keyword
https://read.qxmd.com/read/35331414/eisenmenger-syndrome-jacc-state-of-the-art-review
#21
REVIEW
Alexandra Arvanitaki, Michael A Gatzoulis, Alexander R Opotowsky, Paul Khairy, Konstantinos Dimopoulos, Gerhard-Paul Diller, George Giannakoulas, Margarita Brida, Massimo Griselli, Ekkehard Grünig, Claudia Montanaro, Peter David Alexander, Rebecca Ameduri, Barbara J M Mulder, Michele D'Alto
Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary erythrocytosis, increased thrombotic and bleeding diathesis, high arrhythmogenic risk, progressive heart failure, and premature death. Clearly, care for ES is best delivered in multidisciplinary expert centers. In this review, we discuss the considerable recent progress in understanding the complex pathophysiology of ES, means of prognostication, and improvement in clinical outcomes achieved with pulmonary arterial hypertension-targeted therapies...
March 29, 2022: Journal of the American College of Cardiology
https://read.qxmd.com/read/34931762/usefulness-of-ventilatory-inefficiency-in-predicting-prognosis-across-the-heart-failure-spectrum
#22
JOURNAL ARTICLE
Jingyi Gong, Renata R T Castro, Jesse P Caron, Camden P Bay, Jon Hainer, Alexander R Opotowsky, Mandeep R Mehra, Bradley A Maron, Marcelo F Di Carli, John D Groarke, Anju Nohria
AIMS: The minute ventilation-carbon dioxide production relationship (VE/VCO2 slope) is widely used for prognostication in heart failure (HF) with reduced left ventricular ejection fraction (LVEF). This study explored the prognostic value of VE/VCO2 slope across the spectrum of HF defined by ranges of LVEF. METHODS AND RESULTS: In this single-centre retrospective observational study of 1347 patients with HF referred for cardiopulmonary exercise testing, patients with HF were categorized into HF with reduced (HFrEF, LVEF < 40%, n = 598), mid-range (HFmrEF, 40% ≤ LVEF < 50%, n = 164), and preserved (HFpEF, LVEF ≥ 50%, n = 585) LVEF...
December 21, 2021: ESC Heart Failure
https://read.qxmd.com/read/34903340/characterization-of-circulating-and-urinary-biomarkers-in-the-fontan-circulation-and-their-correlation-with-cardiac-imaging
#23
JOURNAL ARTICLE
Sunil J Ghelani, Alexander R Opotowsky, David M Harrild, Andrew J Powell, Nina Azcue, Sidra Ahmad, Nicole St Clair, Gary Bradwin, Rahul H Rathod
Several circulating biomarkers have been found to play a role in the surveillance and risk stratification of heart failure without congenital heart disease, but these have not been widely studied in patients with single ventricles palliated with a Fontan operation. Imaging predictors of worse outcomes in this population include ventricular dilation and dysfunction. Patients who weighed >30 kg with a Fontan circulation referred for cardiac magnetic resonance imaging were invited to participate in the study...
January 1, 2022: American Journal of Cardiology
https://read.qxmd.com/read/34893302/relation-of-liver-volume-to-adverse-cardiovascular-events-in-adolescents-and-adults-with-fontan-circulation
#24
JOURNAL ARTICLE
Adam M Lubert, Alexander R Opotowsky, Joseph J Palermo, Tarek Alsaied, Cassandra Szugye, Nadeem Anwar, Gregory M Tiao, Angela Lorts, Jonathan R Dillman, Andrew T Trout
Elevated central venous pressure in those with Fontan circulation causes liver congestion and hepatomegaly. We assessed if liver volume by magnetic resonance imaging (MRI) is associated with adverse cardiovascular outcomes. Retrospective study of 122 patients with Fontan circulation who were >10 years old and had a liver MRI with magnetic resonance elastography. Liver volume (ml) was measured by manual segmentation from axial T2-weighted images and was indexed to body surface area. The composite outcome included death, heart transplant, ventricular assist device placement, or nonelective cardiovascular hospitalization...
