The effect of coenzyme Q10 on idiopathic chronic dilated cardiomyopathy in children.

The objective of this study was to assess the effect of coenzyme Q10 (CoQ10) as supplementation to conventional antifailure drugs on quality of life and cardiac function in children with chronic heart failure due to dilated cardiomyopathy (DCM). The study was an open-label prospective study performed in two of the largest pediatric centers in Thailand from August 2000 to June 2003. A total of 15 patients with idiopathic chronic DCM were included, with the median age of 4.4 years (range, 0.6-16.3). Presenting symptoms were congestive heart failure in 12 cases (80%), cardiogenic shock in 2 cases (13.3%), and cardiac arrhythmia in 1 case (6.7%). Sixty-one percent of patients were in the New York Heart Association functional class 2 (NYHA 2), 31% in NYHA 3, and 8% in NYHA 4. Cardiothoracic ratio from chest x-ray, left ventricular ejection fraction, and left ventricular end diastolic dimension in echocardiogram were 0.62 (range, 0.55-0.78), 30% (range, 20-40), and 5.2 cm (range, 3.8-6.5), respectively. CoQ10 was given at a dosage of 3.1 ? 0.6 mg/kg/day for 9 months as a supplementation to a fixed amount of conventional antifailure drugs throughout the study. At follow-up periods of 1, 3, 6, and 9 months, NYHA functional class was significantly improved, as was CT ratio and QRS duration at 3 and 9 months follow-up with CoQ10 when compared to the baseline and post-discontinuation of CoQ10 at 9 months (range, 4.8-10.8). However, when multiple comparisons were taken into consideration, there was no statistical significant improvement. In addition to the conventional antifailure drugs, CoQ10 may improve NYHA class and CT ratio and shorten ventricular depolarization in children with chronic idiopathic DCM.

Cardiac troponin T: a marker in the diagnosis of acute myocarditis in children.

This study was conducted to assess the use of serum cardiac troponin T (cTnT) level as a noninvasive indicator to diagnose acute myocarditis in children. Noninvasive conventional methods often fail to diagnose myocarditis, A median cTnT level of 0.088 ng/ml (0.04-3.11) was reported in pediatric patients with acute myocarditis in our previous study. Hence, we attempted to determine the cutfoff level of cTnT to diagnose acute myocarditis in children. Pediatric patients with clinically suspected myocarditis or dilated cardiomyopathy (DCM) and a control group were recruited. History, physical examination, elctrocardiogram, chest roentgenogram, echocardiogram, cTnT level, and/or endomyocardial biopsy and clinical course were studied. The gold standard to diagnose acute myocarditis was endomyocardial biopsy proved according to the Dallas criteria and/or recovery from cardiovascular problems within 6 months of follow-up. Forty-three patients were admitted due to cardiovascular problems from primary myocardial dysfunction. Twenty-four patients were diagnosed as acute myocarditis (group 1), 19 were idiopathic chronic DCM (group 2), and 21 patients had moderate to large ventricular septal defect and congestive heart failure (group 3). Median cTnT level was statistically higher in (group 1) compared to groups 2 and 3. Ejection fraction (EF) and left ventricular end diastolic dimension (LVEDd) z score of acute myocarditis were 38.5% (range, 21-67) and 1.3 (range, -0.8-3.0), respectively, which were significantly better than DCM [28.0% (range, 17-45) and 6.0 (range, 2.0-10.0)]. The cutoff point of cTnT level to diagnose acute myocarditis was 0.052 ng/ml (sensitivity, 71%; specificity, 86%). cTnT level, EF, and LVEDd z score did not predict short-term outcomes of patients. In acute myocarditis, cTnT level and EF were significantly higher and LVEDd z score was significantly lower than in DCM. However, the three parameters had no significant effect on outcomes of the patients. Our data show that cardiac a cTnT level of 0.052 ng/ml is an appropriate cutoff point for the diagnosis of acute myocarditis.

Predictors of successful transcatheter closure of atrial septal defect by cardiac magnetic resonance imaging.

