Hui Zha , Xuejia Zhai , Lin Wang , Quancheng Yang , Qing Guo , Yanyu Chen , Jie Liu , Hua Peng
{"title":"Transforming pediatric heart failure: Efficacy of low-dose sacubitril/valsartan","authors":"Hui Zha , Xuejia Zhai , Lin Wang , Quancheng Yang , Qing Guo , Yanyu Chen , Jie Liu , Hua Peng","doi":"10.1016/j.cpcardiol.2024.102926","DOIUrl":"10.1016/j.cpcardiol.2024.102926","url":null,"abstract":"<div><h3>Aims</h3><div>Pediatric heart failure is a significant cause of illness and death in children. We aimed to assess sacubitril/valsartan's effectiveness and find the proper dosage for pediatric patients.</div></div><div><h3>Methods</h3><div>Patients unresponsive to traditional medicines for at least 12 months prescribed sacubitril/valsartan from January 2020 to March 2023 were reviewed. The initial dose was 0.2 mg/kg bid, gradually increasing in 0.1 mg/kg increments. The target dose was determined based on blood pressure fluctuations at 70/50 mmHg. Clinical efficacy and quantity-effect relationship were evaluated using echocardiography, NT-proBNP, and the concentration of valsartan, sacubitril, sacubitrilat.</div></div><div><h3>Results</h3><div>A total of 23 pediatric patients with dilated cardiomyopathy and advanced heart failure were enrolled. Mean sacubitril/valsartan dose was 1.84 mg/kg/day. After 6 months, LVEF increased significantly (38.09% to 45% at 3 months, 52% at 6 months; <em>p</em> < 0.001). LV size reduced to 4.4 cm (IQR, 4.1–5.2) and 4.5 cm (IQR, 4–5.1) at 3 and 6 months, respectively, from 4.6 cm (IQR, 4.2–5.6) at baseline (both <em>P</em> < 0.05). NT-proBNP levels reduced by 5.7 at 3 months (<em>p</em> < 0.05) and 5.38 at 6 months (<em>p</em> < 0.001). Sacubitrilat is the active form of Sacubitril. The highest concentration of sacubitrilat was observed at approximately 1.6 mg/kg. The maintenance dose correlated positively with time (<em>p</em> < 0.001) and valsartan/sacubitril concentration (<em>p</em> < 0.05).</div></div><div><h3>Conclusion</h3><div>Low dose sacubitril/valsartan is effective in children with heart failure, with dosage adjustments to avoid hypotension. Adjusted dosing can increase EF and reduce heart size, offering new possibilities for pediatric heart failure treatment.</div></div>","PeriodicalId":51006,"journal":{"name":"Current Problems in Cardiology","volume":"50 1","pages":"Article 102926"},"PeriodicalIF":3.0,"publicationDate":"2024-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142606973","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Robinson Ramírez-Vélez , María Correa-Rodríguez , Juan Carlos Calderón-González , Albeiro Dávila-Grisales , Katherine González-Ruíz , Jorge Enrique Correa-Bautista , Mikel Izquierdo
{"title":"The association between insulin resistance and cytokines in adolescents with excess of adiposity","authors":"Robinson Ramírez-Vélez , María Correa-Rodríguez , Juan Carlos Calderón-González , Albeiro Dávila-Grisales , Katherine González-Ruíz , Jorge Enrique Correa-Bautista , Mikel Izquierdo","doi":"10.1016/j.cpcardiol.2024.102925","DOIUrl":"10.1016/j.cpcardiol.2024.102925","url":null,"abstract":"<div><h3>Background and aims</h3><div>Increased adiposity has been directly associated with insulin resistance (IR), and cytokines released by adipose tissue seem to link adiposity to IR in youth. We used an antibody-based array to investigate the differential levels of serum cytokines according to insulin status in a cohort of overweight/obese and inactive adolescents and evaluated their potential associations with clinical and metabolic characteristics.