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
Peter Kramer, Anastasia Schleiger, Marie Schafstedde, Friederike Danne, Johannes Nordmeyer, Felix Berger, Stanislav Ovroutski
Summary: Aiming at late failure and mortality in adult Fontan patients, we have developed an accurate scoring system to classify Fontan failure and mortality, which requires prospective validation and potential refinement in larger multi-institutional cohorts for risk prediction.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
Article
Gastroenterology & Hepatology
Juliet Emamaullee, Sara Khan, Carly Weaver, Cameron Goldbeck, George Yanni, Rohit Kohli, Yuri Genyk, Shengmei Zhou, Nick Shillingford, Patrick M. Sullivan, Cheryl Takao, Jon Detterich, Paul F. Kantor, John D. Cleveland, Cynthia Herrington, S. Ram Kumar, Vaughn Starnes, Sarah Badran, Neil D. Patel
Summary: The study examined FALD in the young adult population post-Fontan surgery and identified non-invasive markers associated with bridging fibrosis. Bridging fibrosis was observed in 35% of patients within the first decade post-Fontan and was linked to worse survival. Changes in platelet counts, even years before biopsy, and APRI/FIB-4 scores had modest discriminatory power in identifying patients with advanced fibrosis. Steatosis may represent an additional risk factor for disease progression in obese patients. Further prospective studies are needed to develop strategies for screening FALD in adolescents.
Review
Cardiac & Cardiovascular Systems
Jeremy P. Moore, Ariane Marelli, Luke J. Burchill, Henry Chubb, S. Lucy Roche, Ari M. Cedars, Paul Khairy, Ali N. Zaidi, Jan Janousek, David S. Crossland, Robert H. Pass, Jeffrey P. Jacobs, Jonathan N. Menachem, David S. Frankel, Sabine Ernst, Jim T. Vehmeijer, Mitchell Cohen
Summary: Heart failure and arrhythmia are the most important cardiovascular diseases among adults with congenital heart disease, and they often overlap and interact with each other. It is crucial for clinical management to recognize these interactions and have access to the available pharmacologic and interventional resources.
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
(2022)
Article
Sport Sciences
Karoline Krzywda, Kelli M. Teson, Jessica S. Watson, Suma Goudar, Daniel Forsha, Jonathan B. Wagner, David A. White
Summary: Patients with single ventricle heart disease after Fontan palliation are prone to cardiovascular deterioration. A peak oxygen consumption less than 50% of predicted is an independent predictor of Fontan-related morbidity and mortality. The recovery of oxygen consumption after exercise (VO2RD) has not been extensively studied in youth with Fontan physiology.
MEDICINE & SCIENCE IN SPORTS & EXERCISE
(2023)
Article
Medicine, General & Internal
Nili Schamroth Pravda, Oren Zusman, Ilan Richter, Leonard Blieden, Shahar Vig, Ilan Marchushamer, Alexander Dadashev, Yaron Razon, Ran Kornowski, Rafael Hirsch
Summary: This study investigated the self-reported physical and mental health of Fontan patients. The results showed that adult Fontan patients reported worse physical health but better mental health compared to the general population. These findings have important implications for the care of patients with complex congenital heart disease.
JOURNAL OF CLINICAL MEDICINE
(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
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
Adam M. Lubert, Tarek Alsaied, Joseph J. Palermo, Nadeem Anwar, Elaine M. Urbina, Nicole M. Brown, Craig Alexander, Hassan Almeneisi, Fred Wu, Andrew R. Leventhal, Nael Aldweib, Michael Mendelson, Alexander R. Opotowsky
Summary: Fontan circulation is associated with decreased cholesterol levels, and lower high-density lipoprotein cholesterol is linked to adverse outcomes, possibly driven by inflammation. Further studies are needed to explore the relationship between the severity of Fontan-associated liver disease and lipid metabolism.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2021)
Review
Cardiac & Cardiovascular Systems
Ali Abdulkarim, Shawn Shaji, Mahmud El Fituri, Megan Gunsaulus, Muhammad A. Zafar, Ali N. Zaidi, Robert H. Pass, Brian Feingold, Geoffrey Kurland, Jacqueline Kreutzer, Rod Ghassemzadeh, Bryan Goldstein, Shawn West, Tarek Alsaied
Summary: The Fontan operation has improved the survival of patients with single ventricle physiology. However, there are long-term complications associated with this surgery, including pulmonary complications and cyanosis. Understanding cardiopulmonary interactions and improving the recognition and treatment of pulmonary abnormalities may enhance the long-term outcomes of patients.
