Pregnancy in Patients with the Fontan Operation.

Improved survival rates for patients with a Fontan circulation has allowed more women with this complex cardiac physiology to contemplate pregnancy. However, pregnancy in women with a Fontan circulation is associated with a high risk of adverse maternal and fetal outcomes, high rates of miscarriage and preterm delivery. Factors associated with a successful pregnancy outcome are: younger age, normal body weight, absence of significant functional limitation, no Fontan-related complications, and well-functioning single ventricle physiology. Appropriate care with timely preconception counselling and regular, frequent clinical reviews by a multidisciplinary team based at a tertiary centre, improves the chance of a successful pregnancy. Empowerment of patients with education on their specific congenital cardiac condition and its projected trajectory, helps them make informed choices regarding their health, reproductive choices and assists them to achieve their life goals.

Male-female differences in thoracic aortic diameters at presentation of acute type A aortic dissection.

Background: Acute type A aortic dissection (ATAAD) is a highly lethal event, associated with aortic dilatation. It is not well known if patient height, weight or sex impact the thoracic aortic diameter (TAA) at ATAAD. The study aim was to identify male-female differences in TAA at ATAAD presentation. Methods: This retrospective cross-sectional study analysed all adult patients who presented with ATAAD between 2007 and 2017 in two tertiary care centres and underwent contrast enhanced computed tomography (CTA) before surgery. Absolute aortic diameters were measured at the sinus of Valsalva (SoV), ascending (AA) and descending thoracic aorta (DA) using double oblique reconstruction, and indexed for body surface area (ASI) and height (AHI). Z-scores were calculated using the Campens formula. Results: In total, 59 % (181/308) of ATAAD patients had CT-scans eligible for measurements, with 82 female and 99 male patients. Females were significantly older than males (65.5 ± 12.4 years versus 60.3 ± 2.3, p = 0.024). Female patients had larger absolute AA diameters than male patients (51.0 mm [47.0-57.0] versus 49.0 mm [45.0-53.0], p = 0.023), and larger ASI and AHI at all three levels. Z-scores for the SoV and AA were significantly higher for female patients (2.99 ± 1.66 versus 1.34 ± 1.77, p < 0.001 and 5.27 [4.38-6.26] versus 4.06 [3.14-5.02], p < 0.001). After adjustment for important clinical factors, female sex remained associated with greater maximal TAA (p = 0.019). Conclusion: Female ATAAD patients had larger absolute ascending aortic diameters than males, implying a distinct timing in disease presentation or selection bias. Translational studies on the aortic wall and studies on growth patterns should further elucidate these sex differences.

Handling End-of-Life Situations in Small Animal Practice: What Strategies do Veterinarians Contemplate During their Decision-Making Process?

This study researched end-of-life (EoL) decision-making processes in small animal practices in the Netherlands, focusing on strategies veterinarians contemplate during this process. Fourteen veterinarians were interviewed about animal end-of-life decision-making. The results of these interviews show that the decision-making process consists of three steps. The first step is to assess the animal's health and welfare. During the second step, veterinarians consider the position of the owner. Based on steps 1 and 2, veterinarians decide in step 3 whether their advice is to a) euthanize or b) contemplate one or more strategies to come to a decision or potentially alter the decision. These results can support members of the veterinary profession to reflect on their decision-making process. If veterinarians know what strategies their peers use to deal with EoL situations, this can help to reduce the stress they experience in such situations. In addition, veterinarians may find inspiration for new strategies in the study results. For the veterinary profession itself, the current results can be used as a starting point for describing best practices for EoL decision-making in small animal practice.

Reproductive health in adults with congenital heart disease: a review on fertility, sexual health, assisted reproductive technology and contraception.

