Congenital intrapericardial left atrial appendage aneurysms (LAAA) are very rare. Most cases are asymptomatic and this malady is generally incidentally diagnosed in older patients. LAAAs are usually accompanied with supraventricular arrhythmias and life-threatening systemic embolism. Complete surgical correction is recommended immediately after the diagnosis to prevent significant complications, and even for the asymptomatic patients. We report here on the case of a 45-year-old man who presented with cerebral embolism due to LAAA. The patient was successfully treated with a resection of the aneurysm.
Aneurysm ; Arrhythmias, Cardiac ; Atrial Appendage ; Embolism ; Heart Atria ; Humans ; Intracranial Embolism ; Middle Aged ; Stroke

We report a case of an Epstein-Barr virus (EBV)-associated inflammatory pseudotumor-like follicular dendritic cell tumor (IPT-like FDC tumor). The tumor occurred in the spleen of a 64-year-old woman with a history of a diffuse large B-cell lymphoma (DLBCL) of neck nodes that presented four years ago. The splenectomy specimen revealed a 5 cm-sized, tan-colored and well-circumscribed mass. Histologically, spindle or ovoid cells with large vesicular nuclei were admixed with abundant inflammatory cells. Immunohistochemically, spindle cells were positive for FDC marker CD35, but negative for CD20, CD30 and ALK. EBV was detected almost exclusively in spindle cells by EBER in situ hybridization. IPT-like FDC tumors are rare, and are recognized as a distinctive clinicopathologic variant of FDC tumors. Among only 18 similar cases reported in the English language literature, the present case is the first case of a patient with a history of DLBCL.
Dendritic Cells, Follicular* ; Female ; Granuloma, Plasma Cell ; Herpesvirus 4, Human ; Humans ; In Situ Hybridization ; Lymphoma, B-Cell* ; Middle Aged ; Neck ; Spleen* ; Splenectomy

Neurofibromatosis type I is a genetic disease caused by mutations in the neurofibromin 1 (NF1) gene. Although it is characterized by a number of distinct clinical features, including cafe au lait macules, freckling in the axillary or inguinal regions, neurofibromas, and Lisch nodules (iris harmartomas), it can affect all physiological systems in the body [1]. Neurofibromatosis-related pulmonary hypertension has also been reported, and some patients showed a poor prognosis despite having received proper medical treatment [2-4]. We herein describe a case of pulmonary hypertension in a patient with neurofibromatosis type I who had no identified risk factors of pulmonary hypertension. To our knowledge, this is the first such report in Korea.
Humans ; Hypertension ; Hypertension, Pulmonary* ; Korea ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1* ; Neurofibromin 1 ; Prognosis ; Risk Factors

Neurofibromatosis type I is a genetic disease caused by mutations in the neurofibromin 1 (NF1) gene. Although it is characterized by a number of distinct clinical features, including cafe au lait macules, freckling in the axillary or inguinal regions, neurofibromas, and Lisch nodules (iris harmartomas), it can affect all physiological systems in the body [1]. Neurofibromatosis-related pulmonary hypertension has also been reported, and some patients showed a poor prognosis despite having received proper medical treatment [2-4]. We herein describe a case of pulmonary hypertension in a patient with neurofibromatosis type I who had no identified risk factors of pulmonary hypertension. To our knowledge, this is the first such report in Korea.
Humans ; Hypertension ; Hypertension, Pulmonary* ; Korea ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1* ; Neurofibromin 1 ; Prognosis ; Risk Factors

BACKGROUND AND OBJECTIVES: The degree of diastolic dysfunction may explain the difference in clinical symptoms between patients with similar degrees of systolic dysfunction. Pseudonormal mitral filling pattern with increased left ventricular filling pressure is a transitional stage of diastolic dysfunction and is difficult to distinguish from normal. Preload is one of factors affecting the diastolic filling patterns and mitral flow patterns may be influenced by changes in preload in the absence of changes in the left ventricular pressure-volume curve. The changes in the mitral flow velocities caused by preload reduction may be useful in distinguishing patients with a pseudonormal pattern from those with normal. The aim of this study was to establish whether the effect of changes in pulmonary venous flow pattern by preload reduction may be useful in distinguishing pseudonormal pattern from normal. MATERIALS AND METHODS: This study included 40 patients (men 25, female 15, average age 51.0+/-11.2 years) underwent left side cardiac catheterization among patients with normal or pseudonormal patterns. All patients with pseudonormal group had increased LVEDPr (>15 mmHg). The Doppler mitral inflow & pulmonary venous flow parameters at baseline and during reduction of preload using Valsalva maneuver were recorded. RESULTS: 1) There were no difference in sex, diabetes mellitus, hypertension and lipid profiles between both groups. The left ventricular systolic function was better and mean age was younger in normal than pseudonormal group (p<0.05). The incidence of coronary artery disease was more frequent and LV mass was more increased in pseudonormal than normal group (p<0.05). 2) E and A velocities were significantly decreased but E/A ratio was still <0A65B> 1.0 during Valsalva maneuver in normal group (p<0.05). In pseudonormal group, E velocity was significantly decreased but A velocity was not significantly changed and the E/A ratio was <1.0 during Valsalva (p<0.05). Therefore the change revealed masked LV relaxation abnormality pattern. 3) S and D velocities of pulmonary venous flow were significantly decreased and S/D ratio was significantly increased in both groups (p<0.05). The % changes before and after Valsalva maneuver showed that S velocity was less decreased, D velocity was more decreased and S/D ratio was more significantly increased in pseudonormal than normal group (p<0.05). That revealed masked LV relaxation abnormality pattern. CONCLUSIONS: The Valsalva maneuver for preload reduction is a relatively simple, easily applicable, safe and reproducible method of acutely reducing venous return. The assessment of changes in pulmonary venous flow pattern by preload reduction may be helpful in distinguishing pseudonormal and normal diastolic function in addition to changes in mitral inflow pattern.
Cardiac Catheterization ; Cardiac Catheters ; Coronary Artery Disease ; Diabetes Mellitus ; Female ; Humans ; Hypertension ; Incidence ; Masks ; Relaxation ; Valsalva Maneuver

