Prediction of Immune Infiltration Diagnostic Gene Biomarkers in Kawasaki Disease.

Kawasaki disease (KD) is characterized by disorder of immune response with unknown etiology. Immune cells may be closely related to the onset of KD. The focus of this research was to evaluate the significance of the infiltration of immune cells for this disease and find possible diagnostic biomarkers for KD. The Gene Expression Omnibus database was utilized to retrieve two freely accessible gene expression patterns (GSE68004 and GSE18606 datasets) from human KD and control specimens. 114 KD, as well as 46 control specimens, were searched for obtaining differentially expressed genes (DEGs). Candidate biological markers were determined utilizing the support vector machine recursive feature elimination and the least absolute shrinkage and selection operator regression model analysis. To assess discriminating capacity, the area under the receiver operating characteristic curve (AUC) was computed. The GSE73461 dataset was utilized to observe the biomarkers' expression levels and diagnostic significance in KD (78 KD patients and 55 controls). CIBERSORT was employed to assess the composition profiles of the 22 subtypes of immune cell fraction in KD on the basis of combined cohorts. 37 genes were discovered. The DEGs identified were predominantly involved in arteriosclerotic cardiovascular disease, atherosclerosis, autoimmune disease of the urogenital tract, and bacterial infectious disease. Gene sets related to complement and coagulation cascades, Toll-like receptor signaling pathway, Fc gamma R-mediated phagocytosis, NOD-like receptor signaling pathway, and regulation of actin cytoskeleton underwent differential activation in KD as opposed to the controls. KD diagnostic biomarkers, including the alkaline phosphatase (ALPL), endoplasmic reticulum degradation-enhancing alpha-mannosidase-like protein 2 (EDEM2), and histone cluster 2 (HIST2H2BE), were discovered (AUC = 1.000) and verified utilizing the GSE73461 dataset (AUC = 1.000). Analyses of immune cell infiltration demonstrated that ALPL, EDEM2, and HIST2H2BE were linked to CD4 memory resting T cells, monocytes, M0 macrophages, CD8 T cells, neutrophils, and memory CD4 T cells. ALPL, EDEM2, and HIST2H2BE could be utilized as KD diagnostic indicators, and they can also deliver useful information for future research on the disease's incidence and molecular processes.

Case Report: Talaromyces marneffei Infection in a Chinese Child With a Complex Heterozygous CARD9 Mutation.

Talaromyces marneffei (TM) infection is rarely seen in clinical practice, and its pathogenesis may be related to deficiency in antifungal immune function. Human caspase recruitment domain-containing protein 9 (CARD9) is a key molecule in fungal immune surveillance. There have been no previous case reports of TM infection in individuals with CARD9 gene mutations. Herein, we report the case of a 7-month-old Chinese boy who was admitted to our hospital with recurring cough and fever with a papular rash. A blood culture produced TM growth, which was confirmed by metagenomic next-generation sequencing. One of the patient's sisters had died of TM septicaemia at 9 months of age. Whole exome sequencing revealed that the patient had a complex heterozygous CARD9 gene mutation with a c.1118G>C p.R373P variation in exon 8 and a c.610C>T p.R204C variation in exon 4. Based on the culture results, voriconazole antifungal therapy was administered. On the third day of antifungal administration, his temperature dropped to within normal range, the rash gradually subsided, and the enlargement of his lymph nodes, liver, and spleen improved. Two months after discharge, he returned to the hospital for a follow-up examination. His general condition was good, and no specific abnormalities were detected. Oral voriconazole treatment was continued. Unexplained TM infection in HIV-negative individuals warrants investigation for immune deficiencies.

Sitosterolemia With Atherosclerosis in a Child: A Case Report.

