The right ventricle in volume or pressure overload : Insights from novel imaging techniques

This study is inspired by the gap in knowledge regarding the timing of cardiac surgery and interventions in adult patients with congenital heart disease. There are many parameters used assessing right ventricular function; however, most of them have pitfalls. Understanding the pathomechanisms by which the heart adapts to congenital defects is probably key to find the answer when it is time to intervene and start discussing treatment options. Heart defects are the most frequently occurring congenital disorders. Less than 50% of individuals with moderate to severe congenital heart defects, e.g. transposition of the great arteries (TGA) or tetralogy of Fallot (TOF), survive to adulthood without intervention. Advances in cardiac surgery and better identification of individuals at risk for sudden cardiac death have increased survival rates. Currently, more than 96% of patients with congenital heart disease survive to at least 16 years of age; most undergo corrective surgery but are not cured, and only a few have normal physiology and anatomy. In many cases, the heart must develop mechanisms of adaptation to the changed conditions after surgery. Consequently, correction of the defect creates residual disease with a risk of future complications. To prevent clinical deterioration and to identify the development of complications, patients need lifelong, regular follow up. The choice of followup modalities depends on the cardiac malformation. The right ventricle (RV) plays an important role, as it is often part of the defect or is influenced by the surgery. In the past, research was focused on assessment of left ventricular function (LV), and the RV was “the forgotten ventricle.” Observations and studies in the last few decades brought increased interest into the RV and revealed the importance of the RV in the prognosis of various cardiac diseases. An understanding of RV morphology, pathophysiology and adaptive mechanisms is crucial for further studies of prognosis as well as for research linked to the use of particular diagnostic modalities. When the RV is exposed to increased pressure load, e.g. in atrially corrected transposition of the great arteries (TGA), adaptation affects the cavity volume as well as the wall thickness. When the RV is volume overloaded, adaptation involves enhancement of the RV cavity volume while the wall thickness often remains unchanged under long time. RV ejection fraction (RVEF) gives some information about changes in RV function, but information on myocardial contractility and contractile reserve is also needed. New functional parameters such as strain—also known as myocardial deformation—provide some information about intrinsic myocardial function. In Paper I, we studied functional parameters such as ejection fraction and strain (radial and longitudinal strain for both ventricles) in patients with Tetralogy of Fallot (TOF) and TGA. Longitudinal RV strain was depressed in both patient groups in comparison with that in healthy individuals, and there were additional differences between the two patient groups. In Paper II, we validated three-dimensional echocardiography (3DEcho) against the cardiac magnetic resonance (CMR) gold standard. The study population was limited to patients with TOF. In general, 3DEcho underestimated RV volumes but was able to identify patients with RV dilatation on CMR with high sensitivity. RV longitudinal free wall strain measured by CMR with a cut-off set at -14% identified patients with depressed exercise capacity and low peak oxygen uptake. In Paper III, we studied a new CMR method to quantify and visualise turbulent flow in the heart and vessels. Turbulent flow can be harmful to tissue, blood cells, and endothelium and can contribute to tissue remodeling. In patients with TOF, turbulent flow can be seen as variance in 2DEcho color Doppler. In CMR, increased turbulent kinetic energy (TKE) could be seen with four-dimensional flow. The RV TKE was increased in patients with TOF with pulmonary regurgitation compared with that in healthy controls. In Paper IV, we validated “knowledge-based reconstruction” (KBR), a novel method to calculate RV volume, against CMR in patients with various types of congenital heart defects. Two-dimensional echocardiogram-based threedimensional RV reconstruction is a relatively uncomplicated method that creates a three-dimensional RV model based on a limited number of predefined points of interest (RV structures such as tricuspid annulus, RV free wall, or pulmonary valve). KBR showed good agreement with CMR (intraclass correlation coefficient = 0.84 for RV end-diastolic volume and 0.89 for ejection fraction) but tended to underestimate RV volumes, which is in line with other methods based on ultrasound. Conclusions: 3DEcho is an evolving modality that is able to identify patients with RV dilatation. It can be used clinically for the follow up of patients with congenital heart diseases, especially those with mildly to moderately dilated RVs. When an intervention seems likely, 3DEcho results should be verified by CMR. CMR-derived measurements of longitudinal and radial strain provide a new understanding of RV remodeling and ventricular interdependence in patients with TOF and TGA. Depressed longitudinal strain may indicate a risk of depressed exercise capacity and, in patients with TGA, clinical deterioration. Further studies in larger populations of patients with congenital heart defects are needed, as the altered RV morphology in such patients makes quantitative assessment especially challenging.Korrigeringar är gjorda i den elektroniska versionen utifrån publicerad erratalista / The corrections in the published errata list are implemented in the electronic version.</p

