Clinical manifestations of kawasaki disease: What are the significant parameters.

The initial differential diagnosis of Kawasaki disease (KD) from other acute febrile illnesses infants and children is particularly difficult in patients who exhibit incomplete criteria. The objective of this study was to de-termine the differences in the clinical and laboratory findings between KD patients and those who were initially sus-pected of having KD but eventually had other diagnoses. One hundred and fourteen pediatric patients who were initially diagnosed with suspected KD were included. Eighteen cases were finally diagnosed with another disease. The only demographic data that were significantly different between the groups were body height and the duration of fever. The KD group exhibited more classical clinical criteria than those who were finally diagnosed with another disease. The erythrocyte sedimentation rate (ESR) and platelet count were significantly higher in the KD group than in the non-KD group. An ESR ≥ 40 mm/hour had a diagnostic sensitivity of 90.5%, a specificity of 66.6%, a positive predictive value of 93.4%, and a negative predictive value of 57.1%. The incidence of coronary aneurysm in this study was 6.2%. There was no correlation between ESR and coronary aneurysm. We conclude that the clinical criteria are the basis for the diagnosis of Kawasaki disease but the ESR can be helpful in pediatric patients with acute febrile illness who do not exhibit all clinical criteria.

Arrhythmia in early post cardiac surgery in pediatrics: Siriraj experience.

OBJECTIVES: To determine the incidence, risk factors and outcome of early postoperative arrhythmias in pediatric patients with congenital heart disease. MATERIAL AND METHOD: A prospective study was conducted in every pediatric patient who consecutively underwent open-heart surgery at Siriraj Hospital from January 1st to December 31st, 2006. The collected data were demographic data, diagnosis, pre-operative arrhythmia, cardiac surgical data and continuous electrocardiographic monitoring data throughout the post operative intensive care period. RESULTS: A total of 191 pediatric patients underwent cardiac surgery. Forty-five cases (23.5%) developed early post operative cardiac arrhythmias i.e. junctional ectopic tachycardia 18 cases (40%), heart block 7 cases (15.6%), supraventricular tachycardia 2 cases (4.4%). Cardiac arrhythmia occurred mostly within 24 hours after the operation. Patients with single ventricle physiology repaired developed the highest incidence of acute post operative arrhythmia (36.4%). Longer, cardiopulmonary bypass time- and redo-operation were the risk factors. Thirty-nine cases were treated with medications, 7 cases with temporary pacing, and 1 case with electrical cardioversion. Four patients needed long-term anti-arrhythmic agents. Cardiac arrhythmia played role in the causes of death in 2 cases (1.1% of total cases). CONCLUSIONS: Post operative arrhythmias remained common and important complications of pediatric open-heart surgery. Long cardiopulmonary bypass time and redo-operation were risk factors for early post operative arrhythmia.

Pre-operative evaluation with magnetic resonance imaging in tetralogy of fallot and pulmonary atresia with ventricular septal defect.

BACKGROUND: Preoperative evaluation of patients with pulmonary atresia and ventricular septal defect (PA/ VSD) are generally done by echocardiogram and cardiac catheterization. The authors' objective of the present study was to compare the findings of Gadolinium (Gd) enhanced cardiac magnetic resonance angiography (MRA) with cardiac catheterization. MATERIAL AND METHOD: Patients who had PA/VSD were prospectively evaluated using cardiac catheterization and cardiac MRA. A branch of the pulmonary arteries was divided into: main pulmonary artery (MPA), left and right branch pulmonary artery (LPA & RPA), major aortopulmonary collateral arteries (MAPCA) and minor collaterals. Each study was interpreted blindly. The agreement of findings was compared using Kappa statistics. RESULTS: There were 43 patients who received both cardiac catheterization and cardiac MRI within a 2 month period The average age was 13.8 +/- 8.4 (2-30) years-old. There was an agreement among measurement of both MPA and LPA & RPA with Kappa statistics of more than 0.8. Gd-enhanced MRA was able to identify more branches of MAPCA when compared to cardiac catheterization. CONCLUSIONS: The results of the present study indicate that Gd-enhanced MRA is a feasible, fast and accurate technique for identification of all sources of pulmonary blood supply in patients with complex pulmonary atresia. The present study was a noninvasive alternative to cardiac catheterization. Gd-enhanced MRA can better delineate small (minor) branches of collateral.

Truncus arteriosus: Siriraj experience.

