Chest radiographic findings in children with asplenia syndrome.

Background: Early diagnosis of asplenia syndrome is important because prophylactic antibiotic and proper vaccination will prevent serious infection. Most children with asplenia syndrome present with symptoms of congenital heart disease. Chest X-ray is usually the first line imaging modality. Objective: Define useful findings in chest radiograph that could suggest the diagnosis of asplenia syndrome. Methods: Chest radiographs of pediatric patients who had asplenia syndrome diagnosed between January 1, 2002 and June 30, 2008 in a single institute were retrospectively reviewed for the positions of the visceral organs in the chest and abdomen. Results: Three hundred seventy one chest radiographs of 30 patients were reviewed. The mean age at diagnosis was 3 years old. Asplenia was diagnosed by ultrasound in 27 patients, by CT scan in two patients, and by damaged red blood cell scintigraphy in one patient. Six important findings detected from chest radiographs were, 1) bilateral minor fissures, 16 cases (53%), 2) bilateral eparterial bronchi, 16 cases (53%), 3) ipsilateral side of stomach and liver, 12 cases (40%), 4) ipsilateral side of minor fissure or eparterial bronchus and stomach, 10 cases (33%), 5) symmetrical transverse lie of the liver, nine cases (30%), and 6) contralateral side of minor fissure or eparterial bronchus and the liver in seven cases (23%). All except two patients (93%) had at least one of the above findings. All patients had congenital heart diseases. Most of the heart diseases were pulmonary atresia or pulmonary stenosis (88%) and single ventricle (85%). Conclusion: Chest radiographs have high sensitivity in suggesting the diagnosis of asplenia syndrome, when detecting one or more of the above findings, particular in patients with congenital heart disease and decreased pulmonary vasculature.

Idiopathic left ventricular tachycardia in children.

BACKGROUND: Idiopathic left ventricular tachycardia (ILVT) is a rare but well-recognized clinical entity. The clinical characteristics and prognosis of this form of ventricular tachycardia (VT) in Thai children is not known. OBJECTIVE: To define clinical presentations, drug therapies, roles of radiofrequency (RF) catheter ablation, and the short-term outcome of these children in Thailand. PATIENTS AND METHOD: From April 1999 to June 2007, 10 patients were diagnosed as ILVT by specific electrocardiographic features and therapeutic response. All patients had a structurally normal heart. Data were collected retrospectively. Baseline clinical information, 12-lead electrocardiography (ECG) during VT, responses to drug therapy, results of RF catheter ablation therapy, and outcome were determined. RESULTS: Median age at presentation was 9.5 years (range, 3.8 to 14.0 years). Three patients (30%) were male. Eight patients (80%) were diagnosed as supraventricular tachycardia (SVT) before ILVT diagnosis. Median duration from SVT diagnosis to the correct diagnosis was 1.5 years (range, 0 to 6.0 years). Palpitation and chest pain were usual clinical manifestations while congestive heart failure was the presentation in one due to incessant tachycardia. Two patients had recurrent VT episodes during acute febrile illnesses. The majority of patients responded to intravenous verapamil. RF catheter ablation was performed in 3 patients with recurrence of the VT in one. CONCLUSION: Prompt recognition of the ILVTespecially in the emergency department is very important. Verapamil is effective for acute termination as well as prevention of VT recurrence. When VT is refractory to medical therapy, RF catheter ablation is safe and effective. The short-term prognosis was good.

Clinical course and outcome of children with single ventricle physiology at King Chulalongkorn Memorial Hospital.