Catheter closure of atrial septal defect in the elderly (≥ 65 years). A worthwhile procedure

Background Secundum atrial septal defect (ASD2) is one of the most common cardiac malformations diagnosed in adult life. Catheter closure has made treatment possible even in patients of high age. However, published outcome data for elderly patients is limited. The aim of this study was to report, on a national basis, the long-term outcome of ASD2 catheter closure in the elderly. Material and results We report the clinical and echocardiographic outcome of catheter closure of ASD2 in 148 patients aged 65-87 years. Data was obtained from a national registry, medical records and a questionnaire. The proportion of patients in NYHA I increased from 34% to 61% (p < 0.001) one year after closure and remained stable at the latest follow-up 4,4 (SD 2,6) years post-closure. The proportion of patients with moderate/severe enlargement of the right ventricle and atrium fell from 77% and 76% to 25% and 40%, respectively, (p < 0.001) and right ventricular systolic pressure dropped significantly. Improvement of NYHA class was associated with reduced right ventricular systolic pressure but not with remodelling of the right heart. NYHA deteriorated in 9 patients, despite reduced right ventricular size. Overall, the prevalence of atrial fibrillation was unchanged after closure. Major complication rate was 2% and there was no procedure- or device-related mortality. Conclusion Catheter closure of ASD2 in the elderly is a worthwhile procedure since it improves symptoms and has a low complication rate. However, a subset of patients do not improve, in which we suggest that concealed left ventricular dysfunction may play a causative role

Knowledge-based 3D reconstruction of the right ventricle : comparison with cardiac magnetic resonance in adults with congenital heart disease

AIM: Assessment of right ventricular (RV) function is a challenge, especially in patients with congenital heart disease (CHD). The aim of the present study is to assess whether knowledge-based RV reconstruction, used in the everyday practice of an echo-lab for adult CHD in a tertiary referral center, is accurate when compared to cardiac magnetic resonance (CMR) examination. SUBJECTS AND METHODS: Adult patients who would undergo CMR for assessment of the RV were asked to undergo an echo of the heart for further knowledge-based reconstruction (KBR). Echocardiographic images were acquired in standard views using a predefined imaging protocol. RV volumes and ejection fraction (EF) calculated using knowledge-based technology were compared with the CMR data of the same patient. RESULTS: Nineteen consecutive patients with congenital right heart disease were studied. Median age of the patients was 28 years (range 46 years). Reconstruction was possible in 16 out of 19 patients (85%). RV volumes assessed with this new method were smaller than with CMR. Indexed end diastolic volumes were 114±17 ml vs 121±19 ml, P<0.05 and EFs were 45±8% vs 47±9%, P<0.05 respectively. The correlation between the methods was good with an intraclass correlation of 0.84 for EDV and 0.89 for EF, P value <0.001 in both cases. CONCLUSION: KBR enables reliable measurement of RVs in patients with CHDs and can be used in clinical practice for analysis of volumes and EFs

Knowledge-based 3D reconstruction of the right ventricle : comparison with cardiac magnetic resonance in adults with congenital heart disease

