Coartación de aorta en pediatría: características clínicas de niños y adolescentes asistidos en el Centro Hospitalario Pereira Rossell / Coarctation of the aorta in pediatrics: clinical characteristics of children and adolescents treated at the Pereira Rossell Hospital Center / Coarctação da aorta em pediatria: características clínicas de crianças e adolescentes atendidos no Centro Hospitalar Pereira Rossell

La coartación de aorta (CoAo) es una cardiopatía congénita caracterizada por un estrechamiento de la aorta descendente distal al origen de la arteria subclavia izquierda, lo que determina una obstrucción al flujo sanguíneo. Es imperativo realizar un diagnóstico precoz y tratamiento oportuno para evitar complicaciones y la muerte en algunos casos. Se describen las características clínicas y anatómicas, así como el tratamiento y la evolución en niños menores de 15 años asistidos en un hospital pediátrico de referencia de Uruguay.

Coarctation of the aorta (CoAo) is a congenital heart disease characterized by a narrowing of the descending aorta distal to the origin of the left subclavian artery, which determines an obstruction to blood flow. It is imperative to make an early diagnosis and timely treatment to avoid complications and death in some cases. The clinical and anatomical characteristics are described, as well as the treatment and evolution in children under 15 years of age attended in a reference pediatric hospital in Uruguay.

A coarctação da aorta (CoAo) é uma doença cardíaca congênita caracterizada por estreitamento da aorta descendente distal à origem da artéria subclávia esquerda, o que determina uma obstrução ao fluxo sanguíneo. É imperativo fazer um diagnóstico precoce e tratamento oportuno para evitar complicações e morte em alguns casos. São descritas as características clínicas e anatômicas, bem como o tratamento e a evolução em crianças menores de 15 anos atendidas em um hospital pediátrico de referência no Uruguai.

Hospital Survival After Surgical Repair of Truncus Arteriosus with Interrupted Aortic Arch: Results from a Multi-institutional Database.

Truncus arteriosus (TA) is a major congenital cardiac malformation that requires surgical repair in the first few weeks of life. Interrupted aortic arch (IAA) is an associated malformation that significantly impacts the complexity of the TA operation. The aim of this study was to (1) define the comorbid conditions associated with TA and (2) determine the hospital survival and morbidity of patients with TA with and without an IAA. Data was collected from the Vizient Clinical Database/Resource Manager, formerly University HealthSystem Consortium, which encompasses more than 160 academic medical centers in the United States. The database was queried for patients admitted from 2002 to 2016 who were ≤ 4 months of age at initial admission, diagnosed with TA, and underwent complete surgical repair during that hospitalization. Of the 645 patients with TA who underwent surgery, 98 (15%) had TA with an interrupted aortic arch (TA-IAA). Both TA and TA-IAA were associated with a high prevalence of comorbidities, including DiGeorge syndrome, prematurity, and other congenital malformations. There was no difference in mortality between TA and TA-IAA (13.7-18.4%, p value = 0.227). No comorbid conditions were associated with an increased mortality in either group. However, patients with TA-IAA had a longer post-operative length of stay (LOS) compared to those without IAA (30 versus 40.3 days, p value = 0.001) and this effect was additive with each additional comorbid condition. In conclusion, the addition of IAA to TA is associated with an increased post-operative LOS, but does not increase in-hospital mortality.

Single-stage repair for coarctation with ventricular septal defect: results of 100 cases at a single centre.

OBJECTIVES: The outcomes of the single-stage surgical repair of aortic arch hypoplasia (AAH) and/or coarctation of the aorta (CoA) associated with ventricular septal defect (VSD) remain controversial, especially in a lower middle-income country. This study reports the results of a single-stage repair protocol at our institution for AAH/CoA with VSD using selective cerebral perfusion. METHODS: This retrospective study included 100 consecutive patients who underwent single-stage repair via median sternotomy using selective cerebral perfusion for AAH/CoA with VSD from July 2010 to March 2017. RESULTS: The patients consisted of 65 males and 35 females. The median age of the patients was 67 days (range 4-2266 days); the median weight was 3.8 kg (range 2.1-15 kg). The average cardiopulmonary bypass time was 132 ± 28 min, the aortic cross-clamp time was 92 ± 23 min and the selective cerebral perfusion time was 33 ± 10 min. The survival rate of all patients was 94.7 ± 2.3%, with an in-hospital mortality of 5% and no late mortality at a median follow-up of 37 months (range 4-96 months). Four patients required reoperation due to recoarctation. The overall event-free survival rate following surgery was 87.1%. The median pressure gradient across the anastomosis at the last follow-up was 8.3 ± 2.8 mmHg. Multivariate logistic regression analysis revealed proximal aortic arch obstruction as a predictor of mortality (odds ratio = 3.8). The aortic isthmus diameter was identified as a predictor for reintervention by Cox regression (hazard ratio = 6.7). CONCLUSIONS: Single-stage repair for AAH/CoA with VSD is safe and feasible in a developing country.

