Factors influencing U.S. women's interest and preferences for breast cancer risk communication: a cross-sectional study from a large tertiary care breast imaging center.

BACKGROUND: Breast imaging clinics in the United States (U.S.) are increasingly implementing breast cancer risk assessment (BCRA) to align with evolving guideline recommendations but with limited uptake of risk-reduction care. Effectively communicating risk information to women is central to implementation efforts, but remains understudied in the U.S. This study aims to characterize, and identify factors associated with women's interest in and preferences for breast cancer risk communication. METHODS: This is a cross-sectional survey study of U.S. women presenting for a mammogram between January and March of 2021 at a large, tertiary breast imaging clinic. Survey items assessed women's interest in knowing their risk and preferences for risk communication if considered to be at high risk in hypothetical situations. Multivariable logistic regression modeling assessed factors associated with women's interest in knowing their personal risk and preferences for details around exact risk estimates. RESULTS: Among 1119 women, 72.7% were interested in knowing their breast cancer risk. If at high risk, 77% preferred to receive their exact risk estimate and preferred verbal (52.9% phone/47% in-person) vs. written (26.5% online/19.5% letter) communications. Adjusted regression analyses found that those with a primary family history of breast cancer were significantly more interested in knowing their risk (OR 1.5, 95% CI 1.0, 2.1, p = 0.04), while those categorized as "more than one race or other" were significantly less interested in knowing their risk (OR 0.4, 95% CI 0.2, 0.9, p = 0.02). Women 60 + years of age were significantly less likely to prefer exact estimates of their risk (OR 0.6, 95% CI 0.5, 0.98, p < 0.01), while women with greater than a high school education were significantly more likely to prefer exact risk estimates (OR 2.5, 95% CI 1.5, 4.2, p < 0.001). CONCLUSION: U.S. women in this study expressed strong interest in knowing their risk and preferred to receive exact risk estimates verbally if found to be at high risk. Sociodemographic and family history influenced women's interest and preferences for risk communication. Breast imaging centers implementing risk assessment should consider strategies tailored to women's preferences to increase interest in risk estimates and improve risk communication.

Relationship between Race and Access to Newer Mammographic Technology in Women with Medicare Insurance.

Background Racial disparities in breast cancer mortality have been reported. Mammographic technology has undergone two major technology transitions since 2000: first, the transition from screen-film mammography (SFM) to full-field digital mammography (FFDM) and second, the transition to digital breast tomosynthesis (DBT). Purpose To examine the relationship between use of newer mammographic technology and race in women receiving mammography services. Materials and Methods This was a multiyear (January 2005 to December 2020) retrospective study of women aged 40-89 years with Medicare fee-for-service insurance who underwent mammography. Data were obtained using a 5% research identifiable sample of all Medicare fee-for-service beneficiaries. Within-institution and comparable-institution use of mammographic technology between Black women or women of other races and White women were assessed with multivariable logistic and linear regression, respectively, adjusted for age, race, Charlson comorbidity index, per capita income, urbanicity, and institutional capability. Results Between 2005 and 2020, there were 4 028 696 institutional mammography claims for women (mean age, 72 years ± 8 [SD]). Within an institution, the odds ratio (OR) of Black women receiving digital mammography rather than SFM in 2005 was 0.80 (95% CI: 0.70, 0.91; P < .001) when compared with White women; these differences remained until 2009. Compared with White women, the use of DBT within an institution was less likely for Black women from 2015 to 2020 (OR, 0.84; 95% CI: 0.81, 0.87; P < .001). Across institutions, there were racial differences in digital mammography use, which followed a U-shaped pattern, and the differences peaked at 3.8 percentage points less for Black compared with White women (95% CI: -6.1, -1.6; P = .001) in 2011 and then decreased to 1.2 percentage points less (95% CI: -2.2, -0.2; P = .02) in 2016. Conclusion In the Medicare population, Black women had less access to new mammographic imaging technology compared with White women for both the transition from screen-film mammography to digital mammography and then for the transition to digital breast tomosynthesis. © RSNA, 2022 Online supplemental material is available for this article. See also the editorial by Lee and Lawson in this issue.

