We aimed to assess cardiac autonomic balance with heart rate variability by using 24-hour Holter electrocardiography and also to assess susceptibility to ventricular arrhythmias by using microvolt T wave alternance in children with attention deficit hyperactivity disorder.

This study was conducted with age- and gender-matched groups of 40 patients taking long-acting methylphenidate for more than a year and 55 healthy controls. Heart rate variability analysis for cardiac autonomic functions and microvolt T wave alternance measurements for susceptibility to ventricular arrhythmias were evaluated by 24-hour Holter electrocardiography.

The mean age 10.9 ± 2.7 years, mean duration of therapy 22.76 months, and mean methylphenidate doses were 37.64 mg/day. The study group had considerably higher rMSSD, higher HF, and a lower LF/HF ratio (respectively, p : 0.02, p : 0.001 and p : 0.01). While parasympathetic activity parameters were elevated, sympathetic activity parameters were low during the sleep period. Increase in the microvolt T wave alternance values of the study group was not found to be statistically significant (p > 0.05).

In children taking long-acting methylphenidate, the autonomic balance was shown to be in favour of the parasympathetic system. Determination of the vulnerability to life-threatening ventricular arrhythmias has been evaluated for the first time in children with attention deficit hyperactivity disorder. Accordingly, microvolt T-wave alternance values give the notion that drug use is safe.

To determine whether gross motor scores of toddlers after complex cardiac surgery were different from fine motor scores and were adequately represented by motor composite scores and, whether acute care predictors and chronic childhood health markers of gross motor scores differed from those of fine motor.

This prospective inception-cohort outcomes study included 171 toddlers after complex cardiac surgery with cardiopulmonary bypass at age <6 months, born in Northern Alberta from 2009 to 2019, and without known chromosomal abnormalities. At a mean (standard deviation) age of 21.7 (3.7) months, the Bayley Scales of Infant and Toddler Development-III determined motor composite and scaled scores (normative values, 100 (15), 10 (3), respectively). The same variables from surgery and assessment were analysed using multivariate regression to predict gross and fine motor scores; results expressed as effect size (95% confidence interval) with % variance.

Composite, fine, and gross motor scores were 89.7 (14.2), 9.4 (2.5), and 7.2 (2.7), respectively. Predictive variables accounted for 21.2% of the variance for fine motor, and 36.9% for gross motor. Multivariate analysis for gross motor scores included toddlers need for cardiac medication, effect size (95% confidence interval) −0.801 (−1.62, −0.02), gastrostomy, −1.35 (−2.39, −0.319), and single ventricle, −0.93 (−1.71, −0.15). These same variables did not predict fine motor scores.

Gross motor skills commonly were lower than fine motor skills for toddlers after complex cardiac surgery. Predictors for gross motor scores differed from fine motor scores. Separate reporting of gross motor scores could lead to improved identification of predictors of delay and to optimised early intervention.

To describe a method of reducing the risk of sternal wound infection after sternotomy in children with a pre-existing tracheostomy. To report our outcomes using this method from 1 January, 2013 to 31 August, 2023.

We describe a method for temporarily occluding the tracheal stoma with a removable implant with the primary goal of reducing the risk of sternotomy wound infection by preventing soilage due to tracheostomal secretions. We then performed a retrospective review of all children who underwent temporary tracheostomal occlusion between 1 January, 2013 and 31 August, 2023 at our quaternary care children’s hospital. Clinical variables were extracted from the hospital medical records. The rates of antibiotic use and minor and major complications during the period when the stoma plug was in place were recorded.

Totally, 19 patients underwent tracheal stoma plugging prior to sternotomy and were included in our analysis. There were two cases of sternal wound infection; one case occurred while the stoma plug was in place, and one developed four days following plug removal. There was one minor complication, with one patient requiring stoma revision via serial dilation at bedside at the time of recannulation. There were no deaths.

Temporary occlusion of the tracheal stoma with an impermeable plug is a viable option for reducing the risk of sternal wound infection in children with a pre-existing tracheostomy who are undergoing sternotomy.

