Search Results (76)

Background: Despite anatomically successful surgical correction, postoperative hypertension remains a significant concern in patients with coarctation of the aorta, even when repair is performed during infancy. Inflammation and neurohormonal activation have been proposed as contributing mechanisms. Objective: To investigate the association between preoperative inflammatory biomarkers—specifically the interleukin-6 (IL-6) to tumor necrosis factor-alpha (TNF-α) ratio—and the development of hypertension in patients with successful isolated coarctation of the aorta repair under one year of age. Methods: This observational study included 42 infants with isolated CoA. Clinical, echocardiographic, and biochemical parameters were analyzed. Preoperative plasma levels of IL-6, TNF-α, von Willebrand factor (vWF), and renin were measured. Patients were classified based on hypertensive status at 2-year follow-up. Univariate and multivariate logistic regression analyses were performed to identify predictors of postoperative hypertension. Results: Hypertension was diagnosed in 16 out of 41 patients (39%) at follow-up. A preoperative IL-6/TNF-α ratio > 2 was an independent predictor in multivariate analysis for postoperative HT (OR = 6.1, 95% CI = 6.23–9.31, p = 0.02). Conclusions: In this small single-center cohort, an elevated IL-6/TNF-α ratio was associated with postoperative hypertension after coarctation repair. These exploratory findings should be considered hypothesis-generating and warrant confirmation in larger, multicenter studies. Full article

Background/Objectives: Complex cases in pediatric surgery involving multiple congenital anomalies pose significant diagnostic and therapeutic challenges. These conditions require coordinated interdisciplinary care tailored to the individual patient. We present a case of syndromic congenital anomalies in a neonate, later diagnosed with CHARGE syndrome, to illustrate the importance of staged, multidisciplinary management. Methods: A 34-year-old woman in her third pregnancy developed significant polyhydramnios at 31 weeks of gestation, followed by preterm labor. The neonate presented with esophageal atresia with tracheoesophageal fistula (EA/TEF), intrathoracic stomach, aortic coarctation, patent ductus arteriosus, atrial septal defect, and bilateral choanal atresia. A structured treatment protocol was developed and implemented at Klinikum Stuttgart by an interdisciplinary team comprising gynecology, pediatric surgery, cardiology, ENT, neonatology, and genetics. Results: Initial pediatric surgical procedures included ligation of the tracheoesophageal fistula, repositioning of the intrathoracic stomach, and primary esophageal anastomosis. Cardiovascular anomalies were managed through staged interventions. Bilateral choanal atresia was surgically corrected. Genetic testing confirmed CHARGE syndrome. Postoperative care included respiratory support, enteral nutrition, and regular esophageal dilations. Due to persistent reflux esophagitis, antireflux surgery is planned. Conclusions: This case underscores the importance of a highly individualized and interdisciplinary approach in the management of syndromic congenital anomalies. The presence of CHARGE syndrome with multiple system involvement required careful staging of surgical interventions and long-term coordination of follow-up care. Early genetic diagnosis and integrated team planning were critical in optimizing outcomes in this complex neonatal case. Full article

Paediatric hypertension presents significant perioperative challenges due to its variable aetiology and potential for end-organ damage. The prevalence varies with age and is associated with both primary and secondary causes, which differ markedly from adult hypertension. This review outlines the classification, diagnosis, and causes of paediatric hypertension to provide context for its management in the perioperative setting. Emphasis is placed on the identification and preoperative optimisation of hypertension, intraoperative blood pressure control, and the management of hypertensive crises. Specific perioperative strategies, including anaesthetic planning, pharmacological interventions, and postoperative monitoring, are discussed. Specific conditions such as phaeochromocytoma and aortic coarctation require tailored pharmacological strategies and close interdisciplinary collaboration. Postoperative care in an intensive care setting is essential for monitoring complications and achieving long-term blood pressure control. Effective perioperative management of paediatric hypertension requires early identification, thorough preoperative assessment, and prompt intraoperative and postoperative intervention. Multidisciplinary care and an understanding of paediatric specific pathophysiology are key to reducing morbidity and improving outcomes. Full article

