BACKGROUND AND OBJECTIVES: We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis. SUBJECTS AND METHODS: We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014. RESULTS: Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age. CONCLUSION: Patients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.
Arrhythmias, Cardiac ; Arteriovenous Fistula ; Biliary Atresia ; Follow-Up Studies ; Heart ; Heart Diseases ; Heterotaxy Syndrome ; Humans ; Isomerism* ; Medical Records ; Mortality ; Prognosis ; Survival Rate

PURPOSE: We evaluated the hemodynamic statuses of patients after partial closure of atrial septal defects with fenestration due to pulmonary hypertension. MATERIALS AND METHODS: Seventeen adult patients underwent partial atrial septal defect closure and follow-up cardiac catheterization. We analyzed hemodynamic data and clinical parameters before and after closure. RESULTS: The median age at closure was 29 years old. The baseline Qp/Qs was 1.9+/-0.6. The median interval from the operation to the cardiac catheterization was 27 months. The CT ratio decreased from 0.55+/-0.07 to 0.48+/-0.06 (p<0.05). The mean pulmonary arterial pressure decreased from 50.0+/-11.5 mm Hg to 32.5+/-14.4 mm Hg (p<0.05), and the pulmonary resistance index decreased from 9.2+/-3.6 Wood units*m2 to 6.3+/-3.8 Wood units*m2 (p<0.05). Eleven patients (64.7%) continued to exhibit high pulmonary resistance (over 3.0 Wood units*m2) after closure. These patients had significantly higher pulmonary resistance indices and mean pulmonary arterial pressures based on oxygen testing before the partial closures (p<0.05). However, no significant predictors of post-closure pulmonary hypertension were identified. CONCLUSION: Despite improvement in symptoms and hemodynamics after partial closure of an atrial septal defect, pulmonary hypertension should be monitored carefully.
Adult ; Aged ; Cardiac Catheterization/*adverse effects ; Female ; Follow-Up Studies ; Heart Defects, Congenital/epidemiology/*physiopathology/*surgery ; Heart Septal Defects, Atrial/surgery ; Hemodynamics/*physiology ; Humans ; Hypertension, Pulmonary/diagnosis/epidemiology/*physiopathology ; Male ; Middle Aged ; Oxygen ; Postoperative Complications/diagnosis/epidemiology/*physiopathology ; Retrospective Studies ; Treatment Outcome ; Young Adult

BACKGROUND: For more effective and safer usage of antibiotics, the dosing strategy should be individualized based on the patients’ characteristics, including race. The aim of this study was to investigate the population pharmacokinetic (PK) profiles of piperacillin and tazobactam in Korean patients with acute infections. MATERIALS AND METHODS: At least four consecutive 2/0.25 g or 4/0.5 g doses of piperacillin/tazobactam (TZP) were intravenously infused over 1 h every 8 h for patients with creatinine clearance (CL(cr)) ≤50 ml/min or CL(cr) >50 mL/min, respectively. Blood samples from 33 patients at a steady-state were taken pre-dose and at 0 min, 30 min, and 4-6 h after the fourth infusion. The population PK analysis was conducted using a non-linear mixed-effects method. A likelihood ratio test was used to select significant covariates, with significance levels of P <0.05 for selection and P <0.01 for elimination. RESULTS: Both piperacillin PK and tazobactam PK were well described by a two-compartment model with first-order elimination. Creatinine clearance and body weight, as covariates on clearance (CL) and volume of central compartment (V1), were selected among the covariates possibly affecting PK parameters of both drugs. CL was defined as CL = 2.9 + 4.03 × CL(cr)/47 for piperacillin and CL = 1.76 + 4.81 × CL(cr)/47 for tazobactam. V1 was defined as V1 = 19.5 × weight/60 for piperacillin and V1 = 22.6 × weight/60 for tazobactam. CONCLUSION: The PK profiles of TZP at a steady-state in Korean patients with acute infections were well described by a two-compartment model with first-order elimination. Both piperacillin and tazobactam clearances were significantly influenced by creatinine clearance.
Anti-Bacterial Agents ; Body Weight ; Continental Population Groups ; Creatinine ; Humans ; Methods ; Piperacillin

