Predictors of left ventricular outflow tract obstruction after biventricular repair in interrupted aortic arch or aortic coarctation.

BACKGROUND: Left ventricular outflow tract obstruction (LVOTO) re-intervention is a significant cause of morbidity and mortality in patients with coarctation of the aorta (CoA) or interrupted aortic arch (IAA) after aortoplasty. METHODS: This retrospective study analyzed data from neonates with IAA/CoA who underwent biventricular repair between 2012 and 2022. LVOTO events were defined by the detection of color Doppler flow acceleration ≥3.0 m/s at the valvular, subvalvular, or supravalvular regions via transthoracic echocardiography, and the necessity for surgical or catheter intervention to relieve the obstruction. RESULTS: Among 121 neonates with CoA/IAA, 16 (13.7%) primary aortoplasty patients developed LVOTO. Additionally, one patient (25%) who underwent a staged Yasui operation developed LVOTO due to a narrowed ventricular septal defect-pulmonary atresia tunnel. During follow-up, 58% of patients with a bicuspid valve and 25% of patients with a subaortic ridge developed LVOTO. The combination of either a bicuspid valve, subaortic ridge, or an aortic valve annulus Z-score < -3.0 predicted a high re-intervention rate (7/8 [87.5%]). CONCLUSIONS: In patients with IAA/CoA, the presence of multiple risk factors, including a bicuspid valve, subaortic ridge, and an aortic valve annulus Z-score < -3.0, is associated with a significantly increased rate of re-intervention for LVOTO.

Impact of Surgical Strategy and Post-Repair Transverse Aortic Arch Size on Late Hypertension After Coarctation Repair During Infancy.

OBJECTIVE: Late HTN after CoA repair contributes to higher morbidity and mortality. An association between TAA hypoplasia and HTN has been found, but its relationship with surgical strategy is unclear. We studied the association between late HTN and initial surgical strategy pertaining to the TAA. METHODS: We retrospectively reviewed patients who underwent surgical repair of CoA during infancy with at least 10 years follow-up, excluding those with atypical coarctation, major associated heart defects, and residual isthmic narrowing. TAA diameter z-score immediately post-repair was measured as a marker of surgical strategy. Systemic HTN at latest follow-up was assessed using standard criteria. RESULTS: A total of 130 patients underwent surgical repair of CoA (76% via thoracotomy, 24% via sternotomy; type of repair - resection and end-to-end anastomosis 62%, extended end-to-end anastomosis 30%, subclavian flap 5%, arch repair with patch 4%), at a median age of 14 (IQR 7-62) days. Median post-repair TAA diameter z-score was -2.04 (IQR -2.69, 1.24). After a mean follow-up of 17.3 years, 43/130 (33%) patients developed HTN. After controlling for age at repair, gender, and presence of a genetic syndrome, HTN was not associated with immediate post-repair TAA diameter z-score, (p=0.41), type of surgical incision (p=0.99), or type of surgical repair (p=0.66). CONCLUSIONS: In patients undergoing surgical repair of CoA during infancy, late HTN was not associated with immediate post-repair TAA size or surgical strategy pertaining to the TAA. These results suggest that factors other than surgical strategy, such as differential growth of the TAA during childhood, may be important.

Hemodynamics in the treatment of pseudoaneurysm caused by extreme constriction of aortic arch with coated stent.

