Permanent pacemaker implantation in post one-and-a-half ventricle repair with tricuspid valve replacement in Ebstein anomaly: An innovative technique.

INTRODUCTION: Transvenous pacemaker implantation in patients post bidirectional Glenn anastomosis in one-and-a-half ventricle repair is usually not feasible. However, with a modified surgical technique for Glenn anastomosis and a combined interventional and electrophysiologic approach, the transvenous pacemaker was successfully implanted. FINDINGS AND CONCLUSIONS: We reported a novel technique of pacemaker implantation in a 27-year-old woman, underlying Ebstein anomaly of the tricuspid valve, who developed intermittent complete atrioventricular block at 5 years after surgical repair. The patient had a tricuspid valve replacement and a novel modified bidirectional Glenn anastomosis for one-and-a-half ventricle repair. The Glenn circuit was conducted by opening a window between the posterior wall of the superior vena cava (SVC) and the anterior wall of the right pulmonary artery (RPA), combined with putting a Goretex membrane in the SVC below the SVC-RPA window without disconnecting the SVC from the right atrium. The transvenous pacemaker was implanted by perforating the Goretex membrane, then passing the leads from the axillary vein through the perforated membrane and placing them in the coronary sinus and right atrium.

Effect of Balloon Pulmonary Valvuloplasty on Growth of Pulmonary Annulus in Infants with Tetralogy of Fallot.

Percutaneous balloon pulmonary valvuloplasty (PBPV) is an alternative intervention in infants with Tetralogy of Fallot (TOF). It can not only improve hypoxia but also promote pulmonary annulus (PA) growth. In this study, we evaluated the effect of PBPV on PA growth in infants with TOF. To eliminate the effect of the systemic to pulmonary shunt (SPS) that may promote PA growth, we divided TOF infants into 2 groups: group A, patients who underwent PBPV with or without other SPS, and group B, patients who attempted SPS but without PBPV. Sixty patients were included, 28 patients in group A and 32 patients in group B. Age at the time of intervention in group A (range, 0.4-5.4; median 1.4 months) was lower than that in group B (range, 2.3-7.7; median 4.8 months), p-value 0.02. The body weight in group A (range, 3-5.5; median 3.7 kg) was also lower than that in group B (range 4.1-6.4; median 5.9 kg), p-value 0.02. Echocardiographic data at the mean follow-up period of 37.2 months (3-88 months) in group A and 39.6 months (6-95 months) in group B demonstrated an increase in mean PA diameter from 5.0 ± 1.3 mm to 10.2 ± 2.9 mm, p-value < 0.001 in group A; and from 6.2 ± 2 mm to 9.5 ± 2.9 mm, p-value < 0.001 in group B. The median PA z-score increased from - 3.4SD (- 3.9 to - 2.6SD) to - 1.8SD (- 2.5 to - 0.8SD), with the p-value of 0.002 in group A; and increased from - 2.9SD (- 4.5 to - 1.3SD) to - 2.7SD (- 3.6 to - 1.4SD), with the p-value of 0.73 in group B. By using the PA z-score as the absolute value, there was a statistically significant increase in the PA z-score during follow-up in group A, but not in group B. Balloon pulmonary valvuloplasty in infants with TOF can facilitate the growth of the pulmonic annulus even after eliminating the effect of the systemic to pulmonary shunt.

Outcomes of duct stenting and modified Blalock-Taussig shunt in cyanotic congenital heart disease with duct-dependent pulmonary circulation.

