Branch pulmonary artery stenosis is common after surgical repair in patients with biventricular CHD and often requires reinterventions. However, (long-term) effects of percutaneous branch pulmonary artery interventions on exercise capacity, right ventricular function, and lung perfusion remain unclear. This review describes the (long-term) effects of percutaneous branch pulmonary artery interventions on exercise capacity, right ventricular function, and lung perfusion following PRISMA guidelines.

We performed a systematic search in PubMed, Embase, and Cochrane including studies about right ventricular function, exercise capacity, and lung perfusion after percutaneous branch pulmonary artery interventions. Study selection, data extraction, and quality assessment were performed by two researchers independently.

In total, 7 eligible studies with low (n = 2) and moderate (n = 5) risk of bias with in total 330 patients reported on right ventricular function (n = 1), exercise capacity (n = 2), and lung perfusion (n = 7). Exercise capacity and lung perfusion seem to improve after a percutaneous intervention for branch pulmonary artery stenosis. No conclusions about right ventricular function or remodelling, differences between balloon and stent angioplasty or specific CHD populations could be made.

Although pulmonary artery interventions are frequently performed in biventricular CHD, data on relevant outcome parameters such as exercise capacity, lung perfusion, and right ventricular function are largely lacking. An increase in exercise capacity and improvement of lung perfusion to the affected lung has been described in case of mild to more severe pulmonary artery stenosis during relatively short follow-up. However, there is need for future studies to evaluate the effect of pulmonary artery interventions in various CHD populations.

Prematurity impacts myocardial development and may determine long-term outcomes. The objective of this study was to test the hypothesis that preterm neonates develop right ventricle dysfunction and adaptive remodelling by 32 weeks post-menstrual age that persists through 1 year corrected age.

A subset of 80 preterm infants (born <29 weeks) was selected retrospectively from a prospectively enrolled cohort and measures of right ventricle systolic function and morphology by two-dimensional echocardiography were assessed at 32 weeks post-menstrual age and at 1 year of corrected age. Comparisons were made to 50 term infants at 1 month and 1 year of age. Sub-analyses were performed in preterm-born infants with bronchopulmonary dysplasia and/or pulmonary hypertension.

In both term and preterm infants, right ventricle function and morphology increased over the first year (p < 0.01). The magnitudes of right ventricle function measures were lower in preterm-born infants at each time period (p < 0.01 for all) and right ventricle morphology indices were wider in all preterm infants by 1 year corrected age, irrespective of lung disease. Measures of a) right ventricle function were further decreased and b) morphology increased through 1 year in preterm infants with bronchopulmonary dysplasia and/or pulmonary hypertension (p < 0.01).

Preterm infants exhibit abnormal right ventricle performance with remodelling at 32 weeks post-menstrual age that persists through 1 year corrected age, suggesting a less developed intrinsic myocardial function response following preterm birth. The development of bronchopulmonary dysplasia and pulmonary hypertension leave a further negative impact on right ventricle mechanics over the first year of age.

The porcine small intestinal extracellular matrix reportedly has the potential to differentiate into viable myocardial cells. When used in tetralogy of Fallot repair, it may improve right ventricular function. We evaluated right ventricular function after repair of tetralogy of Fallot with extracellular matrix versus bovine pericardium.

Subjects with non-transannular repair of tetralogy of Fallot with at least 1 year of follow-up were selected. The extracellular matrix and bovine pericardium groups were compared. We used three-dimensional right ventricular ejection fraction, right ventricle global longitudinal strain, and tricuspid annular plane systolic excursion to assess right ventricular function.

