Coarctation of the aorta (CoA) is a relatively common congenital cardiac defect often causing few symptoms and therefore can be challenging to diagnose. The hallmark finding on physical examination is upper extremity hypertension, and for this reason, CoA should be considered in any young hypertensive patient, justifying measurement of lower extremity blood pressure at least once in these individuals. The presence of a significant pressure gradient between the arms and legs is highly suggestive of the diagnosis. Early diagnosis and treatment are important as long-term data consistently demonstrate that patients with CoA have a reduced life expectancy and increased risk of cardiovascular complications. Surgical repair has traditionally been the mainstay of therapy for correction, although advances in endovascular technology with covered stents or stent grafts permit nonsurgical approaches for the management of older children and adults with native CoA and complications. Persistent hypertension and vascular dysfunction can lead to an increased risk of coronary disease, which, remains the greatest cause of long-term mortality. Thus, blood pressure control and periodic reassessment with transthoracic echocardiography and three-dimensional imaging (computed tomography or cardiac magnetic resonance) for should be performed regularly as cardiovascular complications may occur decades after the intervention.

The human X and Y chromosomes evolved from a pair of autosomes approximately 180 million years ago. Despite their shared evolutionary origin, extensive genetic decay has resulted in the human Y chromosome losing 97% of its ancestral genes while gene content and order remain highly conserved on the X chromosome. Five 'stratification' events, most likely inversions, reduced the Y chromosome's ability to recombine with the X chromosome across the majority of its length and subjected its genes to the erosive forces associated with reduced recombination. The remaining functional genes are ubiquitously expressed, functionally coherent, dosage-sensitive genes, or have evolved male-specific functionality. It is unknown, however, whether functional specialization is a degenerative phenomenon unique to sex chromosomes, or if it conveys a potential selective advantage aside from sexual antagonism. We examined the evolution of mammalian orthologs to determine if the selective forces that led to the degeneration of the Y chromosome are unique in the genome. The results of our study suggest these forces are not exclusive to the Y chromosome, and chromosomal degeneration may have occurred throughout our evolutionary history. The reduction of recombination could additionally result in rapid fixation through isolation of specialized functions resulting in a cost-benefit relationship during times of intense selective pressure.

One indication for intervention in coarctation of the aorta is a peak-to-peak gradient >20 mmHg. Gradients may be masked in patients under general anaesthesia and may be higher during exercise. Isoproterenol was given during cardiac catheterisation to simulate a more active physiologic state.

We aimed to describe the haemodynamic effects of isoproterenol in patients with coarctation and the impact of intervention on the elicited gradients.

A retrospective study was performed on two-ventricle patients who underwent cardiac catheterisation for coarctation with isoproterenol testing.

25 patients received isoproterenol before and after intervention. With isoproterenol, the mean diastolic (p=0.0015) and mean arterial (p=0.0065) blood pressures proximal to the coarctation decreased significantly. The mean systolic, diastolic, and mean arterial blood pressures distal to the coarctation decreased significantly (p20 mmHg. Post intervention, the median gradient decreased to 2 (0-29) mmHg, versus baseline, p=0.005, and with isoproterenol it decreased to 8 (0-27) mmHg, versus pre-intervention isoproterenol, p<0.0001. There were significant improvements in the gradients by Doppler (<0.0001) and by blood pressure cuff (p=0.0313). The gradients on isoproterenol best correlated with gradients by blood pressure cuff in the awake state (R2=0.76, p<0.0001).

Isoproterenol can be a useful tool to assess the significance of a coarctation and the effectiveness of an intervention. Percutaneous interventions can effectively reduce the gradients elicited by isoproterenol.

Aortic coarctation is one of the most common congenital cardiac pathologies. Repair of native aortic coarctation is nowadays a common and safe procedure. However, late complications, including re-coarctation and aneurysm formation, are not uncommon. The incidence of these complications is dependent on the type of the initial operation. Both endovascular and conventional open repair play important roles in the treatment of late complications after previous coarctation repair. This article will review the incidence of late complications after coarctation repair and will discuss the treatment options for redo coarctation repair in adult patients.

Coarctation of the aorta is an uncommon cause of treatment-resistant hypertension in adults. It is typically detected and treated in infancy or childhood with surgical or endovascular procedures. Most cases of recurrence of coarctation after repair occur in childhood or early adulthood; recurrence in older persons (>70 years) has rarely been reported. A 73-year-old woman was referred to us for the management of treatment-resistant hypertension accompanied by symptoms of claudication and headaches, which had resulted in multiple emergency room visits. Of note, 58 years earlier, a graft from the left subclavian artery had been used to bypass an aortic coarctation. During a hospitalization for severe hypertension accompanied by acute kidney injury and heart failure, diagnostic angiography revealed a complete thrombotic occlusion of the left subclavian-artery-to-descending-aorta bypass graft and a tight coarctation in the descending thoracic aorta. Balloon angioplasty and stenting across the coarctation was only transiently effective; subsequently, an ascending-to-descending graft was placed distal to the coarctation, and within a few days, the blood pressure levels and claudication improved markedly. This case demonstrates that hypertension specialists should suspect the possibility of recurrence of a coarctation in older patients who present with resistant hypertension and have a remote history of coarctation repair. Although such late recurrences are not common, as illustrated in our patient, surgical intervention may contribute to significant improvement in blood pressure control and prevent future complications.