BACKGROUND: Transcatheter ductus arteriosus stenting (DS) is emerging as an alternative method to modified Blalock-Taussig shunt (MBTS) in providing pulmonary blood flow in cyanotic congenital heart disease (CCHD) with duct-dependent pulmonary circulation. OBJECTIVE: To evaluate post-procedural outcomes and survival between patients undergoing DS and MBTS. METHODS: All infants ≤ 60 days of age having CCHD with diminished pulmonary blood flow who underwent palliative procedure either with MBTS or DS at King Chulalongkorn Memorial Hospital during January 1st, 2013 and December 31th, 2017 were retrospectively reviewed. RESULTS: 98 patients were included; 34 patients underwent a transcatheter DS and 64 patients underwent MBTS. There was no significant difference in post-procedural outcomes and overall mortality rate between two groups (17.6% in MBTS group and 6.1% in DS group, p = 0.09). Single ventricle morphology was the major risk factor associated with increased mortality compared with biventricular morphology (aHR 3.9, 95% CI 1.49-10.2, p = 0.01). There was similar number of early and pre-repair additional interventions focusing on MBTS/DS patency between two groups. The MBTS group had a greater number of early interventions on PA branch stenosis related to baseline diagnosis. Risk factors associated with additional intervention were pre-existing pulmonary branch stenosis (aHR 2.54, 95% CI 1.3-4.97, p = 0.006) and body weight less than 2.5 kg (aHR 3.33, 95% CI 1.57-7.08, p = 0.003). Having pulmonic valve perforation or balloon pulmonary valvuloplasty to promote antegrade pulmonary blood flow could result in a lower number of additional interventions required before definitive repair. CONCLUSION: Duct stenting is a feasible and safe alternative to MBTS in cyanotic infants with duct-dependent pulmonary circulation. However, mortality rate was significantly higher in patients with single ventricle that required careful follow-up after procedure.

State-of-the-art preclinical testing of the OMEGATM left atrial appendage occluder.

OBJECTIVES: This study aimed to present a new approach of thorough preclinical testing of a novel left atrial appendage (LAA) occluder device. BACKGROUND: The development of a safe and effective LAA occluder has been shown to be challenging. METHODS: The novel OMEGATM LAA occluder (Eclipse Medical, Ireland) was tested in a porcine model and three-dimensional (3D) human LAA models - this as a prelude to its first-in-human use. RESULTS: In a first series of in-vivo experiments, the OMEGATM LAA occluder was shown to have a satisfactory device biocompatibility in a porcine model. The design of the OMEGATM device was further refined and optimized following three more series of in-vivo experiments. The second generation OMEGATM device was designed with thinner wires, leading to a profile reduction. Based on in-vitro testing of different OMEGATM device sizes implanted at different depths in human three-dimensional (3D) LAA models, it could be determined that (1) the landing zone should be measured at a median depth of 12 mm from the LAA ostium; (2) the distal self-retaining inverted cup should have 10%-25% compression to minimize device embolization risk; and (3) the disc should be slightly inverted, i.e. pulled into the LAA, to promote complete LAA occlusion. The combined in-vivo and in-vitro testing resulted in an optimized pre-procedural planning of the first-in-human case treated with the OMEGATM device. CONCLUSIONS: This series of carefully planned in-vivo and in-vitro experiments allowed demonstration of the safety and efficacy of the OMEGATM LAA occluder. This approach of thorough preclinical testing of medical devices may reduce the risk of complications in first-in-human cases and may become the standard approach for device development and preclinical testing in the future.

New PFO device for closure of patent foramen ovale in patients who had a history of cryptogenic stroke; a report of 14 cases.

OBJECTIVE: The Cocoon PFO Occluder is a device for percutaneous closure of inter-atrial communications. Its self-centering characteristics make it attractive for closure of patent foramen ovales (PFOs) with or without atrial septal aneurysms. The goal of this study is to report the immediate and follow-up results of the first 14 patients in implanted with the Cocoon PFO Occluder. MATERIAL AND METHOD: This is a retrospective report of immediate and short-term clinical and echocardiographic outcome of patients who underwent transcatheter closure of PFO because of paradoxical embolism. Procedural success was defined as successful deployment of the device and effective occlusion (no, or trivial, shunt after device placement). All patients had a transesophageal echocardiography (TEE) with saline contrast injection at baseline and clinical follow-up at 6 months. RESULTS: Between September 2012 and March 2014, 14 patients had successfully undergone transcatheter closure of PFO using Cocoon device. During follow-up none of the patients had a recurrence of stroke after device closure. No residual shunt was observed in any patients at follow-up. CONCLUSION: Transcatheter closure of PFO with the Cocoon PFO device is safe and effective and can be used for preventing recurrent strokes in patients who present with cryptogenic stroke and PFO.

