World Journal of Cardiology最新文献

{"title":"Impact of optimal medical therapy in heart failure certification for hospitalists on guideline-directed medical therapy utilization.","authors":"Farhan Ishaq, Duc T Nguyen, Edward A Graviss, Ebun Ebunlomo, Arvind Bhimaraj, Nadia Fida","doi":"10.4330/wjc.v17.i6.107102","DOIUrl":"10.4330/wjc.v17.i6.107102","url":null,"abstract":"<p><strong>Background: </strong>Significant gaps in guideline-directed medical therapy (GDMT) for heart failure (HF) stem from shortages of cardiologists and advanced HF providers, as well as a lack of optimal HF management knowledge among hospitalists. This study compared the impact of optimal medical therapy in HF (OMT-HF) certification on GDMT implementation and patient outcomes between an intervention group (IG) of hospitalists and a standard-of-care comparison group (SOC-CG).</p><p><strong>Aim: </strong>To evaluate if OMT-HF has a difference in GDMT and patients in outcomes between IG and SOC-CG.</p><p><strong>Methods: </strong>This study was implemented from November 2022 to May 2023. Hospitalized cardiology patients with HF and left ventricular ejection fraction ≤ 40% were randomized to IG or SOC-CG. Exclusion criteria included patients in cardiogenic shock, unable to consent, or at high risk. Follow-up was at 30 days post-discharge. Differences between groups were analyzed using Fisher's exact test for categorical variables and Wilcoxon rank-sum or unpaired <i>t</i>-test for continuous variables. Changes in Minnesota Living with Heart Failure Questionnaire (MLWHFQ) scores were evaluated using a paired <i>t</i>-test.</p><p><strong>Results: </strong>IG patients had lower readmission rates [(9 (42.85%) <i>vs</i> 11 (17.46%), <i>P</i> = 0.03] and a decreased trend in mortality 30-day post discharge. IG patients also showed greater mean improvements in total (-27.03 ± 24.59 <i>vs</i> -5.85 ± 23.52, <i>P</i> < 0.001), physical (-13.8 ± 12.3 <i>vs</i> -2.71 ± 11.16, <i>P</i> < 0.001) and emotional (-4.76 ± 8.10 <i>vs</i> -1.42 ± 5.98) dimensions on the MLWHFQ compared to SOC-CG, however, change in emotional dimension did not reach statistical significance.</p><p><strong>Conclusion: </strong>Hospitalist OMT-HF certification may lead to better 30-day outcomes in hospitalized HF patients including quality of life, mortality and readmission rates. Larger prospective studies are warranted to validate these findings.</p>","PeriodicalId":23800,"journal":{"name":"World Journal of Cardiology","volume":"17 6","pages":"107102"},"PeriodicalIF":1.9,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186193/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Beyond initial recovery: Heart failure with transient <i>vs</i> sustained improvement in left ventricular ejection fraction.","authors":"Rasha Kaddoura, Ammar Chapra, Jassim Shah, Mohamed Izham, Rajvir Singh, Haisam Alsadi, Maha Al-Amri, Tahseen Hamamyh, Manar Fallouh, Farras Elasad, Mohamed Abdelghani, Sumaya Alsaadi Alyafei, Amr Badr, Ashfaq Patel","doi":"10.4330/wjc.v17.i6.106717","DOIUrl":"10.4330/wjc.v17.i6.106717","url":null,"abstract":"<p><strong>Background: </strong>There is no available data about the trajectory of heart failure (HF) with improved ejection fraction (EF) and patient clinical outcomes in Qatar.</p><p><strong>Aim: </strong>To explore the difference in characteristics and outcomes between patients with transient and sustained improvement in left ventricular ejection fraction (LVEF) and to determine the independent predictors for sustained improvement in LVEF.</p><p><strong>Methods: </strong>This is a retrospective cohort study that was conducted at the advanced HF clinic of a tertiary care hospital in Qatar between January 2017 and December 2018. This study included adult patients with improved LVEF and had at least three echocardiographic studies. The patients were divided into two groups: HF with transient improvement in EF (HFtimpEF) and HF with sustained improvement in EF (HFsimpEF).