Pulsed field ablation (PFA) is a non-thermal technique increasingly used for atrial fibrillation, with limited data in atrial tachycardia (AT) and none in Fontan patients. We report a 42-year-old man with complex congenital heart disease post-Fontan presenting with incessant AT. Electrophysiologic study identified multiple tachycardia circuits; two were successfully treated with radiofrequency ablation. A third focus near the left upper pulmonary vein was terminated using PFA, with no further inducible arrhythmias. This case demonstrates the feasibility and potential effectiveness of PFA in complex Fontan-related AT, though long-term outcomes remain unknown.
Background Foetal echocardiography (FE) is widely recommended in pregnancies at increased risk of congenital heart defects. While FE is a safe diagnostic tool, its psychological impact on expectant pregnant women remains poorly understood. Methods We conducted a prospective observational study in eight Canadian centers. Women aged ≥18 years with a normal second-trimester ultrasound between 18 and 26 weeks’ gestation referred for a FE were compared to women who were not. Anxiety was assessed using the Spielberger State–Trait Anxiety Inventory (STAI). The primary outcome was the difference between state and trait anxiety (net situational anxiety) after morphological ultrasound, but before FE (T0). Secondary outcomes included the proportion of participants with significant anxiety (STAI ≥40) and anxiety trajectories after FE among referred participants. Results Eighty-four women were included, of whom 38 were referred for FE. At T0, women referred for FE showed slightly higher median net situational anxiety compared with controls. In multivariable analyses, FE referral was associated with a modest increase although estimates were imprecise. A higher proportion of referred women reached a state anxiety score ≥40, though this association was weak after adjustment. Among the 14 women who completed post-FE questionnaires, anxiety trajectories were heterogeneous, with both increases and decreases observed following FE. Conclusions Referral for FE following a normal second-trimester ultrasound is associated with a small and variable increase in maternal anxiety. These findings suggest that FE referral may not be psychologically neutral and highlight the importance of considering maternal psychological responses as referral criteria broaden.
Background Pulmonary blood flow (Qp) measured by oximetry and Fick’s principle (FP) is unreliable if there is multifocal pulmonary blood supply with differing oxygen contents. This often occurs in single ventricle patients with a bidirectional cavopulmonary connection (BCPC) and aortopulmonary collaterals (APC). We aimed to compare the difference between cardiac magnetic resonance (CMR) measured and Fick-estimated Qp (Fick-Qp) and pulmonary vascular resistance (PVRI) at pre-Fontan assessment. Methods A retrospective analysis of BCPC patients who underwent combined CMR and catheterization under the same general anesthetic. CMR flows were measured in the superior vena cava (CMR-Qsvc), pulmonary veins (CMR-Qpv) and the difference between these was APC flow (Qapc). Oximetric Fick-Qp was compared to CMR-Qsvc and CMR-Qpv. Results Ninety-five patients were included, median (IQR): age 2.92 (2.32 - 3.38) years and weight 12.8 (11.95 - 14.65) kg. The rank order of flows was CMR-Qpv > Fick-Qp > CMR-Qsvc, median values (IQR): 3.37 (2.97 - 3.98) > 2.39 (2.09 - 2.83) > 1.91 (1.67 - 2.25) L/min/m2. Hence PVRI derived from these flows were in the reverse rank order, PVRI-CMR-Qpv < PVRI-Fick-Qp <PVRI-CMR-Qsvc, mean values (95%CI): 1.37 (1.04 - 1.67) < 1.8 (1.49 - 2.18) < 2.38 (1.81 - 2.84). Conclusions In comparison to the reference method of CMR-Qpv, the oximetric Fick method underestimates pulmonary blood flow and overestimates PVRI. In patients with high Fick based PVRI, a reassessment using CMR based measurement of pulmonary blood flow should be considered for more accurate measurement of PVRI and risk stratification.
Background Despite excellent survival following Tetralogy of Fallot (TOF) repair, pulmonary regurgitation (PR) is common. Pulmonary valve replacement (PVR) is frequently performed, but its long-term efficacy remains uncertain. We conducted a meta-regression to assess the impact of PVR on key cardiac outcomes over time. Methods We included studies reporting PR, right ventricular (RV) and left ventricular volumes (LV), QRS duration, RV ejection fraction (RVEF), peakVO2, or NYHA class before and after PVR in patients with TOF. A multilevel random-effects meta-regression was performed using time since PVR as a covariate. Cubic splines were used to explore temporal trends, and meta-analysis to quantify the impact of PVR over time. Results Sixty-nine studies were included. Compared with pre-PVR values, PVR yielded substantial absolute reductions in PR fraction, RV end-systolic and end-diastolic volume. LV end-diastolic volume increased slightly. NYHA class also improved. Median follow-up was 14.4 months (IQR: 10.3-31.1) with only one study extending beyond 10 years. Estimated annual changes after PVR were minimal for all parameters and negligible for key parameters such as PR fraction (0.36%, 95%CI: –0.05% to 0.77%), RV end-systolic volume (–0.58 mL/m2, 95%CI: –1.62 mL/m2 to 0.45 mL/m2), RV end-diastolic volume (–0.49 mL/m2, 95%CI: –0.72 mL/m2 to 1.72 mL/m2). Conclusions The long-term impact of PVR remains uncertain. While short-term improvements are clear, insufficient long-term data preclude firm conclusions on sustained benefits.