Transcatheter treatment of iatrogenic brachiocephalic-jugular arteriovenous fistula and aortopulmonary artery fistula: a case report.

Iatrogenic arteriovenous fistula is not a common complication of central venous catheterization. Duct occluder devices have been developed for patent ductus arteriosus occlusions but they may be used for arteriovenous fistula closures. We report a case of iatrogenic brachiocephalic-jugular and aortopulmonary artery fistulas after central venous catheter insertion. The fistulas were successfully managed with duct occluder devices. Due to increasing number of central venous catheterizations, physicians should be aware of this uncommon complication. Transcatheter closing of brachiocephalic-jugular and aortopulmonary artery fistulas by duct occluder devices seems to be a safe and feasible form of treatment.

A prospective placebo-controlled study on the efficacy of onion extract in silicone derivative gel for the prevention of hypertrophic scar and keloid in median sternotomy wound in pediatric patients.

BACKGROUND: New topical treatments studied in adults have been published to be potentially effective in the treatment of hypertrophic scar and keloids. There is still no study in Thai children. OBJECTIVE: To study the efficacy of 10% onion extract in silicone derivative gel for the prevention of hypertrophic scar and keloid in median sternotomy wound from open heart surgery in pediatric patients. MATERIAL AND METHOD: Thirty-nine pediatric patients who had median sternotomy were recruited in this prospective randomized, double-blinded, placebo-controlled split-scar experimental study. The wound in each patient was divided into upper and lower parts, and the treatment with, either onion extract gel or placebo was randomly applied by block randomization onto each part of the wound twice daily after the seventh day postoperatively for six months. The incidence of scars, serial photographs of the wound, Vancouver Scar Scale (VSS), and adverse effect were assessed at each visit. RESULTS: Thirty patients with the mean age of 4.3 years completed the 6-month study period. Six patients (20%) in onion extract gel group had no scar compared to one patient (3.3%) in placebo group (p = 0.04). Of the 27 patients with hypertrophic scar nine were from onion extract gel group and 18 from the placebo group (p = 0.02). Keloid was not statistically significant diferent in both groups (p = 0.29). VSS was not statistically significant different in all visits. One case had a pustule on the part of the onion extract in silicone derivative application at the sixth month. CONCLUSION: Onion extract in silicone derivative gel can significantly decreased the incidence of hypertrophic scar from median sternotomy wound in pediatric patients. Keloid did not show statistically significant differences in both groups.

Clinical results of large secundum atrial septal defect closure in adult using percutaneous transcatheter Cocoon atrial septal occluder.

