
CJC Pediatric and Congenital Heart Disease, Год журнала: 2023, Номер 2(6), С. 253 - 255
Опубликована: Ноя. 7, 2023
More than 70 years after the description of its first successful surgical repair, tetralogy Fallot (ToF) remains a challenging but fascinating form congenital heart disease. Patients born with ToF can be expected to live into adulthood 30-year survival which exceeds 95%.1Blais S. Marelli A. Vanasse et al.The 30-Year Outcomes Tetralogy According Native Anatomy and Genetic Conditions.Can J Cardiol. 2021; 37: 877-886Abstract Full Text PDF PubMed Scopus (14) Google Scholar Nevertheless, residual cardiac lesions repair are nearly ubiquitous. These include pressure-loading or volume-loading conditions, sometimes both. Consequently considerable heterogeneity in sequelae seen wide spectrum presenting features.2Apitz C. Webb G.D. Redington A.N. Fallot.Lancet. 2009; 374: 1462-1471Abstract (402) This has made risk stratification difficult, especially as our understanding complex interaction between right left ventricle continues evolve. Indeed, there needs balance preventing long-term complications avoiding unnecessary interventions. However, remain many uncertainties pertaining optimal management strategy particularly asymptomatic individuals progressive changes imaging electrical parameters. In this focus issue CJCPC, we assembled collection articles that review state-of-the-art evidence management. Included important topics central malformation: genotyping, phenotyping, stratification, arrhythmias, sudden death, interventions surgery, access care, patient advocacy, prevention, trajectory failure, evolving field machine learning. The importance genetic applications patients is increasingly being recognized knowledge base domain rapidly expand. Bassett colleagues3Bassett A.S. Reuter M.S. Malecki Silversides Oechslin E. Clinically relevant considerations Fallot.CJC Pediatric Congenital Heart Disease. 2023; Abstract provide comprehensive overview evolution testing, genotype-phenotype correlations association profiling clinical outcomes. They also further detail contemporary practice potential for molecular personalized medicine. Cardiopulmonary exercise testing routinely used surveillance children adults repaired characterize cardiorespiratory fitness. While guidelines incorporate cardiopulmonary care algorithms given measures outcomes, thresholds intervention based on variety measurements available have not been clearly elucidated. Leonardi Cifra4 us thoughtful use various test parameters stratify adverse outcomes repair. Cardiac contributed greatly implications hemodynamic making anatomy one best-characterized lesion across Specifically, more 2 decades cardiovascular magnetic resonance (CMR) research provided deepening significance chamber enlargement, ventricular dysfunction, increased mass myocardial scar. issue, two expand population. Crean reviews multi-modality imaging, contributions from CMR computed tomographic imaging.5 illustrative introductory article followed by an elegant thought-provoking Ghonim Babi-Naryan,6 detailing including established scores emerging role artificial intelligence. Finally, Schäfer Mawad7Schäfer M, Mawad W. Advanced Imaging Technologies Assessing Fallot: Insights Into Flow Dynamics. CJC 2023.Google explore promise advanced technologies such 4D-Flow high-frame-rate echocardiography. Studying quantifying blood flow dynamics energetics enable better assess impact hemodynamics caused patterns, energy loss vorticity. authors discuss how these tools may allow earlier detection suboptimal compared conventional markers, help guide treatment. face impacting their health quality life. bradyarrhythmias, postoperative complete atrioventricular block, often necessitating lifelong pacing, junctional ectopic tachycardia tachyarrhythmias, intra-atrial reentry tachycardia, non-automatic focal atrial fibrillation, life-threatening arrhythmias. arrhythmias pose significant risk, underscoring need thorough screening effective high-risk individuals. Vô collaborators8Vô Dib N. Bartoletti al.Navigating Arrhythmias Throughout Lifespan: A Case-based Review.CJC specific management, highlighting connection Advances death involve proactive assessment Our predictive factors improved over past 25 years, shifting towards prediction incorporates extensive data. Kakarla al.9Kakarla J. Denham N.C. Ishikita Alonso-Gonzalez R. Nair K. Risk Stratification Sudden Death Repaired delve strategies ToF. Accurate characterization disease complexity anatomical significantly affects emphasizing routine stratification. informs primary prevention measures, like defibrillator implantation, prompts consideration invasive approaches, ablation pulmonary valve replacement. Multidisciplinary teams experts play crucial assessing stratifying risk. collaboration essential determine interventional addressing staged repairs, re-interventions, replacement throughout patient's Mosa collaborators10Tchervenkov C.I. AL Bernier P.L. Considerations timing existing persisting controversies regarding approached both symptomatic patients. transcatheter lifespan discussed Wong al.11Wong Shorofsky M. Lim D.S. Catheter-based Interventions Across Lifespan.CJC treatments before offering palliative procedures through catheter-based methods. Following initial relief outflow tract obstruction, still varying degrees regurgitation, Primary becoming viable option, alongside replacement, due similar safety profile effectiveness. introduction self-expanding platforms expanded who treated percutaneously. With technological advances growing expertise, greater Vanderlaan Barron12Vanderlaan RD, Barron DJ. Optimal Surgical Management Fallot. outline represents challenge multidisciplinary decision-making will maximize survival, minimize reintervention preserve function lifespan. require specialized care. Mackie collaborators13Mackie AS, Bravo-Jaimes K, Keir Sillman C, Kovacs AH. Access Specialized Care Lifespan reviewed unequal. reality inconsistent paradoxically those it most. offer recommendations improve step reducing morbidity improving last decades, very fortunate paradigm shift restriction prescription promotion no exception. Buchanan collaborators,14Buchanan Spence Khoury Physical Activity Promotion Among Individuals current physical activity Ross Verstappen15Ross S, Verstappen Role Disease Patient Organizations Advocacy, Resources, Support Lifespan. perspective advocacy organizations had patients' lives. Speaking common voice reflecting lived experience disease, uniquely successfully influence priorities, advocate programs resources, ensure issues discrimination mental support prioritized policy-makers providers. chronic condition truly integrated approach great trajectories follow. collaborators16Alonso-Gonzalez R, Ganni E, Ho al. Of Focus On Right Ventricle. map journey start at cellular microarchitectural levels specificities ventricle, mechanisms adaptation remodeling lesions. then identify insidious onset therapies – limitations dealing trying prevent it. Jacquemyn al.17Jacquemyn X. Kutty Manlhiot Lifelong Impact Artificial Intelligence Clinical Prediction Models opportunities challenges intelligence learning diagnosis prognostic There several promising developments machine-learning-based diagnostic aid Perhaps most encouraging valuable feature ability markers apparent traditional analyses, datasets too big analyzed humans. Many hurdles overcome incorporated suspect early adopters get head enhancement when so different profiles considered. population adult ToF, want children. Garagiola collaborators18Garagiola ML, Thorne SA. Pregnancy pregnancy-related maternal fetal rightfully underscore while team optimizes outcome, tolerate demands pregnancy even presence valvular regurgitation. around reproductive should teenage age-appropriate discussions fertility, childbearing contraception. archetype moderately where huge strides recent era excellent. And yet, much learn accomplish people living enjoy same life, tolerance life expectancy general precise phenotyping large cohorts follow-up, structural functional parameters, reintervention-related morbidity, patient-reported outcome measures. Priorities refinement improvement personalization interventions, optimization material reconstruction, advancing precision medicine genomics approaches tailor
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