Pediatric CardiologyPub Date : 2025-08-01Epub Date: 2024-07-22DOI: 10.1007/s00246-024-03589-4
Mariam Mohamed, Ashraf Harahsheh, Nadine Choueiter, Hala M Agha, Hanifa Alrabte, Sima Y Abu Al-Saoud, Hesham Al-Saloos, Khalfan Al Senaidi, Raed Alzyoud, Zainab Al Awadhi, Reda Belbouab, Kenza Bouayed, Asma Bouaziz, Mona El Ganzoury, Zohra Fitouri, Alyaa Kotby, Mohamed S Ladj, Mohammed Mokhtar Bekkar, Najat Rugige, Aso Faeq Salih, Mohamed Sulaiman, Nagib Dahdah
{"title":"Advancing Kawasaki Disease Research in the Arab World: Scoping Literature Review Analysis with Emphasis on Giant Coronary Aneurysms.","authors":"Mariam Mohamed, Ashraf Harahsheh, Nadine Choueiter, Hala M Agha, Hanifa Alrabte, Sima Y Abu Al-Saoud, Hesham Al-Saloos, Khalfan Al Senaidi, Raed Alzyoud, Zainab Al Awadhi, Reda Belbouab, Kenza Bouayed, Asma Bouaziz, Mona El Ganzoury, Zohra Fitouri, Alyaa Kotby, Mohamed S Ladj, Mohammed Mokhtar Bekkar, Najat Rugige, Aso Faeq Salih, Mohamed Sulaiman, Nagib Dahdah","doi":"10.1007/s00246-024-03589-4","DOIUrl":"10.1007/s00246-024-03589-4","url":null,"abstract":"<p><p>To evaluate giant aneurysms (GiAn) prevalence in Arab countries and examine contributing factors; and to review Kawasaki disease (KD) publication trends and collaborations among Arab nations. A scoping literature review was conducted to analyze the publications across the Arab world, spanning 16 countries from 1978 to 2023. The collected articles were a combination of database search with a call on Kawasaki Disease Arab Initiative (Kawarabi) members to share non-PubMed publications. Over 45 years, 50 articles originated from the Arab Countries with a 30% average annual growth rate in KD research output. Publications were evenly split between case reports (42%) and institutional series (52%). Research productivity lagged in developing nations with UAE, KSA and Egypt, contributed to 64% of total publications. Among 26 institutional series, 256 coronary artery aneurysms (CAA) from a total of 1264 KD cases were reported. Of those, 25 CAA were GiAn (prevalence 1.43% [range 0-12.5%]). The initial KD misdiagnosis rate was 4%, and incomplete KD (iKD) averaged 10.6%. Series (38.5%) that did not report iKD correlated with a higher prevalence of CAA, but not of GiAn. Longer fever duration emerged as a pivotal factor for GiAn (OR 5.06, 95%CI 1.51-17). This review unveils the research landscape of KD in the Arab world over 45 years. Initial misdiagnosis, untreated cases, delayed diagnosis and underreporting of iKD are contributing factors for an underestimated epidemiology, explaining the higher GiAn prevalence. This calls for strategic interventions to enhance KD research in these countries, aligning with Kawarabi's mission.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1448-1457"},"PeriodicalIF":1.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141734828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pediatric CardiologyPub Date : 2025-08-01Epub Date: 2024-07-06DOI: 10.1007/s00246-024-03573-y
Scott M Leopold, Diane H Brown, Xiao Zhang, Xuan T Nguyen, Awni M Al-Subu, Krisjon R Olson
{"title":"Early Impressions and Adoption of the AtriAmp for Managing Arrhythmias Following Congenital Heart Surgery.","authors":"Scott M Leopold, Diane H Brown, Xiao Zhang, Xuan T Nguyen, Awni M Al-Subu, Krisjon R Olson","doi":"10.1007/s00246-024-03573-y","DOIUrl":"10.1007/s00246-024-03573-y","url":null,"abstract":"<p><p>AtriAmp is a new medical device that displays a continuous real-time atrial electrogram on telemetry using temporary atrial pacing leads. Our objective was to evaluate early adoption of this device into patient care within our pediatric intensive care unit (PICU). This is a qualitative study using inductive analysis of semi-structured interviews to identify dominant themes. The study was conducted in a single-center, tertiary, academic 21-bed mixed PICU. The subjects were PICU multidisciplinary team members (Pediatric Cardiac Intensivists, PICU Nurse Practitioners, PICU nurses and Pediatric Cardiologists) who were early adopters of the AtriAmp (n = 14). Three prominent themes emerged: (1) Accelerated time from arrhythmia event to diagnosis and treatment; (2) Increased confidence in the accuracy of providers' arrhythmia diagnosis; and (3) Improvement in the ability to educate providers about post-operative arrhythmias. Providers also noted some learning curves, but none compromised medical care or clinical workflow. Insights from early adopters of AtriAmp signal the need for simplicity and fidelity in new PICU technologies. Our research suggests that such technologies can be pivotal to the support and growth of multi-disciplinary teams, even among those who do not participate in early implementation. Further research is needed to understand when and why novel technology adoption becomes widespread in high-stakes settings.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1592-1599"},"PeriodicalIF":1.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11701211/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141545142","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pediatric CardiologyPub Date : 2025-08-01Epub Date: 2024-07-05DOI: 10.1007/s00246-024-03566-x
