Oil Pulling and Polyphenols: Treatment of Gingivitis Patients with 'Itri Extra-Virgin Olive Oil'.

OBJECTIVES: The aim of the study was to evaluate the effectiveness of extra virgin olive (EVO) oil and fruity oil for the treatment of gingivitis. MATERIALS AND METHODS: A sample of 75 patients over 18 years of age with gingivitis induced by plaque bacteria was divided into three groups: study group A, with extra virgin olive oil; study group B, with fruity oil; and control group C. In the two study groups, EVO oil was administered as a mouthwash to patients with gingival inflammation. The protocol included a daily application of the product for 30 days, with three recalls 15 days apart. Clinical parameters of plaque formation and gingivitis, including plaque index (PI) and bleeding index (BI), were assessed at each recall and scored on a specific periodontal chart. The control group received no mouthwash treatment in addition to normal daily oral hygiene procedures, and the same clinical parameters as the study group were evaluated. Data were evaluated using SPSS 27.0 software for Windows (SPSS Inc., Chicago, IL, USA). Then, the pre- and post-treatment values of the groups were compared using Student's t-test, setting p < 0.05 as the significance level. RESULTS: Comparison of the three groups showed that extra-virgin olive oil was an adjuvant in the treatment of gingival inflammation, improving PI and BI. In group A, the mean plaque index showed a 48% reduction, and the bleeding index showed a 64% reduction after 30 days. In group B, the mean plaque index showed a 35% reduction and a bleeding index reduction of 43% after 30 days. CONCLUSIONS: The collected data showed significant improvements in the formation of bacterial plaque and gingivitis. The exact mechanism of such treatment is still to be elucidated. As a result of this, further studies with a different sample of patients than those used and a comparison with other products need to be addressed to verify and demonstrate the antibacterial and anti-inflammatory effects of the components of this natural product.

Impact of Stress on Periodontal Health: Literature Revision.

Stress is a physiological response of the body to stressful life events but may not be when the individual is persistently exposed to the stress trigger, and it negatively affects certain physiological functions, thus triggering psychosomatic diseases. In literature, chronic stress and inadequate coping strategies are found to mediate the risk and development of periodontitis; mechanisms have therefore been proposed to explain the effects of stress on the periodontium. Since stress is a prevalent problem in modern life and given the importance of maintaining oral health, the present literature review aimed to estimate the association between stress and periodontal disease. The research question adopted is the following: "Are psychological stress and periodontal disease related?" The search was conducted in August 2022 and limited to articles in electronic databases from 2017 to 2022 in English, excluding reviews and literature reviews. From the electronic databases, a total of 532 articles were identified and became 306 after reviews and duplicates were removed. An additional bibliographic search was conducted through the same electronic databases, controlled terms and keywords including only systematic reviews, which were previously excluded. Through the bibliography cited in the systematic reviews, an additional 18 articles were identified, with a new total of 324. As a result of reading the title and abstract of these 324 articles, an additional 295 were excluded. Reading the full text of the remaining 29 studies, 2 articles were excluded due to non-adherence to the eligibility criteria. The remaining 27 results were included in our literature review. It has been suggested in the literature that adverse socioeconomic conditions elicit a stress response, which can trigger periodontal inflammation. Most of the 27 articles included in the study confirm and demonstrate a positive association between psychological stress and periodontal disease. Numerous studies have shown the mechanisms through which chronic stress negatively affects periodontal tissues. Therefore, in the light of the results obtained from this review, it is important that oral health professionals, also for general health purposes, consider stress factors among the risk factors of periodontal disease, its severity and decreased efficacy of treatments. It is therefore advisable to act preventively through the interception of chronic stress.

Monoclonal Antibodies against SARS-CoV-2 Infection: Results from a Real-Life Study before the Omicron Surge.