</div></div><div><h3>Methods and results</h3><div>We performed a cross-sectional data analysis from 122 adolescents (11–17 years of age). We assessed body composition, cardiometabolic risk factors, biochemical variables, and physical fitness. The concentration of 55 cytokines was quantified in blood samples. The homeostasis model assessment insulin resistance (HOMA‐IR) and AST/ALT and TG/HDL ratios were calculated. IR adolescents as defined as HOMA-IR >2.5. The number of adolescents with IR in the study was 91 (66 % girls). In the IS group, after controlling for confounders, higher IL-15 levels were significantly associated with higher alanine aminotransferase levels and lower AST/ALT ratio, respectively (<em>Ps</em><0.05). In the same line, there were significantly higher alanine aminotransferase levels and lower AST/ALT ratio, respectively, with FGF-9 (<em>Ps</em><0.05). Likewise, higher alanine aminotransferase levels were significantly associated positively with HGF (<em>p</em>=0.045). Additionally, leptin levels are associated with six adiposity indexes (i.e., fat mass/height index, body fat, body mass index, android fat mass and gynoid fat mass) in overweight/obese adolescents with IR (<em>Ps</em><0.05).</div></div><div><h3>Conclusions</h3><div>These data may provide novel insights into the pathogenic mechanisms underlying IR in youth, offering new targets for prevention.</div></div>","PeriodicalId":51006,"journal":{"name":"Current Problems in Cardiology","volume":"50 1","pages":"Article 102925"},"PeriodicalIF":3.0,"publicationDate":"2024-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142606957","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mohammad Tanashat , Ahmed Zayed MD , Mohammed Ayyad MD , Mouath Abu Daoud , Mohammad Tabbalat , Obieda Altobaishat , Jana Nusier , F.N.U. Deepak , Yazan A. Al-Ajlouni MD, MPhil
{"title":"Current status and challenges of cardiac transplantation in the MENA region: A narrative review","authors":"Mohammad Tanashat , Ahmed Zayed MD , Mohammed Ayyad MD , Mouath Abu Daoud , Mohammad Tabbalat , Obieda Altobaishat , Jana Nusier , F.N.U. Deepak , Yazan A. Al-Ajlouni MD, MPhil","doi":"10.1016/j.cpcardiol.2024.102920","DOIUrl":"10.1016/j.cpcardiol.2024.102920","url":null,"abstract":"<div><h3>Introduction</h3><div>Cardiac transplantation has progressed markedly since 1967, with advances in immunosuppression, surgical techniques, and postoperative care that improve outcomes. However, challenges persist in the Middle East and North Africa (MENA) region due to unique cultural, economic, and infrastructural barriers. This review explores the status, innovations, and challenges of cardiac transplantation within MENA countries, comparing outcomes with global standards.</div></div><div><h3>Methodology</h3><div>A comprehensive literature search was conducted across PubMed, Web of Science, and Cochrane databases, including studies from inception to May 2024. Search terms targeted heart transplantation practices, challenges, and innovations in the MENA region. Articles not in English, non-human studies, and duplicates were excluded. Data extraction focused on patient demographics, transplantation outcomes, and barriers specific to the MENA context.</div></div><div><h3>Results</h3><div>Cardiac transplantation in MENA has made strides, with established programs in Saudi Arabia, the UAE, and Lebanon. Innovations like ex-vivo perfusion and Left Ventricular Assist Devices (LVAD) are improving transplant outcomes, yet organ shortages remain critical. Cultural and religious beliefs influence donation rates, and infrastructure varies widely, with disparities in healthcare resources across countries. Key barriers include low donor registration, inconsistent brain death definitions, and limited public awareness. Economic and infrastructure limitations further complicate access to advanced transplantation techniques.</div></div><div><h3>Conclusion</h3><div>While cardiac transplantation has evolved in MENA, significant barriers hinder widespread adoption. Enhancing public awareness, developing regional networks, and implementing standardized protocols can improve outcomes. Targeted immunosuppressive therapies and continued innovation in organ preservation are essential to advance cardiac transplantation in MENA.</div></div>","PeriodicalId":51006,"journal":{"name":"Current Problems in Cardiology","volume":"50 1","pages":"Article 102920"},"PeriodicalIF":3.0,"publicationDate":"2024-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142606965","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Siddig Ibrahim Abdelwahab , Manal Mohamed Elhassan Taha , Abdullah Farasani , Ahmed Ali Jerah , Saleh M Abdullah , Bassem Oraibi , Yasir Babiker , Hassan Ahmad Alfaifi , Amal Hamdan Alzahrani , Ahmed S. Alamer , Tawfeeq Altherwi , Ibrahim Abdel Aziz Ibrahim , Waseem Hassan