The location, size, and rim of an atrial septal defect (ASD) are major determining factors for transcatheter closure. We compared the measurements of ASD size and the characteristics of atrial septal rim using cardiac magnetic resonance imaging (MRI) with those obtained using transesophageal echocardiography (TEE). Patients with an ASD that met established criteria were selected for evaluation by cardiac MRI and TEE. There were 66 patients who underwent both TEE and cardiac MRI. Bland-Altman comparative analysis was performed to demonstrate agreement between measurement of ASD by MRI and balloon sizing compared to measurement of ASD by TEE and balloon sizing. Twelve patients were excluded from transcatheter closure of ASD. TEE did not demonstrate an adequate measurement of the posterior inferior rim in 10 of 66 patients. Fifty-four patients underwent transcatheter closure using the Amplatzer septal occluder. Of these, 52 patients had successful closure (ASD measurements of 25.9 +/- 5.6 mm using MRI and 21.9 +/- 5.8 mm using TEE). The median device size was 28 mm (range, 11-38). Patients who had successful closure had a significantly smaller major axis of ASD and larger posterior inferior rim compared to those of patients who were excluded from the closure procedure. Cardiac MRI showed a better correlation of ASD diameter measurement to balloon sizing compared to TEE. We believe that the most important predictive factor for successful transcatheter closure of an ASD is an adequate posterior inferior rim, which is best visualized using cardiac MRI.

Immunoglobulin failure and retreatment in Kawasaki disease.

Several cases of Kawasaki disease (KD) were unresponsive to the initial treatment with intravenous immunoglobulin (IVIG). We retrospectively analyzed all children admitted with KD to determine the occurrence and variables associated with the initial IVIG treatment failure. All patients who fulfilled the criteria for KD and were treated with a single dose (2 g/kg) of IVIG between January 1995 and August 2001 were enrolled. An analysis of the patients who had initially failed to respond to IVIG was performed. A total of 120 patients were enrolled during the study period. There were 68 boys (56.7%). Fourteen patients (11.6%) were found to be unresponsive to initial IVIG treatment. Patients who were anemic (Hb<10 G/DL), had a high neutrophil count (> 75%), a high band count, and low albumin were at risk of failure to respond to a single dose of IVIG. We found no correlation among age, gender, days since starting IVIG treatment, and erythrocyte sedimentation rate (ESR) with failure of the initial IVIG treatment. There were 12 patients (10%) who developed coronary artery aneurysms. The failure of a single dose of IVIG treatment occured in up to 11.6% of our Kawasaki patients. We found that low hemoglobin (<10 G/DL), high neutrophil count (> 75%), high band count, and a low albumin were associated with the requirement for retreatment with a second dose of IVIG.

Cardiac troponin T: its role in the diagnosis of clinically suspected acute myocarditis and chronic dilated cardiomyopathy in children.

This study was conducted to assess the use of the serum cardiac troponin T (cTnT) level as a noninvasive indicator to differentiate acute myocarditis and chronic dilated cardiomyopathy in pediatric patients. Myocarditis and dilated cardiomyopathy are clinically difficult to differentiate. Endomyocardial biopsy proved to be quite useful. However, the nature of the procedure--invasiveness, time-consuming, and limited sensitivity--caused some concerns, especially in pediatric patients. Hence, we attempted to find an alternative method that could give a prompt diagnosis of acute myocarditis. Twenty cases with clinically suspected myocarditis or dilated cardiomyopathy and a control group of 21 cases with moderate left-to-right shunt and congestive heart failure were recruited. History, physical examination, electrocardiogram, chest roentgenogram, echocardiogram, cTnT, creatine kinase MB isoenzyme (CK-MB mass), and/or endomyocardial biopsy were compared. The gold standard used to diagnose myocarditis is endomyocardial biopsy (Dallas criteria) and/or recovery from cardiovascular problems within 6 months of follow-up. Ten patients were diagnosed as having myocarditis (group 1) and 10 with chronic dilated cardiomyopathy (group 2). The control group of 21 cases was designated as group 3. The median serum cTnT levels were 0.088 (0.04-3.11), 0.010 (0.010-0.990), and 0.010 (0.010-0.550) ng/ml in groups 1, 2, and 3, respectively. The mean CK-MB mass level for groups, 1, 2, and 3 were 18.35 (7.14-70.00), 4.80 (0.54-108.00), and 2.26 (0.95-7.06) ng/ml. The study showed that both the cTnT and CK-MB mass levels were significantly higher in group 1 than either group 2 or group 3. Histopathology was studied in 9 cases. In 2 of 5 cases and in all 4 cases in group 1 and group 2 histopathology was pathologically proved. Levels of cTnT and CK-MB were significantly higher for myocarditis than for dilated cardiomyopathy and left-to-right shunt with CHF. Further study is needed to assess the optimum cTnT level for differentiating both conditions.