Despite the lightning-fast advances in the management of SARS-CoV after 2 years of pandemic, COVID-19 continues to pose a challenge for fragile patients, who could benefit from early administration of monoclonal antibodies (mAbs) to reduce the risk of severe disease progression. We conducted a prospective study to evaluate the effectiveness of mAbs against SARS-CoV-2 among patients at risk for severe disease progression, namely elderly and those with comorbidities, before the omicron variant surge. Patients were treated with either casirivimab/imdevimab, sotrovimab, or bamlanivimab/etesevimab. The rates and risk factors for clinical worsening, hospitalization, ICU admission and death (unfavorable outcomes) were evaluated. A stratified analysis according to the presence of SARS-CoV-2 IgG was also performed. Among 185 included patients, we showed low rates of unfavorable outcomes (9.2%), which were more frequent in patients with chronic kidney disease (aOR: 10.44, 95% CI: 1.73−63.03; p < 0.05) and basal D-dimer serum concentrations > 600 ng/mL (aOR 21.74, 95% CI: 1.18−397.70; p < 0.05). Patients with negative SARS-CoV-2 serology at baseline showed higher C-reactive protein values compared with patients with positive serology (p < 0.05) and a trend toward a higher admission rate to SICU and ICU compared with patients with positive serology. Our results thus showed, in a real-life setting, the efficacy of mAbs against SARS-CoV-2 before an Omicron surge when the available mabs become not effective.

Oral Hygiene Habits and Use of Fluoride in Developmental Age: Role of Parents and Impact on their Children.

Introduction: In healthcare, the need to pay more attention to the achievement of two objectives within the society arises: health promotion and prevention in terms of nutrition, good education, sport, and health education. Scientific evidence shows that adequate health standards must be learned since childhood through the help of parents and appropriate school projects. Parental intervention must be appropriate to support the responsibility of their children's health. In oral health, it has been established for many years that there is a correlation between parental behaviors and lifestyles and children's attitude. The aim of this study is to verify the close relation between behaviors, habits, lifestyles, and the knowledge of parents about their oral health and, consequently, their focus and care for their own children's oral health. Furthermore, the awareness of parents about the importance and use of fluorine was to be determined. Materials and Methods: The study lasted 15 months and was conducted from April 2018 to July 2019: an anonymous 29-question questionnaire was administered to all parents who accompanied their children (aged between 3 and 12 years) going under treatment in the Pediatric Dentistry Unit of the University Hospital Policlinico Umberto I, Rome. Anamnestic data, sociodemographic context (e.g., educational level and occupation), oral health habits, and prevention of parents and children and fluoride knowledge were investigated. The study received ethical approval. 204 questionnaires were collected. The data gathered were recorded with a specifically designed computer program and collected and analyzed using a Microsoft Excel 10 database. Data were evaluated using standard statistical analysis software; descriptive statistics including mean ± SD values and percentage were calculated for each variable. The relationship between the age of parents, between mother or father and the parents' degree of education levels, and the knowledge for their own children's oral health was explored using the chi-square test of homogeneity and Fisher's exact test (P value of < 0.05 considered as statistically significant). Results: From the acquired data, it is possible to deduce that the major respondents were mothers aged from 36 to 45, while only a small part were fathers aged above 45 years. Questions related to parents' oral hygiene habits were included in the questionnaire, and from the sample taken into consideration, it emerges that 64.7% of the respondents (67.1% mothers and 57.7% fathers) periodically attend a dental office for a checkup, 20.9% tend to postpone the treatment, and 15.2% go there just for emergency. Some of the questions showed that 80% of the interviewed subjects use fluoride toothpaste for their child's oral hygiene. Conclusion: Prevention in childhood, in addition to being synonymous with monitoring the oral health of the child, means first of all to pay attention to parents who are the main behavioral reference. It emerged that there is no adequate knowledge about fluorine, especially when the subjects have a low educational level. A role of fundamental importance for the diffusion of adequate concepts in the field of oral hygiene is covered, according to the data received from the study carried out, by the dentist and dental hygienist.

Musculoskeletal disorders related to dental hygienist profession.