Introduction: Sitosterolemia is a rare condition in children and is often misdiagnosed as familial hypercholesterolemia. Serious complications can result if not treated promptly and effectively. When pediatric patients are diagnosed with sitosterolemia, vascular, and cardiac studies are important to evaluate for the presence of atherosclerosis. Few cases of severe atherosclerotic heart disease in children with sitosterolemia have been reported, making this case worthy of presentation. Case Presentation: Here, we report a case of sitosterolemia in an 8-year-old child. The patient presented with severe hypercholesterolemia and xanthoma. He was diagnosed two and a half years prior with familial hypercholesterolemia because his father had elevated cholesterol levels. After conventional treatment, the patient was dissatisfied with lipid level control and visited our hospital for further management. Genetic tests of the patient and parents found mutations in intron 7 (NM 022436.2, c.904+1G>A) and intron 9 (NM 022436.2, C. 1324+1de1G) of ABCG5. The 7 intron mutation was from his mother, and the 9 intron mutation was from his father. The patient was diagnosed with sitosterolemia. Results: The child was treated with ezetimibe, a low plant sterol diet, and clopidogrel anticoagulant therapy. After 3 months of treatment, the blood lipid level was significantly lower. Conclusion: Genetic testing should be completed as soon as possible to avoid misdiagnosis in children with abnormally elevated hypercholesterolemia who have a family history of elevated cholesterol. In addition, clinicians should rule out great arterial lesions and be vigilant in evaluating patients for systemic arterial disease and atherosclerosis.

Treatment Option for Abernethy Malformation-Two Cases Report and Review of the Literature.

Background: Abernethy malformation is a rare vascular anomaly of the portal venous system, which is also known as congenital portosystemic shunts (CPSS). The clinical manifestations of this anomaly can be serious, including hepatopulmonary syndrome(HPS), which can lead to significant hypoxemia and cyanosis. Case Presentation: This study reports two cases of patients with Abernethy Malformation. Case 1 was a 6-year-old boy whose blood oxygen saturation was 78%. Case 2 was a 6-year-old girl who had a history of open heart surgery and residual cardiac left to right shunt, whose blood oxygen saturation was 83%. These two children had unexplained cyanosis and were diagnosed with pulmonary arteriovenous fistula by contrast echocardiography with agitated saline. A selective retrograde catheter angiography confirmed the presence of a portosystemic shunt. Case 1 was a type I Abernethy malformation and did not receive any specific treatment and could only wait for liver transplantation. Case 2 was with type II Abernethy and underwent transcatheter closure of the CPSS. A 20mm-diameter, 14mm-long Vascular Plug (SHSMA Inc, Shanghai, China) was used to occlude the shunt. Results: In case 1, the boy developed deteriorating cyanosis and dyspnea on exertion. In case 2, the exercise tolerance of the patient improved after shunt closure. During a follow-up of 3 years, her blood oxygen saturation increased from 83 to 98%. Conclusion: The results indicate that children with unexplained cyanosis require special attention since these patients may have Abernethy malformation, and part of them could be treated by transcatheter occlusion with a good outcome. The key to treatment is how it is diagnosed and carefully assessed.

Beta Blocker Therapy for Congenital Hepatic Arteriovenous Fistula in Two Neonates.

Introduction: Hepatic arteriovenous fistula (HAVF) is an abnormal communication between the hepatic arteries and hepatic veins. This condition is treated mainly using interventional closure and surgery. However, these procedures are associated with many postoperative complications and high mortality. Propranolol and other beta blockers have been used widely and effectively to treat infantile hemangiomas. However, no reports describe the use of these drugs to treat congenital HAVF. Case Description:Here, we present two cases in which beta blocker therapy was used to treat congenital HAVF in neonates. In both cases, antenatal examinations revealed cardiac enlargement and hepatic space-occupying lesions. After birth, both patients rapidly presented with respiratory distress, cyanosis, and heart failure. Echocardiography suggested enlargement of the right heart, widening of the pulmonary artery, and severe pulmonary arterial hypertension, and hepatic examinations revealed HAVF. Results:After admission, the patients were treated with dopamine, milinone, and furosemide for heart failure. However, their conditions worsened, as indicated by nod-like breathing and cyanosis. Endotracheal intubation and ventilator-assisted breathing and a small dose of oral propranolol (1 mg/kg/d) were initiated. The patients' conditions improved, as indicated by decreases in levels of the N-terminal pro-hormone BNP, and the ventilators were removed. The propranolol dose was increased gradually to 2 mg/kg/d. After 2 weeks of propranolol treatment, the neonate in case 2 developed bronchospasm, which improved after propranolol treatment ended and metoprolol treatment was initiated. Liver imaging performed 8-9 months after beta blocker therapy suggested the disappearance of the arteriovenous fistulae in case 2, and close to disappearing of the arteriovenous fistulae in case 1. Conclusion:Propranolol and metoprolol can effectively treat HAVF in infants, an observation consistent with that found in earlier studies that have shown beta blockers are a valid medical treatment option for infantile hemangioma. However, future studies should explore the underlying potential mechanism.

