Prenatal diagnosis of CoA is extremely imprecise even with the most advanced imaging; the following points should be kept in mind to be able to differentiate CoA in the fetal period:

During antenatal assessments, we should always consider the potential chance for hypoplasia of left heart structures accompanied with stenosis in the aortic isthmus in fetal period, mandating serial assessments especially using sequential echocardiography:

Some of the above echo findings are more easily diagnosed during early pregnancy especially when performed as serial Z scores; while in late pregnancy, the diagnosis is really much more difficult mandating serial assessments especially using sequential echocardiography (Allan et al. 1987, 1988, 1989; De Smedt et al. 1987; Hornberger et al. 1994; Sharland et al. 1994; Kaine et al. 1996; Eapen et al. 1998; Franklin et al. 2002; Dodge-Khatami et al. 2005; Head et al. 2005; Rosenthal 2005; Matsui et al. 2008; Axt-Fliedner et al. 2009; Weisse 2011).

The clinical presentation of the neonates with CoA includes the signs and symptoms of low cardiac output; if CoA is a severe type lesion, especially when superimposed by closure of ductus arteriosus, clinical presentation of shock could be seen; these patients would be diagnosed as critical neonatal CoA due to a severe juxta-ductal CoA, clinically presenting with different features of heart failure as the following findings:

If the infant with CoA passes the critical neonatal CoA (i.e., the ductus is not closed, and the patients receive appropriate medical treatment), collateral flow may begin to develop; however, during later infancy, nonspecific features of failure to thrive could be the predominant clinical feature along with cachexia and poor feeding. In almost all situations, weak lower limb pulses could be added to the diagnostic criteria (Rosenthal 2005; Kenny and Hijazi 2011).

Often, the more subtle forms of CoA might be undetected until later years of life; however, older children and adolescents with CoA usually present with these findings:

If adequate collateral vessels form during the early years, the above signs and symptoms could be very subtle, and the only remaining feature would be increased blood pressure in the upper extremity which is two standard deviations above normal limit for sex and age accompanied with delayed femoral pulse compared with proximal arterial sites. Also, increased blood flow through the intercostal arteries as the main collaterals would affect the rib margins and result in a typical feature in chest X-ray (CXR) referred to as “rib notching.” Another diagnostic characteristic in chest X-ray is the border of the constricted aorta in the isthmal aortic region called “reverse 3 sign”; it is clear that “rib notching” on CXR is the result of increased intercostal artery flow so it would not be seen in neonates and younger children. These findings are seen in older children and adults and confirmed by transthoracic echocardiography (TTE), CT scanning, MR imaging, MR angiography (MRA), and aortic angiography. Detailed imaging prior to surgical- or catheter-based intervention is mandatory (Gotzsche et al. 1994; Connolly et al. 2003; Matsunaga et al. 2003; Rosenthal 2005; Secchi et al. 2009; Akdemir et al. 2010; De Caro et al. 2010; Feltes et al. 2011; Kenny and Hijazi 2011; Pedersen et al. 2011; Baykan et al. 2014; Cook et al. 2013; Khavandi et al. 2013; Ringel et al. 2013; Vergales et al. 2013; Eckroth-Bernard et al. 2014; Lee and d’Udekem 2014; Tong et al. 2014).

If CoA is not diagnosed during the infancy period, the ductus would be closed by time, and the aorta would become enlarged enough to produce the large-sized aortic segment, typical of the adulthood CoA. However, in this underlying pathology, if undiagnosed and untreated, more than 80 % of patients die before the age of 50. Other associated anomalies seen in adult CoA could be somewhat similar to the lower age range, including the presence of BAV or very rarely left-sided obstructive lesions.

Currently, the adult CoA patient populations comprised of those who have undergone surgical repair, balloon angioplasty, stenting, or a combination of the three methods, with or without residual recurrence of CoA. However, even in those without residual CoA, the clinician should always be aware of the possibility of cardiovascular problems including but not limited to the following:

In 1944 “the first surgical correction of CoA” was first reported. Often, surgical correction of CoA is performed through a posterolateral thoracotomy and has been regarded for many decades as “the gold standard treatment for CoA.” However, in the current era of interventional treatments, there is an ever-growing controversy regarding “the best treatment for CoA” with alternative options (mainly interventional methods including balloon dilatation or stenting) becoming much more popular. As catheter and stent technologies have matured, more centers are choosing stenting or balloon angioplasty as the primary choice for treatment of CoA not only in recurrent CoA but in native CoA. In recent years, with the development of covered stents and smaller delivery systems, many centers have reported improved outcomes and fewer long-term complications as compared to surgical cohorts. Despite this, stenting has not yet become the standard first choice, and some controversies remain yet. In 2011, the American Heart Association released its scientific statement:

for native coarctation of the aorta, surgical repair (extended resection with an end-to-end anastomosis) remains the gold standard” while “balloon angioplasty with or without stent implantation” is considered as an alternative option with less invasiveness; though it should be kept in mind that “transcatheter treatment does not necessarily replace surgical management

However, The Cochrane Database systematic review published in 2012 has declared that “there is insufficient evidence with regards to the best treatment for coarctation of the thoracic aorta” (Mahadevan and Mullen 2004; Forbes et al. 2007a; Turner and Gaines 2007; Botta et al. 2009; Egan and Holzer 2009; Holzer et al. 2010; Feltes et al. 2011; Forbes et al. 2011; Padua et al. 2012; Hijazi and Kenny 2014; Sohrabi et al. 2014).

Some clinical notes should be considered in patients undergoing surgical correction of CoA:

The relatively high rate of postoperative events in CoA patients underscores pathologic basis of the disease: “surgical correction of CoA only resects the local anatomic isthmus but not the underlying vascular impairment generating the disease.”