Objectives: To evaluate the changes in distal vascular morphology and hemodynamics in patients with extremely severe aortic coarctation (CoA) after covered palliative (CP) stent dilation with different surgical strategies. Materials and methods: Perioperative computed tomography angiography and digital subtraction angiography were utilized to construct three aortic models with varying stenosis rates and one follow-up model in a patient with extremely severe CoA. The models included: an idealized non-stenosed model (A: 0%), a model post initial stent deployment (B: 28%), a model post balloon expansion (C: 39%), and a model 18 months after post-balloon expansion (D: 39%). Consistent boundary conditions were applied to all models, and hemodynamic simulation was conducted using the pure fluid method. Results: The narrowest and distal diameter of the stent increased by 34.71% and 59.29%, respectively, from model B to C. Additionally, the distal diameter of the stent increased by -13.80% and +43.68% compared to the descending aorta diameter, respectively. Furthermore, the ellipticity of the maximum cross-section of the aneurysm region in model A to D continued to increase. The oscillatory shear index at the stenosis to the region of the aneurysm were found to be higher in Models A and B, and lower in Models C and D. At the moment of maximum flow velocity, the blood flow distribution in models A and B was more uniform in the widest section of the blood vessels at the distal end of the stenosis, whereas models C and D exhibited disturbed blood flow with more than 2 eddy currents. The time-averaged wall shear stress (TAWSS) decreased in the distal and basal aneurysms, while it significantly increased at the step position. The aneurysmal region exhibited an endothelial cell activation potential value lower than 0.4 Pa-1. Conclusion: In patients with extremely severe CoA, it is crucial to ensure that the expanded diameter at both ends of the CP stent does not exceed the native vascular diameter during deployment. Our simulation results demonstrate that overdilation leads to a decrease in the TAWSS above the injured vessel, creating an abnormal hemodynamic environment that may contribute to the development and enlargement of false aneurysms in the early postoperative period. Clinical Trial Registration: ClinicalTrials.gov, (NCT02917980).

Surgical technical experience of adult aortic coarctation concomitant with poststenotic aneurysm or dissection.

Background: Aortic coarctation (COA) in adults combined with poststenotic aneurysm (PA) or poststenotic dissection (PD) is rare and challenging to manage. The existence of multiple factors such as kinking, comorbidities, previous surgical history, and descending aortic lesions increases the difficulty of treatment, and there are currently few clinical reports. The purpose of this study was to present our surgical experience in dealing with such patients. Methods: A retrospective study was conducted on 20 consecutive patients with COA combined with PA or PD who were treated in our center from December 2015 to April 2019. The basic principles, methods, and short- and mid-term prognosis of surgery are present carefully. This paper introduces the individualized treatment scheme as well as its advantages and disadvantages in detail. Results: The condition of the included patients was complicated, including 12 cases of PA and 8 of PD. Although different surgical schemes were adopted, procedural success rate was 100%. There were no other surgical complications except 2 cases of anastomotic bleeding and 1 case of spinal cord injury. The results of computed tomography angiography (CTA) demonstrated that 9 cases achieved anatomical correction, 10 cases of PA or PD were eliminated or thrombosed to varying degrees, and only 1 case of PA had no obvious change. Up to the follow-up period, except for 1 patient who had a slight cerebrovascular accident and 1 who had no change in PA underwent cheatham platinum (CP) stent surgery, no other cardiovascular adverse events occurred and all patients recovered well. Conclusions: The optimal surgical strategy developed collaboratively by cardiac surgeons and endovascular specialists has achieved satisfactory short- and mid-term results for COA patients combined with PA or PD. Further research is still necessary, due to the limited number of cases.

Combined procedure using a double flap as a surgical option for coarctation of the aorta with delayed diagnosis.

Simple coarctation of the aorta is repaired in an infant by direct end-to-end anastomosis of the aorta or subclavian flap aortoplasty. However, some cases are not detected until late childhood. For school-age patients, greater consideration must be given to risks such as postoperative limb ischemia and the potentially harmful effects of any artificial material on future growth. Here, we describe our technique for these patients, in whom the value of direct anastomosis is uncertain, to minimize the amount of synthetic graft material used while achieving successful anatomical repair.

Coarctation of the Aorta and Late-Onset Sepsis With Supraventricular Tachycardia: A Clinical Case Review.

We report a case of an 11-day-old male infant who presented to our hospital with complaints of breathing difficulty and cough for two days prior to admission. The child had a seizure post-admission which was controlled with phenobarbitone. The child was connected to a mechanical ventilator. The baby also had persistent tachycardia, which upon doing an electrocardiogram (ECG) led to a diagnosis of supraventricular tachycardia (SVT). Additional investigations revealed the existence of late-onset sepsis (LOS), which was treated with appropriate antibiotics. In view of weak femoral pulses with normal brachial pulses, a diagnosis of coarctation of the aorta (CoA) was entertained. The diagnosis was confirmed by an echocardiogram, and the baby was transferred to a higher-level medical center for surgical correction. Regrettably, the baby succumbed to heart failure and shock on the 12th day of life. This case highlights the infrequency of CoA accompanied by SVT. The case delves into the challenges of diagnosing the condition, the necessary medical interventions, and the unforeseen complications that must be considered to reduce mortality in such circumstances.

