Decline of Systolic and Diastolic 2D Strain Rate During Follow-Up of HLHS Patients After Fontan Palliation

Accurate assessment of ventricular function is particularly important in children with hypoplastic left heart syndrome (HLHS) after completion of the total cavopulmonary connection (TCPC). For this purpose, two-dimensional speckle tracking (2DST) is a promising technique as it does not depend on the angle of insonation or the geometry of the ventricle. The objective of this study was to assess changes in systolic and diastolic right ventricular (RV) function within a 5-year follow-up period of HLHS patients after TCPC using conventional and 2DST echocardiography. RV fractional area change (RVFAC), tricuspid annular plane systolic excursion (TAPSE), E/A, E/e′ and 2DST parameters [global longitudinal peak systolic strain (GS) and strain rate (GSRs), global strain rate in early (GSRe) and late (GSRa) diastole] of 40 HLHS patients were compared at 1.6 and at 5.1 years after TCPC. RVFAC, E/A, E/e′ and GS did not change, whereas TAPSE (13.7 ± 3.2 vs. 10.5 ± 2.4 mm/m², p < 0.001), GSRs (?1.56 ± 0.28 vs. ?1.35 ± 0.31 1/s, p < 0.001), GSRe (2.22 ± 0.49 vs. 1.96 ± 0.44 1/s, p = 0.004) and GSRa (1.19 ± 0.39 vs. 0.92 ± 0.39 1/s, p < 0.001) decreased significantly. Systolic and diastolic RV function parameters of HLHS patients decreased from 1.6 to 5.1 years after TCPC in our patients. Changes in global strain rate parameters may be signaling early RV dysfunction that is not detectable by traditional echocardiography. Further study is needed to verify this and to determine whether these changes are clinically relevant.     

Early Ventricular Dysfunction After Anthracycline Chemotherapy in Children

The aim of this study was to determine the effect of induction dose of anthracycline chemotherapy on the biventricular function among children with acute hematological malignancies (AHM) using tissue Doppler imaging (TDI) and 2D speckle tracking echocardiography (2D-STE). Thirty pediatric patients with AHM and a mean age of 9.24 ± 4.14 years performed conventional echocardiography, TDI and 2D-STE. After induction chemotherapy, the RV showed mainly a diastolic alteration in its function manifested in significant reduction in the tricuspid TDI-derived E′ and E′/A′ ratio compared with the baseline (20.40 ± 3.81 vs. 17.47 ± 3.87 cm/s, p = 0.001, 1.29 ± 0.27 vs. 1.03 ± 0.37, p < 0.01, respectively), while the TDI-derived RV MPI and isovolumetric relaxation time of RV were significantly increased (0.32 ± 0.06 vs. 0.36 ± 0.08, p < 0.01, 24.73 ± 8.62 vs. 28.47 ± 11.51 ms, p < 0.05, respectively). The LV showed post-chemotherapy mainly an alteration in its longitudinal systolic function in the form of a reduction in MAPSE (13.61 ± 2.00 vs. 11.95 ± 1.75 mm; p < 0.001), TDI-derived systolic velocity of lateral mitral annulus (10.98 ± 2.34 vs. 10.03 ± 1.83 cm/s, p < 0.05), 2D-STE-derived global longitudinal strain (?21.58 ± 2.54 vs. ?19.18 ± 3.59 %, p = 0.001) and 2D-STE-derived global longitudinal strain rate (?1.76 ± 0.22 vs. 1.55 ± 0.29 1/s, p < 0.05), with preservation of LV diastolic function when compared to baseline. TDI and 2D-STE could be used for early detection of anthracycline-induced cardiotoxicity in the pediatric age group. Early after induction chemotherapy, the RV develops mainly diastolic dysfunction, while the LV showed a relative longitudinal systolic impairment.     

