No abstract available.
Cardiomyopathy, Dilated/etiology/*pathology ; Endomyocardial Fibrosis/*radiography ; Gadolinium ; Heart Ventricles/*physiopathology ; Humans ; Magnetic Resonance Imaging/*methods ; Ventricular Dysfunction, Left/*physiopathology

We report multidetector computed tomography (MDCT) and cardiac magnetic resonance (CMR) findings of a 34-year-old female with isolated left ventricular apical hypoplasia. The MDCT and CMR scans displayed a spherical left ventricle (LV) with extensive fatty infiltration within the myocardium at the apex, interventricular septum and inferior wall, anteroapical origin of the papillary muscle, right ventricle wrapping around the deficient LV apex, and impaired systolic function. MDCT visualized morphologic and also functional findings of this unique cardiomyopathy.
Adult ; Cardiomyopathy, Dilated/*radiography ; Female ; Heart Ventricles/*radiography ; Humans ; Hypoplastic Left Heart Syndrome/*radiography ; Imaging, Three-Dimensional/*methods ; Multidetector Computed Tomography/*methods ; Myocardium/pathology

Cardiac resynchronization therapy is known to reduce morbidity and mortality in patients with advanced heart failure as a result of dyssynchrony and systolic dysfunction of the left ventricle. Placement of the left ventricular (LV) lead via the coronary sinus can be difficult. When LV lead implantation is difficult, a video-assisted epicardial approach can be a good alternative. Although there are several reports of video-assisted epicardial LV lead implantation, mini-thoracotomy and lead implantation under direct vision have been used in most series. A 49-yr-old woman with dilated cardiomyopathy underwent the video-assisted epicardial LV lead implantation because percutaneous transvenous approach was difficult due to small cardiac veins. The patient was discharged without problems and showed improved cardiac function at the 3 follow-up months. We report the first successful total thoracoscopic LV lead implantation (without mini-thoracotomy) in Korea.
*Cardiac Resynchronization Therapy ; Cardiomyopathy, Dilated/diagnosis ; *Defibrillators, Implantable ; Echocardiography ; Female ; Humans ; Middle Aged ; Thoracic Surgery, Video-Assisted ; Tomography, X-Ray Computed ; Ventricular Dysfunction, Left/radiography/surgery/*therapy

OBJECTIVE: Post-contrast T1 values are closely related to the degree of myocardial extracellular space expansion. We determined the relationship between post-contrast T1 values and left ventricular (LV) diastolic function, LV remodeling, and neurohormonal activation in patients with dilated cardiomyopathy (DCM). MATERIALS AND METHODS: Fifty-nine patients with DCM (mean age, 55 +/- 15 years; 41 males and 18 females) who underwent both 1.5T magnetic resonance imaging and echocardiography were enrolled. The post-contrast 10-minute T1 value was generated from inversion time scout images obtained using the Look-Locker inversion recovery sequence and a curve-fitting algorithm. The T1 sample volume was obtained from three interventricular septal points, and the mean T1 value was used for analysis. The N-Terminal pro-B-type natriuretic peptide (NT-proBNP) level was measured in 40 patients. RESULTS: The mean LV ejection fraction was 24 +/- 9% and the post-T1 value was 254.5 +/- 46.4 ms. The post-contrast T1 value was significantly correlated with systolic longitudinal septal velocity (s'), peak late diastolic velocity of the mitral annulus (a'), the diastolic elastance index (Ed, [E/e']/stroke volume), LV mass/volume ratio, LV end-diastolic wall stress, and LV end-systolic wall stress. In a multivariate analysis without NT-proBNP, T1 values were independently correlated with Ed (beta = -0.351, p = 0.016) and the LV mass/volume ratio (beta = 0.495, p = 0.001). When NT-proBNP was used in the analysis, NT-proBNP was independently correlated with the T1 values (beta = -0.339, p = 0.017). CONCLUSION: Post-contrast T1 is closely related to LV remodeling, diastolic function, and neurohormonal activation in patients with DCM.
Adult ; Aged ; Algorithms ; Blood Pressure ; Cardiomyopathy, Dilated/*diagnosis/radiography ; Echocardiography ; Extracellular Space/physiology/*radiography ; Female ; Heart Ventricles/physiopathology/radiography ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Multivariate Analysis ; Natriuretic Peptide, Brain/analysis ; Peptide Fragments/analysis ; *Ventricular Remodeling

