Pulmonary valve replacement in tetralogy of Fallot - who and how?

BACKGROUND AND AIM: Pulmonary regurgitation is the most common complication in repaired tetralogy of Fallot patients. Severe chronic pulmonary regurgitation can be tolerated for decades, but if not treated, it can progress to symptomatic, irreversible right ventricular dilatation and dysfunction. We investigated clinical associations with pulmonary valve replacement among patients with significative pulmonary regurgitation and how interventional developments can change their management. METHODS: All adult patients with repaired tetralogy of Fallot who were followed at an adult CHD Clinic at a single centre from 1980 to 2022 were included on their first outpatient visit. Follow-up was estimated from the time of correction surgery until one of the following events occurred first: pulmonary valve replacement, death, loss to follow-up or conclusion of the study. RESULTS: We included 221 patients (116 males) with a median age of 19 (18-25). At a median age of 33 (10) years old, 114 (51%) patients presented significant pulmonary regurgitation. Among patients with significant pulmonary regurgitation, pulmonary valve replacement was associated with male gender, older age at surgical repair, and longer QRS duration in adulthood. Pulmonary valve replacement was performed in 50 patients, including four transcatheter pulmonary valve implantations, at a median age of 34 (14) years. CONCLUSION: Pulmonary regurgitation affects a large percentage of tetralogy of Fallot adult patients, requiring a long-term clinical and imaging follow-up. Sex, age at surgical repair and longer QRS are associated with the need of PVR among patients with significative pulmonary regurgitation. Clinical practice and current literature support TPVI as the future gold standard intervention.

Cardiovascular magnetic resonance in muscular dystrophies: looking ahead.

Cardiac magnetic resonance (CMR) is an established tool for risk stratification in several cardiomyopathies, and its role in muscular dystrophies (MuD) looks promising. We sought to assess how CMR performs in predicting cardiac events in a real cohort of MuD patients. A prospective single-center study with the enrollment of consecutive adult MuD patients referred to cardiac screening from 2012 to 2018 with the collection of clinical and CMR data. During follow-up (FUP), major adverse cardiac events were considered a composite of device implantation, ventricular tachycardia (VT), hospitalization due to heart failure, and death. Sixty-five patients were included (mean age of 32±16, 51% female); the majority had myotonic dystrophy (34; 52.3%); most were asymptomatic (60; 92.3%) and at sinus rhythm (64; 98.5%). CMR was abnormal in 23 (43.3%) patients: left ventricle ejection fraction (LVEF) <55% was found in 7 patients, and late gadolinium enhancement (LGE) was present in 23 patients, mainly intra-myocardial or subepicardial (10 and 8 patients, respectively). During a median FUP of 77 months (interquartile range: 33), there were 7 deaths, 8 implanted devices, and one sustained VT. LVEF<55% and the presence of LGE were associated with the occurrence of all events (log rank test, p=0.002 and p=0.045, respectively). LVEF<55% was associated with a 6-fold higher risk of events (crude hazard ratio of 6.15; 95% confidence interval of 1.65-22.93), that remained significant after adjusting for LGE presence (adjusted hazard ratio of 4.81, 95% confidence interval of 1.07-15.9). In our cohort, CMR LVEF<55% and the presence of LGE were significantly associated with adverse events during follow-up, reinforcing the role of this technique on risk stratification of MuD populations.

Functional characterization of a novel PRRT2 variant found in a Portuguese patient with hemiplegic migraine.

Familial hemiplegic migraine (FHM) is a rare autosomal-dominant form of migraine with aura. Three disease-causing genes have been identified for FHM: CACNA1A, ATP1A2 and SCN1A. However, not all families are linked to one of these three genes.PRRT2 variants were also commonly associated with HM symptoms; therefore, PRRT2 is hypothesized as the fourth gene causing FHM. PRRT2 plays an important role in neuronal migration, spinogenesis, and synapse mechanisms during development and calcium-dependent neurotransmitter release. We performed exome sequencing to unravel the genetic cause of migraine in one family, and a novel PRRT2 variant (c.938C > T;p.Ala313Val) was identified with further functional studies to confirm its pathogenicity. PRRT2-A313V reduced protein stability, led to protein premature degradation by the proteasome and altered the subcellular localization of PRRT2 from the plasma membrane (PM) to the cytoplasm. We identified and characterized for the first time in a Portuguese patient, a novel heterozygous missense variant in PRRT2 associated with HM symptoms. We suggest that PRRT2 should be included in the diagnosis of HM.