NCCN Guidelines® Insights: Breast Cancer Screening and Diagnosis, Version 1.2023.

The NCCN Guidelines for Breast Cancer Screening and Diagnosis provide health care providers with a practical, consistent framework for screening and evaluating a spectrum of clinical presentations and breast lesions. The NCCN Breast Cancer Screening and Diagnosis Panel is composed of a multidisciplinary team of experts in the field, including representation from medical oncology, gynecologic oncology, surgical oncology, internal medicine, family practice, preventive medicine, pathology, diagnostic and interventional radiology, as well as patient advocacy. The NCCN Breast Cancer Screening and Diagnosis Panel meets at least annually to review emerging data and comments from reviewers within their institutions to guide updates to existing recommendations. These NCCN Guidelines Insights summarize the panel's decision-making and discussion surrounding the most recent updates to the guideline's screening recommendations.

Barriers to Implementation of Contrast-Enhanced Mammography in Clinical Practice: AJR Expert Panel Narrative Review.

Accumulating evidence shows that contrast-enhanced mammography (CEM) has higher diagnostic performance than digital mammography and ultrasound and comparable diagnostic performance to MRI for various indications. CEM also offers certain practical advantages for patients. Nevertheless, the clinical implementation of CEM has been limited because of a range of factors. This AJR Expert Panel Narrative Review explores such factors hindering CEM implementation. These factors include the following: the risks of iodinated contrast media, increased radiation exposure, indications for which CEM is not the preferred test or for which further evidence is needed, workflow adjustments needed when performing CEM examinations, incomplete availability of CEM-guided biopsy systems, and reimbursement challenges. Considerations that currently mitigate or are expected to mitigate these factors are also highlighted.

False-Positive and False-Negative Contrast-enhanced Mammograms: Pitfalls and Strategies to Improve Cancer Detection.

Contrast-enhanced mammography (CEM) is a relatively new breast imaging modality that uses intravenous contrast material to increase detection of breast cancer. CEM combines the structural information of conventional mammography with the functional information of tumor neovascularity. Initial studies have demonstrated that CEM and MRI perform with similar accuracies, with CEM having a slightly higher specificity (fewer false positives), although larger studies are needed. There are various reasons for false positives and false negatives at CEM. False positives at CEM can be caused by benign lesions with vascularity, including benign tumors, infection or inflammation, benign lesions in the skin, and imaging artifacts. False negatives at CEM can be attributed to incomplete or inadequate visualization of lesions, marked background parenchymal enhancement (BPE) obscuring cancer, lack of lesion contrast enhancement due to technical issues or less-vascular cancers, artifacts, and errors of lesion perception or characterization. When possible, real-time interpretation of CEM studies is ideal. If additional views are necessary, they may be obtained while contrast material is still in the breast parenchyma. Until recently, a limitation of CEM was the lack of CEM-guided biopsy capability. However, in 2020, the U.S. Food and Drug Administration cleared two devices to support CEM-guided biopsy using a stereotactic biopsy technique. The authors review various causes of false-positive and false-negative contrast-enhanced mammograms and discuss strategies to reduce these diagnostic errors to improve cancer detection while mitigating unnecessary additional imaging and procedures. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.

Imaging Characteristics of and Multidisciplinary Management Considerations for Atypical Ductal Hyperplasia and Flat Epithelial Atypia: Review of Current Literature.

