Feasibility and 1-year outcome of transcatheter closure of perimembranous ventricular septal defects with different devices.

OBJECTIVE: To analyze feasibility of closing perimembranous ventricular septal defect (pmVSD) with different devices by percutaneous approach and determining initial 1-year outcome of the procedure. BACKGROUND: Transcatheter closure of pmVSD remains controversial due to a previous higher incidence of complete heart block (CHB), especially with the Amplatzer pmVSD occluder. Recently, several devices have been used to minimize the procedure-related complications. METHODS AND MATERIALS: A retrospective longitudinal cohort study of 133 patients who underwent transcatheter closure of pmVSD from September 2009 to March 2015. The median age and weight at intervention were 7.1 years (ranging from 9 months to 28 years) and 21.2 kg (ranging from 6.4 to 93 kg). RESULTS: Transcatheter pmVSD closure was successfully performed in 129 cases (97%) using 13 different devices. There were two new onset severe aortic regurgitation (AR) (1.5%), one new onset severe tricuspid regurgitation (0.7%), and one CHB (0.7%). Immediately after procedure, 41 patients (30.8%) had mild to moderate residual shunt and 27 patients (20.8%) had trivial to mild AR. At 1 year, there was no CHB and 10 patients (9.6%) had tiny to mild residual shunt and 10 patients (9.6%) had trivial AR. CONCLUSION: With proper case selection, good expertise, and judicious use of various devices with respect to anatomic details of pmVSD, transcatheter closure is feasible with satisfactory early outcome.

Percutaneous balloon aortic valvuloplasty at the Queen Sirikit National Institute of Child Health: 25 years' experience.

BACKGROUND: Percutaneous balloon aortic valvuloplasty (BAV) is the treatment of choice in moderate and severe valvular aortic stenosis. In Thailand, the first procedure was performed at the Queen Sirikit National Institute of Child Health (QSNICH) in 1988. No previous studies have been reported regarding the outcome of these procedures at QSNICH. OBJECTIVE: To study the efficacy and complications of percutaneous balloon aortic valvuloplasty in the treatment of severe aortic stenosis. MATERIAL AND METHOD: A retrospective study from the medical records was performed. All patients with a diagnosis of moderate or severe valvular aortic stenosis treated with BAV from January 1988-December 2012 were recruited. Before 2008, the procedures were performed under local anesthesia with light systemic sedation. After that, most of the cases were performed under general anesthesia. The response to treatment was classified as good response, partial response or failure. The short-term complications were classified as vascular complications, arrhythmias and others. RESULTS: Sixty-eight recorded attempts on 60 patients (47 males = 71.67%) were enlisted in the study. The ages at the time of procedures ranged from 1 day to 15 years (mean 65.25, SD 53.54, median 51 months). Fourteen cases were under one year of age (20.58%). Immediate success comprised 85.29% with 65.51% classified as good response. Ten failures in nine cases all occurred before 1997. One of the cases was an attempted balloon aortic valvuloplasty with repeated failure. Three of the cases underwent open aortic valvuloplasty where one case (33.33%) survived. The other six cases refused to continue treatment and were lost to follow-up. Four deaths were reported. Repeated balloon aortic valvuloplasties were performed in eight cases. During the early phases (1988-1996), failures and mortality rates were extremely high. Experience and improved technology improved outcomes. Excluding the four dead cases, the only significant major complication occurred in a three-year-old boy with severe aortic stenosis who tore the chordae tendinae, producing severe, acute mitral regurgitation, together with ventricular tachycardia, requiring direct current cardioversion during the procedure and double valvuloplasty five years later. Other minor complications included transient cardiac arrhythmia in 12 cases, partial occlusion of femoral artery in 11 cases and groin hematoma in 8 cases. CONCLUSION: Percutaneous balloon aortic valvuloplasty was effective and safe for the treatment of moderate and severe aortic valve stenosis in pediatric patients without significant complications.

Survival analysis of Down syndrome with congenital heart disease: a 5-years registry at QSNICH.

