Aortic Root Dilatation Measured by Cardiac Magnetic Resonance in Patients with Repaired Tetralogy of Fallot.

BACKGROUND: Aortic root dilatation (AoD) frequently occurs following repaired tetralogy of Fallot (rTOF). The objective of this study was to assess aortic dimensions, investigate the prevalence of AoD, and identify predictors of AoD in rTOF patients. METHODS: A cross-sectional retrospective study was conducted in repaired TOF patients from 2009 to 2020. Aortic root diameters were measured by cardiac magnetic resonance (CMR). Severe AoD of the aortic sinus (AoS) was defined as a Z-score (z) of >4, reflecting a mean percentile ≥99.99%. RESULTS: Two hundred forty-eight patients, with a median age of 28.2 years (10.2-65.3 years), were included in the study. The median age at the time of repair was 6.6 years (0.8-40.5 years) and the median interval between the repair and CMR study was 18.9 years (2.0-54.8 years). The prevalence of severe AoD was found to be 35.2% when defined by an AoS z greater than 4 and 27.6% when defined by a AoS diameter ≥40 mm, respectively. A total of 101 patients (40.7%) had aortic regurgitation (AR), with 7 patients (2.8%) having moderate AR. Multivariate analysis revealed that severe AoD was only associated with the left ventricular end diastolic volume index (LVEDVi) and a longer duration after repair. The age at the time of repair for TOF was found not to be correlated with the development of AoD. CONCLUSIONS: After repair of TOF, severe AoD was found to be prevalent, but no fatal complications were observed in our study. Mild AR was also commonly observed. Larger LVEDVi and a longer duration after repair were identified as factors associated with the development of severe AoD. Therefore, routine monitoring of AoD is recommended.

Major infections following pediatric cardiac surgery pre- and post-CLABSI bundle implementation.

Background: Postoperative infection contributes to the worsening of congenital cardiac surgery (CCS) outcomes. Surgical site infection (SSI), bloodstream infection (BSI) and ventilator associated pneumonia (VAP) are common. An additional bundle of preventive measures against central-line associated bloodstream infection (CLABSI) bundle was implemented in April 2019. Objectives: To compare the incidence of major infections after pediatric CCS before and after the implementation of the CLABSI bundle and to identify risk factors for major infections. Methods: We conducted a single-center, retrospective study to assess the incidence of major infections including bloodstream infection (BSI), surgical site infection (SSI), and ventilator-associated pneumonia (VAP) after pediatric CCS one year before and after implementation of the CLABSI bundle during April 2018-March 2020. The demographics and outcomes of the patients were explored, and risk factors for major infections were identified using multivariate analysis. Results: A total of 548 children (53% male) underwent CCS with a median age of 1.9 years (range 0.01-17.5 years). The median Aristotle Basic Complexity score was 7.1 (range 3-14.5). The CLABSI bundle was applied in 262 patients. Overall mortality was 5.5%. 126 patients (23%) experienced major postoperative infections. During the year after the implementation of the CLABSI bundle, BSI was reduced from 8.4% to 3.1% (p = 0.01), with a smaller reduction in VAP (21% to 17.6%; p = 0.33). The incidence of SSI was unchanged (1.7% to 1.9%; p = 0.77). The independent risk factors for major infections were age at surgery <6 months (p = 0.04), postoperative ventilator usage >2 days (p < 0.01), central line usage >4 days (p = 0.04), and surgery during the pre-CLABSI bundle period (p = 0.01). Conclusion: Following the implementation of the CLABSI prevention package in our pediatric CCS unit, the incidence of BSI was significantly reduced. The incidence of VAP tended to decrease, while the SSI was unchanged. Sustainability of the prevention package through nurse empowerment and compliance audits is an ongoing challenge.

Risk factors and long-term outcomes after tetralogy of Fallot repair at an Asian tertiary referral center.

