Diffuse myocardial fibrosis following tetralogy of Fallot repair: a T1 mapping cardiac magnetic resonance study

Background

Adverse ventricular remodeling after tetralogy of Fallot (TOF) repair is associated with diffuse myocardial fibrosis.

Objective

The goal of this study was to measure post-contrast myocardial T1 in pediatric patients after TOF repair as surrogates of myocardial fibrosis.

Materials and methods

Children after TOF repair who underwent cardiac magnetic resonance imaging with T1 mapping using the modified look-locker inversion recovery (MOLLI) sequence were included. In addition to routine volumetric and flow data, we measured post-contrast T1 values of the basal interventricular septum, the left ventricular (LV) lateral wall, and the inferior and anterior walls of the right ventricle (RV). Results were compared to data from age-matched healthy controls.

Results

The scans of 18 children who had undergone TOF repair and 12 healthy children were included. Post-contrast T1 values of the left ventricular lateral wall (443?±?54 vs. 510?±?77 ms, P?=?0.0168) and of the right ventricular anterior wall (333?±?62 vs. 392?±?72 ms, P?=?0.0423) were significantly shorter in children with TOF repair than in controls, suggesting a higher degree of fibrosis. In children with TOF repair, but not in controls, post-contrast T1 values were shorter in the right ventricle than the left ventricle and shorter in the anterior wall of the right ventricle than in the inferior segments. In the TOF group, post-contrast T1 values of the RV anterior wall correlated with the RV end-systolic volume indexed to body surface area (r?=?0.54; r²?=?0.30; P?=?0.0238).

Conclusion

In children who underwent tetralogy of Fallot repair the myocardium of both ventricles appears to bear an abnormally high fibrosis burden.     

Hemodynamic effects of laparoscopic surgery in term and preterm infants with cardiac anomalies

Purpose

The aim of this study was to clarify whether cardiac anomalies are a reasonable contraindication to laparoscopic surgery in infants.

Methods

Between January 2004 and January 2013, 131 term and preterm infants with cardiac anomalies underwent laparoscopic surgery within the first 6 months of life. In this retrospective study type of cardiac anomaly, performed operative procedure, operative time, intraabdominal pressure and postoperative complications were evaluated.

Results

Over this 9-year period, 80 preterm and 51 term infants underwent different complex and even long-lasting laparoscopic procedures. Median operative time was 67 min with a median intraabdominal pressure of 13 mmHg. Cardiac anomalies ranged from persistent foramen ovale, atrium septal defect to ventricular septal defect and tetralogy of Fallot. In the postoperative course hemodynamic impairment was noted in three infants (2.3 %). Only one of them presented cardiorespiratory instability.

Conclusion

In this study different laparoscopic procedures could be performed in numerous infants with cardiac anomalies. Due to the inhomogeneity of this group of patients and individual combinations of heart defects with or without hemodynamic relevance, preoperative evaluation by a firm pediatric cardiologist is crucial. Prospective studies are necessary to further clarify the use of laparoscopic surgery in this distinct group of patients.     

Surgical outcome after complete repair of tetralogy of Fallot with absent pulmonary valve: comparison between bovine jugular vein-valved conduit and monocusp-valve patch

Background

The prognosis of tetralogy of Fallot with absent pulmonary valve (TOF/APV) without operation is poor. We evaluated the surgical outcome of TOF/APV in a single center.

Methods

Twenty-two TOF/APV patients underwent complete surgical correction in our hospital. Right ventricular outflow tract reconstruction was performed using bovine jugular vein (BJV)-valved conduit implantation (n?=?10), homograft-valved conduit implantation (n?=?2), or monocusp-valve patch (n?=?10). Health-related quality of life (QOL) was evaluated during follow-up.

Results

The overall survival at 5 and 10 years was 86.4?±?7.3% (confidence interval 69.4–97.2%). The survival rates were significantly different between patients with and without bronchial stenosis (40 and 100%, P?=?0.0003, log-rank test). The survival of patients aged?>?6 months was higher than those?≤?6 months (100 vs. 40%, P?=?0.0003, log-rank test). Patients with BJV-valved conduits had higher systolic gradients from the right ventricle to the pulmonary artery (RV–PA) compared to those with monocusp-valve patches. BJV-valved conduit implantation was a risk factor for post-operative pulmonary-valve stenosis. The QOL score for patients with BJV-valved conduits was lower than those with monocusp-valve patches (P?<?0.05). No reoperation was performed during follow-up.

