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1.
Echocardiography ; 41(6): e15851, 2024 Jun.
Article En | MEDLINE | ID: mdl-38837500

Loeys-Dietz syndrome (LDS) is an autosomal-dominant connective tissue disorder associated with mutations in the transforming growth factor ß receptor. It is characterized by distinctive craniofacial changes, skeletal features, and cardiovascular complications. We present a case of a 24-year-old male with development delay and a one-year history of progressively worsening dyspnea on moderate exertion and orthopnea. Echocardiography revealed right atrial and right ventricle dilation, right ventricle hypertrophy, atrial septal defect, and aneurysmal dilation of the pulmonary artery trunk. This case underscores the importance of early detection and comprehensive imaging in patients suspected of having LDS, particularly considering the potential for atypical vascular manifestations.


Delayed Diagnosis , Echocardiography , Heart Septal Defects, Atrial , Loeys-Dietz Syndrome , Pulmonary Artery , Humans , Male , Loeys-Dietz Syndrome/complications , Loeys-Dietz Syndrome/diagnosis , Heart Septal Defects, Atrial/complications , Heart Septal Defects, Atrial/diagnosis , Pulmonary Artery/diagnostic imaging , Pulmonary Artery/abnormalities , Young Adult , Echocardiography/methods , Dilatation, Pathologic , Diagnosis, Differential
2.
Medicine (Baltimore) ; 103(23): e38413, 2024 Jun 07.
Article En | MEDLINE | ID: mdl-38847735

To evaluate the cardiac index and major adverse cardiovascular events (MACE) events between isolated coronary artery ectasia (CAE) and control groups over 1 year period from diagnosis. A total of 18 patients who were diagnosed with isolated CAE in the Second Hospital of Hebei Medical University from December 2020 to December 2021 were included in CAE group. About 36 patients with non-obstructive coronary artery lesions were included in the control group. All patients in 2 groups completed dobutamine stress echocardiography (DSE) during hospitalization. The chamber size, wall thickness, left ventricular ejection fraction, and left ventricular diastolic function indicators (including E/A ratio, e', and E/e' ratio) were measured. MACE and all-cause death were measured during follow-up after discharge. Interventricular septum thickness (IVSd), left ventricular posterior wall (LVPW) thickness in diastole and E/e' in CAE group were significantly higher than control group (P < .05). No significant differences were found in prognosis including angina, myocardial ischemia (MI), patient readmission and cardiovascular death (P > .05). In CAE group, coronary angiography showed dilation of left anterior descending (LAD) in 1 case, left circumflex (LCX) in 3 cases and right coronary artery (RCA) in 14 cases. Multivariate logistic regression analysis showed that BMI and IVSd were independent risk factors for CAE. IVSd, LVPW thickness in diastole and E/e' in CAE group were significantly higher than control group. BMI and IVSd were independent risk factors for isolated CAE, and had a good predictive value for isolated CAE.


Coronary Artery Disease , Humans , Female , Male , Middle Aged , Risk Factors , Dilatation, Pathologic/diagnostic imaging , Aged , Coronary Artery Disease/complications , Coronary Artery Disease/diagnostic imaging , Coronary Artery Disease/epidemiology , Coronary Vessels/diagnostic imaging , Coronary Vessels/pathology , Coronary Angiography/methods , Prognosis , Echocardiography, Stress
3.
BMC Ophthalmol ; 24(1): 246, 2024 Jun 11.
Article En | MEDLINE | ID: mdl-38862935

BACKGROUND: To compare the ocular features of highly myopic eyes with posterior staphyloma of wide macular type according to its morphological complexity. METHODS: In this cross-sectional study, wide macular posterior staphyloma (WMPS) was classified into the primary (Curtin type I) and the compound (Curtin types VI to X) forms based on the configuration within the staphyloma. The grades of myopic maculopathy and the thicknesses of choroid and sclera were compared between the primary and compound forms of WMPS. RESULTS: A total of 154 eyes (103 patients) with primary WMPS and 65 eyes (49 patients) with compound WMPS were included. Eyes with compound WMPS had worse visual acuity (P = 0.001) and greater axial length (P < 0.001) than those with primary WMPS. Compared to primary WMPS, compound WMPS had a higher grade of myopic macular degeneration (P < 0.001) and a higher frequency of lamellar or full-thickness macular hole associated with myopic traction (21.5% vs. 10.4%; P = 0.028) and active or scarred myopic choroidal neovascularization (33.8% vs. 20.1%; P = 0.030). On swept-source optical coherence tomography, eyes with compound WMPS had significantly thinner choroid and sclera. CONCLUSIONS: The compound form of WMPS had more severe myopic macular changes and worse visual prognosis compared to the primary form of WMPS, and these were associated with more structural deformation in the posterior eyeball. Compound WMPS should be considered as an advanced form of staphyloma.