BACKGROUND: Children with single ventricle physiology comprise 10% of all children with congenital heart disease (CHD) and one-third of children with cyanotic CHD seen at King Chulalongkorn Memorial Hospital. The prognosis of these children is generally thought to be poor but no study of the outcome has previously been done in this hospital and in Thailand. OBJECTIVES: To study the clinical course and outcome of children with single ventricle physiology at King Chulalongkorn Memorial Hospital during the current era. MATERIAL AND METHOD: One hundred and seventeen patients with single ventricle physiology were initially seen at this hospital during the year 1999-2001. Retrospective chart reviews were carried out in 90 children with available medical records. The status of the patients was determined in 2003 at clinic visits, by phone calls and mail. RESULTS: The main diagnoses were tricuspid atresia (TA, n = 10), pulmonary atresia with intact ventricular septum (PA/IVS, n = 16), single ventricle associated with cardiac malposition or heterotaxy syndrome (malposition, n = 35), hypoplastic left heart syndrome (HLHS, n = 11) and other complex single ventricle (others, n = 18). Most children did not have other major anomalies. Survival of patients with TA and PA/IVS was approximately 92 and 87% at 1 and 4 years, respectively. For patients in the other three groups, 1 and 4 year survival was 69 and 42%, respectively. Patients with HLHS had the worse survival, partly because of decisions not to pursue further treatments by the parents. Among HLHS patients who underwent Norwood procedure, the 1 and 4 year survival were 83% and 42%, respectively. Approximately 40 and 90% of all patients underwent heart surgery at 1 and 4 years follow-up, respectively. CONCLUSION: Despite limited resource, the short-term outcome of Thai children with single morphologic left ventricle (TA and PA/IVS) is reasonably good. Timely evaluation and treatment of these children toward Fontan procedure should be ensured. After proper discussion with the parents, palliative care is still acceptable for patients with single morphologic right ventricle or other complex single ventricle due to poor survival in the current era. As access to health care improves, re-evaluation of these outcomes is necessary to find the best strategy for the management of these patients.

Self-expanding nanoplatinum-coated nitinol devices for atrial septal defect and patent ductus arteriosus closure: a swine model.

OBJECTIVES: Our purpose was to evaluate self-expanding nanoplatinum-coated nitinol devices for transcatheter closure of atrial septal defects and patent ductus arteriosus in a swine model. The devices were braided from platinum-activated nitinol wires and filled with polyester to enhance thrombogenicity. The platinum activation of the nitinol wires was carried out with the help of Nanofusion technology. The coating of platinum covers the exposed surface of the nitinol wires and prevents the release of nickel into the blood stream after the implantation of the device but does not affect its shape memory, which makes the device self-expanding after it is loaded from the catheter. METHODS AND RESULTS: Atrial septal defects were created in 12 piglets by balloon dilation of the patent foramen ovale. The size of the device was selected on the basis of the diameter of the balloon and the size of the defect, measured by transthoracic echocardiography. The devices were successfully deployed in all 12 piglets under fluoroscopic study. Transthoracic color Doppler echocardiograms showed complete closure of the atrial septal defect within 15 minutes of device implantation. Twelve patent ductus arteriosus closure devices were deployed in the right or left subclavian arteries in 10 piglets. Angiograms showed complete occlusion of the subclavian arteries within a few minutes of device deployment. In the atrial septal defect cases, the autopsy findings showed complete organizing fibrin thrombus formation and complete neo-endothelialization on the outer surface of the devices within one week and six weeks of implantation, respectively. CONCLUSION: The use of self-expanding nanoplatinum-coated nitinol devices for the transcatheter closure of atrial septal defects and patent ductus arteriosus is feasible. The excellent occlusion result and complete neo-endothelialization of the devices in the swine model is an indication of the potential of these devices in human application.

Infective endocarditis in pediatric patients: an eighteen-year experience from King Chulalongkorn Memorial Hospital.

