Prevalence and predictive factors of malnutrition in Thai children with congenital heart disease and short-term postoperative growth outcomes.

Background: Growth restriction is still a common problem in children with congenital heart disease (CHD). Evidence demonstrates that performing cardiac surgery in appropriate timing may result in better growth outcome. Aim: To investigate prevalence and associated factors of malnutrition in pediatric patients with CHD who underwent cardiac surgery. In addition, post-operative growth outcomes at two weeks following cardiac surgery were also assessed. Methods: A retrospective cohort study was conducted in pediatric patients who underwent cardiac surgery at Chiang Mai University Hospital between January and September 2014. Results: One hundred patients with a median age of 28.5 months (range 14-62 months) were enrolled. Two-third of these patients had at least one form of malnutrition before receiving surgical treatment while wasting, stunting and combined wasting-stunting accounted for 23%, 28%, and 15% of patients, respectively. Multiple logistic regression analysis demonstrated that congestive heart failure-related symptoms were significantly associated with increasing risk of malnutrition (adjusted OR 4.4; 95% CI 1.78-11.26, p = 0.001). Two weeks after hospital discharge, wasting patients with regardless of stunting had significantly improved weight for height (WHZ) and weight for length Z-scores (WLZ) compared to growth parameters at the time of cardiac surgery, p = 0.012 and p < 0.001, respectively. Conclusion: The prevalence of acute and chronic malnutrition in pediatric patients with CHD who underwent cardiac surgery was very high in this study. Children with congestive heart failure had a four-time at risk of undernutrition. In short-term, cardiac surgery may mitigate acute malnutrition of these patients.

Clinical characteristics and immunological status of patients with 22q11.2 deletion syndrome in Northern Thailand.

BACKGROUND: 22q11.2 deletion syndrome is one of the most prevalent microdeletion syndromes in humans. The syndrome is characterized by extensive phenotypic variability. OBJECTIVE: to investigate the clinical characteristics, immunological features, and intellectual status of 22q11.2 deletion syndrome patients at Chiang Mai University Hospital, Thailand. METHODS: Patients who had a confirmed diagnosis of 22q11.2 deletion syndrome by fluorescent in situ hybridization (FISH) were enrolled. Data collated and evaluated included that pertaining to history, physical examination, laboratory testing including T-cell, immunoglobulin, calcium, thyroid and parathyroid levels in the blood, cardiac and urological imaging, and intellectual status. RESULTS: 34 patients diagnosed with 22q11.2 deletion syndrome; 18 (53%) were female. The median age of patients was 18.5 months (IQR; 1.5-35.8). Ninety-one percent of patients had characteristic facial features; 94% had a congenital heart defect with tetralogy of Fallot being the most frequent (72%); 88% had hypocalcemia, and 35% had genitourinary tract abnormalities. Recurrence of 22q11.2 deletion syndrome in the family was detected in 18% of cases. Twenty-eight patients (82%) were found to have a low number or percentage of T-cells. Five patients (15%) had low immunoglobulin levels. Intellectual disability (IQ/DQ scores < 70) were found in 20 out of 25 patients who were evaluated (80%), whereas the other five (20%) performed at a level of borderline intellectual function. CONCLUSIONS: Tetralogy of Fallot, hypocalcemia, immunologic defect, and cognitive impairment were common in our 22q11.2 deletion syndrome study group. We recommend that all affected patients have a multi-system evaluation by a comprehensive care team.

Liver nodules after the Fontan operation: role of the computerised tomography scan.

Currently, there is an increasing prevalence of liver nodules in patients following the Fontan operation. The appropriate non-invasive modalities have been applied to assess a diagnosis of Fontan-associated liver disease. The aims of this study were to determine the prevalence and associated factors for the presence of liver nodules using CT scan. A cross-sectional study of 34 patients older than 15 years of age was recruited. Ultrasound upper abdomen, ultrasound liver elastography, and CT scan of the upper abdomen were performed after the Fontan operation. The median age of patients was 20 years (range 14-36 years). The median age at the Fontan operation was 7 years (range 5-17 years) and the duration after the Fontan operation was 12 years (range 4-22 years). The prevalence of liver nodules was 62% as detected by CT scans. Hepatic vein pressure in patients with liver nodules was significantly higher than in those without liver nodules. Hepatic vein pressure above 13 mmHg was a factor associated with liver nodules. There was little agreement between the ultrasound of the upper abdomen and CT scan of the upper abdomen in the evaluation of liver nodules. Hepatic pressure was the only associated factor for the occurrence of liver nodules in patients following the Fontan operation. The prevalence of liver nodules was very high after the Fontan operation. The upper abdomen CT scan should be performed for the surveillance of liver nodules in every Fontan patient over the age of 15 years.