Conclusions

Bronchial stenosis and lower age (≤?6 months) were the main factors influencing post-operative survival. The use of a BJV-valved conduit was a main reason for RV–PA restenosis; thus, the use of a BJV-valved conduit may increase the need for repeat intervention and decrease the post-operative quality of life.

     

Cardiac Contractility Modulation in a Model of Repaired Tetralogy of Fallot: A Sheep Model

The onset of right ventricular dysfunction in patients presenting with congenital heart disease is associated with a dismal long-term outcome and often represents a therapeutic dead end. Our study had several objectives: (1) to analyse the anatomical, functional, histological and cellular characteristics of an animal model of repaired tetralogy of Fallot with right ventricular dysfunction (2) to test the new electrical treatment known as cardiac contractility modulation in this animal model. Seven sheep underwent a first surgery at the age of three weeks aiming to mimic the characteristics of a repaired tetralogy of Fallot. Five controls were sham-operated. Experimental studies were performed 12 months after the initial operation. The hemodynamic, echocardiographic, and mitochondrial function studies were carried out before and after cardiac contractility modulation in closed- and open-chest conditions. In this animal model of right ventricular dysfunction, short-term cardiac contractility modulation was associated with a significant improvement in (a) right ventricular function, as evidenced by a significant increase in right ventricular dP/dt (p < 0.05) (b) left ventricular function evidenced by the increase in left ventricular dP/dt max (p < 0.05) (c) in mitochondrial function (p < 0.05). In this animal model of chronic right ventricular dysfunction, cardiac contractility modulation significantly improved acute cardiac hemodynamic and mitochondrial functions of both ventricles and may represent a promising option in patients with right heart failure.     

Agenesis of the internal carotid artery: associated malformations including a high rate of aortic and cardiac malformations

Background

Agenesis of the internal carotid artery (ICA) is a rare congenital anomaly occurring in less than 0.01% of the population, often incidentally discovered in pediatric populations. We recognized a high incidence of additional congenital malformations in children with ICA agenesis.

Objective

Our study reports nine cases of ICA agenesis and co-existent malformations and discusses implications of the association.

Materials and methods

We conducted a retrospective chart review of nine children evaluated at our institution with imaging findings of ICA agenesis.

Results

Seven children (78%) had congenital aortic or cardiac anomalies including coarctation (4), hypoplastic left heart (1), tetralogy of Fallot (1), and muscular ventricular septal defect (VSD) (1). Four children were diagnosed with an inherited disorder: Alagille syndrome (1), PHACE syndrome (1), VACTERL association (1), and methylenetetrahydrofolate reductase (MTHFR) gene variant (1). Additional congenital anomalies are also described.

Conclusion

In the setting of ICA agenesis, we report a robust association with congenital aortic and cardiac anomalies, as well as a broad spectrum of additional anatomical abnormalities that can occur in the setting of known genetic syndromes or as isolated findings. Knowledge of the natural history of ICA agenesis and associated anomalies will guide optimal care for these children.     

Evaluation of carotid intima-media thickness, a marker of subclinical atherosclerosis, in children with cerebral palsy

Background

Respiratory and cardiovascular diseases are the most common causes of death in children with cerebral palsy.

Objective

To evaluate sonographic carotid intima-media thickness, an early marker of atherosclerosis, in children with cerebral palsy and in healthy controls.

Materials and methods

One hundred children with cerebral palsy (65 boys), mean age 6.2 (SD, 2.1) years, and 35 age-matched and sex-matched healthy controls were included. Common carotid artery intima-media thickness was measured sonographically. Differences between patients and controls were evaluated with an independent samples t-test.

Results

Age, sex distribution and levels of serum lipids were comparable between patients and controls. Average, right and left carotid artery intima-media were thicker in patients compared with controls (mean ± SD, 0.61?±?0.13 mm vs 0.40?±?0.03 mm; 0.61?±?0.14 mm vs 0.40?±?0.03 mm; 0.61?±?0.13 mm vs 0.40?±?0.03 mm, respectively; all P?Conclusion Carotid intima-media is sonographically thicker in children with cerebral palsy compared with healthy controls, which may express an increased risk of atherosclerotic diseases.     

