Here we present a rare case of pulmonary arterial thrombosis associated with a ductus arteriosus aneurysm that caused severe pulmonary stenosis. A 5-day-old newborn was admitted to our hospital for the evaluation of an intracardiac mass-like lesion found after the detection of a cardiac murmur. Echocardiography and heart computed tomography revealed a mass-like lesion measuring 8.1 mm in diameter across the distal main pulmonary artery to the proximal left pulmonary artery resulting in localized severe stenosis of the left pulmonary artery. Left pulmonary artery angioplasty for surgical resection of the thrombus revealed that the mass was adherent to the proximal part of the left pulmonary artery anterior wall and extended to the ductus arteriosus. Histological examination of the mass showed an old thrombus with dystrophic calcification. Five months after surgery, follow-up echocardiography showed that the left pulmonary artery peak pressure gradient had decreased but the proximal left pulmonary artery stenosis remained. Cardiac catheterization and balloon angioplasty suc cessfully relieved the pulmonary stenosis.

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: Mechanical circulatory support with extracorporeal membrane oxygenation (ECMO) and ventricular assist device has always been the optimal choice for treating the majority of medically intractable low cardiac output case. We retrospectively investigated our institution's outcomes and variables associated with a high risk of mortality. SUBJECTS AND METHODS: From 1999 to 2014, 86 patients who were of pediatric age or had grown-up congenital heart disease underwent mechanical circulatory support for medically intractable low cardiac output in our pediatric intensive care unit. Of these, 9 grown-up congenital heart disease patients were over 18 years of age, and the median age of the subject group was 5.82 years (range: 1 day to 41.6 years). A review of all demographic, clinical, and surgical data and survival analysis were performed. RESULTS: A total of 45 (52.3%) patients were successfully weaned from the mechanical assist device, and 25 (29.1%) survivors were able to be discharged. There was no significant difference in results between patients over 18 years and under 18 years of age. Risk factors for mortality were younger age (<30 days), functional single ventricle anatomy, support after cardiac operations, longer support duration, and deteriorated pre-ECMO status (severe metabolic acidosis and increased levels of lactate, creatinine, bilirubin, or liver enzyme). The survival rate has improved since 2010 (from 25% before 2010 to 35% after 2010), when we introduced an upgraded oxygenator, activated heart transplantation, and also began to apply ECMO before the end-stage of cardiac dysfunction, even though we could not reveal significant correlations between survival rate and changed strategies associated with ECMO. CONCLUSION: Mechanical circulatory support has played a critical role and has had a dramatic effect on survival in patients with medically intractable heart failure, particularly in recent years. Meticulous monitoring of acid-base status, laboratory findings, and early and liberal applications are recommended to improve outcomes without critical complication rates, particularly in neonates with single ventricle physiology.
Acidosis ; Bilirubin ; Cardiac Output, Low* ; Creatinine ; Critical Care* ; Extracorporeal Membrane Oxygenation ; Heart Defects, Congenital ; Heart Failure ; Heart Transplantation ; Heart-Assist Devices ; Humans ; Infant, Newborn ; Intensive Care Units* ; Lactic Acid ; Liver ; Mortality ; Oxygen ; Oxygenators ; Physiology ; Resuscitation ; Retrospective Studies ; Risk Factors ; Survival Rate ; Survivors

