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Lancet Child Adolesc Health. 2025 Jun 23:S2352-4642(25)00127-0. doi: 10.1016/S2352-4642(25)00127-0. Online ahead of print.

ABSTRACT

BACKGROUND: Hypertension affects 6% of all children and adolescents, is increasing in prevalence, and is associated with adverse cardiovascular outcomes. In childhood chronic kidney disease, hypertension is associated with progression to kidney failure. However, direct evidence linking childhood hypertension with long-term adverse kidney outcomes is scarce. We aimed to determine the long-term risk of major adverse kidney events (MAKEs) among children and adolescents diagnosed with hypertension.

METHODS: In this population-based retrospective cohort study, we assessed data from all children and adolescents (aged 3-18 years) diagnosed with hypertension from April 1, 1996, to March 31, 2023, in Ontario, Canada, using validated case definitions in health administrative databases. Each case was propensity score-matched with up to five controls without hypertension by age, sex, birthweight, maternal gestational hypertension, pre-existing diabetes, previous cardiovascular surgery, obesity, previous acute kidney injury, and a propensity score for hypertension diagnosis. The primary outcome was major adverse kidney events (MAKEs; ie, all-cause mortality, incident chronic kidney disease, or kidney failure defined as start of chronic dialysis or receipt of kidney transplantation), assessed using weighted Cox regression using robust variance estimators to estimate hazard ratios (HRs) and 95% CIs.

FINDINGS: 26 324 children and adolescents with hypertension were matched with 126 834 controls without hypertension, who were balanced on baseline covariates by propensity score matching. For children and adolescents with hypertension, median age at entry was 15 years (IQR 12-17), there were 10 868 (41·3%) females and 15 456 (58·7%) males, and previous personal and maternal comorbidities were uncommon (1169 [4·4%] had congenital heart disease, 1787 [6·8%] malignancy, 432 [1·6%] diabetes, 2356 [9·0%] complex chronic conditions, and 379 [3·0%] born to mothers with hypertension). During a median 14·2-year follow-up (IQR 7·4-20·7) in the hypertension cohort and 13·7-year follow-up (7·1-21·2) among controls, MAKE incidence was 5·52 per 1000 person-years (95% CI 5·28-5·76) in children and adolescents with hypertension versus 1·66 per 1000 person-years (1·60-1·72) in matched non-hypertensive controls (7·7% vs 2·4%; HR 3·03 [95% CI 2·86-3·21]).

INTERPRETATION: Children and adolescents diagnosed with hypertension are at greater long-term risk of MAKEs compared with non-hypertensive controls. Improved recognition and control of paediatric hypertension might prevent progressive kidney dysfunction. These findings should be confirmed by large-scale, well-controlled prospective studies.

FUNDING: Department of Pediatrics at McMaster University.

PMID:40578375 | DOI:10.1016/S2352-4642(25)00127-0

Lancet. 2025 Jun 24:S0140-6736(25)01037-2. doi: 10.1016/S0140-6736(25)01037-2. Online ahead of print.

ABSTRACT

BACKGROUND: Since its inception in 1974, the Essential Programme on Immunization (EPI) has achieved remarkable success, averting the deaths of an estimated 154 million children worldwide through routine childhood vaccination. However, more recent decades have seen persistent coverage inequities and stagnating progress, which have been further amplified by the COVID-19 pandemic. In 2019, WHO set ambitious goals for improving vaccine coverage globally through the Immunization Agenda 2030 (IA2030). Now halfway through the decade, understanding past and recent coverage trends can help inform and reorient strategies for approaching these aims in the next 5 years.

