Transverse Arch Hypoplasia Research Articles

A Novel Diastolic Doppler Index Less Affected by Aortic Arch Anomalies Co-existing with Coarctation.

Severity assessment for coarctation of the aorta (CoA) is challenging due to concomitant morphological anomalies (complex CoA) and inaccurate Doppler-based indices. Promising diagnostic performance has been reported for the continuous flow pressure gradient (CFPG), but it has not been studied in complex CoA. Our objective was to characterize the effect of complex CoA and associated hemodynamics on CFPG in a clinical cohort. Retrospective analysis identified discrete juxtaductal (n = 25) and complex CoA (n = 43; transverse arch and/or isthmus hypoplasia) patients with arm-leg systolic blood pressure gradients (BPG) within 24 h of echocardiography for comparison to BPG by conventional Doppler indices (simplified Bernoulli equation and modified forms correcting for proximal kinetic energy and/or recovered pressure). Results were interpreted using the current CoA guideline (BPG ≥ 20 mmHg) to compare diagnostic performance indicators including receiver operating characteristic curves, sensitivity, specificity, and diagnostic accuracy, among others. Echocardiography Z-scored aortic diameters were applied with computational simulations from a preclinical CoA model to understand aspects of the CFPG driving performance differences. Diagnostic performance was substantially reduced from discrete to complex CoA for conventional Doppler indices calculated from patient data, and by hypoplasia and/or long segment stenosis in simulations. In contrast, diagnostic indicators for the CFPG only modestly dropped for complex vs discrete CoA. Simulations revealed differences in performance due to inclusion of the Doppler velocity index and diastolic pressure half-time in the CFPG calculation. CFPG is less affected by aortic arch anomalies co-existing with CoA when compared to conventional Doppler indices.

Virtual Planning and Patient-Specific Graft Design for Aortic Repairs.

Patients presenting with coarctation of the aorta (CoA) may also suffer from co-existing transverse arch hypoplasia (TAH). Depending on the risks associated with the surgery and the severity of TAH, clinicians may decide to repair only CoA, and monitor the TAH to see if it improves as the patient grows. While acutely successful, eventually hemodynamics may become suboptimal if TAH is left untreated. The objective of this work aims to develop a patient-specific surgical planning framework for predicting and assessing postoperative outcomes of simple CoA repair and comprehensive repair of CoA and TAH. The surgical planning framework consisted of virtual clamp placement, stenosis resection, and design and optimization of patient-specific aortic grafts that involved geometrical modeling of the graft and computational fluid dynamics (CFD) simulation for evaluating various surgical plans. Time-dependent CFD simulations were performed using Windkessel boundary conditions at the outlets that were obtained from patient-specific non-invasive pressure and flow data to predict hemodynamics before and after the virtual repairs. We applied the proposed framework to investigate optimal repairs for six patients (n = 6) diagnosed with both CoA and TAH. Design optimization was performed by creating a combination of a tubular graft and a waterslide patch to reconstruct the aortic arch. The surfaces of the designed graft were parameterized to optimize the shape. Peak systolic pressure drop (PSPD) and time-averaged wall shear stress (TAWSS) were used as performance metrics to evaluate surgical outcomes of various graft designs and implantation. The average PSPD improvements were 28% and 44% after the isolated CoA repair and comprehensive repair, respectively. Maximum values of TAWSS were decreased by 60% after CoA repair and further improved by 22% after the comprehensive repair. The oscillatory shear index was calculated and the values were confirmed to be in the normal range after the repairs. The results showed that the comprehensive repair outperforms the simple CoA repair and may be more advantageous in the long term in some patients. We demonstrated that the surgical planning and patient-specific flow simulations could potentially affect the selection and outcomes of aorta repairs.

