Article
Medicine, General & Internal
Agnieszka Bartczak-Rutkowska, Lidia Tomkiewicz-Pajak, Katarzyna Kawka-Paciorkowska, Natalia Bajorek, Aleksandra Cieplucha, Mariola Ropacka-Lesiak, Olga Trojnarska
Summary: A study investigated the maternal and fetal outcomes of women who had undergone the Fontan procedure in two Polish medical centers. Among 26 pregnancies, 11 ended in miscarriages and 15 resulted in 16 live births. Fetal complications were observed in 9 live births, with prematurity being the most common. Obstetrical complications occurred in 6 pregnancies, including placental abruption, premature rupture of membranes, and antepartum hemorrhage. Specialized care from experienced cardiologists and obstetricians is necessary for these women.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Cardiac & Cardiovascular Systems
Salvatore Giannico, Matteo Trezzi, Nicoletta Cantarutti, Giulia Cafiero, Lucilla Rava, Rachele Adorisio, Gianluca Brancaccio, Sonia Albanese, Fabrizio Drago, Adriano Carotti, Antonio Amodeo, Lorenzo Galletti
Summary: Most patients undergoing extracardiac Fontan operation have satisfactory long-term survival, but there are still risks of adverse events and late failure. The risk of late adverse events and heart transplant is associated with the preoperative diagnosis of patients.
EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY
(2022)
Editorial Material
Cardiac & Cardiovascular Systems
Shinichiro Oda, Satoshi Fujita, Hideki Tatewaki, Akira Shiose
Summary: This article reports a case of coronary artery compression associated with aortic root dilation in a patient with congenital heart disease, and introduces an effective method of treatment using valve-sparing aortic root replacement.
ANNALS OF THORACIC SURGERY
(2022)
Article
Cardiac & Cardiovascular Systems
Emma Payne, Frances Garden, Yves D'Udekem, Zoe McCallum, Helen Wightman, Diana Zannino, Dominica Zentner, Rachael Cordina, Robert Weintraub, Thomas G. Wilson, Julian Ayer
Summary: Poor infant growth in patients with a single ventricle is associated with increased long-term Fontan failure. Establishing BMI trajectory models in these patients helps determine optimal BMI targets for patient outcomes.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2022)
Article
Medicine, General & Internal
Ilona Palyga-Bysiecka, Aneta Maria Polewczyk, Maciej Polewczyk, Elzbieta Kolodziej, Henryk Mazurek, Andrzej Pogorzelski
Summary: This study examines the clinical course, therapeutic measures, outcome, and follow-up of plastic bronchitis (PB) in children with congenital heart disease (CHD) and children without cardiac problems. It found that there are differences in the course of the disease in patients without CHD, such as a higher age of first episode of PB, the location of plastic casts, and a stronger role of inflammatory factors and mechanisms.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
Makoto Miyake, Jiro Sakamoto, Hirokazu Kondo, Atsushi Iwakura, Hiraku Doi, Toshihiro Tamura
Summary: This study aimed to investigate the long-term outcomes of patients with single-ventricle physiology after the Glenn procedure without the subsequent Fontan procedure. The study found that the 40-year overall survival after the Glenn procedure in patients with single-ventricle physiology was 40.3%. Dominant left ventricular morphology may be associated with better long-term overall survival than dominant right ventricular morphology.
EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY
(2023)
Article
Surgery
Juliet Emamaullee, Sean Martin, Cameron Goldbeck, Brittany Rocque, Arianna Barbetta, Rohit Kohli, Vaughn Starnes
Summary: This study analyzed the long-term outcomes of patients post-Fontan procedure and found an increased all-cause mortality in Hispanic population and a correlation between the incidence of FALD and age. This emphasizes the need for specific liver surveillance strategies in patients post-Fontan.
Article
Cardiac & Cardiovascular Systems
Maren Ravndal, Lars Idorn, Anne Kathrine Moller Nielsen, Benjamin Kelly, Kim Gjerum Nielsen, Dorte Guldbrand Nielsen, Vibeke Hjortdal
Summary: This study examines the natural evolution of exercise capacity over a 10-year period in Danish Fontan patients and finds that their exercise capacity remains stable during this time. The study also shows that patients with higher levels of physical activity have higher predicted peak VO2 values in exercise tests. This research provides valuable insights for rehabilitation and exercise programs for Fontan patients.