BACKGROUND: Pulmonary venous flow velocity pattern (PVFVP) is widely used to assess LV diastolic function. It is known that the parameters of PVFVP have a significant correlation with the ratio of peak early diastolic filling velocity (E) to peak filling velocity at atrial contraciton (A) measured in the transmitral flow. However, the correlations between parameters of superior vena caval flow (SVCF) and transtricuspid E/A ratio have not been reported. Therefore the present investigation was performed to elucidate these correlations. METHODS: Fifty patients (26 men, mean age 63.1+/-11.1 years), who did not have significant tricuspid valvular disease and restrictive filling pattern on tricuspid and superior vena caval doppler, were included in this study. SVCF was recorded with the transducer positioned at subxiphoid area and the sample volume placed 2 cm within the superior vena cava. Blood flow across the tricuspid valve was obtained from standard four chamber view or modified parasternal four chamber view with the sample volume placed on leaflet tips. Recording was made during midexpiratory apnea. The following doppler parameters were measured: transtricuspid E and A velocity, E/A ratio: systolic (S) and diastolic (D) peak velocities and time velocity integrals (TVI), S/D velocity ratio, S/D TVI ratio, atrial reversal peak velocity (ArV) and TVI (ArTVI) in SVCF. RESULTS: 1) In SVCF, S velocity (63.7+/-11.8 cm/s vs 73.4+/-13.6 cm/sec, p<0.05), S TVI (17.4+/-3.6 cm vs 21.1+/-6.2 cm, p<0.05), ArV (30.0+/-6.9 cm/s vs 37.2+/-7.3 cm/s, p<0.005), and ArTVI (2.7+/-0.8 cm vs 3.3+/-0.8 cm, p<0.01) were significantly decreased in group E/A>1. And D TVI (7.1+/-3.0 cm vs 5.2+/-3.1 cm, p<0.05) and D/S TVI ratio (0.41+/-0.13 vs 0.26+/-0.14, p<0.05) were significantly increased in group E/A>1. 2) As E/A ratio increased, diastolic TVI (r=0.315, p<0.05) and D/S TVI ratio (r=0.448, p<0.001) increased, and ArTVI (r=-0.376, p<0.01) and ArV (r=-0.416, p<0.01) decreased. 3) As E peak velocity increased, SVCF D peak velocity increased (r=0.305, p<0.05). CONCLUSIONS: Tricusupid E/A ratio has positive correlations with D TVI and D/S TVI ratio, and negative correlations with ArTVI and ArV. But there were no correlations in S velocity, D velocity, and S/D velocity ratio as the relation of mitral E/A ratio with PVFVP.
Apnea ; Humans ; Male ; Transducers ; Tricuspid Valve ; Vena Cava, Superior

BACKGROUND: A widened left atrial pressure A wave occurs when left ventricular end-diastolic pressure is increased. It has been reported that increased duration of pulmonary venous flow reversal at atrial systolic pulmonary venous flow is shown to be related to increased left ventricular filling pressure in studies using transesophageal Doppler echocardiography. We evaluate the correlation between LVEDP measured by the invasive method and the mitarl and pulmonary venous flow index recorded by transthoracic Doppler echocardiography. METHODS: Left ventricular pressures at late diastole were measured by fluid-filled catheters in 70-consecutive coronary heart patients undergoing diagnostic cardiac catheterization. Pulmonary venous and mitral flow velocities were recorded by transthoracic pulsed Doppler ultrasound. Adequate recordings were obtained in the 70 patients. Diastolic function differentiated into four categories . RESULTS: Pulmonary venous flow reversal exceeding the duration of the mitral A wave predicted left ventricular end-diastolic pressure > or = 18mmHg with a sensitivity of 0.78 and a specificity of 0.95. Pulmonary venous flow reversal duration (PVad) exceeding 140msec predicted left ventricular end-diastolic pressure > or = 18mmHg with a sensitivity of 0.89 and a specificity of 0.93. This difference in flow duration (PVad-Ad, deltad) correlated well with increased LVEDP (r=0.537, p<0.001). PVad also correlated with increased LVEDP (r=0.503, p<0.001). CONCLUSIONS: If pulmonary venous flow reversal (PVad) exceeds both the duration of the mitral A wave and 140msec, it indicates an exaggerated increase in left ventricular end diastolic pressure.
Atrial Pressure ; Blood Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Catheters ; Coronary Artery Disease* ; Coronary Vessels* ; Diastole ; Echocardiography, Doppler ; Heart ; Humans ; Relaxation ; Sensitivity and Specificity ; Ultrasonography* ; Ventricular Pressure