OBJECTIVES: To review the clinical features and outcome of the pediatric patients diagnosed with infective endocarditis (IE) at King Chulalongkorn Memorial Hospital over an 18-year period. MATERIAL AND METHOD: From January 1987 to December 2004, 57 pediatric patients; 28 females and 29 males, age ranged from 2 months to 15 years, mean of 8.64 +/- 3.82 years, classified as "definite" IE according to the Duke criteria were reviewed. RESULTS: Of the 57 patients, 42 patients (74%) had underlying congenital heart diseases, 7 patients (12%) had underlying rheumatic heart disease, 8 patients (14%) had previously normal heart. Nine patients had history of previously palliative or corrective surgery. Blood cultures were positive in 26 patients (46%). Streptococci and staphylococci were the most commonly isolated organisms. Two-dimensional echocardiography demonstrated vegetations in 56 patients (98%). The location of the vegetations was in the right heart in 30 patients (54%) and in the left heart in 26 patients (46%). Complications occurred in 28 patients (49%). Common complications included congestive heart failure, cerebral emboli, septic shock, and mycotic aneurysm. The overall mortality was 11% (6 patients). By statistical analysis, vegetations in the left heart had a higher incidence of complication than that in the right heart. The vegetation size of > or =10 mm had a significant higher incidence of embolic events. CONCLUSION: The clinical features and outcomes of the present study have a similar pattern as the earlier studies. The rates of complications and mortality are still high.

Pediatric heart surgery waiting time in Thailand and its effect on mortality: A cooperative study from Chulalongkorn, Children and Chiang Mai University hospitals.

BACKGROUND: Thai children who need cardiac surgery are often put on a waiting list. The waiting time and mortality during waiting have not been previously systematically studied. MATERIAL AND METHOD: A cooperative study was conducted at King Chulalongkorn Memorial hospital (Chula), Children hospital (CH), and Chiang Mai University hospital (CMU). The status of children who were referred for cardiac surgery at these hospitals in the year 1999-2000 (Chula and CMU) and the year 2000 (CH) were analyzed by Kaplan-Meier survival curve. The patients who lost to follow up and could not be contacted were censored at the time of last clinic visit. Log-Rank test was used to compare the survival curve and waiting time between three hospitals. RESULTS: The averaged annual referrals for cardiac surgery at the three hospitals were 846 cases (205 for Chula, 462 for CH and 179 for CMU). Mean age was 4.3 +/- 4.2 years and 51% were male. Follow up data were complete in 96.3%. Surgical procedures were correction of simple shunt lesions (ASD, VSD, AV canal) in 33.9%, close heart surgery (PDA ligation, coarctation repair, BT shunt) in 29.9%, total repair TOF in 19.6%, complex surgery in neonate and infants (arterial switch, TAPVR repair, Norwood procedure, truncus and interrupted aortic arch repair) in 4.2% and others in 12.3%. Median waiting time was 195 days and was significantly different between the three hospitals (p < 0.01). Mortality while waiting were approximately 5% at 2 years at Chula and CH, which was significantly higher than CMU (0%, p = 0.02). Further analysis revealed difference in age (lower Chula than CH than CMU) and types of surgery (more complex at Chula and CH) which may be the causes for difference in the mortality observed. CONCLUSION: Waiting time for cardiac surgery for children in Thailand is long and should be viewed as a problem in public health policy. Optimal waiting time at each hospital may not have to be the same, depending on the type and severity of the disease seen at each particular center Attempt should be made to solve this problem at the national level.

Prevalence of idiopathic long QT syndrome in congenital sensori-neural hearing loss students of Songkhla School for the Deaf.

OBJECTIVES: To survey the prevalence of the long QT syndrome (LQTS), especially Jervell-Lange-Nielsen syndrome (JLNS), in Thai children (The first such study). BACKGROUND: LQTS is a rare inherited disease with a prevalence of 0.21 per cent in children with congenital deafness from other reports. These patients carry a high risk of recurrent syncope and fatal ventricular arrhythmia. STUDY DESIGN: Cross-sectional survey from January 2000 to August 2000. METHOD: A total of 276 children with congenital sensori-neural hearing loss were included. A questionnaire was employed and all children were examined by a pediatric cardiologist to rule out organic heart disease. EKGs were obtained and QTc intervals were blindly measured using standard methods in L2, V5 or any other leads with the longest QTc interval by three pediatric cardiologists. If QTc interval is prolonged, additional EKG (up to 3) were done to confirm the finding. Schwartz criteria was used to identify index cases with LQTS after repeated EKGs, and exercise stress tests. Also, echocardiography were done in patients suspected of having LQTS. RESULTS: A total of 14 children needed a third EKG and more work ups due to persistent long QTc interval after 2 consecutive EKG studies with QTc intervals ranged from 456 msec to 466 msec, and Schwartz score from 1.5 to 2.5. There were 6 twins and no triplets in the study. Finally, two subjects (not twins or siblings) had persistent prolonged QTc intervals after 3 EKG studies. After the exercise stress test, both still had a prolonged QTc interval, not corrected to the normal QTc interval even at the exercise peak. There was no cardiac abnormality either structurally or functionally from the 2D echocardiogram and Doppler color flow study. CONCLUSION: The possible prevalence of JLNS was 0.7 per cent (2/276). Both children were in the low-risk group for having LQTS.

