Aortic Coarctation Treatment & Management

Updated: Feb 26, 2018
  • Author: Sandy N Shah, DO, MBA, FACC, FACP, FACOI; Chief Editor: Yasmine S Ali, MD, MSCI, FACC, FACP  more...
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Treatment

Medical Care

Severe coarctation of the aorta

Neonates with severe coarctation of the aorta should first have their condition stabilized:

  • First, support respiratory collapse with intubation.

  • Second, infuse prostaglandin E1 to open the ductus arteriosus.

  • Third, correct acidosis.

  • Finally, provide inotropic support to improve symptoms of congestive heart failure.

Less severe coarctation of the aorta

Patients presenting with less severe coarctation of the aorta beyond the neonatal period usually have chronically increased afterload and show signs of congestive heart failure. These patients should be treated with digoxin and diuretics.

Attempts should be made to postpone intervention, such as surgery or balloon dilatation, until the patient is hemodynamically stable.

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Surgical Care

Surgical repair has approximately doubled the 30-year survival of patients with aortic coarctation, with 72%-98% of these individuals reaching adulthood. [11]

No single technique is superior to others in minimizing the rate of restenosis in patients with aortic coarctation. In addition, there is no consensus regarding the optimal surgical technique in adolescents and adults with aortic coarctation. [3] The preferred method depends on anatomy of the lesion and institutional experience.

Three specific indications currently exist for intervention in patients with coarctation of the aorta, as follows:

  • Significant coarctation or recoarctation of the aorta with long-standing hypertension with or without symptoms
  • Hemodynamically significant aortic stenosis
  • Female patient contemplating pregnancy

Surgical procedures used in the treatment of coarctation of the aorta include the following [1] :

  • Subclavian artery patch aortoplasty
  • Bypass of the coarctation
  • Patch aortoplasty
  • Arch and descending aorta replacement or ascending aorta-to-descending aorta bypass
  • Tube graft replacement
  • Aneurysm replacement
  • Two-stage combined bicuspid valve surgery

In 1944, Blalock and Park performed the first experimental surgical repair of coarctation of the aorta in animals, which involved use of the left common carotid or subclavian artery to bypass the coarctation with end-to-end anastomosis. [13]

Resection of the coarctation site and end-to-end anastomosis to repair coarctation was performed first on humans in 1945 by Crafoord, Nylin, Gross, and Hufagel. [14] This is the preferred surgical method even in the present time. In this technique, the aorta is cross-clamped above and below the obstruction, and the discrete narrowing is resected. The advantages  of this procedure are that the obstructed site is completely resected, and it also avoids the use of prosthetic material and maintains a functioning left subclavian artery. The disadvantages of this procedure involve the sacrifice of spinal and intercostal vessels, resulting in paralysis. Also, a high rate of restenosis exists with use of continuous running suture or circumferential fibrosis. This problem is overcome by the use of interrupted and absorbable sutures, which allows for improved growth of the anastomotic site.

Patch aortoplasty was first performed by Vossschulte in 1961 to repair coarctation of the aorta. [15] This technique involves cutting across the obstruction and augmenting the area with a patch of prosthetic material. The advantages of this procedure include the ability to repair a long segment of coarctation; sparing of the left subclavian, intercostal, and spinal arteries; and preserving native aortic tissue to allow for growth. The disadvantage of this procedure is that it uses prosthetic material, which may gradually result in aneurysm formation.

Left subclavian flap angioplasty, introduced in 1966 by Waldhausen and Nahrwold, involves ligating the left subclavian artery and dividing it distally. [16] A longitudinal incision is made from the descending aorta to the coarctation superiorly into the origin of the left subclavian artery. The subclavian artery is turned down and used to enlarge the narrowing. To prevent subclavian steal phenomena, the vertebral artery is ligated. The advantages  to this procedure include preservation of native vascular tissue and avoidance of circumferential sutures, which allows for better growth of the involved area. The disadvantage to this procedure is the sacrifice of a major artery to the left arm, resulting in poor growth of that extremity.