BACKGROUND: Tetralogy of Fallot is the most common type of cyanotic congenital heart disease. More postoperative tetralogy of Fallot patients grow up than in the past, and these patients need to be followed-up. OBJECTIVE: To investigate the survival and long-term outcomes of patients who underwent total repair of tetralogy of Fallot, and to identify the risk factors for reoperation with pulmonic valve replacement. METHOD: A total of 403 patients who underwent total tetralogy of Fallot repair at our center during 1997 to 2016 were retrospectively included. Demographic, clinical, treatment, outcome, and follow-up data were collected and analyzed. RESULTS: Median age and body weight at the time of tetralogy of Fallot repair was 4.41 years (range: 0.85-55.28) and 13.58 kg (range: 5.5-68), respectively. The median follow-up was 9.0 years, and overall mortality was 3.2%. The actuarial survival rates at 10 and 20 years were 96.4% and 95.2%, respectively, and the freedom from pulmonic valve replacement was 93.4% and 57.4%, respectively. The median time to indicate pulmonic valve replacement was 13.9 years (range: 6.2-20.5). Multivariate analysis revealed transannular patch technique (hazard ratio: 3.023, 95% confidence interval: 1.34-6.83; p = 0.008) and palliative shunt (hazard ratio: 2.39, 95% confidence interval: 1.16-4.91; p = 0.018) to be independent risk factors for reoperation with pulmonic valve replacement. CONCLUSION: The rates of overall survival and freedom from pulmonic valve replacement were both high in this study, and both were comparable to the rates reported from other studies. Overall mortality was as low as 3.47%. The need for a transannular patch or palliative shunt should be considered risk factors for a consequent reoperation.

Contemporary outcomes and mortality risks of Ebstein anomaly: A single-center experience in Thailand.

BACKGROUND: The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their "real world" survival. OBJECTIVES: To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era. METHODS: All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardiographic findings, treatments, and outcomes were explored. The survival analysis was performed at the end of 2017. A multivariate analysis was used to assess mortality risks. RESULTS: A total of 153 patients (25.4 ± 20.4 years, 60% female) were analyzed. Of these, 89 patients had been diagnosed with EA in childhood. During the follow-up [median time of 5.2 years (3 days-23.5 years)], 32 patients (20.9%) died due to major cardiac adverse events. The overall survival at 1, 5, and 10 years were 89%, 82.2%, and 79%, respectively. Of the total 153 patients, 64 patients underwent at least one surgical intervention [median age of 17 years (1 day-64.4 years)]. The survival at 1, 5, and 10 years were 87.5%, 82.4%, and 77.7%, respectively, in patients with EA surgery. This survival is comparable to the survival of 89 nonoperated patients with EA: 89.9%, 87.5%, and 81.8%, at 1, 5, and 10 years, respectively. The significant predictors of mortality were: age at diagnosis ≤2 years, tricuspid valve (TV) z-score >3.80, TV displacement >19.5 mm/m2 , presence of severe tricuspid regurgitation, and absence of forward flow across the pulmonic valve at the initial diagnosis. CONCLUSION: Patients with EA had a moderately good survival in this era. In this paper, we report five simple predictors of death in this patient population.

Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts.

OBJECTIVE: To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort. METHODS: All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded. Baseline characteristics and clinical outcomes were retrieved from the database. The survival analysis was performed at the end of 2016. Prognostic factors were identified using multivariate analysis. RESULTS: A total of 366 consecutive patients (24.5 ± 17.6 years of age, 40% male) with PAH-CHD were analyzed. Most had simple shunts (85 pre-tricuspid, 105 post-tricuspid, 102 combined shunts). Patients with pre-tricuspid shunts were significantly older at diagnosis in comparison to post-tricuspid, combined, and complex shunts. Clinical classifications identified patients as having Eisenmenger syndrome (ES, 26.8%), prevalent left to right shunt (66.7%), PAH with small defect (3%), or PAH following defect correction (3.5%). At follow-up (median = 5.9 years; 0.1-20.7 years), no statistically significant differences in survival rate were seen among the anatomical-pathophysiological shunts (p = 0.1). Conversely, the clinical classifications revealed that patients with PAH-small defect had inferior survival compared to patients with ES, PAH post-corrective surgery, or PAH with prevalent left to right shunt (p = 0.01). Significant mortality risks were functional class III, age < 10 years, PAH-small defect, elevated right atrial pressure > 15 mmHg, and baseline PVR > 8 WU•m.2. CONCLUSION: Patients with PAH-CHD had a modest long-term survival. Different anatomical-pathophysiological shunts affect the natural presentation, while clinical classifications indicate treatment strategies and survival. Contemporary therapy improves survival in deliberately selected patients.

Fetal long QT syndrome manifested as atrioventricular block and ventricular tachycardia: a case report and a review of the literature.

