Novel Magnetic Resonance Imaging Tools for Hypertrophic Cardiomyopathy Risk Stratification.

Hypertrophic cardiomyopathy (HCM) is a common genetic disorder with a well described risk of sudden cardiac death; however, risk stratification has remained a challenge. Recently, novel parameters in cardiac magnetic resonance imaging (CMR) have shown promise in helping to improve upon current risk stratification paradigms. In this manuscript, we have reviewed novel CMR risk markers and their utility in HCM. The results of the review showed that T1, extracellular volume, CMR feature tracking, and other miscellaneous novel CMR variables have the potential to improve sudden death risk stratification and may have additional roles in diagnosis and prognosis. The strengths and weaknesses of these imaging techniques, and their potential utility and implementation in HCM risk stratification are discussed.

Stroke Prevention With Left Atrial Appendage Closure in Patients With Atrial Fibrillation and Prior Intracranial Hemorrhage.

Background: Oral anticoagulation (OAC) is deemed a relative contraindication after intracranial hemorrhage (ICH) if the cause cannot be eliminated and the risk of recurrence is high. That leaves atrial fibrillation (AF) patients at high risk of thromboembolic events. Endovascular left atrial appendage closure (LAAC) can be an alternative to OAC for patients requiring stroke prevention. Methods: We performed a retrospective single-centre analysis of 138 consecutive ICH patients with nonvalvular AF and high stroke risk who underwent LAAC between 2010 and 2022 at Vancouver General Hospital. We report the baseline characteristics, procedural results, and follow-up data, comparing the observed stroke/transient ischemic attack (TIA) rate with the predicted event rate based on their CHA2DS2-VASc scores. Results: The average age was 76.1 ± 8.5 years; the mean CHA2DS2-VASc score was 4.4 ± 1.5; and the mean HAS-BLED score was 3.7 ± 0.9. The procedural success rate was 98.6%, and the complication rate was 3.6% with no periprocedural death, stroke, or TIA. The antithrombotic regimen post-LAAC consisted of short-term dual antiplatelet therapy (1-6 months) followed by aspirin alone for a minimum of 6 months in 86.2%. At mean follow-up of 14.7 ± 13.7 months, 9 deaths (6.5%, 7 cardiovascular, 2 noncardiovascular), 2 strokes (1.4%), and 1 TIA (0.7%) had occurred. The annualized observed stroke/TIA rate was 1.8%, which was lower than the adjusted predicted stroke rate of 7.0% (95% confidence interval: 4.8%-9.2%). Two patients (1.5%) suffered another ICH (both on aspirin monotherapy). One device-related thrombus (0.7%) was confirmed and treated with OAC without sequelae. Conclusion: Endovascular LAAC is a feasible alternative to OAC for stroke prevention in patients with nonvalvular AF and prior ICH.

Contexte: L'anticoagulation par voie orale (ACO) est considérée comme une contre-indication relative après une hémorragie intracrânienne (HIC) si la cause ne peut être éliminée et si le risque de récidive est élevé. Les patients souffrant de fibrillation auriculaire (FA) sont donc exposés à un risque élevé d'événements thromboemboliques. La technique de fermeture percutanée de l'appendice auriculaire gauche (AAG) peut être une solution de rechange aux anticoagulants oraux en prévention des accidents vasculaires cérébraux (AVC). Méthodologie: Nous avons réalisé une analyse rétrospective unicentrique auprès de 138 patients consécutifs qui étaient atteints d'une HIC accompagnée d'une FA non valvulaire ainsi que d'un risque élevé d'AVC et qui ont subi une fermeture de l'AAG entre 2010 et 2022 à l'hôpital général de Vancouver. Nous présentons ici les caractéristiques initiales, les résultats de l'intervention et les données de suivi, en comparant le taux d'AVC/AIT (accident ischémique transitoire) observé avec le taux prédit d'événements sur la base de leurs scores CHA2DS2-VASc. Résultats: L'âge moyen était de 76,1 ± 8,5 ans. Le score CHA2DS2-VASc moyen était de 4,4 ± 1,5, et le score HAS-BLED moyen de 3,7 ± 0,9. Le taux de réussite de l'intervention a été de 98,6 % et le taux de complications de 3,6 %, sans décès périopératoires, ni AVC ou AIT. Le traitement antithrombotique après la fermeture de l'AAG consistait en une bithérapie antiplaquettaire de courte durée (de 1 à 6 mois), suivie de la prise d'aspirine seule pendant au moins 6 mois dans 86,2 % des cas. Après un suivi moyen de 14,7 ± 13,7 mois, 9 décès (6,5 %, 7 d'origine cardiovasculaire et 2 d'origine non cardiovasculaire), 2 AVC (1,4 %) et 1 AIT (0,7 %) sont survenus. Le taux annualisé d'AVC/AIT observé était de 1,8 %, ce qui est inférieur au taux prédit d'AVC après ajustement, soit 7,0 % (intervalle de confiance à 95 % : 4,8 % à 9,2 %). Deux patients (1,5 %) ont souffert d'une autre HIC (tous deux sous aspirine en monothérapie). Un thrombus lié au dispositif (0,7 %) a été confirmé et traité par anticoagulathérapie orale sans séquelles. Conclusion: La technique de fermeture de l'AAG représente une solution de rechange à l'anticoagulation par voie orale dans la prévention des AVC chez les patients souffrant de FA non valvulaire et ayant déjà subi une HIC.