February 15, 2022: American Journal of Cardiology
https://read.qxmd.com/read/34736567/aha-acc-vs-esc-guidelines-for%C3%A2-management-of-adults-with%C3%A2-congenital-heart-disease-jacc-guideline-comparison
#25
REVIEW
Gabriele Egidy Assenza, Eric V Krieger, Helmut Baumgartner, Blanche Cupido, Konstantinos Dimopoulos, Clauden Louis, Adam M Lubert, Karen K Stout, Anne Marie Valente, Katja Zeppenfeld, Alexander R Opotowsky
The American Heart Association and American College of Cardiology published practice guidelines for the management of adult congenital heart disease in 2018 and the European Society of Cardiology published analogous guidelines in 2020. Although there are broad areas of consensus between the 2 documents, there are important differences that impact patient management. This review discusses key areas of agreement and disagreement between the 2 guidelines, with discussion of possible reasons for disagreement and potential implications...
November 9, 2021: Journal of the American College of Cardiology
https://read.qxmd.com/read/34482709/associations-between-clinical-outcomes-and-a-recently-proposed-adult-congenital-heart-disease-anatomic-and-physiological-classification-system
#26
JOURNAL ARTICLE
Cara L Lachtrupp, Anne Marie Valente, Michelle Gurvitz, Michael J Landzberg, Sarah B Brainard, Fred M Wu, Dorothy D Pearson, Keith Taillie, Alexander R Opotowsky
Background American Heart Association and American College of Cardiology consensus guidelines introduce an adult congenital heart disease anatomic and physiological (AP) classification system. We assessed the association between AP classification and clinical outcomes. Methods and Results Data were collected for 1000 outpatients with ACHD prospectively enrolled between 2012 and 2019. AP classification was assigned based on consensus definitions. Primary outcomes were (1) all-cause mortality and (2) a composite of all-cause mortality or nonelective cardiovascular hospitalization...
September 6, 2021: Journal of the American Heart Association
https://read.qxmd.com/read/34474916/adult-survivors-of-moderate-and-great-complexity-congenital-heart-disease-undergoing-general-surgery-procedures-how-do-they-fare
#27
JOURNAL ARTICLE
Bethany J Farr, Manuel Castillo-Angeles, Barbara Okafor, Nikita Patel, Ramsis Ramsis, Nael Aldweib, Alexander R Opotowsky, Deepika Nehra, Samuel E Rice-Townsend
BACKGROUND: Patients with complex congenital heart disease (CHD) are now commonly surviving well into adulthood. We describe the clinical characteristics and outcomes for a cohort of adult patients with moderate and great complexity CHD undergoing general surgery procedures. METHODS: The electronic records of two tertiary centers were queried to identify adult patients with moderate and great complexity CHD who underwent a general surgery procedure between 2007 and 2017...
August 17, 2021: American Journal of Surgery
https://read.qxmd.com/read/34448042/relation-of-magnetic-resonance-elastography-to-fontan-circulatory-failure-in-a-cohort-of-pediatric-and-adult-patients
#28
JOURNAL ARTICLE
Samuel W Brayer, Faizeen Zafar, Adam M Lubert, Andrew T Trout, Joseph J Palermo, Alexander R Opotowsky, Nadeem Anwar, Jonathan R Dillman, Tarek Alsaied
Elevated magnetic resonance elastography (MRE)-derived liver stiffness may be associated with worse outcomes in people with Fontan circulation. We sought to evaluate the association between liver stiffness and Fontan failure or portal hypertension. Single center cross-sectional retrospective study of people with Fontan circulation who underwent MRE between 2011 and 2020. The cohort was divided into adult (age ≥ 21 years) and pediatric (< 21 years) groups. Fontan circulatory failure (FF) was defined as any of the following: death, transplantation, ventricular assist device, heart failure symptoms requiring escalation of diuretics...
December 2021: Pediatric Cardiology
https://read.qxmd.com/read/34436466/metabolomic-profiling-of-adults-with-congenital-heart-disease
#29
JOURNAL ARTICLE
Ari Cedars, Cedric Manlhiot, Jong-Mi Ko, Teodoro Bottiglieri, Erland Arning, Angela Weingarten, Alexander Opotowsky, Shelby Kutty
Metabolomic analysis may provide an integrated assessment in genetically and pathologically heterogeneous populations. We used metabolomic analysis to gain mechanistic insight into the small and diverse population of adults with congenital heart disease (ACHD). Consecutive ACHD patients seen at a single institution were enrolled. Clinical variables and whole blood were collected at regular clinical visits. Stored plasma samples were analyzed for the concentrations of 674 metabolites and metabolic markers using mass spectrometry with internal standards...