Associated Factors of Liver Disease After Fontan Operation in Relation to Ultrasound Liver Elastography.

Although the Fontan operation can improve outcomes, surviving patients still face Fontan-associated liver disease (FALD). The aim of this study was to determine the associated factors of FALD in relation to ultrasound liver elastography. A cross-sectional study was conducted for all patients on whom an ultrasound upper-abdomen and ultrasound liver elastography was performed at more than 1 year after the Fontan operation. The data consisted of the age at operation, type of Fontan operation, and laboratory data such as gamma-glutamyl transferase (GGT), aspartate aminotransferase-to-platelet ratio index (APRI), and fibrosis-4 (FIB-4) score. Cardiovascular evaluations included echocardiography and cardiac catheterization. Eighty patients with a median age of 12 years (range 5-36 years) were eligible for the study. The prevalence of FALD was 41%. For the purpose of univariate logistic regression analysis, the age at the Fontan operation, time elapsed since the Fontan operation, previous Glenn shunt, presence of fenestration, mean pulmonary artery pressure and IVC pressure post-Fontan operation, platelet count, GGT, and FIB-4 score were considered to be factors significantly associated with FALD. Following an adjustment by multivariate logistic regression analysis, age greater than 7 years at the time of Fontan procedure, time elapsed of more than 9 years since the procedure, and GGT level > 130 U/L were found to be significantly associated with FALD. Patients with these factors should be investigated for FALD.

Loss of function in ROBO1 is associated with tetralogy of Fallot and septal defects.

BACKGROUND: Congenital heart disease (CHD) is a common birth defect affecting approximately 1% of newborns. Great progress has been made in elucidating the genetic aetiology of CHD with advances in genomic technology, which we leveraged in recovering a new pathway affecting heart development in humans previously known to affect heart development in an animal model. METHODS: Four hundred and sixteen individuals from Thailand and the USA diagnosed with CHD and/or congenital diaphragmatic hernia were evaluated with chromosomal microarray and whole exome sequencing. The DECIPHER Consortium and medical literature were searched for additional patients. Murine hearts from ENU-induced mouse mutants and transgenic mice were evaluated using both episcopic confocal histopathology and troponin I stained sections. RESULTS: Loss of function ROBO1 variants were identified in three families; each proband had a ventricular septal defect, and one proband had tetralogy of Fallot. Additionally, a microdeletion in an individual with CHD was found in the medical literature. Mouse models showed perturbation of the Slit-Robo signalling pathway, causing septation and outflow tract defects and craniofacial anomalies. Two probands had variable facial features consistent with the mouse model. CONCLUSION: Our findings identify Slit-Robo as a significant pathway in human heart development and CHD.

TFAP2B mutation and dental anomalies.

Mutations inTFAP2B has been reported in patients with isolated patent ductus arteriosus (PDA) and Char syndrome. We performed mutation analysis of TFAP2B in 43 patients with isolated PDA, 7 patients with PDA with other congenital heart defects and 286 patients with isolated tooth agenesis with or without other dental anomalies. The heterozygous c.1006G>A mutation was identified in 20 individuals. Those mutation carriers consisted of 1 patient with term PDA (1/43), 16 patients with isolated tooth agenesis with or without other dental anomalies (16/286; 5.6%), 1 patient with PDA and severe valvular aortic stenosis and tooth agenesis (1/4) and 2 normal controls (2/100; 1%). The mutation is predicted to cause an amino-acid substitution p.Val336Ile in the TFAP2B protein. Tfap2b expression during early mouse tooth development supports the association of TFAP2B mutation and dental anomalies. It is hypothesized that this incidence might have been the result of founder effect. Here we report for the first time that TFAP2B mutation is associated with tooth agenesis, microdontia, supernumerary tooth and root maldevelopment. In addition, we also found that TFAP2B mutations, the common causes of PDA in Caucasian, are not the common cause of PDA in Thai population.