Between January 1st, 1995 and December 31st, 2004, a total of 30 children (17 girls, 56.7% and 13 boys, 43.3%) were diagnosed to have truncus arteriosus at the Department of Pediatrics, Siriraj Hospital. The ages at the first diagnosis ranged from 1 day to 3 years (median 120 days). The predominant clinical presentations were congestive heart failure (53.3%), cyanosis (30%) and feeding problems (26.7%). All patients had heart murmur. Chest roentgenogram demonstrated cardiomegaly and increased pulmonary vascularity in 86.7% and 83.3%, respectively. Electrocardiogram showed a frontal plane QRS axis in a range of 0-90o in 70% of the patients, left ventricular hypertrophy and biventricular hypertrophy in 50% and 40%, respectively. Echocardiogram revealed type I anomaly in the majority of the patients (80%), and type II in the rest of patients. Ten percent of the patients had right-sided aortic arch. The majority of the patients received more than one medication for controlling heart failure. Sixteen patients (53.3%) were operated at the median age of 133 days and median weight of 3.8 kilograms. Thirteen percent of the patients (2 cases) had palliative surgery (pulmonary artery banding) and 87.5% of patients (14 cases) underwent total correction. All patients who had total repair had immediate complications, of which the majority (57.1%) were pulmonary hypertensive crisis. There were a total of 9 deaths (30%); 7 patients died immediately (<14 days) post total repair, 2 patients died preoperatively. During follow-up (median 57.9 months), all patients were asymptomatic except one patient (90.9%) who was re-operated on for conduit replacement due to severe stenosis and truncal valve repair due to severe regurgitation at 22 months after the first operation. Three patients have been waiting for surgery. In the present study, we could not definitely relate the associated risk factor with mortality such as the age at surgery, preoperatively high pulmonary vascular resistance or truncal valve abnormality. However, it seems that the older age at surgery may be the possible risk factor. Therefore, early diagnosis and surgical intervention for this anomaly should be considered.

Efficacy of fetal echocardiography and an evaluation of indications.

Fetal echocardiography has been accepted as a prenatal noninvasive diagnostic tool of cardiovascular diseases in fetuses for more than three decades. There are limited data in Thailand. A retrospective study of prenatal, natal and postnatal data of pregnant women who had fetal echocardiography at Siriraj Hospital, Bangkok, Thailand, from January 1999 to July 2005 was conducted. In total, there were 117 pregnant women who had fetal echocardiography under standard indications. Median age of pregnant women was 30.2 (17.3-44.2) years old. Median gestational age at the first fetal echocardiography was 29 (17-40) weeks. Median number of time the women had fetal echocardiography was 1 (1-10). Indications for fetal echocardiography were; obstetricians suspected of fetal cardiovascular diseases, multiple anomalies, family history of congenital heart diseases, chromosome anomalies, hydrops fetalis, and other indications (53.8%, 12%, 11.1%, 8.5%, 8.5% and 6.0% respectively). There were 49 (41.8%) cases who had abnormal cardiovascular problems, i.e., structural heart diseases 26 cases, rhythm disturbance 15 cases, and cardiac masses 8 cases. Upon comparison with postnatal echocardiography and/or autopsy findings, fetal echocardiography had a sensitivity of 96.9%, specificity of 90.6%, and accuracy of 92.8%. There was various degrees of risk to have cardiovascular problems for each of the indication taking fetal echocardiography and postnatal data into consideration. Fetal echocardiography had an impact on the management in 57.3% of cases. Conclusion: Fetal echocardiography is a reliable prenatal diagnostic tool for cardiovascular problems with high accuracy and has an impact on the management at prenatal, natal and postnatal period. Different indications for fetal echocardiography have an effect on the relative risk of fetal cardiovascular diseases.

Coronary artery bypass graft in Kawasaki disease patients: Siriraj experience.

Kawasaki disease (KD) is an acute systemic vasculitis. It is one of the most common acquired heart diseases in children. Its important acute complication is coronary artery aneurysm. Although most of these aneurysms resolved overtime but some never did. Coronary artery stenosis and myocardial insufficiency or infarction are late complications. Coronary artery bypass graft (CABG) and catheter intervention are the treatment for these patients. We report our first five cases of Kawasaki patients with myocardial insufficiency who underwent CABG at Siriraj Hospital. Patients' ages ranged from 3.3 to 14.4 years old. Only two patients (40%) had angina. Internal thoracic artery was used as a bypass graft in most patients and postoperative course was uneventful. Coronary bypass operation is a safe and reliable surgical modality for coronary artery sequelae in children with Kawasaki disease. A long-term follow-up is necessary to study the long-term outcome of bypass.