METHODS: Based on the Global Burden of Diseases, Injuries, and Risk Factors Study 2023, this study provides updated global, regional, and national estimates of routine childhood vaccine coverage from 1980 to 2023 for 204 countries and territories for 11 vaccine-dose combinations recommended by WHO for all children globally. Employing advanced modelling techniques, this analysis accounts for data biases and heterogeneity and integrates new methodologies to model vaccine scale-up and COVID-19 pandemic-related disruptions. To contextualise historic coverage trends and gains still needed to achieve the IA2030 coverage targets, we supplement these results with several secondary analyses: (1) we assess the effect of the COVID-19 pandemic on vaccine coverage; (2) we forecast coverage of select life-course vaccines up to 2030; and (3) we analyse progress needed to reduce the number of zero-dose children by half between 2023 and 2030.

FINDINGS: Overall, global coverage for the original EPI vaccines against diphtheria, tetanus, and pertussis (first dose [DTP1] and third dose [DTP3]), measles (MCV1), polio (Pol3), and tuberculosis (BCG) nearly doubled from 1980 to 2023. However, this long-term trend masks recent challenges. Coverage gains slowed between 2010 and 2019 in many countries and territories, including declines in 21 of 36 high-income countries and territories for at least one of these vaccine doses (excluding BCG, which has been removed from routine immunisation schedules in some countries and territories). The COVID-19 pandemic exacerbated these challenges, with global rates for these vaccines declining sharply since 2020, and still not returning to pre-COVID-19 pandemic levels as of 2023. Coverage for newer vaccines developed and introduced in more recent years, such as immunisations against pneumococcal disease (PCV3) and rotavirus (complete series; RotaC) and a second dose of the measles vaccine (MCV2), saw continued increases globally during the COVID-19 pandemic due to ongoing introductions and scale-ups, but at slower rates than expected in the absence of the pandemic. Forecasts to 2030 for DTP3, PCV3, and MCV2 suggest that only DTP3 would reach the IA2030 target of 90% global coverage, and only under an optimistic scenario. The number of zero-dose children, proxied as children younger than 1 year who do not receive DTP1, decreased by 74·9% (95% uncertainty interval 72·1-77·3) globally between 1980 and 2019, with most of those declines reached during the 1980s and the 2000s. After 2019, counts of zero-dose children rose to a COVID 19-era peak of 18·6 million (17·6-20·0) in 2021. Most zero-dose children remain concentrated in conflict-affected regions and those with various constraints on resources available to put towards vaccination services, particularly sub-Saharan Africa. As of 2023, more than 50% of the 15·7 million (14·6-17·0) global zero-dose children resided in just eight countries (Nigeria, India, Democratic Republic of the Congo, Ethiopia, Somalia, Sudan, Indonesia, and Brazil), emphasising persistent inequities.

INTERPRETATION: Our estimates of current vaccine coverage and forecasts to 2030 suggest that achieving IA2030 targets, such as halving zero-dose children compared with 2019 levels and reaching 90% global coverage for life-course vaccines DTP3, PCV3, and MCV2, will require accelerated progress. Substantial increases in coverage are necessary in many countries and territories, with those in sub-Saharan Africa and south Asia facing the greatest challenges. Recent declines will need to be reversed to restore previous coverage levels in Latin America and the Caribbean, especially for DTP1, DTP3, and Pol3. These findings underscore the crucial need for targeted, equitable immunisation strategies. Strengthening primary health-care systems, addressing vaccine misinformation and hesitancy, and adapting to local contexts are essential to advancing coverage. COVID-19 pandemic recovery efforts, such as WHO's Big Catch-Up, as well as efforts to bolster routine services must prioritise reaching marginalised populations and target subnational geographies to regain lost ground and achieve global immunisation goals.

FUNDING: The Bill & Melinda Gates Foundation and Gavi, the Vaccine Alliance.

PMID:40578370 | DOI:10.1016/S0140-6736(25)01037-2

JACC Heart Fail. 2025 Jun 26;13(8):102505. doi: 10.1016/j.jchf.2025.05.001. Online ahead of print.

NO ABSTRACT

PMID:40578267 | DOI:10.1016/j.jchf.2025.05.001

JACC Heart Fail. 2025 Jun 26;13(8):102511. doi: 10.1016/j.jchf.2025.102511. Online ahead of print.