Fetal onset of congenital long QT syndrome (LQTS) is a rare manifestation, and prenatal diagnosis is difficult. This report describes a boy who presented with both atrioventricular (AV) block and ventricular tachycardia during the antenatal period. The early postnatal electrocardiogram showed prolongation of the QT interval and AV block, subsequently leading to a polymorphic ventricular tachycardia torsade de pointes. This unique feature of congenital LQTS has a poor outcome, but the boy was successfully treated with beta-blockers and implantation of an automated cardioverter-defibrillator. The intrauterine manifestation of fetal AV block and ventricular tachycardia should raise a high suspicion of congenital LQTS, and the strong association with a malignant clinical course should warrant special evaluation. The literature on the prenatal diagnosis, fetal therapy, and neonatal outcome of this condition also are reviewed.

An interarterial course of anomalous coronary artery and an alternative surgical option.

An interarterial course of anomalous left main coronary artery originating from the right aortic coronary sinus of valsalva is a rare condition among anomalous aortic origin of the coronary artery. Various surgical options are available. We performed an alternative procedure, that is, mobilisation of the pulmonic root, pulmonary bifurcation, and plication of the pulmonic root adjacent to the left main coronary artery, in a 12-year-old boy. Favourable results were achieved after 43 months of follow-up.

Surveillance of pediatric cardiac surgical outcome using risk stratifications at a tertiary care center in Thailand.

Objectives. To determine in-hospital mortality and complications of cardiac surgery in pediatric patients and identify predictors of hospital mortality. Methods. Records of pediatric patients who had undergone cardiac surgery in 2005 were reviewed retrospectively. The risk adjustment for congenital heart surgery (RACHS-1) method, the Aristotle basic complexity score (ABC score), and the Society of Thoracic Surgeons and the European Association for Cardiothoracic Surgery Mortality score (STS-EACTS score) were used as measures. Potential predictors were analyzed by risk analysis. Results. 230 pediatric patients had undergone congenital cardiac surgery. Overall, the mortality discharge was 6.1%. From the ROC curve of the RACHS-1, the ABC level, and the STS-EACTS categories, the validities were determined to be 0.78, 0.74, and 0.67, respectively. Mortality risks were found at the high complexity levels of the three tools, bypass time >85 min, and cross clamp time >60 min. Common morbidities were postoperative pyrexia, bleeding, and pleural effusion. Conclusions. Overall mortality and morbidities were 6.1%. The RACHS-1 method, ABC score, and STS-EACTS score were helpful for risk stratification.

The results of transcatheter closure of VSD using Amplatzer® device and Nit Occlud® Lê coil.

OBJECTIVE: We retrospectively reviewed the result of patients who underwent transcatheter closure of ventricular septal defect (VSD) using Amplatzer® Perimembranous or Amplatzer® muscular VSD device (the Amplatzer® group) and Nit Occlud® Lê VSD Coil (the Pfm group). BACKGROUND: Perimembranous type (PmVSD) and doubly committed subarterial (DCSA) VSD were the major isolated congenital heart defects in Thai children. Transcatheter device closure technique for both types of VSD has emerged as an alternative treatment to surgery. METHODS: Retrospectively, data was reviewed between 2003 and 2009. RESULTS: 116 patients were enrolled. Device or coil was successfully implanted in 94%. Complete closure at 24 hr is slightly higher in the Amplatzer® group. The average size of VSD in the Amplatzer® group was larger than the Pfm group (P = 0.001). The Pfm coil was primarily deployed in DCSA VSD when compared with the Amplatzer® group (P < 0.01). At 6 months follow-up the residual shunt was comparable (P = 0.054). There was only one transient AV block (AVB) in the Pfm group and 5 AVB in the Amplatzer® group. Four pacemakers were placed in the Amplatzer® group. CONCLUSIONS: Transcatheter closure of VSD in both Pm VSD and DCSA can be achieved by using either of the device. The Amplatzer® VSD device had the advantage of closure of larger defects with immediate less residual shunt but appeared to have a significant number of 3° AVB, which required pacemaker implantation. The Nit Occlud® Lê VSD Coil had the advantage of closure of both types of defects, in particular DCSA VSD with only small residual shunt.

Medium and long-term outcomes of Fontan operation.

