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Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis

Pulmonary hypoplasia or agenesis is a rare congenital disorder that results in lung underdevelopment. This disease is usually found in children but rarely encountered in adults. We describe the case of an 84-year-old woman diagnosed with a unilateral pulmonary hypoplasia presenting simultaneously wi...

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Autores principales: Pinheiro, Guiomar, Alves, Ana Margarida, Rodrigues Neves, Isabel, Sequeira, Teresa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SMC Media Srl 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7350963/
https://www.ncbi.nlm.nih.gov/pubmed/32665919
http://dx.doi.org/10.12890/2020_001490
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author Pinheiro, Guiomar
Alves, Ana Margarida
Rodrigues Neves, Isabel
Sequeira, Teresa
author_facet Pinheiro, Guiomar
Alves, Ana Margarida
Rodrigues Neves, Isabel
Sequeira, Teresa
author_sort Pinheiro, Guiomar
collection PubMed
description Pulmonary hypoplasia or agenesis is a rare congenital disorder that results in lung underdevelopment. This disease is usually found in children but rarely encountered in adults. We describe the case of an 84-year-old woman diagnosed with a unilateral pulmonary hypoplasia presenting simultaneously with left pulmonary artery hypoplasia. Due to this condition, the patient had a lifelong history of pulmonary infections that resulted in several bronchiectases in the affected lung. Moreover, the pulmonary artery hypoplasia led to the development of pulmonary hypertension and collateral circulation causing hemoptysis, giving rise to the patient attending the emergency department. Although we were able to medically manage the hemoptysis, it can be fatal and require surgical intervention. Hence, an early diagnosis is essential so that appropriate follow-up and prompt prevention and treatment of complications, such as pulmonary infections, hemoptysis and pulmonary hypertension, are achieved. LEARNING POINTS: Although rare and typically associated with childhood, lung and pulmonary artery hypoplasia can in exceptional cases be present in adult life. The diagnosis of lung and pulmonary artery hypoplasia is challenging in adults as they can easily be mistaken for more common diseases. Symptoms and complications may be life-threatening, including dyspnea, hemoptysis, recurrent pulmonary infections and pulmonary hypertension. An early diagnosis is extremely important because it allows a close follow-up with consequent timely detection and treatment of serious complications, especially pulmonary hypertension.
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spelling pubmed-73509632020-07-13 Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis Pinheiro, Guiomar Alves, Ana Margarida Rodrigues Neves, Isabel Sequeira, Teresa Eur J Case Rep Intern Med Articles Pulmonary hypoplasia or agenesis is a rare congenital disorder that results in lung underdevelopment. This disease is usually found in children but rarely encountered in adults. We describe the case of an 84-year-old woman diagnosed with a unilateral pulmonary hypoplasia presenting simultaneously with left pulmonary artery hypoplasia. Due to this condition, the patient had a lifelong history of pulmonary infections that resulted in several bronchiectases in the affected lung. Moreover, the pulmonary artery hypoplasia led to the development of pulmonary hypertension and collateral circulation causing hemoptysis, giving rise to the patient attending the emergency department. Although we were able to medically manage the hemoptysis, it can be fatal and require surgical intervention. Hence, an early diagnosis is essential so that appropriate follow-up and prompt prevention and treatment of complications, such as pulmonary infections, hemoptysis and pulmonary hypertension, are achieved. LEARNING POINTS: Although rare and typically associated with childhood, lung and pulmonary artery hypoplasia can in exceptional cases be present in adult life. The diagnosis of lung and pulmonary artery hypoplasia is challenging in adults as they can easily be mistaken for more common diseases. Symptoms and complications may be life-threatening, including dyspnea, hemoptysis, recurrent pulmonary infections and pulmonary hypertension. An early diagnosis is extremely important because it allows a close follow-up with consequent timely detection and treatment of serious complications, especially pulmonary hypertension. SMC Media Srl 2020-04-30 /pmc/articles/PMC7350963/ /pubmed/32665919 http://dx.doi.org/10.12890/2020_001490 Text en © EFIM 2020 This article is licensed under a Commons Attribution Non-Commercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/)
spellingShingle Articles
Pinheiro, Guiomar
Alves, Ana Margarida
Rodrigues Neves, Isabel
Sequeira, Teresa
Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis
title Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis
title_full Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis
title_fullStr Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis
title_full_unstemmed Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis
title_short Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis
title_sort left lung and pulmonary artery hypoplasia: a rare case of hemoptysis
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7350963/
https://www.ncbi.nlm.nih.gov/pubmed/32665919
http://dx.doi.org/10.12890/2020_001490
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