Management of coarctation of the aorta

INTRODUCTION — Coarctation of the aorta (CoA) is a discrete narrowing of the thoracic aorta just distal to the left subclavian artery (image 1). The care of a patient with CoA depends upon the severity of the CoA, patient age, and clinical presentation.

The management of CoA, including corrective treatment options and complications, will be reviewed here. The clinical manifestations, natural history, and diagnosis of CoA are discussed separately. (See "Clinical manifestations and diagnosis of coarctation of the aorta".)

MANAGEMENT APPROACH

Overview — Corrective intervention (ie, surgery or transcatheter intervention) should be performed in patients with CoA with any of the following (see 'Indications for intervention' below):

●Critical CoA

●CoA gradient >20 mmHg

●Radiologic evidence of clinically significant collateral flow

●Systemic hypertension attributable to CoA

●Heart failure attributable to CoA

Our management approach is based on the age and size of the patient and is generally consistent with evidence-based guidelines of the American Heart Association (AHA), the American College of Cardiology (ACC), the European Society of Cardiology, and the Canadian Cardiovascular Society for adult and pediatric patients with congenital heart disease (CHD) including CoA [1-4]. These guidelines stress that correction of coarctation should be performed as early as possible (optimally, early in childhood) to reduce the long-term morbidity and improve survival.

Neonates with critical coarctation — Neonates with severe ("critical") CoA are at risk for developing heart failure and death when the ductus arteriosus closes. Identification of these patients is essential in order to maintain patency of the ductus prior to surgical repair. In addition, immediate treatment is required to stabilize patients with heart failure. (See "Clinical manifestations and diagnosis of coarctation of the aorta", section on 'Neonates'.)

Medical therapy consists of the following:

●Continuous intravenous infusion of prostaglandin E1 (also known as alprostadil) to keep the ductus arteriosus open (see "Diagnosis and initial management of cyanotic heart disease in the newborn", section on 'Prostaglandin E1')

●Intravenous inotropic support (eg, milrinone, dopamine, or dobutamine) to improve contractility in those with heart failure (see "Heart failure in children: Management", section on 'Inotropes')

●Supportive care to correct metabolic acidosis, hypoglycemia, respiratory failure, and anemia that may contribute to or be a consequence of heart failure

With the use of prostaglandin E1 and other supportive measures, it is rare for affected neonates to require emergency surgical repair or balloon angioplasty as a palliative emergency procedure [2].

Once the patient is stabilized, surgical repair can be performed. Surgical repair is preferred over balloon angioplasty in neonates because it is associated with a lower risk for reintervention. (See 'Surgery' below.)

Children and adults with noncritical presentation — Management of patients with a noncritical presentation depends on the age and size of the patient.

Indications for intervention — Indications for intervention in patients with CoA include any of the following [1,5-7]:

●Resting CoA gradient >20 mmHg – The gradient can be estimated by the difference in systolic blood pressure from upper to lower extremity or with echocardiography. The peak-to-peak gradient can also be measured directly with catheterization (usually performed at the time of intervention). The threshold for a significant resting gradient may be lower in the setting of low forward stroke volume (eg, significant left ventricular systolic dysfunction). (See "Clinical manifestations and diagnosis of coarctation of the aorta", section on 'Echocardiography' and "Clinical manifestations and diagnosis of coarctation of the aorta", section on 'Cardiac catheterization'.)

●Radiologic evidence of significant collateral circulation.

●Systemic hypertension attributable to CoA.

●Heart failure attributable to CoA, which is most commonly seen in neonates with critical coarctation, though it can occur in older patients.

The rationale for intervening in patients who fulfill one or more of these criteria is based upon natural history data demonstrating poor long-term survival in individuals with unrepaired CoA and the generally favorable outcomes reported following transcatheter or surgical repair (see 'Procedures' below). Patients with clinically significant CoA are at risk of developing long-term complications, including severe systemic hypertension, accelerated coronary artery disease, stroke, aortic dissection, heart failure, and premature death. For individuals with unrepaired CoA, the mean survival age is 35 years, with only approximately 25 percent of patients surviving to the age of 45 years [1]. By contrast, when CoA is identified and treated before development of severe complications, long-term survival is excellent, with an estimated 20-year survival rate of approximately 90 percent [8]. (See "Clinical manifestations and diagnosis of coarctation of the aorta", section on 'Natural history' and 'Prognosis' below.)

Indications for intervention are not based solely upon gradient, because the resting gradient alone may be an unreliable indicator of severity when there is significant collateral circulation [5].

Choice of intervention

Infants and young children — The decision between balloon angioplasty versus surgical repair is determined by the multidisciplinary team based on expertise of the tertiary center and the underlying morphology of the coarctation. Our approach is consistent with the 2011 AHA pediatric guidelines for transcatheter intervention for CHD [2]:

●In neonates and infants <4 months old, we suggest surgical repair rather than balloon angioplasty because it is associated with a lower risk for reintervention. (See 'Surgery' below.)

●For infants with CoA who are ≥4 months old and for children who weigh <25 kg, the decision regarding balloon angioplasty versus surgical repair is determined by the expertise of the center and the underlying morphology of the coarctation. In our center, balloon angioplasty is the preferred procedure if the lesion is discrete and there is no evidence of arch hypoplasia [2]. However, surgical repair continues to be the preferred approach in many centers worldwide for patients <5 years old. (See 'Balloon angioplasty' below.)

●In patients with complex coarctation anatomy or underlying genetic disorder (eg, Turner syndrome), the decision to use balloon angioplasty versus surgical repair is made on a case-by-case basis.

Older children and adults — For patients with discrete CoA who weigh ≥25 kg (ie, large enough to allow use of an adult-size stent), transcatheter intervention with stenting has become the preferred intervention for native CoA in many tertiary centers, including the authors' institutions. (See 'Stent placement' below.)

For most older children and adults who have an indication for intervention for CoA, including those with long-segment lesions (ie, >10 to 15 mm in length), we perform stent placement if the stent can be expanded to an adult size. We also perform stenting (size permitting) in the setting of a failed balloon angioplasty due to vessel recoil.