August 9, 2021: Metabolites
https://read.qxmd.com/read/34190428/reaching-consensus-for-unified-medical-language-in-fontan-care
#30
JOURNAL ARTICLE
Tarek Alsaied, Rahul H Rathod, Jamil A Aboulhosn, Werner Budts, Jeffrey B Anderson, Helmut Baumgartner, David W Brown, Rachael Cordina, Yves D'udekem, Salil Ginde, David J Goldberg, Bryan H Goldstein, Adam M Lubert, Erwin Oechslin, Alexander R Opotowsky, Jack Rychik, Kurt R Schumacher, Anne Marie Valente, Gail Wright, Gruschen R Veldtman
AIMS: The Fontan operation has resulted in improved survival in patients with single-ventricle congenital heart disease. As a result, there is a growing population of teenagers and adults with a Fontan circulation. Many co-morbidities have been increasingly recognized in this population due to the unique features of the Fontan circulation. Standardization of how Fontan co-morbid conditions are defined will help facilitate understanding, consistency and interpretability of research and clinical experience...
October 2021: ESC Heart Failure
https://read.qxmd.com/read/34088498/commentary-time-for-a-new-maxim
#31
EDITORIAL
David S Winlaw, Alexander R Opotowsky, James S Tweddell
No abstract text is available yet for this article.
May 20, 2021: Journal of Thoracic and Cardiovascular Surgery
https://read.qxmd.com/read/33966676/developing-an-adolescent-and-adult-fontan-management-programme
#32
JOURNAL ARTICLE
Adam M Lubert, Tarek Alsaied, Andrew T Trout, Jonathan R Dillman, Joseph J Palermo, Felicia Eichelbrenner, Kelly Kleier, Angela Lorts, Nadeem Anwar, Marc G Schecter, Gregory M Tiao, Clifford Chin, David S Feldman, Meredith Jenkins, David Ls Morales, Alexander R Opotowsky, John C Bucuvalas, Gruschen R Veldtman, Stuart L Goldstein
Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes...
February 2022: Cardiology in the Young
https://read.qxmd.com/read/33926659/lifespan-perspective-on-congenital%C3%A2-heart-disease-research-jacc-state-of-the-art-review
#33
REVIEW
Gerhard-Paul Diller, Alexandra Arvanitaki, Alexander R Opotowsky, Kathy Jenkins, Philip Moons, Alexander Kempny, Animesh Tandon, Andrew Redington, Paul Khairy, Seema Mital, Michael Α Gatzoulis, Yue Li, Ariane Marelli
More than 90% of patients with congenital heart disease (CHD) are nowadays surviving to adulthood and adults account for over two-thirds of the contemporary CHD population in Western countries. Although outcomes are improved, surgery does not cure CHD. Decades of longitudinal observational data are currently motivating a paradigm shift toward a lifespan perspective and proactive approach to CHD care. The aim of this review is to operationalize these emerging concepts by presenting new constructs in CHD research...
May 4, 2021: Journal of the American College of Cardiology
https://read.qxmd.com/read/33880928/hepatic-steatosis-in-patients-with-single-ventricle-and-a-fontan-circulation
#34
JOURNAL ARTICLE
David A Katz, Daniel Peck, Adam M Lubert, Mathias Possner, Faizeen Zafar, Andrew T Trout, Joseph J Palermo, Nadeem Anwar, Jonathan R Dillman, Adam W Powell, Stavra A Xanthakos, Alexander R Opotowsky, Gruschen Veldtman, Tarek Alsaied
Background Hepatic steatosis, caused by nonalcoholic fatty liver disease, is a leading cause of chronic liver disease. The interplay between hepatic steatosis and the development of liver disease following the Fontan procedure is not well understood. This study examined the prevalence and associations of hepatic steatosis in patients with a Fontan circulation. Methods and Results This was a single-center retrospective study of 95 patients with a Fontan circulation with liver magnetic resonance imaging performed between 2012 and 2019...
May 4, 2021: Journal of the American Heart Association
https://read.qxmd.com/read/33787283/fontan-associated-dyslipidemia
#35
JOURNAL ARTICLE
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
Background Hypocholesterolemia is a marker of liver disease, and patients with a Fontan circulation may have hypocholesterolemia secondary to Fontan-associated liver disease or inflammation. We investigated circulating lipids in adults with a Fontan circulation and assessed the associations with clinical characteristics and adverse events. Methods and Results We enrolled 164 outpatients with a Fontan circulation, aged ≥18 years, in the Boston Adult Congenital Heart Disease Biobank and compared them with 81 healthy controls...
