• Clinical and Experimental Pediatrics
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• v.55 no.8
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• pp.297-300
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• 2012

Symptomatic pulmonary arterial hypertension (PAH) in patients with isolated atrial septal defect (ASD) is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg). The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg), and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.

• Journal of Veterinary Clinics
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• v.40 no.2
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• pp.130-134
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• 2023

A 12-year-old Standard Poodle presented with intermittent weakness and occasional dyspnea at the Veterinary Medicine Teaching Hospital of Kangwon National University. A grade of 4 out of 6 systolic murmur with an irregular tachycardic rhythm was auscultated on both sides of the chest. Systolic blood pressure was 140 mmHg. Panting was noticed in the hospital, but there was no crackle sound. Blood analysis revealed mild increases in liver panel levels (alanine aminotransferase 149 [reference interval, 19-70] U/L; and alkaline phosphatase, 185 [reference interval, 15-127] U/L) and severe increases in cardiac biomarker levels (n-terminal pro-brain natriuretic peptide, 4169 [reference interval, 50-900] pmol/L; and cardiac troponin I, 0.22 [reference interval, 0.03-0.12] ng/mL). On electrocardiography, irregularly irregular supraventricular tachycardic rhythm with an f-wave and no distinct p-wave was observed. Generalized cardiomegaly with an enlarged right atrium and left ventricle was confirmed on thoracic radiography. Moreover, hepatomegaly and an enlarged caudal vena cava were observed. Echocardiographic evaluation revealed a fibromuscular diaphragm in the right ventricle. Because of the obstructive lesion in the right ventricle, the right atrium and ventricle were enlarged (right atrial area index, 38.82 cm²/m² [reference interval, 4.2-10.2 cm²/m²]; right ventricle end-diastolic area index, 14.152 cm²/m² [reference interval, 4.9-10.92 cm²/m²]). Accordingly, the patient was diagnosed with double-chambered right ventricle (DCRV). Pimobendan, furosemide, enalapril, diltiazem, and S-adenosylmethionine (SAMe) were prescribed, and all symptoms were relieved. DCRV is a right-sided congenital heart defect resembling pulmonic valve stenosis. If symptoms are not severe, medical therapy can be facilitated without surgery or the balloon dilation.

• Clinical and Experimental Pediatrics
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• v.46 no.7
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• pp.661-667
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• 2003

Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction. Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who underwent total correction. Results : Mean age at retrospective study was 30.8(range : 16-53) years old. Age at total correction was 15.8(range : 2-49) years old. Problems after corrective surgery were assessed. They were arrhythmia, pulmonary valve regurgitation, left pulmonary artery stenosis, residual ventricular septal defect, mitral valve regurgitation, tricuspid valve regurgitation, right ventricle outflow tract obstruction, aortic valve regurgitation, infective endocarditis and protein losing enteropathy. After repair of TOF, such arrhythmias as atrial arrhythmia and AV conduction disturbances were observed in some patients. Cardiomegaly was found significantly in the subjects with arrhythmia(P<0.05), and arrhythmia was less observed in patients who underwent surgery at a young age. Eight patients required a reoperation; the main indications were residual ventricular septal defect, right ventricle outflow tract obstruction and peripheral pulmonary artery stenosis. Conclusion : The majority of the patients seemed to live normal lives after Tetralogy of Fallot repair. However, as residual anatomic and functional abnormalities exist postoperatively, continued careful follow-up is needed to detect and correct structural and functional abnormalities.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.9-15
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• 2007

Purpose : Edema is one of the cardinal features of nephrotic syndrome. Although the pathogenesis of edema is not entirely understood, it is caused by hypovolemia or hypervolemia by different mechanisms. Accordingly it is important to evaluate the volume status of patients in order to treat the edema, but it is difficult to evaluate the patient's volume status only by clinical parameters. The quotient of urine sodium and potassium excretion $U_K/(U_{Na}+U_K)$ is introduced as a more useful way to evaluate volume status. In this study we will propose the usefulness of $U_K/(U_{Na}+U_K)$ in evaluating the volume status of children with nephrotic syndrome. Methods : Primary nephrotic syndrome patients at Yeungnam University Hospital since January 1995 to June 2005, were included in the study. We analyzed clinical parameters such as tachycardia, cardiomegaly, pleural effusion, blood chemistry and urinalysis prospectively. We defined hypovolemia when $U_K/(U_{Na}+U_K)$ exceeded 60%. Intravenous albumin and diuretics were administered to hypovolemic edematous patients. On the other hand, hypervolemic edematous patients were treated only with diuretics. Results : There were 50 cases of primary nephrotic syndrome patients(hypervolemia: 29 vs hypovolemia: 21). There were no significant differences in clinical symptoms and laboratory findings except for FeNa While $F_eNa$ and $U_K/(U_{Na}+U_K)$ had a significant negative correlation, BUN and $U_K/(U_{Na}+U_K)$ had a significant positive correlation. Urine output after edema treatment was effective and there were no treatment-related side effects in both groups. Conclusion : FeNa, BUN and $U_K/(U_{Na}+U_K)$ are a useful parameters for evaluating volume status of edematous nephrotic syndrome patients. We could suggest a therapeutic option for using albumin and/or diuretics according to volemic status by means of measured $U_K/(U_{Na}+UK)$.

