A 29-year-old man was referred to the emergency department with a complaint of abdominal pain and dizziness. He had experienced two previous syncopal episodes. His family history revealed that his mother and his two uncles had received permanent pacemaker implantation. His initial heart rate was 49 beats per minute. The electrocardiography (ECG) showed atrial flutter and right bundle branch block (RBBB) with left anterior fascicular block (LAFB). On admission, 24-hour Holter showed ventricular pause up to 16 seconds during syncope. Radio frequency catheter ablation (RFCA) of atrial flutter was performed. The ECG revealed bifascicular block (RBBB and LAFB) and first-degree atrioventricular block. He received a permanent pacemaker implantation. His brother's and his sister's ECGs also showed trifascicular block and the pedigree showed autosomal dominant inheritance. This patient was diagnosed with a progressive familial heart block (PFHB) type I. This would be the first report of a PFHB type I case documented in Korea.
Abdominal Pain ; Adult ; Atrial Flutter ; Atrioventricular Block ; Bundle-Branch Block ; Catheter Ablation ; Dizziness ; Electrocardiography ; Emergencies ; Heart ; Heart Block ; Heart Rate ; Humans ; Korea ; Mothers ; Pedigree ; Syncope ; Wills

PURPOSE: This study was undertaken to examine whether differences exist in the hemoglobin variability according to the types of erythropoiesis stimulating agent (ESA) in hemodialysis (HD) patients. METHODS: Clinical data were retrospectively analyzed from 72 patients on maintenance hemodialysis who were using darbepoetin alfa (n=27), epoetin beta (n=27), and epoetin alpha (n=18). As parameters of hemoglobin variability, hemoglobin cycling, the variance of hemoglobin and the SD/mean of hemoglobin were analyzed. Hemoglobin cycling was defined as the presence of cycles with an amplitude >1.5 g/dL and lasting more than 2 months. RESULTS: Hemoglobin cycling was present in 53 (73.6%) out of 72 HD patients. Hemoglobin cycling in patients receiving darbepoetin alfa had greater frequency (1.63+/-0.93 vs. 1.00+/-0.88 times/year, p<0.05), amplitude (2.88+/-1.48 vs. 1.88+/-1.60 g/dL, p<0.05), and velocity (1.21+/-0.74 vs. 0.73+/-0.66 g/dL/month, p<0.05) than that in patients receiving epoetin beta. The variance of hemoglobin in patients receiving epoetin beta (0.79+/-0.53 g/dL) was smaller than that in patients receiving darbepoetin alfa (1.29+/-0.70 g/dL, p<0.05) and epoetin alfa (1.08+/-0.52 g/dL, p<0.05). Also, the ratio of SD/mean of hemoglobin in patients receiving epoetin beta (8.20+/-2.59%) was lower than that in patients receiving darbepoetin alfa (10.81+/-2.10%, p<0.05) and epoetin alfa (10.30+/-2.10%, p<0.05). CONCLUSION: Hemoglobin variability is differential according to various ESAs, and it may be less with epoetin beta compared with darbepoetin alpha and epoetin alpha.
Anemia ; Erythropoiesis ; Erythropoietin ; Hematinics ; Hemoglobins ; Humans ; Recombinant Proteins ; Renal Dialysis ; Retrospective Studies ; Darbepoetin alfa ; Epoetin Alfa