Stina Manhem, Michal Odermarsky, Håkan Wåhlander, Britt-Marie Ekman-Joelsson
{"title":"Pulmonary Atresia with Intact Ventricular Septum, a National Comparison Between Interventional and Surgical Approach, in Combination with a Systemic Literature Review.","authors":"Stina Manhem, Michal Odermarsky, Håkan Wåhlander, Britt-Marie Ekman-Joelsson","doi":"10.1007/s00246-024-03566-x","DOIUrl":"10.1007/s00246-024-03566-x","url":null,"abstract":"<p><p>This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS) treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention. Additionally, we conducted a systematic literature review on PA-IVS treatment. All neonates born in Sweden with PA-IVS between 2007 and 2019 were screened for inclusion. The inclusion criterion was decompression of the right ventricle for initial intervention. Medical records were reviewed, as well as the initial preoperative angiogram, and the diagnostic echocardiogram. Comparisons between groups were performed with Mann-Whitney U-test and Fisher´s exact test. A systematic literature review of original studies regarding treatment of PA-IVS (2002 and onward) was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, to assess the outcomes of patients with PA-IVS. 34 (11 females) patients were included, 18 in group A and 16 in group B. There was no mortality in either group. Follow-up time ranged from 2 to 15 years (median 9). All attempted perforations in group A were successful, and 16 out of 18 patients reached biventricular circulation. In the surgical group 15 out of 16 patients reached biventricular circulation. The literature review presented heterogeneity in standards for treatment. This retrospective population-based multicenter study demonstrates that both catheter-based intervention and heart surgery are safe procedures. Our results are comparable to, or exceed, those in the systematic literature review. The systematic literature review displays a great heterogeneity in study design, with no definitive golden standard treatment.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1523-1536"},"PeriodicalIF":1.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12296880/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141535023","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pediatric CardiologyPub Date : 2025-08-01Epub Date: 2024-07-04DOI: 10.1007/s00246-024-03564-z
Robert Spencer, Anita I Sen, David O Kessler, Kristina Salabay, Tammy Compagnone, Yun Zhang, Tarif A Choudhury
{"title":"Critical Event Checklists for Simulated In-Hospital Dysrhythmias in Children with Heart Disease.","authors":"Robert Spencer, Anita I Sen, David O Kessler, Kristina Salabay, Tammy Compagnone, Yun Zhang, Tarif A Choudhury","doi":"10.1007/s00246-024-03564-z","DOIUrl":"10.1007/s00246-024-03564-z","url":null,"abstract":"<p><p>Children with heart disease are at increased risk of unstable dysrhythmias and in-hospital cardiac arrest (IHCA). Clinician adherence to lifesaving processes of care is an important contributor to improving patient outcomes. This study evaluated whether critical event checklists improve adherence to lifesaving processes during simulated acute events secondary to unstable dysrhythmias. A randomized controlled trial was conducted in a cardiac ward in a tertiary care, academic children's hospital. Unannounced simulated emergencies involving dysrhythmias in pediatric patients with underlying cardiac disease were conducted weekly. Responders were pediatric and anesthesiology residents, respiratory therapists, and bedside registered nurses. Six teams were randomized into two groups-three received checklists (intervention) and three did not (control). Each team participated in four simulated scenarios over a 4-week pediatric cardiology rotation. Participants received a brief slideshow presentation, which included a checklist orientation, at the start of their rotation. Simulations were video and audio recorded and those with three or more participants were included for analysis. The primary outcome was team adherence to lifesaving processes, expressed as the percentage of completed critical management steps. Secondary outcomes included participant perceptions of the checklist usefulness in identifying and managing dysrhythmias. We