Endovascular stenting is a recognised treatment strategy for aortic coarctation (CoA) in adults. We assessed systemic hypertension control and the need for antihypertensive therapy after CoA stenting in adults.

Data were collected prospectively on 54 patients (36 men; mean age: 34 ± 16 years) who underwent endovascular stenting for CoA over a 7-year period. Five patients were excluded as they did not attend follow-up appointments. Patients underwent clinical examination, including right arm systolic blood pressure (SBP) and 24-hour ambulatory blood pressure monitoring at baseline, 6-12 weeks and 9-12 months.

There was a significant fall in mean peak-to-peak systolic gradient (PG) across the CoA after stenting (26 ± 11 mmHg vs. 5 ± 4 mmHg; P<0.01). There were successive reductions in right arm SBP and ambulatory SBP at baseline, 6-12 weeks and 9-12 months post-procedure (right arm: 155 ± 18 mmHg vs. 137 ± 17 mmHg vs. 142 ± 16 mmHg, respectively; all P-values <0.01; ambulatory: 142 ± 14 mmHg vs. 132 ± 16 mmHg vs. 131 ± 15 mmHg, respectively; all P-values <0.01). Twenty-four patients had severe CoA (PG >25 mmHg before stenting); baseline SBP was significantly higher in severe versus non-severe patients (160 mmHg vs. 148 mmHg; P=0.02). The absolute reduction in PG after stenting was significantly higher in the severe group (31 ± 7 mmHg vs. 14 ± 5 mmHg; P<0.0001), but there was no significant difference in SBP between groups at 6-12 weeks (141 mmHg vs. 135 mmHg; P=0.21) or 9-12 months (139 mmHg vs. 139 mmHg; P=0.96).

Endovascular stenting of CoA results in a significant reduction in SBP at 6-12 weeks, which is sustained at 9-12 months, with similar outcomes in severe and non-severe CoA groups.

Coarctation of the aorta (CoA) accounts for 5% to 8% of all congenital heart defects. With all forms of interventions for native CoA, repeat intervention may be required due to restenosis and/or aneurysm formation. Restenosis rates vary from 5% to 24% and are higher in infants and children and in those with arch hypoplasia. Although repeat surgery can be done for recurrent CoA, guidelines from a number of professional societies have recommended balloon angioplasty with or without stenting as the preferred intervention for patients with isolated recoarctation. For infants and young children with recurrent coarctation, balloon angioplasty has been shown to be safe and effective with low incidence of complications. However, the rates of restenosis and reinterventions are high with balloon angioplasty alone. Endovascular stent placement is indicated, either electively in adults or as a bailout procedure in those who develop a complication such as dissection or intimal tear after balloon angioplasty. Conventionally bare metal stents are used; these can be dilated later if required. Covered stents, introduced more recently, are best reserved for those who have aneurysm at the site of previous repair or who develop a complication such as aortic wall perforation or tear. Stents produce complete abolition of gradients across the coarct segment in a majority of cases with good opening of the lumen on angiography. The long-term results are better than that of balloon angioplasty alone, with very low rates of restenosis. However, endovascular stenting is a technically demanding procedure and can be associated with serious complications rarely.

Balloon angioplasty (BA) is an important treatment option for coarctation of the aorta. The congenital cardiovascular interventional study consortium (CCISC) represents a multi-institutional and multi-national effort to prospectively investigate congenital cardiac interventions. A prospective observational analysis of the efficacy and safety of balloon aortic angioplasty was conducted.

Data were collected prospectively from 36 CCISC sites from 2004 to 2012. One hundred and thirty patients underwent BA for native (n = 76) and recurrent (n = 54) coarctation. Acute, short-term, and intermediate outcomes are described for BA performed in the setting of native and recurrent coarctation of the aorta. Outcome measures included residual upper to lower extremity blood pressure gradient (ULG), use of antihypertensive medications, aortic wall injury, reobstruction, and need for reintervention.

There was no procedural mortality. Acutely in native and recurrent coarctation, BA achieved an ULG less than 15 mm Hg in 73-80% and to less than 10 mm Hg in 54-68% of patients, respectively. At intermediate follow-up, ULG further improved, particularly for those who underwent initial reintervention for recurrent coarctation. No significant differences in aortic wall complications were seen and intervention free survival was similar for both groups. Following angioplasty, there was no significant difference in aortic wall complications; however follow up integrated imaging decreased over time.

BA is a safe and effective treatment for coarctation of the aorta acutely and at intermediate term. Although aortic injury occurred in patients with both native and recurrent coarctation, at intermediate follow-up, aneurysm was noted more often in those with initial intervention for native coarctation.

The exercise test is considered useful in selecting high-risk patients with repaired coarctation of the aorta (CoA), but it is difficult to obtain the cooperation of pediatric patients. The present study determines the feasibility of the isoproterenol stress test (IST) among pediatric patients with CoA.