INTRODUCTION: Due to the improved survival in individuals with congenital heart disease (CHD), considering their reproductive health has become more important. Currently, this topic is still underexplored. AREAS COVERED: We discuss fertility, sexuality, assisted reproductive technology (ART), and contraception in adults with CHD. EXPERT OPINION: Timely counseling regarding fertility, sexuality, pregnancy, and contraception is necessary, preferably during teenage years. Due to a lack of data, whether or not to perform ART in adults with CHD is almost always based on expert opinion and follow-up in an expert center is recommended. Future research is necessary to fill the gaps in knowledge on the risks and frequency of complications of ART in adults with CHD, but also to be able to differentiate the relative risks in the different types of CHD. Only then will we be able to counsel adults with CHD correctly and not unjustly deprive someone of a chance of pregnancy.

Quantification of a shelter cat population: Trends in intake, length of stay and outcome data of cats in seven Dutch shelters between 2006 and 2021.

Shelter metrics can be used by shelters for self-assessment to optimise the health of their animal population and to identify risk factors for disease outbreaks. However, there is a need for a wider scope of these shelter metrics, as evidenced by the interest from shelters in the benchmarking of shelter progress and the development of national best practices. For the first time, Dutch shelter data were used retrospectively to signal trends using potentially reliable metrics for the analysis of shelter data. The aims of this study were to apply relevant metrics describing the different phases of shelter management for shelter cats (i.e., intake, stay and outcome) and a retrospective analysis of shelter data over the period between 2006 and 2021. Seven of the approximately 120 Dutch animal shelters participated in this study. Quantitative data on the intake of more than 74,000 shelter cats (e.g., stray cats, cats surrendered by their owners and cats obtained from other sources) and their outcomes (i.e., cats rehomed, returned to their owners, deceased, or otherwise lost) have been analysed. Metrics such as rehoming rate, return to owner rate, rates for mortality and euthanasia, length of stay and risk-based live release rate were determined. The main findings of the study during this 16-year period were that, over time, the number of cats per 1000 residents admitted to Dutch shelters was reduced by 39%, the number of feline euthanasia cases decreased by approximately 50%, the length of stay showed a reducing trend, while the return to owner and the risk-based live release rate increased. The shelter metrics examined in this study could be helpful in monitoring and evaluating the management, consequent health, and well-being of cats in shelters and eventually measuring progress of shelters both in the Netherlands and at a European level.

Executive functioning of patients with congenital heart disease: 45 years after surgery.

BACKGROUND: Nowadays, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, long-term impact on neurodevelopment and executive functioning in adults with CHD are not completely understood. PURPOSE: To investigate the self- and informant-reported executive functioning in adults with CHD operated in childhood. MATERIAL AND METHODS: Longitudinal study of a cohort of patients (n = 194, median age: 49.9 [46.1-53.8]) who were operated in childhood (< 15 years old) between 1968 and 1980 (median follow-up time: 45 [40-53] years) for one of the following diagnoses: atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis (PS), tetralogy of Fallot (ToF) or transposition of the great arteries (TGA). Behavior Rating Inventory of Executive Function-Adult version (BRIEF-A) questionnaire was used to assess self- and informant-reported executive functioning. RESULTS: 40-53 years after surgery, the CHD group did show significantly better executive functioning compared to the norm data. No significant difference was found between mild CHD (ASD, VSD and PS) and moderate/severe CHD (ToF and TGA). Higher education, NYHA class 1 and better exercise capacity were associated with better self-reported executive functioning, whereas females or patients taking psychiatric or cardiac medications reported worse executive functioning. CONCLUSIONS: Our findings suggest favorable outcomes (comparable to normative data) regarding executive functioning in adults with CHD, both self- and informant-reported. However, further study is warranted to explore more in detail the different cognitive domains of executive functioning in these patients.

Prevalence and development of aortic dilation and dissection in women with Turner syndrome: a systematic review and meta-analysis.

