High output heart failure due to congenital arteriovenous fistula is a rare disease and is difficult to treat. A 27 year old man, suffering from dyspnea, palpitation, lower extremity edema, and abdominal distension admitted May, 1987. At admission there were continuous thrill and bruit at left supraclavicular and infraclavicular area. The heart sound was irregular and rapid, and grade IV/VI pansystolic murmur was audible at the apex. Chest PA showed marked cardiomegaly with pleural effusion and notching at superior aspect of left 2nd rib. Selective left subclavian artery angiogram showed extensive congenital arteriovenous fistula at the area of left subclavian artery and its branches. After setrial feeding artery embolization and palliative operation, the fistula size decreased much with clinical improvement.
Adult ; Arteries ; Arteriovenous Fistula* ; Cardiomegaly ; Dyspnea ; Edema ; Fistula ; Heart Failure ; Heart Sounds ; Humans ; Lower Extremity ; Pleural Effusion ; Rare Diseases ; Ribs* ; Subclavian Artery* ; Thorax

BACKGROUND: Lidocaine is a well known antiarrhythmic agent. However, recent reports indicate that indocaine has myocardial protective effects on acute myocardial ischemia and reperfusion. The exact mechanism of myocardial protection of lidocaine is still not clearly understood. In this study we intended to assess the effects of lidocaine on high energy phosphate metabolism in cats subjected to myocardial ischemia-reperfusion by using 31P MR spectroscopy. Effect of lidocaine on size of infarct will also be evaluated by 2, 3, 5-triphenyltetrazolium chloride(TTC) staining. METHODS: Twenty-seven cats were used for this study. The animals were divided into three groups : for group 1(n=10) and group 2(n=7), animals were subjected to a 90 min of LAD occlusion followed by a 90 min of reperfusion ; for group 3(n=10), a 20 min of occlusion followed by a 90 min of reperfusion. In group 2 and group 3, lidocaine(5mg/kg/hr) was infused continuously during the occlusion and reperfusion periods with an initial bolus injection(1mg/kg) before ligation of LAD. In-vivo MR spectroscopy was performed on a 4.7T Biospec System(Bruker, Switzerland). A home-made surface coil(diameter : 1.5cm) was used to receive31p signals from the myocardium underwent ischemic and reperfusion damage. RESULTS: Decrease of PCr during ischemic period was not different between each groups : PCr showed less than 30% of the baseline value at L-30 in group 1 and group 2 and at L-20 in group 3. More than 90% recovery of PCr was achieved at R-30 in group 2 and group 3, whereas less than 50% of PCr was recovered in group 1. Decrease of ATP during ischemic period was less pronounced in group 2 than in group 1 : in group 2 ATP depleted down to 25% of the baseline at L-90, whereas in group 1 ATP decreased to 50% of the baseline. Recovery of ATP during reperfusion period was not signiflcant in all three groups. On TTC staining, evidence of infarct was seen in all cases of group 1 : the area of infarct was 12.3+/-2.7% of the left ventricular mass and 23.9+/-6.1% of the area at risk. On the contrary, there was no evidence of infact in any case of group 2 and group 3. CONCLUSION: In this study, we found that lidocaine has myocardial protecitve effects on ischemia-reperfusion in cats. Lidocaine improves high energy phosphorous metabolism during ischemia and reperfusion as well as reduces infarct size.
Adenosine Triphosphate ; Animals ; Cats* ; Ischemia ; Lidocaine* ; Ligation ; Magnetic Resonance Spectroscopy ; Metabolism ; Myocardial Ischemia ; Myocardium ; Polymerase Chain Reaction ; Reperfusion ; Reperfusion Injury

For the purpose of determining the pathogenic role of transforming growth factor-beta1 (TGF-beta1) in the mechanism of chronic rheumatic heart disease, we evaluated the expression of TGF-beta1, proliferation of myofibroblasts, and changes in extracellular matrix components including collagen and proteoglycan in 30 rheumatic mitral valves and in 15 control valves. High TGF-beta1 expression was identified in 21 cases (70%) of rheumatic mitral valves, whereas only 3 cases (20%) of the control group showed high TGF-beta1 expression (p<0.001). Additionally, increased proliferation of myofibroblasts was observed in the rheumatic valves. High TGF-beta1 expression positively correlated with the proliferation of myofibroblasts (p=0.004), valvular fibrosis (p< 0.001), inflammatory cell infiltration (p=0.004), neovascularization (p=0.007), and calcification (p<0.001) in the valvular leaflets. The ratio of proteoglycan to collagen deposition inversely correlated with TGF-beta1 expression in mitral valves (p=0.040). In conclusion, an ongoing inflammatory process, the expression of TGF-beta1, and proliferation of myofibroblasts within the valves have a potential role in the valvular fibrosis, calcification, and changes in the extracellular matrix that lead to the scarring sequelae of rheumatic heart disease.
Adult ; Aged ; Chronic Disease ; Collagen/metabolism ; Female ; Fibrosis ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Mitral Valve/*pathology ; Proteoglycans/metabolism ; Rheumatic Heart Disease/*metabolism/pathology ; Transforming Growth Factor beta1/analysis/*physiology