Thirteen patients with repaired or mild preoperative CoA aged 1-207 (median 13) months underwent 16 IST during cardiac catheterization. Peak-to-peak pressure gradients (PG) over the coarctation site were measured at baseline and at IST. Balloon angioplasty (BAP) was applied to patients with significant stenosis on angiography.

The PG between the ascending and the descending aorta was significantly higher at IST than at baseline (20.5 ± 11.5 vs 5.6 ± 3.9 mmHg, P < 0.0001). Heart rate, the systolic blood pressure measured at the ascending aorta, and pulse pressure were all significantly higher at IST than at baseline. The PG at IST decreased >10 mmHg in three of six patients after BAP.

Significant pressure gradients over the coarctation site develop at IST in pediatric patients with repaired CoA or in preoperative patients with mild coarctation.

To evaluate the mid and long-term prognosis after stenting of native or recurrent CoA, we studied the cardiovascular parameters in the follow-up period up to 13 years.

Between 1993 and 2006, 68 patients underwent stent implantation for aortic coarctation (average age 25.5 years, range 5.7-65 years, average weight 65.5 kg, range 32-122 kg). Forty-six (68%) patients were aged >17 years. Stenting was performed for native coarctation in 41 and for recurrent coarctation in 27 patients, in 23 (34%) patients with a covered stent. Redilation was carried out in 26 (38%) patients. The invasive systolic gradient decreased from mean (±SD) 25 (±15) mm Hg to 5 (±5) mm Hg (P < 0.0005). The descending aorta pressure increased from 80 (±15) mm Hg to 101 (±18) mm Hg. The systolic right arm blood pressure decreased from a mean of 153 (±24) mm Hg to 129 (±18) mm Hg (P < 0.0005). Complications like small dissections were rare. Follow-up (6 days to 13 years, mean 41 months) was available in 66 patients, in 23 after reintervention at a mean of 71 months, range of 8 months to 10.3 years. Fifty-one percent remained clinically hypertensive.

Stenting of aortic coarctation gives good medium-term results. Frequent reintervention relate to deliberately under-dilating stents during the initial procedure. The reintervention rate has reduced since the introduction of covered stents.

Background Traditionally, high-profile/high-pressure balloons have been used for angioplasties, whereas low-profile/low-pressure balloons have been used for valvuloplasties. High-profile balloons require larger introducing sheaths, which can be a limiting factor for percutaneous catheter interventions in infants. This report aims to report the author's experience with the efficacy of low-profile balloons using smaller introducing sheaths for coarctation balloon angioplasty in infants. Methods From April 2004 to April 2008, 15 infants, representing both native coarctation and recoarctation indications, underwent coarctation balloon angioplasty and were retrospectively reviewed. The arterial access was achieved using 4-Fr (Cook) introducing sheaths and Tyshak (NuMED, Hallenweg-Netherlands) balloons 5 to 8 mm in diameter for coarctation angioplasty in the study group. Results In this study, 15 infants (7 with native coarctation and 8 with postoperative recoarctation) underwent balloon angioplasty. These infants ranged in age from 2 to 9 months (median, 4 months) and in weight from 3.5 to 10.8 kg (median, 5.7 kg). The peak-to-peak coarctation gradient was reduced from 46.2 +/- 28 mmHg before angioplasty to 10 +/- 8 mmHg afterward (p = 0.001). The angiographic diameter of the coarctation segment was increased from 2.4 +/- 1.0 mm before angioplasty to 5 +/- 0.8 mm afterward (p = 0.001). There were no immediate major or minor complications. During a follow-up period up to 48 months, only one patient from the native coarctation group experienced recoarctation and underwent successful reballooning, and none of the patients experienced aneurysms. Conclusion This study shows that the use of low-profile/low-pressure balloons is an effective treatment for infants. Furthermore, low-profile balloons required smaller introducing sheaths, which provides a clear advantage of minimizing vascular complications with coarctation ballooning in younger infants.

Intravascular or intracardiac stenoses occur in many forms of congenital heart disease (CHD). Therefore, the implantation of stents has become an accepted interventional procedure for stenotic lesions in pediatric cardiology. Furthermore, stents are know to be used to exclude vessel aneurysm or to ensure patency of existing or newly created intracardiac communications. With the further refinement of the first generation of devices, a variety of "modern" stents with different design characteristics have evolved. Despite the tremendous technical improvement over the last 20 years, the "ideal stent" has not yet been developed. Therefore, the pediatric interventionalist has to decide which stent is suitable for each lesion. On this basis, currently available stents are discussed in regard to their advantages and disadvantages for common application in CHD. New concepts and designs developed to overcome some of the existing problems, like the failure of adaptation to somatic growth, are presented. Thus, in the future, biodegradable or growth stents might replace the currently used generation of stents. This might truly lead to widening indications for the use of stents in the treatment of CHD.

To assess the efficacy, safety, and long-term results of the balloon angioplasty of recoarctation.