Parents of children with CHD face several barriers when trying to access the services needed to support their child’s development. In fact, current developmental follow-up practices may not identify developmental challenges in a timely manner and important opportunities for interventions may be lost. This study aimed to explore the perspectives of parents of children and adolescents with CHD with respect to developmental follow-up in Canada.

Interpretive description was used as a methodological approach for this qualitative study. Parents of children aged 5–15 years with complex CHD were eligible. Semi-structured interviews that aimed to explore their perspectives regarding their child’s developmental follow-up were conducted.

Fifteen parents of children with CHD were recruited for this study. They expressed that the lack of systematic and responsive developmental follow-up services and limited access to resources to support their child’s development placed an undue burden on their families, and as a result, they needed to assume new roles as case managers or advocates to address these limitations. This additional burden resulted in a high level of parental stress, which, in turn, affected the parent–child relationship and siblings.

The limitations of the current Canadian developmental follow-up practices put undue pressure on the parents of children with complex CHD. The parents stressed the importance of implementing a universal and systematic approach to developmental follow-up to allow for the timely identification of challenges, enabling the initiation of interventions and supports and promoting more positive parent–child relationships.

We report the midterm results of our strategy utilizing transatrial-transpulmonary repair for tetralogy of Fallot at a single institution in a low-middle income country.

Medical records were retrospectively reviewed for 532 consecutive patients who underwent definitive repair of tetralogy of Fallot at our institution from 2010 to 2020.

The median age and weight of patients in the study patients were 11.6 months (interquartile range, 8.6–17.2 months) and 7.5 kg (interquartile range, 6.8-8.8 kg). The pulmonary valve annulus was preserved (no transannular patch) in 398 patients (75%) and a mini-transannular patch was utilized for 134 patients (25%). The overall survival was 98% at 1 year, and 97% at 10-years follow-up, respectively. Longer postoperative ventilation time was the only risk factor correlated to early death (p = 0.004; Odds Risk, 1.04; 95% confidence intervals, 1.01–1.07). Fourteen patients required pulmonary valve replacement (2.6%, 14/532), four required surgical resection to relieve right ventricular outflow tract obstruction (0.8%, 4/532), and freedom from reoperation of the right ventricular outflow tract was 87% at 10 years. The only risk factor for right ventricular outflow tract reoperation was a postoperative systolic pressure gradient through the right ventricular outflow tract of greater than 50 mmHg (p < 0.001; HR, 47; 95% confidence intervals, 9.1–244). In total, 94.6% (471/489) of the patients were asymptomatic at the latest follow-up without significant arrhythmia.

At our institution in an low-middle income country, the transatrial-transpulmonary repair for tetralogy of Fallot has excellent midterm results with few reoperations required. Close long-term follow-up is essential for patients who undergo repair with a mini-transannular patch and may eventually require pulmonary valve replacement.

Patients with Fontan failure are high-risk candidates for heart transplantation and other advanced therapies. Understanding the outcomes following initial heart failure consultation can help define appropriate timing of referral for advanced heart failure care.

This is a survey study of heart failure providers seeing any Fontan patient for initial heart failure care. Part 1 of the survey captured data on clinical characteristics at the time of heart failure consultation, and Part 2, completed 30 days later, captured outcomes (death, transplant evaluation outcome, and other interventions). Patients were classified as “too late” (death or declined for transplant due to being too sick) and/or “care escalation” (ventricular assist device implanted, inotrope initiated, and/or listed for transplant), within 30 days. “Late referral” was defined as those referred too late and/or had care escalation.

Between 7/2020 and 7/2022, 77 Fontan patients (52% inpatient) had an initial heart failure consultation. Ten per cent were referred too late (6 were too sick for heart transplantation with one subsequent death, and two others died without heart transplantation evaluation, within 30 days), and 36% had care escalation (21 listed ± 5 ventricular assist device implanted ± 6 inotrope initiated). Overall, 42% were late referrals. Heart failure consultation < 1 year after Fontan surgery was strongly associated with late referral (OR 6.2, 95% CI 1.8–21.5, p=0.004).