ABSTRACT

BACKGROUND: Left ventricular dysfunction is common in potential heart donors after brain death, but specific regional wall motion abnormality (RWMA) patterns in this population have not been well described.

OBJECTIVES: This study aims to define and characterize RWMA patterns in potential heart donors after brain death by using a machine learning algorithm.

METHODS: The Donor Heart Study enrolled 4,333 potential heart donors after brain death. All had a transthoracic echocardiogram (TTE) including RWMA assessment, with each segment analyzed for WMS (Wall Motion Score). Those with any RWMA (ie, any WMS >1) were classified using k-means clustering, and each cluster's associations with donor clinical characteristics, heart use, and recipient survival were assessed.

RESULTS: The final analytical cohort included 913 initial TTEs. We identified 4 unique RWMA phenotypes: focal basal septal (FBS) (n = 500), basal and midventricular (n = 311), apical (n = 66), and global hypokinesis (n = 36). These phenotypes exhibited similar donor characteristics but differed in troponin, N-terminal pro-B-type natriuretic peptide levels (both lowest in FBS), and left ventricular ejection fraction (LVEF) (highest in FBS). On subsequent TTEs (performed in 314 donors with any RWMA), all phenotypes demonstrated significant improvement in LVEF. The FBS phenotype had the highest donor heart acceptance for transplantation (68%). Of the hearts accepted for transplantation, there were no significant differences by RWMA phenotype in recipient survival.

CONCLUSIONS: Left ventricular dysfunction after brain death exhibits distinct RWMA phenotypes, which differ in terms of selected biomarkers and LVEF, but not in recipient survival of the hearts accepted for transplantation.

PMID:40578266 | DOI:10.1016/j.jchf.2025.102511

Am J Cardiovasc Drugs. 2025 Jun 27. doi: 10.1007/s40256-025-00741-0. Online ahead of print.

ABSTRACT

Stem cells have emerged as a promising therapeutic approach for ischemic heart failure, with multiple preclinical and clinical trials demonstrating encouraging outcomes. However, limitations and challenges encountered during clinical trials or follow-up periods hinder stem cell therapy's clinical translation for heart failure. In this review, we will elaborate on the applications of stem cell-based therapy, the main subtypes and fundamental properties of stem cells, and the mechanisms by which stem cells exert their effects in ischemic heart failure, such as remuscularization, paracrine effects, autocrine effects, and endocrine-like effects. We will also demonstrate and explain the extensive clinical trials of stem cell therapy in ischemic heart failure, focusing on safety, efficacy, and primary and secondary outcome measures. To improve transplanted stem cells' viability and retention rates, we will discuss various delivery routes and advanced biomaterials used to encapsulate stem cells, such as injectable hydrogels, cardiac patches, and cell sheets. Several challenges severely obstruct the clinical translation of stem cell therapy for ischemic heart failure, including immunological rejection, post-transplantation hypoxia, inflammatory reactions, the maturity of transplanted stem cells, and cost. Finally, we will focus on the prospects of stem cell-based therapy for ischemic heart failure, emphasizing the ongoing need for further research to address existing challenges and establish clearer avenues toward clinical application.

PMID:40576736 | DOI:10.1007/s40256-025-00741-0

Eur J Cardiothorac Surg. 2025 Jun 27:ezaf194. doi: 10.1093/ejcts/ezaf194. Online ahead of print.

NO ABSTRACT

PMID:40576320 | DOI:10.1093/ejcts/ezaf194

Eur J Transl Myol. 2025 Jun 27;35(2). doi: 10.4081/ejtm.2025.13688. Epub 2025 Mar 25.

