Although hypertrophic cardiomyopathy (HCM) may cause heart failure, HCM and dilated cardiomyopathy (DCM) are generally recognized as separate diseases. This report describes two cases of maternally inherited familial HCM, which, after pregnancy, rapidly deteriorated to heart failure and cardiac chamber dilatation, resembling DCM. Some members of this family also suffered sudden cardiac death (SCD).
Cardiomyopathy, Dilated ; Cardiomyopathy, Hypertrophic ; Cardiomyopathy, Hypertrophic, Familial* ; Death, Sudden, Cardiac* ; Dilatation ; Heart Failure ; Humans ; Pregnancy*

Transmesenteric hernia is a rare cause of small bowel obstruction and is caused by congenital mesenteric defect or postoperative complication. At first because it is a rare type of internal hernia and also clinical simptoms can be nonspecific or intermittent, it is seldom diagnosed before laparotomy or autopsy. We report 3 cases, 1-, 2-, and 4-year-old children and found a small bowel gangrene caused by a congenital defect of mesentery. We describe autopsy findings, clinical simptoms and clinical diagnosis.
Autopsy* ; Child ; Child, Preschool ; Congenital Abnormalities ; Diagnosis ; Gangrene ; Hernia* ; Humans ; Laparotomy ; Mesentery ; Postoperative Complications

An anomalous origin of the left coronary artery (LCA )from the pulmonary artery (ALCAPA syndrome )or Bland-White-Garland syndrome is a rare congenital cardiac anomaly. We report a 65-year old female patient who presented with atypical chest discomfort. Coronary angiography and three-dimensional multidetector computed tomography coronary angiography demonstrated the ectatic right coronary artery (RCA )arising from the aorta, rich collaterals from the RCA to LCA and the ectatic tortuous LCA that originated from the pulmonary trunk.
Aged* ; Aorta ; Bland White Garland Syndrome ; Collateral Circulation ; Coronary Angiography* ; Coronary Vessel Anomalies ; Coronary Vessels* ; Female ; Humans ; Multidetector Computed Tomography ; Pulmonary Artery* ; Thorax

Cardiac papillary fibroelastoma (CPF) is a rare and benign primary cardiac neoplasm of unknown prevalence. The incidence of CPF in the left ventricle is lower than that in other parts of the heart. A 65-year-old female was referred to our cardiology department for evaluation of a cardiac mass of the left ventricle. Transthoracic echocardiography revealed a 1.8 x 1.7 cm highly mobile round mass attached by a stalk to the apical inferior wall of the left ventricle with an echolucent area. The mass was successfully removed without any postoperative complications and was identified as a CPF.
Aged ; Biopsy ; Cardiac Surgical Procedures ; Echocardiography ; Female ; *Fibroma/diagnosis/surgery ; *Heart Neoplasms/diagnosis/surgery ; Heart Ventricles/pathology ; Humans ; Tomography, X-Ray Computed ; Treatment Outcome

BACKGROUND: Recent studies have demonstrated that the size and shape of the hyperenhanced areas on contrast-enhanced magnetic resonance imaging (ceMRI) were nearly identical to areas of irreversible injury, as defined by histochemical staining. We compared the transmural extent of infarct (TEI), as defined by ceMRI, to the initial ECG findings for acute myocardial infarction (AMI), and we also assessed functional contractility according to TEI. METHODS: 12 patients who presented with their first myocardial infarction underwent cine and ceMRI 4 weeks later after their successful revascularization. TEI and wall thickening were determined by using a 30-segment model. RESULTS: Infarction was observed in 81 (23.9%) segments, of which 46 segments (56.8%) had abnormal wall motion and 35 segments (43.2%) had normal wall motion. Of the 35 segments, 33 (94.3%) had subendocardial infarction. 17 segments had infarct of less than 25% of the wall thickness, and all of them had normal wall motion. On the other hand, 11 segments had infarct of more than 75% of wall thickness, of which 11 (100%) had abnormal wall motion. None of segments with nearly transmural infarction were observed in non ST-elevation AMI. The majority of the segments with infarct had non-transmural infarction (87.5%), even if the segments were in ST-elevation AMI (76.1%). Infarct size, as defined by ceMRI, was strongly correlated with peak CK-MB and Troponin-T (r=0.96, p< 0.001, r=0.91, p< 0.001, respectively). CONCLUSION: TEI defined by ceMRI is inversely related to the contractility after revascularization in AMI. We were able to predict the future contractile function of segments with infarction using ceMRI before revascularization.
Contrast Media/administration & dosage ; Female ; Heterocyclic Compounds/administration & dosage ; Humans ; Magnetic Resonance Imaging, Cine/*methods ; Male ; Middle Aged ; *Myocardial Contraction ; Myocardial Infarction/pathology/*therapy ; *Myocardial Revascularization ; Myocardium/*pathology ; Necrosis ; Organometallic Compounds/administration & dosage

