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: 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

PURPOSE: Apical muscular ventricular septal defects (MVSDs), especially in small infants, can be difficult to manage using surgical and percutaneous closure. An intraoperative perventricular procedure is a good option for closing apical MVSDs in small children with or without associated cardiac anomalies. We evaluated the results of hybrid perventricular closure of apical MVSDs performed using an Amplatzer duct occluder (ADO). METHODS: We retrospectively reviewed the medical records of 5 patients who underwent hybrid perventricular closure of MVSDs with ADOs, from March 2006 to May 2011. The median patient age at the time of the procedure was 12 months (range, 25 days to 25 months), and the median body weight was 9.1 kg (range, 4.3 to 15 kg). Two patients had multiple ventricular septal defects (VSDs; additional perimembranous VSD in 1 patient and multiple MVSDs in the other) and 3 patients had associated cardiac anomalies; complete transposition of the great arteries in 1 patient and an atrial septal defect in 2 patients. All the procedures were performed on beating hearts, exception in 1 case. The ADO selected for the aortic side was at least 1 to 2 mm larger than the largest VSD in the left ventricle side. RESULTS: The procedure was successful in all patients and each device was well positioned. During the median follow-up of 2.4 years, a small residual VSD was noted in 2 patients who had multiple VSDs and no leakage was seen in the other 3 patients. CONCLUSION: Perventricular closure of MVSD with an ADO is a good option for patients with apical MVSD. However, careful manipulation is important, especially in the case of small infants.
Arteries ; Body Weight ; Carotenoids ; Child ; Chimera ; Follow-Up Studies ; Heart ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Humans ; Infant ; Medical Records ; Oxygenases ; Retrospective Studies

PURPOSE: This study investigated the clinical course and prognostic factor of very low birth weight infants (VLBWI) with hemodynamically significant congenital heart defects (CHDs). METHODS: Medical records of 1,098 VLBWI with birth weight <1,500 g who had been admitted to the neonatal intensive care unit of Samsung Medical Center from October 1994 to December 2007 were reviewed retrospectively. The data for these patients with hemodynamically significant CHD (n=33) were compared with those without CHD (n=1,065). RESULTS: The incidence of CHD was 3.0% (33 patients) 7 patients (21%) had CHD combined with the congenital abnormalities or chromosomal disorders. The most common CHD was a ventricular septal defect. The incidence of intrauterine growth retardation was higher in patients with CHD than in patients without CHD (34% vs. 20%), but there were no significant differences in gestational age, birth weight, respiratory distress syndrome, bronchopulmonary dysplasia, necrotizing enterocolitis, severe intraventricular hemorrhage (> or =Gr III), and periventricular leukomalacia. Cardiac surgery was performed on 13 patients (39%). Nine patients received staged operations, and 10 patients received early intervention. The overall mortality in patients who had CHD was higher than in the patients who did not have CHD (27% vs. 16%). In patients with CHD, congenital abnormalities or chromosomal disorders were more important factors for increased mortality (86% vs. 11%) than the degree of complexity of CHD (19% vs. 42%). CONCLUSION: The most important prognostic factors of VLBWI with CHD are the associated congenital abnormalities or chromosomal disorders.
Birth Weight ; Bronchopulmonary Dysplasia ; Chromosome Disorders ; Congenital Abnormalities ; Early Intervention (Education) ; Enterocolitis, Necrotizing ; Fetal Growth Retardation ; Gestational Age ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular ; Hemorrhage ; Humans ; Incidence ; Infant ; Infant, Newborn ; Infant, Very Low Birth Weight ; Intensive Care, Neonatal ; Leukomalacia, Periventricular ; Medical Records ; Prognosis ; Retrospective Studies ; Thoracic Surgery

BACKGROUND/AIMS: Polyethylene glycol (PEG) solution is commonly used for precolonoscopic preparation because of its safety and effectiveness. Sulfate free PEG (SF-PEG) solution was developed to reduce the salty taste by removing the sodium sulfate. The aim of this study was to compare the efficacy and patient compliance of SF-PEG and sodium phosphate (NaP) solutions for preparing the bowel before colonoscopy. METHODS: From February through April in 2007, 534 patients who underwent colonoscopy were prospectively enrolled. The efficacy of bowel cleansing was assessed by a doctor's questionnaire and the patient's compliance was assessed by a patient's questionnaire. RESULTS: There was no significant difference between the groups for the "stools and fluids" assessment of bowel cleansing (2.07 vs. 2.14, respectively, p=0.149). However, SF-PEG was more effective on the "air bubbles" assessment (1.34 vs. 1.71, respectively, p<0.001) and the overall assessment (0.72 vs. 0.91, respectively, p=0.010) than NaP. The patients preferred SF-PEG rather than NaP for "Taste" (1.34 vs. 2.25, respectively, p=0.148). However, the patients significantly preferred NaP rather than SF-PEG for "Quantity" (2.46 vs. 1.18, respectively, p<0.001). CONCLUSIONS: The SF-PEG solution showed more effectiveness for bowel cleansing as compared to the NaP solution. SF-PEG tastes better than NaP, but patients are still required to consume 4 liters for the standard preparing regimen.
Colonoscopy ; Compliance ; Humans ; Patient Compliance ; Phosphates ; Polyethylene ; Polyethylene Glycols ; Prospective Studies ; Sodium ; Sulfates ; Surveys and Questionnaires