Introduction: Patch aortoplasty for aortic coarctation may have a high rate of late aneurysm and pseudoaneurysm formation, with an elevated risk of rupture and subsequent mortality when it occurs. Case: A 55-year-old male with irregular follow-up after patch aortoplasty for aortic coarctation 15 years ago, presented with a late post-coarctectomy aortic arch pseudoaneurysm and re-coarctation. Through a redo thoracotomy and under left heart bypass, the pseudoaneurysm and previous patch were partially excised, and reconstruction with interposition of a “Y” shaped Dacron graft was performed, in order to preserve the patency of the left subclavian artery. The postoperative course was uneventful, and at 6 months, the CT-angio control showed adequate graft patency. Comprehensive Literature Review: A comprehensive literature review with the primary focus on the different late complications after CoA repair (post-aortic coarctation aneurysms and recoarctation), and outcomes of open and endovascular approaches was performed. Conclusions: Open surgical repair for post-coarctectomy pseudoaneurysm is feasible with excellent results. However, due to the technical challenges, it may be performed with better results in high-volume aortic centers. Full article

Background: The aim of this study was to evaluate the clinical outcomes of adult patients who underwent repair for delayed isolated coarctation of the aorta (CoA). In addition, we aimed to assess the immediate results of the interventions and long-term follow-up data. Methods: A total of 119 adult patients who were operated on for CoA and remained under follow-up during a 25-year study period were retrospectively analyzed. The pre-, intra-, and postoperative data of the patients were recorded. The surgical methods applied preoperatively and/or postoperatively were classified based on the primary issue as interventions involving the aorta and those not involving the aorta. Results: Of the patients, 81 were males and 28 were females with a mean age of 30.55 ± 10.84 (range: 18 to 67) years. The mean follow-up was 74.79 ± 61.71 (range: 0 to 271) months. A statistically significant difference was found between the presence of pre- and postoperative hypertension and the incidence of postoperative hypertension in patients under the age of 30 (p = 0.021 and p = 0.039, respectively). A total of 13 patients underwent surgery for recoarctation. The overall rate of additional cardiac surgery was 11.80%. The presence of preoperative hypertension and valve morphology (normal vs. bicuspid) were found to be statistically significant for the need for surgery before and after CoA repair. Conclusions: Patients with repaired CoA should be closely monitored due to the lifelong risk of developing complications. In particular, we recommend annual follow-up for patients with BAV. Full article

Introduction: Among aortic diseases in children, congenital defects such as coarctation of the aorta (CoA), interrupted aortic arch (IAA), hypoplastic aortic arch (HAA), and hypoplastic left heart syndrome (HLHS) predominate. Tissue patches are applied in pediatric cardiovascular surgery for the repair of congenital aortic defects as a filling material to replenish missing tissue or as a substitute material for the complete reconstruction of the vascular wall along the course of the vessel. This retrospective single-center study aimed to present the safety and feasibility of extracellular matrix (ECM) biological scaffolds in pediatric aortic surgery. Patients and methods: There were 26 patients (17 newborns and nine children), who underwent surgical procedures in the Department of Pediatric Cardiac Surgery (Poznań, Poland) between 2023 and 2024. The patients’ population was divided into two subgroups according to the hemodynamic nature of the primary diagnosis of the congenital heart defect and the performed pediatric cardiovascular surgery. The first group included 18 (72%) patients after aortic arch repair for interrupted aortic arch and/or hypoplastic aortic arch, while the second group included seven (28%) patients after aortopulmonary anastomosis. In the first group, patches were used to reconstruct the aortic arch by forming an artificial arch with three separate patches sewn together, primarily addressing the hypoplastic or interrupted segments. In the second group, patches were applied to augment the anastomosis site between the pulmonary trunk and the aortic arch, specifically at the connection points in procedures, such as the Damus–Kaye–Stansel or Norwood procedures. The analysis was based on data acquired from the national cardiac surgery registry. Results: The overall mortality in the presented group was 15%. All procedures were performed using median sternotomy with a cardiopulmonary bypass. The cardiopulmonary bypass (CPB) and aortic cross-clamp (AoX) median times were 144 (107–176) and 53 (33–79) min, respectively. There were two (8%) cases performed in deep hypothermic circulatory arrest (DHCA). The median postoperative stay in the intensive care unit (ICU) was 284 (208–542) h. The median mechanical ventilation time was 226 (103–344) h, including 31% requiring prolonged mechanical ventilation support. Postoperative acute kidney failure requiring hemodiafiltration (HDF) was noticed in 12% of cases. Follow-up data, collected via routine transthoracic echocardiography (TTE) and clinical assessments over a median of 418 (242.3–596.3) days, showed no evidence of patch-related complications such as restenosis, aneurysmal dilation, or calcification in surviving patients. One patient required reintervention on the same day due to a significantly narrow ascending aorta, unrelated to patch failure. No histological data from explanted patches were available, as no patches were removed during the study period. The median (Q1–Q3) hospitalization time was 21 (16–43) days. Conclusions: ProxiCor^® biological patches derived from the extracellular matrix can be safely used in pediatric patients with congenital aortic arch disease. Long-term follow-up is necessary to confirm the durability and growth potential of these patches, particularly regarding their resistance to calcification and dilation. Full article

