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............................................................................. interposition grafts are at particular risk of false aneurysms,191

and both should be imaged on a regular basis. • Attention is required for BAV, mitral valve disease, premature CAD, and berry aneurysms of the circle of Willis (routine screening in asymptomatic patients is not recommended). All CoA patients require regular follow-up at least every year. Imaging of the aorta (preferably with CMR) is required to document post-repair or post-interventional anatomy and complications (restenosis, aneurysm, false aneurysm formation). Recommended imaging intervals are commonly every 35 years but also depend on baseline pathology.

4.6.6 Additional considerations

• Exercise/sports: patients without residual obstruction, who are normotensive at rest and with exercise, can usually lead normally active lives without restriction. Patients with arterial hypertension, residual obstruction, or other complications should avoid heavy isometric exercises in proportion to the severity of their problems. • Pregnancy: after successful treatment of CoA, many women tolerate pregnancy without major problems.43 In particular, women with unrepaired CoA  but also those after repair with arterial hypertension, residual CoA, or aortic aneurysms  have an increased risk of aortic rupture and rupture of a cerebral aneurysm during pregnancy and delivery. An excess of miscarriages and hypertensive disorders has been reported192 (see section 3.5.7). • IE prophylaxis: recommended only for high-risk patients (see section 3.4.6).

4.7 Aortopathies

4.7.1 Marfan syndrome and related heritable thoracic aortic diseases 4.7.1.1 Introduction and background Marfan syndrome is the prototype of syndromic HTAD entities, comprising a clinically and genetically heterogeneous group of disorders with aneurysm or dissection of the thoracic aorta as the common denominator. Both syndromic and non-syndromic (or isolated) forms of HTAD are part of the clinical spectrum, with notable clinical overlap between the various entities. For more details on the various syndromes, please refer to the 2014 ESC Guidelines on aortic disease193 and a consensus document on genetic testing in ACHD/HTAD.140 Since most children with mainly syndromic forms of HTAD will be transferred to ACHD units at adult age, these Guidelines focus on specific cardiovascular complications. Marfan syndrome is considered as the model disease  other syndromes are mentioned in case of important differences from Marfan syndrome.

©ESC 2020

Figure 6 Management of coarctation and re-coarctation of the aorta. CoA = coarctation of the aorta. aRight arm ambulatory blood pressure should be considered for diagnosis. bInvasively confirmed measurement. cRelative to the aortic diameter at the diaphragm. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026