An 82-yr-old man was presented with fever and cough accompanied by generalized erythematous rash. He had taken mexiletine for 5 months, as he had been diagnosed with dilated cardiomyopathy and ventricular arrhythmia. Laboratory studies showed peripheral blood eosinophilia and elevated liver transaminase levels. Chest radiographs showed multiple nodular consolidations in both lungs. Biopsies of the lung and skin lesions revealed eosinophilic infiltration. After a thorough review of his medication history, mexiletine was suspected as the etiologic agent. After discontinuing the mexiletine and starting oral prednisolone, the patient improved, and the skin and lung lesions disappeared. Subsequently, mexiletine was confirmed as the causative agent based on a positive patch test. Drug-induced hypersensitivity syndrome is a severe adverse reaction to drugs and results from treatment with anticonvulsants, allopurinol, sulfonamides, and many other drugs. Several cases of mexiletine-induced hypersensitivity syndrome have been reported in older Japanese males with manifestation of fever, rash, peripheral blood eosinophilia, liver dysfunction without other organ involvement. Here, we report a case of mexiletine-induced hypersensitivity syndrome which presented as eosinophilic pneumonia in a Korean male.
Aged, 80 and over ; Anti-Arrhythmia Agents/*adverse effects ; Arrhythmias, Cardiac/drug therapy ; Cardiomyopathy, Dilated/drug therapy ; Drug Hypersensitivity/*diagnosis/etiology ; Exanthema/pathology ; Humans ; Lung/pathology/radiography ; Male ; Mexiletine/*adverse effects ; Pulmonary Eosinophilia/*chemically induced/*diagnosis ; Syndrome ; Tomography, X-Ray Computed

Case 1: A 39-year-old man underwent orthotopic heart transplantation on November 1994 for dilated cardiomyopathy. His postoperative course was unevenful and medications included daily cyclosporin A, Immuran and prednisone. On December 13.1994, he developed cough and sore throat. Chest radiographs revealed multiple patch growing lesions. Sputum fungus culture revealed Aspergillus Fumigatus. The patient was treated with daily infusion of amphotericin B. He remains well without evidence of relapse of Aspergillus, Case 2: This 39-year-old man had undergone orthotopic heart transplantation on November 16 1994 for dilated cardiomyopathy. In December 7.1994. he developed recurrent syncope. Chest radiographs revealed fungus ball like lesion on right lung field. On open lung biopsy and wedge resection of the mass was performed. Aspergillosis and CMV infection was demonstrated in the biopsy specimen. The patient was treated with conventional amphotericin B therapy for over 7weeks and Ganciclovir for over 2weeks. At the end of therapy chest X-ray showed only small residual scar in the area of previous mass.
Adult ; Amphotericin B ; Aspergillosis* ; Aspergillus ; Aspergillus fumigatus ; Azathioprine ; Biopsy ; Cardiomyopathy, Dilated ; Cicatrix ; Cough ; Cyclosporine ; Fungi ; Ganciclovir ; Heart Transplantation* ; Heart* ; Humans ; Lung ; Pharyngitis ; Prednisone ; Radiography, Thoracic ; Recurrence ; Sputum ; Syncope ; Thorax

PURPOSE: ECD is a rare idiopathic condition characterized by infiltration of the bone, heart, lungs, retroperitoneum, skin, CNS and other tissues by a fibrosing process containing xanthomatous histiocytes and multinucleated giant cells of the Touton type. Orbital involvement of ECD is rare and we report two cases of Erdheim-Chester disease. METHODS: A 46-year-old man with dilated cardiomyopathy and chronic renal failure and a 70-year-old woman with pulmonary disease and intracranial mass visited our clinic because of bilateral proptosis. RESULTS: The systemic evaluation revealed multiple organ involvement, such as bone, heart, lung and retroperitoneum ,while radiography of the tibia showed symmetrical osteosclerosis. The biopsy of tibia revealed a diffuse infiltration of foamy histiocytes and multinucleated giant cells of the Touton type. Based on the diagnosis of ECD, the patients were treated with radiation and steroids. CONCLUSIONS: Although most patients with ECD do not have ocular involvement, the unusual occurrence of bilateral proptosis and diffuse infiltrative orbital masses are suggestive of ECD. These findings should alert the ophthalmologist to this potentially serious systemic disease and prompt an evaluation for systemic manifestations.
Aged ; Biopsy ; Cardiomyopathy, Dilated ; Diagnosis ; Erdheim-Chester Disease* ; Exophthalmos ; Female ; Giant Cells ; Heart ; Histiocytes ; Humans ; Kidney Failure, Chronic ; Lung ; Lung Diseases ; Middle Aged ; Orbit* ; Osteosclerosis ; Radiography ; Skin ; Steroids ; Tibia