BACKGROUND: Atrial septal defect (ASD) is a common congenital heart disease in adults. Amplatzer septal occluder is one of the most common devices used for transcatheter closure due to its high success rate and ease to implant. Cocoon atrial septal occluder is a new nitinol-based device, its shape resembles Amplatzer septal occluder but coated with platinum to prevent nickel release. Little is known about clinical outcomes of large ASD closure using Cocoon atrial septal occluder OBJECTIVE: To review our experience in closure of secundum ASD in adults by Cocoon septal occluder and to compare the clinical outcomes and results of the patients who had ASD closure with a device greater than or equal to 30 mm and less than 30 mm. MATERIAL AND METHOD: Between November 2005 and October 2008, 63 consecutive patients underwent transesophageal echocardiography (TEE)--guided transcatheter closure of secundum ASD. The patients were divided into two groups (Groups' 1 and 2) according to device diameter that is greater than or equal to 30 mm (n = 31) and less than 30 mm (n = 32), respectively. Clinical outcomes, complications, and transthoracic echocardiography (TTE) before hospital discharge, one to three months, and one-year were analyzed. RESULTS: Device implantations were successful in 27 patients (87.1%) in group 1 and 31 patients (96.9%) in group 2 (p = 0.196). The maximum size of secundum ASD in group 1 determined by TTE, TEE, and balloon sizing diameter (BSD) were 22.6 +/- 5.0 mm (range 15-32), 28.1 +/- 4.8 mm (range 19-39), and 31 +/- 3.5 mm (range 23-38) respectively. The maximum size of secundum ASD in group 2 determined by TTE, TEE, and BSD were 19.7 +/- 4.4 mm (range 12-31), 20.4 +/- 3.4 mm (range 13-26), and 23.1 +/- 2.9 mm (range 15-30) respectively. The mean device size in groups 1 and 2 were 33.5 +/- 3.1 mm and 24.6 +/- 3.3 mm, respectively. Four patients (12.9%) in group 1 had unsuccessful implantations. All of them were in the first 15 cases of using large device and two of them had device embolization requiring surgical removal. One patient (3.1%) in group 2 had an unsuccessful implantation and had device embolization requiring surgical removal. The patients in both groups gradually improved in clinical symptoms with decreased RVsystolic pressure and decreased RV size with complete ASD closure at one year CONCLUSION: Transcatheter closure of large secundum ASD by Cocoon septal occluder is feasible with hemodynamic benefit. However complication rates are higher with large ASD closure with device size greater than or equal to 30 mm especially during the early "learning curve" period. With experience, the complication rate declines and the success rate is no different from the group with smaller device size.

Balloon pulmonary valvuloplasty in tetralogy of Fallot: effects on growth of pulmonary annulus and transannular patch.

BACKGROUND: Balloon pulmonary valvuloplasty and its benefit on the growth of pulmonary annulus and pulmonary artery in tetralogy of Fallot (TOF) patients remains controversial. The purpose ofthe present study was to determine the growth of pulmonary valve annulus and pulmonary artery and to evaluate the need of transannular patch during total surgical correction in patients with tetralogy of Fallot (TOF) after balloon pulmonary valvuloplasty. MATERIAL AND METHOD: Fifty-one severely hypoxic TOF patients, who underwent balloon dilatation ofpulmonary valve, were included in the present study. The clinical outcomes, oxygen saturation, and echocardiographic parameters before and after balloon dilatation were analyzed. RESULTS: There were 33 males and 18females. The mean age was 3 years 5 months old (range, I month-15 years 8 months old). The mean oxygen saturation increasedfrom 73.9 +/- 9.1 to 84.8 +/- 6.7% immediately after the procedure (p-value < 0.05). There was no serious procedural-related complication. At the mean follow-up period of 2 years and 4 months, the mean Z-score of pulmonary annulus size increased from -2.56 SD to -1.87 SD (p-value < 0.05) and the right pulmonary artery size from -0.29 SD to + 0.46 SD (p-value < 0.05). Thirty-seven patients (66.1%) underwent corrective surgery. Pulmonary transannular patch was performed in 11 of37 patients (29.7%). CONCLUSION: Pulmonary valve dilatation in patients with TOF is safe. It promotes the growth ofthe pulmonary valve annulus and pulmonary artery and may decrease the need of transannular patch at the time of surgical correction.

Serum level of soluble intercellular adhesion molecule-1 correlates with pulmonary arterial pressure in children with congenital heart disease.