ABSTRACT

During my PhD, I worked on the neural regulation of mechanical properties fast and slow muscles. This led me to believe that myosins in fast and slow muscles are structurally distinct and that motor nerves regulate the expression of myosin genes. I devised a method for separating intact fast and slow myosins by gel electrophoresis and confirmed their neural regulation. The electrophoresis method was subsequently improved and used to analyse skeletal and cardiac myosin isoforms in various vertebrate species, including marsupials. This led to the discovery of neonatal myosin heavy chain (MyHC), α and β cardiac MyHCs and of the regulation of cardiac MyHCs by thyroid hormone. Antibodies were raised against 2A, 2X, 2B, masticatory and extraocular MyHCs and used to study the expression and regulation of MyHCs in jaw, laryngeal and Extraocular Muscle (EOM) fibres. Antibodies against masticatory myosin enabled the sequencing of masticatory MyHC and masticatory light chain 2 genes. Cross-bridge kinetics of fibres with different myosin isoforms were analysed. Different MyHC isoforms found in jaw-closing muscles across various species reflected evolutionary adaptations to diverse dietary intake, while MyHC expression changes in cardiac and laryngeal muscles with body mass reflected adaptations to changes in their specific metabolic rate. Transplantation experiments on masticatory and EOMs and cross-innervation experiments between laryngeal and somitic muscles revealed that their capacity to express masticatory or extraocular MyHC were myogenically determined but neural impulse patterns also influence MyHC expression. EOMs are the most complex, expressing 11 MyHC isoforms. Some EOM fibres express faster MyHCs in the endplate zone but slower MyHCs at the end segments, an arrangement helping to linearize the saccade. I suggested that during development, primary and secondary extraocular myotubes specify the synaptic inputs of the innervating neurons to generate impulse patterns which regulate the expression of their MyHCs.

PMID:40576020 | DOI:10.4081/ejtm.2025.13688

Turk Gogus Kalp Damar Cerrahisi Derg. 2025 Apr 30;33(2):165-175. doi: 10.5606/tgkdc.dergisi.2025.26847. eCollection 2025 Apr.

ABSTRACT

BACKGROUND: In this study, we aimed to investigate the prognostic value of the tricuspid annular systolic excursion/pulmonary arterial systolic pressure (TAPSE/PASP) ratio as a marker of right ventricle-pulmonary artery uncoupling in patients listed for lung transplantation.

METHODS: Between January 2011 and December 2020, a total of 173 patients (114 males, 59 females; mean age: 53.1±12.5 years; range, 18 to 77 years) who had advanced lung disease or pulmonary vascular disease and were included in the lung transplant list were retrospectively analyzed. Demographic characteristics, laboratory values, long-term mortality data, and clinical and cardiac catheterization data of the patients were compared using a TAPSE/PASP cut-off value of 0.55 mm/mmHg. The univariate and multivariate regression analyses were performed to identify the value of TAPSE/PASP ratio in predicting long-term mortality. The maximal selective rank test was carried out to determine the optimal cut-off value for TAPSE/PASP ratio.

RESULTS: The univariate regression analysis revealed that the TAPSE/PASP ratio, six-minute walk distance, and albumin level were found to be predictors of mortality (hazard ratio [HR]=0.61, 95% confidence interval [CI]: 0.46-0.80, p=0.007; HR=0.72, 95% CI: 0.56-0.91, p=0.007; and HR=0.77, 95% CI: 0.59-0.99, p=0.04, respectively). In the multivariate regression analysis, the TAPSE/PASP ratio, body mass index, and six-minute walk distance were the predictors of mortality (HR=0.49, 95% CI: 0.34-0.70, p=0.004; HR=0.71, 95% CI: 0.51-0.97, p=0.03; and HR=0.71, 95% CI: 0.54-0.94, p=0.01, respectively).Through the maximal selective rank test, the optimal threshold value for TAPSE/PASP ratio was found to be 0.29 mm/mmHg. Patients with TAPSE/PASP >0.29 mm/ mmHg had an average life expectancy of 47.8 months, while the patients with TAPSE/PASP <0.29 mm/mmHg had an average life expectancy of 17.2 months.

CONCLUSION: Our study results suggest that a TAPSE/PASP ratio of <0.29 mm/mmHg is a poor prognostic factor for long-term mortality in patients on the waiting list for lung transplantation.

