Objectives: The configuration of the aortic arch, particularly a Gothic arch shape, in individuals with corrected coarctation of the aorta (CoA) has been associated with a decreased systolic wave amplitude across the arch, which could potentially impair renal perfusion and elevate the risk of arterial hypertension. This study aims to explore the relationship between the morphological characteristics of the aortic arch and their impact on renal perfusion in patients with CoA. Methods: Seventy-one subjects with corrected CoA underwent continuous 24 h ambulatory blood pressure monitoring, computed tomography to assess the aortic arch, and renal perfusion scanning. Subjects were stratified into three groups based on the height-to-width (H/W) ratio of their aortic arch: Group 1 with a H/W ratio of <0.65, Group 2 with a H/W ratio between 0.65 and 0.85, and Group 3 with a H/W ratio of >0.85. Results: Groups 1 and 2 (53,78% and 62.63%) presented with a higher hypertension prevalence of elevated blood pressure than Group 3 (38.89%). Notable variations were observed among the subjects in the time to peak perfusion (Tmax) in the left kidney across the groups. Group 1 showed a median Tmax at 0.27, Group 2 at 0.13, and Group 3 at -0.38 (p-value = 0.079). The differences in Tmax for the right kidney followed a similar trend but were not statistically significant (Group 1 at 0.61, Group 2 at 0.22, and Group 3 at 0.11; p-value = 0.229). Conclusions: This study suggests that variations in the aortic arch morphology might not significantly influence renal perfusion in CoA patients. This indicates the potential adaptability of the renal blood flow, which appears to compensate for reduced perfusion, thus minimizing adverse effects on the kidney function. This adaptability suggests an inherent physiological resilience, emphasizing the need for further targeted research to understand the specific interactions and impacts on treatment strategies for CoA.

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We previously reported short-term outcomes for stenting of aortic coarctation (CoA) (native or re-coarctation) with newer generation low-profile stents (Valeo, Formula, and Begraft stents) in children under 30 kg. We present here the medium-term outcomes of this procedure. Retrospective review of patients weighing under 30 kg who had percutaneous stent treatments for coarctation between 2012 and 2021 was performed. Clinical and procedural data were collected; 19 patients were included. The median age at the time of procedure was 5.1 [4.1-6.4] years and median weight 21.0 [17.3-22.3] kg. One patient had a history of re-coarctation. Thirteen (68%) patients were on anti-hypertensives pre-procedure. Different types of stents were used (14 Valeo™, 4 Formula® 535, 1 BeGraft), which can all be dilated to 18 mm or larger. One patient required a 9 F sheath, all others required a 7 F sheath. The narrowest diameter in the aorta increased from a median of 3.5 [3.0-4.5] to 9.4 [8.9-9.8] mm, p < 0.001; there was a reduction in the median pressure gradient across the coarctation from 35.0 [30.0-43.0] to 5.0 [0-10.0] mmHg, p < 0.001. There were no intra-procedural complications. Follow-up was for a median of 56.0 [13.0-65.0] months. Five (26%) of patients underwent re-intervention after a median time frame of 40.0 [39.5-52.0] months; four had balloon dilation, one had repeat stent implantation. Five (26%) patients were on anti-hypertensive agent(s) post-intervention. Our single centre experience demonstrates that percutaneous stenting for coarctation of aorta in children under 30 kg, with low-profile stents, had no significant complications during the median follow-up time of 56 months. This study demonstrated that the procedure is safe and effective for short and medium-term therapy in this group of patients with a 26% re-intervention rate. A quarter of patients remained on anti-hypertensive medication post stenting, emphasizing the importance of long-term follow-up.

OBJECTIVE: To characterize long-term outcomes of PHACE syndrome.
METHODS: Multicenter study with cross-sectional interviews and chart review of individuals with definite PHACE syndrome ≥10 years of age. Data from charts were collected across multiple PHACE-related topics. Data not available in charts were collected from patients directly. Likert scales were used to assess the impact of specific findings. Patient-Reported Outcomes Measurement Information System (PROMIS) scales were used to assess quality of life domains.
RESULTS: A total of 104/153 (68%) individuals contacted participated in the study at a median of 14 years of age (range 10-77 years). There were infantile hemangioma (IH) residua in 94.1%. Approximately one-half had received laser treatment for residual IH, and the majority (89.5%) of participants were satisfied or very satisfied with the appearance. Neurocognitive manifestations were common including headaches/migraines (72.1%), participant-reported learning differences (45.1%), and need for individualized education plans (39.4%). Cerebrovascular arteriopathy was present in 91.3%, with progression identified in 20/68 (29.4%) of those with available follow-up imaging reports. Among these, 6/68 (8.8%) developed moyamoya vasculopathy or progressive stenoocclusion, leading to isolated circulation at or above the level of the circle of Willis. Despite the prevalence of cerebrovascular arteriopathy, the proportion of those with ischemic stroke was low (2/104; 1.9%). PROMIS global health scores were lower than population norms by at least 1 SD.
CONCLUSIONS: PHACE syndrome is associated with long-term, mild to severe morbidities including IH residua, headaches, learning differences, and progressive arteriopathy. Primary and specialty follow-up care is critical for PHACE patients into adulthood.

