Previous Coarctation Repair Research Articles

The Fate of the Aorta after Coarctation Repair: Open Surgical Replacement of Descending Aorta in a High-Volume Unit.

Objectives: Complications after aortic coarctation repair are associated with high mortality and require surgical or endovascular reintervention. For patients unsuitable for endovascular therapies, reoperation remains the only therapeutic option. However, surgical experience and up-to-date follow-up data concerning this overall rare entity in the spectrum of aortic reoperations are still highly limited. Thus, the aim of this study was to analyze the short-term outcomes and long-term survival of patients undergoing surgical descending aorta repair after previous coarctation repair in a high-volume unit. Methods: We present a retrospective single-center analysis of 25 patients who underwent open descending aorta replacement after initial coarctation repair. The surgical history, concomitant cardiovascular malformations, and preoperative characteristics as well as postoperative complications and long-term survival were analyzed. Results: The mean age at operation was 45.4 ± 12.8 years. A proportion of 68% (n = 17) of the patients were male. The most common complication necessitating reoperation after coarctation repair was aneurysm formation (68%) and re-stenosis (16%). The average time between initial repair and reoperation was 26.3 ± 9.9 years. Technical success was achieved in all the operations, while recurrent nerve damage (24%) and bleeding requiring rethoracotomy (20%) were identified as the most common perioperative complications. The one-year mortality was 0% and the overall long-term survival was 88% at 15 years. Conclusions: Open surgical descending aorta replacement can be performed safely and with excellent survival outcomes even in the challenging subgroup of patients after previous coarctation repair. Thus, reoperation should be considered a feasible approach for patients who are unsuitable for endovascular therapies. Nonetheless, concomitant cardiovascular anomalies and frequent preoperations may complicate the redo operation in this patient population.

Long-Term Outcomes of Ascending Sliding Arch Aortoplasty.

Background: Coarctation of the aorta can be associated with significant hypoplasia of the aortic arch. In contrast to patch aortoplasty, ascending sliding arch aortoplasty uses viable autologous tissue for potential growth in children. We reviewed the mid- to long-term outcomes of this technique. Methods: Between 2002 and 2023, 28 patients underwent ascending sliding arch aortoplasty for the patients with coarctation of the aorta (n = 22) and interrupted aortic arch (n = 2). Four patients underwent previous surgical coarctation repair at other institutions. The median patient age and body weight were 28.5 months (3 weeks to 15.6 years) and 13.4 kg (3.7-70 kg), respectively. Results: Although one patient had a recurrent nerve injury postoperatively, there were no other major morbidities or mortalities. The last follow-up echocardiography demonstrated that the mean peak velocity improved from 3.9 ± 0.6 to 0.9 ± 0.8 m/s, and the pressure gradient improved from 63.6 ± 21.5 to 7.1 ± 7.7 mm Hg. The postoperative diameters of the ascending aorta, proximal arch, distal arch, and isthmus all increased significantly. The mean postoperative length of stay was 5.9 ± 2.1 days, and the median follow-up time was 7.3 years (10 days to 20.5 years). No reoperation or catheterization-based intervention was performed for residual coarctation. Conclusions: Ascending sliding arch aortoplasty is safe and effective for treating coarctation of the aorta with aortic arch hypoplasia. This technique is applicable for children ranging in size from neonates to older children (or adolescents), recurrent coarctation cases, and provides complete relief of narrowing by utilizing viable native aortic tissue.

Poorer Self-Reported Physical Health and Higher Anxiety Trait in Young Adults With Previous Coarctation Repair