Right Heart Function of Fetuses and Infants with Large Ventricular Septal Defect: A Longitudinal Case–Control Study

The objective of this study was to detect the effect of a large ventricular septal defect (VSD) on right ventricular function before and after birth. All consecutive children with large VSD who were born in our hospital between January 2013–February 2016 and followed up throughout early infancy, and who lacked malformations or chromosomal abnormalities, were identified by a retrospective review of the medical records and included in this retrospective longitudinal case–control study (n = 30). Thirty normal control cases with an equivalent gestational age and gender served as controls. Tricuspid annular plane systolic excursion (TAPSE), right ventricle (RV) Tei index, and tricuspid E/E _m were measured in the fetal, neonatal (day 1–28), and infant (day 29–70) periods. In all periods, the VSD and control groups did not differ in TAPSE values, but VSD associated with higher Tei indices and tricuspid E/E _m values (in the fetal period: VSD group RV Tei was 0.48 ± 0.12 and E/E _m was 11.84 ± 1.53, control group RV Tei was 0.42 ± 0.16 and E/E _m was 10.16 ± 1.61; in neonatal period: VSD group RV Tei was 0.41 ± 0.17 and E/E _m was 12.21 ± 1.59, control group RV Tei was 0.30 ± 0.13 and E/E _m was 7.20 ± 1.28; in the infant period: VSD group RV Tei was 0.39 ± 0.09 and E/E _m was 11.89 ± 2.80, control group RV Tei was 0.28 ± 0.12 and E/E _m was 5.26 ± 1.90, all p < 0.05). In the fetal and neonatal periods, TAPSE correlated negatively with Tei index and tricuspid E/E _m in both groups. However, in the infant period, only the control group exhibited correlations between TAPSE and Tei index or tricuspid E/E _m. Tei index correlated positively with tricuspid E/E _m in both groups in all three periods. The VSD group had smaller correlation coefficients than the control group. Large VSD may already start to impair RV diastolic and global function before birth. This impairment continued and increased after birth. These changes did not associate with obvious RV longitudinal systolic function impairment. Large VSD mainly affected RV function by decreasing diastolic function and myocardial performance.     

Evaluation of Cardiovascular Changes in Children with BAVs

The objectives of this study were to investigate left ventricular (LV) function, aortic dilation, and atherosclerosis in children with mildly deteriorated isolated bicuspid aortic valve (BAV) function using echocardiographic studies and biochemical markers of atherosclerosis and to correlate results with normal children. Biochemical analyses indicating cardiovascular risk of atherosclerosis and vascular changes in the aorta in relation to BAV were performed in 41 children aged 5–15 years old with isolated BAV and in 25 children with tricuspid aortic valves. Evaluations of aortic valve structures and functions; examinations of the LV M-mode and ascending aorta Doppler; and measurements of the LV Tei index (MPI), propagation velocity, ascending aorta at four levels, and carotid intima–media thickness (CIMT) were performed. There were no statistically significant differences in CIMTs, plasma matrix metalloproteinase-9, tissue metalloproteinase inhibitor-1 levels, or other biochemical parameters indicating cardiovascular risk or atherosclerosis between study and control groups. Deterioration of LV function, which could not be seen with M-mode echocardiography, was evident by MPI. MPI values in the study versus control groups were 0.46 ± 0.080 versus 0.40 ± 0.086 (p < 0.05). Diameters of the aorta in the study and control groups were 19.7 ± 4.7 and 17.2 ± 2.8 mm (p < 0.05) at the sinotubular junction level and 20.6 (14.4–40.5) and 18.3 (12.4–24) mm at the ascending aorta level (p < 0.05). Increased aortic valve insufficiency was related to increased aortic diameter. No sign of atherosclerosis was detected in children with BAV. Deterioration of LV function was seen using MPI, and aortic dilation was related to the severity of aortic valve insufficiency.     

Right Ventricular Apical Flattening as an Echocardiographic Screening Tool for Right Ventricular Enlargement

Right ventricular dilation is a common complication after tetralogy of Fallot (TOF) repair. Traditional echocardiographic assessments are imprecise due to the RV’s location and complex geometry. We propose a novel echocardiographic measurement: RV apical flattening (RVAF) as a screening tool to help identify subjects with severe RV dilation. Patients with repaired TOF who had both echocardiograms and CMR’s within 6-month interval at our institution were included in the study. The RVAF was measured in the four-chamber echocardiographic view as the minor length of RV cavity at the level of RV apical endocardium. Subjects were divided into two groups (group I: RVEDVi ≥ 150 ml/m² and group II; RVEDVi < 150 ml/m²). Echocardiogram and CMR data were compared between groups. A total of 75 subjects were included in the study. Mean age was 12.8 ± 3.6 years. Group I had 36 subjects, and group II had 39 subjects. The mean RVAF was significantly higher in group I (2.7 ± 0.5 cm) compared with group II (1.7 ± 0.4 cm; p < 0.001). There was significant correlation between RVAF and RVEDVi (r = 0.81; p < 0.001). By ROC analysis, an RVAF cutoff value of 2.0 cm had 94 % sensitivity and 77 % specificity in identifying severe RV dilation (area under the curve 0.95). RVAF is a simple and effective echocardiographic screening tool to help identify severe RV dilation. In conjunction with other 2D echocardiographic parameters, this technique would help further refine echocardiography-guided patient selection for timing of CMR and pulmonary valve replacement.     