INTERNATIONAL JOURNAL OF CARDIOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Masamichi Ono, Melchior Burri, Benedikt Mayr, Lisa Anderl, Martina Strbad, Julie Cleuziou, Alfred Hager, Juergen Hoerer, Rudiger Lange
Summary: This study evaluated the outcomes of BCPS and identified risk factors for failed Fontan completion. The study found that hypoplastic left heart syndrome, unbalanced atrioventricular septal defect, high pulmonary artery pressure, and reduced ventricular function were risk factors for failure to successfully complete the Fontan procedure.
ANNALS OF THORACIC SURGERY
(2021)
Review
Pediatrics
Guido Mandilaras, Zora Meyer, Richard Muehlberg, Annabell Braun, Nikolaus A. Haas, Andre Jakob, Robert Dalla Pozza, Moritz Wildgruber, Marcus Fischer
Summary: This article introduces the technical details and preliminary results of performing TJLB in Fontan patients. TJLB is an attractive alternative method for obtaining liver specimens in the scope of FALD care. It can be performed safely with very low additional time expenditure.
FRONTIERS IN PEDIATRICS
(2023)
Article
Pediatrics
Julia Moosmann, Christian Schroeder, Robert Cesnjevar, Kathrin Rottermann, Annika Weigelt, Sven Dittrich
Summary: This study demonstrates that NLR and PLR increase over time after Fontan surgery and are associated with the development of lymphatic abnormalities after TCPC. These ratios have potential clinical significance in evaluating the prognosis of single ventricle patients.
FRONTIERS IN PEDIATRICS
(2021)
Article
Cardiac & Cardiovascular Systems
Francisco Buendia-Fuentes, Blanca Gordon-Ramirez, Laura Dos Subira, Pablo Meras, Pastora Gallego, Ana Gonzalez, Raquel Prieto-Arevalo, Teresa Segura, Maria J. Rodriguez-Puras, Silvia Montserrat, Fernando Sarnago-Cebada, Andres Alonso-Garcia, Jose M. Oliver, Joaquin Rueda-Soriano
Summary: This study found that adult patients with single ventricle physiology without Fontan palliation exhibit a high mortality rate and frequent major cardiovascular events. At least moderate atrioventricular valve regurgitation, thrombocytopenia, renal dysfunction, and QRS duration > 120 ms at baseline visit are associated with an increased risk of mortality.
CANADIAN JOURNAL OF CARDIOLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Heather M. M. Giacone, Henry Chubb, Anne M. M. Dubin, Kara S. S. Motonaga, Scott R. R. Ceresnak, William R. R. Goodyer, Debra Hanisch, Anthony V. V. Trela, Nicole Boramanand, Erin Lencioni, Derek Boothroyd, Anna Graber-Naidich, Gail Wright, Christiane Haeffele, Seth A. A. Hollander, Doff B. B. McElhinney, Michael Ma, Frank L. L. Hanley, Sharon Chen
Summary: This study assessed the incidence of ventricular arrhythmias (VAs) in Fontan patients and their implications on transplant-free survival. The results showed that 11% of patients were diagnosed with VA, and VA was highly associated with the risk of transplant or death. Older age at Fontan and a history of right ventricular to pulmonary artery conduit were risk factors for VA. A diagnosis of VA in Fontan patients should lead to increased clinical surveillance.
CIRCULATION-ARRHYTHMIA AND ELECTROPHYSIOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Valtteri Muroke, Mikko Jalanko, Jari Haukka, Juha Sinisalo
Summary: This study aimed to evaluate the long-term mortality and cause-specific mortality of patients with atrial septal defect (ASD) in a nationwide cohort. The results showed that patients with ASD had higher overall mortality, with increased cause-specific mortality in congenital malformations, stroke, and heart diseases.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2023)
Article
Cardiac & Cardiovascular Systems
Jianbin Li, Li Ma, Minghui Zou, Wenlei Li, Xinxin Chen, Yanqin Cui, Xiaoyan Hu
Summary: NT-proBNP is a valuable marker for mid and long-term follow-up after a modified Fontan procedure, with better outcomes seen in children with left ventricular and biventricular type of single ventricle physiology compared to right ventricular and univentricular type.
CARDIOLOGY RESEARCH AND PRACTICE
(2021)
Review
Cardiac & Cardiovascular Systems
Ansley M. Morrish, Janine Smith, Annabelle Enriquez, Gary F. Sholler, Jonathan Mervis, Sally L. Dunwoodie, Edwin P. Kirk, David S. Winlaw, Gillian M. Blue
Summary: Genetic and genomic testing in pediatric CHD is increasingly common, with significant implications in psychosocial, clinical, and reproductive aspects. Proper genetic counseling, integration of clinical genetics and cardiology workflows, and the establishment of a dedicated genetics clinic are essential for managing the information arising from genetic testing effectively.