A cost-benefit analysis of intravenous immunoglobulin treatment in children with Kawasaki disease.

To determine the long-term cost-benefit of intravenous immunoglobulin (IVIG) treatment in Children with Kawasaki Disease (KD), a model was made to compare the total cost for management of these children with and without the use of IVIG. Long-term (10-21 years) follow-up of 594 KD patients treated in the pre-IVIG era reported by Kato, et al. was used to calculate cost using previous cost studies from Chulalongkorn Hospital. Reduction of CAA from 25 per cent to 4 per cent with IVIG treatment was assumed based on previous published data. Total cost was slightly lower for the non-IVIG treatment group compared to the IVIG treatment group (33,451,129 baht vs 35,001,195 baht) for the duration of follow-up in Kato's model. Cost per effectiveness analysis showed more effectiveness in the IVIG treatment group (359,576 baht vs 383,614 baht). Net cost analysis similarly demonstrated lower costs in the IVIG treatment group (25,365,215 baht vs 33,451,129 baht). Incremental cost-effectiveness analysis demonstrated supplementary costs of 13,663 baht for one case in the reduction of coronary involvement and 387,517 baht for one life saved in the IVIG-treated group. Estimation of total costs for follow-up and treatment for healthy life (until 60 years old) was more expensive in the non-IVIG treatment than the IVIG treated group (75,482,803 baht vs 29,883,833 baht). The authors conclude that treatment of all KD cases in Thailand with IVIG is likely to result in lower cost and better outcome when compared to no treatment with the IVIG policy.

Resident recruitment: what are good predictors for performance during pediatric residency training?

The authors evaluated the value of the Cumulative Grade Point Average (GPAX), letters of recommendation and personal interview in predicting performance of first year residents at the Department of Pediatrics, Faculty of Medicine, Chulalongkorn University. The pre-residency data of 25 first year pediatric residents were compared to the results of the Pediatric In-training Examination (PIE) and clinical evaluation by faculty during the first 7 months of training. The Clinical Performance Rating Score (CPRS) developed by the Royal College of Pediatricians of Thailand was used for clinical performance evaluation. GPAX correlated strongly with PIE score (r = 0.69, p < 0.001) while letters or recommendation and interview score were not predictive. For the majority of residents who graduated from Chulalongkorn University, including the undergraduate program into the regression model improved this association (r = 0.90, p < 0.001). Interview score correlated moderately (r = 0.49, p = 0.02) with clinical performance measured by CPRS which entailed the evaluator to assess a resident in 6 specific areas. GPAX and letters of recommendation showed a trend toward positive correlation with clinical performance, but these associations were weak (r = 0.32-0.39). Further analysis of letters of recommendation found a significant difference between the score given by evaluators in community hospital settings compared to the score given by medical school faculty or evaluators in large regional or provincial hospitals. The authors conclude that the cognitive function of pediatric resident candidates can be assessed reliably by GPAX. Research on how to adjust for the variability of GPAX given by an individual undergraduate program will improve the residency selection process. A structured interview may correlate better with global clinical performance than GPAX or letters of recommendation but all methods are at best moderately predictive of clinical performance. Improvement can be made by continuous evaluation and adjustment of the selection process.