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BMC Pediatrics May 2024Patients with Turner syndrome (TS) face an increased risk of developing aortic dilatation (AD), but diagnosing AD in children presents greater complexity compared to...
BACKGROUND
Patients with Turner syndrome (TS) face an increased risk of developing aortic dilatation (AD), but diagnosing AD in children presents greater complexity compared to adults. This study aimed to investigate the application of various assessment indicators of AD in Chinese children and adolescents with TS.
METHODS
This study included TS patients admitted to Shenzhen Children's Hospital from 2017 to 2022. Cardiovascular lesions were diagnosed by experienced radiologists. Patients without structural heart disease were divided into different body surface area groups, then the Chinese TS population Z-score (CHTSZ-score) of the ascending aorta was calculated and compared with other indicators such as aortic size index (ASI), ratio of the ascending to descending aortic diameter (A/D ratio), and TSZ-score (Quezada's method).
RESULTS
A total of 115 TS patients were included, with an average age of 10.0 ± 3.7 years. The incidences of the three most serious cardiovascular complications were 9.6% (AD), 10.4% (coarctation of the aorta, CoA), and 7.0% (bicuspid aortic valve, BAV), respectively. The proportion of developing AD in TS patients aged ≥ 10 years was higher than that in those < 10 years old (16.6% vs. 1.8%, P = 0.009), and the proportion of patients with CoA or BAV who additionally exhibited AD was higher than those without these conditions (31.6% vs. 5.2%, P < 0.001). The ASI, A/D ratio, TSZ-score, and CHTSZ-score of the 11 patients with AD were 2.27 ± 0.40 cm/m, 1.90 ± 0.37, 1.28 ± 1.08, and 3.07 ± 2.20, respectively. Among the AD patients, only 3 cases had a TSZ-score ≥ 2, and 2 cases had a TSZ-score ≥ 1. However, based on the assessment using the CHTSZ-score, 6 patients scored ≥ 2, and 5 patients scored ≥ 1. In contrast, the TSZ-score generally underestimated the aortic Z-scores in Chinese children with TS compared to the CHTSZ-score.
CONCLUSIONS
The applicability of ASI and A/D ratio to children with TS is questionable, and racial differences can affect the assessment of TSZ-score in the Chinese population. Therefore, establishing the CHTSZ-score specifically tailored for Chinese children and adolescents is of paramount importance.
Topics: Humans; Turner Syndrome; Child; Adolescent; Female; China; Dilatation, Pathologic; Male; Retrospective Studies; Aorta; Aortic Coarctation; Bicuspid Aortic Valve Disease; Child, Preschool; Incidence; East Asian People
PubMed: 38720245
DOI: 10.1186/s12887-024-04783-2 -
Cardiovascular Diabetology May 2024In observational and experimental studies, diabetes has been reported as a protective factor for aortic dissection. 3-Hydroxybutyrate, a key constituent of ketone...
BACKGROUND
In observational and experimental studies, diabetes has been reported as a protective factor for aortic dissection. 3-Hydroxybutyrate, a key constituent of ketone bodies, has been found to favor improvements in cardiovascular disease. However, whether the protective effect of diabetes on aortic dissection is mediated by 3-hydroxybutyrate is unclear. We aimed to investigate the causal effects of diabetes on the risk of aortic dissection and the mediating role of 3-hydroxybutyrate in them through two-step Mendelian randomization.
MATERIALS AND METHODS
We performed a two-step Mendelian randomization to investigate the causal connections between diabetes, 3-hydroxybutyrate, and aortic dissection and calculate the mediating effect of 3-hydroxybutyrate. Publicly accessible data for Type 1 diabetes, Type 2 diabetes, dissection of aorta and 3-hydroxybutyrate were obtained from genome-wide association studies. The association between Type 1 diabetes and dissection of aorta, the association between Type 2 diabetes and dissection of aorta, and mediation effect of 3-hydroxybutyrate were carried out separately.