Model-based iterative reconstruction in pediatric chest CT: assessment of image quality in a prospective study of children with cystic fibrosis

Background

The potential effects of ionizing radiation are of particular concern in children. The model-based iterative reconstruction VEO^TM is a technique commercialized to improve image quality and reduce noise compared with the filtered back-projection (FBP) method.

Objective

To evaluate the potential of VEO^TM on diagnostic image quality and dose reduction in pediatric chest CT examinations.

Materials and methods

Twenty children (mean 11.4 years) with cystic fibrosis underwent either a standard CT or a moderately reduced-dose CT plus a minimum-dose CT performed at 100 kVp. Reduced-dose CT examinations consisted of two consecutive acquisitions: one moderately reduced-dose CT with increased noise index (NI = 70) and one minimum-dose CT at CTDI_vol 0.14 mGy. Standard CTs were reconstructed using the FBP method while low-dose CTs were reconstructed using FBP and VEO. Two senior radiologists evaluated diagnostic image quality independently by scoring anatomical structures using a four-point scale (1 = excellent, 2 = clear, 3 = diminished, 4 = non-diagnostic). Standard deviation (SD) and signal-to-noise ratio (SNR) were also computed.

Results

At moderately reduced doses, VEO images had significantly lower SD (P?<?0.001) and higher SNR (P?<?0.05) in comparison to filtered back-projection images. Further improvements were obtained at minimum-dose CT. The best diagnostic image quality was obtained with VEO at minimum-dose CT for the small structures (subpleural vessels and lung fissures) (P?<?0.001). The potential for dose reduction was dependent on the diagnostic task because of the modification of the image texture produced by this reconstruction.

Conclusions

At minimum-dose CT, VEO enables important dose reduction depending on the clinical indication and makes visible certain small structures that were not perceptible with filtered back-projection.     

Prevalence and Pattern of Congenital Heart Disease in Uttarakhand, India

Objective

To find the prevalence and pattern of congenital heart disease (CHD) at a tertiary care hospital in Uttarakhand, India.

Methods

A thorough history and clinical examination was done for all the live births and children up to 18 y of age who presented to the institute over a period of 3 y from July 2008 through June 2011. Those suspected of having a CHD, were further evaluated with ECG, chest radiography and the diagnosis was confirmed by color Doppler echocardiography.

Results

Of the 36541 children examined, 312 were identified as having congenital heart defects, thus giving a prevalence of 8.54 per 1000 children attending hospital. Only one fifth cases were diagnosed in neonatal period and the diagnosis was delayed beyond infancy in more than half of the cases. Ventricular septal defect (30.45%), atrial septal defect (17.63%), patent ductus arteriosus (9.62%), pulmonary stenosis (6.41%), tetralogy of Fallot (5.45%) and transposition of great arteries (5.13%) were the commonest defects observed. Only 9.29% of children with CHD underwent definitive treatment.

Conclusion

The prevalence of CHD at a tertiary referral hospital in Uttarakhand India is 8.54 per 1000 children. VSD and TOF are the most common acyanotic and cyanotic congenital heart defects respectively.     

Clinical and Echocardiographic Outcome in Patients Receiving Carvedilol for Treatment of Dilated Cardiomyopathy

Objective

To determine outcome of children receiving carvedilol in addition to other standard drug therapy for treatment of dilated cardiomyopathy.

Methods

Children receiving carvedilol for treatment of dilated cardiomyopathy with moderate to severe ventricular dysfunction were included into the study. Data on history, clinical examination and investigations were obtained and detailed echocardiography findings were recorded for the initial and all subsequent visits.

Results

Thirty-three children, mean age 26?±?30 mo (range 7 mo to 138 mo) were enrolled. Carvedilol was initiated at a mean dose of 0.14?±?0.03 mg/kg/d and the maintenance dose was 0.46?±?0.14 mg/kg/d. At a follow up of 6–90 mo (mean of 28?±?23 mo), functional class using Ross classification for pediatric heart failure improved from 2.7 to 1.3. The left ventricular ejection fraction rose from a basal value of 22 %?±?7 % (10–40 %) to 42 %?±?15 % (15–65 %) (p?<?0.0001). Similarly, left ventricular fractional shortening increased significantly from 16?±?6 % (8–34 %) to 21?±?7 % (10–44 %) (p?<?0.0001). One patient deteriorated and died of refractory heart failure. Carvedilol was discontinued in two more patients temporarily due to bronchospasm during respiratory infection.