Amiodarone-Related Pure Red Cell Aplastic Anemia and Hypothyroidism in a Child With Total Anomalous Pulmonary Venous Connection.

Introduction: Amiodarone is an effective anti-arrhythmic drug, but there are many clinical side effects that limit its application. There are no case reports of amiodarone-related pure red cell aplastic anemia (PRCA). Case Presentation: Here, we present a case of amiodarone-related PRCA and hypothyroidism in a 7-month-old boy. The patient had a total anomalous pulmonary venous connection (the cardiac type) and had undergone cardiac surgery at the age of 2 months. Eleven days after the operation, atrial tachycardia was observed. Amiodarone was administered orally (15 mg/kg.d), following which the arrhythmia was under control. Subsequently, the patient was prescribed amiodarone (5 mg/kg.d) and discharged. Regular medical consultations were not conducted as required. At 7 months of age (5 months after the operation), the patient returned to the hospital for re-examination. The electrocardiogram showed intermittent sinus bradycardia, occasional junctional escape beats, hemoglobin 7.9 g/DL, and thyroid function-TSH 9.660 uIU/mL. Results: Amiodarone was discontinued. Thyroxine was administered orally. Subsequently, the heart rate improved and TSH returned to normal levels. Nutritional therapy was recommended based on a diagnosis of nutrition-related anemia. A re-visit at 9 months of age showed that the weight was 6 kg, but the routine blood test indicated that hemoglobin was 5.9 g/DL with positive cell anemia and low reticulocyte count. Bone marrow cytology examination suggested PRCA. The hemoglobin level was gradually restored after treatment with prednisone. Conclusion: The use of amiodarone in small infants and young children and its side effects should be carefully monitored. The potential mechanism of amiodarone-related PRCA needs further study.

Chronic Active Epstein-Barr Virus Infection With Systemic Vasculitis and Pulmonary Arterial Hypertension in a Child.

Introduction: A chronic active Epstein-Barr virus (EBV) infection (CAEBV), which is characterized by persistent "infectious mononucleosis-like" symptoms, can lead to cardiovascular complications, including coronary artery aneurysms. No published studies have reported an occurrence of chronic EB virus infection in conjunction with systemic vasculitis and pulmonary hypertension. Case Presentation: Herein, we present a case of a 9-year-old boy with CAEBV, associated with pulmonary arterial hypertension (PAH) and systemic vasculitis. Recurrent skin ulcers were a major early clinical manifestation in this case. The histopathological examination of a dermal biopsy sample from the lesions revealed vasculitis, and the in-situ hybridization test was positive for EBV-encoded small RNA. Results: The patient was administered immunosuppressants (prednisolone and cyclophosphamide) and targeted drugs (sildenafil and bosentan) to control the pulmonary pressure. This combination therapy decreased the systolic pulmonary arterial pressure to 40 mm Hg (on echocardiography), and the N-terminal pro b-type natriuretic peptide level also reduced to 62.3 pg/ml. After discontinuation of prednisone, the child developed shortness of breath, edema, and oliguria. He was again started on prednisone, with an addition of thalidomide. Sildenafil was replaced by riociguat, due to the side effect of penile erection. The patient is being followed up every 2 months at the clinic. The most recent follow-up visit was 2 weeks before this report was written, during which, the child was observed to have no rash, shortness of breath, edema, and other symptoms. Written informed consent was obtained from the parents for the publication of this case report. Conclusion: A CAEBV should be considered among the differential diagnoses while managing a pediatric patient with secondary PAH and systemic vasculitis. However, elucidation of its potential pathophysiological mechanisms requires further study.