Echocardiographic Assessment of Right Atrial Pressure in a Pediatric and Young Adult Population

Right atrial pressure (RAP) reflects right-sided cardiac hemodynamics and is useful in management of patients with cardiac and systemic disease. Studies in older adults demonstrated that inferior vena cava (IVC) diameter, IVC collapsibility index, hepatic vein systolic filling fraction (SFF), and right atrial volume (RAV) correlated with mean RAP at catheterization. This study aimed to assess the utility of echocardiographic parameters for assessment of RAP in children and young adults. Patients with pulmonary hypertension or heart transplantation undergoing right heart catheterization were recruited for this prospective observational pilot study. Transthoracic echocardiographic assessment of RAP was performed simultaneously with catheterization. For each parameter, three consecutive cardiac cycles were recorded. Long- and short-axis images of the IVC were obtained. RAV was assessed by area–length and biplane methods. IVC diameters and RAV were indexed to body surface area (BSA)^0.5 and (BSA)^1.4, respectively. Relationships between echocardiographic parameters and mean RAP were correlated using “Pearson’s r.” Fifty subjects aged 0.3–23 years (median 13, mean 12.3 ± 7 years) were enrolled. Mean RAP correlated modestly with RAV (r = 0.51, p < 0.001). Long-axis IVC_max (r = 0.30, p < 0.05) and tricuspid E wave velocity (r = 0.36, p < 0.01) also correlated with mean RAP. RV free wall tissue Doppler velocities, IVC collapsibility index, and hepatic vein SFF had no relation to mean RAP. In a pediatric and young adult population with pulmonary hypertension or heart transplantation, echocardiographic assessment of RAV and long-axis IVC_max provided a reasonable estimate of mean RAP. IVC collapsibility index and hepatic vein SFF demonstrated no association with mean RAP.     

Surgical intervention for congenital pulmonary airway malformation (CPAM) patients with preoperative pneumonia and abscess formation: “open versus thoracoscopic lobectomy”

Aim

Thoracoscopic lobectomy (TL) and open lobectomy (OL) were compared for treating congenital pulmonary airway malformation (CPAM) with preoperative complications, specifically pneumonia/abscess formation (PA).

Methods

The medical records of 46 CPAM patients treated by lobectomy at our institution from 1990 to 2014 were reviewed retrospectively. Four groups, TL for patients without PA (n = 17; TL?), TL for patients with PA (n = 8; TL+), OL for patients without PA (n = 16; OL?), and OL for patients with PA (n = 5; OL+) were compared for operative time, intra/postoperative complications, blood loss, duration of chest tube insertion, postoperative analgesia, pre: postoperative white blood cell (WBC) ratio, and duration of hospitalization.

Results

Operative time for TL+ was longest, but not statistically significant. Incidences of intra/postoperative complications were similar in all groups. Blood loss was significantly less for TL+ versus OL+ (p < .05). WBC ratio was significantly lower in TL+ versus OL+ (p < .05), similar for TL+ and TL?, and significantly higher in OL+ versus OL? (p < .01). Chest tube insertion was significantly longer in OL? versus TL? (p < .01).

Conclusion

PA would not appear to be a contraindication to perform TL in CPAM. TL is associated with less surgical stress than OL despite longer operative time.

     

Common Variants in the CRP Promoter are Associated with a High C-Reactive Protein Level in Kawasaki Disease

