Successful Repair Of Coarctation Research Articles

Vascular Wall Changes and Arterial Functions in Children with Surgically Repaired Aortic Coarctation.

Objective: We investigated arterial functions by measuring carotid–intima–media thickness, flow-mediated dilatation of the brachial artery, and distensibility and stiffness of the abdominal aorta as early indicators of cardiovascular risk in children followed up after coarctation repair.Materials and Methods: Twenty patients with successful repair of coarctation and 27 healthy children were investigated. Two-dimensional echocardiographic images, and carotid and brachial ultrasound studies were performed. Results: The ages of the study group ranged from 5.3 to 22 years, and those of the control group from 7 to 17 years. The age at time of surgery was between 0.23 and 257 months. Average follow-up duration after repair was between 11 and 257 months. The stiffness index of the abdominal aorta was significantly higher in patients with coarctation repair than controls (mean = 0.625 ± 0.41, mean = 0.11 ± 0.73; P = .007). Flow-mediated dilatation of the brachial artery in the first minute decreased significantly among the patients (mean = 4.5 ± 2.7, mean = 6.9 ± 4.5; P = .005). Age of the patients had a negative correlation with distensibility of the abdominal aorta (r = −0.572; P = .008) and a positive correlation with stiffness of abdominal aorta (r = 0.566, P = .009).Conclusion: This study suggested that vascular wall changes in children and adolescents can be seen even after successful coarctation repair and may progress toward overt atherosclerosis at older ages.

Carotid atherosclerosis, changes in tissue remodeling and repair in patients with aortic coarctation

Background and aimsAfter aortic coarctation (CoA) repair, patients still suffer from cardiovascular complications. The aim of this study was to measure cardiovascular markers, intima-media thickness (IMT) and plaques in controls and patients with CoA. MethodsSixty-four patients with CoA (66% male, mean age 48 ± 15 years) and controls (54% men, mean age 47 ± 16 years) underwent ultrasound of their arteries. A multiplex platform to analyze circulating blood levels biomarkers reflecting inflammation, tissue remodeling and repair was used. ResultsIn men following CoA repair, a significantly increased carotid bulb IMT was observed in comparison to the control group (1.05 [0.72–1.24] vs. 0.67 [0.59–0.95] mm; p = 0.003). Median common carotid artery (CCA) IMT was increased in men compared to controls (0.82 [0.61–0.97] mm vs. 0.58 [0.53–0.76] mm, p < 0.003) and in women compared to controls (0.83 [0.70–0.92] vs. 0.60 [0.55–0.69], p < 0.004). CoA demonstrated an independent association with IMT in both men and women. Men with CoA were also more likely to have a plaque in their carotid arteries (p = 0.010). In women with CoA, we observed significantly lower levels of stem cell factor (SCF, p = 0.004) while in men with CoA we observed significantly lower levels of matrix metalloproteinase-3 (MMP-3, p = 0.048), tumor necrosis factor receptor 1 (TNF-R1, p = 0.032), tumor necrosis factor receptor superfamily member 10B (TRAIL-R2, p = 0.019) and monocyte chemotactic protein 1 (MCP-1, p = 0.015). ConclusionsThis is the first study to show that despite successful CoA repair, patients have more carotid atherosclerosis than can be explained by changes in tissue remodeling and repair.

Multi-centre cross-sectional study on vascular remodelling in children following successful coarctation correction.

Coarctation of the aorta is an arteriopathy with life-long sequelae, with remarkably increased cardiovascular events in young adults even after successful repair and despite blood pressure status. There are data on arterial remodelling in adults after coarctation correction, however, these data are scarce in childhood. Thus, the aim of this cross-sectional study was to evaluate changes in arterial wall function and morphology in children following successful coarctation repair and to compare these changes among patients with different blood pressure status and coarctation correction modes. Blood pressure status, echocardiographic parameters, arterial wall structure and stiffness, endothelial function and central blood pressure measurements were evaluated in 110 children aged 6-18 years following successful coarctation repair with right arm blood pressure not exceeding leg blood pressure by ≥20 mmHg. The prevalence of arterial hypertension was 50%. The mean carotid intima-media thickness SDS was 3.1 ± 1.5 and above 1.65 SDS in 91 of 110 patients. Increased right carotid intima-media thickness was associated with left ventricular hypertrophy, office blood pressure difference between leg and right arm, recoarctation in the past and interventional coarctation correction. Increased local common carotid artery stiffness was associated with increased pulse pressure and central systolic blood pressure. Potentially decreased endothelial function was related to a slight increase of peak and mean systolic gradient in the descending aorta. After successful coarctation repair and with a low blood pressure gradient, children still have a high prevalence of arterial hypertension and significant arterial remodelling, indicating accelerated biological age and advanced arteriosclerosis.

