Aortic Coarctation 

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

Overview

Practice Essentials

Aortic coarctation is a narrowing of the aorta most commonly found just distal to the origin of the left subclavian artery. The vascular malformation responsible for coarctation is a defect in the vessel media, giving rise to a prominent posterior infolding (the “posterior shelf”), which may extend around the entire circumference of the aorta.

Signs and symptoms

Symptoms of aortic coarctation may include the following:

  • Early life: Congestive heart failure, severe acidosis, or poor perfusion to the lower body.

  • Beyond infancy: Usually none; however, hypertension, headache, nosebleed, leg cramps, muscle weakness, cold feet, or neurologic changes may be seen

The diagnosis of coarctation generally can be made on the basis of physical examination. Blood pressure differential and pulse delay are pathognomonic. The following physical findings may be noted:

  • Frequently normal physical appearance (except when coarctation compromises the origin of the left subclavian artery or in cases of XO Turner syndrome)

  • Abnormal differences in upper- and lower-extremity arterial pulses and blood pressures; diminished and delayed pulses distal to obstruction

  • Characteristic murmurs and sounds on auscultation (eg, continuous or late systolic murmur posteriorly over the thoracic spine, bilateral collateral arterial murmurs, aortic ejection sound, short midsystolic murmur, or early diastolic murmur of aortic regurgitation)

  • Associated cardiac defects (eg, left-side obstructive or hypoplastic defects and ventricular septal defects, bicuspid aortic valve, aortic arch hypoplasia, and, rarely, various right-side cardiac obstructive lesions)

  • Extracardiac vascular anomalies (eg, aberrant subclavian artery, berry aneurysms of the circle of Willis, development of large upper-to-lower collateral arteries, or hemangiomas)

  • Extracardiac nonvascular anomalies (eg, head and neck, musculoskeletal, gastrointestinal, genitourinary, or respiratory)

See Presentation for more detail.

Diagnosis

No specific laboratory tests are necessary for coarctation of the aorta. Imaging studies that may be helpful include the following:

  • Chest radiography: Findings vary with the clinical presentation of the patient

  • Barium esophagography: Classic “E sign,” representing compression from the dilated left subclavian artery and poststenotic dilatation of the descending aorta

  • Echocardiography (2-dimensional echocardiography, pulsed-wave Doppler, and color flow mapping): In older patients, surface echocardiography may not suffice, and magnetic resonance imaging (MRI), transesophageal echocardiography (TEE), or cardiac catheterization with angiogram may be necessary

  • Fetal echocardiography

  • MRI: This test is sensitive but expensive, time-consuming, and not universally available; it is seldom used as a primary diagnostic tool

Other studies that may be useful are as follows:

  • Cardiac catheterization

  • Electrocardiography

See Workup for more detail.

Management

Medical treatment of neonates with severe aortic coarctation may include the following:

  • Intubation

  • Infusion of prostaglandin E1 (PGE1) to open the ductus arteriosus

  • Correction of acidosis

  • Inotropic support to improve symptoms of congestive heart failure (CHF)

Medical treatment of less severe aortic coarctation beyond the neonatal period may include the following:

  • Administration of digoxin and diuretics for chronically increased afterload and signs of CHF

  • Postponement of intervention (eg, surgery or balloon dilatation) until the patient is hemodynamically stable

At present, the following 3 specific indications exist for intervention:

  • Significant coarctation or recoarctation of the aorta with long-standing hypertension with or without symptoms

  • Hemodynamically significant aortic stenosis

  • Female patient contemplating pregnancy

The following surgical procedures have been performed to treat aortic coarctation:

  • Resection of the coarctation site and end-to-end anastomosis to repair coarctation (still the preferred surgical method)

  • Patch aortoplasty

  • Left subclavian flap angioplasty

  • Bypass graft repair bridging the ascending and descending aorta

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.

See Treatment and Medication for more detail.

Background

Coarctation of the aorta (CoA), a narrowing of the aorta most commonly found just distal to the origin of the left subclavian artery, is a common condition found in children. Most patients with coarctation have juxtaductal coarctation. Older terms, such as preductal (infantile-type) or postductal (adult-type), are often misleading.

This condition occurs in 40 to 50 of every 100,000 live births and has a male-to-female predominance of 2:1.[1] Aortic coarctation is commonly treated after birth or during childhood.