PMID:40575060 | PMC:PMC12188960 | DOI:10.5606/tgkdc.dergisi.2025.26847

Eur J Cardiothorac Surg. 2025 Jun 17:ezaf187. doi: 10.1093/ejcts/ezaf187. Online ahead of print.

ABSTRACT

OBJECTIVES: Patients with pre-operative atrial fibrillation (AF) undergoing cardiac surgery face a heightened risk of complications and reduced survival. Concomitant surgical ablation (SA) has shown promise in mitigating the arrhythmic burden, prompting guideline upgrades by major scientific societies. However, SA remains underutilized, with performance rates varying between 22% and 48%, depending on the procedure type. This narrative review aims to summarize current evidence to aid physicians in decision-making regarding AF management during cardiac surgery.

METHODS: This review examines existing literature on the prevalence, management, and outcomes of AF in cardiac surgery. We assess epidemiological data, summarize trends in clinical practice, and review the rationale and techniques for treating AF surgically. Emerging challenges, including barriers to implementation and novel therapeutic advancements, are also discussed.

RESULTS: Evidence underscores the detrimental impact of pre-operative AF on perioperative and long-term outcomes, including higher mortality, morbidity, and thromboembolic risk. Concomitant SA, particularly the Cox-maze IV procedure, significantly improves sinus rhythm restoration, reduces mortality, and mitigates complications like stroke. However, the procedure remains underperformed due to concerns about complexity, prolonged operative time, and training gaps. Emerging hybrid techniques, novel mapping systems, and technologies like pulsed field ablation may enhance outcomes and broaden SA adoption.

CONCLUSIONS: Concomitant SA is an effective yet underutilized therapy that can improve survival and reduce AF-related complications in cardiac surgery patients. Addressing implementation barriers and integrating advancements in technology and surgical approaches will be key to optimizing patient outcomes.

PMID:40574669 | DOI:10.1093/ejcts/ezaf187

Cardiol Young. 2025 Jun 27:1-2. doi: 10.1017/S1047951125100735. Online ahead of print.

ABSTRACT

Premature atrial complexes are frequent among patients after heart transplantation. We herein report on the successful use of ivabradine in a 15-year old patient who exhibited marked palpitations caused by atrial premature complexes after orthotopic bicaval heart transplantation.

PMID:40574592 | DOI:10.1017/S1047951125100735

ESC Heart Fail. 2025 Jun 26. doi: 10.1002/ehf2.15357. Online ahead of print.

ABSTRACT

AIMS: Donation after circulatory death (DCD) has emerged as a strategy to increase the donor pool for heart transplantation (HT). Left ventricular assist device (LVAD) patients represent a discrete and unique population. We sought to explore the early outcomes of DCD-HT compared with donation after brain death (DBD) HT in LVAD patients.

METHODS AND RESULTS: We obtained data from the United Network of Organ Sharing database. The main cohort consisted of adults listed for HT between 17 October 2018 and 3 July 2024, with LVAD implanted before or after listing. The primary outcome was survival within the first year post-HT. There were 3336 patients with LVAD underwent HT during the study period (median age 55 years (interquartile range 45-62), 24% women, 29% Black, 89% DBD). The short-term post-HT mortality in LVAD patients who underwent DCD HT was not significantly different from DBD (adjusted hazard ratio [aHR] 1.00, 95% CI 0.70-1.42, P value > 0.9). The likelihood of transplantation within 1 year was higher at centres performing DCD (aHR 1.44, 95% CI 1.39-1.49, P < 0.001). Despite the longer donor-recipient distance in DCD-HT, in-hospital outcomes (stroke and acute kidney injury requiring dialysis) were not different from DBD-HT. A higher incidence of primary graft dysfunction (adjusted risk ratio [aRR] 3.8, 95% CI 2.5-5.7, P < 0.001), and treated rejection was observed with DCD-HT (aRR 1.48, 95% CI 1.14-1.93, P = 0.003).