OBJECTIVES: Musculoskeletal disorders (MSDs) are occupational illnesses concerned with different classes of professionals; dental hygienists are among those. The aim of this trial is to evaluate MSDs prevalence and significance of the symptoms in a sample of dental hygienists. MATERIALS AND METHODS: A 20-question questionnaire was administered to a sample of dental hygienists, via social networks. The variables taken into consideration were personal data, hours of sport, working habits, years of professional activity, working hours and number of patients per week, presence or absence of pain. STATISTICAL ANALYSIS: Data were evaluated using standard statistical analysis software, and an Excel database was created. Descriptive statistics were calculated for each variable. Group comparison was assessed by the chi-square test of homogeneity and Fisher's exact test. (p-value <0.05 as significant). RESULTS: 468 questionnaires were examined: 396 females (85%) and 72 males (15%). The prevailing age was between 25 and 35. Among them, 91% referred to be suffering or have suffered MSDs. The most relevant affected muscular areas are neck (30.6%), shoulder (25.0%) and lumbosacral region (23.3%); the remaining 21.1% is divided among the other regions. Association and statistical analysis among the different variables showed how presence of MSDs negatively influences absenteeism and work performance; further research regarding ergonomics, type of seat, stretching and workout prevention would be important to strengthen the results collected. CONCLUSIONS: Musculoskeletal disorders diffusion among dental hygienists is particularly high due to lack of information; the majority of interviewees showed very little awareness of it; this led to a lack of effort in facing or possibly preventing these pathologies.

Impact of COVID-19 infection on short-term outcome in patients referred to stress myocardial perfusion imaging.

PURPOSE: We assessed the impact of COVID-19 infection on cardiovascular events in patients with suspected or known coronary artery disease (CAD) referred to stress single-photon emission computed tomography myocardial perfusion imaging (MPS). METHODS: A total of 960 consecutive patients with suspected or known CAD were submitted by referring physicians to stress MPS for assessment of myocardial ischemia between January 2018 and June 2019. All patients underwent stress-optional rest MPS. Perfusion defects were quantitated as % of LV myocardium and expressed as total perfusion defect (TPD), representing the defect extent and severity. A TPD ≥ 5% was considered abnormal. RESULTS: During a mean follow-up of 27 months (range 4-38) 31 events occurred. Moreover, 55 (6%) patients had a COVID-19 infection. The median time from index MPS to COVID-19 infection was 16 months (range 6-24). At Cox multivariable analysis, abnormal MPS and COVID-19 infection resulted as independent predictors of events. There were no significant differences in annualized event rate in COVID-19 patients with or without abnormal MPS (p = 0.56). Differently, in patients without COVID-19, the presence of abnormal MPS was associated with higher event rate (p < .001). Patients with infection compared to those without had a higher event rate in the presence of both normal and abnormal TPD. CONCLUSION: In patients with suspected or known CAD, the presence of COVID-19 infection during a short-term follow-up was associated with a higher rate of cardiovascular events.

Diet Implications and Oral Health Status of Women in Central Italy.

OBJECTIVE: The relationships between oral health habits, dietary practices, and oral health status, as well as general health status, in the population of Italian women, are complex, with many mutual and interrelating factors. The purpose of this study is to investigate oral habits, oral status, and dietary habits of a sample of women in Italy, highlighting the links between nutrition and oral health and discussing how oral health care professionals can integrate nutrition counseling that aimed at improving the oral health of their patients into their practices. MATERIALS AND METHODS: In the period between February 2020 and July 2020, an anonymous questionnaire made up of 20 questions was administered to a randomized sample of 120 Italian women. RESULTS: Most of women declare good manual skills in oral hygiene (50%) but just less than half of them brushes their teeth more than three times a day. Statistically significant correlations were found between frequency of dental checkups and dental mobility (p = 0.036), and halitosis (p = 0.006), as well as between frequency of flossing and gum bleeding. Correlation between the type of diet and oral health status showed more halitosis (∼21%), sensitivity (∼26%), and xerostomia (∼53.3%) for vegetarian and vegan women. CONCLUSION: More awareness need to be raised concerning oral hygiene habits, and regarding the importance of regular dental checkups. Brushing at least twice or three times a day needs to be encouraged and the valid support of dental aids has to be in the everyday domestic oral hygiene protocol, as scientific evidence demonstrates. Future clinical studies need to be performed on a more consistent number of vegetarian and vegan patients, to obtain more statistically significant results and support future research that will compare omnivorous, vegetarian, and vegan diets and their influence on oral health status.

