Coarctation Patients Research Articles

Open, Hybrid, and Endovascular Treatment for Aortic Coarctation and Postrepair Aneurysm in Adolescents and Adults

Open, hybrid, and endovascular procedures are used for grown-up patients with aortic coarctation and complications after repair, an expanding population. We sought to characterize patients and procedures, assess early and late outcomes, and describe indications to guide treatment of these complex patients. Between May 1999 and January 2011, 110 patients underwent open (n=40), hybrid (n=11), or endovascular (n=59) repair of coarctation (n=43), recurrent aortic coarctation (n=42), or postrepair aneurysm (n=25). Mean age was 38±14 years. Sixty-eight had previous repairs (median 27 years earlier; range, 1 to 50). Twenty-two had prior cardiovascular operations other than coarctation and 50% had bicuspid valve. Fifty-nine concomitant procedures were performed in 45 patients (40%). Data were from the prospective database, chart review, and Social Security Death Index. Technical success was achieved in 100%, with no hospital deaths, no strokes, and no paraplegia. Complications were uncommon and included respiratory failure (n=2, 1.8%), and temporary renal failure (n=2, 1.8%). Twenty-two patients required reinterventions, but half of those were planned. There was no difference in occurrence of unplanned reintervention between approaches (endovascular 12%, hybrid 18%, open 12.5%). Length of stay was 4.8±4.8 days. Transcoarct gradient fell from 37.6±18 mm Hg preoperatively to 7.0±6.9 mm Hg in coarctation patients. Postrepair aneurysm patients had no late ruptures, and maximum diameter shrunk from 5.9±1.3 cm preoperatively to 4.8±1.3 cm. Estimated survival at 1, 5, and 8 years was 95%, 95%, and 90%, respectively. Coarctation, recurrent coarctation, and postrepair aneurysm/pseudoaneurysm in adolescent and adult patients can be safely and effectively managed with open, hybrid, or endovascular techniques. Optimal results are achievable in this complex population of patients with a multimodality approach tailored to surgical indication and anatomy. All survivors of coarctation repair require lifelong surveillance.

Aortic Coarctation and Coronary Artery Disease

Aortic coarctation (CoA) is a congenital defect involving a constriction of the aortic arch, usually occurring near the site of insertion of the ductus arteriosus. Surgical and endovascular repair techniques and success rates, measured by short-term survival, elimination of the gradient across the stricture, and normalization of systemic blood pressure, improved steadily over the last half of the 20th century. Despite apparently successful correction of the obstruction, however, individuals with a history of CoA demonstrate excess morbidity and premature mortality associated with hypertension (HTN), cerebrovascular accident, coronary artery disease, and aortic dissection/rupture.1–3 These adverse outcomes are to some extent independent of the severity of the original obstruction, type of treatment, restenosis, or presence of prosthetic grafts.2,4 Whereas blood pressure usually normalizes for a time after successful repair, ≈one third of CoA patients develop HTN by adolescence5 and ≈90% by middle age.2 The pathogenesis of the later onset HTN remains poorly understood.6 Normotensive children and young adults who had undergone successful CoA repair were found to have persistent endothelial dysfunction7 and impaired arterial reactivity,8 suggesting that intrinsic vascular abnormalities might contribute to the risk for premature coronary artery disease (CAD), independent HTN. Article see p 16 In this issue of Circulation , a well-designed cross-sectional study from McGill University specifically addresses the CAD risk in CoA.9 Roifman et al9 identified 756 individuals diagnosed with CoA and 6471 with ventriculoseptal defect between the years 1983 and 2005 from Quebec's Congenital Heart Disease Database. They compared the rate of cardiovascular diagnoses in age-matched CoA and ventriculoseptal defect cohorts (median age 30 years). The CoA group had significantly greater rates of HTN (45% versus 16%), CHF (15% versus 7%), and stroke (5.5% versus 2.6%; P <0.0001 for all). CoA patients also …

Percutaneous treatment of native aortic coarctation in adults

Aortic coarctation is a common congenital cardiac defect, which can be diagnosed over a wide range of ages and with varying degrees of severity. We present two cases of patients diagnosed with aortic coarctation in adulthood. Both patients were treated by an endovascular approach. These cases demonstrate the variety of indications in which percutaneous treatment is an excellent alternative for surgical treatment in adult native coarctation patients.