BACKGROUND: Down syndrome (DS) is the most common chromosomal abnormality in children. Atrio-ventricular septal defect (AVSD) is the most common congenital heart disease (CHD) reported in DS. The mortality rate of DS with congenital heart disease (CHD) is 5-7 times higher than normal population. The survival rate in DS has improved with time and has reported up to 91% and 85% at one and ten years of age, respectively. OBJECTIVE: To study the prevalence of CHD, clinical course, treatment, the overall survival in patients with DS compare with those who are associated with CHD. STUDY DESIGNS: DS registry, multidisciplinary approach, single centre. MATERIAL AND METHOD: All DS patients at QSNICH with parental signed consent were enrolled in the registry. The study was conducted for 5 years starting from May 2007 to April 2012. All patients were followed-up according to schedule modified from American Academic of Pediatrics (AAP) health supervision guideline. Standard treatment was given to all those children with diagnosis of CHD and/or other associated diseases. RESULTS: Four hundred and two cases of DS were enrolled. Two cases were excluded due to the parental inconvenience. The mode and mean age of the patients at registration were 1 and 7 months (1-62). Two hundred and seventy-one cases had an initial echocardiographic diagnosis of CHD, which included 91 of Patent ductus arteriosus (PDA), 49 of Ventricular septal defect (VSD), 34 of AVSD, 34 of secondum Atrial septal defect (ASD), 6 of Tetralogy of Fallot (TOF), 2 of Coractation of Aorta (CoA), 11 of other CHD and 44 of combined lesions. During the follow-up period, spontaneous closure of PDA, VSD and ASD occurred in 46, 12 and 15 cases, respectively. After the exclusion of those who had spontaneous closure, the prevalence rate of CHD in DS was 49.8%. VSD was the most common lesion, slightly more than PDA. Ninety-eight cases underwent cardiac surgery; including 39 of associated VSD, 24 of AVSD, 26 of PDA, 3 of TOF, 4 of CoA/AA repaired, one each of Cor triatriatum and primum ASD. Twelve cases had catheter interventions, including PDA occlusion (10 cases), and 1 case of balloon dilatation of re-CoA and coils embolization of isolated major aorto-pulmonary collateral artery. Seven cases were lost at follow-up. By using the hospital information and National Health Security Office (NHSO) database, 47 infants died during the follow-up period. The causes of death were immediate post cardiac surgery in 10 cases (AVSD in 9, VSD in 1) and not associated with surgery in 37 cases including CHF in 8, pneumonia in 7, leukemia in 4, airway disease in 3, others 7 and unknown 8 cases. The overall survival at 1 and 5 years of age were 96% and 86%, respectively. DS with CHD had a significantly lower survival rate than those without CHD (p < 0.001). CONCLUSION: According to this study, the prevalence rate of CHD in DS was high. VSD was the most common lesion and has better prognosis than AVSD. The main cause of death was a cardiac problem. Follow-up patients by using modified AAP guideline and standard treatment can improve their overall survival.

Double-balloon aortic valvuloplasty at Queen Sirikit National Institute of Child Health.

BACKGROUND: Balloon aortic valvuloplasty is the treatment of choice in moderate and severe valvular aortic stenosis. In order to reduce the risk of vascular complications, a double-balloon technique has been used with good results. OBJECTIVE: To present the results od double-balloon aortic valvuloplasty at QSNICH. MATERIALS AND METHOD: Consecutive cases of severe valvular aortic stenosis treated with double-balloon aortic valvuloplasty at QSNICH were recruited in the study. Data were obtained from the medical records starting from the day of presentations to December 2011. RESULTS: There were six cases of severe valvular aortic stenosis treated with double-balloon aortic valvuloplasty at QSNICH from 2004 to 2011. The age and weight ranged from 7 months to 12 years and 6 to 53.8 kilograms, respectively. The presenting symptoms were dyspnea in 3 (50%) and asymptomatic heart murmur in 3 cases (50%). Peak-to-peak pressure gradient (PG) before the procedure ranged from 48-104 mmHg (mean 70.00, SD 18.92 mmHg). Immediately after the procedure, PG significantly decreased to 15-52 mmHg (mean 34.33, SD 14.98 mmHg, p < 0.01). On the following day after the procedure, peak instantaneous pressure gradient (PIPG) obtained from echocardiogram ranged from 17-47 mmHg (mean 36.50, SD 10.93 mmHg). PIPG were not significantly different from PG immediately after valvuloplasty. There were two cases with partial femoral occlusion. The duration of follow-up ranged from 6-54 months (median 24 months). In the follow-up period, all of the patients were asymptomatic with functional class I. Echocardiogram after the procedure revealed no significant aortic regurgitation in any of the cases. CONCLUSION: Double-balloon aortic valvuloplasty can be performed safely with very good intermediate term outcome in selected patients. Long-term outcome in Thai children should be further studied.