Stenting may be less successful in patients with suboptimal anatomy (eg, vessel tortuosity and/or transverse arch hypoplasia) [3]. For these patients, the decision to perform stent placement versus surgical correction is made on a case-by-case basis by the multidisciplinary team.

Timing of intervention — Systemic hypertension, accelerated coronary heart disease, stroke, aortic dissection, and heart failure are common complications in adults with unrepaired CoA and in patients who undergo intervention later in life [1]. CoA repair after early childhood does not prevent persistence or late recurrence of systemic hypertension. As a result, intervention for CoA should be performed in infancy or early childhood to prevent the development of chronic systemic hypertension [9]. If CoA escapes early detection, repair should be performed at the time of subsequent diagnosis. (See 'Long-term cardiovascular complications' below.)

Pregnancy — The following approach is generally consistent with guidelines of the AHA/ACC and European Society of Cardiology [1,10].

Preconception counseling — Individuals with CoA and associated lesions (particularly bicuspid aortic valve, aortic stenosis, and ascending aorta dilation) who are trying to conceive should be evaluated by an adult CHD specialist before pregnancy and should receive appropriate preconception counseling [1]. Potential risks that should be discussed include heritable CHD, gestational hypertension and preeclampsia, aortic rupture or dissection, and intracranial hemorrhage. Issues related to bicuspid aortic valves are discussed in greater detail separately. (See "Bicuspid aortic valve: Management during pregnancy".)

Similarly, patients with Turner syndrome and known or suspected CoA should be evaluated by an adult CHD specialist before considering pregnancy or in vitro fertilization. (See "Management of Turner syndrome in adults", section on 'Management of fertility and pregnancy'.)

Patients with CoA with an indication for corrective intervention should undergo intervention prior to pregnancy (see 'Indications for intervention' above). Individuals with severe CoA should be counseled to avoid pregnancy until intervention is performed. Appropriate counseling on contraception should be provided. (See "Contraception: Counseling and selection".)

Management during pregnancy — The risk of major complications during pregnancy varies depending on the status of the CoA (successfully repaired versus unrepaired, residual, or recurrent coarctation) and the severity of hypertension. Risks associated with hypertension in pregnancy are discussed in greater detail separately. (See "Chronic hypertension in pregnancy: Prenatal and postpartum care", section on 'Risks of chronic hypertension in pregnancy'.)

●Repaired coarctation – Most individuals who have previously undergone successful CoA repair have uncomplicated pregnancies, although the rates of miscarriage and preeclampsia are higher than in the general population [11-14]. Patients with significant recoarctation should undergo corrective intervention prior to pregnancy.

In one retrospective study of 126 pregnancies in 54 individuals with repaired CoA, there were 98 successful pregnancies, 22 miscarriages, and 6 abortions [13]. There were no serious cardiovascular complications during pregnancy and delivery. Hypertension alone was reported in 17 percent of pregnancies and preeclampsia in 4 percent. Five of eight patients who had serial echocardiographic assessments had a ≥15 mmHg increase in the CoA gradient. Though serious pregnancy-related complications are rare in patients with repaired CoA, aortic dissection has been described in case reports [15].

●Unrepaired, residual, or recurrent CoA – Major complications associated with unrepaired, residual, or recurrent CoA during pregnancy are uncommon but can be fatal [11]:

•Individuals with unrepaired CoA are at increased risk of developing gestational hypertension and preeclampsia [14].

•Pregnancy increases the risk of aortic rupture or dissection at the site of narrowing in patients with CoA (repaired or unrepaired) and is associated with systemic hypertension, residual CoA, or aortic aneurysm, or in the ascending aorta in patients with a coexisting bicuspid aortic valve [10]. (See "Bicuspid aortic valve: Management during pregnancy".)

•Intracranial hemorrhage can occur, but hypertension is not a necessary precondition.

•Heart failure is uncommon despite the increased volume load of pregnancy imposed on the already pressure-loaded left ventricle [14,16]. (See "Maternal adaptations to pregnancy: Cardiovascular and hemodynamic changes".)

Individuals who present with an unrepaired CoA during pregnancy should receive careful surveillance of the adequacy of blood pressure control with regular follow-up [10]. A reasonable blood pressure goal during pregnancy is 120 to 140/80 to 90 mmHg, although a lower target may be warranted if there is ascending or other aortic aneurysmal disease. Care should be taken to avoid placental hypoperfusion in those with residual coarctation [10]. Management of hypertension in pregnancy is discussed in greater detail separately. (See "Treatment of hypertension in pregnant and postpartum patients".)

In the absence of an obstetrical indication for early delivery, patients who are normotensive can carry the pregnancy to term and pursue CoA intervention after the physiologic changes of pregnancy have resolved (typically approximately three months after delivery). For patients with poorly controlled hypertension during pregnancy, the decision to perform surgical or transcatheter intervention during pregnancy is made on a case-by-case basis by a multispecialty team. Every effort should be made to manage the patient medically and then intervene after the pregnancy is complete. Percutaneous intervention for recoarctation (using a covered stent) is possible during pregnancy but should only be performed for severe refractory hypertension or maternal/fetal compromise [10]. If stent placement is performed during pregnancy, potential teratogenic exposure from radiation can be reduced by performing the procedure during the second trimester with abdominal shielding. (See 'Stent placement' below.)

Mode of delivery — For patients with CoA (unrepaired or repaired), particularly those with hypertension, spontaneous vaginal delivery with use of epidural anesthesia is generally preferred. Cesarean delivery is generally reserved for obstetric indications. However, it is suggested for gravidas with advanced heart failure and hemodynamic instability despite medical management.

Complications in the offspring — In a systematic review of studies published between 1985 and 2007, the rate of preterm delivery among women with CoA (repaired and unrepaired) was 7.9 percent, a rate comparable with that of the general population [14]. CHD (including CoA and other defects) occurred in 4 percent of offspring, a rate that is approximately fourfold higher than that of the general population. Perinatal mortality was 2.4 percent, which is considerably higher than in the general population (<0.5 percent).