BACKGROUND AND OBJECTIVES: Milrinone is often used in children to treat acute heart failure and prevent low cardiac output syndrome after cardiac surgery. Due to the lack of studies on the long-term milrinone use in children, the objective of this study was to assess the safety and efficacy of the current patterns of milrinone use for > or =3 days in infants and children with heart diseases. SUBJECTS AND METHODS: We retrospectively reviewed the medical records of patients aged <13 years who received milrinone for > or =3 days from January 2005 to December 2012. Patients' characteristics including age, sex, height, weight, and body surface area were recorded. The following parameters were analyzed to identify the clinical application of milrinone: initial infusion rate, maintenance continuous infusion rate, total duration of milrinone therapy, and concomitantly infused inotropes. The safety of milrinone was determined based on the occurrence of adverse events such as hypotension, arrhythmia, chest pain, headache, hypokalemia, and thrombocytopenia. RESULTS: We assessed 730 admissions (684 patients) during this period. Ventricular septal defects were the most common diagnosis (42.4%) in these patients. Milrinone was primarily used after cardiac surgery in 715 admissions (97.9%). The duration of milrinone treatment varied from 3 to 64.4 days (> or =7 days in 149 admissions). Ejection fraction and fractional shortening of the left ventricle improved in patients receiving milrinone after cardiac surgery. Dose reduction of milrinone due to hypotension occurred in only 4 admissions (0.5%). Although diverse arrhythmias occurred in 75 admissions (10.3%), modification of milrinone infusion to manage arrhythmia occurred in only 3 admissions (0.4%). Multivariate analysis indicated that the development of arrhythmia was not influenced by the pattern of milrinone use. CONCLUSION: Milrinone was generally administered for > or =3 days in children with heart diseases. The use of milrinone for > or =3 days was effective in preventing low cardiac output after cardiac surgery when combined with other inotropes, suggesting that milrinone could be safely employed in pediatric patients with heart diseases.
Arrhythmias, Cardiac ; Body Surface Area ; Cardiac Output, Low ; Chest Pain ; Child ; Diagnosis ; Headache ; Heart Diseases* ; Heart Failure ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Humans ; Hypokalemia ; Hypotension ; Infant ; Medical Records ; Milrinone* ; Multivariate Analysis ; Off-Label Use* ; Retrospective Studies ; Thoracic Surgery ; Thrombocytopenia

BACKGROUND AND OBJECTIVES: We hypothesized that fetal echocardiography (echoCG) is an accurate diagnostic tool reflecting well postnatal echoCG findings and outcomes. SUBJECTS AND METHODS: We reviewed the medical records of 290 pregnant women, including 313 fetuses, who were examined by fetal echoCG at the Seoul National University Children's Hospital from January 2008 through April 2011. RESULTS: The mean gestational age at diagnosis was 26.2+/-5.2 weeks. The mean age of mothers at diagnosis was 31.7+/-3.8 years. We identified indications for fetal echoCG in 279 cases. The most common indication was abnormal cardiac findings in obstetrical screening sonography (52.0%). Among the 313 echoCG results, 127 (40.6%) were normal, 13 (4.2%) were minor abnormalities, 35 (11.2%) were simple cardiac anomalies, 50 (16.0%) were moderate cardiac anomalies, 60 (19.2%) were complex cardiac anomalies, 16 (5.1%) were arrhythmias, and 12 (3.8%) were twin-to-twin transfusion syndrome. The most common congenital heart disease was tetralogy of Fallot (23 fetuses, 15.9%). One hundred forty-eight neonates were examined by echoCG. We analyzed differences between fetal echoCG and postnatal echoCG. In 131 (88.5%) cases, there was no difference; in 15 (10.1%), there were minor differences; and in only 2 (1.4%) cases, there were major differences. CONCLUSION: There is a recent increase in abnormal cardiac findings of obstetric ultrasonography screenings that indicate fetal echoCG. Fetal echoCG is still a good, accurate diagnostic method for congenital heart disease.
Arrhythmias, Cardiac ; Echocardiography ; Female ; Fetofetal Transfusion ; Fetus ; Gestational Age ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Infant, Newborn ; Mass Screening ; Medical Records ; Mothers ; Pregnancy ; Pregnant Women ; Prenatal Diagnosis ; Tetralogy of Fallot ; Ultrasonography, Prenatal

The congenital complete atrioventricular block(CCAVB) is a rare disease, which is the most serious complication of neonatal lupus erythematosus. Newborn with CCAVB are at risk of diminished cardiac output and the subsequent development of congestive heart failure. Transplacental steroid and beta-adrenergic agonist therapies are useful for the first and second degree atrioventricular block. But those therapies are usually not effective for complete atrioventricular block. If the fetus has a complete atrioventricular block, delivery should be considered unless other delivery contraindications. In this situation, early pacemaker implantation surgery can improve the survival rate of patients. We report one case of premature newborn with congenital complete atrioventricular block who is successfully recovered by pacemaker implantation.
Adrenergic beta-Agonists ; Atrioventricular Block ; Cardiac Output ; Fetus ; Heart Failure ; Humans ; Infant, Newborn ; Lupus Erythematosus, Systemic ; Rare Diseases ; Survival Rate

Aortic coarctation is usually diagnosed and repaired in childhood and early adulthood. Survival of a patient with an uncorrected coarctation to more than 70 years of age is extremely unusual, and management strategies for these cases remain controversial. We present a case of a 75-year-old woman who was first diagnosed with aortic coarctation and severe aortic valve stenosis 5 years ago and who underwent a successful one-stage repair involving valve replacement and insertion of an extra-anatomical bypass graft from the ascending to the descending aorta.
Aged ; Aorta, Thoracic ; Aortic Coarctation ; Aortic Valve Stenosis ; Female ; Humans ; Thoracic Surgical Procedures ; Transplants

