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Problems after repair of aortic coarctation

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Some years after repair of coarctation of the aorta, up to a third of patients are hypertensive and a similar proportion has developed recoarctation. Coarctation is often associated with a bicuspid aortic valve the clinical significance of which is uncertain. A study in Rotterdam (

) has illustrated the long term problems.

Of 149 patients followed up in a clinic for adults with congenital heart disease, 124 had adequate echocardiographic data. They had had coarctation repair at a median age of 9 years (4–16 years) and were followed up for 18 years (13–25 years). Median age at last follow up was 28 years (20–36 years). Forty six of the 124 patients are reported to have had associated heart abnormalities recorded at operation (ventricular septal defect (n = 18), persistent ductus arteriosus (n = 16), atrial septal defect (n = 4), mitral valve abnormality (n = 4), transposition of the great arteries (n = 3), dextrocardia (n = 1)). Operative techniques included resection and end to end anastamosis (n = 91), subclavian flap (n = 14), and graftplasty (n = 10). (Surgical technique was not recorded in detail for nine patients.)

There were three deaths on follow up in the adults’ clinic, all between 14 and 20 years after repair. One was from acute aortic dissection, one during aortic arch surgery, and one from aortic valve endocarditis and massive acute aortic regurgitation. A bicuspid aortic valve was identified at echocardiography in 48 patients; 30 patients had a trileaflet valve, and the number of leaflets was uncertain for 46 patients. Seventy eight patients developed aortic valve disease (39 aortic stenosis, 36 aortic regurgitation, 3 both) and 27 had intervention for it (surgery for 25 and balloon dilatation for two). The proportion of patients with aortic valve disease was 63% overall and 70% in patients with a bicuspid valve. The ascending aorta was dilated in 26 of the 48 patients known to have a bicuspid valve and 9 of the remaining 76. Thirty had established aortic pathology (arch hyperplasia (n = 10), cervical aortic arch (n = 4), severe kinking of the aorta (n = 8), kinking plus cervical arch (n = 8)). Recurrence of coarctation occurred in 28 patients and was more frequent in patients who had their first repair under the age of 6 years. Recoarctation was treated surgically in 10 patients and with balloon dilatation in 18, at an average of 16 years after the initial repair. Thirty patients were taking antihypertensive treatment at their last follow up visit. These patients were older at last follow up and had their initial repair at an older age than patients not receiving antihypertensive treatment.

Late problems are common in patients who have had repair of aortic coarctation. All patients should be followed up for life.