PURPOSE: Cardiac dysfunction and hyperdynamic systemic circulation may be present in patients with cirrhosis. The purpose of this study was to identify relations between plasma levels of N-terminal-proBNP (NT-proBNP), reflecting early ventricular dysfunction, and the severity of liver disease and cardiac dysfunction in cirrhotic patients. MATERIALS and METHODS: Sixty-three cirrhotic patients and 15 controls (group 1) were enrolled in this study. Plasma levels of NT-proBNP were determined in echocardiographically examined patients, which were allocated to 1 of 3 groups according to Child-Pugh classification or into 2 groups, i.e., a compensated group without ascites (group 2) and decompensated group with ascites (group 3). RESULTS: Plasma NT-proBNP levels were significantly higher in cirrhotic patients (groups 2 and 3) than in age-matched controls (155.9 and 198.3 vs. 40.3pg/mL, respectively, p < 0.05). NT-proBNP levels were significantly increased in Child class C patients than in classes B and A (250.0 vs. 168.6 and 119.6pg/mL, respectively, p < 0.05). Left atrial dimension, wall thickness of left ventricle, and EF or E/E' were significantly increased, and EDT was prolonged in cirrhotic patients than in controls. Increased LVMI and decreased E/A ratio were noted in the group of patients with ascites as compared with the other groups. CONCLUSION: Plasma NT-proBNP levels were high in cirrhotic patients and are likely to be related to the severity of disease. Advanced cirrhosis is associated with advanced cardiac dysfunction, and NT-proBNP levels has predictive value for concomitant cardiac dysfunction and cirrhosis progression.
Adult ; Aged ; Electrocardiography ; Female ; Heart Diseases/*blood/complications/*pathology ; Humans ; Liver Cirrhosis/*blood/complications/*pathology ; Male ; Middle Aged ; Natriuretic Peptide, Brain/*blood

Many cases have been reported hemolytic anemia and left ventricular outflow obstruction with systolic anterior motion developing after bioprosthetic valve replacement. We report a case of hemolytic anemia and left ventricular outflow tract obstruction occured after mitral valve repair using Duran ring and were resolved by preservative therapy.
Anemia, Hemolytic* ; Mitral Valve* ; Ventricular Outflow Obstruction

BACKGROUND AND OBJECTIVES: We sought to characterize the lesion characteristics of mitral valve prolapse (MVP), which is being increasingly recognized as a cause of mitral regurgitation (MR) in Koreans SUBJECTS AND METHODS: 497 Patients with MVP that was diagnosed by echocardiography in 13 university-affiliated hospitals from Jan to Dec 2003 were prospectively enrolled in our study. RESULTS: A total of 497 patients (270 males, 54%) were enrolled and their mean age was 52+/-17 years. Grade 4 MR was present in 272 patients (54.7%); grade 3, 2 and 1 MR as present in 30.2%, 10.7% and 4.2%, respectively. MVP of the anterior and posterior mitral leaflet was present in 170 patients (34.2%) and 223 patients (44.9%), respectively; MVP developed in both leaflets in 104 patients (20.9%). In 37 patients (7.4%), MVP developed in all 6 segments of the mitral leaflet and these patients were younger (37+/-14 versus 54+/-16 years, respectively, p<0.05) and had a lower prevalence of chordae rupture and severe MR compared to the other patients. Among the 266 mitral segments showing prolapse in the 132 patients (26.6%) who underwent transesophageal echocardiography, the posterior medial scallop was the most frequently diseased one (26%), and this was followed by the posterior middle scallop (18%), the medial (17%), lateral (14%) and middle (13%) part of the anterior leaflet, and the posterior lateral scallop (12%). Younger patients with a mean age <45 years showed a lower prevalence of single segment prolapse, hypertension, severe MR and chordae rupture compared to the older patients (p<0.001, each). CONCLUSION: The medial part of both mitral leaflets was the predilection site for the development of MVP in Koreans and the lesion characteristics were different according to the patients' age.
Echocardiography* ; Echocardiography, Transesophageal ; Humans ; Hypertension ; Korea* ; Male ; Mitral Valve Insufficiency ; Mitral Valve Prolapse* ; Mitral Valve* ; Pectinidae ; Prevalence ; Prolapse ; Prospective Studies* ; Rupture