The extracellular matrix group had 11 patients, whereas the bovine pericardium group had 10 patients. No differences between the groups were found regarding sex ratio, age at surgery, and cardiopulmonary bypass time. The follow-up period was 28±12.6 months in the extracellular matrix group and 50.05±17.6 months in the bovine pericardium group (p=0.001). The mean three-dimensional right ventricular ejection fraction (55.7±5.0% versus 55.3±5.2%, p=0.73), right ventricular global longitudinal strain (−18.5±3.0% versus −18.0±2.2%, p=0.44), and tricuspid annular plane systolic excursions (1.59±0.16 versus 1.59±0.2, p=0.93) were similar in the extracellular matrix group and in the bovine pericardium group, respectively. Right ventricular global longitudinal strain in healthy children is reported at −29±3% in literature.

In a small cohort of the patients undergoing non-transannular repair of tetralogy of Fallot, there was no significant difference in right ventricular function between groups having extracellular matrix versus bovine pericardium patches followed-up for more than 1 year. Lower right ventricular longitudinal strain noted in both the groups compared to healthy children.

The role of right ventricular longitudinal strain for assessing patients with repaired tetralogy of Fallot is not fully understood. In this study, we aimed to evaluate its relation with other structural and functional parameters in these patients.

Patients followed-up in a grown-up CHD unit, assessed by transthoracic echocardiography, cardiac MRI, and treadmill exercise testing, were retrospectively evaluated. Right ventricular size and function and pulmonary regurgitation severity were assessed by echocardiography and MRI. Right ventricular longitudinal strain was evaluated in the four-chamber view using the standard semiautomatic method.

In total, 42 patients were included (61% male, 32±8 years). The mean right ventricular longitudinal strain was −16.2±3.7%, and the right ventricular ejection fraction, measured by MRI, was 42.9±7.2%. Longitudinal strain showed linear correlation with tricuspid annular systolic excursion (r=−0.40) and right ventricular ejection fraction (r=−0.45) (all p<0.05), which in turn showed linear correlation with right ventricular fractional area change (r=0.50), pulmonary regurgitation colour length (r=0.35), right ventricular end-systolic volume (r=−0.60), and left ventricular ejection fraction (r=0.36) (all p<0.05). Longitudinal strain (β=−0.72, 95% confidence interval −1.41, −0.15) and left ventricular ejection fraction (β=0.39, 95% confidence interval 0.11, 0.67) were independently associated with right ventricular ejection fraction. The best threshold of longitudinal strain for predicting a right ventricular ejection fraction of <40% was −17.0%.

Right ventricular longitudinal strain is a powerful method for evaluating patients with tetralogy of Fallot. It correlated with echocardiographic right ventricular function parameters and was independently associated with right ventricular ejection fraction derived by MRI.

Different echocardiographic parameters have been studied and validated for assessing the severity of mitral stenosis; however, scant data are available for these markers in the context of balloon mitral valvuloplasty in juvenile age groups (ages ⩽20 years).

The aim of the present study was to find out the utility of echocardiographic parameters such as mitral valve separation index, left atrial volume, right ventricular systolic pressure, tricuspid annular plane systolic excursion, tricuspid annular systolic velocity, and right ventricular Tei index in predicting success of balloon mitral valvuloplasty and their relation to mitral valve area in juvenile mitral stenosis.

We carried out a prospective single-centre study involving 52 juvenile mitral stenosis patients undergoing elective valvuloplasty. Success was defined as an increase in mitral valve area ⩾50% or ⩾1.5 cm2. Echocardiographic measurements were taken before and 24 hours after the procedure and statistical analyses were carried out.

The mean age of the study population was 14.3 years (SD ±4.55), ranging from 7 to 20 years. Valvuloplasty was successful in 49 out of 52 patients. The mean valve area improved from 0.89 (SD ±0.16) to 1.73 (SD ±0.22) cm2/m2 (p<0.01), and the mean mitral valve gradient decreased from 19.87 (SD ±7.89) to 7.45 (SD ±2.07) (p=0.021). All the surrogate parameters improved favourably after valvuloplasty (p<0.01). The decrease in right ventricular systolic pressure was a better indicator of the success followed by the increase in valve separation index (area under the curve 0.81 and 0.76, respectively).

