The image is the key: unilateral absence of a branch of the pulmonary artery

Blanca Jover, Enrique; Bueno García, Maria del Mar; Lozano White, Amy; Gómez Nievas, José Antonio; Uberos Fernández, Jose

La imagen es la clave: ausencia unilateral de una rama de la arteria pulmonar

dc.contributor		en-US
dc.contributor		es-ES
dc.creator	Blanca Jover, Enrique
dc.creator	Bueno García, Maria del Mar
dc.creator	Lozano White, Amy
dc.creator	Gómez Nievas, José Antonio
dc.creator	Uberos Fernández, Jose
dc.date	2023-08-18
dc.date.accessioned	2023-11-29T13:41:03Z
dc.date.available	2023-11-29T13:41:03Z
dc.identifier	http://www.revistachilenadepediatria.cl/index.php/rchped/article/view/3942
dc.identifier	10.32641/andespediatr.v94i4.3942
dc.identifier.uri	https://revistaschilenas.uchile.cl/handle/2250/238989
dc.description	Unilateral absence of pulmonary artery (UAPA) is a rare and underdiagnosed entity. Due to its varied clinical expression, especially respiratory and most frequently associated with congenital heart disease, it can also present in isolation and remain asymptomatic for a long time. There is no consensus on its treatment, which is generally reserved for the presence of complications, mainly pulmonary hypertension, hemoptysis, or recurrent respiratory infections. Objective: To describe two cases of UAPA identified early in life, and the usefulness of routine imaging tests for its diagnosis. Clinical Cases: We present two clinical cases of asymptomatic children referred for pediatric cardiological assessment. The first one was 1 month old, with a prenatal diagnosis of right aortic arch, that was referred to rule out associated anomalies. An echocardiographic evaluation confirmed left isolated UAPA, sustained by plain radiography. CT angiography confirmed UAPA and left lung hypoplasia. Only clinical follow-up was determined. The second case is a 2-week-old neonate who was referred due to a systolic murmur. Right pulmonary artery agenesis was diagnosed by echocardiography with no other associated defects and was confirmed by CT angiography. Plain chest radiography showed no alterations initially, however, after 1.5 months of follow-up, right lung collapse was detected so clinical follow-up was decided. Both cases were suspected by echocardiography and confirmed by CT angiography. At 1.5 years and 6 months old, respectively, they show a good evolution, with good growth, and close monitoring of their intercurrent respiratory processes. Conclusions: UAPA can be asymptomatic when occurs in isolation and go unnoticed. Through simple imaging methods, especially echocardiography, it can be suspected and must be confirmed later by CT angiography or MRI angiography.	en-US
dc.description	La ausencia unilateral de una rama de la arteria pulmonar (AURAP) es una entidad infrecuente y subdiagnosticada. Con expresión clínica variada sobre todo respiratoria, y más frecuentemente asociada a patología cardiaca congénita, puede presentarse aisladamente y permanecer asintomática largo tiempo. No hay consenso sobre su tratamiento, reservado a las complicaciones, principalmente hipertensión pulmonar, hemoptisis o infecciones respiratorias recurrentes. Objetivo: Describir dos casos de AURAP identificados en edades precoces, y la utilidad de las imágenes rutinarias para su diagnóstico. Casos Clínicos: Se presentan dos lactantes asintomáticos remitidos para evaluación cardiológica. El primero, con diagnóstico prenatal de arco aórtico derecho, derivado al mes de vida para descartar anomalías asociadas. En la ecocardiografía se constató AURAP izquierda aislada, apoyada con radiología básica. La angiotomografía confirmó AURAP e hipoplasia de pulmón izquierdo. Se decidió sólo seguimiento clínico. El segundo, neonato de 2 semanas derivado por hallazgo de soplo sistólico. Se diagnosticó ecocardiográficamente agenesia de rama pulmonar derecha sin otros defectos asociados, confirmado con angiotomografía. Radiografía torácica básica inicialmente sin alteraciones. En control a las 6 semanas se identificó colapso pulmonar derecho. Se decidió seguimiento clínico. Ambos casos fueron sospechados mediante ecocardiografía, se confirmaron con angiotomografía. Con 1,5 años y 6 meses respectivamente, presentaron buena evolución, buen crecimiento, y control estrecho de los procesos respiratorios intercurrentes. Conclusiones: La AURAP puede cursar sin síntomas cuando se presenta aisladamente, y pasar desapercibida. Mediante los métodos de imagen simple entre los que destaca la ecocardiografía puede sospecharse, debiendo confirmarse posteriormente mediante la realización de angiotomografía o angioresonancia.	es-ES
dc.format	application/pdf
dc.format	application/pdf
dc.language	spa
dc.language	eng
dc.publisher	Sociedad Chilena de Pediatría	es-ES
dc.relation	http://www.revistachilenadepediatria.cl/index.php/rchped/article/view/3942/4493
dc.relation	http://www.revistachilenadepediatria.cl/index.php/rchped/article/view/3942/4638
dc.relation	http://www.revistachilenadepediatria.cl/index.php/rchped/article/downloadSuppFile/3942/12362
dc.relation	http://www.revistachilenadepediatria.cl/index.php/rchped/article/downloadSuppFile/3942/12363
dc.relation	http://www.revistachilenadepediatria.cl/index.php/rchped/article/downloadSuppFile/3942/12370
dc.relation	http://www.revistachilenadepediatria.cl/index.php/rchped/article/downloadSuppFile/3942/12418
dc.relation	http://www.revistachilenadepediatria.cl/index.php/rchped/article/downloadSuppFile/3942/16507
dc.relation	http://www.revistachilenadepediatria.cl/index.php/rchped/article/downloadSuppFile/3942/16544
dc.relation	http://www.revistachilenadepediatria.cl/index.php/rchped/article/downloadSuppFile/3942/12361
dc.rights	Copyright (c) 2023 Andes Pediatrica	es-ES
dc.rights	http://creativecommons.org/licenses/by/4.0	es-ES
dc.source	Revista Chilena de Pediatría; Vol. 94, Núm. 4 (2023): Julio - Agosto; 529-535	en-US
dc.source	Andes Pediatrica; Vol. 94, Núm. 4 (2023): Julio - Agosto; 529-535	es-ES
dc.source	2452-6053
dc.source	2452-6053
dc.subject		en-US
dc.subject	Unilateral Absence of Pulmonary Artery; Echocardiography; Pulmonary Artery; CT Angiography; Congenital Heart Defect; Pulmonary Hypoplasia	en-US
dc.subject		es-ES
dc.subject	Ausencia Unilateral de Ramas Pulmonares; Ecocardiografía; Arteria Pulmonar; Angiotomografía; Cardiopatía Congénita; Hipoplasia Pulmonar	es-ES
dc.title	The image is the key: unilateral absence of a branch of the pulmonary artery	en-US
dc.title	La imagen es la clave: ausencia unilateral de una rama de la arteria pulmonar	es-ES
dc.type	info:eu-repo/semantics/article
dc.type	info:eu-repo/semantics/publishedVersion
dc.type		en-US
dc.type		es-ES

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