Left Ventricular Mechanics Assessed by 2-dimensional Speckle Tracking Echocardiography in Children and Adolescents With Idiopathic Scoliosis.

STUDY DESIGN: A retrospective review of data collected prospectively. OBJECTIVE: The study aimed to assess the possible effects of idiopathic scoliosis on left ventricular (LV) myocardial performance in children and adolescent patients. SUMMARY OF BACKGROUND DATA: Spine anomaly may impair cardiorespiratory function. It has been confirmed that respiratory function decreased in patients with scoliosis. However, limited study of the effect of scoliosis on heart function has been published, and no assessment of myocardial deformation of these patients has been reported to our knowledge. METHODS: Forty-one patients with a median age of 16 years and a median Cobb's angle of 75 degrees were studied. LV myocardial deformation was evaluated using 2-dimensional speckle-tracking echocardiography. The results were compared with those of 33 controls. The correlations between Cobb's angle and echocardiographic parameters in patients were also explored. RESULTS: Compared with controls, patients had significantly lower global LV longitudinal systolic strain in all 3 views (4 chamber, 2 chamber, and 3 chamber views), lower global radial and circumferential strains in mitral valve (MV) level and papillary muscle (PM) level; reduced LV longitudinal early diastolic strain rate (SRe) in all 3 longitudinal views and reduced radial SRe in MV level and reduced circumferential SRe in apical (AP) level; decreased LV longitudinal late diastolic strain rate in 4-chamber view and decreased radial ones in all 3 short-axis levels. Among patients, Cobb's angle correlated negatively with LV global radial strain of MV level (r=-0.37, P=0.02), global circumferential strain of MV level (r=-0.35, P=0.03), and global circumferential strain of PM level (r=-0.49, P=0.001), whereas positively with LV longitudinal SRa of 4-chamber view (r=0.46, P=0.003), longitudinal SRa of 2-chamber view (r=0.49, P=0.001), and circumferential SRa of AP level (r=0.35, P=0.02). CONCLUSIONS: LV mechanics are impaired in patients with idiopathic scoliosis, which correlate with the severity of scoliosis. Our findings suggest the need and provide a mechanical basis for further studies in these patients.

Pentoxifylline Prevents Driamycin-Induced Myocardial Fibrosis and Apoptosis in Rats.

Adriamycin (ADR) is a potent antineoplastic agent, but long-term treatment is limited by its cumulative, life-threatening cardiomyopathy. Recently, a few reports have shown that pentoxifylline (PTX) might produce cardioprotection in cardiac dysfunction. Here, we investigated the protective effects of PTX on ADR-induced cardiomyopathy in rats. Male rats were randomly assigned either to saline, ADR (adriamycin, 5 mg/kg/week), or A (adriamycin, 5 mg/kg/week) + PTX (pentoxifylline, 50 mg/kg/day) groups. After 3 weeks, these animals were sacrificed and the heart tissue was harvested for histological analysis and assessment of hepatocyte growth factor (HGF) and caspase-3 expression. Histopathological findings showed that PTX can alleviate myocardial damage caused by ADR. Cardiac fibrosis was significantly suppressed in the A+PTX group compared to that in the ADR group. The HGF gene expression was decreased significantly in the ADR group compared with the control group, but was increased in the A+PTX group. Caspase-3 was up-regulated in the ADR group, and down-regulated in the A+PTX group. These results show that treatment with PTX exerts a protective effect against ADR-induced myocardial fibrosis via regulation of HGF and caspase-3 gene expression. PTX may thus represent a useful new clinical tool for the treatment of ADR-induced cardiomyopathy.

Ventricular and atrial mechanics and their interaction in patients with congenital scoliosis without clinical heart failure.

