Biventricular function in preterm infants with patent ductus arteriosus ligation: A three-dimensional echocardiographic study.

BACKGROUND: The detailed hemodynamics after patent ductus arteriosus (PDA) ligation in preterm infants remain unknown. We aimed to clarify the effect of surgical ligation on left ventricular (LV) and right ventricular (RV) volume and function. METHODS: Echocardiography was performed in 41 preterm infants (median gestational age: 25 weeks) before and after PDA ligation. Global longitudinal strain was determined using three-dimensional speckle-tracking echocardiography. These values were compared with those in 36 preterm infants without PDA (non-PDA). RESULTS: Preoperatively, the PDA group had greater end-diastolic volume (EDV) and cardiac output (CO) in both ventricles, a higher LV ejection fraction (LVEF) (53% vs 44%) and LV global longitudinal strain, and a lower RVEF (47% vs 52%) than the non-PDA group. At 4-8 h postoperatively, the two groups had a similar LVEDV and RVEDV. However, the PDA group had a lower EF and CO in both ventricles than the non-PDA group. At 24-48 h postoperatively, the RVEF was increased, but the LVEF remained decreased, and LVCO was increased. CONCLUSIONS: PDA induces biventricular loading and functional abnormalities in preterm infants, and they dramatically change after surgery. Three-dimensional echocardiography may be beneficial to understand the status of both ventricles. IMPACT: Preterm infants are at high risk of hemodynamic compromise following a sudden change in loading conditions after PDA ligation. Three-dimensional echocardiography enables quantitative and serial evaluation of ventricular function and volume in preterm infants with PDA. PDA induces biventricular loading and functional abnormalities in preterm infants, and they dramatically change after surgery.

Feasibility of Three-Dimensional Echocardiographic Analysis in Newborns by Novice Observers.

Three-dimensional echocardiography (3DE) provides better interobserver agreement than conventional methods. However, more evidence of whether there is good agreement between novice and experienced observers, especially in pediatric 3DE analysis, is required. We conducted 3DE analysis training in novice observers and investigated the agreement when analyzing 3DE images between novice and experienced observers. One experienced and 4 novice observers independently analyzed 60 3DE images obtained from neonates. The left and right ventricular end-diastolic volume (LVEDV and RVEDV), end-systolic volume (LVESV and RVESV), ejection fraction (LVEF and RVEF), left ventricular global longitudinal strain, and global circumferential strain (LVGLS and LVGCS) were calculated. The novices received hands-on instruction in the analysis procedure before the analysis and received further feedback after their first 40 analyses. Agreement between the novices and the experienced observer was evaluated by the intra-class correlation coefficient (ICC) and percentage difference in 3 groups of 20 images each (images 1-20, 21-40, and 41-60). The ICC of LVEDV was > 0.85 from the first 20 images and increased with experience. The ICCs for RVEDV and RVESV were low from the first 20 images but increased linearly, reaching an ICC > 0.9 in 3 of the 4 novice observers in the last 20 images. The range of the percentage difference was small for LVEDV, RVEDV, LVEF, and RVEF. Novices show an experienced level of 3DE analysis capability in LVEDV and RVEDV after analyzing 40 to 60 images. Training of novices is effective for analyzing LVEDV and RVEDV in 3DE images.