Increased risk for ascending aortic dilatation in patients with complex compared to simple aortic coarctation

AimsAortic coarctation (CoA) occurs as a “simple” isolated disorder, and in a more “complex” form, combined with associated congenital cardiac abnormalities. Long term outcome of all CoA patients may be complicated by dilatation of the thoracic aorta. The aim of this study was to quantify progressive aortic dilatation, and identify determinants for progressive aortic dilatation. Methods and ResultsCardiovascular Magnetic Resonance Imaging (CMR) and echocardiographic data of 93 CoA patients were analyzed retrospectively on the progression, and determinants, of progressive thoracic aortic dilatation. Outcome of simple- versus complex CoA patients were compared. 93 CoA patients (mean age 39±12years, male 59%) were followed with CMR (follow-up 5.3±1.8years). Twenty-eight patients were classified as simple- and 68 as complex CoA. The mean progression rate of thoracic aortic dilatation was highest in the ascending aorta with 2.2±2.0mm/5years (range 0–7.2mm/5years). History of VSD (β=1.77, P=0.004) and an increased left ventricular mass index (β=0.02, P=0.04) were associated with progressive ascending aortic dilatation. Complex CoA patients show an increased progression rate compared to simple CoA patients with 2.4mm/5years versus 1.5mm/5years respectively. (P=0.03). ConclusionAdult post-coarctectomy patients show an increased mean progression rate of ascending aortic dilatation with 2.2mm/5years. The progression rate of ascending aortic dilatation is increased in complex CoA patients, as compared to simple CoA patients. These findings point towards a more comprehensive genetic subset of patients with an increased risk for progressive ascending aortic dilatation.

Letters to Editor Hypertension without renovascular stenosis: a rare case of abdominal aorta coarctation treated with percutaneous transluminal angioplasty

Letters to Editor Hypertension without renovascular stenosis: a rare case of abdominal aorta coarctation treated with percutaneous transluminal angioplasty

Percutaneous treatment of native aortic coarctation in adults

Aortic coarctation is a common congenital cardiac defect, which can be diagnosed over a wide range of ages and with varying degrees of severity. We present two cases of patients diagnosed with aortic coarctation in adulthood. Both patients were treated by an endovascular approach. These cases demonstrate the variety of indications in which percutaneous treatment is an excellent alternative for surgical treatment in adult native coarctation patients.Electronic supplementary materialThe online version of this article (doi:10.1007/s12471-011-0198-x) contains supplementary material, which is available to authorized users.

Impact of Obesity on Left Ventricular Geometry and Function in Pediatric Patients after Successful Aortic Coarctation Repair

To evaluate if obesity has an additional negative impact on left ventricular (LV) geometry and function in normotensive pediatric patients >12 months after successful treatment of aortic coarctation (CoA). We studied 40 CoA patients (mean age 14 ± 3 years, and male sex 70%), of them 10 were obese and 30 lean. Both groups were age and sex comparable. The entire studied sample underwent 24-ambulatory blood pressure (BP) monitoring, standard echocardiographic evaluation, and speckle tracking study. Both office and 24-hour diastolic BP were significantly increased in obese patients. Obese CoA patients showed increased LV mass (52 ± 13 g/m(2.7) vs. 43 ± 9 g/m(2.7) , P = 0.02), and significant reduction in E/A compared with lean CoA patients. Myocardial deformation properties were significantly reduced in obese CoA patients in all the three studied planes (longitudinal, radial, and circumferential) compared with CoA lean patients. LV twist values showed a significant reduction in the obese CoA group (9.9° ± 2.2° vs. 14.5° ± 2.3°, P < 0.0001). Our study shows that obesity in successfully treated CoA children, has an additional negative effect on BP, LV mass, and cardiac function. These findings are of particular concern, since life expectancy in CoA patients is limited mainly by atherosclerosis, and all the obesity-associated abnormalities found are harbingers of higher cardiovascular risk.