An unusual cause of progressive cyanosis post Fontan operation: congenital extra-hepatic porto-systemic shunt.

OBJECTIVE: To report an unusual case of progressive cyanosis post Fontan operation due to porto-systemic venous shunt and the result of its treatment. MATERIAL AND METHOD: A patient with diagnosis of progressive cyanosis post Fontan operation from porto-systemic venous shunt at QSNICH RESULTS: This is a case of twelve years old girl, who had diagnosis of situs solitus, levocardia, atrio-ventricular concordant, ventriculo-arterial concordant, hypoplastic right ventricle with large ventricular septal defect. She had pulmonary artery banding at 4 months of age followed by a non-fenestrated extra-cardiac conduit Fontan performed at 7 years and 7 months of age. During the first year of post operation, her systemic oxygen saturation (SpO2) was 93-94% after which it decreased to 87%, 84%, 75% at 1.5, 2.5 and 3 years after surgery, respectively. Clinically she also had progressive dyspnea on exertion. Diffuse pulmonary arterio-venous malformation was demonstrated by contrast echocardiogram during cardiac catheterization. Cardiac magnetic resonance angiography showed abnormal extra-hepatic portal vein to inferior vena cava shunt. After balloon test occlusion in the cath lab, which showed no change in the portal venous pressure, complete occlusion of this porto-systemic venous shunt was performed by using Amplatzer Vascular Plug II. Her systemic oxygen saturation increased to 83% with functional class I at one-year post occlusion. CONCLUSION: The present report an unusual case of progressive cyanosis post Fontan operation due to pulmonary arteriovenous malformation, which was secondary to congenital extra-hepatic porto-systemic shunt. The venous blood from the intestinal and splenic veins was partially bypassing the liver into inferior vena cava. The patient's clinical condition and SpO2 improved after transcatheter occlusion of the shunt with the device.

Outcomes of straightforward extracardiac Fontan operation in advanced-age group.

INTRODUCTION: Fontan operation is a palliative procedure used in patients with univentricular heart (UVH). There have been numerous modifications to the technique. The appropriate strategy for treating patients with UVH in Thailand is still a major concern. OBJECTIVE: To describe the outcomes of patients after straightforward Fontan operation at advanced-age and evaluate their post-surgical cardiac performance. MATERIAL AND METHOD: A cross sectional study was performed in post-Fontan operation patients at Rajvithi Hospital between 2000 and 2009. Post-operative cardiac performance was analyzed using the echocardiogram and treadmill exercise stress test (EST). RESULTS: Thirty-nine patients were enrolled in the present study. The mean age was 11.7 years and the mean age at operation was 7.4 years. The predominant systemic chamber was morphologic left ventricle. Straightforward Fontan operation was performed in 60% of cases. Mean of Fontan index, mean of McGoon ratio, and mean of Nakata index were 2.63, 2.32, and 414.15 sqmm/sqm, respectively. Mean of the PVR and PAP were 1.98 U/m2 and 11.05 mmHg. Eighteen percent of patients died in the early post-operative period. Most of the patients died from septicemia. The mean EFwas 58.43%. Systolic dysfunction in 17.9% of the cases was abnormal, whereas diastolic dysfunction was present in half of the cases. Thirty-nine percent had MPI abnormality. During EST three cases developed arrhythmias and 3 cases had hypotension. Patients were categorized into 3 groups (Group 1: normal systolic and diastolic functions, Group 2: diastolic dysfunction, Group 3: impairment of both systole and diastole). Pre-operative cardiac catheterization parameters, surgical data and cardiac performance showed no statistical significance. However, there was a significant correlation between those with abnormal cardiac performance and arrhythmias or hypotension during EST (p = 0.003). CONCLUSION: The selection of suitable cases and good pre-operative evaluation could decrease the morbidity and mortality in patients undergoing the Fontan procedure. This study also found a correlation between abnormal cardiac performance and transient cardiac arrhythmia during exercise. The evaluation of cardiac performance and EST remains to be performed for following-up of patients who have undergone the Fontan operation, even for the asymptomatic cases.