High-risk lesions of the breast are frequently encountered in percutaneous biopsy specimens. While benign, these lesions have historically undergone surgical excision due to their potential to be upgraded to malignancy. However, there is emerging evidence that a tailored management approach should be considered to reduce overtreatment of these lesions. Flat epithelial atypia (FEA) and atypical ductal hyperplasia (ADH) are two of the most commonly encountered high-risk lesions. FEA has been shown to have a relatively low rate of progression to malignancy, and some guidelines are now recommending observation over routine excision in select cases. Selective observation may be reasonable in cases where the target lesion is small and completely removed at biopsy and when there are no underlying risk factors, such as a history of breast cancer or genetic mutation or concurrent ADH. ADH has the highest potential upgrade rate to malignancy of all the high-risk lesions. Most society guidelines continue to recommend surgical excision of this lesion. More recently, some literature suggests that ADH lesions that appear completely removed at biopsy, involve limited foci (less than two or three) with no necrosis or significant atypia, manifest as a small group of mammographic calcifications, or demonstrate no enhancement at MRI may be reasonable for observation. Ultimately, management of all high-risk lesions must be based on a multidisciplinary approach that considers all patient, radiologic, clinical, and histopathologic factors. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.

Risk factors for SARS-CoV-2 infection and transmission in households with children with asthma and allergy: A prospective surveillance study.

BACKGROUND: Whether children and people with asthma and allergic diseases are at increased risk for severe acute respiratory syndrome virus 2 (SARS-CoV-2) infection is unknown. OBJECTIVE: Our aims were to determine the incidence of SARS-CoV-2 infection in households with children and to also determine whether self-reported asthma and/or other allergic diseases are associated with infection and household transmission. METHODS: For 6 months, biweekly nasal swabs and weekly surveys were conducted within 1394 households (N = 4142 participants) to identify incident SARS-CoV-2 infections from May 2020 to February 2021, which was the pandemic period largely before a vaccine and before the emergence of SARS-CoV-2 variants. Participant and household infection and household transmission probabilities were calculated by using time-to-event analyses, and factors associated with infection and transmission risk were determined by using regression analyses. RESULTS: In all, 147 households (261 participants) tested positive for SARS-CoV-2. The household SARS-CoV-2 infection probability was 25.8%; the participant infection probability was similar for children (14.0% [95% CI = 8.0%-19.6%]), teenagers (12.1% [95% CI = 8.2%-15.9%]), and adults (14.0% [95% CI = 9.5%-18.4%]). Infections were symptomatic in 24.5% of children, 41.2% of teenagers, and 62.5% of adults. Self-reported doctor-diagnosed asthma was not a risk factor for infection (adjusted hazard ratio [aHR] = 1.04 [95% CI = 0.73-1.46]), nor was upper respiratory allergy or eczema. Self-reported doctor-diagnosed food allergy was associated with lower infection risk (aHR = 0.50 [95% CI = 0.32-0.81]); higher body mass index was associated with increased infection risk (aHR per 10-point increase = 1.09 [95% CI = 1.03-1.15]). The household secondary attack rate was 57.7%. Asthma was not associated with household transmission, but transmission was lower in households with food allergy (adjusted odds ratio = 0.43 [95% CI = 0.19-0.96]; P = .04). CONCLUSION: Asthma does not increase the risk of SARS-CoV-2 infection. Food allergy is associated with lower infection risk, whereas body mass index is associated with increased infection risk. Understanding how these factors modify infection risk may offer new avenues for preventing infection.

Evaluating educational interventions to increase breast density awareness among Latinas: A randomized trial in a Federally Qualified Health Center.