RESULTS
The IVW method showed that Type 1 diabetes was negatively associated with the risk of aortic dissection (OR 0.912, 95% CI 0.836-0.995), The weighted median, simple mode and weighted mode method showed consistent results. The mediated proportion of 3-hydroxybutyrate on the relationship between Type 1 diabetes and dissection of aorta was 24.80% (95% CI 5.12-44.47%). The IVW method showed that Type 2 diabetes was negatively associated with the risk of aortic dissection (OR 0.763, 95% CI 0.607-0.960), The weighted median, simple mode and weighted mode method showed consistent results. 3-Hydroxybutyrate does not have causal mediation effect on the relationship between Type 2 diabetes and dissection of aorta.
CONCLUSION
Mendelian randomization study revealed diabetes as a protective factor for dissection of aorta. The protective effect of type 1 diabetes on aortic dissection was partially mediated by 3-hydroxybutyrate, but type 2 diabetes was not 3-hydroxybutyrate mediated.
Topics: Mendelian Randomization Analysis; Humans; Aortic Dissection; 3-Hydroxybutyric Acid; Diabetes Mellitus, Type 2; Risk Factors; Aortic Aneurysm; Diabetes Mellitus, Type 1; Genome-Wide Association Study; Risk Assessment; Genetic Predisposition to Disease; Protective Factors; Phenotype; Biomarkers; Mediation Analysis
PubMed: 38715052
DOI: 10.1186/s12933-024-02266-3 -
Heliyon May 2024Prolonged circulatory arrest time is an independent risk factor for postoperative adverse events of type A aortic dissection (TAAD) surgery. Further reduction of the...
BACKGROUND
Prolonged circulatory arrest time is an independent risk factor for postoperative adverse events of type A aortic dissection (TAAD) surgery. Further reduction of the circulatory arrest time is essential to improve surgical outcomes. This study aimed to evaluate the safety and effectiveness of the novel Sutureless Integrated Stented (SIS) graft prosthesis in an animal experiment.
MATERIALS AND METHODS
Straight type of the SIS graft prosthesis was implanted into the descending aorta of 10 adult male sheep, and the use of the device was scored on a scale of 1-10. Aortic digital subtraction angiography (DSA) was performed at 4, 14, and 26 weeks to investigate the prostheses. After 26 weeks, the animals were sacrificed for histological analysis.
RESULTS
The immediate success rate of the surgery was 100 %, and the overall mean score of the use of the device was 9.65 ± 0.99. Three animals died from non-device-related causes during follow-up. Aortic DSA showed filling defects in 5 animals. Histological analysis revealed that all prostheses were intact. Except for 2 early deaths, the other 8 prostheses were endothelialized with mild inflammation, foreign body reactions, and intimal fibrosis. The mean cross-sectional area of the sutureless region was reduced by 26.4 % (range, 1.3-39.1 %).
CONCLUSIONS
The safety and effectiveness of the novel SIS graft prosthesis were acceptable, and the delivery system exhibited a promising performance. Using the SIS graft prosthesis in TAAD surgery was expected to simplify the procedures and shorten the circulatory arrest time. Further large-scale clinical trials are required to verify these findings.
PubMed: 38711632
DOI: 10.1016/j.heliyon.2024.e30323 -
Kidney International Reports May 2024Aortic root dilatation is a reported cardiovascular sequela seen in children and young people (CYP) with chronic kidney disease (CKD) but has yet to be described in...
INTRODUCTION
Aortic root dilatation is a reported cardiovascular sequela seen in children and young people (CYP) with chronic kidney disease (CKD) but has yet to be described in those with autosomal dominant polycystic kidney disease (ADPKD).