Accurate prenatal diagnosis of coarctation of the aorta by 3-step echocardiographic diagnostic protocol.

BACKGROUND: Coarctation of the aorta (CoA) is the most common undiagnosed congenital heart defect during prenatal screening. High false positive and false negative rates seriously affect prenatal consultation and postnatal management. The objective of the study was to assess the utility of various measurements to predict prenatal CoA and to derive a diagnostic algorithm. METHODS: One hundred and fifty-four fetuses with suspected CoA who presented at Fuwai Hospital between December 2017 and August 2021 were enrolled and divided into confirmed CoA cases (n = 47) and false positive cases (n = 107), according to their postnatal outcomes. The transverse aortic arch, isthmus, and descending aorta were measured in the long-axis view of the aortic arch. The angle between the transverse aortic arch (TAO) and the descending aortic arch (DAO) was defined as the TAO-DAO angle and measured in the long axis or sagittal view. Based on the database in GE Voluson E10 and the formula (Z = [Formula: see text]), the standard score (Z-score) of the dimensions of the aorta were calculated in relation to the gestational age. The main echocardiographic indices were combined to design a 3-step diagnostic protocol. The TAO-DAO angle was used as the first step in the diagnostic model. The diameter of the transverse arch and the Z-score of the isthmus were the second step. The third-step indices included a Z-score of the transverse arch, diameter of the isthmus, distance from the left subclavian artery (LSA) to left common carotid artery (LCCA), the ratio of isthmus diameter and LSA diameter and ratio of the distances (the distance between the LSA and LCCA to the distance between the right innominate artery and LCCA). The receiver operating characteristic (ROC) curve determined the predictive capability of each diagnostic parameter, and the kappa test determined the diagnostic accuracy of the proposed model. RESULTS: The cases with confirmed CoA had thinner transverse arches (1.92 ± 0.32 mm vs. 3.06 ± 0.67 mm, P = 0.0001), lower Z-scores of the isthmus (-8.97 ± 1.45 vs. -5.65 ± 1.60, P = 0.0001), smaller TAO-DAO angles (105.54 ± 11.51° vs. 125.29 ± 8.97°, P = 0.0001) and larger distance between the LSA and LCCA (4.45 ± 1.75 mm vs. 2.74 ± 1.07 mm, P = 0.0001) than the false positive cases. The area under the curve (AUC) was 0.947 (95% CI 0.91-0.98) for the TAO-DAO angle ≤ 115.75°, 0.942 (95% CI 0.91-0.98) for the transverse arch diameter ≤ 2.31 mm, 0.937 (95% CI 0.90-0.98) for the Z-score of the isthmus ≤ -7.5, and 0.975 (95% CI 0.95-1.00) for the 3-step diagnostic protocol with 97.8% sensitivity and 97.2% specificity. The kappa test showed that the model's diagnostic accuracy was consistent with postnatal outcomes (kappa value 0.936, P = 0.0001). CONCLUSIONS: The 3-step diagnostic protocol included the three most useful measurements and the additional indices with appropriate cut-off values. The algorithm is useful for the detection of aortic coarctation in fetuses with a high degree of accuracy. TRIAL REGISTRATION: Retrospectively registered.

Transcarotid TAVR for Severe Bicuspid Aortic Stenosis With Virtually Atretic Coarctation of the Thoracic Aorta.

Transcatheter aortic valve replacement by alternate access sites allows for treatment of patients with unfavorable anatomy for a transfemoral approach. To our knowledge, we present the first reported case of successful transcatheter aortic valve replacement via the transcarotid approach in a 65-year-old man with a symptomatic severe bicuspid aortic valve stenosis and virtually atretic coarctation of the thoracic aorta.