• Sleep Medicine and Psychophysiology
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• v.3 no.1
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• pp.85-95
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• 1996

Obstructive sleep apnea syndrome(OSAS) in childhood is unique and different n-om that in adulthood in several aspects, including pathophysiology, clinical features, diagnostic criteria, complications, management, and prognosis. Characteristic features of childhood OSAS in comparison with the adult form are the variety of severe complications such as developmental delay, more prominent behavioral and cognitive impairments, vivid cardiovascular symptoms, and increased death risk, warranting a special attention to the possible diagnosis of OSAS in children who snore. However, the childhood OSAS is often neglected and unrecognized. We, therefore, report a case of very severe OSAS in a 5-year-old boy who was sucessfully treated with continuous positive airway pressure(CPAP) treatment. Interestingly, the patient was comor-bid with the attention deficit hyperactivity disorder. Prior to the initial visit to us, adenotonsillectomy had been done at the age of 4 with no significant improvement of apneic symptoms and heavy snoring. On the initial diagnostic procedures, marked degree of snoring was audible even in the daytime wake state and the patient was observed to be very hyperactive. Increased pulmonary vascularity with borderline cardiomegaly was noted on chest X-ray. The baseline polysomnography revealed that the patient was very sleep-apneic and snored very heavily, with the respiratory disturbance index(RDI) of 46.9 per hour of sleep, the mean SaO2 of 78.8%, and the lowest SaO2 of 40.0%(the lowest detectable oxygen level by the applied oxymeter). The second night polysomnography was done for CPAP titration and the optimal pressure turned out to be $8.0\;cmH_2O$. The applied CPAP treatment was well tolerated by the patient and was found to be very effective in alleviating heavy snoring and severe repetitive sleep apneas. After 18 months of the CPAP treatment, the patient was followed up with nocturnal polysomnography(baseline and CPAP nights) and clinical examination. Sleep apneas were still present without CPAP on the baseline night. However, the severity of OSAS was significantly decreased(RDI of 15.7, mean SaO2 of 96.2%, and the lowest SaO2 of 83.0%), compared to the initial polysomnographic findings before initiation of long-term CPAP treatment. Wechsler intelligence tests done before and after the CPAP treatment were compared with each other and surprising improvement of intelligence(total 9 points, performance 16 points) was noted. Clinically he was found to be markedly improved in his attention deficit hyperactive behavior after CPAP treatment, but with minimal change of TOVA(test of variables of attention) scores except conversion of reaction time score into normal range. On the chest X-ray taken after 18 months of CPAP application, the initial cardiopulmonary abnormalities were not found at all. We found that the CPAP treatment in a young child is very effective, safe, and well-tolerated and also improves the co-morbid attention deficit hyperactive symptoms. Overall, the growth and development of the child has been facilitated with the long-term use of CPAP. Cardiovascular complications induced by OSAS have been also normalized with CPAP treatment. We suggest that early diagnosis and active treatment intervention of OSAS in children are crucial in preventing and ameliorating possible serious complications caused by repetitive sleep apneas and consequent hypoxic damage during sleep.

• Tuberculosis and Respiratory Diseases
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• v.50 no.1
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• pp.106-116
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• 2001

Background : Pulmonary thromboembolism is relatively frequent and potentially fatal. However, it is commonly misdiagnosed. The incidence of pulmonary thromboembolism is not decreasing despite advances in diagnosis and effective prophylatic measures. Its potential for significant sequela necessitates a prompt diagnosis and treatment. Unfortunately, there are many difficulties and problems regarding accurate diagnosis. There is a low prevalence of deep vein thrombosis and pulmonary thromboembolism in Korea and only few reports on this subject are available. Method : The clinical features of 36 patients, who were diagnosed with pulmonary thromboembolism at the Korea University medical center, were reviewed. Results : 1) There was no significant difference in prevalence between men an women, and the mean age was 50.9 years in men 59.2 years in women. 2) The frequent causes of pulmonary thromboembolism were malignancies (22.2%), surgery (22.2%), and heart disease(8.2%). Specific causes were not identified in 33.3%. 3) The most common symptom was dyspnea(72.2%), and the most common sign was tachypnea(61.1%). 4) The EKG findings were normal in 28.6%, an S1Q3T3 pulmonale pattern in 25.7%, ST or QRS changes in others. 5) The chest X-ray findings indicated pulmonary infiltration in 37.5%, cardiomegaly in 15.6%, pleural effusion in 12.5%, and normal in 27.8%. The perfusion lung scan showed a high probability in 66.7%, and intermediate or low probability in 33.3%. 6) The pulmonary arterial pressure(PAP) in the high probability groups was 57.9mmHg with a higher mortality rate(35%). Conclusion : Pulmonary thromboembolism is not uncommon in Korea and its clinical features do not differ greatly from thase reported in the literature. When pulmonary thromboemblism of unknown causes are diagnosed, a search for an occult malignancy is recommended. Rapid diagnosis and treatment are achieved when thromboemblism is suspected.