Little is known about the impact of a coarctation repair on the functional outcomes of young adults. This study aimed to determine (1) the functional and mental health status in young adults with previous coarctation repair, and (2) the impact of late hypertension on their quality of life. A cross-sectional study using validated self-reported questionnaires (Short Form 36 version 2 [SF-36v2], Beck Depression Inventory [BDI], and State-Trait Anxiety Inventory [STAI]) was performed in 54 patients aged 15-47 years with previous paediatric coarctation repair. Questionnaire scores were compared to healthy age- and gender-matched controls. Patients' previously published 24-hour blood pressure monitoring results were included. Late hypertension was present in 64% (34/54) at a mean of 29±8 years after coarctation repair. SF-36v2 mean physical component summary score was significantly lower in coarctation patients compared with controls (53.1±6.8 vs 56.0±4.7, p=0.02), but there was no significant difference in mean mental component summary score (p=0.2). SF-36v2 mean role emotional score tended to be associated with 10 mmHg increases in mean 24-hour systolic blood pressure (regression coefficient 4.3 p=0.06). STAI mean trait anxiety score tended to be higher in coarctation patients compared with controls (36.6±9.0 vs 33.5±7.8, p=0.06). There was no significant difference in BDI scores between patients and controls. Young adults with previous coarctation repair report poorer physical health and tended towards higher anxiety trait compared to healthy controls. Strategies to improve self-reported physical health and anxiety should be explored. Long-term assessment of quality of life outcomes in coarctation patients is warranted.

Carotid-Carotid Transposition for Zone 1 Thoracic Endovascular Aortic Repair

Carotid-carotid bypass is the standard technique for cervical aortic arch debranching to maintain left common carotid artery perfusion with zone I thoracic endovascular aortic repair (TEVAR), while left-to-right carotid-carotid transposition (CCT) has been described as an autologous alternative. We report on our center's experience with CCT in the setting of zone I TEVAR. This is the only published series of this technique. All patients who underwent CCT, defined by CPT code 35509, between 2017 and 2020 were identified at our tertiary care center. Patient demographics, indications for CCT, complications specific to CCT, operative details, post-operative course, and outcomes were retrospectively reviewed. A total of 13 patients underwent CCT prior to zone 1 TEVAR. The indications for intervention were thoracic or thoracoabdominal aortic aneurysms and dissections secondary to hypertension (n = 10), Marfan syndrome (n = 2), and Turner syndrome with aneurysmal degeneration of previous coarctation repair (n = 1). There was a high incidence of preexisting hypertension (92%), malnutrition (69%), and smoking (61%) in this cohort. Operative intervention was performed on both an elective (n = 7, 54%) and an urgent (n = 6, 46%) basis. Complications directly related to CCT included transient unilateral recurrent laryngeal nerve deficit (n = 1, 7.7%). There were no cerebrovascular events, surgical site infections, or procedure-related mortalities. All transpositions with follow-up imaging were patent without stenosis or thrombosis (average 7.2 months, n = 10). There were no late complications related to CCT. CCT is a safe and autologous alternative to carotid-carotid bypass for left common carotid artery revascularization with zone I TEVAR.

Poorer Self-Reported Physical Health and Higher Anxiety in Young Adults with Previous Coarctation Repair

Background: Little is known about the impact of a coarctation repair on the functional outcomes of young adults. This study aimed to determine (1) the functional and mental health status of young adults with previous coarctation repair, and (2) the impact of late hypertension on their quality of life. Methods: A cross-sectional study using validated self-reported questionnaires (Short Form 36 version 2 [SF-36v2], Beck Depression Inventory [BDI], and State-Trait Anxiety Inventory [STAI]) was performed in 54 patients aged 15–47 years with previous paediatric coarctation repair. Questionnaire scores were compared to healthy age- and gender-matched controls. Patients’ previously published 24-hour blood pressure monitoring results were included. Results: Late hypertension was present in 64% (34/54) at a mean of 29 ± 8 years after coarctation repair. SF-36v2 mean physical component summary score was significantly lower in coarctation patients compared with controls (53.1 ± 6.8 vs. 56.0 ± 4.7, p = 0.02), but there was no significant difference in mean mental component summary score (p = 0.2). SF-36v2 mean role emotional score tended to be associated with 10 mmHg increases in mean 24-hour systolic blood pressure (regression coefficient 4.3, p = 0.06). STAI mean trait anxiety score tended to be higher in coarctation patients compared with controls (36.6 ± 9.0 vs. 33.5 ± 7.8, p = 0.06). There was no significant difference in BDI scores between patients and controls. Conclusion: Young adults with previous coarctation repair report poorer physical health and higher anxiety compared to healthy controls. Strategies to improve self-reported physical health and anxiety should be explored. Long-term assessment of quality of life outcomes in coarctation patients is warranted.

