Carotid intima-media thickness and arterial stiffness in pediatric systemic lupus erythematosus.

Objectives The carotid intima-media thickness (CIMT) and carotid arterial stiffness index (CASI) act as the surrogate markers of atherosclerosis. We aim to assess CIMT and CASI in pediatric systemic lupus erythematosus (SLE). Methods Patients ≤ 20 years old fulfilling diagnostic criteria for SLE were enrolled. Patients with active smoking, coronary heart disease, cerebrovascular disease, arterial thrombosis, family history of hypercholesterolemia, chronic liver disease, or other chronic severe diseases were excluded. The patients were categorized into four groups: active SLE, age- and sex-matched control (control A), inactive SLE, and age- and sex-matched control (control I), according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). All subjects underwent ultrasound of carotid arteries to evaluate CIMT and CASI. Results One hundred and two SLE patients (26 active and 76 inactive) and one hundred and three healthy controls (26 control A and 77 control I) were enrolled. The median CIMT in all groups were not significantly different (0.43, 0.41-0.44; 0.43, 0.41-0.44; 0.42, 0.41-0.43; and 0.42, 0.41-0.43 mm, respectively).The CASI in active SLE (13.5, 11.4-17.3) was significantly higher than in control A (8.2, 7.2-9.2) ( p < 0.0001), whereas CASI in inactive SLE (12.7, 10.9-15.7) was significantly higher than in control I (8.9, 7.6-9.8). However, the CASI in active and inactive SLE was not significantly different. Conclusions The higher CASI in active and inactive pediatric SLE, implying functional change of carotid arteries, may be early evidence of increased atherosclerosis in pediatric SLE. This functional dysfunction has been found both in inactive and active SLE.

Appropriate length and position of the central venous catheter insertion via right internal jugular vein in children.

DESIGN: Observational study. SETTING: Tertiary University Hospital. PARTICIPANTS/PATIENTS: Patients, aged 6 days to 19 years, who underwent contrast enhancement computerized tomography imaging of the thorax (CT-thorax). MAIN OUTCOME MEASURES: We measured three lengths consisting of length A in axial plane at the level of the lower border of the 6th C-spine from the skin to RIJV at its mid lumen, length B and C in sagittal plane from the RIJV at the level of the lower of the 6th C-spine to the superior vena cava (SVC) at carina and from carina to SVC-right atrium junction, respectively. Lengths A plus B represented the length of CVC where the tip was expected in the SVC at carina (CVC(carina)). Lengths A plus B and C represented the length of CVC when the tip was expected in the SVC at SVC-right atrium junction (CVC(SVC-RA)). RESULTS: One hundred and sixty-five cases with mean age of 8.1 ± 4.7 years were reviewed. The CVC(SVC-RA) and CVC carina were significantly correlated with age and body surface area (BSA). Using multiple regression analysis, CVC(SVC-RA) (cm) was equal to 6.4 + 2.8[BSA (m2)] + 0.022[age (month)] and CVC(casina) (cm) equal to 4.9 +2.7[BSA (m2)] +0.013[age (month)] (Adjusted R-squared 0.7275, 0.7140). CONCLUSIONS: We recommended the appropriate CVC length via RIJV approach should be between these two calculated lengths and the CVC length in each age according to the BSA.

Echocardiographic parameters of patent ductus arteriosus in preterm infants.

OBJECTIVE: To analyze cardiovascular parameters by echocardiography in preterm infants with patent ductus arteriosus (PDA). SETTING: Tertiary-care pediatric university hospital. DESIGN: Cross-sectional, hospital-based study. PARTICIPANTS: 58 preterm infants, gestational age less than 33 weeks. MEASUREMENTS: A complete 2-dimension, M-mode, color doppler echocardiography was performed in each preterm infant at approximately 48 hours of life. RESULTS: Each preterm was categorized into hemodynamically significant PDA (hsPDA) (n=17, 29.3%), non-hemodynamically significant PDA (non-hsPDA) (n = 12, 20.7%), and no PDA (non-PDA) (n=29, 50%). Gestational age (29.4 ± 1.2 wk) and birth weight (1237 ± 358 g) of infants in hsPDA were significantly lower than those in non-PDA group (30.8 ± 1.3 wk, 1543 ± 361 g, P = 0.001), as compared to those in the non-hsPDA group (29.5 ± 2.3 wk, 1296 ± 462 g). Cardiovascular parameters including left atrium/aorta ratio, left atrium volume index, left ventricular dimensions and volumes, stroke volume, and cardiac output in hsPDA were significantly greater than those in non-hsPDA and nonPDA. LV systolic and diastolic functions were not significantly different in each group. LV global function in hsPDA (0.34 ± 0.13) was significantly lower than that in non-PDA (0.45 ± 0.13, P = 0.01). CONCLUSIONS: In preterm infants with hsPDA, there was a volume load of the left heart causing increased stroke volume and cardiac output. The hsPDA could be detected by echocardiography even in the first 48 hours. The left atrial volume index may be a better indicator of the volume load of the heart.

N-terminal probrain natriuretic peptide and patent ductus arteriosus in preterm infants.

