Significant atrioventricular valve regurgitation at diagnosis in single-ventricle patients has been associated with mortality and morbidity. However, longitudinal data on the effect of valve regurgitation at diagnosis on outcomes in the era of surgical valve interventions are scarce.

This is a retrospective review of single-ventricle patients admitted to a regional centre from 2005 to 2008. Data were reviewed from birth to 18 months, and association of atrioventricular valve regurgitation at diagnosis with mortality and morbidity was evaluated.

A total of 118 patients were studied, 73% with a single right ventricle. At diagnosis, 37 patients (31%) had mild, 5 (4%) had mild to moderate, and 4 (3%) had ≥ moderate atrioventricular valve regurgitation. Moderate or greater valve regurgitation was associated with mortality (HR 5.51, 95% CI 1.24–24.61, p = 0.025), and all four patients with ≥ moderate valve regurgitation died. However, valve regurgitation was not associated with mortality for left ventricle patients. In all, 12 patients (10%) had surgical atrioventricular valve interventions. There were no independent predictors of valve intervention, and no patient having an intervention had > mild valve regurgitation at diagnosis. There was no association between valve regurgitation and days of hospitalisation or chest tube drainage.

Significant atrioventricular valve regurgitation at diagnosis remains a risk factor for mortality in single-ventricle patients, although it may be less important for single left ventricle patients. However, it is not associated with increased morbidity or surgical atrioventricular valve intervention in survivors. Reliably predicting surgical atrioventricular valve intervention remains a challenge in single-ventricle patients.

Adults with simple congenital heart defects (CHD) have increased risk of neurodevelopmental challenges including executive dysfunction. It is unknown if the executive dysfunction is universal or if it is driven by dysfunction in specific clinical subscales and how it might affect psychosocial aspects of everyday life.

The self-reported and informant-reported executive function of adults with an average age of 26 ± 5 (range 18–41) who underwent childhood surgery for atrial septal defects (n = 34) or ventricular septal defects (n = 32) and matched controls (n = 40) were evaluated using the Behavior Rating Inventory of Executive Functions - Adult version (BRIEF-A).

The CHD group reported having more executive dysfunction than controls in all BRIEF-A clinical subscales (p < 0.020) and more than their informants reported on their behalf (p < 0.006). The CHD group had received three times more special teaching (44% compared to 16%) and pedagogical psychological counselling (14% compared to none) and had a three times higher occurrence of psychiatric disorders than controls (33% compared to 11%). Lower educational levels and psychiatric disorders were associated with higher BRIEF-A scores (p < 0.03).

Adults operated for septal defects in childhood report more challenges with all aspects of the executive functions than controls and more than relatives are aware of.

To assess the cardiac catheterisation findings of all children in whom cardiac magnetic resonance imaging found great artery stenosis.

We conducted a retrospective analysis of all 45 consecutive children with congenital cardiac disease who were undergoing cardiac catheterisation for intervention on cardiac magnetic resonance-defined great vessel stenosis, between January, 2006 and August, 2008.

Following cardiac magnetic resonance, 60 significant great vessel stenoses were identified and referred to cardiac catheterisation for intervention. All patients were catheterised within a median and interquartile range of 84 and 4–149 days, respectively, of cardiac magnetic resonance. At cardiac catheterisation, the children were aged 11.5 years – with an interquartile range of 3.8–16.9 years – and weighed 34 kilograms – with an interquartile range of 15–56 kilograms. Comparing cardiac magnetic resonance and cardiac catheterisation findings, 53 (88%) findings were concordant and seven were discordant. In six of seven (86%) discordant observations, cardiac magnetic resonance defined moderate–severe great vessel stenosis – involving three branch pulmonary arteries and three aortas. This was not confirmed by cardiac catheterisation, which revealed mild stenoses and haemodynamic gradients insufficient for intervention. In one patient, a mild, proximal right pulmonary artery narrowing was found at cardiac catheterisation, which was not mentioned in the cardiac magnetic resonance report. There was no difference between discordant and concordant groups on the basis of patient age, weight, interval between cardiac magnetic resonance and cardiac catheterisation, or type of lesion.

Invasive assessment confirmed cardiac magnetic resonance-diagnosed great vessel stenosis in the majority of this cohort. The predominant discordant finding was lower catherisation gradient than predicted by morphologic and functional cardiac magnetic resonance assessment. Flow volume diversion – for example, unilateral pulmonary artery stenosis – and anaesthetic effects may account for some differences. Prospective refinement of cardiac magnetic resonance and interventional data may further improve the validity of non-invasive imaging thresholds for intervention.

Evidence shows that the health of the work environment impacts staff satisfaction, interdisciplinary communication, and patient outcomes. Utilising the American Association of Critical-Care Nurses’ Healthy Work Environment standards, we developed a daily assessment tool.

The Relative Environment Assessment Lens (REAL) Indicator was developed using a consensus-based method to evaluate the health of the work environment and to identify opportunities for improvement from the front-line staff. A visual scale using images that resemble emoticons was linked with a written description of feelings about their work environment that day, with the highest number corresponding to the most positive experience. Face validity was established by seeking staff feedback and goals were set.

Over 10 months, results from the REAL Indicator in the cardiac catheterisation laboratory indicated an overall good work environment. The goal of 80% of the respondents reporting their work environment to be “Great”, “Good”, or “Satisfactory” was met each month. During the same time frame, this goal was met four times in the cardiovascular operating room. On average, 72.7% of cardiovascular operating room respondents reported their work environment to be “Satisfactory” or better.