Coarctation of the aorta is rarely seen in adults[1, 2, 3]  However, when affected adults present, they may have a history of a previous coarctation procedure, rupture of an old repair, heart failure, aortic aneurysm, aortic dissection, undersized grafts of previous repairs, intracranial hemorrhage, hypertension with exercise, and infections.[1]

The prognosis for untreated aortic coarctation is poor. About 80% of untreated patients die of aortic dissection or rupture, heart failure, or intracranial hemorrhage.[1] The traditional treatment for coarctation of the aorta is open surgery. A less-invasive treatment option is endovascular balloon dilatation and stent placement.[1]   

See the Guidelines section for a summary of guidelines for the management of aortic coarctation in adults.

Pathophysiology

The vascular malformation responsible for aortic coarctation is a defect in the vessel media, giving rise to a prominent posterior infolding (the "posterior shelf"), which may extend around the entire circumference of the aorta. The gross pathology of coarctation varies considerably. The lesion is often discrete but may be long, segmental, or tortuous in nature.

Histology

The coarctated aortic segment reveals an intimal and medial lesion consisting of thickened ridges that protrude posteriorly and laterally into the aortic lumen. The ductus (ie, patent embryonic remnant) or ligamentum arteriosus (closed and fibrosed) inserts at the same level anteromedially. Intimal proliferation and disruption of elastic tissue may occur distal to the coarctation. At this site, infective endarteritis, intimal dissections, or aneurysms may occur. Cystic medial necrosis occurs commonly in the aorta adjacent to the coarctation site and acts as a substrate for late aneurysm formation or aortic dissection in some patients.

Embryology

Coarctation is due to an abnormality in development of the embryologic left fourth and sixth aortic arches that can be explained by two theories, the ductus tissue theory and the hemodynamic theory.

In the ductus tissue theory, coarctation develops as the result of migration of ductus smooth muscle cells into the periductal aorta, with subsequent constriction and narrowing of the aortic lumen. Commonly, coarctation becomes clinically evident with closure of the ductus arteriosus. This theory does not explain all cases of coarctation. Clinically, coarctation may occur in the presence of a widely patent ductus arteriosus, and it may occur quite distant from the insertion of the ductus arteriosus, such as in the transverse arch or abdominal aorta.

In the hemodynamic theory, coarctation results from the reduced volume of blood flow through the fetal aortic arch and isthmus. In a normal fetus, the aortic isthmus receives a relatively low volume of blood flow. Most of the flow to the descending aorta is derived from the right ventricle through the ductus arteriosus. The left ventricle supplies blood to the ascending aorta and brachiocephalic arteries, and a small portion goes to the aortic isthmus. The aortic isthmus diameter is 70%-80% of the diameter of the neonatal ascending aorta.

Based on this theory, lesions that diminish the volume of left ventricular outflow in the fetus also decrease flow across the aortic isthmus and promote development of coarctation. This helps to explain the common lesions associated with coarctation, such as ventricular septal defect, bicuspid aortic valve, left ventricular outflow obstruction, and tubular hypoplasia of the transverse aortic arch. However, this theory does not explain isolated coarctation without associated intracardiac lesions.

Etiology

The exact etiology of coarctation of the aorta is not known. Note the following:

  • Genetics: Coarctation is seven times more common in white persons than Asian persons. It has a lower incidence among Native Americans than other population groups in Minnesota.

  • Environment: Environmental variation and seasonal variation have been suggested to influence the development of coarctation. A study showed an increased incidence of coarctation in late fall and winter births.[4]

  • Gridlock mutation: In 1995, Weinstein et al discovered a recessive mutation in zebrafish that causes a focal malformation resembling coarctation in humans.[5]  The mutation, named gridlock, results in failure of vascular channel formation at the site where bilaterally paired dorsal aortas join together and continue posteriorly as a dorsal midline channel, the medial dorsal aorta.

Epidemiology

Coarctation of aorta represents 5%-8% of all congenital heart diseases,[6, 7] with the isolated form comprising 4%-6% of all congenital heart diseases.[8]  The prevalence of isolated forms is about 3-4 per 10,000 live births,[6, 8]  and males are affected more frequently than females.[9]

Race-, sex-, and age-related demographics

Aortic coarctation is seven times more common in white persons than Asian persons. It has a lower incidence among Native Americans than other population groups in Minnesota.