Screening for Intracranial Aneurysms in Coarctation of the Aorta: A Decision and Cost-Effectiveness Analysis.

BACKGROUND: Patients with coarctation of the aorta have a high prevalence of intracranial aneurysms (IA) and suffer subarachnoid hemorrhage (SAH) at younger ages than the general population. American Heart Association/American College of Cardiology guidelines recommend IA screening, but appropriate age and interval of screening and its effectiveness remain a critical knowledge gap. METHODS AND RESULTS: To evaluate the benefits and cost-effectiveness of magnetic resonance angiography screening for IA in patients with coarctation of the aorta, we developed and calibrated a Markov model to match published IA prevalence estimates. The primary outcome was the incremental cost-effectiveness ratio. Secondary outcomes included lifetime cumulative incidence of prophylactic IA treatment and mortality and SAH deaths prevented. Using a payer perspective, a lifetime horizon, and a willingness-to-pay of $150 000 per quality-adjusted life-year gained, we applied a 3% annual discounting rate to costs and effects and performed 1-way, 2-way, and probabilistic sensitivity analyses. In a simulated cohort of 10 000 patients, no screening resulted in a 10.1% lifetime incidence of SAH and 183 SAH-related deaths. Screening at ages 10, 20, and 30 years led to 978 prophylactic treatments for unruptured aneurysms, 19 procedure-related deaths, and 65 SAH-related deaths. Screening at ages 10, 20, and 30 years was cost-effective compared with screening at ages 10 and 20 years (incremental cost-effectiveness ratio $106 841/quality-adjusted life-year). Uncertainty in the outcome after aneurysm treatment and quality of life after SAH influenced the preferred screening strategy. In probabilistic sensitivity analysis, screening at ages 10, 20, and 30 years was cost-effective in 41% of simulations and at ages 10 and 20 in 59% of simulations. CONCLUSIONS: Our model supports the American Heart Association/American College of Cardiology recommendation to screen patients with coarctation of the aorta for IA and suggests screening at ages 10 and 20 or at 10, 20, and 30 years would extend life and be cost-effective.

Application of Modified Sliding Anastomosis in the Repair of Aortic Coarctation.

OBJECTIVES: To evaluate the early and midterm results of a modified sliding anastomosis technique in patients with aortic coarctation. MATERIALS AND METHODS: In this study, we reported a new repair method and compared the early and midterm outcome(s) with a conventional surgical approach for the management of patients with aortic coarctation. Forty-eight aortic coarctation patients with a narrowed segment length longer than 2 cm were operated at our department's pediatric surgical division. Excision of the coarctation and end-to-end anastomosis was carried out in twenty-five patients (control group). In contrast, a modified sliding technique was used for twenty-three cases in the observation group. Other accompanying cardiac anomalies simultaneously repaired included ventricular septal defect and patent ductus arteriosus. All patients received 1.5-10 years of postoperative echocardiographic follow-up. RESULTS: This is a retrospective study carried out between January 2005 and June 2018. The study population consisted of forty-eight patients, which included twenty-six male and twenty-two female patients, with an average age of 5.2 ± 1.9 months (range, 28 days to 1 year). There was no mortality. The operative time, the number of intercostal artery disconnection, the drainage volume, and arm-leg systolic pressure gradient postoperation were less in the observation group as compared to the control group (p < 0.05). Also, cases with an anastomotic pressure gradient exceeding 10 mmHg during follow-up were less in the observation group as compared to the control group (p < 0.05). The postoperative complications encountered were chylothorax (control group 2 cases vs. observation group 0) and pulmonary atelectasis (control group 4 cases vs. observation group 1). They all, however, recovered after conservative treatment. Three patients in the control group underwent balloon angioplasty (reintervention) postoperative 2-4 years due to an increase in the anastomotic pressure gradient (>20 mmHg). After reintervention, the anastomotic pressure gradient reduced to 14 mmHg, 15 mmHg, and 17 mmHg, respectively. CONCLUSIONS: For long segment aortic coarctation patients (longer than 2 cm), the use of the modified sliding anastomotic technique effectively helps to retain more autologous tissues, enlarge the diameter of the anastomosis, and decrease anastomotic tension and vascular injury. Therefore, this technique provides a new idea for the surgical treatment of aortic coarctations.