CONCLUSIONS: In LVAD patients who received DCD HT, early post-transplant survival, stroke, acute kidney injury and length of stay were not significantly different from those who underwent DBD HT. There were increased rates of primary graft dysfunction and treated rejection among LVAD patients who underwent DCD HT. Patients in a DCD centre were significantly more likely to be transplanted earlier.

PMID:40574369 | DOI:10.1002/ehf2.15357

Curr Cardiol Rev. 2025 Jun 25. doi: 10.2174/011573403X362826250619112705. Online ahead of print.

ABSTRACT

INTRODUCTION: Immunologic responses to cardiac allografts initiate before transplantation during brain-dead organ procurement and might persist for years after transplantation, culminating in chronic allograft dysfunction. Despite remarkable advances in post-transplant care and immunosuppressive agents, acute cellular and antibody-mediated rejections as well as chronic allograft vasculopathy significantly affect cardiac allograft and patient survival.

METHODS: Herein, recent findings of the molecular mechanisms involved in the inflammatory responses before and after heart transplantation, including brain death donor inflammation, acute cellular rejection, antibody-mediated rejection, and chronic allograft dysfunction, have been summarized, along with novel therapeutic approaches for their treatment. Finally, recent developments in prognostic and diagnostic biomarkers for immunological complications have been provided, with an overview of the most promising biomarkers to date.

RESULTS: Due to the recent developments in the description of molecular mechanisms involved in the immunopathogenesis of cardiac allograft rejection, some immune cells, proinflammatory cytokines, and adhesion molecules have been proposed as therapeutic targets for the prevention or treatment of alloimmune responses. In addition, several molecules derived from graft tissue or immune cells, e.g. natriuretic peptides, cardiac troponins, exosomal products, microRNAs, and donor-derived cell-free DNA, have been suggested as potential biomarkers for the prediction or diagnosis of cardiac transplant rejection.

CONCLUSION: Considering the need to design non-invasive, low-cost tests for early diagnosis of post-transplant complications and convenient follow-up of the cardiac transplant recipients, peripheral blood biomarkers could be appropriate candidates for this purpose.

PMID:40574363 | DOI:10.2174/011573403X362826250619112705

Vaccines (Basel). 2025 May 23;13(6):554. doi: 10.3390/vaccines13060554.

ABSTRACT

The COVID-19 pandemic posed a huge challenge to global health systems. In addition to searching for effective methods of treating and preventing infection with a new pathogen, we could once again observe that severe respiratory infection and its complications can be become a challenging problem for cardiac patients. Empirical observations are fully confirmed by the results of clinical trials. Patients with risk factors and already diagnosed with cardiovascular diseases are particularly exposed to the severe course of COVID-19, including death. That is why we consider it so important to promote vaccinations against COVID-19 as a safe and effective method of preventing serious infections in this special group of patients, in accordance with the updated recommendations of relevant experts. If an infection is detected, depending on its form and the risk of hospitalization, there are also several antiviral treatment strategies. Nirmatrelvir/ritonavir therapy is particularly effective in selected patient groups, but its use requires analysis of the cardiac pharmacotherapy regimen in the context of potentially significant drug interactions.

PMID:40573885 | DOI:10.3390/vaccines13060554

J Cardiothorac Vasc Anesth. 2025 May 30:S1053-0770(25)00451-3. doi: 10.1053/j.jvca.2025.05.059. Online ahead of print.

ABSTRACT

OBJECTIVES: To evaluate the prevalence of chronic postsurgical pain (CPSP) in children after cardiac surgery.

DESIGN: Cohort study.

SETTING: Quaternary academic pediatric hospital.

PARTICIPANTS: Patients age 6 to 18 years at the time of their surgery and a surgery date ≥6 months prior to the study period (June 1, 2014, to November 1, 2021).

INTERVENTIONS: Patients completed 3 validated surveys measuring chronic pain, the impact of pain on quality of life (QoL), and neuropathic pain symptoms. Respondents' medical records were then reviewed for perioperative management.

