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
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
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
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
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)
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
Biochemistry & Molecular Biology
Pier Paolo Bassareo, Michele D'Alto
Summary: Pulmonary hypertension (PH) is a complex disease that can lead to irreversible pulmonary arterial hypertension (PAH) and death. Metabolomics is a laboratory technique that can provide insights into the metabolic pathways related to PH/PAH. This systematic review identified specific metabolic fingerprints and highlighted the potential for new therapeutic agents targeting the disarranged metabolic pathways.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Cardiac & Cardiovascular Systems
Annika Freiberger, Amely Busse, Peter Ewert, Michael Huntgeburth, Harald Kaemmerer, Niko Kohls, Nicole Nagdyman, Cristina Richter, Christina Roehrich, Fabian von Scheidt, Michael Weyand, Sebastian Freilinger, Caroline Andonian
Summary: This study found that patients with pulmonary hypertension (PH) had worse overall quality of life (QOL), particularly in the areas of mobility, self-care, and usual activities. Anxiety and depression, as well as pain and discomfort, were the most impaired dimensions in both groups of patients.
CARDIOVASCULAR DIAGNOSIS AND THERAPY
(2022)
Article
Cardiac & Cardiovascular Systems
Katherine Kearney, Edmund M. Lau, David Darley, Anitra Romfh, Nicole Bart, Eugene Kotlyar, Don Hayes, Kiran Khush, Anne Keogh
Summary: The study found that in the current era, ES-ASD or ES-VSD patients represented a decreasing proportion of all lung or heart-lung transplants compared to other indications. The odds of transplantation for ES-ASD and ES-VSD patients were significantly lower compared to other candidates for lung transplantation. In the early era, ES-ASD patients who underwent heart-lung transplants had equivalent survival rates to those who underwent lung transplants, while ES-VSD patients had superior survival rates.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(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
Medicine, General & Internal
Katharina Meinel, Martin Koestenberger, Hannes Sallmon, Georg Hansmann, Guido E. Pieles
Summary: Invasive assessment in the catheterization laboratory is the gold standard for diagnosing pulmonary hypertension in children, but transthoracic echocardiography (TTE) is the initial diagnostic tool. Guidelines suggest key echocardiographic variables for screening, but these may not apply to patients with congenital heart disease (CHD). Misinterpretation of TTE variables can lead to delayed diagnosis and therapy, or unnecessary invasive procedures with risks in the pediatric age group.
Article
Cardiac & Cardiovascular Systems
Ann-Sophie Kaemmerer, Matthias Gorenflo, Doerte Huscher, David Pittrow, Peter Ewert, Christine Pausch, Marion Delcroix, Hossein A. Ghofrani, Marius M. Hoeper, Rainer Kozlik-Feldmann, Andris Skride, Gerd Staehler, Carmine Dario Vizza, Elena Jureviciene, Dovile Jancauskaite, Lina Gumbiene, Ralf Ewert, Ingo Daehnert, Matthias Held, Michael Halank, Dirk Skowasch, Hans Klose, Heinrike Wilkens, Katrin Milger, Christian Jux, Martin Koestenberger, Laura Scelsi, Eva Brunnemer, Michael Hofbeck, Silvia Ulrich, Anton Vonk Noordegraaf, Tobias J. Lange, Leonhard Bruch, Stavros Konstantinides, Martin Claussen, Judith Loeffler-Ragg, Hubert Wirtz, Christian Apitz, Rhoia Neidenbach, Sebastian Freilinger, Attila Nemes, Christian Opitz, Ekkehard Gruenig, Stephan Rosenkranz
Summary: This study analyzed management strategies and treatment concepts in PAH-CHD patients based on data from the COMPERA-CHD registry. The findings showed a trend towards more aggressive treatment strategies and combination therapies, emphasizing the importance of focusing on Non-Eisenmenger PAH group and patients with complex CHD in the future.
CARDIOVASCULAR DIAGNOSIS AND THERAPY
(2021)
Article
Cardiac & Cardiovascular Systems
Gerhard Paul Diller, Maria Luisa Benesch Vidal, Aleksander Kempny, Kana Kubota, Wei Li, Konstantinos Dimopoulos, Alexandra Arvanitaki, Astrid E. Lammers, Stephen J. Wort, Helmut Baumgartner, Stefan Orwat, Michael A. Gatzoulis
Summary: This study aims to test the hypothesis that deep learning networks can reliably detect pulmonary arterial hypertension (PAH) and provide prognostic information. The results showed that the DL algorithm achieved high accuracy and sensitivity in detecting PAH and outperformed conventional methods. Automatically determined DL parameters were significantly related to prognosis, and DL parameters were non-inferior to measures obtained manually by expert echocardiographers in predicting outcome.
EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING
(2022)
Article
Respiratory System
Timothy J. W. Dawes, Colm McCabe, Konstantinos Dimopoulos, Iain Stewart, Simon Bax, Carl Harries, Chinthaka B. Samaranayake, Aleksander Kempny, Philip L. Molyneaux, Samuel Seitler, Thomas Semple, Wei Li, Peter M. George, Vasileios Kouranos, Felix Chua, Elisabetta A. Renzoni, Maria Kokosi, Gisli Jenkins, Athol U. Wells, Stephen J. Wort, Laura C. Price
Summary: This study investigated the potential benefit of phosphodiesterase 5 inhibitors (PDE5i) in improving survival for patients with interstitial lung disease-associated pulmonary hypertension (ILD-PH). The results showed that patients treated with PDE5i had longer survival compared to untreated patients, with a particularly significant difference when right ventricular function was normal at presentation. These findings suggest that PDE5i treatment in ILD-PH should be further studied through a prospective randomized trial.
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.
Article
Cardiac & Cardiovascular Systems
Anna C. Mavromanoli, Stefano Barco, Walter Ageno, Helene Bouvaist, Marianne Brodmann, Claudio Cuccia, Francis Couturaud, Claudia Dellas, Konstantinos Dimopoulos, Daniel Duerschmied, Klaus Empen, Pompilio Faggiano, Emile Ferrari, Nazzareno Galie, Marcello Galvani, Alexandre Ghuysen, George Giannakoulas, Menno Huisman, David Jimenez, Matija Kozak, Irene M. Lang, Nicolas Meneveau, Thomas Muenzel, Massimiliano Palazzini, Antoniu Octavian Petris, Giancarlo Piovaccari, Aldo Salvi, Sebastian Schellong, Kai-Helge Schmidt, Franck Verschuren, Irene Schmidtmann, Gerrit Toenges, Frederikus A. Klok, Stavros Konstantinides
Summary: This study analyzed the changes in RV function in patients with intermediate-risk PE who were switched early to oral anticoagulation. The results showed that the majority of patients had normalized RV function within 6 days and maintained normal RV function throughout the first 6 months. However, almost one in four patients continued to have evidence of RV dysfunction over the long term.
CLINICAL RESEARCH IN CARDIOLOGY
(2023)
Editorial Material
Cardiac & Cardiovascular Systems
Andrew Constantine, Konstantinos Dimopoulos, Ee Ling Heng, Aleksander Kempny
INTERNATIONAL JOURNAL OF CARDIOLOGY
(2023)
Letter
Respiratory System
Chinthaka B. Samaranayake, Aleksander Kempny, Robert Naeije, Michael Gatzoulis, Laura C. Price, Konstantinos Dimopoulos, Lan Zhao, Stephen J. Wort, Colm McCabe
EUROPEAN RESPIRATORY JOURNAL
(2023)
Article
Cardiac & Cardiovascular Systems
Kaushiga Krishnathasan, Konstantinos Dimopoulos, Neill Duncan, Piera Ricci, Alexander Kempny, Isma Rafiq, Michael A. Gatzoulis, Ee Ling Heng, Sarah Blakey, Claudia Montanaro, Sonya Babu-Narayan, Darrel P. Francis, Wei Li, Andrew Constantine
Summary: Renal dysfunction in adults with congenital heart disease (CHD) and heart failure is associated with disease progression, increased diuretic doses, and longer hospital stays. Chronic kidney disease (CKD) is common and triples the risk of death, transplantation, or ventricular assist device. Intensified monitoring, optimization of medical therapy, and collaborative management with renal physicians are necessary in these patients.
EUROPEAN JOURNAL OF PREVENTIVE CARDIOLOGY
(2023)
Review
Respiratory System
Kaushiga Krishnathasan, Andrew Constantine, Isma Rafiq, Ana Barrradas Pires, Hannah Douglas, Laura C. Price, Konstantinos Dimopoulos
Summary: Pregnancy is generally contraindicated in PAH due to high maternal mortality. Specialist care and counseling are essential for women with PAH wishing to become pregnant, in order to minimize risk and improve outcomes.