Bicuspid aortic valve: different clinical profiles for subjects with versus without repaired aortic coarctation

ObjectivesA small proportion of patients undergoing bicuspid aortic valve (BAV) intervention have had prior repair of aortic coarctation (CoA). We aimed to describe phenotypic differences between BAV patients, comparing those...

Is the burden of late hypertension and cardiovascular target organ damage in children and adolescents with coarctation of the aorta after early successful repair different to healthy controls?

Cardiovascular morbidity is high in patients with coarctation of aorta even after successful repair. This study aimed to assess the frequency of late hypertension and the relationship between ambulatory hypertension and cardiovascular target organ damage in children and adolescents after early and successful repair of coarctation of the aorta. Twenty-five children and adolescents (mean age 13.5 ± 3.43 years) with repaired coarctation of the aorta (median age at repair 4 months, arm-leg gradient <20 mmHg) and 16 healthy controls were included. Office and ambulatory blood pressure, pulse wave velocity, and left ventricular mass index were assessed. Both day- and night-time systolic blood pressure standard deviation score and left ventricular mass index were significantly higher in patients compared to controls (p ≤ 0.001 for all), whereas pulse wave velocity did not differ. The prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy were 40, 28, and 24%, respectively. Left ventricular mass index was higher in patients with sustained hypertension, masked hypertension, and normotension compared to controls (p < 0.05). In multivariate analysis, higher night-time systolic blood pressure standard deviation score was the only independent predictor of left ventricular mass index. The present study reveals a high prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy in children and adolescents with coarctation of the aorta even after early and successful repair. Ambulatory blood pressure monitoring should be considered to diagnose hypertension. All coarctation of aorta patients should be followed up lifelong and encouraged to establish a healthy lifestyle starting from childhood.

Prevalence of arterial hypertension, hemodynamic phenotypes, and left ventricular hypertrophy in children after coarctation repair: a multicenter cross-sectional study.

This study aimed to evaluate hemodynamic phenotypes and prevalence of left ventricular hypertrophy in children after coarctation repair with right arm and leg blood pressure difference < 20 mmHg. Secondary objectives were analysis of effects of age at intervention, residual gradient across the descending aorta, and type of correction. Blood pressure status and left ventricular hypertrophy were diagnosed according to European Society of Hypertension 2016 guidelines. Of 90 patients with a median age 12.5 (8.9-15.8) years, 8.5 (6.0-11.8) years after coarctation repair who were included, 42 (46.7%) were hypertensive. Isolated systolic hypertension dominated among 29 hypertensive patients with uncontrolled or masked hypertension (25 of 29; 86.2%). Of the 48 patients with office normotension, 14.6% (7) had masked hypertension, 8.3% (4) had ambulatory prehypertension, and 54.2% (26) were truly normotensive. Left ventricular hypertrophy was diagnosed in 29 patients (32.2%), including 14 of 42 (33.3%) hypertensive and 15 of 48 (31.3%) normotensive patients. The peak systolic gradient across the descending aorta was greater in hypertensive subjects (33.3 ± 12.7 mmHg) compared with normotensive subjects (25 ± 8.2 mmHg, p = 0.0008). Surgical correction was performed earlier than percutaneous intervention (p < 0.0001) and dominated in 40 of 48 (83.3%) normotensive versus 24 of 42 (57.1%) hypertensive patients (p = 0.006). Arterial hypertension with isolated systolic hypertension as the dominant phenotype and left ventricular hypertrophy are prevalent even after successful coarctation repair. Coarctation correction from the age of 9 years and older was associated with a higher prevalence of hypertension.