Neonatal management of aortic coarctation with ventricular septal defect: a systematic review and meta-analysis.

INTRODUCTION: Neonatal management of aortic coarctation with ventricular septal defect is still under debate between the one-stage full repair by sternotomy versus the staged repair of the coarctation first by thoracotomy (with or without banding the pulmonary artery) followed later by subsequent closure of the ventricular septal defect. EVIDENCE ACQUISITION: The aim of this review was to synthesize the evidence in literature since 1980 for the neonatal population. A meta-analysis compared mortality between the two strategies. EVIDENCE SYNTHESIS: The analysis did not find a superiority of a strategy over the other regardless of the surgical era studied. Recoarctation rates of both strategies are presented and a management algorithm is suggested. CONCLUSIONS: Instead of comparing between the two strategies, a case-adapted management considering the anatomy of the ventricular septal defect and of the aortic arch is discussed to address this association of lesions though presenting with a wide range of settings.

Retrospective cohort study of prenatally and postnatally diagnosed coarctation of the aorta (CoA): prenatal diagnosis improve neonatal outcome in severe CoA.

Introduction: Prenatal diagnosis of congenital heart disease (CHD) leads to improved outcome but not mortality rate. This may not be the case for coarctation of the aorta (CoA). The objective of this study is to estimate the effect of a prenatal diagnosis of CoA by comparing neonates with CoA by the time of diagnosis.Materials and methods: The study included 38 neonates with CoA diagnosed prenatally and 102 neonates diagnosed postnatally. The postnatal group was divided into two subgroups: (1) severe CoA: symptoms of CoA within the first 7 days (n = 43) and (2) mild CoA: symptoms within the 8-28th day (n = 34). The neonates diagnosed more than 28 days after delivery were excluded from the study (n = 25). Severe CoA was defined as CHD diagnosed postnatally with clinical symptoms that presented in the first week after birth. Mild CoA was defined as CHD that presented clinical symptoms later than 7 days of life.Results: Prostaglandins were initiated at lower doses (p < .001) in the prenatal group. Severe postnatal CoA was associated with more frequent Neonatal Intensive Care Unit (NICU) visits than mild postnatal CoA (p = .005). The length of hospitalization of neonates with severe postnatal CoA was 10 days longer than compared to the prenatal group, but the difference was not statistically significant. The highest mortality rate was in the severe postnatal CoA group (18.6%) which was significantly higher than the mortality rate in the prenatal group (p = .005).Conclusion: 1. Prenatal identification of fetuses at increased risk of developing CoA may reduce mortality and improve outcome only in neonates with severe CoA (symptoms of CoA within the first 7 days after birth); 2. Prenatal diagnosis of severe CoA was associated with lower prostaglandin doses and lower mortality rate.

Long-Term Survival of Patients With Coarctation Repaired During Infancy (from the Pediatric Cardiac Care Consortium).

Patients who undergo coarctation repair during infancy have excellent early survival but long-term survival is unknown. We aimed to describe the long-term survival of patients with coarctation repaired during infancy and determine predictors of mortality. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium for patients with coarctation who underwent surgical repair before 12 months of age between 1982 and 2003. Long-term transplant-free survival was obtained by linkage with the National Death Index and the Organ Sharing Procurement Network. Kaplan Meier survival plots were constructed, and univariate and multivariable analyses were performed to determine predictors of mortality. We identified 2,424 coarctation patients who met inclusion criteria. At 20 years postoperatively, 94.5% of all patients and 95.8% of those discharged after initial operation remained alive, respectively. Significant multivariable predictors of mortality included surgical weight <2.5 kg (hazard ratio [HR] 3.70, 95% confidence interval [CI] 2.19 to 6.24), presence of a genetic syndrome (HR 2.40, 95% CI 1.13 to 5.10), and repair before 1990 (HR 1.91, 95% CI 1.09 to 3.34). None of the other factors examined including age at repair, gender, coarctation type, or surgical approach were found to be statistically significant. Over half of the deaths were due to the underlying congenital heart disease or other cardiovascular etiology. Overall long-term survival of patients who undergo coarctation repair during infancy is excellent. However, patients do experience small continued survival attrition throughout early adulthood. Ongoing monitoring of this cohort is necessary to assess late mortality risk.