</p><p><strong>Results: </strong>A total of 175 patients with HF and improved EF were included. Among them 136 (77.7%) patients showed sustained improvement in LVEF. The remaining patients with HFtimpEF were predominantly males [37 (94.9%) <i>vs</i> 101 (74.3%), <i>P</i> = 0.005] with a higher incidence of ischemic cardiomyopathy [32 (82.1%) <i>vs</i> 68 (50.4%), <i>P</i> = 0.002], dyslipidemia [24 (61.5%) <i>vs</i> 54 (39.7%), <i>P</i> = 0.03], and hypertension [34 (87.2%) <i>vs</i> 93 (68.4%), <i>P</i> = 0.03] than those with HFsimpEF. The latter experienced significantly lower rates of hospitalization [39 (28.7%) <i>vs</i> 20 (51.3%), <i>P</i> = 0.01] and diagnosis of new cardiovascular conditions during the follow-up (<i>e.g.</i>, acute coronary syndrome, stroke, decompensated HF, and atrial fibrillation) [14 (10.3%) <i>vs</i> 10 (25.6%), <i>P</i> = 0.03] without a difference in emergency department visits or in-hospital death. Sustained improvement in LVEF was positively associated with being female [adjusted odds ratio (aOR) = 6.8, 95% confidence interval (CI): 1.4-32.3, <i>P</i> = 0.02], having non-ischemic etiology of HF (aOR = 3.1, 95%CI: 1.03-9.3, <i>P</i> = 0.04), and using a mineralocorticoid receptor antagonist (aOR = 7.0, 95%CI: 1.50-31.8, <i>P</i> = 0.01).</p><p><strong>Conclusion: </strong>Patients with HFsimpEF experienced significantly lower rates of hospitalization and diagnosis of new cardiovascular conditions than patients with HFtimpEF. Sustained improvement in LVEF was positively associated with being a female, having non-ischemic etiology of HF, and using a mineralocorticoid receptor antagonist.</p>","PeriodicalId":23800,"journal":{"name":"World Journal of Cardiology","volume":"17 6","pages":"106717"},"PeriodicalIF":1.9,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186171/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508630","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term prognostic role of adiponectin in stable coronary artery disease: A meta-analysis of prospective studies.","authors":"Sahas Reddy Jitta, Priyanka Vatsavayi, Chenna Reddy Tera, Shobana Krishnamurthy, Saisree Reddy Adla Jala, Diksha Sanjana Pasnoor, Utheja Dasari, Aisha Farooq, Supriya Maramreddy, Kavya Jammula, Medha Reddy Kesani, Sridevi Tripuraneni, Nihar Jena, Rupak Desai","doi":"10.4330/wjc.v17.i6.105452","DOIUrl":"10.4330/wjc.v17.i6.105452","url":null,"abstract":"<p><strong>Background: </strong>The persistent burden of cardiovascular (CV) disease in the United States requires innovative and cost-effective prognostic markers that can be relied upon.</p><p><strong>Aim: </strong>To provide insights into how adiponectin can predict all-cause mortality and major adverse CV events (MACE) in patients with coronary artery disease (CAD) and to determine the prognostic value of adiponectin in predicting all-cause mortality and MACE in patients with stable CAD.</p><p><strong>Methods: </strong>We conducted a systematic search on PubMed, Scopus, and Google Scholar to find relevant studies published through June 2023 evaluating the long-term prognostic role of adiponectin in patients with stable CAD. Using a random effects model with 95%CI, we estimated the odds ratio (OR) while assessing heterogeneity through <i>I²</i> statistics. To ensure robustness, we performed a sensitivity analysis using the leave-one-out approach.</p><p><strong>Results: </strong>After screening, we included five prospective studies involving 3225 patients who were followed up for a median duration of 3.8 years. Within the study population, prevalent risk factors included hypertension, diabetes, hyperlipidemia, and smoking. The commonly prescribed medications were angiotensin-converting enzyme inhibitors, beta blockers, and statins. The combined adjusted OR for all-cause mortality was found to be 2.51 (95%CI: 1.36-4.62), showing heterogeneity (<i>I²</i> = 65.51%, <i>P</i> = 0.03). On the other hand, the combined adjusted OR for MACE was determined to be 1.04 (95%CI: 1.02-1.06) with no significant heterogeneity observed (<i>I²</i> = 0%, <i>P</i> = 0.68). Through a sensitivity analysis, it was discovered that none of the studies significantly impacted the overall results of the meta-analysis, thus indicating their robustness.