AIM: Assessment of right ventricular (RV) function is a challenge, especially in patients with congenital heart disease (CHD). The aim of the present study is to assess whether knowledge-based RV reconstruction, used in the everyday practice of an echo-lab for adult CHD in a tertiary referral center, is accurate when compared to cardiac magnetic resonance (CMR) examination. SUBJECTS AND METHODS: Adult patients who would undergo CMR for assessment of the RV were asked to undergo an echo of the heart for further knowledge-based reconstruction (KBR). Echocardiographic images were acquired in standard views using a predefined imaging protocol. RV volumes and ejection fraction (EF) calculated using knowledge-based technology were compared with the CMR data of the same patient. RESULTS: Nineteen consecutive patients with congenital right heart disease were studied. Median age of the patients was 28 years (range 46 years). Reconstruction was possible in 16 out of 19 patients (85%). RV volumes assessed with this new method were smaller than with CMR. Indexed end diastolic volumes were 114±17 ml vs 121±19 ml, P&lt;0.05 and EFs were 45±8% vs 47±9%, P&lt;0.05 respectively. The correlation between the methods was good with an intraclass correlation of 0.84 for EDV and 0.89 for EF, P value &lt;0.001 in both cases. CONCLUSION: KBR enables reliable measurement of RVs in patients with CHDs and can be used in clinical practice for analysis of volumes and EFs

Turbulent kinetic energy in the right ventricle : Potential MR marker for risk stratification of adults with repaired Tetralogy of Fallot

Purpose: To assess right ventricular (RV) turbulent kinetic energy (TKE) in patients with repaired Tetralogy of Fallot (rToF) and a spectrum of pulmonary regurgitation (PR), as well as to investigate the relationship between these 4D flow markers and RV remodeling. Materials and Methods: Seventeen patients with rToF and 10 healthy controls were included in the study. Patients were divided into two groups based on PR fraction: one lower PR fraction group (11%) and one higher PR fraction group (&gt;11%). Field strength/sequences: 3D cine phase contrast (4D flow), 2D cine phase contrast (2D flow), and balanced steady-state free precession (bSSFP) at 1.5T. Assessment: The RV volume was segmented in the morphologic short-axis images and TKE parameters were computed inside the segmented RV volume throughout diastole. Statistical tests: One-way analysis of variance with Bonferroni post-hoc test; unpaired t-test; Pearson correlation coefficients; simple and stepwise multiple regression models; intraclass correlation coefficient (ICC). Results: The higher PR fraction group had more remodeled RVs (140 6 25 vs. 107 6 22 [lower PR fraction, P &lt; 0.01] and 93 6 15 ml/m2[healthy, P &lt; 0.001] for RV end-diastolic volume index [RVEDVI]) and higher TKE values (5.95 6 3.15 vs. 2.23 6 0.81 [lower PR fraction, P &lt; 0.01] and 1.91 6 0.78 mJ [healthy, P &lt; 0.001] for Peak Total RV TKE). Multiple regression analysis between RVEDVI and 4D/2D flow parameters showed that Peak Total RV TKE was the strongest predictor of RVEDVI (R25 0.47, P 5 0.002). Conclusion: The 4D flow-specific TKE markers showed a slightly stronger association with RV remodeling than conventional 2D flow PR parameters. These results suggest novel hemodynamic aspects of PR in the development of late complications after ToF repair.Funding agencies:  European Research Council [310612]; Swedish Heart and Lung Foundation [20140398]; County Council of Ostergotland; Medical Research Council of Southeast Sweden (FORSS); Swedish Research Council [2013-6077, 2014-6191]</p

Afterload dependence of right ventricular myocardial deformation : A comparison between tetralogy of Fallot and atrially corrected transposition of the great arteries in adult patients

