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

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

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 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

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

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

BACKGROUND AND OBJECTIVES: In the era of stents, lesion length remains an important predictor of restenosis. Drug-eluting stents (DESs) have significantly reduced in-stent restenosis (ISR), but results in long lesions are still lacking. Therefore, we investigated the impact of DESs on clinical outcomes in patients with diffuse coronary lesions. SUBJECTS AND METHODS: Between January 2004 and January 2005, 80 patients (94 lesions) with lesions >20 mm in length were treated with one or more DESs and underwent follow-up coronary angiography. The patients were divided into three groups: Group 1 was composed of those with lesions 21 to 35 mm in length, Group 2 was composed of those with lesions 36 to 50 mm in length, and Group 3 was composed of those with lesions > or =51 mm in length. RESULTS: The mean clinical follow-up duration was 9 months. On the 6-month follow-up angiogram, 6.4% of the lesions had binary ISR (5.0% in group 1, 8.7% in group 2, and 9.1% in group 3). The percent diameter stenosis was 6.0+/-18.15% in Group 1, 12.61+/-21.99% in Group 2, and 19.81+/-31.26% in Group 3(p< 0.05). Late lumen loss was 0.17+/-0.50 mm in Group 1, 0.39+/-0.66 mm in Group 2, and 0.59+/-0.93 mm in Group 3 (p<0.05). Lesion length was associated with an increase in percent diameter stenosis and late lumen loss (of 6.9% and 0.21 mm per 15 mm). CONCLUSION: DES implantation is considered safe and effective in the treatment of diffuse lesions. However, lesion length may be associated with an increase in percent diameter stenosis and late lumen loss at 6-month follow-up.
Constriction, Pathologic ; Coronary Angiography ; Coronary Restenosis ; Coronary Stenosis ; Drug-Eluting Stents ; Follow-Up Studies ; Humans ; Stents

We reported a case of hemolytic transfusion reaction that was related to multiple RBC antibodies such as anti-E, anti-M, anti-Jkb and anti-Lea after serial RBC transfusions. A forty-nine year old female visited the emergency room (ER) with hematochezia. She had previously received 16 units of packed RBCs from 2003 to Jan 2007 for her intermittent esophageal varix bleeding. No specific antibodies were identified before this visiting. At the ER, under the request for packed RBCs, we identified anti-E antibody within her serum. Her blood type was AB, RhD+ with the phenotype of CcDe. She received 5 units of E antigen negative RBCs. However, she showed hemolytic transfusion reactions such as mild fever with a decrease of hemoglobin from 11.4 g/dL to 6.8 g/dL after the transfusion. From the 8th to the 10th hospital day, another 3 units of E-antigen negative with the least incompatible RBCs were transfused to the patient, but the level of hemoglobin was not definitely increased. At the 14th hospital day, she received a final 2 units of leuko-reduced RBCs without E, M and Jkb antigens. Her hemoglobin was increased right after the final transfusion. We found that the patient's serum reacted with multiple RBC antibodies such as anti-E, anti-M, anti-Jkb and anti-Lea antibodies. She finally recovered from acute varix bleeding and was discharged on the 26th hospital day with the level of hemoglobin being 8.3 g/dL.
Antibodies ; Blood Group Incompatibility ; Emergencies ; Esophageal and Gastric Varices ; Female ; Fever ; Gastrointestinal Hemorrhage ; Hemoglobins ; Hemorrhage ; Humans ; Phenotype ; Varicose Veins

BACKGROUND AND OBJECTIVES: Drug-induced electrocardiographic QT interval prolongation is associated with the occurrence of a potentially lethal form of polymorphic ventricular tachycardia, termed 'torsades de pointes' (TdP). Women are at greater risk for the development of drug-induced TdP. To determine whether this may be the result of gender-specific differences in the effect of quinidine on cardiac repolarization, we compared the degree of quinidine-induced QT interval lengthening in young, healthy volunteers. SUBJECTS AND METHODS: Twelve women and 12 men each received a single intravenous dose of quinidine (4 mg/kg) or placebo in a single-blinded, randomized crossover trial. Total plasma concentrations of quinidine were measured, and QT and corrected QT intervals were analyzed. RESULTS: As expected, the mean QTc interval at baseline was longer for women than for men (443.6+/-26.9 vs 402.1+/-31.3 msec, respectively, p=0.037). The mean value of the maximal DeltaQTc after quinidine infusion was higher in women (134.4+/-46.4 vs 117.5+/-37.7 msec, respectively, p=0.029), and the mean value of the minimal DeltaQTc for 1 hour after quinidine infusion was also higher in the female group (47.6+/-15.7 vs 83.7+/-25.4 msec, p=0.034). However, there were no significant differences in the time courses of the changes in the quinidine-induced QTc and DeltaQTc interval between the two groups (p=0.092, and p=0.305, respectively). CONCLUSION: Quinidine causes greater QT prolongation in women at equivalent serum concentrations. This difference may contribute to the greater incidence of drug-induced TdP observed in women taking quinidine, and has implications for other cardiac and noncardiac drugs that prolong the QTc interval.
Asian Continental Ancestry Group ; Electrocardiography ; Female ; Healthy Volunteers ; Humans ; Incidence ; Male ; Plasma ; Quinidine ; Tachycardia, Ventricular

BACKGROUND: There are several echocardiographic parameters, such as early transmitral velocity/tissue Doppler mitral annular early diastolic velocity(E/Ea) or deceleration time, reported to be reliable indices to estimate pulmonary capillary wedge pressure(PCWP). Recently, B-type natriuretic peptide(BNP) level is also reported to increase in accordance with increased left ventricular filling pressure in systolic or diastolic heart failure. This study was performed to compare E/Ea and BNP for the ability to estimate PCWP. METHODS: Several echocardiographic Doppler parameters including especially E/Ea were obtained from transthoracic Doppler echocardiography. Simultaneously, serum BNP level and PCWP estimated by using Swan-Ganz catheter were obtained, respectively. RESULTS: E/Ea revealed a correlation of r=0.88 (p<0.001) with PCWP compared with r=0.45 (p<0.001) between BNP and PCWP. E/Ea > or =11 was the optimal cutoff to predict PCWP > or =15 mmHg (sensitivity, 94%; specificity, 90%), whereas the optimal BNP cutoff was > or =250 pg/mL (sensitivity, 52%; specificity, 74%). CONCLUSION: Mitral E/Ea has a better correlation with PCWP than BNP. Mitral E/Ea appears more sensitive and specific than BNP for PCWP > or =15 mmHg in cardiac patients.
Capillaries ; Catheters ; Deceleration ; Echocardiography ; Echocardiography, Doppler* ; Heart Failure, Diastolic ; Humans ; Natriuretic Peptide, Brain* ; Pulmonary Wedge Pressure* ; Sensitivity and Specificity