Review
Medicine, General & Internal
Amalia Baroutidou, Alexandra Arvanitaki, Adam Hatzidakis, Georgia Pitsiou, Antonios Ziakas, Haralambos Karvounis, George Giannakoulas
Summary: Haemoptysis is a severe bleeding manifestation in the clinical course of pulmonary arterial hypertension associated with congenital heart disease. Dysfunction of the pulmonary vascular bed increases the risk of haemoptysis episodes, and despite treatment strategies like BAE, bleeding recurrences are prevalent. There is currently no clear guideline regarding the use of oral anticoagulation in patients with haemoptysis.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Pharmacology & Pharmacy
Jun Luo, Yuanchang Li, Jingyuan Chen, Haihua Qiu, Wenjie Chen, Xiaoqin Luo, Yusi Chen, Yingjie Tan, Jiang Li
Summary: This study found a relationship between serum uric acid level and the severity of disease and treatment response in patients with PAH-CHD. Serum uric acid can be used as a biomarker for risk stratification and evaluation of treatment effects for PAH-CHD patients.
FRONTIERS IN PHARMACOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Feng Xie, Ruilin Quan, Gangcheng Zhang, Hongyan Tian, Yucheng Chen, Zaixin Yu, Caojin Zhang, Yuhao Liu, Xianyang Zhu, Weifeng Wu, Xiulong Zhu, Zhenwen Yang, Qing Gu, Changming Xiong, Huijun Han, Yingzhang Cheng, Jianguo He, Yanqing Wu
Summary: The purpose of this study was to understand the characteristics, treatments, and survival of patients with PAH-CHD in China. The majority of patients had Eisenmenger syndrome and exhibited significantly impaired exercise tolerance and right ventricular function at diagnosis. The long-term survival of PAH-CHD patients in China needs improvement, and PAH-targeted therapy, including combination therapy, has a positive effect on survival.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
Article
Cardiac & Cardiovascular Systems
Amadeo-Jose Wals-Rodriguez, Maria-Jose Rodriguez-Puras, Rocio Garcia-Orta, Juan Robledo, Eduardo Moreno, Carmen Federero, Rocio Camacho, Begona Manso, Nuria Hernandez, Jose Maria Cubero, Raquel Ladron Abia, Pilar Cejudo Ramos, Antonio Ordonez, Pastora Gallego
Summary: The study found that the survival rate of adults with PAH-CHD receiving vasodilator treatment was 65%, with the highest mortality rate in the pre-tricuspid group. Older age was associated with higher risk of death, and predictors of poor outcome included pericardial effusion, oxygen saturation, and genetic syndromes.
Review
Pharmacology & Pharmacy
Adriana Mares, Debabrata Mukherjee, Richard A. A. Lange, Nils P. P. Nickel
Summary: Pulmonary arterial hypertension (PAH) is a devastating cardiovascular disease that can be treated effectively with PAH-targeted therapies. Patients with congenital heart disease (CHD) are at high risk of developing PAH, and evidence shows that PAH-targeted therapy can be beneficial for them. However, treating the PAH-CHD population is challenging due to the complexity of their cardiac lesions and associated comorbidities.
CURRENT VASCULAR PHARMACOLOGY
(2022)
Article
Cardiac & Cardiovascular Systems
Yang Liu, Yanna Li, Jun Zhang, Yichen Zhao, Kemin Liu, Jiachen Li, Mingming Zhao, Hong Gu, Xiangming Fan, Jiangang Wang
Summary: This study retrospectively analyzed and followed up on pregnant women with Eisenmenger syndrome (ES) admitted to Beijing Anzhen Hospital between 2010 and 2019 to explore the outcomes of the mothers and their offspring. The study found significant differences in age, gestational age, percutaneous oxygen saturation, Apgar score, and heart failure between the maternal death and non-death groups. The study recommends pregnancy termination if ES occurs during early pregnancy and highlights the importance of multidisciplinary cooperation to improve the prognosis of the mothers and their offspring.
INTERNATIONAL JOURNAL OF CARDIOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Liukun Meng, Wen Yuan, Hongjie Chi, Ruijuan Han, Yeping Zhang, Xiangbin Pan, Jian Meng, Ying Liu, Jiawei Song, Jiuchang Zhong, Xiaoyan Liu
Summary: This study revealed a significant role of CMG2 in the pathogenesis of SPS-induced PAH, where CMG2 deficiency exacerbates vascular remodeling, suggesting CMG2 as a potential target for the treatment of CHD-PAH.
