Utility of Cardiac Magnetic Resonance Imaging in Predicting Atrial Arrhythmias in Repaired Tetralogy of Fallot.

Arrhythmias are the leading cause of morbidity and mortality in repaired tetralogy of Fallot (TOF), and over 20% of these patients will develop a sustained atrial arrhythmia during their lifetimes. Cardiac magnetic resonance imaging (cMRI) is frequently performed in TOF, although its ability to identify patients at risk of atrial arrhythmias is uncertain. Adult TOF patients (n = 175) with no history of atrial arrhythmia who underwent cMRI between 2003 and 2020 at a single tertiary care center were identified. Clinical characteristics and imaging findings were evaluated to identify a predilection for atrial arrhythmias using Kaplan-Meier survival analysis and log-rank testing. Multivariable Cox regression was used to determine independent predictors of atrial arrhythmias. Over a median follow-up of 3.6 years, 29 patients (17%) developed atrial arrhythmias. Independent predictors of atrial arrhythmia included age (hazard ratio [HR] 1.06 per 1-year increase, 95% confidence interval [CI] 1.02 to 1.09, p = 0.002), diabetes mellitus (HR 4.26, 95% CI 1.26 to 14.41, p = 0.020), indexed right ventricular end-diastolic volume (RVEDVi), (HR 1.20 per 10-ml/m2 increase, 95% CI 1.05 to 1.39, p = 0.010), and moderate or greater tricuspid regurgitation (TR) (HR 6.32, 95% CI 2.15 to 18.60, p = 0.001). Utilizing Kaplan-Meier analysis, patients with at least mild right ventricular dilation (RVEDVi >100 ml/m2, p = 0.047) and greater than or equal to moderate TR (p <0.001) were found to be significantly more likely to develop atrial arrhythmias. In conclusion, cMRI can help to identify TOF patients at increased risk for atrial arrhythmia beyond standard clinical and imaging data by better quantifying RVEDVi and degree of TR.

Exercise Capacity and Predictors of Performance After Fontan: Results from the Pediatric Heart Network Fontan 3 Study.

Impaired exercise following Fontan is a surrogate of morbidity. Single-center longitudinal data exist, but there is a lack of contemporary multi-center data. Ramp cycle ergometry was re-performed in consented participants who had originally participated in the Pediatric Heart Network's Fontan cross-sectional study. Annualized change was evaluated at maximal and submaximal exercise. Associations between these outcomes and patient characteristics were analyzed. There were 336 participants in Fontan 3, mean age 23.2 years. Paired measurements of peak oxygen consumption (peak VO2) were available for 95; peak exercise data at Fontan 3 were available for 275. Percent-predicted peak VO2 declined by 0.8 ± 1.7% per year (p < 0.001). At Fontan 3, the lowest performing peak VO2 tertile had the highest rate of overweight and obesity (p < 0.001). Female gender was more prevalent in the highest performing tertile (p = 0.004). Paired data at the ventilatory anaerobic threshold (VO2 at VAT) were available for 196; VAT data at Fontan 3 were available for 311. Percent-predicted VO2 at VAT decreased by 0.8 ± 2.6% per year (p < 0.001). At Fontan 3, VO2 at VAT was better preserved than peak VO2 across all tertiles, with higher rates of overweight and obesity in the lower performing group (p = 0.001). Female gender (p < 0.001) and left ventricular morphology (p = 0.03) were associated with better performance. Submaximal exercise is better preserved than maximal in the Fontan population, but declined at the same rate over the study period. The overall longitudinal rate of decline in exercise performance is slower than what has been described previously.

A Minimally Invasive Approach for Placing Sew-On Epicardial Leads in the Child.

Traditionally, pacing leads are placed transvenously, although smaller pediatric patients who require permanent pacemakers may benefit from delaying tranvenous lead placement until they are larger. Alternative, minimally invasive atrioventricular pacing options have not previously existed for this patient population, leaving many of these children with large sternotomies or thoracotomies. Using three port sites and an adjustable shaft dual-needle suturing device, we placed a steroid-eluting, sew-on epicardial lead on the right atrium of a 9-year-old patient. This is one of the earliest reported cases of a minimally invasive technique for sew-on epicardial lead placement on the atrium of a child. Although based on a single case, we believe that this approach is safe, reliable, and reproducible and that it can be used to place leads on any aspect of the heart. Adoption of this technique will allow for earlier atrioventricular pacing, which may decrease the incidence of pacemaker syndrome, and thus improve overall outcomes in this patient population.

Life-Threatening Event Risk in Children With Wolff-Parkinson-White Syndrome: A Multicenter International Study.

OBJECTIVES: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population. BACKGROUND: Children with WPW syndrome are at risk of sudden death. METHODS: This retrospective multicenter pediatric study identified 912 subjects ≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrial fibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of ≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects. RESULTS: Case subjects (n = 96) were older and less likely than subjects (n = 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL ≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had ≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia. CONCLUSIONS: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS.