BACKGROUND: Endothelial activation and vascular inflammation are thought to be the mechanisms of pulmonary hypertension. Increased expression of the intercellular adhesion molecule (ICAM-1) and raised serum level of its soluble form (sICAM-1) are found in various conditions associated with endothelial activation. METHODS: Serum samples from 31 children (14 boys and 17 girls; age, 4.9 +/- 4.6 years) with congenital heart disease (CHD) collected at the time of cardiac catheterization were analyzed for sICAM-1 level. Uni- and multivariable stepwise linear regression analyses were performed for the following variables against the sICAM-1 level: age, hemoglobin, serum creatinine, systemic arterial pressure (SAP), pulmonary arterial pressure (PAP), pulmonary blood flow (Qp) and resistance (Rp), systemic blood flow (Qs) and resistance (Rs), Qp/Qs, Rp/Rs, and pulmonary and systemic oxygen saturation. RESULTS: The sICAM-1 levels in children who had CHD with and without pulmonary hypertension were 411 +/- 110 and 344 +/- 81 ng/ml, respectively (p = 0.11). In the univariable models, age, serum creatinine, systolic PAP, mean PAP, diastolic PAP, Rp, and Rp/Rs were significantly correlated with sICAM-1 level. In the multiple stepwise regression model, only mean PAP remained as an independent predictor of sICAM-1 level (r = 0.55; p = 0.002). CONCLUSION: Children with CHD and pulmonary hypertension had a trend toward elevated sICAM-1 compared with CHD children who had no pulmonary hypertension. A linear correlation was found between mean pulmonary arterial pressure and sICAM-1 level.

Recurrent Takotsubo syndrome in a boy with Duchenne muscular dystrophy: A case report.

Takotsubo syndrome is a rare cause of reversible ventricular dysfunction that imitate an acute coronary syndrome. The entity is unusual among pediatric populations and a recurrent episode is extremely rare. We report a case of recurrent takotsubo syndrome in an eight-year-old boy with Duchenne muscular dystrophy (DMD). His chest pain episodes were aggravated by a strong emotional stimuli. During episodes of chest pain, electrocardiograms (ECG) showed ST elevation while echocardiograms showed left ventricle apical ballooning; however, a coronary angiography was normal. Serial ECG and echocardiogram revealed a spontaneous resolution of ST elevation and normalized apical contraction which were compatible with the diagnosis of takotsubo syndrome. Interestingly, serial cardiac magnetic resonance imaging demonstrated increasing subepicardial enhancement which was compatible with progression of cardiac involvement in DMD. .

Myocardial depression in dengue hemorrhagic fever: prevalence and clinical description.

OBJECTIVES: To determine the prevalence of myocardial depression and its effect on the clinical severity in patients with dengue hemorrhagic fever. DESIGN: Clinical study. SETTING: King Chulalongkorn Memorial Hospital, Bangkok, Thailand. PATIENTS: Ninety-one children (age 10.5 +/- 2.9 yrs, male/female = 52/39) with serologically or polymerase chain reaction-proven dengue virus infection. INTERVENTIONS: Left ventricular ejection fraction (EF) was measured. The proportions of patients with EF <50% were identified in patients with dengue fever (DF, n = 30), dengue hemorrhagic fever without shock (DHF, n = 36), and dengue shock syndrome (DSS, n = 25). Comparisons of clinical findings were made among DSS patients with depressed ventricular function (EF <50%), fair ventricular function (EF > or =50% and <60%), and good ventricular function (EF > or = 60%). Serum troponin T was analyzed in nine patients. MEASUREMENTS AND MAIN RESULTS: EF during toxic stage was significantly lower in patients with DSS than DHF, and lower in DHF than DF (p = .05) with rapid recovery within 24-48 hrs. EF <50% was found in 6.7%, 13.8%, and 36% of patients with DF, DHF, and DSS during the toxic stage, respectively (p = .01). DSS patients with poor ventricular function had significantly more tachycardia and hepatomegaly. While end-diastolic volumes were similarly reduced, patients with lower EF tended to have lower cardiac output, required more aggressive intravenous fluid resuscitation, developed larger pleural effusion, and had higher incidence of respiratory embarrassment. No patient had elevated troponin T level. CONCLUSIONS: Transient myocardial depression is not uncommon in patients with DSS. Cardiac dysfunction in children with DSS may contribute to the clinical severity and the degree of fluid overload in these patients.

Self-expanding nanoplatinum-coated nitinol devices for atrial septal defect and patent ductus arteriosus closure: a swine model.