Impact of in vitro findings on clinical protocols for the adhesion of CAD-CAM blocks: A systematic integrative review and meta-analysis.

STATEMENT OF PROBLEM: Computer-aided design and computer-aided manufacturing (CAD-CAM) blocks have evolved rapidly, making it difficult to establish the best clinical protocol for bonding a given block and whether an established protocol is appropriate for a newly introduced product. PURPOSE: This integrative systematic review and meta-analysis aimed to clarify whether the clinician can select the most efficient adhesion protocols for CAD-CAM blocks by reading published in vitro studies and implementing them in daily practice. MATERIAL AND METHODS: Based on the population, intervention, comparison, and outcome (PICO) strategy, 3 databases were searched for in vitro studies, randomized clinical trials, prospective or retrospective studies, and case reports from January 1, 2015, to July 31, 2021. A meta-analysis analyzed 28 studies to calculate the mean difference between best and worst protocols for each author and block with a random-effects model (α=.05). RESULTS: From 508 relevant studies, 37 in vitro studies, 2 clinical studies, and 1 clinical report were selected for data extraction and qualitative analysis. Vita Enamic, IPS e.max CAD, LAVA Ultimate, and Vita Mark II blocks were the most studied, and RelyX Ultimate was the most used luting cement. The meta-analysis confirmed the null hypothesis that the evidence-based efficacy of clinical protocols to bond CAD-CAM blocks is still controversial (P<.05). CONCLUSIONS: There are objective standards for individual in vitro tests, but the studies lack standardization. Some tested protocols were more efficient than others. Randomized clinical trials and well-documented clinical situations were almost nonexistent, making direct application of in vitro findings in clinical practice impossible.

Effects of Seed Roasting Temperature on Sesame Oil Fatty Acid Composition, Lignan, Sterol and Tocopherol Contents, Oxidative Stability and Antioxidant Potential for Food Applications.

Roasting is a key step for preparing sesame oil that leads to important changes in its organoleptic properties and quality. In this study, white sesame seeds were roasted for 20 min in an electric oven at different temperatures (120, 150, 180, 210, 250 and 300 °C). The oils extracted from unroasted and roasted seeds were compared for their chemical composition: fatty acids (including trans isomers), phytosterols, lignans (sesamin and sesamolin), tocopherols and total phenolic compounds, as well as their oxidative stability and antiradical capacity. There were no obvious differences in the oil densities, refractive indexes or iodine values, but the saponification values were affected by temperature. Relevant primary and secondary lipid oxidation were observed at T > 250 °C, resulting in a higher p-anisidine value and K232 as well as K268 values. Roasting improved oil yield (from 33.5 to 62.6%), increased its induction period (from 5.5 to 10.5 h) and enhanced the total phenolic content (from 152 to 194 mg/100 g) and antiradical activity of the extracted oil. Depending on roasting temperature, a gradual decline was recorded in total amounts of phytosterols (up to 17.4%), γ-tocopherol (up to 10.6%), sesamolin (maximum of 27.5%) and sesamin (maximum of 12.5%). All the investigated oils presented a low quantity in triglyceride polymers, clearly below the maximum tolerated quantity according to the European regulation. The optimal roasting temperature for obtaining high nutritional grade oil within the permissible values was 210 °C. The unsaponifiable components (including lignans and sterols) extracted from roasted seeds have been shown to be natural additives to fresh meatball products to extend shelf life. The results of this study may help to boost the nutritional content of plant-based diets by allowing for the use of roasted sesame seed oil and its components.