The male-to-female predominance is 1.3-2:1 in most series.

The age at detection of coarctation of the aorta is dependent on the severity of the obstruction and the coexistence of other lesions.

Prognosis

Patients who are not treated for coarctation of the aorta may reach the age of 35 years[10] ; about 25% survive to age 46 years,[10] and fewer than 20% survive to age 50 years. If coarctation is repaired before the age of 14 years, the 20-year survival rate is 91%. If coarctation is repaired after the age of 14 years, the 20-year survival rate is 79%. The 30-year survival rate is almost doubled with surgical repair, with 72%-98% of these patients reaching adulthood.[11]

After repair of coarctation of the aorta, 97%-98% of patients are NYHA class I. Impaired diastolic left ventricular function and persistent hypertrophy due to increased pressure gradient at the coarctation site during exercise may result in myocardial hypertrophy despite successful hemodynamic results. Overall, left ventricular systolic function is normal or hyperdynamic in these patients

Most women reach childbearing age. If maternal coarctation is not repaired, risks to fetus and mother are increased. The maternal mortality rate is approximately 3%-8%. Note the following:

  • Despite repair, women have an increased risk of aortic dissection and rupture of cerebral aneurysm in the third trimester and peripartum period due to hemodynamic and hormonal changes.

  • All pregnant women with a history of coarctation, either native or repaired, should be considered high risk.

  • Significant stenosis—native, residual, or recurrent—is a contraindication to pregnancy.

Complications

Late complications of aortic coarctation include recurrent coarctation, malignant hypertension, left ventricular dysfunction, aortic valve dysfunction, and aneurysm formation with risk of rupture.[11]

Postoperative complications include the following:

  • Hoarseness due to damage to the recurrent laryngeal nerve as it loops around the patent ductus arteriosus or ligamentum.

  • Ipsilateral diaphragmatic paralysis may result from injury to the phrenic nerve.

  • Chylothorax can occur due to damage to the thoracic duct that crosses behind the aortic arch and left subclavian artery.

  • Serious postoperative hemodynamic collapse may result from hemorrhage due to injury to the chest wall collateral vessels.

  • Rebound and paradoxical hypertension is observed frequently and may be related to the baroreceptor-mediated increase in sympathetic activity and reflex vasospasm in the vascular territory distal to the coarctation.

  • Postcoarctectomy syndrome is a unique problem early in the postoperative period. Increases in blood flow and pressure in the mesenteric arteries after repair of coarctation may result in abdominal distention and pain, vomiting, and decreased bowel sounds. This syndrome may be masked because of poorly controlled postoperative hypertension and early enteral feeding. By aggressively controlling postoperative hypertension and delaying enteral feeding for 2 days after surgery, the incidence of postcoarctectomy syndrome may be reduced.

  • Paralysis of the lower body resulting from spinal cord injury is the most serious complication. Because of complex collateral vessel formation, ischemia of the spinal cord is often difficult to predict and, therefore, may be unavoidable.

  • Systemic hypertension is the most common long term complication. This may be accentuated by exercise, creating a need to exclude residual or recurrent obstruction.

Patient Education

Most adults with aortic coarctation have previously undergone repair; however, continued education regarding exercise, endocarditis and endarteritis prevention, and pregnancy issues is necessary.

For the rare adult with uncorrected coarctation, extensive patient education is necessary on issues ranging from pathology and repair to lifestyle modification and follow-up care.

The medical practitioner must understand that coarctation is a complex lifelong condition that may be repaired but is never truly corrected.

 

Presentation

History

Early life

Depending on severity of the obstruction and associated cardiac lesions, patients with aortic coarctation may present with congestive heart failure, severe acidosis, or poor perfusion to the lower body.[10]

Newborns typically present with severe narrowing of the upper thoracic aorta below the isthmus and adjacent to the arterial duct.[7]

Beyond infancy

Patients are usually asymptomatic. They may present with hypertension, headache, nosebleed, leg cramps, muscle weakness, cold feet, or neurologic changes.

Physical Examination

The diagnosis of coarctation of the aorta generally can be made on physical examination. A blood pressure differential and brachial-femoral pulse delay are pathognomonic.

