Elucidating left atrial electrical potential with microelectrode catheter: A case of coronary sinus ostial atresia with small persistent left superior vena cava.

A 51-year-old woman presented with recurring palpitations. Electrocardiography revealed narrow QRS tachycardia with short RP configuration. Computed tomography showed coronary sinus (CS) ostial atresia along with a small persistent left superior vena cava (PLSVC).

Improvement in quality of life and cardiac function after catheter ablation for asymptomatic persistent atrial fibrillation.

Background: Catheter ablation (CA) for atrial fibrillation (AF) is widely performed. However, the indication for CA in patients with asymptomatic persistent AF is still controversial.

Methods: Among 259 consecutive patients who were hospitalized for initial CA of AF, a total of 45 patients who had asymptomatic persistent AF were retrospectively analyzed.

Successful Transcatheter Aortic Valve Implantation in a Patient with Radiation-induced Aortic Stenosis for Mediastinal Hodgkin Lymphoma.

Aortic stenosis (AS), a late complication of thoracic radiation therapy for chest lesions, is often coincident with porcelain aorta or hostile thorax. We herein report a 59-year-old man with a history of mediastinal Hodgkin lymphoma treated with radiation therapy but later presenting with heart failure caused by severe AS. Severe calcification in the mediastinum and around the ascending aorta made it difficult to perform surgical aortic valve replacement.

Propranolol Attenuates Late Sodium Current in a Long QT Syndrome Type 3-Human Induced Pluripotent Stem Cell Model.

Background: Long QT syndrome type 3 (LQT3) is caused by gain-of-function mutations in the gene, which encodes the α subunit of the cardiac voltage-gated sodium channel. LQT3 patients present bradycardia and lethal arrhythmias during rest or sleep. Further, the efficacy of β-blockers, the drug used for their treatment, is uncertain.

Occurrence of right ventricular dysfunction immediately after pericardiocentesis.

The changes in cardiac function that occur after pericardiocentesis are unclear. An understanding of the effect of pericardiocentesis on right ventricular (RV) and left ventricular (LV) function is clinically important. This study was performed to assess RV and LV function with echocardiography before and after pericardiocentesis.

Subacute aortic regurgitation due to traumatic tear in the aortic wall.

A 37-year-old man presented with heart failure caused by severe aortic regurgitation (AR). He had a history of being involved in a traffic accident 3 months earlier. Imaging tests at admission detected no abnormalities in the aortic valve or aortic wall; however, the left coronary cusp prolapsed slightly on transthoracic echocardiography.

Very long-term follow-up data of non-ischemic idiopathic dilated cardiomyopathy after beta-blocker therapy: recurrence of left ventricular dysfunction and predictive value of I-metaiodobenzylguanidine scintigraphy.

The management of idiopathic dilated cardiomyopathy (DCM) is well established. However, a subset of patients do not have recovery from or have recurrences of left ventricular (LV) dysfunction despite receiving optimal medical therapy. There are limited long-term follow-up data about LV function and the predictive value of iodine-123-metaiodobenzylguanidine (I-MIBG) scintigraphy, especially among the Japanese population.

Gene-Based Risk Stratification for Cardiac Disorders in Mutation Carriers.

Background: Mutations in (), which encodes lamin A and C, typically cause age-dependent cardiac phenotypes, including dilated cardiomyopathy, cardiac conduction disturbance, atrial fibrillation, and malignant ventricular arrhythmias. Although the type of mutations have been reported to be associated with susceptibility to malignant ventricular arrhythmias, the gene-based risk stratification for cardiac complications remains unexplored.

Methods And Results: The multicenter cohort included 77 mutation carriers from 45 families; cardiac disorders were retrospectively analyzed.

Development of a Patient-Derived Induced Pluripotent Stem Cell Model for the Investigation of SCN5A-D1275N-Related Cardiac Sodium Channelopathy.

Background: TheSCN5Agene encodes the α subunit of the cardiac voltage-gated sodium channel, Na1.5. The missense mutation, D1275N, has been associated with a range of unusual phenotypes associated with reduced Na1.

Allele-specific ablation rescues electrophysiological abnormalities in a human iPS cell model of long-QT syndrome with a CALM2 mutation.

Calmodulin is a ubiquitous Ca2+ sensor molecule encoded by three distinct calmodulin genes, CALM1-3. Recently, mutations in CALM1-3 have been reported to be associated with severe early-onset long-QT syndrome (LQTS). However, the underlying mechanism through which heterozygous calmodulin mutations lead to severe LQTS remains unknown, particularly in human cardiomyocytes.

Patient-Specific Human Induced Pluripotent Stem Cell Model Assessed with Electrical Pacing Validates S107 as a Potential Therapeutic Agent for Catecholaminergic Polymorphic Ventricular Tachycardia.

Introduction: Human induced pluripotent stem cells (hiPSCs) offer a unique opportunity for disease modeling. However, it is not invariably successful to recapitulate the disease phenotype because of the immaturity of hiPSC-derived cardiomyocytes (hiPSC-CMs). The purpose of this study was to establish and analyze iPSC-based model of catecholaminergic polymorphic ventricular tachycardia (CPVT), which is characterized by adrenergically mediated lethal arrhythmias, more precisely using electrical pacing that could promote the development of new pharmacotherapies.

Cardiac sodium channel mutation associated with epinephrine-induced QT prolongation and sinus node dysfunction.

Background: Long-QT syndrome (LQTS) is an inherited arrhythmia characterized by prolonged ventricular repolarization and malignant tachyarrhythmias. LQT1, LQT2, and LQT3 are caused by mutations in KCNQ1 (LQT1), KCNH2 (LQT2), and SCN5A (LQT3), which account for approximately 90% of genotyped LQTS patients. Most cardiac events in LQT1 patients occur during exercise, whereas patients with LQT3 tend to have arrhythmic events during rest or asleep.

Long-Term (>10 Years) clinical outcomes of first-in-human biodegradable poly-l-lactic acid coronary stents: Igaki-Tamai stents.

Background: The purpose of this study was to evaluate the long-term safety of the Igaki-Tamai stent, the first-in-human fully biodegradable coronary stent made of poly-l-lactic acid.

Methods And Results: Between September 1998 and April 2000, 50 patients with 63 lesions were treated electively with 84 Igaki-Tamai stents. Overall clinical follow-up (>10 years) of major adverse cardiac events and rates of scaffold thrombosis was analyzed together with the results of angiography and intravascular ultrasound.