• Childhood Kidney Diseases
• /
• v.10 no.2
• /
• pp.219-227
• /
• 2006

Purpose : We aim to identify the clinical and demographic characteristics in children who underwent renal transplantation(RTx) and to evaluate the influence on growth of RTx in children. Methods : We reviewed 17 medical records of chronic renal failure patients who underwent RTx from April 1992 and June 2004 at Busan Paik Hospital. Age and sex distribution, cause of disease, donor analysis, patient and graft survival rate, and the status of growth after RTx were analysed by retrospective study. Results : Eighteen RTx were performed in 17 patients(8 boys, 9 girls). The mean age at the time of RTx was $15.8{\pm}3.5$ years and the mean duration of dialysis therapy before RTx was $22.4{\pm}18.0$ months. The 1 year and 5 year patient survival rate were each 100%, and the 1 year and 5 year graft survival rate were 88%, 36% respectively. The most common cause of graft failure was chronic rejection. The mean final height of male patients was $162.8{\pm}10.0$ cm(143.0-172.5 cm) and of female patients was $154.5{\pm}12.1$ cm(135.8-160.0 cm). The mean height standard deviation score(Ht SDS) increased after RTx from -1.95 to -1.53 but the increment rate was not statistically significant. Similar changes were noted in individual patient analysis. Also there was no significant difference between the living-related donors and cadaveric donors. Conclusion : Our data shows that even successful RTx rarely results in full growth rehabilitation. To overcome retarded growth in children with chronic renal failure, appropriate combined management of metabolic and nutritional problems, correction of anemia, proper use of recombinant growth hormone therapy, early renal transplantation and shortening of the duration of dialysis would be necessary.

• Journal of the Korean Society of Radiology
• /
• v.82 no.2
• /
• pp.429-434
• /
• 2021

Amyloidosis is a multisystemic disease characterized by the accumulation of abnormal proteins in extracellular spaces in various organs, with frequent involvement of the myocardium. We report a case of a patient who had cardiac amyloidosis with a trend of reduction in native T1 and T2 values and extracellular volume fraction on serial cardiac magnetic resonance imaging after chemotherapy and stem cell transplantation. The native T1 value and the extracellular volume fraction are closely associated with tissue amyloid burden in amyloidosis patients. This case demonstrated that cardiac magnetic resonance imaging may be used as a non-invasive and quantitative biomarker in the treatment monitoring of amyloidosis.

• Journal of Chest Surgery
• /
• v.50 no.5
• /
• pp.317-325
• /
• 2017

Background: Extracorporeal membrane oxygenation (ECMO) has become an important treatment modality in pediatric patients with cardiopulmonary failure, but few studies have been conducted in Korea. Methods: We conducted a retrospective review of pediatric patients younger than 18 years who were placed on ECMO between January 2004 and December 2014 at Samsung Medical Center. Results: We identified 116 children on ECMO support. The overall rate of successful weaning was 51.7%, and the survival to discharge rate was 37.1%. There were 39, 61, and 16 patients on ECMO for respiratory, cardiac, and extracorporeal cardiopulmonary resuscitation, respectively. The weaning rate in each group was 48.7%, 55.7%, and 43.8%, respectively. The survival rate was 43.6%, 36.1%, and 25.0%, respectively. Sixteen patients on ECMO had functional single ventricle physiology; in this group, the weaning rate was 43.8% and the survival rate was 31.3%. Ten patients were on ECMO as a bridge to transplantation (8 for heart and 2 for lung). In patients with heart transplantation, the rate of survival to transplantation was 50.0%, and the overall rate of survival to discharge was 37.5%. Conclusion: An increasing trend in pediatric ECMO utilization was observed. The outcomes were favorable considering the early experiences that were included in this study and the limited supply of specialized equipment for pediatric patients.

• Journal of mucopolysaccharidosis and rare diseases
• /
• v.4 no.1
• /
• pp.14-20
• /
• 2018

Mucopolysaccharidoses (MPSs) are a group of rare inherited metabolic disorders caused by specific lysosomal enzyme deficiencies leading to the sequential degradation of glycosaminoglycans, causing substrate accumulation in various cells and tissues and progressive multiple organ dysfunction. The rare disease medical care team at Mackay Memorial Hospital in Taiwan has been dedicated to the study of MPSs for more than 20 years. Since 1999, more than 50 academic papers focusing on MPSs have been published in international medical journals. Topics of research include the following items regarding MPSs: incidence, natural history, clinical manifestations, gene mutation characteristics, cardiac function, bone mineral density, sleep studies, pulmonary function tests, hearing assessments, percutaneous endoscopic gastrostomy, anesthetic experience, imaging analysis, special biochemical tests, laboratory diagnostics, global expert consensus conferences, prenatal diagnosis, new drug clinical trials, newborn screening, and treatment outcomes. Of these published academic research papers, more than half were cross-domain, cross-industry, and international studies with results in cooperation with experts from European, American and other Asian countries. A cross-specialty collaboration platform was established based on high-risk population screening criteria with the acronym "BECARE" (Bone and joints, Eyes, Cardiac and central nervous system, Abdomen and appearance, Respiratory system, and Ear, nose, and throat involvement). Through this platform, orthopedic surgeons, rheumatologists, ophthalmologists, cardiologists, rehabilitation physicians, gastroenterologists, otorhinolaryngologists, and medical geneticists have been educated with regards to awareness of suspected cases of MPSs patients to allow for a further confirmative diagnosis of MPSs. Because of the progressive nature of the disease, an early diagnosis and early multidisciplinary therapeutic interventions including surgery, rehabilitation programs, symptom-based treatments, hematopoietic stem cell transplantation, and enzyme replacement therapy, are very important.