Conclusions

The present study suggests that improvement in ventricular function and clinical symptoms is seen on oral carvedilol added to standard drug therapy in pediatric patients with dilated cardiomyopathy and moderate to severe ventricular dysfunction. The drug is well tolerated with minimal side effects but close monitoring is required as it may worsen heart failure and bronchospasm.     

Strain measurement by cardiovascular magnetic resonance in pediatric cancer survivors: validation of feature tracking against harmonic phase imaging

Background

Left ventricular strain may be a more sensitive marker of left ventricular dysfunction than ejection fraction in pediatric cancer survivors after anthracycline therapy, but there is limited validation of strain measurement by feature tracking on cardiovascular magnetic resonance (MR) images.

Objective

To compare left ventricular circumferential and radial strain by feature tracking vs. harmonic phase imaging analysis (HARP) in pediatric cancer survivors.

Materials and methods

Twenty-six patients (20.2?±?5.6 years old) underwent cardiovascular MR at least 5 years after completing anthracycline therapy. Circumferential and radial strain were measured at the base, midventricle and apex from short-axis myocardial tagged images by HARP, and from steady-state free precession images by feature tracking.

Results

Left ventricular ejection fraction more closely correlated with global circumferential strain by feature tracking (r?=??0.63, P?=?0.0005) than by HARP (r?=??0.39, P?=?0.05). Midventricular circumferential strain did not significantly differ by feature tracking or HARP (?20.8?±?3.4 vs. ?19.5?±?2.5, P?=?0.07), with acceptable limits of agreement. Midventricular circumferential strain by feature tracking strongly correlated with global circumferential strain by feature tracking (r?=?0.87, P?HARP, particularly at higher values of radial strain. Intraobserver and interobserver reproducibility was excellent for feature tracking circumferential strain, but reproducibility was poor for feature tracking radial strain.

Conclusion

Midventricular circumferential strain by feature tracking is a reliable and reproducible measure of myocardial deformation in patients status post anthracycline therapy, while radial strain measurements are unreliable. Further studies are necessary to evaluate potential relation to long-term outcomes.     

Septal Course of Left Anterior Descending Artery From the Right Aortic Sinus in Tetralogy of Fallot: A Benign Anomaly and Important Lessons Learned

The proximal course of an anomalously arising coronary artery is a decisive factor in the surgical approach for tetralogy of Fallot (TOF). Studies have shown that echocardiography provides a good anatomic definition of the ostium and proximal epicardial course of coronary arteries [1, 2]. This report describes a case of TOF with an atrioventricular canal defect whose preoperative echocardiography showed abnormal origin of the left anterior descending artery (LAD) from right aortic sinus, which was interpreted as crossing the right ventricular outflow tract. Perioperative inspection did not show any abnormal vessel crossing the outflow, and corrective surgery was performed. At the echocardiographic evaluation after surgery, it was noted that the abnormal LAD arising from right aortic sinus was taking a septal course in relation to the posterior aspect of the pulmonary annulus. It is important to recognize this anomalous course because it is benign with no surgical implications.     

The Efficacy of Cardiac Findings in Assessing the Outcome in Preterms with Bronchopulmonary Dysplasia

Objectives

To evaluate if cardiac dysfunctions are important in assessing the outcome in newborns with Bronchopulmonary Dysplasia (BPD), by evaluating cardiac functions with N-terminal prohormone of brain natriüretic peptide (NT-proBNP) levels, M-mode and tissue doppler echocardiography at 6–12 mo of age.

Methods

Twenty eight patients were retrospectively classified as mild, moderate and severe according to the diagnostic criterias for BPD. All cases were assessed with standard M-mode, tissue doppler echocardiography and NT-proBNP levels. Control group consisted of 28 healthy infants, having similar postnatal ages as patients and were assessed with standard M-mode and tissue doppler echocardiography.

