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.

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.

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.

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.

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.

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.

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.

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.

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.

Myocardial diseases in Thai children.

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.