Debilitating patient-related non-cardiac co-morbidity cumulatively increases risk for congenital heart surgery. At our emerging programme, flexible surgical strategies were used in high-risk neonates and infants generally considered in-operable, in an attempt to make them surgical candidates and achieve excellent outcomes.

Between April, 2010 and November, 2013, all referred neonates (142) and infants (300) (average scores: RACHS 2.8 and STAT 3.0) underwent 442 primary cardiac operations: patients with bi-ventricular lesions underwent standard (n=294) or alternative (n=19) repair/staging strategies, such as pulmonary artery banding(s), ductal stenting, right outflow patching, etc. Patients with uni-ventricular hearts followed standard (n=96) or alternative hybrid (n=34) staging. The impact of major pre-operative risk factors (37%), standard or alternative surgical strategy, prematurity (50%), gestational age, low birth weight, genetic syndromes (23%), and major non-cardiac co-morbidity requiring same admission surgery (27%) was analysed on the need for extracorporeal membrane oxygenation, mortality, length of intubation, as well as ICU and hospital length of stays.

The need for extracorporeal membrane oxygenation (8%) and hospital survival (94%) varied significantly between surgical strategy groups (p=0.0083 and 0.028, respectively). In high-risk patients, alternative bi- and uni-ventricular strategies minimised mortality, but were associated with prolonged intubation and ICU stay. Major pre-operative risk factors and lower weight at surgery significantly correlated with prolonged intubation, hospital length of stay, and mortality.

In our emerging programme, flexible surgical strategies were offered to 53/442 high-risk neonates and infants with complex CHDs and significant non-cardiac co-morbidity, in order to buffer risk and achieve patient survival, although at the cost of increased resource utilisation.

Patients with unbalanced common atrioventricular canal can be difficult to manage. Surgical planning often depends on pre-operative echocardiographic measurements. We aimed to determine the added utility of cardiac MRI in predicting successful biventricular repair in common atrioventricular canal.

We conducted a retrospective cohort study of children with common atrioventricular canal who underwent MRI prior to repair. Associations between MRI and echocardiographic measures and surgical outcome were tested using logistic regression, and models were compared using area under the receiver operator characteristic curve.

We included 28 patients (median age at MRI: 5.2 months). The optimal MRI model included the novel end-diastolic volume index (using the ratio of left ventricular end-diastolic volume to total end-diastolic volume) and the left ventricle–right ventricle angle in diastole (area under the curve 0.83, p = 0.041). End-diastolic volume index ≤ 0.18 and left ventricle–right ventricle angle in diastole ≤ 72° yield a sensitivity of 83% and specificity of 81% for successful biventricular repair. The optimal multimodality model included the end-diastolic volume index and the echocardiographic atrioventricular valve index with an area under the curve of 0.87 (p = 0.026).

Cardiac MRI can successfully predict successful biventricular repair in patients with unbalanced common atrioventricular canal utilising the end-diastolic volume index alone or in combination with the MRI left ventricle–right ventricle angle in diastole or the echocardiographic atrioventricular valve index. A prospective cardiac MRI study is warranted to better define the multimodality characteristic predictive of successful biventricular surgery.

The global prevalence of obesity in school-age children and adolescents has increased in recent decades. Obesity modifies some aspects of the cardiovascular system in order to preserve the body homoeostasis. Echocardiography to study ventricular function plays an important role in the evaluation of pathological re-modelling associated with left ventricular dysfunction. The aim of this study was to evaluate the left ventricle function and structure with conventional echocardiography and to analyse the longitudinal deformity of the left ventricle using myocardial-tracking signals in a group of severely obese adolescents.

We carried out a descriptive cross-sectional study. We describe the evaluation of the left ventricle using conventional bi-dimensional echocardiography and the myocardial-tracking signals in severely obese adolescents. There were 34 severely obese adolescents included in our study; 52% had a left ventricular ejection fraction<55%, the left ventricular end-diastolic diameter was increased in 70.5% of patients, and 32.3% had an increase in left ventricular mass. On average, 78.9% had abnormal values of left ventricle longitudinal deformations. The number of segments affected per patient was, on average, 5.8, with the anterior apical segment being the most commonly affected. There was a decrease in global longitudinal deformity in 79.4% of the cases.

More than half of this group of asymptomatic severely obese adolescents showed abnormalities in left ventricular structure and function evaluated using traditional echocardiographic methods, but 100% of the cases showed abnormalities in longitudinal deformation in at least one of the 17 left ventricle segments evaluated using myocardial-tracking signals.

This study aimed to investigate the association between long-term survival and different management of major aortopulmonary collateral arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.

From November, 2009 to October, 2018, a total of 98 consecutive patients with pulmonary atresia, ventricular septal defect, major aortopulmonary collateral arteries, and hypoplastic pulmonary arteries treated with modified Blalock–Taussig shunt or right ventricle–pulmonary artery connection were included. Fifty-five patients who received occlusion or ligation of major aortopulmonary collateral arteries during or after palliative procedure were occlusion group, and the other 43 patients were no occlusion group. The early and late outcomes were compared.