The Role of the Pediatric Dentist in the Multidisciplinary Management of the Cleft Lip Palate Patient.

The focus of this paper is the pediatric dental care of Cleft Lip and Palate (CLP) children and the role of the pediatric dentist in the CLP team. The management of children with cleft lip and palate presents many challenges and a multidisciplinary and prepared team is always required. Affected individuals present a multiplicity of problems: effective management involves a wide range of specialities. The value of a multidisciplinary team is widely known and mentioned in the literature, but very few papers focus on the role and the importance of the pediatric dentist. Therefore, the purpose of this article is to underline the role of the pediatric dentist as a member of the cleft lip and palate team which ranges from prenatal counseling, presurgical prevention and orthopedics, to post-treatment rehabilitation and restoration.

Root Fracture and Extrusive Luxation in Primary Teeth and Their Management: A Case Report.

BACKGROUND: Extrusion, lateral luxation, and intrusion are among the most serious types of dental trauma. Only a few studies have specifically focused on extrusion; the present one was aimed at reporting a case of domestic traumatic dental injury to primary tooth and describing the measures taken in managing the trauma in order to avoid future consequences to the underlying permanent tooth germ. CASE REPORT: A 3.5-year-old boy reported a dental injury with extrusion and root fracture of deciduous tooth 5.1. After intraoral and radiographic evaluation, the element was repositioned and stabilized by an orthodontic flexible splint attached to the adjacent teeth. Several follow-up checkups were made and showed good healing of the tissues and physiological exfoliation of the tooth, with a healthy and unaffected corresponding central permanent incisor. CONCLUSION: This case report strengthens the importance of well-timed diagnosis and treatment and of regular follow-up of traumatized teeth as they may affect both dentitions with a negative impact on Oral Health-Related Quality of Life. Conservative treatment should be taken into consideration when possible, being in some cases more appropriate.

The Role of Coconut Oil in Treating Patients Affected by Plaque-Induced Gingivitis: A Pilot Study.

OBJECTIVES: The aim of the study was to evaluate the coconut oil pulling efficacy as adjuvant in reducing plaque formation and in treating plaque-induced gingivitis. MATERIALS AND METHODS: A sample of 20 patients was divided into two groups: a study and a control group. In the study group, coconut oil, in form of mouthwash, was administered to a sample of patients affected by gingivitis, aged between 18 and 35. The protocol established a daily application of the product for 30 days, where clinical parameters for plaque formation and gingivitis-plaque index (PI), bleeding index (BI)-will be evaluated during the recalls on a specific periodontal chart. The control group did not associate a coadjuvant to the normal daily oral health procedures and the same clinical parameters were evaluated at t0 and after 30 days (t1). The data were statistically analyzed using Student's t-test, establishing the significance level as p < 0.05. RESULTS: PI and BI decreased in both groups, with a more relevant and significant drop in the study group, from a mean value of PI of 58.0 to 19.3 and a mean value of BI of 33.5 to 5.0. In the control group, the values decreased, respectively, from 53.9 to 29.1 for PI, and from 33.5 to 16.2. Furthermore, no significant side effect was reported during coconut oil pulling therapy. CONCLUSIONS: The collected data showed significant and promising improvements in reducing plaque formation and gingivitis. However, further researches have to be performed to have more consistent and statistically significant data on larger samples and to fully understand the mechanisms of action and effectiveness.

Multiple Sclerosis: Impact on Oral Hygiene, Dysphagia, and Quality of Life.

