Epidemiologic study of Kawasaki disease and cases resistant to IVIG therapy in Thailand.

The incidence of Kawasaki disease (KD) in Thailand has never been studied before. We reviewed the data from the National Registry of Thai Children who had KD between 1998-2002 to evaluate the incidence of KD and cases resistant to treatment with intravenous immunoglobulin (IVIG). Resistance to IVIG was defined as remaining febrile at least 48 hours after initial IVIG therapy. There were 710 KD patients in the registry. The incidence of KD was from 2.14 to 3.43 cases per 100,000 children aged 0-5 years. During the acute phase 15.6% of 435 patients were considered as resistant cases. Resistant cases of KD in Thai children are quite common (15.6%) even after IVIG treatment. We found that patients who had high white blood cell counts (> 16,500 cells/mm3) had a higher likelihood of being resistant.

Predicted outcome after repair of tetralogy of Fallot by postoperative pressure ratio between right and left ventricle.

BACKGROUND: The surgical management of tetralogy of Fallot (TOF) has continued to evolve and there are now generally excellent early and long-term results following complete repair. OBJECTIVE: To investigate the early results of the authors' current surgical management of TOF by assessing the perioperative and early to intermediate follow-up period. The authors paid particular attention to the post-operative ratio of right ventricular to left ventricular systolic pressure (RVSP/LVSP), focusing on the presence of low cardiac output, intensive care unit (ICU) stay, prolonged of inotropic support and ventilation support time. STUDY DESIGN: Retrospective study. MATERIAL AND METHOD: Between June 2002 and August 2004, 31 consecutive patients underwent complete repair of TOF. Their mean age was 7.7 +/- 5.1 years (range, 2.9 to 25.3). A previous palliative shunt had been performed in 14 (45.2%) patients. Twenty-three patients (74.2%) were in NYHA FC II. Mean hematocrit and oxygen saturation were 50.9 +/- 10.25% and 80.5 +/- 8.6%, respectively. Mean preoperative ratio of RVSP/LVSP was 1.1 +/- 0.15. The operative approach was transatrial/transpulmonary, and 17 (54.8%) patients required a transannular patch. An extracardiac valve conduit was necessary in 3 (9.7%) patients with pulmonary atresia. RESULTS: There were no operative or late deaths. Two cases were reoperated from cardiac tamponade. Mean postoperative ratio of RVSP/LVSP was 0.53 +/- 0.16. Median ICU and hospital stays were 2.2 and 11 days, respectively. Presence of low cardiac output and prolonged inotropic support were significantly (P < 0.05) related to a RVSP/LVSP ratio of more than 0.5. At median follow-up of 6 months, 29 (93.5%) patients were asymptomatic and all patients were free of significant residual lesion. CONCLUSION: The authors' early results in complete repair of TOF patients are acceptable with a low incidence of morbidity. A postoperative RVSP/LVSP ratio of more than 0.5 was significantly associated to adverse outcome. Late complications may, however, develop, and long term follow-up for early detection of any such complications is essential.

A case report of infant with left ventricular noncompaction.

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy resulting from abnormal arrest during endomyocardial embryogenesis. The authors present a 6-month-old infant with intractable cardiogenic shock and echocardiographic features of LVNC, characterized by excessively prominent ventricular trabeculation and deep intertrabecular recesses as the first case report in Thailand.

Early experience with pediatric cardiac intervention in Songklanagarind Hospital.

BACKGROUND: Nowadays, pediatric cardiac intervention is an effective optional treatment for congenital heart disease (CHD). Several cardiac centers have been established in different regions of Thailand and Songklanagarind Hospital is the newest of these university cardiac centers. OBJECTIVE: To report results and complications of transcatheter treatment for congenital cardiac defects in Songklanagarind Hospital. MATERIAL AND METHOD: The medical database was reviewed for the results and complications of different types of pediatric cardiac intervention from May, 2000 to December, 2003. RESULTS: There were 102 cases of pediatric cardiac intervention. Sixty-seven were patent ductus arteriosus (PDA), 16 were valvular pulmonary stenosis (VPS), 10 were cyanotic CHD which needed balloon atrial septostomy (BAS), 8 were abnormal aorto-pulmonary (AP) collaterals, and 1 was severe valvular aortic stenosis (VAS). Coil embolization was performed in 53 patients with PDA and 8 patients with AP-collateral vessels, 32 of PDAs (60.4%) and all AP-collateral vessels (100%) were completely obliterated within 24 hours. The Amplatzer duct occluder (ADO) was deployed in 14 PDAs with 100% completely obliteration within 24 hours. In those with VPS or VAS, percutaneous balloon valvuloplasty (PBV) was the treatment of choice. The mean peak to peak systolic pressure gradient in VPS was reduced from 62.8 +/- 33.3 mmHg to 33.33 +/- 33.33 mmHg and from 76 mmHg to 49 mmHg in VAS after the procedures. In BAS, the mean diameter of atrial communication increased from 3.0 +/- 0.7 mm to 5.9 +/- 0.4 mm. In coil embolization, 8 had distal PA embolization (15%), 1 had hemolysis (2%) and 1 had decreased dorsalis pedis pulse (2%). One (7%) of the ADO-implanted patients had a weak femoral pulse. Of the VPS cases, 1 died from intractable heart failure, and 1 developed hemiparesis, from which they completely recovered within 6 months. The patient with VAS had a femoral artery complication. CONCLUSION: Pediatric cardiac intervention in Songklanagarind Hospital has satisfactory results with an acceptable complication rate.

Dengue myocarditis.

We report a 13-year-old boy who developed bradycardia and hypotension a day after recovery from dengue hemorrhagic fever. His electrocardiogram, during the bradycardia, showed a junctional rhythm with a rate of 50 beats/minute. This is the first reported case of sinus node dysfunction following dengue infection.

Prevalence of idiopathic long QT syndrome in congenital sensori-neural hearing loss students of Songkhla School for the Deaf.

OBJECTIVES: To survey the prevalence of the long QT syndrome (LQTS), especially Jervell-Lange-Nielsen syndrome (JLNS), in Thai children (The first such study). BACKGROUND: LQTS is a rare inherited disease with a prevalence of 0.21 per cent in children with congenital deafness from other reports. These patients carry a high risk of recurrent syncope and fatal ventricular arrhythmia. STUDY DESIGN: Cross-sectional survey from January 2000 to August 2000. METHOD: A total of 276 children with congenital sensori-neural hearing loss were included. A questionnaire was employed and all children were examined by a pediatric cardiologist to rule out organic heart disease. EKGs were obtained and QTc intervals were blindly measured using standard methods in L2, V5 or any other leads with the longest QTc interval by three pediatric cardiologists. If QTc interval is prolonged, additional EKG (up to 3) were done to confirm the finding. Schwartz criteria was used to identify index cases with LQTS after repeated EKGs, and exercise stress tests. Also, echocardiography were done in patients suspected of having LQTS. RESULTS: A total of 14 children needed a third EKG and more work ups due to persistent long QTc interval after 2 consecutive EKG studies with QTc intervals ranged from 456 msec to 466 msec, and Schwartz score from 1.5 to 2.5. There were 6 twins and no triplets in the study. Finally, two subjects (not twins or siblings) had persistent prolonged QTc intervals after 3 EKG studies. After the exercise stress test, both still had a prolonged QTc interval, not corrected to the normal QTc interval even at the exercise peak. There was no cardiac abnormality either structurally or functionally from the 2D echocardiogram and Doppler color flow study. CONCLUSION: The possible prevalence of JLNS was 0.7 per cent (2/276). Both children were in the low-risk group for having LQTS.

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.