MEASUREMENTS AND MAIN RESULTS: Pain severity, pain trajectory, and analgesic consumption are described for each comparative group of interest (time period, 2014-2019 vs 2020-2021), intraoperative opioid selection (remifentanil vs sufentanil), surgical approach (sternotomy vs thoracotomy), and intraoperative pain management technique (presence/absence of fascial plane block). The response rate was 10%. Reference tables from validated scoring systems were used when available. At the time of survey completion or in the preceding week, 34% reported pain, with none to mild interference in QoL. Twenty percent reported symptoms consistent with neuropathic pain, with no difference between recipients of sternotomy and recipients of thoracotomy. Patients who received intraoperative remifentantil had higher pain scores. Single-shot fascial plane blocks reduced postoperative acute pain scores.

CONCLUSIONS: The validated surveys showed a high prevalence of CPSP, including a sizeable proportion of neuropathic pain. The prevalence of CPSP is within the reported prevalence in adults despite a mean study population age of 10 years. This finding encourages improvements in acute pain management, earlier identification of patients with CPSP for management, and further investigation of CPSP after pediatric cardiac surgery.

PMID:40579245 | DOI:10.1053/j.jvca.2025.05.059

Heart. 2025 Jun 27:heartjnl-2025-325811. doi: 10.1136/heartjnl-2025-325811. Online ahead of print.

NO ABSTRACT

PMID:40579215 | DOI:10.1136/heartjnl-2025-325811

Heart. 2025 Jun 27:heartjnl-2024-325296. doi: 10.1136/heartjnl-2024-325296. Online ahead of print.

ABSTRACT

BACKGROUND: The global prevalence of congenital heart disease (CHD) is increasing. Research on patient-reported outcomes (PROs) predominantly originates from high-income countries, resulting in an incomplete understanding of the true global burden of CHD from the patient perspective. Therefore, we described perceived health, psychological distress and quality of life (QoL) in a large sample of adults with CHD from the globe and explored the relationship between PROs and the income level of the countries.

METHODS: Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart Disease-International Study II (APPROACH-IS II) represents an international cross-sectional investigation of PROs in 8415 patients from 53 centres across 32 countries. Patients completed questionnaires to measure perceived health status (RAND-12 Health Survey; EuroQOL-5D Visual Analog Scale); depressive symptoms (Patient Health Questionnaire-8, PHQ-8); anxiety (Generalized Anxiety Disorder Scale-7) and QoL (Linear Analog Scale). Gross National Income per capita in US dollars was used for stratifying countries according to income levels.

RESULTS: Large intercountry disparities in PROs were observed. Switzerland demonstrated the highest mean scores for physical functioning, self-rated health and QoL, while Senegal had the lowest scores. Patients from Malta demonstrated the highest mean scores for mental health, and Senegal had the lowest scores. With regard to depressive symptoms and anxiety, Pakistan had the lowest mean scores, while Turkey had the highest scores. Patients from high-income nations reported significantly better physical functioning, mental functioning and QoL.

CONCLUSION: Large intercountry disparities in PROs were observed. APPROACH-IS II is a pioneering international endeavour that comprehensively evaluated PROs among adults with CHD, drawing participants from nations with different income levels.

TRIAL REGISTRATION NUMBER: NCT04902768.

PMID:40579213 | DOI:10.1136/heartjnl-2024-325296

BMJ Case Rep. 2025 Jun 27;18(6):e264831. doi: 10.1136/bcr-2025-264831.

ABSTRACT

Tricuspid atresia is a complex congenital cardiac anomaly with single ventricle physiology characterised by intracardiac mixing of oxygenated and deoxygenated blood. Managing such parturient demands an in-depth understanding of the cardiac defect, the stage of surgical palliation, functional status, and the cardiovascular impact of pregnancy and labour. This report details a female in her late twenties with tricuspid atresia and hypoplastic right ventricle, previously treated with a bidirectional Glenn shunt, who underwent a successful emergency caesarean section at 34 weeks using neuraxial anaesthesia. The case underscores the anaesthetic challenges and perioperative strategies involved in managing patients with such complex cardiac physiology.

