Atrial flutter is and infrequent, but potentially unstable tachyarrythmia that occurs in pediatric ages. Transesophageal atrial pacing was used for treatment of 10 episodes of atrial flutter in 7 patients. At the time of atrial flutter conversion, patients were 6 days to 14 years old. 6 patients had associated with congenital heart disease. The atrial cycle length of atrial flutter ranged from 140 to 280 msec with variable atrioventricular conduction. Transesophageal atrial pacing was performed using a bipolar 4 F transesophageal electrode catheter. Atrial flutter conversion was accomplished with stimulation bursts using about 5 seconds of stimuli, 10 msec in duration at 20 to 27 mA. Pacing cycle length was 45 to 110 msec less than the atrial cycle length of tachycardia in 6 episodes. But in a neonate, underdrive pacing converted atrial flutter to sinus rhythm. Conversion attempts were unsuccessful on 2 occasions. Transesophageal atrial pacing is a safe and effective, minimally invasive technique for treatment of atrial flutter in infants and children.
Adolescent ; Atrial Flutter* ; Catheters ; Child* ; Electrodes ; Heart Defects, Congenital ; Humans ; Infant* ; Infant, Newborn ; Tachycardia

BACKGROUND: Valve replacement in children has many problems such as the durability of prosthetic valve, thomboembolism and hemorrhage. But recently, the necessity of valve replacement in children increased and the above problems were solved party. So the number of valve replacement in children increased progressively. RESULTS: Valve replacement in 47 children were done at Seooul National University Chidren's Hospital from March 1986 to July 1991. The patients were composed of 25 males and 33 females. 25 patients had congenital heart disease and 22 patients rheumatic heart disease. 45 patients received single valve repalcement, 2 patients double valve repalcement, and among all of them, 2 patients redo-replacement. The major valve lesion was mitral insufficiency and post-operative status in view of NYHA functinonal class was improved in most patients. The mechanical valves were applied to 45 patients and tissue valves to 2 patients. The indication of valve replacement were progressive increase in ventricular volume, major regurgitant fraction over grade III decrease in exercise tolerance and vegetation. The overall early mortality was 8.5% and late mortality 0%. There were post-operative complication rate of 30% and late complication rate 14%, and among the later, valve faliure was reported in 2 pantients and thromboembolism in 1 patient. The complication-free rate was 97.7% at post-operative 1 month, 91.3% at 12 months, 90% at 36 months and 60% at 48 months. 43 patients received anticoagulation and/or antiplatelet therapy, but there was no critical indication for this. CONCLUSION: These results suggest that cardiac valve replacement in children have been effective therapeutic modality even though various problems still remain, but we propose that sufficient long-term follow-up and clinical research be needed.
Child* ; Exercise Tolerance ; Female ; Follow-Up Studies ; Heart Defects, Congenital ; Heart Valves* ; Hemorrhage ; Humans ; Male ; Mitral Valve Insufficiency ; Mortality ; Rheumatic Heart Disease ; Thromboembolism

We report an extremely specific case of Pendred's syndrome, originally described with an association of thyroid organification defect and hearing impairment; normal-sized thyroid, severe hypothyroidism manifested by profoundly retarded physical and mental development, cardiomegaly and severe hypochromic & microcytic anemia associated with asymmetrical septal hypertrophy.
Adolescent* ; Anemia* ; Cardiomegaly ; Hearing Loss ; Humans ; Hypertrophy* ; Hypothyroidism ; Thyroid Gland

Congenital cystic disease of the lung represents pulmonary sequestration, congenital lobar emphysema, bronchogenic cyst and congenital cystic adenomatoid malformation. It has a variety of clinical manifestations from immediate postnatal respiratory distress to heart failure. Pulmonary sequestration sometimes causes heart failure in neonates through a shunt between an anomalous systemic feeding artery and the pulmonary venous system. We hereby report a case with both pulmonary sequestration and congenital lobar emphysema presenting with congestive heart failure and pulmonary hypertension, which improved after lobectomy.
Arteries ; Bronchogenic Cyst ; Bronchopulmonary Sequestration ; Cystic Adenomatoid Malformation of Lung, Congenital ; Emphysema ; Estrogens, Conjugated (USP) ; Heart Failure ; Humans ; Hypertension, Pulmonary ; Infant, Newborn ; Lung ; Pulmonary Emphysema

