Coarctation of the Aorta Guidelines

Updated: Nov 20, 2018
  • Author: Syamasundar Rao Patnana, MD; Chief Editor: Stuart Berger, MD  more...
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Guidelines

Guidelines Summary

2008 American College of Cardiology/American Heart Association (ACC/AHA) guidelines on coarctation of aorta in adults (adapted)

Recommendations for clinical evaluation and follow-up  [185]

Class I recommendations are as follows:

  • Every patient with systemic arterial hypertension should undergo simultaneous palpation of the brachial and femoral pulses to assess timing and amplitude evaluation to search for the brachial-femoral delay of significant aortic coarctation. Search for differential pressure by measuring the supine bilateral arm (brachial artery) blood pressures and prone right or left supine (popliteal artery) blood pressures (level of evidence: C)

  • For suspected aortic coarctation, useful initial imaging and hemodynamic evaluation is by transthoracic echocardiogram, including suprasternal notch acoustic windows (level of evidence: B)

  • All patients with coarctation (repaired or not) should have at least one cardiovascular magnetic resonance imaging (MRI) or computed tomography (CT) scan for complete evaluation of the thoracic aorta and intracranial vessels (level of evidence: B)

Medical therapy management strategies

Management of hypertension included first-line pharmacotherapy with beta blockers, angiotensin-converting enzyme inhibitors, or angiotensin-receptor blockers. Selection of beta blockers or vasodilators are based in part on the aortic root size, the presence of aortic regurgitation, or both.

Recommendations for interventional and surgical treatment

Class I recommendations are as follows:

  • Intervention for coarctation is recommended in the following circumstances: (1) Peak-to-peak coarctation gradient of at least 20 mm Hg (level of evidence: C) and (2) peak-to-peak coarctation gradient below 20 mm Hg in the presence of anatomic imaging evidence of significant coarctation with radiologic evidence of significant collateral flow (both level of evidence: C)

  • Selection of percutaneous catheter intervention versus surgical repair of native discrete coarctation should be determined by multi-team consultation with adult congenital heart disease cardiologists, interventionalists, and surgeons at the adult congenital heart disease center (ACHD) (level of evidence: C)

  • Percutaneous catheter intervention is indicated for recurrent, discrete coarctation and a peak-to-peak gradient of at least 20 mm Hg (level of evidence: B)

  • Experienced surgeons in congenital heart disease should perform operations for previously repaired coarctation as well as (1) long recoarctation segment and (2) concomitant hypoplasia of the aortic arch (both level of evidence: B)

Class IIb recommendation

Although stent placement for long-segment coarctation may be considered, its usefulness is not well established and the long-term efficacy and safety are unknown (level of evidence: C).

Recommendations for key issues for evaluation and follow-up

Class I recommendations are as follows:

  • Lifelong cardiology follow-up for all patients with aortic coarctation (repaired or not), including an evaluation by or consultation with a cardiologist with expertise in ACHD (level of evidence: C)

  • At least yearly follow-up for patients who have had surgical repair of coarctation at the aorta or percutaneous intervention for aortic coarctation (level of evidence: C)

  • Late postoperative thoracic aortic imaging to assess for aortic dilatation or aneurysm formation, even if the coarctation repair appears to be satisfactory (level of evidence: B)

  • Close observation for the appearance or reappearance of resting or exercise-induced systemic arterial hypertension, which should be treated aggressively after excluding recoarctation (level of evidence: B)

  • 5-Year or shorter interval evaluation of the coarctation repair site by MRI/CT scan, depending on the specific anatomic findings before and after repair (level of evidence: C)

Class IIb recommendation 

Routine exercise testing may be performed at intervals determined by consultation with the regional ACHD center (level of evidence: C).

For the full guidelines, see ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease. [185]

2014 European Society of Cardiology (ESC) guidelines

The ESC guidelines largely agree with the ACC/AHA recommendations (all level of evidence: C). [186]

Their class I recommendation is for intervention in all patients with a noninvasive pressure difference above 20 mm Hg between the upper and lower limbs, regardless of symptoms but with upper limb hypertension (>140/90 mm Hg in adults), abnomal exercise blood pressure response, or significant left ventricular hypertrophy.

Their class IIa recommendation indicates intervention should be considered in hypertensive patients with more than 50% aortic narrowing relative to the aortic diameter at the level of the diaphragm (as seen on MRI, CT scan, or invasive angiography), regardless of the pressure gradient.

Their class IIb recommendation  indicates intervention may be considered in patients with more than 50% aortic narrowing relative to the aortic diameter at the level of the diaphragm (as seen on MRI, CT scan, or invasive angiography), regardless of the pressure gradient and the presence of hypertension.