Comparison of atrial septal defect closure using amplatzer septal occluder with surgery.

Our study reports the results of a comparison of closure of atrial septal defect (ASD) surgically with transcatheter closure using the Amplatzer septal occluder. Patients having an ASD and a surgical closure or transcatheter closure between January 1999 and July 2000 were selected. There were 103 patients who had ASD. All 64 patients in group 1 (surgery) had a successful operation, with only 2 patients with a mild residual shunt. There were 39 patients enrolled for transcatheter closure of the ASD (group 2). Four patients were excluded initially. The median age for group 1 was 25 years (range 2.3-64 years) compared to 11.7 years (range 2-69 years) in group 2 (p = 0.035). In group 1, the mean ASD diameter measured was 28.4 +/- 10.2 mm compared to 23.4 +/- 5.7 mm in group 2 (p = 0.003). In 29 patients, devices were deployed with sizes from 10 mm to 30 mm (median 24 mm). Three patients were excluded because a larger device (>30 mm) was not available and devices were not successfully deployed in another 3 patients. One patient had a device embolized into the right ventricle (surgical removal and closure of the ASD). Complications occurred in 13 patients in group 1 and 4 patients in group 2. Complete occlusion occurred in 27 of 28 group 2 patients (96.4%) during the follow-up period (10.2 +/- 5.4 months). The Amplatzer septal occluder is a new device for closure of different-sized ASDs. The intermediate-term follow-up demonstrated excellent closure results. The benefit for each patient was demonstrated in less morbidity and less time spent in the hospital.

Outcomes of transcatheter balloon angioplasty of obstruction in the neo-aortic arch after the Norwood operation.

Obstruction of the reconstructed aortic arch, or the neoaortic arch, is now known to be an important factor increasing mortality after the Norwood operation for hypoplastic left heart syndrome. Transcatheter balloon angioplasty has been shown to provide effective relief of both native aortic coarctation and obstructions of the aortic arch occurring subsequent to therapeutic intervention. We sought to determine the outcomes of balloon angioplasty used as an initial treatment for obstruction of the neoaortic arch occurring after the Norwood operation. We gathered the characteristics of 58 patients with such obstruction from 8 institutions, noting procedural factors and outcomes of initial balloon dilation. Obstruction occurred at a median interval of 4 months, with a range from 1.5 months to 6.3 years, after a Norwood operation. Ventricular dysfunction was present before dilation in 13 patients. Mean peak to peak systolic pressure gradients were acutely reduced from 31+/-20 mm Hg to 6+/-9 mmHg (p<0.001), with outcome subjectively judged to be successful in 89%. Three patients with pre-existing ventricular dysfunction died within 48 hours of dilation. There were 10 additional deaths during the period of follow-up, with Kaplan Meier estimates of survival after intervention of 87% at 1 month, 77% at 12 months, and 72% after 15 months. In addition, 9 patients required re-intervention during the period of follow-up, with Kaplan Meier estimates of freedom from re-intervention after dilation of 87% at 6 months, 78% at 12 months and 74% after 18 months. Although transcatheter dilation of neoaortic arch obstructions after Norwood operation is successful, there is a high risk of re-intervention and ongoing mortality in this subgroup of patients. Close follow-up is recommended.

Intermediate term follow-up on transcatheter closure of atrial septal defects by Amplatzer septal occluder.

