Although chylothorax is an uncommon complication following paediatric cardiothoracic surgery, it has significant associated morbidities and increased in-hospital mortality, as well as results in higher costs. A lack of prospective evidence or consensus guidelines for management of chylothorax further hinders optimal management. The aim of this survey was to characterise variations in practice in the management of chylothorax and to identify areas for future research.

A descriptive, observational survey investigating conservative management practices of chylothorax was distributed internationally to health-care professionals in paediatric intensive care and cardiology units. The survey investigated five domains: the first providing general information about health-care professionals and four domains focusing on clinical practice including diet composition and duration.

In total, sixty-four health-care professionals completed the survey, representing 38 organisations from 16 countries. The respondents were dietitians (80%), physicians (19%), and nurses (1%). In Australia and New Zealand, management was most commonly directed by physicians’ preference (67%) as compared to unit protocols in Europe (67%), United States of America (67%), and Other regions (55%). Dietitians in Australia/New Zealand, United Kingdom, and Ireland followed the most restrictive diet therapy recommending <5 g long chain triglyceride fat per day (p < 0.00001). The duration of diet therapy significantly varied between regions: Australia/New Zealand: 4 weeks (36%) and 6 weeks (43%); Europe: 4 weeks (25%) and 6 weeks (57%); and North America: 4 weeks (18%) and 6 weeks (75%) (p < 0.00001).

This survey highlights international variations in practice in the management of chylothorax, particularly with respect to treatment duration and dietary fat restriction. Future research should include a multi-centre randomised controlled trial to inform evidence-based practice and reduce morbidity, particularly poor growth.

Early identification of infants with CHD at heightened risk of developmental delays can inform surveillance priorities. This study investigated pre-operative and post-operative neuromotor performance in infants undergoing open-heart surgery, and their developmental status at 6 months of age, to identify risk factors and inform care pathways.

Infants undergoing open-heart surgery before 4 months of age were recruited into a prospective cohort study. Neuromotor performance was assessed pre-operatively and post-operatively using the Test of Infant Motor Performance and Prechtl’s Assessment of General Movements. Development was assessed at 6 months of age using the Ages and Stages Questionnaire third edition. Pre-operative and post-operative General Movements performance was compared using McNemar’s test and test of infant motor performance z-scores using Wilcoxon’s signed rank test. Risk factors for delayed development at 6 months were explored using logistic regression.

Sixty infants were included in this study. In the 23 (38%) infants. A total of 60 infants were recruited. In the 23 (38%) infants assessed pre-operatively, there was no significant difference between pre- and post-operative performance on the GMs (p=0.63) or TIMP (p=0.28). At discharge, 15 (26%) infants presented with abnormal GMs, and the median TIMP z-score was −0.93 (IQR: −1.4 to −0.69). At 6 months, 28 (52.8%) infants presented with gross motor delay on the ASQ-3, significantly negatively associated with gestational age (p=0.03), length of hospital stay (p=0.04) and discharge TIMP score (p=0.01).

Post-operative assessment using the GMs and TIMP may be useful to identify infants requiring individualised care and targeted developmental follow-up. Long-term developmental surveillance beyond 6 months of age is recommended.

Acute pulmonary embolism is a life-threatening condition and rarely occurs in children. In adults, catheter-directed therapy emerges as a potentially safer and effective therapeutic option. However, there is a paucity of data on the safety and efficacy of catheter-directed therapy for pulmonary embolism in children. We report a single-centred experience of catheter-directed therapy for acute pulmonary embolism in children.

This is a retrospective study of children who had no CHD and underwent catheter-directed therapy at Detroit Medical Center during a 12-year period from 2005 to 2017. Demographic and clinical data associated with pulmonary embolism were collected along with the outcome.

