Reeni Soni

Presentation, Diagnosis, and Medical Management of Heart Failure in Children: Canadian Cardiovascular Society Guidelines

Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.

Successful Parental Use of an Automated External Defibrillator for an Infant With Long-QT Syndrome

Congenital long-QT syndrome with 2:1 atrioventricular block presenting in the perinatal period is rare, has a poor prognosis, and leads to high risk for lethal ventricular arrhythmic events. An implantable cardioverter-defibrillator seems to be the most effective treatment in the prevention of arrhythmic sudden cardiac death in patients with long-QT syndrome. Technical limitations and risks associated with implantable cardioverter-defibrillators in asymptomatic infants is considered too great to justify use for primary prophylaxis against sudden cardiac death. In this case report we describe the first successful parental use of an automated external defibrillator prescribed for primary prophylaxis against sudden cardiac death in an infant with long-QT syndrome.

The registry and follow-up of complex pediatric therapies program of Western Canada: a mechanism for service, audit, and research after life-saving therapies for young children.

Newly emerging health technologies are being developed to care for children with complex cardiac defects. Neurodevelopmental and childhood school-related outcomes are of great interest to parents of children receiving this care, care providers, and healthcare administrators. Since the 1970s, neonatal follow-up clinics have provided service, audit, and research for preterm infants as care for these at-risk children evolved. We have chosen to present for this issue the mechanism for longitudinal follow-up of survivors that we have developed for western Canada patterned after neonatal follow-up. Our program provides registration for young children receiving complex cardiac surgery, heart transplantation, ventricular assist device support, and extracorporeal life support among others. The program includes multidisciplinary assessments with appropriate neurodevelopmental intervention, active quality improvement evaluations, and outcomes research. Through this mechanism, consistently high (96%) follow-up over two years is maintained.

Late gadolinium enhancement cardiovascular magnetic resonance in genotyped hypertrophic cardiomyopathy with normal phenotype

A 35 year-old asymptomatic Caucasian female with a family history of hypertrophic cardiomyopathy (HCM) was referred for cardiologic evaluation. The electrocardiogram and transthoracic echocardiogram were normal. Cardiovascular magnetic resonance (CMR) was performed for further assessment of myocardial function and presence of myocardial scar. CMR showed normal left ventricular systolic size, measurements and function. However, there was extensive, diffuse late gadolinium enhancement (LGE) throughout the left ventricle. This finding was consistent with extensive myocardial scarring and was highly suggestive of advanced, non-ischemic cardiomyopathy. Genotyping showed a heterozygous mis-sense mutation (275G>A) in the cardiac troponin T (TNNT2) gene, which is causally associated with HCM. There have been no previous reports of such extensive, atypical pattern of myocardial scarring despite an otherwise structurally and functionally normal left ventricle in an asymptomatic individual with HCM. This finding has important implications for phenotype screening in HCM.

Air Transported Pediatric Rescue Extracorporeal Membrane Oxygenation: A Single Institutional Review

Background: Pediatric extracorporeal membrane oxygenation (ECMO) programs are sophisticated endeavors usually found only in high-volume cardiac surgical programs. Worldwide, many cardiology programs do not have on-site pediatric cardiac surgery expertise. Our single-center experience shows that an organized multidisciplinary rescue-ECMO program, in collaboration with an accepting facility, can achieve survival rates comparable to modern era on-site ECMO. Methods: A retrospective review was conducted of all patients initiated on rescue-ECMO from 2004 to 2009 in a single academic pediatric hospital without a pediatric cardiac surgery program. All aspects of ECMO were formalized using Failure Mode Effects Analysis. Results: Eight patients were initially cannulated for ECMO at our institution. Six were subsequently transported by air to the receiving facility 1,305 km away. Extracorporeal membrane oxygenation was initiated in 0.2% of our Pediatric Intensive Care Unit admissions and in 0.52% of all our pediatric cardiac patients. Mean age was 4.0 years (7 weeks to 15 years). Indications for ECMO initiations were cardiogenic shock (n = 5) and acute respiratory distress syndrome (n = 3). Six had veno-arterial- and two had veno-veno ECMO. Two patients were not transported (one death and one weaned locally). Six patients were successfully transported within 2 to 24 hours, with a survival to hospital discharge rate of 67% (four of six). Median total time on ECMO was 5.5 days. Complication rate was 50% (4/8). Conclusions: Our rescue-ECMO survival results were comparable to that of current published results from established pediatric ECMO programs. Air transport of ECMO patients can be performed safely using an organized multidisciplinary team approach.

Rapidly progressive idiopathic dilation of the right atrium in infancy associated with dynamic obstruction of the airways

We present a young infant with rapidly progressive idiopathic dilation of the right atrium, complicated by symptomatic and dynamic obstruction of the airways secondary to proximal obstruction which was reversed subsequent to surgical reduction of the right atrium. We discuss the surgical implications of the distorted cardiac anatomy, along with post-operative complications, which included refractory arrhythmias.