BACKGROUND: Surgical repair of secundum atrial septal defect (ASD) is a safe, widely accepted procedure with negligible mortality. However, it is associated with morbidity, discomfort and a thoracotomy scar. As an alternative to surgery, a variety of devices for transcatheter closure of ASD have been developed. OBJECTIVES: We report our clinical experience with transcatheter closure of ASD using the Amplatzer Septal Occluder, a new occlusion device with intermediate term follow-up. PATIENTS & METHOD: Patients having ASD met established two-dimensional echocardiographic criteria for transcatheter closure were selected. ASD size was measured by transesophageal echocardiogram (TEE) and balloon occlusion catheter (stretched diameter). The Amplatzer's size was chosen to be equal to or 1 mm less than the stretched diameter. The device was advanced transvenously into a guiding sheath and deployed under fluoroscopic and TEE guidance. Once its position was optimal, it was released. TEE was undertaken to demonstrate the residual shunt. RESULTS: There were 26 patients with a mean age of 17.2 +/- 15.9 years old (2 to 60) and a mean weight of 22 +/- 37.5 kg. (10.7 to 62.5). The mean ASD diameter measured by TEE was 18.3 +/- 5.2 mm. and by stretched diameter was 22 +/- 7.5 mm. Four patients who had ASD stretched diameter over 32 mm were excluded because a larger device was not available. Devices were deployed in 22 patients with sizes from 9 to 30 mm (median = 22mm). Immediately after closure a tiny residual shunt was observed at the core of the device in each case. At 24 hours only two patients had a small (< 2 mm) shunt. One patient with fenestrated ASD had a device embolized into the right ventricle with successful removal and surgical closure. Patients were followed-up for a mean duration of 8 +/- 3.5 months (from 3 to 12 months). Complete occlusion was found in 20 out of 21 patients (95%). CONCLUSION: The Amplatzer Septal Occluder is a new device designed for closure of different sizes of ASD and can be easily and safely deployed. Our experience showed that this device could be used to close an ASD as large as 30 mm. The intermediate term follow-up also demonstrated an excellent closure result. Caution should be undertaken with patients who have a fenestrated atrial septal defect particularly at the septal rim.

Radiofrequency catheter ablation of Wolff-Parkinson-White syndrome: report of 83 cases from Siriraj Hospital.

We described the characteristics of patients and accessory pathway and showed our results of Radiofrequency catheter ablation (RFCA). There were 41 males and 42 females at a mean age of 36 years. Accessory pathway associated with Wolff-Parkinson-White (WPW) syndrome in our population was more prevalent on the right side which is different from previous reports. Most commonly associated heart disease was Ebstein's anomaly. Overall success rate was 96.4 per cent. Right free wall accessory pathway needed a longer procedure time and fluoroscopy time, higher radiofrequency power and more radiofrequency applications compared to other locations. Although the recurrence rate was 12 per cent, all patients with recurrence were successfully reablated. We also described the comparison of our result with previous studies. To our knowledge this is the first report in Thailand with a reasonable number of cases. RFCA is a very effective treatment of WPW syndrome in the Thai population and should be considered in symptomatic patients especially those who are refractory to medication.

Limitation of transthoracic echocardiography in the diagnosis of congenital heart diseases.

Nowadays, it has been widely accepted that echocardiography is the most efficient, non invasive diagnostic tool to diagnose congenital heart diseases. However, cardiac catheterization remains the gold standard to diagnose and obtain hemodynamic data prior to cardiovascular surgery. In order to find out the accuracy of transthoracic echocardiography in relation to the anatomical diagnosis of congenital heart diseases, 175 consecutive patients who underwent diagnostic cardiac catheterization during January 1999 to December 1999 were reviewed. All of them had complete echocardiographic studies prior to the procedure. The male to female ratio was 1.06:1. The median age at the time of echocardiography was 3.36 (0.01-28.8) years old. The indications of the cardiac catheterization were to demonstrate cardiovascular anatomy 64 per cent, to obtain pulmonary artery pressure and pulmonary vascular resistance 13.7 per cent, and to get both information 22.3 per cent. Tetralogy of Fallot (23.4%) was the most frequent cardiac malformation, followed by complex congenital heart diseases (22.3%), simple left to right shunt (12%), pulmonary atresia with ventricular septal defect (8.6%), tricuspid atresia (5.7%), simple d-transposition of great arteries (4%), etc. From cardiac catheterization; 49 cases (28%) revealed additional data which were surgically important, 3 cases (1.7%) resulted in different diagnoses, and 3 cases (1.7%) revealed additional information which was not surgically important. Inadequate imaging technique (36 cases, 65.5%) and limitation of technique (19 cases, 34.5%) were the reasons for missing anatomical findings of transthoracic echocardiography. Age at the time of echocardiography was not a significant factor affecting the accuracy. Persistent left superior vena cava, multiple aorto-pulmonary collateral arteries, pulmonary artery anatomy, and coronary artery anatomy were the most frequent cardiac lesions misdiagnosed by transthoracic echocardiography that were somewhat surgically important. The incorrect echocardiographic diagnoses were aorto-pulmonary window, patent ductus arteriosus, and vascular ring. Thorough and extensive echocardiographic scanning coupled with cooperative or adequately sedated patients by an experienced operator using an efficient echocardiographic machine might improve the accuracy of transthoracic echocardiography in the diagnosis of congenital heart diseases.