All the surrogate markers studied showed favourable improvement, and right ventricular systolic pressure reduction and improved mitral valve separation index were better indicators of successful valvuloplasty.

Aneurysmal dilation of the right ventricular outflow tract complicates assessment of right ventricular function in patients with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion is commonly used to estimate ejection fraction. We hypothesised that tricuspid annular plane systolic excursion measured by cardiac MRI approximates global and segmental right ventricular function, specifically right ventricular sinus ejection fraction, in children with repaired tetralogy of Fallot.

Tricuspid annular plane systolic excursion was measured retrospectively on cardiac MRIs in 54 patients with repaired tetralogy of Fallot. Values were compared with right ventricular global, sinus, and infundibular ejection fractions. Tricuspid annular plane systolic excursion was indexed to body surface area, converted into a fractional value, and converted into published paediatric Z-scores.

Tricuspid annular plane systolic excursion measurements had good agreement between observers. Right ventricular ejection fraction did not correlate with the absolute or indexed tricuspid annular plane systolic excursion and correlated weakly with fractional tricuspid annular plane systolic excursion (r=0.41 and p=0.002). Segmental right ventricular function did not appreciably improve correlation with any of the tricuspid annular plane systolic excursion measures. Paediatric Z-scores were unable to differentiate patients with normal and abnormal right ventricular function.

Tricuspid annular plane systolic excursion measured by cardiac MRI correlates poorly with global and segmental right ventricular ejection fraction in children with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion is an unreliable approximation of right ventricular function in this patient population.

To test the hypothesis that myocardial scars after repair of tetralogy of Fallot are related to impaired cardiac function and adverse clinical outcome.

A total of 53 patients were retrospectively analysed after repair of tetralogy of Fallot. The median patient age was 20 years (range 2–48).

Cardiac MRI with a 1.5 T magnet included cine sequences to obtain volumes and function, phase-sensitive inversion recovery delayed enhancement imaging to detect myocardial scars, and flow measurements to determine pulmonary regurgitation fraction. In addition, clinical parameters were obtained.

An overall 83% of patients were in NYHA class I. All patients with the exception of 2 (96%) had pulmonary insufficiency. Mean ejection fraction and end-diastolic volume index were 46% and 128 ml/m2 for the right ventricle and 54% and 82 ml/m2 for the left ventricle, respectively. Excluding enhancement of the septal insertion and prosthetic patches, delayed enhancement was seen in 11/53 cases (21%). Delayed enhancement of the right ventricle was detected in 6/53 patients (11%) and of the left ventricle in 5/53 patients (9%). The patient group with delayed enhancement was significantly older (p=0.003), had later repair (p=0.007), and higher left ventricular myocardial mass index (p=0.009) compared with the group without delayed enhancement.

This study reveals that scarring is common in patients after surgical repair of tetralogy of Fallot and is associated with older age and late repair. However, there was no difference in right ventricular function, NYHA class, or occurrence of clinically relevant arrhythmias between patients with and those without myocardial scars.

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.

We compared the acute effects on right ventricular haemodynamics of surgical versus transcatheter closure of medium-sized atrial septal defects.

We studied 47 consecutive patients with a defect in the oval fossa and a ratio of pulmonary to systemic flows between 1.5: 1 and 2: 1. They were divided into two groups according to whether the defects were closed by surgery, performed in 23 patients, or by interventional catheterization, achieved in 24 patients. By means of transthoracic cross-sectional echocardiography, we measured right ventricular end-diastolic and endsystolic volumes and calculated ejection fractions. These calculations were performed before, and between 1 and 7 days after closure of the defect.

Before closure of the defect, all patients had an enlarged right ventricle with normal function. After closure by either method, there was no difference in the rate of normalization of end-diastolic volume, but endsystolic volume remained enlarged. Thus, the calculated ejection fraction was lower than before closure.

There was no difference in right ventricular volumes or function early after closure of atrial septal defects, irrespective of whether this was achieved surgically or via transcatheter closure.