Prevalence and risk of progressive aortic aneurysm and dissection in adults with conotruncal anomalies.

AIMS: Conotruncal anomalies share common embryogenic defects of the outflow tracts and great arteries, which result in a predisposition to aortic aneurysms. The purpose of this study was to describe the prevalence and risk of progressive aortic aneurysms in adults with conotruncal anomalies. METHODS AND RESULTS: Retrospective study of adults with conotruncal anomalies that underwent cross-sectional imaging 2003-20. Aneurysm was defined as aortic root/mid-ascending aorta >2.1 mm/m2/>1.9 mm/m2, progressive aneurysm as increase by >2 mm, and severe aneurysm as dimension >50 mm. Of 2261 patients (38 ± 12 years; male 58%), 1167 (52%) had an aortic aneurysm, and 205 (14%) had a severe aortic aneurysm. Mean annual increase in aortic root/mid-ascending aorta was 0.3 ± 0.1 mm/0.2 ± 0.1 mm. The 3-, 5-, and 7-year cumulative incidence of the progressive aortic aneurysm was 4%, 7%, and 9%, respectively. The rate of aneurysm growth decreased with age, with no significant growth after age 40 years. There was an excellent correlation between aortic indices from cross-sectional imaging and echocardiography. Of 950 females, 184 had ≥1 pregnancy, and 81 (44%) of the 184 patients had aortic aneurysm prior to pregnancy. There was no aortic dissection or progression of the aortic aneurysm during pregnancy. Overall, there was no aortic dissection during 7984 patient-years of follow-up. CONCLUSIONS: Aortic aneurysm was common in patients with conotruncal anomalies. However, the risk of progressive aneurysm or dissection was low. Collectively, these data suggest a benign natural history and perhaps a less frequent need for cross-sectional imaging. Further studies are required to determine the optimal timing for surgical intervention in this population.

Effect of Combined Ventricular-Arterial Stiffening on Exercise Hemodynamics in Adults With Repaired Coarctation of Aorta.

BACKGROUND: Patients with coarctation of aorta (COA) have arterial stiffening, and this is associated with impaired cardiac reserve and hypertensive systolic blood pressure (SBP) response during exercise. However, whether patients with COA have concomitant left ventricular (LV) stiffening and the potential impact of combined ventricular-arterial stiffening on exercise hemodynamics are unknown. METHODS: We studied 174 patients with repaired COA (aged 39 ± 11 years and male 103 [59%]) and 174 matched controls. Our study hypotheses are: (1) patients with COA have higher ventricular-arterial stiffness (end-systolic elastance [Ees] and arterial elastance [Ea]) as compared with controls; (2) ventricular-arterial stiffness was associated with LV stroke volume augmentation (ΔLVSV) and SBP augmentation (ΔSBP) during exercise among patients with COA. RESULTS: Despite similar systolic SBP, patients with COA had higher Ea (1.8 ± 0.4 vs 1.4 ± 0.4 mm Hg/mL, P < 0.001), higher Ees (2.41 ± 0.65 vs 2.17 ± 0.40 mm Hg/mL, P < 0.001), but similar Ea/Ees (0.87 ± 0.29 vs 0.83 ± 0.33, P = 0.2). ΔLVSV was 6.1 ± 1.4 mL/beat. Combined ventricular-arterial stiffness had a stronger correlation with ΔLVSV as compared with Ea alone (r = -0.53 vs r = -0.41, P = 0.006) and as compared with Ees alone (r = -0.53 vs r = -0.46, P = 0.02). ΔSBP was 48 ± 21 mm Hg. Combined ventricular-arterial stiffness had a stronger correlation with ΔSBP as compared with Ea alone (r = 0.57 vs r = 0.43, P < 0.001) and as compared with Ees alone (r = 0.57 vs r = -0.39, P < 0.001). CONCLUSION: Patients with COA had combined ventricular-arterial stiffening, and this was associated with impaired cardiac reserve and hypertensive SBP response during exercise. These findings provide foundation for further studies to determine whether drugs that reduce both ventricular and arterial stiffness will improve exercise capacity and hemodynamics in this unique population.

