Validation of Real-time Transthoracic 3D echocardiography in Children with Congenital Heart Disease

Aims: Assessment of feasibility, accuracy and applicability in clinical practice of real-time three-dimensional echocardiography (RT-3DE) in children with congenital heart defects. Methods and results: From September 2004 to June 2005, 100 consecutive children (57 infants, 43 children > 1 year of age), who were scheduled for corrective intracardiac surgery, were enrolled in this study. RT-3DE was performed with Philips Sonos 7500 echo-system and off-line analysis with TomTec Echoview© software. Quantitative and qualitative assessments of the region of interest were performed on the 3D reconstruction, comparing these results with the anatomic findings and measurements performed during intracardiac surgery. Acquisition of RT-3DE datasets was feasible in 92 of the 100 (92%) patients and acquisition time was 6 ± 3 minutes. The overall quantitative analyses showed an excellent correlation (r < 0.90) between RT-3De and surgery. Also the qualitative analyses were accurate compared with surgical findings, in all patients in which RT-3DE was feasible. Conclusion: This study shows that RT-3DE can be used in the clinical practice for the assessment of intracardiac anatomy in children with congenital heart disease. The information derived from the 3D reconstructions can be taken into consideration in the preoperative planning and management regarding interventional or surgical therapy

Electrocardiographic Criteria for Left Ventricular Hypertrophy in Children

Previous studies to determine the sensitivity of the electrocardiogram (ECG) for left ventricular hypertrophy (LVH) in children had their imperfections: they were not done on an unselected hospital population, several criteria used in adults were not applied to children, and obsolete limits of normal for the ECG parameters were used. Furthermore, left ventricular mass (LVM) was taken as the reference standard for LVH, with no regard for other clinical evidence. The study population consisted of 832 children from whom a 12-lead ECG and an M-mode echocardiogram were taken on the same day. The validity of the ECG criteria was judged on the basis of an abnormal LVM index, either alone or in combination with other clinical evidence. The ECG criteria were based on recently established age-dependent normal limits. At 95% specificity, the ECG criteria have low sensitivities (<25%) when an elevated LVM index is taken as the reference for LVH. When clinical evidence is also taken into account, the sensitivity improved considerably (<43%). Sensitivities could be further improved when ECG parameters were combined. The sensitivity of the pediatric ECG in detecting LVH is low but depends strongly on the definition of the reference used for validation

Carbon monoxide poisoning mimicking long‐QT induced syncope

Carbon monoxide (CO)poisoning is a rare cause of QT prolongation, and is therefore easily missed. The case of a patient with unexplained syncope and QT prologation on the electrocardiogram that turned out to be related to CO poisoning is reported here. In patients with QT prolongation, uncommon causes also should be looked for

A low incidence of preoperative neurosonographic abnormalities in neonates with heart defects

Background and aim: To investigate whether neonates with prenatally detected congenital heart defects (CHD) demonstrate cerebral abnormalities on early preoperative cranial ultrasound (CUS), compared to healthy neonates, and to measure brain structures to assess brain growth and development in both groups. Study design, subjects and outcome measures: Prospective cohort study with controls. Between September 2013 and May 2016 consecutive cases of prenatally detected severe isolated CHD were included. Neonatal CUS was performed shortly after birth, before surgery and in a healthy control group. Blinded images were reviewed for brain abnormalities and various measurements of intracranial structures were compared. Results: CUS was performed in 59 healthy controls and 50 CHD cases. Physiological CUS variants were present in 54% of controls and in 52% of CHD cases. Abnormalities requiring additional monitoring (both significant and minor) were identified in four controls (7%) and five CHD neonates (10%). Significant abnormalities were only identified in four CHD neonates (8%) and never in controls. A separate analysis of an additional 8 CHD neonates after endovascular intervention demonstrated arterial stroke in two cases that underwent balloon atrioseptostomy (BAS). Cerebral measurements were smaller in CHD neonates, except for the cerebrospinal fluid measurements, which were similar to the controls. Conclusions: The prevalence of significant preoperative CUS abnormalities in CHD cases was lower than previously reported, which may be partially caused by a guarding effect of a prenatal diagnosis. Arterial stroke occurred only in cases after BAS. As expected, neonates with CHD display slightly smaller head size and cerebral growth

Implantable Cardioverter Defibrillator Therapy for Prevention of Sudden Cardiac Death in Children in The Netherlands

Introduction: Implantable cardioverter defibrillator (ICD) therapy is increasingly used in children. The purpose of this multicenter study is to evaluate mid-term clinical outcome and to identify predictors for device discharge in pediatric ICD recipients. Methods and Results: From 1995 to 2006, 45 patients in The Netherlands under the age of 18 years received an ICD. Mean age at implantation was 10.8 +/- 5.2 years. Primary prevention (N = 22) and secondary prevention (N = 23) were equally distributed. Underlying cardiac disorders were primary electrical disease (55%), cardiomyopathy (20%), and congenital heart disease (17%). The follow-up was 44 +/- 32.9 months. Three patients (7%) died and one patient (2%) underwent heart transplantation. ICD-related complications occurred in eight patients (17%), seven of whom had lead-related complications. Fourteen patients (31%) received appropriate ICD shocks; 12 patients (27%) received inappropriate ICD shocks. Fifty-five percent of 22 ICD recipients under the age of 12 years received appropriate shocks, which was higher as compared with 9% of 23 older ICD recipients (P = 0.003). Although the incidence of appropriate shocks in the present study was larger in secondary prevention (9/23; 39%) as compared with primary prevention (5/22; 23%), this difference did not reach significance. Conclusions: In our population of patients, children <12 years of age had more appropriate shocks than patients 13-18 years. The complication rate is low, and is mainly lead related. (PACE 2010; 33:179-185)

The aorto-left ventricular tunnel from a fetal perspective: Original case series and literature review.

