Purpose: Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non-ventilator-assisted groups. Methods: We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital. Demographic characteristics, pulmonary function, and echocardiography data were investigated. Results: Fifty-four patients with Duchenne muscular dystrophy were divided into 2 groups: home ventilator-assisted and non-ventilator-assisted. The patients in the home ventilator group were older ($16.25{\pm}1.85years$) than those in the nonventilator group ($14.73{\pm}1.36years$) (P=0.001). Height, weight, and body surface area did not differ significantly between groups. The home ventilator group had a lower seated functional vital capacity ($1,038{\pm}620.41mL$) than the nonventilator group ($1,455{\pm}603.2mL$). Mean left ventricular ejection fraction and fractional shortening were greater in the home ventilator group, but the data did not show any statistical difference. The early ventricular filling velocity/late ventricular filling velocity ratio ($1.7{\pm}0.44$) was lower in the home ventilator group than in the nonventilator group ($2.02{\pm}0.62$. The mitral valve annular systolic velocity was higher in the home ventilator group (estimated ${\beta}$, 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions. Conclusion: Noninvasive ventilator assistance can help preserve cardiac function. Therefore, early utilization of noninvasive ventilation or oxygen may positively influence cardiac function in patients with Duchenne muscular dystrophy.
The incidence of fever complicating percutaneous coronary intervention (PCI) is rare. However, little is known regarding the cause of fever after PCI. Therefore, this study aimed to determine the clinical characteristics of patients with acute myocardial infarction (AMI), with or without fever, after PCI. We enrolled a total of 926 AMI patients who underwent PCI. Body temperature (BT) was measured every 4 hours or 8 hours for 5 days after PCI. Patients were divided into two groups according to BT as follows: BT<37.7℃ (no-fever group) and BT ≥37.7℃ (fever group). The 2 years clinical outcomes were compared subsequently. Fever after PCI was associated with higher incidence of major adverse cardiac events (MACE) (hazard ratio [HR], 1.56; 95% confidence interval [CI], 1.07-2.28; P=0.021), all-cause death (HR, 2.32; 95% CI, 1.18-4.45; P=0.014), cardiac death (CD) (HR, 2.57; 95% CI, 1.02-6.76; P=0.049), and any revascularization (HR, 1.69; 95% CI, 1.02-2.81; P=0.044) than without fever. In women, prior chronic kidney disease, lower left ventricular (LV) ejection fraction, higher LV wall motion score index, white blood cell count, peak creatine kinase-myocardial band level, and longer PCI duration were associated with fever after PCI. Procedures such as an intra-aortic balloon pump, extracorporeal membrane oxygenation, continuous renal replacement therapy, central and arterial line insertion, and cardiopulmonary resuscitation were related to fever after PCI. Fever after PCI in patients with AMI was associated with a higher incidence of MACE, all-cause death, CD, and any revascularization at the 2 years mark than in those without fever.
Bcl-2/adenovirus E1B 19 kDa-interacting protein 3 (BNIP3) is a mitochondrial pro-apoptotic protein that has a single Bcl-2 homology 3 (BH3) domain and a COOH-terminal transmembrane (TM) domain. Although it belongs to the Bcl-2 family and can heterodimerize with Bcl-2, its pro-apoptotic activity is distinct from those of other members of the Bcl-2 family. For example, cell death mediated by BNIP3 is independent of caspases and shows several characteristics of necrosis. Furthermore, the TM domain, but not the BH3 domain, is required for dimerization, mitochondrial targeting and pro-apoptotic activity. BNIP3 plays an important role in hypoxia-induced death of normal and malignant cells. Its expression is markedly increased in the hypoxic regions of some solid tumors and appears to be regulated by hypoxia-inducible factor (HIF), which binds to a site on the BNIP3 promoter. Silencing, followed by methylation, of the BNIP3 gene occurs in a significant proportion of cancer cases, especially in pancreatic cancers. BNIP3 also has a role in the death of cardiac myocytes in ischemia. Further studies of BNIP3 should provide insight into hypoxic cell death and may contribute to improved treatment of cancers and cardiovascular diseases.
Purpose: We investigated whether myocardial SPECT had additional usefulness to clinical, functional or surgical indices for the preoperative evaluation of cardiac risks in noncardiac surgery. Materials and Methods: 118 patients (M: F=66: 52, $62.7{\pm}10.5$ years) were studied retrospectively. Eighteen underwent vascular surgeries and 100 nonvascular surgeries. Rest T1-20l/ stress Tc-99m-MIBI SPECT was performed before operation and cardiac events (hard event: cardiac death and myocardial infarction; soft event: ischemic ECG change, congestive heart failure and unstable angina) were surveyed through perioperative periods ($14.6{\pm}5.6$ days). Clinical risk indices, functional capacity, surgery procedures and SPECT findings were tested for their predictive values of perioperative cardiac events. Results: Perioperative cardiac events occurred in 25 patients (3 hard events and 22 soft events). Clinical risk indices, surgical procedure risks and SPECT findings but functional capacity were predictive of cardiac events. Reversible perfusion decrease was a better predictor than persistent decrease, Multivariate analysis sorted out surgical procedure risk (p=0.0018) and SPECT findings (p=0.0001) as significant risk factors. SPECT could re-stratify perioperative cardiac risks in patients ranked with surgical procedures. Conclusion : We conclude that myocardial SPECT provides additional predictive value to surgical type risks as well as clinical indexes or functional capacity for the prediction of preoperative cardiac events in noncardiac surgery.