Physical appearance

Patients may appear healthy. If coarctation compromises the origin of the left subclavian artery, the left arm is smaller than normal. Otherwise, general development is normal.

In XO Turner syndrome, a condition frequently associated with coarctation, a phenotypic female has the following features: short stature, webbed neck, absent or scanty axillary and pubic hair, broad chest and widely spaced hypoplastic or inverted nipples, low posterior hairline, small chin, prominent ears, cubitus valgus, short fourth metacarpals and metatarsals, distal palmar triaxial radii, narrow hyperconvex nails, and extensive pigmented cutaneous nevi.

Arterial pulse

Abnormal differences in the upper and lower extremity arterial pulses and blood pressures are clinical hallmarks of coarctation of the aorta. Pulses distal to the obstruction are diminished and delayed. This may be appreciated best by simultaneous arm and leg pulse palpation.

Auscultation

A continuous "machinery" murmur throughout both systole and diastole or a late systolic murmur is best heard posteriorly over the thoracic spine. Collateral arterial murmurs are crescendo-decrescendo in shape and delayed in onset and termination because of their origins in vessels some distance from the heart. The collateral murmurs are present bilaterally.

If the patient has an associated bicuspid aortic valve, an aortic ejection sound, a short midsystolic murmur, and/or early diastolic murmur of aortic regurgitation may be audible.

Associated cardiac defects

Associated cardiac defects are observed in approximately 50% of patients with aortic coarctation.

The most commonly reported defects are left-sided obstructive or hypoplastic defects and ventricular septal defects. Bicuspid aortic valve is observed in 85% of patients. Also, aortic arch hypoplasia is commonly found in coarctation associated with intracardiac defects.[12]

Right-sided cardiac obstructive lesions, such as pulmonary stenosis, pulmonary atresia, or tetralogy of Fallot, are observed rarely.

Extracardiac vascular anomalies

Extracardiac vascular anomalies commonly occur in patients with aortic coarctation. A right subclavian artery that arises aberrantly from the descending aorta distal to the coarctation occurs in 5% of patients. Rarely, both subclavian arteries originate distal to the coarctation.

Berry aneurysms of the circle of Willis occur in 3%-5% of patients and may result in subarachnoid bleeding.

After years of coarctation, large collateral arteries develop from the upper to lower body (ie, internal mammary arteries connecting to external iliac arteries, and spinal and intercostal arteries connecting to the descending aorta).

Hemangiomas also have been reported with coarctation.

Extracardiac nonvascular anomalies

Extracardiac nonvascular anomalies occur in 25% of patients with coarctation of the aorta. Coarctation of the aorta occurs in 35% of patients with Turner syndrome.

Abnormalities of the musculoskeletal, genitourinary, gastrointestinal, or respiratory system may be observed in as many as 25% of children with coarctation. In addition, an increasing number of children have head and neck abnormalities observed by ultrasonography.

 

DDx

Diagnostic Considerations

The differential diagnosis of coarctation of aorta depends on the patient's clinical presentation.

Neonates with heart failure may present with the following:

  • Coarctation of aorta
  • Severe obstruction to left heart (eg, hypoplastic left heart syndrome, severe critical aortic valve stenosis)
  • Sepsis
  • Myocarditis
  • Perinatal hypoxia

Patients with unequal pulses and blood pressures may present with the following:

  • Peripheral arterial disease (due to atherosclerosis, or arterial thrombosis from previous catheterization)
  • Prior surgical ligation (history of Blalcock-Taussig shunt)
  • Aortic dissection
  • Supravalvular aortic stenosis

Differential Diagnoses

 

Workup

Approach Considerations

The diagnosis of coarctation of the aorta generally can be made on clinical evaluation, particularly the physical examination. No specific laboratory tests are necessary.

Electrocardiogram (ECG) findings include the following:

  • Newborns and older children with milder forms of coarctation may have a normal ECG. If associated cardiac defects are present, then the ECG may be abnormal.
  • In older patients, long-standing coarctation of the aorta or a higher gradient across the coarctation stimulates left ventricular hypertrophy.