A total of nine patients of median age 16 years with the range from 12 to 20 received catheter-directed therapy for sub-massive (n = 6) and massive pulmonary embolism (n = 3). Among nine patients, one patient received Angiojet thrombectomy and balloon angioplasty, whereas eight patients received catheter-directed thrombolysis using tissue plasminogen activator through infusion catheters (n = 3) or EkoSonic ultrasound-accelerated thrombolysis system (n = 5). In four out of five patients treated with EkoSonic, significant clinical improvement was noticed within 24 hours. Among seven patients who survived, two patients had minor gastrointestinal bleeding with median hospital stay of 8 days with the range from 5 to 24 days, and two patients with massive pulmonary embolism died possibly due to delayed institution of catheter-directed therapy.

Catheter-directed therapy with/without EkoSonic is an emerging alternative therapy for sub-massive and massive pulmonary embolism in children. A timely institution of catheter-directed therapy appeared important to improve the outcome.

This research investigated patients who underwent surgery for a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents.

The medical records of 11 patients who underwent aortic surgery for dilatation resulting from a connective tissue disease or inflammatory vasculitis between 2000 and 2017 were retrospectively reviewed.

The median age and body weight of the patients were 9.6 years (range 5.4 months–15.5 years) and 25.8 kg (range 6.8–81.5), respectively. The associated diseases were Marfan syndrome (n = 3), Loeys-Dietz syndrome (n = 3), Kawasaki disease (n = 1), Takayasu arteritis (n = 1), PHACE syndrome (n = 1), tuberous sclerosis (n = 1), and unknown (n = 1). The most common initially affected area was the ascending aorta. During the 66.4 ± 35.9 months of follow-up, two Marfan syndrome patients died, and four patients (one Marfan syndrome and three Loeys-Dietz syndrome) had repeated aortic operation. Except for one patient, the functional class was well maintained in all patients who were followed up.

Cases of surgical treatment for a dilated aorta associated with a connective tissue disease and inflammatory vasculitis are rare in children and adolescents at our institution. Most of the patients in this study showed a tolerable postoperative course. However, the aorta showed progressive dilation over time even after surgical treatment, especially in patients with Loeys-Dietz syndrome. In these patients, close and more frequent regular follow-up is required.

Chylopericardium is the collection of lymph fluid inside the pericardial cavity. The incidence of chylopericardium is very low, as this diagnosis is rarely reported following cardiac procedures in children. While some reports were published worldwide on isolated chylopericardium after cardiac surgeries for diverse reasons, it has never been reported after repair for partial anomalous pulmonary venous return. In addition, management of this diagnosis ends up being surgical with minimal concentration on medical treatment which proved unsuccessful. We present a medical approach with corticosteroids as an effective method to treat isolated chylopericardium.

In this manuscript, we present an approach to treat isolated post-operative chylopericardium in a child following repair of partial anomalous pulmonary venous return. Chylous drainage responded to corticosteroids and completely ceased. There was no need for surgical intervention.

Until now, isolated chylopericardium has never been reported to occur with partial anomalous pulmonary venous return repair. A review of the literature showed that most patients follow a conservative approach consisting of diuretics and non-steroidal anti-inflammatory agents with some of them undergoing surgical re-intervention. With future research on the topic still needed, we hope that this will encourage physicians worldwide to consider administering a trial of corticosteroids as an option to treat chylopericardium.

There is limited experience about transcatheter closure of doubly committed subarterial ventricular septal defects with Amplatzer ductal occluder.

Between March, 2015 and July, 2017, a total of 22 patients with doubly committed subarterial ventricular septal defects received transcatheter closure using Amplatzer ductal occluder and underwent clinical follow-up for at least 6 months.