</p><p><strong>Conclusion: </strong>Higher levels of adiponectin were found to be associated with an increased risk of long-term mortality and MACE in patients with CAD, which highlights its potential as a cost-effective marker for risk assessment and guiding treatment strategies. Further research on the role of adiponectin could greatly influence decision-making and resource allocation in CV care.</p>","PeriodicalId":23800,"journal":{"name":"World Journal of Cardiology","volume":"17 6","pages":"105452"},"PeriodicalIF":1.9,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186166/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508637","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rising trend of E-cigarettes among adolescents of Pakistan and cardiovascular implications of electronic nicotine delivery systems.","authors":"Abdul Basit, Sarmad Javed, Qasim Mehmood, Muhammad Ahmad Hammad, Farzeela, Abdul Mabood Basil","doi":"10.4330/wjc.v17.i6.108567","DOIUrl":"10.4330/wjc.v17.i6.108567","url":null,"abstract":"<p><p>It is a common misconception that e-cigarettes are safer than tobacco smoking, given their adverse cardiopulmonary effects, habituation, and the fact that it is only a fashion based manifestation to sell and use them. Therefore, the use of e cigarettes should not be encouraged as an alternative and pragmatic measure should be taken to profess this agenda.</p>","PeriodicalId":23800,"journal":{"name":"World Journal of Cardiology","volume":"17 6","pages":"108567"},"PeriodicalIF":1.9,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186190/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508641","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hydrogen alleviates right ventricular hypertrophy by inhibiting ferroptosis <i>via</i> restoration of the Nrf2/HO-1 signaling pathway.","authors":"Jun-Cai Bai, Hong-Xiao Yang, Cheng-Chuang Zhan, Lu-Qi Zhao, Jia-Ren Liu, Wei Yang","doi":"10.4330/wjc.v17.i6.104832","DOIUrl":"10.4330/wjc.v17.i6.104832","url":null,"abstract":"<p><strong>Background: </strong>Right ventricular hypertrophy (RVH) occurs because of volume or pressure overload within the right ventricular (RV) system. RVH is associated with complex pathological changes, including myocardial cell injury, apoptosis, myocardial fibrosis, neuroendocrine disturbances, and abnormal water and liquid metabolism. Ferroptosis, a novel type of iron-dependent cell death characterized by lipid peroxide accumulation, is an important mechanism of cardiomyocyte death. However, the role of ferroptosis in RVH has rarely been studied. We hypothesize that hydrogen (H₂), an experimental medical gas with superior distribution characteristics, inhibits ferroptosis.</p><p><strong>Aim: </strong>To explore the protective effect of H₂ on RVH and the mechanism by which H₂ regulates ferroptosis.</p><p><strong>Methods: </strong>An <i>in vivo</i> RVH rat model was induced by monocrotaline (MCT) in 30 male Sprague-Dawley rats. An H9C2 cell model was treated with angiotensin II to simulate pressure overload in the RV system <i>in vitro</i>. H₂ was administered to rats by inhalation (2% for 3 hours daily for 21 days) and added to the cell culture medium. The Nrf2 inhibitor ML385 (1 μM) was used to investigate anti-ferroptotic mechanisms.</p><p><strong>Results: </strong>In MCT-treated rats, H₂ inhalation decreased RVH; the RV wall thickness decreased from 3.5 ± 0.3 mm to 2.8 ± 0.2 mm (<i>P</i> < 0.05) and the RV ejection fraction increased from 45 ± 3% to 52 ± 4% (<i>P</i> < 0.05). In H9C2 cells, H₂ alleviated hypertrophy. H₂ inhibited ferroptosis by modulating the iron content, oxidative stress, and ferroptosis-related proteins, thereby restoring the Nrf2/HO-1 signaling pathway.</p><p><strong>Conclusion: </strong>H₂ retards RVH by inhibiting ferroptosis <i>via</i> Nrf2/HO-1 restoration, suggesting a new treatment strategy.