PROCEDURES — Options for correction of native discrete CoA include transcatheter interventions (eg, balloon angioplasty and/or stent placement) and surgery. The approach to determining the need for intervention and selecting among these procedures is discussed above. (See 'Management approach' above.)

Balloon angioplasty — Balloon angioplasty is a transcatheter alternative to surgical repair for native discrete coarctation in infants >4 months old and young children who weigh <25 kg. It is the preferred intervention for all patients with isolated recoarctation, regardless of age [1-3]. However, stent placement has replaced balloon angioplasty as the procedure of choice in larger children (≥25 kg) and adults with native discrete or long-segment coarctation; in such patients, balloon angioplasty is an option if stent placement is not feasible and surgical intervention is not an option [1]. (See 'Stent placement' below.)

Based on data from retrospective studies and one small randomized trial, balloon angioplasty and surgical correction appear to be equally effective in acutely reducing the pressure gradient [17-21]. In addition, in long-term follow-up of 21 of the patients enrolled in the randomized trial, resting blood pressure and exercise performance were similar between patients who underwent balloon angioplasty and those who underwent surgical repair [19]. Patients treated with balloon angioplasty more frequently require reintervention for recoarctation or aneurysm. (See 'Recoarctation' below and 'Aortic aneurysm, dissection, and rupture' below.)

In a meta-analysis of nine studies (one randomized trial and eight observational studies), the postprocedure gradient was similar in patients treated with surgery compared with balloon angioplasty (weighted mean difference 1.44, 95% CI -1.16 to 4.04) [21]. The risk of severe short-term complications (aortic wall injury, dissection/intimal tear, aneurysm, balloon rupture, femoral artery injury, atrial fibrillation, severe/prolonged hypertension, bleeding, respiratory failure, or death) was greater with surgery compared with balloon angioplasty (38 versus 28 percent, respectively; odds ratio [OR] 2.67, 95% CI 1.37-5.21). However, in mid- to long-term follow-up, the risk of aneurysm formation was considerably lower with surgery (1 versus 16 percent; OR 0.12, 95% CI 0.04-0.34). Similarly, the risk of recoarctation was lower with surgery compared with balloon angioplasty (13 versus 30 percent; OR 0.25, 95% CI 0.12-0.54). Thus, the trade-off with balloon angioplasty is that it avoids some of the immediate risks associated with invasive surgery but does so at the cost of increasing the likelihood of requiring reintervention later in life.

Balloon angioplasty is not recommended for infants <4 months old, especially if there is arch hypoplasia [2]. Arch hypoplasia is commonly seen in neonatal and infantile CoA, and balloon angioplasty is unlikely to be successful in this setting [22,23]. Recoarctation occurs in most neonates after balloon angioplasty, even after a good initial result. Repeat balloon angioplasty is generally required within 5 to 12 weeks following the initial procedure. Palliative balloon angioplasty may be considered in critically ill patients (irrespective of age) who have heart failure due to severe ventricular dysfunction, mitral regurgitation, or low cardiac output [2]. (See 'Neonates with critical coarctation' above.)

Potential complications of balloon angioplasty and their frequencies include [6,18,24-32]:

●Recoarctation (5 to 25 percent) (see 'Recoarctation' below)

●Aortic aneurysm formation (5 to 7 percent) (image 2 and image 3) (see 'Aortic aneurysm, dissection, and rupture' below)

●Femoral arterial injury/thrombosis (up to 15 percent)

●Aortic dissection and rupture is a rare complication since it is not reported in most case series (see 'Aortic aneurysm, dissection, and rupture' below)

Stent placement — In children and adults, stent placement after balloon angioplasty or surgery sustains hemodynamic benefit by improving luminal diameter, thus resulting in minimal residual gradient (image 4) [33-38]. Children who undergo aortic stent placement are more likely to require a planned reintervention compared with adults since the stent often needs to be dilated as the child grows.

Stent placement is generally not recommended in patients who weigh <25 kg, due to potential injury to the femoral artery from the large sheath required for stent delivery. In addition, the aorta in patients <25 kg is small. Although stents have been successfully placed in patients <30 kg, implanting a stent in such patients will commit them to repeated interventions to expand the stent as they grow [39,40]. The risks associated with repeat transcatheter interventions need to be balanced against the more invasive surgical repair. In our practice, we only consider stent placement in patients who are large enough to receive a stent that can be expanded to an adult size [2].

The efficacy and safety of stent placement for both native and recurrent coarctation are supported by data from prospective and retrospective multicenter studies [35,36,41]. In the Coarctation of the Aorta Stent Trial I (COAST), a prospective, multicenter, single-arm clinical trial of 105 children and adults (median age 16 years) who underwent attempted stent implantation for treatment of native or recurrent coarctation, stent placement was successful in 99 percent [41]. All patients experienced immediate reduction in upper- to lower-extremity blood pressure difference, with sustained improvement to two years. There were no deaths or serious adverse events, and no patients required surgical intervention. During the first two years after stent placement, nine patients required reintervention to address aneurysms or for stent redilation (either as part of an intentionally staged approach or to compensate for somatic growth). An additional 10 patients required reintervention after two years (seven for redilation of the stent and three for redilation and to address aneurysms).

In a retrospective multicenter case series of >500 patients with CoA (both native and recurrent) who underwent stent placement (predominantly with bare metal stents), the success rate was 98 percent (defined as reduction in the gradient to <20 mmHg or a ratio of post-stent coarctation to descending aorta of >0.8) [35]. Acute complications occurred in 14 percent of patients, including two procedure-related deaths. Aortic abnormalities were noted in 25 percent of procedures with follow-up imaging, including stent restenosis (10 percent), aneurysms (8 percent), and dissections (3 percent).

Although the complication rates with bare metal stents in these studies were low, it has been suggested that the use of covered stents, which consist of a bare metal stent with a polytetrafluoroethylene sleeve, may reduce the risk of aneurysms [42-44]. In the COAST I trial, 9 of the 105 patients who underwent attempted stent placement with a bare metal stent had evidence of aortic wall injury and therefore had covered stents placed (either at the initial procedure or after one to two years).