• Journal of Chest Surgery
• /
• v.56 no.3
• /
• pp.162-170
• /
• 2023

Background: Extracorporeal cardiopulmonary resuscitation (E-CPR) plays an indispensable role when resuscitation fails; however, extracorporeal life support (ECLS) in infants is different from that in adults. The objective of this study was to evaluate the outcomes of E-CPR in infants. Methods: A single-center retrospective study was conducted, analyzing 51 consecutive patients (age <1 year) who received E-CPR for in-hospital cardiac arrest between 2010 and 2021. Results: The median age and body weight was 51 days (interquartile range [IQR], 17-111 days) and 3.4 kg (IQR, 2.9-5.1 kg), respectively. The cause of arrest was cardiogenic in 45 patients (88.2%), and 48 patients (94.1%) had congenital cardiac anomalies. The median conventional cardiopulmonary resuscitation (C-CPR) time before the initiation of ECLS was 77 minutes (IQR, 61-103 minutes) and duration of ECLS was 7 days (IQR, 3-12 days). There were 36 in-hospital deaths (70.6%), and another patient survived after heart transplantation. In the multivariate analysis, single-ventricular physiology (odds ratio [OR], 5.05; p=0.048), open sternum status (OR, 8.69; p=0.013), and C-CPR time (OR, 1.47 per 10 minutes; p=0.021) were significant predictors of in-hospital mortality. In a receiver operating characteristic curve, the optimal cut-off of C-CPR time was 70.5 minutes. The subgroup with early E-CPR (C-CPR time <70.5 minutes) showed a tendency for lower in-hospital mortality tendency (54.5% vs. 82.8%, p=0.060), albeit not statistically significant. Conclusion: If resuscitation fails in an infant, E-CPR could be a life-saving option. It is crucial to improve C-CPR quality and shorten the time before ECLS initiation.

• Journal of Chest Surgery
• /
• v.40 no.8
• /
• pp.552-557
• /
• 2007

Background: B-type natriuretic peptide (BNP) is a cardiac hormone that is primarily synthesized by the ventricular cardiac myocytes. Increased plasma BNP levels have been observed in patients suffering with congestive heart failure, ventricular hypertrophy and myocaridits and also during heart transplantation rejection. We investigated the serum BNP level as a predictive marker for rejection after heart transplantation. Material and Method: To test the usefulness of measuring the BNP level in cardiac transplant patients, consecutive blood samplings for BNP, right ventricular endomyocardial biopsies, hemodynamic measurements and transthoracic echocardiogram were all done in 10 such patients between January 2004 and August 2005 at the Department of Thoracic and Cardiovascular Surgery in Asan Medical Center. Two groups were identified with using the median value: the low BNP group (n=28, BNP: ${\le}290$ pg/mL) and the high BNP group (n=29, BNP: >290 pg/mL). We retrospectively analyzed rejection, the ejection fraction, tricuspid regurgitation, left ventricular hypertrophy, the pulmonary capillary wedge pressure and the right atrial pressure between the 2 groups. Result: There were no differences in age, gender, rejection, the ejection fraction, tricuspid regurgitation, left ventricular hypertrophy and the right atrial pressure between the 2 groups (p>0.05). However, a higher pulmonary capillary wedge pressure and a higher mean pulmonary atrial pressure were observed in the high BNP group (p<0.05). Further, BNP has linear correlation with the pulmonary capillary wedge pressure (r=0.590, p<0.001). Using the cut-off value of 620 pg/mL, the BNP predicted a high PCWP (>12 mmHg) with a sensitivity of 83.3% and a specificity of 91.1% (AUC: $0.900{\pm}0.045$, p<0.001). Conclusion: The BNP level after heart transplantation does not show any significant correlation with rejection, yet it might be a predictive marker of ventricular diastolic dysfunction.