Background:Arrhythmia identification in the intensive care unit (ICU) is important to prevent ICU morbidity and mortality. Timely arrhythmia detection relies on bedside providers' telemetry interpretation. Machine learning models can function as clinical support tools to facilitate diagnoses. Machine learning model development requires well-curated training data. The differential performance between labelers of different roles and experience is currently unknown. Methods:This was a prospective observational study with frontline providers. A total of 300 (200 original and 100 duplicate) telemetry tracings were labeled as sinus rhythm, second-/third-degree atrioventricular block, junctional ectopic tachycardia, ectopic atrial tachycardia, and reentrant supraventricular tachycardia. Inter-rater reliability was calculated against the ground truth label (read by an electrophysiologist, AB) as the primary performance measure (intrarater reliability for consistency using duplicate labels). Results:A total of 11 participants completed the study: 1 cardiology fellow, 4 pediatric ICU fellows, 2 pediatric cardiac ICU fellows, 3 pediatric cardiac ICU nurse practitioners, and 1 pediatrics resident. The highest level of agreement was moderate (κ = 0.68, P < 0.001), with the majority poor to moderate. Performance varied by rhythm type (median κ): sinus (0.61), atrioventricular block (0.68), junctional (0.47), ectopic atrial tachycardia (0.25), and supraventricular tachycardia (0.49). There was good intrarater reliability (κ = 0.71 [median], P < 0.001). Conclusions:Overall, frontline provider performance was suboptimal, especially for complex arrhythmia classes. These findings highlight the need for thoughtful consideration in labeler training and validate the need for a clinical decision support tool in arrhythmia detection.
Background There are limited data about the prognostic role of N-terminal pro-B-type natriuretic peptide (NT-proBNP) in adults with Fontan palliation. The purpose of this study was to determine the correlates of, and the prognostic value of serial NT-proBNP assay for risk stratification in adults with Fontan palliation. Method Retrospective cohort study of adults with Fontan palliation that had ≥1 NT-proBNP measurements in the outpatient clinic (2003-2023). In patients with ≥3 NT-proBNP measurements, serial NT-proBNP was calculated as the mean of the first 3 measurements. Study outcome was death/transplant. Results Of 369 patients (age 27±8 years; 55% males) with NT-proBNP, 214 (58%) had serial measurements. The baseline level was 217 (82, 562) pg/ml while the serial value was 261 (98, 715) pg/ml. The correlates of higher NT-proBNP levels were older age, atriopulmonary connection, atrial fibrillation, hepatorenal dysfunction, systemic ventricular dilation and systolic dysfunction, and higher Fontan pressures and pulmonary artery wedge pressure. Baseline as well as serial NT-proBNP levels were associated with death/transplant independent of anatomic indices, comorbidities and hemodynamic indices. Serial NT-proBNP levels had superior prognostic value compared to single (baseline) NT-proBNP measurements, echocardiographic, cardiac magnetic resonance imaging indices, and cardiac catheterization indices. Conclusions These findings have important clinical implications in the management of adults with Fontan palliation since NT-proBNP assay is inexpensive, readily available, and noninvasive, and hence ideal for longitudinal assessment. Further studies are required to determine whether NT-proBNP can be used as a surrogate marker to monitor response to therapeutic interventions in this population.
Background:In the acquired heart disease population, heart failure (HF) is broadly classified based on left ventricular (LV) ejection fraction (EF) as HF with reduced EF (HFrEF), HF with midrange EF (HFmrEF), and HF with preserved EF (HFpEF). The prognostic role of this LVEF-based HF classification has not been evaluated in the adult congenital heart disease (CHD) population because of anatomic and physiological heterogeneity within this population. The current study aimed to address this knowledge gap, by assessing the prevalence, predictors (correlates), and prognostic implications of HFrEF in adults with CHD. Methods:This is a retrospective cohort study of adults with CHD and morphologic LV as the systemic ventricle. Patients were classified as HFrEF (LVEF ≤40%), HFmrEF (LVEF 41%-49%), and pEF (LVEF ≥50%). Results:Of 7981 patients, 7203 (90%) had pEF, 451 (6%) had HFmrEF, and 327 (4%) had HFrEF. The correlates of HFrEF were older age, male sex, pacemaker implantation, multiple prior sternotomies, and acquired comorbidities such as hypertension, coronary artery disease, diabetes, and atrial fibrillation. The 15-year mortality risk was 18%, 36%, and 67% in the pEF, HFmrEF, and HFrEF groups, respectively (P < 0.001). Both HFmrEF (adjusted hazard ratio: 1.64 [95% confidence interval: 1.29; 2.09], P < 0.001) and HFrEF (adjusted hazard ratio: 3.33 [95% confidence interval: 2.72; 4.27], P < 0.001) were associated with mortality on multivariable analysis. Conclusions:Given the elevated risk of mortality in patients with LV systolic dysfunction (both mrEF and rEF) and the emerging data suggesting the efficacy of HF therapy in patients with CHD, there is an urgent need to implement HF therapy in this population.
