Técnicas de imagen en la coartación de aorta

Coarctation of aorta is a congenital malformation relatively frequent in the population. The clinical presentation varies widely, from symptoms at birth to incidental diagnosis in adulthood. Imaging techniques allow us to assess the anatomy of the stenosis, hemodynamics, left ventricle function and hypertrophy and the presence of associated complications. Transthoracic echocardiography is the first line technique but computed tomography and cardiac magnetic resonance are the tests of choice for surgical evaluation of the native coarctation and to followup the repaired coarctation.La coartación aórtica es una malformación congénita relativamente frecuente. Sus manifestaciones abarcan un amplio espectro de presentación, pudiendo mostrar síntomas desde el nacimiento, o bien diagnosticarse en la edad adulta. Para su manejo se dispone de una amplia gama de técnicas de imagen que permiten valorar la anatomía de la estenosis, las repercusiones estructurales en el ventrículo izquierdo, las alteraciones hemodinámicas y las complicaciones asociadas. La ecocardiografía transtorácica es la técnica de primera línea. Para la valoración quirúrgica en la coartación nativa y el seguimiento de la coartación reparada, la tomografía computarizada y la resonancia magnética son las técnicas de elección

Time course and predictors for neoaortic root dilatation and neoaortic valve regurgitation during adult life after arterial switch operation

[ES] Introducción y objetivos: Hay pocos datos sobre la evolución en adultos de la dilatación de la raíz neoaórtica (RAO) y la insuficiencia valvular neoaórtica (IA) tras la cirugía de switch arterial (SA) en la transposición de grandes arterias. Métodos: Análisis retrospectivo de 152 pacientes con transposición de grandes arterias, mayores de 15 años, intervenidos mediante SA y seguidos durante 4,9 ± 3,3 años en 2 centros de referencia. Se analizaron los cambios de diámetro de la RAO ajustados a superficie corporal y la progresión a grado moderado/grave de la IA con ecocardiografías seriadas. Se realizó un modelo de regresión de Cox para identificar factores predictores de progresión de la IA. Resultados: Inicialmente, 4 pacientes (2,6%) presentaban IA grave (3 habían precisado cirugía valvular) y 9 (5,9%) moderada. La RAO basal media era 20,05 ± 2,4 mm/m2, y al final del seguimiento, 20,73 ± 2,8 mm/m2 (p < 0,001), con un crecimiento medio de 0,14 (IC95%, 0,07-0,2) mm/m2/año. La IA progresó en 20 (13,5%) y 6 (4%) fueron intervenidos. La progresión de IA se asoció con válvula bicúspide, IA inicial, dilatación de la RAO inicial y crecimiento de la RAO. La válvula bicúspide (HR = 3,3; IC95%, 1,1-15,2; p = 0,037), la IA inicial (HR = 5,9; IC95%, 1,6-59,2; p = 0,006) y el crecimiento de la RAO (HR = 4,1; IC95%, 2-13,5; p = 0,023) resultaron predictores independientes. Conclusiones: La dilatación de la RAO y la IA progresan en el adulto joven intervenido mediante SA. La válvula bicúspide, la IA basal y el crecimiento de la RAO son predictores de progresión de IA.[EN] Introduction and objectives: There are limited data on the long-term development of neoaortic root dilatation (NRD) and neoaortic valve regurgitation (AR) after arterial switch operation (ASO) for transposition of the great arteries during adult life. Methods: We performed a retrospective longitudinal analysis of 152 patients older than 15 years who underwent ASO for transposition of the great arteries and who were followed-up for 4.9 ± 3.3 years in 2 referral centers. Sequential changes in body surface-adjusted aortic root dimensions and progression to moderate/severe AR were determined in patients with 2 or more echocardiographic examinations. Risk factors for dilatation were tested by Cox regression to identify predictors of AR progression. Results: At baseline, moderate AR was present in 9 patients (5.9%) and severe AR in 4 (2.6%), of whom 3 had required aortic valve surgery. Initially, the median neoaortic root dimension was 20.05 ± 2.4 mm/m2, which increased significantly to 20.73 ± 2.8 mm/m2 (P < .001) at the end of follow-up. The mean change over time was 0.14 mm/m2/y (95%CI, 0.07-0.2). Progressive AR was observed in 20 patients (13.5%) and 6 patients (4%) required aortic valve surgery. Progressive AR was associated with bicuspid valve, AR at baseline, NRD at baseline, and neoaortic root enlargement. Independent predictors were bicuspid valve (HR, 3.3; 95%CI, 1.1-15.2; P = .037), AR at baseline (HR, 5.9; 95%CI, 1.6-59.2; P = .006) and increase in NRD (HR, 4.1 95%CI, 2-13.5; P = .023). Conclusions: In adult life, NRD and AR progress over time after ASO. Predictors of progressive AR are bicuspid valve, AR at baseline, and increase in NRD

