To investigate the effect of pulsatility of venous flow waveform in the inferior and superior caval vessels on the performance of functional and “failing” Fontan patients based on two primary performance measures – the conduit power loss and the distribution of inferior caval flow (hepatic factors) to the lungs.

Doppler angiography flows were acquired from two typical extra-cardiac conduit “failing” Fontan patients, aged 13 and 25 years, with ventricle dysfunction. Using computational fluid dynamics, haemodynamic efficiencies of “failing”, functional, and in vitro-generated mechanically assisted venous flow waveforms were evaluated inside an idealised total cavopulmonary connection with a caval offset. To investigate the effect of venous pulsatility alone, cardiac output was normalised to 3 litres per minute in all cases. To quantify the pulsatile behaviour of venous flows, two new performance indices were suggested.

Variations in the pulsatile content of venous waveforms altered the conduit efficiency notably. High-frequency and low-amplitude oscillations lowered the pulsatile component of the power losses in “failing” Fontan flow waveforms. Owing to the offset geometry, hepatic flow distribution depended strongly on the ratio of time-dependent caval flows and the pulsatility content rather than mixing at the junction. “Failing” Fontan flow waveforms exhibited less balanced hepatic flow distribution to lungs.

The haemodynamic efficiency of single-ventricle circulation depends strongly on the pulsatility of venous flow waveforms. The proposed performance indices can be calculated easily in the clinical setting in efforts to better quantify the energy efficiency of Fontan venous waveforms in pulsatile settings.

To analyse the relationship between different heart rate variability indices, resting heart rate, and cardiovascular markers in adolescents.

A cross-sectional study was carried out with information from an ongoing cohort study. The sample was composed of 99 adolescents who complied with the following inclusion criteria: aged between 11 and 14 years; enrolled in a school unit of elementary education; absence of any known diseases; no drug consumption; and a formal consent signed by the parents or legal guardians. Weight, height, heart rate variability, lipid profile, inflammatory markers, blood pressure, resting heart rate, intima-media thickness, blood flow, and trunk fatness were measured. Partial correlation and linear regression (expressed by β and 95% confidence intervals [95%CI]) analyses were used to analyse the relationships between the variables.

In the linear regression analysis, even after adjustments for sex, age, trunk fatness, and somatic maturation, parasympathetic activity presented significant correlations with maximum carotid artery blood flow (β=−0.111 [95%CI=−0.216; −0.007]), systolic blood pressure (β=−0.319 [95%CI=−0.638; −0.001]), and resting heat rate (β=−0.005 [95%CI=−0.009; −0.002]).

Parasympathetic activity at rest is inversely related to maximum and minimum blood flow, triacylglycerol levels, and systolic blood pressure. These findings suggest that heart rate variability has the potential to discriminate pre-pubertal adolescents at increased risk.

Management of “failing” and “failed” Fontan circulation, particularly the indications, timing, and type of re-intervention, currently remains nebulous. Factors contributing to pathogenesis and mortality following Fontan procedure differ between children and adults.

Since organ systems in individual patients are affected differently, we searched the extant literature for a “failing” and “failed” Fontan reviewing the clinical phenotypes, diagnostic modalities, pharmacological, non-pharmacological, and surgical techniques employed, and their outcomes.

A total of 410 investigations were synthesised. Although proper candidate selection, thoughtful technical modifications, timely deployment of mechanical support devices, tissue-engineered conduits, and Fontan takedown have decreased the peri-operative mortality from 9 to 15% and 1 to 3% per cent in recent series, pernicious changes in organ function are causing long-term patient attrition. In the setting of a failed Fontan circulation, literature documents three surgical options: Fontan revision, Fontan conversion, or cardiac transplantation. The reported morbidity of 25% and mortality of 8–10% among Fontan conversion continue to improve in select institutions. While operative mortality following cardiac transplantation for Fontan failure is 30% higher than for other CHDs, there is no difference in long-term survival with actuarial 10-year survival of around 54%. Mechanical circulatory assistance, stem cells, and tissue-engineered Fontan conduit for destination therapy or as a bridge to transplantation are in infancy for failing Fontan circulation.

An individualised management strategy according to clinical phenotypes may delay the organ damage in patients with a failing Fontan circulation. At present, cardiac transplantation remains the last stage of palliation with gradually improving outcomes.

This study evaluated the effect of music intervention on the anxiety and stress responses of patients who underwent an interventional cardiac catheterisation.

The study design was a pre- and post-test randomised controlled trial that included 94 patients who underwent a transcatheter atrial septal defect closure. Patients were allocated to receive either music intervention (n = 47) or usual care (n = 47) during the interventional cardiac catheterisation. Music intervention effectiveness was examined in terms of anxiety, salivary cortisol level, and heart rate variability.

The average age of participants was 45.40 years (±16.04) in the experimental group and 47.26 years (±13.83) in the control group. Two-thirds (66.0%) of the participants in each group were women. State anxiety (F = 31.42, p < 0.001), anxiety-numerical rating scale (F = 20.08, p < 0.001), salivary cortisol levels (F = 4.98, p = 0.021), and low-frequency component/high-frequency component ratio (F = 17.31, p < 0.001) in the experimental group were significantly reduced compared with those in the control group at the end of the music intervention.