{"title":"Bibliometric analysis of ST elevation myocardial infarction research from 1933 to 2023: Focus on top 100 most-cited articles","authors":"Siddig Ibrahim Abdelwahab , Manal Mohamed Elhassan Taha , Abdullah Farasani , Ahmed Ali Jerah , Saleh M Abdullah , Bassem Oraibi , Yasir Babiker , Hassan Ahmad Alfaifi , Amal Hamdan Alzahrani , Ahmed S. Alamer , Tawfeeq Altherwi , Ibrahim Abdel Aziz Ibrahim , Waseem Hassan","doi":"10.1016/j.cpcardiol.2024.102923","DOIUrl":"10.1016/j.cpcardiol.2024.102923","url":null,"abstract":"<div><div>The primary objective of this study was to conduct a bibliometric analysis of the most influential papers on ST-Elevation Myocardial Infarction (STEMI). Using the Scopus database (October 2024), a targeted search was performed to identify relevant publications. Three retrieval options were considered based on the appearance of search terms. Precisely those documents were analyzed where the search terms appeared only in the title, allowing for a focused analysis of the most directly relevant studies. Next, from this search, the top 100 most cited papers, spanning from 1981 to 2018, were selected for detailed examination. Data analysis was conducted using VOSviewer and R Studio to provide insights into publication trends, author productivity, and the thematic focus of STEMI research. Author performance was evaluated through various bibliometric indicators, including total publications (TP), total citations (TC), h-index, g-index, m-index, HG composite, and Q2 index. Key metrics such as mean total citations per article (MeanTCperArt) and mean annual citation rate (MeanTCperYear) were also calculated. Thematic analysis of research topics was conducted using unigrams, bigrams, and trigrams, highlighting primary areas of focus across the most impactful STEMI studies. The results underscore significant trends in STEMI research, with highly cited papers shaping the field's evolution. This bibliometric approach provides valuable insights into research patterns, major contributors, and prevalent themes within STEMI literature.</div></div>","PeriodicalId":51006,"journal":{"name":"Current Problems in Cardiology","volume":"50 1","pages":"Article 102923"},"PeriodicalIF":3.0,"publicationDate":"2024-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142606962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Robinson Ramírez-Vélez , Miguel A. Pérez-Sousa , Emilio González-Jiménez , María Correa-Rodríguez , Juan Carlos Calderón-González , Albeiro Dávila-Grisales , Jacqueline Schmidt-RioValle
{"title":"Using different anthropometric indices of obesity as predictors for type 2 diabetes mellitus in older adults","authors":"Robinson Ramírez-Vélez , Miguel A. Pérez-Sousa , Emilio González-Jiménez , María Correa-Rodríguez , Juan Carlos Calderón-González , Albeiro Dávila-Grisales , Jacqueline Schmidt-RioValle","doi":"10.1016/j.cpcardiol.2024.102924","DOIUrl":"10.1016/j.cpcardiol.2024.102924","url":null,"abstract":"<div><h3>Background and aims</h3><div>There is some evidence that anthropometric measurements can be associated with the risk of type 2 diabetes mellitus (T2DM). Nevertheless, there is no comprehensive consensus on which anthropometric index is the best for evaluating the risk and predicting T2DM, especially in older adults. For this reason, we compared the performance of six indices for detecting T2DM in a population of Colombian older adults.