Compartment boundaries divide the embryo into segments with distinct fates and functions. In the vascular system, compartment boundaries organize endothelial cells into arteries, capillaries, and veins that are the fundamental units of a circulatory network. For vascular smooth muscle cells (SMCs), such boundaries produce mosaic patterns of investment based on embryonic origins with important implications for the non-uniform distribution of vascular disease later in life. The morphogenesis of blood vessels requires vascular cell movements within compartments as highly-sensitive responses to changes in fluid flow shear stress and wall strain. These movements underline the remodeling of primitive plexuses, expansion of lumen diameters, regression of unused vessels, and building of multilayered artery walls. Although the loss of endothelial compartment boundaries can produce arterial–venous malformations, little is known about the consequences of mislocalization or the failure to form SMC-origin-specific boundaries during vascular development. We propose that the failure to establish a normal compartment boundary between cardiac neural-crest-derived SMCs of the 6th pharyngeal arch artery (future ductus arteriosus) and paraxial-mesoderm-derived SMCs of the dorsal aorta in mid-gestation embryos leads to aortic coarctation observed at birth. This model raises new questions about the effects of fluid flow dynamics on SMC investment and the formation of SMC compartment borders during pharyngeal arch artery remodeling and vascular development. Full article

Background: Coarctation of the aorta (CoA) represents 5% to 7% of all congenital heart diseases. Surgery and interventional methods offer great short-term results, but the occurrence of postoperative hypertension associated with cardiovascular and cerebral vascular disease increases mortality and morbidity in the long term. This study aims to investigate risk factors associated with postoperative hypertension in pediatric patients with early repair of isolated aortic coarctation. Subjects and Methods: A total of 41 patients with isolated aortic coarctation were included. The mean age was 35.3 ± 46.34 days. Early repair under one month was performed in 65.9% of patients. In all except two patients, end-to-end anastomosis was used. A follow-up at two years revealed an incidence of 58.5% of hypertension. Using logistic regression, preoperative renin plasma concentration above the upper normal level (46.1 μUI/mL) was independently associated with the occurrence of hypertension (OR = 2.49, 95% CI = 2.001–5.03, p = 0.001). Conclusion: Coarctation of the aorta is not just a simple mechanical obstruction of the aorta and should be seen and managed as a systemic disease. Abnormal preoperative renin concentrations were independently associated with the occurrence of HT at follow-up, suggesting that vascular dysfunction could play a role in hypertension development after successful CoA repair, negatively influencing the long-term prognostic of these patients. Full article

Computational fluid dynamics (CFD) is a tool that allows for the analysis of otherwise unobservable blood flow patterns. In the context of medicine, CFD enables researchers to better understand acute and chronic pathophysiology as well as utilize modeling tools to predict blood flow patterns in response to surgical intervention. Such a tool is particularly useful in the field of congenital heart disease (CHD), where complex geometries and patient-specific pathology are common. Research applying CFD to study CHDs has significantly grown in the last twenty years, with new methodologies and recommendations being published at an even faster pace in the last decade. Many currently available reviews are focused on a particular area of progress or on the technical approaches to CFD geared toward the clinician. This review focuses on CFD application within the major domains of CHD research, specifically single ventricle defects and aortic coarctation, reviewing consensus seminal work while highlighting more recent avenues of study. Balancing discussion of CFD parameters with potential clinical implications of study results, this review not only aims to provide cardiovascular professionals context for the technical advancements being made in the field but also a sense of contemporary CFD’s utility in clinical practice. Full article