Bypass graft repair bridges the ascending and descending aorta. The major disadvantage of this procedure is that the prosthetic material does not grow as the child grows, and it becomes calcified and narrow with time.

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Endovascular Care

Catheter-based intervention is now the preferred therapy for recurrent coarctation when the anatomy permits and necessary skills are available. Its use in native or unoperated coarctation is less well established. Treatment may be with balloon angioplasty alone or with a stent. [17] Outcomes are good in skilled hands, but residual or recurrent coarctation with resultant hypertension and repair site aneurysms can occur. Catheter-based treatment can cause death from aortic rupture and dissection, but mortality compares favorably with surgery if coarctation is recurrent, and perhaps for initial treatment.

In the Coarctation of the Aorta Stent Trial (COAST), investigators assessing the safety and efficacy of the Cheatham Platinum stent when used in 104 patients with native or recurrent coarctation, with follow-up to 2 years, found that the stent is safe and that its use is associated with persistent relief of aortic obstruction. [18] Reintervention was common; it was related to early and late aortic wall injury and to the need for re-expansion of small-diameter stents.

In another study, investigators analyzed their 21-year experience in the percutaneous treatment of complex coarctation of aorta at Reina Sofia University Hospital and found that initial results were maintained at later follow-up, and that the actuarial survival free probability of all complex patients at 15 years was 92%. [19] The study provided evidence that stent repair of complex coarctation of aorta is feasible and safe.

In an institutional study that assessed their 15-year experience of native aortic stenting in 24 patients aged 46 years and older, investigators found implantation of bare-metal and covered stents using femoral access were safe and effective. [20]

A more recent, retrospective study of outcomes of thoracic endovascular aortic repair (TEVAR) in 21 adults with primary coarctation of the aorta or late sequelae of previous open repair indicates this procedure is safe and effective in this population. [2]  In addition, the stent graft successfully dilated the annular constriction of the coarctation site. The 1-year freedom from intervention was 78%, and the 1- and 3-year survival was 95%. [2]

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Endovascular Versus Surgical Care

The immediate improvement in hypertension and morbidity appeared to be similar across all groups when endovascular repair was compared to surgical repair. [21] However, surgical therapy was associated with a low risk of restenosis and recurrence, whereas endovascular therapy had much higher incidence of restenosis and need for repeat interventions.

Endovascular therapy is highly promising in elderly and frail patients with multiple comorbidities who pose a high surgical risk. Overall, long-term outcome of endovascular approaches need to be evaluated.

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Activity

The 2015 American College of Cardiology/American Heart Association (ACC/AHA) scientific statement indicates that, before a patient's participation into a sports, a detailed evaluation should be perfomed, including physical examination, electrocardiography, chest x-ray, exercise testing, and cardiac/aortic imaging (transthoracic echocardiogram, magnetic resonance imaging [MRI], and/or computed tomography angiography [CTA]). [22] The time interval to repeat these tests is not clear, and it should be individualized to the specific patient.

Untreated aortic coarctation

Patients with an unrepaired coarctation can participate in all competitive sports if they meet all of the following criteria (class I) [22] :

  • Normal exercise test
  • Resting arm-leg systolic blood pressure gradient up to 20 mm Hg
  • Peak systolic blood pressure up to the 95th percentile of predicted with exercise
  • No significant ascending aortic dilatation (Z score ≤ 3.0)

Patients with arm-leg systolic blood pressure gradient above 20 mm Hg or exercise induced hypertension (peak systolic pressure >95th percentile of predicted with exercise) or with significant ascending aortic dilatation (Z-score >3.0) may participate only in only intensity class IA sports (billiards, bowling, cricket, curling, golf, and riflery) (class IIb).