Myopia, Degenerative , Sclera , Tomography, Optical Coherence , Visual Acuity , Humans , Female , Male , Cross-Sectional Studies , Myopia, Degenerative/complications , Myopia, Degenerative/diagnosis , Middle Aged , Visual Acuity/physiology , Tomography, Optical Coherence/methods , Aged , Sclera/pathology , Retrospective Studies , Adult , Choroid/pathology , Choroid/diagnostic imaging , Scleral Diseases/diagnosis , Macula Lutea/pathology , Macula Lutea/diagnostic imaging , Dilatation, Pathologic
4.
BMC Gastroenterol ; 24(1): 201, 2024 Jun 17.
Article En | MEDLINE | ID: mdl-38886637

BACKGROUND: Dilatation of common bile duct (CBD) is mostly pathological and mainly occurs secondary to mechanical causes. We aimed to explore the prevalence of CBD dilatation in Intraductal Papillary Mucinous Neoplasms of the pancreas (IPMN) among patients referred to EUS. METHODS: A retrospective study of all patients who had an EUS diagnosis of IPMN from 2011 to 2019 at Galilee Medical Center were extracted. Control group including patients with other types of pancreatic cysts. RESULTS: Overall, 2400 patients were included in the study, of them 158 patients were diagnosed with pancreatic cysts, 117 patients (74%) diagnosed with IPMN (group A), and 41 patients (26%) diagnosed with other pancreatic cysts (group B). Univariate analysis showed significant association of IPMN (OR 3.8, 95% CI 1.3-11.5), resected gallbladder (GB) (OR 7.75, 95% CI 3.19-18.84), and age (OR 1, 95% CI 1.01-1.08) with CBD dilatation. Classifying IPMN to sub-groups using adjusted multivariate logistic regression analysis, only main duct-IPMN (MD-IPMN) significantly correlated with CBD dilatation compared to branch duct (BD)-IPMN and mixed type-IPMN (OR 19.6, 95% CI 4.57-83.33, OR 16.3, 95% CI 3.02-88.08). CONCLUSION: MD-IPMN was significantly correlated with dilated CBD. Assessment of the pancreas is warranted in encountered cases of dilated CBD without obvious mechanical cause.


Common Bile Duct , Humans , Male , Female , Retrospective Studies , Middle Aged , Dilatation, Pathologic/diagnostic imaging , Aged , Common Bile Duct/diagnostic imaging , Common Bile Duct/pathology , Pancreatic Intraductal Neoplasms/pathology , Pancreatic Intraductal Neoplasms/diagnostic imaging , Endosonography , Pancreatic Cyst/pathology , Pancreatic Cyst/diagnostic imaging , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/diagnostic imaging , Carcinoma, Pancreatic Ductal/pathology , Carcinoma, Pancreatic Ductal/diagnostic imaging , Adenocarcinoma, Mucinous/pathology , Adenocarcinoma, Mucinous/diagnostic imaging
5.
BMC Pediatr ; 24(1): 317, 2024 May 08.
Article En | MEDLINE | ID: mdl-38720245

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/m2, 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.


Turner Syndrome , Humans , Turner Syndrome/complications , Child , Adolescent , Female , China/epidemiology , Dilatation, Pathologic/etiology , Male , Retrospective Studies , Aorta/pathology , Aorta/diagnostic imaging , Aortic Coarctation , Bicuspid Aortic Valve Disease/complications , Child, Preschool , Incidence , East Asian People
6.
Curr Opin Ophthalmol ; 35(4): 273-277, 2024 Jul 01.
Article En | MEDLINE | ID: mdl-38700496

PURPOSE OF REVIEW: To review corneal crosslinking for keratoconus and corneal ectasia, and recent developments in the field. This study will review the mechanism of crosslinking, clinical approaches, current results, and potential future innovations. RECENT FINDINGS: Corneal crosslinking for keratoconus was first approved by U.S. FDA in 2016. Recent studies have confirmed the general long-term efficacy of the procedure in decreasing progression of keratoconus and corneal ectasia. New types of crosslinking protocols, such as transepithelial treatments, are under investigation. In addition, adjunctive procedures have been developed to improve corneal contour and visual function in these patients. SUMMARY: Crosslinking has been found to be well tolerated and effective with the goal of decreasing progression of ectatic corneal diseases, keratoconus and corneal ectasia after refractive surgery. Studies have shown its long-term efficacy. New techniques of crosslinking and adjunctive procedures may further improve treatments and results.


