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A reduced exercise capacity is a common finding in adult congenital heart disease and is associated with cardiovascular morbidity and mortality. However, data on exercise capacity in patients after repair of coarctation of the aorta (CoA) are scarce. Furthermore, a high rate of exercise-induced hypertension has been described in CoA patients. This study sought to assess exercise capacity and blood pressure response in asymptomatic patients long-term after CoA repair in relation to left ventricular and vascular function. Twenty-two CoA patients (age 30 ± 10.6 years) with successful surgical repair (n = 12) or balloon angioplasty (n = 10) between 3 months and 16 years of age with a follow-up of > 10 years underwent cardiopulmonary exercise testing at a mean follow-up of 23.9 years. Exercise capacity (peak oxygen uptake; VO2peak) and blood pressure response were compared to age- and gender-matched reference values. Left ventricular function and volumetric analysis was performed using cardiovascular magnetic resonance imaging. CoA patients showed preserved exercise capacity compared to the healthy reference group, with a VO2peak of 41.7 ± 12.0 ml/kg/min versus 44.9 ± 6.7 ml/kg/min. VO2peak/kg showed a significant association with age (p < 0.001) and male gender (p ≤ 0.001). Exercise-induced hypertension occurred in 82% of CoA patients, and was strongly related to left ventricular mass (p = 0.04). Of the 41% of patients who were normotensive at rest, 78% showed exercise-induced hypertension. No significant correlation was found between peak exercise blood pressure and age, BMI, age at time of repair, LVEF, or LV dimensions. Exercise capacity is well preserved in patients long-term after successful repair of coarctation of the aorta. Nevertheless, a high number of patients develop exercise hypertension, which is strongly related to systemic hypertension. Regular follow-up, including cardiopulmonary exercise testing, and aggressive treatment of hypertension after CoA repair is strongly advised. [ABSTRACT FROM AUTHOR]

Objectives: To provide a comprehensive overview of all reported cardiac magnetic resonance (CMR) findings that predict clinical deterioration in pulmonary arterial hypertension (PAH).Methods: MEDLINE and EMBASE electronic databases were systematically searched for longitudinal studies published by April 2015 that reported associations between CMR findings and adverse clinical outcome in PAH. Studies were appraised using previously developed criteria for prognostic studies. Meta-analysis using random effect models was performed for CMR findings investigated by three or more studies.Results: Eight papers (539 patients) investigating 21 different CMR findings were included. Meta-analysis showed that right ventricular (RV) ejection fraction was the strongest predictor of mortality in PAH (pooled HR 1.23 [95 % CI 1.07-1.41], p = 0.003) per 5 % decrease. In addition, RV end-diastolic volume index (pooled HR 1.06 [95 % CI 1.00-1.12], p = 0.049), RV end-systolic volume index (pooled HR 1.05 [95 % CI 1.01-1.09], p = 0.013) and left ventricular end-diastolic volume index (pooled HR 1.16 [95 % CI 1.00-1.34], p = 0.045) were of prognostic importance. RV and LV mass did not provide prognostic information (p = 0.852 and p = 0.983, respectively).Conclusion: This meta-analysis substantiates the clinical yield of specific CMR findings in the prognostication of PAH patients. Decreased RV ejection is the strongest and most well established predictor of mortality.Key Points: • Cardiac magnetic resonance imaging is useful for prognostication in pulmonary arterial hypertension. • Right ventricular ejection fraction is the strongest predictor of mortality. • Serial CMR evaluation seems to be of additional prognostic importance. • Accurate prognostication can aid in adequate and timely intensification of PAH-specific therapy. [ABSTRACT FROM AUTHOR]