The angioplasty was performed in 99 consecutive patients aged 36 days to 32.6 years (median 268 days). Recoarctation to descending aorta diameter ratio increased from 0.44 (0.35/0.50) to 0.66 (0.57/0.77), P < 0.001. Systolic gradient was reduced from 34.0 (26.0/44.75) to 15.0 (8.25/27.0) mmHg, P < 0.001. In seven patients (7.1%) the procedure was ineffective. One patient (1%) with heart failure died within 24 h after a successful angioplasty and in another (1%) an intimal abruption necessitated surgical revision. The follow-up ranged up to 20.7 years (median 8.1 years). Actuarial probability of survival 20.7 years after the procedure was 0.91, and of reintervention-free survival was 0.44. Older age at the angioplasty was associated with a higher incidence of reinterventions (hazard ratio 1.057; 95% confidence interval 1.012-1.103; P = 0.010). The type of surgery and the recoarctation anatomy did not influence the outcome. In 69 patients aneurysm formation was studied by high-sensitive methods with only one positive finding per 462 patient-years.

Angioplasty is safe and effective regardless of the type of surgery used and the recoarctation anatomy. Older age at the angioplasty is associated with a higher incidence of reinterventions.

Obstructive lesions in the aortic arch are comprised of discrete coarctation, tubular hypoplasia and interruption. This review discusses the anatomy of the lesions relevant to interventional treatment. Catheter intervention, using not only balloon angioplasty but also stent implantation for coarctation, has been developed over the past couple of decades as an alternative treatment to surgery. Several studies have reported long-term outcome and the benefits of surgery and catheter intervention for treating obstructive lesions in the aortic arch but more studies are needed for comparable evaluations. The development of imaging and further improvement of surgical and catheter intervention, such as hybrid intervention or new devices, will help in removing the obstruction safely.

Coarctation of the aorta may present in infants, children, or adults, and it requires treatment to prevent serious morbidity and mortality. Recent advances in equipment and a growing collective experience have made placement of balloon-expandable stents a safe and effective alternative to surgery or angioplasty in a growing range of patients. This review seeks to provide a working aid for stenting of coarctation of the aorta, based on the techniques and technical considerations in practice at our institution. Between 1989 and 2005, the Congenital Cardiovascular Interventional Study Consortium (CCISC), a consortium of 17 centers, of which our institution is the largest contributor, performed 588 stent placements for coarctation of the aorta. Of the 588 procedures, 580 (98.6%) were successful, as defined by reduction of the gradient to less than 20 mm Hg or increase of the ratio of the diameter of the coarctation area (CoA) to the diameter of the descending aorta (DAo) to at least 0.8. There were a total of 84 complications occurring in 69/588 (11.7%) cases. The most common significant complications were femoral access vessel related 15/588 (2.6%), aneurysm formation 13/588 (2.2%), aortic dissection 9/588 (1.5%), and cerebrovascular accident 6/588 (1.0%). There were two procedure-related deaths (0.3%) recorded in the 16-year period. Many of these significant complications occurred in the same patients. Balloon-expandable stents should be considered a safe and very effective treatment modality in a significant subset of patients with coarctation of the aorta.

To investigate the utility of MR and X-ray imaging for characterizing aortic coarctation and flow, and guiding the endovascular catheter to place a stent to repair the coarctation.

The descending aorta in eight dogs was looped with elastic band and tightened distal to the subclavian artery. Balanced fast field echo (bFFE) and velocity-encoded cine (VEC) MRI sequences were used for device tracking and measuring aortic flow. A T1-weighted fast-field echo sequence (T1-FFE) was used to visualize the coarctation and roadmap the aorta. Nitinol stents were guided by a nitinol guidewire and placed under MR guidance.

Aortic coarctation was visible on MR and X-ray imaging. The procedure success rate was 88%. VEC MRI measured the changes in aortic flow (baseline = 1.3 +/- 0.2, coarctation = 0.2 +/- 0.02, and stent placement = 0.8 +/- 0.1 liters/minute). A significant reduction in iliac blood pressure was measured after coarctation, but it was reversed by stent placement. The stent lumen was visible on X-ray fluoroscopy, but not on MRI.

Stent deployment to repair aortic coarctation is feasible under MR guidance. The combined use of MR and X-ray imaging is effective for anatomic and functional evaluation of aortic coarctation dilation, which may be crucial for optimal therapy.

This study sought to evaluate the use of covered Cheatham-platinum (CP) stents in the treatment of aortic coarctation (CoA).

Aortic aneurysms and stent fractures have been encountered after surgical and transcatheter treatment for CoA. Covered stents have previously been used in the treatment of abdominal and thoracic aneurysms in adults. We implanted covered CP stents as a rescue treatment in patients with CoA aneurysms or previous stent-related complications and in patients at risk of developing complications because of complex CoA anatomy or advanced age.

Thirty-three covered CP stents were implanted in 30 patients; 16 patients had had previous procedures. The remaining patients had complex or near-atretic CoA.

The mean patient age and weight were 28 (+/-17.5) years (range 8 to 65 years), and 62 (+/-13) kg (range 28 to 86 kg), respectively. The systolic gradient across the CoA decreased from a mean (+/-SD) of 36 +/- 20 mm Hg before to a mean of 4 +/- 4 mm Hg after the procedure (p < 0.0001), and the diameter of the CoA increased from 6.4 +/- 3.8 mm to 17.1 +/- 3.1 mm (p < 0.0001). The follow-up period was up to 40 months (mean, 11 months). All stents were patent and in good position on computed tomography or magnetic resonance imaging performed three to six months later. In 43% of the patients antihypertensive medication was either decreased or stopped.

