Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

Griffin, Nyree; Mansfield, Lucy; Redmond, Karen; Dusmet, Michael; Goldstraw, Peter; Mittal, Tarun; Padley, Simon

doi:10.1016/j.crad.2006.10.006

Cited by 84 publications

(110 citation statements)

References 13 publications

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“…Our view is supported by the literature indicating that reduction in pulmonary blood flow prevents the development of lung and causes lung hypoplasia (8,9).…”

Section: Discussionsupporting

confidence: 79%

Pulmonary Artery Agenesis and Lung Hypoplasia

Sunam

Ceran

2009

ELECTRON J GEN MED

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“…Our view is supported by the literature indicating that reduction in pulmonary blood flow prevents the development of lung and causes lung hypoplasia (8,9).…”

Section: Discussionsupporting

confidence: 79%

Pulmonary Artery Agenesis and Lung Hypoplasia

Sunam

Ceran

2009

ELECTRON J GEN MED

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“…The angiography remains the method of choice for the diagnosis of pulmonary artery agenesis. But is reserved for cases where embolization for massive hemoptysis is indicated or for pre-operatory evaluation of patients undergoing surgical revascularization [4,7]. Treatment strategies of PAA depend very much on the clinical framework.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Artery Agenesis in Young Adult - Two Case Reports

Serra¹

2017

Int J Clin Cardiol

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A 21-year-old woman presented for a history of exertional dyspnoea and atypical chest pain. No history of allergies or tuberculosis. A chest X-ray showed asymmetry of the chest walls from a reduced expansion of the right lung caused by parenchymal loss and right deviation of the mediastinum and ipsilateral elevation of the diaphragm. Transthoracic echocardiogram showed high value of estimated systolic pulmonary pressure (sPAP 45 mmHg) with normal right ventricle ( Figure 1A). Right pulmonary artery not viewable. Pulmonary scintigraphy CASE REPORT

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“…Isolated unilateral pulmonary artery agenesis (UPAA) has a non-specific presentation in adulthood [1]. Congenital absence of one pulmonary artery is rare, with an estimated prevalence of 1 in 200 000 [2].…”

Section: Discussionmentioning

confidence: 99%

Something's missing in the chest

Basu

Lapsia²

2010

BJR

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A 44-year old man presented with symptoms of a productive cough, night sweats and worsening breathlessness on exertion. This was associated with intermittent episodes of left-sided chest pain over several years, together with recurrent chest infections. He was a smoker of at least 10-15 pack years. No haemoptysis or significant weight loss was reported.On clinical examination, the findings were grossly normal. Spirometry revealed a mild obstructive function but no overwhelming evidence to support a diagnosis of bronchial asthma. The patient's inflammatory markers were normal. Microbiology cultures were negative for infective aetiology, including tuberculosis. What did the chest radiograph (Figure 1) show?The chest radiograph illustrated reduced volume of the right hemithorax with compensatory hyperinflation of the left lung. The left pulmonary artery was enlarged. Even when allowing for the slight rotational asymmetry, the right hilum was poorly identified. There was a generalised increase in vascular markings in the right lung, resulting in a hyperlucent left hemithorax. There was shift of the mediastinum to the right side.The differential diagnosis at this stage for a unilateral hyperlucent lung could be categorised by various aetiologies:(1) increased pulmonary air space with decreased blood flow -large airway obstruction with air trapping or endobronchial obstruction -small airway obstruction such as Swyer-JamesMacleod syndrome -pneumothorax in a supine patient;(2) pulmonary vascular cause such as pulmonary artery agenesis, congenital lobar emphysema or central pulmonary embolism; (3) poor radiographic technique; (4) overlying chest wall defect. DiagnosisA definitive diagnosis at this stage was not forthcoming, hence the patient was referred to the lung multidisciplinary meeting where the resulting decision was to perform a staging CT scan of the thorax to help elucidate the cause. What did the CT (Figure 2

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Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

Cited by 84 publications

References 13 publications

Pulmonary Artery Agenesis and Lung Hypoplasia

Pulmonary Artery Agenesis and Lung Hypoplasia

Pulmonary Artery Agenesis in Young Adult - Two Case Reports

Something's missing in the chest

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