BACKGROUND: Coronary artery disease(CAD) has been related to a number of factors, including diabetes, smoking,hypertension, blood lipids, and family history of CAD. However, a few studies have examined the correlation between these factors and severity of CAD. In the present study, we aimed to define the correlation between risk factors and the severity of CAD. METHODS: The study population was 309 consecutive patients who underwent coronary angiography in Kang Nam St. Marys hospital between Mar. 1992 and Aug. 1994. We analyzed risk factors of CA ; age, total cholesterol(TC), triglyceride, high density lipoprotein cholesterol(HDL-C), low density lipoprotein cholesterol cholesterol(LDL-C), the ratio of TC to HDL-C, Lp(a), uric acid, fibrinogen, diabetes, hypertension, smoking, and family history of CAD. All films were reviewed without specific knowledge of blood laboratory results. Significant occlusive disease was defined as >50% diameter obstruction of a major coronary vessel. Extent of disease was defined as the number of major coronalry arteries with significant occlusive disease. RESULTS: 1) Statistically significant risk factors between one vessel disease group and multivessel disease group are age, TC, LDL-C, and diabetes in total patients and age, LDL, and diabetes in male patients. But there is no significant difference in female patients. 2) In patients with multivessel disease, age, HDL-C level are significantly lowere, and TG level and the percentage of smkoing are significantly higher, in male than female. 3) There is increase in four major risk factors(diabetes, hypertension,smoking,hypercholesterolemia) with the increased number of diseasesd coronary arteries; normal coronary group has 1.07 risk factors, one vessel disease group 1.30, two vessel disease group 1.49, and three vessel disease group 1.78. 4) In total and male patients, three independent variables are significantly associated with severity of CAD. These variables are age, smoking, and diabetes. In female patients, diabetes in only significant independent risk factor. CONCLUSION: The significant independent risk factors associated with severity of CAD are age, diabetes, and smoking in total and male patients. Whereas in female patients, diabetes is only significant independent risk factor.
Arteries ; Cholesterol, LDL ; Coronary Angiography ; Coronary Artery Disease* ; Coronary Vessels* ; Female ; Fibrinogen ; Humans ; Hypertension ; Lipoproteins ; Male ; Risk Factors* ; Smoke ; Smoking ; Triglycerides ; Uric Acid

Recently, we experienced a case of subacute infective endocarditis caused by A. haemolyticum on mitral valve prolapse complicated with systemic emboli, which was successfully treated with antibiotics and valve replacement surgery. To our knowledge, this is the first report to address infective endocarditis caused by A. haemolyticum in a immunocompetent patient who had mitral valve prolapse and survived with successful treatment. Greater awareness of this uncommon organism is needed to make an accurate diagnosis and perform a better clinical management in the early stage of the disease. Recommendation for the treatment of septic A. haemolyticum infections has not been established. Therefore, the treatment should be based on clinical experiences and in vitro susceptibility profiles of the individual strain. The site of infection as well as antimicrobial susceptibility profiles should be considered for appropriate antibiotics choice and decision to perform a surgical intervention.
Anti-Bacterial Agents ; Arcanobacterium* ; Diagnosis ; Endocarditis* ; Humans ; Mitral Valve Prolapse* ; Mitral Valve*

Recently, we experienced a case of subacute infective endocarditis caused by A. haemolyticum on mitral valve prolapse complicated with systemic emboli, which was successfully treated with antibiotics and valve replacement surgery. To our knowledge, this is the first report to address infective endocarditis caused by A. haemolyticum in a immunocompetent patient who had mitral valve prolapse and survived with successful treatment. Greater awareness of this uncommon organism is needed to make an accurate diagnosis and perform a better clinical management in the early stage of the disease. Recommendation for the treatment of septic A. haemolyticum infections has not been established. Therefore, the treatment should be based on clinical experiences and in vitro susceptibility profiles of the individual strain. The site of infection as well as antimicrobial susceptibility profiles should be considered for appropriate antibiotics choice and decision to perform a surgical intervention.
Anti-Bacterial Agents ; Arcanobacterium* ; Diagnosis ; Endocarditis* ; Humans ; Mitral Valve Prolapse* ; Mitral Valve*