A Longitudinal Study of Growth and Relation With Anemia and Iron Overload in Pediatric Patients With Transfusion-dependent Thalassemia.

Short stature is one of the most common endocrinopathies in transfusion-dependent thalassemia (TDT). This study aimed to determine the longitudinal pattern of growth in pediatric patients with TDT and study the relationship between growth and hemoglobin level, serum ferritin level/iron overload parameters, and other clinical factors. The interval height-for-age Z-scores (HAZ) of 50 patients with TDT, of a mean age of 13.3±2.8 years, were analyzed using linear mixed model analysis. Nineteen patients (38%) had short stature with HAZ≤-2.0. The prevalence of short stature increased with age. The estimated mean HAZ decreased by 0.19 SD per year from the age of 5 years until approximately 14 years (95% confidence interval [CI], -0.22 to -0.16, P<0.001). Male sex (estimate, -0.28; 95% CI, -0.43 to -0.14; P<0.001), mean 3-year hemoglobin level ≤8 g/dL (estimate, -0.36; 95% CI, -0.53 to -0.19; P<0.001), mean 3-year ferritin level ≥1800 ng/mL (estimate, -0.44; 95% CI, -0.59 to -0.29; P<0.001), and cardiac T2* ≤20 ms (estimate, -1.05; 95% CI, -1.34 to -0.77; P<0.001) were significantly associated with short stature. In conclusion, short stature in patients with TDT is common and relates significantly with increasing age, male sex, hemoglobin level, and iron overload status.

Outcome following acute and recurrent rheumatic fever.

BACKGROUND: Rheumatic carditis is the leading cause of permanent disability caused by damage of the cardiac valve. This study aimed to determine the outcome and predictors of valve surgery in patients with acute rheumatic fever (ARF) and recurrent rheumatic fever (RRF). METHODS: This was a retrospective study of patients diagnosed with ARF and RRF between 2006 and 2021. The predictors of valve surgery were analysed using multivariable Cox proportional regression. RESULTS: The median age of patients with ARF and RRF (n=92) was 11 years (range 5-18). Seventeen patients (18%) were diagnosed with RRF. The most common presenting symptoms included clinical carditis (87%), heart failure (HF) (63%), fever (49%) and polyarthralgia (24%). Patients with moderate-to-severe rheumatic carditis (88%) were given prednisolone. After treatment, the severity of valvular regurgitation was reduced in 52 patients (59%). Twenty-three patients (25%) underwent valve surgery. The incidence of HF, RRF, severe mitral regurgitation on presentation, left ventricular enlargement and pulmonary hypertension was greater in the surgical group than in the non-surgical group. Recurrent rheumatic fever (hazard ratio 7.9, 95% CI 1.9-33.1), tricuspid regurgitation (TR) gradient ≥ 42 mmHg (HR 6.3, 95%CI 1.1-38.7) and left ventricular end-diastolic dimension (LVEDD) ≥6 cm (HR 8.7, 95% CI 2.1-35.9) were predictors of valve surgery (multivariable Cox proportional regression analysis). CONCLUSION: Clinical carditis was the most common presenting symptom in patients with ARF and RRF. The majority of patients responded positively to prednisolone. These findings highlight the predictors of valve surgery following ARF, including RRF, TR gradient ≥ 42 mmHg and LVEDD ≥ 6 cm.Abbreviations: ARF: acute rheumatic fever; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; GAS: group A beta-haemolytic Streptococcus; HF: heart failure; HR: hazard ratio; LVEDD: left ventricular end-diastolic dimension; MR: mitral regurgitation; RHD: rheumatic heart disease; RRF: recurrent rheumatic fever; TR: tricuspid regurgitation.

Pheochromocytoma presenting with QT prolongation and catecholamine-induced myocarditis in a child.

