Electrophysiological and Histopathological Characteristics of Ventricular Tachycardia Associated With Primary Cardiac Tumors.

BACKGROUND: Ventricular tachycardia (VT) associated with primary cardiac tumors (PCTs) originating from the ventricles is rare, but lethal, in young patients. OBJECTIVES: This study aimed to clarify the mechanisms underlying primary cardiac tumor-related ventricular tachycardia (PCT-VT) and establish a therapeutic strategy for this form of VT. METHODS: Among 67 patients who underwent surgery for VT at our institute between 1981 and 2020, 4 patients aged 1 to 34 years, including 3 males, showed PCT-VT (fibroma, 2; lipoma, 1; and hamartoma, 1), which was investigated using a combination of intraoperative electroanatomical mapping and histopathological studies. RESULTS: All 4 patients developed electrical storms of sustained VTs refractory to multiple drugs and repetitive endocardial ablations. The VT mechanism was re-entry, and intraoperative electroanatomical mapping showed a centrifugal activation pattern originating from the border between the tumor and healthy myocardium, where fractionated potentials were detected during sinus rhythm. Histopathological studies of serial sections of specimens acquired from these areas revealed tumor infiltration into the surrounding myocardium with cell disorganization, exhibiting myocardial disarray. Several myocardia entrapped in the tumor edges contributed to the development and sustainment of re-entrant VT activation. In the 2 patients in whom complete resection was unfeasible, encircling cryoablation to entirely isolate the unresectable tumor was effective in suppressing VT occurrence. CONCLUSIONS: The mechanism underlying PCT-VT involves re-entry localized at the tumor edges. Myocardial disarray associated with tumor infiltration is a substrate for this form of VT. Cryoablation along the border between the tumor and myocardium is a promising therapeutic option for unresectable PCT-VT.

Surgery for ventricular tachycardia originating from the left ventricular summit.

OBJECTIVES: Ventricular tachycardia (VT) originating from the left ventricular summit region, the most superior region of the left ventricle surrounded by the major coronary arteries and veins, is frequently refractory to pharmacological therapies and endocardial and epicardial catheter ablation. METHODS: Eleven patients with an age from 31 to 79 (median 56) years old, underwent map-guided surgery for left ventricular summit VT. All patients had undergone 1-5 unsuccessful sessions of catheter ablation for incessant VT, preoperatively. Five patients had suffered VT storm and 1 had a history of cardiopulmonary resuscitation. Four patients had implanted with a defibrillator. Epicardium to endocardium transmural cryothermia was applied at the VT origin determined by intraoperative epicardial mapping with electro-anatomical mapping system. Harmonic scalpel was used to remove the epicardial fat and cryothermia was applied directly to the myocardium, avoiding thermal or mechanical injuries to the coronary vessels. Additional endocardial cryothermia at the VT origin was performed by a cryoprobe introduced into the left ventricular cavity through an aortotomy. RESULTS: There was no surgical mortality or long-term mortality related to VT during a median follow-up period of 60 months (interquartile range: 34-82). Five-year freedom from preoperatively documented left ventricular summit VT and non-documented VT was 91% and 73%, respectively. All the patients with postoperative VT underwent successful catheter ablation. Other patients were free from VT during the follow-up period. CONCLUSIONS: Epicardial to endocardial transmural cryothermia at the VT origin guided by intraoperative electro-anatomical mapping with a close collaboration with electrophysiologists was crucial in successful surgery for left ventricular summit VT.

External Validation of the Kumamoto Criteria in Transthyretin Amyloid Cardiomyopathy Screening　- A Retrospective Cohort Study.

Background: The Kumamoto criteria have been proposed as a non-invasive screen for transthyretin amyloid cardiomyopathy. This study assessed the validity of the Kumamoto criteria externally. Methods and Results: The study included 138 patients (median age 73 years; 65% male) who underwent 99 mTc-pyrophosphate (PYP) scintigraphy. Patients were divided into 4 groups according to total scores on the Kumamoto criteria (i.e., 0-3) for the following 3 factors: high-sensitivity cardiac troponin T ≥0.0308 ng/mL, wide (≥120 ms) QRS, and left ventricular posterior wall thickness ≥13.6 mm. The diagnostic performance and positive predictive value (PPV) of the Kumamoto criteria for positive 99 mTc-PYP scintigraphy were validated. Eighteen (13%) patients were positive on 99 mTc-PYP scintigraphy. The Kumamoto criteria had a favorable diagnostic performance (area under the curve 0.808). The PPV for groups with scores of 0, 1, 2, and 3 was 0% (n=0/42), 11% (n=6/57), 21% (n=7/33), and 83% (n=5/6), respectively, which is lower, particularly for those with a score of 2, than in the original Kumamoto cohort. However, the PPV increased after combining the Kumamoto criteria with a history of orthopedic diseases (spinal canal stenosis and/or carpal tunnel syndrome). Conclusions: This study suggests that the Kumamoto criteria have a favorable diagnostic performance; however, the PPV may decrease depending on the study population. Combining the Kumamoto criteria with the presence of orthopedic disease may improve the PPV.

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.

Long-Term Electrocardiographic Follow-Up of a Patient with Light-Chain Cardiac Amyloidosis.