Permanent cardiac pacing in pediatrics: experience in Thailand.

Permanent cardiac pacing in pediatrics is uncommon. There has been limited data in Thailand. A retrospective study of cardiac data and pacing parameters during follow-up periods in patients who underwent permanent pacemaker implantation at the Department of Pediatrics, Siriraj Hospital, from January 1997 to December 2004 was conducted. There were 31 patients in total who have been followed-up for the median period of 34.4 (1.07-91.13) months. All patients had atrio-ventricular block prior to implantation. The etiology were; post cardiac surgery 38.7%, maternal autoimmune diseases 19.4%, post radiofrequency ablation 3.2%, and unknown 38.7%. Twenty three cases (74.2%) were implanted by epicardial approach, and 18 (25.8% were implanted by endocardial approach. Modes of permanent pacemaker were WIR 45.2%, VVI 35.5%, and DDD 19.4%. Age and body sized of the patients using epicardial approach were significantly lower than endocardial approach. Minor complications occurred in 3 cases (9.6%) i.e. 2 with surgical wound infection, 1 with post pericardiotomy syndrome. Minimum energy threshold, sensitivity, and impedance at implantation and during follow up periods were not different statistically. There was significantly less in minimum energy threshold of endocardial lead than epicardial lead. Epicardial lead failure was found in 3 cases (11.5%) at the median time of 8.9 (7.9-62) months post implantation, but was not significant different from endocardial leads. Survival of epicardial leads were 82% at 8 years. Conclusion: Permanent pacemaker implantation in pediatrics was rare (4.4 cases/year). It was feasible in almost all body size and a rather safe procedure. There was no significant change in pacing parameters at the medium-term follow-up period for both epicardial and endocardial leads. Minimum energy threshold of epicardial lead was significantly higher than endocardial lead.

Combination therapy of prostacyclin for pulmonary hypertension in congenital heart disease.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a recognized complication of congenital heart disease. Despite differences in etiology and pathophysiology, successful therapy for idiopathic PAH may benefit in patients with congenital heart disease. We theorized that combination of oral and aerosolization prostacyclin will benefit this group of patients in long-term. MATERIAL AND METHOD: The study design was single group and open label study with intention to treat for patients with congenital heart disease with pulmonary artery (PA pressure) more than 50% of systemic pressure. All patients were given a combination of orally given beraprost sodium and inhalation of iloprost for 12 months. Data were collected prospectively consisting of functional class, O2 saturation, 6-minute walk test and right ventricular systolic pressure (RVSP). RESULTS: There were 23 patients with an average right ventricular systolic pressure (+/- SD) of 94.8 +/- 14.5 mmHg and with average age of 27.8 +/- 14.9 years (2.5 to 50 years). The average oxygen saturation was 87.9 +/- 7 %. There were 12 patients with post surgical repair or cardiac catheterization interventional procedure and 11 with and Eisenmenger's syndrome. There were significant improvement of 6-minute-walk test from an average of 268 +/- 70 meters to 308 +/- 57 meters at the end of 12 months. The functional class of patients was also improving. However, there were no significant different in oxygen saturation. CONCLUSION: Combination therapy of oral and inhalation of aerosolized vasodilators is a fascinating concept in the therapy of pulmonary hypertension. Treated patients showed an improvement in exercise capacity and right ventricular systolic pressure without a worsening in oxygen saturation.

Heart murmur in the first week of life: Siriraj Hospital.

