PURPOSE: The objectives of this study were to estimate myocardial functions in patients with chronic renal failure by pulsed Doppler echocardiography, and investigate correlation between Doppler and disease parameters. METHODS: We studied 13 patients with chronic renal failure whose creatinine clearance rate less than 25ml/min. Using pulsed Doppler echocardiography with curve following method, we measured peak velocity, mean velocity, velocity-time integral and peak acceleration of aortic and pulmonic flow velocity curves, and peak velocity, mean velocity, area of rapid filling wave in early relaxation phase(E wave) and filling wave by atrial contraction in late relaxation phase(A wave) and A/E ratios of the peak velocity and area. And we compared these parameters with those of the normal controls, and then those with early chronic renal failure(less than 6 months of disease duration) and those with late chronic renal failure(more than 6 months of disease duration). RESULTS: In patients with early chronic renal failure, peak velocity, flow time and area of E wave of the mitral flow velocity curves were significantly decreased, and those of A wave were markedly increased and resulted in marked increase in A/E ratios of the peak velocity and area compared with those of the normal controls. In patients with late chronic renal failure, mitral A/E ratio of the peak velocity continued to be increased, and peak velocity, velocity-time integral and peak acceleration of the aortic flow velocity curves were significantly decreased but pulmonary velocity- time integral was increased markedly compared with those of the normal controls. CONCLUSIONS: Significant increase of mitral A/E ratio in patients with early chronic renal failure and appearance of significant change in aortic flow velocity curves in patients with late chronic renal failure suggest that abnormalities of myocardial relaxation seem to be present in early phase of the chronic renal failure and continued into late phase but abnormalities of myocardial contraction to appear on late phase of the chronic renal failure.
Acceleration ; Creatinine ; Echocardiography, Doppler, Pulsed* ; Humans ; Intussusception ; Kidney Failure, Chronic* ; Myocardial Contraction ; Recurrence ; Relaxation ; Ultrasonography

We report a case of a 13-year-old girl with acute lymphoblastic lymphoma-leukemia, who presented with a cardiac metastasis in the right ventricle, resulting in a pulmonary embolism. At the time of her leukemia diagnosis, a cardiac mass was incidentally found. The differential diagnosis for this unusual cardiac mass included cardiac tumor, metastasis, vegetation, and thrombus. Empirical treatment was initiated, including anticoagulation and antibiotics. She underwent plasmapheresis and was administered oral prednisolone for her leukemia. Five days later, she experienced sudden hemodynamic collapse and required extracorporeal membrane oxygenation insertion and emergency surgery. These interventions proved futile, and the patient died. Pathology revealed that the cardiac mass comprised an aggregation of small, round, necrotic cells consistent with leukemia. This is the first known case of acute lymphoblastic leukemia presenting as a right ventricular mass, with consequent fatal acute pulmonary embolism. A cardiac mass in a child with acute leukemia merits investigation to rule out every possible etiology, including vegetation, thrombus, and even a mass of leukemic cells, which could result in the fatal complication of pulmonary embolism.
Adolescent ; Anti-Bacterial Agents ; Child ; Diagnosis, Differential ; Emergencies ; Extracorporeal Membrane Oxygenation ; Heart Neoplasms ; Heart Ventricles ; Hemodynamics ; Humans ; Leukemia ; Neoplasm Metastasis ; Plasmapheresis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prednisolone ; Pulmonary Embolism ; Thrombosis

Proximal interruption of unilateral pulmonary artery is an uncommon developmental anomaly. Patients without associated cardiac defects may remain asymptomatic but many develop recurrent pulmonary infections, hemoptysis, pneumonitis, bronchiectasis or pulmonary hypertension. We herein reported two cases of interruption of right pulmonary artery which were diagnosed beyond infancy. A remnant of patent ductus arteriosus was revealed on an angiogram in both cases. Surgical intervention could not be considered due to severe hypoplastic right pulmonary artery. Early surgical intervention should be considered to rescue a pulmonary circulation and prevent secondary lung hypoplasia.
Bronchiectasis ; Ductus Arteriosus, Patent* ; Hemoptysis ; Humans ; Hypertension, Pulmonary ; Lung ; Pneumonia ; Pulmonary Artery* ; Pulmonary Circulation