Results

The age of patients with BPD was 9.8?±?2.3 mo and control group was 9.5?±?2.6 mo. There was no significant difference between the postnatal ages of two groups (p?>?0.05). Neither pulmonary hypertension nor pulmonary/tricuspid regurgitation was detected. The M-mode echocardiography measurements did not differ between patients and control group (p?>?0.05). Tissue doppler echocardiography, tricuspid valve medial segment early diastolic myocardial relaxation velocity (TME’) measurements of patients were found significantly lower, peak transtricuspid filling velocity in the early diastole (TE)/TME’ ratios and isovolumetric relaxation time (IVRT) measurements were found significantly higher than control group (p?<?0.05). Tricuspid E, TE/TLE’ (Tricuspid valve lateral segment early diastolic myocardial relaxation velocity), TE/RVLE’(Right ventricular lateral segment early diastolic myocardial relaxation velocity), TE/TME’ levels were also found as significantly abnormal in patients with severe BPD. A significant correlation was found between right ventricular diastolic disfunctions and severity of BPD (p?<?0.05). No statistically significant difference was found between NT-proBNP levels, BPD stages and tissue doppler echocardiography measurements (p?>?0.05).

Conclusions

This is the first study evaluating cardiac findings in patients with BPD by tissue doppler echocardiography and NT-proBNP at the same time. On the basis of cardiac evaluations, tissue doppler echocardiography measurements were found as significant and specific for the early assessment of right ventricular diastolic disfunctions.     

Prognostic value of gradient echo T2* sequences for brain MR imaging in preterm infants

Background

Gradient echo T2*-W sequences are more sensitive than T2-W spin-echo sequences for detecting hemorrhages in the brain.

Objective

The aim of this study is to correlate presence of hemosiderin deposits in the brain of very preterm infants (gestational age <32 weeks) detected by T2*-W gradient echo MRI to white matter injury and neurodevelopmental outcome at 2 years.

Materials and methods

In 101 preterm infants, presence and location of hemosiderin were assessed on T2*-W gradient echo MRI performed around term-equivalent age (range: 40–60 weeks). White matter injury was defined as the presence of >6 non-hemorrhagic punctate white matter lesions (PWML), cysts and/or ventricular dilatation. Six infants with post-hemorrhagic ventricular dilatation detected by US in the neonatal period were excluded. Infants were seen for follow-up at 2 years. Univariate and regression analysis assessed the relation between presence and location of hemosiderin, white matter injury and neurodevelopmental outcome.

Results

In 38/95 (40%) of the infants, hemosiderin was detected. Twenty percent (19/95) of the infants were lost to follow-up. There was a correlation between hemosiderin in the ventricular wall with >6 PWML (P?<?0.001) and cysts (P?<?0.001) at term-equivalent age, and with a lower psychomotor development index (PDI) (P=0.02) at 2 years. After correcting for known confounders (gestational age, gender, intrauterine growth retardation and white matter injury), the correlation with PDI was no longer significant.

Conclusion

The clinical importance of detecting small hemosiderin deposits is limited as there is no independent association with neurodevelopmental outcome.     

The impact of hydration on renal measurements and on cortical echogenicity in children

Background

Kidney ultrasonography is frequently performed in children; to date there are no specific guidelines regarding hydration before the examination.

Objective

Because certain renal measurements can be indicative of pathology, we used sequential US images to investigate the effect of standardised hydration in children relative to renal size, echogenicity and pelvic expansion.

Materials and methods

Thirty-one children (7 years to 14 years old) underwent US examinations before hydration (T0) and at 30 min (T30) and 60 min (T60) after ingesting water. We measured bladder volume, inter-polar kidney size, renal volume and anterior-posterior diameter of the pelvis. Cortical echogenicity was compared to that of the liver and spleen.

Results

On the right side the increase in average inter-polar renal size was 2.5% at T30 and 2.6% at T60 (P?<?0.05) compared to T0. On the left the increase was 3.8% at T30 and 4.3% at T60 (P?<?0.05). Volume expansion for the right kidney was 16.8% at T30 and 14.0% at T60 (P?<?0.05). On the left it was 12.9% at T30 and 10.2% at T60 (P?<?0.05). Cortical echogenicity progressed from hypoechogenicity to isoechogenicity (P?<?0.05). The percentage of expanded pelves increased with hydration (T0: 3.2%, T30: 9.7%, T60: 22.6%).

Conclusion

Oral hydration influences renal length, volume and echogenicity in children. Hydration results in expansion of the pelvis. We recommend standardisation of hydration before US examination.     

Usefulness of Pulsed Wave Tissue Doppler Imaging in Assessment of Left Ventricular Functions in Children with Beta-Thalassemia Major

Objective

To evaluate the changes in the LV systolic and diastolic function in children with beta-thalassemia major (β-TM) using pulsed wave tissue doppler (TD) echocardiography.