The mean duration of follow-up was 30.9 months in no occlusion group and 49.8 months in the occlusion group (p < 0.001). Multivariate analysis showed that only no occlusion of major aortopulmonary collateral arteries was predictive of total mortality (Hazard Ratio: 4.42, 95% CI: 1.27 to 15.42, p = 0.02). The Kaplan–Meier survival curves confirmed that patients without occlusion of major aortopulmonary collateral arteries demonstrated worse survival as compared with the occlusion group (p = 0.013). The Kaplan–Meier survival curves of patients who underwent different palliative procedures showed no differences.

For patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries when a primary repair is not feasible, those without occlusion of major aortopulmonary collateral arteries have a higher risk of death following an initial palliative procedure compared with patients who underwent occlusion of major aortopulmonary collateral arteries. The occlusion of major aortopulmonary collateral arteries is not associated with a higher rate of complete repair or better improvement of pulmonary artery growth.

Newer echocardiographic techniques may allow for more accurate assessment of right ventricular function. Adult studies have correlated these echocardiographic measurements with invasive data, but minimal data exist in the paediatric congenital heart population. The purpose of this study was to evaluate echocardiographic measurements that correlate best with right ventricular systolic and diastolic catheterisation parameters.

Patients with two-ventricle physiology who underwent simultaneous echocardiogram and cardiac catheterisation were included in this study. Right ventricular systolic echocardiographic data included fractional area change, displacement, tissue Doppler imaging s’ wave, global longitudinal strain, and strain rate s’ wave. Diastolic echocardiographic data included tricuspid E and A waves, tissue Doppler imaging e’ and a’ waves, and strain rate e’ and a’ waves. E/tissue Doppler imaging e’, tissue Doppler imaging e’/tissue Doppler imaging a’, E/strain rate e’, and strain rate e’/strain rate a’ ratios were also calculated. Catheterisation dP/dt was used as a marker for systolic function and right ventricular end-diastolic pressure for diastolic function.

A total of 32 patients were included in this study. The median age at catheterisation was 3.1 years (0.3–17.6 years). The DP/dt was 493±327 mmHg/second, and the right ventricular end-diastolic pressure was 7.7±2.4 mmHg. There were no significant correlations between catheterisation dP/dt and systolic echocardiographic parameters. Right ventricular end-diastolic pressure correlated significantly with strain rate e’ (r=−0.4, p=0.02), strain rate a’ (r=−0.5, p=0.03), and E/tissue Doppler imaging e’ (r=0.4, p=0.04).

Catheterisation dP/dt did not correlate with echocardiographic measurements of right ventricular systolic function. Strain rate and tissue Doppler imaging analysis significantly correlated with right ventricular end-diastolic pressure. These values should be further studied to determine whether they may be used as an alternative method to estimate right ventricular end-diastolic pressure in this patient population.

Survivors of single ventricle heart disease must cope with the physical, neurodevelopmental, and psychosocial sequelae of their cardiac disease, which may also affect academic achievement and social relationships. The purpose of this study was to qualitatively examine the experiences of school and social relationships in adolescents with single ventricle heart disease.

A descriptive phenomenological methodology was employed, utilising semi-structured interviews. Demographic and clinical characteristics were obtained via chart review.

Fourteen adolescents (aged 14 to 19 years) with single ventricle heart disease participated. Interviews ranged from 25 to 80 minutes in duration. Four themes emerged from the interviews, including “Don’t assume”: Pervasive ableism; “The elephant in the room”: Uncertain future; “Everyone finds something to pick on”: Bullying at school; “They know what I have been through”: Social support. The overall essence generated from the data was “optimism despite profound uncertainty.”

Adolescents with single ventricle heart disease identified physical limitations and school challenges in the face of an uncertain health-related future. Despite physical and psychosocial limitations, most remained optimistic for the future and found activities that were congruent with their abilities. These experiences reflect “optimism despite profound uncertainty.”

The relationship between different surrogates of insulin resistance and left ventricular geometry in obese children is still unclear.

We sought to explore the relationship between commonly used measures of insulin sensitivity/resistance (homeostatic model assessment index, serum uric acid, and triglycerides to high-density lipoprotein cholesterol ratio) and left ventricular geometry in normotensive obese children.

In this cross-sectional study, 32 normotensive obese children were examined. Transthoracic echocardiography was used to measure left ventricular mass index and relative wall thickness. Homeostasis model assessment index, serum uric acid level, and a ratio of triglycerides to high-density lipoprotein cholesterol were used as markers of the insulin resistance. Simple and partial correlation analyses (to control for the effects of body mass index) were conducted to explore relationship between studied variables and left ventricular mass index or relative wall thickness as outcome variables.

We found positive correlations between homeostasis model assessment index and relative wall thickness (r = 0.47, p = 0.03) which remained significant after controlling for the effect of body mass index, z-score (r = 0.48, p = 0.03). The cutoff level of homeostasis model assessment index with the optimum sensitivity (Sn) and specificity (Sp) derived from the receiver operating characteristic (ROC) curves for predicting concentric remodelling was ≥5.51 with Sn = 83.33 and Sp = 68.75.

There is a positive relationship between homeostasis model assessment index and relative wall thickness of obese normotensive children which may help to distinguish at risk obese normotensive children for the development of concentric left ventricular remodelling.