Over 40% of Fontan patients seen for an initial heart failure consultation were late referrals, with 10% dying or being declined for transplant within a month of consultation. Earlier referral, particularly for those with heart failure soon after Fontan surgery, should be encouraged.

The use of peritoneal catheters for prophylactic dialysis or drainage to prevent fluid overload after neonatal cardiac surgery is common in some centres; however, the multi-centre variability and details of peritoneal catheter use are not well described.

Twenty-two-centre NEonatal and Pediatric Heart Renal Outcomes Network (NEPHRON) study to describe multi-centre peritoneal catheter use after STAT category 3–5 neonatal cardiac surgery using cardiopulmonary bypass. Patient characteristics and acute kidney injury/fluid outcomes for six post-operative days are described among three cohorts: peritoneal catheter with dialysis, peritoneal catheter with passive drainage, and no peritoneal catheter.

Of 1490 neonates, 471 (32%) had an intraoperative peritoneal catheter placed; 177 (12%) received prophylactic dialysis and 294 (20%) received passive drainage. Sixteen (73%) centres used peritoneal catheter at some frequency, including six centres in >50% of neonates. Four centres utilised prophylactic peritoneal dialysis. Time to post-operative dialysis initiation was 3 hours [1, 5] with the duration of 56 hours [37, 90]; passive drainage cohort drained for 92 hours [64, 163]. Peritoneal catheter were more common among patients receiving pre-operative mechanical ventilation, single ventricle physiology, and higher complexity surgery. There was no association with adverse events. Serum creatinine and daily fluid balance were not clinically different on any post-operative day. Mortality was similar.

In neonates undergoing complex cardiac surgery, peritoneal catheter use is not rare, with substantial variability among centres. Peritoneal catheters are used more commonly with higher surgical complexity. Adverse event rates, including mortality, are not different with peritoneal catheter use. Fluid overload and creatinine-based acute kidney injury rates are not different in peritoneal catheter cohorts.

To examine the impact of a Patient and Public Involvement exercise on the development of British Congenital Cardiac Association Fetal Cardiology Standards 2021.

Open-ended, semi-structured interviews were undertaken to inform the design of a study to improve the quality of parents’ experiences during antenatal and perinatal care of their child with CHD. This Patient and Public Involvement exercise was used to inform the final version of the drafted ‘Standards’.

One-on-one interviews with parents who responded to a request on the closed Facebook page of the user group “Little Hearts Matter”: “Would you be interested in helping us to design a study about parents’ experience on learning that their child had CHD”?

Parents of children with single ventricle CHD.

Twenty-one parents (18 mothers, 3 fathers) participated. Parents responses were reported to have variably reinforced, augmented, and added specificity in the later stages of drafting to six of the seven subsections of Section C Information and Support for Parents including: “At the time of the Scan”; “Counselling following the identification of an abnormality”; “Written information/resources”; “Parent support”; “Communication with other teams and ongoing care”; and “Bereavement support”.

This Patient and Public Involvement exercise successfully informed the development of Standards after the initial drafting. It contributed to the establishment of face validity of the ‘Standards’, especially when consistent with what is reported in the literature. Further research is needed to explore approaches to involving and standardising Patient and Public Involvement in the development of clinical standards.

Chronic total coronary occlusion is among the most complex coronary artery diseases. Elevated homocysteine is a risk factor for coronary artery diseases. However, few studies have assessed the relationship between homocysteine and chronic total coronary occlusion.

1295 individuals from Southwest China were enrolled in the study. Chronic total coronary occlusion was defined as complete occlusion of coronary artery for more than three months. Homocysteine was divided into quartiles according to its level. Univariate and multivariate logistic regression models, receiver operating characteristic curves, and subgroup analysis were applied to assess the relationship between homocysteine and chronic total coronary occlusion.