Pheochromocytomas are catecholamine-producing tumors derived from the adrenomedullary chromaffin cells. The presentation is a classic triad of episodic headaches, sweating, and tachycardia. Hypertensive crisis can occur due to profuse catecholamine excess. Unusual manifestations mimicking cardiogenic shock, arrhythmia, and myocarditis have been rarely reported in children. We present a case with uncommon manifestations of pheochromocytoma in a child, including the episodes of exercised-induced presyncope with QT prolongation, and subsequently cardiogenic shock due to fulminant myocarditis. He later developed hypertensive crisis. The adrenal mass on abdominal computed tomography with an increased chromogranin A level and elevated plasma normetanephrine, and the histological study confirmed the diagnosis of pheochromocytoma. Cardiac functions completely recovered after adrenalectomy. Genetic testing was positive for von Hippel-Lindau syndrome. We describe pheochromocytoma crisis presenting with prolonged QT and catecholamine-induced myocarditis. We discuss the clues to assist in the diagnosis of this condition and its appropriate treatment.

Use of cardiac markers for monitoring of doxorubixin-induced cardiotoxicity in children with cancer.

The aim of this study was to evaluate N-terminal probrain natriuretic peptides (NT-pro-BNP), cardiac troponin T, and creatinine kinase, MB isoenzyme (CK-MB) in the determination of subclinical left ventricular (LV) dysfunction by echocardiography in patients treated with doxorubicin. We performed a cross-sectional case study of systolic, diastolic function and tissue Doppler imaging by echocardiography in children with cancer who received a certain cumulative dose of doxorubicin. Blood levels for NT-pro-BNP, cardiac troponin T, and CK-MB were analyzed within 6 hours of the cardiac study. Of 30 patients, 5 (16.7%) had LV dysfunction with an abnormally high NT-pro-BNP level of 363 ± 78 pg/mL, whereas patients with normal LV function had an NT-pro-BNP level of 148 ± 173 pg/mL (P = 0.012). The NT-pro-BNP level not only inversely correlated with fractional shortening (r = -0.43, P = 0.017) and ejection fraction (r = - 0.45, P = 0.013) but also correlated with mitral deceleration time ( r = 0.41, P = 0.021) and a cumulative dose of doxorubicin (r = 0.44, P = 0.014). For tissue Doppler imaging, NT-pro-BNP correlated with a peak systolic velocity at the myocardial segment (Sm) (r = -0.40, P = 0.027). NT-pro-BNP is a sensitive test and has a moderate relationship with the LV systolic and diastolic function, thus making it a useful cardiac marker for the monitoring of early anthracycline cardiotoxicity.

Effects of left ventricular function on the exercise capacity in patients with repaired tetralogy of Fallot.

BACKGROUND: Tissue Doppler imaging has been recently used to evaluate ventricular function. Peak oxygen uptake (V•O2peak) has been demonstrated as a predictor for death in adults with repaired tetralogy of Fallot (TOF). The aim of this study was to determine which Doppler parameters correlated with V•O2peak in patients with repaired TOF. METHOD AND RESULTS: Doppler echocardiography, tissue Doppler imaging, and exercise test were performed in 30 patients with TOF after surgical repair. In 30 patients with repaired TOF (median age 14 years, range 9-25 years), 11 patients (37%) were female. Seven patients (median age 12 years) had normal left ventricular diastolic function, whereas the rest of the patients were classified as diastolic dysfunction grade II (median age 15 years; n=15) and III and IV (median age 18 years; n=8). The oxygen uptake at anaerobic threshold (V•O2AT) and peak exercise in patients with left ventricular diastolic dysfunction was significantly lower than that in those with normal diastolic function. Also, V•O2AT and V•O2 peak in patients with diastolic dysfunction grade III and IV were significantly lower than that in those with diastolic dysfunction grade II. Left ventricular early diastolic myocardial velocity was most closely correlated to V•O2peak (r=0.51; P=0.005). Peak early ventricular filling velocity to early diastolic myocardial velocity ratio was significantly correlated with V•O2peak (r=-0.50; P=0.006). CONCLUSION: Left ventricular diastolic dysfunction is correlated with V•O2peak. Left ventricular diastolic function should be a routine echocardiographic assessment in patients with repaired TOF.

Heart rate variability and exercise capacity of patients with repaired tetralogy of Fallot.