Anesthetic management of extracorporeal membrane oxygenation-supported aortic bypass surgery for atypical coarctation with severe left ventricular dysfunction: A case report.

Atypical aortic coarctation is an exceedingly rare condition, and there are very few reported cases of anesthetic management for bypass surgery in patients with severe impaired cardiac function. We present the anesthetic management of a 68-year-old woman with atypical aortic coarctation due to Takayasu arteritis and severely impaired cardiac function, who underwent ascending-to-abdominal aorta bypass surgery under extracorporeal membrane oxygenation (ECMO). The patient's severe cardiac dysfunction was due to sustained afterload from the coarctation, leading to recurrent episodes of heart failure. Surgical intervention was deemed necessary, and a decision was made to perform a bypass operation. The patient experienced a transient state of shock following induction of anesthesia, but subsequent perioperative care was safely managed with the implementation of ECMO. For bypass surgery performed on patients with severe cardiac dysfunction due to atypical coarctation of the aorta, it is crucial to prepare for potential circulatory collapse during anesthesia induction and the surgical procedure. This preparation includes meticulous planning of the anesthesia induction method and ensuring that ECMO can be established promptly if needed.

Stenting of aortic coarctation before coronary artery bypass surgery in an adult with acute myocardial infarction: a case report.

Background: Aortic coarctation is a major risk factor for high blood pressure and atherosclerotic disease development. Evidence is lacking regarding the treatment of acute coronary syndrome in patients with untreated aortic coarctation. Case summary: A 50-year-old male with a history of hypertension, diabetes, and haemodynamically significant untreated aortic coarctation presented to the emergency department with non-ST-elevation acute myocardial infarction. Coronary catheterization showed severe three-vessel disease. The aortic coarctation was addressed percutaneously using a covered CP stent. A quadruple coronary artery bypass surgery was conducted the following day. He was discharged home 10 days after surgery. Discussion: We describe a successful sequential approach involving the percutaneous repair of a native aortic coarctation followed by a surgical myocardial revascularization in the context of acute coronary syndrome. Discussion within a multidisciplinary Heart Team is key in patients with such complexity.

"Unkinking" the "Kink" Normalizes the Doppler Pattern.

Coarctation of the aorta (CoA) comprises 5-7% of congenital heart disease and can present as an isolated narrowing in the aortic arch just distal to the left subclavian artery or can be associated with cardiac abnormalities such as a bicuspid aortic valve, aortopathy, or ventricular septal defects. With the advances in the medical field, intervention on CoA can either be via surgical repair or endovascular stenting. Echocardiography is the mainstay in diagnosing CoA, with tomographic imaging such as magnetic resonance imaging (MRI) or computed tomography providing supplementary assessment of the aorta, valves, and collateral vessels. We present a case of a young hypertensive male who was noted to have a continuous cardiac murmur with diagnostic Doppler pattern of CoA on echocardiography that normalized soon after percutaneous stenting.

Prognostic implications of residual mild coarctation gradient after interventional repair.

There is limited data on the prognostic implications of residual mild coarctation (RMC) in patients with repaired native coarctation of the aorta (CoA). To explore the association of RMC with mid-term comorbidities in post-interventional patients, and the predictive value of the residual pressure gradient. The authors retrospectively analyzed 79 native CoA patients who received successful intervention at our hospital between October 2010 and June 2023. The outcomes of the study were late arterial hypertension (either raised blood pressure or commencement of hypotensive medications) only in normotensive patients at early follow-up and the composite mid-term comorbidities including new-onset aortic injury, re-stenosis, and re-intervention. At a median follow-up of 60 months, late hypertension and mid-term comorbidities occurred in 16 (28.1%) and nine (11.4%) patients, respectively. Multivariate Cox proportional hazard regression analysis identified invasive peak systolic CoA pressure gradient (PSPG) as the best independent predictor of both outcomes. The maximally selected rank statistics indicated 10 mm Hg as the best PSPG cut-off value for predicting late hypertension. Compared to patients with PSPG < 11 mm Hg, the cumulative event rates of both outcomes were higher in those with PSPG ≥ 11 mm Hg (log-rank test, p < .001 for both endpoints). PSPG ≥ 11 mm Hg was proved to be the independent predictor of late hypertension with a significantly increased risk. In patients with non-surgical CoA repair, the post-interventional RMC and PSPG ≥11 mm Hg are important predictors of clinical comorbidities at mid-term follow-up.