Article
Cardiac & Cardiovascular Systems
Laion R. A. Gonzaga, Walter J. Gomes, Isadora S. Rocco, Bruna C. Matos-Garcia, Caroline Bublitz, Marcela Viceconte, Solange B. Tatani, Vinicius B. Santos, Celia M. C. Silva, Robert Tulloh, Ross Arena, Solange Guizilini
Summary: Patients with Eisenmenger syndrome show a more severe inflammatory profile compared to those with congenital heart disease and pulmonary arterial hypertension. Inflammatory markers are associated with renal dysfunction, right ventricular impairment, and poorer functional capacity in ES patients.
INTERNATIONAL JOURNAL OF CARDIOLOGY
(2021)
Article
Respiratory System
Erika B. Rosenzweig, Usha Krishnan
Summary: Pulmonary arterial hypertension associated with congenital heart defects, particularly with systemic-to-pulmonary shunt lesions, is classified with other forms of PAH according to World Symposium on Pulmonary Hypertension group 1 classification. Prognosis varies based on the type and size of the heart defect, development of PAH, and response to treatment. Univentricular heart-related pulmonary vascular disease forms a challenging subgroup, which requires different diagnostic and prognostic criteria.
CLINICS IN CHEST MEDICINE
(2021)
Article
Respiratory System
Alexandra N. Linder, Jill Hsia, Sheila V. Krishnan, Emile A. Bacha, Sarah Crook, Erika B. Rosenzweig, Usha S. Krishnan
Summary: This study assessed the outcomes of an individualized strategy for managing patients with mildly to moderately elevated pulmonary vascular resistance (PVR) deemed borderline eligible for repair. The results showed that the use of preoperative targeted therapy in conjunction with fenestrated or partial closure of intracardiac shunts improved the clinical outcomes and WHO functional class (FC) in these patients.
Review
Cardiac & Cardiovascular Systems
Katharina Strack, Sebastian Freilinger, Amely J. Busse, Peter Ewert, Michael Hauser, Michael Huntgeburth, Ann-Sophie Kaemmerer, Nicole Nagdyman, Judith Schopen, Harald Kaemmerer, Fabian von Scheidt
Summary: This study retrospectively analyzed clinical data of 103 PAH-CHD patients, showing that many patients benefited from targeted PAH therapy and treatment decisions were mainly based on clinical assessment. Some patients gradually shifted towards combination therapy during the study. Survival rates varied among different patient subgroups.
CARDIOVASCULAR DIAGNOSIS AND THERAPY
(2022)
Article
Medicine, General & Internal
Susanne J. Maurer, Veronika Habdank, Juergen Horer, Peter Ewert, Oktay Tutarel
Summary: This study aimed to investigate the risk factors for poor outcome in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). The results showed that NT-proBNP is an independent predictor of all-cause mortality in PAH-CHD patients. The role of CRP and uric acid should be further studied.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Cardiac & Cardiovascular Systems
Chinthaka B. Samaranayake, Christopher Warren, Karina Siewers, Stuart Craig, Laura C. Price, Aleksander Kempny, Konstantinos Dimopoulos, Michael Gatzoulis, Nicholas S. Hopkinson, Stephen J. Wort, James H. Hull, Colm McCabe
Summary: Patients with IPAH and EIS displayed heightened ventilatory responses during stair-climbing compared to healthy controls, despite similar baseline spirometry and oxygen uptake changes during exercise. EIS patients exhibited early exercise declines in carbon dioxide and arterial oxygen saturation which distinguished them from the IPAH patients. Significant correlations were found between stair-climb time, respiratory rate, and other ventilatory parameters with peak exercise Borg score, indicating more severe dyspnoea perception in EIS patients.
INTERNATIONAL JOURNAL OF CARDIOLOGY
(2021)
Article
Obstetrics & Gynecology
W. Chen, J. Chen, M. Peng, J. Luo, Y. Chen, H. Qiu, J. Li
Summary: This study includes a retrospective case and literature review of 72 pregnant women with ES from 11 studies. The results suggest that targeted drugs may be crucial in reducing maternal mortality in pregnant women with ES.