OBJECTIVES: Women with Turner syndrome (TS) have an increased risk of aortic disease, reducing life-expectancy. This study aimed to systematically review the prevalence of thoracic aortic dilatation, aortic dimensions and growth, and the incidence of aortic dissection. METHODS: A systematic literature search was conducted up to July 2022. Observational studies with an adult TS population were included, and studies including children aged <15 years old or specific TS populations were excluded. RESULTS: In total 21 studies were included. The pooled prevalence of ascending aortic dilatation was 23% (95% CI 19-26) at a mean pooled age of 29 years (95% CI 26-32), while the incidence of aortic dissection was 164 per 100.000 patient-years (95% CI 95-284). Three reporting studies showed aortic growth over time to be limited. Risk factors for aortic dilation or dissection were older age, bicuspid aortic valve, aortic coarctation, and hypertension. CONCLUSION: In adult TS women, ascending aortic dilatation is common and the hazard of aortic dissection increased compared to the general population, whereas aortic growth is limited. Conventional risk markers do not explain all aortic dissection cases; therefore, new imaging parameters and blood biomarkers are needed to improve prediction, allowing for patient-tailored follow-up and surgical decision-making.

Daily life and psychosocial functioning of adults with congenital heart disease: a 40-53 years after surgery follow-up study.

INTRODUCTION: Nowadays, more than 90% of patients with congenital heart disease (CHD) reach adulthood. However, knowledge about their psychosocial functioning is limited. METHODS: Longitudinal cohort study of patients (n = 204, mean age: 50 years, 46.1% female) who were operated during childhood (< 15 years) between 1968 and 1980 for one of the following diagnoses: atrial septal defect, ventricular septal defect, pulmonary stenosis, tetralogy of Fallot or transposition of the great arteries. Psychosocial functioning was measured every 10 years, using standardized and validated questionnaires. Results were compared with the general Dutch population and over time. RESULTS: After a median follow-up of 45 [40-53] years adults with CHD had a significantly lower educational level, occupation level and employment rate, but better health-related quality of life and emotional functioning compared with normative data. Patients with moderate/severe defects reported significantly more self-perceived physical restrictions and lack of physical strength due to their CHD. Compared to 2011, in 2021 patients considered their CHD as more severe and they felt more often disadvantaged. CONCLUSIONS: Overall, despite a lower education, occupation level and employment rate, our sample of patients with CHD had a positive perception of their life and  their psychosocial functioning was even better than the norm. Although the quality of life was very good, their view on their disease was more pessimistic than 10 years ago, especially for patients with moderate/severe CHD.

The impact of the multidisciplinary Endocarditis Team on the management of infective endocarditis.

BACKGROUND: In their latest guidelines for infective endocarditis (IE) (2015), the European Society of Cardiology (ESC) introduced the implementation of the Endocarditis Team (ET) to facilitate the management of IE. This study presents our experiences and the diagnostic and therapeutic impact of the ET on the management of IE. METHODS: From 2016-2020, data of all patients with suspected IE referred to the ET were prospectively collected. The final diagnosis was defined by the ET as either rejected, possible or definite IE. Diagnostic impact was scored as any change in initial diagnosis, the frequency of additional diagnostic tests advised by the ET and any change in diagnosis after these tests. Therapeutic impact was scored as any change in antibiotic therapy or change from conservative to invasive therapy or vice versa. RESULTS: A total of 321 patients (median age 67 [55-77] years, 71% male) were enrolled. The final diagnosis was rejected IE in 47 (15%), possible IE in 34 (11%) and definite IE in 240 (75%) patients. A change of initial diagnosis was seen in 53/321(17%) patients. Additional microbiological tests were advised in 69/321 (21%) patients, and additional imaging tests in 136/321 (42%) patients, which resulted in subsequent change in diagnosis in 23/321 (7%) patients. Any change in antibiotic treatment was advised in 135/321 (42%) patients, and change from initial conservative to additional surgical treatment in 15/321 (5%) patients. CONCLUSION: The ET had a clear impact on the therapeutic policy for patients with suspected IE and is useful in the management of this life-threatening disease. Broad implementation is warranted.

Mitral prolapsing volume is associated with increased cardiac dimensions in patients with mitral annular disjunction.