Radiofrequency catheter ablation: 5 years experience at Siriraj Hospital.

Between February 1995 to May 2000, 626 consecutive patients underwent radiofrequency catheter ablation for various types of cardiac arrhythmia. The mean age was 41 years, range 1-85 years. Mapping and ablation were guided by intracardiac electrogram and anatomical approaches. The initial success, compliction, recurrent and final success rates are shown below:- [table; see text] Conclusion, RFCA is an effective treatment and should be considered as first line treatment for certain tachyarrhythmia.

Utility of intraoperative transesophageal echocardiogram in congenital heart disease.

Intraoperative transesophageal echocardiogram (TEE) has improved the perioperative outcome in adult patients. The purpose of the current study was to assess the benefit of intraoperative TEE in patients with congenital heart disease undergoing surgical correction (repair). The results of the patients who had consecutively undergone intraoperative TEE during their congenital heart surgery between January 1998 to June 2000 were reviewed. There were 104 patients (whose ages ranged from one week old to 50 years old (median 5 years old) and their weights from 3 kg to 79 kg (median 15 kg). A significant impact was said to have occurred if these findings prompted a change in surgical procedure following a prebypass study or rebypass for repair of a residual defect. Prebypass TEE had a significant impact in seven patients (6.7%). TEE could be used as a guide to help repair in three patients. The postbypass TEE examination had a significant impact in 15 patients (14.4%). Of these 15 patients, eight were detected primarily by TEE examination. The group of patients in whom TEE had the most significant impact was in patients who had surgery related to the repair of the atrioventricular valve (complete or partial atrioventricular (AV) canal repair, Ebstein's anomaly; 9 out of 22 patients; 41%). A less significant impact was found in surgery for other complex congenital defects (single ventricle or complete transposition of great arteries; eight out of 46 patients; 17.4%). TEE had limited impact in simple congenital lesion. Intraoperative TEE is valuable in the perioperative care of patients with congenital heart defects. We found the most benefit in patients with complete or partial AV canal and Ebstein's anomaly.

Coarctation of the aorta in children at Siriraj Hospital.

A retrospective analysis of 33 children who were diagnosed to have coarctation of aorta at Siriraj Hospital between January 1989 and December 1998 was undertaken. There were 21 males (64%) and 12 females (36%). Their ages ranged from one day to 11 years (median 2 months). The majority of the patients (78.8%) were presented early within the first year of life. The predominant clinical manifestations were congestive heart failure (69.6%), systemic hypertension of the upper extremities (36.3%) and decreased femoral pulses. Chest roentgenogram revealed cardiomegaly (70%) and increased pulmonary blood flow (84%), reflecting congestive heart failure and associated left-to-right shunting. Electrocardiogram showed normal pattern (33.3%), right ventricular hypertrophy (33.3%), left ventricular hypertrophy (22.2%) and biventricular hypertrophy (11.2%). The younger the patient is, the more right ventricular predominance is demonstrated. According to the echocardiogram and/or aortogram, juxtaductal type was found in 51.5 per cent, postductal type in 27.3 per cent and preductal type in 21.2 per cent. Medical management included prostaglandin E1 infusion in a newborn baby presented with low-cardiac output state, anticongestion and antihypertension, if indicated, followed by surgical correction. The result of coarctectomy with end-to-end anastomosis with or without arch augmentation was good. The operative mortality rate was 5 per cent. The overall mortality in the present study was 9 per cent. The most common causes of death were multiorgan failure and pulmonary infection. Residual coarctation was found in 5 per cent.

The Fontan operation: experience at Siriraj Hospital.