used generalized estimating equations (GEE) models, which accounted for clustering within groups, to evaluate the effects of the intervention. A total of 24 simulations were conducted; one of the 24 simulations was excluded due to an insufficient number of participants. In our GEE analysis, 81.21% (78.96%, 83.47%) of critical steps were completed with checklists available versus 68.06% (59.38%, 76.74%) without checklists (p = 0.004). Ninety-three percent of study participants reported that they would use the checklists during an unstable dysrhythmia of a child with underlying cardiac disease. Checklists were associated with improved adherence to lifesaving processes during simulated resuscitations for unstable pediatric dysrhythmias. These findings support the use of scenario specific checklists for the management of unstable dysrhythmias in simulations involving pediatric patients with underlying cardiac disease. Future studies should investigate whether checklists are as effective in actual pediatric in-hospital emergencies.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1505-1513"},"PeriodicalIF":1.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141535059","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pediatric CardiologyPub Date : 2025-08-01Epub Date: 2024-07-17DOI: 10.1007/s00246-024-03580-z
Nina Morgan, Dalia Lopez-Colon, Lindsey Brinkley, Arun Chandran, Dipankar Gupta
{"title":"Propranolol Therapy in Tetralogy of Fallot: Treating the Echocardiogram or Treating the Patient.","authors":"Nina Morgan, Dalia Lopez-Colon, Lindsey Brinkley, Arun Chandran, Dipankar Gupta","doi":"10.1007/s00246-024-03580-z","DOIUrl":"10.1007/s00246-024-03580-z","url":null,"abstract":"<p><p>Hypercyanotic spells are one of the defining clinical features of Tetralogy of Fallot (TOF). Limited data exist on peak Doppler right ventricular outflow tract (RVOT) gradient as a risk factor for the development of hypercyanotic spells, frequency of prophylactic use of propranolol based on peak RVOT gradient, and its impact on preventing the occurrence of hypercyanotic spells. We aimed to quantify peak RVOT gradients as measured on transthoracic echocardiography in infants with unrepaired TOF and assess for correlation with clinical symptoms of hypercyanotic spells. We also assessed the frequency of pre-operative use of propranolol, indication for medication initiation, and occurrence of hypercyanotic spells with or without propranolol use. Retrospective analysis was performed on patients at our institution who were born between February 1, 2011 and May 31, 2023. Patients were excluded if they were maintained on prostaglandin infusion or underwent palliative shunt placement or balloon valvuloplasty prior to complete surgical repair. Demographics, occurrence of hypercyanotic spells, propranolol use, peripheral oxygen saturation, age at surgical repair, and peak RVOT gradient at the time of propranolol initiation were collected from the electronic medical record. If no propranolol use was recorded, the single highest maximum RVOT gradient prior to surgery was collected. 203 patients were identified, of which 92 patients were included in analysis. Thirty-six (39%) patients received propranolol and 19% of patients developed hypercyanotic spells prior to surgery. Patients with higher peak RVOT gradients were more likely to be started on propranolol even in the absence of overt symptoms, and they also demonstrated more systemic desaturation. Additionally, peak RVOT gradient was found to be a poor predictor for the development of hypercyanotic spells. Wide clinical variation exists in the prophylactic use of propranolol for prevention of hypercyanotic spells. Peak RVOT gradient is not a reliable tool for prophylactic propranolol initiation to prevent hypercyanotic spells.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1643-1648"},"PeriodicalIF":1.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141627312","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pediatric CardiologyPub Date : 2025-08-01Epub Date: 2024-12-26DOI: 10.1007/s00246-024-03757-6
Sohaihra Khan
{"title":"Response to \"Screening for LifeThreatening Arrhythmia in Asymptomatic Patients After Paediatric Cardiac Surgery: A Single Centre Retrospective Analysis of 790 Prehospitaldischarge 24 h Holter Electrocardiogram Recordings\".","authors":"Sohaihra Khan","doi":"10.1007/s00246-024-03757-6","DOIUrl":"10.1007/s00246-024-03757-6","url":null,"abstract":"<p><p>To the editor! We read the study published in your journal with great enthusiasm and appreciate the authors' efforts to perform the research [1]. It sheds light on the discussed cause and aids comprehension of the topic at hand. For sure, it provides the groundwork for future research; nevertheless, we have a few doubts that have caused us to write this letter, and we are confident that if these doubts were addressed, the overall output of the study would have increased manifolds.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1767-1768"},"PeriodicalIF":1.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142896602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pediatric CardiologyPub Date : 2025-08-01Epub Date: 2025-02-22DOI: 10.1007/s00246-025-03804-w