BACKGROUND: Although Escherichia coli is a common cause of bacterial enteritis in Korea, reports on community-acquired E. coli enteritis in Korean children are scarce. This study aimed to determine the clinical characteristics and pathotype distribution of community-acquired E. coli enteritis diagnosed by a multiplex polymerase chain reaction (PCR) assay in Korean children. MATERIALS AND METHODS: The medical records of children aged 18 years or less who were diagnosed with acute gastroenteritis by the attending physician between 2013 and 2016 were retrospectively reviewed. The clinical characteristics of children diagnosed with E. coli enteritis were investigated and compared with those diagnosed with Salmonella enteritis. E. coli and Salmonella infections were diagnosed by a stool PCR assay. RESULTS: Among 279 children, in whom PCR assays for E. coli and Salmonella spp. were performed, Salmonella enteritis and E. coli enteritis were diagnosed in 43 (15.4%) and 39 (14.0%) children, respectively. Among the 39 children with E. coli enteritis, enteropathogenic E. coli (n=21, 53.8%) and enteroaggregative E. coli (n=15, 38.4%) were the most common causative agents. Empirical antibiotics were administered to 33 (84.6%) children. A total of 31 (79.5%) children developed fever, and 25 (80.6%) of them had the fever for 3 days or less, which resolved a median of 1 day (range 0-3 days) after hospitalization. The most frequent gastrointestinal symptom was diarrhea (n=36, 92.3%). Significantly more children with E. coli enteritis were aged 2 years or less as compared with those with Salmonella enteritis (41.0% vs. 21.9%, P = 0.021). Children with Salmonella enteritis more frequently complained of fever (97.7% vs. 79.5%, P = 0.012), abdominal pain (90.7% vs. 64.1%, P = 0.004), and hematochezia (46.5% vs. 10.3%, P < 0.001) than those with E. coli enteritis. Erythrocyte sedimentation rate and C-reactive protein levels were significantly higher in children with Salmonella enteritis than those with E. coli enteritis (P < 0.001). CONCLUSION: Enteropathogenic E. coli was the most frequent pathotype in Korean children with E. coli enteritis that caused mild clinical symptoms. A stool PCR assay for E. coli may be useful for epidemiological purpose and for an early diagnosis of E. coli enteritis.
Abdominal Pain ; Anti-Bacterial Agents ; Blood Sedimentation ; C-Reactive Protein ; Child* ; Diarrhea ; Early Diagnosis ; Enteritis* ; Enteropathogenic Escherichia coli ; Escherichia coli* ; Escherichia* ; Fever ; Gastroenteritis ; Gastrointestinal Hemorrhage ; Hospitalization ; Humans ; Korea ; Medical Records ; Multiplex Polymerase Chain Reaction ; Polymerase Chain Reaction* ; Retrospective Studies ; Salmonella ; Salmonella Infections

While phosphodiesterase type 5 inhibitors have been used for erectile dysfunction with acceptable safety profile, they can induce orthostatic hypotension in patients taking antihypertensive drugs with blood pressure lowering effect. This study evaluated the hemodynamic effects of 100 mg mirodenafil in hypertensive patients taking an amlodipine. Thirteen hypertensive patients who were taking 5 or 10 mg of amlodipine once daily participated in a randomized, double-blind, placebo-controlled, crossover study. A single oral dose of mirodenafil 100 mg or placebo was administered at 4.5 hour after administration of amlodipine. The maximal change in systolic and diastolic blood pressure (ΔmaxSBP and ΔmaxDBP) and pulse rate (ΔmaxPR) were compared between mirodenafil and placebo periods. Twelve patients completed this study and were included analysis. The values of ΔmaxPR in standing and supine position were significantly greater in the mirodenafil period (13.25±7.12 and 11.17±4.86 beats/minute) when compared to the placebo (8.50±4.72 and 6.58±3.90 beats/minute). The ΔmaxSBP and ΔmaxDBP in standing position appeared to be lower in the mirodenafil period, but they were not statistically different from those in the placebo period (ΔmaxSBP = -7.42±5.6 vs -4.42±5.37 mmHg and ΔmaxDBP = -7.17±5.72 vs -3.50±3.37 mmHg). Both ΔmaxSBP and ΔmaxDBP in standing and supine position were not significantly different between mirodenafil and placebo. This study demonstrated that mirodenafil exerted minimal hemodynamic effects in the patients taking amlodipine, that is unlikely associated with a clinically significant hypotensive event.
Amlodipine* ; Antihypertensive Agents ; Blood Pressure ; Cross-Over Studies ; Erectile Dysfunction ; Heart Rate ; Hemodynamics* ; Humans ; Hypotension, Orthostatic ; Male ; Phosphodiesterase 5 Inhibitors ; Posture ; Supine Position

