Chest
Selected ReportsHereditary Telangiectasia and Multiple Pulmonary Arteriovenous Fistulas: Clinical Deterioration during Pregnancy
Section snippets
Case Report
At the age of 21, during the seventh month of pregnancy, the patient presented with symptoms of progressive fatigue, dyspnea and cyanosis. She had been an active child and at the age of 16; right lower lobe A-V fistula was discovered incidentally on a chest x-ray film. She underwent right lower lobectomy. Since her brother and father had multiple cutaneous A-V fistulas, the diagnosis of hereditary telangectasia was made. At age 19, she was hospitalized for symptoms of fatigue and
Discussion
The progression of dyspnea during pregnancy, the large shunt fraction and the severe nature of hypoxemia at the end of pregnancy indicate that the patient's shunt fraction and arterial hypoxemia must have increased significantly as her pregnancy progressed, even though objective documentation of this by serial arterial oxygen tension measurements is lacking. The most likely explanation for this increase in pulmonary arteriovenous shunting is the increase in blood volume which begins at eight
References (6)
- et al.
Clinical physiology in obstetrics
(1980) - et al.
Williams Obstetrics
(1985) - et al.
Pulmonary arteriovenous fistulas: Increase in shunt at high lung volume
Am Rev Respir Dis
(1977)
Cited by (54)
Lack of Growth of Small (≤2 mm Feeding Artery) Untreated Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia
2019, Journal of Vascular and Interventional RadiologyCitation Excerpt :It has been stated that PAVMs grow over time in adulthood, but this has not been well documented or detailed (4,11–13). It is also commonly postulated that growth occurs during pregnancy or puberty, which suggests a possible hormonal influence on their growth (14–17), again with little evidence to support this. This study sought to assess PAVM growth among patients with small untreated PAVMs to inform current practice and future guidelines.
Pulmonary Vascular Abnormalities
2015, Murray and Nadel's Textbook of Respiratory Medicine: Volume 1,2, Sixth EditionPulmonary arteriovenous malformations
2013, ChestCitation Excerpt :Patients with PAVM as well as all with HHT with or without PAVM are advised to be evaluated and followed in centers with high expertise in the management of these conditions. During pregnancy, PAVMs often increase in size and number.4,98,99 A study of 484 pregnancies in 199 women with HHT and PAVM demonstrated that the majority of patients were able to have a normal pregnancy.100
Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment
2010, Blood ReviewsCitation Excerpt :Currently, cerebral AVM development is thought to be complete during childhood,68 and for most individuals, pulmonary AVMs by the end of puberty.105 Further enlargement of AVMs occurs during pregnancy,106–109 and in specific settings. Understanding why vascular abnormalities arise and develop demands knowledge not only of HHT disease gene mutations, but also a broad understanding of normal vascular physiology.
Arterio-venous fistulas and related conditions
2010, Paediatric Cardiology