Kawasaki disease (KD) is an acute self-limiting form of vasculitis that afflicts infants and children and manifests as fever and signs of mucocutaneous inflammation. Children with KD show various laboratory inflammatory abnormalities, such as elevations in their white blood cell (WBC) count, C-reactive protein (CRP) level, and erythrocyte sedimentation rate (ESR). We here performed a genome-wide association study (GWAS) of 178 KD patients to identify the genetic loci that influence 10 important KD laboratory markers: WBC count, neutrophil count, platelet count, CRP, ESR, hemoglobin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, and total protein. A total of 165 loci passed our arbitrary stage 1 threshold for replication (p < 1 × 10^?5). Of these, only 2 SNPs (rs12068753 and rs4786091) demonstrated a significant association with the CRP level in replication study of 473 KD patients (p < 0.05). The SNP located at the CRP locus (rs12068753) demonstrated the most significant association with CRP in KD patients (beta = 4.73 and p = 1.20 × 10^?6 according to the stage 1 GWAS; beta = 3.65 and p = 1.35 × 10^?8 according to the replication study; beta = 3.97 and p = 1.11 × 10^?13 according to combined analysis) and explained 8.1 % of the phenotypic variation observed. However, this SNP did not demonstrate any significant association with CRP in the general population (beta = 0.37 and p = 0.1732) and only explained 0.1 % of the phenotypic variation in this instance. Furthermore, rs12068753 did not affect the development of coronary artery lesions or intravenous immunoglobulin resistance in KD patients. These results indicate that common variants in the CRP promoter can play an important role in the CRP levels in KD.     

A comparison of the characteristics and precision of needle driving for right-handed pediatric surgeons between right and left driving using a model of infant laparoscopic diaphragmatic hernia repair

Purpose

We compared the characteristics and precision of right and left needle driving for right-handed pediatric surgeons using a laparoscopic diaphragmatic repair model.

Methods

Eighteen right-handed pediatric surgeons performed three needle driving maneuvers using both hands. We evaluated the required time and conducted an image analysis. The total path length, velocity, and acceleration of the needle driving were also evaluated.

Results

Obtained results show the findings for the required time (s, Rt 310.78 ± 148.93 vs. Lt 308.61 ± 122.53, p = 0.93), sum of needle driving balances (mm, Rt 5.23 ± 2.44 vs. Lt 5.05 ± 3.17, p = 0.83), the gap of the needle driving interval (Rt 1.2 ± 0.93 vs. Lt 2.17 ± 1.67, p = 0.04), total path length (mm, Rt 594.03 ± 205.29 vs. Lt 1641.07 ± 670.68, p < 0.01), and average velocity (mm/s, Rt 1.92 ± 0.54 vs. Lt 5.3 ± 1.39, p < 0.01).

Conclusion

For right-handed pediatric surgeons, left needle driving showed almost same quality of right needle driving as regarding the precision. But left needle driving also showed too fast but not economical movement unfortunately, implying rough and risky forceps manipulation. Non-dominant hand training is necessary to avoid organ injury.

     

Mesenchymal expression of the <Emphasis Type="Italic">FRAS1/FREM2</Emphasis> gene unit is decreased in the developing fetal diaphragm of nitrofen-induced congenital diaphragmatic hernia

Purpose

Developmental mutations that inhibit normal formation of extracellular matrix (ECM) in fetal diaphragms have been identified in congenital diaphragmatic hernia (CDH). FRAS1 and FRAS1-related extracellular matrix 2 (FREM2), which encode important ECM proteins, are secreted by mesenchymal cells during diaphragmatic development. The FRAS1/FREM2 gene unit has been shown to form a ternary complex with FREM1, which plays a crucial role during formation of human and rodent diaphragms. Furthermore, it has been demonstrated that the diaphragmatic expression of FREM1 is decreased in the nitrofen-induced CDH model. We hypothesized that FRAS1 and FREM2 expression is decreased in the developing diaphragms of fetal rats with nitrofen-induced CDH.

Methods

Pregnant rats were exposed to either nitrofen or vehicle on gestational day 9 (D9), and fetuses were harvested on D13, D15 and D18. Microdissected diaphragms were divided into nitrofen-exposed/CDH and control samples (n = 12 per time-point and experimental group, respectively). Diaphragmatic gene expression levels of FRAS1 and FREM2 were analyzed by qRT-PCR. Immunofluorescence double staining for FRAS1 and FREM2 was combined with the mesenchymal marker GATA4 in order to evaluate protein expression and localization in pleuroperitoneal folds (PPFs) and fetal diaphragmatic tissue.