Seventy five patients underwent modified Fontan operation at Siriraj Hospital from October 1987 to December 1998. Cardiology data was analyzed retrospectively. Four patients' data was unavailable. Median age at operation was 9.7 (1.8-34) years old. Tricuspid atresia accounted for 38 per cent of the patients. Ten patients (14.1%) died in the acute post operative period due to consequence of low cardiac output. Another 3 patients (4.2%) expired in the intermediate and late post operative period. Age at operation, pulmonary artery size, pre-operative oxygen saturation, and mean pre-operative pulmonary artery pressure were not different between those who survived and those who died. Abnormal pulmonary vein, atrioventricular valve regurgitation, and underlying ventricular morphology statistically affected the acute survival of modified Fontan operation. Intraoperative aortic cross clamp time, and post operative mean pulmonary artery pressure on day 0, 1 and 2 post operation were found statistically shorter and lower in the survival group. Survival rate at 5 years was 83 per cent. Modified Fontan operation is the final palliative operation of choice for low risk single ventricle physiology in our institution with acceptable outcome. Thorough pre-operative hemodynamic and anatomic studies and staging modified Fontan procedure may include a higher number of candidates and improve the outcome of the operation.

Radiofrequency catheter ablation in pediatrics: experience at Siriraj Hospital.

UNLABELLED: Tachyarrhythmia is one of the life threatening cardiac electrophysiology problems in children. It also affects quality of life of the patients. Radiofrequency catheter ablation (RFCA) has made a significant impact in the treatment of tachyarrhythmia since 1989. The present report is the first and largest report in Thai children. There have been 24 RFCA procedures in 21 children since it was initially performed at Siriraj Hospital from January 1996 to December 1999. The electrophysiology studies and medical records were analyzed retrospectively. Median age and weight at the time of the procedure were 11 (1.1-13) years old and 38.8 (6.8-78.2) kg respectively. The presenting symptoms were palpitation 66.7 per cent, presyncope 16.7 per cent, congestive heart failure and cardiogenic shock 8.3 per cent, syncope 4.2 per cent, and chest pain 4.2 per cent. Median duration of symptom was 3.5 (0.1-8.0) years. The underlying cardiac arrhythmias were Wolff Parkinson White (WPW) syndrome 50 per cent, concealed accessory pathway 16.7 per cent, atrioventricular node re-entry tachycardia (AVNRT) 16.7 per cent, atrial ectopic tachycardia (AET) 12.5 per cent, and WPW with AVNRT 4.2 per cent. The median fluoroscopy time and procedure time were 25 (4-145) minutes and 125 (60-320) minutes respectively. The median tachycardia cycle length was 332.5 (220-460) seconds. The immediate success rate was 21/24 (87.5%) procedures. The procedural complication was 1/24 (4.2%). Two patients (8.3%) had recurrences of tachycardia and were successfully controlled with antiarrhythmic drugs. CONCLUSION: RFCA is a safe, effective, and curative procedure with high success rate for pediatric tachyarrhythmias.

Results of rapid two-stage arterial switch operation in patients with transposition of the great arteries: one-year postoperation.

The rapid two-stage arterial switch operation is an alternative therapy for patients with simple transposition of the great arteries who present beyond the neonatal period and have low left ventricular pressure. It provides normal ventricular function compared to the atrial switch operation. Between July 1994 and February 1997, there were 13 such infants who had rapid two-stage arterial switch operation performed at Siriraj Hospital. There was 1 late death (11 months after the operation). All 12 survivors (mean age 22.4 +/- 5.7 months) were clinically evaluated and had echocardiography performed at 14.8 +/- 4.9 months after the operation. All were asymptomatic. Echocardiogram revealed a residual small atrial septal defect (1 case), small ventricular septal defect (1 case), mild supravalvar neopulmonary stenosis (2 cases), bicuspid neoaortic valve without stenosis (2 cases), dilated neoaortic sinus of Valsalva (6 cases, 50%) and mild neoaortic insufficiency (11 cases, 91.7%). The left ventricular function was hyperdynamic after pulmonary artery banding and significantly decreased to normal level at the time of study (shortening fraction of 43.8 +/- 10.7 vs 29.2 +/- 3.8%, respectively, p = 0.0005). The wall thickness was significantly increased after pulmonary artery banding and decreased overtime (0.48 +/- 0.08 vs 0.32 +/- 0.05 cm, respectively, p < 0.0005). The left ventricular dimension was significantly increased both after pulmonary artery banding and at the time of study (2.06 +/- 0.42 vs 3.32 +/- 0.30 cm, respectively, p < 0.0005). The left ventricular mass was significantly increased after pulmonary artery banding and at the time of study (21.79 +/- 7.79 vs 33.08 +/- 7.40 g/m2, respectively, p = 0.0005). The mortality and morbidity of rapid two-stage arterial switch operation are low. However, long-term follow-up should be monitored.