Covered CP stents may be used as the therapy of choice in patients with complications after CoA repairs, whereas they provide a safe alternative to conventional stenting in patients with severe and complex CoA lesions or advanced age.

The current study was designed to assess midterm results of stent implantation into the aorta for native and recurrent coarctation (CoA) in children and young adults.

Forty-three patients (native CoA, 8; female, 12) were treated with stent implantation at a median age of 16.8 years (range 7.9-44.8 years). Only stents dilatable to an adult size aorta were implanted. All but two patients with functionally univentricular hearts had arterial hypertension. Exercise tests, 24-h blood pressure, clinical examination, echocardiography, and elective catheterization were used to assess follow-up. The narrowed segment was widened significantly from a median of 8 to 12.4 mm (P < 0.0005). The peak-to-peak gradient between the ascending and the descending aorta was lowered significantly from a median of 22 mmHg to 1 mmHg (P < 0.0005). No major complications occurred. The systolic blood pressure at the right arm was lowered significantly (P < 0.0005) from 144 mmHg before stent implantation to 128 mmHg at the last visit. At a median follow-up of 30 months (3-72 months), 68% of all patients were classified to be normotensive.

Stent implantation for selected patients with recurrent and native CoA is safe and may effectively reduce the blood pressure gradient across the CoA site. We suggest using only stents dilatable to an adult size aorta. However, arterial hypertension persists in a significant number of the patients. Impaired elastic properties of the aorta may be the cause for this finding.

A 64-year-old female patient presented with heart failure with acute pulmonary edema due to severe aortic coarctation. After endotracheal intubation, aortogram was performed and revealed a severe luminal narrowing in the distal thoracic aorta with a peak systolic pressure gradient of 100 mm Hg across the lesion. Stent implantation was performed with 24*100 mm self-expandable Nitinol-S stent after predilation with 10*40 mm balloon. After stenting, the patient's symptom and sign of congestive heart failure were remarkably improved and able to remove endotracheal intubation tube, with no significant adverse cardiac events observed during a 1-year clinical follow-up.

We performed balloon angioplasty in three patients with postoperative coarctation in the transverse arch adjacent to the left common carotid artery. The age at arch reconstruction was 5, 6, and 2 days, while the interval between operation and balloon dilatation was 59 days, 87 days, and 12 months, respectively. Two balloons, one in the stenosis and the other in the left common carotid artery, were introduced over a wire sequentially and inflated simultaneously until the waist of the balloon in the arch disappeared. After balloon dilatation, a significant reduction in the peak-to-peak pressure gradient and an increase in vessel diameters were observed in all patients. Further growth of the transverse arch was documented at follow-up in two patients. No aneurysm has been detected in any patients. We believe that placing a protective balloon in the neck vessel increases safety during balloon dilatation of coarctation in the transverse arch.

Congenital heart defects are the most common birth defects and represent an increasing proportion of adolescent and adult patients followed by cardiologists. While many of these patients have undergone successful palliative or corrective surgery with excellent functional results, most of them still require careful follow-up. Further, even complex lesions may first be diagnosed in adolescence and adulthood. Therefore, cardiologists caring for adults need to become more familiar with these defects. Assessment of the patient with known or suspected congenital heart defects requires a careful history, physical examination, and noninvasive assessment. In addition, the catheterization laboratory remains a critical venue for diagnosis and, increasingly, therapy. Pressure measurements, oximetry, and angiography remain cornerstones of diagnosis in selected patients and a variety of interventional procedures have become viable therapeutic alternatives in both pre- and postoperative patients.

A retrospective study to review the experience of a single center with surgery for aortic coarctation over a period of 30 years (1970-1999).

Criteria for inclusion: (a) aortic coarctation, isolated or associated with congenital heart defect; (b) surgery between 1970 and 1999. Data recorded: (1) date of surgery; (2) age at surgery; (3) associated lesions; (4) surgical technique; (5) simultaneous surgical procedures; (6) early and late surgical results in term of: (a) deaths; (b) need for reoperation because of re-coarctation or other cardiac lesion; (c) residual/recurrent pressure gradient, evaluated at cuff/Doppler at rest; (d) systemic hypertension, requiring medical treatment.

One hundred and forty-one patients underwent surgery for aortic coarctation: 30 neonates, 29 infants, 45 children and 37 adults. Associated lesions were found in 8/37 (=21.6%) adults and in 73/104 (=70.1%) pediatric patients. There were no hospital deaths. During the follow-up there were one late death in the adults group (1/37=2.7%) and three late deaths in the pediatric group (3/104=2.9%), all unrelated with aortic coarctation. Re-operation because of re-coarctation occurred only in ten late survivors of the pediatric group (10/101=9.9%), 9/10 operated on before 1980 (P<0.00001). End-to-end anastomosis, enlarged to the aortic arch in neonates, was associated with the lowest incidence of re-coarctation (P<0.005). A significant (>20 mmHg at rest) pressure gradient was found in none of the adults, and in seven of the 91 pediatric patients (7/91=7.7%) late survivors. Three adults (3/36=8.3%) late survivors are on medical treatment to control systemic hypertension.