Atrio-ventricular conduction following radiofrequency ablation for atrio-ventricular node reentry tachycardia in children.

AIMS The study was designed to assess atrio-ventricular (AV) conduction with non-invasive methods at least 1 year after radiofrequency ablation (RFA) of the slow pathway for AV node reentry tachycardia. METHODS AND RESULTS Medical records of all patients who underwent RFA before their 18th birthday were reviewed. Patients were brought back for clinical evaluation, an electrocardiogram, an exercise stress test, and ambulatory Holter monitoring. Radiofrequency ablation of the slow pathway above the ostium of the coronary sinus was done in 106 children. No procedure resulted in high degree AV block. Follow-up evaluation was possible in 67 patients (63% of the total cohort) who were brought back to the clinic 1-13.7 years, mean 4.7+/-3.0 years after the procedure. Dizzy spells were reported by 36% of examined patients and 2 patients reported syncope. PR intervals were normal in all but two patients when compared with published normal values. One patient presented with persistent, post-procedural first-degree AV block and another developed new onset, symptomatic second degree AV block 2 years after the procedure and required pacemaker implantation. CONCLUSION Non-invasive testing showed normal PR intervals in a cohort of patients who underwent RFA of the slow pathway in childhood or adolescence. Late AV block occurred in one child. Clinical evaluation more than a year after the procedure is warranted in symptomatic patients.

A Simple Effective Protocol to Increase Prenatal Detection of Critical Congenital Heart Disease

Prenatal diagnosis of congenital heart disease (CHD) during routine obstetric sonography has been aptly named the sonographer’s Achilles heel. Although CHD occurs more commonly than any other major congenital abnormality, the detection rate remains low. The goal of this study was to improve the prenatal diagnosis of CHD during routine obstetric sonography through the development and implementation of a simple and effective screening protocol.

Heterotaxy Syndrome and Intestinal Rotation Abnormalities

In this study, 72% of evaluated infants with HS had IRAs. There was no failure of expectant management resulting in midgut volvulus. BACKGROUND: Infants with heterotaxy syndrome (HS) have abnormal lateralization of organs along the right-left body axis. Intestinal rotation abnormalities (IRAs) are a potential source of morbidity and mortality. For this study, our objective was to prospectively observe a cohort of infants with HS and determine the incidence and natural history of IRA. METHODS: Infants ≤6 months of age with HS were enrolled in this prospective observational study. Exclusion criteria were other congenital abnormalities that necessitated abdominal surgery. HS was defined as any arrangement of organs that was not situs solitus or situs inversus along with associated congenital heart disease. The investigation for IRA was at the discretion of each participating center. RESULTS: Infants were recruited from January 2012 to December 2016. Thirty-eight infants from 7 institutions were included; 22 infants had right isomerism and 16 infants had left isomerism. Twenty-nine infants (76%) were evaluated for IRAs; 21 of 29 evaluations (72%) were abnormal. Eight infants were investigated because of symptoms, and 21 infants were evaluated routinely. The median age at symptom presentation was 46 days (range: 5–171 days). Seven infants had a Ladd procedure; 4 were prophylactic, with 3 as part of a combined procedure, and 3 were emergent. No child suffered acute midgut volvulus over a median follow-up of 1.6 years (range: 0.06–4.93 years). CONCLUSIONS: IRAs are common in infants with HS. Infants with symptoms presented by 6 months of age. There was no failure of expectant management resulting in midgut volvulus during a median follow-up of 1.6 years.

Pre-symptomatic prediction of morbitidies in preterm infants with patent ductus arteriosus by targeted neonatal echocardiography and brain-type natriuretic peptide

Objectives: Our objective was to compare patent ductus arteriosus (PDA) diameter, PDA score and brain-type natriuretic peptide (BNP) measurements at 48-72 hours of life, for prediction of neonatal morbidities. We hypothesized that use of a PDA score with BNP, may improve pre-symptomatic prediction of PDAs associated with adverse outcomes. Method: Infants < 31 weeks GA were prospectively studied by targeted neonatal echocardiogram (TNE) at 48-72 hours age, composite PDA score and serum BNP assay; the clinical team remained blinded. PDA was independently diagnosed by echocardiography at time of clinical suspicion (6 ± 2 days), and treated at discretion of the clinical team. Primary outcome was survival with one or more of adverse outcomes (intraventricular hemorrhage [IVH], bronchopulmonary dysplasia [BPD], retinopathy of prematurity [ROP], necrotizing enterocolitis [NEC]). Results: A PDA was present in 56 of 70 infants studied at 48-72 hours; 30 were eventually diagnosed with PDA but never required treatment, 19 required medical treatment, 7 surgical ligation. After adjustment for gestation, PDA diameter did not predict any adverse outcome, PDA score was associated with increased risk of any adverse outcome and high BNP was associated with IVH, BPD, or survival with any adverse outcome. Conclusions: Comprehensive PDA evaluation at 48-72 hours of age may predict the subsequent occurrence of adverse outcomes and may be useful to define the PDA treatment threshold.