Can children's oral hygiene and sleep routines be compromised during the COVID-19 pandemic?

BACKGROUND: During COVID-19 pandemic, children are confined at home, with changes in family routines. AIM: Evaluate sleep disorders among Brazilian and Portuguese children during social distancing, and its association with parental perception of child's oral hygiene. DESIGN: In this cross-sectional study, Portuguese and Brazilian parents/caregivers of 3- to 15-year-old children, practicing social distancing due to COVID-19 pandemic, answered an online questionnaire, from April 24-26, 2020, evaluating sociodemographic characteristics, child's school activities online, child's sleep quality during social distancing. Two questions from the questionnaire, developed based on previous studies, evaluated the parental perception of child's oral hygiene quality and routine changes during social distancing. Parents/caregivers answered five domains of the Portuguese-language version of the Sleep Disturbances Scale for Children, evaluating sleep-breathing disorders, disorders of arousal, sleep-wake transition disorders, disorders of excessive somnolence and sleep hyperhidrosis. Descriptive, Linear-by-linear association, Kruskal-Wallis and post hoc analysis were performed (P ≤ .05). RESULTS: Participated in the study 253 parents/caregivers, 50.2% from Brazil. Most parents (72.2%) reported changes in child's routine during social distancing. Sleep breathing disorders (P = .019), sleep-wake transition disorders (P = .022), and disorders of excessive somnolence (P < .001) were associated with poor oral hygiene during social distancing. CONCLUSION: Sleep disorders are associated with poor oral hygiene during social distancing.

Skeletal open bite treated with clear aligners and miniscrews.

This case report describes the treatment of a 16-year-old female patient with a skeletal open bite and temporomandibular dysfunction. Clear aligners and miniscrews were used to control the occlusal plane and improve the skeletal problem. At the end of treatment, the mandible had rotated counterclockwise, allowing bite closure, upgrading dental and facial esthetics, and improving temporomandibular dysfunction.

Ascending aorta in tetralogy of Fallot: Beyond echocardiographic dimensions.

BACKGROUND: Late after tetralogy of Fallot (TOF) repair some patients exhibit aortic dilatation and stiffness. Noninvasive assessment of aortic stiffness could contribute to understand this aortopathy and may be important in risk stratification for major aortic event. METHODS: We included prospectively 82 adults after TOF repair and 41 age- and sex-matched normal controls. Aortic diameters were measured by two-dimensional transthoracic echocardiography and the aortic z-score was estimated. Aortic deformation was assessed by M-mode strain and global peak circumferential ascending aortic strain (CAAS), derived from two-dimensional speckle tracking echocardiography (2D-STE). Corrected CAAS was calculated as CAAS/pulse pressure. Ascending aorta (AAo) distensibility and stiffness index were calculated. RESULTS: TOF patients (age 29.7 ± 8.4 years; follow-up since TOF repair 23.0 ± 6.8 years) had smaller body surface area but a larger aorta compared to controls. TOF patients had lower AAo distensibility (2.2 [0.0-21.0] vs 5.6 [0.0-12.5] cm2 dyne-1 10-6 , P < .01), higher aortic stiffness index (9.5 [2.7-98.4] vs 7.1 [2.3-20.4], P = .02) and lower CAAS (6.0 ± 3.9 vs 8.1 ± 4.4%, P = .01) compared to controls. CAAS showed a better correlation with AAo z-score (r = -.25, P = .03) compared to M-mode strain. Systemic arterial compliance, arterial stiffness and corrected CAAS (ß = -0.23, P = .02) were independently associated with AAo diameter. CONCLUSIONS: TOF patients have a larger and stiffer AAo compared to controls. CAAS derived from 2D-STE allows a routine noninvasive method for assessing AAo stiffness, with advantages over M-mode strain, and may be used as predictor of major aortic or cardiovascular events.

Position and Eruption of Permanent Maxillary Canines in Cases of Maxillary Lateral Incisor Agenesis in Mixed Dentition.