Collagen , Cross-Linking Reagents , Keratoconus , Photochemotherapy , Photosensitizing Agents , Riboflavin , Ultraviolet Rays , Keratoconus/drug therapy , Humans , Cross-Linking Reagents/therapeutic use , Riboflavin/therapeutic use , Photosensitizing Agents/therapeutic use , Photochemotherapy/methods , Dilatation, Pathologic/drug therapy , Collagen/metabolism , Corneal Stroma/metabolism
7.
Circulation ; 149(25): 1938-1948, 2024 Jun 18.
Article En | MEDLINE | ID: mdl-38804148

BACKGROUND: Ascending aorta dilation and aortic valve degeneration are common complications in patients with bicuspid aortic valve. Several retrospective studies have suggested the benefit of statins in reducing these complications. This study aimed to determine whether atorvastatin treatment is effective in reducing the growth of aortic diameters in bicuspid aortic valve and if it slows the progression of valve calcification. METHODS: In a randomized clinical trial, 220 patients with bicuspid aortic valve (43 women; 46±13 years of age) were included and treated with either 20 mg of atorvastatin per day or placebo for 3 years. Inclusion criteria were ≥18 years of age, nonsevere valvular dysfunction, nonsevere valve calcification, and ascending aorta diameter ≤50 mm. Computed tomography and echocardiography studies were performed at baseline and after 3 years of treatment. RESULTS: During follow-up, 28 patients (12.7%) discontinued medical treatment (15 on atorvastatin and 13 taking placebo). Thus, 192 patients completed the 36 months of treatment. Low-density lipoprotein cholesterol levels decreased significantly in the atorvastatin group (median [interquartile range], -30 mg/dL [-51.65 to -1.75 mg/dL] versus 6 mg/dL [-4, 22.5 mg/dL]; P<0.001). The maximum ascending aorta diameter increased with no differences between groups: 0.65 mm (95% CI, 0.45-0.85) in the atorvastatin group and 0.74 mm (95% CI, 0.45-1.04) in the placebo group (P=0.613). Similarly, no significant differences were found for the progression of the aortic valve calcium score (P=0.167) or valvular dysfunction. CONCLUSIONS: Among patients with bicuspid aortic valve without severe valvular dysfunction, atorvastatin treatment was not effective in reducing the progression of ascending aorta dilation and aortic valve calcification during 3 years of treatment despite a significant reduction in low-density lipoprotein cholesterol levels. REGISTRATION: URL: https://www.clinicaltrialsregister.eu; Unique identifier: 2015-001808-57. URL: https://www.clinicaltrials.gov; Unique identifier: NCT02679261.


Aortic Valve , Atorvastatin , Bicuspid Aortic Valve Disease , Calcinosis , Disease Progression , Heart Valve Diseases , Hydroxymethylglutaryl-CoA Reductase Inhibitors , Humans , Atorvastatin/therapeutic use , Female , Male , Middle Aged , Aortic Valve/diagnostic imaging , Aortic Valve/pathology , Aortic Valve/abnormalities , Aortic Valve/drug effects , Calcinosis/drug therapy , Calcinosis/diagnostic imaging , Calcinosis/pathology , Bicuspid Aortic Valve Disease/diagnostic imaging , Bicuspid Aortic Valve Disease/drug therapy , Heart Valve Diseases/drug therapy , Heart Valve Diseases/diagnostic imaging , Heart Valve Diseases/pathology , Adult , Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use , Dilatation, Pathologic/drug therapy , Follow-Up Studies , Double-Blind Method , Treatment Outcome , Aorta/diagnostic imaging , Aorta/pathology , Aorta/drug effects , Aortic Valve Disease/drug therapy , Aortic Valve Stenosis
8.
World J Gastroenterol ; 30(10): 1420-1430, 2024 Mar 14.
Article En | MEDLINE | ID: mdl-38596496