PURPOSE: The aim of our study was to determine the clinical course and the prognosis of pulmonary hypertension (PHT) with bronchopulmonary dysplasia (BPD). METHODS: Two hundred forty infants who were admitted to the neonatal intensive care unit (NICU) of Samsung Medical Center from January 2002 to December 2006 and were diagnosed with BPD were enrolled in this study. We investigated their medical records retrospectively to investigate any difference between BPD with PHT group and BPD without PHT group in clinical characteristics, mortality and morbidity. RESULTS: Nineteen (7.9%) of the 240 patients with BPD developed PHT. The ratio of females to males was 2.8:1. The severity of BPD, the small birth weight for gestational age, the duration of mechanical ventilation and the maximal peak inspiratory pressure were significantly associated with the development of PHT. (P=0.000, P=0.007 and P=0.000, respectively) The mortality was higher in the BPD with PHT group than in the BPD without PHT group. (P= 0.000) BPD with PHT group required longer duration of oxygen therapy and had more rehospitalization for respiratory illness than the others. (P=0.014) More patients were admitted to the pediatric intensive care unit and received mechanical ventilation therapy in the BPD with PHT group. (P=0.001 and P=0.020, respectively) CONCLUSIONS: PHT is one of the causes associated with high mortality and morbidity in BPD patients. Therefore, the physician should be alert to the development of PHT in premature infants with severe BPD, especially if the baby is female, less than 500 g of birth weight, small for gestational age, or supported by mechanical ventilation for a prolonged duration
Birth Weight ; Bronchopulmonary Dysplasia ; Female ; Gestational Age ; Humans ; Hypertension, Pulmonary ; Infant ; Infant, Newborn ; Infant, Premature ; Infant, Very Low Birth Weight ; Intensive Care Units ; Intensive Care, Neonatal ; Male ; Medical Records ; Oxygen ; Prognosis ; Respiration, Artificial ; Retrospective Studies

PURPOSE: The mechanisms underlying syncope remain unknown in about 20% of patients with recurrent syncope. The implantable loop recorder (ILR) has been shown to be a useful diagnostic tool in patients with unexplained syncope even after negative initial evaluations. Nevertheless, ILR has rarely been used in clinical practice. MATERIALS AND METHODS: This study included 18 consecutive patients who had an ILR implanted at our center because of recurrent unexplained syncope after extensive diagnostic tests between February 2006 and June 2011. RESULTS: Diagnosis was confirmed in 10 (55.6%) of the 18 enrolled patients (13 males, 61+/-15 years). The confirmed diagnoses included sick sinus syndrome (n=6, 60%), advanced atrioventricular block (n=2, 20%) and ventricular tachyarrhythmia (n=2, 20%). The mean follow-up durations of the total study subjects and the diagnosed patients were 11.3+/-10.6 months and 5.6+/-9.2 months, respectively. Of the 10 diagnosed patients, 8 (80%) were diagnosed within 6 months of loop recorder implantation. CONCLUSION: ILR may be a valuable and effective diagnostic tool for patients with unexplained syncope.
Adult ; Aged ; Electrocardiography/instrumentation/methods ; Female ; Humans ; Male ; Middle Aged ; Monitoring, Physiologic/instrumentation/methods ; Syncope/*diagnosis/etiology

BACKGROUND AND PURPOSE: This study assessed the long-term outcomes of disease-modifying therapies (DMTs) in Korean multiple sclerosis (MS) patients treated in real-world clinical settings in Korea. METHODS: We retrospectively evaluated the medical records of 160 patients with an initial diagnosis of clinically isolated syndrome or relapsing-remitting MS who were treated for at least 2 years. A status of 3 for no evidence of disease activity (NEDA3) was defined as no relapse, disability progression, or active lesions in annual magnetic resonance imaging (MRI) evaluations. RESULTS: Patients who were initially treated with interferon β (n=152), glatiramer acetate (n=6), or teriflunomide (n=2) were included. The mean disease duration was 8.2 years. Compared to pretreatment, annualized relapse rates were significantly reduced after treatment [from 1.0±0.8 to 0.2±0.4 (mean±standard deviation), p < 0.001]. At the follow-up, 79 patients (49%) had changed their treatment regimen due to lack of efficacy (33%), side effects (14%), or other reasons (2%). Disability progression was observed in 18% of the patients over a mean treatment duration of 5.7 years. After 2 years, NEDA3 was observed in 38% of the patients. Loss of NEDA3 at 2 years was associated with long-term disability progression [odds ratio (OR)=17.975, p=0.003]. Poor response to first-line treatment was independently associated with a delay in treatment from disease onset (OR=1.238, p=0.049) and 10 or more brain lesions in the initial MRI (OR=3.648, p=0.047). CONCLUSIONS: This study has provided real-world evidence that DMTs are effective in reducing disease activity and disability progression in Korean MS patients.
Brain ; Diagnosis ; Follow-Up Studies ; Glatiramer Acetate ; Humans ; Interferons ; Korea ; Magnetic Resonance Imaging ; Medical Records ; Multiple Sclerosis* ; Recurrence ; Retrospective Studies

