Imaging the right heart: the use of integrated multimodality imaging

During recent years, right ventricular (RV) structure and function have been found to be an important determinant of outcome in different cardiovascular and also pulmonary diseases. Currently, echocardiography and cardiac magnetic resonance (CMR) imaging are the two imaging modalities most commonly used to visualize the RV. Most structural abnormalities of the RV can be reliably described by echocardiography but due its complex geometrical shape, echocardiographic assessment of RV function is more challenging. Newer promising echocardiographic techniques are emerging but lack of validation and limited normal reference data influence their routine clinical application. Cardiac magnetic resonance is generally considered the clinical reference technique due to its unlimited imaging planes, superior image resolution, and three-dimensional volumetric rendering. The accuracy and reliability of CMR measurements make it the ideal tool for serial examinations of RV function. Multidetector computed tomography (MDCT) plays an important role in the diagnosis of pulmonary emboli but can also be used for assessing RV ischaemic disease or as an alternative for CMR if contra-indicated. Radionuclide techniques have become more obsolete in the current era. The different imaging modalities should be considered complimentary and each plays a role for different indication

Mental health-related quality of life in mothers of children with surgically repaired congenital heart disease: a 13-year longitudinal study

Aims: Having a child with congenital heart disease (CHD) can affect parental health-related quality of life (HR-QoL). We investigated the long-term trajectories of mental HRQoL (m-HRQoL) in mothers of children with CHD and examined risk factors for persistent low m-HRQoL. Methods: One hundred twenty-five mothers of children with CHD completed a standardized questionnaire on m-HRQoL (mental subscale SF-12) after the children’s first open-heart surgery and subsequently when the children were 1, 4, 6, 10, and 13 years old. A z-score for m-HRQoL was calculated with national norms. Latent class growth analysis (LCGA) was used to identify subgroups of mothers with regards to their m-HRQoL trajectories over time. Regression analysis investigated predictors for chronically low m-HRQoL. Results: Compared to norms, mothers of children with CHD had significantly lower m-HRQoL immediately after open-heart surgery (β = −0.30 (CI-95: −0.44, −0.15)). Subsequently, m-HRQoL increased to a normal level (m-HRQoL compared to the norm from 1 to 13 years: β ranges between 0.05 and 0.27). LCGA revealed two distinct groups of m-HRQoL trajectories: A group with normal m-HRQoL (75% of mothers, means z-scores range between − 0.76 and 0.62) and a group with chronically low m-HRQoL (25% of mothers, mean z-scores range between −1.32 and −0.10). Chronically, low m-HRQoL was associated with poorer social support (OR = 3.39 (CI-95: 1.40, 8.49), p = 0.008) but not with parental education, migration background, number of open-heart surgeries, diagnosis of a univentricular CHD, or low IQ. Conclusion: A quarter of mothers of children with CHD have chronically low m-HRQoL throughout their child’s development, especially those mothers with poor social support. Further studies of family-oriented approaches are needed to identify and support these mothers and reinforce parental well-being

Physical Activity in Fontan Patients Relates to Quality of Life and Sleep Quality

Background and Aim: Fontan patients tend to have reduced physical exercise capacity. This study investigates physical activity (PA) and its relationship to exercise capacity, heart rates, cardiac function, biomarkers, health-related quality of life (HRQoL), and sleep quality. Methods: Cardiovascular magnetic resonance (CMR), exercise testing (CPET), 24 h-ECG, and blood samples were prospectively performed in 38 patients, age 13 (11-16) years. PA was assessed by accelerometer during 7 consecutive days. HRQoL was self-assessed with KIDSCREEN-27 and SF-36 according to patients' age; sleep quality with Pediatric Sleep Questionnaire (PSQ) and Pittsburgh Sleep Quality Index (PSQI). Results: Daily moderate to vigorous physical activity (MVPA) was in median (IQR) 40 (28-57) mins; 7/38 (18%) patients reached the recommended 60 mins/day of MVPA. MVPA did not correlate with gender, age, single ventricle morphology, time from Fontan, heart rate, ventricular volumes, and ejection fraction at CMR, biomarkers, or CPET. Physical wellbeing (r = 0.33, p = 0.04), autonomy (r = 0.39, p = 0.03), and social support (r = 0.43, p = 0.009) assessed using the KIDSCREEN-27, and both physical (r = 0.57, p = 0.03) and mental (r = 0.54, p = 0.04) domains of the SF-36 questionnaire correlated with daily minutes of MVPA. PSQI global sleeping score (r = -0.7, p = 0.007), and PSQ scales for behavior (r = -0.36; p = 0.03) correlated with daily minutes of MVPA. Conclusion: Only 18% of the Fontan patients meet the recommendation for daily MVPA. Measures of exercise capacity, cardiac function or chronotropic competence are not correlated to daily physical activity. In contrast, HRQoL and sleep quality seem to be associated with regular physical activity

Remodelling of the right ventricle after early pulmonary valve replacement in children with repaired tetralogy of Fallot: assessment by cardiovascular magnetic resonance