Aortic arch repair with extended end-to-side anastomosis in neonates and infants with transverse arch hypoplasia

Background/Aim: The use of patches to repair the aortic arch is believed to have a positive effect on long-term morbidity. In this study, perioperative and follow-up data of patients who underwent transverse arch repair with a patch were compared with the data of patients who underwent end-to-end anastomosis (ESA). Methods: In this retrospective cohort study, the data of 27 patients (including 18 newborns) who underwent aortic arch repair at the Gazi Yasargil Education and Research Hospital between January 2018 and April 2023 were analyzed. The inclusion criteria included a diagnosis of proximal and distal transverse aortic arch hypoplasia, an age younger than 12 months of age, and the completion of aortic arch repair using cardiopulmonary bypass. Patients who underwent recoarctation repair due to residual obstruction, patients with single ventricular physiology, and patients who underwent aortic arch repair via a lateral thoracotomy without undergoing cardiopulmonary bypass were excluded from the study. The patients were divided into two groups. Group 1 included individuals who underwent aortic anterior wall expansion with autologous pericardium in addition to ESA; Group 2 included patients who underwent ESA only. Results: The median age of the patients was 21 days (range: 6–365 days), and the median body weight of the cohort was 3.5 kilograms (range: 2.4–8.9 kilograms). Enlargement with autologous pericardial patch was applied to 11 patients (40.7%). Surgical procedures performed in addition to arch repair included eight ventricular septal defect closures, six instances of pulmonary banding, three atrial septal defect closures, and one subvalvular pulmonary stenosis repair. The in-hospital mortality rate was 11.1% (n=3). Those three patients died due to sepsis. The median follow-up period was 152 days (range: 10–1316 days). Recoarctation requiring re-intervention did not occur in any of the studied patients. The antegrade selective cerebral perfusion time was statistically significantly longer in patients who underwent aortic arch repair using a patch (P=0.03). Conclusion: Repair of the arch with a patch may contribute to a reduction in long-term mortality and morbidity. However, there is a need for more comprehensive and long-term follow-up studies to verify these findings.

Intermediate and Long Term Follow Up of Balloon Angioplasty for Native Coarctation of the Aorta in Neonates and Infants

Background: Balloon angioplasty (BA) became an alternative procedure for the management of CoA, which initially performed at 1983 (5). The effectiveness of BA for the management of native CoA remains controversial in neonates (less than one month) and infants under the first year of life, in spite of its acceptable morbidity and the reduced complication rates. This controversy was due to higher re-stenosis rate and the increased risk of aneurysm formation present with CHF, which might also increase the surgical risk. Objective: To study the intermediate and the long-standing effects of BA for native CoA in neonates and infants and to determine any predictive factors for the outcome. Patients and Method: Included in the study were all patient 12 days to 11 months referred for possible balloon dilation to our Ibn-Albaitar teaching hospital department of congenital cardiac intervention with evidence of discrete coarctation of aorta. The hemodynamic data, angiogram and clinical records of 63 patients were examined with follow up data from 2 to 138 months (median 72 months). Results: Immediate success with balloon angioplasty was achieved in 35 Patients of 63 patients. 33 patients (52 %) with intermediate follow up data ( rang 12 to 138 months ) available are asymptomatic and normotensive with insignificant arm to leg blood pressure gradient (is lellss than 20 mmHg). 28 patients (44.4%) with primary successful result showed increase in gradient at 2 to 86 months after angioplasty requiring re-intervention in 28 Patients. Follow up after 84 months was available in 55 patients, 92% of whom are normotensive and have not required additional intervention. No additional intervention was needed in our patients > 7 years old. 17 of 29 surviving neonates who initially had a successful dilatation required re-angioplasty or operation after angioplasty. Transverse arch hypoplasia showed negligible effect on blood pressure gradient; whereas isthmus hypoplasia is linked with re-intervention in 14 % of patients. A small aneurysm was noted in 1 (1.5%) patients of 63 Patients. Conclusion: BA of native CoA is effective in neonates, infants under the first year of life, and also in the older children, the aneurysm formation is rare, and however life time follow up is reasonable.

Surgical options for proximal and distal transverse arch hypoplasia in infants with coarctation.