PURPOSE: The purpose of this study was to share our experience of transcatheter closure of small patent ductus arteriosus (PDA) by using an Amplatzer vascular plug (AVP). METHODS: We reviewed the medical records of 20 patients who underwent transcatheter closure at Samsung Medical Center and Sejong General Hospital from January 2008 to August 2012. The size and shape of the PDAs were evaluated by performing angiograms, and the PDA size and the AVP devices size were compared. RESULTS: The mean age of the patients was 54.9+/-45.7 months old. The PDAs were of type C (n=5), type D (n=12), and type E (n=3). The mean pulmonary end diameter of the PDA was 1.7+/-0.6 mm, and the aortic end diameter was 3.6+/-1.4 mm. The mean length was 7.3+/-1.8 mm. We used 3 types of AVP devices: AVP I (n=5), AVP II (n=7), and AVP IV (n=8). The ratio of AVP size to the pulmonary end diameter was 3.37+/-1.64, and AVP size/aortic end ratio was 1.72+/-0.97. The aortic end diameter was significantly larger in those cases repaired with AVP II than in the others (P=0.002). The AVP size did not significantly correlate with the PDA size, but did correlate with smaller ratio of AVP size to aortic end diameter (1.10+/-0.31, P=0.032). CONCLUSION: Transcatheter closure of small PDA with AVP devices yielded satisfactory outcome. AVP II was equally effective with smaller size of device, compared to others.
Cardiac Catheterization ; Ductus Arteriosus ; Ductus Arteriosus, Patent ; Hospitals, General ; Humans ; Medical Records ; Vascular Access Devices

BACKGROUND AND OBJECTIVES: The aims of this study were to determine the early and late outcomes of anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate effectiveness of the hemi-Mustard procedure. SUBJECTS AND METHODS: We conducted a retrospective, single-center study of patients who underwent anatomic repair for ccTGA between July 1996 and December 2013. Sixteen patients were included in the study. The median age at the time of the operation was 3.5 years (range: 0.5-29.7), and the median body weight was 13.3 kg (range: 5.8-54). The median follow-up duration was 7.7 years (range: 0.2-17.4). RESULTS: Atrial switch was achieved using the Mustard procedure in 12 patients (hemi-Mustard procedure in 11) or the Senning procedure in four patients. The ventriculoarterial procedure was performed using the Rastelli procedure in 11 patients and arterial switch in five patients. Six patients underwent tricuspid valvuloplasty. The survival rate was 93.8±6.1%. The rate of freedom from reoperation at 5 years was 92.3±7.4% in the Rastelli group. All patients except one were New York Heart Association class I. All patients except one had mild tricuspid regurgitation. CONCLUSION: Anatomic repair can be performed with a low risk of in-hospital mortality. The hemi-Mustard strategy for selected patients is one solution for reducing early mortality and morbidity, and long-term complications such as venous pathway stenosis or sinus node dysfunction.
Arterial Switch Operation ; Arteries* ; Body Weight ; Constriction, Pathologic ; Follow-Up Studies ; Freedom ; Heart ; Heart Defects, Congenital ; Hospital Mortality ; Humans ; Mortality ; Reoperation ; Retrospective Studies ; Sick Sinus Syndrome ; Survival Rate ; Tricuspid Valve Insufficiency

BACKGROUND: Surgical repair of a partial anomalous pulmonary venous connection (PAPVC) to the superior vena cava (SVC) may be complicated by sinus node dysfunction or SVC obstruction. We modified the Warden procedure by using a right atrial auricular flap to decrease the occurrence of these complications. METHODS: Between February 2005 and July 2012, 10 consecutive patients underwent a modified Warden procedure to correct PAPVC. The median patient age was 5.7 years. Eight patients (80%) had an atrial septal defect. To surgically correct the PAPVC, we made a U-shaped incision on the right atrial appendage and sutured the flap to the posterior wall of the SVC. The anterior wall was reconstructed with various patch materials. RESULTS: No early or late deaths occurred, nor did any patient require early or late reoperation for SVC or pulmonary venous obstruction. No new arrhythmias appeared during follow-up, which was complete in all patients (mean, 29.5 months). CONCLUSION: Our modification of the Warden procedure seems to be effective and safe. This technique may lower the risk of SVC obstruction, pulmonary venous obstruction, and sinus dysfunction.
Arrhythmias, Cardiac ; Atrial Appendage* ; Follow-Up Studies ; Heart Septal Defects, Atrial ; Humans ; Reoperation ; Sick Sinus Syndrome ; Vena Cava, Superior

Extrahepatic portosystemic shunts, known as Abernethy malformations, were first reported by John Abernethy in 1793. They are classified into two types: Type I refers to a congenital absence of the portal vein and Type II refers to a shunt involving a side-to-side anastomosis with reduced portal blood flow into the liver parenchyma. This malformation is so rare that less than 100 cases have been reported in the medical literature. We report the case of a 13-month-old boy who had a congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This case was complicated with an atrial septal defect and a large hyperplastic nodule in the liver. The patient was diagnosed with a Type II Abernethy malformation. We planned on surgical occlusion of the extrahepatic portocaval shunt. However, six months later, the patient had a sudden onset of a fever of unknown origin and developed hepatic encephalopathy. Although he underwent a liver transplantation, he died of acute hepatic failure.
Fever of Unknown Origin ; Focal Nodular Hyperplasia ; Heart Septal Defects, Atrial ; Hepatic Encephalopathy ; Humans ; Infant ; Liver ; Liver Failure, Acute ; Liver Transplantation ; Portal Vein ; Portasystemic Shunt, Surgical