METHODS
Single center, cross-sectional study in a dedicated ADPKD clinic. Echocardiograms were evaluated for the presence of dilatation (defined by a z-score ≥2 [≥99th percentile] SDs from the mean) at 4 standardized locations, namely the aortic valve annulus, sinuses of Valsalva (SoV), sinotubular junction (STJ), and the ascending aorta. Measurements were compared with a control group to assess prevalence, severity, and determinants of aortic dilatation.
RESULTS
Ninety-seven children, median age (interquartile range) of 9.3 (6.1, 13.6) years were compared with 19 controls without ADPKD or other CKD. The prevalence of dilatation ranged from 5.2% to 17% in ADPKD, depending on anatomical location with no aortic dilatation identified in the control group. In multivariable regression, aortic root dilatation was significantly associated with cyst burden at the aortic valve annulus and SoV (β = 0.42 and β = 0.39, both < 0.001), with age at SoV (β = -0.26, = 0.02), systolic blood pressure (SBP) z-score at SoV (β = -0.20, = 0.04) and left ventricular mass index (LVMI) at SoV and STJ (β = 0.24, = 0.02 and β = 0.25, = 0.03, respectively) following adjustment for age, sex (male or female), body mass index (BMI) z-score, estimated glomerular filtration rate (eGFR), SBP z-score, and LVMI.
CONCLUSION
Our data suggests increased prevalence of aortic root and ascending aortic dilatation in CYP with ADPKD compared with controls. Further studies are needed to understand the pathogenesis and its contribution to the high cardiovascular morbidity in ADPKD.
PubMed: 38707792
DOI: 10.1016/j.ekir.2024.02.007 -
BMC Cardiovascular Disorders May 2024Some individuals who go to fitness centers for various purposes perform resistance exercise (RE) alone, while others engage in combined exercise (CE) by including cardio... (Comparative Study)
Comparative Study
Some individuals who go to fitness centers for various purposes perform resistance exercise (RE) alone, while others engage in combined exercise (CE) by including cardio exercises along with RE. Studying the effects of these two different training methods on left ventricular (LV) systolic and diastolic parameters and left atrial mechanical function is an important step toward understanding the effects of different types of exercise on cardiac function. This knowledge has significant implications for public health, as it can inform the development of targeted and effective exercise programs that prioritize cardiovascular health and reduce the risk of adverse outcomes. Therefore, the primary aim of this study is to comprehensively investigate the LV systolic and diastolic parameters of athletes who engage in RE and CE using ECHO, to contribute to the growing body of literature on the cardiovascular effects of different types of exercise. Forty-two amateur athletes aged between 17 and 52 were included in our study. The participants consisted of the RE (n = 26) group who did only resistance exercise during the weekly exercise period, and the CE group (n = 16) who also did cardio exercise with resistance exercises. After determining sports age (year), weekly exercise frequency (day), and training volume (min) in addition to demographic information of RE and CE groups, left ventricular systolic and diastolic parameters and left atrial functions were determined by ECHO. Findings from our study revealed that parameters including the left ventricular end-diastolic diameter (LVEDD) (p = .008), left ventricular end-diastolic volume (LVEDV) (p = .020), stroke volume index (SV-I) (p = .048), conduit volume (CV-I) (p = .001), and aortic strain (AS) (p = .017) were notably higher in the RE group compared to the CE group. Also left atrial active emptying volüme (LAAEV) of CE was higher than the RE group (p = .031). In conclusion, the cardiac parameters of the RE group showed more athlete's heart characteristics than the CE group. These results may help to optimize the cardiovascular benefits of exercise routines while minimizing the potential risks associated with improper training.
Topics: Humans; Ventricular Function, Left; Resistance Training; Atrial Function, Left; Male; Athletes; Adult; Young Adult; Adolescent; Diastole; Systole; Female; Middle Aged
PubMed: 38705994
DOI: 10.1186/s12872-024-03908-w -
The American Journal of Cardiology Jul 2024Transcatheter aortic valve replacement (TAVR) has emerged as an alternative treatment for patients with pure severe aortic regurgitation (PSAR) who are contraindicated...