Subjects in the higher homocysteine quartile had a higher rate of chronic total coronary occlusion (P < 0.001). After adjustment, the odds ratio for chronic total coronary occlusion in the highest quartile of homocysteine compared with the lowest was 1.918 (95% confidence interval 1.237–2.972). Homocysteine ≥ 15.2 μmol/L was considered an independent indicator of chronic total coronary occlusion (odds ratio 1.53, 95% confidence interval 1.05–2.23; P = 0.0265). The area under the receiver operating characteristic curve was 0.659 (95% confidence interval, 0.618–0.701; P < 0.001). Stronger associations were observed in elderly and in those with hypertension and diabetes.

Elevated homocysteine is significantly associated with chronic total coronary occlusion, particularly in elderly and those with hypertension and diabetes.

To analyse surgical outcomes of pulmonary artery coarctation in univentricular hearts, focusing on surgical indications and optimal timing.

We retrospectively reviewed 49 patients with pulmonary artery coarctation in univentricular hearts treated at our institution between 1993 and 2022. Twenty-eight patients were diagnosed before first-stage palliation. Of these, 14 underwent systemic-pulmonary shunt only as first-stage palliation (Group 1), and 14 underwent systemic-pulmonary shunt plus surgical pulmonary artery plasty as first-stage palliation (Group 2). Twenty-one patients diagnosed after first-stage palliation underwent surgical pulmonary artery plasty at the time of bidirectional Glenn procedure (Group 3).

Follow-up period after initial palliation was 6±8 years. The Fontan procedure was successful in 35 patients (71%) aged 28±26 months (range 18–139). Freedom from interstage death (Group 1, 53%; Group 2, 85%; Group 3, 93%) and interstage reintervention (Group 1, 50%; Group 2, 75%; Group 3, 73%) rates were significantly lower in Group 1 (p = 0.01). Five and four patients in Group 1 and Group 3, respectively, needed additional shunts before the bidirectional Glenn procedure. In Group 1, one patient with a non-confluent pulmonary artery achieved hemi-lung Fontan circulation. In Group 2, one patient suffering with a non-confluent pulmonary artery could not achieve Fontan circulation, whereas another patient with pulmonary venous obstruction achieved hemi-lung Fontan circulation.

Surgical pulmonary artery plasty performed at first-stage palliation improved outcomes of pulmonary artery coarctation in univentricular hearts, particularly when pulmonary artery coarctation had already progressed during the neonatal period or early infancy.

This study describes the illness burden in the first year of life for children with single-ventricle heart disease, using the metric of days alive and out of hospital to characterize morbidity and mortality.

This is a retrospective single-centre study of single-ventricle patients born between 2005 and 2021 who had their initial operation performed at our institution. Patient demographics, anatomical details, and hospitalizations were extracted from our institutional single-ventricle database. Days alive and out of hospital were calculated by subtracting the number of days hospitalized from number of days alive during the first year of life. A multivariable linear regression with stepwise variable selection was used to determine independent risk factors associated with fewer days alive and out of hospital.

In total, 437 patients were included. Overall median number of days alive and out of hospital in the first year of life for single-ventricle patients was 278 days (interquartile range 157–319 days). In a multivariable analysis, low birth weight (<2.5kg) (b = −37.55, p = 0.01), presence of a dominant right ventricle (b = −31.05, p = 0.01), moderate-severe dominant atrioventricular valve regurgitation at birth (b = −37.65, p < 0.05), index hybrid Norwood operation (b = −138.73, p < 0.01), or index heart transplant (b = −158.41, p < 0.01) were all independently associated with fewer days alive and out of hospital.

Children with single-ventricle heart defects have significant illness burden in the first year of life. Identifying risk factors associated with fewer days alive and out of hospital may aid in counselling families regarding expectations and patient prognosis.

CHD is a lifelong condition with a significant burden of disease to patients and families. With increased survival, attention has shifted to longer-term outcomes, with a focus on social determinants of health. Among children with CHD, socioeconomic status is associated with disparities in outcomes. Household material hardship is a concrete measure of poverty and may serve as an intervenable measure of socioeconomic status.