Arafat Hassan Tariq, Huzaifa Ali
{"title":"Comment on: Evaluation of Cardiac Function in Children Undergoing Liver Transplantation.","authors":"Arafat Hassan Tariq, Huzaifa Ali","doi":"10.1007/s00246-025-03804-w","DOIUrl":"10.1007/s00246-025-03804-w","url":null,"abstract":"<p><p>In response to the article \"Evaluation of Cardiac Function in Children Undergoing Liver Transplantation\" by Bansal et al., I admire the authors for their valuable insights in evaluating biventricular heart function in pediatric patients undergoing Liver Transplantation. However, I bring up several key concerns regarding study's methodology. The diversity of the pediatric cirrhosis population is limited by the study's small sample size, which consists of only 35 children patients. Advanced diagnostic techniques including tissue Doppler echocardiography and cardiovascular magnetic resonance (CMR), which are valuable for diagnosis of heart diseases, are not included in the study. The lack of a reference group of patients without cirrhosis makes it more difficult to confirm whether the cardiac dysfunction shown is unique to cirrhosis or indicates other chronic disease. Additionally, the study does not provides information regarding the outcomes following liver transplantation especially reversibility of cardaic abnormalities.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1758-1759"},"PeriodicalIF":1.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476745","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rita Blandino, Lucia Manuri, Pasqualina Bruno, Laura Ragni, Enrico Piccinelli, Mara Pilati, Micol Rebonato, Gianluca Brancaccio, Gianfranco Butera, Antonella Santilli, Lorenzo Galletti, Roberta Iacobelli
{"title":"Correction: The Use of Pulmonary Vasodilators in Pediatric Patients with Single-Ventricle Palliation: A Ten-Year Experience in a Tertiary Care Center.","authors":"Rita Blandino, Lucia Manuri, Pasqualina Bruno, Laura Ragni, Enrico Piccinelli, Mara Pilati, Micol Rebonato, Gianluca Brancaccio, Gianfranco Butera, Antonella Santilli, Lorenzo Galletti, Roberta Iacobelli","doi":"10.1007/s00246-025-03900-x","DOIUrl":"10.1007/s00246-025-03900-x","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1769"},"PeriodicalIF":1.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144234756","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pediatric CardiologyPub Date : 2025-08-01Epub Date: 2024-07-15DOI: 10.1007/s00246-024-03572-z
Matthias Walser, Leonie Arnold, Guido Mandilaras, Christoph Funk, Robert Dalla-Pozza, Joseph Pattathu, Nikolaus A Haas, André Jakob
{"title":"Fontan Circulation and Aortic Stiffness: Insights into Vascular Dynamics and Haemodynamic Interplay.","authors":"Matthias Walser, Leonie Arnold, Guido Mandilaras, Christoph Funk, Robert Dalla-Pozza, Joseph Pattathu, Nikolaus A Haas, André Jakob","doi":"10.1007/s00246-024-03572-z","DOIUrl":"10.1007/s00246-024-03572-z","url":null,"abstract":"<p><p>Increased aortic stiffness predisposes cardiac afterload and influences cardiac function. Congenital heart diseases involving aortic arch malformation and extended cardiovascular surgery, i.e. univentricular heart diseases, can lead to increased aortic stiffness. This study aimed to investigate whether Fontan patients (FO) have increased aortic stiffness within distinct aortic segments, and whether these parameters relate to Fontan-specific haemodynamics. In a prospective case-control study, 20 FO and 49 heart-transplanted control subjects with biventricular circulation underwent invasive cardiac catheterisation. We invasively measured pulse wave velocity (PWV) in the ascending aorta and along the entire aorta. Haemodynamic parameters, including end-diastolic pressure, pulmonary artery pressure, the cardiac index and systemic vascular resistance index were also assessed. FO exhibited significantly higher ascending aorta PWV (aPWV) than controls (FO: 7.2 ± 2.4 m/s|Controls: 4.9 ± 0.7 m/s, p < 0.001) and compared to the inner group central aorta PWV (cPWV; FO: 5.5 ± 1.2 m/s|Controls: 5.3 ± 1.0 m/s). Multivariate analysis confirmed this aPWV elevation in FO even after adjusting for age and BMI. aPWV and cPWV were almost identical within the control group. Correlation analyses revealed associations between cPWV and blood pressure in controls, while correlations were less apparent in FO. We detected no significant association between the aPWV and other haemodynamic parameters in any of our groups. FO exhibit increased aPWV, indicating specific vascular stiffness in the ascending aorta, while their overall aortic stiffness remains comparable to controls. Further research is needed to understand the implications of these findings on Fontan circulation and long-term cardiovascular health.