OBJECTIVES: This study sought to evaluate left ventricular, right ventricular, and left atrial mechanics and their interactions in patients with congenital scoliosis without clinical heart failure. METHODS: A total of 23 patients with a median age of 14 years and a median Cobb's angle of 61° were studied. Ventricular and atrial myocardial deformation was measured using speckle tracking echocardiography. The results of the patients were compared with 22 controls. RESULTS: Compared with controls, the patients had a significantly greater annular a velocity (p=0.04) and lower e/a ratio (p=0.03); the left ventricular deformation significantly decreased in radial global (p=0.04) and segmental systolic strain and early diastolic strain rate (p=0.03); the left atrial deformation showed a significantly lower positive strain (p=0.02), greater negative strain (p=0.01), and active contractile strain rate (p=0.01). For the patients, the Cobb's angle was negatively correlated with the left ventricular global radial systolic strain (r=-0.65, p=0.001), left atrial positive strain (r=-0.68, p<0.001), and the left atrial negative strain was positively correlated with the left ventricular circumferential late diastolic strain rate (r=0.46, p=0.01). The left atrial conduit strain rate was positively correlated with the left ventricular circumferential early diastolic strain rate (r=0.42, p=0.03). The left atrial active contractile strain rate was positively correlated with the left ventricular longitudinal late diastolic strain rate (r=-0.4, p=0.03). CONCLUSIONS: Impaired left ventricular and altered left atrial mechanics occur relatively early in patients with congenital scoliosis, and are correlated with the severity of their scoliosis. Our findings provide evidence of preclinical heart dysfunction in patients with this disorder.

H2S inhibition of chemical hypoxia-induced proliferation of HPASMCs is mediated by the upregulation of COX-2/PGI2.

The hypoxia-induced proliferation of pulmonary artery smooth muscle cells (PASMCs) is the main cause of pulmonary arterial hypertension (PAH), in which oxidative stress, cyclooxygenase (COX)-2 and hydrogen sulfide (H(2)S) all play an important role. In the present study, we aimed to examine the effects of H(2)S on the hypoxia-induced proliferation of human PASMCs (HPASMCs) and to elucidate the underlying mechanisms. The HPASMCs were treated with cobalt chloride (CoCl(2)), a hypoxia-mimicking agent, to establish a cellular model of hypoxic PAH. Prior to treatment with CoCl(2), the cells were pre-conditioned with sodium hydrosulfide (NaHS), a donor of H(2)S. Cell proliferation, reactive oxygen species (ROS) production, COX-2 expression, prostacyclin (also known as prostaglandin I2 or PGI(2)) secretion and H(2)S levels were detected in the cells. The exposure of the HPASMCs to CoCl(2) markedly increased cell proliferation, accompanied by a decrease in COX-2 expression, PGI(2) secretion and H(2)S levels; however, the levels of ROS were not altered. Although the exogenous ROS donor, H(2)O(2), triggered similar degrees of proliferation to CoCl(2), the ROS scavenger, N-acetyl-L-cysteine (NAC), markedly abolished the H(2)O(2)­induced cell proliferation, as opposed to the CoCl(2)-induced proliferation. The CoCl(2)-induced proliferation of HPASMCs was suppressed by exogenously applied PGI(2). The addition of H(2)S (NaHS) attenuated the CoCl(2)-induced cell proliferation through the increase in the intercellular content of H(2)S. Importantly, the exposure of the cells to H(2)S suppressed the CoCl(2)-induced downregulation in COX-2 expression and PGI(2) secretion from the HPASMCs. In conclusion, the results from the current study suggest that H(2)S enhances hypoxia-induced cell proliferation through the upregulation of COX-2/PGI(2), as opposed to ROS.

Right ventricular function impaired in children and adolescents with severe idiopathic scoliosis.

BACKGROUND: Although it is speculated that scoliosis may induce cardiac dysfunction, there is no report about evaluation of cardiac function, especially right cardiac function in patients with scoliosis. Therefore, we evaluated right ventricular function in idiopathic scoliotic patients with mild to severe curves and compared them with healthy children and adolescents matched in age, then explored relationship between scoliosis and right ventricular function. METHODS: Thirty-seven patients diagnosed with idiopathic scoliosis with a mean age of 16y/o (range, 8-25y/o) and an average spine curve of 77.5°Cobb (range, 30-157°) were studied by echocardiography. TAD was obtained using M-mode echocardiography. Similar examination was performed in a control group of 17 healthy individuals in matched-age. According to the different curve degree, all patients were divided into 3 groups (mild, moderate and severe). Comparison was done among the groups and the relationship between TAD and spine curve of Cobb was analyzed. RESULTS: Patients with severe scoliosis showed depressed TAD. There was good correlation between TAD and spine curve of Cobb. CONCLUSIONS: Patients with severe scoliosis showed a significant lower right ventricular systolic function.