BACKGROUND AND OBJECTIVES: The aim of the study is to evaluate the long-term results after a surgical repair of Ebstein's anomaly. SUBJECTS AND METHODS: Forty-eight patients with Ebstein's anomaly who underwent open heart surgery between 1982 and 2013 were included. Median age at operation was 5.6 years (1 day-42.1 years). Forty-five patients (93.7%) demonstrated tricuspid valve (TV) regurgitation of less than moderate degree. When the patients were divided according to Carpentier's classification, types A, B, C, and D were 11, 21, 12, and 4 patients, respectively. Regarding the type of surgical treatment, bi-ventricular repair (n=38), one-and-a half ventricular repair (n=5), and single ventricle palliation (n=5) were performed. Of 38 patients who underwent a bi-ventricular repair, TV repairs were performed by Danielson's technique (n=20), Carpentier's technique (n=11), Cone repair (n=4), and TV annuloplasty (n=1). Two patients underwent TV replacement. Surgical treatment strategies were different according to Carpentier's types (p<0.001) and patient's age (p=0.022). RESULTS: There were 2 in-hospital mortalities (4.2%; 1 neonate and 1 infant) and 2 late mortalities during follow-up. Freedom from recurrent TV regurgitation rates at 5, 10, and 15 years were 88.6%, 66.3%, 52.7%, respectively. TV regurgitation recurrence did not differ according to surgical method (p=0.800). Survival rates at 5, 10, and 20 years were 95.8%, 95.8%, and 85.6%, respectively, and freedom from reoperation rates at 5, 10, and 15 years were 85.9%, 68.0%, and 55.8%, respectively. CONCLUSION: Surgical treatment strategies were decided according to Carpentier's type and patient's age. Overall survival and freedom from reoperation rates at 10 years were 95.8% and 68.0%, respectively. Approximately 25% of patients required a second operation for TV during the follow-up.
Classification ; Cyanosis ; Ebstein Anomaly* ; Follow-Up Studies ; Freedom ; Heart Defects, Congenital ; Hospital Mortality ; Humans ; Infant, Newborn ; Methods ; Mortality ; Recurrence ; Reoperation ; Survival Rate ; Thoracic Surgery ; Tricuspid Valve

Many complications have been reported during or after anterior cruciate ligament (ACL) reconstruction, including infection, bleeding, tibial tunnel widening, arthrofibrosis, and graft failure. However, arterial injury has been rarely reported. This paper reports a case of an anterior tibial arterial injury during bicortical tibial drilling in arthroscopic ACL reconstruction, associated with an asymptomatic occlusion of the popliteal artery. The patient had a vague pain which led to delayed diagnosis of compartment syndrome and delayed treatment with fasciotomy. All surgeons should be aware of these rare but critical complications because the results may be disastrous like muscle necrosis as in this case.
Adult ; Anterior Cruciate Ligament Reconstruction/*adverse effects ; *Compartment Syndromes ; Fasciotomy ; Humans ; *Iatrogenic Disease ; Male ; Necrosis ; *Postoperative Complications ; Republic of Korea ; Tibia/*surgery ; Tibial Arteries/*injuries

BACKGROUND: A preclinical study was conducted for evaluating a valved conduit manufactured with a glutaraldehyde (GA)-fixed bovine pericardium treated using an anticalcification protocol. METHODS: Bovine pericardia were decellularized, fixed with GA in an organic solvent, and detoxified. We prepared a valved conduit using these bovine pericardia and a specially designed mold. The valved conduit was placed under in vitro circulation by using a mock circulation model, and the durability under mechanical stress was evaluated for 2 months. The valved conduit was implanted into the right ventricular outflow tract of a goat, and the hemodynamic, radiologic, histopathologic, and biochemical results were obtained for 6 months after the implantation. RESULTS: The in vitro mock circulation demonstrated that valve motion was good and that the valved conduit had good gross and microscopic findings. The evaluation of echocardiography and cardiac catheterization demonstrated the good hemodynamic status and function of the pulmonary xenograft valve 6 months after the implantation. According to specimen radiography and a histopathologic examination, the durability of the xenografts was well preserved without calcification at 6 months after the implantation. The calcium and inorganic phosphorus concentrations of the explanted xenografts were low at 6 months after the implantation. CONCLUSION: This study demonstrated that our synergistic employment of multiple anticalcification therapies has promising safety and efficacy in the future clinical study.
Biocompatible Materials ; Bioengineering ; Bioprosthesis ; Calcium ; Cardiac Catheterization ; Cardiac Catheters ; Echocardiography ; Employment ; Fungi ; Glutaral ; Goats ; Heart Valves ; Hemodynamics ; Heterografts ; Pericardium* ; Phosphorus ; Radiography ; Stress, Mechanical

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