OBJECTIVES: Our purpose was to evaluate self-expanding nanoplatinum-coated nitinol devices for transcatheter closure of atrial septal defects and patent ductus arteriosus in a swine model. The devices were braided from platinum-activated nitinol wires and filled with polyester to enhance thrombogenicity. The platinum activation of the nitinol wires was carried out with the help of Nanofusion technology. The coating of platinum covers the exposed surface of the nitinol wires and prevents the release of nickel into the blood stream after the implantation of the device but does not affect its shape memory, which makes the device self-expanding after it is loaded from the catheter. METHODS AND RESULTS: Atrial septal defects were created in 12 piglets by balloon dilation of the patent foramen ovale. The size of the device was selected on the basis of the diameter of the balloon and the size of the defect, measured by transthoracic echocardiography. The devices were successfully deployed in all 12 piglets under fluoroscopic study. Transthoracic color Doppler echocardiograms showed complete closure of the atrial septal defect within 15 minutes of device implantation. Twelve patent ductus arteriosus closure devices were deployed in the right or left subclavian arteries in 10 piglets. Angiograms showed complete occlusion of the subclavian arteries within a few minutes of device deployment. In the atrial septal defect cases, the autopsy findings showed complete organizing fibrin thrombus formation and complete neo-endothelialization on the outer surface of the devices within one week and six weeks of implantation, respectively. CONCLUSION: The use of self-expanding nanoplatinum-coated nitinol devices for the transcatheter closure of atrial septal defects and patent ductus arteriosus is feasible. The excellent occlusion result and complete neo-endothelialization of the devices in the swine model is an indication of the potential of these devices in human application.

Clinical course and outcome of children with single ventricle physiology at King Chulalongkorn Memorial Hospital.

BACKGROUND: Children with single ventricle physiology comprise 10% of all children with congenital heart disease (CHD) and one-third of children with cyanotic CHD seen at King Chulalongkorn Memorial Hospital. The prognosis of these children is generally thought to be poor but no study of the outcome has previously been done in this hospital and in Thailand. OBJECTIVES: To study the clinical course and outcome of children with single ventricle physiology at King Chulalongkorn Memorial Hospital during the current era. MATERIAL AND METHOD: One hundred and seventeen patients with single ventricle physiology were initially seen at this hospital during the year 1999-2001. Retrospective chart reviews were carried out in 90 children with available medical records. The status of the patients was determined in 2003 at clinic visits, by phone calls and mail. RESULTS: The main diagnoses were tricuspid atresia (TA, n = 10), pulmonary atresia with intact ventricular septum (PA/IVS, n = 16), single ventricle associated with cardiac malposition or heterotaxy syndrome (malposition, n = 35), hypoplastic left heart syndrome (HLHS, n = 11) and other complex single ventricle (others, n = 18). Most children did not have other major anomalies. Survival of patients with TA and PA/IVS was approximately 92 and 87% at 1 and 4 years, respectively. For patients in the other three groups, 1 and 4 year survival was 69 and 42%, respectively. Patients with HLHS had the worse survival, partly because of decisions not to pursue further treatments by the parents. Among HLHS patients who underwent Norwood procedure, the 1 and 4 year survival were 83% and 42%, respectively. Approximately 40 and 90% of all patients underwent heart surgery at 1 and 4 years follow-up, respectively. CONCLUSION: Despite limited resource, the short-term outcome of Thai children with single morphologic left ventricle (TA and PA/IVS) is reasonably good. Timely evaluation and treatment of these children toward Fontan procedure should be ensured. After proper discussion with the parents, palliative care is still acceptable for patients with single morphologic right ventricle or other complex single ventricle due to poor survival in the current era. As access to health care improves, re-evaluation of these outcomes is necessary to find the best strategy for the management of these patients.

Hepatosplanchnic circulatory dysfunction in acute hepatic infection: the case of dengue hemorrhagic fever.