Computational Simulations Demonstrate Altered Wall Shear Stress in Aortic Coarctation Patients Treated by Resection with End-to-end Anastomosis

Atherosclerotic plaque in the descending thoracic aorta (dAo) is related to altered wall shear stress (WSS) for normal patients. Resection with end-to-end anastomosis (RWEA) is the gold standard for coarctation of the aorta (CoA) repair, but may lead to altered WSS indices that contribute to morbidity. Computational fluid dynamics (CFD) models were created from imaging and blood pressure data for control subjects and age- and gender-matched CoA patients treated by RWEA (four males, two females, 15 ± 8 years). CFD analysis incorporated downstream vascular resistance and compliance to generate blood flow velocity, time-averaged WSS (TAWSS), and oscillatory shear index (OSI) results. These indices were quantified longitudinally and circumferentially in the dAo, and several visualization methods were used to highlight regions of potential hemodynamic susceptibility. The total dAo area exposed to subnormal TAWSS and OSI was similar between groups, but several statistically significant local differences were revealed. Control subjects experienced left-handed rotating patterns of TAWSS and OSI down the dAo. TAWSS was elevated in CoA patients near the site of residual narrowings and OSI was elevated distally, particularly along the left dAo wall. Differences in WSS indices between groups were negligible more than 5 dAo diameters distal to the aortic arch. Localized differences in WSS indices within the dAo of CoA patients treated by RWEA suggest that plaque may form in unique locations influenced by the surgical repair. These regions can be visualized in familiar and intuitive ways allowing clinicians to track their contribution to morbidity in longitudinal studies.

Redo sternotomy for extra-anatomical correction of aortic coarctation and mitral repair in an adult after failed endovascular treatment

Recently, extra-anatomical bypass surgery has been widely used in complicated adult aortic coarctation cases with concomitant intracardiac repair. Stent implantation has been widely used for primary aortic coarctation as well. The procedure has been shown to be effective with long term follow ups. However, failed stent implantations like stent fracture and dislodgement may complicate the clinical status and subsequent surgical procedure. Extra-anatomic bypass can provide effective results and lower morbidity in cases with concomitant intracardiac problems and stent failure. Here we present an adult aortic coarctation patient who had undergone a Bentall operation and two unsuccessful stent implantations for recurrent aortic coarctation. The patient then got an extra-anatomic bypass for aortic coarctation and concomitant mitral valve commissurotomy through median sternotomy.

Aortic Hemodynamics in Patients With and Without Repair of Aortic Coarctation

The purpose of this study was to characterize hemodynamic alterations and flow-derived vessel wall parameters in aortic coarctation (CoA) patients with and without operative repair by time-resolved, 3-dimensional, and 3-directional velocity sensitive, phase-contrast magnetic resonance imaging (4D PC MRI) in comparison with healthy subjects. Twenty-four patients, 12.5 ± 6.4 years after CoA repair, 4 patients without treatment for CoA, and 19 healthy subjects were examined. The study was approved by the institutional review board and signature of written informed consent was obtained from the participants. Echocardiography was performed in patients before participation. MRI studies were conducted by applying flow-sensitive 4D phase-contrast MRI at either 1.5 T (n = 5 patients) or 3 T (all 19 healthy subjects, n = 23 patients). Blood flow visualization was used to evaluate overall aortic helicity, presence of pronounced or additional localized helix flow, and vortex development. Quantitative evaluation comprised the calculation of regional time-averaged absolute wall shear stress (WSS(mag)), peak velocities, and oscillatory shear index at 8 locations distributed along the thoracic aorta and additionally at the site of CoA. Inter- and intraobserver variabilities of calculations were determined. Volunteers and patients demonstrated the same amount of overall aortic helicity. In contrast, the number of additional localized helix flow or vortex formation was significantly increased in patients (25/28 patients vs. 5/19 normal controls, Fisher exact test: P < 0.001). Vortices in the orifices of the supra-aortic branches were detected in 64.3% (18/28) of patients but in only 11.8% (2/19) of controls (P < 0.001). Quantitative analyses revealed a significant increase in overall aortic WSS(mag) (0.44 ± 0.17 N/m(2) in patients vs. 0.27 ± 0.08 N/m(2) in volunteers, P < 0.005) and a decrease in overall oscillatory shear index. Repeated quantitative analysis showed moderate interobserver and low intraobserver variability. Correlation with echocardiography showed good agreement with MRI which tended to underestimate peak velocities (r = 0.76; Bland-Altman analysis, limits of agreement = -0.57-2.16 m/s, mean = 0.79 m/s). Alterations in aortic hemodynamics after CoA repair are not limited to the specific region of repair, but can be found in the entire aorta. The presented findings highlight the systemic nature of the disease and the need for a systemic diagnostic approach which can be provided by flow-sensitive 4D PC MRI. Furthermore, valuable additional insights on the hemodynamic consequences of coarctation have been shown that may help understanding secondary complications such as restenosis, aneurysm formation, and arterial hypertension.