Mortality in potential arterial switch candidates with transposition of the great arteries.

OBJECTIVES: We reviewed the factors contributing to or causing death before surgery in neonates with d-transposition of the great arteries (TGA) despite anatomy suitable for the arterial switch operation (ASO) to develop strategies to minimize preoperative attrition. BACKGROUND: Currently the ASO for neonates with TGA carries a low operative mortality. However, there is a paucity of information regarding the patients who die before the ASO. Strategies to ensure survival to operation are of importance to improve overall outcome. METHODS: We reviewed all neonates with TGA and patent forearm ovale (PFO) < or = 2 mm, a birthweight <2 kg, or who died before surgery, between 1988 and 1996. RESULTS: We identified 12 out of 295 neonates with TGA (4.1%) with anatomy suitable for the ASO who died prior to surgery. All had TGA/intact ventricular septum (IVS) and presented with a severely restrictive PFO. In 11 of 12 cases the cause of death was attributed to the sequelae of profound hypoxemia from inadequate mixing. Contributing factors were prematurity, 41.7%; severe respiratory distress syndrome, 25%; and persistent pulmonary hypertension of the newborn (PPHN), 16.7%. All patients received prostaglandin E1 (PGE1) infusion. Urgent balloon atrial sepstostomy (BAS) was performed in 66.7% with improved oxygenation. No cases were diagnosed prenatally. In contrast, all patients with a PFO < or = 2 mm who survived to ASO had a significantly better response to PGE1 infusion (p=0.03) than nonsurvivors. The ASO was accomplished without mortality in four of nine with a weight <2 kg. CONCLUSIONS: Of those neonates admitted with TGA, 4.1% died before surgery. Eleven of 12 (3.7%) died due to consequences of inadequate interatrial mixing despite PGE1 infusion. Earlier diagnosis and BAS are critically important in determining survival. Early ASO may improve survival in patients weighing <2 kg. Prenatal diagnosis with delivery in a high-risk obstetrical unit with facilities for immediate BAS and supportive therapy for pulmonary hypertension and ventricular failure may be necessary to salvage this group of patients.

Glucose-6-phosphate dehydrogenase deficiency in the newborn: its prevalence and relation to neonatal jaundice.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is prevalent in Thailand. This condition can cause acute hemolysis during oxidative stress and also severe hyperbilirubinemia in the newborn in some populations. Our aim was to study the prevalence of G6PD deficiency in relation to neonatal jaundice. We performed quantitative red blood cell (RBC) G6PD assay in the cord blood of 505 male subjects. Observation of jaundice and determination of bilirubin level as well as work up for other causes of jaundice were made in the G6PD deficiency group compared to a G6PD normal group. Questionnaires were also sent for further follow up to both groups. The results of the study were as follows: Sixty-one of 505 male (12.08%) had RBC G6PD deficiency (Group I). The rest (444 cases) had normal G6PD (Group II). In Group I, 49.15% developed neonatal jaundice, of which 28.82% were physiologic and 20.33% were pathologic jaundice. In group II, 23.68% developed jaundice; 16.51% were physiologic and 7.17% were pathologic jaundice, respectively. Onset of jaundice, date of peak bilirubin and peak bilirubin level in Group I and Group II were not statistically different. ABO incompatibility was associated with Group I in 17.24% and with Group II in 9.09%. Hospitalization day in Groups I and II were not statistically different. Other associated diseases were found in both groups, ie infection, congenital malformation, respiratory distress syndrome, but there was no significant difference in terms of jaundice. Phototherapy was required in 18.64% and 10.28% in Group I and II with a duration of 3.91 +/- 1.24 and 3.21 +/- 1.75 days, respectively. One case in Group I who was also premature received one exchange blood transfusion due to severe sepsis but he did not survive. One case in Group II who had polycythemia was successfully treated by partial exchange transfusion with plasma.