INTRODUCTION: In patients with mitral annular disjunction (MAD), it can be difficult to assess the severity of mitral regurgitation (MR), as they present with a prolapsing volume (i.e. volume resulting from mitral valve prolapse, blood volume shift) rather than a regurgitant jet. The influence of the mitral prolapsing volume (MPV) on cardiac dimensions is unknown. We hypothesised that the severity of MR is underestimated in these patients. Our aim was to measure MPV and to investigate its influence on cardiac dimensions in patients with MAD. METHODS: We retrospectively included 131 consecutive patients with MAD from our institution's echocardiographic database. Transthoracic echocardiography was used to assess MPV. Additionally, we established a control group of 617 consecutive patients with degenerative mitral valve disease and performed propensity score matching. RESULTS: Median MPV in the MAD group was 12 ml. MPV was an independent predictor for left ventricular end-diastolic (LVEDD) and end-systolic diameter (LVESD) and left atrial volume (all p < 0.001). In patients with large prolapsing volumes (> 15 ml), LVEDD (56 ± 6 mm vs 51 ± 6 mm, p < 0.001), LVESD [38 mm (34-41) vs 34 mm (31-39), p < 0.01] and left atrial volume [105 ml (86-159) vs 101 ml (66-123), p = 0.04] were significantly increased compared to matched patients with degenerative mitral valve disease and similarly assessed severity of MR. CONCLUSION: Due to a volume shift based on the MPV rather than an actual regurgitant jet, MR severity cannot be assessed adequately in MAD patients. Increased MPV induces ventricular and atrial enlargement. These findings warrant future studies to focus on MPV as an additional parameter for assessment of the severity of MR in MAD patients.

Reporting of sex-specific outcomes in trials of interventions for cardiovascular disease: Has there been progress?

In the past decade, the leading international cardiology societies have released statements that emphasize the importance of sex-specific reporting of the findings of clinical trials in cardiovascular research. To find out whether this has led to improvement, we compared sex-specific reporting of efficacy and safety outcomes for trials of cardiovascular drug interventions presented at the major clinical trials sessions of the European Society of Cardiology (ESC), American Heart Association (AHA) and the American College of Cardiology (ACC) before and after publication of these statements. We found that sex-specific efficacy and safety outcomes of the most influential cardiovascular intervention trials are still not systematically presented.

Implementation of the 2015 European Society of Cardiology guidelines for the management of infective endocarditis in the Netherlands.

Because the occurrence of infective endocarditis (IE) continues to be associated with high mortality, a working group was created by the Dutch Society of Cardiology to examine how the most recent European Society of Cardiology (ESC) guidelines for IE management could be implemented most effectively in the Netherlands. In order to investigate current Dutch IE practices, the working group conducted a country-wide survey. Based on the results obtained, it was concluded that most ESC recommendations could be endorsed, albeit with some adjustments. For instance, the suggested pre-operative screening and treatment of nasal carriers of Staphylococcus aureus as formulated in the ESC guideline was found to be dissimilar to current Dutch practice, and was therefore made less restrictive. The recently adapted ESC diagnostic criteria for IE were endorsed, while the practical employment of the relevant diagnostic techniques was simplified in an adapted flowchart. In addition, the presence of a multidisciplinary, so-called 'endocarditis team' in tertiary centres was proposed as a quality indicator. An adapted flowchart specifically tailored to Dutch practice for microbiological diagnostic purposes was constructed. Lastly, the working group recommended the Stichting Werkgroep Antibioticabeleid (SWAB; Dutch Working Party on Antibiotic Policy) guidelines for IE treatment instead of the antibiotic regimens proposed by the ESC.

Infective endocarditis in the Netherlands: current epidemiological profile and mortality : An analysis based on partial ESC EORP collected data.