Combined aortic and pulmonic valvular stenosis: report of 2 cases.

OBJECTIVE: To report 2 cases of severe combined aortic and pulmonic valvular stenosis. MATERIAL AND METHOD: To find patients with a diagnosis of severe combined aortic and pulmonic valvular stenosis, the authors searched in the patient profile at the cardiology unit, Queen Sirikit National Institute of Child Health (QSNICH) from 1995 to 2010. RESULTS: There are 2 cases of severe combined aortic and pulmonic valvular stenosis from 19,416 case records of pediatric cardiac patients. The first one is a 9-year-old girl and the other is a male neonate. The interval between the presentations of these two cases was 10 years. The older patient, who had associated moderate aortic regurgitation, underwent aortic root replacement and pulmonic valvulotomy in the year 2000. The neonate, who presented in 2010 had undergone bilateral percutaneous balloon aortic and pulmonic valvuloplasty and required repeated balloon aortic valvuloplasty at the age of two months. Both patients responded well to treatment and were asymptomatic at the last follow-up of 10 years and 6 months respectively after treatment. CONCLUSION: Combined aortic and pulmonic valvular stenosis is a very rare congenital heart disease. The prevalence is 0.01% of congenital heart disease at QSNICH. Bilateral balloon valvuloplasty of aortic and pulmonic valve is effectively performed in this rare congenital heart disease and can be done safely in a sick neonate.

Pediatric cardiac beriberi: 3 different presentations.

The authors report 3 cases of pediatric cardiac beriberi at Queen Sirikit National Institute of Child Health during the last 10-year-period. The first two cases presented classically while the third case came with an unusual presentation. Cardiac beriberi is an old disease in modern Bangkok which can present in as several different clinical patterns. The diagnosis needs a high index of suspicion. Echocardiographic findings of right heart dysfunction and dramatic response to intravenous thiamine are diagnostic.

Double-balloon valvuloplasty for treatment of severe valvular aortic stenosis: a case report.

The authors report the first case of successful double-balloon valvuloplasty for treatment of severe valvular aortic stenosis at Queen Sirikit National Institute of Child Health, Bangkok, Thailand. The patient was a 7-month-old, 6 Kg weighed infant with the pressure gradient across the aortic valve of 76 mmHg and aortic valve annulus of 10 mm diameter. The procedure was performed under general anaesthesia. The catheterization data revealed left ventricular and ascending aorta pressure of 156/1 and 52/30 mmHg, respectively. After the procedure the left ventricular pressure decreased to 114/0, left ventricular-aortic pressure gradient to 46 mmHg and no significant aortic regurgitation. He was well at 1-month follow-up with the left ventricular-aortic pressure gradient of 12 mmHg and no significant aortic regurgitation.

Prophylaxis of symptomatic patent ductus arteriosus with oral ibuprofen in very low birth weight infants.

BACKGROUND: Patent ductus arteriosus (PDA) is a common cause of mortality and morbidity among very low birth weight infants. Oral ibuprofen suspension has been shown to have the same efficacy and safety as intravenous indomethacin in the prevention and treatment of symptomatic PDA. With lower dosage, the prevalence of side effects may decrease without changes in efficacy. OBJECTIVE: To evaluate the efficacy and side effects of low dose ibuprofen suspension for prevention of symptomatic PDA in very low birth weight infants. PATIENTS AND METHOD: A prospective, double blind, randomized controlled trial was conducted on premature neonates with gestational ages between 28-32 weeks, birth weight 1500 grams or less, at the Neonatal Unit, Queen Sirikit National Institute of Child Health (QSNICH) during October 2005 to October 2006. Only infants who had PDA on echocardiogram were included in the study. Three doses of ibuprofen suspension or placebo were randomly given at the dosage of 10, 5, 5 mg/kg every 24 hours. Daily physical examination, serial laboratory evaluation and echocardiogram were used to evaluate symptomatic PDA, complications and side effects. RESULTS: Sixty-two infants were recruited in the study and randomly assigned into the study and control group. The gestational age and birthweight of the 2 groups were similar The prevalence of symptomatic PDA was less in the ibuprofen group than in placebo group (9.86% vs. 35.48%; p = 0.015). There were no differences in the prevalence of complications and adverse effects between the two groups. CONCLUSION: Prophylactic oral ibuprofen suspension at lower dosage results in less symptomatic PDA without significant side-effects.