However, a randomized trial in 120 adolescent and adult patients (mean age 23.6 years, range 12 to 58 years) with short-segment severe native coarctation did not detect a significant difference in the rates of recoarctation and pseudoaneurysm formation after 31 months of follow-up between patients who underwent implantation using a bare metal stent and those with a covered stent [45]. In both groups, stent placement resulted in a 100 percent success rate, but only three-quarters of patients became normotensive after the procedure.

These results suggest that bare metal stents may be sufficient in many, if not most, patients who undergo stent placement and that further research is needed to determine if there is a subset of patients who truly have incremental benefit from the implantation of a covered stent. Follow-up data will also be important to see if there is a long-term benefit regarding maintaining normal blood pressure using covered stents.

Both bare metal and covered stents are approved for use in patients with CoA and are commercially available in the United States, Europe, and other parts of the world. Covered stents can also be fashioned individually [46].

Surgery — The type of surgical repair performed depends on the size of the defect:

●Resection with end-to-end anastomosis is performed for repair of discrete CoA

●Subclavian flap aortoplasty in infants with long-segment CoA

●A bypass graft across the area of coarctation when the distance to be bridged is too long for an end-to-end repair [47]

Prosthetic patch aortoplasty is generally avoided if possible because of the frequent occurrence of aortic aneurysm or rupture (image 5) [48-51]. (See 'Aortic aneurysm, dissection, and rupture' below.)

Outcomes following surgical CoA repair are generally excellent. However, recoarctation is an important potential long-term complication, especially in neonates and young infants. (See 'Recoarctation' below.)

Perioperative mortality is rare (<1 percent in most series), even in reports limited to neonates and young infants [1,52,53]. In a retrospective study of 167 infants who underwent surgical repair between 1996 and 2006 and who were <90 days old at the time of surgery, survival was 98 percent at a median follow-up of 4.8 years [52]. Actuarial freedom from reintervention at five years was 89 percent. Surgical repair is possible in preterm infants with birth weights <2.5 kg; however, survival is lower compared with term infants (one-year survival rate of 76 percent in one study) [54].

Early postoperative complications can include paradoxical hypertension, left recurrent laryngeal nerve paralysis, phrenic nerve injury, and subclavian steal [1]. Subclavian steal should be suspected in patients with neurologic symptoms and reduced perfusion in the left arm because of compromise of the orifice of the left subclavian artery after subclavian flap angioplasty [55]. Paraplegia due to spinal cord ischemia and mesenteric arteritis with bowel infarction are rare complications.

Rebound hypertension (mean increase as high as 35 mmHg) is due initially to an increase in circulating catecholamines and may be accentuated by activation of the renin-angiotensin system [56,57]. These responses are more common after surgical repair than after balloon angioplasty [57]. Postoperative hypertension may be prevented or blunted by perioperative administration of antihypertensive agents, such as nitroprusside and/or beta blockers in both adult and pediatric patients [1,58-60]. Many patients remain hypertensive and require an oral antihypertensive agent(s) at discharge, as discussed below [60]. (See 'Systemic hypertension' below.)

Published reports of outcomes following surgical repair of CoA have included mostly patients undergoing resection with end-to-end anastomosis or subclavian flap aortoplasty. For patients undergoing bypass graft placement, postoperative outcomes appear to be related to the age of the patient and, hence, the size of the bypass graft. This was demonstrated in a retrospective study of 70 infants and children with aortic arch obstruction who underwent bypass graft placement at a single institution between 1982 and 2013 [47]. Children >1 year at the time of surgery (most of whom received a bypass graft >16 mm in size) had good long-term outcomes with low mortality and morbidity (15-year transplantation-free survival and freedom from aortic arch reoperation were 100 and 92 percent, respectively). By contrast, infants <1 year at the time of surgery (most of whom received a bypass graft <16 mm in size) had relatively high early mortality (16 percent) and poor long-term survival (15-year actuarial transplant-free survival of 64 percent). The authors concluded that aortic bypass graft should be avoided in neonates and infants, except in special circumstances. It should be noted that many of the infants in this case series had concomitant complex congenital heart disease (CHD), whereas only one of the older children had complex CHD.

LONG-TERM CARDIOVASCULAR COMPLICATIONS — Major long-term complications following CoA repair include recoarctation, aortic aneurysm, and systemic hypertension [61,62].

Recoarctation

Incidence — Recurrent coarctation refers to restenosis after an initially successful dilatation or operative repair. The major findings that suggest recurrent stenosis are resting hypertension, headaches (not usually found in children), and, occasionally, claudication. Recoarctation may also be noted on screening imaging studies [5].

●Recoarctation after surgery – The rate of recoarctation after surgery is approximately 5 to 15 percent [52-54,63,64]. It is seen primarily in children, usually due to inadequate aortic wall growth at the site of repair when surgery is performed before the aorta has reached adult size. The rate appears to be similar with the different surgical techniques [64].

●Recoarctation after balloon angioplasty – Reported rates of recoarctation following balloon angioplasty vary according to the age of the patient at the time of intervention [6,19,21,24,25,42]:

•Neonates and young infants – Up to 50 percent [65,66]

•Older children – Approximately 20 to 30 percent [6,19,21,25,67]

•Adolescents and adults – 8 percent [24]

Other risk factors for recoarctation include isthmus hypoplasia and coarctation segment <3.5 mm before or <6 mm after angioplasty [25,67].

Management of recoarctation

Indications for intervention — Indications for intervention for recoarctation are generally similar to those for initial intervention and include any of the following [1,2,5]:

●Hypertension in the setting of imaging evidence of recoarctation (eg, with ≥50 percent aortic narrowing relative to the aortic diameter at the diaphragm level)

●Pressure gradient across the coarctation >20 mmHg

●Imaging evidence of collateral circulation

Transcatheter intervention — Catheter-based intervention is our preferred treatment for discrete recoarctation [1,2]. Numerous observational studies have demonstrated successful repair of discrete recoarctation using percutaneous balloon angioplasty in children, including infants as young as three months of age [7,68-74]. Catheter-based intervention is preferred over surgical repair of recoarctation because mortality for reoperation is higher than for primary repair (1 to 3 percent versus <1 percent) [75,76]. Mortality for reoperation can be as high as 5 to 10 percent if there are significant comorbidities or left ventricular dysfunction. (See 'Balloon angioplasty' above.)