BJOG-AN INTERNATIONAL JOURNAL OF OBSTETRICS AND GYNAECOLOGY
(2023)
Review
Cardiac & Cardiovascular Systems
Maolin Zhao, Jian Liu, Mei Xin, Ke Yang, Honghao Huang, Wenxin Zhang, Jinbao Zhang, Siyi He
Summary: Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a progressive condition with uncertain physiological course. Omics technology, such as genomics, transcriptomics, epigenomics, proteomics, metabolomics, and multi-omics integration, has provided advanced techniques for comprehensive assessment of the disease and identification of treatment strategies. Recent research has made significant progress in understanding PAH-CHD through the application of omics approaches.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2023)
Article
Cardiac & Cardiovascular Systems
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
Summary: Patients with single-ventricle congenital heart disease undergo a series of palliative surgeries before the Fontan procedure, which can lead to various cardiac complications and multi-organ dysfunction. The care for Fontan circulation patients has shifted towards monitoring multiple organ systems to identify those at risk, with the development of multidisciplinary programs for earlier detection and treatment of complications.
CARDIOLOGY IN THE YOUNG
(2022)
Article
Surgery
Bethany J. Farr, Manuel Castillo-Angeles, Barbara Okafor, Nikita Patel, Ramsis Ramsis, Nael Aldweib, Alexander R. Opotowsky, Deepika Nehra, Samuel E. Rice-Townsend
Summary: This study describes the clinical characteristics and outcomes of adult patients with moderate and great complexity congenital heart disease (CHD) undergoing general surgery procedures. The study found that these patients had low in-hospital mortality and morbidity rates.
AMERICAN JOURNAL OF SURGERY
(2022)
Article
Respiratory System
Marc Humbert, Gabor Kovacs, Marius M. Hoeper, Roberto Badagliacca, Rolf M. F. Berger, Margarita Brida, Jorn Carlsen, Andrew J. S. Coats, Pilar Escribano-Subias, Pisana Ferrari, Diogenes S. Ferreira, Hossein Ardeschir Ghofrani, George Giannakoulas, David G. Kiely, Eckhard Mayer, Gergely Meszaros, Blin Nagavci, Karen M. Olsson, Joanna Pepke-Zaba, Jennifer K. Quint, Goran Radegran, Gerald Simonneau, Olivier Sitbon, Thomy Tonia, Mark Toshner, Jean Luc Vachiery, Anton Vonk Noordegraaf, Marion Delcroix, Stephan Rosenkranz
EUROPEAN RESPIRATORY JOURNAL
(2023)
Review
Cardiac & Cardiovascular Systems
Konstantinos Dimopoulos, Andrew Constantine, Paul Clift, Robin Condliffe, Shahin Moledina, Katrijn Jansen, Ryo Inuzuka, Gruschen R. Veldtman, Clifford L. Cua, Edgar Lik Wui Tay, Alexander R. Opotowsky, George Giannakoulas, Rafael Alonso-Gonzalez, Rachael Cordina, George Capone, Judith Namuyonga, Charmaine H. Scott, Michele D'Alto, Francisco J. Gamero, Brian Chicoine, Hong Gu, Alisa Limsuwan, Tosin Majekodunmi, Werner Budts, Gerry Coghlan, Craig S. Broberg
Summary: Congenital heart disease is the most common cardiovascular condition in individuals with Down syndrome, affecting up to 50% of patients. Other factors that contribute to cardiovascular outcomes include pulmonary hypertension, pulmonary, endocrine, and metabolic diseases, and risk factors for atherosclerotic disease. Disparities in cardiovascular care for individuals with Down syndrome, which vary across different locations and healthcare systems, are often overlooked. This review provides a comprehensive summary of the diagnosis, prevalence, and management of cardiovascular disease in patients with Down syndrome, as well as addressing disparities in care based on resource availability.
Review
Cardiac & Cardiovascular Systems
R. Joye, M. Beghetti, J. Wacker, I. Malaspinas, M. Bouhabib, A. Polito, A. Bordessoule, D. Shah
Summary: This article synthesizes the current knowledge on the epidemiological features, pathophysiological insights, electrophysiological features, and treatment options regarding tachyarrhythmias in children and young adults undergoing reparative surgery for congenital heart disease. It summarizes the latest data on treatment options, identifies knowledge gaps, and highlights areas for future research.