Aims Correct timing of pulmonary valve replacement (PVR) is crucial for preventing complications of pulmonary regurgitation and right ventricular (RV) dilatation after repair of tetralogy of Fallot. We sought to assess the remodelling of the RV after early PVR in children, using cardiovascular magnetic resonance (CMR). Methods and results Twenty children with severe pulmonary regurgitation and RV dilatation and mean age 13.9±3 years underwent CMR evaluation 5.6±1.8 months before and 5.9±0.6 months after PVR. PVR was performed when the RV end-diastolic volume exceeded 150 mL/m2, as measured by CMR. The time interval between primary repair and PVR was 12±3 years. Post-operative CMR demonstrated a significant reduction of the RV end-diastolic volume from 189.8±33.4 to 108.7±25.8 mL/m2 (P<0.0001), of the RV end-systolic volume from 102.4±27.3 to 58.2±16.3 mL/m2 (P<0.0001), and of the RV mass from 48.7±12.3 to 35.8±7.7 g/m2 (P<0.0001). The RV ejection fraction did not change significantly. Conclusion Prompt RV remodelling, with reduction of RV volume and mass, is observed after performing PVR if the RV end-diastolic volume exceeds 150 mL/m2. Early PVR may prevent the detrimental complications of severe pulmonary regurgitatio

Modified Blalock Taussig shunt: a not-so-simple palliative procedure

OBJECTIVES Thirty-two consecutive isolated modified Blalock Taussig (BT) shunts performed in infancy since 2004 were reviewed and analysed to identify the risk factors for shunt intervention and mortality. METHODS Sternotomy was the only approach used. Median age and weight were 10.5 (range 1-74) days and 2.9 (1.9-4.4) kg, respectively. Shunt palliation was performed for biventricular hearts (Tetralogy of Fallot/double outlet right ventricle/transposition of great arteries_ventricular septal defect_pulmonary stenosis/pulmonary atresia_ventricular septal defect/others) in 21, and univentricular hearts in 11, patients. Hypoplastic left heart syndrome patients were excluded. Two procedures required cardiopulmonary bypass. Median shunt size was 3.5 (3-4) mm and median shunt size/kg body weight was 1.2 (0.9-1.7) mm/kg. Reduction in shunt size was necessary in 5 of 32 (16%) patients. RESULTS Three of 32 (9%) patients died after 3 (1-15) days due to cardiorespiratory decompensation. Lower body weight (P = 0.04) and bigger shunt size/kg of body weight (P = 0.004) were significant risk factors for mortality. Acute shunt thrombosis was observed in 3 of 32 (9%), none leading to death. Need for cardiac decongestive therapy was associated with univentricular hearts (P < 0.001), bigger shunt size (P = 0.054) and longer hospital stay (P = 0.005). Twenty-eight patients have undergone a successful shunt takedown at a median age of 5.5 (0.5-11.9) months, without late mortality. CONCLUSIONS Palliation with a modified BT shunt continues to be indicated despite increased thrust on primary corrective surgery. Though seemingly simple, it is associated with significant morbidity and mortality. Effective over-shunting and acute shunt thrombosis are the lingering problems of shunt therap

Multivendor Evaluation of Automated MRI Postprocessing of Biventricular Size and Function for Children With and Without Congenital Heart Defects

BACKGROUND Manually segmenting cardiac structures is time-consuming and produces variability in MRI assessments. Automated segmentation could solve this. However, current software is developed for adults without congenital heart defects (CHD). PURPOSE To evaluate automated segmentation of left ventricle (LV) and right ventricle (RV) for pediatric MRI studies. STUDY TYPE Retrospective comparative study. POPULATION Twenty children per group of: healthy children, LV-CHD, tetralogy of Fallot (ToF), and univentricular CHD, aged 11.7 [8.9-16.0], 14.2 [10.6-15.7], 14.6 [11.6-16.4], and 12.2 [10.2-14.9] years, respectively. SEQUENCE/FIELD STRENGTH Balanced steady-state free precession at 1.5 T. ASSESSMENT Biventricular volumes and masses were calculated from a short-axis stack of images, which were segmented manually and using two fully automated software suites (Medis Suite 3.2, Medis, Leiden, the Netherlands and SuiteHeart 5.0, Neosoft LLC, Pewaukee, USA). Fully automated segmentations were manually adjusted to provide two further sets of segmentations. Fully automated and adjusted automated segmentation were compared to manual segmentation. Segmentation times and reproducibility for each method were assessed. STATISTICAL TESTS Bland Altman analysis and intraclass correlation coefficients (ICC) were used to compare volumes and masses between methods. Postprocessing times were compared by paired t-tests. RESULTS Fully automated methods provided good segmentation (ICC > 0.90 compared to manual segmentation) for the LV in the healthy and left-sided CHD groups (eg LV-EDV difference for healthy children 1.4 ± 11.5 mL, ICC: 0.97, for Medis and 3.0 ± 12.2 mL, ICC: 0.96 for SuiteHeart). Both automated methods gave larger errors (ICC: 0.62-0.94) for the RV in these populations, and for all structures in the ToF and univentricular CHD groups. Adjusted automated segmentation agreed well with manual segmentation (ICC: 0.71-1.00), improved reproducibility and reduced segmentation time in all patient groups, compared to manual segmentation. DATA CONCLUSION Fully automated segmentation eliminates observer variability but may produce large errors compared to manual segmentation. Manual adjustments reduce these errors, improve reproducibility, and reduce postprocessing times compared to manual segmentation. Adjusted automated segmentation is reasonable in children with and without CHD. EVIDENCE LEVEL 3. TECHNICAL EFFICACY Stage 2