A single centre experience with an evolving approach for the repair of coarctation of the aorta.

BACKGROUND: Isolated coarctation of the aorta can be repaired by either lateral thoracotomy or sternotomy approach with end-to-end anastomosis. Most commonly, neonates with coarctation of the aorta also have hypoplasia of the arch, requiring median sternotomy and extended end-to-side anastomosis with arch augmentation. The aim of this study was to describe our experience as the institution adopted the median sternotomy approach for repair, by reviewing complications, mortality, and reintervention. METHODS: Retrospective chart review of 66 patients aged 0-1 year who had arch repair performed by a single surgeon over an 8-year period was performed. Median age at surgery was 22 days (4-232) and median weight was 3.08 kg (1.25-8.0). Forty-one (62%) patients underwent median sternotomy. RESULTS: There was 1 death from a noncardiac cause. Eighteen per cent of our patients were ≤2.5 kg. Vocal cord paresis occurred in 16% of patients under 2.5 kg and 9.5% of patients 2.5 kg or above at the time of surgery. Hypertension at 6-month follow-up was greater in patients under 2.5 kg (44%) than patients 2.5 kg or above (15%). Total surgical reintervention rate was 6%. For patients above 2.5 kg, the surgical reintervention rate was 5.4% and for patients below 2.5 kg, the surgical reintervention rate was 8.3%. CONCLUSION: We concluded that for neonates with coarctation of the aorta and hypoplastic arch, median sternotomy is a safe surgical approach with low morbidity and mortality with the possible advantage of reduced surgical re-intervention and mortality in the population below 2.5 kg.

Risk factors of mortality and recoarctation after coarctation repair in infancy.

OBJECTIVES: The main challenge of aortic coarctation (CoA) repair in infants is to obtain durable results without morbidity. We aimed to describe predictors of aortic arch reintervention after aortic CoA repair. METHODS: Between January 2000 and March 2014, we retrospectively included consecutive infants with isolated CoA or CoA with ventricular septal defect (CoA + VSD) who had surgical repair of the aortic arch before 3 months of age. RESULTS: Five hundred and thirty patients were included: 308 (58%) patients had isolated CoA and 222 (42%) patients had CoA + VSD. Three hundred and eighty-five patients (72.6%) had CoA repair, 51 patients (9.6%) had CoA repair with closure of VSD and 94 patients (17.8%) had CoA repair with pulmonary artery banding. Mean age at operation was 13 ± 1.6 days, with 294 patients (55.5%) operated on before 2 weeks. Median follow-up was 7.57 years. Sixty-one patients (11.5%) needed reintervention on the aortic arch. Freedom from aortic arch reintervention was 90% at 1 year and 88.5% at 5 years. Proportions of aortic arch reintervention were similar in the different surgical strategy groups (P = 0.80). However, in patients receiving prostaglandin E1 (PGE1), the end-to-end repair was at higher risk of recoarctation compared to the extended end-to-side repair (P = 0.033). The risk factors of aortic arch reintervention were age at repair <15 days (P = 0.034) and the need for PGE1 infusion at surgery (P = 0.0043). CONCLUSIONS: CoA repair in young infants has an overall good outcome. The use of PGE1 may modify the aortic arch anatomy and mask the boundaries of the resection to be performed. PGE1 treatment should be studied more specifically in another study to improve preoperative management.

Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta.