A comparison of oral chloral hydrate and sublingual midazolam sedation for echocardiogram in children.

OBJECTIVE: To compare the efficacy and safety of oral chloral hydrate and sublingual midazolam to sedate the children undergoing echocardiography. MATERIAL AND METHOD: A double-blind, randomized trial study in the children judged to require sedation prior echocardiogram were performed. Two hundred sixty-four patients between 6 months and 5 years of age were randomized to chloral hydrate or midazolam groups. Either 50 mg/kg of chloral hydrate orally or 0.3 mg/kg of midazolam sublingually was given in each groups. If the child was not responded within 30 minutes after the first dose, another half dose of each drug for the second dose will be required. The action duration time, sedation score level and the ability to complete echocardiogram were collected. RESULTS: Both groups were comparable with respect to age, sex, body weight, underlying heart disease, baseline O2 saturation and functional heart classification. The children in chloral hydrate group needed the second dose for sedation more than midazolam group (10.6%, 5.3% p = 0.111). The onset, action duration and total study time were significantly shorter in midazolam than in chloral hydrate group (p < 0.001). The number of the patients who had the action duration within the optimal time (< 45 min) were significantly more cases in midazolam than in chloral hydrate group (93.1%, 43.5% p < 0.001). Success rate of echocardiogram was 99.2% in each group. There was no difference in echocardiographic time performed in both groups. The children in chloral hydrate group had deeper in level of sedation (p < 0.001). Both groups showed no significant difference in term of the ability to complete echocardiographic examination. The reaction of the children to take the medication and the number of the patients who had systemic O2 saturation change more than 5%from the baseline were higher in chloral hydrate group significantly (14.4%, 4.5% p = 0.006 and 9.9%, 3.1% p = 0.025). CONCLUSION: Sublingual midazolam at the dose of 0.3 mg/kg can be used to sedate the children at age group between 6 months to 5 years who undergoing echocardiogram with comparable rate of success and safety as 50 mg/kg of chloral hydrate orally. The less depth in the level of consciousness after sedation with midazolam compare to chloral hydrate may be advantage in a high risk patient to avoid deep sedation but may be disadvantage in case who need more comprehensive echocardiographic evaluation.

Natural aortic valve complications of ventricular septal defect: a prospective cohort study.

OBJECTIVE: To study the incidence and onset of aortic valve prolapse (AVP) and aortic regurgitation (AR) in the ventricular septal defect (VSD). STUDY DESIGN: A prospective cohort study POPULATION: The less than one-year-old children with diagnosis of isolated VSD were studied from October 2000 to September 2006 at Queen Sirikit National Institute of Child Health. Clinical follow-up and echocardiographic studies were scheduled every 2-3 months in the first year of age and then every 6 months to evaluate the size, location, flow across VSD, aortic valve morphology and aortic regurgitation. RESULTS: Three hundred and twenty-one cases of VSD were followed up. One was excluded due to associated hypoplastic RV An overall of 2,644 echocardiograms were performed. The percentage of perimembranous, subpulmonic, muscular inlet and multiple types were 70.3%, 19.4%, 5.6%, 3.1% and 1.6%, respectively. Size of the VSD was diagnosed to be small, moderate, and large VSD in 62.5%, 15.9% and 21.6% respectively. At the end of the study, the incidence of AVP in subpulmonic VSD was 87.1% compared to 16.4% in perimembranous VSD, with a relative risk of 5.30 and the incidence of AR in subpulmonic VSD was 37.1% compared to 5.3% in perimembranous VSD, with a relative risk of 6.95. From the survival analysis, the patient with subpulmonic VSD developed AVP at 46%, 77%, 90% and 94% compare to 8%, 13%, 20% and 23% of perimembranous VSD at 12, 24, 36 and 48 months of age respectively (p < 0.001). The patient with subpulmonic VSD developed AR at 8%, 17%, 35% and 38% compare to 2%, 4%, 5% and 7% of perimembranous VSD at 12, 24, 36 and 48 months of age respectively (p < 0.001). At the end of the study, ninety-six cases (30%) underwent cardiac operation with the indication of heart failure or the occurrence of AR. Sixty one cases (19.1%), including two cases of subpulmonic type had spontaneous closure of VSD. Seven cases (2.2%) had lost to follow up and five cases (1.6%) died during the follow up period. CONCLUSION: The incidence of AVP and AR are high in subpulmonic VSD being much higher than perimembranous VSD with a relative risk of 5.30 and 6.95 respectively. These complications are significantly from infancy period and are an indication for early cardiac surgery.