INTRODUCTION: Infective endocarditis (IE) is associated with a high in-hospital and long term mortality. Although progress has been made in diagnostic approach and management of IE, morbidity and mortality of IE remain high. In the latest European guidelines, the importance of the multi-modality imaging in diagnosis and follow up of IE is emphasized. AIM: The aim was to provide information regarding mortality and adverse events of IE, to determine IE characteristics and to assess current use of imaging in the diagnostic workup of IE. METHODS: This is a prospective observational cohort study. We used data from the EURO-ENDO registry. Seven hospitals in the Netherlands have participated and included patients with IE between April 2016 and April 2018. RESULTS: A total of 139 IE patients were included. Prosthetic valve endocarditis constituted 32.4% of the cases, cardiac device related IE 7.2% and aortic root prosthesis IE 3.6%. In-hospital mortality was 14.4% (20 patients) and one-year mortality was 21.6% (30 patients). The incidence of embolic events under treatment was 16.5%, while congestive heart failure or cardiogenic shock occurred in 15.1% of the patients. Transthoracic and transoesophageal echocardiography were performed most frequently (97.8%; 81.3%) and within 3 days after IE suspicion, followed by 18F­fluorodeoxyglucose positron emission tomography/computed tomography (45.3%) within 6 days and multi-slice computed tomography (42.4%) within 7 days. CONCLUSION: We observed a high percentage of prosthetic valve endocarditis, rapid and extensive use of imaging and a relatively low in-hospital and one-year mortality of IE in the Netherlands. Limitations include possible selection bias.

Quantification of the health-status of the Dutch Labrador retriever population.

Health issues in purebred dogs are currently considered one of the biggest problems in companion animal health. The Labrador retriever (LR) is one of the most popular dog breeds. The aim of this study was to quantify LR breed health in comparison with mixed-breed dogs (MB), by using four different data sources: a veterinary practice management system (appr. 35,000 unique individuals LR + MB), data from two animal insurance companies (appr. 15,500 and 4500 individuals respectively), and a histopathological laboratory (appr. 4000 individuals). After extensive recoding of the data, health parameters utilised to quantify breed health were longevity, frequency of practice visits and insurance expense claims, and diagnostic codes. A Kaplan-Meier univariate and multivariable Cox proportional hazard model were used to evaluate longevity. A negative binomial model was used to analyse the frequency of visits, claims, and diagnostic codes in both sets of insurance data. Logistic regression was used to look into the categorical diagnostic codes in the laboratory data. The median lifespan of the LR was similar (12 years, practice data) or longer (10 versus 8 years, insurance data) than MB for individuals with a known birth and death date. When including censored individuals, survival time in the LR was comparable to MB individuals up to 10 years of age. Above 10 years of age, the LR lived a similar length as MB with a medium to large body size, but shorter than all MB. The LR visited the veterinary practice more often (risk ratio (RR) 1.2, 95% confidence interval 1.2-1.3), and also showed a higher frequency of insurance expense claims (RR 2.2 (2.1-2.3) and RR 1.2 (1.1-1.3) respectively for the two insurance data sets). The largest difference in organ systems between the LR and MB in insurance claims was related to ears (RR 5.3 (4.8-5.8) and RR 2.6 (2.3-3.1)), followed by airways (RR 2.6 (2.4-2.8)), tendons & muscles (RR 2.4 (2.2-2.6) and RR 1.4 (1.1-1.7)), and joints (RR 1.7 (1.3-2.1)), without a difference in median age at diagnosis. The data from the histopathological laboratory suggested a higher disease burden related to oncology for the LR compared to MB (OR 1.2, 95% CI 1.0-1.3). Oncological diagnoses were made at a younger age in the LR (8.8 versus 9.4 years). The disease burden was significantly higher for the LR than MB, but these results may suffer from substantial bias such as selection bias towards the database, and different behaviour of LR versus MB owners with regards to veterinary care. In the future, longer term population data can corroborate these results.

Influence of age and sex on left ventricular diastolic strain analysis.