</div></div><div><h3>Methods and results</h3><div>We conducted a cross-sectional study of 3453 older adults (≥60 years old; 2023 women), analysing demographic characteristics, biochemical markers, and anthropometric indices including body mass index (BMI), waist circumference (WC), waist-to-height ratio (WHtR), adiposity index (VAI), conicity index (C-Index), and body roundness index (BRI). T2DM was defined as fasting plasma glucose ≥126 mg/dl (≥7.0 mmol/l). All the anthropometric indices correlated significantly with the presence of T2DM. An analysis of the receiver operating characteristic curve showed that for men the VAI (AUC = 0.71; moderate ES (0.78); OR = 4.13), BMI (AUC = 0.68; moderate ES (0.68); OR = 3.38), and WC (AUC = 0.68; moderate ES (0.68); OR = 3.38) are the best predictors for identifying T2DM. For women, however, the WC (AUC = 0.63; ES = 0.46; OR = 2.34) and C-Index (AUC = 0.63; ES = 0.46; OR = 2.34) were better indicators for predicting T2DM. Cut-off points for all the anthropometric indices were provided.</div></div><div><h3>Conclusions</h3><div>In summary, the VAI, BMI, and WC in men, and WC and the C-Index in women have a moderate discriminating power for detecting T2DM in Colombian older adults, evidencing that these anthropometric indices are suitable screening tools for use in the elderly.</div></div>","PeriodicalId":51006,"journal":{"name":"Current Problems in Cardiology","volume":"50 1","pages":"Article 102924"},"PeriodicalIF":3.0,"publicationDate":"2024-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142607052","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Konstantin V. Zavadovsky MD, PhD, DrSc , Vyacheslav V. Ryabov MD, PhD, DrSc , Evgeny V. Vyshlov MD, PhD, DrSc , Olga V. Mochula MD, PhD , Maria Sirotina MD , Artur Kan MD , Alexander V. Mukhomedzyanov MD, PhD , Ivan A. Derkachev MD , Nikita S. Voronkov MD, PhD , Andrey V. Mochula MD, PhD , Alexandra S. Maksimova MD, PhD , Leonid N. Maslov MD, PhD, DrSc
{"title":"Intra-myocardial hemorrhage and cardiac microvascular injury in ischemia/reperfusion. A systematic review of current evidences","authors":"Konstantin V. Zavadovsky MD, PhD, DrSc , Vyacheslav V. Ryabov MD, PhD, DrSc , Evgeny V. Vyshlov MD, PhD, DrSc , Olga V. Mochula MD, PhD , Maria Sirotina MD , Artur Kan MD , Alexander V. Mukhomedzyanov MD, PhD , Ivan A. Derkachev MD , Nikita S. Voronkov MD, PhD , Andrey V. Mochula MD, PhD , Alexandra S. Maksimova MD, PhD , Leonid N. Maslov MD, PhD, DrSc","doi":"10.1016/j.cpcardiol.2024.102918","DOIUrl":"10.1016/j.cpcardiol.2024.102918","url":null,"abstract":"<div><div>The in-hospital mortality rate in acute myocardial infarction (AMI) remains high despite the undoubted achievements in treatment of this disease achieved in the last 40 years. The dangerous complications of AMI remain cardiac microvascular injury (CMI) and intramyocardial hemorrhage (IMH). IMH is a widespread pathology that occurs in 42 – 57% of patients with ST-segment elevation myocardial infarction and percutaneous coronary intervention. IMH is associated with larger infarct size and contractile dysfunction. IMH is accompanied by inflammation. The appearance of IMH is depending on the duration of ischemia and requires reperfusion of the heart. IMH is accompanied by contractile dysfunction and adverse remodeling of the heart. The most likely cause of IMH is CMI. Pretreatment with ATL-146e, melatonin, tanshinone IIA, relaxin, empagliflozin, dapagliflozin, and astragaloside IV can mitigate I/R-induced CMI. CMI is accompanied by an increase in the myocardial and plasma proinflammatory cytokine levels and also the downregulation of tight junction proteins in cardiac vascular endothelial cells. However, there is no convincing evidence that proinflammatory cytokines trigger CMI. An increase in the proinflammatory cytokine levels and CMI could be two independent processes.