Respiratory Muscle Function and Exercise Performance in Adult Patients with Fontan Circulation

© 2023 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).At rest, a good Fontan circulation can provide a normal cardiac output (CO). However, as a consequence of its unique hemodynamic nature, the limitations of the Fontan circuit are exposed during exercise. We aimed to provide a comprehensive assessment of the pathophysiology of exercise in adult Fontan patients (FPs) and identify factors limiting their functional capacity (FC). In a single-center study conducted in 37 FPs aged ≥16 years and 19 healthy-controls (HCs) who underwent CPET on a cycle ergometer in February and March 2022, the mean peakVO2 was 21 ± 5.4 mL/kg/min, which was 55% of the predicted value. Morphologically, the left single ventricle showed a higher peakVO2% predicted value (57.4 ± 14.4% vs. 43.4 ± 8.1%, p = 0.045). The factors associated with low peakVO2 values were an early flattened or descending O2 pulse at maximal exertion (52 ± 14% vs. 62 ± 12.5, p = 0.04 and 47.6 ± 9% vs. 60 ± 14, p = 0.018, respectively) and chronotropic insufficiency (53 ± 12% vs. 69.8 ± 20%, p = 0.008). The OUES was found to be a useful parameter to assess the FC in FPs in maximal and submaximal exercise testing. A strong positive correlation was observed between the %OUES and peakVO2%predicted (r = 0.726, p > 0.001). The lung function was impaired in the FPs, mostly with a mild restrictive pattern (56.8%). The FPs showed lower inspiratory muscle strength compared to the HCs but it was not statistically associated with either the peakVO2 or VE/VCO2 slope. Regular intense physical activity improves one’s FC. Although FPs have inspiratory muscle weakness, its impact on their FC is unclear. The peakVO2% predicted grew progressively higher as the level of physical activity increased (low level 49.5 ± 14%, moderate level 55 ± 12%, intense level 69 ± 20%).Peer reviewe

Pulmonary hypertension in adults with congenital heart disease. Clinical phenotypes and outcomes in the advanced pulmonary vasodilator era

[Background] Mortality of pulmonary hypertension associated with congenital heart disease (PAH-CHD) in adults remains high.[Objectives] To identify predictors of death and to assess the impact of treatment on outcome.[Methods] Retrospective, multicenter cohort study of 103 adults with PAH-CHD followed-up for 8.6 ± 4.6 years. Patients were grouped according to underlying shunt type into pre-tricuspid, post-tricuspid and complex. Survival rates were analyzed and predictors of death were investigated with Cox regression models.[Results] In the post-tricuspid and complex groups (38 and 37 patients, respectively), the most common clinical PAH-CHD subgroup was Eisenmenger syndrome (76.3% and 59.5%, respectively) whereas, in the pre-tricuspid group (28 patients), 46.5% of patients had small or corrected defects. Overall, 88 patients received vasodilators; 39% required combination-therapy. Overall survival at 10 years was 65%. Mortality was highest in the pre-tricuspid group, FC-III-IV and amongst patients receiving monotherapy (p < 0.050). On multivariate analysis, predictors of poor outcome were pericardial effusion (HR: 4,520 [1,470–13,890]; p = 0,008), oxygen saturation(HR: 0.940 [0,900 - 0,990]; p = 0,018) and genetic syndromes(HR: 3,280 [1,098–9,780]; p = 0,033).[Conclusions] Patients in advanced stages at initiation of treatment were at high risk of death and strong consideration should be given for more aggressive therapy.This study was supported by Clinical Research Grant 2017 from the Spanish Society of Cardiology. The regional Registry of Adult Congenital Heart Disease in Andalusia (RACCA) is supported by the Spanish Society of Cardiology and was launched through a research grant from Actelion Pharmaceuticals.Peer reviewe