OBJECTIVE: To examine the contemporary long-term outcome after coarctation repair. METHODS: This is a retrospective cohort study of 834 patients aged ≥16 years who underwent coarctation repair under single-centre follow-up. Repair was performed at a median age of 3 years (lower-upper quartile: 1 month to 15 years) by surgery in 83% (690/834) and angioplasty/stenting in 17% (144/834). Survival was compared with an age- and gender matched normal population. Other outcomes included arch reintervention, aortic valve intervention, ascending aortic intervention, and residual/re-coarctation and resting hypertension at latest follow-up. RESULTS: After a median follow-up of 27 years (lower-upper quartile: 18-36), there were 38 late deaths (5%, 38/834). Overall survival was 99%, 88% and 65% at 30, 50 and 70 years of age, respectively, significantly reduced compared with a matched normal population (standardised mortality ratio: 3.20, log-rank: p<0.001). Thirty per cent (246/834) required ≥1 arch reintervention, 13% (111/834) an aortic valve intervention and 5% (43/834) an ascending aortic intervention. Freedom from aortic valve and ascending aortic intervention was 83% and 92% at 50 years and 53% and 81% at 70 years of age, respectively. Residual/re-coarctation (gradient ≥25 mm Hg or repair site/diaphragm ratio ≤70%) at latest follow-up was present in 60% (282/474) and resting hypertension in 57% (379/661). CONCLUSIONS: Long-term survival in contemporary adult survivors of coarctation repair is significantly lower than a matched normal population with accelerated decline after the third decade. Nearly 60% of patients eventually develop hypertension, whereas approximately 50% require further invasive cardiovascular treatment by 50 years of age. Our risk-stratifying data may enable personalised follow-up strategies for this common congenital heart condition.

Surgical repair of aortic coarctation in pediatric age: A single center two decades experience.

BACKGROUND: To evaluate early and long-term results of surgical treatment of aortic coarctation (CoAo) in infants and children. METHODS: A retrospective clinical review of patients less than 18 years with CoAo, undergoing surgery between 1995 and 2015. Data were retrieved from our institutional database, to identify preoperative and postoperative characteristics. Statistical analysis was performed by bivariate, Cox's, and logistic regression analysis. RESULTS: Three hundred forty-one consecutive patients (male/female: 192/149; the median age at surgery of 25 days; interquartile range [IQR], 10-143 days) were included; 187 patients were less than 1 month (54.9%); 276 underwent extended end-to-end anastomosis (EEEA) (80.9%). Hypoplastic aortic arch (HAA) occurred in 34.6% and bicuspid aortic valve in 21.1%. The isolated type was present in 249 (73.0%). Significant postoperative complications occurred in 5.6%. Thirty-day mortality was 1.4%. At a median follow-up of 10.2 years (IQR, 6-15 years; FU completeness, 91.2%), there were eight late deaths (2.6%), most in the complex type. Among 298 survivors, 284 (95.3%) were in NYHA class I; 10 (3.0%) were on antihypertensive treatment. Reinterventions on aortic arch occurred only in 4.5%, being HAA a significant risk factor for reoperation ( P = 0.00173). Freedom from mortality and reintervention on aorta at 21 years were 93.5% and 93.6%, respectively. CONCLUSIONS: Surgical repair of CoAo by EEEA without CPBP is a safe and low-risk procedure, concerning either early or late outcomes, despite the presence of HAA and neonatal age can influence recoarctation. Most patients are clinically well in the long-term, and only a few require antihypertensive therapy.

Aortic coarctation repair through left thoracotomy: results in the modern era.

OBJECTIVES: Surgical repair of coarctation of the aorta (CoA) is often possible through left thoracotomy and without the use of cardiopulmonary bypass. Recent studies reporting the outcome after CoA repair through left thoracotomy are limited. Therefore, the aim of this study is to evaluate the results of CoA repair through left thoracotomy in children who were operated on in our centre over the past 21 years. METHODS: From January 1995 to December 2016, 292 patients younger than 18 years underwent primary CoA repair through left thoracotomy at our 2 institutions. Peri- and postoperative data and follow-up data collected from our hospital and the referring hospitals were retrospectively reviewed. RESULTS: Median age at operation was 64 days (range 2 days-17 years). Most patients underwent the resection of the CoA followed by an (extended) end-to-end anastomosis (93%). Six patients died perioperatively and 2 more patients died during the follow-up, of which 7 patients had other major comorbidities. Actuarial survival was 97% at 5 years, 96% at 10 years and 96% at 15 years. Second arch interventions due to recoarctation were performed in 9.9% (n = 29) of patients, consisting of balloon dilatation in all but 2 patients. Recoarctation occurred significantly more often after initial repair in the neonatal period (21%) and could occur as late as 14 years after initial surgery. There were 7 re-recoarctations, and 14% of patients were on hypertensive medication during the follow-up. CONCLUSIONS: Repair of CoA through left thoracotomy is a safe procedure with low rates of mortality. The long-term follow-up is necessary due to the significant risk of recoarctation requiring reintervention.