Right to left ventricular volume ratio is associated with mortality in congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with high neonatal mortality. We performed this study to test the hypothesis that left ventricular (LV) and right ventricular (RV) volumes assessed by three-dimensional echocardiography may be associated with mortality in CDH. METHODS: This study was a single-center retrospective cohort study involving 35 infants with CDH. RV and LV end-diastolic volume (RVEDV and LVEDV, respectively) were measured by three-dimensional echocardiography and were corrected by birth body weight (BBW) on day 1. RVEDV/BBW, LVEDV/BBW, and LVEDV/RVEDV were compared between CDH survivors and non-survivors. Receiver-operating characteristic curve analysis was performed to assess the predictive ability for mortality of the echocardiographic parameters. RESULTS: Comparing CDH non-survivors (n = 6) with survivors (n = 29), respectively, RVEDV/BBW was significantly larger (2.54 ± 0.33 vs 1.86 ± 0.35 ml/kg; P < 0.01), LVEDV/BBW was significantly smaller (0.86 ± 0.21 vs 1.22 ± 0.33 ml/kg; P < 0.001), and LVEDV/RVEDV was significantly lower (0.34 ± 0.06 vs 0.66 ± 0.18; P < 0.001). The area under the curve for LVEDV/RVEDV was the largest (0.98). CONCLUSIONS: Three-dimensional echocardiographic volume imbalance between the RV and LV was remarkable in CDH non-survivors. The LVEDV/RVEDV ratio may be associated with mortality in CDH. IMPACT: Mortality with congenital diaphragmatic hernia (CDH) is high, and evaluating left and right ventricular structures and functions may be helpful in assessing the prognosis. Three-dimensional (3D) echocardiography indicated that the left ventricular end-diastolic volume/right ventricular end-diastolic volume ratio within 24 h after birth was associated with mortality in CDH infants. The usefulness of this ratio should be validated in prospective multicenter studies involving larger numbers of patients.

Successful Pacemaker Setting Using Treadmill Exercise Test in a Child with Fontan Circulation.

The usefulness of a treadmill exercise test for determining optimal pacemaker settings remains unknown. An 8-year-old boy with Fontan circulation and a dual-chamber pacemaker for a rate-dependent advanced atrioventricular block suffered from dullness and poor school attendance. Although the pacemaker log showed an atrial tachycardia/fibrillation episode, treadmill exercise testing revealed sinus tachycardia, which converted to a 2:1 atrioventricular block. The pacemaker setting was optimized, with improvement in the patient's condition.Treadmill exercise testing helps optimize dual-chamber pacemaker settings in children with complex congenital heart disease involved in energy-consuming physical activities and having a wide working range in sinus rate.

Is ibuprofen superior to indomethacin for patent ductus arteriosus in Japanese preterm infants?

BACKGROUND: Many clinical trials have indicated that ibuprofen (IBU) has similar effects to indomethacin (IND) on the closure of patent ductus arteriosus (PDA) with fewer adverse effects. Owing to the scarce evidence on IBU use in Japan because of its recent approval we performed this observational study to compare the efficacy and safety of IBU with the efficiency and safety of IND. METHODS: We included infants (gestational age < 30 weeks) with hemodynamically significant PDA under a prophylactic IND protocol for intraventricular hemorrhage who were treated with either IND (n = 30) or IBU (n = 30). We compared a PDA closing effect, changes in ultrasonography findings, and adverse effects between the groups. RESULTS: There was no significant difference in the rates of PDA closure in the first treatment course (IND vs IBU: 46.7% vs 50.0%, P = 0.796) and surgical closure (IND vs IBU: 20.0% vs 20.0%, P = 1.000) between the groups. Both groups showed significant oliguria (IND vs IBU: 30.0% vs 23.3%, P = 0.559) and increased serum creatinine levels after treatment. However, an increase in serum creatinine level by >0.3 mg/dL, a criterion for acute kidney injury, was less frequent in the IBU group (35.7%) compared with that in the IND group (84.2%, P = 0.004). There were no significant differences in echocardiographic changes and jaundice and hypoglycemia incidence rates between the groups. CONCLUSIONS: Except for an increase in serum creatinine levels by >0.3 mg/dL, which was less frequent with IBU, IBU had similar efficacy and safety as IND for preterm PDA. Ibuprofen and IND should be cautiously administered.

Efficacy of a Subcutaneous Implantable Cardioverter Defibrillator in a Child with Early Repolarization Syndrome.