</p>","PeriodicalId":23800,"journal":{"name":"World Journal of Cardiology","volume":"17 6","pages":"104832"},"PeriodicalIF":1.9,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186143/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508633","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Incidence, risk factors and clinical outcomes of pericardial effusion in left ventricular assist device patients.","authors":"Muhammad Zubair Khan, Yevgeniy Brailovsky, Mohammad Alfrad Nobel Bhuiyan, Gregary Marhefka, Abu S M Faisal, Adrija Sircar, Parker O'Neill, J Eduardo Rame, Sona Franklin, Muhammad Waqas, Hadia Shah, Indranee Rajapreyar, Rene J Alvarez","doi":"10.4330/wjc.v17.i6.105330","DOIUrl":"10.4330/wjc.v17.i6.105330","url":null,"abstract":"<p><strong>Background: </strong>Development of pericardial effusion in patients with left ventricular assist devices (LVADs) can be detrimental to health outcomes. This study aims to elucidate the prevalence and risk factors for pericardial effusion in patients with LVADs.</p><p><strong>Aim: </strong>To elucidate risk factors associated with the presence of pericardial effusion in patients with LVADs and compare the clinical outcomes of those with and without pericardial effusion. The secondary goal is to determine the incidence of pericardiocentesis and pericardial window placement in patients with LVADs experiencing pericardial effusion.</p><p><strong>Methods: </strong>Data were obtained from the National Inpatient Sample database between 2016 and 2018. Statistical analysis was performed using Pearson <i>χ</i> ² test and multivariate logistic regression analysis to determine clinical outcomes of pericardial effusion and to identify variables associated with pericardial effusion in LVAD patients, respectively.</p><p><strong>Results: </strong>The prevalence of LVAD was 9850 (0.01%) among total study patients (<i>n</i> = 98112095). The incidence of pericardial effusion among LVAD patients was 640 (6.5%). The prevalence of liver disease (26.6% <i>vs</i> 17.4%), chronic kidney disease (CKD; 54.6% <i>vs</i> 49.4%), hypothyroidism (21.9% <i>vs</i> 18.1%), congestive heart failure (98.4% <i>vs</i> 96.5%), atrial fibrillation (Afib; 58.59% <i>vs</i> 50.5%), coronary artery disease (CAD; 11.7% <i>vs</i> 4.4%), dyslipidemia (31.3% <i>vs</i> 39.3%), and having undergone percutaneous coronary intervention (PCI; 1.6% <i>vs</i> 0.7%) was higher in the pericardial effusion cohort <i>vs</i> the non-pericardial effusion cohort. Multivariate regression analysis demonstrated that CAD (OR = 2.89) and PCI (OR = 2.2) had the greatest association with pericardial effusion in patients with LVADs. These were followed by liver disease (OR = 1.72), hypothyroidism (OR = 1.2), electrolyte derangement (OR = 1.2), Afib (OR = 1.1), and CKD (OR = 1.05). Among patients with LVADs, the median length of stay (33 days <i>vs</i> 27 days) and hospitalization cost (847525 USD <i>vs</i> 792616 USD) were significantly higher in the pericardial effusion cohort compared to the non-pericardial effusion cohort. There was no significant difference in mortality between cohorts. The prevalence of cardiac tamponade was 109 (17.9% of LVAD patients with pericardial effusion). Ten (9.2% of LVAD patients with cardiac tamponade) patients underwent pericardiocentesis and 44 (40.3%) received a pericardial window.</p><p><strong>Conclusion: </strong>This study shows that liver disease, CKD, PCI, hypothyroidism, electrolyte derangement, Afib, and CAD had a significant association with pericardial effusion in LVAD patients. Hospitalization cost and length of stay were higher in the pericardial effusion group, but mortality was the same.</p>","PeriodicalId":23800,"journal":{"name":"World Journal of Cardiology","volume":"17 6","pages":"105330"},"PeriodicalIF":1.9,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186126/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508636","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnosis and treatment options for sinus of Valsalva aneurysms: A narrative review.","authors":"Kevan English","doi":"10.4330/wjc.v17.i6.102722","DOIUrl":"10.4330/wjc.v17.i6.102722","url":null,"abstract":"<p><p>Sinus of Valsalva aneurysm (SoVA) is a rare cardiac defect that may be congenital or acquired. It is characterized by abnormal dilatation of the aortic root due to a weakened elastic lamina at the junction of the annulus fibrosus and the aortic media. SoVAs are present in approximately 0.09% of the general population and comprise up to 3.5% of all congenital cardiac defects. It is usually found incidentally on cardiac imaging, with