Heart rate variability (HRV) has been used as a reliable method to detect cardiac autonomic nervous system activity. Peak oxygen uptake (VO(2) peak) has been a predictor of death for adults with repaired tetralogy of Fallot (TOF). This study investigated the correlation between HRV and exercise capacity in 30 patients with TOF after surgery for total correction. The median age of the patients was 14 years (range, 9-25 years), and the median follow-up period was 11.6 months (range, 5.3-20.2 months). Low- and high-frequency-domain HRV significantly correlated with VO(2) peak (r = 0.56, P = 0.001 and r = 0.44, P = 0.02, respectively). After the 1-year follow-up evaluation, VO(2) peak and HRV analysis did not differ from those at entry to the study. However, low- and high-frequency-domain HRV still correlated significantly with VO(2) peak (r = 0.43, P = 0.03 and r = 0.52, P = 0.007, respectively). Left ventricular early diastolic myocardial velocity was most closely correlated with the VO(2) peak (r = 0.51, P = 0.005). Impaired cardiovascular autonomic control and left ventricular diastolic dysfunction may be responsible for exercise intolerance in patients with repaired TOF. Long-term follow-up evaluation with exercise testing and 24-h Holter monitoring are warranted.

Risk factors for morbidity and mortality after a bidirectional Glenn shunt in Northern Thailand.

OBJECTIVES: Owing to the evolution of surgical techniques, the survival rate of patients undergoing a bidirectional Glenn shunt has improved. However, the morbidity and mortality are still high. The aims of this study were to determine the survival rate and risk factors influencing the morbidity and mortality in patients with a functional univentricular heart after a bidirectional Glenn shunt. METHODS: One hundred and fifty-one patients who had undergone a bidirectional Glenn operation were enrolled. Early worse outcomes were defined as postoperative death within 30 days and a hospital stay ≥ 30 days. RESULTS: The median age was 7.1 years (range 0.3-26 years). The median age at the time of the Glenn operation was 2.2 years (range 0.2-15.9 years). The survival rates of patients at 1-, 5-, 10- and 15-year after the Glenn operation were 89%, 79%, 75%, and 72%, respectively. The predictors for the mortality were preoperative mean pulmonary artery pressure ≥ 17 mmHg, preoperative pulmonary vascular resistance index ≥ 3.1 Wood Units·m2 and atrioventricular valve regurgitation. In addition, the independent predictors of an early worse outcome included preoperative mean pulmonary artery pressure ≥ 17 mmHg and diaphragmatic paralysis. CONCLUSION: The presence of preoperative atrioventricular valve regurgitation, preoperative mean pulmonary artery pressure ≥ 17 mmHg, preoperative pulmonary vascular resistance index ≥ 3.1 Wood Units·m2, or diaphragmatic paralysis were found to be independent risk factors requiring the good patients' selection for the Glenn operation and early aggressive management of the diaphragmatic paralysis for reducing morbidity to ensure successful candidature for Fontan completion.

Survival and predictors of mortality in patients after the Fontan operation.

BACKGROUND: As a result of the surgical techniques now being employed, the survival rate in patients after undergoing the Fontan operation has improved. The aims of this study were focused on determining the survival rate and predictors of early mortality. METHODS: In a retrospective cohort study, 117 consecutive patients who underwent the Fontan operation were recruited. Multivariate Cox proportional regression analysis was used to assess the predictors of early mortality, defined as death within 30 days after the Fontan operation. RESULTS: The median follow-up time was 6.1 years. The median age at the time of the Fontan operation was 5.7 years. Survival rates in the patients at 5, 10, and 15 years postoperatively were 92%, 87% and 84%, respectively. Using univariate Cox regression analysis, the predictors of early mortality were found to be postoperative mean pulmonary artery pressure ≥23 mm Hg (hazard ratio 26.0), renal failure (hazard ratio 9.5), heterotaxy syndrome (hazard ratio 5.3), and uncorrected moderate or severe atrioventricular valve regurgitation (hazard ratio 9.4). After adjusting for confounding factors using multivariate Cox regression analysis, the predictors of early mortality were found to be postoperative mean pulmonary artery pressure ≥23 mm Hg (hazard ratio 23.2) and uncorrected moderate or severe atrioventricular valve regurgitation (hazard ratio 8.2). CONCLUSIONS: Uncorrected moderate or severe atrioventricular valve regurgitation and postoperative mean pulmonary artery pressure ≥23 mm Hg are independent predictors of early mortality after the Fontan operation. Patients with these factors should undergo aggressive management to minimize morbidity and mortality.