Congenital heart disease (CHD) is the most common form of cardiovascular diseases in children. In Thailand, there has not been available information about congenital heart disease in neonates. Between January 1st and December 31st, 2000, all full-term babies born at Siriraj Hospital with detected heart murmur within the first week of life were consulted to pediatric cardiologists. Echocardiography was performed for diagnosis in every baby. Total livebirths during that period were 11,245 cases. Heart murmurs were detected in 83 cases. The incidence of heart murmur within the first week of life was 7.38:1,000 livebirths. Innocent murmurs were found in 34 cases and echocardiogram revealed no detectable cardiac anomalies (2 cases), mild tricuspid regurgitation (2 cases), physiologic branch pulmonary stenosis (4 cases), and small size PDA (< 2 mm., 26 cases). Forty-nine cases had CHDs. The incidence of CHD was 4.36:1,000 livebirths. At the time of initial diagnosis, 22 cases (44.8%) were asymptomatic. Among these patients, 1 case had serious cardiac anomaly, i.e., tetralogy of Fallot. There were 27 cases with symptoms, including 15 cases (30.6%) with tachypnea, 8 cases (16.4%) with cyanosis and 4 cases (8.2%) with congestive heart failure. The 3 most common cardiac diseases were ventricular septal defect (9 cases, 18.4%), patent ductus arteriosus greater than 2 mm. (8 cases, 16.3%), and atrial septal defect (8 cases, 16.3%). Those with CHDs were treated with anticongestive medications (22 cases, 44.8%), prostaglandin E1 (5 cases, 10.2%), laser pulmonary vulvulotomy (1 case, 2%), palliative surgery within the first week of life (4 cases, 8.2%) and corrective surgery (4 cases, 8.2%). During follow-up for the period of 1 year, 2 cases died from sepsis. Early diagnosis and proper management are important to reduce morbidity and mortality in the newborn with CHD.

Transcatheter closure of perimembranous ventricular septal defect with immediate follow-up.

BACKGROUND: Surgical closure of membranous ventricular septal defect (VSD) is performed by open heart surgery with a small but significant morbidity and mortality. The authors reported here the first group of patients who underwent transcatheter closure of membranous VSD. METHOD: Patients who had membranous VSD with significant left to right shunt as shown by echocardiogram were selected for closure. A standard right and left heart catheterization was done under general anesthesia. A complete arteriovenous wire loop from the aorta to the left ventricle and VSD out into right ventricle was formed in order to guide the delivery sheath into the VSD from the right ventricle. The authors used the new Amplatzer Membranous VSD Occluder (AGA Medical Corp., USA) to deploy in the VSD position. RESULTS: There were 4 patients in the present study with age range of 2 to 24 years old (median: 4 years old). Their weight ranged from 10 to 45 kg (median: 12 kg). Qp:Qs ranged from 1.7-2.5 to 1. The device diameter selected was from 6 to 10 mm. All of them were placed without any residual shunt. At one month follow-up all the patients had echocardiographic examination which showed no evidence of residual shunt. CONCLUSIONS: The authors demonstrated that transcatheter closure of membranous VSD could be safely and effectively performed in small children. This device also provided an opportunity for closure of VSD in patients with pulmonary hypertension.

Balloon-expandable stenting, percutaneous coil embolization, and amplatzer septal occluder; treatments after complicated lateral tunnel Fontan: a case report.

The authors report a 7-year-old girl with univentricular heart physiology who developed prolonged pleural effusion due to discrete narrowing of the proximal right pulmonary artery, and progressive cyanosis which resulted from leakage of the atrial baffle, multiple veno-venous collaterals after the lateral tunnel Fontan operation. Percutaneous balloon-expandable stent implantation was used to correct the right pulmonary artery stenosis with an excellent result. Cyanosis was improved by coil embolization of the collaterals and occlusion of the baffle leakage with Amplatzer septal occluder. This is the first successful report in Thailand.

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.

Cardiac magnetic resonance imaging of atrial septal defect for transcatheter closure.

BACKGROUND: The location, size of the defect and age of the patient are the major determining factors for transcatheter closure of an atrial septal defect (ASD). The precise shape and anatomy surrounding the defect cannot always be understood by the traditional transesophageal (TEE) echocardiographic technique. OBJECTIVES: The authors compared the measurement of ASD size and atrial septal rim using cardiac Magnetic Resonance Imaging (MRI) and TEE to the balloon sizing technique and device size. PATIENTS AND METHOD: Patients having an ASD which met established criteria were selected for evaluation with cardiac MRI and TEE for a closure procedure. Comparison of the ASD imaging and sizing between the different methods was made. RESULTS: There were 22 patients who had complete transcatheter closure. The mean age and standard deviation of the patients was 33.2+/-15.1 (8-67) years old. The mean weight of the patients was 51.6+/-13.1 (20-99) kg. The average cardiac MRI measurement of the ASD was 24.9+/-6.4 mm compared to the TEE measurement of 20.8+/-5.5 mm. The transcatheter balloon measurement of the ASD was 25.2+/-6.9 (11-36) mm and the device closure size was 24.8+/-6.6 (11-36) mm. The correlation coefficient of cardiac MRI to device closure size was r = 0.784 (p < 0.001) when compared to TEE measurement to device closure size; r = 0.761 (p = 0.001). CONCLUSION: The authors demonstrated the capability of the cardiac MRI in assessment of the ASD morphology and anatomy for transcatheter closure of the ASD with an Amplatzer Septal Occluder. Cardiac MRI can provide information about the type, location, size of the defect and direct visualization of the atrial septum anatomy. This detailed information enabled us to provide a safer, more effective application of the ASD occluder.