BACKGROUND: Atrial fibrillation (AF) has a tendency to become persistent over time and is known to induce atrial electrical, mechanical and ionic remodeling. However, the underlying mechanisms by which AF persists were not fully determined. The present study was carried out to assess alterations in the gene expression, including the oxidative stress-related genes of atrial myocardial cells in patients with persistent AF, and ultrastructural remodeling, as assessed by electron microscopy (EM) in pacing-induced sustained AF canine models. METHODS: cDNA microarray technique and Western blot studies were performed, with tissue samples (right atrial appendage) from 10 patients, 4 with persistent AF and 6 used as controls, which had undergone coronary artery bypass surgery. Four dogs were subjected to continuous left atrial pacing at 400 bpm for at least 12 weeks to induce AF. One dog in sinus rhythm was used as a control sham operation. Tissue samples (1 mm3) were obtained from 4 sites of both atria for EM examination. RESULTS: Thirty up-regulated and 25 down-regulated gene expressions were observed in the patients with AF. Eight of the up-regulated and 6 of the down-regulated genes were oxidative stress-related, which were confirmed by Western blot analyses. The characteristics of ultrastructural remodeling by persistent AF were: 1) an increased number of minimitochondria, 2) disarrayed myofilaments, 3) rarefaction of myofilaments, 4) disintegrated cristae and alignment in mitochondria and 5) vacuolization. CONCLUSIONS: Persistent AF leads to alterations in the gene expression related to oxidative stress in the atrium, and also results in ultrastructural changes similar to those of an ischemia-reperfusion injury.
Animals ; Atrial Fibrillation* ; Blotting, Western ; Coronary Artery Bypass ; Dogs ; Gene Expression* ; Humans ; Microscopy, Electron ; Mitochondria ; Muscle Cells ; Myofibrils ; Oligonucleotide Array Sequence Analysis ; Oxidative Stress ; Reperfusion Injury

BACKGROUND: Granulocyte stimulating factor (G-CSF) and bone marrow mononuclear cells (BM-MNC) were reported to improve cardiac function after myocardial infarction (MI). This study was to examine their combined beneficial effects and mechanisms of actions in reperfused MI, which have not been verified yet. METHODS:Fifteen pigs were divided into 3 groups after a 1-hour balloon occlusion and reperfusion of the left anterior descending coronary artery. G1 (n=5) was a control, G2 (n=5) was a G-CSF injected group (10 ug/kg/day, from day1 to day7 after MI), and G3 (n=5) was an autologous intracoronary BM-MNC infused group after G-CSF treatment RESULTS:Modified wall motion indices by echocardiography were similar among 3 groups at 24 hours after MI. However, they improved significantly in G2 and G3 at 35days after MI (p<0.05). The percentage of infarct area/left ventricular myocardial area measured from a triphenyltetrazolium chloride (TTC) stain was lower in G3 than in G1 or G2 (p=0.026). The number of vWF-positive vessels and the expressions of vWF and VE cardherin by RT-PCR were higher in G3 and G2 than in G1 (p<0.05). The number of TUNEL-positive cells and bcl2/bax ratio were not significantly different among 3 groups. CONCLUSION: This study suggests that intracoronary BM-MNC infusion with G-CSF treatment in reperfused MI reduced infarct size, improved left ventricular function and prevented ventricular remodeling.
Balloon Occlusion ; Bone Marrow ; Coronary Vessels ; Echocardiography ; Granulocyte Colony-Stimulating Factor ; Granulocytes ; Myocardial Infarction ; Reperfusion ; Swine ; Tetrazolium Salts ; Ventricular Function, Left ; Ventricular Remodeling