Methods

Clinical, conventional echo doppler and pulsed wave tissue doppler imaging parameters were compared in 40 beta-thalassemia major patients (mean age, 6.52?±?3.5 y) and 25 age and sex matched normal subjects (mean age, 6.5?±?2.7 y).

Results

There were no significant statistical differences between mean fractional shortening (FS) and ejection fraction (EF) of left ventricle (LV) of the patients and control group. Children with beta-thalassemia had significantly lower E′ wave velocities measured at the left ventricular septal annulus (8.1?±?3.3 vs. 13?±?2.5, P?<?0.001), lateral margin of the mitral annulus (9.1?±?5.4 vs. 13.3?±?2.5, P?<?0.001) and lateral margin of the tricuspid annulus (9.3?±?3.9 vs. 13.3?±?2.5, P?<?0.001) when compared to the control group. Furthermore children with beta-thalassemia had significantly lower E′/A′ wave ratio at the left ventricular septal annulus (0.76?±?0.34 vs. 1.36?±?0.23), lateral margin of the mitral annulus (0.83?±?0.17 vs. 1.28?±?0.22), and lateral margin of the tricuspid annulus ((0.90?±?0.27 vs. 1.26?±?0.23, (P?<?0.05) when compared to the control group.

Conclusions

This study showed that patients with beta-thalassemia major and normal conventional echo doppler parameters had statistically significant changes detected by pulsed wave tissue doppler imaging.     

Estimation of radiation dose and risk to children undergoing cardiac catheterization for the treatment of a congenital heart disease using Monte Carlo simulations

Background

Children diagnosed with congenital heart disease often undergo cardiac catheterization for their treatment, which involves the use of ionizing radiation and therefore a risk of radiation-induced cancer.

Objective

The purpose of this study was to calculate the effective and equivalent organ doses (H_T) in those children and estimate the risk of exposure-induced death.

Materials and methods

Fifty-three children were divided into three groups: atrial septal defect (ASD), ventricular septal defect (VSD) and patent ductus arteriosus (PDA). In all procedures, the exposure conditions and the dose-area product meters readings were recorded for each individual acquisition. Monte Carlo simulations were run using the PCXMC 2.0 code and mathematical phantoms simulating a child's anatomy. The H_T values to all irradiated organs and the resulting E and risk of exposure-induced death values were calculated.

Results

The average dose-area product values were, respectively, 40?±?12 Gy·cm² for the ASD, 17.5?±?0.7 Gy·cm² for the VSD and 9.5?±?1 Gy·cm² for the PDA group. The average E values were 40?±?12, 22?±?2.5 and 17?±?3.6 mSv for ASD, VSD and PDA groups, respectively. The respective estimated risk of exposure-induced death values per procedure were 0.109, 0.106 and 0.067%.

Conclusion

Cardiac catheterizations in children involve a considerable risk for radiation-induced cancer that has to be further reduced.     

Reference ranges of kidney dimensions in term newborns: sonographic measurements

Background

Ultrasonographic measurement of kidney dimensions is important in evaluation of renal disease during the neonatal period, when renal abnormalities are common and renal size rapidly changes with age.

Objective

To determine the reference ranges of kidney dimensions in newborns and to provide a reference chart for daily practice.

Materials and methods

In this prospective study, kidney dimensions were evaluated in 385 healthy newborns with a gestational age ≥37 weeks. Each neonate seen at an obstetrics clinic and neonatal intensive care unit was examined with sonography within the first week of life. Relationships of all dimensions with gender, gestational age, height and weight were statistically analyzed.

Results

All dimensions of the kidneys were smaller in girls than in boys (P?P?P?P?Conclusion The reference values of kidney lengths and diagrams from this study may be useful in the sonographic evaluation of kidneys in newborns.     

Prevalence and Predictors of Decreased Glomerular Filtration Rate in Tibetan Children with Congenital Heart Disease

Objectives

To assess the prevalence of decreased glomerular filtration rate (GFR) in Tibetan children with congenital heart disease (CHD) and its associated risk factors.

Methods

A total of 207 Tibetan children attending authors’ center for treatment of CHD from May 2012 through November 2012, were included in the study. GFR was estimated with the Schwartz formula (eGFR).