Major Device-Dependence of Measured Hypertensive Status From 24-Hour Ambulatory Blood Pressure Monitoring After Aortic Coarctation Repair

Twenty-four-hour (24-hr) ambulatory blood pressure monitoring (ABPM) is often considered the gold standard to detect hypertension. We aimed to determine the short-term progression of 24-hour blood pressure after coarctation repair and to compare ABPM between two different devices. We performed a cross-sectional study using 24-hour ABPM (Oscar 2) in 47 patients aged 16-48 years with previous paediatric coarctation repair and not on antihypertensive medication. Results were compared to a previous ABPM using paired analyses. A subset (10/47, 21%) had an additional previous ABPM performed using a Spacelabs device. After a mean follow-up of 27±6 years after repair, hypertension and prehypertension on Oscar 2 ABPM was present in 57% (27/47) and 11% (5/47), respectively. Mean follow-up time between Oscar 2 ABPMs was 3.9±1.4 years, and between first Oscar 2 and Spacelabs and between Spacelabs and second Oscar 2 ABPM was 1.4±0.8 and 1.8±0.3 years, respectively. There was no difference in the proportion of hypertensive patients between Oscar 2 ABPMs (55% [26/47] vs. 57% [27/47], p=1.0) but 17 patients (17/47, 36%) had a reclassification of 24-hour ABPM status. Mean 24-hour systolic blood pressure was higher in both Oscar 2 ABPMs compared to Spacelabs (142.4±11.7 vs. 120.4±11.8mmHg, p=0.0001; and 137.4±12.2 vs. 120.4±11.8mmHg, p=0.0001; respectively). There was high intra-device reproducibility of 24-hour ABPM results using an Oscar 2 device but poor inter-device reproducibility in patients with repaired coarctation. Device-specific reference values may be required to ensure reliable 24-hour ABPM interpretation.

Coarctation repair-redo challenges in the adults: what to do?

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.

A Cross-Sectional Study of the Prevalence of Exercise-Induced Hypertension in Childhood Following Repair of Coarctation of the Aorta

Exercise-testing may be a more tolerable method of detecting hypertension in children after coarctation repair compared to gold-standard 24-hour ambulatory blood pressure (BP) monitoring (ABPM). This study aims to determine the prevalence of exercise-induced hypertension and end-organ damage in children after coarctation repair, and the effectiveness of exercise-testing compared to 24-hour ABPM in this population. Exercise-testing (Bruce protocol), transthoracic echocardiogram, 24-hour ABPM, and pulse wave velocity were performed in 41 patients aged 8 to 18 years with previous coarctation repair. Median age at repair was 13 days. Exercise-testing data were compared to healthy paediatric controls. Hypertension was defined as BP >95th percentile on 24-hour ABPM compared to normalised data, and systolic BP (SBP) arbitrarily >200mmHg on exercise-testing. After 13±3years, 39% (14/36) were hypertensive on 24-hour ABPM and 12% (5/41) on exercise-testing. Coarctation patients had a higher peak exercise SBP and reduced endurance compared to controls (164±26mmHg vs. 148±19mmHg, p=0.003; and 13.0±1.7mins vs. 14.2±2.4mins, p=0.007; respectively). All patients with a peak exercise SBP >190mmHg were hypertensive on 24-hour ABPM. Pulse wave velocity was higher in hypertensive patients on exercise-testing and 24-hour ABPM compared to normotensive patients (p=0.004 and p=0.06; respectively). Exercise-testing may be a useful tool to detect hypertension in children and young adults after coarctation repair, particularly in those who do not tolerate 24-hour ABPM. Normative peak exercise BP data for age should be obtained to improve the accuracy of exercise-testing in detecting hypertension.

