The Varied Manifestation of Pulmonary Artery Agenesis in Adulthood

Bouros, Demosthenes; Paré, Peter D.; Panagou, Panagiotis; Tsintiris, Kostas; Siafakas, Nikolaos

doi:10.1378/chest.108.3.670

Cited by 241 publications

(276 citation statements)

References 25 publications

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“…Our findings both extend the existing knowledge and provide novel observations regarding the clinical characteristics of isolated UAPA in adulthood. Almost 85% of adult patients with isolated UAPA in our study were symptomatic, close to 70-87% reported in previous studies (4,13,14). However, the median duration of these symptomatic patients between onset of symptoms Data are presented as number (%).…”

Section: Discussionsupporting

confidence: 83%

Isolated unilateral absence of pulmonary artery in adulthood: a clinical analysis of 65 cases from a case series and systematic review

et al. 2017

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Background: Isolated unilateral absence of pulmonary artery (UAPA) in adulthood is a rare congenital anomaly. Although some case reports exist, the clinical symptomatology, lung parenchymal features, collateral circulation and therapeutic approaches in adult patients with isolated UAPA remain unknown. The objectives of this study are to investigate the clinical characteristics, elucidate the correlation between clinical symptomatology and radiology, and summarize treatment of adult patients with isolated UAPA. Methods: Cases of adult patients with isolated UAPA who had been diagnosed at our hospital and identified from PubMed, EMBASE and Web of Science from 1990 to 2016 were analyzed. Results: Hemoptysis was present in 41.5% of patients, exertional dyspnea in 41.5%, and recurrent respiratory infection in 35.4%. Lung parenchymal abnormalities were found on chest computed tomography (CT) scan, including bronchiectasis, which occurred in 30.2% of the patients, interstitial changes in 14.0%, and multiple bullae in 14.0% of the patients. Exertional dyspnea was more frequent in patients with pulmonary hypertension than in those without pulmonary hypertension (P<0.001). Recurrent respiratory infection were more frequent in patients with bronchiectasis than in those without bronchiectasis (P<0.001).Hypertrophic bronchial, phrenic, internal thoracic and intercostal arteries were found in 71.9%, 46.9%, 43.8%, and 43.8% of the patients, respectively. Pneumonectomy reduced hemoptysis in seven cases. Oral phosphodiesterase inhibitors or endothelin receptor antagonist improved exertional dyspnea in three cases with pulmonary hypertension. Conclusions: Clinicians should be aware of undiagnosed cases of isolated UAPA in adults with unexplained hemoptysis or exertional dyspnea. Early recognition and management of isolated UAPA in adult patients are crucial to avoid the devastating effect of massive hemoptysis or severe pulmonary hypertension (PHT) in the long term.

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Section: Discussionsupporting

confidence: 83%

Isolated unilateral absence of pulmonary artery in adulthood: a clinical analysis of 65 cases from a case series and systematic review

et al. 2017

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“…Its prevalence as an isolated lesion is estimated at 1 in 200 000‐to‐300 000 adults,10, 18, 19, 20 and 80% of reported cases involving the left PA have been associated with coexisting CHD, such as TOF or truncus arteriosus 10, 19. In 2011, a review of the literature reported 352 cases of unilateral “absence” of pulmonary artery; two thirds (n=237) were associated with other CHD 21.…”

Section: Conditions In Which Segmental Ph May Developmentioning

confidence: 99%

Definition and Management of Segmental Pulmonary Hypertension

Dimopoulos

Diller

Opotowsky

et al. 2018

JAHA

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“…Pulmonary artery hypoplasia and agenesis are the other vascular diseases in the differential diagnosis. Whilst volume decrease is detected in pulmonary artery hypoplasia, lung volume is decreased in the involved side, no air trapping is observed on radiographs obtained during expirium, almost no perfusion is observed in the involved side on V/Q scintigraphy, and ventilation is normal in the pulmonary artery agenesis [16,17]. Moreover, pulmonary artery agenesis is usually seen together with other cardiovascular anomalies.…”

Section: Discussionmentioning

confidence: 98%