Background Prior studies suggested that myocardial deformation is superior to conventional measures for assessing ventricular function. This study aimed to evaluate right ventricular (RV) myocardial deformation in response to increased afterload. Patients with the RV in the systemic position were compared with patients with the RV in the sub-pulmonic position with normal or only slightly elevated systolic right ventricular pressure. Correlations between global longitudinal strain (GLS), radial strain, atrioventricular plane displacement (AVPD), and exercise capacity were evaluated. Methods 44 patients with congenital heart defect were enrolled in the study. The control group consisted of seven healthy volunteers. All patients underwent cardiovascular magnetic resonance (CMR) and cardiopulmonary exercise testing. We assessed biventricular myocardial function using CMR based feature tracking and compared the results to anatomic volumes. Results Strain analysis and displacement measurements were feasible in all participants. RVGLS and RVAVPD were reduced in both study groups compared to the control group (p&lt;0.001). Left ventricular (LV) radial strain was significantly lower in patients with a systemic RV than in those with a subpulmonic RV and lower than in controls (p&lt;0.001). Both LVAVPD and RVAVPD were significantly depressed in patients compared to controls (p&lt;0.05). RVAVPD was more depressed in patients with a high systolic RV pressure than in those with normal RV pressure (p&lt;0.001). RVAVPD did not correlate with exercise capacity in either study group. Exercise capacity in both patient groups was depressed to levels reported in previous studies, and did not correlate with RVGLS. Conclusions Both study groups had abnormal myocardial deformation and increased RV volumes. RVGLS in patients was lower than in controls, confirming the effect of increased afterload on myocardial performance.Funding Agencies|ALF Grant, Region Ostergotland [LIO-281281]</p

Symptoms during pregnancy in primiparous women with congenital heart disease.

Background: As more women with congenital heart disease (CHD) are reaching childbearing age, it becomes more common for their symptoms to be evaluated during pregnancy. However, pregnancy-related symptoms are similar to those caused by heart disease. This study investigated the prevalence of factors associated with symptoms during pregnancy in women with CHD. Methods: The national birth register was searched for primiparous women with CHD who were registered in the national quality register for patients with CHD. Results: Symptoms during the third trimester were reported in 104 of 465 evaluated women. The most common symptom was palpitations followed by dyspnea. Factors associated with symptoms were tested in a univariable model; higher NYHA classification (&gt;1) (OR 11.3, 95%CI 5.5-23.2), low physical activity (≤3 h/week) (OR 2.1 95%CI 1.3-3.6) and educational level ≤ 12 years (OR 1.9 95%CI 1.2-3.0) were associated with having symptoms. In multivariable analysis, low physical activity level (OR 2.4 95%CI 1.2-5.0) and higher NYHA class (OR 11.3 95%CI 5.0-25.6) remained associated with symptoms during pregnancy. There were no cases with new onset of impaired systemic ventricular function during pregnancy. Conclusion: Symptoms during pregnancy are common in women with CHD but are often already present before pregnancy. Because ordinary symptoms during pregnancy often overlap with symptoms of heart disease, it is important to know if symptoms were present before pregnancy and if they became worse during pregnancy. These results should be included in pre-pregnancy counselling and considered in the monitoring during pregnancy.CC-BY 4.0This work was supported by the Swedish Heart-Lung Foundation, the Heart Foundation of Northern Sweden, the Swedish Children’s Heart Association, and the Swedish Heart and Lung Association.</p

Breastfeeding in primiparous women with congenital heart disease : a register study

Background: The number of pregnant women with congenital heart disease (CHD) is rising, and the disease poses increased risks of cardiovascular and obstetric complications during pregnancy, potentially impacting breastfeeding success. This study aimed to investigate breastfeeding in primiparous women with CHD compared to primiparous women without CHD, and to examine potential hindering factors for breastfeeding in women with CHD. Methods: The data were gathered between 2014 and 2019 and obtained by merging the Swedish Congenital Heart Disease Register (SWEDCON) with the Swedish Pregnancy Register. Primiparous women ≥ 18 years of age with CHD (n = 578) were matched by age and municipality to 3049 women without CHD, giving birth after 22 gestational weeks. Multivariable logistic regression analysis was used to identify factors associated with non-breastfeeding in women with CHD. Results: Fewer women with CHD breastfed than women without CHD two days (94% vs. 97%, p = 0.001) and four weeks after birth (84% vs. 89%, p = 0.006). When all women were analysed, having CHD was associated with non-breastfeeding at both two days and four weeks after birth. For women with CHD, body mass index (BMI) ≥ 30 (OR 3.1; 95% CI 1.4, 7.3), preterm birth (OR 6.4; 95% CI 2.1, 19.0), self-reported history of psychiatric illness (OR 2.4; 95% CI 1.2, 5.1), small for gestational age (OR 4.2; 95% CI 1.4, 12.2), and New York Heart Association Stages of Heart Failure class II − III (OR 6.0; 95% CI 1.4, 26.7) were associated with non-breastfeeding two days after birth. Four weeks after birth, factors associated with non-breastfeeding were BMI ≥ 30 (OR 4.3; 95% CI 2.1, 9.0), self-reported history of psychiatric illness (OR 2.2; 95% CI 1.2, 4.2), and preterm birth (OR 8.9; 95% CI 2.8, 27.9). Conclusions: The study shows that most women with CHD breastfeed, however, at a slightly lower proportion compared to women without CHD. In addition, factors related to the heart disease were not associated with non-breastfeeding four weeks after birth. Since preterm birth, BMI ≥ 30, and psychiatric illness are associated with non-breastfeeding, healthcare professionals should provide greater support to women with CHD having these conditions

