Seizures and Syncope Due to Complete Atrioventricular Block in a Patient with Acute Myocarditis with a Normal Left Ventricular Systolic Function

A 43-year-old man was admitted to our hospital presenting with seizures and syncope. He had a history of a cold with a fever of 39°C occurring three days earlier. Electrocardiography (ECG) showed complete atrioventricular block (AV block) with a maximum pause of 32 seconds, for which temporary pacing was performed. Echocardiography showed mild hypertrophy of the left ventricle (LV) with a normal ejection fraction of 61%. Coronary angiography showed normal coronary arteries. Then, an endomyocardial biopsy was performed, the results of which indicated a diagnosis of acute myocarditis. After admission, the complete atrioventricular block disappeared together with normalization of the LV wall thickness

Cardiogenic Shock due to Left Ventricular Outflow Obstruction and Complete Atrioventricular Block in a Patient with Hypertrophic Cardiomyopathy with Acute Myocarditis

A 67-year-old woman was referred to our hospital with a sudden syncopal attack. She suffered from cardiogenic shock due to left ventricular (LV) outflow stenosis with simultaneous complete atrioventricular (AV) block. An endomyocardial biopsy of the left ventricle demonstrated myocardial disarray and myocardial fibrous and edematous tissue with infiltration of mononuclear cells. Cardiac magnetic resonance imaging (cMRI) detected a damaged septal area that was likely associated with the conduction disturbance. The diagnosis was hypertrophic cardiomyopathy accompanied by acute myocarditis. Although the LV outflow stenosis was transient, the complete AV block was persistent, thus requiring permanent pacemaker implantation

Relationships Between Clinical Characteristics and Decreased Plakoglobin and Connexin 43 Expressions in Myocardial Biopsies From Patients With Arrhythmogenic Right Ventricular Cardiomyopathy

Reduced expressions of plakoglobin and connexin 43 have been reported in the myocardium of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). However, the relationships between these expression abnormalities and the clinical features of ARVC remain unknown. The expressions of plakoglobin and connexin 43 in myocardial biopsy specimens from 10 patients with confirmed ARVC, and 13 control patients without ARVC (non-ARVC; hypertrophic cardiomyopathy, n = 7; dilated cardiomyopathy, n = 6), were examined by immunostaining to evaluate the relationships between these expressions and the clinical characteristics of ARVC. The ratios of plakoglobin/N-cadherin and of plakoglobin/connexin 43 expressions were significantly lower in the ARVC group than in the control group. Significantly more patients had decreased plakoglobin expression in the ARVC group than in the control group (9/10 versus 7/13; P = 0.0376). Sustained ventricular tachycardia occurred more frequently in patients with ARVC and with decreased expressions of both plakoglobin and connexin 43 than in those with decreased expression of plakoglobin alone (5/5 versus 1/4, P = 0.048). Decreased expressions of both connexin 43 and plakoglobin in the myocardium might be associated with the development of arrhythmia in ARVC

Cyclophosphamide-induced Cardiotoxicity with a Prolonged Clinical Course Diagnosed on an Endomyocardial Biopsy

A 31-year-old woman with primary mediastinal large B-cell lymphoma refractory to conventional chemotherapy was treated with high-dose chemotherapy containing cyclophosphamide (CY). Subsequently, she was treated with auto peripheral blood stem cell transplantation. Although a complete remission was obtained, heart failure developed two months later. Echocardiography showed an impaired systolic function with peri-cardial effusion. A biopsy of the endomyocardial region from the left ventricle demonstrated spotty myocar-dial hemorrhage and myocardial fibrosis with disruption and aggregation of mitochondrial cristae. Based on these findings, CY-induced cardiotoxicity was diagnosed. The patient was treated with conventional therapy for heart failure, which required approximately one year to improve her condition

Mass lesions surrounding coronary artery associated with immunoglobulin G4-related disease

Immunoglobulin G4 (IgG4)-related diseases have been reported to be systemic diseases characterized by elevation of serum IgG4 concentration and infiltration of IgG4-positive plasma cells within the target organ. However, the involvement of coronary artery is very rare. Here, we report a 62-year-old man with mass lesions surrounding coronary artery and abdominal aorta associated with IgG4-related disease diagnosed by a needle biopsy of the mass lesion surrounding the coronary artery using echocardiography and computed tomography. After we started to treat the patient with prednisolone, his serum IgG4 level decreased, and the mass lesions of coronary and abdominal artery were markedly reduced in size after 4 months. In conclusion, IgG4-related disease should be considered in addition to tumors such as malignant lymphoma when mass lesions surrounding coronary artery are detected