Results

Relative mRNA expression of FRAS1 and FREM2 were significantly reduced in PPFs of nitrofen-exposed fetuses on D13 (1.76 ± 0.86 vs. 3.09 ± 1.15; p < 0.05 and 0.47 ± 0.26 vs. 0.82 ± 0.36; p < 0.05), developing diaphragms of nitrofen-exposed fetuses on D15 (1.45 ± 0.80 vs. 2.63 ± 0.84; p < 0.05 and 0.41 ± 0.16 vs. 1.02 ± 0.49; p < 0.05) and fully muscularized diaphragms of CDH fetuses on D18 (1.35 ± 0.75 vs. 2.32 ± 0.92; p < 0.05 and 0.37 ± 0.24 vs. 0.70 ± 0.32; p < 0.05) compared to controls. Confocal laser scanning microscopy revealed markedly diminished FRAS1 and FREM2 immunofluorescence in diaphragmatic mesenchyme, which was associated with reduced proliferation of mesenchymal cells in nitrofen-exposed PPFs and fetal CDH diaphragms on D13, D15 and D18 compared to controls.

Conclusion

Decreased mesenchymal expression of FRAS1 and FREM2 in the nitrofen-induced CDH model may cause failure of the FRAS1/FREM2 gene unit to activate FREM1 signaling, disturbing the formation of diaphragmatic ECM and thus contributing to the development of diaphragmatic defects in CDH.

     

Improved outcomes after technical modifications in tubularized incised plate urethroplasty for mid-shaft and proximal hypospadias

Purpose

To investigate and compare the outcomes after tubularized incised plate (TIP) urethroplasty in mid-shaft and proximal hypospadias using a standard and a modified technique.

Methods

We conducted a retrospective study in 104 consecutive children who underwent mid-shaft or proximal TIP repairs from Jan 2007 to Sept 2015. Patients in Cohort One had dorsal dartos (DD) neourethral coverage while patients in Cohort Two had either de-epithelialized split preputial (DESP) or tunica vaginalis (TV) flap coverage. TV flap was used only when DESP flap was not sufficient to cover the neourethra.

Results

There were 52 patients each in Cohort One (DD, n = 52) and Cohort Two (DESP, n = 38; TV, n = 14) with no difference in ratio of mid-shaft/proximal between the two cohorts. At a median follow-up of 28 months, 36 patients (34.6 %) developed 47 complications including fistula (n = 19; 18.3 %) and neourethral dehiscence (n = 4; 3.8 %). Cohort One patients had significantly more fistula (28.8 vs 7.7 %; p = 0.005) and neourethral dehiscence (7.7 vs 0 %; p = 0.04) than Cohort Two. There was no difference between the two cohorts in the complication rates of meatal stenosis, recurrent ventral curvature and neourethral stricture.

Conclusions

Both DESP and TV flap appear to be superior to DD in preventing fistula and neourethral dehiscence in non-distal TIP repairs.

     

Intervention in Patients with Critical Pulmonary Stenosis in the Ductal Stenting Era

We aimed to assess early and midterm outcomes of balloon valvuloplasty (BVP) procedure in patients with critical pulmonary stenosis (CPS) and to describe the predictors of the need for additional pulmonary flow and reintervention in this subgroup of patients. From 2005 to 2014, 56 neonates were diagnosed with CPS and were included in this study. All echocardiographic, catheterization and angiographic data obtained prior to the initial BVP and at follow-up were reviewed. BVP was successful in 55 neonates (98 %). Twenty-one neonates needed pulmonary blood flow augmentation after BVP (38 %). Ductal stenting (DS) was performed in 20. The patients’ mean tricuspid valve (TV) annulus diameter was 10.4 ± 2 mm, and the Z score was ?1.29 ± 1 (?3.7 to 0.78). The mean pulmonary valve (PV) annulus diameter was 6 ± 0.9 mm, and the Z score was ?1.74 ± 1 (?4.34 to 0.05). A transcatheter or surgical reintervention was performed in 11 patients. A TV Z score < ?1.93 SD predicted the need for pulmonary blood flow augmentation after a successful BVP, with a sensitivity of 63.2% and a specificity of 84.4%. A PV Z score < ?1.69 SD predicted the need for pulmonary flow augmentation, with a sensitivity of 74 %. The presence of bipartite RV was found to be a significant predictor of the need for reintervention (odds ratio 9.6). Our study showed the excellent immediate outcomes of BPV and DS in a pure cohort of patients with CPS. Prophylactic DS in selected cases seems reasonable and safe.     