Midabdominal aortic coarctation presenting with severe pre-eclampsia

This is a case report of a 23-year-old pregnant woman with uncontrolled hypertension, resulting in delivery of her baby at 28 weeks. Postpartum, she further developed claudication and postprandial abdominal discomfort and was diagnosed with coarctation of the midabdominal aorta. She underwent thoracoabdominal aortic repair from the descending thoracic to infrarenal aorta. A bifurcated graft was used to complete the aorto-common hepatic artery and aorto-superior mesenteric artery bypasses. A third graft limb was sewn to revascularize the left renal artery. At 1-year follow up, all symptoms had resolved. This is an interesting coarctation patient treated successfully with thoracoabdominal reconstruction.

Exercise capacity after coarctation repair relates to the c.46A &gt; G genomic polymorphism of the ß2-adrenoreceptor and the c.704T &gt; C angiotensinogen polymorphism

Even after excellent repair of aortic coarctation without restenosis there are limitations in exercise capacity at long-term follow-up. This study was performed to assess the contribution of inherited genomic polymorphisms to exercise capacity in patients without restenosis. 122 patients aged 17-72 years, 46 female, 76 male, seen 2-27 years after repair of aortic coarctation with a residual brachial-ankle-gradient ≤20 mmHg were investigated. Genomic polymorphism of angiotensin converting enzyme (ACE I/D), angiotensinogen (AGT, c.704C > T), angiotensin II receptor type 1 (AGTR1, c.1166A > C), endothelin 1 (EDN1, EDN1/ex5-c.5665G > T), G protein (GNB3, c.825C > T), and two polymorphisms each of the ß1-adrenoreceptor (ADRB1, c.145G > A and c.1165C > G), ß2-adrenoreceptor (ADRB2, c.46A > G and c.79C > G), and endothelial NO synthase (NOS3, intron 4 I/D and NOS3, c.894G > T) were determined by PCR amplification and fragment length analysis. Exercise capacity was determined by an upright bicycle exercise test. Only the c.46A > G polymorphism of the ADRB2 (p = 0.024) and the c.704T > C AGT polymorphism (p = 0.042) were positively correlated with peak workload. Patients with one or especially two polymorphic alleles showed a significant higher exercise performance compared with those patients homozygous for the wild type. In contrast to a previous study in heart failure patients, after coarctation repair adults had a better exercise capacity with the G allele of the ß2-receptor c.46A > G polymorphism. Therefore, the exercise capacity of coarctation patients does not profit from an enhanced down regulation of their beta receptors.

Masked hypertension in young patients after successful aortic coarctation repair: impact on left ventricular geometry and function

Life expectancy is still reduced in aortic coarctation (AoC) patients despite a successful repair because of late arterial hypertension and atherosclerosis. Masked hypertension (MH) consists of an elevated daytime or awake ambulatory blood pressure (BP) in the presence of a normal BP on conventional measurement at the office. To assess the prevalence of MH among AoC normotensive young patients successfully treated and to evaluate the impact of MH on left ventricular (LV) geometry and function.We studied 76 AoC patients (mean age 14.5±5.7 years, male 64%). According to 24 h ambulatory BP monitoring (ABPM) our sample was divided in real normotensive patients (Group RN, n=40) and MH patients (Group MH, n=36). There was an increased pressure gradient in the aortic arch (15 mm Hg±4 vs 13 mm Hg±4.7, P<0.05), increased LV mass (51 g m(-2.7)±13 vs 46 g m(-2.7)±12, P<0.05), in MH AoC patients. Regional longitudinal deformation properties of the basal septal segment (-15%±2.4 vs -20%±5, P<0.01) and LV twist values (14°±1.6 vs 12°±1.9, P<0.0001) were reduced in the MH group. There is a high prevalence of MH in young patients with repaired AoC, which is associated with abnormal LV structure and function. Clinicians should consider 24 h ABPM measurements in apparently normotensive patients followed up for AoC repair.