Multicenter experience with endovascular treatment of aortic coarctation in adults.

OBJECTIVE: The objective of this study was to evaluate outcomes of endovascular treatment of aortic coarctation in adults. METHODS: Clinical data and imaging studies of 93 consecutive patients treated at nine institutions from 1999 to 2015 were reviewed. We included newly diagnosed aortic coarctation (NCO), recurrent coarctation, and aneurysmal/pseudoaneurysmal degeneration (ANE) after prior open surgical repair (OSR) of coarctation. Primary end points were morbidity and mortality. Secondary end points were stent patency and freedom from reintervention. RESULTS: There were 54 (58%) male and 39 (42%) female patients with a mean age of 44 ± 17 years. Thirty-two patients had NCO (mean age, 48 ± 16 years) and 61 had endovascular reinterventions after prior OSR during childhood (mean, 30 ± 17 years after initial repair), including 50 patients (54%) with recurrent coarctation and 11 (12%) with ANE. Clinical presentation included asymptomatic in 31 patients (33%), difficult to control hypertension in 42 (45%), and lower extremity claudication in 20 (22%). Endovascular treatment was performed using balloon-expandable covered stents in 47 (51%) patients, stent grafts in 36 (39%) patients, balloon-expandable uncovered stents in 9 (10%) patients, and primary angioplasty in 1 (1%) patient. Mean lesion length and diameter were 64.5 ± 50.6 mm and 19.5 ± 6.7 mm, respectively. Mean systolic pressure gradient decreased from 24.0 ± 17.5 mm Hg to 4.4 ± 7.4 mm Hg after treatment (P < .001). Complications occurred in nine (10%) patients, including aortic dissections in three (3%) patients and intraoperative ruptures in two patients; type IA endoleak, renal embolus, spinal headache, and access site hemorrhage occurred in one patient each. The aortic dissections and ruptures were treated successfully by deploying an additional covered stent proximal to the site of dissection or rupture. Two patients died within 30 days of the index procedure. After a mean follow-up of 3.2 ± 3.1 years, nearly all patients (98%) were clinically improved and all stents were patent. Reintervention was needed in 10 (11%) patients. Freedom from reintervention at 5 years was 85%. Two additional patients died during follow-up of coarctation-related causes, including rupture of an infected graft and visceral ischemia. Patient survival at 5 years was 89%. CONCLUSIONS: Endovascular repair is effective with an acceptable safety profile in the treatment of NCO and postsurgical complications of coarctation after initial OSR. Aortic rupture is an infrequent (2%) but devastating complication with high mortality. Balloon-expandable covered stents are preferred for NCO, whereas stent grafts are used for ANE. The rate of reinterventions is acceptable, with high procedural and long-term clinical success.

Distal Aortic Arch Hypoplasia and Coarctation Repair: A Tailored Enlargement Technique.

BACKGROUND: Several techniques have been described to correct coarctation associated with distal arch hypoplasia. However, in neonates, residual gradients are frequently encountered and influence long-term outcome. We reviewed our experience with an alternative technique of repair combining carotid-subclavian angioplasty and extended end-to-end anastomosis. METHODS: From 1998 through 2014, 109 neonates (median age, 9 days) with coarctation and distal arch hypoplasia (n = 106) or type A interrupted aortic arch (n = 3) underwent repair using this technique. Thirty patients had isolated lesions (group 1), 44 associated ventricular septal defect (group 2), and 35 associated complex cardiac lesions (group 3). Median follow-up was 98 months. RESULTS: Repair was performed via left thoracotomy in 97%. There was one procedural-related death (0.9%) and overall five patients died during index admission (4.6%). Ten deaths were recorded at follow-up. Actuarial five-year survival was 86% (100% in group 1, 91% group 2, and 66% in group 3). Recurrent coarctation (clinical or invasive gradient >20 mm Hg) developed in 15 patients, all but 2 successfully treated by balloon dilatation. Freedom from any reintervention (dilatation or surgery) at five years was 86%. Only two patients were on antihypertensive drugs at last follow-up. CONCLUSIONS: This combined technique to correct distal arch hypoplasia and isthmic coarctation results in low mortality and acceptable recurrence rate. It preserves the left subclavian artery and allows enlargement of the distal arch diameter. Late outcome is excellent with very low prevalence of late arterial hypertension.