An early repolarization (ER) pattern or J waves are considered to be a benign finding observed in the healthy population, however, it has been pointed out that the ER pattern seen in the inferolateral leads could be an independent risk factor for fatal arrhythmias. We present a pediatric case in which early repolarization syndrome (ERS) was suspected due to the presence of ER or J waves in the inferior leads, which eventually disappeared after the administration of pilsicainide. During the follow-up period, several fatal ventricular arrhythmias were recorded after implantation of a subcutaneous implantable cardiac defibrillator (S-ICD). This report describes the efficacy of S-ICDs in a child with an ER pattern after aborted sudden cardiac death.

Basilar Artery Dissection Complicated with Infective Endocarditis.

A 14 year-old boy developed infective endocarditis of the mitral valve caused by Methicillin-sensitive Staphylococcus aureus and became comatose. Isolated basilar artery dissection was initially observed on the 3rd day by magnetic resonance imaging (MRI), ie, it did not exist on day 1. He underwent successful urgent mitral valve repair on the 5th day because of highly mobile vegetations and a newly emerged brain infarction under optimal antibiotic administration. Postoperatively, he recovered well and the basilar artery dissection was found to have recovered on an MRI on the 25th day without any specific intervention. This clinical course indicated that intracranial artery dissection may occur as a complication of infective endocarditis and supports the importance of the careful evaluation of brain MRI in patients with infective endocarditis.

Successful Percutaneous Thrombectomy of the Left Pulmonary Artery in a Neonate After a Patent Ductus Arteriosus Clipping.

We report a neonate with a successful percutaneous thrombectomy of a total thrombotic occlusion of the left pulmonary artery (LPA) after a surgical clipping for a patent ductus arteriosus (PDA). We suspected the compression of the LPA by the clipping and postoperative hemodynamic instability caused the LPA obstruction. After the surgical removal of the PDA clip and division of the PDA, we could safely retrieve the LPA thrombus with a non-hydrodynamic thrombectomy catheter for coronary arteries.

Steroid-Refractory Protein-Losing Enteropathy with Gastrointestinal Bleeding in a Patient with Fontan Circulation.

Protein-losing enteropathy (PLE) is one of the major complications after a Fontan operation. Some PLE patients suffer from concurrent gastrointestinal bleeding. An effective treatment regimen for such patients has not been established yet. Further, it remains unknown whether PLE and gastrointestinal bleeding coexist independently, or protein losing is associated with gastrointestinal bleeding. We report a 7-year-old steroid-refractory post-Fontan PLE case suggesting the latter pathogenesis together with a literature review.

Blood reservoir function in patients with Fontan circulation and asplenia syndrome.

Splanchnic circulation constitutes a major portion of the vasculature capacitance and plays an important role in maintaining blood perfusion. Because patients with asplenia syndrome lack this vascular bed as a blood reservoir, they may have a unique blood volume and distribution, which may be related to their vulnerability to the haemodynamic changes often observed in clinical practice. During cardiac catheterisation, the mean circulatory filling pressure was calculated with the Valsalva manoeuvre in 19 patients with Fontan circulation, including 5 patients with asplenia syndrome. We also measured the cardiac output index and circulatory blood volume by using a dye dilution technique. The blood volume and the mean circulatory filling pressure and the venous capacitance in patients with asplenia syndrome were similar to those in the remaining patients with Fontan circulation (85 ± 14 versus 77 ± 18 ml/kg, p = 0.43, 31 ± 8 versus 27 ± 5 mmHg, p = 0.19, 2.8 ± 0.6 versus 2.9 ± 0.9 ml/kg/mmHg, p = 0.86). Unexpectedly, our data indicated that patients with asplenia syndrome, who lack splanchnic capacitance circulation, have blood volume and venous capacitance comparable to those in patients with splanchnic circulation. These data suggest that (1) there is a blood reservoir other than the spleen even in patients with asplenia; (2) considering the large blood pool of the spleen, the presence of a symmetrical liver may represent the possible organ functioning as a blood reservoir in asplenia syndrome; and (3) if this is indeed the case, there may be a higher risk of hepatic congestion in patients with Fontan circulation with asplenia syndrome than in those without.