OBJECTIVE: To determine whether plasma N-terminal probrain natriuretic peptide (NT-proBNP) in premature infants could identify hemodynamically significant patent ductus arteriosus (HsPDA) and to determine the correlation between serial plasma NT-proBNP and echocardiographic assessment of ductal shunting. STUDY DESIGN: An observational study involving 35 preterm infants who underwent echocardiographic assessment for PDA on day 2, 4 and 7 of life with simultaneous blood sampling for determination of NT-proBNP concentrations. HsPDA was diagnosed by left-to-right ductal shunt on color Doppler, measuring diameter >1.5 mm on two-dimensional echocardiography plus > or =2 clinical features of PDA. RESULT: Plasma NT-proNBP levels on day 2 in the HsPDA group (n=12) were significantly higher than in non-HsPDA group (n=23) with a median of 16,353 pg ml(-1) (interquartile range (IQR), 12,360-33,459; range, 10,316-104,998) vs 3914 pg ml(-1) (IQR, 2601-5782; range, 1535-19,516) (P<0.001), respectively. Eight infants (67%) in the HsPDA group responded to an initial course of indomethacin or ibuprofen and their NT-proBNP levels significantly decreased within 48 h after treatment compared with non-responders (P=0.007). NT-proBNP concentrations were significantly correlated with left atrial to aortic root ratio. A cut-off NT-proBNP on day 2 of 10,180 pg ml(-1) offered the best predictive values for HsPDA with a sensitivity of 100% and a specificity of 91%. CONCLUSION: Plasma NT-proBNP on day 2 was found as a sensitive marker for predicting HsPDA in preterm infants. Successful closure of PDA was also correspondent with the decline in plasma NT-proBNP.

Case report: Giant coronary aneurysm caused by Kawasaki disease: follow-up with echocardiography and multidetector CT angiography.

Giant coronary artery aneurysms caused by Kawasaki disease are not common; however, they are one of the most serious complications and can be fatal. Here, we describe a 5-year-old girl with Kawasaki disease who initially had normal coronary arteries. Despite intravenous immunoglobulin, she developed progressive giant coronary artery aneurysms. Echocardiography is a non-invasive tool for imaging the condition but it does have some limitations, whereas selective coronary angiography is the gold standard. However, multidetector CT may be a better non-invasive tool, alternate to invasive catheterized selective coronary angiography, in the long-term follow-up of patients with a giant coronary aneurysm.

N-terminal pro-brain natriuretic peptide as a marker in follow-up patients with tetralogy of Fallot after total correction.

Patients with tetralogy of Fallot (TOF) after total correction usually have residual pulmonary regurgitation resulting in right ventricular (RV) dilatation and dysfunction. This study was performed to evaluate N-terminal pro-brain natriuretic peptide (NT-proBNP) in predicting RV dilatation and RV dysfunction in TOF after total correction. Twenty-one patients with TOF after total correction (12 males and 9 females, 12.06 +/- 2.54 years old) underwent echocardiography, cardiac magnetic resonance imaging (MRI), and blood sampling for NT-proBNP. Mean time after total correction was 7.59 +/- 2.30 years. From cardiac MRI study, mean right ventricular end diastolic volume index (RVEDVi) was 148.36 +/- 64.50 ml/m2 and mean right ventricular ejection fraction (RVEF) was 35.50 +/- 10.50%. Right ventricular dilatation was considered if RVEDVi was >108 ml/m2 and RV dysfunction was considered if RVEF was <40%. A plasma NT-proBNP level of 115 pg/ml was identified by receiver operating characteristic analysis in predicting RV dilatation and/or dysfunction. At this value, the sensitivity and specificity for predicting RV dilatation, RV dysfunction, and both RV dilatation and dysfunction were 71 and 100%, 71 and 71%, and 83 and 78%, respectively. In conclusion, plasma NT-proBNP level may be helpful in follow-up patients. Plasma NT-proBNP levels >115 pg/ml can be used as a marker in the detection of RV dilatation and dysfunction.

Diagnosis of tetralogy of Fallot with anatomically corrected malposition of the great arteries and single coronary artery by multidetector CT.

We present a case of an 8-year-old boy diagnosed as a tetralogy of Fallot with anatomically corrected malposition of the great arteries by echocardiography and cardiac catheterization. Multidetector CT nicely elucidated the course of the single coronary artery from the right coronary cusp, which traverses across the right ventricular outflow tract.

Chromosome 22q11 deletions in patients with conotruncal heart defects.