CONTEXTE: La coarctation de l'aorte (CA) s'accompagne d'une rigidification des artères, qui est à son tour associée à une altération de la réserve de force du cœur et à une augmentation de la pression artérielle systolique (PAS) à l'effort. On ne sait toutefois pas si la CA entraîne aussi une rigidification du ventricule gauche, et on ne connaît pas les répercussions possibles d'une rigidification ventriculaire et artérielle sur les paramètres hémodynamiques à l'effort. MÉTHODOLOGIE: Nous avons étudié 174 patients présentant une CA corrigée (âge : 39 ± 11 ans; hommes : 103 [59 %]) et 174 témoins appariés. Nos hypothèses étaient les suivantes : 1) la rigidité ventriculaire et artérielle (élastance télésystolique [ETS] et élastance artérielle [EA]) est plus importante chez les sujets présentant une CA que chez les sujets témoins; 2) la rigidité ventriculaire et artérielle est associée à une augmentation du volume d'éjection ventriculaire gauche (ΔVEVG) et à une augmentation de la pression artérielle systolique (ΔPAS) à l'effort chez les patients présentant une CA. RÉSULTATS: Malgré une PAS comparable, les patients présentant une CA avaient une EA plus élevée que les sujets témoins (1,8 ± 0,4 vs 1,4 ± 0,4 mmHg/ml, p < 0,001) et une ETS également plus élevée (2,41 ± 0,65 vs 2,17 ± 0,40 mmHg/ml, p < 0,001), mais un rapport EA/ETS similaire (0,87 ± 0,29 vs 0,83 ± 0,33, p = 0,2). La ΔVEVG était de 6,1 ± 1,4 ml/battement. La rigidité ventriculaire et artérielle mixte était plus fortement corrélée avec une ΔVEVG, comparativement à l'EA seule (r = -0,53 vs r = -0,41, p = 0,006) et à l'ETS seule (r = -0,53 vs r = -0,46, p = 0,02). La ΔPAS était de 48 ± 21 mmHg. La rigidité ventriculaire et artérielle mixte était plus fortement corrélée avec la ΔPAS, comparativement à l'EA seule (r = 0,57 vs r = 0,43, p < 0,001) et à l'ETS seule (r = 0,57 vs r = -0,39, p < 0,001). CONCLUSION: La CA s'accompagnait d'une rigidification ventriculaire et artérielle, elle-même associée à une altération de la réserve de force du cœur et à une augmentation de la PAS à l'effort. Ces résultats pourront servir de fondements à des études complémentaires visant à déterminer si un traitement qui réduit la rigidité ventriculaire et artérielle pourrait améliorer la capacité à l'effort et les paramètres hémodynamiques dans cette population particulière.

Recurrent sustained atrial arrhythmias and thromboembolism in Fontan patients with total cavopulmonary connection.

BACKGROUND: Total cavopulmonary connection (TCPC) is associated with a lower risk of incident atrial arrhythmias as compared to atriopulmonary Fontan, but the risk of recurrent atrial arrhythmias is unknown in this population. The purpose of this study was to determine the incidence and risk factors for recurrent atrial arrhythmias and thromboembolic complications in patients with TCPC. METHODS: This is a retrospective multicenter study conducted by the Alliance for Adult Research in Congenital Cardiology (AARCC), 2000-2018. The inclusion criteria were TCPC patients (age > 15 years) with prior history of atrial arrhythmia. RESULTS: A total of 103 patients (age 26 ± 7 years; male 58 [56%]) met inclusion criteria. The mean age at initial arrhythmia diagnosis was 13 ± 5 years, and atrial arrhythmias were classified as atrial flutter/tachycardia in 85 (83%) and atrial fibrillation in 18 (17%). The median duration of follow-up from the first episode of atrial arrhythmia was 14.9 (12.1-17.3) years, and during this period 64 (62%) patients had recurrent atrial arrhythmias (atrial flutter/tachycardia 51 [80%] and atrial fibrillation 13 [20%]) with annual incidence of 4.4%. Older age was a risk factor for arrhythmia recurrence while the use of a class III anti-arrhythmic drug was associated with a lower risk of recurrent arrhythmias. The incidence of thromboembolic complication was 0.6% per year, and the cumulative incidence was 4% and 7% at 5 and 10 years respectively from the time of first atrial arrhythmia diagnosis. There were no identifiable risk factors for thromboembolic complications in this cohort. CONCLUSIONS: Although TCPC provides superior flow dynamics and lower risk of incident atrial arrhythmias, there is a significant risk of recurrent arrhythmias among TCPC patients with a prior history of atrial arrhythmias. These patients may require more intensive arrhythmia surveillance as compared to other TCPC patients.