A longitudinal survey study was conducted at multiple time points (at acute hospitalisation, then 12–24 months later in the chronic phase) to determine the prevalence of household material hardship among parents of children with advanced heart disease and quality of life during long-term follow-up.

The analytic cohort was 160 children with a median patient age of 1 year (IQR 1,4) with 54% of patients <2 years. During acute hospitalisation, over one-third of families reported household material hardship (37%), with significantly lower household material hardship in the chronic phase at 16% (N = 9 of 52). For parents reporting household material hardship during acute hospitalisation, 50% had resolution of household material hardship by the chronic phase. Household material hardship-exposed children were significantly more likely to be publicly insured (56% versus 20%, p = 0.03) with lower quality of life than those without household material hardship (64% versus 82%, p = 0.013).

The burden of heart disease during the chronic phase of illness is high. Household material hardship may serve as a target to ensure equity in the care and outcomes of CHD patients and their families.

The present study aimed to investigate the relationship between unexplained chest pain in children with parents’ mental problems, parental attitudes, family functionality, and the child’s mental problems.

A total of 433 children (between 11 and 18 years of age) applied to the Pediatric Cardiology Outpatient Clinic due to chest pain in the last year. A clinical interview was conducted by a child psychiatrist with 43 patients and 33 controls included in the study due to unexplained chest pain.

Family history of physical illness was significantly higher in the chest pain group. When evaluated in terms of psychosocial risk factors, life events causing difficulties, derangement in the family, loss of a close person, and exposure to violence were statistically significantly higher in the group with chest pain. Mental disorders were observed in 67.4% of the children in the chest pain group as a result of the clinical interview. The total score of the DSM-5 somatic symptoms scale, which evaluates other somatic complaints in the chest pain group, was also significantly higher. When the family functions of both groups were evaluated, communication, emotional response, behaviour control, and general functions sub-dimensions were statistically significantly higher in families in the chest pain group.

We recommend that psychiatric evaluation be included in diagnostic research to prevent unnecessary medical diagnostic procedures in children describing unexplained chest pain, as well as to prevent the potential for diagnosing mental disorders in both children and adults.

Childhood obesity tends to persist into adulthood and associated with increase in developing ischemic and non-ischemic cardiovascular diseases. We aimed to evaluate the effect of obesity on cardiac functions, atrial electromechanical coupling, and heart rate response, which are considered to be predictors of atrial fibrillation and sudden cardiac arrest.

Study population included 52 obese children and 52 healthy controls. We performed 12-lead electrocardiography, echocardiographic examination, and treadmill exercise testing. Mitral, septal, and tricuspid segments were analysed by tissue Doppler imaging.

Myocardial performance index (p = 0.011, p < 0.001, and p = 0.001, respectively) was higher and E'/A' ratio (p = 0.011, p < 0.001, and p < 0.001, respectively) was lower in obese group than controls. Atrial electromechanical coupling was longer in the obese group at all three segments (p < 0.001, p = 0.009, and p = 0.04, respectively). They had significantly longer interatrial (p < 0.001) and intra-atrial (p = 0.003) electromechanical conduction delay. While chronotropic index was similar between two groups, heart rate reserve was lower in obese children than controls (p = 0.043). The 1st- and 2nd-minute heart rate recovery indices of the obese group were lower compared to controls (p < 0.001 and p = 0.03, respectively). Body mass index was positively correlated with intra- and inter-atrial conduction times, whereas it was negatively correlated with heart rate recovery indices.

We showed a deterioration in the diastolic function, atrial conduction, and heart rate response properties in children with obesity. Given the prognostic importance of these parameters, obese patients are might be at risk for atrial fibrillation and severe dysrhythmias from a young age.

The aims of this study were to evaluate the 16-year experience with  arterial switch operation at Beijing Children’s Hospital and to determine early and late mortality and late morbidity, to explore risk factors for late complications and reintervention, and finally to evaluate whether the neoaortic sinotubular junction reconstruction technique reduces late complications of arterial switch operation.