Multiple sclerosis (MS) is an autoimmune disease in which the immune system reacts by damaging the central nervous system, specifically myelin and oligodendrocytes. It is the most debilitating neurological disease among young adults, causing personal, familiar, social, and professional limitations. Multiple sclerosis can cause disturbances in the orofacial district, due to a demyelination process on the nerves of the head and neck district. The aim of this study was to evaluate the oral health status, dysphagia, and quality of life of patients affected by MS. For this study, 101 patients aged between 12 and 70 (47 males, 54 females) affected by MS were selected, and three questionnaires were handed out and anonymously filled in by them: An oral hygiene test, DYMUS (DYsphagia in MUltiple Sclerosis), and IOHIP-14 (Italian version Oral Health Impact Profile). Through the analysis of the questionnaires it was possible to observe pathological conditions, such as gingival inflammation, xerostomia, dysphagia, neuralgia, and dysarthria. Through the analysis it was possible to outline how the roles of a medical team, composed of a dentist, otolaryngologist, and dental hygienist, are fundamental in coping with other medical figures, during the whole development of the diseases, as well as to prevent possible complications.

Tea Tree Oil versus Chlorhexidine Mouthwash in Treatment of Gingivitis: A Pilot Randomized, Double Blinded Clinical Trial.

OBJECTIVE: The study evaluated the efficacy of tea tree oil for the treatment of gingivitis. MATERIALS AND METHODS: The tea tree oil was administered in the form of mouthwash and then compared with a mouthwash with chlorhexidine 0.12%. Both treatments were domestic and lasted for 14 days. Patients were chosen according to random criteria, aged between 18 and 60 years, and who showed a clinically evident gingivitis. In clinical evaluation, the following clinical criteria were taken into consideration: gingival index (GI), plaque index (PI), bleeding index (BI), probing depth (PD), the presence of dental dyschromia, and the presence of taste alteration. The subjects were evaluated before (T0) and after the treatment (T1), and the data collected for each patient were recorded on a periodontal chart. RESULTS: The comparison showed that tea tree oil offered a better improvement in the evaluation of PI, BOP, and PD; furthermore, it did not cause dental dyschromia and taste alteration. In group A, treated with tea tree oil, PI decreased from 53.25 to 5.50% and BI from 38.41 to 4.22%. In group B, treated with chlorhexidine PI decreased from 47.69 to 2.37% and BI from 32.93 to 6.28%. Instead, the subjects using chlorhexidine 0.12% blamed a distaste for the product that caused a slight taste alteration; 20% of them showed iatrogenic dental dyschromia. CONCLUSIONS: The collected data showed the efficacy of both treatments. Although further research works will be necessary, this study showed that tea tree oil could be an effective nontoxic substitute for the therapy of gingivitis.

Cardiac Structural and Functional Consequences of Amyloid Deposition by Cardiac Magnetic Resonance and Echocardiography and Their Prognostic Roles.

OBJECTIVES: This cross-sectional study aimed to describe the functional and structural cardiac abnormalities that occur across a spectrum of cardiac amyloidosis burden and to identify the strongest cardiac functional and structural prognostic predictors in amyloidosis using cardiac magnetic resonance (CMR) and echocardiography. BACKGROUND: Cardiac involvement in light chain and transthyretin amyloidosis is the main driver of prognosis and influences treatment strategies. Numerous measures of cardiac structure and function are assessed by multiple imaging modalities in amyloidosis. METHODS: A total f 322 subjects (311 systemic amyloidosis and 11 transthyretin gene mutation carriers) underwent comprehensive CMR and transthoracic echocardiography. The probabilities of 11 commonly measured structural and functional cardiac parameters being abnormal with increasing cardiac amyloidosis burden were evaluated. Cardiac amyloidosis burden was quantified using CMR-derived extracellular volume. The prognostic capacities of these parameters to predict death in amyloidosis were assessed using Cox proportional hazards models. RESULTS: Left ventricular mass and mitral annular plane systolic excursion by CMR along with strain and E/e' by echocardiography have high probabilities of being abnormal at low cardiac amyloid burden. Reductions in biventricular ejection fractions and elevations in biatrial areas occur at high burdens of infiltration. The probabilities of indexed stroke volume, myocardial contraction fraction, and tricuspid annular plane systolic excursion (TAPSE) being abnormal occur more gradually with increasing extracellular volume. Ninety patients (28%) died during a median follow-up of 22 months (interquartile range: 10 to 38 months). Univariable analysis showed that all imaging markers studied significantly predicted outcome. Multivariable analysis showed that TAPSE (hazard ratio: 1.46; 95% confidence interval: 1.16 to 1.85; p < 0.01) and indexed stroke volume (hazard ratio: 1.24; 95% confidence interval: 1.04 to 1.48; p < 0.05) by CMR were the only independent predictors of mortality. CONCLUSIONS: Specific functional and structural abnormalities characterize different burdens of cardiac amyloid deposition. In a multimodality imaging assessment of a large cohort of amyloidosis patients, CMR-derived TAPSE and indexed stroke volume are the strongest prognostic cardiac functional markers.