Speckle tracking analysis (STE) of the left ventricle offers a new method to assess left ventricular (LV) diastolic function. LV diastolic strain measurements offer a non-invasive, global and direct measure of LV diastolic function. However, there is little data on normal values and the influence of anthropomorphic factors which is crucial in clinical practice for new techniques. The aims of this study were to formulate reference values for LV diastolic strain rate, elucidate effects of age and sex on LV diastolic strain analysis and compare STE measurements with conventional LV diastolic measurements. One-hundred-forty-seven healthy subjects aged 20-72 years (≥ 28 subjects per age decade) were prospectively included (Mean age 44 ± 13.7 years, 50% female) and examined with electrocardiography and 2D-echocardiography, including speckle tracking. Left ventricular peak early diastolic strain rate (Sre) was measured in the apical windows, using STE. Men had significantly lower LV Sre values than women (1.02 ± 0.22 vs. 1.18 ± 0.23, p value < 0.001). Left ventricular Sre was inversely associated with age, with values decreasing with ageing. An inverse relation was also found with blood pressure and body surface area. Linear regression analysis showed that LV Sre was independently associated with both age and sex. A multivariable linear regression analysis for LV Sre with conventional LV diastolic variables accounted for 70.9% of the variation of LV Sre, showing good model performance. Reference values for LV Sre are reported and found to be both age- and sex-dependent. Therefore we recommend age- and sex-specific references values to be used in daily clinical practice.

PETscan: measuring incidence of disease phenotypes to prioritize genetic studies in companion animals.

Reliable incidence measurement of diseases is necessary for identification of hereditary diseases in companion animal populations. The data collection system 'PETscan' was developed to facilitate standardized registration of diagnoses in veterinary practice. In the development, we attempted to counter challenges known from other primary practice data systems. PETscan includes a comprehensive list of potential diagnoses and supports the veterinary professionals in the diagnostic process. Demographics, individual data and standardized diagnostic data are collected through practice management software in a central database for epidemiological analysis. A preliminary data analysis from PETscan showed specific health issues in four canine breeds. As a real-time prospective monitoring tool, PETscan summaries can objectively assess the incidence of disorders in companion animal populations and can be used to prioritize disease-gene identification studies and evaluate the effects of breeding strategies, for example, after implementation of a new DNA test in the breeding strategy.

Heterozygosity testing and multiplex DNA panel screening as a potential tool to monitor health and inbreeding in a small, closed dog population.

BACKGROUND: Selective breeding in populations with a limited effective population size may result in a loss of genetic diversity, which can cause an increased concentration of specific disease liability genes. The Dutch Shepherd Dog (DSD) in the Netherlands is an example of such a breed with a small effective population. OBJECTIVE: To evaluate the measurement of genetic diversity and multiplex DNA panel screening for implementation in a breeding strategy for the Dutch Shepherd Dog (DSD) and to investigate the clinical relevance of potentially identified mutations in the multiplex DNA panel screening. RESULTS: Genome-wide SNP testing showed genetic isolation and reduced genetic diversity within coat variety subgroups of the DSD. Panel screening identified a Von Willebrand's Disease type I mutation. Although decreased Von Willebrand's Factor proteins were significantly lower in DSDs carrying the VWD-I allele compared to the wildtype, clinical follow-up did not show a significant association between the clinical phenotype and VWD-I genotype. CONCLUSIONS: Genetic relationship measurement within a breed population may be a useful tool to enable breeding strategies to conserve genetic diversity. Results from a disease panel screening need to be evaluated for clinical relevance before breed selection restrictions can be considered.

Intratumoral injection of radioactive holmium ( 166 Ho) microspheres for treatment of oral squamous cell carcinoma in cats.

BACKGROUND & AIMS: A "microbrachytherapy" was developed as treatment option for inoperable tumours by direct intratumoral injection of radioactive holmium-166 ( 166 Ho) microspheres (MS). 166 Ho emits ß-radiation which potentially enables a high, ablative, radioactive-absorbed dose on the tumour tissue while sparing surrounding tissues. MATERIALS & METHODS: Safety and efficacy of 166 Ho microbrachytherapy were evaluated in a prospective cohort study of 13 cats with inoperable oral squamous cell carcinoma without evidence of distant metastasis. RESULTS: Local response rate was 55%, including complete response or partial response (downstaging) enabling subsequent marginal resection. Median survival time was 113 days overall, and 296 days for patients with local response. Side effects were minimal. Tumour volume was a significant predictor of response. DISCUSSION: Response rate may be further improved by optimizing the intratumoral spatial distribution of 166 Ho MS. CONCLUSION: 166 Ho microbrachytherapy has potential as a minimally invasive, single procedure radio-ablation treatment of unresectable tumours with minimal morbidity.