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Objectives: To determine guideline adherence pertaining to pulmonary valve replacement (PVR) referral after tetralogy of Fallot (TOF) repair. Methods: Children and adults with cardiovascular magnetic resonance imaging scans and at least moderate pulmonary regurgitation were prospectively enrolled in the Comprehensive Outcomes Registry Late After TOF Repair (CORRELATE). Individuals with previous PVR were excluded. Patients were classified according to presence (+) versus absence (−) of PVR and presence (+) versus absence (−) of contemporaneous guideline satisfaction. A validated score (specific activity scale [SAS]) classified adult symptom status. Results: In total, 498 participants (57% male, mean age 32 ± 14 years) were enrolled from 14 Canadian centers (2013-2020). Mean follow-up was 3.8 ± 1.8 years. Guideline criteria for PVR were satisfied for the majority (n = 422/498, 85%), although referral for PVR occurred only in a minority (n = 167/498, 34%). At PVR referral, most were asymptomatic (75% in SAS class 1). One participant (0.6%) received PVR without meeting criteria (PVR+/indication–). The remainder (n = 75/498, 15%) did not meet criteria for and did not receive PVR (PVR–/indication–). Abnormal cardiovascular imaging was the most commonly cited indication for PVR (n = 61/123, 50%). The SAS class and ratio of right to left end-diastolic volumes were independent predictors of PVR in a multivariable analysis (hazard ratio, 3.33; 95% confidence interval, 1.92-5.8, P

Objectives: A quality improvement initiative was introduced to the adult congenital cardiac surgery program at Toronto General Hospital in January 2016. A dedicated Adult Congenital Anesthesia and intensive care unit team was introduced within the cardiac group. The use of factor concentrates was introduced. The study compares perioperative mortality, adverse events, and transfusion burden before and after this process change. Methods: We performed a retrospective analysis of all adult congenital cardiac surgeries from January 2004 to July 2019. Two groups were analyzed: patients undergoing operation before and after 2016. The primary outcome was in-hospital mortality. One-year mortality and prevalence of key morbidities were analyzed as secondary outcomes. A separate analysis looked at patients who had and had not attended an anesthesia-led preassessment clinic. Results: In-hospital mortality was significantly reduced in patients undergoing operation after 2016 (1.1% vs 4.3%, P = .003) despite a higher risk profile. One-year mortality (1.3% vs 5.8%, P = .003) and ventilation times (5.5 hours [3.4-13.0] vs 6.3 hours [4.2-16.2], P = .001) were also reduced. The incidence of stroke and renal failure was similar between groups. Blood product exposure was comparable, but the incidence of chest reopening decreased (1.8% vs 4.8%, P = .022), despite more patients with multiple previous chest wall incisions, on anticoagulation, and with more complex cardiac anatomy. There were no significant outcome differences between those who did or did not attend the preassessment clinic. Conclusions: Both in-hospital and 1-year mortality were significantly reduced after the introduction of a quality improvement program, despite a higher risk profile. Blood product exposure remained unchanged, but there were less chest reopenings.

