Background: Repaired aortic coarctation(CoA) is associated with high long-term cardiovascular mortality and morbidity. Persisting hypertension and left ventricular dysfunction are possibly associated with residual or recurrent aortic arch obstruction (ReCoA) and abnormal activation of vasoactive hormones. Furthermore, knowledge regarding these patients' functional health status late after repair is missing.
Study subjects: A total of 133 adults who underwent surgical repair of CoA in childhood and youth (84 men) were examined in this observational cohort study. Median age (range) at surgery was 10 (0.1-40) years and 44 (26-74) years at examination. Thirty-six age and gender-matched healthy subjects served as controls.
Outcome measures: Prevalence of previous cardiovascular reintervention, current cardiac and valvular function, exercise capacity, blood pressure levels, as well as the presence of residual or recurrent aortic arch obstruction (ReCoA) and aortic aneurysms.
Methods: Echocardiography (including tissue Doppler), bicycle exercise testing, 24-h ambulatory blood pressure monitoring, MRI/ CT scan of the thoracic aorta were performed. Analysis of renal function and vasoactive hormones was performed by blood and urine tests at rest and after maximal physical effort. Functional health status was assessed by means of the SF-36 health survey.
Results: The prevalence of hypertension was high (44% of the cohort had blood pressure levels above the recommended levels, half of those despite medication). Reinterventions were common (26%) and most often performed due to aortic valve dysfunction and ReCoA. Above half of the cohort had a bicuspid aortic valve, which was strongly associated with ascending aorta aneurysms and aortic valve regurgitation. A total of 48% of the patients had a mild to moderate ReCoA, which was only weakly associated with the presence of hypertension as well as to exercise capacity and echocardiographic measurements of cardiac function. Both normotensive and hypertensive patients had increased left ventricular mass, normal ejection fraction, reduced long-axis systolic function, and impaired diastolic function compared with controls, with differences being more pronounced in hypertensive patients. Natriuretic hormone levels were slightly increased among normotensives, whereas renin-angiotensin-aldosterone and renal function parameters were normal at rest and during exercise. Mild to moderate ReCoA had no significant influence on the measured parameters. SF-36 scores among patients were only slightly lower compared with those from controls. However, patients with reduced exercise performance and those taking daily cardiovascular medication scored significantly lower in several mental and physical categories compared with patients with unmedicated patient and with those with preserved exercise capacity.
Conclusions: Surgical correction of CoA only repairs the anatomical narrowing, but not the associated vasculo- and valvulopathy. Increased left ventricular mass, systolic and diastolic dysfunction, aortic valve dysfunction, aortopathy, and hypertension are common. Morbidity is only weakly associated with mild and moderate degrees of ReCoA, and not associated with changes in vasoactive hormone levels and renal function. Despite late morbidity, functional health status is overall only slightly impaired in patients after surgical correction of CoA compared with healthy subjects. Nevertheless, the subgroup with reduced exercise capacity and need for cardiovascular medications have a considerable impairment of both physical and mental aspects of functional health.