Medical-Surgical Implications of Branching Variation of Human Aortic Arch Known as Bovine Aortic Arch (BAA).

(1) Background: The aortic arch (AA) branching model is challenging, considering the multiple anatomical variations documented in existing research. The bovine aortic arch (BAA) is the most prevalent anatomical variation among these. This variant of AA branching has long been considered a nonsymptomatic malformation, having been discovered incidentally during imaging investigations for other causes. However, more recent studies have demonstrated that BAA shows a frequent association with coarctation of the aorta (CoA), thoracic aortic disease (TAD), and stroke. At the same time, given the current context of increasing activity in the fields of interventional and surgical procedures in the aorta and its branches, it is very important to know the medical-surgical implications of this anatomical variant. (2) Methods: We conducted a comprehensive review using PubMed and Embase, focusing specifically on randomized trials and cohort analyses that examined the medical-surgical implications of BAA. We assessed information related to studied groups, medical procedures, and study outcomes. Initially, we identified 8454 studies, and after rigorous evaluation, we narrowed down our review to 25 articles. (3) Discussions: The intervention consisted of assessing the risks associated with BAA through different imaging investigation methods such as computer tomographic angiography (CTA), magnetic resonance imaging (MRI), or ultrasonography (US). The following results were evaluated: the prevalence of the BAA, the importance of imaging investigations in establishing the diagnosis and the therapeutic management and monitoring the evolution of patients with the BAA, the association of the BAA with CoA, TAD, and stroke, and the potential risks of interventional treatment in patients with the BAA. (4) Conclusions: The prevalence of the BAA differs both between different ethnic groups and between genders. Advanced imaging methods such as CTA and 4D flow MRI allow detailed descriptions of supra-aortic vascular anatomy and information about blood flow velocities, direction, and turbulence in the AA. US remains an easy and valuable imaging investigation, with the potential to detect and correctly diagnose the BAA and its hemodynamic implications. Anatomical variations in the AA are associated with increased rates of TAD, CoA, and stroke, necessitating early diagnosis and increased supervision of patients with such incidentally observed abnormalities. In addition, there is a need to further develop and refine the surgical techniques used and personalize them to the individual characteristics of patients with the BAA.

The Management of Aortic Coarctation Associated with Hypoplastic Arches and Particular Arch Anatomies: A Literature Review.

The surgical management of aortic coarctation in newborns needs to ensure postoperative evolution and long-term results as much as possible. Patients with a Gothic arch have a higher rate of postoperative hypertension, while newborns with a bovine arch have higher rates of restenosis and, thus, an additional risk of mortality. Late hypertension, even in anatomically successfully repaired patients, confers a high risk for cardiovascular events. This review of the literature focuses on the management of aortic coarctations associated with hypoplastic arch and particular arch anatomies, focusing on surgical techniques and their outcomes.

Coarctation of the aorta complicated with bilateral iliac artery dissection: A rare case.

The coarctation of the aorta (CoA) combined with heart defects or cerebral artery aneurysms is more prevalent in clinical practice. However, cases of concurrent bilateral iliac artery dissection (IAD) are extremely rare and have not been reported. Here, we described a case with CoA combined with bilateral IAD. The patient, a 62-year-old male, presented with acute intermittent claudication accompanied by pain and aching in both lower limbs after walking. Following a thorough medical history inquiry and examination, the patient was diagnosed with acute bilateral IAD combined with CoA. The patient underwent endovascular treatment. Postoperatively, the aortic diameter recovered, and the bilateral IAD disappeared, yielding satisfactory therapeutic results. Conclusively, endovascular treatment of aortic coarctation combined with IAD is an effective therapeutic approach, enhancing patient survival and improving their quality of life.

Partial atrioventricular canal defect and aortic coarctation associated with variants in GDF1 and NOTCH1 genes: A case report.