a higher incidence observed in the Western populations and a male-to-female ratio of 4:1. A transthoracic two-dimensional echocardiogram is the initial diagnostic test of choice, which may reveal the characteristic \"windsock deformity\" that clinches the diagnosis. Other imaging modalities, such as transesophageal echocardiography and cardiac computed tomography angiography, help provide more extensive details of the aneurysm and its adjacent structures. Management options for ruptured and unruptured SoVA include surgical repair or transcatheter closure, which serves as a game-changing development in treatment. This article aims to provide background information on the epidemiology, pathophysiology, diagnosis, and recent advancements over the past decade in the management of SoVAs.</p>","PeriodicalId":23800,"journal":{"name":"World Journal of Cardiology","volume":"17 6","pages":"102722"},"PeriodicalIF":1.9,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186169/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508631","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of gut microbiome on atrial fibrillation: Mechanistic insights and future directions in individualized medicine.","authors":"Ajit Singh Brar, Shree Laya Vemula, Vishnu Yanamaladoddi, Sohail Sodhi, Juniali Hatwal, Aalam Sohal, Akash Batta","doi":"10.4330/wjc.v17.i6.107386","DOIUrl":"10.4330/wjc.v17.i6.107386","url":null,"abstract":"<p><p>Atrial fibrillation (AF) is a growing global health burden, with a prevalence of over 52.55 million cases. Rising disability-adjusted life-years, increasing age, and disparities in care have contributed to the worsening severity and mortality of AF. Modifiable risk factors, such as hypertension, obesity, and diabetes mellitus, are associated with alterations in gut microbiota, making the gut-heart axis a potential therapeutic target. Gut dysbiosis influences AF pathogenesis through inflammation, metabolic disruption, and autonomic dysfunction. Key mechanisms include gut barrier dysfunction, short-chain fatty acid (SCFA) depletion, lipopolysaccharides (LPS)-induced inflammation, and ferroptosis-mediated atrial remodeling. Trimethylamine N-oxide, bile acids, and tryptophan metabolites contribute to arrhythmogenic remodeling. Emerging evidence suggests that dietary interventions, including prebiotics and probiotics, as well as gut surveillance, may help mitigate AF progression. Clinical implications of gut modulation in AF include personalized dietary strategies, microbiome assessment through metagenomic sequencing, and targeted interventions such as SCFA-based therapies and ferroptosis inhibition. Metabolite surveillance, including LPS and indoxyl sulfate monitoring, may influence the effectiveness of anticoagulant and antiarrhythmic therapy. Despite growing mechanistic evidence linking gut dysbiosis to AF, clinical applications remain unexplored. This review summarizes the current understanding of the gut microbiome's role in AF.</p>","PeriodicalId":23800,"journal":{"name":"World Journal of Cardiology","volume":"17 6","pages":"107386"},"PeriodicalIF":1.9,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186181/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508634","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Navigating women with congenital heart disease during pregnancy: Management strategies and future directions.","authors":"Bibhuti B Das, Varun Aggarwal, Shriprasad R Deshpande","doi":"10.4330/wjc.v17.i6.106295","DOIUrl":"10.4330/wjc.v17.i6.106295","url":null,"abstract":"<p><p>Women with adult congenital heart disease (CHD) face unique challenges during pregnancy, as gestational cardiovascular (CV) and hemodynamic changes can exacerbate underlying cardiac conditions. While these adaptations are well tolerated in women with structurally and functionally normal hearts, they pose significant risks for those with adult CHD (ACHD), whether repaired, palliated, or with residual defects. Maternal CHD is associated with an increased risk of adverse CV events, including stroke, heart failure, arrhythmias, and thromboembolic complications during pregnancy and the peripartum period. Effective management