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Doppler-related pressure recovery. The gradient is then overestimated. A diastolic tail in the descending aorta and diastolic forward flow in the abdominal aorta are findings of significant (re-)CoA. • CMR and CCT, including 3D reconstruction, are the preferred non-invasive techniques to evaluate the entire aorta in adolescents and adults. Both depict site, extent, and degree of the aortic narrowing, the aortic arch and head and neck vessels, the preand post-stenotic aorta, and collaterals. Both methods detect complications such as aneurysms, false aneurysms, restenosis, or residual stenosis.184 • Imaging of intracerebral vessels is indicated in the case of symptoms and/or clinical manifestations of aneurysms/rupture. • Cardiac catheterization with manometry (a peak-to-peak gradient >_20 mmHg) indicates a haemodynamically significant CoA in the absence of well-developed collaterals and is performed in the setting of interventional treatment. It should be noted that, in patients under general anaesthesia, invasive measurement of gradient may be underestimated.

4.6.4 Surgical/catheter interventional treatment Indications for intervention are summarized in the Recommendations for intervention in coarctation and re-coarctation of the aorta table and in Figure 6. In native CoA, as well as re-coarctation with appropriate anatomy, stenting has become the first-choice treatment in many ACHD centres.185 The use of covered stents is preferred because of lower short- and long-term complication rates.186 Biodegradable stents are in development but are mainly applied in children when the aorta is still expected to grow. Balloon angioplasty in adults is only indicated for re-dilatation of previously stented aortas. While paediatric surgical techniques include resection and end-toend anastomosis, resection and extended end-to-end anastomosis, prosthetic patch aortoplasty, subclavian flap aortoplasty, interposition of a (tube) graft, and bypass tube (jump) grafts, only the latter two are generally feasible in adults. Ascending-to-descending aorta conduits may be preferable in adults with difficult anatomy. Although the surgical risk in simple CoA may currently be <1%, it increases significantly beyond the age of 3040 years. Spinal cord injury has become extremely rare.187 As coarctation is not a localized disease of the aorta, associated lesions that may require structural interventions have to be considered: • Associated significant aortic valve stenosis or regurgitation (BAV). • Aneurysm of the ascending aorta with a diameter >50 mm or rapid progression of diameter. • Aneurysm and false aneurysms at the previous CoA site. • Symptomatic or large aneurysms of the circle of Willis. Treatment should be performed in centres with extensive experience in the treatment of CHD. 4.6.5 Follow-up recommendations Residua, sequelae, and complications are listed below:

• Arterial hypertension at rest or during exercise is common, even after successful treatment, and is an important risk factor for premature CAD, ventricular dysfunction, and rupture of aortic or cerebral aneurysms.188 • The geometry of the arch (gothic, crenel, normal) and smaller aortic size in the stented region may play a role in the development of hypertension. Right arm 24-h ambulatory blood pressure measurement better detects hypertension than a one-off measurement.189 The significance of isolated, exercise-induced hypertension is a matter of debate. • An increased pressure gradient (systolic >_20 mmHg) between upper and lower extremities indicates re-coarctation and warrants invasive assessment for confirmation and treatment. • Medical treatment of arterial hypertension should follow the 2018 ESC/ESH Guidelines.190 • Recurring or residual CoA may induce or aggravate systemic arterial hypertension and its complications. • Aneurysms of the ascending aorta or at the intervention site present a risk of rupture and death. Patch repairs (e.g. with Dacron) are at particular risk of repair-site aneurysms, while

Recommendations for intervention in coarctation and re-coarctation of the aorta

Recommendations Classa Levelb

Repair of coarctation or re-coarctation (surgically or catheter based) is indicated in hypertensive patientsc with an increased non-invasive gradient between upper and lower limbs confirmed with invasive measurement (peak-topeak >_20 mmHg) with preference for catheter treatment (stenting), when technically feasible. I C Catheter treatment (stenting) should be considered in hypertensive patientsc with >_50% narrowing relative to the aortic diameter at the diaphragm, even if the invasive peak-to-peak gradient is <20 mmHg, when technically feasible. IIa C Catheter treatment (stenting) should be considered in normotensive patientsc with an increased non-invasive gradient confirmed with invasive measurement (peak-to-peak >_20 mmHg), when technically feasible. IIa C Catheter treatment (stenting) may be considered in normotensive patientsc with >_50% narrowing relative to the aortic diameter at the diaphragm, even if the invasive peak-to-peak gradient is <20 mmHg, when technically feasible. IIb C

aClass of recommendation. bLevel of evidence. cRight arm ambulatory blood pressure monitoring should be considered for the diagnosis of hypertension. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026