Pulmonary Hypertension in Thalassemia Patients

Pulmonary hypertension (PH) is defined in children as a mean pulmonary arterial pressure (PAP) greater than 25 mmHg at rest or 30 mmHg during physical activity, with increased pulmonary artery capillary wedge pressure and an increased pulmonary vascular resistance greater than 3 Wood units × M2. it...

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Autores principales: Alwataify, Ahmed Shemran Mutlaq, Alfatlawy, Sabih Salih, Altufaily, Yahia Abid Alshahid
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Publicado: IntechOpen 2021
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Acceso en línea:https://www.intechopen.com/chapters/79310
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author Alwataify, Ahmed Shemran Mutlaq
Alfatlawy, Sabih Salih
Altufaily, Yahia Abid Alshahid
author_browse Alfatlawy, Sabih Salih
Altufaily, Yahia Abid Alshahid
Alwataify, Ahmed Shemran Mutlaq
author_facet Alwataify, Ahmed Shemran Mutlaq
Alfatlawy, Sabih Salih
Altufaily, Yahia Abid Alshahid
author_sort Alwataify, Ahmed Shemran Mutlaq
building IntechOpen
collection Acceso abierto
description Pulmonary hypertension (PH) is defined in children as a mean pulmonary arterial pressure (PAP) greater than 25 mmHg at rest or 30 mmHg during physical activity, with increased pulmonary artery capillary wedge pressure and an increased pulmonary vascular resistance greater than 3 Wood units × M2. it is the main cause of morbidity and mortality in the group of thalassemia, if no treatment leads to right ventricular heart failure and death. The development of pulmonary arterial hypertension (PAH) is assumed to be the result of many multifactorial pathogenic mechanisms including chronic hemolysis, iron overload, hypercoagulability, and erythrocyte dysfunction as a result of splenectomy, inflammation and nitric oxide (NO) depletion. PAH symptoms are non-specific, their signs consist of right ventricular lift, an accentuated pulmonary component of the second heart sound, a (gallop rhythm) right ventricular third heart sound, and parasternal heave meaning a hypertrophied right ventricle. The diagnosis of PAH requires a clinical suspicion based on symptoms and physical examination. Echocardiography is frequently used to screen for PAH, monitor progression over time and allow identification of patients for whom diagnostic right heart catheterization (RHC) is warranted and its treatment includes hemoglobinopathy specific treatment and PAH specific therapy.
doi_str_mv 10.5772/intechopen.101052
first_indexed 2026-10-15T21:39:27Z
format Book Chapter
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spelling intech-793102021-11-25 Pulmonary Hypertension in Thalassemia Patients Ahmed Shemran Mutlaq Alwataify Sabih Salih Alfatlawy Yahia Abid Alshahid Altufaily Health Sciences Pulmonary hypertension (PH) is defined in children as a mean pulmonary arterial pressure (PAP) greater than 25 mmHg at rest or 30 mmHg during physical activity, with increased pulmonary artery capillary wedge pressure and an increased pulmonary vascular resistance greater than 3 Wood units × M2. it is the main cause of morbidity and mortality in the group of thalassemia, if no treatment leads to right ventricular heart failure and death. The development of pulmonary arterial hypertension (PAH) is assumed to be the result of many multifactorial pathogenic mechanisms including chronic hemolysis, iron overload, hypercoagulability, and erythrocyte dysfunction as a result of splenectomy, inflammation and nitric oxide (NO) depletion. PAH symptoms are non-specific, their signs consist of right ventricular lift, an accentuated pulmonary component of the second heart sound, a (gallop rhythm) right ventricular third heart sound, and parasternal heave meaning a hypertrophied right ventricle. The diagnosis of PAH requires a clinical suspicion based on symptoms and physical examination. Echocardiography is frequently used to screen for PAH, monitor progression over time and allow identification of patients for whom diagnostic right heart catheterization (RHC) is warranted and its treatment includes hemoglobinopathy specific treatment and PAH specific therapy. IntechOpen 2021-11-25 Chapter, Part Of Book https://www.intechopen.com/chapters/79310 doi:10.5772/intechopen.101052 en ISBN:978-1-83969-716-6 https://creativecommons.org/licenses/by/3.0/ https://www.intechopen.com/books/10876 ; Blood - Updates on Hemodynamics and on Thalassemia
spellingShingle Health Sciences
Alwataify, Ahmed Shemran Mutlaq
Alfatlawy, Sabih Salih
Altufaily, Yahia Abid Alshahid
Pulmonary Hypertension in Thalassemia Patients
title Pulmonary Hypertension in Thalassemia Patients
title_full Pulmonary Hypertension in Thalassemia Patients
title_fullStr Pulmonary Hypertension in Thalassemia Patients
title_full_unstemmed Pulmonary Hypertension in Thalassemia Patients
title_short Pulmonary Hypertension in Thalassemia Patients
title_sort pulmonary hypertension in thalassemia patients
topic Health Sciences
url https://www.intechopen.com/chapters/79310
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