The mechanism of shock in patients with dengue hemorrhagic fever (DHF) has not yet been fully understood. In this study, we investigated the possibility of splanchnic venous pooling as a contributor for circulatory dysfunction in these patients. Ultrasonographic studies of portal vein and inferior vena cava were done in 45 patients with serologically or PCR-confirmed diagnosis of dengue virus infection. The size of portal vein and inferior vena cava, mean blood flow velocity in the right portal vein, and modified portal vein congestion index were compared between patients with dengue fever (DF, n = 20), DHF without shock (n = 14), and dengue shock syndrome (DSS, n = 11) during the toxic stage, convalescent stage, and at follow-up. The portal vein was significantly more dilated in patients with shock (DSS) than DHF without shock and than DF during the toxic and convalescent stages (P < 0.05), but not at follow-up. The change in the size of inferior vena cava followed the opposite trend (not statistically significant). Portal vein blood flow velocity was lower and congestion index was higher in shock cases (DSS) than DHF without shock and than DF at toxic and convalescent stages (P < 0.01). The differences disappeared at follow-up. Hepatosplanchnic venous pooling and/or dysfunction occur and correlate with the severity of circulatory derangement and shock in patients with DHF. The cause(s) and significance of hepatosplanchnic circulatory dysfunction in DHF and possibly other viral hepatic diseases deserve further study.

Infective endocarditis in pediatric patients: an eighteen-year experience from King Chulalongkorn Memorial Hospital.

OBJECTIVES: To review the clinical features and outcome of the pediatric patients diagnosed with infective endocarditis (IE) at King Chulalongkorn Memorial Hospital over an 18-year period. MATERIAL AND METHOD: From January 1987 to December 2004, 57 pediatric patients; 28 females and 29 males, age ranged from 2 months to 15 years, mean of 8.64 +/- 3.82 years, classified as "definite" IE according to the Duke criteria were reviewed. RESULTS: Of the 57 patients, 42 patients (74%) had underlying congenital heart diseases, 7 patients (12%) had underlying rheumatic heart disease, 8 patients (14%) had previously normal heart. Nine patients had history of previously palliative or corrective surgery. Blood cultures were positive in 26 patients (46%). Streptococci and staphylococci were the most commonly isolated organisms. Two-dimensional echocardiography demonstrated vegetations in 56 patients (98%). The location of the vegetations was in the right heart in 30 patients (54%) and in the left heart in 26 patients (46%). Complications occurred in 28 patients (49%). Common complications included congestive heart failure, cerebral emboli, septic shock, and mycotic aneurysm. The overall mortality was 11% (6 patients). By statistical analysis, vegetations in the left heart had a higher incidence of complication than that in the right heart. The vegetation size of > or =10 mm had a significant higher incidence of embolic events. CONCLUSION: The clinical features and outcomes of the present study have a similar pattern as the earlier studies. The rates of complications and mortality are still high.

Pediatric heart surgery waiting time in Thailand and its effect on mortality: A cooperative study from Chulalongkorn, Children and Chiang Mai University hospitals.

BACKGROUND: Thai children who need cardiac surgery are often put on a waiting list. The waiting time and mortality during waiting have not been previously systematically studied. MATERIAL AND METHOD: A cooperative study was conducted at King Chulalongkorn Memorial hospital (Chula), Children hospital (CH), and Chiang Mai University hospital (CMU). The status of children who were referred for cardiac surgery at these hospitals in the year 1999-2000 (Chula and CMU) and the year 2000 (CH) were analyzed by Kaplan-Meier survival curve. The patients who lost to follow up and could not be contacted were censored at the time of last clinic visit. Log-Rank test was used to compare the survival curve and waiting time between three hospitals. RESULTS: The averaged annual referrals for cardiac surgery at the three hospitals were 846 cases (205 for Chula, 462 for CH and 179 for CMU). Mean age was 4.3 +/- 4.2 years and 51% were male. Follow up data were complete in 96.3%. Surgical procedures were correction of simple shunt lesions (ASD, VSD, AV canal) in 33.9%, close heart surgery (PDA ligation, coarctation repair, BT shunt) in 29.9%, total repair TOF in 19.6%, complex surgery in neonate and infants (arterial switch, TAPVR repair, Norwood procedure, truncus and interrupted aortic arch repair) in 4.2% and others in 12.3%. Median waiting time was 195 days and was significantly different between the three hospitals (p < 0.01). Mortality while waiting were approximately 5% at 2 years at Chula and CH, which was significantly higher than CMU (0%, p = 0.02). Further analysis revealed difference in age (lower Chula than CH than CMU) and types of surgery (more complex at Chula and CH) which may be the causes for difference in the mortality observed. CONCLUSION: Waiting time for cardiac surgery for children in Thailand is long and should be viewed as a problem in public health policy. Optimal waiting time at each hospital may not have to be the same, depending on the type and severity of the disease seen at each particular center Attempt should be made to solve this problem at the national level.