IntroductionAorto-left ventricular tunnel (ALVT) accounts for &lt;0.1% of congenital heart defects. Evidence on the prognosis from a fetal perspective is limited. With this retrospective international case series, we provide information on the outcome of fetuses with ALVT.MethodsAll members of the Association for European Pediatric and Congenital Cardiology's (AEPC) fetal working group and fetal medicine units worldwide were invited for participation. We observed antenatal parameters, neonatal outcome and postnatal follow-up. Additionally, a systematic search of the literature was performed.ResultsTwenty fetuses with ALVT were identified in 10 participating centers (2001-2019). Fetal echocardiographic characteristics of ALVT included an increased cardiac-thorax ratio (95%), left ventricular end-diastolic diameter (90%) and a dysplastic aortic valve (90%). Extracardiac malformations were rare (5%). Eight fetuses died at a median gestational age (GA) of 21&nbsp;+&nbsp;6&nbsp;weeks (range, 19-24): all showed signs of hydrops prior to 24&nbsp;weeks or at autopsy. All others (60%, 12/2) were live-born (median GA 38&nbsp;+&nbsp;4, range 37-40), underwent surgery and were alive at last follow up (median 3.2&nbsp;years, range 0.1-17). The literature reported 22 ALVT fetuses with similar outcome.ConclusionsIn the absence of fetal hydrops, ALVT carries a good prognosis. Fetuses who survive to 24&nbsp;weeks without hydrops are likely to have a good outcome

The aorto-left ventricular tunnel from a fetal perspective : Original case series and literature review

Introduction Aorto-left ventricular tunnel (ALVT) accounts for &lt;0.1% of congenital heart defects. Evidence on the prognosis from a fetal perspective is limited. With this retrospective international case series, we provide information on the outcome of fetuses with ALVT. Methods All members of the Association for European Pediatric and Congenital Cardiology's (AEPC) fetal working group and fetal medicine units worldwide were invited for participation. We observed antenatal parameters, neonatal outcome and postnatal follow-up. Additionally, a systematic search of the literature was performed. Results Twenty fetuses with ALVT were identified in 10 participating centers (2001-2019). Fetal echocardiographic characteristics of ALVT included an increased cardiac-thorax ratio (95%), left ventricular end-diastolic diameter (90%) and a dysplastic aortic valve (90%). Extracardiac malformations were rare (5%). Eight fetuses died at a median gestational age (GA) of 21 + 6 weeks (range, 19-24): all showed signs of hydrops prior to 24 weeks or at autopsy. All others (60%, 12/2) were live-born (median GA 38 + 4, range 37-40), underwent surgery and were alive at last follow up (median 3.2 years, range 0.1-17). The literature reported 22 ALVT fetuses with similar outcome. Conclusions In the absence of fetal hydrops, ALVT carries a good prognosis. Fetuses who survive to 24 weeks without hydrops are likely to have a good outcome

Implantable cardioverter defibrillator implantation in children in The Netherlands.

Contains fulltext : 47626reimer.pdf (publisher's version ) (Closed access)To evaluate the indications, underlying cardiac disorders, efficacy and complications involved with implantable cardioverter-defibrillators (ICDs) in paediatric patients in The Netherlands, the records of all patients aged 18 years or younger who underwent ICD placement were reviewed retrospectively. Between January 1995 and September 2002, 23 patients (11 males, 12 females; median age 12 years, range 6 months to 16 years) underwent ICD implantation. The ICD was implanted for aborted sudden cardiac death ( n = 14), syncope ( n = 5) or for primary prevention of sudden cardiac death ( n = 4). Underlying cardiac disorders were electrical diseases ( n = 16), hypertrophic or dilated cardiomyopathy ( n = 4) and congenital cardiac malformations ( n = 3). Five patients had an epicardially placed ICD, while 18 underwent a transvenous approach. The generator was placed in an abdominal position in eight patients, whereas it was placed in the subpectoral region in 15. There was no early mortality. Median hospital stay was 5 days (range 2-30 days). Median follow-up time was 29 months (range 1 month to 7 years). Seven patients experienced an inappropriate shock after a median period of 7 months; five patients an appropriate shock after a median period of 3 months. The reasons for inappropriate shock were supraventricular tachycardia ( n = 1), sinus tachycardia ( n = 4) or T-wave sensing (n = 2). One patient with malignant long QT syndrome died of intractable arrhythmias and irreversible cardiomyopathy. Generator replacement was necessary in four patients after 28, 44, 51 and 54 months respectively. CONCLUSION: Implantable cardioverter-defibrillator implantation in paediatric patients is a safe procedure with a good medium-term outcome. The most serious problem is the occurrence of a significant number of inappropriate shocks