Case of Incomplete Kawasaki Disease with No Symptoms Except Fever Causing the Development of Coronary Aneurysm.

Incomplete Kawasaki disease (iKD), which does not satisfy the standard KD diagnostic criteria because the required number of principal symptoms is not met, sometimes causes coronary aneurysms. Here we report the case of a patient with iKD who presented with only one principal symptom that resulted in the development of coronary aneurysm, as evidenced by angiography.

Improvement in Dyssynchrony with Pharmacological Ablation of Right-Sided Accessory Pathway-Induced Cardiomyopathy in Infants.

Right-sided accessary pathways in patients with Wolff-Parkinson-White (WPW) syndrome may cause cardiac dyssynchrony and dilated cardiomyopathy, with a characteristic septal shape, irrespective of any supraventricular tachycardia episodes. We report on two infants (13 and 5 months), whose right-sided accessary pathway-induced dilated cardiomyopathy was successfully treated by flecainide for the first time. After the flecainide administration, an abnormal aneurysmal dilation of the basal interventricular septum was almost restored to normal, and the decreased ejection fraction recovered. Flecainide use may be an important therapeutic option for this entity to avoid catheter ablation during infancy.

Importance of dynamic central venous pressure in Fontan circulation.

We tested our hypotheses that central venous pressure (CVP) shows an excessive increase in response to volume overload in Fontan circulation according to the extent of the reduction in venous capacitance (Cv), and that the maximum CVP after volume loading is associated with hepatic congestion. Changes in CVP after angiography (volume loading) were examined in 40 patients with Fontan circulation and 29 controls with biventricular circulation. CVP significantly increased with angiography in both groups, but the changes were much more evident in the Fontan group than in controls (3.3 ± 2.0 vs. 0.9 ± 1.4 mmHg, p = 0.0003). Multivariate analysis demonstrated that reduced Cv was the only significant determinant of CVP increase, independent of the amount of injected contrast medium, blood volume, pulmonary resistance, and ventricular diastolic stiffness (p < 0.05). Importantly, the use of a venodilator was associated with increased Cv and the resultant suppression of CVP elevation with volume load. In addition, CVP levels both at baseline (p = 0.02) and after volume loading (p = 0.01) were weakly but significantly correlated with the plasma levels of γ-glutamyl transpeptidase, a marker of hepatic congestion; however, multivariate analysis revealed that the CVP level after volume loading was a more important determinant of hepatic congestion. The results of this study highlight the importance of assessing dynamic in addition to static CVP for a better understanding of Fontan circulation. The potential importance of Cv as a therapeutic target for improving Fontan physiology needs further elucidation.

Spontaneous regression of severe aortic coarctation in trisomy 18.

Spontaneous regression of severe aortic coarctation with ductus dependency has not been reported. We experienced a case of trisomy 18 with spontaneous regression of severe aortic coarctation complicated by ventricular septal defect and patent ductus arteriosus. The aortic isthmus diameter was 1.2 mm at birth. After 5 months, it increased to 4.5 mm, and the shape of the isthmus was fully normalised.

First Pediatric Case of Infective Endocarditis Caused by Serratia Liquefaciens.

Infective endocarditis (IE) caused by Serratia liquefaciens has been reported in only 2 adults. We experienced the first pediatric (neonatal) case of IE caused by S. liquefaciens, with mitral valve vegetation 4 days after a palliative heart surgery. This report underscores the importance of treating for both gram-positive and gram-negative bacteria in IE cases until the blood cultures elucidate the details.

A 1-year-old boy with long pauses caused by paroxysmal atrioventricular block and sinus arrest: Vagal reflex and effect of pacing.

Paroxysmal atrioventricular block (PAVB) is rare in children. A 1-year-old boy presented with PAVB and sinus arrest that resulted in refractory life-threatening symptomatic long pauses. Continuous heart rate variability analysis with high time resolution (wavelet analysis) revealed an abrupt increase in parasympathetic activity just before a long pause, which indicated a vagal reflex. Although a pacemaker is not always effective because of a concomitant vasodepressive response in such cases, the complete stabilization after pacemaker implantation in this case supports the necessity and usefulness of pacemaker implantation in patients with reflex-induced highly symptomatic bradycardia.

