of 40 PVs (90%). Conductive tissue cooling spread radially from the balloon-left atrium-PV contact point. The lowest tissue temperatures were dependent on the distance of the tissue thermocouples to the balloon surface (r=0.85; P<0.001). In addition, blood flow leaks around the balloon had a warming effect on the balloon and tissue temperature profiles. Chronic isolation (mean, 48±16 days) was achieved in 27 of 36 PVs (75%). In 8 of 9 acutely isolated but with chronic reconnection PVs, the blood flow leak location was concordant with chronic reconnection gap. Although only 1 esophageal ulcerated lesion was observed, neither phrenic nerve palsy nor severe PV stenosis was seen in any dogs. CONCLUSIONS: Variance in tissue thermodynamics during cryothermal ablation depends on the distance from balloon and peri-balloon blood flow leaks. This information may be useful for successful PVI without severe complications.

Dilated phase of hypertrophic cardiomyopathy caused by Fabry disease with atrial flutter and ventricular tachycardia.

We describe a case of a 60-year-old male with dilated phase of hypertrophic cardiomyopathy caused by Fabry disease. He was diagnosed to have a cardiac variant of Fabry disease by an enzyme assay and a right ventricular endomyocardial biopsy which revealed specific features of this disease and cardiac involvement was the sole manifestation. He has developed dilated cardiomyopathy with sustained atrial flutter and frequent non-sustained ventricular tachycardia requiring isthmus ablation and cardiac resynchronization therapy with defibrillator.