Background: Despite recent advances in surgical technique and perioperative care of total anomalous pulmonary venous return (TAPVR), post-repair pulmonary vein stenosis (PVS) remains as a serious complication. We thought that the most important factors of TAPVR repair to prevent PVS were good exposure, proper alignment, and sufficient stoma size. We analyzed our experience retrospectively. Material and Method: Between Jan. 1995 and Feb. 2005, we studied 74 patients diagnosed with TAPVR suitable for biventricular repair. Supra-cardiac type (n=41, 55.4%) was the most common. Mean CPB time, ACC time, and TCA (40.5%, 30/74) time were $92.1{\pm}25.9\;min,\;39.1{\pm}10.6\;min$, and $30.2{\pm}10.7\;min$, respectively. Mean follow-up duration was $41.4{\pm}29.1$ months and follow-up was possible in all patients. Result: The median age and body weight at operation were 28.5 days ($0{\sim}478$ days) and 3.4 kg $(1.4{\sim}9\;kg)$. Early mortality was 4.1% (3/74). Causes of death were pulmonary hypertensive crisis, sepsis, and sudden death. There was PR-PVS in 2 patients (early: 1, late: 1). Both patients were cardiac type TAPVR drained to coronary sinus. Re-operations were done but only one patient survived. Cumulative survival rate in 5 year and percent freedom from PVS were $94.5{\pm}2.7%\;and\;97.2{\pm}2.0%$, respectively. Conclusion: There was no PVS in patients who underwent extra-cardiac anatomosis between LA and CPVC. Therefore it could be said that our principle might be effective in preventing PR-PVS in patients suitable two-ventricle.
We developed an experimental model of brain death using dogs. Brain death was induced by increasing the intracranial pressure (ICP) gradually by continuous Infusion of saline through an epidural Foley catheter in 5 mongrel dogs (weight, 18~22kg). Hemodynamic and electrocardiographic changes were evaluated continuously during the process of brain death and obtained the following results. 1. The average volume and time required to induce brain death was 4.8$\pm$1.0ml and 143.0$\pm$30.9minutes respectively. 2. There was a steady rise of the ICP after starting the constant infusion of saline, and ICP rised continuously until the brain death (122.0$\pm$62.5mmHg). After reaching to the maximal value (125.0$\pm$47.7mmHg) at 30 minutes after brain death, the ICP dropped and remained approximately constant at the slightly higher level than the mean arterial pressure (MAP). 3. MAP showed no change until the establishment of brain death and it declined gradually. The peak heart rate reached to 172.6$\pm$35.3/min at 30 minutes after the brain death. 4. Even though the body temperature and all hemodynamic variables, such as cardiac output, mean pulmonary arterial pressure, left ventricular (LV) end-diastolic pressure and LV maximum + dp/dt, were slightly greater than those of basal state, at the point of brain death, there was no statistically significant change during t e process of brain death. 5. There was no remarkable arrhythmias during the experiment except ventricular premature beats which was observed transiently in one dog at the time of brain death. Hemodynamic changes in the brain death model induced by gradual ICP increment were inconspicuous, and arrhythmias were rarely seen. Hyperdynamic state, which was observed at the point of brain death in another brain death model caused by abrupt ICP increase, was not observed.
Primary cardiac tumors are rare disease and they present nonspecific symptom. They are divided in benign and malignant tumors and require surgical therapy and/or additional therapy. From March 1995 to March 2003, twenty one patients were diagnosed as having primary cardiac tumors. We analysed them retrospectively in terms of various perioperative factors and early and late results. 6 men and 15 women and their average age was 45.44$\pm$18.76. Pathology revealed eighteen benign (fourteen myxoma, two fibroelastoma, one hemangioma and one paraganglioma) disease and three malignant (one angiosarcoma, one mesothelioma and one myxofibrosarcoma) disease. There was one (myxoma) operative mortality and three late death (hemangioma, angiosarcoma and mesothelioma) during additional therapy and follow up. Surgical treatment of primary cardiac tumor is important and sometimes additional therapy is required but the prognosis of malignant cardiac tumor is still very poor.