TRENDS IN CARDIOVASCULAR MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
Jacob Y. Cao, Supreet P. Marathe, Diana Zannino, David S. Celermajer, Robert N. Justo, Nelson Alphonso, Yves d'Udekem, David S. Winlaw
Summary: Early Fontan operation does not pose a risk factor for short- or long-term adverse outcomes in our cohort.
EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY
(2022)
Review
Pediatrics
Derek L. Tran, Hannah Gibson, Andrew J. Maiorana, Charlotte E. Verrall, David W. Baker, Melanie Clode, David R. Lubans, Diana Zannino, Andrew Bullock, Suzie Ferrie, Julie Briody, Peter Simm, Vishva Wijesekera, Michelle D'Almeida, Sally E. Gosbell, Glen M. Davis, Robert Weintraub, Anthony C. Keech, Rajesh Puranik, Martin Ugander, Robert Justo, Dominica Zentner, Avik Majumdar, Leeanne Grigg, Jeff S. Coombes, Yves d'Udekem, Norman R. Morris, Julian Ayer, David S. Celermajer, Rachael Cordina
Summary: Despite advancements in surgical techniques and medical care, people with a Fontan circulation still face long-term complications, indicating a need for non-invasive therapies to optimize circulation. Exercise intolerance is common among this population, but exercise training may be important for improving aerobic capacity and circulatory function in the setting of Fontan physiology. Further research is needed to fully understand the mechanisms behind these improvements and to determine optimal methods of exercise prescription.
FRONTIERS IN PEDIATRICS
(2022)
Review
Cardiac & Cardiovascular Systems
S. Watkins, O. Isichei, T. L. Gentles, R. Brown, T. Percival, L. Sadler, R. Gorinski, S. Crengle, E. Cloete, M. W. M. de Laat, F. H. Bloomfield, K. Ward
Summary: The experience of diagnosis, decision-making and management in critical congenital heart disease is complex and has a significant impact on families. This study synthesised the current evidence and identified three themes related to the family experience.
PEDIATRIC CARDIOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Jessica M. Suna, Supreet P. Marathe, Prem Venugopal, Robert Justo, Nelson Alphonso, Gregory Merlo, Lisa Hall
Summary: This study examined stakeholder preferences for paediatric cardiac surgery performance data in Australia and New Zealand, finding that mortality information was of greatest interest and that most preferred hospital-level reporting and annual reports distributed electronically.
CARDIOLOGY IN THE YOUNG
(2023)
Review
Cardiac & Cardiovascular Systems
David S. Celermajer, David W. Baker, Rachael L. Cordina, Michael Gatzoulis, Craig S. Broberg
Summary: Due to advances in cardiac care, more patients with congenital heart disease are able to reach adulthood. It is important for general cardiologists to have a good understanding of adult congenital heart disease, including common errors and when to refer to a specialist center. This review provides case studies to help healthcare providers address common issues in adult congenital heart disease.
EUROPEAN HEART JOURNAL
(2023)
Article
Cardiac & Cardiovascular Systems
Alexander D. Hussey, Thomas L. Gentles, Fiona Perelini, John Beca, Kirsten Finucane
Summary: The outcomes of emergency arterial switch operation (ASO) for infants with d-loop transposition of the great arteries and persistent pulmonary hypertension of the newborn were analyzed retrospectively. The emergency ASO group had higher 30-day postoperative mortality, but there was no significant difference in abnormal neurodevelopmental outcome among the survivors compared to the nonemergency group. The emergency group required more treatments for low cardiac output syndrome, experienced more postoperative seizures, and had a longer length of stay.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2023)
Article
Nutrition & Dietetics
Nicholas A. Koemel, Alistair M. Senior, David S. Celermajer, Amanda Grech, Tim P. Gill, Stephen J. Simpson, David Raubenheimer, Michael R. Skilton
Summary: This study evaluated the associations between macronutrient intake and all-cause, cardiovascular, and cancer mortality. It found that a high-calorie diet consisting of moderately high protein, moderate fat, and moderate carbohydrate levels was associated with the highest mortality risk. Lower mortality risk was observed in two separate regions consisting of higher protein, higher carbohydrate, and lower fat levels or lower protein, moderate carbohydrate, and higher fat levels. These findings highlight the complex nonlinear and interactive association between macronutrients and all-cause mortality.
