Abstract

CAMBRONERO-CORTINAS, Esther et al. Unusual distal right pulmonary artery origin from right ductus arteriosus with uncommon left-sided aortic arch. Rev. Colomb. Cardiol. [online]. 2019, vol.26, n.5, pp.300-303. ISSN 0120-5633.  https://doi.org/10.1016/j.rccar.2018.08.004.

A bilateral persistent ductus arteriosus with right proximal pulmonary artery agenesis is a very unusual anatomic variant. These malformations are assumed to occur during the transformation of the sixth aortic arch with the pulmonary trunk in the development of the aorta and its branches.

A 2-months preterm baby was referred by severe bronchopulmonary dysplasia. Transthoracic echocardiography showed a large pulmonary trunk and left pulmonary artery. No right proximal pulmonary artery was found. Cardiac computed tomography study showed bilateral ductus arteriosus. The right proximal pulmonary artery was originated from an arterial structure coming off the right subclavian artery, a right ductus arteriosus. The aortic arch was left-sided, with a peculiar variant at the exit of the supraaortic vessels. Right subclavian artery emerged as the first supraaortic vessel, followed by a common trunk, giving rise to both carotids and the left subclavian artery as the last vessel. Right lung was hypoplastic with interstitial disease.

The calibre of the right proximal pulmonary artery was inadequate to perform a corrective surgery. LDA closure was performed, keeping a prostaglandin infusion to maintain right ductus arteriosus patency until right proximal pulmonary artery size will be big enough to perform a successful surgical reconstruction. Unfortunately, right ductus arteriosus became narrow at its origin and she developed severe pulmonary hypertension and the patient died.

Keywords : Bilateral ductus arteriosus; Unilateral absence of the proximal right proximal pulmonary artery; Pulmonary hypertension..

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