Transcatheter aortic valve replacement (TAVR) has emerged as an alternative treatment for patients with pure severe aortic regurgitation (PSAR) who are contraindicated for surgery or have a high surgical risk. However, the therapeutic efficacy and safety of TAVR in low Society of Thoracic Surgeons (STS) score risk patients remain to be clarified. This study aimed to explore the feasibility of TAVR treatment in different STS-risk patients and to compare the adverse events between the groups. In this study, patients with PSAR who underwent TAVR at Zhongshan Hospital, Fudan University, China, during the inclusion period were included and categorized into 3 groups based on STS scores. The baseline data, imaging results, and follow-up data of the patients were documented. Therefore, of 75 TAVR patients, 38 (50.7%) were categorized as low risk (STS <4), and 37 (49.3%) patients were categorized as intermediate and high risk (STS ≥4). Compared with patients at intermediate and high risk, those in the low-risk group were younger, had a lower body mass index, had a lower prevalence of hypertension, chronic obstructive pulmonary disease, and previous percutaneous coronary intervention, and had better cardiac function (p all <0.05). In the hospital and at the 1-month follow-up, the degree of aortic regurgitation and cardiac function were significantly improved. No significant difference was found between the 2 groups in the hospital or during the 30-day follow-up. In conclusion, TAVR for PSAR in low-STS-risk patients is safe and efficient during 30 days of follow-up compared with intermediate- and high-STS-risk groups. TAVR for PSAR should not be limited to inoperable or STS-defined high-risk patients. Long-term follow-up is needed for further investigation.
Topics: Humans; Transcatheter Aortic Valve Replacement; Aortic Valve Insufficiency; Male; Female; Aged; Treatment Outcome; Severity of Illness Index; Risk Assessment; Retrospective Studies; China; Risk Factors; Follow-Up Studies; Aged, 80 and over; Time Factors
PubMed: 38703883
DOI: 10.1016/j.amjcard.2024.04.056 -
World Journal of Cardiology Apr 2024Pacemaker post-transcatheter aortic valve replacement is related to multifactorial risk. Nwaedozie brought to the body of evidence electrocardiogram and clinical...
Pacemaker post-transcatheter aortic valve replacement is related to multifactorial risk. Nwaedozie brought to the body of evidence electrocardiogram and clinical findings. However, procedural characteristics have at least as much impact on the final need for a permanent pacemaker and potentially on the pacing rate. In this regard, long-term follow-up and understanding of the impact of long-term stimulation is of utmost importance.
PubMed: 38690219
DOI: 10.4330/wjc.v16.i4.168 -
Orphanet Journal of Rare Diseases Apr 2024This cross-sectional controlled study aims to assess health-related quality of life (HRQoL) of children and adolescents with a molecular diagnosis of Marfan syndrome...
BACKGROUND
This cross-sectional controlled study aims to assess health-related quality of life (HRQoL) of children and adolescents with a molecular diagnosis of Marfan syndrome (MFS) or related disorders and to evaluate the factors associated with HRQoL in this population. Sixty-three children with MFS and 124 age- and sex-matched healthy children were recruited. HRQoL was assessed using the Pediatric Quality of Life Inventory (PedsQL™) generic questionnaire. The correlation between HRQoL scores and the different continuous parameters (age, body mass index, disease severity, systemic score, aortic sinus diameter, and aerobic physical capacity) was evaluated using Pearson's or Spearman's coefficient. A multiple linear regression analysis was performed on the two health summary self-reported PedsQL™ scores (physical and psychosocial) to identify the factors associated with HRQoL in the MFS group.
