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 (>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.

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.

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 (>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 ± 25 vs. 107 ± 22 [lower PR fraction, P < 0.01] and 93 ± 15 ml/m2 [healthy, P < 0.001] for RV end-diastolic volume index [RVEDVI]) and higher TKE values (5.95 ± 3.15 vs. 2.23 ± 0.81 [lower PR fraction, P < 0.01] and 1.91 ± 0.78 mJ [healthy, P < 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 (R2 = 0.47, P = 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. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018;47:1043-1053.

Ischemic Stroke in Adults With Congenital Heart Disease: Cumulative Incidence and Associated Factors.

BACKGROUND: When more patients with congenital heart disease reach adult age, increased incidence of long-term complications, including ischemic stroke, are expected. The aim of this study was to analyze cumulative incidence of ischemic stroke, associated factors, and case fatality in adult congenital heart disease. METHODS AND RESULTS: The study is based on Swedish national registers on congenital heart disease and stroke. Patients with congenital heart disease were followed between 2001 and 2018 for first-ever ischemic stroke events (ischemic stroke due to patent foramen ovale excluded). Factors possibly associated with ischemic stroke were analyzed using Cox regression models. Out of 8914 adult patients with congenital heart disease, 108 suffered ischemic stroke over a mean period of 7.6±4.7 years. The mean age at ischemic stroke was 53.8 years, and the cumulative incidence was 0.15% at 1 year, 0.5% at 5 years, and 1.5% at 10 years. In multivariable analysis, age (hazard ratio [HR], 1.04 [95% CI, 1.03-1.06]), diabetes (HR, 2.9 [95% CI, 1.3-6.4]), ejection fraction <50% (HR, 1.9 [95% CI, 1.1-3.4]), atrial septal defect (HR, 3.0 [95% CI, 1.03-8.5]), and aortic valve lesions (HR, 4.8 [95% CI, 1.6-14.1]) were associated with increased risk. Among those with ischemic stroke, approximately half were on antithrombotic treatment (anticoagulants or antiplatelets) before admission. Case fatality was 6.5%. CONCLUSIONS: The cumulative incidence of ischemic stroke was 1.5% after 10 years. In adult congenital heart disease, the type of heart lesion, diabetes, ejection fraction <50%, and age were important factors associated with ischemic stroke. Despite a relatively young age, mortality is a significant threat. At time of the ischemic stroke event, approximately half of the patients were on antithrombotic treatment.

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.

An uncommon case of congenital thoracic venous anomaly and extracardiac sinus venosus defect in an asymptomatic adult first presenting with brain abscesses: a case report.

Background: Congenital thoracic venous anomalies (CTVAs) with right-to-left shunt constitute an uncommon source of paradoxical embolization in adults. We present a case of a healthy and physically fit individual with a rare asymptomatic anomaly first presenting with brain abscesses after a visit to the dental office; persistent left superior vena cavae (PLSVC) without bridging vein, over-riding right-sided superior vena cavae (RSVC) connected to the left atrium (LA), and an extracardiac sinus venosus defect. Case summary: A 29-year-old male presented to the neurosurgical unit due to intracranial abscesses requiring intervention following a visit to his dentist. The abscess cultures isolated bacteria commonly found in the normal oral flora. Transthoracic echocardiography revealed an enlarged coronary sinus consistent with PLSVC. An agitated saline study was performed and raised suspicion of simultaneous extra- and intracardiac shunting. Magnetic resonance angiography confirmed the presence of a PLSVC and revealed an RSVC connected to the LA; however, no intracardiac shunt was evident. Electrocardiogram-gated computed tomography was therefore conducted and discovered the rudimentary remains of the physiologic RSVC forming a connection to the right atrium, explaining the bilateral contrast loading seen on the agitated saline study and diagnosing an extracardiac sinus venosus defect (SVD). The patient recovered and has been referred for surgery. Discussion: This case illustrates a CTVA and a forme fruste type SVD resulting in a severe complication in a healthy adult. We highlight the diagnostic challenges posed, suggest early usage of agitated saline studies, and discuss the rationale for surgical correction of this patient.

Three-dimensional echocardiography to identify right ventricular dilatation in patients with corrected Fallot anomaly or pulmonary stenosis.

BACKGROUND: 3-Dimensional Echocardiography allows measuring volumes and parameters of myocardial deformation (strain). Myocardial strain has been suggested to be superior to conventional echo parameters in the assessment of right ventricular (RV) function. Myocardial strain can be assessed by cardiac magnetic resonance (CMR) or two- and three-dimensional echocardiography (2D and 3DEcho). We performed a comprehensive assessment of the RV based on 3DEcho and compared the results with those based on CMR and 2DEcho. METHODS: 36 patients with corrected heart defects underwent CMR and 3DEcho to assess RV volume, strain and cardio pulmonary exercise testing with peak VO2 measurement. 2DEcho was used for reference. RESULTS: There was a moderate correlation between 3DEcho and CMR for measuring RV end-diastolic and end-systolic volumes (r = .82 and .72). 3DEcho tended to underestimate the RV volumes, mean difference EDV 8.5 ± 33 ml (CI -2.8; 19.7 ml) and ESV 13.2 ± 29 ml (CI 3.3; 23 ml). According to method-specific reference values for RVEDV, 34/35 (3DEcho) and 29/36 (CMR) were dilated. Among those dilated according to CMR, all were identified by 3DEcho. The coefficient of correlation between RV atrioventricular plane displacement measured by CMR and tricuspid annular plane systolic excursion measured by 3D and 2DEcho was r = .6 for both. 2DEcho measured lower LV volumes than CMR. LVEF and GLS were similar in 2DEcho, 3DEcho and CMR. Patients with CMR-determined RV free wall strain ≤ -14% tended to have lower peak VO2 . CONCLUSIONS: Although 3DEcho underestimated RV volumes, it successfully identified all patients with RV dilatation based on method-specific reference values.

Outpatient volumes and medical staffing 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 follow-up 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 adolescents with 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.

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<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<0.001). Both LVAVPD and RVAVPD were significantly depressed in patients compared to controls (p<0.05). RVAVPD was more depressed in patients with a high systolic RV pressure than in those with normal RV pressure (p<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.

Catheter closure of atrial septal defect in the elderly (≥65years). 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 65-87years. 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.