The REAL Indicator has become a valuable tool in assessing the specific issues of the clinical area and identifying opportunities for improvement. Given the feasibility of and positive response to this tool in the cardiac catheterisation laboratory, it has been adopted in other patient-care areas where staff and leaders believe that they need to understand the health of the environment in a more specific and frequent time frame.

Systemic right ventricular systolic dysfunction is common late after atrial switch surgery for transposition of the great arteries. Total isovolumic time is the time that the ventricle is neither ejecting nor filling and is calculated without relying on geometric assumptions. We assessed resting total isovolumic time in this population and its relationship to exercise capacity.

A total of 40 adult patients with transposition of the great arteries after atrial switch – and 10 healthy controls – underwent transthoracic echocardiography and cardiopulmonary exercise testing from January, 2006 to January, 2009. Resting total isovolumic time was measured in seconds per minute: 60 minus total ejection time plus total filling time.

The mean age was 31.6 plus or minus 7.6 years, and 38.0% were men. There were 16 patients (40%) who had more than or equal to moderate systolic dysfunction of the right ventricle. Intra- and inter-observer agreement was good for total isovolumic time, which was significantly prolonged in patients compared with controls (12.0 plus or minus 3.9 seconds per minute versus 6.0 plus or minus 1.8 seconds per minute, p-value less than 0.001) and correlated significantly with peak oxygen consumption (r equals minus 0.63, p-value less than 0.001). The correlation strengthened (r equals minus 0.73, p-value less than 0.001) after excluding seven patients with exercise-induced cyanosis. No relationship was found between exercise capacity and right ventricular ejection fraction or long-axis amplitude.

Resting isovolumic time is prolonged after atrial switch for patients with transposition of the great arteries. It is highly reproducible and relates well to exercise capacity.

Hypoxaemic congenital heart disease (CHD) patients are at higher risk of complications. The aim of this study was to compare and follow-up blood and 24-hour urine analytical data in hypoxaemic and non-hypoxaemic CHD patients.

The inclusion criteria for this study were as follows: patients older than 14 years of age with a structural CHD with or without associated hypoxaemia.

In total, 27 hypoxaemic and 48 non-hypoxaemic CHD patients were included in order to compare blood and 24-hour urine analytical data. Among hypoxaemic patients, 13 (48.1%) were male, two (7.4%) had diabetes mellitus, one of whom was a smoker, one (3.7%) had systemic arterial hypertension, and 11 (40.7%) showed pulmonary arterial hypertension. The mean follow-up time was 3.1±1.9 years. Hypoxaemic CHD patients showed higher proteinuria concentrations (g/24 hours) (0.09 (0.07; 0.46) versus 0.08 (0.07; 0.1), p=0.054) and 24-hour albumin excretion rate (µg/min) (16.5 (11.2; 143.5) versus 4.4 (0.0; 7.6), p<0.001) compared with non-hypoxaemic CHD patients; however, no significant differences were found in the proteinuria levels and in the 24-hour albumin excretion rate in CHD patients with associated hypoxaemia, both at baseline and at follow-up. When divided into groups, hypoxaemic patients with palliative shunts showed significantly higher proteinuria concentrations compared with hypoxaemic patients not operated on or with Fontan procedures (p=0.01). No significant differences were seen in 24-hour proteinuria and 24-hour albumin excretion rate during the follow-up of patients with palliative shunts.

Hypoxaemic CHD patients have significant higher 24-hour proteinuria concentration and 24-hour albumin excretion rate compared with non-hypoxaemic CHD patients. Among hypoxaemic CHD patients, those with palliative shunts showed the highest 24-hour proteinuria concentrations.

Staged left ventricular rehabilitation is a novel surgical approach in patients undergoing single ventricle palliation for borderline hypoplastic left cardiac disease, in an attempt to salvage the left ventricle. The procedure includes resection of endocardial fibroelastosis from the left ventricular free wall and apex. We hypothesised that endocardial fibroelastosis removal may significantly affect ventricular conduction and myocardial electrical characteristics.

This study included 27 patients with borderline hypoplastic left cardiac syndrome who underwent staged left ventricle rehabilitation with endocardial fibroelastosis resection following single ventricle palliation. The effect on electrical synchrony was measured by ventricular depolarisation timing (QRS duration) on electrocardiogram. Patients were evaluated for a change in QRS duration before and after fibroelastosis removal and at most recent follow-up.

The QRS change in the immediate period after endocardial fibroelastosis resection ranged from −16 to 36 milliseconds with a median of 0 (p = 0.09). However, long-term conduction delay was common in 44% (12/27) of patients having a QRS duration greater than 98th percentile for the age at the most recent electrocardiogram. Only one patient had QRS duration greater than 98th percentile before any surgical procedure. Two patients developed left bundle branch block and one developed right bundle branch block with left, but anterior-fascicular block. Overall, the QRS duration correlated with left ventricular size (R = 0.54, p = 0.006) at the most recent electrocardiogram.

Electrical dyssynchrony is a common finding in patients undergoing staged left ventricular rehabilitation after single ventricle palliation; however, it is not acutely related to surgical endocardial resection. Left ventricular size is correlated with QRS duration. Diligent follow-up is required to evaluate the effects of left ventricular growth and consideration of resynchronisation in this population.