The long-term results of our retrospective study confirm that surgery has to be considered the gold standard for the treatment of aortic coarctation. The interventional angioplasty techniques have to provide long-term outcome at least similar to the results obtained with surgery.

The aim of this study was to evaluate the use of endovascular stents in native and recurrent coarctation of the aorta (CoA).

Stents have been used successfully in various locations. Their use in CoA can be an alternative to surgery or balloon angioplasty (BA).

Thirty-four patients with CoA (13 native and 21 re-coarctation after surgery or BA) with a mean age of 16 +/- 8 years (range 4 to 36 years) underwent attempted stent implantation between 1993 and 1999. Successful outcome was defined as peak systolic pressure gradient after stent implantation < 20 mm Hg.

Stents were implanted in 33/34 patients, and successful outcome occurred in 32/33 patients. Peak systolic pressure gradient decreased from 32 +/- 12 mm Hg to 4 +/- 11 mm Hg (p < 0.001). Coarctation site to descending aorta diameter ratio increased from 0.46 +/- 0.16 to 0.92 +/- 0.16 (p < 0.001). Two patients underwent successful stent re-dilation 16 and 21 months after initial implantation. Six patients (18%) developed complications, including two patients who underwent surgery. Follow-up for 29 +/- 17 months (range: 5 to 81 months) demonstrated no evidence of re-coarctation, aneurysm formation, stent displacement or fracture. Systolic blood pressure (SBP) decreased from 136 +/- 21 mm Hg before stent placement to 122 +/- 19 mm Hg at follow-up (p = 0.002). The SBP gradient decreased from 39 +/- 18 mm Hg to 4 +/- 6 mm Hg, and peak Doppler gradient decreased from 51 +/- 26 mm Hg to 13 +/- 11 mm Hg at follow-up (p < 0.001).

Intravascular stent placement for native and recurrent CoA has excellent results in the short and intermediate terms. Long-term outcome remains to be evaluated.

The purpose of the present study was to assess the usefulness of balloon expandable Palmaz intravascular stents in the transcatheter treatment of children and young adults with native and recurrent coarctation of the aorta, and to develop an improved intravascular stent and balloon delivery catheter specifically developed for vascular obstructions associated with congenital heart disease, including coarctation of the aorta. Twenty-one patients, 8 native and 13 recurrent coarctations, were successfully treated with the Palmaz stent. However, limitations and disadvantages in stent design and the single balloon delivery system were uncovered. Therefore, the NuMED CP stent and BIB delivery catheter were developed and used to treat 25 patients with native (17) and recurrent (8) coarctation successfully. Improvements in stent design and long-term follow-up using three-dimensional spiral CT scan will be helpful in determining the role of transcatheter stent therapy for native and recurrent coarctation of the aorta. Cathet Cardiovasc Intervent 2001;54:112-125.

We report the use of a new biliary stent (IntraStent Double Strut LD) adapted for use in a 16-year-old young man with moderate-severe transverse arch hypoplasia/coarctation of the aorta following two surgical attempts at correction/relief of the coarctation. The stent implantation procedure resulted in complete relief of the coarctation.

Coarctation of the aorta can be managed in different ways depending upon the age of presentation, anatomical details of the coarctation, the aortic arch anatomy, and whether the coarctation is native or a recurrence following surgery. In infants, surgery is the preferred treatment of choice, whereas in older children and adults, percutaneous procedures, such as balloon angioplasty or stent implantation, are becoming increasingly popular methods for treating coarctation. Various types of stents have been used and this paper specifically addresses the use of covered or graft Jomed stents in a small group of patients with coarctation.

Optimal timing and mode of treatment for patients with aortic coarctation remains controversial, particularly when the degree of obstruction is mild. Surgery, balloon dilatation, and stent implantation have all proven effective in the treatment of moderate or severe obstruction. In this report, we describe the use of stents to treat coarctation in a heterogeneous population, including patients with relatively mild obstruction.

Retrospectively, we studied the results of stent implantation in 33 patients, children and young adults, who underwent catheterization for treatment of coarctation. Patients with isolated coarctation, as well as those with associated cardiac defects, were included. The median systolic pressure gradient of our patients was 25 mm Hg.

Patients had an acute decrease in systolic blood pressure gradient (25 mm Hg to 5 mm Hg, P <.001) and an increase in lumen diameter (8 mm to 13 mm, P <.001). When 16 patients were recatheterized during the follow-up period, gradients remained decreased (30 mm Hg to 14 mm Hg, P <.001) compared with prestent values. Ventricular end-diastolic pressure, which was increased in 82% of patients at the time of initial catheterization, decreased from 17 mm Hg to 14 mm Hg (P =.002). Although the procedure was generally safe, serious complications did occur.

Stent implantation represents a therapeutic option that can safely and effectively reduce gradient in challenging patients with mild postoperative coarctation. Furthermore, our data suggest that aortic obstruction often coexists with ventricular diastolic dysfunction in these patients and that relief of obstruction may play a role in improvement of function.

