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.
We aimed to provide a comprehensive assessment of exercise physiology of adult Fontan patients (FP) and factors limiting functional capacity (FC). A prospective single-centre study of 37 FP aged ≥16years and 19 healthy-controls (HC), who underwent CPET on cycle-ergometer in February-March 2022. Lung function was impaired in FP, mostly mild restrictive pattern (56.8%). Mean peakVO2 was 21±5.4ml/kg/min, 55% predicted. Morphologically left-single ventricle showed higher peakVO2%predicted (57.4±14.4% vs 43.4±8.1%, p=0.045). A lower peakVO2%predicted was observed in patients with an early flattened and/or descending O2 pulse at maximal exertion (52±14% vs 62±12.5, p=0.04 and 47.6±9% vs 60±14 respectively, p=0.018) and chronotropic insufficiency (53±12% vs 69.8±20%, p=0.008). Strong positive correlation was observed between %OUES and peakVO2%predicted (r=0.726, p>0.001). PeakVO2%predicted was progressively higher as the level of physical activity increased (low-level 49.5±14%, moderate-level 55±12%, intense-level 69±20%). FP showed lower inspiratory muscle strength compared to HC but it was no statistically associated with either peakVO2 or VE/VCO2 slope. In FP OUES is a useful submaximal parameter in those who fail to reach maximal exertion. Chronotropic insufficiency and early flattened or descending oxygen pulse slope were factors associated with low peakVO2. Regular intense physical activity improves FC. Although FP have inspiratory muscle weakness, its impact on FC is unclear.
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