Tissue Doppler echocardiography reliably reflects severity of iron overload in pediatric patients with beta thalassemia.

AIMS: Tissue Doppler imaging has been recently used to evaluate ventricular function in patients with beta thalassemia. In clinical practice, serum ferritin is commonly used to assess the severity of iron overload. The aim of this study was to determine which Doppler findings correlated with serum ferritin. METHODS AND RESULTS: Thirty-one pediatric patients with transfusion-dependent beta thalassemia with normal LVFS were evaluated. Seven patients with serum ferritin <2500 ng/ mL, 13 patients with serum ferritin 2500-5000 ng/mL, and 11 patients with serum ferritin >5000 ng/mL were studied. Diastolic dysfunction was absent in all patients with serum ferritin <2500 ng/mL, and was present in all patients with serum ferritin >5000 ng/mL. Deceleration time (DT) has a significant correlation with serum ferritin (r = -0.59, p < 0.0001). Difference of pulmonary vein atrial reversal flow and mitral valve A wave duration (PVAR - MVA) and early ventricular filling velocity to early diastolic myocardial velocity ratio (E/Em) significantly correlated with serum ferritin (r = 0.49, p = 0.006; r = 0.56, p = 0.001, respectively). CONCLUSION: Decreased DT, increased PVAR-MVA duration, and increased E/Em ratio reliably reflected severe iron overload in pediatric patients with beta thalassemia. Systolic and diastolic LV function is preserved in patients who have serum ferritin <2500 ng/mL.

Thyroid functions in children with Down's syndrome.

OBJECTIVE: To evaluate thyroid function in children with Down's syndrome, and to ascertain the presence of a relationship between overt thyroid diseases and congenital anomalies. MATERIAL AND METHOD: One hundred and forty Down's syndrome patients, aged from 3 days to 13 years 9 months, were evaluated for karyotype, thyroid functions and the coexistence of congenital anomalies. RESULTS: Trisomy 21 was found in the majority of cases (95.7%). Fifty-six patients (40%) had abnormal thyroid functions: 53 (37.9%) hypothyroidism and 3 (2.1%) hyperthyroidism. Ten patients (7.1%) were diagnosed with overt thyroid disease: congenital hypothyroidism 3.6%, acquired hypothyroidism associated autoimmune thyroiditis 1.4% and hyperthyroidism 2.1%. None of the patients with congenital hypothyroidism had athyreosis or ectopic thyroid gland. Sub-clinical hypothyroidism accounted for 32.9% of all cases; 10.7% showed a spontaneous decrease to normal TSH levels and 13.6% had persistently elevated TSH levels with the median follow-up time of 6 and 12 months, respectively. Congenital heart disease, gastrointestinal anomalies and hematological disease were found in 73.6, 10 and 3.6 percent of patients, respectively. There was no statistical correlation between the coexistence of cardiovascular or gastrointestinal disease in Down's syndrome patients with overt thyroid diseases or sub-clinical hypothyroidism to those having normal thyroid functions. CONCLUSION: Sub-clinical hypothyroidism was the most common thyroid abnormality in children with Down's syndrome. A longitudinal and timely-scheduled evaluation of thyroid function is needed to establish the natural course of this abnormality and the proper management guideline.

Predictors of intravenous immunoglobulin resistance and coronary artery aneurysm in patients with Kawasaki disease.