BACKGROUND: A peculiar subgroup of patients with partial or complete atrioventricular canal defect exhibits a spectrum of left-sided obstructions including right ventricular dominance and aortic coarctation. The association of atrioventricular canal defect with left-sided obstructions is found in several genetic syndromes; however, the molecular basis of nonsyndromic atrioventricular canal defect with aortic coarctation is still poorly understood. Although some candidate genes for nonsyndromic atrioventricular canal defect are known, a complex oligogenic inheritance determined in some cases by the co-occurrence of multiple variants has also been hypothesized. CASE REPORT: We describe a nonsyndromic infant with mesocardia with viscero-atrial situs solitus, partial atrioventricular canal defect, mild right ventricular dominance, and coarctation of the aorta. Next generation sequencing genetic testing revealed variants in two genes, GDF1 and NOTCH1, previously reported in association with atrioventricular canal defect and left-sided obstructive lesions, respectively. CONCLUSION: The present report could support the hypothesis that the co-occurrence of cumulative variants may be considered as genetic predisposing risk factor for specific congenital heart defects.

Relationship between obesity, cardiac hemodynamics, and heart failure in adults with coarctation of aorta.

Objectives: Patients with coarctation of aorta (COA) have arterial stiffening and left ventricular (LV) diastolic dysfunction similar to patients with heart failure with preserved ejection fraction (HFpEF) and obese subjects. However, the relationship between obesity, cardiac hemodynamics, and HF in adults with COA is unknown. The purpose of this study was to compare cardiac hemodynamics and prevalence of HFpEF between COA patients with vs without obesity, and to assess the relationship between obesity and HFpEF in this population. Methods: Adults with COA who underwent right heart catheterization were divided into an obese group (body mass index, BMI > 30 kg/m2) or a non-obese group (BMI ≤ 30 kg/m2). We also selected a control group of subjects without structural heart disease and with normal invasive hemodynamics at rest (n = 36). HFpEF was defined as having clinical symptoms of HF (exertional dyspnea or fatigue), LV ejection fraction of at least 50%, and pulmonary artery wedge pressure (PAWP) greater than 15 mm Hg at rest. Results: Of 99 COA patients, 29 (29%) had obesity. The obese COA group had higher right atrial pressure and PAWP, and worse pulmonary and systemic vascular function compared with the non-obese COA group and the control group. The overall prevalence of HFpEF in adults with COA was 32%, and the prevalence was higher in COA patients with obesity (55%) compared with those without obesity (23%). Obesity was associated with HFpEF after adjustment for demographic indices, comorbidities, and vascular function. Conclusions: The abnormal hemodynamics and higher prevalence of HFpEF in COA patients with obesity underscores the need for intervention to address obesity in this population.

Review of speckle tracking analysis to measure the size, shape, and contractility of the fetal heart in fetuses with congenital heart defects.

Evaluation of the fetal heart involves two approaches. The first describes a screening protocol in which the heart is imaged in transverse planes that includes the four-chamber view (4CV), left and right outflow tracts, and the 3-vessel-tracheal view. The second approach is a fetal echocardiogram that requires additional cardiac images as well as evaluating ventricular function using diagnostic tools such as M-mode and pulsed Doppler ultrasound. Speckle tracking analysis of the ventricular and atrial endocardium of the fetal heart has focused primarily on computing longitudinal global strain. However, the technology enabling this measurement to occur has recently been adapted to enable the clinician to obtain numerous additional measurements of the size, shape, and contractility of the ventricles and atrial chambers. By using the increased number of measurements derived from speckle tracking analysis, we have reported the ability to screen for tetralogy of Fallot, D-transposition of the great arteries (D-TGA), and coarctation of the aorta by only imaging the 4CV. In addition, we have found that measurements derived from speckle tracking analysis of the ventricular and atrial chambers can be used to compute the risk for emergent neonatal balloon atrial septostomy in fetuses with D-TGA. The purpose of this review is to consolidate our experience in one source to provide perspective on the benefits of speckle tracking analysis to measure the size, shape, and contractility of the ventricles and atria imaged in the 4CV in fetuses with congenital heart defects.