The clinical data of 185 patients with transposition of the great arteries who underwent arterial switch operation in Beijing Children’s Hospital from January 2006 to January 2022 and 30 patients who underwent modified arterial switch operation with neoaortic sinotubular junction reconstruction technique in Fuwai Hospital during the same period were retrospectively analysed. Propensity score matching was also used to match the neoaortic sinotubular junction reconstruction patients in Fuwai Hospital with 30 non-neoaortic sinotubular junction reconstruction patients in Beijing Children’s Hospital.

There were 13 early deaths (7.03%) and five late deaths (3.01%). Nineteen patients (11.45%) developed new aortic valve regurgitation and 28 patients (16.87%) developed aortic root dilation. Late right ventricular outflow tract obstruction occurred in 33 patients (19.88%). Late reintervention occurred in 18 cases (10.84%). Multivariate analysis showed that aorto-pulmonary diameter mismatch, previous pulmonary artery banding, and mild moderate or above new aortic valve regurgitation at discharge were independent risk factors for late new aortic valve regurgitation and aortic root dilation. Low surgical weight was an independent risk factor specific to new aortic valve regurgitation, and bicuspid native pulmonary valve was an independent risk factor specific to aortic root dilation. Older surgical age and aortic root dilation were independent risk factors for late right ventricular outflow tract obstruction. Older surgical age, operation before 2014, late right ventricular outflow tract obstruction, and late aortic root dilation were independent risk factors for late intervention. Propensity score matching showed that new aortic valve regurgitation and aortic root dilation were not followed up in the neoaortic sinotubular junction reconstruction group, while seven cases of aortic root dilation and five cases of new aortic valve regurgitation occurred in the non-neoaortic sinotubular junction reconstruction group, respectively, and the differences were statistically significant (P = 0.003; P = 0.015).

The increased incidence of new aortic valve regurgitation, aortic root dilation, and right ventricular outflow tract obstruction as children age is a major concern outcome in the future and may mean more late reintervention. neoaortic sinotubular junction reconstruction technique may reduce the incidence of new aortic valve regurgitation and aortic root dilation, and improve the late prognosis of arterial switch operation. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with aorto-pulmonary diameter mismatch, previous pulmonary artery banding, mild new aortic valve regurgitation at discharge, low surgical weight, and bicuspid native pulmonary valve structures.

Understanding parents’ communication preferences and how parental and child characteristics impact satisfaction with communication is vital to mitigate communication challenges in the cardiac ICU.

This cross-sectional survey was conducted from January 2019 to March 2020 in a paediatric cardiac ICU with parents of patients admitted for at least two weeks. Family satisfaction with communication with the medical team was measured using the Communication Assessment Tool for Team settings. Clinical characteristics were collected via Epic, Pediatric Cardiac Critical Care Consortium local entry and Society for Thoracic Surgeons Congenital Heart Surgery Databases. Associations between communication score and parental mood, stress, perceptions of clinical care, and demographic characteristics along with patient demographic and clinical characteristics were examined. Multivariable ordinal models were conducted with characteristics significant in bivariate analysis.

In total, 93 parents of 84 patients (86% of approached) completed surveys. Parents were 63% female and 70% White. Seventy per cent of patients were <6 months old at admission, 25% had an extracardiac abnormality, and 80% had a cardiac surgery this admission. Parents of children with higher pre-surgical risk of mortality scores (OR 2.875; 95%CI 1.076–7.678), presence of surgical complications (72 [63.0, 75.0] vs. 64 [95%CI 54.6, 73] (p = 0.0247)), and greater satisfaction with care in the ICU (r = 0.93922; p < 0.0001) had significantly higher communication scores.

These findings can prepare providers for scenarios with higher risk for communication challenges and demonstrate the need for further investigation into interventions that reduce parental anxiety and improve communication for patients with unexpected clinical trajectories

Early diagnosis of acute coronary syndrome is more and more important because of its mortality and morbidity. Hypertension is one of the pathogenesis of acute coronary syndrome, which often leads to stenosis and ischaemia. Ischaemia-modified albumin is sensitive for the occurrence of ischaemia, which attracted us in the significance of ischaemia-modified albumin in patients with chest pain, especially patients complicated with hypertension.

