Unilateral absent pulmonary artery in an adult - A diagnostic and therapeutic challenge.

Autor:	Seedat F; Department of Internal Medicine, Faculty of Health Sciences, University of Witwatersrand, Johannesburg, South Africa., Kalla IS; Division of Pulmonology, Department of Internal Medicine, Charlotte Maxeke Johannesburg Academic Hospital and Faculty of Health Sciences, University of Witwatersrand, Johannesburg, South Africa., Feldman C; Division of Pulmonology, Department of Internal Medicine, Charlotte Maxeke Johannesburg Academic Hospital and Faculty of Health Sciences, University of Witwatersrand, Johannesburg, South Africa.
Jazyk:	angličtina
Zdroj:	Respiratory medicine case reports [Respir Med Case Rep] 2017 Sep 14; Vol. 22, pp. 238-242. Date of Electronic Publication: 2017 Sep 14 (Print Publication: 2017).
DOI:	10.1016/j.rmcr.2017.09.004
Abstrakt:	Unilateral absent pulmonary artery (UAPA) is a congenital abnormality rarely diagnosed in adults. UAPA has a myriad of clinical presentations and pulmonary hypertension is present in a quarter of all cases. Isolated UAPA commonly affects the right pulmonary artery and occurs as a result of abnormal development of the sixth aortic arch segment. Due to its rarity, it remains a diagnostic and therapeutic challenge. We describe a case of UAPA in an adult presenting with severe pulmonary hypertension. We describe the appropriate diagnostic approach to a patient with pulmonary hypertension and illustrate the importance of a detailed evaluation to determine the underlying aetiology, particularly in rare causes. Furthermore, we review the clinical presentation, diagnosis and management challenges of UAPA in adults.
Databáze:	MEDLINE
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