Impact of Time to Diagnosis on Morbidity and Survival in Children With Malignant Central Nervous System Tumors.

OBJECTIVE: The aim was to determine the impact of time to diagnosis (TTD) on morbidity and mortality and to identify factors associated with overall survival (OS) in pediatric patients with malignant central nervous system (CNS) tumors. METHODS: This is a retrospective review of all malignant CNS tumors presenting to 2 tertiary care pediatric hospitals from 2000 to 2019. Cox proportional hazard model analysis outcomes included TTD and OS as well as morbidity; stratified by tumor category, age, relapse, and presence of metastatic disease. RESULTS: There were 197 children with malignant CNS tumors (mean age 8.7 y, 61% male). Tumors included medulloblastoma (N=58, 29.4%), ependymoma (N=27, 13.7%), high-grade glioma (N=42, 21.3%), germ cell tumors (N=47, 23.9%), and other embryonal tumors (N=23, 11.7%). Median TTD from symptom onset was 62 (interquartile range: 26.5 to 237.5 d) and 28% had metastatic disease. Three-year progression free survival was 55% and 3-year OS was 73.1%. Increased OS was associated with increased TTD (parameter estimate 0.12; confidence interval [CI]: 0.019-7.06; P =0.019), high-grade glioma (hazard ratio [HR]: 2.46; CI [1.03-5.86]; P =0.042), other embryonal tumor (HR: 2.84; CI [1.06-7.56]; P =0.037), relapse (HR: 10.14; CI: 4.52-22.70; P <0.001) and metastatic disease (HR: 3.25; CI: 1.51-6.96; P =0.002). Vision change (HR: 0.58; CI: 0.313-1.06; P =0.078), hearing loss (HR: 0.71; CI: 0.35-1.42; P =0.355), and cognitive impairment (HR: 0.73; CI: 0.45-1.19; P =0.205) were not associated with TTD in this model. CONCLUSIONS: Increased median TTD is associated with higher OS in pediatric patients treated for malignant CNS tumors. Tumor biology and treatment modality are more important factors than TTD for predicting morbidity and long-term outcomes in pediatric patients with CNS tumors.

Effects of Bisphosphonate Therapy on Bone Mineral Density in Boys with Duchenne Muscular Dystrophy.

The objective of this study was to estimate the comparative effectiveness of bisphosphonate therapy on bone mineral density (BMD) in patients with corticosteroid-treated Duchenne muscular dystrophy (DMD). A retrospective, comparative effectiveness study evaluating changes in BMD and fragility fractures in patients with DMD presenting to British Columbia Children's Hospital from 1989 to 2017 was conducted. Marginal structural generalized estimating equation models weighted by stabilized inverse-probability of treatment weights were used to estimate the comparative effectiveness of therapy on BMD. Of those treated with bisphosphonates (N = 38), 7 (18.4%), 17 (44.7%), and 14 (36.8%) cases were treated with pamidronate, zoledronic acid, or a combination of both, respectively, while 36 cases of DMD were untreated. Mean age of bisphosphonate initiation was 9.2 (SD 2.7) years. Mean fragility fractures declined from 3.5 to 1.0 following bisphosphonate therapy. Compared to the treated group, the untreated group had an additional 0.63-SD decrease (95% confidence interval [CI]: -1.18, -0.08, P = .026) in total BMD and an additional 1.04-SD decrease (95% CI: -1.74, -0.34; P = .004) in the left hip BMD, but the change in lumbar spine BMD (0.15, 95% CI: -0.36, 0.66; P = .57) was not significant. Bisphosphonate therapy may slow the decline in BMD in boys with corticosteroid-treated DMD compared to untreated counterparts. Total number of fragility fractures decreased following bisphosphonate therapy.