Surgical intervention for recoarctation is reserved for cases with confounding features, such as long recoarctation segment, hypoplasia of the aortic arch, or aortic aneurysm or pseudoaneurysm [1,5], or after failed catheter intervention. Surgical repair should be performed by a surgeon with training and expertise in congenital heart disease (CHD). (See 'Aortic aneurysm, dissection, and rupture' below.)

Aortic aneurysm, dissection, and rupture — An aortic aneurysm may develop at the site of prior coarctation following surgery, balloon dilatation, or stent implantation of native coarctation [48-51,77,78]. Aneurysm formation occurs at and around the coarctation site due to an inherent aortic wall medial abnormality, which is characterized by fragmentation of elastic fibers, an increase in ground substance, and a reduction in the number of smooth muscle cells [79]. While aneurysms typically occur at the site of previous repair (image 6), they may also affect the ascending aorta. Proposed mechanisms for developing aortic aneurysm and/or dissection after intervention for CoA include failure of surgical repair to remove abnormal tissue or damage from balloon angioplasty [80,81]. In addition, an abnormal aorta related to a coexisting bicuspid aortic valve may predispose to ascending aortic aneurysm formation or dissection [82,83]. (See "Clinical manifestations and diagnosis of bicuspid aortic valve in adults".)

Risk factors for developing aneurysms include older age at the time of CoA repair and the use of patch angioplasty (image 5) [50,78,84]. In addition, the risk of aortic dissection is increased during pregnancy, which is associated with hemodynamic, physiologic, and hormonal changes superimposed on the preexisting aortic wall medial changes. (See 'Pregnancy' above and "Maternal adaptations to pregnancy: Cardiovascular and hemodynamic changes".)

The reported incidence of aortic aneurysm after surgical repair or balloon angioplasty for CoA ranges from <1 to 9 percent [24,50,51,78]. In a retrospective series of 58 adult and adolescent patients (mean age 24 years) who underwent balloon angioplasty, 8 percent of patients developed aneurysms within one year after the procedure [24]. In another series of 29 patients (age range 15 to 71 years) who underwent balloon angioplasty, no aneurysms were detected at a mean follow-up of 8.5 years [85]. The risk of developing aortic aneurysm is particularly high after patch angioplasty [50,51], and, therefore, in the contemporary era, this procedure is generally avoided if possible. In a report of 891 patients who were evaluated 1 to 24 years after CoA repair, aortic aneurysms were noted in 8.7 percent of patients repaired using synthetic patch aortoplasty compared with 1.2 percent of patients who underwent resection with end-to-end anastomosis [51]. (See 'Surgery' above.)

Clinical features of acute aortic dissection and thoracic aortic aneurysm are discussed separately. (See "Clinical features and diagnosis of acute aortic dissection" and "Clinical manifestations and diagnosis of thoracic aortic aneurysm".)

Aortic aneurysm or pseudoaneurysm is generally treated surgically. Alternatively, endovascular stent grafts have been used to repair aortic aneurysms at the site of prior CoA repair. In one case series of six adult patients with aortic aneurysm following CoA repair (age range 31 to 68 years), placement of a stent graft was successful in all cases with no related morbidity or mortality at one-year follow-up after intervention [86]. In a second case series of nine patients, endovascular stenting was successfully performed without any major complications at a mean follow-up of 24 months [87]. (See "Endovascular repair of the thoracic aorta" and "Management of acute type B aortic dissection", section on 'Endovascular stent-grafting'.)

Systemic hypertension — Systemic hypertension is one of the major long-term problems following repair of CoA. Although the blood pressure typically falls after successful repair, persistent or recurrent hypertension and disproportionate systolic hypertension with exercise are observed, especially in patients whose repair is performed later in life. Recoarctation should be excluded in patients who develop hypertension following CoA repair. (See 'Recoarctation' above.)

Hypertension and left ventricular hypertrophy are among the factors that contribute to premature death from coronary and cerebrovascular disease in patients with repaired CoA [88]. Thus, control of hypertension is an important aspect of management. (See 'Long-term follow-up' below and 'Prognosis' below.)

Hypertension should be controlled by beta blockers, angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers, or calcium channel blockers [1]. The choice of agents may be influenced by the ascending aortic size and the presence of aortic regurgitation, though data to guide these decisions are very limited. If there is aortic dilation, beta blockers may be preferred, based on the theoretical benefit of their anti-impulse effect in this setting (see "Management of thoracic aortic aneurysm in adults", section on 'Antihypertensive therapies'). Calcium channel blockers are generally avoided in the setting of aortic dilation, based on animal and limited human data suggesting that they may increase the risk of aortic complications (see "Management of Marfan syndrome and related disorders", section on 'Drugs to avoid'). If there is severe aortic regurgitation, beta blockers are typically avoided since they prolong diastole and theoretically may increase aortic regurgitation. Patients with refractory hypertension may require a combination of drugs to effectively control blood pressure. (See "Overview of hypertension in adults", section on 'Pharmacologic therapy' and "Nonemergent treatment of hypertension in children and adolescents", section on 'Management approach'.)

Hypertension is more common in patients whose repair was performed after 20 years of age compared with those who were repaired in early childhood; however, the risk of hypertension increases over time in all patients [24,62,89-91]. This was demonstrated in a report on the long-term follow-up of 138 patients who underwent surgical CoA repair at a single center between 1949 and 1968 [91]. Among patients <20 years old at time of repair, >90 percent were normotensive at follow-up 10 years after surgery; this fell to approximately 80 and 40 percent at 15 and 25 years post-surgery, respectively. Among patients ≥20 years old at time of repair, approximately 70 percent were normotensive at 10-year follow-up, which fell to 55 and 35 percent at 15 and 25 years, respectively. In another single-center report of 92 patients who underwent surgical repair of CoA in childhood (mean age nine years) between 1948 and 1976 and who returned for follow-up evaluation ≥50 years after the original operation, 35 percent had systemic hypertension [90]. In a study of 58 adolescent and adult patients who underwent balloon angioplasty for repair of discrete CoA and who were followed for up to 22 years, 50 percent required at least one antihypertensive medication [24]. Normotensive patients, especially those repaired at an older age, often have an exaggerated rise in systolic pressure in response to exercise [90,92,93].