Treated aortic coarctation (surgery or balloon and stent)

Patients who have undergone coarctation repair (surgery or trancatheter intervention) may participate in competitive sports that do not pose a danger of bodily collision (class IIb) and do not require high-intensity static exercise (classes IIIA, IIIB, and IIIC) after 3 months following the corrective procedure, if the criteria listed above are met, there is no aneurysm at the site of coarctation intervention, and there is no significant concomitant aortic valve disease. [22]

Patients with evidence of significant aortic dilation (Z score >3.0) or aneurysm formation (not yet of a size requiring surgical repair) may participate only in low-intensity (class IA and IB) sports (class IIb). 

Recreational sports

Patients with normal blood pressure, no residual coarctation, and a normal ascending aorta diameter can participate in all activities without restriction.

Patients with residual coarctation or a dilated ascending aorta are managed on a case-by-case basis. The degree of narrowing of the coarctation segment and dilatation of the ascending aorta dictates the level of sport activity.

Classification of sports

Levels of intensity of static exercise are classified as follows [23] :

  • I. Low: Less than 20% of the estimated maximal voluntary contraction (MVC)
  • II. Moderate: 20%-50% of MVC
  • III. High: Over 50% of MVC

Levels of intensity of dynamic exercise are classified as follows [23] :

  • A. Low: Less than 40% of the estimated maximal oxygen uptake (MaxO 2)
  • B. Moderate: 40%-70% of MaxO 2
  • C. High: Over 70% of MaxO 2

Examples of sports classifications in increasing level of static component with a low dynamic component [23]

  • IA sports (<20% MVC, <40% MaxO 2): Billiards, bowling, cricket, curling, golf, riflery
  • IIA sports (20%-50% MVC, <40% MaxO 2): Archery, auto racing, diving, equestrian, motorcycling
  • IIIA sports (>50% MVC, <40% MaxO 2): Bobsledding/luge, field events (throwing), gymnastics, martial arts, sailing, sport climbing, water skiing, weight lifting, windsurfing

Examples of sports classifications in increasing level of static component with a moderate dynamic component [23]

  • IB sports (<20% MVC, 40%-70% MaxO 2): Baseball/softball, fencing, table tennis, volleyball
  • IIB sports (20%-50% MVC, 40%-70% MaxO 2): US football, field events (jumping), figure skating, rodeoing, rugby, running (sprint), surfing, synchronized swimming
  • IIIB sports (>50% MVC, 40%-70% MaxO 2): Body building, downhill skiing, skateboarding, snowboarding, wrestling

Examples of sports classifications in increasing level of static component with a high dynamic component [23]

  • IC sports (<20% MVC, >70% MaxO 2): Badminton, cross-country skiing (classic technique), field hockey, orienteering, race walking, racquetball/squash, running (long distance), soccer, tennis
  • IIC sports (20%-50% MVC, >70% MaxO 2): Basketball, ice hockey, cross-country skiing (skating technique), lacrosse, running (middle distance), swimming, team handball
  • IIIC sports (>50% MVC, >70% MaxO 2): Boxing, canoeing/kayaking, cycling, decathlon, rowing, speed-skating, triathlon
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Long-Term Monitoring

The American College of Cardiology/American Heart Association recommends all patients with coarctation of the aorta (repaired or not) be monitored by a cardiologist. [24]

Patients should have undergone at least one magnetic resonance imaging (MRI) or angiographic study following repair of the coarctation.

Lifelong surveillance following surgical repair is mandatory. [25]  Close surveillance and aggressive management are necessary for residual hypertension, heart failure or intracardiac disease, associated bicuspid aortic valve, recurrent coarctation of the aorta, or significant arm-leg blood pressure gradient at rest or with exercise.

Ascending aortic dilation in the presence of a bicuspid aortic valve, new or unusual headache from berry aneurysm formation, late dissection proximal or distal to the repair site, and aneurysm formation at the site of coarctation repair are late sequelae of this disease.

Endocarditis prophylaxis

Endocarditis prophylaxis is indicated  for the following [10] ​:

  • Past history of endocarditis
  • Repair that involved insertion of a conduit
  • Six months after intervention if prosthetic material or a stent was used

Endocarditis prophylaxis is not indicated for the following:

  • Uncomplicated native coarctation
  • 6 months after successful repair of native or recoarctation
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