Body Mass Index (BMI) is Associated with the Therapeutic Response to Oral Rehydration Solution in Children with Postural Tachycardia Syndrome

We aimed to explore whether the body mass index (BMI) was associated with the therapeutic response to oral rehydration solution (ORS) in children with postural tachycardia syndrome (POTS). Fifty-four children diagnosed as having POTS were included in this study. Fifty-six healthy children served as the controls. Children with POTS were treated with ORS, and their treatment response was evaluated. The baseline BMI was obtained by measuring the height and weight. The Pearson correlation was made between the baseline BMI and change in heart rate from supine to upright (ΔHR) and between baseline BMI and symptom score change (post-treatment vs. pre-treatment) in the POTS group. The value of BMI in predicting the therapeutic response to ORS was assessed by analyses of the receiver operating characteristic (ROC) curve. The BMI in the POTS group was significantly lower than that in the control group (18.22 ± 3.23 vs. 20.62 ± 3.05 kg/m², p < 0.01). There were no statistical differences between responders and non-responders in symptom scores before treatment (p > 0.05), but symptom score was lower in responders than that in non-responders (1.52 ± 0.95 vs. 2.32 ± 1.22, p < 0.01) after treatment. The BMI in responders to ORS was significantly lower than that of non-responders (16.32 ± 2.28 vs. 20.43 ± 2.74 kg/m², p < 0.01). The BMI was correlated negatively with ΔHR in the POTS group (n = 54, r = ?0.766, p < 0.01) and with the decrease in symptom scores after treatment in POTS patients (n = 54, r = ?0.28, p < 0.05). ROC curve revealed the area under the curve to be 92.3 % (95 % confidence interval 0.829–0.996). A cutoff value of the BMI of 18.02 kg/m² had high sensitivity (92 %) and high specificity (82.8 %) for predicting the effect of ORS treatment for POTS. BMI is associated with the therapeutic response to ORS in children with POTS.     

Predictors of Early Extubation After Pediatric Cardiac Surgery: A Single-Center Prospective Observational Study

This prospective, observational, single-center study aimed to determine the perioperative predictors of early extubation (<24 h after cardiac surgery) in a cohort of children undergoing cardiac surgery. Children aged between 1 month and 18 years who were consecutively admitted to pediatric intensive care unit after cardiac surgery for congenital heart disease between January 2012 and June 2014. Ninety-nine patients were qualified for inclusion during the study period. The median duration of mechanical ventilation was 20 h (range 1–480), and 64 patients were extubated within 24 h. Four of them failed the initial attempt at extubation, and the success rate of early extubation was 60.6 %. Older patient age (p = .009), greater body weight (p = .009), absence of preoperative pulmonary hypertension (p = .044), lower RACHS-1 category (OR, 3.8; 95 % CI 1.35–10.7; p < .05), shorter cardiopulmonary bypass (p = .008) and cross-clamp (p = .022) times, lower PRISM III-24 (p < .05) and PELOD (p < .05) scores, lower inotropic score (p < .05) and vasoactive-inotropic score (p < .05), and lower number of organ failures (OR, 2.26; 95 % CI 1.30–3.92; p < .05) were associated with early extubation. Our study establishes that early extubation can be accomplished within the first 24 h after surgery in low- to medium-risk pediatric cardiac surgery patients, especially in older ones undergoing low-complexity procedures. A large prospective multiple institution trial is necessary to identify the predictors and benefits of early extubation and to facilitate defined guidelines for early extubation.     

Measurements of Coronary Artery Aneurysms Due to Kawasaki Disease by Dual-Source Computed Tomography (DSCT)

Diameters of coronary artery aneurysms (CAAs) complicating acute phase KD can strongly predict the long-term prognosis of coronary artery lesions (CAL). Recently, computed tomographic angiography (CTA) has been used to detect CAL, and the purpose of this study was to determine whether coronary artery diameters measurements by CTA using dual-source computed tomography (DSCT) can be used instead of coronary angiogram (CAG) measurements. Twenty-five patients (22 males and three females) with CAL due to KD, who had undergone both CTA and CAG within one year, were retrospectively evaluated between 2007 and 2013. A prospective electrocardiogram-triggered CTA was performed on a DSCT (SOMATOM^® Definition, Siemens Healthcare, Germany). Two pediatric cardiologists independently measured the diameters of CAAs twice in each maximum intensity projection (MIP), curved multiplaner reconstruction (MPR) and CAG. We measured 161 segments in total (segment 1–3, 5–7, 11, 13). Diagnostic accuracy was expressed as κ coefficient. A Bland–Altman analysis was also used to assess the intra-observer, inter-observer and inter-modality agreement. The diagnostic quality of CTA was excellent (κ = 0.93). Excellent inter-observer agreement for the diameters of CAAs was obtained for MIP, MPR and CAG and for the intra-observer agreement. The inter-modality agreement was also excellent in measurements of CAA (MPR–CAG: y = 0.9x + 0.40, r = 0.97, p < 0.0001 MIP–CAG: y = x + 0.1, r = 0.94, p < 0.0001). These values in normal coronary arteries were also obtained. We found a significant correlation between CTA and CAG in measuring the coronary arteries. We conclude that measuring coronary artery diameters by CTA is reliable and useful.     