The factors responsible for the persistent risk of hypertension after coarctation repair are not well understood. Contributing factors may include structural and functional abnormalities that decrease compliance in the precoarctation arterial wall and increased left ventricular hypertrophy, stiffness, and hypercontractility post-repair [93-95].

Other cardiovascular disease — Despite coarctation repair, individuals with CoA are at increased risk for the following:

●Coronary artery disease and stroke [88,96,97] – The increased prevalence of coronary artery disease appears to be caused by traditional cardiovascular risk factors, as illustrated by a study of patients from the Quebec Congenital Heart Disease Database [96]. Rates of coronary artery disease, heart failure, stroke, peripheral vascular disease, hypertension, and hyperlipidemia were higher in 756 individuals with CoA (repaired or unrepaired) compared with 6481 individuals with a ventricular septal defect. Multivariate analysis demonstrated that the increased risk was largely accounted for by traditional cardiovascular risk factors, such as hypertension, hyperlipidemia, diabetes mellitus, male sex, and age. These findings highlight the importance of screening for and treating associated cardiovascular risk factors (eg, hypertension and dyslipidemia) in patients with CoA to prevent late cardiovascular sequelae. (See "Overview of the management of the child or adolescent at risk for atherosclerosis" and "Overview of primary prevention of cardiovascular disease".)

●Bicuspid aortic valve – Patients with CoA commonly have concomitant bicuspid aortic valve (up to 50 percent) [98]. These patients, despite repair of the coarctation, require lifelong follow-up to screen for aortic valve disease because of increased risk for stenosis or regurgitation and associated dilatation of the ascending aorta. (See "Bicuspid aortic valve: General management in adults".)

●Cardiac arrhythmias and sudden cardiac death – The risk of sudden death from presumed arrhythmias is low during the first 20 years after CoA repair; however, the incidence increases thereafter, particularly in patients who develop ventricular dysfunction [3]. (See "Primary prevention of sudden cardiac death in patients with cardiomyopathy and heart failure with reduced LVEF".)

●Intracranial aneurysms – Adults with CoA are at risk for development of intracranial aneurysms, as described separately (see "Clinical manifestations and diagnosis of coarctation of the aorta", section on 'Intracranial aneurysms'). The management of intracranial aneurysms is also discussed separately. (See "Unruptured intracranial aneurysms".)

LONG-TERM FOLLOW-UP

Monitoring — All patients with CoA (repaired or not) should have lifelong congenital cardiology follow-up and imaging because of the associated long-term cardiovascular risks and potential need for reintervention [62]. (See 'Long-term cardiovascular complications' above.)

Patients who have undergone surgical or transcatheter intervention for CoA should receive at least yearly clinical follow-up. Follow-up of adults should include evaluation by or consultation with a cardiologist with expertise in adult congenital heart disease (CHD) [1].

Follow-up monitoring includes the following [1]:

●Clinical evaluation for symptoms and signs of complications including hypertension, progression of bicuspid aortic valve disease, aortic recoarctation, heart failure (eg, related to left ventricular hypertrophy and/or coronary artery disease), or intracranial aneurysm [3].

●Measurement of blood pressure – Patients should have blood pressure measurements at all visits to closely monitor for the appearance or reappearance of systemic hypertension at rest and with exercise. Ambulatory blood pressure monitoring and/or exercise testing can help assess blood pressure control. Development of a significant arm-leg pressure gradient at rest is suggestive of recoarctation. Hypertension should be treated aggressively, and recoarctation should be excluded. (See "Nonemergent treatment of hypertension in children and adolescents" and "Overview of hypertension in adults".)

●Imaging of repair site – Patients should have periodic imaging of the coarctation repair site to detect long-term complications (eg, aneurysms and recoarctation) [99]. (See 'Imaging' below and 'Long-term cardiovascular complications' above.)

●Echocardiography – Echocardiography should be performed periodically to assess function of bicuspid aortic valve (when present), aortic root and ascending aorta diameters (particularly among patients with bicuspid aortic valve), and ventricular function. The recommended frequency depends on the associated cardiovascular conditions. (See "Bicuspid aortic valve: General management in adults", section on 'Surveillance'.)

●Exercise testing – The role of routine exercise testing for follow-up in adults is not well established; however, it can be helpful in assessing blood pressure control, particularly if blood pressure is labile. In addition, exercise testing should be performed in patients who wish to participate in competitive sports [1]. (See 'Competitive sports' below.)

●Monitoring for intracranial aneurysms – Because CoA is associated with an increased risk of intracranial aneurysms (ie, berry aneurysms), it is reasonable to perform periodic cranial imaging surveillance with either magnetic resonance angiography (MRA) or computed tomography angiography (CTA) [100]. The optimal timing for this surveillance is uncertain. In our practice, we perform MRA when the patient is old enough to cooperate with the test (usually after age 10 years). Repeat imaging is generally not necessary if the patient remains normotensive. For patients with hypertension, we repeat imaging every five years. (See "Clinical manifestations and diagnosis of coarctation of the aorta", section on 'Intracranial aneurysms'.)

Imaging — The choice of routine follow-up imaging depends on the age of the patient:

●Young children – In infants and younger children, acoustic windows permit excellent echocardiographic imaging so that magnetic resonance imaging (MRI) or CTA are generally not needed. Echocardiography is preferred over MRI or CTA in young children because it does not require sedation and does not expose the child to radiation. Although MRI is more sensitive compared with echocardiography [101], MRI has the disadvantage of requiring sedation in young children. Thus, most centers use echocardiography initially as a screening test until the patient is old enough to undergo MRI without sedation.