Impact of arch reobstruction and early hypertension on late hypertension after coarctation repair.

Late hypertension after coarctation repair is associated with high mortality, but its risk factors remain unclear. This study aims to determine early and late postoperative risk factors for late hypertension after coarctation repair. A cross-sectional study including transthoracic echocardiogram and 24-h blood pressure (BP) monitoring was performed in 144 patients aged ≥10 years with previous coarctation repair. Median age at repair was 39 days (interquartile range 0-3 years). Early postoperative hypertension was evaluated by calculating the mean of BP measurements taken on the same day before hospital discharge or the need for antihypertensives prior to discharge. Multivariable analyses were performed to adjust for gender, surgical age, and follow-up age. After a mean follow-up period of 22 ± 7 years, 59% (84/142) of patients were hypertensive: 58% (82/142) on 24-h BP monitoring and 1% (2/142) on antihypertensives. Early postoperative hypertension was present in 58% (73/126): 39% (49/126) on BP measurements and 19% (24/126) on antihypertensives. Early and late arch reobstruction (transthoracic echocardiogram peak gradient ≥25 mmHg) was present in 37% (23/62) and 23% (33/144), respectively. On multivariable logistic analysis, early postoperative hypertension and maximum descending arch velocity on echocardiography were associated with late hypertension on 24-h BP monitoring (odds ratio 2.21, 95% confidence interval 1.05-4.66, P = 0.04; and odds ratio 2.28, 95% confidence interval 1.08-4.81, P = 0.03; respectively). There is a high prevalence of late hypertension after coarctation repair. Arch reobstruction may be a major determinant of late hypertension. Early postoperative hypertension may identify very early in life those at risk of developing late hypertension.

Left ventricular hypertrophy in adults with previous repair of coarctation of the aorta; association with systolic blood pressure in the high normal range

BackgroundArterial hypertension is common in adults with repaired coarctation of the aorta (CoA). The associations between the diagnosis of hypertension, actual blood pressure, other factors affecting left ventricular overload, and left ventricular hypertrophy (LVH) are not yet fully explored in this population. Material and resultsFrom the national register for congenital heart disease, 506 adult patients (≥18years old) with previous repair of CoA were identified (37.0% female, mean age 35.7±13.8years, with an average of 26.8±12.4years post repair). Echocardiographic data were available for all patients, and showed LVH in 114 (22.5%) of these. Systolic blood pressure (SBP) (mmHg) (OR 1.02, CI 1.01–1.04), aortic valve disease, (OR 2.17, CI 1.33–3.53), age (years) (OR 1.03, CI 1.01–1.05), diagnosis of arterial hypertension (OR 3.02, CI 1.81–5.02), and sex (female) (OR 0.41, CI 0.24–0.72) were independently associated with LVH. There was an association with LVH at SBP within the upper reference limits [130, 140] mmHg (OR 2.23, CI 1.05–4.73) that further increased for SBP>140mmHg (OR 8.02, CI 3.76–17.12). ConclusionsLVH is common post repair of CoA and is associated with SBP even below the currently recommended target level. Lower target levels may therefore become justified in this population.

Longer-term outcome of perventricular device closure of muscular ventricular septal defects in children.

To describe the longer-term clinical experience and follow-up with perventricular device closure of ventricular septal defects (VSD) in children. Between January 2005 and December 2013, muscular ventricular septal defect closure with the Amplatzer Muscular VSD Occluder was undertaken using a hybrid perventricular approach. Data including demographic, echocardiographic parameter, and clinical outcome were reviewed retrospectively. Median age at the time of procedure was 8.9 months (range 1.9-31.0 months) and median weight was 6.6 kg (range 4.5-12.9 kg). All had a moderate to large muscular VSD, three had more than one VSD, four had previous coarctation repair, and five had previous pulmonary artery (PA) banding. A single Amplatzer muscular occluder (range 8-18 mm) was deployed in each patient without cardiopulmonary bypass under echocardiographic guidance. Two of ten patients subsequently required a short period of cardiopulmonary bypass for reconstruction of PA after de-banding and closure of atrial septal defect in one. Occluder removal was necessary in one patient due to entrapment of the tricuspid valve and progressive tricuspid regurgitation. This patient underwent surgical repair with a good result and no device-related valve damage. In the remaining nine patients, no severe complications such as device embolization, arrhythmia, or significant valve regurgitation were noted in the post-operative period or follow-up. At a median time of 6.5 years (range 0.9-8.4 years) post device implantation, complete closure was achieved in five patients and four had small residual leaks, which were not hemodynamically significant. Perventricular muscular VSD closure is effective in small children with suitable muscular defects and may avoid the morbidity associated with cardiopulmonary bypass and conventional surgical repair. There have been no late complications with this approach.

