Comparison of Clinical and Echocardiographic Characteristics between Cardiac Myxomas and Masses Mimicking Myxoma.

BACKGROUND AND OBJECTIVES: Cardiac myxoma is the most frequent benign cardiac tumor that can result in cardiac and systemic symptoms. We investigated clinical and echocardiographic characteristics of patients with cardiac masses suggesting myxoma. METHODS: We investigated 265 consecutive patients with an echocardiographic diagnosis of cardiac myxomas in 4 teaching hospitals in Korea. RESULTS: The mean age was 61±16 years and 169 patients (63.8%) were female. The most frequent referral reason for echocardiography was an evaluation of cardiac symptoms (43.4%). Tumors were incidentally detected in 82 patients (30.9%). Left atrium (LA) was the most frequently involved site (84.5%) and 19 patients (7.2%) had non-atrial tumors. The mean tumor size was 38.7×26.0 mm (range, 4-96 mm). Of 186 patients (70.2%) who had pathological diagnosis, 174 (93.5%) were confirmed with myxoma, 8 (4.3%) with other tumors and 4 (2.2%) with thrombi. Compared to myxoma, smaller size (20.4×12.6 mm vs. 41.4×27.6 mm, p<0.01) and non-LA location (87.5% vs. 10.5%, p<0.001) were associated with non-myxoma tumors, and more frequent atrial fibrillation (AF, 75.0% vs. 7.0%, p<0.001) and larger LA diameter (55.0±14.6 mm vs. 41.3±7.7 mm, p=0.001) were related to thrombi. CONCLUSIONS: Of 265 patients with an echocardiographic diagnosis with cardiac myxomas, 174 (65.7%) were surgically confirmed with myxomas. Compared with cardiac myxoma, other tumors were smaller and more frequently found in non-atrial sites. Thrombi were associated with AF and larger LA diameter.

Diastolic Dysfunction Induced by a High-Fat Diet Is Associated with Mitochondrial Abnormality and Adenosine Triphosphate Levels in Rats.

BACKGROUND: Obesity is well-known as a risk factor for heart failure, including diastolic dysfunction. However, this mechanism in high-fat diet (HFD)-induced obese rats remain controversial. The purpose of this study was to investigate whether cardiac dysfunction develops when rats are fed with a HFD for 10 weeks; additionally, we sought to investigate the association between mitochondrial abnormalities, adenosine triphosphate (ATP) levels and cardiac dysfunction. METHODS: We examined myocardia in Wistar rats after 10 weeks of HFD (45 kcal% fat, n=6) or standard diet (SD, n=6). Echocardiography, histomorphologic analysis, and electron microscopy were performed. The expression levels of mitochondrial oxidative phosphorylation (OXPHOS) subunit genes, peroxisome-proliferator-activated receptor γ co-activator-1α (PGC1α) and anti-oxidant enzymes were assessed. Markers of oxidative stress damage, mitochondrial DNA copy number and myocardial ATP level were also examined. RESULTS: After 10 weeks, the body weight of the HFD group (349.6±22.7 g) was significantly higher than that of the SD group (286.8±14.9 g), and the perigonadal and epicardial fat weights of the HFD group were significantly higher than that of the SD group. Histomorphologic and electron microscopic images were similar between the two groups. However, in the myocardium of the HFD group, the expression levels of OXPHOS subunit NDUFB5 in complex I and PGC1α, and the mitochondrial DNA copy number were decreased and the oxidative stress damage marker 8-hydroxydeoxyguanosine was increased, accompanied by reduced ATP levels. CONCLUSION: Diastolic dysfunction was accompanied by the mitochondrial abnormality and reduced ATP levels in the myocardium of 10 weeks-HFD-induced rats.

Churg-Strauss syndrome associated with rapid deterioration of left ventricular diastolic dysfunction and conduction disturbance.