We suggest echocardiographic assessment of the area of coarctation within six months from intervention or earlier if there are clinical concerns (eg, elevated blood pressure, weak femoral pulses). If the initial imaging is reassuring, echocardiography is performed annually until the patient is old enough to cooperate for MRI imaging without sedation.

●Older children and adults – In adults and children who are old enough to cooperate, computed tomography (CT) and MRI are more sensitive than echocardiography for detecting both recoarctation and aneurysm formation [102]. MRI is preferred since it avoids the cumulative radiation exposure of multiple CT scans.

We suggest that MRI be done one year after intervention. The interval for subsequent imaging depends on the specific anatomic findings before and after repair and the clinical status. If the initial postintervention MRI is reassuring, follow-up MRI can be done every five years or as needed for change in clinical status.

Endocarditis prophylaxis — Antibiotic prophylaxis for infective endocarditis is generally not necessary for patients with CoA, except in the following circumstances (see "Prevention of endocarditis: Antibiotic prophylaxis and other measures"):

●If there is a previous history of endocarditis

●If the repair involved insertion of a conduit

●For six months after intervention if prosthetic material or stent was used

Physical activity — Data are lacking on the impact of exercise on patients with CoA. Recommendations have been developed by expert panels to provide guidance in this area.

Competitive sports — The 2015 scientific statement of the American Heart Association (AHA) and American College of Cardiology (ACC) provides competitive athletic participation guidelines for patients with CHD, including CoA [103]. We agree generally with these recommendations but stress that, as with any guidelines, recommendations need to be tailored to the patient and a comprehensive evaluation by an experienced clinician is required. Before a decision is made regarding sports participation, a detailed evaluation should be conducted, which should include a physical examination, electrocardiography, exercise testing, and cardiac/aortic imaging (with transthoracic echocardiogram, MRI, and/or CTA). The time interval for repeating this extensive testing is unclear and should be individualized to the specific patient.

●Patients with unrepaired coarctation can participate in all competitive sports if they meet all of the following criteria:

•Normal exercise test

•Resting arm/leg systolic blood pressure gradient <20 mmHg

•Peak systolic blood pressure ≤95^th percentile of predicted with exercise

•No significant ascending aortic dilation (Z-score ≤3.0)

●Patients who have undergone CoA repair (surgery or transcatheter intervention) may participate in competitive sports that do not pose a danger of bodily collision and do not require high-intensity static exercise (classes IIIA, IIIB, and IIIC) (figure 1) after three 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.

●Patients with an arm/leg systolic blood pressure gradient >20 mmHg or exercise-induced hypertension (peak systolic blood pressure >95^th percentile of predicted with exercise) or with significant ascending aortic dilation (Z-score >3.0) may participate only in low-intensity class IA sports (figure 1).

●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 (classes IA and IB) sports (figure 1).

Guidelines for patients with bicuspid aortic valve with or without aortic root dilation are discussed separately. (See "Bicuspid aortic valve: General management in adults", section on 'Physical activity and exercise'.)

Recreational sports — In our practice, the recommendation for the level of sport participation depends on the clinical status of the patient as assessed by echocardiography and blood pressure measurements in the upper and lower extremities. If the level of activity is comparable with that required by competitive athletes, an exercise stress test is performed.

●Patients with normal blood pressure, no residual coarctation, and normal ascending aorta diameter can participate in all activities without restriction (ie, normal life activity).

●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.

Physical activity and exercise in patients with CHD are discussed in greater detail separately. (See "Physical activity and exercise in patients with congenital heart disease".)

PROGNOSIS — The estimated 10-year survival following CoA repair is >90 percent [62,90,91]. Survival is best among patients who undergo successful repair early in life [62,88,90,91,104]. Factors associated with decreased survival include older age at initial repair (ie, older than 20 years) and preoperative hypertension [62].

In a single-center series of 819 patients (mean age at repair 17.2±13.6 years) who underwent surgical repair of isolated CoA at the Mayo Clinic between 1946 and 2005, the survival rates at 10, 20, and 30 years after primary repair were 93, 86, and 74 percent, respectively [62]. In an earlier report from the same center, the most common cause of late death was coronary artery disease, followed by sudden death, heart failure, cerebrovascular accident, and ruptured aortic aneurysm [88].

Other reports demonstrate similar survival rates [90,91]. Data from the United Kingdom Northern Congenital Abnormality survey of children born between 1985 and 2003 demonstrated that an estimated 20-year survival rate for individuals with CoA was 90 percent [8]. A report from the European Heart Survey on adult congenital heart disease (CHD) described 551 patients with CoA; 90 percent had a previous repair with a mean age of 26 years at the beginning of the study period [105]. At the end of the study, the five-year mortality rate was 0.7 percent.

Data on long-term prognosis after transcatheter intervention are more limited [35,41,85,106]. In studies reporting outcomes beyond 18 months after stent placement, most patients had durable improvement in the coarctation gradient, with 85 to 90 percent of patients maintaining a gradient of <15 mmHg [41,106]. Blood pressure remained <95^th percentile in 75 percent of patients. Approximately one-quarter to one-third of patients required antihypertensive medication. Reintervention was required in 10 to 20 percent of patients. In a report of intermediate outcomes in patients enrolled in the Coarctation of the Aorta Stent Trial I (COAST), five-year freedom from reintervention was 75 percent [41]. In a large multicenter registry study of 302 patients who underwent stent placement for CoA, 12 percent required reintervention, of which 36 percent were unplanned and 64 percent were performed as part of a planned staged procedure or to account for somatic growth [106].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Congenital heart disease in infants and children".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient education" and the keyword[s] of interest.)