BACKGROUND: Various animal models have been used to explore the pathogenesis of choledochal cysts (CCs), but with little convincing results. Current surgical techniques can achieve satisfactory outcomes for treatment of CCs. Consequently, recent studies have focused more on clinical issues rather than basic research. Therefore, we need appropriate animal models to further basic research. AIM: To establish an appropriate animal model that may contribute to the investigation of the pathogenesis of CCs. METHODS: Eighty-four specific pathogen-free female Sprague-Dawley rats were randomly allocated to a surgical group, sham surgical group, or control group. A rat model of CC was established by partial ligation of the bile duct. The reliability of the model was confirmed by measurements of serum biochemical indices, morphology of common bile ducts of the rats as well as molecular biology experiments in rat and human tissues. RESULTS: Dilation classified as mild (diameter, ≥ 1 mm to < 3 mm), moderate (≥ 3 mm to < 10 mm), and severe (≥ 10 mm) was observed in 17, 17, and 2 rats in the surgical group, respectively, while no dilation was observed in the control and sham surgical groups. Serum levels of alanine aminotransferase, aspartate aminotransferase, total bilirubin, direct bilirubin, and total bile acids were significantly elevated in the surgical group as compared to the control group 7 d after surgery, while direct bilirubin, total bilirubin, and gamma-glutamyltransferase were further increased 14 d after surgery. Most of the biochemical indices gradually decreased to normal ranges 28 d after surgery. The protein expression trend of signal transducer and activator of transcription 3 in rat model was consistent with the human CC tissues. CONCLUSION: The model of partial ligation of the bile duct of juvenile rats could morphologically simulate the cystic or fusiform CC, which may contribute to investigating the pathogenesis of CC.


Choledochal Cyst , Humans , Female , Rats , Animals , Choledochal Cyst/surgery , Reproducibility of Results , Rats, Sprague-Dawley , Models, Animal , Dilatation, Pathologic , Bilirubin , Disease Models, Animal
10.
Gene ; 916: 148450, 2024 Jul 20.
Article En | MEDLINE | ID: mdl-38588932

BACKGROUND: Although the implication of receptor of advanced glycation endproducts (RAGE) has been reported in coronary artery disease, its roles in coronary artery ectasia (CAE) have remained undetermined. Furthermore, the effect of RAGE polymorfisms were not well-defined in scope of soluble RAGE (sRAGE) levels. Thus, we aimed to investigate the influence of the functional polymorphisms of RAGE -374T > A (rs1800624) and G82S (rs2070600) in CAE development. METHODS: This prospective observational study was conducted in 2 groups selected of 2452 patients who underwent elective coronary angiography (CAG) for evaluation after positive noninvasive heart tests. Group-I included 98 patients with non-obstructive coronary artery disease and CAE, and Group-II (control) included 100 patients with normal coronary arteries. SNPs were genotyped by real-time PCR using Taqman® genotyping assay. Serum sRAGE and soluble lectin-like oxidized receptor-1 (sOLR1) were assayed by ELISA and serum lipids were measured enzymatically. RESULTS: The frequencies of the RAGE -374A allele and -374AA genotype were significantly higher in CAE patients compared to controls (p < 0.001). sRAGE levels were not different between study groups, while sOLR1 levels were elevated in CAE (p = 0.004). In controls without systemic disease, -374A allele was associated with low sRAGE levels (p < 0.05), but this association was not significant in controls with HT. Similarly, sRAGE levels of CAE patients with both HT and T2DM were higher than those no systemic disease (p = 0.02). The -374A allele was also associated with younger patient age and higher platelet count in the CAE group in both total and subgroup analyses. In the correlation analyses, the -374A allele was also negatively correlated with age and positively correlated with Plt in all of these CAE groups. In the total CAE group, sRAGE levels also showed a positive correlation with age and a negative correlation with HDL-cholesterol levels. On the other hand, a negative correlation was observed between sRAGE and Plt in the total, hypertensive and no systemic disease control subgroups. Multivariate logistic regression analysis confirmed that the -374A allele (p < 0.001), hyperlipidemia (p < 0.05), and high sOLR1 level (p < 0.05) are risk factors for CAE. ROC curve analysis shows that RAGE -374A allele has AUC of 0.713 (sensitivity: 83.7 %, specificity: 59.0 %), which is higher than HLD (sensitivity: 59.2 %, specificity: 69.0 %), HT (sensitivity: 62.4 %, specificity: 61.1 %) and high sOLR1 level (≥0.67 ng/ml)) (sensitivity: 59.8 %, specificity: 58.5 %). CONCLUSION: Beside the demonstration of the relationship between -374A allele and increased risk of CAE for the first time, our results indicate that antihypertensive and antidiabetic treatment in CAE patients causes an increase in sRAGE levels. The lack of an association between the expected -374A allele and low sRAGE levels in total CAE group was attributed to the high proportion of hypertensive patients and hence to antihypertensive treatment. Moreover, the RAGE -374A allele is associated with younger age at CAE and higher Plt, suggesting that -374A may also be associated with platelet activation, which plays a role in the pathogenesis of CAE. However, our data need to be confirmed in a large study for definitive conclusions.