OBJECTIVE: Assess whether the permanent maxillary canine (MC) has a natural tendency to erupt mesially in children with maxillary lateral incisors agenesis (MLIA), compared to children without agenesis. STUDY DESIGN: This retrospective, observational, cross-sectional study consisted of children between 5 and 12 years old divided into three groups: the first group with unilateral MLIA, in which an intraindividual analysis was performed, the second group presented bilateral MLIA, and the third group with patients without agenesis. These last two groups were matched for comparison interindividual, being pared by sex and maturation of the MC. RESULTS: The canine position in the horizontal sector showed a clear mesial positioning of the MC on the agenesis side in individuals with unilateral MLIA (group 1) when compared with the counter lateral side; and in individuals with bilateral MLIA (Group 2) compared with control Individuals without agenesis (group 3). Even with the maintenance of this deciduous tooth in the dental arch, the MC keeps its tendency to mesial eruption. CONCLUSION: There is a greater tendency for mesial angulation of the maxillary canine in patients with MLIA, regardless of the presence or absence of deciduous lateral incisor.

Left Ventricular Mechanical Reverse Remodelling Not Followed by Electrical Reverse Remodelling: A Case Report.

Patients with severely depressed left ventricular ejection fractions (LVEFs) receive implantable cardioverter-defibrillators (ICDs) for the primary prevention of sudden death. However, in some patients, LVEFs may improve or even normalize over time, and these patients would no longer be qualified for ICD implantation based on the original criteria for which they have initially received an ICD. We report a patient with idiopathic dilated cardiomyopathy whose LVEF recovered to normal values after pharmacological therapy. Meanwhile, the patient had life-threatening ventricular fibrillation, aborted by the ICD. We reflect on the pathological features of left ventricular reverse remodelling and ventricular arrhythmogenesis, where the myocardial substrate appears to play an important role. Also, after LVEF improvement in a patient with a cardiac device, there is still a debate on whether we should perform a battery replacement.

Esthetic Assessment of the Effect of Gingival Exposure in the Smile of Patients with Unilateral and Bilateral Maxillary Incisor Agenesis.

PURPOSE: The aim of this study was to determine the dental esthetic perception of the smile of patients with maxillary lateral incisor agenesis (MLIA); the perceptions were examined pre- and post-treatment. Esthetic determinations were made with regard to the gingival exposure in the patients' smile by orthodontists, general dentists, and laypersons. MATERIALS AND METHODS: Three hundred eighty one people (80 orthodontists, 181 general dentists, 120 laypersons) rated the attractiveness of the smile in four cases before and after treatment, comprising two cases with unilateral MLIA and contralateral microdontia and two with bilateral MLIA. For each case, the buccal photograph was adjusted using a computer to apply standard lips to create high, medium, and low smiles. A numeric scale was used to measure the esthetic rating perceived by the judges. The resulting arithmetic means were compared using an ANOVA test, a linear trend, and a Student's t-test, applying a significance level of p < 0.05. The predictive capability of the variables, unilateral, or bilateral MLIA, symmetry of the treatment, gingival exposure of the smile, group, and gender were assessed using a multivariable linear regression model. RESULTS: In the pre- and post-treatment cases, medium smile photographs received higher scores than the same cases with high or low smiles, with significant differences between them. In all cases, orthodontists were the least-tolerant evaluation group (assigning lowest scores), followed by general dentists. In a predictive linear regression model, bilateral MLIA was the more predictive variable in pretreatment cases. The gingival exposure of the smile was a predictive variable in post-treatment cases only. CONCLUSION: The medium-height smile was considered to be more attractive. In all cases, orthodontists gave the lowest scores, followed by general dentists. Laypersons and male evaluators gave the highest scores. Symmetrical treatments scored higher than asymmetrical treatments. The gingival exposure had a significant influence on the esthetic perception of smiles in post-treatment cases.

Spirito-Maron echocardiographic score: a marker for morphological and physiological assessment of patients with hypertrophic cardiomyopathy.