BACKGROUND: Patients with Kawasaki disease (KD) who have intravenous immunoglobulin (IVIG) resistance are at increased risk for development of coronary artery abnormalities. Although in Japan several risk scoring systems are able to predict patients with IVIG-resistant (KD), they do not accurately predict non-responders in other regions. AIM: The objectives of this study were to determine the predictors of IVIG resistance and coronary artery aneurysm (CAA) and to develop risk scoring systems for predicting IVIG-resistant KD in the Thai population. METHODS: A total of 217 patients with KD between 2004 and 2014 were retrospectively reviewed. All patients including 116 with complete KD and 101 with incomplete KD were diagnosed and treated with 2 g/kg IVIG. RESULTS: Twenty-six patients (85% male) with IVIG-resistant KD had a reduced platelet count and increased neutrophil-to-lymphocyte ratio compared with those with an IVIG response. Fifty-five patients with CAA eight weeks after diagnosis had a longer duration of fever (≥8 days) and increased platelet count (≥550 × 109/L) than those with non-CAA. Based on analysis by multivariate logistic regression, haematocrit ≤30%, platelet count ≤300 × 109/L, aspartate aminotransferase ≥40 U/L and neutrophil-to-lymphocyte ratio ≥3.2 were predictors of IVIG resistance. The new scoring system using these significant factors had a sensitivity of 80.8% and a specificity of 66.8% in identifying patients with IVIG resistance. Japanese scoring systems had low sensitivity and specificity. CONCLUSIONS: KD patients with reduced mean haemoglobin, increased AST level, increased neutrophil-to-lymphocyte ratio and reduced platelet count should be considered for conjunctive therapy such as a corticosteroid in combination with standard treatment. Duration of fever ≥8 days and platelet count ≥550 × 109/L were predictors of CAA. To prevent cardiovascular complications, patients should be treated promptly after KD has been diagnosed.

Factors determining immediate and medium-term results after pulmonary balloon valvuloplasty.

OBJECTIVE: To determine factors influencing immediate and medium-term results of Pulmonary Balloon Valvuloplasty (PBV) for pulmonary valve stenosis. MATERIAL AND METHOD: Between 1995 and 2001, the authors carried out PBV in 25 consecutive patients. Two treatment outcomes including immediate and medium-term results were analyzed. The immediate results were classified into two groups based on the pulmonary systolic pressure gradient (PG) immediately after dilation: group 1 with a PG < or = 35 mmHg and group II with a PG > 35 mmHg. At medium-term follow-up of 6 to 60 months, echocardiographic evaluations were analyzed and divided into two groups: group A with a PG < or = 25 mmHg and group B with a PG > 25 mmHg. Demographic characteristics and hemodynamic parameters of immediate and medium-term results were compared and analyzed. RESULTS: Immediately after PBV the right ventricular systolic pressure (RVSP) decreased from 115.3 +/- 37.6 mmHg to 67.0 +/- 28.5 mmHg (p < 0.001). The pulmonary systolic pressure gradient decreased from 90.4 +/- 37.9 mmHg to 39.3 +/- 25.6 mmHg (p < 0.001). Nine of the 25 patients (group 11; 36%), with incomplete immediate relief of the obstruction, had more symptoms, a higher baseline PG, higher right atrial pressure, higher RVSP, and a higher systolic pressure ratio. Six of the 25 patients (group B; 24%), with suboptimal medium-term results, had a higher right ventricular voltage on the electrocardiogram, higher pre-dilation PG, and higher RVSP. CONCLUSION: Successful medium-term outcomes following pulmonary balloon valvuloplasty were achieved in 76% of the patients, with a greater success rate (91%) in patients with a lower right ventricular voltage electrocardiogram (R wave amplitude in V1) < or = 21 mm, a prevalvuloplasty systolic gradient < or = 90 mmHg, and right ventricular systolic pressure < or = 125 mmHg.

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.

A mediastinal mass resembling lymphoma: an unusual manifestation of probable case of invasive zygomycosis in an immunocompetent child.

A 2-year-old girl presented with prolonged fever and progressive dyspnea for 3 weeks. A chest radiograph revealed a left lung infiltrate and associated pleural effusion. Echocardiography revealed a large posterior mediastinal mass extending to the left atrial wall and massive pericardial effusion. The presumptive diagnosis was lymphoma. At operation, a large brownish-yellow mass was noted at the posterior mediastinum, with matted hilar, and subcarinal lymph nodes. Pericardial and pleural effusions with left lung consolidation were also noted. Histopathological examination of biopsy specimens revealed a granulomatous inflammatory reaction with a diffuse eosinophilic infiltrate and broad septated fungal hyphae with right angle branching compatible with zygomycosis. Surgical removal of the mass could not be performed due to the adjacent great vessels and carina. She subsequently died from airway obstruction and respiratory failure ten days later.