We performed this study to determine the frequency of 22q11 deletions and associated phenotypic features and abnormalities in conotruncal heart defects. Sixty-one patients with conotruncal heart defects, including tetralogy of Fallot (TOF; n = 32), pulmonary atresia/ventricular septal defect (PAVSD; n = 12), double-outlet right ventricle (DORV; n = 5), transposition of the great arteries (TGA; n = 4 ), truncus arteriosus (TA; n = 4), subpulmonary ventricular septal defect (SPVSD; n = 3), and interrupted aortic arch (IAA; n = 1), were enrolled in this study and screened for 22q11 deletions by the fluorescence in situ hybridization technique. Phenotypic features and associated abnormalities, including submucosal cleft palate, abnormal facies, square nose, nasal voice, abnormal ears, long and slender fingers, delayed development, mental retardation, delayed growth, short stature, and hypocalcemia, were examined in these patients. Nine of 61 patients (14.8%) had 22q11 deletions, including 100% of IAA, 50% of TA, 33.3% of SPVSD, 33.3% of PAVSD, and 3.1% of TOF. Deletions were not detected in DORV and TGA. In all patients with 22q11 deletions, > or =1 phenotypic features or associated abnormalities were observed. A subgroup of patients with IAA, TA, SPVSD, and PAVSD associated with phenotypic features or abnormalities warrants evaluation for the presence of 22q11 deletions.

Role of amiodarone in the treatment of fetal supraventricular tachyarrhythmias and hydrops fetalis.

We report three consecutive hydropic fetuses with fetal tachyarrhythmias treated with amiodarone-two in combination with digoxin and one with digoxin, procainamide, and propranolol. Sinus rhythm was achieved in one case and ventricular rate control was achieved in two cases. All fetuses treated with amiodarone gradually improved. Observed side effects of amiodarone were a maternal rash in one mother and transient neonatal hypothyroidism in one infant. We conclude that amiodarone might be effective and safe for fetal tachyarrhythmias and impending hydrops. The small number of patients suggests that a multicenter cooperative approach is required in order to determine if this is correct.

Residual patent ductus arteriosus shunting after single Gianturco coil occluding.

Transcatheter occlusion with Gianturco coils has become the treatment of choice for small patent ductus arteriosus (PDA). Coil occlusion was attempted in 20 patients with ductus diameter less than 4 mm who did not require other cardiac surgery. Sixteen of 20 patients had successful implantation. The mean age was 4.2 years. Their mean weight was 14.1 +/- 5.9 kg. The mean ductus diameter was 2.21 +/- 0.91 mm (range 1-3.7 mm). Nine patients had complete occlusion but 7 had residual shunting immediately after the procedure. However, 4 patients had spontaneous resolution of residual shunts at 6 months after the procedure. The other 3 who had diameter of ductus greater than 3 mm still had significant residual shunt at 6 months and 1 year after the procedure. The second coil was successfully implanted in one of these 3 patients and the closure of PDA was accomplished. We concluded that the second coil should be implanted if the ductus diameter is greater than 3 mm and significant residual shunt is still demonstrated angiographically after the first coil implantation.

Factors affecting mortality in Ebstein's anomaly of the tricuspid valve.

Ebstein's anomaly of the tricuspid valve is a relatively uncommon congenital heart defect. Twenty-one patients (11 boys and 10 girls) with Ebstein's anomaly were reviewed regarding clinical characteristics and factors related to the outcome of this lesion. Their ages at presentation ranged from 1 day to 13 years (median = 11 months). Eleven patients presented in infancy period, 6 of them were neonates. Common clinical findings were systolic murmur (85.7%) and cyanosis (57.1%). Laboratory findings included cardiomegaly on chest X-rays (95.2%), right bundle branch block pattern (76.2%) and right atrial enlargement (61.9%) on electrocardiography. Diagnosis and grading of severity were established by echocardiography. Among 21 patients, 4 were lost during follow-up. Seventeen patients were followed for 3-72 months. Six patients (28.6%) required surgery, 5 of whom died following surgery. Two patients died during the medical follow-up. Factors affecting cardiac death were the younger age at presentation, onset of cyanosis in infancy period, associated PS or PA, the lower insertion of the septal leaflet of the tricuspid valve and the higher ratio of the combined area of right atrium and atrialized right ventricle to that of functional right ventricle and left heart chambers.

Balloon pulmonary valvuloplasty in children, one year follow-up experience at Ramathibodi Hospital.

UNLABELLED: Balloon valvuloplasty has become the treatment of choice in pulmonary valvular stenosis. The objective of this report is to review the experience of this procedure in children at the Department of Pediatrics, Ramathibodi Hospital in the past 4 years (1991-1994) with at least one year follow-up. During the study period, 19 children aged 2.1-14.3 years (mean 5.11 years) with the diagnosis of pulmonary valvular stenosis had successful pulmonary balloon valvuloplasty at this institution. The immediate peak systolic pressure gradient across the pulmonary valve by cardiac catheterization decreased from 92.05 +/- 46.92 to 34.26 +/- 25.30 mmHg, by Doppler from 78.58 +/- 26.55 to 34.83 +/- 15.60 mmHg. Peak pressure gradient across the pulmonary valve by Doppler of the last examinations, one to 4 years after the procedure (mean 1.9 years), was 23.05 +/- 9.40 mmHg in 17 patients. The mean ratio of balloon size and pulmonary valve ring was 1.06. One patient still has a residual gradient of 54 mmHg and two were lost to follow-up. No serious complication was encountered during the procedures. CONCLUSION: Balloon pulmonary valvuloplasty is effective in the management of children with valvular pulmonary stenosis. The intermediate term result is excellent.