Obstruction of the left ventricular outflow tract may be associated with hypoplasia of the left heart, which importantly influences the options for treatment. Although the influence of the size of the left heart on the outcome for critical aortic stenosis has been described, less is known about the spectrum of such hypoplasia seen with neonatal aortic coarctation, and how this influences outcome. To determine, first, the spectrum and influence of hypoplasia of the left heart in neonatal coarctation, second, if the previously described critical values for adequacy of the left heart in neonates with critical aortic stenosis are applicable to neonates with coarctation, and, third, if any of the variables or associated abnormalities are risk factors for recoarctation, we studied 63 neonates who underwent repair of coarctation. From the initial echocardiogram, we measured multiple structures in the left heart, and calculated a score for adequacy as has been done for critical aortic stenosis. The sizes were compared to previously reported minimal values. We then analyzed the influence of the variables and the associated anomalies on outcome. There were no deaths. There was a broad spectrum of sizes that did not correlate with the need for re-intervention. The calculated score for adequacy would have predicted survival in only 56% of the patients, and 73% of the neonates had at least one parameter measured in the left heart below the previously reported minimal values. There is, therefore, a broad spectrum of sizes for the left heart in neonates with aortic coarctation that is not predictive of outcome. Minimal sizes, and the score for adequacy used for critical aortic stenosis, are not applicable to neonates with coarctation.

This study was undertaken to evaluate the long-term results of balloon angioplasty (BA) for postsurgical recoarctation in infants.

Balloon angioplasty is a well-accepted modality for the treatment of recoarctation. However, infants remain a group of concern because of their size, risk for complications and the potential for restenosis with growth. Age <12 months has been determined to be a risk factor for the development of recoarctation after angioplasty for native coarctation. Although studies on postsurgical coarctation have found no relationship between age at angioplasty and the development of recoarctation, few studies specifically addressing infants have been performed.

Clinical, echocardiographic, hemodynamic and angiographic data on 22 consecutive children <1 year of age who underwent BA between 1986 and 1996 were reviewed.

A successful result, defined as a postprocedure gradient of < or =20 mm Hg, was achieved in 20 of 22 (91%) infants with a reduction in the systolic peak pressure gradient from 48 +/- 27 to 9 +/- 10 mm Hg (p < 0.001) and an increase in coarctation diameter from 2.7 +/- 1.1 to 5.2 +/- 1.5 mm (p < 0.001). At long-term follow-up of a median of 56 months (0.6 to 12 years), the restenosis rate after an initial optimal result was 16% (3 of 19). Five (24%) infants required reintervention (2 initially unsuccessful; 3 recoarctation), with a success rate of 95% after two procedures. Suboptimal long-term outcome correlated with a lower infant weight.

Balloon angioplasty can be safely performed in infants, with good long-term results. The risk of restenosis is low and can be successfully managed with repeat angioplasty.

To determine the early results of balloon expandable stent implantation for aortic coarctation or recoarctation.

Prospective observational study.

Two paediatric cardiology tertiary referral centres.

17 patients, median age 17 years (range 4.4 to 45) and median weight 61 kg (17 to 92). Six had native aortic coarctation and 11 had aortic recoarctation; 14 had upper limb systolic hypertension. Of those with recoarctation, eight had had at least one previous balloon dilatation attempt and two of these patients also had further surgical interventions.

Balloon expandable Palmaz iliac stent implantation.

Systolic pressures gradients, minimum aortic diameter, upper limb blood pressures, and incidence of aneurysm formation.

18 stents were implanted during 18 procedures in the 17 patients. Mean peak systolic pressure gradient fell from 26 mm Hg (95% confidence interval (CI), 21 to 31 mm Hg) before to 5 mm Hg (2 to 8 mm Hg) after stent implantation (p < 0.001), and mean minimum aortic diameter increased from 7 mm (95% CI, 6 to 8 mm) before to 11.3 mm (10 to 12.6 mm) after implantation (p < 0.001). Complications occurred in five patients (bleeding in two, stent migration in two, and aneurysm formation in one). Two patients remained borderline hypertensive and eight were receiving antihypertensive treatment at most recent assessment.

Stent implantation for aortic recoarctation and native coarctation gives good immediate results. Careful follow up is necessary to evaluate complications and the long term effect on blood pressure.

Operation for aortic recoarctation and/or residual hypoplastic arch represents a surgical challenge because of surrounding scar tissue in the coarctation area, hazard of spinal cord ischemia due to aortic cross-clamping, laceration of the recurrent nerve, and the choice of the best approach. We demonstrate the first results of an anatomically guided technique via the prior left thoracotomy approach without establishment of cardiopulmonary bypass.

Since 1989, five patients underwent anatomically positioned ascending-descending bypass grafting for treatment of recoarctation. Indication was a non-dilatable hypoplastic aortic arch segment; in two cases an additional isthmic restenosis was present. Inclusion criteria for our technique was an aorta ascending diameter large enough to allow partial clamping. Primary repair of aortic coarctation was end-to-end anastomosis in four patients and patch angioplasty in one. Mean age at primary repair was 5.5 years and at reoperation 16.1 years. Systolic pressure gradients at rest ranged from 35 to 70 mmHg; upper extremity hypertension was present in all patients. Operative technique consisted of performing a dacron or PTFE aorta ascending-descending bypass graft parallel to the aortic arch, size 18 or 20 mm in diameter, via the prior left thoracotomy.