Epidemiologic study of Kawasaki disease and cases resistant to IVIG therapy in Thailand.

The incidence of Kawasaki disease (KD) in Thailand has never been studied before. We reviewed the data from the National Registry of Thai Children who had KD between 1998-2002 to evaluate the incidence of KD and cases resistant to treatment with intravenous immunoglobulin (IVIG). Resistance to IVIG was defined as remaining febrile at least 48 hours after initial IVIG therapy. There were 710 KD patients in the registry. The incidence of KD was from 2.14 to 3.43 cases per 100,000 children aged 0-5 years. During the acute phase 15.6% of 435 patients were considered as resistant cases. Resistant cases of KD in Thai children are quite common (15.6%) even after IVIG treatment. We found that patients who had high white blood cell counts (> 16,500 cells/mm3) had a higher likelihood of being resistant.

Balloon atrial septostomy at Queen Sirikit National Institute of Child Health in the year 2001.

BACKGROUND: Balloon atrial septostomy (BAS) is a safe and effective palliative procedure for patients with cyanotic congenital heart disease. The first BAS in Thailand was performed in 1987 at our institute and there have been many changes since that time. OBJECTIVE: To evaluate the immediate and intermediate outcomes of BAS in the new century. PATIENTS AND METHOD: Thirty patients who underwent BAS from January to December 2001 were included in this study. Epidemiologic data, echocardiographic diagnosis, changing techniques from the previous report and the clinical course for patients were reviewed. The end point of follow-up for this study was December 2002. RESULTS: Thirty patients underwent BAS from January to December 2001 with an age range from 1 to 213 days (mean 30.40 +/- 41.97 days). BAS was selectively tried via the umbilical vein in 6 cases. This was successful in 4 cases. Others were performed successfully via the femoral vein. The most common diagnoses were complete transposition of the great arteries (TGA), pulmonary atresia (PA) with intact ventricular septumand transposition of the great arteries with ventricular septal defect (TGA, VSD) respectively. No immediate complications were found in the present study. There were 5 deaths prior to surgery and 2 post-operative deaths accounting for a total mortality of 23.3 per cent. CONCLUSION: BAS is a safe and effective palliative procedure for patients with various types of cyanotic congenital heart disease even in infants up to 7 months of age. The umbilical venous route can be used effectively without significant problems. Although the overall mortality is high, it is not related to BAS.

Aortic valve prolapse in subpulmonic ventricular septal defect.

BACKGROUND: Ventricular septal defect (VSD) is the most common congenital heart disease worldwide. Subpulmonic type VSD is an interesting subtype due to the aortic valve complications rate, which can change the prognosis of the VSD. Higher prevalence rates have been reported with this subtype in Eastern countries but there has been no report from Thailand so far. OBJECTIVE: 1. To determine the prevalence of subpulmonic VSD. 2. To determine the prevalence and demographic data of aortic valve prolapse (AVP) and aortic regurgitation (AR) in subpulmonic VSD. METHOD: A retrospective study of 1,977 patients with isolated VSD, diagnosed from January 1995 to June 2002 at the Cardiology Unit, Queen Sirikit National Institute of Child Health was reviewed to differentiate types of VSD. Color flow doppler echocardiogram was performed in all cases to confirm the diagnosis and differentiate the types of VSD. Patients with subpulmonic VSD were studied to find out the presence of the aortic valve prolapse and aortic regurgitation. Those who had subpulmonic VSD were called for reevaluation of aortic valve complications, from January 2000 to June 2002. MAIN OUTCOME MEASURE: Subpulmonic VSD, aortic valve prolapse (AVP) and aortic regurgitation (AR). RESULTS: Subpulmonic VSD was diagnosed in 312 cases (17.5%). At the mean age of 3.47 yr, AVP was found in 101 cases (32.4%) and AR was found in 54 cases (17.3%) at the first echocardiogram. Subsequent echocardiography follow-up showed that the overall number of AVP cases was 153 (49%) and AR was 84 (26.9%) at the mean age of 5.5 and 6.3 year respectively. CONCLUSION: The prevalence of subpulmonic VSD was high among Thai children. Aortic valve complication is common in this group and can occur from early infancy. The present findings support the progressive increase in this complication with age.