BACKGROUND: Alpha-1-antitrypsin (A1AT) deficiency is the only established genetic resk factor for emphysema. This study was undertaken to investigate the prevalence of the genotypes of A1AT genotypes in healthy Koreans. METHOD: The study population consisted of 380 healthy Koreans enrolled at the Health Promotion Center in Kyungpook National University Hospital. The polymerase chain reaction (PCR) and restriction fragment length polymorphim (RFLP) for detecting the A1AT variants M1(Ala), M1(Val), M2, S and Z were used. RESULTS: The genotypes of subjects were as follows : M1(Val)/M1(Val), 254(66.8%) ; M1(Val)/M2, 105(27.6%) ; M2/M2, 19 (5.0%) ; and M1(Val)/M1(Ala), 2 (0.5%). There was no case with 'deficiency' alleles such as S and Z found in this study. CONCLUSION: These results suggest that A1AT deficient alleles are either extremely rare or not present in Koreans.
Alleles ; Emphysema ; Genotype* ; Gyeongsangbuk-do ; Health Promotion ; Polymerase Chain Reaction ; Prevalence*

Twenty-one patients undergoing aortic valve replacement for pure aoritic regurgitation were studied retrospectively to evaluate the left ventricular function and internal dimension change before, 1-6 weeks(early postoperative) and 2-36 months after(late postoperative) aortic valve replacement by serial echocardiography. Postoperatively, NYHA function class improved remarkably (from 3.3+/-0.6 to 1.4+/-0.7). Early postoperatively, left ventricular end-diastolic dimension (EDD), left ventricular end-systolic dimension(ESD), left ventricular fractional shortenting(FS) significantly decreased in all patients(7.6+/-1.2cm vs 5.8+/-1.5cm P<0.001, 5.5+/-1.3cm vs 4.7+/-1.3cm P<0.001, 39+/-12% vs20+/-8% P<0.001 respectively). Interventricular septum thickness(IVS) and posterior wall thickness (PW) were slightly thickened before(1.4+/-0.3cm, 1.3+/-0.3cm respectively) and in the early postoperative period (1.3+/-0.4cm, 1.3+/-0.3cm respectively) without significant interval change. Late postoperatively, EDD and ESD decreased significantly (7.8+/-1.2cm vs 5.1+/-0.8cm P<0.01, 5.1+/-1.1cm vs 3.4+/-0.8cm P<0.001. respectively), and FS increased significantly (25+/-9% vs 34+/-9%, P<0.05). Among 3 patients of so called high risk group mentioned by Henry(22,33), ESD and FS improved to normal range in 2 patients, and ESD decreased to 4.4cm and FS increased to 33% in the other one. EDD and ESD decreased significantly in both group I(preoperative ESD<5.5cm) and group II(preoperative ESD<5.5cm), without no decrement difference between two groups, and there was a significant difference of FS decrement between group I and group II at early postoperative period. Preoperative ESD correlated highly with the early postoperative EDD(r=0.89) and ESD(r=0.87) with statistical significance, and moderately high with late postoperative EDD(r=0.45), ESD(r=0.50) and FS(r=0.42) without statistical signiticance. We concluded that there was significant improvement in left ventricular function in pure aortic regurgitation patients postoperatively. Preoperative left ventricular and systolic dimension above 5.5cm and fractional shortenting below 25% are not so reliabel index of poor postoperative prognosis.
Aortic Valve Insufficiency* ; Aortic Valve* ; Echocardiography* ; Humans ; Postoperative Period ; Prognosis ; Reference Values ; Retrospective Studies ; Ventricular Function, Left