BackgroundAlthough various surgical techniques have been reported for aortic arch reconstruction for proximal and distal transverse arch (PDTA) hypoplasia, no consensus has been reached on a surgical option for initial arch reconstruction. This study was undertaken to review various arch reconstruction options for PDTA hypoplasia in Chinese infants.MethodsA retrospective review of 121 infants who underwent initial arch reconstruction of the proximal and distal aortic arches between 2010 and 2020 was performed. Freedom from recoarctation was analyzed using Kaplan-Meier analysis. Univariate and multivariable Cox regression analyses were performed to determine perioperative data associated with an increased risk of recoarctation after surgery.ResultsAortic arch reconstruction was performed by end-to-side anastomosis (ESA) (n=37) or patch repair [autologous pericardial patch (APP), n=53; bovine pericardial patch (BPP), n=20; autologous pulmonary artery patch (APAP), n=11]. The relative diameter of the proximal arch was 0.51±0.07, and the relative diameter of the distal arch was 0.43±0.07. The median follow-up time was 679 (range, 388–1,362) days. Recoarctation was observed in 44 (36.4%) patients. ESA was an independent risk factor for further development of recoarctation after the initial aortic arch reconstruction [hazard ratio (HR) =2.13; P=0.020].ConclusionsAortic arch reconstruction via ESA was an independent risk factor for late recoarctation of the proximal and distal aortic arches in patients who underwent the initial surgery in infancy.Trial RegistrationChinese Clinical Trials Registry ChiCTR2100048212.

The immediate and late results for balloon angioplasty of native coarctation of aorta in pediatrics age group

Background: percutaneous balloon dilation of corotation of aorta is a less invasive and alternative to surgical repair for patients with discrete coaction of aorta and although the used of balloon angioplasty in patients with recurrent postoperative coarctation gained a wide consensus, the use this technique for native coarctation is still controversial in children less one years. 
 Objective: to evaluate the immediate and late result of balloon dilation of native coarctation of aorta in infant and children.
 Type of the study: A prospective study. 
 Subjects & Methods: The study was done on forty-five patients who were referred for cardiac catheterization and balloon angioplasty of native coarctation of the aorta at Ibn-AL-Bitar center for cardiac surgery between January 2015 to May 2016.Left heart catheterization was performed with evaluation of the morphology and pressure gradient across the stenotic segment of aorta. Follow up evaluations were done between 1 week and 18 months after discharge (mean 6 months) with transthoracic echocardiography. 
 Results: The age of patients range from 1month to 4 years (median age at time of procedure was12.3month). Associated lesions in 11 patients (24.4%). Immediate results were reduction in gradient from 42.5± 17.3 to 10.3± 8.2 mm Hg in forty-two patients (93.3%). Late result during follow up period, 29 patients (69%) had maintain gradient less than 20mmHg. Complications were reported in 6 patients (13.3%). there was one procedure related death (2.2%). 
 Conclusions: Balloon angioplasty for treatment of native coarctation of the aorta is effective with sustained benefit on long-term follow-up in infants and children >6 months. Transverse arch hypoplasia and children <6 months old has major effect on late outcome of recurrent coarctation of aorta.

The aorta after coarctation repair \u2013 effects of calibre and curvature on arterial haemodynamics

BackgroundAortic shape has been proposed as an important determinant of adverse haemodynamics following coarctation repair. However, previous studies have not demonstrated a consistent relationship between shape and vascular load. In this study, 3D aortic shape was evaluated using principal component analysis (PCA), allowing investigation of the relationship between 3D shape and haemodynamics.MethodsSixty subjects (38 male, 25.0 ± 7.8 years) with repaired coarctation were recruited. Central aortic haemodynamics including wave intensity analysis were measured noninvasively using a combination of blood pressure and phase contrast cardiovascular magnetic resonance (CMR). 3D curvature and radius data were derived from CMR angiograms. PCA was separately performed on 3D radius and curvature data to assess the role of arch geometry on haemodynamics. Clinical findings were corroborated using 1D vascular models.ResultsThere were no independent associations between 3D curvature and any hemodynamic parameters. However, the magnitude of the backwards compression wave was related to the 1st (r = − 0.36, p = 0.005), 3rd (r = 0.27, p = 0.036) and 4th (r = − 0.31, p = 0.017) principle components of radius. The 4th principle componentof radius also correlated with central aortic systolic pressure. These aortas had larger aortic roots, more transverse arch hypoplasia and narrower aortic isthmuses.ConclusionsThere are major modes of variation in 3D aortic shape after coarctation repair witha modest association between variation in aortic radius and pathological wave reflections, but not with 3D curvature. Taken together, these data suggest that shape is not the major determinant of vascular load following coarctation repair, and calibre is more important than curvature.