BACKGROUND: The Fontan operation had been proposed as the final palliative surgery in the patients with single ventricle physiology. Even though modifications of the operation were developed to improve outcomes, long-term complications remain significant with time. The present study reviewed long-term survival rate, morbidities associated with time, and risk factors during the follow-up period after Fontan operation. MATERIAL AND METHOD: A retrospective study was conducted. Every patient who underwent the Fontan operation at Siriraj Hospital between January 1987 and December 2007 and had available data was included in the present study. The data was collected until the most recent follow-up in December 2008. Demographic data, diagnosis, echocardiographic data, cardiac catheterization data, surgical data, type of modified Fontan procedure, and perioperative data were collected. The follow-up clinical data, cardiac investigation data, complications, and management were also collected and analyzed. RESULTS: Survival rates were 88.7%, 85.3%, and 83.8% at 1 year, 5 years, and 10 years, respectively. The median follow-up time was 4.75 years (0-17.45). The 10-years survival rate of tricuspid atresia, single ventricle and the heterotaxy syndrome were 94.5%, 79%, and 83.3%, respectively, which were not significantly different (p = 0.09). The 10-years survival rates of the patients that underwent lateral tunnel, extracardiac conduit and atriopulmonary connection were 80.7%, 88% and 84.3%, respectively. A mean pulmonary artery pressure of more than 18 mmHg was the only factor that affected the survival rate after Fontan surgery (p = 0.008). The incidence of postoperative arrhythmia was 7.9%. Age at operation, diagnosis, type of operation, fenestration, systemic EDP, or PVR before operation did not significantly affect the survival rate. Diagnosis and type of surgery did not affect long-term outcome regarding arrhythmia, re-intervention, systemic atrioventricular valve regurgitation, and systemic ventricular dysfunction. Patients post Fontan operation had good survival rate. CONCLUSION: Cardiac diagnoses were not significantly different in the medium and long-term survival rate of post Fontan patients, freedom from arrhythmia, re-intervention and systemic atrioventricular regurgitation. Types of Fontan operation did not affect long-term survival rate or long-term complications. Mean pulmonary artery pressure of more than 18 mmHg was the only risk factor to the survival rate.

N-terminal pro brain natriuretic peptide and cardiac function in doxorubicin administered pediatric patients.

OBJECTIVE: Assess the use of N terminal pro brain natriuretic peptide (NT-pro BNP) to early diagnose ventricular dysfunction in doxorubicin-administered children. MATERIAL AND METHOD: Fifty-five cancer patients who received accumulative dose of doxorubicin <300 mg/m2 (group 1), 49 cases with accumulative dose > or = 300 mg/m2 (group 2) and 52 cases as a control group (group 3) were included in the study. Electrocardiogram, chest roentgenogram, echocardiogram, and serum NT-pro BNP were studied. RESULTS: At age 1-10 years, there were significantly higher NT-pro BNP in group 2 than group 1 (384 +/- 291 vs. 92.2 +/- 89 pg/ml; p = 0.001), and than group 3 (79 +/- 92 pg/ml; p = 0.001). Patients with NT-pro BNP level > 1 SD of the control group were more likely to have abnormal > or = 2 echocardiographic parameters of left ventricular diastolic dysfunction than patients with NT-pro BNP < or = 1 SD (OR = 3.8, 95% CI 1.18-12.5). Patients in group 2 were more likely to have abnormal > or = 2 parameters of left ventricular diastolic dysfunction than patients in group 1 (OR = 2.8, 95% CI 1.07-7.7) and more likely to have NT-pro BNP >1 SD than group 1 (OR = 8, 95% CI 1.96-38.4). There were association of NT-pro BNP > 1 SD, accumulative dose of doxorubicin > or = 300 mg/m2, and early left ventricular diastolic dysfunction by echocardiogram. CONCLUSION: Serum NT-pro BNP > 1 SD has a high probability to diagnose early doxorubicin-induced cardiomyopathy in patient 1-10 years old.

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.

Management of chylothorax and chylopericardium in pediatric patients: experiences at Siriraj Hospital, Bangkok.