Arch Augmentation via Median Sternotomy for Coarctation of Aorta With Proximal Arch Hypoplasia.

BACKGROUND: Coarctation of the aorta can be associated with hypoplasia of the proximal transverse aortic arch. One approach to manage this condition is via left thoracotomy and extended end-to-end anastomosis with the expectation that the proximal arch will grow over time. Our preferred approach is to augment the aorta via midline sternotomy. We hypothesized that this approach is safe, durable, and allows reliable growth of the aorta. METHODS: We identified the records of patients with biventricular anatomy who had coarctation of the aorta, hypoplasia of the proximal transverse arch, and no other cardiac lesion that would mandate cardiopulmonary bypass use and midline sternotomy. The records of 62 such patients operated on between 2005 and 2016 were retrospectively reviewed. Patient demographics, clinical variables and outcome data were collected and analyzed using SAS 9.4. Data are presented as median (interquartile range [IQR]). RESULTS: Sixty-two patients (23 girls [37%]) underwent repair at 10 (IQR, 5 to 21) days of life. Forty-nine (79%) patients were on prostaglandin infusion to maintain ductal patency. Fifteen (24%) patients presented in shock with end organ dysfunction, 17 (27%) were on inotropes, and 26 (42%) were mechanically ventilated. The proximal transverse arch was 41% (IQR, 34% to 47%) of the size of ascending aorta as measured by echocardiography (z-score, -5 [IQR, -5.8 to -4.3]). Following median sternotomy, repair was carried out on cardiopulmonary bypass (41 [IQR, 37 to 47] minutes). The arch was reconstructed with (n = 26 [42%]) or without (n = 36 [58%]) coarctectomy usually using homograft patch aortoplasty (n = 58 [94%]). In all but 2 patients, repair was undertaken with circulatory arrest (27 [IQR, 22 to 31] minutes). Patients were extubated 4 (IQR, 3 to 5) days later and discharged home in 12 (IQR, 8 to 18) days. There was no mortality, and 8 morbidity events (3 recurrent nerve injury, 2 chylothorax, 1 phrenic nerve injury, 1 seizure, and 1 superficial wound infection) in 7 (11%) patients. All patients are alive at 41 (IQR, 11 to 64) months of follow-up. Reintervention was required in 6 (10%) patients (5 catheter based and 3 surgical) for recurrent distal coarctation. Reintervention-free survival at 1, 3, and 5 years was 87%. Only 1 child was currently on antihypertensive therapy, and all were in New York Heart Association functional class I symptoms. At last echocardiogram, the proximal transverse arch was 97% (IQR, 84% to 103%) of the diameter of the ascending aorta (z-score, 0.8 (IQR, 0.3 to 1.3]), ejection fraction was 70% (IQR, 60% to 76%), and only 2 patients had significant left ventricular hypertrophy. CONCLUSIONS: Arch augmentation via median sternotomy is a safe and effective procedure that can be accomplished with low morbidity and mortality. The reconstructed arch retains excellent growth potential resulting in a very favorable physiologic outcome.

Endovascular treatment of coarctation and related aneurysms.

Today, surgical repair has almost doubled the 30-year survival rate in patients with coarctation of the aorta (CoA), and 72% to 98% of patients now reach adulthood. Possible late complications include malignant hypertension, left ventricular dysfunction, aortic valve dysfunction, recurrent CoA, and aneurysm formation with risk of rupture. Treating postoperative CoA-related aneurysms with observation alone is associated with a mortality rate of 36%, compared with 9% for surgical repair. Even in the best surgeons' hands, aortic surgery has associated complications, and the complexity of reoperative surgery makes the risks substantially greater. For patients with CoA-related aneurysm, endovascular treatment constitutes a good alternative to reoperative surgery because it poses a lower risk of morbidity and mortality. Implanting an endograft has been shown to be successful in treating CoA and related aneurysms, producing excellent intermediate outcomes and minimal morbidity and mortality. Despite evidence that using covered stents improves outcomes, the superiority of any particular stent type has yet to be established. With a variety of endografts available, the decision of which stent to use depends on anatomy, availability, and operator preference.

Outcomes of thoracic endovascular aortic repair in adult coarctation patients.