Concomitant dengue infection and Kawasaki disease in an infant: a case report and literature review.

A previously healthy 11-month-old girl presented with fever and rash for 6 days. Physical examination revealed an irritable infant with a high fever, injected conjunctivae, red cracked lips, posterior auricular lymphadenopathy, hepatomegaly, generalized erythematous maculopapular rash and petechial hemorrhage on trunk, face and extremities. Complete blood count showed atypical lymphocytosis and thrombocytopenia. Dengue infection was initially diagnosed. The persistent fever and clinical manifestations of Kawasaki disease (KD) were observed on day 8 with high erythrocyte sedimentation rate (56 mm/hr). Treatment of KD included intravenous immunoglobulin on day 9 of the illness. Desquamation of the fingers was found on day 15 of the illness. Ectasia of left coronary artery with small aneurysmal dilatation was detected by echocardiography on day 15 of the illness. Hemagglutination-inhibition test and enzyme-linked immunosorbent assay for dengue virus eventually showed a four-fold rising. According to the literature review, this is the second reported case of dengue infection concomitant with KD. The natural course of each disease may be modified and causes some difficulties in diagnosis and management.

Clinical presentation and course of long QT syndrome in Thai children.

BACKGROUND: Congenital long QT syndrome (LQTS) is a genetically transmitted cardiac channelopathy that can lead to lethal arrhythmia and sudden cardiac death in healthy young people. The clinical characteristics of LQTS are variable and depend on the subtype of long QT syndrome, which differ among populations. This single hospital-based case review study examined the clinical presentation of long QT syndrome and the outcomes of its treatment in 20 Thai children at King Chulalongkorn Memorial Hospital in Bangkok, Thailand. METHODS: Inpatient and outpatient records of children (aged 0-14 years) diagnosed with long QT syndrome from January 1, 1998, to September 30, 2013, were retrospectively reviewed. Presentation at diagnosis, treatments, and clinical courses were collected and analyzed. In the 20 subjects, total Schwartz scores totaled 5.2±0.9 points, and mean age at diagnosis was 7.6±4.4 years (range, 1 day-13.8 years). The patients were assigned to one of 3 groups based on trigger events: 50% of patients had events at rest (sleep or at rest), 35% experienced adrenergic-mediated events (e.g., stress, exercise, startle), and 15% were asymptomatic. Excluding the 3 patients who died at first presentation, 100% of patients received a beta blocker, and 47.1% were treated with an automatic implantable cardioverter-defibrillator (AICD). RESULTS: At follow-up (median=959 days; range, 1-4170 days), 4 patients (20%) were known to have died, 3 of whom died shortly after the diagnosis. Among patients who survived the initial event, 52.9% (9 of 17) experienced cardiac events (appropriate AICD shock, death, and/or syncope) during the follow-up period. The mean duration from diagnosis to cardiac event was 1420±759 days (range, 497-2499 days). CONCLUSIONS: All 20 patients with LQTS were mostly symptomatic at presentation. Owing to the geographical region and ethnicity of the Thai population, we conclude that the ratio of patients who develop cardiac symptoms at rest or during sleep might be higher than in other Asian countries.