Coronary Artery Disease , Polymorphism, Single Nucleotide , Receptor for Advanced Glycation End Products , Humans , Female , Male , Middle Aged , Receptor for Advanced Glycation End Products/genetics , Receptor for Advanced Glycation End Products/blood , Coronary Artery Disease/genetics , Coronary Artery Disease/blood , Prospective Studies , Aged , Dilatation, Pathologic/genetics , Genetic Predisposition to Disease , Scavenger Receptors, Class E/genetics , Coronary Vessels/metabolism , Coronary Vessels/pathology , Case-Control Studies , Alleles , Coronary Angiography , Gene Frequency , Genotype , LDL-Receptor Related Proteins , Membrane Transport Proteins
13.
J Cardiothorac Surg ; 19(1): 175, 2024 Apr 04.
Article En | MEDLINE | ID: mdl-38575998

BACKGROUND: The persistent left superior vena cava (PLSVC) is an infrequent vascular variant. PLSVC with absent right superior vena cava, also known as isolated PLSVC, is an exceptionally rare entity. In this case we present a patient with isolated PLSVC draining to coronary sinus, diagnosed incidentally during echocardiography. CASE PRESENTATION: A 35-year-old man underwent a transthoracic echocardiography which showed an enormously dilated coronary sinus. Hand-agitated saline was injected via peripheral intravenous cannulas. The contrast appeared firstly in the coronary sinus before it opacified the right atrium. Since this was also visible by the right antecubital saline injection, it indicated an extremely rare case of PLSVC with the absence of right superior vena cava which was confirmed by cardiac magnetic resonance. CONCLUSIONS: The finding of a distinctively dilated coronary sinus in echocardiography led us to further investigation using agitated saline that revealed an infrequent anomaly termed isolated PLSVC. The in-depth diagnosis of this vascular variant is crucial considering that it may lead to important clinical implications, such as difficulties with central venous access, especially in the current era of a rapid development of cardiac device therapies.


Coronary Sinus , Persistent Left Superior Vena Cava , Vascular Malformations , Male , Humans , Adult , Vena Cava, Superior/diagnostic imaging , Vena Cava, Superior/abnormalities , Echocardiography , Vascular Malformations/diagnostic imaging , Coronary Sinus/diagnostic imaging , Dilatation, Pathologic
15.
World J Surg ; 48(2): 446-455, 2024 02.
Article En | MEDLINE | ID: mdl-38686786

BACKGROUND: The diseased bile duct in bilobar congenital biliary dilatation is extensive and often requires major hepatectomy or liver transplantation associated with a higher risk. We aimed to evaluate the safety and benefit of modified mesohepatectomy, in comparison with trisectionectomy, to treat bilobar congenital biliary dilatation. METHODS: This study included 28 patients with type IV and V bilobar congenital biliary dilatation. An innovative mesohepatectomy comprising the hepatectomy technique beyond the P/U point and bile duct shaping was applied to 14 patients to address the extensively diseased bile duct and difficulty in hepaticojejunostomy. Another 14 patients received trisectionectomy. The perioperative and long-term outcomes of these patients were compared. RESULTS: The ratio of residual liver volume to standard liver volume in the mesohepatectomy group was higher (78.68% vs. 40.90%, p = 0.005), while the resection rate of the liver parenchyma was lower (28.25% vs. 63.97%, p = 0.000), than that in trisectionectomy group. The mesohepatectomy group had a lower severe complication (>Clavein III, 0% vs. 57.70%, p = 0.019) and incidence of posthepatectomy liver failure (7.14% vs. 42.86%, p = 0.038). No significant difference was observed in blood loss and bile leakage (p > 0.05). All the patients in the mesohepatectomy group achieved optimal results in the long-term follow-up. CONCLUSIONS: mesohepatectomy provides an efficient treatment option for bilobar congenital biliary dilatation and can achieve radical resection, retain more liver parenchyma, and reduce the difficulty of hepaticojejunostomy, especially for patients that are not eligible for major hepatectomy and liver transplantation.