Usefulness of Stenting in Aortic Coarctation in Patients With the Turner Syndrome

We report our experience with stent implantation for treatment of aortic coarctation in patients with Turner syndrome. Ten consecutive patients with Turner syndrome and aortic coarctation (median age 12 years, range 9 to 24) underwent coarctation stenting. Of these, 6 patients were treated for isolated coarctation and 4 for recurrent coarctation (3 after balloon dilation and 1 after balloon dilation and surgical repair). Bare metal stents were implanted in 7 patients and covered stents in 3. Immediately after stent implantation, peak systolic gradient decreased from 46.1 ± 24.3 to 1.9 ± 2.1 mm Hg (p <0.001). Aortic diameter at coarctation site increased from 5.1 ± 3.2 to 15.3 ± 2.0 mm after stenting (p <0.001). There were no deaths or procedure-related complications. During a median follow-up of 30.5 months, no patient developed restenosis. Two patients developed late aortic aneurysms at the coarctation site. In conclusion, stent implantation for aortic coarctation in patients with Turner syndrome appears to be a safe and effective alternative to surgical repair. Larger cohorts and longer-term follow-up are required to determine the effects of the procedure on the aortic wall.

Clinical evaluation of aortic coarctation with 4D flow MR imaging

To show that 4D Flow is a clinically viable tool for evaluation of collateral blood flow and demonstration of distorted blood flow patterns in patients with treated and untreated aortic coarctation. Time-resolved, 3D phase contrast magnetic resonance imaging (MRI) (4D Flow) was used to assess blood flow in the thoracic aorta of 34 individuals: 26 patients with coarctation (22 after surgery or stent placement) and eight healthy volunteers. Direct comparison of blood flow calculated with 2D and 4D phase contrast data at standard levels for analysis in coarctation patients showed good correlation and agreement (correlation coefficient r = 0.99, limits of agreement = -20% to 20% for collateral blood flow calculations). Abnormal blood flow patterns were demonstrated at peak systole with 4D Flow visualization techniques in the descending thoracic aorta of patients but not volunteers. Marked helical flow was seen in 9 of 13 patients with angulated aortic arch geometries after coarctation repair. Vortical flow was seen in regions of poststenotic dilation. 4D Flow is a fast and reliable means of evaluating collateral blood flow in patients with aortic coarctation in order to establish hemodynamic significance. It also can detect distorted blood flow patterns in the descending aorta after coarctation repair.

Computational simulations from cardiac magnetic resonance imaging reveal altered hemodynamics in coarctation patients previously treated by patch aortoplasty

Computational simulations from cardiac magnetic resonance imaging reveal altered hemodynamics in coarctation patients previously treated by patch aortoplasty

Left Ventricular Dysplasia is Associated with Congenital Aortic Coarctation

Objective: To determine the incidence of left ventricular dysplastic features in a large cohort of patients with congenital coarctation of the aorta (CoA) using cardiovascular magnetic resonance imaging (CMRI). Background:CoAhas been shown to be associatedwith other features of left heart obstruction including bicuspid aortic valve, subaortic stenosis, parachutemitral valve and supravalvularmitral ring.Theoccurrenceof associated left ventricular dysplastic features as part of the coarctation spectrum has not been fully appreciated. Method: We retrospectively reviewed 282 CMRI examinations of patients with CoA, the majority of whom had undergone prior surgical repair. Imaging was performed on a 1.5 T MRI (GE). A full series of steady state free precession images were obtained in short and long axis and four chamber views. Myocardial delayed enhancement was performed in a limited number of patients. Findings: Features of left ventricular dysplasia in coarctation patients included

Surveillance of cardiovascular risk in the normotensive patient with repaired aortic coarctation