</div></div>","PeriodicalId":51006,"journal":{"name":"Current Problems in Cardiology","volume":"50 1","pages":"Article 102918"},"PeriodicalIF":3.0,"publicationDate":"2024-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142606795","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Effectiveness of Emotional-Focused Coping on Heart-Focused Anxiety in Patients Prior to Cardiac Catheterization.","authors":"Baneen Kamil Gatie, Hayder H Al-Hadrawi","doi":"10.1016/j.cpcardiol.2024.102917","DOIUrl":"https://doi.org/10.1016/j.cpcardiol.2024.102917","url":null,"abstract":"<p><strong>Background: </strong>Heart-focused anxiety involves a concentration on and a fear of cardiacrelated feelings and their anticipated negative outcomes (such as a life-threatening arrhythmia or abrupt cardiac death). It results in continuing concerns about heart function, avoiding activities that are thought to cause cardiac symptoms, and frequent behavior of requesting assistance in medical settings. This study aimed to measure the effectiveness of Emotional-Focused Coping on Heart-Focused Anxiety in Patients Prior to Cardiac Catheterization.</p><p><strong>Materials and methods: </strong>A quasi-experimental study using (pretest and posttest design with a control group). A purposive sampling technique was used to gather the study information from 120 patients prior to cardiac catheterization by using the beck anxiety inventory scale. The study is conducted in Al-Najaf Al-Ashraf Government, Al-Najaf Center for Cardiac Surgery and Trans-Catheter Therapy.</p><p><strong>Results: </strong>A statistically significant difference is found in participant's anxiety level prior to cardiac catheterization between the first measure before applying the coping techniques and the second measure after the application of coping strategies (Mean difference =13.500, P <0.0005).</p><p><strong>Conclusion: </strong>The emotional focused coping strategies are proving as easy interventions to apply and effective in terms of reducing the level of heart-focused anxiety prior to cardiac catheterization.</p>","PeriodicalId":51006,"journal":{"name":"Current Problems in Cardiology","volume":" ","pages":"102917"},"PeriodicalIF":3.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142569916","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Laís Manata Vanzella PhD, Felipe Ribeiro PhD, Maria Júlia Lopez Laurino PhD, Carolina Takahashi PhD, Franciele Marques Vanderlei PhD, Anne Kastelianne França da Silva PhD, Denise Brugnoli Balbi Dagostinho MSc, João Pedro Lucas Neves Silva MSc, Luiz Carlos Marques Vanderlei PhD
{"title":"Unveiling the link between physical parameters and safety in cardiac rehabilitation: Longitudinal observational study","authors":"Laís Manata Vanzella PhD, Felipe Ribeiro PhD, Maria Júlia Lopez Laurino PhD, Carolina Takahashi PhD, Franciele Marques Vanderlei PhD, Anne Kastelianne França da Silva PhD, Denise Brugnoli Balbi Dagostinho MSc, João Pedro Lucas Neves Silva MSc, Luiz Carlos Marques Vanderlei PhD","doi":"10.1016/j.cpcardiol.2024.102916","DOIUrl":"10.1016/j.cpcardiol.2024.102916","url":null,"abstract":"<div><h3>Objective</h3><div>To identify the associations between cardiorespiratory fitness and quadriceps muscle strength and the occurrence of minor adverse events in a cardiac rehabilitation (CR) program.</div></div><div><h3>Design</h3><div>Prospective cohort study.</div></div><div><h3>Setting</h3><div>Output of a CR programme for primary or secondary prevention of cardiovascular disease (CVD).</div></div><div><h3>Patients</h3><div>Seventy individuals who were diagnosed with CVD and/or risk factors and 7 who were excluded due to a low adherence rate in exercise sessions (<70%), 4 due to errors in oxygen consumption recorded during the cardiopulmonary exercise test (CPET) and 11 because they decided to withdraw from the study. The data of 38 participants were analyzed.</div></div><div><h3>Interventions</h3><div>Not applicable.