This study provides practical evidence of a reduction in anxiety and stress response from music intervention preceding an interventional cardiac catheterisation, indicating that this intervention should be considered in clinical management.

Iron cardiomyopathy remains the major cause of death in β-thalassaemia major. Excessive iron loading could lead to cardiac dysfunction and arrhythmias. Reduced heart rate variability is associated with a higher risk of arrhythmia and sudden death after myocardial infarction and heart failure. Previous data have reported on reduced heart rate variability in patients with marked cardiac iron accumulation. In this study, we compared heart rate variability among β-thalassaemia major (TM) patients with or without cardiac siderosis.

Out of 70 β-thalassaemia major patients with preserved ejection fractions, 38 patients with cardiac T2* magnetic resonance imaging assessment were included in our study. Time domain heart rate variability parameters were analysed from 24-hour recorded electrocardiograms and were compared with the control group.

The mean T2* magnetic resonance imaging value was 22.9 ± 13.3 (4.7–47.5). In 21 patients with β-thalassaemia major, the T2* magnetic resonance imaging values were greater than 20 ms and these patients were considered to be in the early stage of the disease. When we compare these patients with control subjects, the standard deviation of all NN intervals was still significantly lower (133.0 ± 32.2 versus 162.8 ± 32.9, p = 0.001) in β-thalassaemia major patients despite normal T2* magnetic resonance imaging values. On the contrary, the standard deviation of all NN intervals was not correlated with haemoglobin levels in these patients (p > 0.05).

Heart rate variability parameters were reduced even in β-thalassaemia major patients without evident cardiac siderosis, as specified by magnetic resonance imaging data. The results of this study show that reduction of heart rate variability may start before cardiac iron loading is demonstrated by T2* magnetic resonance imaging in β-thalassaemia major.

This prospective study was designed to monitor severe pulmonary hypertension during extra corporeal membrane oxygenation using echo Doppler variables.

All neonates treated with extracorporeal membrane oxygenation also have severe pulmonary hypertension. A study which monitors the reaction of the pre-existing pulmonary hypertension during extracorporeal oxygenation by frequent sampling of those variables related to pulmonary pressure is still lacking. Such a study is necessary to analyze the complex haemodynamic changes in patients undergoing extracorporeal membrane oxygenation.

In 29 neonates, we estimated pulmonary arterial pressure using peakflow velocity of regurgitation across the tricuspid- and pulmonary valve, peakflow velocity of shunting across persistent arterial ductus, and systolic time intervals of the right ventricle. Correlation between the several estimations of pulmonary arterial pressure were analysed with the Spearman correlation coefficient.

Systolic pulmonary arterial pressure measured by the velocity of tricuspid regurgitation illustrated severe pulmonary hypertension prior to extra corporeal membrane oxygenation (mean 63 mmHg, sd 20). Similar levels for the systolic pulmonary arterial pressure could be derived (mean 73 mmHg, sd 17) from ductal shunting. A fair correlation of 0.76 (p< 0.002) could be demonstrated. Pulmonary hypertension responded well and quickly to treatment by extra corporeal membrane oxygenation, with reductions within 24 hours to mean systolic levels of 35 mmHg, sd 23. This very early reaction has not previously been demonstrated and could be of importance in defining parameters for weaning from cardiopulmonary bypass. Diastolic pulmonary arterial pressure was investigated because of its relation to vascular resistance. It proved more difficult to measure because of the low incidence of pulmonary regurgitation. Derived diastolic pressures did not show any good correlations.

Pulmonary hypertension is well documentated prior to extra corporeal membrane oxygenation and respons very quickly to the institution of treatment. Ultra sound techniques are indicated at the bedsite, and prove useful in monitoring pulmonary blood pressure during the procedure.

To analyse the relative frequency and gender ratios in the procedures (both surgical and catheter-based interventions) performed for cardiac diseases in Indian children.

Not many studies are available in the developing countries with regard to the prevalence, relative frequency, or gender distribution of cardiac diseases in children. As universal newborn screening is not carried out for congenital cardiac diseases, the statistics are difficult to ascertain. Do female and male children with cardiac disease get equal parental preference in terms of surgical correction and catheter interventions in India? This question is also unanswered.

Analysis of 15,066 consecutive Indian children aged <18 years who were operated upon or had catheter intervention at a single tertiary care centre. Relative frequency and male/female ratios of cardiac lesions in these children were determined and compared with the studies in literature.

Overall male/female ratio in the study was 1.4:1. Ventricular septal defect was the most common lesion (24.2%) with male/female ratio of 1.5:1, followed by tetralogy of Fallot (18.7%, 1.6:1), atrial septal defect (14.4%, 0.9:1) and so on. Male children dominated the total number of procedures performed.

In most of the individual cardiac lesions, the relative frequency was different from that of international studies. The gender ratio for the majority of the individual type of cardiac problems was different from that of international references. These findings may suggest a preference for the male child in the treatment of cardiac diseases in India, which could possibly be related to social causes.