<abstract xmlns="http://www.w3.org/1999/xhtml"><p>Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular anomalies, such as tetralogy of Fallot, septal defects, right-sided aortic arch, or pulmonary atresia. Left-sided pulmonary artery agenesis is less frequent than the right-sided one. Diagnosis of unilateral pulmonary artery agenesis can be difficult. We report the case of a 15 year-old boy who presented with reduced exercise tolerance, shortness of breath and cyanosis. He was diagnosed with left pulmonary artery agenesis, associated with subaortic-ventricular septal defect, right-sided aortic arch, and severe pulmonary arterial hypertension (PAH), that precluded the surgical repair. Pulmonary vasodilator therapy was initiated in this case. The mortality rate of this rare anomaly is high due to its complications. It is essential to establish an early and correct diagnosis, in order to provide adequate treatment and prevent complications in this disease.</p></abstract>

Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular anomalies, such as tetralogy of Fallot, septal defects, right-sided aortic arch, or pulmonary atresia. Left-sided pulmonary artery agenesis is less frequent than the right-sided one. Diagnosis of unilateral pulmonary artery agenesis can be difficult. We report the case of a 15 year-old boy who presented with reduced exercise tolerance, shortness of breath and cyanosis. He was diagnosed with left pulmonary artery agenesis, associated with subaortic-ventricular septal defect, right-sided aortic arch, and severe pulmonary arterial hypertension (PAH), that precluded the surgical repair. Pulmonary vasodilator therapy was initiated in this case. The mortality rate of this rare anomaly is high due to its complications. It is essential to establish an early and correct diagnosis, in order to provide adequate treatment and prevent complications in this disease.

Left Pulmonary Artery Agenesis in a Pediatric Patient – Case Report

University of Medicine and Pharmacy, Tîrgu Mureș, Romania

County Emergency Clinical Hospital, Tîrgu Mureș, Romania

Journal of Interdisciplinary Medicine

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

{"article-title":"Left Pulmonary Artery Agenesis in a Pediatric Patient \u2013 Case Report"}

Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular...

Left Pulmonary Artery Agenesis in a Pediatric Patient – Case Report

Online veröffentlicht: 06. Juli 2016

Seitenbereich: 88 - 91

Eingereicht: 14. Apr. 2016

Akzeptiert: 17. Mai 2016

DOI: https://doi.org/10.1515/jim-2016-0017

Schlüsselwörter
pulmonary artery, congenital heart disease, pulmonary hypertension

© 2016 Cristina Blesneac et al., published by De Gruyter Open

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Left Pulmonary Artery Agenesis in a Pediatric Patient – Case Report

Online veröffentlicht: 06. Juli 2016

Seitenbereich: 88 - 91

Eingereicht: 14. Apr. 2016

Akzeptiert: 17. Mai 2016

DOI: https://doi.org/10.1515/jim-2016-0017

Schlüsselwörterpulmonary artery, congenital heart disease, pulmonary hypertension

© 2016 Cristina Blesneac et al., published by De Gruyter Open

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Schlüsselwörter
pulmonary artery, congenital heart disease, pulmonary hypertension