BACKGROUND/AIMS: Although colonic diverticular disease is less common in Koreans than in Western people, its incidence has been on the increase in Korea. We investigated the clinical characteristics and related complications of colonic diverticular disease in Koreans. METHODS: We retrospectively reviewed the medical records of 9,006 patients who underwent colonoscopy at the Seoul Paik hospital between July 2002 and January 2008. RESULTS: Of the 9,006 patients, there were 654 cases (7.3%) of colonic diverticulosis (472 men, 182 women). The mean age of the patients was 54.6+/-12.0 years. The right colon was involved in 535 cases, the left colon was involved in 86 cases and both the left and right colon was involved in 33 cases. Among the patients, a single diverticulum was seen in 253 cases and two or more diverticuli were seen in 401 cases. Related complications were diverticulitis (11 cases, 1.7%) and diverticular bleeding (3 cases, 0.5%). CONCLUSIONS: The incidence of colonic diverticular disease in Korea shows an increasing trend. Colonic diverticular lesions are frequently found in the right colon.
Colon ; Colonoscopy ; Diverticulitis ; Diverticulitis, Colonic ; Diverticulosis, Colonic ; Diverticulum ; Diverticulum, Colon ; Hemorrhage ; Humans ; Incidence ; Korea ; Male ; Medical Records ; Retrospective Studies

Hyponatremia associated with the use of both a sodium phosphate (NaP) and polyethylene glycol (PEG) solution for colonoscopy preparation has been reported in patients with impaired renal handling of water. A PEG solution is believed to affect serum electrolytes less than NaP, but the use of a PEG solution can lead to nausea, vomiting, intestinal hyperactivity and dehydration, often resulting in a raised plasma antidiuretic hormone (ADH) concentration. Non-osmolar stimuli such as pain, stress, nausea, and vomiting can stimulate ADH release. We report a case of severe hyponatremic encephalopathy after sulfate-free PEG ingestion for a colonoscopy in a healthy middle-aged woman. We think that physicians should be familiar with the medical history and current medication schedule of patients prior to bowel preparation. The levels of serum electrolytes should be checked in patients with impaired ability to excrete free water and any mental aberrations before undertaking colonoscopy procedures.
Appointments and Schedules ; Colonoscopy ; Dehydration ; Eating ; Electrolytes ; Female ; Handling (Psychology) ; Humans ; Hyponatremia ; Mortuary Practice ; Nausea ; Phosphates ; Plasma ; Polyethylene ; Polyethylene Glycols ; Sodium ; Vomiting

Leiomyosarcoma is a rare tumor of the liver. It usually arises from many other organs including uterus, gastrointestinal tract, retroperitoneum, and soft tissues. Primary hepatic leiomyosarcoma progresses very slowly and is not associated with chronic liver disease. When the tumor is detected early enough to be treated by operation, the prognosis is favorable. While several cases of primary hepatic leiomyosarcoma have been reported in Korea, there was no case associated with acute bleeding. We report a 80-year old male patient with huge primary hepatic leiomyosarcoma, who presented with acute bleeding and IVC obstruction. The patient was treated by embolization and IVC stenting.
Acute Disease ; Aged, 80 and over ; Balloon Occlusion ; Hemorrhage/*diagnosis ; Humans ; Leiomyosarcoma/blood supply/*diagnosis/pathology ; Liver Neoplasms/blood supply/*diagnosis/pathology ; Male ; Stents ; Tomography, X-Ray Computed ; Tumor Markers, Biological

A duodenal duplication cyst is rare congenital anomaly, which accounts for 5% of all gastrointestinal duplication cysts. Most of the duodenal duplication cysts are usually found during infancy or early childhood, and present with obstructive symptoms. The most common clinical manifestations are an intestinal obstruction or, less commonly, hemorrhage, perforation, biliary obstruction or pancreatitis. The traditional treatment of a duodenal duplication cyst has been complete surgical resection, but very few cases of endoscopic treatment of a duodenal duplication cyst have been previously reported recently in the literature. Moreover, endoscopic treatment of a duodenal duplication cyst has not been reported in Korea. We report our first experience of a duodenal duplication cyst, including diagnosis and endoscopic management with a detachable snare.
Diagnosis ; Hemorrhage ; Intestinal Obstruction ; Korea ; Ligation* ; Pancreatitis ; SNARE Proteins*