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

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

BACKGROUND AND OBJECTIVES: Angiotension II, the active component in the renin angiotensin system, modulates blood pressure via vasoconstriction and sodium retention. In normal subjects, the active level of the RAS is reflected by the plasma renin activity (PRA). Importantly, when blood pressure is elevated, the level of RAS is reactively suppressed, and that of PRA approaches zero. Therefore, this study was conducted to find the characteristics of hypertensive patients according to the level of circulating renin. SUBJECTS AND METHODS: The subjects were 275 essential hypertensive patients, with a mean age of 53.2+/-11.9 years, 9.5% of which had diabetes. Patients were classified into 3 groups: low, normal and high renin (high PRA>4.5 ng/mL/h, normal PRA: 0.75-4.5 ng/mL/h and low PRA<0.75 ng/mL/h). The relationship between the PRA and other parameters, such as plasma aldosterone, SBP, DBP, heart rate, lipid profile, body mass index (BMI) and left ventricular mass index (LVMI), were compared. The mean SBP, DBP, heart rate, lipid profile, BMI and LVMI values were compared between the 3 groups. The distribution of the PRA value was compared by sex, age, diabetes, dyslipidemia and obesity. RESULTS: The percentages of the study population with low renin essential hypertension (LREH), normal renin essential hypertension (NREH) and high renin essential hypertension (HREH) were 47, 46 and 8%, respectively. A greater proportion of female patients showed LREH (58.6 vs. 36.1% of male), as did elderly (>55 year-old) compared to younger patients (58.7 vs. 36.2%, both p<0.001). However, there were no significant differences in the hemodynamics (SBP, DBP and heart rate), LVMI and cardiovascular risk factors (obesity, dyslipidemia and diabetes) between the 3 groups. CONCLUSION: Women and elderly people are more likely to have LREH compare to men and younger people, who are more likely to have HREH.
Aged ; Aldosterone ; Blood Pressure ; Body Mass Index ; Dyslipidemias ; Epidemiology ; Female ; Heart ; Heart Rate ; Hemodynamics ; Humans ; Hypertension ; Male ; Obesity ; Plasma* ; Renin* ; Renin-Angiotensin System ; Risk Factors ; Sodium ; Vasoconstriction

Epithelioid hemangioendothelioma (EHE) is a well-differentiated and rare vascular tumor. Systemic metastases are uncommon. Herein, we present a patient with skin metastasis of pulmonary EHE (PEH) that was treated by wide excision. A 76-year-old male was evaluated due to pulmonary thromboembolism and a solitary pulmonary nodule. A biopsy was performed and pathological examination of the mass confirmed EHE. No metastasis was observed. The patient returned to care approximately two years later due to a painful nodule in the right lower leg. A skin biopsy showed metastatic EHE from the lung. We used a safety margin of 1 cm based on clinical experience, because no prior case had been reported regarding the resection margin appropriate for primary cutaneous EHE and skin metastases of PEH. At four months after surgery, the patient recovered without complications or recurrence. Skin metastasis of PEH is extremely rare, and only two cases have been reported in the literature. In this case, we report a rare case of PEH with histologically diagnosed skin metastasis that was successfully treated by curative resection. It is expected that this case report will provide a helpful contribution to the extant data regarding PEH metastases.
Aged ; Biopsy ; Dermatologic Surgical Procedures ; Hemangioendothelioma, Epithelioid* ; Humans ; Leg ; Lung ; Male ; Neoplasm Metastasis* ; Pulmonary Embolism ; Recurrence ; Skin* ; Solitary Pulmonary Nodule

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/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

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