Though isolated repair of CoA is associated with favorable results, all surgical approaches are associated with a chance of restenosis, i.e., re-CoA (with a rate of about 20–40 %); advanced microsurgical techniques accompanied with vigorous and sophisticated approximation of the two ends of the anastomosis may decrease restenosis rate, while weight and age at the time of operation could affect the chance of re-CoA. Fortunately, restenosis is often nonlethal and could be treated by surgical approach or by catheter-based intervention; each of these two methods has their merits and risks (Connors et al. 1975; Backer et al. 1995; Bouchart et al. 2000; Azakie et al. 2005; Suarez de Lezo et al. 2005; Abbruzzese and Aidala 2007; Hijazi and Awad 2008; Kuroczynski et al. 2008; Botta et al. 2009; Vohra et al. 2009; Kische et al. 2010; Feltes et al. 2011; Luijendijk et al. 2012; Pedersen 2012).

For many years, surgical repair of CoA was considered the only available definitive therapy for CoA patients; however, in 1979 Sos et al. collected coarcted segments of the aorta from postmortem specimens and demonstrated the ability to dilate the tissue; this finding was the first step in application of definitive nonsurgical therapies (Sos et al. 1979). Afterward, Singer and colleagues reported successful dilatation of CoA in a 42-day-old infant; it is interesting that this first case of balloon dilatation was done after unsuccessful surgical repair leading to restenosis (Singer et al. 1982).

Indications for balloon dilatation of CoA are the same as surgical repair and include:

However, based on the current evidence, the following could be considered as the main applications of balloon dilatation angioplasty for CoA patients:

A considerable number of studies have demonstrated balloon dilatation angioplasty as a good alternative among the first line of corrective treatments in order to remove isthmal stricture in discrete CoA, neonates, adolescents, and adults, with excellent long-term outcome. Also, the results of balloon dilatation are safe and effective, even years after primary therapy, i.e., during later clinical follow-up. However, other studies have shown that balloon dilatation of CoA causes neointimal proliferation of undifferentiated smooth muscle cells into the aortic lumen, causing restenosis after primary balloon dilatation. One of the issues with balloon dilatation which has been controversial is its application for native CoA especially when compared with other methods regarding the risk of aneurysm formation. Current evidence has not clearly resolved this controversy (Fawzy et al. 1992, 1997, 1999, 2004, 2008, Takahashi et al. 2000; Hassan et al. 2007a, b; Hijazi and Awad 2008; Rothman et al. 2010; Feltes et al. 2011).

Complications of balloon angioplasty are similar to stent dilatation, discussed more in the next section under “stent dilatation”; they include the following in brief:

Stenting includes transcatheter insertion of stents in order to implant and dilate the stent at the location of the isthmal stricture; this method has been described for the first time in 1991 with good results and is now considered as a first-line therapy in most adolescents and adults and those with restenosis. Short-term results of stenting in CoA (i.e., decreasing the gradient across the isthmal stricture) and also long-term outcomes (especially the incidence of post dilatation aneurysm formation and restenosis) are promising. Improvements in stent technology have decreased the age of stenting to smaller patients to as young as 3 months with these patients requiring multiple re-dilations to accommodate a growing patient’s aorta. Success rate of stenting in CoA patients is more than 95 % with an immediate drop in systolic blood pressure gradient and increase in aortic diameter. Stenting for CoA has been compared with both balloon dilatation and surgical repair of CoA. Stenting leads to favorable clinical outcomes regarding relief of hypertension especially after surgical correction of CoA. Interestingly, some patients with no arm–leg gradient at rest may develop a blood pressure gradient with exercise (the so-called posttreatment exercise-induced hypertension “EIH”); however, CoA patients treated with stenting do not often experience this problem. Also, covered stents have been introduced for CoA patients as safer devices in order to prevent unwanted complications of bare metal stents including aortic wall trauma, aneurysm formation, and migration of stent; however, the current available evidence is in favor of equal safety and efficiency of the two types of stents though most experienced operators opt for covered stents in high-risk patients. Those include patients including those with underlying aortic aneurysm, patients with nearly occluded aorta and aortic atresia, patients with age of 40 or more, and patients with Turner syndrome.

The complications of stenting in CoA patients are infrequent, i.e., the chance for acute complications, especially rupture of the aorta, is very low (about 2 %); while the rate of long-term complications is relatively less than other therapeutic options. The rate of aneurysm formation is 5–10 % and the rate of restenosis about 10 % or less than that; however, sophisticated care is needed to detect and, if necessary, treat any untoward complication; these include:

Preoperative care depends mainly on the age of diagnosis; if the patient is diagnosed in the neonatal or infantile period, the main goal in preoperative care would be to stabilize hemodynamic status, correct the acidotic milieu of the under-perfused organs, and improve the underlying failing heart as much as possible; while in adolescent and adult CoA patients, we mainly aim to control blood pressure especially in the upper trunk and upper extremity.

Anesthesia induction and maintenance

Either intravenous or inhalational anesthesia agents or a combination of them could be used for induction; however, extreme caution should be exerted to prevent blood pressure drop after induction in patients with ductal-dependent distal flow. Also, for maintenance of anesthesia, both intravenous and inhalational agents could be used. Thoracic paravertebral block could have benefit both for intraoperative and postoperative analgesia; however, there is always the risk for masking signs of early postoperative paraplegia by the block; the same could be correct for thoracic epidural analgesia (Turkoz et al. 2013)

Lung ventilation should be kept at normocapnia to prevent potential cerebral vasoconstriction and reduce the risk of cord ischemia. One lung ventilation management is another challenge for anesthesiologist especially in the very young patients.