Cardiac involvement in Churg-Strauss syndrome (CSS) is a major cause of mortality. Here we report a case of a 75-year-old woman with eosinophilic endomyocarditis due to CSS. An electrocardiogram showed intraventricular conduction delay, and echocardiography showed an impaired relaxation pattern and biventricular apical thickening. Magnetic resonance imaging revealed subendocardial delayed enhancement with biventricular apical thrombi. Endomyocardial biopsy showed perivascular eosinophilic infiltration. Despite resolution of the hypereosinophilia after steroid therapy, her left ventricular (LV) diastolic function worsened into a restrictive pattern and she died with a ventricular escape rhythm on her 14th day in the hospital. This case is unusual in that there was rapid progression of the LV diastolic dysfunction and conduction disturbance due to CSS.

Hydroxychloroquine-induced cardiomyopathy that presented as pulmonary hypertension: a newly noted complication.

We examined a 63-year-old woman with progressive dyspnea. Two years prior to admission to our hospital, she had been diagnosed with rheumatoid arthritis and treated with hydroxychloroquine (HCQ) with a cumulative dose of 164 g. In addition, 2 months earlier, she had been diagnosed with connective tissue disease-related pulmonary artery hypertension. We performed an electrocardiogram and noted complete atrioventricular block. A transthoracic echocardiogram showed pulmonary hypertension. Due to the unclear nature of the pulmonary hypertension, we performed cardiac catheterization and right ventricular endomyocardial biopsy. Cardiac catheterization revealed that pulmonary hypertension was due to left ventricular dysfunction. Electron microscopy of the cardiac biopsy demonstrated a curvilinear body, diagnostic of HCQ toxicity. Thus, we diagnosed pulmonary hypertension owing to left heart disease and complete atrioventricular block that resulted from HCQ toxicity. Insertion of a permanent pacemaker and discontinuation of HCQ dramatically improved the disease state. This is the first report of this type of cardiac complication with HCQ; it raises the awareness that HCQ may cause cardiac complications despite a small cumulative dose relative to doses reported in other cases. Furthermore, we emphasize that cardiac catheterization played a critical role in the differential diagnosis from pulmonary hypertension associated with connective tissue disease.

A primary left atrial leiomyosarcoma mimicking myxoma: echocardiographic findings.

Echocardiography is the initial imaging modality to delineate the cardiac mass. Clinically echocardiographic information about morphology, location, motion and hemodynamic consequences of the tumor is very important for decision making in terms of additional imaging studies and treatment. We present a case of left atrial leiomyosarcoma masquerading as myxoma preoperatively and discuss the echocardiographic features for distinguishing leiomyosarcoma from a benign tumor, such as myxoma.

Isolated left ventricular noncompaction cardiomyopathy accompanied by severe mitral regurgitation.

Isolated left ventricular noncompaction cardiomyopathy (IVNC) is a cardiomyopathy thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium. This abnormality is often associated with other congenital cardiac defects. A 21-year-old man presented to the emergency department with worsening exertional dyspnea during the previous 2 months. Two-dimensional and Doppler echocardiography revealed an enlarged left atrium (LA) and a markedly dilated left ventricle (LV) with preserved LV systolic function, severe mitral valve regurgitation, and prolapse due to chordae rupture. The myocardium of the LV and right ventricle (RV) had excessively prominent trabeculations and deep intertrabecular recesses. He is the first patient in Korea who has undergone mitral valve replacement surgery because of severe mitral valve regurgitation and prolapse due to chordae rupture accompanied by IVNC.

Extensive primary cardiac lymphoma diagnosed by percutaneous endomyocardial biopsy.

Primary cardiac lymphoma (PCL) is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium. It is rare in immunocompetent patients and represents 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas. The clinical behavior is aggressive and the early symptoms are cardiac failure, syncope, arrhythmia, or pericardial effusion. Although echocardiography, computed tomography (CT) scan, magnetic resonance image (MRI) are the mainly used imaging techniques to detect cardiac tumors, pathologic examination is always required to confirm the diagnosis. Diagnosis of PCL is difficult due to non-specific clinical manifestations and requires invasive approach to get histopathologic evidence. While surgery with systemic chemotherapy or in combination with irradiation has been attempted, the only effective treatment is chemotherapy. However, the prognosis remains poor. We report on a 42-year-old woman who is diagnosed histopathologically as PCL by cardiac catheterization assisted percutaneous endomyocardial biopsy and treated successfully by anthracycline based chemotherapy.