Device implantation was finally successful in 21 (95.4%) patients despite failed occlusion in one patient and intra-procedural replacement of unsuitable occluders in four (19.0%) patients. In mean 12.3 months of follow-up, there were no major complications (death, aortic valve or sinus rupture, device dislocation or embolisation, grade 2 new-onset aortic regurgitation, etc.), resulting in clinical occlusion success of 95.4%. Mechanical haemolysis occurred in one patient and resolved with medication. Residual shunt was observed in 11 (52.4%) patients (9 mild, 2 moderate-severe) post-procedurally, 14 (66.7%) patients (12 mild, 2 moderate-severe) in hospital stay, and 2 (9.5%) patients (2 mild, 0 moderate-severe) at the last follow-up. Device-induced new-onset aortic regurgitation was found in nine (42.8%) patients (9 mild, 0 moderate-severe) post-procedurally and in hospital stay, which was resolved in two (9.5%) patients and unchanged in seven (33.3%) patients at the last follow-up. Another four (19.0%) patients newly developed mild aortic regurgitation during follow-up.

Transcatheter closure of doubly committed subarterial ventricular septal defects with Amplatzer ductal occluder is technically feasible in the selected patients. However, further study is needed to confirm its long-term clinical outcomes.

Systemic inflammatory response syndrome, which is marked by fever, is a possible complication after open-heart surgery for CHD. The inflammatory response following the use of cardiopulmonary bypass shows similar clinical signs with sepsis. Therefore serial measurements of procalcitonin, an early infection marker, can be helpful to differentiate between sepsis and systemic inflammatory response syndrome.

To evaluate serial levels of procalcitonin in children who underwent open-heart surgery for cyanotic and acyanotic CHD, and identify factors associated with elevated level of procalcitonin.

Children and infants who had open-heart surgery and showed fever within 6 hours after surgery were recruited. Procalcitonin levels were serially measured along with leukocyte and platelet count. Other data were also recorded, including diagnosis, age, body weight, axillary temperature, aortic clamp time, bypass time, duration of mechanical ventilation, risk adjustment for congenital heart surgery score-1, and length of stay in Cardiac ICU. The patients were categorised into cyanotic and acyanotic CHD groups.

High mean of procalcitonin level suggested the presence of bacterial infection. Cyanotic CHD group had significantly higher mean of procalcitonin level compared to acyanotic CHD group in the first two measurements. Both groups had no leukocytosis, though platelet count results were significantly different between the two groups. There was no significant difference of procalcitonin level observed in culture results and adverse outcomes.

Serial procalcitonin measurement can be helpful to determine the cause of fever. Meanwhile other conventional markers such as leukocyte and platelet should be assessed thoroughly.

Fetal echocardiography is the main modality of prenatal diagnosis of CHD. This study was done to describe the trends and benefits associated with prenatal diagnosis of complex CHD at a tertiary care centre.

Retrospective chart review of patients with complex CHD over an 18-year period was performed. Rates of prenatal detection along with early and late infant mortality outcomes were studied.

Of 381 complex CHD patients born during the study period, 68.8% were diagnosed prenatally. Prenatal detection rate increased during the study period from low-50s in the first quarter to mid-80s in the last quarter (p=0.001). Rate of detection of conotruncal anomalies increased over the study period. No infant mortality benefit was noted with prenatal detection.

Improved obstetrical screening indications and techniques have contributed to higher proportions of prenatal diagnosis of complex CHD. However, prenatal diagnosis did not confer survival benefits in infancy in our study.

The objective of this study was to assess differences in myocardial systolic and diastolic function and vascular function in children 2−5 years of age born to diabetic as compared to non-diabetic mothers.

This study was a retrospective cohort conducted in 2016 at The Aga Khan University Hospital, Karachi, Pakistan. It included children between 2 and 5 years of age born to mothers with and without exposure to diabetes in utero (n = 68 in each group) and who were appropriate for gestational age. Myocardial morphology and function using echocardiogram and carotid intima media thickness (cIMT) and pulse wave velocity was performed to evaluate cardiac function as well as macrovascular remodelling in these children. Multiple linear regression was used to compare the groups.

There was no significant difference in cardiac morphology, myocardial systolic and diastolic function, and macrovascular assessment between the exposed and unexposed groups of AGA children. Subgroup analysis demonstrated a significantly decreased mitral E/A ratio in children whose mothers were on medications as compared to those on dietary control (median [IQR] = 1.7 [1.6–1.9] and 1.56 [1.4–1.7], respectively, p = 0.02), and a higher cIMT in children whose mothers were on medication as compared to controls (0.48 [0.44–0.52] and 0.46 [0.44–0.50], respectively, p = 0.03).