Successful Repair of Coarctation of the Aorta and Obstructive Total Anomalous Pulmonary Veins Connection in a One-Day-Old Newborn

Abstract A clinical case of successful repair of a combination of total anomalous pulmonary venous connection (TAPVC) with collector stenosis in one-day-old newborn. Only one case of successful correction of such a pathology is reported previously. The operation was performed with cardiopulmonary bypass and temporary antegrade brain perfusion. The narrowed aortic area was resected, the integrity of the aortic arch was restored using an extended anastomosis, and TAPVC correction was performed using a “sutureless technique”. The postoperative period was uneventful. The newborn was discharged from hospital on the 12th day.

Exercise Capacity in Asymptomatic Adult Patients Treated for Coarctation of the Aorta

A reduced exercise capacity is a common finding in adult congenital heart disease and is associated with cardiovascular morbidity and mortality. However, data on exercise capacity in patients after repair of coarctation of the aorta (CoA) are scarce. Furthermore, a high rate of exercise-induced hypertension has been described in CoA patients. This study sought to assess exercise capacity and blood pressure response in asymptomatic patients long-term after CoA repair in relation to left ventricular and vascular function. Twenty-two CoA patients (age 30 ± 10.6 years) with successful surgical repair (n = 12) or balloon angioplasty (n = 10) between 3 months and 16 years of age with a follow-up of > 10 years underwent cardiopulmonary exercise testing at a mean follow-up of 23.9 years. Exercise capacity (peak oxygen uptake; VO2peak) and blood pressure response were compared to age- and gender-matched reference values. Left ventricular function and volumetric analysis was performed using cardiovascular magnetic resonance imaging. CoA patients showed preserved exercise capacity compared to the healthy reference group, with a VO2peak of 41.7 ± 12.0 ml/kg/min versus 44.9 ± 6.7 ml/kg/min. VO2peak/kg showed a significant association with age (p < 0.001) and male gender (p ≤ 0.001). Exercise-induced hypertension occurred in 82% of CoA patients, and was strongly related to left ventricular mass (p = 0.04). Of the 41% of patients who were normotensive at rest, 78% showed exercise-induced hypertension. No significant correlation was found between peak exercise blood pressure and age, BMI, age at time of repair, LVEF, or LV dimensions. Exercise capacity is well preserved in patients long-term after successful repair of coarctation of the aorta. Nevertheless, a high number of patients develop exercise hypertension, which is strongly related to systemic hypertension. Regular follow-up, including cardiopulmonary exercise testing, and aggressive treatment of hypertension after CoA repair is strongly advised.

P735Utility of 18F-FDG PET/CT imaging in detecting aortic wall inflammation of adult patients after successful coarctation repair

P735Utility of 18F-FDG PET/CT imaging in detecting aortic wall inflammation of adult patients after successful coarctation repair

The Scope of Cardiac Surgery at a National Cardiothoracic Center of Excellence (NCTCE) in Nigeria: A 3-Year Review