BackgroundRepaired coarctation of the aorta is associated with premature atherosclerosis and an increased risk of cardiovascular events even in normotensive subjects. To date clinical risk stratification has focused on brachial blood pressures ignoring the complex pulsatility of the aortic wave form. The aim of this study was to assess components of this pulsatility in a clinical setting and to suggest possible techniques to improve risk stratification. MethodsThis was a prospective study recruiting patients from a tertiary referral centre. Pulse wave morphology was assessed using applanation tonometry. B-mode ultrasound and cardiac magnetic resonance were used to assess carotid intimal–medial thickness and left ventricular mass. ResultsForty-six subjects with repaired coarctation of the aorta (range 16–62 years; mean 31 years) and 20 matched controls were studied. Baseline brachial systolic and diastolic blood pressures were not statistically different between the 2 groups. Peripheral (62.5 mmHg (11.3) vs. 50.6 mmHg (15.0), p=0.0008) and central (34.5 mmHg (7.7) vs. 28.7 mmHg (4.7), p=0.005) pulse pressures were elevated in the coarctation patients compared to controls. The reflected pressure wave returned to the ascending aorta earlier in the coarctation group (p=0.007) and the tension time index (TTI) was increased (p=0.03). The sub-endocardial viability index (SVI) was reduced in the coarctation subjects (159 (33) vs. 186 (31)%; p=0.009) but there was no differences in central augmentation index (p=0.35). ConclusionsThis study demonstrates that there are patients with repaired coarctation who have an excellent mid-term outcome free from ventricular hypertrophy, carotid intima medial thickening and with relatively preserved vascular reactivity. However even in this “best outcome” cohort there were abnormal vascular characteristics that may predispose to increased cardiovascular risk. Simple non-invasive investigations can more extensively characterise these sub-clinical abnormalities and by utilised in long-term surveillance.

Abdominal Aortic Pulsed Wave Doppler Patterns Reliably Reflect Clinical Severity in Patients with Coarctation of the Aorta

There are situations in which standard echocardiography does not adequately define the aortic arch. We sought to determine what additional information could be gained by analyzing abdominal aortic Doppler flows in coarctation. Previously recorded echocardiographic data were reviewed in 70 controls and 248 patients with coarctation, including abdominal aortic values for pulsatility indices, pulse delay, and presence of early diastolic reversal. Ability of these variables to distinguish controls from coarctation patients and to assess coarctation severity was assessed. Corrected maximum instantaneous gradient and all abdominal aortic flow variables were associated with severity of obstruction. Early diastolic reversal was universally absent in significant coarctation. Threshold values for other parameters associated with significant obstruction were: corrected pulse delay >or=3.4 msec(1/2), pulsatility index <2.0, and systolic to diastolic velocity ratio <3.6. A combined abdominal aortic "variable" (absence of early diastolic reversal and corrected pulse delay >or=2.8 msec(1/2)) was found to be the best predictor of clinical coarctation status (positive predictive value = 93%, negative predictive value = 88%). In the absence of a ductus arteriosus, abdominal aortic Doppler parameters can reliably predict the presence of significant coarctation. When early diastolic reversal was present, obstruction was always absent. Lack of early diastolic reversal with a prolonged pulse delay was the best predictor of significant obstruction. Abdominal aortic Doppler evaluation should become a routine part of the evaluation of patients with known or suspected coarctation.

Evaluation of Bicuspid Aortic Valve and Aortic Coarctation With 4D Flow Magnetic Resonance Imaging

Time-resolved, 3D, phase-contrast magnetic resonance imaging (4D flow) is an effective means of evaluating dynamic multidirectional blood flow in the thoracic aorta.1 We have used the technique for characterization of abnormal flow features in a 14-year-old boy with aortic coarctation and bicuspid aortic valve (BAV) but without evidence of aortic stenosis or regurgitation. In addition to the expected flow disturbance in the region of the juxtaductal coarctation (Figure 1), we show an unusual flow feature in the ascending aorta that has not been previously reported in this clinical setting and that may be unique to BAV: 2 discrete nested helices of midsystolic blood flow in a nonaneurysmal aorta (Figure 2). Figure 1. Fourteen-year-old boy with BAV and aortic coarctation. A, Three-dimensional contrast-enhanced magnetic resonance angiography that demonstrates a focal juxtaductal coarctation and prominent internal mammary and …