Amyloid light-chain (AL) cardiac amyloidosis can cause restrictive cardiomyopathy, which has a poor prognosis. Although electrocardiography (ECG) is useful for its diagnosis and management, there are few reports on the long-term follow-up of electrocardiographic changes in affected patients. The present patient was a 62-year-old woman who visited our hospital for assessment of palpitations and lower leg edema. A chest radiograph showed cardiac enlargement, and ECG revealed sinus rhythm, first-degree atrioventricular block, low QRS voltage in the limb leads and a pseudo-myocardial infarction pattern in the precordial leads. Echocardiography revealed left ventricular hypertrophy with systolic and diastolic dysfunction. Immunoelectrophoresis demonstrated M-protein (IgGλ), and bone marrow biopsy suggested IgGλ-type plasmacytoma. Myocardial biopsy findings were compatible with cardiac amyloidosis. On the basis of these findings, we diagnosed AL cardiac amyloidosis. Melphalan-prednisolone (MP) therapy was started in conjunction with treatment for non-sustained ventricular tachycardia and congestive heart failure. Two years and 4 months later, the sinus rhythm converted to atrial tachycardia. At a follow-up examination at 4 years and 8 months, right branch block appeared. After that, intraventricular conduction worsened, and the low voltage in the limb leads was not observed. Seven years after diagnosis, she was hospitalized for treatment of pneumonia and heart failure with tachycardia. On the seventh day of hospitalization, heart rhythm changed to atrial stand-still with escaped ventricular rhythm and she died of cardiac arrest. These ECG changes are valuable information regarding the pathophysiological changes that occur in AL cardiac amyloidosis.

Surgical procedure for targeting arrhythmogenic substrates in the treatment of ventricular tachycardia associated with cardiac tumors.

BACKGROUND: Complete tumor resection is a standard strategy in the surgical treatment of ventricular tachycardia (VT) associated with cardiac tumors. Recently, an intraoperative electroanatomic mapping system (CARTO) has enabled surgeons to target the localized arrhythmogenic substrate for partial resection and/or cryoablation in nonresectable cardiac tumors. OBJECTIVE: The purpose of this study was to evaluate the surgical procedures and late outcomes of the treatment of VT associated with cardiac tumors. METHODS: We examined six patients (age 1-65 years) who had undergone surgical treatment of VT associated with cardiac tumors between 2010 and 2016. The 4 pathologies of the cardiac tumors were lipoma 2, fibroma 2, hemangioma 1, and lymphoma 1. Intraoperative epicardial mapping using CARTO was performed in 5 patients(80%). Surgical procedures and long-term outcomes were evaluated. RESULTS: Arrhythmogenic substrates with abnormal electrograms, such as fractionated or late potential, were identified locally or circumferentially beside the tumor in every patient. Complete tumor resection with cryoablation was performed in 3 patients. Two patients underwent partial tumor resection with cryoablation. Cryoablation without tumor resection was performed in 1 patient. No mortality and morbidity occurred. Additional catheter ablation was required in 2 patients to treat occurrence of nonclinical VT and induction of clinical VT during hospital stay. Mean follow-up time was 90 ± 52.5 months. There was no recurrence of clinical VT. CONCLUSION: The outcomes of surgical treatment of VT associated with cardiac tumors were excellent. Intraoperative CARTO mapping was beneficial to eliminate the VT substrates associated with nonresectable cardiac tumors.

Intraoperative cardiac mapping in the treatment of an infant congenital fibroma.

Surgical treatment for ventricular tachycardia associated with congenital cardiac tumors is rare. Intraoperative electroanatomic mapping was performed in a 23-month-old female infant to identify the arrhythmogenic substrate of the epicardium before tumor resection. Verification of the localized abnormal electrocardiogram on the tumor in the treatment of ventricular tachycardia was useful for successful partial resection and cryoablation of the giant fibroma.

Intraoperative electroanatomic mapping.

PURPOSE: An electroanatomic mapping system using an electromagnetic navigation technology constructs a 3-dimensional structure of the heart with high geometric accuracy of the data that provides a precise localization of the substrates of arrhythmias. The system was tested for the feasibility and efficacy in intraoperative mapping. DESCRIPTION: The strength of the magnetic field is measured by a location sensor with three different frequencies generated by a location pad placed beneath the operating table, and the spatial location of the sensor is determined. By roving the catheter on the heart while the local electrogram is recorded simultaneously, the 3-dimensional figure of the heart is reconstructed and an activation or voltage map is generated. EVALUATION: The system was used in 19 patients with ventricular tachycardia or other arrhythmias. The focus or reentrant circuit of the tachycardia was precisely located and a map-guided procedure was successfully performed in all patients. Cardiopulmonary bypass allowed for the tachycardias to be mapped without any hemodynamic compromise. CONCLUSIONS: Intraoperative mapping using the electroanatomic mapping system enables a precise localization of the tachycardia substrate.

Electroanatomical mapping-guided endocardial and epicardial ablation of sustained ventricular tachycardia originating from alcohol septal ablation-induced scar in a patient with hypertrophic obstructive cardiomyopathy.

Ventricular Tachycardia After Alcohol Septal Ablation. A 76-year-old female developed 2 different ventricular tachycardias (VTs) 5 years after alcohol septal ablation (ASA) for symptomatic hypertrophic obstructive cardiomyopathy. VT#1 was a small macroreentry at the anterior border of the low-voltage zone, suggesting the ASA-scar and eliminated by endocardial ablation at a site recording fractionated potentials covering the mid-diastolic and presystolic periods. VT#2 was a focal VT and eliminated by epicardial cryoablation at the basal posterior left ventricle, suggesting the posterior border of the ASA-scar. Using the electroanatomical mapping, we demonstrated that the mechanism of the VTs was reentry at the edge of the ASA-scar.