Pediatric cardiology at Siriraj Hospital: past, present and future.

The incidence of congenital heart disease (CHD) at Siriraj Hospital for the year 2000 was 4.36 patients per 1,000 livebirths. Types of congenital heart diseases seen by the authors were VSD (18.3%), PDA (16.3%), ASD (16.3%), combined simple left to right shunt lesion (24.7%), tetralogy of Fallot (TF; 6%), D-TGA 2 per cent, other complex congenital heart 8 per cent. Overall 3 out of 1,000 livebirths will have congenital heart disease that will require immediate intervention including cardiac catheterization and surgical intervention. At the same period of time an average of 750 new cases of children were referred annually for evaluation and treatment of cardiac related problems. Reports of acquired heart disease such as acute rheumatic fever, myocarditis, Kawasaki's disease and arrhythmia problem were summarized here. The Division of Pediatric Cardiology performs both diagnostic and intervention cardiac catheterization in almost 310 children and adults with congenital heart disease yearly. Out of this 35 per cent had interventional procedures including balloon valvuloplasty, balloon angioplasty and stenting, device closure of atrial septal defect and patent ductus arteriosus and radiofrequecy ablation of abnormal conduction pathway. Major progress has been made in pediatric cardiac imaging over the past two decades. At Her Majesty's Cardiac Center, cardiac MRI has been used to evaluate patients with congenital heart disease since June 2000. There were 146 congenital heart disease patients who had cardiac MRI performed for the evaluation of anatomy, function, left to right shunt, and flow regurgitation quantification. Conclusion: Pediatric Cardiology practice has evolved over the past decade and expanded from clinical practice to therapeutic intervention procedures.

Prenatal diagnosis of cardiovascular diseases.

Fetal echocardiography is a safe means to obtain reliable anatomical and hemodynamic data of the fetal heart. The procedure is essential for prenatal diagnosis of cardiovascular abnormalities. In addition, fetal echocardiography is useful to follow-up the progression, monitoring during treatment and making a plan of treatment especially in life-threatening cardiac conditions. The objective of this study was to find the distribution of indications for fetal echocardiography, fetal cardiac anomalies detected, outcome of the fetuses and to assess the accuracy of the procedure. A retrospective study was done at Siriraj Hospital, Mahidol University, Bangkok, Thailand. Pregnant women whose fetuses had prenatal echocardiographic data and gave birth from January 1999 to December 2001 were included. The total number of pregnant women was 54 cases. The indications for fetal echocardiography were suspicion of fetal cardiovascular disease by the obstetrician 42.6 per cent, family history of congenital heart diseases 14.8 per cent, multiple organ system anomalies 14.8 per cent, chromosomal anomalies 11.1 per cent, hydrops fetalis 9.3 per cent, maternal systemic diseases 5.6 per cent and previous pregnancy with a dead fetus in utero 1.9 per cent. The gestational age at the time of the first fetal echocardiography ranged from 17 to 39 weeks (median 28 weeks). The number of fetal echocardiography done in each case ranged from 1 to 10 (median 1.4). Prenatal diagnosis of the abnormal cardiovascular system in fetuses was found in 19 cases (35.2%). There were abnormal cardiac anatomies in 42 per cent, cardiac tumors or abnormal masses in 37 per cent and rhythm disturbances in 21 per cent. When compared with postnatal echocardiography and/or autopsy finding, fetal echocardiography had a sensitivity of 100 per cent, specificity of 96.3 per cent and accuracy of 97.8 per cent. Conclusion: Fetal echocardiography has good accuracy in the diagnosis of cardiovascular diseases. Obstetrician's suspicion is important in the prenatal diagnosis of heart disease that would have affected the short and long-term outcome of the fetus.