Desmoid tumors are a rare entity, especially in the pediatric population. There are no reports of such a tumor in newborns. They are associated with high rates of morbidity and mortality, even though they are benign soft tissue tumors. This is due to them exhibiting locally aggressive growth with the compression or invasion of adjacent structures. Abdominal localization is most commonly reported, although there are reports of mediastinal desmoid tumors. We present the case of a 6-day male patient with a mediastinal desmoid tumor that led to severe aortic coarctation with hemodynamic instability. The tumor also compressed the left pulmonary artery and obstructed the left main bronchus. The initial management consisted of successful emergency surgery with partial resection of the tumor mass and coarctation repair. In the postoperative setting, the patient evolved with severe respiratory dysfunction which was managed with tracheostomy, allowing weaning the child from the mechanical ventilation one month after surgery, along with chemotherapy. We also review the literature, focusing on the management of desmoid tumors. Full article

Background/Objectives: Bicuspid aortic valve (BAV) is a prevalent congenital heart defect that continues to present a significant challenge in the management of paediatric patients. The assessment of left ventricle systolic function is typically conducted through the measurement of the left ventricular ejection fraction. Currently, left ventricle global longitudinal strain (LV GLS) is regarded as a more sensitive indicator, enabling the quantitative assessment of global and segmental ventricular function through the determination of myocardial deformation. Methods: A prospective study was conducted between 10 January 2023 and 10 January 2024 in a tertiary paediatric cardiology referral centre. The study enrolled children aged 6 to 17 years with BAV who were undergoing periodic evaluation, as well as a control group. The primary objective was to analyse the systolic function (global and segmental LV) using the classical method (LV EF) and speckle tracking echocardiography (STE). Results: The study group comprised 73 patients with a mean age of 13 years and was predominantly male. The control group comprised 55 patients. The phenotype IB with aortic regurgitation (AR) was the most prevalent. The results of the STE evaluation in the control group demonstrated mean GLS values between −22.1% and −22.8%. A comparison of the BAV group and the control group revealed a significant difference in GLS for the apical four-chamber view (p = 0.022). Conclusions: Although the analysis of global LV function demonstrated normal values of EF in patients with BAV, the strain analysis revealed significantly reduced strain in the inferior segment and in the apical four-chamber view, as well as in the anterior segment. Further investigation is required to determine whether reduced LV GLS in paediatric patients with BAV will ultimately result in the development of clinical heart failure. Additionally, it is necessary to ascertain whether this can identify patients with subclinical heart failure and whether early detection can result in a reduction in morbidity. Full article

Background: Aortic coarctation, a condition characterized by localized narrowing of the aorta, can be managed with either surgical or endovascular techniques. This study aims to compare these approaches concerning long-term outcomes, particularly re-coarctation rates and late arterial hypertension. Methods: We retrospectively analyzed data from patients with native, isolated aortic coarctation treated by surgical or endovascular methods between 2015 and 2024. Clinical and demographic data were collected from electronic health records. Blood pressure was measured using oscillometric devices, and transthoracic echocardiography (TTE) was performed by an experienced sonographer. The primary endpoint was to identify which treatment predicted re-coarctation during follow-up, while the secondary endpoint assessed the incidence of late arterial hypertension. Results: Sixty-nine patients were included, with a mean age of 18.14 ± 8.18 years (median 16 years; range 8 to 37 years) and a median follow-up of 3 years (range 6 months to 8 years). Of these, 67 (97.1%) underwent elective repairs. Repair techniques included endovascular treatment (24.6%), surgical end-to-end anastomosis (47.8%), and surgical patchplasty (27.5%). The endovascular group was significantly older (29.82 ± 5.9 years vs. 14.33 ± 4.25 years, p = 0.056) and had shorter procedure durations and hospital stays. One-year freedom from reintervention was significantly higher in the surgical group (98.7%) compared to the endovascular group (88.23%) (p < 0.001). Conclusions: Both techniques effectively treat aortic coarctation, but surgical repair offers better long-term outcomes, while endovascular repair provides shorter recovery times. These findings should inform the choice of treatment modality based on patient-specific factors and clinical priorities. Full article