Repair of primary or complicated aortic coarctation in the adult with cardiopulmonary bypass and hypothermic circulatory arrest

Patients with primary aortic coarctation or complications of a previous coarctation repair may seek treatment as adults. Management options include open, endovascular, hybrid, and extra-anatomic repairs. We evaluated the results of open direct repair with total cardiopulmonary bypass and hypothermic circulatory arrest. Twenty-seven patients with primary coarctation with or without associated aortic abnormalities or with complications of a previous coarctation repair were treated during a 20-year interval. Mean age was 37 years (range, 15-71 years). Thirteen patients had primary coarctation (7 with associated aneurysms), 3 patients had aneurysms of the aorta above or below a noncritical narrowing of the aorta at the isthmus ("pseudocoarctation"), and 11 patients had previous repair of a coarctation. The previous repairs were bypass grafting of the coarctation (n=5), interposition grafting (n=3), patch angioplasty (n=2), and primary repair (n=1). Mean durations of cardiopulmonary bypass, hypothermic circulatory arrest and hypothermic fibrillation were 104, 36, and 76 minutes, respectively. There were no in-hospital deaths, and no patients sustained stroke, spinal cord ischemic injury, or renal or respiratory failure. Thirteen patients received no blood products. No patient has had evidence of recurrent coarctation or aneurysm formation. Cardiopulmonary bypass with hypothermic circulatory arrest is a safe and suitable technique for treatment of primary and recurrent coarctation. It is associated with low operative risk and absence of major complications.

Detection of Serious Complications by MR Imaging in Asymptomatic Young Adults with Repaired Coarctation of the Aorta

Despite early repair, patients with aortic coarctation (CoA) continue to have a reduced life expectancy due to the development of late complications. We sought to define the rate of aortic abnormalities in patients with previous CoA repair, referred for surveillance magnetic resonance (MR) imaging. We evaluated 59 asymptomatic adults consecutively for repaired CoA with MR imaging between 2008 and 2012. Patients were aged 29 ± 8 (16-49) years; 34 males (58%) and 34 with bicuspid aortic valve (58%). Median age at the time of initial repair was two years. Surveillance MR imaging was performed 23 ± 8 years post-procedure. The three most common interventions performed were subclavian-flap repair (25%), end-to-end repair (33%) and patch aortoplasty (33%). Re-intervention with balloon angioplasty or repeat surgery had been performed in 22% of subjects. There were 28 cases of recoarctation (48%), of which seven were at least moderate in severity. Repair site and ascending aortic aneurysm occurred in eight (14%) and four (7%) subjects, respectively. Freedom from any degree of recoarctation and other aortic complications was observed in eight subjects (14%). MR imaging detected a high rate of aortic abnormalities in asymptomatic adults after CoA repair, including 27% with clinically significant recoarctation and/or local aneurysm formation.

Type A aortic dissection in patients with bicuspid aortic valves: clinical and pathological comparison with tricuspid aortic valves

ObjectiveBicuspid aortic valve (BAV) is associated with a higher risk of type A aortic dissection (AD) compared with tricuspid aortic valve (TAV). We sought to study differences between patients with...