Self-rated health in primiparous women with congenital heart disease before, during and after pregnancy : A register study

Background. Poor maternal self-rated health in healthy women is associated with adverse neonatal outcomes, but knowledge about self-rated health in pregnant women with congenital heart disease (CHD) is sparse. This study, therefore, investigated self-rated health before, during, and after pregnancy in women with CHD and factors associated with poor self-rated health. Methods. The Swedish national registers for CHD and pregnancy were merged and searched for primiparous women with data on self-rated health; 600 primiparous women with CHD and 3062 women in matched controls. Analysis was performed using descriptive statistics, chi-square test and logistic regression. Results. Women with CHD equally often rated their health as poor as the controls before (15.5% vs. 15.8%, p = .88), during (29.8% vs. 26.8% p = .13), and after pregnancy (18.8% vs. 17.6% p = .46). None of the factors related to heart disease were associated with poor self-rated health. Instead, factors associated with poor self-rated health during pregnancy in women with CHD were ≤12 years of education (OR 1.7, 95%CI 1.2–2.4) and self-reported history of psychiatric illness (OR 12.6, 95%CI 1.4–3.4). After pregnancy, solely self-reported history of psychiatric illness (OR 5.2, 95%CI 1.1–3.0) was associated with poor self-rated health. Conclusion. Women with CHD reported poor self-rated health comparable to controls before, during, and after pregnancy, and factors related to heart disease were not associated with poor self-rated health. Knowledge about self-rated health may guide professionals in reproductive counselling for women with CHD. Further research is required on how pregnancy affects self-rated health for the group in a long-term perspective

Outpatient volumes and medical staf fing resources as predictors for continuity of follow-up care during transfer of adolescents with congenital heart disease

Background: Providing continuous follow-up care to patients with congenital heart disease (CHD) remains a challenge in many settings. Previous studies highlight that patients with CHD experience discontinuation of followup care, but mainly describe a single-centre perspective, neglecting inter-institutional variations. Hospital-related factors above and beyond patient-related factors are believed to affect continuity of care. The present multicentre study therefore investigated (i) proportion of "no follow-up care"; (ii) transfer destinations after leaving paediatric cardiology; (iii) variation in proportions of no follow-up between centres; (iv) the association between no follow-up and outpatient volumes, and (v) its relationship with staffing resources at outpatient clinics. Methods: An observational, multicentre study was conducted in seven university hospitals. In total, 654 adolescentswith CHD, born between 1991 and 1993, with paediatric outpatient visit at age 14-18 years were included. Transfer status was determined 5 years after the intended transfer to adult care (23y), based on medical files, self-reports and registries. Results: Overall, 89.7% of patients were receiving adult follow-up care after transfer; 6.6% had no follow-up; and 3.7% were untraceable. Among patients in follow-up care, only one remained in paediatric care and the majority received specialist adult CHD care. Significant variability in proportions of no follow-up were identified across centres. Higher outpatient volumes at paediatric outpatient clinics were associated with better continued follow-up care after transfer (OR = 1.061; 95% CI = 1.001 - 1.124). Medical staffing resources were not found predictive. Conclusion: Our findings support the theory of hospital-related factors influencing continuity of care, above and beyond patient-related characteristics