Long-Term Outcomes of Coarctation Repair Through Left Thoracotomy

Optimal surgical approach for repair of coarctation of the aorta (CoA) remains controversial. This study aimed to evaluate reintervention rates and its predictors by using a strategy of resection with extended end-to-end anastomosis (REEEA) through left thoracotomy. A retrospective analysis was performed for all patients who underwent isolated CoA repair or simultaneous repair of CoA and ventricular septal defect repair by REEEA between January 2000 and December 2015 at Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois. Patients with complex congenital heart disease were excluded. Transverse arch hypoplasia was defined as echocardiographic z-score lower than-2 or by documentation in medical or operative reports. Reintervention was defined as the need for balloon angioplasty or reoperation. Hypertension was defined as antihypertensive medication use or blood pressure greater than or equal to the 95th percentile. A total of 251 patients with median age at repair of 14.6 days met inclusion criteria. Repair was by left thoracotomy in 226 (90%). Follow-up data were available for 186 of 251 patients, with median follow-up time of 5.4 years (range, 0.2 to 15.3 years); 169 (91%) of these patients underwent thoracotomy. There were no early deaths or early reoperations. A proximal transverse arch z-score lower than-4.1 or a distal transverse arch z-score of less than-2.8 was predictive of repair through sternotomy. Only 4 (2%) patients required reintervention (2 patients had balloon angioplasties, 2 had reoperations). Transverse arch hypoplasia was a risk factor for reintervention (p= 0.048), but surgical approach was not (p= 0.35). Late hypertension was identified in only 33 of 186 (18%) patients. Repair of CoA, even with associated transverse arch hypoplasia, by REEEA through left thoracotomy has a low mortality, low reintervention rate, and low incidence of late hypertension.