Long-term results of endovascular aortic repair for thoracic pseudoaneurysms after previous surgical coarctation repair

Late aneurysm formation has been reported after every type of surgical coarctation repair in up to 10%, with rupture of such aneurysms being responsible for approximately 7% of all deaths. Secondary surgical repair carries a significant mortality (up to 15%) and morbidity rate (recurrent laryngeal nerve paralysis ∼20%, phrenic nerve injury ∼5%). According to the positive experience with endovascular therapy of atherosclerotic thoracic aortic aneurysms, it is worthwhile to evaluate the concept of minimally invasive endovascular stent grafting for secondary repair of postsurgical aneurysms. Data were collected prospectively on consecutive patients who presented with postcoarctation false aneurysms. Since 1999, in a cohort of 210 endovascularly treated patients with thoracic and thoracoabdominal aortic pathologies, four patients with postcoarctation false aneurysms underwent endoluminal stent-graft placement. All of these procedures were technically successful without 30-day or one-year procedure-related mortality. After a follow-up of 71months in median (range, 7-93months; mean, 60.5months), all aneurysms remain excluded without any endoleak. According to the current limited experience of small series, the endoluminal repair seems to be a promising alternative to redo open operations for postsurgical thoracic aneurysms associated with coarctation repair. Long-term follow-up of our small cohort confirmed the durability of the stent-graft treatment.

Aortic coarctation complicated by wall aneurysm

To evaluate the management of aneurysms associated with aortic coarctation by covered stent deployment. Aortic aneurysm formation complicating aortic coarctation carries a risk of rupture with high mortality rate. Surgical repair has a significant mortality and may be associated with central nervous system injury, including paraplegia. Transcatheter intervention alternatively carries a risk of aneurysmal rupture. Between July 2004 and September 2009, 140 consecutive patients underwent percutaneous stent implantation to treat aortic coarctation. Eleven (7.9%) patients had coarctation associated with aortic wall aneurysm: three with native coarctation with wall aneurysm, three with previous surgical coarctation repair, three with previous balloon angioplasty, and two with previous bare stent implantation. The e-PTFE-covered 8Zig Cheatham-Platinum stents were used to treat all 11 patients. Median age at procedure was 13 years (range, 6-66 years). Successful device deployment was achieved in all patients. Systolic pressure gradient reduced significantly from a median baseline of 30 mm Hg (range, 25-50 mm Hg) to a postprocedural median of 5 mm Hg (range, 0-20 mm Hg) (P < 0.01). Aortic diameter increased from a median value of 6 mm (range, 0.5-11 mm) to a median value of 12 mm (range, 10-22 mm) (P < 0.001). No early complications occurred, with successful relief of stenoses and complete sealing of all aneurysms. Median follow-up was 50 months (range, 16-61 months). Redilatation was required in four patients (one patient with intrastent restenosis secondary to significant endothelial growth, whereas the other three patients showed restenosis secondary to somatic growth). Redilatation with a larger balloon was performed without complication in all cases. Our data show that covered stents are a safe and effective treatment with low risk of complications for the treatment of coarctation associated with aortic wall aneurysm.

Use of Minimized Extracorporeal Circulation System in Noncoronary and Valve Cardiac Surgical Procedures—A Case Series

The aim of this report is to explore application of minimized cardiopulmonary bypass (CPB) circuits in areas of cardiac surgery other than coronary bypass grafting and aortic valve surgery. We report three cases operated under minimal extracorporeal circulation support. Replacement of the descending thoracic aorta was performed in two patients; one with a descending aortic aneurysm and one with pseudoaneurysm formation after previous coarctation repair. We have also implanted a left ventricular assist device for destination therapy. The minimized extracorporeal circulation system provides optimal circulatory support, while it is associated with reduced postoperative morbidity, minimizing the side effects from the use of CPB. Moreover, when off-pump technique is attempted, it can be used as a standby circuit connected to the patient so as to enhance safety of the procedure. Minimized extracorporeal circulation systems can be used with safety and efficacy in a wide range of cardiac surgeries including descending aorta pathology and assist device implantation.