Treatment of Kawasaki disease using locally product intravenous immunoglobulin.

BACKGROUND: Kawasaki disease (KD) is an acute febrile illness of unknown origin. Several kinds of IVIG have been shown to be effective in the decrease of the incidence of coronary artery abnormalities (CAA) which is the most serious complication. Nowadays, the National Blood Centre, Thai Red Cross Society can manufacture IVIG from the plasma of Thai blood donors which is much cheaper than the imported intravenous immunoglobulin (IVIG's). The effectiveness of this Thai Red-Cross IVIG in Kawasaki disease has never been documented. OBJECTIVE: To determine the initial treatment response and prevalence of CAA in KD treated with a high dose (2 g/kg) of Thai Red-Cross IVIG. PATIENTS AND METHOD: All patients with a diagnosis of KD who had initial treatment with 2 g/kg of Thai Red-Cross IVIG at Queen Sirikit National Institute of Child Health from December 2001 to February 2003 were reviewed retrospectively. RESULTS: All 22 cases (100%) had good response to a single treatment with a high dose of IVIG. The only patient who did not complete the IVIG treatment had a severe reaction following the administration of the drug and needed discontinuation and intervention. During the convalescent phase, there were only 2 cases (9.09%) with CAA in the first month and 1 case (4.55%) at the 3-month follow-up. This only case with CAA at 3 months had multiple aneurysms at the left main and left anterior descending coronary arteries. No giant aneurysms were found in the present study. CONCLUSION: The efficacy of a high dose (2 g/kg) of Thai Red-Cross IVIG in Kawasaki disease is excellent. However, the severe reaction in one patient needs further surveillance.

Myocardial diseases in Thai children.

UNLABELLED: Myocardial diseases are among the important causes of mortality and morbidity in children. This drew the authors attention to the study of myocardial diseases in children to find out the outcome, factors affecting the outcome, and management strategies. The authors retrospectively studied children who had been diagnosed with primary myocardial diseases at six university hospitals in Thailand from January 1996 to December 2000. The total number of cases was 209 which accounted for 1.2 per cent of cardiovascular diseases in children. The patients' ages ranged from 0.1-15 years. These myocardial diseases included dilated cardiomyopathy (DCM) 45 per cent, acute myocarditis 27.3 per cent, hypertrophic cardiomyopathy (HCM) 18.2 per cent, hypertrophic obstructive cardiomyopathy (HOCM) 8.1 per cent and restrictive cardiomyopathy (RCM) 1.4 per cent. Fifty-six per cent of the patients were female. Congestive heart failure was the most common presenting symptom (75%). Median ejection fraction (EF) of acute myocarditis was 42 per cent (15-79%) which was significantly higher than DCM (33.5%, 10-57%). Serum cardiac troponin T (cTnT) was also significantly higher in acute myocarditis than in DCM (0.08 ng/ml, 0.01-0.16 vs 0.01 ng/ml, 0.01-0.10). Within the follow-up period of 1 year (0.1-5.5 years), the mortality rates were 18.8 per cent, 17.0 per cent, 5.4 per cent and 33.3 per cent in DCM, acute myocarditis, HCM and RCM respectively. Factors associated with the mortality rate in acute myocarditis were admission to ICU and low left ventricular EF at presentation while IVIG administration and cTnT level did not. CONCLUSION: Primary myocardial diseases are uncommon. Most of the patients had compromised cardiovascular reserve. Admission to ICU and low EF were factors that affected the mortality in acute myocarditis while intravenous immunoglobulin administration did not. Mortality rate in the subacute follow-up period was high in all groups.