BACKGROUND: The objective of this randomized trial was to evaluate the short-term effect of bilingual written and interpersonal education regarding mammographic breast density (MBD). METHODS: Latinas aged 40 to 74 years who were presenting for screening mammography were recruited and randomized 1:1:1 to receive a letter with their mammogram and MBD results (usual care [UC]), a letter plus a brochure (enhanced care [ENH]), or a letter plus a brochure and telephonic promotora education (interpersonal care [INT]). Surveys were administered at enrollment (T0 ) and 2 weeks to 6 months after intervention delivery (T1 ). Differences were assessed with χ2 , Kruskal-Wallis, and McNemar tests and pairwise comparisons as appropriate. INT metrics and audio recordings were analyzed with descriptive statistics and qualitative content analysis. RESULTS: Between October 2016 and October 2019, 943 of 1108 Latina participants (85%) completed both surveys. At T1 , INT participants were more likely (P < .001) to report seeing their MBD results in the letter (70.2%) than UC (53.1%) or ENH participants (55.1%). The percentage of INT women who reported speaking with a provider about MBD (29.0%) was significantly greater (P < .001) than the percentage of UC (14.7%) or ENH participants (15.6%). All groups saw significant (P < .001) but nondifferential improvements in their knowledge of MBD as a masking and risk factor. In the INT group, the promotora delivered education to 77.1% of the 446 participants randomized to INT and answered questions at 28.3% of the encounters for an average of $4.70 per participant. CONCLUSIONS: Among Latinas in a low-resource setting, MBD knowledge may increase with written or interpersonal education, but with modest investment, interpersonal education may better improve MBD awareness and prompt patient-provider discussions.

Association of breast cancer risk, density, and stiffness: global tissue stiffness on breast MR elastography (MRE).

PURPOSE: Quantify in vivo biomechanical tissue properties in various breast densities and in average risk and high-risk women using Magnetic Resonance Imaging (MRI)/MRE and examine the association between breast biomechanical properties and cancer risk based on patient demographics and clinical data. METHODS: Patients with average risk or high-risk of breast cancer underwent 3.0 T breast MR imaging and elastography. Breast parenchymal enhancement (BPE), density (from most recent mammogram), stiffness, elasticity, and viscosity were recorded. Within each breast density group (non-dense versus dense), stiffness, elasticity, and viscosity were compared across risk groups (average versus high). Separately for stiffness, elasticity, and viscosity, a multivariable logistic regression model was used to evaluate whether the MRE parameter predicted risk status after controlling for clinical factors. RESULTS: 50 average risk and 86 high-risk patients were included. Risk groups were similar in age, density, and menopausal status. Among patients with dense breasts, mean stiffness, elasticity, and viscosity were significantly higher in high-risk patients (N = 55) compared to average risk patients (N = 34; all p < 0.001). Stiffness remained a significant predictor of risk status (OR = 4.26, 95% CI [1.96, 9.25]) even after controlling for breast density, BPE, age, and menopausal status. Similar results were seen for elasticity and viscosity. CONCLUSION: A structurally based, quantitative biomarker of tissue stiffness obtained from MRE is associated with differences in breast cancer risk in dense breasts. Tissue stiffness could provide a novel prognostic marker to help identify high-risk women with dense breasts who would benefit from increased surveillance and/or risk reduction measures.

Accuracy of Posttreatment Imaging for Evaluation of Residual in Breast Disease After Neoadjuvant Endocrine Therapy.

BACKGROUND: Neoadjuvant endocrine therapy (NET) can help downstage certain breast cancers prior to surgical resection. This study measured the accuracy of conventional mammography (MMG), ultrasound (US), magnetic resonance imaging (MRI), and contrast-enhanced mammography (CEM) for assessing breast tumor size in response to NET. PATIENTS AND METHODS: Patients who underwent surgery after NET from 2013 to 2021 were identified. The maximal dimension of residual tumor on imaging was compared with the maximal dimension on final pathology. Lin's concordance correlation coefficient (rc) and Spearman's rank correlation coefficient (r) were used to assess agreement. RESULTS: In total, 119 patients with invasive breast cancer underwent NET, posttreatment imaging, and surgery. Tumor size reported on posttreatment CEM correlated with size on final pathology to within 1 cm in n = 42 (58%) of patients, equivalent to the accuracy of MRI (n = 35, 58%). Size was accurately predicted by US in 54% and in 48% of MMG. Posttreatment imaging tumor size was moderately correlated with final tumor size on pathology CEM (r = 0.49; rc = 0.38), MRI (r = 0.52; rc = 0.45), and US (r = 0.41; rc = 0.28). MMG was weakly correlated (r = 0.21; rc = 0.16). Similar findings were shown in subgroup analysis; in those who received all four post-NET imaging, CEM and MRI again performed comparably, with r = 0.36 and 0.41, respectively, US (r = 0.43) and MMG (r = 0.28). CONCLUSIONS: Compared with mammography and US, CEM and MRI had higher accuracy in estimating final tumor size for breast cancers treated with NET. Contrast-enhanced imaging is a helpful adjunct when response to preoperative therapy will impact clinical management.