AIMS: The heterogeneous distribution of hypertrophy in hypertrophic cardiomyopathy (HCM) limits the echocardiographic conventional measurements accuracy in the evaluation of left ventricular hypertrophy (LVH). The aim of this study was to assess the correlation of the echocardiographic Spirito-Maron score (SMS) with left ventricle (LV) mass quantification by cardiac magnetic resonance (CMR) and with LV diastolic function. METHODS AND RESULTS: Left ventricle diastolic function parameters, SMS, LV mass (American Society of Echocardiography formula), and maximal wall thickness (MWT) were evaluated by two-dimensional (2D) transthoracic echocardiography. The SMS was obtained by adding the MWT of 4 LV segments, at the mitral valve or papillary muscles short-axis views. Echocardiographic parameters of LVH, including SMS, were correlated with LV mass obtained by CMR and with E/e' ratio. We included 45 patients (60% male, mean age 48 ± 18 years), who underwent 2D echocardiography. Twenty-two of them performed a CMR study. A positive correlation was found between SMS and CMR LV mass (r = 0.80; P < 0.001), whereas MWT (r = 0.62; P = 0.002) and the 2D LV mass (r = 0.60; P = 0.011) presented a lower correlation with CMR LV mass. The SMS was significantly correlated with E/e' ratio (r = 0.60; P = 0.007), whereas a nonsignificant correlation was found with MWT (r = 0.41; P = 0.081) and 2D LV mass (r = 0.22; P = 0.400). CONCLUSION: Spirito-Maron score presents a highly positive correlation with CMR LV mass and with diastolic dysfunction severity in HCM patients. SMS is a reliable quantitative LVH measurement method and seems to provide more comprehensive morphological and physiological information than 2D echocardiographic conventional parameters used to estimate LVH.

Treatment of an asymmetric malocclusion: a case report.

AIM: This case report describes an adult female patient who presented with dental asymmetry associated with bilaterally missing lower first molars, accompanied by extruded and mesially-tipped lower second and third molars. Masticatory dysfunction and temporomandibular joint (TMJ) pain were present during mouth opening and likely related to a posteriorly positioned condyle and anterior disc displacement with reduction. METHODS: Orthodontic treatment consisted of occlusal plane levelling and mandibular repositioning, achieved using a Multiloop Edgewise Archwire (MEAW) technique. Differential MEAW activation on each side and temporary occlusal composite bite blocks on the lower left third molar were necessary to increase the vertical dimension of the Class II side, which allowed extrusion of the left premolars to a Class I relationship. RESULTS: The bilateral uprighting of the second and third molars was achieved by tip-back wire activation to level the occlusal plane and intrude these teeth. Elastics worn vertically on the right side and Class II elastics on the left side facilitated occlusal correction. Dental implants for fixed prosthetic rehabilitation of the missing lower left first molar were placed to stabilise the posterior occlusion. Articular disc recovery removed TMJ symptoms and the case was finished with stable sagittal and vertical occlusal relationships. CONCLUSION: Successful management of a canted occlusal plane and the stabilisation of the posterior occlusion, resulted in a recovery of a displaced articular disc and improvement of facial symmetry.

Clear Aligners and Miniscrews in a Scissor Bite Adult Treatment.

Scissor bite does not correct spontaneously. It gradually worsens by overeruption, negatively affecting masticatory function. It is intended with this manuscript to evaluate the different treatment strategies to correct this malocclusion in adult patients, exploring treatment with clear aligners, bite ramps, and MS (miniscrews), especially in this case of a patient with unilateral right scissor bite, with high dental compensation in the three planes of space, asymmetrical sagittal dental position, overeruption on the scissor bite condition, and a high mandibular arch constriction and maxillary expansion. A comprehensive literature research was performed from 2002 until March 2023. PubMed and BVS databases were used, with the following keywords: "scissor bite OR brodie bite" AND "malocclusion" AND "treatment OR correction OR therapeutics". Since correcting skeletal asymmetries after the growth completion is challenging, adult patient cases often involve a combined orthodontic-surgical approach. In the present clinical case, the severe limitations to decompensating tooth positions for a surgical treatment, with the necessity to perform lower asymmetric extraction and a must longer orthodontic treatment, were the major reasons to avoid the surgical approach, after the scissor bite correction. In spite of this, the efficiency of the clear aligners and auxiliaries like bite ramps, MS, and elastics in successfully correcting a complex scissor bite in an adult patient was demonstrated, with significant esthetic and functional commitment, demonstrated by the case reliability PAR (peer assessment rating) index.