In utero exposure to uncontrolled maternal diabetes has an effect on the cardiovascular structure and function in children aged 2−5 years. However, future work requires long-term follow-up from fetal to adult life to assess these changes over the life course.

Developmental care of neonates with CHD is essential for proper neurodevelopment. Measurement of developmental care specific to these neonates is needed to ensure consistent implementation within and across cardiac ICUs. The purpose of this study was to psychometrically test the Developmental Care Scale for Neonates with Congenital Heart Disease, which measures the quality of developmental care provided by bedside nurses to neonates in the cardiac ICU.

Psychometric testing was conducted with 119 cardiac ICU nurses to provide evidence of internal consistency reliability and construct validity. Participants were predominantly young (median = 32 years), white (90%) females (93%) with bachelor’s degrees (78%) and a median experience in the cardiac ICU of 7 years.

Evidence of internal consistency reliability (α =.89) was provided with corrected item-total correlations ranging from .31 to .77. Exploratory factor analysis provided evidence of construct validity as a unidimensional scale, as well as a multidimensional scale consisting of four subscales: creating the external environment, assessment of family well-being, caregiver activities toward the neonate, and basic human needs.

Evidence of reliability and validity of the 31-item Developmental Care Scale for Neonates with Congenital Heart Disease was established with nurses caring for neonates in the cardiac ICU. This instrument will serve as a valuable outcome measure tasked with improving developmental care performance and makes it possible to identify relationships between developmental care performance and neonatal neurodevelopmental outcomes in future research.

Recurrent aortic arch obstruction following the Norwood procedure is recognised as an important complication. Balloon arch angioplasty is associated with a high recoarctation rate.

We sought to evaluate the prevalence and outcome of stent implantation for recoarctation in children following Norwood or Damus–Kaye–Stansel procedure over the past decade at a single national cardiology centre.

Of 114 children who underwent Norwood procedure or Damus–Kaye–Stansel procedure between January 2003 and June 2013, 80 patients survived. Of these 15 children underwent stent implantation for recoarctation. Six of these patients had previous balloon angioplasty. The median age at stent implantation was 4.4 months (range 2–82 months). The median peak aortic arch gradient at catheterisation decreased from 26mmHg (range 10–70mmHg) to 2mmHg (range 0–20mmHg). The median luminal diameter increased from 4.7 mm (range 3.2–7.9 mm) to 8.6 mm (range 6.2–10.9 mm). The median coarctation index increased by 0.49 (range = 0.24–0.64). A Valeo stent was employed in 11 children, a Palmaz Genesis stent in 2 patients, a MultiLink stent in 1 child, and a Jomed covered stent in 1 child. Two factors were associated with the need for stent placement: previous arch angioplasty (p valve < 0.001, χ-square 11.5) and borderline left ventricle (p = 0.04, χ-square = 4.1). Stent migration occurred in one child. There were two deaths related to poor right ventricular systolic function and severe tricuspid regurgitation. Six patients underwent redilation of the stent with no complications.

The prevalence of recurrent aortic arch obstruction following Norwood/Damus–Kaye–Stansel procedure was 18%. Stent implantation is safe and reliably eliminates the aortic obstruction. Redilation can be successfully achieved to accommodate somatic growth or development of stent recoarctation.

Concerns exist over the long-term consequences of subclavian artery ligation in subclavian flap repair for coarctation of the aorta. We sought to analyse upper limb structural and functional performance in adults who have had surgery in childhood for coarctation of the aorta, using either subclavian flap repair or end to end aortic anastomosis.