Hepatectomy , Humans , Hepatectomy/methods , Male , Female , Treatment Outcome , Retrospective Studies , Dilatation, Pathologic/surgery , Infant , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Child, Preschool
16.
Article En | MEDLINE | ID: mdl-38522872

The Ross procedure is an excellent option for aortic valve replacement resulting in outstanding hemodynamic performance and the ability to avoid systemic anticoagulation. The long-term durability of the autograft is generally good but concerns for later aortic root dilation with ensuing neoaortic insufficiency have prompted efforts to stabilize the autograft, root, sinuses and Sino-tubular junction in order to delay or entirely avoid late reinterventions on the neoaortic root. We have employed an inclusion technique, supporting the Auto-graft in a Terumo Gelweave™ Valsalva graft. We performed a retrospective study of all 129 patients undergoing the Ross procedure from 1992 to 2019 at Children's Wisconsin. Fifty-one underwent the supported Ross (SR) and 78 underwent unsupported Ross (UR). Structured clinical data was collected and echocardiograms were reviewed. Median follow-up was 4.9 years (up to 22.6 years) for UR patients and 3.6 years (up to 11.4 years) for SR patients. In order to provide a fair comparison, we sub -analyzed patients aged 10 to 18 years who underwent the Ross procedure, 16 who underwent the UR and 18 patients who underwent the SR. Change in aortic annulus diameter (P = 0.002), aortic sinus diameter (P = 0.001) change in left ventricular function (P = 0.039) and change in aortic insufficiency (P = 0.008) were all worse in UR. The SR is simple, reproducible, and predictable. It seems to prevent change in annulus diameter, sinus diameter and to reduce late neoaortic insufficiency. Longer follow-up with a larger group of patients is required to draw definitive conclusions.


Aortic Valve Insufficiency , Aortic Valve Stenosis , Pulmonary Valve , Child , Humans , Autografts , Retrospective Studies , Dilatation , Transplantation, Autologous , Aortic Valve/surgery , Aortic Valve Insufficiency/surgery , Aortic Valve Stenosis/surgery , Dilatation, Pathologic , Pulmonary Valve/surgery , Follow-Up Studies
17.
Childs Nerv Syst ; 40(7): 2061-2069, 2024 Jul.
Article En | MEDLINE | ID: mdl-38532147

PURPOSE: Post-hemorrhagic ventricular dilation (PHVD) leads to developmental delays in premature infants, yet the optimal timing of neurosurgical interventions is unknown. Neuroimaging modalities have emerged to delineate injury and follow the progression of PHVD. Fronto-temporal horn ratio (FTHR) is used as a marker of ventricular dilation and can be a standardized tool to direct the timing of neurosurgical intervention. Our study determined a pre-operative FTHR measurement threshold to predict short- and long-term outcomes. METHODS: This is a retrospective cohort study of premature infants with severe intraventricular hemorrhage (IVH) who developed PHVD requiring neurosurgical intervention and were treated in a level IV NICU between 2012 and 2019. Receiver operating characteristic (ROC) curve and area under the curve (AUC) analyses were performed to evaluate the accuracy of pre-operative FTHR for predicting developmental delay. In-hospital outcomes and developmental assessments were analyzed. RESULTS: We reviewed 121 charts of infants with IVH and identified 43 infants with PHVD who required neurosurgical intervention. We found FTHR measurements were an excellent predictor of cognitive and motor delay with an AUC of 0.89 and 0.88, respectively. An average pre-operative FTHR of ≥ 0.67 was also associated with worse lung and feeding outcomes. There was excellent inter-observer reliability of individual components of FTHR measurements. CONCLUSIONS: Early intervention for PHVD is ideal but not always practical. Identification of ventricular size thresholds associated with better outcomes is needed to direct timing of neurosurgical intervention.