PURPOSE: In the Spring 1995, there was an outbreak of adenoviral infection, which caused four death out of ten patients with adenoviral pneumonia in our hospital. Clinical courses of ten patients with severe pneumonia were similar each other, and two were confirmed as adenoviral pneumonia by postmortem autopsy. Although not proven, we believe eight patients had adenoviral pneumonia. Therefore, we report clinical features in ten cases of severe adenoviral pneumonia. METHODS: Two cases with adenoviral pneumonia and eight cases with presumed adenoviral pneumonia were admitted in this hospital from March to June, 1995. Age and sex distribution, clinical manifestations, laboratory data, chest X-ray findings were reviewed. RESULTS: They were young children between 4 to 25 months of age(mean 12.7+/-6.1 months), and male to female ratio was 9:1. They presented with abrupt fever, cough, tachypnea, and dyspnea. Mean duration of fever were 12.7+/-6.1 days. Crackles on auscultation were heard in all patients. Studies for Mycoplasma and Tuberculosis were all negative. Cultures of bacteria and fungi were negative, and they did not respond to the antibiotics. The chest X-ray revealed the diffuse lobar consolidation with varying amount of pleural effusions. The findings of pleural fluid showed characteristics of transudate with predominant monocyte. Eight of our severe adenoviral pneumonia patients were enjoying normal health previously. Only two patients had previous medical problems, one with chronic cytomegalovirus pneumonia and the other with neutropenia induced by phenobarbital. The course of illness suggests that the infection was hospital acquired and the final outcome was fatal. Three of them developed seizure with fever, five change of consciousness, four conjunctivitis, three otitis media, and two gastro-intestinal symptoms. Autopsy was done in two of four patients. Grossly, the lungs were heavy and dark- red in color. There were bilateral pneumonic consolidation with patchy areas of hemorrhage. Microscopically, severe necrotizing bronchitis and bronchiolitis with numerous intranuclear inclusion of Cowdry type A and B were found. Alveoli were edematous and filled with fibrinous exudate, and covered with hyaline membrane. Ultrastructurally, typical adenoviral particles showing hexagonal shape in paracrystalline array symmetry were found in the nucleus of aleveolar lining cells. CONCLUSIONS: Yet, occasionally, adenoviral infection becomes most aggressive form of pneumonia. We should consider adenoviral pneumonia when clinical findings of pneumonia are very similar with baterial pneumonia except poor response to broad spectrum antibiotics. There is no specific treatment for adenoviral infection. So, for prevention of adenoviral pneumonia, we recommend isolation in suspicious adenoviral infection.
Adenoviridae ; Anti-Bacterial Agents ; Auscultation ; Autopsy ; Bacteria ; Bronchiolitis ; Bronchitis ; Child ; Conjunctivitis ; Consciousness ; Cough ; Cytomegalovirus ; Dyspnea ; Exudates and Transudates ; Female ; Fever ; Fibrin ; Fungi ; Hemorrhage ; Humans ; Hyalin ; Intranuclear Inclusion Bodies ; Lung ; Male ; Membranes ; Monocytes ; Mycoplasma ; Neutropenia ; Otitis Media ; Phenobarbital ; Pleural Effusion ; Pneumonia* ; Respiratory Sounds ; Seizures ; Sex Distribution ; Tachypnea ; Thorax ; Tuberculosis

PURPOSE: This research was designed to evaluate the chronic effect of isolated lung perfusion (ILP) with cisplatin on dogs. MATERIALS AND METHODS: Fifteen dogs were divided into three groups. Group I was in ILP without cisplatin, group II with 2.5 mg/kg and group III with 5.0 mg/kg of cisplatin for 30 minutes respectively. Serial blood samples were taken before and after ILP for quantitative analysis of serum lactate dehydrogenase (LDH) and blood urea nitrogen/creatinine (BUN/Cr). The specimens from the lung were obtained 2 weeks after ILP. RESULTS: There were no statistic significant differences in LDH concentration according to the time interval among the groups. The LDH concentration peaked at 1 week after ILP and declined thereafter to the pre-ILP concentration. The concentration of BUN/Cr was in normal range. Histologic examination showed no pathologic change. No significant histopathologic differences were found in the pulmonary parenchyme and vasculature among the groups. All of the dogs survived without complication 2 weeks after ILP. CONCLUSION: In ILP with cisplatin of 5.0 mg/kg in normal dog, the toxicity of cisplatin itself was not observed. With further study about the technique of ILP with cisplatin it would be effective to deliver high concentration of cisplatin into the target tissue minimizing lung damage.
Animals ; Cisplatin* ; Dogs* ; Drug-Related Side Effects and Adverse Reactions ; L-Lactate Dehydrogenase ; Lung* ; Perfusion* ; Reference Values ; Urea

The presence of a single coronary artery is a rare congenital anomaly; such patients often present with severe myocardial ischemia. We experienced the case of a 13-year-old girl with the right coronary artery originating from the left circumflex artery. She visited our Emergency Department owing to severe chest pain; her cardiac enzyme levels were elevated, but her initial electrocardiogram (ECG) was normal. Echocardiography showed normal anatomy and normal regional wall motion. When she presented with recurrent chest pain on admission, the ECG showed significant ST-segment elevation in the left precordial leads and inferior leads with ST-segment depression in aVR lead, suggesting myocardial ischemia, and her cardiac enzyme levels were also elevated. We performed coronary angiography that showed a single right coronary artery originating from the left circumflex artery without stenosis. We confirmed the presence of a single coronary artery using coronary computed tomography. In addition, the treadmill test that was performed showed normal results. She was discharged from the hospital without any medications but with a recommendation of a regular follow-up.
Adolescent ; Arteries* ; Chest Pain ; Child* ; Constriction, Pathologic ; Coronary Angiography ; Coronary Vessels* ; Depression ; Echocardiography ; Electrocardiography ; Emergency Service, Hospital ; Exercise Test ; Female ; Humans ; Myocardial Ischemia