Chylothorax and chylopericardium are rare conditions occurring in infants and children. Both may be traumatic or nontraumatic in origin. We reviewed our experiences with the management of sixteen pediatric cases (10 males, 6 females; 11-days to 14-years old) of chylothorax and chylopericardium from 1997 to 2003. There were fifteen cases of chylothorax (9 left, 2 right, 4 bilateral) and two cases of chylopericardium (1 isolated and 1 associated with chylothorax), and their incidences of occurrence after cardiothoracic surgery were 0.89% and 0.12%, respectively. Of the fifteen cases occurring after cardiothoracic surgery, thirteen patients had corrective or palliative surgery for complex congenital heart disease, and two patients had removal of thymolipoma and neuroblastoma, respectively. Included also in this review was an 11-day old preterm infant with hydrops fetalis and congenital heart disease who developed chylothorax. Characteristics of chylous effusion included a presence of whitish opaque fluid in the pleural cavity and the pericardial cavity, having a triglyceride content ranging from 59 to 1689 mg/dl which was higher than a plasma triglyceride, a protein content of 2.4 to 7.4 g/dl, and a presence of lymphocyte predominance. The average latent period for diagnosis of chylothorax or chylopericardium was 13 days (range 3-30). All patients were treated primarily with nutritional modification using medium-chain triglycerides (MCT) instead of long-chain triglycerides in their diet; and only a few cases needed bowel rest with total parenteral nutrition (TPN). Twelve patients completely responded to a MCT-rich diet; two cases resolved after switching to TPN and another case needed surgery for ligation of lymphatic vessels around the thymus gland. The mean duration of lymph drainage was 12.1 days (range 3-29) and the average length of time of continued conservative treatment (MCT-rich diet and TPN) was 29.8 days (range 18-47). Fourteen patients (87.25%) had good outcome, i.e. resolution from chylothorax or chylopericardium and return to normal diet. Two of the fourteen patients developed severe infections; one was diagnosed with suspected bacterial endocarditis and the other had candidemia. However, both responded well to antibacterial and antifungal drugs, respectively. One case succeeded after surgery. A case that had low compliance with dietary recommendations and required repeated placement of drainage devices died due to infection with enterococcal septicemia. Early and good compliance with MCT-rich diet is essential for achieving a favorable outcome in the management of chylothorax and chylopericardium in children.

Permanent cardiac pacing in pediatrics: experience in Thailand.

UNLABELLED: 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.

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.

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.

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.

Transcatheter coil occlusion of small patent ductus arteriosus: experience at Siriraj Hospital.

UNLABELLED: Transcatheter coil occlusion of small-to-moderate-size patent ductus arteriosus (PDA, < 3.5 mm) is well established as a procedure of first choice in many institutions. Its much lower cost compared with surgical ligation or other devices makes it an attractive option, especially in Thailand. PATIENTS AND METHOD: Between September 1995 and June 2000, all patients diagnosed with PDA with audible murmur and echo-Doppler confirmation of diameter less than 3.5 mm were scheduled for transcatheter coil occlusion at the Department of Pediatrics, Faculty of Medicine Siriraj Hospital. The hemodynamic studies were obtained both pre and post occlusion. The immediate and late outcome, including complication were assessed. RESULTS: A total of 77 cases, 78 procedures of transcatheter PDA coil occlusion were performed. Seventy cases (90.9%), comprised of 19 males (27.1%) and 51 females (72.9%) were successfully deployed with coils. The remainder were unsuccessfully deployed and later referred to surgery. The median age of the successful group was 6 years and 6 months and median weight was 16.5 kg. Twenty cases (28.6%) had other associated intra and extracardiac anomalies. All patients were asymptomatic, except one case having bronchopulmonary dysplasia (BPD) from prolonged ventilation. Single-coil occlusions were performed in 74.3 per cent and double-coil occlusions in 25.7 per cent. Conventional 0.038-inch Gianturco coils were deployed in 86.5 per cent. The mean procedure time was 78.1 +/- 35.1 minutes. The mean fluoroscopic time was 20.2 +/- 15.6 minutes. The total complete occlusion rate was 87.7 per cent. Tiny residual flow of PDA was demonstrated by follow-up echocardiogram in 12.3 per cent. Seven per cent of the patients were lost to follow-up. There was no significant difference in PDA size and hemodynamics between the groups of patients with complete occlusion and having residual shunt. Minor complications occurred in 12.9 per cent, including mild left pulmonary artery stenosis (10%), coil embolization to distal pulmonary artery (8.6%), slippage of catheter with coil (2.9%) and decreased dorsalis pedis pulse (1.4%). One late death was found in a BPD patient from pneumonia 2 months after the procedure. CONCLUSION: Transcatheter coil occlusion of PDA is as effective, feasible, safe and less costly than surgical ligation. With improvement in technique and device selection and appropriate case selection, there should be an increase rate of complete occlusion and a decrease in complications.

Prenatal diagnosis of cardiovascular diseases.

UNLABELLED: 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.