●Basics topics (see "Patient education: Aortic coarctation in adults (The Basics)" and "Patient education: Aortic coarctation in children (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Management approach – Our approach to management of coarctation of the aorta (CoA) is as follows (see 'Management approach' above):

•Indications for intervention – We recommend intervention for patients with clinically significant CoA as defined by one or more of the following (Grade 1B):

-Neonates with critical coarctation (ie, dependent on ductal patency for survival) (see 'Neonates with critical coarctation' above)

-Infants, children, and adults with CoA gradient >20 mmHg, radiologic evidence of collateral flow, or hypertension or heart failure attributable to CoA (see 'Children and adults with noncritical presentation' above)

•Choice of intervention – Surgery and catheter-based intervention (ie, balloon angioplasty with or without stent placement) are both reasonable options to correct discrete CoA and should be performed in early childhood for optimal results and survival. In our centers, the following approach for repair of discrete CoA is based on the age of the patient and the morphology of the underlying lesion (see 'Management approach' above):

-For infants with CoA who are <4 months old, we suggest surgical CoA repair rather than catheter-based intervention (Grade 2C). Neonates with critical CoA typically require medical therapy to stabilize their condition prior to surgical repair (eg, infusion of prostaglandin E1 [alprostadil] to maintain ductal patency, inotropic support, and other supportive measures). Surgical repair is preferred over balloon angioplasty in neonates and young infants because it is associated with a lower risk for reintervention. In addition, arch hypoplasia is commonly present in neonatal and infantile CoA, and balloon angioplasty is unlikely to be successful in this setting. (See 'Neonates with critical coarctation' above and 'Surgery' above.)

-For infants with CoA who are ≥4 months old and for children who weigh <25 kg, the decision regarding balloon angioplasty versus surgical repair is determined by the expertise of the center and the underlying morphology of the coarctation. In our center, balloon angioplasty is the preferred procedure if the lesion is discrete and there is no evidence of arch hypoplasia. However, surgical repair continues to be the preferred approach in many centers worldwide for patients <5 years old. In patients with complex coarctation anatomy or underlying genetic disorder (eg, Turner syndrome), the decision to perform balloon angioplasty versus surgical repair is made on a case-by-case basis by the multidisciplinary team. (See 'Infants and young children' above.)

-For patients with discrete CoA who weigh ≥25 kg (ie, large enough to allow use of an adult-size stent), we suggest transcatheter intervention with stent placement rather than surgical repair or balloon angioplasty without stent placement (Grade 2C). For patients with suboptimal anatomy (eg, vessel tortuosity and/or transverse arch hypoplasia), the decision to perform stent placement versus surgical correction is made on a case-by-case basis by the multidisciplinary team. (See 'Older children and adults' above.)

•Special considerations during pregnancy – Individuals with CoA and associated lesions (eg, bicuspid aortic valve, aortic stenosis, ascending aorta dilation) who are trying to conceive should be evaluated by an adult congenital heart disease (CHD) specialist and should receive appropriate preconception counseling. Pregnancy increases the risk of aneurysm, dissection, and intracerebral hemorrhage both in women who have undergone intervention for CoA and those who have not. In addition, the risk of maternal hypertension, preeclampsia, and premature birth is greater in pregnant individuals with CoA compared with the general obstetrical population. Blood pressure control is an important aspect of pregnancy management in individuals with CoA. A reasonable blood pressure goal during pregnancy is 120 to 140/80 to 90 mmHg, although a lower target may be warranted if there is ascending or other aortic aneurysmal disease. If hypertension is not adequately controlled with medical management, the decision to perform surgical or transcatheter intervention during pregnancy is made on a case-by-case basis by a multispecialty team. (See 'Pregnancy' above and "Treatment of hypertension in pregnant and postpartum patients".)

●Long-term complications – Complications following intervention (either surgical or catheter-based) include (see 'Long-term cardiovascular complications' above):

•Systemic hypertension (see 'Systemic hypertension' above)

•Recoarctation(see 'Recoarctation' above)

•Aortic aneurysm (image 2 and image 3 and image 5) (see 'Aortic aneurysm, dissection, and rupture' above)

•Aortic dissection (see 'Aortic aneurysm, dissection, and rupture' above)

In addition, many patients with CoA have concomitant bicuspid aortic valve with associated risk of disease of the ascending aorta, which requires monitoring to assess for the need for intervention. (See "Bicuspid aortic valve: General management in adults".)

●Management of recoarctation – For patients with recoarctation with a gradient of >20 mmHg, imaging evidence of collateral circulation, or development of hypertension as a consequence of recoarctation, we suggest transcatheter intervention (ie, balloon angioplasty with or without stent placement) rather than surgical repair or no intervention (Grade 2C). Surgical repair of recoarctation is reserved for patients with confounding features (eg, long coarctation segment or concomitant arch hypoplasia). (See 'Management of recoarctation' above.)

●Follow-up – Patients with either uncorrected or corrected CoA should receive regular cardiovascular follow-up care, including (see 'Monitoring' above):

•Blood pressure should be measured at all visits. If hypertension is present, it should be treated aggressively, and the presence of recoarctation should be excluded by imaging studies. (See "Nonemergent treatment of hypertension in children and adolescents" and "Overview of hypertension in adults".)

•Aortic imaging is performed periodically following intervention to detect potential complications such as recoarctation or aneurysm. (See 'Imaging' above.)

•Echocardiography is performed periodically to assess bicuspid aortic valve function, ascending aorta, and ventricular function.

•Exercise testing may be warranted if there are concerns about blood pressure control or if the patient wishes to participate in competitive sports.

•Neuroimaging may be considered to evaluate for intracranial aneurysms.

•Preparticipation evaluation for patients who wish to participate in competitive sports (this includes a clinical evaluation, electrocardiogram, exercise testing, and cardiac/aortic imaging). (See 'Physical activity' above.)

●Prognosis – Ten-year survival following CoA repair is >90 percent; however, long-term survival is decreased compared that of with the general population. Operative and procedure-related mortality are rare (<1 percent of cases). Causes of late mortality include coronary artery disease, sudden death, heart failure, cerebrovascular accident, and ruptured aortic aneurysm. Older age (≥20 years) at initial repair and preoperative hypertension are risk factors for mortality. (See 'Prognosis' above.)

ACKNOWLEDGMENTS — The UpToDate editorial staff acknowledges Brojendra N Agarwala, MD, and QiLing Cao, MD, who contributed to an earlier version of this topic review.