BACKGROUND AND OBJECTIVES: Stress induced cardiomyopathy has been reported as reversible left ventricular dysfunction with electrocardiographic changes. Although the exact mechanism of this dysfunction has not been clarified, catecholamine "surge" is suspected as a potential cause of this disease. It has not been undergone the studies about the effect of chronic or recurrent psychological stress on the myocardium. We suspect that reversible ischemic change of myocardium could be induced by chronic or recurrent emotional stress. MATERIALS AND METHOD: The clinical, echocardiographic and angiographic data of 189 patients (72 women) who presented with ischemic symptoms and eletrocardiographic changes were participated. BAI (Beck anxiety inventory) and BDI (Beck Depression inventory) were obtained and analyzed for evaluation of degree of psychological stress. RESULTS: 54 patients who had left ventricular apical wall motion abnormalities without significant angiographical stenosis in the coronary artery were younger than the others with left ventricular wall motion abnormalities and angiographic stenosis. And they increased the BAI and BDI as tools of evaluation of psychological stress (p<0.05). CONCLUSION: Data of this study suggested that psychological stress can be associated with myocardial dysfunction. It can be postulated that psychological stress should be considered as one of the cause of non-coronary myocardial injury.
Anxiety ; Cardiomyopathies ; Constriction, Pathologic ; Coronary Vessels ; Depression ; Echocardiography ; Electrocardiography ; Heart Ventricles* ; Humans ; Myocardium ; Stress, Psychological ; Ventricular Dysfunction, Left

Isolated noncompaction of the ventricular myocardium (INVM) is a rare cardiomyopathy resulting from a failure of normal endomyocardial embryogenesis and it has been categorized as a form of unclassified cardiomyopathy. The disorder is characterized by an excessively prominent trabecular meshwork with deep intertrabecular recesses. Although the disorder is sporadic, familial incidence may occur. Clinical symptoms and prognosis of INVM may differ markedly, and range from an asymptomatic course to a severe cardiac disability. The diagnostic method of choice for IVNM is echocardiography, which reveals multiple prominent trabeculations with deep intertrabecular spaces communicating with the left ventricular cavity in the middle and apical segments of the left ventricle. The authors report a case of INVM in a family in which three adult members (a brother and two sisters) were found to be affected by this disorder. They were all asymptomatic. The diagnosis of the disorder was made first in the 36-year-old brother by transthoracic echocardiography (TTE) and multidetector CT (MD CT), during the process of preoperative evaluation for surgical treatment of low back intervertebral herniated disc. TTE and MD CT showed similar and peculiar findings of INVM. Echocardiographic screening in all first-degree relatives of this patient, in order to identify asymptomatic patients, demonstrated INVM in two elder sisters.
Adult ; Echocardiography ; Heart Defects, Congenital/*diagnosis ; Humans ; Male ; Tomography, X-Ray Computed

BACKGROUND AND OBJECTIVES: The coronary sinus (CS) is a small tubular structure just below the tricuspid valve. The dilatation of the coronary sinus as well as the inferior vena cava and the hepatic vein may provide echocardiographic signs of systemic congestion. The aim of this study was to evaluate the correlation of size of coronary sinus that had abnormal echocardiographic findings with or without congestive heart failure. SUBJECTS AND METHODS: Echocardiographic examinations of coronary sinus were performed on 58 patients (M : F=20 : 38) who had abnormal echocardiographic findings with current or previous symptoms or signs of congestive heart failure (group 1), and on 63 patients (M:F=27:36) who had abnormal echocardiographic findings but that had never had symptoms or signs of heart failure(group 2) and 94 healthy volunteers (control group, M:F=52:42). The CS was mesured using a two-dimensional echocardiogram in the right ventricular inflow view (5-10 mm below the Thebesian valve at end-systolic phase). RESULTS: In the normal control group, the median size of the CS was 6.2+/-1.2 mm, and showed no difference between gender and age. The sizes of the CS in groups 1 and 2 were 9.0+/-2.3 and 6.4+/-1.3 mm, respectively. There were differences between groups 1 and the normal control group (p<0.001), and between groups 1 and 2 (p<0.001), but a slight change between group 2 and the normal control group. In group 1, the size of the coronary sinus was related with the duration of heart failure (r=0.32, p=0.016), but no correlations to body surface area, left ventricle dimension and TR peak velocity were shown. In the presence of heart failure, as diagnosed according to the size of the CS, the predictive value was high when the size of the CS exceeded 8.0 mm, with an accuracy of 84%. In heart failure, the median size of the IVC was 16.6+/-5.4 mm, the variation rate of the IVC during the respiratory cycle was 0.40+/-0.13, and the variation rate of the CS during cardiac cycle was 0.31+/-0.20. The size of the CS was not related with the size of the IVC, but there was an inverse correlation between the size of the IVC and its variation rate (r=-0.434, p=0.037). The size of the IVC was inversely correlated with the variation rate of the CS (r=-0.490, p=0.024). There was a correlation between the variation rate of the CS and that of the IVC (r=0.411, p=0.021). Comparing the groups with and without systolic flow reversal into the CS in congestive heart failure patients with tricuspid regurgitation, in the former there wrer distensions of the CS, IVC and LA dimensions and reductions in the variation rates of the CS. CONCLUSION: The measurement of the size of the CS and the variation rate of CS may provide valuable information concerning the presence and duration of congestive heart failure.
Body Surface Area ; Coronary Sinus* ; Dilatation ; Echocardiography ; Estrogens, Conjugated (USP) ; Healthy Volunteers ; Heart Failure* ; Heart Ventricles ; Heart* ; Hepatic Veins ; Humans ; Tricuspid Valve ; Tricuspid Valve Insufficiency ; Vena Cava, Inferior