There were no intraoperative complications and all patients survived. Postoperative complications were left lung atelectasis with necessity of reintubation, pericardial effusion, and transient left diaphragm elevation, each in one patient. After 7-90 months all patients are free of symptoms, have normal blood pressure (with two patients being under anti-hypertensive medication), and have no echocardiographically measurable pressure gradients.

Anatomically positioned aorta ascending-descending bypass grafting via the prior left posterolateral thoracotomy without cardiopulmonary bypass is a safe and efficient method for operation of complex recoarctation in patients with an acceptable size of the aorta ascendens.

Although immediate and intermediate-term results after balloon dilatation of congenital stenotic lesions of the heart in children are well studied, long-term results have not been documented. Therefore, we reviewed our experience along with the limited published data to address this issue. Late follow-up after balloon pulmonary and aortic valvuloplasty shows low-residual gradients, reintervention-free rates in the mid-80s for pulmonic and in the mid-50s for aortic stenosis, and an increase in degree and prevalence of similunar valve insufficiency. Balloon angioplasty of aortic coarctation results in low-residual gradients, residual hypertension in a minority of patients, low prevalence of aneurysms, and high rates of recurrence in the neonate and young infant. Overall, balloon dilatation is a useful technique in relieving congenital obstructive lesions of the heart in the pediatric patient, but continued study of (1) late pulmonary and aortic insufficiency after valvuloplasty, (2) recurrence and aneurysms after balloon angioplasty of coarctations and, (3) femoral artery compromise in lesions requiring transfemoral artery approach is warranted.

Over the past decade, transcatheter interventions have become increasingly important in the treatment of patients with congenital heart lesions. These procedures may be broadly grouped as dilations (e.g., septostomy, valvuloplasty, angioplasty, and endovascular stenting) or as closures (e.g., vascular embolization and device closure of defects). Balloon valvuloplasty has become the treatment of choice for patients in all age groups with simple valvar pulmonic stenosis and, although not curative, seems at least comparable to surgery for congenital aortic stenosis in newborns to young adults. Balloon angioplasty is successfully applied to a wide range of aortic, pulmonary artery, and venous stenoses. Stents are useful in dilating lesions of which the intrinsic elasticity results in vessel recoil after balloon dilation alone. Catheter-delivered coils are used to embolize a wide range of arterial, venous, and prosthetic vascular connections. Although some devices remain investigational, they have been successfully used for closure of many arterial ducts and atrial and ventricular septal defects. In the therapy for patients with complex CHD, best results may be achieved by combining cardiac surgery with interventional catheterization. The cooperation among interventional cardiologists and cardiac surgeons was highlighted in a report of an algorithm to manage patients with tetralogy of Fallot or pulmonary atresia with diminutive pulmonary arteries, involving balloon dilation, coil embolization of collaterals, and intraoperative stent placement. In this setting, well-planned catheterization procedures have an important role in reducing the overall number of procedures that patients may require over a lifetime, with improved outcomes.

Immediate- and short-term follow-up results of balloon dilatation of aortic recoarctation following surgery have been well documented, but there is sparse data on long-term follow-up. During a 10-year period ending in August 1995, 33 children, aged 2 months to 14 years old, underwent balloon angioplasty of aortic recoarctation. Prior surgery included resection and end-to-end anastomosis (n = 9), subclavian flap (n = 16) or prosthetic (Dacron or Gore-Tex) patch (n = 5) angioplasty, and repair of an interrupted aortic arch (n = 3). Recoarctation developed 1 month to 14 years (mean +/- SD 29 +/- 44 months) after surgery. The indications for angioplasty were peak-to-peak systolic gradients > 20 mm Hg and systemic hypertension and/or congestive heart failure. After balloon angioplasty, the peak-to-peak systolic pressure gradient across the coarctation decreased from 48 +/- 22 to 13 +/- 15 mm Hg (p <0.01), and the size of the coarcted segment increased from 3.3 +/- 1.4 to 6.5 +/- 2.3 mm (p <0.01). Follow-up angiography and/or magnetic resonance imaging were performed in 20 children 17 +/- 12 months after angioplasty. No aneurysms were observed and improvement in the diameter of the coarcted aortic segment (9 +/- 3 mm) persisted. One- to 10-year (median 5) clinical follow-up was available in 32 children. During follow-up, 2 children required surgery to repair a long tubular isthmic narrowing. The residual gradients, determined by arm-leg systolic blood pressure difference, were 5 +/- 8 mm Hg. No patient was symptomatic and only 1 patient (3%) was hypertensive, controlled with antihypertensive medications. We conclude that balloon angioplasty of aortic recoarctation following all types of surgical repair is feasible, safe, and effective with good long-term results. We recommend balloon angioplasty as the procedure of choice in the management of postsurgical recoarctation with hypertension and/or congestive heart failure.

Percutaneous balloon angioplasty is an alternative therapy for the treatment of the typical type of coarctation. Its associated morbidity and mortality compares favorably when compared to the standard treatment surgery. While atypical coarctations are rare, the described cases have been treated surgically. We present a case of unusually located aortic coarctation successfully treated with percutaneous balloon angioplasty.