Multiple variations of the cerebral arteries associated with tetralogy of Fallot: a case report.

Tetralogy of Fallot (TOF) can be associated with vascular malformations or variations, especially of the aortic arch or supracardiac major venous systems. We report its association with an extremely rare combination of three such variations of the cerebral arteries-a right persistent hypoglossal artery, an extremely rare left carotid-right anterior cerebral artery (ACA) anastomosis, and a left accessory middle cerebral artery arising from the A1-A2 junction of the left ACA-which were diagnosed by magnetic resonance angiography in a 9-year-old girl during evaluation of multiple acute cerebral infarctions.

Novel mechanisms for cerebral blood flow regulation in patients with congenital heart disease.

BACKGROUND: The mechanisms that regulate cerebral flow in patients after surgery for congenital heart diseases (CHDs) remain poorly understood. We tested our hypothesis that postoperative patients with CHD have disease- or hemodynamic-specific compensatory mechanisms for maintaining cerebral perfusion. METHODS: A total of 89 children with specific hemodynamics including Glenn (n = 14), Fontan (n = 19), repaired tetralogy of Fallot (n = 24), and control patients (n = 32) were enrolled. The resistance and blood flow distribution between the brain (Rc and CIc) and lower body (Rs and CIs) were calculated by measuring the hemodynamic changes resulting from inferior vena cava occlusion during cardiac catheterization. RESULTS: Despite considerable differences in cardiac index and superior vena cava pressure (SVCp), cerebral blood flow was preserved in all noncontrol groups, with a ratio between the vascular resistances in the cerebral and lower body circulation (Rc/Rs) that was significantly lower than that in controls. Interestingly, the reduced Rc/Rs of Glenn patients was mediated by the reduced Rc, whereas augmented Rs was conducive to the reduced Rc/Rs in the Fontan and tetralogy of Fallot groups. Multivariate analysis revealed that high SVCp was significantly associated with low Rc. Although low cardiac index was significantly associated with increased Rc and Rs, its impact was much greater on Rs than on Rc. CONCLUSIONS: Compensatory mechanisms for cerebral flow regulation occur according to hemodynamic abnormality type in postoperative patients with CHD. Because such a regulation mechanism implies cerebral circulation fragility, further investigations are needed to address the impacts of cerebral circulation properties on neurodevelopmental outcomes.

Prevalence, implication, and determinants of worsening renal function after surgery for congenital heart disease.

Accumulating data in adults indicate the prognostic importance of worsening renal function (WRF) during treatment of acute heart failure. Venous congestion appears to play a dominant role in WRF; however, data regarding WRF in children with congenital heart disease (CHD) are limited. The present study was conducted to elucidate the prevalence and characteristics of WRF after surgery for CHD in children. We also tested our hypothesis that, similar to adult heart failure, venous congestion is an important determinant of WRF independent of cardiac output in this population. Fifty-five consecutive pediatric patients who underwent cardiovascular surgery for CHD were studied (median age 0.7 years; range 3 days to 17 years). The degree of WRF was assessed by the difference between the maximum levels of postoperative serum creatinine (Cr) and preoperative serum Cr. There was a high prevalence of WRF in the present cohort: an increase in Cr level was observed in 47 patients (85 %) and a Cr increase ≥0.3 mg/dL was seen in 23 (42 %). Importantly, WRF was significantly associated with a worse clinical outcome of a longer stay in the intensive care unit and hospital (both p < 0.05), even after controlling for age and operative factors. In addition, multivariate regression analysis revealed that central venous pressure, rather than cardiac output, was an independent determinant of WRF. Postoperative management to relieve venous congestion may help ameliorate or prevent WRF and thereby improve outcomes in patients with CHD.