Management and outcome of Ebstein's anomaly in children

Abstract Objectives To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly. Background Data on long-term outcome of children with Ebstein's anomaly are scarce. Methods Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention. Results A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1-16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%. Conclusion In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcom

Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease

Cardiovascular magnetic resonance (CMR) has taken on an increasingly important role in the diagnostic evaluation and pre-procedural planning for patients with congenital heart disease. This article provides guidelines for the performance of CMR in children and adults with congenital heart disease. The first portion addresses preparation for the examination and safety issues, the second describes the primary techniques used in an examination, and the third provides disease-specific protocols. Variations in practice are highlighted and expert consensus recommendations are provided. Indications and appropriate use criteria for CMR examination are not specifically addressed

Effect of phosphodiesterase-5 inhibition on SystEmic Right VEntricular size and function - a multi-center, double-blind, randomized, placebo-controlled trial - SERVE.

BACKGROUND AND AIMS In adults with congenital heart disease and systemic right ventricles, progressive right ventricular systolic dysfunction is common and is associated with adverse outcomes. Our aim was to assess the impact of the phosphodiesterase-5-inhibitor tadalafil on right ventricular systolic function. METHODS AND RESULTS This was a double-blind, randomized, placebo-controlled, multi-center superiority trial (NCT03049540) involving 100 adults with systemic right ventricles (33 women, mean age: 40.7 years, SD 10.7), comparing tadalafil 20mg once daily versus placebo (1:1-ratio). Primary endpoint was the change in right ventricular endsystolic volume after three years of therapy. Secondary endpoints were changes in right ventricular ejection fraction, exercise capacity and NT-proBNP-concentration. Primary endpoint assessment by intention to treat analysis at three years of follow up was possible in 83 patients (42 patients in the tadalafil group and 41 patients in the placebo group). No significant changes over time in right ventricular endsystolic volumes were observed in the tadalafil and the placebo-group, and no significant differences between treatment groups (3.4ml, 95% CI, -4.3 to 11.0, p=0.39). No significant changes over time were observed for the pre-specified secondary endpoints for the entire study population, without differences between the tadalafil and the placebo-group. CONLCUSIONS In this trial in adults with systemic right ventricles, right ventricular systolic function, exercise capacity and neuro-hormonal activation remained stable over a three-year follow-up period. No significant treatment effect of tadalafil was observed. Further research is needed to find effective treatment for improvement of ventricular function in adults with systemic right ventricles. This article is protected by copyright. All rights reserved

Effect of phosphodiesterase-5 inhibition on SystEmic Right VEntricular size and function - A multicentre, double-blind, randomized, placebo-controlled trial - SERVE

AIMS In adults with congenital heart disease and systemic right ventricles, progressive right ventricular systolic dysfunction is common and is associated with adverse outcomes. Our aim was to assess the impact of the phosphodiesterase-5-inhibitor tadalafil on right ventricular systolic function. METHODS AND RESULTS This was a double-blind, randomized, placebo-controlled, multicentre superiority trial (NCT03049540) involving 100 adults with systemic right ventricles (33 women, mean age: 40.7 ± 10.7 years), comparing tadalafil 20 mg once daily versus placebo (1:1 ratio). The primary endpoint was the change in right ventricular end-systolic volume after 3 years of therapy. Secondary endpoints were changes in right ventricular ejection fraction, exercise capacity and N-terminal pro-B-type natriuretic peptide concentration. Primary endpoint assessment by intention to treat analysis at 3 years of follow-up was possible in 83 patients (42 patients in the tadalafil group and 41 patients in the placebo group). No significant changes over time in right ventricular end-systolic volumes were observed in the tadalafil and the placebo group, and no significant differences between treatment groups (3.4 ml, 95% confidence interval -4.3 to 11.0, p = 0.39). No significant changes over time were observed for the pre-specified secondary endpoints for the entire study population, without differences between the tadalafil and the placebo group. CONCLUSIONS In this trial in adults with systemic right ventricles, right ventricular systolic function, exercise capacity and neuro-hormonal activation remained stable over a 3-year follow-up period. No significant treatment effect of tadalafil was observed. Further research is needed to find effective treatment for improvement of ventricular function in adults with systemic right ventricles