Premature ventricular contraction-induced cardiomyopathy in children.

BACKGROUND: Adults with high premature ventricular contraction burden can develop left ventricular dilation, dysfunction, and strain, consistent with a cardiomyopathy, which is reversible with radiofrequency ablation of the premature ventricular contractions. Evidence in children with similar ectopy burden is limited. We performed a single-centre retrospective review to examine the prevalence of premature ventricular contraction-induced cardiomyopathy, natural history of ventricular ectopy, and progression to ventricular tachycardia in children with frequent premature ventricular contractions. METHODS: Children aged between 6 months and 18 years, with premature ventricular contractions comprising at least 20% of rhythm on 24-hour Holter monitor, were included in our study. Those with significant structural heart disease, ventricular tachycardia greater than 1% of rhythm at the time of premature ventricular contraction diagnosis, or family history of cardiomyopathy - except tachycardia-induced - were excluded. Cardiomyopathy was defined by echocardiographic assessment. RESULTS: A total of 36 children met the study criteria; seven patients (19.4%, 95% CI 6.2-32.6%) met the criteria for cardiomyopathy, mostly at initial presentation. Ectopy decreased to <10% of beats without intervention in 16.7% (95% CI 4.3-29.1%) of the patients. No patient progressed to having ventricular tachycardia as more than 1% of beats on follow-up Holter. Radiofrequency ablation was performed in three patients without cardiomyopathy. CONCLUSIONS: Our study demonstrates a higher prevalence of cardiomyopathy among children with high premature ventricular contraction burden than that previously shown. Ectopy tended to persist throughout follow-up. These trends suggest the need for a multi-centre study on frequent premature ventricular contractions in children. In the interim, regular follow-up with imaging to evaluate for cardiomyopathy is warranted.

Hyperpolarized helium-3 MR imaging of pulmonary function.

Recent advances in HP MR imaging contrast agents have led to novel tests of pulmonary function. Many of these tests show promise in the clinical arena.

Determination of regional VA/Q by hyperpolarized 3He MRI.

Alveolar ventilation/perfusion ratio (VA/Q) is a key parameter in functional imaging of the lung. Herein, regional VA/Q was calculated from regional values of alveolar partial pressure of oxygen (PAO2) measured by hyperpolarized 3He gas MRI (HP 3He MRI). Yorkshire pigs (n = 7, mean weight = 25 kg) were paralyzed and maintained under isoflurane anesthesia. Animals were placed into a birdcage coil, then transferred to the bore of a 1.5 T MRI unit. Prior to imaging, animals were manually ventilated with room air for 5 min, then a 3He gas mixture was administered during breathhold and imaging performed. PAO2 was measured based on the decay rate of 3He signal. Subjects' blood gas concentrations were measured and these values and PAO2 values entered into a system of four equations with four unknowns. Calculated VA/Q values were analyzed by preparing frequency distributions for the entire lung and compared to VA/Q frequency distributions previously established in the literature as normal using other diagnostic techniques. Distributions were consistent with those in the literature, indicating that HP 3He MRI may be an accurate, quantitative, noninvasive, and nonradioactive method for acquiring VA/Q for small regions of the lung.

An evaluation of pulmonary atelectasis and its re-expansion: hyperpolarized 3He MRI in the Yorkshire pig.

RATIONALE AND OBJECTIVES: Atelectasis, the collapse of small airways, is a significant clinical problem. We use hyperpolarized (HP) 3He magnetic resonance imaging (MRI), or HP 3He MRI, to describe atelectasis in the normal Yorkshire pig, the pig with atelectasis, and the pig with re-expansion of atelectasis. We compare HP 3He MRI findings with depictions of atelectasis by proton MRI. MATERIALS AND METHODS: During end-expiration in the anesthetized and paralyzed Yorkshire pig (n = 6), HP 3He gas produced by the optical pumping spin-exchange method, was delivered via an endotracheal tube. For two separate groups, atelectasis was either induced by Fogarty-catheter occlusion balloon inflation (n = 3), or lateral chest wall administration of sodium hydroxide (NaOH) (n = 3). MRI was performed at time zero, at 5, 9, 13, 15, and 19 minutes after atelectasis production, 30 minutes after balloon deflation, and 10 and 30 minutes after recruitment of atelectatic areas with increased tidal volumes and added positive end-expiratory pressure. High-resolution, cross-sectional MR images were procured, and comparison was made with the traditional proton MRI. RESULTS: Atelectatic areas by HP 3He MRI were easily distinguishable in both subject groups, and correlated with those located by proton MR. HP 3He MR images showed absence of ventilation, whereas proton MR images depicted dense, white areas. Re-expansion of atelectasis was well delineated by HP 3He MRI. CONCLUSION: HP 3He MRI may overcome many of the shortcomings of other well-established radiographic methods. HP 3He MRI is a novel, informative method for describing atelectasis and its re-expansion.