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......................................................................................................................................................................... up is also necessary to detect and observe late restenosis, progressive AR, and complications such as arrhythmias, heart block, and iatrogenic VSD. Follow-up should include evaluation in specialized ACHD centres, and the frequency is related to the expected disease progression.

4.5.3.6 Additional considerations

• Exercise/sports: see valvular AS (section 4.5.1). • Pregnancy: only contraindicated in severe, symptomatic SubAS where surgery should be performed before pregnancy (even in asymptomatic severe SubAS, surgery should be considered) (see section 3.5.7). • IE prophylaxis: recommended only for high-risk patients (see section 3.4.6).

4.6 Coarctation of the aorta

4.6.1 Introduction and background CoA is considered as part of a generalized arteriopathy, and not only as narrowing of the aorta. It occurs as a discrete stenosis or as a long, hypoplastic aortic (arch) segment. Typically, CoA is located in the area where the ductus arteriosus inserts, and only in rare cases occurs ectopically (ascending, descending, or abdominal aorta).

Associated lesions include BAV (up to 85%), ascending aortic aneurysm, SubAS, or SupraAS, (supra)mitral valve stenosis (including parachute mitral valve), Shone complex, or complex congenital heart defects. CoA can be associated with Turner syndrome and WilliamsBeuren syndrome. Extracardiac vascular anomalies have been reported in CoA patients including anomalous origin of the right subclavian artery (in 45% of cases), collateral arterial circulation, and intracerebral aneurysms (in up to 10%).

4.6.2 Clinical presentation and natural history Signs and symptoms depend on the severity of CoA. Patients with severe CoA usually present with signs and symptoms early in life, while particularly mild cases may not become evident until adulthood, where CoA is detected in the work-up of arterial hypertension. Key symptoms may include headache, nosebleeds, dizziness, tinnitus, shortness of breath, abdominal angina, claudication, and cold feet. Patients with CoA who reach adolescence demonstrate very good long-term survival up to age 60 years. Long-term morbidity is common, however, largely related to aortic complications and longstanding hypertension.183 The natural course may be complicated by left heart failure, intracranial haemorrhage (from berry aneurysm), IE, aortic rupture/dissection, premature coronary and cerebral artery disease, and associated heart defects.

4.6.3 Diagnostic work-up See section 3.3 for general principles. Office blood pressure measurement in the upper and lower extremities are the primary studies required in all coarctation patients. A blood pressure gradient between upper and lower extremities (systolic >_20 mmHg) indicates significant CoA. Weak or absent pulses in the lower extremities or radiofemoral pulse delay also indicate significant coarctation. • Ambulatory blood pressure measurements (right arm) are recommended to detect/confirm arterial hypertension (24-h mean systolic >130 mmHg and/or diastolic >80 mmHg). • Other findings consist of a suprasternal thrill (proximal obstruction), an interscapular (systolic) murmur, or continuous murmurs (due to collateral vessels). In the case of a pinpoint CoA, murmurs may be completely absent. • Chest X-ray findings may be characterized by rib notching of the third and fourth (to the eighth) ribs due to the collaterals. • Echocardiography provides information regarding site, structure, and extent of CoA, LV function and LVH, associated cardiac abnormalities, and aortic and supra-aortic vessel diameters. Doppler gradients are not useful for quantification, neither in native nor in post-operative coarctation. In the presence of extensive collateral arteries, gradients are not reliable and are often underestimated. After surgical repair or stenting, increased systolic flow rates may develop, even in the absence of significant narrowing, due to decreased/absent aortic compliance and

Recommendations for intervention in subaortic stenosis

Recommendations Classa Levelb

In symptomatic patients (spontaneous or on exercise test) with a mean Doppler gradient >_40 mmHgc or severe AR, surgery is recommended. I C Asymptomatic patients should be considered for surgery when one or more of the following

findings are present: • Mean gradient <40 mmHg but LVEF <50%. • AR is severe and LVESD >50 mm

(or 25 mm/m2 BSA) and/or EF <50%d. • Mean Doppler gradient is >_40 mmHgc and

marked LVH present. • Mean Doppler gradient is >_40 mmHgc and there is a fall in blood pressure below baseline

on exercise. IIa C Asymptomatic patients may be considered for

surgery when one or more of the following findings are present: • Mean Doppler gradient is >_40 mmHg,c LV is

normal (EF >50% and no LVH), exercise testing is normal, and surgical risk is low. • Progression of AR is documented and AR

becomes more than mild (to prevent further progression). IIb C

AR = aortic regurgitation; BSA = body surface area; EF = ejection fraction; ESC = European Society of Cardiology; LV = left ventricle/ventricular; LVEF = left ventricular ejection fraction; LVESD = left ventricular end systolic diameter; LVH = left ventricular hypertrophy. aClass of recommendation. bLevel of evidence. cDoppler-derived gradients may overestimate the obstruction and may need confirmation by cardiac catheterization. dSee 2017 ESC Guidelines on the management of valvular heart disease.25 Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026