Background: The heart with its intrinsic and indispensable function is a vital organ in the human body. Surgical management of diseases affecting it was initially thought to be incompatible with life. Surgeons who desired the respect of their colleagues were barred from operating on the heart, on the ground that human knowledge is very limited in that area. This notion was however changed in 1893 when laceration in the left ventricle was successfully repaired at Chicago Provident Hospital in USA. Three years later, the same feat was successfully reproduced several times in Frankfurt Germany. Also by 1938 in Boston Children Hospital, Massachusetts, USA, Robert Gross successfully interrupted a PDA in a 7-yearold child. This was followed by successful repair of coarctation of the aorta and the famous blue baby operation, all in 1944, thus unequivocally, proving that successful operation on the heart was tenable in the western world. On the contrary, in the early 1940s and 1950s, cardiac diseases requiring surgery for whole or part of their management were thought to be rare in Nigeria in particular and Africa in general. The establishment of cardiac registry at UCH, Ibadan in western Nigeria, in 1964 revealed all types of cardiac diseases including those requiring surgical intervention. Today, many cardiac diseases are treated by surgery in NCTCE, UNTH, Enugu and other centers in Nigeria. The western world is abandoning the perfected conventional open access cardiac surgery for minimal invasive cardiac surgery with comparable results vis-a-vis efficacy. Aim/Objective: To determine the scope of cardiac surgery at the National Cardiothoracic Center of Excellence and compare same with the continental and international standard. Materials and method: This is a retrospective study. From March, 2013 to February, 2016, (3 years) all case records of those that underwent cardiac operations at NCTCE were retrieved and analyzed using simple arithmetic method. Those who were diagnosed but could not be operated on, who either died or referred abroad due to the complexity of their cardiac diseases were excluded from the study. Results: During this period, spanning 3 years, a total of 209 cardiac operations were carried out, about 69.7 cases per year. The age range of the patients that were operated was 1 year to 72 years with a mean of 2.9. Of this number, 128 (61.2%) were males and 81 (38.8%) were females with a ratio of 1.6: 1. Also, open heart procedures constituted about 125 (59.6%), while closed heart procedures were 84 (40.4%). Out of 125 cases of open heart procedures, 79 (63.1%) were for acquired adult heart diseases while 46 (36.9%) were for congenital heart diseases. Mean hospitalization at the intensive care unit was 72 hours while the mean hospital stay was ten days. Mortality was about 25 (12.0%). Conclusion: From the results, NCTCE carried out significant number of cases during the period when compared to previous years. However, the youngest age group was 1 year old, suggesting that those below that age group were referred out. The mean hospital stay is relatively longer, suggesting that complications might have contributed.

Off-pump midline repair of coarctation of aorta when associated with intracardiac defects: an alternate approach for single-stage correction.

Strategies for the optimal surgical management of coarctation of aorta with associated intracardiac defects continue to be debated upon. We describe a previously unreported surgical technique for the management of this combination of defects and present our early results. Thirty-one patients (median age: 73 days and median weight: 3.9 kg) underwent repair of coarctation of aorta with associated cardiac defects, in a single-stage, through a midline sternotomy. Twenty-eight patients had associated distal arch hypoplasia and 1 had a type I interruption. The coarctation and when necessary, the distal arch, was repaired prior to the institution of cardiopulmonary bypass. Cardiopulmonary bypass was subsequently instituted for the repair of the associated intracardiac defects. All patients had successful repair of coarctation and distal arch hypoplasia without the need for rescue cardiopulmonary bypass. In addition, 29 patients had closure of one or more ventricular septal defects, 3 patients had an arterial switch procedure and 1 had repair of double outlet right ventricle. Repair of supramitral ring and subaortic membrane was performed in another 3 patients. One patient underwent concomitant mitral valve repair. There was one in-hospital mortality (3.22%) due to fulminant sepsis. The median follow-up was 29.5 months (range: 1-58 months). There was no late mortality. Two patients required balloon dilatation for recurrent coarctation. Overall event-free survival rate was 88.5% at both 2 and 3 years of follow-up after surgery. However, intervention-free survival at 2 and 3 years of follow-up was 91.7% among the survivors. Repair of coarctation and distal arch hypoplasia or type I arch interruption is feasible prior to the institution of cardiopulmonary bypass without the use of hypothermic circulatory arrest or regional cerebral perfusion, with acceptable and reproducible results.

Aortic stiffening and its impact on left atrial volumes and function in patients after successful coarctation repair: a multiparametric cardiovascular magnetic resonance study.