• Journal of Chest Surgery
• /
• v.23 no.3
• /
• pp.438-451
• /
• 1990

Pneumatic total artificial heart[TAH] has been clinically applied for the purpose of permanent or temporary use followed by cardiac transplantation in the patients with end stage heart diseases. In spite of the good durability of the pneumatic TAH, thrombus formation, bleeding and infection resulted in death. The Tomasu heart, which is a type of pneumatic TAH, was used in this study. This model is a modified Jarvik heart and consists of atrial cuffs, outflow vascular grafts and thin-layer seamless diaphragm type of ventricles. Cardiac outputs of the left artificial heart were measured by Donovan`s mock circulation under variable conditions of driving parameters, and an experimental artificial heart implantation was performed in 4 calves to observe the changes of hemodynamic parameters in early postoperative period and hematologic and bio-chemical changes in a long-term survival case. In the mock circulation test, cardiac output of the heart was increased with the increase of the left atrial pressure and left driving pressure. Maximum cardiac output was obtained at the heart rate of 120 to 130/min and percent systole of 40 to 45Zo under the condition of a constant left driving pressure of 180mmHg and left atrial pressure of 10mmHg. During the first 24 hours of TAH pumping, driving pressure ranged from 178$\pm$5mmHg to 187$\pm$8mmHg for the left heart and from 58$\pm$6mmHg to 78$\pm$28mmHg for the right heart. The Mean arterial pressure significantly increased between 2 and 8 hours after the start of pumping. The survival time ranged from 27 hours to 46 days. The causes of death were respiratory failure in 2 cases, mechanical valve failure in one, and left ventricular outflow obstruction due to thrombus in a 46-day survival case. This study demonstrated that Tomasu artificial heart operated effectively during the first 24 hours of artificial heart pumping, but thrombus formation around the valve holding area was the main problem in long-term survival case.

• Korean Journal of Radiology
• /
• v.24 no.6
• /
• pp.512-521
• /
• 2023

Objective: There is increasing recognition that left atrial (LA) strain can be a prognostic marker of various cardiac diseases. However, its prognostic value in acute myocarditis remains unclear. Therefore, this study aimed to evaluate whether cardiovascular magnetic resonance (CMR)-derived parameters of LA strain can predict outcomes in patients with acute myocarditis. Materials and Methods: We retrospectively analyzed the data of 47 consecutive patients (44.2 ± 18.3 years; 29 males) with acute myocarditis who underwent CMR in 13.5 ± 9.7 days (range, 0-31 days) of symptom onset. Various parameters, including feature-tracked CMR-derived LA strain, were measured using CMR. The composite endpoints included cardiac death, heart transplantation, implantable cardioverter-defibrillator or pacemaker implantation, rehospitalization following a cardiac event, atrial fibrillation, or embolic stroke. The Cox regression analysis was performed to identify associations between the variables derived from CMR and the composite endpoints. Results: After a median follow-up of 37 months, 20 of the 47 (42.6%) patients experienced the composite events. In the multivariable Cox regression analysis, LA reservoir and conduit strains were independent predictors of the composite endpoints, with an adjusted hazard ratio per 1% increase of 0.90 (95% confidence interval [CI], 0.84-0.96; P = 0.002) and 0.91 (95% CI, 0.84-0.98; P = 0.013), respectively. Conclusion: LA reservoir and conduit strains derived from CMR are independent predictors of adverse clinical outcomes in patients with acute myocarditis.

• Journal of Chest Surgery
• /
• v.22 no.1
• /
• pp.1-9
• /
• 1989

We have performed one case of autotransplantation and 11 cases of orthotopic homotransplantation using Korean mongrel dogs, and have scrutinized the hematologic and hemodynamic results. The mean weight of recipients was 15.42*1.2kg and varied from 14kg to 20kg. During the operation, anesthesia and other technical procedures including cardiopulmonary bypass were similar to the usual methods in human cardiac transplantation. It was found that the hematologic values were similar to those of human beings although there was wide variance. Hemodynamically the mean systolic and diastolic arterial pressures were 165.0* 12.9 mmHg and 100.0 *11.8 mmHg respectively, and the mean heart rate was 155.5*23.5/min. All cases died within 24hrs, and the mean survival in all but 6 cases where operative death occurred was 6.83*8.01 hrs[range 2-21 hrs]. The major causes of deaths were bleedings in 7 cases, failure to protect myocardium during the procedure in 2 cases, pulmonary edema in 1 case and multiorgan failure in 2 cases. From the above results we concluded that the most frequent complication was bleeding, and the cardiopulmonary bypass flow of 50-500ml/kg min was not suitable to the dog in CPB. In further experiment after this study, the technical and the bypass flow was increased. Bleeding was not significant. And the immunosuppresion during operation and postoperative period was tried.

• Journal of Chest Surgery
• /
• v.45 no.2
• /
• pp.116-119
• /
• 2012

A 61-year-old female patient was diagnosed with dilated cardiomyopathy with severe left ventricle dysfunction. Two days after admission, continuous renal replacement therapy was performed due to oliguria and lactic acidosis. On the fifth day, an intra-aortic balloon pump was inserted due to low cardiac output syndrome. Beginning 4 days after admission, she was supported for 15 days thereafter with an extracorporeal left ventricular assist device (LVAD) because of heart failure with multi-organ failure. A heart transplant was performed while the patient was stabilized with the LVAD. She developed several complications after the surgery, such as cytomegalovirus pneumonia, pulmonary tuberculosis, wound dehiscence, and H1N1 infection. On postoperative day 19, she was discharged from the hospital with close follow-up and treatment for infection. She received follow-up care for 10 months without any immune rejection reaction.