Background: Aortic coarctation (CoA) is a congenital heart disease affecting 5–8% of patients, with long-term complications persisting despite successful correction. Stress echocardiography (SE) is increasingly used for evaluating cardiac function under stress, yet its role in repaired CoA remains under-explored. Objective: This study aimed to assess the predictive value of SE and myocardial strain in repaired CoA patients with a history of hypertension without significant gradients or with borderline gradients at rest. Methods: Between June 2020 and March 2024, we enrolled 35 consecutive CoA patients with successful repairs and either a history of hypertension or borderline Doppler gradients. Baseline and peak exercise echocardiographic measurements, including left ventricular mass index (LVMi) and global longitudinal strain (LVGLS), were recorded. Patients were followed for up to 4 years. Results: At baseline, the positive SE group had higher systolic blood pressure (SBP) and diastolic blood pressure (DBP) compared to the negative SE group. The positive SE group also exhibited significantly higher basal and peak trans-isthmic gradients. Positive SE was found in 45.7% of patients, with 68.7% of these requiring re-intervention during follow-up. A peak trans-isthmic gradient > 61 mmHg during exercise predicted recoarctation with 100% sensitivity and 71% specificity (AUC = 0.836, p < 0.004). Conclusions: SE identifies at-risk patients post-CoA repair, aiding in early intervention. A peak trans-isthmic gradient > 61 mmHg during exercise is a strong predictor of recoarctation. These findings support incorporating SE into routine follow-up protocols for CoA patients, particularly those with a history of hypertension and borderline gradients, to improve long-term outcomes and quality of life. Full article

Aortic coarctation surgery in pediatric patients requires the placement of two arterial cannulas to monitor pressure gradients and surgical correction adequacy. Near-infrared spectroscopy (NIRS) monitoring provides insight into regional blood flow. This study aimed to investigate the correlation between NIRS values and invasive arterial pressures, exploring NIRS monitoring as a potential substitute for arterial cannulation. In a cohort of 21 consecutive pediatric patients undergoing aortic coarctation surgery, recordings of NIRS and invasive arterial pressure values were evaluated at various time intervals. Pearson correlation evaluated the relationship between NIRS values and invasively measured arterial pressures. Moderate to strong correlations were observed between the mean arterial pressure (MAP) of the upper and lower arteries and cerebral (rSO₂-C) and somatic (rSO₂-S) NIRS values 5 min after cross-clamp placement (r = 0.621, p = 0.003; r = 0.757, p < 0.001). Strong correlations were found 15 min after cross-clamp placement (r = 0.828, p = 0.002; r = 0.783, p = 0.004). Before transfer to the ICU, a strong correlation existed between the upper artery MAP and rSO₂-C (r = 0.730, p < 0.001), but there was no correlation between the lower artery MAP and rSO₂-S. These findings are promising, but further studies are required to validate it as a reliable substitute for invasive pressure monitoring in this patient population. Full article

Objectives: Complications after aortic coarctation repair are associated with high mortality and require surgical or endovascular reintervention. For patients unsuitable for endovascular therapies, reoperation remains the only therapeutic option. However, surgical experience and up-to-date follow-up data concerning this overall rare entity in the spectrum of aortic reoperations are still highly limited. Thus, the aim of this study was to analyze the short-term outcomes and long-term survival of patients undergoing surgical descending aorta repair after previous coarctation repair in a high-volume unit. Methods: We present a retrospective single-center analysis of 25 patients who underwent open descending aorta replacement after initial coarctation repair. The surgical history, concomitant cardiovascular malformations, and preoperative characteristics as well as postoperative complications and long-term survival were analyzed. Results: The mean age at operation was 45.4 ± 12.8 years. A proportion of 68% (n = 17) of the patients were male. The most common complication necessitating reoperation after coarctation repair was aneurysm formation (68%) and re-stenosis (16%). The average time between initial repair and reoperation was 26.3 ± 9.9 years. Technical success was achieved in all the operations, while recurrent nerve damage (24%) and bleeding requiring rethoracotomy (20%) were identified as the most common perioperative complications. The one-year mortality was 0% and the overall long-term survival was 88% at 15 years. Conclusions: Open surgical descending aorta replacement can be performed safely and with excellent survival outcomes even in the challenging subgroup of patients after previous coarctation repair. Thus, reoperation should be considered a feasible approach for patients who are unsuitable for endovascular therapies. Nonetheless, concomitant cardiovascular anomalies and frequent preoperations may complicate the redo operation in this patient population. Full article