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.

Concomitant dengue infection and Kawasaki disease in an infant: a case report and literature review.

A previously healthy 11-month-old girl presented with fever and rash for 6 days. Physical examination revealed an irritable infant with a high fever, injected conjunctivae, red cracked lips, posterior auricular lymphadenopathy, hepatomegaly, generalized erythematous maculopapular rash and petechial hemorrhage on trunk, face and extremities. Complete blood count showed atypical lymphocytosis and thrombocytopenia. Dengue infection was initially diagnosed. The persistent fever and clinical manifestations of Kawasaki disease (KD) were observed on day 8 with high erythrocyte sedimentation rate (56 mm/hr). Treatment of KD included intravenous immunoglobulin on day 9 of the illness. Desquamation of the fingers was found on day 15 of the illness. Ectasia of left coronary artery with small aneurysmal dilatation was detected by echocardiography on day 15 of the illness. Hemagglutination-inhibition test and enzyme-linked immunosorbent assay for dengue virus eventually showed a four-fold rising. According to the literature review, this is the second reported case of dengue infection concomitant with KD. The natural course of each disease may be modified and causes some difficulties in diagnosis and management.

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.

Selection of coil for transcatheter closure of small patent ductus arteriosus.

The authors reported the results in transcatheter coil occlusion of patent ductus arteriosus (PDA) less than 4 mm, based on a policy in selection of the appropriate type and number of coils for size of PDA. The authors used one 0.035 inch detachable coil, 5 mm in diameter, in PDA less than or equal to 2 mm, and two 0.035 inch detachable coils or one controlled release 0.052 inch Gianturco coil in PDA larger than 2 mm. The present study included 32 pediatric patients. There were 31 cases of successful coil implantation and 1 case failed. Of the 31 successful cases, PDA size varied from 1.4 to 4.0 mm (mean of 2.7 +/- 0.9 mm). Ten patients had a PDA size of less than or equal to 2 mm (group A), while the other 21 patients had a PDA size of larger than 2 mm (group B). In group A, 9 cases had single-detachable-coil occlusion and one case had double-detachable-coil occlusion. In group B, double-detachable-coil occlusion was performed in 17 cases and controlled release 0.052 inch coil in 4 cases. There were no cases of coil migration or other serious complications. The immediate complete occlusion rate was 58 per cent (18 of 31 cases), which rose to 97 per cent (30 of 31 cases) at the mean follow-up of 2.6 +/- 2.5 months (range from 1 day to 9 months). Transcatheter coil occlusion is an alternative to surgical closure of small PDA (less than 4 mm). Selection of type and number of coils appropriate to the size of PDA will allow safe and excellent results.