BACKGROUND AND OBJECTIVES: Vascular rings refer to anomalies of the great arteries that cause respiratory or feeding problems. The purpose of this study was to analyze a series of patients with vascular rings and evaluate associated risk factors for mortality. SUBJECTS AND METHODS: A retrospective review of all patients identified with vascular rings between 1997 and 2010 in the Seoul National University Children's Hospital. RESULTS: Thirty-five patients were diagnosed with vascular rings (median age at diagnosis, 7 months). The vascular rings of 32 patients were confirmed by cardiac computed tomography or magnetic resonance imaging. The types of vascular rings were: a double aortic arch in ten patients, a right aortic arch with persistent left ligamentum arteriosum in seven, an aberrant subclavian artery in seven, a pulmonary sling in eight, and others types in three patients. Eleven patients were asymptomatic. Gastrointestinal and respiratory symptoms were seen in ten and sixteen patients, respectively. Associated cardiovascular anomalies were present in fifteen patients. Twenty patients with definite symptoms underwent surgical treatment. The median interval between diagnosis and operation was 6 days. Four patients eventually died; three deaths were associated with complex heart diseases, and one had pulmonary artery sling with severe tracheal stenosis. Only the presence of a complex heart disease significantly influenced mortality (p=0.002). CONCLUSION: Vascular rings include several types of anomalies, each with a different prognosis and symptoms. The presence of a complex heart disease was significantly associated with mortality. Early diagnosis and timely surgery in symptomatic patients are essential.
Aneurysm ; Aorta, Thoracic ; Arteries ; Cardiovascular Abnormalities ; Deglutition Disorders ; Early Diagnosis ; Heart Diseases ; Humans ; Magnetic Resonance Imaging ; Prognosis ; Pulmonary Artery ; Retrospective Studies ; Risk Factors ; Subclavian Artery ; Tracheal Stenosis

PURPOSE: This study analyzed the type of acute urinary retention (AUR) and evaluated the treatments used, including trial without catheter (TWOC). MATERIALS AND METHODS: This study was based on 299 patients who were treated for AUR from January 2007 to August 2009. The patients were classified into the spontaneous AUR group (group S) and the precipitated AUR group (group P), in which AUR was consecutive to triggering events. The treatment modalities including TWOC, the success rate of TWOC, age, prostate-specific antigen (PSA) levels, the volume of the prostate, and the drained volume at catheterization were analyzed in each group. RESULTS: Of 299 men with AUR, 160 (54%) had spontaneous AUR and 139 (46%) had precipitated AUR. Compared with group P, patients in group S were more likely to be treated by surgery, either immediately (16.9% vs. 3.6%, p<0.05) or after prolonged catheterization (42.2% vs. 29.1%, p<0.05). The success rate of TWOC was lower in men of older ages (> or =70 years) and in those with enlarged prostates (> or =50 ml), higher PSA levels (> or =3 ng/ml), and a large drained volume at catheterization (> or =1,000 ml). CONCLUSIONS: In this group of AUR patients, there were slightly more patients with spontaneous AUR (54%) than with precipitated AUR (46%). The success rate of TWOC was more than 70% regardless of the type of AUR. Although TWOC is recommended primarily in the treatment of AUR, early surgical intervention should be considered if the patient has an enlarged prostate (> or =50 ml) or a large drained volume at catheterization (> or =1,000 ml).
Catheterization ; Catheters ; Humans ; Male ; Prostate ; Prostate-Specific Antigen ; Prostatic Hyperplasia ; Urinary Catheterization ; Urinary Retention

BACKGROUND: Although patients with tuberculous-destroyed lung (TDL) account for a significant proportion of those with chronic airflow obstruction, it is difficult to distinguish patients with airway obstruction due to TDL from patients with pure chronic obstructive pulmonary disease (COPD) on initial presentation with dyspnea. We investigated clinical features differing between (i) patients with TDL and airway obstruction and (ii) those with COPD admitted to the intensive care unit (ICU) due to dyspnea. METHODS: We reviewed the medical records of patients with TDL who had a forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) of <70% on a pulmonary function test (PFT; best value closest to admission) and patients with COPD without a history of pulmonary tuberculosis (TB) who were admitted to the ICU. Ultimately, 16 patients with TDL and 16 with COPD were compared, excluding patients with co-morbidities. RESULTS: The mean ages of the patients with TDL and COPD were 63.7 and 71.2 years, respectively. Mean FVC% (50.4% vs. 71.9%; p<0.01) and mean FEV1% (39.1% vs. 58.4%; p<0.01) were significantly lower in the TDL group than in the COPD group. More frequent consolidation with TB (68.8% vs. 31.3%; p=0.03) and more tracheostomies (50.0% vs. 0.0%; p=0.02) were observed in the TDL than in the COPD group. CONCLUSION: Upon ICU admission, patients with TDL had TB pneumonia more frequently, more diminished PFT results, and more tracheostomies than patients with COPD.
Airway Obstruction ; Dyspnea ; Forced Expiratory Volume ; Humans ; Intensive Care Units ; Lung ; Lung Diseases, Obstructive ; Medical Records ; Pneumonia ; Pulmonary Disease, Chronic Obstructive ; Respiratory Function Tests ; Tracheostomy ; Tuberculosis, Pulmonary ; Vital Capacity