The superior vena cava (SVC) syndrome results from the disturbance of blood flow in superior vena cava caused by the obstruction. The most common etiology of this condition is the external compression by a malignant tumor. Other causes include thrombus from a pacemaker, defibrillator or central venous catheters. The conventional treatment is radiation and chemotherapy. Recently stenting has been used as a first-line therapeutic strategy for non-malignant cases with balloon and self-expanding stents. In our report, a 77 year-old woman had the SVC syndrome without identification of an underlying disease. A percutaneous endovascular intervention was performed. The stent was placed successfully but just after the procedure, the venous return immediately increased and acute pulmonary edema developed. The patient improved after intravenous diuretics and oxygen. Here we report our experience and review the medical literatures for the management of the non-malignant SVC syndrome, with percutaneous endovascular intervention and the rare complication of pulmonary edema.
Central Venous Catheters ; Defibrillators ; Diuretics ; Female ; Humans ; Oxygen ; Pulmonary Edema ; Stents ; Thrombosis ; Vena Cava, Superior

BACKGROUND: Cystic fibrosis is a chronic progressive autosomal recessive disorder caused by the CFTR gene mutations. It is quite common in Caucasians, but very rare in Asians. Sweat chloride test is known to be a screening test for the cystic fibrosis due to the fact that electrolyte levels in sweat are elevated in patients. In this study, sweat chloride levels in Korean population were measured and analyzed by using standardized pilocarpine iontophoresis sweat chloride test. METHODS: The sweat chloride test was performed in 47 patients referred to Yondong Severance Hospital from August, 2001 to April, 2007 and 41 healthy volunteers. The sweat chloride tests were conducted according to the CLSI C34-A2 guideline using pilocarpine iontophoresis method, and the chloride concentrations in sweat were measured by mercurimetric titration. RESULTS: Four patients showed sweat chloride concentrations higher than 60 mmol/L. Reference interval was calculated as 1.4-44.5 mmol/L by analysis of the results of healthy volunteers (n=41). Four patients who exhibited high sweat chloride levels, had characteristic clinical features of cystic fibrosis and their diagnoses were confirmed either by repeated sweat chloride test or genetic analysis. CONCLUSIONS: Standardized sweat chloride test can be utilized as a useful diagnostic tool for cystic fibrosis in Koreans. In cases of sweat chloride levels higher than 40 mmol/L, the test should be repeated for the possible diagnosis of cystic fibrosis. All the confirmed Korean cases of cystic fibrosis showed sweat chloride level above 60 mmol/L.
Adolescent ; Adult ; Child ; Child, Preschool ; Chlorides/*analysis/*standards ; Cystic Fibrosis/*diagnosis/genetics ; Female ; Humans ; Infant ; Iontophoresis/methods ; Korea ; Male ; Middle Aged ; Pilocarpine/chemistry ; Sequence Analysis, DNA ; Sweat/chemistry/*secretion

BACKGROUND: Cystic fibrosis (CF) is one of the most common hereditary disorders among Caucasians. The most common mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene have been well established among Caucasian populations. In Koreans, however, there are very few cases of genetically confirmed CF thus far, and the spectrum of mutations seems quite different from that observed in Caucasians. METHODS: In the present study, we describe the cases of 2 Korean CF patients, present sequencing results identifying mutations in their CFTR gene, and summarize the results of CFTR mutational spectrum from previously reported Korean CF patients. The mutations described were identified by performing direct sequencing analysis of the complete coding regions and flanking intronic sequences of the CFTR gene, followed by multiplex ligation-dependent probe amplification (MLPA) analysis in order to detect gene deletions or duplications that could not be identified by a direct sequencing method. RESULTS: Three CFTR mutations were identified in the 2 patients, including p.Q98R, c.2052delA, and c.579+5G>A. In an analysis of 9 Korean CF patients that included the 2 patients presented in this study, p.Q98R mutation was the only recurrently observed mutation with a frequency of 18.8% (3/16 alleles). Furthermore, only one of the mutations (c.3272-26A>G) was found among the 32 common mutations in the screening panel for Caucasians from the Cystic Fibrosis Mutation Database. CONCLUSIONS: Sequencing of the entire CFTR gene followed by MLPA analysis, rather than using the targeted sequencing-based screening panel for mutations commonly found in Caucasian populations, is recommended for genetic analysis of Korean CF patients.
Adult ; Alleles ; Asian Continental Ancestry Group/*genetics ; Cystic Fibrosis/*genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/*genetics ; Female ; Heterozygote ; Humans ; Male ; Mutation ; Republic of Korea ; Sequence Analysis, DNA ; Young Adult

No abstract available.
Drug Therapy, Combination* ; Small Cell Lung Carcinoma*