In total, 200 patients with acute chest pain were included in the study. According to the diagnostic criteria, patients were divided into acute coronary syndrome group and non-ischaemic chest pain group. Cardiac biomarkers were measured with 30 minutes in emergency department, including cardiac troponin T, creatine kinase MB, and ischaemia-modified albumin. Receiver operating characteristic curve (ROC) analysis was used for the sensitivity and specificity of ischaemia-modified albumin in the early diagnosis of acute coronary syndrome. Comparisons between ischaemia-modified albumin and cardiac Troponin T were done between groups.

The demographics in two groups were not significantly different in most aspects. Compared with non-ischaemic chest pain group, serum levels of ischaemia-modified albumin and cardiac Troponin T were significantly higher in acute coronary syndrome group. ROC analysis showed that ischaemia-modified albumin had a good sensitivity and specificity in early diagnosis of acute coronary syndrome. The level of ischaemia-modified albumin in acute coronary syndrome patients with hypertension was higher than that in non-ischaemic chest pain patients.

In patients complained with acute chest pain, the serum measurement of ischaemia-modified albumin is potential valuable for the early diagnosis of acute coronary syndrome, especially combined with ECG. The serum level of ischaemia-modified albumin in acute coronary syndrome patients is significantly associated with hypertension.

Family-centred rounds benefit families and clinicians and improve outcomes in general paediatrics, but are understudied in subspecialty settings. We sought to improve family presence and participation in rounds in a paediatric acute care cardiology unit.

We created operational definitions for family presence, our process measure, and participation, our outcome measure, and gathered baseline data over 4 months of 2021. Our SMART aim was to increase mean family presence from 43 to 75% and mean family participation from 81 to 90% by 30 May, 2022. We tested interventions with iterative plan-do-study-act cycles between 6 January, 2022 and 20 May, 2022, including provider education, calling families not at bedside, and adjustment to rounding presentations. We visualised change over time relative to interventions with statistical control charts. We conducted a high census days subanalysis. Length of stay and time of transfer from the ICU served as balancing measures.

Mean presence increased from 43 to 83%, demonstrating special cause variation twice. Mean participation increased from 81 to 96%, demonstrating special cause variation once. Mean presence and participation were lower during high census (61 and 93% at project end) but improved with special cause variation. Length of stay and time of transfer remained stable.

Through our interventions, family presence and participation in rounds improved without apparent unintended consequences. Family presence and participation may improve family and staff experience and outcomes; future research is warranted to evaluate this. Development of high level of reliability interventions may further improve family presence and participation, particularly on high census days.

Patients with complete atrioventricular canal have a variable clinical course prior to repair. Many patients balance their circulations well prior to elective repair. Others manifest clinically significant pulmonary over circulation early in life and require either palliative pulmonary artery banding or complete repair. The objective of this study was to assess anatomic features that impact the clinical course of patients.

In total, 222 patients underwent complete atrioventricular canal repair between 2012 and 2022 at a single institution. Twenty-seven (12%) patients underwent either pulmonary artery banding (n = 15) or complete repair (n = 12) at less than 3 months of age (Group 1). The remaining 195 (88%) underwent repair after 3 months of age (Group 2). Patient records and imaging were reviewed.

The median post-operative length of stay following complete repair was 25 [7,46] days for those patients in Group 1 and 7 [5,12] days for those in Group 2 (p < 0.0001). There was relative hypoplasia of left-sided structures in Group 1 versus Group 2. Mean z-score for the ascending aorta was −1.2 (±0.8) versus −0.3 (±0.9) (p < 0.0001), the aortic isthmus was −2.1 (±0.8) versus −1.4 (±0.8) (p = 0.005). The pulmonary valve to aortic valve diameter ratio was median 1.47 [1.38,1.71] versus 1.38 [1.17,1.53] (p 0.008).

Echocardiographic evaluation of the systemic and pulmonary outflow of patients with complete atrioventricular canal may assist in predicting the clinical course and need for early repair vs pulmonary artery banding.