Serial evaluation of myocardial function using the myocardial performance index in Kawasaki disease

Background

Kawasaki disease (KD) is known as systemic vasculitis, and more than half of the patients with KD have myocarditis, which can induce ventricular dysfunction. In this study, we evaluate left ventricular (LV) dysfunction in patients with KD based on the myocardial performance index (MPI) using pulse Doppler (PD) and tissue Doppler imaging (TDI), from the acute to convalescent phases.

Methods

We retrospectively studied 89 children diagnosed with KD from January 2010 to August 2012. We assessed the presence of coronary artery lesions (CALs) and the LV ejection fraction, PD-MPI, and TDI-MPI at diagnosis, and 2, 14, and 56 days after intravenous immunoglobulin (IVIG) treatment. We enrolled 70 healthy children as a control group.

Results

The ejection fraction in patients with KD at diagnosis (67.3 ± 0.9%) was lower than that in the control group (69.8 ± 0.8%, P = 0.035), and the LV TDI-MPIs for patients with KD at diagnosis (0.49 ± 0.01) and 2 days after IVIG treatment (0.48 ± 0.01) were higher than those in the control group (0.45 ± 0.01, P = 0.002, P = 0.033, respectively). No significant differences were found in the LV dysfunction between the patients with complete and incomplete KD. Septal TDI-MPIs in patients with KD with CAL at diagnosis (0.52 ± 0.02) were higher than those in patients with KD without CAL (0.47 ± 0.01, P = 0.019).

Conclusions

Transient LV dysfunction occurred in patients with complete and incomplete KD in the acute stage. In patients with KD with CAL at diagnosis, the LV dysfunction was more prominent. The PD-MPI and TDI-MPI are useful parameters for assessing LV function in patients with KD.

     

Outcomes in Patients with Persistent Ventricular Dysfunction After Stage I Palliation for Hypoplastic Left Heart Syndrome

We sought to describe the clinical course for patients with hypoplastic left heart syndrome and persistent ventricular dysfunction and identify risk factors for death or transplantation before stage II palliation. 138 children undergoing stage I palliation from 2004 to 2011 were reviewed. Twenty-two (16 %) patients (seven Hybrid, 15 Norwood) with two consecutive echocardiograms reporting at least moderate dysfunction were included and compared to case-matched controls. Eleven of the 22 patients with dysfunction (50 %) underwent stage II, seven (32 %) were transplanted, and four (18 %) died prior to stage II. Of the patients who survived to hospital discharge (n = 17) following stage 1, 14 (82 %) required readmission for heart failure (HF) compared to only two (10 %) for controls (p < 0.001). Among patients with ventricular dysfunction, there was an increased use of ACE inhibitors or beta-blockers (82 vs. 25 %; p = 0.001), inotropes (71 vs. 15 %; p = 0.001), ventilation (58 vs. 10 %; p = 0.001), and ECMO (29 vs. 0 %; p = 0.014) for HF management post-discharge when compared to controls. There was a lower heart transplant-free survival at 7 months in patients with dysfunction compared to controls (50.6 vs. 90.9 %; p = 0.040). ECMO support (p = 0.001) and duration of inotropic support (p = 0.04) were significantly associated with death or transplantation before stage II palliation. Patients with ventricular dysfunction received more HF management and related admissions. Longer inotropic support should prompt discussion regarding alternative treatment strategies given its association with death or transplant.     