Endovascular repair of a type B aortic dissection with a ventricular septal defect occluder.

We report a successful endovascular repair of a type B aortic dissection (TB-AD) with a ventricular septal defect (VSD) occluder. In a 39-year-old male patient with TB-AD, the proximal entry tear was 5 mm distal to the orifice of the left subclavian artery (LSA). Ascending aorta-left common carotid artery (LCCA)-LSA bypass was performed, followed by obliterating the proximal entry tear with a VSD occluder endovascularly. One-month follow-up computed tomography angiography showed optimal position of the VSD occluder, absence of leak, complete false lumen thrombosis in the thoracic segment, expansion of the true lumen, and shrinkage of the false lumen.

[Effects of transcatheter closure in children with congenital coronary artery fistula].

OBJECTIVE: To observe the effects of transcatheter closure method for treating congenital coronary artery fistula (CAF) in children. METHODS: Twenty-three children with CAF received transcatheter closure. Under anesthesia, heart catheterization and selective coronary angiography were performed to show the CAF size and relationship with normal coronary artery. CAF with the narrowest inner diameter < 3 mm (n = 16) were occluded with coil device, and CAF with narrowest inner diameter > 3 mm (n = 7) were closed with Amplatzer duct or VSD occluder. RESULTS: Transcatheter closure was successfully performed in 21 cases and failed in 2 cases (CAF is too tortuous in one case and right CAF outlet near the right coronary artery main stem in another case) and CAF were closed by surgery in these 2 patients. No residual shunt or other complications were observed during the 3 months to 3 years follow up. CONCLUSION: Transcatheter closure was an effective and mini-traumatic method for CAF treatment in children.

[Early complications following transcatheter occlusion of perimembranous ventricular septal defects in children].

OBJECTIVE: To evaluate the early complications during and after transcatheter closure of perimembranous ventricular septal defects (PMVSDs) in children. METHODS: A total of 223 patients received transcatheter closure of PMVSDs from March 2002 to December 2005 in our hospital were included in this retrospective study. RESULTS: The overall complications rate was 26.9% (60/223). Major complications occurred in 9 patients (4.0%) including III degrees atrioventricular block (AVB) in 2 (0.9%), hemolysis in 3 (1.3%) and surgical interventions in 4 patients (1.8%) because of device malposition (1), mild aortic regurgitation (2) and device embolization (1) and all 4 patients recovered without further complications. The 2 patients with III degrees AVB were completely recovered to normal sinus rhythm after 7 days treatment with temporary pacemaker and corticosteroid. Hemolysis in 3 patients disappeared after corticosteroid treatment. Minor complications occurred in 51 patients (22.8%) including bundle branch block (BBB) in 37 (16.6%), first-degree AVB in 2 (0.9%), second-degree AVB in 1 (0.4%), new-onset mild aortic regurgitation in 5 (2.2%) and new-onset mild to moderate tricuspid regurgitation in 6 patients (2.6%). Except for right bundle branch blocks, other BBBs were treated with albumin and corticosteroid and completely recovered. No treatment was applied for new-onset valve regurgitations. There was no death in all 223 patients. CONCLUSIONS: Early complications post PMVSDs in children are mostly minor with good prognosis and the prognosis for major complications post PMVSDs is good after proper treatment.

[A follow-up study on transcatheter closure of patent ductus arteriosus with Amplatzer duct occluder in children].