Invited Commentary

The surgical repair of arch obstruction dates back to the first report of successful repair of aortic coarctation by Clarence Crafoord in 1944 [1Crafoord C. Nylin G. Congenital coarctation of the aorta and its surgical treatment.J Thorac Surg. 1945; 14: 347-361Abstract Full Text PDF Google Scholar]. The spectrum of aortic arch obstruction extends from the simplest form with isolated coarctation to the most complex form of obstruction, which involves complete interruption of the aorta. Although little controversy exists about the technique to repair either isolated coarctation or aortic interruption, there is a tremendous amount of controversy about the surgical management of transverse aortic arch hypoplasia. Perhaps the most controversial area is surgical management of proximal transverse arch hypoplasia, which Gray and colleagues [2Gray W.H. Wells W.J. Starnes V.A. Kumar S.R. Arch augmentation via median sternotomy for coarctation of aorta with proximal arch hypoplasia.Ann Thorac Surg. 2018; 106: 1214-1219Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar] have described in their article. In dealing with arch hypoplasia, there is no commonly accepted way to describe the problem. Gray and colleagues [2Gray W.H. Wells W.J. Starnes V.A. Kumar S.R. Arch augmentation via median sternotomy for coarctation of aorta with proximal arch hypoplasia.Ann Thorac Surg. 2018; 106: 1214-1219Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar] use a proximal transverse arch diameter that is less than 50% of the diameter of the ascending aorta. Surgical lore suggests that an arch size that is the patient’s weight in kilograms plus one is an acceptable standard. Z scores are probably the most common means of determining deviation from normal, but even so, there are multiple different z score programs that exist that are not identical in their measurements. A similar degree of controversy exists regarding the surgical approach to transverse arch hypoplasia. Gray and colleagues [2Gray W.H. Wells W.J. Starnes V.A. Kumar S.R. Arch augmentation via median sternotomy for coarctation of aorta with proximal arch hypoplasia.Ann Thorac Surg. 2018; 106: 1214-1219Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar] champion a median sternotomy approach for their patients, but there are plenty of data to suggest that the relief of distal obstruction through a thoracotomy approach will allow for improved flow through the area of proximal hypoplasia and, ultimately, growth in this area [3Kotani Y. Anggriawan S. Chetan D. et al.Fate of the hypoplastic proximal aortic arch in infants undergoing repair for coarctation of the aorta through a left thoracotomy.Ann Thorac Surg. 2014; 98: 1386-1393Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar, 4Siewers R. Ettedgui J. Pawl E. TallmanT del Nido P. Coarctation and hypoplasia of the aortic arch: will the arch grow?.Ann Thorac Surg. 1991; 52: 608-613Abstract Full Text PDF PubMed Scopus (68) Google Scholar]. This controversy may be completely addressed only by a randomized controlled multiinstitutional trial. Ultimately, the ideal approach to the management of arch hypoplasia will be identified only after much longer-term follow-up. The goal of congenital heart surgery is not survival measured in months or years but in decades. The quality of that survival will also be critical in determining the best operative management, by taking into account such factors as the need for both operative and percutaneous reintervention, the need for further medical management, and detailed long-term neurodevelopmental outcomes in these patients. In particular, longitudinal neurodevelopmental outcomes are paramount to determining the success of this surgical procedure related to the consequences of the bypass technique [5Ohye R. Goldberg C. Donohue J. et al.The quest to optimized neurodevelopmental outcomes in neonatal arch reconstruction: the perfusion techniques we use and why we believe in them.J Thorac Cardiovasc Surg. 2009; 137: 803-806Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar]. In this paper, Gray and colleagues [2Gray W.H. Wells W.J. Starnes V.A. Kumar S.R. Arch augmentation via median sternotomy for coarctation of aorta with proximal arch hypoplasia.Ann Thorac Surg. 2018; 106: 1214-1219Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar] definitely present a safe and effective management strategy, but the quest to determine the ideal strategy remains elusive. Arch Augmentation via Median Sternotomy for Coarctation of Aorta With Proximal Arch HypoplasiaThe Annals of Thoracic SurgeryVol. 106Issue 4PreviewCoarctation 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. Full-Text PDF

Mid-Term Outcomes of a Modification of Extended Aortic Arch Anastomosis with Pulmonary Artery Banding in Single Ventricle Neonates with Hypoplastic Transverse Arch.

There is less certainty regarding the best strategy for treating neonates with functional single ventricle (SV) and hypoplastic aortic arch. We have applied a modified extended aortic arch anastomosis (EAAA) and main pulmonary artery banding (PAB) as an initial palliation in neonates with transverse arch hypoplasia and assessed the mid-term outcomes. In total, 10 neonates with functional SV and extensive hypoplasia or interruption of the arch underwent a modified EAAA (extended arch anastomosis with a subclavian flap) concomitant with main PAB through a thoracotomy without cardiopulmonary bypass. Patient age and weight ranged from 4 to 14 days and 2.3 to 3.8 kg, respectively. There were no hospital deaths although there were two late deaths. Gradients across the arch were 0 to 7 mmHg at postoperative day 1 and no arch reoperations were required. Two patients required balloon aortoplasty. Nine underwent bidirectional cavopulmonary shunt and two of them needed concomitant Damus-Kaye-Stansel (DKS) anastomosis. Six have completed Fontan. Our modification of EAAA with main PAB for SV neonates may benefit a certain population with transverse arch hypoplasia as an option to be considered. Patients with the potential for developing outflow obstruction may be best managed with an initial DKS-type palliation.