</div></div><div><h3>Main outcome measures</h3><div>Quadriceps muscle strength was assessed by an isokinetic dynamometer and by a manual dynamometer. Functional capacity was assessed by the CPET and by a six-minute walk test (6MWT). Participants were monitored by a physiotherapist during 24 exercise sessions to identify and register adverse events.</div></div><div><h3>Results</h3><div>Significant associations were detected between adverse events and quadriceps muscle strength assessed by an isokinetic dynamometer (peak torque, B=-2.0(-2.0;0.0), p=0.047), between functional capacity assessed by the CPET (peak torque, B=-0.3(-2.4;0.0), p=0.019), between fatigue and functional capacity assessed by the CPET (VO2max, B=-1.3(-2.9;0.0), p=0.005) and between quadriceps muscle strength assessed by an isokinetic dynamometer (peak torque, B=-10.0(-2.7;0.0); p=0.010).</div></div><div><h3>Conclusions</h3><div>Lower functional capacity and quadriceps muscle strength seem to be associated with a greater incidence of adverse events during exercise sessions.</div></div>","PeriodicalId":51006,"journal":{"name":"Current Problems in Cardiology","volume":"50 1","pages":"Article 102916"},"PeriodicalIF":3.0,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142569955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Daniela Bacich , Chiara Tessari , Marco Andreis , Veronica Geatti , Irene Cattapan , Nicola Pradegan , Marny Fedrigo , Giovanni Di Salvo , Giuseppe Toscano , Annalisa Angelini , Gino Gerosa
{"title":"Heart transplantation in juvenile-onset systemic sclerosis with primary cardiac involvement: report of two cases and comprehensive literature review","authors":"Daniela Bacich , Chiara Tessari , Marco Andreis , Veronica Geatti , Irene Cattapan , Nicola Pradegan , Marny Fedrigo , Giovanni Di Salvo , Giuseppe Toscano , Annalisa Angelini , Gino Gerosa","doi":"10.1016/j.cpcardiol.2024.102891","DOIUrl":"10.1016/j.cpcardiol.2024.102891","url":null,"abstract":"<div><div>Juvenile onset systemic sclerosis is a rare chronic multisystem connective tissue disease characterized by skin induration, microangiopathy, autoimmune disturbances and widespread fibrosis of internal organs. Primary cardiac involvement in systemic sclerosis (SSc) is associated with a variable phenotype, including heart failure and arrhythmias, which lead to poor short-term prognosis. Isolated heart transplantation is a rare approach for the treatment of advanced heart failure in patients with systemic sclerosis. We report on two juvenile SSc patients receiving cardiac transplantation due to heart failure with malignant arrhythmias. One patient presented with severe dilated cardiomyopathy with recurrent ventricular tachycardia. Following the appearance of Raynaud phenomenon, he was subsequently diagnosed a rare form of systemic sclerosis sine scleroderma, without cutaneous manifestations or other organs involved. His cardiac condition was unresponsive to antiarrhythmic therapy and immunosuppression used to treat SSc, therefore he underwent successful heart transplantation. The second patient presented diffuse scleroderma with mild pulmonary, esophageal and renal involvement. While extracardiac manifestations were effectively kept under control with immunosuppressive therapy, cardiac involvement rapidly progressed with detection of fibrosis at cardiac magnetic resonance imaging and appearance of severe ventricular arrhythmia. Herein, an extensive multidisciplinary evaluation was pivotal in defining the entity and clinical stability of extracardiac involvement, and thus the patient could profit from heart transplantation. Our experience highlights the importance of considering heart transplantation in carefully selected SSc patients with primary cardiac involvement as a lifesaving procedure.</div></div>","PeriodicalId":51006,"journal":{"name":"Current Problems in Cardiology","volume":"50 1","pages":"Article 102891"},"PeriodicalIF":3.0,"publicationDate":"2024-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142560824","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}