Contrast-Enhanced Mammography for Newly Diagnosed Breast Cancer in Women With Breast Augmentation: Preliminary Findings.

In 17 women with newly diagnosed breast cancer who underwent contrast-enhanced mammography (CEM) and MRI, both modalities were found to be concordant for the index cancer. In six of the 17 women, CEM showed an additional lesion that was confirmed by MRI. Of these six additional lesions, three were multifocal, one was multicentric, and two were contralateral; two of the six were malignant. MRI did not identify any additional cancers that were not identified on CEM. CEM may have a role in women with breast augmentation and either a contraindication or limited access to MRI.

Contrast-enhanced Mammography: How Does It Work?

Contrast-enhanced mammography (CEM) is an imaging technique that uses iodinated contrast medium to improve visualization of breast lesions and assessment of tumor neovascularity. Through modifications in x-ray energy, high- and low-energy images of the breast are combined to highlight areas of contrast medium pooling. The use of contrast material introduces different workflows, artifacts, and risks related to the contrast medium dose. In addition, the need to acquire multiple images in each view introduces different workflows, artifacts, and risks associated with the radiation dose. Although CEM and conventional mammography share many underlying principles, it is important to understand how these two mammographic examinations differ and the mechanisms that facilitate image contrast at CEM. ©RSNA, 2021.

Contrast-Enhanced Spectral Mammography is Comparable to MRI in the Assessment of Residual Breast Cancer Following Neoadjuvant Systemic Therapy.

PURPOSE: To evaluate the performance of contrast-enhanced spectral mammography (CESM) compared to MRI in the assessment of tumor response in breast cancer patients undergoing neoadjuvant systemic therapy (NST). METHODS: The institutional review board approved this study. From September 2014 to June 2017, we identified patients with pathologically confirmed invasive breast cancer who underwent NST. All patients had both CESM and MRI performed pre- and post-NST with pathological assessment after surgical management. Size of residual malignancy on post-NST CESM and MRI was compared with surgical pathology. Lin concordance and Pearson correlation coefficient were used to assess agreement. Bland-Altman plots were used to visualize the differences between tumor size on imaging and pathology. RESULTS: Sixty-five patients were identified. Mean age was 52.7 (range 30-76) years. Type of NST included chemotherapy in 53 (82%) and endocrine therapy in 12 (18%). Mean tumor size after NST was 14.6 (range 0-105) mm for CESM and 14.2 mm (range 0-75 mm) for MRI compared with 19.6 (range 0-100) mm on final surgical pathology. Equivalence tests demonstrated that mean tumor size measured by CESM (p = 0.009) or by MRI (p = 0.01) was equivalent to the mean tumor size measured by pathology within - 1 and 1-cm range. Comparing CESM versus MRI for assessment of complete response, the sensitivity was 95% versus 95%, specificity 66.7% versus 68.9%, positive predictive value 55.9% versus 57.6%, and negative predictive value 96.7% versus 96.9% respectively. CONCLUSIONS: CESM was comparable to MRI in assessing residual malignancy after completion of NST.