Turner Syndrome in Girls Presenting with Coarctation of the Aorta

To evaluate the frequency of Turner syndrome in a population-based, statewide cohort of girls with coarctation of the aorta. The Utah Birth Defects Network was used to ascertain a cohort of girls between 1997 and 2011 with coarctation of the aorta. Livebirths with isolated coarctation of the aorta or transverse arch hypoplasia were included and patients with complex congenital heart disease not usually seen in Turner syndrome were excluded. Of 244 girls with coarctation of the aorta, 77 patients were excluded, leaving a cohort of 167 girls; 86 patients (51%) had chromosomal studies and 21 (12.6%) were diagnosed with Turner syndrome. All patients were diagnosed within the first 4 months of life and 5 (24%) were diagnosed prenatally. Fifteen patients (71%) had Turner syndrome-related findings in addition to coarctation of the aorta. Girls with mosaicism were less likely to have Turner syndrome-associated findings (3/6 mosaic girls compared with 12/17 girls with non-mosaic 45,X). Twelve girls (57%) diagnosed with Turner syndrome also had a bicommissural aortic valve. At least 12.6% of girls born with coarctation of the aorta have karyotype-confirmed Turner syndrome. Such a high frequency, combined with the clinical benefits of an early diagnosis, supports genetic screening for Turner syndrome in girls presenting with coarctation of the aorta.

Persistent transverse arch hypoplasia is associated with systemic hypertension after coarctation of aorta repair

Methods A retrospective review of clinical, CMR and exercise data on patients after coarctation repair was performed to include patients who had CMR from 2005-2014. Patients with atypical coarctation, major heart defects, residual coarctation (right arm-leg blood pressure gradient > 20 mm Hg) or >mild semilunar or atrioventricular valve stenosis/regurgitation were excluded. Patients satisfying standard criteria for resting hypertension and those on anti-hypertensive medication were classified as being “hypertensive”. In a subset of patients, exercise testing was used to evaluate the increase in right arm systolic blood pressure (SBP) and arm-leg SBP gradient during peak exercise. CMR images were analyzed to calculate the minimum TAA diameter z-score, the ratio of TAA and thoracic descending aorta (DAO) diameters, isthmus diameter z-score and, LV mass/BSA.

The Neonatal Hypoplastic Aortic Arch: Decisions and More Decisions

Neonatal patients with hypoplasia of the aortic arch constitute a heterogeneous group with a wide spectrum of severity. The milder end of the spectrum comprises patients with aortic coarctation and isthmus hypoplasia. At the other end of the spectrum are patients with severe transverse arch hypoplasia or hypoplastic left heart syndrome. The aim of this paper is to discuss the various strategies and surgical approaches available for this group of patients, focusing on the surgical decisions that influence individual patient management. Many of the things discussed are applicable to any neonatal arch problem. We also describe and discuss in detail our surgical technique for patients who undergo neonatal repair of a hypoplastic aortic arch via median sternotomy.

Foetal echocardiographic assessment of borderline small left ventricles can predict the need for postnatal intervention

We sought to prospectively determine foetal echocardiographic factors associated with neonatal interventions in borderline hypoplastic left ventricles. Foetuses were included who had a left ventricle that was 2-4 standard deviations below normal for length or diameter and had forward flow across the mitral and aortic valves. Factors associated with an intervention in the first month of life or no need for intervention were sought using univariate and multivariate logistic regression models. From 2005 to 2008, 47 foetuses meeting the criteria had an additional diagnosis (+foetal coarctation/+transverse arch hypoplasia): atrioventricular septal defect 7 (+2/+0), double outlet right ventricle 2 (+0/+0), Shone's complex 19 (+9/+4), and ventricular disproportion 19 (+13/+11; 4 both). There were seven pregnancies terminated, three foetal demises, and five had compassionate care. There were 32 livebirths that either had a biventricular repair (n = 20, n = 2 dead), univentricular palliation (n = 2, both alive), or no intervention (n = 9). Overall survival of livebirths to 6 months of age was 79%. Factors associated with early intervention on first foetal echocardiogram were: obstructed or retrograde arch flow (p = 0.08, odds ratio 3.3), coarctation (p = 0.05, odds ratio 11.4), and left ventricle outflow obstruction (p = 0.05, odds ratio 12.5). Neonatal factors included: Shone's diagnosis (p = 0.02, odds ratio 4.9), bicuspid aortic valve (p = 0.005, odds ratio 11.7), and larger tricuspid valve z-score (p = 0.05, odds ratio 3.6). A neonatal factor associated with no intervention was a larger mitral valve z-score (mean 23.8 versus 24.2 intervention group, p = 0.04, odds ratio 2.8). The need for early intervention in foetuses with borderline hypoplastic left ventricle can be predicted by foetal echocardiography.