Background: Implantable cardioverter defibrillators (ICDs) are effective in preventing arrhythmic sudden cardiac death in patients with tetralogy of Fallot (TOF). Although ICD therapies for malignant ventricular arrhythmias can be life-saving, shocks could have deleterious consequences. Substrate-based ablation therapy has become the standard of care to prevent recurrent ICD shocks in patients with ischemic cardiomyopathy. However, the efficacy and safety of this invasive therapy in the prevention of recurrent ICD shocks in patients with TOF has not been well evaluated. Methods: Records of a total of 47 consecutive TOF patients (mean age: 43.1 ± 13.2 years, male sex: n = 34 [72.3%]) who underwent ICD implantation for secondary prevention between 2000 and 2018 were reviewed. Results: Twenty (42.6%) patients underwent invasive therapy (radiofrequency catheter ablation, n = 8; surgical ablation with pulmonary valve replacement, n = 12) before ICD implantation. Twenty-seven patients (57.4%) were managed noninvasively. During follow-up (median 80.5 [interquartile range, 28.5-131.0] months), 2 (10.0%) patients in the invasive group and 10 (37.0%) patients in the noninvasive group received appropriate ICD shocks (P = 0.036). Logistic regression analysis showed that invasive therapy was associated with a decreased risk of ICD shocks by 81.1% (odds ratio, 0.189; 95% confidence interval, 0.036-0.990; P = 0.049). Furthermore, invasive therapy was associated with decreased risk of the composite outcomes of ICD shock, death, cardiac transplantation, and hospital admission (odds ratio, 0.090; 95% confidence interval, 0.025-0.365; P = 0.013) compared with noninvasive therapy. Conclusions: Invasive substrate modification therapy was associated with a lower likelihood of ICD shocks and improvement of long-term outcomes in TOF patients. Résumé: Contexte: Les défibrillateurs cardioverteurs implantables (DCI) sont efficaces pour prévenir la mort cardiaque subite provoquée par une arythmie chez les patients présentant une tétralogie de Fallot (TF). Bien que le traitement des arythmies ventriculaires malignes par DCI puisse sauver des vies, les chocs administrés peuvent avoir des conséquences délétères. L’ablation du substrat est devenue le traitement de référence pour prévenir l’administration à répétition de chocs par DCI chez les patients atteints d’une cardiomyopathie ischémique. L’efficacité et l’innocuité de ce traitement invasif pour prévenir l’administration de chocs répétés chez les patients présentant une TF n’ont toutefois pas été bien évaluées. Méthodologie: Nous avons examiné les cas consécutifs de 47 patients présentant une TF (âge moyen : 43,1 ± 13,2 ans; hommes : n = 34 [72,3 %]) ayant reçu un DCI en prévention secondaire entre 2000 et 2018. Résultats: Au total, 20 (42,6 %) patients ont subi un traitement invasif (ablation par cathéter par radiofréquence, n = 8; ablation chirurgicale et remplacement de la valve pulmonaire, n = 12) avant l’implantation d’un DCI. Vingt-sept patients (57,4 %) ont été pris en charge de façon non invasive. Au cours de la période de suivi (durée médiane de 80,5 [intervalle interquartile : 28,5 à 131,0] mois), 2 (10,0 %) patients du groupe ayant subi une intervention invasive et 10 (37,0 %) patients du groupe ayant subi une intervention non invasive ont reçu un choc approprié par DCI (p = 0,036). Les résultats de l’analyse par régression logistique montrent que le traitement invasif est associé à une réduction du risque de choc par DCI de 81,1 % (rapport des cotes : 0,189; intervalle de confiance à 95 % : de 0,036 à 0,990; p = 0,049). En outre, le traitement invasif est associé à une réduction du risque de survenue d’un des événements du paramètre d’évaluation composé, soit un choc administré par DCI, le décès, une transplantation cardiaque ou une hospitalisation (rapport des cotes : 0,090; intervalle de confiance à 95 % : de 0,025 à 0,365; p = 0,013) par rapport au traitement non invasif. Conclusions: La modification invasive du substrat a été associée à une probabilité plus faible de choc administré par DCI et à une amélioration des résultats à long terme chez les patients présentant une TF.

Background: Anomalous origin of a coronary artery from the pulmonary artery (PA) is a rare condition that usually presents in childhood. The aim of this study is to describe its characteristics in unrepaired adults. Methods: We conducted a retrospective study of 18 patients with left and right anomalous coronary artery from the PA (ALCAPA and ARCAPA) diagnosed in adulthood. Patients ≥18 years old at diagnosis and assessed at least once in our institution were included. Results: Median age at diagnosis was 29.5 years [IQR 24.0–48.8]. Presentation mode varied, with two patients (11%) diagnosed following aborted sudden cardiac death. Diagnosis was most frequently made by coronary angiography (44%) and cardiac computed tomography (28%). The mean left ventricle ejection fraction (LVEF) at presentation was 56 ±10% and moderate or severe mitral regurgitation was present in 5 patients. Surgical or interventional repair was performed in 14 patients (78%), 9 of whom (50%) underwent reimplantation. Median follow-up was 8.5 years [IQR 0.98–13.8]. One patient with severe pulmonary hypertension died during the follow-up. Most patients (79%) were in NYHA functional class 1 at last follow-up with a mean LVEF of 53±12%. Conclusions: Anomalous origin of a coronary artery from the PA can present in adulthood in various ways. Multimodality imaging allows the diagnosis and assessment of functional impact. Surgical correction of ALCAPA with establishment of a dual coronary pathway was the most frequent management strategy in our cohort. The majority of patients experienced favorable long-term outcomes.