Initial Experience of Tomosynthesis-Guided Vacuum-Assisted Biopsies of Tomosynthesis-Detected (2D Mammography and Ultrasound Occult) Architectural Distortions.

OBJECTIVE: As experience and aptitude in digital breast tomosynthesis (DBT) have increased, radiologists are seeing more areas of architectural distortion (AD) on DBT images compared with standard 2D mammograms. The purpose of this study is to report our experience using tomosynthesis-guided vacuum-assisted biopsies (VABs) for ADs that were occult at 2D mammography and ultrasound and to analyze the positive predictive value for malignancy. MATERIALS AND METHODS: We performed a retrospective review of 34 DBT-detected ADs that were occult at mammography and ultrasound. RESULTS: We found a positive predictive value of 26% (nine malignancies in 34 lesions). Eight of the malignancies were invasive and one was ductal carcinoma in situ. The invasive cancers were grade 1 (4/8; 50%), grade 2 (2/8; 25%), or grade 3 (1/8; 13%); information about one invasive cancer was not available. The mean size of the invasive cancers at pathologic examination was 7.5 mm (range, 6-30 mm). CONCLUSION: Tomosynthesis-guided VAB is a feasible method to sample ADs that are occult at 2D mammography and ultrasound. Tomosynthesis-guided VAB is a minimally invasive method that detected a significant number of carcinomas, most of which were grade 1 cancers. Further studies are needed.

Nipple Discharge: Imaging Variability Among U.S. Radiologists.

OBJECTIVE: The purpose of this study was to assess radiologists' choice of imaging modality for the evaluation of clinical symptoms of physiologic nipple discharge (e.g., bilateral discharge, multiple-duct orifices, and yellow, green, or white color) and pathologic nipple discharge (e.g., unilateral discharge, single-duct orifices, spontaneous and serous discharge, and clear or bloodstained color). MATERIALS AND METHODS: An online survey was sent to lead interpreting physicians at mammography facilities accredited by the American College of Radiology (ACR). Statistical analysis was performed using chi-square tests for frequency data and multinomial logistic regression. RESULTS: A total of 849 responses to 8170 distributed surveys were received, for a response rate of 10.4%. For the workup of physiologic nipple discharge, 30% of respondents recommended screening mammography (SM); 24%, diagnostic mammography (DM) only; and 46%, both DM and targeted ultrasound (US) (DM plus US). For the workup of physiologic nipple discharge, practitioners in nonacademic settings and those who read breast images during less than 50% of their practice were significantly more likely to recommend DM (with or without US), compared with SM (the standard recommended by the ACR). Those reading breast images less than 50% of the time were also more likely to recommend MRI after conventional imaging revealed negative results. For the workup of pathologic nipple discharge, 91.0% of respondents recommended DM plus US; 8.5%, DM only; and fewer than 1.0%, SM. Nonacademic providers and those who read breast images less than 50% of the time were significantly less likely to recommend DM plus US (the standard recommended by the ACR), compared with DM only. CONCLUSION: The present study shows variability in imaging modality selection among U.S. radiologists handling the imaging workflow for benign and pathologic nipple discharge. Radiologists do not uniformly follow ACR practice guidelines, which potentially leads to unnecessary workups and extra health care costs.

The Future of Contrast-Enhanced Mammography.

OBJECTIVE: The purpose of this article is to discuss facilitators of and barriers to future implementation of contrast-enhanced mammography (CEM) in the United States. CONCLUSION: CEM provides low-energy 2D mammographic images analogous to digital mammography and contrast-enhanced recombined images that allow assessment of neovascularity similar to that offered by MRI. The utilization of CEM in the United States is currently low but could increase rapidly given the many potential indications for its clinical use.

Variability of Postsurgical Imaging Surveillance of Breast Cancer Patients: A Nationwide Survey Study.