312 Exercise testing coupled with Doppler echocardiography: a simple, safe and effective method to assess the severity of coarctation of the aorta

Peak systolic Doppler velocity at the aortic isthmus alone is not a good predictor of severity in patients with native or residual coarctation (CoA). A major argument in favour of a hemodynamically significant CoA is the presence of a diastolic gradient (DG) with characteristic “sawtooth” appearance of the Doppler pattern. to evaluate, in patients with some degree of aortic isthmus narrowing but without significant DG at rest, if exercise could unmask DG and thus hemodynamically significant CoA. Fourteen patients aged from 12 to 56 years underwent treadmill exercise testing coupled with Doppler echocardiography. Thirteen had previous CoA repair 8 to 43 years before, one had aortic kinking with mild isthmic narrowing on CT-scan. MRI or CT-scan were performed in 11 patients, showing significant residual stenosis (narrowing > 30%) in 8/11. Doppler measurements were performed during exercise testing and 5 minutes after at the suprasternal notch, using a CW-Doppler 2 MHz pedoff probe. The peak systolic and diastolic gradients through aortic isthmus were measured at rest and at the end of the exercise. A peak DG > 17 mmHg was considered significant. Mean peak systolic gradient increased from 29 to 65 mmHg with exercise. Significant DG appeared in 4 of 8 patients without any DG at rest. Among the 6 patients with non significant DG at rest, DG remained non significant in 2 and became significant in 4. For the 11 patients with MRI or CT-scan aortic arch evaluation, the specificity of significant DG at exercise was 100%, the sensitivity 75%, predictive positive value 100%, negative predictive value 60%. Apparition of a significant isthmic Doppler DG at exercise can reliably predict the hemodynamic significance of restenosis after CoA repair, as well as native narrowing of the aorta. This simple, safe and effective non-invasive method may help to identify patients requiring surgery or percutaneous stenting.

Aggressive tumoral calcinosis in an infant thoracotomy scar

The authors report a case of tumoral calcinosis (TC) in a six-month-old infant, which developed within the thoracotomy scar from previous aortic coarctation repair. After initial resection of the lesion, the child returned with a large mass of TC restricting movement of the left shoulder. Repeated total resection was successful with no recurrence in 12 months' follow-up. This is the first report of TC that developed in a postoperative scar and is unusual in its recurrence and aggressive growth. Pathogenesis, diagnosis and treatment of this rare event is discussed.

Late Outcomes of Reintervention on the Descending Aorta After Repair of Aortic Coarctation

After repair of aortic coarctation, patients may develop restenosis, aneurysms, and pseudoaneurysms at the site of prior repair. We assessed the outcomes of late reintervention on the descending aorta after aortic coarctation repair. From March 1954 to July 2008, 130 patients had operations or endovascular procedures on the descending aorta after previous coarctation repair. We excluded patients who had complex left-sided cardiac lesions or interrupted aortic arch. Mean age at reintervention was 32±24 years and 28% were female. The interval between coarctation repair and reintervention was 17±13 years. Seventy-four percent of patients had hypertension. Reasons for reintervention were restenosis (n=122 [94%]), aneurysm (n=4 [3%]), and pseudoaneurysm (n=4 [3%]). Ninety-five patients (73%) underwent operative procedures including an extra-anatomic conduit (n=41), patch repair (n=32), interposition graft (n=14), end-end anastomosis (n=6), and subclavian flap (n=2). Thirty-five patients underwent endovascular treatment (balloon dilatation, n=22 or stenting, n=13). There was no early mortality. In the surgical group, 5 patients required early reoperation for bleeding and 5 patients had early vocal cord paralysis. One patient in the endovascular group had aortic rupture at the time of intervention requiring urgent operation. Survival was 97% at 10 years. At 5 years, freedom from a second repeat procedure on the descending aorta was 96% in the surgical group and 72% in the endovascular group (P<0.001). Five years after reintervention, fewer patients required treatment for hypertension (57% versus 74%, P<0.001) and a median of 1 antihypertensive medication was prescribed compared with a median of 2 medications preintervention. Operative and endovascular management of recoarctation can be performed safely with good late outcomes.