BackgroundThe increased cardiovascular morbidity of adults with late repair of aortic coarctation (CoA) has been well documented. In contrast, successful CoA repair in early childhood has a generally good prognosis, though adverse vascular and ventricular characteristics may be abnormal, which could increase long-term risk. This study sought to perform a comprehensive analysis of aortic elasticity and left ventricular (LV) function in patients with aortic coarctation (CoA) using cardiovascular magnetic resonance (CMR). In a subgroup of patients, we assessed structure and function of the common carotid arteries to probe for signs of systemic vascular remodeling.MethodsFifty-one patients (median age 17.3 years), 13.9 ± 7.5 years after CoA repair, and 54 controls (median age 19.8 years) underwent CMR.We determined distensibility and pulse wave velocity (PWV) at different aortic locations. In a subgroup, common carotid artery distensibility, PWV, wall thickness and wall area were measured. LV ejection fraction (EF), volumes, and mass were measured from short axis views. Left atrial (LA) volumes and functional parameters (LAEFPassive, LAEFContractile, LAEFReservoir) were assessed from axial cine images.ResultsIn patients distensibility of the whole thoracic aorta was reduced (p < 0.05) while PWV was only significantly higher in the aortic arch (p < 0.01). Distensibility of the descending aorta at the level of the pulmonary arteries and PWV in the descending aorta, both correlated negatively with age at CoA repair. LA volume before atrial contraction and minimal LA volume were higher in patients (p < 0.05). LAEFPassive and LAEFReservoir were reduced (p < 0.05), and LAEFReservoir correlated negatively with aortic arch PWV (p < 0.05). LVEF, volumes and mass were not different from controls. Carotid wall thickness and PWV were higher in patients compared to controls (p < 0.05).ConclusionsPatients after CoA repair have impaired bioelastic properties of the thoracic aorta with impact on LV diastolic function. Reduced descending aortic elasticity is associated with older age at time of CoA repair. The remodeling of the common carotid artery in our sub-study suggests systemic vessel wall changes.

第5大動脈弓遺残に第4大動脈弓離断を合併した大動脈縮窄の2手術例

第5大動脈弓遺残に第4大動脈弓離断を合併した大動脈縮窄の2手術例

Abnormalities of aortic arch shape, central aortic flow dynamics, and distensibility predispose to hypertension after successful repair of aortic coarctation.

Systemic hypertension (HT) is a major long-term complication even after successful repair of aortic coarctation (CoA), and many factors are involved in this pathophysiology. To investigate the role of abnormalities in the aortic arch shape, central aortic flow dynamics, and distensibility in developing HT after successful repair of CoA. We selected a group of 26 normotensive patients (mean age 16.9±7.3 years, range 9-32 years) with anatomically successful repair of CoA among 140 patients regularly followed after repair of CoA and analyzed their last clinical and echocardiographic data. Bicycle exercise test and ambulatory blood pressure monitoring (ABPM) were also obtained. Mean age at surgical repair was 3.2±3.9 years (range 10 days-15 years); 12 patients underwent surgical correction during the first year of life. Repair of CoA was performed by end-to-end anastomosis (TT) in 23 patients (extended TT in 6 patients with arch hypoplasia), patch aortoplasty in 2, and subcalvian flap aortoplasty in 1. The postsurgical follow-up was 13.8±7.2 years (range 3.5-29.4 years). The shape of the aortic arch was defined by magnetic resonance imaging (MRI) on this global geometry (normal-gothic-crenel), ratio of the height-transverse diameter (A/T), percentage of residual stenosis, and growth index of the transverse arch segments. Flow mapping by phase-contrast imaging in the ascending and descending aorta was performed in order to measure the systolic waveforms and central aortic distensibility. Twenty normal age-matched patients submitted to the same MRI protocol were used as controls. Six patients were found to have a gothic and 20 a normal aortic arch shape. Patients with gothic aortic arch shape had an increased A/T ratio (0.80±0.07 vs 0.58±0.05, P<.001), a greater loss of systolic wave amplitude across the aortic arch (43%±2% vs 34%±5%, P<.001), and a lower distensibility of the ascending aorta compared to those with normal shape (4.87±1.06 mm Hg(-1)×10(-3) vs 7.20±1.73 mm Hg(-1)×10(-3); P=.005) and controls (4.87±1.06 mm Hg(-1)×10(-3) vs 8.57±1.71 mm Hg(-1)×10(-3); P<.001). The maximal systolic blood pressure (SBP) on exercise as well as SBP, diastolic blood pressure, and percentage of >135 mm Hg on ABPM were higher in the gothic than in the normal arch group. There was a correlation between nocturnal SBP, 24 hours pulse pressure on ABPM in the whole group, and different MRI variables (A/T, distensibility of the ascending aorta, and percentage of loss of systolic wave amplitude). In the long-term follow-up after CoA repair, a gothic arch shape is associated with a decreased ascending aorta distensibility with an increased loss of systolic wave amplitude across the aortic arch. These findings explain at least in part the association between this abnormal arch geometry and late HT at rest and on exercise, left ventricular hypertrophy, and adverse cardiovascular outcome in this group of young adults with successful repair of CoA.