Cerebral Ventricles , Humans , Male , Female , Retrospective Studies , Cerebral Ventricles/diagnostic imaging , Cerebral Ventricles/surgery , Infant, Newborn , Infant , Infant, Premature , Dilatation, Pathologic/diagnostic imaging , Dilatation, Pathologic/surgery , Developmental Disabilities/etiology , Developmental Disabilities/diagnostic imaging , Cerebral Hemorrhage/surgery , Cerebral Hemorrhage/diagnostic imaging , Cerebral Hemorrhage/complications , Cohort Studies , Treatment Outcome , Neurosurgical Procedures/methods
18.
J Vasc Surg ; 80(1): 107-113, 2024 Jul.
Article En | MEDLINE | ID: mdl-38485071

OBJECTIVE: Though initially protected from vessel dilation by estrogen, women may experience rapid abdominal aortic aneurysm (AAA) growth post-menopause. The rate of growth has been poorly defined in prior literature. Here, we describe aneurysm growth in a cohort of women found through an AAA screening program. METHODS: Women with AAAs were retrospectively identified. Aortic imaging was reviewed, and measurements of maximum transverse and anterior-posterior diameters were completed. Growth was stratified by the type of aortic pathology (fusiform aneurysm, aortic ectasia, dissection with aneurysmal degeneration, saccular aneurysm) as well as size category (<3 cm, 3.0-3.9 cm, 4.0-4.9 cm, ≥5.0 cm) at diagnosis. RESULTS: A cohort of 488 women was identified; 286 had multiple scans for review. The mean age of the entire cohort was 75 ± 9.9 years. Stratified by type of pathology, the mean age was 76 ± 8.9 years in patients with a fusiform AAA, 74 ± 9.8 years in ectasia, 65 ± 13.7 years in dissection, and 76 ± 5.6 years in saccular aneurysms. The maximum growth was highest in women with fusiform AAAs, followed by dissection, ectasia, and saccular pathology (9.7 mm, 7.0 mm, 3.0 mm, and 2.2 mm, respectively; P < .001). Comparing mean growth by year, the highest mean growth was in fusiform AAAs (3.6 mm vs 1.75 mm in dissection; P < .001). The Shapiro-Wilk test demonstrated that mean growth per year was non-normally distributed with a right skew. Stratified by aortic diameter at the time of diagnosis, mean growth/year increased with increasing size at diagnosis in fusiform AAAs and dissection (0.91 mm for <3 cm, 2.34 mm for 3.0-3.9 cm, 2.49 mm for 4.0-4.9 mm, and 6.16 mm for ≥5.0 cm in patients with fusiform AAAs vs 0.57 mm, 0.94 mm, 1.87 mm, and 2.66 mm, respectively, for patients with dissection). Smoking history was associated with a higher mean growth/year (2.6 mm vs 3.3 mm; P < .001). Conversely, patients with a family history of AAA had a lower mean growth/year (3.2 mm vs 1.5 mm; P < .001). CONCLUSIONS: The rate of aneurysm growth in women varies based on pathology and aneurysm size, and women experience rapid aneurysm growth at sizes greater than 4.5 cm. Current screening guidelines are inadequate, and our results demonstrate that the rate of growth of fusiform aneurysms in women is faster than in men at a smaller size and may warrant more frequent surveillance than current Society for Vascular Surgery recommendations to prevent risk of increased morbidity.


Aortic Aneurysm, Abdominal , Disease Progression , Humans , Female , Aortic Aneurysm, Abdominal/diagnostic imaging , Aortic Aneurysm, Abdominal/epidemiology , Retrospective Studies , Aged , Middle Aged , Aged, 80 and over , Risk Factors , Time Factors , Aorta, Abdominal/diagnostic imaging , Aorta, Abdominal/pathology , Computed Tomography Angiography , Aortography , Sex Factors , Aortic Dissection/diagnostic imaging , Aortic Dissection/epidemiology , Dilatation, Pathologic , Postmenopause
19.
Neurosurg Focus ; 56(3): E15, 2024 03.
Article En | MEDLINE | ID: mdl-38428011