Unprovoked deep vein thrombosis (DVT) is uncommon in pediatric patients and, among those, combined hereditary thrombophilia is particularly rare. We present a 9-year-old Korean boy who developed lower extremity pain with swelling, and was diagnosed with unprovoked DVT due to hereditary (combined hereditary thrombophilia). Coagulation test revealed antithrombin III and protein S deficiency. The genetic work up confirmed the first case of combined antithrombin III deficiency and protein S deficiency by SERPINC1 heterozygous termination mutation [c.685C>T (p.Arg229*)] and PROS1 heterozygous missense mutation [c.1597G>A (p.Val533Met)]. He was treated with continuous heparin and catheter intervention but those were ineffective or transiently effective. His DVT gradually improved only after prolonged anticoagulation.
Antithrombin III Deficiency ; Antithrombin III* ; Catheters ; Child ; Heparin ; Humans ; Lower Extremity ; Male ; Mutation, Missense ; Protein S Deficiency* ; Protein S* ; Thrombophilia* ; Venous Thrombosis*

PURPOSE: Spontaneous pneumomediastinum (SPM) is a rare entity, with only a few cases reported, especially in adolescents. We aimed to analyze the clinical characteristics of SPM in adolescents and the diagnostic implications of computed tomography (CT) and esophagography therein. MATERIALS AND METHODS: This retrospective descriptive study was conducted as a review of medical records of 416 adolescents (10-18 years of age) with chest pain from March 2005 to June 2013. Information on clinical presentation, methods of diagnosis, hospital stay, and outcomes were collected and analyzed. RESULTS: Among adolescents complaining of chest pain, 11 patients had SPM (11/416, 2.64%). All patients presented with pleuritic chest pain, and 54.5% reported neck pain as the most common associated complaint. Clinical findings were nonspecific, and initial chest X-ray assessment was diagnostic only in three of 11 patients. However, reassessment of chest X-ray revealed diagnostic findings of SPM in five of the remaining eight patients. CT was diagnostic in all patients, while esophagography and echocardiogram were uninformative. Symptomatic improvement was noted within 2.45+/-1.2 hours (range, 0.5 to 4) after supportive care; mean hospital stay was 4.54+/-0.99 days (range, 2 to 6). No recurrence was observed. CONCLUSION: SPM is a rare disease that should be considered in adolescent patients with pleuritic chest pain. Careful reading of initial chest X-rays is important to avoiding further unnecessary investigations. SPM is self-limited and treatment is supportive; nevertheless, if there are no indications of esophageal rupture, urgent esophagography is not recommended.
Adolescent ; Analgesics/*therapeutic use ; Chest Pain/diagnosis/*etiology ; Child ; Female ; Follow-Up Studies ; Humans ; Length of Stay ; Male ; Mediastinal Emphysema/complications/*diagnosis/*therapy ; Medical Records ; *Oxygen Inhalation Therapy ; Rare Diseases ; Retrospective Studies ; Risk Factors ; Tomography, X-Ray Computed ; Treatment Outcome

PURPOSE: In patients with the congenitally corrected transposition of great arteries (CCTGA), systemic ventricle tends to be hypertrophic due to longstanding systemic high pressure that may lead to dysfunction of ventricle. The aim of this study was to assess the global and segmental wall function of systemic right ventricle in CCTGA. METHODS: Twelve patients with the CCTGA patients were enrolled. Ejection fraction of systemic right ventricle, myocardial performance index (MPI), strain and strain rate were assessed. RESULTS: The median age was 32.5 years with a range of 4 to 66 years. Ejection fraction (EF) using M-mode and Simpsons method was significantly lower in the CCTGA patients (48.5+/-4.6% and 51.2+/-4.1%, respectively, vs 63.7+/-3.6% and 66.6+/-3.8% respectively). 5 patients had diastolic dysfunction (3/12; pseudo-normalization pattern, 2/12; relaxation abnormality). Strain and strain rate revealed segmental systemic right ventricle dysfunction, and were lower in the CCTGA patients at mid and apical segment of septum and apical segment of anterior wall. The extent of dysfunctional segment was related to the age and MPI. CONCLUSION: There was global dysfunction in the CCTGA patients and it was related with age. Segmental dysfunction was especially shown at mid and apical segment of septum and apical segment of anterior wall and more frequently at apical segment of each wall. The extent of dysfunctional segment was related with age.
Arteries* ; Heart Ventricles ; Humans ; Relaxation ; Transposition of Great Vessels ; Ventricular Function, Right*