Renal sympathetic denervation for resistant hypertension in a child after aortic coarctation surgery

Canniffe et al. report that the current prevalence of hypertension lateafter successful coarctation repair is 32.5% (range 25 –68%) [1].Ahigherage at the time of surgery, a longer time of follow up and a better de fini-tion of hypertension are factors that increase the prevalence. There areinsufficient long term data to adequately compare catheter based inter-vention to surgical procedures for native coarctation in terms of the lateprevalence of hypertension. A causative role of the autonomic nervoussystemresultinginarterialhypertensionmaybeoverlooked.Wepresentthe first paediatric patient in whom successful interventional renalsympathetic denervation improved postcoarctation repair hypertension.A 16 yearold boy wasoperated at the age of 13 years. Hypertensionandaorticcoarctationwere firstnoticedduringsurgeryforsportsinjury.Arm/leg pressure gradient was 90 mm HG (194/103 vs 104/69). MRimaging showed severe coarctation and hypoplastic isthmus (Fig. 1).Surgery comprised of proximal descending aorta replacement with an18 mm Dacron tube (Uni-Graft™, B. Braun, Berlin, Germany). Longterm postoperative hypertension with average blood pressures of upto 171/92 mm Hg occurred in circadian monitoring despite fourfoldmedication (Fig. 2). A careful examination of his autonomic nervoussystem showed a highly reduced heart rate variability indicatingsympathetic predominance. Adding carvedilol improved heart rate var-iabilityonly.Maximal Doppler flow was2.8 m/sin the aorticgraft with-outarm/legpressuregradient.Theboysufferedfromchronicfatigueandheadache. He was also found to have an intracranial aneurysm, wasobese (BMI 28.6 kg/m

A tetrad of bicuspid aortic valve association: A single-stage repair

We report a 27 years old male who presented with a combination of both congenital and acquired cardiac defects. This syndrome complex includes congenital bicuspid aortic valve, Seller's grade II aortic regurgitation, juxta- subclavian coarctation, stenosis of ostium of left subclavian artery and ruptured sinus of Valsalva aneurysm without any evidence of infective endocarditis. This type of constellation is extremely rare. Neither coarctation of aorta with left subclavian artery stenosis nor the rupture of sinus Valsalva had a favorable pathology for percutaneus intervention. Taking account into morbidity associated with repeated surgery and anesthesia patient underwent a single stage surgical repair of both the defects by two surgical incisions. The approaches include median sternotomy for rupture of sinus of Valsalva and lateral thoracotomy for coarctation with left subclavian artery stenosis. The surgery was uneventful. After three months follow up echocardiography showed mild residual gradient across the repaired coarctation segment, mild aortic regurgitation and no residual left to right shunt. This patient is under follow up. This is an extremely rare case of single stage successful repair of coarctation and rupture of sinus of Valsalva associated with congenital bicuspid aortic valve.

Rationale and design of a trial on the effect of high dose statins on cardiovascular risk in adults after successful coarctation repair

BackgroundHMG-coA-reductase-inhibitors (statins) have been proven to reduce atherosclerosis progression as observed by carotid intima-media thickness in patients with known coronary heart disease, independent of lipid lowering. Cardiovascular complications are common in patients after successful coarctation repair. The effect of statins on cardiovascular risk in adults after successful coarctation repair has not yet been established. MethodsWe designed a multicentre, prospective, randomised, open label trial to evaluate the effect of the HMGcoA-reductase-inhibitor (Atorvastatin) on atherosclerotic progression in adult post-coarctectomy patients. The primary endpoint in this study is the carotid intima-media thickness as measured by Bmode ultrasonography of the carotid arteries. ConclusionThis large prospective, randomised, open label trial will establish the effect of HMG-coA-reductase inhibitors (Atorvastatin) on cardiovascular risk in adult patients after successful coarctation repair.

Vascular remodeling in adults after coarctation repair: impact of descending aorta stenosis and age at surgery.