Results

The mean eGFR was 104.3?±?16.6 mL/min/1.73 m², and decreased in 21 children (10.1 %). In the cyanotic category, eGFR was decreased only in severely cyanotic individuals. In the acyanotic category with left ventricular overload, children with decreased eGFR were younger, more commonly lived in areas above 4,700 m, and had higher left ventricular internal dimensions indexed by body surface areas (LVID/BSA) (53.8?±?6.9 vs. 40.1?±?6.8 mm/m², P?P?P?2 (sensitivity: 75.0 %, specificity: 93.9 %). In the remaining category, decreased eGFR was only observed in those living above 4,700 m.

Conclusions

One tenth of Tibetan children with CHD had decreased eGFR. The risk factors included severe cyanosis, younger age, living above 4,700 m and higher LVID/BSA.     

Increasing the accuracy of lung perfusion scintigraphy in children with bidirectional Glenn circulation

Background

In children who have undergone a bidirectional Glenn procedure without antegrade or additional pulmonary blood flow, we have often noted a discrepancy between apparent lung perfusion on scintigraphy and superior vena cava angiography when evaluating right and left pulmonary blood flow. We found a tendency for radionuclide, tracer 99mTc-MAA, when administered through a single upper extremity vein, to preferentially accumulate in the ipsilateral lung.

Objective

In the present study, we examined whether the ratio of right-to-left pulmonary flow varied when 99mTc-MAA was administered via either the right upper or the left upper extremity vein.

Materials and methods

We studied six children (median age 1.3?±?0.23 years) who underwent a bidirectional Glenn before total cavopulmonary connection. Five children who underwent biventricular repair served as a control. Perfusion scintigraphy using 99mTc-labeled macroaggregated albumin (99mTc-MAA) was performed in all children. First, we injected radionuclide via the right upper extremity and calculated the pulmonary accumulation in both lungs (R-image). Second, we injected the same dose of radionuclide via the left upper extremity and calculated the pulmonary accumulation (B-image), which represented the resulting administration via both upper extremities. The lung accumulation that resulted from radionuclide administration via the left upper extremity (L-image) was determined by subtracting the R-image from the B-image. We evaluated the right-to-total pulmonary blood flow ratio (radionuclide accumulation in right lung / radionuclide accumulation in both lungs) in the R-, L- and B-images.

Results

The right-to-total pulmonary blood flow ratios in the R-, L- and B-images were 815?±?15.3%, 39.8?±?11.7% and 61.3?±?11.8%, respectively, and there were significant differences among the three images (P?<?0.01). On the other hand, in the control group, the right-to-total pulmonary blood flow ratios in the R-, L- and B-images were 59.3?±?22.4%, 57.8?±?26.4% and 58.8?±?23.7%, respectively, and there was no significant difference.

Conclusion

In children with bidirectional Glenn circulation without antegrade or additional pulmonary blood flow, the venous blood of each arm tends to flow into the ipsilateral lung. The administration of radionuclide via both arms is important for accurate evaluation of lung perfusion scintigraphy in children who have undergone a bidirectional Glenn procedure.     

Effect of Pseudoephedrine on Cardiac Rhythm of Children with Rhinitis

Objective

To investigate the effect of pseudoephedrine on heart rhythm of children with rhinitis.

Methods

The study included 25 children diagnosed with rhinitis from March 2009 through February 2010 in the Department of Pediatrics. Holter records were obtained for 24 h before and at the fourth day of pseudoephedrine treatments.

Results

Study group consisted of 18 girls (72%) and 7 boys (28%) with a mean age of 8.7?±?3.4 (4–17.9 years). Common complaints of the patients were rhinorrhea (100%), cough (68%) fatigue (48%), sore throat (36%), and headache (28%). Of the 25 patients whose Holter recordings were evaluated, rare supraventricular extrasystoles were observed in one prior to the administration of pseudoephedrine, which were not repeated on this patient’s follow-up recording on day four. There were two ventricular extrasystoles in the day four Holter recording of another patient. None of the patients complained of chest pain or palpitation. There were no observations of supraventricular tachycardia, ventricular tachycardia or ventricular fibrillation. No statistical differences could be found (p?>?0.05) in the values before treatment and those on day four of treatment of either the time-dependent Heart rate variability (HRV) parameters SDNN, SDNN index, SDANN and RMSSD, or the frequency-dependent parameters (TP, HF, LF). No statistical difference could be determined between heart rate values of the patients before treatment and those on day four of treatment (p?>?0.05).

Conclusions

This study has established that therapeutic doses of pseudoephedrine do not cause an additional dysrhythmia risk for children with no health problem except rhinitis.