Article
Cardiac & Cardiovascular Systems
Benjamin Anderson, Robert Justo, Cameron Ward
Summary: This retrospective study analyzed the outcomes of using the Bentley BeGraft Aortic stent for the treatment of aortic arch pathology in children. The results showed that the stent can be safely deployed in children and has acceptable medium-term patency. However, larger and long-term follow-up studies are needed to assess its performance.
HEART LUNG AND CIRCULATION
(2023)
Article
Medicine, General & Internal
Natalie Soszyn, Elza Cloete, Lynn Sadler, Monique W. M. de laat, Sue Crengle, Frank Bloomfield, Kirsten Finucane, Thomas L. Gentles
Summary: This study retrospectively analyzed the relative influence of fetal and maternal factors on the choice-of-care pathway (CCP) and outcome in fetuses with hypoplastic left heart syndrome (HLHS). The study found that delayed diagnosis and ethnic disparities were related to prenatal decisions and postnatal outcomes, suggesting systemic inequity and the need for further investigation.
Review
Psychology, Developmental
Karen J. Eagleson, Desiree McCombs, Tiffany M. Gerlich, Robert N. Justo, Nadine A. Kasparian, Samudragupta Bora
Summary: This systematic review aims to identify instruments that quantitatively assess psychosocial adaptation and outcomes in families of children with congenital heart disease (CHD) and evaluate the psychometric properties of these instruments. The results show that there is a wide variation in the instruments used to assess psychosocial adaptation and outcomes among families of children with CHD. It is recommended to use instruments with robust key psychometrics for assessment and to improve the quality of psychometric reporting. The development of both a toolkit approach and a comprehensive CHD-specific family instrument are also among the key recommendations.
JOURNAL OF PEDIATRIC PSYCHOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Rachel Webb, Nicola Culliford-Semmens, Andrew ChanMow, Robert Doughty, Elizabeth Tilton, Briar Peat, John Stirling, Thomas L. Gentles, Nigel J. Wilson
Summary: A screening study conducted in an urban area of South Auckland, New Zealand, revealed a high prevalence of rheumatic heart disease (RHD) in adults, comparable to lower-middle-income countries in Africa. Non-rheumatic cardiac abnormalities were also identified.
Review
Medicine, Research & Experimental
Robyn W. May, Gonzalo Maso D. Talou, Alys R. Clark, Jonathan P. Mynard, Joseph J. Smolich, Pablo J. Blanco, Lucas O. Mueller, Thomas L. Gentles, Frank H. Bloomfield, Soroush Safaei
Summary: Computational modeling is widely used in the study of cardiovascular hemodynamics for medical research and improving the diagnosis and treatment of cardiovascular diseases. Most existing cardiovascular models focus on the adult circulatory system, but the perinatal period is unique as cardiovascular physiology undergoes significant changes. This review discusses computational models of the cardiovascular system in early life, their applications, and proposes a roadmap for developing an open-source model spanning fetal, perinatal, and postnatal periods.
WIRES MECHANISMS OF DISEASE
(2023)
Review
Clinical Neurology
Bridget R. Abell, Karen Eagleson, Benjamin Auld, Samudragupta Bora, Robert Justo, William Parsonage, Pakhi Sharma, Sanjeewa Kularatna, Steven M. McPhail
Summary: This scoping review identified and mapped the components of neurodevelopmental follow-up care for children with congenital heart disease (CHD). Common attributes of care pathways included enrolling high-risk children, centralized clinics, referral before discharge, periodic follow-up, standardized assessments, and multidisciplinary teams. Implementation barriers included financial resources, patient burden, and lack of knowledge. Stakeholder engagement and integration with other services were key drivers of success.
DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Gautham Shetty, Jason Zouki, Geraldine Lee, Aditya Patukale, Kim S. Betts, Robert N. Justo, Supreet P. Marathe, Prem Venugopal, Nelson QPCR Grp, Nelson Alphonso
Summary: The study aimed to determine the incidence of pneumothorax and/or pleural effusion after chest drain removal following pediatric cardiac surgery. The results showed a low incidence of clinically significant pneumothorax/pleural effusion, with most patients not requiring chest drain reinsertion, suggesting that routine chest radiographs following chest drain removal may not be necessary for most pediatric cardiac surgical patients.
INTERDISCIPLINARY CARDIOVASCULAR AND THORACIC SURGERY
(2023)