Right Pulmonary Artery Thrombosis After Hybrid Stage 1 Palliation: Resolution After Direct Tissue Plasminogen Activator Infusion

A patient with unbalanced right ventricular dominant atrioventricular septal defect with ascending and transverse arch hypoplasia underwent hybrid stage 1 palliation. On postoperative day 7, he experienced irritability and acute cyanosis. Echocardiography demonstrated a thrombus occluding the right pulmonary artery band. Emergency cardiac catheterization confirmed thrombosis of the right pulmonary artery at the site of the band. Direct infusion of tissue plasminogen activator successfully lysed the thrombus.

Stenting across head and neck vessels using covered stents for persisting aortic arch obstruction

To describe endovascular stent placement using partially covered stents to preserve flow in head and neck vessels. Endovascular stent placement has become established as a first-line therapy for native coarctation of the aorta or re-coarctation in older children and adults. Increasingly covered stents are becoming the preferred option over bare-metal stents because of the perceived lower risk of aneurysm formation. Open-cell bare-metal stents are chosen when there is a high likelihood of jailing a head and neck vessel. Here we describe partial uncovering of a covered stent before implantation to allow flow through the uncovered portion of the stent to the branch vessel but preserve the covering over the majority of the remaining stent. We describe two cases with aortic arch hypoplasia and re-coarctation, both of which required two partially uncovered stents for a satisfactory result. Endovascular stent placement is becoming the preferred option in the management of coarctation of the aorta in older children and adults. Strategies to deal with transverse arch hypoplasia and multiple levels of aortic arch obstruction frequently involving branch vessels or aneurysms need to be considered before these procedures are embarked upon. Partially uncovering stents may afford more protection than using bare-metal stents in the transverse and distal arch while preserving flow in head and neck branches, and is a technically straightforward procedure.

Surgical Strategy for Aortic Coarctation Repair Resulting in Physiologic Arm and Leg Blood Pressures

The goal of aortic coarctation repair is laminar aortic blood flow resulting in a negative or absent arm:leg blood pressure (BP) gradient. Despite satisfactory relief of coarctation, associated arch hypoplasia can result in residual obstruction and postoperative upper body hypertension. We devised a surgical strategy to create a tension-free anastomosis with a diameter as large as both the adjacent proximal and distal aorta using a radically extended end-to-end anastomosis via sternotomy and/or thoracotomy. Sternotomy is chosen when there is significant transverse arch hypoplasia defined as a distal transverse arch ≤ diameter of the left carotid artery, presence of a common brachiocephalic trunk, or coexisting intracardiac lesion requiring repair. Thoracotomy is used in all other cases. From 2000 to 2008, 95 consecutive patients were repaired using this approach, 35 with sternotomy and 60 with thoracotomy. At a mean follow-up of 50 ± 23 months, mean systolic BP was 94 ± 10 mm Hg, and 84% of patients had no residual arm:leg BP gradient. Mean arm:leg BP gradient was not statistically different between groups (-8.5 ± 15 sternotomy and -7.0 ± 10 mm Hg thoracotomy, P= .7). With Doppler echocardiography, 96% of patients demonstrated normal early diastolic reversal of blood flow in the descending thoracic aorta. For aortic coarctation repair in infancy, a strategy designed to directly address aortic arch hypoplasia results in excellent intermediate-term results with normal BP, physiologic arm:leg BP relationship, and near normal descending aortic blood flow velocities by Doppler.