April 6, 2021: Journal of the American Heart Association
https://read.qxmd.com/read/33313813/tuning-and-external-validation-of-an-adult-congenital-heart-disease-risk-prediction-model
#36
JOURNAL ARTICLE
Laurie W Geenen, Alexander R Opotowsky, Cara Lachtrupp, Vivan J M Baggen, Sarah Brainard, Michael J Landzberg, David van Klaveren, Hester F Lingsma, Eric Boersma, Jolien W Roos-Hesselink
AIM: Adequate risk prediction can optimize the clinical management in adult congenital heart disease (ACHD). We aimed to update and subsequently validate a previously developed ACHD risk prediction model. METHODS AND RESULTS: A prediction model was developed in a prospective cohort study including 602 moderately or severely complex ACHD patients, enrolled as outpatients at a tertiary centre in the Netherlands (2011-2013). Multivariable Cox regression was used to develop a model for predicting the 1-year risks of death, heart failure, or arrhythmia (primary endpoint)...
December 12, 2020: European Heart Journal. Quality of Care & Clinical Outcomes
https://read.qxmd.com/read/33289459/body-composition-and-exercise-performance-in-youth-with-a-fontan-circulation-a-bio-impedance-based-study
#37
JOURNAL ARTICLE
Adam W Powell, Samuel G Wittekind, Tarek Alsaied, Adam M Lubert, Clifford Chin, Gruschen R Veldtman, Rachael Cordina, David A Katz, Wayne A Mays, Sandra K Knecht, Alexander R Opotowsky
Background Adults with a Fontan circulation tend to have myopenia and elevated adiposity when measured by dual energy x-ray absorptiometry. Bioelectrical impedance analysis is an alternative validated approach to assess body composition. We used bioelectrical impedance analysis to compare body composition between pediatric patients with a Fontan circulation and control individuals without heart disease. Methods and Results A retrospective chart review identified all patients aged <22 years with a Fontan circulation who presented for cardiopulmonary exercise testing and bioelectrical impedance analysis from April 2019 to January 2020...
December 15, 2020: Journal of the American Heart Association
https://read.qxmd.com/read/33243381/implications-of-elevated-pulmonary-artery-pressure-for-transcatheter-mitral-repair-time-for-comprehensive-hemodynamic-investigation
#38
EDITORIAL
Alexander R Opotowsky, Konstantinos Dimopoulos, Jonathan Buber
No abstract text is available yet for this article.
December 1, 2020: Journal of the American College of Cardiology
https://read.qxmd.com/read/33084842/association-of-damaging-variants-in-genes-with-increased-cancer-risk-among-patients-with-congenital-heart-disease
#39
JOURNAL ARTICLE
Sarah U Morton, Akiko Shimamura, Peter E Newburger, Alexander R Opotowsky, Daniel Quiat, Alexandre C Pereira, Sheng Chih Jin, Michelle Gurvitz, Martina Brueckner, Wendy K Chung, Yufeng Shen, Daniel Bernstein, Bruce D Gelb, Alessandro Giardini, Elizabeth Goldmuntz, Richard W Kim, Richard P Lifton, George A Porter, Deepak Srivastava, Martin Tristani-Firouzi, Jane W Newburger, J G Seidman, Christine E Seidman
Importance: Patients with congenital heart disease (CHD), the most common birth defect, have increased risks for cancer. Identification of the variables that contribute to cancer risk is essential for recognizing patients with CHD who warrant longitudinal surveillance and early interventions. Objective: To compare the frequency of damaging variants in cancer risk genes among patients with CHD and control participants and identify associated clinical variables in patients with CHD who have cancer risk variants...
October 21, 2020: JAMA Cardiology
https://read.qxmd.com/read/32947058/interobserver-agreement-of-the-anatomic-and-physiological-classification-system-for-adult-congenital-heart-disease
#40
JOURNAL ARTICLE
Cara L Lachtrupp, Anne Marie Valente, Michelle Gurvitz, Michael J Landzberg, Sarah B Brainard, Alexander R Opotowsky
The Anatomic and Physiological (AP) classification system proposed in the 2018 American College of Cardiology/American Heart Association adult congenital heart disease (ACHD) guidelines assigns 2 dimensions to each patient: anatomic class (AnatC) and physiological stage (PhyS). This approach has not been tested in practice; we assessed interrater reliability and identified sources of disagreement. METHODS: Consensus definitions for AP categories were developed with input from 4 experts...
November 2020: American Heart Journal
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