PEDIATRIC CARDIOLOGY
(2023)
Review
Cardiac & Cardiovascular Systems
J. H. Huang, S. G. Wittekind, A. R. Opotowsky, K. Ward, A. Lyman, N. Gauthier, M. Vernon, A. W. Powell, D. A. White, T. J. Curran, W. B. Orr, P. Stephens, B. Robinson, T. D. Pham, W. A. Mays, D. Burstein, M. Carr, S. Paridon, J. Rhodes, P. Koenig
Summary: Exercise medicine has shown significant clinical value in managing children and adolescents with heart disease, but there are no standardized training recommendations for exercise physiology in pediatric cardiology fellowship. To address this, the Pediatric Cardiology Exercise Medicine Curriculum Committee was formed to establish core and advanced exercise physiology training recommendations. These expert consensus recommendations aim to provide minimum knowledge and training for general pediatric cardiology practice.
PEDIATRIC CARDIOLOGY
(2023)
Article
Medicine, General & Internal
Leon Genecand, Gaetan Simian, Roberto Desponds, Julie Wacker, Silvia Ulrich, Benoit Lechartier, Jean-Marc Fellrath, Olivier Sitbon, Maurice Beghetti, Frederic Lador
Summary: This study aimed to establish a mathematical model for evaluating the probability of classifying PcPH or unclassified PH by using direct Fick method instead of thermodilution to measure cardiac output. The results showed that this model can help clinicians evaluate the potential benefit of using direct Fick method for measuring cardiac output in the diagnostic work-up and determine its utility in confirming or ruling out a diagnosis of PcPH.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Pediatrics
Adam W. Powell, Wayne A. Mays, Samuel G. Wittekind, Clifford Chin, Sandra K. Knecht, Sean M. Lang, Alexander R. Opotowsky
Summary: This study investigated the impact of sport and school restrictions during the early stages of the COVID-19 pandemic on exercise performance and body composition in children and young adults with heart disease. The results showed that the pandemic and related lifestyle changes did not have substantial negative effects on aerobic fitness or body composition in this population.
FRONTIERS IN PEDIATRICS
(2023)
Article
Pediatrics
Tanguy Dutilleux, Nesrine Farhat, Ruth Heying, Marie-Christine Seghaye, Maurice Beghetti
Summary: This case report presents a 7-year-old boy with idiopathic pulmonary arterial hypertension (IPAH), who initially presented with cardiac murmur and exercise intolerance. Pulmonary hypertension was confirmed by echocardiography and cardiac catheterization. Despite treatment with sildenafil and bosentan, the patient's pulmonary artery pressure remained stable but not decreased. Subsequent follow-up showed an increase in estimated pulmonary pressure and worsening of the child's condition.
Article
Cardiac & Cardiovascular Systems
Humberto Garcia Aguilar, Matthias Gorenflo, D. Dunbar Ivy, Shahin Moledina, Biagio Castaldi, Hidekazu Ishida, Jacek Kusa, Oliver Miera, Joseph Pattathu, Ken-Pen Weng, Laszlo Ablonczy, Christian Apitz, Marta Katona, Kenichi Kurosaki, Tomas Pulido, Hiroyuki Yamagishi, Kazushi Yasuda, Galia Cisternas, Melanie Goth, Susanne Lippert, Anna Radomskyj, Soundos Saleh, Stefan Willmann, Gabriela Wirsching, Damien Bonnet, Maurice Beghetti, Pawel Czegniewicz
Summary: This study evaluated the safety and pharmacokinetics of oral riociguat in pediatric patients with PAH. The results showed that riociguat plasma concentrations in pediatric patients were consistent with those in adult patients, confirming a suitable dosing strategy for pediatric patients. Although adverse events were reported in some patients, most of them were mild or moderate, and the serious adverse events related to the study drug were resolved. The data suggest that riociguat may have an acceptable safety profile and potential efficacy.
PULMONARY CIRCULATION
(2022)
Proceedings Paper
Computer Science, Artificial Intelligence
Mustafa Besirli, Kerim Ture, Maurice Beghetti, Catherine Dehollain, Marco Mattavelli, Diego Barrettino
Summary: This paper presents an energy and area efficient capacitively-coupled chopper instrumentation amplifier (CCIA) designed for implantable bridge sensor systems. Chopper stabilization is used to reduce offset and 1/f noise, and a switched-capacitor ripple reduction loop is employed to suppress the resulting ripple. The proposed CCIA, fabricated in a standard 0.18 μm CMOS process, achieves low input noise, worst-case input offset, and output ripple, making it suitable for use with various bridge sensors.