Complex Aortic Coarctation, Regurgitant Bicuspid Aortic Valve with VSD and Ventricular Non-Compaction: A Challenging Combination

Left ventricular non-compaction is a myocardial disorder characterized by excessive trabeculations and deep recesses that communicate with the ventricular cavity, which is thought to result from a failure of the trabecular regression that occurs during normal embryonic development. It carries a high mortality from heart failure or sudden cardiac death. A 15-year-old female patient was referred to our institution for moderate symptoms of heart failure. Echocardiography and MRI showed a bicuspid aortic valve with severe regurgitation, subaortic VSD, dilated left ventricle and left ventricular non-compaction with a moderately decreased ejection fraction, as well as isthmic coarctation and transverse arch hypoplasia. We elected to perform transaortic VSD closure and aortic valve replacement using a mechanical prosthetic valve on an arrested heart, and to address aortic coarctation and transverse arch hypoplasia using an extra-anatomic ascending-to-descending aorta bypass. Aortic cross-clamping was limited to 41 minutes. The postoperative recovery was rapid and the girl was discharged in NYHA class I with an estimated LVEF of 39%. Although management must be individualized, extra-anatomic bypass is a good single-stage approach for patients with complex coarctation and concomitant cardiovascular or myocardial disorders, reducing ischemic time and offering a better chance of successful weaning from cardiopulmonary bypass.

Coarctation of the Aorta: Midterm Outcomes of Resection With Extended End-to-End Anastomosis

We began using the technique of resection with extended end-to-end anastomosis for infants and children with coarctation of the aorta in 1991. The purpose of this review is to evaluate the midterm outcomes of this technique, specifically determining the incidence of and risk factors for transcatheter or surgical reintervention. A retrospective analysis of the cardiac surgery database was performed to identify all patients who had a diagnosis of coarctation of the aorta with or without ventricular septal defect and had resection with extended end-to-end anastomosis from 1991 to 2007. Perioperative course and follow-up with physical examination, echocardiogram, and cardiology evaluation were obtained. From 1991 through 2007, 201 patients had repair of coarctation of the aorta with resection with extended end-to-end anastomosis. The median age was 23 days, and the median weight was 4.0 kg. Surgical approach was by left thoracotomy in 157 patients (78%) with a mean cross-clamp time of 18 +/- 4 minutes. Median sternotomy approach was used in 44 patients (22%) to repair a hypoplastic transverse aortic arch (n = 16) or because of associated ventricular septal defect (n = 28) with a mean circulatory arrest time of 14 +/- 9 minutes. Early mortality occurred in 4 patients (2.0%). Three patients (1.5%) required early arch revision: 2 intraoperatively and 1 on postoperative day 1. Follow-up data were available for 182 patients (91%) with a mean follow-up of 5.0 +/- 4.3 years (908 patient-years). Reinterventions (n = 8; 4.0%) included three balloon angioplasties and five reoperations; 75% of the reinterventions occurred in the first postoperative year. Hypoplastic transverse aortic arch was not a risk factor for reintervention (p = 0.36), but was a risk factor for mortality (p = 0.039). Aberrant right subclavian artery was the only risk factor for recoarctation (p = 0.007). Repair of coarctation of the aorta with resection with extended end-to-end anastomosis has a low early mortality, effectively addresses transverse arch hypoplasia, and at midterm follow-up has a low rate of reintervention for recurrent coarctation.

A Modification of Extended Aortic Arch Anastomosis Augmented with Subclavian Flap Aortoplasty for Interrupted or Hypoplastic Aortic Arch

Surgical repair for hypoplastic aortic arch in neonates carries a substantial risk of recurrent obstruction. Simple arch anastomosis is not always a solution in cases of extended arch hypoplasia. We present our modified technique of extended aortic arch anastomosis augmented with subclavian flap aortoplasty. We describe two neonates: interrupted aortic arch and transverse arch hypoplasia associated with aortic coarctation, who underwent a modification of extended aortic arch anastomosis augmented with subclavian flap aortoplasty. The patients recovered without any pressure gradient at the anastomotic site. Postoperative aortography showed no arch obstruction and they successfully underwent second stage repair. Our technique provides extensive augmentation of the aortic arch with a tension-free, wide and non-circumferential suture line which preserves potential for growth. The technique described may avoid persistent or repeat arch obstruction.