The Relationship between Personality Profiles and the Esthetic Perception of Orthodontic Appliances.

Introduction: In orthodontics, patients' esthetic expectations involve the final esthetic result as well as the treatment's appliance choice. Personality traits can influence patients' perception, treatment modality selection, expectations, compliance, and satisfaction with the treatment outcome, although there are very few studies on this interconnection between personality and orthodontic appliances. Objective: The aim of this study is to evaluate the relationship between personality traits and the esthetic perception of different orthodontic appliances. Materials and Methods: The online questionnaire is composed of three parts: (1) sociodemographic variables; (2) questions on the esthetic perception of different orthodontic appliances; (3) general personality assessment via the NEO-FFI. A total of 461 questionnaires were accepted. Results: There were statistically significant differences between laypeople and professionals related to assessing smiles and the need for orthodontic treatment. Laypeople identified a statistically significant relationship between personality traits and orthodontic appliances, in which esthetic perception is associated with high traits of agreeableness and openness. Neuroticism is most associated with orthodontic treatment with fixed appliances, which are the most conventional. Conclusions: Professionals tend to have a more critical judgment than laypeople as far as esthetics is concerned. Personality traits play an important role in esthetic perception and may influence orthodontic treatment.

Optimization of a Photobiomodulation Protocol to Improve the Cell Viability, Proliferation and Protein Expression in Osteoblasts and Periodontal Ligament Fibroblasts for Accelerated Orthodontic Treatment.

Numerous pieces of evidence have supported the therapeutic potential of photobiomodulation (PBM) to modulate bone remodeling on mechanically stimulated teeth, proving PBM's ability to be used as a coadjuvant treatment to accelerate orthodontic tooth movement (OTM). However, there are still uncertainty and discourse around the optimal PBM protocols, which hampers its optimal and consolidated clinical applicability. Given the differential expression and metabolic patterns exhibited in the tension and compression sides of orthodontically stressed teeth, it is plausible that different types of irradiation may be applied to each side of the teeth. In this sense, this study aimed to design and implement an optimization protocol to find the most appropriate PBM parameters to stimulate specific bone turnover processes. To this end, three levels of wavelength (655, 810 and 940 nm), two power densities (5 and 10 mW/cm2) and two regimens of single and multiple sessions within three consecutive days were tested. The biological response of osteoblasts and periodontal ligament (PDL) fibroblasts was addressed by monitoring the PBM's impact on the cellular metabolic activity, as well as on key bone remodeling mediators, including alkaline phosphatase (ALP), osteoprotegerin (OPG) and receptor activator of nuclear factor κ-B ligand (RANK-L), each day. The results suggest that daily irradiation of 655 nm delivered at 10 mW/cm2, as well as 810 and 940 nm light at 5 mW/cm2, lead to an increase in ALP and OPG, potentiating bone formation. In addition, irradiation of 810 nm at 5 mW/cm2 delivered for two consecutive days and suspended by the third day promotes a downregulation of OPG expression and a slight non-significant increase in RANK-L expression, being suitable to stimulate bone resorption. Future studies in animal models may clarify the impact of PBM on bone formation and resorption mediators for longer periods and address the possibility of testing different stimulation periodicities. The present in vitro study offers valuable insights into the effectiveness of specific PBM protocols to promote osteogenic and osteoclastogenesis responses and therefore its potential to stimulate bone formation on the tension side and bone resorption on the compression side of orthodontically stressed teeth.