RESULTS
Except for emotional functioning, all other domains of HRQoL (psychosocial and physical health, social and school functions) were significantly lower in children with MFS compared to matched healthy children. In the MFS group, the physical health summary score was significantly lower in female than in male patients (self-report: absolute difference [95%CI] = -8.7 [-17.0; -0.47], P = 0.04; proxy-report: absolute difference [95%CI] = -8.6 [-17.3; 0.02], P = 0.05) and also negatively correlated with the systemic score (self-report: R = -0.24, P = 0.06; proxy-report: R = -0.29, P = 0.03) and with the height Z-score (proxy-report: R = -0.29, P = 0.03). There was no significant difference in the physical health summary scores between the different genetic subgroups. In the subgroup of 27 patients who performed a cardiopulmonary exercise test, self- and proxy-reported physical health summary scores were highly correlated with their aerobic physical capacity assessed by peak oxygen consumption (VOmax) and ventilatory anaerobic threshold (VAT). In the multivariate analysis, the most important independent predictors of decreased physical health were increased height, decreased body mass index, decreased VAT and use of prophylactic therapy.
CONCLUSIONS
This study reports an impaired HRQoL in children and adolescents with MFS or related conditions, in comparison with matched healthy children. Educational and rehabilitation programs must be developed and evaluated to improve exercise capacity and HRQoL in these patients.
TRIAL REGISTRATION
ClinicalTrials.gov, NCT03236571 . Registered 28 July 2017.
Topics: Humans; Quality of Life; Marfan Syndrome; Male; Female; Cross-Sectional Studies; Child; Adolescent; Surveys and Questionnaires
PubMed: 38685042
DOI: 10.1186/s13023-024-03191-0 -
Radiology Case Reports Jul 2024Vertebral osteomyelitis is a well-documented disease entity in literature with various known etiologies. However, vertebral diskitis-osteomyelitis secondary to an...
Vertebral osteomyelitis is a well-documented disease entity in literature with various known etiologies. However, vertebral diskitis-osteomyelitis secondary to an infected aortic aneurysm is an uncommon and life-threatening complication. We present the case of a 65-year-old male patient who presented with chronic low back pain that acutely worsened for 1 to 1.5 months and was diagnosed with vertebral diskitis-osteomyelitis secondary to a contiguous infection from an adjacent mycotic aortic aneurysm. To our knowledge, this is one of the few cases reported of vertebral diskitis-osteomyelitis secondary to mycotic aortic aneurysm. We discuss the findings on CT and MRI, as well as the value of imaging in guiding management.
PubMed: 38680736
DOI: 10.1016/j.radcr.2024.02.039 -
Surgical Case Reports Apr 2024Kommerell diverticulum (KD) with right aortic arch and aberrant left subclavian artery (ALSCA) is a rare congenital aortic anomaly. To improve organ compression symptoms...
BACKGROUND
Kommerell diverticulum (KD) with right aortic arch and aberrant left subclavian artery (ALSCA) is a rare congenital aortic anomaly. To improve organ compression symptoms and avoid rupture of aneurysms in adulthood (19 years old-), surgical treatment is considered the only curative option. However, in childhood (-18 years old), several problems regarding approach and technique selection have been reported. Surgical treatment for KD in infancy (birth-2 years old) has been reported recently, but rarely in adolescence (13-19 years old). We herein report a case of KD in which the patient underwent graft replacement during adolescence.
CASE PRESENTATION
A 13-year-old boy was admitted to our hospital presenting with dysphagia and body weight loss. Esophagography showed upper esophageal stenosis caused by extrinsic compression. Contrast-enhanced computer tomography showed saccular aneurysm formation of KD with right aortic arch (RAA) and ALSCA. Elective surgery including KD resection and graft replacement of the descending aorta was performed via right thoracotomy under partial extracorporeal circulation. The ALSCA was reconstructed by graft interposition. No postoperative complication was observed. Follow-up esophagography showed no residual stenosis.
CONCLUSION
We experienced a case of KD with dysphagia and weight loss in adolescence, which was successfully treated with surgery. Graft replacement could be an effective treatment option, facilitating recovery even during the growth period.
PubMed: 38678483
DOI: 10.1186/s40792-024-01904-y