OBJECTIVE: To document the five-year follow-up results of transcatheter Amplatzer duct occluder (ADO) occlusion of patent ductus arteriosus (PDA) in children and to assess the safety and effectiveness of this method for PDA closure. METHODS: A retrospective cohort study was completed in patients with PDA underwent transcatheter closure by the ADO in Guangdong Cardiovascular Institute from April 1998 to December 2003. RESULTS: Transcatheter closure of PDA with ADO was attempted in 250 children patients. The median age was 5.3 years and median weight was 15.1 kg. The PDAs were from 1.8 mm to 11.0 mm (median 4.2 mm) in the narrowest diameter, of which 52 were larger than 5 mm (20.0%). All PDAs were occluded with 4 approximately 14 mm ADO through 6F (n = 205) or 7F (n = 45) sheaths. Devices were successfully implanted in 245 patients (98.0%). The Qp/Qs decreased from 1.90 +/- 0.60 to 1.03 +/- 0.21 (P < 0.05). All shapes of the PDA could be closed. Late complication occurred in five patients, including hemolysis in three patients and the los of the pulse of femoral artery in two patients. Follow-up after device implantation was accomplished in 205 patients (82.0%). The incidence of residual shunt at follow-up periods of 1 d, 1 m, 6 m, 12 m, 24 m, 36 m, 48m and 60 m after device occlusion was 9.2%, 2.8%, 1.2%, 0.8%, 0, 0, 0 and 0, respectively. Five patients (2.0%) required re-intervention to treat residual shunt with or without hemolysis. Event-free rates were 98.0% at one year and five years. CONCLUSION: This long-term follow-up result confirmed the safety and effectiveness of ADO closure of PDAs in children.

[Applying interventional treatment for the atrial septal defect in 165 children under five years of age].

OBJECTIVE: To study the clinical technology of transcatheter closure of secundum atrial septal defects (ASD) with Amplatzer device in younger and lower body weight children. METHODS: The transcatheter closure of ASD using Amplatzer septal occluder (ASO) was performed in 165 children under 5 years of age (75 boys and 90 girls) with secundum ASD from Aug 1998 to May 2004. The age of the cases ranged from 2 to 5 (mean 3.7 +/- 1.1) years. The body weight ranged from 9 to 18 (mean 12.6 +/- 2.3) kg. The ratio of pulmonary circulation quantity to the systemic circulation quantity (Qp/Qs) was 3.2 +/- 1.9. All the patients underwent clinical examination, X-ray, electrocardiography (ECG) and echocardiography (Echo) for diagnosis of secundum ASD. The transthoracic echocardiography (TTE) was used to detect and measure the defect of the patients and even trans-esophageal echocardiography (TEE) had to be used when it was necessary. With Echo and X-ray guidance, the measuring balloon was used in the body and outside the body to determine the balloon-stretch diameters of ASD, and proper occluders were selected accordingly for the patients for interventional treatment of ASD. RESULTS: The devices were implanted successfully in 163 (98.8%) cases. One failure occurred in a case in whom the device moved into the left atrium after release, and the other failure was that the position of the device was uncertain because of temporary unavailability of a special transducer for TEE. Surgical operations were performed for these two cases. The stretch diameter of ASD was from (8 - 30) mm, (mean 18.3 +/- 5.1) mm. The size of device was selected according to the stretch diameter of ASD. The diameter of the occluders selected was from (8 - 30), (mean 18.6 +/- 5) mm in this series. The occlusion procedure was monitored by fluoroscopy and TTE and in 5 cases (3%) by TEE. The diameter of right ventricle was improved within 2 days after occlusion from (mean 16.4 +/- 4.9) mm to (mean 12.6 +/- 3.8) mm, (p < 0.01). One hundred and forty seven cases belonged to the simple secundum ASD(89%). Thirteen cases who were complicated with other cardiac deformity were treated successfully with different interventional procedure. Six cases had multiple openings and three of these cases had tumour-like changes of the atrial septum which were closed completely just by one occluder. In only one case small quantity of residual shunt remains. No other severe complication was found in this group. About 100 cases (60%) had large ASD, so the procedure was more difficult in those cases. CONCLUSION: The clinical effectiveness of treatment of ASD in children under 5 years of age with Amplatzer occluders was satisfactory and therefore this therapeutic procedure is feasible for this age group of patients. Nevertheless, we do not recommend to use the technique for infants and children under 2 years of age. Strict selection of indications and proper size of occluder and good cardiologic and surgical settings are among the basic factors for successful interventional occlusion of ASD in young children.