PMID:40579205 | DOI:10.1136/bcr-2025-264831

JACC Case Rep. 2025 Jun 25;30(16):104200. doi: 10.1016/j.jaccas.2025.104200.

ABSTRACT

BACKGROUND: Cor triatriatum sinister is a rare congenital heart disease that can mimic left atrial obstructive lesions. Symptoms vary based on the severity of obstruction.

CASE SUMMARY: We report a case of a 14-year-old girl with severe exercise intolerance. Echocardiography and computed tomography scan revealed a fibromuscular membrane causing left atrial obstruction and an anomalous venous drainage with a persistent left superior vena cava to the coronary sinus. Surgical intervention is definitive, typically via sternotomy.

DISCUSSION: Given the complexity and lack of expertise in minimal invasive thoracotomy for such lesions, we used 3-dimensional (3D) heart printing for virtual surgical planning. This approach facilitated a safe and precise repair. To our knowledge, this is the first reported case using 3D printing to enable a minimally invasive thoracotomy for cor triatriatum sinister correction.

TAKE-HOME MESSAGES: Minimally invasive approaches should not be dismissed for complex congenital heart defects. Multimodal imaging can enhance surgical feasibility.

PMID:40579109 | DOI:10.1016/j.jaccas.2025.104200

JACC Case Rep. 2025 Jun 25;30(16):104341. doi: 10.1016/j.jaccas.2025.104341.

ABSTRACT

BACKGROUND: Right ventricle-pulmonary artery (RV-PA) conduit stenosis is a relatively uncommon complication after a childhood Ross-Konno procedure.

CASE SUMMARY: A man in his 20s presented with progressive dyspnea for 1 year. He had previously undergone modified Konno and Ross-Konno procedures for congenital subaortic stenosis followed by pulmonic balloon valvuloplasty for RV-PA stenosis. Echocardiography demonstrated reduced left ventricular ejection fraction, severe pulmonary hypertension, and right ventricular outflow tract obstruction. Cardiac computed tomography revealed right ventricular outflow/conduit stenosis with calcification. Right heart catheterization showed elevated right ventricular outflow/conduit pressure gradient, confirming severe conduit stenosis. He was referred for surgical pulmonic valve and conduit repair.

DISCUSSION: Surgical conduit replacement remains the most definitive treatment strategy in small, calcified conduits. Multimodal imaging strategies aid in comprehensive assessment of anatomy and function of RV-PA conduit and valves to guide intervention strategies in adults with complex congenital heart disease.

PMID:40579096 | DOI:10.1016/j.jaccas.2025.104341

JACC Case Rep. 2025 Jun 25;30(16):103884. doi: 10.1016/j.jaccas.2025.103884.

ABSTRACT

BACKGROUND: Determining the optimal surgical approach for complex congenital heart disease (CHD) can be challenging using conventional 2-dimensional transthoracic echocardiography (TTE) and cross-sectional imaging, including cardiac magnetic resonance (CMR) and computed tomography.

CASE SUMMARY: An 11-month-old female patient presented with heterotaxy syndrome, dextrocardia, double-outlet right ventricle, and complex pulmonary valve stenosis. Fetal echocardiography and postnatal TTE established the complexity of the cardiac anatomy and led to a multidisciplinary discussion to plan a complex staged cardiac repair.

DISCUSSION: Three-dimensional (3D) printed and virtual heart models, along with CMR angiography, provided detailed anatomical visualization and spatial conceptualization, which aided in assessing the feasibility of a staged biventricular repair. Postoperative CMR guided subsequent surgical procedures, evaluated newly created structures such as baffles, and identified complications.

TAKE-HOME MESSAGE: This case highlights the critical role of multimodality imaging, including advanced imaging with virtual and 3D printed modeling, in surgical planning in a patient with complex CHD.

PMID:40579094 | DOI:10.1016/j.jaccas.2025.103884