Two-group observational design using anatomical and upper limb functional performance measures. Purposive sampling from our specialist adult congenital heart disease database of patients who received subclavian flap repair or end to end anastomosis for coarctation of the aorta as children. Upper limb measurements were completed using MRI and blood flow velocity with ultrasound imaging. Bilateral standardised upper limb functional testing of assessment of strength, dexterity and a standardised self-report of upper limb disability was completed.

Eighteen right-handed patients, 9 with subclavian repair, (38 ± 12 years, 78% males) were studied. Age at repair was 4.7 ± 5.9 years; mean time from initial repair 32 ± 9 years. The subclavian group had a larger difference between right and left when compared the end to end anastomosis group in: lower arm muscle mass (94.5 ± 42.3 mls versus 37.8 ± 94.5 mls, p = 0.008), lower arm maximal cross-sectional area, (5.9 ± 2.8 cm2 versus 2.9 ± 2.6 cm2, p = 0.038) and grip strength (14.7 ± 8.3 lbs versus 5.9 ± 5.3 lbs, p = 0.016) There were no significant functional differences between groups.

In adults with repaired coarctation of the aorta, those with subclavian flap repair had a greater right to left arm muscle mass and grip strength differential when compared to those with end to end anastomosis repair.

The Fontan procedure is the final stage of surgical palliation for a single-ventricle circulation. Significant complications are common including rhythm disturbance necessitating implantation of a permanent pacemaker. This has been widely considered a negative prognostic indicator.

This single-centre, retrospective case control study involved all patients who underwent the Fontan procedure at the Leeds Congenital Heart Unit between 1990 and 2015 and have had regular follow-up in Yorkshire and Humber, United Kingdom. 167 Fontan patients were identified of which 2 were excluded for having a pre-procedure pacemaker. Of the remainder, 23 patients required a pacemaker. Outcomes were survival, early and late complications, need for further intervention and oxygen saturation in long-term follow-up.

There was no difference in survival (30-day survival pacemaker 92.6%, sinus rhythm 90.5%, p = 0.66, 1-year pacemaker 11.1%, sinus rhythm 10.1%, p = 1). The pacemaker group was more likely to have cerebral or renal complications in the first-year post-procedure (acute kidney injury: sinus rhythm 0.8%, pacemaker 19.1%, p = 0.002). No difference was observed in longer term complications including protein losing enteropathy (sinus rhythm 3.5%, pacemaker 0% p = 1). There was no difference in saturations between the two groups at follow-up. Paced patients were more likely to have required further intervention, with a higher incidence of cardiopulmonary bypass procedures (sinus rhythm 6.3%, pacemaker 35%, p < 0.001).

Despite an increase in early complications and the need for further interventions, pacemaker requirement does not appear to affect long-term survival following the Fontan procedure.

To evaluate the health status and quality of life of young patients who had cone reconstruction for Ebstein anomaly.

We reviewed all patients who had cone reconstruction from 2007 to 2016 at our institution. Prospective surveys were mailed to all eligible patients. Quality of life was assessed using the PedsQL 4.0 Generic Core Scales, including four domains: physical, emotional, social, and school functioning.

Of 116 eligible patients, 72 (62%) responded. About 96% reported their health as excellent or good, and 52% were symptom-free. Only 37% of patients were taking any medications, the most common of which was aspirin (30%). Only 19% had been hospitalised for cardiac reasons following cone reconstruction. The average self-reported quality of life was 85.3/100, whereas the average parent proxy-reported quality of life was 81.8/100. There was no difference by self or parent proxy-report in quality of life between cone reconstruction patients and healthy children; however, quality of life was significantly better compared with children with other chronic health conditions. By self-report and parent proxy-report, 15.1 and 16.7% of patients were deemed “at risk” for reduced quality of life, respectively. Socially, 63/64 (98%) patients over 5 years old were either full-time students or working full-time.

Children with Ebstein anomaly following cone reconstruction have excellent quality of life comparable with healthy peers and significantly better than other children with chronic health conditions. Families of children with Ebstein anomaly can expect excellent quality of life, long-term health status, and social functioning following cone reconstruction.