Myocardial Edema and Prognosis in Amyloidosis.

BACKGROUND: Prognosis in light-chain (AL) and transthyretin (ATTR) amyloidosis is influenced by cardiac involvement. ATTR amyloidosis has better prognosis than AL amyloidosis despite more amyloid infiltration, suggesting additional mechanisms of damage in AL amyloidosis. OBJECTIVES: The aim of the study was to assess the presence and prognostic significance of myocardial edema in patients with amyloidosis. METHODS: The study recruited 286 patients: 100 with systemic AL amyloidosis, 163 with cardiac ATTR amyloidosis, 12 with suspected cardiac ATTR amyloidosis (grade 1 on 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid), 11 asymptomatic individuals with amyloidogenic TTR gene mutations, and 30 healthy volunteers. All subjects underwent cardiovascular magnetic resonance with T1 and T2 mapping and 16 underwent endomyocardial biopsy. RESULTS: Myocardial T2 was increased in amyloidosis with the degree of elevation being highest in untreated AL patients (untreated AL amyloidosis 56.6 ± 5.1 ms; treated AL amyloidosis 53.6 ± 3.9 ms; ATTR amyloidosis 54.2 ± 4.1 ms; each p < 0.01 compared with control subjects: 48.9 ± 2.0 ms). Left ventricular (LV) mass and extracellular volume fraction were higher in ATTR amyloidosis compared with AL amyloidosis while LV ejection fraction was lower (p < 0.001). Histological evidence of edema was present in 87.5% of biopsy samples ranging from 5% to 40% myocardial involvement. Using Cox regression models, myocardial T2 predicted death in AL amyloidosis (hazard ratio: 1.48; 95% confidence interval: 1.20 to 1.82) and remained significant after adjusting for extracellular volume fraction and N-terminal pro-B-type natriuretic peptide (hazard ratio: 1.32; 95% confidence interval: 1.05 to 1.67). CONCLUSIONS: Myocardial edema is present in cardiac amyloidosis by histology and cardiovascular magnetic resonance T2 mapping. T2 is higher in untreated AL amyloidosis compared with treated AL and ATTR amyloidosis, and is a predictor of prognosis in AL amyloidosis. This suggests mechanisms additional to amyloid infiltration contributing to mortality in amyloidosis.

Extracellular volume with bolus-only technique in amyloidosis patients: Diagnostic accuracy, correlation with other clinical cardiac measures, and ability to track changes in amyloid load over time.

BACKGROUND: Extracellular volume (ECV) by T1 mapping requires the contrast agent distribution to be at equilibrium. This can be achieved either definitively with a primed contrast infusion (infusion ECV), or sufficiently with a delay postbolus (bolus-only ECV). For large ECV, the bolus-only approach measures higher than the infusion ECV, causing some uncertainty in diseases such as amyloidosis. PURPOSE: To characterize the relationship between the bolus-only and current gold-standard infusion ECV in patients with amyloidosis. STUDY TYPE: Bolus-only and infusion ECV were prospectively measured. POPULATION: In all, 186 subjects with systemic amyloidosis attending our clinic and 23 subjects with systemic amyloidosis who were participating in an open-label, two-part, dose-escalation, phase 1 trial. FIELD STRENGTH: Avanto 1.5T, Siemens Medical Solutions, Erlangen, Germany. ASSESSMENT: Bolus-only and infusion ECV were measured in all subjects using shortened modified Look-Locker inversion recovery (ShMOLLI) T1 mapping sequence. STATISTICAL TESTS: Pearson correlation coefficient (r); Bland-Altman; receiver operating characteristic (ROC) curve analysis. Linear regression model with a fractional polynomial transformation. RESULTS: The difference between the bolus-only and infusion myocardial ECV increased as the average of the two measures increased, with the bolus-ECV measuring higher. For an average ECV of 0.4, the difference was 0.013. The 95% limits of agreement for the two methods, after adjustment for the bias, were ±0.056. However, cardiac diagnostic accuracy was comparable (bolus-only vs. infusion ECV area under the curve [AUC] = 0.839 vs. 0.836), as were correlations with other clinical cardiac measures, and, in the trial patients, the ability to track changes in the liver/spleen with therapy. DATA CONCLUSION: In amyloidosis, with large ECVs, the bolus-only technique reads higher than the infusion technique, but clinical performance by any measure is the same. Given the work-flow advantages, these data suggest that the bolus-only approach might be acceptable for amyloidosis, and might support its use as a surrogate endpoint in future clinical trials. LEVEL OF EVIDENCE: 1 Technical Efficacy: Stage 4 J. Magn. Reson. Imaging 2018;47:1677-1684.