Graves disease (GD) can lead to complications such as cardiac arrhythmia and heart failure. Although dilated cardiomyopathy (DCMP) has been occasionally reported in adults with GD, it is rare in children. We present the case of a 32-month-old boy with DCMP due to GD. He presented with irritability, vomiting, and diarrhea. He also had a history of weight loss over the past few months. On physical examination, he had tachycardia without fever, a mild diffuse goiter, and hepatomegaly. The chest radiograph showed cardiomegaly with pulmonary edema, while the echocardiography revealed a dilated left ventricle with an ejection fraction (EF) of 28%. The thyroid function test (TFT) showed elevated serum T3 and decreased thyroid stimulating hormone (TSH) levels. The TSH receptor autoantibody titer was elevated. He was diagnosed with DCMP with GD; treatment with methylprednisolone, diuretics, inotropics, and methimazole was initiated. The EF improved after the TFT normalized. At follow-up several months later, although the TFT results again showed evidence of hyperthyroidism, his EF had not deteriorated. His cardiac function continues to remain normal 1.5 months after treatment was started, although he still has elevated T3 and high TSH receptor antibody titer levels due to poor compliance with drug therapy. To summarize, we report a young child with GD-induced DCMP who recovered completely with medical therapy and, even though the hyperthyroidism recurred several months later, there was no relapse of the DCMP.
Adult ; Arrhythmias, Cardiac ; Cardiomegaly ; Cardiomyopathy, Dilated* ; Child* ; Child, Preschool ; Compliance ; Deoxycytidine Monophosphate ; Diarrhea ; Diuretics ; Drug Therapy ; Echocardiography ; Fever ; Follow-Up Studies ; Goiter ; Graves Disease* ; Heart Failure ; Heart Ventricles ; Hepatomegaly ; Humans ; Hyperthyroidism ; Male ; Methimazole ; Methylprednisolone ; Physical Examination ; Pulmonary Edema ; Radiography, Thoracic ; Receptors, Thyrotropin ; Recurrence ; Tachycardia ; Thyroid Function Tests ; Thyrotropin ; Vomiting ; Weight Loss

Graves disease (GD) can lead to complications such as cardiac arrhythmia and heart failure. Although dilated cardiomyopathy (DCMP) has been occasionally reported in adults with GD, it is rare in children. We present the case of a 32-month-old boy with DCMP due to GD. He presented with irritability, vomiting, and diarrhea. He also had a history of weight loss over the past few months. On physical examination, he had tachycardia without fever, a mild diffuse goiter, and hepatomegaly. The chest radiograph showed cardiomegaly with pulmonary edema, while the echocardiography revealed a dilated left ventricle with an ejection fraction (EF) of 28%. The thyroid function test (TFT) showed elevated serum T3 and decreased thyroid stimulating hormone (TSH) levels. The TSH receptor autoantibody titer was elevated. He was diagnosed with DCMP with GD; treatment with methylprednisolone, diuretics, inotropics, and methimazole was initiated. The EF improved after the TFT normalized. At follow-up several months later, although the TFT results again showed evidence of hyperthyroidism, his EF had not deteriorated. His cardiac function continues to remain normal 1.5 months after treatment was started, although he still has elevated T3 and high TSH receptor antibody titer levels due to poor compliance with drug therapy. To summarize, we report a young child with GD-induced DCMP who recovered completely with medical therapy and, even though the hyperthyroidism recurred several months later, there was no relapse of the DCMP.
Adult ; Arrhythmias, Cardiac ; Cardiomegaly ; Cardiomyopathy, Dilated* ; Child* ; Child, Preschool ; Compliance ; Deoxycytidine Monophosphate ; Diarrhea ; Diuretics ; Drug Therapy ; Echocardiography ; Fever ; Follow-Up Studies ; Goiter ; Graves Disease* ; Heart Failure ; Heart Ventricles ; Hepatomegaly ; Humans ; Hyperthyroidism ; Male ; Methimazole ; Methylprednisolone ; Physical Examination ; Pulmonary Edema ; Radiography, Thoracic ; Receptors, Thyrotropin ; Recurrence ; Tachycardia ; Thyroid Function Tests ; Thyrotropin ; Vomiting ; Weight Loss