Cardiac catheterization can be used to determine the severity of coarctation, as follows:

  • If the peak gradient across the coarctation is below 20 mm Hg, the coarctation is mild.
  • A peak gradient above 20 mm Hg across the coarctation is suggestive of the need for intervention.

Imaging Studies

Radiography

Radiographic findings include the following:

  • Radiographic findings vary with the clinical presentation of the patient. In coarctation diagnosed early in life, chest radiography shows cardiac enlargement and pulmonary venous congestion. Associated cardiac defects may mask these findings.

  • In older children, chest radiographic findings are usually normal. The study typically shows a prominent aortic knob, and the stenotic region may be observed as an indentation of the proximal thoracic descending aorta in the shape of a number 3.

  • Rib notching is observed as irregularities and scalloping on the undersurface of the posterior ribs. This finding is unusual in children younger than 5 years and is observed more frequently in patients with significant gradient across coarctation of long standing.

Esophagography

Barium esophagrams show the classic "E sign," representing compression from the dilated left subclavian artery and poststenotic dilatation of the descending aorta.

Echocardiography

Echocardiography can be used to dianose aortic coarctation. Note the following:

  • Diagnosis of coarctation of the aorta is made by two-dimensional echocardiography, pulsed-wave Doppler ultrasonography, and color flow mapping. Classic findings are narrowing of the isthmus and posterior indentation or shelf. Blood flow velocities proximal and distal to obstruction, measured by Doppler tracings, can be used to estimate pressure gradient across the coarctation by a modified Bernoulli equation. Color flow mapping shows changes in color at the site of obstruction due to increases in blood velocity and turbulence.

  • In older patients, coarctation may be difficult to diagnose by surface echocardiography. For these patients, magnetic resonance imaging (MRI), transesophageal echocardiography (TEE), or cardiac catheterization with angiography may be necessary to make the diagnosis.

Fetal echocardiography can be used to diagnose aortic coartation. Consider the following:

  • Detection of coarctation in utero is a difficult task for the echocardiographer.

  • Some features suggestive of the presence of coarctation include enlargement of the right ventricle compared to the left ventricle, reduction of the isthmus and transverse aortic diameters of less than 3% for gestational age, hypoplasia of left-sided structures, and decrease or reversal of flow in the foramen ovale.

MRI

MRI can be a useful study in suspcted aortic coartation. Note the following:

  • MRI is a sensitive test for location and extent of coarctation as well as involvement of adjacent vessels and presence of collaterals. However, it is expensive, time consuming, and not universally available.

  • MRI is seldom used as a primary diagnostic tool. It is a useful tool for detecting and monitoring aneurysms and restenosis.

 

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.

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 advantagesof 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.

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]

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.

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

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 notindicated for the following:

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

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[24]

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 includes 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.[24]

2014 European Society of Cardiology (ESC) guidelines

The ESC guidelines largely agree with the ACC/AHA recommendations (all level of evidence: C).[6]

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.

 

 

Medication

Medication Summary

No specific medications are used to treat coarctation of the aorta because it is a mechanical obstruction. In the neonate, management of concomitant congestive heart failure may include prostaglandin E1 to maintain patency of the ductus arteriosus. Beyond the neonatal period, management of congestive heart failure may include digoxin and diuretics.

Hypertension should be controlled by beta-blockers, angiotensin-converting enzyme inhibitors, or angiotensin-receptor blockers as first-line medications. The choice of beta-blockers or vasodilators may be influenced in part by the aortic root size, the presence of aortic regurgitation, or both.[24]

Prostaglandins

Class Summary

This agent promotes vasodilatation by direct effect on the vasculature and smooth muscle of the ductus arteriosus.

Alprostadil intracavernous/urethral (Prostin VR)

Used to maintain patency of ductus arteriosus when cyanotic lesion or interrupted aortic arch presents in newborn. Most effective in premature infants.

Cardiac glycosides

Class Summary

These agents increase the contractility of cardiac muscle in a dose-dependent manner (ie, positive inotropic effect).

Digoxin (Lanoxin)

Cardiac glycoside with direct inotropic effects in addition to indirect effects on cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

Loop diuretics

Class Summary

These agents inhibit electrolyte reabsorption in the thick ascending limb of the loop of Henle, thus promoting diuresis.

Furosemide (Lasix)

Commonly used diuretic with moderate diuretic potency.