Myocardial Layers Specific Strain Analysis for the Acute Phase of Infant Kawasaki Disease

Recently, the prevalence of infant Kawasaki disease (KD) has increased. However, the myocardial functional analysis of infant KD can be difficult and rarely reported. The purpose of this study was to investigate layer specific myocardial strain analysis for better assessment of the acute period in infant KD. The study retrospectively reviewed the echocardiographic data of 25 infant patients with KD at the acute phase. With advanced imaging, pulsed tissue Doppler velocity data, myocardial strain with three layers specific analysis was performed. Then the data were compared with 25 age-matched healthy control infants. The measures of longitudinal strain and radial strain were decreased in infant KD compared to healthy controls. The circumferential strain was significantly decreased in infant KD at all three myocardial layers, especially in the endocardial layer (KD: ?20.5 ± 6.4 % vs. control: ?25.6 ± 7.6 %, endocardium, p = 0.00001; ?14.6 ± 4.4 % vs. ?18.1 ± 4.0 %; middle myocardium, p = 0.01; ?9.7 ± 3.3 % vs. ?11.4 ± 3.8 %; epicardium, p = 0.04). The acute phase of infant KD demonstrated decreased myocardial strain measurement. Circumferential strain was the lowest in the endocardial layer. Further continuous long-term follow up for myocardial assessment should be recommended even after recovery with appropriate treatment.     

Serum Hepcidin as a Diagnostic Marker of Severe Iron Overload in Beta-thalassemia Major

Objectives

To investigate potential usefulness of serum hepcidin in the diagnosis of iron overload in children with β-thalassemia.

Methods

A study was conducted on 30 thalassemia major (TM), 30 thalassemia intermedia (TI) and 60 healthy children as controls. Serum hepcidin was measured by Human Hepcidin, ELISA Kit.

Results

β-thalassemia patients had a higher serum hepcidin compared to the controls (p < 0.001). TM group had higher hepcidin and ferritin compared to the TI group (p = 0.034; < 0.001, respectively). Among controls, hepcidin did not correlate with age (r = 0.225, p = 0.084). Among β-thalassemia patients, it correlated positively with age (r = 0.4; p = 0.001), disease duration (r = 0.5; p < 0.001), transfusion frequency (r = 0.35; p = 0.007), total number of transfusions (r = 0.4; p = 0.003), and ferritin (r = 0.3; p = 0.027). Total hemoglobin and serum ferritin were significantly related to hepcidin, which tended to increase by 0.514 ng/ml with each 1 g/dl rise in hemoglobin (p = 0.023) and by 0.002 ng/ml with each 1 ng/ml rise in serum ferritin (p = 0.002). Iron overload [serum ferritin (SF) ≥ 1500 ng/ml] was independently associated with TM (p = 0.001) and elevated serum hepcidin (p = 0.02). The overall predictability of serum hepcidin in severe iron overload was statistically significant when compared to hepcidin to serum ferritin ratio.

Conclusions

Serum hepcidin is elevated in children with β-thalassemia; but this elevation is more evident in TM patients with severe iron overload. Thus, hepcidin can be a potential marker of severe iron overload in patients with TM. Further studies are recommended to compare serum hepcidin and serum ferritin in the prediction of severe iron overload in steady state and during infection or inflammation.

     

Persistent Left Superior Vena Cava Connected to the Coronary Sinus in the Fetus: Effects on Cardiac Structure and Flow Dynamics

Ventricular size discrepancy may be due to a persistent left superior vena cava (PLSVC) in utero. We sought to investigate for differences in cardiac structure measures and hemodynamics between fetuses with isolated PLSVC connected to the coronary sinus (CS) and normal. Fetuses diagnosed with isolated PLSVC in the second and third trimester were enrolled. We defined two groups: group 1, twenty-five fetuses in the second trimester (22–27 W + 6d); group 2, twenty-two fetuses in the third trimester (28–39 W + 6d). Fifty-three fetuses without intra-cardiac or extra-cardiac anomalies and gestation age-matched were divided into normal control groups: group 3, 28 fetuses in the second trimester; group 4, 25 fetuses in the third trimester. Parameters of cardiac structure and hemodynamics were measured, including: left- and right-side heart size, the diameter of foramen ovale, aorta (AO), aortic isthmus and pulmonary artery (PA), and ratios of cardiac structure RV/LV, RA/LA and PA/AO were calculated. Hemodynamic parameters measured included: flow velocity across mitral valve, tricuspid valve and foramen ovale. In the second trimester, the ratio of RV/LV and PA/AO of the PLSVC fetuses was significantly larger than normal, while the AO diameter, aortic isthmus diameter were smaller than normal (P < 0.05). However, in the third trimester, only the ratio of PA/AO of the PLSVC fetuses was significantly larger, and the aortic isthmus diameter was still smaller than normal (P < 0.05). Isolated PLSVC connecting to the CS is associated with differences in cardiac structure size from normal. These differences appear to diminish with gestational age. A dilated CS may have an influence on development of fetal left heart structures.