requires a multidisciplinary team, including cardiologists, perinatologists, anesthesiologists, and other skilled care providers. Risk stratification tools such as the modified World Health Organization classification, CARPREG II, and ZAHARA scores are useful for predicting maternal and fetal outcomes and guiding clinical decision-making. Preconception counseling plays a critical role in assessing individual risks, optimizing cardiac function, and educating patients about potential complications. Future research should prioritize innovative therapies, including targeted pharmacological agents and minimally invasive interventions, alongside improved screening methods to identify high-risk patients before symptomatic disease manifests. This review synthesizes current literature on managing pregnant women with ACHD, highlights gaps in clinical practice, and explores future directions to enhance care. Addressing these challenges is essential to improving maternal and fetal outcomes and ensuring comprehensive, patient-centered care throughout the reproductive journey.</p>","PeriodicalId":23800,"journal":{"name":"World Journal of Cardiology","volume":"17 6","pages":"106295"},"PeriodicalIF":1.9,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186142/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508640","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"<i>RAF1</i> mutation expands the cardiac phenotypic spectrum of Noonan syndrome: A case report.","authors":"Nan Ma, Zhong-Wei Li, Jia-Jia Liu, Xing-Guang Liu, Xing Zhou, Bo-Wen Wang, Yan-Ling Li, Tian-Cheng Zhang, Ping Xie","doi":"10.4330/wjc.v17.i6.106525","DOIUrl":"10.4330/wjc.v17.i6.106525","url":null,"abstract":"<p><strong>Background: </strong>Noonan syndrome is a relatively common autosomal dominant genetic disorder characterized by cardiovascular defects owing to functional abnormalities in key genes such as <i>RAF1</i>. Mutations in <i>RAF1</i> are typically associated with hypertrophic cardiomyopathy (HCM). However, in this case, the patient exhibited atrial and ventricular septal defects (VSDs).</p><p><strong>Case summary: </strong>This case report describes an 11-year-old boy diagnosed with Noonan syndrome, in whom genetic testing revealed a c.770C>T (p.Ser257 Leu) mutation in <i>RAF1</i>. The patient presented with intermittent chest discomfort and shortness of breath, symptoms that significantly worsened after physical activity. Clinical evaluation revealed marked growth retardation and multiple physical abnormalities. Electrocardiographic and echocardiographic assessments revealed VSDs, atrial septal defects, and left ventricular outflow tract obstruction. Following multidisciplinary consultation, the patient underwent cardiac surgical intervention, which led to clinical improvement; however, they subsequently developed a third-degree atrioventricular block, necessitating the implantation of a permanent pacemaker. During follow-up, echocardiographic findings demonstrated near-complete resolution of the shunt across the atrial and ventricular septa, significant improvement in left ventricular outflow tract obstruction, and notable reduction in ventricular septal thickness. A genetic mutation at the c.770C>T (p.Ser257 Leu) locus of <i>RAF1</i> is typically associated with HCM and pulmonary hypertension. However, this patient's clinical phenotype manifested as HCM, atrial septal defect, and VSD, suggesting that this mutation may involve a different pathophysiological mechanism.</p><p><strong>Conclusion: </strong>This case confirms the genotype-phenotype heterogeneity of Noonan syndrome and highlights the complex management requirements of <i>RAF1</i> mutation-associated cardiac pathologies. Early surgical intervention can ameliorate structural defects, but it must be integrated with genetic counseling and lifelong monitoring to optimize patient outcomes.</p>","PeriodicalId":23800,"journal":{"name":"World Journal of Cardiology","volume":"17 6","pages":"106525"},"PeriodicalIF":1.9,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12186123/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508628","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}