OBJECTIVE: Because of observed clinical variance and the discretion of referring physicians and radiologists in patient follow-up, the purpose of this study was to conduct a survey to explore whether broad discrepancy exists in imaging protocols used for postsurgical surveillance. SUBJECTS AND METHODS: An online survey was created to assess radiologists' use of diagnostic versus screening mammography for women with a personal history of breast cancer and determine whether the choice of protocol was associated with practice characteristics (setting, region, and reader type). RESULTS: Of 8170 surveys sent, 849 (10%) completed responses were returned. Seventy-nine percent of respondents recommended initial diagnostic mammography after lumpectomy (65% at 6 months, 14% at 12 months); 49% recommended diagnostic surveillance for up to 2 years before a return to screening mammography; and 33% continued diagnostic surveillance for 2-5 years before returning to screening. For imaging after mastectomy, 57% of respondents recommended diagnostic mammography of the unaffected breast. Among the 57%, however, 37% recommended diagnostic screening for only the first postmastectomy follow-up evaluation, and the other 20% permanently designated patients for diagnostic mammography after mastectomy. CONCLUSION: The optimal surveillance mammography regimen must be better defined. This preliminary study showed variability in diagnostic versus screening surveillance mammography for women with a history of breast cancer. Future studies should evaluate why these variations occur and how to standardize recommendations to tailor personalized imaging.

Breast MRI phenotype and background parenchymal enhancement may predict tumor response to neoadjuvant endocrine therapy.

Neoadjuvant endocrine therapy (NET) is increasingly used for the treatment of estrogen receptor positive, HER2 negative breast cancer. We evaluated whether MRI phenotype and background parenchymal enhancement (BPE) can predict response to NET. Patients with localized breast cancer treated with NET and had a pre-treatment breast MRI were identified. Baseline MRI phenotype and BPE was interpreted by a single radiologist blinded to the results of systemic therapy. Response was defined as stable disease or reduction in tumor size on clinical and/or ultrasound examination. Of the 21 patients identified, 17 were responders; all patients with minimal/mild BPE had a response compared to 5/9 (56%) patients with moderate/marked BPE (P = 0.02). All four nonresponders had moderate/marked BPE as compared to 5/17 (29%) responders (P = 0.02). This pilot study suggests that minimal/mild BPE may be predictive of a positive response to NET. A higher degree of background enhancement was significantly predictive of negative response to NET.

The mental health of adolescents and pre-adolescents living with inherited arrhythmia syndromes: a systematic review of the literature.

Potentially fatal arrhythmias add to the mental health challenges of adolescence. This systematic review sought to summarise current knowledge regarding the mental health of adolescents and pre-adolescents diagnosed with inherited arrhythmia syndromes. Searches combining psychological problems with inherited cardiac arrhythmia diagnoses identified 16 studies with paediatric (<18 years) inherited arrhythmia patients. All studies were cross-sectional; 8/16 required an implantable cardioverter defibrillator. Methods were quantitative (n=11), qualitative (n=4), or mixed (n=1), with 14-100% of participants having an inherited arrhythmia syndrome. Mean/median age in 13/16 studies was 12-16 years. Patients and parents reported lower quality of life, particularly in relation to physical function, social relationships, restriction of peer activities, bodily pain, and mental and emotional health. Self-perceptions and behaviour were similar to healthy populations. Rates of anxiety and depression (15-33% of these patients) were not increased in these studies where patients were assessed 2+ years after diagnosis. Higher mental health risk occurred among patients who have a diagnosed sibling, those with cardiomyopathy, and those who report decreased quality of life. Mental health research among youth with inherited arrhythmias is extremely limited and of low quality. Data, primarily from patients 2-4 years after diagnosis or treatment with an implantable cardioverter defibrillator, indicate that quality of life may be decreased and 15-33% experience mental health issues. Future research is required to examine the mental health and quality of life of paediatric patients with inherited arrhythmia syndromes, whether or not they have an implantable cardioverter defibrillator, from time of diagnosis.