EXPERT REVIEW OF RESPIRATORY MEDICINE
(2023)
Article
Cardiac & Cardiovascular Systems
Piera Ricci, Konstantinos Dimopoulos, Melissa Bouchard, Cheryl Chong Zhiya, Virginia Castro Meira, Danielle Pool, Michelle Lambell, Isma Rafiq, Aleksander Kempny, Ee Ling Heng, Michael A. Gatzoulis, Loredana Haidu, Andrew Constantine
Summary: Less than one-third of adolescents with congenital heart disease (CHD) successfully transition to adult care and miss out on education about their condition. We assessed the efficacy of our transition clinic and identified factors associated with successful transition.
EUROPEAN HEART JOURNAL-QUALITY OF CARE AND CLINICAL OUTCOMES
(2023)
Review
Critical Care Medicine
Samuel Seitler, Konstantinos Dimopoulos, Sabine Ernst, Laura C. Price
Summary: Managing acute medical emergencies in patients with pulmonary hypertension (PH) is challenging, and this review focuses on both pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), which are the more severe groups. Assessment and management protocols are provided, encompassing respiratory and cardiac emergencies, as well as PH-specific emergencies. Multidisciplinary team approach and communication with specialist centers are emphasized.
SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Cardiac & Cardiovascular Systems
Elena Surkova, Andrew Constantine, Zhuoyuan Xu, Teresa Segura de la Cal, Daniela Bispo, Cathy West, Roxy Senior, Konstantinos Dimopoulos, Wei Li
Summary: This study aimed to evaluate the additional prognostic significance of echocardiographic parameters in patients with a systemic right ventricle (SRV). The results showed that echocardiographic parameters can predict mortality and transplantation rates in these patients, and the assessment of subpulmonary left ventricular (LV) size and function is closely related to prognosis. Accurate echocardiographic evaluation of SRV and subpulmonary LV is therefore crucial for risk stratification and patient management.
EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING
(2023)
Article
Cardiac & Cardiovascular Systems
Ana Barradas-Pires, Pablo Meras, Andrew Constantine, Giulia Costola, Teresa Segura de la Cal, Isma Rafiq, Aleksander Kempny, Wei Li, Sonya V. Babu-Narayan, J. Andreas Hoschtitzky, Michael A. Gatzoulis, Antonio Martinez Rubio, Konstantinos Dimopoulos
Summary: This study investigates the indications and outcomes of aortic valve replacement (AVR) in young adults with severe aortic regurgitation, as well as the relationship between presurgical echocardiographic parameters and postoperative left ventricular size and function. The results show that a higher presurgical left ventricular end-systolic diameter is associated with a lack of left ventricular normalization after AVR. Pre- and postoperative left ventricular dimensions and postoperative left ventricular ejection fraction predict clinical events during follow-up.
JOURNAL OF THE AMERICAN HEART ASSOCIATION
(2023)
Article
Cardiac & Cardiovascular Systems
Sian Chivers, Aoife Cleary, Rachel Knowles, Sonya Babu-Narayan, John M. Simpson, Heba Nashat, Konstantinos Dimopoulos, Michael A. Gatzoulis, Dirk Wilson, Milos Prica, James Anthony, Paul F. Clift, Victoria Jowett, Petra Jenkins, Bernadette Khodaghalian, Caroline B. Jones, Antonia Hardiman, Catherine Head, Owen Miller, Natali A. Y. Chung, Umar Mahmood, Frances A. Bu'Lock, Tristan K. W. Ramcharan, Ashish Chikermane, Jennifer Shortland, Andrew Tometzki, David S. Crossland, Zdenka Reinhardt, Clive Lewis, Leila Rittey, Dominic Hares, Olga Panagiotopoulou, Benjamin Smith, Muhammad L. Najih, Tara Bharucha, Piers E. F. Daubeney
Summary: This study examines the impact of COVID-19 on patients with congenital heart disease (CHD) and identifies risk factors for adverse outcomes. The study finds that children with CHD have a lower risk of death and hospitalization due to COVID-19, but younger children have higher hospital admission rates. In adults, the likelihood of death increases with age and pulmonary arterial hypertension, while the likelihood of hospitalization increases with age, comorbidities, and genetic disease.
Meeting Abstract
Cardiac & Cardiovascular Systems
Zhuoyuan Xu, Andrew Constantine, Konstantinos Dimopoulos, Hong Gu
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
(2023)
Meeting Abstract
Respiratory System
A. Constantine, C. J. Rhodes, P. Ricci, W. Li, L. C. Price, C. Mccabe, J. Wharton, M. R. Wilkins, L. S. Howard, K. Dimopoulos, S. J. Wort
EUROPEAN RESPIRATORY JOURNAL
(2022)