PURPOSE: In this multi institutional study, the data of 604 men with clinically localized prostate cancer, who underwent radical prostatectomy, with updated nomograms predicting the pathological stage, were analyzed. MATERIALS AND METHODS: Prostate biopsies and prostatectomy specimens from men treated with radical prostatectomy, obtained between 1990 and 2003, were included. The patient distribution with respect to clinical stage, serum prostate-specific antigen (PSA) and biopsy Gleason score, as well as final pathological findings, including organ-confined disease (OCD), extracapsular extension (ECE), seminal vesicle invasion (SVI), and lymph node metastasis (LNM), were analyzed for the construction of nomograms representing the percent probabilities of each respective pathological outcome. RESULTS: The median serum PSA at the time of surgery and biopsy Gleason score were 9.9ng/ml and 7, respectively. The preoperative serum PSA was 4ng/ml or less in 38 (6.3%) patients and the tumor was impalpable in 292 (48.2%) of patients. The biopsy Gleason scores were 7 and 8 or higher in 186 (30.7%) and 169 (27.9%), respectively. Throughout the clinical stages and PSA ranges, the Gleason score was 7 or higher in more than 50% of patients, but 8-10 in 20-30%. The overall OCD, ECE, SVI and LNM rates were 57.1, 27.8, 10.9 and 4.2%, respectively. CONCLISIONS: A significantly high proportion of prostate cancers arising in Korean men exhibited poor differentiation, with Gleason scores of 7 or higher, regardless of the clinical stage or initial serum PSA. Updated nomograms acknowledging such characteristics have been developed, which may aid in the treatment planning of these individuals.
Biopsy ; Humans ; Lymph Nodes ; Male ; Neoplasm Grading ; Neoplasm Metastasis ; Nomograms* ; Prostate* ; Prostate-Specific Antigen ; Prostatectomy ; Prostatic Neoplasms* ; Seminal Vesicles

BACKGROUND/OBJECTIVES: We compared changes in heart-femoral pulse wave velocity (hfPWV) in response to low sodium and high sodium diet between individuals with sodium sensitivity (SS) and resistance (SR) to evaluate the influence of sodium intake on arterial stiffness. SUBJECTS/METHODS: Thirty-one hypertensive and 70 normotensive individuals were given 7 days of low sodium dietary approach to stop hypertension (DASH) diet (LSD, 100 mmol NaCl/day) followed by 7 days of high sodium DASH diet (HSD, 300 mmol NaCl/day) during 2 weeks of hospitalization. The hfPWV was measured and compared after the LSD and HSD. RESULTS: The hfPWV was significantly elevated from LSD to HSD in individuals with SS (P = 0.001) independently of changes in mean arterial pressure (P = 0.037). Conversely, there was no significant elevation of hfPWV from LSD to HSD in individuals with SR. The percent change in hfPWV from the LSD to the HSD in individuals with SS was higher than that in individuals with SR. Subgroup analysis revealed that individuals with both SS and hypertension showed significant elevation of hfPWV from LSD to HSD upon adjusted analysis using changes of the means arterial pressure (P = 0.040). However, there was no significant elevation of hfPWV in individuals with SS and normotension. CONCLUSION: High sodium intake elevated hfPWV in hypertensive individuals with SS, suggesting that high sodium intake increases aortic stiffness, and may contribute to enhanced cardiovascular risk in hypertensive individuals with SS.
Arterial Pressure ; Diet* ; Hospitalization ; Humans ; Hypertension ; Lysergic Acid Diethylamide ; Pulse Wave Analysis* ; Sodium* ; Sodium, Dietary ; Vascular Stiffness

BACKGROUND/OBJECTIVES: We compared changes in heart-femoral pulse wave velocity (hfPWV) in response to low sodium and high sodium diet between individuals with sodium sensitivity (SS) and resistance (SR) to evaluate the influence of sodium intake on arterial stiffness. SUBJECTS/METHODS: Thirty-one hypertensive and 70 normotensive individuals were given 7 days of low sodium dietary approach to stop hypertension (DASH) diet (LSD, 100 mmol NaCl/day) followed by 7 days of high sodium DASH diet (HSD, 300 mmol NaCl/day) during 2 weeks of hospitalization. The hfPWV was measured and compared after the LSD and HSD. RESULTS: The hfPWV was significantly elevated from LSD to HSD in individuals with SS (P = 0.001) independently of changes in mean arterial pressure (P = 0.037). Conversely, there was no significant elevation of hfPWV from LSD to HSD in individuals with SR. The percent change in hfPWV from the LSD to the HSD in individuals with SS was higher than that in individuals with SR. Subgroup analysis revealed that individuals with both SS and hypertension showed significant elevation of hfPWV from LSD to HSD upon adjusted analysis using changes of the means arterial pressure (P = 0.040). However, there was no significant elevation of hfPWV in individuals with SS and normotension. CONCLUSION: High sodium intake elevated hfPWV in hypertensive individuals with SS, suggesting that high sodium intake increases aortic stiffness, and may contribute to enhanced cardiovascular risk in hypertensive individuals with SS.
Arterial Pressure ; Diet* ; Hospitalization ; Humans ; Hypertension ; Lysergic Acid Diethylamide ; Pulse Wave Analysis* ; Sodium* ; Sodium, Dietary ; Vascular Stiffness