Background:Pediatric Marfan syndrome (MFS) is associated with changes in aortic stiffness, endothelial function, and ventricular performance. Resting measurements may not fully capture cardiovascular functional reserve. Exercise stress echocardiography can reveal subclinical impairments not evident at rest. Methods:This study included 31 pediatric patients with and 31 age-matched controls. Resting and exercise echocardiography were performed to assess systolic and diastolic function, aortic dimensions, and vascular parameters. Results:Patients with MFS had larger baseline height and body surface area values compared with controls. Significant baseline differences in aortic dimensions, diastolic function, endothelial function, and vascular stiffness were observed in patients with MFS. Despite these differences, patients with MFS demonstrated preserved systolic and diastolic reserve during exercise. Vascular responses to exercise were comparable to controls, reflecting increased stiffness and altered endothelial function but preserved adaptability. Conclusions:Preserved cardiovascular reserve during exercise suggests that baseline abnormalities may not result in significant dysfunction, supporting safe participation in endurance activities for pediatric patients with MFS.
Background:We aimed to compare health-related quality of life (HRQL) using a disease-specific and a generic instrument in children after complex cardiac surgery in early infancy. Methods:A prospectively followed inception cohort of children having complex cardiac surgery at age ≤6 weeks had HRQL assessed at age 4-5 years using Pediatric Quality of Life Inventory (PedsQL) 3.0 Cardiac Module. Scores were compared with the PedsQL Generic Core Scales in the same population. Predictors of Cardiac Module dimensions were determined by univariate and multivariate linear regression, with P ≤ 0.05 considered statistically significant. Results:Among 425 patients who had the PedsQL 3.0 Cardiac Module, the HRQL mean (standard deviation) scale scores were as follows: Heart Problems and Treatment, 78.7 (19.0); Treatment II, 94.5 (15.2); Perceived Physical Appearance, 93.6 (13.9); Treatment Anxiety, 71.1 (29.5); Cognitive Problems, 67.3 (22.7); and Communication, 67.5 (31.2). Correlations between Cardiac Module and Generic Core Scales were as follows: Heart Problems and Treatment × Physical Functioning 0.57; Perceived Physical Appearance × Psychosocial Summary 0.21; Cognitive Problems × School Functioning 0.48; Treatment Anxiety × Physical Functioning 0.38; and Communication × School Functioning 0.30. Thirteen predictor variables were independently associated with at least 1 dimension of the Cardiac Module, and these only partially overlapped with independent predictors of Generic Core Scale summary scores. Conclusions:The Cardiac Module measured a different HRQL construct than the Generic Core Scales, reflected by low correlations among scores and different independent predictors in the same population of children. Future studies should determine the relation of these scores with longer-term outcomes including school achievement, mental health, and well-being.
Background:Bronchial length ratio (BLR) is widely used for situs determination. However, BLR may also be influenced by the position of the aortic arch. We aimed to evaluate the effect of aortic arch position on BLR. Methods:Patients were identified using the keywords left aortic arch (LAA), right aortic arch (RAA), double aortic arch, situs inversus, right isomerism, and left isomerism. The ratio between the longer and shorter bronchi was calculated. BLR and bronchopulmonary arterial relationship were compared across groups with different combinations of body situs and aortic arch position. Results:Patients included 69 with situs solitus and LAA (group 1A), 20 with situs inversus and RAA (group 1B), 43 with situs solitus and RAA (group 2A), 2 with situs inversus and LAA (group 2B), 11 with situs solitus and double aortic arch (group 3), and 14 with right or left isomerism (group 4). Group 2 demonstrated a significantly smaller BLR than group 1 (P = 0.001). Group 3 showed a smaller BLR than group 1 but without statistical significance. Group 4 had a significantly smaller BLR than groups 1-3 (P < 0.001). None of group 1 had a BLR within the heterotaxy range. BLR ranges of 11% of group 2 and 9% of group 3 overlapped with the heterotaxy range. Bronchopulmonary arterial relationship was consistent with the situs of the remainder of the body in all. Conclusions:BLR is influenced by aortic arch position, limiting its use for situs determination. Bronchopulmonary arterial relationship should be used as the primary criterion for situs determination.