OBJECTIVE: Ethmoidal dural arteriovenous fistulas (DAVFs) are often associated with cortical venous drainage (CVD) and a higher incidence of hemorrhage compared with DAVFs in other locations. They may be treated with open surgical disconnection or with endovascular treatment (EVT). In this systematic review and meta-analysis, the authors compare the outcomes of ethmoidal DAVFs treated with open microsurgery versus EVT and report four additional cases of ethmoidal DAVFs treated with open microsurgery in their institution. METHODS: A literature search of the PubMed and Scopus databases was conducted between December 2021 and May 2022 to identify relevant articles published between 1990 and 2021 using the PRISMA guidelines. References were reviewed and screened by two authors independently, and disagreements were resolved through consensus. Exclusion criteria included non-English-language studies, those with an incorrect study design, those reporting DAVFs in a nonethmoidal location, and studies whose outcomes were not stratified based on DAVF location. Inclusion criteria were any studies reporting on ethmoidal DAVFs treated by either microsurgery or EVT. A risk of bias assessment was performed using the Newcastle-Ottawa Scale. The authors performed a pooled proportional meta-analysis to compare patient outcomes. RESULTS: Twenty studies were included for analysis. Of 224 patients, 142 were treated with surgery, while 103 were treated with EVT. Seventy percent (148/210) of the patients were symptomatic at presentation, with hemorrhage being the most common presentation (48%). CVD was present in 98% of patients and venous ectasia in 61%. The rates of complete DAVF obliteration with surgery and EVT were 89% and 70%, respectively (95% CI -30% to -10%, p < 0.03). Twenty percent (21/103) of endovascularly treated fistulas required subsequent surgery. Procedure-related complications occurred in 10% of the surgical cases, compared with 13% of the EVT cases. The authors' case series included 4 patients with ethmoidal DAVFs treated surgically with complete obliteration, without any postoperative complications. CONCLUSIONS: The complete obliteration rates of ethmoidal DAVF appear to be higher and more definitive with microsurgical intervention than with EVT. While complication rates between the two procedures seem similar, patients treated with EVT may require further interventions for definitive treatment. The limitations of this study include its retrospective nature, the quality of studies included, and the continued evolving technologies of EVT. Future studies should focus on the association between venous drainage pattern and the proclivity toward venous ectasia or rate of hemorrhage at presentation.


Central Nervous System Vascular Malformations , Embolization, Therapeutic , Humans , Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/surgery , Central Nervous System Vascular Malformations/complications , Dilatation, Pathologic/complications , Dilatation, Pathologic/therapy , Embolization, Therapeutic/methods , Hemorrhage , Treatment Outcome , Microsurgery
20.
Int J Cardiol ; 405: 131948, 2024 Jun 15.
Article En | MEDLINE | ID: mdl-38471650

BACKGROUND: We aim to compare the short and long-term outcomes for aortic stenosis (AS) patients undergone TAVR with and without ascending aorta dilation (AAD). METHODS: Consecutive patients diagnosed with native severe AS who underwent TAVR from September 2012 to September 2021 were enrolled. They were stratified into the moderate/severe dilation group (greatest ascending aorta width ≥ 45 mm) and the non/mild dilation group. Survival outcomes were illustrated using Kaplan-Meier curves and evaluated with the log-rank test. Data from patients with CT follow-up of >6 months was used to investigate the progression rate of AAD. RESULTS: The study cohort comprised 556 patients, with a mean age of 75.5 ± 7.3 years. Among them, 107 patients (19.2%) had a moderate/severe AAD (≥45 mm), with an average diameter of 48.6 mm (±2.8). During hospitalization, both groups witnessed two cases of ascending aortic dissection (1.9% vs 0.4%, P = 0.380). The median follow-up duration was 3.9 years (95% CI: 3.8-4.0 years). No deaths were caused by aortic events and no patients experienced a new aortic dissection. The AAD cohort's 4-year all-cause and cardiovascular mortality rates were not significantly different to the non/mild dilation group's (log-rank test, P = 0.109 and P = 0.698, respectively). Follow-up CT data revealed that the rate of aortic dilation progression in the moderate/severe dilation group was not significantly different from that in the non/mild group (0.0 mm/year, 25-75%th: -0.3-0.2 vs 0.1 mm/year, 25-75%th: -0.3-0.4, P = 0.122). CONCLUSION: This study found no significant difference regarding short-term and long-term outcomes in AS patients with/without moderate/severe AAD undergoing TAVR.


Aorta , Aortic Valve Stenosis , Transcatheter Aortic Valve Replacement , Humans , Female , Male , Transcatheter Aortic Valve Replacement/methods , Aged , Aortic Valve Stenosis/surgery , Aortic Valve Stenosis/diagnostic imaging , Aortic Valve Stenosis/mortality , Aged, 80 and over , Treatment Outcome , Aorta/diagnostic imaging , Aorta/surgery , Follow-Up Studies , Cohort Studies , Retrospective Studies , Dilatation, Pathologic
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