2022 20TH IEEE INTERREGIONAL NEWCAS CONFERENCE (NEWCAS)
(2022)
Meeting Abstract
Medicine, General & Internal
G. Milano, M. Reinero, S. Allie, F. Porte-Thome, M. Beghetti
SWISS MEDICAL WEEKLY
(2022)
Meeting Abstract
Medicine, General & Internal
G. Omar, A. Maggio, Q. Chatelain, A. Carcaterra, A. Planchat, M. A. El-Aji, M. Beghetti, J. Wacker Bou Puigdefabregas, A. Schneider-Paccot, M. Namdar, M. Albinski, V. Gabus, P. Meyer
SWISS MEDICAL WEEKLY
(2022)
Meeting Abstract
Cardiac & Cardiovascular Systems
S. E. Fahnhorst, K. Brandewie, T. Perry, A. R. Opotowsky, A. M. Lubert, A. Lorts, D. L. Morales, C. R. Villa
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2022)
Article
Cardiac & Cardiovascular Systems
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
Summary: This study developed and validated a risk prediction model for adult congenital heart disease (ACHD), which showed good accuracy and applicability in predicting one-year risk of death, heart failure, or arrhythmia.
EUROPEAN HEART JOURNAL-QUALITY OF CARE AND CLINICAL OUTCOMES
(2022)
Article
Cardiac & Cardiovascular Systems
Kashvi Gupta, Ioannis Mastoris, Andrew J. Sauer
HEART FAILURE CLINICS
(2024)
Review
Cardiac & Cardiovascular Systems
Alexander L. Wallner, Salvatore Savona, Rami Kahwash
HEART FAILURE CLINICS
(2024)
Article
Cardiac & Cardiovascular Systems
Muhammad Shahzeb Khan, Anousheh Awais Paracha, Jan Biegus, Rafael de la Espriella, Julio Nunez, Carlos G. Santos-Gallego, Dmitry Yaranov, Marat Fudim
Summary: The increase of preload in heart failure can lead to excessive intracardiac pressures. The reduction of preload can be achieved through pharmacological and nonpharmacological interventions, with nonpharmacological interventions aiming to reduce blood inflow or increase splanchnic vascular blood pooling.
HEART FAILURE CLINICS
(2024)
Article
Cardiac & Cardiovascular Systems
Parin J. Patel, Asim S. Ahmed
HEART FAILURE CLINICS
(2024)
Article
Cardiac & Cardiovascular Systems
Husam M. Salah, Claudia Baratto, Dmitry M. Yaranov, Karl-Philipp Rommel, Satyanarayana Achanta, Sergio Caravita, Vinay Kumar Reddy Vasanthu, Marat Fudim
HEART FAILURE CLINICS
(2024)
Article
Cardiac & Cardiovascular Systems
Gregory R. Jackson, Abhinav Singh
HEART FAILURE CLINICS
(2024)
Review
Cardiac & Cardiovascular Systems
Ronald D. Bass, Joseph Phillips, Jorge Sanz Sanchez, Priti Shah, Stephen Sum, Ron Waksman, Hector M. Garcia-Garcia
Summary: NIRS-derived LCBI is an effective measurement for identifying vulnerable patients and plaques at risk of future adverse events. Patients with an elevated LCBI have higher odds of enduring a future adverse event.
HEART FAILURE CLINICS
(2024)
Article
Cardiac & Cardiovascular Systems
Adam Bland, Eunice Chuah, William Meere, Thomas J. Ford
Summary: CMD is a challenging condition to manage due to its heterogenous pathophysiology, presentation, and response to therapy. Although awareness of CMD is improving, there is a lack of randomized trials for therapy. Invasive assessment of the coronary microcirculation can improve patient-centered outcomes. Beta-blockers are the cornerstone of therapy for CMD angina, and non-pharmacological interventions play a central role in management. Further research is needed to assess the impact of traditional and novel pharmacological therapies on symptoms and clinical events in different CMD endotypes.
HEART FAILURE CLINICS
(2024)