Magnetic Resonance in Transthyretin Cardiac Amyloidosis.

BACKGROUND: Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure. Cardiac magnetic resonance (CMR), with late gadolinium enhancement (LGE) and T1 mapping, is emerging as a reference standard for diagnosis and characterization of cardiac amyloidosis. OBJECTIVES: The authors used CMR with extracellular volume fraction (ECV) measurement to characterize cardiac involvement in relation to outcome in ATTR. METHODS: Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 99mTc-DPD (99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 99mTc-DPD), and 12 asymptomatic individuals with amyloidogenic transthyretin (TTR) mutations. Fifty patients with cardiac light-chain (AL) amyloidosis acted as disease comparators. RESULTS: Unlike cardiac AL amyloidosis, asymmetrical septal left ventricular hypertrophy (LVH) was present in 79% of patients with ATTR (70% sigmoid septum and 30% reverse septal contour), whereas symmetrical LVH was present in 18%, and 3% had no LVH. In patients with cardiac amyloidosis, the pattern of LGE was always typical for amyloidosis (29% subendocardial, 71% transmural), including right ventricular LGE (96%). During follow-up (19 ± 14 months), 65 patients died. ECV independently correlated with mortality and remained independent after adjustment for age, N-terminal pro-B-type natriuretic peptide, ejection fraction, E/E', and left ventricular mass (hazard ratio: 1.164; 95% confidence interval: 1.066 to 1.271; p < 0.01). CONCLUSIONS: Asymmetrical hypertrophy, traditionally associated with hypertrophic cardiomyopathy, was the commonest pattern of ventricular remodeling in ATTR. LGE imaging was typical in all patients with cardiac ATTR. ECV correlated with amyloid burden and was an independent prognostic factor for survival in this cohort of patients.

New and developing therapies for AL amyloidosis.

INTRODUCTION: Systemic light-chain (AL) amyloidosis is an infiltrative disorder associated with an underlying plasma cells dyscrasia, in which monoclonal immunoglobulin light chains accumulate in an abnormal misfolded form as amyloid fibrils in the extracellular space. Symptoms and prognosis are governed by which organs are affected, and cardiac involvement is the major determinant of survival. Diagnosis requires demonstration of amyloid deposition and confirmation of the fibril protein type. Areas covered: This review will focus on the available treatments for systemic AL amyloidosis and on new drug targets and therapeutic approaches. Expert opinion: At present, the choice of upfront treatment lies between autologous stem cell transplantation (ASCT) and combination chemotherapy. Chemotherapy agents include dexamethasone, melphalan, cyclophosphamide, thalidomide, bortezomib, lenalidomide, bendamustine in various combinations. Few randomized controlled trials have been performed in AL amyloidosis and treatment has been substantially influenced by clinical practice in myeloma. It has become clear that the best prospects of survival and preservation or improvement in amyloid related organ function require as near complete suppression as possible of the underlying hematological disorder. Future directions include therapies designed to target amyloid deposits directly, in particular anti-amyloid antibodies which are now well advanced in development and are showing great potential.