Resilience is a complex, yet rather unexplored topic in patients with congenital heart disease (CHD). The goal of this study was to assess and compare resilience in children with CHD with healthy controls during the COVID-19 pandemic.
From June 2020 to June 2021, 124 children with various CHDs (14.6±2.1 years, 49 girls) and 124 matched healthy controls (14.8±2.0 years, 49 girls) completed the Resilience Scale-11 short version.
Resilience was significantly reduced in children with CHD compared with healthy controls (CHD: 59.0±10.0 vs healthy controls: 64.4±6.5, p<0.001). That reduction was prominent in all CHD subgroups except those with left heart obstruction (aortic stenosis and coarctation of the aorta) and patients with transposition of the great arteries. Complex CHD had the lowest resilience of 57.6±8.4 (p<0.001) after adjusting for age and sex according to group differences. There was no difference between native CHD and CHD with open-heart surgery (native: 59.5±12.2 vs surgery: 58.8±9.3, p=0.758).
Resilience was reduced in children and adolescents with CHD compared with healthy peers during the COVID-19 pandemic. Children with complex severity appeared to be particularly affected. These findings emphasise continued efforts to provide a holistic and multidisciplinary approach in medical aftercare of these patients and their families.

We aimed to explore the feasibility of 4D flow magnetic resonance imaging (MRI) for patients undergoing thoracic aorta endovascular repair (TEVAR). We retrospectively evaluated ten patients (two female), with a mean (±standard deviation) age of 61 ± 20 years, undergoing MRI for a follow-up after TEVAR. All 4D flow examinations were performed using a 1.5-T system (MAGNETOM Aera, Siemens Healthcare, Erlangen, Germany). In addition to the standard examination protocol, a 4D flow-sensitive 3D spatial-encoding, time-resolved, phase-contrast prototype sequence was acquired. Among our cases, flow evaluation was feasible in all patients, although we observed some artifacts in 3 out of 10 patients. Three individuals displayed a reduced signal within the vessel lumen where the endograft was placed, while others presented with turbulent or increased flow. An aortic endograft did not necessarily hinder the visualization of blood flow through 4D flow sequences, although the graft could generate flow artifacts in some cases. A 4D Flow MRI may represent the ideal tool to follow up on both healthy subjects deemed to be at an increased risk based on their anatomical characteristics or patients submitted to TEVAR for whom a surveillance protocol with computed tomography angiography would be cumbersome and unjustified.

Femoral arterial access is challenging in infants. Furthermore, after cardiac catheterization, femoral arterial occlusion (FAO) can be underestimated and easily missed on physical examination. Ultrasound is recommended for femoral arterial access and the correct diagnosis of FAO; however, few studies have reported its effectiveness.To investigate the frequency and risk factors of acute loss of the arterial pulse (ALAP) and persistent femoral arterial occlusion (PFAO) in infants with congenital heart disease who underwent ultrasound-guided femoral arterial access (US-GFAA) and were diagnosed with FAO by ultrasound.We obtained data related to patient characteristics, access variables of US-GFAA, and ultrasonography findings of the femoral artery from our pediatric cardiac catheterization database between August 2017 and August 2022. We divided the patients into groups based on the presence of ALAP and PFAO. We identified ALAP in 99 (19%) patients and PFAO in 21(4%) of 522 patients in the study. The median patient age was 132 days (interquartile range: 75-202 days). The logistic regression analysis identified younger age, aortic coarctation, previous catheterization of the same femoral artery, larger sheath size (5F), and longer duration of cannulation as independent risk factors for ALAP and younger age as an independent risk factor for PFAO (all p < 0.05). This study showed that younger age at procedure was a risk factor for both ALAP and PFAO, while aortic coarctation, previous arterial catheterization, use of a larger sheath and longer duration of cannulation were risk factors for ALAP in infants. The majority of FAO is reversible and secondary to arterial spasm, and the of FAO increases inversely with patient age.