BACKGROUND: Aortic coarctation (AC) is most commonly identified in pediatric patients; however, adults can present with late sequelae of untreated coarctation or complications of prior open repair. To date, there are limited data about the role of thoracic endovascular aortic repair (TEVAR) in this group of patients. The purpose of this analysis was to describe our experience with management of adult coarctation patients using TEVAR. METHODS: All TEVAR patients treated for primary coarctation or late sequelae of previous open repair (eg, pseudoaneurysm, recurrent coarctation or anastomotic stenosis related to index open coarctation repair) were reviewed. Demographics, comorbidities, procedure-related variables, postoperative outcomes, and reintervention were recorded. Computed tomography centerline assessments of endograft morphology were completed to delineate stent anatomy at the coarctation site. Survival and reintervention were estimated using life-table analysis. RESULTS: A total of 21 patients were identified (median age, 46 years [range, 33-71 years]; 67% male [n = 14]). Nine patients (43%) were treated for symptomatic primary (n = 6) or recurrent (n = 3) coarctation. Other indications included degenerative thoracic aneurysm (n = 6), pseudoaneurysm (n = 4), and dissection (n = 2). Technical success was 100% (95% confidence interval [CI], 84%-100%). No 30-day mortality or paraplegia events occurred; however, two patients (10%) experienced postoperative nondisabling stroke. In primary or recurrent coarctation patients with available computed tomography imaging (n = 8 of 9), nominal stent graft diameters were achieved proximal and distal to the coarctation (range, -0.4 to -1.2 mm of desired final stent diameter). Specific to the coarctation site, there was a significant increase in aortic diameter after TEVAR (before stenting, 11.5 [95% CI, 6.8-12.3] mm; after stenting, 15 [95% CI, 13.7-15.7] mm; P = .004). Concurrently, systolic arterial blood pressure at time of discharge was significantly lower (before stenting: 147 mm Hg; 95% CI, 137-157 mm Hg; after stenting: 124 mm Hg; 95% CI, 118-134 mm Hg; P = .02). For all patients, median clinical follow-up time was 8 months (interquartile range, 3-13 months; range, 1-106 months). Three endoleaks were detected, all of which were type II related to left (n = 2) or aberrant (n = 1) subclavian arteries. Four patients (19%) underwent reintervention (median time, 7 months; range, 2-12 months), with three of four being subclavian artery embolization; one was an aortic root replacement for ascending aneurysm with bicuspid aortic valve. One-year freedom from reintervention was 78% ± 9% (95% CI, 42%-92%). The 1- and 3-year survival was 95% ± 5% (95% CI, 71%-99%). One late death was related to complications from pre-existing congenital heart disease. CONCLUSIONS: Adult AC patients can be treated safely with TEVAR, and the annular constriction of an AC can be successfully dilated by the stent graft. Given these findings, a greater number of patients with longer term follow-up is warranted to further define the role of TEVAR in the management of adult AC patients.

Postnatal outcome of fetal isolated ventricular size disproportion in the absence of aortic coarctation.

OBJECTIVE: Cardiac ventricular size disproportion is a marker for aortic coarctation (CoA) in fetal life, but approximately 50% of fetuses do not have CoA after birth. The aim of this study was to evaluate the postnatal outcome of cases with fetal ventricular size disproportion in the absence of CoA after birth. METHODS: All cases with fetal isolated ventricular size disproportion diagnosed between 2002 and 2015 were extracted from a prenatal congenital heart defects regional registry. Cases were stratified according to presence or absence (non-CoA) of aortic arch anomalies after birth. Postnatal outcome of non-CoA cases was evaluated by assessing the presence of cardiac and other congenital malformations, genetic syndromes and other morbidity after birth. Non-CoA cases were further classified according to whether they had cardiovascular pathology requiring medication or intervention. RESULTS: Seventy-seven cases with fetal ventricular size disproportion were identified, of which 46 (60%) did not have CoA after birth. Of these, 35 did not require cardiovascular intervention or medication, whereas 11 did. Of the 46 non-CoA cases, six presented with clinical pulmonary hypertension requiring treatment after birth, cardiac defects were present in 24 cases and syndromic features were seen in four. Overall, 43% of all non-CoA children were still under surveillance at the end of the study period. CONCLUSIONS: The postnatal course of cases with fetal ventricular size disproportion is complicated by prenatally undetected congenital defects (46%) and pulmonary or transition problems (35%) in a significant number of cases that do not develop CoA. Proper monitoring of these cases is therefore warranted and it is advisable to incorporate the risks for additional morbidity and neonatal complications in prenatal counseling. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.