</p><p><strong>Central message: </strong>Fontan patients show increased aortic arch pulse wave velocity, suggesting specific vascular stiffness.</p><p><strong>Perspective statement: </strong>Our study offers rare insights into pulse wave velocity in Fontan patients, highlighting increased arterial stiffness in the aortic arch. Vascular stiffness was particularly increased in the area of surgical reconstruction. This indicates the need for further research on vascular stiffness in Fontan circulation to understand its impact on cardiovascular health.</p><p><strong>Clinical trial registration: </strong>German clinical trial registration, DRKS00015066.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1581-1591"},"PeriodicalIF":1.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12296750/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141616916","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pediatric CardiologyPub Date : 2025-08-01Epub Date: 2024-06-25DOI: 10.1007/s00246-024-03562-1
Zhangwei Wang, Kai Ma, Shoujun Li
{"title":"Long-Term Outcomes of Surgical Repair of Supracardiac Total Anomalous Pulmonary Venous Connection.","authors":"Zhangwei Wang, Kai Ma, Shoujun Li","doi":"10.1007/s00246-024-03562-1","DOIUrl":"10.1007/s00246-024-03562-1","url":null,"abstract":"<p><p>The conventional surgery (CS) of supracardiac total anomalous pulmonary venous connection (TAPVC) is not always effective particularly in the setting of complex anatomy such as the mixed variety of TAPVC. This study aimed to analyze the outcomes of surgical treatment of supracardiac TAPVC and determine the optimal strategy. From December 2009 to December 2023, patients with supracardiac TAPVC undergoing surgical repair in our institution were included. The Kaplan-Meier curve was used to demonstrate the survival estimates. The Cox proportional hazard model was used to identify risk factors for death and postoperative pulmonary venous obstruction (PVO). One hundred and eighty-three patients with supracardiac TAPVC underwent surgical repair [CS group, n = 102; modified L-shaped incision technique (MLIT) group, n = 81]. There were 8 in-hospital deaths and 16 late deaths. The survival rates at 1, 5, and 10 years were 89.0%, 85.0%, and 85.0%, respectively in the whole cohort. Multivariable analysis showed that lower weight (P = 0.031), prolonged CBP time (P = 0.007), preoperative PVO (P = 0.020), and emergency surgery (P = 0.001) were incremental risk factors for death, but using the MLIT was a protective factor for death (p = 0.028). In the CS group, patients with emergency operation had worse survival than patients with elective surgery (P < 0.001). However, in the MLIT group, patients with emergency operation had comparable survival to patients with elective surgery (P = 0.332). Postoperative PVO occurred in 30 patients. Fourteen patients underwent PVO-related reintervention. In the whole cohort, freedom from postoperative PVO at 1, 5, and 10 years were 87.5%, 80.6%, and 80.6%, respectively. Patients who underwent MLIT repair had a lower incidence of postoperative PVO (P < 0.001), and PVO-related reintervention (P = 0.019). Neonates(P = 0.033), aortic cross-clamp time (P = 0.012), preoperative PVO (P = 0.002), and using the CS (P = 0.005) were associated with postoperative PVO. In terms of postoperative PVO, MLIT had a protective effect compared with CS. In the CS group, Infant and Children patients had better freedom from postoperative PVO than Neonate patients (P < 0.001). However, in the MLIT group, Neonate patients had comparable freedom from postoperative PVO to Infant and Children patients (P = 0.332). The MLIT can achieve satisfactory outcomes for supracardiac TAPVC repair. Compared with CS, the MLIT was significantly associated with decreased death, postoperative PVO, and PVO-related reintervention. It is especially significant in improving the survival rate of patients undergoing emergency surgery and reducing the incidence of postoperative PVO in neonatal patients.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1474-1487"},"PeriodicalIF":1.4,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141451128","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}