A fetal cardiology consultation involves using two-dimensional drawings to explain the cardiac anatomy which can result in inherent variation in how the congenital heart disease (CHD) is conveyed. In this pilot study, we incorporated three-dimensional printed (3DP) models into fetal counseling to demonstrate feasibility and evaluate the impact on parental knowledge, understanding, and anxiety. Parents with a prenatal diagnosis of a muscular ventricular septal defect (VSD) and/or coarctation of aorta were enrolled. Providers were randomized into a Model or Drawing Group and crossed after six months. Parents completed a survey after the consultation which evaluated knowledge of the CHD lesion, expectant surgical management, self-rated understanding, attitude towards the visualization tool, and anxiety. Twenty-nine patients enrolled over a 12 month period. Twelve consultations were done for coarctation of aorta, 13 for VSD, and four for coarctation with a VSD. Both Model and Drawing groups scored similarly in self-reported understanding and confidence, helpfulness of and improvement in communication with the visualization tool. The Model group had higher scores on questions related to the CHD anatomy and surgical intervention [5 [4-5] versus 4 [3.5-5]], p = 0.23 although this didn\'t reach statistical significance. For the majority (83%) of consultations, the cardiologist agreed that the 3D model improved communication. In this pilot study, we demonstrate the use of 3DP cardiac models during prenatal CHD counseling is feasible and produces results related to parental understanding and knowledge that are equal to and possibly better than the current standard of care.

BACKGROUND: Infantile hemangiomas (IHs) can be part of PHACE (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies) syndrome when they are segmental, extensive, and located on the face or neck. The initial assessment is codified and well known, but there are no recommendations for the follow-up of these patients. The aim of this study was to assess the long-term prevalence of different associated abnormalities.
METHODS: Patients with a history of large segmental IHs of the face or neck. diagnosed between 2011 and 2016 were included in the study. Each patient underwent an ophthalmological, dental, ENT (ear, nose, and throat), dermatological, neuro-pediatric, and radiological assessment at inclusion. Eight patients including five with PHACE syndrome were prospectively evaluated.
RESULTS: After a mean follow-up of 8.5 years, three patients presented with an angiomatous aspect of the oral mucosa, two with hearing loss, and two with otoscopic abnormalities. No patients developed ophthalmological abnormalities. The neurological examination was altered in three cases. Brain magnetic resonance imaging follow-up was unchanged in three out four patients and revealed atrophy of the cerebellar vermis in 1 patient. Neurodevelopmental disorders were found in five of the patients and learning difficulties were observed in five patients. The S1 location appears to be associated with a higher risk of neurodevelopmental disorders and cerebellar malformations, while the S3 location was associated with more progressive complications, including neurovascular, cardiovascular, and ENT abnormalities.
CONCLUSIONS: Our study reported late complications in patients with a large segmental IH of the face or neck, whether associated with PHACE syndrome or not, and we proposed an algorithm to optimize the long-term follow-up.

UNASSIGNED: Stanford type B aortic dissection (TBAD) is a rare cardiovascular emergency with rapid onset and great harm. Currently, no relevant studies have analyzed the difference in clinical benefits of endovascular repair in patients with TBAD in acute and non-acute stages. To investigate the clinical characteristics and prognosis of endovascular repair in patients with TBAD at different surgical timing.
UNASSIGNED: The medical records of 110 patients with TBAD from June 2014 to June 2022 were retrospectively selected as the study subjects. The patients were divided into an acute group (onset time ≤14 days) and a non-acute group (onset time >14 days) according to the time to surgery, and the two groups were compared in terms of surgery and hospitalization, aortic remodeling, and follow-up results. Univariate and multivariate logistic regression were used to analyze the factors affecting the prognosis of TBAD treated with endoluminal repair.
UNASSIGNED: The proportion of pleural effusion, heart rate, the rate of complete thrombosis of the false lumen and the difference in the maximum diameter of the false lumen in the acute group were higher than those in the non-acute group (P=0.015, <0.001, 0.029, <0.001). The length of hospital stay and the maximum postoperative diameter of the false lumen was lower than in the non-acute group (P=0.001, 0.004). There was no statistically significant difference between the two groups in the technical success rate, overlapping stent length, overlapping stent diameter, immediate postoperative contrast type I endoleak, incidence of renal failure, ischemic disease, endoleaks, aortic dilatation, retrograde type A aortic coarctation, and death (P=0.386, 0.551, 0.093, 0.176, 0.223, 0.739, 0.085, 0.098, 0.395, 0.386); coronary artery disease [odds ratio (OR) =6.630, P=0.012], pleural effusion (OR =5.026, P=0.009), non-acute surgery (OR =2.899, P=0.037), and involvement of the abdominal aorta (OR =11.362, P=0.001) were all independent risk factors affecting the prognosis of TBAD treated with endoluminal repair.
UNASSIGNED: Acute phase endoluminal repair of TBAD may contribute to aortic remodeling, and the prognosis of TBAD patients can be assessed clinically in combination with coronary artery disease, pleural effusion, and involvement of the abdominal aorta for early intervention to reduce the associated mortality.