Operations for cardiac valvular disease has been progressed in various ways. Since 1949 when Lord Russel operated mitral stenosis by closed technique at Johns Hopkins Hospital then much progress has been achieved and that nowadays severely diseased cardiac valve has been replaced by prosthetic valve, which is almost ideal in hemodynamic aspect, but still it has many problems such as thromboembolism, destruction of red blood cell, pressure gradient, and disturbance of left ventricular function, so in case of delicate situations, valve replacement should be decided carefully. Besides prosthetic valve, there are some kinds of reconstructive procedures and these have been resulted in better prognosis than prosthetic valve replacement in selected cases. So, authors have reviewed 61 Cases of cardiac patients who have been operated reconstructive valvular surgery by cardiopulmonary bypass, at Yonsei University, from Jan. 1963 to Mar. 1976. Out of 61 cases, 9 patients were replaced by prosthetic valve and rest of the patients were operated upon in various reconstructive procedures such as commissurotomy, valvotomy, valvuloplasty, and annuloplasty. Twenty cases of congenital heart diseases with valvular lesion, which had been operated for valvular lesion were also included in this statistics. Out of 9 cases of prosthetic valvular replacement five cases of prosthetic valvular replacement was done combined with other reconstructive procedures after attempted valvuloplasty. Comparative prognosis of both procedures are somewhat variable by reporters, average 19% of mortality after reconstructive surgery and 38% of mortality after prosthetic valve replacement in long term results. Most common cause of death in postoperative period was low output syndrome in both cases. It seems that good preoperative evaluation and proper reconstructive surgery will afford good prognosis in selected cardiac valvular diseased patient.
Seventy-three patients with isolated total anomalous pulmonary venous connection the patients associated with other major cardiac anomalies such as single ventricle, DORV[Double Outlet Right Ventricle and large VSD[Ventricular Septal Defect were excluded were underwent surgical repair from January 1980 through October 1993. There were 45 boys and 28 girls. The mean age at operation was 19.9 months[range 6 days to 24 years and mean body weight was 7.1kg[range 2.6kg to 45kg . The anomalous locations of connection were supracardiac in 38, cardiac in 21, infracardiac in 5, and mixed in 9. In 38 patients[52% , the venous drainage was obstructed. The obstruction ratios according to the connection type were as follows: 53%[28/38 in supracardiac, 52%[11/21 in cardiac, 100%[5/5 in infracardiac, 22%[2/9 in mixed type. The associated cardiac anomalies were persistent left SVC[2 , tricuspid valve regurgitation[3 , cor triatriatum[1 , and mitral cleft[1 . And associated noncardiac anomalies were imperforate anus[1 and Neil Weightman syndrome[1 . The operative mortality was 23%. The causes of death were pulmonary hypertensive crisis, perioperative myocardial failure, pneumonia with sepsis, arrhythmia and etc. The statistically significant factors in postoperative mortality were the pulmonary venous obstruction and age [p<0.01 . The operative mortality was high in groups of age under 1 month and pulmonary venous obstruction. The mean follow-up was 27.1 months. There were two late deaths. The first patient was three months old boy with supracardiac type and severe obstructive symptoms. The postoperative echocardiography was showed anastomotic stenosis and reoperations were performed twice but the patients expired due to pneumonia and sepsis. The second patient was three month old boy with supracardiac type and total correction was done and was doing well postoperatively. Eight years later, he expired suddenly due to arrhythmia. But all the other patients were in NYHA Fc I and received no medications. The 5-year survival rate excluding early expired patients is 97.1 $\pm$ 0.03 %. In conclusion, although the operative mortality of total anomalous pulmonary venous connection was relatively high compared to other major cardiac anomalies, we could expect excellent long-term results by early surgical correction.
Cardiovascular diseases (CVDs) are one of the most cause of death around the world and fields of interest for cardiac stem cells. Also, current use of terminally differentiated adult cardiomyocytes for CVDs has limited regenerative capacity therefore any significant cell loss may result in the development of progressive heart failure. Human embryonic stem cells (hESCs) derived from blastocyst-stage embryos spontaneously have ability to differentiate via embryo-like aggregates (endoderm, ectoderm and mesoderm) in vitro into various cell types including cardiomyocyte. However, most effective molecule or optimized condition which can induce cardiac differentiation of hESCs is rarely studied. In this study, we developed both spontaneous and inductive cardiomyocyte-like cells differentiation from hESCs by treatment of induced-factors, 5-azacytidine, BMP-4 and cardiogenol C. On the one hand, spontaneous and inductive cardiomyocyte-like cells showed that cardiac markers are expressed for further analysis by RT-PCR and immunocytochemistry. Interestingly, BMP-4 greatly improved homogeneous population of the cardiomyocyte-like cells from hESCs CHA15 and H09. In conclusion, we verified that spontaneously differentiated cells showed cardiac specific markers which characterize cardiac cells, treated extrinsic factors can manage cellular signals and found that hESCs can undergo differentiation into cardiomyocytes better than spontaneous group. This finding offers an insight into the inductive factor of differentiated cardiomyocytes and provides some helpful information that may offer the potential of cardiomyocytes derived from hESCs using extrinsic factors.
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