BackgroundPatients after successful repair of coarctation of aorta (CoAo) are at risk of hypertension at rest and associated end-organ damage. The aim of the study was to assess arterial stiffness and function in adults after coarctation repair in relation to descending aorta (AoD) residual coarctation and patient’s age at operation.Methods85 patients after CoAo repair (53 males) aged 34.6 ± 10.3 years; median age at operation 0.9 ± 8.2 years. The control group—30 individuals (18 males) at mean age 33.6 ± 8.2 years. The following central parameters: augmentation pressure (AP) and augmentation index (AI) as well as peripheral vascular parameters: flow-mediated dilatation (FMD), nitroglycerin-mediated vasodilatation (NMD), intima-media thickness (IMT) and pulse wave velocity (PWV) were measured.Results47 CoAo-repaired patients were normotensive, and compared to control, they presented higher values of central parameters AP (7.3 ± 4.6 vs. 4.4 ± 3.6 mmHg; p = 0.002) and AI (18.6 ± 10.4 vs. 13.5 ± 4.3%; p = 0.03); as well as the increased PWV (6.8 ± 1.2 vs. 5.4 ± 0.9 m/s; p = 0.003), while IMT was comparable (0.53 ± 0.01 vs. 0.51 ± 0.01 mm; p = 0.06). The vasodilatation was impaired in the normotensive patients: FMD (4.8 ± 2.8 vs. 8.5 ± 2.3%; p = 0.00003) and NMD (11.3 ± 4.6 vs. 19.8 ± 7.2%; p = 0.00001). The comparison of recoarctation (46, 54%) to non-recoarctation (39, 46%) patients did not reveal any significant differences in resting systolic and diastolic pressures, as well as the values of AI and the peripheral vascular parameters; the value of AP was higher in the recoarctation patients (10.5 ± 6.9 vs. 7.5 ± 4.1; p = 0.02) and correlated positively with the gradient across AoD (r = 0.295, p = 0.01). There was no significant linear correlation between age at the time of surgery and any of peripheral arterial parameters.ConclusionsResidual stenosis in AoD does not affect the arterial vasodilatation nor stiffness in patients after CoAo repair. Early operation has no impact on peripheral vascular remodeling or central pressure which supports the claim that coarctation of the aorta is a systemic vascular disorder which leads to progressive vascular and end-organ damage despite early correction.

Early Detection of Anomalous Origin of Left Coronary Artery from the Right Pulmonary Artery After Successful Repair of Critical Coarctation of the Aorta

Left ventricular (LV) function is impaired by increased afterload in neonates with severe coarctation of the aorta, which may result in endocardial fibroelastosis. Repair of the coarctation usually solves the problem, with LV function normalizing after a few weeks. This report describes a patient who underwent successful repair of critical coarctation with normalization of LV function despite signs of endocardial fibroelastosis but with persisting elevation of cardiac troponin T. Cardiac catheterization showed the rare coincidence of anomalous origin of left coronary artery from the right pulmonary artery (ALCAPA) and coronary sinus orifice atresia with left superior vena cava.

Exercise Testing is Useful to Screen for Residual Coarctation in Children

Twenty-seven children (ages 8 to 18 years) who had a history of successful coarctation repair (mean transisthmic Doppler gradient [TI-D] <20 mmHg at rest) and had not received antihypertensive medications underwent echocardiogram at rest and immediately following peak exercise with a standard treadmill test. All were normotensive or had isolated systolic hypertension at rest. Right arm-ankle (RA-A) systolic blood pressure (SBP) difference and TI-D were measured. Eleven children (41%) demonstrated exercise-induced hypertension (peak exercise SBP >98th percentile per body surface area) and 17 (63%) had a mean TI-D > or =40 mmHg. Eight underwent MRI or angiography; a residual coarctation (RCoA) was confirmed in three and excluded in five. RCoA was excluded in three other children who were normotensive at rest and had normal postoperative echocardiographic findings. No individual test (SBP, RA-A SBP, TI-D) at rest or with exercise testing was statistically useful to identify RCoA. However, the requirement for a RA-A SBP difference > or =20 mmHg and a TI-D > or =40 mmHg during exercise testing was a useful screen: RCoA was present in three of five children who fit these criteria but was absent in six of six who did not.