BACKGROUND: Coronary stenting is one of effective and well-accepted treatments for coronary artery disease. On the other hand, side branch occlusion (SBO) is a known complication of percutaneous transluminal coronary angioplasty (PTCA) and coronary stenting. Accordingly, this study was designed to determine the incidence, predictors and acute clinical outcomes of SBO. METHODS: Coronary angiographic findings of 45 patients who had total 98 side branches originating from the stented segments were analized before and just after coronary stenting. Bifurcation lesions were divided into 3 types : type 1, type 2, type 3 and each type was subdivided into type A with significant ostial narrowing (diameter stenosis >or=50%) and type B without significant ostial narrowing of side branches. Side branch occlusion was defined as development of total occlusion or morphologic changes from type B to type A or reduction of TIMI flow more than grade 1 compared with pre-stenting flow of side branches. RESULTS: After coronary stenting, SBO occurred in 20 of 98 side branches (20.4%). SBO was significantly related with history of previous myocardial infarction (p=0.02), threatened side branch morphology (p=0.016) and poor pre-stenting flow of side branches (p=0.014). There were no serious clinical events such as myocardial infaction and death associated with acute SBO. CONCLUSION: Acute SBO can be developed in a few stented patients. Signifiant clinical and angiographic predictors of SBO just after coronary stenting were the history of previous myocardial infarction, threatened side branch morphology and poor pre-stenting flow of side branches.
Angioplasty, Balloon, Coronary ; Constriction, Pathologic ; Coronary Artery Disease ; Coronary Stenosis ; Hand ; Humans ; Incidence ; Myocardial Infarction ; Stents*

A case of intravenous leiomyomatosis (IVL) invading the vena cava and extending to the right atrium, a rare benign smooth-muscle tumor, is described. Despite their histological benignity, these lesions have a tendency to metastasize and are closely related to the condition called "benign metastasizing leiomyoma" and "intracaval mass and cardiac extension". A 50-year old woman was admitted to the hospital with dyspnea beginning 6 months ago and previous history of hysterectomy due to uterine myoma. Echocardiography revealed severe tricuspid valve insufficiency and a mobile elongated mass which occupied the inferior vena cava and was extending into the right ventricular cavity. Pelvic ultrasonography and pelvic MRI identified suspicious metastasis to both ovaries and peritoneum. She underwent resection of the cardiac tumor, concomitant tricuspid annuloplasty with a closure of the foramen ovale and both salphyngo-oophorectomy with vaginal stump mass biopsy. All of them could be histologically identified as IVL.
Biopsy ; Dyspnea ; Echocardiography ; Female ; Foramen Ovale ; Heart Atria ; Heart Neoplasms ; Humans ; Hysterectomy ; Leiomyoma* ; Leiomyomatosis ; Magnetic Resonance Imaging ; Middle Aged ; Neoplasm Metastasis ; Ovary ; Peritoneum ; Tricuspid Valve Insufficiency ; Ultrasonography ; Vena Cava, Inferior