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
(2023)
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
Eva van den Bosch, Sjoerd S. M. Bossers, Vivian P. Kamphuis, Eric Boersma, Jolien W. Roos-Hesselink, Johannes M. P. J. Breur, Arend D. J. Ten Harkel, Livia Kapusta, Beatrijs Bartelds, Arno A. W. Roest, Irene M. Kuipers, Nico A. Blom, Laurens P. Koopman, Willem A. Helbing
Summary: This study found that blood biomarkers such as NT-proBNP, GDF-15, vWF, DLK-1, ST-2, FABP-4, and IGFBP-7 levels are related to the long-term prognosis of young patients who have undergone the Fontan procedure.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2021)
Article
Cardiac & Cardiovascular Systems
Jack Rychik
Summary: The article discusses the mixed emotions and uncertainty surrounding the care of patients with Fontan circulation, highlighting the underwhelming results of the FUEL trial and the search for improved therapies. Dr. Rychik's expertise in this area provides valuable insights into factors affecting pulmonary blood flow in Fontan patients.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2021)
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
William R. Miranda, C. Charles Jain, Robert P. Frantz, Hilary M. Dubrock, Heidi M. Connolly, Luke J. Burchill, Alexander C. Egbe
Summary: This study reviewed the experience of using inhaled nitric oxide (iNO) during cardiac catheterization in 30 adults post-Fontan. The results showed that iNO could decrease pulmonary artery pressures and PVRi without increasing pulmonary artery wedge pressure, and had no effect on cardiac index.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
Review
Cardiac & Cardiovascular Systems
Lucy Geraghty, Pasquale Santangeli, Usha B. Tedrow, Kalyanam Shivkumar, Saurabh Kumar
HEART LUNG AND CIRCULATION
(2019)
Editorial Material
Cardiac & Cardiovascular Systems
Jason S. Bradfield, Osamu Fujimura, Noel G. Boyle, Kalyanam Shivkumar
Editorial Material
Cardiac & Cardiovascular Systems
Kenneth A. Ellenbogen, Roderick Tung, Kalyanam Shivkumar, John M. Miller
JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY
(2019)
Article
Biochemical Research Methods
Rosemary C. Challis, Sripriya Ravindra Kumar, Ken Y. Chan, Collin Challis, Keith Beadle, Min J. Jang, Hyun Min Kim, Pradeep S. Rajendran, John D. Tompkins, Kalyanam Shivkumar, Benjamin E. Deverman, Viviana Gradinaru
Review
Cardiac & Cardiovascular Systems
Jorge Romero, Kalayanam Shivkumar, Luigi Di Biase, Ricardo Avendano, Robert D. Anderson, Andrea Natale, Saurabh Kumar
Article
Cardiac & Cardiovascular Systems
David Hamon, Mohammed Amer Swid, Pradeep S. Rajendran, Albert Liu, Noel G. Boyle, Kalyanam Shivkumar, Jason S. Bradfield
JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY
(2019)
Review
Medicine, General & Internal
Kalyanam Shivkumar
NEW ENGLAND JOURNAL OF MEDICINE
(2019)
Article
Cell Biology
Katharina Scherschel, Katja Hedenus, Christiane Jungen, Marc D. Lemoine, Nicole Ruebsamen, Marieke W. Veldkamp, Niklas Klatt, Diana Lindner, Dirk Westermann, Simona Casini, Pawel Kuklik, Christian Eickholt, Nikolaj Kloecker, Kalyanam Shivkumar, Torsten Christ, Tanja Zeller, Stephan Willems, Christian Meyer
SCIENCE TRANSLATIONAL MEDICINE
(2019)
Article
Cardiac & Cardiovascular Systems
Kalyanam Shivkumar, Noel G. Boyle
Editorial Material
Cardiac & Cardiovascular Systems
Jason S. Bradfield, Kalyanam Shivkumar
Editorial Material
Cardiac & Cardiovascular Systems
Jeremy P. Moore, Kalyanam Shivkumar
Article
Cardiac & Cardiovascular Systems
Bastiaan J. D. Boukens, Michael Dacey, Veronique M. F. Meijborg, Michiel J. Janse, Joseph Hadaya, Peter Hanna, M. Amer Swid, Tobias Opthof, Jeffrey L. Ardell, Kalyanam Shivkumar, Ruben Coronel
Summary: Enhanced sympathetic activity during acute ischemia causes arrhythmias, with a mechanism involving a diastolic current flowing from the ischemic to non-ischemic myocardium. Stimulation of the left stellate ganglion shortens repolarization in non-ischemic myocardium, leading to more negative T waves in the ischemic zone and ventricular premature beats from normal tissue nearby.
CARDIOVASCULAR RESEARCH
(2021)
Biographical-Item
Cardiac & Cardiovascular Systems
Kalyanam Shivkumar, Noel G. Boyle
Article
Medicine, Research & Experimental
Ching Zhu, Pradeep S. Rajendran, Peter Hanna, Igor R. Efimov, Guy Salama, Charless C. Fowlkes, Kalyanam Shivkumar
Summary: This study used a high-resolution pipeline and computer vision algorithms to investigate microstructural changes on the heart after myocardial infarction (MI) and their association with electrical dysfunction. The results established clear structure-function relationships and identified potential neural substrates for cardiac arrhythmia after MI, providing a framework for understanding arrhythmogenic mechanisms with microscopic precision.
Editorial Material
Neurosciences
Kalyanam Shivkumar, Zhilin Qu, Robert Harvey
JOURNAL OF PHYSIOLOGY-LONDON
(2023)