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Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management

Haemoptysis represents one of the most severe major bleeding manifestations in the clinical course of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Accumulating evidence indicates that dysfunction of the pulmonary vascular bed in the setting of PAH predisposes...

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Autores principales: Baroutidou, Amalia, Arvanitaki, Alexandra, Hatzidakis, Adam, Pitsiou, Georgia, Ziakas, Antonios, Karvounis, Haralambos, Giannakoulas, George
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8836348/
https://www.ncbi.nlm.nih.gov/pubmed/35160084
http://dx.doi.org/10.3390/jcm11030633
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author Baroutidou, Amalia
Arvanitaki, Alexandra
Hatzidakis, Adam
Pitsiou, Georgia
Ziakas, Antonios
Karvounis, Haralambos
Giannakoulas, George
author_facet Baroutidou, Amalia
Arvanitaki, Alexandra
Hatzidakis, Adam
Pitsiou, Georgia
Ziakas, Antonios
Karvounis, Haralambos
Giannakoulas, George
author_sort Baroutidou, Amalia
collection PubMed
description Haemoptysis represents one of the most severe major bleeding manifestations in the clinical course of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Accumulating evidence indicates that dysfunction of the pulmonary vascular bed in the setting of PAH predisposes patients to increased hemorrhagic diathesis, resulting in mild to massive and life-threatening episodes of haemoptysis. Despite major advances in PAH targeted treatment strategies, haemoptysis is still correlated with substantial morbidity and impaired quality of life, requiring a multidisciplinary approach by adult CHD experts in tertiary centres. Technological innovations in the field of diagnostic and interventional radiology enabled the application of bronchial artery embolization (BAE), a valuable tool to efficiently control haemoptysis in modern clinical practice. However, bleeding recurrences are still prevalent, implying that the optimum management of haemoptysis and its implications remain obscure. Moreover, regarding the use of oral anticoagulation in patients with haemoptysis, current guidelines do not provide a clear therapeutic strategy due to the lack of evidence. This review aims to discuss the main pathophysiological mechanisms of haemoptysis in PAH-CHD, present the clinical spectrum and the available diagnostic tools, summarize current therapeutic challenges, and propose directions for future research in this group of patients.
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spelling pubmed-88363482022-02-12 Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management Baroutidou, Amalia Arvanitaki, Alexandra Hatzidakis, Adam Pitsiou, Georgia Ziakas, Antonios Karvounis, Haralambos Giannakoulas, George J Clin Med Review Haemoptysis represents one of the most severe major bleeding manifestations in the clinical course of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Accumulating evidence indicates that dysfunction of the pulmonary vascular bed in the setting of PAH predisposes patients to increased hemorrhagic diathesis, resulting in mild to massive and life-threatening episodes of haemoptysis. Despite major advances in PAH targeted treatment strategies, haemoptysis is still correlated with substantial morbidity and impaired quality of life, requiring a multidisciplinary approach by adult CHD experts in tertiary centres. Technological innovations in the field of diagnostic and interventional radiology enabled the application of bronchial artery embolization (BAE), a valuable tool to efficiently control haemoptysis in modern clinical practice. However, bleeding recurrences are still prevalent, implying that the optimum management of haemoptysis and its implications remain obscure. Moreover, regarding the use of oral anticoagulation in patients with haemoptysis, current guidelines do not provide a clear therapeutic strategy due to the lack of evidence. This review aims to discuss the main pathophysiological mechanisms of haemoptysis in PAH-CHD, present the clinical spectrum and the available diagnostic tools, summarize current therapeutic challenges, and propose directions for future research in this group of patients. MDPI 2022-01-26 /pmc/articles/PMC8836348/ /pubmed/35160084 http://dx.doi.org/10.3390/jcm11030633 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Baroutidou, Amalia
Arvanitaki, Alexandra
Hatzidakis, Adam
Pitsiou, Georgia
Ziakas, Antonios
Karvounis, Haralambos
Giannakoulas, George
Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management
title Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management
title_full Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management
title_fullStr Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management
title_full_unstemmed Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management
title_short Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management
title_sort haemoptysis in pulmonary arterial hypertension associated with congenital heart disease: insights on pathophysiology, diagnosis and management
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8836348/
https://www.ncbi.nlm.nih.gov/pubmed/35160084
http://dx.doi.org/10.3390/jcm11030633
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